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complication following fracture of a central venous catheter can be catastrophic to both the patient and the attending doctor .
catheter fracture has been attributed to several factors namely prolong mechanical force acting on the catheter , and forceful removal or insertion of the catheter .
the tip of the catheter was notably missing , and an emergency chest radiograph confirmed our diagnosis of a retained fracture of central venous catheter .
the retained portion was removed by the interventional radiologist using an endovascular loop snare and delivered through a femoral vein venotomy performed by the surgeon .
endovascular approach to retrieval of retained fractured catheters has helped tremendously to reduce associated morbidity and the need for major surgery .
the role of surgery has become limited to instances of failed endovascular retrieval and in remote geographical locations devoid of such specialty .
werner forssman first reported the insertion of a central venous catheter ( cvc ) almost 86 years ago .
thirty years later , introduction and wide spread use of the seldinger technique made the procedure safer and easily reproducible ( 1 ) .
patients with end stage renal impairment are often the most common subset of patients whom require insertion of central venous catheters .
the catheter acts as a hemodialysis access point pending creation of a more permanent arterio - venous fistula ( avf ) .
it is also inserted in patients presenting with blocked avf , for the purpose of emergency hemodialysis ( 2 ) .
central venous catheter related complications can be classified into mechanical events , infection and venous thrombosis ( 3 ) .
since the first report of an embolised catheter fragment in 1954 , overall complication rates have remained low and are only reported to occur in less than 1% of cases .
prior to introduction of endovascular approach , retrieval of a fractured segment almost always required high risk open surgery such as thoracotomy ( 5 ) . since the late 70 's , combined endovascular and open approach has seen much success and gained popularity as first line treatment in the advent of a retained fractured central venous catheter ( 6 ) .
limitation of the use of this approach would be in rural health care settings devoid of access to an interventional radiologist .
we report our experience with a successful retrieval of a central venous catheter utilizing the combined endovascular and open approach .
a third year medical resident noticed an incomplete catheter tip post removal of a right subclavian central venous catheter in a 56 year old man diagnosed to have end - stage renal disease .
the patient was otherwise well and did not complain of chest pain or shortness of breath .
the catheter had been earlier used as a temporary access for hemodialysis pending maturation of a more permanent brachiocephalic arterio - venous fistula ( avf ) . during the visit to the nephrology outpatient clinic
, it was decided that the avf was ready for use and that the central venous catheter was no longer needed .
emergency chest radiograph was requested , and it confirmed the diagnosis of retained fractured central venous catheter ( figure 1 ) .
the case was discussed with the interventional radiologist , and a central venogram was performed to identify the exact location of the fractured segment and to plan for retrieval approach .
the venogram noted the fractured catheter segment to lie at the junction of the right brachioceohalic vein and subclavian vein ( figure 2 ) .
following discussion with the vascular surgeons interventional radiologists , decision was made to proceed with combined endovascular and open approach of retrieval . under image intensifier guidance ,
a 15 mm amplatz gooseneck snare ( ev3 inc , usa ) was inserted through the right femoral vein , and the fractured catheter fragment pulled - back all the way to the groin region .
the surgical team then externalized the retrieved fragment through a femoral vein venotomy , all the while ensuring good proximal and distal control ( figures 3 , 4 ) .
the venotomy site was then closed using non - absorbable sutures in the usual manner .
the patient was discharged the next day and continued with his hemodialysis through his matured brachiocephalic avf .
chest radiograph showing the right subclavian catheter prior to planned removal retained portion of catheter seen on venogram the tip of the retained part as being extracted from femoral vein via groin incision the fractured retained part of the catheter after being completely retrieved in one piece
central venous catheters are increasingly used worldwide in critically ill patients for administration of intravenous resuscitation fluids and medication , parenteral nutrition , chemotherapy agents , and monitoring of central venous pressure .
they have become ubiquitous in the intensive care unit and are available as single and multi - lumen variety . despite being a commonly performed procedure , it is not devoid of risks and complications .
mechanical complications include hemorrhage , pneumothorax , cardiac arrhythmias , malposition , catheter fracture and distant embolization of the fractured fragment into cardiac chambers or pulmonary artery .
venous thrombosis and central vein occlusion have also been noted to occur with long - term central vein catheter placement ( 3,4 )
patients with a fractured catheter may remain asymptomatic or experience shortness of breath , chest pain and even syncope .
diagnosis is often incidental and made in the presence of a radiopaque fragment on routine chest radiograph .
incomplete catheter tip noted during the process of catheter removal may also give rise to clinical suspicion that would warrant further radiological confirmation .
mechanism of catheter fracture has been largely attributed to mechanical shearing forces acting on the catheter over a prolonged period .
although it is a less popular approach , the supraclavicular technique is actually a safer option as it avoids many of the complications otherwise noted in the infraclavicular counter - part .
complications such as guide wire kinking , catheter compression and catheter fracture are less frequently seen in supraclavicularly placed catheters .
the reason for the apparent superiority has been studied and linked to the coaxial lie of the catheter itself within the vessel , and absence of need to maneuver through narrow musculo - osseous spaces ( 7 ) .
besides being fractured , the retained portion of the catheter may also dislodge and embolise distally .
mortality rate associated with intravascular foreign body embolization has been reported to range anywhere from 24% to 60% ( 3,8 ) .
retrieval of retained intravascular devices may be approached through the conventional open technique or percutaneous method .
to date , there are no consensus or clear guidelines for management of a fractured and retained central venous catheter . over the years
, endovascular approach to retrieval of such intravascular foreign bodies has been reported with much success ( 6 ) .
the lack of need for major open surgery has propelled its popularity due to the obvious reduction in overall morbidity and mortality .
patients who were subject to the endovascular approach were also able to assume normal activity of daily living sooner and experience early return to work . given such positive results , conventional surgical approaches have been limited to instances of failed endovascular approach or in rural areas devoid of access to an interventional radiologist .
percutaneous retrieval of intravascular foreign bodies was initially reserved for retained catheters and wire fragments . in recent years
, the spectrum of intravascular device and objects has broadened significantly to include items such as vena cava filters , embolization coils , and endovascular stents . a wide variety of percutaneous tools
they include snares , biopsy forceps , dormia baskets , and tip - deflecting wires ( 6 ) .
the amplatz gooseneck snare ( ev3 inc , usa ) used in our patient is a nitinol based micro snare that has gained tremendous popularity over the years . the snare 's super - elastic construction makes it less likely to be kinked or deformed during introduction into the vessel of choice .
the snare catheter also contains gold tungsten loops that provide excellent radiopacity for improved procedural precision ( 9 ) .
given the possible catastrophic complications following fracture of an intravascular catheter or device , it has become prudent to ensure that all cases of catheter fracture or retained catheter fragments are treated as an emergency situation that mandates prompt removal .
due to the superiority and good safety profile , percutaneous retrieval technique should be the treatment of choice ( 8) .
loop snares such as the amplatz gooseneck snare ( ev3 inc , usa ) should be used when and if available . in the advent of failure to retrieve the retained catheter using a loop snare ,
the likelihood of success with other percutaneous tools is low , and open surgery should be considered ( 8,9 ) .
this , however , does not rule out the need for surgery in case of failed endovascular therapy . caudal regression syndrome ( crs ) is a rare disorder of distal spinal segments affecting the development of the spinal cord , with attendant sequelae .
the exact etiology is elusive , though maternal diabetes , genetic factors , and hypoperfusion might play roles .
we report late presentation of crs in a 9 year old with scoliotic deformity with an asymptomatic cervical syrinx , in absence of any systemic abnormality .
a 9-year - old girl was referred in view of a lateral deformity of dorso lumbar spine to explore the possibility of an underlying skeletal dysplasia .
there was no history of trauma , associated weakness of any part of body , bowel and bladder involvement or protruding mass over the spine .
child was born of third degree consanguious mating at full term by normal vaginal delivery at home . according to mother
she was of normal intelligence with intelligence quotient ( iq ) of 105 on developmental assessment scales for indian infants ( dasii ) score .
clinical examination revealed short stature with short trunk , the height being less than 3 centile with upper to lower segment ratio 0.9 .
examination revealed multiple hypo pigmented patches over the left ear lobule , left angle of mouth , and mid dorsum of right leg .
additional finding was evidence of dysgenesis at lumbo sacral region with abnormal orientation of sacrum [ figure 1 ] . magnetic resonance imaging ( mri )
spine revealed butterfly vertebrae at d10 level , the first three sacral segments were hypoplastic , distal sacrum and coccyx were absent .
a syrinx was present in cervico - thoracic region opposite c5-t1 vertebrae [ figure 2 ] .
x - ray showing lumbosacral dysgenesis with abnormal orientation of sacrum saggital neuroimaging shows rudimentary disc at l1-l2 , l3-l4 , visualization of only s1 and s2 segments and non - visualization of distal sacral segments and coccyx
crs is a rare congenital malformation characterized by varying degrees of developmental failure first described by duhmel in 1964 to explain the spectrum of sacrococcygeal malformations .
the developmental defects include the lower extremities , the lumbar spine , the coccygeal and thoracic vertebrae , and corresponding segments of spinal cord .
a male to female ratio of 2.7:1 has been reported . however , upto 22% cases of crs are associated with diabetes mellitus in the mother , diabetic women being 200 times to 400 times more likely to have a child with crs .
group 1 has blunt spinal cord termination above l1 , and is the most severely affected .
group 2 has less severe dysgenesis with low - lying tapered spinal cord and tethered cord , which may be caused by tight filum , lipoma , etc .
, thus , the best diagnostic clue for crs is dysgenetic lumbosacral vertebrae and abnormal distal spinal cord .
the latter may explain or at least aggravate the scoliosis that was the only clinical sign in this child .
the sacral dysgenesis below s2 suggests this is a group 2 case , therefore an associated tethering mechanism should be suspected .
alternatively , probably the scoliosis may be explained by vertebral anomalies at d10 , l1 - 2 and l3 - 4 instead of a tethering mechanism , it is unlikely that a child with spinal cord tethering associated with a crs would merely present with a scoliosis . on the other hand , it is even more unlikely to presume there would be a scoliosis without an associated tethering mechanism , unless the scoliosis is related to the malaligned dysgenetic vertebra as seen in our case .
the exact etiology of crs at embryonic level is thought to result from defect in induction of caudal elements in the embryo before the 4 week of gestation .
the insult occurs at midposterior axis mesoderm , causing the absence of the development of the mesoblastic caudal bud .
the proximity and interdependence of developing caudal neurons , spinal , hindgut and mesonephric elements involved in closure of the neural tube result , in the constellation of neural , distal vertebral , anorectal , renal , and genital abnormalities .
the attenuation of bone morphogenetic protein signaling at the posterior primitive streak of embryos leads to the caudal dysmorphogenesis including the cloaca and fusion of both hind limbs .
hedgehog - responding cells derived from peri - cloacal mesenchyme contribute to the urogenital / reproductive organs .
these findings indicate the existence of developmental programs for the coordinated organogenesis of urogenital / reproductive tissues based on growth factor function and crosstalk .
interestingly , structures that are developmentally separate from these caudal elements such as the brain , proximal spine and spinal cord , are generally spared by crs .
the consequences of the disruption after the maturation of spinal cord 's caudal portion ensue after the 4 week of gestation are different .
this results in motor deficits and neurologic impairment , varying from incontinence of urine and feces to complete neurologic loss . understandably , most children affected by crs- except the very mild cases - would have some problem with genito - urinary and/or anorectal anatomy and function while our case was surprisingly asymptomatic till 7 years of age . short trunk , late onset scoliosis and absence of anomaly of any other system are the unusual manifestations of our case . the presence of an asymptomatic cervical syrinx in association with crs in our case is also intriguing .
the overall radiological incidence of syringomyelia in patients with distal spinal abnormalities has varied from 19% to 22.5% in asymptomatic group to 48.5% in symptomatic group . if one were to consider the most accepted theories concerning the pathogenesis of syringomyelia in children with crs , chiari ii malformation , hydrocephalus and tethered cord favour the formation of a cavitating lesion of the spinal cord .
in fact , a sizeable spina bifida population , screened with cranial and spinal ultrasound in infancy , showed the absence of syringomyelia .
the syrinx may start after the 1 year of life , and remain asymptomatic for a variable amount of time . in our child
it was interesting to note the presence of segmental vitiligo in our patient with crs , which might be a chance assoction .
neurosurgical consultation taken advocated stringent follow - up , but decided against prophylactic untethering in absence of neurological symptoms .
given the fact that genetic and environmental causes may produce a very similar phenotype , a sharp division between syndrome and association is nearly impossible and makes counselling for recurrence risk challenging . the term
caudal regression is probably incorrect since more than caudal structures are involved and nothing regressed that was previously present . | backgroundcomplication following fracture of a central venous catheter can be catastrophic to both the patient and the attending doctor .
catheter fracture has been attributed to several factors namely prolong mechanical force acting on the catheter , and forceful removal or insertion of the catheter.case detailsin the present case , the fracture was suspected during the process of removal .
the tip of the catheter was notably missing , and an emergency chest radiograph confirmed our diagnosis of a retained fracture of central venous catheter .
the retained portion was removed by the interventional radiologist using an endovascular loop snare and delivered through a femoral vein venotomy performed by the surgeon.conclusionendovascular approach to retrieval of retained fractured catheters has helped tremendously to reduce associated morbidity and the need for major surgery .
the role of surgery has become limited to instances of failed endovascular retrieval and in remote geographical locations devoid of such specialty . |
it is caused by trauma , stress , sepsis , adrenal tumors , anticoagulation , hemorrhagic disorders and pregnancy .
idiopathic adrenal hematoma. the symptoms vary from subclinical to clinical , such as nausea , abdominal pain , fever and hypotension due to circulatory collapse .
adrenal tumors associated with hemorrhage primarily include pheochromocytomas , adrenocortical cancers and metastatic lesions from other organs . from a clinical perspective
, whether the lesion is benign or malignant is an important issue but is difficult to determine prior to surgery .
a 59-year - old japanese man was referred to our hospital for evaluation of a 7.0 cm mass in the posterior segment of the liver or right adrenal gland in november 2009 .
this mass was discovered when abdominal ultrasonography was obtained to evaluate a weight loss of 8 kg from may to november 2009 .
his body temperature was 37.0c , his blood pressure was 124/73 mm hg , and his pulse was regular at 67/min .
hormonal examination revealed a slightly elevated metanephrine level ( 0.35 mg / day ; normal range 0.040.19 ) .
tumor marker levels , including serum -fetoprotein , carcinoembryonic antigen and carbohydrate antigen 19 - 9 were all within normal ranges . only pivka - ii was slightly elevated at 53 mau / ml ( normal < 37 ) .
abdominal computed tomography ( ct ) demonstrated a well - demarcated , 7.0 cm lesion in either the posterior segment of the liver or in the right adrenal gland .
the peripheral region was slightly enhanced and the central part was hypovascular , indicating central necrosis ( fig .
there was no evidence of lymph node enlargement or distant metastasis . on magnetic resonance imaging ( mri ) ,
, the mass showed arterial enhancement and a ct filling defect in the right superior and inferior adrenal arteries and arterial portography , respectively ( fig .
i - meta - iodo - benzylguanidine ( mibg ) scintigraphy demonstrated no aberrant accumulation in the mass , which ruled out the possibility of a pheochromocytoma .
for the purposes of securing a definite diagnosis and treatment , an open right adrenalectomy was planned for march 2010 .
the resected specimen was 7.0 5.5 cm in size , and the cut surface of the mass was heterogeneous and dark red and white in appearance ( fig .
hematoxylin - eosin staining revealed hemorrhage , necrosis and hemosiderin deposit with normal adrenal tissue .
the patient had an uneventful postoperative course and was discharged home on postoperative day 12 .
small adrenal branches from the three main adrenal arteries form a subcapsular plexus , and the gland is drained by relatively few venules .
for example , stress increases adrenal vascularity and increases adrenal venous pressure due to vasoconstriction , resulting in intraglandular hemorrhage .
idiopathic adrenal hematomas are difficult to diagnose prior to surgery . in japan , koizumi et al . described 14 cases of idiopathic adrenal hematomas from 1983 to 2010 .
of these 14 cases , 13 had been suspected to be adrenal tumors including malignant lesions preoperatively , but were then found to be idiopathic adrenal hematomas on pathological examination after surgery . only 1 patient was diagnosed with an idiopathic adrenal hematoma without surgery .
the adrenal hemorrhage was not suspected to be caused by an adrenal tumor on the basis of ct , hormonal assay and mibg results , and this mass spontaneously regressed after 2 weeks .
although imaging modalities such as ct and mri are helpful in detecting adrenal hemorrhage , it is difficult to determine whether the hemorrhage is associated with tumors or not .
adrenal hemorrhage associated with pheochromocytoma ( 48% ) was the most frequently observed situation , and the second most frequently observed was a malignant lesion ( 20% ) such as adrenocortical cancer or metastasis from another organ .
hematomas derived from pseudocysts or adenomas comprised 17% of all cases . therefore , even if pheochromocytoma is ruled out by hormonal evaluations and imaging studies , the possibility that the mass may be a malignant lesion remains approximately 50% .
furthermore , the size of adrenal incidentalomas , which are adrenal gland masses discovered serendipitously on imaging , is an important factor in differentiating benign tumors from malignant lesions .
nieman recommended routine surgical resection for adrenal incidentalomas > 4 cm in diameter without a clear - cut diagnosis . in our case , metastatic liver tumors , hemorrhaged hepatic cellular carcinoma with central necrosis and adrenal tumor were raised as differential diagnoses at the time of the initial consultation because of ring enhancement with hypovascularity on enhanced ct and a slightly elevated pivka - ii level .
subsequently , abdominal angiography revealed the origin of the mass to be the right adrenal gland .
although vital signs , laboratory data and mibg scintigraphy ruled out the possibility of a pheochromocytoma , the possibility of another malignancy could not be completely ruled out .
therefore , surgery was strongly recommended for a definite diagnosis as well as for treatment .
the reason for the patient 's weight loss and the increase in the pivka - ii in our case was not clear , but may have been related to exhaustion during the summer season from may until november that kept him from eating sufficiently .
in fact , his weight loss ceased after he consulted our hospital , and the pivka - ii value also normalized prior to surgery . in conclusion , we report herein a case of idiopathic adrenal hematoma .
an accurate diagnosis of idiopathic adrenal hemorrhage is quite difficult to make prior to surgery .
some imaging modalities are useful in generating a differential diagnosis , but if the potential for malignancy is not excluded , surgical resection should be taken into consideration .
parturient with congenital heart block may be asymptomatic but can present with sudden vascular collapse , especially during labour .
few patients with congenital heart block may have sudden cardiac death ( scd ) for which there are no predictors .
we present our experience of spinal anaesthesia in a 29-year - old female with congenital complete heart block for lower segment caesarean section ( lscs ) .
a 29-year - old parturient ( gravida 3 , para 0 , abortion 2 ) was admitted to our hospital with 9 months of amenorrhea .
she had undergone appendicectomy 8 years back under spinal anaesthesia , and the procedure was uneventful .
past obstetric history revealed two abortions and an episode of perioperative seizures during intravenous sedation for medical termination of pregnancy .
the episode was associated with bradycardia non - responsive to atropine , during which she was evaluated as having a congenital complete heart block .
she had been prescribed orciprenaline by a local practitioner and had received orciprenaline 10 mg bd for 2 months preceding the present pregnancy and till 10 days before presenting to the hospital .
it was stopped by our cardiologist as it does not help to increase heart rate in complete heart block .
her general condition was stable ; pulse rate was 46/min and blood pressure ( bp ) was 110/70 mmhg . clinically , cardiorespiratory and central nervous system examinations were normal .
per - abdomen examination showed a foetus in cephalic presentation , uterine height at 38 weeks , foetal heart rate 136 beats / min and regular with clinical and ultrasonographic evidence of reduced liquor .
electrocardiography showed a complete heart block with an atrial rate of 80/min , ventricular rate of 46/min and a narrow qrs complex [ figure 1 ] .
she was accepted for anaesthesia under asa ii and was explained about the anaesthetic technique . patient was kept nil oral for 8 h. tab .
metoclopramide 10 mg the next morning 2 h before the surgery . on the morning of the surgery
, the patient was taken for cardiac catheterization in supine position with a wedge under the right buttock and temporary pacemaker insertion ( ventricular , ventricle inhibition
the position of the lead in the right ventricle was confirmed by fluoroscopy , with a lead shield cover over the abdomen of the patient .
a pacemaker can also be inserted using electrocardiographic and echocardiographic guidance to avoid foetal exposure to ionizing radiation , but our cardiologist could not get enough window to do the same . immediately after the procedure ,
a spinal anaesthesia was given in the l3-l4 interspace with a total of 1.5 ml , which is a combination of a 1.0 ml hyperbaric 0.5% bupivacaine with 0.5 ml fentanyl ( 25 g ) .
intra - operatively , the first episode of hypotension after the spinal anaesthesia was treated by increasing the pacing rate to 70 beats / min and the second episode was treated by 3 mg of intravenous ( iv ) ephedrine .
a healthy male baby weighing 2.5 kg was born with an apgar score of 8/10 in the 1 minute and 9/10 in the 5 minute .
post - operatively , the pacemaker rate was changed to 60 beats / min and the patient was shifted to the post - operative ward for continuous monitoring .
post - operative pain relief was achieved with iv tramadol 50 mg and diclofenac 75 mg iv alternatively 8 hourly .
the temporary pacemaker was removed after 24 h and the patient was haemodynamically stable after the removal .
her post - natal period was uneventful and she was discharged on the 5 day with an advice for the placement of permanent pacemaker as early as possible .
heart block may be congenital or acquired . congenital heart block may occur alone or in association with other cardiac abnormalities .
conversely , in isolated complete heart block , 8590% of all births live beyond the neonatal period , even up to late adulthood .
if congenital complete heart block occurs alone , then it is relatively benign , as the block to conduction is at the level of the av node .
the ventricular pacemaker is proximal to the bifurcation of the bundle of his , and therefore the qrs complexes are narrow , and the ventricular conduction system intact .
the rate is relatively high and can vary from 40 to 80 beats / min , and may increase with exercise , atropine or sympathomimetics .
acquired heart block in children or early adulthood is mostly secondary to cardiac surgery involving closure of perimembranous or infundibular ventricular septal defect ( vsd ) or muscle bundle resection near the conduction tissues , but can occur as an isolated condition also . in the chronic type ,
the atrio ventricular ( av ) junction or bundle branches are usually involved , the qrs complexes are wide and the heart rate is lower and is not increased by exercise or atropine .
prophylactic placement of a pacemaker is not indicated in an asymptomatic pregnant patient with complete heart block as it does not cause unusual problems.[35 ] if the patient is symptomatic during her first and second trimesters , then the placement of a permanent pacemaker is indicated .
we used temporary pacing in this patient because the patient 's heart rate was resistant to exercise and atropine , and the same can not adapt to her changing bp .
increase in the heart rate during labour is essential to increase in the cardiac output and to maintain the haemodynamics , which is not possible in the patient .
hence , for a safe delivery , temporary pacemaker insertion was essential , which ideally we did before operative delivery .
there are quite a few anaesthetic problems in patients with complete heart block undergoing incidental surgeries .
the anaesthetic technique that least alters the cardiac stability should be wisely planned and executed for the procedure .
general anaesthesia carries a potential risk to these patients because both the inhalational and the intravenous agents alter the haemodynamics to such an extent to put them in peril.[68 ] if general anaesthesia is planned , drugs with minimal effects in depressing the heart rate have to be preferred , such as ketamine for induction , pancuronium for relaxation and isoflurane for maintenance .
combined spinal epidural is another option , but we did not opt for it due to cost constraints and brevity of surgery .
modi et al . successfully managed such a case with the epidural anaesthetic technique . in our patient ,
we opted for intrathecal opioids , especially fentanyl , which gives adequate anaesthesia with minimal effects on the cardiovascular system .
we added 25 g of fentanyl with almost half the usual dose of hyperbaric 0.5% bupivacaine .
we did use 3 mg of vasopressor ephedrine , but such a single low dose to maintain adequate haemodynamic stability was found satisfactory to us .
we did insert a temporary pacemaker to compensate for any possible haemodynamic eventuality that can occur during anaesthesia , and the immediate post - partum period .
the pacemaker rate was set at 50/min in order to preserve her native rhythm till her blood pressure is maintained in the intra - operative period .
long - term pacing suppresses the native rhythm , and our intention was to avoid it .
also , the fluoroscopy time for positioning the lead was minimized to less than 10 s. the patient was discharged with an advice for permanent pacemaker implantation ( ppi ) . as per the recent guidelines , ppi is indicated for all congenital complete heart blocks ( class 2a / b ) as trials have shown that there is a subgroup of patients who may have scd for which there are no predictors available .
to conclude , we successfully managed a case of congenital complete heart block for operative delivery with temporary pacemaker in situ with intrathecal low - dose bupivacaine | we report herein a case of idiopathic adrenal hematoma .
a 59-year - old japanese man was referred to our hospital for evaluation of a 7.0 cm mass in the right upper abdominal cavity .
the tumor was suspected to originate from either the posterior segment of the liver or the right adrenal gland .
his chief complaint was weight loss of 8 kg over the previous 6 months .
he had no past medical history and took no medications , including no anticoagulants .
laboratory data were almost normal except for a slight elevation of pivka - ii .
the origin of the tumor was found to be the adrenal gland , as angiography revealed the blood supply to the mass to derive from the right superior and inferior adrenal arteries .
a fine needle biopsy of the lesion was unable to confirm the diagnosis .
open right adrenalectomy was performed .
the histopathological findings of the surgical specimen revealed a hematoma with normal adrenal tissue . in the absence of any obvious etiology , the diagnosis was idiopathic adrenal hematoma . |
congenital scrotal anomalies are unusual and include penoscrotal transposition , bifid scrotum , ectopic scrotum , and accessory scrotum ( as ) . among these , as is the least frequent , with only 42 cases reported in the english literature . as is characterized by additional scrotal tissue lacking a testis , besides a normally developed scrotum .
various associated anomalies have been reported . in particular , contiguous subcutaneous tumor is the most frequently associated abnormality and is reported to be related to the etiology of as . although prenatal screening techniques have advanced , most reported cases of congenital perineal mass have been identified after birth .
a 28-year - old woman was referred to our hospital for the evaluation of a fetal perineal mass at a gestational age of 31 weeks .
prenatal ultrasonography and magnetic resonance imaging ( mri ) showed a mass of 1.0 1.2 cm located posterior to the scrotum in a male fetus .
1 ) the likely diagnosis was lipoma and the mass maintained a stable appearance until delivery .
the male newborn was delivered vaginally at 38 weeks of gestation and his body weight was 2208 g. there were no specific symptoms after birth .
arrowheads indicate a 1.0 1.2 cm mass located posterior to the scrotum . on physical examination , the soft peduncular mass , measuring 2.0 cm in diameter , was attached to a midperineal skin tag .
there was also a rugged pigmented swelling on the mass , measuring 0.7 cm in diameter , which resembled the scrotum .
the perineal mass showed high signal intensity on t1- and t2- weighted images and the signal intensity was suppressed on fat - suppressed t1-weighted images .
mri revealed no associated abnormalities of the intraabdominal organs , musculoskeletal system , or genitourinary system .
a. a soft peduncular mass with a rugged and pigmented swelling is located posterior to the normally developed scrotum . c. the mass is attached at the midperineum with a skin tag .
the preoperative diagnosis was as with perineal lipoma , and we completely excised the mass under general anesthesia at one month of age .
the postoperative course was uneventful , and the patient was discharged on the day after the operation .
there was no recurrence or functional sequelae within a follow - up period of six months .
a histological examination revealed that the peduncular mass consisted of mature adipose tissue . in this case
, it was difficult to distinguish between lipoma and normal adipose tissue pathologically . however , our clinical diagnosis was lipoma because the peduncular mass was separated from normal perineal region by the skin tag .
the rugged swelling on the peduncular mass showed smooth muscle fibers in the subcutaneous layer , which represented the tunica dartos .
3 ) the swelling was definitively diagnosed as as . histological examination . b. the peduncular mass
smooth muscle fibers in the subcutaneous layer of the rugged swelling represent the tunica dartos .
these swellings appear at four weeks of gestation and migrate to the caudal portion after 12 weeks of gestation .
abnormal migration or early division of the labioscrotal swellings is possibly related to the etiology of congenital scrotal anomalies .
the least frequent congenital scrotal anomaly is as , characterized by additional scrotal tissue without a testis , besides a normally developed scrotum .
( table ) characteristics of accessory scrotum various anomalies associated with as have been reported . in particular , contiguous subcutaneous tumor has a high incidence ( 72.5% ) of association with as .
histologically , one case of subcutaneous tumor was lipoblastoma , three cases were hamartoma , and the others were consistent with lipoma .
( table ) it is assumed that the contiguous subcutaneous tumor is related to the etiology of as .
sule hypothesized that as develops when intervening mesenchymal tissue ( i.e. , the developing subcutaneous tumor ) disrupts the continuity of the developing caudal labioscrotal swelling .
however , the complete etiology of as is not explained by this hypothesis because as can occur with no contiguous tumor .
takayasu hypothesized that as develops from the early division and teratoid growth of pluripotential labioscrotal tissue elements .
however , two cases associated with skeletal abnormalities were located in the pubic area , and one case was located on the distal penile shaft .
our case was detected at a gestational age of 31 weeks , and the other two reported cases were detected at 24 weeks and 32 weeks of gestation .
a congenital perineal mass is unusual in itself , and most reported cases have been diagnosed after birth . however , detection is possible with careful prenatal screening .
the differential diagnosis of a fetal perineal mass includes lipoma , lipoblastoma , infantile hemangioma , hamartoma , and choristoma . if a fetal perineal mass is detected during the antenatal period , it is important to look for any associated congenital anomalies .
the prognoses of surgically treated patients are good , and only one has died , from an associated anomaly before surgery .
the reported ages at surgery range from four days to 46 years ( median , nine months ) , and three adult cases are recorded in the literature .
our patient was operated upon in the neonatal period because the mass was considered to be excisable without complications and the associated subcutaneous tumor had a low probability of malignancy .
although a fetal perineal mass is difficult to diagnose , it can be detected with careful prenatal screening .
many ass are associated with contiguous subcutaneous tumors , which are assumed to be related to the etiology of as .
the recognition that elevated intracranial pressure ( icp ) is transmitted through the optic nerve and its sheath has been known for many years . this physiological process is the basis for the physical exam finding of papilledema on fundoscopic examination .
recently , interest has turned to measurement of the optic nerve sheath diameter ( onsd ) through non - invasive imaging technologies to provide surrogate markers for early elevated icp . in this issue of critical care , geeraerts and colleagues
present their research correlating magnetic resonance imaging ( mri ) measurements of onsd with icp . in a retrospective review of 38 patients with traumatic brain injury requiring both invasive icp monitoring and mri
, they found a significant positive relationship between onsd measured by mri and icp ( r = 0.71 ) .
the best cut - off value to detect an icp > 20 cm h2o based on a receiver operating characteristic curve was found to be onsd = 5.82 mm with a sensitivity of 90% and a specificity of 92% .
the optic nerve is surrounded by cerebrospinal fluid ( csf ) , which is contiguous with intracranial csf .
increased icp is transmitted through this subarachnoid space causing distention of the dural optic nerve sheath , especially the retrobulbar segment .
the optic nerve and its surrounding sheath can be imaged and measured on mri using a fat - suppressed t2-weighted sequence .
mri has been used to demonstrate increased onsd in idiopathic intracranial hypertension , and interestingly , decreased onsd in csf hypotension .
the onsd has also been shown on mri to decrease after drainage of subdural hematomas .
the research presented by geeraerts and colleagues is unique in its comparison of onsd with simultaneous direct measurements of icp through invasive monitoring .
their findings generally correlate with a growing body of research using bedside ultrasound measurements of onsd to detected elevated icp .
original research with lumbar intrathecal infusions performed by hansen and helmke demonstrated rapid changes in the onsd with alteration of csf pressures . in emergency department patients with traumatic brain injury , the onsd correlates with signs of elevated icp on computed tomography scans .
more recently , researches have compared bedside ultrasound measurements of onsd to invasive icp [ 11 - 13 ] .
while there is some variation in the optimal cut - off value , the correlation between onsd and icp remains consistent . in their current article ,
geeraets and colleagues provide further evidence of this physiological relationship and an intriguing possibility for non - invasive assessment of icp using mri . the obvious drawbacks to mri include its expense , long acquisition times , need for patient transport , and limited availability .
however , some research has shown that mri may provide more precise measurements then ultrasound . geeraerts and colleagues used a conventional t2 sequence with relatively large slice thickness and interslice spacing , resulting in an overall feasibility of measuring the onsd in 95% of patients .
greater accuracy and reliability would be expected in coronal t2 slices with thinner slices . as mri becomes more accessible and faster , non - invasive mri measurements
may prove to be useful in certain clinical settings and as a potential reference standard for further research . continued research with larger studies is required to confirm the precision and accuracy of mri measurements of onsd , as well as the optimal measurement technique .
additionally , the time course of onsd distention and reduction needs to be further delineated . currently , non - invasive assessments of icp do not obviate the need for invasive icp monitoring .
invasive monitoring detects minute to minute variations in icp and , in the case of intraventricular drains , can also be therapeutic .
however , non - invasive screening tests may be useful in select populations who would not otherwise require invasive monitoring and could undergo mri scans , such as patients with liver failure , meningitis , stroke , and moderate traumatic brain injury . in summary ,
the study by geeraerts and colleagues adds to a growing body of research demonstrating a correlation between increased onsd and elevated icp . by demonstrating the correlation of mri measurements of the onsd with invasive icp monitoring
, they illustrate the potential of yet another non - invasive method to screen for elevated icp .
while this technique will not replace invasive icp monitoring , it may be useful in select patient populations that would not otherwise have invasive monitoring but are at high risk for elevated icp .
further research is required before we can use measurements of the onsd to predict exact values of icp , but it may be useful as a screening test to estimate the probability of elevated icp .
csf : cerebrospinal fluid ; icp : intracranial pressure ; mri : magnetic resonance imaging ; onsd : optic nerve sheath diameter .
| abstractwe report a case of accessory scrotum ( as ) in the perineal region with peduncular lipoma , diagnosed prenatally .
a male fetus of 31 weeks gestation was referred to our department with a perineal mass .
prenatal ultrasonography and magnetic resonance imaging showed a mass of 1.0 1.2 cm located posterior to the scrotum .
no other abnormalities were noted during pregnancy .
the patient was delivered vaginally at 38 weeks of gestation . on physical examination , a soft peduncular mass with a rugged and pigmented swelling was located between the normally developed scrotum and the anus .
there were no specific symptoms or any other associated congenital anomalies .
we completely excised the mass at one month of age .
a histological examination revealed lipoma , with tissue suggestive of scrotum , so a definite diagnosis of as was made . as is a rare congenital anomaly of the scrotum .
we review the literature . |
children spend from 31 to 60% of their school day either
writing or performing other fine motor tasks2 .
the preparatory skills
for writing are coordination of multiple joints , visual perception , vision - motor
integration , and proprioception3 .
together with stereognosis ,
proprioception allows smooth joint movement when vision is impaired or absent5 .
children with poor proprioception have
problems with handwriting legibility because their grip on a pen is too strong or too
weak4 . generally , proprioception can be
measured with joint position sense ( jps ) and kinesthetic sense ( ks)6 . while there are many studies investigating children s
handwriting , quantitative studies on the association between handwriting and proprioception
involving jps and ks are lacking7 .
the aim of this study was to investigate the association between proprioception , involving
jps and ks , and handwriting legibility in children .
nineteen healthy children ( 15 boys and 4 girls ) with an average age of 9.7 0.36 years
participated in this study .
prior to the study , the children and their parents were informed
about the purpose of the study and the general procedures to be undertaken . all children and
the study was approved by the kaya university of
human health science studies committee .
the investigator moved the child s right arm passively
through 80 of flexion at the elbow ( from 30 to 110 ) .
the children were then directed to
repeat this motion 10 times following a metronome set at 1 s intervals .
their movements were
recorded by a compact measuring system ( cms ) 10 for 3d motion analysis ( using the winarm
software zebris medical gmbh , germany ) .
cms markers were placed at the greater tubercle and
lateral epicondyle of the right humerus and at the right wrist .
angles of deviation from the
targeted range of flexion were analyzed using matlab version 2014a ( the match works inc . ,
2014 ) .
ks was measured by the kinesthesia item in the sensory integration and praxis tests ( sipt ,
wps , torrance , ca , usa ) .
the investigator moved a finger of the subject passively along a line from
beginning to end , allowing the child to learn the line direction and distance .
then ,
blindfolded and after one trial , the children were directed to trace five lines for each
hand . only the results of the right hand were analyzed in this study .
the investigator then
measured the distance between the test endpoints and the real endpoints of the lines .
legibility was evaluated using
form , alignment , space , size , and slope8 .
the legibility score was calculated as the ratio of
the number of clearly written words to the total number of words ( score for legibility ( % ) =
number of letters that received 5 points/30 100 ) .
all data were analyzed using ibm spss statistics 20.0 ( ibm corp . , armonk , ny , usa ) .
spearman s rank correlation was used to determine the relationships between legibility of
handwriting and jps and ks , with significance defined as p<0.05 .
values obtained for jps and ks in relation to handwriting legibility are shown in table 1table 1.handwriting legibility and proprioception test results ( n=19)mean sdjoint position sense 110 ( degree)22.530 10.063joint position sense 30 ( degree)14.256 10.408kinesthetic sense ( cm)2.044 0.703handwriting legibility ( % ) 33.521 15.852*standard deviation .
there was no correlation between writing legibility and jps at either 30 or
110 of elbow flexion ( p>0.05 ) .
a high ks correlated significantly with legible
handwriting ( p<0.05 , table 2table 2.correlation between handwriting legibility and proprioception ( n=19)handwriting legibility ( % ) joint position sense 110 ( degree)0.016joint position sense 30 ( degree)0.009kinesthetic sense ( cm)0.370*p<0.05 ) .
this study investigated the association between the legibility of handwriting and jps and
ks in young children .
children aged between 7 and 8 years are expected to be proficient in
building up their speed of handwriting , ensuring consistency in size and proportions of
letters as well as the spacing between letters and words9 .
the average age of the participants in this study was 9.7 years
old .
our results showed that a highly accurate ks was
associated with higher legibility scores .
ks provides ongoing error information and memory storage to be recalled when
writing is repeated .
a high ks leads to programmed error correction , and the upgraded
program generates better writing legibility .
the results of this study support the
hypothesis that ks reinforces the linkage between visual and motor control required for
clear handwriting15 .
we
studies jps at the elbow joint because proprioception at the elbow joint is necessary for
performing fine manipulative tasks including handwriting16 .
studies have reported that proprioception at the wrist joint17 and finger joints18 influences handwriting legibility .
we looked at children within a limited age range between 9 and 10 years of age .
further , we did not control for other factors that affect writing legibility , such as fine
motor control and visual - motor integration19,20,21 .
finally , although handwriting quality is measured in terms of legibility and speed , this
study investigated only writing legibility . it includes hidradenitis suppurativa , acne conglobata , dissecting cellulitis of the scalp and pilonidal sinus .
though each of these conditions are commonly encountered on their own , as a symptom complex follicular occlusion tetrad has rarely been reported in the literature . here
we present a case of hidradenitis suppurativa in a 36-year - old male patient who also had the above mentioned associations .
a 36-yeasr - old male patient presented to us with a history of recurrent boils since18 years .
they initially started in the groin and buttocks with boils and gradually broke down to ooze pus .
the boils healed with scarring after weeks to months . the process progressively started to involve the axillae and then the chest .
the patient was treated with multiple antibiotics for over a decade that gave only symptomatic relief .
the patient was diagnosed with pilonidal sinus at the age of 28 and he underwent surgery for the same .
there was no family history of similar complaints , though his father suffered from psoriasis .
on general examination , there were multiple areas of cicatricial alopecia on the scalp [ figure 1 ] and nodular scars on lower half of the face [ figure 2 ] .
on query , the patient gave history of severe acne with nodules and pustules healing with scars on the face .
he also had patchy swellings over the scalp region associated with pain in the past , which was diagnosed as folliculitis by a physician .
patch of cicatricial alopecia on the occiput region of the scalp pustules , cysts and nodule - reminiscent of acne conglobata - on the lower half of the face on local examination , there were skin colored - to - erythematous nodules , grouped comedones and band like scars in the groin , buttocks , axillae and chest [ figure 3 and 4 ] .
the patient had difficulty in lifting arms due to pain and scars in the axillae .
multiple nodules , cysts and band like scars in the axillary region multiple nodules and pustules over the buttocks with surgical scar ( done for pilonidal sinus ) seen in the upper part of gluteal cleft a thorough general examination was followed by lab work - up .
liver function tests and renal function tests were within normal limits and so was fbs , ppbs and fasting lipid profile .
as the patient was obese , he was advised to follow a strict diet chart to lose weight .
we also started him on oral isotretinoin 0.5 mg / kg body weight once daily .
the patient came back after six weeks for prescription refill but was thereafter lost for follow - up .
follicular occlusion tetrad is a condition that includes hidradenitis suppurativa ( hs ) , acne conglobata , dissecting cellulitis of the scalp and pilonidal sinus .
hidradenitis suppurativa was first described in 1839 by velpeau ; verneuil gave it its name in 1854 and associated it with the sweat glands .
later , hs was classified as a member of the follicular occlusion triad , along with acne conglobata and dissecting cellulitis of the scalp . in 1975
, pilonidal cyst was added as a member to this triad , forming the follicular occlusion tetrad . in 1989 , plewig and steger introduced the term acne inversa to substitute the term hs .
hidradenitis suppurativa or acne inversa is a chronic , inflammatory , recurrent , debilitating skin disease that usually presents after puberty with painful , deep - seated , inflamed lesions in the apocrine gland - bearing areas of the body , most commonly the axillae , inguinal and anogenital regions .
the exact pathogenesis of this group of diseases is unknown but evidence suggests that they share the same pathological process initiated by follicular occlusion .
the pathogenesis and aetiology are unknown , but is thought to originate from poral occlusion of the pilosebaceous units .
diagnostic criteria of hidradenitis suppurativa ( adopted by the 2 international conference on hidradenitis suppurativa , march 5,2009 , san francisco , ca us ) .
typical lesions , i.e. deep - seated painful nodules :
blind boils in early lesions ; abscesses , draining sinus , bridged scars and tombstonedouble - ended pseudo - comedones in secondary lesionstypical topography , i.e. axillae , groins , perineal and perianal region , buttocks , infra and inter mammary foldschronicity and recurrences .
blind boils in early lesions ; abscesses , draining sinus , bridged scars and tombstonedouble - ended pseudo - comedones in secondary lesions typical topography , i.e. axillae , groins , perineal and perianal region , buttocks , infra and inter mammary folds chronicity and recurrences .
dissecting cellulitis(also called perifolliculitis capitis abscedens et suffodiens ) manifests with perifollicular pustules , nodules , abscesses and sinuses that evolve into scarring alopecia .
it predominantly occurs in african american men between 20 - 40 years of age , but can occasionally affect other races and women too
the tendency of dissecting cellulitis to cause severe alopecia , fluctuant nodules , and sinus tracts helps to distinguish it from acne keloidalis nuchae .
culture of dissecting cellulitis does not produce a positive fungal culture and nuchal palpation does not reveal palpable lymph nodes , though reports have noted an inflammatory tinea capitis ( kerion ) that mimicked dissecting cellulitis in adolescents .
its follicular papules and pustules spread peripherally , leaving central scarred patches of alopecia without nodules or sinuses . tufted folliculitis resolves with patches of scarring alopecia within which multiple hair tufts emerge from dilated follicular orifices .
acne conglobata is an uncommon nodulocystictype of acne vulgaris that is often resistant to therapy .
this disorder typically begins in adulthood and presents as numerous comedones , papules , pustules , nodules , abscesses , and draining sinus tracts involving the chest , back and buttocks .
these lesions frequently become secondarily infected with gram - positive bacteria and often heal with scarring .
pathology usually reveals inflammatory infiltrate around follicles , which can often disrupt the normal dermal architecture .
acne conglobata is particularly disfiguring and socially detrimental to patients because of its chronicity , severity , and treatment challenge .
acne conglobata ( ac ) resembles acne fulminans because both cause numerous inflammatory nodules on the trunk .
unlike acne conglobata , large nodules of acne fulminans tend to become painful ulcers with overhanging borders surrounding exudative necrotic plaques that become confluent .
the therapy of choice for ac includes isotretinoin 0.5 - 1 mg / kg for 4 - 6 months .
simultaneous use of systemic steroids such as prednisone 1 mg / kg / d for 2 - 4 weeks may also prove beneficial , particularly if systemic symptoms are evident .
oral tetracycline antibiotics should not be combined with oral isotretinoin because of an increased risk of pseudotumor cerebri . for treatment - resistant cases ,
dapsone 50 - 150 mg / d is recommended ; this treatment should be carefully monitored .
pilonidal sinus was described as far back as 1833 when mayo described a hair - containing cyst located just below the coccyx .
no specific laboratory studies or tests are needed to diagnose pilonidal disease and its sequelae or differentiate it from other disease entities ; it is a clinical diagnosis best elicited by history and physical examination findings .
treatment of infection or abscess with oral antibiotics followed by surgical excision of the sinus is practiced . | [ purpose ] this study investigated the association between proprioception , including joint
position sense and kinetic sense , and handwriting legibility in healthy children .
[ subjects and methods ] assessment of joint position sense , kinetic sense , and handwriting
legibility was conducted for 19 healthy children .
joint position sense was assessed by
asking the children to flex their right elbow between 30 to 110 while blindfolded .
the
range of elbow movement was analyzed with compact measuring system 10 for 3d motion
analysis .
kinetic sense was assessed using the sensory integration and praxis test .
the
children were directed to write 30 words from the korean alphabet , and the legibility of
their handwriting was scored for form , alignment , space , size , and shape . to analyze the
data , descriptive statistics and spearman correlation analysis
were conducted using ibm
spss statistics 20.0 .
[ results ] there was significant negative correlation between
handwriting legibility and kinetic sense . a significant correlation between handwriting
legibility and joint position sense
was not found .
[ conclusion ] this study showed that a
higher kinetic sense was associated with better legibility of handwriting .
further work is
needed to determine the association of handwriting legibility and speed with joint
position sense of the elbow , wrist , and fingers . |
this was a retrospective cohort study approved by our institutional review board as a quality improvement research .
the records of catheter days 1 year before the introduction of midlines ( group a : august 1 , 2011 , to july 31 , 2012 ) and catheter days of 1 year after the regular use of midlines ( group b : november 1 , 2012 , to october 31 , 2013 ) in the ventilator unit were collected ( table 1 ) . during the period from august 2012 to november 2012
, internal medicine residents and nursing staff underwent training on the use and insertion of midline catheters and their care .
this dedicated team was responsible for replacing cvcs by midlines as per our new practices .
the midlines that we used were powerwand ( access scientific , san diego , calif ) . before midline catheter ,
patient days : number of days the patient was in the ventilator unit ; catheter days : number of days the patient had a central venous catheter and after midline catheter , patient days : number of days the patient was in the ventilator unit ; catheter days : number of days the patient had a midline catheter we chose this unit because it has a constant denominator of the number and type of patients .
most of the patients in this unit are ventilator dependent and being treated for problems such as health care associated pneumonia , urinary tract infections , wound and pressure ulcer infections , or other long - term conditions requiring acute care that can not be administered at a nursing home facility .
the patients here have longer length of stay compared with other units and generally have difficult venous access .
the most common reason for cvc use in this unit is for difficult and long - term intravenous access .
indications of cvcs in these patients included antibiotic therapy , infusion therapy , diagnostic procedures , transfusions , and blood draws .
the department of infection control of the hospital reports all the clabsi per 1000 catheter days in the hospital , categorized by location and unit .
the number of clabsi during this period along with the type of bacteria and the date of culture was obtained from the microbiology department .
we compared the clabsi rates between group a and group b to see whether it decreased through the use of midlines in comparison with central lines .
we trained a team of residents for a period of 3 months in the insertion of ultrasound - guided midline catheters , which would replace cvcs by midline catheters whenever possible .
the same antiseptic precautions used in cvc catheter placements under ultrasound guidance were applied to the placement of midlines .
the policy for replacing the central lines included the following guidelines : any patient with a cvc in a femoral vein had their line removed and replaced by a midline catheter .
all patients who were not on an ionotropic agent or total parenteral nutrition had their cvc replaced by midlines .
a midline catheter replaced any cvc in place longer than a week . a patient on antibiotic therapy being sent to nursing home for further management received a midline catheter .
the total number of catheter days was compared with the rate of clabsi in the 2 groups .
catheter days were calculated as the number of central line catheters on the unit every day .
adding the total number of catheters on the unit per day and adding this daily number for the length of time of the study help calculate catheter days .
central line associated bsi was reported as rate per 1000 catheter days and can be calculated as follows : ( total number of clabsi / total number of catheter days ) 1000 .
we used a test to compare the number of catheter days per patient days in the 2 groups as well as to compare infections based on the number of catheter days in each group ( table 2 ) . comparing the number of catheter days per patient days in the 2 groups , as well as comparing infections based on the number of catheter days in each group
there was a significant decrease in the total number of catheter days on the ventilator unit in group a from 2408 catheter days in the 1 year ( august 1 , 2011 , to july 31 , 2012 ) before the introduction of midline catheters to 1521 catheter days in group b in the following year ( november 1 , 2012 , to october 31 , 2013 ; p < 0.05 in both groups ) .
the total number of clabsi infections in these periods was also significantly decreased from 8 to 0 ( table 3 ) .
this calculates to 3.32 clabsi per 1000 catheter days and 0 clabsi per 1000 catheter days , respectively ( add ) .
the number of inpatient days during these periods was 3058 and 2948 days ( table 4 and 5 ) .
continuation of the test test : observed frequency test : expected ( theoretical ) frequency , asserted by the null hypothesis there were no bsis associated with midlines in this study .
the centers for disease control and prevention has put in place guidelines to reduce the incidence of clabsi .
these guidelines brought about by numerous studies for years have helped bring down the rate of clabsi .
these measures include the following : reducing the number of dwell days by removing catheters as early as possible ; reducing the use of central lines by using other means of venous access ; proper care and technique for cvcs ; maximum sterile barrier precautions during insertion ; use of chlorhexidine for skin disinfection before catheter insertion ; avoidance of the femoral insertion site ; and use of recommended insertion site dressing care practices .
prolonged dwell time has been shown to increase clabsi rates rapidly after 9 catheter days .
replacing central lines with midlines decreases the dwell time and also the total number of catheter days .
it may be argued that the decrease in the use of central lines and thereby reducing the number may be responsible in decreasing clabsi ; however , our denominator is constant for comparison .
the use of midlines reduces the use of and the dwell days for central lines , and with this study , we show that it can be a factor in reducing clabsi as well .
another study showed that clabsi rates were higher in patients who had central lines for longer than 7 days . in our study , by replacing central lines with midlines in patients , we essentially decreased risk factors such as dressing changes , catheter care , and duration of central line use .
a more obvious outcome is the decrease in use of central lines itself , causing a drop in the infection rates .
the fall in infection rate may be attributed to the fewer number of catheters in place for longer than 7 days . before the introduction of midlines , difficult intravenous access in these chronically ill patients mandated the use of central lines .
there are other complications of central lines , including inadvertent arterial puncture ( 3% ) , hemothorax , or pneumothorax ( 1%-2% ) .
central lines and picc lines require x - ray confirmation of tip placement , exposing the patient to radiation .
midlines have complications as well ; our most common problem was the loss or malfunction of the midline including extravasations in 2 patients .
the bsi rate of midlines in various studies has been reported to be between 0% and 0.9% .
the combination of better available products and the increasing use of ultrasound guidance for intravenous catheter placement has renewed interest in midline catheters
. some limitations of midline catheters are inability to use for vasopressors , total parenteral nutrition or when large peripheral veins are not available such as in amputates and patients with arteriovenous fistulas .
we conclude that the use of midline catheters to replace central lines for difficult intravenous access decreases the rate of clabsi in a ventilator unit in a community hospital . pectus excavatum ( pe ) , characterized by posterior displacement of the sternum and cartilaginous - rib attachments , is one of the most common congenital chest wall deformities.1)2 ) the physiological impact of pe varies .
symptomatic patients frequently complain of dyspnea with exertion , progressive loss of endurance , tachycardia , palpitations and chest discomfort.3 - 5 ) in a critical location , even small depressions of the chest can create significant cardiac dysfunction when the right heart and pulmonary outflow tract are compressed to varying degrees by the depressed sternum.5 - 7 ) an index of severity can be calculated by measuring the inner width of the chest ( at the lowest level of the pectus defect ) and dividing it by the distance between the posterior surface of the sternum ( at the lowest part of the defect ) and the anterior surface of the spine ( with normal being approximately 2.52).2)3 ) in general , an index of 3.2 or greater is considered severe however symptoms do not necessarily correlate with severity of index.2 - 4 ) echocardiography plays a significant role in the evaluation of patients with pe .
the degree of right heart compression however is often difficult to assess by transthoracic echo ( tte ) especially with severe deformities that prevent the operator from obtaining the normal transthoracic views.7 - 9 )
a 32-year - old woman presented to out - patient clinic for further evaluation of a 1-year history of progressive chest pain , fatigue , dizziness , paroxysmal tachycardia and dyspnea with exertion . with even mild exertional effort she experienced sharp , " stabbing " chest pain along the left lower and mid - sternum .
a 12-lead electrocardiogram demonstrated a right - bundle branch block with left posterior fascicular block .
she had a history of severe pe ( index of > 4 ) for which she had undergone operative repair 18 months prior by an open resection of cartilage attachments and sternal " flip " as described by hawkins & colleagues.10 ) prior to her first correction , she had some dyspnea with exertion , however , cardiac work - up , including echocardiogram had been reported to not show any abnormalities .
she noted onset of her current symptoms approximately 6 months after her pe repair . a subsequent operation with superficial anterior remodeling of the chest wall cartilage on the left side of the sternum had failed to relieve her progression of pain and symptoms .
she exhibited post - operative abnormal remodeling of the chest wall with residual as well as some recurrent pe .
the sternum protruded anterior with bilateral depression of the costal - sternal attachments creating a " wave - like " appearance ( fig .
close attention to the anteroposterior planes ( best seen from the apical four - chamber views ) clearly demonstrated extrinsic compression and deformation of the lower mid - right ventricle ( rv ) by the chest wall which is more obvious during diastole ( fig .
3b ) . biplane and live 3-d images of the preoperative transesophageal echocardiogram ( tee ) improved the visualization and localization of extrinsic compression of the right ventricle ( fig .
tricuspid valve prolapse is seen likely due to partial compression of the rv resulting in distortion of the tricuspid annulus ( fig .
open revision of her chest wall deformity was performed with placement of two stainless steel support bars ( lorentz surgical , jacksonville , fl , usa ) and a trabecular metal implant ( zimmer , inc . ,
minneapolis , mn , usa ) which elevated the sternum and depressed regions 3 - 4 centimeters anterior to the rv with good cosmetic results ( fig .
symptomatic pe patients often present for cardiovascular evaluation with tte being the most commonly utilized diagnostic modality .
documenting the effects of a depressed chest wall on the right heart and outflow tract as well as any associated interference with diastolic filling is critical for decision making in patient treatment and the need for potential surgical intervention .
modifying standard views such as biplane transthoracic and transesophageal views may be necessary in some patients due to limitations from the abnormal anatomy of the deformed anterior chest wall . in some cases , rv compression is often difficult to assess by tte especially with severe deformities that prevent the operator from obtaining the normal transthoracic window views .
technical tips for assessment of the rv include 1 ) narrowing the 2-d sector width to optimize only rv structures ; 2 ) use of harmonic imaging and adjustment of gain and compression for good contrast and endocrinal edge detection ; 3 ) making all measurements at end - diastole or the frame demonstrating the largest chamber dimension ; and 4 ) for 2-d measurements , obtain all acquisitions during quiet respiration or full - expiration .
apical four - chamber views when seen clearly can usually visualize any extrinsic compression to the rv of the heart .
use of all three possible traditional acoustic windows ( parasternal short - axis , apical and subcostal ) may be necessary . in an effort to minimize foreshortening of the rv , the transducer can be positioned down an intercostal space and laterally until the rv apex is clearly seen . in some patients
a transesophageal and transgastric view may be necessary to better evaluate the right heart chambers and rv outflow obstruction .
many patients with pe have associated alterations in rv morphology and function . assessment for localized sacculation of the rv wall , global dilation of the ventricle , prominent trabeculae , and/or hypertrophy of the moderator band is important . identifying abnormalities , including mitral valve prolapse and aortic root measurements are especially critical in suspected or confirmed cases of marfan syndrome .
resolution of mitral valve prolapse with release of the chest wall entrapment is seen in more than half of patients after pe correction.7)11)12 ) more importantly , many patients improve their symptoms such as exertional dyspnea and chest pain which may be related to extrinsic compression of the rv and reduced preload.4)5)7 ) further studies are needed to better understand the pathophyiology of symptoms in relation to cardiac and chest wall function in patients with pe . in conclusion ,
echocardiographic evaluation is critical in patients with symptomatic pe to assess the degree of rv compression .
we presented a case of a pe in a patient with symptoms including severe chest pain with exertion that was diagnosed with the expertise of echocardiography and subsequently surgically corrected .
subtle abnormalities in cardiac structure and chest wall compression may result in debilitating symptoms in a small population of patients.2 - 4 ) when astutely performed , echocardiography can accurately provide clinically relevant information about cardiac size and any hemodynamic compromise in patients with pe . | hypothesisour objective was to evaluate whether the use of midline venous catheters in place of central line venous catheters , when appropriate , decreased the overall incidence of central line associated bacteremia in a ventilator unit.methodsthe time interval between february 2012 and february 2013 was divided into 2 periods .
group a was the first half of the year , before the introduction of midline catheters , and group b was the second half of the year , 6 months after their introduction .
central line associated bloodstream infection ( clabsi ) was calculated using the equation : ( total number of clabsi / total number of catheter days ) 1000 .
the z test was used for proportions between independent groups to compare the significance in the difference in clabsi between groups a and b.resultsthere was a significant decrease in the total number of catheter days on the ventilator unit in group a from 2408 catheter days in 1 year ( august 1 , 2011 , to july 31 , 2012 ) before the introduction of midline catheters to 1521 catheter days in group b in the following year ( november 1 , 2012 , to october 31 , 2013 ; p < 0.05 for both groups).conclusionsmidline catheters in place of central lines decrease the rate of clabsi in a ventilator unit .
in addition , no bloodstream infections were associated with midline catheters . |
the adenoid cystic carcinoma ( acc ) is a relatively rare epithelial tumor of the salivary glands .
it accounts for about 5 - 10% of all salivary gland neoplasms , representing 2 - 4% of malignant occurrences of the head and neck area .
approximately , 31% of lesions affect minor salivary glands , particularly the palate , although they can also be observed in the sub - mandibular and parotid glands .
the frequency reported in the tongue is 19.8% , with 85% observed at the base of the tongue .
we report one such rare case of tongue neoplasm which turned out to be acc in a middle aged lady .
a 45-year - old - female patient presented with an asymptomatic growth of the tongue , which was perceived just 2 weeks before consultation .
the intra - oral examination at that time revealed a mass in the dorsum of the tongue with light pain to pressure , without any evidence of cervical lymphadenopathy .
the mass was firm , same color as that of the surrounding mucosa and asymptomatic otherwise [ figure 1 ] .
as a pre - operative assessment of the lesion , a fine needle aspiration was done and the smear revealed a salivary neoplasm consisting of well delineated , tightly cohesive clusters of basaloid cells surrounding mucoid , hyaline globules , or clear spaces also forming honeycomb ( cribriform ) pattern [ figure 2 ] . at places
dense aggregates of monomorphic small cells with uniform round to oval hyperchromatic nuclei and scanty cytoplasm were seen .
smears also showed individual tumor cells with high n : c ratio and nuclear moulding .
macroscopically , the mass had firm consistency with an irregular form and surface , brown color and measured 2.5 1.5 1.0 cm .
the histopathologic study revealed multiple pseudocystic spaces of variable sizes surrounded by cuboidal cells with scarce cytoplasm and oval nuclei , filled with eosinophilic material and hence was consistent with the diagnosis of acc [ figure 3 ] .
however , there was no evidence of perineural infiltration on serial sections . clinical photograph showing a swelling on the dorsum of the tongue cytological smears show well - delineated clusters of basaloid cells surrounding hyaline globules with uniform round to oval hyperchromatic nuclei and scanty cytoplasm ( papanicolaou stain , 400 ) histopathological section showing multiple pseudocystic cavities of variable size composed of cuboidal cells with scarce cytoplasm and oval nuclei ( h and e , 400 )
minor salivary gland neoplasms occur less commonly than the major salivary gland tumors and tongue is a relatively uncommon site for salivary gland neoplasms
acc is a malignant neoplasm that originates in both the minor and major salivary glands , characterized by slow growth , diffuse invasion , and potential to produce distant metastases , mainly to the lungs and bones .
it is an infrequent lesion , as it represents approximately 1 - 2% of all malignant neoplasms of the head and neck , and up to 10 - 15% of all malignant salivary gland neoplasms .
the most common intra - oral site for minor salivary gland tumors is the hard palate , followed by the base of the tongue where up to 96% of all tumors are malignant , and acc represents 30% of them . on the other hand ,
one of the least frequent sites of presentation for acc is the mobile tongue , as several authors have reported an incidence of only approximately 3% of the cases .
, analyzed 178 cases of salivary gland tumors , out of which only six cases were located on the tongue .
cytologically , cribriform variety of acc can be diagnosed by hypercellular smears composed of clusters of small , relatively monomorphic epithelial cells with hyperchromatic nuclei .
these appear bright magenta in may - grunwald giemsa mgg stains and pale blue with papanicolaou stain .
finger - like process of similar material can also be found in between the groups of cells in tubular variety .
the solid variant of acc also exhibits the same material and the cells resemble that of small cells of anaplastic carcinoma .
the globules of amorphous material surrounded by the monomorphic hyperchromatic cells was a clue to the diagnosis of acc in our case , but since the hyaline globules are also found in other tumors like basal cell adenoma , pleomorphic adenoma , polymorphous low grade adenocarcinoma , epithelial myoepithelial carcinoma etc .
it is important to distinguish the adenomas from acc because of the conservative mode of management in case of adenomas .
we ruled out the adenomas because of the nature of the globules and the cytological morphology . unlike the adenomas , the hyaline globules were dense and stained intensely with mgg and the cells were relatively monomorphic , hyperchromatic with coarse chromatin and irregular nuclear membrane like that of acc .
of three histologic variants - tubular , cribriform and solid ; in our case cribriform pattern was the dominant one without any evidence of perineural infiltration .
the main factors associated with patient survival were tumor location , clinical stage , and the observed histologic variable .
conversely , spiro et al . , have not found histologic classification to be of any benefit , and deny a correlation between microscopic appearance and prognosis . due to the slow growth pattern of the tumor
however , due to local recurrence and late metastasis , surgery remains the mainstay of management with or without radiotherapy . the use of synthetic material such as prolene mesh in prolapsus surgery has become a popular approach .
it has been proven to be very effective and became a standard of care in the treatment of most cases with severe prolapsus .
sacropexy is a surgical repair technique that restores pelvic anatomy by attaching synthetic graft material into the vagina and sacrum .
erosion of vaginal wall or bowel can be seen as a long term complication . in this paper
, we present a patient suffering from mesh migration into the rectum after abdominal sacral colpopexy .
a 69-year - old woman was admitted to the hospital with a complaint of sensation of fullness and a feeling of a foreign material protruding during defecation .
she had been diagnosed with uterine prolapsus and stress incontinence in 2008 and underwent total abdominal hysterectomy , bilateral salpingo - oophorectomy and sacral colpopexy with prolene mesh .
the patient was admitted to our clinic five years after the procedure with complaints mentioned above . there was a foreign material palpated in rectum with digital examination .
prolene mesh was detected in sacral region but resection of the mesh could not be conducted because of high levels of adhesions in that region .
genital prolapse or genital hernia is described as the protrusion of pelvic organs along the vagina .
it is one of the common gynecological conditions that affect the quality of life in women .
it may be seen in up to 50% of multipara women , and its incidence increases with age .
high rates of recurrence with traditional techniques led to the development of new surgical techniques .
the use of synthetic mesh has become more popular surgical approach in cystocele and rectocele repair .
mesh migration is a well - known clinical pathology and have been reported in literature .
yolen and grossman suggested that intra - abdominal foreign bodies ( like mesh ) transmigrate into the small or large bowel by triggering an inflammatory reaction .
persistent inflammatory reaction causes an opening into a hollow organ assisted by the peristaltic movement of the bowel .
insufficient fixation of a mesh is another factor for migration of synthetic materials in some patients .
larger pores greater than 75 nm permit the migration of macrophage and leukocyte migration and reduce the infection rate .
large pores also improves flexibility of the mesh and cause tissue ingrowths and healthy collagen deposition .
complications reported after sacropexy include ileus , intraoperative vessel injury , ureter injuries , recurrent descensus and mesh tearing . the use of a mesh as a graft material results in higher success rates but also causes a higher number of complications , such as mesh erosions or chronic infections .
taoka reported a case of rectal migration of mesh in a 64-year - old woman who presented with a recto - cutaneous fistula 11 months after a tension - free vaginal ( tvm ) repair ; the patient was treated by removal of the infected mesh and closure of the rectal wall defect under cover of a temporary colostomy . by contrast with
the troublesome symptoms reported in such patients in the literature , the only presenting complaint of our patient was protrusion of foreign material from the rectum .
in conclusion , mesh migration is a serious complication after sacral colpopexy . sometimes surgical resection of migrated mesh with laparotomy can be difficult due to dense adhesions .
scu was responsible for writing , conception and design of the study ; ob contributed toward analysis and interpretation of data ; nas , oa , aa- performed acquisition of data ; bk drafted the manuscript . | adenoid cystic carcinoma is a relatively rare epithelial tumor of the salivary glands accounting for about 5 - 10% of all salivary gland neoplasms .
approximately , 31% of salivary gland neoplasms affect minor salivary glands particularly the palate .
it involves tongue in only 19.8% of cases and even rarely the dorsum of the tongue .
we report such a rare case that affected dorsum of the tongue in a 45-year - old - female patient . |
congenital seminal vesicle cysts associated with abnormalities of the upper urinary tract are uncommon.1 they can be asymptomatic and discovered incidentally or can be associated with dysuria , urinary tract infections , and infertility .
diagnosis is more frequently made in adult life during the period of the greatest sexual or reproductive activity.1
2 we present a case of a congenital seminal vesicle cyst associated with ipsilateral renal agenesis incidentally discovered on ultrasound at the age of 4 years and its natural evolution until adolescence .
the association of congenital seminal vesicle cyst with ipsilateral renal malformations is rare and was first described by zinner in 1914 .
it is reported in literature as zinner syndrome.3 this condition is considered as the male equivalent of mayer - rokitansky - kustner - hauser ( mrkh ) syndrome described in females.4
a 4-year - old boy with known right renal agenesis discovered on antenatal ultrasound , presented in our radiology department for his annual control .
the cyst was anechoic , measured 8 mm , and was not present on previous ultrasounds .
it was initially considered as an ureterocele or bladder diverticula , however , neither of these diagnosis was confirmed on intravenous urography performed at that time .
the cyst remained unchanged on size and aspect during 11 years of follow - up .
however , at the age of 15 years its aspect on the ultrasound had changed : its content was hyperechoic , it had increased in size ( measuring 1.9 cm ) , and protruded in the bladder .
it was associated to magma of some echogenic , round retrovesical masses , initially considered as lymph nodes ( fig .
1b , c ) . magnetic resonance imaging ( mri ) of the urinary tract and pelvis was further performed ( coronal , axial , and sagittal t2 spin - echo and axial t1 with fat saturation before and after gadolinium injection images ) .
it revealed a dilatation of the seminal vesicles , ipsilateral to renal agenesis ( corresponding to the magma of retrovesical round masses seen on ultrasound ) , ending to a relatively small seminal vesicle cyst ( diameter of 2 cm ) that protruded in the bladder ( fig .
2 a c ) .
ultrasound ( a ) at the age of 4 years detects a right retrovesical anechoic cyst ( + ) ( b ) at the age of 15 years the cyst has increased in size and it has become hyperechoic ( white arrow ) .
( c ) it is associated to magma of round retrovesical masses ( black arrow ) .
( b ) t2 spin - echo axial and ( c ) sagittal images of the pelvis show dilatation of the right seminal vesicles ( white arrow ) ending to a seminal vesicle cyst ( * ) that protrudes in the bladder .
so far , the patient was asymptomatic and had never presented with dysuria , signs of bladder obstruction or urinary tract infection .
he had neither any perineal discomfort nor pain , he described normal ejaculation without pain , no hematospermia , and had never experimented any urinary infection or prostatitis and also he was not sexually active . in this particular case , in the absence of symptoms and because of the relatively small size of the cyst a conservative treatment was decided , with an annual clinical and ultrasonographic control , at least until the first symptoms appear .
the association of congenital seminal vesicle cyst with ipsilateral renal malformations is rare and there are approximately 200 cases reported in the literature.4
this association is explained by the common embryologic origin of both the organs ( kidneys seminal vesicles ) from the mesonephric duct1 and is due to an insult in embryogenesis between the 4th and 13th week of gestation.4 the ureteral bud arises from the dorsal part of the distal mesonephric duct and extends dorsocranially to meet and induce differentiation of the metanephric blastema , from which the kidney will develop .
the mesonephric duct will differentiate to epididymis , ejaculatory duct , vas deferens , seminal vesicle , and hemitrigone .
complete failure of the mesonephric duct results to absence of ipsilateral kidney , ureter , hemitrigone , and seminal vesicle .
anomalous development of the distal mesonephric duct results to atresia of the ejaculatory ducts and abnormal ureteral budding ; the former leads to obstruction and cystic dilatation of the seminal vesicles with development of seminal cysts , the latter leads to renal agenesis or dysplasia.2
5 zinner syndrome could be considered as the male equivalent of mrkh syndrome described in females.4
the cysts are present since birth but enlarge and become symptomatic on late adolescence or adult life , usually in the third to fifth decade , at the period of greatest reproductive or sexual activity.1
2 at this moment the accumulation of secretions in the seminal vesicle due to atresia of the ejaculatory ducts and consequently insufficient drainage leads to formation of cysts in the seminal vesicle.2
cysts smaller than 5 cm are usually asymptomatic and are discovered incidentally .
larger cysts can irritate the bladder and be related to pain , dysuria , frequency , hematuria , urinary tract infection , epididymitis or prostatitis , infertility , hemospermia , bladder outlet , or colonic obstruction.1
2
6 malignant degeneration of the cysts has been also reported.2
4
this progressive dilatation of seminal vesicle remains often asymptomatic until the dilatation increases to 4 to 5 cm,4
5 unless the obstruction triggers vas deferens dilatation at younger age,5 which was not the case for our adolescent .
this dilatation develops slowly , by accumulation of secretions in the seminal vesicle , for many years after puberty , due to congenital atresia of the ejaculatory ducts and consequently insufficient drainage2 : literature reports a majority of cases becoming symptomatic around the third or fourth decade of life .
diagnosis can also be made earlier in front of infertility linked to contralateral distal ejaculatory pathway compression by this cystic seminal vesicle.3
in our case , the cyst was first seen at the age of 4 years , mimicking an ureterocele on ultrasound . in adolescence
it increased in size , but remained small and , for this reason , asymptomatic .
diagnosis was made on mri , based on its anatomic connection and to the similar signal intensity with the seminal vesicles .
differential diagnosis includes other pelvic cystic masses such as utricular or mullerian cyst , ureterocele , dilated ureter , abscess or lymph nodes,2
4 and acquired seminal vesicle cysts .
acquired cysts are usually bilateral and concern older patients with chronic prostatitis or postprostate surgery .
ultrasound raises the suspicion of this malformation but the final diagnosis is usually made by mri ( or computed tomography , with this last method being irradiating ) . in adults
more invasive methods as cystoscopy and vasovesiculography are also used for diagnosis.2
there is a medical consensus to propose conservative follow - up for asymptomatic or minimally symptomatic cysts.1
4
7 treatment is considered only for symptomatic patients and is surgical .
different surgical options exist , from the less invasive transrectal or transperineal aspiration of the cyst which gives a transitory relief of the symptoms,1 to more aggressive technics .
transurethral unroofing of the cyst by transurethral resection of the ejaculatory duct provides improvement of the quality of semen and improves the paternity rate,3 but this procedure can create injury to rectum , bladder neck , and external sphincter , and induce consequent retrograde ejaculation , and epididymitis . radical treatment , vesiculectomy with resection of ureteral or renal remnants if present , offers the better outcome .
it was traditionally performed by open exploration but is now well described laparoscopically , with low morbidity and good results in term of symptoms
relief and semen parameters.1
4
7
8
9
10
11 few children with surgical indication benefited from this approach.12
13
concerning fertility , for patients presenting with difficulty to procreate , with low volume of semen and poor quality of spermogram , treatment shows semen quality improvement , and paternity obtained without other medical help.3
4 assisted reproductive procedures should be kept for patients with infertility persisting after successful surgical procedure.4
our case is original as it shows the natural history and evolution of this malformation , from childhood to adolescence .
cases reported in literature concern , in their high majority the adult population ( or late adolescence ) and these cysts are rarely detected at a younger age .
congenital seminal vesicle cysts in patients with ipsilateral renal agenesis are rare but this association is known and should be considered in the differential diagnosis of cystic pelvic masses in males with renal agenesis or dysplasia .
ultrasound is useful for diagnosis but mri provides a more detailed analysis and accurate diagnosis .
congenital seminal vesicle cysts in patients with ipsilateral renal agenesis are rare but this association is well known .
pediatric surgeons should be aware and consider this entity in the differential diagnosis of cystic pelvic masses in males with renal agenesis or dysplasia .
ultrasound is useful for diagnosis but magnetic resonance imaging provides a more detailed analysis and accurate diagnosis . tori and exostoses are nodular protuberances of calcified bone and are designated according to their anatomic location.1 torus palatinus ( tp ) and torus mandibularis ( tm ) are the two most common types of intraoral osseous overgrowths.2,3 tp is a sessile , nodular bony mass commonly seen on the midline of the hard palate .
tm is bony protuberance found on the lingual aspect of the mandible , in the canine and premolar region .
buccal and palatal exostoses are multiple bony nodular masses found less frequently than tori.1,2,4 buccal exostoses occur as bilateral , smooth bony growth along the facial aspect of the maxillary and/or mandibular alveolus . commonly found to appear in the premolar - molar region . on palpation ,
ulcerations may be seen as a result of trauma or any injury to the mucosa .
their size may increase to several centimeters thus contributing to periodontal disease of adjoining teeth by retaining food during chewing instead of flushing away .
usually no treatment is required , but for those possibly affecting the periodontal condition , or when the protruberances cause pain or discomfort to the patient , or when these bony enlargements cause pseudo swelling over the lip , then conservative surgical excision can be performed .
some of the suggested causes include genetic factors , environmental factors , masticatory hyperfunction and continued jaw bone growth.5,6 the histologic features of tori and exostoses are identical.2 a very small exostosis and tori consist entirely of compact bone but when large and nodular , it consists of cancellous bone surrounded by cortical bone .
the diagnosis of a buccal exostosis is based on the clinical examination along with radiographic interpretations .
clinically , the torus may appear as numerous rounded protruberanes or calcified multiple lobules , whereas the exostosis is a single , smooth broad - based mass , may have a sharp , pointed bony projection producing tenderness just beneath the mucosa.8 lesions may slowly enlarge up to 3 - 4 cm in greatest diameter , but it does not have malignant transformation potential .
buccal exostoses are usually found only on the facial surface of the maxillary alveolar bone , especially in the posterior segment .
radiographically , exostosis appears as well - defined round or oval calcified structure superimposing the roots of teeth .
the patients are having multiple bony growths or lesions which are not in the classic torus or buccal exostosis locations should be evaluated for gardner syndrome .
this autosomal dominant syndrome shows other features such as intestinal polyposis and cutaneous cysts or fibromas.2,5 no bony exostosis or tori requires treatment unless it becomes large enough to interfere with periodontal health , denture placement , or cause recurrent traumatic ulcerations . when treatment is elected , the lesions should be cut - off or removed from the cortex using bone cutting bur or hand instruments .
the diagnosis of a buccal exostosis is based on the clinical examination along with radiographic interpretations . clinically , the torus may appear as numerous rounded protruberanes or calcified multiple lobules , whereas the exostosis is a single , smooth broad - based mass , may have a sharp , pointed bony projection producing tenderness just beneath the mucosa.8 lesions may slowly enlarge up to 3 - 4 cm in greatest diameter , but it does not have malignant transformation potential .
buccal exostoses are usually found only on the facial surface of the maxillary alveolar bone , especially in the posterior segment .
radiographically , exostosis appears as well - defined round or oval calcified structure superimposing the roots of teeth .
the patients are having multiple bony growths or lesions which are not in the classic torus or buccal exostosis locations should be evaluated for gardner syndrome .
this autosomal dominant syndrome shows other features such as intestinal polyposis and cutaneous cysts or fibromas.2,5 no bony exostosis or tori requires treatment unless it becomes large enough to interfere with periodontal health , denture placement , or cause recurrent traumatic ulcerations . when treatment is elected , the lesions should be cut - off or removed from the cortex using bone cutting bur or hand instruments .
a 20-year - old female patient reported to the department of periodontology , maharana pratap college of dentistry and research centre , gwalior ( madhya pradesh ) with bilateral masses just above the premolar and molar region in maxilla interfering in her smile and aesthetics ( figure 1 ) .
she had noticed slow , but steady enlargement of the masses over the past 5 years .
physical examination of the oral cavity revealed large , bilateral overgrowths located on the buccal aspect of the maxilla in the premolar and molar areas ( figure 2 ) .
the overlying mucosa was thin and blanched , and generalized moderate gingivitis with minimal bone loss was present .
the exostoses were oblong in shape , measuring approximately 1.7 cm 1 cm on the right side and 2 cm 1 cm on the left side .
radiographic examination showed a well - defined radiopaque area covering the roots of the premolar and molar teeth .
these bony protruberance caused by the thickening or enlargement of the cortical plate of the facial surface of the maxilla without any systemic abnormality helped to reach to diagnosis that it was multiple buccal exostoses . bilateral enlargement seen in premolar and molar region .
generally , no treatment for buccal exostoses is required but as a patient was not happy with the bony masses seen during speech and smile .
the full thickness flap was raised to expose the exostoses adequately ( figures 3 and 4 ) .
the bony growth was cut with bone cutting carbide bur , no 702 ss white bur under continuous saline irrigation ( figure 5 ) .
smoothening of the rough surface was carried out with bone file and granulation tissue were curetted .
the surgical site was washed thoroughly with a solution of povidine iodine and saline in 1:1 proportions and flap was closed .
a follow - up appointment was scheduled after 10 days of surgery to check the site and for suture removal .
the tissue appeared healed , and the patient was totally asymptomatic after 10 days ( figure 6 ) .
flap reflection revealing the bony mass of 2 cm 1 cm on left side flap reflection revealing bony mass of 1.7 cm 1 cm on right side .
the multiple masses in the maxilla are consistent with multiple buccal exostoses , which are bony protuberances that arise from the cortical plates in the maxilla and mandible .
they usually occur in the late teens and early adult years , and many continue to enlarge slowly over time .
the etiology of the multiple exostoses remains unknown , although it has been suggested to be the outcome of a mild , chronic periosteal inflammation .
furthermore , the patients with multiple bony growths , not in the classic buccal exostoses locations should be evaluated for gardner s syndrome .
intestinal polyposis and cutaneous cysts or fibromas are other common features of the autosomal dominant gardner s syndrome.2,5 neither the torus nor the bony exostosis require treatment unless it becomes large enough to interfere with function , denture placement , cause recurring traumatic surface ulceration ( usually from sharp food such as potato chips or fish bones ) or as used to get autograft as it is a potent donor site.7 when treatment is elected , the bony mass may be removed using bone cutting bur or chiseled off through the base of the lesion .
the case report presented above illustrates a unique and rare presentation of exostoses on the buccal side of the maxillary premolar - molar region , bilaterally .
exostosis is rarely found on the facial surface of maxilla thus should not be ignored and should be carefully differentially diagnosed . | zinner syndrome , the association of congenital seminal vesicle cyst and ipsilateral renal agenesis , is more often reported in adults or older adolescents .
we present a case of a boy , followed up in our hospital since birth for right renal agenesis who at the age of 4 years presented a right paravesical cyst on ultrasound .
the cyst was initially considered as an ureterocele .
the diagnosis of zinner syndrome was made later , at the age of 15 years by ultrasound and magnetic resonance imaging ; at that moment the cyst had increased in size and had changed in aspect .
this malformation should be considered in the differential diagnosis of a pelvic cyst in male patients with renal agenesis . |
hemangiomas are categorized as racemose , capillary , cavernous , and venous , according to the size of their vascular spaces .
cavernous and venous hemangiomas have low blood flow , because they lack arterial or capillary components .
histopathologically , venous hemangiomas contain dilated vessels with thick , fibrous walls , whereas cavernous hemangiomas have capillary - sized vessels lined by flat endothelial cells .
we report the first case of a venous hemangioma of the pps and discuss its typical radiographic findings .
a 49-year - old female patient was referred for evaluation of swelling in the right submandibular region .
she had swelling for the first time 3 weeks earlier , and it had not improved after medical treatment .
clinical examination revealed a soft mass in the right submandibular region and a bulge at the right lateral pharyngeal wall . a contrast - enhanced computed tomography ( ct ) scan revealed a cystic lesion , 4.63.0 cm , with contrast non - enhancement in the parapharyngeal space ( pps ) .
mri showed a well - circumscribed non - enhancing mass with high signal intensity on t2-weighted images ( fig .
the cystic lesion of the pps was surgically removed using a transcervical approach . during surgery
the lesion bled profusely , but the mass was bluntly dissected from the surrounding structures .
the surgical specimen , measuring 4.34.52 cm , showed a pale - to - dark brown soft cut surface with a blood - filled spongy vascular lesion .
microscopic examination revealed blood - filled sinusoidal spaces with large irregular lumens and thick walls lined by endothelial cells ( fig .
postoperative recovery was uneventful , and there has been no evidence of cranial nerve palsy or tumor recurrence after one year .
pps has a complex anatomy and close proximity to vital anatomical structures , with which it may become involved by various pathological processes . presenting symptoms of pps tumors may be attributed to the size of the mass and compression of neighboring structures ( 1 ) .
we had difficulty with the preoperative diagnosis , due to the absence of typical symptoms or signs of a vascular lesion in the pps , such as pulsation or bruit .
fine - needle aspiration cytology ( fnac ) was also not helpful in reaching a diagnosis .
although an exact tissue characterization of hemangioma in the pps could not be made on fnac , contrast - enhanced ct and magnetic resonance imaging ( mri ) should be included in differentiating these lesions from other tumors of the pps . in general ,
mr imaging is superior to ct imaging in its ability to ascertain the soft tissue characteristics of pps tumors ( 2 ) .
changes in the blood flow dynamics within a hemangioma result in thrombus formation and phleboliths ( 3 ) .
phleboliths are calcified nodules that can be regarded as a characteristic property of venous or cavernous hemangiomas .
ct with contrast is an excellent imaging technique for revealing phleboliths . although mri is not sensitive for the detection of small amounts of calcification , large amounts of calcification show discrete low signal intensity on all pulse sequences
mri can produce high signal intensities , representing the blood , as well as focal heterogeneities , representing areas of thrombosis , fibrosis , or calcification ( 4 ) .
although mri is very useful for the detection of vascular lesions , the detectability of phleboliths on ct images is superior to that by mri .
some reports have shown that plain x - ray films may also reveal calcified lesions .
when imaging shows calcification in the pps , the differential diagnosis may include pleomorphic adenoma of the deep lobe of the parotid gland and metastatic thyroid carcinoma of the pps .
pleomorphic adenoma with foci of chondroid or osteoid stroma can demonstrate opacities on imaging , but usually shows minute , scattered flecks .
additionally , in cases of metastatic thyroid carcinoma of the pps , large flocculent calcification is seen on ct images ( 5 ) .
venous hemangioma can not be clinically or radiologically differentiated from cavernous hemangioma , because of their similar characteristics .
however , perfusion and blood pool scintigraphy has been demonstrated to have high sensitivity for detecting head and neck hemangioma , and can also differentiate between cavernous and venous hemangiomas ( 6 ) .
they tend to be larger and less well circumscribed , and show no tendency to regress ( 7 ) .
attention needs to be given to the tumor location , extent , growth rate , and accessibility as well as the patient 's age and esthetic concerns .
the excision of large tumors in the pps can be challenging , given the risk for severe hemorrhage and nerve injury .
a surgical approach should be chosen according to the tumor size and location , its relationship to the great vessels , and any suspicion of malignancy .
generally , hemangiomas in the pps , including venous hemangioma , can be resected by using a transcervical approach .
profuse bleeding often occurs during surgery , but after all of the pathology has been removed , the bleeding will cease . when surgery is impossible , the use of corticosteroids , cryotherapy ,
feeding vessel ligation , embolization , and fibrosing agents can be considered as alternatives ( 8) . in summary , we present the first reported case of venous hemangioma occurring in the parapharyngeal space . considering that a presumptive diagnosis of most pps tumors can be made based on imaging studies , preoperative imaging findings are very important in approaching these lesions .
it may be that multiple , spotty , calcific nodules , 13 mm in size , within a cystic lesion on ct images indicate the pathognomonic finding of hemangioma in the pps . humans are infected naturally by 3 species of hookworms ; necator americanus , ancylostoma duodenale , and ancylostoma ceylanicum .
individual people can be infected with 1 species , 2 species , or all 3 species of hookworms [ 1 - 5 ] . although a. ceylanicum is often considered to be a zoonotic pathogen , since it is also found in dogs and cats , its importance as a significant soil - transmitted helminth of humans is increasingly being recognized .
two haplotypes of a. ceylanicum have been identified ; one largely found in humans and another that infects humans , dogs , and cats .
a. ceylanicum is considered to be of emerging importance as a helminth of humans in the asia pacific region .
a fourth hookworm also reported from humans , ancylostoma caninum represents a canine species with zoonotic potential .
this species has mainly been recorded as causing eosinophilic enteritis in people in tropical australia , but never establishes a patent infection and hence humans are regarded as accidental hosts .
human hookworm is common in the solomon islands , with an estimated 192,000 people ( 36% of the population ) infected , but the different species involved are yet to be determined [ 9 - 11 ] .
the report to the rockefeller hookworm campaign in 1928 described only n. americanus as being present on these islands .
the only previous report of a. ceylanicum from the solomon islands was a 5-year - old boy who imported the species in 1938 from the shortland island group of the solomon islands to australia .
the solomon islands is a small pacific nation to the north east of australia , with a predominantly melanesian population .
the regional assistance mission to solomon islands ( ramsi ) occurred between 2003 and 2013 , at the request of the solomon island s government , to assist in stabilizing internal troubles occurring within the nation .
ramsi consisted of 2,200 police and soldiers from many pacific island nations and was led by australia .
this case report describes the finding of a. ceylanicum in an australian soldier who developed symptomatic disease after returning from a deployment in solomon islands with ramsi .
the patient was a 26-year - old male living in townsville , north queensland , australia who presented in march 2004 with central poorly defined abdominal pain of 4 weeks duration and increased frequency of defecation .
a peripheral eosinophilia had been noted on blood tests in february prior to symptom development , and in march , when eosinophils were 6.2010/l whilst red blood cell indices were normal ( hemoglobin 146 g / l , mcv 93 fl , hematocrit 0.44 , red cell count 4.710/l ) .
the patient had served with ramsi in the solomon islands from late july to early december 2003 .
his duties involved travel to various remote areas and provided multiple opportunities to become infected with hookworms .
routine treatment with albendazole had been given by the australian defense force on return to townsville in december , 2 months prior to development of symptoms .
initially , in march 2004 no parasite eggs were detected in feces by formalin - ethyl acetate concentration , but infective larvae of hookworms were found on harada - mori culture .
infective larvae were sheathed , had inconspicuous buccal spears , the intestine was narrower than the esophageal bulb , and inconspicuous transverse striations were noted on the sheath in the tail region .
the infective larvae , measured after preservation in hot formalin , had the following dimensions , meansd ( range ) : length 657.19.6 m ( 633 - 669 m ) , width 21.40.9 m ( 20.2 - 22.2 m ) , esophagus 156.73.5 m ( 151 - 162 m ) , tail 77.93.8 m ( 69 - 81 m ) , length of sheath 763.78.6 m ( 779 - 746 m ) ; esophagus / length 23.80.5% , and tail / length 11.90.6% . due to the failure of single dose post - deployment albendazole therapy to eradicate this infection , the patient was treated with 100 mg of mebendazole twice daily for 3 days .
twelve adult hookworms ( 6 females and 6 males ) were recovered by dissection of stools passed during the 48 hr immediately following treatment . the en face view revealed a robust ventral cutting plate bilaterally with a prominent point on the dorsal end and less obvious point on the ventral end ( fig .
the width of the bursa in lateral view was greater than its length , and the mediolateral and posteriolateral bursal rays were parallel ( fig .
the morphology of the adult hookworms was consistent with a. ceylanicum with key features being : the large ventral cutting plates terminating at the dorsal end in a large single tooth ; and in the male the parallel mediolateral and posteriolateral bursal rays .
although the morphology and dimensions of the infective larvae are more consistent with a. ceylanicum than n. americanus , differentiating them from infective larvae of a. duodenale is unreliable .
although molecular taxonomy is valuable in identifying hookworm species , it was not available in this case and , as the specimen was subsequently accidentally disposed of , molecular taxonomic studies can not be performed now . however , the morphological features were sufficient to identify this species as a. ceylanicum .
this represents only the second report of human a. ceylanicum infection having been acquired in this nation , with the first being made almost 80 years ago .
this finding not only expands the known range of human a. ceylanicum infection but also raises the question of whether a. ceylanicum is in fact a common cause of human hookworm infection in some regions of the solomon islands , as it has recently been described as being in many parts of asia . the only previous reported description of hookworm species in the country was almost 80 years ago and was anecdotal , not providing descriptions of number of people tested or how the species identifications were arrived at .
a. ceylanicum infection has recently been reported from two people in western australia . prior to this , a. ceylanicum had been reported from dogs and cats in northern australia , including those from townsville .
hence , the infection could have been acquired in north queensland or while deployed with ramsi .
the likelihood of infection with ancylostoma from humans in the solomon islands is high , but negligible in north queensland since human feces are disposed of safely .
however , since a. ceylanicum is present in dogs and cats in north queensland , there is a possibility of a zoonotic australian source even though this infection has not been seen in humans in this region .
many ramsi personnel were infected with hookworms during their mission to the solomon islands , and an isolated case of a. ceylanicum infection had been reported from this area early in the 20th century .
this adds to the likelihood that the case presented here was acquired in the solomon islands and not elsewhere .
the clinical symptoms displayed by our patient were consistent with the phase of hookworm infection during which the host attempts to eliminate the parasite using an allergic response , manifesting morphologically as eosinophilic enteritis . for n. americanus
this response begins about 10 days after infection and persists for up to a month .
a. caninum and a. duodenale have a more complicated life cycle in which some infective larvae can enter a state of hypobiosis ( arrested development ) and can orchestrate entry to the intestine to coincide with the wet season and physiological events [ 23 - 26 ] . whether a. ceylanicum has
the failure of a routine post - deployment albendazole treatment to eliminate the infection in our patient supports a hypothesis that the current infection was due to hypobiotic larvae activated after the anthelmintic was given .
acute enteritis has been described previously in soldiers with a. ceylanicum acquired in west papua ( formerly dutch new guinea ) and in a traveller returning from myanmar to france .
treatment with albendazole in the latter patient cleared the gut infection but symptoms and parasites recurred after 3 months adding evidence for hypobiosis in a. ceylanicum .
our patient was treated after diagnosis with mebendazole due to its far greater tissue penetration and therefore greater capacity to kill hypobiotic larvae in the tissue .
the key points from this case report are : a. ceylanicum should be considered as a cause of acute eosinophilic enteritis in returned travellers , workers , or defense force personnel who have lived or visited tropical and subtropical countries ; laboratory confirmation may require persistence to make the diagnosis of hookworms and will require special tests ( morphology of adults after treatment ; molecular taxonomy on stools or infective larvae ) to identify the species ; and human infection with a. ceylanicum is probably endemic in the solomon islands and further study of the prevalence of different hookworm species present in this country is warranted . | a hemangioma of the parapharyngeal space ( pps ) is an extremely rare tumor and is responsible for 0.5 - 1% of all tumors occurring in the pps .
we report a case of pps venous hemangioma in a 49-year - old woman presenting with diffuse swelling in the submandibular region . a preoperative computed tomography ( ct ) scan showed a cystic mass with multiple calcifications in the pps .
the calcific nodules were round and about 2 mm in diameter .
the hemangioma was completely resected via a transcervical approach . during surgery
, we found several calcific nodules , which represented phleoboliths or areas of thrombosis with dystrophic calcification . despite its rarity ,
a venous hemangioma of the pps should be considered in a differential diagnosis when a cystic mass with calcification is found by ct scan . to our knowledge , this is the first reported case of a pps venous hemangioma ; we describe its pathognomonic findings on imaging . |
verruciform xanthoma ( vx ) , which has almost similar histologic features , is also a rare lesion usually found on the oral mucosa or the genital area .
it is presumably associated with the inflammatory response to mucosal damage.1 however , xanthoma and vx of the esophagus are extremely rare . since the first report by remmele and engelsing2 only 13 cases of esophageal xanthoma have been reported,1,2,3,4,5,6,7,8,9 and since the report by herrera - goepfert et al.,10 only four cases of vx of the esophagus have been reported.10,11,12,13 the etiologies of both lesions are not understood .
we describe herein a new case , including a review of all reported cases of xanthoma and vx of the esophagus .
a 70-year - old man with an unremarkable medical history was hospitalized with a complaint of epigastric pain .
serum total cholesterol , triglyceride , high density lipoprotein cholesterol , and low density lipoprotein cholesterol levels were 151 , 215 , 33 , and 102 mg / dl , respectively .
multiple shallow gastric ulcers and a duodenal ulcer were detected and suspected to be the cause of the pain .
besides the ulcers , in the upper esophagus 20 cm from the incisors , a 3-mm yellowish granular elevated mucosal lesion was found and a biopsy was performed ( fig .
