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dichlorvos ( dimethyl-2 , 2-dichloro vinyl phosphate ) is an organophosphorous ( op ) insecticide with moderate human toxicity , used for insect control in food storage areas , greenhouses , barns and control of insects on livestock . parenteral dichlorvos administration with suicidal intention is rarely reported in the literature . in one case report , extensive muscle necrosis was reported following intramuscular injection of dichlorvos . in another case , following injection of dichlorvos subcutaneously in both wrists , a young patient developed compartment syndrome requiring fasciotomy . in both cases , i describe the case of a young female patient , who developed atypical signs of op poisoning following subcutaneous injection of dichlorvos . the present case report is about a 37-year - old , previously healthy female , who was admitted to our intensive care unit 18-h after alleged self - injection of 76% dichlorvos ( nuvos ) 3 ml in the upper left arm ( 3 times with a 1 ml insulin syringe ) . within an hour of the injections , she had normal body temperature with heart rate of 90/min , blood pressure 110/70 mmhg , respiratory rate 14/min and pulse oximeter showing 100% saturation on room air . no crackles were heard on chest auscultation . on the nervous system examination , she was jittery with slurred speech and normal cranial nerves . she had hypertonia , brisk tendon reflexes , bilateral sustained ankle clonus and bilateral extensor plantar reflex . local examination revealed a blister and a black necrotizing lesion over the anterior aspect of the left arm [ figure 1 ] . she was rapidly atropinized with incremental boluses of atropine ( total dose 12 mg ) in next 30 min ( targeting a dry mouth ) followed by infusion of 1.8 mg / h . pralidoxime infusion was started at 500 mg / h following a loading dose of 1000 mg over 20 min . intravenous clindamycin ( 600 mg q 8 h ) was administered empirically for presumed local wound infection . debridement of the wound showed predominant involvement of the subcutaneous tissue plane with minimal necrosis of the superficial fibers of biceps brachii . initial routine laboratory investigations were unremarkable except elevated total count ( 16200/mm ) and mild respiratory alkalosis . local skin necrosis with surrounding blisters her clinical course was complicated by respiratory arrest 9-h after hospital admission ( 27-h after the injection of dichlorovos ) leading to hypoxia and bradycardia . arterial blood gas analysis post resuscitation showed - ph 7.28 , po2 - 36 mmhg , pco2 - 54 mmhg , hco3 - 22 mmhg . serum choline esterase ( pseudo cholinesterase ) level of the same day showed a very low value of < 1 ku / she could be successfully weaned off from the ventilator on day 4 . on day 5 , while on atropine infusion she was found to be delirious . she was also noticed to have extrapyramidal manifestations including rigidity and left sided gaze preference . in this case , other than the unusual route of administration , a number of atypical manifestations were found . this includes relatively delayed respiratory failure and central nervous system ( cns ) manifestations with few muscarinic signs . two independent mechanisms are thought to be responsible for respiratory failure following op insecticide poisoning cholinergic cns depression in the early stage ( associated with other cholinergic features ) and late progressive neuromuscular transmission defect . second mechanism is part of the syndrome described as type ii paralysis by wadia et al . or intermediate syndrome by senanayake and karalliedde . our patient developed respiratory arrest 27-h after exposure to dichlorovos . in her clinical course she never had neck or proximal muscle weakness , though the clinical findings were not correlated electrophysiologically . usually cns signs are manifested early in the course in the presence of peripheral muscarinic signs . in a large series published on neurological manifestations of op compounds , 8 of 200 patients had pyramidal tract signs in the form of only hyper - reflexia manifesting within 24 h of exposure . all these patients were unconscious with florid cholinergic manifestations , including constricted pupils . in their original series of intermediate syndrome senanayake and karalliedde , observed hyperreflexia in one of their patients ( with flexor plantar reflex ) and even she had typical muscarinic signs . extrapyramidal signs of op poisoning are often transient and may be missed in a sick patient . in a taiwanese series cns manifestations ( both pyramidal tract and extrapyramidal ) were delayed and were associated with relatively scanty peripheral muscarinic signs . possible explanation for these atypical manifestations in our patient could be route specific ( in this case subcutaneous ) effect of op compound ( dichlorovos ) causing isolated delayed cns depression , with few peripheral cholinergic signs . animal experiments have shown route specific ( subcutaneous or intravenous ) cardiovascular and neuromuscular effect of op compounds in rats .

current case report describes a 37-year - old female patient who was admitted to the hospital following subcutaneous injection of dichlorvos with an insulin syringe . the only peripheral cholinergic sign observed on admission was excessive salivation with bilateral pyramidal tract signs . locally she had necrosis of skin and subcutaneous tissue with surrounding blisters . in the subsequent course of her illness , she developed respiratory arrest requiring ventilator support . she also had delayed extrapyramidal manifestations . relevant literature is reviewed . possibility of route - specific , delayed predominant central nervous system effect of dichlorvos postulated .

acute appendicitis is considered as the most frequent cause of acute abdomen in young patients with a slight predominance in males . although its clinical diagnosis is simple , 20 - 30% of appendicitis cases are reported to be gangrenous or perforated because of delayed diagnosis , which can lead to increased morbidity and mortality . additionally , up to 10% of patients especially older ones , immunocompromised patients , and pregnant women are diagnosed with atypical presentations such as appendiceal mass which requires long hospital admissions , intravenous antibiotics , and often needs percutaneous drainage . using colonoscopy for the diagnosis and treatment of asymptomatic acute appendicitis and appendiceal abscess are rare . in unusual appendicitis cases , especially in the presence of abdominal comorbidities , colonoscopy is used ; although colonoscopy is not considered as the first line intervention because of complications risk such as bleeding or perforation . furthermore non - invasive tests such as ultrasonography and computed tomography with more sensitivity can be used . we report an atypical case of appendiceal abscess , which was diagnosed during colonoscopy . a 30-year - old man referred for evaluation to our center with epigastric pain from three days prior to his evaluation . the pain was nonspecific and colicky distributed in periumblical region , did not radiate to any site and was unrelated to bowel movements . he had experienced also nausea , anorexia and intermittent vomiting . there was a history of marijuana and alcohol usage from several years ago . upon physical examination , he was ill with restlessness . other laboratory values such as hemoglobin , platelets , serum amylase , erythrocyte sedimentation rate , aspartate aminotransferase , alanine aminotransferase , alkaline phosphatase , and electrolytes were normal . his workup on admission including ; chest x - ray , abdominal x - ray , and abdominal ultrasonography were reported normal . an upper gastrointestinal endoscopy was performed after four days of admission that was normal . within second week after admission , the appendiceal orifice was discovered in the floor of cecum which was draining pus into the lumen ( figure 1 ) . purulent discharge from the appendiceal orifice before ( up ) and after ( down ) pressing by forceps ( arrow ) result of the biopsies of the mucosa at the base of the appendix showed mild nonspecific inflammation with some necrotic materials and staphylococcus organism . the biopsies of the mucosa at terminal ileum , left and right colon demonstrated mild nonspecific inflammation . a second abdominal ultrasonography was performed that showed a fluid collection ( about 70 40 mm ) in favor of appendiceal abscess in right lower quadrant ( figure 2 ) . the patient was scheduled for spiral abdomen - pelvic computed tomographic scan which showed a hypodense well defined collection with air fluid level ( about 70 40 30 mm ) in right lower quadrant . the collection was extended to pelvic cavity associated with inflammation of the surrounding fat in favor of appendiceal abscess ( figure 3 ) . ultrasonography showing a fluid collection in favor of appendiceal abscess computed tomographic scan showing a hypodense well defined collection with air fluid level associated with inflammation of surrounding fat in favor of appendiceal abscess ( arrow ) . one week after antibiotic therapy a third abdominal ultrasonography was performed that showed a smaller fluid collection ( about 40 15 mm ) around the appendix . three weeks after antibiotic therapy the patient was asymptomatic with normal white blood cell count . on the 5th week after antibiotic therapy , exploratory laparotomy did not show any mass or collection in the appendiceal region and elective appendectomy was performed . after 3 months of follow - up the patient was well without any complaint or physical finding . acute appendicitis is one of the most frequent causes of acute abdomen . despite the use of scores in the radiological evaluation , and laparoscopic advances , in a cohort study of patients with appendiceal abscess or phlegmon due to acute appendicitis concluded that initial conservative treatment should be considered . colonoscopy is reported as a diagnostic and therapeutic approach for delayed or atypical presentations of acute appendicitis . our patient is one of these unusual cases among whom complicated acute appendicitis was incidentally diagnosed by colonoscopy . although the appendiceal orifice is routinely examined during colonoscopy , however there are only few published reports describing acute appendicitis diagnosis in the appendiceal orifice . in a review article , khawaja fi recommended that colonoscopy should only be done in selected patients with atypical appendicitis or uncertain diagnosis of appendiceal perforation . benatta ma reported an atypical case of pericecal appendiceal abscess diagnosed and treated at colonoscopy , while initial presentation was without any suggestive symptoms of appendicitis . wade ad et al reported an atypical case of appendicitis in a patient with ulcerative colitis diagnosed during colonoscopy . in this report christoffersen mmw et al reported an atypical case of periappendicular abscess diagnosed and treated at colonoscopy , while the patient was admitted for colonoscopy polyp control without any suggestive symptoms . kawahara y et al reported a case of appendiceal abscess with draining pus from appendiceal into the lumen diagnosed at colonoscopy and ct scan . in this report because of spontaneous drainage of abscess into the lumen , the appendiceal abscess had resolved by the time of surgery . kapral c et al . reported a case of pericecal abscess that treated by drainage at colonoscopy and antibiotic therapy . they intubated the appendix by sphincteroltomy catheter which was placed into the abscess cavity . petro m reported a case of the earliest stage of acute appendicitis in an asymptomatic patient undergoing colonoscopy for evaluation of colorectal cancer . for evaluation of the role of colonoscopy in the diagnosis of atypical appendicitis , a retrospective study in 21 patients performed by chang hs et al . some of acute appendicitis cases are reported to be complicated , due to delayed diagnosis . this unusual case highlights a patient with an appendiceal abscess diagnosed by colonoscopy . because of spontaneous drainage of abscess into the lumen of colon and antibiotic therapy , although the diagnostic and therapeutic role of colonoscopy in these patients is uncertain , however , it s use can be selected in special situations in the future .

although complicated acute appendicitis is occasionally observed in general practice , the diagnosis of appendiceal abscess by colonoscopy is unusual . we report a case of appendiceal abscess with spontaneous drainage into the lumen of colon diagnosed during colonoscopy and resolved by the time of surgery .

they possess binding sites specific to carbohydrates and have the capacity to interact with molecules of biological fluids and those present in cellular surfaces . lectins can substitute natural linkers and activate cellular responses through different roads of intracellular signalling or endocytosis of formed compounds [ 3 , 4 ] . camaratu or camaratuba bean ( cratylia mollis ) is forage of the northeast semi - arid region from brazil . molecular forms of c. mollis seed lectin ( cramoll 1 , cramoll 2 , cramoll 3 , and cramoll 4 ) have been highly purified and characterized ( de souza et al . , cramoll 1 , cramoll 2 , and cramoll 4 specifically bind glucose / mannose , while cramoll 3 is a galactose - specific glycoprotein . preparations containing isoforms ( cramoll 1,4 ) and isolectins ( cramoll 1,2,3 ) , as well as cramoll 1 , have been studied in structural analyses and for the most several biotechnological applications ( de souza et al . , 2003 ) [ 612 ] . the use of nuclear technique for the evaluation of biomolecules and medicines although old is not widely used even nowadays . since the discovery of the element technetium , in 1947 scintigraphy imaging provides a dimensional method for exactly locating gamma emitters in a noninvasive procedure under in vivo conditions . for the characterization of drugs , and biomolecules , molecular imaging techniques are extremely helpful to follow biodistribution in experimental animal studies . in this study , the isolectins cramol 1,4 were radiolabeled with tc-99 m for the evaluation of the biodistribution and in order to forecast a possible use as radiopharmaceutical in healing process . the labeling processes were made using 150 l of cramol ( isoform 1,4 ) solution incubated with stannous chloride ( sncl2 ) solutions ( 80 l / ml ) ( sigma - aldrich ) for 20 minutes at room temperature . then , this solution was incubated with 100 ci ( approximately 300 l ) of technetium-99 m ( ipen / cnen ) for other 10 minutes in order to label the cramol 1,4 ( here just called cramol ) with tc-99 m . in order to characterize the labeled cramol , thin - layer chromatography ( tlc ) was made using paper whatman no . the tlc was performed using 2 l of a labeled sample in acetone ( proquimios ) as a mobile phase . the radioactivity of the strips was verified in a gamma counter ( packard , cobra ii ) as described in table 1 . the institutional review board and the animal ethics committee approved the study protocol for this study . the labeled samples ( 3,7 mbq/0,2 ml ) planar images were obtained 1 hour after injection at a millennium gamma camera ( ge healthcare , cleveland , usa ) . counts were acquired for 5 min in a 15% window centered at 140 kev ( figure 1 ) . then , animals were sacrificed , their were organs removed and weighted , and the radioactivity uptake was counted in a gamma counter ( packard - cobra ii ) . results were expressed as the percentage of injected dose per organ and percent of injected dose per gram of tissue ( table 2 , figures 2 and 3 ) . , the results showed that the acetone can be used for this purpose . in a triplicate test , the free pertechnetate was eluted to the top of the chromatograms , and the cramol stayed in the bottom . moreover , the results observed in the chromatogram elucidated the question about the efficacy of the labeling process , corroborating that the cramol was efficiently labeled with tc-99 m . tc - cramol scintigraphy 1 h after injection shows liver , kidneys , and bladder . as we can see , the great part of the lectin was processed by the liver . this is absolutely normal especially for injectable drugs , since almost all the drugs are first processed by the hepatic system ( figure 2 ) . otherwise , when dissecting , weighting and counting the organs , a suspicious data appeared . as a protein , we were expecting that its clearance would be made by the kidney , and in fact , it occurred . however , the rate between the bowel and the kidney is over the double . in this case , the clearance time would be reduced and the reabsorption of the drug could be facilitated by the microvilli of the bowel . in this case a dosage error could occur or even a toxic reaction due its accumulation in the bowel . in both cases , further studies should be made to clarify this mechanism and the relevance of that information in a developed drug based on lectin similar to the cramol 1,4 . it is interesting to notice , analyzing both figures 1 and 2 , that the lectin can not pass the hematoencephalic barrier . and in this case , the lectin does not represent a risk for the brain function . the radiolabeling of the cramol showed that this lectin has an uncommon behavior specially related to the clearance mechanism that we believe must be more studied . it also showed that the nuclear technique can be very helpful for the development of drugs and medicines .

the cramoll 1,4 is a well - studied lectin . however , few studies about its biodistribution have been done before . in this study , we radiolabeled the cramol 1,4 with tc-99 m and analyzed the biodistribution . the results showed that the cramol has an abnormal uptake by the bowel with reflections on its clearance mechanism .

turmeric ( curcuma longa l. ) of family zingiberaceae is industrially as well as pharmaceutically important crop extensively cultivated in india . it is a vegetatively propagated , polyploid crop commonly known as golden spice . because of its multipurpose use as food preservative , spice , therapeutic agent and natural dye , it is acquiring more importance in cosmetics , food and pharmaceutical industries , . in turmeric number of cultivars has been developed with different characteristics like high curcumin content , high essential oil content , rhizome yield and disease tolerance etc . but their asexual mode of reproduction and poor genetic makeup has creates confusion towards authentic identification . single nucleotide polymorphism ( snp ) study could be useful to solve these problems by comparing sequence of each variety with each other . in this current study , we conducted reference based transcriptome assembly of suvarna , a well - known turmeric cultivar which could latter on used to get potential snps by comparing with other varieties towards proper identification . fresh mature rhizomes of suvarna variety has been collected from centre for biotechnology , siksha o anusandhan university , bhubaneswar , india . these rhizomes were washed properly with distilled water and suspended in rna later solution for further analysis . rna extraction and library preparation has been done by using illumina truseq rna library tool kit . 75 bp paired - end sequencing has been done using illumina nextseq500 sequencing platform which developed5 gb of raw data . we have precisely given detail information about the reference based transcriptome assembly of suvarna cultivar ( table 1 ) . the transcriptome assembly of suvarna developed 42994 numbers of transcripts , with median transcript length 773 and n50 value 1216 . we annotated the transcripts using various databases like go , kegg , kog , plantcyc etc . as per our understanding transcriptome analysis of cultivar suvarna has been done for the first time and this result could be efficiently used for development of markers such as ssrs and snps for identification of suvarna from its closely similar turmeric cultivars . fresh mature rhizomes of suvarna variety has been collected from centre for biotechnology , siksha o anusandhan university , bhubaneswar , india . these rhizomes were washed properly with distilled water and suspended in rna later solution for further analysis . rna extraction and library preparation has been done by using illumina truseq rna library tool kit . 75 bp paired - end sequencing has been done using illumina nextseq500 sequencing platform which developed5 gb of raw data . we have precisely given detail information about the reference based transcriptome assembly of suvarna cultivar ( table 1 ) . the transcriptome assembly of suvarna developed 42994 numbers of transcripts , with median transcript length 773 and n50 value 1216 . we annotated the transcripts using various databases like go , kegg , kog , plantcyc etc . as per our understanding transcriptome analysis of cultivar suvarna has been done for the first time and this result could be efficiently used for development of markers such as ssrs and snps for identification of suvarna from its closely similar turmeric cultivars .

turmeric is an economically valued crop , because of its utility in the food , pharmaceutical industries and ayurvedic medicine , attracts the attention in many areas of research work . in the present study , we executed resequencing through transcriptome assembly of the turmeric cultivar suvarna ( cl_suv_10 ) . resequencing of suvarna variety has generated 5 gbases raw data with 75 bp paired - end sequence . the raw data has been submitted to sra database of ncbi with accession number srr4042181 . reads were assembled using cufflinks-2.2.1 tool which ended up with 42994 numbers of transcripts . the length of transcripts ranged from 83 to15565 , with a n50 value 1216 and median transcript length 773 . the transcripts were annotated through number of databases . for the first time transcriptome profiling of cultivar suvarna has been done , which could help towards identification of single nucleotide polymorphisms ( snps ) between suvarna and other turmeric cultivars for its authentic identification .

ventriculoperitoneal ( vp ) shunting is commonly utilized as a diversion procedure for patients with hydrocephalus . multiple techniques for initial placement of the catheters have been described , including conventional minilaparotomy , peel - away introducer sheaths , as well as full laparoscopic approaches . vp shunts fail for a variety of reasons , including failure secondary to disconnection of abdominal tubing with migration of the tubing , most commonly further into the peritoneal cavity ; this occurs in 2.5% to 4% of shunts placed according to current published series . initial shunt failure rates continue to be high , with a reported failure rate of 31% at 6 months and an infection rate of 7% . common presenting features of shunt failure include headache , mental status changes , and vomiting . laparoscopy avoids laparotomy and may provide improved confirmation of shunt functionality and placement at the time of revision . a 31-year - old obese female was initially treated for pseudotumor cerebri with placement of a lumbar peritoneal shunt in 2003 . after 5 failed revisions for nonfunction , a ventriculoperitoneal shunt was placed in september 2007 . the patient subsequently presented 11 days postoperatively with headaches , dizziness , and formation of a seroma in her right upper quadrant . ct scan revealed the vp catheter tubing coiled in the subcutaneous tissue of the right upper quadrant . four 5-mm titanium clips were placed on the catheter in an attempt to prevent repeated migration of the tubing . one month later , the patient presented with recurrence of headaches , nausea , and right upper quadrant abdominal pain . ct scan was again obtained , revealing that the shunt tubing had migrated back into the subcutaneous tissue ( figure 1 ) . a laparoscopic approach was again utilitzed for retrieval and attempted improved fixation of the vp shunt . adhesions were taken down at the original entry site of the shunt , revealing a 2-cm fascial defect in the abdominal wall with the shunt tubing coiled within it ( figure 2 ) . the tubing was brought down through the defect out of the subcutaneous space and into the peritoneal cavity ( figure 3 ) . one port was upsized to 10 mm , and the tubing was externalized through the port and secured to a 3-cm x 10-cm piece of ptfe mesh with 30 prolene sutures ( figure 4 ) . csf flow was confirmed , and the tubing attached to the mesh was reintroduced into the peritoneal cavity . the mesh was secured to the abdominal wall by using spiral tacks and 0 gore - tex sutures ( figure 5 ) . the separate 2-cm fascial defect was repaired with a 10-cm x 10-cm piece of ptfe mesh placed as an onlay with the vp shunt exiting from its inferior border . it was secured with spiral tacks and 0 gore - tex sutures for transabdominal fixation ( figure 6 ) . the patient was seen in follow - up 7 months postprocedure and reported no recurrence of her symptoms . traditionally , laparotomy was necessary for both peritoneal shunt placement as well as subsequent shunt revisions . the advent of laparoscopy has afforded a minimally invasive approach that has resulted in decreased patient morbidity related to shunt placement . retrieval and repositioning of a displaced vp shunt in the peritoneal cavity using laparoscopic techniques has been previously described . in the present case , our subsequent laparoscopic approach utilized ptfe mesh , well described in the use of laparoscopic ventral hernia repair , to fixate the tubing to the abdominal wall to prevent further tubing migration . this approach also allowed us to simultaneously repair the fascial defect associated with the shunt entry site into the peritoneal cavity , allowing us to reap the benefits of decreased recurrence and decreased patient morbidity associated with this technique . the technique described was well tolerated without complication and resulted in improvement of clinical symptoms .

a 31-year - old female with a history of pseudotumor cerebri presented with headache and abdominal discomfort after placement of a ventriculoperitoneal ( vp ) shunt . the vp shunt was placed after prior failure and revision of a lumbar peritoneal shunt . computed tomography demonstrated shunt migration into the subcutaneous tissue . laparoscopy was used to reposition the vp shunt , directing the shunt toward the pelvis . the patient presented for further evaluation one month later , at which point the shunt was shown to have migrated into the subcutaneous tissue once again . laparoscopy was again used to reposition the shunt and affix it to the abdominal wall by using polytetrafluoroethylene ( ptfe ) mesh .

angiomyolipoma ( aml ) is a benign mesenchymal tumour consisting of varying amounts of mature adipose tissue , smooth muscle , and thick walled blood vessels . it is relatively rare benign tumour appearing in about 0.3% of general population and accounts for 3% of solid renal masses . extra renal amls ( eramls ) are rare tumours that present as incidentalomas upon imaging for other conditions . case reports of extra renal aml are rare with < 60 reported cases since they were first described by friis and hjortrup in 1982 . our case described one of the largest ever reported myolipomas , which despite its size , can be rather asymptomatic . magnetic resonance imaging ( mri ) revealed 9.5 cm 8 cm 2 cm right adrenal mass [ figure 1 ] . the tumour was predominantly hyperintense with signal intensity similar to fat on t1/t2-weighted images and lost signal on the fat - suppressed sequences . a well - encapsulated , firm globular mass separate from the right kidney and no definable adrenal was found [ figures 2 and 3 ] . gross post - operative evaluation of the adrenal mass showed approximately 10 cm 8 cm 2 cm greyish white solid , smooth , and firm mass [ figure 4 ] . histological examination revealed benign adrenal cortical elements , with a predominance of mature benign adipose tissue interspersed with pockets of haemopoietic ( erythro - myeloid ) intermediates , and megakaryocytes which was confirmatory for the diagnosis of adrenal aml . angiomyolipoma is a rare clinical entity mostly involving kidney and is part of the group of tumour known as tumours of perivascular epithelioid cell origin . the most widely accepted theory of origin is adrenocortical cell metaplasia in response to stimuli , such as necrosis , inflammation , infection or stress ( meyer et al . ) . the presence of perivascular epithelioid cells is characteristic of aml since these cells show immunoreactivity for muscle markers ( epithelial membrane antigen , keratin , vimentin , desmin , and actin ) and hmb-45 . positive immunoreactivity for hmb-45 , a monoclonal antibody , is characteristic of amls and can be used to differentiate amls from other similar appearing lesions . one is isolated aml , and the other is that is associated with tuberous sclerosis . most patients of isolated cases are in the age group of 27 - 72 years , mean age being 43 years . although the majority of eramls are benign , two cases of metastatic and recurrent eramls have been reported . the demonstration of fat density ( hypodense ) within an adrenal mass by computed tomography is virtually diagnostic of aml . mri is sometimes required to demonstrate origin of the tumour , to define the tissue planes when the tumour is large and heterogeneous , and to distinguish benign from malignant lesions by comparing signal intensity ratios of adrenal to liver . a small , < 5 cm , asymptomatic myelolipoma could be followed - up over 1 - 2 year period with imaging controls . on the contrary , asymptomatic lesion or a large > 5 cm myelolipoma should be surgically excised , since there are reports of spontaneous rupture and haemorrhage of the mass presented with life - threatening cardiovascular shock . unstable patients may benefit from emergent tumour embolisation and a subsequently staged surgical resection . since it is a benign disease , its prognosis is good . nevertheless , follow - up is recommended because of atypical morphology . currently , there is no agreed protocol on follow - up but an ultrasound three to 6 months following the surgery with annual clinical examination for large tumours is recommended . the adrenal myolipoma is a rare urological entity , which seems to increase its frequency , probably due to causes affecting primarily the function and physiology of the adrenals . ever since its introduction by gagner in 1992 , laparoscopic adrenalectomy has become the standard of care for the treatment of functioning and non - functioning adrenal tumours . many authors have found a decrease in perioperative morbidity and convalescence after this procedure when compared with open surgery .

angiomyolipoma ( aml ) is a rare mesenchymal tumour arising from perivascular epithelioid cells . it is most commonly seen in kidney , but rarely aml can arise in extra renal sites . adrenal aml is a very rare clinical entity , and very few cases have been reported so far . we present our experience with a 43-year - old female , who presented with right flank pain . magnetic resonance imaging showed a right adrenal mass . laparoscopic adrenelectomy was performed , and the histopathology report confirmed the diagnosis of aml . patient was discharged uneventfully .

fitz hugh curtis syndrome is an extra - pelvic manifestation of pelvic inflammatory disease ( pid ) and is characterized by perihepatic adhesions between liver capsule and diaphragm or anterior peritoneal surface(13 ) . most fitz hugh curtis syndrome patients are women of child bearing age and rarely has the syndrome been reported in males . the predominant symptom is pain in the right upper quadrant , which may be confused with biliary disease an abdominal computed tomography ( ct ) scan may reveal subcapsular enhancement of the liver in arterial phase ( 4 ) . we herein report a case of fitz hugh curtis syndrome in a male patient that was diagnosed via laparoscopy . a 29 year - old african american male with russel - silver dwarfism presented with one day history of diarrhea , nausea , vomiting , right side abdominal pain , and abdominal distention . the pain was constant , gradually increasing in severity , and not related to food intake . his medical history was significant for russel - silver dwarfism , calcium deficiency , cardiomegaly , and bilateral testicular implants for undescended testicles . the patient was sexually active only with his girlfriend and denied any history of sexually transmitted disease . on examination patient was afebrile and his vitals were stable . laboratory workup revealed white blood cell ( wbc ) count of 14,000/l with normal liver function tests . ct scan of the abdomen and pelvis showed a small amount of free fluid in pelvis ; the proximal appendix appeared normal , however the distal appendix was not visualized . the liver capsule appeared normal and there was no subcapsular fluid collection ( figure 1 ) . ct scan showing normal appearing liver capsule and no perihepatic fluid collection the patient was admitted to the surgical service . he was made nil per os ( npo ) , and placed on intravenous fluids and pain medication . he was refusing any surgical intervention at this point . on hospital day 5 , a repeat abdominal ct scan demonstrated a normal appearing liver , small bowel , large bowel , and appendix , with a mild increase in pelvic free fluid . as the patient 's symptoms did not improve with conservative management , he ultimately agreed to undergo a diagnostic laparoscopy , and was taken to the operating room on hospital day 9 . the small bowel was run in a retrograde fashion starting at the caecum , and no stricture , mass , or perforation was noticed . appendectomy was not performed as per patient'swishes . following the procedure , the patient reported complete resolution of his symptoms . his diet was gradually advanced , which he tolerated well , and was discharged on post operative day 2 . fitz -hugh curtis syndrome was first described in 1920 by carlos stajano . in the 1930 's thomas fitz hugh and arthur curtis also described the syndrome and made a connection between right upper quadrant pain following a pelvic infection and violin string like perihepatic adhesions(5 ) . the first case of gonococcal perihepatitis in a male the incidence ranges from 4% to 14% in women with pid , but is as high as 27% in adolescents with pid , whose less mature genitourinary tract anatomy makes them more susceptible to infection(1 ) . the inflammation of the liver capsule has been attributed to the direct bacterial spread from an infected fallopian tube via the right paracolic gutter . in men , hematogenous and lymphatic spread to liver the predominant symptoms are right upper quadrant pain , tenderness , and pleuritic right sided chest pain(2 ) . these symptoms can pose diagnostic challenges as they may be confused with biliary tract symptoms . in a clinical setting , the diagnosis is adequately established by excluding other possible causes of right upper quadrant pain . on laboratory examination , white blood cell count can be elevated in nearly half of the patients , while liver function tests are normal in most patients . because urethral cultures frequently fail to demonstrate the presence of gonorrhea and chlamydia , the serologic microimmunofluorescence antibody test is helpful in diagnosis ( 2 ) . ct scan may show subcapsular fluid collection , thickening of hepatic capsule in the arterial phase , and wedging enhancement of the involved liver parenchyma in more than 50% of patients . most cases of fitz hugh curtis syndrome are managed with antibiotics against gonorrhea and chlamydia . it occurs mostly in premenopausal women , however , cases in males have also been reported .

fitz hugh curtis syndrome is a condition characterized by inflammation of the liver capsule with concomitant pelvic inflammation without involvement of liver parenchyma . it is classically seen in young women who present with sharp , pleuritic right upper quadrant pain , usually but not always accompanied by symptoms of pelvic inflammatory disease ( pid ) and is frequently confused with biliary tract disease . rarely the syndrome has been reported in males , hematogenous and lymphatic spread to liver is thought to be the underlying mechanism . serological tests and computed tomography ( ct ) scan may aid in diagnosis of fitz hugh curtis syndrome . definitive diagnosis is made by laparoscopy , which provides both diagnostic and therapeutic benefits . we report a case of fitz hugh curtis syndrome in a young male patient , which was diagnosed and treated by laparoscopy . we also include a review of the literature .

leydig cell tumours are rare stromal tumours of the testis accounting for 13% of testicular neoplasms . they exhibit a peak incidence in the preadolescent as well as in the older ( > 50 years ) age groups [ 1 , 2 , 3 , 4 ] . the diagnosis of a malignant leydig cell tumour is not always easy since no definite histological criteria for malignancy exist . a 35-year - old man presented to our hospital with a 3-month history of a painless left testicular mass . blood concentrations of chorionic gonadotrophin , fetoprotein and human placental lactogen were within the reference range . a left radical orchidectomy was performed and the specimen submitted for histopathological examination . on microscopic examination , a malignant leydig cell tumour was found . the cells displayed acidophilic cytoplasm , intranuclear inclusions and increased mitotic activity ( > 3/10 hpf ) . many cells with large atypical pleomorphic nuclei could also be observed ( fig . 1 , fig . 2 ) . there was no angiolymphatic invasion , foci of necrosis or extension beyond the capsule of the testis . the immunohistochemical study showed that the tumour cells were positive for vimentin , melan a and inhibin , and negative for ckae1 , ckae3 , s100p , p63 , cea , afp and actin . 3 ) . retroperitoneal lymph node dissection was suggested , but the patient declined further surgery . leydig cell tumours are rare and only about 3% of them are bilateral . about 1520% of the patients already present with metastatic disease , particularly in the lymph nodes , lung and liver . these tumours may be hormonally active , and gynaecomastia is seen in 30% of the cases [ 2 , 3 , 6 ] . leydig cell tumours in an undescended testis may exhibit only manifestation of endocrinological disorders such as gynaecomastia , impotence and loss of libido . however , among the 480 cases of leydig cell tumours reported in the literature , only 20 cases were associated with cryptorchidism , and there is no evidence that undescended testes are more prone to develop leydig cell tumours [ 2 , 5 , 7 ] . features associated with malignancy include a large tumour size ( 5.7 cm ) , nuclear atypia , a mitotic count of > 3/10 hpf , foci of necrosis , angiolymphatic invasion , infiltrative margins , dna aneuploidy and an increased expression of ki67/mib-1 and p53 [ 2 , 3 , 8 , 9 , 10 ] . our patient was young and did not fit into either of the known age incidence peaks . microscopic features such as the severe nuclear atypia and increased mitotic activity ( > 3/10 hpf ) favoured the diagnosis of malignancy . however , there was no angiolymphatic invasion or extension beyond the capsule of the testis . also , the small size of the tumour was remarkable .

leydig cell tumour is a benign testicular non - germ cell tumour , and malignant transformation is rare . we report a case of a 35-year - old man who came to our hospital with a painless left testicular mass measuring 1.2 1 cm . histological evaluation of the tumour showed features of a malignant leydig cell tumour but no infiltration beyond the capsule or metastasis . the small size of the tumour was remarkable .

during 20092010 , serum samples from patients with suspected dengue were received by the fdoh for dengue diagnostic testing ; the samples came from 16 of florida s 67counties . specific , real - time rt - pcr ( 8) and igm anti - denv elisa ( 9 ) . samples with highly positive rt - pcr results were spread onto cultured ae . albopictus c6/36 cells , and the presence of virus and genome were confirmed by immunofluorescence ( 10 ) and rt - pcr , respectively ( 11 ) . isolates were further propagated and viral rna was extracted from culture supernatants by using the universal biorobot system ( qiagen , valencia , ca , usa ) . the envelope glycoprotein ( e ) gene was amplified ( technical appendix ) , and the e gene open - reading frame ( 1,485 bp ) was sequenced . multiple sequence alignments were performed by using the muscle module available in mega 5 ( www.megasoftware.net ) . evolutionary history was inferred by using maximum likelihood and phylogenetic trees constructed by using neighbor - joining methods . evolutionary distances were computed , and several e gene sequences from genbank were included in the phylogenetic tree to support tree topology by genotype ( technical appendix ) . in 2009 , five denv-1positive cases were identified by rt - pcr in key west . subsequently , in 2010 , the fdoh tested 195 serum samples by real - time rt - pcr . fifty - six ( 29% ) samples were positive for denv rna : denv-1 ( 37 [ 66% ] samples ) , denv-2 ( 13 [ 23% ] samples ) , denv-3 ( 3 [ 5% ] samples ) , and denv-4 ( 3 [ 5% ] samples ) . monroe county submitted 73 serum samples , of which 31 ( 42% ) had results positive for recent dengue infection : denv-1 was detected in 22 by rt - pcr , and 9 had positive igm anti - denv elisa results . none of the denv-1 patients from monroe county had a history of recent travel to a dengue - endemic region before the onset of symptoms . fifteen other florida counties submitted serum samples : 13 counties submitted < 10 specimens , 1 submitted 2030 specimens , and 1 submitted > 60specimens . denv-1 was found in 15 serum samples from 6 of these counties ; however , all the patients had a history of recent international travel . we sequenced the e gene of 12 denv-1 strains isolated in florida during 20092010 to determine their genetic relatedness ; of the 12 strains , 8 were from key west and 1 each was from dade , broward , orange , and pinellas counties . in addition , 23 denv-1 e sequences published in genbank , including the 2010 key west isolates obtained from a blood donor and a mosquito pool ( 6,7 ) , were used to construct a maximum - likelihood phylogenetic tree . this phylogenetic analysis showed that all the florida denv-1 isolates belong to the american african genotype ( genotype v ) ( 12,13 ) together with other viruses isolated throughout the americas ( figure 1 ) . maximum - likelihood phylogenetic tree of dengue virus type 1 , including isolates from key west , florida , usa , and representative isolates from 5 genotypes with global geographic distribution . solid circles , 8 key west viruses ( monroe county ) isolated during 20092010 ; solid diamonds , isolates from other florida counties ( dade , pinellas , orange , and broward counties ) . each taxon represents a single virus isolate and is labeled with the geographic origin and collection year . thirty - six envelope glycoprotein gene sequences obtained from genbank were included to support tree topology and identify genotypes . all genotypes except the american african genotype ( v ) have been collapsed . key west denv-1 viruses grouped among central american viruses , which configure a distinct lineage separate from the caribbean viruses . this divergence between the central american and caribbean lineages is well supported by high bootstrap values . moreover , the key west and monroe county viruses grouped together and indicated a distinct sublineage supported by a high bootstrap value ( 99% ) , separating them from viruses isolated in dade , orange , pinellas , and broward counties that were more closely related to other central american viruses ( figure 1 ) . one 2009 isolate ( iq425062 ) from a key west patient is related to this group , suggesting a separate introduction of denv-1 in key west . the sequence similarity between the 2009 and 2010 key west strains was < 0.9% ; however , the evolutionary distance and taxa positions between the 2009 and 2010 strains presented in the phylogenetic tree suggests that the 2010 strains diverged from the 2009 strains . the observed differences between e gene sequences for the key west strains ( 20092010 ) and the rest of the strains in this phylogeny were < 2.1% with the other florida strains , < 1.2% with central american strains , and < 4.8% with the rest of the american african genotype . evolutionary distances and the topology of the central american lineage suggest this lineage is the genetic origin of the florida denv-1 strain . most viruses isolated in monroe county diverged from the central american lineage into a distinct sublineage the key west denv-1 strain associated with the 20092010 outbreak . the high level of genetic similarity among the viruses isolated in monroe county , their close evolutionary distances , and the lack of recent international travel for the case - patients suggest endemic transmission and microevolution of this denv . conversely , the scattered and separate phylogenetic positioning of virus strains from patients with travel - associated cases from other florida counties indicates a different origin from the majority of key west isolates . although the 2009 broward isolate ( jq425067 ) is positioned near the central american lineage , the low bootstrap value ( 53% ) does not support lineage ancestry . the epidemiologic and phylogenetic evidence suggests that the 2010 cases appeared to be a continuation of the 2009 outbreak . unlike cases along the texas mexico border ( 14 ) , in addition , denv-1 was detected in a blood donation from the monroe county in 2010 , further supporting local transmission of denv ( 7 ) . collectively , these findings indicate that endemic denv-1 was transmitted in key west over a period of > 2 years . table showing the dengue virus type 1 ( denv-1 ) envelope gene reverse transcription pcr and another showing denv-1 strains used in a study of the genetic relatedness of dengue viruses in key west , florida , usa , 20092010 .

sequencing of dengue virus type 1 ( denv-1 ) strains isolated in key west / monroe county , florida , indicate endemic transmission for > 2 years of a distinct and predominant sublineage of the american african genotype . denv-1 strains isolated elsewhere in florida grouped within a separate central american lineage . findings indicate endemic transmission of denv into the continental united states .

a 56-year - old man suffering from diabetes and hypertension was admitted to the neurology department in a hospital elsewhere with signs of epileptic seizure . after a brief period , a diagnostic laparotomy with gastrotomy was performed , at which over 3 liters of blood were found , but no active bleeding . the site of origin of hemorrhage was suspected at the gastroesophageal junction , although no peptic ulcer could be discovered . gastroscopy was performed 1 day later , demonstrating a large , ulcerating tumor at the cardia , of which biopsies were taken . because the patient was now diagnosed with a bleeding tumor at the gastroesophageal junction , he was transferred to the icu of the canisius - wilhelmina hospital in nijmegen ( in formation with the radboud university hospital comprising the esophagus centre eastern netherlands , scon ) . a computed tomography ( ct ) scan was performed , showing a large tumor at the gastroesophageal junction , extending towards the pancreatic tail ( fig . a second gastroscopy with new biopsies , 8 days later , proved the same results . at the time of the third gastroscopy , a second ct scan revealed a remarkable decline of the entire process , emanating from the pancreatic tail as a pseudocyst ( fig . our patient experienced hypovolemic shock following massive gastrointestinal hemorrhage caused by a burst - through of a pancreatic pseudocyst to the stomach . the incidence of upper gastrointestinal hemorrhage in the netherlands is 45 - 70 per 100,000 per year , most of which are caused by peptic ulcers of the stomach and duodenum . a pseudocyst of the pancreas consists of one or several collections of pancreatic fluid , surrounded by inflamed pancreatic tissues . the wall is formed by the enclosing structures , i.e. stomach , transverse colon or omentum . the pseudocyst is usually connected to the pancreatic duct . the distinctive feature between a genuine cyst and a pseudocyst is the lack of an epithelial layer . pseudocysts of the pancreas arise as a result of episodes of acute pancreatitis or blunt force abdominal trauma . the cause is often disruption or obstruction of the pancreatic duct , with subsequent accumulation of pancreatic fluids , containing high concentrations of pancreatic enzymes . in acute pancreatitis , ( pseudo)cyst and necrosis can be difficult to distinguish by contrast - enhanced ct scan , whereas magnetic resonance imaging ( mri ) is distinctive . pseudocysts can damage adjacent arteries by means of mechanical pressure and digestion by pancreatic enzymes , hereby causing the formation of pseudoaneurysm with possible subsequent hemorrhage into the pancreatic duct . usually , this involves the splenic artery , but also the gastroepiploic arteries and gastroduodenal branches can be affected . the old motto is to drain pseudocysts larger than 6 cm or existing longer than 6 weeks , although literature on the subject scarcely supports this . the possibilities for drainage are to marsupialize the pseudocyst through laparotomy or laparoscopy to the stomach or jejunum , or to perform resection of the pancreatic tail pseudoaneurysms or hemorrhages are eligible for endovascular embolization , and transgastric drainage of cysts is contraindicated during hemorrhages , unless embolization is performed first . case series and case reports in the available literature on hemorrhaging pseudocysts all advocate endovascular embolization , followed by resection of the pancreatic tail [ 5 , 8 , 9 , 10 , 11 , 12 , 13 ] . our patient was diagnosed with hemorrhaging pseudocyst 16 days after admission and initial laparotomy . because of the improving clinical condition and decline of the pseudocyst on ct scan , it was decided to pursue a the initial plan was to perform pancreatic tail resection after clinical recovery ; however , ultrasonography and ct scan revealed a rapid decline and full disappearance of the pseudocyst . our patient made a full recovery and has adjusted his life style . in all case reports , operative intervention is advocated , precipitated by endovascular embolization in case of serious hemodynamic instability . one case series of 16 patients reports a 62% mortality rate , in which endovascular embolization is combined with pancreatic tail resection . there is evidence to support the role of angiography in case of acute upper gastrointestinal hemorrhage , simultaneously offering the possibility for endovascular treatment [ 2 , 5 , 8 , 9 , 10 , 11 , 12 , 13 ] . embolizing the splenic artery does not inevitably necessitate splenectomy , for the remaining blood supply to the spleen is routed through the vasa brevia , which should be sufficient . furthermore , the course of our patient proves that surgical intervention such as pancreatic tail resection is not always an absolute necessity . however , surgery is nowadays not the only option for treatment , as possibilities in radiological endovascular diagnostics and techniques are increasing and will eventually be the first step in treating acute upper gastrointestinal hemorrhage .

acute upper gastrointestinal tract hemorrhage represents a frequent morbidity which can be localized and treated endoscopically . when endoscopic treatment alone is failing , radiological or surgical treatment may be warranted . a case history will be presented regarding a rare cause of intestinal hemorrhage with an extraordinary course of illness .

