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wyncoll and camporota 's correspondence provides a valuable tool allowing clinicians the ability to assess the number needed to treat ( nnt ) and cost - effectiveness of various ventilator - associated pneumonia ( vap ) prevention strategies . subglottic secretion drainage ( ssd ) technology is one vap - reduction strategy for which nnt can easily be calculated based on its established relative risk reduction . smulders and colleagues conducted a randomized clinical trial in 150 patients receiving mechanical ventilation and found that intermittent ssd reduced the risk of vap by 75% ( p = 0.014 ) . utilizing wyncoll and camporota 's table , and assuming a vap incidence of 4% , utilizing ssd in 33 patients will prevent one episode of vap . in the united states , the average hospital has approximately 131 patients per year who require mechanical ventilation > 96 hours ( unpublished observations ) . utilizing ssd in these average hospitals in addition , ssd is a cost - effective intervention . at the assumed vap rate of 4% , wyncoll and camporota determined an upper threshold of 300 ( $ 470 usd ) additional cost per 10 days of ventilation to be a cost - effective investment in technologies that reduce vap by up to 75% . wyncoll and camporota 's framework illustrates that use of ssd is both a clinically and cost - effective strategy to reduce vap . nnt : number needed to treat ; ssd : subglottic secretion drainage ; vap : ventilator - associated pneumonia . sk would like to thank mary erslon , rn , msn , mba ( covidien ) for writing assistance .

the number needed to treat can be calculated for ventilator - associated pneumonia reduction strategies such as subglottic secretion drainage technology based on previous work establishing its relative risk reduction . assuming an incidence of 4% , employing subglottic secretion drainage in 33 patients will prevent one case of ventilator - associated pneumonia , and thus potentially 4 cases annually in an average hospital in the united states . with a previously described limit of 300 ( $ 470 usd ) additional cost per 10 days of ventilation as a threshold of investment for technologies to reduce ventilator - associated pneumonia , subglottic secretion drainage technology is both clinically and cost effective .

it is not synthesized in human body and supplied exclusively in non - vegetarian diet . psychiatric disturbances include apathy , depression , dementia , delirium , delusions , hallucinations , irritability , incoherent speech , and catatonia are commonly reported in adults / elderly . till date , the psychiatric presentations of cobalamin deficiency are reported in four children . second , oral vitamin - b12 monotherapy showed dramatic improvement in psychosis , but relapsed a year later after stopping ; however , author did not mention serum vit - b12 level or other manifestations . third , inadequate dietary intake of vitamin b12 and folate presented as neuropsychiatric changes and megaloblastic anaemia . last , a case of psychotic disorder and thrombotic manifestations of vitamin b12 and folate deficiency . however , there is no report of vegetarian pediatric case presenting solely as psychosis due to dietary deficiency . authors report a first case of dietary deficiency of cobalamin in lacto - vegetarian adolescent presenting solely as schizoaffective disorder without hematological / neurological manifestations . a 13-year - old lacto - vegetarian class viii boy was presented from lower socioeconomic status with nil significant family and personal history . no past history of gastrointestinal pathology , surgeries , or use of alcohol or proton - pump blockers . patient admitted with 1 week history of mutism , rigidity , immobility , staring look , disturbed sleep , ideas of worthlessness and hopelessness , aimless wandering , guilt , and suicidal ideas . physical examination ( pe ) found no abnormality . on mental status examination ( mse ) , patient had partial mutism , manneristic posture , depressed mood , paranoid delusions , thought echo / broadcast , running commentary , and command hallucinations . complete blood count with peripheral blood smear , blood sugar , renal / liver function test , serum iron , total iron binding capacity , urinary iron binding capacity , transferring , and transferrin saturation were normal . he responded quickly with lorazepam-6 mg / day ( stopped third day ) , olanzapine-15 mg / day and sertraline-50 mg / day , and discharged in 5 days . subsequently patient was asymptomatic , but had functional disability for which aripiprazole 15 mg / day was added and followed up for the next 2 months . then patient relapsed despite good adherence with 1 week history of suspiciousness , hearing voices , over - talkativeness , over - cheerfulness , inflated self - esteem , decreased need for sleep , increased appetite , increased pleasurable activities , and disruptive socio - education . mse revealed inflated self - esteem , over - familiarity , spontaneous over - productive speech , increased psycho - motor activity , elated affect , flight of ideas , thought echo and broadcasting , delusions of persecution and reference , command and commentary second person hallucinations , impaired judgment , and absent insight . sertraline stopped and divalproate sodium-500 mg / day added along with olanzapine-15 mg / day and aripiprazole-15 mg / day . divalproate increased to 1 gm and lithium carbonate 600 mg and haloperidol-10 mg were added . two days later , patient was re - admitted for worsening of symptoms for evaluation of organicity . no abnormality in magnetic resonance imaging of brain , thyroid function test , and gastric mucosal biopsy by upper gastrointestinal endoscopy was reported . serum cobalamin level was 112 ng / ml ( normal range : 180 to 914 ng / ml ) while serum folate was normal . patient started on alternate days 1 ml intramuscular injection vitcofol ( vitamin b12 = 500 mcg , folate = 15 mg andniacinamide = 200 mg per ml ) for six doses , then 2 ml monthly . patient became asymptomatic soon after second dose of cobalamin and discharged in 1 week . during followups , normal serum cobalamin level was maintained and psychiatric medications tapered and stopped on the 52 day of vitcofol . atypical presentation of case consists of acute onset , fluctuating course of psychotic spectrum initially with catatonia , depressive symptoms , first rank symptoms , ( frs ) , and later as mania , frs , and relapsed soon on medications . patient reached quickly and maintained the premorbid level in the short - term follow - up with only cobalamin supplementation . dietary deficiency of cobalamin associated with psychosis is significant in developing country like india for two reasons . first , the high prevalence of dietary deficiency of cobalaminin indian children , and second , course and prognosis with supplementation is better . this emphasizes the importance of diet history and performing serum cobalamin level as a routine investigation in first episode , atypical presentation , and treatment resistant cases , especially in vegetarian . early diagnosis and treatment of cobalamin deficiency in neuropsychiatry is recommended for following reasons : first , better prognosis is expected if treated in window period to avoid irreversible neuronal injury . second , to avoid treatment resistance and last , finding that high vitamin b12 level is associated with better treatment outcomes . methylene - tetrahydrofolate reductase , an enzyme of cobalamin metabolism , is involved in metabolism of tetrahydrobiopterin and latter is required for synthesis of dopamine and serotonin . short - term followup is a limitation , but , rapid disappearance of florid psychopathology and maintaining pre - morbid level solely on cobalamin supplementation is vital . early diagnosis and treatment of cobalamin deficiency during narrow window period is an opportunity to revert pathophysiology . this highlights the importance of diet history and serum cobalamin level in atypical psychiatric presentations .

cobalamin is an important nutrient . it is not synthesized in human body and supplied only in nonvegetarian diet . its deficiency reported with range of psychiatric disorders . only four pediatric cases have been reported as psychiatric disorders . authors report a case of dietary deficiency of cobalamin presenting solely as schizoaffective disorder without hematological / neurological manifestations . early diagnosis and treatment of cobalamin deficiency is an opportunity to reverse pathophysiology . this case highlights the importance of diet history and serum cobalamin level in atypical psychiatric presentations .

it is a rare condition in children with only few cases been reported in literature . we report a case of bronchobiliary fistula which occurred as a complication of ruptured liver abscess and this complication has not been reported earlier . a 3-year - old girl presented with complaints of fever , cough and respiratory distress with expectoration of yellowish sputum since past 12 days . past history revealed that she had an episode of high - grade fever with yellowish discoloration of conjunctiva , pain abdomen and generalized swelling of body with altered sensorium 6 months back . for these complaints , she had then been evaluated and ct abdomen had shown ruptured abscess of right lobe of liver , for which she had underwent exploratory laparotomy , drainage of liver abscess and peritoneal wash . she had also undergone a second laparotomy and adhesiolysis , 40 days following the first surgery for intestinal obstruction . subsequently she remained asymptomatic for next 2 months and then started developing fever , persistent cough with yellowish expectoration and difficulty in breathing . at admission she was febrile with respiratory distress ( nasal flaring and subcostal / intercostal retractions were present ) and oxygen saturation in room air was 89% . chest examination revealed decreased air entry on right side and bilateral scattered crepitation ( r > l ) and conducted sounds . liver was palpable 1.5 cm below costal margin with span of 7.5 cm , and spleen was just palpable . investigations revealed hemoglobin of 10.4 gm / dl , total leukocyte count of 11,700/mm with 69% neutrophils , 30% lymphocytes , 1% eosinophils and platelet count of 4.1 10 per microliter . gastric aspirate for acid - fast bacilli ( afb ) was negative on two occasions . the sputum smears and cultures were negative for bacteria and afb . human immunodeficiency virus ( hiv ) serology was also negative . other immunodeficiency work - up including cd4/cd8 counts , nitroblue tetrazolium tests ( nbt ) test for chronic granulomatous disease and immunoglobulin profile were within normal limits . chest x - ray showed homogenous opacity of right lower zone with blunting of right costophrenic angle [ figure 1a ] . ultrasound abdomen was done which showed altered architecture of right lobe of liver with minimal right pleural effusion . ( a ) chest radiograph pa view shows right - sided pleural effusion and a large air - fluid level at right lower hemithorax ( block arrow ) ( b ) axial cect image of liver reveals reduced volume of the right lobe ; associated with diffuse hyperdense attenuation of the entire lobe . note absent opacification of the right branch of portal vein whereas left branch is well visualized ( arrow ) ( c ) coronal reformatted image of cect of chest and abdomen reveals extensive right lower lobe consolidation with areas of cavitation ( d ) mrcp shows a hyperintense track containing fluid could be traced ( block arrow ) ; extending from the liver ( right hepatic duct ) to the lower lobe bronchi , suggesting a broncho - biliary fistula bronchoscopy revealed yellowish frothy secretion coming out of right main bronchus . a possibility of bronchobiliary fistula was kept , and broncho - alveolar lavage ( bal ) fluid was sent for relevant investigations . bal fluid revealed bilirubin of 2.5 mg / dl with conjugated fraction of 2 mg / dl . cect chest and abdomen revealed trans - diaphragmatic bronchobiliary fistula with resolving abscess in right lobe of liver and consolidation of right lower lobe of lung [ figure 1b and c ] . magnetic resonance cholangiopancreatography ( mrcp ) revealed abnormal tract communicating between a tributary of right branch of the hepatic duct and a branch of right lower lobe bronchus [ figure 1d ] . hida scan revealed good hepatocyte function , bronchobiliary fistula with extension of tracer in to right bronchus , trachea and abnormal tracer accumulation in right lower lobe of lung with patent bilio - enteric pathway [ figure 2 ] . child underwent right lateral thoracotomy and right lower lobectomy with surgical excision of sinus tract under general anesthesia . on follow up after 3 months , she remained asymptomatic with no respiratory distress and was gaining weight . ( a ) hida scan reveals reflux of the tracer agent through an abnormal communication in right bronchus ( b ) abnormal tracer agent also accumulated in the right lower lobe of lung with patent bilio - enteric pathway we report a young girl who developed bronchobiliary fistula as a complication of ruptured liver abscess and was treated with surgery . bbf may be congenital ; however , in most cases , it is acquired and parasitic infection of liver ( hydatid cyst ) is the commonest cause . other causes of bbf include trauma , sub - diaphragmatic abscess , post - surgical states , and lithiasis in biliary trees , cholecystitis / pancreatitis , and liver / biliary tree tumor and radiofrequency ablation . although the majority of bbf cases have been reported in neonates , age of presentation may vary from infants to adults . bbf occurring as a complication of ruptured liver abscess in children has not been reported yet . sutherland et al . have described false bile ptyalism ( excessive salivation ) in patients with sickle cell disease and hemolytic crisis . however , if this condition can be excluded , the presence of bile in the sputum is pathognomonic of bronchobiliary fistula . our case presented with recurrent fever , chronic cough following treatment of ruptured liver abscess . she developed yellowish , bile - stained sputum ( bilioptysis ) suggesting formation of a track from liver to pleural cavity through diaphragm and lately developed bronchobiliary fistula . . a two - stage approach can be used : ( 1 ) external biliary drainage by percutaneous or surgical drainage of subphrenic abscess and/or direct percutaneous drainage of the intrahepatic biliary tract ; and ( 2 ) treatment of the underlying cause . a delay in surgery might result in further damage of the lung . in our patient the right lower lobe of lung was completely damaged and chronic track had formed from liver to pleura . we conclude that bronchobiliary fistula is rare in children ; a high index of suspicion is required for early diagnosis .

bronchobiliary fistula ( bbf ) is a rare condition , defined by the presence of abnormal communication between biliary tract and bronchial tree . we describe a 3-year - old child who developed bbf after rupture of liver abscess . she underwent exploratory laparotomy and peritoneal wash for ruptured liver abscess . seven months later she presented with fever and cough with yellow - colored expectoration ( bilioptysis ) . an abnormal communication between right branch of the hepatic duct and a branch of right main bronchus was identified . child underwent right lateral thoracotomy and right lower lobectomy with surgical excision of sinus tract . on follow - up child was asymptomatic and doing well .

partial gastrectomy , gastroenterostomy , pyloromyotomy , pyloroplasty and endoscopic dilatation have all been recommended with variable results . a 54-year - old white female is presented with the onset of symptoms of idiopathic hypertrophic pyloric stenosis one year prior to operation . idiopathic hypertrophic pyloric stenosis in adults can be treated with laparoscopic pyloroplasty , offering a minimally invasive alternative to open repair . adult idiopathic hypertrophic pyloric stenosis ( aihps ) was first described by jean cruveilhier in 1835 . it is a rare disease and presents in adult life as pyloric obstruction , without a history of vomiting in infancy or other gastrointestinal symptoms . optimal treatment should provide relief of obstruction , low recurrence and low operative morbidity , since aihps is a benign disease . pyloromyotomy , pyloroplasty , gastrojejunostomy , endoscopic dilatation and gastrectomy have all been recommended for aihps . the patient is a 54-year - old white female with a history of postprandial pain and vomiting for one year . the patient was placed in a modified lithotomy position with the surgeon at the foot of the operating table and an assistant at each side . a veress needle was inserted , and the abdomen was insufflated with co2 gas up to a pressure of 14 cm h2o . laparoscopic pyloroplasty requires five ports arranged similar to that used in nissen fundoplication : one 12-mm port for the laparoscope , three 5-mm working ports , and one additional working port for retraction of the liver and falciform ligament . two 5-mm ports were placed in the right upper and left upper quadrant at the anterior axillary line . the two remaining 5-mm ports were placed at the left and right midclavicular line , both at the level of the umbilicus . alternatively , the ports may be placed lower in the abdomen , or a sixth port may be used . the duodenum was mobilized with a modified kocher maneuver to avoid tension on the anastomotic line . a nasogastric tube was required for decompression of the stomach , and suction was available to avoid spillage of duodenal contents . the pyloric opening was then approximated in a transverse fashion with one layer of full thickness , 2 - 0 ethibond interrupted sutures ( weinberg pyloroplasty ) . the first approximating sutures were tied extra - corporeally to relieve tension , and the remaining were tied intracorporeally . radiographic study with gastrografin on the first postoperative day showed no leak of contrast and no evidence of obstruction . the nasogastric tube was removed , and the patient was placed on a clear liquid diet . on postoperative day three , a soft diet was introduced , and the patient was discharged home . thirteen months after surgery , the patient is tolerating a regular diet and is completely symptom free . the first type is the late stage of infantile hypertrophic pyloric stenosis , which is easily diagnosed from the history of symptoms during infancy . the second type is hypertrophic pyloric stenosis commencing in adult life but secondary to other disease in the upper gastrointestinal tract . this can be a hiatal hernia , duodenal ulcer , gastric ulcer , tumors or inflammatory diseases . this type is the most common and is recognized by a long - standing history of gastrointestinal complaints recently complicated by obstructive symptoms . primary hypertrophic pyloric stenosis presenting in adult life without any apparent cause is the third type . in this entity aihps is further divided into two groups . in one group , there are no complications from the stenosis . in the complicated variety , there is gastritis or peptic ulcer . the sequence of events can differentiate this type from the secondary type of hypertrophic pyloric stenosis . the length of a normal pyloric canal is no more than 1 cm . in aihps , a convex indentation at the base of the duodenal bulb , known as kirklin 's sign , is another indication of aihps . an eccentric or concentric narrowing of the pyloric region is the string sign . the twining 's sign is a barium filling defect , which can project to either or both sides of the pylorus . it is located 4 to 6 cm proximal to the base of the duodenal bulb . some or all these findings can distinguish aihps from other diseases . in contrast , there are reports that typical cases are rare , and the radiological picture is highly variable . in addition , patients with pyloric carcinoma have had x - rays similar to those seen in aihps . its appearance has been described as the cervix sign , or even as a donut . the pylorus fails to close completely even though the peristaltic waves appear to culminate in some degree of contraction in the pyloric area . the main advantage of endoscopy is that it can easily differentiate aihps from other diseases that cause gastric outlet obstruction . pyloroplasty is considered technically difficult if the pylorus is grossly thickened and is reserved for the debilitated patient . the benign nature of the disease prompted levine et al to suggest non - operative diagnosis and treatment . the authors have concluded that laparotomy is not required for the diagnosis and that gastroscopic findings are more important than radiographic findings . endoscopic balloon dilatation performed for benign pyloric stenosis from other causes resulted in a high recurrent obstruction rate . in two separate studies , the authors suggested that endoscopic dilatation may palliate symptoms but should be reserved only for patients with high operative risk . the first laparoscopic pyloromyotomy for infantile hypertrophic pyloric stenosis was performed in 1990 . since then , many series have shown that the laparoscopic approach is safe , efficient , offers improved cosmesis , earlier postoperative recovery and shorter hospitalization . the benign nature of the disease in adults mandates a surgical treatment that can be performed safely and effectively with low morbidity and mortality rate . the diagnosis can be performed more safely with endoscopy , and pyloroplasty has less morbidity than gastrectomy . it provides treatment while preserving the benefits of a minimally invasive surgical technique in the debilitated patient . laparoscopic pyloroplasty is safe , effective and offers decreased pain , hospital stay and postoperative disability . larger studies and longer follow - up are required for its establishment as the treatment of choice .

background and objectives : idiopathic hypertrophic pyloric stenosis , in adults , is a rare disease . partial gastrectomy , gastroenterostomy , pyloromyotomy , pyloroplasty and endoscopic dilatation have all been recommended with variable results . a 54-year - old white female is presented with the onset of symptoms of idiopathic hypertrophic pyloric stenosis one year prior to operation . two endoscopic pyloric sphincter balloon dilatations provided only temporary relief.method:a laparoscopic pyloroplasty was performed.result:the patient tolerated a solid diet on postoperative day three . the patient was symptom - free at a 13 month follow-up.conclusions:idiopathic hypertrophic pyloric stenosis in adults can be treated with laparoscopic pyloroplasty , offering a minimally invasive alternative to open repair .

graphene thin film was obtained by mechanically peeling of natural graphite onto silicon substrate with 300-nm thermal oxide . a 10-nm siox thin film was evaporated onto graphene using e - beam evaporator operated at 10 torr . this fresh oxide surface was functionalized with p(s - r - mma ) random copolymer ( polymer source , mn : 11000 , mw / mn : 1.15 , styrene 55 mol% , functionalized -hydroxyl and -tempo moiety ) by spin coating a thin film from 1 wt% toluene solution . this film was annealed at 170 c for 72 hours to anchor the polymer onto oxide surface through end hydroxyl group , and then rinsed with toluene to remove unanchored polymer . p(s - b - mma ) with molecular weight of 77000 g mol ( ps - pmma : 55000 - 22000 , mw / mn : 1.09 ) and 47700 g mol ( ps - pmma : 35500 - 12200 , mw / mn : 1.04 ) were purchased from polymer source and dissolved in toluene . the block copolymer thin film was prepared on the neutralized surface by spin coating 1 wt% filtered polymer solution at 2500 rpm followed by annealing at 180 c for 12 hours . the degraded pmma domains were removed by immersing in glacial acid for 20 min followed by extensive water rinsing . we used reactive ion etcher ( sts mesc multiplex advanced oxide etcher ) to etch down to graphene layer . firstly , an o2 plasma process ( 50 w , 4 mtorr ) was used to remove exposed random copolymer . controlled over etching here then , chf3 plasma ( 50 w , 6 mtorr ) was employed to punch holes into evaporated siox to expose underlying graphene layer . additional o2 plasma was used to completely etch away exposed region of graphene or to further undercut the graphene nanomesh . tem samples were prepared by spin coating 300 nm thick of pmma polymer resist onto the graphene mesh substrate and baked at 100 c . the pmma - graphene mesh film was then lifted off in hf solution and transferred onto the lacey film coated copper grid . after pmma was removed by acetone vapour , the sample was characterized by jeol 1200 operated at 80kv . tapping mode afm graphene thin film was obtained by mechanically peeling of natural graphite onto silicon substrate with 300-nm thermal oxide . a 10-nm siox thin film was evaporated onto graphene using e - beam evaporator operated at 10 torr . this fresh oxide surface was functionalized with p(s - r - mma ) random copolymer ( polymer source , mn : 11000 , mw / mn : 1.15 , styrene 55 mol% , functionalized -hydroxyl and -tempo moiety ) by spin coating a thin film from 1 wt% toluene solution . this film was annealed at 170 c for 72 hours to anchor the polymer onto oxide surface through end hydroxyl group , and then rinsed with toluene to remove unanchored polymer . p(s - b - mma ) with molecular weight of 77000 g mol ( ps - pmma : 55000 - 22000 , mw / mn : 1.09 ) and 47700 g mol ( ps - pmma : 35500 - 12200 , mw / mn : 1.04 ) were purchased from polymer source and dissolved in toluene . the block copolymer thin film was prepared on the neutralized surface by spin coating 1 wt% filtered polymer solution at 2500 rpm followed by annealing at 180 c for 12 hours . the degraded pmma domains were removed by immersing in glacial acid for 20 min followed by extensive water rinsing . we used reactive ion etcher ( sts mesc multiplex advanced oxide etcher ) to etch down to graphene layer . firstly , an o2 plasma process ( 50 w , 4 mtorr ) was used to remove exposed random copolymer . controlled over etching here then , chf3 plasma ( 50 w , 6 mtorr ) was employed to punch holes into evaporated siox to expose underlying graphene layer . additional o2 plasma was used to completely etch away exposed region of graphene or to further undercut the graphene nanomesh . tem samples were prepared by spin coating 300 nm thick of pmma polymer resist onto the graphene mesh substrate and baked at 100 c . the pmma - graphene mesh film was then lifted off in hf solution and transferred onto the lacey film coated copper grid . after pmma was removed by acetone vapour , the sample was characterized by jeol 1200 operated at 80kv . tapping mode afm

graphene has significant potential for application in electronics1 - 5 , but can not be used for effective field - effect transistors operating at room temperature because it is a semimetal with a zero bandgap6,7 . processing graphene sheets into nanoribbons with widths of less than 10 nm can open up a bandgap that is large enough for room temperature transistor operation8 - 19 , but nanoribbon devices often have low driving currents or transconductances18,19 . moreover , practical devices and circuits will require the production of dense arrays of ordered nanoribbons , which is of significant challenge20,21 . here we report the production of a new graphene nanostructure - which we call graphene nanomesh - that can open up a band gap in a large sheet of graphene to create a semiconducting thin film . the nanomeshes are prepared with block copolymer lithography and can have variable periodicities and neck widths down to 5 nm . graphene nanomesh field - effect transistors can support currents nearly 100 times greater than individual graphene nanoribbon devices , and the on - off ratio - which is comparable with the values achieved in individual nanoribbon devices - can be tuned by varying the neck width . the block copolymer lithography approach used to make the nanomesh devices is intrinsically scalable and could allow for the rational design and fabrication of graphene - based devices and circuits with standard semiconductor processing .

osteosarcoma may rarely originate from the axial bones such as pelvis or vertebrae [ 1 , 2 ] . only 1 - 3 % of all osteosarcomas involve the spine ; the lower lumbar and sacral vertebrae are the most common spinal locations . in some pelvic and most primary vertebral tumors , contraindications for resection are unusually large extraosseous extensions with sacral plexus or major vascular involvement . on rare occasions , vertebral and sacral resections have been attempted . in general , these tumors can not be resected with negative margins and are best treated by radiotherapy and chemotherapy . patients with primary tumors of the axial skeleton have a poor outcome because local control is rare . the prognosis for these patients may improve with a more aggressive surgical approach and more effective chemotherapy . patients whose tumors can be completely resected should be approached with curative intent ; radiotherapy may provide significant palliation in individuals with unresectable primary tumors and has been reported in a small series to be associated with an improved survival . within the cooperative osteosarcoma study ( coss ) , overall survival for patients with primarily spinal tumors was less than 2 years and the local failure rate was near 70% in 22 patients who were studied . the patient who is presented in this article is a case of sacral osteosarcoma which has been treated with chemotherapy and radiation therapy and his disease has been controlled during 48 months of follow up . a 14-years - old boy came with pain in lower lumbar region lasting for 5 months . physical examinations revealed that he suffered only mild to moderate tenderness on sacral region with a very mild bulging with firm consistency . muscular force in the proximal of lower extremities was normal while there was a moderate weakness in left foot muscles . plain radiography of the pelvis and lumbosacral region revealed a large sclerotic lesion with ill defined borders in the first sacral vertebral body , with extra skeletal extension . mri of this region revealed a large destructing bone lesion in sacrum ( figure 1 ) . complete blood counts and serum biochemistry showed normal profile except increased serum level of lactic dehydrogenase ( ldh=886 ) and alkaline phosphatase ( alp=1893 ) . chest radiography was normal and whole body bone scintigraphy revealed an increased uptake only in sacral region . open biopsy of the lesion was done and pathologic study performed . in microscopic evaluation sarcomatous neoplasm was seen with foci of tumoral ossification composed of branching woven bone trabeculas as well as foci of poorly differentiated hyper cellular regions and extensive areas of chondroblastic differentiation with small areas of mixoid tissues . grading of tumor was estimated 2 out of 4 and final pathologic diagnosis was chondroblastic osteosarcoma grade 2 ( figure 2 ) . according to the site of origin and large volume of the tumor it was surgically unresectable ; therefore the patient took chemotherapy plus radiotherapy . chemotherapy regimen consisted of 2 drugs of cisplatin and doxorubicin ; after 4 courses of this regimen pain subsided dramatically and patient received radiotherapy which was delivered with cobalt-60 teletherapy unit with dose of 6000 centigray in 30 fractions during 7 weeks to the gross tumor with a 3 to 5 cm margin in all directions . after completion of radiotherapy , chemotherapy continued for extra 3 courses of the same regimen . at the end of treatment and until the last follow up at 48 months later , the patient was completely asymptomatic with normal performance status and there was not any evidence of active loco regional tumor or distant metastasis . the last pelvic ct scan revealed that sclerotic lesion of the sacral vertebrae with its soft tissue extension remained without any progression . the present case is an example of successful non- surgical treatment for axial bone osteosarcoma . presented a case report of 14-years - old girl with third lumbar vertebra which was treated with total vertebrectomy , chemotherapy and radiotherapy . they concluded that therapeutic approach for the spinal tumor should use the developed techniques of treatment of extremity osteosarcoma because of their encouraging results . toshifumi ozaki et al . analyzed 22 patients with spinal osteosarcoma ( 15 with sacrum tumor and 7 with tumors in other sites ) who received chemotherapy . the median survival was 23 months , and 3 patients survived disease free for more than 6 years . in this study there was a significant difference in overall survival between patients who underwent either wide or marginal surgery and patients who underwent either intralesional surgery or no surgery . among the patients who underwent no surgery or intralesional surgery results tended to be better in those patients who received irradiation compared with patients who did not receive irradiation . evaluated local control of osteosarcoma in patients whom a resection with satisfactory margins was not achieved and evaluated the efficacy of radiotherapy in this setting . in this study 41 patients with osteosarcomas that either were not resected or were excised with close or positive margins had undergone radiotherapy with external beam photons and/or protons . patients with gross or subtotal resection had a greater rate of local control , survival , and disease - free survival compared with those who underwent biopsy only at 5 years . although no definitive dose - response relationship for local control of tumor was seen , the local control rate was better for those patients who received doses equal or higher than 55 grays vs. lower doses . they concluded that radiotherapy could improve local control of diseases in situations which achieving a safe surgical margin was not possible . in our patient who was treated with chemotherapy and radiotherapy , a reasonable local control and disease free survival was achieved . based on current data for osteosarcomas which are unresectable with safe margin , chemotherapy and radiotherapy should be considered . in some patients in our patient who was treated with chemotherapy and radiotherapy , a reasonable local control and disease free survival was achieved . based on current data for osteosarcomas which are unresectable with safe margin , chemotherapy and radiotherapy should be considered . in some patients

osteosarcoma may rarely originate from the axial bones such as pelvis or vertebrae . in some pelvic and most vertebral primary tumors , resection often is not possible completely . in general , these tumors can not be resected with negative margins so they need additional radiotherapy and chemotherapy , but results are unfavourable because of poor local control and high incidence of distant metastases . this is a case report of sacral osteosarcoma which was treated successfully with chemotherapy and radiation therapy . the patient is a 14-year - old boy with a large osteosarcoma tumor in the first sacral vertebral body , with extra skeletal extension . the patient took radiotherapy ( 6000 centigray ) plus chemotherapy regimen consisting of doxorubicin and cisplatin . in the last follow up 48 months later , the patient was completely asymptomatic with normal performance and there was not any evidence of local progression or distant metastasis .

this disorder has been described to occur commonly in obese , post - menopausal women and is associated with weakness and mental disturbances such as depression , confusion , lethargy , and dementia . however , it begins in most cases prior to the post - menopausal period and can also occur in men [ 2 , 3 ] . the cause is unknown , and there is no specific treatment [ 1 , 2 ] . this case is reportable because adiposis dolorosa is thought to occur primarily on the body and not the head [ 4 , 5 ] , but our patient had involvement of scalp with severe headache . a 46-year - old woman noticed painful thickening of the scalp in bilateral parieto - occipital areas and vertex while combing hair for 1 year . six weeks prior to the presentation to our service , she developed intermittent severe shooting pain in the occipital region of scalp which used to get aggravated on lying supine or pressure on occiput , which compelled the patient to sleep in lateral or prone position . the frequency and severity of pain increased to an extent to disturb sleep and daily activities . patient had multiple small , subcutaneous fatty lumps all over the body which started painlessly in adolescence ; few of them had slightly enlarged with tenderness on palpation , whereas few subcutaneous lumps had spontaneous pain of mild intensity without affecting sleep or daily activities . computerized tomography ( ct ) and magnetic resonance imaging ( mri ) scan of head revealed diffuse thickening of scalp tissue in vertex and bilateral parieto - occipital areas suggestive of diffuse lipomatosis of scalp with no evidence of intracranial / spinal abnormality of cervical region ( fig . the patient was put on tricyclic antidepressants and multiple analgesics by referring physician , which produced no relief . she was referred to our department when she developed acid peptic disorder due to pain killers . the patient was subjected to psychiatry and neurology evaluation to rule out other causes of headache . two ml of 0.5% bupivacaine was injected to each site , which were approximately 2 cm lateral and 1 cm inferior to the external occipital protuberance , just medial to the occipital artery . the pain resolved completely , within 10 min after the block , but the patient had recurrence of headache after few hours . therefore , we advised her bilateral surgical greater occipital neurectomy , but she did not give consent . the procedure was repeated at same sites with injection of 1 ml of 0.5% bupivacaine followed by 1 ml of 95% ethyl alcohol after 10 min to each site , because injection of alcohol alone might be very painful . massage was not done to prevent fat necrosis due to spread of alcohol along the rest of the scalp . surprisingly this has produced lasting relief of pain . at 9 months of follow - up , the patient developed hypoesthesia of occipital region which she readily accepts at cost of pain relief . the patient has also developed tiny patches of alopecia at injection sites which is easily hidden in her hair.fig . 1a coronal image of ct scan of brain suggestive of abnormal increased thickening of scalp . c sagital image of mri scan of brain suggestive of lipomatous thickening of scalp a coronal image of ct scan of brain suggestive of abnormal increased thickening of scalp . b coronal image of mri scan of brain suggestive of lipomatous thickening of scalp . adiposis dolorosa is a disease of unusual distribution of fatty tumors in which the patient is easily dismissed as a malingerer , as pain in these tumors seems out of proportion to the physical findings . pain may be relieved by steroids , intravenous lidocaine , or analgesics [ 1 , 2 ] . surgical treatment consists of excision or liposuction of the painful masses in drug refractory cases . diffuse dercum s disease of scalp is difficult to treat as other causes of headache and psychological factors have to be ruled out . these lesions can not be treated by liposuction or excision of mass unlike other body parts . we believe that severe headache in our patient may be caused by pressure over the greater occipital nerves due to abnormally increased fat content in scalp . the greater occipital nerve ( gon ) block has been described to be effective in treatment of cluster headache , cervicogenic headache occipital neuralgia and post lumbar puncture headache [ 710 ] . this is a simple , outpatient procedure and can be repeated depending upon the recurrence of pain . we did chemical neurectomy in our patient as intractable pain dramatically responded to bilateral gon blocks and the patient did not give consent for surgical neurectomy . chemical neurolysis has been used in the treatment of severe or intractable pain associated with cancer , trigeminal or post herpetic neuralgia and morton s neuroma [ 1114 ] ; however , its use in adiposis dolorosa has not been reported . we believe that it may be considered by a pain clinician when the drug refractory intractable pain is confined along a specific superficial sensory nerve territory . patient should be explained about the potential risk of permanent hypoaesthesia and local tissue necrosis . chemical neurolysis of nerve in the territory of headache may be considered a treatment modality for lasting relief .

a 46-year - old female , known case of adiposis dolorosa since adolescence , noticed painful thickening of scalp in bilateral parieto - occipital areas and vertex 1 year back . six weeks prior to the presentation to our service , she developed severe occipital headache refractory to drug treatment . she improved after bilateral greater occipital nerve blocks . she was subjected to bilateral greater occipital chemical neurolysis which has given her complete pain relief .

starting in june 2005 , several players of a dutch soccer team , consisting of 35 members , noted soft - tissue infections . in october 2005 , the municipal health service received a report of a patient , a member of this soccer team , who had been hospitalized for an abscess resulting from mrsa . other members of the team had skin infections as well , and screening was started . a case was defined as a patient who had a culture - confirmed mrsa infection during the outbreak period october 2005 through january 2006 . healthcare staff obtained specimens by swabbing the patients ' nose , throat , or wound . a total of 56 persons were screened : 42 members of the soccer club and 14 of their roommates . the 42 members consisted of soccer players , coaches , and people who used the same training facilities , locker room , and showers . the roommates screened were all those who lived with an mrsa - positive player and those who lived with an mrsa - negative player but had skin infections . of the 56 persons screened , we identified an mrsa infection in 11 persons : 9 soccer players and 2 roommates ( table ) . most infections lasted for several weeks . for all players who had soft tissue infections , mrsa was diagnosed . among those in whom one roommate had an abscess in the armpit , but the soccer player she lived with was mrsa negative . * mrsa , methicillin - resistant staphylococcus aureus ; gi , gastrointestinal ; na , not available ; nt , not tested ; ns , not specified . this player was the index case - patient and was hospitalized . to prevent further mrsa transmission , on october 28 the soccer club was instructed to increase hand hygiene , not share personal items , use liquid soap and disposable towels , put a towel on the bench before sitting , increase frequency of cleaning the facilities , and provide more ventilation to the locker room and showers . all patients were treated with cotrimoxazole for 1 week or , if needed , longer until wound infections were healed . perineum cultures from 3 patients showed carriage of mrsa in the gastrointestinal tract , and rifampicin was added to cotrimoxazole for 1 week or , if needed , longer until wounds were healed . furthermore , patients used chlorohexidine , gluconate scrub , povidone iodine , and mupirocin ointment for 5 days . a patient was declared mrsa free after 3 cultures , taken at 1-week intervals , were mrsa negative . one patient , who also had eczema , remained mrsa positive . in november 2005 , mrsa was isolated from a 33-year - old soccer player with a boil , who was a member of a neighboring team . this soccer player had competed against the team with the mrsa - positive players on october 8 , before mrsa - positive results were known and hygiene measures recommended . immediately , this player 's team was screened for mrsa ; all other players were mrsa negative . the mrsa isolate from this player was included in the analysis . to characterize the mrsa strains , the following typing methods were used : pulsed - field gel electrophoresis ( pfge ) , staphylococcal protein a ( spa ) typing , multilocus sequence typing , sccmec typing , and pcr of pvl genes ( luks - lukf ) . all mrsa isolates were identical and identified as the european ca - mrsa st80-mrsa - iv strain . all strains were pfge type 28 ( according to the dutch pfge classification system ) , spa type t044 , st80 , sccmec iv , and pvl positive . all mrsa isolates had identical susceptibility patterns ; they were resistant to oxacillin ( and thus to all -lactam antimicrobial drugs ) , tetracycline , and fusidic acid . they were susceptible to rifampicin , ciprofloxacin , gentamicin , erythromycin , clindamycin , vancomycin , teicoplanin , and cotrimoxazole . this study shows transmission of the ca - mrsa st80-iv strain among members of a dutch soccer team . transmission apparently occurred not only between members of this team but also to a competing soccer team . transmission of the usa300 strain between members of different teams was previously described for football teams ( 8) . we can not rule out the possibility of an independent colonization of the st80-mrsa - iv strain in the competing team , but the 2 teams competed against each other during the period when the first symptoms were noted by members of the first team . because soccer involves much less contact than football , rugby , or wrestling , mrsa transmission may not necessarily have been caused by skin - to - skin contact but this possibility has also been suggested in a report about a ca - mrsa outbreak among sports participants ( 12 ) . to our knowledge , this is the first report of an outbreak of the ca - mrsa st80-iv strain in a sports team . as with the usa300 strain , more outbreaks of ca - mrsa st80-iv are likely . therefore , to identify and control an outbreak as early as possible , sports physicians and coaching staff should be alerted and informed about ca - mrsa .

an outbreak of community - acquired methicillin - resistant staphylococcus aureus occurred among members and close contacts of a soccer team . typing of the isolates showed the outbreak was caused by the well - known european st80-iv strain . to our knowledge , this is the first report of an outbreak of this strain among members of a sports team .

approximately 2,000 horses are stabled at the facility in which this outbreak occurred . in september and october 2014 , the numbers of pyretic horses in september and october during 20092013 were 16.0 4.4 and 17.6 1.7 ( mean number sd ) , respectively . edta - treated blood samples and nasal swab samples from 49 and 48 pyretic horses , respectively , were collected during september 25november 2 . paired serum samples were collected from 19 febrile horses during the acute ( september 15october 12 ) and convalescent ( 24 weeks later ) phases . viral rna and dna were extracted from the blood samples and nasal swab samples by using a nucleic acid isolation kit ( magna pure lc total nucleic acid isolation kit , roche diagnostics , mannheim , germany ) . reverse transcription pcr ( rt - pcr ) was conducted using a primer pair targeting nonstructural protein 1 ( nsp1 ) of getah virus ( onestep rt - pcr kit , qiagen , hilden , germany ) by using the rna extracted from the blood samples ( 8) . pcr was also used to detect the specific genes of equid herpesviruses 1 and 4 within the blood samples ( 9 ) . reverse transcription loop - mediated isothermal amplification was used to detect equine influenza virus in the nasal swab samples ( 7 ) . equine arteritis virus was tested by real - time rt - pcr by using the blood samples ( 10 ) . a virus neutralization test for getah virus was conducted on vero cells using the mi-110 strain , which was isolated in 1978 ( 11 ) and is the current vaccine strain , as described previously ( 12 ) with slight modification . the neutralizing antibody titers were determined as the reciprocal of the highest serum dilution that inhibited viral cytopathic effects . seroconversion was defined as > 4-fold increase in the antibody titer between paired serum samples . serum collected from horses vaccinated after august 1 were not used in this study because the neutralization test can not distinguish an increase in antibodies induced by natural infection from that induced by vaccination . the nsp1 and capsid genes in the rt - pcr positive samples ( strain designation miho-2014 ) and mi-110 strain were amplified by using previously described primer pairs ( 8) and were sequenced commercially ( fasmac co. , ltd . sequences were analyzed with the vector nti advance 11 software ( invitrogen , carlsbad , ca , usa ) . phylogenetic analyses of the nucleic acid sequences were conducted with mega 5.2 software ( 13 ) . phylogenetic trees based on the nsp1 and capsid genes were constructed by using the neighbor - joining method . the statistical analysis of the trees was conducted with the bootstrap test ( 1,000 replicates ) . the accession numbers registered in genbank / embl / ddbj are as follows : the partial sequences of the nsp1 gene miho-2014 ( lc012885 ) and mi-110 ( lc012887 ) ; and the partial sequences of the capsid gene miho-2014 ( lc012884 ) and mi-110 ( lc012886 ) . of the 49 blood samples tested among horses 27 years of age , 25 were positive for getah virus by rt - pcr ( table ) . all blood samples were negative for equid herpesviruses 1 and 4 and equine arteritis virus , and all nasal swab samples were negative for equine influenza virus . in the neutralization test , 16 of 19 paired serum samples showed seroconversion ( > 4-fold increase ) to the mi-110 strain of getah virus ( table ) . in total , 33 febrile horses were positive for getah virus infection by rt - pcr , neutralization test , or both . seventeen horses had edema in their legs , and 4 had rashes on their bodies ; these horses constituted a small percentage of febrile horses . all the horses that were positive for getah virus infection recovered after treatment of signs . the first and last samples that were positive by neutralization test , rt - pcr , or both were collected from pyretic horses on september 15 and october 25 , respectively . these results show that the getah virus infection occurred among racehorses at the miho training center from mid - september through late october 2014 . we analyzed the sequences of the nsp1 and capsid genes of 10 positive samples and the mi-110 strain . the nucleic acid sequences of the nsp1 ( 381 bp ) and capsid ( 552 bp ) genes were completely identical among the 10 getah viruses detected in 2014 . the nucleic acid sequence identities between the getah virus detected in 2014 and mi-110 were 98.7% for the nsp1 gene and 99.1% for the capsid gene . phylogenetic analyses were performed with the nucleic acid sequences of the getah virus nsp1 and capsid genes , including those of isolates from horses , mosquitoes , and pigs ( figure ) , and showed that the getah viruses detected in 2014 clustered apart from the mi-110 strain . phylogenetic analyses of the nucleotide sequences of the ( a ) nonstructural protein 1 ( nsp1 ) gene ( nt 218598 ) and ( b ) capsid gene ( nt 76458196 ) of getah virus isolated in japan , 2014 . the genome positions of the nsp1 and capsid genes correspond to those of kochi/01/2005 strain ( genbank accession no . ab859822 ) ( 14 ) . closed and open circles represent miho2014 , the strain isolated in this study , and mi-110 , the strain isolated in 1978 , respectively . the percentage bootstrap support is indicated by the value at each node ; values < 70 are omitted . november 1978 , and another outbreak in 2014 occurred around the same period at the same facility . in 1978 , of 1,903 stabled horses , 722 were affected ( 11 ) . in 1979 and 1983 , several small outbreaks of getah virus infection occurred among unvaccinated horses at several facilities other than the miho training center . we have no data on the vector mosquitoes or the climatic conditions in this region . among the affected horses , eight 2-year - old horses had received the initial vaccination just 14 days before disease onset . clearly , the vaccine against getah virus could not provide some of the affected horses with sufficient protective immunity . the phylogenetic analyses showed that the currently circulating viruses differ genetically from the mi-110 vaccine strain isolated in 1978 . from these results , we infer that this outbreak might be partly attributable to the antigenic differences between the vaccine strain and the currently circulating strain .

an outbreak of getah virus infection occurred among racehorses in japan during september and october 2014 . of 49 febrile horses tested by reverse transcription pcr , 25 were positive for getah virus . viruses detected in 2014 were phylogenetically different from the virus isolated in japan in 1978 .

case 1 a 27-year - old male patient consulted to us with abdominal pain lasting nearly six hours . his medical history was unremarkable . on physical examination localized tenderness , and guarding were palpated on the left lower quadrant . his laboratory findings were nonspecific ( wbc 11.800/mm , hemoglobin 16 gr / dl , crp 0.6 mg / dl ) . on us , a hyperechoic omental fatty tissue in the lower left quadrant measuring 43x17 mm was observed . on ct , an increase in density on the rim of the mesentery , and in the vicinity of sigmoid colon which suggested the presence of epiploic appendicitis ( figure 1 ) . he was discharged , and in the fifth month of his follow - up period any disease recurrence was not detected . case 2 a 72-year - old female patient presented to the outpatient clinic with abdominal pain lasting for 3 days . his laboratory findings were nonspecific ( wbc 10.500/mm , hemoglobin 13 gr / dl , crp 1.2 mg / dl ) . on ct , pericolon fatty tissue was inflamed , edematous , and increased density on mesentery was seen which was in compliance with epiploic appendicitis ( figure 2 ) . epiploic appendices are oval - shaped 12 cm thick small peritoneal processes measuring 0.55 cm in length which are found as arrays of two rows along taenia coli , and attached with a vascular pedicle to the serous coat of the large intestine . although they are more diffuse in sigmoid colon ( 57% ) , and ileocecal region ( 26% ) only 50100 epiploic appendages are seen in the whole colon [ 3 , 4 ] . they are perfused by one or two small terminal branches of colonic vasa recta , and drained through a single vein . their role in immunity as is seen with omentum , and their involvement in colonic absorption have been already indicated . in our cases , inflammation was localized in the sigmoid colon in compliance with the literature findings . epiploic appendicitis is primary or secondary inflammation of epiploic appendices . in intraabdominal inflammatory events as diverticulitis , appendicitis , and cholecystitis primary epiploic appendicitis ( pea ) is ischemic or hemorrhagic infarct , and inflammation as a result of appendiceal torsion or spontaneous venous thrombosis . right - sided pea can be confused with acute appendicitis , right collonic diverticulas , however left - sided pea is frequently mistaken for sigmoid colon diverticulitis . it is important to consider these disease groups with different treatment protocols in the differential diagnosis of epiploic appendicitis . primary epiploic appendicitis can be seen between 12 , and 82 years of age which peaks in the 4.5 . rarely nausea , vomiting , loss of appetite , and other gastrointestinal symptoms rarely accompany this clinical entity . palpable masses have been reported in 1030% of the cases . in our first case , sharply localized lower left quadrant pain was found , while in our second case tenderness on upper , and lower left quadrant was detected . operator - dependency , and suboptimal results obtained in obese patients are disadvantages of us . on us they are seen as well - circumscribed , non - compressed , heterogenous ovoid or round masses which contain peripheral hypoechoic rim , and they are localized in the close vicinity of the large intestine . on colour - doppler , us blood flow can not be detected . as firstly reported by danielson et al . in the year 1986 it encounters us as round or oval masses on the anterolateral aspect of the colon with a slightly higher density when compared with peritoneal fat . a hyperdense peripheral rim is present around the lesion . in the center linear , and punctuate hyperdensity which represents thrombosed vein diverticulitis , omental infarct , mesenteric panniculitis , primary omental tumour , and metastases which contaminate fatty tissue are considered in the differential diagnosis . acute diverticulitis is frequently seen in elder patients , and often consolidation of the wall of the long segment was found . signs of diverticulitis including abscess , fistula , obstruction , and perforation are not usually seen in pea . findings of ct , include focal , subtle , soft tissue infiltration of the omentum or solitary , great , non - contrasted heterogenous , and hyperdense fatty mass localized on the greater omentum . hyperattenuated ring , and central spot seen in pea are not observed in omental infarct . omental cake is seen as a thick soft tissue mass adjacent to the ventral aspect of the transverse colon . ct is a noninvasive tool which enables us to make a differential diagnosis among all these diseases . during our controls , though regressed , we have seen persistence of ct signs . since its first description of pea in the year 1968 , it is treated conservatively , and with analgesic , and antibiotic use , the patient is discharged within less than 10 days . in suspect cases , laparoscopy is predominant in that it enables us to make a diagnosis , and apply treatment . in the past , the standard treatment of pea was excision because it could be diagnosed only intraoperatively . it can be confused with acute appendicitis , acute diverticulitis , ovarian torsion , enteritis , typhlitis , mesenteric lymphadenitis , and colorectal cancer . it can cause prolonged hospital stay , unnecessary antibiotic use , and surgical intervention . in conclusion , in cases with suspect lower abdominal quadrant pains , ct is effective in making a diagnosis of pea with increased sensitivity , and specificity . when we detect a benign disease of pea , we can prevent unnecessary invasive procedures .

epiploic appendices , first described in 1543 by vesalius , are fatty structures which are attached through the length of the colon and consisted of visceral peritoneum . epiploic appendicitis is an uncommon and self - limiting disease . in this report , we aimed to present two patients with epiploic appendicitis .

most cases were reported around the turn of the century . a literature review conducted in 1998 noted 256 reported cases of ingested foreign bodies within the appendix occurring over the last 100 years . since that review , only 2 cases have been reported . lead shot or pellets retained in the appendix after ingestion account for approximately a third of all reported cases . a 45-year - old male general medical practitioner presented to a rheumatologist with a few months ' history of back pain and paresthesia affecting both hands . as part of his investigations , radiographs were taken of his spine including his pelvis . the films confirmed a diagnosis of cervical nerve root irritation due to cervical spondylosis . however , as an incidental finding , opacities were seen above the right sacro - iliac joint ( figure 1 ) . he was referred for a surgical opinion because the appearance suggested the opacities might be lead shot in the appendix . a further history from the patient revealed that he had eaten pigeon as a child on numerous occasions . the patient was eager to have his appendix removed laparoscopically despite normal blood lead levels . lead shot in the appendix is a rare phenomenon . a potential risk exists of lead intoxication and acute appendicitis developing . laparoscopic appendectomy has been shown to be a reliable and safe method.5 it is therefore suggested that patients who present with lead shot in their appendix should be given the option of an elective appendectomy .

we describe a patient presenting with lead shot in his appendix . a plain radiograph of his lumbar spine was performed for back pain , and an incidental finding of lead shot retained within the appendix was seen . lead shot in the appendix is associated with appendicitis , and 2 cases have been reported of lead intoxication . we suggest that an elective laparoscopic appendectomy should be offered to patients as a possible management option .

we divided the analysis into 3 periods : period a ( before the outbreak ) was from january 1 , 2003 , through april 30 , 2011 ; period b ( early phase of the outbreak ) was from may 1 ( when the first outbreak - associated case - patient fell ill ) through may 18 ( date when the hus cluster was detected ) ; and period c ( late phase of the outbreak ) was from may 19 through june 22 . data on timeline events for all reported stec and hus cases were collected from the gssnd ( 7 ) . timeline events comprised the following dates : symptom onset ( onset of diarrhea ) , diagnosis , notification ( date when the notification arrived at the local health department ) , and reporting ( date when the report arrived at rki ) . dates of symptom onset and diagnosis were excluded when they were after the date of notification . for each case , intervals between timeline events were calculated from the dates available . intervals were then assigned to 1 of the 3 periods ( a , b , or c ) according to the first date of the interval . median times and interquartile ranges ( iqr ) were calculated in days for each type of interval for each period . statistical analyses were done by using stata software version 11.0 ( statacorp lp , college station , tx , usa ) . for the 1,394 hus cases with available information , the median times from symptom onset in patients to diagnosis and to notification were similar in periods a and b ( 8 days and 910 days , respectively ) and shorter in period c ( 4 and 5 days , respectively ) ( table 1 ) . the median time from symptom onset to reporting decreased from 20 days in period a to 12 and 8 days in periods b and c , respectively . the median time from diagnosis to notification was longer in period b ( 4.5 days ) than in periods a and c ( 1 and 0 day , respectively ) . among the 14 hus cases with available the interval from notification of the local health department to report to rki was longer in periods a and b than in period c ( 7 and 8 days vs. 3 days , respectively ) . for the 13,400 stec cases with available information , we noticed in period b a longer delay from symptom onset in patients to reporting ( 15 days vs. 12 days for hus ) but a shorter delay from diagnosis to notification ( 2 days vs. 4.5 days for hus ) ( table 2 ) . the figure shows the increasing numbers of patients with disease onset on may 9 , diagnosis and notification on may 18 , and reporting on may 24 . * period a , 2003 jan 12011 apr 30 ; period b , 2011 may 12011 may 18 ; period c , 2011 may 192011 jun 22 ; med , median ; iqr , interquartile range ; lhd , local health department ; rki , robert koch institute . classification of the interval in 1 of the 3 periods according to the first date of this interval . hemolytic uremic syndrome cases . * period a , 2003 jan 12011 apr 30 ; period b , 2011 may 12011 may 18 ; period c , 2011 may 192011 jun 22 ; med , median ; iqr , interquartile range ; lhd , local health department ; rki , robert koch institute . classification of the interval in 1 of the 3 periods according to the first date of this interval . patients having available data for the first date of the interval . no . patients having available data for this date / total number of shiga toxin producing escherichia coli cases . hemolytic uremic syndrome ( hus ) cases by date of symptom onset ( a ) , date of diagnosis ( b ) , date of notification ( c ) ( i.e. , the date that the local health department was notified of the case ) , and date of reporting ( d ) ( i.e. , the date that the robert koch institute received the report of the case from the local health department ) during outbreak of shiga toxin producing escherichia coli infection and hus , may vertical lines indicate may 19 , when the robert koch institute received reports about a cluster of hus cases in children . dark gray bars represent outbreak - related cases ; white bars represent cases not related to the outbreak . only cases with available information a median of 11 days passed between onset of symptoms and notification of stec cases in period a. a study by hedberg et al . in 6 us states reported a delay of 7 days for the same interval for e. coli o157 infections ( 8) . considering that period b is biased for the interval onset of symptom to reporting , because the second date of the interval is likely to be in period c when the reporting flow was accelerated we found that a median of 20 days occurred between symptom onset and reporting for stec and hus cases . this result is comparable to the 18 days reported for foodborne infections in the netherlands ( 9 ) . however , the duration between symptom onset and reporting can be reduced to 8 days , as was seen in period c. we also found that most of the hus cases in period b were notified later than mandated by law . although the number of cases was small , this is a remarkable result . it might be explained by the limited experience of nephrologists in notifying adult hus cases . however , this also shows the need to motivate and to assist clinicians to notify within 24 hours ( e.g. , with an automatic electronic notification tool that could alert clinicians of their obligation to notify the disease when entering the diagnosis of hus ) . by looking only at the timeline events directly under control of public health authorities , we found that the interval from notification of the local health department to reporting to rki could be shortened from 1 week to 3 days if the local health department and the state health department routinely transmitted data on a daily basis . this outbreak is a good example of circumstances in which single cases occur initially in multiple local health administrations in different federal states . in such situations , early outbreak detection and investigation become crucial to ensure early and continuous reporting to authorities at the national level . given the current delays in diagnosis , notification , and reporting , this outbreak would have been detected at the national level considerably later than may 19 if the hamburg health department had not promptly contacted rki . this illustrates that state health departments and rki need to receive local notifications earlier to successfully apply detection algorithms that would indicate potential multicounty or multistate outbreaks ( 10 ) . a revision of the notification and reporting system should be considered in germany , with the goal of timely detection of increases in infectious diseases while being sustainable and specific . this result could be achieved if physicians and heads of laboratories could feed their data into a centralized database shared by local health departments , state health departments , and rki with different access rights .

in the context of a large outbreak of shiga toxin producing escherichia coli o104:h4 in germany , we quantified the timeliness of the german surveillance system for hemolytic uremic syndrome and shiga toxin producing e. coli notifiable diseases during 20032011 . although reporting occurred faster than required by law , potential for improvement exists at all levels of the information chain .

during his historic trip to west berlin in the early 1960s , john fitzgerald kennedy stated that the plight of the citizens of west berlin was the plight of the world and , as such , we had all become berliners . in that vein , we also became washingtonians , vir - ginians , pennsylvanians , bostonians , san franciscans , and los angelenos . i saw the events of 11 september 2001 unfold . perhaps one day , a better wordsmith than i will coin the superlative that will capture the collective numbness that overcame us all . for me , amidst the relative comfort afforded by the edgy passage of time that facilitates a more thoughtful retrospect , one thought that seems to have been particularly predominant on that day was one of helplessness . like so many other physicians and other health care professionals , i was paralyzed knowing that i was unable to give help and comfort in the ways in which i was trained . that sentiment was driven home during the evening of 11 september 2001 when the hospital at which i work ( a tertiary care institution in an outlying borough ) , after gearing up for the carnage and after receiving a few victims with ' lesser ' injuries , downgraded its disaster status to a lesser degree of readiness . this occurred because most of the resources and efforts would be redirected towards morgue and clean - up duty . it seems almost clich to claim that the day began innocently enough , but for me that sentiment is true . it was the first day of the class i teach at medical school and , as such , i was afforded the restful luxury of sleeping in , and even drove my son to his school . from a weather standpoint , it was about as comfortable and clear as a september day in new york can be . in fact , it was the type of day that afforded an unimpeded view of the entire manhattan skyline from a few select vantage points . i noticed smoke seemed to be coming from one of the towers of the world trade center . my first thought suggested this was merely a power plant smoke stack that i had not previously noticed . a few minutes later , i nervously crossed the throg 's neck bridge from long island to the bronx , a venue that affords an even clearer and more expansive view of the entire skyline . i looked to my left and saw the plume of smoke and then , as ' rubberneckers ' slowed traffic , gawking in disbelief , i witnessed and heard the fireball erupt from the second tower as the second hijacked plane struck . i doubt that this vision will ever leave me . shortly thereafter , arriving at the medical school , others and myself watched from a high - rise departmental office as the towers successively collapsed . since our view was several miles north , it took a few minutes to confirm what we had all suspected and the minutes in between were spent in ashen horror asking one another whether any semblance of a structure still stood behind the smoke . the next few hours melted into an emotional blur , fueled by the local and national news descriptions , along with the personal uncertainties of locations of loved ones , families , and barely comprehending the carnage before our eyes . for me , once the safety of my wife and son were confirmed , i knew that i had to get to my hospital and pitch in if needed . when word was received that some bridges and roadways had reopened several hours later , i began the drive to the hospital , making sure that i had a full tank of gas and a bottle or two of water in view of the probable traffic , detours , and closings . the usual 45 min drive took longer than 3 hours because of all of the diversions . this was expedited only by producing my physician 's identification badge , allowing me to proceed on roads that were otherwise closed . this route had a clear view of the manhattan skyline , a ghastly caricature of its former glory . the air also bore an acrid flavor as smoke covered much of the sky like an eerie cloud . a few facts about the events and the overall state of mind among health care workers throughout the city merit mention . we all tried to offer assistance wherever and whenever we could , not only in our own institutions but also at hospitals and centers close to the carnage we knew we would eventually go home to find out about the death of friends , neighbors , acquaintances , colleagues , and perhaps even family members who were likely to have been in or near the world trade center . more than 1 month since the attack , people still talk about funerals and memorials that they have attended , cars that remained parked at train stations for several days , and offices destroyed and people displaced . thankfully , we also hear stories about people who did not go to work on 11 september because they did not feel well , they overslept , or they otherwise had reason to rightfully claim providence . general conversation is now punctuated by a greater sense of sincerity that goes beyond the usual perfunctory greetings . when we ask someone ' how are you ? ' we truly want to know ' how they are ' . like many parents in town , i took my son to school the next day and walked him to class , not only to find out how his teachers would approach the events , but also to see if parents , friends , or others in his school had become victims . the case mix in my hospital 's emergency room returned to ' usual ' on 12 september 2001 . my colleagues and i passionately wished that this was not so because if more had survived , our medical skills would have been needed . it is over 1 month since the world trade center towers were destroyed , and life in new york and elsewhere has returned to some semblance of normalcy . we remain cognizant , however , that the term ' normalcy ' now has a new definition , although it rests on a new platform and possesses an altered frame of reference . from my standpoint , it is a periphery that stands all too close to the site of the carnage and terror . from a medical perspective , i realize we must now begin to focus on practical aspects related to the events and medical preparedness , including the logistics of emergency response and communication , uninjured bystander first aid response , application of trauma algorithms in the field , the realities of disaster triage , and delayed traumatic injury treatment . the need has further hit home to focus on the emerging yet suddenly real potential threats of bioterrorism , chemical attacks , and other weapons of mass destruction . i know that these are things we must now consider , but the smoke has not fully cleared from my memory of 11 september 2001 as i look towards the southern manhattan skyline that now stands forever changed , and us with it . dc is an attending intensivist and the director of the division of research at the department of emergency medicine at maimonides medical center in brooklyn , new york , usa . he is also appointed as associate clinical professor of epidemiology and social medicine at albert einstein college of medicine , bronx , new york . this article , and the series it is part of , is dedicated to the first responders fire , police and medical personnel who attended the world trade center disaster of 11 september 2001 . they did not hesitate to place themselves in harm 's way to rescue the innocent , and without their efforts many more would have perished .

having personally witnessed the destruction at the world trade center on 11 september 2001 , this paper presents my personal feelings and observations as an observer of both disaster and terror . aside from the unimaginable horror as a result of the carnage , a feeling of helplessness was particularly prominent due to the inability to be able to care for casualties since most victims were fatalities . the passage of time has enabled a return to normalcy , however ' normalcy ' carries a new definition due to the vastness of the tragedy and the sudden threat of bioterrorism and other weapons of mass destruction .