1 ) . microscopically , large round cells were aggregated in the lamina propria immediately beneath the squamous epithelium .
the etiologies are different , as xanthoma is caused by hyperlipidemia and vx arises presumably as a result of an inflammatory response to continuous mucosal damage.1 however , the etiologies of the two lesions arising in the esophagus are not understood .
the characteristics of all reported cases of xanthoma and vx of the esophagus are summarized in table 1 .
fourteen cases of xanthoma and four cases of vx of the esophagus have been reported .
however , some reports loosely stratified vx into esophageal xanthoma , whereas others have excluded it.6,8 in terms of clinical data , both diseases were found predominantly in men than in women : 9 versus 3 in xanthoma and 3 versus 1 in vx .
the median age was 59 years ( range , 37 to 74 ) in xanthoma and .
the predominant location was the lower esophagus for xanthoma ( lower , 7 ; middle , 2 ; upper , 3 ) , whereas vx was not reported in the lower esophagus ( upper , 2 ; middle , 2 ) .
the median size was not different : 3 mm ( range , 2 to 10 ) for xanthoma and 4 mm ( range , 3 to 20 ) for vx .
the associated medical conditions were diverse ; however , two patients with malignant tumors were included in each group : hepatocellular carcinoma and ileocecal lymphoma in xanthoma , and gastric cancer and multifocal cancer ( cancer of the glottis , liver , and trachea ) in vx , although there was no definite association .
vx is characterized by its histologic features , including papillomatosis , acanthosis , and hyperparakeratosis.11 also , the external morphology is verrucoid .
nevertheless , findings of large round foam cells in the lamina propria under the squamous epithelium are the same as those in xanthoma .
it is difficult to differentiate between the two lesions on the basis of gross examination when they arise on the esophagus .
exophytic and verrucoid features seen in vx of the skin were not observed in the esophagus because most of the reported cases were small in size.10,12 considering that xanthoma and vx are nonneoplastic lesions , differentiating between them could be a waste of effort .
however , these lesions have to be grossly distinguished from ectopic sebaceous glands and small subepithelial tumors such as carcinoid and granular cell tumor because most of the reported esophageal xanthomas are yellowish or white mucosal elevated lesions . in terms of microscopic findings , signet ring cell carcinoma , which contains round cells with abundant cytoplasm ,
while signet ring cell carcinoma has an eccentrically located nucleus because of the intracellularly abundant mucin , xanthoma has a centrally located and small nucleus .
positive immunohistochemical staining for cd68 , which indicates a histiocytic origin , is another characteristic finding of xanthoma.5 moreover , esophageal cancer and ectopic sebaceous glands do not commonly stain with lugol 's solution ; thus , endoscopists need to be aware of these lesions for the differential diagnosis.6,14 with more case reports of esophageal xanthoma and vx of the esophagus , the characteristics of both lesions will be more clearly elucidated . the sizing of pda for device implantation is based on the automatic calculations of dimension by the inbuilt sizing software in the cardiac catheterisation laboratory and many experienced interventionists believe their eyeball measurements more .
the estimations can be erroneous if the calibrations are not proper , leading to catastrophic embolisation of the device , needing emergency surgical removal as was needed in our patient .
calibration by comparing with the fluoroscopic images of measured metallic sizing devices placed outside body can be misleading because of magnification errors.1 in order to circumvent these problems we suggest some novel ways which take care of magnification errors and avoid the need for aortic root angiography .
an eight year child with echocardiographically documented 6 mm patent ductus arteriosus ( pda ) was planned for device closure .
femoral venous access was obtained using 8f sidearm sheath and the artery cannulated with a 6f sidearm sheath .
a 6f pigtail catheter was advanced through the femoral arterial sheath into the descending aorta at the mouth of the pda and an angiogram obtained in a lateral projection delineating the ductus ( figure 1 ) .
the minimum diameter at the aortic end was 6 mm and a 8 - 10 mm pda device was selected for deployment .
a 7f swan - ganz catheter was advanced into the the pulmonary artery through venous route and a 0.35 " terumo wire advanced through the pda into the descending aorta .the terumo wire was exchanged with an 0.35 " extra stiff amplatzer wire and the swan - ganz catheter with the pda delivery system . an 8 - 10 mm size pda device was released across the ductus and after confirming the proper position by repeat angiogram which showed a small residual shunt flow which may be expected .
angiogram in lateral plane showing a small residual left to right shunt with pda device in place before unscrewing of the stylet after a short while , the device suddenly embolised into the left pulmonary artery ( figure 2 ) .
the patient had a brief episode of mild hypotension which improved spontaneously and his oxygen saturations remained static .
an attempt was made to retrieve the device percutaneously by a snare and later by a bioptome but the trial was given up as the device tended to go more distally , in which case even surgical removal would be difficult .
the patient was discussed with the cardiac surgeon who shifted the patient for emergency surgery .
after considerable debate surgeon decided for a left thoracotomy hoping to avoid cardiopulmonary bypass and closed the ductus . later the left pulmonary artery was separated from the surrounding tissues and umbilical tape passed around it proximally for control of bleeding . a 1.5 cm longitudinal incision was made distal to the umbilical tape which was kept under traction and a long curved forceps passed toward the pulmonary hilum and the device retrieved .
cine image in lateral plane showing device embolized into left pulmonary artery soon after its release from stylet . showing the pda device being retrieved from left pulmonary artery via left thoracotomy without cardiopulmonary bypass . in order to prevent such eventualities we tried to devise some alternative ways of sizing a pda .the
first involves measuring a fully inflated tyshak pulmonary balloon outside the human body with a caliper and then inflating the balloon to the same pressure in the right ventricular outflow tract passed over a 0.35 " extra - stiff wire , and repeating the measurements , and using the same calibration factor for sizing of pda visualized by aortic arch angiogram .
previously , some radio opaque sizing devices have been used placing them outside human body which can have magnification errors if not placed exactly at the level of heart.2 the second method involves advancing a tyshak balloon 2 - 4 mm bigger than the echocardiographic dimension across the pda and inflating the balloon with dilute dye under low pressure without dilating the ductus so that it forms an impression of the ductus which is recorded in a cine mode in multiple planes , and the plane delineating the exact anatomy of the ductus is used for measurements after proper calibrations as mentioned above without the need for aortic arch angiogram ( figures 4 and 5 ) . showing measurement of diameter of inflated tyshak balloon by a caliper after inflating it with fixed quantity of contrast .
angiogram in lateral plane showing an inflated tyshak balloon across the pda making an impression of the ductus .
technological advances have made nonsurgical closure of the pda a simple and a routine percutaneous intervention .
the use of the amplatzer device occluder ( ado ) has further simplified the method and improved the results with minimal complications .
however there are situations where complications are encountered and surgical help is required to ameliorate them .
faella and colleagues reported 15 procedure related complications in 316 patients which included hemolysis , left pulmonary artery artery stenosis , device protrusion into aorta causing coarctation , device misplacement and one death following device embolisation.2 late embolisation of the device to the left pulmonary artery has been reported with impaired left pulmonary perfusion six months after implantation requiring surgical removal .
m vavuranakis et al reported severe hemolysis with jaundice , anemia and hemogloginura on the second day following deployment of smaller sized coil due to improper sizing which needed removal and replacement by an ado after repeat sizing using balloon tipped catheter.3 the complications can be reduced by proper expertise and optimal sizing of the ductus .
sizing of atrial septal defect ( asd ) by inflating a balloon across the asd till a circumferential waist is created and measuring the waist ( stretched balloon diameter ) and also inflating the balloon outside by same amount of dye across sized rings , is a standard method.45 using a similar method for the pda is not routine since angiographic visualization is usually adequate .
however making an impression of the ductus by inflating a balloon across it can give a detailed anatomy about the length and breadth of the ductus in multiple planes without the need for multiple dye injection and may help in the selection of proper sized device . | xanthoma is an uncommon nonneoplastic lesion resulting from the accumulation of histiocytes .
it predominantly shows cutaneous manifestations associated with dyslipidemia . however , xanthoma of the esophagus is extremely rare . to the best of our knowledge ,
only 14 cases have been reported thus far .
the clinical significance of this lesion has not been established .
however , this lesion should be distinguished grossly from ectopic sebaceous glands and small subepithelial tumors such as carcinoid and granular cell tumor . moreover , signet ring cell carcinoma , which contains round cells with abundant cytoplasm and has similar histologic features to xanthoma , should be distinguished microscopically . |
soft tissue coverage for wounds remains a difficult management problem for patients sustaining traumatic injury and burns .
there are several methods to achieve wound coverage secondary healing , primary suturing , skin grafting , and flap surgeries as described in reconstruction ladder .
a skin graft is the most commonly used modality for coverage of wounds in reconstructive plastic surgery . the skin graft needs to undergo various stages of healing for a good take on the recipient bed .
there are different methods employed to secure the graft to recipient bed for a few days with a basic idea to ensure that the graft is not elevated off the bed by formation of haematoma / seroma under it .
repeated tie over dressings are required in situations where the dressing needs to be changed more frequently as in cases of infected raw area , bleeding tendency , patients on anticoagulant drugs , and in convex areas of body such as buttocks , breast , and the scalp , where the dressing is difficult to secure .
in this novel method , a sterile sample container was cut at its upper part [ figures 1 and 2 ] .
the skin graft was applied on the raw area and fixed with skin staplers and tie over sutures . once paraffin gauze and adequate padding is applied on the raw area , the tie over threads were passed from inside out of the container [ figures 3 and 4 ] and pulled at the appropriate tension to keep the dressing in place .
the lid of the container was tightened to complete the dressing ensuring that the graft was maintained in close approximation with the wound surface .
sterile plastic container upper part of the sterile container is cut tie over threads being passed from inside out the lid is tightened over the dressing the dressing can be changed repeatedly with sterile precautions depending on the requirement , as an outpatient procedure by unscrewing the lid [ figures 5 - 8 ] which can then be easily reapplied .
the procedure can be used on wounds of any size by changing the size of the sterile container chosen .
post - toilet mastectomy raw area covered with the skin graft the tie over dressing applied in ot tie over dressing repeated in an outpatient department we have used this method in eight cases of the post toilet mastectomy raw areas with very good results [ figures 9 and 10 ] . post - toilet mastectomy raw area
skin graft once applied has to be covered with petrolatum gauze to avoid its separation from the wound bed at the time of change of dressing .
an ideal method of graft fixation should be simple , rapid , repeatable , able to be performed in the outpatient department , prevent hematoma or seroma formation , soak the exudates well , and allow the graft bed to be inspected easily .
there are multiple methods of securing dressings over the skin graft , some of them can be applied only once and some can be repeated .
the dressings that can be applied only once are like foam , hydro cellular dressing ( highly absorbent and can be easily changed ) , negative pressure therapy dressing ( stabilises the graft , increases the vascularity of bed , takes away toxic chemicals ) , and gas bag ( transparent , can see graft and monitor any haematoma ) .
these traditional methods can stabilize the graft till the first dressing post operatively . in some contaminated wounds ,
the dressing needs to be removed earlier , especially if there is drainage or foul smell .
this approach may also be proper for graft , used to cover defects of some anatomical regions with increases risk of contamination , such as perineal , axillary , and genital or it can be used in areas where base of wound is difficult to immobilize like breast / pectoral region .
repeated tie over dressings can be done by keeping interrupted sutures long to be used as tie over dressings .
these ties over dressing can be made of sutures or rubber bands . when taking a tie over stitch , both
the threads can be left long and only one thread is tied at a time , the other thread is left long for next time .
these techniques are difficult for small dressings especially the bra hooks ; the silk loops method is very cumbersome and takes long time to do .
the novel method being discussed has a very small learning curve and is very fast .
it hardly takes 5 min in the hands of a plastic surgeon to complete the dressing .
this dressing technique maintains the advantage of conventional tie over dressing with rapidity and repeativity .
good graft take can be expected whether split - thickness or full - thickness with appropriate methods of stabilisation .
we recommend a novel , low cost , simple , rapid method of graft fixation that can be used repeatedly and can be applied to a wound of any size .
we tested a structured relaxation program on nine patients with a diagnosis of schizophrenia suffering from akathisia .
all patients were rated on barnes akathisia scale ( bas ) before the relaxation program , immediately after and again one week later .
the mean bas score was before the relaxation 3.3 which reduced to 1.4 immediately after to finally 1.0 a week later .
a wilcoxon signed ranks test revealed a significant reduction in bas score from baseline to endpoint ( p = 0.026 ; z = 2.232 ) and a highly significant reduction from baseline to follow - up ( p = 0.008 ; z = 2.636 ) .
although the study has a number of limitations the relaxation program appears to be a promising alternative to traditional treatment of akathisia .
the patients appreciated the relaxation session but none of them managed to carry it out on their own without professional encouragement .
akathisia ( inability to sit still ) is a movement disorder characterized by objective movements and restlessness and/or distress , which is common among patients under - going treatment with psychotropic drugs .
it is generally agreed that the syndrome of akathisia comprises both an objective and a subjective component.1 although one of the main motivating factors for the development of second generation antipsychotics was to reduce the incidence and severity of treatment - emergent akathisia and other extrapyramidal side effects , these remain problematic and not uncommon:2 furthermore , akathisia can also be associated with antidepressant treatment.3 although often extremely upsetting , the widely held view of an association between akathisia and suicidal behavior has not been proven in larger methodologically sound studies,4 and is mainly based on case reports .
it is nevertheless a distressing problem that can reduce quality of life and may impede treatment compliance in some patients undergoing pharmacological treatment . in an attempt to make sense of the condition some patients develop a dysfunctional interpretation of the stressful symptoms.5
there is no general agreement on how to diagnose akathisia , and this has hampered both research and clinical practice.6 if akathisia is recognized , current treatment options include reducing the daily dosage or withdrawal of the implicated medication , or the addition of other medications .
the most widely used pharmacological interventions include anticholinergic drugs , beta - blockers , and benzodiazepines , but the evidence base to support these interventions is limited and treatment may entail risks such as development of hypotension , tolerance , and dependence.7 in fact the only small , randomized controlled trial on biperiden showed no difference compared to saline water in the treatment of akathisia.8 some of the most important differential diagnoses of akathisia are the presence of agitation and anxiety symptoms , and these can be helped significantly with structured relaxation programs.9 however to our knowledge a relaxation approach has not been examined in patients experiencing akathisia associated with antipsychotic drugs , therefore the aim of this short report is to investigate whether a relaxation approach would reduce akathisia in patients suffering from this syndrome .
nine patients with a primary diagnosis of schizophrenia and currently experiencing distressing akathisia ( measured on the barnes akathisia scale [ bas ] ) were invited to participate in a structured relaxation program , lasting 12 minutes and consisting of breathing ( four exercises ) and tension - relaxation ( six exercises ) .
the patients were selected specifically because they suffered from akathisia all participants were receiving , or had recently received , pharmacological interventions for akathisia such as benzodiazepines , beta blockers , or procyclidine ( see table 1 ) leading to an unsatisfactorily response
. however , the responsible clinicians had been reluctant to lower the dose of antipsychotic medication due to fears of deterioration of psychotic symptoms .
one patient ( number 9 ) underwent a repeat of the relaxation program with tl during the week before the final follow - up assessment .
all patients were rated ( by lkh ) using the bas,10 before the intervention ( baseline , bas median , 3 ) , after the relaxation session ( endpoint , bas median 2 ) , and again one week later ( follow - up bas median , 2 ) .
the short follow - up period was decided on to minimize the risk of changes to the medication regime that could have made the patients unsuitable for the study .
all patients were given a written version of the program following the sessions , but according to self - reports , none managed to undertake the program on their own in the week before follow - up .
there were no changes to medication regimes in any patient , during the study period .
a wilcoxon signed rank test showed a significant reduction in bas score from baseline to endpoint ( p = 0.026 , z = 2.232 ) and a highly significant reduction from baseline to follow - up ( p = 0.008 ; z = 2.636 ) . no significant difference was however found between endpoint and follow - up ( p = 0.257 ; z = 1.134 ) .
these findings are promising , although it is possible that the benefits were at least partly due to factors such as increased attention and greater contact with other patients and staff .
other limitations in this report include the small sample size , preliminary pilot nature of the data , use of a potentially biased ( lkh ) rater , and the uneven completion of the relaxation program in some patients .
although the number of patients was limited , all but one patient appeared to have benefited from the relaxation program .
the approach was received enthusiastically by the participating patients , but no patient managed to perform the program on their own without professional intervention .
the structured relaxation program appeared to be well accepted and to be associated with a notable reduction in the severity of symptoms of akathisia in this group of patients with chronic schizophrenia , treated with antipsychotic drugs .
the pathophysiology underlying akathisia is not fully understood , and current treatment approaches are less than ideal .
it is important to develop evidence - based alternatives to current interventions that are feasible in routine clinical practice .
the mechanism underlying the reduction in symptoms of akathisia with the structured relaxation program is uncertain but biofeedback methods may be at least partly responsible for the beneficial effect .
biofeedback is a process that enables a person to change physiological activity ( eg , heart rate , muscle activity ) .
it is conceivable that the relaxation program improved the patients ability to minimize the restlessness characteristic of akathisia . the sustained effect at 1-week follow - up is intriguing and warrants further study , possibly comparing structured relaxation with current pharmacological treatment options , such as use of beta - blockers or benzodiazepines in an open label clinical trial . | tie overdressing is commonly used to secure the graft against the raw surface and prevent loss due to of hematoma or seroma . a conventional tie over dressing with silk sutures ,
is a useful method of securing the graft to raw area .
refixation is difficult when repeated tie over dressings are needed .
we assessed a low cost repeated tie over dressing method using sterile sample collection containers and silk suture threads in eight patients .
after the graft is applied on the bed , tie - over stitches are taken , and paraffin gauze is applied over with adequate padding ; the tie over sutures are passed through the container and the lid is tightened over it to complete the dressing .
the lid can be unscrewed easily at any time to inspect the graft and can be easily reapplied in the outpatient department .
the skin graft take in all the patients was complete without any seroma or hematoma .
a novel and low - cost tie over dressing that enables simple fixation of the dressing , to maintain proper position of grafts that require repeated fixation is reported here . |
since 2006 , surveillance physicians have listed and collected blood from those patients encephalitis , defined as fever or history of fever with axillary temperature > 38.5c ( 101.3f ) and altered mental status , new onset of seizures , or new neurologic deficit in patients admitted to 3 nipah surveillance hospitals : rajshahi , rangpur , and faridpur medical college hospitals .
the institute for epidemiology disease control and research and us centers for disease control and prevention tested serum with an igm - capture enzyme immunoassay to detect niv igm , and we defined laboratory - confirmed niv encephalitis as niv igm in serum . during december 2012march 2013 , surveillance physicians interviewed accompanying caregivers of all hospitalized patients whose illness met the encephalitis case definition on admission in the inpatient ward .
study physicians asked about patients consumption of raw or fermented date palm sap and contact with other persons with fever and altered mental status in the month before illness onset ; if caregivers were unaware of the patient s exposures , study physicians asked them phone the patient s friends and colleagues about exposures .
hospital physicians used personal protection equipment and provided it to caregivers of each patient with encephalitis and a history of these exposures . as part of subsequent epidemiologic studies
, we also conducted detailed case investigations at each niv encephalitis case - patient s household .
we interviewed surviving patients directly , or appropriate proxies among family , friends and relatives for patients who died , about their exposures to encephalitis patients or to fresh or fermented date palm sap before illness .
we calculated the sensitivity , specificity , positive predictive value ( ppv ) , and negative predictive value ( npv ) of the screening questions asked on admissions to hospitals by comparing with the niv igm results .
we repeated the calculations for patients hospitalized during january and february , when the prevalence of niv encephalitis is highest .
we compared the answers provided by caregivers during patient hospitalization with those provided during interviews in the community as part of our epidemiologic studies .
icddr , b s ethical review committee reviewed and approved the protocol for niv surveillance and case investigation .
they collected and tested blood samples from 328 ( 91% ) patients for niv igm .
seventeen ( 5% ) had niv igm ( table 1 ) , of whom 15 ( 88% ) niv encephalitis case - patients were identified during january and february 2013 . of the 17 confirmed case - patients , family caregivers of 14 reported either a history of drinking raw or fermented date palm sap or contact with other persons with fever and altered mental status in the month before illness onset .
therefore , the sensitivity of the screening questions was 82% , specificity was 86% , ppv was 24% and npv was 99% ( table 2 ) .
the sensitivity during january february was 93% , specificity was 82% , ppv was 37% , and npv was 99% . * drinking raw or fermented date palm sap or having contact with encephalitis patients in month before illness onset . at admission , 3 ( 18% )
niv encephalitis case - patients had no reported history of drinking raw or fermented date palm sap or of contact with persons who had encephalitis ( table 1 ) .
however , during the epidemiologic investigations in the community , family members of 2 case - patients reported that the patients drank fermented date palm sap in the month before illness onset .
of the 14 niv encephalitis case - patients who , at admission , had reported 1 of the risk exposures , results were consistent with exposures reported during the epidemiologic investigation .
screening patients with possible encephalitis at the time they seek hospital care regarding recent exposure to date palm sap and to other patients with encephalitis demonstrated high sensitivity and specificity for detecting niv encephalitis , particularly during peak months of niv encephalitis incidence .
the high npv of the screening questions suggests that focusing infection control efforts toward patients with these exposures is an efficient use of scarce resources to prevent transmission .
although three fourths of encephalitis patients had reported histories of exposure , they possibly could have had other infections , including other bat - borne viruses , that were transmitted through similar routes or could have lacked niv igm , despite having niv infection ( 13 ) .
alternatively , recent consumption of date palm sap by these patients might have been purely coincidental because this practice is common in bangladesh during this season , but nipah infection is rare . for 2 niv
encephalitis case - patients , caregivers did not report a history of drinking fermented date palm sap during hospital interview , but this behavior was reported in later community investigations . because 90% of bangladeshis are muslim , and consumption of alcohol is prohibited by islam ( 14 ) and illegal in bangladesh
, patients might be reluctant to report drinking traditional liquor made of fermented date palm sap .
therefore , caregivers should be asked about socially stigmatized behaviors privately and confidentially to increase the odds that these stigmatized behaviors are reported .
exposure - based screening can detect patients at high risk for niv encephalitis in low - income , resource - constrained settings , such as bangladesh .
we deployed screening questions on admission to inpatient wards but screening earlier , at triage in emergency wards , could further reduce risk .
surveillance for other diseases with well - described exposures that put healthcare workers at risk , such as ebola virus infection , and where laboratory diagnosis is limited or delayed could also deploy this approach . dental caries in primary teeth has been widely studied in many countries worldwide since it is known to be one of the most common oral diseases of childhood .
however , figures to quantify the prevalence of such disease in palestine are almost inexistent .
it is extremely important to study the prevalence of dental caries in preschool children in palestine to be able to provide a clear picture about the situation of oral and dental health in such population so as to help the local authorities to make future plans to reduce as much as possible the incidence of dental caries .
many countries have developed different strategies to reduce or even eradicate dental caries from preschool children following dental caries surveys .
some have already obtained excellent results using water and salt fluoridation , dental health education , school oral health program , and so forth . in palestine ,
dental caries surveys are rare and are unable to draw the attention of the local health authorities to take any reaction in fighting such common disease .
the aim of this study is to give an idea about the actual situation of dental caries among preschool children in palestine .
the results found here would probably be helpful in making future plans concerning the best methods to lower the level of such oral disease and make necessary improvements in the palestinian health system especially in the field of oral and dental health problems .
the prevalence of dental caries in primary teeth is commonly evaluated using the dmft index . the number of decayed , missing , and filled primary teeth is calculated in each child to obtain a sum that is known to be the mean dmft score in such child .
children of both sexes aged between 4 years and 5 years ( n = 1376 ) who were accompanied by their parents to the dental centre of the arab american university of jenin during the first 10 months of 2013 were examined clinically by one of the three calibrated examiners in the clinics of pediatric dentistry department after gaining the permission of the parents . as the families come from different geographic areas of northern palestine , the examined population was subdivided as shown in table 1 .
medical and dental histories were taken for each child by the help of one of the parents before starting the clinical examination .
the parent was asked about the mother 's level of education ( elementary school , secondary school , college graduated , or postgraduate studies ) .
children who had previous history of receiving any specific organized preventive treatment were excluded from the study .
clinical examination intraorally was achieved using sterile dental mirror and sterile dental explorer under dental unit 's light .
the presence of cavitation has been considered to be indicative of carious lesion in accordance with the criteria recommended by the who in 1997 [ 24 ] .
teeth which were missing due to trauma or congenitally absent teeth were excluded from the data processing ; therefore , they did not contribute to the final score .
the number of dental restorations has also been registered for each child which contributed to the f component in the dmft score .
the dmft scores of the clinically examined children were registered as shown in table 2 .
it was found that only 330 children ( 24% ) were caries - free ( dmft = 0 ) at the age of 4 - 5 years while the other 1046 children have already experienced dental caries at this age . as for the ( d ) component
, it was found that 932 children have had at least dental decay or caries in one or more primary teeth at the cavitation stage by the age of 4 - 5 years as shown in figure 1 .
however , the number of decayed teeth in the whole population studied here was 2895 .
the mean dt would be 2.10 while the number of children who had at least one missing primary tooth due to dental caries was only 98 representing 7.1% of the overall sample .
the figure below shows the prevalence of missing teeth ( mt ) in the studied population ( figure 2 ) . the number of missing teeth in the whole studied population was 163 .
when looking to the number of filled teeth representing the ( f ) component here , it was found that the number of children who had at least one restored primary tooth ( filled tooth ) was 154 representing 11.2% of the whole sample as shown in figure 3 , while the total number of restored teeth was 325 .
when asked about their level of education , mothers of examined children were divided into 4 groups as shown in table 3 . as mentioned above , the number of children who had at least one restored ( filled ) tooth was 154 .
mothers of such children have been found to have a high level of education in comparison to mothers of children who have decayed ( cavitated ) teeth or missing teeth ( table 4 ) .
correlation between mother 's level of education and the presence of fillings in her child 's mouth has been found to be statistically significant ( p value < 0.05 ) .
the overall caries prevalence in the study population was 76% with an overall mean dmft score of 2.46 of which decayed component is 2.10 , missing component 0.12 , and filled component 0.24 .
difference between males and females in caries prevalence at the age of 4 - 5 years has not been found to be significant as demonstrated by table 5 . as seen above , caries prevalence in primary teeth of 4- to 5-year - old girls
is 74.6% which is very close to that of boys at the same age ( 77.2% ) .
the table below shows the mean dmft scores for both sexes ( table 6 ) .
the prevalence of dental caries in primary dentition in 4- to 5-year - old children in northern palestine would be about 76% which means that almost 3 children out of 4 have already experienced dental caries by the age of 5 years in northern palestine .
figures found here seem to be far from the who / fdi goals for 2000 ; that is , 50% of 5- to 6-year - old children should be caries - free . when compared to other developing countries , recent studies in pakistan and india revealed that caries prevalence in preschool children in different regions of both countries is about 5060% which is considered much better than what we found in our study here [ 69 ] .
on the other hand , caries prevalence in preschool children in some arab countries like saudi arabia has been found to be high ( approaching the 75% ) [ 1012 ] . in the united arab emirates ,
a high prevalence of caries among preschool children has been registered as well ( 7080% ) [ 13 , 14 ] .
kuwaiti kindergarten schoolchildren who are caries - free at the age of 4 - 5 years do not represent more than 2432% of such population according to a national epidemiologic survey done in kuwait in 2010 .
these findings are almost similar to what we found here in northern palestine , although they are still far from the figures published by many developed countries as we could find in the united kingdom ( 4060% caries prevalence in 5-year - old children ) or in sweden ( 69% of 3-year - old preschool children are caries - free in 2003 ) as well as in brisbane , australia ( 66% of 4- to 6-year - old children are caries - free in 2002 ) [ 1618 ] . a probable explanation for such discrepancy can be the following : inequality in economic conditions and resources , effective fluoridation policy , efficiency of healthcare system , availability and consumption of refined sugars , standard of oral health awareness among public , dietary and oral hygiene lifestyles , and motivation status of parents and children .
as expected , highly educated mothers tend to take their children to the dentist early in their lives so as to make regular check - ups and treat dental caries as soon as it appears , which best explains the high f component ( ft ) in case of children of college - graduated mothers .
the mean dmft scores as well as caries prevalence in primary teeth of 4- to 5-year - old palestinian children reflect a considerable defect in the oral health care at home and at school , showing a real need to establish school oral health programs in the different regions of northern palestine [ 1 , 7 ] .
such programs are possible to implement in cooperation with the ministry of health so as to be able to finance them properly .
it is always possible to prevent dental caries in primary dentition and lower the caries prevalence in young children in northern palestine starting with good dental health education of the parents and teaching them how to take care of their children 's teeth as soon as they start to erupt .
emphasis on babies feeding habits as well as the use of kids toothpastes is also important [ 1 , 20 ] .
parents should be encouraged to take their children to the public dental clinics directed by the ministry of health or to their private dentist before the age of 1 year .
they would be able to have an idea about dental health education programs and topical fluoride application campaigns .
preventive measures campaigns including topical fluoride application , fissure sealants , and healthy diet promotion would be a lot of help to improve the situation of the oral and dental health in young children who go to nurseries and kindergartens . it is always possible to find volunteer dentists locally or to employ freshly graduated dentists to provide preventive measures to children in nurseries and kindergartens in different northern palestinian districts including fluoride varnish application to all primary teeth as early as possible , educating the teachers as well as the parents about the best hygiene methods and toothpastes to be used by preschool children , making regular dental check - ups to the children , and giving them advices about healthy nutrition . | we measured the performance of exposure screening questions to identify nipah virus encephalitis in hospitalized encephalitis patients during the 201213 nipah virus season in bangladesh . the sensitivity ( 93% ) ,
specificity ( 82% ) , positive predictive value ( 37% ) , and negative predictive value ( 99% ) results suggested that screening questions could more quickly identify persons with nipah virus encephalitis . |
parasitic infections are increasing worldwide due to rapid urbanization of cities , global warming , and international traveling .
the diagnosis of parasitic diseases of the respiratory system is challenging because clinical manifestation and radiological findings are not specific .
the larvae can cause airway inflammation , whereas migration of the mature adult worm may cause mechanical obstruction .
we present a case of interstitial pneumonitis caused by parasitic infestation , which was diagnosed on transbronchial lung biopsy ( tblb ) .
a 54-year - old female presented with a history of exertional breathlessness and dry cough for two months .
she was afebrile with spo2 of 91% on room air and bilateral crepitations on respiratory examination .
l ( 46.4% ) , fvc of 1.45 l ( 45.2% ) , and fev1/fvc 83% with reduction in dlco 1.46 mmol / min / kpa ( 18% ) and corrected value with alveolar volume 0.49 mmol / min / kpa ( 30% ) .
high - resolution computed tomography ( hrct ) of chest showed multiple ill - defined nodular opacities in both lung fields showing centrilobular distribution associated with ground glass opacities bilaterally .
fine reticular densities were seen at places with segments of traction bronchiectasis [ figure 1 ] .
total serum ige was 145 iu / ml and specific ige for pigeon droppings ( < 0.10
tblb showed thickened alveolar septa with mild lymphoplasmacytic inflammatory infiltrate in the interstitium with cross - sections of calcified parasitic larvae with foreign body giant cell reaction around it [ figure 2 ] .
travel history revealed frequent travels in the himalayan belts yearly for 20 years during which she had frequent episodes of diarrhea .
she was given albendazole 400 mg for three days and oral corticosteroids for 12 weeks .
bronchoalveolar lavage ( bal ) for tuberculosis culture at 6 weeks was negative . at 6 weeks
high - resolution computed tomography chest showing multiple ill - defined centrilobular opacities with ground glass opacities bilaterally before treatment histopathology image of transbronchial lung biopsy showing calcified larva with thickened alveolar septa with mild lymphoplasmacytic inflammatory infiltrate in the interstitium high - resolution computed tomography chest showing remarkable improvement after treatment
diffuse lung diseases can be further divided into transient pulmonary infiltrates and alveolar or interstitial lung diseases .
ascariasis , anchylostomiasis , and toxocariasis usually cause transient pulmonary infiltrates , whereas schistosomiasis , strongyloidiasis , and tropical pulmonary eosinophilia can cause diffuse interstitial changes , as in the current case .
strongyloidiasis causes reticulonodular opacities because of secondary infection , hemorrhage , inflammatory pneumonitis , and bacterial abscess formation .
schistosomiasis eggs that are not passed into bladder or intestinal lumen are the main cause of chronic lung diseases causing granulomatous reaction and fibrosis .
tropical pulmonary eosinophilia typically results from a hypersensitivity reaction to wuchereria bancrofti and brugia malayi .
tropical pulmonary eosinophilia is an immunological response to microfilariae rather than acute infection , which usually present as reticulonodular opacities .
the diagnosis of parasitic infections is difficult on chest radiography or hrct of chest because of nonspecific presentations .
bronchoscopy may be helpful for diagnosis of parasitic infestation by variety of ways like direct visualization , bal , brushing , and tblb .
parasitic lung diseases can also show microscopic pulmonary calcification on lung biopsy . in the current report ,
transbronchial lung biopsy revealed showed thickened alveolar septa with mild lymphoplasmacytic inflammatory infiltrate in the interstitium with cross - sections of calcified parasitic larvae .
tblb is performed for obtaining tissue specimen from peripheral lung masses and focal or diffuse lung infiltrates .
the technique is useful in patients with suspected lung cancer , fungal and mycobacterial lung infections , unexplained infiltrates in immunocompromised hosts and in patients with suspected pulmonary sarcoidosis , lymphangitic carcinomatosis , and in selected cases of pulmonary langerhan 's cell histiocytosis , lymphangioleiomyomatosis , and cryptogenic organizing pneumonia .
with increasing travels and worldwide migration , parasitic infections should be considered in differential diagnosis of interstitial lung diseases particularly in the evaluation of diffuse lung infiltrates .
bronchoscopy and transbronchial lung biopsy can be useful in the diagnosis of diffuse lung infiltrates .
ventriculoperitoneal shunt ( vps ) is a well - established cerebrospinal fluid ( csf ) diversion procedure in cases of hydrocephalus .
there are a lot of complications associated with this procedure and shunt extrusion via different natural orifices is one of them .
transanal and per abdomen shunt extrusion are well documented in literature while shunt extrusion via urinary bladder , vagina , uterus , gall bladder , urethra , and scrotum are sporadically reported in the literature .
the distal end of shunt erodes through the wall of hollow viscera , approaches their lumen , and protrudes outside through natural orifices .
the distal end of shunt protrusion depends on stiffness of shunt tip , thickness of the wall of hollow viscera , condition of the visceral wall , underlying infection , fixity of viscera , and the operative hand of the surgeon .
the incidence of perforation is inversely related to the mobility of gut , and colon is the most frequently perforated viscus due to its immobility .
a 10-month - old male child presented with protrusion of shunt tubing from the anus , incidentally noticed by his parents .
the patient had a history of excessive cry along with extrusion of shunt tube through anal orifice [ figure 1a ] .
the abdomen was soft , nontender , and bowel sounds were audible on abdominal auscultation .
his developmental milestones were delayed , and rests of the neurological examinations were within normal limit .
x - ray abdomen erect imaging showed no pneumoperitoneum [ figure 1b ] . computed tomography head showed dilated lateral and third ventricles .
the patient was taken up for the removal of previous shunt assembly and placement of new one . at the time of distal end removal , lower end of shunt
had retracted back into rectum ; hence , we planned to remove that via incision at abdominal insertion point .
two incisions were given , one at the cranial end of shunt tubing , and another one at the abdominal insertion site .
the shunt was divided at the abdominal insertion point , and cranial part was pulled out from cranial incision while abdominal part was pulled through the abdominal incision .
the shunt tube had perforated the appendix and entering through the perforation [ figure 2a and b ] .
an urgent gastrosurgery opinion was taken and urgent laparotomy via extension of same incision was performed by the gastrosurgeon .
postoperative period was uneventful , and patient discharged from hospital after removal of stitches and is under follow - up .
( a ) protrusion of the lower end of the shunt through anus , ( b ) a radiograph of a patient showing the course of the lower end of the shunt through a large intestine ( a ) an intraoperative photograph showing the appendix coming out of the abdominal incision while pulling the lower end of the shunt which was perforating the appendix at the tip ( black arrow ) , ( b ) the perforated appendix during appendicectomy
vps is most commonly used and universally accepted surgical procedure for the management of hydrocephalus in children .
a number of complications associated with this procedure are mentioned in literature with abdominal complications accounting for 1030% of all .
bowel perforation by the tip of the catheter and extrusion of the shunt through external orifices is one of them . although this is a rare complication and have incidence rate of 0.10.7% only .
the first case of anal extrusion of distal vps was reported by wilson and bertan in 1966 .
the duration between shunt surgery and bowel perforation was found to be minimum in infants and was related with the age of the patient .
the interval between shunt insertion to the protrusion of catheter from anus ranges from 2 to 20 months with an average of 6.1 months . in our case , this period was 6 months .
nonenteric visceral perforation has also been sporadically reported in the literature and that includes urinary bladder , urethra , scrotum , vagina , gall bladder , and uterus .
factors associated with perforation of gut are foreign body reaction , stiff tip of shunt , thin bowel wall in pediatric patients , abdominal infections , silicon allergy , use of trocar for insertion of peritoneal end , previous abdominal surgery , and chronic wear and tear produced by tip of the shunt .
many patients do not present with significant abdominal symptoms because the fibrous tract formed at the perforated site usually seals the perforation and prevents spillage of fecal matter into the peritoneum , which would otherwise lead to peritonitis .
hence , the correct diagnosis may be delayed until a very later stage at which gram - negative or anaerobic meningitis , encephalitis or ventriculitis has been fully established , leading to significant morbidity and/or mortality to the patients .
mechanism of shunt extrusion is not well - established but most accepted hypothesis is that after bowel perforation shunt tubing propels outside with sequential peristaltic movement of the gut . in the cases of
shunt extrusion through anal opening , most common site of bowel perforation is colon ( 70% ) while in cases of oral extrusion most common site is the stomach .
early diagnosis , adequate clinical , radiological and biochemical evaluation , and prompt treatment are the key to successful treatment .
the standard method of treatment is the removal of the extruded shunt system , control of infection , improvement of general condition followed by csf diversion procedure .
the different available alternatives are laparotomy with the revision of the peritoneal end of shunt , conventional exploratory laparotomy and repair of bowel perforation , endoscopic localization of enterotomy site and removal of shunt , shunt removal , external diversion of csf and use of antibiotics , and later on replacement of vps when no infection are seen . in uncomplicated cases of
shunt extrusion , shunt tubing can be removed via extruding orifice and bowel perforation can be managed conservatively while in complicated cases shunt removal is advised via a laparotomy .
appendicitis after ventriculoperitoneal shunting is a known complication but to best of our knowledge shunt extrusion via appendicular perforation is not documented in literature yet . in such cases ,
shunt removal via extruding orifice may be quite difficult . theoretically , there are high possibilities of associated appendicitis which can result in the development of frank peritonitis or appendicular abscess after shunt removal through extruding orifice .
so in our opinion , such type of cases whether complicated or not should be placed in special category and shunt removal should be done via laparotomy and followed by prophylactic appendectomy .
. such type of case should be consider in special category in which shunt removal without proper surgical exploration and added appendicectomy may be quite difficult and hazardous for the patient .
optimal management plan in such type of cases either uncomplicated or complicated is laparotomy and removal of the shunt with added appendicectomy .
| parasite infections are increasing worldwide due to increasing migration and traveling .
parasitic infections can affect lungs and present as a focal or diffuse lung diseases .
high index of suspicion and detailed history are most important .
we present a case of interstitial pneumonitis caused by parasite infestation , which was diagnosed on transbronchial lung biopsy . |
recently , new oral anticoagulants have been approved as alternatives to warfarin for patients with atrial fibrillation .
rivaroxaban is one of the novel anticoagulants , which is an oxazolidinone derivative and inhibits both free factor xa and factor xa bound with the prothrombinase complex .
it is a highly selective direct factor xa inhibitor with oral bioavailability and rapid onset of action .
there are some advantages of the new agents compared with warfarin including rapid anticoagulation after an oral dose and lack of dietary or drug - drug interaction . however , there are no specific antidotes for the anticoagulant effect of rivaroxaban in the event of a major bleeding , unlike warfarin
. we present a case of spontaneous rectus sheath hematoma ( rsh ) during rivaroxaban therapy for atrial fibrillation in an elderly female patient .
a 75-year - old woman presented to the emergency department with the complaints of fatigue and abdominal pain after coughing .
the patient had been started on new oral anticoagulant agent rivaroxaban therapy for nonvalvular atrial fibrillation for 3 days .
the dose of 20 mg / day rivaroxaban was started because the creatinin clearance of the patient was above 50 ml / min .
she had a blood pressure of 70/40 mmhg and an irregular heart rate of 115 beats / min on admission .
the patient had no history of any trauma or surgery ; she reported that the symptoms started after vigorous coughing .
blood analyses revealed leukocytosis ( 26.5 k / ul ) accompanied by severe anemia ( 5.4 g / dl ) .
platelet counts were within normal ranges and her international normalized ratio ( inr ) was 1.48 .
her abdominal x - ray was normal and the stool occult blood test was negative .
after the first treatment , the patient was transferred to the intensive care unit ( icu ) .
repeated abdominal examination in the icu revealed increased tenderness and a palpable mass on the left side of the umbilicus .
noncontrast abdominal computerized tomography scan showed a left - sided rsh , 102 45 mm in size [ figure 1 ] . a specific antidote for rivaroxaban
is not available then the patient was treated with fluid resuscitation and packed red blood cells .
computed tomography scan of the abdomen shows a left - sided rectus sheath hematoma ( arrow )
rivaroxaban is an oral anticoagulant agent that directly inhibits factor xa and interrupts both the intrinsic and extrinsic pathway of the coagulation cascade .
rivaroxaban is currently indicated for use in patients for atrial fibrillation and prophylaxis of deep venous thrombosis .
it does not require inr monitoring like warfarin . with the increasing use of the new anticoagulant agents like rivaroxaban in atrial fibrillation , bleeding complications due to these agents
there are no specific antidotes for the anticoagulant effect of rivaroxaban and other new oral anticogulants unlike warfarin , thus the management of the bleeding complications include support and observation .
currently , no available specific antidote exists for the management of rivaroxaban - associated bleeding events , but supporting therapy is useful which are likely to be effective for the majority of patients because of the short half - lives of these agents .
recent studies showed that rivaroxaban was noninferior to warfarin for the primary endpoint of stroke and systemic embolism .
there was no reduction in rates of mortality or ischemic stroke , but a significant reduction in hemorrhagic stroke and intracranial hemorrhage .
the primary safety endpoint was the composite of major and clinically relevant nonmajor bleeding , which was not significantly different between rivaroxaban and warfarin but , with rivaroxaban , there was a significant reduction in fatal bleeding , as well as an increase in gastrointestinal bleeds and bleeds requiring transfusion .
the main causes of the rsh include anticoagulant therapy , hematological disorders , trauma , excessive physical exercise , coughing , sneezing , and pregnancy . especially in elderly patients the risk of rsh may be increased due to the impaired functional status and weakened rectus muscle
. early recognition , rapid assessment and treatment are important to reduce the complications such as hemodynamic instability , abdominal compartment syndrome , multiorgan dysfunction and even death .
the treatment of such a hematoma includes transfusion with packed red blood cells and supporting therapy based on regularly monitoring of hemoglobin levels .
rivaroxaban has a mean terminal half - life of 7 - 11 h so in bleeding events supporting therapies are likely to be effective for the majority of patients .
several studies have shown that prothrombin complex concentrate may be useful in reversing the effects of rivaroxaban .
other possible measures include the use of recombinant factor viia to reduce bleeding or the use of activated charcoal to reduce absorption in cases of overdose .
several factors are reported which increase the risk of patients developing hemorrhage while receiving rivaroxaban , these include advanced age , hypertension , history of hepatic / renal disease , previous stroke , coagulopathy , concomitant use of antiplatelet agents and alcohol consumption .
jaeger et al . have reported a 61-year - old female patient who developed a spontaneous spinal epidural hematoma after being treated by rivaroxaban .
boland et al . also reported acute onset severe gastrointestinal tract hemorrhage in a postoperative patient taking rivaroxaban after total hip arthroplasty . in our patient
, there was no other medication except rivaroxaban that could cause the hematoma . based on naranjo 's scale
, a score of 7 showed that the rivaroxaban was the probable cause of the rsh .
several case reports of muscle hematoma due to the antiplatelet and anticoagulant agents have been reported previously , but this is the first reported case of spontaneous rsh due to the rivaroxaban . spontaneous splenic rupture occurs usually secondary to abdominal trauma , most often in a spleen affected by infection or haematological malignancy .
spontaneous splenic rupture is extremely rare , especially presenting in a patient subsequently diagnosed with splenic marginal cell lymphoma ( smzl ) , which itself is rare and as a result only one case has been described in the literature so far .
we report a case of a 71-year - old woman with a past medical history of inflammatory myelitis , osteoporosis , asthma , coeliac disease and chronic back pain who was admitted as an emergency with new onset and severe back and abdominal pain .
she described a 5-day history of persistent bilateral back pain radiating to the shoulder tips and groin worse when lying flat .
she attended the emergency department 3 days before with a similar complaint but discharged home the same day .
the inflammatory markers were following : crp 88 and white cell count 12.3 ; biochemistry and clotting screen was normal .
laparotomy was performed and a large splenic haematoma with 1 l of blood in the abdomen and a splenectomy was performed .
the postoperative period was complicated by a lower respiratory tract infection and some difficulty with mobilization but the patient made good recovery otherwise . during the post - operative period she developed a hypoglossal nerve palsy , which was assessed by the neurologist .
figure 1ct chest / abdomen demonstrating a large splenic haematoma with hyper- and hypo - dense areas .
ct chest / abdomen demonstrating a large splenic haematoma with hyper- and hypo - dense areas .
high attenuation fluid is present within the pelvis consistent with blood . the excised spleen weighed 560 g with blood clots and was macroscopically normal .
microscopically ( figs 24 ) , the histology and immunohistochemistry was consistent with a diagnosis of smzl .
subsequently , a bone marrow biopsy and staging ct were performed and no distant spread of disease was demonstrated .
cd79a highlights the presence of marginal expansion of the white pulp lymphoid mantle with satellitosis ( nodular infiltrates ) in the red pulp .
the monomorphic population of medium - sized lymphoid cells also stained positive for cd20 , bcl2 , igm and igg consistent with smzl .
figure 4marginal expansion of the white pulp lymphoid mantle with satellitosis in the red pulp .
cd79a highlights the presence of marginal expansion of the white pulp lymphoid mantle with satellitosis ( nodular infiltrates ) in the red pulp .
the monomorphic population of medium - sized lymphoid cells also stained positive for cd20 , bcl2 , igm and igg consistent with smzl .
the term spontaneous rupture is poorly defined in the literature and distinguished from pathological rupture of the spleen . dating back to 1958
peskin and orloff described true spontaneous rupture in cases where there is no trauma pre- or intraoperativly , the spleen is not affected by any disease , no perisplenic adhesions present indicating previous splenic trauma and on gross and histological examination of the spleen must be normal .
however , in the current literature , spontaneous splenic rupture describes a splenic rupture occurring without trauma whether the spleen is involved in pathology or not .
first , in haematological malignancies , the splenic parenchyma gets congested with blast cells causing increased intrasplenic tension , which exceeds the capacity of the non - distensible splenic capsule and eventually results in rupture .
secondly , vascular occlusion secondary to reticular endothelial hyperplasia results in thrombosis and infarction , which may distort the splenic architecture making it weaker and thirdly deranged coagulation , which may be part of a systemic disease or caused by thrombocytopenia secondary to massive splenomegaly .
splenic rupture is a life - threatening event and a differential diagnosis in a patient presenting with acute abdomen , although not all cases present with abdominal pain .
haemodynamic instability and kehr 's sign , defined as left hypochondriac pain radiating to the left shoulder , occurs in 20% of cases although neither signs are sensitive nor specific .
ultrasound scanning , which is usually readily available in emergency departments , is often a choice of initial imaging and is 7278% sensitive and 91100% specific .
treatment of splenic rupture depends on the site and size of rupture , whether the patient is haemodynamically stable and the underlying pathology causing the rupture . in
haemodynamically stable patients it is becoming more common to treat patients by splenic artery embolization , whilst unstable patients require surgical management [ 5 , 6 ] .
splenic marginal zone lymphoma is classified under the non - hodgkin 's lymphomas ( nhl ) and account for < 1% of these .
marginal cell lymphomas are indolent small b - cell lymphomas , which originate from the marginal zone of the lymphoid follicle , which consists of a germinal centre surrounded by a corona that is divided into the marginal zone and the mantle zone .
the world health organization classifies the marginal zone - derived lymphomas into splenic ( smzl ) , lymph node ( nodal marginal zone lymphoma ) and extra - nodal mucosa - associated lymphoid tissue ( malt lymphoma ) despite having a common origin in the marginal zone of the b - follicle as they have distinct clinical and molecular characteristics [ 2 , 7 ] .
smzl usually presents as massive splenomegaly and/or with abnormalities in the full blood count , especially anaemia and thrombocytopaenia usually secondary to splenic sequestration rather than bone marrow infiltration [ 5 , 7 ] .
retrospective analysis of blood results dating back a number of years , revealed no abnormalities in the full blood count ; therefore , it appears that splenic rupture was the first presentation in this case and she is in remission at present after undergoing splenectomy as no distant disease was found .
the treatment of smzl is not standardized and there are no reported prospective studies comparing outcome with immunotherapy , chemotherapy or splenectomy . in the past splenectomy was regarded as a choice of treatment until rituximab was introduced , which has shown responses of up to 100% .
this trial concludes that splenectomy should not be regarded as first - line treatment but considered in patients who do not respond to immunotherapy or chemtotherapy .
patients with splenomegaly may suffer abdominal discomfort , early satiety and cytopaenias where splenectomy is indicated for palliative purposes [ 7 , 8 ] . as this lady presented with spontaneous splenic rupture
, it raises the question that when and if splenectomy should be performed prophylactically if picked up on a scan or whether surveillance is indicated but at present the literature does not address this question but ultimately comes down to assessing each individual case taking into account their overall health and stage of disease . | rivaroxaban is an oral anticoagulant agent that directly inhibits factor xa and interrupts both the intrinsic and extrinsic pathway of the coagulation cascade and is currently indicated for use in patients for atrial fibrillation and prophylaxis of deep venous thrombosis .
the present case reports of spontaneous rectus sheath hematoma during rivaroxaban therapy for atrial fibrillation in a 75-year - old woman . |
neuromyelitis optica ( nmo ) or devic 's disease is a rare inflammatory , demyelinating disease of the central nervous system ( cns ) that predominantly targets the optic nerves and spinal cord 1 . the disease was first described in 1870 by albutt and 24 years later , devic described the clinical characteristics of nmo
the discovery of a specific nmo immunoglobulin ( nmoigg ) opened a new era in the classification and understanding of the pathogenesis of nmo 3 .
nmoigg binds to aquaporin4 , which is the main channel that regulates water homeostasis in the cns .
diagnostic criteria for nmo with aquaporin4 antibodies ( aqp4ab ) requires at least one core clinical characteristic , a positive test for aqp4ab using best available detection method ( cellbased assay recommended ) and exclusion of alternative diagnoses 1 .
the core clinical characteristics are , for example , optic neuritis , acute myelitis , acute brainstem syndrome , symptomatic narcolepsy , or symptomatic cerebral syndrome with typical nmo brain lesions 1 .
neuromyelitis optica must be distinguished from other demyelinating diseases , for example , multiple sclerosis .
the presence of aqp4ab differentiates nmo from multiple sclerosis ( ms ) with high specificity 4 .
in contrast to typical ms , the clinical events in nmo are usually more severe 5 , 6 .
cerebrospinal fluid ( csf ) findings in nmo are also known to differ significantly from those in classical ms . csfrestricted oligoclonal igg bands are absent in most nmo patients .
however , pleocytosis is usually mild , and frequently includes neutrophils , eosinophils , activated lymphocytes , and/or plasma cells 6 , 7 .
studies carried out in europe , south east and southern asia , the caribbean and cuba suggest that the incidence and prevalence of nmo ranges from 0.050.4 to 0.524.4 per 100,000 , respectively 9 .
the disease is mainly sporadic , although a few familial cases have been reported 10 .
we describe a case of an unusual and severe course of nmo affecting almost the entire spinal cord and brain .
examination on the day of admission revealed normal results as regards ecg , troponin i , and computed tomography ( ct ) of the chest and abdomen .
the next day , the patient reported headache and neurological examination showed rightsided hemiparesis and afferent pupillary defect of the left eye suggesting an afferent optic nerve defect . within a few hours , the patient showed a rapid neurological deterioration with progressive tetraplegia and global decline .
mri of the spinal cord showed myelitis in the spinal cord segments c2 to th5 ( fig .
csf examination revealed polymorphonuclear pleocytosis ( leukocytes 1210 10/l , neutrophils 95% ) and an increased total protein concentration ( 2273 mg / l ) .
due to spinal cord mri and csf findings , infectious transverse myelitis could not be excluded and the patient was treated with dexamethasone , acyclovir , ceftriaxone , ampicillin , and levofloxacin .
( a ) sagittal t2fse mri of the spinal cord showing high signal changes . ( b )
axial t2flair brain mri showing high signal changes in thalami , internal capsule , and corpus callosum .
( c ) axial t2flair brain mri showing high signal changes in pons , medulla oblongata , cerebellum , and middle cerebellar peduncle .
the next day , the patient 's condition worsened ; she became comatose and had a respiratory failure that required assisted ventilation ; this might be caused by bilateral phrenic nerve involvement as its roots originate from c3 to c5 where the lesion was also seen ( fig .
brain mri showed high signal changes in thalami , internal capsule , and corpus callosum ( fig .
1b ) and also similar changes in pons , medulla oblongata , cerebellum , and middle cerebellar peduncle ( fig .
treatment with methylprednisolone and immunoglobulin was attempted and as serum aqp4ab was confirmed as positive ( indirect immunofluorescence assay was used , the titer was 19.85 , normal < 10 ) and the patient did not respond to the treatment , the patient was also treated by means of plasmapheresis .
histological examination of the cns revealed extensive , sharply limited demyelination and axon defect ( fig . 2a and b ) .
the spinal cord was almost entirely affected . only the lumbar area was partly spared .
smaller demyelination foci were found in the periventricular area of the hippocampus and in the corpus callosum .
demyelination was verified by showing both cd68positive macrophage infiltration and betaapp positivity as signs of axonal damage .
( b ) the axon defect is shown by immunostaining ( brown staining ) of betaamyloid precursor protein ( app ) .
this is the first case report of nmo described from finland from the aqp4ab era .
there is only one older publication of a finnish nmo patient from the preaqp4antibody era 11 .
the patient was referred to hospital with an acuteonset chest pain , which is an unusual first symptom of nmo .
demyelination affected almost the entire spinal cord , sparing only partly the lumbar cord , which is unusual at first myelitis .
lesions involving the lumbar or sacral spinal cord in addition to the cervical and thoracic portions have been reported only in 11% of patients at first myelitis .
previous reports have revealed that 92% of the patients have at least one spinal cord lesion extending over three or more vertebral segments at their first myelitis .
it has been reported that seropositive women have more severe clinical attacks than males , as evidenced by high lesion load in the spinal cord and other types of coexisting autoimmunity 6 .
brain mri abnormalities are relatively common and may be relatively unique by virtue of localization and configuration 12 , as seen also in our patient .
the histopathological findings in the cns , csf , and aqp4ab seropositivity are consistent with neuromyelitis opticatype demyelination , although the disease course of our case was not typical of nmo due to its rapid and severe course . according to hospitalbased observational studies , mortality of nmo ranges from 2.9% to 25% and
reported that disease duration at the time of death ranged from 6 months to 23.6 years 6 .
in addition , our patient 's histological examination revealed extensive , sharply limited demyelination of the spinal cord and brain in the acute phase of the disease . to the best of our knowledge ,
the treatment in this particular case was targeted to multiple causes of the symptoms due to the unknown etiology in the beginning of the disease .
acute attacks and relapses of nmo are generally treated with intravenous glucocorticoids followed by plasmapheresis for refractory or progressive symptoms 13 , 14 .
however , there are no controlled trials evaluating the treatment of nmo , and recommendations are primarily supported by data from observational studies and by the clinical experience of experts . despite several forms of treatment , the patient did not survive .
autoimmune hepatitis ( aih ) is characterized by chronic necroinflammation , manifesting as severe lobular necrosis and inflammation with broad areas of parenchymal collapse with no known cause .
in fact , aih might be associated with a number of other autoimmune diseases,1 but the exact mechanisms of onset remain unknown . in aih ,
the most common are antinuclear antibodies ( ana ) , smooth muscle antibodies ( sma ) , and antibodies to liver and kidney microsomes ( anti - lkm).1 evidence suggests that aih is induced by antibody - dependent cell - mediated cytotoxicity , which involves both antibody - mediated and cellular immunity against specific liver antigens on hepatocyte membranes.2 several studies have documented the involvement of genetic factors including human leukocyte antigen ( hla ) types dr3 and dr4.3 some reports have described aih cases in which a virus such as hepatitis a,4 hepatitis b,5 or epstein barr6 was the causative agent .
sera from patients with multiple autoimmune diseases have been found to have elevated cytomegalovirus ( cmv ) igm titers , perhaps implying an as yet unidentified association between cmv and autoimmune diseases.7 we present a case of fluorescence ana - negative aih - associated primary biliary cirrhosis ( pbc ) that appears to have been triggered by reactivated cmv infection .
in 2010 , a 63-year - old japanese woman was admitted to a local hospital , after which she was transferred to our hospital for investigation of liver dysfunction .
one month prior , she had experienced fever . she had no history of medicine or drug use .
the findings of physical examinations of admission to our hospital were the following : body temperature , 36.8c ; pulse rate , 69 beats / minute and regular ; blood pressure , 122/77 mmhg ; respiratory rate , 16/minute ; no sign of anemia at the conjunctiva palpebra , but an icteric finding was made at the conjunctiva bulbi .
the results of urinalysis were normal , with the exception of a positive result for urobilinogen .
the results of biochemical analysis of the blood were the following : total bilirubin , 6.6 mg / dl ( normal range : 0.21.2 ) ; direct bilirubin , 4.4 mg / dl ( normal range : 0.00.2 ) ; aspartate aminotransferase , 344 iu / l ( normal range : 1040 ) ; alanine aminotransferase , 238 iu / l ( normal range : 545 ) ; alkaline phosphatase , 572 iu / l ( normal range : 100325 ) ; -guanosine triphosphate , 129 iu / l ( normal value : < 30 ) ; igg , 2,498 mg / dl ( normal range : 8701,700 ) ; and igm , 279 mg / dl ( normal range : 46260 ) .
cmvigm antibody was high ; igg antibody was above the normal ranges at admission ( table 1 ) .
tests for hepatitis a igm antibody , hepatitis b surface antigen , and anti - hepatitis c virus antibody were negative .
ana was negative by fluorescence assay ( < 40 ) , but it was positive as measured using enzyme - linked immunosorbent assay ( elisa ) ( 44.1 index ; normal range < 20 ) , anti - mitochondria m2 antibody level was 18.4 ( normal value : < 7.0 ) , anti - lkm antibody was negative , anti - sma was < 20 , and anti - dsdna antibody ( < 7.0 iu / ml ; normal value : < 20 iu / ml ) was negative .
the level of anti - ribonucleoproteins antibody was 20.3 ( normal value : < 17 ) ( table 1 ) .
histological examination showed severe lobular necrosis , inflammation with broad areas of parenchymal collapse , and both nondestructive and destructive cholangitis surrounded by many lymphocytes and plasma cells ( figure 2 ) .
regarding results of electron microscopy , migrating lymphocytes were found more frequently in a medium - sized interlobular bile duct surrounded by many inflammatory cells .
complete absence or partial loss of microvilli and microvillous bleb formation was visible in the same bile duct epithelial cell ( figure 3 ) .
the clinical and pathological findings were highly suggestive of aih , the score of which was calculated according to the system proposed by the revised international aih group.8 the negative ana result assessed by fluorescent ana was used in the calculation .
the clinical / pathological findings of anti - mitochondria m2 antibody and chronic nonsuppurative destructive cholangitis indicated pbc .
her severe jaundice was improved , and her bilirubin and transaminase levels began to decline after administration of prednisolone for 3 days .
the role of viruses in aih has been proposed repeatedly , but convincing evidence has linked two viruses hepatitis a and epstein barr virus to type 1 of the disease , only in rare cases where a genetic predisposition exists and the viral infection occurs at the right time . in spite of an impressive amount of information conclusively showing molecular mimicry between cytochrome p4502d6 sequences ( the target autoantigen of autoantibodies characteristic of aih type 2 ) and viral ( hepatitis c virus , herpes simplex virus 1 , cmv , human t lymphotropic viruses 1 and 2 ) or bacterial ( salmonella typhimurium ) antigens , no infectious agent has been clearly implicated for causing type 2 of the disease.9 at admission and at 2 , 4 , and 8 weeks after admission , the respective cmv igm indices ( normal value : < 0.8 ) were 2.17 , 0.93 , 1.08 , and 1.407 ; and igg indices ( normal value : < 2.0 ) were 10 , 34 , 89.4 , > 128 , and > 128 . consequently , serial cmv ig measurements demonstrated a slight increase of igm at admission which declined gradually over time , whereas igg increased with time to very high levels .
these findings suggested cmv infection , consistent with a previous report that some patients with secondary cmv infection had long - lasting igm antibodies , and that all had igg antibodies to cmv.10 the present case is a rare case of negative fluorescence - ana and low - titer elisa - ana aih type 1 with coincident cmv infection .
although the clinical features strongly suggest aih , calculation of aih score using the result did not recommend a diagnosis of aih from negative fluorescence - ana .
immunofluorescence assay is the ana detection method used in the calculation of aih score.8 although immunofluorescence is a sensitive test , it is laborious when testing large quantities of patient samples and is subject to errors of human interpretation and variation in fluorescence microscopes.11 the enzyme immunoassay test system is an excellent alternative to the immunofluorescence assay system for screening patient serum for the presence of clinically significant anas .
the enzyme immunoassay efficiently screens large quantities of patient samples and reduces risks of human error.12 both methods have different issues with respect to precision .
our patient was positive for ama , and liver biopsy showed a mixed portal inflammatory infiltrate with destructive and nondestructive cholangitis ( figure 3c ) .
the reported frequency of ama in patients with definitive aih was 18 in 206 ( 7.5% ) patients as assessed by immunoblotting.13 therefore , ama positivity is not a typical aih finding . in the present case ,
the findings of ama positivity and destructive biliary injury resembling chronic nonsuppurative destructive cholangitis in the biopsy strongly suggest a diagnosis of pbc.14 although aih and pbc might share some common histological features , the patterns seen on liver biopsy typically differ .
interface hepatitis or lobular hepatitis with infiltration of plasma cells is a characteristic of acute or chronic aih.15 nondestructive ductular reaction is detected more frequently in acute aih than in chronic disease , but the destructive cholangitis lesions that are typical of pbc are not present.15,16 moreover , for electron microscopy , the bile duct epithelial cell membrane facing the lymphocyte was disrupted .
bile duct epithelial cell detachment is an important ultrastructural lesion associated with extensive bile duct destruction in pbc livers.17 cellular immune mechanisms involving t cell reaction were thought to be significantly involved in the formation of chronic nonsuppurative destructive cholangitis and bile duct loss.18 in our case , the presence of portal inflammation and mixed destructive and nondestructive cholangitis changes , supported by other biochemical changes , led to a final diagnosis of overlapping syndrome of acute exacerbation aih and pbc .
this case is rare because these changes coincide or even precede cmv infection . in the present case ,
elisa assay for ana and liver biopsy led to aih and early treatment with prednisolone , which produced a good clinical outcome . | key clinical messageneuromyelitis optica is a rare inflammatory , demyelinating disease of the central nervous system that predominantly targets the optic nerves and spinal cord .
our case represents an unusual and severe course of neuromyelitis optica . despite several forms of treatment
, our patient died after a severe and shortterm attack . |
there was a time when numerous asthmatics had to be treated with systemic corticoids either on a regular basis or with repeated short courses in order to achieve asthma control . while inhaled 2 agonists became available in the seventies it took until late eighties to have corticoids administered by the inhaled route ( ics ) .
it was then demonstrated that regular treatment with inhaled beclomethasone and budesonide was superior to regular inhalation of short acting 2 agonists in improving asthma symptoms and airway caliber and also reducing the use of rescue inhaled bronchodilators .
it soon appeared at the population level that asthma mortality was decreasing in parallel to the increasing use of ics .
in the mid - nineties it became clear that adding long acting 2 agonists ( laba ) to ics markedly improved airway caliber and symptoms , and in particular night symptoms , in those patients who remained uncontrolled with low dose of ics . due to their marked efficacy ,
the combination therapy rapidly became the gold standard of asthma treatment , and given the high prevalence of asthma in the general population , this drug association became a blockbuster in western world countries .
at the same time was launched cysteinyl - leukotriene receptor antagonist ( ltra ) as an alternative to ics for maintenance asthma treatment in those patients who did not tolerate or were afraid of taking ics . in randomized controlled trials ( rcts ) ,
ltra proved to be less efficient than low dose of ics in improving airway caliber , reducing symptoms , and controlling airway eosinophilic inflammation in asthmatics selected on the basis of a high reversibility to 2 agonists .
however , the superiority of ics disappeared when comparing the two classes of drugs in mild to moderate asthmatics in a real - life setting where ltra performed equally to ics to improve asthma control and quality of life , probably because of a better adherence to treatment given by the oral as compared to the inhaled route . at the turn of the century it was demonstrated that the combination of ics / laba was particularly effective in reducing asthma exacerbation in moderate to severe asthma , both ics and laba contributing to this important effect . the combination of ics / laba has shown not only efficacy in rcts but also effectiveness in real - life setting where patient selection criteria are less stringent , allowing a greater range of patients to participate .
the technique of induced sputum has been pivotal in the emergence of inflammatory phenotype concept in asthma and it has become usual to classify asthmatics according to the proportion of eosinophils and neutrophils in the airways .
it appeared that while eosinophilic asthmatics display a good clinical and functional response to a few - week treatment with ics , it was not the same for those patients without raised eosinophilic inflammation , and in particular in those with high sputum neutrophil counts , who really seemed to be insensitive to ics .
the concept of severe asthma has recently been reshaped in a practical way that severe asthmatics are those patients in whom control may not be achieved with or requires a combination of high dose ics / laba .
the first class of drug that was shown to bring clinical benefit is monoclonal antibody directed towards ige .
omalizumab proved to be efficient in reducing exacerbation rate and improving quality of life in severe allergic asthmatics who remained uncontrolled despite a combination of high dose ics / laba .
furthermore , the effectiveness of this medication has also been demonstrated in real life partly because of the magic and the regular follow - up imposed by the subcutaneous injection route .
similar to mild to moderate asthma , severe asthma was found to be heterogeneous with respect to the type of airway inflammation .
more than half of severe asthmatics display residual eosinophilic inflammation despite receiving high dose of ics and even oral corticoids by some of them . in those patients , increasing the dose of ics or even giving oral corticoids results in a significant reduction of exacerbation . in those severe eosinophilic asthmatics , mepolizumab , which is an antibody directed towards interleukin ( il)-5 , reduces exacerbation rate while allowing for a reduction and even a stop of oral corticoids in some patients .
efficacy of anti - il-5 regarding exacerbation rate seems to be particularly related to the blood eosinophil count .
it has recently been demonstrated that asthmatics who had both high blood and sputum eosinophil counts were more prone to exacerbation and poorer asthma control .
the effects of anti - il-5 on day - to - day asthma control and lung function are more controversial but shown in some studies using reslizumab .
it has recently been shown that the effect of omalizumab in reducing exacerbation rate was essentially limited to those patients with elevated feno ( fractional ex - haled nitric oxide ) and blood eosinophil counts .
monoclonal antibodies directed towards other cytokines , and in particular towards tnf , have yielded disappointing results so far .
some of them exhibit a marked increase in neutrophil counts in the airways . in those subjects , a few - week treatment with clarithromycin
may reduce the neutrophil counts and slightly improve the quality of life but fails to improve day - to - day asthma control .
long - term effect of such a treatment on asthma exacerbation remains unknown , but this point has to be clarified in the future . in this view
it is worth noting that , in a pilot study , asthmatics uncontrolled with moderate to high doses of ics and displaying a low blood eosinophil count had their exacerbation rate reduced by chronic treatment with low dose azithromycin .
effects of macrolides on severe neutrophilic asthma have to be confirmed in large scale rcts .
we can however ask whether choosing neutrophils as the main target of treatment may be a double - edged sword , as these cells play a critical role in innate immunity operating in the airways .
while ics have a potent effect on eosinophilic inflammation , their ability to oppose the airway remodeling is much more controversial .
thermoplasty , a new technique that delivers high energy in the bronchi during an endoscopic procedure , may bring significant benefit there . applying this technique three times three weeks apart
has proved to be safe and has resulted in a reduction of exacerbation and hospitalization together with improved quality of life in asthmatics uncontrolled by a combination of ics / laba .
this treatment , approved by the food and drug administration in usa , has still to make its way in europe before becoming the official treatment for asthma .
in particular it remains to determine in prospective studies which population of asthmatics may be the most suitable to benefit from this treatment procedure . as thermoplasty requires a technical expertise
ics have dramatically changed the course of daily life in many asthmatics over the past decades and are responsible for the sharp reduction in asthma mortality observed since the nineties .
nevertheless , this class of drugs may not be sufficient or even inefficient in some patients .
the respiratory physician dealing with asthma , and in particular with severe asthma , has to proceed with detailed functional and inflammatory investigations to better phenotype his / her patient thereby allowing to choose the most appropriate treatment . juvenile polyposis syndrome , a rare disorder in children , is characterized with multiple hamartomatous polyps in alimentary tract .
a variety of manifestations include bleeding , intussusception , or polyp prolapse . in this study , we present an 8-month - old male infant of juvenile polyposis syndrome initially presenting with chronic anemia . to the best of our knowledge ,
we report a rare case of an 8-month - old male infant who presented with chronic anemia and gastrointestinal bleeding initially .
panendoscopy and abdominal computed tomography showed multiple polyposis throughout the entire alimentary tract leading to intussusception .
technetium-99m - labeled red blood cell ( rbc ) bleeding scan revealed the possibility of gastrointestinal tract bleeding in the jejunum .
histopathological examination on biopsy samples showed peutz - jeghers syndrome was excluded , whereas the diagnosis of juvenile polyposis syndrome was established .
however , polyps recurred and occupied the majority of the gastrointestinal tract in 6 months .
juvenile polyposis syndrome is an inherited disease , so it is not possible to prevent it .
concerning of its poor outcome and high mortality rate , it is important that we should increase awareness and education of the parents at its earliest stages .
it has been shown that affected children are susceptible to cancers and fatal medical conditions .
the common presentations include anemia , recurrent gastrointestinal bleeding , diarrhea , rectal prolapse , intussusception , protein - losing enteropathy , starvation , and malnutrition .
an 8-month - old male infant presented melena and iron deficiency anemia with hematocrit of 10.6% and hemoglobin of 2.9 g / dl .
panendoscopy revealed several polypoid lesions with ulceration over the body and the prepyloric area , which were bleeding to touch ( fig .
computed tomography depicted numerous nodules throughout the entire alimentary tract , indicating intestinal polyposis ( fig .
panendoscopy : at least 6 polypoid lesions over gastric body and prepyloric area with ulceration and touch bleeding .
abdominal computed tomography showed multiple nodules within the stomach , small intestine ( including duodenum , jejunum , and ileum ) , and descending colon leading to intussusception . owing to uncorrectable anemia as well as sonography constantly demonstrating intussusceptions ( figs . 3 and 4 ) , laparoscopy - assisted enteroscopic polypectomy and reduction of intussusceptions were performed ( fig .
the pathology report described hamartomatous polyps with elongation , tortuosity , and dilatation of the gastric foveae and intestinal mucosal glands .
the underlying stroma is characterized by broadband smooth muscle fibers , intermingling with the glands ( fig .
technetium-99m - labeled rbc bleeding scan : the possibility of gastrointestinal tract bleeding in the jejunum .
-1 polypectomy and reduction of intussusceptions : ( a ) 2 intussusceptions were found , 1 at 20 cm distal to treitz ligament and another 1 at jejunoileal junction .
( b , d ) numerous polyps were found in whole small bowel , especially in jejunum .
microscopically , hamartomatous polyps composed of elongation , tortuosity , and dilatation of the gastric foveolae and intestinal mucosal glands .
the underlying stroma is characterized by broadband smooth muscle fibers , intermingling with the glands .
thereafter , the patient suffered from multiple episodes of anemia , gastrointestinal hemorrhage , rectal prolapsed polyps , symptomatic colic - colic intussusceptions requiring radiologic reductions , protein - losing enteropathy , and immunodeficiency .
it was our impression that the immunodeficiency was secondary to the remarkable protein - losing enteropathy and malnutrition .
human immunodeficiency virus ( hiv ) infection was excluded due to negative maternal hiv testing during prenatal checkups .
it is not a routine in our institution to conduct another hiv examination before 24 months of age in infants with prior negative virologic tests .
the serology study showed notably hypoglobulinemia with igg of 115 mg / dl and igm of 33 mg / dl .
complement levels were also significantly below the normal limits with c3 of 51 mg / dl and c4 of 7 mg / dl .
intravenous immunoglobulin was administered . in 6 months , polyps recurred and occupied the majority of the gastrointestinal tract .
juvenile polyps are mostly solitary , influencing approximately 1% of preschool and school - aged children .
juvenile polyposis syndrome may extensively affect a large portion of alimentary tract , and be usually related to malignant potential .
it has been known with 3 subtypes : diffuse juvenile polyposis of infancy ( < 6 months of age ) , diffuse juvenile polyposis ( 6 months5 years of age ) , and juvenile polyposis coli ( 515 years of age ) .
histologically , the polyps in juvenile polyposis syndrome are composed of mucous filled , dilated glands that are often associated with inflammatory cell infiltration . unlike those in peutz - jeghers syndrome , smooth muscle proliferation is rarely seen .
nevertheless , the polyps consisted of smooth muscle bands in stroma in this case . peutz - jeghers syndrome was initially suspected . however , the patient had neither relevant family history nor oral pigmented lesions that were highly associated with peutz - jeghers syndrome .
peutz - jeghers syndrome was excluded , whereas the diagnosis of juvenile polyposis syndrome was established .
it is featured with widespread hamartomatous polyps in the entire gastrointestinal tract in infants < 6 months of age .
the initial manifestations of anemia and gastrointestinal bleeding were present at 8 months of age .
we presumed that this patient should be categorized to juvenile polyposis of infancy . with regard to treatment , surgery is the mainstay to remove polyps .
however , repeated operations are usually required because recurrence is not uncommon . in our case ,
redo enteroscopic polypectomy was technically difficult as well as might bear much higher risks , so it was abandoned . as nearly the entire small bowel is involved , resection of affected bowels was less considered because it would lead to prominent intestinal failure .
the decision was made to perform supportive treatment . to the best of our knowledge , | inhaled corticoids ( ics ) made a dramatic breakthrough in the management of asthma in the late eighties resulting in a sharp reduction in morbidity and mortality in the following decades . soon after , the association between ics and long acting 2 agonists ( laba ) soon became the gold standard of maintenance asthma treatment . with the advent of sputum induction
it has become clear that asthma could not be considered as a unique entity but rather a display of several inflammatory phenotypes .
eosinophilic phenotype shows good response to ics while non - eosinophilic , and in particular the neutrophilic phenotype , seems to be more resistant .
severe asthmatics show insufficient asthma control despite ics / laba .
those who are allergic and eosinophilic may benefit from add - on treatment with anti - ige or anti - il-5 .
severe neutrophilic asthma could benefit from maintenance treatment with macrolides while thermoplasty offers some promise to those in whom airway smooth muscle hypertrophy contributes to disease instability . |
the phylum apicomplexa includes a large group of protozoan parasites responsible for a wide range of animal and human diseases . among the human pathogens are plasmodium falciparum and plasmodium vivax , the major causative agents of human malaria , as well as cryptosporidium parvum and toxoplasma gondii , which are particularly pathogenic in immunocompromised patients .
apicomplexa are intracellular obligatory parasites that multiply in a so - called parasitophorous vacuole . although the alterations of the host cell harboring toxoplasma or plasmodium parasites have been extensively documented at the cellular level , still little is known about how the parasite manipulates the host cell at the molecular level , with the notable exception of plasmodium infection of host erythrocytes .
so far , most molecular studies on the host - parasite interface have focused on the role of parasite factors that are secreted by the invading parasite , as well as by the resident intracellular parasite , into the host cell .
these intracellular parasites are expected to profoundly reorganize the host cell for their own needs to ensure safe growth and persistence , and presumably to deploy the most sophisticated mechanisms to this end .
emerging evidence indicates that viruses and bacteria manipulate the microrna ( mirna ) pathways of the host cells they infect .
mirnas are the most abundant class of small , non - coding , single - stranded rnas and are involved in regulating gene expression at the post - transcriptional level . in silico target prediction
suggests that mirnas may control up to 30% of the translation of the human transcriptome . as such
, they govern a variety of fundamental cell functions , including cell proliferation and apoptosis , and are key regulators of cell metabolism . when homeostatic conditions are disrupted - for example , when cells encounter micro - organisms - these regulatory pathways might also contribute to host cell responses / defenses ( i.e. , the inflammatory response ) against the foreign bodies .
for example , cell infection by mammalian viruses might be counteracted by cellular mirnas that target either the virus itself , as in the case of the rhabdoviral vesicular stomatitis virus , or a host factor critical to the virus , as for the lentiviral hiv .
conversely , mirnas can also act in favor of the micro - organism , either when it is pathogen - encoded ( e.g. , mammalian virus - encoded mirnas ) or when the micro - organism subverts host mirnas to its own benefit .
effectors from the bacteria pseudomonas syringae have been recently shown to suppress transcriptional activation of some mirnas generated upon sensing of pamps ( pathogen - associated molecular patterns ) by arabidopsis .
recent data have begun to show how two apicomplexan parasites , cryptosporidium and toxoplasma , are able to target mirnas in the host cell to alter the cellular environment in ways that favor their intracellular development .
cryptosporidium is able to trigger the down - regulation of let-7i ( a mirna with complementarity to toll - like receptor ( tlr)-4 mrna ) in the host cell , leading to the up - regulation of tlr4 , a key pathogen recognition molecule that plays a central role in epithelial innate immunity to cryptosporidium infection .
various studies have further substantiated the ability of cryptosporidium to alter mirna expression in cholangiocytes [ 9 - 11 ] .
it is emerging from these studies that following cryptosporidium infection , specific mirna cluster genes are activated by the binding of the nf-b ( nuclear factor - kappa b ) p65 subunit to their promoter , and that inhibition of these mirnas increases parasite burden .
these results mirror those showing differential alterations in mature mirna expression profiles in primary human fibroblast cells following toxoplasma infection .
zeiner et al . showed that toxoplasma infection specifically increased the transcription of the mir-17/92 loci by two- to three - fold in human fibroblasts .
the effect is apparently a specific response to toxoplasma infection since levels of the mature mir-17/92-derived mirnas remained unchanged upon infection by the closely related parasite neospora caninum .
microarray data comparing the mirna profiles of cells infected by toxoplasma or cryptosporidium or treated with lipopolysaccharide ( lps ) have revealed several important findings .
for example , mir-155/bic is up - regulated upon toxoplasma infection but remains unaffected or is down - regulated when exposed to cryptosporidium or lps , respectively . of note
, mir-155 has an important role in the mammalian immune system , regulating , at least in part , cytokine production .
two other mirnas , mir-198 and mir-320 , are both up - regulated upon toxoplasma infection whereas they are down - regulated after cryptosporidium infection and unaffected after lps stimulation .
these data point to specific modifications of host mirna profiles upon cell infection by apicomplexa parasites .
obviously , any change in the host cell mirna pattern might indicate either a defense mechanism by the cell or a subversion strategy by the parasite , two processes that can be differentiated by evaluating the consequences on parasite growth of disruption or over - expression of the target mirna pathway .
given the propensity of apicomplexan parasites to co - opt cellular pathways and activities for their benefit , it is perhaps not surprising that these parasites could also reshape their cellular environment by reprogramming the host s rna interference machinery .
specific host mirnas could either counteract the intracellular growth of parasites or facilitate it , the two possibilities being not mutually exclusive and depending on the physiological context .
these findings open an exciting opportunity to pursue a deep understanding of how the host proteome can be reprogrammed dynamically and reversibly upon apicomplexa infection .
an additional line of research should explore the upstream regulatory mechanisms , that is , how the parasites directly interfere with rna silencing pathways and , more specifically , the parasite effectors that are involved in the process . as discussed above , in response to cryptosporidium and toxoplasma infections , the expression of specific mirna genes is altered at the transcriptional level .
mirna are generated through the concerted action of multi - subunit complexes that promote the sequential cleavage , export , and loading of mirna into silencing complexes .
an increasing number of reports suggest that , beyond the transcriptional control of genes that code cluster mirnas , each of these steps serves as a potential point of regulation , and therefore adds additional complexity to mirna - dependent gene regulation
. could parasite regulators of host mirnas be ribonucleic acids ? unlike in cryptosporidium and plasmodium species , the toxoplasma genome encodes elaborate rna silencing machinery that generates endogenous small silencing rnas , including specific mirnas .
thus , an attractive hypothesis is that toxoplasma has the potential to secrete its own mirnas to hijack the host cell mirna defense pathway , similar to what some viruses are able to do .
it is known that apicomplexan parasites inject various molecules into the host cell resulting in extensive remodeling of the host cell gene expression profile and metabolic pathways [ 17 - 19 ] .
the expression of host mirnas can also be altered in response to parasite recognition by cell surface tlrs .
both intrinsic and extrinsic acting factors could then interfere with target host mirnas , at any point of the processing of the pri - mirnas and biogenesis of the mirnas - transcription , processing , or export .
we have observed a case of renal involvement complicated by granular corneal dystrophy type ii ( gcd2 ) .
gcd2 , also known as avellino corneal dystrophy ( cd ) , is an autosomal dominant disorder caused by a mutation in the transforming growth factor--induced ( tgfbi ) gene .
this mutation can be found in several distinct autosomal dominant genetically determined cases of cd ; however , it is not known whether this mutation produces other clinical manifestations other than cd .
tgfbi proteins ( tgfbip ) interact with several extracellular matrix ( ecm ) components [ 3 , 4 ] .
we believe that our study was a type of oculorenal syndrome associated with a tgfbi mutation , which remains to be acknowledged .
the following clinical laboratory values were noted : serum urea nitrogen ( bun ) , 14.9 mg / dl ; creatinine ( cre ) , 0.79 mg / dl ; total cholesterol , 189 mg / d ; total protein , 6.4 g / dl ; and albumin , 3.9 g / dl .
the levels of c - reactive protein , immunoglobulins ( ig ) , and total complement , c3 , c4 , and c1q were all normal .
tests for antinuclear antibody , hepatitis b virus surface antigen , hepatitis c virus antibody , and cryoglobulins were all negative .
renal ultrasound and computed tomography revealed normal kidneys . a kidney biopsy , performed using light microscopy , revealed 11 glomeruli , 1 of which was obsolete or sclerosed ( fig 1a ) .
light microscopy did not demonstrate any remarkable changes in the glomeruli ( fig 1b ) .
focal tubular atrophy with dilation of peritubular capillaries and focal infiltration of small round cells were observed .
after 7 years , the patient developed mild hypertension and began taking 4 mg / day of losartan potassium .
the patient 's mild proteinuria ( 11.5 g / g cre ) continued , and her renal function was mildly decreased .
after 10 years , the patient was re - admitted for additional evaluation of proteinuria .
laboratory testing revealed the following : urinary protein level of 1.5 g / day , bun level of 15.0 mg / dl , and cre level of 0.94 mg / dl .
approximately 2 years before her second admission , the patient complained of mild blurred vision and was diagnosed with cd .
slit - lamp examination revealed a large number of gray - white central granular and linear opacities in both eyes ( fig 2 ) ; therefore , we diagnosed her condition as gcd2 .
a second kidney biopsy was performed under light microscopy , revealing 18 glomeruli , 6 of which were obsolete or sclerosed ( fig 3a ) .
segmental double contours of the glomerular capillary walls were also observed ( fig 3c ) .
focal tubular atrophy with mild interstitial inflammation , dilation of peritubular capillaries , and segmental thickening of tubular basement membranes ( tbm ) were observed .
the subendothelial space was widened , and irregularity of the glomerular basement membrane ( gbm ) was segmentally observed .
segmental irregular thinning , basket - waving , duplication , lamellation , and reticulation of gbm and tbm were observed partially and slightly ( fig 3e
immunostaining of the -5 chains of type iv collagen was normal . upon her renal pathological findings
, we assumed the existence of a genetic cause . after obtaining informed consent , we collected dna from the patient .
the genome dna was extracted from the whole blood , and targeted next - generation sequencing of candidate genes for inherited renal diseases was negative ( online suppl .
. real - time polymerase chain reaction using a simple buccal swab ( avellino labs universal test ; alut ) revealed tgfbi heteromutation ( r124h ) .
we speculated that this condition was a novel case of oculorenal syndrome associated with tgfbi mutation .
her 74-year - old father had the same mutation of tgfbi ( r124h ) and was diagnosed with relatively mild gcd2 .
her 77-year - old mother and 26-year - old daughter , however , did not have mutated tgfbi ( r124h ) or gcd2 .
her father had no proteinuria but had a slightly elevated level of urinary n - acetyl--d - glucosaminidase ( 3.0 u / g cre ) ; however , his renal function was normal ( serum cre , 0.79 mg / dl ) .
subsequently , the patient was again treated in our outpatient clinic with 4 mg / day of losartan potassium but with no immunosuppressive agents .
these two conditions may coincidentally coexist ; however , findings demonstrating an association between renal involvement and gcd2 have been presented .
the tgfbip ( also known as ig - h3 , keratoepithelin ) is a 68-kda ecm protein with four evolutionary conserved fasciclin-1 domains and a carboxy - terminal arg - gly - asp sequence .
this protein participates in many physiological processes , including morphogenesis , adhesion / migration , tumorigenesis , angiogenesis , wound healing , and inflammation .
tgfbip is found in ecm of several human tissues and is abundant in the cornea .
mutations of the human tgfbi gene have been linked to several autosomal dominant multiple types of cd , including gcd2 .
almost all cases of gcd2 are caused by tgfbi gene mutations ( 5q31 ) , particularly p.arg124his ( r124h ) . in a previous study ,
tgfbi gene mutation was estimated to have a prevalence of at least 11.5 affected people per 10,000 individuals in korea . according to embryonic expression studies using a mouse knock - out model of tgfbi , schorderet et al .
mutations in adhesion and ecm molecules , such as integrins and laminin-2 , play an important role in the pathogenesis of focal segmental glomerulosclerosis ; however , thus far , the relationship between tgfbi mutation and kidney disease has not been established . in an autopsy patient with tgfbi - related cd , pathologic deposits caused by tgfbip accumulation
were only observed in the cornea and in no other tissue or organ , including the kidney ; however , an electromicroscopic examination was not performed in that report .
tgfbip was present in the capsule and tbm of the developing kidney and was predominantly localized in the epithelial cells of the collecting ducts as well as the distal proximal tubules .
tgfbip is secreted into the extracellular space and may bind to fibronectin , laminin , and type i , ii , and iv collagens as well as integrins [ 12 , 13 ] .
proteomic analysis revealed that tgfbip is a component of glomerular ecm [ 3 , 4 ] , and it exhibits protein - protein interactions between the following ecm proteins : -2 macroglobulin ; -1 , -2 chain type i collagen ; -1 chain type ii collagen ; -1 , -2 , -3 , and -4 chain type iv collagen ; fibronectin ; and fibrillin-1 .
proteoglycans directly bind to tgfbip and affect collagen vi aggregation and possibly the interaction between integrin and collagen vi .
binding allows tgfbip , including ecm proteins , to play an important role in cell - collagen signaling interactions that comprise bm , bone formation , and development as well as cell migration and growth . in our patient ,
various pathological findings of gbm and tbm and widening of the subendothelial space of gbm were observed by electron microscopy .
a negative genetic analysis of well - known monogenic kidney diseases prompted us to consider that tgfbi mutation could affect the bm of the developing kidneys and produce such bm findings .
the pathophysiological mechanisms and the incidence of this condition or genotype - phenotype correlation for tgfbi mutations are not obvious .
if detailed examination of the corneas is not performed , gcd2 may not be observed until middle - age and older . it should be noted that detailed ocular examinations , including cornea assessments , are valuable when diagnosing nephropathy associated with tgfbi mutations .
presently , alut testing is beneficial for laser - assisted in situ keratomileusis to protect patients from accelerated vision loss . as demonstrated with our patient , this test is easy and safe to perform when diagnosing patients with nephropathy associated with tgfbi mutations . in conclusion ,
we have reported the first case of a unique nephropathy complicated by tgfbi - related cd .
tgfbi - related nephropathy remains unknown and is difficult to diagnose without electron microscopic examination .
further reports should be accumulated to determine whether the incidence of renal diseases associated with this mutation may presently be more frequent .
patients with tgfbi - related cds , including gcd2 , should be examined for renal abnormalities .
the authors have no ethical conflicts to disclose , and the patient provided informed consent .
| rna silencing plays an important role in development through the action of micrornas , which fine tune the expression of a large portion of the genome .
it is also very important in innate immune responses , especially in antiviral and antibacterial defenses in plants , insects , and animals .
two recent papers now indicate that apicomplexan parasites display the ability to interfere with host microrna populations . |
a 60-year - old male patient , with a known history of arterial hypertension and type-2 diabetes mellitus , was referred to the outpatient echocardiography laboratory to undergo stress echocardiography because of recent episodes of chest pain occurring on mild exertion .
he was on drug therapy with angiotensin - receptor blocker , thiazide diuretic , nondihydropyridine calcium antagonist , and dronedarone , which had been started because of recent episodes of paroxysmal atrial fibrillation and discontinued 2 days before the stress test .
the patient received a standard protocol of high dipyridamole infusion in two doses ( 0.56 mg / kg and 0.28 mg / kg ) followed by atropine administration ( 1 mg in four 0.25 mg doses ) . at rest , no ischemic abnormalities were observed on electrocardiogram ( ecg ) and transthoracic echocardiography ( tte ) [ figure 1 and videos 13 ] .
after completion of dipyridamole infusion , the patient complained a mild chest discomfort , without any significant ecg changes and any apparent wall - motion abnormalities on tte [ videos 46 ] .
after atropine injection , a worsening of the anginal symptoms combined with a descending st - depression in v3 occurred ; despite the absence of relevant echocardiographic changes , two - dimensional ( 2d ) strain analysis showed lower longitudinal strain of the anterior interventricular septum from rest to peak dose [ figure 2 ] .
afterward , as recommended , aminophylline was administered ; interestingly , a more pronounced st - depression and deep inverted t - waves in v2v4 appeared [ figure 3 ] .
the patient was admitted to the cardiology department and underwent coronary angiography from the radial access , which revealed a long myocardial bridge ( mb ) of the left anterior descending ( lad ) artery with systolic milking [ figures 4 , 5 and videos 79 ] .
the patient was , thereafter , treated with a beta - blocker and discharged without symptoms and ischemic abnormalities on rest ecg .
electrocardiogram at rest two - dimensional strain analysis showing global longitudinal strain at rest ( upper panel ) and at peak dose of dipyridamole ( lower panel ) electrocardiogram after injection of atropine and aminophylline coronary angiography images in diastole ( left ) and systole ( right ) showing myocardial bridge of the left anterior descending artery with systolic milking angiographic images did not change after intracoronary nitroglycerine administration
this case depicts a clinical scenario of positive dipyridamole stress test in a patient affected by mb of the lad .
although mb has been classically deemed a benign coronary artery abnormality , it has been recently related to acute myocardial infarction and sudden cardiac death .
moreover , mb has been also associated with endothelial dysfunction , early atherosclerosis , and coronary vasospasm . in particular ,
a worsening of systolic coronary narrowing of mb has been found when using vasodilator agents , such as nitroglycerine , which are usually not administered in these patients . in our case ,
dipyridamole provoked chest pain associated with minor ecg ischemic changes , such as an only one - lead ( v3 ) st - depression ; these abnormalities worsened after the administration of atropine and later , aminophylline , likely because of drug - induced positive inotropic and chronotropic effects .
atropine might also have determined myocardial ischemia through a paradoxical coronary vasoconstriction induced by acetylcholine as observed in the presence of endothelial dysfunction and mb .
this case has the following interesting implications : ( 1 ) the ability to detect mb also using a vasodilator stress test ( and not only dobutamine echocardiography or exercise test ) , particularly with the addition of atropine injection , ( 2 ) the utility of 2d strain analysis in confirming subtle regional wall - motion abnormalities , and ( 3 ) peculiar diagnostic features , on stress echocardiography , suggesting mb rather than obstructive coronary artery disease . in particular , the observed ecg and echocardiographic ischemic changes , although suggestive of a lad disease , appeared to be late occurring and less extended than usually observed in patients with stable hemodynamic lad obstruction , which should have determined more pronounced ecg and wall - motion abnormalities .
indeed mb , differently from a fixed coronary obstruction , is a dynamic stenotic lesion , requiring a consistent increase in heart rate and myocardial contractility to provoke myocardial ischemia .
hence , mb could have been clearly unmasked only whenever positive inotropic and chronotropic agents had been added to the vasodilator stress caused by dipyridamole .
thus , the finding of worsening ischemic abnormalities after the administration of atropine ad aminophylline , during dipyridamole stress echocardiography , may represent a particular diagnostic feature of mb .
forty - five - year - old male shelter resident , chronic alcoholic , chronic smoker , and marijuana abuser presented to emergency department ( ed ) with complaints of intermittent fever with night sweats and productive - cough for 10 days with yellowish sputum associated with pleuritic chest pain .
he complained of generalized weakness and decreased appetite and claimed to have lost approximately 10 pounds in 2 weeks .
laboratory results showed leukocytosis of 17,000/mm with neutrophil differential of 11,200/mm ( 65% ) with band neutrophils of 10% .
chest radiograph was reported as right middle lobe consolidation but careful observation revealed a foreign body ( fb ) , likely a tooth in the right bronchus intermedius ( fig .
ct scan of chest showed a well calcified fb likely a tooth at the level of carina obstructing right intermedius bronchus and consolidation in right lower lobe indicating post - obstructive pneumonia ( fig .
2 ) . patient was admitted and prescribed intravenous antibiotics targeting gram - negative organisms and anaerobes .
deep conscious sedation was achieved by propofol infusion in the presence of an anesthesiologist and cardiothoracic surgery backup .
bronchoscopy showed tooth in right intermediate bronchus with ~75% occlusion , and purulent secretions from the right main stem ( figs .
the fb was removed successfully with rat tooth forceps ( figs . 7 and 8) .
post bronchoscopy inspection showed right main stem , bronchus intermedius , and right middle and lower lobe bronchi to be well patent .
closer view of the foreign body , identifiable as tooth in right main stem bronchus .
the aspirated tooth removed by rat tooth forceps . upon further questioning , the patient said that he did not remember having aspirated the tooth and hence the duration of aspiration could not be known .
he was discharged home on oral antibiotics a couple of days after the bronchoscopy and counseled to quit alcohol and drugs .
fba is more common in children than adults with about 80% occurring in children aged less than 15 years .
it is the fourth most common cause of unintentional injury deaths in the united states . while choking is a hazard for all ages , choking deaths peaked at age 84 in 2011 as per national safety council injury facts 2015 ( 1 ) .
fba in adults is often overlooked as a potential cause of airway obstruction especially if there is no asphyxiation .
adults with risk factors such as alcoholism , drug abuse , mental retardation , and neuromuscular conditions are predisposed to aspiration .
however , accidental aspiration in adults without the aforementioned risk factors has been described ( 25 ) . in adults ,
most common foreign bodies aspirated are food and broken fragments of teeth ( 68 ) .
ct scan is more sensitive and specific than chest radiograph in diagnosing radiolucent foreign bodies and for characterizing the attenuation of a suspected fb ( 9 ) .
unlike in children , less than half of fbs are lodged in the proximal airways in adults .
most of the fbs are lodged in the right bronchial tree , whereas in children no significant difference was seen between right and left bronchial tree ( 6 ) .
bronchoscopy for the removal of fb was introduced by gustav killian , an otolaryngologist in 1897 .
animal studies performed by zavala and rhodes ( 11 ) showed that fbr could be used to retrieve different kinds of fbs by using grasping forceps through the bronchoscope .
fbr is considered as the diagnostic test of choice for initial diagnosis of fb in adults .
the advantages of fbr over rigid bronchoscopy are that it can be performed under local anesthesia , visualization of smaller peripheral airway is better with relatively easier manipulation , and can be performed in patients with deformities of c - spine and pharynx .
it is also a relatively easy and safer procedure in experienced - hands ( 10 , 12 ) .
rigid bronchoscopy is recommended if fbr fails , if the fb is centrally located , if firmly embedded in scar tissue , and for removal of sharp objects which require maneuvering to minimize mucosal trauma ( 13 ) .
there have been several case series which have reported successful removal of foreign bodies by fbr .
cunanan ( 12 ) reported a success rate of 89% in using fbr for the removal of fb in 300 mentally retarded and physically handicapped patients .
they reported a decrease in mortality from 12 to 1% with the use of fbr over rigid bronchoscope .
they , however , attribute to the increased mortality from rigid bronchoscopy to complications of general anesthesia in patients with comorbidities .
limper and prakash had a success rate of 60% with fbr , whereas rigid bronchoscopy showed a success rate of 98% . in their experience , fbr is advantageous in distally lodged fb , in cases with cervical instability , mechanically ventilated patients , and also in removal of small fbs , which can be securely grasped with a fiberoptic bronchoscope ( 14 ) .
( 15 ) report a success rate of 97% for fb removal via fbr with low mortality and morbidity .
they also reported that granulation tissue can be removed without much bleeding ; thus , removal of fb can be achieved in a repeat fbr a week later .