esophageal cancer is a devastating disease with rapidly increasing incidence in western . despite important advances in therapy , > 50% of patients have incurable disease at diagnosis and only 510% of these patients have life expectancy > 5 years . dysphagia is the most common symptom that afflicts these patients , causing 70% of complaints , and leading to severe malnutrition and reduced quality of life [ 1 , 2 ] . this factor prevents curative treatment in the majority of them , thus making palliative care a more realistic option [ 1 , 3 ] . although surgical resection remains the treatment of choice for early cancers , palliative esophagectomy is not recommended due to high mortality rates . however , surgical procedures in order to bypass the dietary route with relief of dysphagia have been reported in the literature but have fallen into disuse because of the morbidity and mortality of the procedure itself and advances in endoscopic treatment . this paper demonstrates the use of surgical treatment using the laparoscopic approach in the palliative care of a patient with advanced esophageal cancer . a 69-year - old male presented with progressive dysphagia , retrosternal pain and weight loss of 10 kg in 2 months . the finding was a circumferential lesion from 19 to 25 cm of the incisors , with biopsy diagnosing squamous cell carcinoma . radiological examinations showed lymphadenopathy in the celiac trunk and in the mediastinal area . a bronchoscopy diagnosed tracheal infiltration by neoplasia . the patient was treated with external radiotherapy ( 13 250 cgy ) with symptom relief and regained performance status ( from 3 to 1 ) . however , after re - staging exams , the persistence of the disease was diagnosed , with dysphagia worsening . the technique , as described by lacerda et al . in a previous report , involves the creation of a gastric tube close to 3 cm in width and with an average length of 30 cm ( fig . 1 ) and preservation of the right gastroepiploic vessels by laparoscopy . figure 1:gastric tube . the tube is then passed along a retrosternal path and end - to - side anastomosis using a circular stapler is performed with the cervical esophagus prepared earlier by left cervicotomy . a laminar drain is applied in the cervical incision ( fig . 2 ) . the patient was discharged from the hospital on the fifth post - operative day with oral intake . no leakage or other complication the patient survived for 4 months , gained 10 kg in this period and did not refer any dysphagia after the procedure . currently , there are several methods for this purpose , including the esophageal stent , endoscopic dilation , radiotherapy ( both external and endoluminal brachytherapy ) , chemotherapy and laser recanalization ( yag ) . the choice of procedure and the response to treatment should be properly related to life expectancy , which has also been related to performance status , weight loss , tumor extension , clinical stage , sex and age . dysphagia from inoperable esophageal cancer is a common and complex management problem , and there is no consensus on the ideal treatment approach . all methods have their own advantages and disadvantages in rapid or late onset effect in symptom relief as in recurrence . no single intervention palliates dysphagia at all time for every patient ; therefore , healthcare providers must assess the risks and benefits of each palliative intervention for individual patients . surgical resection is still cited in the literature as a way to palliate esophageal cancer but is restricted to patients with severe risk of complications ( e.g. perforation or incontrollable tumor bleeding ) due to high morbidity and mortality in relation to non - surgical palliative options . that there are no randomized controlled studies to compare the addition of surgery to palliative chemotherapy with chemotherapy alone . they also state that such studies may become possible and worthwhile if minimal access surgery can be achieved that has reduced complications and better recovery of health - related quality of life than standard open surgery . in 1979 , postlethwait proposed a technique for esophageal bypass surgery with an isoperistaltic gastric tube , and this method is still used despite its morbidity . this report demonstrates that an update in the technique proposed by postlethwait for palliation of esophageal cancer with the use of minimal access surgery is feasible . according to the current literature , the bypass procedure in order to palliate dysphagia could be reserved for patients in which the stent has failed or contraindicated . high costs can sometimes make the lesser invasive procedure more prohibitive to the patient than surgery itself .

esophageal cancer is a devastating disease with rapidly increasing incidence in western countries . dysphagia is the most common complication , causing severe malnutrition and reduced quality of life . a 69-year - old male with persistent esophageal cancer after radiation therapy was subjected to palliative by - pass surgery using a laparoscopic approach . due to the advanced stage at diagnosis , palliative treatment was a more realistic option . dysphagia is a most distressing symptom of this disease , causing malnutrition and reducing quality of life . the goal of palliation is to improve swallowing . the most common methods applied are endoscopic stenting , radiation therapy ( external or brachytherapy ) , chemotherapy , yttrium - aluminum - garnet laser rechanneling or endoscopic dilatation . palliative surgery is rarely proposed due to morbidity and complications . this paper demonstrates an update in the technique proposed by postlethwait in 1979 for palliation of esophageal cancer .

a 35-year - old female presented with a painless , slowly growing mass in the right inferior orbit for the past four years . she did not complain of diminution of visual acuity , diplopia or field defect . on examination , visual acuity in both eyes the right side demonstrated a solid , non - tender , freely mobile mass in the inferior orbit , which became more prominent on applying pressure on the upper part of the globe . the mass measured approximately 20 mm 15 mm and the size did not vary with valsalva maneuver , ocular movements or posture . slit - lamp and fundus examination of the eye showed normal anterior and posterior segments . b - scan ultrasonography revealed an extraconal , homogenous lesion in the inferior part of the right orbit . computed tomography revealed a non - enhancing well- defined homogenous mass measuring 20 mm 12 mm in the right inferior orbit which was displacing the inferior rectus and inferior oblique muscle upwards . the mass was separate from the above - mentioned muscles and produced no globe indentation or displacement [ figure 1a ] . a provisional diagnosis of orbital dermoid was made and the patient was taken up for an excision biopsy . peroperative findings were of a smooth , well - encapsulated mass with no attachments to the surrounding muscles . on histopathological examination , the gross specimen consisted of a smooth well - encapsulated solid mass measuring 25 mm in the greatest diameter [ figure 1b ] . microscopically , a biphasic pattern of tumor cells was seen with areas of closely packed spindle cells having fusiform nuclei and eosinophilic cytoplasm ( antoni a ) admixed with looser myxoid tissue having ovoid cells ( antoni b ) . at places , these tumors cells were arranged in palisades and this arrangement of cells is referred to as verocay bodies . orbital schwannoma is a tumor of adulthood usually presenting between 20 to 70 years of age . schwannomas are usually asymptomatic when small and may produce progressive , painless proptosis on enlargement . a variable combination of signs and symptoms may be present due to the variable origin and location of the tumor in the orbit.2 displacement of the globe is related to the site of the tumor mass . most schwannomas arise from branches of either the supraorbital or supratrochlear nerves and hence produce downward displacement of the globe . less commonly , the tumor may arise from the infraorbital nerve and produce upward displacement of globe . larger tumors may produce diplopia , particularly when they arise from the orbital portion of the third , fourth or sixth nerve . the growth of the tumor may cause compression of the optic nerve with papilledema or optic atrophy . surgical excision is the treatment of choice and the tumor should be removed intact at the earliest to prevent compression of the optic nerve.3 incomplete excision can lead to recurrence or even intracranial extension . highly cellular tumors have greater chance of recurrence and malignant transformation . therefore an early treatment is indicated to avoid the complications related to progressive growth of the tumor . in our patient , we were successful in achieving the above objectives with timely and complete excision of the tumor . orbital schwannoma arising from the infraorbital nerve is rare and clinical diagnosis is often difficult . extensive literature search revealed only a few case reports of an orbital schwannoma arising from the infraorbital nerve.4 - 10 our patient presented with the tumor in this uncommon location . a solitary schwannoma , though rare , should be considered as a preoperative differential diagnosis of a unilateral slow- growing orbital mass in an adult and prompt management is warranted to prevent development of vision - threatening complications .

a rare case of unilateral orbital schwannoma arising from the infraorbital nerve is presented . an excision biopsy with complete removal of the mass in the inferior orbit was performed . a definitive diagnosis was made on histopathological examination . the clinical and histological features of schwannoma are discussed . a need for early removal of such tumors is recommended to prevent complications .

risperidone has been reported to be effective in management of disruptive behaviors , including hyperactivity , irritability , aggression , and temper tantrums . increased appetite , weight gain , headache , and sedation literature search revealed only four published reports of risperidone - related bleeding , including hemorrhagic cystitis,1 ) nasal bleeding,2,3 ) and gastrointestinal bleeding.4 ) we hereby describe the first pediatric case of gingival bleeding during risperidone treatment . an 11-year - old - girl was referred to our out - patient clinic for her hyperactivity , temper tantrums , sleep problems , and self - injurious behaviors . according to her psychiatric assessment and psychometric evaluation , she was started on risperidone 0.25 mg / day treatment for her disruptive behaviors and two weeks later the dose was increased to 0.5 mg / day . one - week after the dose increase , she experienced gingival bleeding when she brushed her teeth . risperidone was considered to be the reason for bleeding , therefore we decided to reduce the dose to 0.25 mg / day . gingival bleeding ceased within a week . because worsening of her behavioral problems , her mother increased the dose to 0.33 mg / day . she experienced gingival bleeding rarely ( twice a month ) and mildly during this dose regime , therefore we stopped the medication . we decided to change risperidone to methylphenidate ; however , her parents refused to continue to the treatment because of their worries about the potential side effects . she had no history of any bleeding disorder or any other medical condition including current allergies . she was not taking any other medication other than risperidone at the time of the development of bleeding . there are several case reports about risperidone - related bleeding ; however , to our knowledge , there is no report of gingival bleeding associated with risperidone in the literature . we presented a case who experienced gingival bleeding when risperidone dose was increased to 0.5 mg / day , and subsided after decreasing the dose to 0.25 mg / day , suggesting a dose - dependent side - effect . however , the chronological relationship between the time of risperidone 0.5 mg / day administration and emergence of bleeding in the absence of an identifiable medical condition suggests risperidone to be the causative agent . there was no other agent likely to be the cause of bleeding , except risperidone , and bleeding ceased when the dose was decreased . although an etiological relationship between risperidone use and gingival bleeding can not be drawn from a single case , the naranjo probability scale score ( 8) reveals a probable relationship.5 ) the bleeding side effect of risperidone might be caused by several mechanisms , including thrombocytopenia and 5-hydroxytryptamine 2a ( 5-ht2a ) receptor antagonism.3 ) thrombocytopenia is a known adverse effect of atypical antipsychotics and has also been reported during risperidone.6 ) in our reported case platelet count was normal , therefore , gingival bleeding could not be a consequence of thrombocytopenia . antagonism of 5-ht2a receptor was suggested to cause bleeding by inhibiting the release of vasoconstrictors from platelets and reducing the platelet aggregation.4 ) therefore , we may speculate that risperidone s high affinity to 5-ht2a receptors might have probably resulted gingival bleeding . this case suggests that bleeding associated with risperidone may be a dose - dependent side effect . however , these potential explanations may not describe the entire mechanism of this adverse reaction . it is also possible that she might have some undetermined predisposing factors for bleeding that is triggered by risperidone . there are several reports on gingival bleeding during anti - depressants;7 ) however , this is the first case on gingival bleeding associated with risperidone . although bleeding is a rare side effect , clinicians should be aware that risperidone may cause bleeding . risperidone may also increase the risk of bleeding in susceptible patients with a history of coagulation disorders , and with concomitant use of other drugs , including non - steroidal anti - inflammatory drugs , aspirin , or other medications that affect coagulation .

there are several case reports on risperidone - related bleeding ; however , to our knowledge , there is no report about gingival bleeding associated with risperidone in the literature . we presented a case who experienced gingival bleeding when risperidone dose was increased to 0.5 mg / day , and subsided after decreasing the dose to 0.25 mg / day , suggesting a dose - dependent side - effect . the bleeding side effect of risperidone might be caused by several mechanisms , including 5-hydroxytryptamine 2a receptor antagonism . although bleeding associated with risperidone is rarely reported , clinicians should be aware of this side effect .

intestinal obstruction can be a result of mechanical or functional obstruction of the intestines , thereby preventing the normal transit of the products of digestion . intestinal pseudo - obstruction ( adynamic ) , characterized by abdominal pain , nausea , vomiting , constipation , and severe abdominal distension , is a clinical syndrome caused by severe impairment in the ability of the intestines to push the food through , in the absence of any lesion in the intestinal lumen . secondary pseudo - obstruction is more common than the primary and is commonly associated with neuroleptics , opiates , diabetes mellitus , and severe metabolic illnesses . intrathecal baclofen has been reported to cause intestinal pseudo - obstruction and life - threatening constipation . though baclofen might sometimes cause constipation , there are only very few reports on intestinal pseudo - obstruction complicating oral baclofen therapy . a 50-year - old male , post cervical discectomy ( c3 - 4 ) for prolapsed intervertebral disc with quadriparesis , neurogenic bladder , and spasticity , was on baclofen at a dose of 50 mg / day for 6 months . he presented to the emergency department with acute onset of bilious projectile vomiting , abdominal distension , and constipation since 3 days . one and half months back , he had a traumatic urethral injury for which open suprapubic cystostomy was performed . he had no history of other co - morbidities or drug intake other than baclofen . on examination , his vitals were stable but he was dehydrated . plain radiograph of the abdomen revealed dilated large and small bowel loops without any air fluid levels . the possibility of adhesive intestinal obstruction was considered and the patient was kept nil per oral with nasogastric aspiration . a diagnosis of pseudo - obstruction was considered secondary to long - term baclofen intake , as he had gradually improved over 1 week without any active intervention except for the discontinuation of baclofen since admission . the patient was able to tolerate oral diet after 7 days of admission with no recurrence of symptoms . currently , at 1 month of follow - up , he is asymptomatic with no recurrences . colonic pseudo - obstruction is characterized by distension of the colon with features of colonic obstruction , in the absence of mechanical obstruction . primary pseudo - obstruction is rare and is a motility disorder involving the autonomic innervation of the intestinal wall . secondary pseudo - obstruction is more common and it has been associated with conditions like severe metabolic illness , diabetes mellitus , myxedema , scleroderma , parkinson 's disease , hyperparathyroidism , and drugs like neuroleptics and opiates . baclofen , a derivative of gamma - aminobutyric acid ( gaba ) , is an agonist for the gaba b receptors , and is used to treat spasticity following spinal cord injury , multiple sclerosis , and cerebral palsy . tolerance to baclofen mainly develops after many years to intrathecal route , but this beneficial property can be potentially harmful necessitating baclofen withdrawal in patients developing adynamic intestinal obstruction . intestinal pseudo - obstruction has been reported after intrathecal baclofen , but till date , only a single case has been reported in literature describing adynamic intestinal obstruction caused by therapeutic dose of oral baclofen at 20 mg / day for 2 years in a 75-year - old male . animal studies have shown that gaba - ergic mechanisms are involved in synaptosomal nitric oxide synthesis resulting in relaxation of rat ileum . in human preparations obtained from patients with malignant tumors , baclofen suppressed both amplitude and frequency of spontaneous and pharmacologically induced contractions in the longitudinal muscle of jejunum , eventually causing a complete block at higher concentrations while not affecting spontaneous motility of circular or longitudinal colon muscles . gabaergic neurons , which are predominantly inhibitory interneurons , are distributed throughout the central nervous system and the enteric nervous system in humans . hence , baclofen being a competitive gaba b agonist , affects the gastrointestinal function by acting both peripherally in the intestinal tract by inhibiting the myenteric plexus and centrally on brainstem nuclei which coordinate afferent input from and efferent output to the intestine by crossing the blood brain barrier . in our case , we considered the possibilities of baclofen - induced pseudo - obstruction of the intestine and adhesive intestinal obstruction . hence , baclofen was discontinued and patient was put on nasogastric decompression . considering the remote possibility of adhesive obstruction , water - soluble contrast enema , colonoscopy , or neostigmine was not tried . colonoscopic decompression was planned in case of deterioration , but the patient recovered completely with prompt discontinuation of baclofen , nasogastric decompression , and maintenance of electrolytes . considering the temporal relationship of the occurrence of symptoms after 6 months of baclofen therapy , improvement after withdrawal of the drug , and presence of pharmacological explanation for the occurrence of obstruction and evidence of similar reports earlier , we considered the possibility of baclofen - induced pseudo - obstruction . on causality assessment , baclofen - induced pseudo - obstruction in our case belongs to the probable / likely category as per the world health organization - uppsala monitoring centre ( who - umc ) system and to the objective evidence in the form of blood levels of baclofen was not available , and re - challenging was not attempted because intestinal pseudo - obstruction could be fatal . the remote possibility of adhesive obstruction is mostly unlikely as the patient recovered with withdrawal of baclofen . oral baclofen used for spasticity can cause constipation , but intestinal pseudo - obstruction is a rare possibility and in such cases , prompt discontinuation of the drug can be therapeutic . this rare cause of intestinal pseudo - obstruction needs to be borne in mind to avoid potentially morbid investigations like gastrograffin enema or colonoscopy , which are associated with complications like perforation .

baclofen is a gamma- aminobutyric acid b ( gaba b ) agonist used for the management of spasticity associated with spinal cord injury . oral baclofen might cause constipation , but intestinal pseudo - obstruction is very rare . we report a 50-year - old male with spasticity following cervical discectomy ( c3 - 4 ) on oral baclofen for 6 months with intestinal pseudo - obstruction . he had undergone open suprapubic cystostomy for traumatic urethral injury , 45 days prior to the presentation and adhesive intestinal obstruction was also considered a possibility . however , there were no air fluid levels on abdominal radiographs and ultrasound abdomen was non - contributory . withdrawal of baclofen was therapeutic in this patient . this case is being reported to highlight the rare possibility of oral baclofen induced intestinal pseudo - obstruction .

a summary of methods is provided below and a detailed description of methods is included in the supplementary information . the toxin nanosponges were prepared by fusing rbc membrane vesicles on preformed poly(lactic - co - glycolic acid ) ( plga ) nanoparticles through an established extrusion process . the size of the nanosponges was obtained from three dynamic light scattering ( dls ) measurements using a malvern zen 3600 zetasizer . the morphology of the nanosponges after absorbing toxins was measured by transmission electron microscopy ( tem ) . for preparation of human rbc nanosponges , the rbcs were collected from whole human blood ( bioreclamation ) and the characterization results were shown in fig . reconstitution of the lyophilized samples was performed by solubilizing the samples in water and the characterization results were included in fig . the in vitro toxin neutralization ability of the nanosponges was examined by mixing 3 g of -toxin with 200 l of 1 mg / ml nanosponges for 30 min , followed by adding into 1.8 ml of 5% purified mouse rbcs . the in vitro toxin absorption capacity of the nanosponges was determined through titrating -toxin to a fixed amount of nanosponges . the interaction of the nanosponges with cells was examined by a scanning fluorescence microscopy by incubating fluorescent nanosponges and rbc membrane vesicles with human umbilical vein endothelial cells ( huvec ) . the in vitro cellular cytotoxicity of nanosponge - sequestered toxins was examined by incubating nanosponges of different concentrations with varied amounts of -toxin , streptolysin - o , and melittin for 30 min , followed by adding to huvecs for 24 hr . the in vivo toxin neutralization ability of the nanosponges was tested through subcutaneous injection of the nanosponge / toxin mixture to the flank region of nude mice , followed by histological analyses . on - site neutralization of -toxin by the nanosponges was conducted by subcutaneously injecting 50 l of 36 g / ml of -toxin solution , immediately followed by a 100 l injection of 2 mg / ml nanosponges . the mice were imaged 3 days later for visualization of skin lesion formation ( fig s11 ) . the in vivo detoxification efficacy was tested through intravenous injection of nanosponges before or after administration of a lethal dose of -toxin to icr mice , followed by monitoring the survival rate of the mice . for the in vivo hepatotoxicity study , one group of mice was sacrificed on day 3 following the injection of the toxin - bound nanosponges and another group was sacrificed on day 7 .

detoxification treatments such as toxin - targeted anti - virulence therapy1 , 2 offer ways to cleanse the body of virulence factors that are caused by bacterial infections , venomous injuries , and biological weaponry . because existing detoxification platforms such as antisera3 , monoclonal antibodies4 , small - molecule inhibitors5 , 6 , and molecularly imprinted polymers7 act by targeting the molecular structures of the toxins , customized treatments are required for different diseases . here we show a biomimetic toxin nanosponge that functions as a toxin decoy in vivo . the nanosponge , which consists of a polymeric nanoparticle core surrounded by red blood cell membranes , absorbs membrane - damaging toxins and diverts them away from their cellular targets . in a mouse model , the nanosponges markedly reduce the toxicity of staphylococcal alpha - hemolysin ( -toxin ) and thus improve the survival rate of toxin - challenged mice . this biologically inspired toxin nanosponge presents a detoxification treatment that can potentially treat a variety of injuries and diseases caused by pore - forming toxins .

assessment of effects of different assisted reproductive techniques ( art ) like in vitro fertilization ( ivf ) and intra cytoplasmic sperm injection ( icsi ) on speech and language development needs comprehensive studies . some studies showed that they are more susceptible to cerebral palsy which causes speech and language defects . development of speech and language consists of four periods : prelinguistic , linguistic , school and adolescence period . defects in prelinguistic behavior , i.e. physical defect like cleft palate , neurological defect like cerebral palsy or developmental defect like down syndrome produce speech defects . in two studies in belgium and finland in infants of art there was no difference between development of these children with infants of normal conception[5 , 6 ] . in another study no differences in mental , motor , social and expressive language development were found , and while receptive language development was in the normal range , ivf infants scored lower than control infants . one study did not report any excess of neurodevelopmental disorders in ivf / icsi children . the majority of studies followed the children during infancy , thereby precluding pertinent conclusion on the risk of neurodevelopmental disorders which express at older ages such as fine manipulation disability or dyslexia . one study shows that a large proportions of preterm deliveries in ivf children have increased risk of cerebral palsy . regarding the importance of this subject and the lack of a comprehensive study on the prelinguistic behavior of infants of art in iran , this study was designed . the aim of this study is to evaluate prelinguistic behavior of 9 month old art infants of royan institute . in this descriptive , cross sectional study , 151 term infants of art from royan institute have been evaluated in children 's health and development research center of tehran from august 2007 until august 2009 . the research ethics committee of the academic center of education , culture and research ( acecr ) and royan institute approved the study . the sampling method was non random sequential with the inclusion criteria of infants conceived through one of the art methods ( ivf / icsi ) , born in term and being resident in tehran . preterm born infants were excluded . after signing of research consents by parents , prelinguistic behavior of 151 infants who were born full term ( > 37 weeks ) are assessed . this scale is used for assessment of development of speech and language in children from birth to 3 years old . it has sensitivity of 90 - 95% for primary detection of speech , language and cognition defects . the method of art , sex of infants , age of mother at pregnancy , time and speech and language defect of parents at present and past time also were mentioned in questionnaire . the prelinguistic behavior of infants include : crying at birth , different cries , smiling , cooing , babbling , reduplicated babbling and echolalia , which are assessed in this study . one - hundred one term infants of art were evaluated . in this study , 76 ( 50.5% ) were male and 75 ( 49.5% ) female . there was 129 ( 85.4% ) of infants conceived by icsi and 22 ( 14.5% ) by ivf . prelinguistic behavior delay which had been assessed according to elm-2 is shown in table 1 . number of infants with delay in reduplicated babbling in icsi method was more than un ivf . prevalence of prelinguistic behavior delay in assisted reproductive techniques methods ivf : invitro fertilization ; icsi : intra cytoplasmic sperm injection the prelinguistic behavior delays in different sex and mothers age is shown in table 2 . there was a significant difference only in echolalia delay in the two sexes . prevalence of prelinguistic behavior delay in different sexes and mothers age mothers of 118 infants were younger than 35 years and 33 ones were older . two studies showed that art infants had normal cognition development [ 11 , 12 ] . zhu jl in showed that infertility treatment , especially icsi , may be associated with a slightly delayed cognitive language development . another study showed that there was no difference between cognitive development of infants of art and normal infants . in this study infants showed delay in reduplicated babbling and echolalia . in a study by sutcliffe children were assessed with the griffiths mental development scales . icsi children were around the midpoint for the griffiths scales and did not differ significantly for griffiths quotients and suhquotients . one study reported significantly lower mental scores in 1 year old infants born after icsi than in age matched infants born after ivf and naturally conceived infants . stratification for gender revealed that lower mental development index scores were only found in boys , not in girls . in our study delay of reduplicated babbling and echolalia were more in boys . there is no study about effect of mother 's age on speech and language development in infants . bonduelle and his collegues in their study , by selection of infants of mothers with the same age exclude affect of this variable . in our study delay of reduplicated there was no relation between speech and language defect of parents and prelinguistic behavior of infants . limited sample size , difficult access to this group of infants and unwillingness of parents to complete the questionnaires render less reliable results . this study was a new study in our country which shows differences between art infants in some prelinguistic behavior such as reduplicated babbling and echolalia . this study showed that prelinguistic behavior of art infants are affected by art method , infants sex and mother 's age at the time of pregnancy . detection and correction of any defect or delay in this period prevent major speech and language problems . more studies with larger sample size to compare art infants with infants of normal conception would be desirable .

objectivethe aim of this study is assessment of effects of different assisted reproductive techniques ( art ) like in vitro fertilization ( ivf ) and intra cytoplasmic sperm injection ( icsi ) on prelinguistic behavior of infants conceived by these techniques.methodsin this descriptive , cross sectional study , prelinguistic behavior of 151 full term art infants of royan institute have been assessed in children 's health and development research center of tehran from august 2007 until august 2009 . questionnaires were completed by parents at 9 months old . the questionnaire was standard according to early language milestone scale-2 ( elm-2 ) . data were analyzed by spss version 16 and using chi - square test.findingstwenty-two ( 14.5% ) of infants were conceived by ivf and 129 ( 85.4% ) by icsi . number of infants with delay in reduplicated babbling in icsi method was more than in ivf . there was only a significant difference in echolalia delay in the two sexes . echolalia was delayed more in boys . delay of reduplicated babbling was more in infants of younger mothers . there was no relation between speech and language defect of parents and infants.conclusionthis study showed that prelingustic behavior of art infants are affected by kind of art method , infant sex , and mother 's age at the time of pregnancy .

indications for covered self - expandable metallic airway stent removal include recurrent mucous plugging of the stent , excessive or recurrent granuloma formation , migration of the stent , stent infection , recurrence of stenosis , stent failure , stent fractures and accomplishment of treatment . usually , it is tedious to remove a stent because of their embedding in the bronchial mucosa and epithelization of the inner surface . granuloma formation at the extremities is a relative common occurrence in covered metallic stents possibly resulting from the radial force applied against the airway wall and the friction exerted . we report a case of covered self expandable metallic stent removal with a rigid bronchoscope under general anesthesia . the case is unique because it had almost all the common complications associated with the stent placement but it was successfully removed without much ado . to the best of our knowledge this is the first case of successful removal of a complete stent reported from india . a 21 year old female presented with complaints of increasing dyspnoea and cough with pain chest for last two weeks . patient had a past history of benign tracheal stenosis which was opened up by endobronchial electro surgery followed by placement of a self expandable metallic airway covered stent . x - ray chest done one and a half year back showed stent well in place [ figure 1 ] . x - ray chest done one and a half year back showing stent well in place bronchoscopy was done under local anesthesia and a stenosis was observed well above the level of the stent [ figure 2 ] with granulation tissue inside the stent [ figure 3 ] , lower end of the stent was fractured , carina was not visualized and stent was seen entering upper part of right main bronchus [ figure 4 ] . the beveled edge of the rigid scope was advanced between the stent wall and the airway mucosa thus creating a space between them . the circular extraction loop at the upper end of the metallic stent was grasped with a rigid optical alligator forceps and pulled in the lumen of rigid bronchoscope with continuous winding movements gradually separating the stent from the tracheal wall . immediately after that this is one of the rare cases of successful removal of self expandable metallic airway covered stent in benign tracheal stenosis . stenosis above the level of the stent granulation tissue inside the stent and at the margins fracture of stent at its lower end ( stent is seen at upper part of right main bronchus , carina is not visualized ) the stent after removal the management of patients with tracheal / bronchial strictures of benign etiology can be quite challenging . though surgical tracheal sleeve resection and reconstruction remains the gold standard for most benign airway strictures , non - surgical modalities such as laser photocoagulation and resection , balloon dilatation , electrocautery and stenting of the airway have been developed to deal with airway stenosis . various inoperable benign airway disorders are considered for airway stenting , like post intubation tracheal stenosis , tracheal burn or trauma , tracheo - broncho - malacia and extrinsic compression of trachea . though silicone prostheses are considered to be first choice in benign diseases their use is less suited , especially in airway wall malacia or distal and angular stenosis where sems are preferred . the disadvantages of the silicone stents are high migration rate , small lumen to wall thickness ratio and difficulty in clearing the secretions . on the other hand , the major drawback for placement of sems in benign situations is their difficult repositioning and removal in situations like excessive granulation tissue , stent fractures and stent migration . the complications of stent removal include significant oozing , tracheal mucosal dehiscence , tracheal puncture requiring thoracotomy , retained stent pieces , need for restenting , respiratory failure , tracheotomy , and even death . in our case , various authors have come up with different methods of removal of the stent , like the technique of nashef et al . , which was similar to that of rolling spaghetti on a fork , but much more difficult and at least equally messy . they further observed that there may be several fractures of the stent , which have to be removed piece by piece . in our case filler et al . , used a metal suction catheter to dissect the stent from the airway wall before extraction with the forceps . zakaluzny et al . , employed a rigid suspension laryngotracheoscope and used optical forceps to dissect the stent from the airway wall . alligator forceps were then employed to grasp and extract the stent through the rigid scope while the scope was advanced further into the airway . we first separated the stent from the tracheal wall and then removed all the works ( scope , forceps and the stent ) in one piece . to conclude , this case is unique for many reasons , firstly , it had almost all of the complications that can be associated with stent placement ( stenosis of trachea at the upper end of the stent , extensive granuloma formation , migration of the stent downwards and fracture of the stent at its lower end ) , secondly , we were able to remove the stent in toto [ figure 5 ] and lastly , there were no complications after removal of the stent . further studies are needed to re evaluate their removal successfully and safely and to produce better designed and technically superior stents like bio - absorbable stents which may not have to be removed at all .

covered self expandable metallic airway stents ( sems ) have been used for benign tracheal stenosis , post intubation tracheal stenosis , tracheal burn or trauma , tracheo - broncho - malacia , and extrinsic compression of trachea . their placement is considered to be permanent , with open surgery the only way to remove the stent , though there are few cases reports of their removal with the bronchoscope , but the complications after their removal are very high . in our patient , one and a half years after placement of sems , she developed cough with dyspnoea , video bronchoscopy showed stenosis above the level of stent with granulation tissue inside the stent , stent fracture in lower part and stent migration to right main bronchus , thus she had all conceivable complications of stent placement . the stent was removed with the help of rigid bronchoscope under general anaesthesia . she was discharged the following day . the case is being reported because it was unique in having all the possible complications of stent placement , and rare as we could take out the stent in toto . thirdly , the stent could be removed without any complication .

takayasu 's arteritis ( ta ) is a chronic idiopathic occlusive inflammation of aorta and its major branches predominantly affecting females in over 85% of cases . the major clinical finding is loss of palpable pulses in the upper limbs and neck . the unsuspected ischemia of vital regional vascular beds may render these patients at risk for inappropriate management . this case report describes the management of a trauma patient in whom the diagnosis of ta was established by specific investigations done for the same . the role of meticulous monitoring and importance of anticipation of such disease for prompt diagnosis and management is emphasized . a 26-year - old female was brought to the emergency room ( er ) with history of motor vehicle accident . on initial evaluation her physical examination revealed unrecordable upper limb peripheral pulses and blood pressure , although lower limb pulses were easily palpated . the radiological investigations revealed multiple left - sided rib fractures with bilateral pleural collections and small lung contusions . after infusion of two liters of lactated ringer 's solution , her blood pressure ( bp measured in right upper limb ) increased to 80/50 mmhg and heart rate ( hr ) was 140/min . she also had shaft and inter - trochanteric femur fractures on the right and left side , respectively . focused assessment sonography in trauma ( fast ) was negative and x - ray of the pelvis was normal . bilateral chest tubes were inserted and the patient was transferred to the intensive care unit ( icu ) with ongoing resuscitation with fluids , blood products and dopamine infusion ( 7 g / kg / min ) . fluid resuscitation was continued to maintain a central venous pressure ( cvp ) of around 10 mmhg . invasive arterial blood pressure monitoring was established via the right femoral artery and the bp recorded was 140/90 mmhg . dopamine infusion was then discontinued and thereafter the patient was subjected to arterial doppler and computed tomography ( ct ) angiography studies to rule out traumatic aortic rupture as a differential diagnosis . arterial doppler showed low - velocity monophasic flow in bilateral axillary , brachial and ulnar arteries . the erythrocyte sedimentation rate ( esr ) and c - reactive protein ( crp ) were normal . electrocardiography ( ecg ) , 2d echocardiography ( 2d echo ) and funduscopy were normal . thoracic epidural catheter was inserted for pain relief with continuous infusion of 0.125% bupivacaine and fentanyl ( 2 mcg / ml ) . epidural catheter was removed on 5 day of hospitalization and patient was moved to the ward in a stable condition with advice for regular follow - up and advised to check bp measurements in the lower limb if ever required . the occlusive thromboaortopathy ( ta ) is a group of diseases in which there is no or feeble pulses in upper half of body . the natural course of disease often gives rise to four main complications : takayasu 's hypertensive ischemic retinopathy , secondary hypertension , aortic regurgitation and aortic or arterial aneurysm . most patients with ta present during their child - bearing age , and a high index of suspicion is required to make an early diagnosis . patient may be entirely asymptomatic and the incidental finding of unequal pulse and blood pressure in upper and lower limb , bruit and hypertension may prompt further evaluation . stroke , congestive heart failure , and rarely ruptured aneurysm may be the heralding events . hemorrhagic shock is the commonest type of shock in trauma patients . any hypotensive trauma patient is presumed to be hypovolemic and resuscitative attempts are made with fluid infusion as was with our patient . there was no contributory past history of symptoms or signs suggestive of ta in our patient . a cvp of around 10 mmhg suggested a normovolemia status unlike the impression got by the low blood pressure ( as recorded in upper limbs ) . the presence of tachycardia in our patient could be attributed to pain and anxiety associated with trauma . there was no fall in hematocrit from the baseline ( perhaps due to hemo - concentration ) . dopamine infusion was started in our patient in an attempt to maintain perfusion and restore blood pressure but was discontinued when invasive monitoring revealed a normal bp . normal value of esr and c - reactive protein suggested that the disease was not active at that moment . our patient was perhaps asymptomatic for the disease and was incidentally diagnosed during workup after trauma . but ta as a differential diagnosis of exclusion may be considered for a pulseless trauma patient . normocytic normochromic anemia , hyper - gammaglobulin , and elevated esr may occur , but are not pathognomonic . medical treatment includes corticosteroids , anticoagulants and symptomatic therapy for which anti - hypertensive agents , digitalis and antibiotics are frequently used . follow - up in cases of ta includes monitoring for disease activity and its complications like hypertension , organ failure , seizures etc . long - term complications of corticosteroid use are also to be considered in the follow - up . anesthesiologists may encounter these patients in operation theater for anesthesia or in icus or emergency units after trauma or after one of its complications like a ruptured aneurysm . tas may be considered as a differential diagnosis in trauma patients , if examination reveals hypotension or unequal peripheral pulses in upper and lower limb in a young adult , provided hypovolemic shock is excluded . a thorough detailed physical examination with palpation of all the peripheral pulses as stressed in atls guidelines is reemphasized . following the basic atls guidelines would assist clinicians in picking up this discrepancy as part of their routine evaluation for all trauma patients . then , the clinician may suspect an atypical cause for hypotension if a blood pressure cuff on an upper extremity is reading a hypotensive measurement but the patient has strong , 2 + pulses in the lower extremities . these patients may be issued a special identification card containing the details of the disease and treatment course for future reference .

hemorrhagic shock is the most common reason to explain the inability to feel pulse in a trauma patient . however , clinicians should always suspect atypical causes for differential pulses in this population and takayasu 's arteritis ( ta ) is one such example . we report a case of aorto - arteritis in a patient who presented with trauma and was later diagnosed with ta . she had blood pressure discrepancy between upper and lower limbs noted upon her initial trauma evaluation .

brucellosis is a zoonotic disease mostly transmitted to humans through consumption of unpasteurized dairy products and can lead to a systemic disease with any organ involvement . in this report , we describe a case of brucellosis - induced avascular necrosis of the hip . brucellosis was diagnosed through serological tests , and avascular necrosis of the femoral head was confirmed by pelvic mri . the patient was treated with a combination of antimicrobial treatments and referred to the orthopedic service for total hip arthroplasty . brucellosis may present with unusual manifestations and should be always taken into consideration , particularly in endemic areas . brucellosis is a zoonotic disease mostly transmitted to humans through consumption of unpasteurized dairy products of infected animals ( 1 ) . it is a serious public health problem particularly in endemic areas , and is accompanied by . the disease is endemic in iran and has a high prevalence in lorestan province , central iran ( 3 ) . brucellosis is a systemic disease that may involve any organ in the body ( 4 ) . osteoarticular involvements including arthritis , spondylitis , osteomyelitis , tendonitis , and bursitis occur frequently and are reported in 3085% of patients with brucellosis ( 5 ) . the involvement of the large peripheral joints is usually manifested as monoarthritis ( 1 ) . although the hip joints involvement may happen in brucellosis , brucellosis - induced avascular necrosis of the hip , we describe a case of brucellosis that complicated by avascular necrosis of the femoral head . the patient was a 50-year - old rural farmer and rancher who presented with groin pain for six months before admission . he reported no history of trauma , underlying diseases , and steroids and alcohol use . over the previous six months , he had experienced symptoms including .fever , night sweats , weakness , fatigue , anorexia , and weight loss of 10 kg . the patient was visited by a local therapist ; in addition there was a delay in timely referral due to his addiction to opium . the groin pain was his most severe complaint on admission so that he could not bear his weight on the right leg . the physical examinations on admission showed stable vital signs , there was no organomegaly , and the right hip was in flexion and external rotation position so that any active and passive motion increased the pain . the results of laboratory study were as follows : complete blood count ( cbc ) : nl , liver function test ( lft ) : nl , rheamatic factor ( rf ) : neg , antinuclear antibody ( ana ) : neg , blood culture : neg , elevated erythrocyte sedimentation rate ( esr ) , c - reactive protein ( crp ) : positive , and standard agglutination test for brucellosis : positive ( table 1 ) . pelvic radiography and magnetic resonance imaging ( mri ) were administered showing avascular necrosis of right femoral head ( fig . 1 , 2 ) . the patient was subsequently treated with standard antibrucellosis regimen : streptomycine 1gr / d i m for three weeks , doxycycline 100mg / bid po , and rifampin 600mg / d po . .despite the improvement in the patient s general condition and laboratory evidence showing infection control , the patient was suffered from right groin pain and limitation of motion in the hip joint . although osteoarticular involvements , especially arthritis in the large peripheral joints , are among the most common manifestations of brucellosis , no joint destruction has been reported in many studies ( 5 ) . despite few reports on the permanent joint complications of brucellosis - induced peripheral joint arthritis , hip joints pyogenic infection has a poor prognosis , particularly if treatment is delayed ( 6 ) . delays in diagnosis and treatment of brucellosis - induced hip arthritis could lead to complications including dislocation and avascular necrosis of the femoral head ( 4 ) . although aspiration and examination of the joint fluid were not performed due to lack of synovial effusion on admission , considering the positive serological tests for brucellosis and concurrent avascular necrosis of the femoral head on mri , it seems that the delay in diagnosis and treatment of brucellosis in this patient had led to avascular necrosis of the femoral head . finally , the following questions arise : was avascular necrosis caused by increased intra - articular pressure related to the infection or due to direct involvement of the femoral head by the organism ? since brucellosis is a systemic infection with a broad clinical spectrum ranging from asymptomatic forms to deaths in severe cases , this disease should be taken into considerations when dealing with any patient with various and nonspecific symptoms in endemic areas with extension of the clinical signs of brucellosis . moreover , early diagnosis and long - term treatment and follow - up are of great importance in brucellosis .

backgroundbrucellosis is a zoonotic disease mostly transmitted to humans through consumption of unpasteurized dairy products and can lead to a systemic disease with any organ involvement . in this report , we describe a case of brucellosis - induced avascular necrosis of the hip . brucellosis was diagnosed through serological tests , and avascular necrosis of the femoral head was confirmed by pelvic mri . the patient was treated with a combination of antimicrobial treatments and referred to the orthopedic service for total hip arthroplasty . brucellosis may present with unusual manifestations and should be always taken into consideration , particularly in endemic areas .

primary retroperitoneal tumors ( prts ) of vascular origin are a diverse group of rare abdominal neoplasms , both benign and malignant . the most frequent malignant tumors are liposarcoma and leiomyosarcoma , while the most often found benign tumors are lipoma , leiomyoma and cavernous hemangioma [ 25 ] . hemangiomas are a group of neoplasms originating from vascular tissue where benign tumors prevail . among these capillary hemangioma , cavernous hemangiomas most frequently occur in the liver . other described localizations are skin , muscles , bones , central nervous system and retroperitoneal organs ( intestines , kidneys , adrenal glands , urinary bladder , uterus ) [ 616 ] . an interventional staged therapy is becoming more popular with the aim of reducing the diameter of the main hemangioma , especially that surrounded with diffuse hemangiomatosis . successful combination of transcatheter arterial embolization ( tae ) prior to the surgery of cavernous hemangioma of the liver was reported recently . nevertheless , the treatment of choice for primary retroperitoneal tumors is still radical surgical resection that leads to recovery . surgical techniques used for treatment of prts are : open procedures , laparoscopy , surgical endoscopy and percutaneous radiofrequency ablation [ 8 , 19 ] . the present case is a patient with giant retroperitoneal cavernous hemangioma originating from the ilium with atypical clinical course who was referred for surgical treatment . a 71-year - old female patient was referred to the department of surgical oncology with a giant retroperitoneal tumor located in the left iliac fossa . the patient discovered the abdominal tumor 15 years earlier , but as it did not cause any problems she refused to seek medical help . several months prior to admission to the department the first symptoms appeared : left lower limb edema , exertional dyspnea , abdominal distension and anemia with pallor and lowered hemoglobin level . physical examination revealed a large , skin modeling , nonpulsatile mass in the left iliac fossa ( fig . b computed tomography scan revealing a large , left - sided retroperitoneal mass when abdominal contrast - enhanced computed tomography ( ct ) was performed , a tumor 20 17 18 cm in diameter lying on the iliac ala was found ( fig . an ultrasonography - guided fine needle biopsy was performed , revealing blood cells with necrotic masses and connective tissue which prevented precise diagnosis . blood laboratory findings were : anemia ( hb 8.8 g / dl ) , elevated white blood cell count ( 21.81 g / l ) and high c - reactive protein level ( 100.8 mg / l ) . because of uncertainties regarding the histological type of the tumor , escalation of abdominal pain and left lower limb edema in recent weeks and the patient s strong willingness to remove the tumor , the decision to perform laparotomy was made ( figs . 2 a , b ) . a giant tumor with sigmoid colon tight on it . c the tumor after resection ( arrow peduncle connecting the tumor to the ala ) during the surgery a large mass on the ala was found . there were no signs of infiltration of neighboring organs , and the sigmoid colon was tight on the tumor . after separating the sigmoid and identification of the left ureter and iliac vessels massive damage to the internal surface of the ala was found in the place where the tumor grew . pathologic examination showed a tumor 20 19 16 cm in diameter , weighing 3000 g ( fig . histochemically the cells lining the lacunae were stained positively by markers for cd34 ( figs . 3 a , b ) . a microscopic image with h&e ( enlargement 10 ) . diag . staining with markers for cd34 confirm an endothelial origin of the cells lining the lacunae the postoperative follow - up is 3 years . within this time limb edema and this is caused by its initially symptomless course and therefore delayed presentation to a physician . the most frequent symptoms of retroperitoneal tumors are non - specific abdominal pain ( 51.8% ) and abdominal distension ( 18.7% ) . less frequent symptoms include constipation ( 8% ) , fever ( 8% ) , dysuria ( 4.3% ) , lower limb edema ( 6.5% ) , weight loss and cachexia ( 5% ) . the time of these symptoms occurrence varies from several months for sarcomas to years for benign tumors . patients usually visit a physician when they discover a pathological mass in the abdomen ( 60.4% ) . in the present case her first symptoms ( abdominal distension and limb edema ) occurred 15 years after discovering the tumor . on ct images retroperitoneal tumors present as heterogeneous pathological masses with hypoechogenic areas corresponding to necrotic zones in the tumor . we considered an interventional staged therapy with tae for its possible advantage of reduction of the diameter of the main tumor mass , and surrounding diffuse hemangiomatosis , which was reported in the case of cavernous hemangioma of the liver . the age of the patient with possible diffuse arteriosclerosis and the necessity to resect the symptomatic tumor were the arguments to abandon the percutaneous option . however , one can find publications on treatment of hepatic cavernous hemangioma with percutaneous radiofrequency ablation . in the present case the size and localization of the tumor and urgent necessity made the operation a high - risk procedure with possibility of resecting vital structures , such as the sigmoid colon or ureter . in the present case the long - term history of abdominal tumor indicated a benign tumor , while radiological and clinical findings suggested malignant disease . the literature review shows that in differential diagnosis of giant abdominal tumors , rare benign neoplasms should be considered in every case . radical surgical resection resulted in full recovery in our patient , which corresponds to commonly found conclusions despite the huge diameters and the origin of retroperitoneal tumors [ 20 , 21 ] . in the literature reports we found only 15 cases of retroperitoneal cavernous hemangiomas in kidneys , adrenal glands , urinary bladder , uterus and the retroperitoneal part of the rectum [ 615 ] . however , no description of cavernous hemangioma of the ilium was found ; therefore our report seems to be a unique contribution to contemporary knowledge ( tab . because of their initially symptomless course , primary retroperitoneal tumors are usually diagnosed in an advanced stage of the disease , which is the reason for many uncertainties regarding safe surgical resection . radical surgical resection remains the treatment of choice for primary retroperitoneal cavernous hemangiomas , although the decision of performing such operations should be taken after thorough analysis of indications ( escalation of symptoms ) and contraindications ( risk of damaging vital retroperitoneal organs with massive bleeding ) .