a 65-year - old male who was a chronic smoker for the last 30 years presented with generalized weakness , breathlessness , occasional hemoptysis , and multiple cutaneous nodules ( 8 in number ) all over the body since two months . the nodules were present over the chest ( 2 ) , abdomen ( 1 ) , back ( 2 ) , face ( 1 ) , and both the extremities ( 2 ) . they were of varying sizes , ranging from 2 to 6 cm and were firm , nontender , and mobile . a clinical diagnosis of chronic obstructive pulmonary disease ( copd ) with cutaneous lymphoma or soft tissue tumor was made . clinical photograph showing nodule over abdominal wall hematological investigations revealed the following : hb ( hemoglobin ) : 14.4 gm% ; tlc ( total leukocyte count ) : 19,100/cmm ; dlc ( differential leukocyte count ) : p87l10e0m03 . esr ( erythrocyte sedimentation rate ) : 28 mm at the end of one hour . chest x - ray ( cxr ) and computed tomography ( ct ) showed neoplastic right lung and bronchial mass with secondary cavitation . excision biopsy of the noninfected nodule from the back was done and the specimen was received by the department of pathology . on gross examination , it was an irregular tissue measuring 63.51 cm with a nodule 3 cm in diameter which was surrounded by adipose tissue and muscle . the histopathological sections from the nodule showed metastatic deposits from squamous cell carcinoma , moderately differentiated ( grade ii ) [ figures 2 and 3 ] . photomicrograph showing metastatic deposits from squamous cell carcinoma ( grade ii ) [ h & e , 10x ] photomicrograph showing pleomorphic tumor cells infiltrating muscle fibers [ h & e , 40x ] cutaneous metastasis refers to the growth of cancer cells in the skin originating from internal cancer . in most cases , cutaneous metastasis develops after initial diagnosis of the primary internal malignancy and late in the course of the disease . in very rare cases , skin metastasis may occur at the same time or before the primary cancer has been detected . in the present case the most frequent source of metastases in men are the lung ( 24% ) , colon ( 19% ) , melanoma ( 13% ) , and oral cavity ( 12% ) . in women , they are the breast ( 69% ) , colon ( 9% ) , melanoma ( 5% ) , ovaries ( 4% ) , and lung ( 4% ) . in men younger than 40 years , the most common source of cutaneous metastases is melanoma , whereas in males older than 40 years , the most common source is carcinoma of the lung . our case was a 65-year - old male and the source of cutaneous metastases was carcinoma of the lung . the percentage of patients with lung cancer who develop cutaneous metastases ranges from 1 to 12% . commonly , the skin metastases are seen over the chest , abdomen , followed by the scalp , head and neck , extremities , and back . clinically , the common presentation of cutaneous metastases is in the form of solitary or multiple nodules . other morphological patterns include carcinoma erysipeloides , carcinoma en cuirrase , carcinoma telangiectaticum , sister mary joseph 's nodule , alopecia neoplastica , and cicatricial and bullous forms . usually they present as fast - growing solitary or multiple nodules with a diameter of 5 mm10 cm and are firm , mobile , and covered with normal skin . sometimes exudative or ulcerative lesions are also seen . in the present case , the nodules varied in size from 2 to 5 cm in diameter , and were firm and mobile . the most common histologic type of carcinoma of the lung that metastasizes to skin is adenocarcinoma followed by squamous cell carcinoma , and small - cell and large - cell carcinoma . carcinoma of the breast and carcinoma of the oral cavity spread via lymphatics , whereas the rest of the cancers spread mainly via a hematogenous route . lymphatic dissemination may explain why skin metastases tend to be close to the primary site of the tumor . however , in our case , the metastatic nodules were seen all over the body , suggesting lymphatic as well as hematogenous spread . the combination of radiotherapy and chemotherapy , patients with lung cancer with cutaneous metastases have a poor prognosis ; median survival of patients with lung cancer metastases is 36 months .

a 65-year - old male with a history of smoking since 30 years presented with breathlessness , hemoptysis , multiple swellings all over the body , and weakness in september 2010 at our hospital . clinically , a diagnosis of chronic obstructive pulmonary disease ( copd ) with cutaneous lymphoma or soft tissue tumor was made . chest x - ray ( cxr ) and computed tomography ( ct ) scan revealed a neoplastic lesion in the right lung with secondary cavitation . biopsy of the cutaneous nodules showed metastatic deposits from squamous cell carcinoma . metastatic skin cancer is a relatively rare complication of internal malignancy . the clinical features of metastatic skin disease vary enormously . they may present as erysipeloid , sclerodermoid , alopecia neoplastica or in an inflammatory or bullous form or as multiple nodules as in our case . a high index of suspicion for metastatic deposits is required in an elderly male patient who is a known case of lung cancer or even one who is a chronic smoker and presents with such cutaneous lesions .

ganciclovir , a drug against cytomegalovirus ( cmv ) infection , is generally well tolerated , but can cause neurotoxicity such as encephalopathy . although ganciclovir - induced encephalopathy has been described in several reports , a literature search revealed that ganciclovir concentrations in the blood or cerebrospinal fluid were previously measured in only 3 patients with encephalopathy . prompt and accurate diagnosis is thus sometimes difficult , and is derived solely from accumulated clinical information of definite cases , since ganciclovir concentrations , not routinely measured , become available after several days or a few weeks . here , we summarize clinical information of all patients with definite ganciclovir - induced encephalopathy including our own patient , who had severe symptoms , with the highest reported trough concentration of ganciclovir in the blood , and underwent therapeutic dialysis with complete recovery . encephalopathy can be caused by neurotoxicity with prophylactically or therapeutically administered drugs such as acyclovir , ganciclovir , and their prodrugs , valacyclovir and valganciclovir . the drugs are structurally similar nucleoside analogues , but their effects on neurons are poorly understood . acyclovir - induced encephalopathy is more widely known than ganciclovir - induced encephalopathy , possibly because acyclovir is used against herpes virus infection , which is more common than cytomegalovirus ( cmv ) infection , for which ganciclovir is used . ganciclovir was also reported to be effective against hepatitis b infection , but has rarely been used for this indication recently . although ganciclovir - induced encephalopathy has been documented previously , a literature search revealed that ganciclovir concentrations in the blood or cerebrospinal fluid ( csf ) have been reported in only 3 patients [ 1 , 2 , 3 ] . previous reports on patients with such definite ganciclovir - induced encephalopathy have suggested that the trough concentration of ganciclovir in the blood is important . here , we summarize clinical information of patients with definite ganciclovir - induced encephalopathy including our own patient , who had severe symptoms , with the highest reported trough concentration of ganciclovir in the blood , and underwent therapeutic dialysis . a 55-year - old man started to receive hemodialysis because of diabetic renal failure 2 years previously . he underwent renal transplantation 1.5 years previously , and had been receiving immunosuppressants since then . eight months after transplantation , the serum creatinine level increased to 4.4 mg / dl . he had cmv enteritis with occult blood in the stool and an elevated cmv pp65 ( c7-hrp ) antigen level in blood mononuclear cells . intravenous ganciclovir ( 150 mg / day ) was administered for 11 days , followed by valganciclovir ( 450 mg / day ) . because the enteritis was very severe , ganciclovir and valganciclovir two days after starting valganciclovir , he had unsteady gait , but could walk unaided . on the next day , the patient needed assistance with walking . his consciousness was mildly disturbed ( e3 , v5 , and m6 on the glasgow coma scale ) . two days later , he was found on the floor after falling , without major injuries . nine days after starting valganciclovir , his level of consciousness worsened ( e3 , v3 , and m5 ) , and he could not receive oral drugs , including valganciclovir . encephalitis was unlikely , since no meningeal signs or fever was noted ; the cell count was normal ( 0.33 cells/l ) in the csf , and the protein concentration marginally elevated ( 54 mg / dl ) . cmv , herpes simplex virus , varicella - zoster virus , and epstein - barr virus dna was later found to be negative in the csf . because of the risk of further falls , hemodialysis using a vps-15 ha membrane , a vitamin e - coated polysulfone membrane ( asahi kasei kuraray medical , japan ) was performed twice in 2 days . his consciousness improved considerably after the first session of dialysis ( e3 , v4 , and m6 ) and was completely restored on the next morning after the second session ( e4 , v5 , and m6 ) . the trough levels of ganciclovir in the serum and csf were retrospectively measured and are shown in table 1 . generally , ganciclovir is well tolerated , but caution is required in patients with renal impairment . additionally , the severe symptoms in our patient may have been attributed to high doses of ganciclovir or valganciclovir for treatment of severe cmv enteritis . although the trough blood ganciclovir concentration in our patient was the highest reported to date , his csf ganciclovir concentration was the second highest among the 4 patients reported to date in the literature . such discrepancies between the csf data and blood data may be explained by other factors , including penetration rates ( how much ganciclovir passes through the blood - brain barrier ) , which are reported to vary among individuals . however , csf concentrations may not necessarily reflect the severity of encephalopathy . as an extreme example , 1 previous patient with an undetectable level of ganciclovir in the csf ( patient 2 in table 1 ) still had disturbed consciousness . we speculate that the severe symptoms in our patient might have been attributed to peripheral nervous system involvement , which may be more sensitive to blood ganciclovir concentrations . although definitive conclusions must await further studies , blood trough concentrations of ganciclovir may be more closely related to symptom severity than csf concentrations . however , a fall , as observed in our patient , can cause devastating complications , an extended hospital stay , or both . in such patients dialysis also has a positive effect on the function of the transplanted kidney . in summary , our experience suggests that therapeutic dialysis is a safe and effective treatment for encephalopathy as well as for a damaged transplanted kidney . the measurement of ganciclovir concentrations in the csf may not be feasible for the management of encephalopathy in individual patients because it is not routinely performed , and because several days to several weeks are required for the results . nonetheless , measurement of ganciclovir concentrations can play an important role in confirming the diagnosis and evaluating disease severity . we believe that accumulated knowledge on such patients with confirmed encephalopathy will lead to prompter and more accurate diagnoses .

backgroundganciclovir , a drug against cytomegalovirus ( cmv ) infection , is generally well tolerated , but can cause neurotoxicity such as encephalopathy . although ganciclovir - induced encephalopathy has been described in several reports , a literature search revealed that ganciclovir concentrations in the blood or cerebrospinal fluid were previously measured in only 3 patients with encephalopathy . symptoms usually include confusion and disturbed consciousness , which mimic cmv encephalitis . prompt and accurate diagnosis is thus sometimes difficult , and is derived solely from accumulated clinical information of definite cases , since ganciclovir concentrations , not routinely measured , become available after several days or a few weeks.case presentationhere , we summarize clinical information of all patients with definite ganciclovir - induced encephalopathy including our own patient , who had severe symptoms , with the highest reported trough concentration of ganciclovir in the blood , and underwent therapeutic dialysis with complete recovery.conclusionour summary of patients with definite encephalopathy could lead to prompt and accurate diagnoses .

lowering core temperature has complex and divergent effects on the myocardium , stimulating or inhibiting contractility and heart rhythm through several different mechanisms . the effects vary with the depth of hypothermia and are influenced by volume status , heart rate ( which itself is affected by hypothermia ) , endocrine factors , and ( crucially ) extracellular and intracellular electrolyte concentrations . in general , moderate hypothermia ( 30 to 34c ) improves myocardial contractility whereas deep hypothermia ( < 28c ) has the opposite effect . under specific circumstances ( presence of tachycardia , hypovolemia , or electrolyte disorders ) , electrolytes , particularly calcium , play a key role in maintaining myocardial contractility through mechanisms that are also temperature - dependent . in the previous issue of critical care , rungatscher and colleagues present the results of a study on a key issue affecting deep hypothermic circulatory arrest ( dhca ) : reversing calcium - mediated persistent myocardial dysfunction after rewarming from dhca . moderate hypothermia ( 30 to 34c ) increases intracellular ca transients in a dose - dependent fashion , thereby increasing myocardial contractility . however , deep hypothermia ( < 28c ) can lead to intracellular calcium overload , especially when hypothermia is maintained for more than 30 minutes [ 3 - 5 ] . intracellular hypercalcemia is corrected only slowly during and after rewarming from deep hypothermia , often leading to persistent myocardial dysfunction [ 3 - 5 ] . in addition , deep hypothermia can induce calcium desensitization , which usually develops during rewarming after dhca . diastolic relaxation , in turn , is rate - limited by removal of ca from the cytoplasm and the rate of cross - bridge detachment . these issues are further complicated by hypothermia - induced changes in excretion of calcium and other electrolyte levels as well as on electrolyte shifts and changes in intracellular ph . urinary electrolyte excretion can increase markedly during induction of hypothermia , and a pronounced intracellular shift of potassium , magnesium , phosphate , calcium , and ( to a lesser extent ) sodium will occur [ 7 - 9 ] . all this can have profound effects on the myocardium ; this applies in particular to hypocalcemia , which can cause myocardial dysfunction , hypotension , arrhythmias , and failure to respond to drugs that act through calcium - mediated mechanisms ( norepinephrine , dopamine , digoxin , and so on ) . deficiencies of magnesium , potassium , and phosphate can also induce myocardial dysfunction and arrhythmias . conversely , intracellular influx of magnesium can help stabilize cellular membranes , improve intracellular energy management , and mitigate effects of calcium overload ( which can occur during deep hypothermia but also during ischemia ) . in addition , intracellular ph increases during hypothermia , potentially enhancing myocardial contractility by offsetting the calcium - desensitizing effect of hypothermia . intracellular na can also increase , leading to increased production of reactive oxygen species ( ros ) in the mitochondria . this may initially improve intracellular homeostasis through more effective intracellular signaling and energy management , but when the rise is excessive , ros can severely damage intracellular structures . however , in yet another twist , mild hypothermia itself mitigates excessive ros production , countering the potentially dangerous nitric oxide synthase toxicity . thus , the effects of moderate hypothermia on electrolytes in general and on calcium in particular are convoluted , with numerous and often conflicting effects on myocardial tissue . however , with deep hypothermia , some myocardial dysfunction will almost invariably occur , which is mediated to a substantial degree by intracellular calcium overload . rungatscher and colleagues compared epinephrine with the calcium sensitizer levosimendan to reverse myocardial dysfunction after rewarming from dhca in a rat model . they report that levosimendan was significantly more effective in reversing systolic and diastolic myocardial dysfunction , independent of volume status . levosimendan also improved ventricular relaxation , better preserved myocardial atp content , and reduced plasma lactate concentrations . the same authors previously reported that levosimendan has better inotropic and lusitropic effects than epinephrine during rewarming from dhca . others have published similar findings , with positive inotropic effects of levosimendan irrespective of the temperature . given these experimental data , levosimendan appears to be a highly promising drug to improve myocardial function during and after deep hypothermia , with a number of theoretical advantages over the commonly used epinephrine . the drug now needs to be evaluated in clinical studies , not just in dhca but also for accidental hypothermia , in which rewarming is often complicated by major hemodynamic problems . given the mechanisms outlined above , hemodynamic effects of hypothermia can be hard to predict . moderate hypothermia ( 30 to 34c ) , induced under controlled conditions ( euvolemia , preventing discomfort through sedation or other means , preserving normal serum electrolyte levels ) , will usually decrease heart rate , reduce risk of arrhythmias , markedly improve systolic function , and induce mild diastolic dysfunction . under optimal conditions , the diastolic dysfunction has only minimal effects on myocardial performance , and the overall effect of cooling will be an improvement in myocardial contractility , increase in stroke volume , reduction in cardiac output ( co ) because of the decrease in heart rate , and stabilization or slight increase in blood pressure . indeed , several clinical studies have reported the successful use of hypothermia to treat refractory cardiac shock . the balance between metabolic supply and demand improves because the decrease in metabolic demand ( 7% to 10% per c decrease in core temperature ) exceeds the drop in co . however , under different conditions ( especially the presence of tachycardia ) , myocardial contractility may be adversely affected even under moderate hypothermia . several studies have shown that increasing heart rate under normothermic conditions improves myocardial contractility and co but that increasing heart rate under hypothermic conditions decreases myocardial contractility . there is also a dichotomy in temperature effect on heart rhythm : membrane stabilization and decreased risk of arrhythmias with moderate hypothermia , increased risk of arrhythmias with deep hypothermia ( < 28c ) . the results of the study by rungatscher and colleagues for deep hypothermia suggest that levosimendan could potentially also be used to reverse the mild diastolic dysfunction that occurs in many patients during moderate hypothermia . however , it is too early to recommend the use of this promising drug outside the context of clinical trials . co : cardiac output ; dhca : deep hypothermic circulatory arrest ; ros : reactive oxygen species .

this article discusses the potential of levosimendan to treat calcium - induced myocardial dysfunction associated with deep hypothermia . moderate hypothermia ( 30 to 34c ) usually improves myocardial contractility and stabilizes heart rhythm , but deep hypothermia can cause severe myocardial dysfunction , which is mediated by intracellular calcium overload . in experimental studies , levosimendan appears effective in reversing this . clinical studies are needed to confirm these findings and to determine whether levosimendan could also be used for accidental hypothermia and perhaps to mitigate diastolic dysfunction under moderate hypothermia .

most of these are benign but malignant variants , called myoepithelial carcinomas ( mcs ) , are even rarer comprising about 10% of myoepitheliomas . most of these occur in the parotid gland ; a few other sites of origin have been described such as lungs , trachea , oral cavity , larynx and breast . to our knowledge there are very few countable reports of mcs of the nasopharynx in the literature which makes the nasopharynx an unusual location for mcs and considered difficult to access for the head and neck surgeon . we present a case of myoepithelial carcinoma in the nasopharynx and discuss its diagnostic and therapeutic aspects . a 60-year - old female reported to the department with a non - painful left laterocervical swelling measuring 3 cm in diameter . the patient complained of nasal blockage , hyponasal speech , conductive hearing loss and ear fullness since two months though the cranial nerve examination results were within normal limits . posterior rhinoscopy examination revealed a fungating tumor on the roof and left lateral aspect of the nasopharynx . endoscopic nasopharyngeal biopsy was taken which reported the lesion as a mucus - secreting minor salivary gland tumor . fine needle biopsy of the nasopharygeal swelling was performed , and the lesion was diagnosed as myoepithelial cell carcinoma . computed tomography ( ct ) scan depicted an irregular hypodense lesion measuring 4.5/3.8 cm in the left half of the nasopharynx , obliterating the eustachian tube opening and rosenmuller fossa . on the left side the lesion was extending to the pterygopalatine fossa and involving the parapharyngeal space . on the ipsilateral side , chest x - ray and ultrasound ( usg ) abdomen were performed as a part of the metastatic workup . axial ct scan showing the extent of the tumor in the nasopharynx the patient underwent excision of the tumor with paramedian mandibulotomy in conjunction with transpalatal approach [ figure 2 ] . histopathology reports revealed myoepithelial carcinoma of the nasopharynx with infiltrating margins and the presence of numerous sheets and cords of both spindle - shaped and epithelioid neoplastic cells [ figure 3 ] . tumor was immunoreactive for actin , smooth muscle actin ( sma ) , s-100 and cytokeratin ( ck ) markers . the postoperative period was uneventful and the patient received external beam radiotherapy to the primary site { 70 gy/ 35 fractions } as well as to the neck { 56 gy/ 28 fractions } which was started within three weeks of surgery . the patient is on regular follow - up since 28 months with no evidence of recurrence at the loco - regional level till date . intraoperative photograph showing the surgical site after excision of the tumor histopathological picture of myoepithelial carcinoma showing spindle - shaped cells in the background mcs are tumors of epithelial origin that may occur with preexisting benign lesions like pleomorphic adenomas or benign myoepitheliomas , but they also may arise de novo . nilles and associates and tuncel and colleagues reported only two cases of malignant myoepithelioma in the rhinopharynx that were confirmed histologically and immunohistochemically . in 1983 , nofal described a poorly differentiated spindle - cell carcinoma that could have been the third case of rhinopharyngeal myoepithelial carcinoma . mc is usually insidious in onset , a painless mass that originates in the parotid gland . however , other sites such as the palate , larynx , gums , retromolar area , and breast have also been reported . in the parotid gland , mcs usually affect patients over 50 years of age with no sex predilection . because they are relatively painless tumors , their diagnosis can be delayed by months or even years . when mcs occur in the nasopharynx , the symptoms are the same as those of other tumors that affect this region , such as nasal obstruction , ear fullness , serous otitis media , and conductive hearing loss . to ensure a correct early diagnosis of nasopharyngeal tumors , the lesion should first be evaluated via fiberoptic nasopharyngoscopy to identify the mass , which is usually covered with unaltered mucosa . diagnostic imaging ( ct and magnetic resonance imaging ( mri ) ) allows the site and the extension of the tumor to be established , thus permitting a correct surgical approach . the diagnosis can be further ascertained by taking a biopsy specimen for histological confirmation of the presence of a tumor and its type . histologically , mcs appear to have pleomorphic spindle - shaped or more rounded cells , occasionally with eosinophilic cytoplasm ( otherwise known as plasmacytoid cells ) . and torlakovic and associates demonstrated that tumoral cells are immunoreactive to cytokeratin , smooth muscle actin , and s-100 protein . our patient was no exception , and this profile allowed the final histological diagnosis of myoepithelial carcinoma . the criteria that indicate the malignancy of myoepithelial neoplasms include their destructive infiltrating growth , cellular pleomorphism , necrosis , and an increase in mitotic activity . locally , this type of tumor is particularly destructive , but its clinical and biological features are not yet fully understood . some authors believe that metastases are uncommon , while others have reported metastases in 30% of the cases . it has been reported that mcs in the parotid may spread to the submandibular and supraclavicular lymph nodes , hence requiring neck dissection procedures . the treatment of choice for mcs is surgical excision , but there are no specific guidelines for the treatment of nasopharyngeal mcs . however , it seems reasonable to apply the criteria used for mcs involving the most common site , the parotid . so any type of selective neck dissection will prove to be a diagnostic as well as a therapeutic procedure . unlike certain nasopharyngeal tumors for which chemotherapy and radiotherapy are the preferred treatments in some cases , the application of these treatments to mcs has not been encouraging . several surgical approaches have been proposed , depending on the size and site of the tumor.[6812 ] we adopted excision of the tumor with paramedian mandibulotomy in conjunction with a transpalatal approach . to our knowledge , this patient is the only case of nasopharyngeal mc treated with this technique . it enabled complete resection of the lesion with negative margins , and there have been no signs of recurrence and metastasis during the 28-month follow - up period with stable dental occlusion , no velo - pharyngeal incompetence and no signs of trismus .

myoepithelial carcinomas are rare tumors of salivary glands . most occur in the parotid gland buta few other sites of origin have also been described . myoepithelial carcinoma of the nasopharynx has only been reported very few times . because the lesion is so rare in the nasopharynx , there are no specific guidelines for its treatment . we present a rare case of myoepithelial carcinoma in the nasopharynx and discuss its diagnostic and therapeutic aspects .

the 22q11.2 deletion syndrome is one of the most prevalent genetic disorders and has been found to be one of the most common causes of learning disability and mild mental retardation . it is caused by micro deletion in the long arm of chromosome 22 and its clinical characteristics include congenital heart disease ; palatal abnormalities ; hypocalcaemia and t - cell immunodeficiency ; and abnormal faces that include a bulbous nasal tip and prominent nasal root , narrow and with flat cheeks , narrow palpebral fissures , small mouth , receding chin and small cupped ears . children with 22q11.2 deletion syndrome have been found to have a substantially greater risk for the development of schizophrenia and bipolar disorder with approximately 25 - 30% presenting psychotic symptoms in adolescence . we describe the case of an 18-year old patient suffering from 22q11.2 deletion syndrome . since adolescence , he manifested with behavioral disorders , aggression , verbal abuse and sleep disorders . diagnosis of 22q11.2 was confirmed at the age of 3 with a special chromosomal detector ( d22 s75 , oncor ) . after a heated argument with his parents , he presented intense anger , started breaking and throwing things and was verbally aggressive . the treatment calmed him down , but a few hours later , he presented with cervical dystonia and emergence of torticollis and trunk dystonia with continuous twisting movements of the trunk and limbs , as well as accompanying dysarthria and pain . the patient visited the emergency outpatient clinic of the hospital , where he was given 1 amp biperiden i.m . his acute dystonia was considered a side effect of aloperidin administration and he was subsequently subjected to full laboratory testing to investigate the risk factors associated with acute dystonia . the brain magnetic resonance imaging ( mri ) revealed no abnormal findings and he was then subjected to a datscan to investigate a possible dysfunction of the basal ganglia , which proved negative . after two weeks the patient was hospitalized in a psychiatric clinic and was administered quetiapine 200 mg , p.o . this particular treatment improved his clinical picture , without manifesting the usually induced side effects evoked by haloperidol . the keywords were selected from titles , abstract and keywords and they were the following : dystonia parkinsonism and di george syndrome . suggest that 22q11.2 deletions represent a novel genetic risk factor for early - onset of parkinson disease ( pd ) with variable neuropathological presentation reminiscent of lrrk2-associated pd neuropathology . shulman et al . emphasize that the 22q11.2ds - associated microdeletion affects approximately 30 genes , including several intriguing candidates with potential links to pd pathophysiology . among the genes in this region is comt , encoding a key enzyme involved in dopamine catabolism and an established therapeutic target in pd . an increased risk to first degree relatives , even in apparently sporadic cases , suggests multifactorial causation of pd . hereditary forms account for only about 1 - 3% of all pd cases and tend to have younger age at onset . mutations in parkin , pink1 and dj1 are known to cause autosomal recessive early onset pd and mutations in alpha synuclein , ubiquitin c terminal hydrolase and lrrk2 can cause autosomal dominant pd with variable age of onset . report a case of 22q11 deletion in a 17-year - old girl that was initially diagnosed as paranoid schizophrenia . in the case describe , transitory hypocalcemia induced dystonic symptoms that were believed to be catatonic symptoms or neuroleptic secondary effects . describe a new male patient , 33-year - old , with 10p partial deletion affected by hypocalcemia , basal ganglia calcifications and a severe autistic syndrome associated with mental retardation . the patient presented with dysmorphic features and at neurological evaluation , bilateral complete cataract , hypotonia , symptoms have been observed in 14% to 28% of children with di george syndrome and the receipt of antipsychotic treatment increase the risk of dystonic symptoms . dystonia induced by aloperidin may arise from an imbalance between dopaminergic and muscarinic receptors activity in nigrostriatum in the brain . the possible side effects of antipsychotic medications either accelerating or reveal dystonic symptoms merit further study . studies of di george syndrome add to the global understanding of the pathophysiology of the neurological symptoms of this common neurodegenerative disease .

the 22q11.2 deletion syndrome ( di george syndrome ) is one of the most prevalent genetic disorders . the clinical features of the syndrome are distinct facial appearance , velopharyngeal insufficiency , conotruncal heart disease , parathyroid and immune dysfunction ; however , little is known about possible neurodegenerative diseases . we describe the case of an 18-year old patient suffering from 22q11.2 deletion syndrome . since adolescence , he presented with behavioral disorders , recommended treatment with 2 mg aloperidin and he presented cervical dystonia and emergence of torticollis and trunk dystonia . antipsychotic medications either accelerate or reveal dystonic symptoms .

a 69-year - old female presented with the complaints of headache and pain in the left eye of one month duration . she was a known patient of unilateral pseudoexfoliation glaucoma , who underwent manual small incision cataract surgery with mitomycin c ( 0.2 mg / ml for 2 min ) augmented trabeculectomy in her left eye . it was a single site combined surgery in the superior quadrant of the eye , with a fornix based conjunctival flap and a single releasable 10 - 0 nylon suture anchoring the scleral flap using a modified ( first bite in peripheral cornea ) kolker 's technique . surgery was performed 3 years back and she lost to follow - up after 1 month of surgery . at present , she had no history of diminution of vision or trauma to the left eye and was not a diabetic . anterior segment examination of the right eye showed pseudoexfoliation material on the pupillary ruff with posterior chamber intraocular lens in the bag and the left eye showed clear cornea with a releasable suture , a low noncystic bleb with an elevated area and congestion overlying it , normal anterior chamber with pseudoexfoliative material on the pupillary ruff and a posterior chamber intraocular lens in the bag [ fig . 1 ] . the intraocular pressure was 12 mmhg in both eyes and fundus examination showed a cup disc ratio of 0.4 with healthy neuroretinal rim in the right eye and 0.9 with bipolar notch in the left eye and age related macular degeneration changes in both eyes . the releasable suture in the left eye had mucus debris and the bleb showed collection of pus [ fig . left eye photograph showing clear cornea , normal anterior chamber , pseudoexfoliative material on pupil and intraocular lens a collection in the bleb with releasable suture on gonioscopy , ostium was free and posterior segment examination showed no signs of vitritis . nasolacrimal duct of both sides were free of infection and patient 's random blood sugar was within the normal limits . the releasable suture was removed and inoculated in blood agar and the collection of pus was expressed out along the same suture tract [ fig . 3 ] . the patient was started empirically on oral ciprofloxacin 500 mg twice daily for 5 days and topical 5% cefotaxime and 2% amikacin eye drops hourly . bleb appearance following suture removal and pus expression the blood agar culture report revealed growth of pseudomonas aeruginosa sensitive to the above antibiotics and hence the patient was advised to continue the same medications . after a week , the patient was symptomatically better , but on examination , we noticed a small recollection of pus in the bleb area [ fig . 4 ] . the collection was again expressed and inoculated in blood agar and the patient was continued on the same topical antibiotics . after 3 days and her last review visit , her left eye best corrected visual acuity was 20/30 , the eye was quiet with a low bleb devoid of collection and ostium free of infection . intraocular pressure was 10 mmhg and posterior segment continued to be free of infection . a small recollection in the bleb bleb infections following trabeculectomy are not uncommon and are associated with several risk factors such as bleb leak , thin walled bleb , presence of releasable suture , ocular adnexal infections , and the host compromised immune status . a study from a tertiary eye care hospital in south india revealed streptococcus species as the predominant isolate from blebitis associated with releasable sutures . the gram - positive organisms like staphylococcus and streptococcus species were the most frequent organisms isolated from bleb associated infections followed by gram - negative organisms . p. aeruginosa has been reported to cause bleb associated endophthalmitis with fulminant presenting features and poor visual outcomes . however , in our patient the infection was confined to the bleb with subtle signs and responded well to the antibiotics without any intraocular spread until the clearance of infection . the use of mitomycin c in trabeculectomy to enhance bleb survival and filtration has led to an increase in the incidence of bleb associated infections . releasable sutures were introduced as a means to titrate the intraocular pressure following surgery and its maximum effect is seen when removed within the first 3 postoperative weeks . complications due to releasable sutures include windshield wiper keratopathy , corneal epithelial abrasions , failure to release the suture and more importantly the suture tract remaining as a potential source of infection . the single releasable suture was not removed because the postoperative intraocular pressure was in target range and not for fear of complications such as bleb leak , shallow and flat anterior chamber , hypotony , and suprachoroidal hemorrhage . combined trabeculectomy and manual small incision cataract surgery is a popular technique in our institution for managing cataract and glaucoma simultaneously since it offers an equally effective alternative to phacotrabeculectomy . the procedure does not require expensive automated equipment and can be done with minimal of instrumentation . for such reasons patients in the lower socioeconomic status prefer this procedure over more advanced surgical technique . this case report attempts to highlight the fact that regular follow - up should be emphasized to patients undergoing small incision cataract surgery with trabeculectomy , which can also be done by a local ophthalmologist if the patient is from afar . we recommend that the releasable suture should be removed in all patients and if a decision is taken that removal of the suture is not required then the suture should at least be cut flush with the limbus , so that it retracts into the bleb and there is no track connecting the exterior of the eye to the filtering bleb . a regular long - term postoperative follow - up is very crucial following trabeculectomy , as it plays a major role in bleb survival and in preventing or detecting its complications at the earliest .

we report a case of blebitis that occurred 3 years later following a combined glaucoma and cataract surgery . it was an atypical presentation , as patient had no classical fiery looking signs of blebitis despite the isolated organism being pseudomonas aeruginosa . improvized surgical techniques like use of mitomycin c , releasable flap sutures though considered as part of the recommended procedure for better surgical outcomes , their role as potential risk factors for visually blinding complications like endophthalmitis are often overlooked . this case report throws light on such risk factors for bleb associated infections and recommends removal or trimming of all releasable sutures and the need for a regular postoperative follow - up .

incidence of chylopericardium after cardiac surgery is very low and varies from 0.2% to 1.0% ( 1 ) . a recent review of the past decade identified 33 patients with chylopericardium , few of them after cardiac surgery ( 2 , 3 , 4 ) . thomas and mcgoon ( 2 ) reported the first case of chylous pericardial effusion after cardiac surgery . there have been case reports of chylous pericardial effusion after mitral valve replacement from grinberg and colleagues ( 3 ) and also after minimally invasive mitral valve repair for degenerative disease ( 5 , 6 , 7 ) . we would like to describe one of the first cases of chylous pericardial effusion after mitral valve repair for rheumatic valve disease at our institution . a 40 year old woman presented at our clinic with severe mitral valve rheumatic stenosis . intraoperative findings showed a typical rheumatic disease with commissural fusion and thickened valve leaflets , but a relatively preserved mitral valve apparatus . we performed a mitral valve repair consisting of bilateral commissurotomy papillary muscle splitting and placement of a rigid anuloplasty ring ( seguin , st . the postoperative period was uneventful and the patient was discharged on the 7 postoperative day in good conditions . the patient was readmitted at our institution a week after on an emergency basis with clinical signs of cardiac tamponade . after confirmation by echocardiography of the presence of massive pericardial effusion , a central venous 18 f catheter was placed in the pericardial space . the average daily drainage was 75 ml but it did not decrease and stop during this period . under these circumstances and taking in consideration the prevention of other complications we decided to reexplore the patient in sternotomy in an attempt to find the place of the leakage and close it . the patient was given to eat fat 12 hours before surgery and on the operating room by gastric tube . we ligated and sutured thymus gland remnants and the pericardial edges with double suture prolen 4/0 . additionally bioglue ( bio glue surgical adhesive , cryolife ) was place over the sutured tissues to increase the possibility of closing the point of leakage . the right pleural space was opened for drainage and the patient was closed in the standard manner . macroscopic view during surgery microscopic view of the liquid the postoperative period was without any complications . the drain tubes were taken off three days after with no signs of chyle leakage . on echocardiography there was no pericardial or pleural fluid 10 days after the intervention on discharge of the patient and 25 days later on control . chylopericardium is a rare disorder that may be primary ( idiopathic ) or secondary to injury of the thoracic duct or thymus gland . pediatric cardiac operations are more commonly related to this complication because thymus gland is very active in this population and atrophies in the adult patients . the thoracic duct carries chyle from the intestinal tract to the bloodstream and usually extends from the cisterna chyli , which lies just anterior to the first or second lumbar vertebra and passes through the aortic hiatus of the diaphragm . the thoracic duct continues cephalad in the right thorax between the aorta and the azygos vein until the fifth thoracic vertebra where it crosses over the vertebral column behind the esophagus and continuing into the left posterior mediastinum . the thoracic duct passes behind the aortic arch , along the left border of the esophagus , and behind the left subclavian artery over which it arches in the anterolateral aspect of the superior mediastinum . then it descends to empty into the junction of the left jugular and left subclavian vein ( 5 ) . it is very unusual to injure the thoracic duct during adult cardiac surgery through a median sternotomy , because the course of the thoracic duct is never directly within the operative field , although some authors suggest a traction injury in which indirect forces were transmitted that tore the thoracic duct during manipulation of the heart and aorta during the operation ( 6 ) . possible mechanisms of leakage in our case is the injury to an active thymus gland or abnormal lymph channels of the pericardium . the diagnosis of chylopericardium is usually confirmed by a triglyceride level of 110 mg / dl or greater , the presence of chylomicrons in the drainage , a positive sudan stain , or if needed a lipoprotein electrophoresis . conservative and expectant management has been the mainstay during previous years with few reports of surgical approach . total parental nutrition with complete cessation of all oral intake , somatostatin , medium - chain triglyceride diets and thoracic duct ligation have been attempted to treat different clinical scenarios . rodrigues et al suggests that early definitive surgical treatment is a feasible option , which shortens the hospital stay and minimizes the complications related to chylous leak , especially protein malnutrition and reduced immunity ( 7 ) . conservative therapy is reserved for patients with idiopathic chylopericardium , those with an untreatable etiology , those considered at high risk for surgical treatment , or those with a predictably short lifespan ( 4 ) . we believe that conservative treatment may be effective in some cases but we need to know its limitations and cut - offs in terms of expecting time and amount of daily fluid drainage to establish good indications to go on the option of surgery . early intervention decreases the possibility of complications related to metabolic , immune and nutritional impairment and also costs and hospital stay . further experience and investigation is needed to have a good algorithm for the treatment of this complication after cardiac surgery . we present a case of chylopericardium after mitral valve repair for rheumatic disease , due to thymus gland tributaries injury .

abstractchylopericardium is a rare disorder that may be primary ( idiopathic ) or secondary to injury of the thoracic duct or thymus gland . pediatric cardiac operations are more commonly related to this complication because thymus gland is very active in this population and atrophies in the adult patients . we present a case of chylopericardium after mitral valve repair for rheumatic disease , due to thymus gland tributaries injury .

giant enlargement of atria in adults is rare and most commonly occurs in rheumatic valvular heart disease . despite massive enlargement many patients may remain asymptomatic .. we report a case of giant atria in the context of rheumatic mitral stenosis and severe tricuspid regurgitation in an elderly lady a 68-year - old woman with a history of rheumatic mitral stenosis and atrial fibrillation presented with dyspnea nyha class iii , palpitations , and ankle edema for 3 months . she had been diagnosed with rheumatic mitral stenosis 30 years back , on irregular follow - up and not compliant with injectable penicillin . physical examination revealed a blood pressure of 100/80 mm hg and an irregular heart rate of 136 beats / min . auscultation revealed a loud s1 at the apex , a diastolic rumble at the apex , and a holosystolic murmur at the lower left sternal border . chest radiography revealed a marked cardiomegaly with a cardiothoracic ratio of 95% suggesting massively dilated right atrium ( ra ) and left atrium ( la ) . two - dimensional echocardiogram [ figures 1 and 2 ] revealed a giant la measuring 15.2 cm 10.1 cm , an area of 170 cm , and a volume of 1326 ml . the right ventricle was small with right ventricular dysfunction which was evident from a reduced tricuspid annular plane systolic excursion of 10 mm and a right ventricular ejection fraction of 35% . tricuspid valve was thickened with noncoaptating leaflets and severe tricuspid regurgitation as evident from vena contracta width of 0.8 cm and hepatic vein systolic flow reversal [ video 12 ] . echocardiogram apical four - chamber view with giant left atrium 15.2 cm 10.1 cm echocardiogram apical four - chamber view with giant right atrium 13.1 cm 7.05 cm the patient was stabilized with intravenous furosemide , amiodarone , oral digoxin , warfarin , and spironolactone . the patient did not consent to surgery and hence was discharged home after medical stabilization . massive enlargement of atria in adults is rare and most commonly occurs in rheumatic valvular heart disease . the most common causes of enlarged ra in adults are chronic pulmonary disease , severe mitral valvular abnormalities with pulmonary hypertension , pulmonary emboli , and tricuspid valvular abnormalities . mitral valve disease ( mitral regurgitation more than mitral stenosis ) often leads to giant la . hurst defined a giant la as one that touches the right lateral side of the chest wall on chest x - ray and that the condition is almost always caused by rheumatic mitral valve disease . is widely accepted which defines giant la by the following two echocardiography criteria : large la depicted by m - mode echocardiography with diameter > 65 mm , left ventricular posterobasal wall bent inward and lying between the dilated left atrial cavity and left ventricular cavity . large la depicted by m - mode echocardiography with diameter > 65 mm , left ventricular posterobasal wall bent inward and lying between the dilated left atrial cavity and left ventricular cavity . in our case , the right atrial enlargement was due to the severe pulmonary hypertension as a consequence of mitral stenosis and severe tricuspid regurgitation . kelesidis et al . reported a giant ra ( volume of 760 ml ) in an 84-year - old woman with severe tricuspid regurgitation and severe pulmonary hypertension . patra et al . also reported a similar case of giant ra in a patient with rheumatic heart disease . according to bando et al . many of the surgeons believe that the effect of rheumatic process on left atrial elastic fibers is irreversible , and hence they tend to go only for mitral valve surgery without la reduction . complications of giant atria include thromboembolism , atrial fibrillation , and ortner 's syndrome . giant atria due to rheumatic etiology are still prevalent in developing nations . despite massive enlargement pressure symptoms , atrial fibrillation , and thromboembolism risk are very high among symptomatic patients . early recognition and appropriate primary and secondary prophylaxis of rheumatic fever could mitigate such complications of rheumatic heart disease .

dilation of atria occurs in patients with valvular heart disease , especially in rheumatic mitral regurgitation , mitral stenosis , or tricuspid valve abnormalities . we report a case of giant left and right atrium in the context of rheumatic mitral stenosis and severe tricuspid regurgitation in a 68-year - old woman .

an athletic 38 year old african american gentleman presented to the emergency room after a bout of food poisoning with chest pain and back discomfort . the patient denied any history of hematemesis , melena , dysphagia , or weight loss . he underwent a computed tomography ( ct ) scan which showed a large posterior mediastinal mass measuring 9.4 cm 7.8 cm 8.4 cm extending laterally from the esophagus and compressing the left lower lobe of the lung at the level of t8t11 ( fig . 2 ) . there were no other lesions or masses identified , no lymphadenopathy , and no free fluid . an esophageal contrast study showed extrinsic compression of the posterior esophagus with no mucosal lesion ( fig . an adjacent lymph node at 47 cm was observed , when biopsied was consistent with malignant melanoma . further workup with fluoro - deoxyglucose positron emission tomography ( fdg pet ) , magnetic resonance imaging of the brain , and fundoscopic exams showed no evidence of distant disease . a video - assisted thoracoscopy was performed to rule out gross dissemination , which would make resection futile . the tumor was observed to extend directly from the esophagus to the left lower lobe at the fissure but appeared resectable ( fig . the esophagus and stomach were mobilized and the kocher maneuver was carried out with dissection across the hiatus into the lower chest freeing up the tumor with the attached portion of the lower lobe . a left lower lobectomy was then performed to be included en bloc with the esophagus . a pyloroplasty was performed and the gastric conduit anastamosed to the proximal esophagus using an end to end anastomosis stapler . pathology of the specimen revealed a malignant melanoma , circumscribed , bordering the esophageal adventitia and the pleura of the left lower lobe ( fig . he has subsequently undergone regular ct and pet ct scans and remains disease free for over 7 years . the first described case was by baur in 1906 , and since then approximately 340 have been reported in the literature . in a recently published series of 910 esophageal biopsies in japan , this is consistent with previously published data in which pmme accounted for 0.10.2% of malignant esophageal lesions . the average age of onset is 60.5 years with a male to female ratio of 2:1 . in 1964 das gupta and brasfield demonstrated that malignant melanoma metastasized to the esophagus in 4% of autopsies of patients who had died of melanoma arising from cutaneous sites . it was not until 1963 that typical melanocytes were demonstrated in the esophagus in 4% of cadavers who did not die of malignant melanoma . until then , typical melanocytes had not been described in the esophagus , so a diagnosis of pmme was controversial . melanocytosis of the esophagus , sometimes a consequence of chronic esophagitis , may be a precursor of pmme . regardless of the pathophysiology , the prognosis of pmme is very poor and with few exceptions is uniformly fatal . reported an overall survival of 9.8 months , 14.2 months for radical resections and 9 months after limited local resection . of the patients undergoing palliative or therapeutic resection , they reported a 5-year survival rate of 4% for patients undergoing surgery in a series of 139 patients . more recently , volpin et al . calculated an improved 5-year survival rate of 37% in a review 25 patients undergoing surgical resection based on the literature between 1989 and 2000 . radical excision extends mean survival to just over a year , and the role for chemotherapy and interferon therapy is unclear . there are five reports of patients surviving more than 7 years , several of whom had chemotherapy in addition to surgical resection . in 2004 , uetsuka et al . reported a disease - free 8-year survivor who underwent sub - total esophagectomy and proximal gastrectomy after two weeks of preoperative chemotherapy with dacarbazine , nimustine , cisplatin , and tamoxifen , which resulted in shrinkage of the tumor by approximately 30% . the patient also underwent post - operative chemotherapy with 12 courses of the same regimen and continued on daily tamoxifen and monthly interferon - beta injections . in 2007 , kawada et al . reported another 7-year survivor who underwent a similar treatment with 1 course of pre - operative and 9 courses of post - operative therapy with dacarbazine , nimustine , and vincristine in addition to postoperative injections , interferon beta was injected preoperatively directly into the tumor . a patient treated with resection alone was found to have metastasis to a supraclavicular lymph node 30 months after surgery and treated with radiation and 5-fluoro uracil . this patient went on to be disease - free for at least 9 years after the original operation . report a woman who was alive and disease free 11 years after resection of pmme . interestingly , the longest reported survivor had positive lymph nodes and did not receive chemotherapy as hamdy et al . reported a woman who had a large 12 cm esophageal melanoma with positive lymph nodes found at the time of surgery . she was treated with surgical resection alone and despite positive nodes she was disease - free for at least 12 years . the patient we report here did not receive chemotherapy but is a long - term survivor who has been disease - free for 7 years . while the role of chemotherapy and interferon therapy is unclear , radical surgical resection of pmme offers the opportunity for long - term survival despite a poor overall prognosis . the patient provided written , informed consent to access his medical record and publish his case report . dr . rifat mannan interpreted and provided the pathological images and description of the histopathology .

highlightsprimary malignant melanoma of the esophagus is a rare and aggressive disease.only a handful of long term survivors have been reported.we report the case of a 7 year survivor treated with surgical resection who remains disease free.we review the literature for the treatment of primary malignant melanoma of the esophagus , which remains controversial .

haemolytic uraemic syndrome ( hus ) is a thrombotic microangiopathy characterized by coombs - negative haemolytic anaemia , thrombocytopenia and microvascular thrombosis , with many patients also experiencing acute renal failure . approximately 90% of cases have typical hus , which is secondary to infection by a shiga - like toxin - producing escherichia coli . atypical hus ( ahus ) is a relatively rare non - shiga toxin - associated form of hus and accounts for the remaining 10% of cases . ahus is associated with dysregulation of the complement system , which causes chronic uncontrolled complement activation and leads to a pro - coagulant , platelet - activation state endothelial swelling and , ultimately , thrombotic microangiopathy [ 1 , 2 ] . in approximately 50% of ahus cases , mutations in genes encoding complement regulatory proteins [ e.g. membrane cofactor protein ( mcp ) ] ahus has a poor prognosis , with 50% of patients progressing to end - stage renal disease or dying within the first year of diagnosis , with a high risk of recurrence after kidney transplantation . patients with ahus do not always respond to plasma exchange . as ahus is linked to complement system dysregulation , inhibition of this system has been suggested as a rational therapeutic approach . eculizumab ( soliris ; alexion pharmaceuticals ) is a humanized monoclonal antibody that binds to the complement protein c5 , preventing cleavage of c5 to c5a and c5b , thereby inhibiting the generation of the terminal complement complex c5b-9a . eculizumab is approved for the treatment of paroxysmal nocturnal haemoglobinuria and case reports indicate that eculizumab may also be beneficial in ahus [ 2 , 7 , 9 , 10 ] . a 44-year - old man was admitted to hospital with prolonged diarrhoea ( lasting 1 week ) , fever and anuria . on admission , blood analysis showed haemolytic anaemia : haemoglobin 6.9 g / dl , schistocytes in the blood smear , lactate dehydrogenase elevated to 1837 u / l and platelet count 111 000/dl . anti - nuclear , anti - phospholipid , anti - topoisomerase iii and anti - adamts-13 antibodies were negative . three weeks after being admitted , the patient still required haemodialysis despite receiving 4 u of fresh frozen plasma and initiating plasmaphaeresis . following 21 sessions of plasmaphaeresis , haemolysis , classical and alternative complement pathway analysis revealed normal plasmatic factor h ( 26 mg / ml , normal range 1256 ) , 70% plasmatic concentration of factor i ( normal range 71115 ) , without antibodies against factor h. the expression of mcp on leucocytes was 51% compared to control ( normal range 91109 ) and heterozygous mutation of intron 2 ( c.286 + 1g > c ) of the mcp gene . eculizumab was initiated 90 days after hospital admission at a dose of 900 mg weekly for 5 weeks , then 1200 mg every 2 weeks until week 27 . the patient received an anti - meningococcal vaccine 15 days prior to starting eculizumab . during eculizumab treatment , haemolysis was inhibited completely , thrombocytes and platelets returned to normal levels and diuresis increased slowly to 1.5 l / day . renal biopsy ( figure 1 ) conducted the second week after admission revealed occlusive thrombosis in several small- and medium - sized arteries with secondary ischaemic glomerular changes . there was extensive acute tubular necrosis in the renal tubules with focal areas of tubular atrophy . a second renal biopsy ( figure 2 ) performed 2.5 months after admission , and 3 weeks before starting eculizumab , showed changes similar to those observed at the first biopsy : thrombotic microangiopathic lesions persisted and there was deterioration of interstitial fibrosis and tubular atrophy . ahus is a rare and devastating disease linked to complement dysregulation and chronic uncontrolled complement activation . mcp mutations occur in 1015% of ahus patients and may be familial or sporadic with incomplete disease penetrance in families . ahus usually manifests in childhood , but sporadic cases have been reported that have variable onset and are often related to infections , drugs or other clinical situations that trigger complement activation . in the case reported here , the clinical disease onset seems to have a been triggered by an infection . patients with ahus , particularly those with mcp mutations , often have a poor response to plasmaphaeresis and/or fresh frozen plasma infusions [ 1 , 6 ] and require alternative or additional therapy . as an inhibitor of terminal complement activation , eculizumab may offer an effective treatment option . our patient presented with typical hus features , but a lack of response to plasmaphaeresis and no evidence of shiga toxin suggested ahus , although no family history of the disease was apparent . based on positive results from other cases [ 2 , 7 , 9 , 10 ] , we began eculizumab therapy 90 days post - admission . eculizumab was associated with inhibition of haemolysis and normalization of thrombocyte and platelet levels , allowing cessation of haemodialysis . unfortunately , renal function recovery was not possible with eculizumab in our patient . renal biopsy revealed that irreversible damage to the kidney had already been established by the time eculizumab was initiated . our case supports previous findings that initiation of eculizumab late in disease progression is unable to reverse pre - existing renal damage . early use of eculizumab in patients with ahus may prevent the thrombotic microangiopathy resulting from uncontrolled complement activation and could therefore help to avoid irreversible renal damage . indeed , eculizumab treatment has resulted in recovery of renal function in other ahus cases [ 7 , 10 ] . ahus caused by a mutation in the mcp gene has a good prognosis post - transplant , as the normal kidney corrects the defect and complement activation is maintained within the normal range . in conclusion , this case describes how eculizumab normalized the haematological parameters in a patient with ahus . probably due to the late initiation of treatment , eculizumab was unable to have an impact on the irreversible renal damage that had already occurred . consequently , we propose that early treatment initiation with eculizumab is warranted in patients with ahus in order to avoid irreversible renal damage .

atypical haemolytic uraemic syndrome ( ahus ) is a rare and life - threatening disease caused by complement system dysregulation leading to uncontrolled complement activation and thrombotic microangiopathy . we report the case of an adult patient with plasmaphaeresis - resistant ahus and hypertension treated with the complement inhibitor eculizumab . eculizumab was shown to completely inhibit haemolysis , normalize thrombocyte levels and increase diuresis . full recovery of renal function was not possible due to irreversible renal damage prior to eculizumab initiation . these findings highlight the importance of early treatment with eculizumab in patients with poor response to standard therapy , in order to avoid irreversible renal damage .

due to the high mortality that is still associated with necrotizing fasciitis of the genital and perineal regions , this condition continues to be a major challenge to the medical community despite enormous advances in both antibiotic therapy and intensive medical treatment . it was originally reported by baurienne in 1764 and is named after jean alfred fournier ( 1832 - 1914 ) , a parisian dermatologist who was the first to describe this fulminating infection of subcutaneous tissue and superficial fascia as being specific to the scrotum and penis . in 1883 and 1884 , fournier presented five cases of a rapidly progressing gangrenous infection in five otherwise healthy young men [ 2 , 3 ] . in the following decades , further cases were reported in the literature , and different terms were used to describe this clinical entity . meleney , for example , described a more generalized form of the disease in 1924 , denoting it as necrotizing fasciitis, which is still used today . whereas the cardinal points of fournier 's description of the disease included origin in healthy young men , sudden onset of clinical signs , rapid progression to gangrene , and the absence of a definite cause , table 1 shows the diagnostic criteria for necrotizing fasciitis according to fisher et al . . diagnostic criteria for necrotizing fasciitis ( according to fisher et al once neurons are destroyed , pain diminishes fournier 's gangrene is a polymicrobial infection of perineoscrotal region that manifests as a rapidly progressing necrotizing fasciitis . the morbidity and mortality in this severe complication depend on early diagnosis and aggressive surgical management . a 78-year- old male , 15 days before referral to our department had his left hydrocelectomy ( winkelmann 's procedure ) at another hospital . clinically , the patient had a temperature of 38 degrees c , peripheral pulses were palpable , cbc revealed leukocytosis ( 26000 , range : 4000 - 10000 ) , crp was elevated ( 300 , range : 0 - 8 ) , ultrasound revealed and enlarged left testis with swelling and fluids in the scrotal content as well as an enlarged epididymis . further surgical exploration of the scrotum was done , after obtaining an informed consent from the patient . histological examination of the excised scrotal tissue revealed necrosis , phlegmonous inflammation , and abscesses of the scrotum , as well as necrotizing fasciitis ( fig . histological examination of the excised scrotal tissue revealed necrosis , phlegmonous inflammation , and abscesses of the scrotum , as well as necrotizing fasciitis . the patient was further managed with hyperbaric oxygen chamber and parenteral administration of broad spectrum antibiotics ( imipenem 3 g daily and cefotaxim daily 6 g as well as metronidazol 2 g dialy ) in an intensive care unit . following resolution of his critical condition , the patient was referred to the department of plastic surgery for mesh - grafting and vacuum assisted closure and skin auto transplantation . fournier 's gangrene begins as a local infection that is caused by bacteria inhabiting the lower gastrointestinal tract or the perineum . it occurs next to the portal of entry , which is often difficult to identify . the infection progresses to an inflammatory response that spreads to the fascia , with resultant obliterative endarteritis , thrombosis of the cutaneous and subcutaneous vessels , and tissue necrosis . the synergistic action of aerobic and anaerobic organisms plays a major role in the progressive course of the infection . an understanding of the spread of fournier 's gangrene requires knowledge of the fascial structures of the pelvic floor and anogenital region . colles fascia , which is the superficial fascia of the perineum , plays an important role in this context . colles fascia is attached to the urogenital diaphragm and merges with scarpa 's fascia of the anterior abdominal wall . the spread of infection along these fascial planes was reported in fournier 's original description of a gangrene de la verge. buck 's fascia surrounds the deeper structures of the penis and the periurethral region . infection may spread from buck 's fascia to colles fascia and the dartos fascia especially in association with injuries . there is general agreement in the literature about the initial treatment of patients with fournier 's gangrene . rapid fluid resuscitation and the restoration of cardiopulmonary function have an undisputed role in the management of septic patients , who are often critically ill . likewise , initial therapy should always include broad - spectrum antibiotics to treat the infection , which is often polymicrobial in origin . since enteric bacteria are known to be frequently involved in the disease process , the antibiotic regimen must provide cover against these organisms . in the majority of cases , double or triple drug therapy with a combination of a third - generation cephalosporin , metronidazole , and an aminoglycoside for gram - negative aerobes and other organisms is instituted . the key to survival for patients with fournier 's gangrene , however , is prompt and aggressive surgical intervention . the objective of surgery is the removal of devitalized tissue in order to halt the progress of infection and eliminate the systemic effects of necrotic material , toxins , and bacteria . surgical debridement of necrotic tissue must be repeated until a clean and healthy wound bed is present . standard surgical procedures for hydrocele may cause postoperative discomfort , temporary limitation of normal activities and complications , such as hematoma , infection . frequent complications were reported after scrotal surgery and infections ( 3.6% ) . the potential patient and surgical risk factors considered were immune - compromised status , undergoing a bilateral procedure , or a high american society of anesthesiologists score . fournier 's gangrene is a well - known , often fatal fasciitis of the pelvic floor following urologic infections . predisposing factors include diabetes because of microangiopathy and polyneuropathy , alcoholism , immune - defects , and consumptive diseases . according to bnner in most cases this infection is a mixture of aerobic and anaerobic microbial agents , which result in streptococcal toxic shock - like syndrome ( tsls ) . we considered the recommendations and guidelines in the literature in our management , such as : wide surgical debridement ; intensive care unit and hyperbaric oxygenation ; broad - spectrum antibiotic coverage ; and referral for plastic surgery . fortunately we succeeded in our patient management and he recovered from the above - mentioned lethal complication .

an uncommon case of fournier 's gangrene following hydrocelectomy is described . a 78-year - old male with no remarkable previous history , who underwent hydrocelectomy in another hospital , developed fournier 's gangrene 15 days later . the patient required wide aggressive surgical debridement , hyperbaric oxygen chamber and broad - spectrum antibiotic coverage . afterwards the patient was referred for plastic surgery . fournier 's gangrene is a polymicrobial infection of perineoscrotal region that manifests as a rapidly progressive necrotizing fasciitis . fournier 's gangrene following hydrocelectomy is uncommon . the morbidity and mortality in this severe complication depend on early diagnosis and aggressive surgical management .

pyoderma gangrenosum ( pg ) is a rare dermatological condition characterized by the rapid progression of a painful , necrolytic ulcer with an irregular , undermined border and commonly affects the lower extremities , mainly in the pretibial area . the peak of incidence occurs between the ages of 20 and 50 years with women being more often affected than men . although pg is most common in the pretibial area , it can occur at any site including breast , hand , trunk , head and neck , and peristomal skin . a 54-year - old female who is known to have diabetes and hypertension presented with a painful ulcer on the sole of the right foot . she was initially treated in a primary care center with antibiotics and debridement of the ulcer . when she presented to us , she had a painful necrotic patch of 3 cm 4 cm on the sole of right foot . blood investigations showed a white blood cell count of 31,700 cells / cumm , c - reactive protein level of 218 mg / l , and erythrocyte sedimentation rate of 105 mm / h . wound swab was taken , the necrotic patch was debrided totally , and tissue and blood culture samples were sent . empirical antibiotic therapy with cefoperazone + sulbactam and linezolid the next day on review multiple ecchymotic lesions were found surrounding the ulcer which progressed over 2 days to form new necrotic patches [ figure 1 ] . infectious disease consultation was sought and the antibiotics were stepped up to meropenem and teicoplanin . review the lesions were found to be spreading in a similar fashion with violaceous purple border and multiple pustules , and it was extremely painful . antinuclear antibody profile , anticardiolipin antibody igm and igg , cryoglobulin , rheumatoid factor , anti - lupus antibody were negative . a dermatology opinion was sought , pathergy test was done , and a clinical suspicion of pg was made which was confirmed with the tissue biopsy . giemsa , ziehl neelson and grams staining of the tissue specimen were also done which revealed no organisms . the patient was started on oral steroids ( prednisolone 60 mg ) under antibiotic coverage , and the daily dressing was done . she is now kept on regular follow - up . spreading ulcer on the sole with pustules at the floor classically , lesions begin as tender papules , papulopustules or vesicles , evolving into painful and rapidly enlarging ulcers . on histology , pg lesions do not display specific features , being typically characterized by a prominent dermal neutrophilic infiltrate with abscess formation , with no signs of infection and usually without significant vasculopathy . pg has a reported incidence of 3 - 10million cases per year , with nearly half of the cases associated with an underlying systemic disease , such as inflammatory bowel disease , hematologic conditions , or autoimmune disorders . pg is a diagnosis of exclusion and can only be made after common causes of ulcers such as infection and malignancy have been ruled out . clinically , it can be mistaken as necrotizing fasciitis , hidradenitis suppurativa , or herpes infection . pathologically , both pg and necrotizing fasciitis can be characterized by sterile infiltration of the skin by neutrophils with additional nonspecific features of vasculitis , necrosis , edema , and abscess formation . while the treatment for necrotizing fasciitis and hidradenitis includes antibiotics and aggressive surgical debridement , pg is unresponsive to antibiotic therapy and debridement exacerbates pg through a process called pathergy . its ability to mimic superficial wound necrosis of infectious etiology could lead to a delay in diagnosis and correct management . differential diagnosis for pg includes : infections - bacterial including tuberculosis , viral ( herpetic ulcers ) , parasitic , topical mycoses ( sporotrichosis ) , vascular arterial or venous ulcers , vasculitis ( antiphospholipid syndrome , vasculitic rheumatoid arthritis , systemic lupus erythematosus , wegener 's granulomatosis , behcet 's disease ) , malignancies ( squamous cell carcinoma , cutaneous lymphoma ) , insect bites , self - inflicted ulcerations , and sweets syndrome . the mainstay of treatment is long - term immunosuppression often with high dose of corticosteroids ( prednisolone 0.5 2 mg / kg / day ) or low dose of cyclosporin ( 3 - 6 mg / kg / day ) . other immunosuppressors and cytostatics can be used as steroid - sparing agents , namely azathioprine , sulfasalazine , dapsone , thalidomide , minocycline , clofazimine , methotrexate , mycophenolate mofetil , tacrolimus , intravenous immunoglobulin ( ivig ) , and cyclophosphamide . the common combinations are ( 1 ) methotrexate and infliximab , and ( 2 ) cyclosporine , mycophenolate mofetil , and prednisone . ivig is a useful therapeutic option in refractory pg and can be considered in cases of resistance to or intolerance of standard immunomodulatory therapy . when a patient is presenting with a rapidly expanding painful ulcer in a vascular limb which is refractory to antibiotic treatment and is exacerbating on debridement , the possibility of pg must be considered . early diagnosis and treatment with corticosteroids and immunosuppressants can heal the lesion with minimal scarring and prevent devastating consequences such as limb amputation and death .