( 13 ) indicate that fbr can be used to remove foreign bodies especially if they are small and peripheral and that fbr may be superior to rigid bronchoscopy for grasping tiny and far - reaching foreign bodies .
rigid bronchoscopy is recommended if fbr fails and especially if the fb is large and located in central bronchi or trachea .
however , in children who may have central , asphyxiating foreign bodies , the rigid bronchoscope under general anesthesia is preferentially indicated .
team - based approach with designated roles for team members improves the chances of a successful procedure .
anesthesia and ct surgery backup is essential . team - based analysis of the nature , location of the fb , and appropriate accessory instruments is equally necessary .
steroids can be given to minimize inflammatory changes as they may lead to difficulty in fb extraction .
orotracheal approach is preferred over nasotracheal , as it avoids fb from being lost in the nasal passages if accidently dislodged .
care should be taken to keep fb in the center of visual field and to avoid pushing fb distally into the airways .
once fb has been secured , bronchoscope , accessory instrument , and the secured fb are all withdrawn from the airway simultaneously .
rare complications of fbr are likelihood of asphyxiation from losing the fb in the subglottic area and hemoptysis ( 10 ) .
another approach is to reinsert flexible bronchoscope to push the fb into more peripheral airway thus resolving the central airway obstruction . in case of hemoptysis ,
airway can be better managed by rigid bronchoscope , but this complication is very rare if performed by trained operators .
incidence of massive hemoptysis even in patients with significant granulation tissue around the fb is extremely low ( 15 ) .
organic materials can cause severe inflammation in a short period of time and tend to absorb water with development of airway obstruction relatively earlier .
in contrast , inorganic fbs are inert , and so patients might be asymptomatic for a prolonged period ( 16 ) . grasping forceps , baskets , magnet extractors ,
yag laser , and cryoprobes are available as accessories for fb removal via flexible bronchoscope ( 10 ) .
rat tooth forceps is a type of a grasping forceps , which is particularly useful for removing hard , flat , or thin organic or inorganic foreign bodies .
these are used primarily when the fb is hard as a firm grip can be achieved .
these can not be used for the retrieval of organic friable objects which tend to fracture due to the firmness of the grip provided by these forceps ( 10 ) .
zero - tip basket is used in urological procedures like retrieval of ureteral stones . the tipless design allows close approximation to fb .
they are used to retrieve fbs lodged in distal bronchi and mobile fbs ( 16 , 17 ) .
fish net basket , a modified polypectomy snare , is useful in removing bulky fbs ( 10 ) .
magnet extractors contain flexible probe tipped with a magnet , useful for retrieval of metallic fbs ( 10 ) .
fbs embedded in granulation tissue can be removed by yag laser , which vaporizes the surrounding granulation tissue .
cryoprobe is used to freeze friable organic and small inorganic materials . freezing the foreign bodies
high index of suspicion is needed especially for high - risk patients presenting with compatible symptoms .
fbr is the initial diagnostic and therapeutic procedure recommended with low complication rates in experienced hands .
rigid bronchoscopy is performed if the flexible bronchoscopy fails or if the fb is large and centrally located , in case of significant hemoptysis and if significant granulation tissue is present with a fb which is deeply embedded .
| a 60-year - old male patient was submitted to dipyridamole - atropine stress echocardiography ( dse ) for chest pain during exertion . at rest , no electrocardiographic ( ecg ) and transthoracic echocardiographic ( tte ) abnormalities were observed .
after dipyridamole infusion , the patient complained a mild chest discomfort , without ecg changes and tte wall - motion abnormalities .
subsequently , worsening of the anginal symptoms combined with descending st - depression and t - negative waves occurred after atropine and unexpectedly , aminophylline administration .
coronary angiography was performed showing a myocardial bridge ( mb ) of the left anterior descending artery .
the occurrence , during dse , of worsening ischemic abnormalities after atropine and aminophylline administration may be a particular diagnostic feature of mb . |
inflammatory bowel disease ( ibd ) is a common diagnostic and therapeutic problem , affecting patients at increasingly young ages .
a standard diagnostic method includes macroscopic assessment of the intestinal mucosa during colonoscopy and histopathological evaluation of the obtained biopsies . according to porto criteria ,
the diagnosis of ibd is based on the clinical presentation , intestinal endoscopic and histological features , laboratory tests , and radiological examination .
recently , reports on the usefulness of a noninvasive examination , that is , faecal calprotectin measurement in ibd diagnosis , have been published .
evaluation of faecal calprotectin ( fc ) seems to be a screening test selecting patients requiring further invasive diagnostics .
furthermore , there have been reports on using calprotectin assays in monitoring the treatment of crohn 's disease and ulcerative colitis in adults and children [ 14 ] . to our knowledge ,
there are no published data on the usefulness of faecal calprotectin assays in the diagnosis of other atypical forms of bowel inflammation , such as eosinophilic , lymphocytic , or nonspecific colitis .
the aim of the study is to assess the usefulness of faecal calprotectin measurement in children with various types of ibd and to evaluate fc concentration in children with crohn 's disease and ulcerative colitis in relation to disease activity .
91 patients were included in the analysis , including 49 boys ( 53.85% ) and 42 girls ( 46.15% ) , ranging from 6 to 18 years of age ( the mean age was 13.38 years ) .
the study group comprised 71 children with various types of ibd , who were subsequently divided into six subgroups : b124 ( 33.8% ) children with crohn 's disease ( cd ) , b216 ( 22.5% ) with ulcerative colitis ( uc ) , b37 ( 9.8% ) with eosinophilic colitis ( ec ) , b48 ( 11.26% ) with lymphocytic colitis ( lc ) , and b516 ( 22.5% ) with nonspecific colitis ( nc , colitis indeterminata
the control group ( k ) comprised 20 healthy , age- and sex - matched subjects .
patients with ibd underwent following procedures : anamnesis , physical examination , laboratory tests ( inflammatory state markers , biochemical parameters of liver , pancreas , and kidney function , sweat test , coprological tests , and immunoassays ) , diagnostic imaging ( abdominal ultrasound ) , and endoscopy with histopathological evaluation . in all patients ,
faecal calprotectin was measured by means of elisa method , using phical test ( calpro ) .
calprotectin concentrations ranging from 0 to 50 mg / kg were considered to be normal reference values .
the results were evaluated by using the following tests : komogorow - smirnow , t - student , u mann - whitney , fisher , and yates ; the analysis of correlation was based on the spearman 's rank correlation coefficient . a p value of < 0.05 was considered statistically significant .
all patients and their caregivers gave informed consent to participate in the study , which was approved by the bioethics committee of the medical university of silesia in katowice ( consent no .
a statistically significant increase in the mean concentrations of faecal calprotectin was observed in the group of children with cd and uc , as compared to the control group .
concentrations of fc were also higher in children with uc than in patients with cd .
faecal calprotectin concentrations were within the normal limits in patients with eosinophilic , lymphocytic , and nonspecific colitis , similarly to the healthy subjects ( table 1 , figure 1 ) in children with cd , faecal calprotectin concentrations positively correlated with the disease severity assessed according to the pcdai scale . in patients suffering from uc , faecal calprotectin
also positively correlated with the truelove - witts scale and the rachmilewitz endoscopic index ( figures 2 , 3 , and 4 ) . a significant increase in faecal calprotectin concentrations
was observed in children suffering from cd , with lesions located in both small and large intestine , and in patients presenting with inflammatory changes in 5 or more sections of the intestine ( figure 5 ) .
faecal calprotectin is a promising , noninvasive screening method for diagnosing patients suffering from gastrointestinal disorders , such as abdominal pain or diarrhea , which are also typical for ibd [ 57 ] .
so far , many authors have considered calprotectin as a useful marker in differential diagnosis of ibd and functional gastrointestinal disorders ( e.g. , irritable bowel syndrome ) [ 8 , 9 ] .
many authors evaluated faecal calprotectin concentrations in patients with suspected inflammatory process of the large intestine .
demonstrated in the group of paediatric patients that this assay is characterised by 95% sensitivity and 93% specificity , and high calprotectin concentrations show strong positive correlation with the presence of inflammatory lesions in the large intestine . according to these authors
so far , only limited studies , based on very small groups of patients , evaluated faecal calprotectin concentrations in patients with other types of bowel inflammations less common than cd and uc , such as lymphocytic , eosinophilic , and nonspecific colitis , which seem to be a considerable clinical problem in everyday pediatric practice . in studies conducted by bunn et al . , two children with nonspecific colitis and 3 children with allergic colitis were included in the analysis . in both cases ,
calprotectin concentrations were found to be within the normal ranges . in our study involving 31 children : 7 with eosinophilic colitis , 8 with lymphocytic colitis , and 16 with nonspecific colitis , respectively ,
these results support the hypothesis that in the aforementioned types of bowel inflammation , histopathological examination does not reveal infiltrations of neutrophil cells , whose cytosols contain calprotectin . therefore , its concentration in faeces is directly related to the number of neutrophils in the large intestine lumen [ 11 , 12 ] . in paediatric patients , the diagnosis of nonspecific colitis ( indeterminata colitis ) remains unchanged in approximately 36% . over time in some patients , the diagnosis may be changed into ulcerative colitis ( in approximately 3372.5% ) or crohn 's disease ( in approximately 1727.5% ) . in our group of 6 cd patients , previously diagnosed conditions included single cases of ulcerative colitis , lymphocytic colitis , and nonspecific colitis , whereas eosinophilic colitis was found in 3 subjects .
increased calprotectin concentrations may be useful when making a decision on extending diagnostic procedures in patients with less frequent types of bowel inflammation . in our study ,
the mean calprotectin concentration in the examined patients with ibd was higher than it was observed by bremner et al . ; however , patients in remission were also enrolled in the latter research . in the presented material , a significant correlation was demonstrated between calprotectin activity and the disease severity assessed by the modified pcdai scale for cd and the modified truelove - witts scale and the rachmilewitz endoscopic index for uc .
, demonstrating a strong positive correlation between calprotectin concentrations and the disease severity according to the modified truelove - witts scale only in uc . analyses performed in children assessed a correlation between fc concentrations and intensity of macroscopic and microscopic inflammatory lesions in the large intestine , observed in the course of ibd .
studies carried out by fagerberg et al . included 39 children with ibd , in whom calprotectin concentrations strongly correlated with intensity and extent of micro- and macroscopic abnormalities .
norwegian researchers also confirmed such a correlation and , moreover , suggested that intensity of inflammatory lesions rather than their extent influences faecal calprotectin concentration .
this suggestion can be supported by data obtained from our study , indicating that in patients with uc calprotectin concentration depends on disease activity and not on the extent of lesions , in contrast to patients suffering from cd . in the latter group , calprotectin concentrations correlated with the disease activity and
were significantly higher in children with inflammatory lesions present in both small and large intestine and located in 5 or more sections of the large intestine .
these results support conclusions from the studies on clinical expression of the disease , which demonstrated that in children and adolescents the disease is more severe and cd lesions are located in the small intestine [ 17 , 18 ] . so far
faecal calprotectin measurement seems a promising test to evaluate disease activity and a tool for monitoring ibd treatment . in everyday practice ,
a disadvantage of this method is its low specificity , which is connected with the fact that in patients with increased calprotectin concentration many organic diseases should be excluded .
it seems that measurement of faecal calprotectin concentration can be a useful , safe , and noninvasive test in children suspected for ibd , since it is found to be increased in children with cd and uc as compared to patients with other inflammatory diseases ( eosinophilic , lymphocytic , and nonspecific colitis ) and also in the reference to healthy subjects . when the faecal calprotectin concentration is increased in children with less common types of bowel inflammation , a further follow - up of such patients
faecal calprotectin concentration correlates positively with the disease severity in cd and uc patients ; thus , it may be useful when choosing or modifying the appropriate treatment regimen . giant cell tumour is the commonest benign bone tumour arising at the epiphyseometaphyseal regions of long bones . around the knee
there are very few similar cases reported worldwide and it is the purpose of this report to describe the management of such a case .
a 17 year old girl presented with swelling of ankle and pain while walking for six months .
radiographs were suggestive of a giant cell tumour , computerised tomography revealed cortical break , en block resection was done with ipsilateral proximal fibula used in reconstruction of ankle mortise .
giant cell tumour of long bones are common but those involving the distal fibula are exceedingly rare .
the management of such tumours with high recurrence rates can be easily accomplished by en block resection and reconstruction of the ankle mortise with proximal fibula ensuring good range of motion of the joint post operatively .
first described by sir astley cooper in the year 1818 , giant cell tumour of bone or osteoclastoma is the commonest benign bone tumour encountered by an orthopaedic surgeon .
it is characterised radiographically as a lytic lesion occurring in the ends of bones and has well known propensity for local recurrence after surgical management .
current treatment modalities including a meticulous curettage with extension of tumour removal using high speed burrs and adjuvant local therapy has significantly lowered the recurrence rates to less than 10% from 60% in the past with curettage alone .
the commonest age is the 3rd or the 4th decade with a slight female predominance .
the other less common infrequent sites are sacrum , distal tibia , proximal humerus , proximal femur and proximal fibula .
involvement of distal fibula by benign aggressive and malignant tumors usually necessitates resection of the involved segment of fibula .
the incidence of giant cell tumour of distal fibula was found to be less than 1% of 1182 cases .
schajowicz , in his series of 362 cases has reported only a single case affecting the lower end of the fibula ( 0.28% ) .
a seventeen year old girl presented with swelling around the right ankle for six months associated with pain while walking and restriction on squatting .
the swelling was six by four by two centimetres in size , firm to hard in consistency , no tenderness on deep palpation .
[ fig 1 ] clinical picture showing swelling and radiographs showing expansile lesion with soap bubble appearance .
anteroposterior and lateral radiographs were taken which showed single epiphyseal expansile lesion with soap bubble appearance .
magnetic resonance imaging could not be done as the facility was not available then in our government hospital and patient s financial background prevented us getting an imaging from private centres .
all routine haematological investigations were found to be normal and chest radiograph was also found to be normal .
an excisional biopsy was planned with reconstruction using the proximal end of the ipsilateral fibula . under pneumatic tourniquet without exsanguination an en bloc excision of the lateral malleolus with lower third of the fibula
the level of resection of distal fibula was determined by the computerised tomography , clinical intra operative findings and by pre operative radiographs .
the proximal fibula was reversed with head of fibula incorporating into the ankle mortise and fixed to the remaining fibula using plate and screws .
meticulous haemostasis was achieved after release of the tourniquet , and the wounds were closed in layers .
patient kept non weight bearing for three months and full weight bearing at six months after the removal of screws .
patient was followed up and at the end of one year patient had full range of motions with mild restriction of dorsiflexion of the affected ankle [ figs 3 ] .
b- fixation of the reversed proximal end of fibula and incorporation into the ankle mortise .
d - photomicrograph of the specimen showing multinucleate giant cells suggestive of giant cell tumor six month follow up showing good radiological union ( a ) and clinical photograph showing good dorsiflexion and plantar flexion ( b , c ) .
the proximal fibula can be sacrificed for the purposes of reconstruction as is recommended for lower end fibula and distal radius .
giant cell tumour of the bone has an unpredictable behaviour , not always related to radiographic or histological appearance .
many earlier studies had shown very high local recurrence rates after curettage and bone grafting . the use of modern imaging techniques and extended curettage through the use of power burrs and local adjuvants have improved outcome with reduced recurrence rates .
phenol , liquid nitrogen , bone cement , hydrogen peroxide , zinc chloride and more recently , argon beam cauterization have been employed as local adjuvants .
chemical or physical agents work by inducing an additional circumferential area of necrosis to extend the curettage . in distal fibular resection without reconstruction ,
soft - tissue reinforcement , even when it is possible , can not fully compensate for the loss of stability .
resection of the lateral ankle can cause a varus instability or a collapse into valgus .
this technique of ankle resection and reconstruction has provided good oncological and functional results and recommended in young active patients requiring resection of distal fibula .
giant cell tumour of long bones are common but those involving the distal fibula are exceedingly rare .
the management of such tumours with high recurrence rates can be easily accomplished by en block resection and reconstruction of the ankle mortise with proximal fibula ensuring good range of motion of the joint post operatively .
resection arthrodesis which was the method primarily employed for bone tumours involving ankle can now be replaced with ankle reconstruction .
distal fibula gct being an extremely rare entity and its management not been described , reconstruction of ankle with proximal giant cell tumour of distal fibula are extremely rare and such benign tumours with high recurrence rates with the evidence of medial cortical break should be managed by an en block resection and reconstruction of the ankle mortise and the preferable method would be by the usage of proximal fibula graft .
this method produced no recurrence and ensured good range of motions and can effectively replace resection arthrodesis as management in cases which require resection of lateral malleolus . | introduction .
the aim of the study was to assess the usefulness of the fc measurement in children with various types of ibd and relation to the disease activity .
patients and methods .
91 patients ( 49 boys : 53.85% and 42 girls : 46.15% , mean age : 13.38 years , range 618 years ) were included in the analysis .
patients were divided into the groups : b124 children with cd , b216 patients with uc , and a group comprising 31 children with other types of colitis ; the control group ( k ) comprised 20 healthy children .
fc was assayed by elisa method , using phical test ( calpro ) .
results .
the mean faecal calprotectin concentrations were higher in children with cd and uc as compared to healthy controls , patients with eosinophilic , lymphocytic , and nonspecific colitis .
a positive correlation was observed between fc concentrations and the disease activity ( the pcdai scale , the truelove - witts scale , and the endoscopic rachmilewitz index ) .
conclusion .
it seems that the fc concentrations can be a useful , safe , and noninvasive test in children suspected for ibd , since fc concentration is higher in children with cd and uc than in patients with other inflammatory diseases . |
acute promyelocytic leukemia ( apl ) is one of the most characteristic subtypes of aml in which abnormal promyelocytes predominate within peripheral blood or bone marrow .
also , t(15;17)(q22;q21 ) shows a characteristic chromosomal translocation in apl , observable in 70 - 90% of apl patients .
owing to all trans - retinoic acid ( atra ) combined with chemotherapy , apl has one of the highest cure rates of all types of aml .
seventy to eighty percent of newly diagnosed apl patients with the pml - rara rearrangement are cured or under long - term remission , yet some of them have a poor prognosis [ 2 - 5 ] . because cytogenetics is one of the most powerful prognostic factors for the outcome of aml , cytogenetic abnormalities can cause change in treatment response , relapse , and clinicopathological characteristics .
incidence of secondary cytogenetic abnormalities has been observed in ~40% of apl cases , but their prognostic significance is still unclear [ 5 - 7 ] .
about 1% of the reported secondary cytogenetic abnormalities in apl patients are ider(17)(q10)t(15;17)(q22;q12 ) , an infrequent type of additional recurrent chromosomal abnormality , according to a recent study . however , ider(17)(q10)t(15;17 ) associated with the pml - rara rearrangement in microgranular variant apl is even more rare . as far as we know ,
only 2 cases of the ider(17)(q10)t(15;17 ) abnormality in microgranular apl have been previously reported [ 8 , 9 ] . here
, we describe an unusual microgranular apl case associated with ider(17)(q10)t(15;17 ) , identified by both conventional cytogenetics and fish analyses at the initial diagnosis .
a 59-yr - old woman who had previously been diagnosed with cerebral infarction was brought to our hospital due to right side weakness in november 2007 .
the initial complete blood count showed pancytopenia , hb level of 9.9 g / dl ( reference range 12 - 16 g / dl ) , platelet count of 83,000/l ( reference range 150,000 - 350,000/l ) , and white blood cell count of 1,000/l ( reference range 4,000 - 10,000/l ) .
bone marrow aspiration showed a hypercellular marrow replaced by increased promyelocytes with a paucity or absence of granules , accounting for 36% of all nucleated cells ( fig .
the results of special staining of bone marrow specimens were as follows : myeloperoxidase , positive ; periodic acid schiff , negative ; nonspecific esterase , negative .
flow cytometric analysis was conducted and showed that the blasts were positive for cd13 ( 91.1% ) , cd33 ( 83.9% ) , cd117 ( 59.2% ) , cd2 ( 43.9% ) , and cd45 ( 25.4% ) , and negative for hla - dr ( 3.4% ) , cd3 ( 1.3% ) , cd7 ( 0.6% ) , cd10 ( 1.8% ) , cd14 ( 2.4% ) , cd19 ( 5.1% ) , cd34 ( 1.4% ) , cd41 ( 2.9% ) , cd56 ( 1.2% ) , and tdt ( 0.9% ) .
fish signals from pml - rara probes ( abbott molecular / vysis , des plaines , il , usa ) yielded the results of nuc ish(pml , rara)4(rara con pml3)[24/138 ] , ( pml , rara)6(rara con pml5)[14/138 ] , ( pml , rara)3(rara con pml2)[13/138 ] , consistent with the abnormal fusion signal patterns seen in 37% of the nuclei examined ( fig .
the patient was diagnosed with apl and treated with induction chemotherapy consisting of daunorubicin , cytosine arabinoside , and atra . after completing induction chemotherapy ,
follow up bone marrow examination in january 2008 showed no evidence of morphologically visible residual leukemia .
the concurrent karyotype analysis result was 46,xx in all analyzed cells ; and pml - rara fish showed " nuc ish ( pml , rara)2 " in which the abnormal signal pattern was not observed .
there was no evidence of a pml - rara fusion gene in the reverse transcriptase - pcr ( rt - pcr ) analysis .
as indicated by follow - up bone marrow biopsies conducted until september 2008 , the patient remained in complete remission . during this period
, the rt - pcr analysis did not show any signs of the pml - rara fusion gene while other cytogenetic studies also indicated normal findings .
apl is a distinct subtype of aml and constitutes about 5 - 8% of all cases of aml diagnosis . according to the 2008 who classification
, apl can be diagnosed when there is a t(15;17 ) or a pml - rara rearrangement , even if peripheral blood or bone marrow studies show less than 20% promyelocytes . as recently reported by manola et al . and
our study group , the ider(17)(q10)t(15;17 ) , an isochromosomal abnormality that occurs on the long arm of ider(17)t(15;17 ) after reciprocal translocation of t(15;17 ) , is a relatively rare type of an additional recurrent cytogenetic abnormality that has been reported in 62 apl patients worldwide [ 8 - 13 ] . according to these studies , the influence of ider(17)(q10)t(15 ; 17 ) on the prognosis of adult apl patients
indeed , 4 previously reported apl cases in children were all related to poor prognosis [ 8 , 13 - 15 ] , inferring that a more close and careful interpretation is necessary for childhood apl cases .
what is interesting is that so far , reports of ider(17)(q10)t(15;17 ) from microgranular variant ( aml - m3v ) type are extremely rare .
out of 62 total cases , information on apl morphology type were available in 42 cases , and most of these cases ( 40/42 ) were of the hypergranular apl type , except for 2 cases that clearly indicated aml - m3v ( table 1 ) [ 8 , 9 ] .
therefore , further research is required to determine whether ider(17)(q10 ) and aml - m3v have a low association , and more careful observation should be conducted to prevent underestimating aml - m3v patients among ider(17)(q10)t(15;17 ) cases .
furthermore , double ider(17 ) ( q10)t(15;17 ) is so rare in the international public databases that only 2 cases of apl patients indicating double ider(17)(q10)t(15;17 ) chromosomal abnormalities have been reported ( table 2 ) [ 16 , 17 ] . in double ider(17)(q10)t(15;17 ) , a gene dosage effect is observed owing to chromosomal abnormalities such as the pml - rara fusion gene on chromosome 17 or the quadruplication of der(17q ) . in addition , since the deletion of the tumor suppressor gene tp53 occurs by the loss of 17p , further research is necessary to resolve the adverse prognosis of the apl group related to such copy number variations .
owing to the limited amount of clinical data in the literature , the relatedness between double ider(17)(q10)t(15;17 ) and an adverse prognosis is still unclear [ 16 , 17 ] . in the case of our patient
, it was hard to determine a strong association between the additional genetic aberration and prognosis because of the small clonal size of the " double ider(17)(q10)t(15;17 ) " abnormality .
nevertheless , at least from a diagnostic perspective and as indicated in the authors ' recent studies [ 13 , 18 ] , minimal residual disease detection using such multiple abnormal fusion signals through the pml - rara fish analysis in apl patients associated with ider(17)(q10)t(15;17 ) or double ider(17)(q10)t(15;17 ) would be considered to be a useful follow - up marker in clinical laboratories or hospitals .
additional study would contribute toward a better understanding of the influence of ider(17)(q10)t(15;17 ) on the prognosis , survival , and treatment response of such apl cases in adults or children . to the best of our knowledge
, however , this is the third case report of microgranular variant apl associated with ider(17)(q10)t(15;17 ) . administrative qof data describing performance of family practices under the program were analyzed for the years 20042008 ( 3 ) .
data for each family practice included the number of registered diabetic subjects , the proportion of eligible subjects who achieved the targets , and the proportion of diabetic subjects excluded from evaluation of each target as exceptions .
exceptions arise because practices are permitted to identify some individuals as ineligible for evaluation if the target is regarded as clinically inappropriate ( 4 ) .
the targets included in this report were the percent of diabetic subjects with the last a1c 7.5% , with last blood pressure 145/85 mmhg , or with the last measured total cholesterol 5 mmol / l .
we estimated the total number of registered diabetic subjects , the total number excluded as ineligible , and the number ( and percent ) of subjects who achieved the target after allowing for exclusions .
the linear association between outcomes and year was estimated using robust standard errors to allow for repeated measures .
data were analyzed for family practices in england that remained independent and had more than 750 registered patients or more than 500 patients per doctor , in the study year .
data were analyzed for 8,423 practices in 20042005 , 8,264 in 20052006 , 8,192 in 20062007 , and 8,255 in 20072008 , representing 98% of all practices .
the median number of registered diabetic subjects per practice increased from 181 ( interquartile range [ iqr ] 107284 ) in 20042005 to 218 ( iqr 130342 ) in 20072008 ( table 1 ) .
the estimated resident population of england is 51 million ( 5 ) , giving an overall prevalence of 4% .
the median practice - specific proportion of diabetic subjects declared ineligible for the a1c target was 9.4% in 20042005 but declined to 8.7% in 20072008 ( p < 0.001 ) .
the median proportion excluded for the blood pressure target was 6.3% in 20042005 declining to 5.7% in 20072008 ( p < 0.001 ) and for cholesterol was 9.0% in 20042005 declining to 8.4% in 20072008 ( p < 0.001 ) .
the median practice - specific proportion achieving the a1c target of 7.5% increased from 59.1% in 20042005 to 66.7% in 20072008 ( table 1 ) .
the proportion achieving the blood pressure target of 145/85 mmhg increased from 70.9% in 20042005 to 80.2% in 20072008 .
the proportion achieving the cholesterol target of 5 mmol / l increased from 72.6% in 20042005 to 83.6% in 20072008 . the estimated annual increase in percent of diabetes subjects achieving targets was 3.03% ( 95% ci 2.953.10 ; p
< 0.001 ) for the a1c target , 3.26% ( 3.183.34 ; p < 0.001 ) for the blood pressure target , and 3.99% ( 3.924.07 ; p < 0.001 ) for the cholesterol target .
the total number of diabetic subjects in england achieving the a1c target , after allowing for exclusions from assessment , increased by 341,173 between 20042005 and 20072008 , representing 16% of diabetic subjects registered in 20072008 . over the same period ,
the number achieving the blood pressure target increased by 453,785 ( 22% of 20072008 registrations ) , and the number achieving the cholesterol target increased by 452,347 ( 22% of 20072008 registrations ) .
practices were classified as low performing if they achieved less than the 25th centile for the a1c target across all practices in 20062007 .
there were 57% of practices classified as low performing in 20042005 . among the 10 english regions , 69.9% of practices were low performing in london compared with
the overall proportion of low - performing practices declined to 47.4% in 20052006 , 25.0% in 20062007 , and 26.0% in 20072008 . in 20072008 , the proportion of low - performing practices ranged from 37.5% in london to 11.6% in the north east .
in the u.k . , the care of subjects with type 2 diabetes is increasingly undertaken outside of specialist clinics by family physicians and practice nurses in primary care . this has led to concerns that some patients may experience poor - quality care ( 6 ) . the new national contract for family practices introduced in 2004 appears to have achieved favorable results in its initial year ( 4,7 ) and may have contributed to reducing socioeconomic inequalities in care ( 8,9 ) .
lower - performing practices have shown the greatest improvements , and regional variations in care have reduced .
there has been a substantial increase in the proportion of all diabetic subjects achieving intermediate outcome targets . in our previous report ( 7 ) , we analyzed clinical data from individual patient records for 26 practices during the period of 20002003 that gave results consistent with administrative data from the qof .
two other reports , including data from the first or second years of qof , suggest that qof data are consistent with audits of individual patient records ( 10,11 ) . in a single group study , without any control practices ,
it is not possible to conclude that pay - for - performance incentives caused the observed changes .
there was already evidence of improving quality of care before the introduction of qof ( 7,12 ) .
the qof targets are designed for audit rather than best practice , and practitioners may be utilizing clinical practice guidelines that recommend more stringent targets .
recommendations for a widespread use of statins were introduced in many countries at the start of this period , leading to improvements even in the absence of pay - for - performance .
the greater improvement of low - performing practices may , in part , be accounted for by a ceiling effect , which restricted the potential improvement in high - performing practices .
we caution that it is not clear that proposed benefits from pay - for - performance would be observed if this model is adopted in systems with different organizational arrangements and models of practitioner remuneration . | we present a rare case of microgranular variant acute promyelocytic leukemia ( apl ) associated with ider(17)(q10)t(15;17)(q22;q12 ) of an old - age patient .
the initial chromosome study showed a 46,xx , del(6)(?q21q25),der(15)t(15;17)(q22;q12),ider(17)(q10)t(15;17)[10]/47,sl,+ider(17)(q10)t(15;17)[3]/46,xx[16 ] .
fish signals from a dual color dual fusion translocation pml - rara probe were consistent with the results of conventional cytogenetics .
because of the rarity of ider(17)(q10)t(15;17 ) in microgranular apl , further studies on both gene dosage effect of this chromosomal abnormality and the influence of ider(17)(q10)t(15;17 ) on clinical features such as prognosis , survival , and treatment response of apl cases are recommended . |
the transversus abdominis plane ( tap ) block provides effective post - operative analgesia after abdominal surgery .
rafi first described tap block , who injected local anesthetic through the ilio - lumbar triangle of petit within the tap between the internal oblique muscle and transversus abdominis muscle utilizing the double - loss of resistance technique .
virtually , the ultrasound - guided tap block is highly effective and easy technique for rendering analgesia for post anterior abdominal incision as supported by literature ; however , it can also be used as a sole anesthetic technique for abdominal surgeries where the autonomic innervation is partly or not involved .
a 67-year - old woman , 55 kg was admitted to hospital with a 3-day history of abdominal pain , nausea and vomiting .
she was diagnosed to have chronic obstructive pulmonary disease ( copd ) 5 years ago and had four episodes of prior hospitalization secondary to acute exacerbation .
she often required nebulisers and had an exercise tolerance of approximately 100 yards . in the week prior to admission she had received oral antibiotics and steroids for an infective exacerbation of copd . on admission to the hospital she had signs of peritonitis and her abdomen
examination revealed reduced bi - basal air entry with crepitations all over the chest and chest x - ray showed consolidation of the right middle and lower lobe .
her arterial blood gas revealed type i respiratory failure ( fio2 0.60 , ph 7.37 , po258 mmhg , pco245 mmhg ) .
intravenous acetaminophen was administered for pain relief , but despite this she could not cough or take deep inspiration due to ongoing pain .
she was categorized as american society of anesthesiologists ( asa ) physical status grade iv / e and was planned for emergency laparotomy .
we planned to avoid general , epidural or spinal anesthesia , so we opted for tap block under ultrasound guidance as a sole anesthetic technique .
after proper explanation to the patient about the technique of tap block , she was taken to operation theatre table and monitors attached .
hence oxygen supplementation was carried out with venturi mask with oxygen flow at 6 l / min .
saturation improved to 92% . after securing an iv access with 18 g ( gauge ) needle on the right dorsum of the hand ,
she was sedated with intravenous dexmedetomidine infusion at 4 mcg / kg / min to make her comfortable , cooperative , and pain free for performing the block .
then she was laid supine with the anterolateral abdominal wall exposed bilaterally from the iliac crest to the sub - costal margins and scrubbed aseptically .
the block was performed using an aseptic ultrasound guided in - plane technique ( s - nerve sonosite , hfl38 ( company brand name of the 6.0 to 13.0 mhz linear probe ) probe , 100 mm sonoplex needle ) . when the needle tip position is within the tap neuro - fascial plane a mixture of 20 ml of 0.25% bupivacaine , 20 ml of 1% lidocaine and 0.2 mg adrenaline
a remarkably good clinical effect was achieved within 30 min with almost complete resolution of pain .
she made a slow post - operative recovery to be discharged from hospital 14 days after admission .
the use of tap blocks for control of post - operative analgesia has been described following a variety of abdominal operations such as appendectomy , hernia repair , caesarean section , abdominal hysterectomy , and prostatectomy etc .
bilateral blocks can be given for midline incisions or laparoscopic surgery with careful safe dosing .
to our knowledge , the use of ultrasound - guided bilateral tap for a sole anesthetic technique for upper abdominal surgery in a very high - risk patient has been rarely reported . the skin , muscles and parietal peritoneum of the anterior abdominal wall are innervated by the lower six thoracic nerves and the first lumbar nerve .
after leaving the respective intervertebral foramina the anterior rami ( sensory afferents ) of these nerves course around the transverse process , then pierce the musculature of the lateral abdominal wall to course through a muscle neurovascular plane superficial to the transversus abdominis . in the midaxillary line ,
the sensory afferents branch out as a lateral cutaneous branch and continue within the tap to perforate anteriorly supplying the skin as far as the midline .
the tap plane thus provides a space into which the local anesthetic can be deposited to achieve myocutaneous sensory blockade .
whilst early studies showed a t7 to l1 spread with a single posterior injection making the block suitable for midline abdominal incisions , other studies , however , failed to demonstrate a spread cephalad to t10 making it more suited for lower abdominal surgery . in a small cadaveric study , t11 , t12 , and l1 were most consistently present in the tap while t10 was present in 50% of the cases .
however , augmentation with a subcostal injection will help attain a higher block up to t7 which is a modification of the original technique in which the ultrasound probe is placed just beneath the costal margin and parallel to it .
the needle is then introduced from the lateral side of the rectus muscle in the plane of the ultrasound beam and 10 ml of local injected into the tap to extend the analgesia provided by the posterior tap block above the umbilicus . in our patient bilateral ,
sub - costal tap block under ultrasound guidance resulted in highly effective myocutaneous sensory blockade .
as the patient was categorized as asa iv / e general anesthesia or central neuraxial blockade for the emergency operation for such a high - risk case would have resulted in untoward and fatal complications both intra - operatively and postoperatively .
the abdominal wall sensory afferents , which course through the tap plane could be blocked successfully and effectively by abdominal field blocks or tap block under ultrasound guidance then the abdominal incision and operation could be carried out without patient 's discomfort .
pain caused by visceral stimulation of the celiac plexus may still challenge intraabdominal surgical success which is a major limitation .
when the surgeons manipulated the intestine , the patient complained of pain due to stimulation of autonomic nervous system through celiac plexus ( vagus ) , but dexmedetomidine infusion helped in relieving the pain or retching sensation .
anatomically , sympathetic and somatic innervation are closely related near the neuraxis , but become separated peripherally .
thus , spinal , epidural or paravertebral blocks will cause significant sympathetic block , resulting in major cardiovascular changes and other physiological effects . on the other hand ,
if complete denervation of viscera is required , vagal afferents have to be blocked by celiac plexus block .
the extent and spread of the local anesthetic solution in the tap affecting anterior abdominal wall sensory afferents depend on time factor .
it seems that the full effect of analgesia takes at least 20 - 25 min after injection of local anesthetic solution .
mcdonnell et al . suggest that local anesthetic spreads within the tap plane progressively over the several hours and an early assessment of the extensive tap block may be missed .
as ultrasound - guided bilateral transversus abdominais neurofascial plane block is quite simple , quick , safe , and effective especially for a very high - risk patient with multi - medical problems and geriatric patients needing an elective or emergency abdominal surgery , the surgeons and anesthesiologists should encourage this technique , even in this advanced era , when it is deemed suitable . a retrospective review of clinical records of late postpartum eclampsia patients who had presented with acute onset of visual and/or neurological symptoms to the emergency department of a medical college hospital in south india between april 2009 and may 2013 was done .
all patients had underwent mri with diffusion weighted imaging ( dwi ) and apparent diffusion coefficient ( adc ) mapping to differentiate vasogenic from cytotoxic cerebral edema .
patient records were analyzed for presence of visual symptoms , peak systolic and diastolic blood pressures , associated neurological symptoms , location of mri lesions and time taken for recovery .
the mr images were graded for extent and severity of cortex and white matter vasogenic edema , degree of confluence , mass effect and ventricular distortion on a scale from 1 to 5 .
statistical analysis was performed using chi - square test and a p value of less than 0.05 was considered to be statistically significant .
clinical and imaging findings are summarized in table 1 . clinical profile of patients with pres the 10 patients ranged in age from 21 to 32 years ( average-26 3.49 years ) .
mean peak systolic and diastolic blood pressures were 144 21.71 and 93 9.19 mm of hg , respectively .
bilateral visual loss was noted in five patients of whom three patients were able to perceive hand movements and two patients had only perception of light .
the most common abnormality noted on mri included bilateral symmetrical hyperintensities on t2-weighted images and fluid attenuated inversion recovery ( flair ) sequences in the parieto - occipital regions [ figs . 1 and 2 ] .
dwi showed high signal intensity with no areas of restricted diffusion while adc mapping did not show corresponding low signal intensity thus suggesting vasogenic edema .
axial mr image ( fluid attenuated inversion recovery ) showing confluent edema in subcortical and deep white matter ( grade 4 ) seen as hyperintensities in bilateral parieto - occipital lobes ( patient 6 ) axial mr image ( fluid attenuation inversion recovery ) showing edema in subcortical white matter ( grade 2 ) seen as hyperintensities in bilateral occipital lobes ( patient 9 ) five patients had additional abnormalities in the cerebellar hemispheres and three patients had involvement of frontal lobes . average time taken for visual recovery was 2.6 1.34 days .
follow - up mri in four patients after 1 month revealed complete resolution of these abnormalities . in the remaining , clinical symptoms and signs
follow - up scans were not performed due to financial constraints . the extent of cerebral edema in patients with and without visual loss was compared and results summarized in table 2 .
posterior reversible encephalopathy syndrome ( pres ) is a rare neurotoxic state that presents with altered mental status , headache , seizures , and visual disturbances along with neuroimaging features of vasogenic edema involving the posterior cerebral circulation .
although several cases of pres have been reported in neurology and neuroradiology literature , exposure in ophthalmic literature has been rather limited .
the causes of pres are diverse and include pre - eclampsia , eclampsia , renal insufficiency , solid organ transplantation and immunosuppressive therapy .
hypertensive encephalopathy is said to be the cause of this syndrome which has been demonstrated by various clinical and experimental studies .
patients with hypertensive encephalopathy have the same clinical signs as those with pres and they also have rapid resolution of clinical and imaging abnormalities once the blood pressure is lowered .
the most widely accepted theory states that sudden elevation of blood pressure causes failure of autoregulation in the cerebral blood vessels leading to hyperperfusion , breakdown of blood brain barrier , and vasogenic edema . the posterior circulation is preferentially affected since it has less sympathetic innervation than the carotid circulation , thus rendering it less able to adjust to blood pressure fluctuations . however , this theory is not comprehensive because pres can affect normotensives .
high degree of suspicion is required in these patients of late postpartum eclampsia because it occurs between 48 hours postpartum and 1 month after delivery frequently in women who have had a normal pregnancy and delivery and no signs of a pre - eclamptic syndrome .
alternative theory implicates endothelial dysfunction as the cause for occurrence of pres in eclampsia and sepsis .
this is supported by the fact that elevations in markers of endothelial dysfunction such as lactate dehydrogenase and abnormal red blood cell fragmentation have been reported in pres .
vasospasm with subsequent ischemia has also been hypothesized to cause loss of integrity of the blood brain barrier in eclampsia .
although reversible by definition early recognition and prompt treatment is essential to prevent secondary complications like intracerebral hemorrhage and infarction .
a large retrospective study of pres in diverse clinical situations revealed the occurrence of visual symptoms in 20% and headache in 26% while seizures was observed in 74% of cases .
the visual disturbances reported with pres include cortical blindness , visual neglect , homonymous hemianopia and blurred vision .
cortical visual loss was observed in five of our patients ( 50% ) while the remaining denied any visual disturbances .
our findings are in line with liman et al . , who observed that visual disturbances like cortical blindness , blurred vision and hemianopia are more common in eclampsia related pres ( 50% ) .
patients with pres due to other etiologies more often present with severe symptoms like altered mental status or neurological deficit and lesser visual disturbances ( 27.8% ) .
roth et al . , have also reported a higher percentage of disturbed vision in pre - eclampsia - eclampsia - related pres .
this could be because of the younger age and lesser associated comorbidities in these patients as compared to patients with pres due to other etiologies .
all patients ( with / without visual loss ) demonstrated bilateral symmetrical hyperintensities in the parieto - occipital regions on t2-weighted images .
this feature along with predominant involvement of the white matter distinguishes this syndrome from bilateral posterior cerebral artery territory infarction .
although several studies have reported visual symptoms of varying degrees in pres , to the best of our knowledge no study has attempted to correlate the visual symptoms with imaging abnormalities .
the limitations of our study include its retrospective design , and smaller sample size of postpartum eclampsia patients .
we conclude that there is a higher prevalence of cortical visual loss in patients with pres associated with late postpartum eclampsia .
patients with higher degree of vasogenic edema of the posterior cerebral white matter might present with visual loss .
ophthalmologists should be aware of this clinical entity since it is reversible and readily treated by controlling the blood pressure .
further prospective studies comprising larger sample size and different etiologies of pres are warranted in this regard . | although transversus abdominis plane ( tap ) block is an effective way of providing analgesia in post - operative abdominal surgery patients ; however , it can be considered as an anesthetic technique in high - risk cases for surgery .
we report a case of a geriatric female with chronic obstructive pulmonary disease in the respiratory failure , hypotension , posted in an emergency with old perforation leading to peritonitis .
the surgery was successfully conducted under bilateral tap block , which was used as a sole anesthetic technique .
tap block can be considered as an anesthetic technique for abdominal surgery in moribund patients . |
institutional review board approval was obtained from the wake forest university , and informed consent forms were completed for all participants .
items for four concepts identified in the literature were glucose control , well - being and side - effects , lifestyle burden , and treatment complexity and convenience and were evaluated in a series of five focus groups made up of five to eight patients drawn from an evaluation study of community diabetes clinics in north carolina ( 11 ) .
participants were male and female and white and nonwhite with simple and complex medication regimens and a1c levels that ranged from well controlled to uncontrolled .
the resulting 35-item prototype instrument was administered by mail to a convenience sample of 75 patients ( the exploratory sample ) , who were treated with diabetes medications at our study community - care site , to assess item reliability , mean and distribution , redundancy or uniqueness , skewness , and construct validity .
also examined were item correlations with a1c level , the multidimensional diabetes questionnaire ( 12 ) lifestyle interference scale , the medical outcomes studies ( mos ) health worries scale score ( 13 ) , and global items assessing extent that blood glucose has been unacceptably high or low . an item performance score was constructed ( 0 , weak ; 1 , moderate ; or 2 , ideal performance ) to guide item retention .
fifty - five ( 73% ) patients completed the survey , and nine items were removed based on skewness or redundancy ( r > 0.75 ) with other items . in the initial test sample
, patients of a large family - medicine practice treated for diabetes with a recent a1c value within the last 3 months ( the evaluation sample ) were invited to complete the study survey packet including the revised 26-item instrument and validation instrument described above .
medication complexity was assessed using a score of 0 or 1 ( no / yes ) for common diabetes medications and a score of 0 or 2 ( no / yes ) for insulin , a more demanding regimen .
self - reported adherence to medications was by recall of skipped or missed doses over the last 10 days .
packets were mailed to patients with instructions and a voucher for a 25 usd gift certificate .
exploratory factor analysis ( efa ) of the dmsat items was conducted using sas ( version 8 ; sas , cary , nc ) to assess whether the common factor model was appropriate ( 14 ) based on kaiser 's sampling adequacy , scree plot , and model fit .
discriminant validity of the dmsat was examined by comparing means across levels of a1c ( < 8% and 8% ) , treatment complexity ( low and high ) , self - reported adherence , and mos health worries . for the final test sample ,
another sample of patients from our community diabetes care clinics ( 11 ) and from an academic medical center was recruited to conduct and evaluate confirmatory factor analysis of the dmsat and confirm validity
in the evaluation sample , 194 ( 63% ) of 307 eligible patients returned the survey packet ; of these , 140 reported current medication use .
participants had a mean age of 63 years , and most had completed high school ( 77% ) and had been diagnosed with diabetes at least 5 years previously ( 61% ) .
one - third ( 2939% ) were taking one , two , or three medications for diabetes , with 16% taking insulin ; 14% had a recent a1c > 8.0% , and 19% rated their adherence to their medication regimen in the last 10 days as less than complete .
ten items displayed high inter - item correlations ( > 0.75 ) and were removed .
initial factor analysis of the reduced 16-item questionnaire identified a four - factor structure consistent with our domains of lifestyle , medical control , convenience , and well - being and explained 75% of the total variance .
as shown in table 1 , dmsat scales and total score discriminated ( p < 0.05 ) between high and low levels of treatment complexity , self - rated glucose control , mos health worries scale score , and clinical value for recent a1c ( < 8% vs. 8% ) in the expected direction .
correlation of the dmsat scores with continuous a1c values was 0.24 ( p = 0.0049 ) . in the final , confirmatory sample ,
as shown in table 1 , dmsat scales and total scores discriminated between validity groups as in the previous sample and were highly correlated with the dtsq ( r = 0.68 ; p < 0.001 ) . unlike the dmsat , the dtsq total score did not discriminate between levels of treatment complexity and clinical a1c value .
the dmsat is intended as a brief measure of diabetes medication treatment satisfaction and discriminates between important correlates of patient management .
it performed as well as the dtsq in detecting self - rated glucose control and health worries but showed superior properties in correspondence with treatment complexity and a1c .
note that appraisals of cost of medications or specific side effects that may be caused by diabetes or its treatment , such as diminished sexual functioning , bloating , or weight gain , are not separately assessed and may require assessment elsewhere .
, we believe that the 16-item dmsat offers a comprehensive assessment of satisfaction with diabetes therapy and may aid in individualizing patient diabetes treatment . the incidence of infective endocarditis ( ie ) remained relatively stable from 1950 through 2000 at about 3.6 to 7.0 cases per 100,000 patient - years.1 in selected areas , the incidence may rise because of the concentration of populations at uniquely high risk of infection , specifically intravenous ( iv ) drug abusers .
the risk of ie among iv drug abusers , 2 to 5% per patient - years , is several - fold greater than that for patients with rheumatic heart disease or prosthetic valves .
ie is located on the tricuspid valve in 46 to 58% of patients with iv drug abuse .
staphylococcus aureus causes more than 50% of ie occurring in iv drug abusers overall and 60 to 70% of infection involving the tricuspid valve .
the clinical manifestation of ie in iv drug abusers depends on the valve involved and , to a lesser degree , on the infecting organism .
the sensitivity of transthoracic echocardiography ( tte ) for the detection of vegetations in patients with native valve endocarditis ( nve ) is approximately 65% , whereas that of transesophageal echocardiography ( tee ) in these patients is 85 to 95%.2 tee is the preferred approach in patients in whom tte is technically suboptimal and is the procedure of choice for imaging the pulmonic valve.3 when initial tee is negative and the clinical suspicion of ie remains , repeating tee within 7 to 10 days is advocated.4 perivalvular abscess or intracardiac fistula formation occurs in 10 to 14% of patients with nve.6 persistent , otherwise unexplained , fever despite appropriate antimicrobial therapy in patients with ie suggests infection extending beyond the valve leaflet .
tee is superior to tte for detecting invasive infection in patients with nve and prosthetic valve endocarditis ( pve ) .
cardiac surgery should be considered to debride abscesses , allowing the eradication of uncontrolled infection , and to reconstruct cardiac structures , restoring homodynamic and alleviating congestive heart failure .
a 21-year - old man , who was an iv drug abuser , presented with fever and dyspnea .
tte showed highly mobile , large vegetation on the anterior leaflet of the tricuspid valve and no vegetation on the aortic valve , mitral valve , and pulmonic valve .
the patient was admitted to the cardiology ward for antibiotic therapy and received vancomycin and gentamycin .
the blood culture of the patient became positive for staphylococcus areus 3 times in 24-hour intervals . despite the antibiotic therapy for 10 days
tee revealed severe aortic insufficiency , moderate tricuspid regurgitation , and an echo - lucent space between the tricuspid and aortic valves ( figures 1 ) .
color doppler demonstrated a flow within the echo - lucent space and a connection between that and the left ventricle , suggesting a perivalvular abscess of the tricuspid valve opening in the left ventricle ( figure 2 ) .
after consultation with a cardiac surgeon , the patient was transferred to the operating room , where he unfortunately expired due to severe bleeding and disseminated intravascular coagulation .
ie is a serious complication of iv drug abuse , with a reported mortality of 5 to 10%.1 endocarditis in iv drug abusers commonly involves the tricuspid valve , and staphylococcus aureus is the most common causative organism .
dyspnea , cough , and chest pain are the common complaints of iv drug users .
this is likely related to the predominance of tricuspid valve endocarditis in this group and secondary embolic showering of the pulmonary vasculature . within a week after the initiation of effective antimicrobial therapy , almost 70% of patients with nve or pve are afebrile and 90% have defervesced by the end of the second week of treatment.5 persistence or recurrence of fever more than 10 days after the initiation of antibiotic therapy identifies patients with increased mortality rates and with complications of infection or therapy.5 patients with a prolonged or recurrent fever should be evaluated for intracardiac complications .
perivalvular infection beyond the valve leaflet results in abscesses in the annulus or adjacent structures , intracardiac fistulas , and purulent pericarditis .
periannular extension is common , occurring in 10 to 40% of all native valve ie and complicates aortic valve endocarditis more commonly than mitral or tricuspid valve endocarditis.3 intra - cardiac fistulas are rarely seen and they are estimated to account for < 1% of all cases of ie.6 fistulization of the paravalvular abscess has been found in 6 to 9% of all cases.7 perivalvular abscess and intracardiac fistula of the tricuspid valve is very rare .
ie is a lethal disease if not treated aggressively with parental antibiotics , often in combination with surgery .
cardiac surgery should be considered in patients with perivalvular abscess and intracardiac fistula to debride abscesses and to reconstruct cardiac structures , restoring homodynamic and alleviating congestive heart failure .
| objective to develop and test a patient questionnaire on treatment satisfaction with diabetes regimens.research design and methods survey items were developed from community clinic focus groups , pretested in patients with diabetes , and examined in two samples of treated patients.resultssixteen items performed well in assessing treatment experiences : ease and convenience , lifestyle burdens , well - being , and medical control .
construct validity was supported by associations ( p < 0.05 ) with treatment complexity , self - rated glucose control , health worries , and a1c .
internal consistency ranged from 0.89 to 0.95.conclusionsthe diabetes medication satisfaction tool offers a comprehensive assessment of patient acceptability , with diabetes therapy useful for individualizing therapeutic decision making . |
six hundred officers were recruited from the police departments of new york , ny , and oakland and san jose , calif , usa . fifty - two percent of the sample was caucasian .
the mean age was 36,50 years ( standard deviation [ sd])=6.96 ) . years in the police force everaged 12.37 ( sd=6.78 ) .
number of exposure to critical incidents ranged from 2 to 670 ( mean [ m]= 171.27 , sd=130.93).the incident selected for completing the questionnaires had occured on average 6.50 ( sd= 5.11 ) years ago .
the peritraumatic distress scale ( pds ) was used to assess emotional , cognitive , and physical reactions occuring during a critical incident and immediatly after .
dissociation at the time of the incident was measured with the peritraumatic dissociative experience questionnaire ( pdeq).the impact of event scale - revised ( ies - r ) was used to measure ptsd symptoms in the last 7 days .
the mississippi scale ( mcs ) was used to measure ptsd and associated symptoms since the critical incident .
we conducted a cronbach alpha reliability analysis and an oblique principal factor analysis with promax rotation on the items of the pds .
two series of hierarchical multiple regression analyses were conducted using sociodemographics ( gender , ethnicity , years of service ) , exposure , the pdeq and pds as predictors of either the mcs or the ies - r
six hundred officers were recruited from the police departments of new york , ny , and oakland and san jose , calif , usa . fifty - two percent of the sample was caucasian .
the mean age was 36,50 years ( standard deviation [ sd])=6.96 ) . years in the police force everaged 12.37 ( sd=6.78 ) .
number of exposure to critical incidents ranged from 2 to 670 ( mean [ m]= 171.27 , sd=130.93).the incident selected for completing the questionnaires had occured on average 6.50 ( sd= 5.11 ) years ago .
the peritraumatic distress scale ( pds ) was used to assess emotional , cognitive , and physical reactions occuring during a critical incident and immediatly after .
dissociation at the time of the incident was measured with the peritraumatic dissociative experience questionnaire ( pdeq).the impact of event scale - revised ( ies - r ) was used to measure ptsd symptoms in the last 7 days .
the mississippi scale ( mcs ) was used to measure ptsd and associated symptoms since the critical incident .
we conducted a cronbach alpha reliability analysis and an oblique principal factor analysis with promax rotation on the items of the pds .
two series of hierarchical multiple regression analyses were conducted using sociodemographics ( gender , ethnicity , years of service ) , exposure , the pdeq and pds as predictors of either the mcs or the ies - r
the pds scores ranged from 0.10 to 3.57 and the mean was 1.37 ( sd=0.56 ) .
the scale was internally consistent ( =0.80 ) and showed strong convergent validity with the pdeq , r(599)=0.55 , p<0.001 .
the pds factor solution is presented in table i items defining factor 1 included dysphoric emotions such as helplessness , sadness and grief , frustration and anger , and horror .
factor 2 was mostly defined by items related to loss of safety and arousal , such as being afraid , thinking one might die , and having intense bodily reactions ( sweating , shaking , heart - pounding ) .
items loading on factor 3 were related to the loss of positive beliefs about the self and others , such as thinking that one had done all he or she could during the critical incident , not felling prepared by one 's experience , and not believing tha others understood .
we labeled the factors negative emotions , perceived life threat and bodily arousal , and appraisal .
the sum of communality estimates was 7.58 , explaining 38% of the communality estimates was 7.58 , explaining 38% of the total variance and 93% of trace .
intercorrelations among the pds factors were low , ranging from -0.25 to 0.12 ( p<0.05 ) .
the low pds factor intercorrelation coupled with correlations of 0.17 to 0.42 ( p<0.001 ) with the outcome measures ( ies - r and mcs ) suggest that various forms of peritraumatic distress , as captured by the pds , can lead to the development of ptsd symptoms .
two stepwise regression analyses ( not fully reported here ) were conducted . in predicting the mcs and ies - r ,
the pdeq , entered in the second step , explained 20% and 16% of unique variance on the mcs and ies - r , respectively . entering the pds in step 3 explained 11% and 8% unique variance on the mcs and ies - r , respectively .
we repeated this set of analyses with the inclusion order of the pdeq and pds reversed .
entered in the second step , the pds explained 29% and 17% of unique variance on the mcs and ies - r , respectively . entered in the third step , the pdeq explained 3% of unique variance on both the mcs and the ies - r .
the items and factors of the pds provide insight as to what some of the salient peritraumatic dimensions may be , in addition to peritraumatic dissociation . in this study ,
the pds explained a significant amount of variance over and above peritraumatic dissociation which is currently considered among the mos powerful predictors of ptsd symptoms .
test - retest data for the pds is currently being gathered as well as data from individuals not working in the police . in future
, it would be useful to investigate prospectively the power of the pds in predicting ptsd diagnosis rather than symptoms , as well as other trauma - related disorders . gastrointestinal perforations constitute one of the commonest surgical emergency encountered by surgeons [ 1 , 2 ] .
management of these patients continues to be highly demanding despite the advances made in diagnosis and surgical therapy .
the etiological spectrum of perforation peritonitis in india differs significantly from its western counter parts [ 35 ] .
our study was carried out to highlight the spectrum of perforation peritonitis ( diffuse ) in a single unit at lok nayak hospital , a tertiary care hospital in delhi .
the retrospective study was conducted at the department of surgery , maulana azad medical college and associated lok nayak hospital , delhi , from may 2010 to june 2013 .
the study population included 400 patients of perforation peritonitis ( diffuse or localized ) presenting to the surgical emergency of lok nayak hospital , delhi , who underwent exploratory laparotomy .
cases were studied with respect to clinical features at the time of presentation , comorbid conditions , radiological investigations , operative findings , and postoperative course . after establishing the clinical diagnosis of perforation peritonitis
on performing exploratory laparotomy , the operative findings were noted and the source of peritonitis was found and managed accordingly .
all patients were then treated in the postoperative ward initially under the cover of parenteral broad spectrum antibiotics and fluids ; orals were started on the appearance of bowel sounds .
98% patients presented with the history of abdominal pain , 62.5% with altered bowel habit , 41.5% with nausea and vomiting , and 28% with abdominal distention .
15% patients had positive history of nsaid intake for more than 6 months ( table 1 ) . in our study ,
the commonly associated comorbidity was chronic obstructive pulmonary disease followed by renal disease , diabetes , and hypertension .
multiple air fluid levels on abdominal x - ray in erect position were present in 28% patients .
electrolyte imbalances included hyponatremia in 21% , hypokalemia in 19% and elevated serum creatinine in 18% patients .
most of the patients were operated within 24 hours of presentation under the cover of broad spectrum antibiotics after adequate resuscitation and correction of electrolyte imbalances .
the commonest cause of perforation peritonitis in our study was gastroduodenal perforation due to acid peptic disease ( 45% ) followed by appendicitis ( 18.5% ) , typhoid fever ( 12% ) , tuberculosis ( 10% ) , and trauma ( 9% ) , ( table 2 ) .
patients of peptic ulcer perforation usually had a short history of pain starting in epigastrium followed by generalized tenderness .
175 such were managed by an omental pedicle repair , in the other 4 cases a feeding jejunostomy was also done due to the large size of the perforation .
8% of these patients were managed by a limited resection with ileo - ascending anastomosis due to associated unhealthy caecum .
patients of typhoid perforation had an initial history of high grade fever prior to abdominal complaints .
of the 40 patients of tubercular perforation , 60% had previous history of tuberculosis and 50% of these patients took antitubercular therapy for < 6 months . in cases of traumatic perforation ,
the most common site was jejunum ( 49% ) followed by ileum ( 42% ) .
the most commonly performed procedure was omental pedicle closure of peptic ulcer perforation ( 43.75% ) , followed by exteriorization of the gut in the form of ileostomy or colostomy ( 22.5% ) .
appendectomy was the third most common procedure ( 17% ) , ( table 2 ) .
the most common complication was wound infection followed by dyselectrolytaemia , abdominal collection , and respiratory complications .
the morbidity rate was higher in the patients with intestinal perforation ( 58% ) than those with gastroduodenal perforation ( 32% ) .
factors involved in death included septicemia due to fecal peritonitis , respiratory complications , pulmonary embolism , and late presentation .
it is commonly seen in a younger age group in the tropical countries ( mean age in our study was 37.8 years ) as compared to the studies in the west [ 79 ] .
more commonly the perforations involve the proximal part of the gastrointestinal tract ; [ 1013 ] this being in contrast to studies from the western countries , where perforations are common in the distal part [ 1416 ] .
etiological factors also show a wide geographical variation . according to a study from india ,
infections formed the most common cause of perforation peritonitis , around 50% cases in this study were due to typhoid . in our study 22% of the cases were due to typhoid and tuberculosis .
in contrast to this , noon et al . from texas in their study reported only 2.7% cases due to infections .
also studies from the west have shown that around 1520% cases are due to malignancy [ 19 , 20 ] , this being in stark contrast to our study where malignancy was ascertained to be the cause of perforation peritonitis in only 3% of the cases .
this shows that malignancy is not a common cause of perforation peritonitis in our setup as compared to our western counterparts .
adequate preoperative resuscitation ( with fluids , etc . ) , correction of electrolyte imbalances followed by an early surgical intervention , to remove the source of infection and stop further contamination , is imperative for good outcomes minimizing morbidity and mortality .
peptic ulcer perforation , perforating appendicitis , typhoid , and tubercular perforations are the major causes of gastrointestinal perforations .
early surgical intervention under the cover of broad spectrum antibiotics preceded by adequate aggressive resuscitation and correction of electrolyte imbalances is imperative for good outcomes minimizing morbidity and mortality . | posttraumatic stress disorder ( ptsd ) occurs when significant intrusion , avoidance , and hyperarousal symptoms are manifest for at least 1 month following exposure to a traumatic event , with at least 1 month following exposure to a traumatic event , with at least 1 month elapsed betwenn the event and the diagnossis ( diagnostic and statistical manual of mental disorders , 4th edition , 1994 [ dsm - iv]).1 however , such symptoms are not necessarily manifest in the immediate aftermath of the trauman,2 nor does their intial presence strongly predict who will develop ptsd.3 one immediate response to trauma which has been convicingly linked to ptsd symptoms is peritraumatic dissociation.4 in this poster , we briefly introduce a new scale assessing immediate responses distinct from peritraumatic dissociation , and we examine its power to predict ptsd symptoms . |
the term isotopic response connotes the occurrence of a new skin disorder at the site of another , unrelated , and already healed skin disease . since the introduction of this phenomenon in dermatology , a variety of dermatoses
we hereby describe a young girl who developed lesions of lichen planus pemphigoides ( lpp ) over preexisting atrophic scars , an example of wolf 's isotopic response . to the best of our knowledge , such a clinical presentation of lichen planus ( lp ) pemphigoides is not documented yet in the english literature .
an 18-year - old girl presented with multiple itchy hyperpigmented papules and plaques , along with intermittent blisters over the lower limbs and buttocks for last 3 months [ figures 1 and 2 ] .
past history was significant for painful ulcers over the lower limbs and buttocks 6 years back .
the diagnosis of pyoderma gangrenosum was made ( as evident from documents available with patient ) , and she was treated for a year , resulting in complete resolution of ulcers .
the bulla was noted over both lichenified plaques and otherwise normal appearing skin of scars .
interestingly , most of the lichenified plaques and bullae were found over preexisting scars only [ figure 3 ] .
skin biopsy was done from multiple sites , for both histopathological examination and direct immunofluorescence ( dif ) .
the histological features of lichenified plaque were orthokeratotic hyperkeratosis , acanthosis , wedge shaped hypergranulosis , basal layer degeneration and band - like of infiltrate of lymphocytes in the papillary dermis ; thus corroborating with a diagnosis of lp [ figure 4a c ] .
histopathology of bulla showed a subepidermal bulla with eosinophilic infiltrate ; which were suggestive of bullous pemphigoid ( bp ) [ figure 5a c ] .
the scar tissue was characterized histologically by the presence of abundant collagen fibers without any features of inflammation [ figure 6 ] .
dif from plaque showed a linear pattern of deposition of igg and c3 along the basement membrane zone [ figure 7 ] .
dif from the perilesional skin surrounding a bulla showed linear igg and c3 along basement membrane zone ; a feature suggestive of bp [ figure 8 ] .
thus , in conjunction with the clinical , histopathological and dif findings , the final diagnosis for our case was lp pemhigoides developing over preexisting scars , as an isotopic phenomenon .
atrophic scars with overlying lichenoid papules and plaques ; and tense bulla on lower limbs atrophic scars with overlying lichenoid papules and plaques on buttocks close - up of lesions on the leg .
lesions are restricted to area of scar only histopathology from plaque showing hyperkeratosis , hypergranulosis , saw toothing of rete ridges , band - like infiltration in upper dermis ( a ) ( h and e , 40 ) and ( b ) ( h and e , 100 ) with basal layer degeneration ( c ) ( h and e , 400 ) histopathology from bulla showing partial loss of epidermis including basal layer ( a ) ( h and e , 40 ) and ( b ) ( h and e , 100 ) and plenty of eosinophils in split ( c ) ( h and e , 400 ) histopathology from scar showing unremarkable epidermis , increased collagen in dermis and no inflammatory cells ( h and e , 40 ) direct immunofluorescence from plaque showing linear deposition of ig g and c 3 along basement membrane zone direct immunofluorescence from perilesional skin of bulla showing linear deposition of ig g and c3 along basement membrane zone
lpp is an uncommon variant of bp , clinically manifested with bullous lesions over papules and plaques of lp , as well as on the uninvolved skin .
it was first described by kaposi in 1892 as a classical case of lp complicated by extensive bullous dermatosis ; which was termed as lpp .
it can be precipitated by certain medications like cinnarizine , captopril , ramipril , psoralen and ultraviolet a ( puva ) , simvastatin , and antituberculous medications .
igg autoantibodies against both bpag2 and bpag1 are thought to play a pivotal role in the development of the dermatosis .
the widely accepted mechanism proposed is that lp or sometimes , puva causes damage to basal cells leading to unmasking of hidden antigens ; thus paving the pathway for the formation of antibodies and development of lesions of bp .
a report described the correlation of levels of antibodies against bpag2 with the disease activity in lpp .
the salient points of difference between lpp and bullous lp have been summarized in table 1 .
dif testing show a smooth linear distribution of immunoreactants ( c3 , igg , igm , and iga ) along the basement membrane zone of dermoepidermal junction .
however , a unique 200 kd antigen has been recognized on the epidermal side of salt split skin of patients with lpp and thus , lpp may represent a clinically distinct entity and not merely , a co - existence of lp and bp .
effective treatment modalities include topical and systemic steroids , tetracycline , nicotinamide , dapsone , isotretinoin , methotrexate and other immunosuppressive drugs .
this could be explained by the hypothesis that lpp has a unique antigenic target and thus , represents a distinct entity from bp .
lpp has been reported in association with keratoacanthoma and colon adenocarcinoma . a unilateral presentation has also been reported .
the occurrence of a different or unrelated dermatological disease at the site of the healed disease is termed as an isotopic phenomenon .
the proposed etiologies of isotopic response include viral , immunologic , neural , vascular and locus minoris resistentiae ( a site of lessened resistance ) .
however , development of lpp over preexisting scars is extremely unique and to the best of our knowledge , is not described in the english literature .
it is a well - known fact that bulla formation in lpp is mediated by immunological damage against bp antigen along basement membrane zone .
the predilection of the blisters to develop on the lower extremities could be explained by sluggish circulation in those regions . in our case ,
scars involving the dermis and subcutaneous tissue with largely unaffected epidermis might have altered the blood circulation in that particular area .
this could explain the preferential deposition of immunoglobulins in the scar area and subsequent bulla formation .
wolf 's isotopic response in lpp has probably not been described earlier in the medical literature .
wolf 's isotopic response in lpp has probably not been described earlier in the medical literature .
wolf 's isotopic response in lpp has probably not been described earlier in the medical literature . charcoal was originally used for localizing a non - palpable tumor mass ( 1 ) , however , it is being increasingly used for localizing suspicious metastatic cervical lymph nodes or recurrent tumors in the thyroid bed after surgery ( 2).although , charcoal is known to be safe and stable when injected subcutaneously for preoperative tumor localization , it may cause foreign body reactions and granuloma , if it remains in situ for more than 6 months ( 1 ) . until recently ,
charcoal granuloma in the neck was not reported in the literature . under the approval of the institutional review board of our hospital
, we reported a case of a charcoal granuloma that was suspected to be a recurrent tumor on ultrasonography ( us ) and f - fluorodeoxyglucose ( fdg)-positron emission tomography / computed tomography ( pet / ct ) , but was correctly identified after us - guided core biopsy .
a 47-year - old woman was referred to our department for us - guided biopsy due to a persistent hypermetabolic nodule in the right level iv nodal station .
she was diagnosed with invasive ductal carcinoma of the right breast and cervical lymph node metastasis at the right level iv ( tnm stage ; t2n3m0 ) .
she had undergone breast - conserving surgery and excision of a metastatic lymph node ( fig .
before excision of cervical lymph node metastasis , us - guided localization had been performed using charcoal .
after surgery , she received chemotherapy and radiotherapy ( up to 50.4 gy ) for the right breast and the right lower cervical area . on her first follow - up fdg - pet / ct ( fig .
1d ) , which was performed 6 months after completion of treatment , a focal hypermetabolic lesion ( maximum standardized uptake value [ suvmax ] = 4.0 ) was noted at the lateral aspect of the previous operative bed in the right level iv .
the second follow - up pet / ct examination , performed 1 year after the first follow - up pet / ct scan , showed a persistent hypermetabolic lesion ( suvmax = 4.3 ) at the same site ( fig
the lesion was ovoid , measured 9.8 mm in its maximal longitudinal diameter , and had an indistinct margin from the adjacent sternocleidomastoid muscle ( fig .
on pre - contrast ct , the image noise and suspicious beam hardening artifact related to overlapped clavicles and shoulder girdles , rendered identification of the lesion difficult .
however , the lesion seemed to be slightly hyperdense relative to the adjacent sternocleidomastoid muscle ( mean attenuation : 89 - 100 hounsfield unit ) and did not significantly enhance after contrast administration ( fig .
1 g ) . since there was no evidence of recurrence at any other site of the body except the right side of the neck , and given the conflicting results of the previous cytological and pet / ct or chest ct examinations were , we decided to closely monitor and follow - up again 6 months later .
six months later , us showed a change in the echogenicity of the nodule with no change in size , but a slight change in shape ( fig .
as the morphology of the nodule changed , we could not rule out the possibility of a recurrent tumor .
thus , we decided to perform a core - needle biopsy , as the previous us - guided fine - needle aspiration did not yield sufficient tissue for an accurate diagnosis .
us - guided core - needle biopsy was performed thrice using an 18-g semi - automatic biopsy needle ( tsk stericut ; tsk laboratory , soja , japan ) .
microscopically , these dark - pigmented specimens were foreign body granulomas that comprised extensive deposits of charcoal and multinucleated giant cells , as well as fibrosis ; no tumor cells were detected ( fig .
patients who undergo surgery for malignant lesions in the neck may subsequently show benign conditions mimicking recurrent tumors . these include reactive lymph nodes , traumatic neuroma , postoperative fibrosis , and suture granuloma ( 3 , 4 , 5 ) .
charcoal is originally used for localization of the non - palpable breast mass ( 1 ) .
charcoal is known to be a biologically inert material when injected subcutaneously and may remain in situ for up to 60 days without triggering a foreign body reaction ( 1 ) . however , in an experimental study , charcoal particles were ingested by macrophages and very slowly carried away from the injection site , causing inflammatory processes ( 6 ) .
charcoal particles that remained in the surgical bed might have facilitated an inflammatory reaction during the healing process after surgery in our patient .
our patient also received radiotherapy after excision of the metastatic lymph node , possibly resulting in synergistic effects leading to fibrotic charcoal granuloma .
the us features of charcoal granuloma in our report were characterized by ill - defined irregular shaped lesion with initial hyperechogenicty that changed over time , and posterior shadowing .
indistinct margin and posterior shadowing of the charcoal granuloma in our patient was in accordance with the finding of charcoal granuloma in the breast ( 7 ) .
conversely , our lesion was hyperechoic at first , and decreased in echogenicity over time .
initial hyperechogenicity of our lesion might be a helpful finding for differentiating a tumor recurrence from a non - tumorous lesion , such as a charcoal granuloma .
tumor recurrence was reported to be hypoechoic to markedly hypoechoic in the surgical bed in the post - thyroidectomy neck ( 8) .
although the size of the nodule was not helpful to differentiate recurrence from benign postoperative change at one time point ( 8) , the interval change of the nodule size could become one of the important imaging findings to differentiate recurrence from benign lesion .
in our case , the shape and echogenicity of the lesion was changed over time , however , the lesion did not significantly increase in size during a follow - up , which suggested a benign status .
ct and fdg - pet imaging findings in our patient were consistent with those of charcoal granulomas in the peritoneal cavity related to intraoperative , intraperitoneal chemotherapy ( 9 ) .
the lesions showed high attenuation on precontrast ct with no remarkable enhancement on post - contrast scans .
higher attenuation of the lesion compared to the adjacent muscles might be attributed to the high concentration of carbon particles ( 9 ) .
both suture granulomas and charcoal granulomas can present hypermetabolic lesions due to increased utilization of glucose by activated multinucleated giant cells ( 9 , 10 ) .
therefore , such foreign body granulomas can be a common cause of false positive findings in the evaluation of tumor recurrence in the postoperative neck .
ultrasonography is the first line of imaging follow - up in patients with neck dissection or thyroid surgery .
suture granulomas as a result of the knotted suture materials after thyroid surgery , usually had characteristic irregularly shaped , multiple or paired central or paracentral echogenic foci > 1 mm , and heterogeneous hypo- to isoechoic lesions on us ( 4 ) .
although us alone is not easy to distinguish tumor recurrence from other benign lesions , initial hyperechogenicity might be a feature of a non - tumorous condition .
ct may be helpful to differentiate the 2 entities because of the hyperattenuation of the charcoal granuloma .
following imaging workup , us - guided fine - needle aspiration biopsy is generally indicated for the evaluation of recurrent nodal metastases . however , as in our patient , charcoal granuloma with charcoal materials may not be diagnosed properly with fine needle aspiration cytology alone .
cases suspected of charcoal granuloma are better diagnosed by us - guided core - needle biopsy . in summary , we reported us , ct , and fdg - pet / ct findings of a charcoal granuloma in the neck after excision of a metastatic lymph node resulting from breast cancer .
these findings included an irregular , heterogeneous hyperechoic nodule with subsequent decreased echogenicity and development of posterior acoustic shadowing on us , a high attenuating nodule on precontrast ct and a hypermetabolic lesion on pet / ct .
charcoal granulomas should be included in the differential diagnosis of incidentally detected mass - like lesions at the site of presurgical localization using charcoal . | an 18-year - old girl presented with multiple itchy hyperpigmented papules and plaques , along with tense blisters over the lower limbs and buttocks for last 3 months .
these papules , plaques , and bullae were mostly localized to preexisting scars .
the histopathological findings from papule and bulla were consistent with lichen planus ( lp ) and bullous pemphigoid , respectively .
direct immunofluorescence ( dif ) of perilesional skin around bulla showed linear deposition of igg and c3 .
considering clinical , histopathological and dif findings , diagnosis of lp pemphigoides ( lpp ) was made .
the preferential localization of lpp lesions over preexisting scars was a very interesting finding in our case an extremely rare instance of the isotopic phenomenon . |
.
the baff / april axis plays an important role in the pathogenesis of various autoimmune diseases including sle ; inhibitors of this axis are therefore useful in treatment of these disordersresults from bliss-52 and bliss-76 trials have shown belimumab to be efficacious in treating patients with sleserious adverse effects noted with belimumab such as progressive multifocal leukoencephalopathy , depression , suicidal tendency , and malignancy are to be watched for .
the baff / april axis plays an important role in the pathogenesis of various autoimmune diseases including sle ; inhibitors of this axis are therefore useful in treatment of these disorders results from bliss-52 and bliss-76 trials have shown belimumab to be efficacious in treating patients with sle serious adverse effects noted with belimumab such as progressive multifocal leukoencephalopathy , depression , suicidal tendency , and malignancy are to be watched for .
.
the baff / april axis plays an important role in the pathogenesis of various autoimmune diseases including sle ; inhibitors of this axis are therefore useful in treatment of these disordersresults from bliss-52 and bliss-76 trials have shown belimumab to be efficacious in treating patients with sleserious adverse effects noted with belimumab such as progressive multifocal leukoencephalopathy , depression , suicidal tendency , and malignancy are to be watched for .
the baff / april axis plays an important role in the pathogenesis of various autoimmune diseases including sle ; inhibitors of this axis are therefore useful in treatment of these disorders results from bliss-52 and bliss-76 trials have shown belimumab to be efficacious in treating patients with sle serious adverse effects noted with belimumab such as progressive multifocal leukoencephalopathy , depression , suicidal tendency , and malignancy are to be watched for . cementoblastoma in the current world health organization classification of odontogenic tumor , is in the category of tumors of mesenchyme and/or odontogenic ectomesenchyme with or without odontogenic epithelium .
it generally occurs in young persons , comprises < 1 - 6.2% of all odontogenic tumor and is characterized as being attached to the roots , most frequently associated with first permanent molar .
the majority of these tumor are radiopaque , but radiolucent tumor may occur in rare instances .
histologically , it presents as a well - circumscribed tumor composed of cementum like tissue surrounded by a fibrous capsule .
this was a case report of a 16-year - old male patient who reported to the department of oral medicine , gdc , ahmedabad with the chief complaint of swelling and mild pain in the right side of the jaw since 6 months , with a history of extraction of 46 because of pain before 6 months in a private dental clinic . on clinical examination , there was diffuse bony hard swelling present in the right body of mandible with normal overlying skin .
intra - orally , there was diffuse bony hard swelling in 46 region with normal overlying mucosa and expanded buccal and lingual cortical plates [ figure 1 ] .
adjacent teeth were immobile and undisplaced . radiological examination revealed a well - defined round radiopacity with radiolucent rim in the right body of mandible [ figure 2 ] .
he had pre - extraction intraoral periapical radiography ( iopa ) radiograph of 46 region , which showed a well - defined radiopacity surrounded by radiolucent rim attached to roots of 46 [ figure 3 ] .
the typical radiographic presentation of radiopacity attached to root with radiolucent rim suggested of benign cementoblastoma with differential diagnosis of hypercementosis , osteoblastoma , periapical cemental dysplasia and condensing osteitis .
histopathologically it showed a well - circumscribed tumor composed of cementum like tissue surrounded by a fibrous capsule [ figure 4 ] .
intra - oral view showing diffuse swelling in right lower quadrant with missing 46 and expansion of buccal and lingual cortical plates right lateral oblique of mandible showing well defined radiopacity surrounded by radiolucent zone in body of mandible with missing 46 pre - extraction radiograph showing radiopaque lesion attached to roots of 46 microphotograph showing sheet of cementum like tissue with intervening loose fibrovascular connective tissue stroma
the male to female ratio of the prevalence has been reported to be 2.1:1 with a mean age of 20.7 years .
benign cementoblastoma is also reported in the maxillary sinus and associated with deciduous and unerupted permanent tooth and multiple teeth . clinical sign and
the radiographic appearance of benign cementoblastoma is well - defined radiopacity with radiolucent zone . though it is a benign tumor , but
some cases reported in the literature exhibited signs of local aggressiveness and destruction , including bony expansion , erosion of cortical plates , displacement of adjacent teeth , maxillary sinus involvement , invasion of the pulp chamber and root canals and extension to and incorporation of adjacent teeth .
expansion , pain and erosion or perforation of bony cortex were seen in a higher percentage of recurrent tumors , but were also seen in non - recurrent tumors .
the differential diagnosis for a periapical radiopacity include cementoma , osteoblastoma , periapical cemental dysplasia , condensing osteitis and hypercementosis .
histologically , this tumor presents sheets of cementum like tissue , which may contain a large number of reversal lines with active cementoblasts .
cementoblastoma and osteoblastoma are essentially identical histologically and the only distinguishing feature is attachment of cementoblastoma to the root of a tooth .
histologically , the cementoblast in cementoblastoma may be plump with pleomorphic and hyperchromatic nuclei , however , mitotic figures are not seen in cementoblastoma .
in contrast to osteoblastoma , the cementoblastoma is an odontogenic tumor that recapitulates cementum deposition similar to that during root formation in the later stages of odontogenesis .
furthermore , the cementoblastoma is continuous with the cemental layer of the apical third of the tooth root and remains separated from bone by a continuation of the periodontal ligament , all of which supports an odontogenic origin . whereas osteoblastoma arises in the medullary cavity of bones .
the treatment of choice is complete removal of the lesion with extraction of associated tooth , followed by thorough curettage and peripheral ostectomy .
cases have been also reported of endodontically preserving the tooth while surgical removal of benign cementoblastoma is done .
we presented a rare case of benign cementoblastoma in mandible and it should be considered in differential diagnosis of bony swellings of mandible . when extraction is attempted in such cases leaving the lesion behind
makes the clinical diagnosis difficult . though the patient had pre - extraction records , which helped in formulating the diagnosis . | belimumab is the only approved biological agent for the treatment of systemic lupus erythematosus ( sle ) .
it is a fully humanized igg1 monoclonal antibody directed against soluble b lymphocyte stimulator ( blys ) .
it is indicated as an add - on therapy for the treatment of adult patients with active , autoantibody - positive sle , who are receiving standard therapy .
belimumab is generally well - tolerated , common adverse effects include infections , infusion reactions , hypersensitivity , headache , nausea , and fatigue . psychiatric events including suicidal tendency , progressive multifocal leukoencephalopathy and malignancies too have been reported .
apart from sle , the drug is also being tried for other autoimmune disorders . |
liquid - based cytology ( lbc ) as a technique was introduced and tried on pap smears .
thereafter , this technique was performed for non - gynecological cytology , including conventional fine needle aspiration cytology ( fnac ) , guided fnac and fluid cytology .
the thin - prep ( tp ) smears were consistently devoid of obscuring elements and the cells were adequately preserved and evenly dispersed .
pitfalls noticed as cytomorphologic alterations in lbcs are small - sized cell clusters , with more single cells than sheets .
the cells are generally smaller , occasionally show spindling , chromatin detail is attenuated , nucleoli are more prominent , intranuclear inclusion is difficult to visualize , decrease of extracellular particles is seen , there is a decrease in the number of small mononuclear cells / red blood cells / myoepithelial cells and the background matrix is altered
. the pathologist should be cautious in interpreting fine needle aspirations prepared using tp if that is the only methodology employed .
the advantages of lbc , independent of diagnostic accuracy and morphology , have been well described .
although studies on lbc are documented for breast , non - gynecological specimen , thyroid , salivary gland and soft tissues , the authors supplemented bone lesions to all the above in their study .
lbc in fnac smears can be performed on aspirates from different organs like salivary gland , thyroid , lymph nodes , breast , bone and soft tissue and other usual and guided fnac samples .
processing of the samples is carried out using the tp kit , marketed by cytec corporation , malborough , ma , usa . in a comparison study , usually two passes are made .
the first pass is performed for the conventional preparation ( cp ) and the second pass is for the tp preparation .
tp smears are superior to cp smears in fnac ; this fact can be proved by performing a wilcoxon 's signed rank test , p value < 0.001 .
tp preparations are nowadays used in some sophisticated investigations like immunohistochemistry studies in breast lesions , non - hodgkin 's lymphoma ( nhl ) and hodgkin 's lymphoma ( hl ) in effusions , analysis of proliferating cell nuclear antigen ( pcna ) , cell blocks from scraping of cytosmears and comparison with conventional cell blocks .
the aim of our study was to determine the efficacy of lbc technology over conventional smear methods in fnac samples .
the study was performed on patients attending the cytology outpatient department ( opd ) of the hospital over a period of 2 years .
, samples were processed using the liquiprep processing kit and a special kit marketed by cytec corporation .
processing was performed manually in three steps , i.e. collection , concentration and cellular encapsulation .
the lp cytology processing kit contains a preservative solution and a cellular base . using the lbc processing kit ,
, the samples were pushed into a preservative solution , approximately three - times the volume of the sample , and left as such for at least 1 h. this is followed by the next step , i.e. concentration , where the preserved sample is centrifuged at 3000 rpm for 30 min . for cystic fluids , the supernatant fluid is discarded and , after adding the cleaning solution , it is again centrifuged for 15 min to increase its concentration .
a pellet is produced , the supernatant fluid is discarded and a base solution of about 50 microl is added to the pellet to make a thin , homogenous suspension .
this suspension was placed on an ethanol - cleaned glass slide and two circular smears of 1 cm diameter were made .
the second option , which uses a cellular base solution , was used in certain cases , particularly if the sample contains necrotic substance or mucin .
about 4 ml of cleansing solution was used in selected cases , along with the preservative solution , and the processing was performed in a similar fashion .
two passes were performed , the first pass was for cp and the second pass yielded material for the tp preparation , which was processed by the tp kit marketed by cytcec corporation .
different stains were used for staining the cp and lp smears , like he , pap and diff quick .
the representative cs and lb preparations were compared by a semiquantitative scoring system using several criteria [ table 1 ] .
the concentration method used in the procedure was useful in the diagnosis of cystic lesions like aneurysmal bone cysts , warthin 's tumor of salivary glands and metastatic carcinoma .
statistical analysis was performed using the wilcoxon signed rank test on the spss program ( chicago , il , usa ) .
every cytological diagnosis was recorded and tabulated and compiled to yield the p - value .
a total of 110 cases were studied , which were distributed among 30 cases of breast ( 10 fibroadenoma , four mastitis and 12 ductal carcinoma ) , 40 cases of lymph node ( 20 reactive hyperplasia , 12 granulomatous lymphadenitis , three lymphoma and five metastatic carcinoma ) , 10 cases of salivary gland ( two sialadenitis , five mixed tumor , one warthin 's tumor and two mucoepidermoid carcinoma ) , 18 cases of thyroid ( 10 colloid goiter , four thyroiditis and four carcinoma cases ) and 12 cases of soft tissue and bone lesions ( five benign and seven malignant ) .
comparison between cytological features in lbc and cs was carried out using the wilcoxon signed rank test separately for each organ in a tabular form , as shown in table 2 . in our study , in two cases of pleomorphic adenoma , one case of warthin 's tumor and two cases of colloid goiter , a support of cs was needed due to pitfalls in lbc described above .
wilcoxon signed rank test statistics of lbc versus cp for breast cases , fibroadenoma , mastitis and ductal carcinoma were better diagnosed on lbc preparations due to clarity of the nuclear features [ figure 1 ] .
comparison data for lymph nodes suggested that the rs cells were more easily diagnosed on lbc smears due to monolayering .
liquiprep cytosmear of the breast showing pleomorphic - looking ductal epithelial cells in a cluster and acinar pattern in a clear background in case of salivary gland lesions , the mixed salivary tumor was better visualized in the conventional smear because of preservation of the stromal component .
cystic neoplasms of the salivary glands were better appreciated in lbc smears because of concentration . in sialoadenitis ,
lbcs were not very useful in goiter and infectious lesions , but they proved very useful in neoplastic lesions [ figure 2 ] . in lytic bone lesions like aneurismal bone cyst ( abc ) , giant cell tumor ( gct ) and metastasis ,
liquiprep cytosmear showing papillary carcinoma of the thyroid in a clear background with nuclear grooving the study conducted by mygdakos et al .
lbc was favored over cs for evaluating gynecological specimens , which was approved by the fda in 1996 .
thereafter , lbc has been found to be useful in non - gynecological cytologic specimens like fnac and body fluids , both small and large volume , which have drawn variable conclusions . in conventional fnac
smears , cells are admixed with debris , blood and exudates , which make the interpretation difficult .
there is suboptimal preservation of cells and cellular obscuring by unwanted material , leading to a high proportion of cases reported as inadequate or unsatisfactory for assessment . to overcome this problem , the lbc technique was introduced , which preserves cells in a liquid medium and removes all debris , blood and exudates , either by filtration or density gradient interpretation
, there is even distribution of cell material , lack of obscuring factors , no drying artifact and monolayering . in our study , fnac was performed from various sites like breast , lymph node , salivary glands , thyroid and bone and soft tissue . in fibroadenoma breasts , although stromal fragments were lost , lbc proved to be useful in the diagnosis based on visualization of ductal aggregates and bipolar cells . for duct carcinomas ,
the wilcoxon signed ranked test for thyroid lesions [ table 2 ] , lbc was not useful in goiter and infectious lesions .
lbc was not useful in cystic neoplasms like warthin 's tumor , mucoepidermoid and adenoid cystic carcinoma and comparable to the study by parfitt et al . for bone and soft tissues , good results were obtained in lbc because of background clearing and concentration phenomenon . in all lesions of the lymph node ,
lbc proved to be a better option . because of the pitfalls of lbc , as described above , in some selected cases of granulomatous lymphadenitis ( lacking epitheloid cells ) , mucinous tumors ( lack of mucin ) , colloid goiter ( thin colloid fragmented ) and pleomorphic adenomas of the salivary gland ( myxoid background poor and present in droplets ) , a support on cs may prove useful .
as observed by the authors , lbc of bony lesions do not require support of cs .
tp preparations are superior to cps with regard to clear background , monolayer cell preparation and cell preservation .
it is easier and less time consuming to screen and interpret tp preparations because the cells are limited to smaller areas on clear backgrounds , with excellent cellular preservation .
however , tp preparations are more expensive than cp and require some experience for interpretation .
lbc is strongly advocated in the best interest of public health , by improving the quality of the sample and reducing the likelihood of false - negative cytology results . eligible subjects were healthy individuals who voluntarily received routine comprehensive health check - ups , including computed tomography ( ct ) scan analyses of abdominal adipose tissue , from january to december 2008 at the seoul national university hospital healthcare system gangnam center .
data were collected through a questionnaire , anthropometric measurements , blood samples , and ct scans .
detailed descriptions of the method used for the measurement of abdominal adipose tissue have been published previously ( 8) . in brief , a 5-mm thick umbilical level abdominal section from 16-detector row ct imaging of the abdomen was obtained .
the cross - sectional area ( cm ) of the abdominal fat was calculated using rapidia 2.8 ct software ( infinitt , seoul , korea ) .
the vat area was defined as intra - abdominal fat bound by parietal peritoneum or transversalis fascia , and the sat area was calculated by subtracting the vat area from the total adipose tissue area . because of the high colinearity between sat and vat , adjustments to the regression model or stratification of vat are insufficient to control for the effects of vat on sat .
the ratio between sat and vat ( sat / vat ratio [ svr ] ) was therefore calculated and used in the current study .
mets was defined according to the modified national cholesterol education program adult treatment panel iii ( 9 ) and asia - pacific abdominal obesity criterion ( 10 ) . means with 95% ci and
numbers of participants ( % ) with metabolic risk factors were compared across the svr quintiles .
we used multivariate logistic regressions to evaluate the association between mets and svr after adjusting for age , smoking , alcohol consumption , bmi , menopausal status , and hormonal replacement therapy ( women only ) .
data are expressed as odds ratio ( or ) [ 95% ci ] ; and p for trend .
detailed descriptions of the method used for the measurement of abdominal adipose tissue have been published previously ( 8) . in brief , a 5-mm thick umbilical level abdominal section from 16-detector row ct imaging of the abdomen was obtained .
the cross - sectional area ( cm ) of the abdominal fat was calculated using rapidia 2.8 ct software ( infinitt , seoul , korea ) .
the vat area was defined as intra - abdominal fat bound by parietal peritoneum or transversalis fascia , and the sat area was calculated by subtracting the vat area from the total adipose tissue area . because of the high colinearity between sat and vat , adjustments to the regression model or stratification of vat are insufficient to control for the effects of vat on sat .
the ratio between sat and vat ( sat / vat ratio [ svr ] ) was therefore calculated and used in the current study .
mets was defined according to the modified national cholesterol education program adult treatment panel iii ( 9 ) and asia - pacific abdominal obesity criterion ( 10 ) .
means with 95% ci and numbers of participants ( % ) with metabolic risk factors were compared across the svr quintiles .
we used multivariate logistic regressions to evaluate the association between mets and svr after adjusting for age , smoking , alcohol consumption , bmi , menopausal status , and hormonal replacement therapy ( women only ) .
data are expressed as odds ratio ( or ) [ 95% ci ] ; and p for trend .
forty percent of men and 25% of women had elevated blood pressure , 31% of men and 15% of women had elevated fasting glucose , and 25% of men and 14% of women had mets .
the mean sat and vat areas were 139.8 cm and 134.4 cm in men and 173.1 cm and 80.0 cm in women , respectively .
the prevalence of elevated fasting glucose , elevated triglycerides ( tgs ) , and reduced hdl cholesterol ( hdl - c ) significantly decreased as the svr quintile increased ( 26 , 16 , and 37% in quintile 1 vs. 5 , 4 , and 11% in quintile 5 , respectively ; all p for linear trend < 0.05 ) robust linear decreases in these risk factors were also seen among men ( supplementary table 1b ) .
ors of mets significantly decreased across all 5 quintiles of the svr after adjusting for covariates in both men ( or 1.0 for quintile 1 vs. 0.5 in quintile 5 ; 95% ci 0.30.7 for quintile 5 ; p for linear trend < 0.01 ) and women ( or 1.0 for quintile 1 vs. 0.2 in quintile 5 ; 95% ci 0.10.5 for quintile 5 ; p for linear trend < 0.01 ) ( table 1 ) .
unadjusted and adjusted ors and 95% ci from logistic regressions predicting the likelihood of mets for each of the svr quintiles * adjusted ors take into consideration age , smoking , alcohol consumption , bmi , and menopausal status , hormone replacement therapy ( women only ) .
ors of mets significantly decreased across all 5 quintiles of the svr after adjusting for covariates in both men ( or 1.0 for quintile 1 vs. 0.5 in quintile 5 ; 95% ci 0.30.7 for quintile 5 ; p for linear trend < 0.01 ) and women ( or 1.0 for quintile 1 vs. 0.2 in quintile 5 ; 95% ci 0.10.5 for quintile 5 ; p for linear trend < 0.01 ) ( table 1 ) .
unadjusted and adjusted ors and 95% ci from logistic regressions predicting the likelihood of mets for each of the svr quintiles * adjusted ors take into consideration age , smoking , alcohol consumption , bmi , and menopausal status , hormone replacement therapy ( women only ) .
the purpose of this study was to assess the cross - sectional correlation of abdominal sat with mets in a korean study population .
we found that as the svr increased , mets components such as reduced hdl - c , elevated tgs , and elevated fasting glucose decreased in both sexes . furthermore , this relationship was maintained even after the adjustment for a variety of variables .
small adipocytes in sat play the role of powerful buffers , uptaking circulating free fatty acids and tgs in the postprandial period .
but if they lose their function in adipogenesis or fat storage capacity in sat , fat begins to accumulate in tissues not suited for lipid storage such as vat .
peroxisome proliferatoractivated receptor- ( ppar- ) agonists stimulate the differentiation of adipocytes , resulting in increased accumulation capacity in sat ( 12 ) .
the ppar- coactivator 1 mrna expression is significantly higher in subcutaneous fat than in omental fat ( 13).this mechanism explained the finding that treatment with thiazolidinedione improves insulin sensitivity ( 6 ) . vat and sat differ in cytokine secretion profile such as leptin , adiponectin , interleukin-6 , interleukin-8 , plasminogen activator inhibitor 1 , and angiotensin , which may play some role in the development of mets ( 14,15 ) .
longitudinal associations of vat / sat change with mets , and with leptin and/or adiponectin level should be the focus of future studies .
gluteofemoral sat , which may act as a confounding factor , was not considered in the study .
however , the current study included the analysis of a large dataset of ct - measured sat and vat .
moreover , the study population was generally healthy , which implies that the results can be applied to the general korean population . | background : liquid - based cytology ( lbc ) is fast becoming a useful method in evaluating both gynecological and non - gynecological preparations , including fine needle aspiration ( fna ) cytology
. even distribution of cells , decreasing obscuring background elements like blood and mucus , well preserved nuclear and cytoplasmic details and rapid fixation helps in better visualization of cells.aim:this study was conducted to asses the diagnostic accuracy of liquid based cytology versus conventional smears in fine needle aspiration samples.study design : in this prospective study , we had 110 cases , including 30 cases of breast , 40 of lymph node , 10 of salivary glands , 18 of thyroid and 12 of bone and soft tissue . in each case , two passes were performed .
the first pass was for conventional preparation ( cp ) and the second pass yielded material for thin - prep ( tp ) preparation .
both cp and tp smears were compared for cellularity , background blood and necrotic cell debris , cell architecture , informative background , presence of a monolayer of cells and nuclear and cytoplasmic details by a semiquantitative scoring system .
wilcoxon 's signed rank test on an spss program was used for statistical analysis.results:diagnostic accuracy was better in lbc smears compared with cp smears due to lack of background debris and better cell morphology , which was performed according to wilcoxon 's signed rank test , yielding a p - value of < 0.001 .
however , in some cases , because of a decrease in cell size , clustering and altered background in lbc , a support of cp was essential.conclusion:lbc performed on fna samples can be a simple and valuable technique . only in few selected cases , where background factor is an essential diagnostic clue , a combination of both cp and tp is necessary . |
the study protocol was approved by the ethics committee of the ministry of health and the school boards .
a total number of 30 clusters including 7,493 girls ( aged 620 years ) were selected .
students with a history of acute or chronic diseases or any chronic medication use were excluded . in all subjects , height , weight , and pubertal stages
general physicians determined pubertal stages under the supervision of specialists by visual inspection and palpation , using the criteria and definitions described by marshall and tanner.10 accordingly , breast stages 15 were determined by both inspection and palpation .
breast stage b2 corresponded to the breast bud stage with palpable glandular breast tissue and elevation of the papilla , stage b3 with further enlargement of the breast and areola with no separation of the contours , stage b4 where the areola and papilla form a secondary mound above the level of the breast , and stage b5 was assigned in case of the mature breast with projection of the papilla only .
the onset of puberty was measured as the age at breast development at tanner stage 2 ( b2 ) .
stage ph2 was assigned with the presence of long , slightly pigmented hair ( straight or curled ) .
participants were grouped by 1-year age intervals , which extended from the day of their birthday to the day before the next birthday . for comparison of different areas
, we used the national classification of the provinces according to their climate , ethnicity of the population , geographic locations , and socioeconomic variables .
classification of iranian provinces to regions according to climate , geographic location , ethnicity and socioeconomic status we estimated the mean of the time of men - arche , b2-b5 and ph2-ph5 stages .
statistical analysis was done using spss version 15 ( spss , chicago , il ) .
analysis of variance ( anova ) and post - hoc tests were used to compare the mean ages of menarche , pubarche , and telarche in different regions .
we estimated the mean of the time of men - arche , b2-b5 and ph2-ph5 stages .
statistical analysis was done using spss version 15 ( spss , chicago , il ) .
analysis of variance ( anova ) and post - hoc tests were used to compare the mean ages of menarche , pubarche , and telarche in different regions .
we estimated the mean of the time of men - arche , b2-b5 and ph2-ph5 stages .
statistical analysis was done using spss version 15 ( spss , chicago , il ) .
analysis of variance ( anova ) and post - hoc tests were used to compare the mean ages of menarche , pubarche , and telarche in different regions .
tehran had the lowest mean age of menarche , being 11.99 1.35 years , which was significantly lower than other 10 regions .
the second region with lowest age at menarche was fars ( 12.40 1.27 years ) .
the mean age at b2 stage of breast development was significantly lower in ghazvin - zanjan region ( 8.97 1.45 years ) followed by the region consisting of khorasan jonobi and sistan balochestan ( 10.11 1.61 years ; p = 0.003 ) , boshehr - khouzestan region ( 10.05 1.21 years ; p = 0.01 ) , tehran - relatd cities ( 10.46 1.67 years ; p < 0.0001 ) , fars ( 11.01 1.88 years ; p < 0.0001 ) and kordestan - lorestan - ilam region ( 9.94 1.25 years ; p = 0.02 ) . in fars region , the mean age at b2 stage of breast development ( 11.01 1.88 years ) was higher than other regions except for mazendran - guilan and tehran - related cities regions .
figure 2 shows the mean age at b1-b5 of breast development in different regions in iran .
mean age ( years ) at menarche in different regions mean age ( years ) at various stages of telarche in different regions in iran the mean age at ph2 stage of pubarche in kordestan - lorestan - ilam region ( 10.70 1.23 years ) was significantly higher than khorasan jonobi - sistan balochestan region ( p < 0.0001 ) , boshehr - khozestan region ( p < 0.0001 ) , tehran - relatd cities ( p < 0.0001 ) , chaharmahal - kerman - isfahan region ( p < 0.0001 ) , ghazvin - zanjan region ( p < 0.0001 ) , and boshehr - khozestan region ( p < 0.0001 ) .
the mean age at ph2-ph5 of pubic hair development in different geographic locations of iran is presented in figure 3 .
to the best of our knowledge , this is the first study of its kind to compare the pubertal stages in different geographic locations in a country .
it should be acknowledged that such comparison is not feasible in many countries with small population and no significant diversity in climate and other geographic characteristics .
we found that the mean age of menarche was significantly lower in tehran than all other regions .
tehran province , including the metropolitan city of tehran , is located in central part of the country with multiethnic population .
not only multi - ethnicity but also higher socioeconomic status and more industrialized lifestyle habits in tehran might be associated with earlier menarche age . in another study
the mean age of menarche was 12.68 years in tehran in 2004.11 in our study conducted 2 years later , this age decreased to 11.99 1.35 years . decreasing trend of menarche age
is observed in many developed and developing countries.1213 it might be attributed to changes in socioeconomic status of people14 and increasing rate of obesity.12 ethnicity is documented as a factor that describes some international varieties of puberty age.1516 in the current study , the mean age at menarche and ph2 was higher in girls with kurd ethnicity than in other ethnicities .
kurd ethnicity is the third most prevalent ethnicity in iran after persian and azeri ethnicities .
it can be suggested that the higher age of puberty in girls of this region might be attributed to their ethnicity as well as the cold climate and their high altitude mountainous region of living .
in addition to ethnicity , the geographic characteristics may influence the age of puberty . in our study , the lowest mean age at b2
was documented in zanjan - ghazvin region , with mediterranean weather and spring rains , and population of azeri ethnicity .
the earlier telarche in this region in comparison to other regions with azeri population might be attributed to environmental factors .
premature telarche is suggested to be associated with endocrine disrupting chemicals found in pesticides and plastics , with possible estrogenic and androgenic activities.1718 further investigations on environmental pollution might be necessary in this regard .
the considerably high prevalence of overweight among young iranian children19 might decrease the age of puberty in the near future .
the main limitation of this study was its cross sectional nature , and as a result , casualty can not be ascertained .
in addition , we considered the prominent ethnicity in each region ; but some participants might have other ethnicities .
we found significant differences in the age of pubertal stages of girls living in various regions with different ethnicity and geographic characteristics .
considering the impact of pubertal age on general health , more studies should be done about the lifestyle and environmental factors affecting the onset of puberty .
mem participated in the design and conducting the study ; ar participated in the design and conducting the study and helped to draft the manuscript ; rk drafted and edited the manuscript ; pm conducted the statistical analyses and helped to draft the manuscript ; ss participated in conducting the study ; ga participated in the design and conducting the study ; ha participated in conducting the study ; np participated in conducting the study ; sk in conducting the study ; pp helped to draft and edit the manuscript ; as participated in conducting the study . the anterior choroidal artery ( achoa ) supplies several important structures including the optic tract , geniculate body , posterior limb of internal capsule , globus pallidus , cerebral peduncle , uncus , amygdala , ventrolateral nucleus of thalamus , and choroid plexus .
the vascular injury of achoa may result in severe neurological deficits such as hemiplegia , hemianesthesia , hemianopsia , decreased consciousness , and extrapyramidal symptoms .
therefore , complete knowledge about vascular anatomy of achoa is very important before surgical or endovascular manipulations .
although several detailed reports about anatomical variations of achoa have been reported , transposition of achoa as to posterior communicating artery ( pcoma ) origin was demonstrated only in 3 reports2 - 4 ) .
we report a case with ruptured cerebral aneurysm arising from pcoma , which originated from the internal carotid artery ( ica ) distal to the origin of achoa .
a 40-year - old woman presented with sudden onset of headache . computed tomography ( ct ) revealed subarachnoid hemorrhage ( sah ) which was slightly predominant in the left basal and sylvian cisterns .
two small aneurysms arose from both distal supraclinoid ica , these were observed on ct angiograms ( fig .
cerebral angiography demonstrated a small aneurysm arising from left fetal type of pcoma with suspicion of rupture and the other small aneurysm originating from right pcoma or perforating branch of ica . in this study , left achoa originated from ica proximal to the origin of fetal type of pcoma ( fig .
achoa usually arises from the posterolateral wall of ica , a few mm distal to the origin of pcoma , and it provides blood supply to the important neural structures around crural and ambient cisterns .
the achoa can have several variations in the course , size , number , branching pattern , and brain region supplied by this artery1 ) . among them
, there have been several anomalies related to the origin of achoa including transposition of achoa and pcoma , origin from pcoma , origin from junction of ica and pcoma , and origin from middle cerebral artery4,5 ) .
the clinical findings of transposed achoa cases including present one are summarized in table 1 .
all of the transpositions of achoa occurred in the left side . among all the cases ,
our case showed sah due to ruptured aneurysm arising from fetal type of pcoma among multiple cerebral aneurysms founded and achoa originating from ica proximal to pcoma .
typically , achoa runs posteromedially within the crural cistern and continues ambient cistern to enter the choroidal fissure .
the proximal portion of this artery is cisternal segment and following portion becomes intraventricular segment1 ) .
the transposed achoa in the previous cases including ours did not seem to affect the vascular territory or course despite of unusual vascular arrangements .
the achoa can be exposed or manipulated in surgical approaches into the pathological lesions around temporal lobe or involving supraclinoid ica .
although the achoa aneurysms accounting for 4% of all cerebral aneurysms are not so common , vascular injury of achoa with small caliber supplying the eloquent areas may be critical because of the possibility of significant morbidity and mortality . and
, another caution is that achoa do not have significant collateral flows despite a potential anastomosis with posterior choroidal artery3 ) .
we tried to preserve pcoma itself and all perforating branches arising from it because of the possibility to compromise the collateral flows of fetal type pcoma .
it is notable that careful review of the preoperative angiograms helps to find unexpected vascular anomalies which may impose an additional surgical risk . | background : this study aimed to compare the timing of puberty between various geographic locations and different ethnicities.methods:this national survey was conducted in 20 provinces in iran .
healthy iranian girls were selected from public schools using cluster random sampling .
a total number of 30 clusters including 7493 girls , aged 6.020 years , were selected . in order to compare different areas , the national classification of the provinces based on climate , ethnicity , geographic locations , and socioeconomic variables were used .
accordingly , there are 11 regions in iran .
analysis of variance was used to compare the mean ages of menarche , pubarche , and thelarche in different regions.results:tehranian girls , with 11.99 1.35 years ( mean sd ) , had the lowest age of menarche which was statistically significantly .
the second region with lowest age at menarche was fars ( 12.40 1.27 years ) .
the mean age at breast bud stage ( b2 ) was significantly lower in ghazvin - zanjan region ( 8.97 1.45 years ) . in fars region ,
the mean age at b2 stage of breast development ( 11.01 1.88 years ) was higher than other regions except for mazendran - guilan and tehran - related cities .
the mean age at public hair development at tanner stage 2 ( ph2 ) in kordestan - lorestan - ilam region ( 10.70 1.23 years ) was significantly higher than other regions.conclusions:we found significant differences in the age of pubertal stages of girls living in various regions with different ethnicity and geographic characteristics .
considering the impact of pubertal age on general health , more studies should be done about the lifestyle and environmental factors affecting the onset of puberty . |
in recent years , video - assisted intubation devices have flooded theaters , and , to a lesser extent , are also being used in icus . but can such new technology really contribute to making tracheal intubation in the icu less life - threatening , or even more life - saving ? noppens and colleagues , in a study just published in critical care , evaluated the efficacy of the c - mac video laryngoscope for endotracheal intubation in an icu managed by anesthesiologists and compared the results to those obtained during a previous period when conventional direct laryngoscopy was used . in patients with at least one predictor for difficult intubation , poor glottic views ( cormack and lehane grades iii and iv )
were reduced from 38% to 19% , and intubation success on the first attempt increased from 56% with direct laryngoscopy to 79% when the c - mac video laryngoscope was used . even in all patients , the incidence of at least two intubation attempts
though this was not statistically significant , halving this incidence may have substantial clinical impact .
although the overall intubation success was 100% in either group , successful tube placement in the trachea on the first attempt is crucial in icu patients with poor tolerance to apnea .
after implementation of a video laryngoscope , however , intubations were more often performed by less experienced physicians .
even if one reason may be that younger physicians are more open to the newer technique , it must be guaranteed that physicians ' experience is not substituted solely by technical equipment . similarly , the use of endoscopic intubation was reduced substantially in the intervention phase ( 4% versus 13% ) , so experience with such devices may decrease , causing problems if video laryngoscopy is not possible or is contraindicated .
video laryngoscopy for every patient ? sparing such devices for the really difficult cases ? to answer these questions it needs to be emphasized that all video - assisted and optical intubation devices have different view angles , thus producing different images with particular distortions , and even experts in ' old - fashioned ' airway management need a substantial level of training with a certain device before using it safely and successfully in critical situations and patients .
video laryngoscopes , regardless of a particular brand or device , can not be used intuitively - they require expert skills and routines to be tamed and turned into life - saving tools .
tube placement can turn out to be difficult and even impossible , despite the excellent glottic exposure video laryngoscopes most often provide .
the reason is that the operator 's eye is no longer in a straight - optical and anatomical - line with the larynx , but at the tip of the laryngoscope blade .
optical and anatomical axes divert , and tube placement is sometimes difficult or fails . becoming alert to these potential problems associated with video laryngoscopy and constantly improving one 's technical skills to overcome such pitfalls can only be achieved when such devices are used on a daily basis and in routine patients .
apart from the primary aim of the study - evaluating video laryngoscopy - it needs to be highlighted that noppens and colleagues routinely used capnography to verify correct tube placement after intubation . capnography has become a mandatory requirement in any operating room , but its use in the icu seems still to be somewhat dowdy . as of 2010 , only 25% of icus in the uk and the republic of ireland used capnography to confirm correct tube placement after intubation . such refusal may produce catastrophic outcomes : data collected from all uk national health service hospitals over a period of one year in 2008/2009 revealed that 61% of adverse events during airway management in the icu resulted in death or permanent brain damage . in 74% of such fatal cases no capnography was used !
doubtless , video laryngoscopy is a helpful tool in the hands of experienced physicians , but should not be considered a bailout procedure for beginners in airway management . and
as attractive and simple video laryngoscopy appears , we must always remind ourselves to maintain our skills in endoscopic intubation .
both authors receive continuous material support for clinical studies from karl storz gmbh & co. kg , the manufacturer of the c - mac video laryngoscope . in gipuzkoa , 27 human listeriosis episodes were reported during january 2013february 2014 . all cases produced sepsis in the patients , except 1 case that produced diarrheal disease in a 34-year - old parturient woman who had undergone a splenectomy .
eleven episodes ( 40.7% ) occurred in pregnant or parturient women , and 8 of the children of these patients were affected : 5 newborns ( 4 of them premature infants ) became ill , 2 pregnancies ended in miscarriage , and 1 infant was stillborn .