retroperitoneal hemangiomas are very rare . this paper presents the case of a 71-year - old female patient with giant cavernous hemangioma of the retroperitoneum who underwent surgical treatment for abdominal pain and left lower limb edema . interventional staged treatment with percutaneous transcatheter arterial embolization prior to surgery was considered . radical resection of the tumor was performed , which caused the symptoms to abate . additionally a literature review of cases involving cavernous hemangioma in the retroperitoneal space is presented . no description of retroperitoneal cavernous hemangioma originating from the bowel was found in the analyzed reports .

hidradenomas are benign cutaneous tumors of sweat gland origin , with the clear cell type constituting the most frequent histologic variety . usually , they are diagnosed in the elderly population , the peak incidence being the fifth to sixth decade . we describe a young girl , in whom nodular hidradenoma developed at the age of 6 years . hence , our case demonstrates that nodular hidradenoma is a rare differential diagnosis of skin tumors , even in the pediatric age group . a 10-year - old girl presented to us with a swelling on the left thigh , which had been progressively enlarging over the preceding 4 years . cutaneous examination revealed a solitary erythematous dome - shaped , well - circumscribed tumor , measuring approximately 6 cm 4.5 cm . there was spontaneous dripping of straw - colored serous fluid at the rate of 2030 drops / min , the discharge accentuating on pressure . biochemical analysis of the serous fluid revealed glucose 25 mg / dl , protein 4 g / dl , sodium 131 mmol / l , and potassium 4.3 mmol / l , almost similar to that of serum , the low concentration of glucose compelling us to think of something else . the mass was excised . the solid portion was largely composed of a cell type having finely granular , faintly eosinophilic cytoplasm with a dark - colored round to oval nucleus . the cyst was lined by a single layer of cuboidal epithelium without evidence of decapitation secretion [ figure 5 ] . solitary dome - shaped , well - circumscribed tumor over the left thigh photomicrograph showing a well - encapsulated mass composed of solid and cystic portions ( h and e , 40 ) photomicrograph showing a well - encapsulated mass with large cystic spaces within it . ductal luminal structures can be appreciated ( h and e , 100 ) photomicrograph showing clear cells in the center along with cystic spaces ( h and e , 400 ) note the large cystic cavity within the mass . clear cell hidradenoma or eccrine acrospiroma of the skin was first described by liu , in 1949 , as clear cell papillary carcinoma of the skin . it was reported under various designations such as nodular hidradenoma , eccrine acrospiroma , solid - cystic hidradenoma , clear cell acrospiroma , clear cell myoepithelioma , and eccrine sweat gland adenoma . clinically , nodular hidradenoma presents as a slow - growing , red- , blue- , or brown - colored solitary , 530 mm in size , freely mobile and firm nodule , with an occasional cystic appearance . these are most commonly found on the scalp , face , thorax , abdomen , and gluteal region . this tumor is found mainly in adults and is excised more commonly in women than in men . our patient was a young girl who presented with a gradually increasing swelling on the lower limb , a site that is unusual for the development of nodular hidradenoma . literature search showed very few cases of nodular hidradenoma on the lower limb , that too in the pediatric age group . however , a case of nodular hidradenoma masquerading as umbilical polyp has been reported in a 1-year - old male child our patient complained of serous discharge from the lesion , for the preceding 3 months . clinical differential diagnoses for our case were cutaneous lymphoma , dermatofibrosarcoma protuberans , epidermoid cyst , and sweat gland tumor . however , histology reliably excluded all the possibilities . it was predominantly composed of a cell type having finely granular , faintly eosinophilic cytoplasm with a dark - colored round to oval nucleus . the chronically discharging nature of the tumor in our patient was attributable to the fluid - filled cavity within the lesion . biochemical analysis of the fluid was almost consistent with that of serum , however , the low concentration of glucose hinted that it might be normal sweat . recently , nuclear grooving has been described as a useful morphological feature to aid in its diagnosis . immunohistochemical staining of the tumor cells demonstrates positive staining with antibodies against ck - cam 5.2 , ber - ep4 , p63 , epithelial membrane antigen , s-100 protein , smooth muscle actin , and vimentin . however , due to unavailability of resources and financial constraints , the immunohistochemical analysis could not be performed in our case . the high rate of local recurrence ( 10% ) and potential for malignant transformation make surgical removal with wide margins as the widely accepted modality of treatment . the high recurrence is attributable to incomplete resection and to tumor tissue located between the dermis and subcutaneous tissue . these are characterized by increased mitotic activity , angiolymphatic invasion , local extension into deeper tissues , and a dispersed pattern of growth . nodular hidradenoma in the pediatric age group is extremely rareit is usually located over the head , neck , and trunk . in our case , the lesion was present in the lower limbthe presence of an unusually large cystic cavity within the tumoral mass , leading to serous discharge , makes our case even more interesting . nodular hidradenoma in the pediatric age group is extremely rare it is usually located over the head , neck , and trunk . in our case , the lesion was present in the lower limb the presence of an unusually large cystic cavity within the tumoral mass , leading to serous discharge , makes our case even more interesting . nodular hidradenoma in the pediatric age group is extremely rareit is usually located over the head , neck , and trunk . in our case , the lesion was present in the lower limbthe presence of an unusually large cystic cavity within the tumoral mass , leading to serous discharge , makes our case even more interesting . nodular hidradenoma in the pediatric age group is extremely rare it is usually located over the head , neck , and trunk . in our case , the lesion was present in the lower limb the presence of an unusually large cystic cavity within the tumoral mass , leading to serous discharge , makes our case even more interesting . nodular hidradenoma in the pediatric age group is extremely rareit is usually located over the head , neck , and trunk . in our case , the lesion was present in the lower limbthe presence of an unusually large cystic cavity within the tumoral mass , leading to serous discharge , makes our case even more interesting . nodular hidradenoma in the pediatric age group is extremely rare it is usually located over the head , neck , and trunk . in our case , the lesion was present in the lower limb the presence of an unusually large cystic cavity within the tumoral mass , leading to serous discharge , makes our case even more interesting .

a 10-year - old girl presented with a swelling on her left thigh that was associated with dripping of serous fluid from the lesion . based on histological features of the excised tumor , a diagnosis of nodular hidradenoma was made . atypical features including large size of the tumor , location on the lower limb , a weeping presentation , and histological finding of a nodular hidradenoma with an unusually large cystic cavity discharging fluid prompted us to report the case .

carpal instability is broad category consisting of various patterns of injury , with dissociative type more common . a 13 -year -old boy presented at 6 months following a fall with restriction of wrist movements . patient had sustained a closed distal one -third both bones fracture forearm fixed with k -wire , and volar lunate instability was found during sequential follow -up . posttraumatic carpal instability should be identified at the earliest to avoid poor hand function and morbidity associated with it . carpal instability and its biomechanics have always been evolving due to the advancement in the field of radiology and arthroscopy . lunate plays a pivotal role and acts like an intercalated segment in carpal stability , loss of anchorage of this key structure cause intercalated segment instability . volar intercalated segment instability ( visi ) is a less common manifestation of dissociative carpal instability . a 13-year - boy had sustained distal one - third radius and ulna fracture after fall on an outstretched hand . open reduction and k - wire fixation was performed for both bone fracture forearm ( fig . 2 ) . he noticed restriction of dorsiflexion of wrist , while other movements normal . serial x - rays showed a loss of normal alignment of radio - lunate in comparison with the opposite ( fig . 3 , 4 ) . serial radiographs during follow - up showed a volar facing lunate with alteration of scapholunate and lunatocapitate angle and was diagnosed with a volar intercalated lunate instability ( fig . the patient was put on strict rehabilitation protocol and the dorsiflexion improved from 0 - 10 to 0 - 40. at 1 year follow - up patient is still having restriction of dorsiflexion ( fig . ( a ) post - operative radiograph of right wrist posterior - anterior and ( b ) lateral view showing reduced distal one - third radius and ulna fracture with implants in situ . ( a ) radiograph of wrist posterior - anterior and ( b ) lateral view at 2 months with uniting distal one - third radius and ulna fracture . ( a ) radiograph of both wrist posterior - anterior view left and ( b ) right showing union of distal one - third of radius and ulna . loss of gilula arc with flexed scaphoid suggesting carpal instability at 6-month follow - up . ( a ) radiograph of both wrists lateral view left and ( b ) right at 6 month follow - up showing volar facing lunate with loss of carpal alignment . ( a ) radiograh of right wrist at 1 year follow - up , post - anterior view and ( b and c ) lateral views , ( a ) showing healed distal one - third radius and ulna fracture , signet ring sign of scaphoid and moon like appearance of lunate . ( b ) volar facing lunate with loss of alignment between radius , lunate and capitate . ( c ) reduction in the scapholunate angle ( 16 ) and increased capitolunate angle ( 40 ) . ( a ) clinical photograph at 1 year follow - up showing reduction of dorsiflexion ( 0 - 40 ) and ( b ) normal palmar flexion ( 0 - 80 ) of wrist . carpal instability should be considered as a differential in patients presenting with chronic wrist pain and restricted movements following trauma . classified carpal instability into four categories and lunato - triquetral ligament is involved in stage three which leads to volar lunate instability . visi is a condition in which there is pathologic volar flexion of the lunate , with or without a similar posture of the other proximal row carpal bones . posttraumatic volar lunate instability is an entity that can be missed during the initial traumatic event because of its subtle nature . posttraumatic carpal stability diagnosed at the earliest avoids advanced surgical procedures such as reconstruction and fusion of joint . lunate instability in plain radiograph can be diagnosed with the alteration of various angles namely the loss of scapholunate angle , loss of alignment between the lunate , capitate and radius and increase in the capitolunate angle . treatment options include from closed reduction and k - wire fixation , dorsal capsulodesis , tenodesis , arthroscopic or open repair with reconstruction and finally arthrodesis . this injury could be subtle in nature before reduction and fixation that led to delay in diagnosis or iatrogenic in nature after reduction of the fracture . wrist function is a complex interaction of various biomechanics involving the carpal bones , ligaments , and the distal radio - ulnar complex . awareness of carpal instability associated with fractures or injuries around the wrist is needed by the treating surgeon to avoid delay in diagnosis and treatment . to suspect visi following a wrist injury and also following reduction with k - wire fixation . follow - up of patient at regular intervals to prevent morbidity due to this deformity .

introduction : carpal instability is broad category consisting of various patterns of injury , with dissociative type more common.case report : a 13 -year -old boy presented at 6 months following a fall with restriction of wrist movements . patient had sustained a closed distal one -third both bones fracture forearm fixed with k -wire , and volar lunate instability was found during sequential follow -up.conclusion : posttraumatic carpal instability should be identified at the earliest to avoid poor hand function and morbidity associated with it .

it behaves highly aggressive and spreads widely throughout the skin , recurs locally , and metastasizes early . standard treatment with resection and adjuvant radiotherapy results in local control in approximately 50% of patients at 1 year and median survival is approximately 8 months . considerable evidence has implicated matrix metalloproteinases ( mmp ) in the degradation of the extracellular matrix ( ecm ) during the metastatic process . murakami et al . reported the expression of mmp-2 on subcutaneous angiosarcoma in canine . in human , however , there is no english report suggesting the expression of mmp-9 on human angiosarcoma of the skin . in this report , we describe a case of angiosarcoma in which complete remission was archived with low dose docetaxel and bisphosphonate after the standard treatment of angiosarcoma . a 78-year - old woman consulted us with a 6-month history of an asymptomatic nodule on her scalp . her nodule has been surgically resected at a private clinic 3 months before her visit . two weeks after the resection , skin nodules had suddenly enlarged . on her first visit , physical examination revealed a skin - colored nodule with a surgery scar , teleangiectasia and purpura on her scalp ( fig . histologically , irregular anastomosing vascular channels lined by single layers of enlarged endothelial cells existed between collagen bundles with dense infiltration of lymphocytes ( fig . immunohistochemical staining revealed that these enlarged endothelial - like cells were strongly positive for cd31 and vimentin , and positive for factor viii and thrombomodulin , and negative for cd34 , s-100 , ck , sma , and desmin . moreover , these enlarged endothelial - like cells and tumor stromas were positive for mmp-9 ( fig . we used rabbit polyclonal anti - human mmp-9 antibody ( abcam , tokyo , japan ) at a dilution of 1:100 . in addition , we employed immunohistochemical staining for 9 cases of angiosarcomas that were histologically diagnosed in our institution , and about 78% ( 7/9 ) cases of angiosarcomas were positive for mmp9 . from these results , we diagnosed this patient as mmp-9-expressing angiosarcoma . positron emission tomography ( pet ) scans showed no evidence of metastases . then , we administered docetaxel monthly at 40 mg / m body surface area intravenously and weekly with oral administration of 17.5 mg sodium risedronate hydrate . one and a half years after the surgical treatment , there was no evidence of local recurrence or metastasis . in our present case , we describe a case of mmp-9-expressing angiosarcoma treated with low - dose docetaxel and bisphosphonate . like paclitaxel , docetaxel promotes microtubule assembly and inhibits the depolymerization of tubulin , thus stabilizing microtubules , but docetaxel has a higher potency [ 4 , 7 , 8 ] . however , in studies conducted in europe , docetaxel monotherapy was rather ineffective for soft - tissue sarcoma , including angiosarcoma , and could not be recommended for further use . mmp-9 is a stromal factor that regulates the mobilization of hematopoietic stem cells from the bone marrow niche by solubilizing the membrane - bound form of c - kitl . because it remodels the extracellular matrix and promotes the sprouting and growth of new blood vessels by making vegf available to the vegfr-2/flk receptor on endothelial cells , mmp-9 is a linchpin in tumor progression . moreover , recently , several reports revealed the expression of mmp-9 on tumor correlated with the progression or prognosis of several skin tumors such as malignant melanoma , squamous cell carcinoma , basal cell carcinoma , and mycosis fungoides . in addition , koontz et al . reported two cases of successful treatment of angiosarcoma with the administration of the anti - vegf antibody bevacizumab along with radiotherapy prior to surgery . more recently , it was reported that pharmacological inhibition of mmp-9 by amino - bisphosphonate decreased pro - mmp-9 and vegf in the serum and abrogated the induction of immunosuppressive macrophages , myeloid - derived suppressor cells ( mdscs ) , in the tumor microenvironment [ 15 , 16 ] . in aggregate , inhibition of mmp-9 by bisphosphonate could be one of the optimal supportive therapies for the treatment of angiosarcoma by inhibiting tumor - induced angiogenesis and by the induction of anti - tumor immunity . in conclusion , the combination of docetaxel with bisphosphonate was effective for mmp-9-expressing angiosarcoma and resulted in complete remission after the standard therapy for angiosarcoma .

we describe a 78-year - old japanese patient with angiosarcoma on the scalp . interestingly , immunohistochemical staining revealed this tumor as positive for matrix metalloproteinase 9 ( mmp-9 ) . after conventional therapy for angiosarcoma with surgical treatment and radiation therapy , we intravenously administered docetaxel at 40 mg / m2 body surface area together with oral administration of 17.5 mg sodium risedronate hydrate . one and a half years after the standard treatment , there was no evidence of local recurrence or metastasis .

erythromelalgia ( em ) is a rare disease characterized by painful swelling and erythema on the extremities [ 1 , 2 , 3 ] . the symptoms are often triggered by minor trauma , exercise , increased skin temperature , or heat exposure and often alleviated by cooling and elevation of the involved extremity . there are three distinct types : primary em , secondary em and em with thrombocythemia . primary em is observed most commonly in patients aged less than 30 years without underlying disorders . secondary em is associated with several autoimmune disorders [ e.g. systemic lupus erythematosus ( sle ) , insulin - dependent diabetes mellitus ] . in contrast , em with thrombocythemia is usually treated with aspirin , since the burning pain is relatively easy to control . in this report , we describe a case of adult - onset em successfully controlled with steroid pulse and pregabalin . a 68-year - old japanese woman presented at our outpatient clinic with a 10-year history of painful , recurrent erythema on her bilateral hands . there was no erythema on her feet . she had been diagnosed with idiopathic em and treated with oral intake of vitamin e 150 mg / day , transamin capsules 750 mg / day , prostaglandin e1 15 g / day and aspirin 100 mg / day , without any improvement . on her initial visit , physical examination revealed dark erythematous swelling on her bilateral hands ( fig . the biochemical profile revealed normal levels of antinuclear antibodies ( 40 ) and a slightly decreased igg level ( 702 mg / dl ) . no specific autoantibodies ( e.g. dsdna , ssdna , ss - a , ss - b ) were detected . prothrombin time , activated partial thromboplastin time , serum fibrinogen , and platelet count were within normal ranges . the patient was found to be euthyroid by a measurement of the basal tsh and free t4 levels as well as by trh provocative test . internal malignancy was excluded at the internal department . from the above findings , we diagnosed the patient as having adult - onset em of unknown origin . we administered methylprednisolone sodium succinate 1,000 mg / day intravenously for 3 days , and after that we treated her with oral prednisolone 60 , 40 and 20 mg / day for 2 days each . two days after the administration of methylprednisolone sodium succinate , eruption and pain had improved ( fig . 1b ) . the visual analog scale ( vas ) score improved from 10 to 2 ( fig . however , 6 days after therapy start , her pain gradually developed again ( vas score 2 to 5 ) . the vas score improved again , and the patient has been able to control her pain for half a year , although slight erythema could still be observed . em is a rare condition characterized by episodic bouts of burning pain , erythema , and edema , most commonly involving the extremities . symptoms are often triggered by minor trauma , exercise , or heat exposure and often alleviated by cooling and elevation of the involved extremity . several reports have suggested successful treatments for em [ 2 , 3 , 4 , 5 ] , but the optimal therapy is still under discussion . in this report , we describe a case of adult - onset em successfully controlled with steroid pulse and pregabalin as evaluated by vas . previous reports suggested an association between secondary forms of em and autoimmune disorders [ 3 , 6 , 7 ] . indeed , there is evidence that secondary em might be associated with an autoimmune condition in which there are autoantibodies directed against the sodium channel encoded for by the scn9a gene . in addition , jackson and oates reported a case of autoimmune - related em ; however , these autoantibodies were not suggested to be directly associated with em like sle . on the contrary , the primary form of em is associated with a mutation in the sodium channel nav1.7 , which is selectively expressed within the dorsal root ganglion and sympathetic ganglia and encoded by the scn9a gene [ 3 , 8 ] . once diagnosis is established , potential secondary causes must be excluded [ 6 , 9 ] . other causes associated with em include sle , raynaud 's disease , pernicious anemia , thrombotic thrombocytopenic purpura , infectious mononucleosis , metabolic , endocrine , and vascular origin and diabetic neuropathy [ 6 , 7 , 9 ] . although there was no evidence for autoimmune - associated em in our present case , late onset of em might indicate the possible involvement of autoantibodies . in addition , suh et al . reported that adult - onset idiopathic em could be successfully treated with corticosteroids and pentazocine . furthermore , pfund et al . for the above reasons , we decided to administer steroid pulse and pregabalin , and monitored the patient 's pain with vas . vas is a simple assessment tool consisting of a 10-cm line with 0 on one end , representing no pain , and 10 on the other end , representing the worst pain ever experienced , which a patient marks to indicate the severity of his or her pain ( fig . , the vas score and erythema on the hands immediately improved after administration of high - dose methylprednisolone sodium succinate and were maintained by oral pregabalin . although we describe a single case and the observation is limited , our case might suggest a possible , optimal therapy for adult - onset em .

adult - onset erythromelalgia ( em ) is a rare disease characterized by episodic bouts of burning pain and erythema for which the optimal therapy is unclear . in this report , we describe a 68-year - old japanese woman with adult - onset em . intravenous administration of methylprednisolone sodium succinate 1,000 mg / day dramatically improved her pain as evaluated by the visual analog scale . although the patient 's pain gradually developed again , it could be controlled with pregabalin . our present case might suggest a possible , optimal therapy for adult - onset em .

ischemic heart disease is rare during pregnancy , occurring in approximately one in 10,000 live births . the risk of myocardial infarction ( mi ) in pregnancy is reported from one per 37,500 to 6.2 per 100,000 deliveries.13 the diagnosis is by clinical findings , electrocardiogram ( ecg ) , and measurement of the serum level of the cardiac specific contractile protein , troponin 1.4 , 5 the most common forms of angina are stable and unstable angina , which are usually due to atherosclerosis . but coronary spasm , coronary dissection , and thrombus have been reported as other causes.6 , 7 prinzmetal s angina ( variant angina ) is rare and it accounts for only 2 out of every 100 cases of angina . we report a pregnant woman who presented with signs and symptoms of acute mi and whose ecg findings and troponin 1 level were compatible with mi , leading to the final diagnosis of prinzmetal s angina . a 35-year - old woman , gravid 3 ( with a history of two cesarean section operations ) , developed acute mi at the 30 week of gestation . she was a non - smoker and had no history of drug abuse or systemic disease . she referred to the emergency ward due to a sudden , severe pain in the neck and between the two scapulas that had started during sleep at 5 am , with cold sweat and nausea . her blood pressure was 200/150 mm hg ; she had a history of increased blood pressure of two weeks duration . the patient also had tachycardia , rales in the bases of the lungs , signs of anteroseptal mi in the ecg ( figure 1 ) , and increased troponin 1 blood level . we performed a cesarean section operation because of severe preeclampsia , repeat cesarean section , and transverse lie . four days after delivery , the patient s blood pressure was 140/80 mmhg and coronary angiography showed total occlusion of the second obtuse marginal artery ( om2 ) in addition to diffuse spasm of the left circumflex coronary artery ( lcx ) ( figure 2 ) . at the seventh day after delivery in the ward , the patient had chest pain and st - segment elevation in the inferior leads ( figure 3 ) as well as signs of pulmonary edema . the ecg changes reversed after intravenous trinitroglycerin ( figure 4 ) . based on the ecg , the diagnosis was inferior wall ischemia . we performed angiography for a second time and surprisingly the lcx and om2 coronary arteries were normal and there was narrowing in the rca ( figure 5 ) . we conclude that this discrepancy between the first and second ecgs and angiographic findings were due to prinzmetal s angina . a few days later , the patient was discharged ( blood pressure = 130/80 mmhg ) on diltiazem , atorvastatin , captopril , nitrocontin , furosemide , and clopidogrel while the ecg showed previous anteroseptal mi ( figure 6 ) . an ecg three months after discharge showed the same findings too ( figure 7 ) . mi in pregnancy is rare but can produce significant maternal and neonatal morbidity . prompt diagnosis and immediate therapy are necessary to lower the high likelihood of the mortality of mother and fetus . the highest incidence of mi seems to occur in the third trimester and in multigravidas older than 33 years old . acute mi in pregnancy is commonly located in the anterior wall , and acute maternal death rate is reported to be 1930%.13 , 8 complications of pregnancy that are significantly associated with acute mi are preeclampsia , postpartum hemorrhage , postpartum infection , and fluid and electrolyte imbalance.1 , 9 shock from postpartum hemorrhage,10 ergonovine,11 and prostaglandin e1 ( pge1)12 is reported as a risk factors for mi in pregnancy . our patient was a 35-year - old , obese , multiparous , pregnant woman with preeclampsia , and her symptoms of mi appeared at rest in the early hours of morning . prinzmetal s angina usually occurs at rest and happens between the midnight and early morning . people with prinzmetal s angina are often younger than those with the other forms of angina . the gold standard for diagnosis is coronary angiography with injection of provocative agents ( like ergonovine ) into the coronary arteries . . also administration of intracoronary nitroglycerin in cases of vasospasm can cause marked diffuse vasodilatation.13 treatment with calcium - channel blockers or nitrates eliminates spasm in most of these patients . in our patient , the discrepancy between the first and second angiographic findings was the confirmatory evidence that she had prinzmetal s angina . this conclusion was derived from the fact that the first angiographic examination showed that the lad coronary artery was normal despite the definite occurrence of an mi in its territory , thus suggesting transient spasm . furthermore , a subsequent bout of inferior wall ischemia with confirmation of transient spasm by angiography was another piece of confirmatory evidence . last but not least , asymptomatic rca spasm , detected in the last angiography , was another clue to the propensity of the coronary arteries to vasospasm , which is the sine qua non of prinzmetal s angina .

acute myocardial infarction ( mi ) during pregnancy is rare and mi due to prinzmetal s angina is much rarer . we present a 35-year - old , obese , multigravida , and pre - eclamptic woman , who developed acute anterior wall mi at the 30th week of gestation . on coronary angiography , the second obtuse marginal branch was totally occluded and the right coronary artery ( rca ) was normal . three days later , she had chest pain and st elevation in the inferior leads . on second angiography , there was narrowing in the rca , while the obtuse marginal branch was patent . we presume that this discrepancy between the first and second electrocardiograms and angiographic findings was due to prinzmetal s angina .

however , cardiac metastasis is diagnosed in less than 1% of patients with malignant melanoma because less than 10% of these patients present with cardiac symptoms.1 - 3 ) identification of cardiac metastasis from melanoma usually means that the patient is suffering systemic metastasis . unlike typical cardiac metastasized patients , we report a first case of a patient with a metastatic malignant melanoma in the heart without an identifiable primary source or additional metastasis in korea . a 59-year - old woman was admitted for cough and pleuritic chest pain with no history of malignancy or heart disease . her initial blood pressure was 110/70 mm hg , pulse rate 70 beats / min , respiratory rate 20/min , and body temperature was 36.1. jugular veins were not distended . laboratory studies , including a complete blood count , liver , and chemical profiles were in normal ranges . chest computed tomography showed a large amount of pericardial effusion and a mass in the right atrium ( ra ) ( fig . transthoracic echocardiography showed a large mass measuring 4231 mm in the ra , which did not obstruct tricuspid valve flow . effusion analysis showed a red blood cell count of 1.910/mm and a white blood cell count of 3300/mm ( lymphocytes , 55% ; neutrophils , 14% ) . cardiac mri also revealed a large mass surrounding ascending aorta spread into transverse sinus and around pulmonary trunk ( fig . considering the risk of open heart surgery and poor prognosis of extensive cardiac metastasis , pericardial window operation and epicardial mass biopsy were performed to relieve symptoms and confirm the pathological diagnosis . pathological analysis including immunohistochemistry revealed the final diagnosis to be malignant melanoma ( fig . positron emission tomography revealed no other distant organ metastasis except for heart and mediastinal lymph nodes . malignant melanoma has aggressive biological behavior and the greatest tendency for metastasis to the heart.1 ) although autopsy studies reported an incidence of 50% to 71% , cardiac metastasis is diagnosed in less than 1% of patients with malignant melanoma because less than 10% of these patients present with cardiac symptoms.2 ) such metastases most frequently occur after multifocal hematological dissemination and may develop anywhere in the heart.3 ) melanotic metastases can invade the wall of any of the 4 cardiac chambers , and the ra is involved most frequently.4 ) the clinical signs and symptoms of cardiac metastasis are unclear and non - specific , although when present , the clinical signs and symptoms include fatigue , weakness , pericardial effusion , congestive heart failure , cardiac arrhythmia , superior vena cava syndrome , right ventricular outflow and inflow obstruction , and transient ischemic attack.5 ) however , patients with malignant melanoma who have cardiac metastases may present symptoms only caused by tumors in other organ systems . although cardiac involvement occurs during the course of the disease , it is rare that the initial manifestation is cardiac metastasis . a tumor 's anatomic location and extent of invasion determine the feasibility of surgical intervention , which should optimally be performed during the early stages of the disease.6 ) a complete resection of an intracardiac melanoma prevents potential morbidities that are associated with progressive intracardiac growth , such as superior vena cava syndrome , right ventricular outflow and inflow obstruction , dysrhythmia , cardiac tamponade , and heart failure.6 ) even when total resection is not possible , conservative surgery can relieve symptoms and prevent imminent cardiac failure . conservative surgery improves the quality of a patient 's life , as in our patient 's case . although more than 90% of melanomas have a cutaneous origin,7 ) melanomas may sometimes present metastatically in the absence of a primary lesion , termed melanomas of unknown primary origin . most authors estimate that 2 - 6% of patients are diagnosed with metastatic melanoma of unknown primary site . in particular , such a metastatic melanoma in the heart such as in this patient without a known primary cutaneous origin is a rare presentation and chiefly an anecdotal finding of metastatic melanoma . several reported cases can be found in the literature about cardiac involvement with cutaneous primary malignant melanoma and multiple metastasis in korea , as well as worldwide.8)9 ) as far as we know , malignant cardiac melanoma without a primary origin has not yet been reported in korea . the survival of patients with unknown primary melanoma was demonstrated to be similar to that of patients with known primary tumors when corresponding stages were compared.10 - 12 ) those patients with metastases to any other visceral sites are described to have a 1 year survival rate of 41% and a median survival of approximately 6 months.11)13)14 ) accordingly , we expected that the outcome of this patient might be very unfavorable . although total resection was not possible because of extensive cardiac metastasis , a pericardial window operation could resolve her symptoms . after surgery , chemotherapy was planned , including cisplatin , dacarbazine , casmustine , and tamoxifen . however , the patient refused further chemotherapy after completion of two sessions . as only few reports about cardiac metastasis of malignant melanoma without cutaneous origin have been published , we are uncertain if conservative surgery is associated with prolonged survival and if the role of surgery for survival is worth further investigation .

malignant melanoma has a very high propensity to metastasize to the heart . however , melanoma may sometimes present as a metastatic lesion in the absence of a primary lesion , which are called melanomas of unknown primary origin . we report a case in which a patient presented with a metastatic maligant melanoma in the right atrium with pericardial effusion and without a primary origin .

imbalance of muscles around the knee can cause changes in the alignment of the knee joint1 . therefore , to prevent knee injuries in females , improved muscle balance around the knee during dynamic motions such as landing and balanced strengthening of lower limb muscles are necessary . kim et al.1 found kinematic differences in landing motions between male and female college students and reported that the valgus angles of females were more increased than those of males during a vertical landing motion . the decline squat exercise moves the line of gravity backwards and increases external momentum during extension in knee joints2 ; thus , the squat exercise on a decline board has had great effectiveness in reducing knee pain due to the selective strengthening of lower extremity muscles3 . this study investigated the effect of the single - leg , lateral oblique , decline squat exercise on sacroiliac ( si ) joint pain with knee pain . a 39-year - old female had severe pain in the right medial buttock and right anterior knee . she complained of continuous low back pain for 8 months , and examination revealed that her pelvis was tilted posteriorly . the patient was unable to sleep in the side - lying position due to the pain . the purpose and methods of the study were explained to the participant before her inclusion in the study , and she provided informed consent according to the principles of the declaration of helsinki . the pelvic inclination was measured with a palpation meter ( palm ; performance attainment associates , st . paul , mn , usa ) by one examiner . at the initial assessment , the anterior pelvic tilt angles were 3.5 and 6.5 on the right and left sides ( normal range , 11 4 ) , respectively . on palpation of the right medial buttock and right anterior patella , the visual analog scale ( vas ) scores were 7/10 and 6/10 , respectively . the si joint pain provocation tests used in this study were the gaenslen test and patrick test . the single - leg , lateral oblique , decline squat exercise is performed with descent to 90 knee flexion , followed by ascent to the initial position at the individual s natural speed . this study assessed the anterior pelvic tilt angle and the response to pain provocation tests before and after 4 weeks of performing the exercise . following the course of exercise , the anterior pelvic tilt angles were 8 and 10 on the right and left sides , respectively , and were higher than the initial values ( 3.5 and 6.5 ) . on palpation of the right medial buttock and right knee , the initial vas scores of 7/10 and 6/10 decreased to 2/10 for both regions . the decline squat exercise induces anterior pelvic tilt as the trunk is pushed backward to compensate for the feeling of the trunk tilting forward2 , 3 . the subject in the current study also experienced considerable reduction in lateral knee pain , due to decreased tension in the tensor fasciae latae or iliotibial band of the lateral knee . in particular , the pain in the si joint also subsided after the course of exercise . the decline board used for the single - leg , lateral oblique , decline squat exercise induced foot supination , which promoted ideal alignment of the hip and pelvis , preventing knee valgus . in addition , the single - leg position can activate muscles that provide pelvic stability more effectively than the two - leg position4 . manual pelvic compression was demonstrated to be a compensatory strategy to enhance the force closure mechanism and thus normalize the altered motor responses5 . the stability of the si joint through form and/or force closure mechanisms was proposed to facilitate load transfer to the pelvis5 . therefore , the single - leg , lateral oblique , decline squat exercise can be effective for treating si joint pain with knee pain caused by an abnormal quadriceps angle in females .

[ purpose ] this study investigated the effect of the single - leg , lateral oblique , decline squat exercise on sacroiliac joint pain with knee pain . [ subjects and methods ] a 39-year - old female had severe pain in the right medial buttock and right anterior knee . this study assessed the anterior pelvic tilt angle and pain provocation tests before and after single - leg , lateral oblique , decline squat exercise for 4 weeks . [ results ] following the course of exercise , the anterior pelvic tilt angles were increased , and the visual analog scale pain scores for both the right buttock and right knee were 2/10 . [ conclusion ] single - leg , lateral oblique , decline squat exercise may be effective for treating si joint pain with knee pain in females .

the past 15 years have seen major advances in our understanding of severity assessment in community - acquired pneumonia ( cap ) . prognostic tools have been promoted to guide all major management decisions in cap , including admission to the critical care unit . several recent studies , including the study by renaud and colleagues , have challenged us to re - evaluate how we consider severe cap , a concept for which there is still no universally accepted definition . since the development of the pneumonia severity index in 1997 , severe cap has been considered in terms of a patient 's risk of 30-day mortality determined by a combination of age , co - morbidities and physiological parameters measured on admission . the two most widely used scores , the pneumonia severity index and the curb65 score , were developed to predict 30-day mortality . it is recognised that the majority of pneumonia mortality occurs in older people , however , and that many patients who die are treated palliatively . nearly 50% of all deaths in patients with pneumonia and more than one - quarter of deaths within 30 days are related to co - morbidities rather than being directly pneumonia related . these scores therefore have important limitations arising from the use of 30-day mortality as an outcome . the scores may underestimate severity in young people and they perform less well when considering outcomes such as intensive care unit ( icu ) admission or requirement for mechanical ventilation or vasopressor support [ 7 - 9 ] . as few as 20% of patients in the highest pneumonia severity index class ( class v ) require icu admission , illustrating the system 's limited value for the critical care community . there is a growing consensus that icu admission and , more specifically , mechanical ventilation or vasopressor support are more useful outcomes than 30-day mortality to define severe cap and to identify the most acute ill patients [ 7,10 - 12 ] . the requirement for mechanical ventilation or vaso - pressor support is preferred to simply using icu admission , as evidence suggests that icu admission rates and criteria vary widely across different healthcare systems . this helps to explain why we see icu admission rates of 17% in spain compared with 8.7% in the uk or 4% in hong kong . the revised british thoracic society cap guidelines are due to be published in 2009 and will recommend using the curb65 criteria to determine icu admission . the infectious disease society of america american thoracic society guidelines recommend the revised american thoracic society criteria , which comprise two major criteria ( the requirement for mechanical ventilation and vasopressor support ) or three minor criteria ( comprising respiratory rate , pao2/fio2 ratio , multilobar infiltrates , confusion , uraemia , leucopenia , thrombocytopenia , hypothermia and hypotension requiring aggressive fluid resuscitation ) . alongside these criteria , renaud and colleagues , charles and colleagues and espana and colleagues it is reassuring that the high - risk features identified in each of these studies are similar , with acidosis , systolic blood pressure , respiratory rate , uraemia , confusion , hypoxaemia and multilobar infiltrates featuring in each of the derived scores . the abundance of severity criteria , however , reveals the lack of consensus over which patients should be initially managed in the icu . delayed transfer to the icu is associated with increased mortality , and therefore early recognition of these patients is important . the risk of early admission to intensive care unit score has been shown to predict patients with delayed admission to the icu . this group probably consists of patients in whom severity was underestimated on admission , of patients with treatment failure and of patients with unstable co - morbidities and nosocomial superinfection . all of the new scores are complex , making them difficult to implement in clinical practice . evidence suggests that current severity criteria , such as the curb65 score , are under - utilised . it may therefore be impractical to expect staff to use the curb65 score to decide on the site of care , then use smart - cop or the american thoracic society criteria to decide whether a patient requires icu care , and then use the risk of early admission to intensive care unit score to assess their risk of requiring icu subsequently . a perfect scoring system may not exist , but it should ideally predict both 30-day mortality and the requirement for mechanical ventilation or vasopressor support . the scoring system should be simple , composed of the fewest possible factors , and easy to remember in a busy emergency department . the system should function equally well in older patients and young patients , and should be based on physiological derangement and organ dysfunction rather than on age or co - morbidities . the hope is that future studies can identify physiological scoring systems or biomarkers that can achieve these goals and provide an effective adjunct to clinical judgement in the early management of cap . cap : community - acquired pneumonia ; curb65 : confusion , urea > 7 mmol / l , respiratory rate 30/min , blood pressure < 90 mmhg systolic and/or diastolic blood pressure 60 mmhg , and age 65 years ; icu : intensive care unit ; smart - cop : systolic blood pressure , multilobar chest radiograph involvement , albumin , respiratory rate , tachycardia , confusion , oxygenation and arterial ph .

the past 15 years have seen major advances in our understanding of severity assessment in community - acquired pneumonia ( cap ) . prognostic tools have been promoted to guide all major management decisions in cap , including admission to the critical care unit . several recent studies , including the study by renaud and colleagues , have challenged us to re - evaluate how we consider severe cap , a concept for which there is still no universally accepted definition . existing severity scores such as the pneumonia severity index and the curb65 score are designed to predict 30-day mortality . as a result , they are heavily weighted by age and co - morbidity . they perform less well when predicting other outcomes such as requirement for icu admission and are of limited use in the critical care environment . this commentary discusses recent attempts to develop useful severity criteria to guide the use of icu resources in patients with severe cap .

huntington 's disease ( hd ) is a dominantly inherited neurodegenerative disease caused by mutations in the it-15 gene on chromosome 4 , which was subsequently renamed htt . more specifically , the disease is linked to expansion of the cag repeat tract of this gene , which codes for polyglutamine repeats in the huntingtin protein . george huntington provided the classic description of the disorder in 1872 from cases in the east hampton area of long island , n.y . the best known manifestation is chorea , which consists of random , fluid involuntary hyperkinetic movements . the term is derived from the greek word o , meaning a traditional circle dance . it is important to note that , while movements in chorea may resemble a dance , they are not rhythmic or repetitive . psychiatric manifestations may include irritability , anxiety , obsessive compulsive disorder , paranoid ideation , and psychosis . depression is the most common psychiatric feature of hd and suicidal ideation occurs in 510% of hd patients . other neurologic manifestations include eye movement abnormalities , cognitive disturbance , pendular knee reflexes , motor impersistence , and postural instability . we describe a mild case of adult onset hd early in the disease course along with mri and diffusion tensor imaging ( dti ) features . increased fractional anisotropy ( fa ) of striatum the patient is a 50-year - old right - handed caucasian male who was diagnosed with hd at age 47 by an outside movement disorders specialist . the patient received a clinical diagnosis of hd given mild neurological findings in the context of genetically confirmed family history . there is a history of depression since his diagnosis , which has become worse over the past week . he was admitted for psychiatric evaluation due to suicidal ideation with plans to overdose on sleeping pills . in addition , he complains of mild cognitive disturbance for at least one year , with difficulty processing information in distracting environments . he has a history of alcohol abuse and quit alcoholics anonymous about six months ago . his medications include paroxetine 30 mg q.h.s . , aripiprazole 5 mg q.a.m . , naltrexone 50 mg q.a.m . , memantine 10 mg b.i.d . , and trazodone 200 mg q.h.s . upon examination , he was alert and oriented to person , place , and time . speech was fluent . cranial nerve examination was significant for saccadic pursuit , slow saccade initiation and velocity in the horizontal and vertical directions , and motor impersistence exhibited by inability to fully protrude the tongue for five seconds . there was only mild intermittent chorea in the fingertips bilaterally and rare lower facial chorea . he improved after one week of inpatient psychotherapy and was released for outpatient psychiatric and neurologic follow - up . images were obtained on a 3.0-tesla phillips achieva mri system . bicaudate ratio ( bcr ) , an imaging measure that correlates with caudate atrophy in hd , was obtained by measuring the shortest inter - caudate distance ( seen as indentation of the frontal horns ) on axial slice and dividing it by the distance between the outer tables of the skull along the same line , multiplied by 100 . phillips fibertrak software was used to analyze dti values on regions of interest which were defined as caudate head bilaterally and putamen bilaterally on axial slices . 1 ) . axial bcr in the patient was increased compared to control at 16 versus 12 , respectively . 2 ) was increased in the bilateral caudate and putamen versus control ( table 1 ) . depression and suicidality were managed with inpatient psychiatry admission and psychotherapy , followed by outpatient therapy . subtle neurologic manifestations included slowing of saccade imitation and velocity , saccadic pursuit , difficulty with tongue protrusion due to motor impersistence , pendular knee reflexes , and postural instability demonstrated by the inability to maintain tandem stance . tetrabenazine is fda approved for the treatment of hd chorea and its efficacy is supported by clinical evidence . caudate atrophy and ex vacuo dilatation of the frontal horns , which is typical for hd , was visualized . increase in fa in white matter is linked to more coherent fiber orientation and normal structure , but in striatal grey matter increased fa is due to neurodegeneration . propose that increased fa in striatal grey matter structures is due to the loss of efferent fibers which in turn leads to increased coherence . for example , in hd there is preferential loss of putamino - pallidal connections that are oriented mediolaterally as relative preservation of anterior - posterior and dorsal - ventral cortical - putaminal fibers . hence , loss of these mediolateral fibers may lead to more uniform fiber orientation and higher fa values . in conclusion , we have presented a patient who is early in the course of huntington 's disease and who already exhibits basal ganglia neurodegeneration with atrophy and changes in fa . dr . fekete received honoraria from medlink , inc . , and serves as a consultant for teva neuroscience , inc . , and lundbeck , llc .

huntington 's disease ( hd ) is a dominantly inherited neurodegenerative disease best known for chorea . the disorder includes numerous other clinical features including mood disorder , eye movement abnormalities , cognitive disturbance , pendular knee reflexes , motor impersistence , and postural instability . we describe a mild case of hd early in the disease course with depression and subtle neurological manifestations . in addition , we review mri and diffusion tensor imaging features in this patient . the bicaudate ratio , a measure of caudate atrophy , was increased . fractional anisotropy values of the bilateral caudate and putamen were increased , signifying neurodegeneration of these structures in hd .