pyoderma gangrenosum ( pg ) is a rare disease and that affecting specifically the sole of the foot , is even rarer . here , we report the case of a 54-year - old female admitted with a painful ulcer on the sole of the right foot which was initially treated with empirical antibiotics and debridement . the disease was found to spread rapidly after each debridement . the culture reports were negative ; rheumatology workup and doppler study were within normal limits . a clinical suspicion of pg was made and was confirmed with tissue biopsy . she was started on oral steroids following which she dramatically improved . thus , when a patient presents with a rapidly expanding painful ulcer in a vascular limb that is refractory to antibiotic treatment and exacerbating on debridement , it is imperative to consider the possibility of pg .

mastocytosis is a rare disease , defined as a disorderly infiltration with mast cells in several tissues . the prevalence of the disease is approximately one in 25 - 30,000 and its clinical manifestations vary with age of onset ( pediatric vs. adult ) , disease variant ( systemic vs. cutaneous ) , severity ( indolent vs. aggressive ) and associated hematologic disorders . a 4-month - old female infant presented with generalized blisters that were first noted when she was 3-months - old . the lesions were first observed on the hands and feet and spread to the scalp , face and trunk . the parents also complained that the child was having recurrent episodes of bouts of cough , with respiratory distress along with increased body temperature and the child becomes restless and the whole body becomes red during these attacks . no family history of any skin diseases or atopy . physical examination revealed multiple , tense vesicles , bullae , erosions and hemorrhagic crusted lesions over the face , scalp and trunk [ figure 1a and b ] . chest x - ray was done for repeated episodes of respiratory distress , which shows reticulogranular patterns on both lung fields , but we were unable to find out any infectious cause for such infiltration . ( a ) multiple tense bullae and erosions developed with peau dorange - like skin on the face , scalp , and trunk , ( b ) bilateral pulmonary infiltration in chest x - ray the histological examination of a biopsy taken from the thigh revealed sub - epidermal bullae with a dense infiltration of mast cells and in the upper dermis [ figure 2 ] . the toluidine blue and giemsa stains showed that almost all of the infiltrating cells in the dermis were mast cells [ figure 3 ] . the diagnosis of dilated cardiomyopathy ( dcm ) was made based on these clinical and histopathological findings . the patient was treated with oral betamethasone and oral hydroxyzine . on follow - up after 1 month , the skin lesions were improved , pulmonary infiltration were regressed and there was no such sudden attacks of the bouts of coughs or respiratory distress after starting steroid and antihistaminic . ( a ) sub - epidermal bullae with a dense cellular infiltration in the upper dermis ( h and e , 200 ) . ( b ) dense mast cell infiltration with some eosinophils in the upper dermis ( h and e , 400 ) mast cells were stained with toluidine blue patients usually presented with sudden onset tachypnoea , tachycardia , respiratory distress , anorexia , restlessness , irritability with sudden onset high fever and the whole body becomes flushed . these symptoms may precipitate due to any cause that stimulates the release of vasodialtory cytocines from the mast cell masses in the skin or lungs . pulmonary infiltrations usually resolved in parallel with cutaneous lesions and usually do not require special treatment if the child does not present with repeated anaphylaxis . approximately 65% of individuals with mastocytosis present with disease in childhood ; nearly 55% of these patients have manifestations of disease by the age of 2 years . the remaining 35% of those that develop their disease after puberty are classified as adult onset . although the occurrence of mastocytosis appears to be sporadic , there are reports of familial mastocytosis with dominance in several families . in most children mastocytosis is limited to the skin ( cutaneous mastocytosis ) and often transient as compared with that in adults in whom mastocytosis is usually progressive and systemic . generally , we recognize three more common forms of cutaneous mastocytosis ; maculopapulous mastocytosis ( formerly urticaria pigmentosa ) , mastocytoma of skin and diffuse cutaneous mastocytosis . the changes observed are reticulo - nodular opacities , nodules and cysts . for a diagnosis of mastocytosis , at least one major and one minor , or at least 3 min or criteria should be met . major criteria includes the presence of multifocal dense infiltrates of mast cells in bone marrow or other extracutaneous organ(s ) ( > 15 mast cells in aggregate ) and minor criterias are : ( 1 ) mast cells in bone marrow or other extracutaneous organ(s ) show an abnormal morphology ( > 25% ) . ( 2 ) c - kit mutation at codon 816 in extracutaneous organ(s ) . stains such as toluidine blue or giemsa , and the demonstration of mast cell tryptase by immunohistochemistry have been used to identify mast cells within cutaneous tissues . a total tryptase > 20 ng / ml in association with a total tryptase to tryptase ratio of > 20 identifies patients more likely to have systemic mastocytosis . > 80% of cases of systemic mastocytosis show the presence of the c - kit d816v activating mutation . the allele - specific assay to detect the d816v mutation in deoxyribonucleic acid ( dna ) from archived formalin - fixed paraffin - embedded tissues has been found to be more sensitive than the traditional dna sequencing techniques . these mutations confer resistance to imatinib and those patients positive for d816v , are in general fully or partially resistant to tyrosine kinase inhibitors . the extent of the skin abnormality can be evaluated by the scorma score . from a theoretical point of view combined h1 and h2-blockers anti immunoglobulin e ( ige ) ( omalizumab ) has been shown to reduce attacks in mastocytosis patients . pre - treatment with anti - ige probably will only be effective after several weeks of treatment and only in situations where ige - mediate processes occur . imatinib inhibits wild type kit ( wtkit ) and suppresses proliferation of the human mast cell ( hmc)- 1v560 g cell line . it has been shown to have a therapeutic role in both systemic and cutaneous mastocytosis carrying c - kit mutation , while it is ineffective on inhibiting the growth of hmc-1v560 g , d816v cells . apart from wtkit , kit molecules carrying mutations in the extracellular , transmembrane and juxtamembrane domains , such as v560 g , f522c and k509i , remain sensitive to imatinib . in summary , review of the literature suggests that those at potential risk for experiencing shock or sudden death include children with extensive bullous cutaneous involvement , those with symptoms of vasodilatation , flushing and hypotension and those with early onset of disease . pulmonary infiltration with mast cells in cutaneous mastocytosis

mastocytosis is defined as a heterogeneous group of disorders characterized by an accumulation of mast cells in one or more organs , particularly in the skin , bone marrow , liver , spleen and lymph nodes . however here we describe an 11-month - old girl child presented with diffuse cutaneous mastocytosis with bullous lesion , having pulmonary involvement , which is very rare and there is no available case report from india .

thymic carcinoma is a rare , invasive neoplasm , and its clinical course tends to be more aggressive than that of thymoma [ 1 , 2 , 3 ] . the extent of disease at presentation often precludes complete surgical resection , and systemic chemotherapy plays a very important role in treatment . because of its rare occurrence , only small numbers of cases have been analyzed in previous studies published thus far [ 4 , 5 , 6 , 7 ] . previous case reports have described s-1 as a novel agent with good activity against advanced thymic carcinoma [ 8 , 9 , 10 ] . here , we report a case of advanced thymic carcinoma in which there was a partial response to combination chemotherapy consisting of carboplatin plus s-1 . a 74-year - old female was diagnosed with thymic carcinoma in october 2012 . magnetic resonance imaging ( mri ) of the chest revealed a large anterior mediastinal mass , pleural dissemination , and pericardial effusion ( fig . a ct - guided percutaneous biopsy of the anterior mediastinal mass was done and small tissue fragments were obtained . histologically , the tumor was composed of epithelioid cells with high cellularity , which had nuclear atypia and eosinophilic cytoplasm in fibrotic stroma ( fig . these findings were consistent with thymic carcinoma . according to the masaoka classification , the patient had unresectable , locally advanced disease ( iva ) . in consideration of the complication of the pericardial effusion and the fact that the patient had adequate organ function , we initially treated her with carboplatin plus paclitaxel however , an allergic reaction to paclitaxel occurred . in november 2012 , the patient provided written informed consent before treatment and she was started on carboplatin ( auc , 5 ) plus oral s-1 ( 40 mg / m twice per day ) on days 114 as second - line chemotherapy in cycles which were repeated every 3 or 4 weeks . after 2 cycles of this regimen , mri of the chest revealed a 32% decrease in the diameter of the anterior mediastinal tumor and a reduction of the pericardial effusion , indicating a partial response based on the recist criteria ( fig . hematologic toxicity consisted of grade 3 neutropenia and grade 1 thrombocytopenia , and nonhematologic toxicity consisted of grade 2 nausea , grade 2 anorexia , and grade 1 fatigue . no dose reduction was necessary . since the completion of treatment with carboplatin plus oral s-1 , the patient has remained well , and no disease progression has been observed up until the time of writing this case report ( september 2013 ) . since several reports have indicated that combination chemotherapy regimens such as carboplatin and paclitaxel [ 4 , 5 , 6 ] , and carboplatin and amurubicin are effective against unresectable thymic carcinoma , a carboplatin - containing regimen is a valid treatment option , the same as adoc ( cisplatin , doxorubicin , vincristine , and cyclophosphamide ) and code ( cisplatin , vincristine , doxorubicin , and etoposide ) . this report describes successful chemotherapy with carboplatin plus s-1 in a patient with unresectable thymic carcinoma . s-1 is an oral fluoropyrimidine agent that consists of tegafur , 5-chloro-2 , 4-dihydroxypyridine ( gimeracil ; cdhp ) , and potassium oxonate ( oxo ) in a molar ratio of 1:0.4:1 [ 11 , 12 ] . cdhp causes the serum 5-fluorouracil ( 5-fu ) concentration to rise by competitively inhibiting dihydropyrimidine dehydrogenase , which catalyzes the degradation of 5-fu , thereby increasing serum 5-fu concentrations . s-1 is expected to act more intensively than older fluoropyrimidine agents , but higher 5-fu concentrations in the intestinal mucosa can lead to severe gastrointestinal toxicities . therefore , gastrointestinal toxicity during intensive s-1 treatment is reduced by including oxo . a phase 3 trial demonstrated noninferiority of carboplatin plus s-1 to paclitaxel plus carboplatin as first - line treatment for advanced non - small cell lung cancer . also , updated results of the above study established the efficacy of carboplatin and s-1 for patients with squamous cell carcinoma as well as for patients with non - squamous carcinoma . several case reports have described s-1 as a novel agent with good activity against advanced thymic carcinoma [ 8 , 9 , 10 ] . it is reasonable to say that an s-1-containing regimen such as carboplatin plus s-1 may be effective against thymic carcinoma . to our knowledge , this is the first report to demonstrate the efficacy of carboplatin plus s-1 chemotherapy against thymic carcinoma . because of the activity of this chemotherapy regimen against thymic carcinoma and the rare occurrence of this disease , a multi - institutional prospective study to evaluate this treatment option is warranted .

thymic carcinoma is a rare but aggressive neoplasm . although there is no clearly optimal first- or second - line chemotherapy regimen for thymic carcinoma , platinum - based chemotherapy has repeatedly been shown to be of benefit to patients with advanced thymic carcinoma . some case reports have described s-1 as a novel agent with good activity against advanced thymic carcinoma . a 74-year - old female was diagnosed with thymic carcinoma complicated by pleural dissemination and pericardial effusion of carcinomatosa . she was treated with carboplatin on day 1 plus s-1 on days 114 in cycles repeated every 3 or 4 weeks . four cycles of this regimen were administered , and a partial response was confirmed . there were no severe hematological or nonhematological toxicities , and no dose reduction was necessary . to our knowledge , this is the first report to demonstrate the efficacy of combination chemotherapy consisting of carboplatin and s-1 against thymic carcinoma .

a 30-year - old , right - hand dominant woman was presented with a rapidly growing mass of the right thumb , without any history of trauma . she had first noticed a small mass in the dorsoradial aspect of the right thumb approximately one month ago . there was no generalized fever or local inflammatory signs . her medical and family histories were unremarkable . on physical examination , a 2 2 cm sized solid nontender mass was present on the radial side of the proximal phalanx of her right thumb . 1 ) . range of motions of the thumb and the neurovascular status were normal . radiographs of the right thumb showed a well - marginated cortical erosion of the radial aspect of the proximal phalanx with soft tissue swelling ( fig . the well - defined mass measured 1.4 2.3 1.5 cm , and isointense on t1-weighted images and heterogeneously high signal intensity on t2-weighted images ( fig . 3 ) . after a gadolinium contrast injection , the lesion presented diffuse enhancement , but some areas were not enhancement ( fig . although the lesion was not aggressive in radiologic studies and the size of the mass was less than 3 cm , we performed an incisional biopsy of the mass to rule out a sarcoma . at operation , the well - circumscribed , pear - shaped mass had compressed the digital neurovascular bundle and had eroded the radial side of the proximal phalanx . on histopathological examination , the specimen showed a well - circumscribed lesion alternating abundant spindle cells admixed with hyalinized hypocellular areas . the high - power view showed plump , randomly orientated spindle cells surrounded by myxoid stroma . although cellularity of the lesion was high , hyperchromatism and variations in the size and shape of the nuclei were mild . mitotic activity ranged from 5 to 10 mitotic figures per 10 high - power fields , with no atypical forms identified ( fig . the spindle cells were positive for vimentin and alpha - smooth muscle actin ( fig . its pathogenesis is unclear , but it may be triggered by trauma or a local inflammatory process . nodular fasciitis is a self - limiting reactive process , not a true neoplasm.1,6 ) it most often occurs in young adults , between 20 to 40 years of age , and rarely in old adults over 60 years of age.1 ) it typically involves the upper extremity ( 48% ) , trunk ( 20% ) , head and neck ( 17% ) , and the lower extremity ( 15%).2,6 ) nodular fasciitis is rare in the hands and feet , and very rare in the fingers.1 - 3 ) clinically , nodular fasciitis is characterized by rapid growth , and sometimes accompanied by pain and tenderness . the lesions appear as solitary , well - circumscribed , round to oval , superficial small nodules less than 2 cm in diameter.1 ) nearly all nodules have been effectively treated by local excision . recurrence is rare and occurs in 1 - 2% of all patients.3 ) mr features of nodular fasciitis are nonspecific . the lesions of nodular fasciitis appears as well - defined , round to oval masses . histological variability in tissue composition and cellularity may account for varying mr features.2,4 ) histologically , younger nodular fasciitis lesions of fibroblastic proliferation is associated with a myxoid matrix rich in acid mucopolysaccharide.7 ) older lesions tend to present a more fibrous histology . in this regard , nodular fasciitis can be subdivided into three lesions : myxoid ( type 1 ) , cellular ( type 2 ) , and fibrous ( type 3 ) . myxoid and cellular lesions are iso- to hyperintense to skeletal muscle on t1 weighted images and iso- to hyperintense to fat on t2 weighted images . fibrous lesions are markedly hypointense on t1 and t2 images.7 ) following a gadolinium injection , nodular fasciitis usually shows diffuse or peripheral enhancement . since these tumors usually show nonspecific mr imaging features , a surgical biopsy is needed to obtain proper diagnosis . microscopically , nodular fasciitis consists predominantly of plump , immature fibroblasts that are arranged in short irregular bundles and fascicles , accompanied by a dense reticulin meshwork with small amounts of mature collagen . although mitotic figures are fairly common , atypical mitosis is virtually never seen.1,6 ) a chief diagnostic criterion is the abundance of ground substance , which gives a loosely textured feathery pattern , similar to the appearance of a tissue culture.6 ) immunohistochemical stains for desmin , cytokeratin , and s-100 are typically negative in nodular fasciitis.2,3 ) nodular fasciitis in the hand needs to be distinguished from other tumorous lesions . a giant cell tumor of tendon sheath can be differentiated from nodular fasciitis clinically by a slow growth of the tumor and fixity to underlying tendon.8 ) mr features of giant cell tumor of tendon sheath are highly specific , which is low signal intensity on t2-weighted images , such as pigmented villonodular synovitis . histologically , it is characterized by a pleomorphic cell population , which includes lipid - laden foam cells , multinucleated giant cells , and round or polygonal stromal cells , which are quite distinct from the histological features of nodular fasciitis.8 ) nodular fasciitis in the hand is also often mistaken for a soft tissue sarcoma , such as malignant fibrous histiocytoma or fibrosarcoma , because of its rapid growth , rich cellularity , and high mitotic activity.2,3,6 ) thus , an incisional biopsy is usually recommended for a definite diagnosis . unlike malignant histiocytoma , nodular fasciitis can be recognized by its more loosely arranged short bundles of fibroblasts and its prominent myxoid matrix . additionally , the long , sweeping fascicular or herringbone pattern of malignant spindle cells , typical of fibrosarcomas , is never observed in nodular fasciitis.2 ) cortical erosion is associated with a large range of soft tissue tumors and other conditions . to the best of our knowledge , bone involvement associated with nodular fasciitis in the hand has been described in a few reports.9,10 ) most occurrences of nodular fasciitis are indeed superficial , subcutaneous or fascia based , and distant from bone and periosteum . moreover , nodular fasciitis usually presents as a rapidly growing mass leading to prompt surgical excision before bone involvement secondary to remodeling might be observed.9 ) we experienced a very rare case of nodular fasciitis in the hand , associated with cortical erosion . since this lesion may be confused with other neoplastic disease , we should emphasize the importance of a careful radiologic and histologic evaluation to avoid unnecessary aggressive surgery .

nodular fasciitis is a benign , reactive myofibroblastic tumor that is often mistaken for a sarcoma because of its histological appearance and rapid growth . involvement of a finger is extremely rare . we report a case of nodular fasciitis of the thumb , accompanied by bone erosion . magnetic resonance findings suggested the possibility of a malignancy , which could have led to misdiagnosis as a malignant soft tissue sarcoma . instead , the lesion was treated by excisional biopsy , which confirmed nodular fasciitis . there has been no evidence of local recurrence at recent follow - up , 1 year after surgery . this case illustrates that , to avoid unnecessarily aggressive surgery , nodular fasciitis must be included in the differential diagnosis for any finger lesion that resembles a sarcoma , even if bone erosion is present .

the patient may present with subcutaneous nodules or indurated plaques , which may precede or appear subsequently to the joints involvement of chronic tophaceous gout . pathogenesis of gouty panniculitis is not fully understood but it has been postulated that overproduction and accumulation of uric acid are triggered by preexisting subcutaneous tissue damage with concomitant localized inflammation . we report a case of gouty panniculitis presenting as extensive subcutaneous involvement with no significant history of gout . a 40-year - old man presented with a 2-year history of widespread nontender , firm , and white to yellow nodules and plaques over the trunk , arms , legs , and dorsum of both feet . the patient reported intermittent arthralgia and occasional swelling of both ankles over the past 4 years . the patient denied other concomitant medical conditions , including gouty arthritis or history of alcohol abuse . he reported a negative familial history of gout or renal diseases . physical examination revealed a body mass index ( bmi ) of 34 and blood pressure of 140/80 mmhg . other systemic evaluation was unremarkable except for the presence of nodules and plaques with some ulceration covered with a chalk - like substance over all extremities , trunk , and dorsal feet ( figure 1 ) . clinical signs of arthritis and arthralgia were not appreciated at the time of examination . upon further investigation , the patient s serum uric acid was elevated to 12.2 mg / dl ( normal range 3.67.7 mg / dl ) . complete metabolic panel and complete blood cell count were within normal range except for the x - rays of the hands and feet which showed some osteophytes without significant bony destruction . a skin biopsy demonstrated basophilic , amorphous material surrounded by foreign body granulomas , located in the deep dermis and subcutaneous fat lobules with focal lymphohistiocytic infiltrate ( figure 2a d ) . periodic acid - schiff ( pas ) and acid fast bacilli ( afb ) stain were negative for microorganisms . given the clinical and laboratory findings , the diagnosis of disseminated gouty panniculitis was made . gouty panniculitis has been described as a rare cutaneous manifestation of gout characterized by the presence of monosodium urate crystal deposition in the subcutaneous tissue with predominantly lobular inflammation . while an association with elevated serum uric acid levels is evident , our current knowledge of the pathogenesis is incomplete . pre - existing tissue damage induced by venous stasis and micro - trauma may play roles . literature review suggested that gouty panniculitis can occur before or after the development of classic tophaceous gout . gouty panniculitis clinically presents as indurated nodules or plaques with an irregular surface , found predominantly on the lower extremities with a tendency to ulcerate and drain a chalk - like substance [ 1,35 ] . unlike previous reports , our case demonstrated extensive skin lesions found not only on the lower extremities , but over the trunk and upper extremities . the condition has been reported to be related with the elevation of serum uric acid level in chronic tophaceous gout with a suboptimal treatment as a risk factor for the development and progression of gouty panniculitis . in our case , the diagnosis of disseminated gouty panniculitis was made according to a combination of generalized cutaneous lesions and histopathological findings of deep dermal and subcutaneous deposits of amorphous material with needle - shaped spaces surrounded by granulomatous reaction with doubly refractile crystals and negative birefringence under polarized microscopy . however , in our case , we did not perform an alcohol - fixed skin biopsy to demonstrate the more prominent , brownish , negative - birefringent , needle - shaped crystals under polarization as another helpful diagnostic method to detect urate deposits . differential diagnosis of widespread gouty panniculitis includes other crystal deposition disease , such as pseudogout and oxalosis . for pseudogout , translucent , rhomboid and rod - shaped crystals with cutaneous oxalosis demonstrates the yellow - brown crystals or blue birefringence under polarized light in the patients with chronic kidney disease with chronic dialysis and cutaneous involvement of oxalosis as the result of vascular complication such as livedo reticularis , acrocyanosis and peripheral gangrene in the setting of hyperoxaluria . we believe the presentation of our patient , both clinical and histopathological , is similar to the previously proposed term gout nodulosis . obesity , chronic venous insufficiency , long - term use of furosemide and glucocorticoids are considered to be the risk factors for cutaneous deposit of uric acid [ 4 , 5 ] . therefore , obesity was considered to be the possible risk factor for the development of gouty panniculitis in our case . high - dose allopurinol 6001,200 mg / day and colchicine have been reported to improve the lesions of gouty panniculitis and prevent formation of new lesions . our patient was treated with allopurinol 600 mg / day , which resulted in the gradual improvement of skin lesions over 3 months period . serum uric acid level decreased to 8.4 mg / dl over 6 months period and there were no episodes of arthritis or arthralgia at the latest follow - up visit . disseminated gouty panniculitis is an extremely unusual presentation of cutaneous tophi and may not be apparent to the physician in early stages of its development . clinical suspicion should be taken in patients with known history of gout or long - standing hyperuricemia with the onset of new cutaneous lesions as described .

gouty panniculitis is a rare cutaneous illness characterized by the deposition of subcutaneous mono - sodium urate crystals with lobular panniculitis . only a small number of cases with gouty panniculitis have been reported in the literature with unclear pathogenesis . in this article , we present a case of disseminated gouty panniculitis in the patient who had never been diagnosed of gout but revealed significant hyperuricemia at the time of diagnosis .

in infants and children , vallecular cysts present most commonly with stridor and feeding difficulty but may cause life - threatening airway obstruction [ 1 , 2 ] . in adults , most vallecular cysts are asymptomatic but may present with globus , voice change , dysphagia , odynophagia , or dyspnea [ 3 , 4 ] . vallecular cysts may also be discovered during administration of anesthesia , where they may obscure the view of the glottis and cause difficult endotracheal intubation [ 5 , 6 ] . a 70-year - old woman was referred to our laryngology service after vallecular lesions were identified on a barium swallow study performed after several months of dysphagia ( figure 1 ) . interestingly , the patient also reported a history of angioedema , having presented to the emergency department 2 months earlier with tongue swelling . at that time , her enalapril was discontinued , and she was successfully treated with corticosteroids and antihistamines . this occurrence was followed by multiple episodes of lip and periorbital swelling , which ceased when she was started on daily cetirizine . on flexible laryngoscopy , 2 cysts were seen between the base of the tongue and the epiglottis , pushing the epiglottis posteriorly ( figure 2 ) . both cysts were approximately 2 cm in diameter , benign in appearance , nonerythematous , and with prominent overlying vasculature . a portion of the swallowed food became lodged between the cysts and base of the tongue . with multiple swallows , the vallecula was well healed with no evidence of cyst remnants ( figure 4 ) . vallecular cysts , also called epiglottic mucus retention cysts or base of tongue cysts , arise when the duct of a mucous gland or lingual tonsillar crypt becomes obstructed and dilates [ 1 , 2 ] . these cysts have therefore been classified as ductal cysts , retention cysts , and lymphoepithelial cysts and are caused by inflammation , irritation , or trauma [ 3 , 4 ] . ductal cysts may occur at any location lined by mucosa and can be found at any site in the larynx other than the free edge of the true vocal cords . ductal cysts are the most common laryngeal cysts and occur most frequently at the true vocal fold , followed by the epiglottis and vallecula [ 3 , 4 ] . vallecular cysts resembling tonsillar crypts due to associated lymphoid tissue have been separately classified as lymphoepithelial cysts and may also occur in the aryepiglottic fold , vestibule , and piriform sinus . given this pathogenesis , it would not be surprising for multiple cysts to codevelop . as stated by desanto et al . , however , to our knowledge , no specific cases of multiple vallecular cysts have been reported in the literature . additionally , an association has recently been established between infected vallecular cysts and severe supraglottic infection including epiglottitis . this patient 's history of angioedema raises the question of whether there might be an association between that disease process and the formation of vallecular cysts . infants with vallecular cysts are considered to be at risk of airway obstruction and death . therefore , all such cysts in infants and children should be removed surgically , with marsupialization via co2 laser or electrocautery being the most commonly used method [ 1 , 2 , 7 ] . it is our bias to remove the cyst in its entirety to avoid recurrence as a result of epithelial remnants . other authors have shared this preference [ 3 , 8 ] and have posited that use of a co2 laser may be superior because of potential vaporization of the epithelial lining . in adults , vallecular cysts are more common but less dangerous . the peak incidence is in the fifth decade of life , and the majority of cysts occur in men [ 2 , 3 ] . nearly two - thirds of vallecular cysts are asymptomatic and are diagnosed incidentally on routine laryngeal examination . the incidence of vallecular cysts on laryngoscopy has been reported as 1 in 1,250 to 1 in 4,200 , but the true incidence is difficult to estimate . in the case described here , the patient 's large , bilateral vallecular cysts caused dysphagia to both solids and liquids . although rare , vallecular cysts should be considered in the workup of dysphagia . additionally , vallecular cysts factor into the differential diagnoses of voice difficulty , odynophagia , and dyspnea . cysts of the vallecula may account for an array of clinical symptoms . in adults , vallecular cysts are most often asymptomatic and discovered on routine laryngoscopy or during induction of anesthesia . however , globus , dysphonia , dysphagia , odynophagia , and dyspnea may occur . in evaluating these symptoms , if a vallecular cyst is found , complete transoral laser excision will often result in cyst resolution and improved symptoms .

cysts of the vallecula are rare , accounting for 10.5% to 20.1% of all laryngeal cysts . vallecular cysts may present with diverse symptoms affecting the voice , airway , and swallowing . we describe the evaluation and treatment of a 70-year - old woman who presented with dysphagia caused by large bilateral vallecular cysts .

systemic lupus erythematosus ( sle ) is an autoimmune disease of unknown pathogenesis that affects several organs ( 13 ) . sle can affect any part of the respiratory system and have a variety of pulmonary manifestations , including the following : pleuritis , pleural effusion , pneumonitis , diffuse alveolar hemorrhage , bronchiolitis obliterans , vasculitis , pulmonary hypertension , and impaired function of the respiratory and diaphragm muscles ( 4 ) . however , the prevalence of cavitary lesions in systemic lupus is very rare ; therefore , lesions are usually secondary to infection or pulmonary embolism ( 5 ) . other diseases that may cause similar lesions include malignancy and both wegener and rheumatoid arthritis ( 6 , 7 ) . with regard to systemic lupus as a rare differential diagnosis of pulmonary cavitary lesions , early diagnosis can help prevent a need for unnecessary or invasive diagnostic measures and therefore decrease the likelihood of the development of complications that can sometimes be life - threatening . the aim of this research was to present a rare case of sle manifested with cavitary pulmonary lesions . the patient was a 19-year - old female with sle complicated by class iii lupus nephritis and was under treatment for 3 months with cellcept 300mg / tds and prednisolone 60mg / d that was gradually tapered to 35mg / d . she was referred because of a fever of 38.5 degrees celsius and a non - productive cough that lasted 3 weeks . the patient s past medical history showed joint pain ( arthralgia ) in the knees , proximal interphalangeal joints ( pip ) , malar rash , oral ulcers , and leukopenia . vital signs were as follows : blood pressure 100/70 mmhg , pulse 100 bpm , respiratory rate 20/min , and body temperature 38.5c . she had decreased breath sounds in one - third to the right lower chest area . laboratory results were : wbc ( 13800/ul ) , hb ( 14g / dl ) , crp ( + 2 ) , ana ( + ) , rf( ) , bun ( 23mg / dl ) , cr ( 0.62mg / dl ) , sgpt ( 86 iu / l ) , ldh ( 738 u / l ) , proteinuria ( + 1 ) , blood ( + 2 ) , wbc ( 1820 ) , and rbc ( 2022 ) . urine culture , blood culture , wright test , coombs wright test , widal test , c - anca ( for wegener ) , sputum smear and culture , and ppd test were reported negative . in the same way , bronchoscopy and bronchoalveolar lavage for malignancy , bacterial , viral , fungal , and m. tuberculosis infections were tested negative . coagulation tests and other laboratory tests including echocardiography and venous doppler ultrasound of lower extremity for dvt were normal . chest radiography revealed several cavities in both lungs and a blunting of the right costophrenic angle ( figure 1 ) . the general condition of the patient improved briefly after she was treated with intravenous methyl prednisolone , but a few days later she began to experience respiratory distress . a spiral computed tomography scan of the chest showed several cavities and nodules in both lungs , some of which were cavitary . mild pleural effusion and pneumothorax were seen on the right side ( figure 2 ) . the patient was treated with nasal oxygen therapy and a chest tube placed in the right side , but after a few days the patient s general condition worsened and she developed respiratory distress . in addition , a pneumothorax occurred in the left lung and a chest tube was placed in this side . sle with cavitary lesions is reported very rarely . in the study of webb and gamsu ( 8) , seven patients with sle and four were cases of infection , one a case of pulmonary embolism , and two cases with unknown etiology . in a study by torok et al . ( 4 ) , an sle case with cavitary lesions was reported who improved after the initiation of immunosuppressive therapy . also , in the study of maden et al . ( 5 ) , an sle case with cavitary lesions was reported where the lesions improved after steroid therapy . ( 9 ) also reported cavitary lung masses caused by cmv for two lupus cases on corticosteroid treatment . ( 10 ) reported cavitary pulmonary lesions in a patient with lupus under immunosuppressive therapy who had cmv pneumonitis . in this case report , a female sle case complicated by lupus nephritis with cavitary pulmonary lesions was reported . other diseases ( including malignancy , bacterial , viral , fungal , and m. tuberculosis infections , septic emboli , pulmonary thromboembolism , wegener and rheumatoid arthritis ) that lead to such lesions were ruled out . the patient improved briefly after the initiation of immunosuppressive therapy , but unfortunately was unresponsive to supportive treatment , developed respiratory distress due to pneumothorax , and died . a cavitary lung lesion associated with sle is extremely rare and may be observed in the absence of other factors that cause cavitation . this case presentation suggests that sle should be considered in the differential diagnosis of cavitary lung lesions .

systemic lupus erythematosus ( sle ) is an autoimmune disease of unknown pathogenesis . the frequency of sle with cavitary lesion manifestation is very rare and is thought to be due to infection or pulmonary embolism . a 19-year - old female diagnosed with sle complicated by lupus nephritis and cavitary pulmonary lesion is presented in this case report . other diseases that can lead to such lesions were ruled out in the patient . the patient improved briefly after the initiation of immunosuppressive therapy , but was unresponsive to supportive treatment due to pneumothorax . pneumothorax is caused by cavitary lesions and possibly bronchopleural fistulas these later caused respiratory distress and death . the patient did not show any improvement in the lesions after the initiation of immunosuppressive therapy . this case report suggests that the differential diagnosis of cavitary lung lesions should include sle .

a 41-year - old man , 174 cm in height , weighing 72 kg , with american society of anesthesiologists ( asa ) physical status i , was scheduled to under go right shoulder arthroscopy for rotator cuff repair under isb . a preoperative examination revealed there was equal air entry on both sides of his chest with no evidence of wheezing . all laboratory evaluations were within the normal limits , including electrocardiogram and chest roentgenogram ( fig . 1 ) . he agreed to undergo regional anesthesia . in the operating room , after applying routine monitors , his blood pressure , heart rate , respiratory rate and oxygen saturation was 130/80 mmhg , 70 beats / min , 16 - 18 breaths / min , and 99% , respectively . a nerve stimulator ( stimuplex-dig , b / braun , germany ) connected to a 50 mm insulated contiplex a needle and cannula ( contiplex a , b / braun , germany ) was used . after obtaining pectoralis major muscle twitching at a threshold of 0.5 ma , a 22-gauge catheter was inserted 5 cm through the cannula into the brachial sheath . intraoperatively , he was sedated with sufentanil 10 g and a propofol infusion at 50 g / kg / min . throughout the procedure his blood pressure , heart rate , respiratory rate and oxygen saturation was 115/70 mmhg , 60 beats / min , 14 breaths / min , and 97% , respectively . 0.2% ropivacaine ( naropin , astrazeneca , sweden ) infusion was started in the recovery room through the interscalene catheter using a portable battery - powered pump ( accumate 1,000 , wooyoung medical , korea ) at a basal rate of 6 ml / h . the patient had the option of self administering a 3 ml bolus of 0.2% ropivacaine ( with a 20-minute lock out time ) . the infusion was continued over the subsequent 2.5 days . on the first postoperative day , the continuous isb produced adequate analgesia ( visual analogue scale , from 0 - 100 mm , was 40 mm ) , which avoided the need for additional analgesics . the chest x - ray revealed an elevation of the right hemidiaphragm , which was associated with basal atelectasis of the right lower lobe without pleural effusion ( fig . on the second postoperative day , he reported moderate chest discomfort that was on right side that was increased by deep inspiration . he was afebrile , and his blood pressure , heart rate and respiratory rate was 110/80 mmhg , 70 beats / min , and 20 breaths / min , respecively . the chest x - ray revealed an elevation of the right hemidiaphragm associated with basal atelectasis of the right lower lobe , and pleural effusion without pneumothorax ( fig . his pain was well controlled ( visual analogue scale , from 0 - 100 mm , was 20 mm ) . rescue analgesia with 60 mg of i m diclofenac was available on demand . on the fourth postoperative day , his symptoms appeared to have improved but the chest x - ray showed no improvement . although the right hemidiaphragm had returned to normal , the chest x - ray on the sixth postoperative day showed residual pleural effusion and atelectasis . the chest x - ray showed complete expansion of the collapsed right lower lobe ( fig . the 100% incidence of short - term hemidiaphragmatic paresis reported in patients undergoing isb has been related to the spread of the local anesthetic solution , either on the c3-c5 roots or through the scalene anterior fascia . no prophylactic measure has been found to decrease the incidence of phrenic nerve block after a single - shot isb . this is not prevented by digital pressure and occurs with a variety of local anesthetics and doses . impairment of diaphragmatic movements can be rated from 50% to 77% for supraclavicular , 24% to 26% for proximal paresis of one hemidiaphragm is not normally associated with adverse clinical symptoms , even though the lung volume , pulmonary function tests , and arterial blood gas analyses are all adversely affected . a normal individual can compensate for these changes by utilizing the accessory and intercostal muscles and enhanced respiratory effort . however isb can lead to acute respiratory failure in patients with chronic respiratory disease or with preexisting contralateral phrenic nerve paralysis , and is contraindicated in such patients [ 11 - 13 ] . , the paresis can occur as high as 75% of cases and often persists until the end of the infusion . in contrast to single - shot isb , continuous isb producing long - duration phrenic nerve block is more likely to induce prolonged hemidiaphragmatic paresis and hypoventilation . the atelectasis of the right lower lobe caused by a prolonged phrenic nerve block can lead to the formation of a plural effusion . in our case , the relationship between the phrenic nerve block and the pleural effusion was not obvious at first . on the first postoperative day , the chest x - ray revealed an elevation of the right hemidiaphragm associated with basal atelectasis of the right lower lobe , but no pleural effusion . however , on the second postoperative day , the chest x - ray showed an elevation of the right hemidiaphragm associated with basal atelectasis of the right lower lobe and pleural effusion . it was assumed that the atelectasis and pleural effusion on the same side of the continuous isb was due to a phrenic nerve block . the rapid resolution of both clinical and radiologic signs after discontinuing the continuous isb supports this hypothesis . souron et al . reported a patient who developed atelectasis of the lung and pleural effusion revealed by chest pain after continuous isb . sardesai et al . also reported a case of left lower lobe collapse after interscalene local anesthetic infusion administered at home . as in our case , the treatment is to stop the local anesthetic first and check the chest x - ray regularly . in conclusion , during continuous isb , chest discomfort can indicate pleural effusion secondary to an ipsilateral phrenic nerve block , even in patients with a normal preoperative respiratory function . it is recommended that a chest x - ray be acquired rapidly to identify this uncommon complication and exclude a pneumothorax .

an interscalene brachial plexus block is an effective means of providing anesthesia - analgesia for shoulder surgery . however , it has a multitude of potential side effects such as phrenic nerve block . we report a case of a patient who developed atelectasis of the lung , and pleural effusion manifested as chest discomfort during a continuous interscalene brachial plexus block for postoperative analgesia .

a reappraisal of the priorities of cardiopulmonary resuscitation by pepe and colleagues calls attention to the evidence that defibrillation may not be the optimal initial intervention . initial precordial compression after more than perhaps 3 minutes of untreated cardiac arrest greatly improves the likelihood of successful conversion of ventricular fibrillation with restoration of spontaneous circulation . since it is often very difficult to gauge this time interval , and whether it exceeds 3 min , a number of both preclinical investigators [ 4 - 6 ] and clinical investigators have sought an electrocardiographic predictor of the likelihood that an electrical shock will restore circulation . it is to this extent that we applaud the authors ' call for caution lest the availability and promotion of automated external defibrillators diminishes the preparedness of the rescuer to proceed with more conventional basic life support . pepe and colleagues then extend their discussion to advanced cardiac life support , and especially pharmacological interventions . in the context of the time dependency of interventions , they are not prepared to discard the possibility that high - dose epinephrine will improve outcome . indeed , they favor the use of cocktails , entertaining the possibility that epinephrine may be administered conjointly with antioxidants and anti - arrhythmic drugs . although we agree with pepe and colleagues in implicating the myocardial energy / supply relationship as an important issue , we wish to point out that beta - adrenergic agonists , and to a lesser extent the alpha1 actions , greatly increase myocardial energy consumption and thereby intensify the severity of myocardial injury . although the alpha - adrenergic effects increase coronary perfusion pressure and transiently increase myocardial blood flow , the downside is major . the inotropic and chronotropic effects produce greater global myocardial ischemia , greater post - resuscitation ventricular ectopy and recurrent ventricular tachycardia , and recurrent ventricular fibrillation . even more importantly , the adrenergic inotropic and chronotropic actions result in greater severity of post - resuscitation myocardial dysfunction . although epinephrine has been used as a resuscitative drug for more than a century , and although there is evidence that epinephrine may facilitate initial resuscitation , there is no proof of ultimate clinical benefit in terms of survival . to the contrary , we suspect that vasopressor agents with no inotropic and chronotropic actions are likely to come to the fore . this has prompted interest in more selective vasopressor agents , including -methylnorepinephrine and nonadrenergic vasopressin . moreover , as yet unpublished preclinical studies from our group now suggest a place for beta - adrenergic blocking agents . looking to the future , we also see an opportunity for much improvement in sequencing interventions . in support of the conclusions reached by pepe and colleagues , additional precision in sequencing cardiopulmonary resuscitation interventions has a high likelihood of improving outcomes . perhaps one measurement , not cited by the authors , is end - tidal carbon dioxide . both experimentally and clinically , end - tidal carbon dioxide has been a noninvasive monitor of blood flow generated by precordial compression . it serves as quality control of precordial compression and allows chest compression to remain uninterrupted . no longer will advanced cardiac life support rescuers need electrocardiographic confirmation of the return of spontaneous circulation because it is overtly signaled by an overshoot in the end - tidal carbon dioxide .

the contribution by pepe and colleagues provides additional evidence that initial defibrillation is not necessarily the optimal intervention for victims of cardiac arrest and especially when cardiac arrest has been untreated for more than 3 min . precordial compression therefore remains the mainstay of basic life support cardiopulmonary resuscitation after sudden death . it is increasingly apparent that neither epinephrine whether in conventional or high doses nor vasopressin improve ultimate survival . to the contrary , there is evidence favoring 1-adrenergic blockade .

zoon 's balanitis or balanitis circumscripta plasmacellularis is a benign chronic disease of unknown origin . zoon 's balanitis , unlike some other inflammatory penile dermatoses , is generally not thought to be a precursor for neoplasia . we herein report the case of an uncircumcised man , who developed clinically and histopathologically evident squamous cell carcinoma of the penis in an area affected by zoon 's balanitis . a 71-year - old uncircumcised man presented with a 3-month history of extremely painful genital sores associated with swollen foreskin and dysuria , not preceded by any flu - like symptoms . the patient reported that he has suffered from zoon 's balanitis for 12 years , confirmed by a histopathological examination . he treated the condition with topical corticosteroids with relative improvement and paid periodic visits for 7 years ; after that he stopped performing any follow - ups . clinical examination revealed a well - demarcated , highly indurated , glistening erythematous plaque ( about 3 cm in diameter ) on the glans and adjacent preputial mucosa . a 3-mm punch biopsy of the glans was performed showing typical features of a moderately differentiated squamous cell carcinoma ( nests of atypical cells with abundant eosinophilic cytoplasm and large vesicular nucleus ) ( fig . zoon 's balanitis is characterized by a solitary red - orange plaque of the glans and prepuce . although the etiology is unknown , different factors have been reported to be involved in its genesis ( local infections , poor hygiene , heat , friction , and constant rubbing ) . histopathologic examination demonstrate an inflammatory infiltrate of predominantly plasma cells , atrophy of epidermis , spongiosis , tortuous dermal blood vessels , and exocytosis of inflammatory cells [ 2 , 3 ] . it is generally considered to be a benign condition , and its association with malignancies has been rarely reported . in 1999 , a case of penis carcinoma arising in a patient with zoon 's balanitis was described by joshi , implying that this inflammatory lesion could be a premalignant condition or could contribute to the development of squamous cell carcinoma . after this report , the hypothesis that zoon 's balanitis may be followed by penile carcinoma or premalignant lesions started to arise . in 2001 , bunker claimed that there were zoonoid changes in clinical and histological features in some cases of lichen sclerosus , lichen planus , bowenoid papulosis and penile cancer . these zoonoid changes could suggest that zoon 's balanitis per se is a premalignant condition . recently , starritt and lee reported a case of erythroplasia of queyrat of the glans on a background of zoon 's plasma cell balanitis . the hypothesis of the possible association between zoon 's balanitis and penile cancer could also be linked to the concept of inflammation as a cofactor in carcinogenesis . in 1863 , virchow noted a connection between inflammation and cancer and sustained that this link may have implications for cancer prevention and treatment . therefore , prevention of penis carcinoma is possible , including early detection and relative treatment of risk factors ( phimosis , inflammatory conditions , multiple sexual partners , and hpv infections ) and precancerous lesions [ 9 , 10 ] . this case highlights the importance of frequent follow - ups for zoon 's balanitis ; topical treatment , such as corticosteroids and calcineurin inhibitors [ 4 , 5 , 7 ] , may be important to prevent the development of a secondary carcinoma . in case of resistance to topical treatments , circumcision should be recommended because it could be curative and also protective against penile carcinoma .

zoon 's balanitis or balanitis circumscripta plasmacellularis is a chronic disease of unknown origin . this condition usually manifests in middle - aged or elderly uncircumcised men . although of unknown etiology , different factors have been reported to be involved in its genesis ( local infections , poor hygiene , heat , friction , and constant rubbing ) . it is generally considered to be a benign condition , and its association with malignancies has been rarely reported . we report the case of an uncircumcised man , who developed clinically and histopathologically evident squamous cell carcinoma of the penis in an area affected by zoon 's balanitis .

bupropion extended - release ( xl ) is an important pharmacological option except nicotine for the treatment of smoking cessation . it is thought to act on nicotine addiction by blocking dopamine reward pathway and reducing withdrawal symptoms that arise due to noradrenaline pathway furthermore , bupropion is used for the treatment of major depression because of it acts as noradrenaline dopamine reuptake inhibitor.13 ) the most common side effects with the use of bupropion can be listed as insomnia , headache , dry mouth , rash , nausea , sweating and hypertension.4 ) hypomania , psychotic relapse and visual hallusinations may also occur via use of bupropion.5,6 ) according to the diagnostic and statistical manual of mental disorders , fifth edition ( dsm-5 ) , tardive dyskinesia is characterized by choreiform or atetoid involuntary movements at least continuing several weeks due to neuroleptic use at least several months . these involuntary movements are often seen on face , arms , legs , jaws and tongues.7 ) additionally , tardive dyskinesia may develop with drugs except neuroleptics.8 ) here , we report an adult patient who developed tardive dyskinesia during treatment with bupropion . forty - six year old man was admitted to our psychiatry outpatient unit with complaints of malaise , unwillingness , fatigue , lack of energy , lack of sexual desire , hypersomnia . his physical and laboratory examination ( complete blood count , thyroid function , b12 and folate tests ) were normal . the patient received 15 points in the hamilton rating scale for depression ( ham - d ) scale . bupropion xl 150 mg per day treatment was started to the patient with a diagnosis of major depression according to the dsm-5 . after a month , due to continued complaints of depression , bupropion xl dose was increased to 300 mg per day . after two months of dose increase , the patient was admitted to our outpatient unit with complaints of involuntary movements on his tongue and lips . bupropion xl dose was decreased to 150 mg per day . after a month , due to continued involuntary movements , bupropion was stopped . naranjo adverse drug reaction probability scale was evaluated as 7 points , a probable adverse effect associated with bupropion.9 ) to our knowledge , this is the first case report regarding tardive dyskinesia associated with bupropion xl in a patient with no history of neuroleptic use or neurologic disorders . anormal involuntary movements apperared two months after bupropion dose was increased to 300 mg per day and disappeared five months after discontinuation of bupropion with simultaneous use of lorazepam . in literature , there has been three reported cases of dyskinesia associated with bupropion . in first case , 70 year old woman with bipolar disorder , dyskinesia developed 2 days after bupropion ir 75 mg per day was added to her lithium treatment.10 ) in second case , 63 year old man with major depression , dyskinesia developed one week after bupropion xl dose was increased to 300 mg per day and this patient had a history of neuroleptic medication.11 ) in third case , 64 year old woman with major depression , dyskinesia developed one week after bupropion dose was increased to 300 mg per day.12 ) in all three cases , dyskinesia developed acutely . patients were elderly in previous three cases therefore early stages of parkinson disease or other neurologic disorders has been associated with dyskinesias after initiation of bupropion . according to dsm-5 , dyskinesia associated with bupropion in our patient noted as tar - dive because of involuntary movements occured two months after bupropion dose was increased to 300 mg per day and disappeared five months after discontinuation of bupropion . also , our patient was 46 years old so he was quite young for onset of neurologic diseases compared with other cases in literature and there was no history of neuroleptic use in our patient . involuntary movements may occur with short term use of drugs as bromocriptine and l - dopa or lasting than a few weeks in which case the condition is called acute dyskinesia , if these movements develop with use of drugs for at least a few months and lasting a few weeks is called tar - dive dyskinesia according to dsm-5.7,13 ) in some patients acute dyskinesia may develop after reduction or discontinuation in dosage of drugs which is called withdrawal dyskinesia lasting less than 48 weeks . if dyskinesia persists for longer than this time is called tardive dyskinesia.7 ) yet , there is no effective and safety treatment and main treatment strategy is preventive approaches for tardive dyskinesia and it causes permanent disability , therefore tardive dyskinesia is important to early diagnosis of tardive dyskinesia in clinical practice.14,15 ) neuroleptic induced tardive dyskinesia develops via dopamin receptor hypersensitivity , reduction in gaba cycle and increased glutamate and aspartate levels after d2 receptor blokage.16 ) the mechanism of tardive dyskinesia associated with bupropion is still unclear . excessive dopaminergic transmission in striatum caused by bupropion could be the underlying mechanism of bupropion associated tardive dyskinesia.13 ) other prodopaminergic agents such as modafinil , methylphenidate , levodopa , amphetamine and other stimulants have been reported to be associated with involuntary movements.13,1719 ) finally , clinicians should be careful about tardive dyskinesia associated bupropion when they used this drug even if the patient is not elderly . new studies may contribute to understand the mechanism of bupropion associated tar - dive dyskinesia .

present report describes a 46 year old male patient with a diagnosis of major depression who developed tardive dyskinesia during bupropion therapy . our patient had no history of neuroleptic use and his laboratory and neurologic examinations were normal . he had no family history of neurologic diseases . although bupropion induced dyskinesia has been previously reported in the literature , it is rare and our case is the first case regarding tardive dyskinesia .

the aneurysms originating from carotid - opthalmic artery , supraclinoid internal carotid artery , and internal carotid artery bifurcation are more likely to present with symptoms of anterior visual pathway compression . a 65 years old , gentleman presented with headache of 3 months duration , and loss of vision in right eye of 15 days duration . he was suffering from non specific headache for which he did not seek medical attention . about 15 days back his vision decreased in right eye , which progressed to complete blindness within a week . he never had sudden onset of headache with vision loss . at admission he was conscious and alert . his best corrected visual acuity was no perception of light in right eye and 6/6 in left eye . he had relative afferent pupillary defect in right eye , and brisk pupillary reaction to light in left eye . the visual fields assessment was done with computerized static visual field analysis using zeiss humphrey . the visual evoked potential revealed absence of all waves in right eye , and increased latency ( n75 - 77.1 ms , p100 - 113.1 ms , and n145 - 157.8 ms ) in left eye . the mri of brain showed a well defined rounded suprasellar lesion measuring 26 25 23 mm , which was composed of concentric rings of differing intensities , suggestive of partially thrombosed giant aneurysm . post contrast mri showed an aneurysm arising from anterior communicating artery ( acoma ) , which was directed posteriorly [ figure 1 ] . digital subtraction angiography ( dsa ) confirmed the findings on mri [ figure 2 ] . as there was uncertainty about visual recovery he did not opt for any treatment and was discharged against medical advice . mri of brain ( a ) t1w , axial section showing a rounded suprasellar lesion which is composed of alternate concentric rings of iso and hyperintensities ( b ) t2w , coronal section showing suprasellar lesion with hypointense rim and center of heterogeneous intensities ( c ) t2w , saggital section showing suprasellar lesion of mixed intensities ( d ) t1w , after contrast administration saggital section showing suprasellar lesion with brillian enhancement in center in continuity with anterior cerebral artery ( a ) dsa , right carotid injection , antero - posterior view , showing an aneurysm arising from anterior communicating artery directed inferiorly ( b ) dsa , lateral view , showing an aneurysm arising from anterior communicating artery directed posteriorly large or giant aneurysm arising from anterior part of circle of willis can result in visual symptoms as it is in close proximity to anterior visual pathways . the visual loss due to aneurysms is characterized by fluctuation , unlike that from tumors where visual loss is gradually progressive . the common origins of aneurysms that present with symptoms of anterior visual pathway compression are carotid - opthalmic artery , supraclinoid internal carotid artery , and internal carotid artery bifurcation . aneurysms arising from these arteries are often large or giant and can compress the visual apparatus . the acoma aneurysms rupture before becoming large enough to compress visual pathways , hence they present with subarachnoid hemorrhage rather than visual symptoms . visual apparatus compression can occur from giant acoma aneurysm directed posteriorly and inferiorly as in our case . presentation with only monocular acute visual loss is very rare , and when it occurs , contralateral eye is more often affected . the other patterns of visual loss due to acoma aneurysms are central scotoma , bilateral heteronymous deficits , monocular or binocular inferior field loss , asymmetrical bitemporal hemianopsia , and incongruous homonymous hemianopsia . in our case , the opposite side temporal field defect was detected only on detailed visual field assessment , for which patient was asymptomatic . the visual field deficit might have started with bitemporal hemianopsia due to chiasm compression , which would have not been noticed by the patient . the right optic nerve would have got compressed later , presenting with monocular visual loss . the common mechanism of visual loss is compression and ischemia due to giant aneurysm , as in our case , and direct hemorrhage in the optic nerve or chiasm due to rupture of smaller aneurysm . the penetration , splitting , or fenestration of optic nerves by aneurysms is extremely rare . the predictability of recovery of vision is poor in case of aneurysmal visual loss . in a review of 165 cases of aneurysmal visual loss , visual function improved in 70% cases , remained unchanged in 21% , and worsened in 9% after surgical clipping . there was no significant relationship between visual recovery and aneurysm location , aneurysm size , or presence of subarachnoid hemorrhage . as the numbers of cases of visual loss due to acoma are rare the exact figure of visual outcome is not available . a patient with only a direct compression of the nerve by the aneurysm with no bleed can be expected to improve after surgery .

anterior communicating artery aneurysm rarely presents with symptoms of compression of anterior visual pathways . we report a case of 65 years old man , who had complete loss of vision in right eye and temporal hemianopsia in left eye due to giant anterior communicating artery aneurysm .

the following case report highlights a potentially life threatening complication of undertaking an incision biopsy on a soft tissue tumour , namely surgically uncontrollable haemorrhage from an unsuspected vascular lake . the case report demonstrates the value of interventional radiology for acute bleeding and radiotherapy for more chronic tumour bleeding . a 75 year old male presented with a 3 month history of painful progressive left buttock swelling and non - productive cough . past medical history included carcinoma of the prostate 5 years previously , which had been treated with surgery and radiotherapy . examination revealed a fixed 10 by 10 cm mass in the left buttock extending over the sacro - iliac joint . a pleural aspiration cytology confirmed a malignant effusion , although did not differentiate the cell type . the pelvic mri scan demonstrated a large 10 x 10 x 5 cm heterogeneous lesion in the left buttock , occupying much of the posterior portion of the gluteus maximus and invading the iliac bone and sacrum . the primary lesion was not operable and the presence of the malignant effusion denoted stage 4 disease , ( t2 , n0 , m1 ) . percutaneous needle core biopsy of the lesion was inconclusive and incision biopsy was requested after drainage and talc pleurodesis of the pleural effusion . at the time of incision biopsy the capsule of the lesion this resulted in profuse bleeding from a deep , fresh blood filled , cavity within the cystic tumour . a large vascular lake in the centre of the tumour was evident with a significant vascular flow rate . the cavity was therefore tightly packed with haemostatic material and firm pressure applied to the wound , causing partial cessation of the haemorrhage . full surgical control of the bleeding was not possible due to the fact that the tumour was in - operable and the feeding vessels originated from the internal iliac vessels running through the invaded bone of the ileum . angiography revealed a tumour blush in the gluteal region supplied by the median sacral artery and the posterior division of the internal iliac artery ( iia ) . the iia was catheterised revealing multiple feeding branches with extravasation from one ( figure 1 ) . angiograms pre - embolisation with histo - acryl angiograms post - embolisation with histo - acryl over the ensuing 3 weeks , despite the initial successful embolisation the patient haemorrhaged on a further 2 occasions despite pressure dressings to the wound area . on both of these occasions on each occasion , a different set of feeding vessels was noted to be bleeding and was embolised . histology from the incision biopsy revealed a diagnosis of epithelioid angiosarcoma ( figure 2 ) which is known to be associated with the development of vascular lakes . ( cd31 immunohistochemistry : immunoperoxidase , original magnification x100 ) to prevent the occurrence of further haemorrhage , the patient was given a course of radiotherapy ( 30gy in 10 treatments over 14 days ) . palliative chemotherapy to control respiratory symptoms from the pleural effusion was declined and the patient died 6 months after initial presentation . the epithelioid phenotype of angiosarcoma was first described in 1976 by rosai and colleagues who documented cases of cutaneous angiosarcomas . it was later documented by fletcher and colleagues in 1991 who described deeper lesions found in the soft tissues where they are now most commonly found . ( 1 ) epithelioid angiosarcomas are rare , high - grade , malignant , vascular tumours , which have an aggressive course and tend to recur locally , spread widely , and have a high rate of lymph node and systemic metastases.(2 ) it often presents in middle - aged and elderly males with a peak incidence in the seventh decade of life . prognosis for these types of tumours is very poor with death usually occurring from 6 months to 2 years after presentation . epithelioid angiosarcomas have been described arising in various soft tissues around the body and are most often metastatic in origin . histologically these tumours consist of solid and infiltrative sheets of epithelioid cells characterised by large , oval to round cells with abundant eosinophilic cytoplasm , vesicular nucleus and eosinophilic nucleolus.(3 ) in most cases there are vascular channels or cystically dilated spaces . vascular lakes are therefore a common finding associated with these types of sarcoma . a distinctive starry - sky histological pattern has also been reported in epithelioid angiosarcomas . immunohistochemically the neoplastic cells show positivity for endothelial cell markers such as cd31 , cd34 , factor viii - related antigen , and vimentin.(4 - 5 ) due to cytokeratin expression and epithelioid growth pattern these tumours can occasionally be misdiagnosed as carcinomas . endothelial differentiation can be confirmed ultrastructually by the presence of weibel - palade bodies , intermediate filaments and basal lamina . intracytoplasmic vacuoles containing red blood cells are often present in some cells.(4 ) irradiation of soft tissues for the treatment of various primary cancers is well documented as a cause for several types of sarcoma . as in this case , the patient had undergone a course of radiotherapy for prostate cancer several years prior to presenting with the pelvic sarcoma and this therefore may have been the causative factor . other risk factors for the development of angiosarcomas include carcinogens , chronic lymphoedema and hormonal status . however one such case documented a lesion of the mons pubis 4 years following radiotherapy for vulval carcinoma.(5 ) angiography and tumour embolisation is a well established treatment of tumours both pre- and post - operativley . tumour embolisation may be carried out for acute bleeding from tumour vessels , and to reduce the blood supply to tumours prior to surgery . treatment usually consists of delivering one or two fractions of 5 - 8 gy each on successive days .

epithelioid angiosarcoma is a rare , highly malignant tumour with a poor prognosis . we present the case of a 75 year old man who underwent an incision biopsy to diagnose the soft tissue tumour and suffered from surgically uncontrollable haemorrhage . the case report demonstrates the value of interventional radiology for acute bleeding and radiotherapy for more chronic tumour bleeding .

disulfiram is an aldehyde dehydrogenase inhibitor which is useful in maintaining abstinence from alcohol in patients of alcohol dependence syndrome . it inhibits the enzyme called aldehyde dehydrogenase , probably after conversion into the active metabolite . in addition to the physical symptoms associated with the concomitant use of alcohol , disulfiram may lead to adverse drug reactions when used alone , which are flushing , throbbing headache , perspiration , etc . disulfiram has been used as an aversion technique in chronic alcoholics who have been motivated and sincerely desire to leave the habit . according to de melo and lopes , if patient has a family history of psychosis , disulfiram - induced psychosis is more likely due to genetic background . however , because of its adverse drug reactions and its dangerous effects with alcohol , it has to be prescribed with caution and always monitored by medical supervision . a 35-year - old male patient dependent on alcohol for 10 years is currently abstinent from alcohol since 20 days . he was detoxified in the hospital for alcohol dependence and was instituted with disulfiram 250 mg twice daily for initial 5 days then once daily for 10 days . while patient was in home , he was completely abstinent from alcohol as confirmed by his mother and also investigation of serum gamma - glutamyl transferase levels were also 15 iu / l suggestive of no recent intake of alcohol . patient 's mother reported that the patient had one episode of loss of consciousness with the movement of limbs suggestive of generalised tonic - clonic seizures . no abnormalities were found on physical examination and neurological examination . computed tomography scan of the brain revealed no abnormality and electroencephalography ( eeg ) revealed generalized spike wave activity during photic stimulation . investigations such as complete blood count , urea , creatinine , serum electrolytes - sodium , potassium , chloride , hepatic transaminases , blood sugar , urinanalysis , thyroid function , and uric acid levels were all within normal limits ruling out other causes of seizures . disulfiram ( tetraethylthiuram disulfide ) is a quaternary ammonium compound , it has been used for more than 50 years as an aversion therapeutic agent in the treatment of alcohol dependence . it is used to manage the impulsive drive to drink in patients with alcohol dependence , and it is recognized for its physical and psychological effects . other significant action of disulfiram is its inhibition of dopamine--hydroxylase causing an increase in the concentration of the dopamine in the mesolimbic system . in addition to the physical symptoms associated with concomitant use of alcohol , disulfiram may lead to adverse reactions when used alone , including psychosis . however , it is estimated that 2575% of patients under disulfiram treatment have concomitant alcohol consumption , and produces sensitivity to alcohol , leading to an unpleasant reaction called ethanol - disulfiram reaction ( aldehyde syndrome ) , caused by the accumulation of acetaldehyde through the inhibition of the enzyme acetaldehyde dehydrogenase with histamine release . on eeg patient showed photoparoxysomal response with spike wave activity but no other epileptic form discharges ; however , patient did not have any history of past or family history of epilepsy . basal ganglia are one of the major targets of disulfiram neurotoxicity which may involve carbon disulfide ( cs2 ) metabolite . the most important toxic metabolites are diethyldithiocarbamate ( ddc ) and its metabolite cs2 . ddc chelates copper , thus impairing the activity of dopamine betahydroxylase , an enzyme that catalyzes the metabolism of dopamine to norepinephrine . in this way , ddc causes depletion of presynaptic norepinephrine and accumulation of dopamine , so increasing the levels of dopamine and reducing those of norepinephrine in the mesolimbic region . disulfiram and ddc increase the release of glutamate from striato - cortical synaptic vesicles , both in vitro and in rats , suggesting yet another possible mechanism for ddc - mediated neuronal damage . acute exposure to cs2 causes rapid onset of headache , confusion , nausea , hallucinations , delirium , seizures , coma , and potentially death . many case studies on disulfiram have been reported , and only one study reported disulfiram causing seizures with convulsions . michael a peszke reported a case of seizures with convulsions in a patient on disulfiram therapy , who was abstinent from alcohol over a period of 6 months . then seizures occurred in a context of clinical delirium over a 56 weeks period of time . liddon and satran , in a study of 52 patients described to have disulfiram psychosis , actually only five were , in fact , psychotic and the others had toxic delirium but not seizures and convulsions . apart from disulfiram alcoholic reaction , evidences suggest that disulfiram when used alone can cause various effects and adverse effects including seizures , which are rare but needs to be considered . this would open up areas for further research on mechanisms by which disulfiram causes seizures with convulsions and about prevention .