( for this study , a pregnancy - associated episode was counted only once , whether the causative strain was isolated from mother , child , or both . ) ten cases ( 37.0% ) occurred among the elderly ( > 70 years of age ) and 6 ( 22.2% ) in adults 4560 years of age . of the 6 patients in the 45- to 60-year age group ,
a total of 29 human l. monocytogenes isolates were available for microbiological characterization : 22 from blood , 3 from placental membranes , 2 from cerebrospinal fluid , and 1 each from stool and dermal exudate .
serotypes were established by agglutination ( listeria - o - antisera , difco ; bd diagnostics , sparks , md , usa ) and by multiplex - pcr ( 4 ) .
the predominant serotypes identified were 1/2b ( n = 17 , 58.6% ) and 4b ( n = 8 , 27.6% ) ; 4 isolates were serotype 1/2a ( 13.8% ) .
pulsed - field gel electrophoresis ( pfge ) was performed by using the restriction endonucleases smai and asci ( 5 ) .
the sts of l. monocytogenes isolates were determined by using the multilocus sequence typing ( mlst ) primers and conditions described by the pasteur institute ( 6 ) .
mlst showed that 16 of 17 serotype 1/2b isolates we tested were st87 ; the remaining serotype 1/2b isolate was st3 .
only 2 human l. monocytogenes st87 serotype 1/2b isolates , both from japan , were listed in the pasteur institute mlst database ( 6 ) .
further , pfge showed 2 large clusters within serotype 1/2b , and epidemiologic research detected 2 main outbreaks .
all listeriosis episodes during the study period that were caused by isolate types other than serotype 1/2b and st87 were sporadic .
in the first case cluster ( first outbreak ) , 5 episodes were detected during august
all isolates from 1 woman at 28 weeks gestation , 1 parturient woman and her newborn child , and 2 newborn twins ( no samples from the mother were available ) showed the same pfge pattern with the restriction enzyme asci ( arbitrarily named as pattern a ) and were st87 ( figure ) .
another 2 l. monocytogenes isolates from cases apparently not related to this first outbreak , isolated in january 2012 and in april 2013 from a 53-year - old man with meningitis and an 84-year - old woman , respectively , showed the same pfge pattern .
pulsed - field gel electrophoresis patterns of 6 listeria monocytogenes serotype 1/2b isolates collected from listeriosis patients in gipuzkoa , northern spain , during january 2013february 2014 .
lanes 1 and 2 , isolates from first outbreak ( pattern a ) ; lanes 3 , 4 , and 5 , isolates from second outbreak ( pattern b ) , including ( lane 5 ) 1 isolate from a food product of foie gras ; lane 6 , l. monocytogenes sequence type 3 serotype 1/2b isolate , unrelated to outbreaks .
for this first outbreak , epidemiologic interviews were conducted starting with the cases observed during september 2013 .
patients were asked about their alimentary habits , focusing on consumption of raw or semicooked animal or animal derivate products .
although no specific food was identified as the possible source of the outbreak , all but 1 of the patients remembered eating cooked ham bought in the butcher s department of a certain supermarket chain located in different villages of the province .
were investigated by pcr and culture after selective enrichment ( 24 listeria enrichment broth ; oxoid , basingstoke , uk ) ) . in the microbiological investigation ( 7 )
, 1 of 6 brands of cooked ham studied grew l. monocytogenes at a low level , but the sample strain was not the serotype 1/2b identified in the outbreak .
no listeria spp . were identified in the remaining 5 brands of cooked ham studied . in the second outbreak ,
10 episodes were reported during early november 2013 through late february 2014 ; patients were 5 elderly persons , 4 previously healthy parturient women , and 1 parturient women who had undergone a splenectomy .
no isolates could be obtained from the mother of an infected newborn and from a miscarriage .
the 10 isolates available for study ( 1 per episode ) were of the same serotype and sequence type , 1/2b and st87 , as isolates from the first outbreak , but these isolates differed in pfge asci pattern ( this pattern arbitrarily named pattern b ) .
isolates from these 2 outbreaks had the same pfge pattern when the smai enzyme was used ( figure ) .
after the epidemiologic survey for this outbreak , we conducted microbiological analysis of several foods .
a foie gras product kept by a patient in his refrigerator yielded a positive culture of l. monocytogenes that had the same phenotype and molecular profile as isolates from the second outbreak .
the presence of the outbreak strain was confirmed in high amounts ( 5.2 10 cfu / g ) by several other cultures from 3 unopened samples from the same brand of foie gras .
after this food source was identified as the source of this second outbreak , all patients from the first outbreak were specifically interviewed again about the consumption of this product , but none remembered having eaten it .
l. monocytogenes infection is serious and has high fatality rates . among the 35 persons infected in this region of spain since 2013 , a total of 6 deaths have occurred : 3 adults , 2 fetuses ( miscarriages ) , and 1 child ( stillbirth ) . without rapid case detection and early treatment ,
l. monocytogenes infections mainly affect elderly persons , pregnant women , newborns , and immunocompromised adults ( 810 ) .
humans are usually infected after eating contaminated food , although the source of the infection is infrequently detected in sporadic cases . for outbreaks , after the epidemiologic survey , a food is usually implicated as the source of infection , but it is not always possible to obtain microbiological confirmation ( 11 ) . in this study , the strain ( same phenotype and genotype ) that caused the second outbreak was obtained from a recently consumed food in the home and in several unopened samples from the same batch of food . however , this source could not be identified as related to the first outbreak .
pregnant women were frequently infected during these outbreaks ; epidemic clones infected 8 pregnant women and their offspring . however , during 2012 and 2013 , serotype 4b isolates of different genotypes also infected 5 pregnant women , resulting in 1 miscarriage , 3 premature newborns , and 1 stillborn infant and 1 premature newborn in a twin pregnancy . in summary , we defined 2 epidemiologically unrelated outbreaks of listeriosis caused by a rare type of l. monocytogenes that occurred at the same time in a small region of spain .
management of this frequently fatal disease requires careful investigation of the source of infection to stop its spread and prompt treatment of infected persons to prevent severe illness and death . | the c - mac video laryngoscope substantially reduced poor glottic views and increased intubation success in icu patients with at least one predictor for difficult intubation . however , all video - assisted and optical intubation devices have different view angles , thus producing different images with particular distortion , and even experts in ' old - fashioned ' airway management need a substantial level of training with a certain device before using it safely and successfully in critical situations and patients .
video laryngoscopes , regardless of a particular brand or device , can not be used intuitively - they require expert skills and routines to be turned into a life - saving airway management tool . |
hepatitis c virus ( hcv ) infection is responsible for considerable morbidity and mortality worldwide .
moreover the hcv genotype has been shown to have an important role in clinical and histological features as well as the response to antiviral treatment .
most studies suggest that subtype 1b isolate possibly represent more aggressive variants of the virus and better responses to ifn are found in patients infected with either hcv genotypes 2 or 3 in comparison with those infected with genotypes 1 and 4 .
considering that approximately 1% of iran s population is infected
, chronic hepatitis c remains a serious medical problem with considerable burden on iran s health care system .
these observations have led us to determine hcv genotypes , particularly in the region of khuzestan province , southwest iran , which has no available data on hcv genotypes .
herein , we report the results of our cross - sectional analyses of hcv genotype distribution and its association with certain clinical features in patients with chronic hcv infection in khuzestan province conducted from september 2006 to september 2009 .
during a three year period ( between september 2006 and september 2009 ) , we conducted a cross - sectional study on 223 consecutive hcv positive individuals who referred to the ahwaz jundishapour university hospitals ( ajsuh ) and hepatitis clinic .
the presence of anti - hcv antibody was determined by an elisa test ( ortho hcv 3.0 diagnostics , raritan , nj , usa ) .
the presence of hcv rna in anti - hcv positive and indeterminate samples was detected by rt - pcr with the qualitative amplicor hcv test v.2.0 ( roche molecular systems , branchburg , nj , usa ) .
hcv genotyping / sub - typing was performed by two rt - pcr assays , restriction length polymorphism analysis ( rflp ) of the 5noncoding region ( 5ncr ) and nested pcr with type specific primers following primary rt - pcr with consensus primers designed for the core region of the hcv genome .
hcv genotyping by rflp was carried out using the restriction enzymes avaii and rsai on pcr - amplified from 5ncr as previously described .
type - specific pcr assay was accomplished by means of the nested pcr assay originally developed by okamoto et al . for sub typing genotypes 1 and 2 , and
sequences of the hcv core region were detected after synthesis of cdna and nested pcr with conserved primers covering positions 439 to 751 of the hcv genome .
rank - sum and kruskal - wallis tests compared continuous variables such as age between the groups . fisher exact test assessed associations .
, 1989 - 2002 , chicago , il , usa ) for windows in addition to univariate analysis and multivariate logistic regression .
all 223 patients had documented positive anti - hcv antibodies for at least six months .
there were 170 males ( mean age : 34.6 years ) and 53 females ( mean age : 49.1 years ) with an age range of 13 - 70 years .
samples comprised 42 patients with unknown risk factors and 179 with parenteral risk of infection [ 11 dialyzed , 10 hemophiliacs , 95 intravenous drug users ( ivdu ) , with tattoos , 54 polytransfused , and needle sticks in 2 cases ] .
possible routes of infection were recognized in 181 ( 81.2% ) patients . among these , patients with a history of ivdu , blood transfusion recipients , undergoing chronic hemodialysis , history of tattooing , accidental inoculation and non - marital sexual contact comprised 42.6% , 23.3 % , 4.93% , 3.15% , 0.9% and 0.9% of samples , respectively ( table 1 ) .
rflp identified hcv genotypes in 181 of 223 samples , whereas 42 cases could not be sub - typed with this method .
patients ages were analyzed as a categorical variable ( < 40 and > 40 years ) .
genotypic distribution was as follows : 1a ( 41.7% ) ; 1b ( 2.7% ) ; 2 ( 4.1% ) ; 3a ( 31.4% ) and 4 ( 1.8% ) .
there were 42 samples ( 18.84% ) not classified into any of the known hcv subtypes .
univariate analysis demonstrated an independent association between ivdu and genotypes 1 ( 60.7% 1a ) and 3a ( 30% ) .
statistical analysis did not show a difference in the frequency distribution of genotypes according to gender and age ( p > 0.56 ) .
in this study , 64 ( 28.7% ) of 223 patients received blood transfusions before 1995 , whereas 5 ( 2.25% ) of 64 patients were positive for hcv rna who received transfusions after 1995 .
hepatitis c infection is currently the most common cause of end - stage liver disease in many countries , however the whole population , prevalence is less than 1% in iran .
a comparison of hcv genome sequences from various geographical regions of the world has shown substantial heterogeneity of nucleotide sequences within several regions of the viral genome .
based on these genomic differences ,
six major genotypes with several subtypes have been identified and a nomenclature for these was given following a consensus proposal .
in the first report on iranian patients , zali et al . in tehran studied the prevalence of specific genotypes in 15 samples with the following results : type i /1a ( 7 ) , type ii/1b ( 3 ) , type v/3a ( 4 ) and type 4 ( 1 ) . a recently published article by samimi - rad et al . in iranian patients with anti - hcv ab positive from tehran and
five cities from different locations of iran showed a predominance of genotype 1a ( 47% ) , followed by 3a ( 36% ) , 1b ( 8% ) and 4 ( 7% ) , respectively .
the current study has shown that in khuzestan province , southwest iran , genotype 1a accounted for the majority of hcv infections ( 41.7% ) .
this is the first study of the genotype distribution in this area which suggests a pattern similar to that reported in other parts of iran , where most cases studied thus far belonged to genotype 1 .
however the results of our study differed from other middle - east countries such as the republic of yemen , kuwait , iraq and saudi arabia where genotype 4 has been identified as the most prevalent .
a study of saudi patients detected hcv genotype 4 in 50% of cases and genotype 1b in nearly 40% .
from other parts of the world , studies have revealed that genotype 3 is prevalent in south east asia whereas genotype 1 is common in the usa and western europe .
patients with risk factors for hcv infection enrolled in the present study were very few and therefore we were unable to reach any conclusion of genotype distribution within this group .
the distribution of the two most common hcv viral types ( 1a and 3a , ) were not statistically different in terms of mean age or gender .
the relative influence of hcv genotype , risk factors for infection , duration of infection , and host factors on progression of liver disease seemed to be important .
our study indicated that ivdu and blood transfusions were the leading risk factors for hcv acquisition ( 71.3% ) , of which ivdu was the most frequent risk factor ( 42.6 % , ) .
all hcv infected ivdu patients were male , which was consistent with other reports .
the route of hcv transmission mainly causes this epidemiological pattern in genotype distribution such that ivdu associated with genotype 1a and 3a is currently the primary risk factor of hcv infection .
screening for hcv is routine for all blood donors since 1995 in iran but 5 ( 2.25% ) out of 64 patients who received transfusions after 1995 were positive for hcv rna .
therefore , more careful pre transfusion screening of blood for anti - hcv must be introduced in our blood banks .
there were 42 cases ( 18.8% ) unable to be classified for hcv genotype by rflp technique .
this may be due to multiple infecting genotypes in these patients , difficulties in sample handling or unusual hcv genotypes in this patient group which could not be classified by this method .
we can conclude that genotype 1 , particularly the subtype 1a and genotype 3 , subtype 3a are predominate in this geographic part of iran .
knowledge on the distribution of various genotypes in our area is essential for its prognostic implications in chronic hepatitis c infection .
further investigations with larger sample numbers are necessary to determine the major genotypes that cause hcv infection in various clinical conditions and potential associations with disease severity .
this work was supported by the tropical and infection research center , ahwaz jundishapour university of medical sciences , ahwaz , iran .
we are grateful to the research deputy of ahwaz jundishapour university of medical sciences for his encouragement and constructive advice during the preparation of the manuscript .
we thank the subjects for their compliance in participating in this study , and danesh lab center for genetic studies , sequence alignment and immunoassays .
septic shock is consistently the most common causative factor identified for acute kidney injury ( aki ) in critical illness , and has been associated with nearly 50% of cases internationally . despite advances in our understanding of the pathophysiology of septic aki ,
treatment aimed at reversing or preventing septic aki remains primarily based on supportive haemodynamic management . in the previous issue of critical care , legrand and colleagues examine the association between haemodynamic targets of resuscitation ( cardiac output , mean or diastolic blood pressure , central venous pressure ( cvp ) and central venous oxygen saturation ) and development or persistence of aki in a single centre study of patients with septic shock admitted to a surgical icu .
the authors found that only higher cvp and low diastolic blood pressure were associated with increased risk of development of new aki , or persistence of renal dysfunction present at icu admission . in these patients
importantly , the association between cvp and aki remained when potentially confounding effects of positive fluid balance and higher positive end - expiratory pressure were accounted for in a multivariable analysis .
so , a 5 mmhg increase in cvp predicted 2.7-fold odds of new or persistent aki .
furthermore , when the association between different levels of mean cvp and aki was examined in a non - parametric logistic regression , there was a trend for higher cvp to be associated with worse renal outcome for all levels of cvp from 4 mmhg upward , so that a cvp of 15 mmhg was associated with an approximately 80% risk of new or persistent aki , compared to approximately 30% at a cvp of 6 mmhg .
these findings are important because current surviving sepsis campaign guidelines recommend fluid resuscitation of patients with sepsis - induced tissue hypoperfusion to target a cvp of 8 to 12 mmhg ( or 12 to 15 mmhg in mechanically ventilated patients ) within 6 hours of presentation . in patients with sepsis - induced hypotension
the rationale for cvp targeted fluid resuscitation is to ensure ' adequate cardiac preload and hence maintain cardiac output and organ perfusion .
however , absolute levels or changes in cvp poorly predict cardiovascular response as sepsis - induced hypotension is multi - factorial , related to changes in myocardial performance , vascular tone , regional blood flow distribution , venous reservoir capacity and capillary permeability . in contrast
, elevated cvp will cause a direct and predictable increase of renal venous pressure that , experimentally , has been associated with elevated renal interstitial and intra - tubular pressure .
resultant renal venous congestion can reduce renal blood flow ( lower trans - renal pressure gradient ) and decrease the pressure gradient for ultrafiltration ( higher intra - tubular pressure ) . in septic shock ,
. aggressive fluid resuscitation beyond reversal of hypovolaemia to arbitrary cvp targets could result in increased venous congestion without substantial benefit to forward renal perfusion , thus predisposing to renal dysfunction .
the association between elevated cvp and renal dysfunction has been made previously in the setting of chronic cardiac failure .
now legrand and colleagues have provided evidence that such an association is observed in septic shock and its treatment .
their findings are also in accord with secondary analysis of the vasopressin in septic shock trial , which reported that a more positive fluid balance and elevated cvp were associated with increased mortality in patients with septic shock even when accounting for differences in baseline illness severity and demographics .
although these observational findings are hypothesis - generating rather than confirmatory , they add weight to the literature suggesting the adverse effects of fluid overload in critical illness and aki in particular , effects that may be mediated in part by venous congestion causing impaired organ function .
it is now important to determine whether uncritical pursuit of cvp targets such as those advocated by the surviving sepsis campaign might , in some patients , add insult to sepsis - induced organ injury .
prospective data are required to evaluate the true contribution of raised venous pressure to renal and other organ dysfunction and to determine better endpoints for fluid resuscitation in sepsis .
rajkumar rajendram bsc mbbs mrcp frca fficm , specialty registrar in anaesthesia , royal free hospital , london .
john r prowle ma msc md mrcp fficm , consultant in renal medicine and intensive care , the royal london hospital , london . | background
hepatitis c virus ( hcv ) infection is responsible for considerable morbidity and mortality worldwide .
the hcv genotype has a geographic distribution and an important role in clinical and histological outcomes .
this study determined hcv genotypes and their related risk factors among patients from khuzestan province , southwest iran .
methods
in a cross - sectional study , 223 patients infected with hcv who referred to ahwaz jundishapour university hospitals ( ajsuh ) and hepatitis clinic were enrolled .
specific and nested polymerase chain reaction ( pcr ) and restriction fragment length polymorphisms ( rflps ) were performed to determine viral infection and genotype analysis .
liver enzymes including alt and ast and the correlated risk factors were also determined .
results
the hcv genotype distribution was as follows : genotype 1a ( 41.7% ) ; genotype 1b ( 2.7% ) ; genotype 2 ( 4.1% ) ; genotype 3a ( 31.4% ) ; and genotype 4 ( 1.8% ) .
there were 42 samples ( 18.84% ) not classified into any of the known hcv subtypes .
no patient was infected with more than one genotype .
hiv was found in four ( 1.8% ) cases , of which all were intravenous drug users .
univariate analysis demonstrated an independent association of intravenous drug use ( ivdu ) and genotypes 1a ( 60.7% ) and 3a ( 30% ) .
conclusion
our findings suggest that genotypes 1a and 3a are the most common ones among iranian patients with chronic hepatitis c infection in khuzestan province , southwest , iran . |
one hundred forty - seven clinical isolates of m. tuberculosis were obtained from the molecular mycobacteriology laboratory , department of microbiology , faculty of medicine , siriraj hospital , mahidol university , thailand , and the t-2 project from 1997 to 2001 ( table ) .
these strains were isolated from 74 cerebrospinal fluid ( csf ) samples and 73 sputum samples from 147 different patients .
dna from these isolates was isolated by an enzymatic method and submitted for genotyping by performing the is6110 restriction fragment length polymorphism with the standard method ( 12 ) and for sequencing the pks15/1 region ( 8) .
* csf , cerebrospinal fluid . using the genotyping results , we categorized m. tuberculosis isolates into beijing , single - banded , few - banded ( 25 bands ) , nonthaburi , and heterogeneous with > 5 bands ( table and figure 1 ) , as recently reported ( 13,14 ) .
all m. tuberculosis genotypes were sequenced around the junction of pks15 and pks1 ( corresponding to the m. tuberculosis h37rv sequence ) to determine whether they contained an intact pks15/1 or separated pks15 and pks1 .
unexpectedly , the results showed that the 7-bp insertion of pks15 that causes a frameshift mutation resulting in an intact pks15/1 was found in most strains of all genotypes , except the heterogeneous group with > 5 bands ( table and figure 2 ) .
r indicates the m. tuberculosis mt 14323 strain used as the positive control for is6110 typing .
sequence alignment of region corresponding to the 3 portion of pks15 and 5 portion of pks1 in various mycobacterium tuberculosis genotypes .
b ) m. tuberculosis strains isolated from sputum . letters in brackets refer to is6110 restriction fragment length polymorphism patterns : bj , beijing ; sb , single banded ; fb , 25 bands ; nb , nonthaburi ; h , heterogeneous .
the 7-bp insertion is shown in boldface , and the start codon of the pks1 gene is underlined .
the intact pks15/1 has been shown to be responsible for the production of phenolic glycolipids and is seemingly found in m. tuberculosis w - beijing family , but it was not found in m. tuberculosis cdc1551 and h37rv ( 8) .
previous studies suggested that pgls produced by the m. tuberculosis w - beijing family were associated with the hypervirulent phenotype by inhibiting the innate immune response ( 6,7 ) .
the intact pks15/1 has also been shown to be nonpolymorphic in the w - beijing family ; it was found in all 102 w - beijing strains tested ( 15 ) . from this observation
, we hypothesized that if the ability to produce pgls is among the factors that make this family more virulent than others , the intact pks15/1 should be absent in strains other than the w - beijing family .
our results showed that the 7-bp insertion of the pks15/1 was not only present in the w - beijing family but also in other m. tuberculosis genotypes .
although almost all beijing strains contain the intact pks15/1 ( 97% ) , 38.5%100% of strains of other genotypes also contain it .
these strains could , therefore , produce pgls and cause both pulmonary and disseminated diseases as the w - beijing strains do .
our results showed no significant difference in the percentage of m. tuberculosis isolates with an intact pks15/1 gene between csf isolates ( 65 [ 87.8% ] of 74 ) and sputum isolates ( 62 [ 84.9% ] of 73 ) .
the hypothesis that the hypervirulence of the w - beijing family is solely attributable to pks15/1 is still inconclusive .
this family may have only recently been transmitted globally and may have had more chances to cause infections and disease than other families .
although pgls are involved in the hypervirulence of the pgl - producing strains , they are not a unique characteristic of the w - beijing family
. if w - beijing strains are more virulent than others , other virulence determinants besides pgls must be responsible for the hypervirulent phenotype . although , the incidence of multicentricglioma accounts nearly 2.5% of all gliomas , mostcases are glioblastoma or anaplastic gliomas .
oligodendroglioma cells are detectablein the cerebrospinal fluid in up to 14% of patients , and we recently treated a case of multiple anaplastic oligodendrogliomas , describe the case in detail and discuss the distinction between multicentric and dissemination via cerebrospinal pathways and the treatment .
a 72-year - oldman revealed aphasia , abnormal behavior , and rapidly progressivedementia since october 13 , 2009 , and hospitalized in our department on oct .
17 , 2009.magnetic resonance imaging ( mri ) enhanced by contrast media demonstrated multiplebrain tumors in left parietal lobe and left paraventricular region [ figure 1a ] . computed tomography ( ct ) scan showed high density of microbleeding in the left paraventricularregion [ figure 1b ] .
biopsy for the tumor in the left parietal tumor was performed . in the permanent section ,
the hematoxylin and eosin - stained specimen showed that thetissue was composed of cells with clear cytoplasm and with round or irregular nucleushaving dense chromatin ( so called honey comb appearance ) that existed in between highly proliferative vasculature [ figure 2 ] .
the nuclei had pleomorphism and a variety ofsize , a lot of mitosis were observed .
microvascular proliferation and necrotic findingswere also seen . occasionally , calcification deposit and microbleeding were observed.immunocy to chemical study showed positive for glial fibrillary acidic protein , stronglypositive for the olig-2 ( oligodendrocyte lineage genes ) stain .
these findings were compatible for anaplastic oligodendroglioma , so geneticalevaluation was performed , showing loss of heterozygosity ( co - deletions ) in chromosome1p19q .
local brain for the left parietal regionradiotherapy was given to a total dose of 50gy , in34 fractions of 1.5gy daily on weekdays from november to december , 2009 .
at the sametime , temozolomide of 120 mg daily was orally administrated for 42 days .
mri was performed just after these combined chemo- and radiotherapy showed lessening of themass effect in the left parietal tumor , but enlarging the size of the tumor in the left paraventricular region [ figure 3 ] .
we considered that enlargement of the paraventriculartumor was pseudoprogression , as consolidating therapy , temozolomide of 240 mg daily ( 150mg / m2/day ) for 5 days was orally administered for 5 times from february to october 2010 at outpatient clinic . despite of the high levels of promoterhypermethyltransferase ( mgmt ) activity , temozolomide is the first linechemotherapeutic agent against anaplastic oligodendroglioma , so we tried temozolomide . but during those period , cerebellar tumor appeared and graduallyenlarged and hydrocephalus was observed [ figure 4 ] . in october 2010 ,
one cycle of temozolomidevia intravenous administration was performed and lessening of the masseffect was seen , so the patient was transferred to rehabilitation hospital . in the end
ofnovember 2010 , the patient 's consciousness deteriorated because of the enlargement ofthe 4th ventricle tumor , hydrocephalus and abnormality of serum electrolyte .
theconsciousness of the patient recovered for a while by correcting the electrolyte balance , but consciousness level worsened again , and respiration and clinical state wasdeteriorated , and died on january , 2011 .
( a ) magnetic resonance imaging ( mri ) enhanced by contrast media obtained atthe first hospitalization.multiple brain tumors in the left parietal and leftparaventricular region were observed .
the tissue was composed ofcells with clear cytoplasm and with round or irregular nucleus having dense chromatin ( so called honey comb appearance ) .
hematoxylin and eosinstained specimen , 400 mri after radiotherapy 50gy and temozolomide of 120 mg daily for 42 days .
although , lessening of the mass effect of left parietal tumor was observed , enlargementof the paraventricular tumor was seen , which seemed to be pseudoprogression mri performed about 1 year after initial hospitalization .
salvati m. et al.reported 25 cases with multicentricgliomas , 11 cases aremulticentricglioblastomas , 6 cases are multicentric anaplastic astrocytomas , and othercases are combinations of glioblastomas , anaplastic astrocytomas , and low gradegliomas , and no oligodendrogliomas were found .
solomon etal . stated a clear distinction must be made , in cases of multiple tumors , amongdissemination via cerebrospinal pathways , direct extension across commissures , satellitosis , and true multicentrism .
multicentric tumors are widely separated , arisingin different lobes or hemispheres , which is not readily explained by one of the pathwaysmentioned above.in our case , the 4th ventricle tumor which arose about 1 year afterinitial treatment might have been a metastasis via cerebrospinal fluid , although one ofthe multiple sites can not be dismissed entirely . however , left parietal tumor and lefttrigonal area tumor were found at the same time , and those two tumors were separated , arising in the distinct sites , and were not explained either by the dissemination viacerebrospinal pathways or direct extensions , so we considered those tumors weremulticentric.the pathogenesis of multicentricgliomas is unknown.the most quoted hypothesis is the two - step process reported by willis . in the firststage
a large area of the brain undergoes neoplastic transformation , while in the secondstage one or more foci of tumor proliferation develop within the affected area , givingorigin to separate lesions .
the pathogenesis of multicentricglioma include theembryonal rest theory and the two - stage theory , but zlch pointed out the metastasisvia an unknown pathway .
multicentricgliomas are characterized by rapid progression , which may be related tothe fact that they tend to exhibit malignant features on histology , this fact is also validfor the oligodendrogliomas .
the course of our case from initial diagnosis to death was 1year and 3 months , despite of the combined chemoradiotherapy.in our case , the 4thventricle portion tumor which appeared about one year after the initial treatment andwas not detected in the mri taken at initial hospitalization , might be the metastasis viacerebrospinal fluid , as histological type of malignant oligodendroglioma is a risk factor of cerebrospinal fluid dissemination .
finally , the tumor in the left paraventricular region showed progression even just after the temozolomide treatment , and we presumed it was pseudoprogression , because in the first 6 months of post - chemoradiotherapy period , showed a high incidence of pathologically confirmed pseudoprogression associated with treatment - related necrosiswithout viable tumor cells .
various imaging techniques including mr spectroscopy havebeen studied but neither has been found to be powerful to identify pseudoprogression , and unfortunately , the diagnosis of pseudoprogression still depends on pathologicexamination.the disruption of blood - brain barrier with the resultant observation of an earlyradiologic increase in contrast enhancement at mri , which may falsely mimic trueprogression , and this fact made the treatment of this case more complicated . | to determine whether intact pks15/1 is unique to the w - beijing family , we investigated 147 mycobacterium tuberculosis strains with different is6110 genotypes .
intact pks15/1 was found in 87.8% of cerebrospinal fluid and 84.9% of sputum isolates .
it was found not only in w - beijing strains ( 97% ) but also in other genotypes ( 38.5%100% ) . |
cholelithiasis presented as chronic right back pain in the case discussed in this report . according to the clinical practice guidelines , gallbladder lithiasis is a common condition . around two - thirds of the cases
are asymptomatic , with the most frequent clinical presentation being the presence of attacks of acute abdominal pain . however , chronic right back pain persisting for months as a main symptom is uncommon .
this report presents a patient with right back and subscapular pain for 9 months , demonstrating both biliary lithiasis and chronic cholecystitis .
it is important to take into account the atypical manifestations of a prevalent illness as well as the limitations of the diagnostic tests , since the presence of gallstones can easily be missed or misinterpreted and delay in diagnosis may lead to complications which could worsen the prognosis of the patient .
a 47-year - old male presented with a history of chronic neck pain that had started 10 years back .
he underwent a magnetic resonance ( mr ) which showed cervical canal stenosis of degenerative etiology and also had an episode to self - limited abdominal spasmodic pain that appeared 10 years ago , considered as being functional by the gastroenterologist .
abdominal ultrasonography was performed twice , and both times it was normal . one and a half years ago , the patient had right subscapular and paralumbar back pain at the t10l2 level for 9 months .
he described it as a permanent pain , which persisted throughout the day , with very intense exacerbations , especially in the evening and at night .
these exacerbations usually lasted for 4 - 6 h , worsening on inhalation and sometimes radiating to subcostal and right hemithorax .
the only feature of note on physical examination was the palpation of painful spinal processes from t10 to l2 .
blood analysis showed the level of gamma - glutamyl traspeptidase to be 79 u / l , while all the other laboratory parameters were normal .
abdominal computed tomography ( ct ) showed an extensive hepatic steatosis and a moderately relaxed gallbladder , with no stones observed within thin walls [ figure 1 ] .
abdominal ct : gallbladder without gallstones despite the regular use of analgesics and the rehabilitation treatment that followed , the patient presented a torpid evolution . due to the pain in the right paralumbar area ,
an abdominal ultrasonography was performed , which showed moderate hepatic steatosis and a gallbladder with multiple inner calculi [ figure 2 ] .
on diagnosis of cholelithiasis , a cholecystectomy was carried out , which revealed a gallbladder with a large number of yellow lithiasic structures , thereby confirming an anatomopathological diagnosis of chronic cholecystitis [ figure 3 ] .
the previous pain completely disappeared after surgery and the patient remained asymptomatic 7 months later .
back pain affects 80% of adults at some point of their life and occurs at all ages .
the first objective is to rule out a visceral disorder or a serious potentially life - threatening condition , which occurs in 2.7% of the cases
. back pain may be the manifestation of thoracic involvement as in esophageal disease , pleurisy , aortic aneurysm , or coronary heart disease , or an abdominal process , such as gastrointestinal ulcer , gastric cancer , pancreatic cancer , pancreatitis , or biliary pathology .
only one - third of the cases of gallstones are symptomatic and give an indication for surgery .
the most frequent symptomatic manifestation is episodic upper abdominal pain called biliary colic , with complications such as cholecystitis , acute pancreatitis , cholangitis , or choledocholithiasis being less frequent .
characteristically , this pain is severe and located in the epigastrium and/or the right upper quadrant .
the pain may radiate to the upper back or the right scapula in 60% of the cases and might be associated with nausea or vomiting , usually occurring in the late evening or at night , in 80% of the cases .
one study carried out to characterize the presentation in cholelithiasis reported that all patients described pain in the right upper quadrant of the abdomen , including the epigastrium .
the pain was located in the right subcostal area ( 20% ) and epigastrium ( 14% ) , which radiated to the back in 63% .
in fact , chronic cholecystitis is thought to be a result of a delay in diagnosis .
the test of choice to diagnose cholelithiasis is transabdominal ultrasound , with a sensitivity and specificity greater than 95% for detecting gallstones larger than 4 mm .
abdominal ct scan has a very low sensitivity and gallstones may be visible due to most being isodense .
microlithiasis ( stones <3 mm in diameter ) is not detectable by transabdominal ultrasonography ; however , it may cause complications such as acute pancreatitis . in some patients
the diagnosis can be confirmed by the detection of biliary crystals by microscopic examination of bile collected from the duodenum . in patients with a clinical history of biliary colic with negative transabdominal ultrasonography ,
endoscopic ultrasonography is an important diagnostic tool , since it can detect microlithiasis in a proportion of patients ranging from 41 to 78% .
patients with pain and a negative abdominal ultrasound show an endoscopic ultrasound and/or a positive analysis of the bile .
most of these patients show positive results on cholecystectomy , with resolution of abdominal pain and improvement in their quality of life .
non - vertebral causes such as the presence of gallstones should be considered in patients with back pain .
physicians should be aware of atypical manifestations of common diseases , so as to avoid diagnostic delays .
gallbladder disorders should be taken into account in patients with dull , undefined right back pain , despite a normal physical examination .
abdominal ultrasound should be chosen as the first diagnostic procedure when considering gallstones , since ct scans have a very low sensitivity for their detection . in a patient with a typical clinical report of biliary colic and a negative abdominal ultrasound ,
this was the case of our patient in whom the abdominal ultrasonography was normal 5 years earlier , thereby making correct differential diagnosis crucial . spending hours in plastic surgery opd in a government medical college and dealing with the kind of queries posed to us by patients and often having to see delayed and neglected patients made us question and wonder on various views that people have of plastic surgery .
most of the patients had no idea of the range of our services and had reached us after having visited a series of generalists and eventually being referred to us .
hence , we undertook this study with an aim to ascertain current awareness among the medical students and residents ( the future practitioners ) about the surgical procedures that a plastic surgeon performs .
this study was conducted in four medical colleges chosen from four zones of india : king george medical college ( kgmu ) , lucknow ; all india institute of medical sciences ( aiims ) , new delhi ; king edward memorial ( kem ) hospital , mumbai ; and stanley medical college ( smc ) , chennai .
the questionnaire had 35 surgical situations , whereby the respondents were to refer a patient to any department or departments where a particular problem can be managed .
a total of 2000 questionnaires were distributed and 1552 responses were received . there were 440 respondents from kgmu , 312 from aiims , 418 from kem , and 382 from smc .
the respondents were categorized into four groups : mbbs students , interns , junior residents , and senior residents .
the y - axis reveals percentage of referral to plastic surgeons and x - axis shows the group studied .
facial fractures , craniofacial surgery , temporomandibular joint dysfunction cleft lip palate , burns liposuction , rhinoplasty , hair grafting wound cover , pressure ulcer , scalp avulsion peripheral nerve injury , hand anomalies and trauma
it was observed [ table 1 ] that there was a good deal of awareness ( > 60% ) regarding the role of plastic surgeons in dealing with burn injuries , rhinoplasty , liposuction , hair grafting and cleft lip and palate .
there was a moderate degree of awareness ( 4060% ) regarding wound management and pressure ulcer , being managed by plastic surgeons .
only 1530% respondents were aware that craniofacial anomalies , congenital hand anomalies , facial fractures , scalp avulsion , hand injuries and peripheral nerve injuries are reconstructed by us .
there was a poor knowledge ( < 15% ) about brachial plexus surgery and temporomandibular joint ( tmj ) ankylosis being managed by plastic surgeons .
table 1 is an ascending order depiction of the awareness regarding treatment of various conditions by plastic surgeons .
the findings of our study are similar to those of previous studies on different population and socioeconomic groups which show the public to be poorly informed about plastic surgery . in his paper
perception of plastic surgery in the society , the author concludes that indian public associates plastic surgeons with cosmetic and burn surgeries . the knowledge is also limited among medical students and nurses and the source of their knowledge is magazines and newspapers . in their study
perception and reality a study of public and professional perceptions of plastic surgery conducted in the british public population , medical students and general practitioners , plastic surgery was associated with reconstruction for trauma and cancer and procedures with a strong aesthetic element by all the three groups and hand was associated with orthopaedic surgeons . in a study conducted in philadelphia in the usa , among consecutive patients visiting a primary care facility , medical students , and primary care physicians , the authors demonstrate that public perception of the plastic and reconstructive surgeon is limited and grossly underestimates this specialty .
although the plastic surgeons are associated with reconstructive surgery , they are not necessarily identified as primary surgeons for procedures fundamental to this specialty .
many people who do not come into contact with plastic surgery in their hospitals are under the popular misconception that plastic surgeons spend their time fixing noses and altering the size of breasts .
it is not really surgery since it can be done in the office and is not covered by insurance .
the media is also little informed about the versatility of plastic surgeons and ends up building false expectation in public and wrong images of plastic surgeons as scavengers for money . in their paper plastic surgery in the cinema
, the authors concluded that films usually incorporate cosmetic surgery and burns in the screenplay .
also , plastic surgeons are depicted to be living in affluent localities , making a lot of money .
patients visiting plastic surgeons are also shown to be ultra rich.[710 ] this often deters many patients from primarily consulting us .
patients visiting the department late in the course of disease process have often revealed that they had feared to visit our department fearing the expenditure they would incur . although the findings of this paper may not surprise practicing plastic surgeons , it does quantify a reality we all share that various works of our specialty are not easily identified by public and medical professionals .
we have made an attempt to reason out the cause for the same in our country and suggest remedial measures .
also , the commonly followed textbook of general surgery in the undergraduate curriculum discusses mostly burns and cleft lip and palate under plastic surgery section . to add to these ,
the lectures devoted to plastic surgical topics are few and often delivered by general surgeons who are less equipped to spread awareness among medicos .
hence , plastic surgery department must be developed in all colleges , and medical students and surgical residents must be rotated for at least a week or two as part of their training programme .
this is a long - term goal , and till it is in place , a few committed and reverent teachers in the society may come forward to deliver planned lectures to mbbs students in colleges where the department is lacking .
this can be put forward by the association of plastic surgeons of india ( apsi ) as part of expansion of plastic surgery .
we should also develop methods to educate the general practitioners because they are the people who reach out to public at large .
our primary aim then should be to educate and sensitise the media regarding the role of plastic surgeon even in day - to - day life of a common man
. the contribution of prominent members of plastic surgery society will be more than sufficient to open the eyes of media regarding various subspecialities .
apsi may invite media personnel on an awareness trip to well - established centres to see a variety of procedures and the necessity of plastic surgeon in trauma centres , burn care , as reconstructive surgeons in trauma , congenital and oncological surgeries besides their role as an aesthetic surgeon .
we should encourage media personnel to project a real picture to the general public and publicise the various conditions in which plastic surgeons play a role . at the village level , this may be done by plays and distributing pamphlets and posters showing preoperative and postoperative photos .
regular monitoring of progress in the endeavour to spread awareness can be conducted yearly , and necessary intervention may be added as and when required welcoming newer ideas for the same .
our study clearly shows that knowledge about the field of plastic surgery is highly lacking and lot needs to be done to spread awareness .
the best way to describe plastic surgeons is that we are a problem solving specialty .
the amazing developments made in our field must be made available and accessible to the mass and a collective part played by plastic surgeons can go a long way in making our specialty really known to even layman as to it being a necessity rather than a privilege . | chronic right back pain is a symptom in both biliary lithiasis and chronic cholecystitis
. ten percent of the population in the world suffers from biliary lithiasis .
only 20% are symptomatic .
the first diagnostic test of choice is an abdominal ultrasound .
when a suggestive clinical sign of biliary colic with negative abdominal ultrasound is identified , we should consider the option of carrying out an endoscopic ultrasound in order to rule out microlithiasis .
the case discussed in the report presented with chronic right back pain , which is an atypical manifestation of biliary lithiasis and chronic cholecystitis .
it is important to know about the atypical manifestations of the prevalent illnesses as well as the limits of the diagnostic tests , in order to avoid diagnostic delays which may cause complications that could worsen a patient 's prognosis .
this case should contribute to the medical knowledge and must have educational value or highlight the need for a change in clinical practice , especially in primary care . |
the prostate is the site of two common diseases in aging men , benign prostatic hyperplasia and prostate cancer ( pca ) .
epidemiological studies show that , in the united states , pca accounts for 28.7% of new non - skin cancers and 12.7% of cancer - related deaths.1 androgen deprivation therapy ( adt ) retards pca progression in 60%80% of patients but may cause cardiovascular complications and alterations in body composition , lipoprotein metabolism , and sexual function . in general , adt may result in a decreased quality of life .
furthermore , studies in men demonstrate that low serum testosterone ( t ) levels are associated with several cardiovascular risk factors,2,3 such as lower high density lipoprotein cholesterol , higher triglyceride concentrations , hyperinsulinemia , and increased abdominal adiposity .
these factors are all characteristic of the metabolic insulin resistance syndrome . in addition , many patients treated with adt experience rapid bone loss that increases the risk of debilitating osteoporotic fractures.46 reports indicate that total and free levels of serum t decline in aging men and that abdominal visceral fat is inversely associated with t levels . resting energy expenditure ( ree ) , which accounts for 60%70% of total daily energy expenditure ,
decreases with age , physical inactivity , sympathetic nervous system and endocrine status.7,8 to our knowledge , the effects of adt on ree have not been fully explored in controlled trials .
the goal of this prospective study was to determine the effects of adt on body composition and resting metabolic rate in patients with metastatic pca .
sixteen men , aged 63 to 96 years ( mean age 71 ) , with untreated metastatic pca were enrolled in this study . prior to orchiectomy , all patients were evaluated clinically and by laboratory analysis to establish a baseline . after a careful medical history had been taken from each patient , we assessed several exclusion criteria , namely , cognitive alterations , depression and emotional liability .
laboratory examinations included tests of fsh - lh serum concentrations , and an evaluation of body mass index , fat / lean body mass , bone mineral density , carbohydrate / lipid oxidation , and resting energy expenditure .
following orchiectomy , the same evaluation was carried out monthly and after 12 months , the period chosen for final analysis . to evaluate body composition
, all patients underwent densitometry testing ( dual - energy x ray absorptiometry - dexa).9 the ree , fat and carbohydrate oxidation levels were measured using indirect calorimetry , with a delta - trac metabolic monitor.10 body composition analyses and indirect calorimetry were carried out at baseline and after 12 months following the orchiectomy procedure . written consent was obtained from all patients , and this protocol was approved by the ethics committee at our institution .
we used the non - parametric two - sided wilcoxon t - test , and p<0.05 was considered significant to appropriately compare time differences for each variable.11
we used the non - parametric two - sided wilcoxon t - test , and p<0.05 was considered significant to appropriately compare time differences for each variable.11
statistically significant differences in body composition pre and post orchiectomy were seen in terms of weight , body mass index ( bmi ) and lean body mass ( lbm ) .
total fat body mass ( fbm ) exhibited a tendency to increase but failed to reach significance .
no significant changes were observed in bone mineral density ( bmd ) and ree , but we identified a statistically significant change in terms of increased carbohydrate oxidation ( choox ) and a decrease in lipid oxidation ( lipidox ) .
aging is associated with sarcopenia ( loss of muscle mass ) , with an average 12 kg loss of lbm between the ages of 25 and 70 years and an increase in fbm of 18%36% during the same period.2 testosterone levels exhibit a positive correlation with lbm and a negative correlation with fbm .
this has led to the hypothesis that adt may help mitigate age - related alterations in body composition .
our study reports two major findings : 1 ) adt was associated with decreased body weight and with decreased lbm ; 2 ) adt led to increased choox and decreased lipidox .
androgens modulate body composition , including lipid and protein metabolism.12 smith et al,13 galvo et al,14 levy et al,15 and van londen et al16 observed significant changes in metabolic and body composition parameters after 6 months of induced hypogonadism in all patients .
the researchers observed a significant increase in fbm ( 20.29.4 to 21.99.6 kg,13 13.82.3%,14 1.172.3 % , 15 and 2167.15 g,16 respectively ) and a significant decrease in lbm ( 63.28.0 to 62.35.4 kg,13 2.4 0.4%,14 1.152.20%;15 and 1785.81 g,16 respectively ) with no significant changes in total body weight , lipid readings or glucose metabolism .
they also reported a positive correlation between the changes in fbm and insulin concentrations ( r=0.56 ; p=0.013 ) .
these results are in agreement with other studies that demonstrated a relationship between t and abnormalities in carbohydrate and lipid metabolism.17 in healthy male populations , low t concentrations are negatively correlated with the degree of central abdominal obesity . in hypogonadal males , there is a tendency toward increased visceral adiposity and reduced muscle mass that can be reversed by androgen replacement.18 the underlying mechanisms for these observations are not well defined , but androgen receptors are known to be present on visceral adipocytes .
it is likely that t is directly involved in the mobilization of free fatty acids.19 we observed significant changes in metabolic and body composition in our patients after adt .
we identified significant decreases in total body weight , bmi and lbm ( 2.5 kg , p = 0.01 ; 0.8 kg / cm , p = 0.02 , 11.7 kg , p = 0.002 , respectively ) .
fbm was found to increase ( 7.15 kg , p = 0.06 ) with no significant change in bone mineral density ( bmg ) .
there were no changes in ree , but we did identify a significant increase in choox and a decrease in lipidox ( 52.62% , 4.10% respectively ) . in one study , suppression of t levels after 10 weeks of gnrh agonist administration was found to reduce ree in young men .
in this study , researchers were unable to determine whether the effect was directly mediated by a reduction in t levels or if it was due to a subsequent reduction in t aromatization to estrogen.20 we were unable to show alterations in ree in our study , possibly due to the size of our study sample .
the altered concentrations of gonadotropins following orchiectomy may be linked with the effects of this procedure on the pituitary gland .
it is important to consider that adt not only acts on pca but also affects metabolism , including hematogenesis , bone , fat , protein , and sarcho - metabolism . despite pca being one of the leading causes of death , a substantial proportion of aging patients die of unrelated causes that are due to comorbidities , often in the context of metabolic syndrome .
to our knowledge , this is the first prospective study to examine changes in body composition , resting energy expenditure , and consequences involving the oxidation of carbohydrates and lipids in the context of adt .
a study on a larger group of patients would be of great interest once a limited number of patients included from only one centre may be considered a limitation in this study .
after 12 months of androgen deprivation therapy , elderly males with metastatic prostate cancer exhibit declines in lean body mass and lipid oxidation , together with an increase in carbohydrate oxidation . celiac disease is an under diagnosed condition in which gluten ingestion in genetically susceptible individuals results in small - bowel mucosal inflammation and villous atrophy .
most patients are asymptomatic or suffer from mild symptoms only , ( 1 ) and many present with extraintestinal manifestations such as neurologic disorders ( 2 ) .
the anti - tissue transglutaminase antibody ( ttga ) test is a sensitive and specific tool in disclosing celiac disease with overt villous atrophy ( 3 ) with a specificity of approximately 95% , false positive tests are uncommon , and ttga may appear in serum at an early stage in the disease , in other words before the clinical manifestations and even before the development of villous atrophy ( 4 ) .
psychiatric disorders are also common in untreated celiac disease , especially depressive symptoms ( 58 ) .
hallert & derefeldt reported that nine out of 42 studied subjects had attended a psychiatric clinic because of neurotic problems and most of them are involved with depressive disorders ( 7 ) .
celiac disease was considered relatively uncommon in iran , until recently an estimated population prevalence of 1:166 was reported ( 9 ) .
greater awareness of its varying presentation and the availability of new serologic tests have shown celiac disease to be relatively common ( 10 ) .
these observations prompted us now to assess the association between celiac disease and severe chronic depression and schizophrenia .
this cross - sectional study was carried out in 2006 - 2007 . by random sampling
, 200 inpatient men comprising 100 with chronic depression and 100 with schizophrenia ( mean age 37 years , range 18 - 68 years ) were enrolled in razi hospital , tabriz , iran .
chronic depression was defined according to dsm - iv criteria , ( 11 ) diagnosed and treated by semi - structural clinical interview by two psychiatric experts .
the duration of the diseases was more than two years , and the diseases were unbearable without antipsychotic drugs .
patients with schizophrenia suffered from different types of the disease such as paranoid , phrenetic or undistinguished .
two hundred healthy males were selected as controls , matched for age and birthplace ( mean age 32 , range 4 - 77 years ) .
a written informed consent was obtained from patients ( or from next of kin if necessary ) and the study was approved by the institutional ethics committees of the research center for gastroenterology and liver disease , tabriz medical university .
iga class ttga antibody was measured by enzyme - linked immunosorbent assay using a commercially available kit ( eu - ttg iga , eurospital , trieste , italy ) .
a titer of > 7 u / ml was considered positive as recommended by the manufacturer .
a frequency of 0.6% celiac disease has been reported in iran ( 9 ) . assuming this frequency in 200 controls and a tenfold frequency in psychiatric patients ( 6% , as has been reported in many autoimmune conditions ) the statistical power of 0.80 at a significance level of 0.05
percentages were compared by rates and proportion ; 95% confidence intervals ( ci ) were reported .
a frequency of 0.6% celiac disease has been reported in iran ( 9 ) . assuming this frequency in 200 controls and a tenfold frequency in psychiatric patients ( 6% , as has been reported in many autoimmune conditions ) the statistical power of 0.80 at a significance level of 0.05
percentages were compared by rates and proportion ; 95% confidence intervals ( ci ) were reported .
none of the 200 patients had a history of chronic diarrhea and all were taking antipsychiatric drugs ( antipsychotic such as risperidone , haloperidol or perphenazine and anticholinegic for schizophrenic patients , and fluoxetine and tricyclic antidepressants for depressive patients ) .
three patients with chronic psychiatric disorders were ttga positive , in which one ( age 52 years ) with schizophrenia and two ( both 30 years of age ) with chronic depression . of these three patients ,
two refused duodenal biopsy and one died during the study period . in the control group ,
one ( age 25 years ) out of 200 individuals was positive , but duodenal histology proved normal .
the prevalence of positive celiac disease serology in patients with chronic psychiatric disorders was thus slightly but not significantly higher than in controls ; 1.5% , ( 95% ci : 0.38 - 4.03 ) and 0.5% ( 95% ci : 0.00025 - 2.44 ) , respectively ( p = 0.06 ) . clinical features and laboratory findings among the patients with schizophrenia and depression are shown in table 1 .
five in the study group and none in the control group had selective iga deficiency .
clinical and laboratory features of male patients with schizophrenia and depression
ttga iga - class anti - tissue transglutaminase antibody ; meanstandard deviation
in our reports , the frequency of positive celiac disease serology in iranian inpatients suffering from depression or schizophrenia was 1.5% . by comparison , pynnnen et al .
have shown the prevalence of celiac disease in patients with depression to be 0.7% ( 5 ) .
the same authors have reported that in adolescent celiac disease patients the frequency of depression and disruptive behavioral disorders was higher than in controls , 31% and 7% , respectively ( 6 ) .
the present observations support earlier findings that celiac disease is not increased in patients with schizophrenia .
( 12 ) studied 7754 schizophrenia patients in denmark and found a frequency of untreated celiac disease of 0.05% . in uk , west et al . showed that in subjects with celiac disease the prevalence of schizophrenia was 0.25% , the adjusted odds ratios showing no association between the two conditions ( celiac disease vs. controls 0.76 , 95% ci : 0.41 - 1.4 ) ( 13 ) .
by contrast , a study in the uk revealed that patients with celiac disease developed schizophrenia 3 times more frequently than non - celiac controls ( 14 ) .
some studies have suggested that schizophrenia and celiac disease may be associated with similar or adjacent genes ( 15 , 16 ) .
it has indeed been reported that genetic susceptibility in schizophrenia lies in human leukocyte antigen ( hla ) dq , similarly to autoimmune disorders such as celiac disease ( 17 ) .
by contrast , a recent study showed no such hla association in schizophrenia ( 18 ) . in a case report
, the symptoms of schizophrenia were improved in a celiac patient aged 33 years after the introduction of gluten free diet ( 19 ) .
here we had no opportunity to investigate the effect of gluten - free diet , since two patients refused and one died during the study . in this present study ,
the frequency of positive celiac disease serology in patients with chronic depressive ( 2% ) and schizophrenia ( 1% ) was in fact similar to that found in healthy blood donors in iran ( 0.6% ) ( 9 ) . in the latter study ,
the frequency of celiac disease in males ( 1.8% ) was higher than in females ( 0.5% ) , although usually 60%-70% of celiac disease patients are female . we could not investigate females with psychiatric disorders , which may be considered as a limitation to the current study . on the other hand ,
all our patients were inpatients , indicating that they suffered from severe manifestations of chronic psychiatric disorders .
patients with selective iga deficiency remain negative by ttga iga class screening , and we had no opportunity to test our 5 such subjects by igg class ttga . in blood donors
there may thus be additional celiac case in our study group , but we consider that this would not change our conclusions . to conclude , mass screening for celiac disease in patients with depression or schizophrenia is not advocated . despite this , alertness to celiac disease should be high , since early diagnosis and treatment by gluten - free diet may ameliorate the symptoms and quality of life of these patients . | introductiontestosterone is needed for normal male development , muscle strength , bone mineralization , hematopoietic function , and sexual and reproductive functions .
the main purpose of androgen deprivation therapy in prostate cancer is to reduce tumor progression , but therapy is often accompanied by significant adverse effects.objectivethis study aimed to determine the effects of androgen deprivation therapy on body composition and resting metabolic rate in patients with prostate cancer.patients and methodsa prospective study was performed to evaluate the body composition of 16 elderly males ( aged 6396 ; median age 71 ) with prostate cancer scheduled for orchiectomy , one year before and after surgery .
body composition was measured by dexa , and energy expenditure , fat and carbohydrate oxidation were measured by indirect calorimetry.resultsbody weight ( p=0.01 ) , lean mass ( p=0.004 ) , and lipid oxidation ( p=0.001 ) decreased significantly .
carbohydrate oxidation ( p=0.02 ) , fsh ( p=0.0001 ) and lh ( p=0.0001 ) levels increased significantly .
changes in fat mass ( p=0.06 ) and bone mineral density ( p=0.48 ) were not significant.conclusionsafter 12 months of androgen deprivation therapy , elderly men with metastatic prostate cancer exhibit a decline in lean body mass and lipid oxidation , together with increased carbohydrate oxidation . |
we carried out a retrospective study by testing blood and serum samples on 1,740 patients who consulted the emergency department of cayenne hospital seeking treatment for fever compatible with malaria and/or dengue during a 1-year period , july 2004june 2005 ( figure ) .
diagnosis of malaria has always been quick ; dengue diagnosis was initially conducted only in malaria - negative patients . in our study ,
dengue investigations were conducted retrospectively at the pasteur institute of french guiana for 99% of patients ( 1,723/1,740 ) by using serum samples obtained at admission and frozen at 80c .
comparison of confirmed cases of dengue fever and of symptomatic malaria in patients examined at the emergency department of cayenne hospital , cayenne , french guiana , january 2004march 2006 .
the black frame corresponds to the period of the retrospective study ( july 2004june 2005 ) .
* cases confirmed by positive test results from reverse transcription pcr or virus isolation ( pasteur institute , cayenne ) .
cases diagnosed based on recorded fever or history of fever in the previous 24 h associated with microscopic detection of asexual forms of plasmodium spp . by blood smear .
malaria diagnosis was based on the identification of hematozoa on a thin blood film and/or on a thick blood film stained with giemsa .
the asexual parasite load ( pl ) was quantified in percent parasitized erythrocytes for values > 0.1 % . for lower values
, classes were created using thick smears : class 1 , < 0.00125% but positive ; class 2 , > 0.00125% but < 0.0125% ; and class 3 , > 0.0125% but < 0.125% .
carriage was considered for classes 1 and 2 ( in the absence of prior antimalarial treatment and for case - patients residing > 1 year in an area of malaria transmission ) . virus isolation or reverse transcription
( 8) was performed on all serum samples obtained during the acute phase of infection , between day 0 and day 4 ( n = 264 ) . for malaria - positive samples ,
virus isolation was conducted on all samples without a date of onset of disease ( n = 163 ) .
serologic immunoglobulin ( ig ) m testing was performed on all serum samples ( n = 1,723 ) .
dengue was detected in 238 case - patients ( 13.8% ) ; among these , 73% ( 174/238 ) were confirmed by positive virologic diagnosis ( isolation or rt - pcr ) , whereas 27% were probable dengue cases ( positive igm serology only ) .
the first group was named early dengue cases ( edc ) and the second group late dengue cases ( ldc ) . of the 1,723 patients , 393 ( 22.8% ) had smear - positive malaria ; of those , 251 ( 63.9% ) were p. vivax , 133 ( 33.8% ) were p. falciparum , 2 were p. malariae , and 7 were mixed p. vivax and p. falciparum .
most malaria - positive case - patients had a parasite count above class 2 ( 371/393 [ 94.4 % ] ) , indicating acute malaria .
concurrent dengue ( edc and ldc ) and malaria were confirmed in 17 of the 1,723 patients ( 1% ) , corresponding to 7.1% ( 17/238 ) of dengue cases and 4.1% ( 16/393 ) of malaria cases , respectively ( table ) .
when considering acute malaria associated with edc , the percentages of confirmed associations were 3.4% for dengue ( 6/174 , 95% confidence interval [ ci ] 0.76.2 ) and 1.6% for malaria ( 6/371 , 95% ci 0.32.9 ) .
all 17 associations were considered clinically as malaria , including the 2 case - patients with low parasite counts .
antimalarial drugs were administered promptly in every case . dengue serology and virology reports were available after the initial episode ; however , these results did not influence patient management . among the 6 acute concurrent infections ,
three patients were hospitalized , all in the igm - seropositive group , i.e. , ldc ; only 1 was severely ill .
this patient , who had p. vivax malaria infection , was hospitalized for interstitial pneumonia with severe anemia .
intubation , blood transfusion , and antimicrobial drugs were required , but he was discharged from the intensive care unit after 11 days .
the second patient was hospitalized for diabetes , the third because treatment with riamet ( arthemether + lumefantrine ) was only available to inpatients . * igm , immunoglobulin m ; rt - pcr , reverse transcription
pcr ; iso , isolate ; pv , plasmodium vivax ; , negative ; + positive ; den , dengue ; nd , not done ; pf , p. falciparum ; tp , thrombocytopenia++ ( < 100,000 platelets/l ) ; hb , hemoglobin ( reference range < 10
g / l ) ; bp , arterial blood pressure ( systolic / diastolic ) ; sat , blood oxygen saturation ; hr , heart rate ; bpm , beats per minute .
for diabetes requiring insulin . for treatment with riamet ( arthemether + lumefantrine ) .
malaria and dengue must be suspected in febrile patients living in or returning from areas endemic for these infections .
although the usual places of contamination by malaria and dengue viruses are quite different in french guiana , considering that the incubation phase is longer for malaria than for dengue and that the population s mobility is high , a simultaneous clinical expression of the 2 diseases is plausible . moreover , in french guiana , dengue viruses have spread to malaria - endemic rural areas ( 9 ) .
the confirmation of malaria is rapid , and after malaria is confirmed , dengue is usually ruled out without screening for it .
two methods can confirm dengue : dengue - specific igm seroconversion or detection of dengue virus particles during the acute phase ( day 0 to day 4 after onset of fever ) by rt - pcr , which is faster and more specific . in published case reports ( 17 ) , the diagnosis of dengue infection is usually made based on positive dengue igm ; however , this can not confirm recent dengue , because igm can persist for months and cross - react with other arboviruses ( 10 ) .
if rt - pcr requires a specific laboratory and can not be performed on site , a new test , the platelia , is now easily included in any laboratory and is indicated particularly for early - acute phase samples ( 11 ) . to investigate the frequency of dengue and malaria co - infection ,
the platelia test should be used in all cases of dengue - like or malaria - like syndrome , even when malaria diagnosis was positive , in regions where both infections may overlap . of the 1,723 patients investigated in this study , 17 had concurrent dengue and malaria . in 10 of these patients , recent acute dengue fever could not be confirmed ( ldc ) .
two patients , 1 of whom was part of the edc group , could have been asymptomatic carriers of plasmodium spp .
( 1 patient with p. falciparum and 1 with p. vivax ) because of low parasitemia ( 12 ) . a true acute concurrent infection ( strictly defined diagnosis )
concurrent acute malaria and recent dengue fever had a lower frequency than predicted by the multiplication of both prevalences , but such reasoning implies the same overlapping contamination areas for malaria and dengue , which it is not the situation in french guiana
. the greater prevalence of ldc than edc associated with acute malaria infection illustrates the prolonged persistence of specific igm or igm cross - reaction , which increases the probability of a malaria case when comparing the short 45 day period corresponding to edc .
virologic investigations using isolation or rt - pcr techniques were not performed on samples taken after the 4th day because of the usual disappearance of viremia .
additional associations where fever was initially caused by malaria and followed by dengue after the 4th day of malaria fever could have been undiagnosed .
edc were diagnosed on average after 4 days of fever , never 5 .
thus , delayed complications of dengue or malaria may not be detected using this definition .
one of these patients had pneumonia , which has recently been described as a complication of p. vivax ( 13 ) .
although acute concurrent infections were benign in our study , special attention should be given to the possibility of co - infection with malaria and dengue , especially when p. falciparum is implicated .
the distinction between severe dengue and severe malaria must be made in an emergency department or hospital setting because in both situations , early diagnosis is essential for patient care . lysosomes are cellular organelles that play a pivotal role in the cell homeostasis through their involvement in degradation and recycling processes of extracellular material that has been internalized by endocytosis and intracellular components that have been sequestered by autophagy ( 1 ) .
lysosomes may also fuse with the plasma membrane , emptying their contents outside the cell .
this is important for processes such as cellular immune response and plasma membrane repair , both in normal and pathological conditions ( 2 , 3 ) .
mutations that cause lysosomal enzyme deficiencies result in different syndromes , known as lysosomal storage disorders ( lsds ) ( 4 ) .
in addition , they are characterized by intracellular deposition and protein aggregation , events also found in age - related neurodegenerative disorders , such as alzheimer s and parkinsons s diseases ( 57 ) .
these studies underline the importance of the lysosome as a central player in cell metabolism .
hence , the characterization of genes participating in lysosomal biogenesis and function is a critical step toward the understanding of basic processes in cell biology and pathogenic mechanisms in many human diseases .
recently , it was found that most lysosomal genes exhibit coordinated transcriptional behavior and are regulated by the transcription factor eb ( tfeb ) , which also links autophagy to lysosome biogenesis ( 8 , 9 ) . gene expression at the post - transcriptional level can be regulated by micrornas ( mirnas ) .
mirnas play important roles in diverse biological processes , including development , cell differentiation , proliferation and apoptosis , in which the lysosomal system also plays an important role .
notably , mirnas have been recently identified as involved in the regulation of autophagy ( 10 , 11 ) .
the human lysosome gene database ( hlgdb ) is the first searchable database focused on the census of genes belonging to the lysosomal system and on their regulation by mirnas .
no database resources entirely dedicated to the regulation of lysosomal genes by mirnas or other regulators are currently available .
several lists of lysosomal genes were collected from public gene databases , published proteomics articles and reviews edited by biochemists and cell biologists working in the lysosome field .
we paid special attention on balancing predictions , which were as follows : ( i ) more suitable to look for confirmatory evidence ( targetscans ) ( 15 ) ; ( ii ) more suitable to identify any possible target for a particular mirna , to form the basis for in vitro or in vivo experiments ( pictar four - way and five - way ) ( 16 ) ; ( iii ) more suitable to find in silico evidence for the interaction between a mirna and a gene of a certain family or function ( pita , miranda ) ( 17 ) . to increase mirna - target mrna information , experimentally verified mirna targets from mirtarbase were also reported .
hlgdb aims to providing a useful resource to anyone studying the lysosomes and a tool for identifying common regulatory features of lysosomal genes .
hlgdb provides a user - friendly interface through which information can be easily retrieved , including the union and intersection of different gene lists , searches for mirna predictions and visualization on the gene transcript sequence of the mirna target predictions .
the data reported in the current version 1.1 , derived from ncbi pubmed searches for review articles regarding human ( 8 , 1825 ) and murine ( 22 , 2629 ) proteome of the lysosome and from lists of lysosomal genes present in the gene ontology ( 30 ) , kegg ( 31 ) , reactome ( 32 ) and uniprot databases ( 33 ) [ uniprot : lysosome ( kw-0458) and organism : homo sapiens ( human ) ( 9606) ; kegg : lysosome ( ko04142) ; go : go:0005764 data stamp from the source 20120303 ] .
the references listed within each review article ( 34 , 35 ) were examined and lists of genes were extracted from either the full text or the supplementary information following a manual curation .
there are 16 sources of information divided in four main categories : proteomic studies , databases , reviews and system biology approaches .
each gene has been associated to its official hgnc gene symbol ( 36 ) and to its entrez gene i d ( mappings were based on data provided by entrez with a date stamp from the source of 7 march 2012 ) .
the gene transcripts associated to each gene are annotated accordingly to ncbi refseq or genbank ( release 57 ) .
mirna target predictions were extracted from the tables downloaded from the websites of the different algorithms used to predict the binding between mirna and gene transcripts .
coordinated lysosomal expression and regulation ( clear ) is a nucleotide motif ( gtcacgtgac ) found to be highly enriched in the promoter set of lysosomal genes ( 8) .
we mapped this motif on both strands on the human genome ( hg19 ) by means of fuzznuc utility of the emboss package allowing one single mismatch ( 37 ) .
the binding sites of the tfeb come from a chip - seq experiment carried out on hela cell lines ( 38 ) .
hlgdb is a mysql 5.0.95 database ( constructed in the fourth normal form , some redundancy being kept to increase retrieval performance ) , and the interface is built in php .
search for gene lists : hlgdb can be used to retrieve and combine lists of lysosomal genes from different sources . the any and all options allow the user to either merge or intersect lists .
search for a gene using the gene symbol is also allowed ( figure 1 ) .
search for mirna targets : once the user has selected or created a gene list , he / she can find mirna ( or families of mirna ) targets choosing different combinations of prediction softwares .
results are returned in a table showing information about the gene ( gene symbol and gene name ) and the mirna ( identifier and number of softwares predicting each binding ) .
gene symbols are hyperlinked to gene - centered page where additional gene information is provided ( figure2a ) . on each gene transcript associated to the selected gene ,
search for tfeb binding sites / clear motifs : once the user has selected or created a list , he / she can choose the filter and find the lists of genes with tfeb binding sites or clear motifs within a range around the transcription start site ( tss ) .
results are provided in the table showing gene - centered information , as the hit count and distance from tss of tfeb binding sites / clear motifs .
all data in hlgdb are freely available for download as tab - delimited text files without password protection for any user
. concerning other organisms , currently the database provides a parallel orthology annotation for mouse : the user can select the species of interest using the upper right botton .
it collects information about these genes and their transcriptional regulation such as tfeb binding sites and mirnas .
hlgdb was designed to become a lysosomal gene census . when new lysosomal genes will be discovered
lysosomal genes include lysosomal hydrolases , lysosomal membrane proteins , lysosomal proteins involved in acidification and non - lysosomal proteins fundamental for this organelle biogenesis .
in addition , there is increasing evidence that lysosome genes play a role in the pathogenesis of common neurodegenerative diseases such as alzheimer s , parkinson s and huntington s .
researchers may benefit from hlgdb because they have in a single reference to the broadest compendium of lysosomal gene lists .
results of mirna targets may be directly compared with other transcriptional regulation elements such as the distance from the tss of tfeb binding site or the distance to a clear sequence to identify common features of regulation .
hlgdb has been designed to integrate additional layers of biological information , such as experimental data and comparative genomics . currently the database present information for human and mouse species ; in the next versions , additional species , such as rat , will be integrated .
finally , hlgdb provides a powerful resource to system biology approaches and network analysis to dissect the map of interactions taking place in the lysosomal system .
| dengue malaria co - infection reports are scarce . of 1,723 consecutive febrile patients in cayenne hospital ,
238 had dengue ( 174 early dengue fever cases ) and 393 had malaria ( 371 acute malaria ) ; 17 had both .
diagnosis of 1 of these 2 infections should not rule out testing for the other infection . |
a malignant peripheral nerve sheath tumor ( mpnst ) is a sarcoma that arises from peripheral nerves or cells of the associated nerve sheath ( schwann cells , perineural cells , fibroblasts ) .
the term " mpnst " has substituted previously used terms such as malignant schwannoma , neurofibrosarcoma , and neurogenic sarcoma1,2 ) .
the mpnsts comprise approximately 5% to 10% of all soft tissue tumors and have an incidence of 0.001% in the general population and the etiology is unknown .
however , more than half of mpnst cases develop in patients with neurofibromatosis type-1 ( nf-1 ) and there is a higher incidence in patients that have undergone prior radiotherapy1,2,5,8,13 ) .
mpnsts are most likely to metastasize to the lungs , followed by the bone and finally the pleura , but , spinal cord and brain metastasis is very rare in non - nf-1 mpnst3,7 ) .
the authors report a very rare case of spinal cord and brain metastasis of mpnst in the lumbar spine that occurred in a 18-year - old man without nf .
an 18-year - old man presented initially with a 6-month history of low back pain that worsened over time .
one month prior to admission , the patient developed radiating pain to his anterior thigh .
neither caf au lait spots nor neurofibromas were seen and his family history was absent of nf-1 .
the neurologic examination showed normal motor power in his lower extremities . magnetic resonance imaging ( mri ) showed a paraspinal mass that extended from the central space of l2 to the right psoas muscle through right l2 - 3 foraminal space .
it was an asymmetrical dumbbell shaped mass and the thecal sac was displaced left by the mass .
the mass showed iso - signal intensity on t1-weighted images and mixed signal intensity on t2-weighted images .
a paramedian incision was made from the l1 to l4 and we exposed the right l2 and l3 lamina with transverse process .
right l2 hemilaminectomy was performed using a high speed drill and kerrison rongeur to expose the thecal sac .
the margin of mass was relatively distinct from neural tissue , thus gross total resection was possible under microscopic view . to remove the mass extended to the psoas muscle ,
the right transverse process of l2 and l3 was resected and the mass was visible .
after the operation , his symptom had improved . however , a few days later , his confirmed pathologic report was presented as mpnst ( fig .
the contrast - enhanced mri was followed at 1 month after the operation and revealed a total removal of tumor ( fig .
however , the patient declined the external beam radiation due to the fear of potential radiation risk .
spine and brain mri showed that the tumor recurrence with leptomeningeal , multiple spinal cord and brain metastases ( fig .
4 ) . complete surgical clearance of the tumor was not technically feasible , owing to the multiple metastases .
therefore , the patient was referred for adjuvant chemotherapy . in addition , intrathecal chemotherapy was performed .
after 4 months of chemotherapy , he was referred to the hospice hospital and died within a month .
an 18-year - old man presented initially with a 6-month history of low back pain that worsened over time .
one month prior to admission , the patient developed radiating pain to his anterior thigh .
neither caf au lait spots nor neurofibromas were seen and his family history was absent of nf-1 .
the neurologic examination showed normal motor power in his lower extremities . magnetic resonance imaging ( mri ) showed a paraspinal mass that extended from the central space of l2 to the right psoas muscle through right l2 - 3 foraminal space .
it was an asymmetrical dumbbell shaped mass and the thecal sac was displaced left by the mass .
the mass showed iso - signal intensity on t1-weighted images and mixed signal intensity on t2-weighted images .
a paramedian incision was made from the l1 to l4 and we exposed the right l2 and l3 lamina with transverse process .
right l2 hemilaminectomy was performed using a high speed drill and kerrison rongeur to expose the thecal sac . for removal of intradural portion
the margin of mass was relatively distinct from neural tissue , thus gross total resection was possible under microscopic view . to remove the mass extended to the psoas muscle ,
the right transverse process of l2 and l3 was resected and the mass was visible .
after the operation , his symptom had improved . however , a few days later , his confirmed pathologic report was presented as mpnst ( fig .
the contrast - enhanced mri was followed at 1 month after the operation and revealed a total removal of tumor ( fig .
however , the patient declined the external beam radiation due to the fear of potential radiation risk .
spine and brain mri showed that the tumor recurrence with leptomeningeal , multiple spinal cord and brain metastases ( fig .
4 ) . complete surgical clearance of the tumor was not technically feasible , owing to the multiple metastases .
in addition , intrathecal chemotherapy was performed . however , no significant effect was observed and the patient 's condition deteriorated gradually .
after 4 months of chemotherapy , he was referred to the hospice hospital and died within a month .
non - nf-1 mpnsts are very rare and the occurrence of mpnsts occurring in adolescents is very rare
. mpnsts generally occur in adulthood typically between the ages of 20 and 50 years of age .
approximately 10 - 20% of cases have been reported to occur in the first 2 decades of life , with occasional cases involving infants as young as 11 months of age5,6 ) .
hruban et al . described 43 patients with mpnst and of these patients , 28 ( 65% ) had distant metastasis3 ) .
sites of metastasis included the lungs ( 22 cases ) , bone ( 9 cases ) , pleura ( 6 cases ) , retroperitoneum ( 4 cases ) , diaphragm ( 3 cases ) , inguinal lymph nodes ( 2 cases ) , liver ( 2 cases ) , chest wall ( 2 cases ) , soft tissue ( 2 cases ) , pulmonary hilum , pericardium , thyroid gland , and adrenal gland ( 1 case each ) .
described 11 distant metastasises in 54 patients with mpnst that included metastases to lymph nodes , pleura , lung , liver , adrenal gland , leptomeninges and brain7 ) .
william et al.4 ) reported a non - nf-1 mpnst case of metastasis to the spinal cord4 ) .
our case showed that leptomeningeal , spinal cord and brain metastasis of non - nf-1 mpnst for relatively short period after tumor removal in young male patient , reflecting aggressive nature of this malignancy .
the outcome with respect to both local recurrence and distant metastasis largely depends on grade of surgical excision .
radiation therapy combined with wide surgical excision offers statistical significant reduction in the rates of local disease recurrence .
however , it has not had a meaningful reduction in either rate of distant metastasis or overall survival15 ) .
benefit of chemotherapy is unproven and its application is limited to high grade metastatic disease3 ) .
the italian and german soft tissue sarcoma cooperative group reported an overall pediatric response rate of 45% , with the highest response noted within the ifosfamide group9,11,12 ) .
the recurrence rate for mpnsts has been reported to range from 40 - 68%7,10,14 ) .
the 5-year survival rate has been reported at 16 - 52% and a favorable prognosis has been related with complete surgical excision of the tumors that are sized less than 5 cm7,10 ) .
historically , mpnsts have been difficult tumors to treat due to their inherently aggressive nature and dismal prognosis .
our patient underwent complete surgical excision , but the patient was not treated with radiotherapy .
follow - up images revealed multiple spinal cord and brain metastases . the patient was managed with chemotherapy , but expired several months later . despite complete surgical excision ,
we reported a case of mpnst that had metastasized to the brain and spinal cord .
because the mpnst showed both rapid and aggressive progression , patients should be followed carefully to identify local recurrence or metastasis and be mandatory an adjuvant radiotherapy . osteoid osteoma is a benign bone tumor consisting of an osteoid nidus in a highly vascular connective tissue stroma .
these tumors are exquisitely painful , and demonstrate characteristic findings on clinical and radiographic examinations .
the major drawback of this traditional approach is prolonged surgery and hospital stay , weakening of the bone requiring a prophylactic fixation , and its subsequent removal and additional time off school due to open surgery . to overcome this drawback
, different minimally invasive techniques have been described as alternative therapeutic options ( radiofrequency ablation , laser photocoagulation , and percutaneous resection ) .
computed tomography ( ct ) guidance has become a very useful and easy method for the percutaneous treatment of these lesions .
we started using ct guided radiofrequency ablation for osteiod osteoma 5 years ago and immediately noted many beneficial effects in terms of duration of hospital stay , morbidity , and overall patient comfort prompting us to do this study .
we report our experience with ct - guided percutaneous radiofrequency ablation ( rfa ) of osteoid osteoma in common and technically challenging locations in 30 pediatric patients and evaluate technical and clinical results .
this is a retrospective observational study of 30 pediatric and adolescent patients who underwent ct - guided percutaneous rfa of osteoid osteoma between june 2009 to may 2014 .
all patients reported severe pain that usually increased at night and required nonsterioidal anti - inflammatory drugs for pain relief .
the osteoid osteoma was diagnosed from clinical and imaging findings ( radiography , ct , and/or magnetic resonance imaging ( mri ) scan ) , demonstrating a nidus and other findings that are typical of osteoid osteoma .
patients and their legal guardians were fully informed of the procedure as well as of the surgical and medical alternatives , and informed consent was obtained .
all procedures were performed under general anesthesia in the ct room on six slice ct scanner ( philips brilliance , massachusetts , usa ) .
a guidewire ( k - wire ) was drilled into the center of the nidus using either a hand drill ( aesculap inc .
b braun , center valley , pa ) or a battery operated drill ( stryker corp . , kalamazoo , mi ) with a cannulated drill bit ( 2.54.5 mm ) ( zimmer , warsaw , in ) along the planned tract by the pediatric orthopedist ; keeping the cannulated drill bit in nidus of osteoid osteoma
, k - wire was exchanged for the stiff end of the amplatz wire ; a 5f sheath which was cut near hub and a linear cut along its entire length was made to allow it to expand and peel off when rfa probe was inserted through ; subsequently , a 4 mm cannulated bone drill was exchanged for a 5f sheath over an amplatz wire .
( 17 gauge 2 cm diameter , 12 cm long , three tines ) angiodynamics , inc .
, ga , usa ] was inserted into the nidus of osteoid osteoma through the sheath .
sheath was withdrawn approximately 2 cm back , and the tines were opened and confirmed to be within the lesion with check ct sections . using the radiofrequency waves from rf generator ( model 1500x ; angiodynamics , inc .
, ga , usa ) , the tines were heated to a target temperature of 90c and power of 60100 w and the peak temperature was maintained for 6 min .
after 6 min , the tines were withdrawn into the probe and then the rf probe was removed .
the treatment success was evaluated in terms of pain relief before and after procedure ( 1 day , 1 month , and 3 months ) .
patients were also contacted by telephone at 6 months for follow - up regarding complications or recurrence of pain .
technical success was defined as the ability to localize the radiolucent nidus and placement of an electrode under ct guidance with ablation performed for the desired period .
clinical success was defined as complete relief of pain without the use of oral pain medication within 1 month of the procedure .
from june 2009 to may 2014 , 30 pediatric patients underwent ct - guided rfa of osteoid osteoma .
there were 25 boys and 5 girls , with a male - to - female ratio of 5:1 .
their age ranged between 4 years to 20 years with a mean age of 13.16 years .
lesions were grouped into common and challenging location . among the common location ( n = 25 )
, lesions were located in the femur ( n = 21 ) [ figure 1 ] and tibia ( n = 4 ) . among the challenging locations ( n = 5 ) , four were near articular surface ( one each at glenoid fossa of right scapula , head of right radius , talocalcalcaneal joint of right calcaneum [ figure 2 ] and left femoral head ) and one was in the left sacrum .
radiography ( a ) and ct scan bone window axial image ( b ) show a radiolucent nidus and surrounding reactive sclerosis .
( d ) ct scan post procedure shows rf ablation tract a 17-year - old boy with a right calcaneal osteoid osteoma near the talocalcaneal joint margin .
( a ) magnetic resonance imaging ( stir ) sagittal image shows central nidus surrounded by hypointense reactive sclerosis .
rest of the surrounding calcaneum appear hyperintense due to reactive edema due to osteoid osteoma .
( b ) ct scan shows rfa probe in situ in nidus technical success was achieved in all patients ( 100% ) .
primary clinical success was 96.66% ( 29 of total 30 patients ) , despite pediatric population and challenging location .
one patient with an osteoid osteoma in the shaft of right femur had persistent pain after 1 month and was treated successfully with a second procedure ( secondary success rate 100% ) .
one patient from the challenging location group with osteoid osteoma at right radial head had immediate post - procedure weakness of wrist and finger extension because the lesion was very close to posterior interosseous nerve , which slowly recovered with physiotherapy .
since the promising results of rosenthal et al . , in the management of osteoid osteoma with rfa , a large number of studies evaluating rfa of osteoid osteoma have been reported in the literature .
most of these studies found very high technical success rates ( 100% ) and good primary success rates with a single session of ablation ranging from 76% to 100% .
hence , this minimally invasive technique has become the method of choice for treatment of osteoid osteomas , provided that the diagnosis is based on a typical clinical , scintigraphy , and ct presentation . to our knowledge
, there are few reports in the literature regarding the role of percutaneous rfa in treating osteoid osteomas in children at atypical locations .
our technical success rate was 100% , which is similar to most of the other studies ( 100% ) . in our study in the pediatric population , the primary and secondary clinical success ratings were 96.66% and 100% , respectively , which are comparable to success rates in most other studies among adults , where primary success and secondary success ranged from 76% to 100% and 87% to 100% , respectively . in a study done by donkol et al .
on the efficacy of rfa of osteiod osteoma in children showed that the technical success , primary clinical success , and secondary clinical success rates were 91.3% , 78.2% , and 82.6% respectively .
and vanderschueren et al . , in their studies , showed that lower age can be a risk factor for lower clinical success rate .
this can be explained by the greater technical difficulty during ablation of osteoid osteoma in children due to the small body mass , difficulty in positioning and fixation of the needle , and shorter ablation time ( range : 26 min in their study ) for each procedure .
our study showed that if technical success is 100% and if strict desired temperature ( 90c ) can be maintained for desired time ( 6 min ) using controlled power ( wattage ) delivery ( 60100 w ) then high clinical success can be achieved even in pediatric population similar to adult population . in our study ,
this happened in a child with radial head ( technically challenging position ) osteoid osteoma who had developed an immediate post procedure weakness of wrist and finger extension as the lesion was very close to posterior interosseous nerve .
there are few case reports of likely articular cartilage damage in weight bearing joints such as acetabulum following ct - guided percutaneous rfa of juxta - articular osteoid osteoma , however , it was not confirmed whether the articular cartilage was damaged only by head due to rfa or if it had also been weakened before by the osteiod osteoma .
on rfa of intra - articular osteoid osteoma of the hip showed that there is good ossification and bone regeneration following rfa and that it is a safe and effective treatment even for intra - articular lesions .
no delayed complications were observed in our study . in comparison to percutaneous ct - guided curettage of osteiod osteoma , during rfa there is lack of confirmation of histological diagnosis . however , there are other disadvantages of curettage technique that it is little more traumatic with complications of neuropraxia , skin abrasions , damage to blood vessels especially in femur leading to avascular necrosis , and frequently incomplete curettage .
we suggest that when the clinical and radiological features are not typical , the technique should be supplemented with a core biopsy from the nidus area .
this is specially indicated when the differential diagnosis is infection as management will be required .
limitations of our study are that it is an observational study , lack of confirmation of histological diagnosis , and lack of imaging follow - up .
percutaneous ct - guided rf ablation is a relatively safe , highly effective , and minimally invasive procedure for the treatment of osteoid osteoma in pediatric population despite atypical location .
| a malignant peripheral nerve sheath tumor ( mpnst ) is a type of sarcoma that arises from peripheral nerves or cells of the associated nerve sheath .
this tumor most commonly metastasizes to the lung and metastases to the spinal cord and brain are very rare .
we describe a case of young patient with spinal cord and brain metastases resulting from mpnst .
an 18-year - old man presented with a 6-month history of low back pain and radiating pain to his anterior thigh . magnetic resonance imaging showed a paraspinal mass that extended from the central space of l2 to right psoas muscle through the right l2 - 3 foraminal space .
the patient underwent surgery and the result of the histopathologic study was diagnostic for mpnst .
six months after surgery , follow - up images revealed multiple spinal cord and brain metastases .
the patient was managed with chemotherapy , but died several months later . despite complete surgical excision ,
the mpnst progressed rapidly and aggressively .
thus , patients with mpnst should be followed carefully to identify local recurrence or metastasis as early as possible . |
dr . coyne - beasley receives research funding from merck & co. none of these funds were used in the conduction or completion of the research contained in this manuscript .
this article was supported by grants from the national institutes of health 1r01ai113305 - 01 ( cates pi ) , and by the north carolina translational and clinical sciences institute , through support from the national center for advancing translational sciences ( ncats ) , national institutes of health , grant award number 1ul1tr001111 .
the content is solely the responsibility of the authors and does not necessarily represent the official views of the nih . periodontitis causes loss of tooth supporting bone . the bone loss may be vertical , horizontal , or combined .
regenerative periodontal therapy aims to predictably restore the structure and function of these lost tissues . for this purpose ,
these grafting materials are available in different forms . among the graft materials , autogenous bone grafts
have been considered the gold standard and can be harvested in either particulate or in the form of bone blocks . with the advancement in techniques and materials used for regeneration ,
sources of bone include bone from healing extraction wounds , bone from edentulous ridges , bone trephined from within the jaw , exostoses , and bone removed during osteoplasty and ostectomy .
bone removed from the donor site during osteoplasty has the advantage of eliminating the need for additional donor site while accomplishing the objective of obtaining desirable bone contour in the surgical site .
the obvious advantage of this technique is the ease of obtaining bone from already exposed surgical sites , but the disadvantages are its relatively low predictability and inability to procure adequate material for large defects . in this case report ,
bone from ledges present on the mandibular posterior area was removed with the simple technique of scrapping with a sharp sickle scaler and the resulting graft was used to fill a two wall defect distal to first molar .
a 37-year - old male patient reported to the department of periodontology government dental college , calicut with a chief complaint of bleeding from gums and bad breath of 3 months duration . on clinical examination , generalized moderate to deep periodontal pockets and bleeding on probing was noticed .
based on the history , clinical and radiographic findings , the patient was diagnosed with chronic generalized periodontitis .
six weeks after performing thorough scaling and root planning and following a strict plaque control regime , the case was reevaluated .
since the pocket depth was 7 mm distal to 36 , it was decided to proceed with flap surgery [ figure 1 ] . after giving block anesthesia in the left mandibular posterior area ,
meticulous defect debridement and root planing was done to remove subgingival plaque , calculus , and inflammatory granulation tissue .
a deep two wall defect was observed distal to first molar , heavy ledges were noticed extending bucally from second premolar to the second molar area [ figure 2 ] .
first it was decided to remove the ledges as a part of osteoplasty procedure and then utilize the removed bone to fill the two wall defect . for this purpose , a sharp sickle scaler ( pdt cruise scaler u15 - 33 r113 , pdt inc . , us ) [ figure 3 ] was used in a scrapping motion with sufficient force such that the bone was essentially shaved off [ figure 4 ] .
the harvested bone was carefully collected in a dappen dish and then transferred to the defect with a periosteal elevator [ figures 5 and 6 ] .
the flap was repositioned , single interrupted sutures were placed and periodontal dressing was given [ figures 7 and 8 ] . the patient was reviewed after 1-week , the healing was found to be uneventful .
the patient was put on the maintenance phase with oral prophylaxis performed every 3 months .
clinically , there was a reduction in probing pocket depth from 7 mm to 3 mm [ figure 9 ] .
radiographs revealed radio opacity in the site distal to first molar , suggestive of bone fill in the defect [ figure 10 ] .
defect exposed , ledges seen ( a and b ) pdt cruise scaler u15 - 33 r113 used to remove ledge autograft obtained from ledge removal with sickle scaler autogenous graft placed in the defect distal to first molar postoperative view after 1-year ( a ) preoperative radiograph , ( b ) postoperative radiograph after 1-year
there is ample evidence that autogenous bone grafts shore up the formation of new attachment .
autogenous bone , long considered the gold standard of grafting materials , is currently the only osteogenic graft available to clinical practitioners .
grafted autogenous bone heals into growing bone through the processes of osteogenesis , osteoinduction , and osteoconduction .
moreover , auto - grafts are bioabsorbable , nonallergenic , easy to handle , and not costly .
rapid revascularization occurs around autogenous bone graft particles , and the graft can release growth and differentiation factors .
although iliac crest is one of the most preferred site , it is not always recommended due to its associated problems , such as postoperative infection ; exfoliation and sequestration ; varying rates of healing ; root resorption and rapid recurrence of the defect , in addition to increased patient expense and patient morbidity .
intramembranous ( craniofacial origin ) bones have been found to have more survival potential than endo - chondral ( long bones ) origin .
insulin - like growth factor - ii and transforming growth factor- have been found to be in a greater concentration in calvarial bone than in bone from the iliac crest or vertebral body .
this increase in concentration of growth factors leads to a greater capacity for bone repair and graft retention .
the use of intraoral graft material is , however , limited by the restricted donor sites in the oral cavity for extensive grafting .
the use of bone removed from ledges as a part of osteoplasty procedure offers the added advantage of eliminating the nonphysiologic bony architecture .
the goal of osseous respective therapy is to reshape the marginal bone to resemble that of the alveolar process undamaged by periodontal disease .
the reshaping process is an attempt to gradualize the bone sufficiently to allow soft tissue structures to follow the contour of the bone . in this case ,
radicular blending was done using the sharp blade of scaler to gradualize the bone over the entire radicular surface providing a smooth , blended surface for good flap adaptation .
many techniques and devices are available to harvest intraoral autogenous bone grafts , such as : bone scraper , rotary instruments , bone chisels , rongeur pliers and piezoelectric devices .
particulate graft material is easy to procure and is best suited for two , three , or more walled defect , as they usually provide adequate space for placing and retaining the bone graft material for a sufficient period .
the commonly used technique to procure particulate grafts is the use of carbide bur at speeds between 5000 and 30,000 rpm .
the inability to procure adequate material for large defects is a major disadvantage of using rotary instruments .
conventional osteotomy or milling procedures has some limitations like overheating of the bone when water cooling is insufficient , which leads to possible structural bone changes on living cells .
a simple technique for removal of bone using a sharp sickle was done , in this case , which turned out to be effective means for harvesting sufficient amounts of bone graft .
although specialized bone scrapers have been used for obtaining grafts from intraoral sites before . to the best of our knowledge , there are no previous reports of bone harvesting done with scaler alone .
reported higher percentages of cultured osteoblast cells for both rotary drill and the piezoelectric device , 88.9% and 87.9% , respectively .
they concluded that the harvesting methods are not different concerning the detrimental effect on the viability .
another study by bacci et al . showed contradictory results , their histological findings revealed that smaller particle size bone chips , obtained with a piezoelectric device , had less vital bone ( 64.83% ) compared with the small particle size bone chips harvested with a bone scraper ( 75.34% ) . in this case , sufficient amounts of autogenous particulate bone were obtained during the ledge removal process to fill the two wall defect distal to first molar .
instead the sharp blade of the sickle scaler was utilized , which was also less time consuming .
autogenous grafts have long remained and will continue to remain as the first preference for bone grafting procedures because of its superior biologic properties .
ledges are frequently found in posterior regions and if properly managed can act as a source of autogenous graft .
routinely used scaling instruments may be utilized in certain situations instead of using specialized bone scrapping instrument . | a significant barrier to the delivery of hpv vaccine is reluctance by both healthcare providers and parents to vaccinate at age 11 or 12 , which may be considered a young age .
this barrier has been called vaccine hesitancy in recent research . in this commentary
, we suggest using social marketing strategies to promote hpv vaccination at the recommended preteen ages .
we emphasize a critical public health message of a sexually transmitted infection ( sti ) as preventable and vaccination against hpv as a way to protect against its consequences .
the message tackles the issue of vaccine hesitancy head on , by saying that most people are at risk for hpv and there is a way to prevent hpv 's serious consequences of cancer .
our approach to this conversation in the clinical setting is also to engage the preteen in a dialog with the parent and provider .
we expect our emphasis on the risk of sti infection will not only lead to increased hpv vaccination at preteen ages but also lay important groundwork for clinical adoption of other sti vaccines in development ( hiv , hsv , chlamydia , and gonorrhea ) as well as begin conversations to promote sexual health . |
temporary external ventricular drainage for refractory thoracolumbar cerebrospinal fluid ( csf ) leak is not reported in the literature .
we present a young man who had multiple complex spinal surgeries for resection of innumerable metastatic ependymoma lesions .
as a last resort , we decided to place an external ventricular catheter for proximal controlled csf drainage with great success .
the 32-year - old man had a history of spinal myxopapillary ependymoma world health organization ( who ) grade i with local metastatic spinal disease . the patient first presented in 2005 with a 1-year history of severe low back pain . he was found to have an intradural lesion , and subsequently had a t12l3 laminectomy for resection of the tumor .
small amounts of the tumor adherent to the nerve roots were purposefully left behind . each time an attempt was made to remove these residual small pieces of the tumor , significant amounts of neuromonitoring activity was noted .
hence , the residual tumor was left behind so as to not cause a neurologic deficit .
approximately 10 days after surgery , the patient fell at home and noticed significant amounts of a clear fluid consistent with csf leaking from his incision .
he was taken to the operating room at that time and a dural defect was identified and closed primarily , then covered with gelfoam ( baxter healthcare , deerfield , il , united states ) and tisseel ( baxter healthcare ) . the patient again noted another csf leak from a different area of his incision 5 days later , and he was taken back to the operating room for another primary closure , as well as placement of a fat graft and more tisseel .
adjuvant radiation treatment was undertaken after he recovered from the initial surgeries . in 2008 ,
he was found to have a dorsally located intradural lesion at the t8t9 region , which was resected via a t7t10 laminectomy . in 2010 ,
he was found to have growing intradural lesions at l4 and l5 . at that time
one year later , our patient presented yet again with multiple thoracolumbar intradural lesions ( fig .
he underwent redo decompressive laminectomies from t8s1 with a total of 13 intradural tumors being excised ( fig .