total talar dislocations are uncommon injuries and usually seen following high velocity injuries . total talar dislocations we hereby report a closed total talar dislocation in a 25 year old male without an associated fracture around ankle . a 25 year old male presented to orthopaedic causality with injury to right ankle following a road traffic accident . patient complained of severe pain and deformity of ankle following injury . on examination ankle was deformed and swollen . plain radiographs of right ankle joint revealed total anterolateral dislocation of talus without any accompanying ankle fracture . total talar dislocations are very rare injuries and should be treated as impending open fractures . there is no consensus on treatment of such complex injuries as very few cases have been reported in literature . talar fracture dislocations are very rare injuries accounting for 0.06% of all dislocation and 2% of all talar injuries and are usually associated with malleolar fracture or a talar fracture itself[1 - 2 ] . total dislocation of the talus bone from all of its three joints ( tibiotalar , subtalar , and talonavicular ) without a fracture is a extremely rare injury[3 - 5 ] . these type of injuries are usually caused by high velocity injury with disruption of almost all ligaments and capsular attachments of the talus . it usually leads to degenerative changes in neighbouring joints and frequently avascular necrosis is a predictable outcome . we present a case of total talus dislocation without any fracture which was treated by closed reduction and immobilization for six weeks . a 25 years old male presented to orthopaedic causality with injury to right ankle following a road traffic accident . patient complained of severe pain and deformity of ankle following injury . on examination ankle was deformed and swollen with very tense skin . plain radiographs revealed total anterolateral i dislocation of talus without accompanying ankle fracture [ fig.3 , 4 ] , ct scan with 3d reconstruction also confirmed our radiographic findings [ fig.5 ] . under spinal anaesthesia and fluoroscopic guidance , closed reduction was performed by longitudinal traction and pushing the talus in the posteromedial direction . following a 3 mm k - wire was passed from calcaneum to tibia through talus [ fig.6 , 7 ] . plain radiographs at one year follow up showed no signs of avascular necrosis of talus or arthritis of subtalar joint . pre- operative clinical photograph showing talar dislocation pre operative clinical photograph showing abrasion over lateral malleolus . proposed treatments for total talus dislocation varied from primary talectomy or arthodesis to closed reduction and below knee cast . the few case reports found in the literature and the non - existent guidelines add to the confusion regarding the best method of treatment . depending on whether the foot comes to supination or pronation the dislocation can be either anterolateral ( the most usual ) or posteromedial . this type of injury is supposed to be in continuation of subtalar dislocation when the force magnifies and continues . dislocation of subtalar joint is the first stage of the injury . when the force progress , talonavicular joint dislocates and finally tibiotalar joint dislocation occurs . because of all capsular and ligamentous attachments of the talus are ruptured in this injury and dependence of talus bone vascularization on these ligaments , avascular necrosis of the talus is mostly predictable . there is a deference of opinion regarding best method of treatment for closed talar dislocations . most of the authors suggest open reduction for open talar dislocations [ table 1 ] . ritsema et al suggested open reduction as the best choice of treatment for closed injuries . anterolateral incision is preferred approach for open reduction as it gives an excellent approach to ankle joint . taymaz and gunal reported a case of closed total talus dislocation treated by closed means with excellent result . hadji et al . also reported good result after closed reduction of a complete talus dislocation on a three year follow - up . total talar dislocations are very rare injuries seen following a high velocity injuries and are associated with ankle fractures . closed reduction at the earliest is the best method of treatment for closed talar dislocations .

introduction : total talar dislocations are uncommon injuries and usually seen following high velocity injuries . total talar dislocations ( missing talus ) without fractures around the ankle are extremely rare . there are no consensuses on the best treatment of such injuries . to best of our knowledge very few cases have been reported in literature . we hereby report a closed total talar dislocation in a 25 year old male without an associated fracture around ankle . he was managed with emergency closed reduction and below knee splint . at one year follow up there were no complications.case report : a 25 year old male presented to orthopaedic causality with injury to right ankle following a road traffic accident . patient complained of severe pain and deformity of ankle following injury . on examination ankle was deformed and swollen . plain radiographs of right ankle joint revealed total anterolateral dislocation of talus without any accompanying ankle fracture . ct scan with 3d reconstruction also confirmed our radiographic findings . under spinal anaesthesia and fluoroscopic guidance closed reduction was performed . to maintain reduction a 3 mm k - wire was passed from calcaneum to tibia through talus . a posterior below knee splint was applied . patient was followed every three months till one year . at one year follow up patient had good range of motion at ankle and subtalar joint.conclusion:total talar dislocations are very rare injuries and should be treated as impending open fractures . there is no consensus on treatment of such complex injuries as very few cases have been reported in literature .

airprom : airway disease , predicting outcomes through patient specific computational modeling ( fp7 ) ; bioshare - eu : biobank standardization and harmonization for research excellence in the european union ( fp7 ) ; ict : information communication technology ; medall : mechanisms of the development of allergy ( fp7 ) ; naepp - epr3 : national asthma education and prevention program , expert report 3 ; ncd : non - communicable disease ; p4 , predictive , preventive , personalized and participatory ; u - biopred : unbiased biomarkers in prediction of respiratory disease outcomes ( fp7 ) ; un : united nations ; who : world health organization . the authors declare that they have no competing interests in relation to the content of this article . may 2008 : 61st world health assembly . who recommended a worldwide priority policy on ncd prevention and control ( 2008 to 2013 ) , including cardiovascular disease , cancer , chronic respiratory diseases and diabetes , not least because they often have common environmental risk factors . may 2010 : united nations ( un ) general assembly unanimously adopted resolution a / res/64/265 : ' tackling ncds constitutes one of the major challenges for sustainable development in the 21st century ' . december 2010 : the council of the european union adopted conclusions based on innovative and global approaches for ncds in public health and healthcare systems to further develop population - based and patient - centered policies . 2010 : us center for disease control and prevention ( cdc ) says that ' an essential strategy for keeping older adults healthy is preventing ncds and reducing associated complications ' . the classical definition of prevention includes : primary prevention : to avoid the development of disease . secondary prevention : recognize a disease before it results in morbidity ( or co - morbidity ) . tertiary prevention : to reduce the negative impact of established disease by restoring function and reducing disease - related complications . expanding on the traditional model of prevention , gordon proposed a three - tiered preventative intervention classification system on the basis of the population for whom the measure is advisable based on a cost - benefit analysis : universal prevention addresses the entire population ( for example , national , local community , school , and district ) and aims to prevent or delay risk factor exposure . all individuals , without screening , are provided with information and skills necessary to prevent the problem . the subgroups may be distinguished by characteristics such as age , gender , family history , or economic status . indicated prevention involves a screening process . according to these definitions , health promotion should be used for primary universal and selective prevention strategies , whereas p4 medicine ( predictive , preventive , personalized and participatory ) should be used for primary , secondary and tertiary indicated prevention strategies . to prevent the occurrence of ncds by implementing effective action at societal and individual levels : to detect and diagnose disease at an early stage , when it can be controlled effectively . to stratify patients into groups , enabling the selection of optimal therapy . to reduce adverse drug reactions through the predictive or early assessment of individual drug responses and assessing genes leading to ineffective drug metabolism . to improve the selection of new biochemical targets for drug discovery . to reduce the time , cost , and failure rate of clinical trials for new therapies . to shift the emphasis in medicine from reaction to prevention and from disease to wellness . part of the conceptual work presented has received support from the european commission fp7 projects airprom ( grant agreement fp7 270194 ) , bioshare - eu ( grant agreement fp7 261433 ) , medall ( grant agreement fp7 264357 ) , synergy - copd ( grant agreement ) and u - biopred ( grant agreement imi 115010 ) . jb , jma , ac - t , fk , mlk , sp , cp and ca were supported by medall ; pjs , i m a and kfc were supported by u - biopred ; jr and aa were supported by synergy - copd ; cb was supported by airprom ; ac - t was supported by bioshare - eu . the positions , proposals and ideas expressed in this paper have been discussed by several authors ( ca , zc , lh , ab , jb , ac , sa , dc , dn ) during the inaugural event of the european institute for systems biology and medicine of the systemoscope international consortium at the biovision world life sciences forum in lyon on 28 march 2011 .

we propose an innovative , integrated , cost - effective health system to combat major non - communicable diseases ( ncds ) , including cardiovascular , chronic respiratory , metabolic , rheumatologic and neurologic disorders and cancers , which together are the predominant health problem of the 21st century . this proposed holistic strategy involves comprehensive patient - centered integrated care and multi - scale , multi - modal and multi - level systems approaches to tackle ncds as a common group of diseases . rather than studying each disease individually , it will take into account their intertwined gene - environment , socio - economic interactions and co - morbidities that lead to individual - specific complex phenotypes . it will implement a road map for predictive , preventive , personalized and participatory ( p4 ) medicine based on a robust and extensive knowledge management infrastructure that contains individual patient information . it will be supported by strategic partnerships involving all stakeholders , including general practitioners associated with patient - centered care . this systems medicine strategy , which will take a holistic approach to disease , is designed to allow the results to be used globally , taking into account the needs and specificities of local economies and health systems .

recently , a single center study conducted by oiva and coworkers and published in critical care demonstrated that phospho - specific whole blood flow cytometry could be used to assess activated signaling path - ways in leukocytes isolated from pancreatitis patients . pancreatitis is an inflammatory process in which pancreatic enzymes are activated leading to autodigestion of the pancreas and adjacent tissues . during pernicious cases , the initial inflammatory response is excessive and can result in tissue injury , organ failure , and death . to counter this inflammation , these steps can include leukocyte apoptosis , anti - inflammatory cytokine production , and anergy . when patients become leukopenic and immunosuppressed , they often become susceptible to infections with organisms to which a healthy patient would normally be resistant . although therapy aimed at blunting the initial over - exuberant inflammation may be beneficial if applied early , it may also be detrimental by impairing the host 's ability to fight infection that is a common cause of death in these patients . to complicate the potential use of immune - modulating therapy , inflammatory and anti - inflammatory processes can occur along different time - lines for the varied leukocyte subsets . for example , during immune response , t lymphocytes might be undergoing apoptosis while neutrophils are primed to produce reactive oxygen species . thus , during prolonged pancreatitis , leukocyte subsets may be concurrently exhibiting both pro- and anti - inflammatory processes . as noted above , a potential promising therapy of pancreatitis is modulation of the immune response . this is a difficult undertaking as the host response will be affected by a number of variables , including genetic background , co - morbidities , age , gender , and so on . in addition , the patient 's immune response will vary during the course of the disease . thus , what is needed for immunotherapy to be practical is a rapid , robust measure of the host immune system . in the manuscript published by oiva and co - workers , the authors determined the signaling profiles of circulating leukocytes isolated from pancreatitis patients using phospho - specific whole blood flow cytometry . this is a powerful new technology that allows for simultaneous single - cell determination of leukocyte subsets using cell surface markers as well as intracellular protein phosphorylation . this work represents a continuation of work published previously . in this earlier work , oiva and co - workers demonstrated that stimulated monocytes isolated from patients with acute pancreatitis had decreased phosphorylated erk 1/2 , nf-b , and stat1 and 3 . the authors concluded that these changes could lead to impaired monocyte recruitment as well as increased susceptibility to infections . here , changes in activated t lymphocyte p38 , nf-b , stat1 and stat6 activity was observed that could be interpreted as being pro- or anti - inflammatory . interestingly , they determined that patients ' lymphocytes exhibited decreased phosphorylated nf-b and stat1 and increased phosphorylation of p38 and stat6 , suggesting a transition from a th1 to th2 phenotype . additionally , this minimally invasive methodology could be used to generate immune status within 6 hours under ideal conditions . thus , this methodology represents an essential step prior to targeting the underlying immune response to pancreatitis . although beyond the scope of the report , a potentially necessary step after immune status determination , and prior to treatment with inflammatory altering treatments , would be to first treat the whole blood cells isolated from the patients with the potential therapeutic agent and evaluate the immune effector cell response . phospho - specific flow cytometry could also be used to determine if the leukocytes responded in a way that would be beneficial . thus , the minimally invasive , relatively quick methodology developed in this paper could be utilized to determine immune status as well as provide a method to test potential therapies . one unavoidable limitation to the report is that the signaling processes were determined using only peripheral leukocytes . a possibility exists that peripheral leukocytes may not respond similarly to ex vivo stimuli as leukocytes isolated from the inflammatory site(s ) . when feasible , future studies need to be undertaken to compare the response of peripheral leukocytes to these tissue leukocytes . rapid results from whole blood phospho - specific flow cytometry during pancreatitis will allow for immune status determination , likely improve early diagnosis and provide a rational basis for immune targeting therapies . this work was supported in part by national institutes of health grants gm44118 and gm55194 ( to rsh ) , gm72760 ( to ccc ) , and the alan a and edith l wolff foundation .

recently , a single center study conducted by oiva and coworkers and published in critical care demonstrated that phospho - specific whole blood flow cytometry could be used to assess activated signaling pathways in leukocytes isolated from pancreatitis patients . the authors demonstrated that this methodology had the potential to determine the current status of a patient 's immune state . although the experimental cohort was clinically homogeneous , the observed data were heterogeneous . altogether , these results suggest that prior to administering immune - modulatory therapies in inflammatory diseases , it will be beneficial to first determine immune status . rapid results from whole blood phospho - specific flow cytometry may allow for determination of immune status , improve early diagnosis , and provide a rational basis for immunomodulatory therapies .

a 50-year - old male ( 182.9 cm , 78.5 kg ) was scheduled for arthroscopic debridement of the left shoulder . the patient had a history of old pulmonary tuberculosis and tb pleurisy ( 20 years ago ) and had no previous surgeries . his mallampati class was 2 and he had been npo for more than 8 hours . 1 ) showed a lesion suspicious for tracheal stenosis , but it 's formal decipher by radiologist was no active lung lesion . so , no further evaluation of airway have been done before surgery . in the operating room , electrocardiogram ( lead ii ) , pulse oximetry , non - invasive blood pressure , end - tidal carbon dioxide concentration ( etco2 ) and bispectral index were monitored . following 3 minutes of preoxygenation , general anesthesia was induced with lidocaine 60 mg , propofol 120 mg , rocuronium 60 mg and remifentanil 0.5 - 1.0 mcg / kg / min . on direct laryngoscopy , cormack and we tried to insert a 7.5 mm reinforced endotracheal tube with a stylet , but this procedure failed . we then attempted to perform a blind intubation by inserting a 6.5 mm reinforced endotracheal tube with a stylet by another anesthesiologist , but we felt resistance under the vocal cords . we decided to try intubation with optiscope pm 201 ( video assisted system , kyung won medical , seoul , korea ) , but this attempt failed because a 6.0 mm reinforced endotracheal tube stopped at subglottic level . 2 ) was chosen , and the device was inserted without difficulty on the first attempt . it was inserted blindly , until mild resistance was felt , as recommended by the manufacturer . the patient 's lungs were ventilated with a tidal volume of 650 ml at a rate of 12 breaths per minute . the i - gel provided a good seal , and allowed for controlled mechanical ventilation with acceptable peak pressures ( 12 - 15 mmhg ) while the patient was in the beach - chair position . the operation time was 1 hour 40 minutes and the time under anesthesia time was 3 hours . after neuromuscular recovery was demonstrated , the patient was awakened , and the i - gel was removed . many anesthesiologists use a narrow endotracheal tube . however , with this method , there is an associated risk of failed intubation . the procedure can also lead to complications such as tracheal mucosal trauma , perforation or bleeding . in some patients with tracheal stenosis , airway control might be achieved by the use of an endotracheal tube positioned above the stenosis . if the stenotic segment is in the proximal trachea , however , proper fixation of the tube is difficult . trauma , neoplasm , inflammation , wegener 's granulomatosis and prolonged endotracheal intubation are the causes of the tracheal stenosis . in the case radiography can be very useful , but chest radiographs can also appear normal in patients with tracheal stenosis . sads have advantages such as easy insertion and high effectiveness in securing a difficult airway . the i - gel is a single - use non - inflatable supraglottic airway device which is launched throughout the uk in january 2007 . it is composed of styrene ethylene butadiene styrene , which is soft and covers the larynx without cuff inflation . the seal pressure improves over time due to the thermoplastic properties of the gel cuff which may form a more efficient seal around the larynx after warming to body temperature . when correctly inserted , the tip of the i - gel will be located in the upper esophageal opening . it is designed to achieve a mirror of the supraglottic anatomy ( pharynx and larynx ) and the noninflatable cuff is semirigid . donaldson and michalek reported a successful use of i - gel for subglottic stenosis patient who was undergoing laparoscopic cholecystectomy . the i - gel has an integral bite block and may decrease rotation within the oropharynx . suggested that the anatomic position of the i - gel does not vary with head and neck position and that ventilation with the i - gel can be effectively performed with the head and neck in the extended or rotated position . in this report , we have presented a case of failed intubation in a patient with subglottic stenosis successfully managed with the i - gel. we suggest that the i - gel is a suitable choice in situations in which endotracheal tube insertion is difficult or has failed .

the airway management of patients with subglottic stenosis poses many challenges for the anesthesiologists . many anesthesiologists use a narrow endotracheal tube for airway control . this , however , can lead to complications such as tracheal mucosal trauma , tracheal perforation or bleeding . the asa difficult airway algorithm recommends the use of supraglottic airway devices in a failed intubation / ventilation scenario . in this report , we present a case of failed intubation in a patient with subglottic stenosis successfully managed during an i - gel supraglottic airway device . the device provided a good seal , and allowed for controlled mechanical ventilation with acceptable peak pressures while the patient was in the beach - chair position .

during the last years tyrosine kinase inhibitors ( tkis ) have changed the natural history of metastatic non - small - cell lung cancer ( nsclc ) harboring epidermal growth factor receptor ( egfr ) mutations . eight important studies were conducted to evaluate the efficacy and tolerability of tkis on advanced nsclc in comparison with standard platinum - based chemotherapy . not surprisingly , the use of tkis was correlated with a higher response rate , a longer progression - free survival and a better quality of life in patients with advanced nsclc activating egfr mutation . the iressa pan - asia study ( ipass ) , which enrolled 1,217 patients , was the largest trial in which patients were randomized to receive gefitinib or standard chemotherapy , and in the group of tkis therapy the primary endpoints were reached obtaining a statistically significantly higher response rate , a longer progression - free survival and better symptom control . similar results were reported by first - signal and by west japan thoracic oncology group ( wjtog 3405 ) studies . the north - east japan study group ( nej002 ) trial was stopped early because gefitinib showed a significantly higher progression - free survival in comparison with standard chemotherapy in patients with advanced lung adenocarcinoma activating egfr mutation . impressive results were also reported with the use of other tkis such as erlotinib or afatinib versus chemotherapy in patients carrying the same egfr mutations . better responses were observed in patients with mutations in exons 1821 of the tyrosine kinase domain of egfr . however , egfr gene mutations were also identified in small - cell lung cancer ( sclc ) [ 3 , 4 ] and in large - cell neuroendocrine carcinoma ( lcnec ) of the lung . lcnec is a high - grade carcinoma ( > 10 mitoses/2 mm ) belonging to the neuroendocrine tumors of the lung . it represents about 3% of all pulmonary malignancies and is characterized by neuroendocrine cytologic features ( formation of rosettes , trabeculae and perilobular palisading pattern ) and markers ( neuron - specific enolase , cd56 , synaptophysin , chromogranin and leu7 ) . in fact , the cytologic and biologic features of lcnec are different from those of large - cell carcinoma . the molecular alterations that are commonly found in lcnec are p53 , bcl-2 overexpression and rb mutation . to our knowledge , few cases of lcnec with egfr gene mutation have been described up to now , and only one case was treated with gefitinib , with a good response [ 7 , 8 ] . a 47-year - old caucasian woman with no family history of neoplastic diseases and no comorbidities was examined by a general practitioner after the appearance of back pain unresponsive to usual non - steroidal anti - inflammatory drugs . standard chest x - ray showed a left lung perihilar lesion , probably suggesting pneumonia . as a consequence , thus , after 2 weeks , chest x - ray was repeated and showed persistence and stability of the left lung lesion . about 1 month later , the patient came for the first time to our attention for appearance of vomiting , dyspnea , fatigue and abdominal pain ( visual analog scale 7 ) . abdominal physical examination revealed a painful hepatomegaly . she underwent a total body computed tomography ( ct ) scan that showed multiple focal liver lesions , solid left lung tissue and multiple secondary brain lesions ( two left frontal cerebral lesions , one right parietal lesion and two cerebellar lesions ) ( fig . 1 ) . as a result , a liver biopsy was performed . since all investigated tumor markers ( carcinoembryonic antigen , carbohydrate antigen 19 - 9 , carbohydrate antigen 125 , neuron - specific enolase , glycoprotein hormones alpha polypeptide ) were increased , it was not possible to identify the primary site of localization of the tumor and to reach a definitive diagnosis . given the rapidly progressive impairment of her clinical conditions and performance status , we administered an empirically not targeted chemotherapy with gemcitabine 1,000 mg / m die 1 and oxaliplatin 100 mg / m die 2 q 2 weeks although we did not yet have definitive histopathological results . although the sample was poor , the diagnosis was evocative of lung adenocarcinoma ( ttf-1 positive , cytokeratin 7 positive ) . however , since a further deterioration of her clinical condition was observed , a biopsy was repeated in order to have an additional sample for molecular analysis . therefore , we started tki therapy and gefitinib was administered at 250 mg p.o . once a day . the patient reports a good quality of life and no relevant side effects ( skin toxicity grade 1 ) have been registered . a restaging total body ct scan showed a significant improvement of disease , with a partial response > 50% . particularly , the brain ct scan showed a significantly reduced volume of the lesions ( valuable only 2/5 lesions ) and complete resolution of edema ( fig . lcnec is a rare tumor which is usually treated with cisplatin - based chemotherapy as sclc as it shares many characteristics with it . usually , the administration of tkis is considered only for advanced lung adenocarcinoma because the most important studies demonstrating higher activity of tki , in comparison with standard platinum - based chemotherapy , were conducted in patients affected by nsclc with egfr mutations . some features are associated with a higher possibility that egfr mutations are present in nsclc . the research of egfr mutations is not performed routinely on lcnec since this mutation is not commonly present in this subtype . the prognosis of this tumor with standard chemotherapy is poor , thus we need to evaluate alternative diagnostic strategies such as the investigation of egfr mutational status in order to explore new effective treatments . we report the case of a patient affected by lcnec carrying all of the predictive characteristics associated with egfr mutation ( caucasian , female , never - smoker ) . when the patient came to our attention she was very symptomatic , so in an attempt to reach a better and faster response , we decided to test egfr mutational status . strikingly , the mutation was present , and we were allowed to administered gefitinib , obtaining an impressive and objective response . therefore , we suggest the research of egfr mutations also in patients with lcnec to offer them one more therapeutic option .

large - cell neuroendocrine carcinoma ( lcnec ) of the lung is a high - grade carcinoma belonging to the neuroendocrine tumors of the lung and is different from typical lung large - cell carcinoma . it represents about 3% of all pulmonary malignancies and is characterized by neuroendocrine cytologic features . the treatment usually is platinum - based chemotherapy , however the outcome remains poor . therefore new therapeutic options are needed . tyrosine kinase inhibitors have demonstrated greater efficacy and better tolerability than standard chemotherapy in non - small - cell lung cancer harboring epidermal growth factor receptor ( egfr ) mutations . egfr gene mutations were also rarely identified in lcnec . we report a patient with lung lcnec activating egfr mutations who showed an impressive response to gefitinib .

tetralogy of fallot ( tof ) accounts for approximately 5% to 7% of all cases of congenital heart disease . complications related to surgical repair of tof are clinically relevant and include pulmonary regurgitation , residual obstruction of the right ventricular ( rv ) outflow tract , rv systolic dysfunction , and arrhythmias . among these , residual rv outflow tract obstruction with accompanying pulmonary regurgitation is the most frequently reported sequela after initial surgical correction of tof . therefore , a systolic murmur that is audible over the base of the heart is frequently interpreted as a residual rv outflow tract lesion . however , this is not always true . we herein report a rare case of subaortic stenosis in association with a previous tof surgical repair that was not considered to be a differential diagnosis . discrete subaortic stenosis is generally thought of as a disease of children or young adults ( < 25 years of age ) and is thus rarely reported in subjects older than 25 years , particularly in association with tof . a 29-year - old woman presented for a routine check - up of surgically corrected congenital heart disease . at 7 years of age , she had undergone surgical repair of tof with excision of the infundibular stenosis and closure of a 10-mm ventricular septal defect . she had been asymptomatic until her first decade of life , and only a 5-year clinical follow - up had been performed after the operation . approximately 6 months prior to presentation , she began to feel exertional dyspnea that was progressively aggravated . on physical examination , she was alert and well - oriented . her blood pressure was 100/60 mmhg , pulse 60 beats / min , respiration rate 18/min , and body temperature 36.6. the left ventricular ( lv ) impulse was almost normal in location . on auscultation , a grade 3 of 6 mid- to late - systolic murmur was audible over the base of the heart and heard clearly on both sides of the sternum . routine blood examinations , including a complete blood count , chemistry panel , coagulation panel , and c - reactive protein level , were all within normal ranges . transthoracic and transesophageal echocardiograms both demonstrated severe subaortic stenosis due to a subvalvular membrane , on which a mobile mass - like structure that was suspicious of a vegetation was noted ( fig . there was a mild degree of tricuspid regurgitation , but no clinically significant obstruction of the rv outflow tract . the maximum velocity measured across the tricuspid valve was 2.8 m / sec , and thus the maximal pressure gradient was estimated to be 31.4 mmhg using the simplified bernoulli equation . due to the up to 20 mmhg pressure gradient across the pulmonary valve her rv was not dilated , and its systolic function was good . due to the suspicion of infective endocarditis despite the absence of clinical evidence , blood cultures were performed three times ; however , no pathogen was cultured . the patient underwent resection of the subaortic membrane and myomectomy of the lv outflow tract along with aortic valve commissurotomy . the membrane , with the exception of the noncoronary cusp area , was located beneath the aortic annulus ( i.e. , inverted u shape ) . the mobile mass - like structure described on transesophageal echocardiography was proven to be loose tissue attached to the subaortic membrane . although subaortic stenosis is the second most - common form of lv outflow tract obstruction , it is predominantly found in children or adolescents [ 4 - 8 ] . subaortic stenosis is strongly associated with other congenital heart defects ; however , subaortic stenosis in association with tof , especially in women , is extremely rare . according to a medline database review of the last 30 years furthermore , all were diagnosed in childhood or adolescence ( < 20 years of age ) with two exceptions . the present case is peculiar in that subaortic stenosis manifested more than 20 years after the initial tof surgical correction . although we could not definitively say that the subaortic stenosis in the present case was acquired , we consider it an acquired or progressive form given the long interval ( > 20 years ) before echocardiographic confirmation . thomas and foster suggested that acquired subaortic stenosis can develop gradually due to hemodynamic disturbances derived from coexisting lesions or after surgical correction . moreover , the mass - like mobile structure observed on transesophageal echocardiography could be regarded as a degenerative byproduct of flow convergence induced by the subaortic membrane , further supporting the concept of an acquired or progressive form of the disease . discrete subaortic stenosis is well - known to be linked to important complications , such as bacterial endocarditis and significant aortic regurgitation . flow turbulence distal to the obstructing subaortic membrane plays a crucial role in progressive damage to the aortic valve with resultant thickening of its leaflets and regurgitation . the secondary jet lesion also predisposes the patient to bacterial endocarditis . in this context , in particular , patients with surgically corrected tof tend to be misinterpreted as having only residual rv outflow obstruction without consideration of the lv outflow tract lesion . transthoracic and transesophageal echocardiography can assist differentiation of the rv from the lv outflow tract obstruction . therefore , periodic performance of echocardiography should be kept in mind during follow - up of patients with surgically corrected tof .

we herein report a rare case of subaortic stenosis in association with a previous tetralogy of fallot ( tof ) surgical repair , which was not taken into account as a differential diagnosis . echocardiography plays a pivotal role in identification of this rare combination . therefore , echocardiography should be performed periodically during follow - up of patients with surgically corrected tof . given the clinical complications that can result from subaortic stenosis ( i.e. , aortic regurgitation and infective endocarditis ) , early and aggressive management of this rare combination should be performed .

in a recent issue of critical care , meyer and colleagues report interesting surveillance data from icus in 30 german hospitals , based on a large amount of microbiology and pharmacy data gathered between 2001 and 2008 . one of the main study findings is a decreasing rate of methicillin - resistant staphylococcus aureus ( mrsa ) and a dramatic increase of third generation cephalosporin ( 3gc ) resistant enterobacteriaceae over the study period . in recent years , most european countries have succeeded in reducing the burden of disease caused by mrsa . this progress has , however , been partly off set by the increase across europe in the prevalence of multiresistant gram - negative pathogens producing extended - spectrum betalactamases ( esbls ) or carbapenemases . the current study confirms this worrisome finding and also shows an increase in carbapenem , 3gc and fluoroquinolone use in german icus with a stable volume of overall antibiotic use . the latter two antibiotic classes have been repeatedly identified as risk factors for carriage of multidrug resistant gram - negatives . carbapenems on the other hand are first - line drugs for the treatment of infections due to esbl - producing bacteria . it is tempting to assume that overuse of fluoroquinolones and 3gc antibiotics contributed to the observed increase in esbl producers , which subsequently increased carbapenem use . the ecological nature of the data , however , makes it difficult to infer clear cause - and - effect relationships , as does the failure to differentiate between hospital- and community - acquired isolates and clinical versus surveillance cultures . in addition , the analysis of trends is hampered by variation in the number of participating icus over time . nevertheless , the increased burden of multidrug - resistant gram - negative bacteria is a real phenomenon . in contrast to mrsa , esbl producers - notably strains of escherichia coli carrying plasmids of the ctx - m family - are mostly imported from the community into the hospital . assuming a relationship with antibiotic use in animals and subsequent transmission of antibiotic resistant e. coli via the food chain is alluring , but strong epidemiological evidence is still lacking . the recently started european union - funded saturn project ( impact of specific antibiotic therapies on the prevalence of human host resistant bacteria ) will gather more information on these risk factors . as mentioned above , antibiotic overuse in humans probably plays a central role in the spread of esbl producers . in the context of this study it is noteworthy that germany has a relatively high level of fluoroquinolone use in the community . as to antibiotic use in hospitals , the overall quantity of antibiotic use in german icus is comparable to that of other countries . although there is important heterogeneity between icus , inappropriate antibiotic use is still common in germany ( as in many other countries ) , where infectious diseases as a stand - alone speciality and antibiotic stewardship programmes are still underdeveloped . compared to other european countries or highly publicised health threats , such as bioterrorism and swine flu , public awareness and political commitment to control multidrug - resistant microorganisms has been slow to rise in germany . only recently ( 2009 ) has a german reference centre for surveillance of gram - negative bacteria been established , which focuses , among other things , on detection of carbapenemase producing bacteria . a first outbreak of carbapenemase producing klebsiella pneumoniae in germany has recently been reported , probably linked to an index patient with previous healthcare contact in greece . the fact that two icus in 2008 reported carbapenem resistant k. pneumoniae in the current study raises the concern that these strains might already be more common in central europe than previously assumed since detection of these strains may be difficult with routine laboratory techniques . with regard to esbl - producers the growing community reservoir makes it unlikely that we will be able to control the spread by conventional measures targeted at nosocomial infection control . the promotion of prudent antibiotic use in the community and animal husbandry should therefore be a key priority . as to carbapenemase - producing enterobacteriaceae , early identification of these strains and aggressive infection control measures seem essential . examin ing novel decolonization strategies for gram - negative enterobacteriacae might be a further strategy worth evaluating . if we manage to enforce all these measures , we will hopefully be able to think positive again - even with regard to gram - negatives . 3gc : third generation cephalosporin ; esbl : extended - spectrum betalactamase ; mrsa : methicillin - resistant staphylococcus aureus . preparation of this commentary was supported in part by the 6th and 7th framework programme of the european community in the context of the projects ' changing behaviour of health care professionals and the general public towards a more prudent use of anti - microbial agents ' ( champ , contract sp5a - ct-2007 - 044317 ) and ' impact of specific antibiotic therapies on the prevalence of human host resistant bacteria ' ( acronym saturn , agreement fp7-health-2009-n241796 ) .

the increasing prevalence of multiresistant gram - negative bacteria of the enterobacteriaceae family in europe is a worrisome phenomenon . extended spectrum betalactamase - producing escherichia coli strains are widespread in the community and are frequently imported into the hospital . of even more concern is the spread of carbapenem - resistant strains of klebsiella spp . from regions where they are already endemic . antibiotic use is a main driver of antibiotic resistance , which again increases broad spectrum antibiotic use , resulting in a vicious circle that is difficult to interrupt . the present commentary highlights important findings of a surveillance study of antimicrobial use and resistance in german icus over 8 years with a focus on gram - negative resistance .

toxic epidermal necrolysis ( ten ) is a rare life threatening idiosyncratic mucocutaneous drug reaction characterized by widespread epidermal necrosis followed by epidermal detachment . drug - induced ten is the commonest cause and antiepileptics , antibiotics , nonsteroidal anti - inflammatory drugs ( nsaids ) and allopurinol are the most commonly implicated agents . here we report a case of ten which was induced by multiple drugs some of which have been rarely implicated in the causation of ten . a 42-year - old woman was referred to medical emergency with extensive epidermal detachment and ocular , genital , and oral lesions of 5 days duration leading to a diagnosis of toxic epidermal necrolysis ( ten ) . seven days prior to this admission , she had presented to a general practitioner with symptoms of vertigo , anxiety , and restlessness . although no provisional or differential diagnosis was found in the documents provided by the patient , she was prescribed antihistamines ( cinnarizine and dimenhydrinate ) , paracetamol / metoclopramide combination and multivitamins . after 8 - 12 hours , she developed fever , malaise , sore throat , and erythematous maculopapular rash in the perioral region . she discontinued all medications and was admitted to a general hospital where she was administered piperacillin / tazobactam and dexamethasone . it is unknown whether paracetamol or antihistamines were restarted in the hospital . her condition deteriorated and she was referred to our hospital . on presentation , she was conscious and afebrile ; her heart rate was 110/min and blood pressure was 190/80 mm hg . cutaneous examination showed involvement of about 70% total body surface area , with skin necrosis , tenderness , and a positive nikolsky 's sign . she had a history of hypertension and was on some antihypertensive medication ( unknown ) for a few years . test reports at three time - points she was administered intravenous fluids , antihypertensive treatment ( amlodipine 5 mg / day ) , injection hydrocortisone ( 50 mg iv twice daily ) , and continued on the same antibiotics . wound care was given by applying topical antiseptics on eroded areas along with nonadherent dressings . biochemical reports showed a decrease in blood urea ( from 68 mg / dl on admission to 57.6 mg / dl on the second day after admission ) and a decrease in serum creatinine ( from 1.1 meq / l on admission to 0.78 parenteral therapy was gradually replaced by oral drugs . on the fourth day , she developed herpetic lesions on the oral mucosa and for that tablet acyclovir was advised thrice a day along with chlorhexidine mouthwash , syrup mucaine gel ( antacid ) , and white petroleum jelly for lips . a tzanck smear , done on fifth day after admission , failed to show any giant cells or acantholytic cells ( acc ) [ table 1 ] . this test was done to rule out pemphigus . the patient showed steady improvement with the therapy given and was discharged , after 8 days , without any sequelae . this is most likely a case of drug - induced ten as other causes like upper respiratory tract infection , viral infection , or malignancy were not present . as this was an acute presentation , which occurred right after the drugs were administered , systemic lupus erythematosus ( sle ) and rheumatological disorders were ruled out . ten was diagnosed from the history and typical clinical features and biopsy was not needed . the prodrome was shorter ( 8 - 12 hrs ) than the usual 1 - 3 days . this might have been due to the use of multiple drugs causing an unknown interaction or more likely due to a previous exposure to any or all of these drugs . causality assessment was done for each of the drugs separately by the method of kramer et al . paracetamol and metoclopramide were categorized as probable ( score 5 ) and antihistamines and multivitamins as possible ( score 3 ) causal drugs for this adverse drug reaction . since this drug intake was uneventful for a long time , the chances of this medication causing this episode of ten are minimal . there have been two case reports of ten caused by paracetamol and a case control study showing increased risk of sjs or ten with paracetamol use . there have been no case reports of ten due to a combination of both the drugs , nor have there been any reports regarding ten being caused by antihistamines or multivitamins . however , the probability of other drugs being responsible could not be ruled out completely . ten in this patient was probably caused by paracetamol and metoclopramide , but a possibility of an interaction as well as the role of other drugs can not be ruled out .

toxic epidermal necrolysis ( ten ) and steven - johnson syndrome ( sjs ) are serious disorders commonly caused as idiosyncratic reactions to drugs , the most common ones being oxicams , anticonvulsants , allopurinol , and sulfonamides . we present a case of ten in a patient who developed the lesions after ingesting multiple drugs including paracetamol , metoclopramide , antihistamines , and multivitamins . these drugs have rarely been implicated in this disorder . the suspected drugs in this case were paracetamol and metoclopramide . however , the role of other drugs could not be ruled out definitely . the patient was managed with antibiotics , corticosteroids , and parenteral fluids and recovered well .

pycnodysostosis was first reported in 1923 by montanari , and he called it as atypical achondroplasia . the main characteristics are short stature , cranial dysplasia , increased bone density and fragility . other clinical features include open cranial sutures , hypoplastic paranasal sinuses , dysplastic lateral clavicle , shortened terminal phalanges , proptosis , blue sclera and frontal or occipital bossing . oral manifestations include obtuse gonial angle , grooved palate , anterior cross - bite , malpositioned teeth associated with increased incidence of dental caries and periodontitis , hypoplastic maxilla , receded chin , delayed eruption of permanent teeth , delayed exfoliation of deciduous teeth and hypoplasia of root - obliterated pulp spaces . a 47-year - old man reported to the department with a complaint of deformed lower jaw for the past 10 years . history revealed that the patient had undergone extraction of his teeth that was uneventful , following which there was fracture of jaw at the extraction site . subsequently , there was frequent exfoliation of teeth with fracture at different sites in the lower jaw . his medical history revealed that he had multiple fractures of the upper and lower limbs and a history of diabetes mellitus and hypertension . general examination revealed that the patient 's height was 127 cm and weight was around 49 kg , with proportionate dwarfism [ figure 1 ] . the hand and feet had short digits with overlying cutaneous wrinkles that tapered off with large overriding nails [ figure 2 ] . a 47-year - old man , 127 cm in height shortened digits and cutaneous wrinkles on extraoral examination , there was facial dysmorphia with prominent forehead ( frontal bossing ) , proptosis , beaked nose , deep nasolabial skin folds , micrognathia and obtuse mandibular angle on the right side [ figure 3 ] . mild proptosis and facial dysmorphia intraoral examination revealed multiple clinically missing teeth , chronic periodontitis , narrow and grooved palate , no features of enamel hypoplasia , malposed teeth [ figure 4 ] and evidence of sequestrum in relation to the tooth 46 [ figure 5 ] . based on the history and clinical presentation , a provisional diagnosis of a bone dysplasia , probably pycnodysostosis , was made and differential diagnosis of cleidocranial dysplasia and osteopetrosis was included . grooved palate and clinically missing maxillary anteriors sequestrum in relation to tooth 46 laboratory findings were within normal limits , including hemoglobin conc . , differential count calcium , phosphate , alkaline and acid phophatase level . computed tomography of the bone window of the skull showed open sutures and fontenalles with nonaerated paranasal sinuses , flattening of the mandibular angle on the right side with evidence of fracture and loss of bone architecture on the left side involving the ramus of the body of the mandible and hypoplastic maxilla [ figures 68 ] . orthopantomograph revealed generalized bone loss , multiple missing teeth and obtuse gonial angle with loss of bone structure on the left side of the mandible involving the body and ramus [ figure 9 ] . 3d - reconstructed computed tomography showing open sutures and fontanelles 3d - reconstructed computed tomography showing fractured body of the mandible and obtuse gonial angle 3d - reconstructed computed tomography showing fractured body of the mandible opg showing generalized bone loss , multiple missing teeth , and obtuse gonial angle lateral skull showing open fontanelles with nonaerated paranasal sinus and fractured body of the mandible the patient was surgically managed for osteomyelitis by removal of the sequestrum and curettage , and further mandibular reconstruction was performed . the patient presents with characteristic facies , dwarfism , beaked nose , prominent head and generalized increase in the density of bones not sufficient to obliterate medullary canals or cranial orifices . frequent fractures due to trauma can aid in diagnosing this condition . in our case , the patient was negligent about the condition and reported to us with a fractured jaw . intraoral clinical presentation included altered pattern of exfoliation of deciduous teeth and eruption of the permanent dentition . the disease is diagnosed at an early age , wherein the main reasons for consultation are generally short stature and open anterior fontanelles . in later stages , consultation is usually for fracture resulting from slight or moderate trauma , given the severe bone fragility . symptoms include dental abnormalities , with hypoplasia of the enamel , obliterated pulp chambers and hypercementosis . protrusion of the incisors with anterior open bite may be found , and dental crowding associated with extensive caries and periodontitis is frequent . in our case , multiple clinically missing teeth , chronic periodontitis , narrow and grooved palate , features of enamel hypoplasia and malposed teeth these conditions cause the premature loss of dentition that may already be complete by the fourth decade of life , similar to our patients . our patient showed evidence of sequestrum in relation to the tooth 46 as a result of osteomyelitis . orofacial infections are commonly encountered by the dentist and there are wide ranges of modalities that can be implemented in managing them . this is caused by the increased bone volume of the sella turcica that , on compressing the pituitary gland , causes its hypoplasia and a deficient production of the growth hormone . our patient 's height was 127 cm and weight was around 49 kg , with proportionate dwarfism . the hand and feet had short digits with overlying cutaneous wrinkles that tapered off with large overriding nails . diagnosis of pycnodysostosis is based on the clinical presentation , and medical treatment for the condition is symptomatic . the differential diagnosis of pycnodysostosis includes osteopetrosis , acroosteolysis , mandibular acral dysplasia and cleidocranial dysplasia . unlike osteoporosis , hepatosplenomegaly and difference between pycnodysostosis and cleidocranial dysplasia is that dense and brittle bones are found in pycnodysostosis but not in cleidocranial dysplasia . new treatment modalities like gene therapy and bone marrow transplant can be expected to be the mainstay in the future , now that the abnormal expression of cathepsin k and the gene defect has been located . now - a - days , symptomatic treatment is provided for patients with pycnodysostosis , with the main intention of prevention of fractures . as pycnodysostosis is associated with inappropriate bone remodeling , it can pose a challenge for a dental health care professional to provide treatment as there can be serious complications , such as osteomyelitis arising as a result of dental infections . tooth extractions in these patients demand certain special care , such as carrying out the surgery atraumatically with proper asepsis . oral hygiene practices and frequent visits to

pycnodysostosis , a sclerosing bone dysplasia , is a rare autosomal recessive disorder with an estimated prevalence rate of one in one million . patients with pycnodyostosis usually have normal intelligence , sexual development and life span . this condition is characterized by increased bone density and fragility along with oral manifestations like malposition teeth , hypoplastic maxilla , receded chin and delayed eruption of permanent teeth with discharging sinuses in the jaws because of poor blood supply . this is one such rare case report of a 47-year - old patient presenting with a complaint of fractured jaw and reviewing the clinical and radiographic characteristics of pycnodysostosis .