disulfiram is the aversive therapeutic agent which has been used to treat alcohol dependence more than 50 years . it causes the complications like neurological toxicity , postural hypotension , circulatory collapse , mental confusion , etc . the aim of our study was to report a rare case of disulfiram - induced seizures in a patient of alcohol dependence syndrome . this case study is about a 35-year - old male patient who had one episode of seizures during treatment with disulfiram .

second line antitubercular drug ethionamide is 2-ethylpyridine-4-carbothioamide , a congener of thioisonicotinamide . the drug is a prodrug and mycobacterial etha a , a nicotinamide adenine dinucleotide phosphate - oxidase ( nadph ) -specific , flavin adenine dinucleotide ( fad)-containing monooxygenase , converts it to a sulfoxide , and then to 2-ethyl-4-aminopyridine . ethionamide inhibits mycobacterial growth by inhibiting the activity of the inha gene product , the enoyl- acyl - carrier - protein ( acp ) reductase of fatty acid synthase ii . ethionamide is a part of the world health organization ( who ) regime for multidrug resistant tuberculosis ( mdr - tb ) along with ethambutol , prothionamide , ofloxacin , pyrazinamide , aminoglycoside and capreomycin for six months followed by ethambutol , ethionamide , prothionamide , ofloxacin and pyrazinamide for further 12 to 18 months . the most common adverse effects associated with this drug are anorexia , nausea and vomiting , gastric irritation and a variety of neurologic symptoms . severe postural hypotension , mental depression , drowsiness and asthenia are common and convulsions and peripheral neuropathy are rare . most standard text books and reference books of pharmacotherapeutics have not mentioned about it , and to the best of our search , we could not find any case reports available from a search made in pubmed . in this case report , we have presented the case of unilateral painful gynecomastia , which on the who causality assessment scale is probably / likely associated with the use of ethionamide . a 43 year old male patient presented to the department of pulmonary medicine of dr . r. p. government medical college , kangra in the month of august 2010 with a history of consumption of category i antituberculosis treatment from 19.12.2008 . this was followed by category ii antituberculosis treatment from 10.03.2009 , as category i failed . the drug treatment failed again and the patient was referred to department of pulmonary medicine . culture and sensitivity testing for mycobacterium tuberculosis revealed resistance to streptomycin , isoniazid , rifampicin and ethambutol . pretreatment evaluation was done and patient was put on injection kanamycin 750 mg intramuscular ( i m ) daily 6 days a week , tablet levofloxacin 750 mg once daily , tablet ethionamide 250 mg twice daily , capsule terizidone 250 mg twice daily , gr . pas 10 g once at bed time , tablet pyrazinamide 1500 mg once daily and tablet pyridoxine 100 mg once at bed time from 28.10.2010 . at the end of one month of treatment , patient became sputum smear and culture negative . on 24.02.2011 , the patient complained of pain in the right breast due to friction by clothes since one week , and noticed a swelling of the nipple which was tender on palpation . physical examination revealed a 3 3 cm tender nodule behind the right nipple that was not fixed to underlying tissues [ figure 1 ] . ultrasonography ( usg ) revealed a hypoechoic mass of size 2.5 0.92 2.6 cm in the right breast region behind the nipple , without any infiltration to the deeper structures . patient was advised diclofenac sodium 50 mg once daily for 5 days and for another 5 days if required ; without any change in the antituberculosis regime . patient was asked to report if the pain did not subside . on the next follow up , the patient then stopped the treatment on his own on completion of one year of therapy without doctor 's consent and informing the doctor . within a week of stopping the treatment he reported that the nodule had also disappeared . the term comes from the greek word gyn ( stem gynaik ) meaning woman and masts meaning breast . breast prominence can result from hypertrophy of breast tissue , chest adipose tissue ( fat ) or skin , and is usually a combination of all . the causes of gynecomastia remain uncertain , although it has generally been attributed to an imbalance of sex hormones or lack of tissue responsiveness to them . drug - induced gynecomastia is common and might account for up to a quarter of all cases . most of these have only been implicated by means of case reports , which document a temporal association of the offending drug and occurrence of gynecomastia . the mechanisms by which many of these drugs induce gynecomastia are not yet understood ; however , an imbalance between estrogen and testosterone has been suggested . amongst the antituberculosis drugs , isoniazid was the first found to be causing gynecomastia . in 1953 , a report from france implicated this drug as a cause of gynecomastia . another report also described painless , bilateral , gynecomastia in a 52 year old man who was receiving 600 mg of isoniazid daily ( 10 mg / kg / day ) for four months . the authors hypothesized that a disturbance in vitamin b6 complex activation in liver could have caused an alteration in estrogen - androgen metabolism . it has also been postulated that isoniazid may act by means of a re - feeding mechanism in men with tuberculosis . after isoniazid , thiacetazone has been suggested as a cause of gynecomastia in a single report . a causality assessment using the who uppsala monitoring centre scale showed that the present adverse reaction is probably / likely associated with the use of ethionamide . similar reports are not available in published literature and an awareness of this possible adverse drug reaction due to ethionamide might guide the prescribing doctors in their choice of the antitubercular drugs .

a 43 year old male patient , known case of multidrug resistant tuberculosis , was prescribed antitubercular drugs : kanamycin , levofloxacin , ethionamide , terizidone , para - aminosalicylate sodium ( pas ) , pyrazinamide and pyridoxine . after 4 months of treatment , the patient developed a lump in the right breast which was approximately around 3 3 cm in size , tender on palpation , and not fixed to the underlying tissues . ultrasonography ( usg ) revealed a hypoechoic mass of size 2.5 0.92 2.6 cm in the right breast region behind the nipple without any infiltration to the deeper structures . gynecomastia due to ethionamide was suspected and the patient was advised anti - inflammatory drugs for 5 days without any change in drug therapy . the pain subsided ; however , the nodule remained . treatment was continued without any change till the patient stopped using the drugs on his own and without doctor 's consent . within a week of stopping of treatment the nodule also disappeared .

complete androgen insensitivity syndrome ( cais ) , or testicular feminization , is a rare x - linked recessive disease characterized by variable defects in virilization of individuals with male karyotype ( 46,xy ) and an absence of sex chromatin . cais is caused by mutations in the androgen receptor gene , resulting in impaired embryonic sex differentiation and producing a female external phenotype . although their risk of malignancy is extremely low , malignant transformation of dysgenetic male gonads ( undescended testes ) has shown a significant association with aging . we describe here a 58-year - old patient , never previously diagnosed with cais , who presented with a large , late stage malignant seminoma as the primary presentation of cais . a 58-year - old woman was referred from a primary clinical to the department of general surgery at asan medical center ( seoul , korea ) for an inguinal hernia . she had noticed reducible swellings on both inguinal areas for 10 years but did nothing until the swellings became problematic . ultrasound examination showed a well - defined cyst in the right inguinal area , and a mass with cystic and solid portions in the left inguinal area . computed tomography showed a 7.4 cm round homogeneously enhanced mass in the left inguinal canal with an enlarged lymph node in the left para - aortic area ( figure 1 ) . the mass excised from the left inguinal area was well - defined and firm , measuring 776 cm in size . the cut surface was homogenously creamy white and vaguely lobulated with focal hemorrhage ( figure 2a ) . the mass in the right inguinal canal was heterogeneously golden yellow to pinkish gray and encapsulated ( figure 2b ) . microscopically , the mass of left inguinal area consisted of large uniform cells growing in broad sheet divided by thin septa which infiltrated by lymphocytes . the nuclei of the tumor cells were centrally located and round to polygonal with finely granular chromatin and prominent nucleoli . the cytoplasm was eosinophilic to clear and had distinct cell border ( figure 3a ) . adjacent to the mass was soft tissue composed of atrophic hyalinized seminiferous tubules and interspersed leydig cell nests in between , compatible with testicular tissue ( figure 3b , 3c ) . the mass from right inguinal canal also had testicular tissue with atrophic hyalinized seminiferous tubules , but the leydig cell nests were larger and more abundant , consistent with leydig cell hyperplasia ( figure 3d ) . this mass was removed surgically , as was the reducible mass in the right inguinal canal , followed by herniorrhaphy . postoperatively , the surgeon in charge requested a detailed examination of this patient by a gynecologist for further evaluation and treatment . the patient was amenorrheic for her entire life , is unmarried , and a virgin . physical examination showed that her breasts were normally developed , but her pubic hair and axillary hair were sparse . she had slightly enlarged external genitalia with a blindly - ending vagina about 2 cm long . chromosomal analysis showed a 46,xy karyotype , but none of her previous gynecological examinations had diagnosed any distinct features . her serum testosterone concentration was 0.25 ng / ml ( normal male range , 2.6 - 15.9 ng / ml ) and her estradiol concentration was 12.1 pg / ml ( normal male range , 0 - 44 pg / ml ) . based on these findings , the patient was diagnosed with ais . because computed tomography suggested metastasis along the left para - aortic lymph node , adjuvant radiotherapy and chemotherapy cais , originally called complete testicular feminization , is a phenotype resulting from a defect in androgen receptor function and causes peripheral androgen resistance . since the androgen receptor is encoded by a gene located on chromosome xq11 - 12 , cais is an x - linked recessive disease . it is characterized as a male karyotype with a normal female phenotype , such as female breast development , although there is little or no axillary and pubic hair . the external genitalia appear normal or slightly underdeveloped and the vagina is of varying length , but is usually shortened and with a blind ending . internal genitalia , including the cervix and uterus , are absent except for undescended testes in the abdominal cavity or inguinal or labia majora along the normal course of testicular descent . these patients have a plasma testosterone concentration within the normal range in males . however , some patients have elevated plasma testosterone due to increased stimulation by luteinizing hormone . in contrast , our patient had a decreased testosterone level , probably due to her completely atrophied testes . the prevalence of cais in the general population ranges between 1 in 20,000 and 1 in 60,000 in females . it is the third most frequent cause of primary amenorrhea and is the most common form of male pseudohermaphroditism . fortunately , cais is usually diagnosed at puberty after an individual presents with primary amenorrhea or an inguinal hernia . a few retrospective studies have estimated that 0.8% to 2.4% of girls with inguinal hernias have cais . lack of awareness of the association between inguinal hernias in girls and cais has often led to failure to make an early diagnosis , particularly in the past . for example , 3.6% of 25-year - old and 33% of 50-year - old ais patients are likely to have tumors . tumors in these patients are due to undescended testes , accounting for 10% of patients with testicular tumors . the two most frequent types of testicular tumors associated with ais are sertoli cell and germ cell tumors . several long - term follow - up studies have shown that the timing of gonadectomy in women with cais can be delayed until sexual maturation is complete , because patients with cais have a normal pubertal growth spurt and feminize at the time of expected puberty , and usually do not develop malignant tumors until puberty . therefore , prophylactic gonadectomy after puberty is recommended . routine ultrasound examination is recommended to monitor potential malignant changes in the gonads of these patients . we describe here an older woman with advanced seminoma who presented with a bilateral inguinal hernia and was first diagnosed with cais . the diagnosis of cais was delayed in this patient , and she failed to receive appropriate care , resulting in the development of a seminoma and its progression to an advanced stage .

a 58-year - old woman who presented with inguinal hernia for the first time was diagnosed as seminoma and complete androgen insensitivity syndrome ( cais ) . the patient received a late diagnosis , and therefore she could not take a proper management . cais is a rare x - linked recessive disease with an xy karyotype that is caused by androgen receptor defects . it usually present with primary amenorrhea or inguinal hernia . the risk of malignant transformation of undescended testis increases with age , thus gonadectomy should be performed after puberty . we present a case of large advanced seminoma in a woman with cais who was neglected and diagnosed lately .

these include isolates from disease outbreaks in africa , europe , the caribbean , and , more recently , from the russian / trans - caucasian epizootic and attenuated variants . in brief , swine red bone marrow cell culture was used for asfv isolate amplification , and swine anti - asfv serum and erythrocytes were subsequently added to the culture . asfv isolates for which the hemadsorption phenomenon was inhibited by serum belonging to the same group within serogroups 18 were clustered into a homologous serogroup . only asfv hemadsorbing strains could be analyzed by this method , and some hemadsorbing asfv isolates could not be placed into existing serogroups because hai was not observed with available reference serum ( 8,9 ) . asfv isolates from the depository at vniivvim were also classified by using a standard asfv genotyping protocol previously published by bastos et al . , the variable part of the p72 ( b646l ) gene was amplified by conventional pcr , and the amplicons were directly sequenced by using a 3130xl genetic analyzer ( applied biosystems , foster city , ca , usa ) according to manufacturer s recommendations . sequences determined at vniivvim were aligned with other publically available asfv sequences and analyzed by using minimum evolution ; a rooted tree was constructed with mega 5.0 software ( 14 ) and edited with figtree v1.4 ( http://tree.bio.ed.ac.uk/ ) ( figure 1 ) . phylogenetic tree of african swine fever virus ( asfv ) isolates maintained in a collection at the national research institute for veterinary virology and microbiology in okrov , russia ; the variable part of b646l gene relative to the 22 known p72 genotypes ( labeled i - xxii ) was used for analysis . the tree was reconstructed by using the minimum evolution method with 1,000 replicates . * the isolates were selected from the african swine fever virus ( asfv ) collection at the national research institute for veterinary virology and microbiology ( vniivvim ) in okrov , russia . asfv genotypes are assigned according to p72 ( b646l ) nucleotide sequencing and phylogenetic tree reconstruction . serogroups are defined on the basis of results of hemadsorption inhibition assay ( hai ) with reference serum ( serogroups 18 ) . democratic republic of congo ; , not applicable ; nk , not known ; nd , not defined . if a country name changed since the virus was isolated , the current name of the country is shown in parentheses . isolates bartlett , magadi , and davis could not be placed into an existing serogroup because no hai was observed with available reference serum , so exact serogroups for these isolates are not defined . isolate spenser does not belong to any of the 22 known genotypes and remains untyped . newly identified asfv genotypes and known serogroups were mapped together so their geographic distribution in africa and europe ( including the european part of the russian federation ) could be visualized . the results ( figure 2 ) show that genotypic and serogroup diversity are greatest in a relatively limited area , mainly in southeastern africa . in contrast , non - asfv endemic countries , where asf outbreaks were caused by asfv of a single genotype , exhibited low or no serogroup diversity . world distribution of african swine fever virus ( asfv ) isolates maintained in a collection at the national research institute for veterinary virology and microbiology in okrov , russia . results of p72 genotyping and hemadsorption inhibition assay of asfv isolates are summarized on the map . genotype ii of asfv isolates from lithuania , latvia , estonia , poland , and belarus was identified by cisa - inia ( animal health research center ; european union reference laboratory for african swine fever ) . asfv isolate o-77 , which was isolated in 1977 from odessa , ukraine ( at the time , part of the union of soviet socialist republics ) , was used in this study . on the basis of cisa - inia results , currently circulating isolates in ukraine belong to genotype ii . the oval with a 1 inside indicates switzerland ; the oval with a 2 inside indicates so tome and principe . country names are presented as 2-letter country codes as designated by the international organization for standardization country codes ( iso 3166 , http://www.iso.org/iso/country_codes.htm ) . single genotype clades of asfv were observed to contain viruses of multiple serogroups ( table ) . for example , asfv isolates belonging to serogroups 1 , 2 , and 4 were specifically clustered within genotype i , and did not group with other genotypes . we also found several serogroups of asf viruses within genotype x. the asfv isolates tsp80 ( serogroup 5 ) and ts-7 ( serogroup 6 ) were subsequently isolated from 1 field sample derived from a naturally infected pig in tanzania . of note , 1 serogroup 2 isolate ( spenser ) demonstrated a novel genotype within the p72 phylogenetic tree and relative to other serogroup 2 viruses ( figure 1 ) , indicating that the p72 genotype , in addition to lacking serotype resolution , has potential to be incongruous relative to serogroup . together , these data indicate that the antigenic heterogeneity of asfv strains is not fully captured by using the standard genotyping approach . the virus elements responsible for protective and cross - protective immune responses are not well known . given the structural and genetic complexity of asfv , it is likely that genes encoding different antigens will be more suited for virus typing . substantial genetic variability can exist between strains and predominate in specific genomic regions , and it is these regions that may provide improved targets for genotyping . our findings support that of a previous study that showed that geographic areas with asfv of high genotypic and serotypic diversity are located in countries where multiple mechanisms of asf transmission ( mixed sylvatic and domestic cycle ) are established ( 11 ) . hai serology provides a measure of asfv typing that , compared with p72 genotyping , better discriminates biologically pertinent phenotypes . viruses belonging to one p72 genotype may be serotypically heterogeneous : strains that are closely related genetically , even from a single isolate , may have different phenotypes and form homologous serogroups . our assessment of asfv genotyping relating to hai serotyping shows the serologic diversity within a p72 genotype . our results highlight the potential for using serogroup classification to understand issues of homologous cross - protection among asfv isolates and virus determinants that influence disease emergence . the key finding from our study is that p72 asfv phylogenetic analysis fails to accurately define asfv hai serogroups . thus , conventional asfv genotyping can not discriminate between viruses of different virulence or predict the efficacy of a specific asfv vaccine . this finding also suggests that for vaccination - based control of asf , it may be more important to determine serogroups rather than genotypes of asfv isolates .

african swine fever virus ( asfv ) causes highly lethal hemorrhagic disease among pigs , and asfv s extreme antigenic diversity hinders vaccine development . we show that p72 asfv phylogenetic analysis does not accurately define asfv hemadsorption inhibition assay serogroups . thus , conventional asfv genotyping can not discriminate between viruses of different virulence or predict efficacy of a specific asfv vaccine .

testosterone secretion produced by leydig cells leads to vas deferens development arising from nephric duct ( wolffian duct ) between eighth and twelfth gestational week . the appendix epididymis is the remnant of cranial nephric duct and a portion of nephric duct that is contiguous with the testis develops to epididymis . congenital anomalies of accessory structures of testis are commonly combined with anomalies of testicular descent into the inguinal canal or sexual maldevelopment , albeit , some cases of isolated anomalies or as a part of some syndromes have been reported ( 1 ) . epididymal anomalies ( i.e. , fused caput medusa , epididymal tail , elongated epididymis , and epididymal atresia ) are seen in 35%-75% of patients with cryptorchidism . congenital absence of the vas deferens ( cavd ) commonly is secondary to cystic fibrosis transmembrane conductance regulator ( cftr ) gene mutation . two types of cavds are : congenital bilateral absence of the vas deferens ( cbavd ) , usually because of cftr gene mutation ; and congenital unilateral absence of the vas deferens ( cuavd ) that is commonly concomitant with ipsilateral anomalies of kidney and seminal vesicle . cuavd is commonly secondary to different disorders of mesonephric duct morphogenesis before the 7th gestational week ( 1 ) . isolated epididymal agenesis have been reported only in animals , rams and bulls , and there is not any report of this condition in human , yet . a 32 year - old patient without any history of previous surgery or drug use with 3 years of infertility got surveyed . at physical examination , secondary sex characteristics were completely developed and external genitalia ( i.e. , penis , meatus , and scrotum ) were normal . both testes were located in the scrotum with normal size and shape and related vasa deferentia were normal . prostatic volume was about 20cc , symmetric , and soft at digital rectal examination ( dre ) . semen analysis revealed normal semen volume and normal range of semen fructose , but azoospermia . based on clinical examinations , imaging and laboratory studies , we decided to operate the patient and vasoepididymostomy was our selected surgery . a longitudinal incision in scrotal midline raphe made a good exposure of both testes and tissues inside both hemiscrotum . there was no evidence of epididymis in any hemiscrotum and vas deferens was directly attached to the testis ( figures 1 and 2 ) . the vasoepididymostomy was not possible , thus , biopsy of both testes was performed for pathological assessments and freezing for future artificial reproductive surgeries . because the epididymis , vas deferens , rete testis and seminal vesicle , altogether are developed from wolffian ducts ( 1 ) , therefore , epididymal anomalies are typically seen concomitant with other anomalies in organs that are developed from wolffian ducts . any epididymal agenesis in animals in previous case reports was unilateral ( 2 , 3 ) , but our patient had bilateral epididymal agenesis . along with fetal development , the effect of testosterone on wolffian duct leads to development of epididymis , vas deferens , and other organs that are related to wolffian duct . sometimes in disorders of sex development , evidences of incorrect or imperfect effect of testosterone on sex organs are seen , but isolated lack of epididymis has not been reported yet . because of isolated epididymal agenesis with normal development of testes and vasa deferentia in our patient , this anomaly is probably due to unknown fetal mutation after vasal development . because of normal range of testosterone and normal development of other testosterone dependent organs , it is not reasonable that hormones interfere with this anomaly .

epididymal agenesis is defined as the absence of the epididymis totally or segmentally , unilateral or bilateral , which is secondary to the wolffian duct malformation ( 1 ) . rete testis , epididymis , vas deferens and seminal vesicle are believed to develop from wolffian ducts .

although some asymptomatic or incidental lesions can be followed up clinically and radiologically , surgery is the first line treatment for most of the patients . gross total resection of the tumor is the aim , however , it is not always possible , particularly in astrocytomas or higher grade gliomas due to the absence of a well defined plane between normal and abnormal nervous tissue . occasionally at the end of procedure edema of the spinal cord impedes safe closure of the dura . in this case paper we demonstrate the utility of intraoperative ultrasound imaging during surgery for intramedullary tumors . this modality is particularly helpful for differentiation between real edema of the spinal cord and pseudoswelling caused by the herniation of the spinal cord through dural incision . aloka b - mode ultrasound with 10 mhz probe is used in our institution intraoperatively for localization of the intramedullary lesion , confirmation of gross total tumor excision and recognition of treatable spinal cord transdural herniation as well as its differentiation from spinal cord swelling . we describe the case of a 40-year - old fit and healthy woman who presented with about five months history of progressive paraparesis and a recent right foot drop mccormick grade 3 . magnetic resonance imaging of her spine showed an expansion of the conus medullaris with heterogeneous change of signal on t2 but no contrast enhancement . she initially had an open biopsy in another neurosurgical unit that did not confirm any histological abnormality . the size of the abnormality increased in three months as per further follow - up imaging . based on radiological findings , the impression was of a low grade intramedullary tumor and the patient was offered surgery for debulking / excision of tumor . a revisional surgery with extension of laminectomy and durotomy was performed to expose the spinal cord . the cord was split in the midline in classical fashion to expose a greyish tumor . initially a good plane separating the tumor from the nervous tissue was seen but progressive loss of tumor margins were noted towards the deeper aspects . intraoperative specimens were sent for pathological consultation confirmed presence of abnormal tissue but were unable to comment neither regarding the histological differentiation between astrocytoma / ependymoma nor the degree of malignancy . assurance of no further neurological deficit was also ascertained through continuous intraoperative neurophysiological monitoring of somatosensory and motor evoked potentials . just prior to the closure of the dural defect , the spinal cord was found to be pushed dorsally and the edges were seen herniating out through the dural defect . the ultrasound probe was used again at this point and a significant collection of cerebrospinal fluid ( csf ) in a pocket anterior to the spinal cord pushing the cord posteriorly was visualized . once this csf was drained , the spinal cord settled within the thecal space and the closure of the dural defect was performed without any complications . the rest of the wound was closed in a routine fashion and the patient made a good postoperative recovery with preservation of preoperative mobility and normal sphincter function . the patient was referred to the oncology service for further treatment . left : preoperative mri image ( t2 weighted images ) of the spinal cord showing a heterogeneous expansile lesion within the conus medullaris . right : postoperative mri image of the same area showing a reduction in the swelling and mass in the area with the tumor cavity being filled with cerebrospinal fluid . left column : intraoperative ultrasound and photograph of the spinal cord showing the tumor causing the expansion of the cord . middle column : pseudo - swelling of spinal cord due to an anterior pocket of trapped cerebrospinal fluid with transdural herniation of the cord post resection of the tumor . the dural margin is very close to the swollen spinal cord preventing a watertight dural closure . right column : relaxed spinal cord after release of the cerebrospinal fluid trapped in the anterior pocket facilitating dural closure the use of intraoperative ultrasound for neurosurgical procedures was described by dohrmann and rubin in 1981 for cranial and in 1982 for spinal pathology . they described the techniques to ensure identification of maximal normal and abnormal anatomy . since then its use has been widely popularized and published extensively mainly due to its effectiveness as a real time imaging tool . surgery for an extensive intramedullary spinal cord tumor can cause a significant amount of swelling and either a duroplasty is required or the dura is left open with meticulous closure of the wound . ultrasound is very helpful to identify pathology anterior to the cord that could be intradural or extradural . in our case there was a pocket of csf anterior to the cord that was pushing it dorsally giving the impression of the cord being swollen . such a case of pseudoswelling of the spinal cord secondary to anterior csf collection has not been reported before to best of our knowledge . the intraoperative ultrasound , which is routinely used in our department for localization of intradural pathology , along with intraoperative neurophysiological monitoring ( somatosensory and motor evoked potentials ) was invaluable . not only it helped us in identifying the intramedullary tumor prior to opening of the dura , but also prevented neurological deterioration and the potential complications associated with duroplasty or leaving the dura open . surgery for an intramedullary spinal cord tumor can cause a significant amount of swelling and either a duroplasty is required or the dura is left open with meticulous closure of the wound . ultrasound is helpful to identify pathology anterior to the cord and this valuable adjunct prevented the potential complications associated with duroplasty or leaving the dura open . the authors do not have any conflicts of interest and have no disclosures to report .

we present the case of a woman who presented with weakness of both legs due to a low grade tumor of the spinal cord . excision of the tumor was performed and confirmed with intraoperative ultrasound . prior to dural closure the spinal cord was found to be pushed dorsally with herniation of the cord through the dural defect . intraoperative ultrasound showed a collection of cerebrospinal fluid in an anterior pocket giving the impression of the cord being swollen . once cerebrospinal fluid was drained , the cord settled within the thecal space and closure of the dural defect was performed . surgery for an intramedullary spinal cord tumor can cause a significant amount of swelling and either a duroplasty is required or the dura is left open with meticulous closure of the wound . ultrasound is helpful to identify pathology anterior to the cord and prevents the potential complications associated with duroplasty or leaving the dura open .

dual malignancy in young adults is a rare entity . of all well - differentiated thyroid carcinoma patients , 5% are familial . majority familial thyroid cancer are nonmedullary familial thyroid cancer , and have been shown to be present in familial cancer syndromes such as familial adenomatous polyposis , cowden syndrome , carney complex , pendred syndrome , and werner syndrome . herein , we report a rare case of a 17-year - old female patient diagnosed with papillary carcinoma of thyroid ( ptc ) with an antecedent history of dysgerminoma of the right ovary . to our knowledge , no association has been reported between dysgerminoma of the ovary with carcinoma thyroid in literature . a 17-year - old female , following right lobectomy of the thyroid gland , with a histopathological report of ptc was referred to the department of surgical oncology at sri aurobindo medical college and post graduate institute in indore , madhya pradesh in india for further management in 2014 . examination of the neck revealed a scar from a previous surgery , with no palpable neck nodes . blood investigations revealed serum t3 1.19 ng / ml ( 0.8 - 2 ng / ml ) , serum t4 of 5.88 ug / dl ( 5.1 - 14.1 ug / dl ) , serum tsh of 6.04 uiu / ml ( 0.27 - 4.20 uiu / ml ) , serum level of lactate dehydrogenase ( ldh ) 157 iu / l ( 140 - 280 previous history revealed hysterectomy with right salpingo - oophorectomy performed 3 years back during evaluation of primary amenorrhea with right ovarian mass [ figure 1 ] . gross examination revealed 1300 g right ovarian mass with fallopian tube with didelphys of rudimentary uterus . microscopy confirmed the diagnosis of dysgerminoma of the right ovary stage i a [ figure 2a ] . the patient had taken chemotherapy bleomycin , etoposide , and cisplatinum ( bep ) regime , i.e. , bleomycin iv per week x9 ; dose at 20 u / m , etoposide 100 mg / m days 1 - 5 q3wk x3 , cisplatin 20 mg / m days 1 - 5 q3wk x3 at a 4 week interval in the adjuvant setting . magnetic resonance imaging ( mri ) of the neck and the pelvis was performed before completion ; thyroidectomy revealed postoperative changes with irregular nodular soft tissue in the region of the right lobe of thyroid with subcentimeter lymph node in bilateral deep upper cervical region [ figure 3a ] and left ovary was normal in appearance , measuring 2.9 cm 4.7 cm [ figure 3b ] . completion thyroidectomy with bilateral central neck node dissection was performed that was negative for residual malignant disease . radioactive iodine scan ( i ) performed 1 year after completion thyroidectomy did not reveal any residual disease . cect abdomen showing mass replacing the right ovary with nonvisualization of the uterus ( a ) microphotograph showing islands of large polygonal tumor cells surrounded by lymphoid cells . the tumor cells possess clear cytoplasm and centrally placed nuclei with vesicular chromatin and prominent nucleoli . ( h&e 400 ) and ( b ) microphotograph showing tumor cells arranged in papillary fronds having vesicular coffee bean nuclei . ( h&e 100 ) ( a ) mri of the neck showing postoperative changes in the neck with irregular nodular soft tissue in the region of the right lobe of thyroid with subcentimeter lymph node in bilateral deep upper cervical region and ( b ) mri of the pelvis showing normal - appearing left ovary with absent right ovary and uterus two or more histologically different malignancies identified within the first 6 months of the first malignancy is defined as synchronous and if the second tumor is identified beyond 6 months , it is defined as metachronous . both the tumors should be malignant and neither should be metastasis of each other and both the tumors should be morphologically and microscopically distinct from each other . in our case , there were two distinct pathologies of dysgerminoma of the ovary and ptc , both anatomically and histologically different entities with a gap of 3 years between both [ figure 2a and b ] . dysgerminomas account for 1 - 5% of all ovarian malignancies in the first two decades of life . of the cases , 80% are reported in females in the second decade of life with complaints of delayed menarche and primary amenorrhea , which were the presenting complaints of our patient as well , 3 years back . whose contralateral ovary has been preserved , some diseases can develop in 5 - 10% of the retained gonads over the next 2 years . the possibility of developing dysgerminoma in the contralateral ovary was ruled out with mri of the pelvis that reported a normal left ovary and sr ldh 157 histopathology report following right lobectomy of the thyroid revealed ptc , following which , completion thyroidectomy with central neck dissection ( cnd ) has been performed . a large retrospective study evaluating second primary malignancy in thyroid cancer patients found second primary malignancy in 0.45% of cases , out of which only 0.067% cases had thyroid cancer as the second primary malignancy and all had a history of exposure to radiation . the association between carcinoma thyroid , cancer of breast , kidney , salivary glands , brain , central nervous system , scrotum , and leukemia has been reported . but association between dysgerminoma and carcinoma thyroid has not been reported in literature to the best of our knowledge . despite a thorough pubmed search , we were unable to find a previous reported case of metachronous papillary carcinoma thyroid following dysgerminoma of the ovary . metachronous malignancy with the index tumor of ovarian cancer has been reported , but the case of index tumor of dysgerminoma of the ovary with a second primary tumor diagnosed as ptc is unusual . the case of dual primary malignancy has not been documented in literature ; we present this case due to the rarity in presentation . sc contributed in the conception of the work , conducting the study , writing and approval of the final version of the manuscript , and agreed for all aspects of the work . smd contributed in the conception of the work and approval of the final version of the manuscript , and agreed for all aspects of the work . dym contributed in revising the draft and approval of the final version of the manuscript , and agreed for all aspects of the work . ss contributed in the conception of the work , revising the draft , and agreed for all aspects of the work .

dual malignancy is rare in adolescents . dual malignancy with the second malignancy of thyroid is rare . no association has been reported between dysgerminoma of ovary and carcinoma thyroid in medical literature . despite a thorough pubmed search ( key words papillary carcinoma of thyroid , metachronous , dysgerminoma ovary ) , we were unable to find a previous reported case of metachronous papillary carcinoma of thyroid ( ptc ) following dysgerminoma of the ovary . after surgery , the patient is being regularly followed up for recurrence / development of new primary . we report this unusual and rare case in a 17-year - old female patient .

retrograde jejuno - gastric intussusception is a rare complication of gastrojejunostomy and billroth ii reconstruction . about 200 cases of jejuno - gastric intussusception have been reported so far , in literature with the first case reported in 1914 by bozzi after gastrojejunostomy . a 42-year - old female presented with acute , severe continuous pain in the upper abdomen for 3 days . it was associated with multiple episodes of coffee colored vomiting and swelling in the left hypochondrium . patient had similar complaints 1-year earlier for which she was hospitalized and was managed conservatively . she had open cholecystectomy 10 years earlier for cholelithiasis and some upper abdominal surgery 20 years ago , details of previous surgery were not available . she was poorly built and anemic . patient had mild tachycardia , but blood pressure , and respiration rate was within normal limits . on abdominal examination , there were two well healed surgical scars , one in the upper midline and another in right the subcostal region . the abdomen was not distended , and there was a diffuse mass about 8 cm 5 cm in the left hypochondruim . abdominal x - ray showed dilated stomach with dilated small bowel loops on the left side of the abdomen . contrast - enhanced computed tomography of the patient showed serrated intragastric mass of about 8.4 cm with intra gastric contrast around it giving a claw sign suggestive of the retrograde intussusception . the coronal view showed efferent jejunal loop with areas of central fat attenuation ( 42 hu ) suggestive of jejunal mesenteric fat along with vessels intussuscepting into stomach and dilated afferent jejunal loop . intragastric lesion due to jejuno - gastric intussusception [ figures 2 - 4 ] . the lumen of the stomach ( s ) is forming crescent around it axial contrast - enhanced computed tomography showing a large intragastric mass with serrated margins and contrast around it leading to claw sign suggestive of retrograde intussusception through the gastrojejunostomy coronal contrast - enhanced computed tomography showing efferent loop with areas of central fat attenuation ( 42 hu ) suggestive of jejunal mesenteric fat along with vessels intussuscepting into stomach ( s ) and dilated afferent jejunal loop ( aff ) sagittal computed tomography section showing a target type intragastric lesion due to jejuno - gastric intussuception at laparotomy , there was retro colic , posterior gastrojejunostomy . the efferent jejunal loop was intussuscepting into the stomach , and afferent jejunal loop was dilated . the efferent loop was edematous , thick walled and viable having circumferential hyperemic ring on its serosa . the efferent loop was sutured to the anterior abdominal wall and transverse mesocolon to prevent recurrence . postoperative course was uneventful . on follow - up after 3 months , patient is asymptomatic and doing well . it is anatomically divided into three types depending on afferent or efferent loop intussusception , type i : afferent loop intussusception , type ii : efferent loop intussusception type iii : both efferent and afferent loop intussusception . type ii is observed in 80% of cases and our case also fits into type ii . it has been suggested to be more common due to greater mobility of efferent loop than afferent loop . various functional and mechanical factors have been implicated in its etiology , but none has been proved . the causes postulated are antiperistalsis with vomiting , adhesions with kinking of bowel , dilatation of bowel following gastrectomy , hyperacidity , jejunitis causing retrograde peristalsis , shortening of the mesentery of the jejunal loop and jejunal stenosis with obstruction facilitating antiperistalsis . jejuno - gastric intussusception have been reported in immediate postoperative period and even 55 years after gastrojejunostomy . initially , vomitus is clear then it becomes coffee colored , and later massive hematemesis occurs . chronically , it presents as repeated episodes of epigastric fullness and vomiting due to intermittent reversible intussusception . in our case , patient probably had one previous episode of intussusception 1-year back which resolved spontaneously , and now it has presented in the acute form . high index of suspicion is required for early diagnosis as mortality increases from 10% to 50% with a delay of 48 h of surgery in acute intussusception . a water - soluble upper gastrointestinal ( gi ) contrast reveals a coiled - spring appearance ( of the jejunal intussusception ) within the stomach . ultrasonography showed intragastric tubular mass with peristalsis and computed tomography ( ct ) scan shows intragastric intussusception . upper gi endoscopy helps in the diagnosis and can be therapeutic in reducing the intussusception . in this case , diagnosis was suspected on ultrasonography and later confirmed by ct scan . although spontaneous reduction has been reported , but surgical intervention is the treatment of choice after the diagnosis is made as it is not possible to predict which patient will undergo spontaneous reduction or progress to gangrene . various surgical options include reduction alone , plicating the mesentery of the jejunum in the area of intussusception , fixing the efferent loop to adjacent tissues such as transverse mesocolon , colon , stomach , and parietal wall , converting to billroth i anastomosis . if a small intestine is gangrenous then resection of gangrenous segment is the only option . although there are reports of endoscopic reduction of intussusception , but it is contraindicated when signs of peritoneal irritation are present . due to a limited view on endoscopy , it is difficult to ascertain the viability of the intussuscepted loop . endocopic reduction was not done in this case as facilities of endoscopic reduction are not available at our hospital . in our case , after reduction of viable efferent loop , it was fixed to transverse mesocolon and anterior parietal wall to prevent recurrence . retrograde jejuno - gastric intussusception is a rare but well known complication after gastrojejunostomy and billroth ii reconstruction .

retrograde jejuno - gastric intussusception is a rare complication following gastric surgery . we present a case of retrograde jejuno - gastric intussusception in a 42-year - old female who presented with upper abdominal pain , vomiting and swelling in left hypochondruim . intussusception was suspected on ultrasound of the abdomen and later confirmed with computed tomography scan . at laparotomy , efferent loop was intussuscepting into stomach . this was reduced and fixed to the abdominal wall and transverse mesocolon . it should be suspected in a patient with the previous history of gastric surgery as it is a rare complication . early diagnosis and management can prevent further complications like bowel gangrene and its associated morbidity and mortality .

during july 2007july 2011 , six patients from the mediterranean coast city of elche , spain , who had high fever and inoculation eschars received a diagnosis of infection with r. sibirica mongolitimonae ( table ) . for laboratory confirmation , dna was extracted from eschars , lymph nodes ( fine - needle aspiration ) , and blood samples by using the qiaamp tissue kit ( qiagen , hilden , germany ) , according to the manufacturer s instructions . for molecular detection , 200400 ng of dna from each sample was subjected to pcr targeting the 23s-5s rrna intergenic spacer , followed by hybridization with specific probes by reverse line blotting , as described ( 15 ) . when using the probe for r. sibirica mongolitimonae , a positive hybridization signal was obtained from eschar samples from all 6 patients ; this result was confirmed by sequencing ( 100% similarity to a reference r. sibirica mongolitimonae strain [ genbank accession no . hq710799 ] in all cases in the 357 bp sequenced ) . to further confirm this result , nested pcr targeting the gene for outer membrane protein a was performed as described ( 15 ) ; these sequences ( 514 bp ) also showed 100% similarity to a reference r. sibirica mongolitimonae strain ( genbank accession no . * na , not available ; ast , aspartate aminotransferase ; alt , alanine aminotransferase . serologic response was analyzed by using an in - house microimmunofluorescence assay for igg and igm , performed as described ( 4 ) ; r. conorii and r. sibirica mongolitimonae were used as antigens , and cutoff values were 1:40 for igg and 1:20 for igm . acute- and convalescent - phase serum samples were obtained from 3 case - patients and single serum samples from the other 3 case - patients . results for samples from 2 case - patients were negative , but results for the remaining 4 samples showed low to medium titers . these results are consistent with previous reports ( 6 ) , in which 30% of cases had a positive igm result and 50% had negative or near - cutoff igg results . all 6 case - patients lived in elche and its surroundings ( 230,112 inhabitants ) . three of the cases occurred during the spring , which is when 10/18 cases reported in the literature occurred ( 414 ) . all 6 case - patients had fever ( 38.5c39.5c ) , myalgia , and headache ; in the cases from the literature , 18/18 patients had fever , 13/18 myalgia , and 11/18 headache . in our study , 1 case - patient was confused and drowsy on arrival at the emergency department . all 6 case - patients had a single inoculation eschar develop : 2 on the neck , 2 on a lower limb ( figure ) , 1 on the scalp , and 1 on an upper limb . five ( 83% ) case - patients had enlarged lymph nodes in the region from which the eschar drained , as reported for 10/18 ( 55% ) cases from the literature . three case - patients ( 50% ) had lymphangitis extending from the eschar to the draining lymph nodes ( figure ) , compared with 6/18 ( 33% ) in cases from the literature . inoculation eschar on popliteal area and discrete maculopapular elements in patient with lymphangitis infected with rickettsia sibirica mongolitimonae , spain , 2011 . for 4/6 ( 67% ) case - patients , a generalized maculopapular rash developed on the palms and soles but not the face ; for 2 case - patients these findings are consistent with our review of the literature , which indicated rash occurring in 13/18 ( 72% ) cases . all 6 case - patients recovered without sequelae after antimicrobial drug treatment using doxycycline or azithromycin . fournier et al . ( 4 ) , who reported 7 cases of r. sibirica mongolitimonae infection in 2005 , proposed the name lymphangitis - associated rickettsiosis for the disease , on the basis of associated clinical features . however , for the case - patients reported here , the most common clinical signs and symptoms were fever and skin eschar , similar to those from previously reported case series ; 5 of the case - patients reported here showed regional lymph node enlargement , 4 rash , and 3 lymphangitis . because only 24 total cases have been reported and other rickettsioses produce lymphadenopathy and lymphangitis , the term lympangitis - associated rickettsiosis may be unwarranted for this disease . in our case series , 1 case - patient had mental confusion after 10 days of a febrile disease before hospitalization and was found to be hyponatremic . in this patient , the eschar was located on the scalp , and neither rash nor other clinical clues were suggestive of rickettsiosis . the patient had increased c - reactive protein plasma levels and the highest serologic antibody titers for r. sibirica mongolitimonae of the 6 case - patients ( table , patient 1 ) . since r. sibirica mongolitimonae was isolated from h. asiaticum ticks in 1991 ( 1 ) , it has been recovered from h. truncatum ticks in sub - saharan africa ( 6 ) and h. anatolicum excavatum ticks in greece ( 7 ) . however , in spain , r. sibirica mongolitimonae has been detected in 2/8 tick species tested ( rhipicephalus pusillus and rh . bursa ) at similar percentages ( 3.7% and 3.6% , respectively ) but not from hyalomma spp . pusillus ticks from portugal was positive for r. sibirica mongolitimonae ( 8) , while testing of other tick species , including h. lusitanicum , rh . ( h. lusitanicum and h. marginatum ) ticks have not been found to be infected in spain does not mean that these ticks are not vectors for r. sibirica mongolitimonae . ticks are a vector for this rickettsia on the iberian peninsula , and our findings confirm that this rickettsia species is circulating in spain , where specific vectors have yet to be described . in summary , we report 6 cases of human infection with r. sibirica mongolitimonae that occurred in the same geographic area of spain . our results indicate that pcr of eschar samples is the most useful diagnostic procedure for this pathogen ; samples from all 6 case - patients had positive results , while test results for 1 whole blood sample and 2 lymph node samples were negative . however , the limited number of samples does not make it possible to infer specific diagnostic sensitivities . the epidemiology and pathogenicity of illness caused by r. sibirica mongolitimonae infection require further investigation . an active search for the vector of r. sibirica mongolitimonae in countries of the mediterranean region is necessary to complete the epidemiology of this rickettsiosis , which is likely to be more widespread than originally assumed . in particular , clinicians caring for patients who have traveled to the mediterranean coast of spain should consider this rickettsiosis in the differential diagnosis .

human infection with rickettsia sibirica mongolitimonae was initially reported in 1996 , and reports of a total of 18 cases have been published . we describe 6 additional cases that occurred in the mediterranean coast region of spain during 20072011 . clinicians should consider this infection in patients who have traveled to this area .

chronic lymphocytic leukaemia ( cll ) , the commonest type of leukaemia , is a generalised malignancy of lymphoid tissue originating in the bone marrow . it is characterised by an accumulation of monoclonal lymphocytes , usually of b cell type . the malignant lymphocytes spread from the bone marrow to blood , lymph nodes and other organs . cll is regarded as small lymphocytic lymphoma when lymph nodes are involved but the blood film and , in particular , the white blood cell count is normal . we report a case of a 71-year - old man who had cll discovered incidentally in the gall bladder wall following laparoscopic cholecystectomy with no history of cll prior to surgery . a 71-year - old man underwent elective laparoscopic cholecystectomy following an episode of gallstone pancreatitis . the gall bladder was received opened with an empty lumen , the presence of gallstones having been noted at the time of surgery . the mucosa was its usual green , velvety appearance and the serosal surface showed shaggy adhesions . the gall bladder was sampled in the standard way , namely with sections from the neck , fundus and head . the sections from the neck and fundus showed acute - on - chronic mucosal inflammation with focal mucosal erosion . histology of the section from the head of the gall bladder showed an acute - on - chronic inflammatory infiltrate within the mucosa along with a dense lymphoid infiltrate in the gall bladder wall ( figure 1 ) . the lymphocytes were small and , other than the monomorphous appearance , were cytologically unremarkable . initial immunohistochemical staining showed that the infiltrate was predominantly b - cell in nature ( cd20 and bcl-2 positive with the t - cell marker cd3 decorating a much smaller population of t - cells dispersed within the infiltrate ) , confirmed on immunohistochemical staining with the b - cell marker cd20 ( figure 2 ) . further analysis by the regional haematological malignancy diagnostic service confirmed the cll phenotype ( cd10 negative , bcl - l negative , bcl-2 positive , cd5 positive , cd20 positive , cd23 positive , cd79 positive , bcl-6 equivocal , cd27 equivocal ) . high power photomicrograph of an immunohistochemical stain for the cd20 antigen decorating small b - lymphocytes . following the incidental finding of cll in the gall bladder wall , the patient underwent further clinical evaluation , which ruled out lymphadenopathy and hepatosplenomegaly . a peripheral blood smear revealed a normal myeloid lineage with small to medium size lymphocytes . computed tomography of the chest , abdomen and pelvis was essentially normal , showing no evidence of hepatosplenomegaly or mediastinal , abdominal and pelvic lymphadenopathy . a bone marrow biopsy was not performed in view of the normal peripheral blood smear and imaging . at haematological review a bone marrow biopsy confirmed marrow infiltration by b - cell chronic lymphocytic leukaemia . in the absence of b symptoms , cll primarily affects adults in later life and is characterised by a proliferation of b - lymphocytes associated with suppression of the immune system , failure of the bone marrow and infiltration of malignant cells into organs . cll originates in the bone marrow and can spread to the blood and organs of the haemopoietic system . in end - stage disease , cll can spread to non - haemopoietic structures such as the liver , lungs , gastrointestinal tract , skin , central nervous system , bone , heart , adrenal glands and kidneys ( 1 ) . unusual cases of early stage cll with infiltration into prostate , pituitary , myocardium , thyroid , lung , and orbit have been reported ( 2,3,4,5 ) . extramedullary involvement is more frequently seen in patients with acute lymphoblastic leukaemia where common sites of infiltration include the central nervous system , spleen , liver , lymph nodes , ovaries and testes . skin involvement ( leukaemia cutis ) and gingival infiltration are seen in approximately 10% of patients with acute myeloid leukaemia ( 6 ) . extramedullary involvement of the gastrointestinal tract is relatively rare and the reported autopsy incidence ranges from 5.7% to 13% and is as high as 20% in cases of acute lymphoblastic leukaemia ( 7 ) . in one of the largest autopsy series , cornes and colleagues evaluated almost 15,000 consecutive autopsies and identified 264 cases of leukaemia . the most frequently involved sites were the stomach , ileum , and proximal colon , whereas the duodenum and distal colon were less frequently affected ( 8) . the only case of gall bladder involvement by cll that has been published to date in the reviewed literature , concerned a 62 year old woman who underwent an emergency cholecystectomy for acute cholecystitis . she had a history of rai stage iv b - cell chronic lymphocytic leukaemia and was in partial remission , having been treated with prednisolone and chlorambucil . histology revealed transmural infiltration of the gall bladder with small lymphoid cells , which on immunohistochemistry were positive for cd20 with aberrant expression of cd5 ( 9 ) . this is the first reported case of gall bladder involvement by cll without a previous diagnosis of cll . this patient was subsequently confirmed to have leukaemic marrow infiltration with cll and remains stable with no b symptoms . this case reinforces the necessity of subjecting all gall bladder specimens for histopathological examination following routine cholecystectomy contrary to recent suggestions by some studies that this is not necessary if the gall bladder is marcoscopically normal ( 10 ) .

extramedullary involvement in early stage chronic lymphocytic leukaemia ( cll ) is rare . we report the first case of an incidental finding of gall bladder infiltration in a patient who underwent a cholecystectomy for gallstone pancreatitis with no preceding history of cll . this case reiterates the importance of subjecting even routine cholecystectomy specimens for histopathology examination in the context of this unusual presentation .

colonic interposition is a procedure practiced for the management of both benign and malignant diseases of the esophagus . the left colon is preferred for the transposition due to its smaller diameter , but right colon or transverse colon interpositions are performed as well . malignant tumor of the transposed colon is a rare long - term complication of the procedure and is related to the irritation of the colonic mucosa by gastric acid content or bile . gastric pull - up and anastomosis of the proximal esophagus via a right thoracotomy ( ivor lewis procedure ) substitutes the resected transposed colon . herein , we present a patient who was diagnosed with interposed colonic carcinoma at the level of the anastomosis to the remnant stomach . in a previous operation in early childhood , the proximal stomach was removed in the index operation , precluding a gastric pull - up procedure . a 59-year - old woman presented to our clinic complaining of difficulties in swallowing both solid food and clear fluids . at the age of 3 , proximal gastrectomy and pyloroplasty were performed due to peptic disease and perforation . after this surgery , she suffered from anastomotic stricture , which was treated with repeated dilatations . due to persistent stricture , a long - segment transverse colon interposition was carried out 1 year later . colon interposition was performed using the ivor lewis approach , with the colon being anastomosed to the proximal esophagus via a right thoracotomy . endoscopy revealed a large , ulcerated lesion obstructing the distal interposed colon . a biopsy specimen taken from the mass revealed adenocarcinoma of a colonic origin . computerized tomography of the chest and abdomen showed the same lesion in the distal interposed colon without distant metastases . a barium swallow revealed an apple core lesion in the intra - abdominal portion of the interposed colon at the level of the anastomosis to the remnant stomach ( fig . , the tumor was found at the level of the anastomosis of the colon to the remnant stomach . the interposed colon was the transverse colon based on its main blood supply the middle colic artery and vein . the colon was anastomosed to the remnant stomach at the level of the gastric antrum , but the remnant gastric antrum was too short for gastric pull - up . considering the surgical anatomy discovered during the procedure , we decided to avoid reinterposition surgery , as this would have resulted in a very short remnant colon length . during surgery , the colon was dissected from the hiatus to enable resection in an adequate distance from the tumor . roux - en - y coloenterostomy and enteroenterostomy were performed , and the patient recovered uneventfully . the tumor measured 3.5 cm in diameter , but all 15 lymph nodes were negative for tumor cells ( t3no ) . no further oncological treatment was offered to the patient , and she was followed up in the outpatient clinic . at the 6-month follow - up , she was in good health without recurrent disease . only scattered case reports describe this clinical entity [ 3 , 4 , 5 ] . the tumor probably develops from long - standing colonic mucosa irritation by gastric juice and bile . with regard to cologastric junction carcinoma , debate raises on whether these tumors should be considered colonic or gastric in nature . as pathology showed that the tumor in our patient was of a colonic origin , we decided to manage it as adenocarcinoma of the colon . the short antral segment found during examination indicated the surgical procedure in our patient : surgical resection of the interposed colon with clear margins . preservation of the middle artery and vein enabled preservation of the short colonic segment anastomosed to the esophagus in a previous surgery . in cases of interposed colonic carcinoma , further oncological treatment is considered based on the tnm classification , and close postoperative follow - up is mandatory . a high index of suspicion for colonic adenocarcinoma is needed in all patients who underwent colonic interposition due to the deleterious effect of long - standing mucosal irritation .

a 59-year - old female with dysphagia presented to our clinic . in childhood , she underwent colonic interposition due to anastomotic stricture after a previous proximal gastrectomy for gastric ulcer perforation . imaging studies revealed a space - occupying lesion obstructing the distal interposed colon . at surgery , completion gastrectomy with segmental colectomy was carried out , and roux - en - y coloenterostomy and enteroenterostomy were performed .

erlotinib is a selective epidermal growth factor receptor inhibitor ( egfri ) utilized in the treatment of solid tumors . cutaneous side effects , including changes in hair texture and alopecia , have been reported . in this case report , we observed a new finding of loose anagen hairs and pili torti in two patients treated with erlotinib . her hair progressively became curlier , brittle , and dull and the overall hair volume decreased . the second patient who was a 60-year - old female with metastatic adenocarcinoma of the lung had been on erlotinib for over a year when she noticed increased hair loss . examination of both revealed a band of nonscarring alopecia along the entire hair margin [ figure 1a and b ] associated with diffuse thinning and eyelash trichomegaly in the second patient . trichoscopy showed irregularly shaped shafts and bending at different angles , numerous black dots and broken hairs and no inflammation [ figure 2a ] . high magnification revealed anagen hairs devoid of sheaths and multiple twisting of flattened shafts through 180 at irregular intervals [ figure 2c and d ] . scalp biopsies , performed in the first patient , revealed normal telogen count of 10% . anagen follicles showed irregular thinning of the outer root sheath ( ors ) and a serrated vitreous layer . several follicles showed features of hair breakage with pigmented casts , increased number of apoptotic cells in the ors , and disintegrated inner root sheath ( irs ) . diagnosis of hair breakage associated with pili torti and loose anchoring of anagen hairs induced by erlotinib was made . topical steroids and minoxidil 5% were empirically prescribed to the second patient , with significant improvement after 3 months [ figure 1c ] . pronounced nonscarring alopecia along the entire hair margin in ( a ) patient one and ( b ) patient two ; ( c ) patient two after 3-month therapy trichoscopic and histopathologic findings in patients treated with erlotinib . trichoscopy shows ( a ) black dots , twisting of hair shafts , and broken hairs at different lengths ( 20 ) ; ( b ) features resembling black rectangular granular structures ( 20 ) ; ( c ) high magnification of a flattened hair shaft reveals multiple twisting ( 250 ) ; ( d ) the pull test results in numerous anagen hairs devoid of sheaths ( 250 ) ; ( e ) scalp biopsy from the alopecic area shows two hair follicles corresponding to the broken hairs on trichoscopy as their hair shafts are replaced by pigmented casts in the hair canal and are surrounded by abnormal , disintegrated inner root sheath , and irregularly thinned outer root sheath . note the absence of sebaceous glands ( h and e , vertical sections , 4 ) ; ( f ) two anagen follicles at the level of subcutaneous fat reveal corrugated and thicker pink vitreous layer ( h and e , horizontal sections , 10 ) reported egfri - induced hair changes include curlier and brittle hair on scalp and extremities , trichomegaly or curling of the eyelashes and eyebrows , and facial hypertrichosis as well as inflammatory and noninflammatory alopecia . in mice harboring a disruption of the epidermal growth factor receptor - allele , hair follicles fail to enter catagen and remain in an aberrant anagen state and display thinning or loss of the irs and ors . irregular atrophy of the distal ors , as observed in our biopsies , has been described as arrow sign . irs abnormalities might be responsible for the loose anchoring of anagen hairs in our patients , as also reported in loose anagen hair syndrome ( lahs ) . another possible similarity to lahs were dermoscopic features resembling black rectangular granular structures , which have been recently associated with this syndrome . hair texture modification and fragility , including pili torti , have also been linked to anomalies in the irs . the absence of sebaceous glands detected is in accordance with previously published data reporting marked disruption of sebaceous gland growth in egfri - treated patients . it is possible that the elastic bands used to fasten the wigs led to increased friction over the scalp rim explaining the pronounced marginal alopecia . 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 .

erlotinib is a selective epidermal growth factor receptor inhibitor utilized in the treatment of solid tumors . cutaneous side effects , including changes in hair texture and alopecia , have been described . in this case report , we describe two patients with a new finding of loose anagen hairs and pili torti leading to nonscarring marginal and diffuse alopecia and discuss potential mechanisms underlying erlotinib - induced hair changes .

transoral vertical ramus osteotomy ( tovro ) is an orthognathic surgical technique used to correct mandibular prognathism . complications commonly reported include extensive bleeding due to major blood vessel injury , unpredictable fracture , postoperative infection , neurosensory deficit related to the inferior alveolar nerve , displacement of proximal segment , and mal - union or non - union of bony segments . it is rare to have bony segment necrosis after tovro [ 13 ] given the bone healing pattern and revascularization of the mandible . there have been no cases of necrosis of bony segments reported since the introduction of perioperative antibiotic use and improved surgical techniques . the authors present a case of an otherwise healthy 37-year - old female patient who underwent lefort i osteotomy and tovro to correct a skeletal deformity , complicated by a partial necrosis of the proximal segment . a 37-year - old female patient who underwent orthognathic surgery was re - admitted with persistent pain , swelling and purulent discharge from her right submandibular region . the patient underwent lefort i osteotomy and tovro by a private practice oral surgeon 10 weeks prior . the post - operative day 2 panoramic radiograph was un - remarkable ( fig . 1 ) . the patient received peri - operative intravenous antibiotics and was discharged home with two weeks of oral antibiotics . the working diagnosis from the history and physical examination was osteomyelitis of right proximal segment of mandible . this was supported by the panoramic radiograph and computed tomography ( fig . 2 , 3 ) that demonstrates radiolucency and destructive bony change at the angle of right mandible with an evidence of sequestering bony segment . the patient was started on intravenous antibiotics ( combination of 3rd generation cephalosporin and metronidazole ) upon admission , and she was taken to the operating room for removal of the sequestered bony segment and a drain placement . intra - operatively , an approximately 15 mm sequestrum at the inferior aspect of the proximal segment was noted and removed . a three month post - operative panoramic radiograph ( fig . necrosis of the proximal segment after tovro is extremely rare . the case reported by tuinzing and greebe does not represent the majority of patients undergoing orthognathic surgery , as their patient s condition was compromised by systemic illness , including diabetes . in 1976 , bell and kennedy studied bone healing and revascularization after pedicled and nonpedicled vertical ramus osteotomy using 15 adult rhesus monkeys . the microangiographic and histologic studies showed osseous union , minimal osteonecrosis , and minimal vascular ischemia when the proximal condylar segment is pedicled to the articular capsule and lateral pterygoid muscle . in contrast , when the proximal segment is not pedicled to soft tissue , there were complications including intraosseous necrosis , vascular ischemia , and delayed healing . bell and kennedy believed that necrosis or non - union of the inferior portion of proximal segments can be prevented by creating the osteotomy more posterior and above the gonial angles . however , this study is based on intraosseous circulation and viability based on microangiographic and histologic evaluation . therefore , it is difficult to predict the extent of proximal segment necrosis in clinical practice . in 1980 , lownie et al . performed l - osteotomy and c - osteotomy on 10 baboons and observed the effects of disrupting the blood supply of the baboon mandible . the lateral pterygoid muscles were left attached , the periosteum on the lateral surface was raised , and a silastic sheet was placed to prevent reattachment of the periosteum . fourteen days post - operatively , the mandibles were removed and examined histologically . surprisingly , avascular necrosis was evident even on the control side . this study contradicts bell and kennedy s conclusion that necrosis of proximal segments can be prevented as long as the lateral pterygoid attachment is maintained . instead , lownie et al . demonstrated the importance of periosteum reattachment as well as temporomandibular joint capsule and lateral pterygoid muscle attachment to prevent avascular necrosis of the bony segments . in addition , vertical ramus osteotomy for correcting mandibular prognathism involves the posteriorly positioned distal segments with bony segments overlap . it is a common technique to detach the muscles of mastication except the temporomandibular joint capsule and lateral pterygoid muscles to ease the reposition of the bony segments . park reported that detachment of those muscles does not affect the normal healing of the proximal segments and anticipated no complication associated with low blood supply . considering all factors , the authors believe that the blood supply to the bony segments is re - established as long as the periosteum reattaches in the setting of surgical detachment of most muscles of mastication . transient ischemia of the bony segments can occur in the initial healing phase , but the revascularization and normal healing process follows unless the transient ischemia is prolonged by damage to the periosteum inhibiting the reattachment , or systemic illness such as diabetes delaying wound healing . the authors report a rare case of a partial osteonecrosis of mandibular proximal segment following a routine tovro procedure . the patient was a healthy individual without any systemic diseases or conditions that could delay wound healing . the operative note describes the surgical technique involving a preservation of temporomandibular joint capsule and lateral pterygoid muscle attachment . the surgeon said that the patient had severe swelling after surgery due to bleeding from the masseteric artery during surgery . there was a large cleft between the end of proximal segment and distal segment in the post - operative day 2 panoramic radiograph ( fig . 1 ) , so that end of the proximal segment may have overstepped the bounds of mucoperiosteum , the etiology of a rare osteonecrosis of the bony segment on this healthy patient after the routine operation is unclear , but we may assume that osteonecrosis can be caused by ischemia due to detachment of periosteum and hematoma formation . consequently surgeons must be aware and conscientious about a rare potential complication of osteonecrosis following orthognathic surgery .