2 ) . the dural closure proved to be quite complex , as there were several areas that were macerated and very difficult to reapproximate .
fat grafts , duraform ( depuy synthes , west chester , pa , united states ) , and tisseel were all used on top of the attempted primary dural closure .
preoperative sagittal t2-weighted magnetic resonance imaging ( mri ) ( a ) of the thoracic spine revealing multiple intradural tumors as well as an axial t2-weighted mri ( b ) revealing significant compression of the spinal cord .
postoperative t2-weighted sagittal ( a ) and axial ( b ) magnetic resonance imaging revealing gross total resection of multiple intradural tumors .
radiographic evidence of a refractory thoracolumbar cerebrospinal fluid leak was not readily evident on the immediate postoperative films . during his hospital course , the patient developed a spontaneous csf leak from his wound .
it was initially treated by oversewing the skin , but the leak persisted over the next day .
we decided to bring the patient back to the operating room for an exploration and an attempt at repairing the dural defects .
multiple dural defects were noted . as a result of the significant dural maceration , another primary closure was not attempted .
we placed more pieces of duraform , fat graft , and tisseel over the areas of csf leakage , and we again performed a tight multilayer closure of the muscle , fascia , fat , and subcutaneous tissue . despite our best efforts to stop the csf leak , the leak continued through the incisions from multiple areas .
as a last resort , we decided to place a right frontal external ventricular drain ( evd ; integra life sciences , plainsboro , nj , united states ) after obtaining a preprocedure computed tomography scan of the head , to ensure the ventricular size could accommodate the catheter ( fig .
he was placed on antibiotics for both the pneumonia as well as the csf leak and subsequent meningitis . on the day 8 of the evd in place
, we continued to see cessation of the csf leak and decided to perform a 3-day clamping trial .
no further csf leak was noted from the incision during the clamping trial . the evd was removed , and the patient was eventually discharged home without further complications .
preprocedure computed tomography scan of the head to ensure the ventricular size could accommodate an external ventricular drain ( a ) .
postprocedure scan showing successful placement of a right frontal external ventricular drain ( b ) .
a watertight dural closure is essential in reducing the risk of csf leakage in spinal surgery .
eismont et al recommended the meticulous closure of the dura at the time of surgery and reoperation and repair of dural tears upon the discovery of a persistent csf leak.1 conservative management may be initially attempted with bed rest for 3 days with subsequent slow advancement to ambulation .
eighty - six of eighty - eight patients with dural tears studied by wang et al had resolution of csf leaks with primary dural closure at the time of surgery followed by bed rest.2 others have confirmed the efficacy of treating such leaks via csf diversion in the lumbar region.3
a review of the literature revealed scarce reports of csf leakage in more complex patients such as the patient in this case .
this complexity arose from the multiple surgeries that were performed due to the recurrence of this intradural disease .
they are benign , slow - growing , who grade i tumors that are amenable to surgical resection with optimal outcomes when gross total resection is achieved .
although pathologically they may be low grade , they can be metastatic locally within the spine via the csf route .
recurrence of the disease is usually in the same region and is substantiated by subtotal or piecemeal resection.4
spine surgeons occasionally encounter lumbar csf leak .
routine iatrogenic csf leaks can usually be repaired primarily by suturing the dural defect , with the subsequent placement of a liquefied dural sealant such as tisseel .
our patient 's dura was manipulated on multiple occasions over the span of half a decade . with each surgery to remove more metastatic lesions , the dura became more macerated .
this damaged dura created multiple avenues for csf to leak , which was essentially refractory to all of our attempts at a repair . a case report by yeager demonstrated the use of an evd with subsequent conversion to a shunt for a persistent cervical csf leak after a schwannoma resection.5 however ,
temporary external ventricular drainage for refractory lumbar spinal fluid leaks is not reported in the literature .
lumbar drains have been utilized in the case of persistent cervical csf leaks.6 we feel that a lumbar drain in our particular case would not have been best suited for the patient we presented .
we placed an evd in this situation as opposed to a lumbar drain because maximal csf diversion from the macerated dura would take place with a proximal avenue for controlled csf drainage , which would essentially decrease the pressure gradient of csf in an attempt to traverse the dural defects more distally .
although the placement of external ventricular drains comes with associated risks , if they are placed safely after a preprocedure computed tomography scan is done , their usage can be a viable option for temporary proximal csf diversion for refractory thoracolumbar csf leaks . restoration of structure and function using autologous free fat grafts has always been a challenge .
the advent and refinement of liposuction and lipoinjection techniques , abundant donor - tissue availability , and relative ease of harvesting have made autologous fat an attractive material for use as soft - tissue filler .
fat is harvested using either ( 1 ) syringe aspiration or ( 2 ) liposuction aspiration,(1 ) performed with a liposuction pump ( byron medical , tucson , ariz . ) . a standard coleman aspirator cannula ( byron medical ) with a curettage tip
( 500 mm mercury).(2 ) syringe aspiration is tedious if the amount of fat required is substantial .
when the liposuction aspiration using a liposuction pump is performed the whole suction system , and the container needs to be kept sterile .
( figure 1 ) we have devised a novel method of lipoaspiration to overcome these problems .
the above picture helps us to compare the three modalities of lipoaspiration and also helps us to note the advantage of our technique
materials required 20 - 50 cc disposable syringe , a piece of 2 2 cm polypropylene mesh , lipoaspiration suction pump ( figure 2a ) . ( a ) materials required for the procedure , ( b ) connected syringe mesh system , ( c ) close - up view of the mesh in liposuction aspiration , a suction pump is used . in our technique , a piece of polypropylene mesh is kept at one end of the lipoaspiration syringe , which is then connected to the suction pump .
( figure 2b & 2c ) as one syringe fills , it is replaced by another one until the required amount of fat is obtained .
these syringes are then centrifuged and the emulsified fat thus obtained is used for grafting ( video 1 ) . by using a polypropylene mesh in our technique , we are able to separate the transfusate from the harvested fat during harvesting itself
in our experience , the fat graft obtained by this method is found to be concentrated and has fewer contaminants .
this method also has the added advantage of having a short processing time , from harvesting to grafting .
fat grafting remains unpredictable , and various anecdotal harvesting and preparation techniques have been advocated .
our method is a more economical and an easier way to extract sterile emulsified fat .
as it is a simple method , it can be practiced even in day care centers .
hence , we recommend our technique as a reliable and routine method for extracting sterile emulsified fat in an economical way .
| study design case report .
objective temporary external ventricular drainage for refractory thoracolumbar cerebrospinal fluid ( csf ) leak is not reported in the literature .
we describe a recent case that utilized this technique .
methods retrospective review of the patient 's case notes was performed and the literature on this subject reviewed .
results the patient underwent multiple complex spinal surgeries for resection of innumerable metastatic ependymoma lesions . a case of significant refractory csf leak developed and as a last resort a right frontal external ventricular drain was placed .
the csf leak ceased , and the patient was eventually discharged home without further complication .
conclusion external ventricular drainage can be a viable option for temporary proximal csf diversion to treat refractory thoracolumbar csf leaks . |
turner syndrome ( ts ) is caused by partial or complete monosomy of the x chromosome .
only a few cases of ts with ischemic stroke have been reported [ 2 , 3 , 4 , 5 ] . in previously reported cases , various arteriopathies affecting the cerebral arteries such as fibromuscular dysplasia , hypoplasia , moyamoya syndrome , and
, we report a case of ts with multiple embolic infarcts caused by a cryptogenic embolism .
a 28-year - old woman with ts was referred to our hospital because of abnormal findings on brain magnetic resonance imaging ( mri ) .
she was diagnosed with ts [ karyotype : 45 , x/46 , x , + mar ] at the age of 16 years due to amenorrhea .
she denied alcohol , tobacco , or drug use and had no family history of neurologic disorders .
the patient was afebrile with a blood pressure of 180/130 mm hg and a heart rate of 89 beats / min .
she was obese with a body mass index of 27.3 kg / m ( 160 cm height , 70 kg weight ) .
she visited hospital due to sudden - onset severe headache with high blood pressure ( 240/140 mm hg ) .
she underwent brain computed tomography and mri at the referring hospital for differential diagnosis of thunderclap headache including hypertensive intracranial hemorrhage , subarachnoid hemorrhage , arterial dissection , or reversible cerebral vasoconstriction syndrome .
brain computed tomography was unremarkable , but diffusion - weighted imaging showed restricted diffusion in multiple lesions involving multiple vascular territories ( fig 1a ) .
cerebral magnetic resonance angiography ( mra ) ( fig 1b ) and carotid sonography demonstrated no extracranial or intracranial arterial stenosis .
laboratory tests revealed diabetes mellitus ( fasting plasma glucose 219 mg / dl , hemoglobin a1c 11.0% ) and hyperlipidemia ( low - density lipoprotein 189 mg / dl ) .
transthoracic echocardiography revealed normal valvular structure and function with a normal left ventricular ejection fraction .
patent foramen ovale , atrial septal aneurysm , or any aortic pathologies including aortic atheroma were not detected in transesophageal echocardiography .
the results of the hypercoagulability panel , vasculitis panel , and cerebrospinal fluid examination were all negative .
aspirin was administered , and medications for hypertension , hyperlipidemia , and diabetes were also started . within few hours after blood pressure control ,
the patient was discharged from the hospital without any symptoms of stroke , and recurrence was not observed in the 2 years after discharge .
cerebrovascular arteriopathies such as fibromuscular dysplasia , congenital hypoplasia , moyamoya syndrome , and premature atherosclerosis have been shown to be causes of ischemic stroke in previous case reports . in this case
furthermore , our patient showed multiple lesions in different vascular territories that were strongly suggestive of embolic stroke .
atrial fibrillation ( af ) could also be considered a potential source of embolic stroke in ts , as p - wave dispersion , a potential substrate for af , is increased in ts .
the classical risk factors for developing af include hypertension , diabetes mellitus , and valvular disease , and these are more common in ts than in the general population .
however , we could not completely exclude the possibility of paroxysmal af , as holter monitoring has a low yield for af detection ( 35% ) .
other possible cardio - aortic sources of embolism include intracardiac shunts , thrombi , calcifications / vegetations in the mitral valve , or aortic atheroma plaque .
we sought to identify possible cardio - aortic sources on transthoracic echocardiography , transesophageal echocardiography , and transcranial doppler shunt test ; however , all tests were negative .
some case reports have described deep venous thrombosis and portal vein thrombosis in ts [ 11 , 12 ] .
these factors include fibrinogen , d - dimer , factor viii , von willebrand factor , and proteins c and s .
we also considered the possibility of hypercoagulability as a cause of stroke in this case ; however , levels of d - dimer , fibrinogen , protein c and s were normal .
the embolic source of stroke in our patient remains unclear ; however , this is the first report of a ts patient with an embolic stroke pattern .
our case suggests another possible mechanism of stroke in ts that is different from previous case reports .
ischemic stroke in ts could be due to embolism as well as various cerebral arteriopathies .
further investigation is needed to more fully understand the various mechanisms of ischemic stroke in ts .
ischemic stroke in ts could be due to embolism as well as various cerebral arteriopathies , as documented in previous reports .
further studies are needed to determine the extent of the underlying mechanisms of ischemic stroke in ts .
this case report has been approved by the institutional review board of the inha university hospital .
stroke , with a high prevalence and long - term disabilities , is a major health problem in the
world1 , 2 , and it was reported that about two million people suffer from
stroke each year3 .
impairments including
loss of strength , sensation , and coordination abilities , which result in walking
difficulties , balance disorders , and limb function disturbance , occur in 7080% of stroke
patients4 .
this affects the life quality
of patients adversely and leads to a heavy economic burden on society .
subsequently ,
effective rehabilitation interventions to optimize recovery in stroke patients have received
high attention . in the treatment of balance and gait dysfunction ,
exercise therapy is regarded as one of
the commonly used methods that bring greater benefits in physical function for stroke
patients5 .
the effect of exercises on
balance and gait dysfunction in stroke patients has been confirmed by many clinical
studies3 , 6 ,
7 .
in addition , core stability exercises
have been widely carried out in many fields , including medical rehabilitation , sports
training , and medical care .
core stability exercises were shown to improve dynamic standing
balance , functional autonomy , static balance , flexibility , and stability8 . in recent years , core stability exercises have been reported to improve the rehabilitation
effect in stroke patients . in a study of 20 stroke patients ,
the control group underwent
standard exercise therapy , while the experiment group underwent both the core
stability - enhancing exercise and standard exercise therapy simultaneously .
after 4 weeks , yu
found that the mean trunk impairment scale score and muscle activity of the lower trunk
increased in the experiment group significantly9 . in study by cabanas - valdes , 80 patients were randomly assigned to
an experimental group and a control group , both groups underwent conventional therapy
including exercises , and the experimental group performed core stability exercises .
the
results showed that core stability exercises improve the efficacy of conventional
exercises10 .
although these studies
confirmed the effect of core stability exercises in the rehabilitation of stroke patients ,
few comparative studies have been carried out to compare the effect of core stability
exercises and conventional exercises . which is better in improving the stability and balance
of stroke patients , core stability or conventional exercises ?
therefore , a prospective study including 40 participants was carried out in the department
of neurology of yidu central hospital of weifang between january 2014 and february 2015 .
the
objective of the study was to determine which is better in the rehabilitation of stroke
patients , core stability exercises or conventional exercises .
participants with stroke - induced hemiplegia were recruited from the department of neurology
of yidu central hospital of weifang between january 2014 and february 2015 .
the diagnosis
and lesion side of these participants were confirmed by history evaluation , physical
examination , brain imaging , and examination of their medical records .
the inclusion
criteria9 were ability to walk more than
32 feet , duration of disorder > 6 months , no musculoskeletal problems , absence of any
cardiac disorders , complete understanding of this research , and ability to communicate .
the
included participants were randomly divided into either an experimental or control group by
a random computer - generated sequence .
the randomization was managed by a physician who was
not involved in the study .
all the included patients
signed an informed consent form , and the study was approved by the ethics committee of our
hospital .
the patients in the control group performed conventional exercises including limb
stretching , passive mobilization of joints , walking between parallel bars , and occupational
therapy11 .
the patients in the
experiment group performed core stability exercises including the plank , side plank , bridge ,
straight leg raise , and modified push - up12 . before the beginning of the study ,
the patients were given
individual instruction by the researchers . during the exercises , physicians provide
necessary assistance to help the patients in executing the exercises .
the exercises in both
groups were performed for one hour per day , six times a week for six weeks . the modified barthel index ( mbi)13 and
berg balance scale ( bbs)13 were used to
assess the clinical outcomes of the two groups .
the mbi , which demonstrates the ability of
patients to deal with daily activities , consists of ten items including personal hygiene ,
taking a bath alone , eating , using the bathroom , ascending stairs , dressing , defecating ,
controlling urine , walking or using a wheelchair , and moving to a chair or bed .
each item is
rated on a scale of 5 ranging from independent conduct to impossible to conduct .
the bbs ,
which can evaluate static and dynamic balance abilities of patients , consists of 14 items
covering functional tasks in three domains , sitting , standing , and changing posture , that
are scored on a scale of 04 .
ibm spss statistics 21.0 ( ibm corp . , armonk , ny , usa ) was used for statistical analysis ,
and p < 0.05 was considered significant .
the differences in the baseline data including
age , gender , and disease course were compared using the t test or test .
in the current study , 40 patients with stroke - induced hemiplegia were included and divided
randomly into experimental and control groups , with 20 participants in each group at the
beginning of the study .
the baseline clinical data including age , gender , disease course ,
bbs , and mbi were recorded .
there was no significant difference in baseline data between the
two groups ( p>0.05 ) .
the patients in the control group performed conventional exercises
for six weeks and those in the experimental group carried out core stability exercises for
six weeks .
three patients in the experimental group and two in the control group withdrew from the
study two weeks after treatment , and in total thirty - five patients completed the training .
the final results showed that , compared with the baseline , the mean mbi scores of the
experimental and control groups increased significantly from 53.5 and 55.1 to 66.8 and 61.2
( p<0.05 ) , respectively , and that the mean bbs scores increased significantly from 30.2
and 31.9 to 38.6 and 35.7 ( p<0.05 ) , respectively .
in addition , mbi scores were
significantly lower ( p<0.05 ) in the control group than in the experimental group .
the bbs
scores in the control group were relatively lower than those in the experimental group , but
there was no significant difference between the two groups ( p>0.05 ) .
a prospective study was carried out to compare the effect of core stability exercises and
conventional exercises on patients with stroke - induced hemiplegia . to the best of our
knowledge
the core , described as a box in the trunk with the
abdominals , paraspinals , and gluteals ; diaphragm and pelvic floor ; and hip girdle
musculature serving as the front wall , back wall , roof , and bottom , respectively , makes up
the largest part of the body and plays an important role in stabilizing the body and
controlling movement of daily activities9 .
core stability is regarded as a prerequisite for maintaining proper posture of the lumbar
and pelvic regions during sports activities9 . as a result ,
core stability exercises have many advantages in the
field of rehabilitation and can improve the activation and cooperative contractions of
abdominal and multifidus muscles , facilitating the function and movement of the limbs9 , 12 .
in the current study , the mbi and bbs scores after treatment were significantly increased
in both the experimental and control groups when compared with those before treatment .
this
indicates that both core stability exercises and conventional exercises can improve the
abilities of patients to deal with daily activities and control body balance , which confirms
the viewpoints of many published studies9 , 10 .
in addition , after treatment , the mbi scores were significantly lower in the control group
than those in the experimental group , and the bbs scores in the control group were also
lower than those in the experimental group .
the outcomes demonstrate that compared with
conventional exercises , core stability exercises can result in better effectiveness in
patients with stroke - induced hemiplegia . however , in terms of comparison of the bbs scores
at the end of treatment , although the mean bbs score in the experimental group was larger
than that in the control group , there was no significant difference between the two groups .
such a condition may be attributed to the relatively small sample size , and in a
larger - scale study , the difference between the two groups may be significant .
consequently ,
the outcomes in the present study can demonstrate that core stability exercises present with
better effectiveness than conventional exercises .
first , the sample size was small , and a large - scale
clinical study may be better in a comparative study .
second , the exercises results were
evaluated using self - reported measures of function instead of an objective measurement , and
this may have had some adverse influence on the final outcomes .
third , there was a lack of
long - term follow - up in the study , and it is unclear whether the effectiveness of core
stability exercises will decrease in patients with hemiplegia with time . | only a few cases of turner syndrome ( ts ) with ischemic stroke have been reported .
various arteriopathies of the cerebral arteries , including fibromuscular dysplasia , congenital hypoplasia , moyamoya syndrome , and premature atherosclerosis have been assumed to be the cause of ischemic stroke in ts .
there has been no case report of a ts patient presenting with an embolic stroke pattern without any cerebral arteriopathy . a 28-year - old woman with ts
was referred to our hospital because of abnormal brain magnetic resonance imaging ( mri ) findings .
she underwent brain mri at the referring hospital because she experienced sudden - onset diffuse headache .
diffusion - weighted imaging revealed multiple acute embolic infarcts in different vascular territories .
intracranial and extracranial arterial disease was not detected on cerebral magnetic resonance angiography and carotid sonography .
embolic source workups , including transthoracic and transesophageal echocardiography , holter monitoring , and transcranial doppler shunt study , were all negative .
hypercoagulability and vasculitis panels were also negative .
our patient was diagnosed with cryptogenic embolic stroke .
this is the first report of a ts patient with an embolic stroke pattern .
our case shows that ischemic stroke in ts could be due to embolism as well as the various cerebral arteriopathies documented in previous reports . |
a 3-year - old male , neutered , domestic shorthair cat with a history of chronic regurgitation since being obtained as a kitten was presented for weight loss and regurgitation of all ingested food .
the cat was in poor body condition and had a firm swelling in the ventral neck at the time of presentation .
thoracic radiographs showed severe dilation of the entire cervical and cranial intrathoracic esophagus to the level of the heart base .
computed tomographic angiography ( cta ) showed a persistent right aortic arch with an aberrant left subclavian artery and severe dilation of the cervical and intrathoracic esophagus cranial to the heart base .
cta also showed a bicarotid trunk and kommerell s diverticulum to be present , which are rare vascular structures in the cat .
removal of the obstructing trichobezoars resulted in resolution of clinical signs , and the cat was able to drink water and eat a canned food slurry without regurgitation .
vascular ring anomaly ( vra ) should be considered in all cats with a history of regurgitation , regardless of their age at the time of presentation .
cta is a valuable diagnostic imaging procedure that allows differentiation of a vra from other causes of esophageal obstruction and provides information about the vra that can be used to determine amenability to surgical correction .
a 3 year - old male , neutered , domestic shorthair cat was presented with a 2 week history of severe regurgitation of all ingested food .
the cat had a history of a voracious appetite and regurgitation of solid food since being acquired as a kitten , but was able to consume liquid meals without regurgitating until 2 weeks prior to presentation .
the physical examination revealed a poor body condition score ( bcs 2/9 ) and a 4 cm firm tubular structure in the ventral neck , believed to be within or associated with the esophagus .
as a result of the cat s fractious nature , it was necessary to induce general anesthesia for further evaluation .
thoracic radiographs showed severe distention of the entire cervical and intrathoracic esophagus to the level of the heart base with heterogeneous soft tissue opaque material surrounded by a thin gas rim ( figure 1 ) .
differentials for the radiographic findings were constriction due to a vascular ring anomaly ( vra ) , most commonly a persistent right aortic arch , a mural defect or benign esophageal stricture .
the cervical and thoracic esophagus ( dashed lines ) is severely distended cranial to the carina , displacing the trachea ( dotted lines ) ventrally and to the right .
note the marked leftward deviation of the trachea at the level of the heart base computed tomographic angiogram .
structures of note include the persistent right aortic arch ( praa ) , bicarotid trunk ( bct ) , right subclavian artery ( rsa ) , aberrant left subclavian artery ( alsa ) , descending aorta ( dao ) , esophagus ( es ) , cranial vena cava ( crvc ) , pulmonary trunk ( pt ) and right auricle ( rau ) computed tomographic angiography ( cta ) was performed in order to confirm and classify the suspected vra and to determine if surgical correction would be possible .
the esophagus cranial to the heart base was almost entirely filled with heterogeneous soft tissue , gas and mineral opaque material . at the level of the heart base ,
the esophagus was focally and concentrically narrowed by the trachea and aorta on the right and the main pulmonary artery on the left .
the site of constriction was apparent at the anatomic level of the ligamentum arteriosum ; however , the ligamentum arteriosum could not be specifically identified on cta .
the aortic arch and proximal descending aorta were located just to the right of midline , with the distal descending aorta positioned immediately ventral to the thoracic spine .
a bicarotid trunk and right subclavian artery were present , and branched from the aortic arch in close succession .
additionally , the origin of the aberrant left subclavian artery from the aorta was markedly dilated and then abruptly narrowed distally to a normal diameter ; this finding is known as kommerell s diverticulum .
although the aberrant left subclavian artery created a dorsal indentation in the intrathoracic esophagus , it did not appear to be the primary cause of the constriction .
the esophagus remained dilated cranial to the level of the constriction and pulsation of the major vessels against the wall of the esophagus was observed at the level of the luminal narrowing ( see video in the supplementary material ) .
evidence of mild - to - moderate esophagitis , presumably due to stagnation of trichobezoars and putrefaction of food , was observed cranial to the constriction .
it was possible to advance the endoscope past the constriction and no gross abnormalities were noted in the stomach .
following recovery from anesthesia , the cat was able to drink water and eat a canned food slurry without complication or regurgitation .
surgical ligation and transection of the vascular ring anomaly with concurrent endoscopic balloon dilatation of the esophageal stricture was recommended as the treatment of choice .
the cat was discharged with instructions to continue feeding a canned food slurry in an upright position .
the owner was contacted prior to submission and reported that the cat was doing well with multiple small feedings of canned food and had not regurgitated since discharge .
although vras are common in dogs , with several reports of diagnosis in adult dogs , they are uncommon in cats and infrequently diagnosed in adult cats .
the most common feline vra is persistent right aortic arch ( praa ) with a left ligamentum arteriosum ; however , other described abnormalities include praa with coarctation of the aorta at the level of the left ligamentum arteriosum , double aortic arch , left aortic arch with right ligamentum arteriosum and praa with concurrent left cranial vena cava .
recently , cta has become more widely available as a diagnostic modality and has increased the frequency and accuracy of diagnosis .
cta proved valuable in this adult cat as it could confirm a congenital defect was present as the cause of the esophageal constriction and allow a surgical procedure to be planned . the vascular abnormality known as kommerell s diverticulum
this vascular structure is classically defined as a dilation of the aorta at the insertion of either the left or right subclavian artery that is 1.5 times greater than the diameter of the distal subclavian artery .
the presence of a bicarotid trunk branching directly from the aortic arch in this cat is also an unusual finding .
the treatment of choice for vras in companion animals is surgical ligation and transection of the anomalous vessel(s ) . if possible
, periesophageal fibrosis should be reduced , and the stricture site should be dilated intraluminally with a balloon dilatation catheter .
this procedure has traditionally been performed via a thoracotomy , but thoracoscopy has also been used , and offers a less invasive treatment option .
ligation and transection of the aberrant left subclavian artery can also be performed if the vessel contributes to the esophageal constriction . in an effort to decrease the severity of esophageal dilation and dysfunction ,
although surgical correction is the treatment of choice , clinical signs may persist even after a successful procedure .
esophageal dilation may cause irreversible myenteric nerve degeneration and esophageal hypomotility ; however , a 90% or greater recovery rate has been reported in dogs .
nutritional management is critical in the postoperative period but is rarely successful as a primary treatment for vra .
many of these patients require lifetime management with elevated feedings of canned or pured food in small meals , but it is possible to transition those with less severe obstructions to a more solid diet . in cases of severe esophageal dysfunction or malnutrition , a gastrostomy tube may be necessary to achieve adequate nutritional supplementation .
congenital causes of esophageal obstruction should be considered in cats presented with a history of regurgitation , regardless of age at the time of presentation .
cta confirmed the presence of an extraluminal constriction and provided valuable information about the vascular anomaly in the event that surgical correction would have been pursued .
surgical removal of the extraluminal constriction with balloon dilatation of the esophageal stricture was recommended and is considered the treatment of choice .
however , considering the guarded prognosis for return of normal esophageal function in an adult cat with chronic esophageal dilation , medical management provided a viable alternative for this cat . we wish to report some corrections to our study , none of which alters the interpretation of the data or the conclusions drawn . after publication
, we noticed that one of the micro - array hybridizations ( on sample nb11 ) was performed on the same patient 's material as another hybridization ( sample nb4 ; see table 1 ; a corrected version of table 5 ) .
as this error leads to an incorrect subclassification of the patients into the ' favourable ' and ' unfavourable ' neuroblastoma subgroups , we would like to exclude this data point from the differential expression analysis of favorable versus unfavorable neuroblastoma given under the heading ' differential expression analysis of favorable and unfavorable neuroblastoma ' in the results section of .
careful reanalysis after exclusion of nb11 did not lead to important changes in the generated gene lists and conclusions ; the changes are given in the corrected paragraph and table 2 ( a corrected version of table 4 ) , and the additional data files 1 and 2 ( corrected versions of additional data files 2 and 3 ) available online with this article . we also noticed that sample nb1 is stage 1 instead of stage 4s and that sample nb2 was not localized to the adrenals ( see table 1 ) .
so far , most published microarray studies on neuroblastomas mainly compared favorable with unfavorable neuroblastomas in order to identify prognostic markers or pathways that are involved in these clearly different neuroblastoma tumor types . in order to add value to such an analysis
, we contrasted similar differentially expressed gene lists with the normal neuroblast expression profile ( additional data file 1 ) . in a first step , we compared the differentially expressed genes between these two tumor types with published prognostic gene lists .
we found that 23 of the 193 genes on our list were previously reported , including the well established markers mycn , ntrk1 , and cd44 ( see nbgs analysis in additional data file 2 ) .
subsequently , we looked for the corresponding gene expression levels of the differentially expressed genes in the normal counterpart cells , aiming to select neuroblastoma candidate genes .
of the 100 genes that are more highly expressed in favorable tumors ( compared to unfavorable ) 41 also have a significant differential expression ( either higher or lower ) compared to neuroblasts , whereas 43 of the 93 genes that are more highly expressed in unfavorable tumors exhibit differential expression compared to the neuroblasts ( table 2 ) . from this analysis ,
a few putative positional tumor suppressor candidates emerge : cdc42 on 1p36 , cacna2d3 on 3p21 and dlk1 on 14q .
the latter two genes are of particular interest because they are highly expressed in neuroblasts and favorable neuroblastomas and their expression is significantly lower in unfavorable neuroblastomas . among the genes that are
more highly expressed in unfavorable neurob - lastomas than in favorable ones and neuroblasts , the proven oncogenic transcription factor mycn emerges ( and putative downstream genes kifap3 , ophn1 , rgs7 , ascl1 , odc1 , twist1 and tyms , according to nbgs ) , as well as several other genes that have been identified or studied in the context of neuroblastoma such as alk and prame , and positional candidates on 17q including birc5 and rnu2 .
so far , most published microarray studies on neuroblastomas mainly compared favorable with unfavorable neuroblastomas in order to identify prognostic markers or pathways that are involved in these clearly different neuroblastoma tumor types . in order to add value to such an analysis
, we contrasted similar differentially expressed gene lists with the normal neuroblast expression profile ( additional data file 1 ) . in a first step , we compared the differentially expressed genes between these two tumor types with published prognostic gene lists .
we found that 23 of the 193 genes on our list were previously reported , including the well established markers mycn , ntrk1 , and cd44 ( see nbgs analysis in additional data file 2 ) .
subsequently , we looked for the corresponding gene expression levels of the differentially expressed genes in the normal counterpart cells , aiming to select neuroblastoma candidate genes .
of the 100 genes that are more highly expressed in favorable tumors ( compared to unfavorable ) 41 also have a significant differential expression ( either higher or lower ) compared to neuroblasts , whereas 43 of the 93 genes that are more highly expressed in unfavorable tumors exhibit differential expression compared to the neuroblasts ( table 2 ) . from this analysis ,
a few putative positional tumor suppressor candidates emerge : cdc42 on 1p36 , cacna2d3 on 3p21 and dlk1 on 14q .
the latter two genes are of particular interest because they are highly expressed in neuroblasts and favorable neuroblastomas and their expression is significantly lower in unfavorable neuroblastomas . among the genes that are
more highly expressed in unfavorable neurob - lastomas than in favorable ones and neuroblasts , the proven oncogenic transcription factor mycn emerges ( and putative downstream genes kifap3 , ophn1 , rgs7 , ascl1 , odc1 , twist1 and tyms , according to nbgs ) , as well as several other genes that have been identified or studied in the context of neuroblastoma such as alk and prame , and positional candidates on 17q including birc5 and rnu2 .
additional data files 1 and 2 containing the corrected data available online with this article .
corrected version of additional data file 2 . corrected version of additional data file 3 .
clinical and genetic data of carefully selected neuroblastoma samples that were included in this study samples were subdivided into favorable or unfavorable type based on mycn amplification , ploidy and age at diagnosis .
nd , not determined or unknown . genes that are differentially expressed in favorable vs unfavorable neuroblastoma genes that are differentially expressed compared with neuroblasts among the differentially expressed genes in favorable neuroblastoma ( nb ) vs unfavorable nb , with an indication of the number of neuroblastoma microarray studies in which these genes were found through nbgs analysis . | case summarya 3-year - old male , neutered , domestic shorthair cat with a history of chronic regurgitation since being obtained as a kitten was presented for weight loss and regurgitation of all ingested food .
the cat was in poor body condition and had a firm swelling in the ventral neck at the time of presentation .
thoracic radiographs showed severe dilation of the entire cervical and cranial intrathoracic esophagus to the level of the heart base .
computed tomographic angiography ( cta ) showed a persistent right aortic arch with an aberrant left subclavian artery and severe dilation of the cervical and intrathoracic esophagus cranial to the heart base .
cta also showed a bicarotid trunk and kommerell s diverticulum to be present , which are rare vascular structures in the cat .
esophagoscopy showed esophageal dilation and multiple compact trichobezoars obstructing the esophagus .
removal of the obstructing trichobezoars resulted in resolution of clinical signs , and the cat was able to drink water and eat a canned food slurry without regurgitation .
surgical correction was not pursued.relevance and novel informationvascular ring anomaly ( vra ) should be considered in all cats with a history of regurgitation , regardless of their age at the time of presentation .
cta is a valuable diagnostic imaging procedure that allows differentiation of a vra from other causes of esophageal obstruction and provides information about the vra that can be used to determine amenability to surgical correction . |
the ultrasound ( us ) appearance of testicular microlithiasis ( tm ) was first described by doherty et al . in 1987 .
the prevalence of tm in symptomatic korean men was found to be 6.0% with significant co - occurrence of tm , testicular cancer , and infertility by yee et al .
although the cause - and - effect relationships are unclear , tm has been seen in patients with cryptorchidism , varicoceles , infertility , testicular torsion , klinefelter syndrome , pulmonary alveolar microlithiasis , neurofibromatosis , acquired immunodeficiency syndrome , intratubular germ cell neoplasia , and most importantly , primary testicular neoplasms .
reported that 74% of testes with tumors had associated ipsilateral tm on radiological inspection ; whereas , only 8% of testicular specimens with benign conditions had microcalcifications .
although chen et al . reported that there was a significant difference in the rate of malignancy in males with tm compared with those without tm , the question remains whether tm independently increases the risk of testicular malignancy in taiwanese men .
, after reporting an association between tm and testicular tumor specimens , suggested in 1982 that radiographic studies be incorporated into diagnosing tm because of the perceived risk for testicular cancer in testicles with microlithiasis
performed us follow - up in 9 of 22 patients with tm for a mean of 32 months without any newly developing tumors being diagnosed .
reported similar results ; therefore , a regular scrotal us is controversial in asymptomatic tm patients .
the current recommendations , including those of the european association of urology , are that the presence of microlithiasis alone is not an indication for a regular scrotal us in the absence of other risk factors ( size<12 ml or atrophy , inhomogeneous parenchyma ) .
however , there have been no reports describing the changes in calcification over time in pediatric patients with tm .
the study protocol was approved by the institutional review board of the asan medical center .
the medical records from july 1997 to august 2014 of the asan medical center , a tertiary referral center , were retrospectively screened for patients diagnosed with tm by scrotal us .
twenty - three tm patients were included who had undergone scrotal us at least twice .
we analyzed the patient characteristics , clinical manifestations , specific pathological features , and clinical outcomes .
we measured the calcified area in maximum cross - sectional area , and we compared the calcific density in the initial diagnostic us and the final follow - up us .
we defined the testis and the calcified area in terms of their maximal cross - sectional area and calculated those areas using image j software ( national institutes of health , bethesda , md , usa ) ( fig .
patients that showed microlithiasis in three or more sections were defined as diffuse type , and patients with microlithiasis in less than three sections were defined as focal type ( fig .
we classified the patients into three groups according to the change of microlithiasis : an increased group , 20% increase in microlithiasis ; a decreased group , 20% decrease in microlithiasis ; and a no change group , < 20% increase or decrease .
differences were analyzed by a paired t - test , and crosstabs were used to assess dependent samples . in this study p - values < 0.05 were considered statistically significant .
the mean age at presentation was 11.34.6 years , and the follow - up period ( interval of fist us and last us ) was 79.138.8 months ( range , 25.4 - 152.9 months ) .
the mean age at last follow - up was 17.85.8 years ( range , 6.4 - 26.9 months ) .
follow - up occurred for 19 of 23 patients over puberty ( defined as > 13 years old ) .
of the 23 patients 20 had bilateral tm , 2 patients had unilateral tm , and 1 patient had an atrophic testis .
less frequent comorbid conditions included testicular torsion ( 3 patients , 13.0% ) , epididymitis ( 3 patients , 13.0% ) , varicocele ( 2 patients , 8.7% ) , hydrocele ( 2 patients , 8.7% ) , and epididymal cyst ( 2 patients , 8.7% ) .
calcific density was increased at the last follow - up us compared with the initial us ; however , this finding was not statistically different ( 3.74%6.0% vs. 3.06%4.38% , respectively , p=0.147 ) .
we divided the subjects into two groups ( focal vs. diffuse ) based on the distribution of tm .
we classified 23 testes as having focal tm and 20 testes as having diffuse tm .
in focal tm calcific density decreased but not significantly ( 0.72%0.55% vs. 0.66%1.03% , p=0.813 ) . on the other hand ,
the calcific density of diffuse tm show a trend toward increase ( 5.8%5.2% vs. 7.3%7.4% , p=0.457 ) ( table 2 ) ( fig .
3 ) . to clarify the tendency of tm calcification toward increase vs. decrease we divided the subjects into three groups based on the calcific density change of tm ( increase : increased > 20% ; decrease : decreased > 20% ; no change : < 20% increase or decrease ) .
we categorized 14 testes as increased , 18 testes as decreased , and 11 testes as no change .
half of the patients with diffuse tm were assigned to the increased group , a proportion significantly higher than focal tm ( 10/20 [ 50% ] in diffuse tm , 4/23 [ 17.4% ] in focal tm , p=0.049 ) ( table 3 ) .
in addition , 5 of 8 testes with cryptorchidism ( including 2 with bilateral cryptorchidism ) were categorized into the increased group .
previous tm studies have focused only on the relationship between tm and testicular cancer , and not on the natural course of this disease progression . in our current study , we report the natural course of calcific density changes in pediatric tm during pubertal development .
the mean age at presentation was 11.3 years and the mean follow - up period of approximately 5 years allowed follow - up of 23 patients over pubertal development . despite a follow - up interval that allowed potential pubertal changes to the testis , none of our patients developed testicular cancer or new abnormal symptoms . by contrast , there are some reported cases of patients with a known tm on us exam that eventually developed a primary testicular cancer .
reported a yolk sac tumor developing in a 17-year - old boy being followed for bilateral tm that was originally detected because of an initial sonographic evaluation of unequal sized testes .
reported the case of a man with tm seen on a sonograph performed due to bilateral testicular pain who presented three years later with a metastatic germ cell tumor of the left testicle .
although , an association between tm and subsequent testicular tumors appears likely , whether there is a true cause - and - effect relationship remains unknown .
( 7 patients , 45 months)-used us to follow patients with tm and , similarly to our present results , did not detect the appearance of new testicular tumors .
compared with these prior studies , our current study incorporates more patients and a longer - term follow - up extending through puberty .
an additional strength of our study over previous studies is in our investigation of changes in calcified density of the tm .
one earlier study has reported nonquantitative changes in the prominence of tm on yearly us follow - up .
the tm was less prominent in one patient , unchanged in four , and two patients were lost to follow - up . to our knowledge , our present study is the first report to provide a quantitative analysis of calcific density in tm .
calcific density on us showed a nonstatistically significant trend toward increase over time in our study subjects .
reported a relationship between the number of microliths and testicular cancer after subgrouping subjects into four ranges based on the number of microliths . in our present study , we divided tm into two nearly equal groups based on the distribution pattern of calcification ( focal type [ 23 testes ] vs. diffuse type [ 20 testes ] ) that appear to differ in terms of calcification trends .
calcific density shows a trend toward a decrease in focal tm but toward an increase in 50% of the testes with diffuse tm .
notably , the majority of testes ( 5/8 ) in our study series with cryptorchidism , including those with bilateral cryptorchidism , were categorized as being in the increased calcification group .
during the follow - up through puberty of the microlithiasis in the 23 boys in our present study , we observed no significant changes in the density of their microliths and no development of testicular cancer ; however , we found that diffuse tm and cryptorchidism tend to increase calcific density .
hence , close observation is recommended in cases of tm combined with cryptorchidism and diffuse microlithiasis .
a 35-year - old male farm - worker presented to the emergency room with alleged history of horse bite to the right forearm about 2 hours prior to presentation while feeding the horse .
there was deformity of the forearm with multiple puncture wounds , deep abrasions and small lacerations on the distal - third of the forearm .
copious irrigation with normal saline was done and he was administered anti - tetanus and postexposure rabies prophylaxis .
he underwent emergency wound debridement , and the ulna was stabilised with an intra - medullary square nail .
he had an uneventful recovery and at three - month follow - up , the fractures had healed radiographically in anatomic alignment . at two - year follow - up , he is doing well , is pain free and has a normal range of motion compared to the contralateral side .
horse bites behave as compound fractures however rabies prophylaxis will be needed and careful observation is needed .
early radical debridement , preliminary skeletal stabilisation , re - debridement and conversion osteosynthesis to plate , and antibiotic prophylaxis were the key to the successful management of our patient .
falls and kicks are common mechanism of injuries in people handling horses [ 1 - 3 ] .
we present a case of forearm open fracture due to horse bite and its management .
a 35-year - old male farm - worker presented to the emergency room with alleged history of horse bite to the right forearm about 2 hours prior to presenting to us .
he had recently bought the horse for renting it for marriages and other social and religious functions . on examination , there was deformity of the forearm with multiple puncture wounds , deep abrasions and small lacerations on the distal - third of the forearm .
1(a ) and ( b ) ) front and dorsal view of the forearm on presentation showing the bite wounds .
he was given 0.5 ml tetanus toxoid and 500 iu tetglob ( human tetanus immunoglobulin bp ) , and received post - exposure rabies prophylaxis with vaxirab ( purified duck embryo vaccine for rabies ip ) .
radiographs revealed fracture of radius and ulna in the mid - shaft region ( fig .
he was taken to the operating room ( or ) where careful debridement of the wounds was done under loupe - magnification .
the ulna was stabilised with an intra - medullary talwalkar square nail ( inor , mumbai , india ) .
seventy - two hours later , he was taken to the or for re - debridement .
the nail in ulna was removed and conversion osteosynthesis using dynamic compression plate and screws for radius and ulna was done ( fig .
he was changed to oral cephalexin and ciprofloxacin on day 6 which were given till suture removal .
he had an uneventful recovery and was discharged from the hospital on the 10th day from injury .
the patient , however , sold the horse as he was too scared to keep it . at three - months
three - month follow - up radiographs showing fracture healing in anatomic alignment . at two - year follow - up
, he is doing well , is pain free and has a normal range of motion compared to the contralateral side .
he is able to use his hand for all routine work and has returned to his work in the farm .
in horse - related accidents , the majority of injuries occur when the person falls from a horse [ 1 - 3 ] .
blow by a horse , getting struck by an object while riding a horse , being kicked by a horse , the horse falling on the patient , being entangled by reins , and being bitten by a horse are other less common horse - related accidents .
most people bitten by horses do not seek medical advice as most bite injuries are minor and self - treated or do not require admission to hospital .
thomas et al in their study estimated that 102,900 people are treated yearly in emergency rooms in the us due to nonfatal horse related injuries , and about 1800 patients are treated each year after horse bites .
our patient was too frightened to keep the horse and risk another bite , and hence , sold it not caring for the implications of financial loss .
domestic animals at home differ between societies , and therefore , local traditions affect the epidemiology of animal - related injuries . comparing animal bite wounds , cat bites result in punctured deep wounds , dog bites cause rather superficial abrasion and laceration type wounds ; because of the great deal of force exerted by an equine in closing its jaws , in horse and donkey bites , the severity of injuries may range from mild superficial pressure trauma , cutaneous breaks of the skin , deep lacerations with loss of tissue , to amputations of digits and even the nose .
peel et al reported a case of fracture of the forearm bones following horse bite that was treated with open reduction and internal fixation primarily .
he sustained repeated infections with purulent wound discharge from which mixed cultures of bacteria , including staphylococcus aureus , prevotella melaninogenica , escherichia coli , and pasteurella multocida were isolated .
more than 3 months after the initial attack by the horse actinobacillus suis was isolated by bone biopsy specimen . though the acute trauma is readily apparent from a biting event ,
there is also the risk of exposure to various microbes in the oral secretions of equines .
transmission of such agents with zoonotic potential can also occur from the non - bite exposures to the oral and respiratory secretions .
because of the large number of bacteria in the mouth , animal bite wounds are generally contaminated and their treatment is difficult , particularly in extensive lesions .
the occurrence of bacterial infection after animal bites depends on several factors , such as species of animal aggressors ( humans would be associated with a higher infection risk ) , type and site of the injuries ( wounds located in hands have a higher infection risk ) , care given to the wound , inherent factors in the individual ( greater risk in elderly , those with diabetes mellitus , vascular disease , etc ) . in relation to wound type , puncture wounds
have been reported to have a higher infection rate after animal bites , possibly due to the deposition of bacteria deep in the skin .
there is evidence that the use of antibiotic prophylactic after bites of the hand reduces infection .
horse bites most commonly lead to infections with burkholderia , streptococcus , staphylococcus , rhodococcus , actinobacillus , yersinia , and pasteurella , escherichia , neisseria , prevotella , pseudomonas , listeria , hendra virus , vesicular stomatitis virus species .
bites , kicks or strikes , animal contact and cutting or scratching were the most frequent mechanisms of injury reported .
nearly 20% of reported horse - related injuries , 14% of cattle and cats and 11% of dog - related injuries resulted in admission .
brouwer et al reported a case of streptococcus equi meningitis complicated by brain abscesses , as a result of a horse bite .
kse et al performed a retrospective evaluation of 24 patients presenting with animal bites ( 19 horse and 5 donkey bites ) .
the head and neck were the most frequent bite sites ( 14 cases ) , followed by the extremities ( eight cases ) and the trunk ( two cases ) .
our primary aim was damage - control to aggressively debride and at the same time give stability to the limb for wound dressings .
preliminary stabilisation of the forearm by an intramedullary talwalkar square nail ( inor , mumbai , india ) in the ulna was done following debridement of the wounds and excision of devitalised tissues . fixing the ulna with a nail also helped in maintaining forearm length .
re - debridement at 72 hours , showed no features of infection and decision of internal fixation with plate and screws was taken .
we also wish to highlight to the journal reader community that putting in implants when there is any doubt of contamination is risking long term bone infection and other associated complications of delay - in - union and non - union with compromised hand function .
intra - medullary nail was preferred over external fixator in this case , though in fractures with severe soft tissue damage the use of external fixator is indicated .
the problems of using external fixation in the forearm include : pin - track - infection , nerve damage due to insufficient anatomical exposure and a relatively high rate of non - union .
conversion osteosynthesis to internal fixation after primary external fixation is associated with high rates of osteomyelitis .
plating is established as the standard method in the operative treatment of forearm fractures in adults .
we successfully converted the fixation from intra - medullary nail to plating with no incidence of infection or delay - in - union .
therefore , it makes sense to debride wounds which have necrotic tissue early rather than later .
considering the most common complication of zoonotic infection related to animal bite injuries , our successful surgical treatment without complication appears to have definite clinical relevance .
the systematic treatment protocol of early radical debridement , preliminary skeletal stabilisation , re - debridement and conversion osteosynthesis to plate , and antibiotic prophylaxis were the key to the successful management of our patient .
horse or other animal bites are uncommon injuries . the management of these cases should be staged with the principles of damage control and infection control taking precedence . through this publication
we wish to highlight these rare injuries in the orthopaedic community and the journal readers . | purposetesticular microlithiasis ( tm ) is a relatively rare clinical entity of controversial significance characterized by the existence of hydroxyapatite microliths located in the seminiferous tubules .
the aim of this study was to observe the natural course of changes in the calcific density of pediatric tm.materials and methodswe included a total of 23 tm patients undergoing scrotal ultrasound ( us ) on at least two occasions from july 1997 to august 2014 .
we retrospectively analyzed the patient characteristics , clinical manifestations , specific pathological features , and clinical outcomes .
we measured the calcified area and compared the calcific density between the initial and final uss.resultsthe mean age at diagnosis was 11.34.6 years , and the follow - up period was 79.138.8 months ( range , 25.4 - 152.9 months ) . during the follow - up period ,
no patients developed testicular cancer .
calcific density on us was increased in the last versus the initial us , but not to a statistically significant degree ( 3.74%6.0% vs. 3.06%4.38% , respectively , p=0.147 ) .
when we defined groups with increased and decreased calcification , we found that diffuse tm was categorized into the increased group to a greater degree than focal tm ( 10/20 vs. 4/23 , respectively , p=0.049 ) .
in addition , five of eight cases of cryptorchidism ( including two cases of bilateral cryptorchidism ) were categorized in the increased calcification group.conclusionsdiffuse tm and cryptorchidism tend to increase calcific density .
close observation is therefore recommended for cases of tm combined with cryptorchidism and cases of diffuse tm . |
malignant salivary gland neoplasms account for < 0.5% of all malignancies and approximately 35% of all head and neck cancers .
progress in understanding the cell biology of salivary gland carcinomas ( sgcs ) and detecting vulnerable molecular pathways may lead to the development of new targeted therapy options in these rare cancers with poor prognosis .
anti - egfr agents include ( i ) monoclonal antibodies ( cetuximab or erbitux , panitumumab ) which block the binding of natural egfr ligands like egf or tgf- resulting in inhibition of downstream signal - transduction pathways and ( ii ) small molecule tyrosine kinase inhibitors ( tkis ) which act by binding the atp pocket within the kinase domain of the egfr and impairing its catalytic activity ( gefitinib , erlotinib , lapatinib ) . downstream signaling pathways triggered by egfr include the ras - raf - extracellular signal - regulated kinase / mitogen activated protein kinase ( mek / mapk ) pathway , which is mainly correlated to cell proliferation , and the p13k - pten - akt axis .
recently , we were able to demonstrate that frequent egfr overexpression and the absence of drug - resistance egfr mutations in sgc plead in favor of further therapeutic trials with egfr - targeting monoclonal antibodies .
one of the signaling effectors downstream of egfr , kras , was shown by us to be rarely mutated in sgc [ 2 , 3 ] .
wildtype kras is one of the clinically proven prerequisites for a successful anti - egfr therapy and therefore anti - egfr monoclonal antibodies are approved only for metastatic colorectal cancer patients whose tumors display wildtype kras . in the absence of kras mutations , resistance to anti - egfr treatments could be caused by alterations of other members of the ras - raf - mapk pathway .
braf ( v - raf murine sarcoma viral oncogene homolog b1 ) , a serine / threonine kinase , is the downstream effector of kras in the ras - raf - mapk signaling pathway . a somatic mutation ( v600e ) in exon 15 of braf has been identified in multiple human cancers with a mutation rate of 66% in malignant melanomas and at lower frequency in other human carcinomas .
recently , it was demonstrated that wildtype braf is required for the response of patients with metastatic colorectal cancer to cetuximab and panitumumab .
the aim of this study was to determine the braf v600e mutation frequency in a large cohort of sgcs of the main histopathological types and to design an allele - specific pcr as an effective screening method .
surgically removed , formalin - fixed tumor samples were obtained from 65 patients ( 35 males and 30 females with a median age at diagnosis of 55 years ) treated with the histopathological diagnosis of an sgc according to the who classification .
egfr - targeted therapy was not applied . the study cohort consisted of adenoid cystic carcinoma ( n = 25 ) mucoepidermoid carcinoma ( n = 10 ) , myoepithelial carcinoma ( n = 8) , acinic cell carcinoma ( n = 12 ) and adenocarcinoma ex pleomorphic adenoma ( n = 10 ) . genomic dna was extracted and pooled from oral mucosa samples of five healthy individuals .
this pooled dna was used as a normal dna control for the development of the pcr assay .
heterozygous mutant control dna was extracted from cells of the colorectal cancer cell line ht 29 which contains the heterozygous braf v600e mutation .
the basis for discrimination using allele - specific pcr is that a pcr primer mismatched at its 3 end with the dna template will react less efficiently than one that is entirely complementary .
our allele - specific multiplex pcr was designed with one common forward ( bf ) and two separate reverse primers ( br and bmu ) : bf : 5-ctcttcataatgcttgctctgatagg-3 , br : 5-agttgagaccttcaatgactttctagt-3 , bmu : 5-cccactccatcgagatttct-3. the forward primer bf and the reverse primer br amplify a 273 bp fragment of both mutant and wildtype alleles and thus serve as amplification control .
the second reverse primer ( bmu ) is specific for the mutated allele at the 3 end .
this primer together with bf generates an 143 bp product only in the presence of the v600e ( gtg > gag ) mutation ( figure 1(a ) ) . a series of annealing temperatures ( 52c60c ) , primer concentrations ( 0.10.4 mol / l ) , and mg concentrations ( 1.53.5
reactions consisted of : 80100 ng genomic dna ; 200 mol / l dntp ; 0.1 mol / l of primers ; 1.5 mmol / l mgcl2 ; 0.5 u taq polymerase ( roche diagnostics , penzberg , germany ) .
final cycling conditions were as follows : 5 minutes of denaturing at 94c and 30 cycles of 94c for 30 seconds , annealing 55c for 45 seconds and 72c for 60 seconds .
a volume of 10 l of the pcr products was electrophoresed on a standard 2.5% agarose gel stained with sybr - green i for visualization under uv light .
the allele - specific pcr for the detection of the braf v600e mutation demonstrated high specificity ( i.e. , detection of only the normal or only the mutant allele ) , high sensitivity ( i.e. , no spurious pcr fragments ) , and acceptable yield . all 65 sgc in this cohort ( 100% ) presented the braf wildtype ( 95% exact confidence limit 00.07 ) .
the 273 bp pcr fragment was always amplified confirming the integrity of the isolated dna from clinical tissue samples ( figure 1(a ) ) . to test the sensitivity of the mutation - specific pcr
, we made a serial dilution of ht 29 dna ( which contains the heterozygous braf v600e mutation ) , in control dna with wildtype braf .
no braf v600e mutation was detected in additionally screened dna samples from microdissected normal tissue adjacent to the tumor cells ( 5 cases ) .
the activation of the egfr - ras - raf signaling cascade is an important pathway in cancer development and is considered a key pathway for therapeutic molecules .
egfr transmits signals to the nuclei instructing cancer cells to proliferate and metastasize , and kras and braf are those downstream signaling molecules .
anti - egfr therapies interrupt the cancer - triggering signaling cascade , however , if the kras or the braf gene is mutated , their proteins are locked into an active conformation , regardless of whether the egfr is therapeutically blocked .
cetuximab ( erbitux ) has already been tested in two phase ii studies in patients with recurrent and/or metastatic sgc including mainly acc , a cancer with generally poor outcome . in 50% of patients , clinical benefit ( i.e. , response or stable disease for 6 month )
gefitinib was associated with a 53% stable disease rate ( 10/19 ) in acc , but had no effects on patients with salivary duct tumors and mucoepidermoid cancer .
lapatinib , inhibiting erbb1 and erbb2 tyrosine kinases , was studied in a phase ii trial and stabilized disease for greater than 6 months in 47% of acc patients .
key molecules of the egfr - ras - raf signaling cascade and predictive markers of treatment outcome under anti - egfr therapies have not been comprehensively examined in sgc .
investigations of the mutation status of proteins in the cascade downstream of egfr identified markers for egfr - targeted therapy in colorectal cancer . in recent studies , wildtype braf as well as wildtype kras and intact pten pik3ca
were found to be required for the response of colorectal cancer patients treated with cetuximab or panitumumab [ 5 , 10 , 11 ] .
we were able to demonstrate in this and in a former study that kras and braf mutations seem to be extremely rare in sgc .
these findings imply that salivary gland carcinomas which rarely acquire mutations that result in constitutive activation of the signaling cascade downstream of egfr may be good candidates for anti - egfr therapies .
molecular analyses of alternative members of the egfr signaling cascade , such as akt-1 and mek-1 , may further contribute to elucidating predictive markers of treatment outcome under anti - egfr therapies in sgc . because of its universal availability as a standard methodology in molecular medicine , we designed a braf mutation screening assay based on pcr .
allele - specific pcr , also known as amplification refractory mutation system ( arms ) , is a well - established method for discriminating between different alleles at specific loci resulting from single base mutations [ 12 , 13 ] .
we used the methodology to establish an assay with three pcr primers which allows the discrimination of allele - specific pcr fragments by agarose gel electrophoresis without the need of capillary electrophoresis devices . with our assay
interpretation of the results can be made by simple visual inspection of the stained gel to determine whether or not a specific primer pair amplified a fragment with the template dna .
because microdissected tumor areas were used for the allele - specific pcr , and a control amplification was incorporated into the pcr reaction to ensure dna integrity , we can exclude false negative results .
so far , genomic screening for braf mutations has been based mainly on direct sequencing .
our protocol provides an alternative rapid , sensitive , and cost - effective braf screening method . choriocarcinoma is one of the malignant tumors arising from trophoblastic cells characterized by the secretion of human chorionic gonadotrophin ( hcg ) .
the tumor consisted of two basic cell types : cytotrophoblast and hcg - positive syncytiotrophoblast , the typical histologic features of choriocarcinoma ( 1 - 5 ) .
the serum hcg level is typically elevated , with resultant isosexual pseudoprecocity in children , and menstrual abnormalities , breast enlargement , androgenic changes , or combinations thereof , in adults ( 1 , 2 ) .
it usually arises from fetal trophoblasts and rarely arises from germ cells in the testis or ovary or derives from dedifferentiation of other carcinomas ( 2 , 3 ) .
the most common sites of systemic metastatic disease are lungs , liver and brain via hematogenous spread ( 1 ) .
skin is a rare site for metastatic choriocarcinoma , and even more rare as the initial presenting site for the disease ( 3 - 5 ) . to the best of our knowledge ,
we describe a case of 52-yr - old korean female patient with cutaneous metastasis of choriocarcinoma , diagnosed from a skin biopsy that provided important diagnostic clues to the primary lesion .
a 52-yr - old korean female presented with dyspnea of 2 months duration and cutaneous nodules of 1 month duration on the left side of the neck and the right side of the upper back .
her past medical history was significant for behet 's disease , which had been treated with colchicine for the past 4 yr . routine blood tests showed leukocytosis with a wbc of 17,780/l , anemia with a hemoglobin of 9.4 g / dl and slightly elevated hepatic enzyme levels of got / gpt at 45/51 iu / l .
chest radiography , chest and abdomen - pelvis ct revealed pleural effusion , innumerable variable sized pulmonary metastatic nodules on both lungs and multiple metastatic lesions on the liver , left adrenal gland and left kidney .
a skin biopsy was performed on the nodule at the right side of upper back . at low magnification
, there were several hemorrhagic foci and multiple cellular infiltrations throughout the dermis ( fig .
2a ) . at high magnification , the infiltrated tumor cells consisted of two cell types , and a biphasic pattern , in close proximity .
one element had polygonal shaped , clear cytoplasm and large , irregular shaped , vesicular nuclei with prominent nucleoli , which were identified as cytotrophoblasts .
the other cell population showed eosinophilic cytoplasm with cytoplasmic vacuoles and many dark nuclei , which were identified as syncytiotrophoblasts ( fig .
the immunohistochemical staining for hcg antigen ( 1:600 , polyclonal rabbit , dakocytomation , denmark ) in the cytoplasm of syncytiotrophoblasts ( fig .
magnetic resonance ( mr ) images of the abdomen - pelvis detected diffuse enlargement of the uterus body and a protruding mass in the uterine cavity , which was the primary origin of the tumor ( fig .
her serum hcg level was 700,000 miu / ml ( upper normal limit : 10 miu / ml ) .
she was referred to the gynecological oncology department , where combination chemotherapy was commenced . during combination chemotherapy ,
the serum hcg level has fallen off under 2.0 miu / ml and her skin lesions have almost disappeared .
however , 3 months after 17 cycles , total abdominal hysterectomy and bilateral salpingo - oophorectomy were performed due to elevated serum hcg level ( 4,447.7 miu / ml ) , and she is scheduled to receive post - operative adjuvant chemotherapy .
compared to other organs , skin is an uncommon site of metastatic cancer in the body as it is usually associated with a disseminated disease and grave outcome . occasionally , however
, as in our case , cutaneous metastasis may be the first harbinger of a visceral malignancy of unknown origin and gives an important diagnostic clue ( 3 , 4 , 6 - 8 ) .
cutaneous metastasis of a visceral malignancy has been estimated to be in the wide range of 1.4% to 10.4% according to the type of study undertaken and the site of primary tumor studied ( 6 - 11 ) .
involvement of the skin is likely to be near the area of the primary tumor and in proportion to the incidence of internal malignancy .
the chest , abdomen , and scalp are common anatomic sites to which metastasis localize with the back and extremities being relatively uncommon areas .
breast cancer in women and lung cancer in men are the most common causes of chest metastatic lesion ( 6 - 8 ) . occasionally
, a cutaneous metastatic lesion may show typical histologic features which give a decisive diagnostic clue to the primary origin .
such cases include mucin - containing cells in colon cancer , bile - containing glandular structure in hepatoma , clear cells in renal cell carcinoma and , like our case , cytotrophoblasts and syncytiotrophoblasts in choriocarcinoma ( 6 ) .
choriocarcinoma , in women , is often preceded by a gravid state , which is most often hydatidiform mole ( 50% ) , less frequently abortion ( 25% ) , and normal term pregnancy ( 15% ) .
, it commonly arises from germ cells in the testis ( 1 - 5 ) .
the gross appearance of choriocarcinoma is often dark red or purple colored and resembles that of a friable segment of placenta ( 1 ) .
microscopically , it is characterized by multiple foci of hemorrhage and necrosis around the neoplastic cells , by large sheets of cytotrophoblastic and syncytiotrophoblastic cells , biphasic pattern , and by the absence of hydropic villi .
the cytotrophoblasts have cuboidal shaped , pale cytoplasm and large , irregular shaped , vesicular nuclei with prominent nucleoli .
the syncytiotrophoblasts show eosinophilic cytoplasm with cytoplasmic vacuoles , many hyperchromatic and pleomorphic nuclei with indistinct border , looking like multinucleated giant cells , and may form syncytial knots .
the immunohistochemical staining for hcg antigen is positive in the cytoplasm of syncytiotrophoblasts but not in cytotrophoblasts ( 1 - 5 ) .
other germ cell tumors such as polyembryoma , embryonal carcinoma , dysgerminoma and mixed germ cell tumor can also show hcg positive syncytiotrophoblastic cells but these tumors do not demonstrate a biphasic pattern consisting of cytotrophoblasts and syncytiotrophoblasts , as in choriocarcinoma ( 1 ) .
the most common metastatic sites are , in a descending order of frequency , lung ( 60 - 95% ) , vagina ( 40 - 50% ) , vulva ( 10 - 15% ) , brain , liver ( 10% ) , kidney and spleen ( < 5% ) .
although choriocarcinomas respond relatively well to chemotherapy , cutaneous metastasis is an extremely uncommon presentation and a poor prognostic sign as it is associated only with disseminated disease ( 1 - 5 ) .
reported a case of cutaneous metastatic testicular choriocarcinoma where the patient died 10 days after the initiation of chemotherapy ( 4 ) .
shimizu et al . also reported a case of metastatic disease to the upper back of testicular choriocarcinoma , where the patient died 3 months after the appearance of a cutaneous metastatic lesion . in their literature review , of 7 patients with metastatic choriocarcinoma to the skin
, 5 patients died within 3 months after the appearance of skin metastasis ( 3 ) .
reported another case of choriocarcinoma with metastasis to the chest wall , which responded well to 12 cycles of combination chemotherapy ( 5 ) .
we described an extremely rare patient with metastatic choriocarcinoma presenting initially as a cutaneous lesion .
the diagnosis was made from the typical histological findings of a cutaneous metastatic lesion , which was confirmed by immunohistochemical staining for hcg and from the abdomen pelvis mr images .
this case highlights the importance of a histological examination of skin metastatic lesion , which can provide conclusive evidences to the diagnosis . | braf is the main effector of kras in the ras - raf - mapk axis , a signaling pathway downstream of egfr .
the activation of this cascade is an important pathway in cancer development and is considered a key pathway for therapeutic molecules .
recent studies in metastatic colorectal cancer found that an oncogenic activation of braf by a point mutation in exon 15 ( v600e ) could bypass the egfr - initiated signaling cascade with the effect that patients bearing the mutant braf allele are not likely to benefit from egfr - targeted therapies .
we designed an allele - specific pcr and screened 65 salivary gland carcinoma ( sgc ) of the main histopathological types for the braf v600e mutation .
all 65 sgc in this cohort ( 100% ) presented the braf wildtype . in a previous study
, we found a kras wildtype in 98.5% of sgc .
these findings imply that sgc rarely acquires mutations that result in a constitutive activation of the signaling cascade downstream of egfr and this pleads in favor of further therapeutic trials with egfr - targeting monoclonal
antibodies . |
tramadol has low affinity for - and -opioid receptors and inhibits the reuptake of both nor - epinephrine and serotonin ( 5-hydroxytryptamine ) neurotransmitters .
it stimulates the dopamine ( d2 ) receptors and also inhibits the gamma amino butyric acid release in central nervous system .
common therapeutic doses of tramadol are 50 mg orally and 100 mg with parenteral and rectal route of administration up to 400 mg / day .
the main adverse drug reactions of tramadol are nausea , dizziness , somnolence , drowsiness , increased sweating , vomiting , and dry mouth .
seizure and apnea are the most important life - threatening clinical presentations of tramadol in therapeutic and toxic doses .
from this view , evaluation of laboratory findings including plasma electrolytes , kidney and liver function tests , and blood gas analysis have a critical role for patient monitoring .
as there are few studies about laboratory findings in tramadol acute poisoning , we evaluated the clinical and laboratory findings in acute tramadol - intoxicated cases and their role in the prediction of seizure .
this was a retrospective study on patients with acute tramadol poisoning who referred to loghman hakim hospital poison center , tehran , iran from january to april 2012 .
the exclusion criteria were co - ingestion , intoxication with unknown dose of tramadol , uncertainty about time of tramadol ingestion , onset of a seizure before admission on hospital , the past medical history of epilepsy and history of drug / substance abuse .
data such as patients age , sex , time of ingestion , ingested dose , cause of intoxication , respiratory rate , pulse rate , systolic and diastolic blood pressure , temperature , coma grade scale on admission time , and therapeutic interventions and patients outcome were extracted from the medical records .
laboratory findings including blood sugar , blood urea nitrogen ( bun ) , creatinin ( cr ) , sodium ( na ) , potassium ( k ) , liver function tests , cell blood count , and blood gas on admission time were retrieved from patients medical records .
the data were expressed as mean standard deviation / standard error ( se ) for continuous or discrete variables and as frequency and percentage for categorical variables .
we used the student 's t - test and mann whitney u - test for statistical analyses of continuous variables with and without normal distribution , respectively .
linear correlations between variables were assessed by spearman and expressed as the spearman correlation coefficient .
the data were expressed as mean standard deviation / standard error ( se ) for continuous or discrete variables and as frequency and percentage for categorical variables .
we used the student 's t - test and mann whitney u - test for statistical analyses of continuous variables with and without normal distribution , respectively .
linear correlations between variables were assessed by spearman and expressed as the spearman correlation coefficient .
we used spss software ( version 13 , spss inc . , chicago , il , usa ) .
a total of 144 patients including 111 men ( 77% ) and 33 ( 23% ) women with the mean age of 23.7 6.9 ( range = 15 - 57 ) years old included in this study .
the average time between ingestion and admission on hospital ( mean se ) was 292.2 30 min ( range = 30 - 3600 min ) .
mean duration of hospitalization was 17.9 10.6 h ( range = 3.6 - 80 h ) . in all of the cases , the route of exposure was oral , and the most drug dosage form was tablet ( n = 142 ) and then capsule ( n = 2 ) .
the mean ingested dose ( mean se ) was 1971.2 233.4 mg ( range = 100 - 20000 mg ) . in most of the cases ( n = 99 , 68.8% ) the cause of intoxication was suicide and then abuse ( n = 45 , 31.2% ) [ table 1 ] .
demographic findings in tramadol - intoxicated patients major cases had stable vital signs on admission and the related data are summarized in table 2 .
128 ( 88.9% ) of patients were conscious , and 16 of them had a decreased level of consciousness .
seizure ( 47.9% ) , nausea ( 29.9% ) , vomiting ( 22.2% ) , drowsiness ( 20.1% ) , dizziness ( 18.1% ) , lethargy ( 6.3% ) , apnea ( 5.6% ) , agitation ( 4.2% ) , headache ( 1.4% ) , blurred vision ( 1.4% ) , ataxia ( 0.7% ) , anxiety ( 0.7% ) , sweating ( 0.7% ) , and nystagmus ( 0.7% ) were the most clinical findings in tramadol - intoxicated patients [ table 2 ] .
laboratory findings on admission time in the intoxicated patients have been summarized in table 3 .
clinical findings in tramadol - intoxicated patients on admission time laboratory findings in tramadol - intoxicated patients on admission time we divided the cases with regard to occurrence of seizure during hospitalization .
the results showed that there were significant differences between cases with seizure and cases without seizure according to time interval between tramadol ingestion and hospital admission ( tibtiha ) ( mean se ) ( 330.3 53.2 vs. 257.3 30.1 min , p = 0.01 ) , ingested dose ( mean se ) ( 1395.7 218.3 vs. 2500.7 390.7 mg , p = 0.006 ) , with odds ratio 2.7 ( 1.03 - 7.09 , 95% confidence interval [ ci ] ) , dizziness ( 3 cases vs. 23 cases , p = <
0.0001 ) , with odds ratio 0.1 ( 0.29 - 0.36 , 95% ci ) , pco2 ( 51.2 8.5 vs. 48.4 8.6 mmhg , p = 0.03 ) , with odds ratio 0.58 ( 0.27 - 1.24 , 95% ci ) , and total bilirubin ( 0.5 0.2 vs. 0.7 0.4 mg / dl , p = 0.002 ) . there was a correlation between ingested dose ( r = 0.2 , p = 0.006 ) , paco2 ( r = 0.2 , p = 0.03 ) , tibtiha ( r = 0.2 , p = 0.01 ) , total bilirubin ( r = 0.3 , p = 0.002 ) , dizziness ( r = 0.3 , p = 0.000 ) , and seizure .
tramadol abuse and overdose is one of the most frequent health problems in iran and worldwide . in this study , we report clinical and paraclinical findings in 144 cases with pure tramadol poisoning who referred to a referral - poisoning center in tehran , iran .
furthermore , the results indicated that oral route is the most common route of exposure , which is similar with the findings of our previous studies .
the frequency of other clinical manifestations including lethargy , coma , nausea , vomiting , agitation , and respiratory depression in our study were different with previous studies .
tramadol is metabolized by cytochrome p450 ( cyp450 ) enzymes ( mainly 2d6 isoenzyme ) to its active metabolites m1 ( o - desmethyl tramadol ) , m2 ( n - desmethyl tramadol ) , m3 ( n , n - didesmethyl tramadol ) , m4 ( o , n , n - tridesmethyl tramadol ) , and m5 ( o , n - didesmethyl tramadol ) .
m1 metabolite has more affinity ( 200 times ) for the -opioid receptors and also it has more inhibitory effect on biogenic amine reuptake than that of parent drug molecule and may be responsible for tramadol induce analgesia or seizure in intoxicated patients . in this
regard , genetic polymorphism in humans may affect the tramadol metabolism and its peak blood concentration resulting to a different frequency of tramadol adverse effects or clinical presentations during therapeutic doses or intoxication . although the increasing of liver function tests , serum bun and cr due to liver and kidney damage have been demonstrated in chronic administration of tramadol in experimental model , but in our study due to acute onset of toxicity we did not observe any increase in liver function tests , bun and cr and this is in concordance of the previous study .
rhabdomyolysis and rise of creatine phosphokinase ( cpk ) have been reported as a rare and serious complication in tramadol intoxication in the previous studies , which was observed in our study too .
although in the previous studies , prolong immobilization and multiple seizures have been described as one of the reasons for the rise of cpk and rhabdomyolysis , but in our study there was no significant difference in level of cpk in the tramadol - intoxicated patients with seizure in comparison to cases without seizure .
le berre et al . , reported tramadol induced hyponatremia which described as a result of inappropriate antidiuretic hormone secretion . in this study , the level of na , and k was within the normal range .
the pco2 level was above normal range which could be attributed to tramadol - induced respiratory depression , which has been reported previously .
in this study , the mean of ingested dose in the seizure group was less than those cases without seizure which is in contrasts with the result of the previous study .
one of the explanations is the difference between two groups with regard to tibtiha , which was significantly longer in seizure group .
furthermore , as mentioned previously , the other reason could be the genetic polymorphism in patients .
the main limitation of this study is its retrospective design , which should be considered in the interpretation of the results . with this regard
there were significant differences between seizure and nonseizure cases with regard to tibtiha , ingested dose , and pco2 .
furthermore , we showed poor correlation between tramadol ingested dose , tibtiha , pco2 , and seizure in tramadol - intoxicated cases .
hrr involving in the data collection and data analysis . ss and ksn involving in the study design , conducting of the study and data analysis . familial glucocorticoid deficiency ( fgd ) is a rare autosomal recessive disorder resulting from adrenal unresponsiveness to adrenocorticotropic hormone ( acth ) .
this disease is characterized by low serum cortisol concentrations in the presence of grossly elevated plasma acth levels .
affected individuals typically present with hyperpigmentation , hypoglycemic seizures , failure to thrive , failure to thrive and recurrent infections .
causal mutations of fgd have been identified in mc2r , mrap , mcm4 , txnrd2 , star and nnt .
mc2r accessory protein ( mrap ) , a 19-kda single - transmembrane domain protein , is essential for trafficking of mc2r from the endoplasmic reticulum ( er ) to the cell surface and subsequent signaling in response to acth .
mutations in mrap are responsible for 1520% of fgd cases , named fgd type 2 . until now
, this mutation has not been reported in chinese han cases of fgd . in the current paper
, we describe a chinese han child who presented with isolated hyperpigmentation at birth and a homozygous splice mutation ( c.106 + 1delg ) in mrap .
a female infant was referred to our neonatal unit at the age of nine days for evaluation of hyperpigmentation of the skin . she was the product of an uncomplicated pregnancy and was born by caesarian section for concerning cardiotocographic changes during labor .
her sibling had died on the third day of life with hyperpigmentation of the skin .
birth size was normal ( weight 2800 g , 0.99 sds ; length 46 cm , 1.69 sds ; head circumference 33 cm , 0.74 sds ) and apgar scores was 8 and 9 at 1st and 5th minute , respectively . on physical examination ,
weight was 2740 g ( 1.31 sds ) , length 47 cm ( 2.47 sds ) , and head circumference was 34.5 cm ( 1.5 sds ) .
endocrine investigations revealed low baseline 8 am serum cortisol ( < 1.0 g / dl ) with extremely elevated acth ( 1250 pg / ml ) .
testosterone , 17-oh progesterone , estradiol , progesterone , luteinizing hormone , folliculin , and prolactin , free t4 , and tsh levels were normal .
based on these findings , a familial glucocorticoid deficiency was suspected and oral hydrocortisone treatment at dose of 20 mg / m per day was started .
hydrocortisone dose ( varying from 10 to 15 mg / m per day ) adjustment was based on acth and cortisol concentrations .
after 4 weeks , acth level was 278.0 pg / ml and cortisol level was 42.4 g / dl , and hydrocortisone dose was reduced to 15 mg / m per day ( given in divided doses twice daily ) .
the acth level was suppressed to slightly above the normal limit and the cortisol level is normal during the follow - up period . on her most recent visit , at the age of 12 months ,
hydrocortisone dose was 11 mg / m per day ( 5 mg was give once daily in the morning ) , the acth level was 25.10 pg / ml and cortisol level was 33.4 g / dl , and her skin was slightly lightened ( fig .
her weight was 10 kg ( 0.84 sds ) , length 70 cm ( 1.67 sds ) , and head circumference was 46.5 cm ( 1.12 sds ) .
genomic dna was extracted from peripheral blood leucocytes from the infant and her family after informed consent was obtained . for the molecular diagnosis
, a custom panel - based next - generation sequencing approach has been used to sequence all known adrenal gland diseases - associated genes in this child .
we found a homozygous deletion of one nucleotide at the canonical 5 donor splice site ( c.106 + 1delg ) in intron 3 of mrap gene .
this would result in the skipping of exon 3 and a prematurely terminated translation product ( ( 1 ) ) .
her parents and maternal grandmother were found to be heterozygous carrier for the same mutation ( fig .
we report a case of fgd with homozygous mutation of mrap in a chinese han neonate .
this girl presented with isolated hyperpigmentation , have low cortisol and high acth with normal electrolytes . a homozygous splice mutation ( c.106 + 1delg ) of mrap confirmed the diagnosis of fgd type 2 . her parents and maternal grandmother had the same mutation .
until now , this mutation has not been reported in chinese han cases of fgd .
fgd is a rare autosomal recessive disorder characterized in laboratory testing by glucocorticoid deficiency and markedly elevated acth levels .
patients with fgd usually present during neonatal period to late childhood with symptoms related to cortisol deficiency and acth excess . in the neonatal period
, these symptoms may include hyperpigmentation , hypoglycemia , irritability , jitteriness , lethargy , respiratory distress , cyanosis , apnea , hypotonia , seizures , shock , or sudden death .
newborns can present with a positive family history of early - unexplained infant deaths or other affected family members supports a diagnosis .
the severe pigmentation of the skin is due to the over - stimulation of mc1r ( cutaneous msh receptors ) by high circulating msh which is a byproduct of acth synthesis from proopiomelanocortin .
this hyperpigmentation fades once proper treatment is initiated with glucocorticoids , which reduce acth concentrations .
this would suggest that the fetal corticotrophs could produce excessive plasma acth in response to low fetal cortisol , which in turn acts on melanocytes to promote eumelanin synthesis before birth . in most of the fgd patients suppression of acth levels
is difficult , and therefore is not used as a goal for therapy . in this case , replacement treatment with hydrocortisone suppressed acth level to slightly above the normal limit and partially resolved the hyperpigmentation . acquired causes of adrenal insufficiency such as adrenal hemorrhage , trauma and infections were excluded by history and laboratory findings .
congenital adrenal hyperplasia was excluded by hormone analysis ; congenital adrenal hypoplasia was also unlikely , because she had normal serum 17-oh progesterone and no mutation of the dax1 gene .
mrap , located at 21q22.1 , is a small single - pass transmembrane domain protein , which is essential for the processing of the acth receptor ( melanocortin 2 receptor , mc2r ) and its trafficking from the er to the cell surface .
this mutation will lead to skipping of exon 3 and early truncation of the protein and the absence of the mc2r interacting transmembrane domain . as a result
, mc2r is retained within the er and fails to reach the cell surface which lead to acth resistance and adrenal insufficiency .
mutations in mrap causing fgd2 were first described in 2005 . so far over 9 different mutations of mrap in fgd type 2 patients
have been documented all of which are splice site or nonsense mutations and are predicted to produce proteins lacking the transmembrane domain essential for interaction with mc2r , . in our patient , we found a newborn with fgd type 2 , presenting with severe hyperpigmentation .
she was found to have a splice - site mutation in the mrap gene , hence explaining the early presentation seen in the case . in their study , metherell et al . identified the 1-bp deletion , c. 106 + 1delg , in 6 individuals from 5 families with glucocorticoid deficiency , making this the second frequent mutation causing fgd unrelated to defects in the mc2r gene . to our current knowledge , 1-bp deletion ( c. 106 + 1delg ) in intron 3 of mrap gene , identified in the dna of the patient ,
cases of the condition have been reported in white , , black , indian , and middle eastern populations . to our knowledge
, our patient is the first reported chinese han patient with fgd type 2 , with a known mrap mutation .
the patient was the offspring of non - consanguineous parents and her sibling had died in the neonatal period , possibly due to glucocorticoid insufficiency .
prolonged acth excess or glucocorticoid deficiency increases linear growth , while early diagnosis and appropriate therapy in this case enabled the patient to achieve normal developmental milestones .
further studies describing new cases and mutations causing fgd will contribute to understanding the mechanism of this rare and potentially life - threatening disease . | background : tramadol is a centrally acting analgesic with opioid and nonopioid properties , which extensively used in the relief of mild to moderate pain .
tramadol poisoning is a common cause of acute pharmaceutical poisoning in iran .
there are a few studies about clinical and laboratory findings related to acute tramadol poisoning . therefore , the aim of this study was to demonstrate the clinical and laboratory findings in tramadol acute poisoning cases.materials and methods : this was a retrospective descriptive study of patients with acute tramadol poisoning who referred to loghman hakim hospital poison center during january to april 2012 .
data such as patient 's age , sex , time of ingestion , ingested dose , cause of poisoning , mean duration of hospitalization , patient 's clinical presentations , laboratory findings , therapeutic measures , and patient 's outcome have collected in a predesigned checklist.results:a total of 144 patients including 111 men ( 77% ) and 33 women ( 23% ) with acute tramadol poisoning was included in this study .
the mean ingested dose was 1971.2 mg ( 100 - 20000 mg ) .
seizure ( 47.91% ) was the most frequent clinical symptom .
blood gas on admission showed ph ( 7.3 0.1 ) , pco2 ( 49.7 8.6 mmhg ) and hco3 ( 24.1 3.8
meq / l ) , indicating pure acute respiratory acidosis may be occurred in tramadol - intoxicated patients . there were significant differences between tramadol - intoxicated cases with and without a seizure with regard to the time interval between ingestion and admission on hospital , ingested dose and pco2.conclusion:seizure and rise of pco2 were the most findings in this study . |
pulmonary arteriovenous malformation ( pavm ) , first described in 1897 by churton , is characterized by abnormal communications between pulmonary veins and arteries , and is known to disturb the filtering action of pulmonary capillaries , causing thromboembolic event in systemic circulation .
most of the pavms have no symptoms , and are detected as abnormal shadow in the chest .
conclusive diagnosis is generally made by means of pulmonary arteriography or three - dimensional computed tomography ( 3d - ct ) angiography .
while hypervascular lesions can mimic pavm , we observed a rare case of a lung granuloma mimicking pavm , and performed video - assisted thoracic surgery ( vats ) .
a 76-year - old woman , who was otherwise symptom - free , was admitted to our hospital because an abnormal shadow was detected in the left lung field on her chest x - ray .
chest computed tomography ( ct ) revealed a 20 mm 14 mm nodule with well - defined margins and smooth contours in the left upper segment ( fig .
an enhanced solitary lung nodule , connected with linear structures suggestive of feeding artery and drainage vein , was revealed by contrast - enhanced 3d - ct ( fig .
arterial blood gas analysis showed hypoxemia , with arterial oxygen pressure ( pao2 ) 56 mmhg in room air .
first , the patient was placed in the right lateral decubitus position , and the left lung was deflated .
intraoperatively , elastic hard nodule was palpable in the left upper segment and bruit was not convincing .
the nodule was nontortuous in shape , covered with the visceral pleura , and neither the feeding artery nor the drainage vein was detected .
histopathological findings revealed multiple foci of coagulative necrosis surrounded by epithelioid cell granuloma containing langhans - type multinucleated giant cells , involving the medium - sized blood vessels in the pulmonary parenchyma .
the lung granuloma was difficult to be preoperatively distinguished from pavm , because hypervascular lesion such , as inflammatory changes can present as strongly enhanced nodules after injection of contrast material .
the patient had an uneventful postoperative course and was discharged 14 days after the operation .
causes of acquired or secondary pavm include chest trauma , thoracic surgery , hepatic cirrhosis , infections ( actinomycosis , schistomiasis ) , metastatic carcinoma and systemic disease . twenty eight percent of cases are considered to have no symptoms .
it was reported that when a single isolated pulmonary arteriovenous malformation is 2 cm or smaller , no symptoms appear . generally ,
when pavm is 2 cm or more , symptoms such as breathing difficulty , cyanosis , hypoxemia , finger clubbing and polycythemia occur , and the rate of occurrence of severe complications , such as rupture of the malformation , hemoptysis , cerebral infarction and cerebral abscess , is approximately 30% .
the patient in the present case was asymptomatic and had no history of chest trauma , surgery , infection or systemic disease .
abnormal vascular structures , such as pavm are not convincing in histopathological findings . coincidentally , the inflammatory granuloma of the lung looked almost identical to pavm because of the nontortuous shape with linear structures suggestive of feeding artery and drainage vein .
the resection of lung granuloma is highly successful for an isolated malformation , and mortality rate is low in this surgery .
embolization is an appropriate treatment modality in multiple pavm for which surgery is not suitable .
complications of embolization include pleuritic chest pain , pulmonary infection , air embolism , migration of coils and paradoxical embolism .
although pulmonary angiography or contrast - enhanced 3d - ct has been the standard diagnostic tool for pavm , it has limitations in detecting pavms presenting as small nodules and enhanced nodules . in our case , since the enhanced lung nodule size was 22 mm in diameter , it mimicked pavm . in this patient , it was difficult to distinguish the lung granuloma from pavm , preoperatively . though there is a report which shows lung cancer mimicking pavm , to our knowledge , this is the first report of a lung granuloma mimicking pavm .
in conclusion , clinicians should pay particular attention to enhanced nodules to rule out a variety of disorders , including neoplasm , infection , inflammatory and vascular abnormality when pavm was suspected .
for the purpose of not only diagnosis but also for safety in treatment of pavm , the surgical approach seems appropriate .
written informed consent was obtained from the patient for publication of this case report and accompanying images .
a copy of the written consent is available for review by the editor - in - chief of this journal on request .
yoshinobu ichiki : study design , data collections , data analysis , writing ; junji kawasaki : data collections ; takayuki hamatsu : data collections ; taketoshi suehiro : data collections ; makiko koike : data collections ; fumihiro tanaka : study design , data collections , data analysis ; keizo sugimachi : data collections . c57bl/6j male mice ( 25 months of age ) were housed individually on a 12 hr/12 hr light / dark schedule with lights on at 7 a.m. ( zt0 ) and handled for 6 days .
mice were sleep - deprived ( sd ) in their home cages for 5 hours by gentle handling beginning at zt5 or left undisturbed ( non - sleep - deprived mice , nsd ) . for contextual fear conditioning experiments , animals were placed in a novel chamber for 3 minutes , and received a 2-second , 1.5 ma footshock after 2.5 minutes .
mice received intra - peritoneal injections of rolipram ( rol ; 1 mg / kg ) or vehicle ( 2% dmso in 0.9% saline ) immediately and 2.5 hours post - training .
testing of contextual memory was performed 24 hours after training in the trained context and 48 hours after training in a novel chamber .
1-train ltp was induced by a single 100 hz , 1-second duration train of stimuli .