myositis is an acute , sub - acute , or chronic inflammation of skeletal muscles . a previously healthy 12-year - old boy was admitted to driscoll children s hospital in corpus christi , tx , usa , with a 9 day history of fever , and 7 day history of pain and weakness in the lower extremities . three days prior to admission , he was seen by a nurse practitioner and diagnosed with otitis media and pharyngitis . his temperature was 36.8c and the physical examination was significant for antalgic , broad - based gait and tender gastrocnemius soleus muscles . sensation and deep tendon reflexes were normal with absent romberg s and babinski s sign . the white blood cell count was 8300 cells per cubic mm with 60% neutrophils and 40% lymphocytes , hemoglobin was 13.5 g / dl , and the platelet count was 172,000 cells per cubic mm . creatinine phosphokinase ( cpk ) was elevated at 2394 /l , aspartate aminotransferase ( ast ) was 222 /l and , alanine aminotransferase ( alt ) was 143 /l . a diagnosis of myositis was made and the patient was given analgesics and oral prednisone as dermatomyositis and polymyositis were amongst the differential diagnosis . rhabdomyolysis was considered in the differential diagnosis and the patient was started on one and a half maintenance intravenous fluids . our patient s clinical course was not severe and did not develop the complications usually associated with rhabdomyolysis . on day 2 of his hospital stay , he was able to stand up with support and cpk levels came down to 1713 cerebrospinal fluid analysis showed a protein concentration of 62 mg / dl and white blood cell count of 4 . a multiplex respiratory viral pcr was positive for rhinovirus and negative for influenza a and b , parainfluenza , metapneumovirus and adenovirus . arboviral titers for west nile virus was negative and so was the rocky mountain spotted fever titers . on the day 3 of his hospitalization , serum was evaluated by use of an indirect immunofluorescence antibody ( ifa ) test kit for immunoglobulin g ( igg ) to rickettsia typhi and rickettsia rickettsii ( focus diagnostics , cypress , ca , usa ) . the complete recovery of the patient took about 48 hours which included 100 mg of doxycycline given twice daily by mouth . he was discharged home on doxycycline to complete a 10 day course and prednisone was discontinued . myositis can be caused by strenuous activity , muscle trauma , illicit drug injections , genetic diseases , connective tissue disorders , diabetes and infections . infectious myositis can involve single or multiple muscle groups in the limbs with the proximal muscles being predominantly affected the exception being trichinosis which commonly involves orbital muscles . signs and symptoms of myositis include fever , malaise , muscle swelling , weakness and pain that is worse with movement . with this presentation , other diseases that should be considered are polymyositis , dermatomyositis , rhabdomyolysis , guillain barre syndrome , cellulitis , osteomyelitis , deep vein thrombosis , metabolic and other inflammatory myopathies ( table 1 ) . rhabdomyolysis was considered as one of the top differential diagnosis for our patient and given intravenous fluids . it presents 1 - 2 weeks after the bite of an infected flea although a history of such exposure is rarely elicited . . the rash may not be present in 50% of the patients with murine typhus . doxycycline is the treatment of choice , which our patient was prescribed at the time of discharge . it has been known that murine typhus resolves even without treatment . to our knowledge , this is the first pediatric case reported of murine typhus associated with myositis and paraparesis . physicians practicing in or near rickettsia typhi - endemic areas need to consider this in the differential diagnosis of children with evidence of myositis . making this diagnosis may have a significant impact on the course of the disease because of the availability of antimicrobial therapy against rickettsial organism .

a 12-year - old boy presented with fever , lower extremity pain and weakness . examination revealed paraparesis , thigh and calf tenderness . labs showed high creatinine phosphokinase and rickettsia typhi titers . this case illustrates endemic typhus should be considered in the differential diagnosis of myositis especially in areas with high prevalence of the disease . to our knowledge , this is the first reported case of myositis and paraparesis associated with a case of murine typhus .

all chemicals and reagents were of high performance liquid chromatography ( hplc ) or analytical grade . pyrrole ( 99 % ) , thiophene ( 99 % ) , and tetrabutylammonium perchlorate ( 98 % ) were purchased from sigma - aldrich ( schnelldorf , germany ) . medical steel wires ( ni cr , = 750 m ) were purchased from b. braun surgical s.a . polypyrrole ( ppy ) and polythiophene ( pth ) coatings were synthesized by electropolymerization with a linear sweep voltammetry technique . the three - electrode cell was filled with electrolyte solution consisting of 100 mm pyrrole / thiophene ( fig . 4 ) and 250 mm tetrabutylammonium perchlorate in acetonitrile.fig . 4chemical structures of ppy ( a ) and pth ( b ) spme fiber coatings chemical structures of ppy ( a ) and pth ( b ) spme fiber coatings polymerization was performed using medical steel wires as working electrodes . an ag / ag electrode was applied as a reference electrode , with platinum net bent into a cylinder as a counterelectrode . for ppy , a potential range from 0.2 to + 2.5 v and seven scans second , an additional 14 scans in the higher potential range from 0.2 to 2.7 v were used ( fig . 5electropolymerization of pyrrole ( a ) and thiophene ( b ) electropolymerization of pyrrole ( a ) and thiophene ( b ) the morphologies of the polypyrrole and polythiophene fibers were investigated using a scanning electron microscope ( leo 1430vp ; carl zeiss smt , oberkochen , germany ) coupled with a backscattered electron ( bse ) detector . small angle x - ray scattering ( saxs ) data were collected using a nanostar system ( bruker axs gmbh , karlsruhe , germany ) with pinhole collimation and a two - dimensional hi star detector with resolution of 1,024 1,024 pixels , mounted on an x - ray tube with a copper anode and equipped with crossed gbel focusing mirrors . samples were mounted between two mica windows in sample holders with thickness of 1 or 2 mm . the sample - to - detector distance was 650 mm , and the exposure time for a single frame was 10,000 s. each sample was measured three times , and the obtained data were averaged . the saxs data were recorded within the scattering vector range of 0.15 nm < s < 3.5 nm ( where s = 4sin/ , 2 is the scattering angle , and is the x - ray wavelength ) . the saxs data were corrected for the detector response and normalized to the intensity of the incident beam , and the background scattering ( empty holder ) was subtracted using the saxs_nt v4.1 program package ( bruker axs gmbh , karlsruhe , germany ) . electropolymerization was performed using a homemade electrochemical cell coupled with a high performance potentiostat galvanostat ( pgstat128n series ; metrohm - autolab b.v . , all chemicals and reagents were of high performance liquid chromatography ( hplc ) or analytical grade . pyrrole ( 99 % ) , thiophene ( 99 % ) , and tetrabutylammonium perchlorate ( 98 % ) were purchased from sigma - aldrich ( schnelldorf , germany ) . medical steel wires ( ni cr , = 750 m ) were purchased from b. braun surgical s.a . polypyrrole ( ppy ) and polythiophene ( pth ) coatings were synthesized by electropolymerization with a linear sweep voltammetry technique . the three - electrode cell was filled with electrolyte solution consisting of 100 mm pyrrole / thiophene ( fig . 4 ) and 250 mm tetrabutylammonium perchlorate in acetonitrile.fig . 4chemical structures of ppy ( a ) and pth ( b ) spme fiber coatings chemical structures of ppy ( a ) and pth ( b ) spme fiber coatings polymerization was performed using medical steel wires as working electrodes . an ag / ag electrode was applied as a reference electrode , with platinum net bent into a cylinder as a counterelectrode . for ppy , a potential range from 0.2 to + 2.5 v and seven scans second , an additional 14 scans in the higher potential range from 0.2 to 2.7 v were used ( fig . 5electropolymerization of pyrrole ( a ) and thiophene ( b ) electropolymerization of pyrrole ( a ) and thiophene ( b ) the morphologies of the polypyrrole and polythiophene fibers were investigated using a scanning electron microscope ( leo 1430vp ; carl zeiss smt , oberkochen , germany ) coupled with a backscattered electron ( bse ) detector . small angle x - ray scattering ( saxs ) data were collected using a nanostar system ( bruker axs gmbh , karlsruhe , germany ) with pinhole collimation and a two - dimensional hi star detector with resolution of 1,024 1,024 pixels , mounted on an x - ray tube with a copper anode and equipped with crossed gbel focusing mirrors . samples were mounted between two mica windows in sample holders with thickness of 1 or 2 mm . the sample - to - detector distance was 650 mm , and the exposure time for a single frame was 10,000 s. each sample was measured three times , and the obtained data were averaged . the saxs data were recorded within the scattering vector range of 0.15 nm < s < 3.5 nm ( where s = 4sin/ , 2 is the scattering angle , and is the x - ray wavelength ) . the saxs data were corrected for the detector response and normalized to the intensity of the incident beam , and the background scattering ( empty holder ) was subtracted using the saxs_nt v4.1 program package ( bruker axs gmbh , karlsruhe , germany ) .

abstractpolymeric polypyrrole and polythiophene solid phase microextraction ( spme ) coatings were prepared using electropolymerization with a linear sweep voltammetry technique . physicochemical properties were measured using different methods , in particular small angle x - ray scattering and scanning electron microscopy . by using innovative approaches for pore size measurement , we were able to calculate a maximum of the pore size range from 80 to 90 nm . additionally , film thicknesses measured from 90 to 150 m . using scanning electron microscopy , we describe the characteristics of polymer growth on the support surface.graphical abstract

supernumerary teeth are a relatively frequent disorder of odontogenesis characterized by an excess number of teeth , with mesiodens being the most frequent.1 the term of mesiodens is used to refer to an unerupted supernumerary tooth in the midline of maxilla , between the central incisors.2,3 the incidence of mesiodens among caucasians is 0.153% for permanent teeth,4,5 and is found two times more frequently in males , compared to females.46 a mesiodens can manifest bilaterally,46 or in an inverted ( upward ) position.4,5 the frequency of inverted mesiodens constitutes to approximately 967% of all reported cases.711 to date , a total of 278 single inverted mesiodentes have been reported ( table 1 ) . the mesiodens may present a rudimentary morphology with a cone - shaped crown , generally smaller in size than the adjacent normal teeth . it may also be found to mimic a natural tooth shape . the root is often totally formed , and may be curved or globular.12 mesiodens is frequently associated with several craniofacial disturbances , including cleft lip and palate13 and cleidocranial dysostosis;14 and to a lesser extent with gardner s syndrome or chondorectodermal dysplasia.15 only 25% of all mesiodentes spontaneously erupt into the oral cavity.6 in general , they remain impacted and asymptomatic ; and are commonly discovered during routine radiographic examination.16 the most common complications of mesiodentes are the delay or prevention of eruption ( 2652% ) and displacement / rotation ( 2860% ) of maxillary permanent incisors . relatively less common complications include crowding , diastema , dilaceration of permanent teeth , cyst formation and eruption into the nasal cavity.6 this case reports presents radiographic features of a unique occurrence of two impacted , inverted mesiodentes in a child patient ; and the 24-month radiographic and clinical follow - up after surgical removal . an 8-year - old boy was referred to the department of pediatric dentistry with a chief complaint of crowding in the maxillary anterior region . being born to non - consanguineous parents , the patient s health status was excellent . panoramic radiograph revealed the presence of two supernumerary teeth , located between the roots of the permanent central incisors ( figure 2 ) . occlusal and periapical radiographs of the region showed that these two teeth were inverted mesiodentes ( figure 3 ) . apparently , the roots of the permanent central incisors had migrated distally , owing to the presence of the supernumerary teeth . the lateral cephalogram showed that the mesiodentes were located buccally to the apices of the central incisors ( figure 4 ) . the treatment plan consisted of surgical removal the inverted mesiodentes . the teeth were removed under general anaesthesia without any complication ( figure 5 ) . following removal of sutures one week later , the patient was scheduled for bimonthly recalls . at the second - month recall , the extraction cavities were completely filled with bone ( figure 6 ) and the permanent central incisors responded favorably to thermal and electrical pulp tests . removal of the inverted mesiodentes had slightly aided to self - correction of the axial rotations of the central incisors . although mesiodentes are often located palatally,2 the present case demonstrates the possibility of their occurrence in a more vestibular location . in addition to routine ortopantomography or periapical / occlusal radiographs which can rapidly solve the initial diagnostic phase of mesiodentes , further techniques may be required to better define their position in relation to the adjacent teeth and anatomic structures . in the present case , the exact route ( i.e. , buccal / palatal ) for surgical removal of the inverted mesiodentes undoubtedly , greater detail concerning their location could be obtained through computerized axial tomography ( cat),17 which also can help evaluate their relationship with the adjacent tooth structures . in this regard , periapical and occlusal radiographs were used in conjunction with the lateral cephalogram , since cat was not available in our centre . extraction is not the only treatment choice for impacted mesiodentes . if the mesiodens remains in place without symptoms and does not adversely affect the adjacent teeth , it may be left in place and observed periodically.16 in the present case , surgical removal of the mesiodentes was judged necessary , since these teeth had caused axial rotation of the permanent central incisors . there are no precise indications concerning the ideal time for surgical removal of impacted mesiodens . according to atwan,18 a mesiodens can be best removed when the permanent central incisors start to erupt , but this may not be always possible . contrary , primosh19 discourages early extraction of mesiodentes due to the risk of iatrogenic damage to the developing adjacent permanent teeth . in the present case , the timing for surgical removal of the inverted mesiodentes was judged to be suitable , since both maxillary central incisors had totally erupted , showing complete root formation and advanced apical closure . the case presented herein was considered to be extremely rare , since inversion of two mesiodentes in a single patient has only been reported once20 ( table 1 ) . the clinical significance of the inversion is questionable , since both mesiodentes remained intact ; i.e. , did not erupt ectopically . however , the occurrence of two mesiodentes was definitely regarded as a contributory factor to the crowding in the anterior maxillary region , as evidenced by the deviation in the vertical axis of both central incisor teeth . fortunately , the post - surgical phase was uneventful , and did not affect commencement of fixed orthodontic therapy after a relatively short follow - up period .

this report presents an extremely rare occurrence of two inverted mesiodentes in a child patient . extraction of both mesiodentes was indicated , owing to the axial rotation of the permanent central incisors caused by these impacted supernumerary teeth . radiographic evidence of complete healing was observed 24 months following surgical removal of the inverted mesiodentes .

juvenile ossifying fibroma ( jof ) is an actively growing aggressive lesion containing cells rich in the fibrous stroma containing cellular osteoid , trabeculae of woven bone without osteoblastic lining , with or without clusters of giant cells . el - mofty first identified the two histopathological variants of jof : trabecular and psammomatoid . rare cases of mandibular jof have been reported . clinical examination reveals it as asymptomatic slow growing swelling causing facial asymmetry , extending to a substantial size and behaving as an aggressive lesion . it has higher male predominance with the mean age of occurrence ranging from 2 to 15 years . the radiographic features show unilocular or multilocular radiolucency with well - delineated borders and few opacification in the center . the suggestive features of the aggressive variant are the presence of the perforation , cortical thinning , tooth displacement , and root resorption . the advanced imaging studies show more invasive and destructive features apart from conventional radiographic features . histopathologically both the entities are nonencapsulated , well demarcated from the surrounding bone consisting of neoplastic cellular stroma formed by spindle shape fibroblast . in contrast , the psammomatoid pattern forms concentrically laminated ossicles that vary in shape and having peripheral eosinophilic osteoid rims with basophilic centers typically . the overall clinical features , histopathological features , and advanced imaging were indispensable to provide the diagnosis as jof . a nonaggressive form of this lesion is managed by curettage and local excision whereas aggressive form is managed by complete surgical excision , en - bloc resection or hemimaxillectomy to prevent recurrences . reported here is a case of a trabecular variant of jof in a 17-year - old female patient . a 17-year - old female represented with a chief complaint of pain and slowly progressive swelling of the left side of face along with reduced mouth opening for the last 2 months . initially , the swelling was smaller in size and increased progressively to attain final size causing facial asymmetry with reduced mouth opening . extraoral examination revealed an ill - defined , diffuse , and bony hard swelling of approximately measuring about 6 cm 4 cm on the left side of the face , extending superior - inferiorly from left zygomatic arch till angle of the mandible and anteroposteriorly from nasolabial fold till tragus of the left ear with normal overlying skin [ figure 1a and b ] intraorally , swelling was solitary , slightly tender with the expansion of buccal and lingual cortical plates with no pathological changes to the overlying mucosa . there was reduce mouth opening measuring about 15 mm in the greatest dimension [ figure 2a ] . orthopantomogram revealed a large well - bordered , well - defined unilocular , circular , mixed lesion ( calcification specks ) approximately , 2 cm in size involving the ramus , coronoid , and angle of the mandible [ figure 3 ] posteroanterior view of mandible showed a well - defined , mixed lesion involving the coronoid process , ramus and the angle of the left side of the mandible [ figure 4 ] . axial and sagittal view of computed tomography confirmed the routine radiographic features and further showed a heterogeneously enhancing mixed lytic - sclerotic lesion with a sclerotic rim extending into adjacent soft tissue measuring 5.12 cm 3.83 cm with exterior and superior extension into the left infratemporal fossa and medial extension up to pterygoid muscle [ figure 5a and b ] . biopsy was taken from the involved site , and the h and e section showed the presence of trabeculae of fibrillar osteoid and woven bone . in few areas , multinucleated giant cells were also noted . based on clinical , radiographic , advanced imaging studies and histopathological report provided the diagnosis of the trabecular variant of jof affecting left mandible with differential diagnosis of cement - ossifying fibroma , osteoblastoma , and osteogenic sarcoma . the lesion was excised by segmental resection using transmandibular approach and reconstruction was done [ figure 7a and b ] . postoperative healing was uneventful ; mouth opening was increased to the value of 30 mm [ figure 2b ] . postoperatively , the esthetics , oral function of the patient such as speaking , and chewing were intact and normal [ figure 8a and b ] . the patient is still under regular follow - up as the lesion has high recurrence rate . ( a and b ) preoperative extraoral view of the patient showing swelling on the left side of face ( a ) preoperative mouth opening , ( b ) postoperative mouth opening preoperative panoramic view of the patient preoperative posteroanterior view of the patient ( a ) axial view of computed tomography , ( b ) sagittal view of computed tomography histopathological section ( a ) postoperative panoremic view of the patient , ( b ) postoperative posteroanterior view of the patient ( a and b ) postoperative extraoral view of the patient with intact facial symmetry jof mostly strikes children with slightly male predominance with a mean age of 11 years . clinically , it is asymptomatic , however rarely it is aggressive and symptomatic . in the present case , both maxilla and mandible are affected with more prevalence for maxilla . in the present case , the duration of swelling noted was 2 months . the characteristic radiographic features reveal a central opacification with well - defined unilocular to multilocular pattern giving it a ground glass appearance . the 50% of cases of jof are recorded in multiple sinuses with few occurring in the single sinus . the duration , clinical and radiographic features and extension of the lesion into the adjacent soft tissue favors the aggressive type of jof as previously reported . the features like resorption and perforation of cortices , tooth displacement and aggressive growth of the lesion favor the diagnosis as an aggressive variety of jof . in this case , except for tooth resorption all other features like perforation of the cortices and extension into the surrounding tissue was noted . because of the high recurrence rate , there is no follow - up protocol mentioned in the literature and immediate reconstruction is not advised . resection is considered where there is recurrence , invasion into surrounding tissues , or when preserving the inferior border is not feasible . jof is an uncommon clinical entity , because of its aggressiveness and high recurrence rate it is necessary to arrive at an early diagnosis , applying the appropriate clinical , diagnostic approaches , and appropriate treatment for effective results and long - standing follow - up . 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 there are no conflicts of interest . 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

one of the rarest entities of fibro - osseous lesions that arise within the craniofacial bones is juvenile ossifying fibroma ( jof ) . it is an intraosseous expansile lesion of the jaw that imitate odontogenic lesions . who has described two distinct histopathological variants of jof ; trabecular and psammomatoid . histologically , they are characterized by the presence of fibrous connective tissue stroma along with osteoblastic and osteoclastic cells . clinical , characteristics show an early age of onset , typical histological patterns , high rate of aggressive behavior and recurrence . this article presents a rare clinical case of the trabecular variant of jof , its clinical , radiological , histological , and treatment aspects .

the first case was described by cruveilhier in 1835 and termed as hamartoma . till date , less than 200 cases have been described with synonyms of brunner gland adenoma , brunner gland hamartoma ( bgh ) or brunneroma . there is no sex predilection and patients present in the fifth to sixth decades of life . they are usually seen as incidental findings on imaging with duodenal bulb as the most common location . however , it may present with a variety of clinical presentations depending on location and tumor size such as abdominal pain , dyspepsia , nausea , vomiting , upper gastrointestinal ( gi ) bleeding and occasionally obstructive symptoms with associated chronic pancreatitis . a 42-year - old man presented with history of pain epigastrium since one year and recurrent vomiting within half an hour of taking food since one month . a biopsy of duodenum was performed by the same procedure which was diagnosed as well differentiated adenocarcinoma . the patient was further subjected to abdominal computed tomography ( ct ) which showed circumferential thickening of the second part of the duodenum ( 15.2 mm maximum thickness ) abutting head of pancreas with loss of fat planes . laboratory investigations showed normal blood counts , liver , and kidney function tests but elevated serum amylase and lipase levels . this patient underwent radical surgery ( whipple s procedure ) at our institute with clinical diagnosis of carcinoma periampullary region and the specimen was sent for histopathological examination . gross examination of the specimen revealed circumferential thickening of the second part of the duodenum and bulky head of the pancreas . cut section revealed a diffuse grey white area measuring 4x4 cm with few cystic and hemorrhagic areas . six lymph nodes were isolated from the specimen and a single celiac node was examined which was received separately . microscopy showed a tumor composed of lobules of brunner s glands with a dilatation of few glands . these lobules were separated by fine fibrous septa and constituted > 50% of the thickness of the duodenal wall ( figures 1 , 2 and 3 ) . hyperplasia of brunner s glands spanning > 50% of wall thickness ( h&e stain , 4x ) lack of features of anaplasia in proliferating brunner s glands ( h&e stain , 20x ) smooth muscle actin stain highlighting the intervening smooth muscles ( ihc , 10x ) brunner glands are branched acinotubular glands with submucosal location , found exclusively between the pyloric ring and the papillae of vater . their major role is the protection of duodenal epithelium from acidic gastric chyme by secretion of neutralizing alkaline fluid composed of viscous mucin . the pathogenesis of bgh has been linked to excessive local irritation from acidic gastric chyme , vagal stimuli , or unidentified antral hormones . hence , it is associated with conditions related to gastric hyperacidity and changes in gastric emptying such as esophagitis , hiatus hernia , and gastric - peptic ulcer . the association of this tumor with uremia and chronic pancreatitis has also been reported . in a study of patients undergoing pancreaticoduodenectomy , 76% of the patients had diffuse hyperplasia of brunner s gland , raising the question whether this is an adaptation to pancreatic insufficiency . an alternate suggestion for this hyperplasia is that these glands proliferate in reaction to inflammationas evidenced by presence of lymphocytes in the lesion . support to this hypothesis is not rationalized as lymphocytes may be present in normal submucosa of the gastrointestinal tract . in a study on the role of helicobacter pylori infection in the pathogenesis of bgh , 5 of 7 cases with bgh had concurrent helicobacter pylori infection . a definite relationship has not yet been proven because of the high prevalence of helicobacter pylori and rarity of this lesion in the general population . initially , the hyperplasia of brunner s gland can be focal , localized , and solitary usually involving a lobule . however , if stimulus persists then the lesion enlarges involving multiple lobules leading to formation of diffuse and annular lesion infiltrating into the wall and circumference of the duodenum . endoscopically these can be nodular , polypoid or diffuse with thickening of the duodenal wall . due to this presentation , these lesions can be misdiagnosed as gastrointestinal stromal tumor ( gist ) , lymphoma , carcinoid , peutz jeghers polyp , prolapsed pyloric mucosa , or aberrant pancreatic tissue . although surface biopsy of this lesion can be misleading as in our case , a deeper biopsy will be more diagnostic . microscopic diagnosis of hyperplasia requires presence of lobules of brunner s glands within the submucosa in at least 50% of the length of the biopsy showing no features of dysplasia . adenoma has been used for lesions > 1 cm and the term hamartoma if it contains mesenchymal elements also . conflicts regarding this terminology exist as some authors suggest that mere change in size indicate progression of hyperplasia and do not imply neoplastic behavior . ductal malignancy arising from brunner gland adenoma has been reported in one case which is a very rare circumstance ( 0.5% ) . brunner gland adenoma in the duodenal wall with a focus of atypical cells supported by mib-1 and p53 positivity has also been reported . the treatment of bgh is done with surgical or endoscopic removal in symptomatic patients taking into account the size and gross appearance . although endoscopic techniques of removal are more helpful , this procedure can be limited by difficult anatomical sites . recurrence in such a lesion is not known . even though diagnosis of bgh can be made by various imaging studies , in our case hence , these possibilities should be kept in mind while dealing with duodenal masses .

brunner s gland hamartoma is a rare benign tumour of the duodenum . it was first described by cruveilhier in 1835 . presently around 200 cases have been reported in literature . no sex predilection is seen . patients usually present in the fifth to sixth decades of life . they may be clinically silent or may present with variable symptoms and occasionally obstructive symptoms and chronic pancreatitis . endoscopic presentation can be nodular , polypoid or diffuse glandular proliferation with thickening of duodenal wall and hence can be misdiagnosed as malignancy . we describe a case of duodenal tumor reported outside ( on biopsy ) as well differentiated adenocarcinoma which out as brunner gland hamartoma upon complete resection . brunner gland hamartoma may sometimes have a very unusual presentation . extensive pre - operative evaluation is necessity to avoid radical surgical procedure .

metastasis of transitional cell carcinoma ( tcc ) of the renal pelvis to the eye is a very rare event and has only been reported in one case in the literature . once diagnosed , these cases usually show widespread metastases and carry a very poor prognosis . we present a case report of a male patient with unilateral choroidal metastasis secondary to tcc of the renal pelvis . review of the relevant literature will be addressed to highlight the involvement of the choroid in cases of metastatic disease , the unusual presentation of tcc , diagnostic methods , and the various therapeutic modalities used to treat these cases . a 61-year - old male , who has a history of congestive heart failure , is a nonsmoker , and has no significant family history of malignancies , presented to the ophthalmology clinic with sudden - onset loss of vision of the left eye associated with intermittent left eye pain , relieved by analgesics . ophthalmic evaluation revealed a visual acuity of 20/40 in the right eye and 20/400 in the left eye . funduscopic examination of the left eye showed an amelanotic macular choroidal lesion associated with inferior exudative retinal detachment ( fig 1a ) . a b - scan ocular ultrasound showed a dome - shaped , 2.8-mm choroid thickening with variable internal reflectivity , associated with inferior exudative retinal detachment ( fig 1b ) . fluorescein fundus angiography revealed early choroid blockage ( fig 1c ) and late leakage associated with hot spots at tumor margin . all of these clinical features are suggestive of the clinical diagnosis of left choroidal metastasis . physical examination of the patient also revealed multiple suspicious chest wall nodules . in search for a primary malignancy , imaging studies were done , which revealed multiple pulmonary and liver metastasis , subcutaneous metastasis , multiple lymphadenopathies , and left renal upper pole mass ( fig 2 ) . ct - guided left renal mass biopsy and ultrasound - guided biopsy of the left chest wall subcutaneous mass were performed . they are negative for ck20 , pax2 , ttf-1 , synaphopysin , and s100 ( fig 3 ) . external beam radiation therapy ( ebrt ) is a common mode of treatment and will be considered if there is no improvement of visual symptoms with chemotherapy . choroidal metastasis is the most common intraocular malignancy , due to its rich vascular supply and high oxygen concentration [ 4 , 5 ] . less common primary cancers include the thyroid , prostate , kidney , testicles , pancreas , ovary , and liver . rarely is it seen in cases of tcc , as there have only been 5 previous reports in the literature involving choroidal metastasis , 3 from tumors involving the bladder , and 2 from other parts of the urinary tract . choroidal metastasis is commonly associated with disseminated disease and , unfortunately , carries a very poor prognosis . tcc usually metastasizes to the regional lymph nodes ( 78% ) , liver ( 38% ) , lung ( 36% ) , bone ( 27% ) , adrenal gland ( 21% ) , and intestine ( 13% ) . the heart , brain , kidney , spleen , pancreas , meninges , uterus , ovaries , prostate , and testes have been reported as unusual sites of presentation of tcc . other less common clinical presentations include flashes ( 5% ) and floaters ( 5% ) . diagnosis is based on clinical findings , which are typically seen as yellow , plateau - shaped lesions associated with subretinal detachment . a - scan and b - scan ultrasonography show irregular internal reflectivity and a solid plateau - shaped mass , respectively . fluorescein fundus angiography typically shows mottled hypofluorescence in early stage and leakage in late stage . the treatment of choroidal metastasis should be individually tailored , taking into consideration the patient 's systemic state , disease activity , and the presence of visual symptoms . its aim is palliation , to improve the quality of life of the patient and to restore or maintain visual function . local therapeutic regimens include ebrt , plaque radiotherapy , and complete enucleation for painful , blind eyes [ 13 , 14 ] . ebrt delivered at a dose of 40 gy over 20 fractions was effective in improving and maintaining vision in patients with choroidal metastasis [ 15 , 16 ] . in cases of widespread metastases , systemic chemotherapy alone or in combination with local therapy is an accepted therapeutic option . in patients with choroidal metastasis alone , local therapy has been reported to be safe , maintaining visual functions without the systemic side effects of chemotherapy . choroidal metastasis related to tcc of the renal pelvis is an extremely rare event and is associated with widespread disease and a very poor prognosis . systemic chemotherapy with carboplatin and gemcitabine is a palliative treatment option for this patient . ebrt offers local palliation and will be considered according to the patient 's visual response to chemotherapy .

metastases of transitional cell carcinoma ( tcc ) of the renal pelvis to the eye is a very rare event , as only one previous case has been described in the literature . we present a patient with choroidal metastasis secondary to tcc of the renal pelvis , with a review of the relevant literature . a 61-year - old male presented with sudden - onset loss of vision of the left eye . upon evaluation and assessment , the patient was found to have unilateral choroidal metastasis secondary to tcc of the renal pelvis . he is currently on palliative chemotherapy with carboplatin and gemcitabine . external beam radiation therapy is a common mode of treatment and will be considered accordingly .

pasteurella multocida was first described by revolee in 1877 and further characterized by pasteur in 1880 . pasteurella species usually have low virulence in humans , but serious manifestations sometimes occur and can lead to bacteremia . we present a patient who developed septic shock as a result of p. multocida bacteremia , presumptively from an intra - abdominal infection . a 52-year - old african american female was brought to the emergency department for generalized abdominal discomfort , altered mental status , diarrhea , fevers and chills for 4 - 5 days . her past medical history was significant for alcohol abuse and alcohol - related liver cirrhosis . in the emergency department , vital signs were as follows : blood pressure of 74/42 mmhg , heart rate of 112 beats / min , respiratory rate of 20 breaths / min and temperature of 98.6 f. remarkable findings on physical examination included jaundice as well as abdominal distention and tenderness to palpation , especially in the right upper quadrant . as per family , she did not have recent travels or exposure to sick people . they denied having domestic animals at home or any contact to the patient with pets . admission diagnosis was systemic inflammatory response syndrome with sepsis and septic shock with multi - organic dysfunction syndrome , presumptively secondary to community - acquired pneumonia , possible biliary tract infection ( ascending cholangitis ) , probable spontaneous bacterial peritonitis ( sbp ) and alcoholic hepatitis . empiric intravenous antimicrobial therapy , composed by piperacillin / tazobactam ( 2.25 g every 8 h ) and azithromycin ( 500 mg every 24 h ) , was initiated . laboratory results on admission were : a complete blood cell count of 21,000/mm(90% neutrophils ) , a platelet count of 51,000/mm , a sodium level of 127 meq / l , a potassium level of 5.3 meq / l , a bicarbonate level of 15 meq / l and a creatinine level of 6.3 mg / dl . alkaline phosphatase level was 290 iu / l , gamma - glutamyl transpeptidase level was 290 u / l and lactate dehydrogenase level was 482 total bilirubin level was 14.5 mg / dl , with a direct bilirubin level of 10.1 mg / dl . arterial blood gases showed a ph of 7.15 , a pco2 of 23 , and a po2 of 132 while the patient was receiving mechanical ventilation with fio2 of 60% . after 24 h of admission , patient remained in critical condition and requiring maximum doses of vasopressors , despite of what it seems to be the appropriate empiric antimicrobial therapy . blood culture system bact - alert 3d was used for the recovery of the organism . no minimal inhibitory concentrations for these antimicrobials were reported . in spite of all medical efforts , p. multocida is a small gram - negative non - spore forming facultative anaerobe organism that is a natural inhabitant of the normal gastrointestinal flora and the upper respiratory tract of wild and domestic animals , especially cats and dogs . it is easily overgrowing by other flora in the sputum and might be regularly missed , as it resembles haemophilus influenzae , francisella tularensis and yersinia pestis . the five species that cause the majority of pasteurellosis are : multocida , septica , canis , stomatis , and dagmatis . human p. multocida infections have been reported to occur with and without animal exposure , the former mostly associated with the domestic cat and dog bites or scratches . most of the infections involve the skin and soft - tissues and they can be complicated by abscess formation , osteomyelitis or septic arthritis . the respiratory tract is the second most common site of infection with a wide spectrum of diseases that includes rhinosinusitis , tracheobronchitis , epiglottitis , pneumonia , empyema and lung abscesses . the majority of p. multocida pneumonia cases occur in elderly patients with underlying chronic pulmonary disease . invasive forms of p. multocida infection usually occur in immunocompromised patients , such as those with advanced age , chronic renal failure , solid tumors , hematological malignancies , diabetes mellitus or liver cirrhosis . p. multocida causes a wide variety of disease , including abdominal and pelvic infections , endocarditis , meningitis and endophtalmitis . von graevenitz et al . reviewed 21 proven cases of p. multocida bacteremia . a remarkable number of those cases involved patients with liver diseases , including liver cirrhosis of any etiology , hepatitis and infiltrating tumors . impaired function of the reticuloendothelial system and the presence of portosystemic shunts in patients with liver disease seem to play a major role in the development of bacteremia . this case seems peculiar in that no direct contact with a domestic animal could be documented . our patient had a history of alcohol - induced liver cirrhosis , which predisposed her to the development of bacteremia . however , retrospectively , it is difficult to establish the primary source of infection in this case . the findings of the abdominal examination may suggest that the primary source was the intra - abdominal cavity . unfortunately , the medical team was not able to performed paracentesis to obtain ascitic fluid for cytology and cultures due to patient s medical condition . no abdominal imaging was available . on the other hand , the chest radiography findings of infiltrate and pleural effusion may point the respiratory tract as the primary source of infection . we believed that , given the history of symptoms on admission and the presence of underlying liver disease , the intra - abdominal cavity was the most likely primary source of infection in this patient . nasopharyngeal colonization with p. multocida with transient bacteremia and seeding of the peritoneal cavity in immunosupressed cat owners could play an important role in the development of sbp . ten of these patients were exposed to animals , nine had positive blood cultures and four patients died . however , penicillin - resistant strains in human infections have been described . in these cases , 2 and 3 generation cephalosporins , macrolides , fluoroquinolones , tetracyclines , and trimethoprim - sulfamethoxazole outcome is associated with severity of the infection , the extent of the underlying disease and the early initiation of appropriate therapy . although cases of bacteremic p. multocida infections has been infrequently reported in the literature , clinicians should considered this organism as an important and potentially lethal pathogen in humans , where it can cause life - threatening infections . p. multocida should be included in the microbiologic differential diagnosis in patients with underlying chronic liver diseases who presents with possible intra - abdominal infection , even without history of exposure to domestic animals .

pasteurella multocida is a gram - negative organism characterized morphologically as coccobacillus . it is the commonest organism infecting pet bites . however , severe infections may occur in the absence of animal bites or scratches . pasteurella multocida serves as an opportunistic pathogen in humans , especially in patients with depressed immune system . few cases in the literature identify pasteurella multocida as the causative agent of septic shock , especially in cirrhotic patients . we report a patient who presented with septic shock as a result of pasteurella multocida bacteremia , without prior history of animal exposure .

a 27-year - old malay male patient presented two weeks after alkali splashed into his left eye while working . prior to reporting to us , the patient consulted a general practitioner who prescribed some topical eye drops ( details of the treatment were not available ) and the eye condition worsened . anterior segment examination revealed a central corneal ulcer ( 1 1 mm ) with stromal infiltration ( 4 6 mm ) , limbal ischemia , normal anterior chamber depth and visual acuity of counting fingers . a gentle b - scan ruled out any posterior segment abnormality . a clinical diagnosis of left corneal ulcer with limbal ischemia secondary to alkali injury with secondary infection was made . culture from corneal scraping was negative . despite intensive therapy with oral ( tab ciprobay 500 mg twice daily , bayer ) and topical ciprofloxacin ( gutt ciloxan 0.3% hourly , alcon ) ( no topical steroids used ) with other supportive therapy ( tab vitamin c 1 g daily , pharmaniaga ) , further deterioration led to formation of central corneal perforation with surrounding stromal thinning and flat anterior chamber . we performed a gunderson conjunctival flap under local anesthesia ( la ) , which retracted and failed after one week . slit - lamp examination revealed a large central corneal perforation ( 4 5 mm ) with surrounding stromal thinning involving two- thirds of the cornea without any signs of infection . in order to maintain the shape of the eyeball and due to the problem of non - availability of donor cornea , we decided to use processed bp xenograft ( lyolemb ) supplied by national tissue bank , universiti sains malaysia after obtaining the consent of the patient . a 360 peritomy was done and the graft was sutured with 7 - 0 polyglycolic and polylactic acids ( vicryl ) to the perilimbal area with conjunctival advancement . topical ( gutt ciloxan 0.3% hourly , alcon ) and oral ciprofloxacin ( tab ciprobay 500 mg twice daily , bayer ) was prescribed in the postoperative period [ figure 1 ] . nine months follow - up showed a well - taken graft without any exposure / dehiscence and minimal inflammation [ figure 2 ] . slit - lamp examination could not reveal the details of the anterior chamber and beyond due to conjunctival advancement and vision was noted as perception of light with inadequate projection of rays . conjunctival flap is unlikely to seal large corneal perforations associated with corneal melting following chemical injury . efficacy of amt alone or with glue in treating small corneal perforation has been well established but found unable to promote corneal stability in patients with severe corneal thinning and therefore , an additional urgent tectonic procedure has to be undertaken.3 non - availability of donor cornea poses a considerable problem in some countries . processed bp is a tough lyophilised collagen sheet used as a surgical armamentarium in wrapping orbital implants.4,5 it renders the material antigenically inert and adds to its strength , pliability and longevity.5 it has been shown to be well tolerated with minimal inflammation in animal studies.4 although theoretic concerns about prion disease can be raised , no evidence of this problem has been reported from over 90,000 human implantations.6 devron reported no significant ophthalmic reactions due to this xenograft in his cases of orbital implants.7 at the national tissue bank , universiti sains malaysia , the procured pericardium sheet was washed using chemical agent ( sterile phosphate buffer at ph 7.4 ) and frozen to 40c . it was then freeze - dried and packaged in double - layer plastic bag . the processed bp was radio - sterilized at 2.5 mega rads using gamma radiation and was then ready for use.8 we used this locally processed bp in our case . its use in complicated ocular surface diseases such as large corneal perforation with corneal melting can be promising . its use needs to be further studied more extensively as an alternative graft material in large corneal perforations .

to describe use of a locally processed bovine pericardium ( bp ) to cover a large central corneal perforation following alkali injury and discuss postoperative outcome.a 27-year - old malay male patient presented two weeks after alkali splashed in his left eye while working . a clinical diagnosis of left central corneal ulcer with limbal ischemia following alkali injury with secondary infection was made . after failed medical therapy , we performed a gunderson conjunctival flap under local anesthesia that retracted after one week and resulted in a large central corneal perforation with surrounding stromal thinning . the perforation was covered with a locally processed bp xenograft ( lyolemb ) supplied by the national tissue bank , university sains malaysia . nine months follow - up showed a well - taken graft without any exposure / dehiscence and minimal inflammation.amniotic membrane transplantation when used as a patch graft needs an urgent tectonic graft to promote corneal stability in patients with severe corneal thinning . the use of processed bp can be a viable option in treating such cases .

single incision laparoscopic surgery ( sils ) for cholecystectomy procedure has been introduced as early as 1999 to achieve less pain , less scarring , and less hospitalization period . one of the major difficulties of this procedure seems to be the traction of the gallbladder in order to expose the tissues during operation without additional ports . the use of transabdominal 20 nylon sutures attached to keith needles and the use of a kirschner wire hook introduced through subcostal area are reported methods used for traction of gallbladder and better exposition of the calot triangle . however , all these techniques consist puncture of the gallbladder with sharp needles especially if it is distended and carry the risk of bile leakage and contamination afterwards . herein , we describe our technique in establishing single port access for cholecystectomy in five patients that involves the use of a 2 mm in diameter grasper , karl storz 27290f , that is generally used by urologists for percutaneous nephrolithiasis intervention . after induction of general anesthesia , an umbilical skin incision , 1.5 cm in length , is made vertically and a covidien sils port having 5 mm12 mm four holes is inserted to the peritoneal cavity under direct vision and pneumoperitoneum is created . thereafter , a minigrasper , karl storz 27290f , 2 mm in diameter ( figure 1 ) , was inserted thoroughly an incision , 2 mm in length , created at the transaction point of the midclavicular vertical line and umbilical horizontal line ( figure 2 ) . it is used to hold the fundus of the gallbladder gently and push the organ in an cephalad direction to visualize calot 's triangle ( figure 3 ) . this atraumatic device helped to mobilize gallbladder whenever needed during surgery without causing any wound or leakage . then , the dissection of the gallbladder was completed in a standard fashion from bottom to top and after the organ was dissected free from the liver , it was removed directly through the umbilical incision . no complications including gas leakage around the minigrasper were encountered during and after the surgery . followup was done in the first month and minimal scarring was observed in the umbilicus and the minigraspers ' small incision was almost invisible . single incision laparoscopic cholecystectomy ( silc ) was first described by navarra et al . in 1997 , , silc technique has been performed by many clinicians with promising results and many advantages like few complications , acceptable operation period , less scarring , less pain , and quicker recovery . there are many technical challenges in silc surgery as one of them seems to be the retraction of the fundus cephalad and traction of the hartmann 's pouch laterally to ensure safe cholecystectomy . for this purpose , some surgeons use transabdominal 20 nylon sutures attached to keith needles for fixation and assist gallbladder retraction and some other used kirschner wire hook which is introduced through subcostal area to pull gallbladder in an upright direction during the operation . yet , all these techniques consist puncture of the gallbladder with sharp needles especially if it is distended and carry the risk of bile leakage and contamination afterwards . they also have limited ability to mobilize whenever needed . to achieve gallbladder retraction and cephalad traction , this minigrasper is used not only in performing sufficient traction of the gallbladder in an upright position , but also it can allow good mobilization of the gallbladder in order to dissect calot 's triangle safely . use of this instrument has led no perforation and leakage of bile in the patients . through a very small incision , 2 mm in length , without using any port , it has been introduced directly into the abdomen under camera control and made the dissection trouble - free as the manipulation of the gallbladder could be done easily to every direction needed . no gas leakage has been noted around the instrument after direct introduction into the abdomen in all cases . the most remarking effect of using this technique was its influence on the operation time . previous reports using port systems or transabdominal sutures declare their mean operating time to be approximately at least 30 min to 70 min . our procedure even on the first patient took only 40 min to conclude without any complications . we believe learning curve would be low and it would be possible to significantly decline operation periods in following patients . in conclusion , with the help of this instrument the operation period may be lessened , less scarring is achieved and surgical procedure is done more easily and safely without any additional costs .

purpose . we present our experience in single incision laparoscopic cholecystectomy by using a grasper directly without using a trocar in five patients . methods and results . the technique involves the use of karl storz 27290f grasper in order to perform gallbladder retraction in single port cholecystectomy . the grasper was introduced directly into the skin through abdominal wall without using any trocar and used to mobilize gallbladder whenever needed during surgery without causing any perforation or leakage of the gallbladder . there were no intraoperative and postoperative complications in 5 patients with the advantages of shorter operation time and almost invisible postoperative skin scar formation . conclusion . we claim that the use of this instrument in sils surgery might be advantageous than the conventional placement of sutures for the gallbladder mobilization .