transoral vertical ramus osteotomy ( tovro ) procedure can result in a variety of complications . complications commonly reported include extensive bleeding due to major blood vessel injury , unpredictable fracture , postoperative infection , neurosensory deficit related inferior alveolar nerve , insufficient osteosynthesis , and temporomandibular joint problem . the authors describe a case of partial necrosis of the mandibular proximal segment following tovro , a rarely reported complication . a 37-year - old otherwise healthy woman underwent lefort l osteotomy and tovro to correct mandibular prognathism . postoperatively , she developed pain and swelling in the right submandibular region and was found to have a partial necrosis of proximal segment .

they present with many of the same features as adrenal pheochromocytomas including paroxysms of headache , sweating , and palpitations . these symptoms are due to the secretion of catecholamines by the tumor . in the past because of the advances in localization of these tumors by computed tomographic ( ct ) , magnetic resonance imaging ( mri ) , and i - metaiodobenzylguanidine ( mibg ) scans , laparoscopy is increasingly being used . a 45-year - old caucasian male was investigated with a lumbosacral mri for chronic backache . mri revealed an incidental mass in the aortocaval region . during fine needle aspiration cytology of this mass , the patient developed severe hypertension . the patient also reported intermittent symptoms of severe headaches occurring approximately once per week , excessive sweating , palpitations , and elevations in blood pressure . the diagnosis was confirmed by a plasma normetanephrine level of 4.26 nmol / l ( upper limit of normal level is 0.66 nmol / l ) and by ( mibg ) scan that showed an increased uptake in the mid abdomen ( figure 1 ) . ct revealed a 2.42.72.2-cm interaortocaval soft - tissue density mass at approximately the l2-l3 level without invasion of the aorta or inferior vena cava ( ivc ) ( figure 2 ) . the patient 's past medical history is significant for lumbar disk disease , melanoma , and seizures . i - metaiodobenzylguanidine ( mibg ) scan showing increased uptake in the mid abdomen at the l2-l3 level . axial ( a ) and coronal ( b ) views from a contrast enhanced computed tomographic ( ct ) scan revealing the position of the tumor between the inferior vena cava and aorta without evidence of invasion . the patient was prepared for surgery with alpha - adrenergic blockade with doxazosin . at surgery a 10-mm trocar was inserted just below the level of the umbilicus in the midclavicular line . three 5-mm trocars were also inserted : midline in the epigastrium to retract the liver , at the lateral border of the rectus abdominis mid way between the umbilicus and the costal margin , and at the anterior axillary line in the subcostal region . the right colon and the duodenum were mobilized to expose the inferior vena cava ( ivc ) . the mass was then exposed between the ivc and aorta with care taken not to manipulate the tumor initially , to minimize intraoperative hypertension . the ivc and the tumor were mobilized and , 10-mm and 5-mm hem - o - lok ( weck - teleflex , medical research triangle park , nc ) clips placed on the venous tributaries from the tumor . dissection was then carried out between the aorta and the ivc , which mobilized the tumor from the interaortocaval region . hem - o - lok clips were again used to control the multiple arteries feeding the tumor . the mass was placed in a specimen retrieval bag and removed through the umbilical incision . during mobilization of the tumor , there was significant elevation of the patient 's blood pressure with a maximum bp of 220/90 . the blood pressure was controlled by the anesthesiologist with a total dose of 50 mg of labetolol and 1.5 mg of nicardipine hydrochloride . he was not allowed anything by mouth , and on postoperative day 1 ( pod 1 ) an esophagogastroduodenoscopy was performed that revealed erosions in the stomach with no signs of recent bleeding . they develop from chromaffin cells of sympathetic paraganglia , which are derivatives of the neural crest . the most common sites for these tumors include the carotid body , jugular foramen , mediastinum , organ of zuckerkandl , and the periaortic region but can occur anywhere paraganglia are present . they occur more often in men and usually in the fourth or fifth decade of life but can occur at any age . they can be either functional or nonfunctional in nature , and are functional in the majority of cases of retroperitoneal paragangliomas . when functional , they produce symptoms identical to adrenal pheochromocytomas including paroxysms of hypertension , diaphoresis , headaches , tachycardia , and palpitations secondary to increased circulating catecholamines . most paragangliomas are sporadic in nature , but approximately 10% of these tumors may be associated with genetic disorders , such as familial paraganglioma , neurofibromatosis type 1 , von hippel - lindau , carney triad , and multiple endocrine neoplasia ( men ) type 2 . when compared with adrenal pheochromocytomas , paragangliomas are more likely to be malignant , multicentric , and metastatic , with 29% to 40% being malignant at the time of diagnosis . the diagnosis of a catecholamine - secreting tumor can be made by a select number of tests . fractionated plasma catecholamines and metanephrines , along with 24-hour urine catecholamines , will be elevated in the vast majority of cases . mibg scintigraphy is less sensitive than ct or mri , but is highly specific for paragangliomas and adrenal pheochromocytomas . when all 3 modalities of imaging are used in concert , the accuracy of localization has been reported to be 97% . this is unusual and in retrospect unnecessary and potentially dangerous because of the severe hypertensive episode that followed . this report underscores the importance of a thorough history and biochemical evaluation to rule out a paraganglioma , rather than proceeding with a biopsy . some of the reported benefits of laparoscopic resection over open surgery include shortened hospital stay , less postoperative pain , decreased adhesion formation , better cosmesis , and lower complication rates . another benefit of laparoscopy is that the magnified view provided by the endoscope can theoretically improve operative precision . however , preoperative imaging of the retroperitoneal vessels is important , and conversion to open surgery may be occasionally required . prior to any surgical manipulation of the tumor , preoperative - , sometimes followed by - , adrenergic blockades should be performed to aid in preventing intraoperative hypertensive crises . paragangliomas are rare tumors that can occur in multiple locations and can have severe consequences if not removed in a timely manner . , laparoscopy has become a viable solution to the treatment of these tumors with fewer adverse side effects compared with that of open surgical resection .

we present the case of a 45-year - old male with an interaortocaval paraganglioma . the tumor was incidentally diagnosed during magnetic resonance imaging ( mri ) of the lumbosacral spine to investigate back pain . the mri was followed by fine needle aspiration cytology that confirmed the diagnosis . the patient , however , did report a history of intermittent episodes of headaches , increased sweating , palpitations , and elevations in blood pressure . the tumor was excised laparoscopically without complications . needle biopsy of a retroperitoneal mass that could be a paraganglioma must be avoided .

we report a case of intracerebral fibroma , an exceedingly rare neoplasm found in the central nervous system . since 1985 , only two cases have been reported . a 41-year - old woman with no known history of seizures presented to the emergency department after a witnessed fall from standing . the patient had transient loss of consciousness , suffered a seizure and experienced post - ictal amnesia . the patient reported a history of falls , occurring approximately once per month over the past 23 years . diagnostic computed tomography ( ct ) and magnetic resonance imaging ( mri ) studies of the brain revealed a right - sided temporoparietal 2.5 2.0 cm high - attenuation lesion with associated vasogenic edema and heterogeneous enhancement after gadolinium administration ( fig . 1 ) . ct body imaging also indicated a 5-mm pulmonary nodule within the left upper lobe and a pelvic soft tissue irregularity with fluid in the region of the uterus . subsequent gynecologic workup revealed this to be a simple cyst ; however , given the body imaging profile , our differential diagnosis included meningioma or metastatic neoplasm . there was a pial interface between the lesion and the brain , allowing for gross total resection of the tumor and its capsule . the final pathology report reflected intracerebral fibroma , which was reviewed both at our institution and confirmed at johns hopkins reference laboratories . there were no whorls , psammoma bodies , meningothelial cells or other stigmata of meningioma . immunohistochemistry for ema , s-100 and sma were non - contributory ( figs 3 and 4 ) . the patient had an uneventful postoperative hospital course and was subsequently discharged to home on postoperative day 6 . she was seen 2 weeks later in clinic , and the neurological examination revealed no deficits . furthermore , she has remained seizure - free since the operation . an electronic search of the literature dating back to 1950 , using the keywords intracerebral fibroma , yields only 10 previously reported cases [ 17 ] , with only 2 cases reported since 1985 [ 8 , 9 ] . of the 10 prior cases , all but one were located supratentorially . similar to the tumor in our patient , most of these early described tumors reported a hard rubbery intracerebral mass unrelated to the meninges . in only two cases did the authors report an attachment to the falx or dura [ 6 , 7 ] . previously reported specimens indicate a tumor composed of elongated cells with the features of fibroblasts , embedded in abundant collagen bundles ; however , immunohistochemical staining was not described . table 1previously documented cases of intracerebral fibroma [ 19]case numberagesexlocationpathologic specimensizecd expression111 monthsmalesupratentorial , right frontal lobetotal resection5 5 5 cmna29 yearsfemalesupratentorial , left temporoparietal lobeopen biopsy , subtotal resection3 3 3 cmcd34 negative310 yearsfemalesupratentorial , right frontal lobeobserved upon autopsy5 5 5 cmna411 yearsfemalesupratentorial , right lateral ventricletotal resection3 cmna517 yearsmalesupratentorial , right parietal lobetotal resectionnana619 yearsfemalesupratentorial , right temporoparietal lobetotal resectionnana719 yearsmaleinfratentorial , left cerebellar hemispheretotal resection5 4 4 cmna824 yearsfemalesupratentorial , left frontotemporoparietal lobeobserved upon autopsynana942 yearsnasupratentorial , temporoparietal lobetotal resectionnana1052 yearsmalesupratentorial , right parietal lobetotal resection5 2.5 5 cmna previously documented cases of intracerebral fibroma [ 19 ] a closely related intracranial finding is that of solitary fibrous tumor ( sft ) of the meninges . the ultrastructural findings of sfts are similar to intracranial fibromas , in that they have spindle - cell proliferation with areas of collagenization . the main difference is found in the immunohistochemical staining and site of dural attachment . recent immunohistochemical and ultrastructural studies indicate that these tumors arise from mesenchymal fibroblast - like cells in the meningeal covering , and are classified as mesenchymal , non - meningothelial tumors , which is an important differential consideration . the immunohistochemical profile of sfts includes strong reactivity for cd34 and vimentin but not ema or s-100 . cd34 is a transmembrane glycoprotein , which is expressed by hematopoietic progenitor cells , endothelium and certain populations of mesenchymal stromal cells in the dermis and , by all accounts , is the most sensitive marker for sfts . intracerebral fibromas do not stain for cd34 , which is a clear difference in differentiating intracerebral fibroma and sft . currently , mri appears to be the modality of choice for initial detection of these tumors . however , there is no clear imaging differentiation between intracerebral fibroma and other fibrous tumors found within the intracranial cavity such as sft , meningioma or schwannoma . this increases the importance of adequate histological and immunohistochemical evaluation of these lesions in order to arrive at the appropriate tumor diagnosis . ultimately , the diagnosis of intracerebal fibroma was based on the tumor being unassociated with the dura , the histological evaluation of spindle - shaped cells interspersed with a dense collagen matrix and the immunohistochemical profile . few cases have been described , and little is known regarding the pathogenesis and diagnosis of these lesions . the diagnosis depends greatly on immunohistochemical and histologic correlation , and complete surgical resection remains the goal of treatment . further reports are needed to refine the true incidence of intracerebral fibromas and to provide a more clear definition of these lesions .

intracerebral fibromas are among the most rare neoplasms found in the central nervous system . ten previously reported cases have been documented in the literature including only two reported cases since 1985 . as a result , little is known about these uncommon intracerebral fibrous tumors . we report a case of an intracerebral fibroma without dural or leptomeningeal attachment , discuss the pertinent diagnostic findings and briefly review all prior reports of this entity .

the pilot observational study by weber - carstens and colleagues provides important contributions to a mechanistic explanation of the puzzling and complex phenomena of icu - acquired weakness ( icu - aw ) . earlier findings from this research group suggested that icuaw is primarily a myopathy and confirmed that initial pathology manifests , on average , 7 days after icu admission among the most severely ill [ 2 - 4 ] . in the current subanalysis with 40 of the original 52 subjects , multiple factors were examined for association with myopathy : molecular ( il-6 , c - reactive protein ( crp ) , and insulin growth factor binding protein ( igfbp)-1 ) ; serum osmolarity ; medication use ( norepinepherine , dobutamine , hydrocortisone , aminoglycosides , analgesics , sedatives and neuromuscular blocking agents ) ; and multisystem factors ( simplified acute physiology ( saps-2 ) and sequential organ failure assessment ( sofa ) scores ) . the authors suggest systemic , inflammatory - mediated pathology is the most significant risk factor for icu - aw . while il-6 had a significant contribution to the statistical model , the hazard ratio of 1.006 indicated a higher il-6 ( > 230 picograms / ml ) is little better than chance in predicting inexcitable muscle membranes ( see figure 7 in ) . crp , the second inflammatory biomarker , was not associated with abnormal muscle excitability ( p = 0.075 ) . . it may simply be that there are insufficient numbers of results to derive a meaningful cox regression equation - allowing a reasonable 10 samples per factor / covariate , a sample size of 160 would provide more value to the statistical model ( 16 covariates ; see table 2 in ) . the authors also report a hazard ratio for norepinepherine similar to that for il-6 . along with the relative differences in the presence of septic shock and organ dysfunction in table 1 in among participants with / without inexcitable muscle membrane , this finding lends support to oxidative stress or the interaction of oxidative stress and pro - inflammatory biomarkers as risk factors for myopathy in icu patients . multiple measures and more complex clinical data , such as a heterogeneous sample as in this report , make it difficult to derive important conclusions from small samples . building a framework to identify icu - aw early and to evaluate efficacy of treatments is essential . between 25 and 50% of patients who receive mechanical ventilation for 7 or more days experience neuromuscular abnormalities and these abnormalities can result in weakness and impaired function years after discharge from the icu . in the united states , from 1997 to 2006 , the number of icu patients who received mechanical ventilation and were subsequently discharged to home has decreased while transfers to long - term acute care increased significantly without concomitant changes in survival . for older adults discharged with new or additional dependency in daily activities after hospitalization , less than 31% return to prehospital function . determining interventions that alter muscle pathology and associated dysfunction among patients who experience prolonged mechanical ventilation , whether from a mechanistic or a holistic perspective , has the potential to reduce the duration of mechanical ventilation and length of hospital stay . the role of il-6 and other cytokines in muscle dysfunction is not yet clear . in healthy adults , very high levels yet il-6 is also associated with proteolysis and myosin loss . among patients with chronic inflammatory conditions / diseases , the sources of il-6 - muscle versus leukocyte - may also be important to muscle pathology . developing understanding of basic pathology and establishing predictive biomarkers will provide the opportunity for new hypothesis testing . in this exploratory report of risk factors associated with abnormal responses to direct muscle stimulation , molecular to multisystem levels of covariates were examined . future studies will be more compelling when focused on single - level , inter - related pathways . investigations related to molecular cascade interactions are providing insight into the genetic , signaling , bioenergetic , and metabolic processes that contribute to muscle health and disease . understanding of molecular determinants of common diseases encountered in the critically ill can provide the rationale for selection of therapeutic targets . if a serum il-6 value > 230 picograms / ml is confirmed in future studies as an early indicator of muscle dysfunction , then the efficacy of prevention and treatment strategies may be measured rapidly and inexpensively by il-6 . observational data like this report provide important information with which to calculate effect size and determine promising biologic pathways for future investigations . results also suggest that the timing of interventions to prevent icu - aw may need to occur earlier than typically occurs in many settings as serum il-6 and muscle stimulation responses were abnormal quite early in the majority of patients who went on to manifest icu - aw . crp : c - reactive protein ; icu - aw : icu - acquired weakness ; igfbp : insulin growth factor binding protein ; il : interleukin ; saps : simplified acute physiology ; sofa : sequential organ failure assessment . cw has received research and travel funding from hill - rom . 2007 - 2009 . samantha razavi , rn and jeffrey ruf , rn reviewed the literature and assembled articles for this commentary .

a pilot observational study by weber - carstens and colleagues contributes to a mechanistic explanation of the puzzling and complex phenomena of icu - acquired weakness ( icu - aw ) . the authors suggest systemic , inflammatory - mediated pathology is the most significant risk factor for icu - aw . while this finding is somewhat equivocal , it provides important direction for future investigations and illustrates the challenges of interpreting significance in small observational studies .

granuloma inguinale ( gi ) ( donovanosis ) is an acquired chronic , slowly progressive , mildly contagious disease of venereal origin , caused by klebsiella ( calymmatobacterium ) granulomatis a gram - negative encapsulated rod characterized by granulomatous ulceration of the genitalia and neighboring sites , with little or no tendency to spontaneous healing . the first description of donovanosis is attributed to mcleod , professor of surgery at the medical college of calcutta , india , in 1882 . the first use of the term gi is not known , but is believed to be derived from the anatomic description as follows : granuloma indicates a growth of granulation tissue , and inguinale indicates involvement of the groin region . amongst the numerous nomenclatures suggested for this disease , donovanosis was proposed in 1950 to honor donovan , who noted the characteristic donovan bodies in macrophages and epithelial cells of the stratum malpighii . rajam and rangiah found carcinoma , either as a complication of or a sequel to long - standing donovanosis , to be a rare occurrence seen in 0.25% of 2000 cases . batla et al . in their study have reported cutaneous metastases of rectal mucinous adenocarcinoma mimicking as gi . squamous cell carcinoma ( scc ) of penis is a malignant growth found on the skin or in the tissues of the penis usually originating in the glans or foreskin . a 55-year - old , otherwise healthy male had 5 years history of multiple painless papulo nodular lesions on the penis . the nodular lesions slowly evolved and coalesced to form red ulcerated growth over the last 2 months . physical examination revealed multiple fungating , nontender , coalesced ulcers ( 2 cm 1 cm ) over prepuce with granulomatous base , and was associated with foul smelling discharge and phimosis [ figure 1 ] . there was no h / o significant weight loss , fever or any other complaints . the rest of the physical examination and routine blood and urine analysis including serotesting for syphilis and hiv were unremarkable . excisional biopsy was performed and histopathological examination revealed hyperkeratosis , parakeratosis and acanthosis along with papillary hyperplasia and downward proliferation of mature squamous cells with smooth contours ( pseudoepitheliomatous hyperplasia ) . the underlying subepithelial tissue showed dilated lymphatics and congested vessels , deposits of chronic inflammatory cells and vacuolated macrophages [ figure 2 ] . pseudoepitheliomatous hyperplasia and vacuolated macrophages ( 100 ) patient was treated with cotrimoxazole ( 800 mg sulfamethoxazole and 160 mg trimethoprim ) twice a day for 21 days . current literature proposes to reclassify this organism as k. granulomati based on more detailed analysis now available . the name has been changed after sequencing the phoe and 165 ribosomal ribonucleic acid genes and demonstrating close homology with klebsiella pneumoniae and klebsiella rhinoscleromatis . the incubation period for gi is uncertain , ranges between 1360 days , 340 days , 1428 days , and 17 days . this wide range is probably multi - factorial and may reflect either late presentation and denial or nonsexual transmission . the penis , scrotum , and glans are the most commonly affected sites in males ; and the labia and perineum are the most commonly affected in females . vaginal and cervical involvement has also been reported and sometimes mistaken for scc . presentation can be through single or multiple nodules that erode into slow growing ulcerations that easily bleed . although gi is generally regarded as an sexually transmitted infections mainly affecting the genital area , the possibility remains that lesions are not always sexually transmitted , but also occur through fecal contamination and autoinoculation . the differential diagnosis of gi includes primary syphilis ; chancroid ; condyloma acuminata ; leishmaniasis ; scc . penile cancer is rare , comprising less than 1% of all male cancer and scc is the most common variant . scc presents as ulcerative , exophytic , papillary or fungating mass with foul smelling discharge . patient may complain of burning or itching over the glans or bleeding from the lesion . inguinal lymphadenopathy is a common finding in about 60% cases . in macrophages from patient tissue samples calymmatobacterium granulomati appears as bipolar - staining intracellular inclusions ( donovan bodies ) , which give appearance of safety pin due to chromatin condensation at the extremities when stained with giemsa or wright stains . staining was not done in our case as we did not suspect it as gi because of age of patient , scc like clinical appearance , chronicity of the lesion and patient 's denial of any sexual exposure . histopathology of gi shows pseudoepitheliomatous hyperplasia and the dermis has dense infiltrate of plasma cells and histocytes . in this infiltrate though pseudoepitheliomatous hyperplasia can be seen in early stages of scc of penis , there was no clear cut evidence of malignancy in the form of horn pearls , atypia of individual cells , or mitotic figures . available therapeutic options are gentamicin , tetracycline , ciprofloxacin , doxycycline , azithromycin , and cotrimoxazole . secondary infectious inoculation may occur , as well as more extensive and deep ulcerations with necrosis , fistula formation , and tissue mutilation . in advanced disease , with vast tissue obliteration and scarring , surgical excision may be required . in our case , the diagnosis was confirmed by biopsy and patient was successfully treated by giving cotrimoxazole twice a day for 21 days . in our case , subsequent medical therapy and excision eventually cured the disease and even after 4.5 years of follow - up patient has not developed any recurrence . once again it proves how efficient a tool histopathology is in dermatologist 's armor in diagnosis of uncommon cases .

granuloma inguinale ( gi ) is an acquired chronic , slowly progressive , mildly contagious disease of venereal origin , characterized by granulomatous ulceration of the genitalia and neighboring sites , with little or no tendency to spontaneous healing caused by klebsiella ( calymmatobacterium ) granulomatis . a 55-year - old male presented with fissured , foul smelling , fungating growth over prepuce with phimosis mimicking squamous cell carcinoma ( scc ) without lymphadenopathy . it started with painless papulonodular showed pseudoepitheliomatous hyperplasia , infiltration in dermis , acanthosis and vacuolated macrophages suggestive of gi and not showing any histopathological features of scc . patient was successfully treated by giving cotrimoxazole twice a day for 21 days . here , we presented a case of gi mimicking scc of penis , which was diagnosed on basis of histopathology and treated with excision followed by medical therapy with cotrimoxazole .

cancer is a prothrombotic condition that predisposes patients to various thromboses such as lower extremities thromboses and pulmonary embolism [ 1 , 2 ] . obstruction of blood flow from external compression , frequently from lymph node masses , is also common , especially malignancy of the lower abdomen and pelvis which can cause lower extremity edema and in the mediastinum which can cause superior vena cava syndrome from mediastinal lymphadenopathy . in contrast , compression of pulmonary vessels from mediastinal lymphadenopathy is less common but is important to correctly diagnose because the treatment and prognosis are different from those of pulmonary embolism . we report the case of a man with metastatic bladder carcinoma who had massive mediastinal lymphadenopathy causing external pulmonary artery compression leading to a rapidly fatal outcome . a 59-year - old man was admitted to the hospital with severe fatigue and dyspnea at rest . he had undergone a radical cystectomy with neobladder construction for transitional cell carcinoma of the urinary bladder seven months previously and had received five cycles of adjuvant dose - dense mvac ( methotrexate , vinblastine , adriamycin , and cisplatin ) chemotherapy after the operation . a few days before admission , restaging cts showed multiple liver metastases , two right kidney lesions , a lytic lesion in the thoracic spine , massive right hilar lymphadenopathy , right pelvic lymphadenopathy , and a right pleural effusion . on admission , the patient was pale , with dyspnea at rest , tachypnea , and tachycardia , but he had no chest pain , cough or hemoptysis . his blood pressure was 110/70 mmhg and the o2 saturation was 97% when the patient was on room air . laboratory evaluation showed a leukocytosis at 45,000 /l with neutrophilia at 43,700 /l and lymphopenia at 500 /l as well as a hemoglobin of 9.5 gm / dl and platelets at 66,000 /l . other abnormal values from the biochemical evaluation included the alkaline phosphatase at 218 iu / l ( normal range 35104 iu / l ) , the ldh at 590 iu / l ( normal range 135225 iu / l ) , crp at 12.66 mg / dl ( normal range 010 mg / dl ) , inr at 1.52 , and fibrinogen at 567 mg / dl . a chest x - ray showed right middle lobe atelectasis and bilateral infiltrates ( figure 1 ) . ct angiography of pulmonary arteries showed absence of arterial thrombi in the vessels but confirmed extensive mediastinal and hilar lymphadenopathy obstructing and impeding blood flow in both pulmonary vessels ( figure 2 ) . in preparation for emergency radiation treatment , the constellation of symptoms as described in this patient 's acute presentation is suggestive of acute pulmonary embolism , a condition not uncommon in patients with neoplastic diseases . cancer is a prothrombotic state and cancer patients frequently have other causes promoting thromboses such as reduced mobility , central catheters , and surgery . in addition to pulmonary embolism due to thrombosis , cancer patients may suffer from embolism due to neoplastic emboli , a condition termed pulmonary tumor embolism . this has been seen in up to 26% of cancer patients in autopsy studies [ 5 , 6 ] and has been documented in transitional cell carcinoma of the bladder . contrast - enhanced computer tomographic ( ct ) arteriography of the pulmonary vessels and ventilation - perfusion scintigraphy ( v / q scanning ) are the studies used for diagnosis of pulmonary embolism . contrast - enhanced ct arteriography has the advantage over ventilation - perfusion scintigraphy in being able to visualize and characterize nonvascular structures . it is also particularly useful in the presence of pleural effusions , a common occurrence in malignancy , where v / q scanning loses its diagnostic accuracy . signs of pulmonary embolism with ct arteriography include intraluminal filling defects , total cutoff of vascular enhancement , enlargement of the occluded vessel , pleural - based wedge - shaped areas with no contrast enhancement , and linear atelectasis [ 8 , 9 ] . in contrast , ct arteriography can not distinguish pulmonary embolism from the rare intra - arterial tumor masses . in this case diagnosis ct arteriography in our patient did not show pulmonary embolism but instead revealed external pressure on the pulmonary arteries from metastatic adenopathy , illustrating this advantage of ct arteriography . lymphadenopathy compressing the circulation of lower extremities is a common problem in patients with pelvic malignancies while mediastinal lymphadenopathy , most commonly from lung cancers , is a common cause of the superior vena cava syndrome . bladder cancer has a tendency to metastasize to intraabdominal lymph nodes , liver , bones , and lung [ 1113 ] while thoracic lymphadenopathy is also common although less than intraabdominal lymphadenopathy . mediastinal metastases from transitional cell carcinoma of the bladder either are asymptomatic or may more commonly produce the superior vena cava syndrome or dysphagia from esophageal invasion or compression . pulmonary arterial compression as seen in our patient is far less common and indeed this is to the best of our knowledge the first case with this occurrence reported in the english literature . nevertheless it needs to be included in the differential diagnosis of a cancer patient presenting with symptoms suggestive of acute pulmonary embolism because the treatments of the two conditions are different . prompt initiation of anticoagulation is mandatory in a patient diagnosed with pulmonary embolism while treatment for external compression includes radiation therapy with the possible addition of steroids to reduce concomitant soft tissue edema and improve reactive bronchoconstriction . as the molecular biology of metastatic bladder cancer becomes clearer and therapeutic options increase with both conventional chemotherapy and targeted therapies [ 1618 ] , successful management of these patients may become more relevant .

transitional cell carcinoma of the urinary bladder is a malignancy that metastasizes frequently to lymph nodes including the mediastinal lymph nodes . this occurrence may produce symptoms due to compression of adjacent structures such as the superior vena cava syndrome or dysphagia from esophageal compression . we report the case of a 59-year - old man with metastatic transitional cell carcinoma for whom mediastinal lymphadenopathy led to pulmonary artery compression and a rapidly fatal outcome . this rare occurrence has to be distinguished from pulmonary embolism , a much more frequent event in cancer patients , in order that proper and prompt treatment be initiated .

an atlanto - axial synovial cyst is very rare . since the first report of this lesion by onofrio and mih1 ) in 1988 , to our knowledge . however , the current report described a patient with a large hemorrhagic cystic mass which was seen around prevertebral space of the atlantoaxial joint on the left side and the obstruction of the nasopharyngeal cavity on cervical magnetic resonance image ( mri ) . we report a symptomatic case associated with rheumatoid atlanto - axial subluxation , which regressed after the surgical management . a 72-year - old woman presented with sudden severe headache in her left occipital area with dyspnea . a laboratory examination of the patient 's blood revealed no remarkable abnormality indicative of inflammation , but high - titer of rheumatoid factor ( 95.9 iu / ml ) . seven months ago before visiting to our department , she checked brain mri due to sudden attack of severe headache on left occipital area , which revealed non - specific lesion except rheumatoid pannus with small prevertebral cyst of c1 - 2 junction ( fig . , there was a large hemorrhagic cystic mass around prevertebral space of the atlanto - axial joint on the left side , obstructing the nasopharyngeal cavity on cervical mri ( fig . the cystic mass was connected to atlanto - axial joint capsule on axial view of computed tomography ( ct ) . multiple bony erosion , rheumatoid atlanto - axial instability including left tilted c1 - 2 subluxation and cranial settling were demonstrated . and there were loss of lordosis and retrolisthesis in c 3 - 4 , 4 - 5 ( fig . 3 ) . in the first operation stage , considering patient 's dyspnea , aspiration of the cystic lesion was performed via transoral approach with otolaryngology surgeon ( fig . after 0.5 cm - sized mucosal incision on left side oropharyngeal wall , residual material of the cyst was squeezed by forceps and removed by suction tools . in the second operation stage , there were rheumatoid atlanto - axial instability and retrolisthesis in c 3 - 4 , 4 - 5 , so it was followed by posterior occipito - cervical fusion that connected from occiput to c5 , using vertex screw & rod system and iliac bone graft ( fig . 5 ) . the patient was tolerable on her postoperative course and showed good respiration and relieved headache . cervical synovial cysts are rare and located at the c1 - 2 junction or lower cervical spine . only 24 cases of synovial cysts of the c1 - 2 junction have been reported in the literature5 ) . moreover , the pathogenesis of spinal synovial cysts remains unclear but is thought to be attributable to degenerative changes of the facet joints or excessive joint motion2,3,5,7 ) . it revealed multiple bony erosions and a large pannus formation of c1 - 2 junction on this case . this patient also showed atlanto - axial instability caused by rheumatoid arthritis , such as left tilted antlanto - axial subluxation and basilar impression . the atlantoaxial articulation is a true synovial joint and is responsible for a large proportion of normal cervical mobility . the etiology of articular cysts is unclear , but they are assumed to be degenerative because minor chronic damage to articular surfaces produces a reactive proliferation of synovium or fibrocartilage that includes loculated collections of mucinous fluid . so , we hypothesized that hemorrhagic event was developed as a result of microtrauma caused by rheumatoid atlantoaxial instability . it is known that the higher titer of rheumatoid factor , the more destructive manifestations of joint occur1 ) . the patient was in the controlled state in serologic inflammatory marker , such as esr(erythrocyte sedimentation rate ) and crp ( c - reactive protein ) . however , the high titer of rheumatoid factor , in spite of long standing medication of rheumatology , was related factor of progressive destruction of joints . we report a rare case of large hemorrhagic cyst on prevertebral space of left side c1 - 2 area associated with rheumatoid arthritis causing airway obstruction and left occipital pain , successfully managed anterior and posterior approach . we suggest repeated microtrauma due to atlanto - axial subluxation associated with rheumatoid arthritis as a main cause of hemorrhagic event on the cyst .

synovial cyst on prevertebral space of c1 - 2 joint is rare but may be associated hemorrhagic event . we describe a case of a 72-year - old woman who presented with sudden severe headache in her left occipital area with dyspnea . she had rheumatoid arthritis for 14-years . large hemorrhagic cystic mass was seen around prevertebral space of the atlantoaxial joint on the left side on cervical mri ( magnetic resonance image ) and it obstructed the nasopharyngeal cavity . aspiration of the cystic lesion was performed via transoral approach , followed by posterior occipito - cervical fusion . the specimen was xanthochromic , suggesting old hemorrhage . the patient was tolerable on her postoperative course and showed good respiration and relieved headache . we suggest that repeated microtrauma due to atalantoaxial subluxation associated with rheumatoid arthritis as a main cause of hemorrhagic event on the cyst .

oromandibular dystonia ( omd ) is a form of focal dystonia , characterized by involuntary repetitive movements of the jaw and forceful contractions of the face . , the masticatory muscles are mainly affected however , lower facial and tongue muscles may also be involved . it causes functional abnormalities in speech , swallowing and chewing and often leads to severe psychosocial discomfort . a 40-year old woman declared progressive speech difficulties , embarrassing grimacing movements and obstructed swallowing almost 2 years before the first visit to our out - patient clinic . at her admission she presented sustained jaw movement towards the left , severe difficulty in jaw opening during speech or eating , jaw protrusion and contraction of the platysma during speech and as a dystonic posture . the severe dystonic movements and contractions were present most of the time forcing her to use external assistance to open her mouth whenever attempting to eat . levels of serum copper , ceruloplasmin and levels of urine copper were normal thus , dismissing wilson s disease . six months before her admission the patient underwent a complete thyroidectomy due to papillary thyroid carcinoma as biopsy revealed . subsequently , the patient was placed on thyroid hormone replacement while , no subsequent improvement with regard to focal dystonia had occurred . for these reasons the paraneoplastic syndrome was ruled out . finally , assiduous neuropsychological assessment was normal . oral medication including clonazepam , diazepam and anticholinergics proved to be ineffective . the patient was finally treated with injections of botulinum a toxin in the following muscles as shown on table 1 . to assess the effectiveness of this specific treatment video recordings for the evaluation of the improvement of the symptoms the dystonia movement scale ( ds 0 - 8 ) was used . we followed an extra - oral approach and therefore , the injection was conducted with electromyography ( emg ) guidance using the allergan s electomyograph signal amplifier ( irvine , ca , usa ) . a needle electrode was inserted 3 - 4 cm until it was contacted with the muscle . one month after the bilateral treatment an obvious improvement both in speech and swallowing was observed . the patient herself experienced an 80% reduction of her prior symptoms while also no side effects were reported . the involuntary movements were abolished and she maintained correct occlusion without external assistance ( figure 2 ) . the dystonia scale rating was remarkably reduced ( table 1 ) . however , the difficulty in opening the mouth and the lateral jaw deviation persisted in a lesser degree . these were associated with the omission of injection of the upper head of the lateral pterygoid . considering the reduced activity of the treated muscles and the alteration of the dystonic pattern the total units of injected toxin were minimized during the second treatment session . one month later no mandibular displacement was observed and the patient experienced almost complete relief of the previous symptoms ( table 1 ) . therefore , the treatment has to be individualized in order to relief patients symptoms . for practical reasons and based on the clinical picture , the jaw - dystonia has been classified as jaw - closing , jaw opening , or jaw - deviation dystonia . , patients who suffer from jaw - closing and jaw - deviation dystonia have shown to have a better response to treatment when compared to those who have the jaw - opening subtype . most studies suggest botulinum toxin injection in contralateral lateral pterygoid and ipsilateral temporalis in jaw - deviation dystonia . the muscles involved in jaw - closing dystonia include masseter , temporalis and medial pterygoid . our patient is a rather complicated case which combines jaw closing , jaw - deviation dystonia and jaw protrusion . hence , the therapeutic protocol we followed was based to the emg findings during injection . the recommended by the literature therapeutic process does not include platysma in the potential injected muscles . we additionally treated platysma with high doses of botulinum toxin a. this particular muscle depresses the mandible and soft tissue of the lower face resulting in tensing the skin of the neck . botulinum toxin treatment of the lateral pterygoid lead to a marked discomfort palliation which was estimated from the horizontal jaw movements limitation for which this muscle is considered to be the most important.9 furthermore , the upper head of the lateral pterygoid muscle is mainly related to opening mouth movements while the lower head to the closing ones , a reason for the upper head to be spared from botulinum a toxin treatment in a jaw closure case , as ours . a precise anatomic localization with the help of emg guidance , the accurate injection of the lower head was achieved . the dystonic spontaneous activity of the muscle was found to be markedly decreased during the second injection . our patient presented a favorable response in the first treatment cycle . during the repeat treatment ` the doses were markedly reduced ( reduction by 40% ) , and the temporalis and sternocledomastoid muscles were omitted . peak improvement , as shown by the scales was succeeded after the first botulinum toxin injection . the following treatment session did not demonstrate so striking results ( table 1 ) , while the patient s satisfaction was increased , as there was a better cosmetic result . during the third and fourth follow up visits ( 12 and 16 months respectively after the initial treatment ) the obsessive symptoms were abolished and subsequent injections were made only in the lateral head of lateral pterygoid muscle . therefore , the accurate diagnosis of the muscles involved is of a great importance in order to accommodate effective treatment .

the objective of the present case report is to punctuate the importance of individualized therapy procedures and the accurate diagnosis of the muscles involved in oromandibular dystonia and underline the role of electromyography ( emg ) . we report a woman who presented sustained jaw movement towards the left , severe difficulty in jaw opening and jaw protrusion . the patient was treated with injections of botulinum a toxin in lateral pterygoid , masseter , platysma , sternoclidomastoid , temporalis muscles with emg guidance . she experienced an 80% reduction of her symptoms after the first injection . in jaw deviation dystonia symptoms impressively respond to botulinum toxin treatment of the pterygoid muscle . individualized therapy procedures are necessitated .

a 52-year - old male was admitted to medical ward for the management of a severe headache and raised blood pressure . subsequently , he suffered a seizure and developed an irrelevant speech for which a psychiatric consultation was sought . history revealed alcohol consumption from the age of 22 years . by the age of 45 years , he had features of alcohol dependence in the form of craving , tolerance , loss of control , withdrawal discomfort , the primacy of alcohol use , and sociooccupational deterioration . he consumed around 750 ml of rum daily . before 2 days admission , he abruptly stopped drinking after repeated pleading by family members . he had been detected to have hypertension in 2007 and diabetes mellitus ( type ii ) along with dyslipidemia in 2008 . he is educated up to 9 class and has been employed for last 26 years . he had tachycardia , raised blood pressure , bilateral coarse digital tremors , and diaphoresis . he was disoriented and had vivid visual and auditory hallucinations along with secondary delusions of persecution . a diagnosis of alcohol dependence syndrome ( complicated withdrawal with seizures and delirium ) was made , and the patient was treated with chlordiazepoxide in tapering doses , thiamine supplementation and parenteral haloperidol for behavioral control . his response to treatment was tardy and though his orientation improved he had difficulty in comprehending instructions even a week later . mini- mental state examination ( mmse ) score improved from 12 at admission to 26 . however , he remained dull and was unable to participate in routine neurocognitive evaluation even a fortnight after admission . laboratory investigations alcohol withdrawal scale and mini - mental state examination scores contrast enhanced magnetic resonance imaging ( mri ) brain done then , revealed a nonenhancing area of altered signal intensity in central pons suggestive of cpm [ figure 1 ] . brain spect showed hypoperfusion of frontal lobes ( l > r ) but yielded no further information about the pons [ figure 2 ] . electroencephalogram showed no abnormality . by the 3 week , cognitive functions started improving , and he was co - operative . evaluation of lifetime drinking history yielded total alcohol consumption of 496.61 kg over 30 years , with an average drinking day consumption of 80.35 g / day . repeated after 8 weeks brain , spect showed improvement in frontal lobe perfusion [ figure 3 ] . detailed neuropsychological evaluation done at 2 , 6 , and 10 weeks of admission is depicted in table 3 . even though there was relative improvement in scores on neuropsychological evaluation in the 6 and 10 week , cognitive deficits remained . contrast enhanced magnetic resonance imaging of brain ill - defined area of altered signal intensity in central pons ( 7.0 mm 6.7 mm 8.1 mm ) . ( c ) on magnetic resonance imaging diffusion there is true restriction of diffusion on diffusion weighted imaging and apparent diffusion coefficient map initial brain single photon emission computed tomography hypoperfusion in frontal lobes ( left hypoperfusion [ 15.96% ] > right hypoperfusion [ 20.87% ] ) repeat brain single photon emission computed tomography ( 8 weeks later ) . perfusion in frontal lobes ( left [ 17.91% ] , right [ 22.24% ] ) is better than in previous scan neuropsychological evaluation various mechanisms have been proposed for the development of osmotic demyelination following chronic alcohol use . oligodendroglia are particularly susceptible to dehydration and volume changes due to their physically tight alignment in the basis pontis . during rapid correction of hyponatremia , intracellular electrolyte corrections are swift , but the brain is unable to correct the loss of organic osmolytes quickly enough , resulting in cellular dehydration , damage to the myelin sheath , and oligodendrocyte degeneration . the process of maintaining an iso - osmolar environment with respect to the serum is energy consuming . if the patient is malnourished , as is typically the case in alcohol dependence , the cells may lack sufficient energy reserve to maintain the na / k atpase pump activity and to synthesize organic osmoles . alcohol - associated thiamine deficiency may exacerbate the problem because it decreases brain glucose uptake . this energy supply demand imbalance results in a pro - apoptotic drive . in this patient , no hyponatremia was detected , though he had been initially treated with intravenous ringer 's lactate in the medical ward for dehydration . after a protracted alcohol withdrawal , he manifested significant impairment in all cognitive domains which improved partially and gradually thereafter . nicols et al . found that two - thirds of patients of active chronic alcoholism exhibited frontal lobe impairment demonstrated by neuropsychological testing and spect , independent of brain atrophy . reported that decreased blood flow in the inferior frontal gyrus assessed by spect was associated with executive function deficits which were related to relapse in detoxified alcohol - dependent individuals . they should be investigated using neuroimaging and caution should be exercised while correcting their fluid and electrolyte imbalances .

osmotic demyelination syndrome includes central pontine myelinolysis and extrapontine myelinolysis . this condition has been described in cases of chronic alcohol dependence syndrome and in rapid correction of hyponatremia . though we frequently see patients with alcohol dependence syndrome presenting with complicated withdrawal , central pontine myelinolysis remains largely undetected and under - reported in literature . we present here a case of protracted delirium tremens where mri brain revealed central pontine myelinolysis . subsequently cognitive assessment revealed significant dysfunction and brain spect showed hypo - perfusion of the frontal lobes . osmotic demyelination syndrome should be suspected in protracted delirium tremens .

gunshot wounds ( gsw ) to the abdomen carry a high morbidity and mortality rate . however , there are few cases that report indirect injury as a result of the pressure wave effect created by the bullet . even fewer cases exist for which indirect pressure wave injury resulted in the need of open surgery . a 17 year old boy was brought into our major trauma centre , who sustained a single close range ( 2 m ) gunshot wound ( unknown calibre ) to the left upper quadrant of his abdomen . the patient had a palpable but weak ( compared to the contralateral side ) right femoral and popliteal pulse with absent pedal pulses that were easily palpable on the left . the patient was resuscitated based on advanced trauma life support guidelines and our own major trauma protocols . a contrast ct scan encompassing his chest , abdomen , pelvis and lower limbs was then performed , which demonstrated multiple bowel injuries as well as intraperitoneal free fluid consistent with blood , possibly arising from the superior mesenteric artery territory . a non - occlusive luminal irregularity of the right external iliac artery and a small calibre right femoral artery were also noted . the single bullet was found lodged in the right ilium , in close proximity to the right iliac artery with no signs of contrast extravasation ( fig . 1 , fig . 2 ) . the origin of the bleeding was found to be a mesenteric vessel , which was subsequently ligated . there were multiple bowel injuries , which were all closed and stapled off in a damage control manner . the retroperitoneum was opened , exposing the right external iliac artery , which demonstrated significant bruising and damage along its length despite the arterial wall maintaining its integrity . a right iliofemoral bypass with reversed great saphenous vein was performed on the patient . following this , there were no postoperative complications and on examination a day after surgery , the patient had a warm and well - perfused foot . doppler examination showed triphasic signals over the posterior tibial artery and biphasic signals over his anterior tibial artery . unfortunately , the patient did not return for any follow - up after the operation , and thus no subsequent information is available . in patients with gsw , the range of injuries is not limited to the track of the projectile . kinetic energy transfer from the bullet to adjacent tissues can cause diffuse disruption in tissue morphology and subsequent injury to vessels and viscera remote from the point of entry . multiple factors affect the amount of kinetic energy released from a fired projectile . these include the initial velocity of the projectile , projectile shape , angle of the yaw upon impact , and type of the impacted tissue . the subsequent cavitation effect produced by a travelling projectile causes displacement of tissues surrounding its path and the kinetic energy dissipated may extend up to 7 cm from the missile track . this destructive energy has the potential to cause organ injury far from the track of the projectile . thus , patients who sustain gsw may have vascular injuries remote from the visible point of entry and track of the projectile . indirect vascular injury after gsw has been documented in the literature3 , 4 and is not limited to only the vasculature . multiple reports of indirect injury to colon , spinal cord as well as other viscera has been described.5 , 6 a high clinical suspicion of injury to vasculature and viscera remote from the projectile track is paramount in patients presenting with gsw . our case report demonstrates the devastating nature of gsw and the indirect trauma that can arise secondary to the pressure wave effect in the vicinity of the projectile path , which compromised flow through the right external iliac artery in the patient despite the arterial wall integrity being maintained .

in patients presenting with gunshot wounds , a high clinical suspicion of injury to vasculature and viscera remote from the projectile track is paramount . we present a case of a 17 year old male who sustained a gunshot wound to his abdomen and subsequently developed a right external iliac artery contusion requiring surgery as an indirect effect of the pressure wave from the bullet .

the rifle criteria were born in vicenza ( italy ) back in 2004 when a group of smart clinicians realized that none of them was able to provide the same definition of acute kidney injury ( aki ) , formerly known as acute renal failure . aware of their unique expertise in the field of renal disorders in critically ill patients , these pioneers had the winning idea of creating the first definition of acute renal disease on the basis of one commonsense rule : to work , such a definition has to be universally utilized and , hence , based on just two , simple and easily available parameters , serum creatinine and urine output . a second innovation was to consider renal damage as a spectrum of severities that could be easily classified ( even though only by convention rather than by strict clinical relevance ) into three classes ( risk , injury and failure ) and two outcomes ( loss of function and end stage kidney disease ) . the final chapter of this story is that hundreds of authors tried to apply and eventually validated the rifle criteria with epidemiological studies on aki . it has to be noted that a ' definition ' should not be confounded with the concept of ' aetiological diagnosis ' : rifle criteria were not born to identify the cause of aki but only to identify it in a standardized way . consequently , a renewed interest in aki diagnosis , prevention and treatment was raised by this huge amount of standardized information : today , awareness of actual aki incidence and of its lethal effects ( even after milder forms of renal impairment ) in several clinical settings has dramatically increased and some centers have already implemented rifle score in their databases and medical records . the other side of the coin was that , even if sometimes unintentionally , each study tended to slightly modify the original rifle definition in order to best adapt it to its research . to cite just a few , in retrospective epidemiological studies , urine output ( uo ) criteria have been discarded because this kind of data is not easily achievable in databases . in other cases , baseline creatinine ( essential in rifle scoring ) has been argued about , estimated or not evaluated because it is not available for all patients . some authors have finally verified the rifle criteria on large cohorts of critically ill patients for very short periods of time , such as the first 24 hours after icu admission . it must be acknowledged that this variation may have significantly affected findings on aki prevalence , incidence and prognosis . in the previous issue of critical care wlodzimirow and coworkers show in a prospective observational study how rifle criteria should be correctly used . they substantially simulated routine use of rifle classification with uo criteria ( rifle serum creatinine ( scr)+uo ) and without uo criteria ( rifle scr ) for a whole week after icu admission . the authors also included patients without known pre - icu creatinine and estimated it , assuggested by rifle , with the modification of diet in renal disease formula . according to their findings , the use of rifle without the urine criteria significantly underscores the incidence and grade of aki , significantly delays the diagnosis of aki and underestimates mortality rates . remarkably , the accurate analysis by wlodzimirow and colleagues reveals that aki diagnosis during the first week of icu admission was doubled by rifle scr+uo , probably due to transient periods of oliguria not followed by increases in creatinine level . interestingly , prowle and coauthors , driven by a similar idea , recently concluded that oliguria ( < 0.5 ml / kg / h ) is a modest predictor of subsequent creatinine increase , especially when lasting for shorter periods of time ( < 12 hours ) . this aspect should be taken into account in clinical practice and , obviously , aki should not be classified after 30 minutes of anuria : as a matter of fact , the uo - risk class of rifle needs at least 6 hours of oliguria before the criterion is reached . however , uo is by far one of the earliest clinical signs of acute renal impairment . furthermore , we know that subclinical aki , either identified by pathological levels of early biomarkers ( such as neutrophil gelatinaseassociated lipocalin ) , or by transient azotemia ( defined as rapidly recovering aki with return to no - aki rifle class within 72 hours of onset ) is indeed associated with poor outcomes . on the other hand , creatinine alone may underscore the severity of aki , due to its delayed increase in the setting of rapidly evolving aki , especially in patients with fluid overload and hemodilution . alertness to milder forms of aki , those with the potential of worsening and that should be targeted by specific treatment , is one of the most important goals of the rifle classification . the original aim of its creators was not to predict mortality or to associate it with any hard clinical outcome , but to find a common language on aki diagnosis . new criteria have been proposed as substitutes of rifle in an attempt to improve the sensitivity and specificity of the aki identification : they apparently failed to achieve significant results . it should be considered whether a fi ne - tuning of the original rifle criteria might have worked better . moreover , as an exclusively personal opinion , rifle customizations and new classifications might have induced confusion and uncertainty in readers and clinicians , those originally intended to be the main users of such criteria . it must be remembered that the acute respiratory distress syndrome definition , born under the same rationale as rifle ( but many years earlier ) , has been recently modified after about two decades of worldwide application . in conclusion , the time has come to apply rifle clinically , at the bedside , applying both uo and creatinine criteria , with the purpose of longitudinally monitoring the renal condition of critically ill patients . the next step of rifle will be to understand if it is actually possible to standardize aki prevention strategies or therapeutic approaches to the different severity classes : early attempts have already been performed with success . aki : acute kidney injury ; rifle : risk : injury : failure : loss of function and end stage kidney disease ; scr : serum creatinine ; uo : urine output .

diagnosis and classification of acute kidney injury was addressed systematically only 8 years ago when the classification called rifle ( acronym of risk , injury , failure , loss of function and end stage kidney disease describing progressive severity of renal damage ) was created . since then , several studies have tried to apply , validate , criticize and modify this initial scheme : as a matter of fact , rifle is today one of the most appreciated and utilized medical classification systems worldwide . after an initial period of epidemiological research , it is acceptable to apply it now at the bedside , following both urine output and creatinine criteria , with the purpose of routinely monitoring renal function of critically ill patients .

pain experienced over long periods , such as with herniated intervertebral disc ( hivd ) , shows abnormal aspects in terms of its neurological mechanism1 . changes in the neurological mechanism in hivd patients lead them to perform abnormal patterns of movement2 . hivd patients have shown both asymmetry and atrophy of the spinal muscles on the side of the pain . lumbar traction reduces pressure on the vertebral foramen by releasing tension in the spinal muscles2 , 3 . lumbar traction reduces tension in soft tissue and pressure on the vertebrae3 , 4 . a previous study showed significant improvements in vas and the oswestry scale scores between traditional physical therapy and traction3 . some studies have reported no effect of traction , continuous lumbar traction , vertical traction for patients with radiating pain , self - traction for location and size of disc herniation , and traction for acute disc herniation4 . the connective tissue of the body is one unit in terms of structure and function ; the hamstring is attached to the hipbone and the knee . hamstring flexibility reduces back pain , increasing flexibility and coordination of the muscles without pain ; hamstring stretching resulted in significant improvements in a previous study5 . thus , we investigated the effect of stretching with lumbar traction on vas and oswestry scale scores of lumbar 45 hivd patients . they had no acute inflammation , instability of the cervical spine or joints , cervical flexion or extension , outflow of disc fragments , or lumbar , cardiac , or respiratory dysfunction , and none of them were pregnant . their average age , height , and weight were 41.111.03 years , 168.48.8 cm , and 69.511.03 kg , respectively . this study was approved by the inje university faculty of health sciences human ethics committee . an assistant stood next to the side of the subject to be exercised and held the leg under the ankle and on top of the knee . the assistant lifted the leg as far as possible while keeping the knee straight . lumbar traction was applied to produce a lordosis angle of 15 at the lumbar 45 disc with an electric ortho traction apparatus ( stc-200n , shin jin medical co. , seoul , republic of korea ) . subjects were positioned supine with the knee flexed on a wedge . the chest and pelvic were belted to provide support . for the first time , the traction was 25% of the body weight and was increased gradually by 2.25 kg / day . the maximum lumbar traction was 25% of the body weight plus13.5 kg . stretching with lumbar traction was provided 6 days / week for 4 weeks . the vas and oswestry scales were measured on the day before and 4 weeks after the intervention . the participant s perceived disability was assessed using the modified oswestry disability questionnaire ( odi ) . the contents of the odi include 10 items concerning pain intensity , personal care , lifting , walking , sitting , standing , sleep , sex life , social life , and traveling . the 10 items are scored from 0 to 5 . the sum total score is then doubled and reported as a percentage , from 0100 , with a higher score indicating greater disability6 . the significance level was set at p < 0.05 . the spss software ( ver . the results showed a significant decrease in vas scale scores for stretching with lumbar traction in lumbar 45 hivd patients , from 181.29 to 2.11.35 ( p < 0.05 ) . the oswestry scale scores also decreased significantly , from 20.352.01 to 3.52.84 , after stretching with lumbar traction ( p < 0.05 ) . in this study , we investigated vas and oswestry scale scores of lumbar 45 hivd patients with stretching and lumbar traction . the vas scores decreased significantly after stretching with lumbar traction , as did the oswestry scale scores . gose7 suggested that lumbar traction influenced disc herniation , degenerative discs , and facet joint syndrome and thus affected pain , mobility , and daily living function . kisner and colby8 suggested that there was a limit to correction of the symmetric stability in scoliosis using lumbar traction alone . subsequently , it was shown that traction with exercise affected thoracic expansion in scoliosis . therapeutic application of a stretching motion in scoliosis to increase flexibility may affect abdominal and pelvic muscles9 , 10 . previous in vivo studies assessing the effect of stretching on the hamstring muscle - tendon complex have demonstrated significant load - relaxation behavior11,12,13 . these results indicate that stretching to reduce muscle spasm influences vas and oswestry scale scores , as does stretching with lumbar traction . a study of children with scoliosis also showed that traction significantly influenced related exercises11 . the present results indicate that stretching with lumbar traction reduced vas and oswestry scale scores in lumbar 45 hivd patients . we believe that lumbar stretching with lumbar traction was shown to be more beneficial for reducing the tension on back muscles .