4-train ltp consisted of 4 trains applied with a 5-minute inter - train interval ; for massed 4-train ltp a 5-second inter - train interval was used .
theta - burst stimulation ( tbs ) consisted of 40-ms duration , 100 hz bursts delivered at 5 hz for 3 seconds ( 15 bursts of 4 pulses per burst , for a total of 60 pulses ) .
chemical ltp was induced by treatment of slices for 15 minutes with 5 m forskolin ( fsk ) in 0.1% ethanol , or a combination of 50 m forskolin and 30 m 3-isobutyl-1-methylxanthine ( ibmx , in water ) .
rolipram ( 0.1 m in 0.1% dmso ) was applied for 60 minutes , beginning 30 minutes before tetanization .
camp assays on ca1 regions of hippocampal slices 10 minutes after treatment for 15 minutes with forskolin ( 50 m ) , forskolin + ibmx ( 30 m ) , or vehicle ( 0.1% etoh ) were performed by radioimmunoassay according to kit instructions .
camp - specific pde activity assays29 and western blots for pde4a530 were performed as previously described .
full methods and any associated references are available in the online version of the paper at www.nature.com/nature .
| introductionwhile hypervascular lesions in the lung are known to mimic pulmonary arteriovenous malformation ( pavm ) , here we report a rare case of lung granuloma mimicking pavm , on which video - assisted thoracic surgery ( vats ) was performed.presentation of casea 76-year - old woman without any symptom was admitted to our hospital because of abnormal shadow in the left lung field on chest x - ray .
a 20 mm 14 mm nodule with well - defined margins and smooth contours in the left upper segment was detected in her chest computed tomography ( ct ) .
contrast - enhanced three - dimensional ct ( 3d - ct ) revealed an enhanced solitary lung nodule , which was connected with linear structures suggestive of feeding artery and drainage vein .
thus , we made a preoperative diagnosis of pavm by performing partial pulmonary resection by vats .
intraoperatively , elastic hard nodule was palpable in the left upper segment and bruit was not convincing .
histopathological findings revealed multiple foci of coagulative necrosis surrounded by epithelioid cell granuloma containing langhans - type multinucleated giant cells , involving the medium - sized blood vessels in the pulmonary parenchyma .
abnormal vascular structures , such as pavm were not convincing .
based on these findings , a diagnosis of left lung granuloma was made.discussionpreoperatively , it was difficult to distinguish the left lung granuloma from pavm , because hypervascular lesion , such as inflammatory changes can present as strongly enhanced nodules after injection of contrast material.conclusionsurgical approach seems appropriate , not only for the purpose of diagnosis , but also for the safety in treatment of a pavm . |
the treatment of patients with multiple trauma requires a different approach to that of patients with regular trauma because they are threatened not only by the injuries themselves , but also by the metabolic disruptions that follow.1 delay in surgery , blunt trauma , extensive soft tissue damage , and combined orthopedic and vascular injuries have been associated with an increased risk of amputation , while associated nerve and bone injuries with extensive soft tissue damage are risk factors for a poor outcome.2 acute renal failure is the main cause of death in patients with war wounds and trauma of the extremities
. it would be helpful to minimize mortality in these patients by managing shock in a timely manner and taking the decision to amputate appropriately and promptly.3 severe head injury is known to be a major determinant of mortality in patients with multiple injuries , but other injuries also contribute to the clinical outcome.4,5 different mechanisms of injury , such as motor vehicle crashes , falls , or pedestrians being struck by a motor vehicle , impart varying degrees of force and energy transfer that may impact outcomes ; this was found to predict mortality and functional impairment independently at hospital discharge.6 acute lower extremity compartment syndrome is a devastating complication that often presents silently in critically injured patients.7 patients who underwent delayed fasciotomy had twice the rate of major amputation and a three - fold higher mortality rate.8 the aim of this study was to evaluate if injuries of the extremities are associated with a higher one - month mortality rate than other types of associated trauma .
this prospective , observational , cohort study was carried out in the regional emergency center of hospital de base after prior approval by the ethics research committee of the so jos do rio preto medical school .
the emergency department follows a systematic pathway to provide initial assistance to accident victims using the atls ( advanced trauma life support ) protocol .
all live accident victims treated in the emergency department from july 2004 to june 2005 were included .
patients who were dead on arrival and not submitted to any type of inhospital resuscitation procedure were not included in the study .
accident victims were allocated to two groups , ie , those with severe injuries to the extremities or pelvis ( abbreviated injury scale [ ais ] 34 ) and those without injuries or with minor injuries to the extremities ( ais 02 ) .
the fisher s exact test and relative risk were used for statistical analysis , and an alpha error of 5% ( p 0.05 ) was considered statistically significant .
a total of 3489 patients were evaluated in this study ; 3244 ( 92.98% ) did not have severe trauma of the extremities ( ais 02 ) , 34 ( 1.05% ) of whom died .
severe injuries of the extremities ( ais 34 ) occurred in 245 ( 7.02% ) of the patients , with 13 ( 5.31% ) dying ( fisher s exact test : p = 0.001 , relative risk 5.063 , 95% confidence interval [ ci ] : 2.7079.467 , table 1 ) .
table 2 shows the age , type of injury sustained , and time until death after trauma for the patients who died .
of the 245 patients with ais 34 , 71 ( 28.98% ) were women and 174 ( 71.02% ) were men , with the mean age of the men being 40.1 20.5 years and of the women 60.2 23.6 years .
this study assessed whether severe injuries of the extremities affect the overall mortality rate in accident victims .
the death rate was found to be higher for patients with ais 34 than in those without injuries or with minor injuries to the limbs .
thus , these data serve as a warning in respect to increased risk of death in orthopedic patients compared with general trauma patients . when the severity of specific injuries in accident victims is reported in the literature ,
head trauma is cited as one of the main causes of death.5,6 however , there are few data in the literature about the association between death and injuries to the extremities .
one study reported that the mortality rate in accident victims with extremity injuries was higher in pedestrians struck by motor vehicles ( 20% ) , and for those with head injuries , it was higher for motor cycle crash victims ( 16%).6 the first phase of management for these patients aims to control bleeding , by surgical intervention if necessary , and to prevent further wound contamination .
the second phase consists of resuscitation in the intensive care unit , and the third phase aims at definitive repair of the injuries sustained.9 pelvic injuries represent a thorny and stubborn therapeutic challenge .
rapid diagnosis and effective treatment ( damage control ) of these injuries play a key role in the patient s survival , inasmuch as the mortality of multiply injured patients with pelvic ring disruption remains high ( 20%35%).10 the preclinical management of patients with multiple trauma influences the prognosis regarding mortality and morbidity .
diagnostic overview , protection of vital functions in the special circumstance of shock , immobilization of the spine , and treatment of isolated injuries are an essential part of preclinical management.11 the type of trauma is known to influence the mortality rate.12 in this study , all the patients received specialized pre - hospital assistance , and were treated in a regional trauma reference center . in spite of all the care given at the scene of the accident through to discharge from hospital , trauma of the extremities was a significant cause of death .
accident victims with injuries of the extremities are at higher risk for death than those with other types of trauma . osmotic demyelination syndrome ( ods ) including central pontine myelinolysis ( cpm ) and extrapontine myelinolysis ( epm ) following overly rapid correction of hyponatremia has been well recognized515 ) .
although ods occurring after transsphenoidal resection of pituitary adenoma has been reported occasionally111618 ) , to our knowledge , few preoperative epm cases with pituitary adenoma have been reported14 ) .
unfortunately , the outcomes of patients with ods are often disappointing with high rate of mortality and irreversible neurological deficits5 ) . here
, we report a case of preoperative epm with nonfunctional pituitary macroadenoma who made a great clinical recovery after supportive therapy and transsphenoidal adenoma resection .
a 38-year - old man was admitted to our hospital in december 2012 because of nausea , malaise , altered mental status , behavioral disturbances , dysarthria and dysphagia .
the patient had been well until 2 months before admission , when nausea and malaise developed .
his wife also reported that the patient had two episodes of transient loss of consciousness .
he was sent to the emergency department of an outside hospital . at presentation , the patient had no sign of dehydration like poor skin turgor , orthostasis or decreased urine output , nor sign of volume overload including peripheral edema , ascites or elevated jugular vein pressure .
serum sodium of 102 mmol / l , potassium of 3.9 mmol / l and phosphate of 1.08
mmol / l were reported . the complete blood count and the remainder of metabolic panel including
intravenous hypertonic saline was given with an average correction rate of 8 mmol/24 h. despite slow rate of correction , neurologic symptoms including restlessness and irritability developed . in the following days , the patient developed apathy , confusion and obtundation , followed by dysarthria , dysphagia , paraparesis , behavioral disturbances and disorientation .
endocrine studies revealed a serum cortisol concentration ( 8 : 00 a.m. ) of 62.4 nmol / l ( normal , 138635 nmol / l ) , plasma adrenocorticotrophin ( acth ) of 1.03 pmol / l ( normal , 2.217.6 pmol / l ) .
miu / l ) ; serum free thyroxine ( ft4 ) was 6.91 pmol / l ( normal , 11.522.7 pmol / l ) , and serum total thyroxine ( t4 ) was 43.0 nmol / l ( normal , 65155
cranial magnetic resonance imaging ( mri ) was performed and a pituitary macroadenoma ( fig .
one week before admission , mri was repeated and epm was detected at bilateral basal ganglion and thalamus ( fig .
the patient was referred to our hospital because of persistent neurologic deficits . on physical exam , he was unresponsive to verbal or pain stimuli .
hormone studies showed normal atch , cortisol , tsh , t4 , and ft4 , decreased growth hormone and insulin - like growth factor 1 , normal prolactin , decreased gonadotrophin ( follicle - stimulating hormone and luteinizing hormone ) , estrogen and testosterone .
the patient had several episodes of restlessness and agitation with shouting and fighting lasting 10 minutes every day .
the decision of surgery was made then based on the improved general state of the patient and the intent to remove the underlying cause of hypopituitarism and hyponatremia .
pathologically , the mass was confirmed to be nonfunctional adenoma by hematoxylin and eosin ( h&e ) staining and immunohistochemical examination .
pseudorosette patterns were found on h&e staining and nuclear division was less than one per 10 high power field .
the tumor cells were scattered positive for p53 and ki-67 . and ki-67 index was < 1% .
the immunostainings for acth , fsh , lh , prl , tsh and gh were negative .
recent hormone studies showed normal level of acth , cortisol , tsh , ft4 and t4 .
the patient was able to resume his work as a bank accountant three months after the surgery .
the latest follow - up mri was obtained 7 months postoperatively , and these images showed demyelination at bilateral basal ganglions and thalamus resolved and pituitary adenoma resected ( fig .
among various etiologies of hyponatremia , endocrine disorders , including adrenal insufficiency and hypothyroidism , are uncommon causes which may be overlooked1712 ) .
while hyponatremia can be caused by hypopituitarism , severe hyponatremia occurring as the presenting manifestation of pituitary adenoma is rare8 ) . at the onset of hyponatremia ,
given the endocrine abnormality and histology - confirmed pituitary adenoma , we propose that secondary adrenal insufficiency and hypothyroidism resulting from hypopituitarism was the underlying cause of his hyponatremia .
however , demyelination can occur even with this correction rate ( as in our case ) , especially in those with other ods risk factors5 ) .
except for the rate of correction , serum sodium concentration at presentation and the duration of the hyponatremia are also important risk factors . on presentation ,
our patient had been symptomatic for 2 months and the serum sodium level was as low as 102
therefore , patients with more than one risk factors for ods may still be at risk for development ods at " normal " rates of correction and may require slower rates . if ods does develop despite the steps taken to avoid overly rapid correction , effective management is rare .
although reintroduction of hyponatremia , plasmapheresis and corticosteroids has been used , most evidence comes from animal models and data in humans are limited to case reports2513 ) .
a more recent review of 32 german patients with ods showed a much better outcome with only 30% having irreversible and incapacitating neurological deficits9 ) .
this review suggests that patients with ods can survive if the nonspecific secondary complications of transient illnesses such as aspiration pneumonia , deep venous thrombosis , and pulmonary embolism can be avoided .
some patients with ods can recover function after prolonged periods of severe neurologic impairment59 ) .
as in our case , the patient had developed progressive neurologic symptoms for two months on admission to our hospital .
the patient was able to resume the job as a banking account after surgery , indicating complete recovery from neurologic and cognitive deficits .
therefore , supportive therapy should be continued for at least six to eight weeks before concluding that the deficits are irreversible .
surgery is the ultimate effective treatment for nonfunctioning adenoma , which not only removes the underlying cause of hypopituitarism , but also reverses or prevents vision impairment . according to a systemic review and meta - analysis evaluating the outcome of surgical treatment for nonfunctioning pituitary adenomas , pituitary hypofunction
improved in as many as 30 percent after surgery10 ) . while for patients with epm , appropriate surgical timing of adenoma resection is of great importance .
neurologic deficits of preoperative epm such as decreased level of consciousness , paraparesis , dysarthria and dysphagia , posed great challenge to the transsphenoidal operation of pituitary adenoma .
risk of complications such as aspiration , respiratory failure , and deep vein thrombosis is much higher than patients without neurologic deficits49 ) . to minimize the risk of surgery and to remove the underlying cause of hyponatremia
, we selectively arrange the operation after dysphagia and dysarthria resolved , and the patient became ambulant and able to obey simple commands .
we report a case of preoperative epm with nonfunctional pituitary macroadenoma who made a great clinical recovery after supportive therapy and transsphenoidal adenoma resection .
more cases are required to clarify the prognosis of ods and identify the factors associated with better outcome . | background : the aim of this study was to evaluate one - month hospital mortality in victims with injuries of the extremities.methods:all accident victims admitted to the hospital de base in so jos do rio preto , brazil , during the period from july 2004 to june 2005 , were evaluated in an observational study .
patients were classified using the abbreviated injury scale ( ais ) .
patients with severe injuries of the extremities ( ais 34 ) were compared with those without injuries or with minor extremity injuries ( ais 02).results : a total of 3489 accident victims were evaluated ; 3244 ( 92.98% ) did not suffer injuries or had minor injuries of the extremities ( ais 02 ) and 245 ( 7.02% ) had severe injuries ( ais 34 ) .
of the 245 patients with ais 34 extremity injuries , 13 ( 5.31% ) patients died , and of those without severe injuries to the extremities , 34 ( 1.05% ) died ( fisher s exact test p = 0.0000 , relative risk 5.063 , 95% confidence interval [ ci ] : 2.7079.467).conclusion : patients with injuries of the extremities are at greater risk of death than accident victims with other types of trauma . |
in recent years , coupling of theoretical and experimental approaches in the study of protein folding has resulted in providing fruitful clues .
experimental and computational protein design provides vital clues to understand the protein folding process , and it is of considerable interest in the area of protein science to engineer proteins with novel folds and desired functions .
the field of protein design has a unique history where researchers from diverse discipline come together to explore novel catalytic , pharmaceutical , structural , and sensing properties of amino acids in proteins .
interestingly , a designed eleven amino acid sequence folded as a helix in one position and as a sheet in another position in the protein sequence .
this work has enabled to explore the role of nonlocal interactions in the formation of secondary structure .
subsequently , helices were transmuted into sheets to understand the conformation change phenomenon and illustrate that not all the amino acids play an equal role in specifying a fold . utilizing the knowledge offered by several protein design groups , kuhlman et al . in 2003
have computationally designed a 93-residue / protein called top7 and found that the protein could be experimentally folded and extremely stable .
this pioneering work has enabled further research to understand the contribution of each amino acid residue in a protein to adopt a certain fold .
hence , emphasizing that protein design could be a powerful experiment to understand the processes that underlie conformational plasticity in proteins .
explored how two proteins with almost similar amino acid sequences change their fold and function . following the contribution of various theoretical and experimental protein science research groups , several such engineered proteins with selective
nevertheless , the design of such a pair of proteins with high sequence identity with completely different topologies can be viewed as a challenge to the well - accepted paradigm that similar sequences always tend to fold into similar three - dimensional structures .
an analysis of the literature reveals that the design of two highly identical proteins with different folds and functions is challenging and time bound as shown in [ table 1 ] .
streptococcus protein g contains two types of domains ( ga and gb ) that bind to serum proteins in blood .
the natural versions of ga and gb domains share no significant sequence homology and have different folds , 3 and 4 + , respectively . from the above two parent proteins , high - identity versions of ga and gb were synthesized .
interestingly , small and critical differences in the sequences of the two proteins determine the topology of the protein early on the folding pathway .
in addition , two proteins named ga88 ( pdb i d : 2jws ) and gb88 ( pdb
i d : 2jwu ) by mutation experiments from the streptococcus protein g with 88% sequence identity adopt different structures and functions and these proteins are valuable tools to understand the contribution of residues to adopt a particular fold .
these two proteins vary only at seven positions out of 56 amino acids , which are shown in [ figure 1 ] .
this design has made a breakthrough in the field of protein science and contradicts the general statement that
following this , we have carried out computational sequence and structural analysis on these two designed proteins .
we have performed secondary structure prediction of these two proteins and observed that the methods such as multivariate linear regression combiner can predict some regions as extended structures for the helical protein sequence ga , which gave us a clue that there may be structural plasticity at the region of first 15 residues , which are identical in the both proteins .
we also discovered some patterns in the nonidentical positions of two proteins with a rare combination of residues that are not present in any publicly available sequence databases . by analyzing the structures of the two designed proteins , we predicted nucleation sites at various positions in the sequence , which may start or terminate secondary structural elements ( helix , sheet , and coil ) .
we also observed drastic difference in the surrounding environment of nonidentical residues ( 7 out of 56 ) and difference in interaction energy . by observing the structural plasticity at the amino and carboxyl terminal of the sequences of two designed proteins and the influence of surrounding environment of each residue
, we concluded that early on during the process of folding , both proteins may choose different energetically favorable pathways to attain the different folds .
literature review of design of two proteins with high sequence identity adopting different folds sequence , dictionary of secondary structure of proteins assigned secondary structures and tertiary structures of pair of homologous heteromorphs ( the seven residues that vary in both sequences are indicated in rectangular boxes ) other researchers have characterized the folding of these two proteins using biophysical and computational experiments .
they also indicated that the final native structures of these proteins were dictated very early along the folding pathway by performing equilibrium unfolding of ga88 and gb88 , folding and unfolding kinetics and molecular dynamics simulations experiments .
concurrently , energy calculations were performed on the two designed proteins in a vacuum , which indicated that current computer modeling / simulations experiments can not explain why two highly similar sequences fold into different structures .
however , it was suggested that improved modeling / simulations tools should be developed to predict the pair of sequences with different structures , which differ , by only few residues . in a recent study , folding and unfolding kinetics experiments performed on these two designed proteins indicated a detectable residual structure in the denatured state of gb88 whereas the denatured state of ga88 is unstructured .
interestingly , they explored these two proteins by value analysis based on 132 site directed mutants and concluded that the protein 's topology is committed very early along the folding pathway . based on the above studies , we suggest that , along with the suitable protein design experiments , better theoretical models including folding simulations coupled with structure prediction and sequence search in databases can shed light on the phenomenon of protein folding and conformation switching which may ultimately lead us to understand the contribution of each amino acid in these proteins to adopt a specific fold . we identified isolates for sequencing from 29 invasive gas cases diagnosed in patients in a northern arizona hospital during january
july 2015 and randomly selected an additional 99 gas isolates from a repository of > 2,000 arizona gas isolates collected during 20022006 ( no isolates from patients in arizona were available for 20072014 ) .
four additional isolates from central arizona identified in 2015 were included in the analysis ( technical appendix table ) .
all isolates were grown on 5% sheep blood tryptic soy agar plates ( hardy diagnostics , santa maria , ca ) , and incubated at 37c with 5% co2 .
dna was extracted by using a dneasy blood and tissue kit ( qiagen , valencia , ca , usa ) following manufacturer s protocol .
genomic dna libraries were prepared by using the nextera xt library prep kit ( illumina , san diego , ca ) and sequenced with paired - end reads ( 250 bp ) on an illumina miseq instrument , as previously described ( 9 ) . the finished genome of the emm59 canadian clone mgas15252 ( genbank accession no .
cp003116 ) and high - quality publicly available sequence - read data from 44 us isolates , from ncbi short read archive ( bioproject # prjna194066 ) , were included in the subsequent phylogenetic analyses .
the final core genome ( all nucleotide loci found in all genomes ) for single - nucleotide polymorphism ( snp ) detection was 1,636,024 bp ( 98.6% of reference ) .
we used nasp snp analysis pipeline ( http://tgennorth.github.io/nasp/ ) for whole - genome snp typing as previously described ( 10 ) .
we used mega version 5.2.2 software ( 11 ) to generate maximum parsimony phylogenetic trees .
regions of high snp density were identified as possible regions of recombination and were further analyzed for impact on the consistency index .
gas emm subtypes were assigned by using blast ( http://blast.ncbi.nlm.nih.gov/blast.cgi ) , querying the study genome assemblies against the centers for disease control and prevention s ( cdc ) emm type - specific sequence database ( http://www.cdc.gov/streplab/m-proteingene-typing.html ) .
we resolved dual emm - type hits using cdc s emm typing sanger sequencing primers ( http://www.cdc.gov/streplab/protocol-emm-type.html ) as a blast query and noting hit locations .
we identified 18 of the 29 contemporary northern arizona isolates as subtype emm59 ; the remaining isolates were composed of 6 additional emm types : emm1 ( n = 2 ) , emm5 ( n = 2 ) , emm58 ( n = 1 ) , emm81 ( n = 2 ) , emm83 ( n = 1 ) , emm89 ( n = 2 ) , and emm94 ( n = 1 ) .
the 99 historical and 4 contemporary background arizona isolates included 25 distinct emm types ( technical appendix table ) .
no emm59 isolates were identified in this background set , and none had been previously reported in arizona .
an emm59-only phylogenetic analysis demonstrated the apparent presence of multiple lineages of emm59 in the 2015 arizona isolates ( figure 1 ) .
a distinct clone consisting of 14 of the 18 emm59 isolates were separated from each other by only 04 snps , genomically supporting the presence of an ongoing outbreak ; > 8 of these patients were epidemiologically linked to physical contact , cohabitation , or both with 1 other person ( data not shown ) .
the additional emm59 isolates make up additional lineages separated from one other by 828 snps .
a relatively large number of snps and indels were seen within an approximate 23-kilobase region ( figure 1 ) .
this region has been previously reported to contain mutational hotspots associated with virulence ( 12,13 ) . considering the presumptive positive selective force on this region , snps within the region
phylogenetic single - nucleotide polymorphism ( snp ) tree of emm59 isolates from a northern arizona hospital displaying distribution of mutations in a 23 kb positively selected region during invasive group a streptococcus outbreak , southwestern united states .
maximum parsimony tree of all snp loci ( n = 58 ) in emm59 isolates ( n = 18 ) from arizona , 2 recent new mexico isolate genomes , and the canadian clone reference isolate mgas15252 .
branch lengths represent numbers of snps between isolates ; unit bar is in the figure .
numbered circles distinguish lineages of selected mutations in scpa , enn , sfbl , mga , sfbx , and sof genes in a 23-kb hotspot mutational region .
when compared with all other publicly available us emm59 isolate genomes , nearly all the genomes identified in the united states were closely related to each other and to the canadian clone mgas15252 ; individual isolate snp branch lengths ranged from 0 to 10 ( figure 2 ) .
the arizona outbreak isolates were separated from 2 new mexico isolates by 4 and 5 snps each ; these isolates fell within the overall arizona clade and were subsequently included in the arizona - only phylogenetic analysis ( figure 1 ) .
conversley , the isolate from patient m appears more distant from the larger arizona population .
the arizona clades , with the exception of that of the isolate from patient m , all appear to arise from the large minnesota polytomy .
the previously estimated 1.32.1 snps / year mutation rates for gas ( 14,15 ) further support the arizona outbreak as being caused by a single clone , likely originating from new mexico and being spread over 612 months .
phylogenetic single - nucleotide polymorphism ( snp ) tree of emm59 isolates from arizona during invasive group a streptococcus outbreak in the southwestern united states , previously analyzed us emm59 isolates , and the canadian clone .
maximum parsimony tree of all 177 snp loci ( 44 parsimony informative snps ) in emm59 isolates from arizona ( n = 18 ) , minnesota ( n = 29 ) , oregon ( n = 8) , new mexico ( n = 3 ) , colorado ( n = 2 ) , and california ( n = 1 ) and the canadian clone reference isolate mgas15252 . tree has regions of recombination removed and is rooted with minnesota isolate srr11574570 .
the emm59 subtype of gas , the etiologic agent of a substantial nationwide outbreak of invasive gas in canada during 20062009 ( 4 ) , is now present in arizona , causing at least 1 outbreak of epidemiologically and genomically linked cases and several additional epidemiologically unrelated cases .
the lack of emm59 in background isolates in arizona from the previous decade , along with its low genetic diversity , suggests that emm59 emerged recently in arizona .
following the emm59 epidemic in canada , this subtype was subsequently seen in a few us states ; a retrospective analyses of the centers for disease control and prevention active bacterial core surveillance ( abcs ) system ( http://www.cdc.gov/abcs/reports-findings/survreports.pdf ) identified 40 us emm59 isolates during 20002009 ( 6 ) and an additional 67 isolates during 20102012 ( 7 ) . of note , only 5 ( of the 40 emm59 isolates from 20002009 ( 2 from minnesota , 2 from california , and 1 from oregon ) appeared to be closely related to the canadian clone ( defined by the authors as being separated by < 16 snps ) ( 6 ) ; in contrast , all of the strains from the 20102012 survey appeared to be more closely related to the canadian clone .
the more recent abcs analysis identified an increasing number of southwestern isolates , including 4 from colorado and 6 from new mexico ( 7 ) , although no outbreaks were specifically described in these states ( arizona is not included in the abcs system ) .
( 7 ) , in an analysis of 60 mn emm59 isolates from case - patients with identified race , determined that 25 ( 42% ) were from native americans ; of 5 isolates from new mexico in that study , 3 were from native americans .
given the apparent distal nature of the arizona / new mexico isolates to the minnesota population in our study , it is reasonable to propose an unidentified epidemiologic relationship between these case populations .
however , caution must be used in drawing conclusions regarding the relationships of isolates from disparate geographic regions because only limited comparable sequence data from previous emm59 studies in the united states ( 7 ) were publicly available to compare to the arizona isolates .
epidemiologic investigations , along with healthcare provider and patient education activities , are ongoing in arizona to further determine the extent of the current outbreak and the associated risk factors and to help mitigate effects and limit or prevent further spread to at - risk populations .
| in our study , we have concluded that two proteins with 88% homology choose different energetically favorable pathways in the very early stage of the folding process to attain their native folds .
subsequent reports from other investigators by performing folding and unfolding kinetics experiments concur with our findings .
we herewith discuss the key papers revealing computational and experimental analysis of two designed proteins with similar sequence distant folds .
further we suggest that the theoretical / computational analysis of protein sequences and structures along with the relevant experiments provide a better understanding of the relationship between protein sequence , folding , and structure . |
primary tumors of the trachea are rare and account for 0.1% of all malignancies . in adults ,
approximately 90% of the primary tracheal tumors are malignant , whereas in children , the majority of these tumors are benign [ 2 , 3 ] . in a retrospective study by webb et al . , 55.4% of the patients were male and 77.3% of them were smokers .
the frequency of this rare disease has been estimated to be 0.10.4% of all malignancies , with an annual incidence of 2.6 new cases per million per year .
primary tracheal tumors can arise from the respiratory epithelium , salivary glands and mesenchymal structure of the trachea .
squamous cell carcinoma ( scc ) and adenoid cystic carcinoma ( acc ) make up about 71.6% of all adult primary tracheal tumors . the remaining portion ( 28.4% )
scc occurs predominantly in men in the sixth and seventh decades , whereas acc is equally distributed between the sexes and peaks in incidence in the fourth and fifth decades .
in contrast to acc , scc ( the most common type of tracheal tumors ) has a more aggressive course .
median survival time ranges from 6 to 14 months , and tumor disappearance is dependent on whether the primary lesion is resected .
sccs spread to the lymph nodes and 1020% of the patients will have distant metastasis at presentation .
carcinoids , lymphomas , granular cell tumors and small cell carcinomas have variable prognoses ; yet they seem to behave better than sccs , adenocarcinomas or sarcomas .
the majority of the tracheal neoplasms are primary in nature , and other primary sites that can metastasize to the trachea are the lung and esophagus , the latter being the most common .
patients with surgically resectable primary tracheal tumors have a better prognosis than those with tumors that can not be resected .
preoperative radiation therapy has been attempted in some patients , yet the most compelling evidence for adjuvant radiation therapy comes from postoperative cases .
the present study reports a rare case of primary tracheal malignancy treated with surgery and adjuvant radiation therapy .
our 60-year - old diabetic and hypertensive male patient had a 1-month history of cough associated with marked respiratory distress and occasional dyspnea in the right lateral lying position .
the patient consulted a chest physician and underwent bronchoscopy , which showed a polypoid growth in the trachea extending to the larynx ( fig .
a computed tomography ( ct ) scan revealed irregular soft tissue thickening in the region of the trachea extending up to the adjacent larynx with no soft tissue calcification , and there was no definite lymphadenopathy in the cervical region and no involvement of the underlying bone . a repeat ct scan of the neck ( fig .
2 ) showed evidence of an ill - defined , irregular , nodular , infiltrating soft tissue growth arising from the posterior tracheal wall , bridging the lumen and reaching up to the anterior wall , with an infiltration of about 2.2 1.8 cm .
the location was opposite the c7-d1 level involving the 5th or 6th tracheal ring , approximately 7 cm proximal to the carina with no significant evidence of cervical and mediastinal lymphadenopathy .
excision of the tracheal growth through a cervical transverse incision was performed , and a tracheostomy tube was inserted .
soon after surgery , the cough subsided and the patient attended square hospital , dhaka , bangladesh , for postoperative irradiation .
ninety - five percent of the planning target volume ( ptv ) covered 97.3% of the prescribed dose .
the minimum and maximum dose received by the ptv was 50.05 and 63.68 gy , respectively .
3 ) . three multileaf collimator fields ( left anterior oblique , right anterior oblique and posterior ) were used for three - dimensional conformal radiation therapy ( 3dcrt ) .
complete response was documented , and the patient is still alive without any evidence of disease during 30 months of follow - up .
primary malignant tumors of the trachea are uncommon , and therefore limited data supporting a standard management are available .
the vast majority of the primary tracheal tumors in adults are malignant and most of them usually diagnosed at a later stage , which is due to a delayed presentation of specific symptoms like cough , dyspnea and hemoptysis .
it is clear that patients who can be resected have a better prognosis than those who can not , prompting the recommendation of surgical resection for most primary tracheal tumors .
all resected patients need postsurgical irradiation , regardless of tumor burden , margin status , histology or nodal status .
grillo and mathisen experienced a median survival of 34 months in scc and 118 months in acc patients who underwent a combined modality like surgery and radiation therapy .
attained a 61-month median survival using a combined surgery and radiation therapy in their study .
. showed that 73% of the patients with a 5-year survival underwent radiation therapy postoperatively .
the role of external beam radiation therapy as an adjuvant to surgical resection is better established .
grillo and mathisen advocated maximal ( 4,5006,500 cgy ) radiation therapy after scc and acc resection because of the close margins necessary for resection and the high likelihood of local recurrence .
modern ct - based 3dcrt or intensity - modulated radiation therapy theoretically allows higher and safer doses delivered to the trachea . for postoperative cases ,
all patients with this tumor in the postoperative or definitive setting should be treated with the 3dcrt technique .
an intraluminal boost technique after external beam radiation therapy may decrease the risk of late side effects .
there are many studies showing the benefit of postoperative irradiation therapy in primary tracheal malignancies such as sccs and accs .
however , little experience has been shared in the case of primary adenocarcinomas of the trachea , the tumor type described in our report .
the prognosis of patients with malignant tumors of the trachea remains gloomy , and long - term median survival of tracheal adenocarcinoma patients undergoing combined modalities is unknown to us .
primary adenocarcinoma of the trachea needs to be diagnosed at an early stage , and combined multimodal approaches may be explored to attain an extended median survival .
none of the authors has any conflicts of interest regarding the content of this article . intractable nausea and vomiting are very rare symptoms of medullary compression and there are approximately less than 10 cases reported in the literature of the aforementioned symptomatology mostly due to tumors .
there is one reported case where vertebral artery compression of the medulla led to intractable nausea and vomiting .
a 69-year - old woman presented with a 10-month history of intractable nausea and vomiting resulting in a 50 pound weight loss .
she had an extensive medical workup at multiple outside hospitals including a comprehensive gastrointestinal workup which was significant for celiac disease .
her pertinent past medical history includes a history of breast cancer , mastectomy , and cholecystectomy . during her evaluation by the gastroenterologists at our hospital ,
the diagnosis of celiac disease was confirmed , and she was also noted to have a duodenal ulcer .
hematologic and biochemical workup was only significant for anemia attributable to her ulcer and celiac disease .
magnetic resonance imaging / angiography ( mri / mra ) of the brain and neck , and diagnostic four vessels cerebral angiogram were obtained to further evaluate her dizziness and double vision .
the left vertebral artery angiogram revealed a tortuous left vertebral artery with a 9.6 5.6 mm dissecting aneurysm in the v3 segment .
this , along with dolichoectasia of the vertebro - basilar arteries , resulted in compression of the medulla oblongata , which was also confirmed in the contrasted mri / mra of the brain and neck [ figure 1 ] . computed tomography angiography ( cta ) also confirmed that the vertebral artery was pushing the medulla medially [ figure 2 ] .
the patient was offered a microvascular decompression ( mvd ) of the vertebral artery to attempt to decompress the brainstem and alleviate her intractable nausea and vomiting .
preoperative magnetic resonance imaging ( mri ) t2 sequenceshowing compression of the medulla by the vertebral artery preoperative computed tomography angiography ( cta ) showing a tortuous left vertebral artery crossing the midline a left retrosigmoid craniotomy was completed to approach the vertebral artery and lower brainstem . the elongated , ectatic vertebral artery
additionally , indentation of the lower part of the medulla by the vertebral artery was also recognized .
apericranial graft was used as a sling and tacked to the dura , to decompress the brainstem .
the postoperative course was uneventful , and the patient was kept in the hospital to transition her from gastrostomy tube feedings to regular oral feeding .
postoperative cta demonstrated that the vertebral artery had been mobilized [ figure 3 ] away from the medulla .
postoperative cta showing left vertebral artery away from the medulla the patient was discharged from the hospital without any antiemetic medication and was documented to have started gaining weight . at her 2-year postoperative visit , the patient had a nonfocal neurologic exam without recurrence of her prior nausea or vomiting .
patients with central lesions often wait months or years and undergo countless tests before a central etiology is added to the differential diagnosis because most cases do not present with localizing neurological deficits
. sustained hiccups with vomiting or isolated spontaneous vomiting with negative gastrointestinal symptoms should prompt further workup , including neuroimaging , to search for a central cause .
the patient in our case was incidentally found to have dizziness and diplopia during the initial workup , but was not demonstrating positional vomiting or headaches , which would have prompted a neurological workup sooner . the tortuous vertebral artery in this case
was found on the diagnostic mri / mra of the head and neck obtained secondary to her history of breast cancer and new onset symptoms of diplopia and dizziness . without a proper neurological examination
this patient 's celiac disease and gastrointestinal reflux would have continued to mask the true cause of her symptoms . while compression of the medulla by a dilated vertebral artery is much less common than other brainstem compression syndromes such as trigeminal and glossopharyngeal neuralgias ; it is still significant .
vascular compression of the medulla can cause disabling positional vertigo , hypertension , and hemifacial spasm ; but there are only a few reports of intractable nausea and vomiting as a symptom of an ectatic vertebral artery .
less than 10 cases of nausea and vomiting due to vertebral artery compression have been reported in the literature .
of the 20 cases of vertebral artery compression of the medulla reported in a meta - analysis by savitz et al .
, only one patient experienced nausea and vomiting as a symptom . the majority of patients presented with hemiparesis and cranial nerve dysfunction .
mvd was the choice of treatment for 17 of the patients , including the patient with symptoms of nausea , and it was shown to relieve symptoms in 16 patients .
medullary compression can produce a wide spectrum of signs and symptoms from very few to several .
regardless of the etiology , compressive forces on the neuronal tracts and nuclei that lie within the medulla can produce a number of clinical scenarios .
when the etiology is vascular in origin , ischemic injury can produce symptoms such as headache , transient ischemic attacks ( tias ) , or infracts depending on the location of the compression . at the floor of the rhomboid fossa
it contains specialized cells consisting of ependymal cells and tanycytes that allow for direct communication of cerebro spinal fluid and blood because it lacks an intact blood brain barrier . as a circumventricular organ ,
the area postrema is able to detect toxins and drugs in the blood as well as hormones and other humoral signals to help maintain autonomic homeostasis .
stimulation of the area postrema by vagal afferents from the nucleus ambiguous and gastrointestinal system or from emetogenic drugs and cytokines in the blood may trigger vomiting .
the nucleus tractus solitarius ( nts ) lies adjacent to the area postrema , serves as a relay center to collect all afferent signals and activate the appropriate visceral nuclei to coordinate the action of vomiting .
efferent pathways from the area postrema and nts project to the central pattern generator , ventral medulla , and hypothalamus .
the central pattern generator is proposed to coordinate the activation of these nuclei within the medulla .
the efferent signals stimulate the appropriate parasympathetic and sympathetic neurons , which produce the different phases of vomiting .
the signal also diffuses through the brain via microglial messengers and cytokines such as substance p to allow for the cognitive recognition of emesis . a lesion to any of the afferent relay tracts or emetic reflex center proper may produce an abnormal emetic reflex and could produce intractable nausea and vomiting , as was observed in our case . on the preoperative mri ,
the vertebral artery was seen to be compressing the medulla , where the area postrema is located , and nts at the inferior and posterior limit of the fourth ventricle .
the potential disruption of the area postrema in this case may have caused an overstimulation of the emetic reflex .
the first mvd was performed in 1966 by dr peter jannetta to relieve the facial pain in a patient with a compressed trigeminal nerve .
it is now used to treat compression of the facial and glossopharyngeal nerves , as well as vascular abnormalities .
the indications of mvd could potentially be expanded to treat compressive symptomology ; including , for example , intractable vomiting due to vascular compression of the medulla as demonstrated in this case report .
this case demonstrated the importance of neurological investigations in the isolated intractable vomiting patient without a clear evidence of peripheral cause and including a central nervous system etiology on the differential , and the role of mvd in achieving cure . | primary adenocarcinoma of the trachea is extremely rare and a standard treatment does not exist due to nonavailability of evidence - based randomized control studies . this paper reports the case of a 60-year - old male , who presented with cough and occasional respiratory distress .
bronchoscopic examination and a computed tomography scan revealed a soft tissue mass in the trachea arising from the posterior tracheal wall .
cytological examination and immunochemistry confirmed primary adenocarcinoma of the trachea .
excision of the tumor followed by three - dimensional conformal radiation therapy was performed , and a dose of 56 gy was delivered to the primary site .
two and a half years after treatment , the patient has no clinical or radiological evidence of the disease , and no late complication has occurred . |
in modern society , the fashion of wearing tight clothes for stylish dressed states or
comeliness is getting very popular1 .
however , specialists reported that excessively pressing certain areas of the human body
could cause many problems in the cardiovascular systems and visceral organs1 .
in addition to this pressure inflicted on
the human body may deform muscles , the skeletal system , and even the overall body type2 , 3 .
jeans that are too tight compress a nerve that cuts off sensation to the thighs , and this is
consistent with human anatomy and physiology4 .
trousers that are too tight can squeeze a sensory nerve under the
hip bone , causing a tingling , burning sensation called paresthesia1 , 4 , 5 . with this as the background , the present study aimed to understand
the hazards of the habit of wearing tightly fitting clothes in relation to deformations of
the musculoskeletal system and the movement of the lumbar spine and pelvis for the purpose
of providing basic data regarding proper habits in wearing clothes for the prevention of
pain in the musculoskeletal systems2 , 3 .
so the present study evaluated the effect
of wearing tight pants on the trunk flexion and pelvic tilting angles in stand - to - sit
movement and in a seated posture .
this study was performed on nine males aged 2027 years ( 23.22.0 years , meansd ) whose
height and weight were 175.13.2 cm and 62.13.4 kg , respectively .
subjects with conditions
that might affect trunk mobility , such as injury or neurologic deficits of the hip and lower
extremities during the past year , were excluded from study . the study purpose and methods
were explained to all the subjects , who provided informed consent according to the
principles of the declaration of helsinki before participating .
data were collected at a sampling rate of 100 hz with a motion capture system
( vicon mx , oxford metrics , oxford , uk ) that consisted of eight infrared cameras .
sixteen reflective markers were
attached to the lower body according to the plug - in - gait marker set ( oxford metrics ) using
double - sided tape .
the software used for kinematic data collection was nexus 1.4.1 ( oxford
metrics ) , and the data were analyzed with the polygon 3.1 software ( oxford metrics ) .
the
experimental protocol required the completion of two stand - to - sit trials for each of the two
pants conditions .
the worn pants were made from the same material , cotton , woven into a
rugged cotton textile .
we used general and tight pants from the same company ( g company ) .
the pants were worn under two conditions in this study : ( 1 ) general pants , worn with sizes
equivalent to 105110% of the subjects hip , thigh , and calf circumferences , and ( 2 ) tight
pants , worn with sizes equivalent to 9095% of the subjects hip , thigh , and calf
circumferences .
each subject was asked to stand up at a
self - selected speed from a seated posture and to stand in an erect spine posture . for time
normalization , the time required for a complete stand - to - sit movement cycle ,
that is , from
movement onset to completion , was considered to be 100% ; values were determined for each 2%
of the movement , beginning at 0% .
the changes in trunk flexion and pelvic posterior tilting
angles were calculated based on the difference between the maximal and initial angles . as
the analysis was performed with a withwn - subject design ,
the paired t - test was conducted to
test for differences in pelvic and trunk kinematics values during the maneuver .
all
significance levels were set at p<0.05 , and spss version 12.0 ( spss , chicago , il , usa )
was used for statistical analyses .
the change in the posterior pelvic tilting angle ( 9.3 4.1 ) during the stand - to - sit
movement when wearing the tight pants increased significantly compared with that when
wearing the general pants ( 7.0 3.3 ) ( p<0.05 ) .
the change of the trunk flexion angle
( 19.3 5.4 ) during the stand - to - sit movement when wearing the tight pants increased
significantly compared with that when wearing the general pants ( 12.0 3.8 ) ( p<0.05 ) .
the change in the posterior pelvic tilting angle ( 13.3 3.2 ) in the seated posture when
wearing the tight pants increased significantly compared with that when wearing of the
general pants ( 10.3 2.9 ) ( p<0.05 ) .
the change in the trunk flexion angle ( 25.3 6.1 )
in the seated posture when wearing the tight pants increased significantly compared with
that when wearing of the general pants ( 20.1 5.4 ) ( p<0.05 ) .
park and yoo reported that the tightness of a waist belt might restricts forward movement
of the center of mass and that pelvic inclination might be increased as a compensatory
mechanism2 .
they showed that the wearing
a tight belt could interrupt normal lumbo - pelvic coordination , which might contribute to
muscle imbalance2 .
elevated abdominal
pressure has been shown to cause multidirectional stiffness of the spine5 , 6 .
a
previous study that used a wide belt reported that the intramuscular pressure on the erector
spinae could influence spinal stiffness separately from muscle7 .
the results of the present study showed that the trunk flexion and
posterior pelvic tilting angles during the stand - to - sit movement when wearing tight pants
significantly increased when compared with wearing general pants .
also , the trunk flexion
and posterior pelvic tilting angles in the seated posture when wearing tight pants
significantly increased when compared with those when wearing of general pants .
the lumbar
and hip rhythm and interaction between the lumbar spine and hip are important kinematic
factors that are used not only in experimental research but also in clinical
examination8 , 9 . wearing tight jeans induced excessive lumbar flexion during stand
to sit movement and in the seated posture .
the exaggerated lumbar flexion may overstretch
posterior connective tissues , such as the interspinous ligament , apophyseal joint capsule ,
and thoracolumbar fascia , or increase stress on discs and apophyseal joints5 .
posterior pelvic tilt decreases lordosis
via flexion of the lumbar spine , causes posterior movement of the nucleus pulposus , and
increases the diameter of the intervertebral foramina5 , 8 .
slump sitting reduces the
activation of the spinal stabilizing muscles and is associated with increases in loading on
the intervertebral disc and connective tissue8 , 9 .
therefore , wearing tight pants could
produce musculoskeletal disorders via abnormal movement and posture in the lumbar spine and
pelvis .
so the effects of wearing tight pants need to be investigated in further studies to
reveal their direct relationship to musculoskeletal problems .
the effects of tight clothes
and accessory items on the human body should also be investigated in order to provide basic
data for proper use .
further studies will become the starting point for studies regarding
the effects of wearing tight clothes and tight fashion accessories on the musculoskeletal
system . a retrospective chart review was performed for cases of infectious scleritis seen from january 2007 to august 2011 .
the diagnostic criteria for a case of infectious scleritis included the presence of single or multiple inflamed anterior scleral nodules or ulcerations , which revealed a causative organism on microbiological culture .
microbial cultures were considered significant if growth of the same organism was present on more than one solid medium or if there was a confluent growth on inoculation on one solid medium or if growth of one medium was consistent with direct microscopy findings .
data regarding patient demographics , predisposing factors , causative organisms , and clinical features for all 12 patients was extracted .
all patients except one were followed up for a period of at least 6 months and , therefore , the treatment and outcome details were analyzed for 11 patients .
data was analyzed by the chi - square test using spss version 11.0.1 to determine the prognostic factors .
twelve eyes of 12 patients were identified to have culture - positive infectious scleritis . demographic data and clinical features
preceding surgical procedures performed included manual small - incision cataract surgery ( 4 ) , pterygium excision ( 1 ) , and trabeculectomy ( 1 ) .
none of the patients had a history of fever , joint pain , respiratory illness , skin rashes , etc .
, one patient ( case 2 ) had progeria , another had chronic liver disease ( case 6 ) , and 2 patients had diabetes ( case 8 and 10 ) .
patients with multifocal scleritis were associated with growth of either gram - negative bacilli ( gnb ) or nocardia on culture an example of which is shown in fig .
2a and b. demographics , clinical features , organisms , management , and outcomes of infectious scleritis ( a ) multifocal scleral abscesses owing to pseudomonas sp .
( b ) healed scleritis with minimal scleral thinning 2 months after treatment with topical fortified antibiotics and dexamethasone ( case 11 ) ( a ) nocardia asteroides : gram stain showing filamentous forms fragmented into bacillary and coccoid forms ( magnification = 1000 ) .
( b ) nocardia asteroides : orange coloured , centrally heaped , dry colony all patients received topical antimicrobials based on the culture and sensitivity report .
seven patients were also treated with topical steroids due to severe intra - ocular inflammation .
in addition to medical treatment , 5 patients underwent various surgical procedures in an attempt to decrease the load of infective organism or to preserve the globe as shown in table 1 and fig .
of these , only 2 patients had a good final visual acuity ( better than 6/18 ) on the last follow - up visit .
six months after corneo - scleral patch graft following infectious scleritis post - cataract surgery ( case 8) we determined the prognostic factors of infectious scleritis using the chi - square test .
a poor visual acuity at presentation ( less than counting fingers at 1 m ) usually resulted in a worse visual outcome ( p = 0.005 ) .
the causes of poor visual acuity at last follow - up include corneal scarring following keratitis ( 1 ) , failed corneal graft ( 1 ) , secondary angle closure glaucoma and optic atrophy ( 1 ) , choroidal detachment ( 1 ) , and endophthalmitis ( 5 ) .
the visual outcome was not dependant on any particular organism ( p = 0.099 ) or the presence of multifocal abscesses ( p = 0.209 ) .
the addition of surgical intervention did not result in a significantly better visual outcome than medical management alone ( p = 0.209 ) , but resulted in a higher rate of globe preservation ( p = 0.045 ) .
a clinical suspicion of infectious scleritis in all cases of scleral inflammation following trauma has been emphasized previously .
however , in our series , 4 cases were preceded by cataract surgery and only 1 by pterygium surgery .
another case series of indian eyes also showed maximum number of their cases had cataract surgery prior to an episode of infectious scleritis .
this may be due to the large number of scleral - tunnel small incision cataract surgeries performed in india as compared to clear corneal phacoemulsification performed in developed countries .
pseudomonas aeruginosa is the leading cause of microbial scleritis in reported literature . in our series , 33.33% had gnb , 25% had nocardia , and 25% had fungal infection .
one of the fungi was identified to be rhodotorula spp , which we have reported elsewhere . in cases of scleritis following trauma
where infection is suspected but routine microbial culture is negative , polymerase chain reaction tests may be used to confirm the diagnosis .
if these tests are also negative , the possible diagnosis of surgically induced necrotizing scleritis ( sins ) should be considered .
sins is a believed to be a delayed - type hypersensitivity response to any form of surgical trauma . up to 90% of patients who develop sins
may have an undiagnosed systemic vasculitis and , therefore , a detailed systemic investigation is warranted .
this is often seen as late as 9 months after the surgical intervention and , after ruling out an infectious cause , requires treatment with intensive systemic immunosuppression .
when infectious scleritis occurs long after surgery , it has been hypothesized to be due to an underlying sins with secondary microbial infection , which can make diagnosis and treatment difficult .
these patients may benefit from a systemic evaluation to rule out an autoimmune vasculitis , which will require appropriate management .
some presented with scleritis and developed secondary intraocular spread , which has been described in other reports .
others had scleritis , uveitis , and endophthalmitis at presentation ( e.g. , case 7 where the sclera in the area of the bleb was necrotic with vitreous inflammation ) . the wide use of anti - metabolites at the time of trabeculectomy increases risk of scleral melt and inflammation , which may have resulted in secondary infection and endophthalmitis .
pseudomonas is most often associated with this presentation , which has been attributed to an intra - scleral dissemination facilitated by its proteolytic products and motility .
our series had 6 cases of multifocal scleral abscesses , 3 of which were due to p. aeruginosa , 2 due to nocardia , and 1 due to klebsiella sp .
a review of past literature suggests that multifocal scleritis can be caused by several organisms and may be related to factors such as the load of infection , rather than specific organisms .
the visual acuity at presentation is the most important prognostic factor determining the final visual acuity , and this has been seen in other studies .
this suggests that early diagnosis should help preserve the patient 's remaining vision if effective treatment is initiated .
there is a lot of discrepancy among researchers regarding the effect of surgical debridement on the outcome of infectious scleritis .
a large series showed that despite adjuvant surgery in 46 of 56 eyes , approximately 50% lost functional vision .
however , unlike other studies , we did not find any difference in the final vision between the medically and surgically treated groups . in conclusion ,
manual small incision cataract surgery is a common predisposing factor to infectious scleritis in developing countries .
the visual acuity at presentation is the most important prognostic factor determining the final visual outcome .
aggressive surgical intervention improves the chance of globe preservation but does not improve the visual outcome . | [ purpose ] the purpose of this study was to evaluate the effect of wearing the tight pants
on the trunk flexion and pelvic tilting angles in the stand - to - sit movement and a seated
posture .
[ subjects ] nine male subjects were recruited .
[ methods ] the trunk flexion angle
and pelvic posterior tilting angle were measured using a motion - capture system during the
stand - to - sit movement and in a seated posture . [ results ] the trunk flexion and the
posterior pelvic tilting angles during the stand - to - sit movement and in the seated posture
when wearing tight pants significantly increased compared with those when wearing of
general pants .
[ conclusion ] therefore , wearing tight pants could produce musculoskeletal
disorders via abnormal movement and posture in the lumbar spine and pelvis .
so the effects
of wearing tight pants need to be investigated in further studies to reveal their direct
relationship to musculoskeletal problems . |
lipoma is a common benign soft tissue neoplasm that sometimes may have mixed tissue components .
ossification of a lipoma was first described in 1959 , and it is rarely reported7,16 ) .
several names have been used to describe ossification of lipomas , including secondary calcification , ossified lipoma , ossifying lipoma , or osteolipoma , and some authors have used these terms interchangeably . as with classic lipomas , lipomas with ossifications may be found in any part of the body , but are usually found in the head , neck , oral cavity , and extremities , adjacent to bone .
only two cases of osteolipoma arising in connection with vertebrae have been described . here ,
we present a case of an intramuscular osteolipoma on the posterior neck , independent of vertebrae , and review the clinicopathologic features of osteolipoma previously published in the literature .
a 51-year - old female presented with a 5-year history of a painless , progressively enlarging mass on the posterior neck .
physical examination revealed a soft , non - tender , uniform mass without surface abnormalities . computed tomography ( ct ) and magnetic resonance imaging ( mri ) showed a circumscribed mass involving the posterior neck muscles in the midline between the c2 and c6 spinous processes with a large calcified component ( fig .
the ovoid mass was measured 4 cm in width , 6 cm in length , and 3 cm in height , without infiltration of the cervical spine .
subsequently , the patient underwent a whole body bone scan , which revealed an amorphous calcification of the posterior neck without metastasis ( fig .
the mass had a well - demarcated margin and the outer wall was very firm without adhesion to ligaments or paravertebral muscles .
gross examination revealed a well - circumscribed mass composed of adipose and bony tissue , with red bone marrow visible on the cut surface ( fig .
3 ) . histological study showed a lipomatous lesion with large foci of osseous metaplasia at the periphery of the mass ; the bony portion was surrounded by mature adipose tissue ( fig .
microscopic examination of the red bone marrow showed a meshwork of bone trabeculae and hematopoietic marrow elements ( fig .
the patient was diagnosed with an intramuscular osteolipoma of the posterior neck and no recurrence was observed at the 6-month follow - up .
a lipoma is a common soft tissue neoplasm , but lipomas with distinct osseous components are rare2 - 4,8,10 - 12,14,15 ) . as noted by heffernan et al.11 ) , in allen 's review of 635 cases , less than 1% of lipomas were ossified .
further , as reported by de castro , et al.5 ) , when furlong analyzed histological findings from a group of 125 lipomas of the oral and maxillofacial region , there was no variant that presented as osseous metaplasia .
lipomas presenting with osseous components have been reported as secondary calcification of lipoma or ossified lipoma4,12,14 ) .
secondary calcification of lipoma involves calcification that progresses from the periphery inwards as a result of necrosis after impaired blood supply or repeated microtrauma5,14 ) .
an ossified lipoma is defined as a histologic variant of lipoma that has undergone osseous metaplasia , and can be classified into 2 groups according to the predominance of the osseous component : ossifying lipoma and osteolipoma .
if the adipose component of the lipoma is more dominant than the osseous component , it is an ossifying lipoma , whereas an osteolipoma presents with a dominant osseous component14 ) .
osteolipomas have been reported in middle aged or elderly patients presenting with large painless masses with a long duration , even years , and they may be found incidentally2,7 ) .
they have been reported at various sites located adjacent to bone or periosteum , including upper and lower extremity , oral mucosa , soft tissue of neck , and intracranial regions , including tuber cinereum , hypothalamus , suprasellar cistern , and the interhemispheric area1 - 5,7,10 - 15,17,18 ) .
one patient was an 8-year - old female who presented with a progressively enlarging swelling over the lumbar region with a dermal sinus tract12 ) , and other was 20-year - old female with a cervical osteolipoma contained within the spinal canal14 ) .
presented here , the osseous lipoma was located in the deep neck musculature , independent of bony vertebral structures .
there are several hypotheses regarding the pathogenetic mechanisms that influence the osseous metaplasia of lipomas , but the details are still not clear .
first , these tumours may originate from multipotent mesenchymal cells that can differentiate into lipoblasts , chondroblasts , fibroblasts , angioblasts , and osteoblasts .
this could account for the several lipomatous variations of classic lipomas , i.e. lipoblastoma , lipomatosis , fibrolipoma , angiolipoma , myolipoma , myelolipoma , hibernoma , and atypical lipoma , and mesenchymoma is defined as a rare soft tissue lesion that is composed of fibrous tissue associated with two or more types of well differentiated mesenchymal cells that would not normally be found in the same area 2,7 ) .
second , ossification may also have been induced by poor nutritional supply in the centre of a large lipoma after repetitive trauma , metabolic changes , or ischemia leading to transformation of fibroblasts into osteoblasts2,8 ) .
fritchie et al.8 ) reported cytogenetic analyses of three osteolipomas and reported that the translocations in all cases were consistent with the karyotypic features of lipoma .
other benign tumors that may contain bone including teratomas or dermoids , masses with secondary ossification due to trauma , liposarcomas with metaplastic changes , or congenital malformations , should be considered in the differential diagnosis .
in addition , tumour calcinosis , calcification in a bursa , and other conditions such as ossifying fibromas , myositis ossificans , and osteosarcomas should be taken into consideration .
the use of ct scanning provides excellent visualization of the calcified or ossified components of a lipoma and confirmation of proximity to adjacent bone , and mr imaging can also provide detailed information that is useful for further evaluation2,9 ) .
definitive diagnosis of osteolipoma can be made with histopathologic findings after surgical excision , which is usually the recommended treatment . a histopathologic appearance of diffuse , mature ossification within adipose tissue and gross features demonstrating a dominant osseous component confirm the diagnosis .
histologically confirmed osteolipomas are benign neoplasms , as with classic lipomas , and do not recur2,4 - 6,11 ) .
to the best of our knowledge , it is the first case report of an intramuscular osteolipoma within the posterior neck independent of adjacent bone .
we suggest that although osteolipoma is a rare variant , it is important to keep it in mind when a soft tissue mass of the posterior neck mixed with osseous component is encountered . immunocompromised patients might bear a variety of presentations such as rhino - orbito - cerebral , pulmonary , gastrointestinal , cutaneous , central nervous system and disseminated form .
rhino - orbito - cerebral and pulmonary mucormycosis are the most common forms ; the latter progresses rapidly to devastating diseases .
presentation of pulmonary mucormycosis may be confused with several infectious and non infectious diseases ; therefore its diagnosis is difficult .
chest ct scan of a patient with pulmonary mucormycosis might demonstrate such presentations as air crescent sign , ct halo sign , solitary or multiple pulmonary nodules or masses , bronchopulmonary fistula and pulmonary artery pseudo aneurysm .
the occurrence of pneumothorax and its subsequent rhs along with sinusitis , supported by radiology and pathology in a 33 years old patient , is considered as a rare case of pulmonary mucormycosis .
following is the treatment history of a man , 33 years old in july 2014 ( day + 14 ) , when he died in our hospital as a result of multiorgan failure .
he was a farmer , married , with no history of alcohol consumption , illicit drugs use and extramarital relationships . on day 330 : because of edema he visited a medical center in another city , where he was admitted and membranous glomerulonephritis was diagnosed .
consequently the followings administered ; prednisolone and cyclosporine for 2 months , cellcept for 1 month , prednisolone and cyclophosphomide for 7 months . on day 75 : because of progressive generalized edema he was admitted again .
he was discharged under treatment with prednisolone ( 50 mg / daily ) and tacrolymus ( 1 cap / bd ) . during the next 2 months he was feeling fine . on day 28 , he travelled to village for a few days and he had close contact with sheep . on day
14 the patient reported fever , cough and dyspnea with mild erythema and pain in right eye .
anorexia , weakness , dyspnea and conjunctivitis developed and persisted despite of outpatient antibiotic treatment . in the emergency department (
day 0 ) : the patient suffered from dyspnea , weakness and right eye pain . on examination , he was agitated but completely conscious with diaphoretic skin .
the vital signs have been as follows ; temperature , 36.7 c ; blood pressure , 100/60 mmgh ; pulse rate , 99 beats per minute ; respiratory rate , 22 beats per minute and oxygen saturation in air room , 99% .
in right eye , there was moderate conjunctivitis with eyelid erythema ; its movement was normal .
the first day chest ct scan revealed large pleural effusion with pneumothorax in the left side and a shift of mediastinum to the right side .
1 ) . consequently drainage with chest tube was administered by the surgeon immediately . because of the immunocompromised state of the patient , parenteral trimethoprim- sulfamethaxazol ( 160 mg / iv / tds ) and meropenem ( 1 g / iv / bd ) were administered for pneumocystis jiroveci and multi drug resistant organisms .
we also started fluconazole for oral lesions . on day + 3 , right eye erythema was extended with small size necrosis in right nasal ala without bleeding ( fig .
with doubt of rhino - orbital mucormycosis , biopsies of tongue and nasal septa were carried out and amphotericin b deoxycholate ( 1 mg / kg / daily ) was administered . on day + 4
, paranasal sinuses ( pns ) ct scan revealed soft tissue density lesion in left ethmoid sinus and nasal passage with extension to left orbit ( fig .
, he was transferred to intensive care unit ( icu ) because of respiratory distress and was intubated after 48 h ; his conscious level was 14+t/15 .
smear of sputum for acid fast bacilli , stain and culture of sputum for pneumocystis jiroveci were negative .
hiv antibody test , d - dimer enzyme -linked immunosorbent assay ( elisa ) and collagen vascular diseases screening tests were negative ( table 1 ) .
pleural fluid cytologic examination and polymerase chain reaction ( pcr ) for fungi were negative . on day + 6 , laboratory results revealed pancytopenia ( table 1 ) , however in peripheral blood smear there was no schistocyte or immature cells .
bone marrow biopsy which could help in finding the cause of pancytopenia was nevertheless postponed due to the patient 's poor condition and low platelet count .
meanwhile his blood pressure was decreased and we started inotropic drugs.hemodynamic instability of the patient prohibited further attempts such as bronchoscopy and ct guided lung biopsy to identify the causative agent . on day + 9 ,
section of tounge biopsy showed ulcerated squamous epithelial mucosa with subepithelial fibro fatty tissue and skeletal muscle fibers of tongue that is on site of ulceration .
the inflammatory granulation tissue and inflammatory multinucleated giant cells were also seen in gimsa staining , but no cyst and trophozoite of pneumocystis jiroveci were identified .
pulmonary mucormycosis is a devastating infection when it affects immunocompromised patients such as diabetes mellitus , hematologic malignancies , chronic renal failure , post transplantaion and immunosuppressed persons . herein , we present a rhino - orbital mucormycosis with pulmonary involvement .
reticulation inside the rhs , presence of pleural effusion , multiple nodules and concomitant rhino - orbital mucormycosis as well as documented pathology , collectively were considered as indicating that pulmonary involvement has been due to mucormycosis .
the pathological finding is achieved by tongue tissue ; lung biopsy has not been possible due to poor condition of the patient .
our case was an immunocompromised patient with documented rhino - orbital mucormycosis ( figs . 3 , 5 and 6 ) .
chest ct scan showed bilateral pleural effusion , multiple nodules and rhs with thin reticulation ( figs . 1 and 4 ) .
although histopathology findings are usually needed to establish the diagnosis of pulmonary mucormycosis , using ct imaging and clinical presentation we can avoid the invasive procedures such as lung biopsy
. there are few case reports of pneumothorax in pulmonary mucormycosis , some of which are iatrogenic during mechanical ventilation and bronchoscopic procedure [ 911 ] .
combined mucormycosis and methicillin - resistant staphylococcus aureus ( mrsa ) were found to be responsible for life threatening pneumothorax in an immunocompromised patient .
mucormycosis and aspergillus have been recognized as leading to pneumonia and pneunothorax in a dialysis patient .
however , spontaneous pneumothorax in pulmonary mucormycosis that is followed by rhs seems to be a unique case . based on patient 's background and clinical signs , amphotericin b deoxycholate ( 1 mg
the lower dose of antifungal drug has been due to the patient 's renal insufficiency .
ambisome would have certainly been a better choice than amphotericin b ; however , the former has not been available in iran .
however the lack of bone erosion in sinus ct scan , delay in pathologic result and patient 's poor clinical condition , lead into the reluctance of surgeon for extensive debridement that had negative impact on patient 's prognosis .
pneumothorax which is due to mucormycosis can be fatal particularly in immunocompromised persons , so clinicians should take immediate measures ( follow up imaging and pathology ) to confirm or reject the presence of invasive fungal infection . if confirmed , antifungal therapy is necessary as soon as possible .
| ossified lipoma or osteolipoma are rarely reported .
it is defined as a histologic variant of lipoma that has undergone osseous metaplasia .
osteolipoma presents with a dominant osseous component within a lipoma .
we report a case of a histologically confirmed osteolipoma on the nuchal ligament independent of bone . the patient was a 51-year - old female who presented with a 5-year history of a painless , progressively enlarging mass on the posterior neck .
computed tomography and magnetic resonance imaging showed a circumscribed mass compatible with fat between the c2 and c6 spinous processes with a large calcified irregular component .
the mass with dual components was totally removed under general anesthesia and no recurrence was observed after 6 months of follow - up .
we also reviewed the clinicopathologic features of previously reported osteolipomas in the literature and suggest that although osteolipoma is a rare variant of lipoma , it should be considered in the differential diagnosis when a lipoma of the posterior neck mixed with a bony component is encountered . |
due to industrial development and the consequent increase in daily comfort , physical
activities have gradually decreased , leading to increased body weight with reduced physical
strength , as well as more sedentary lifestyles and significantly decreased amounts of
exercise . due to incorrect exercise habits , even among those who exercise regularly , bad
posture over extended periods , and inappropriate daily life and work habits ,
the resulting continuous back tension has increased the
number of patients with low back pain1 .
mennell2 presented the term joint
dysfunction for arthrokinematic dysfunction in the absence of pathological changes in the
joints , including capsules and ligaments .
he also attributed muscular pain and muscle spasm
to difficulties with normal arthrokinematic mobilization in joint capsules , which limit
joint movement when patients attempted to move joints suffering from the symptoms of joint
dysfunction .
joint mobilization can be performed to achieve a neurophysiological effect to
reduce muscle pain and guarding , and a mechanical effect such as stretch or burst of
contracted tissues .
one study reported increased active exercise by patients as a result of
joint mobilization3 .
the physiological effects of joint mobilization , which is aimed at increasing the range of
joint motion and pain reduction , can be explained by the gate control theory proposed by
melzack and wall4 .
the vicious cycle of
muscle pain and spasm can be broken by closing the gate where the pain stimulus is largely
transmitted through thin filaments , which have slow stimulus conduction velocity , while
proprioceptive neurons of thick filaments are stimulated .
the present study aimed to determine the effect of an 8-week program of joint mobilization
on changes in pelvic obliquity and pain level in seventeen female university students aged
in their 20 s with sacroiliac joint dysfunction by dividing them into two groups : a joint
mobilization group , and a control group .
seventeen subjects were selected from female university students aged in their 20 s
attending n university in cheon - an city , korea .
the subjects had sacroiliac joint syndrome ,
but experienced no problems with daily living and had no previous experience of joint
mobilization exercise .
the subjects were randomly assigned to a joint mobilization group of
eight , and a control group of nine who performed joint mobilization exercise . the mobilization with movement ( mwm ) group was 21.131.46 years old , 158.596.91 cm tall ,
and 59.5912.93 kg in weight . the control group was 23.202.15 years old ,
inbody 720 ( biospace , korea ) was used to measure the subjects body composition while
4d - formetric ( germany ) was used for pelvic analysis .
body fat and lean body mass were measured using inbody 7.0 ( biospace , korea ) . the direct
segmental multi - frequency bioelectrical impedance analysis method ( dsm - bia ) was used for
body composition measurement .
a pressure footstool ( pedoscan , diers , germany ) and a trunk measurement system ( formetric
4d , diers , germany ) , a 3d image processing apparatus with high resolution for the vertebrae
used in the studies of lippold and colleagues5 and schroder6 ,
were used to measure 3d trunk images of vertebrae and pelvis obliquity , as well as static
balance ability .
the posterior innominate and anterior innominate methods were used for joint mobilization
( mobilization with movement ) .
these two methods have been well described in the literature
by mulligan7 , both procedures were
repeated 10 times for one set , and for three sets ( figs
1 and 2fig .
posterior innominate of mwm anterior innominate of mwm spss pc for windows ( version 18.0 ) was used for data processing . in order to analyze the
inter - group effect , two - way anova with repeated measures
if a main effect was
found , the paired t - test was conducted to test the difference between the groups , and
one - way anova was conducted to determine the difference of each parameter between the
groups .
all the subjects understood the purpose of this study and provided their written informed
consent prior to their participation in the study in accordance with the ethical principles
of the declaration of helsinki .
the pelvic obliquity dl - dr decreased by 3.25 from 4.88 before the intervention to 1.63
after the mobilization exercise in the control mwm group , but increased by 0.22 from 1.89
to 2.11 in the control group .
the pelvic torsion l - r decreased by 0.5 from 2.50 to 2.00 in the mwm group , but
increased by 0.66 from 1.56 to 2.22 in the control group .
the trunk length increased by 8.38 mm from 440.25 mm to 448.63 mm in the mwm group , but
decreased by 1.89 mm from 451.67 mm to 449.78 mm in the control group ( table 1)table 1 .
change of pelvis malpositiongrouppre - testpost - testpelvic obliquity dl - dr ( )mwm4.882.481.631.92**control1.891.692.111.76pelvic l - r torsion ( )mwm2.500.762.000.53*control1.561.012.221.20**trunk length ( mm)mwm440.2517.62448.6315.62*control451.6716.82449.7815.93***p<0.05 , * * p < 0.01 : paired t - test . # # p<0.01 : independent t - test . * p<0.05 , * * p < 0.01 : paired t - test .
# # p<0.01 : independent t - test the mean velocity decreased by 0.28 mm from 1.26 mm to 0.98 mm in the mwm group , but
increased by 0.1 mm from 1.13 mm to 1.23 mm in the control group .
the sway area decreased by 0.14 cm from 0.21 cm to
0.07 cm in the mwm group , but increased by 0.07 cm from
009 cm to 0.16 cm in the control group . the mean frequency decreased by 0.42 hz from 0.97hzto 0.55 hz in the mwm group , but was
unchanged at 0.66 hz in the control group ( table
2table 2 .
change of static stabilitygrouppre - testpost - testmean velocity ( cm / s)mwm1.260.270.980.12*control1.130.131.230.14sway area ( cm)mwm0.210.220.070.03control0.090.040.160.11mean frequency ( hz)mwm0.970.520.550.28*control0.660.260.660.18*p<0.05 , * * p < 0.01 : paired t - test . # # p<0.05 : independent t - test ) . * p<0.05 , * * p < 0.01 : paired t - test . # # p<0.05 : independent t - test
the sacroiliac joint refers to the posterior joint of the bony pelvis between the sacrum
and the ilium of the pelvis . with its extremely limited mobility and small joint
mobilization
, this joint rarely causes any pathological problem except for body imbalance
due to sacroiliac joint obliquity and changes in iliac , ischium , and length of the
ilium8 .
the analysis of the pelvis
obliquity showed the intervention of this study provided the greatest statistically
significant interaction effect on left and right pelvis obliquity ( p<0.001 ) .
furthermore ,
a group performing combined joint mobilization and functional exercise showed statistically
significant differences between before and after the exercise whereas the control group
performing simple joint mobilization did not ( p<0.001 ) . in a previous study , yang9 reported the measurement results of changes
around the sacroiliac joint after 12 weeks of rolling massage for patients with chronic low
back pain .
he found that ilium deviation was reduced by about 2.37 mm , sacroiliac joint
deviation by about 2.25 mm , and ischium deviation by about 2.5 mm , resulting in a
significant difference overall in the sacroiliac joint - related areas ( p<0.001 ) .
however ,
lee10 reported that changes in low back
pain found in experimental and control groups after manipulative therapy administered to
patients with sacroiliac joint dysfunction with muscle imbalance differed significantly
between the two groups before and after the experiment ( p<0.001 ) , whereas changes in
pelvis rotation between the experimental and control groups showed significant difference
between before and after the experiment ( p>0.05 ) , in contrast to our study s results .
we
attribute difference in these study results to the difference between our joint mobilization
and lee s manipulative therapy , and to the lack of any functional exercise . for pain control , it is advantageous to discover and treat specific lesions causing the
pain ( trigger point , over - loaded muscle , weakened or abnormal movement types , or joint
dysfunction ) , not only to reduce the symptoms ( pain ) but also to induce functional
recovery11 .
the results for the joint mobilization intervention used in this study show that two groups
of showed statistically significant decreases of pain compared to the control group
( p<0.001 ) . in a previous study , lee12 reported
significant reductions of low back pain , functional disorder level , and low back instability
in patients with chronic low back pain after lower extremity strengthening exercise along
with low back stabilization exercise ( p<0.05 ) .
he also claimed that a program of combined
exercise performing low back stabilization exercise and lower extremity strengthening
exercise was more effective at decreasing low back pain , functional disorder level , and low
back instability than a stabilization exercise alone .
han13 reported that a combination of
functional exercises resulted in significant pain relief from 4.61 to 1.94 on a subjective
pain index ( vas ) , while simple exercise resulted in non - significant pain relief from 3.93 to
1.57 .
furthermore , im14 reported that vas
showed a significant difference between before and after chuna therapy and spinal
stabilization exercises for 16 weeks .
significant differences were also found between two
groups : a group of single treatment with chuna therapy and a group of combined treatment of
chuna therapy and spinal stabilization exercise . patients with univentricular heart malformations are at increased risk of suffering from thromboembolic events ( te ) . at least 20% of patients with univentricular hearts have reported to experience te , of which 25% are fatal . despite the high incidence of te , no consensus has been reached regarding the role of long - term anti - thrombotic treatment in this group of patients . here ,
we present a case of a 19-year - old woman with a univentricular heart who suffered a major stroke .
a 19-year - old woman born with a univentricular heart was found unconscious in her bed in the morning .
she was respiratory and circulatory stable with no fever at admission to the local hospital .
the glasgow coma scale score was 5 ( eyes , 1 ; verbal , 1 ; motor , 3 ) .
an electrocardiogram showed sinus rhythm , left axis deviation , and left - sided hypertrophy , but was otherwise normal .
a test of arterial blood gasses revealed a fully compensated metabolic acidosis with ph 7.37 ( normal range 7.377.45 ) and base excess 7.7 mm ( 3.0 to 3.0 mm ) .
venous blood tests showed raised plasma lactate 3.7 mm ( 0.72.1 mm ) , plasma myoglobin 280 g / l ( 1949
g / l ) , plasma glucose 8.6 mm ( 4.27.2 mm ) , plasma fibrin d - dimer 1.0 mg / l ( 0.00.5 mg / l ) , and inr 1.5 ( < 1.2 ) , whereas the remaining standard tests were all normal , including hemoglobin , leucocyte differential count , electrolytes , c reactive protein , liver and pancreas enzymes , renal parameters , plasma ethanol , plasma paracetamol , and plasma salicylate .
shortly after admission , the patient developed babinski reflexes and a pronounced decorticate posture with spontaneous flexion of the arms over the chest and extended legs with feet turned inward .
after a tracheal tube was inserted and assisted ventilation was initiated , the patient was transferred to the neurological intensive care unit at a tertiary hospital .
a repeated computed tomography scan and magnetic resonance imaging of the head were performed , as well as a computed tomography angiography of the head and the neck .
these scans unveiled a major stroke located in the left cerebral ( figure 1 ) and cerebellar hemispheres , corresponding to the areas supplied by the left middle and posterior cerebral arteries and the left superior cerebellar artery .
a segmental occlusion in the top of the basilar artery was identified ( figure 3 ) .
finally , a transthoracic echocardiography was performed , revealing a well - functioning univentricular heart with no detectable thrombi .
warfarin was prescribed and the patient gradually regained consciousness . however , a severe right - sided hemiparesis persisted and the patient was transferred to a local neurorehabilitation unit .
later , a magnetic resonance imaging scan of the thorax and upper abdomen was performed ( figure 4 ) .
the patient had been referred to a cardiologist at the age of 5 months due to shortness of breath and failure to thrive .
cardiac ultrasound and catheterization had revealed a double inlet left ventricle ( figure 5 ) and a hypoplastic right ventricle without transposition of the great arteries .
symptoms were caused by heart failure due to high pulmonary flow , which was treated by pulmonary artery banding . at the age of 6 years , a total cavopulmonary connection ( tcpc ) , including a lateral tunnel with fenestration to the right atrium , was established .
the surgical procedure markedly improved the patient s well - being , and , at the age of 13 years , the fenestration was closed .
life - long prophylactic antithrombotic treatment with salicylic acid was prescribed . during the last surgical intervention ,
the patient suffered mild brain damage and was now described as behaving at the level of a 12-year - old .
postoperative echocardiography revealed good systolic function of the left ventricle and mild right atrioventricular valve regurgitation .
since no cardiac thrombi were identified , it is not known whether the described stroke was caused by a cardiac - derived embolus or spontaneous cranial thrombi .
spontaneous thrombi in 19-year old patients are , however , extremely rare . hence , seen in the light of a known cardiac malformation , the probability of a cardiac - derived cerebral embolus is very high .
the incidence of univentricular hearts is reported to be between 0.5 and three cases per 10,000 live births.1 today , patients with univentricular heart conditions are usually treated surgically with a three - stage tcpc / fontan procedure,2 which separates the systemic and pulmonary venous return a precondition for adequate oxygenation of the arterial blood entering systemic circulation .
the technique has evolved from the classic fontan3 ( right atrium - to - pulmonary artery connection ) through an intracardiac lateral tunnel procedure , and it is currently performed in many centers as an extracardiac tunnel procedure by insertion of a tube graft between the inferior vena cava and the pulmonary artery.4 te , both systemic venous and arterial , are a major cause of early and late mortality in tcpc patients .
the reported incidence of te in these patients varies from 3% to 25% , depending on study design , imaging technique , and follow - up period duration .
studies with longer follow - up periods and more sensitive imaging studies suggest an incidence of at least 20% , of which the mortality rate is 25%.59 asymptomatic pulmonary emboli have been detected in 16% of tcpc patients .
although the etiology for the high risk of te is not well defined , possible explanations include abnormal blood flow in the univentricle , arrhythmias , venostasis , dehydration , protein - losing enteropathy , and coagulation abnormalities .
no stratification of te risk has been made between the different types of surgical intervention .
such data would be very useful and should be compared against the efficacy of each treatment .
procoagulant factors ( factors ii , v , vii , ix , and x ; plasminogen ; and fibrinogen ) and anticoagulant factors ( protein c and antithrombin iii ) are lower than normal controls prior to stage two or three of tcpc completion.10,11 increased platelet reactivity prior to tcpc completion has also been shown . a recent report , however , found no significant differences in thromboelastography ( a global whole - blood assay of coagulation ) in pediatric tcpc patients compared with healthy children.12 despite the high incidence of te , no consensus has been reached about the role of long - term antiplatelet or anticoagulation therapy in those patients who remain in stable sinus rhythm .
some studies advise against routine anticoagulation,13 whereas others recommend routine antiplatelet14 or anticoagulation therapy.15,16 one recent study suggests that antiplatelet and anticoagulation therapy are equally effective in preventing te.17 te reduce quality of life18 and cause sudden death19 in adult patients with fontan circulation .
therefore , in these patients , the benefit of long - term prophylactic antiplatelet or anticoagulation therapy must be carefully considered and weighed against the risk of detrimental hemorrhagic side effects .
disagreement about antithrombotic therapy in tcpc patients warrants future study that compares the different therapeutic strategies .
future studies should most likely be observational case - control studies due to the practical and ethical problems associated with randomized controlled trials .
the need for such studies is emphasized by the presented case , which might suggest that more aggressive antithrombotic strategies should be routinely introduced . | [ purpose ] the present study aimed to determine the effect of an 8-week program of joint
mobilization on changes in pelvic obliquity and pain level in seventeen female university
students aged in their 20 s with sacroiliac joint dysfunction by dividing them into two
groups : a joint mobilization group ( mwm ) and a control group .
[ subjects ] seventeen
subjects were selected from female university students aged in their 20 s attending n
university in cheon - an city , korea , the subjects had sacroiliac joint syndrome , but
experienced no problems with daily living and had no previous experience of joint
mobilization exercise .
the subjects were randomly assigned to a joint mobilization group
of eight and a control group of nine who performed joint mobilization exercise .
[ methods ]
body fat and lean body mass were measured using inbody 7.0 ( biospace , korea ) . the direct
segmental multi - frequency bioelectrical impedance analysis method ( dsm - bia ) was used for
body composition measurement .
a pressure footstool ( pedoscan , diers , germany ) and a trunk
measurement system ( formetric 4d , diers , germany ) , a 3d image processing apparatus with
high resolution for vertebrae , were used to measure 3d trunk images of the vertebrae and
pelvis obliquity , as well as static balance ability .
[ result ] the mwm group showed a
significantly better balance than the control group .
in addition , the results of the
left / right and the front / rear balance abilities were significantly better than those of
the control group . [ conclusion ] this study proved that a combination of mobilization with
movement and functional training was effective in reducing pelvis malposition and pain ,
and improving static stability control . |
lichen sclerosus et atrophicans ( lsa ) is a chronic inflammatory muco - cutaneous disorder , characterized by sclerotic and atrophic lesions , most commonly found in adult women .
it affects the anogenital area in 8598% of the cases and less frequently the extrgenital area .
the most commonly affected extragenital areas are the neck and the shoulders , but also the inner thighs , the submammary area , the wrist and occasionally the oral mucosa .
the involvement of the scalp is not frequent and its outcome could be similar to scarring alopecia , which could be the result of different diseases .
the etiology of lsa is still unknown . besides the genetic and local factors ( koebner phenomenon ) and the autoimmune hypothesis , supported by the association of different autoimmune disorders , especially of thyroid origin ( 30% of cases ) , an infectious hypothesis has also been proposed .
a 57-year - old caucasian woman presented with a history of asymptomatic frontoparietal lesion . such lesion , which had been developing over the 3 previous years , was initially erythematous and became progressively atrophic and sclerodermic .
the patient had been living in a highly endemic area for borrelia burgdorferi , but she could not recall any tick bite or erythema chronicum migrans .
she also reported the simultaneous onset of migrating diffused myoarthralgias involving knees , hands , ankles , elbows , shoulders , as well as short - term memory impairment for two years , worsened previous seasonal insomnia for one year .
moreover , she presented migrating paresthesias involving the left side of the body for one month .
the dermatological examination revealed an atrophic - sclerodermic lesion of about 10 cm in length and 3 cm in width , from the scalp to the centre of the forehead as reported in figure 1 .
the skin was thin , inelastic , mother - of - pearl in shade , with erythematous margins of the lesion with subsequent scarring alopecia as well ( figure 1 ) .
figure 1lichen sclerosus et atrophicans with frontoparietal distribution , mimicking scleroderma en coup de sabre .
a skin biopsy was obtained from the scalp and after histological examination three different pathologists confirmed independently the diagnosis of lsa .
it consisted of epidermal atrophy , oedema with superficial layer , hyperkeratosis and collagen production with cell rarefaction .
lymphoid infiltrate was observed even in the dermal - subepidermal junction indicating a possible later evolution of the lsa toward morphoea ( figure 2 ) .
figure 2h&e stained section of the lesion ( a ) 2,5 magnification of the entire histological section , ( b ) 20 magnification of the lichen sclerosus et atrophicans features ( c ) 40 magnification of the lymphatic infiltrate .
h&e stained section of the lesion ( a ) 2,5 magnification of the entire histological section , ( b ) 20 magnification of the lichen sclerosus et atrophicans features ( c ) 40 magnification of the lymphatic infiltrate .
serological igm and igg for borrelia burgdorferi with enzyme - linked immunoassay test ( elisa ) confirmed by western blot analysis was negative .
anti - nuclear antibodies ( ana ) , extractable nuclear antigen antibodies ( ena ) , anti - native dna antibodies ( n - dna ) , anti - neutrophil cytoplasmic antibodies ( anca ) and erythrocyte sedimentation rate were negative .
pcr analysis for the detection of borrelia burgdorferi was performed on dna obtained from formalin - fixed paraffin - embedded skin biopsy , blood and urine as previously reported .
the diagnosis of lyme borreliosis was made on the basis of clinical data , supported by the pcr positivity for borrelia genome .
the patient underwent antibiotic treatment with 2 cycles ceftriaxone 2 gr / day i.v for 21 days .
after about 6 months since the beginning of antibiotic therapy , the lesion had not progressed , all other clinical symptoms improved and blood pcr resulted negative .
the patient received vitamin e 400 mg 2x / day per os for 3 months and applied topic vitamin e prior to uva-1 therapy .
thanks to this combined therapy a remarkable regression of the atrophic - sclerodermic lesion was observed .
this report describes a case of lsa , which was unusual for the involvement of the scalp
. the lesion was mimicking scleroderma en coup de sabre , which is a frontal or frontoparietal linear morphoea characterized by a linear band of depressed atrophy on skin and scalp .
the classical features of lsa are represented by hypopigmented papules that coalesce into white plaques with epidermal atrophy .
although anogenital lsa is associated with a risk of 45% of squamous cell carcinoma , extragenital lesions do not seem to carry any risk of malignant degeneration .
the isolated linear frontoparietal involvement was described in few cases and may clinically simulate scleroderma en coup de sabre , requiring careful histopathological recognition . in this case
it has already been reported that overlap of histologic features between lsa and morphoea may occur , however in the reported case the clinical and the histologic features were not ambiguous of lsa .
the possible later evolution toward morphoea in this case , due to the migration of the lymphatic infiltrate , is not unusual since morphoea and lsa may be closely related such that the latter could be considered the superficial expression of the same disease process which results in morphoea . regarding the possibility of an infectious etiology of lsa , since the first proposal by aberer and stanek in 1987 several european studies
some atrophic skin diseases have been proposed as manifestation of lyme borreliosis with contradictory results .
the detection methods , the examined specimens , such as sera , skin biopsies and urine , together with the different geographic region could explain the conflicting results on the association of borrelia with morphoea and lichen sclerosus et atrophicus .
moreover in the manifestation of long standing infection of borrelia the paucity of microorganisms could lead to a low detection rate by pcr , especially when the analysis is performed on archival biopsies .
no decision can be made to date as to whether bb plays a role as causative agent of different types of circumscribed scleroderma and lsa . with regard to the disparate findings in different geographic areas
, it can be speculated that lsa may be caused in some cases by bb genotypes which are present in that area only . to support this infectious etiology in endemic regions ,
the fact that in this case borrelia dna was detected both on dna obtained from the biopsy and from blood and urine , strongly supports the hypothesis that borrelia has a causative role on the onset of this unusual lsa .
moreover , the geographical location , middle europe , has been highly associated with borrelia prevalence .
indeed , it is well known that there are significant geographic differences in borrelia infections with a higher prevalence in areas in middle europe . to further support this theory ,
lyme disease affects mostly the skin : about 80% of all lyme borreliosis cases present skin manifestations .
we recognise that classical dermatological events include erythema chronicum migrans ( ecm ) , lymphadenosis benigna cutis ( labc ) or borrellial lymphocytoma ( bl ) and acrodermatitis cronica et atroficans ( aca ) , but there is growing evidence that some cases of other cutaneous manifestations could be related to borrelial late infection , mostly borrelia afzelii .
common laboratory tests are not usually revealing for the diagnosis of lyme borreliosis and serologic tests support the diagnosis , but are not always essential in this regard . in this case of lsa mimicking scleroderma en coup de sabre , an associaton with lyme borreliosis
is proposed . to our knowledge , this is the first case of lsa , mimicking scleroderma en coup de sabre , which was associated with lyme borreliosis .
our findings indicate an association between this particular form of lsa and borrelia , suggesting that borrelia burgdorferi itself could represent a causative agent of this atypical form of lsa , however it can not be excluded that borrelia could be only one of the predisposing agent triggering it .
in conclusion we propose that tissue pcr for dna of borrelia should be performed in patients with lsa in endemic area , because it could represent a rare manifestation of borreliosis , and in those cases lsa should be treated with proper antibiotic therapy in order to eradicate the microorganism . antiphospholipid syndrome ( aps ) is an autoimmune disease characterized by thrombosis and morbidity , specifically in pregnancy , due to antiphospholipid antibodies .
about half of the cases of aps occur as a primary disorder , while the rest arise in association with other autoimmune diseases , such as systemic lupus erythematosus ( sle ) . some diseases , such as pulmonary thrombosis and pulmonary hypertension ,
are known to be complicated by aps ; however , aps with pleural effusion is extremely rare . here ,
we present a case of aps complicated by unilateral pleural effusion that responded well to oral corticosteroid therapy .
a 75-year - old japanese man was admitted to our hospital for spreading erythema on his trunk and extremities , as well as dyspnea .
one year prior to admission , he visited us with a 1-year history of erythema and purpura on his legs , accompanied by intermittent fever .
results of laboratory examinations for antiphospholipid antibodies , lupus anticoagulant ( using the phospholipid neutralization test ) , and anticardiolipin antibody had been positive 12 weeks apart .
in addition , he showed positive antinuclear antibody ( 1:80 , homogeneous pattern ) , but was negative for anti - dsdna antibody , anti - sm antibody , anti - rnp antibody , anti - ss - a antibody , anti - ss - b antibody , antitopoisomerase i antibody , and anticentromere antibody .
mpo - anca , pr3-anca , and cryoglobulin were negative . given the diagnosis of aps , we initiated combination therapy with aspirin ( 100 mg daily ) and warfarin ( target international normalized ratio , 2.03.0 ) , but the skin lesions continued to gradually worsen .
violaceous erythema , purpura , and pigmentation were widely noted on his trunk and extremities ( fig .
1 ) ; they were associated with low platelets ( 93,000/l ) and elevated partial thromboplastin time ( 48.4 s ) .
a biopsy specimen revealed marked thrombosis in the dermal and subcutaneous small vessels ( fig .
2 ) . there were interface changes of the dermo - epidermal junction and mild inflammatory infiltrates in the perivascular area of the dermis , but mucin deposition and thickening of the basal layer of the epidermis were not apparent .
in addition , a chest x - ray and computed tomography demonstrated a large pleural effusion in the left lung ( fig .
3 ) , without evidence of large vessel thrombus . electrocardiogram and echocardiogram were normal . despite serial thoracenteses , effusion recurred .
bacterial and fungal cultures , as well as cytology analyses for malignant cells , were all negative . after excluding infectious diseases , malignancies , pulmonary thrombosis , and heart failure , we added oral prednisolone ( 30 mg daily ) to his prior anticoagulant regimen .
the skin lesions and the pleural effusion improved rapidly , eventually disappearing without complication ( fig .