two types of cnt were investigated , nc7000 from nanocyl and mwnt sa from sigma aldrich ( catalog reference 659258 ) , both produced by chemical vapor deposition ( cvd ) . nc7000 has a diameter of 710 nm , and mwnt sa has a diameter of 110170 nm . the cnt were functionalized by a 1,3-dipolar cycloaddition reaction of azomethine ylides using a one - pot functionalization procedure described elsewhere13 leading to the formation of pyrrolidine - type groups bonded to the cnt surface . the functionalization was carried out at 250 c over 5 h. the unzipping of the cnt was performed using an ultrasonic processor up100h from hielscher , equipped with a sonotrode ms2 . cnt suspensions were prepared by mixing functionalized cnt ( 5 mg ) in ethanol ( 8 ml ) . a control experiment was conducted using pristine cnt ( 5 mg ) in ethanol ( 8 ml ) . the suspensions were centrifuged ( 8000 rpm , 1 h ) to separate the unzipped cnt , and the gnr supernatant solutions were collected . these solutions were analyzed by uv - visible spectroscopy on a shimadzu uv-240 1 pc , using 10 mm light path quartz cells . the micro - raman analysis was conducted in the backscattering configuration on a jobin yvon hr800 instrument ( horiba , japan ) , using a 1800 l mm grating and the 532 nm laser line from a nd : yag dpss laser ( ventus , laser quantum , uk ) . a 50x objective ( spot size : 3 m , na=0.75 , olympus , japan ) was used to focus the laser light onto the sample ( < 0.15 mw m ) and to collect the backscattered raman radiation to be detected by a peltier cooled ( 223 k ) ccd sensor . the spectrometer was operated in the confocal mode , setting the iris to 300 m , while the acquisition time was set to 30 s with 2 accumulations . transmission electron microscopy ( tem ) of the gnr mwnt sa was performed on an energy filtered 200 kv transmission electron microscope hr-(ef)tem tem analysis of the gnr nc7000 was carried out on a titan chemistem 80200 kv probe cs corrected microscope . low - magnification tem and high - resolution tem ( hrtem ) images were acquired with a gatan ultrascan 1000 p camera controlled with digital micrograph software integrated in the microscopes user interface . the samples were prepared by adding a drop of the gnr solution onto a lacey carbon cu grid ( 300 mesh , ted pella ) and allowing it to dry under vacuum . x - rays diffraction experiments were performed on a panalytical xpert pro xrd system using the cu k1 wavelength of 0.15406 nm from a copper x - ray tube operated at 45 kv and 40 ma . a pixcel-3d detector was used , and the scan range was from 4 to 40 in 2. the gnr samples were deposited on glass lamellae by solvent evaporation . the unzipping process produces functionalized gnrs with widths equal or larger than 35 nm for gnr nc7000 and 350 nm for gnr mwnt sa . for this reason , a model of functionalized graphene ( see figure 6 ) , as opposite to finite gnr , was chosen . graphene layers and their intermolecular interactions were modelled with the mm3 force field that has been found to give accurate intermolecular structures.17 all the calculations were performed with the tinker molecular mechanics suite18 using three - dimensional periodic boundary conditions.19 the shape and dimensions of unit cells containing five functionalized graphene layers with different concentrations of functional groups were systematically obtained by energy minimization . additional data for the characterization for the gnr : thermogravimetric analysis ( tga ) and fourier - transform infrared spectroscopy ( ftir ) , is provided as supporting information . as a service to our authors and readers , this journal provides supporting information supplied by the authors . such materials are peer reviewed and may be re - organized for online delivery , but are not copy - edited or typeset . technical support issues arising from supporting information ( other than missing files ) should be addressed to the authors .

graphene nanoribbons ( gnr ) were generated in ethanol solution by unzipping pyrrolidine - functionalized carbon nanotubes under mild conditions . evaporation of the solvent resulted in regular few - layer stacks of graphene nanoribbons observed by transmission electron microscopy ( tem ) and x - ray diffraction . the experimental interlayer distance ( 0.490.56 nm ) was confirmed by computer modelling ( 0.51 nm ) . computer modelling showed that the large interlayer spacing ( compared with graphite ) is due to the presence of the functional groups and depends on their concentration . stacked nanoribbons were observed to redissolve upon solvent addition . this preparation method could allow the fine - tuning of the interlayer distances by controlling the number and/or the nature of the chemical groups in between the graphene layers .

in iden 2012 , many diverse experiences associated with basic procedures to advanced techniques of endoscopic retrograde cholangiopancreatography ( ercp ) were highlighted in great enthusiastic lectures by world renowned experts . interesting cases entitled " interesting cases in pancreatobiliary endoscopy " were introduced in video forum . in this highlight summary of interesting presentations , i will present a summarized review about basic procedures of ercp like balloon dilation for common bile duct ( cbd ) stones , advanced techniques like endoscopic necrosectomy for necrotizing pancreatitis , recently proposed management for the prevention of post - ercp pancreatitis , and spyglass direct visualization system through which we can directly observe the cbd and pancreatic duct . eplbd is easy to use and effective for the removal of common bile duct ( cbd ) stones , but still debates exist on safety issue . since eplbd requires only a small endoscopic sphincterotomy ( est ) or none at other occasions , eplbd is generally believed to avoid the complications of a full est . lee and han1 conducted a large retrospective multicenter study , in which a total of 946 consecutive patients with large cbd stones were enrolled in this study . serious adverse events occurred in 95 patients ( 10% ) , after which the following guidelines of eplbd in order to pursuit zero mortality were suggested ; 1 ) eplbd should be avoided in patients with distal cbd strictures ; 2 ) full est should be avoided immediately before eplbd ; 3 ) the balloon should be inflated gradually ; 4 ) inflation should be discontinued in cases of persistent balloon waist ( 75% rule ) ; 5 ) not to be inflated beyond the maximum diameter of the dilated cbd ; and 6 ) convert to an alternative stone removal or drainage method any time there is difficulty in removing the stone . because it was multicenter study analyzing the safety of eplbd based on large numbers of cases , experienced experts will agree with the above referred recommendations , though it was studied retrospectively . kahaleh and freeman2 recently made recommendations to minimize risks of ercp in international digestive endoscopy network ( iden ) 2012 as follows ; adequate selection of patients undergoing ercp , skilled operators using novel techniques for prompt identification , which is key to successful prevention and management . pancreatitis is the most common complication associated with ercp procedure with average rate of about 5% . risk factors for the occurrence of post - ercp pancreatitis include younger age , indication of suspected sphincter of oddi dysfunction , history of previous post - ercp pancreatitis , absence of elevated serum bilirubin levels , and female sex are usually at increased risk . techniquerelated issues have long been recognized to be important in causing post - ercp pancreatitis . he also mentioned about specific techniques to reduce risk of post - ercp pancreatitis , such as pancreatic stents , more promising pharmacological agents including non - steroidal anti - inflammatory drug , and enema for prevention of post - ercp pancreatitis . new possibility that eus - guided endoscopic pancreaticobiliary drainage can replace the percutaneous techniques and obviate surgery was shown in iden 2012 . kahaleh3 referred that endoscopic debridement and stent insertion can reduce high morbidity and mortality of surgery in severe necrotizing pancreatitis . endoscopic necrosectomy using repeats session of debridement and plastic stents insertion has been more frequently used within the last decade and half . fully covered self - expandable metal stents might provide a safer and more efficient drainage through a larger diameter stent . additionally he described techniques of transmural drainage and endoscopic debridement , how to make the transenteric access into the pancreatic necrosis , how to make active endoscopic irrigation with a gastroscope and debridement of cystic contents using biopsy forceps , and roth nets and polypectomy snares in detail . this technique is evolving continuously as we attempt to optimize the post - procedural outcomes . there were attractive video lectures during iden 2012 dealing with advanced techniques for pancreaticobiliary visualization including direct peroral cholangioscopy , spyglass direct visualization system , forward - viewing echoendoscopy , and contrast - enhanced eus and elastography accompanied with actual interesting cases for each . kahaleh4 mentioned that the single operator cholangioscopy ( soc ) system ( spyglass ; direct visualization system , natick , ma , usa ) may offer an interesting compromise in terms of size ( 10 fr diameter ) and complexity of use . soc is challenged by the size of the biopsy obtained and the stiffness of the forceps ( spybite ; boston scientific , natick , ma , usa ) fitting within the working channel of the system . sensitivity of forceps biopsy through the cholangioscope was far higher for intrinsic ( 66% ) than extrinsic ( 8% ) malignant lesions . conclusively , spyglass ( direct visualization system ) has not only been used as a platform for advanced intraductal imaging with probe based endomicroscopy and spyglass - guided stone fragmentation ; but also for photodynamic therapy to treat bile duct cancer . soc can be a great step to realize intraductal visualization as well as therapy , but the best is yet to come . pancreatobiliary endoscopy is continuously evolving area with new techniques and we attempt to optimize the postprocedural outcomes .

the pancreatobiliary organ is composed of one of the most complicated structures and complex physiological functions among other digestive organs in our body . this is why endoscopic procedure in pancreaticobiliary system requires rather complicated techniques . in international digestive endoscopy network ( iden ) 2012 , many interesting pancreatobiliay endoscopy related topics were presented . basic procedures like endoscopic papillary balloon dilation ( epbd ) , advanced techniques like endoscopic necrosectomy , prevention and management of post - ercp pancreatitis , and spyglass system are reviewed in this highlight summary .

these lesions though may cause life threatening hemorrhage , watchful observation is still considered as a treatment option in these patients . a 71-year - old male presented with pulsatile swelling over the right side of face and over the forehead in october 2009 . patient is a known diabetic and hypertensive for the past 9 years on regular medications . patient was evaluated with computed tomography ( ct ) angiogram , which showed aneurysm of the external carotid artery with thrombus within and multiple aneurysm of the branches of external carotid arteries . treatment option of excising the aneurysm with ligation of external carotid aneurismal branches was discussed with the patient . since the swelling was asymptomatic for the past 34 years , patient was not willing for any procedure . he is assessed with clinical examination on each visit and ultrasound examination performed once in a year to assess the size of the aneurysm . the patient has been on follow up for the past 4 years without any new symptoms or increase in size of the aneurysm . aneurysm of the external carotid artery and its branches have been described in cases due to trauma , iatrogenic injury , dental extraction , and also due to head and neck cancer because of tumor infiltration or due to radiation therapy . geriatric patients with aneurysm are first consulted by a primary care physician and hence primary care physician at the community level has a major role in following these patients and referring these patients to specialized vascular centre . increase in size of the aneurysm or impending rupture necessitates the need for referral to specialized center . the need for surgical intervention or endovascular stenting does not arise in our patient who has this aneurysm for 34 years and was on follow up for the past 4 years without any symptoms [ figure 1 ] . pulsatile swelling over the face , forehead , and scalp in this setting , the role of primary care physician is important to decide upon surgical or conservative management after having discussion with the patient . multiple aneurysm of external carotid artery this case was discussed for the rarity of idiopathic multiple aneurysm [ figure 3 ] of the external carotid artery [ figure 4 ] and the need for individualized treatment protocol to be followed , as in this case , only watchful observation . in this world of evolving surgical techniques and newer treatment modalities , conservative treatment still has a role to play . even in the era of advanced medical technology practices , treatment should be individualized and the choice rests with the patient . primary care physicians have a major role in follow up of these patients and decide upon referring these patients to specialized vascular center thus lessen the burden of vascular centre in following these patients .

aneurysms of external carotid artery are rare . treatments for these are undertaken for the prevention of complications like hemorrhage or rupture , and embolism . we present a 71-year - old male with idiopathic multiple aneurysm for the past 34 years on conservative management and regular follow up for the past 4 years . this case was discussed for the rarity of idiopathic multiple aneurysm of the external carotid artery and the need for individualized treatment protocol to be followed as in this case , only watchful observation considering the age and patient compliance . in this world of evolving surgical techniques and newer treatment modalities , conservative treatment still has a role to play . primary care physicians at the community level have a major role in following these patients and referring them as and when the need arises .

a 78-year - old woman presented with weakness , fatigue and hemoglobin of 8.3 g / dl , to another clinic in september , 2009 , and she was given erythrocyte suspension replacement . she had regression in her existing complaints with blood replacement therapy , but after one month , same complaints reappeared with a low hemoglobin value and she was referred for further investigation to the outpatient clinic of hematology of our university faculty of medicine . her hematologic parameters ar that time were as follows : white blood cell count of 6390/mm , neutrophils 3785/mm , hemoglobin 8.1 g / dl , mean corpuscular volume ( mcv ) 95 fl , and platelets 211.000/mm . with the provisional diagnosis of myelodysplastic syndrome due to an isolated anemia unrelated to nutritional status , bone marrow aspiration biopsy was performed . her bone marrow aspiration showed normal cellularity ; and biopsy revealed increase in the number of megakaryocytes , signs of dismegakaryopoiesis , minimal dysplasia in erythroid series , and normal morphology in myeloid series . upon conventional cytogenetical studies she had been lost - to - follow up for approximately a year and during that time she had received a total of 4 units of erythrocyte suspension , and erythropoietin treatment was started . however , erythropoietin treatment discontinued because she did not respond to the maximum dose of 10.000 iu three times a week . she did not attend to her follow - up visits regularly and in the mean time she had transfusions monthly at another clinic . since her transfusion dependency increased , she was considered for lenalidomide treatment in april , 2013 . after 10 days of treatment , her liver enzymes increased more than 3 times of normal values and she had thrombocytopenia ( platelet count : 38.000/mm ) then her treatment was postponed for two weeks she restarted lenalidomide treatment at daily doses of 5 mg after liver enzymes and platelet counts returned to normal . during further follow - up , increase in liver enzymes and severe thrombocytopenia were not detected ; her hemoglobin value increased and transfusion dependency was not observed ( figure 1 ) . in myelodysplastic syndrome , interstitial deletion in the long arm of chromosome 5 is a common cytogenetical abnormality seen in 16 - 28% of the patients . it is characterized by hypoproliferative anemia and normal megakaryocytes with hypolobated nuclei or increased dysplastic megakaryocytes in bone marrow [ 7 , 8 ] . most of the patients are transfusion - dependent because endogen erythropoietin production is usually increased . one of the major problems during the progression of the disease in transfusion - dependent mds patients is the iron overload secondary to transfusion and the dependency for chelation therapy to treat iron overload . the immunomodulatory drug lenalidomide that has additional benefits in the regulation of erythropoiesis , inhibition of angiogenesis , and reorganization of erythroid production , targets the mds clone directly [ 9 , 10 ] . this syndrome is rarely seen ; therefore , there is not enough data about isolated 5q - syndrome as well as lenalidomide therapy in our country . isolated 5q - syndrome presents with normal or increased thrombocyte counts in peripheral blood and anemia with frequent transfusion replacement dependency . in clinical studies lenalidomide provided long term blood transfusion independency and erythroid response in patients with low- risk del(5q ) and without del(5q ) . in a phase ii study of low- risk del(5q ) patients ; after 24 weeks , evaluation of erythrocyte response showed that 67% of the patients with 5q deletion who were treated with lenalidomide became transfusion independent . in low- risk 5q deletion mds patients , daily doses of 10 mg for 21 days are used , and repeated with 28 day - intervals that can be modified if needed . in order to evaluate the response , adverse events can be easily overcome with dose modifications . in clinical studies , thrombocytopenia and leukopenia are frequently observed but these adverse reactions usually appear during the first 8 weeks of treatment . therefore , weekly clinical follow - up at the beginning of the therapy is recommended . less often adverse events of fatigue , rash and diarrhea can be noted . in conclusion , in isolated 5q - syndrome which is a mds subgroup and characterized by transfusion dependency ; lenalidomide can decrease transfusion dependency with a tolerable adverse event profile that can be overcome with dose modifications . assessment of all prevalent cases in our country will be helpful to reveal the efficacy and adverse event profile of lenalidomide .

5q - syndrome is a special subgroup of myelodysplastic syndrome in terms of follow - up and treatment . lenalidomide is an immunomodulatory drug that is frequently used in the treatment of multiple myeloma . some clinical studies have shown that lenalidomide treatment is effective in 5q syndrome and significantly decreases the transfusion dependency in these patients . in this paper , we would like to share a dramatic response of lowered transfusion dependency after treatment with low - dose lenalidomide in a patient who received myelodysplastic syndrome diagnosis and isolated 5q anomaly in our clinic .

traumatic arterial occlusion following major or minor blunt trauma , especially in the absence of any other bony injury , is a rare phenomenon . motor - scooter handlebar syndrome is one such type of arterial occlusion affecting vascular structures following direct blow by a handlebar of a motorbike or bicycle to the groin . given their superficial course at this location , femoral vessels are the most common vascular structures affected . in all instances , injury to iliac vessels remains exceedingly rare , given its posterior position within the pelvis , representing only 0.4% of vascular injuries . in this case , we highlight a delayed presentation of external iliac artery occlusion secondary to motor - scooter handlebar syndrome , in a paediatric patient . pathophysiology and management of vascular injuries in the paediatric population vary significantly compared with the adult population . additional factors which need to be considered include : smaller vessel size or vessel spasm , higher risk of infection , tendency for re - stenosis and continuing growth . we review previous paediatric cases of this unusual vascular injury to highlight the pathology and most appropriate management option . a 15-year - old male presented to the emergency department following a direct blow from his bicycle handlebars to his groin . his abdominal examination was unremarkable , except for a small abrasion and visible mass in his right groin . after exclusion of other abdominal or chest injuries , he was discharged on the same day with a diagnosis of right groin haematoma and follow - up in 68 weeks . his symptoms included pain and paraesthesia in his right buttocks on mobilization > 100 m. he was found to have absent peripheral pulses in his right leg , although it appeared well perfused with a normal capillary refill . an arterial doppler ultrasound showed a right external iliac artery thrombus occluding the proximal two - thirds of the vessel . a computed tomography scan of the abdomen and pelvis with intravenous contrast identified complete occlusion of the right external iliac artery 1 cm beyond its origin . however , the common femoral artery and profunda femoris remained patent via collaterals ( fig . 1 ) . he underwent a right external iliac thromboendarterectomy with patch repair using a saphenous vein graft . a suprainguinal incision was initially made in attempt to expose the proximal external iliac artery . due to its extent , retrieval of the entire thrombus was incomplete and a second groin incision near the junction of the common femoral with external iliac ( at the inguinal ligament level ) was required . the patient subsequently underwent a proximal and distal thrombectomy , and the arteriotomy was extended between the two incisions identifying an intimal stricture with significant fibrosis ( fig . 2 ) . an intimal flap was identified in the distal region and tacked down with a 7 - 0 prolene suture . the long saphenous vein was harvested and the defect closed with patch repair extending from proximal iliac to proximal femoral vessel . at the initial 2-week follow - up , the vessels remained patent and patient was progressing well . subsequently , regular biannual follow - up was planned to monitor for any longer - term complications . figure 2:occlusion of right external iliac artery 1 cm below its origin ( white arrow ) . occlusion of right external iliac artery 1 cm below its origin ( white arrow ) . motor - scooter handlebar syndrome is an uncommon form of arterial blunt injury following a direct blow by the handlebar of motorbikes or bicycles . only a handful of paediatric cases have been reported [ 1 , 2 , 47 ] . the common femoral vessel is the most commonly affected vascular structure . a common site for these injuries is at the inguinal ligament , where the femoral artery is superficial and courses anterior to the superior pubic ramus and femoral head . as such , it is prone to compression between the handlebar and posterior osseous structures . in addition , it is a relatively immobile structure , tethered by arterial branches , periadventitial connective tissue and the femoral sheath . they proposed that a circumferential tear of the intima leads to dissection and prolapse of the inner aortic layers , causing complete luminal occlusion . , patients may remain asymptomatic until a period of growth spurt or they resume more rigorous physical activity . this can lead to delay in the diagnosis , and a high index of suspicion needs to be maintained to avoid ischaemic complications such as limb length discrepancy [ 8 , 9 ] . in such instances , it is important to rule out any secondary occlusion of the femoral artery and vein that may predispose to the development of thrombosis within these vessels . duplex sonography should be carried out to assess flow velocities and waveform characteristics in these patients , and is especially suited to the paediatric population as they have reduced abdominal fat , and there is no radiation . identified ultrasound , in the right hands , to be highly sensitive ( 100% ) and specific ( 97.5% ) for the detection of arterial injuries when compared with surgical findings . open arterial thromboendarterectomy with graft or patch repair is the standard of care for such cases in trauma centres . previously documented cases include patch repair or bypass using saphenous vein , bovine pericardium and synthetic material [ 13 , 5 , 6 ] . one case was successfully treated with iv heparinzation alone . however , in most injuries , the presence of significant groin haematoma may limit anticoagulation , requiring more urgent operative re - vascularization . in our patient , delayed presentation and presence of significant collaterals meant definitive operative management could be planned as a semi - elective procedure . the role of endovascular intervention in this paediatric population has previously been documented mainly as a temporizing measure for revascularization [ 4 , 5 ] . used a nitinol stent in the distal superficial femoral artery of a 13-year - old patient whose arterial and venous calibre at the time of injury were of inappropriate size for reconstruction . the main disadvantage of endovascular interventions in paediatric patients is the constantly enlarging calibre of the vessel with a fixed stent diameter . this can predispose to complications such as restenosis , stent fracture , stent dislocation and acute on chronic ischaemia . as a result , future vascular reconstruction may be compromised and in extreme cases , this may progress to significant ischaemia and even limb loss . we provide an unusual cause of external iliac occlusion secondary to bicycle handlebar injury to the groin . all patients presenting with groin injury from this mechanism should be carefully investigated with duplex sonography and monitored for risk of vascular injury . the presence of collaterals , particularly in the paediatric population , can lead to delay in diagnosis . literature seems to advocate open primary surgery for management of these injuries ; however , endovascular and conservative medical management have also been used successfully . the latter two were contraindicated in our patient and open surgery was the most appropriate option . long - term follow - up , beyond the 12-month period , is needed , particularly in relation to known vascular complications including pseudoaneurysms , arteriovenous fistula formation and restenosis .

arterial occlusion following blunt trauma is an uncommon occurrence . we report an unusual case of delayed external iliac artery occlusion in a young male following blunt abdominal injury . he was successfully treated with thromboendarterectomy and saphenous vein patch repair . there have only been a handful of documented cases occurring in the paediatric population . all patients presenting with groin injury from this mechanism should be carefully investigated and monitored for risk of vascular injury .

congenital heart defects / diseases contribute substantially to morbidity and mortality in childhood , especially in the third - world countries where facilities for management are often deficient.1 about 35,000 infants ( 1 out of every 125 ) are born with heart defects every year in the united states.2 the incidence in the tropics is not expected to differ from the 8 - 10 per 1000 live - births documented in the developed world.3 the defect may be so slight that the baby may be asymptomatic for many years after birth , or so severe that it is life threatening . heart defects are the leading cause of birth defect - related deaths.4 however ; the use of echocardiography has improved description of congenital heart diseases and their early diagnosis.5 this , has led to dramatic increases in survival of children with serious heart defects . cor triatriatum is a rare congenital cardiac anomaly , in which a fibromuscular membrane divides the atrium in two . it was first reported in 1868.6 cor triatriatum , a heart with 3 atria ( triatrial heart ) , is a congenital anomaly in which the left atrium ( cor triatriatum sinistrum ) or right atrium ( cor triatriatum dextrum ) is divided into 2 parts by a fold of tissue , a membrane , or a fibromuscular band.7 however , variable types of subtotal cor triatriatum are also noted , with only the right or left pulmonary veins draining into the upper chamber.8 cor triatriatum represents 0.1% of all congenital cardiac malformations and may be associated with other cardiac defects in as many as 50% of cases . examples of associated cardiac defects include atrial septal defect , persistent left superior vena cava with an unroofed coronary sinus , partial anomalous pulmonary venous connection , ventricular septal defect and more complex cardiac lesions such as tetralogy of fallot , atrioventricular canal and double outlet right ventricle . associated bicuspid pulmonary valve , aortic valve atresia and heterotaxy have also been described.9 congenital pulmonary vein stenosis is a very rare association with cor triatriatum.10 the morbidity and mortality of cor triatriatum sinistrum is high in those who are symptomatic in infancy . this is due to the severely restrictive opening in the accessory membrane and the association with major cyanotic or acyanotic congenital heart lesions . significant sequel is unusual with cor triatriatum dextrum as it is not commonly associated with life - threatening symptoms or major congenital cardiac defects . a female baby seen on the second day of life was delivered by a 25-year - old primiparous youth corper . the mother attended antenatal clinic in federal medical centre owerri from the 16 week of gestation . however , during the 8 week of pregnancy , the mother had threatened abortion for which some drugs were given ( injections and tablets names unknown ) at a hospital in lagos . she also had some liquid herbal preparation at 8 months of gestation ; other drugs she took were essentially haematinics . she gave birth to a 3.6-kg baby girl who cried immediately after , and breastfeeding commenced . the child was transferred to the special care baby unit from the postnatal ward on account of history of fever on day 2 of life . examination revealed a full - term newborn , febrile ( 38.1 ) with a respiratory rate of 100 cycles per minute , acyanotic , heart rate of 110 beats / min heart however , the child completed a course of antibiotics ( ampicillin sulbactam and gentacin ) and , with clinical improvement , was discharged home . at follow - up at the 2 week of life , the child weighed 3.5 kg , heart rate was 130 beat / min and a grade 3 pansystolic murmur was heard , following which a chest radiograph and electrocardiograph ( ecg ) was requested . at 1 month of life , a review of ecg showed : heart rate : 128 beats / min , t-18 , which suggested supraventricular arrhythmia , abnormal right superior axis deviation . murmur was still grade 3/6 , pansystolic maximal at the left - lower sternal edge . echocardiograph was subsequently requested and the result showed that a 2d echocardiogram was done [ figure 1 ] with the following results : echocardiogram showing the distal chamber ( x ) and the left atrium ; the right atrium ( ra ) , left ventricle ( lv ) , and right ventricle ( rv ) separate left atrium with dividing membranous running in the direction of long axis of left atrium close to the lateral wall . mitral valve draining the larger , more medial chamber , normal atrio - ventriculo - arterial connection . high velocity turbulent flow in the main artery noted ( pa vmax = 335 cm / s pg = 45 mm ) . secundum atrial septal defect [ figure 1 ] the child is presently being followed - up regularly . during her last visit , at 7 months of age , she weighed 9.6 kg , length was 73 cm , occipito - frontal circumference was 47 cm and mid - upper - arm circumference was 15 cm ( all within normal for age ) . cardiovascular examination revealed a child with heart rate of 120 beats / min , apex beat located at the 4 left intercostals space , with a grade 3/6 systolic murmur heard loudest at the upper left sternal edge . the respiratory rate was 42 cycles / min and no organs palpably enlarged on digestive system examination . this was the first case of a five - chambered heart seen and managed in the federal medical centre owerri . the case demonstrates the need for comprehensive evaluation of patients and provision of cardiac investigative facilities ( x - ray , ecg and echocardiograph machines ) in health facilities . in our patient , it was an incidental finding at a routine follow - up clinic . subsequent investigative evaluations led to the confirmation of the diagnosis of a five - chambered heart in a child who has remained asymptomatic . the dearth of facilities in our hospital is obvious here , where the patient had to be referred to the university of nigeria teaching hospital enugu for echocardiograph . clinical manifestations are often delayed due to the presence of a large opening ; and include an asymptomatic murmur . finding is mostly incidental on routine cardiac imaging ; it therefore highlights the need for exhaustive evaluation and management because such conditions , although rare , can still be found here . in conclusion , we reported a case of an asymptomatic congenital - heart defect a five - chambered heart - cor triatriatum , an extremely rare condition .

five - chambered heart is extremely rare in children . we report a case of asymptomatic five chamber heart detected in infancy . the patient is 2-day - old and managed in a special care baby unit ( scbu ) for neonatal sepsis . during routine follow - up at the age of 1 month , she was found to have an asymptomatic murmur . echocardiograph reported five - chambered heart , concluding that it is cor triatriatum , supravalvular pulmonary stenosis and secundum atrial septal defect . the child is still been followed - up and is still asymptomatic at 7 months . five - chambered heart , although rare , can occur even if asymptomatic .

cutaneous horn ( synonyms ; cornu cutaneum : cornu humanum ) is a conical , hyperkeratotic protrusion that often resembles an animal horn . the term cutaneous horn is a morphologic designation referring to unusually cohesive keratinized material and not a true pathologic diagnosis . the earliest documented case of cutaneous horn , or cornu cutaneum , was that of an elderly welsh woman in london who was displayed commercially as an anomaly of nature in 1588 . there were several other accounts of cutaneous horns in the sixteenth and seventeenth centuries , including those described by danish anatomist thomas bartholin in 1670 . illustrations from that time portray these growths as grotesque , and numerous natural and supernatural theories arose regarding their etiology . for centuries cutaneous horns have been a subject of curiosities and controversies . in the late eighteenth century , the london surgeons everard home and his brother - in - law john hunter are generally credited with the characterization of cutaneous horns as a medical disorder . though cutaneous horn has been described at various sites , horn over the penis is very rare and represents the most unusual site . the first case report of a penile horn was published as early as 1854 . since then there has been sporadic reporting of cases by dermatologists , surgeons and urologists mainly for curiosity and various conditions which may present as a penile horn . cutaneous horn occurs mainly in individuals who are above 50 years of age and penile horns are no exception to this rule . they are common in males after 50 years and probably coincide with the age group of occurrence of penile cancer . to this date lowe and mc cullogh reported that penile horn may be benign in 4256% of cases , premalignant in 2237% or frankly malignant in 2022% . cutaneous horn is a morphologic designation for a protuberant mass of keratin produced by unusual cohesiveness of keratinized material . it is rather intriguing to note that why only few patients develop penile horn when compared with other patients with similar disease . the roles of chronic irritation , phimosis , surgical trauma and radiotherapy that have been implicated in penile horn formation have also been found to predispose to carcinoma penis . the emphasis is on the long - standing phimosis with chronic , prolonged preputial inflammation ; however , it has also been seen in circumcised men . adult circumcision has been found to precede horn formation by a period ranging from 2 weeks to a year . the analysis of the cases reported so far do not reveal any common factor that predisposes to the development of horn over the underlying pathology . the occurrence of horn in different disorders ranging from benign viral disease to malignant carcinoma further augments the view that the keratinous growth probably represents a reactionary event , which does not depend on the underlying pathology . pseudoepitheliomatous , keratotic and micaceous balanitis is another rare disease , which has been found to be associated with penile horn . it was , originally described by jacob and civatte who considered it to be benign . similarly , verrucous carcinoma is another tumor which has been found to be associated with penile horn . this tumor is a highly keratinizing tumor , and this probably predisposes for penile horn formation . the progression of cutaneous horn to malignancy though has been reported probably represents the manifestation of underlying tumor . causes of penile horn the role of human papillomavirus ( hpv ) in penile carcinoma is well studied . the hpv 16 and 18 are implicated as the causative agents . in penile horns that arise from malignancies such as verrucous carcinoma , penile horns present as elongated , keratinous , white or yellowish projections that range from a few millimeters to centimeters in size arising from the glans penis [ figures 1 and 2 ] . traumatic breakage of the horn is rare and can occur due to the projectile nature of the growth . hard keratotic mass arising from glans penis the patients seek treatment for the disfigurement and difficulty posed during intercourse . in general horns arising from malignant lesions tend to be harder on their bases due to the inflammatory process . their surface is rough , irregular , laminated or fissured , the ends pointed , blunt or clubbed . the color varies ; it is usually grayish - yellow , but may be even blackish . commonly they are small in size , a fraction of an inch or an inch or thereabouts in length , but exceptionally attain considerable proportions . the base , which rests directly on the skin , may be broad , flattened , or concave , with the underlying and adjacent tissues normal or the papill hypertrophied ; and in some cases there is more or less inflammation , which may be followed by suppuration . they are , as a rule , painless but become painful when knocked or irritated . the histopathology of the cause is diagnostic in most cases . from the histopathological point of view cutaneous horn can be classified as benign , premalignant and malignant based on the origin . it consists of closely agglutinated epidermic cells , forming small columns or rods . in the columns they have their starting - point in the rete mucosum , either from that lying above the papilla or that lining the follicles and glands . keratinous mass overlying the epidermis ( 40 ) part two is the underlying pathology beneath the keratotic mass . though histopathology is diagnostic certain other investigations are required particularly in patients with underlying malignancy . approximately one - third of penile cutaneous horns are associated with an underlying malignancy , and so magnetic resonance imaging is helpful when there is uncertainty regarding the depth of infiltration or proximal extension . as kaposi noted more than a century ago , cutaneous horns can be removed by simple detachment and cauterization of the papillary base . stage one is establishing the diagnosis and next stage is a definitive treatment based on the histopathology . penile horn can hide either benign or malignant lesions . therefore , what is really important is not the cutaneous horn itself , but the subjacent disease . hence , it is justifiable to perform a local surgical excision for the histopathological diagnosis of its base . a wide local surgical excision is performed on the lesion in case of suspected malignancy to obtain the histopathological diagnosis . though various methods including electrosurgical excision , laser and cryosurgery , have been described as effective laser therapy with carbon dioxide or neodymium : yttrium aluminum garnet laser leaves lesser scarring with more cosmetic results . they are not preferred as they alter the histopathology of the lesion . in case of a benign lesion , excision of the horn would suffice . in case of verrucous carcinoma , wide local surgical excision should be followed up regularly . if the biopsy reveals squamous cell carcinoma , the treatment of choice is partial or complete penectomy with urethral diversion and perineal urethrostomy . the penile horn continues to fascinate the dermatologists and surgeons alike because of the morphological appearance and presentation . the penile horn is only a morphological entity and the true pathology masked by it .

cutaneous horn refers to unusually cohesive keratinized material and not a true pathologic diagnosis . though cutaneous horn has been described at various sites , horn over the penis is very rare and represents the most unusual site . the role of chronic irritation , phimosis , surgical trauma and radiotherapy have been implicated in penile horn formation . penile horns present as elongated , keratinous , white or yellowish projections that range from a few millimeters to centimeters in size arising from the glans penis . histopathology of the keratotic mass reveals nothing but keratin . the underlying mass may vary from verruca vulgaris to squamous cell carcinoma . the treatment is based on the pathology .

primary peritonitis constitutes less than 1% of peritonitis and spontaneous bacterial peritonitis usually occurs in patients with comorbid conditions . in absence of comorbid conditions , including liver cirrhosis , immunosuppression , or nephrotic syndrome , primary peritonitis is rare , particularly in cases of healthy young subjects . primary peritonitis due to s. pyogenes is an unusual condition because streptococcus pyogenes usually causes pharyngitis , erysipelas , and necrotizing fasciitis . in this report , we present the first case to the best of our knowledge of primary peritonitis due to s. pyogenes in korea . 29-yr - old woman presented to the emergency room with lower abdominal pain on april 20 , 2011 . she had no travel history and had not taken any medication . on physical examination , she was alert and cooperative . her body temperature was 38 , blood pressure 80/60 mmhg , pulse 113 beats per minute , respiratory rate 20 breaths per minute , and oxygen saturation 99% while breathing ambient air . results of gynecological examination from gynecologists were also normal and no history of abnormal vaginal discharge was known . initial blood tests showed leukocyte count of 6,248/l , platelet count of 133,000/l , c - reactive protein ( crp ) level of 12.36 mg / dl , and procalcitonin level of 36.9 ng / ml . renal and liver function tests , clotting screen , and arterial blood gas were all within normal range . abdominal ct revealed edematous swelling of the intestinal wall and ascites with peritoneal enhancement suggesting peritonitis ( fig . as physical , laboratory , and radiologic findings suggested acute peritonitis , laparoscopy searching for etiology was performed . purulent ascites was found in the pelvic cavity but both ovaries and fallopian tubes were intact ( fig . there were no intra - abdominal abnormalities such as bowel perforation , appendicitis , or necrosis . reports of blood culture , ascites culture , and cervical swab culture showed gram - positive cocci . initial empiric antibiotics ( ampicillin / sulbactam 2 g/1 g every 6 hr ) switched to penicillin g ( 300 mu every 4 hr ) and metronidazole ( 500 mg every 8 hr ) . primary peritonitis caused by streptococcus pyogenes is uncommon and rarely diagnosed in a healthy person without underlying diseases . spontaneous bacterial peritonitis due to streptococcus pyogenes in a cirrhotic child was reported in korea ( 1 ) but s. pyogenes peritonitis in a healthy person has not yet been reported in korea . therefore , this is the first case of primary peritonitis due to streptococcus pyogenes in a healthy person in korea . it is reported that most instances of spontaneous primary peritonitis are due to streptococcus pneumoniae ( 2 ) . there are a few cases describing s. pyogenes peritonitis in healthy women , even though the entry site of s. pyogenes in peritonitis is not uncertain . ( 3 ) suggested that in some women , s. pyogenes in peritonitis may be via the genital tract , despite lack of gynecological symptoms even though many studies have shown an absence of s. pyogenes as normal flora of the female genital tract . the hematogenous route may be an alternate , possibly from pharyngeal or cutaneous primary sites ( 3 ) . in this case , ascending genital infections was considered to be the entry site because of the positive cervical swab culture . the majority of primary peritonitis is diagnosed retrospectively when secondary causes are excluded after surgical approach ( 4 ) . preference for laparoscopy or laparotomy is established by the surgeon 's choice and laparotomy is predominantly performed in previous cases . however , farooq and ammori ( 5 ) claimed that laparoscopy could be used as a diagnostic tool in the management of generalized peritonitis . we chose laparoscopy as a diagnostic tool in this case because no significant abnormalities suggesting secondary peritonitis were found on abdominal ct . the severe infection of s. pyogenes requires a high index of suspicion , prompt diagnosis , and rapid initiation of appropriate antibiotics ( 6 ) . in this case , reports of blood culture , abdominal ascites culture , and cervical swab culture showed gram - positive cocci , so ampicillin / sulbactam was applied . after final result of culture confirmed s. pyogenes , antibiotics were switched to penicillin g and metronidazole . it is reported that some s. pyogenes serotypes are more commonly associated with invasive group a streptococcal disease than other gas isolates ( 7 - 9 ) . we did not obtain the serotype of s. pyogenes , so further study on s. pyogenes serotypes in primary peritonitis is required . in summary , we report the first case of primary peritonitis in korea in a young , healthy woman due to s. pyogenes . the port of entry was thought to be via the genital tract , despite no local symptoms . laparoscopy as a diagnostic tool was performed in this case and we recommend laparoscopy over laparotomy when no significant abnormalities suggesting secondary peritonitis are seen on abdomen computed tomography . an appropriate diagnostic approach and prompt antibiotic therapy is essential in primary gas peritonitis .

we describe the first case of primary peritonitis in korea of a healthy person due to streptococcus pyogenes . in the absence of comorbid conditions , such as liver cirrhosis , immunosuppression , or nephrotic syndrome , primary peritonitis is uncommon in a young healthy woman . abdomen computed tomography revealed ascites in the lower abdomen and peritoneal enhancement suggesting peritonitis . in diagnostic laparoscopy , purulent ascites was found in the pelvic cavity but both ovaries and fallopian tubes were intact . there were no intra - abdominal abnormalities such as bowel perforation , appendicitis , or necrosis . the reports of blood culture , ascites culture , and cervical swab culture confirmed s. pyogenes . after use of antibiotics , the patient was cured and discharged without sequelae .

central serous chorioretinopathy ( cscr ) is a chronic idiopathic condition affecting young to middle - aged patients with men affected more commonly than women . it is characterized by serous detachment of the neurosensory retina , with focal or multifocal areas of leakage of the retinal pigment epithelium ( rpe ) affecting the macular area . the exact cause of cscr is not clearly understood . nevertheless , it is associated with psychological stress and systemic corticosteroid therapy , which causes hyperpermeability of choriocapillaries , leading to dysfunctional degeneration of the rpe [ 1 , 2 ] . a 23-year - old man presented with a sudden decrease of visual acuity of the right eye ( re ) 4 days after rhinoplasty for a deviation of the nasal septum . general anesthesia was induced with propofol 2 - 5 mg / kg i.v . and sufentanil 0.5 g / kg i.v . no corticosteroids , antibiotics or other postoperative treatment was used . at presentation , best - corrected visual acuity of the re was 6/9 with metamorphopsia . fundus examination revealed a well - delineated serous elevation of the macula and fluorescein angiography ( fa ) showed a focal rpe leakage . the optical coherence tomography ( oct ) sections through the macula depicted a mild neurosensory retinal detachment with an increase in retinal thickness equal to 245 m ( fig . two weeks later , without any treatment , a gradual improvement of the symptoms was assessed . one month after the operation , visual acuity of the re returned to 6/6 and the neurosensory retinal detachment disappeared . also , fa revealed the disappearance of rpe leakage and the retinal thickness of the macula measured with oct was 183 m ( fig . cscr is a multifactorial disease with controversial etiopathology and it is usually associated with steroid use , pregnancy and stress . the exact mechanism by which glucocorticoids are involved in the development of cscr is still unknown . it is possible that a constitutionally determined susceptibility of the posterior blood - retinal barrier must be present so that , under the effect of exogenous or endogenous glucocorticoids , cscr develops . found elevated values of urinary free cortisol in patients suffering from cscr not exposed to exogenous glucocorticoids and without biological signs of endogenous cushing 's syndrome . psychologic stress has been known to produce hypothalamic - pituitary - adrenal axis abnormalities like endogenous cortical excess , which has been found in some patients with cscr . in fact , the use of imidazole derivatives like oxy- and xylometazoline , which directly stimulate alpha - receptors and are more alpha-2-selective , are associated with the manifestation of cscr [ 6 , 7 ] . in our case , the use of xylometazoline may have enhanced the post - traumatic stress and induced adrenergic stimulation leading to an increase in the choroidal circulation and alteration of the pump action of the rpe , which is related with the development of the cscr . however , the simultaneous increase in choroidal resistance has no effect on choroidal blood flow , which will be regulated to be maintained constant . in summary , we present a case of cscr after rhinoplasty , which to the best of our knowledge has not been previously reported . this case shows a possible association between the postoperative stress or the use of xylometazoline and cscr and widens the spectrum of drugs associated with the occurrence of the disease .

we report a case of central serous chorioretinopathy after rhinoplasty for deviation of the nasal septum in a 23-year - old caucasian man . the patient complained of deterioration of vision in the right eye 4 days after rhinoplasty . at presentation , visual acuity of the right eye was 6/9 with metamorphopsia . fluorescein angiography revealed a focal retinal pigment epithelium leakage and optical coherence tomography an increase in macular thickness to 245 m . the left eye was normal . one month after the operation , without any treatment , visual acuity returned to 6/6 , the leakage of the retinal pigment epithelium disappeared and the macular thickness returned to 183 m . to the best of our knowledge , central serous chorioretinopathy after rhinoplasty has not been previously reported . this case report shows a possible association between the postoperative stress and central serous chorioretinopathy . moreover , it widens the spectrum of drugs associated with the occurrence of the disease .