[ purpose ] this study investigated the effect of stretching with lumbar traction on vas and oswestry scale scores of lumbar 45 herniated intervertebral disc ( hivd ) patients . [ subjects ] we recruited 20 lumbar 45 hivd patients . [ methods ] we performed stretching with lumbar traction for lumbar 45 hivd patients during 4 weeks . the vas and oswestry scales were measured before and 4 weeks after the intervention . [ results ] the results showed a significant decrease in vas scale scores for stretching with lumbar traction in lumbar 45 hivd patients , from 181.29 to 2.11.35 . the oswestry scale scores also decreased significantly , from 20.352.01 to 3.52.84 , after stretching with lumbar traction . [ conclusion ] thus , we suggest stretching with lumbar traction for lumbar 45 hivd patients .

the filarial parasite loa loa causes a chronic infection in humans that has two very characteristic clinical features : calabar swellings ( localized angioedema found predominantly on the extremities ) and subconjuctival migration of the adult parasites ( " eyeworm " ) ( 1 ) . since two initial reports of suspected loiasis in korea ( 2 , 3 ) , there have been no further cases . we report a case of loiasis occurring in an african ( mauritanian ) female living in korea for the past five years with a history of staying in cameroon for four years before arriving in korea . the diagnosis was made by the removal of an adult loa loa worm from the eye and the presence of microfilaremia and calabar swelling . a 29-yr - old female patient suffered from sudden eyelid swelling and a sensation of motion on the left eyeball , persisting for one day . she found a moving threadlike worm at the upper bulbar conjunctiva on the visiting day using a mirror . she was born in mauritius in east africa , and had lived in korea for the past five years without ever visiting her home country . from 1996 to 2000 , before coming to korea , however , she had been in cameroon , which is included in the endemic area of the identified parasite . upon exposure to slit - lamp illumination , a sudden movement of the worm toward the fornix was noted . during the dim slit - lamp examination , painless swelling ( oval , 5 3.5 cm ) on the right forearm was also noted on physical examination . under minimal illumination and with topical anesthesia , parasitologic analysis confirmed the worm to be a threadlike female adult loa loa , with the vulva located at about 3 mm from the anterior end ( fig . microfilariasis was detected using the knott method , which allows up to 1 ml of blood to be concentrated per 10 ml of 2% formalin so that the sediment can be examined with giemsa stain for microfilariae . microfilariae were sheathed and averaged 261.1 m in length . when stained , the body nuclei were continuous to the tip of the tail ( fig . ivermectin ( 200 g / kg ) was prescribed for a single dose to prevent recurrence after confirmation of systemic microfilariasis . the patient departed from korea owing to unavoidable circumstances , and so long - term follow - up was not available . loa loa is restricted to africa , with a distribution that stretches from south - eastern benin in the west to southern sudan and uganda in the east , and from a latitude of 10 n to , perhaps , zambia in the south ( 4 ) . the adult worms live and migrate in the subcutaneous and deep connective tissues , and the microfilariae are found in the blood , where they can be ingested by mango flies or deerflies ( chrysops spp . ) . once ingested by a fly , the microfilariae become infective in approximately 10 to 12 days . development into adult worms takes about 6 to 12 months , and they can survive up to 17 yr . the first clinical signs may occur as soon as 5 months post - infection ( 5 ) but clinical prepatency may last up to 13 yr ( 6 ) . in this case , the patient 's country of origin , mauritius , is not reported to be an endemic area . she did have a history of staying in cameroon for four years , which is probably where she became infected with the worm , before her arrival in korea . adult loa migrate actively throughout the subcutaneous tissue of the body and derive their popular name ( eye worm ) from the fact that they are most conspicuous and irritating when crossing the conjunctiva . calabar swellings , named for the coastal nigerian town where they were first recorded , may be several inches in diameter and is a type of allergic reaction to the metabolic products of the worms or to dead worms . these swellings can occur anywhere , but are more frequently seen on the limbs , especially the forearms . painless swelling ( oval , 5 3.5 cm ) on the right forearm was observed in this patient . one of the main characteristics of human infection with loa is that a certain proportion of subjects with a recorded history of eye worm remain amicrofilaremic ( 7 , 8) . therefore , loiasis may be suggested by the presence of fugitive swelling in association with high eosinophilia in persons who have visited or lived in endemic areas ( 9 ) . two korean patients previously reported in korea had a history of either living in nigeria or traveling to cameroon , and had calabar swellings on their forearm or leg , high eosinophilia , and high antibody titer without microfilaremia ( 2 , 3 ) . specific igg4 measurement by enzyme - linked immunosorbent assay ( elisa ) to detect an occult infection is reportedly not very sensitive ( 10 , 11 ) . the best technique currently available for the diagnosis of loiasis , especially occult infection , is pcr for the detection of species - specific sequences of the repeat-3 region of the gene encoding a 15-kda protein ( 12 - 14 ) . for the detection of microfilaria , we used the knott 's concentration technique , which hemolyzes the red blood cells and concentrates leukocytes and microfilariae ( 15 ) , allowing us to observe moving microfilariae on the blood smear . loiasis can be effectively treated by surgical removal of the worms , leading to complete recovery . the most favorable removal time is when the worms are migrating through the corneal conjunctiva or across the bridge of the nose . in the present case , it should be noted that ivermectin treatment administered at the standard dose ( 150 g / kg ) may induce serious adverse events including encephalopathy , which may be fatal , in patients with very high loa loa microfilaremia ( 16 , 17 ) . our patient did not exhibit high microfilaremia or other side effects except fever and chills . long - term follow - up of this patient was not available because she departed from korea owing to unavoidable circumstances . as international exchange ( including travel ) makes the distinction between endemic and nonendemic areas less meaningful , loa loa infection should be considered in the differential diagnosis for patients with eosinophilia and calabar swelling in korea . in summary , we described a case with subconjunctival loiasis and calabar swelling on the limbs treated with surgical excision of the worm and an oral course of ivermectin .

loa loa is unique among the human filariae in that adult worms are occasionally visible during subconjuntival migration . a 29-yr - old african female student , living in korea for the past 5 yr without ever visiting her home country , presented with acute eyelid swelling and a sensation of motion on the left eyeball . her symptoms started one day earlier and became worse over time . examination revealed a threadlike worm beneath the left upper bulbar conjunctiva with mild eyelid swelling as well as painless swelling of the right forearm . upon exposure to slit - lamp illumination , a sudden movement of the worm toward the fornix was noted . after surgical extraction , parasitologic analysis confirmed the worm to be a female adult loa loa with the vulva at the extreme anterior end . on blood smear , the microfilariae had characteristic features of loa loa , including sheath and body nuclei up to the tip of the tail . the patient also showed eosinophilia ( 37% ) measuring 4,100/l . she took ivermectin ( 200 g / kg ) as a single dose and suffered from a mild fever and chills for one day . this patient , to the best of our knowledge , is the first case of subconjunctival loiasis with calabar swelling in korea .

laparoscopic surgery of the prepared upper and lower gastrointestinal ( gi ) tract has been well reported . successful closure of ruptured duodenal ulcers associated with peritoneal soiling has demonstrated that laparoscopic techniques can be applied to repair of the perforated viscus . more controversial is the therapeutic role of laparoscopic surgical techniques in perforations of the lower gi tract . , a 24-year - old male , presented with an injury sustained when he fell upon a pneumatic jack - hammer . upon his arrival in the emergency room , his only complaint was that of perianal pain . he was found to have a single complex laceration involving the perianal skin , subcutaneous tissue and completely lacerating the internal sphincter and the lower 4 cm of the rectum . the patient remained in the emergency room for two hours prior to being taken to the operating room holding area . there , he was found to have developed a complaint of generalized abdominal pain and findings of tachycardia and peritoneal signs . a proctoscopic examination revealed a transmural laceration of the anterior aspect of the rectum at 6 cm . no further studies were performed as it was felt that a patient with evolving peritoneal signs , penetrating trauma and free air needed to be explored . under general anesthesia , a transanal , single layer repair of the rectum was performed entirely via transanal approach , with continuous sutures of polyglycolic acid . the external sphincter and cutaneous margins of the complex laceration were then approximated with interrupted polyglycolic acid sutures . following this , laparoscopic entry to the abdomen was obtained at the umbilicus by the open hasson technique . a small volume of bloody serous fluid was noted in the pelvis and was aspirated and submitted for culture . using atraumatic bowel graspers ( snowden - pencer ) , the small bowel was run from the ligament of trietz to the ileo - cecal valve and no injury was detected . the bladder appeared intact upon inspection and , on gentle elevation of the bladder flap , the repaired rectal tear was visualized . a jackson - pratt drain was placed and positioned along the sigmoid gutter into the pelvis . the patient was placed on empiric therapy with imipenam and was observed in the icu . he was further evaluated by the urology service with a voiding cystourethrogram which was normal . cultures of the peritoneal cavity revealed gram negative rods which proved to be e. coli . his white blood count , 17,000 upon admission , decreased progressively and he was transferred from the icu . the patient passed flatus on postoperative day ( pod ) 3 and was allowed clear liquids . the drain was producing only small amounts of serous fluid following his first stools and was removed on pod 5 . his recovery at home was uneventful other than the development of a keyhole deformity of the anus which was repaired , uneventfully , six weeks later . there was no loss of sexual function or change in voiding or bowel habits , and he has since returned to work without disability . less well described , and more controversial , is its use in the treatment of acute lower gi pathology , including perforation . the approach to the issue of right vs. left - sided colonic perforation and the creation of a temporary stoma vs. primary resection or repair has evolved to the point where recent papers describe primary resection or repair without colostomy as the method of choice in selected left colon injuries . our patient presented with penetrating trauma to an unprepped left colon and bacterial peritonitis . while the perforation proved to be amenable to transanal closure , laparoscopy offered the opportunity to inspect the pelvic and abdominal organs , irrigate the abdomen , place a drain and test the integrity of the suture line . our experience in laparoscopic colon surgery would have allowed sutured or stapled closure , or resection , with or without colostomy , should the injury have been more proximal or associated with more extensive tissue damage or gross soilage . this result would certainly agree with the suggestion by others that the laparoscopic approach be considered first in dealing with an expected single perforation from iatrogenic injury during colonoscopy and may suggest that a subset of perforated diverticulitis patients may exist in whom the standard two - stage hartmann procedure , and its resultant disability , may prove unnecessary .

elective laparoscopic colonic surgery is increasingly recognized as feasible and perhaps preferential . a case of laparoscopically assisted surgery for trauma to the rectum with bacterial peritonitis is presented . it presents an example of the application of this modality to the treatment of iatrogenic colon perforations and perhaps selected diverticulitis .

prescribing psychotropic medication in pregnancy is a complex issue involving assessment of the risk of leaving an untreated psychiatric illness with its attendant complications versus the risk of adverse effects on the fetus . olanzapine , one of the 2 generation antipsychotics , is a category c drug and there is no unequivocal evidence of harm to the fetus . here , we report a case of microcephaly with anopthalmos in a patient who was treated with olanzapine for unspecified non - organic psychosis . to the best of our knowledge , ms . a , a 25-year - old married lady presented in the hospital 's walk - in clinic with the symptoms of suspiciousness and hearing voices . she also suffered few episodes suggestive of dissociative spells in which she would call herself goddess kali . there was also occasional history of disinhibition in the form of taking of her clothes in front of her family members . she was diagnosed as having unspecified non - organic psychosis on international classification of diseases , tenth edition . after 1 month of treatment with olanzapine , the patient became pregnant ( this was her first pregnancy ) and sought consultation for safety of medications during the 2 month of gestation . considering the severity of the symptoms in the past and duration of illness , on mutual discussions , it was decided to continue olanzapine . she was referred to the obstetrics service of the hospital to keep a close watch for any untoward event . she followed - up there regularly as advised by the obstetrician . a discussion with the consulting obstetrician and review of records revealed no major untoward event during pregnancy . antibody titers for herpes simplex , varicella , cytomegalovirus , toxoplasma and rubella were conducted and found to be normal . fasting and postprandial blood glucose levels performed at the time of first consultation and repeated in the 2 and 3 trimesters were found to be in the normal range . on follow - up , the fetus was detected to have microcephaly on ultrasonography . a delivered a female baby weighing 3.4 kg with microcephaly and congenital anopthalmos ( bilateral ) at full term . the head circumference was 30.5 cm at birth , which falls below the third percentile . no additional causative factors could be elicited despite the above mentioned specialist referrals . however , detailed imaging and genetic investigations could not be done due to cost limitations . the history was corroborated by the patient 's mother as well as husband although this adverse event scored only three on the the naranjo et al . adverse drug reaction probability scale , the lower scores were due to non - applicability of some questions to this situation . for instance , the issue of improvement on discontinuation of drug or administration of antagonist obviously does not apply to this case . similarly , re - administration and replication of the adverse effect also can not be done in this case for ethical reasons . one study involving pregnancy outcomes in 151 patients on different atypical antipsychotics ( 60 were on olanzapine ) did not find any statistically significant differences in various pregnancy outcomes between the exposed and comparison groups , except the rate of low birth weight , which was 5 times higher in the exposed babies and a higher rate of therapeutic abortions . one case of encephalocoele with cleft lip and aqueductal stenosis was reported in a patient on olanzapine . arora and praharaj reported a case of meningocoele and ankyloblepharon in the child of a patient on olanzapine . reis and klln reported craniosynostosis and ureteral reflux in one , an upper limb reduction defect in a second and a ventricular septal defect and upper gastrointestinal tract malformation in the third infant whose mothers were exposed to olanzapine during pregnancy . another study on 37 prospective and 11 retrospective pregnancies with exposure to olanzapine did not find any increase in the rate of spontaneous abortion and malformation compared with the general population . of the prospective pregnancies , 84% had normal delivery and postnatal course . the remaining 16% suffered problems such as prematurity , postmaturity and low or high birth weight . newport et al . measured placental passage of medication from mother to fetus by measuring levels in umbilical cord serum and documented neonatal outcomes in 54 women followed through pregnancy . they concluded that olanzapine has the highest rate of placental passage , compared with haloperidol , risperidone and quetiapine . neonates exposed to olanzapine showed trends toward lower birth weights and more neonatal intensive care unit admissions than neonates exposed to other antipsychotic medication . some authors have reported varying results among pregnant women treated with olanzapine while others have not reported any harm to either mother or fetus . to the best of our knowledge , this is the first report of microcephaly along with anopthalmos following intranatal exposure to olanzapine . we made attempts to rule out alternative causes and several of the causes that are responsible for baseline rates of congenital anomalies were ruled out . our patient was continuously exposed to olanzapine even before conception until birth of the infant . authors have earlier hypothesized that this may increase the risk as the early developmental period is crucial for organogenesis . they also highlighted that many negative studies had olanzapine exposure after the 8 week of pregnancy . thus , we hypothesize that early and sustained exposure to olanzapine may impair embryonic development in ways that may not occur with shorter or later periods of exposure . calmodulin antagonism during early development has been proposed as a possible mechanism for antipsychotics in general ; although , its specificity to olanzapine is unclear . however , our report by virtue of being a case report has its obvious limitations of not being able to conclusively prove a causal link . furthermore , for reasons mentioned earlier , some of the investigations could not be performed . the largest of the studies in pregnant women so far had only 60 patients on olanzapine . the purpose of this case report is more to stimulate further research in this direction .

olanzapine , a 2nd generation antipsychotic , is in use in the clinical practice for nearly a decade and a half now . it is classified as a category c drug with very few reports of its toxic effects on the fetus . in general , the risk benefit analysis warrants its use in pregnancy . we report a case of microcephaly and anopthalmos associated with the use of olanzapine in pregnancy . although a causal role can not be unequivocally proven , it calls for larger studies to explore this issue .

square jaw refers to an enlarged masseter muscle , along with an increased bony growth in the mandibular angle area . in general , masseter muscle hypertrophy appears and is characterized by sleep bruxism symptoms , etc . in most cases , square jaw is associated with masseter muscle hypertrophy in the caucasian , while it is associated with mandibular protrusion in the asian . caucasians generally have dolichocephalic head forms , while asians have brachycephalic or mesocephalic head forms . the shape of the mandibular angle is important in determining the impression of the face ; therefore , the square - shaped face of asians may cause a negative impact aesthetically . first , a botulinum toxin injection or radiofrequency therapy can be performed , which reduces the volume of the masseter muscle . second , a mandibular angle reduction can be done via an extraoral or intraoral approach , or a cortical bone resection in cases where the mandibular width is wide or the mandibular angle area protrudes outward . complications include excessive bleeding due to negligence during the surgery , overcorrection or undercorrection , asymmetry , infection , mandibular condylar fracture , inferior alveolar nerve injury and trismus12 . the aims of this case report were to describe a patient who experienced trismus , dysesthesia and a newly formed secondary angle after receiving bilateral mandibular angle reduction and partial masseter muscle resection for square jaw in a plastic surgery clinic . this study analyzed complications that appeared after mandibular angle reduction and masseter muscle resection , and considered the methods needed to resolve and prevent them . a 20-year - old male patient visited the department of oral and maxillofacial surgery at smg - snu boramae medical center ( seoul , korea ) with major complaints of bilateral mandibular angle trismus and dysesthesia after having undergone surgery . the patient received bilateral mandibular angle reduction and partial masseter muscle resection via a pre - auricular approach at a plastic surgery clinic approximately 10 months previously , but did not receive additional treatment during the postoperative recovering period . the patient did not have a history of systemic disease , and clinical and radiological diagnostic examinations were performed . upon palpation , he complained of dysesthesia in the bilateral mandibular area with a maximum mouth opening of 11 mm , based on the edge of maxillary and mandibular central incisors , thereby showing severe trismus symptoms . 1 ) for the radiological examination , routine panoramic and temporomandibular joint panoramic radiographs were taken . the radiographic images showed that the bilateral mandibular angle area and inferior margin were resected very close to the mandibular canal , and the secondary angle was formed in the resected anterior and posterior margins.(fig . 2 . a ) in addition , it was observed that only a rotational movement occurred without translation in the bilateral mandibular condyle upon opening the mouth.(fig . b ) despite these postoperative complications , the plastic surgery clinic that conducted the surgery did not perform any treatment to improve the patient 's trismus and dysesthesia signs and symptoms , which became more severe over time after the surgery . in this hospital , physical therapy ( transcutaneous electrical nerve stimulation , heat therapy , and ultrasound physical therapy ) and mouth opening exercises combined with botulinum toxin injection therapy were planned in order to improve the patient 's trismus . if the improvement effect is minimal , increasing the mouth opening via scar revision will be taken into consideration . in case of further need , bilateral coronoidotomy and masseter muscle stripping in addition , conservative treatment for his sensory disturbance is planned including muscle physical therapy or the administration of antidepressants . square jaw surgery methods include extraoral and intraoral approaches . in this case , the patient was treated with partial masseter muscle resection and mandibular angle reduction simultaneously via a pre - auricular approach . the extraoral approach has advantages in that it provides a good field of view and carries a small risk of infection ; however , its shortcomings include scar formation , and high risks for facial nerve injury . the need for a masseter muscle resection in square jaw patients must be carefully approached because the reason for square jaw in asians is bony growth , and not muscle hypertrophy , in most cases . moreover , postoperative swelling caused by bleeding within the muscle , muscle atrophy , and scar formation due to the masseter muscle resection may result in short - term or long - term trismus . furthermore , masseter muscle resection may cause facial nerve injury , which is less predictable , as compared to mandibular angle ostectomy3 . according to a survey that was conducted on 40 plastic surgeons in 2006 , 55% did not conduct masseter muscle resection during a mandibular angle ostectomy , and 45% conducted masseter muscle resection and mandibular angle ostectomy for certain cases4 . in this case , since masseter muscle resection was performed along with mandibular angle ostectomy , trismus occurred due to severe atrophy of the masseter muscle and scar tissue formation . around 22% of masseter muscle reductions occur without special complications after a mandibular angle ostectomy . in the case of severe masseter muscle hypertrophy that requires a masseter muscle resection5 , the masseter muscle can be reduced with an additional botulinum toxin injection . even in cases of square jaw symptoms due to masseter muscle hypertrophy after a mandibular angle ostectomy after surgery in the oral and maxillofacial area , trismus and sensory disturbance can occur often . but there are few reports about complications related to trismus and nerve injury after mandibular angle surgery combined with masseter muscle resection . this is why there is still no consensus on the efficacy of masseter muscle resection . also , masseter muscle resection is limitedly performed in severe cases of masseter muscle hypertrophy6 . if necessary , minimal resection of the masseter muscle in the deep portion and an intraoral approach may prevent major complications including trismus and nerve injury . yoshida reported injecting botulinum toxin , coronoidotomy and masseter muscle stripping for oromandibular dystonia patient - related limited mouth opening were effective7 . daily muscle physical therapy or the administration of antidepressants can also be effective therapies for dysesthesia patients8 . in this case , the secondary angle was a newly formed angle between the resection line and the mandibular inferior border . in such cases , the dissatisfaction of patients becomes more evident and revision surgery is sometimes conducted . such a phenomenon occurs when the mandibular angle area is resected in a straight line without considering the mandibular contour ; however , this can be prevented using a careful curve resection or mandibular outer cortical bone resection2 . for square jaw surgery , the approach or surgical technique must be carefully selected in order to obtain effective and satisfactory results . in order to prevent the problems seen in this case , the anatomical structures around the jaw must be sufficiently understood , and preventive methods and solutions must be identified for any complications that may occur . after surgery - related problems occur , the advice of a surgical technique specialist who can provide the patient with a proper treatment , must be obtained as early as possible in order to fix major complications .

a patient , who underwent partial masseter muscle resection and mandibular angle reduction at a plastic surgery clinic , visited this hospital with major complaints of trismus and dysesthesia . a secondary angle formation due to a wrong surgical method was observed via clinical and radiological examinations , and the patient complained of trismus due to the postoperative scars and muscular atrophy caused by the masseter muscle resection . the need for a masseter muscle resection in square jaw patients must be approached with caution . in addition , surgical techniques must be carefully selected in order to prevent complications , and obtain effective and satisfactory surgery results .

the triceps tendon usually ruptures when there is fall on an outstretched hand with the elbow in incomplete extension with or without a concomitant blow to the posterior aspect of the elbow.1 acute rupture of triceps following trauma usually occurs at the osteotendinous junction whereas a rupture at the myotendinous junction occurs less often.2 predisposing factors include local steroid injection , olecranon bursitis , and hyperparathyroidism.3 careful examinations of the active range of motion of the elbow determine the character of the tear , whether partial or complete.4 initial diagnosis may be difficult because a palpable defect is not always present and pain may limit the motion . when the diagnosis is missed , it leads to prolonged disability to the extensor mechanism of the elbow . mri also plays a vital role in diagnosing this condition and determining its character.5 here , we report a chronic triceps insufficiency managed with extensor carpi radialis longus and palmaris longus tendon grafts . a 25-year - old male carpenter presented with a 1 year history of a significant fall sustaining multiple injuries . the major disabling injury following trauma was a fracture in both bones of left forearm which was treated with an open reduction and internal fixation . the patient also told a history of pain and swelling in the posterior aspect of the right elbow following the initial trauma . soon after the swelling subsided , he noticed a depression in the posterior aspect of the elbow just above the olecranon for which he never sought any intervention . when he returned to his work as a carpenter after healing of other fractures , he felt disability limiting his activity which included inability to hammer and lack of extension . clinical examination revealed a depression just above the olecranon at the osteotendinous junction of triceps . he was able to do everything related with flexion , pronation , and supination , but was depending only on gravity for extention . radiographs revealed avulsion of a fleck of bone from the olecranon which migrated proximally attributing to the chronicity of the conditions [ figure 1 ] . plain x - ray of the elbow ( anteroposterior and lateral view ) showing avulsion of a fleck of bone from olecranon ( write arrow ) surgery was planned to fill the defect in the triceps and to reinforce it to the olecranon . the exposure of the tendon by midline posterior exposure showed a gap of 7 cms [ figure 2a ] . extensor carpi radialis longus tendon was released from its insertion by a small horizontal incision over the second metacarpal base . the palmaris longus tendon was released at the level of flexor crease of the wrist and also pulled out through a proximal incision . ( a ) intraoperative picture showing the insufficient triceps . ( b ) anchoring the graft to the olecranon . ( c ) postpulvertaft weaving of the graft to the triceps after harvest , a double - stranded graft measuring 15 cm in length and 6 mm in width was made and sutured together . the double - stranded graft was passed through the tunnel with equal length on both sides . the grafts on either side were brought together proximally making four strands which were sutured as a single unit [ figure 2b ] . the proximally retracted triceps was anchored to the quadruple strand of the graft by the pulvertaft weave technique [ figure 2c ] . a full range of motion of the elbow was checked , and an above elbow plaster slab was given with the elbow in 15 of flexion . the above elbow plaster slab was maintained for 6 weeks to allow adequate tendon to bone healing . , the patient regained full range of motion of the elbow [ figure 3 ] . on assessment of function using mayo elbow performance score , the maximum score of 100 was obtained which attributes to the full functional status of the patient.6 clinical photograph showing postoperative range of motion the study has been reviewed by the appropriate ethics committee and has therefore been performed in accordance with the ethical standards laid down in an appropriate version of the 1964 declaration of helsinki . the tear usually occurs when there is eccentric load to a contracting triceps , most commonly during sports.78 the best management is to avoid misdiagnosis and to treat the condition at the earliest.9 acute ruptures are successfully managed with nonabsorbable sutures passed through drill holes in the olecranon and anchored to the triceps.10 a chronic rupture is difficult to be reattached because of the retracted muscle belly.9 the literature provides various methods for correction oflarge triceps insufficiencies . it includes a turn down flap of triceps , anconeus rotation flap , allograft of tendo achillis , and autograft of hamstring tendon.1112 the use of extensor carpi radialis longus and palmaris longus to compensate a insufficient triceps has never been reported previously . extensor carpi radialis longus muscle has been used in various types of procedures for corrective hand surgery and is a proper muscle for correction of finger clawing.13 similarly , palmaris longus tendon is also being used for various procedures without significant functional impairment of the donor site.14 the easy availability of these tendons under regional anesthesia from the same limb without causing any significant functional impairment of the donor site led to their choice for our procedure . tendon to bone healing is said to be more secure , successful and functionally adaptive and hence preferred in this case.1517 the diameter of the tendon was fit to the size of the tunnel to prevent any wear and tear of the tendon . until adequate tendon to bone healing was achieved , the limb was immobilised . the patient achieved complete range of motion after 10 weeks with adequate physiotherapy . at the end of rehabilitation , the patient had almost full strength of extension . functionally , as graded by the mayo elbow scoring system , the patient had maximum points and a full function of the affected elbow.6

chronic triceps insufficiency , causing prolonged disability , occurs due to a missed diagnosis of an acute rupture . we report a 25 year old male with history of a significant fall sustaining multiple injuries . since then , he had inability in extending his right elbow for which he sought intervention after a year . diagnosis of triceps rupture was made clinicoradiologically and surgery was planned . intraoperative findings revealed a deficient triceps with a fleck of avulsed bone from olecranon . ipsilateral double tendon graft including extensor carpi radialis longus and palmaris longus were anchored to triceps and secured with the olecranon . six - months follow revealed a complete active extension of elbow and a full function at the donor site .

infantile tremor syndrome ( its ) is characterized by anemia , skin pigmentation , tremors , physical , and mental regression without a defined etiopathogenesis and low incidence . we have studied 9 patients over 1 year for the changing clinical and laboratory variables of patients with its . neuroregression and anemia were presented in all followed by tremors in 5 and hypotonia in 2 . infantile tremor syndrome ( its ) is a syndrome characterized by anemia , skin pigmentation , tremors , physical , and mental regression . it has been reported in children between 5 months and 3 years of age with a male predominance . the purpose of this case series was to determine factors responsible for its continuing prevalence despite better knowledge and weaning practices . we have studied 9 patients presenting to us with typical features of its and pre - its in a tertiary care hospital of hilly terrain [ table 1 ] . a detailed history and examination investigations included complete hemogram , peripheral smear to type the anemia and malaria parasite , serum electrolytes , renal and liver function tests along with sepsis screen , and urine routine examination . blood and urine cultures , serum b12 levels , chest x - ray , and tuberculosis screen were done when indicated . written informed consent was taken from all . total 9 patients , 3 males and 6 females presented over the period of 1 year . the mean age of presentation was 13.5 months , ranging from 7 months to 18 months . all ( 100% ) patients admitted with the complaint of lethargy and weakness with a mean duration of 21 days . neuroregression was present in all ( 100% ) with a mean delay of 7 months ranging from 4 to 11 months in all spheres of development . tremors were present in 5 ( 55% ) and hypotonia in 2 ( 22% ) reflecting neurological changes . seven ( 78% ) were exclusively breastfed and 2 ( 22% ) improperly weaned with diluted milk . protein energy malnutrition was present in 5 ( 55% ) out of which 2 ( 40% ) had grade i and 3 had grade ii ( 60% ) protein energy malnutrition as per iap classification . ofc records of all patients were within normal range for their age [ table 2 ] . all had severe pallor ( 100% ) with knuckle hyperpigmentation and sparse hair in most . hepatomegaly was present in 4 ( 44% ) , splenomegaly in 2 ( 22% ) , and pneumonia in 1 ( 11% ) . peripheral blood film examination revealed the macrocytic picture in 4 ( 44% ) , dimorphic in 4 ( 44% ) , and microcytic in 1 ( 11% ) . chest x - rays had infiltrated in 3 ( 33% ) but workup for tubercular screening was negative . urine cultures were positive in 2 ( 22% ) with escherichia coli and pseudumonas in 1 each . serum b12 levels were low in 4 out of 5 patients but could not be performed in all due to financial constraints . the mean duration of hospital stay was 9 days ranging from 4 to 16 days . total 6 ( 66% ) with positive sepsis screen or cultures received antibiotics for the average duration of 9 days . improved after treatment with supplements such as vitamins a , d , k , b - complex , zinc , calcium , and magnesium along with dietary management . on monthly follow - up , those with pre - its had better catch up in growth and development in all spheres . infantile and pre - its is a well - known clinical entity reported primarily from the developing nations . despite its decreasing incidence , the finding of additional clinical features made us present this series . associated nutritional deficiencies such as protein , vitamin a , d , c , and b - complex may be present . our series also found that all children with this syndrome had anemia , lethargy , and neuroregression and presented throughout the year with no seasonal clustering as previously stated . furthermore , the type of anemia in this disease is not specific . on the other hand , it reflects the pattern of prevalence of the hematinic deficiencies in the region . however , unlike previous reports , there were more female patients , which may be due to bias in nutritional status . the study adds the need for rational antibiotic use in patients of its , unlike many previous reports where all patients have been administered antibiotics . the disease process may be superadded by urinary infections probably due to lower immunity state as a result of malnutrition , the leading cause of acquired immunodeficiency . it is also a reflection of changing epidemiology with no seasonal variation , showing the role of non - viral etiologies although previous studies show a seasonal pattern of disease . it highlights the need for better education for weaning practices and need for better community support .

background : infantile tremor syndrome ( its ) is characterized by anemia , skin pigmentation , tremors , physical , and mental regression without a defined etiopathogenesis and low incidence.materials and methods : we have studied 9 patients over 1 year for the changing clinical and laboratory variables of patients with its . neuroregression and anemia were presented in all followed by tremors in 5 and hypotonia in 2.result:sepsis screen was positive in 6 and urine cultures in 2 . antibiotics were required in 6 . its with changing parameters still significantly contributes to healthcare burden.conclusion:it is important to screen for urinary infection and septicemia to avoid antibiotic abuse .

lichen planus pigmentosus ( lpp ) , an uncommon variant of lp , is characterized by mottled or reticulated hyperpigmented , dark brown macules in sun - exposed areas and flexural folds . the histopathologic findings of lpp consist of hyperkeratosis , atrophic epidermis with vacuolar alteration of the basal cell layer , and scarce lymphohistiocyte or lichenoid infiltrates in the dermis with pigmentary incontinence and the presence of melanophages ( 1 ) . although there have been a few reports of linear lichen planus , there has been no report of lpp with a linear pattern ( 2 , 3 ) . we describe two patients with lpp who had skin lesions in a linear distribution related to blaschko 's lines on their extremities . a 23-yr - old korean woman presented with asymptomatic dark brown macules on the left leg for 2 yr . one or two dark brown macules first appeared and spreaded gradually without any preceding erythematous or scaly skin eruption . there was no history of prolonged sun exposure or trauma on the lesion site . on examination , she had linear streaks of dark brown macules from the left ankle , across the calf and to the thigh , consistent with the pattern of blaschko 's lines ( fig . skin biopsy showed atrophic epidermis , basal hydropic degeneration with sparse perivascular lymphohistiocytic infiltrates , and numerous melanophages ( fig . these findings were consistent with lpp . a 16-yr - old korean woman presented with linear dark brown pigmentation on the left arm for one year . she stated that recurrent asymptomatic erythematous papules first appeared and then the lesion had developed into dark brown macules during 1 - 2 weeks . examination showed two linear streaks of dark brown macules from the left dorsum of hand to the upper arm probably related to blaschko 's lines ( fig . skin biopsy showed orthokeratosis , focal basal liquefaction , a sparse perivascular inflammatory infiltrate , and pigmentary incontinence , confirming the diagnosis of lpp ( fig . a 23-yr - old korean woman presented with asymptomatic dark brown macules on the left leg for 2 yr . one or two dark brown macules first appeared and spreaded gradually without any preceding erythematous or scaly skin eruption . there was no history of prolonged sun exposure or trauma on the lesion site . on examination , she had linear streaks of dark brown macules from the left ankle , across the calf and to the thigh , consistent with the pattern of blaschko 's lines ( fig . skin biopsy showed atrophic epidermis , basal hydropic degeneration with sparse perivascular lymphohistiocytic infiltrates , and numerous melanophages ( fig . a 16-yr - old korean woman presented with linear dark brown pigmentation on the left arm for one year . she stated that recurrent asymptomatic erythematous papules first appeared and then the lesion had developed into dark brown macules during 1 - 2 weeks . examination showed two linear streaks of dark brown macules from the left dorsum of hand to the upper arm probably related to blaschko 's lines ( fig . skin biopsy showed orthokeratosis , focal basal liquefaction , a sparse perivascular inflammatory infiltrate , and pigmentary incontinence , confirming the diagnosis of lpp ( fig . lpp has been described as a condition of unknown etiology which clinically differs from the classical lichen planus by exhibiting dark brown macules and/or papules and a longer clinical course without pruritus or scalp , nail , or mucosal involvement . the lpp is most common on sun - exposed areas such as the face , neck , and flexural folds including the axilla , inguinal , and submammary regions ( 1 ) . some authors observed a striking predominance of lesions in an intertriginous location , with most of them in the axillae , thus they proposed the designation lpp - inversus ( 4 ) . less common presentations include zosteriform pattern on the trunk ( 5 ) and involvement of non sun - exposed areas such as thigh ( 6 ) . the linearity of the lesions is probably related to blaschko 's lines , which suggests that the predisposition to develop lpp might be determined during embryogenesis . the differential diagnoses of our cases include lichen striatus ( 7 ) , linear and whorled nevoid melanosis and incontinentia pigmenti . in particular , because lichen striatus may have post - inflammatory hyperpigmentation ( 8) and lacks a well - defined histopathological picture ( 9 ) , it should be differentiated in our patients . lichen striatus almost always have preceding inflammatory papules or scaly eruption , which last for 4 months to 4 yr ( 8) . there was no previous papule in patient 1 and the short duration of preceding papules ( 1 - 2 weeks ) favor the lpp in patient 2 . there were no epidermal changes consisting of spongiosis and exocytosis , and a deeper dermal inflammatory infiltrate around adnexal structures which are features frequently seen in lichen striatus ( 9 ) . in summary , lpp can present with a linear pattern and thus should be considered in the differential diagnosis of linear hyperpigmented skin lesions .

we report two cases of lichen planus pigmentosus ( lpp ) that developed in a unilateral linear pattern . the patients presented with unilateral linear brown macules on the extremities . skin biopsy showed orthokeratosis , basal hydropic degeneration with scarce lymphohistiocytic infiltrates , and numerous melanophages in both patients . these patients , to the best of our knowledge , are the first cases of lpp presenting with a linear pattern . lpp should be considered in the differential diagnosis of linear hyperpigmented skin lesions .

similarly to its human ortholog , docrl associates with the membrane of several class of endosomes . docrl insures that ptdins(4,5)p2 pools are principally restricted at the plasma membrane by dephosphorylating this phosphoinositide on endomembranes . when docrl is knocked - down by rnai , drosophila cells in culture abnormally accumulate ptdins(4,5)p2 at the surface of giant endocytic vacuoles . interestingly , it has been recently reported that ocrl1 also regulates ptdins(4,5)p2 levels on endosomes of human cells . similarly to what we observed in drosophila , hela cells rnai - depleted for ocrl1 , present abnormal , enlarged endosomes enriched in ptdins(4,5)p2 . therefore , regulation of ptdins(4,5)p2 homeostasis and control of endosomal morphology by ocrl proteins is a general mechanism conserved across evolution . in addition , the function of ocrl proteins in the establishment of ptdins(4,5)p2 homeostasis is likely to participate to the underlying causes of the lowe syndrome since cells from patient suffering from this disease have been shown to present elevated levels of ptdins(4,5)p2 . we established that docrl does not preferentially associate with one specific endosomal compartment , and is found at the surface of early , late and recycling endosomes . however , two - hybrid experiments have revealed that its human ortholog , ocrl1 , interacts with 16 members of the rab protein family , which regulate membrane trafficking . furthermore , it has been shown that rab5 and rab6 directly stimulate the inositol 5-phosphatase activity of ocrl1 . therefore , it is tempting to speculate that rab proteins regulate ocrl proteins recruitment at the surface of endosomes to control homeostasis of ptdins(4,5)p2 . inactivation of ocrl proteins in drosophila and in human , leads to a strong disorganization of the endocytic compartments with the apparition of enlarged endosomes . in drosophila , these large endocytic vacuoles appear to be the result of an unregulated fusion of early , late and recycling endosomes . the molecular explanation of this defect is still unclear but our observations suggest that the abnormal accumulation of ptdins(4,5)p2 on endocytic compartments , disrupt the phosphoinositide signature of each family of endosomes and trigger homotypic fusion of these undefined endosomes . we found that when docrl is depleted by dsrna , up to 40% of drosophila cells fails cytokinesis and become multinucleated . this cytokinesis failure is characterized by an abortive cleavage furrow that still forms but regresses rapidly and does not successively separate the two daughter cells . this phenotype is directly linked to the deregulation of ptdins(4,5)p2 homeostasis : during cytokinesis , the abnormal accumulation of ptdins(4,5)p2 on endomembrane mis - targets essential components of the cytokinetic ring such as rhoa , actin , myosin and anillin . these components are recruited on endomembranes , at the expense of the cleavage furrow , and can not establish a stable , efficient , cortical cytokinetic ring . these observations brought into light the essential role of ptdins(4,5)p2 as one of the major spatial cue that secures cleavage furrow positioning and stability during cytokinesis . rnai depletion of ocrl1 in human cells delays abscission of the intercellular bridge connecting the two daughter cells after cytokinesis . in human cells , rab35 recruit ocrl1 at the intercellular bridge where it locally dephosphorylates ptdins(4,5)p2 and modifies lipid and actin composition . this remodeling of the intercellular bridge was proved to be necessary to the subsequent step of abscission . accordingly , intercellular bridge of cells from lowe syndrome patient show an abnormal accumulation of ptdins(4,5)p2 and actin that delays cell abscission . while ocrl proteins are important for cytokinesis in drosophila and in mammals , their inactivation does not trigger similar cell division defects . one possible explanation is that human cells also express inpp5b that could partially substitute for ocrl1 function during cell division . interestingly , we have found that human inpp5b could partially rescue docrl function during cytokinesis in drosophila cells ( unpublished results ) . these two studies have identified a novel and unexpected role of ocrl proteins during cell division . drosophila which expresses only one ortholog of ocrl1 appears as a powerful and promising model to further dissect in vivo the different functions of proteins of the ocrl family .

inositides are intrinsic components of cell membranes that regulate a wide variety of cellular functions . ptdins(4,5)p2 , one of the most abundant phosphoinositides , is restricted at the plasma membrane where it regulates numerous functions including cell division . we have recently established that the drosophila inositol 5-phosphatase , docrl , is essential for cytokinesis , the last step of cell division ( ben el kadhi et al . 2011).8 we demonstrated that docrl is required for the dephosphorylation of ptdins(4,5)p2 at the surface of endosomes , resulting in the restriction of this phosphoinositide to the cell cortex during cytokinesis . docrl is the drosophila ortholog of human ocrl1 , a ptdins(4,5)p2 phosphatase mutated in the x - linked disorder oculocerebrorenal lowe syndrome . here , we discuss the relevance of our findings with reference to the role of human ocrl1 in non - pathological and pathological conditions .

vogt - koyanagi - harada ( vkh ) disease is a rare multi - systemic inflammatory disorder which affects tissues containing melanin , including eyes , meninges , and skin . the classic clinical course of vkh is divided into three stages : stage 1 ( the prodromal stage ) includes flu - like symptoms ; stage 2 ( the acute uveitic stage ) includes auditory and neurological manifestations followed by visual disturbance associated with exudative retinal detachments and optic disc hyperemia , and stage 3 ( the convalescent stage ) can last for months or be chronic with sunset glow fundus , cutaneous hypopigmentation , and poliosis . macular hole ( mh ) secondary to uveitis is known to occur rarely , and only two cases with mh secondary to vkh disease at the convalescent stage have been reported in peer - reviewed literatures . the following describes a case with vkh who developed a unilateral mh at the acute uveitic stage . a 71-year - old japanese woman presented with visual loss accompanied by headaches and tinnitus . no history of ocular disorders was reported except for cataract surgery in the right eye ( re ) . the best - corrected visual acuity ( bcva ) at the initial examination was 0.02 in the re and 0.1 in the left eye ( le ) . slit - lamp ophthalmoscopy revealed inflammatory signs in the anterior chamber and vitreous cavity in both eyes . comprehensive fundus examinations showed circumferential choroidal detachments , multiple serous retinal detachments , and optic disc hyperemia ( fig . an increased number of mononuclear cells were detected in the cerebrospinal fluid and homozygous hla - dr4 alleles were identified . the patient was diagnosed with vkh disease based on the clinical findings , and high - dose corticosteroid therapy with gradual tapering was initiated 2 days after the presentation . the inflammatory signs , choroidal detachments , and serous retinal detachments disappeared within 2 weeks , and the bcva was recovered to 1.2/0.7 in the re / le after 4 weeks ( fig . six weeks after the initial administration of steroid ( i.e. in the gradual tapering period ) , vitreomacular traction ( vmt ) was found by optical coherence tomography ( oct ) in the le ( fig . two weeks later , the vmt progressed to mh ( stage 4 ) , and the bcva dropped to 0.2 ( fig . twenty - three weeks after the initial treatment , vitrectomy was performed with 23-gauge instruments . the standard surgical procedures for an idiopathic mh were employed , including inner limiting membrane ( ilm ) peeling around the fovea and air tamponade . the entire posterior vitreous detachment ( pvd ) , which had been present preoperatively , was confirmed during the operation . after keeping a face - down position for 4 days a case with mh secondary to the acute uveitic stage of vkh was treated successfully with vitrectomy . to our knowledge , this is the first report of mh which was developed at the acute uveitic stage of vkh . the serial oct images indicate that the uveitic inflammation induced severe vitreomacular adhesion and pvd formation , which resulted in vmt syndrome and progressed to mh . kobayashi et al . reported two cases both at the convalescent stage of vkh that developed mh associated with epiretinal membrane ( erm ) . based on the electron microscopic findings of the excised membranes , they speculated that the retinal pigment epithelial ( rpe ) cells might have migrated / proliferated on the ilm at the earlier uveitic stage ; then the mh was formed by adhering of the rpe cells to the vitreous cortex which eventually contracted . while the rpe migration / proliferation in erm secondary to uveitis has been thought unusual , some investigations have shown its possibility . in a histologic review of 168 erm samples , clarkson et al . reported that 1 out of 16 samples which contained rpe cells had a history of chronic uveitis . there is also an immunopathologic study of vkh which suggested rpe migration into the retina . although there are no available data indicating rpe migration in our case , rpe migration / proliferation could be a cause of the rapid pvd formation and severe vmt at the acute uveitic stage of vkh . to conclude , at the early stage of vkh , there is a possibility of mh formation due to the rapid progress of vitreous traction , and vitrectomy with ilm peeling could be an effective treatment . the study and treatment protocol was approved by the local ethics committee of national tokyo medical center .

we describe a case with macular hole ( mh ) associated with vogt - koyanagi - harada ( vkh ) disease . a 71-year - old japanese woman presented with visual loss and headaches . the best - corrected visual acuity ( bcva ) was 0.02 in the right eye ( re ) and 0.1 in the left eye ( le ) . the patient was diagnosed with vkh based on circumferential choroidal detachments , multiple serous retinal detachments , and optic disc hyperemia . the multiple serous retinal detachments improved with high - dose corticosteroid therapy and gradual tapering . the bcva was recovered to 1.2/0.7 in the re / le . six weeks after the initial administration of steroid , vitreomacular traction was found by optical coherence tomography in the le , which progressed to stage 4 mh with the bcva of 0.2 in the le . twenty - three weeks after the initial treatment , vitrectomy was performed with the standard surgical procedures , including inner limiting membrane peeling around the fovea and air tamponade . the mh was closed successfully and the bcva was 0.4 in the le 5 weeks after the vitrectomy . this is the first report of a case with mh secondary to the acute uveitic stage of vkh . successful closure of mh was achieved with the standard surgical intervention for an idiopathic mh . to conclude , at the early stage of vkh , there is a possibility of mh formation due to the rapid progress of vitreous traction following the inflammation , and the surgical procedure could be effective to resolve this secondary disorder .

in the previous issue of critical care , rumpf and colleagues evaluated the potential contribution of measuring end - tidal carbon dioxide ( co2 ) for suspected pulmonary embolism ( pe ) in the prehospital setting . capnography has been studied for decades as a potential diagnostic tool for patients with suspected pe . indeed , pe is expected to create areas of reduced arterial flow with normal or increased alveolar ventilation , resulting in increased alveolar dead space volume and reduced global expired co2 . this should create a difference between arterial and end - tidal co2 values , as first demonstrated by robin and colleagues in 1959 . however , during the two following decades , several authors pointed out the numerous pitfalls and sources of errors in assessing the arterial to end - tidal co2 difference in the clinical suspicion of pe , and this test was finally abandoned until the nineties [ 3 - 5 ] . three elements explain the current resurgence of expired co2 measurement in the suspicion of pe . first , technical improvements now allow measuring co2 not only for monitoring purposes in intubated patients in operating rooms but also as a diagnostic tool in spontaneously breathing patients in the emergency department or even in the field . second , volumetric capnography , which displays expired co2 as a function of the expired volume of the patient , did much to renew interest in capnography because of its potential for better performance in diagnosing pe than the arterial to endtidal co2 difference , even though that expectation could not be confirmed by recent results . finally , in the era of non - invasive strategies for pe combining several tests of various types , such as clinical evaluation , biological tests , and imaging , the evaluation of a potential role for co2 measurement in combination with those other instruments made sense . numerous studies are available , and although none to date has been able to prove the safety of such a non - invasive strategy incorporating capnography with a high enough level of evidence to allow its recommendation in daily clinical practice , the venue remains interesting [ 7 - 11 ] . where then can we place the endeavor of rumpf and colleagues ? they included 131 consecutive patients suspected of pe who had an abnormal rapid point - of - care d - dimer result in a prehospital setting and evaluated them with a combination of clinical probability of pe ( two - level wells score ) and measurement of the end - tidal partial pressure of co2 ( pco2 ) . pe was diagnosed in the emergency department by a positive spiral computed tomography , a high - probability v / q scan , or a positive pulmonary angiogram . the combination of a normal end - tidal co2 value ( defined as higher than 28 mm hg based on a receiver operating characteristic analysis ) and an unlikely probability of pe had a 100% sensitivity and 100% negative predictive value ( 95% confidence interval [ ci ] 90% to 100% ) for ruling out pe . in contrast , the association of a low end - tidal co2 value ( less than 28 mm hg ) and a high clinical probability had only an 86% positive predictive value for pe , and further tests would certainly be required in such patients . clearly , those results are preliminary . this is a small series and it was designed to set the cutoff value for this particular capnography technique and assess its feasibility in the field . moreover , as acknowledged by the authors themselves , the clinicians who established the diagnosis were not blinded to either clinical assessment or capnography results . finally , the prevalence of pe is unusually high , although this would tend to bias the results toward lower , not higher , sensitivity . but the sheer simplicity of the technique used by rumpf and colleagues is appealing and certainly deserves validation in a large - scale prospective study . indeed , it emphasizes the use of expired co2 alone without associated arterial pco2 , and this is a pragmatic issue in modern emergency medicine . also , the use of capnography in the prehospital setting is interesting : there might be situations in which a rapid and rough evaluation of the patient 's expired co2 status would help emergency physicians in making vital decisions , such as starting thrombolysis for a suspected fulminant pe , as well as in monitoring the hemodynamic effect of thrombolysis in such patients . finally , the merit of the article by rumpf and colleagues is to remind us that clinical applications of capnography are still growing , especially amongst spontaneously breathing patients . physicians dealing with acute medicine should make every effort to become familiar with expired co2 measurement . inconclusive capnographic results related to tachypneic or apprehensive patients do not overcome the potential for expired co2 to be placed inside the diagnostic algorithm of a challenging disease like pe . co2 : carbon dioxide ; pco2 : partial pressure of carbon dioxide ; pe : pulmonary embolism .

capnography has been studied for decades as a potential diagnostic tool for suspected pulmonary embolism . despite technological refinements and its combination with other non - invasive instruments , no evidence to date allows recommending the use of expired carbon dioxide measurement as a rule - out test for pulmonary embolism without additional radiological testing . further investigations are , however , still warranted .

to try to address these concerns with ppe , mdecins sans frontires convened a meeting on 3 april 2014 , at the galveston national laboratory in galveston , texas . representatives were present from the cdc viral special pathogens branch , the world health organization , the national institutes of health 's integrated research facilities at frederick , maryland and rocky mountain laboratories at hamilton , montana the galveston national laboratory , the public health agency of canada 's special pathogens unit , the ppe divisions of dupont , 3 m , and microgard , and the cdc national institute for occupational safety and health . this meeting brought together , for the first time , experts in the virology of filoviruses , worker protection and protective equipment , epidemiologists , and outbreak response agencies . the presumed portal of entry for filovirus disease in the occupational setting is exposure of the mucus membranes of the eyes , nose , and mouth to the virus . intact skin is assumed to be an effective barrier , although this can be bypassed by sharp penetrating injury or disruption of its integrity . given this , the consideration of ppe was taken up in two parts : protection from the neck down and from the neck up . protection from the neck up involves the highest risk of virus reaching the portals of entry . below the neck , the protection afforded by intact skin and gravity drawing fluids away from the mucus membranes leave , as the chief risk , the deposition of viral particles onto the skin or clothes that could subsequently be translocated to the mucus membranes . the primary role of gowns , coveralls , and aprons is to prevent deposition of the virus on the skin or clothes on the assumption that they prevent viral penetration . most commercially available garments that are engineered to provide resistance to infectious fluids have their resistance measured by their ability to prevent passage of a tracer bacteriophage , phi x 174 , in a synthetic blood medium under varying degrees of pressure . the tests that employ this bacteriophage are iso 16604 , which is a test method comprising part of the aforementioned european standard en 14126 , and the similar astm f1671 . phi x 174 is a nonenveloped dna virus 27 nm in diameter , whose physical characteristics are rather different from filoviruses , which are lipid - enveloped , thread - like forms 80 approximately 800 nm . as filoviruses are much larger than phi x 174 , materials passing the standard tests for protection from fluid - borne infectious agents may well be more resistant than is needed for protection from filoviruses . as higher degrees of resistance correlate with decreasing gas permeability , setting too high it may be that protection with material passing the iso 16604 or astm f1671 is unnecessary . healthcare worker deaths in past outbreaks were stopped with the introduction of ppe that used available surgical gowns that did not meet these standards . this may be the result of either the available materials being sufficiently resistant or of any virus penetrating this material being inactivated or removed before it could be transferred to the healthcare worker 's mucus membranes . a material that was not guaranteed to prevent filovirus penetration could still be employed , provided that the wearer 's skin were intact and that the wearer changed clothing and engaged in a skin decontamination procedure after doffing their ppe . this assumes that the worker does not have skin microbreaks of which they are unaware . the priority above the neck is the protection of the eyes , nose , and mouth . protective measures must first account for direct entry of virus , such as from coughed droplets or contaminated hands . however , protection must also account for indirect entry , such as the movement of contaminated material deposited onto the face or carried there by the hands , which may be drawn by gravity to the eyes or mouth when the wearer is perspiring . some ppe configurations have allowed for the skin of the head or neck to be exposed , while others offered complete coverage . the risk of infectious material migrating the short distance to the mucus membranes was considered high by most attendees . the decontamination procedures that might allow for tolerance of deposition of infectious material on intact skin below the neck do not apply above the neck . hair is not easily decontaminated by simple decontamination procedures , such as wiping with disinfectant or soap . more thorough decontamination procedures , such as a shower , may cause infectious material to run toward mucus membranes . as such , complete coverage above the neck with impermeable protection is advisable . above - neck ppe included facial protection that allowed for maximum visibility of the face while still providing complete coverage . if a nonenclosed face shield is employed , it should be supplemented by a mask to cover the mouth and nose from unconscious movements of the hands to the face . furthermore , the skin of the lower face should be disinfected after ppe removal to account for any infectious material deposited by unconscious hand movements . a fully enclosed solution that would prevent touching of the face while still permitting full visibility of the face would require ventilation to prevent fogging of the face shield . powered air purifying respirators have been employed in deployed field laboratories during filovirus outbreaks , but their suitability and practicality in the clinical environment has not yet been assessed . the limited evidence for the effectiveness of ppe used in filovirus outbreaks has led to choices being made with little empiric support . this weak evidence base prevented the attendees of the meeting from making strong recommendations with regard to the choice of protective equipment . as a first step , it was agreed that the materials currently in use for protective gowns , coveralls , and hoods should undergo testing in a biosafety level 4 ( bsl-4 ) laboratory to demonstrate their resistance to filovirus using standard ( iso 16604/astm f1671 ) methodology . the most gas - permeable material that prevented filovirus penetration under these test circumstances would be a suitable option for ppe use . tolerance for the use of protective material that has not been shown to be resistant to filovirus penetration is based on the assumption that intact skin is an effective barrier . as there is no evidence for this , it was agreed that this assumption should be tested in a nonhuman primate study in a bsl-4 laboratory . furthermore , a strategy would be needed to effectively monitor skin condition to prevent infection via nonintact skin , which could potentially result from insect bites , sunburn , shaving nicks , dermatitis ( including disinfectant - related dermatitis ) , and so forth . because the principle danger imposed by ppe was its potential for inducing heat illness , it was agreed that current ppe configurations should be tested in an environment simulating the temperature and humidity of the typical outbreak environment to determine the length of time they could be safely worn under normal working conditions . in addition , standardized test methods designed to quantify breathability of garment fabrics , such as iso 11092 , could be useful . these three investigations would establish an improved evidence base for selecting ppe for use in filovirus epidemics that would provide sufficient protection from the virus while minimizing the risk of heat illness in the wearer . given the unprecedented scope of the current ebola epidemic , and the need for the most effective ppe , the proposed research must be a very high priority .

personal protective equipment ( ppe ) is an important part of worker protection during filovirus outbreaks . the need to protect against a highly virulent fluid - borne pathogen in the tropical environment imposes a heat stress on the wearer that is itself a safety risk . no evidence supports the choice of ppe employed in recent outbreaks , and standard testing procedures employed by the protective garment industry do not well simulate filovirus exposure . further research is needed to determine the appropriate ppe for filoviruses and the heat stress that it imposes .

in april 2008 , fecal specimens were collected from 116 macaques ( 36 years of age ) with diarrhea on a monkey farm in people s republic of china . feces were suspended to 10% ( wt / vol ) in phosphate - buffered saline ( 0.01 m , ph 7.4 ) , and total rna was extracted from 200 l by using trizol reagent ( invitrogen , carlsbad , ca , usa ) . viral rna was dissolved in 30 l rnase - free water and stored at 80c . primers ( outside - l 5-ctagagagcttggccgtcgg-3 , outside - r 5-gtaccttctgggcatccttc-3 , inside - l 5-ggccttataccccgacttgc-3 , and inside - r 5-ggccttacaactagtgtttg-3 ) ( 12 ) were used for reverse transcription nested pcr to identify diverse hpev genotypes by amplification of a 518-bp fragment located in the 5 untranslated region ( utr ) . the expected - size dna bands were excised from an agarose gel , purified with the axyprep dna gel extraction kit ( axygen , union city , ca , usa ) , cloned into pmd-18 t vector ( takara , dalian , china ) , and sequenced ( applied biosystems 3730 dna analyzer ; invitrogen ) . the hpev sequences were compared with those of the hpev genotype reference strains by using blast ( www.ncbi.nlm.nih.gov/blast ) . five of the 6 sequences showed closest identity to the 5 utr of hpev-1 ( 90%94% ) . the viral protein ( vp)3/vp1 region of these 5 viruses was then pcr amplified and sequenced to confirm type 1 identity ( 13 ) . a 674-nt region of the 5 utr , an open reading frame ( orf ) encoding a polyprotein precursor of 2,182 aa , and a partial 3 utr of 88 nt ( 7,311 bp ) were sequenced . the near full genome showed 96% nucleotide identity with the genotype 6 reference genome ( ab252582 ) . the polyprotein encoded capsid proteins vp0 ( 312 aa ) , vp3 ( 229 aa ) , and vp1 ( 234 aa ) and nonstructural proteins 2a ( 150 aa ) , 2b ( 122 aa ) , 2c ( 329 aa ) , 3a ( 117 aa ) , 3b ( 20 aa ) , 3c ( 200 aa ) , and 3d ( 469 aa ) . the integrin binding motif arginine glycine aspartic acid was identified close to the c terminus of vp1 ( 8,14 ) . we performed phylogenetic analysis using the nearly full genome of sh6 and 17 representative hpev and related viruses ( figure ) . results confirmed that sh6 belonged to genotype 6 and clustered closely with the reference genome from japan and a strain from the netherlands ( eu077518 ) , forming an hpev-6 subgroup ( figure ) . phylogenetic tree was constructed by the neighbor - joining method with 1,000 bootstrap replicates using mega4.0 software ( www.megasoftware.net ) with an alignment of the nearly full genome isolated in this study and 17 human parechovirus ( hpev ) and related genomes . we detected hpev genotypes 1 and 6 in the feces of 6 of 116 monkeys with diarrhea . a similar analysis of a healthy monkey control group is needed to determine whether an association exists between hpev infections of monkeys and diarrhea . on the basis of the close similarities between virus feces derived sequences and hpev , these viruses might have been transmitted by the fecal oral route from humans to monkeys .

information about human parechovirus ( hpev ) infection in animals is scant . using 5 untranslated region reverse transcription pcr , we detected hpev in feces of monkeys with diarrhea and sequenced the complete genome of 1 isolate ( sh6 ) . monkeys may serve as reservoirs for zoonotic hpev transmissions and as models for studies of hpev pathogenesis .

we present a unique case of a dual injury , renal as well as ureteric , in a crossed ectopic kidney without fusion that was successfully managed without surgical excision . a 40-year - old male patient was admitted to the department of urology , calcutta national medical college , with a complaint of clear fluid discharge from an intra - abdominal drain placed on the right side of his lower abdomen . he had a history of an open appendicectomy done at a primary health care center 3 weeks previously for clinically suspected acute appendicitis . according to the operating surgeon 's brief the surgeon was able to dissect out the appendix with difficulty . during the dissection , the surgeon suspected a ureteral injury but was unable to repair it ; hence , he inserted a drain and referred the patient to us for further management . on examination , the patient was anxious but his clinical parameters , hemogram , renal function tests , and serum biochemistry were normal . there was an output of approximately 500 to 600 ml per day from the abdominal drain . analysis of the drain fluid revealed a high creatinine level , which suggested that the output was urine . the right kidney and ureter were normal on the intravenous pyelogram ( ivp ) but the left kidney was not seen in its anatomical position . on careful examination of the ivp , a faint shadow with areas of contrast excretion could be seen on the right side , especially in late films at the level of the fourth and fifth lumbar vertebrae ( fig . 1a ) . considering the possibility of crossed renal ectopia , contrast enhanced computed tomography ( cect ) was performed , which confirmed the diagnosis ( fig . the ectopic left kidney was small and showed excretion of contrast with evidence of hydronephrosis . a retrograde pyelogram ( rgp ) on the left side showed a hydronephrotic kidney with distorted pelvicalyceal system . there was also an area of narrowing in the ureter suggestive of stricture , which presumably led to the hydronephrosis ( fig . the patient was diagnosed to have a dual injury , renal as well as ureteric . a 5-fr double j ( dj ) stent was placed in the ectopic kidney , albeit with some difficulty , under fluoroscopic guidance ( fig . abdominal ultrasound , performed on the fifth postoperative day , did not reveal any abdominal collection . the dj stent was removed in the sixth postoperative week . at the 9-month follow - up , the patient was doing well . the diethylene triamine pentaacetic acid ( dtpa ) scan performed in the follow - up period a kidney located on the side opposite of its ureteral insertion is known as crossed ectopia . the anomaly occurs more commonly in males , and left - to - right ectopia is three times as common as right - to - left ectopia . abnormal umbilical arteries , ureteral abnormalities , induction of the contralateral nephronal anlage , malalignment , and abnormal rotation of the caudal part of the fetus have been postulated as possible causes . ninety percent of crossed ectopic kidneys are fused to their ipsilateral uncrossed renal unit . crossed renal ectopia without fusion is rare , with only 62 patients reported to date . the uncrossed kidney is usually normal in size , anatomical position , and orientation , whereas the crossed , ectopic kidney lies inferiorly in a skewed position with an anteriorly placed pelvis . the two renal units are separated from each other by a variable distance , and each unit has its own separate fascia of gerota . it is not unusual for crossed ectopic kidneys to remain undiagnosed throughout the life of a person , because they may function normally . they may be incidentally diagnosed when a patient is being evaluated for unrelated conditions . in some cases , the ectopic kidney may be the cause of pain or urinary problems . diagnosing a disease in such ectopic kidneys is often challenging . ultrasonography , ivp , dtpa , cect , and magnetic resonance imaging may be helpful in the anatomical as well as the functional evaluation of such kidneys . location of the ureteric orifices at cystoscopy supplemented by rgp may also be helpful in confirming the diagnosis . owing to the rarity of this condition , it is not exceptional for a surgeon to be unmindful of this abnormality and to injure it during surgery . the injury may necessitate the removal of the ectopic kidney . ignjatovic et al . also suggested that surgery in ectopic kidneys , if performed , should be done only once and should preferably be a nephrectomy . on the other hand , there are occasional reports of such injuries being managed conservatively . in the case presented above , both the ectopic kidney and the ureter were injured during the appendectomy , leading to hydronephrosis and urinary leakage . to the best of our knowledge , this is the first case report of conservative management of a dual injury in a crossed ectopic kidney without fusion . in conclusion , our case highlights that conservative management is possible in a crossed ectopic kidney without fusion and should be tried before contemplating surgery .

crossed renal ectopia is a condition in which a kidney is located on the side opposite of its ureteral insertion . ninety percent of crossed ectopic kidneys are fused to their ipsilateral uncrossed renal unit . crossed renal ectopia without fusion is rare , with only 62 patients reported in the literature to date . these kidneys may suffer iatrogenic injury during an unrelated surgical intervention . the injury , unless self - limiting , may necessitate the removal of the ectopic kidney . we present a unique case of a dual injury , renal as well as ureteric , in a crossed ectopic kidney without fusion that was successfully managed without surgical excision .

a 42-year - old female patient was admitted to the department of dentomaxillofacial radiology , faculty of dentistry , ankara university with a complaint of bleeding on the gums . the patient 's medical history included having undergone treatment for pernicious anemia and a gastric ulcer two years earlier . on intraoral examination , the patient was asymptomatic except for a few restored and missing teeth . a lesion was found incidentally on routine periapical radiographs taken for the restored teeth and edentulous areas ( fig . radiopaque masses were coincidentally observed in the periapical radiographs but the exact boundaries of the masses could not be seen . for further and more detailed examination , cone - beam computed tomography the panoramic radiograph and cbct revealed maxillary bilateral and symmetrical , well - defined , round , radiopaque masses in contact with the root of the maxillary right second molar and left first molar teeth . lobular , irregularly shaped radiopacities with clear radiolucent demarcation were also present in the right edentulous mandibular molar area ( figs . 2 and 3 ) . the bucco - lingual aspects of the lesions were best visualized on the axial cbct image ( fig . cbct added valuable radiographic information regarding the relationships of the lesions with significant anatomical structures , cortical plates , and tooth roots . dirty yellow , bone - hard tissues of 1 cm1 cm0.6 cm dimensions were analyzed in this examination . on the basis of the histopathologic findings , the tumor was diagnosed as fod ( fig . the patient was advised to have routine follow - up examinations at six - month intervals . the case has been followed up over the last 14 months and has remained asymptomatic . osseous dysplasias ( periapical , focal , florid osseous dysplasias , and familial gigantiform cementoma ) are a group of disorders that involve the same pathological processes.10 the clinical and radiological features of osseous dysplasia help to distinguish these lesions from others . pod is mostly confined to the anterior mandible , within the space between the canines.8 fod is a widespread form of pod and is a single lesion.1,5 the differential diagnosis of fod includes paget 's disease , chronic osteomyelitis , and gardner 's syndrome.1,10 paget 's disease is often polyostotic and shows changes in the level of serum calcium , phosphorous , and alkaline phosphatase.1,2,10 gardner 's syndrome may involve jaw bone changes similar to those with fod , but fod does not show any of the other skeletal changes that are seen in gardner 's syndrome.10 chronic osteomyelitis is a primary infection of the mandible presenting with unilateral pain , soft tissue swelling , fever , and lymphadenopathy.8,10 the secondarily infected lesion of fod has a wider radiolucent border than chronic osteomyelitis.1 steven et al reported a case that was diagnosed as chronic osteomyelitis with underlying infected fod.8 macdonald - jankowski published a systematic review on fod in 20036 that reviewed 158 cases , the mean age of which was 49 years . half of these cases had pain , swelling , and pus discharge / fistula with the frequencies of 52% , 31% , and 18% , respectively . fods can affect several areas of the jaws : posterior mandible area ( 99% ) , posterior maxilla ( 73% ) , anterior mandible ( 64% ) , and anterior maxilla ( 36% ) . the fod features observed in the present case report were in accordance with the results of that study . ong and siar provided their patient with surgical treatment to reduce the mass of the swellings.2 in rare cases , fod can be a hereditary condition with an autosomal dominant familial heredity.4 in our case , no familial aspect was observed . gndz et al7 reported a case of fod in which the lesion was seen throughout the alveolar processes of all quadrants of the maxilla and the mandible . the lesion was identified in three quadrants of the jaws in our case . while some reports have described diagnosing fod by clinical and radiological evaluation,5,7,10,13 others performed a biopsy.4,8,12 in the present case , a biopsy was also taken to confirm the diagnosis . in line with the various other reported cases , our patient was asymptomatic and the lesion was noticed incidentally on periapical radiographs taken for other purposes.4,5,7,10,13 up to now , two cases diagnosed by ct have been reported in the literature7,13 cbct images are useful diagnostic tools for identifying the location and extent of the lesion . cbct can be utilized to discriminate fod from lesions that display a similar sclerotic appearance on panoramic and periapical radiography.7 unlike the previous reports , in the diagnosis of the present case , not only clinical and radiological methods but also histological methods were used . clinicians should know the properties of such lesions and should be aware of their radiographic appearance . periapical radiographs may be insufficient to determine multifocal localization and the limits of the lesion . therefore , panoramic radiographs and ct images are preferable for a broader and more detailed examination . however , when infection of the lesion , resulting from trauma occurs , it requires surgical debridement and enucleation since antibiotics may not be effective due to the avascular nature of the lesion .

florid osseous dysplasia ( fod ) is an uncommon , benign , cemento - osseous lesion of the jaws . the etiology of fod is still unknown . it is often asymptomatic and may be identified on routine dental radiographs . the classic radiographic appearance of fod is amorphous , lobulated , mixed radiolucent / radiopaque masses of cotton - wool appearance with a sclerotic border in the jaws . in our case the lesion was found incidentally on routine periapical radiographs taken for restored teeth and edentulous areas . for further and detailed examination , a panoramic radiograph and cone - beam computed tomograph ( cbct ) were taken . the panoramic radiograph and cbct revealed maxillary bilateral and symmetrical , non - expansile , well - defined , round , radiopaque masses in contact with the root of the maxillary right second molar and left first molar teeth . our aim in presenting this case report was to highlight the importance of imaging in diagnosis of fod .