4 ) . on follow - up clinical examinations , no symptoms related to sle or other collagen diseases were noted .
common causes of pleural effusion include malignancies , infectious diseases , pulmonary embolism , collagen vascular disease , and heart failure .
aps - related pleural effusion has rarely been reported , and those cases that have been reported appeared to be complications of accompanying pulmonary embolism , sle , or catastrophic aps [ 4 , 5 , 6 ] .
pleuritis , which can induce pleural effusion , is the most common pleuropulmonary manifestation of sle . in the present case , after excluding these differential diagnoses , aps was determined to be the direct cause of the pleural effusion .
however , a strong possibility still exists that the pleural effusion may be associated with occult collagen vascular disease , particularly sle or lupus - like disease ( lld ) heretofore undiagnosed .
a long - term follow - up study in 128 patients with primary aps demonstrated that 11 patients ( 8% ) developed sle , while 6 ( 5% ) developed lld during a median follow - up period of 8.2 years ( range , 114 years ) .
the results of this study suggest that the pleural effusion may be attributed to a coexisting condition like lld , although our patient has not fulfilled american college of rheumatology diagnostic sle criteria to date .
this may be supported by the fact that oral corticosteroid therapy was a remarkably effective treatment of the pleural effusion that had previously been unsuccessfully treated by anticoagulant therapy and repeated drainage .
corticosteroids and immunosuppressants continue to be the treatment of choice for severe sle complications , including pleural effusion .
furthermore , the clinical manifestations of primary aps and aps associated with sle are similar , which makes it more difficult to differentiate these diseases . as pleural effusion can be life - threatening
corticosteroids might be an effective choice of treatment for intractable pleural effusion in aps patients .
| lichen sclerosus et atrophicans ( lsa ) is a chronic , inflammatory skin disease of unknown etiology , characterized by atrophy.we report a case of lsa with frontoparietal distribution , mimicking scleroderma en coup de sabre , causing scarring alopecia .
the case was associated with borrelia infection .
the lesion improved with 2 cycles of antibiotic therapy with ceftriaxone 2 gr / day i.v for 21 days associated with uva-1 therapy and local and systemic vitamin e supply ( 400 mg 2x / day per os for 3 months ) .
this case stresses the importance of identifying clinical manifestations associated with lyme disease and the use of tissue pcr to detect borrelial dna in patients with these lesions , but characterized by negative serology for borrelia . |
to describe the surgical technique and initial experience with a single - port retroperitoneal renal biopsy ( sprrb ) .
a single 1.5 cm incision was performed under the 12th rib at mid - axillary line , and an 11 mm trocar was inserted .
after lower pole exposure , a laparoscopic biopsy forceps was introduced through the nephroscope working channel to collect a renal tissue sample .
the mean operative time was 32 minutes , and mean estimated blood loss was less than 10 ml .
the hospital stay of all patients was two days because they were discharged in the second postoperative day , after remaining at strict bed rest for 24 hours after the procedure .
sprrb is a simple , safe and reliable alternative to open and videolaparoscopic approaches to surgical renal biopsy .
image - guided percutaneous renal biopsy is the most widely used method to sample renal parenchyma for the evaluation of malignancy or diffuse renal disease .
the risks of this procedure are minimal and the overall success rate of all renal biopsies varies from 70 to 100% ( 1 ) . its major indications rest on diagnosis and follow - up of several systemic and nephrological conditions that lead to glomerular damage and renal function impairment , providing useful data for treatment and prognosis .
it may also be used for evaluation of solid renal masses and cystic renal lesions ( 1 ) .
however , this method has absolute and relative contraindications that may hamper or preclude it , such as the presence of a solitary kidney , uncontrolled arterial hypertension , coagulation disorders , renal artery aneurysm , previous percutaneous needle biopsy failure and obesity .
bleeding and inadequate amounts of renal tissue for diagnosis are not infrequent , and constitute potential disadvantages of the procedure .
in addition , children may be unable to cooperate , requiring general anesthesia . in these settings ,
open and laparoscopic approaches are well - established alternatives and should be considered , although with a higher level of invasiveness and complexity . in search for an alternative that could minimize surgical aggressiveness of these procedures and
hence spread its use , we outlined a renal biopsy technique through a single retroperitoneal laparoscopic access using standard urological instruments .
the aim of this paper is to describe the technique now standardized in our institution and our initial experience with the single port retroperitoneal renal biopsy ( sprrb ) .
after receiving general anaesthesia , orogastric and bladder catheterization , the patient is usually positioned in the left flank position , as the kidney is more easily accessible at the right side due to its lower position .
a 1.5 cm incision is carried out just below the tip of the 12th rib , at the mid - axillary line , and is followed by blunt access to the retroperitoneum space .
an initial digital dissection is done aiming to identify the lower renal pole , while also displacing the peritoneum anteriorly . during this step
, care must be taken in order to avoid peritoneal tearing , as the pneumoperitoneum resulting from gas insufflation would hamper the maintenance of adequate retroperitoneal working space .
next , a rubber balloon is positioned between the kidney and the posterior abdominal wall , and is filled with 300 - 400 cc of saline , creating a virtual cavity .
the saline is drained after a few minutes , to achieve hemostasis , and the balloon is then removed .
an 11 mm trocar is inserted and carbon dioxide is used to maintain pneumoretroperitoneum at 12 to 15mmhg .
retroperitoneal inspection and identification of the psoas muscle and the lower pole of the kidney are now performed with a standard 26 french nephroscope , as shown in figure-1 .
it is frequently possible to expose the renal surface bluntly , by using gentle movements of the tip of the scope to drag the perirenal fat away from the intended site of biopsy . alternatively ,
standard laparoscopic surgical aspirator , scissors or hooks can be inserted through the working channel of the nephroscope , and then be used to dissect , cut and coagulate nearby structures , allowing a clear renal surface to be assessed .
once the biopsy site is cleared from fat , one or two samples are taken with the aid of a toothed biopsy forceps , also through the nephroscope ( figure-2 ) . bleeding is expected to be negligible , as the injury caused by the forceps is shallow ( figure-3 ) , but the parenchyma can be coagulated with the same instruments , and a cellulose hemostatic bolster can be applied , if needed .
finally , the pneumoretroperitoneum is evacuated and , if no bleeding is observed , the trocar is removed and the access port is closed .
figure 2sampling renal parenchyma with a toothed biopsy forceps , through the nephroscope working channel .
figure 3aspect of kidney surface after a tissue sample was taken , with only minimal bleeding .
at our institution , laparoscopic retroperitoneal renal biopsy is currently often performed for pediatric patients with nephrological conditions ( 2 ) . as the surgical team s experience progressed and the procedure was standardized
, however , we felt that it should be even less invasive , especially for this very young population .
additionally , in order to spread and popularize its execution , we devised how to use instruments that are already present in a regular urological operating room , such as the nephroscope and laparoscopic scissors and forceps , in a different fashion .
a similar approach has been described previously , in pediatric surgery , for appendectomies and varicocelectomies , but with only one case of renal biopsy ( 3 ) . between january and april/2013
, five children underwent sprrb in our hospital , referred from the nephrology clinic for renal biopsy .
informed consent was previously obtained from parents , respecting our institution s ethics committee recommendations and approval .
the procedure was successfully performed with the technique above described , by a supervised resident in - training .
the overall mean operative time was 32 minutes , and mean estimated blood loss was less than 10 ml . no open conversion was needed .
the hospital stay of all patients was two days because they were kept in absolute bed rest for 24 hours after the procedure , before being discharged home .
pain and analgesics use were low , and there were no significant detected complications . regarding the obtained samples , the average number of glomeruli present in the specimens was 31 , and the histopathological findings showed focal proliferative lupus glomerulonephritis in two cases , diffuse mesangial proliferative glomerulonephritis in another two , and nephritis related to henoch - schnlein purpura in one child .
these results are comparable to those previously shown by us , with laparoscopic renal biopsy in children , regarding operative time , blood loss , hospital stay and success in obtaining adequate samples ( 2 ) .
table 1clinical features of patients submitted to single - port retroperitoneal renal biopsy.patientgenderage ( years)bmi ( kg / m)ot ( min.)bl ( ml)gndiagnosiscomplications1m0723.625423diffuse mesangial proliferative glomerulonephritisnone2f0924.5371338nephritis related to henoch - schnlein purpuranone3f1121.827530focal proliferative lupus glomerulonephritisnone4m1024.0401743diffuse mesangial proliferative glomerulonephritissmall skin ecchymosis5f1232.031621focal proliferative lupus glomerulonephritisnoneaverage-9.825.1832931 - -
bmi = body mass index ( kg / m ) ; ot = operative time ( minutes ) ; bl = blood loss ( milliliters ) ; gn = number of glomeruli per biopsy .
bmi = body mass index ( kg / m ) ; ot = operative time ( minutes ) ; bl = blood loss ( milliliters ) ; gn = number of glomeruli per biopsy .
although it is likely that the same approach could be used in adult patients as well . our experience with this very initial group
was composed entirely of children , and sprrb has been shown to be a very simple , safe and reliable alternative to other laparoscopic approaches .
the use of a nephroscope , instead of a regular laparoscope , obviates the need to place an additional trocar for using an auxiliary instrument to dissect the perirenal fat , as is the standard practice ( 4 , 5 ) .
its working channel finely substitutes that , sparing one incision , the cost of another trocar and also surgical time to place it . because a second trocar traditionally would be only 5 mm wide
, it may seem that the benefit here is not strongly relevant in terms of postoperative pain or cosmetic results , but it is our understanding that no technical difficulty was added whatsoever , by using only one access .
moreover , especially children could benefit the most even of a small effect , and coincidently they constitute the majority of patients requiring a surgical renal biopsy in our hospital .
mini - perc nephroscopes are not available at our institution at this time , but its use could be a step forward , in this regard , and further decrease the required size of the access port incision . additionally , the ease for urologists in using regular urological equipment , and the possibility that the surgeon simultaneously controls both the camera and laparoscopic scissor / biopsy forceps , are other advantages of this alternative method . in our hospital , retroperitoneal laparoscopy is the procedure of choice for renal biopsy in children and the sprrb is an even less invasive option for these patients , performed through a single incision and with very satisfactory results and only minor pain . a girl weighing 3,190 g was delivered by a caesarean section at 38 weeks and five days of gestation . at the time of birth , a systolic murmur
was noted during a physical examination , and transthoracic echocardiography revealed a peri - membranous ventricular septal defect ( vsd ) with a septal aneurysm , a small patent foramen ovale ( pfo ) , and a small right - sided patent ductus arteriosus ( pda ) from the innominate artery .
the vsd was measured as having a diameter of 3.5 mm and a shunt flow less than 2.5 m / sec .
due to the presence of neonatal hyperbilirubinemia , echoencephalography was conducted , and no abnormalities were found .
five months later , the patient was referred to konkuk university medical center for vsd and pda .
her body weight was 6,700 g ( 25th percentile ) , and her height was 61.6 cm ( 10th percentile ) .
transthoracic echocardiography revealed a vsd approximately 6 mm in size with a minimal aneurysm , a left - sided pda 3.6 mm in diameter from the right aortic arch and an aberrant left subclavian artery .
a subsequent computed tomographic scan demonstrated isolation of the left subclavian artery with a right aortic arch , a left pda , and a vsd ( fig .
the intraoperative findings were a perimembranous vsd , a pfo , a mildly patent right ductus arteriosus , and isolation of the left subclavian artery connected to the left pulmonary artery via a left pda .
the left subclavian artery was disconnected from the left pulmonary artery and reimplanted to the left common carotid artery by end - to - side anastomosis with monofilament polypropylene 6 - 0 sutures ( fig .
ductus arteriosus is usually located on the left side , between the descending aorta and the junction of the main pulmonary artery and left pulmonary artery .
however , ductus arteriosus may also be present on the right side or , very rarely , may occur bilaterally in association with aortic arch anomalies or conotruncal anomalies .
in such aortic arch anomalies , isolation of the left subclavian artery with right aortic arch is also uncommon . here
, isolation refers to the fact that the left subclavian artery connects to the pulmonary artery via either the ligamentum arteriosum or a patent ductus arteriosus without any connection to the aorta .
isolation of the left subclavian artery with a right aortic arch is known to be commonly associated with congenital heart disease , but may also occur with normal intracardiac anatomy , although few such cases have been described .
isolation of the left subclavian artery with a right aortic arch may be related to the 22q11 deletion .
bilateral ductus arteriosus and isolation of the left subclavian artery with a right aortic arch can be explained through the hypothetical double aortic arch plan suggested by edward .
regression takes place on two levels in the double aortic arch plan : on one level , regression occurs between the left common carotid artery and the left subclavian artery ; and on the other level , regression occurs at the left dorsal aortic root distal to the left ductus arteriosus .
and then right ductus arteriosus remains persistent , left ductus arteriosus connects the left subclavian artery to the left pulmonary artery ( fig .
however , the right ductus arteriosus regressed , and only the left ductus arteriosus remained patent .
if the left ductus arteriosus is patent , blood may be supplied to the left subclavian artery via the left ductus arteriosus .
if the left ductus arteriosus regresses , the blood supply to the left subclavian artery may involve a mediastinal , thoracic anastomosis , or vertebral pathway .
isolation of the left subclavian artery usually presents with no apparent symptoms in neonates , but it may present with congenital pulmonary steal syndrome , subclavian steal syndrome , or may even present in adults with late symptoms due to sporadic progression .
hayabuchi et al . reported the case of a three - month - old girl with cerebral atrophy and an underdeveloped left arm .
reported the case of a 15-year - old boy with an underdeveloped left arm . due to these symptoms and signs ,
the therapeutic management of isolation of the left subclavian artery remains controversial , especially when it is associated with complicated congenital heart disease .
some authors have suggested that adequate collateral circulation must be ensured , meaning that reconstruction of the isolated subclavian artery is optional , regardless of the symptoms and signs .
successful results have been reported after ligation or device closure of the pda and ligation of the left subclavian artery .
however , reconstruction of the left subclavian artery due to pulmonary steal syndrome after right pda closure in bilateral pda has been reported . in one report , ischemic symptoms in the left arm and vertebrobasilar insufficiency occurred years after ligation of the left subclavian artery .
hokari et al . reported that a man with peutz - jeghers syndrome presented with his first vertigo attacks due to subclavian steal syndrome at 29 years of age .
our patient presented with no symptoms and signs related to subclavian or pulmonary steal syndrome , and had shown normal findings on an echoencephalography study conducted at our medical center due to neonatal hyperbilirubinemia .
however , brain computed tomography angiography performed after surgery revealed hypoplasia of the left vertebral artery .
we suggest that this hypoplasia would have led to vertebrobasilar insufficiency or underdevelopment of the left arm without surgical reconstruction .
since surgical reconstruction of the isolated left subclavian artery leads to antegrade flow in the left subclavian artery , it can prevent hypoplasia of the left vertebral artery and subclavian / pulmonary steal syndrome .
our case shows that early surgical reconstruction is reasonable , regardless of the symptoms , in cases of isolation of the left subclavian artery . | objective to describe the surgical technique and initial experience with a single - port retroperitoneal renal biopsy ( sprrb).materials and methods between january and april 2013 , five children underwent sprrb in our hospital .
a single 1.5 cm incision was performed under the 12th rib at mid - axillary line , and an 11 mm trocar was inserted .
a nephroscope was used to identify the kidney and dissect the perirenal fat .
after lower pole exposure , a laparoscopic biopsy forceps was introduced through the nephroscope working channel to collect a renal tissue sample.results sprrb was successfully performed in five children .
the mean operative time was 32 minutes , and mean estimated blood loss was less than 10 ml .
the hospital stay of all patients was two days because they were discharged in the second postoperative day , after remaining at strict bed rest for 24 hours after the procedure .
the average number of glomeruli present in the specimen was 31.conclusion sprrb is a simple , safe and reliable alternative to open and videolaparoscopic approaches to surgical renal biopsy . |
the head and neck posture of an individual can influence soft - tissue relationships in the
cervical and shoulder region1 , 2 .
a common concern in the modern workplace is upper extremity
disorders arising from overhead work , which is associated with neck and shoulder disorders
and pain3 .
long - term overhead working
postures result in strain and fatigue of the shoulder muscles because arm elevation is
associated with shoulder muscle fatigue4 , 5 .
previous studies have focused on risk
factor analysis and the development of therapeutic exercises for overhead work - related
disorders rather than prevention6 , 7 .
some studies have been performed on
postural ergonomic interventions including working techniques for overhead work6 , 7 .
however , we found that few studies have focused on protective ergonomic devices for overhead
workers .
therefore , this study investigated a new neck support tying ( nst ) method that used
a thera - band for the prevention of neck and shoulder pain in workers performing overhead
work .
the new nst method supports the neck during hyperextension and prevents excessive
upward rotation of the scapula during overhead work .
the purpose of the present study was to
investigate the effect of this nst method on cervical rom and shoulder pain after overhead
work .
the subjects were divided into two groups as follows : a control group consisting of 7 males
without nst , and a nst group consisting of 7 males with nst .
the initial cervical rom and
initial ppts of the ut and mt were not significantly different between the two groups .
the
initial values of cervical flexion , extension , and right and left lateral flexion in the
control group were 63.44.2 , 72.86.0 , 53.92.9 , and 51.35.6 degrees , respectively .
the
initial values for cervical flexion , extension , and right and left lateral flexion in the
nst group were 62.35.1 , 72.53.9 , 53.33.0 , and 52.22.4 degrees , respectively .
all
participants gave their informed , written consent according to the protocol approved by the
human ethics committee of the yonsei university faculty of health science .
this study
examined a new nst method that uses a thera - band for the prevention of neck and shoulder
pain in workers performing overhead work . for the nst method
, we used the grey thera - band
( 60 cm length ) which was applied as follows .
the midpoint of the thera - band supported the
posterior aspect of the neck , and both ends of the thera - band were passed under both
axillae , and tied behind the back .
the nst provided support for neck hyperextension and
prevented excessive upward scapular rotation during overhead work .
cervical flexion ,
extension , and right and left lateral flexion were measured with a cervical range of motion
( crom ) instrument ( performance attainment associates , st .
a dolorimeter pressure algometer ( fabrication enterprises , white plains ,
ny , usa ) was used to measure the pressure pain threshold ( ppt ) of the right side upper
trapezius ( ut ) and the lower trapezius ( lt ) muscles .
a 1-cm rubber plate
delivers pressure from the probe to the body , and the pressure is read from a needle gauge .
all subjects performed one
trial of overhead work with their arms over their heads for 15 min .
the overhead work was
performed at a height of 25 cm above each subject 's head .
differences in cervical rom and ppt between the nst and control groups
after the overhead work were tested with the independent t - test using the spss statistical
package ( version 18.0 ; spss , chicago , il , usa ) .
the cervical flexion , extension , and lateral flexion angles of the nst group were
significantly larger than those of the control group ( p < 0.05 ) .
the cervical flexion ,
extension , and right and left lateral flexion of control group were 50.48.2 , 64.711.3 ,
41.77.9 , 43.29.2 degrees , respectively .
the cervical flexion , extension , and right and
left lateral flexion of nst group were 61.511.2 , 69.46.9 , 48.75.6 , 49.86.7 degrees ,
respectively .
the ppt of ut of the nst group ( 7.21.8 lb ) was significantly higher than
those of the control group ( 6.32.0 lb ) ( p < 0.05 ) .
the ppt of mt of the nst group
( 5.81.4 lb ) was significantly higher than those of the control group ( 5.01.2 lb ) ( p <
0.05 ) .
repeated and sustained working with elevated arms is known to lead to neck and shoulder
pain8 .
this study proposed a new neck
support tying method using thera - band and investigated its effect on cervical rom and
shoulder pain after overhead work .
reductions in rom have implications for the safety and
efficiency of functional activities , and lead to a loss of corrective or protective
reactions1 , 9 .
rom losses can occur from inactivity and structural changes of the
tissues in the cervical spine , and result in an increase in connective - tissue density ,
shortening of collagen tissue , and muscle fibrosis1 ,
9 . in this study , the cervical flexion ,
extension , and lateral flexion angles of the nst group
shoulder forward flexion with scapular upward rotation requires the activation of the upper
trapezius , and overstretches the middle trapezius through scapular protraction3 , 5 , 7 .
the ppts of the ut and mt were
significantly lower in the nst group than those of the control group .
these results indicate
that the nst supported the neck and prevented excessive scapular elevation and upward
rotation during overhead work .
the thera - band , which provides varied resistance through the
range of movement , has been used for rehabilitation in combination with therapeutic
exercise10 .
it is light and portable ,
has low resistance , and can be adjusted to accommodate various situations11 .
the nst method prevented rom reduction
and pain in the cervical and shoulder regions .
the nst method can be easily and simply
applied using a thera - band and is also inexpensive .
we suggest that industrial workers could
use the nst method when performing overhead work . in this issue of critical care , laporta and coworkers review a multidisciplinary working group 's analysis of a case study on the causes of and solutions for staff turnover in an intensive care unit ( icu ) setting .
this issue is of profound significance to health care leaders in western countries because the workforce is shrinking as a result of impending baby boomer retirements and , as the population ages , the demand for intensive care services will grow considerably .
these demographic factors are further compounded by the fact that the complexity of care provided in the icu demands professionals who are highly trained and skilled . in this environment
, turnover can be costly to the organization because of the significant expenses associated with recruiting and training workers .
there are many well documented reasons for staff turnover in the intensive care setting that are highlighted by laporta and coworkers as core reasons .
these core reasons include job dissatisfaction due to inflexible scheduling practises , insufficient opportunity for professional development , as well as a lack of collaborative decision making around clinical and practice issues .
the authors discuss that data on icu turnover comes from nursing literature and that this research may be applicable to other health care professionals .
however , it is important not to assume that reasons for turnover are the same among different groups of health care providers and that staff turnover is something to be avoided at all costs .
for example , misra - hebert and coworkers state that one contributor to physician turnover is conflict between the physician 's and organization 's philosophy and goals .
physician turnover in this case may be beneficial both to the physician and organization if the two parties can not reconcile their differences and the conflict impacts on the ability of both parties to move forward .
there are other important reasons for turnover that should be considered by icu leaders , and these include burnout and generational diversity .
burnout is a prevalent phenomenon in icus , and the nursing literature suggests that issues such as moral distress when engaging in futile care contributes to burnout . in the medical literature causes of physician burnout include volume of work , increased expectations of the public , lack of sleep and the possibility of being sued .
the consequence of burnout is that there is a negative impact on quality of care and staff morale , which can ultimately cause turnover .
for example , gunderson indicates that physicians who are dissatisfied may engage in inappropriate prescribing patterns .
neuhauser , furthermore , discusses how environments with rigid systems and attitudes among the leadership will decrease staff morale because staff desire flexible policies and autonomy in decision - making .
the generational diversity found in the icu environment can also be a source of turnover of staff .
it is well documented that generation x ( born in 19651980 ) and the millennial generation ( born in 19802000 ) have a strong desire for more balanced work life than veterans ( born in 19251945 ) and baby boomers ( born in 19461964 ) .
research conducted by lorin and coworkers on internal medicine residents of the millenial generation showed that although 41% considered a fellowship in critical care , only 3.4% chose this training because of lack of leisure time and stress levels among faculty and fellows .
clearly , it is important for leaders to be attuned to these generational differences when developing recruitment and retention plans and redesigning the workplace environment .
the review from laporta and coworkers also highlights the importance of icu leadership working with frontline staff to create a vision and strategy that addresses the core reasons for turnover .
it is essential that this vision be aligned with the vision , mission and values , and strategic plan of the health care organization .
furthermore , the team should assess whether their hospital is highly reputable , has high patient satisfaction , and sufficient resources and equipment to provide care .
all of these components are signs of a positive work environment , and leadership can build on these attributes to recruit and retain staff . the other key factor in this process is the use of a team work approach
. team work training in the areas of conflict resolution , learning styles and giving feedback will help the staff to work together to create and achieve an inspiring vision .
although the financial and human resource investments required to engage in this process are considerable , there is substantial evidence in the literature that highly functioning , satisfied teams lead to more efficient patient care and better outcomes .
staff turnover is a critical issue that icu leaders need to understand and address in their unit settings .
attention to this issue with a systematic , evidence - based approach that focuses on team work and collaboration will not only improve retention but will also make the icu a highly competitive and desirable place to work .
| [ purpose ] this study proposed a new neck support tying ( nst ) method using thera - band for
the prevention of neck and shoulder pain in workers doing overhead work .
the purpose of
this study was to investigate the effect of the new nst method using thera - band on
cervical rom and shoulder pain after overhead work .
[ subjects ] fourteen male subjects were
recruited .
[ methods ] this study measured the cervical rom and pressure pain threshold
( ppt ) of the upper and middle trapezius ( ut and mt ) muscles after the control and nst
groups had performed overhead work .
[ results ] the cervical flexion , extension , and lateral
flexion angles of the nst group were significantly larger than those of the control group .
the ppts of ut and mt of the nst group were significantly higher than those of the control
group [ conclusion ] the nst prevented rom reduction and pain in the cervical and shoulder
regions . |
fungi have been extensively isolated and investigated from skin in various parts of the world .
fungal diseases have markedly increased during the recent years ( 1 ) ; although more increase is observed in opportunistic mycoses .
determining the mycoflora of normal people is important when the role of skin is considered as a reservoir for microorganisms .
infectious diseases , mostly the ones that affect the epidermal or mucous membrane , are serious troubles all over the world because of hygiene and education deficiency .
microbiome of human skin refers to complete collection of microorganisms comprising bacteria , fungi and virus .
the kind and quantity of skin microbes are different from one individual to another depending on the location of the body .
fungi are among the most significant groups of these skin pathogens ( 2 ) .
cutaneous mycoses refer to skin infection and its appendages are involved in yeasts and filamentous fungi .
they have the affinity to parasitized tissues with rich keratin and create the skin inflammatory responses and cause severe itching , burning and redness . moreover , these diseases cause cosmetic outcomes .
candidiasis includes the infections that vary from superficial ; for example , oral thrush and vulvovaginitis , to visceral and potentially life - threatening diseases
. superficial infections of dermal inflammation and discomfort are frequent in numerous human societies .
toe webs harbor fungi more than the less occluded parts such as trunk , legs , and arms ( 2 ) . at present a normal healthy skin in adults
is expected to transmit a number of representatives of the genera candida , malassezia and geotrichum as well as few anthropophilic dermatophytes as inhabitants of the normal skin .
direct examination , and culture and molecular analyses are employed to recognize the skin microbial inhabitants .
direct examination is based on finding the microbial elements such as unicellular yeast , mycelium and pseudohyphae .
the presence of malassezia spp . in healthy human skin was identified in previous investigation in the nineteenth century .
the incidence and density of colonization depended on the activity of sebaceous gland and age .
the species most commonly associated with this disease are , malassezia
globosa , m.
furfur and m.
sympodialis ( 3 - 5 ) . this disorder could be observed in temperate climates , particularly during summer in humid months .
the malassezia yeast form generates dicarboxylic acids similar to azelaic acid which prohibit tyrosine kinase that consequences in hypopigmentation of skin ( 4 )
diagnosis of this disease is clinically easy and it is confirmed by microscopic examination of skin scraping on potassium hydroxide or using scotch tape .
conversion from yeast form of malassezia to mycelia form was promoted with warm and humid weather .
immunodeficiency , poor hygiene , diabetes and poor nutrition are other factors related to pityriasis versicolor .
the seborrheic areas are chest , back , abdomen , neck , and proximal arms .
the lesions possess different colors such as yellow , brown , pink , red or hypo pigmented .
keratinophilic fungi are the groups which could infect the skin , hair and nail in human ( 6 , 7 ) .
anthropophilic type of theses fungi can apparently live in healthy human skin which may contribute to transmission of the fungi ( 8 , 9 ) .
the current study aimed to investigate the incidence of fungal flora on interdigital spaces of the human foot .
samples were collected from toe webs of 865 girl students who lived in the dormitories of ahvaz jundishapur university of medical sciences in autumn 2008 .
the tested web spaces did not show any signs of infection such as erythema , scaling , blistering and itching .
the samples were collected into sterilized clean pockets and transferred immediately to the mycology medical laboratory .
a part of the samples were digested with 20% koh and screened by a light microscope for fungal elements .
another part of the samples were cultured on sabouraud glucose agar ( sga ) and sga containing 0.05 mg / ml chloramphenicol and 0.5 mg/ ml cyclohexmide .
the filamentous fungi were identified after slide culturing according to their gross and morphological features .
yeasts were identified on the bases of germ tube formation , morphology on the corn meal agar medium , and assay of unease activity .
out of the 865 samples , 616 ( 71.2% ) were positive in direct examination or culture .
the culture was positive in 349 ( 40.3% ) specimens ; and 22 ( % 2.5% ) samples were also positive for both direct examination and culture .
the most common fungal isolates in direct test were yeast ( 29.4% ) , followed by conidia ( 0 .
92% ) , melanised hypha ( 0.35% ) and non - septated hypha ( 0.23% ) .
skin surface is moderately dry , fairly acidic and dead cells are the prime source of nutrition .
skin has an environment which inhibits the growth of numerous microorganisms , however , some have adapted to living on the skin .
candida and malassezia species are two good examples that inhabit on the skin ( 3 , 4 , 10 , 11 ) .
the current study performed a comprehensive and quantitative analysis of the mycoflora on the surface of interdigital skin of girl students .
overall fungal structure was found on the skin of 30.9% of the students in the direct examination with koh .
the rates of the total yeast counts were much higher than those of molds in samples of skin in the direct examination .
the species of candida live on skin and cause the frequent skin infection named candidiasis .
candida species were isolated from 1.2% of interdigital spaces of people in the current study .
although skin candidiasis is not life threatening , it can affect the emotional and physical status of the patients .
malassezia species is considered as an opportunistic fungus , since it stays benign until the conditions that convey it to cause infection are provided . in the current study ,
various fungi which can be considered as possible agents for skin diseases were isolated . in the present study ,
various fungi were isolated from the skin which could be considered as possible causes of skin diseases . compared to many fungal diseases ,
host - fungus association in dermatophytosis was significant because dermatophytes affect immunocompetent persons ; however they usually invade just superficial keratinized tissues .
currently , not much has been recognized from the relevant factors which mediate adherence of these fungi to host .
the capability of t.
rubrum to adhere to epithelial cells is a trait to carbohydrate - specific adhesin on microconidia ( 12 ) .
morphological observation detected the fibrillar projections in t.
mentagrophytes through the adherence stage ( 13 , 14 ) . in the current research various saprophytic fungi
actually , many of the fungal spores recovered from the skin can be found in the atmosphere .
the present study demonstrated the incidence of fungal flora on interdigital spaces of human foot .
the obtained results showed that fungi can survive on the surfaces of skin without showing the sign of infection . implant - supported overdenture prostheses can be divided into bar overdentures and single attachment overdentures .
single attachment elements for overdentures include single retentive anchors , single magnet anchors , and individually cast telescopic copings.1 among these , telescopic copings have the benefit of implant splinting found in bar overdentures and the retrievability of single attachment overdentures
. however , this method is typically fabricated using gold materials , so it is not an economical treatment option .
also , if an inner crown will be worn , it is difficult to maintain appropriate retentive forces . in this case report
, a telescopic implant - supported overdenture prosthesis was made using a new material , polyaryletherketone ( paek ) based polymer ( pekkton ivory , cendres + mtaux sa , biel / bienne , switzerland ) .
it shares benefits of typical telescopic coping , in additional to being highly economical , wear resistant , and light in weight compared to conventional implant overdenture prostheses .
1 ) presented to the department of prosthodontics at chonnam national university dental hospital . after clinical and radiographic examinations
after maxillary teeth extractions and use of provisional maxillary complete denture for six months , six small diameter implant fixtures ( 3.0 10.0 mm usii , osstem implant co. ltd . , seoul , korea )
the definitive prosthesis was planned as a telescopic overdenture using paek based polymer . after a making definitive impression by polyvinylsiloxane ( honigum , dmg , hamburg , germany ) , a polymer telescopic abutment and an outer overdenture frame were fabricated with consideration of the patient 's vertical dimension ( fig .
telescopic abutment and framework design were laid out by cad software ( exocad dental cad , exocad gmbh , darmstadt , germany ) . the milling machine
( s1 , vhf camfacture ag , ammerbuch , germany ) made the final framework and abutment according to the design .
polymer abutment and titanium link were sandblasted by 110 um grit aluminum oxide , and bonded with primer ( sr link , ivoclar vivadent , schaan , liechtenstein ) and bonding agent ( multilink n , ivoclar vivadent , schaan , leichtenstein ) .
after that , the definitive prosthesis was made by autopolymerized pour - type resin ( press lt , retec , rosbach , germany ) ( fig .
4 ) ; the design and weight of the prosthesis were adjusted to achieve acceptable esthetics and phonetics . after 6 months , there were no problems with alveolar bone around the implant fixtures and retention of the overdenture prosthesis .
however , no treatment modality meets all criteria for successful treatment , and conventional overdenture material can sometimes be limited by economic , functional , and technical considerations .
now , many new prosthetic materials are available to overcome these limitations , and as in this case , a new polymer can be used to make telescopic crowns and frameworks to obtain satisfactory results .
paek based polymer , pekkton ivory , as used in this case , is a member of the high performance semi - crystalline thermoplastic resin group , recognized for its keto and ether group ratio .
paek has good dimensional stability at high temperature , high chemical and mechanical resistance against wear , and high tensile , fatigue and flexural strength , making it an attractive material with expanded uses in medicine and dentistry.3 however , peek ( polyetheretherketone ) , a conventional paek - based polymer , can not be used as a permanent material due to its relatively weak physical properties . a new material , pekkton , is mainly composed of pekk ( polyetherketoneketone ) ; its molecular structure has an added ketone to the structure of peek with and has a wide range of uses due to its amorphous and crystalline structure .
pekk reveals up to 80% greater compressive strengths than peek , so this polymer may be used in permanent prostheses according to the manufacturer.4 thus , this new polymer can be considered to have greater strength than peek and have greater esthetics titanium , higher resin bond strength than zirconia , and a lighter weight ( 1.4 g / cm ) than metal .
therefore , this material is found to be mechanically suitable for fpd frameworks , milled overdenture bars , clasps , telescopic crowns , and other applications . despite the reportedly good bond strength ,
bonding between the titanium link and the telescopic abutment is still very sensitive , with the risk of fracture of the thin abutment wall due to connecting titanium link ; hence more studies are needed on such cases .
she reported satisfaction with its strength and esthetics , and no negative symptoms . due to the lack of evidence on the long term retentive capabilities of this material ,
due to its functional and economic advantages , paek based polymer is a good alternative material to conventional materials and methods in the fabrication of implant overdenture . | background : fungi have been extensively isolated and investigated from skin in various parts of the world . determining the mycoflora of normal people is important when the role of skin is considered as a reservoir for microorganisms.objectives:the current study aimed to investigate the incidence of fungal flora on interdigital spaces of the human foot.patients and methods : samples were collected from interdigital spaces of 865 girl students who lived in the dormitories of ahvaz jundishapur university of medical sciences .
a part of the sample was digested with 20% koh and screened by a light microscope for fungal elements .
another part of the sample was cultured on sabouraud glucose agar ( sga ) and sga containing 0.05 mg / ml chloramphenicol and 0.5 mg / ml cycloheximide .
the fungal colonies were identified based on morphological and microscopic characteristics and biochemical tests.results:in the current study , out of the 865 samples , 616 ( 71 .
2% ) were positive in direct examination or culture .
of these , 267 samples ( 30 .
9% ) were positive in direct examination .
the most common fungal isolates in direct test were yeast ( 29 .
4% ) , followed by conidia ( 0 .
92% ) , melanised hypha ( 0 .
35% ) and non - septated hyphae ( 0 .
23% ) .
trichophyton mentagrophytes was isolated in one of the specimens.conclusionsthe present study demonstrated the incidence of fungal flora on interdigital spaces of human foot .
the current study results showed that fungi can survive on surfaces of skin without showing the sign of infection . |
xanthogranulomatous pyelonephritis ( xgp ) is a rare , distinct and aggressive form of chronic infectious pyelonephritis .
it accounts for lesser than 1% of chronic pyelonephritis . though common in fifth to sixth decade
complications can occur in the form of psoas abscess , nephro cutaneous fistula , enterocolonic fistula , paranephric abscess and sepsis .
it is essential to suspect and diagnose this condition early to prevent the morbidity and mortality . owing to its rarity and clinical curiosity
a 75-year - old man presented with difficulty in micturition since 15 days , fever , abdominal and flank pain and burning micturition since 7 days .
x - ray , ultrasonography ( usg ) and computed tomography ( ct ) scan abdomen findings include pyonephrosis and cortical atrophy in the right kidney .
intravenous urography ( ivu ) revealed non - excretory right kidney . left kidney showed normal excretion
resected specimen was yellowish lobulated renal mass measuring 13 8 6 cm with ureter [ figure 1a ] .
cut section showed dilated pelvis , calyces and cortical atrophy due to extensive destruction of renal parenchyma , which were covered with thick purulent material [ figure 1b ] .
histopathology revealed atrophic and dilated renal tubules showing thyroidisation and sclerosed glomeruli and interstitial fibrosis [ figure 2 ] .
many areas showed histiocytes with abundant foamy cytoplasm , lymphoplasmacytic inflammatory cells with foci of polymorph nuclear leukocytes [ figure 3 and inset ] ziehl nelsen stain was negative for acid fast bacilli .
( a ) yellowish lobulated renal mass with ureter ; ( b ) dilated pelvis , calyces and cortical atrophy covered with thick purulent material atrophic and dilated renal tubules showing thyroidisation , sclerosed glomeruli and fibrosis .
inset show giant cells and cholesterol clefts ( h and e , 400 ) foamy histiocytes , inflammatory cells .
xgp is a severe chronic renal inflammatory condition leading to focal or diffuse kidney destruction .
it may be due to defect in degradation of bacteria in the macrophages especially when associated with infection and obstruction by stones .
three forms of xgp are recognised : diffuse is characterized by diffuse involvement of kidney , segmental by segmental involvement and focal - is located within the cortex .
it is often misdiagnosed pre - operatively as pyelonephritis , tuberculosis , perinephric abscess and renal cell carcinoma ( rcc ) .
symptoms include flank or abdominal pain , fever , palpable mass , gross hematuria , pyuria , dysuria and weight loss . in our case , symptoms were attributed to bph .
other organisms include staphylococcus aureus , group b streptococcus , candida , klebsiella and bacteroides .
elevation of serum creatinine and bun in our case can be attributed to impaired renal function .
non - functioning , ct scan is the main stay of diagnostic imaging for xgp .
xgp has been shown to be associated with transitional cell carcinoma of renal pelvis and rcc .
they have even been reported in renal allograph . in diffuse or advanced stage xgp ,
nephrectomy is the treatment option . focal or segmental xgp if diagnosed early pre - operatively can be treated with antibiotics .
pre- and post - operative broad spectrum antibiotics and symptomatic management are also key factors for successful management and better prognosis .
we presented this unusual case of elderly male to stress the importance of through evaluation of renal function who gave a history of recurrent urinary tract obstruction and infection .
chronic renal infection and obstruction are two common etiological factors for xgp . in all patients of prostatic
enlargement , renal function must be assessed for the extent of damage . in non - functioning kidney polymerase chain reaction ( pcr ) is a commonly used laboratory procedure nowadays for a variety of tasks , such as dna cloning , sequence determination and snp detection .
consequently , numerous primers need to be designed for dna amplification in order to produce enough dna . for dna sequencing , to design primers for the promoter and exon regions of a gene , one needs to retrieve the required sequence information for each single exon and promoter , including their corresponding flanking sequences , convert them to the correct format , switch to a primer design application like primer 3 ( 1 ) , import the sequence information and adjust parameter settings if necessary before submitting the request . while this is tolerable for primer design for a gene that has a single exon , a human , mouse or rat gene can easily have more than 10 exons , and each of the above steps may thus need to be repeated 10 times or more .
furthermore , if an exon is too long to sequence properly in one run , several primers have to be designed to map overlapping sections of the exon .
the whole process requires many manual steps for repeated window opening , browsing , application switching , copying and pasting , typing and so on , which can be tedious , time - consuming and error prone .
in fact , for a gene with 10 exons , the process can easily exceed 300 steps . to improve the situation , we have developed primerz to replace almost all of these manual steps so that users can easily complete a primer design task using just a few clicks for a gene or batched human snps .
more than 2000 primers have been designed with primerz at our institute since 2004 and the success rate is over 70% .
there are a multitude of user - definable options available including product size , maximum exon length , excluded regions of the query sequence , gc - content and maximum allowable local alignment score . simply by submitting a candidate gene name ,
a snp i d or up to 100 batched snp ids , in most cases all the primer sequences should be returned in a minute or two . the generated information , including gene transcript graph , primer data from primer3 , and direct links to ucsc in - silico pcr ( 3,4 ) for pcr product prediction , to ncbi blast and to ensembl source data , is integrated and displayed on a single page for convenient viewing .
additionally , all the primer data can be exported in csv format for further processing .
primerz takes advantage of the well - developed public - domain database ensembl , through its api ( application programming interface ) .
when a gene query is received , primerz will access the ensembl database through ensj api ( 5 ) to retrieve promoter and exon information . by default but with an adjustable setting ,
primerz retrieves one region of 1440 bp upstream from the start of 5-utr which it treats as the promoter region .
the promoter region is thereafter divided into four 360 bp non - overlapping segments plus their respective flanking sequences .
all exons are directly flanked with 240 bp sequences , except when an exon of the gene is > 360 bp , when the sequence will be split into segments of 360 bp for a better quality sequencing result .
for example , an exon of 1000 bp will produce two 360 bp segments and one 280 bp segment . the above sequence information , plus parametric settings packaged by primerz are then fed into primer3 for primer design .
all returned primer results , together with a transcript graph , are integrated into a one - page report for final output .
the self - explanatory workflow of the whole primerz system is shown in figure 1 .
the software development environment included the following software :
java , jdk : j2sdk1.4.2_06 , server vmstruts framework 1.2red hat enterprise linux academic editionmysql 4.1tomcat 5.0 web serverensembl java api java , jdk : j2sdk1.4.2_06 , server vm red hat enterprise linux academic edition tomcat 5.0 web server the api provided by ensembl is used for gene information retrieval .
java language is used to pipeline gene data into primer 3 and merge the returned results .
primerz is an easy - to - use tool to design primers for genes and snps , using only a few simple steps to design the wanted primers .
it currently provides gene primer design for all ensembl species while snp primer design is currently only available for human snps . for gene primer design ,
there are some essential parameters required for optimal design of primers , such as maximum exon length , exon flanking region , product size range and excluded region .
the excluded region value allows the program to bypass regions with low sequence quality or containing repetitive elements such as alus or lines for primer design . for snp primer design , a snp rsid or an affy_probid(6 ) as well as a batch file containing mixed
i d types can be accepted as input , where the maximum number of snps per batch is limited to 100 .
the result page comprises three major parts : initial input data and parameters , the gene transcript diagram ( only available for gene primer design ) and designed primer information .
the first part lists all the input data and parameters plus ensembl database version , for ease of reference .
the second part shows each promoter fragment and exon of a transcript diagram , and links to their accompanying original primer3 output . finally , the third section presents tabulated primer information and incorporates executable links to ncbi blast and ucsc in - silico pcr to check specificity and product prediction .
ensembl link button offers the ensembl exon report of the transcript so that a user can trace all primers to their original sequence information .
a csv format text file of the results can be downloaded at this results page .
in a manual operation benchmarking test , it took a person very familiar with all processes about 1260 s and 380 discrete steps to design the primers of hadhsc ( l-3-hydroxyacyl - coenzyme a dehydrogenase , short chain ) , our benchmark gene with 10 exons .
with automated primerz , the same operation took only 16 s and three steps .
this comparison clearly demonstrates the superior efficiency and ease - of - use of primerz for gene primer design .
a benchmark test on batched snps showed a similar dramatic reduction in workload from 90 discrete steps to two steps , from 840 to 49 s , and from 40 result pages to a single page .
primerz has been designed to obtain reliable primers for pcr experiments and to allow standardized , automated primer design for batch operation .
users can access the ucsc in - silico pcr directly from the result page to verify their primers to achieve higher accuracy and lower cost .
primerz also allows users to modify the conditions of primer design , including the maximum exon size , the flanking region of the target sequence , the exclusion region and the maximum allowed polya and ca - repeats in the pcr products .
in addition , primerz will offer primer design from ncbi transcripts in the near future , which should be of great interest to those users who use ncbi data to design primers .
the results from ncbi and ensembl will be shown in the same page . following the release of primer3 web interface in november 2006 , we are installing and testing a local copy of primer3 web to alleviate the burden on the original primer3 website and the restriction on the number of snps allowed .
primer z is a simple - to - use program that greatly facilitates and enhances the traditionally time - consuming task of accurate primer design for pcr , and should be an excellent additional tool for the modern molecular biologist . | xanthogranulomatous pyelonephritis ( xgp ) is rare and aggressive form of chronic infectious pyelonephritis .
no single clinical or radiological feature is diagnostic of xgp . a 75-year - old man with prostatic enlargement presented with difficulty and burning micturition fever , abdominal and flank pain .
x - ray , ultrasonography and computed tomography scan diagnosis was right kidney pyonephrosis .
intravenous urography revealed non - excretory right kidney .
right nephrectomy was done .
histological diagnosis of xgp was made . in all patients of prostatic
enlargement , renal function must be assessed for the extent of damage .
surgery is the treatment choice in most cases .
pre- and post - operative antibiotics are key factors for successful management and better prognosis . |
synovial chondromatosis is a disease with unknown etiology , originating from synovia and characterized by the presence of metaplastic cartilaginous nodules in the synovial cavities , bursa or tendon sheaths .
the disease is commonly seen in men and between the 3rd and 5th decades of life . although the exact etiology is not known
the knee , hip and elbow joints are frequently reported to be involved by the condition . however , shoulder and ankle joints are involved extremely rarely .
the disease is classified in 3 stages and evaluated according to following criteria : the early stage with intrasynovial differentiation without loose bodies , the transitional stage by intrasynovial cartilaginous nodules with loose bodies and late stage with multiple loose bodies .
the treatment decision is made according to the patient 's age , symptoms and the disease stage .
the main advantages of the arthroscopic approaches are decreased morbidity , synchronous visualization and treatment feature for intra and extra articular pathologies .
the hypertrophic synovia and multiple loose bodies are typical arthroscopic findings . in this case report
, we presented an arthroscopically managed adult patient with anteriorly localized right ankle chondromatosis and discussed the potential benefits of arthroscopic surgery .
a twenty - eight year old male patient was admitted to our hospital with decreased range of motion , swelling and increased pain during movement in the right ankle joint .
he had no history of trauma , systemic inflammatory disease or family history of bone or joint diseases .
the physical examination revealed that he had mild tenderness around the anterior ankle joint on palpation with palpable loose bodies .
multiple nodules 39 mm in diameter with calcifications were located at the anterior aspect of the right ankle on the plain anteroposterior and lateral x - ray images ( fig .
1 ) . magnetic resonance imaging ( mri ) revealed multiple calcified well - circumscribed loose bodies at the same location and synovitis in the ankle joint ( fig .
the laboratory tests were within the normal limits and the patient was scheduled for arthroscopic surgery with the diagnosis of anterior impingement syndrome due to right ankle synovial chondromatosis .
the ankle joint was entered via anteromedial and anterolateral arthroscopic portals during spinal anesthesia and tourniquet application .
multiple loose bodies and hypertrophic synovia around the anterior ankle joint were seen ( fig .
3 ) . arthroscopic partial synovectomy and excision of loose bodies were performed ( fig .
the drain was removed in the 1st postoperative day and the active and passive range of motion exercises was started .
the patient was allowed partial weight bearing with crutches and at the 2nd week he was mobilized with full weight .
there were multiple cartilaginous loose bodies , with the biggest and smallest dimensions of 0.9 cm 0.7 cm 0.5 cm and 0.4 cm 0.3 cm 0.2 cm in the permanent pathology report respectively ( fig .
the patient 's dorsiflexion and plantar flexion degrees were 25 and 30 , respectively , at the end of the 11th postoperative month .
no complications were diagnosed in the follow - up period with no recurrence on the plain x - ray images and mri .
trauma , degenerating joint diseases , osteochondritis dissecans , rhomatoid arthritis and tuberculosis arthritis are examples of the secondary form .
our case was evaluated in the primary synovial osteochondromatosis group due to the absence of previous trauma or inflammatory pathologies .
complaints of pain , swelling of the joint ( especially after physical activity ) with or without accompanying pain , decreased range of motion , palpable mass , locking paresthesias and joint clicking are main symptoms and signs in patients with synovial chondromatosis .
the suspected diagnosis was confirmed by the appropriate radiologic investigations and pathologic examination after history taking and physical evaluation .
the calcified form synovial osteochondromatosis could be seen in the anteroposterior and lateral plain x - ray images .
mri investigation enabled the diagnosis of the disease in the early stage , the exact localization of the disease and intrinsic property of chondroid tissue .
the tenosynovial giant cell tumor , calcifying aponeurotic fibroma , periosteal chondroma , osteocartilaginous loose bodies and soft tissue chondrosarcoma must be kept in mind in the differential diagnosis . the disease is slowly progressive and is considered to be a self - limiting condition . in the early stages of the disease and in asymptomatic patients , treatment can be planned conservatively with frequent follow - up visits .
degenerative changes could occur in the later stages of the disease in patients without appropriate therapy .
although the classical treatment approach for ankle joint chondromatosis is open surgery , arthroscopic surgery is rarely encountered in the literature .
some important advantages of arthroscopic surgery are wide visualization areas , easy access to difficult to reach areas , lower morbidity , no need for casting and immobilization , early rehabilitation and quick recovery period . however , there is the possibility of limited synovectomy and residual loose bodies .
arthroscopic surgery enables wide regional visualization , lowers morbidity , promotes early rehabilitation , shortens the recovery period and decreases the immobilization period .
written informed consent was obtained from the patient for publication of this case report and accompanying images .
a copy of the written consent is available for review by the editor - in - chief of this journal upon request .
nevres hurriyet aydogan , ahmet ozmeric , onur kocadal , murad pepe , talip kara took part in surgical procedure , literature search , preparation of the paper and review .
serap gozel took part in pathological investigation.key learning pointssynovial chondromatosis is a disease originating from synovia and characterized by the presence of metaplastic cartilaginous nodules.pain , swelling of the joint especially after physical activity and decreased range of motion are main symptoms.treatment is arranged according to the patient 's complaints , age and disease stage.there are many advantages of arthroscopic surgery in selected patients .
synovial chondromatosis is a disease originating from synovia and characterized by the presence of metaplastic cartilaginous nodules.pain , swelling of the joint especially after physical activity and decreased range of motion are main symptoms.treatment is arranged according to the patient 's complaints , age and disease stage.there are many advantages of arthroscopic surgery in selected patients .
synovial chondromatosis is a disease originating from synovia and characterized by the presence of metaplastic cartilaginous nodules .
pain , swelling of the joint especially after physical activity and decreased range of motion are main symptoms . the rh1 alloimmunization responsible for the hemolytic disease of the newborn occurs when the rh1-negative mother s blood comes into contact with the foetus s rh1 positive red blood cells . after the passing of foetal red blood cells into the maternal circulation , the rh1 antigens on foetal red blood cells , which are foreign antigens to the maternal immune system , trigger the immunological processes producing anti - rh1 allo - antibodies of the immunoglobulin class igg .
these antibodies cross the placenta , attack foetal red blood cells and lead to a foetal hemolytic anemia .
the immunoprophylaxis by anti - rh1 immunoglobulins has been established since the 1970s , but this disease remains the leading cause of fetal anemia .
the severe forms of hemolytic disease are observed in 10% of fetuses or newborn affected by this disease .
it exposes to fetal complications such as hydrops , hypoxic brain damage and fetal death .
we report a case of dramatic outcome of an observation of severe hemolytic anaemia in a newborn due to rh1 incompatibility , which led to death .
a male newborn presenting the antecedents of consanguinity was admitted 30 minutes of life to the pediatric department of mohammed v military teaching hospital for the issue of hydrops fetalis on rhesus incompatibility ; the birth weight was 1800 g and his blood group was a rh1 .
after birth , the baby was intubated and placed on mechanical ventilation due to respiratory distress and hypoxia .
the blood group of his mother , aged 31 , was ab rh1-negative and that of his father aged 37 was a rh1 .
the mother had a history of 4 term deliveries , 3 abortions , and 1 living child .
, she was sent to gynecology department of mohammed v military teaching hospital after the discovery of fetal ascites .
preterm birth was induced at 30 weeks of gestation by cesarean section under spinal anaesthesia and she was transferred to the medical intensive care unit and the newborn was transferred to the paediatric department .
the laboratory tests of the newborn on the first day of life showed hyperbilirubinaemia ( total serum bilirubin level = 30 mg / l ) , hyperuremia ( 1.03
g / l ) , hyperkalaemia ( 7.2 mmol / l ) and hyponatremia ( 134
the blood count showed bicytopenia with macrocytic regenerative anemia ( hemoglobin = 4g / dl , mean corpuscular volume = 183 fl , reticulocyte count = 176600/l ) associated with thrombocytopenia at 120 000/l .
the blood smear showed erythroblastosis ( 1256 erythroblasts per 100 leukocytes ) , howell jolly bodies , anisocytosis and many macrocytes ( figure 1 ) .
after drainage of the ascites fluid , the newborn was transfused with red blood cell concentrates and was also treated with conventional phototherapy .
the evolution was unfavorable with a steady increase of total serum bilirubin level ( 71 mg / l ) , hemoglobin ( 9.4 g / dl ) , reticulocytes ( 187203/l ) and circulating erythroblasts ( 1386 erythroblasts per 100 leukocytes ) and a decrease in platelet count ( 72 000/l ) on the second day ( table i ) and died three days after the death of his mother , who died from pulmonary embolism in the intensive care unit .
our case report shows that there is rh1 incompatibility between the ab rh1-negative mother and the a rh1 newborn .
the feto - maternal blood incompatibility constitutes the major cause of autoimmune hemolytic anaemia among newborns and must be evoked first before a neonatal anemia with early onset jaundice .
the allo - antibodies of the most common obstetrical interest are anti - rh1 , anti - rh4 and anti - kel1 , representing respectively 35% , 37% and 13% of identified allo - antibodies ; they are responsible for 88% , 8% and 2% respectively for severe fetomaternal incompatibilities .
our patient presented anemia associated with erythroblastosis , howell jolly bodies , many macrocytes and high reticulocytosis showing a very active erythropoiesis , to compensate for the hemolysis .
biological signs of autoimmune hemolytic anemia are regenerative anemia which can be macrocytic or normochromic normocytic anemia , a decrease in haptoglobin , an increase in lactate dehydrogenase related to the importance of hemolysis ; a hemoglobinemia with hemoglobinuria in the case of intravascular hemolysis and sometimes an increase in unconjugated bilirubin and a decrease in the glycated hemoglobin and the direct antiglobulin test is positive in 95% of cases .
the direct antiglobulin test is based on the detection of erythrocytic autoantibodies either in serum , or when they are attached on red blood cells . in this pathology , it is necessary to exclude physiologic jaundice due to newborn s immature liver .
however , the physiologic jaundice of the newborn is never present at birth and appears from the 36th hour to reach a maximum on the 3rd-4th day and disappears before the 10th day .
it is also necessary to eliminate abo incompatibility which is exclusively found in newborns with a or b blood type and whose mothers are o blood type and neonatal jaundice associated with hyperhemolysis due to common congenital hemolytic anemia : red blood cell membrane disorders ( hereditary spherocytosis , hereditary elliptocytosis , and hereditary pyropoikilocytosis ) , red blood cell enzyme defects ( glucose 6 phosphate dehydrogenase deficiency , pyruvate kinase deficiency and other red blood cells enzymopathies ) and neonatal hemolysis due to hemoglobinopathies(-thalassaemia major and -globin and -globin chain structural abnormalities ) .
the cases of polycythemia vera and certain infectious syndromes can also be accompanied by jaundice . in case of prolonged jaundice
the irregular agglutinin test is an important test for pregnancy monitoring as part of the prevention of anti - rh1 alloimmunization and management of feto - maternal incompatibilities .
it aims at detecting and identifying red cell alloantibodies directed against erythrocyte antigens other than a or b of unexpressed abo system on the surface of its own red blood cells capable of inducing , by feto - maternal incompatibility , hemolytic disease in the fetus and/or newborn .
the irregular agglutinin test is done 2 times ( 1st and 6th or 7th prenatal examinations ) in rh1 pregnant women without transfusion history and 4 times ( 1 , 4 , 6 and 7th prenatal examinations ) in rh1 women with a history of transfusion or pregnancy and in the rh1 negative women .
this test is also practiced at childbirth in rh1 negative women before the anti - d immunoglobulin injection .
postnatal management of hemolytic disease of the newborn due to rh1 incompatibility aims at preventing postnatal death from anemia complications and neonatal kernicterus and may include : intensive phototherapy which is the most commonly used treatment and its effectiveness is evaluated by regular monitoring of the concentration of total serum bilirubin , exchange transfusion which is the last resort in the treatment of hyperbilirubinaemia and its adverse effects are numerous : hypocalcaemia and thrombocytopenia , convulsions , necrotizing enterocolitis , apnea , bradycardia , hyperkalemia and hypoglycemia .
the treatment of hyperbilirubinaemia can also be done using intravenous immunoglobulin ( ivig ) ( 0.51 g / kg ) .
a few small randomized controlled trials showed that the use of ivig reduced the need for exchange transfusion , the duration of intensive phototherapy and length of hospitalization , but a randomized controlled trial conducted in the netherlands did not confirm these results .
other drugs which have been proposed in the treatment of neonatal jaundice are : d - penicillamine and metalloporphyrins which inhibit hemeoxygenase and reduce the production of bilirubin , albumin which increases bilirubin transport capacity in the blood and reduces the blood concentration of unconjugated bilirubin , and phenobarbital which increases bilirubin uptake , conjugation and excretion .
blood transfusions may also be needed to correct severe anemia . despite blood transfusion and treatment by intensive phototherapy ,
our patient died four days after his birth with hemolysis , kidney failure , jaundice and hypoxia .
the best treatment is to prevent causal anti - d immunization with intravenous ( iv ) anti - rh1 immunoglobulin in rh1 negative pregnant women to neutralize the foetal red blood cells in the maternal vascular compartment .
when the newborn is rh1 negative , the rhesus is confirmed on the second sample .
if negativity is confirmed , anti - d immunization in mothers is unnecessary . if the newborn is rh1 , the prophylaxis of alloimmunization to rh1 antigen is based on the iv injection of anti - rh1immunoglobulins .
it is necessary first of all to perform the double determination of abo group and the phenotype of rh - kell of the newborn , a direct antiglobulin test on the red blood cells of the newborn , the irregular agglutinin test on maternal serum at childbirth and kleihauer test on maternal blood collected at least one hour after delivery .
the iv injection of anti - rh1 immunoglobulins is carried out within 72 hours at the latest following delivery .
the monitoring of these high - risk pregnancies requires specialized centres and collaboration between the gynecologist and the blood transfusion centre biologist .
hemolytic disease of the newborn related to rh1 incompatibility is rare but serious . in order to avoid this drama ,
it is necessary to strengthen the prevention and clinico - biological monitoring in patients with a history of feto - maternal rhesus alloimmunization by sensitizing and advising all rh1 negative unimmunized women that rh1 prophylaxis should be applied after all birthing of rh1 child and must also always be carried out after any miscarriage . | introductionsynovial chondromatosis is characterized by the presence of metaplastic cartilage nodules originating from the synovia , bursa and tendon sheaths .
although it is extremely rare in the ankle joint , malignant transformation is possible .
the choice of treatment is usually open surgery for excision of loose bodies and synovectomy .
limited data is available concerning arthroscopic approaches.presentation of casea 28-year - old male patient was evaluated for pain and swelling of the right ankle joint . based on the findings of physical examination and radiographic investigations
, arthroscopic surgery was performed due to ankle impingement syndrome .
a diagnosis of synovial osteochondromatosis was made following the pathological survey.discussionsynovial chondromatosis is slowly progressive and is considered to be a self - limiting situation .
treatment strategies are decided on according to the patient 's complaints , age and disease stage .
open or arthroscopic surgery . can be performed .
some advantages of arthroscopic surgery are wide visualization areas , easy access to areas difficult to reach , lower morbidity , no necessity for casting and immobilization , early rehabilitation and quick recovery period.conclusionin conclusion , arthroscopic management can be successful in selected patients with synovial osteochondromatosis localized to the ankle joint . |
the incidence of bladder forming a part of an inguinal hernia is 14% . with correct knowledge of anatomy and careful dissection , injuries to the bladder during hernia repair
we hereby report a case where the patient first presented with a scrotal abscess and vesicocutaneous fistula after surgical repair of sliding hernia .
a 65-year - old morbidly obese man underwent mesh hernioplasty for large right inguinal hernia . after removing the urethral catheter , he developed gradually increasing right scrotal swelling with fever .
thereafter , he developed continuous urine leakage from the site of incision and drainage , figure 1 .
we carried out a cystogram via the urethral catheter that revealed a fistulous communication between bladder and scrotal skin , figure 1 .
, cystoscopy revealed normal anterior and posterior urethra , non - obstructing prostatic lobes and a defect in the anterior bladder wall with no evidence of mesh erosion .
almost the whole of the bladder was lying in the right scrotum and densely adherent to the right testis and cord structures and mesh
. there was a fistulous opening at the dome of the bladder wall well away from the mesh .
our main concerns were inguinal hernia repair and creation of extraperitoneal space to reposition the bladder in the normal position , which was not possible without performing right high inguinal orchiectomy .
hence , we performed right high inguinal orchiectomy and removal of mesh and extraperitoneal space was made to reposition the urinary bladder to its normal position .
fistula opening was repaired in two layers and the bladder was put on continuous drainage via 20 french urethral catheter , figure 2 .
post - operatively at 2 weeks , there was no urinary leak on cystogram and the urethral catheter was removed and normal voiding was restored .
scars of previous surgery with vesicocutaneous fistula and cystogram showing contrast in the left hemiscrotum the entire urinary bladder lying in the scrotum , with the bladder re - positioned into the normal position
levine coined the term scrotal cystocoele in 1951 for inguinoscrotal herniation of the bladder .
urinary bladder herniations are usually diagnosed at the time of inguinal herniorraphy and are commonly repaired through the same incision .
they are sometimes found incidentally during the evaluation of a patient with lower urinary tract symptoms and associated inguinal hernias .
two - stage micturition is the classical symptom , with the second stage facilitated by some form of external pressure on the bladder .
the para - peritoneal type is the most common type and the extra - peritoneal type is the least common .
because imaging all patients with large hernias may not be cost - effective , imaging studies are performed only when bladder herniation is suspected .
the diagnostic triad of lateral displacement of the distal one - third of the ureter , small asymmetric bladder and incomplete visualization of the bladder base on an intravenous urogram has been described by reardon and lowman .
iatrogenic injury to the bladder during hernia repair can be due to multiple factors , such as an inexperienced surgeon in the early part of the learning curve or an obese patient with large hernial sac with unrecognized bladder component . in our patient
, there could have been an injury to the bladder that was not recognized at the time of hernia repair , which led to subsequent scrotal abscess formation resulting in a vesicocutaneous fistula .
if unrecognized , these usually present immediately after catheter removal , but presentation can sometimes be delayed in case the fistula is very small and there is no infravesical obstruction .
management includes immediate repair in case it is recognized intraoperatively . in case of unrecognized injury and with delayed presentation , the first step is to put a wide caliber per urethral catheter followed by thorough evaluation with urine culture examination and cystogram . a small fistula can be healed with only continuous bladder drainage with per urethral catheter or preferably suprapubic cystostomy , provided lower tract infravesical obstruction has been ruled out . a larger fistula needs open surgical management .
careful dissection is needed in the extraperitoneal space while separating the sac from cord structures as the bladder forms a part of the posterior wall of the sac . after completing bladder dissection ,
the fistula is repaired in two to three layers and an adequate space is created in the extraperitoneal plane to reposition the bladder .
sometimes , large hernias could be treated by resection of the herniated bladder as described by thomas and gomella . in our case , the whole bladder was lying in the scrotum and extensive adhesions were present between the cord structures and the bladder .
high inguinal orchiectomy was performed to create space for the bladder and for proper closure of the inguinal canal .
urinary bladder rarely forms a part of an inguinal hernia and , with the correct knowledge of anatomy and careful dissection injuries to the bladder during hernia repair , surgery can be prevented .
surgically creation of an extraperitoneal space for bladder repositioning is of paramount importance , sometimes needing inguinal orchiectomy in the elderly . prostate cancer ( pca ) is the fifth - most - common cancer among men in singapore , with an age - standardized rate of 17.4 cases per 100,000/y , and the incidence has been increasing steadily over the past 35 years .
the average annual rate of increase between 1968 and 2002 was 5.6% , with the past 10 years showing a somewhat steeper increase .
pca could become a major public health issue with the aging of our country 's population . during the past decade
, a considerable number of modifications have been made to improve the technique of pca biopsy .
total prostate volume is also an important factor , and higher pca detection rates have been reported in men with smaller prostates .
the current concept regarding prostate biopsy is that systematic sextant biopsies , even when directed laterally , do not provide adequate prostate sampling .
several extended biopsy techniques have been introduced to improve the pca detection rate compared with that of systematic sextant biopsy .
these techniques vary in the number of cores taken and the location from which samples are obtained , but none have taken into consideration the age of the patient or the volume of the prostate gland .
life expectancy is based on patient age and has a pivotal role in diagnosis and treatment . over - diagnosis of clinically insignificant pca
is considered a major potential drawback of prostate - specific antigen ( psa ) screening , especially in older patients .
pca volumes to be detected can be larger in older patients , and thus fewer cores are needed , which reduces over - diagnosis of tumors ( insignificant pca ) at biopsy . on the basis of the above information and using data from the european prostate cancer detection study , the use of the vienna nomogram ( vn ) prostate biopsy model was developed .
this model indicates the optimal number of cores based on patient age and total prostate volume .
thus , the use of the vn should result in higher pca detection rates , especially in younger patients and those with larger prostates , and , at the same time , should avoid the detection of insignificant cancers , especially in older patients .
, we used the vn to determine the efficacy of this model in the detection of pca in our local population .
we also assessed the incidence of complications due to the use of such a template .
with approval from our institutional review board , 120 men were enrolled prospectively between january 2006 and june 2007 .
the study population consisted of consecutive referrals for evaluation of elevated psa scores ( > 4 ng / ml ) and/or abnormal digital rectal examination ( dre ) findings .
all patients underwent transrectal ultrasound ( trus ) examination of the prostate , which was followed by prostatic biopsies .
patients were excluded from the study if they had a history of pca , acute or chronic prostatitis , histologic evidence of prostatic intraepithelial neoplasia of any grade , urinary retention , indwelling urinary catheter , or confirmed urinary tract infection . before the procedure ,
patients were observed for a minimum of 4 hours after the procedure for immediate complications and were given advice to return to hospital if they had delayed complications . in each of the 120 patients ,
6 to 18 cores were taken from the peripheral zone for trus - guided biopsy , as indicated by the vn ( table 1 ) .
each biopsy core was labeled according to location on the prostate and was sent separately for histologic review .
biopsy tissue was considered positive if adenocarcinoma was diagnosed , and the number of positive cores , the gleason score , and the grade were reported .
all other findings ( high - grade prostatic intraepithelial neoplasia , atypia , and dysplasia ) were considered negative . in this study ,
trus - guided biopsy performed according to the vn protocol was restricted to the first biopsy .
further management was dependent on individual urologists , and complication rates were subsequently updated by chart reviews .
chicago , il , usa ) and stratified for age , psa , and trus findings .
the patients ' mean age was 62.68.3 years ( range , 40 - 86 years ) .
the mean psa score was 13.42 ng / ml , and the mean number of cores obtained was 9.683.1 . according to the vn , 27 out of a total of 120 patients had pca , for a detection rate of 22.5% . in the group of patients with psa scores
< 10 ng / ml , the detection rate was 14.9% ( 14 of 94 patients ) .
the group of patients with psa scores > 10 ng / ml had a detection rate of 50% ( 13 of 26 ) .
histopathologic features presented on prostate biopsy in 27 pca patients whose gleason scores were 3 + 3 , 3 + 4 , 4 + 3 , 4 + 4 , and 4 + 5 in 11.1% ( 3 patients ) , 37.0% ( 10 ) , 29.6% ( 8) , 18.5% ( 5 ) , and 3.7% ( 1 ) respectively ( fig .
two of these cases were diagnosed on repeat trus biopsy , and two were discovered on transurethral prostatectomy , for a false - negative rate of 3.3% .
four patients ( 3.3% ) had bleeding per rectum : one of them required adrenaline injection , another required hemostasis under spinal anaesthesia , and the other two had rectal bleeding that resolved spontaneously without further intervention .
detection rates of pca have varied , and review of the literature from several asian countries found that the detection rate of pca in patients with raised psa scores is in the range of 14.6% to 26.5% . the austrian study that used the vn had a detection rate of 36.7% ( table 2 ) .
data from other countries in asia uniformly reported a low cancer detection rate in connection with psa scores ranging from 4 to 10 ng / ml . in comparison ,
the detection rate of pca with psa scores ranging from 4 to 10 ng / ml has always been about 25% in western countries .
the low positive predictive value of elevated psa scores in asian countries may be due to the low incidence of pca in this geographic area , but it also may be partly due to inadequate sampling .
recent reports in the literature have queried the adequacy of sextant biopsy for the detection of small nonpalpable pca . in our study , we used the vn , and the positive predictive value was 22.5% , a value that is comparable to other published data from asian countries .
the key to higher pca detection rates with the use of the vn is varying the number of cores according to prostate volume . because larger prostate glands can result in more sampling errors during biopsy
the studies by remzi et al . and ung et al . stressed the importance of prostate volume in pca detection and showed that detection rates are , in fact , dependent on prostate volume .
. also found that the most important factor in a failure to diagnose pca at the primary screening was a large prostate volume .
evaluating the variation of pca detection in relation to prostate size through random systematic sextant biopsies , uzzo et al
. found that 23% of the patients had pca in a large prostate ( > 50 ml ) , whereas 38% of patients with smaller prostates had pca ( p<0.01 ) .
although a study by lecuona and heyns found that the detection rates for the vn and an eight - core prostate biopsy were similar , the detection rate in the vn group was higher for patients with larger prostate volume ( > 50 ml ) . thus , the vn could be useful in patients with larger prostates in which potential undersampling could occur .
the rationales for using age as one of the parameters to determine the number of biopsy cores taken are twofold : first , younger men have a longer life expectancy , which makes even smaller cancers clinically significant , and , thus , more cores are needed ; and , second , older men may require fewer biopsy cores to avoid oversampling and overtreatment . if the time to critical pca volume is less than life expectancy , there might be no need for pca detection , as these pcas will be clinically insignificant .
standard sextant biopsies have reported high false - negative rates of 15% to 28% . by combining systematic and target sampling ,
the advantages of using a vn are , first , higher pca detection rates , especially in younger patients and larger prostates ; second , at the same time , the avoidance of detection of insignificant cancers , especially in older patients ; and , third , the fact that it provides urologists with a clear and fixed number of biopsy cores on the basis of patient age and prostate volume . in our study , the complication rate was low ( 7.5% ) .
this rate was comparable to the rates in large - scale studies looking at morbidities of trus prostatic biopsy .
moreover , our complication rate was low despite our having performed more biopsies on each patient ( than we would have with the sextant biopsy ) .
with the use of the vn , our pca detection rate of 22.5% is comparable to published data for asian patients .
the nomogram offers an easy tool with which to select the optimal number of prostate biopsy cores on the basis of patient age and total prostate volume . with this biopsy strategy | sliding inguinal hernias are usually direct inguinal hernias containing various abdominal viscera .
the incidence of bladder forming a part of an inguinal hernia , called as scrotal cystocele ,
is 14% .
the risk of bladder injury is as high as 12% when repairing this type of hernia .
this case report emphasizes this aspect in a 65-year - old man who presented with urinary leak through the scrotal wound following right inguinal hernia repair . |
holt - oram syndrome ( hos ) is an autosomal dominant condition with complete penetrance . manifested in 1:1 , 00 , 000 live births and characterized by forelimb deformities , congenital heart disease and/or cardiac conduction abnormalities .
it is linked to a single - gene tbx5 protein - producing mutation with gene map locus 12q24 and is the most commonly occurring heart - hand syndrome .
congenital cardiac and upper limb malformations frequently occur together and are classified as heart hand syndromes .
the most common among the heart hand disorders is hos , which is characterized by cardiac septation defects and preaxial radial ray abnormalities .
this condition with a high rate ( 3085% ) of new non - familial cases was first described by holt and oram in 1960 in a 4-generation family with atrial septal defects ( asd ) and thumb abnormalities .
the most common cardiac disorder is an ostium secundum asd , followed by ventricular septal defect ( vsd ) and ostium primum asd .
electrocardiogram ( ecg ) abnormalities such as various degrees of atrioventricular ( av ) block have also been reported .
a full term female neonate born out of a nonconsanguineous marriage by cesarean section ( indication - previous cesarean section with polyhydramnios ) to a 25-year - old ( weight 58 kg , height 155 cm ) booked g3p1l1a1 with unremarkable antenatal history .
family history revealed that the father has radial ray deformity of left upper limb without any cardiac anomaly .
physical examination revealed an active baby weighing 2790 g and length of 49 cm , heart rate of 146/min , blood pressure of 70/30 mm of hg , respiratory rate of 40/min , and systemic oxygen saturation of right upper limb being 83% in room air and that of right lower limb being 74% in room air [ figure 1 ] . on musculoskeletal examination , left upper limb shortening was noticed with absent radius bone , radial flexion deformity of the wrist and also absent thumb [ figure 2 ] .
no obvious deformities were noticed elsewhere . on cardio - vascular system examination , the pansystolic murmur of grade iii at the mitral and left parasternal area was heard
picture showing the baby of holt - oram syndrome left upper limb showing radial ray deformity with absent thumb right hand showing triphalangeal thumb on further investigation , chest x - ray showed normal thoracic situs with cardiomegaly , plain radiograph of both upper limbs revealed absent radius on left side with absent carpal bones and absent first metacarpal bone and phalanges ( thumb ) , right side showing absent carpal bones and triphalangeal thumb [ figure 4 ] .
plain radiograph showing the bony deformities of the upper limb with cardiomegaly the baby developed cyanosis couple of hours after delivery , following which an ecg was done which was normal and a 2d echocardiography was done which revealed severe aortic atresia with hypoplastic arch , large perimembranous vsd and asd as well [ figure 5 ] .
the neonate was referred to a cardiac center for further management , however due to lack of resources the baby died on day 4 of life .
holt - oram syndrome is an autosomal dominant disorder characterized by distinctive malformation of bones of the upper limbs and abnormalities of the heart .
cardinal manifestations of hos are dysplasia of upper limb that ranges from minor findings including hypoplasia of thumb , clinodactyly , brachydactyly , triphalangeal thumbs , carpal bone dysmorphism , shortness of ulna , shortness of humerus , aplasia of radius to phocomelia and cardiac abnormalities .
although bilateral , left side is often affected more significantly . in a study of 98 subjects with hypoplastic thumbs , 16% proved to be the cases of hos .
there are many well described heart - hand syndromes characterized by deformities of the radial ray and congenital heart defects such as thrombocytopenia absent radius syndrome , roberts syndrome , thalidomide embryopathy , and fanconi anemia .
the unique feature that helps to differentiate these from hos is that the radial aplasia is associated with hypoplasia / absence of the thumb without any hematological abnormalities and there is often a family history of heart and limb defects .
the associated congenital heart defects are the most important determining factors in morbidity and mortality in these patients .
other cardiac associations include pulmonary stenosis , mitral valve prolapse and arrhythmias in the form of atrioventricular blocks .
more complex cardiac lesions such as tetralogy of fallot , endocardial cushion defects , and total anomalous pulmonary venous return are observed in 18% of subjects with hos .
the association with aortic atresia is extremely rare . as per best of our knowledge ,
till date there were no cases reported in the literature having a description of hos with aortic atresia .
the electrocardiographic abnormalities such as variable degree of av blocks have also been reported , but in our case no conduction defects were noted .
the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s )
has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal .
the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed .
the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s )
has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal .
the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . though limited in efficacy in many cases , the control methods available today represent a major progress when compared to the lack of any means for the control of these plants one or two decades ago .
crops can be protected by resistance , by selective fungicides , by biocontrol agents , and by cultural methods that did not existed before . the current focus in applied
breeding is leveraging biotechnological tools to develop more and better markers to allow marker assisted selection with the hope that this will speed up the delivery of improved cultivars to the farmer . to date , however , progress in marker development and delivery of useful markers has been slow in legumes .
we are now also facing an accelerated progress in the genomic and biotechnological research , which should soon provide important understanding of some crucial developmental mechanisms in both the parasites and their host plants and will provide candidate genes for resistance to ascochyta blight .
the application of ngs technologies will provide a new research framework and molecular tools to be applied in resistance to ascochyta blight in legumes .
the authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest . | holt - oram syndrome ( hos ) is a rare autosomal dominant disorder that causes abnormalities of the upper limbs and heart .
it is seen in 1:1 , 00 , 000 live births .
it is linked to a single - gene tbx5 protein - producing mutation with gene map locus 12q24 .
most commonly it is characterized by the cardiac septation defects and pre - axial radial ray abnormalities .
we are reporting a case of hos with aortic atresia which is a rare association . |
marginal zone b - cell malignant lymphoma is a low - grade malignant non - hodgkin 's lymphoma that develops in mucosa - associated lymphoid tissue ( malt ) .
this disease frequently develops in the stomach and also occurs in the salivary gland , thyroid , and lung .
most cases are diffuse large b - cell lymphoma , and the incidence of hepatic malt lymphoma is low among cases of primary hepatic malignant lymphoma [ 2 , 3 , 4 , 5 ] . here
, we describe a rare case in which a lesion was initially thought to be a single tumor but was ultimately diagnosed as 2 contiguous tumors ( malt lymphoma and hemangioma ) using contrast - enhanced ultrasonography ( ceus ) with sonazoid ( daiichi sankyo , tokyo , japan ) .
the patient was a 60-year - old female in whom a tumor of 15 mm in diameter was detected in the couinaud 's segment ( s6 ) of the liver on the grayscale us in a medical examination .
she had no subjective symptoms , relevant medical or family history , and did not drink alcohol .
physical findings on admission were : blood pressure 136/80 mm hg , pulse rate 80/min , and body temperature 36.4c .
blood tests on admission showed hb 10.4 g / dl ( normal 14.017.0 g / dl ) , suggesting a mild anemia .
tumor markers were normal , tests for hepatitis b virus ( hbv ) and hepatitis c virus ( hcv ) were negative , and there were no other abnormal findings .
grayscale us showed a tumor with a snowman - like appearance and a relatively clear boundary in the s6 of the liver , with hypo- and hyperechoic areas in the lateral and medial parts of the lesion , respectively ( fig .
the tumor had a pale , low - density area on unenhanced ct , and prolonged enhancement in the equilibrium phases ( fig .
, the whole lesion gave a low - intensity signal on t1-weighted imaging , but isointensity in the lateral part and high intensity in the medial part were seen on t2-weighted imaging .
similarly , the lateral part showed high intensity and the medial part had a higher intensity on heavy t2-weighted imaging ( fig .
the lateral part was enhanced in the arterial phase , and enhancement persisted in the portal phase .
in contrast , the medial part was gradually enhanced in the arterial phase , compared to the portal phase .
ceus was performed using an aplio xg ( toshiba medical systems , tokyo , japan ) with a convex probe ( pvt-375bt , 3.75 mhz frequency ) .
the mechanical index for the acoustic output was set to 0.2 , and a single focus point was set at the lower margin of the lesion .
sonazoid ( 0.5 ml ) was injected into the left cubital vein followed by a flushing with 10 ml of normal saline .
the lateral hypoechoic region was homogenously hyperenhanced in the vascular phase ( 040 s ) early after injection , and the contrast medium was washed out after about 30 s. the medial hyperechoic region was gradually stained from the margin toward the central region ( fig .
the tumor showed a defect in both hypo- and hyperechoic regions in the post - vascular phase ( after 15 min ) .
similar findings were observed after a second intravenous injection of 0.5 ml of sonazoid using defect reperfusion imaging in the postvascular phase .
liver hemangioma was suspected for the medial part of the lesion based on the typical contrast findings on mri and ceus . in the lateral part ,
the contrast medium was washed out in the vascular phase on ceus early after the intravenous injection of sonazoid .
furthermore , the lateral part showed a defect in the postvascular phase , and this defect led to the suspicion of a malignant tumor , including hepatocellular carcinoma ( hcc ) .
thus , surgical resection was performed . in a macroscopic examination of the resected specimen ,
the medial part was whitish and the lateral part was yellowish - white . on hematoxylin and eosin
( he ) staining , the medial part comprised blood vessels formed by a single layer of flattened endothelial cells and an interstitium formed by thin connective tissue , with the vascular lumen filled with blood .
based on these findings , the medial part of the tumor was diagnosed as hemangioma . in the lateral part ,
lymphocyte infiltration in a dense arrangement was observed on he staining , and most lymphocytes contained a moderately sized nucleus , but some cells contained a large nucleus and noticeable nucleolus ( fig .
similar findings were present in the germinal center , with atypical lymphocytes invading the germinal center . based on these findings ,
the lateral part of the tumor was diagnosed as marginal zone b - cell lymphoma .
isaacson and wright first proposed the name of malt lymphoma for extranodal malignant lymphoma of marginal zone b - cell origin in 1983 .
malt lymphoma is a low - grade malignant non - hodgkin 's lymphoma that develops in mucosa - associated lymphoid tissue , and accounts for 78% of all cases .
malt lymphoma frequently develops in the stomach , and also occurs in the salivary gland , thyroid , and lung .
primary hepatic malignant lymphoma is rare , with most cases being diffuse large b - cell lymphoma and less than 10% being malt lymphoma [ 2 , 3 , 4 , 5 ] .
many cases of malt lymphoma are solitary , imaging findings are diverse , and it is difficult to make a definite diagnosis based on imaging alone .
exclusion of hcc may not be possible and a definite diagnosis can only be made histopathologically after surgical resection in many cases [ 8 , 9 , 10 ] . in our patient ,
the lesion was initially considered to be a single tumor , but imaging findings indicated that it had 2 distinct regions .
a literature search indicated that 2 cases of simultaneous malt lymphoma and hemangioma in the liver have been reported , with focal nodular hyperplasia also present in 1 of these cases [ 11 , 12 ] . in both previous cases ,
malt lymphoma and hemangioma were separate , and thus there has been no previous case in which the tumors initially appeared to be a single tumor . in our patient ,
malt lymphoma and hemangioma were in contact , but each tumor was independent , rather than pathologically continuous , on histopathological examination .
concomitant malt lymphoma and hemangioma were considered to have no causal relationship in the 2 previous cases [ 11 , 12 ] .
the characteristic imaging findings of hepatic malt lymphoma are nonspecific , but include a relatively hypoechoic mass without a clear hypoechoic margin on grayscale us , hypoenhancement of the tumor in the arterial phase on dynamic ct , and low and high intensities on t1- and t2-weighted images on mri , respectively . in ceus
using sono view ( bracco , milan , italy ) in 2 patients with hepatic primary malt lymphoma , foschi et al .
found that the lesions were inhomogeneously hyperenhanced in the arterial phase and hypoenhanced in the portal and late phases .
these 2 patients were hbv - positive : one was an hbv - inactive carrier , and the other had chronic hbv hepatitis .
dynamic ct in both patients showed a slight hyperenhancement in the arterial phase , and hypoenhancement in the portal phases , and hcc was suspected .
it was difficult to make a preoperative diagnosis , and the tumor was finally diagnosed histopathologically as hepatic malt lymphoma .
the development of a malt lymphoma is thought to involve persistent chronic inflammation , and helicobacter pylori infection is well - known in gastric malt lymphoma . in primary hepatic malt lymphoma
, chronic liver disorders such as hbv- or hcv - associated chronic hepatitis , hepatic cirrhosis , and primary biliary cirrhosis are occasionally found in the background liver .
our patient was negative for viruses and the background liver was normal , but homogenous hyperenhancement was observed in the vascular phase early after sonazoid injection , and the contrast medium was washed out after about 30 s on ceus .
a defect was also noted in the postvascular phase , based on which the possibility of a malignant tumor , including hcc , could not be ruled out .
however , the presence of 2 contiguous tumors was indicated by real - time evaluation hemodynamics in the tumor using ceus , which indicates the utility of ceus for a proper diagnosis
. tumor penetration by existing blood vessels on dynamic ct and mri is a characteristic finding in hepatic malignant lymphoma [ 8 , 10 ] .
however , in our case , no blood vessel penetrating the tumor was evident in any imaging .
this feature may not have been visualizable in our case , or the absence of blood vessels penetrating the tumor may differentiate primary hepatic malt lymphoma from other malignant lymphomas
. only a few reported cases of primary hepatic malt lymphoma have included ceus findings , and no typical enhancement pattern on ceus has been established .
the presence of blood vessels penetrating the tumor is useful for the diagnosis of malignant lymphoma , and ceus evaluation of intratumoral hemodynamics in real time may be more likely to visualize penetrating blood vessels , compared to dynamic ct and mri .
it is possible that the absence of this feature differentiates primary hepatic malt lymphoma from other malignant lymphomas .
confirmation of this possibility will require further evidence from ceus evaluation of more cases of primary hepatic malt lymphoma .
the lesion in our patient was initially considered to be a single tumor , but contiguous hepatic malt lymphoma and hemangioma were actually present .
the concomitant occurrence of these tumors is rare , and no cases with 2 tumors in contact have been previously reported .
the tumors gave different enhancement patterns on ceus and their presence was confirmed histopathologically , based on which we were able to make the final diagnosis . this study was conducted in tugela ferry , south africa , where tb incidence is 1,100 cases/100,000 population and > 80% of tb
mdr tb and xdr tb incidence was 118 cases and 72 cases/100,000 population , respectively , in 2007 ( 4 ) .
ethical approval for this study was obtained from albert einstein college of medicine , yale university , university of kwazulu - natal , and the kwazulu - natal department of health .
we performed a prospective cross - sectional study actively identifying patients with suspected tb in medical and tb wards , the hiv clinic , and the outpatient department at the tugela ferry district hospital during february 2008april 2009 .
a person with suspected tb was defined as someone having a self - reported cough of any duration or > 2 other signs or symptoms , including fever , night sweats , weight loss , or shortness of breath for any duration .
patients could be either newly manifesting tb symptoms or have been receiving tb treatment for > 2 months but currently reporting active tb symptoms ( i.e. , treatment failures ) .
sputum for this study was tested by microscopic analysis of auramine- and ziehl - nielsen stained smears and middlebrook 7h11 agar and mycobacterial growth indicator tube 960 broth culture .
dst of positive cultures was performed by using the 1% proportional method on middlebrook 7h11 agar for isoniazid ( critical concentrations : isoniazid 0.2 g / ml , rifampin 1.0 g / ml , ethambutol 7.5 g / ml , streptomycin 2.0 g / ml , ofloxacin 2 g / ml , kanamycin 5.0 g / ml , capreomycin 10 g / ml , and ethionamide 5.0 g / ml ) .
dst was repeated on all drug - resistant isolates to confirm the observed resistance pattern .
the proportion of patients with xdr tb and drug - susceptibility patterns were described by using simple frequencies .
xdr tb treatment outcomes were reported as of november 2009 ; standard international definitions were used ( 11 ) .
of 912 enrolled patients with suspected tb , 209 ( 23% ) had culture - positive tb ( figure 2 ) .
of these patients , 30 ( 14% ) had mdr tb , of which 19 ( 63% of those with mdr tb ; 9% with culture - positive results ) had xdr tb .
determination of prevalence of tuberculosis ( tb ) and drug resistance among persons with suspected tb , tugela ferry , south afica , 20082009 .
dst , drug susceptibility testing ; mdr tb , multidrug - resistant tb ; xdr tb , extensively drug - resistant tb . among xdr
tb isolates , all 19 ( 100% ) were resistant to all 6 drugs routinely tested in kwazulu - natal province ( isoniazid , rifampin , ethambutol , streptomycin , ofloxacin , and kanamycin ) , which extended the trend seen in previous years toward increasing drug resistance ( figure 1 ) . of these isolates , 4 ( 21% )
were also resistant to capreomycin , and 13 ( 68% ) were resistant to capreomycin and ethionamide ( table 1 ) .
thus , an 8-drug resistance pattern was the predominant dst type among xdr tb patients in this cohort . * xdr tb , extensively drug - resistant tuberculosis ; inh , isoniazid ; rif , rifampin ; emb , ethambutol ; sm , streptomycin ; ofl , ofloxacin ; km , kanamycin ; cap , capreomycin ; eto , ethionamide . of 13 patients with 8-drug resistance xdr tb , 5 ( 38% ) were women ( median age 33.5 years , range 2451 years ) ( table 2 ) .
although 5 ( 38% ) had previously received ( or currently showed failure to ) first - line tb treatment , none had ever received treatment with second - line drugs for mdr tb .
twelve ( 92% ) patients were hiv infected ( median cd4 cell count 183.5 cells / mm , range 22670 cells / mm ) ; only 2 ( 17% ) were receiving antiretroviral therapy at the time of tb screening . *
mdr tb , multidrug - resistant tuberculosis ; xdr tb , extensively drug - resistant tb .
6-drug resistance , resistance to isoniazid , rifampin , ethambutol , ofloxacin , kanamycin , and streptomycin ; 7-drug resistance , resistance to isoniazid , rifampin , ethambutol , ofloxacin , kanamycin , streptomycin , and capreomycin ; 8-drug resistance , resistance to isoniazid , rifampin , ethambutol , ofloxacin , kanamycin , streptomycin , capreomycin , and ethionamide .
first - line drugs used for treatment of persons with new tb cases or confirmed drug - susceptible tb include isoniazid , rifampin , ethambutol , and pyrazinamide .
second - line drugs used for treatment of persons with confirmed mdr tb include ofloxacin , kanaymycin , ethionamide , p - aminosalicylic acid , and cycloserine or terizidone . among 13 xdr tb patients with 8-drug resistance , 7 ( 54% ) died ( median time to death 59 days , range 16205 days ) .
two patients were lost to follow - up , and 4 ( 31% ) are still living and receiving xdr tb treatment ( range 190502 days of follow - up ) . no trend in survival of patients with xdr tb was observed by drug - resistance pattern ( 6-drug vs 7-drug vs. 8-drug ) .
routine drug - resistance surveillance to first- and second - line drugs is conducted in tugela ferry , which has a high incidence of tb and hiv co - infection . in this study
, we expanded second - line testing for 2 additional bactericidal drugs ( capreomycin and ethionamide ) for treatment of patients with xdr tb .
resistance to 8 first - line and second - line drugs is the predominant pattern for xdr tb in tugela ferry , thereby severely limiting effective therapeutic options with available medications .
according to the standard xdr tb regimen used in this province , patients were receiving <3 active drugs ( pyrazinamide , p - aminosalicylic acid , and cycloserine ) , which increases the risk for treatment failure and further amplification of drug resistance .
these findings underscore the need for routine surveillance for resistance to all first - line and second - line drugs used and for tailoring regimens accordingly to improve treatment success and reduce emergence of more drug - resistant xdr tb strains .
first , the reliability of second - line dst is variable , and only recently have methods and critical concentrations been standardized ( 12 ) .
however , all drug - resistant isolates in this study had dst repeated to confirm observed results .
second , dst for other first - line drugs , such as pyrazinamide , and other second - line drugs was not conducted , although these drugs are often used for xdr tb treatment .
thus , the degree of drug resistance was likely to be only a minimum estimate .
third , although the proportion of xdr tb cases in this survey was high , the absolute number of xdr tb cases was low .
this small sample size limits our ability to make conclusions about treatment outcomes for patients with increasing drug - resistant isolates . however , previous studies from our site have shown poorer survival rates with increasing drug resistance ( 4 ) . expanded dst for second - line and third - line drugs is critical for xdr tb patient care . given
continued high and rapid number of deaths from xdr tb , better and more rapid methods for second - line dst are urgently needed to improve diagnosis and guide treatment .
although new drugs are being developed , efforts must target prevention of xdr tb and its transmission , earlier identification of cases , support of treatment completion for tb and mdr tb , and greater use of antiretroviral therapy for patients who are co - infected with hiv . | primary hepatic marginal zone b - cell malignant lymphoma of mucosa - associated lymphoid tissue ( malt lymphoma ) is extremely rare .
we present a case in which a lesion was diagnosed as 2 contiguous tumors ( malt lymphoma and hemangioma ) using contrast - enhanced ultrasonography ( us ) with sonazoid .
there has been no previous case of contiguous hepatic malt lymphoma and hemangioma .
the present case was a female with no medical history .
we detected a snowman - like appearance , which was a tumor of 15 mm in diameter with hypo- and hyper - echogenicities in the lateral and medial parts , respectively , in the couinaud 's segment ( s6 ) of the liver on us .
the tumor appeared as a single lesion with a low - density area in the unenhanced phase and prolonged enhancement in the equilibrium phases on dynamic ct . on mri , the whole lesion showed a low - intensity signal on t1-weighted imaging , but isointensity in the lateral part and high intensity in the medial part were seen on t2-weighted imaging .
on contrast - enhanced us , the lateral hypoechoic region was homogenously hyperenhanced in the early vascular phase , and the contrast medium was washed out after about 30 s ; in contrast , the medial hyperechoic region was gradually stained from the margin toward the central region .
the tumor showed a defect in both hypo- and hyperechoic regions in the postvascular phase .
hemangioma was suspected for the medial part based on the typical image findings , but the lateral part was not given a diagnosis .
thus , surgical resection was performed .
the medial part was a hemangioma , and the lateral part was a malt lymphoma by histopathological findings . |
proteus syndrome ( ps ) is a rare and sporadic disorder that causes postnatal overgrowth of tissues in a mosaic pattern .
the complications of ps include , progressive skeletal deformities , invasive lipomas , benign and malignant tumors , and deep venous thrombosis with pulmonary embolism .
we report a rare case of ps that presented with hypertrophy of index and middle finger without any other abnormalities or complications .
incidentally we noticed that he had enlarged index and middle fingers of both hands and thumb of right hand [ figure 1 ] . on probing patient revealed that it was present since childhood with onset around the age of 5 years and gradual progression over years to the present size .
no similar tissue growth in other parts of the body and there was no one in the family with similar features . on examination
hypertrophy of index and middle finger of both the hand ( a , b , c ) and thumb of the right hand ( c ) laboratory investigations revealed normal renal and liver function tests .
his x - ray of hands showed hyperostosis of involved fingers [ figure 2 ] .
x - ray hands showing hyperostosis of both index and middle fingure ( a , b ) and thumb of right hand ( b )
proteus who had the ability to change his shape and was proposed by wiedemann , et al . in 1983 .
happle , et al . in 1987 hypothesized that the syndrome might be due to somatic alteration of a gene leading to mosaic effects that would be lethal if the mutation were carried in nonmosaic fashion . the dysregulated tissue growth in mosaic pattern results in various phenotypic presentations and hence the clinical manifestations of ps are highly variable .
the tissue overgrowth is usually absent or mild at birth and progressive in nature but usually appears to plateau after adolescence .
the disproportionate overgrowth of tissue is usually asymmetrical and involves the arms , legs , hands , feet , and digits .
characteristic manifestations include hyperostoses , often near epiphyses with associated impaired mobility and cerebriform connective tissue nevus seen most commonly on plantar surface .
other findings are lipomas , epidermal nevi and capillary vascular malformations [ table 1 ] .
criteria for the diagnosis of proteus syndrome there is no specific molecular marker , or laboratory test , for the diagnosis of ps . the diagnosis is mainly based on history , clinical examination and imaging studies .
because of its variable presentation , ps may be confused with other conditions . the two disorders most commonly confused with ps are klippel - trenaunay syndrome and hemihyperplasia / lipomatosis syndrome .
the important points in ps that help in the differential diagnosis are : differential diagnosis of proteus syndrome
sporadic and progressive nature of tissue overgrowththe absent or mild tissue growth at birthabsence of bone tumor , enchondromasabsence of specific gene mutations differentiates from neurofibromatosisabsence of familial inheritance ( postzygotic somatic mutation of genes )
sporadic and progressive nature of tissue overgrowth the absent or mild tissue growth at birth absence of bone tumor , enchondromas absence of specific gene mutations differentiates from neurofibromatosis absence of familial inheritance ( postzygotic somatic mutation of genes ) there are no effective treatment modalities for ps . the patients should be followed up regularly for development of complications and their management . the management is also challenging because of progressive nature of tissue growth . both benign and malignant
two relatively common tumors include cystadenomas of the ovary and monomorphic adenomas of the parotid gland .
presented with macrodactyly of index and middle finger of both the hands and thumb of right hand ( one criteria of category b ) and he met all the three general criteria .
his skeletal survey was normal except for hyperostosis of index and middle finger and there were no associated complications except for the limitation of his affected finger movements .
although the patient did not satisfy the proposed criteria [ table 1 ] , a literature search revealed that out of the 205 cases reported 90 satisfied the criteria highlighting the variability in clinical presentation in cases of ps .
our patient was managed as a case of inferior wall mi and is presently on anti - ischemic medications with no new complications . in conclusion , ps is a very rare and highly variable , progressive tissue overgrowth disorder .
patients should be kept under regular follow - up for the development of complications and their management . craniopharyngioma is an uncommon tumor of the nervous system ; it is well - known to recur even several years after surgery .
we are here with reporting a case of craniopharyngioma which recurred at a site removed from the original site 5 years after surgery and radiotherapy .
a 4-year - old girl was admitted for progressive deterioration of vision of 2 months duration . in addition
there was no history of endocrinopathy , fits or any symptom of raised intracranial pressure . on examination ,
visual acuity was questionable perception of light on the right side and counting fingers at 3 m distance on the left .
imaging revealed a solid and cystic craniopharyngioma in the sellar - suprasellar region [ figure 1a and b ] .
she underwent a right frontotemporal craniotomy and transsylvian exploration and almost total excision of the tumor .
postoperative mr showed a tiny residual tumor adherent to the pons [ figure 2a and b ] .
( a and b ) showing the preoperative images ( before first surgery ) ( a and b ) images after first surgery showing no residual tumour in the primary site but a small fragment adherent to the pons she was given a course of radiotherapy for this residue 54 gy in 30 fractions .
follow - up imaging at the end of 2 years did not reveal any residual tumor [ figure 3 ] .
two years after surgery and radiotherapy no recurrence imaging was being done periodically to check for recurrence .
the 5-year surveillance imaging showed a recurrence in the right sylvian fissure along the route taken during the first surgery .
there was no evidence of tumor in the sellar - suprasellar area [ figure 4a and b ] .
she underwent reexploration by the same route , and a tiny fragment densely adherent to the middle cerebral artery was left behind .
( a and b ) showing remote recurrence five years after first surgery and radiotherapy .
although craniopharyngiomas are benign tumors they are known to recur even after years and even after the administration of radiotherapy , recurrence rates ranging from 25% to 70% .
recurrences at a site removed from the original site are very rare < 25 cases have been reported .
these ectopic recurrences are not to be misinterpreted as ectopic primary occurrences since craniopharyngioma can occur anywhere along the obliterated rathke 's pouch
. these ectopic recurrences may occur along the surgical pathway or at a site , not along the surgical pathway .
the cells of the tumor may get implanted and may subsequently metamorphose into a fresh neoplasm .
these tumor cells may in turn give rise to the regrowth of the tumor . the usual time to recurrence is around 4 years . but why this has to be a peculiarity of craniopharyngiomas can not be explained . another way the tumor may get seeded at a distant site
the evidence for this is strong since tumor cells have been observed in the csf .
although most recurrences are along the surgical corridor an instance where the recurrence has occurred in the spine has been recorded .
when the transsphenoidal route is used , the csf spaces are not violated this may explain the absence of recurrences after transsphenoidal route .
it is not surprising to observe that the histologic examination of the primary and recurrent lesions are the same .
recurrences rates are said to be low after total surgical excision . but recurrences even decades after a quiescent period are well - known .
it can be assumed that radiotherapy would have sterilized the surgical corridor and ectopic recurrences will not occur .
but this was not the case in our patient , and in the few that have been reported .
total excision is not an assurance that recurrences at ectopic or primary site will not occur .
certain measures have been proposed to minimize these ectopic recurrences , protecting the operative field with patties to prevent seeding , emptying the cyst prior to removal of tumor , thorough irrigation of the field before dural closure .
probably , a higher mib-1 index and expression of p53 may predispose to these recurrences . but eternal vigilance and regular imaging are mandatory to detect recurrences . another point worthy of note
is why this phenomenon is not seen with respect to other benign tumors like meningiomas or even malignant tumors . | proteus syndrome ( ps ) is a rare hamartomatous disorder characterized by various cutaneous and subcutaneous lesions , including vascular malformations , lipomas , hyperpigmentation , and several types of nevi .
partial gigantism with limb or digital overgrowth is pathognomonic of ps .
we report a rare case of ps in a 50-year - old man who presented with inferior wall myocardial infarction and was incidentally detected to have hypertrophy of index and middle fingers of both the hands . |
cystic fibrosis ( cf ) is a worldwide disease occurring among virtually all ethnic groups . in caucasians
although approximately 1 in 25 are heterozygous carriers , the incidence of clinical disease is approximately 1 in 2500 live births .
the condition results from mutations in a single gene of chromosome 7 , which encodes the cf transmembrane conductance regulator ( cftr ) .
the cftr protein is a membrane - bound camp - regulated chloride channel thought to regulate other cell membrane ion channels . to date
, more than 1000 different mutations have been identified ; however a phenylalanine deletion in amino acid position 508 is present in approximately 66% of patients .
early genetic tests demonstrating a molecular defect in the cftr gene confirms the clinical diagnosis of cf , improves quality of life and prolongs survival .
recent studies support the theory that cfrd is primarily caused by insulin deficiency due to a loss of beta cells which may occur via a number of mechanisms , including oxidative stress .
cftr mutations affect epithelial ion and water transport , primarily in cells in the respiratory , gastrointestinal , hepatobiliary and reproductive tracts , in addition to the sweat glands .
the lack of chloride secretion in the pancreatic duct is responsible for obstruction and autodigestion of the pancreas early in embryonic life leading to severe exocrine pancreatic insufficiency in approximately 85% of cf newborns .
diagnosis is based on clinical findings and sweat chloride levels greater than 60 meq / l . in iran ,
thus , the present study aims to assess the characteristic demographic findings of cf patients who attended the children s hospital medical center during a ten - year - period .
during a ten - year period ( 1991 - 2000 ) , all patients hospitalized with cf or diagnosed with cf during hospitalization in the children s hospital medical center , tehran , iran were enrolled and related data were extracted from their medical records . sweat chloride tests
the diagnosis of cf was established when relevant clinical manifestations were associated with a positive sweat chloride test .
clinical manifestations included respiratory signs such as chronic cough or recurrent pneumonia and gi manifestations in the form of chronic diarrhea or fatty diarrhea , failure to gain weight and failure to thrive ( ftt ) .
among the 233 patients , 91 ( 39% ) were girls and 142 ( 61% ) were boys .
onset of disease was before the first month of life in 12.1% , between 1 - 6 months of age in 75.1% and between 6 - 12 months of age 6.9% of patients .
a positive family history of cf or suspected clinical signs was present in 26.6% of patients .
barium swallow was performed for 138 patients ; of those , 102 ( 74% ) had gastroesophageal reflux disease .
other findings such as nasal polyps ( 6 ) , gallstones ( 1 ) , sinusitis ( 14 ) , cholestasis ( 9 ) and diabetes ( 2 ) were also noted .
edema ( 19.4% ) , growth failure in the form of weight below the fifth percentile ( 89.1% ) , anemia ( 69.7% ) and hypoalbuminemia ( 60.5% ) were additionally present .
endoscopy was performed in 65 patients and the most frequent finding was esophagitis ( 81.5% ) . in stool samples , fat droplets greater than 100 per hpf were reported in 100% , whereas 62.7% had decreased trypsin activity .
among patients with respiratory symptoms ,
chest radiography was performed in 207 cases and frequent findings were : hyper - aeration with pneumonia ( 35% ) , pneumonia ( 19% ) and hyper - aeration ( 22% ) . death was documented in 27 patients which was attributed to respiratory failure ( 96.3% ) and septicemia ( 3.7% ) ( table 1 ) .
* in hospital inpatient mortality
currently , due to newer , more appropriate , modern enzymatic and antibiotic therapies in addition to nebulizer treatments , improvements in lifespan and quality of life are seen .
recent researches and numerous advancements in the field of gene therapy , which can be the definitive therapy of cf , increased the hope for an extended life .
therefore maintenance therapy , with the aim to perform gene - therapy , is of major importance in maintaining growth , preventing respiratory complications and malnutrition .
it is important to keep this disease in mind when dealing with patients who present with the vast spectrum of clinical findings of cf , which are to some extent non - specific .
thus , children who receive multiple courses of antibiotics for respiratory or gi diseases will need to undergo additional diagnostic tests .
the prevalence of cf in european caucasians is 1:2500 and is rare in asia .
based on the results of the present study and other reports from various locations in asia ; we have assumed that cf is not rare , as presumed in iran ( table 1 ) . in all studies ,
the male to female ratio was 1:1.5,the most frequent age of onset of symptoms occurred in the first six months of life ( 78% ) and consanguineous marriages were significant ( 42% ) .
this might have been due to the fact that barium swallows were performed only in cases with suspected symptoms , whereas it was performed in all patients mentioned in textbooks .
the incidence of ftt in the asian population was almost equal ( 75% - 100% ) .
however in developed countries with the use of new nutritional methods such as alternate tpn in the hospital or at home , and nasogastric tube feedings at night , sufficient calories were obtained and growth failure was less commonly reported .
death occurred in 13.4% of patients in the present study which was less than actual statistical values because a number of cf patients were not followed . in a study from shiraz ( iran ) ,
the cf mortality rate was 70% but in another study it was 0% , which probably resulted from the lack of follow up .
one of the earliest signs of cf was meconium ileus , which ranged from 8% to 20% in different studies .
the authors thank miss maral sayyad and the personnel of the archive of medical documents , endoscopy and gastroenterology departments of children hospital medical center for their assistance .
nasal dermal sinus is a very rare congenital anomaly that is frequently associated with inclusion cysts ( dermoid or epidermoid).1
2
3 at the end of the second month of gestation , the nasal and frontal bones are separated by the fonticulus frontalis . in this period ,
the dura projects into the nasal area through the anterior skull base opening ( foramen cecum ) , and lies in contact with the skin at the tip of the nose .
failure during ossification to obliterate this transcranial connection is the embryological pathway to nasal dermal sinus tract development.1
2
3
4 in the present case , combined use of high resolution magnetic resonance imaging and computed tomography ( ct ) clearly demonstrated the detailed anatomical relationship of the dermal sinus associated with a dumbbell - shaped dermoid to the surrounding structures such as the fonticulus frontalis and foramen cecum.3
5
the patient was a boy aged 1 year and 4 months who had had a small pit at his nasion from birth and had developed swelling of the forehead .
1a ) . the sagittal view of a t2-weighted image demonstrated a dumbbell - shaped , mixed intense dermoid at the foramen cecum . the sinus tract was depicted as a strand of isointensity between the dermoid and the nasion .
a subcutaneous abscess was noted adjacent to the dermoid , and the subcutaneous swelling of the forehead was demonstrated as hyperintensity ( fig .
serial sagittal views of t1-weighted images ( t1wi ) revealed the capsule of the dermoid enhanced with contrast medium ( gadolinium - diethylenetriamine penta - acetic acid [ gd - dtpa ] ) , and that the subcutaneous abscess was in continuity with the dermoid cyst .
on axial view of the gd - enhanced t1wi , the subcutaneous abscess was also noted adjacent to the dumbbell - shaped dermoid ( fig .
serial sections of the sagittal and coronal ct scans clearly showed an enlarged fonticulus frontalis and foramen cecum remnant and dehiscence of the crista galli ( fig .
1f , g , h ) . three - dimensional ct imaging showed a bony defect at the midline of the junction of the frontal bone and the anterior fossa , with a bifid and bulging crista galli ( fig .
( a ) photograph showing the swelling of the forehead . the black arrow indicates a small pit on the nasion .
inset is the magnified view of the pit , which seems to be closed and has no purulent discharge .
there is a dumbbell - shaped mixed intense dermoid ( white arrow ) at the junction of the frontal bone and the anterior fossa ( at the foramen cecum ) , and a strand of isointensity ( nasal sinus tract ) between the tumor and the nasion .
an abscess in the subcutaneous tissue of the forehead ( black asterisk ) is noted adjacent to the dermoid .
the capsule of the dermoid is enhanced with contrast medium ( gd - dtpa ) .
the dermoid is dumbbell - shaped ( white arrow indicates the neck of the dumbbell ) .
both the dermoid ( white arrow ) and subcutaneous abscess ( black asterisk ) are demonstrated as a hyperintensity .
( f , g , h ) serial sections of the sagittal ( f , h ) and coronal ( g ) computed tomography scan .
the enlarged fonticulus frontalis remnant ( white arrow ) , enlarged foramen cecum remnant ( white dotted line ) , and dehiscence of the crista galli ( white arrow heads ) are clearly visible .
a bony defect is evident at the midline of the junction of the frontal bone and the anterior fossa with bifid crista galli ( white arrow heads ) .