multicentric reticulohistiocytosis ( mrh ) is a rare multisystem syndrome characterized by polyarthritis and papulonodular skin lesions with typical dermal infiltration of histiocytes and multinucleated giant cells . the disease may involve the skin , tendon sheath , synovium , bone , liver , salivary gland , kidney , lymph node , heart and lung . in mrh , an association with hyperlipidemia ( 3058% ) , a positive skin tuberculin test ( 1250% ) , systemic vasculitis and autoimmune disease has been described . of utmost clinical importance such an association has been documented in up to 28% of all reported cases in the world literature , the most common being bronchial , breast , stomach , and cervical carcinomas . here , we report , for the first time , a case of mrh presenting with liver carcinoma , thus highlighting the association of mrh with malignant disease . a 61-year - old man presented with a 4-month history of a nonpruritic rash , which started on his forearm and face after sun exposure and subsequently spread to the ear , scalp and upper chest , after which asymptomatic skin lesions appeared on the lateral margin of his finger . the patient denied any history of photosensitivity but complained of low - grade fever and painful swelling of the joints in his wrist , knee and ankle , accompanied by restricted activity for 1 month . he had a sensation of a foreign body in his pharynx with difficulty in swallowing for about 1 month . the patient also had a history of hepatitis b , which was diagnosed in the 1970 's , and severe knee joint pain with bony spurs , which were confirmed by x - ray , for at least 2 years . treatment with hydroxychloroquine and thalidomide for solar dermatitis in other hospitals showed no therapeutic effect . physical examination showed a confluent erythematous eruption affecting his scalp , face , neck and the extensor aspect of his forearm , which was characterized by ruby - red , translucent and grouped papules ( fig . red nodules could also be found on the dorsum of the nose , lateral area of the fingers and tongue tip ( fig . an extensive laboratory evaluation , including white cell count , urinalysis , stool studies , renal function , electrolytes , cholesterol and triglycerides , erythrocyte sedimentation rate , antistreptolysin o level , rheumatoid factor and antinuclear antibody were normal . u / l ) , alt 125 u / l ( < 50 u / l ) , alp 159 u / l ) , ggt 100 u / l ( 345 u / l ) , total bilirubin 30 mol / l ( 3.420.4 mol / l ) , tba 21 mol / l ( < 6.8 mol / l ) and total protein , albumin and globulin were normal . hepatitis index studies showed hbsag(+ ) , hbsab < 0.2 iu / l ( > 10 iu / l ) , hbeab(+ ) , hbsab < hbeag( ) , hbeab(+ ) , hbcab(+ ) , hbcigm( ) , havigm( ) and hcv( ) . the afp level was significantly elevated ( 6,820 g / l , normal range < 10 type - b ultrasonic examination revealed a solid tumor ( 91 69 mm ) on the right lobe of the liver , which was suspected to be a small hepatocellular carcinoma . five - fu , oxaliplatin and thp were used as anticancer drugs . the afp level after the interventional treatment was 1,027.5 g / l , and the tumor mass decreased to 67 66 mm . the cutaneous symptoms improved significantly within 1 month ( several large nodules disappeared immediately ; later , the erythematous rash and grouped papules resolved and the patient 's arthralgia and swelling of joints gradually improved ) . skin biopsy on the right forearm showed infiltration of the dermis with polymorphic histiocytes and giant cells with the histiocytic marker cd68 was positive , as were leukocyte common antigen , hla - dr , lysozyme and vimentin ( fig . langerhans cell markers ( s-100 and cd1a ) were negative and the histological appearances were consistent with mrh . mrh , often referred to as lipoid dermato - arthritis , was first reported in 1937 by weber and freudenthal . mrh is so rare that fewer than 300 cases have been reported in the literature . in 1969 , barrow and holubar first raised the question of whether there was an association between mrh and malignancy . snow and muller reported a malignancy rate of 25% in 133 cases of mrh reported in the literature until 1994 . eleven [ 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 ] of the 66 cases we reviewed ( english - language literature from 1995 until now ) had cancer and were evaluated for the following parameters : age , sex , type of neoplasm at presentation , duration preceding or following the diagnosis of cancer that mrh developed and treatment response of both the tumor and mrh . the results are presented in table 1 ( including the present case ) . for most of these cases ( including the present case ) , the onset of malignancy and mrh occurred within approximately 3 years , and in some cases , the onset of mrh also occurred in close proximity to the time of recurrence of a previously diagnosed malignancy [ 13 , 15 ] . this is the first case report of mrh with liver carcinoma . although the etiology of mrh is unknown , the possibility that mrh reflects a constellation of symptoms seen within paraneoplastic syndrome is supported by the frequency of associated neoplasm ( 18% based on our review ) , the remission of the lesions after tumor treatment ( 6 of 12 cases [ 6 , 7 , 8 , 10 , 11 , 12 ] ) and the onset of mrh just prior to the relapse of the neoplasm ( case no . 8 of table 1 ) .

we report a unique case of multicentric reticulohistiocytosis ( mrh ) associated with liver carcinoma . a 61-year - old man presented with a 4-month history of nonpruritic , generalized , ruby - red papules and nodules , accompanied by fever , joint swelling and difficulty in swallowing . skin histology showed polymorphic histiocyte infiltration with typical ground glass cytoplasm . further immunohistochemical studies characterized the lesions as positive for leukocyte common antigen , hla - dr and cd68 . the patient had a history of hepatitis b , and systemic examination , including carcinoma index and type - b ultrasonic examination , revealed high levels of afp and a solid tumor , which was considered malignant , localized on the right lobe of the liver . treatment of the liver carcinoma resulted in a significant improvement of the skin symptoms . this is the first case study to report an association between mrh and liver carcinoma . a review of the english - language literature reveals the close linkage between mrh and malignancy . all patients with mrh should be evaluated and monitored carefully to determine the underlying neoplasm .

an 83-year - old woman presented with sudden visual loss that had developed in both eyes ( oculus uterque , ou ) the previous day . she did not complain of any associated headache , scalp tenderness , jaw claudication or constitutional symptoms such as weight loss , fever , malaise or sweats . her visual acuities included perception of light in the right eye ( oculus dexter , od ) and perception of hand motion in the left eye ( oculus sinister , os ) . fundus examination revealed mild retinal arterial narrowing and chalky - white disc swelling ou ( fig . the results of the hardy - rand - rittler test and ishihara test showed total dyschromatopsia ou . although electroretinography findings were within normal limits , visual evoked potentials showed delayed p100 latency ou . brain magnetic resonance imaging scans and angiography results showed diffuse bilateral stenosis of vertebral arteries and external carotid arteries without significant intracranial vessels stenosis . the crp level , esr and platelet count were elevated and measured to be 5 mg / dl ( upper normal limit , 0.5 mg / dl ) , 55 mm / h ( upper normal limit , 20 mm / h ) , and 510 k/l ( upper normal limit , 400 k/l ) , respectively . following a presumptive diagnosis of silent gca - associated aaion ou , the patient was hospitalized and treated with intravenous 250 mg methylprednisolone every 6 hours for 3 days . biopsy of the left temporal artery was performed , and 3 cm of the temporal artery was acquired . lymphocytes , epithelioid histiocytes , and multinucleated giant cells had diffusely infiltrated into the entire vessel wall , especially in the arterial wall media ( fig . after 7 days , the patient 's visual acuity os improved slightly to being able to count fingers . the crp level and esr decreased to 1.07 mg / dl and 30 mm / h , respectively . oral prednisolone therapy was slowly tapered down from 60 mg per day , and steroid treatment was maintained with deflazacort 30 mg per day . however , after 4 months , the patient 's visual acuities deteriorated to no light perception od and light perception os . gca should be strongly suspected when patients greater than 50 years of age present with headaches . the incidence of gca in scandivian countries and north america ranges between 6.9 and 32.8 per 100,000 . however , the occurrence of gca is rare in african americans , hispanics , and asians [ 8 - 10 ] . there have only been a few reports of gca among asians [ 5,6,11 - 15 ] . in japan , a nationwide gca survey revealed an extremely low prevalence of 1.47 per 100,000 population , which is approximately 1 / 140 of that reported in the us . pereira et al . reported that gca was seen 20 times less frequently in asians than in caucasians . . stated that , for over a period of 22 years , only 7 patients were diagnosed with gca by temporal artery biopsy in a tertiary medical center in saudi arabia . the incidence of gca in asians was far lower than that in caucasians ; however , the incidence is now increasing in the asian population . after 36 years of no reported cases of gca , in 2010 , aui - aree et al . reported 4 gca cases in thailand . cullen et al . noted that of the 7 biopsy - confirmed gca cases reported over the past 10 years in singapore , 3 were reported in 2009 . this trend may be associated with an increase in the maximum life span of the asian population . suspected diagnosis of gca by rheumatologists and ophthalmologists , along with extensive laboratory tests , may be other important factors . however , a nation - wide epidemiologic study would be needed to clarify the association of life span and gca incidence in asians . in korea , only a few biopsy - confirmed gca cases have been reported ; however , there have been no gca - associated aaion cases so far . to our knowledge , this is the first biopsy - confirmed report of gca - associated aaion in korea . our patient was diagnosed with silent gca , and the clinical features overlapped with non - arteritic aion . when asian patients aged > 50 years present with acute visual loss and disc swelling and no other symptom , non - arteritic aion accounts for more than 90% of these cases . in such circumstances , laboratory parameters , such as esr , crp level and platelet count can serve as indicators in the diagnosis of gca . reported that the crp level has a sensitivity of 100% for gca , and the combination of crp level and esr has a specificity of 97% . in a large population - based cross - sectional study , documented that the odds of a positive biopsy were 1.5 times greater with an esr of 47 to 100 mm / h , 5.3 times greater with a crp of > 2.45 mg / dl , and 4.2 times greater with a platelet count of > 400,000 l . the above 3 parameters were elevated in our patient ( crp , 5 mg / dl ; esr , 55 mm / h ; and platelet count , 510 k/l ) . these test results are known to be normal in non - arteritic aion . in conclusion , although this disease is rare in asians , gca - associated aaion should be considered when an elderly patient presents with sudden visual loss and disc edema . gca should be suspected and laboratory tests should be performed , even in the absence of typical symptoms .

giant cell arteritis ( gca ) is a rare disease among asians . arteritic anterior ischemic optic neuropathy , which accompanies gca , has not yet been reported in koreans . diagnosis of gca is difficult if typical symptoms other than visual loss are absent . here , we report a case of an 83-year - old korean woman presenting with sudden visual loss in both eyes ( oculus uterque , ou ) . her visual acuities included perception of light in the right eye ( oculus dexter , od ) and perception of hand motion in the left eye ( oculus sinister , os ) . the results of the hardy - rand - rittler test and ishihara test showed total dyschromatopsia ou . the goldmann perimetry test revealed a total field defect od and paracentral island os . fundus examination revealed chalky - white disc swelling ou . other systemic symptoms and signs were unremarkable . the erythrocyte sedimentation rate , c - reactive protein and platelet count were highly elevated . temporal artery biopsy revealed multiple lymphocytes and multinucleated giant cells in the arterial media layer . to our knowledge , this is the first report of gca in a korean that has been confirmed with temporal artery biopsy . in conclusion , silent gca can occur in koreans , and hence , elderly patients presenting with chalky - white disc swelling , and corresponding laboratory findings must be evaluated for gca .

sixty - three fecal samples were collected from laboratory ferrets ( mustela putorius furo ) at the national institute of infectious diseases , tokyo , japan , on may 24 , 2013 . these ferrets had been imported from a farm in the united states for influenza research 7 days before sample collection . fecal specimens were diluted with 10 mmol / l phosphate - buffered saline to prepare a 10% suspension , shaken at 4c for 1 h , and clarified by centrifugation at 10,000 g for 30 min . the supernatant was passed through a 0.45-m membrane filter ( millipore , bedford , ma , usa ) , and stored at 80c until use . rna was extracted by using the magna pure lc total nucleic acid isolation kit ( roche applied science , mannheim , germany ) according to the manufacturer s recommendations . reverse transcription was performed by using the superscript ii rnase h reverse transcription procedure ( invitrogen , carlsbad , ca , usa ) and primer tx30sxn as described ( 14 ) . ferret hev rna was detected by using a nested , broad - spectrum reverse transcription pcr ( 15 ) . sequences were similar to those detected in ferret serum samples in the united states ( 11 ) , which suggested that the laboratory ferrets were infected in the united states and then transported to japan . rna from 2 ferret hevs was randomly selected , and the full - length genome was amplified by using reverse transcription pcr with primers based on nucleotide sequences derived from strains from the netherlands and united states ( table 1 ) . sequence of the 5-terminal noncoding regions of the genome was determined by using rapid amplification of cdna ends kits ( invitrogen ) according to the manufacturer s instructions . all pcr products were purified by using the qiaquick pcr purification kit ( qiagen , valencia , ca , usa ) and cloned into the ta cloning vector pcr2.1 ( invitrogen ) . nucleotide sequencing was conducted by using an abi 3130 genetic analyzer automated aequencer ( applied biosystems , foster city , ca , usa ) . * values in parentheses indicate positions of the primer corresponding to the entire genome of hepatitis e virus ( jn998607 ) isolated from ferret . both ferret hev genomes consisted of 6,820 nt and a poly ( a ) tail ( genbank accession nos . ab890001 and ab890374 ) , and nucleotide sequence identity was 99.6% . genomic structure of strains from the united states was similar to that of 2 strains from the netherlands . the amino acid alignment of orf2 indicated that ferret hev orf2 has an additional 6 amino acids at the n terminus . however , because the seventh codon is aug , it is unclear which codon was used for the orf2 translation initiation . the orf1 of strains from the united states encodes 1,589 aa , which is 7 aa shorter than orf1 of both strains from the netherlands . in addition , the ferret hev strains from the united states have 2 aa insertions between amino acid residues 596597 ( thr ) and 631632 ( ile ) and 9 aa deletions in amino acid residues 645653 ( cys - leu - arg - ser - ser - pro - lys - pro - pro ) , which corresponds to those of strains from the netherlands . similar to ferret hev from the netherlands , an additional putative 183-aa orf 4 ( nt 30581 ) was found in strains from the united states . ab890375ab890379 ) showed that nucleotide identities among them were 98.9%99.5% , which indicated that genetically similar ferret hev strains had circulated at the ferret farm in the united states . nucleotide and deduced amino acid sequence identities between ferret hev from the united states and other hevs are shown in table 2 . the entire genome of strains from the united states shared relatively high nucleotide sequence identities ( 82.4%82.5% ) with strains from the netherlands . these trees showed that although strains from the united states were closely related to strains from the netherlands , they formed a new and distinct cluster ( figure ) . we observed similar phylogenetic clustering when we analyzed nucleotide sequences of orf1 and orf3 separately . although we can not conclude whether ferret hev from the united states is a new genotype , these results indicated that there is genetic variety in ferret hev . researchers should also bear in mind that some laboratory ferrets are contaminated with ferret hev . phylogenetic relationships among genotypes 14 and wild boar , rabbit , rat , avian , bat , and ferret isolates of hepatitis e virus . phylogenetic trees with 1,000 bootstrap replicates were generated by using the neighbor - joining method ( njplot 2.3 , http://njplot.sharewarejunction.com/ ) based on a ) the entire genome and b ) open reading frame 2 . we amplified the entire genome of 2 ferret hev strains isolated from laboratory ferrets imported from the united states . nucleotide sequence comparisons showed that 2 ferret hev strains from the united states had high ( 99.6% ) identity and shared 98.6%100% identities with partial sequences of orf1 that were detected in the united states ( 11 ) , which indicated that genetically similar ferret hev was circulating in laboratory ferrets . although nucleotide sequence identities of the entire genome for strains from the united states and the netherlands was 82.4%82.5% , orf2 showed relatively high amino acid identities ( 94.2%94.8% ) , which suggested that isolated from the united states and the netherlands share similar antigenicity . ferret hev like particles derived from 1 of the isolates from the netherlands were cross - reactive with serum from hev - infected laboratory ferrets in the united states ( 11 ) . in conclusion , we isolated and identified 2 ferret hev strains from laboratory ferrets imported from the united states .

the complete genome of hepatitis e virus ( hev ) from laboratory ferrets imported from the united states was identified . this virus shared only 82.4%82.5% nt sequence identities with strains from the netherlands , which indicated that the ferret hev genome is genetically diverse . some laboratory ferrets were contaminated with hev .

cough is one of the most distressing symptoms in cancer patients with lung or mediastinal metastasis or due to complications of cancer treatment . twenty to 42% of cough do not respond to conventional medications and are referred to as refractory cough and these are often a medical challenge . it shows features such as abnormal throat sensation ( laryngeal parasthesia ) , increased cough sensitivity to tussigens ( hypertussia ) , and cough triggered by nontussive stimuli such as cold or talking ( allotusia ) . gabapentin is known to be effective in treating neuropathic pain with central sensitization and findings from the present case predicts its effectiveness in treating refractory cancer related cough . it further affirms the fact that vagal neuropathy may be associated with refractory chronic cough . a 15-year - old boy , who had previously been diagnosed with malignant spindle cell sarcoma , was referred to the department of palliative care and psycho - oncology for chronic refractory cough . following histopathological diagnosis of spindle cell sarcoma of right lower limb in the year 2014 , he had undergone surgery followed by radiotherapy for local control . in may 2015 , he presented to the clinical oncology department with a history of persistent dry cough of 6 months duration . a computed tomography scan of thorax [ figure 1 ] revealed a large heterogenous hypodense soft tissue mass ( 10.5 cm 10.3 cm ) in the right lower lobe with extension to the left atrium through the right inferior pulmonary vein . in addition , there were enlarged right paratracheal , subcarinal , and hilar lymph nodes . computed tomography scan of thorax showing the mass in the right lower lobe with extension to the left atrium through the inferior pulmonary vein the child had persistent cough for the duration of 6 months . the cough intensity on a numerical rating scale ( nrs ) was 10 ( out of 10 ) . each bout of cough lasted for 30 s and there were 20 such bouts in a day . cough was severe in the morning and was triggered by activities that involved walking , talking , or exposure to the cold environment . his eastern cooperative oncology group ( ecog ) performance status score at the time of presentation was 3 . a trial of codeine phosphate started by the primary treating team did not reduce cough but instead caused excessive drowsiness and giddiness due to which he had to stop the medicines . patient was started on gabapentin 50 mg 3 times a day and advised to increase the dose by 50 mg every 3 days until response was obtained or the child felt drowsy ( in the latter case , the drug dose would be decreased ) . the patient responded significantly well with 200 mg / day of gabapentin after 1 week without any side effects such as drowsiness or giddiness and he continues to improve . there was a decrease in the cough intensity to 2 ( out of 10 on nrs ) and both cough duration and bouts ( 2/day ) . the child is now able to enjoy his routine life and socialize with friends and relatives . patients with primary or metastatic cancer of the lung often present with distressing symptoms such as breathlessness and cough which significantly impacts their quality of life . there are multimodal options for treatment in cancer related cough which include corticosteroids , bronchodilators , brachytherapy , laser therapies , and opioids . our patient had an extensive mass crossing the mediastinum which precluded radiation to the mass as this involved significant risk to the mediastinal structures . opioids are commonly used in cancer patients for the management of pain , cough , and breathlessness . opioids act by stimulating the mu receptors in the cough centers of the brain and helps in suppressing the cough . in one study , slow release morphine sulfate showed a significant improvement in the cough related quality of life as compared to placebo and this was not associated with cough reflex sensitivity which is consistent with central action of morphine . the treatment , on the contrary , caused significant drowsiness and giddiness affecting his quality of life . cough reflex is mediated by the stimulation of the vagal primary afferent nerve distributed along the tracheobronchial tree . thus , rapidly adapting receptors of the vagal afferents are known to be evoked by mechanical stimuli and deformity in the airway epithelium , which results in cough . thus , an unresectable mass in the mediastinum acts as a constant source of irritation for the airway which in turn triggers cough . we used gabapentin in our patient with successful control in cough intensity , duration , and frequency . gabapentin is a lipophilic structural analog of the neurotransmitter gamma aminobutyric acid which is proven to have a central action . it is known to act on the alpha 2 and delta receptor of calcium channel inhibiting the release of neurotransmitters such as substance p , a tussigenic agent , and possibly inhibits the n - methyl - d - aspartate receptors . recent trials have shown the superiority of gabapentin in reducing the frequency and intensity of cough . peripheral cough sensitivity to capsaicin was not changed by gabapentin which proves its central effect . in the same study , there was a significant improvement in cough related quality of life in the gabapentin arm . the common side effects of gabapentin include dizziness , fatigue , headache , and confusion which was not reported by our patient . there is , thus , a need to explore the use of neuromodulatory agents in symptom conditions which are known to have a neural origin such as cough , pruritus , and hiccoughs .

vagal sensory neuropathy or vagal hypersensitivity has been implicated in the pathophysiology of chronic cough . earlier reports have shown gabapentin to be effective in sensory laryngeal neuropathy and symptom conditions that have a proven neural origin . we present a case report of a patient with chronic refractory cough due to a soft tissue mass in the lung that caused compression of the mediastinal structures . the patient was successfully treated with gabapentin with reduction in the cough intensity , duration , and frequency .

this may be spontaneous or secondary to mechanical factors like vigorous scratching or combing the hair . a 19-year - old female patient presented with short , rough , and unruly hair on the scalp since childhood . patient gave a history of knots getting caught in fine - toothed comb whenever the scalp was combed with breakage of few hairs . there was no history of excessive cosmetic use or parlor activities for hair care . on examination , the scalp hairs were dry , curly , and short in length up to the nape of neck , transverse split ends ( trichoschisis ) were seen in majority of hairs [ figure 1 ] . microscopic examination of the hair shaft revealed knotted portions of hair ; no other shaft abnormalities were noted except for trichoschisis at the distal portion of the shaft [ figure 5 ] . short curly hair on the scalp small nodules on the distal portion of hair shaft sparse hair in the temporal region trichonodosis and trichoschisis in light microscopy there are two types , one rare variety of unknown etiology involves abnormally growing scalp and body hair with spontaneous trichonodosis which is predisposed to splintering and fracturing . the second commoner type , encountered in patients with normal scalp and body hair , is considered secondary to mechanical forces such as combing and brushing . the hairs in this type are dry and curly , but no abnormality in structure or growth pattern occurs . rarely , more than one knot is found on a single hair and most were a simple half hitch knot . when the hair of subjects with trichonodosis was combed or brushed , knots were observed to catch in fine toothed combs and to a lesser extent , in brushes . in those with extensive trichonodosis , breakage usually occurred at the knot , but proximal breakage and several uprooted telogen hairs were also seen . kinky hair tends to be flat in cross section , whereas curly or straight hair is oval to round . these flat or ribbon - shaped hairs do not lie flat on a level surface but remain spiral . this recoiling may lead to an entanglement which , by chance , forms a knot that results in trichonodosis . mechanical factors such as scratching , combing , washing or friction may produce tangling and knotting of hairs . the pubic and thigh regions are most frequently involved since curliness of the hairs in those areas predisposes them to knotting . if the knots are pulled excessively tight , transverse splintering of the hair shaft and simulation of trichorrhexis nodosa may result . these knots resemble the ova of body , head and pubic lice and light microscopic examination is necessary for differentiation . the nodules in trichonodosis are located distally , whereas the ova are located in the proximal shaft . acquired type of trichonodosis due to mechanical factors is a common occurrence but reported less in literature . spontaneous type of trichonodosis associated with abnormal scalp and body hair is a rare occurrence . this type starts at an early age and hair breaks away after a particular length due to the fracture of hair shaft at the knot . trichonodosis must be considered when patients with kinky hair complain of hair loss or breakage , and the cause is not apparent .

trichonodosis is characterized by knotted hair on the distal portion of the hair shaft . this may be spontaneous or secondary to mechanical factors like vigorous scratching or combing the hair . we report a case of spontaneous trichonodosis with abnormal scalp and body hair .

it is a relatively rare odontogenic lesion which exists either as a cystic or a solid variant and is characterised by varied clinical , radiographical and biological features . the central ccot appears as a unilocular or multilocular destructive radiolucent lesion containing irregular calcifications . various terminologies used for ccot include calcifying odontogenic cyst , gorlin cyst , calcifying ghost cell odontogenic tumour and epithelial odontogenic ghost cell tumour , keratinizing calcifying odontogenic tumour . in 2005 , the world health organization classification of head and neck tumors reclassified ccot as an odontogenic tumor and gave it the name of calcifying cystic odontogenic tumor . is a developmental cyst of odontogenic origin and constitutes about 0.37% to 2% of all odontogenic tumours . ccot are cysts of primordial origin and not associated with crown of any impacted tooth . ccot may occur as a central lesion or as a peripheral lesion ( although rare ) . a comprehensive review of the available literature relating to malignant transformation of ccot was undertaken using medline , pubmed , google scholar and scopus in all languages . we used the following keywords for searching : calcifying cystic odontogenic tumour , malignancy and ghost cell odontogencic carcinoma from 20032013 . we also used the related articles feature of pubmed to identify further references of interest within the primary search . these articles were obtained , and from their references lists , pertinent secondary references were also identified and acquired . ghost cell odontogenic carcinoma ( gcoc ) is a rare tumour which is a malignant counterpart of ccot . ghost cell odontogenic carcinoma may arise as a denovo tumour or from previously existing ccot . one third cases of ghost cell odontogenic carcinoma are reported to be derived from a preexisting ccot and malignant transformation may take several years . however some of the ghost cell carcinoma may develop without history of ccot [ 1214 ] . recurrent ccot and gcoc are more common in the maxilla [ 8 , 15 ] . painful swelling with local paraesthesia is the most common symptom of ghost cell odontogenic carcinoma . some authors reported of infiltrative growth , root resorption and tooth displacements in cases of gcoc [ 16 , 17 ] . ct scan demonstrated bone expansion and bone destruction with irregularly shaped calcified inside the lesion . magnetic resonance images showed a mass with high signal intensity . li et al . reported an ameloblastoma - like epithelia with prominent features being presence of lots of ghost cells , dysplastic uncalcified dentin or osteodentin . increased nuclear / cytoplasmic ratio with 12 nucleoloi and atypical mitotic figures were also reported . according to motsugi et al . , tumour cells densely proliferates the epithelial component and the nucleus of tumour cells were enlarged and variable in size . immunohistochemical analysis of gcoc by motossugi et al . revealed that 70% of tumour cells were reactive for p53 and ki-67 index was 4% to28% . expression of ki-67 , mmp-9 and timp-1 was stronger in gcoc when compared to ccot . mmp-9 in stroma is associated with invasive ability of ccot and gcoc and ki-67 is associated with increased cellular proliferation . according to gomes et al . , there is a variable expression of syndecan-1 in stellate reticulum , stromal cells and basal cells of ccot and gcoc and might be associated with the biology of these tumors . a total of 8 cases have been reported in the literature from 2003 - 2013 where ghost cell odontogenic carcinoma has probably developed from ccot . these cases are enlisted in table 1 , [ 8 , 13 , 14 , 1618 , 21 , 22 ] . of the 29 patients diagnosed , 5 died of local recurrence and metastasis to brain and lung has been reported . the most commonly employed treatment was surgery with wide excision . in some cases radiotherapy and after reviewing the literature we conclude that recurrent and long standing case of ccot can undergo malignant transformation . gcoc , the malignant form of ccot can metastasize and can even lead to deaths . so it is mandatory to follow up the patients with ccot for possible eventual development of malignant counterparts .

calcifying cystic odontogenic tumour ( ccot ) has been classified as an odontogenic tumour . ghost cell odontogenic carcinoma ( gcoc ) is the malignant counterpart of ccot . this paper aims to review the literature regarding malignant transformation of ccot.a literature search was done via the national library of medicine pubmed interface , searching for articles relating to malignant transformation of ccot . from these articles , references were obtained , and from their references lists , pertinent secondary references were also identified and acquired.after reviewing the literature , we found 26 cases of gcoc which developed from ccot . malignant transformation of ccot was seen more commonly in the maxilla . histologically , changes such as increased nuclear / cytoplasmic ratio , atypical mitotic figures have been reported after malignant transformation . immunohistochemical analysis has shown an increased expression of ki-67 and p53 in tumour cells.malignant transformation of ccot , although rare , mostly takes place in recurrent and long standing cases .

chest physiotherapy is routinely employed as a prophylactic measure prior to major surgery and postoperatively to prevent respiratory complications such as atelectasis and pneumonia.1 at present , only limited evidence is available for some of the physiotherapeutic techniques used in patients with copd.2,3 physiotherapy treatment enhances sputum evacuation4 and can be applied as a single technique but usually a combination of techniques is applied to patients with copd . intrapulmonary percussive ventilation ( ipv ) is a ventilatory technique that uses a device to deliver small bursts of high - flow air into the lungs at high rates , superimposed upon the spontaneous breathing pattern . this causes airway pressures to oscillate between 5 and 35 cm h2o and the airway walls to vibrate in synchrony with these oscillations . a unique sliding venturi , called a phasitron , which is powered by compressed gas at 0.6 to 6 bar , generates these oscillations in the range of 80 to 650 cycles per minute.5 although several studies have addressed the physiological effects of ipv when used in copd patients , there is need for confirmation of its clinical effectiveness . previously , the effects of ipv in copd patients were assessed using lung function parameters , arterial blood gases , and duration of hospitalization . recently developed assessment techniques may give new insights into the effectiveness of airway clearance techniques . one promising new technique is computational fluid dynamics applied to the three - dimensional ( 3d ) images made by ct scanning ; this technique allows evaluation of flow and resistance of separate parts of the lung.6,7 in the present study , this novel imaging was used , in addition to more conventional outcome parameters , to visualize the effects of a single ipv treatment in copd patients . five moderate to severe copd patients ( three females and two males ) with global initiative for chronic obstructive lung disease stages 3 to 4 , who were hospitalized for an acute exacerbation , were included in this study . tests were performed before and after ipv treatment in a specific order to minimize the influence on other tests . for pre - treatment tests , the sequence was set as follows : forced oscillation technique ( fot ) in upright sitting and supine positions , conventional lung function measurements ( including spirometry and body plethysmography measurements ) , in- and expiratory muscle strength , diffusion capacity , arterial blood gases sampling , and finally a 3d low - dose ct scan was taken within 1 hour prior to the ipv treatment . patients were asked to score their dyspnea on a borg dyspnea scale before and after the treatment . no significant changes were evident in the spirometric or body plethysmographic indices due to ipv treatment . respiratory muscle strength after a single session tended to decrease , but the changes in inspiratory and expiratory muscle strength were not significantly different . we observed a small but nonsignificant change in the dlco / va ( diffusion capacity of carbon monoxide ) : corrected for alveolar volume ratio ( p = 0.066 ) . four out of five patients coughed up one or more flumes during the ipv treatment . all patients reported that they subjectively felt better after the treatment ( the change in borg dyspnea score , however , was not significant : p = 0.083 ) . arterial saturation tended to increase after a single ipv session but the changes were not statistically significant ( sao2 at baseline and post - ipv treatment : 95% 3% and 96% 1% , respectively ; p = 0.066 ) . 3d airway reconstructions based on the computed tomography ( ct ) images were made for each patient . for example , some peripheral airways that were blocked before the treatment ( possibly due to mucus plugging ) were reopened after the treatment . some airway branches were opened up after ipv and other airways were closed after a single ipv session ( figure 2 ) . the resistance measurements by body plethysmography and fot both showed a tendency toward an increase in airway resistance . the increase , however , was not statistically significant ( p = 0.109 and p = 0.080 alveolar volume for body plethysmography and fot , respectively ) . computational fluid dynamics ( cfd ) were used to calculate the local resistances for the different branches in the airways . specific airway resistance , calculated for the local changes in airway resistance , showed changes after the ipv treatment ; however , none of the changes were statistically significant . although the 3d computer models showed no changes in the overall airway resistance , local changes were observed , as indicated in figure 2 . branches with airway blockage in the preintervention scan were reopened in the post scan . in these branches , airway resistance seemed to decrease , possibly due to movement of mucus ; however , this is yet to be confirmed by the use of a control group . table 2 gives an overview of the airway changes observed in the different patients after a single ipv session . although data shown here are not compared with the data of a control group , 3d ct imaging has the potential to evaluate the displacement of mucus plugs and removal of mucus plugs in some copd patients after an ipv treatment . although we saw changes , the use of a control group is needed to confirm our findings . our study focused on the visualization of the short - term effects of a single ipv treatment . we used functional imaging to demonstrate that the airway geometry was changed by the ipv session whereas lung function parameters did not show any significant differences . the sample size in the present study is small ; more patients are needed to verify these findings . the accuracy of ct imaging has been confirmed for the assessment of the bronchodilator response in asthmatic patients8 and for the particle deposition of an aerosol in the lung.9 the usefulness of 3d imaging and cfd processing has also been shown in the assessment of changes in upper airways 10 and small airways.11 for example , cfd can detect changes in airway resistance in patients with asthma.8 one of the main advantages of the cfd method is that this technique allows investigators to make specific models of the patient s airways for use in analyses.12 in our study , we demonstrated that the effect of a single ipv treatment could be evaluated using functional imaging . despite its small sample size , this study demonstrated that local treatment effects could be visualized with 3d imaging of the airways . the resulting models allow a calculation of the change in airway volume and change in airway resistance . the technique can be used for comparison with traditional outcome parameters , which opens up perspectives for evaluation of physiotherapeutic drainage techniques , and may allow standardization and validation of different airway clearance techniques . further research should focus on the relationship between mucus displacement and local changes in airway flow and resistance .

objective : chest physiotherapy enhances sputum evacuation in copd patients . it can be applied as a single technique or as a combination of techniques including intrapulmonary percussive ventilation ( ipv ) . recently developed assessment techniques may provide new insights into the effect of airway clearance techniques.participants:five moderate to severe copd patients ( three females and two males ; mean forced expiratory volume in 1 second of 39.49% predicted ) who were admitted in the hospital for an acute exacerbation were included in this study.methods:a novel imaging technique was used , together with other conventional techniques , to visualize the short - term effects of a single ipv treatment in copd patients.results:no significant changes were noted in the lung function parameters or arterial blood gases measured within 1 hour after the end of the ipv session . computed tomography images detected changes in the airway patency after the ipv treatment compared with before treatment . local resistances , calculated for the three - dimensional models , showed local changes in airway resistance.conclusion:the effects of a single ipv session can be visualized by functional imaging . this functional imaging allows a calculation of changes in local airway resistance and local changes in airway volume in copd patients without affecting conventional lung function parameters .

pars plana vitrectomy ( ppv ) is a proven and basically safe procedure with a low incidence of complications [ 1 , 2 ] . however , serious adverse events can and do occur , the most serious complications being endophthalmitis , hemorrhages and nonarteritic anterior ischemic optic neuropathy ( naion ) , a potentially blinding condition . an investigation in a random sample of 5% of the american medicare beneficiaries over a period of 12 years ( 19942005 ) yielded an incidence of approximately 7% for postprocedural blindness after ppv without an appreciable trend over time . whereas endophthalmitis is widely considered and investigated in the literature concerning post - ppv complications [ 1 , 2 , 3 ] , the incidence of postprocedural loss of vision due to naion appears to be somewhat underreported . following the concept of vascular occlusion as a key factor in sudden blindness , the conventional mainstay for the treatment of postsurgical loss of vision is the systemic administration of corticosteroids and anticoagulants , e.g. acetylsalicylic acid [ 6 , 7 , 8 ] . however , there is no generally accepted , well - proven treatment for naion currently . in the past decade , a number of novel protein - based direct thrombin inhibitors became available for the treatment of diseases associated with undesired blood clotting . one of those agents is dabigatran etexilate , presently approved for the prophylaxis of venous thrombosis after hip or knee arthroplasty and stroke in patients with atrial fibrillation . after all conventional methods of reversing the visual loss in our patient had failed , we resorted to a therapeutic trial of dabigatran etexilate , and the results are reported here . a 46-year - old caucasian female underwent ppv of the right eye with endolaser treatment and c2f6 ( hexafluoroethane ) gas tamponade as per the current guidelines for retinal detachment . after the procedure , the vision of the treated eye was practically lost , and the patient was only able to distinguish hand movements . the afferent pupillary defect ( apd ) was positive , and perimetry revealed a severe loss of the visual field ( fig . the administration of a conventional therapeutic regimen [ consisting of prednisolone ( 100 mg / day for 3 days , 80 mg / day for 3 days , 60 mg / day for 3 days , 40 mg / day for 3 days , and 20 mg / day for 3 days ) and acetylsalicylic acid ( 100 mg ) ] improved visual acuity only marginally to logmar 1,4 ( fig . 3 ) with positive apd . since pronounced corticosteroid side effects ( sleeplessness , facial edema , restlessness , sweating and tachycardia ) burdened the patient and the treatment showed practically no effect , we decided to try to improve her vision with the administration of dabigatran etexilate ( 2 110 mg ) , a novel direct thrombin inhibitor , 24 days after the initiation of the prednisolone / acetylsalicylic acid treatment . promptly after the first administration , visual acuity improved to logmar 1,0 and continued to improve to logmar 0,4 ( fig . perimetry done 26 days afterwards showed a marked improvement in central sensitivity from 7 to 25 and 21 db , respectively ; the nasal part of the visual field also showed an increase in sensitivity ( fig . strictly speaking , the diagnosis of naion requires the exclusion of arteritic alterations , usually by temporal artery biopsy . however , a sudden loss of vision after ocular surgery without signs of retinal detachment or other intraocular alterations strongly suggest ischemic neuropathy , and tentative diagnosis and treatment are warranted . since naion is not a disease as such but rather the common endpoint of a large number of local and systemic conditions , there is no established standard therapy . however , systemic corticosteroids in combination with anticoagulation to reduce swelling and improve venous blood flow are basically accepted modalities . when this approach failed in the present case , we resorted to the off - label administration of a novel direct thrombin inhibitor , resulting in a dramatic recovery of visual function . naion is known to show spontaneous remission in approximately 40% of the cases , but this assumption notwithstanding , improvement of blood flow is an accepted measure whose indication to preserve or restore vision is undisputed . therefore , the striking improvement of vision after the administration of dabigatran etexilate in the present case may warrant a systematic clinical trial of a combination of corticosteroids and dabigatran etexilate in comparison with ( for instance ) warfarin as is common practice under different indications for anticoagulation [ 12 , 13 , 14 ] . however , when ophthalmologists consider the administration of dabigatran etexilate for naion , certain peculiarities and disadvantages of the drug ( such as the lack of an antidote [ 12 , 15 ] ) need to be borne in mind to avoid potential hazards .

a 46-year - old caucasian female underwent pars plana vitrectomy ( ppv ) for retinal detachment . after the procedure , the patient could only distinguish hand movements ; the condition was tentatively diagnosed as nonarteritic anterior ischemic optic neuropathy . conventional treatment with systemic corticosteroids and acetylsalicylic acid was ineffective and yielded substantial steroid - related side effects . additional administration of 2 110 mg dabigatran etexilate ( pradaxa ) , a novel direct thrombin inhibitor , resulted in a prompt and marked improvement of visual acuity , which indicated improved blood flow in the central vessels of the optic nerve . dabigatran etexilate may provide a promising alternative for the treatment of postprocedural vision loss after ppv .

hyperthyroidism is commonly associated with atrial fibrillation ( af ) , particularly in the elderly . we hereby report a case of patient with primary hypothyroidism presenting with af and pericardial effusion which resolved with levothyroxine therapy . he complained of chest pain which was substernal , nonradiating , and throbbing in nature . he smoked 10 cigarettes per day for past 25 years , and denied use of alcohol , caffeine , or drugs . physical examination was unremarkable except for the irregular heart rate ( approximately 130 beats per min ) and muffled heart sounds on auscultation . electrocardiogram [ figure 1 ] confirmed af with rapid ventricular rate , which responded to initial treatment with metoprolol [ figure 2 ] . af with rapid ventricular rate normalization of af with metoprolol thyroid function tests revealed a sensitive thyroid stimulating hormone ( tsh ) concentration of 34.70 iu / ml ( normal : 0.40 - 4.70 iu / ml ) , and thyroxine ( t4 ) of 5.57 g / dl ( normal : 8 - 12 g / dl ) confirming primary hypothyroidism . transthoracic echocardiography showed normal left ventricular systolic function ( ejection fraction of 63% ) , decreased left ventricular diastolic compliance , and massive pericardial effusion ( posterior 17 mm , anterior 13 mm ) . right atrial and right ventricle showed 30% collapse during diastole , mild left ventricular hypertrophy was present with no regional wall motion abnormalities . patient was treated with levothyroxine which lead to resolution of symptoms and restoration of normal sinus rhythm . repeat echocardiography showed no collapse of right atrium and right ventricle during diastole ) and mild pericardial effusion ( 11 mm circumferential ) . the most common cardiovascular signs and symptoms of hypothyroidism include bradycardia , mild hypertension ( diastolic ) , narrow pulse pressure , cold intolerance , pericardial effusion , cardiomyopathy and fatigue . hypothyroidism is often associated with electrocardiographic changes like bradycardia , right bundle branch block , flat or inverted t wave , qrs prolongation , qt prolongation , and infrequently ventricular arrhythmia , torsades de pointes . treatment of hypothyroidism is well - documented to cause af due to inadvertent overdosing of levothyroxine . although atrial arrhythmias are common and ventricular ectopy is rare in patients with hyperthyroidism , it is rarely associated with hypothyroidism . af is seen in 5 - 15% patients of hyperthyroidism and may be the presenting feature . increased chronotrophic activity due to increased thyroid hormones and increased sympathetic tone are the proposed underlying mechanisms . the physiological chronotropic response and normal tension of the heart muscle in diastolic phase depend on the action of triiodothyronine in the heart cells and its stimulating influence on na - k atpase and ca atpase in endoplasmic reticulum . normal contractility is also related to triiodothyronine stimulated transcription of the myosin heavy - chain alpha gene and inhibition of the heavy - chain beta gene . moreover , triiodothyronine acts on the cardiac muscle affects the number of beta adrenergic receptors and their sensitivity to catecholamines . few case reports have demonstrated that hypothyroidism may cause a prolongation of the qt interval which predisposes the patient to ventricular irritability . the canadian registry of atrial fibrillation investigators reported that 1.5% of 726 patients with af had hypothyroidism over a period of 1.7 years . studies have also reported that up to 8% of patients with atrial fibrillation were hypothyroid . hypothyroidism is known to cause nodal defects , but the mechanism of af in hypothyroidism is not known . it is also not clear whether its presence is causally related or is a mere manifestation of an underlying unrecognized structural heart disease . however in our case , reversal of af with levothyroxine treatment negates underlying structural damage of the heart nans suggests causal relationship . previous case reports have shown association of af with hypothyroidism but pericardial effusion was absent in those cases . this is possibly the first reported case of af and pericardial effusion in a case of hypothyroidism . young patients with no evidence of organic heart disease may be started on a full replacement dose of thyroxine . older patients or those with known or suspected ischemic heart disease , should initially be given about 25% of the anticipated replacement dose , and the dose should be increased gradually at 6 - 8 week intervals .

a 72-year - old man presented with palpitation , dyspnea , and chest discomfort . initial investigations revealed atrial fibrillation ( af ) and pericardial effusion , further investigations unraveled primary hypothyroidism ( thyroid stimulating hormone ) of 34.7 iu / ml and total thyroxine ( t4 ) of 5.57 g / dl ) . treatment with levothyroxine led to resolution of symptoms , af , and pericardial effusion .

first described in 1910 , purtscher s retinopathy is seen in severely traumatized patients and is characterized by sudden visual loss ( purtscher 1912 ) . the characteristic ophthalmoscopic findings are multiple areas scattered throughout the posterior pole of superficial retinal whitening , which appear as focal areas of retinal arteriolar occlusion on fluoresceinangiography ( grass 1997 ) . similar clinical findings have been reported in association with childbirth ( blodi et al 1990 ; shaikh et al 2003 ) , and have been termed purtschers - like retinopathy . the visual prognosis in purtschers - like retinopathy after childbirth is guarded , and to date , there is no definitive treatment . however , there are encouraging reports of visual recovery in patients with purtscher s retinopathy receiving high dose intravenous corticosteroid therapy ( atabay et al 2003 ) . we report a patient with purtschers - like retinopathy following childbirth who received a sub - tenon s capsule injection of triamcinolone with subsequent increase in visual acuity and decrease in retinal swelling . ten days after an emergent caesarian section for severe preeclampsia , rh immunization , and fetal anemia , a 24 year - old primigravida primiparus patient presented with complaints of decreased vision in both eyes , right greater than left . there were no other systemic abnormalities such as elevated liver enzymes , low platelets or other hematologic abnormalities . dilated fundus examination revealed bilateral purtschers - like retinopathy ( figures 1a , 1b ) , with widespread areas of macular ischemia and edema , as confirmed by optical coherence tomography ( figures 2a , 2b ) . of note are the superficial areas of high intensity signal , indicative of retinal ischemia . the central thickness in the right eye was 272 microns , and in the left 239 microns . a sub - tenon s injection of 0.5 cc of triamcinolone ( 40 mg / cc ) was given in the right eye on day 15 after delivery . at one week following the injection , her visual acuity was unchanged and her intraocular pressure was normal . she noted a marked improvement in the vision in her right eye . on examination a her acuity had improved to 20/60 in the right eye and 20/20 in the left , with normal intraocular pressures . her fundus examination showed marked improvement , with most of the superficial ischemic areas resolving . repeat optical coherence tomography in both eyes ( figures 3a , 3b ) demonstrated a reduction in the edema of the right eye by 30% from baseline , whereas the left , untreated eye showed a reduction in swelling of 14% . her visual acuity remained 20/60 in the right eye and 20/20 in the left eye . nearly a century ago otmar purtscher described a case of visual loss in a severely traumatized patient whose exam showed multiple superficial retinal hemorrhages and white patches throughout the posterior pole . since that time , similar findings have been associated with other conditions including compressive chest injuries , acute pancreatitis , fat embolism , retro - bulbar anesthesia , connective tissue diseases ( grass 1997 ) , and childbirth ( blodi et al 1990 ; shaikh et al 2003 ) . the pathogenesis of purtschers - like retinopathy post - partum is unknown , but may be related to arteriolar obstruction by complement induced leukoemboli produced during parturition ( blodi et al 1990 ) . similar clinical findings have been reported in the setting of amniotic fluid embolism . however , in the absence of a patent foramen ovale or pulmonary arteriovenous - shunts , it is unlikely that amniotic emboli are the direct cause of the observed retinal arteriolar obstructions . other investigators have theorized that subclinical amniotic fluid emboli may activate complement and induce granulocyte microemboli , which could occur on both sides of the pulmonary capillary bed . the visual recovery of postpartum patients with purtschers - like retinopathy is varied ( blodi et al 1990 ; shaikh et al 2003 ) . of the four patients described by blodi , three enjoyed significant improvement in central acuity , another case of purtscher - like retinopathy has been described in a patient with hellp syndrome during antepartum . generally purtscher - like retinopathy has a favorable prognosis but this case resulted in permanent loss of vision ( stewart et al 2007 ) . currently , there is no definitive treatment for postpartum purtschers - like retinopathy . in vivo , corticosteroids have been shown efficacious in inhibiting complement - induced granulocyte aggregation ( hammerschimidt et al 1979 ) . clinically , there are two reports of visual recovery after high dose , intravenous corticosteroid therapy in post - traumatic patients ( atabay et al 1993 ) . the case presented here is unique in that the patient had bilateral , asymmetric purtschers - like retinopathy post - partum , with quantitative oct improvement following unilateral local steroid therapy .