since the last description of rebase in the 2003 nar database issue ( 1 ) , there has been considerable growth in the size of the database primarily due to the large number of restriction modification ( rm ) genes that can be found in the sequence databases . more than 200 bacterial and archaeal genomes are available from genbank ( 2 ) and it is now clear that rm systems are much more common than had once seemed likely . mainly , this is because of the difficulty of detecting type i systems or solitary dna methyltransferases by biochemical or genetic assay . putative rm genes identified in these genomes are named systematically according to recently published nomenclature rules ( 3 ) and all have the suffix the rebase website ( http://rebase.neb.com/rebase/rebase.html ) summarizes all information known about every restriction enzyme and their associated proteins . this includes source , commercial availability , sequence data , crystal structure information , cleavage sites , recognition sequences , isoschizomers and methylation sensitivity . within the reference section of rebase , links are maintained to the full text of all papers whenever that is freely available on the web . rebase includes links to genbank and pubmed , and ncbi 's linkout utility uses rebase , pubmed and genbank record numbers to hook directly into rebase 's enzyme , sequence , reference and genome data . links to other major databases such as swissprot ( 4 ) , pdb ( 5 ) and pfam ( 6 ) are also maintained . there are currently 3681 biochemically characterized restriction enzymes in rebase and of the 3612 type ii restriction enzymes , 588 are commercially available , including 221 distinct specificities from a total of 253 total specificities known . as can be seen from figure 1 , the major growth in rebase during the previous two years has been in the number of putative genes for rm system components . more than 620 restriction enzyme genes and 2200 dna methyltransferase genes can be identified in genbank entries . the method used to identify putative rm genes in dna sequences has three fundamental components : the rebase database itself , an expert - derived set of rm system features and a computer program designed to spot these features in anonymous sequences . each sequence analyzed is checked for its overall sequence similarity to rebase gene sequences . for dna methyltransferase sequences , which are the primary indicator of an rm system , the presence , proper order and characteristic spacing of well - conserved motifs suggest the candidates . the more widely divergent genes of the restriction enzymes reside close to the genes for their cognate methyltransferases . such associations point to potential restriction enzyme genes , even when they lack any similarity to genes of known enzymes . publicly available sources of non - eukaryotic sequences are also analyzed frequently by this system . specialized information is available from the rebase lists icon and information about the sensitivity of restriction enzymes to dna methylation can be found by clicking on the rebase methylation sensitivity icon . in the latter case , the data is shown in double - strand format so that the effects of hemi - methylation and double - strand methylation are clearly differentiated . nebcutter analyzes dna sequences for the presence of restriction enzyme recognition sites ( 7 ) . rebsites will generate theoretical digests of an input dna with each of the 253 known specificities . rebpredictor is a tool for predicting restriction enzyme recognition sites that is an updated version of tables ( 8) and a specific blast ( 9 ) option permits a new sequence to be analyzed for rm genes . the rebase genomes icon leads to data for the currently sequenced 193 bacterial and 21 archaeal genomes . schematic representations of the whole genomes and the individual rm system within them are available and , from the pages showing the sequence schematics , there are links to the major database entries for these genes as well as links that will identify the closest neighboring sequences . this can be extremely useful in making predictions about the recognition sequence specificity of newly sequenced systems . this whole section of rebase provides a valuable resource for the annotation of the rm genes in a newly sequenced bacterial genome , particularly given the large numbers of rm systems that are often found . scientists interested in using the sequence information in rebase to annotate microbial genomes are encouraged to contact the rebase staff . special thanks are due to the many individuals who have so kindly contributed their unpublished results for inclusion in this compilation and to the rebase users who continue to steer our efforts with their helpful comments . this database is supported by the national library of medicine ( lm04971 ) and new england biolabs .

rebase is a comprehensive database of information about restriction enzymes , dna methyltransferases and related proteins involved in restriction modification . it contains both published and unpublished work with information about recognition and cleavage sites , isoschizomers , commercial availability , crystal and sequence data . experimentally characterized homing endonucleases are also included . additionally , rebase contains complete and up - to - date information about the methylation sensitivity of restriction endonucleases . an extensive analysis is included of the restriction modification systems that are predicted to be present in the sequenced bacterial and archaeal genomes from genbank . the contents of rebase are available by browsing from the web ( http://rebase.neb.com/rebase/rebase.html ) and through selected compilations by ftp ( ftp.neb.com ) and as monthly updates that can be requested via email .

a 64-year - old healthy man underwent phacoemulsification with intraocular lens implant on the left eye . preoperative fundus examination was normal and his best - corrected visual acuity in the left eye was 6/24 because of nuclear sclerosis . the patient was administered a single uneventful peribulbar injection consisting of 5 ml of lidocaine 2% and epinephrine 1:200,000 using a 25-gauge 25 mm peribulbar needle through the temporal portion of the inferior lid . the patient had no complaints and his visual acuity had improved to 6/6 . however , on examination , a left afferent pupillary defect was noted . dilated ophthalmoscopy of the left eye revealed several large confluent peripapillary patches of retinal whitening mimicking soft exudates ( purtscher flecken ) [ fig . fundus fluorescein angiography revealed multiple hypofluroscent areas in the peripapillary region corresponding to the areas of the soft exudates [ fig . 2 ] . swept - source optical coherence tomography of the left eye revealed edema of the inner retinal layers with hyperreflectivity of the nerve fiber layers corresponding to the areas of retinal whitening [ fig . 3 ] . as the patient was symptomless , no treatment was initiated for the treatment of the retinopathy . the patient underwent a comprehensive medical screening including liver function tests , pancreatic enzyme assay , carotid doppler , and transthoracic echocardiography to rule out other causes of purtscher - like retinopathy . subsequent follow - ups at 2 and 4 weeks revealed gradual disappearance of the cotton - wool spots [ fig . 1b and c ] , resolution of the peripapillary scotoma and decrease in the afferent papillary defect . ( c ) follow - up at 4 weeks fundus fluorescein angiography of the left eye ( postoperative day 4 ) revealed blocked fluorescence in the sites corresponding to the retinal and preretinal hemorrhages , capillary dropout in the areas of purtscher flecken and cotton - wool spots optical coherence tomography of the left eye ( postoperative day 4 ) revealed edema of the inner retinal layers with hyperreflectivity of the nerve fiber layers corresponding to the areas of retinal whitening unilateral purtscher 's retinopathy has been reported following facial trauma , periorbital steroid injection , and retrobulbar anesthesia . there have been two previous case reports of purtscher - like retinopathy after retrobulbar anesthesia . to the best of our knowledge , peribulbar anesthesia is known to be safer , but it has still shown to have the tendency to cause potentially dangerous complications including central retinal artery occlusion through the remote effects of the anesthetic agent , amount injected , speed of injection , and postinjection mechanical compression . the pathogenesis of purtscher 's retinopathy is still a subject of debate with several theories being forwarded since the initial mechanical theory proposed by purtscher . the most currently accepted theory is microembolization , causing arteriolar precapillary occlusion and microvascular infarct of retinal nerve fiber layer , forming cotton - wool spots . although bilateral purtscher - like retinopathy has been linked to the activation of complement and formation of leukoemboli , it is difficult to correlate this systemic mechanism to a local event . in this unilateral case , which was triggered by a local event of peribulbar anesthetic injection , the peripapillary distribution of the cotton - wool spots suggests an infarction of the peripapillary arterioles in an area of no collaterals . it could be hypothesized that the sudden increase in orbital volume might have increased the hydrostatic pressure and reproduced the conditions of a valsalva maneuver . however , this theory is challenged by the fact that considering the significantly larger volume of the extraconal space in comparison to intraconal space , 5 ml of anesthetic agent is unlikely to produce significant hydrostatic pressure to produce vascular occlusion . another possibility is that the infarction might have been caused by the accidental embolization of the central retinal artery or a posterior ciliary artery by either some residual air bubbles in the syringe or an orbital fat embolus mobilized by the needle . most likely , the infarction may have been caused by the vasospastic effects of adrenaline accentuated by the intrinsic vasoconstrictive properties of lidocaine . reported a decrease in retinal blood flow velocity by 1015% , 15 min , respectively , following peribulbar anesthesia without a vasoconstrictive agent like adrenaline . we report this case to inform ophthalmic surgeons and anesthetists that although peribulbar anesthesia avoids direct optic - nerve injury , indirect injury may occur from vasospasm in response to the injection .

purtscher and purtscher - like retinopathy is a distinctive retinal syndrome characterized by ischemic retinal whitening in a peripapillary pattern . we report a case of purtscher - like retinopathy in a healthy 64-year - old man after a routine peribulbar anesthetic injection for cataract surgery . although peribulbar anesthesia is considered to be a safer alternative to retrobulbar anesthesia , it has been associated with unusual but grave complications including central retinal artery occlusion .

hemiplegic migraine ( hm ) is a rare type of migraine which involves reversible motor weakness and other aura symptoms . the disease is divided into familial and sporadic form by genetic studies or clinical history . although gene mutation such as cacna1a , atp1a2 and scna1a were known to be related with familial hemiplegic migraine ( fhm ) , there are no special diagnostic tool or guideline for sporadic hemiplegic migraine ( shm).1 a recent study has suggested that electroencephalogram ( eeg ) may be used for determining charateristics of migraine and reported that increased beta band of t3 , f7 , o1 and o2 channels in migraine patients.2 other literature presented that patients with migraine showed more common spikes than the control group . during attacks of basilar migraine , unilateral or bilateral delta waves are recorded frequently.3 as in cases with hm , there have been a few findings of eeg reported as slow sharp waves and diffuse slowing with epileptiform discharges during hemiplegic migraine attack.46 however , these eeg of hemiplegic migraine were performed without sleep deprivation ( sd ) . in the present case , we report a shm patient who showed specific eeg findings after sd that were not observed during routine eeg without sd . a 52-year - old women visited our hospital due to severe headache during the evening mass . she had a history of migraine for two years that occurred about ten times per year . the headache was accompanied by symptoms of right arm weakness , confusion and motor aphasia which started with a duration of one year . these symptoms started with the headache simultaneously and usually persisted less than a few hours . on admission day , she complained with pressure - like pain on the head diffusely that was aggravated by loud noise or activity of daily living . her headache was accompanied by right arm weakness and mild motor aphasia that were developed simultaneously . she had no family history of hm , and the cacna1a gene mutation was negative . neurologic examination showed right arm paresis ( mrc grade iv~v- ) , mild confusion and motor aphasia . the magnetic resonance imaging ( mri ) revealed no specific findings except focal vascular dilatation on the right posterior communicating artery ( fig . she had been taken medical treatment for prophylactic and symptomatic control with propranolol 10 mg twice a day , flunarizine 5 mg once a day , tofisopam 50 mg twice a day , tramadol 37.5 mg and acetaminophen 325 mg daily . she underwent total five times of eeg including two times that had been done before admission and only the last eeg was performed with sd . every eeg was performed in symptom - free state for about 30 minutes , including activation process such as hyperventilation and photic stimulation . however , the last eeg which was performed after sd showed intermittent medium amplitude of 56 hz theta slowing on the left parieto - occipital area which was also partially seen during photic stimulation ( fig . more prominent large amplitude of posts was observed on the left occipital area during sleep stage i ( fig . her headache was resolved during hospitalization and she became symptom free after three months of follow - up . the patient in the present case suffered from headaches accompanied by reversible motor aphasia and right arm weakness , lasting a few hours that satisfies the criteria of hm . we could diagnose the patient as shm because of the aforementioned symptoms and the absence of familial history with hm ( table 1 ) . gene mutations of cacna1a , scn1a , and atp1a2 can cause fhm and we investigated only cacna1a gene mutation in the present case that revealed negative result.7 however , gene mutation is not one of the imperative criteria for shm according to the beta version of the third edition of international classification of headache disorders.1 the pathophysiologic mechanism of hm is not well known . intracellular calcium influx significantly increased in the cerebral cortex of a cacna1a - transgenic mouse . higher concentration of intra - cellular calcium induces more excitable neuron which may result in excitotoxicity.8 specific eeg findings about hm patients are known to be characterized by slow sharp waves on the hemisphere contralateral to the hemiplegic limb.4 other researches showed diffusely slow and polymorphic theta activity with some epileptiform discharges over the cerebral hemisphere contralateral to the symptomatic hemiparesis.5,6 recent report revealed the temporospatial dynamics of eeg during the full duration of a sporadic hemiplegic migraine attack . they suggested that eeg slow waves may reflect recovery of cortical spreading depression and large amplitude of slow waves during hemibody pain may be due to vasodilation of arteries.9 the eeg findings of the present case such as slow waves on the contralateral side of symptomatic limb and high amplitude of posts may also be explained by cortical spreading depression or vasodilation of arteries . they could not found the cause of permanent neurologic deficits except suggesting irreversible neuronal damage and did not focus on sleep deprivation of the patient . sd may be a burden in brain functioning and might have induced severe hemiplegic migraine or eeg changes in both cases . we tried to search other possible factors that might affect the eeg changes after sd . however , she had neither structural lesions on brain mri nor histories of taking antiepileptic or antipsychotic drugs . detection rate of interictal epileptiform discharges ( ieds ) in patient with epilepsy are increased after sd that is explained by stabilization of sleep instability and eeg cyclic alternating pattern.1012 this case showed specific eeg changes in symptom - free state of shm after sd . as the ieds in patient with epilepsy are increased after sd , this may be also applied to shm which could help clinical diagnosis .

hemiplegic migraine ( hm ) is an uncommon type of migraine which is classified into sporadic and familial subtype . the noticed electroencephalogram ( eeg ) findings during hm attack are diffuse slowing contralateral to the weakened limb , but are usually normal in asymptomatic states.a 52-year - old woman who suffered from headache accompanying right arm weakness and aphasic symptoms admitted to our hospital . she underwent total five times of eeg including 2 times before admission . only the last eeg exam after 24 hours of sleep deprivation ( sd ) showed intermittent slowing and higher amplitude of positive occipital sharp transients ( posts ) on the left parieto - occipital area . here , we report a case with hm who revealed abnormal eeg findings after sd , which was not observed in the routine eeg study without sd .

the patient , aged 54 , was admitted to the clinic for planned surgical treatment of an ascending aortic aneurysm with substantial aortic valve insufficiency . his medical history included previous pericarditis ( several years before , no hospital records available ) , without any other significant chronic conditions . for the past several months , the patient had been experiencing a reduction in exercise capacity , which prompted him to seek medical attention . echocardiography revealed significant aortic insufficiency with vena contracta of 7 mm and substantial dilatation of the ascending aorta up to 6 cm ; cardiac cavity dimensions were normal , and ejection fraction was estimated at 60% . the conducted physical examination revealed an enlarged liver as well as slight edemas on the lower extremities . the patient underwent a planned bentall - de bono procedure , conducted under general anesthesia . after the chest was opened via median sternotomy , a significantly thickened and calcified pericardium was revealed , covering the anterior and inferior cardiac surface , primarily over the right ventricle and the right atrium . on the inferior cardiac surface below the pericardium , there was a large reservoir filled with a whitish greasy substance , which was drawn for bacteriological and tuberculosis testing . dissecting the coronary openings , especially in the case of the right coronary artery , subsequently , heparin was administered , extracorporeal circulation was started with aortic arch and right atrial cannulation , and the heart was stopped with crystalloid cardioplegia under moderate hypothermia . jude valve conduit with the bentall - de bono method . after the patient 's body temperature was raised and reperfusion was performed , the extracorporeal circulation was stopped , and the heart resumed its hemodynamic function without any complications . after hemostasis was achieved , the chest was closed in a typical fashion , and drainage was maintained . the postoperative course was uneventful , and the bacteriological examination of the intraoperative material was negative . on the 7 day , the patient was discharged from the clinic in good general condition and was referred to the cardiac ward for further treatment . during a follow - up visit 4 weeks after the surgery , the patient reported a significant improvement in exercise capacity . non - specific viral and bacterial pericardial infections constitute the second most frequent group of diseases which may lead to constrictive pericarditis . the rising significance of radiotherapy due to neoplastic disease and previous cardiac surgery procedures in the etiology of this condition has been observed in recent years . tuberculotic etiology was not confirmed in our patient ; we suspect that the previous pericarditis was of non - specific infectious etiology , which consequently led to constrictive pericarditis within a relatively short period of time ( 2 years ) . the clinical signs presented by the patient , who complained mostly of a gradual but significant decrease in exercise capacity , were linked to aortic insufficiency ; as a result , the patient 's condition was not diagnosed even though imaging examinations ( lung x - ray ) described clear calcifications overlapping the cardiac shadow . even the peripheral edemas and the enlarged liver , signs more specific to constrictive pericarditis , did not raise any suspicion that another cause of the signs of circulatory insufficiency may exist alongside the evident pathology of the aorta and aortic valve . the presented case may serve as a warning , so that we do not focus exclusively on what is obvious in the clinical picture of the patient , but that we also take other , less common diseases into consideration , especially if basic tests provide us with indications concerning the diagnosis .

we report the case of a 54-year - old patient who was admitted to our institution for planned surgical treatment of an ascending aortic aneurysm and aortic valve insufficiency . a decision was made to perform an elective bentall - de bono procedure . constrictive pericarditis was revealed intraoperatively . the bentall - de bono procedure and the resection of calcified pericardium were performed successfully .

spontaneous rupture of the rectum is a rare occurrence with less than seventy cases being reported in literature . this has to be differentiated from rectal perforations which occur due to underlying pathology or direct rectal trauma . a spontaneous rupture of the rectum is defined as a rectal perforation in the absence of the above two conditions and is usually attributed to a sudden increase in the intra - abdominal and intra - rectal pressures . the traditional management is via a laparotomy with repair of the tear in the rectum with or without a covering colostomy . a total laparoscopic approach to rectal perforation we report an unusual case of a young boy who developed a spontaneous rupture of the rectum following a trivial fall which was successfully managed laparoscopically . a 17-year - old boy slipped in the bathroom and landed on his gluteal region . when he got up , he developed pain in his lower abdomen and noticed bleeding from his rectum along with mass . he was taken to a nearby hospital where they documented it to be prolapsed omentum which was digitally repositioned and was referred to our centre . on examination at our centre , about 3 h after his fall , he had tachycardia but was haemodynamically stable . a magnetic resonance imaging ( mri ) of the abdomen was performed which picked up a tear in the rectum about 23 cm just above the peritoneal reflection with the omentum plugging it [ figure 1a and b ] . on repeated questioning he was administered broad spectrum antibiotics and taken up for a diagnostic laparoscopy during which a 3 cm transverse tear in the left anterolateral aspect of the rectum just at the peritoneal reflection with the omentum plugging it was seen with minimal contamination [ figure 2 ] . the tear was repaired in two layers [ figure 3 ] using four ports , one 10 mm and three 5 mm . eight weeks later , a rectal contrast study was done which showed no leak of contrast , and a sigmoidoscopy to rule out an underlying pathology was normal following which his colostomy was closed . he was repeatedly questioned but denied any direct trauma , in any form that could have caused the injury . ( a ) axial sections of magnetic resonance imaging of the abdomen showing a tear in the rectum about 23 cm just above the peritoneal reflection with the omentum plugging it , ( b ) axial section of t2 fs images showing a bulky tail of the pancreas with mild fat stranding in the tail region laparoscopic view showing the tear in the rectum at the level of the peritoneal reflection along with the omentum that was plugging it intra - operative photograph showing the repaired rectal tear usually seen in older patients , cases as young as 6 years of age have been reported . in the younger patients , it is imperative to rule out a history of deliberate or accidental trauma as the cause . although termed as spontaneous , the pathogenesis of such rupture is a sudden increase in the intra - luminal pressures which can occur as a result of blunt abdominal trauma , lifting heavy weights , straining while passing stools , sneezing , micturation and even vomiting . in our case , we believe that the sudden contraction of the abdominal muscles and anal sphincters as a protective mechanism during the fall would have led to an increase in the intra - abdominal and intra - rectal pressures . at least one case of a spontaneous rupture occurring during sleep has been described . these ruptures occur in the presence of pre - disposing factors such as rectal prolapse , chronic constipation or the presence of a deep rectovesical / uterine pouch . the location of rupture in the rectum , it is almost always just proximal to the peritoneal reflection in its anterior aspect . only occasionally , a prolapse of omentum or small bowel as a presentation of rectal perforation has been reported . in the presence of peritoneal signs or prolapsed bowel / omentum , no imaging is really required to clinch the diagnosis and the patient proceeds to surgery . in our case , there were no peritoneal signs , and since we did not find a prolapsed omentum , notwithstanding the findings of the previous hospital , we proceeded to do an mri for the patient . this was also because we did not believe that a fall , so trivial , on the gluteal region could cause a rectal rupture . once suspected , an urgent surgery is needed and follows the principles of colorectal trauma . a laparoscopic approach to repair of rectal perforation is feasible , especially in cases which present early . one of the first reports of this approach was by najah and pocard in 2015 . the challenge in these cases would be to suture the rent which is just at the peritoneal reflection , deep in the pelvis and in a limited space the site of perforation is identified and the extent of contamination assessed . the prolapsed bowel or omentum , the rectal tear is repaired and a biopsy of the edges is warranted if an underlying pathology is suspected . although there are some reports that have avoided a colostomy in the absence of gross contamination , a covering stoma is usually done as this would avoid the devastating pelvic sepsis in the event of a leak from the repair . we believe that this may be necessary only in the presence of large tears with significant contamination or a faecal loading of the sigmoid colon proximal to the tear as it would render the diversion ineffective . some authors suggest obliteration of the deep pouch if present to prevent recurrences . an associated rectal prolapse needs to be dealt later , possibly at the time of colostomy closure . spontaneous rupture of the rectum is a rare occurrence . it can occur in younger age groups and even in the absence of significant trauma . in all such cases , one needs to diligently bring out a history of a history of rectal trauma , deliberate or otherwise .

spontaneous rupture of the rectum is a rare occurrence . a total laparoscopic approach to rectal perforation has only occasionally been reported . we report an unusual case of a young boy who developed a spontaneous rupture of the rectum following a trivial fall . a magnetic resonance imaging revealed a tear in the rectum at the peritoneal reflection with the omentum plugging it . he denied any history of rectal instrumentation or abnormal sexual activity . he had no history of constipation or rectal prolapse . the tear was repaired laparoscopically and a covering loop sigmoid colostomy was added . he made an uneventful post - operative recovery . spontaneous rupture of the rectum can occur in younger age groups and even in the absence of significant trauma . one needs to diligently bring out a history of rectal trauma . equally important is to rule out any underlying pathological condition . a laparoscopic approach is feasible , especially in early cases .

acinic cell carcinomas ( accs ) are uncommon salivary gland tumors that most commonly occur during the fifth and sixth decades of life . there are no clear characteristics of accs found on ct , mri and ultrasound imaging . a 62-year - old female was referred to the ent service as a result of an incidental finding on an mri scan of the brain . the mri revealed a mass in the right parapharyngeal space . the ct revealed an iso - hypodense solid cystic expansive mass in the right parapharyngeal space , measuring 3 cm in diameter . the mass was well defined , showing moderate enhancement following intravenous contrast injection ( fig . 1 ) . it displaced the anterior and medial internal carotid arteries and the internal jugular vein ( fig . lymphadenopathy was present in the carotid and bilateral submandibular spaces , measuring up to 14 mm in di - ameter . the mri revealed a solid cystic expansive mass in the right parapharyngeal space arising from the deep parotid lobe . 3 ) , and a hyperintense signal was seen on the stir image ( fig . a moderate peripheral enhancement was observed after an intravenous injection of paramagnetic contrast material ( fig . the head and neck tumor board recommended complementary radiation therapy based on the previous surgical decision of incomplete tumor resection . accs are a type of uncommon salivary gland tumors that were considered benign . however , in 1953 , buxton demonstrated their ability to metastasize and recur locally . the majority of accs ( almost 80% ) occur in the parotid gland , and approximately 1317% involve the minor intraoral salivary glands [ 2 , 3 ] . the female to male ratio is approximately 1.5:1 , and the age distribution is fairly even from the second to the seventh decades of life , with a slight peak in the fifth and sixth decades [ 4 , 5 ] . histopathologically , the tumor is well circumscribed with a distinct capsule , may be solid or cystic and distinct morphological growth patterns are seen . these are described as solid , microcystic , follicular and papillary cystic tumors [ 7 , 8 ] . they are composed of diverse cell types and include acinic cells , vacuolated cells , intercalated cells , nonspecific glandular cells and clear cells . large lobules or nests of tumor cells with little intervening stroma are characteristics [ 9 , 10 , 11 ] . the histologic grading of acinic cell carcinomas is controversial , and unlike clinical staging , histomorphology has not proven to be reliable in predicting its behavior . no known imaging characteristics of parotid gland accs have been found on ct , mri , or ultrasound imaging . the diagnosis of accs using only imaging studies is complex due to its great radiologic similarity with benign tumors . ultrasonography , which is an easy noninvasive and widely available test , is useful in evaluating tumor size , location , and nature . in addition , it is also used to perform ultrasound - guided fine needle aspiration biopsies . a ct scan usually demonstrates a slight contrast enhancement and may be appropriate for the evaluation of tumor size , involvement , relationship to facial nerves , other structures and distant metastasis . accs usually demonstrate nonspecific signal intensity patterns on mri , and low t1 and t2 signals can be detected on some images . the signals correlate with the histology , suggesting vascularity , hemosiderin deposition , fibrosis and calcification within the tumor itself . the treatment of choice for accs is the complete surgical excision of the tumor by intraoral or extraoral approach . radiation therapy should be considered in cases of poor prognosis , positive surgical margins , stage t3 or t4 tumors , high histologic grade , multiple positive lymph nodal involvement and vascular or perineural invasion . cure rates have been found to be 76 , 63 , and 55% at the 5- , 10- , and 15-year marks , respectively . distant metastases ( liver , lungs and orbit ) have been reported between 7 and 29% . the recurrence rate for these tumors ranges from 30 to 50% , and when the deep lobe of the parotid gland is reached , local recurrences are higher than in superficial tumors ( 72 and 18% , respectively ) [ 11 , 14 , 15 ] . the 5-year local regional control rate in salivary gland cancer patients managed exclusively by radiation therapy ( inoperable tumors , refusal of surgery or incompletely resectable tumors ) ranged from 56 to 70% at 5 years , and from 40 to 59% at 10 years . local regional control rates following surgery and postsurgical radiotherapy ranged from 88 to 91.4% at 5 years , and were 83 and 67% at 10 and 20 years , respectively . accs are a type of uncommon salivary gland tumors with the ability to metastasize and recur locally . the diagnosis of acc using only imaging studies is complex due to its great radiologic similarity to benign tumors . radiation therapy should be considered in cases with positive surgical margins , multiple positive lymph nodes and vascular or perineural invasion .

we report on a patient who was referred to the ent service following an incidental finding on an mri scan of the brain . it revealed a mass in the right parapharyngeal space , and additional imaging confirmed the presence of a solid cystic expansive mass with moderate enhancement following contrast media injection . the patient was treated with a total parotidectomy followed by radiotherapy . currently , the patient is disease - free without any complications .

we obtained data regarding temporal trends in fri incidence from military sentinel surveillance , which has been monitoring weekly fri rates since october 2011 . monthly numbers of patients with pneumonia ( inpatients , outpatients , and emergency room patients ) were extracted from a computerized data warehouse that stores data from all military hospitals . we identified pneumonia cases by using the international classification of diseases and related health problems , 10th revision , codes j12j18 . the influenza season , which starts in october and ends the following may , was used as a surrogate for the hadv season in this study . the trends in fri rates showed an unusual surge during the 201415 influenza season ( figure 1 , panel a ) . the fri rate increased for 15 weeks in the 201415 season , compared with 10 weeks in the 201213 season and 5 weeks in the 201314 season . peak fri rate in the 201415 season ( 10.4% ) was higher than rates in the preceding 2 seasons ( 4.7% and 7.5% ) . the numbers of pneumonia cases in 201415 and 201516 seasons were 3,140 and 3,145 patients , respectively , a 191% increase from the mean number during 3 preceding seasons . a ) weekly febrile respiratory illness ( fri ) rate ( solid line ) and monthly number of pneumonia patients ( dashed line ) in the south korea military , 20112016 . b ) positive rate of human adenovirus from respiratory specimens ( red line ) and the number of respiratory virus pcr requested ( blue bar ) from a tertiary military hospital , south korea , 20142016 . the rate and number for each month a multiplex real - time pcr for identifying 15 viruses from respiratory specimens was introduced at the armed forces capital hospital , the only tertiary hospital in the south korea military health care system ( detailed methods in technical appendix ) . a total of 1,484 nonduplicate specimens were tested by the end of may 2016 ( figure 1 , panel b ; technical appendix table 1 , figure ) . hadv was identified in 490 ( 33.0% ) of total specimens , and it accounted for 79.7% ( 282/354 ) and 53.2% ( 150/282 ) of positive results in the 201415 and 201516 seasons , respectively . we reviewed the demographic and clinical information of 878 military patients with fri or pneumonia who were tested for respiratory viruses from october 2014 through may 2016 ( tables 1 , 2 ) . soldiers of lower rank were markedly more likely to infected with hadv ; soldiers serving in the air force were less likely . patients who had been referred from other hospitals were twice as likely to be hadv - infected than patients who visited the armed forces capital hospital directly . rhinorrhea , sore throat , diarrhea , and nausea / vomiting were more common in patients with hadv infection . the proportion of patients with pneumonia and the hospitalization rate did not differ between those with and without hadv infection . however , hadv - infected patients had a significantly higher risk of requiring intensive care or mechanical ventilator support . in the hadv - infected group , 8 patients required intubation and 1 died ; no one in the noninfected group died or required intubation . length of hospital stay was also significantly longer among those the hadv - infected group than among those in the noninfected group ( 12.6 vs. 9.4 days ) . hadv , human adenovirus ; na , not available ; or , odds ratio ; pfc , private first class . hadv , human adenovirus ; na , not available ; or , odds ratio.n = 877 . we conducted molecular typing by the sequencing of hexon and fiber genes with 74 hadv - positive respiratory specimens collected from march through june 2016 ( methods and general characteristics of the patients are available in the technical appendix table 2 ) . among them , 49 samples were successfully sequenced ( genbank numbers in online technical appendix ) . phylogenetic analyses showed that all 49 hadv strains from south korea clustered with hadv-55 strains from china , singapore , taiwan , spain , and the united states ( figure 2 ) . phylogenetic analysis of human adenoviruses based on the partial nucleotide sequences of hexon ( a ) and fiber ( b ) genes , south korea , 2016 . phylogenetic trees were generated by the neighbor - joining method , using the kimura 2-parameter method . the percentage of replicate trees in which the associated taxa clustered together in the bootstrap test ( 1,000 replicates ) are shown next to the branches . hadv is a well - known major cause of fri in the military , accounting for > 50% of fri and pneumonia cases in military recruits ( 1 ) . our study also confirmed the predominance of hadv , which was identified in 49.1% of specimens from patients with fri or pneumonia . these findings are similar to those of previous studies from south korea and the united states ( 9,10 ) . the most notable finding of our study is the emergence of hadv-55 in the south korea military . hadv-55 is a novel type that has been associated with a severe clinical course and death in healthy young adults ( 7,8 ) . we also found that hadv infection was associated with intensive care , mechanical ventilator support , and longer hospital stay . in addition , we found that the only patient who died was hadv infected . from a molecular perspective , hadv-55 is a novel type with a hexon gene recombination between hadv-11 and hadv-14 ( 11 ) . phylogenetic analysis by using the hexon and fiber gene sequence of 49 strains collected in our study showed that they clustered with previously reported hadv-55 strains . spread of infection of traditionally military - associated hadv types into civilians has been recently reported in the united states and china ( 3,12,13 ) . thus , surveillance of hadv types among both military and civilian populations is warranted ; such measures are being implemented by the us centers for disease control and prevention ( atlanta , ga , usa ) ( 12 ) . first , our findings may not be generalizable due to the retrospective nature of the study . however , the military health system in south korea provides healthcare exclusively to all military personnel ; therefore , epidemiologic information gathered from our surveillance is accurate and comprehensive . second , we conducted molecular typing with samples collected from february 2016 , which was substantially later than the onset of the epidemic . however , hadv-55 had already been identified in a case series from our center during june 2014may 2015 ( 8) . because evidence shows that hadv-55 has been already circulating since early 2014 , we believe we can assume that hadv-55 was the causative agent of the outbreak described in this study . previously , the hadv typing study conducted in 2007 reported hadv-7 as the most prevalent type ( 14 ) . lack of continuous surveillance makes it difficult to estimate exactly when this novel type was introduced into south korea . further genomic analysis of the collected samples and enhanced surveillance , including of civilian populations , would provide more information on the epidemiology of hadv infection . detailed methods of the study of febrile respiratory illness associated with human adenovirus in south korea military , 20142016 .

an outbreak of febrile respiratory illness associated with human adenovirus ( hadv ) occurred in the south korea military during the 201415 influenza season and thereafter . molecular typing and phylogenetic analysis of patient samples identified hadv type 55 as the causative agent . emergence of this novel hadv necessitates continued surveillance in military and civilian populations .

endometrial cancer is the most common gynecologic malignancy in developed countries , and its incidence has more than doubled over the last 10 years in korea.1 clear cell carcinoma typically occurs in the ovaries , is rarely seen in the cervix or vagina , and very rarely occurs in the endometrium , accounting for less than 3% of all endometrial cancers.2 clear cell carcinoma is characterized by abundant cytoplasm like in clear cell renal cell carcinoma and clear hobnail cells,3 and it is presumed that clear cell carcinomas are of mllerian duct origin.3 since the study reported by herbst and scully4 in 1970 , the association of exposure to diethylstilbestrol ( des ) or other nonsteroidal follicle stimulating hormones has been described . the authors experienced a rare case of a 66-year - old woman who visited our hospital due to vaginal bleeding without a specific history and we report the case with a brief review of literature . a 66-year - old woman ( 141 cm , 46 kg , body mass index [ bmi ] 23.2 kg / m ) , gravida 3 was admitted to our obstetrics & gynecology hospital , and her chief complaint was vaginal spotting which occurred a month ago . she had hypertension and took medical therapy . under the impression of endometrial mass , tumor marker , complete blood cell count , cancer antigen 125 ( ca-125 ) is 22.5 u / ml , usg image showed large mass in endometrial cavity and hysteroscopic biopsy result is clear cell carcinoma ( fig . 1 ) . positron emission tomography ( pet)-computed tomography ( ct ) image showed diffuse heterogeneous hypermetabolic lesion in the endometrial cavity and focal hypermetabolic lesion between the right aspect of rectum and uterus ( fig . laparoscopic assisted hysterectomy with bilateral salpingo - oophorectomy ( bso ) , pelvic lymph node dissection and rectal shelf mass removal was done ( fig . pathologic report was clear cell carcinoma of the endometrium , depth of invasion was 0.2 cm out of 0.5 cm thickness , rectal shelf mass was clear cell carcinoma . postoperative radiotherapy was performed and the patient is currently under outpatient follow - up with subclinical conditions . endometrial cancer is the seventh most common type of cancer among women , accounting for 3.9% of all cancers and 1.7% of all cancer deaths in women.5 although prevalence rates vary according to studies , clear cell carcinoma represents about 3% of all endometrial carcinomas.67 clear cell carcinomas developing in any of the female reproductive organs are identical in cytological variants , histological patterns and ultrastructure . since the descriptive term clear cell carcinoma originating from the mllerian duct was first introduced by scully and barlow in 1967 , it is generally accepted that clear cell carcinoma is developmentally derived from the mllerian duct.8 although the exact cause or pathogenesis has not yet been clarified , multiple risk factors have been suggested including des use during pregnancy , genetic predisposing factors , instability of microsatellite repeat sequences , human papilloma virus infection , overexpression of b - cell lymphoma 2 ( bcl-2 ) protein , variation of the p53 gene , and external or environmental factors.9 however , clear cell carcinoma of the endometrium like the case in this study is rarely seen , and has not been detected in children of mothers who took des in some previous studies.10 the present study was unable to identify the exact cause associated with des exposure or genetic predisposing factors . most common symptoms are vaginal discharge or vaginal bleeding lasting for several weeks or years , and most underling disease are hypertension , diabetes , obesity or other symptom may be associated . although these symptoms align with the triggers of endometrial cancer , this finding has not yet been confirmed . clear cell cancer is often diagnosed by sonographic appearance of endometrial tumors or endometrial biopsy when the above symptoms are associated . photopulus classified 22 cases of clear cell carcinoma of the endometrium according to the extent of involvement , and their clinical stages were stage i , 23% ; stage ii , 50% ; and stages iii and iv , 27%.6 in the case of this study , although invasion was confined to less than half of the endometrium , stage iii was confirmed as clear cell carcinoma was found in tumors of the rectal shelf having high pet signal . like most other endometrial cancers , total laparoscopic hysterectomy ( tlh ) , bso and radiation therapy are considered as treatment options , and only tlh and bso can be performed or radiation therapy can be added for treatment of early - stage endometrial clear cell carcinoma . the addition of radiation therapy is not the exact protocol , and its definite roles still remain controversial . this is because that this therapy is used infrequently and radiation doses and methods vary according to institutions . a recent study on the addition of chemotherapy addressed that the addition of radiation therapy had no influence on survival rate and chemotherapy was more effective.2 clear cell carcinoma is characterized by late recurrence compared to other carcinomas . recurrences typically occur 17 months after primary treatment , and are seen in about 21% of patients 5 years later.11 this type of tumor is suggested to have a relatively poor prognosis compared to other endometrial cancers according to our literature review.3610 photoplus proposed that poor prognosis was attributable to a higher frequency of poorly - differentiated tumors in clear cell carcinoma.6 for this reason , clear cell carcinoma is currently classified as grade 3 ( poorly differentiated ) , and surgical procedure and radiation therapy or chemotherapy should be performed . however , this type of carcinoma shows poorer prognosis compared to the prognosis of grade 3 endometrial carcinomas , and the 5-year survival rates range from 40% to 54% between those of stage iii - iv endometrioid cancer and clear cell carcinoma.2 thus , active and radical treatment is warranted . the authors experienced a clear cell carcinoma of the endometrium ( stage iiib ) , and we report the case with a brief review of literature .

endometrial cancer is the most common gynecologic malignancy in developed countries . clear cell carcinoma typically occurs in the ovaries , and very rarely occurs in the endometrium ; it accounts for less than 3% of all endometrial cancers . it is presumed that clear cell carcinomas are of mllerian duct origin , and an association with exposure to diethylstilbestrol ( des ) or other nonsteroidal follicle stimulating hormones has been described . we report a case of a postmenopausal woman who presented with vaginal bleeding without a specific medical history . under the impression of an endometrial mass , we performed a laparoscopic operation . pathologic results showed clear cell carcinoma of the endometrium . depth of invasion was 0.2 cm out of a 0.5 cm total thickness , and the rectal shelf mass was clear cell carcinoma . we report the case with a brief review of the relevant literature .

osteosarcoma constitutes a small percentage of rare malignant non - epithelial neoplasms of the thyroid , and has been reported in only several dozen cases . one of the conclusive tests is immunohistochemical staining , which excludes epithelial differentiation of the tumour . we present a case of a female patient who underwent surgery due to a fast - growing tumour of the right thyroid lobe , with an unexpected final diagnosis . a female patient , aged 76 , was qualified for surgical removal of a tumour of the right thyroid lobe , within the substernal goitre , due to its rapid growth over a period of two months . the estimated size of the goitre , measured in cross - section through both lobes , was 9 6 cm . no qualifying biopsy was carried out before surgery , opting for radical strumectomy . with an incision at the base of the neck , having cut through the short muscles , the thyroid lobes were identified . the left lobe was the size of a plum , with nodular reconstruction , whilst the right lobe was the size of a large fist , expanding down the lower pole , past the sternum and pushing the trachea to the left ( approx . having freed the lateral parts of the lobes of the thyroid from the trachea , the small pyramidal lobe was removed . the early post - operative period was complicated by acute respiratory failure requiring intensive care treatment . the impairment of vocal cord mobility resulted in the necessity to insert a tracheotomy tube on the laryngology ward . the evaluation of the single - piece surgical material revealed a nodular tissue structure , with an irregular external surface and dimensions of 9 6 7.5 cm , without separated thyroid lobes . a correct thyroid texture was observed only in the left lobe , with a diameter of approx . the remaining part of the material , corresponding to the right thyroid lobe of a diameter of approx . 7 cm , was a greyish - white tumour of increased density , with extensive calcified foci . a high proliferative activity of the tumour was observed ( mib-1 reaction ) in the form of a positive reaction in approx . staining h&e , magnification 100 a month after the operation , in quite good general condition , the patient reported for oncological treatment . the postoperative scar at the base of the neck was fully healed , with no infiltration . the peripheral lymph nodes were slightly enlarged , with small oedema of the subcutaneous tissue . computed tomography with local staging dab , magnification 100 the reconsultation revealed negative staining with cytokeratin , and positive with vimentin , thereby confirming the mesenchymal origin of the tumour , with the final diagnosis being primary thyroid osteosarcoma . taking into consideration the histopathological diagnosis , the extremely low radiation sensitivity of the tumour , the patient 's age , the radical surgical treatment and persisting respiratory failure , radiotherapy was rejected in favour of further follow - up . osteosarcoma , the most frequent malignant bone tumour , may also occur in various organs outside the osteoarticular system . however , these are rare incidents , described in single cases and involving the heart , lung , prostate , urinary bladder and breasts [ 14 ] . 5% ) , especially in the thyroid , where , so far , it has been diagnosed in 28 cases , and in only two cases with fine - needle biopsy [ 6 , 7 ] . this tumour is characterised by fast growth and requires quick surgical intervention due to pressure - related symptoms of dyspnoea . poorly differentiated thyroid carcinoma and anaplastic carcinoma with osseous metaplasia , the differentiating diagnosis should additionally involve medullary thyroid carcinoma , spindle epithelial tumour with thymus - like differentiation ( settle ) , synovial sarcoma and metastasis of osteosarcoma . the tumour is characterised by a variety of histological images , from extensive monomorphic infiltrations of primary mesenchymal cells with high mitotic activity , in which it is generally difficult to observe osteoplasia , through spindle - cell and epithelioid areas with accompanying giant tumour cells with multiple nuclei . the presence of bone matrix and mature bone trabeculae is a frequent component of the tumour . cytological material is usually rich in cells and appears in compact tissue fragments with slight discohesion , frequently showing a high degree of cell polymorphism . there are spindle cells and epithelioid cells with varying amounts of cytoplasm , from trace to abundant , frequently with the presence of intracytoplasmic vacuoles . the nuclei of these cells are usually elongated or oval - shaped , with coarse - grained chromatin and quite distinct chromocentres , often eccentric as in plasmocytes . in the background , there are usually binuclear and polynuclear cells with considerable polymorphism , some of which resemble osteoclasts [ 6 , 7 ] . beside cells , there may be masses partially composed of fibrillar structures , adjacent to tumour cells . thus , the cytological image may be ambiguous and requires histopathological confirmation , supported by immunohistochemical examinations . immunohistochemical examinations with vimentin ( positive reaction ) , cytokeratin , ttf1 , calcitonin , chromogranin , synaptophysin and s-100 ( negative staining ) are of decisive significance . the clinical course is characterised by rapid progression , which , within weeks , leads to death in the mechanism of lung metastasis or the superior vena cava syndrome as a result of extensive infiltration of the perithyroid tissue and large carotid vessels .

a rare localization of primary osteosarcoma is presented . a woman aged 76 years was operated on for rapid growth of thyroid right lobe tumour . histopathology showed anaplastic cancer with numerous foci of osseous metaplasia , negative with thyroglobulin , calcitonin , synaptophysin and chromogranin . a high proliferative activity of the tumour was observed ( mib-1 reaction ) in the form of a positive reaction in approx . 40% of the tumour cell nuclei . the tumour stage was evaluated as pt4anxmx according to the tnm scale . the reconsultation revealed negative staining with cytokeratin , and positive with vimentin , thereby confirming the mesenchymal origin of the tumour , with the final diagnosis being primary thyroid osteosarcoma . taking into consideration the histopathological diagnosis , the extremely low radiation sensitivity of the tumour , the patient 's age , the radical surgical treatment and persisting respiratory failure , radiotherapy was rejected in favour of further follow - up . the patient remains under oncological and endocrinological care .

nevertheless , the vast majority of all interactions are not yet identified and are subject to current research ( 1 ) . an important step towards the mechanistic descriptions of such interactions is the 3d structural information of macromolecules . however , complexed proteins are difficult to co - crystallize and the number of publicly available x - ray structures in the protein data bank ( pdb ) ( 2 ) is very limited ( 3 ) . thus , a structure - based analysis of particular interacting macromolecules is often only possible by using docking models . for systematic analyses the problem of the low number of protein protein complexes such types of interfaces often exhibit a similar behaviour like those of interacting proteins ( 4 ) . for instance , knowledge - based potential functions that represent the co - occurrence of certain residues might be similar for contacts between domains of a single chain as well as contacts between proteins . another approach is the utilization of the interfaces between domains or chains to detect structural similarities to binding sites of interacting proteins . consequentially , some structure - based databases exist that focus on the interacting parts of proteins . scoppi ( 6 ) , snappi ( 7 ) and pibase ( 8) classify interfaces between domains , the domain information was retrieved from scop ( 9 ) , cath ( 10 ) or pfam ( 11 ) . since they depend on domain definitions extracted from secondary databases especially structures solved during the last few years are normally not yet classified ( 12 ) . hotsprint ( 13 ) focuses on conserved residues in chain contact sites and is regularly updated but domain information is ignored . dockground ( 3 ) comprises the so far most comprehensive data set of interacting proteins and chains respectively . the excellent database also provides user defined data sets of the associated unbound protein - binding sites . although each application is useful to enlighten questions the database is specialized for , a comprehensive web resource that combines all the different kinds of interfaces between macromolecules is not available . furthermore , the database should be characterized by regular updates and the opportunity to download appropriate data sets . to overcome this lack we developed jail , a structure - based interface library for macromolecules . currently , the database contains more than 184 000 interfaces that are composed of four different fractions : 81 000 interfaces between domains classified by scop , 76 000 interfaces between different protein chains , 8000 interfaces between proteins and nucleic acids and 19 000 interfaces which were calculated based on the assumed biological units . the interfaces result from the evaluation of 52 000 different asymmetric unit files as well as the associated biological unit files provided by the pdb . an interface is defined as those atoms of a chain or domain that are located within a range of 10 around the c-atoms of the interacting counterpart . in the case of nucleic acids the backbone ( p / c4)-atoms were considered . each binding site has to consist of at least five c-atoms as the case may be backbone atoms of the nucleic acids . assumed biological units were calculated based on the first two models build up by reflection of the unit cells . the pdb - ids of nucleic acids containing structures were retrieved from the nucleic acid database ( 15 ) . all chains of the database were sequentially clustered using the regular updated lists provided by the pdb calculated by the cd - hit program ( 16 ) . thus , it is possible , to select interfaces of proteins , which are similar in sequence to each other as well as to download non - redundant data sets based on protein sequences . structural clustering was implemented by the selection of representative interfaces of each family family or superfamily superfamily contact between domains classified in scop . a protein can be identified by a detailed search form that allows searches by pdb - id , protein name , ec - number , uniprot accession number or scop - id . an implemented full text search allows the screening to the full header information of the structure - file as well as the scop domain descriptions . visualization of the interfaces was implemented by pre - generated thumbnails of each interface and on the other hand by the interactive protein visualizer jmol ( http://www.jmol.org ) . structurally , clustered interfaces based on the scop domain definitions as well as sequentially clustered interfaces based on protein chain clustering are separately retrievable but can also be combined . parameters like the scop - hierarchy ( family / superfamily ) or the sequence identity level ( 50% , 70% , 90% and 95% ) are selectable . the database is automatically updated six times a year . one of them is the comparative study of molecular recognition of nucleic acids and proteins . for instance , experimental evidence exists , that proteins mimic nucleic acids to usurp the role of interacting macromolecules ( 17,18 ) . shape comparisons of different kinds of interfaces ( protein protein / protein nucleic acids ) may help to identify cases of molecular mimicry . a matter of particular interest in this context is the identification of protein domains that interact with other proteins as well as with nucleic acids . figure 1 shows an example of a search for such a case by using the implemented show related proteins-option . figure 2 shows the resulting interfaces of a fab - fragment in complex with an enzyme . ( a ) entry of pdb - id 2fr4 . the highlighted link ( show related proteins ) yields a list of homologous proteins . the last entry is pdb - id 2j88 and is shown in figure 2b . figure 2.two similar fab - fragments ( sequence identity > 95% ) which interact with different kinds of macromolecules . the complexes were identified with the homology search option ( see figure 1 ) of jail . ( a ) fab - fragment in complex with a stem - loop dna ( pdb - id : 2fr4 ) . ( b ) a monoclonal igg fab - fragment in complex with hyaloronidase ( pdb - id : 2j88 ) . two similar fab - fragments ( sequence identity > 95% ) which interact with different kinds of macromolecules . the complexes were identified with the homology search option ( see figure 1 ) of jail . ( a ) fab - fragment in complex with a stem - loop dna ( pdb - id : 2fr4 ) . ( b ) a monoclonal igg fab - fragment in complex with hyaloronidase ( pdb - id : 2j88 ) . deutsche forschungsgemeinschaft ( dfg sfb-449 ) ; the international research training group on genomics and systems biology of molecular networks ( grk1360 ) ; german federal ministry of education and research ( goforsys grant nr . this work is licensed under a creative commons attribution - noncommercial - share alike 3.0 license .

the increasing number of solved macromolecules provides a solid number of 3d interfaces , if all types of molecular contacts are being considered . jail annotates three different kinds of macromolecular interfaces , those between interacting protein domains , interfaces of different protein chains and interfaces between proteins and nucleic acids . this results in a total number of about 184 000 database entries . all the interfaces can easily be identified by a detailed search form or by a hierarchical tree that describes the protein domain architectures classified by the scop database . visual inspection of the interfaces is possible via an interactive protein viewer . furthermore , large scale analyses are supported by an implemented sequential and by a structural clustering . similar interfaces as well as non - redundant interfaces can be easily picked out . additionally , the sequential conservation of binding sites was also included in the database and is retrievable via jmol . a comprehensive download section allows the composition of representative data sets with user defined parameters . the huge data set in combination with various search options allow a comprehensive view on all interfaces between macromolecules included in the protein data bank ( pdb ) . the download of the data sets supports numerous further investigations in macromolecular recognition . jail is publicly available at http://bioinformatics.charite.de/jail .

the patient presents with the characteristic appearance of external genitalia with or without urinary incontinence . female hypospadias is an infrequent birth deformity consisting in the fissure of the posterior ( lower ) wall of the urethra . female hypospadias is characterized by shortening of the urethra and ectopia of the external urethral opening . history did not reveal any evidence of urinary tract infection , trauma or operative interventions . the child was voiding good quantity of urine . to quantify her incontinence it was around 45 pads / day . note the bifid clitoris with deficient dorsal wall x - ray pelvis showed a defect in symphysis pubis [ figure 2 ] . x - ray pelvis shows defect in the symphysis pubis a vertical parallel incision was taken along the urethra [ figure 1 ] . the triangular area over the bifid clitoris was marked , and the raw area was sutured to obliterate the dead space . postoperative ultrasonography was done at 6 weeks and 12 weeks with bladder capacity of 140 ml and 160 ml , respectively . a 7-year - old girl presented to us with complaints of only true urinary incontinence . on examination of external genitals , urethral meatus was caudally directed , and continuous leaking of urine was noted . was done prior to a definitive procedure revealed short urethra ( < 1 cm ) . both the ureteric orifices were normal , and bladder was normal with a capacity around 120 ml . on insertion of 12 french foleys catheter , the bulb of the catheter could be easily visualized [ figure 3 ] . the wide open urethral plate above the meatus and below the clitoral opening was mobilized by taking an inverted u incision [ figure 3 ] . after careful mobilization of the urethra , it was tabularized over 12 french foleys catheter using absorbable suture [ figure 4 ] . the neometus formed after the reconstruction was sutured to the vagina after mobilization of the periurethral tissue . note : that the bulb of the catheter is visualized suggestive of short urethra urethra being reconstructed over a 12 french foley 's catheter history did not reveal any evidence of urinary tract infection , trauma or operative interventions . the child was voiding good quantity of urine . to quantify her incontinence it was around 45 pads / day . note the bifid clitoris with deficient dorsal wall x - ray pelvis showed a defect in symphysis pubis [ figure 2 ] . x - ray pelvis shows defect in the symphysis pubis a vertical parallel incision was taken along the urethra [ figure 1 ] . the triangular area over the bifid clitoris was marked , and the raw area was sutured to obliterate the dead space . postoperative ultrasonography was done at 6 weeks and 12 weeks with bladder capacity of 140 ml and 160 ml , respectively . a 7-year - old girl presented to us with complaints of only true urinary incontinence . on examination of external genitals , urethral meatus was caudally directed , and continuous leaking of urine was noted . was done prior to a definitive procedure revealed short urethra ( < 1 cm ) . both the ureteric orifices were normal , and bladder was normal with a capacity around 120 ml . on insertion of 12 french foleys catheter , the bulb of the catheter could be easily visualized [ figure 3 ] . the wide open urethral plate above the meatus and below the clitoral opening was mobilized by taking an inverted u incision [ figure 3 ] . after careful mobilization of the urethra , it was tabularized over 12 french foleys catheter using absorbable suture [ figure 4 ] . the neometus formed after the reconstruction was sutured to the vagina after mobilization of the periurethral tissue . catheter was kept for 11 days . the child was continent and voided adequate amount of urine with good flow . note : that the bulb of the catheter is visualized suggestive of short urethra urethra being reconstructed over a 12 french foley 's catheter early diagnosis and early treatment play an important role to reduce the psychological and psychosocial problems and prevent urinary incontinence . the importance of a careful examination should be emphasized , especially in female patients with chronic wetting of unknown cause . davis has divided the female epispadias into three degrees : ( 1 ) patulous urethra ( milder form ) ; ( 2 ) urethra dorsally split ( intermediate degree ) and ; ( 3 ) cleft involving the whole urethra and bladder neck ( severe degree ) . the genital defect is characterized by bifid clitoris and mons is depressed in shape and coated by a smooth area of skin . these children should undergo a complete radiological evaluation to identify and rule out other causes of incontinence . the role of the urodynamic study is indefinite but would help to justify the poor outlet resistance . cystourethroscpy is required to access urethral length , bladder capacity and position of the ureteric orifice . female hypospadias is characterized by shortening of the urethra and ectopia of the external urethral opening . this deformity is rarely diagnosed because physicians are not well - informed about female hypospadias . these include : low vaginal ectopia of the external urethral openinghigh vaginal ectopia of the external opening of the urethraurovaginal ( vesicovaginal ) fusion of the neck of the urinary bladder with vagina accompanied with enuresisurogenital sinus in females ( ectopia of the external urethral opening in the urogenital sinus ) . low vaginal ectopia of the external urethral opening high vaginal ectopia of the external opening of the urethra urovaginal ( vesicovaginal ) fusion of the neck of the urinary bladder with vagina accompanied with enuresis urogenital sinus in females ( ectopia of the external urethral opening in the urogenital sinus ) . all the variants of female hypospadias must be surgically corrected as transposition of the external opening of the urethra from the vagina on the perineum under the clitoris in chronic urethritis . the second case mentioned above mimics sinus urogenitalis , but is not so because the majority of children born with a urogenital sinus have genital ambiguity . at the same time , in the second case there was a healthy ventral urethral plate which is suggestive that it is a case of female hypospadias rather than sinus urogenitalis . diagnosis of female urethral anomalies and its treatment possess a great challenge to pediatric urologists . the objectives of surgical repair include achievement of urinary continence with preservation of the upper urinary tracts and the reconstruction of functional and cosmetically acceptable genitalia .

female urethral anomalies such as epispadias and hypospadias have been rarely reported in the literature . clinical diagnosis of female urethral anomalies is significant for management . diagnosis may be missed leading to mismanagement of the clinical condition . herein , we report a case of female epispadias and hypospadias managed by us at tertiary care center .

short scalp hair is a presenting feature for many syndromes which are mostly genetic or most commonly associated with other abnormalities . short anagen syndrome ( sas ) is an uncommon condition characterized by short hair and increased hair in telogen phase . the condition is unassociated with hair breakage , total hair loss , and any other serious association . we report here a case of 30-year - old women with a short scalp hair diagnosed as sas after clinical evaluation and microscopic examination of the hair shaft in a trichogram . an apparently healthy looking 30-year - old woman presented to our clinic with a short , spare , and pigmented hair , since birth , which never grew longer than nape of neck , apparently she never had a haircut . she had a scanty faint color hair ; otherwise , she was healthy and her development had been normal . the maximum scalp hair length was 17 cm at frontal but most of hair showed average growth of 5 - 6 cm with decrease density , fine texture and was pigmented but no bald areas were seen [ figure 1 ] . examination of the eyebrows , eyelashes , teeth , and nails was within normal limits . microscopic examination of the hair shaft in the trichogram revealed increased numbers of telogen hairs with normal hair shaft and tapering ends indicating uncut hairs . the anagen to telogen ratio was significantly reduced ( 2:1/64:36 ) ( as against 90:10 normally ) . after shaving a small area of the scalp and following up the growth rate of the shaved hair histologic examination of biopsy from the parietal region revealed sparse superficial perifollicular infiltrate and normal number of vellous and intermediate follicles . a standardized questioner dermatology life quality index ( dlqi ) was used to assess the impact of this condition on quality - of - life ( qol ) of patient . a score of 6 indicated moderate effect on qol . the incidence of sas is poorly documented in all reports , patients are caucasian and usually have fine blond hair . the diagnosis of sas can be made by presence of normal hair shafts , unbroken hair , and decreased number of hair in anagen . the case was differentiated from other differential conditions such as loose anagen syndrome , trichodental syndrome , congenital hypotrichosis , and five functional sub - types of telogen effluvium , depending on the number of hairs in anagen phase , age of onset , and the histopathologic examination . the inability to grow longer hair can be traumatic to young patients , more so to indian women of marriageable age where the concept of beauty incorporates longer hair . our patient never grew hair below nape of neck and had to use a wig for last 18 years . she showed an overall score of 6 on dlqi indicating not very significant affection of qol , but her score of 3 out of 6 under leisure and personal relationships headings was high indicating a significant affection of quality - of - life , substantiated by fact that she wore a wig for last 18 years . our patient is skin type v indian woman of age 30 with black hair . to our knowledge , this is the first report of sas in an indian patient . it is diagnosed clinically with pathognomonic criteria of normal hair shaft with short growth phase , decreased number of hair in anagen phase , and hair loss not explained by hair breakage . further research is needed to establish diagnostic criteria and better treatment modalities for this condition .

short anagen syndrome ( sas ) is a recently described entity characterized by idiopathic shortening of anagen phase . the condition is poorly described in indian population . we describe the 1st indian case with clinico - pathological features of a 30-year - old woman diagnosed with sas . case was diagnosed on the basis of clinical examination , trichogram , microscopic examination of the hair shaft , histopathologic examination of scalp , and measurement of hair growth rate .

thrombotic thrombocytopenic purpura ( ttp ) is a microangiopathic hemolytic disorder that is induced by marked reduction in the level of von willebrand factorcleaving protease , adamts13 . it is characterized by development of microthrombi in small blood vessels leading to consumptive thrombocytopenia and hemolytic anemia . although ttp is commonly associated with neurologic and renal dysfunction 1 , 2 , cardiac involvement is rarely seen . unfortunately , most cases with ttp with cardiac involvement are diagnosed on autopsy where a massive myocardial necrosis is usually evident 4 . in this report , we present a case of ttpinduced myocardial infarction treated successfully with plasma exchange . a 59yearold man with history of hypertension , coronary artery disease ( cad ) , and medications noncompliance presented to the emergency room with a 2day history of chest pain , lethargy , and headache . the chest pain was retrosternal , pressurelike , increasing in severity , radiating to the left arm , and associated with diaphoresis . the patient was supposed to be on low dose aspirin , metoprolol tartrate , and atorvastatin . laboratory studies showed elevated troponin t at 0.33 ng / ml , low platelet count at 9,000/l , low hemoglobin ( 6.4 gm / dl ) , and elevated creatinine of 3.3 mg / dl . the patient prothrombin time , international normalized ratio , and partial thromboplastin time were within normal limits . ekg . due to the severity of chest pain , he was started on nitroglycerin infusion and transferred to the cardiac intensive care unit . further workup revealed elevated lactate dehydrogenase at 857 units / l , undetectable haptoglobin level and elevated indirect bilirubin level at 2.0 mg / dl . peripheral blood smear revealed increased number of schistocytes ( 5 per high power field ; fig . ttp was diagnosed and adamts13 level was requested . a oncedaily plasma exchange was initiated . after 6 days of daily plasma exchange , platelet count increased to 195,000/l , hemoglobin level stabilized at 10.1 gm / dl , creatinine level decreased to 1.45 mg / dl , troponin t level decreased to 0.09 ng / ml , lactate dehydrogenase level decreased to 246 units / l , and his chest pain improved with complete resolution of hemolysis and marked improvement of energy level . 3 ) . peripheral blood smear showing increased number of schistocytes ( arrows ) . this is the second ekg which demonstrates improvement of the st segment depression as compared to fig . prior to discharge , the patient underwent cardiac catheterization and revealed no evidence of occlusive cad . three weeks later , a repeat echocardiogram showed an improved left ventricular ejection fraction to 6570% . in this report , we present a case with typical acute coronary syndrome ( acs ) features including leftsided chest pain , elevated troponin t level , and risk factors for atherosclerosis such as hypertension and prior history of cad . ttp can rarely present with acs due to development of microthrombi in the small vessels supplying the myocardium , which makes it difficult to distinguish from atherosclerotic acs , particularly in patients with multiple risk factors , as in our case . the presence of anemia , thrombocytopenia , and evidence of hemolysis should raise the suspicion for ttpinduced acs . in addition , cardiac involvement in ttp patients can manifest as mi , tachyarrhythmias , conduction disturbances , congestive heart failure ( chf ) , cardiogenic shock , myocarditis , myocardial necrosis , or sudden cardiac death 4 , 5 , 6 , 7 , 8 . in fact , most ttp patients with cardiac involvement are diagnosed postmortem at the time of autopsy , where thrombosis of the small vessels supplying the myocardium is seen in as high as in 77% of patients 9 , 10 , 11 , 12 . the presence of microthrombi in the microcirculation of the myocardium and the presence of anemiarelated high cardiac output may contribute to cardiac dysfunction , leading to symptomatic chf 11 . in a study conducted by balasubramaniyam et al . , the 20012010 nationwide inpatient sample database was used to identify patients aged 18 years with the diagnosis of ttp and acute mi . in this study , multiple variables were examined including age , gender , tobacco use , hypertension , diabetes mellitus , dysplipidemia , and prior history of cad or stroke . conducted a study on 14 patients with ttp who underwent coronary angiography due to mi . our patient had angiography after six sessions of plasma exchange , and did not demonstrate significant cad . unlike our patient since cardiac involvement in ttp carries a higher mortality rate , prompt recognition of cardiac involvement is vital 5 , 15 . a study conducted by benhamou et al . showed that a troponin i level higher than 0.25 g / l in patients with ttp predicts more refractory disease and is associated with a threefold increase in the risk of death , regardless of cardiac symptoms . the authors of this study concluded that troponin level should be used as a prognostic indicator in patients with ttp 16 . due to its rarity however , plasma exchange improves symptoms in the majority of these patients and should be initiated as soon as possible . in our patient , cardiac , neurologic , hematologic , and renal manifestations of ttp improved with plasma exchange . invasive procedure such as coronary angiography have low yield in these patients and may even exacerbate ttp . as a result , it should be deferred until organ functions improve and hemolytic markers normalize . 17 , 18 , 19 , 20 . based on our case , it is reasonable to initiate plasma exchange transfusion for such patients without delay in addition to any supportive care measures . imaging of the coronary arteries and other invasive procedures can be performed following clinical stabilization of the patient and improvement in laboratory parameters which will make such procedures safer .