( j ) schematic drawing demonstrating the anatomical relationship of the dermoid ( d ) , subcutaneous abscess ( a ) , and swelling ( s ) with surrounding structures .
the subcutaneous abscess was evacuated through a coronal skin incision on the frontal region , and then the purulent dermoid cyst including the capsule was removed .
the part of the dermoid capsule that was tightly adherent to the dura was carefully coagulated .
( a ) intraoperative photograph taken after removal of the dermoid through a coronal skin incision showing the bony defect ( white arrows ) .
inset is a three - dimensional computed tomography image shown in the same orientation as ( a ) the white box indicates the extent of the operative field .
( b ) the sinus tract including the pit at the nasion was dissected with a tiny skin incision .
the dermoid cyst wall is lined by a keratinizing squamous epithelium ( white asterisk ) .
intraluminal keratin ( black asterisk ) and hair shafts ( black arrow ) are also demonstrated .
the nasal sinus tract is a ductal structure ( black star ) lined by stratified squamous epithelium .
the patient was a boy aged 1 year and 4 months who had had a small pit at his nasion from birth and had developed swelling of the forehead .
1a ) . the sagittal view of a t2-weighted image demonstrated a dumbbell - shaped , mixed intense dermoid at the foramen cecum . the sinus tract was depicted as a strand of isointensity between the dermoid and the nasion .
a subcutaneous abscess was noted adjacent to the dermoid , and the subcutaneous swelling of the forehead was demonstrated as hyperintensity ( fig .
serial sagittal views of t1-weighted images ( t1wi ) revealed the capsule of the dermoid enhanced with contrast medium ( gadolinium - diethylenetriamine penta - acetic acid [ gd - dtpa ] ) , and that the subcutaneous abscess was in continuity with the dermoid cyst .
on axial view of the gd - enhanced t1wi , the subcutaneous abscess was also noted adjacent to the dumbbell - shaped dermoid ( fig .
serial sections of the sagittal and coronal ct scans clearly showed an enlarged fonticulus frontalis and foramen cecum remnant and dehiscence of the crista galli ( fig .
1f , g , h ) . three - dimensional ct imaging showed a bony defect at the midline of the junction of the frontal bone and the anterior fossa , with a bifid and bulging crista galli ( fig .
( a ) photograph showing the swelling of the forehead . the black arrow indicates a small pit on the nasion .
inset is the magnified view of the pit , which seems to be closed and has no purulent discharge .
there is a dumbbell - shaped mixed intense dermoid ( white arrow ) at the junction of the frontal bone and the anterior fossa ( at the foramen cecum ) , and a strand of isointensity ( nasal sinus tract ) between the tumor and the nasion .
an abscess in the subcutaneous tissue of the forehead ( black asterisk ) is noted adjacent to the dermoid .
the capsule of the dermoid is enhanced with contrast medium ( gd - dtpa ) .
the dermoid is dumbbell - shaped ( white arrow indicates the neck of the dumbbell ) .
both the dermoid ( white arrow ) and subcutaneous abscess ( black asterisk ) are demonstrated as a hyperintensity .
( f , g , h ) serial sections of the sagittal ( f , h ) and coronal ( g ) computed tomography scan .
the enlarged fonticulus frontalis remnant ( white arrow ) , enlarged foramen cecum remnant ( white dotted line ) , and dehiscence of the crista galli ( white arrow heads ) are clearly visible .
a bony defect is evident at the midline of the junction of the frontal bone and the anterior fossa with bifid crista galli ( white arrow heads ) .
( j ) schematic drawing demonstrating the anatomical relationship of the dermoid ( d ) , subcutaneous abscess ( a ) , and swelling ( s ) with surrounding structures .
the subcutaneous abscess was evacuated through a coronal skin incision on the frontal region , and then the purulent dermoid cyst including the capsule was removed .
the part of the dermoid capsule that was tightly adherent to the dura was carefully coagulated .
( a ) intraoperative photograph taken after removal of the dermoid through a coronal skin incision showing the bony defect ( white arrows ) .
inset is a three - dimensional computed tomography image shown in the same orientation as ( a ) the white box indicates the extent of the operative field .
( b ) the sinus tract including the pit at the nasion was dissected with a tiny skin incision .
the dermoid cyst wall is lined by a keratinizing squamous epithelium ( white asterisk ) .
intraluminal keratin ( black asterisk ) and hair shafts ( black arrow ) are also demonstrated .
the nasal sinus tract is a ductal structure ( black star ) lined by stratified squamous epithelium .
1j demonstrates the detailed anatomical relationship between the sinus tract with dumbbell - shaped dermoid and the surrounding structures .
the body of the dumbbell - shaped dermoid was located in the enlarged fonticulus frontalis and foramen cecum remnant ; the head of the dumbbell was the intracranial extension of the dermoid with bulging dura .
a bifid and bulging crista galli was also attributed to the intracranial extension of the dermoid . although bacteriologic examination failed to reveal the causative agents , the microscopically opened sinus tract resulted in the formation of the subcutaneous abscess adjacent to the purulent dermoid . | background
cystic fibrosis ( cf ) is an autosomal recessive disease caused by a cf trans - membrane regulator ( cftr ) defect .
its prevalence is 1:2500 in caucasians , 1:15300 among african americans and is rare in southeast asia .
the present study aims to review demographic data , clinical manifestations and laboratory findings of iranian children diagnosed with cf who referred to a children s hospital medical center in tehran , iran during a ten - year period .
methods
in a retrospective study from 1991 - 2000 , all hospitalized patients with documented cf were reviewed .
diagnosis was based on clinical findings and sweat chloride levels above 60 meq / l .
results
a total of 233 patients [ females : 91 ( 39.1% ) , males : 142 ( 60.9% ) ] were enrolled .
the onset of symptoms was before the first month of life in 12.1% , between 1 - 6 months of age in 75.1% , and between 6 - 12 months of age in 6.9% of patients .
consanguinity of parents was present in 42.5% of patients .
respiratory ( 81.5% ) and gastrointestinal ( 73.4% ) symptoms , in addition to growth retardation were the most common presentations eighty - eight percent of patients weighted below the fifth percentile .
of the 207 chest radiographs performed , the most frequent finding was hyper - aeration associated with pneumonia . among 138 patients in whom barium swallows were performed , 102 ( 74% ) had gastroesophageal reflux .
a total of 27 patients expired , mostly from respiratory failure ( 96.3% ) .
conclusion
cf is not a rare disease in iran .
we suggest early diagnosis and appropriate maintenance therapy for improving morbidity and mortality amongst cf patients . |
nearest neighbor approaches were developed to predict the folding stabilities of nucleic acid secondary structures ( 1 ) .
these parameter sets utilize empirical rules , generally derived from optical melting experimental data , as the basis of the predictions . for rna , rules exist for predicting both free energy and enthalpy change of watson crick helices , gu pairs and loops ( 25 ) . parameters for dna have also been assembled for predicting watson
crick pair free energy and enthalpy change and free energy changes of loops ( 6,7 ) .
these parameter sets are the basis of computer programs that predict low free energy secondary structures .
such programs include mfold / unafold ( 8,9 ) , the vienna rna package ( 10 ) , rna structure ( 2 ) , rnasoft ( 11 ) and sfold ( 12 ) .
additional approaches that use statistical learning of parameters for rna folding have also used the rules from the nearest neighbor methods and derived new parameter values ( 13,14 ) .
nearest neighbor parameter sets include both a set of rules , called either equations or features , for predicting stability and a set of parameter values used by the equations ( 14 ) . for rna ,
separate rules exist for predicting stabilities of helices , hairpin loops , small internal loops , large internal loops , bulge loops , multibranch loops , exterior loops and pseudoknots .
given the number of rules and constraints on the length of journal publications , it is difficult to assemble all the parameters in one publication and provide meaningful tutorials for using the parameters .
this is a barrier to software development for novel algorithms that could take advantage of the parameters .
for example , many software packages that use rna parameters still implement the set of parameters assembled in 1999 ( 4 ) , in spite of the fact the rna parameters were updated in 2004 ( 2 ) based on experimental results .
the nearest neighbor database ( nndb ) is a web - based tool for assembling and archiving complete nearest neighbor sets , including rules and values .
currently , the 1999 and 2004 sets of rna folding parameters are provided ( 25 ) .
the nndb is built using a set of static html , specifically xhtml 1.0 transitional pages with a page hierarchy shown in figure 1 .
text is encoded in unicode ( utf-8 ) to facilitate display of equations in pages with diverse browsers running on diverse operating systems .
the top - level page provides access to a help page , available parameter sets and a page of references to rna optical melting experiments .
additionally , links provide downloading of the whole database in either zip or gzipped tar format .
the help page introduces the purpose of the database and defines basic terms , including the set of structural features defined by secondary structures .
for example , figure 2 , from the help page , shows an rna secondary structure that illustrates the loop features covered by nearest neighbor parameter sets .
the basic equations for utilizing the parameters to extrapolate folding free energy changes to temperatures other than 37c and to predict melting temperatures are also provided .
this figure illustrates the page hierarchy by following the linked pages down through the 1999 parameters and down to the hairpin loop pages .
note that there are five example calculations for hairpin loops to illustrate the separate sequence - dependent rules that are used depending on the specific loop .
figure 2.an rna secondary structure illustrating the types of features included in nearest neighbor parameter sets .
internal loops have nucleotides not in canonical pairs on each of two strands , but bulge loops have nucleotides not in canonical pairs on only one strand .
formally , a pseudoknot occurs when there are at least two pairs , with indices i paired to j and i paired to j , that satisfy the condition i < i < j < j. the pseudoknot helix is often considered to be composed of the fewest pairs that need to be removed to relieve the pseudoknot ( 19 ) . in this structure ,
this figure illustrates the page hierarchy by following the linked pages down through the 1999 parameters and down to the hairpin loop pages .
note that there are five example calculations for hairpin loops to illustrate the separate sequence - dependent rules that are used depending on the specific loop .
an rna secondary structure illustrating the types of features included in nearest neighbor parameter sets .
internal loops have nucleotides not in canonical pairs on each of two strands , but bulge loops have nucleotides not in canonical pairs on only one strand .
formally , a pseudoknot occurs when there are at least two pairs , with indices i paired to j and i paired to j , that satisfy the condition i < i < j < j. the pseudoknot helix is often considered to be composed of the fewest pairs that need to be removed to relieve the pseudoknot ( 19 ) . in this structure ,
the tan nucleotides are in pairs that could be removed to relieve the pseudoknot . for each set of parameters ,
for example , the 1999 rna rules predict only folding free energy changes ( 4 ) , but the 2004 rules can be used to predict both folding free energy and enthalpy changes ( 2,5 ) . for each structural feature
, a page defines the basic equations and provides links to parameter values ( in plain text and html ) , references and tutorial pages ( e.g. figure 3 ) .
the number of tutorials varies from feature to feature ; the set of tutorials is designed to cover each type of rule that can be encountered in practice .
crick helix parameters are covered with two tutorials , one for self - complementary and one for non - self - complementary strands .
these two tutorials also demonstrate the difference in the calculation when there are terminal au base pairs , which receive a free energy and enthalpy change penalty ( 3 ) , because the self - complementary duplex example has two terminal au pairs and the non - self - complementary case has no terminal au pairs .
this tutorial demonstrates the prediction of folding free energy change for a hairpin loop of six unpaired nucleotides using the 2004 parameters ( 2,3 ) . an example tutorial from the database .
this tutorial demonstrates the prediction of folding free energy change for a hairpin loop of six unpaired nucleotides using the 2004 parameters ( 2,3 ) .
individual pages above the level of value tables have top banner , a left navigation bar that allows the user to navigate back up the hierarchy to any level above and a bottom bar with the date of last editing . for pages edited after the database has gone online , previous versions of the page are available using this bottom content bar . to facilitate indexing by search engines ,
all pages have a descriptive title , including the set of parameters to which it belongs ( if applicable ) .
the first release of the nndb contains the rna folding rules assembled in 1999 and 2004 ( 25 ) .
these rules represent the most recent set of parameters and a prior set that is widely used in software packages . because folding rules are derived to work as a set , the two versions of rules and values should not be mixed and the website hierarchy reinforces this .
it is anticipated , for example , that additional pages will be written to include nearest neighbors for dna folding ( 6,7 ) and for predicting rna pseudoknot stabilities ( 1518 ) .
additionally , the values derived from the re - estimation of the values of the 1999 parameter set using the set of known rna secondary structures will also be included ( 14 ) .
the nndb is designed to provide a convenient location for assembling parameter sets for predicting the stability of nucleic acid secondary structures .
it is modular in design , which facilitates its future expansion to contain additional parameter sets .
furthermore , the web format makes it feasible to provide extensive tutorials for utilizing the parameters , which is generally not possible in print .
the creation of the nndb was supported by united states national institutes of health grants gm076485 to d.h.m . and gm22939 to d.h.t . funding for open access charge : united states national institutes of health . mucocele of the appendix ( collection of mucus within the appendiceal lumen ) is a rare lesion , found in only 0.2% to 0.3% of 43,000 appendectomies reviewed .
currently , the assessment of pelvic masses relies heavily on usg as the primary diagnostic tool .
in such cases , clinical findings and other investigative modalities are warranted to aid the diagnostic process . in spite of extensive preoperative investigations ,
the diagnosis may still remain elusive and may only be made at the time of surgery . some regard this lesion as benign , a result of obstruction of the proximal lumen by fibrosis ; others believe it to be a neoplasm of the appendix .
is the method of choice in the management of simple mucocele and for cystadenoma with an intact base .
several studies ( mostly case reports ) on laparoscopic resection of mucocele have been reported .
a 60-year - old female presented with pain in lower part of abdomen and palpable tender lump in the right ileac fossa .
ultrasound of the abdomen reports a cystic mass of size 12 15 cm with thin internal septations in the right adnexa .
the pneumoperitoneum was created with veress needle using carbon dioxide and the pressure was kept at 11 mmhg .
a 0 telescope was introduced through the umbilical port for the complete examination of the abdomen .
diagnostic laparoscopy revealed approximately 14 15 cm large bluish mucocele of the appendix with omental adhesions .
two 5-mm ports were placed in the supra pubic area below the pubic hair line as the working port .
the mucocele of the appendix was isolated after separating the mesoappendix from it with the help of bipolar cautery . following this ,
mucocele of the appendix [ figure 1 ] was retrieved out in a plastic bag through the umbilical port .
hemostasis was obtained and a suction drain left in situ which was removed when non - productive .
cut section showed appendix was filled with mucin - like material [ figure 2 ] .
she was started orally after 4 hours of operation and solid food on the next day .
appendicular lump from the distal portion of appendix after removal the appendicular lump filled with mucinus material
mucocele of the appendix is a descriptive term for an appendix distended by mucus , secondary to mucinous cystadenoma ( 63% ) , mucosal hyperplasia ( 25% ) , mucinous cystadenocarcinoma ( 11% ) , and retention cyst
. clinical presentation may include right lower quadrant pain , change in bowel habits , per rectal bleeding , or a palpable mass .
approximately 23 - 50% of patients are asymptomatic , with the lesions being discovered incidentally during surgery , radiological evaluations , or endoscopic procedures .
the preoperative clinical diagnosis of appendiceal mucoceles can therefore be difficult because of this lack of clinical symptomotology .
the initial detection of the lesion may be facilitated by radiological , sonographic , or endoscopic means . on barium enema ,
the lesion may be seen as a sharply outlined sub - mucosal or extrinsic mass indenting the cecum and laterally displacing it .
purely cystic lesions with anechoic fluid , hypoechoic masses with fine internal echoes as well as complex hyperechoic masses can be seen depending on the contents .
ct of the abdomen usually shows a cystic well - encapslated mass sometimes with mural calcification , in the expected location of the appendix .
it may be causing extrinsic pressure on the cecal wall without any surrounding inflammatory reaction .
colonoscopic findings include the volcano sign , the appendiceal orifice seen in the center of a firm mound covered by normal mucosa or a yellowish , lipoma - like submucosal mass . in our case , usg was unable to provide a preoperative diagnosis . in our case , the decision for excision of the appendiceal mucocele was made as a result of diagnostic laparoscopy and a need to rule out malignancy .
therefore mucocele of the appendix can mimic an adnexal mass and prove to be a diagnostic challenge . in a woman presenting with right iliac fossa mass and with clinical features not indicative of gynecological pathology , an appendiceal origin should be considered in the differential diagnosis .
surgery is the treatment of choice and should be done early as tumor can not be ruled out as the causative factor for the mucocele .
pre - operative diagnosis is important to avoid unintended rupture and the development of pseudomyxoma peritonei during surgery .
however , laparoscopic dissection , grasping of the appendix specimen , pneumoperitoneum , or transport of the specimen through the abdominal wall might contribute to peritoneal dissemination of a tumor , if present .
these setbacks can be avoided by taking precautions like using bowel holding graspers ( non - traumatic ) to handle the mucocele and using a non - permeable bag to deliver the specimen out of the port .
mucocele of the appendix can mimic an adnexal mass and prove to be a diagnostic challenge .
laparoscopic resection of mucocele of the appendix is feasible in spite of the danger of malignancy , provided necessary precautions are taken . | the nearest neighbor database ( nndb , http://rna.urmc.rochester.edu/nndb ) is a web - based resource for disseminating parameter sets for predicting nucleic acid secondary structure stabilities . for each set of parameters ,
the database includes the set of rules with descriptive text , sequence - dependent parameters in plain text and html , literature references to experiments and usage tutorials .
the initial release covers parameters for predicting rna folding free energy and enthalpy changes . |
reactivation of latent tuberculosis ( tb ) is a serious hindrance to continuation of therapy .
we present here a case of pleural tb in a patient on infliximab for ankylosing spondylitis .
a 36-year - old male presented to our hospital in 2006 with low back ache inflammatory type with symmetric joint pains involving large joints such as shoulder joints , hip joints , knee joints , and small joints such as metacarpophalangeal joints , elbow joints , and metatarsophalangeal joints .
there was associated early morning stiffness for over an hour . on examination , synovitis was present in peripheral joints with restriction of movement in all joints . modified schober 's test was positive , and the chest expansion ( <3 cm ) was restricted . on evaluation , he had anemia and elevated erythrocyte sedimentation rate ( esr ) ( 46 mm / h ) and c - reactive protein ( crp ) ( 18 mg / l ) .
imaging revealed kyphoscoliosis of thoracic spine , syndesmophytes at multiple levels giving the appearance of bamboo spine [ figure 1 ] .
diffuse ossification of interspinous and paraspinal ligaments with fusion of thoracic and lower cervical vertebra was present .
radiograph showing bamboo spine in view of hla - b27 , more than 2 spa features and typical radiological features , ankylosing spondylitis was considered .
he was started on nonsteroidal anti - inflammatory drugs ( nsaids ) and dose escalated for symptomatic relief . in view of persisting symptoms and elevated esr and crp in spite of optimal nsaids ,
following three doses of infliximab and 10 months after initiation of therapy , patient came with complaints of fever and cough for 1 week . on examination ,
breath sounds were reduced over the right side , and there was dullness on percussion .
diagnostic thoracocentesis showed lymphocytic ( total leukocyte count - 4800 cells / cumm , lymphocytes - 90% ) exudative type of effusion with high adenosine deaminase ( ada ) ( 114
iu / l ) . according to light 's criteria ( effusion protein - 6 g / dl , serum protein - 3.37 g / dl , effusion lactate dehydrogenase ( ldh ) - 575
ankylosing spondylitis is a chronic , systemic , inflammatory disease that affects primarily the sacroiliac joints and spine . it is a spondyloarthropathy with a prevalence of 0.1%0.4% globally .
data from india are sparse . its more commonly seen among males under 30 years of age . diagnosis is made after thorough clinical examination and radiography .
infliximab is one such biologic which acts by inhibiting a pro - inflammatory cytokine tnf- and reducing inflammation .
target - related adverse effects with tnf inhibitors are infections , opportunistic infections , malignancies , demyelinating conditions , hematologic abnormalities , congestive heart failure , autoantibodies ( antinuclear antibody and anti - double - stranded dna ) , hepatotoxicity , dermatologic reactions , and lupus - like syndromes , whereas the agent - related adverse effects are administration reactions and immunogenicity .
tnf- is a cytokine that plays an important role in the mediation of inflammation and immune regulation .
they are required for inflammatory response against intracellular organisms . in experimental models , fungal and bacterial infections
pneumocystis carinii and histoplasma capsulatum are some of the fungal pathogens , whereas the bacterial agents are listeria monocytogene , mycobacterium tuberculosis , and mycobacterium avium .
upper and lower respiratory tract infections are the most commonly seen ones . there was also an increased risk of serious infections compared with controls ( 3.6% vs. 1.7% ) .
registries of rheumatoid arthritis patients have shown that the relative risk for infection ( 3.34.1 ) as well as serious infection ( 2.72.8 ) was significantly higher among patients receiving tnf inhibitors .
to conclude , severity of disease , use of other medications such as corticosteroids , and the presence of comorbidities also contribute to infections in addition to tnf inhibitors alone .
opportunistic infections following tnf inhibitor therapy include disseminated m. tuberculosis . in a study of seventy cases of tb following infliximab therapy ,
thirty were pulmonary tb and of forty were extrapulmonary disease , only two were cases of pleural tb . around a quarter of the cases were disseminated disease .
the majority of cases of tb were observed within a median period of 12 weeks after initiation of therapy and is likely due to reactivation of latent tb .
a study by grover et al . in india has shown a high incidence of tb ( 21% ) following biologic therapy .
it was also seen that among those who received low doses of infliximab ( 3 mg / kg body weight ) did not develop tb .
another study by malaviya et al . has found a lower incidence ( 9.4% ) of tb among those on tnf inhibitors .
the rate of development of active tb among rheumatoid arthritis patients on anti - tnf therapy has dropped by 83% with the help of screening .
suppressing the action of tnf- can help in relieving the symptoms of ankylosing spondylitis by reducing the inflammatory process , but at the same time , it weakens immune response to microbes such as tubercle bacilli .
hence , meticulous screening and close monitoring of patients on infliximab for any symptoms and signs of tb are important as there is a risk even though the screening tests have come out be negative .
myoepithelial carcinoma ( mc ) is a rare tumor with an incidence of 0.2% of all salivary gland tumors . most of the reported cases of mc arise in the parotid gland ( 4875% ) , followed by minor salivary glands , and the submandibular gland .
the first case was described by higashiyama et al . , in 1998 . since then , only seven cases have been reported in literature .
a mentally retarded 13-year - old girl , with a history of congenital hypothyroidism and cystic lymphangioma in the left dorsal region , operated in 2004 , had consulted for cough and dyspnea in september 2010 .
physical examination showed a decrease in vesicular breath sounds at the basal areas of chest bilaterally , without fever .
pulmonary computed tomography ( ct ) showed bilateral pleural masses measuring 6.3 cm and 7.4 cm at the right and left bases , respectively , with lymph node metastases [ figure 2 ] .
( a ) bilateral pulmonary opacities ( b ) disappearance of pulmonary opacities pulmonary computed tomography ( ct ) : bilateral pulmonary masses a histopathological study of the left pleural biopsy revealed a monomorphic proliferation of round cells with clear cytoplasm and a weak , mitotic hyperchromatic oval nucleus [ figure 3 ] .
there were some cohesive layers , separated by bands of sclerosis ; the stroma was sparse with foci of necrosis .
the immunohistochemistry ( ihc ) study was focally positive for vimentin , cd99 , and ps100 .
the abdominal ultrasound , bone scan , and metaiodobenzylguanidine ( mibg ) scintigraphy were normal .
the histopathological and the ihc review at the institut bergoni in france concluded the diagnosis of myoepithelial carcinoma of the soft tissues with intermediate malignancy .
ihc was positive for pancytokeratin ( ae1/ae3 ) and ps100 , and negative for ema , cd34 , desmin , and cd99 .
the progression was marked by the disappearance of the pulmonary opacities [ figure 1 ] .
they include mucoepidermoid carcinoma , adenoid cystic carcinoma , acinic cell carcinoma , oncocytoma , epithelial
it arises from the submucosal bronchial glands of the lower respiratory tract . in the world health organization classification , published in 2004 ,
mc was cited as being synonymous with epithelial myoepithelial carcinoma . as mc and epithelial
myoepithelial carcinoma of the salivary gland are distinguished by the presence or absence of ductal cells , their pulmonary counterparts must also be differentiated .
myoepithelial tumors are low - grade lesions without recurrences or metastasis described after resection , whereas , the rate of metastasis in mc is high , as seen in our case .
our patient represents the first pediatric case , described in the literature , having primitive pulmonary mc .
the tumor was peripheral and bilateral , measuring 6 cm and 7 cm , which was in agreement with the literature .
in fact , the size of the mc ranged from 15 mm to 130 mm ( mean 50.7 mm ) [ table 1 ] . characteristics of pulmonary mc in our case , the histopathological study oriented to the diagnosis of a primitive neuroectodermal tumor or neuroblastoma .
the pathological review , with further ihc analysis in france , had concluded the diagnosis of mc .
the following markers were found to be useful in myoepithelial carcinoma : cytokeratins ( ae1/ae3 ) and vimentin ( reported to be positive in neoplastic myoepithelial cells and negative in normal myoepithelial cells ) .
other variable markers , such as , ps100 , calponin , smooth muscle actin ( sma ) , muscle - specific actin ( msa ) , smooth muscle myosin , and p63 protein , could be positive .
however , neoplastic transformation of myoepithelial cells could result in a loss or a modification of their smooth muscle phenotype . in our case ,
although the eight patients reported in literature were treated with optimal surgery , metastases were reported in seven of them ( 87.5% ) ( contralateral lung , forearm , liver , and brain ) .
it was significant that a patient who had not developed metastasis , had the lowest tumor mitotic rate of 5/10 hpfs .
this fact reasoned that the mitotic rate could be an important prognostic factor of the clinical outcome and survival in primary myoepithelial carcinoma of the lung .
given the rarity of these tumors , recommendations regarding chemotherapy or radiation , either pre- or postoperatively , are difficult to formulate .
the originality of our case is the disappearance of the pulmonary opacity spontaneously , without any treatment .
our case represents , to the best of our knowledge , the first pediatric case having primitive pulmonary mc .
the histopathological study familiarizes the diagnosis , but a further ihc study is needed to confirm the diagnosis and to eliminate other etiologies .
surgery represents the main treatment for the operable forms . to the best of our knowledge ,
we have reported the first case , with spontaneous regression of this tumor , without any treatment . | we present a case of pleural tuberculosis ( tb ) in a patient on infliximab for ankylosing spondylitis .
a 36-year - old male presented to our hospital with low back ache of inflammatory type along with multiple symmetric inflammatory type of joint pain .
further clinical examination , laboratory and radiological investigations were suggestive of ankylosing spondylitis .
he was initially treated with nonsteroidal anti - inflammatory drugs but citing poor response it was decided to initiate biologic therapy using infliximab ( antitumor necrosis factor - alpha ) .
mantoux test and chest radiograph were done before the therapy to rule out tb . following three doses of infliximab ,
patient came with complaints of fever and cough for 1 week . on investigation
, it was found to be a case of pulmonary tb .
this shows the importance of close monitoring of patient for tb among patients on infliximab even though the screening test has come out to be negative . |
as environmental and personal sanitation improves , the incidence of typhoidal salmonellosis tends to decrease , while the incidence of non - typhoidal salmonellosis markedly increases , .
the most common clinical manifestation of nts is gastroenteritis , and the condition includes bacteremia , focal infection , and an asymptomatic carrier state . of the manifestations , urinary tract infection ( uti ) is unusual and rare , , and occurs in immunocompromised individuals , including patients with a malignancy , human immunodeficiency virus infection , or diabetes mellitus and patients receiving corticosteroid therapy or treatment with other immunotherapeutic agents , .
uti caused by nts presents as either pyelonephritis or cystitis , , ; cases of hemorrhagic cystitis are extremely rare .
we report a case of severe hemorrhagic cystitis caused by nts that resulted in shock and syncope in a patient with uncontrolled diabetes .
a 41-year - old man came to the emergency room for a fever that had developed 10 days earlier , and was accompanied by pus - like urine .
the patient had watery diarrhea and had lost five kilograms of weight in one month .
he had been diagnosed with diabetes mellitus 5 years earlier and his blood sugar level was not well controlled .
the patient was a baker , did not have any pets , and did not have a history of recent travel . on admission ,
his temperature was 38 c , his blood pressure was 110/60 mm hg , and his heart rate was 102 beats / min .
platelets were 209,000/mm , hemoglobin was 13.6 g / dl , hematocrit was 40.9% , bun / creatinine was 34.0/1.0 mg / dl , fasting / postprandial two - hour blood sugar was 330/482 mg / dl , and hba1c was 11.1% .
a widal test showed a typhoid o titer of 1:1280 and typhoid h titer of 1:640 , and a urine culture grew nts ( group d ) .
no bacteria were isolated from the stool or blood culture . on abdomen - pelvis ultrasonography ,
the urinary bladder was filled with pus , the bladder wall was thickened diffusely ( fig .
1a ) , and hydronephrosis and hydroureter were observed on both sides of the bladder ( fig .
the level of serum creatinine and urine output remained a consistent level during this period .
after 10 days in the hospital , the patient 's urine output decreased suddenly and he developed severe lower abdominal distension and syncope .
the patient 's systolic blood pressure fell to 80 mm hg and hemoglobin to 7.9 g / dl .
an emergency ct scan showed that the urinary bladder was severely distended and filled with a mass suspicious of hematoma ( fig .
3 ) . however , there was no stone , mass or focal mechanical injury , i.e foley catheter injury .
blood pressure was stabilized after transfusion of three packs of rbc and hemoglobin was elevated to 10.2 g / dl .
he responded well to treatment and was discharged in good condition with oral antibiotics and oral hypoglycemic agents .
despite improvements in individual and collective sanitation as well as the careful monitoring of food processing , sporadic episodes and outbreaks of salmonellosis continue to occur in industrialized countries .
the overall incidence of typhoidal salmonellosis has decreased , whereas that of non - typhoidal salmonellosis has increased , .
non - typhoidal salmonellosis is a disease of great public health importance . unlike s. typhi and s. paratyphi ,
the main mode of transmission is from food products contaminated with animal products or waste , most commonly eggs and poultry , but also undercooked meat , unpasteurized dairy products , seafood , and fresh produce .
nts is an enteroinvasive bacterium and causes infections that may have one of four different clinical presentations .
however , invasion beyond the gastrointestinal tract occurs in approximately 5% of patients with nts gastroenteritis , resulting in bacteremia .
extraintestinal manifestations , which have a rare incidence of about 28% , include endocarditis , pericarditis , arteritis , soft tissue infection , uti and pneumonia .
the gastrointestinal carrier state is the fourth clinical presentation and is defined as the excretion of nts for months or years after the initial onset of disease .
uti caused by nts is so rare that it comprises only 0.01%3% of positive urinary cultures , , and 3% of nts infections .
nts has been postulated to enter the urinary tract either hematogenously or by direct invasion of fecal flora to the bladder via the urethra .
the risk factors of uti caused by nts include old age , chronic illness , immunosuppressive therapy and structural abnormality of the urinary tract .
uti related to nts presents as cystitis , pyelonephritis , renal abscess , or asymptomatic pyuria , .
a retrospective analysis of 28 cases of bacteriuria due to nts showed that twenty - one patient ( 75% ) had symptoms of uti ( 16 , cystitis ; 3 , pyelonephritis ; and 2 , renal abscess ) .
another review of nineteen patients with bacteriuria due to nts showed that the frequency of uti due to nts was 0.07% ( proportion of positive urine cultures ) .
eighteen patients ( 94.7% ) had symptoms of uti ( 12 , cystitis ; 6 , pyelonephritis ) , and 1 remained asymptomatic . however , case of hemorrhagic cystitis due to nts was very rare . furthermore , severe hemorrhagic cystitis caused by nts with massive bleeding that resulted in shock and syncope was extremely rare .
uti from salmonella does not differ clinically from uti caused by other members of the enterobacteriaceae
.
our patient had uncontrolled diabetes for 5 years and had worked at a job in food handling for a long time .
we are not sure of the mode of transmission in our patient 's case ; however , we propose that he might have been a salmonella carrier or that his illness was caused by bacteremia from a previous gastrointestinal infection . in uncomplicated salmonella gastroenteritis , antibiotic therapy is not recommended because it does not shorten the disease duration .
however , in cases of sepsis or local extraintestinal infection , prompt antibiotic administration is needed .
traditionally , antibiotics such as ampicillin , amoxicillin , chloramphenicol , and trimethoprim - sulfamethoxazole are used , but currently the frequency of antimicrobial resistance in salmonella isolates is increasing , .
in korea , according to recent reports , group b salmonella is the most common drug - resistant serotype , while the group d serotype has relatively low drug resistance .
the treatment of focal extraintestinal salmonella infection often requires both surgical drainage and antibiotic therapy in cases associated with structural abnormality of the urinary tract .
early surgical intervention might be combined with more prolonged therapy to eradicate uti caused by nts , . in this report
, we describe a case of hemorrhagic cystitis due to nts , which caused massive bleeding , shock , and syncope in a patient with uncontrolled diabetes .
amisulpride came into the indian market a few years back with hypes and hopes in the management of schizophrenia .
its broad spectrum effectiveness with lower chances of extrapyramidal symptoms ( eps ) and metabolic syndrome did help psychiatrists to treat schizophrenia and related disorders more effectively .
although this antipsychotic does not block serotonin receptors at all , it is a high - affinity and highly selective d3/d2 receptor antagonist with atypical properties .
its selective affinity for dopamine receptors in the limbic structures , but not in the striatum , leads to a low risk of extrapyramidal side effects .
all available reports suggest that chance of eps is very less with amisulpride at doses < 400 mg / day .
however , there are sporadic reports of drug - induced eps including dystonia and akathisia even in patients receiving low doses of amisulpride . here
a 30-year - old male with schizophrenia for the past 10 years now presented with predominantly negative symptoms .
he was on olanzapine 15 mg / day for more than 6 months without much improvement .
hence , amisulpride was instituted with a starting dose of 50 mg / day with a gradual increment up to 300 mg / day within 14 days .
the patient came after 14 days to the casualty with features of parkinsonian syndrome such as slowed gait , mild rigidity , salivation , and bradykinesia .
he was hospitalized , amisulpride was immediately stopped , and trihexyphenidyl 4 mg / day was given to manage the side effect .
his eps gradually subsided and for negative symptoms , clozapine was introduced at a small dose of 25 mg / day and gradually increased to 200 mg / day over a period of 10 days . at the time of discharge , on the 14 day , he was free from parkinsonian symptoms .
subsequent follow - up showed no parkinsonian symptoms and he had modest improvement in negative symptoms . a 48-year - old male with schizophrenia for the last 20 years
was treated with various antipsychotics without much improvement . since the last 6 months , he was on olanzapine 15 mg / day .
as there was no significant improvement , his olanzapine dose was gradually tapered and stopped over a period of 14 days and was started on amisulpride 100 mg / day and was increased to 200 mg / day over a period of 3 weeks .
the patient returned on the 24 day with severe parkinsonian symptoms . in this patient also , there was no prior history of parkinsonism .
we managed him with injection promethazine 25 mg intramuscular bid first 3 days along with trihexyphenidyl 2 mg bid after stopping amisulpride .
after 7 days , parkinsonian symptoms improved considerably and clozapine was introduced at a dose of 25 mg / day which was subsequently increased to 100 mg / day on the 10 day and the patient was discharged .
since the discovery that clozapine induces fewer eps and is more effective for negative symptoms than conventional antipsychotics for the treatment of schizophrenia , psychopharmacological research has focused on the development of drugs that block central 5-ht2 receptors more than d2 receptors .
combined 5-ht2/d2 receptor antagonism is the most current explanation for the so - called atypical profile of some antipsychotics .
amisulpride at low doses binds selectively to dopamine d2 , d3 autoreceptors , thereby enhancing dopaminergic transmission and thus might be effective for negative symptoms .
it has no affinity for d1 , d4 , and d5 receptor subtypes . at higher doses
, it blocks postsynaptic receptors , thus inhibiting dopaminergic hyperactivity . at the same time , amisulpride has greater specificity for the limbic system and thus has low incidence of eps .
amisulpride binds more loosely than dopamine to the dopamine d2 receptor and is rapidly dissociated from the dopamine d2 receptor .
low - dose therapy with amisulpride is associated with a significantly lower blockade of striatal dopamine d2 receptors than is seen during high - dose treatment . however , a significant striatal d2 blockade was demonstrated at therapeutically effective dose ranges , and a good relationship between the degree of striatal dopamine d2 receptor occupancy and the amisulpride plasma concentration or the administered dose was shown . in general , asians are slow metabolizers .
low body weight and slow metabolism may increase the plasma concentration of drugs causing side effects .
reported a low postsynaptic d2 occupancy in the striatum at low doses of amisulpride ( 50100 mg / day ) .
it has also been suggested that extrastriatal binding could mediate the effect on negative symptoms .
the probable causes of eps with low doses of amisulpride could be that it blocks postsynaptic d2 receptors significantly in striatum without much effect in the mesolimbic pathway .
, the lower incidence of eps which is claimed by western researchers as well as pharmaceutical companies should be studied well in the indian context .
we should at least keep this side effect in our minds while starting or increasing the doses .
| hemorrhagic cystitis is defined by lower urinary tract symptoms that include dysuria , hematuria , and hemorrhage and is caused by viral or bacterial infection or chemotherapeutic agents .
reports of hemorrhagic cystitis caused by non - typhoidal salmonella ( nts ) are extremely rare.we report a case of a 41-year - old man with hemorrhagic cystitis from nts that caused massive bleeding and shock .
the patient was hospitalized for uncontrolled diabetes and obstructive uropathy related to severe cystitis .
a urine culture was positive for group d nts .
this case demonstrated that hemorrhagic cystitis in a patient with a risk factor such as diabetes can be a manifestation of local extra - intestinal nts infection . |
in spite of the relatively high accuracy of endoscopic ultrasound - assisted fine - needle aspiration ( eus - fna ) in diagnosing lymphomas , inadequate sampling by eus - fna often makes it difficult to perform immunohistochemical analysis , thus limiting its application in the classification of lymphoma .
natural orifice transluminal endoscopic surgery ( notes ) is a surgical technique by which procedures such as exploration , biopsy , organ resection , and anastomosis can be performed using an endoscope passed through a natural orifice [ such as the mouth , stomach , colon ( or rectum ) , vagina , bladder , or esophagus ] and then entered into the abdominal cavity , mediastinum , or thoracic cavity through an internal incision .
the advantages of notes include reduced trauma , faster recovery , absence of scarring , and painlessness , and such procedures have been regarded as third - generation surgery . here
we report an eus - assisted retroperitoneal lymph node biopsy performed in a patient who had developed enlarged retroperitoneal lymph nodes with an unknown cause .
this procedure was carried out on november 10 , 2014 , after obtaining the approval of the ethics committee and informed consent documents signed by the patient .
a 60-year - old male patient was admitted to our hospital complaining of epigastric discomfort , which had persisted for 1 month . a computerized tomography ( ct ) scan suggested the presence of multiple soft - tissue density masses in the patient 's abdominal cavity , the largest of which was 7.7 cm 7.2 cm [ figure 1a and b ] .
positron emission tomography - ct ( pet - ct ) showed that these masses had abnormal f - fdg uptake [ figure 2a and b ] .
the patient then underwent eus examination ; multiple enlarged retroperitoneal lymph nodes were found between the body of the pancreas and the gastric wall .
we then performed eus - fna [ figure 3 ] to obtain a tissue sample for biopsy .
pathological examination revealed only a few heterotypic cells [ figure 4 ] . because of the lack of definite pathological evidence , diagnosis of the patient 's condition was extremely difficult .
ct scan showing multiple , enlarged soft tissue - density images in the abdominal cavity pet - ct showing the accumulation o f abnormal radioactivity in soft tissue - density images in the abdominal cavity eus - fna of a lymph node eus - fna showing a few heterotypic cells to obtain adequate tissue samples of the enlarged lymph nodes for immunohistochemical analysis , we performed eus - assisted retroperitoneal lymph node biopsy .
a standard single - channel gastroscope ( epk - i , pentax , tokyo , japan ) was used throughout the endoscopic procedure ; a linear array ultrasonic endoscope ( eg3830ur ; pentax precision instrument corporation , orangeburg , ny , usa ) was used to evaluate the size of the lymph nodes , their echo characteristics , and localization . a triangle - tip knife and an insulated - tip ( it ) knife ( both from olympus corporation , tokyo , japan )
were used for resection of the gastric wall and enucleation of the lymph node . a pair of hot forceps ( fd-410lr , olympus corporation , tokyo , japan ) was used for gastric wall hemostasis .
adequate preoperative communication with the patient and his family was performed and associated issues including the necessity , feasibility , safety , and probable complications of the operation were explained thoroughly .
for the procedure , the patient was placed in a supine position and he received standard intravenous anesthesia with propofol .
the site nearest to the retroperitoneal lymph nodes in the posterior wall of the gastric body was chosen for puncture .
a methylthioninium chloride and saline compound solution was injected into the puncture channel for labeling when the needle was retrieved .
the lymph node was also marked by a triangle tip knife with the cautery under eus - guidance .
then , the ultrasound transducer was pulled out ; therapeutic gastroscopy with a transparent cap was then performed .
a triangle - tip knife was used to incise the full thickness of the gastric wall along the labeled site .
the triangle - tip knife was used to separate the tissues surrounding the stomach wall sufficiently until the targeted lymph node capsule was exposed .
then , enucleation of the targeted lymph node was performed using an it knife [ figure 5 ] .
after tissue samples were obtained , the hot forceps were used to stop the bleeding .
the endoscope was retrieved from the stomach and the procedure was completed by closing the incision in the stomach wall using metal clips [ video 1 ] .
the results showed : cd3(large cell- ) ; vimentin(+ ) ; pax-5(- ) ; cd15(- ) ; cd20(large cell+ ) ; cd21(+ ) ; ki-67(large cell8%+ ) ; cd30(- ) ; cd68(partly+ ) ; ck(- ) ; mum-1(- ) ; cd10(- ) ; bcl-6(+ ) ; bcl-2(+ ) .
the diagnosis was : non - hodgkin lymphoma , germinal center b - cell - like diffuse large b - cell lymphoma [ figure 6 ] .
( a ) the gastroscope entered into the abdominal cavity after incision of the full thickness of the gastric wall .
the prelabeled lymph node was found , ( b ) the removed lymph node tissues immunohistochemical staining : cd3(large cell- ) ; vimentin(+ ) ; pax-5(- ) ; cd15(- ) ; cd20(large cell+ ) ; cd21(+ ) ; ki-67(large cell8%+ ) ; cd30(- ) ; cd68(partly+ ) ; ck(- ) ; mum-1(- ) ; cd10(- ) ; bcl-6(+ ) ; bcl-2(+ ) .
the diagnosis was non - hodgkin lymphoma , germinal center b - cell - like diffuse large b - cell lymphoma the patient was given standard postoperative treatments and nursing care including ecg monitoring , ceftazidime as prophylaxis against infection , proton pump inhibitors , and nutritional support .
the patient 's highest temperature after the procedure was 37.2c and he only felt mild epigastralgia .
the blood test results after the procedure were : white blood cell ( wbc ) count 12.8 10/l and percentage of neutrophils 80.3% .
the wbc count decreased to 6.5 10/l and the percentage of neutrophils decreased to 61.8% , 4 days after the procedure .
the patient gradually returned to a normal diet and normal physical activities by 3 days after the procedure .
the patient was then transferred to the department of hematology to undergo further therapy . the r - chop regimen (
pathological evidence is an indispensable part of the diagnosis and differential diagnosis of lymphoma and is significant for the classification of lymphomas .
precise pathological classification is critical for the choice of chemotherapeutic regimen in cases of lymphoma . in the early 1990s ,
eus - fna was first used for tissue biopsy of tumors around the gastrointestinal tract . in spite of its high accuracy
, the lack of sufficient tissue material obtained using this technique often renders immunohistochemical staining inconclusive and limits its application in the classification of lymphomas . how to obtain an adequate sample that can be used for immunohistochemical testing is a primary problem in need of a solution . in a study by mohamad et al .
, the results of eus - fna of two patients who had suspected lymphoma were negative .
however , positive results were obtained by use of eus - guided trucut biopsy ( eus - tcb ) .
the authors suggested that the use of eus - tcb with 19 g needle could permit more tissue materials to be obtained in those who had a negative eus - fna result .
this minimally invasive procedure can be used for the preoperative diagnoses of some difficult cases .
analyzed and compared the efficacies of eus - fna and eus - tcb in the diagnosis and classification of lymphoma . among the 24 included patients , 23 patients underwent eus - fna .
only one patient received eus - tcb alone , while the other 22 patients received both eus - fna and eus - tcb .
the results showed that the accurate diagnostic rate of eus - guided biopsy was 79% ( 19/24 ) and the accurate rate of eus - guided biopsy for classification was just 66.6% ( 16/24 ) .
this indicated that eus - guided biopsy was of limited value in the classification of lymphoma .
there have been more than 300 reports of notes clinical applications from around the world . over 50 cases of cholecystostomy and endectomy by transvaginal notes
have been performed in germany , and 116 notes surgeries ( including 77 transgastric cholecystotomy cases ) have been reported in brazil . however , there are few case reports regarding the clinical application of notes in china .
wang et al . presented a case of laparoscopy - assisted transgastric endoscopic biopsy of a retroperitoneal lymph node . in this case ,
an endoscopic full - thickness resection ( eftr ) with the aid of laparoscopy was used to extirpate an enlarged retroperitoneal lymph node .
pathological and immunohistochemical tests confirmed the diagnosis of retroperitoneal b - cell lymphoma ( diffuse large b - cell lymphoma ) . in recent years ,
these studies showed that eus was very useful for creating transgastric access and locating the targets . in this study , we successfully used eus - assisted notes to perform enucleation of a retroperitoneal enlarged lymph node without laparoscopic assistance .
the use of a minimally invasive procedure not only enables more tissue materials to be obtained but also causes less trauma than a laparoscopy - assisted approach . in this patient
, blood test results recovered to normal levels by 4 days after the procedure and the highest postoperative body temperature was just 37.2c .
he was also able to return to a normal diet 3 days after the procedure .
our experience suggests that this is an alternative and minimally invasive approach for the biopsy of retroperitoneal lymph nodes .
thirty patients with zygomatic complex fractures were treated with one point fixation [ figures 13 ] .
preoperative peripheral nerve stimulation x - ray preoperative computed tomography scan under general anesthesia , nasoendotracheal intubation was done .
the incision can be made from anterior to posterior or from medial to lateral and should extend through mucosa , submucosa , and any buccinators muscle fibers [ figure 4 ] .
rowe 's zygomatic elevator was then inserted behind the infra temporal surface of the zygoma , and bone was reduced into its correct anatomical position using superior , lateral and anterior force . an audible click and fullness of the cheek together with palpation for normal contour of the zygomatic bone and orbital rim gave an idea about the adequacy of the reduction .
one hand over the side of the face was used to assist in the reduction .
a four hole plate with a gap was fixed with 4 mm 2.5 mm screws on the zygomatic buttress [ figure 5 ] .
immediate post operative immediate peripheral nerve stimulation x - ray six months postoperative and peripheral nerve stimulation x - ray
for all the patients , immediate postoperative and 6 months postoperative peripheral nerve stimulation x - rays were taken , and the x - rays review successful reduction .
none of the patients complained of any paresthesia , bony movements or pain in the frontozygomatic or zygomatic buttress region .
since intraoral approach was used , all the patients had an aesthetic facial profile without any unsightly scars .
the integrity of the zygoma bone is critical in maintaining normal facial width and prominence of the cheek .
the zygomatic bone is a major contributor to the orbit and plays an important role in protecting the eyes .
zygomatic bone alone is rarely involved in fractures ; usually its articulating surfaces which are maxilla , temporal , frontal and sphenoid bones are also involved .
the fractured fragments of a tripod or tetrapod zygomatic complex fracture near these suture lines needs to be restabilized by open reduction followed by fixation .
studies suggest that two point gives a considerable stabilization , and three point fixation gives the maximum stabilization .
however other studies suggest that one point fixation for zymatic complex fractures gives an excellent results considering the esthetics and stabilization for simple tripod fractures without any comminution of the zygomatic bone or the lateral orbital wall one point fixation with a single mini plate in the frontozygomatic area through the lateral eyebrow incision have been suggested by many authors .
i n these cases it was found that when a tripod fracture without any comminution or mild or no displacement can be stabilized very well with a single point fixation in the frontozygomatic area without any complications of diplopia or enopthalmos .
however , zygoma provides the attachment point for muscles of mastication and facial animation , but amongst these , it is the masseter that provides the most significant intrinsic deforming force on the zygomatic body and arch .
the integrity of zygomatic buttress is necessary for withstanding the contraction force of the masseter muscle . in 2002
fujioka et al .
in vivo studies successfully proved that one point fixation at the zygomaticomaxillary complex gives three point alignment and sufficient rigidity when the fractures are not comminuted . in 2011
kim et al . found out that lateral eyebrow incision for mini plate fixation at the frontozygomatic area led to unaesthetic scar and few patients underwent plate removal through a second surgical re - entry through the existing scar of the lateral eyebrow incision which further enhanced the unsightly scars and compromised facial esthetics . since the skin over the lateral eyebrow region is thin there are more chances of palapation of the mini plates after fixation , and it may lead to pain . as early as in 1994 tarabichi et al .
proved that in vitro studies are misleading regarding the mini plate fixation along the orbital margins and successfully applied transsinus reduction through anterior comminuted sinus wall . in 2012 kim et al .
successfully reduced the zygomatic complex fractured fragments through intraoral approach and gained sufficient rigidity and excellent esthetics with one point fixation at the zygomatic buttress region .
we also found that one point fixation with a single mini plate at the zygomatic buttress through intraoral incision provided excellent stability and esthetics in the selected cases of simple zygomatic complex fractures without any comminution of the zygoma or the lateral orbital rim without or with minimal displacement and none of our patient complained of pain or palpation or bony movements in the postoperative study period of 6 months rather they were happy to get operated without any unaesthetic facial scars . | since its introduction in the early 1990s , endoscopic ultrasound - assisted fine - needle aspiration ( eus - fna ) has been used for sampling of extraintestinal mass lesions and peri - intestinal lymphadenopathy .
although eus - fna is highly accurate , lymphomas can be challenging to diagnose using eus - fna .
we present the case of a 60-year - old male who had experienced upper abdominal discomfort for 1 month .
computerized tomography ( ct ) examination revealed multiple soft - tissue shadows located above the pancreatic body .
the biggest shadow had a cross - sectional area of 7.7 cm 7.2 cm .
positron emission tomography - ct ( pet - ct ) imaging showed increased uptake of 18f - fdg by these soft - tissue shadows . to investigate further ,
eus was performed and it revealed the presence of multiple hypoechoic round lymph nodes . during the procedure ,
eus - fna was performed , but only a few dyskaryotic cells were observed by cytological evaluation .
eus - assisted retroperitoneoscopy and lymph node biopsy were performed to obtain more tissue for immunohistochemical analysis and subclassification of lymphoma .
finally , the patient was diagnosed with non - hodgkin lymphoma , germinal center b - cell - like diffuse large b - cell lymphoma by this technique .
eus - assisted transendoscopic retroperitoneal lymph node biopsy is an alternative procedure for the diagnosis of lymphomas . |
sotos syndrome is a dysmorphic syndrome characterized by early overgrowth , developmental delay , advanced bone age and characteristic craniofacial appearance .
sotos syndrome results from mutation involving the nuclear receptor set - domain - containing protein ( nsd1 ) gene , located on chromosome 5q .
the mutational mechanism can be a point mutation in the nsd1 gene or a microdeletion that includes nsd1 .
fluorescence in situ hybridization ( fish ) did not detect microdeletion of 5q35 in this patient .
nsd1 gene mutations are also found in weaver syndrome where camptodactyly is a common feature .
this report describes camptodactyly for the first time in a girl with sotos syndrome and provides further evidence that sotos and weaver syndrome are allelic disorders .
we describe a two and half years old girl born of non - consanguineous tamilian parents .
milestones were delayed with head holding at 6 months , walking at 2 years and delayed speech .
family history was unremarkable . at two and a half years she weighed 15 kg ,
height was 97 cm and head circumference was 52.5 cm ( all above 90 centile ) .
she had a high forehead with frontal bossing , dolichocephaly , large ears , pre - auricular pits , down - slanting palpebral fissures , high arched palate , pointed chin and pectus carinatum [ figure 1 ] .
she had three caf au lait spots distributed on the chest and trunk , large hands and camptodactyly of the left hand [ figure 2 ] .
fish studies carried out using probe rp11 - 265k23 did not reveal a microdeletion of the 5q35 region .
face showing the facial features of sotos syndrome left hand showing camptodactly a clinical diagnosis of sotos syndrome was made based on the criteria comprising of rapid early growth , advanced bone age , developmental delay and characteristic facial appearance .
camptodactyly in sotos syndrome has not been previously described in literature to the best of our knowledge .
a high frequency of congenital heart defects has been reported in patients with intragenic mutations of the nsd1 gene and phenotypic overlap with other overgrowth syndromes , in particular with weaver syndrome is seen . though this sotos patient is likely to have a point mutation of the nsd1 gene , this could not be confirmed due to lack of facilities .
sotos syndrome was first recognized as a distinct clinical syndrome in new england in 1964 .
the diagnosis is based on the clinical criteria of rapid early growth ( pre and post natal ) , advanced bone age , developmental delay and characteristic facial appearance .
growth is rapid in the first years of life but final height may not be excessive . intellectually , the iq ranges from 21 to 103 with a mean of 74 and almost half of affected children achieve normal schooling .
behavioral issues are common and are one of the key areas that can influence the outcome .
hypotonia is usually present from birth and although this improves during childhood , subtle evidence may remain even in adults .
congenital heart disease is not very common in this condition and overall incidence of cardiac defects is approximately 8% .
an association of sotos syndrome with tumor development was documented over 30 years ago and has been a point of debate ever since .
gorlin et al . estimated a risk of 3.9% of benign or malignant tumors in sotos syndrome .
handicaps in sotos syndrome are fewer than previously believed and tend to improve with age . in patients with sotos syndrome harboring a chromosomal translocation kurotaki et al
. isolated the nuclear receptor set - domain - containing protein ( nsd1 ) gene from the 5q35 breakpoint .
added noted a large difference between japanese and non - japanese patients in the frequency of microdeletions , which occurred in 49 ( 52% ) of the 95 japanese but in only 1 ( 6% ) of the 17 non - japanese .
there was a strong correlation between presence of an nsd1 alteration and clinical phenotype , in that 28 of 37 ( 76% ) patients with typical sotos or sotos - like phenotype had nsd1 mutations or deletions .
studied nsd1 gene in a series of typical sotos patients ( 23/39 ) , sotos - like patients ( lacking one major criteria , 10/39 ) and weaver patients ( 6/39 ) .
they conclude that nsd1 mutations account for most cases of sotos syndrome and a significant number of weaver syndrome cases . comparing the clinical phenotype of children carrying either a deletion or a mutation , rio et al . failed to detect distinctive features except for the severity of mental retardation .
they reported , 4/6 children carrying a nsd1 deletion were extremely severely mentally retarded with no language at all , major delay in motor milestones and autistic features .
by contrast , in patients carrying nsd1 mutations , mental retardation was usually mild to moderate with verbal skills being more affected .
the major differential diagnoses for sotos syndrome are other conditions with overgrowth including beckwith - weidemann , weaver , nevo and simpson golabi behmel syndromes .
. however nsd1 gene mutations have been found in beckwith - weidemann syndrome , weaver syndrome and the 11p15 abnormalities seen in beckwith - wiedemann syndrome have been found in some cases of sotos syndrome . camptodactly has not been previously described in literature to the best of our knowledge with sotos syndrome but is seen in weaver syndrome where nsd1 mutations have been described .
these could be due to , allelic heterogeneity , effect of other modifying genes ethnic background and nutritional status adding to the overall expression of a syndrome .
microdeletions of chromosome 5 were not detected in our case suggesting a likely point mutation in the nsd1 gene and further evidence of that weaver and sotos syndrome are allelic .
further delineation of the phenotype with molecular studies will provide correct genotype - phenotype correlations . in march 2013 , cases infected with a novel reassortant avian - origin influenza a ( h7n9 ) virus emerged in china and had high mortality . that month
, a patient with h7n9 influenza was admitted to our hospital , and daily lung ultrasound was performed . a 54-year - old woman , who ran a convenience store beside a poultry market , complained of cough and high fever for 4 days .
her temperature was 38.6c , and she had a heart rate of 113 beats per minute and a respiratory rate of 26 breaths per minute .
her white blood cell count was 2.7 10/l , and neutrophil , lymphocyte , and monocyte levels were 72.4% , 22% , and 5.2% , respectively .
her partial pressure of oxygen in arterial blood was 72 mm hg , and her fraction of inspiratory oxygen ( fio2 ) was 40% .
avian - origin influenza a ( h7n9 ) virus was confirmed from the pharyngeal swabs by real - time reverse transcriptase - polymerase chain reaction .
treatment with oseltamivir ( 150-mg capsule taken by mouth twice a day ) was initiated , and she was admitted to an isolated room in the infectious diseases department .
bothell , wa , usa ) with c60 convex probe ( 2 to 5 mhz ) was performed , and the lung ultrasound score ( lus ) was recorded , and both effectively reflected the progression of pneumonia ( figure 1 ) .
dynamic changes of chest computed tomography ( ct ) , radiography , and lung ultrasound in a patient with h7n9 influenza .
( a - c ) ct showed pneumonia in the left upper lung , with partial consolidation on admission .
( d - f ) lung ultrasound corresponding to ct in ( a - c ) showed multiple abutting b2 lines , and some regions presented a tissue pattern ( arrow ) .
( g - i ) ct on day 6 after admission showed that the pneumonia was partially absorbed .
( j - l ) lung ultrasound corresponding to ct in ( g - i ) showed that the number of b lines was obviously decreased , and the consolidation disappeared .
( m - o ) ct on day 9 after admission showed that the size of the lesion was obviously reduced .
( p - r ) lung ultrasound corresponding to ct in ( m - o ) indicated that only the a line and few b lines were visible .
( s - u ) chest radiography on days 1 , 3 , and 6 after admission showed no obvious change of the pneumonia .
( v ) dynamic changes of lung ultrasound score ( lus ) ( total of 48 for normal lung ) .
the onset of h7n9 influenza in this case was manifested by hyperpyrexia and flu - like symptoms and progressed to lobar pneumonia 4 days later .
chest radiograph is the routine tool for assessment of pneumonia , but its sensitivity and accuracy were not so good .
ct is regarded as the gold standard , but its application is limited in cases with this new emerging virus since strict protection should be followed to avoid person - to - person transmission .
ultrasound has many advantages , including convenience , rapidness , non - invasiveness , availability for repeated examination , and absence of radiation .
the right lung presented an a line with few isolated b lines , whereas the left lung presented multiple abutting b lines and consolidation . when the patient improved , both b lines and the area of consolidation were decreased and the lus was synchronously increased .
this case highlights that ultrasound can be an adjutant to chest radiography and ct in caring for patients with h7n9 influenza .
written informed consent was obtained from the patient for publication of this letter and accompanying images . | we describe a girl with sotos syndrome presenting at two and a half years age with developmental delay .
she has camptodactyly which has not previously been reported in sotos syndrome but is a common finding in weaver syndrome .
both these conditions have been reported to have nsd1 gene mutations .
this report is consistent with the conditions being allelic . |
a 23-year - old engineering graduate presented with primary palmoplantar hyperhidrosis , for which he was advised an alternate day schedule of tap water iontophoresis .
on his next visit , he presented with a very simple iontophoresis device that he devised on his own .
the device was constructed with a rechargeable 12 volt battery , two aluminum trays and copper wires , and connecting clamps [ figure 1 ] .
hence , using his engineering background he constructed this simple device based on basic mechanism behind iontophoresis .
he followed an alternate day schedule of 20 min utes immersion for initial 4 weeks , followed by once a week for next 8 weeks .
he achieved an excellent reduction in palmoplantar sweating without any adverse effect , within 3 months of starting iontophoresis . a simple user - made iontophoresis device
iontophoresis is defined as passing of an ionized substance through intact skin by application of direct current ( dc ) .
tap water iontophoresis is a reliable and effective method for the treatment of palmar and plantar hyperhidrosis , when practiced with appropriate technique and timing .
many dermatologists consider simple tap water iontophoresis to be first line therapy for primary focal palmar and plantar hyperhidrosis .
the mechanism of production of anhidrosis is not completely understood ; however , obstruction of sweat duct has been suggested as a possible cause .
few brands of iontophoresis devices are commercially available ; however , they are expensive and are not readily available . commercially available
construction of an iontophoresis device has been described by levit , in which output of the 115 volt isolation transformer is rectified by the full wave selenium rectifier and then filtered by the choke and capacitors , and the potentiometer acts as a voltage divider .
levit had suggested that such devices can be constructed at home , and circuit diagram could be found in his original report , but we feel that the procedure for assembling such a device will be difficult for a layperson . for tap water iontophoresis , patients are instructed to apply petroleum jelly with a cotton swab to cover any cuts over the treated area before the session .
the trays should be filled with tap water , then , the affected areas should be immersed in the tap water .
a monday - wednesday - friday schedule should be followed until the condition improves ; subsequently , the treatment should be tapered once a week for 8 weeks and then once a month for maintenance .
additionally , the patient should wear rubber or plastic footwear and should keep himself from directly touching the floor . burning and pin pricking sensations are very common and
erythema and vesiculation are transient ; topical corticosteroids cream can be applied for persistent erythema and vesiculation .
pregnant women , people with pacemaker or metal implants , cardiac conditions , or epilepsy are contradictions for the use of iontophoresis machine .
once a home device is obtained and the patient has received adequate education and training , the maintenance cost and effort are minimal for the patient and health care provider alike .
iontophoresis machines basically produce a voltage sufficient to drive a dc of 15 - 20 ma through the hands of patients .
an ampere - meter could be used to measure the output current of such user - made devices and can upgrade the voltage of the battery , provided the output is low in terms of current .
simple user - made devices such as this one would make the process of iontophoresis very easy , safe , and cost - effective . there are no conflicts of interest .
a 61-year male presented to the emergency department with a history of road traffic accident .
he arrived hemodynamically stable with a blood pressure of 126/76 mmhg and a heart rate of 78 beats per minute . on plain radiograph ( fig .
1a ) anteroposterior and two judet 45 oblique view1 ) and computed tomography ( ct ) scan of pelvis ( fig .
1b ) , the findings revealed both column fracture of acetabulum without hip dislocation , but no presence of femoral head fracture or onfh .
buttress plating through ilioinguinal approach was performed using a reconstruction plate , which was supplemented by a compact hand plate .
the patient was transfused 8 units of whole blood , 3 units of fresh frozen plasma and 8 units of packed red blood cells .
intra - operative hb was 9.7 gm% ; the average mean arterial pressure was 91.82 mmhg during the operative procedure .
post - operatively the patient was transfused 2 units of whole blood and 1 unit of fresh frozen plasma .
the hb postoperatively was 10.3 gm% , the patient was shifted to intensive care unit for a day , later was transferred to the ward .
the post - operative x - ray ( fig . 2a ) and ct scan revealed an acceptable reduction of the fracture fragments and a concentric hip .
sitting up was performed on the first postoperative day ; the patient subsequently began formal physical therapy and active range of motion exercises .
partial toe touch weight bearing ( 20 to 30 lb ; 9 to 13.6 kg ) with a walker was maintained for 6 - 8 weeks .
progression to full weight bearing was started on the basis of the follow - up radiographs .
the patient 's 4-month postoperative x - ray revealed a radiolucent lesion in the superolateral part of femoral head , crescent sign , and sclerosis .
2b ) showed collapse and sclerosis , findings consistent with onfh . a ct scan ( fig .
on was diagnosed only when the radiographic findings provided a clear differentiation from wear of the femoral head2 ) . the joint pain increased due to the onfh
, we performed a total hip replacement ( fig . 3b ) 12 months after the index surgery .
late complications of acetabulum fractures include heterotopic ossification and onfh , which are present in less than 10% of the population3 ) .
the incidence of onfh is known to be high in transverse and posterior wall fractures associated with posterior dislocation6 ) . on
also occurs in conjunction with approximately 3% of anterior hip dislocations and in more than 13% of posterior hip dislocations . in a recent meta analysis of 3,670
surgically treated displaced acetabular fractures the incidence of onfh showed an overall incidence of 5.6%3 ) , suggesting that it is grossly overestimated and that most of the observed changes in the head of the femur are probably due to osteoarthritis5 ) .
onfh is caused by inadequate blood supply to the affected segment of the subchondral bone .
when posterior surgical approaches have been used , on rates as high as 42% within the first year after surgery have been reported7 ) .
many systemic conditions are associated with on , but 25% of all cases are described as idiopathic and can contributes as a cause9 ) .
trauma is one of the most common causes of on , interruption of the blood supply to the affected segment of the bone being the cause of ischemia . in this case
the exact cause of onfh eludes us , especially in the absence of any patient related predisposing risk factors , except presence of fracture without hip dislocation and subsequent intervention by an ilio - inguinal approach .
a probable theory of etiology could be the intra - operative hypovolaemia , low mean arterial pressure , causing compromised flow to the femoral head being so as to act as the final blow .
alteration of the blood supply to vital organs during hypovolaemia is well established . with mean arterial pressure usually in the range of 50 to 60 mmhg ,
the flow to the femoral head is potentially compromised10 ) so as to act in an accumulative stress theory , as suggested by kenzora and glimcher9 ) .
it is questionable as to whether this alone would be enough to explain the development of on . | iontophoresis is defined as passing of an ionized substance through intact skin by application of direct electric current .
tap water iontophoresis is reliable and effective method for treatment of palmar and plantar hyperhydrosis when practiced with appropriate technique and timing .
one of the major setback for using iontophoresis is that the apparatus is expensive and is not readily available .
a simple user - made iontophoresis device have been described here , which could be easily constructed and used at home . |
pericardiocentesis is an invasive procedure which is usually performed in a patient who has pericardial effusion to resolve the pressure in the pericardial sac . in 1653 , riolanus ( 1 ) first described as a trephination of the sternum to relieve fluid surrounding the heart . due to frequent complications this procedure was out of interest until ultrasound guided technique emerged ( 2 ) .
herein , we report a case of iatrogenic tension pneumopericardium , which exhibit impending cardiac arrest .
a 70-year - old male presented with severe dyspnea and general weakness on march 8 , 2013 .
he was referred from a local hospital for pericardiocentesis and further work - up due to a large pericardial effusion .
he had a past medical history of ischemic stroke 15 years prior to his visit to the hospital and of syncope a day before his visit to the hospital .
the vital signs of the patient in the emergency room ( er ) were as follows : blood pressure of 163/93 , heart rate of 135 beats per minute , respiratory rate of 30 breaths per minute , body temperature of 37.0c , and oxygen saturation of 91% .
the patient had distended neck veins and muffled heart sounds but did not have low blood pressure .
echocardiography was performed by an emergency physician , and a right ventricle ( rv ) free wall collapse in was observed in the diastolic phase .
an emergency pericardiocentesis was indicated , and it was performed by a subxiphoid approach . approximately 700 ml of serous effusion was drained through a catheter ; the patient s heart rate dropped to 110 and his blood pressure was maintained above 130 .
a water - sealed chest tube bottle was connected at the end of the catheter for further drainage .
the patient was transferred to a computerized tomography ( ct ) room for a chest ct to examine the cause of his pericardial effusion . before he left
after the patient returned to the er from the ct room , his heart rate fell to 30 , and he exhibited impending cardiac arrest .
an intravenous dose of 0.5 mg atropine was given , and the patient was hydrated with crystalloid solution .
the patient s chest ct confirmed tension pneumopericardium , and imaging showed that pericardial air was compressing the right ventricle and that the catheter tip was placed behind the left ventricle ( lv ) ( fig .
1 ) . approximated 500 ml of pericardial air was evacuated rapidly through the previously implanted catheter , and the patient s vital sign became stable .
the patient was admitted to the intensive care unit and subsequently transferred to a long - term care hospital . a contrast - enhanced computed tomography scan of the chest . ( a ) axial view showed the air compressing the right ventricle ( arrows ) and tip of the catheter inside pericardium ( arrow head ) .
pneumopericardium is defined as the presence of air inside the pericardial space . in 1910 , wenkebach first described the x - ray findings of pneumopericardium , and in 1967 , cimmino ( 6 ) described the diagnostic features of pneumopericardium . in a review of the literature , toledo et al .
( 7 ) classified the etiology of pneumopericardium into four categories : iatrogenic , pericarditis , fistula formation between the pericardium and an adjacent air - containing organ , and trauma .
( 8) reported that trauma and positive pressure ventilation are the major causes of pneumopericardium .
iatrogenic cases of pneumopericardium associated with various procedures , such as thoracentesis , paracentesis , poststernal bone marrow aspiration , postcauterization of esophageal webs , pericardiocentesis , radiofrequency ablation , and pacemaker insertion , have been reported ( 7910 ) .
if the patient has tension pneumopericardium , physical examination may show tachycardia , distended neck veins , and hypotension . in 1844 , bricheteau was the first to describe the mill wheel murmur in auscultation , which is a characteristic churning or splashing auscultatory sound due to blood mixing with air in the pericardial sac . a chest x - ray showing air surrounding the heart and the small heart sign
pericardial air can be mistaken for mediastinal air , but the air in the pericardial sac does not rise above the pericardial reflection of the proximal great vessels . also a chest x - ray taken from the decubitus position may show a shift of air if the air is in the pericardium , but mediastinal air does not shift ( 12 ) .
a small amount of air in the pericardial sac is indistinguishable by plain chest x - ray but can be detected by ct scan .
bedside echocardiography in the er is an alternative method for detecting air in the pericardial sac , but a large amount of air may hinder the penetration of the ultrasound beam , so diagnosis by this method may be challenging .
the needle should be placed 1 cm inferior to the left xiphocostal angle at a 30-degree angle to the skin .
it should be aimed toward the left shoulder and advanced while maintaining negative pressure ( 13 ) .
after the insertion of the catheter , a drainage tube is connected to a water sealed device . in our case , a conventional chest tube bottle was used , and during transportation to the ct room , the drainage device was not clamped .
, the water in the bottom of the bottle could have swung back and forth .
originally , the tube connected to the catheter would have been sealed with water , but this swinging motion would have provided the opportunity for air to enter the catheter and , subsequently , the pericardial sac .
iatrogenic pneumopericardium is rarely reported after pericardiocentesis , but it can lead to tension pneumopericardium , which is a life threatening condition .
physicians should be aware of this serious complication of pericardiocentesis and take extra precautions in handling drainage devices because this iatrogenic complication can lead to cardiac arrest and a medical dispute .
she was referred to saitama hospital due to severe headache and nausea on october 2008 .
brain mri detected a 1.5 cm abscess mass with extensive edema in the right frontal lobe .
we performed intensive therapy using some antibiotics that included cefotaxime and meropenem and depressants for intracranial pressure for six weeks .
there was a good prognosis for the woman and her fetus without any sign of neurological abnormalities .
early medical intervention is required before it is too late for brain abscess in pregnancy .
brain abscess caused by bacterial infection has extremely low incidence , and a high mortality rate of 30% .
it causes poor prognosis for both mother and fetus , regardless of the state of pregnancy . unlike non - pregnant women
a 24-year - old woman who lived in saitama , japan had three pregnancies , two childbirths , body mass index ( bmi ) of 22.3 , and unremarkable past medical and family histories .
she also had an uneventful first trimester , but developed a fever of > 39c at 22nd week , 1st day of pregnancy .
because of prolonged headache and nausea , she was referred to our hospital in saitama for complete physical examination on october 2008 . on admission , she had blood pressure of 103/51 mmhg , heart rate of 100 beats per min ( bpm ) , body temperature of 39.0c , mild stiffness in the neck , and cold extremities .
however , brain computed tomography ( ct ) for the prolonged headache revealed a 1.5 cm mass in the right frontal lobe , while hematological analysis showed an elevated white blood cell count of 12,400 cells/l ( neutrophils , 87.7% ) .
cerebrospinal fluid findings were positive for gram - positive bacteria , an increased cell count ( especially for neutrophils ) of 2,332 cells/l , and a low glucose concentration of 30 mg / dl .
brain mri revealed a 1.5 cm mass with a high intensity signal inside and a low intensity t2 signal on the margin in the deep white matter of the right frontal lobe .
based on the above findings and a high intensity zone surrounding the mass on diffusion - weighted images , she was immediately placed on intensive therapy with concurrent administration of antibiotics cefotaxime ( 2 g / day ) and meropenem ( 3 g / day ) , as well as glycerin 20 g / day to reduce intracranial pressure .
table 1 shows a list of examinations performed in search of causal factors , while the results show the isolation of methicillin - sensitive staphylococcus aureus ( mssa ) from the throat . on the other hand , she had no dental problems . because of unremarkable upper gastrointestinal endoscopy findings and a negative fecal occult blood test result , the possibility of brain metastasis of a malignant tumor was ruled out .
after six weeks of intensive therapy with concurrent administration of two antibiotics and glycerin , the headache and nausea disappeared along with a reduction in the number of white blood cells .
subsequent brain mri at 28th week , 4th day of pregnancy showed no enlargement of the abscess and disappearance of the surrounding edema , with no indication of puncture drainage . at this point , she was switched to oral administration of amoxicillin 750 mg / day for four weeks and was discharged at 29th week , 3rd day of pregnancy .
body temperature slowed down after the day 7 causes of brain abscess mssa : methicillin - sensitive staphylococcus aureus , cns : coaglese negative staphylococcus she vaginally delivered a 2,890 g girl baby at 38th week , 5th day of pregnancy , with no abnormalities .
no neurological abnormalities were evident during a five - year follow - up observation conducted over the phone .
mri findings at the 22nd and 28th week of pregnancy are shown in figure 2 .
a : axial image at 22ne week of pregnancy shows the large right frontal abscess with severe edema .
b : axial image at 28th week of pregnancy shows no enlargement of the abscess and disappearance of the surrounding edema
despite the extremely low incidence , brain abscess caused by bacterial infection has a high mortality rate of 30% and is therefore a disease with poor prognosis for both mother and fetus , regardless of the state of pregnancy .
although we listed previous reports on brain abscess during pregnancy ( table 2 ) , it should be noted that the number is extremely small ( 16 ) .
approximately , 7% of the previous cases were related to dental treatment ( 7 ) , but no dental abnormalities were observed in the present case . during the pregnancy , maternal immunity is reduced due to a hormonal imbalance , and according to lanciers et al .
, 26.6% of pregnant women , as opposed to 11.0% of non - pregnant women , are significantly infected with helicobacter pylori ( 8) .
it goes without saying that organisms with low pathogenicity under normal circumstances can cause serious infection during pregnancy . in this case , the clear source of infection was not identified .
it seems that the pregnant woman whose immunity was diminished is vulnerable to mssa , which was extremely rare and considered as a serious case .
brain abacess in pregnancy ( literature review ) the symptoms of brain abscess include headache , nausea , and localized neurological abnormalities ( 9 ) .
headache is the most common symptom , occurring in 75% of pregnant women , followed by 67% of neurological abnormalities and 58% of altered consciousness ( 10 ) .
although no adverse effects of mri have been reported ( 11 , 12 ) , the ct should be avoided as much as possible because there are some problems about the degree of radiation exposure in pregnant women .
therefore , mri may be a safer and is a highly sensitive diagnostic imaging modality for use in pregnancy ( 13 ) .
yet , because of potential thermal tissue damage due to the high magnetic field , the national radiological protection board recommends that pregnant women avoid mri examination during the first trimester . to treat a brain abscess ,
it is necessary to select antibiotics capable of effectively crossing the blood brain barrier and their sensitivity should be proven in bacterial culture .
the use of steroidal drug is also recommended to prevent an increase in intracranial pressure and the development of brain edema ( 13 ) .
however , because intensive therapy for a brain abscess with antibiotics and steroidal drugs takes somewhere between six to eight weeks , its effect on the fetus is a huge concern .
betamethasone and dexamethasone , which are transported via the placenta , should be avoided because they may affect the development of the fetal central nervous system .
furthermore , the early administration of antiepileptic drugs is recommended because 70% of patients with a brain abscess develop epilepsy ( 13 ) .
even infection by vulnerable bacteria becomes serious and early treatment intervention is desirable because immunity power diminishes during the pregnancy .
our treatment obtained ethics approval from the regional ethics committee responsible for human experimentation and conformed to the provisions of the declaration of helsinki . | pneumopericardium is defined as the presence of air inside the pericardial space .
usually , it is reported as a complication of blunt or penetrating chest trauma , but rare iatrogenic and spontaneous cases have been reported .
pneumopericardium is relatively stable if it does not generate a tension effect on the heart .
however , it may progress to tension pneumopericardium , which requires immediate pericardial aspiration .
we report a case of iatrogenic pneumopericardium occurred in a 70-year - old man who presented dyspnea at emergency department .
the patient underwent pericardiocentesis for cardiac tamponade due to large pericardial effusion , and iatrogenic tension pneumopericardium occurred due to misuse of the drainage device . after evacuating the pericardial air through the previously implanted catheter , the patient became stable .
we report this case to increase the awareness of this fatal condition and to help increase the use of precautions against the development of this condition during emergency procedures . |
caustic esophageal injury in infants is a devastating insult to the gastrointestinal tract and will often require major reconstructive surgery to replace the damaged esophagus . esophageal replacement with colonic
interposition has been utilized since dale and sherman performed the first retrosternal colonic interposition in 1955 . up to 80% of patients with colonic interposition
endoscopic dilation is relatively safe and effective for the initial treatment of anastomotic strictures , but surgical management is indicated in refractory cases . when surgery is required , graft revision utilizing both a thoracotomy and laparotomy is common .
we report a case of cologastric stricture treated with resection and reconstruction of the anastomosis solely through an abdominal approach , which can offer less morbidity and mortality .
a 31-year - old male developed a caustic esophageal injury after ingestion of an alkaline solution when he was 2 years old .
he required emergent esophagectomy , proximal gastrectomy and reconstruction with a colonic interposition graft from the cervical esophagus to the stomach .
the patient also had a pyloric stricture , for which a gastrojejunostomy was performed . over the next three decades , he required frequent hospital admissions for abdominal pain and dysphagia , and had multiple endoscopic dilations performed for a severe cologastric anastomotic stricture ( fig .
the patient had severe malnourishment , with a body mass index of 14 kg / m .
he had a jejunostomy feeding tube for nutritional support , which had been removed due to abdominal pain a few months prior to presentation .
endoscopic evaluation revealed the severe cologastric stricture , severe inflammation just proximal to the cologastric anastomosis , and significant ulcerative disease at his gastrojejunal anastomosis .
the patient underwent resection of the cologastric anastomosis and the gastrojejunal anastomosis with ulcerated stomach , neo - cologastric anastomosis and neo - gastrojejunal anastomosis via a transabdominal approach without a thoracotomy .
intraoperative endoscopy was utilized during the case to ensure that the cologastric anastomotic stricture was entirely resected .
figure 2:preoperative cologastric and pyloric strictures ( a ) and post - operative changes including new cologastric , gastrojejunal , duodenojejunal anastomoses ( b ) .
preoperative cologastric and pyloric strictures ( a ) and post - operative changes including new cologastric , gastrojejunal , duodenojejunal anastomoses ( b ) .
the patient tolerated the procedure well and was transferred to the intensive care unit . on post - operative day 2 , he was transferred to the floor .
his post - operative course was remarkable for development of an intra - abdominal fluid collection , which required percutaneous drainage , but was otherwise uncomplicated .
severe esophageal damage may require resection with creation of a neo - esophagus . because these operations occur in children , complications in regards to the colonic interposition graft
classically , a transthoracic approach has been used to resect the entire colonic graft . in this situation
, a repeat colon graft may be needed , which carries high morbidity and mortality .
in particular , the majority of the proximal colon graft , which was functional , was able to be spared .
given our patient 's preoperative nutritional status , he would be at a predisposed risk for wound healing complications . via transabdominal approach ,
to the best of our knowledge , this is the first written report of using a completely transabdominal approach for revision of the colonic graft .
our patient had previously been refused surgery by multiple surgeons . if this relatively less complex surgical management was used , perhaps he would have had definitive treatment much earlier .
our patient did well with our transabdominal approach given the chronicity of his symptoms . in light of his clinical dilemma of continued non - operative versus operative intervention , his symptoms were relieved immediately .
this surgical approach should be considered in patients with history of colonic interposition requiring reoperation for complications of the colonic graft . sternoclavicular joint septic arthritis ( ssa ) and its clinical presentation are infrequently seen and often difficult to manage .
presenting symptoms of ssa can vary , with chest and shoulder pain being the most common clinical features .
after thorough literature search , no cases have yet been reported on ssa leading to vocal cord palsy .
vocal cord palsy is an important sign of thoracic and head and neck pathology that is caused by an extremely wide set of pathology .
a 67-year - old gentleman presented to the emergency department with a 3-week history of worsening dysphagia and hoarse voice .
routine examination of the patient in the emergency department revealed that he was haemodynamically stable and apyrexial and exhibited tenderness in the left anterior neck .
examination by the otolaryngology team demonstrated no evidence of cervical lymphadenopathy but tenderness of the lower left anterior triangle , as well as evident swelling , erythema and mild bruising of the anterior chest wall . on questioning the patient regarding this , he revealed that he burnt his chest using a hot water bottle 3 weeks previously and he also admitted to having stiffness and pain in the left shoulder over this same period .
indirect laryngoscopy with flexible nasendoscopy revealed non - discrete swelling / oedema of the left pharayngeal wall and reduced mobility of the left vocal cord .
routine haematological investigation revealed a white cell count of 18.6 10/l , c - reactive protein of 288 mg / l and platelets of 499 10/l .
no other haematological abnormality was noted on admission . with a working differential of parapharyngeal space infection and possible malignancy ,
the patient was referred for a computed tomography ( ct ) scan of the neck and thorax with contrast .
the patient was also started empirically on intravenous co - amoxiclav as treatment for neck space collection .
ct imaging , performed 24 h after admission , revealed no evidence of malignancy or indeed any paraphayrngeal space collection .
few small lymph nodes were noted on the left side of the neck , but were deemed to be reactive in nature , and left vocal cord palsy was evident ( fig . 1 ) .
the key finding was that of a left sternoclavicular joint collection and closely associated superficial anterior chest wall , soft tissue swelling and oedema ( fig . 2 ) .
this inflammatory process was also noted deep to the manubrium and sternum and extending somewhat into the mediastinum with evident enlarged mediastinal lymph nodes ( fig .
the ct findings were in keeping with ssa with associated superficial and deep tissue inflammation and oedema . with no other
cause found , the vocal cord palsy was attributed to the inflammation within the mediastinum , which in turn was caused by superficial burn from hot water bottle use .
findings : left vocal cord palsy indicated by the para - median position of the left vocal cord in comparison with the right .
findings : left sternoclavicular joint collection and closely associated superficial anterior chest wall , soft tissue swelling and oedema .
findings : soft tissue oedema of the chest seen retrosternally ( marker a ) and superficially on the left anterior chest ( marker b ) as well as evidence of mediastinal lymph node enlargement .
computed tomography . a 67-year - old male with ssa . findings : left vocal cord palsy indicated by the para - median position of the left vocal cord in comparison with the right .
findings : left sternoclavicular joint collection and closely associated superficial anterior chest wall , soft tissue swelling and oedema .
computed tomography . a 67-year - old male with ssa . findings : soft tissue oedema of the chest seen retrosternally ( marker a ) and superficially on the left anterior chest ( marker b ) as well as evidence of mediastinal lymph node enlargement .
the antibiotic regimen for the patient was converted to intravenous benzylpenicillin ( 1.2 g four times per day ) and flucloxacillin ( 1 g four times per day ) .
response to antibiotic therapy was limited in the first few days of admission with little change in haematological inflammatory markers .
after discussion with the microbiologist , the flucloxacillin was increased to 2 g four times a day on the fourth day of admission .
the patient made slow but positive improvements over the course of the next 8 days while on intravenous antibiotics , after which he was successfully discharged .
he continued on oral antibiotics and on outpatient review 3 weeks after his admission , his external swelling , erythema and voice had returned to normal .
repeat ct performed 3 months following discharge demonstrated complete resolution of the deep and superficial inflammatory process as well as the mediastinal lymph nodes .
vocal cord palsy can be due to weakness in one or both vocal cords , and diagnosis is made when reduced mobility is evident by laryngoscope examination . in a review of 117 cases ,
benninger et al . attributed the following as the most common causes of vocal cord palsy : surgical trauma ( 44% ) , malignancies ( 17% ) , endotracheal intubation ( 15% ) , neurological disease ( 12% ) and idiopathic causes ( 12% ) .
furthermore , a review of 389 vocal cord palsy cases by holinger et al . makes no reference to the cause being related to a septic sternoclavicular joint .
explanations for this unusual presentation of ssa seen in this case include tracking infection from the septic sternoclavicular joint , resulting in a vocal cord palsy due to reactive mediastinal lymphadenopathy . spreading infectious sequeale of ssa
significant contributing factors to these outcomes are the methicillin - resistant staphylococcus aureus ( mrsa ) strains , which are becoming increasingly prevalent .
a literature review of 180 cases shows mediastinitis as a clinical feature in up to 13% of patients with ssa .
reports of fibrosing mediastinitis and descending necrotizing mediastinitis leading to vocal cord palsy have been documented .
the mild reactive mediastinal inflammation seen in the presented case has not been presented in the literature as a cause of vocal cord palsy .
therefore , as mentioned previously , it is most likely explained by mediastinal lymphadenopathy , which is a known cause of left vocal cord palsy in malignancy and atypical infections such as tuberculosis .
it has been shown that if radiological findings indicate a limited extent of disease , then medical therapy alone should be sufficient .
this empirical antibiotic coverage should be active against s. aureus , the most common infective agent in ssa .
joint resection would only be indicated if extensive bony destruction , chest wall phlegmon or abscess , retrosternal abscess , mediastinitis or pleural extension is evident on ct / mri imaging .
it is clear from the haematological and radiological findings , as well as the response to treatment , that all the presenting features of this patient were as a result of the septic focus in the sternoclavicular joint .
| a 31-year - old gentleman who had undergone an emergent esophagectomy and reconstruction with a colon interposition graft , presented with a long - standing cologastric stricture .
he had undergone multiple attempts at endoscopic dilation over multiple decades with little symptomatic relief .
he underwent a resection and reconstruction of the anastomosis entirely through an abdominal approach .
he did well from surgery and experienced complete symptomatic relief immediately .
complications of colon interposition grafts can occasionally be treated using an abdominal incision only . |
a 60-year - old male patient was referred to an otorhinolaryngology clinic due to a lump on the left side of his jaw , which had grown in 2 months .
ultrasound sonography test examination revealed a cystic mass that was 2417 mm in size with smooth contours .
multiple echogenic and reactive lymph nodes with partially visible hila were visualized in the neighboring upper jugular chain , with the largest being 1610 mm in size . following a neck magnetic resonance imaging and a preliminary diagnosis of wt ,
left superficial parotidectomy materials were sent for pathologic examination in two pieces , which were 53.22 cm and 4.531.2 cm in size .
cross section analysis showed an off white - yellowish , well - contoured nodular tumor with a bleeding center of 42.52.2 cm .
microscopic examination indicated that the tumor had epithelial components with basaloid and oncocytic columns of cells neighboring lymphoid components ( fig .
in addition to the lymphoid follicles with distinct germinal centers , infiltration of large neoplastic cells with bizarre and extremely atypical morphology was seen in the lymphoid component ( figs . 2 , 3 ) .
4b ) , leukocyte common antigen , igg , cd138 , mum1 , and focal positivity for kappa .
staining for lambda , igm , iga , cd3 , cd5 , cd10 , cd15 , cd56 , epithelial membrane antigen , bcl2 , bcl6 , cyclind1 , s100 , pancytokeratin , cytokeratin 20 , human melanoma black 45 , actin , and desmin were negative .
latent epstein - barr virus ( ebv ) was shown to be negative in tumor cells by using ebv - encoded rna chromogenic in situ hybridization . due to these findings
, the patient was diagnosed with " wt and cd30 positive diffuse large b - cell lymphoma in the parotid gland . " following the lymphoma diagnosis , a full body screen was performed .
in addition to these findings , the left suprarenal gland showed two nodular mass lesions , which were assessed as likely adenomas ; however , this preliminary diagnosis was not confirmed by histopathology .
the patient was stage 3a and received six courses of rituximab , cyclophosphamide , doxorubicin , vincristine , and prednisone ( r - chop ) therapy . during 6-month follow - up , the patient was free of disease .
wt is the second most common type of salivary gland tumor . in 10 - 15% of cases , it is bilateral , and it accounts for 70% of all bilateral salivary gland tumors.2 the male / female ratio is 1.6/1 , and it typically develops in the 6th and 7th decades . smoking increases the risk of developing wt.5 microscopically the tumors are typically composed of proliferative epithelial components accompanied by lymphoid stroma with lymphoid follicles that have distinct germinal centers .
histogenesis of the lymphoid stroma in wt has been a topic of discussion for many years .
lymphoid stroma can arise as a cell response to epithelial neoplasms or as a normal lymph node due to residue held by the epithelial neoplasm.5,6 the most widely accepted hypothesis suggests that wt is a neoplasm that develops in the heterotopic salivary gland ductus within or around the parotid lymph nodes.7 transformation to carcinoma in wt is a well - known phenomenon ; however , the development of lymphomas from wts is very rare.4,8 although some cases contain a normal residual lymphoid component , in others cases the lymphoid component contains entirely neoplastic lymphoid cells.4 in the present case , non - neoplastic lymphoid tissue was also present in the neighboring areas .
the pathogenesis of malignant transformation of wt remains unclear ; however , exposure to radiation is of particular interest , as the relationship between previous radiotherapy and lymphomas arising from wts has been determined by some authors.4,5,9 chronic immune sialadenitis is thought to play an important role , independent of the presence of sjgren syndrome symptoms.4,7,10 in this case , there was no history of radiotherapy or sialadenitis , but a history of smoking may have provoked the development of wt .
saxena et al.1 state that because the lymphoid stroma of wt is part of the systemic lymphoid tissue , in patients with lymphomatous spread of wt , disseminated disease is present during the staging either at the time of the diagnosis or after . in the present case ,
with screening techniques , lymphadenopathies of a pathologic size were found in the inguinal and iliac regions .
some researchers suggested that although the relationship between wt and lymphoma could be coincidental , it might also be of a pathogenic nature . according to the latter statement , a single agent can affect different tissues or one tumor could trigger the formation of another . from this point of view , the epithelial component is a continuous antigenic stimulator for the lymphoid component , which provides the stimulus for the development of lymphoma.1,6,8 according to this theory , the frequently observed reactive follicular hyperplasia in wt may be histological evidence of chronic antigen stimulation.1 it has been suggested that the lymphomas seen with wt are typically non - hodgkin lymphomas ; however , there are a few cases reporting hodgkin 's lymphomas.11,12 the majority of non - hodgkin 's lymphomas in wt are follicular lymphomas .
dlbcl , small lymphocytic lymphoma , extranodal marginal zone lymphoma of mucosa associated lymphoid tissue , and mantle cell lymphoma have also been reported.4,6,8,9 a small number of t - cell lymphomas such as peripheric t - cell lymphoma and t - cell lymphoblastic lymphoma have also been described in wt.4,8,13 in summary , malignant lymphomas in wt are very rare .
the presented case is a diffuse large b - cell lymphoma expressing cd30 positivity . to the best of our knowledge
this is the first case in literature describing dlbcl with expression of cd30 in wt . renal transplantation rates are low among patients highly sensitized to human leukocyte antigen ( hla ) because of the high rate of antibody - mediated rejection and subsequent graft loss .
it was recently reported , however , that preoperative desensitization using an anti - cd 20 antibody ( rituximab ) and intravenous immunoglobulin improved transplantation rates in patients highly sensitized to hla . in contrast , the significance of a positive lymphocytotoxic crossmatch in living donor liver transplantation ( ldlt ) is controversial .
successful ldlt using a liver graft in which the lymphocytotoxic crossmatch was highly positive is reported .
the recipient was a 41-year - old woman with end - stage liver disease due to alcoholic liver cirrhosis ( model for end - stage liver disease score 21 ) . at the age of 20 , she was gravida one , para one .
she was considered a candidate for liver transplantation because of repeated episodes of encephalopathy . because of the severe shortage of cadaveric donor grafts in japan , we planned an ldlt , and her husband was willing to donate his partial liver .
the abo blood type was identical , but the t lymphocytotoxic crossmatch titer was over 10,000 and the b lymphocytotoxic crossmatch titer was 128 ( complement method with the dilution technique according to the standard national institutes of health technique ) .
in addition , an examination of anti - hla antibodies using fluorescent microspheres revealed that the recipient had donor specific antibodies ( b51 and b52 ) .
after obtaining written informed consent from the patient and donor and the approval of the intra - institutional committee , we proceeded to the preoperative preparations .
for preoperative desensitization , the patient was first infused with rituximab 2 weeks before the scheduled surgery ( due to a catheter - associated infection , however , the operation was postponed and ldlt was performed 21 days after initiation of the rituximab therapy ) . as the antibody to hepatitis b core antigen was positive , entecavir ( 0.5 mg / day ) was administered for 3 weeks preoperatively to prevent a possible hepatitis b virus breakthrough .
on postoperative days 1 and 4 , 20 mg of anti - cd25 antibody ( basiliximab ) was administered in addition to the routine methylprednisolone and tacrolimus , as we were anxious about hyperacute rejection . besides , mycophenolate mofetil ( mmf ; 2,000 mg / day ) was started on postoperative day 7 .
the postoperative course was uneventful except for an episode of mild acute cellular rejection ( banff score 3 ) on postoperative day 27 , which responded promptly to steroid recycle therapy .
the liver biopsy specimen obtained at the time of the acute rejection showed mild infiltration of lymphocytes in the portal area and around the bile ducts .
one year after the ldlt , the lymphocytotoxic crossmatch remained negative and the patient has been well with good graft function.fig .
acr acute cellular rejection , alt alanine aminotransferase , mmf mycophenolate mofetil , mp methylprednisolone , pe plasma exchange , tb total bilirubin , pod postoperative day the clinical profile of the present patient .
acr acute cellular rejection , alt alanine aminotransferase , mmf mycophenolate mofetil , mp methylprednisolone , pe plasma exchange , tb total bilirubin , pod postoperative day
the impact of a lymphocytotoxic crossmatch - positive liver graft on acute cellular rejection and graft survival remains controversial , both in deceased donor liver transplantation [ 3 , 4 ] and in ldlt [ 57 ] .
some institutions have reported significantly unfavorable outcomes in ldlt recipients with a positive lymphocytotoxic crossmatch [ 6 , 7 ] .
in contrast , our previous results showed that if the titer is low ( no more than 32 ) , a positive lymphocytotoxic crossmatch does not adversely affect the graft or survival in patients without desensitization .
although the significance of a quantitative assessment of the lymphocytotoxic crossmatch has not been reported , the high titer in our present patient led to the need for perioperative desensitization to prevent early graft loss due to antibody - mediated rejection . after considering the results in the present patient ,
we have settled the indication criteria for preoperative desensitization therapy at the titer of 1,000 ( t lymphocyte crossmatch ) . in this patient ,
therefore , we applied preoperative desensitization using rituximab and plasmapheresis to reduce the high titer of preformed antibodies and b lymphocytes . as a result ,
the lymphocytotoxic crossmatch was negative after the 3rd plasmapheresis , and negativity was sustained thereafter .
preoperative desensitization using rituximab was introduced in abo - incompatible ldlt in 2003 and has dramatically improved the outcomes of abo - incompatible ldlt .
the appropriate dosage of rituximab is still controversial , but many previous studies have reported the administration of 375 mg / m of rituximab 13 weeks before the transplant . following these successful cases , we planned the administration of 375 mg / m ( 500 mg / body ) of rituximab 2 weeks before the operation [ 8 , 9 ] .
splenectomy is also considered to be effective to reduce antibody production , as the spleen is the site of antibody production .
after the operation , the suppression of t - cell function to prevent the initiation of t - cell - mediated antibody production was regarded as indispensable .
we have routinely used tacrolimus and steroid as an immunosuppressive regimen , and in this particular patient , we added basiliximab ( postoperative days [ pods ] 1 and 4 ) and mmf .
mild acute cellular rejection occurred about 3 weeks after the ldlt , but response to the steroid recycle therapy was prompt , and the lymphocytotoxic crossmatch was negative during this episode . in summary , we report a successful ldlt using a lymphocytotoxic crossmatch highly positive graft .
perioperative desensitization using plasmapheresis and rituximab may provide significant benefits for reducing anti - hla antibodies . | warthin 's tumor is the second most common type of salivary gland tumor .
microscopically , warthin 's tumor displays a proliferative epithelial component and lymphoid stroma .
carcinomas arising from the epithelial component are well known , but malignant transformations of the lymphoid stroma are rare . when they do occur , they are most commonly b - cell type non - hodgkin lymphomas . a 60-year - old male patient
underwent surgical resection of a parotid mass .
after superficial parotidectomy , microscopic examination indicated that the tumor was of epithelial components with basaloid and oncocytic columns of cells neighboring lymphoid components .
in addition to the lymphoid follicles with distinct germinal centers , there were large , bizarre and extremely atypical neoplastic cells seen in the lymphoid component .
large neoplastic cells were diffusely cd20 and cd30 positive . the patient was diagnosed with " warthin 's tumor and diffuse large b - cell lymphoma with expression of cd30 . "
the histopathologic and clinical features are discussed along with a review of the literature . |