key clinical messagein patients presenting with thrombotic thrombocytopenia purpura and nonst elevation myocardial infarction , prompt initiation of plasma exchange takes precedence over other invasive diagnostic procedures for coronary artery disease . such procedures should be delayed until clinical condition and laboratory parameters have been stabilized .

thelazia callipaeda ( tc ) and thelazia californiensis are the two common nematode parasites known to cause human thelaziasis . table 1 shows probable global data of ocular thelaziasis . an infected person or carnivorous / bovine animal harbors the first stage larvae in the lacrimal secretions . arthropod vectors feeding on such infected lacrimal secretions ingest these larvae , which undergo three molts inside the midgut of the vector in 2 - 3 weeks time and develop into infective third stage larvae . third stage larvae are transmitted accidentally to another susceptible host by the vector when it feeds on such lacrimal secretions . the third stage larvae develops into adult form within 35 days in the eye of the infected person . thelazia callipaeda ( tc ) and thelazia californiensis are the two common nematode parasites known to cause human thelaziasis . table 1 shows probable global data of ocular thelaziasis . an infected person or carnivorous / bovine animal harbors the first stage larvae in the lacrimal secretions . arthropod vectors feeding on such infected lacrimal secretions ingest these larvae , which undergo three molts inside the midgut of the vector in 2 - 3 weeks time and develop into infective third stage larvae . third stage larvae are transmitted accidentally to another susceptible host by the vector when it feeds on such lacrimal secretions . the third stage larvae develops into adult form within 35 days in the eye of the infected person . a 74-year - old hindu farmer from sakaleshpura taluk reported to department of ophthalmology with symptoms of foreign body sensation in his right eye for the last two days following a hit with the tail of cattle . the patient reportedly hails from a hilly region and rears the cattle in his farm house . on examination , eye was quiet , no conjunctival congestion , cornea and pupils were normal with visual acuity 20/20 . he was prescribed antibiotic eye drops and advised to come after a week . on 28 july 2008 , he returned with the same complaint . on examination , during the upper eyelid eversion , a white thread - like worm was seen near the lateral canthus . slit lamp bio microscopic examination revealed four to five white , motile , translucent worms creeping in the superior fornix coming through a tunnel in the conjunctival sac . it was observed that two worms were already crawling on the corneal surface ; five worms were removed with a plane forceps after instilling 4% lignocain hydrochloride eye drops . the patient was observed for next three days for more worms , but no worms were found . after two years of follow up , there was no recurrence and the patient was symptomatically free . the worms were transferred to a sterile normal saline container and were sent to microbiology department for further evaluation . the worms were identified as nematode parasites using inverted microscope , which showed sluggish motility , internal systems were clearly visible , and both the ends were straight , hook - like projections were appreciated figs . 1 - 3 . for further confirmation of the species , the worms and the microphotographs were sent to cdc atlanta and confirmed as male worms of tc . anterior end demonstrating the mouth and esophagus the body with transverse cuticular striations posterior curved end of the worm two species of the genus thelazia , tc and thelazia californiensis have been found in the human eye . tc is responsible for most of the cases of human ocular thelaziasis ( hot ) in asia . although these parasites commonly affect the anterior segment of the eye , it can cause severe damage to the posterior segment . a case of intraocular thelaziasis with rhegmatogenous retinal detachment and intraocular inflammation involving vitreous causing visual disturbances and recovering the worm from human vitreous through pars cattle rearing , contact with stray dogs , mountainous terrain , and rainy season make the humans vulnerable to ocular thelaziasis . in the present case , cattle rearing was the predisposing factor , which correlates with the second case report of ocular thelaziasis in 2008 . conjunctival and cornel injuries , traumatic conjunctivitis facilitate the introduction of the larvae into the sub - conjunctival space and vitreous cavity . the natural cycle of vector activity of ocular thelaziasis has seasonal distribution , during ( july - august ) which period our patient had been reported . the main mode of transmission in this case was probably injury with the cattle tail and might be through contaminated towels . the towels being contaminated with cow dung containing deposited eggs / larvae of the worm and the same is used for wiping or rubbing the eyes after the injury is the possible mode of entry of eggs / larvae into the eyes . although the treatment of canine infection by tc is with topical organophosphates , 1% moxidectin , 10% imidacloprid is effective in mammals , mechanical removal of the parasite is the only curative treatment for hot . in conclusion , hot is a disease associated with very poor living conditions , low socio - economic status , and improper personal hygiene commonly seen among rural population . prevention of thelaziasis is by protective measures like using bed nets at night , keeping the eyes , face and nose clean while sleeping to prevent the vectors , maintaining personal hygiene , keeping the surroundings clean , and creating public awareness about the disease .

thelaziasis is an arthropod - born disease of the eye and adnexa caused by thelazia callipaeda , a nematode parasite transmitted by drosophilid flies to carnivores and humans . because of its distribution mainly confined to south asian countries and russia , it is commonly known as oriental eye worm . it is often under - reported and not been given its due clinical importance . we report first case of human thelaziasis from hassan district , karnataka . five creamy - white , translucent worms were removed from the conjunctival sac of a 74-year - old male patient . based on morphological characters , the worms were identified as nematodes belonging to the genus thelazia and speciation was confirmed by cdc , atlanta as callipaeda . rarity of the disease and its ability to cause both extra and intraocular manifestations leading to ocular morbidity is the reason for presenting this case . from the available data , this is the first case report from karnataka , india .

pulmonary lipomas originate most commonly in the trachea or bronchi and they have been estimated to compose about 0.1% of all pulmonary tumors.1 endobronchial lipomas may cause clinically significant problems including recurrent pneumonia and irreversible pulmonary damage.24 bronchial asthma - like symptoms due to endobronchial lipoma is extremely rare and only a few cases have been reported.2,5 we present a case of endobronchial lipoma , which had been treated as bronchial asthma for four years . a 53-year - old woman was admitted to our hospital with the complaints of cough , sputum , wheezing , and dyspnea for four years and has been treated as bronchial asthma . spirometric pulmonary function tests resulted as follows : forced expiratory volume in one second ( fev1 ) , 64% ; forced vital capacity ( fvc ) , 57% ; fev1/fvc , 92.3% ; not reversible with bronchodilators ( figure 1 ) . a chest x - ray on admission demonstrated a linear atelectasis on the right lower zone with elevation of the right hemidiaphragm . since neither the patients historical features with physical examination findings nor spirometric results with radiologic features were consistent with bronchial asthma we thought that bronchial asthma diagnosis was not true . we performed computed tomographic ( ct ) scans of thorax which showed an endobronchial lesion within intermediate bronchus ( figure 2 ) and fiberoptic bronchoscopy that revealed a well - circumscribed polypoid endobronchial lesion that almost completely obstructed the lumen of the intermediate bronchus ( figure 3 ) . since histopathologic examinations of the biopsy specimens were not sufficient for final diagnosis , the patient underwent a right thoracotomy and bronchiotomy . the postoperative course was uneventful and the patient was discharged on the 5th hospital day . benign tumors of tracheobronchial tree are rare entities and lipomas are among the rarest benign tumors . endobronchial lipomas consist of mature adipose tissue and some fibrous components lined with normal bronchial epithelium . therefore , small biopsies taken by fiberoptic bronchoscopy may not be diagnostic,3,6 as in our case . the clinical problems of endobronchial lipomas are fundamentally caused by obstruction of airflow including recurrent pneumonia , bronchiectasis , and irreversible pulmonary damage . bronchial asthma - like symptoms are extremely rare and only a few cases have been reported.2,5 in the literature , muroaka and colleagues reviewed 64 patients with endobronchial lipomas . forty - eight patients ( 75% ) were symptomatic , including cough , sputum , hemoptysis , fever , and dyspnea . about 80% of the patients had abnormal radiographic findings , which were atelectasis , consolidation , mass shadows , and pleural effusion.7 our patient was female and she suffered from cough , sputum , wheezing , and dyspnea . she had been treated for bronchial asthma and had developed recurrent pneumonia three times . computed tomography and magnetic resonance imaging ( mri ) are reported to be helpful in establishing the diagnosis by demonstrating a fatty tumor within the bronchial lumen . ct is highly specific and sensitive for the detection of fat , and the presence of fat within a lesion suggests a benign etiology , such as lipoma or hamartoma.8,9 several reports of mri appearance of lipoma have shown high signal intensity on t1-weighted images and intermediate signal density on proton density and t2-weighted images , compatible with normal fat.10 although fiberoptic bronchoscopy is the most valuable method of diagnosis of endobronchial lesions , it is not usually sufficient to make diagnosis of endobronchial lipomas , as in our case . thus , surgical or bronchoscopic resection must be performed in order to reach final diagnosis . although endobronchial removal with laser therapy is the preferred treatment option,9 the patient underwent right thoracotomy and bronchiotomy since we had technical dificulties related to interventional bronchoscopy . bronchial asthma - like picture may also be seen in ( 1 ) upper airway obstructions including both extrathoracic ( eg , vocal cord dysfunction syndrome , epiglottidis ) and intrathoracic causes ( eg , tracheal stenosis , endobronchial tumors , right - sided aortic arch ) , and ( 2 ) lower airway obstructions such as aspiration , chronic obstructive pulmonary disease , or bronchiectasis . thus , the diagnosis of conditions other than bronchial asthma should be considered when the initial evaluation suggests their presence or when the patients do not respond to conventional asthma medications,11 as in our case . physical examinatin findings , spirometric results , and radiologic features are helpful for the exact diagnosis . in conclusion , although endobronchial lipomas occur rarely , it must be kept in mind in differential diagnosis of bronchial asthma and also in patients with a history of recurrent pneumonia .

endobronchial lipoma is a rare neoplasm of the tracheobronchial tree and it may cause irreversible pulmonary damage due to recurrent pneumonia . rarely , it may mimic bronchial asthma . we present a 53-year - old woman with an endobronchial lipoma , which had been treated as a bronchial asthma for four years . she also had developed recurrent pneumonia three times .

cutaneous pseudolymphoma refers to a heterogeneous group of benign , reactive t- or b - cell lymphoproliferative processes of multifactoral origin that clinically and/or histologically simulate lymphomas . the inflammatory infiltrate is band like , nodular , or diffuse and is composed predominantly of lymphocytes with or without other inflammatory cells . depending on the predominant cell type in the infiltrate , cutaneous pseudolymphomas are divided into t- and b - cell pseudolymphomas . a 63-year - old woman living in the countryside referred to our department with a 2-month history of a red nodule localized on the right breast ( nipple and areolar region ) . the patient did not report either a tick bite or the presence of erythema migrans in previous months . the lesion was a smooth , raised , nonscaling , erythematous nodule , with a diameter of 2 cm ; this nodule was movable on the plane below , had a tough - elastic consistence upon palpation , and was asymptomatic [ figure 1 ] . the fact that the patient lived in the countryside and the appearance of the breast nodule in september led us to the suspicion of a tick - borne bacterial disease . a smooth , raised , nonscaling , erythematous nodule on the right nipple and areola punch biopsy , histological examination , and immunohistochemical analyses were performed . immunohistochemistry was performed with monoclonal antibodies directed toward the markers cd3 , cd5 , cd10 , cd20 , cd21 , cd79a , bcl-2 , bcl-6 , mib-1 , kappa , and lambda chain , as previously described . serological tests to evaluate the igg and igm antibody response to borrelia were performed using elisa and western blot . histological examination revealed a normal appearance of the epidermis and an upper location of the dense dermal infiltrate of lymphocytes ( top - heavy pattern ) , characterized by the formation of follicles with germinal centers . in the latter localization , centrocytes , centroblasts , and the prominent presence of tingible - body macrophages ( starry sky pattern ) were evident . moreover , a mixed reactive infiltrate of histiocytes , eosinophils and numerous polyclonal plasma cells was present in the interfollicular areas [ figure 2 ] . histological and immunohistochemical analyses revealed a normal appearance of the epidermis and an upper location of the dense dermal infiltrate immunohistochemical analyses documented the predominance of cd20+/cd79a+ b - lymphocytes , but also some cd3 + , cd5 + t - lymphocytes were found around b - cell areas and cd21 + follicular dendritic cells . b cells appeared cd10 , bcl-2 , bcl-6 negative with a normal degree of proliferation as detected by mib-1 marker . cd79a+/cd20- plasma cell expressed both kappa and lambda immunoglobulin light chains , revealing a polyclonal pattern . on the basis of clinical , histological , immunohistochemical , and serological findings we diagnosed borrelial lymphocytoma , which was successfully treated with doxycycline 100 mg 2/daily for 20 days and ceftriaxone 1 g / daily 20 days thereafter . borrelial lymphocytoma cutis is a late cutaneous manifestation of lyme borreliosis that in general arises some months after the tick bite . the most frequent onset is reported in august and september . in endemic regions , it is the stereotypical example of cutaneous b - cell pseudolymphomas . the lesions are most frequently located on the earlobe in children and in the areolar region or on the nipple in adults . other rare locations are the nose , scrotum , upper arm , shoulders , nape of the neck , axilla , and back of the foot . lyme borreliosis is endemic to various areas of italy and the main vector is the tick ixodes ricinus , a species widespread in countryside and mountain regions . several cases of lyme borreliosis have been reported to the health authorities in italy since 1990 , when systematic national surveillance started . in our area , borrelial lymphocytoma is uncommon , but not rare and it represents the least common manifestation within the spectrum of borrelia burgdorferi - associated skin diseases . nevertheless , information on the epidemiology of these diseases is still scanty , owing to inadequate notification . in the present case , clinical diagnosis was not possible solely on the presence of a classical nodular form without lymphoadenopathy . an absence of a prior tick bite and a previous or concomitant erythema migrans at clinical presentation rendered a more challenging diagnosis . other pseudolymphomas can clinically simulate borrelial lymphocytoma [ table 1 ] , but the most important differential diagnosis is with malignant large b - cell lymphoma . the key histologic features that suggest lymphocytoma cutis include the presence of a mixed infiltrate that includes histiocytes , eosinophils and plasma cells , in addition to lymphocytes . in lymphocytoma cutis , the infiltrate tends to be top - heavy , whereas most lymphomas are centered in the deep dermis . moreover , unlike the follicles in follicular lymphomas , all of the follicles in lymphocytoma cutis contain numerous tingible - body macrophages . malignant b - lymphocytes usually show positivity for cd10 , bcl-6 ( outside follicles ) , bcl-2 ( within follicles ) and a monoclonal restriction to either kappa or lambda immunoglobulin light chains , whereas benign infiltrates are often cd10 , bcl-6 , bcl-2 negative and exhibit a polyclonal pattern with expression of both light chains . finally , immunostaining for proliferating cells ( detected by mib-1 marker ) is a helpful clue for the diagnosis , revealing a high or normal proliferation rate of the germinal centers cells in most cases , in contrast to the decreased proliferation observed in follicle center cell lymphoma . depending on the predominant cell type in the infiltrate , cutaneous pseudolymphomas are divided into t- and b - cell pseudolymphomas we reported this case due to a typical clinical presentation of borrelial lymphocytoma , but without history of prior tick bite , to highlight the importance of lyme borreliosis in the differential diagnosis of a patient living in the countryside who presents a red nodule on the nipple and areola .

a 63-year - old woman living in the countryside referred to our department with a 2-month history of a red nodule localized on the right breast . histological examination , immunohistochemical analyses and serologic evaluation conducted with elisa and western blot were performed . clinical diagnosis of borrelial lymphocytoma was not possible solely on the clinical presentation of a classical nodular form without lymphoadenopathy . an absence of a referred prior tick bite and a previous or concomitant erythema migrans at clinical presentation rendered a more challenging diagnosis . the fact that the patient lived in the countryside , the appearance of the breast nodule in september , and serologic , histologic , and immunohistochemical analysis facilitated the diagnosis of borrelial lymphocytoma . we report this case to highlight the importance of an investigation of lyme borreliosis when a patient living in the countryside presents with a red nodule of the nipple and areola .

a 52-year - old woman presented at the clinic with pelvic pain and floating of three months duration . there was no history of comorbidities or regular medication use ( gravidity : 1 , parity : 0 , abortion : 1 ) . serum tumor markers were normal ( human chorionic gonadotropin [ hcg ] : < 1 miu / ml , cancer antigen [ ca]-125 : 12 u / ml , ca-15 - 3 : 19 u / ml , ca-19 - 9 : 5 u / ml , alpha fetoprotein [ afp ] : 1.8 ug / l , carcinoembryonic antigen [ cea ] : 1.2 ng / ml , lactic dehydrogenase [ ldh ] : 193 iu / l ) . complete blood count ( cbc ) results were : hemoglobin : 12.2 gr / dl , hematocrit : 36% , thrombocyte : 178,000 mm , white blood count ( wbc ) : 10700 mm . pelvic computed tomography ( ct ) showed solid , smooth contoured mass ( 60x50x60 mm ) located in the left adnexal area . pathological evaluation : the tumor was a whitish - gray , solid mass ( 55x55 mm ) with a smooth surface . smooth muscle cells with bundles of elongated cells and spindled nuclei ( he200 ) can be seen in figure 2 . since the first report of primary ovarian leiomyomas in 1862 , few additional cases have been reported [ 2 , 3 ] . data from prior reports indicate that primary ovarian tumors are benign , unilateral , asymptomatic , less than 3 cm in size , and occur in premenopausal women [ 1 , 2 ] . in this report , a case of a postmenopausal woman with ovarian leiomyoma is presented . most accepted theory for the histogenesis of leiomyoma in the ovary is growth of the tumor in smooth muscle cells of the ovarian ligament or smooth muscle fibers of the vascular wall or stroma [ 3 , 4 ] . ovarian leiomyomas are usually asymptomatic and are found either during routine physical examination or incidentally at surgery . abdominal pain is a commonly encountered symptom , and rarely , some women present with elevated cancer antigen ( ca)-125 , hydrothorax , and ascites [ 1 , 57 ] . the present patient presented at the clinic with pelvic pain . preoperative evaluation of ovarian solid tumors requires ultrasonography , tumor markers and mri ( magnetic resonance imaging ) . ultrasonography shows solid mass isoechoic with myometrium , so it is difficult to distinguish from other solitary tumors or pedunculated myomas . preoperative evaluation of this case included ultrasonography ( isoechoic mass in left ovary ) and normal serum tumor marker levels . differential diagnosis includes other solitary tumors of the ovary ( fibroma , thecoma , fibrothecoma , cellular fibroma , sclerosing stromal tumors , sex cord - stromal tumors ) , leiomyomas arising from broad ligament or uterus later becoming parasitic . although only some 70 cases have been reported , as a result of incidental findings , the authors believe some cases have gone unreported . literature indicates that primary ovarian leiomyomas are a benign condition without recurrence , and oophorectomy is the preferred treatment , but in young women , fertility sparing , minimally invasive procedures should be considered . in conclusion ,

leiomyomas are benign neoplasms that can develop wherever smooth muscle is present . primary leiomyomas of the ovary originate from smooth muscle cells of ovarian tissue and are rare , solitary tumors . approximately 70 cases have been reported . they usually present in premenopausal women . the present case is a report of left ovarian leiomyoma in a postmenopausal woman .

it causes ill - health among millions of people each year and ranks as the second leading cause of death from an infectious disease worldwide . in 2011 , there were an estimated 8.7 million new cases of tb and 1.4 million deaths from tb ( 1 ) . urinary tract as initial infection is infrequent and associated extensive calcification of the whole urinary system is rare . we report a 66-year - old man who presented with diffuse calcification of the urinary system and disseminated miliary tb due to gutb . a 66-year - old male , without notable medical history , was admitted to a local hospital with complains of urinary frequency , urgency and urine pain for half a month in january 2010 . he was diagnosed with urinary tract infection and a course of treatment with levofloxacin was recommended . but over the next 1 year he had replacing urinary irritation symptoms and gross hematuria . plain x - ray abdomen and the ultrasound showed urinary lithiasis on the left side of the ureter . the symptoms were abated , but still break out repeatedly . until march 2012 , he was admitted to our hospital . abdominal ct images showed extensive calcifications within bilateral kidneys , ureters and bladder with walls thickening and contracture malformation ( fig . 1a , b , d , e ) , and thoracic ct images showed diffuse inhomogeneous nodules insufficiently integrated in both lungs ( fig . laboratory test showed serum urea=49.98mmol / l ( normally 3.2~8.2mmol / l ) , creatinine=956-umol / l ( normally 53~97umol / l ) . m. tuberculosis had been found through acid - fast bacilli staining , ( 3+/4 + ) . tb infected t cells detection esat-6 and cfp10 was 10 and > 50 sfcs ( normal level < 6 sfcs ) respectively . anti - hiv , hbsag , anti - hcv were all negative . the patient was diagnosed with gutb and hematogenous disseminated pulmonary tb , and a decision was made to start antitubercular treatment . bedside radiography of abdomen demonstrated the shaping calcifications of the kindneys expending along the ureters ( fig . unfortunately , the patient 's condition deteriorated rapidly , and he died 7 days later . after 2 weeks , cultures of urine and sputum samples yielded a strain of m. tuberculosis that was susceptible to all antituberculous agents respectively . most of patients who present with tuberculous genitourinary disease have a known history of prior pulmonary tb or have radiographic evidence of prior subclinical pulmonary infection . our patient with urinary tract as initial infection site and secondary spread to the lungs is relatively rare . some patients are asymptomatic and only found sterile pyuria in routine urine examination , so they are easy to be misdiagnosed as urinary tract infection ( 3 ) . gross hematuria is seen in only 10% , but microscopic hematuria is present in up to 50% of the cases ( 4 ) . renal calcifications are common manifestation of tb . however , calcification of the ureter is rare ( 5 ) . our patient was characterized by multiple calcifications of the bilateral pelvicalyceal system and associated bladder and bilateral ureteral calcification . the extensive calcification of the whole urinary system just like this patient is very rare . the definitive diagnosis of tb involves demonstration of m. tuberculosis by microbiological or histopathological methods . presented here , m. tuberculosis has been found through acid - fast bacilli staining and the urine culture was positive for m. tuberculosis . the onset of gutb is often insidious , and there are many limitations of noninvasive testing in establishing the diagnosis ( 6 ) . we should be alert to the possibility of gutb when the urinary irritation symptoms occur repeatedly even without a history of tb . tb must be kept on the differential diagnosis for many lesions and pathologies of the genitourinary system . through this case

genitourinary tuberculosis ( gutb ) is difficult to diagnose in the earlier stage owing to the non - specific symptoms . usually , renal tuberculous involvement is unilateral and the imaging finding is renal calcification , but associated calcifications of bilateral ureter and bladder are rare . we report a 66-year - old man who presented with diffuse calcification of the urinary system ( including bilateral pelvicalyceal system , both ureters and bladder ) and disseminated miliary tuberculosis due to gutb . he had been misdiagnosed with urinary tract infection and urinary lithiasis for two years before the diagnosis of gutb was confirmed by microbiological examination of the urine . this case highlights the importance of maintaining a high index of clinical suspicion for gutb .

the incidence of non - tuberculosis mycobacterial ( ntm ) infection has increased recently , and this condition currently accounts for 10%15% of all mycobacterial infection cases.1,2 while the mycobacterium avium complex ( mac ) and m. kansasii are responsible for more than 90% of cases of mycobacterial infection , mycobacterium species have diversified and now include species such as m. szulgai.3 m. szulgai is an uncommon mycobacterial pathogen in humans.4 it causes pulmonary disease resembling the common type of m. tuberculosis infection , as well as extrapulmonary infection . disseminated infection of m. szulgai is rare and usually occurs in immunocompromised patients.5 here , we report an unusual case of disseminated mycobacterial infection progressing to acute respiratory distress syndrome ( ards ) in a non - immunocompromised hepatitis b virus ( hbv ) carrier . a 59-year - old man was admitted to our hospital because he was experiencing severe respiratory distress . he had been diagnosed with intestinal catarrh when he was 1 year old , nephritic syndrome when he was 13 years old , and pleuritis when he was 57 years old . he lived in a rural district composed mainly of paddy fields and worked as an engineer in an office . he was an ex - smoker , having quit smoking at the age of 30 years . six months before hospital admission , he presented with edema of the left leg and low - grade fever . at the time of presentation , he tested positive for anti - chlamydia pneumoniae immunoglobulin antibodies and was treated with clarithromycin . however , 4 months before hospital admission , soon after the treatment was discontinued , he developed low - grade fever , a skin eruption resembling folliculitis in the left upper arm , and adenopathy of multiple lymph nodes . two weeks before hospital admission , he presented with bilateral swelling of the fingers and feet ( fig . chest x - ray ( cxr ) findings were normal , but computed tomography ( ct ) revealed the presence of tiny miliary nodules in both lung fields ( fig . five days before admission , the patient developed a fever ( temperature > 38 c ) . 3 ) , and ct revealed the presence of multiple nodules and infiltrates in both lung fields ( fig . blood gas analysis in room air revealed a partial pressure of oxygen in arterial blood ( pao2 ) of 46 mmhg and a ph of 7.46 . the other blood parameters were as follows : hemoglobin level , 11.3 g / dl , was within normal limits ; leukocyte count , 14.4 10 cells / l , was high ; and c - reactive protein level , 20.8 mg / l , was high . the brain natriuretic peptide level was 30.0 pg / ml , which was within the normal limits . we initiated treatment with a broad - spectrum panel of antimicrobial agents , namely , meropenem and ciproxacin , along with methylprednisolone pulse therapy . however , over the next 24 hours , the patient developed severe respiratory failure ( pao2/fraction of inspired oxygen [ pf ratio ] < 200 ) and required intubation and mechanical ventilation with positive end - expiratory pressure . positive results were obtained in ziehl - neelsen staining of a sputum smear and bal fluid , but negative results were obtained for gram staining . we therefore strongly suspected mycobacterial infection . although m. tuberculosis and the mac were not detected in polymerase chain reaction analysis of the patient s samples , we continued to suspect a miliary mycobacterial infection . therefore , we initiated treatment with a 4-drug regimen comprising rifampicin 450 mg / day , isoniazid 300 mg / day , pyrazinamide 1500 mg / day , and streptomycin 1 g / day . meropenem and ciproxacin were discontinued 17 days after hospital admission because the patient s condition improved . skin biopsy which was done at 1 weak after admission revealed diffuse infiltration of mixed inflammatory cells in the dermis , which was consistent with the diagnosis of cutaneous ntm infection ( fig . 4).6 cxr and ct revealed a gradual improvement in the patient s condition , and the patient was weaned off mechanical ventilation 23 days after admission . about 1 month after admission , we identified m. szulgai in cultures of the sputum , skin ulcer exudate , and bal fluid using the colorimetric microdilution plate hybridization method.7 about 2 months after admission , the bilateral infiltrates had almost completely disappeared from the lungs ( fig . m. szulgai infection is very rare , and this bacterium accounts for < 0.5% of all isolates obtained from human patients with ntm infection.8,9 in japan , only 27 cases of m. szulgai infection have been reported between 1989 and 2008 , most of which involved pre - existing disease.10 in a study in turkey , mycobacterial growth was positive in 10041 of 45981 samples from 19553 patients with a prediagnosis of pulmonary tuberculosis between november 2004 and january 2009 ; m. szulgai was isolated from only 3 patients among this study population.11 cultures yielding m. szulgai usually have a pathologic significance , because this bacterium is rarely recovered from the environment.8,9 consequently , in appropriate clinical circumstances , 1 positive culture may suffice for diagnosing m. szulgai infection . in the present case , cultures of the skin ulcer exudate , sputum , and bal fluid all revealed m. szulgai infection only , implicating this bacterium as the sole cause of the miliary infection . the clinical features of this case , including the acute onset , bilateral shadows , low pf ratio ( < 200 ) , and no clinical evidence of elevated left atrial pressure , were compatible with the diagnostic criteria for ards.12 ntm infection - induced ards is very rare.13 in contrast , miliary tuberculosis is a well - known cause of ards.14,15 this case is the first reported case of m. szulgai infection progressing to ards . miliary tuberculosis accounts for 2.3% of all cases of tuberculosis infection in japan.14 additionally , in a series study in south korea , only about 20% of miliary tuberculosis patients developed ards.16 thus , miliary infection - induced ards probably accounts for less than 1% of all tuberculosis cases . on the other hand , the incidence of miliary infection among all cases of m. szulgai infection can not be easily estimated , because infection with m. szulgai is very rare . in conclusion , m. szulgai can cause severe disease by hematogenous spread . m. szulgai infection - induced ards is extremely rare , but can be successfully treated with antituberculosis drugs .

a 59-year - old man presented with a skin eruption and bilateral swelling of the legs . soon after the initial presentation , he developed acute respiratory distress syndrome ( ards ) with miliary lung nodules . culture of samples from the skin ulcers , sputum , and bronchoalveolar lavage fluid all revealed mycobacterium szulgai infection . the patient was successfully treated with antituberculosis drugs . m. szulgai infection is very rarely reported worldwide , and disseminated infection usually occurs in immunocompromised patients . however , the present patient was a non - immunocompromised case , although he was a hepatitis b virus carrier . while the progression to ards from m. tuberculosis infection is well known , this is the first case of m. szulgai infection progressing to ards .

darier disease is a rare autosomal dominant inherited genodermatosis with onset around puberty , characterized by yellow to brown , oily keratotic papules and plaques primarily affecting the seborrheic areas of the face and chest . the disorder may show different clinical manifestations , such as palmoplantar pits and nail abnormalities . in this report we describe a flare - up of darier disease during treatment with interferon--2a ( ifn-2a ) . after excision and sentinel node biopsy of an amelanotic malignant melanoma located on the left lower leg , with a breslow tumor thickness of 3.5 mm , our 48-year - old female patient was treated with subcutaneous injections of 3 million international units of ifn-2a thrice weekly . the therapy was initially well tolerated . the patient had been diagnosed in puberty with darier disease , affecting her face and sternum . since then she had had almost yearly disease flare - ups , especially at the beginning of the summer period . one month after the initiation of ifn-2a treatment the patient presented with an exacerbation of darier disease , with multiple , disseminated , erythematous and brownish , partly follicular , hyperkeratotic papules on the trunk and extremities accompanied by severe itching ( fig . 1 ) . some of the skin lesions , especially on the lower extremities , showed a glossy and lichenoid form ( fig . we discontinued ifn-2a treatment and the patient was admitted to our inpatient department for further diagnostic investigation and therapy . the epidermis showed irregular hyperplasia and partly significant suprabasal acantholysis with dyskeratotic keratinocytes ( fig . close to these areas a dense subepidermal , band - like , lymphocytic infiltration under a narrow epidermis was observed ( fig . a biopsy from another skin lesion demonstrated compact orthohyperkeratosis , acanthotic epidermis with scattered suprabasal areas with acantholysis and dyskeratosis . oral antihistaminics and external application of steroids , urea and polidocanol improved both the skin lesions and itching . as we had ruled out drug eruption , we continued the treatment with ifn-2a at the same dose , and the patient was further monitored in the context of postsurgical tumor follow - up . despite the initial improvement of the lesions , they did not heal and persisted during the therapy with ifn-2a . darier disease , also known as dyskeratosis follicularis or darier - white disease , is a rare autosomal dominant inherited skin disorder which usually becomes apparent in the second decade of life . mutations in the atp2a2 gene , encoding serca2 , a calcium pump of the sarco-/endoplasmic reticulum , are responsible for the disorder . . however , the mechanism by which decreased activity of the serca2 calcium pump leads to these changes is still under investigation . mechanical trauma , heat , humidity , ultraviolet b radiation and oral corticosteroids are known trigger factors of the disease . moreover , lithium is a recognized exacerbating factor of darier disease . suggested that lithium exacerbates the symptoms of darier disease through reducing the epidermal levels of serca2 to a critical point , promoting the acantholytic and other histologic disturbances of the disorder . diltiazem has also been reported to worsen the disease , acting possibly on the same axis with the gene defect of the disorder . our patient had flare - ups of the disease in the face and chest almost every year at the beginning of the summer period , but this flare - up came extremely early , with a remarkable , unexpected manifestation on the extremities , partly with lesions that resembled lichen nitidus . lichen nitidus is a rare skin eruption characterized by multiple , small , discrete , glistening , flesh - colored papules that may occur anywhere in the skin . due to this fulminant course of the eruption , we suspected ifn as a possible trigger . to our knowledge dry skin , itching , mild hair loss and induction or exacerbation of psoriasis vulgaris are known cutaneous side effects of ifn. there are reports of flare - up of psoriasis vulgaris after starting ifn therapy for several diseases . the pathogenetic interpretation of these findings is difficult due to the lack of a convincing etiologic concept . in order to understand the mechanism of such an association it induces immune activation through enhancement of interleukin-22 ( il-22)-producing t cells and provides the interface between immune activation and epidermal remodeling by increasing keratinocyte responsiveness to il-1 . . demonstrated that ifn strengthens the responsiveness of epidermal keratinocytes to il-22 , an important mediator in the pathogenesis of psoriasis , via increased il-22 receptor expression . it is also known that ifn activates the jak - stat pathway , whose cytokine components play a crucial role in common skin disorders , including psoriasis vulgaris and atopic dermatitis [ 9 , 10 ] . however , whether ifn really worsens the course of darier disease remains unclear .

darier disease / dyskeratosis follicularis is a genodermatosis characterized by brown , oily keratotic papules and plaques in the seborrheic areas of the face and chest . responsible for the disease are mutations in the atp2a2 gene , encoding serca2 , a calcium pump of the sarco-/endoplasmic reticulum . mechanical trauma , heat , humidity , ultraviolet b radiation , oral corticosteroids and lithium are known trigger factors of the disorder . we report on a 48-year - old german woman with a flare - up of darier disease under interferon--2a ( ifn-2a ) therapy with clinical signs of lichen nitidus . due to the fulminant course of the eruption , we suspected ifn as a possible trigger . to our knowledge there are no reports regarding exacerbation of darier disease during ifn therapy . possible pathogenetic mechanisms are being discussed .

tb cases from all 50 states and the district of columbia were recorded in the us national tb surveillance system ( ntbss ) . report forms collected standard information at the time of diagnosis , including country of origin , age at diagnosis , and years since arrival in the united states ( centers for disease control and prevention , unpub . us - born persons were defined as those either born in the united states ( or its jurisdictions ) or those born in a foreign country who had at least 1 us parent . our analyses included all cases reported from 1999 to 2001 among persons who had emigrated from china , haiti , india , south korea , philippines , or vietnam within 10 years before their diagnosis in the united states . case reports that were missing information on country of origin were not included in this analysis . while immigrants from mexico are also at high risk for infection and disease , we excluded cases among this group because the large proportion of unauthorized immigrants precluded a reasonable estimation of population denominators for this subgroup . we used us immigration statistics to document the number and ages of persons arriving from each of these countries in the years 19892001 ( available from http://uscis.gov/graphics/shared/aboutus/statistics/immigs.htm ) . to estimate the appropriate population denominators from which these tb cases arose , we applied age - specific mortality rates ( categorized as < 14 years , 514 years , 1524 years , 2544 years , 4564 years , and 65 years ) for us immigrants and assumed that these populations were depleted only by death ( no emigration from the united states ) . confidence intervals for the country- and age - specific incidence rates were calculated by assuming that tb case counts were poisson distributed . from 1999 to 2001 , a total of 5,198 cases of tb were recorded in ntbss among persons who had emigrated from the study countries within the preceding 10 years . figure 1 depicts the country - specific incidence rate of tb by time since arrival in the united states . incidence rates among immigrants who have been in the country for < 1 year at the time of their diagnosis are similar in relative rank and magnitude to the estimated incidence rates in the country of origin ( table 1 ) ( 7 ) . tb incidence rates appear to decrease sharply over the first 2 years in the united states . figure 2 depicts the age - specific incidence rates by time since arrival for us immigrants from each of 6 countries . when incidence rates during the first 2 years were compared with those after 510 years in the united states , significantly higher rates were found to occur immediately after arrival in all age groups examined ( table 2 ) . because incidence rates immediately after arrival will overestimate the true incidence if prevalent cases are misclassified as incident cases , we tested the sensitivity of these findings to the incidence rates calculated for the first 2 years after arrival . we found that this significant elevation in early ( < l year after immigration ) versus late ( years 510 ) incidence persists in almost every age group for all countries even if we assume that we overestimated incidence by a factor of 2 in the early years ( table 2 ) . * p value comparing the equivalence of incidence rates in 1 year to years 510 . p value comparing incidence rates after 1 year to years 510 , based on assumption that incidence rate in years 1 year was overestimated by a factor of 2 . numerous studies have documented elevated rates of tb among groups migrating from high - incidence areas to low - incidence areas . although some researchers have reported an immediate drop in incidence ( 6 ) , most have found , as we did , that the highest rates of incident disease occur nearest to the time of emigration and that a declining , although still elevated , risk persists for at least a decade . one exception is a recent study from the netherlands , which reported persistent elevations of incidence for a decade after emigration ( 4 ) ; of note , this study excluded all patients with cases occurring within 6 months of arrival on the basis that these early cases were likely to be prevalent cases . peri - immigration stress , malnutrition , and early overdiagnosis have been suggested as potential explanations for the fact that the highest tb incidence rates occur near arrival . the elevated risk that persists for decades has been attributed to the reactivation of latent infections ( 6 ) , continued transmission among a relatively insular immigrant population ( 8) , and disease secondary to migration back and forth from the country of origin ( 9 ) . we believe that the pattern of early decline and late stability in incidence seen here and in most studies ( and which may also have been seen by vos et al . had they not excluded the cases occurring in the earliest interval ) is consistent with the well - described 2-component phenomenon of disease occurrence after infection . the first phase ( lasting 12 years ) is dominated by the rapid progression of recent infections acquired in the country of origin , and the second phase ( lasting perhaps decades ) is defined by the relatively slow reactivation of latent infections acquired during a person 's lifetime before emigration ( 10 ) . the fact that the oldest immigrants from each country continue to have high incidence of disease 10 years after arrival likely reflects the increased lifetime risk for infection that they acquired while living in the high - incidence region . these dynamics , rather than stress , immune suppression , or malnutrition , may explain the patterns of disease occurrence after arrival . the steep decline in incident disease among the oldest cohorts within the first 2 years after arrival deserves special attention . while the current incidence of disease in the countries of emigration is very high , the annual risk for infection with tb over the past several decades has steadily declined . thus , a large proportion of those in the oldest age groups would have been infected with mycobacterium tuberculosis early in their lives . the observed decline in incidence after arrival is strikingly similar to observed patterns of declining risk for disease by time since infection ( 10 ) and provides compelling evidence that recent infection in the country of emigration is likely to be responsible for much early incident disease in the oldest age groups . the fact that most of these persons would have also been infected early in their lives argues that exogenous reinfection plays a key role in tb dynamics in high - incidence countries . this conclusion is consistent with other epidemiologic studies that report simultaneous decreases in incident disease across all age groups as the force of infection declines ( 11 ) ; molecular studies that document reinfection in high- ( 12 ) , moderate- ( 13 ) , and low - incidence settings ( 14 ) ; and theoretical work that supports the role of reinfection as necessary to explain historical trends of disease ( 15 ) . together , these studies argue against the notion that previous infection imparts immunity to future infection and disease . the degree to which natural infection protects a person from subsequent exposures remains an important unanswered question . our data support the assertion that persons living in areas with intense transmission are likely to be infected multiple times throughout the course of their lives and , if they progress to disease , are most likely to do so because they were recently infected . although the importance of exogenous reinfection to tb epidemics has been debated , our data reinforce arguments that reinfection likely plays a major role in high - incidence areas . this finding has numerous implications for designing and evaluating treatment programs in areas of high incidence . for example , in areas of intense transmission , persons receiving treatment who have intermittent or persistent smear - positive sputum may not be treatment " failures " but rather patients with multiple infections . those who plan empiric treatment strategies for patients with active disease should consider the drug - resistance profiles of currently circulating strains rather than those of strains observed in the past .

mathematical models and molecular epidemiologic investigation support the argument that exogenous reinfection plays an important role in tuberculosis transmission in high - incidence regions . we offer additional data from tuberculosis cases among recent us immigrants which strengthen the claim that reinfection in areas of intense transmission is common .

gossypiboma is the term used to refer to an intraoperative mistake discovered postoperatively in which one or more surgical sponges , gauze pads , or other form of textile is left behind in the operative field after the patient is closed . retained surgical sponges may become a nidus for infection and are often grounds for malpractice lawsuits . a retained foreign body after any surgery has medicolegal consequences , including mental agony , humiliation , huge monetary compensation , and imprisonment on the part of the surgeon , as well as increased morbidity , mortality , and financial loss on the part of the patient . surgical personnel s forgetting textile material or instruments during a procedure occurs during a complicated , extended , or emergency surgery . of course , the exact incidence of surgical gauze or other materials left behind in operated - upon patients is unknown . gossypibomas are most commonly found in the abdomen ( 56% ) , pelvis ( 18% ) , and thorax ( 11% ) . in 2010 , israel s health ministry enacted an ordinance regarding the counting of instruments and textile materials used in surgery . in 2012 , it was forced to declare cases of neglect concerning surgical materials . in the two subsequent years , they found 13 cases . in 2014 , a committee for patient safety control concluded that it was necessary to review and correct the ordinance of 2010 . despite precautions , a 34-year - old woman was admitted to the emergency room with abdominal pain and fever for the previous month ; she had undergone an emergency cesarean section nine years earlier . she suffered from abdominal pain for two months after the cesarean section ; however , subsequent visits to the treating hospital attributed the pain to surgical site infection . she was a healthy woman with two pregnancies : one normal delivery and the last , a cesarean section , as described . neither tumor nor hernia could be identified . a computed tomography scan of her abdomen and pelvis demonstrated an intra - abdominal and pelvic abscess covered by intestine in the lower abdomen . the patient was informed of the findings and the necessity of surgery , and she gave her consent . under general anesthesia , a midline laparotomy was performed in the lower abdomen , starting from the umbilicus . it revealed a large tumor may have been formed by the omentum and small intestine . the small bowel was easily separated from the tumor . after isolating the tumor conforming to the omentum , all the cultures of the pus were negative , and the direct visualization shows polymorphonuclear leukocytes . she was discharged home on postoperative day 3 and had no complications at the time of this writing . an exhaustive effort was carried out to find the operative count during the cesarean section , and we found a normal amount of devices and surgical sponges . the risk factors for foreign bodies forgotten in a patient are gynecologic , emergency , and general surgery . obesity patients also have an increased risk , and changes in the treatment team can also contribute to the issue . surgical sponges are the most common foreign materials retained ( 70% ) in the abdominal cavity because of their frequent usage and small size . moreover , a blood - soaked sponge in a hemorrhagic abdomen can be difficult to distinguish from blood . the clinical presentation of a retained foreign body looks like a pseudotumor or migration through the bowel . the symptoms are not necessarily specific ; they include abdominal pain , an abdominal mass , rectal bleeding , bowel obstruction , fever , diarrhea , and weight loss , , . serra describes a series of seven patients in which the median interval time between the operation and gauze removal was five years . liessi s paper reported on nine patients with an interval time between the operation and the diagnosis of seven days to 21 years . one patient per year in every hospital suffers from a forgotten foreign body . besides the morbidity affecting the patient , a forgotten foreign body could involve a lawsuit for damages against the surgeon and the nurses affected . the compensation that courts decide upon in these cases is extremely high ; however , above all , the surgeon loses his or her good name and reputation . the promotion of safety requires that all personnel involved in a healthcare procedure be aware that the potential for errors exists , and that teamwork and communication are essential for preventing errors . however , whenever accounting for material depends on humans , mistakes will continue to be committed . retained sponge and other material remains a major problem for the patient , even today , despite preventive measures taken by the surgeon and the hospital . consent was obtained from the patient and her family after we explained that this publication is anonymous . sergio susmallian : conception and design , acquisition of data , critical revision of the article , final approval of the version to be published . benjamin raskin : acquisition of data , drafting the article , final approval of the version to be published . royi barnea : analysis and interpretation of data , drafting the article , final approval of the version to be published .

highlightsgossypiboma consist in retained surgical sponges are more usual than the reported.a young patient nine years after cesarean section with abdominal pain and fever.an abscess was diagnosed in the lower abdomen by ct.during laparotomy , a sponge was extracted from a large abscess.she had a normal post - operative course .

a 32-year - old woman presented with progressive swelling , pain and a pulsatile mass in the right antecubital space . she had undergone aneurysmectomy and autologous saphenous vein graft interposition at the same site in another hospital three years ago . 1 ) . physical examination revealed a pulsatile mass in the right antecubital space measuring 54 cm in size . her vital signs were stable with a heart rate of 72 beats / min , systolic blood pressure of 120 mmhg , respiratory rate of 18 breaths / min , body temperature of 36.5. other laboratory examination findings were within their normal ranges . the aorta , pulmonary arteries and the other peripheral arteries were normal on preoperative computed tomography . a longitudinal incision was made on the prior surgical incision scar and adhesiolysis was performed . the saphenous vein graft which is anastomosed during the previous surgery was intact , but the distal part of the anastomotic site showed aneurysmal change . aneurysmectomy including the implanted saphenous vein graft and autologous vessel graft interposition with the new saphenous vein graft which was harvested at the distal portion of the left leg were performed . after anastomosis of the saphenous vein graft at both the proximal and distal part of the brachial artery , the distal radial artery showed good patency on intraoperative doppler examination . the histopathologic examination revealed elastic fiber disruption of the brachial artery and saccular dilatation of aneurysm ( fig . the patient was discharged without complications on the 10th day after surgery . during follow - up at the outpatient department the mechanism of aneurysm formation is considered to be compression of the arterial wall , producing contusion of the media and subsequent weakness of the wall and fusiform dilatation . as with other arterial aneurysms , there are two types of aneurysms ; true aneurysms and pseudoaneurysms of the brachial artery . the pseudoaneurysm can be a complication of cardiac catheterization performed for congenital heart disease , arterial and venous puncture for the evaluation of blood gases , and invasive arterial monitoring etc . true aneurysm , which is even more rare , can be caused by arteriosclerotic , congenital , and metabolic disorders , and can be associated with diseases such as kawasaki 's syndrome , buerger 's disease , kaposi 's sarcoma , and cystic adventitial disease . in our case , the patient did not have any of the previously mentioned diseases and other diseases of thromboembolic origin . we suggest that trauma to the vessel or to the remnant aneurysmal sac of the previous surgery could be the cause of recurrence . most of the patients visited the hospital with a pulsatile mass in the antecubital space [ 1 - 6 ] . doppler ultrasonography , computed tomographic arteriography ( cta ) , and selective upper extremity angiography can be performed for establishing the diagnosis of aneurysm . but , cta is the preferred method for diagnostic evaluation of upper limb vascular diseases , due to its high imaging capacity , no arterial invasion , and absence of radiation exposure . since the brachial artery aneurysm is a rare disease globally , the exact surgical procedure and definite treatment option for true upper extremity artery aneurysm has not been established . ultrasound - guided compression and injection of thrombin , radiologic intervention and surgical resection , all could be considered as one of the therapeutic modalities . ultrasound - guided compression and thrombin injection has the advantages of a non - invasive procedure , but significant disadvantages such as pain and less efficacy in anticoagulation patients are associated with it . radiologic intervention for the brachial artery aneurysm was not possible or was not advisable around the elbow joint . a covered stent would have been technically feasible but certainly not indicated in an area developing such flexion amplitude . since there are not many reports of brachial artery aneurysm , comparison of the nonsurgical and the surgical treatment is controversial . considering that many authors have reported surgical resection as the therapeutic modality [ 1 - 5 ] , the best therapeutic option in brachial artery aneurysm could be operative repair , and it should be performed without any delay , in order to prevent upper extremity ischemic sequelae . when surgical resection is performed , it is important that the aneurysm wall with the changes should be resected with adequate resection margins . in our case , the previous saphenous vein graft was intact without any aneurysmal changes , but the distal portion of the anastomosis of the brachial artery showed aneurysmal changes . it can be considered that the changes in the remnant aneurysm , either macroscopic or microscopic , were the sites of recurrence after surgery . it is also considered that a traumatic procedure and instrument application such as careful snaring and vessel clamping is needed so as not to injure the native vessels . we have reported a case of recurrent true brachial artery aneurysm with review of the literature .

true aneurysm of the brachial artery is a rare disease entity . the mechanism of aneurysm formation is considered to be compression of the arterial wall , producing contusion of the media and subsequent weakness of the wall and fusiform dilatation . it can be caused by arteriosclerotic , congenital , and metabolic disorders , and can be associated with diseases such as kawasaki 's disease . doppler ultrasonography , computed tomography , arteriography , and selective upper extremity angiography may be performed for establishing the diagnosis of aneurysm . the best therapeutic option is operative repair , and it should be performed without any delay , in order to prevent upper extremity ischemic or thrombotic sequelae . here , we report a case of recurrent brachial artery aneurysm with review of the literature .

central serous chorioretinopathy ( csc ) is characterized by an idiopathic serous detachment of the neurosensory retina at the macula resulting from deficient pumping and altered barrier function at the level of the retinal pigment epithelium ( rpe ) . it is characterized by multifocal , irregularly distributed and often widespread rpe changes associated with varying degrees of low - grade leakage . chronic csc may be associated with persistent subretinal exudation , extensive rpe atrophy , cystoid macular degeneration , and choroidal neovascularization . these factors lead to a less favorable visual prognosis [ 3 , 4 ] . vascular endothelial growth factor ( vegf ) anti - vegfs , through their antipermeability characteristics and hence leakage reduction , may be useful in the treatment of cases with refractory csc and may be helpful as an alternative management . early studies indicated that intravitreal injections of bevacizumab ( avastin ) appeared to be safe and effective for the management of chronic csc . however , clinical and experimental investigations have reported possible circulatory changes as a complication of intravitreal bevacizumab , consisting of significant depletion of choriocapillaries in primate eyes , progression of capillary nonperfused areas in rabbit eyes , and multiple retinal hemorrhages in eyes with diabetic retinopathy [ 6 , 7 ] . here , we report a case of hemorrhagic macular infarction after intravitreal bevacizumab for chronic multifocal csc . a 56-year - old male presented with complaints of gradual loss of vision and distortion in the right eye which had started 2 years before . his best - corrected visual acuity ( bcva ) in the right eye was 20/40 on snellen 's chart and intraocular pressure was 10 mm hg . anterior segment findings on slit - lamp examination showed immature cataract in both eyes , the rest was within normal limits . dilated fundus examination ( fig . 1 ) of the right eye showed presence of retinal pigmentary changes and absence of foveal reflex . oct showed a shallow rpe elevation , with reflectivity under the rpe layer suggestive of nonserous pigment epithelial detachment ( ped ) , along with minimal subretinal fluid . 1 ) of the right eye revealed a 2.5-mm disc diameter of mottled hyperfluorescence increasing in size and intensity in the macular area diagnostic of chronic multifocal csc and the left eye showed mottled hyperfluorescence that was not increasing in size or intensity . after obtaining signed informed consent , the patient received an intravitreal injection of 0.05 ml bevacizumab under all aseptic precautions , and prophylactic antibiotic eye drops were given for 7 days subsequently . one - day and one - week follow - up visits showed stable fundus with a right - eye bcva of 20/40 on snellen 's chart . one month after the injection , the patient presented with a sudden diminution of vision in the right eye starting 1 week before . the bcva in the right eye was counting fingers at 1 m and intraocular pressure in the right eye was 10 mm hg . 2 ) , there were multiple hemorrhages , ped and extensive hard exudates at the macula , inferior to the arcade as well as nasal to the disc . 2 ) showed an increase in the sensory detachment with fluid accumulation in the sensory retina and a small ped . small areas of blocked fluorescence were also noted , which was suggestive of hemorrhagic macular infarction . the reported case shows the clinical and angiographic findings of hemorrhagic macular infarction after intravitreal bevacizumab for chronic multifocal csc . bevacizumab is not currently approved by the fda for intravitreal injection ; however , several reports state that off - label intravitreal bevacizumab for chronic csc results in an overall improvement in visual acuity and macular anatomy and appears to be safe and effective in the short term . in recent years , choroidal hyperpermeability has been assumed to be the cause of csc based upon indocyanine green angiography and has been considered a basis of photodynamic treatment . an intraocular injection of anti - vegf bevacizumab may improve the symptoms of csc by blocking the activity of vegf . to date , however , no studies have demonstrated a correlation between hyperpermeability of the choroidal vessel and vegf . . showed that in cases with chronic csc , intravitreal bevacizumab injection improved bcva and reduced central macular thickness and recurrence did not occur in any case during the follow - up period . there are also case reports discussing hemorrhagic macular infarction after intravitreal bevacizumab for macular edema in vascular occlusions and age - related macular degeneration [ 8 , 9 , 10 ] . in all these cases , patients had one or more significant medical history ( i.e. hypertension , diabetes , ihd , etc . ) and they were on a number of medicines ( i.e. aspirin , anticoagulants , other antiplatelet and antithrombotic agents , etc . ) , which , along with avastin , could increase the possibility of drug - induced hemorrhagic infarct . the peculiarity of our case is that our patient had had no significant ocular or medical history until the time he visited us for the first time . clinical trials for bevacizumab in the treatment of metastatic colon cancer demonstrated increased risk of arterial thromboembolic events , hypertension , proteinuria and congestive heart failure . even the fda has reported severe adverse events with avastin such as serious or fatal hemoptysis in patients with adenocarcinoma ( 4% incidence rate ) and gastrointestinal perforation in patients with colorectal cancer ( 2% incidence rate ) . however , there is no substantial data on the safe use of intravitreal bevacizumab and it remains off - label for ocular use . to conclude , we have hereby reported a case of hemorrhagic macular infarction after intravitreal bevacizumab for chronic multifocal csc , which has not been reported before to the best of our knowledge . we urge clinicians to carefully evaluate their patients before giving bevacizumab for ocular indications , and we hope that the medical community will take time to report such events if they come across them in practice . we might find a warning sign , and we may prevent any unintended harm to our patients .

we hereby report a case of hemorrhagic macular infarction after intravitreal bevacizumab for chronic multifocal central serous chorioretinopathy ( csc ) . issues regarding safety and adverse effects of bevacizumab are discussed . to the best of our knowledge , this is the first reported case of hemorrhagic macular infarction after intravitreal bevacizumab for chronic multifocal csc .

the germ cell nuclear factor ( gcnf , also known as retinoid acid receptor - related testis - associated receptor , neuronal cell nuclear receptor or nr6a1 ) is an orphan receptor in the nuclear receptor superfamily found in mammals , amphibians and fish . the mouse gcnf gene is expressed in the placenta and the developing nervous system and germ cells , and responds to retinoic acid . we have defined the intron - exon structure of the mouse gcnf gene and found that it contains 11 exons . exons 1 - 4 encode the 75 amino acid amino - terminal domain and exon 4 also encodes the core dna - binding domain . the carboxy - terminal extension is encoded by exon 5 , exons 6 and 7 encode the hinge region , and exons 7 - 11 encode the putative ligand - binding domain . unusually , the two zinc - finger motifs in the dna - binding domain are encoded by separate exons . the germ cell nuclear factor ( gcnf , nr6a1 ) is a member of the nuclear receptor superfamily . originally isolated from mouse cdna libraries , homologs of gcnf as no ligand has been identified , gcnf is designated an orphan receptor . also known as rtr ( retinoid acid receptor - related testis - associated receptor ) or ncnf ( neuronal cell nuclear receptor ) , evolutionary studies have defined gcnf as the only known member of a sixth subfamily of nuclear receptors . the mouse gcnf gene is highly expressed in the developing nervous system , in the labyrinthine layer of the placenta and in the developing germ cells . two transcripts of approximately 7.5 kb and 2.4 kb are present in testis , but only the larger transcript is found in somatic cells . hybridization experiments reveal that the size difference is at least partially due to the use of different polyadenylation sites . interestingly , gcnf expression is transiently up - regulated and later down - regulated again when embryonal carcinoma cells are triggered to differentiate by retinoic acid . we have isolated genomic clones encompassing the mouse gcnf gene , and have defined the intron - exon structure of the gene . sequence analysis reveals that the coding region of gcnf comprises 11 exons and 10 introns ( table 1 ) . a bacteriophage lambda library and a cosmid library of genomic dna of the mouse 129 strain were screened with the full - length gcnf cdna . the dna from colonies that hybridized was cloned into pbluescript ( sk ) for further sequence analysis . exons 3 and 4 were identified from bacteriophage subclones , and exons 6 - 11 were identified in cosmid - derived subclones . additional intron - exon boundaries and the 5'-untranslated region ( 5'-utr ) were identified by genome walking analysis following the manufacturer s instructions ( clontech ) . dna sequencing was performed on an abi 377-sequencer using the dye terminator protocol ( perkin elmer ) and on a dna sequencer model 400 ( li - cor ) . the dna sequences were processed using the wisconsin package version 10.0 of the genetics computer group ( gcg ) , madison , wisconsin . all intron - exon junctions obeyed the gt / ag rule ( and table 1 ) . the location of the intron - exon junctions relative to the peptide sequence is shown in figure 1 . exon 1 contains the 244 bp untranslated sequence at the 5 ' end of the cdna and codes for the first 33 amino acids ( figure 2 ) . (; genbank entry mmu09563 ) , starts with an ecori site that is present in the genomic dna . the t at position 174 is a g in our genomic isolate , which could represent a genomic variant . as no promoter has been identified for gcnf , the sequence preceding the ecori site may contain promoter elements . it is also possible , however , that the promoter precedes a not - yet - identified additional exon in the 5'-utr of gcnf . the amino - terminal domain of 75 amino acids is encoded by exons 1 - 4 . exon 4 also codes for the core dna - binding domain ( dbd ) of 66 amino acids and for three additional amino acids ( figure 1 ) . the dbd consists of two zinc - finger motifs that are encoded by separate exons in most vertebrate nuclear receptor genes , except for those of the coup transcription factor subfamily . evolutionary studies do not provide further evidence that these receptors are closely related to gcnf . a further domain important for dna binding and for homodimeric interactions , and known as the dbd carboxy - terminal extension , is encoded by the 56 bp of exon 5 . the sizes of intron 2 and intron 4 were determined by pcr amplification of mouse genomic dna . exons 6 and 7 code for the hinge region , whereas exons 7 - 11 code for the putative ligand - binding domain . a variant of the typical auaaa polyadenylation signal ( aguaaa ) and the cleavage site that is used in the testis are part of the eleventh exon . we think the genomic structure of this first , and at present only , member of the sixth subfamily of nuclear receptors will be useful for further studies of this unique receptor . the location of the different exons in the gcnf amino - acid sequence . the core dna - binding domain is underlined . the location of the ecori site ( gaattc ) marking the 5'-end of the gcnf cdna ( genbank entry mmu09563 ) and the putative translational start codon ( atg ) are underlined . organization of the mouse gcnf gene lower - case letters are used for the intron sequence and capital letters for the exon sequence . schaller for her support of this work in her institute , and m. dumigen and w. kullmann for sequencing .

background : the germ cell nuclear factor ( gcnf , also known as retinoid acid receptor - related testis - associated receptor , neuronal cell nuclear receptor or nr6a1 ) is an orphan receptor in the nuclear receptor superfamily found in mammals , amphibians and fish . the mouse gcnf gene is expressed in the placenta and the developing nervous system and germ cells , and responds to retinoic acid.results:we have defined the intron - exon structure of the mouse gcnf gene and found that it contains 11 exons . exons 1 - 4 encode the 75 amino acid amino - terminal domain and exon 4 also encodes the core dna - binding domain . the carboxy - terminal extension is encoded by exon 5 , exons 6 and 7 encode the hinge region , and exons 7 - 11 encode the putative ligand - binding domain . unusually , the two zinc - finger motifs in the dna - binding domain are encoded by separate exons.conclusions:the protein - coding region of gcnf is contained in 11 exons . the genomic structure of this nuclear receptor gene will be useful for further studies .