sub - tenon s injection of triamcinolone was performed in the right eye of a patient with bilateral purtschers - like retinopathy after childbirth . the response of macular edema to local triamcinolone was investigated with optical coherence tomography . five weeks following the injection , optical coherence tomography improvement was seen following unilateral sub - tenon s triamcinolone injection .

in india , 23.9 lakh people were living with hiv / aids ( plha ) in 2009 and children under 15 years accounted for 3.5% of all infections . according to who / unaids estimates , 450,000 children were receiving antiretroviral therapy ( art ) and more than 2 million children still needed art in 2010 . zidovudine ( azt ) , being an important component of standard regimen for first - line art in children , is widely used for managing pediatric hiv . as more and more children are exposed to this drug , the range of adverse reactions can also be expected to expand . nail pigmentation with azt is a well - documented occurrence in adults , especially dark - skinned individuals . , we report a case of zidovudine - induced nail pigmentation in a 12-year - old boy . in december 2011 , a 12-year - old boy was brought by guardians to dermatology opd for nail pigmentation involving all fingernails . about 4 years back , art was started for him with stavudine - based regimen ( 12 mg stavudine + 60 lamivudine + 100 mg nevirapine ) because of too low hemoglobin level . but after improvement in hemoglobin level , he has been shifted to zidovudine - based regimen ( zidovudine 300 mg + lamivudine 150 mg + nevirapine 200 mg ) since last 1 year . after 3 months of initiating the new regimen , he noticed bluish - brown discoloration of thumbnails that gradually spread to other fingernails [ figure 1a and b ] and grew in intensity over time [ figure 1c and d ] . there was no history of trauma , use of coloring agents , any triggering factor , or abnormal skin or mucosal pigmentation . ( a ) pigmentation involving all nails ( december 2011 ) ; ( b ) thumbnail involvement ( december 2011 ) ; ( c ) increased pigmentation in all nails ( march 2012 ) ; ( d ) increased pigmentation in thumbnails ( march 2012 ) on examination , he was averagely built , nourished , and fully immunized . local examination revealed diffuse bluish - brown discoloration of all fingernails , but it was more marked in thumbnails . nails were brittle with loss of lunulae and periungual changes [ figure 1c and d ] . the patient and guardians were counseled about harmless nature of this adverse drug reaction ( adr ) and he is receiving the same regimen at present . nail pigmentation with zidovudine was first described by furth and kazakis in two black patients in whom progressive hyperpigmentation of all fingernails developed . since then , several such case reports have been published from various parts of the world.[68 ] but all these patients were adults , and we did not come across any case report on pediatric patients . though our patient was receiving three antiretroviral drugs , zidovudine was thought to be the most likely cause of nail pigmentation , as pigmentation developed after 3 months of initiating zidovudine - based regimen . the causality assessment by naranjo algorithm showed that this adr was probable with zidovudine . in previous studies , patients taking zidovudine had noticed nail changes in 48 weeks or even after 56 months.[1011 ] dark - skinned individuals are at higher risk of this adverse effect and it appears to be reversible and dose dependant . nail pigmentation occurs in variety of medical conditions and is also associated with use of several drugs , especially chemotherapeutic agents . the pattern of nail pigmentation may be transverse bands , longitudinal bands , or diffuse discoloration . histopathologic findings of nail biopsy show deposits of brown pigmented granules containing melanin throughout the epidermis . as more patients receive zidovudine , it is important to alert patients about nail pigmentation . though it is harmless and reversible , psychological aspects of this noticeable side effect may hamper adherence and efficacy of therapy . this may also lead to unnecessary investigations and treatment for misdiagnosis such as cyanosis and melanoma . physicians should also focus on preventing adverse effects and distinguishing serious adrs from self - limiting adrs in order to manage prejudiced and fearful patients with available art .

zidovudine is an important component of first - line antiretroviral treatment ( art ) regimens used to manage pediatric hiv . nail pigmentation with zidovudine is a well - documented occurrence in adults , especially dark - skinned individuals . but it has so far not been reported in children . here , we report a pediatric case of zidovudine - induced nail pigmentation . a 12-year - old boy receiving art with zidovudine , lamivudine , and nevirapine presented to dermatology opd with complaint of diffuse bluish - brown discoloration of all fingernails . the pigmentation was noticed by the patient after 3 months of initiating zidovudine - based regimen . it first appeared in thumb nails , gradually involved all fingernails , and increased in intensity over time . though harmless and reversible , psychological aspects of this noticeable side effect may hamper adherence to therapy and may lead to unnecessary investigations and treatment for misdiagnosis such as cyanosis or melanoma .

oro - facial - digital syndromes ( ofds ) are a rare heterogeneous group of development disorders in which at least nine different forms have been described . ofd ii mohr 's syndrome is transmitted as an autosomal recessive condition characterized by malformations of the oral cavity , face and digits . facial and oral features include tongue nodules , cleft or high - arched palate , missing teeth , broad nose and cleft lip . digital features include clinodactyly , polydactyly , syndactyly , brachydactyly and duplication of the hallux . other systemic features include conductive deafness , choroidal coloboma , renal and congenital heart defects in variable combination . the incidence of mohr 's syndrome is very rare and occurs in one in 3 lakh live births . we report a case of young indian female suffering from ofd type ii ( mohr 's syndrome ) with otolaryngological manifestations . a 15-year - old indian female from western maharashtra born out of consanguineous marriage at full term presented with difficulty in speech and decreased hearing in both ears since birth . her mother had a first pregnancy with premature delivery at 7 months and child died immediately after birth . she has two siblings who are normal . during infancy , she had difficulty in feeding due to the high arched palate and tongue nodules . there was no history of radiation exposure or any significant drug intake or any trauma or any major illness during pregnancy period of her mother . vital signs revealed pulse 90/min , regular in nature , blood pressure : 120/80 mmhg right arm supine position . clinical examination revealed mild pallor , bilateral polysyndactyly with duplicated thumbs on both hands and bilateral polysyndactyly of halluces [ figures 1 and 2 ] , missing central incisors , broad nose , high arched palate , ocular hypertelorism and unusual presentation low set ears [ figure 3 ] and tongue nodules [ figure 4 ] . clinical image shows bilateral polysyndactyly of hand , bilateral duplicated thumbs clinical image shows bilateral polysyndactyly of halluces clinical image shows missing central incisors and broad nose and high arched palate and low set ears clinical image shows tongue nodule cardiac , respiratory , abdominal , neurological examinations were unremarkable and intelligence was normal . ophthalmic examination revealed normal fundus . on investigations , hemoglobin - 10.4 g% , total leukocyte count - 7900 cells / mm , dlc- p-79% , l-13% , e-08% , platelets - 275,000/cumm . an x - ray of both hands with forearms anteroposterior ( ap ) view [ figure 5 ] revealed six metacarpals in right hand showing fusion proximally of third and fourth metacarpals . an x - ray of both feet ap view [ figure 6 ] revealed seven metatarsals on the right side and six metatarsals on the left side . x - ray of hand shows polysyndactyly and bilateral duplication of thumbs x - ray of foot shows polysyndactyly and bilateral duplication of hallux ofds are a heterogeneous group of rare malformative diseases , characterized by abnormalities of the oral cavity , maxillo - facial region and digits . such phenotypical pattern was first described by mohr in 1941 and later defined as oro - digital - facial dysostoses by papillon - lage and psaume in 1954 and finally named ofds in 1967 by rimoin and engerton . the brachydactyly characters observed in the patient , which is one of the major symptoms of mohr syndrome is explained by norwegian geneticist mohr . there are atleast nine different forms of ofds on the basis of inheritance transmission pattern and phenotypical spectrum , of which the first two types are of common occurrence as compared with other varieties . ofds ii is a rare autosomal recessive disease whose diagnosis is based only on clinical evidence . frequency is rare , one in 3 lakhs live births . the molecular genetic basis is still unknown . because of the variable clinical expression , even intrafamilial , the attribution of the correct diagnosis among the several forms of ofds is often difficult . in addition , the molecular genesis is still unknown for all ofds except for the ofd i which is related to the cxorf 5 gene ( xp22.222.3 ) coding for ofd 1 protein . ofd ii ( mohr syndrome ) is similar to ofd i in that affected individuals usually have hand abnormalities , lobulated tongues and cleft abnormalities . however , a broad nose with a bifid tip is seen in ofd ii instead of the alar hypoplasia which characterizes ofd i. the bilateral hallux , polysyndactyly when present is strongly suggestive of ofd ii . ofd ii is characterized by distinct tongue nodules , rather than the bifid tongue more commonly seen in ofd i. the histopathology of tongue nodules of ofd ii would be either hamartoma or lipoma . although both ofd i and ofd ii may present with porencephaly , corpus callosum agenesis is not seen in ofd ii . conductive hearing loss , typically not seen in ofd i , has been reported in ofd ii . ofd i is the only type in which renal cysts occur which grossly resemble adult polycystic kidney disease , although sometimes types vi and vii may also manifest with renal abnormalities . laryngeal hypoplasia and tracheal stenosis have also been described in a small subset of individuals with ofd ii . however , cowden syndrome is characterized by multiorgan hamartomas , whereas in ofd the hamartomas are limited to the tongue alone . unique and uncommon features noted in this case : bilateral duplication of thumbslow set ears . bilateral duplication of thumbs with the clinical knowledge and typical radiological appearance , we diagnosed this case as mohr 's syndrome type ii ofds . ofd type ii mohr 's syndrome is rarer than ofd i and gets easily confused with , therefore distinctive features and clinico - radiological knowledge between these two is essential and be considered prior to its implications in genetic counseling of such patients . 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 there are no conflicts of interest . 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

we present a case of oro - facial - digital syndrome type ii ( mohr 's syndrome ) which is characterized by malformations of the oral cavity , face and digits . the facial and oral features include tongue nodules , cleft or high - arched palate , missing teeth , broad nose ; cleft lip . the digital features include clinodactyly , polydactyly , syndactyly , brachydactyly and duplication of the hallux .

the lancefield group g beta - hemolytic streptococci ( ggs ) have been recognized as pathogens causing serious infections in humans , although ggs can have a commensal relationship when present on the skin , pharynx , intestinal tract , and vagina.1 the major pathological conditions predisposing ggs infections include malignancy , alcoholism , cardiovascular disease , diabetes mellitus , bone and joint diseases , and cirrhosis of the liver . we report a rare case of endogenous endophthalmitis caused by streptococcus equisimilis , a ggs organism . a 74-year - old woman became confused in her daily living , and the confusion was concomitant with a high - grade fever on april 29 , 2008 . she was admitted to a local hospital on the following day and was given intravenous ceftriaxone for 9 days . she had a history of cardiac and brain infarctions and was at the postoperative stage of uterine cancer . ggs was detected in cultures of her blood sample and she was suspected to have infective endocarditis . although the hospital staff had noticed a moderate swelling of her left eyelid since may 1 , 2008 , an ophthalmologist did not examine her until she was referred to us on may 9 , 2008 . physical examination showed the patient to be a very ill woman with a body temperature of 38c . her left eyelids were swollen , and a moderate degree of conjunctival infection with chemosis was observed in her left eye . transthoracic echocardiography disclosed a mobile mass associated with the mitral valve with moderate valvular regurgitation ( figure 1 ) . laboratory tests demonstrated an elevated leucocyte count of 11850/l with 82% of polymorphonuclear cells , a c - reactive protein level of 10.65 mg / dl , lactate dehydrogenase level of 519 iu / l , amylase level of 175 iu / l , and brain natriuretic peptide level of 450.0 pg / ml . she was immediately hospitalized and on the same day ( may 9 , 2008 ) underwent emergency mitral valvuloplasty and both intravitreal and subconjunctival injections of both 1 mg / ml vancomycin and 1 mg / ml meropenem . after the surgery , she was treated with topical antibiotics ( levofloxacin ) and ointment ( oflx ) for approximately 1 month , intravenous gentamicin ( 200 mg / day ) , and intravenous penicillin g potassium ( 240,000 iu / day ) for 9 days . her general condition and ocular inflammation gradually improved with the medical therapy . however , her best - corrected visual acuity remained at light perception . subsequently , cultures from both anterior chamber and vitreous biopsy specimens grew s. equisimilis , which was sequenced by polymerase chain reaction . the s. equisimilis was found to be susceptible to penicillin g , ampicillin , sulbactam sodium / ampicillin sodium , imipenem / cilastatin , levofloxacin , and vancomycin , and was resistant to erythromycin and clarithromycin . s. dysgalactiae subsp . equisimilis hosts variants within the lancefield group a , c , l , and g carbohydrates , and was recently determined by gene sequencing to be a subspecies of group g of s. equisimilis . exogenous ggs endophthalmitis has been reported following cataract surgery , penetrating keratoplasty and trabeculectomy.2 on the other hand , endogenous ggs - related endopthalmitis is extremely rare.1 so far , there have been only 8 cases of endogenous ggs - related endophthalmitis;35 4 cases associated with endocarditis , 1 with cellulites of the foot , 1 with facial trauma , 1 with an abscessed tooth , and 1 of unknown origin . we demonstrated a case of an endogenous ggs - related endophthalmitis where s. equisimilis was first identified from both anterior chamber and vitreous biopsies , and where identification was also first made by polymerase chain reaction . the first - line therapy of s. equisimilis - induced infections is penicillin . besides penicillin , s. equisimilis was sensitive to all of the antimicrobial agents we administered . in our case , we suggest that a delay in the diagnosis would have worsened the general conditions of the patient , and would then lead to a poorer prognosis . at present , the efficacy of immediate ocular therapies including vitrectomy and intravitreal antibiotics against ggs - related endogenous endophthalmitis is still controversial.3 only further investigations will answer this question .

we report a rare case of endogenous endophthalmitis caused by streptococcus equisimilis . a 74-year - old woman with endocarditis developed endogenous endophthalmitis . the patient underwent emergency mitral valvuloplasty , and intravitreal and subconjunctival injections of vancomycin and meropenem . after the surgery , she was treated with topical antibiotics , ointment , intravenous gentamicin and intravenous penicillin g potassium . the causative organism was identified as s. equisimilis . s. equisimilis should be considered as a pathogen that can cause severe endogenous endophthalmitis .

the interventions for carotid artery stenosis , vein graft and acute myocardial infarction could embolize a large amount of plaque debris , especially unstable , ulcerative , and thrombus containing lesions ( 1 ) . distal protection devices such as filterwire ex have been widely used in carotid artery stenting to prevent distal embolization of plaque debris ( 2 ) . the large amount of atherothrombotic debris entrapped in the filter could reduce or stop antegrade flow and may be shown as no reflow phenomenon in case using filter type distal protection device . we present a case of pseudo no - reflow phenomenon after postdilatation of the stent in severe carotid artery stenosis and successful recovery with aspiration thrombectomy using export aspiration catheter . a 70-yr - old man was diagnosed with asymptomatic carotid artery stenosis during preoperative risk assessments . he had been treated in our hospital for congestive heart failure and stable angina for 2 yr . carotid angiogram showed no significant stenosis in the left carotid artery and a 90% stenosis in the right internal carotid artery ( fig . shuttle sheath ( 7f , cook medical , bloomington , in , u.s.a . ) was positioned to right common carotid artery via right femoral artery . to prevent distal embolization of plaque debris during angioplasty , filterwire ex ( boston scientific , natick , ma , u.s.a . ) was positioned in the right cervical internal carotid artery distal to stenotic lesion . after predilation with 4.0 mm balloon , 8 mm self - expanding wall stent ( boston scientific ) was positioned across the lesion and was deployed in stenotic lesion . after postdilation of the stent with 6.0 mm balloon , carotid angiogram showed abrupt complete flow interruption ( fig . we decided to perform aspiration thrombectomy using export aspiration catheter ( medtronic ave , santa rosa , ca , u.s.a . ) ( fig . after several passes of export aspiration catheter into the basket ( over the filterwire ex ) , antegrade flow was fully restored ( fig . 3 ) . after retrieval of filterwire ex , whitish debris was noted within the basket ( fig . the patient complained of slight speech disturbance and this neurological abnormality was completely recovered within 24 hr after stenting . obstructive carotid artery lesions are known to contain friable , ulcerated and thrombolic material that have the potential to embolize during intervention and may be responsible for the majority of the neurologic events during carotid artery stenting ( 1 ) . a number of " distal protection " strategies , designed to capture embolic debris released during carotid intervention , have been evaluated for their efficacy in minimizing the risk of embolic neurologic events . the intravascular filter type distal protection device offers the advantage of a constant cerebral perfusion during carotid artery stenting and allows more time for careful and precise intervention ( 2 ) . during carotid artery stenting using filter type distal protection device , microscopic particles larger than the size of the filter pores ( 80 to 130 m ) could be captured . ( 3 ) reported that carotid artery stenting with filter type distal protection device experienced flow impairment due to filter obstruction in 7.9% of procedures , which was resolved in all cases after filter removal . in our case , we suspected this pseudo - no - reflow phenomenon was induced by obliteration of filter pore by massive atherosclerotic debris . we considered to remove filter by retrieval catheter , however , this process may cause entrapment of filter device to retrieval catheter and embolize massive amount of atherosclerotic debris . therefore , we choose aspiration thrombectomy using export aspiration catheter . after aspiration thrombectomy , flow interruption in the right internal carotid artery was completely restored , therefore , we confirmed this phenomenon as pseudo - no - reflow phenomenon instead of true no - reflow phenomenon . yadav ( 4 ) recommended practically in his review paper if there is no forward blood flow during carotid angioplasty with filter type distal protection device , this should be aspirated before the filter is collapsed . our case is the first case report of pseudo - no - reflow phenomenon in carotid artery stenting using filter type distal protection device and successful recovery of this phenomenon by aspiration thrombectomy using export aspiration catheter . ( 5 ) recently reported that successful management of pseudo - no - reflow phenomenon by the export aspiration catheter in ostial saphenous vein graft intervention using filterwire ex protection . in conclusion , the large amount of atherothrombotic debris entrapped in the filter during carotid intervention could stop antegrade flow . this phenomenon is pseudo - no - reflow phenomenon instead of true no - reflow phenomenon .

distal protection devices such as filterwire ex have been widely used in carotid artery stenting , however , the large amount of atherothrombotic debris entrapped in the filter could reduce or stop antegrade flow . we present a case of pseudo - no - reflow phenomenon after postdilatation of the stent in a patient with asymptomatic carotid artery stenosis . after several passes using an export aspiration catheter , normal flow in the internal carotid artery was restored . aspiration thrombectomy can successfully recover pseudo - no - reflow phenomenon .

neurofibromatosis affects 1:3,000 individuals , and characterized by largely benign but often debilitating tumors that grow in the nervous system . its course is unpredictable : it can cause a variety of benign nerve tumors including plexiform , dermal , and optic glioma tumors ; in some cases malignant peripheral nerve sheath tumors can develop in the plexiform tumours . down 's syndrome is one of the most common and easily recognized genetic conditions in humans . the estimated prevalence in the united states is approximately 15 per 10,000 live births ( ie , 1 out of every 700 ) most often , it is the result of nondisjunction on chromosome 21 during maternal meiotic division . we herein present the case of a 17-year - old boy with complaints of skin lesions over the back associated with mild itching since 3 months . he was a known case of down 's syndrome with a history of seizures in childhood [ figure 1 ] . the lesions gradually increased in size and number , and similar lesions started developing over his forehead since 23 weeks . on examination , multiple skin colored papules of varying size were present over the entire back and the forehead . velvety thickening of the skin and hyperpigmentation of the axillae suggestive of acanthosis nigricans was present . the characteristic features of down 's syndrome , including simian crease , mongoloid facies , and mental retardation were present . canities and a solitary keloid over the chest were also seen apart from the clinical features of down 's syndrome . on oral examination , scrotal tongue , abnormal dentition , and other investigations such as ct scan brain , 2d echo , and ecg were normal . histopathology of the nodule from the back revealed focally thinned out epidermis with intact basal layer ; the papillary dermis showed a mild perivascular infiltrate . deeper dermis showed a benign spindle cell proliferation suggestive of neurofibromatosis [ figure 3 ] . physical appearance of down 's syndrome ( a ) neurofibromas ( b ) sebaceous cyst ( c ) acanthosis nigricans , and axillary freckling ( d ) scrotal tongue focally thinned out epidermis with intact basal layer with the papillary dermis showing a mild perivascular infiltrate . the presentation of a patient with two unrelated genetic disorders is uncommon , although not statistically impossible . however , in two of these reports , a third medical condition was also present . in one report , breast cancer was reported . in the other , our patient had down 's syndrome , neurofibromatosis , dental anomalies , and ocular defects and keloids . in the large majority of cases , trisomy 21 there is no current evidence to support the idea that this is anything other than a chance occurrence . the two conditions are not related , and the likelihood of a person being born with these two conditions is approximately 1 in 2,700,000 births . they however overlap in their manifestations . both are associated with intellectual impairment to differing degrees . macroglossia occurs in both conditions , as may facial , dental , and occlusal abnormalities . hearing and speech are affected in both conditions , as may the ability to maintain an acceptable level of oral hygiene . mutations in the gene results in abnormal control of cell growth , differentiation , and aberrant myelination . our patient with of neurofibrmatosis type 1 with down 's syndrome is the first such to have a keloid and sebaceous cyst apart from myopia and dental anomalies . the unpredictable nature and course of the two genetic disorders along with multiple acquired conditions in this patient make it difficult for patients , teachers , care givers , and medical / dental providers to create and maintain long - term care plans . 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 .

we report a patient with down 's syndrome and neurofibromatosis who presented with a keloid , sebaceous cyst and acanthosis nigricans , along with dental and ophthalmological defects . the coexistence of neurofibromatosis type 1 and down 's syndrome which are two unrelated genetic conditions is itself a rarity .

in this article we will use the term variable number tandem repeat ( vntr ) to encompass both microsatellites and minisatellites however not all tandem repeat domains are polymorphic in repeat copy number . sva elements only represent 0.13% of the genome , representing ~2700 elements , constituting the youngest of the retrotransposable elements in the human genome and are hominid specific . they consist of a hexamer repeat ( ccctct ) , an alu - like sequence , a gc - rich vntr , a sine and a poly a - tail as with other retrotransposons much effort has been expended on the retrotransposition event itself giving rise to disease . this has resulted in the identification of at least 8 well characterized diseases in which the retrotransposition event has caused the disease , in the majority of cases by affecting alternative slicing our hypothesis is that the sva element could impart a hominid specific regulatory twist on regulation of the gene in which they have inserted ( assuming that insertion is not in an exon or destroys intron / exon boundaries etc ) . in the promoter they would act as classical mediators of gene expression . analysis of human genome release 19 ( hg19 ) indicates that of the 2676 sva elements in the human genome , 433 are present within 10 kb 5 of the main transcriptional start site 1 . sva elements are functional in conventional reporter gene assay both in vitro ( cell line ) and in vivo ( chick embryo ) 2 . sva elements are polymorphic , most clearly via vntr domains ( flanking and internal ) a. we have previously shown in other genes ( as have many others ) that vntrs are both polymorphic biomarkers associated with disorders and transcriptional regulatory domains in vivo and in vitro b. vntrs are common regulatory domains in viruses ; i. they in part control hsv-1 virus latency in dorsal root ganglia ( via a similar cccctc repeat to that found in the flanking sequence of the sva element ) ii . sva elements can be associated with active chromatin adjacent to : a. positive affymetrix probe arrays in the human cns b. encode active histone marks 4 . sva elements have the potential to form structures which may be strong modulators of genome structure / function : a. potential g4 quadruplex b. strong cpg component approaching the classical requirements to be defined as cpg islands , which is consistent with their differential methylation in tumors in our analysis of the fus sva element we generated reporter gene constructs that were analyzed in both the neuroblastoma cell line , sk - n - as and a chicken embryo model the distance of the sva element from its site of action on a specific promoter or transcriptional start site can be quite large . it has been demonstrated by us and others when attempting to reproduce endogenous gene expression in transgenic models , that regulatory domains utilized by a locus can be 100kb+ away from the gene itself . our own studies on the endogenous expression of the neuropeptide , substance p encoded by the tac1 gene demonstrated that although the gene itself from 5 to 3 utr was 8 kb , we required a dna fragment of 350 kb to reproduce the appropriate expression patterns the recent evolution of sva elements has imposed restricted sequence variation in each of the sva subclasses ; this may allow a concerted response to challenge by groups of sva elements with similar primary sequence based on sequence specific dna binding proteins . in this manner it might be possible for sva elements containing similar primary sequence such as is present in the hexamer repeat or central vntrs to bind similar transcription factors based on shared consensus dna binding domains . thus alterations in active transcription factor complement in the cell might modulate several sva elements by the same signal transduction pathways . the frequency of sva element retrotransposition is estimated to be 1:916 births which would correspond to 7 10 our recent work suggests that sva elements can in part modulate genome function via their action as a transcriptional or epigenetic modulator . polymorphism in the vntrs within these retrotransposons demonstrates a mechanism not only for differential regulatory function associated with genotype but a way to rapidly integrate polymorphism in the sva element as a potential biomarker for disease association without the requirement for retrotransposition .

endogenous mobile genetic elements can give rise to de novo germline or somatic mutations that can have dramatic consequences for genome regulation both local and possibly more globally based on the site of integration . however if we consider them as normal genetic components of the reference genome then they are likely to modify local chromatin structure which would have an effect on gene regulation irrelevant of their ability to further transpose . as such they can be treated as any other domain involved in a gene environment interaction . similarly their evolutionary appearance in the reference genome would supply a driver for species specific responses / traits . our recent data would suggest the hominid specific subset of retrotransposons , sine - vntr - alu ( sva ) , can function as transcriptional regulatory domains both in vivo and in vitro when analyzed in reporter gene constructs . of particular interest in the sva element , were the variable number tandem repeat ( vntr ) domains which as their name suggests can be polymorphic . we and others have previously shown that vntrs can be both differential regulators and biomarkers of disease based on the genotype of the repeat . here , we provide an overview of why polymorphism in the sva elements , in particular the vntrs , could alter gene expression patterns that could be mechanistically associated with different traits in evolution or disease progression in humans .

however , it is difficult to determine an appropriate resection line using only the laparoscopic approach . for more appropriate resection , laparoscopic and endoscopic cooperative surgery ( lecs ) on the other hand , gastric plexiform angiomyxoid myofibroblastic tumor ( pamt ) has recently emerged as a new entity among gastrointestinal mesenchymal tumors which was first described by takahashi et al . in 2007 . this is a rare tumor with equal gender distribution and occurs primarily in adults with a wide age range of 783 years [ 3 , 4 ] . however , the bland nuclear features , low proliferative index and absence of necrosis , vascular invasion , recurrence and metastasis in all cases of pamt reported to date justify its characterization as a benign tumor . suggested that vascular invasion and extragastric extension of the tumor were not typically observed in pamt . currently , distal or partial gastrectomy remains the treatment of choice . to our knowledge , a 39-year - old male patient visited our hospital because of epigastric pain . in an upper gastrointestinal endoscopy examination , gastroscopy revealed a 3.5 3.0 cm sessile polypoid mass with a smooth surface in the anterior wall of the gastric antrum with a recessed area . we considered that this tumor was located in the muscle layer ( fig . 1 ) . the ct scan showed a heterogeneous tumor in the gastric antrum , which was drastically enhanced with contrast medium , and consisted of a number of highly stained small nodules around the tumor rim ( fig . 2 ) . radiological imaging suggested differential diagnoses of angioma and solitary fibromyxoma because of the hypervascular nature of the tumor . however , because the tumor did not protrude into the lumen of the stomach , these diagnoses were unlikely . lecs combines laparoscopic gastric resection with endoscopic imaging for tumor location and determining an appropriate resection line . aggregations of small polypoid nodules , red - colored and angiomatous , were characteristically seen on the serous membrane surface , and appeared like a metastatic lymph node nodule ( fig . first , we located the tumor and cut the surrounding mucosa of the lesion by endoscope . we perforated the gastric wall at the distal side of the tumor using needle knife without injuring the pylorus . next , the seromuscular layer was dissected laparoscopically with an ultrasonically activated device tracing the mucosal line cut . a partial gastrectomy by lecs revealed the tumor at the antrum of the greater curvature side of the stomach , and the incision line was closed on suturing by layer - to - layer anastomosis . the tumor was composed of bland spindle cells which were separated by abundant myxomatous stroma and showed plexiform growth in the entire stomach wall ( fig . the tumor cells were negative for cd117 ( c - kit ) , cd34 and s-100 protein , but diffusely positive for smooth muscle actin consistent with predominant myofibroblastic differentiation ( fig . the tumor was especially characterized by multiple nodules protruding outward from within the serosa , and we diagnosed the patient with pamt . the patient received supportive care without any specific medication during hospitalization and had no recurrence or metastasis during follow - up for 9 months after the surgery . pamt is a rare mesenchymal tumor of the stomach , which consists of spindle cells with myofibroblastic characteristics . estimated that the frequency of pamt is less than 1/150 that of gastrointestinal stromal tumor , but only 28 cases of pamt have been reported , including the present case . the prevalence of pamt does not vary by sex ( m : f = 1 : 1 ) and it can develop at any age ( range : 783 years ) . there are few reports describing the ct findings for pamt [ 2 , 7 ] . they reported that ct findings of tumor location in the gastric antrum , a heterogeneous internal enhancement effect , and small nodules with a strong enhancement in the rim could be used for radiologic diagnosis of pamt . in our case , all three ct findings were evident . pamt is considered a benign tumor . however , due to its rarity , the true biological potential of pamt remains unknown . so , complete resection when functional preservation is possible is appropriate . on the other hand , lecs is the best procedure for complete resection while preserving the pylorus because of the gastric antrum of predilection for pamt . to our knowledge , when a myxoid spindle cell lesion is observed in endoscopic biopsy or when there are characteristic ct findings , pamt should be included in the differential diagnosis .

plexiform angiomyxoid myofibroblastic tumor ( pamt ) is a recently described distinctive gastric mesenchymal entity with a peculiar plexiform pattern , bland spindle cells and a myxoid stroma rich in arborizing blood vessels . in this study , we report a new case of this rare gastric tumor resected by laparoscopic and endoscopic cooperative surgery ( lecs ) . a 39-year - old japanese man was admitted with a gastric mass . gastroscopy showed an elevated mass in the anterior wall of the gastric antrum . endoscopic ultrasound examination revealed a focal hypoechoic lesion protruding into the lumen . a partial gastrectomy by lecs was performed , and the patient made an uneventful recovery and remains well 9 months later . the tumor in this case depicted all the typical histopathologic and immunochemical features of gastric pamt ( c - kit negative and smooth muscle actin - positive ) . especially , it was characterized by multiple nodules protruding outward within the serosa . therefore , it is important that the resection line is determined on the serosa to ensure the complete resection of these nodules together .

myelodysplastic syndromes ( mdss ) represent a group of clonal hematological disorders characterized by progressive cytopenia reflecting defects in erythroid , myeloid and megakaryocytic maturation . anemia , neutropenia and thrombocytopenia , separated or in combination , despite a hyper- or normocellular marrow define mds . hypoplastic mds ( h - mds ) accounts for 1217% of all patients with mds . clonality studies of mature blood cells and immature progenitors suggest that a myeloid lymphoid stem cell might be the principal target for clonal transformation at least in a major part of mds . an association between mds and autoimmune disorders and with chronic t - cell disorders like large granular lymphocytic leukemia ( lgl ) has been recognized and is suggestive of an abnormal functioning immune system in mds . lymphoproliferative disorders , especially t - prolymphocytic leukemia ( pll ) developing in an h - mds patient , is extremely rare and has an aggressive course . we are reporting a unique case of t - pll developing in h - mds , and this is probably the first case report in the literature . a 45-year - old male was admitted with generalized weakness , palpitation , gradual weight loss and frequent syncopal attacks for more than 2 years . on examination , the iron profile was : serum iron 115.50 g / dl , total iron binding capacity 177.10 ng / ml and markedly elevated b12 levels ( > 2000 pg / ml ) . hemoglobin was 3.9 gm% , total leukocyte count was 2100 cells / cumm , platelet count was 70,000 cells / cumm and reticulocyte count was 0.8% at the time of admission . pancytopenia [ figure 1a ] was persistent on every visit and the peripheral smear showed normocytic and macrocytic rbcs , leukopenia , hypogranular and pseudo pelger - huet neutrophils [ figure 1b and 1c ] , which were characteristic with relative lymphocytosis and no blasts in the peripheral smear ; the platelet count was reduced . the bone marrow aspiration smears and cell block preparation showed severe hypocellularity ( < 20% for his age ) [ figure 1d ] . sparse erythroid cells with dyserythropoiesis [ figure 2a and 2b ] , minimal dysmyelopoietic ( hypogranular and hyposegmented myeloid cells - 10% ) and dysmegakaryopoietic ( hypolobated and micromegakaryocytes [ figure 2c and 2d ] features were evident with blasts < 5% and absence of fibrosis in biopsy sections . the possibility of aplastic anemia and hypoplastic acute myeloid leukaemia ( h - aml ) were ruled out . a diagnosis of primary h - mds was made in the absence of chemotherapy and radiotherapy . the patient was transfusion dependent and later started on thalidomide . on follow - up within few months , because of persistent pancytopenia , the patient was subjected for repeat bone marrow study , where the smear and section showed cellular marrow with diffuse infiltrate of mature lymphocytes ( 60% ) [ figure 3a and b ] with no evidence of improved hematopoiesis . the neoplastic cells showed a mature t - cell immunophenotype , cd3 + , cd4 + , cd5 + , cd7 + and cd8 + , and tdt- , cd20- , cd23- , cd79a- , cd34- , bcl2- and cyclind1- , and was diagnosed as t - pll . ( d ) hypocellular marrow - cell block preparation h and e , 10 ( a ) dyserythropoiesis with multinucleation in bma . ( c ) dysmegakaryopoiesis - cell block h and e , 40. ( d ) dysmegakaryopoiesis bma ( a ) lymphoid infiltrate in bma . the diagnosis may be difficult in h - mds if the aspirate is so sparsely cellular as to preclude cytogenetic studies . significant clonal cytogenetic abnormalities are present in only half of all mds cases , and full karyotype analysis , which requires cells in metaphase , may not be possible because cells may be scarce or senescent . we encountered similar problems when there was hypocellular marrow , but later , when diagnosed as t - pll , the marrow culture showed a normal karyotype . there is growing scientific evidence supporting the hypothesis that cytogenetic abnormalities are not the initiating clonal event but are acquired during disease progression . h - mds has yet to be shown to alter the disease course or prognosis , but some studies suggest benefit from immunosuppressive therapeutics . it is presently unknown whether h - mds patients have a preceding , but unrecognised , phase of excessive apoptosis insufficiently compensated by hyperproliferation , or whether their disease is more related to aplastic anemia . several years ago , in vitro studies have proven that autologous cytotoxic t - lymphocytes can exert an inhibitory effect on mds myelopoiesis . an association between mds and autoimmune disorders and with chronic t - cell disorders like lgl has been recognized and is suggestive of an abnormal functioning immune system in mds . but , occurrence of t - pll in h - mds is extremely rare and , possibly , this is the first case in the literature . the immunophenotype is that of mature t - cell , which is usually cd4 + but , occasionally , can be cd8 + , which was evident in our case . expression of cd7 helps to distinguish it from other clpds , like sezary syndrome and adult t - cell leukemia / lymphoma . to conclude , the finding of activated and clonal t - cell population in mds is further argument for the use of immunosuppression to treat the cytopenias of mds .

myelodysplastic syndrome ( mds ) represents one of the most challenging health - related problems in the elderly , characterized by dysplastic morphology in the bone marrow in association with ineffective hematopoiesis . hypoplastic mds ( h - mds ) accounts for 1217% of all patients with mds and has yet to be shown to alter the disease course or prognosis . the concept that t - cell - mediated autoimmunity contributes to bone marrow failure in mds has been widely accepted due to hematologic improvement after immunosuppressive therapy . t - cell expansion is known to occur in these patients , but development of chronic t - cell disorders , especially t - prolymphocytic leukemia ( pll ) in a hypocellular mds is extremely rare , which has an aggressive course . the possible explanation for the association between the two disorders is that t - pll might arise from a clonally arranged mds stem cell . we report a unique case of h - mds with non - progressive pancytopenia and severe hypocellular marrow for 2 years , followed by t - pll within few months .

a 49-year old woman presented in october 2007 with abdominal bloating , pain , and obvious ascites . after imaging data was obtained , in december 2007 the patient underwent total abdominal hysterectomy ( tah)/bilateral salpingo - oophorectomy ( bso ) and debulking surgery to remove as much of the tumour as possible . after surgery the patient was diagnosed with serous ovarian adenocarcinoma , stage iii c. first - line chemotherapy was the recommended taxol / carboplatin combination standard - of - care for 3 cycles , which finished in march 2008 . in june 2010 the patient relapsed and there was recurrence of disease , presenting as rapidly accumulating ascites . the patient was rechallenged with platinum second - line ; treatment was taxol / carboplatin for 6 cycles , which was completed in october 2010 . the patient then agreed to participate in a phase ii study investigating the ca-125 doubling time in patients with advanced disease treated with tamoxifen citrate . in april 2011 there was recurrence of disease , and in august 2011 the patient received trabectedin + pld combination for 5 cycles as fourth - line therapy . the patient achieved a good clinical response according to ca-125 measurement , and the chemotherapy was stopped given the good clinical status of the patient . in january 2012 the patient experienced disease progression and received subsequent treatment with gemcitabine / carboplatin / bevacizumab combination therapy for 6 cycles , plus bevacizumab 15 mg / kg every 3 weeks as maintenance therapy . in june 2013 the patient experienced further disease progression and received taxol / carboplatin as weekly treatment for 6 cycles until january 2014 , after which the patient 's disease was stable . disease recurrence occurred in march 2014 , with ascites , para - aortic lymph node involvement , and peritoneal disease . the patient was subsequently re - challenged with trabectedin + pld as seventh - line of chemotherapy . 1a ) , and ct imaging showed a clinical response and improvement in symptoms ( fig . the dose of trabectedin + pld was adjusted for this patient due to grade 3 fatigue . the trabectedin dose was reduced from 1.1 to 0.9 mg / m , and pld was reduced from 30 to 25 mg / m . the management plan for this patient is to continue treatment with trabectedin + pld until disease progression . the patient has finished 11 cycles so far and is still in remission , and continues to do well . in summary , this case study describes a new concept in the management of a roc patient who has received 7 different lines of chemotherapy and has experienced recurrent disease . trabectedin + pld combination was effective as a non - platinum / non - taxane alternative to intercalate between platinum - based therapies . in fact , trabectedin has been administered for 6 or more cycles in 52% of patients treated with the combination dose and schedule respectively . the combination regimen has been used for up to 21 cycles , and no cumulative toxicities have been observed in patients treated with multiple cycles . so whilst doxorubicin is limited by its cumulative dose due to cardiotoxicity , trabectedin plus pld can be used to disease progression . in the case study described here the patient is doing well , and will continue maintenance treatment with trabectedin + pld , with repeat echo every 12 weeks , until disease progression . as with all cytotoxic regimens there are some practical considerations to treating patients with trabectedin + pld . infusion at a dose of 1.1 mg / m , immediately after pld 30 mg / m , for the treatment of ovarian cancer ( fig . the following steps must be taken when administering the combination : all patients must receive corticosteroids e.g. 20 mg of dexamethasone i.v . infusion of trabectedin 1.1 mg / m , immediately after pld , every 3 weeks through a central venous line all patients must receive corticosteroids e.g. 20 mg of dexamethasone i.v . infusion of trabectedin 1.1 mg / m , immediately after pld , every 3 weeks through a central venous line as with other cytotoxic regimens , monitoring of patients receiving trabectedin + pld is recommended ( box 1 ) . the patient must satisfy the following criteria for initiation of treatment and prior to each re - treatment : absolute neutrophil count ( anc ) 1,500/mmplatelet count 100,000/mmbilirubin upper limit of normal ( uln)alkaline phosphatase 2.5 uln ( consider hepatic isoenzymes 5-nucleotidase or gamma glutamyl transpeptidase ( ggt ) to determine whether the elevation is of hepatic or osseous origin.albumin 25 g / l.alanine aminotransferase ( alt ) and aspartate aminotransferase ( ast ) 2.5 mol / l ) or creatinine clearance 60 ml / mincreatine phosphokinase ( cpk ) 2.5 ulnhaemoglobin 9 g / dl absolute neutrophil count ( anc ) 1,500/mm platelet count 100,000/mm bilirubin upper limit of normal ( uln ) alkaline phosphatase 2.5 uln ( consider hepatic isoenzymes 5-nucleotidase or gamma glutamyl transpeptidase ( ggt ) to determine whether the elevation is of hepatic or osseous origin . alanine aminotransferase ( alt ) and aspartate aminotransferase ( ast ) 2.5 uln serum creatinine 1.5 mg / dl ( 132.6 mol / l ) or creatinine clearance 60 ml / min creatine phosphokinase ( cpk ) 2.5 uln otherwise treatment should be delayed and/or reduced for up to 3 weeks until the criteria are met , as highlighted in the case study above ( box 2 ) . a single institution 's experience with trabectedin + pld combination , as a non - platinum / non - taxane alternative to intercalate between platinum - based therapies is reported here . a real - life case study is presented of a heavily - treated patient with advanced disease who was treated with trabectedin + pld fourth - line and subsequently rechallenged at seventh - line , who continues to do well on treatment . this case study demonstrates the utility of the combination at any line of relapse in roc patients with advanced disease . professor tahir received speaker fees from pharmamar for his participation at the bgcs 2014 symposium .

the goal of recurrent ovarian cancer ( roc ) treatment is no longer just palliation , but prolonging survival . this is usually through administering a new line of chemotherapy at each relapse . a novel treatment sequencing strategy to achieve this is through the intercalation of an effective non - platinum alternative , in between platinum - based therapies . trabectedin in combination with pegylated liposomal doxorubicin ( pld ) has been fully available privately in the uk since 2009 for treating patients with roc . a single institution 's experience with the trabectedin + pld combination , as a non - platinum / non - taxane alternative , to intercalate between platinum - based therapies is reported here . to date 6 patients have been successfully treated with trabectedin + pld at broomfield hospital , chelmsford , essex . here we describe a new , practice - changing treatment approach in a real - life case study of a heavily - treated patient with advanced roc treated with trabectedin + pld at fourth - line and then subsequently rechallenged at seventh - line ; with treatment continuing until disease progression .

presentations vary , complications differ and yet , management remains the same , which is removal . nothing galvanizes the team as when the code for a foreign body ( fb ) is communicated , especially if the case is a kid where such situation is usually encountered . presentation is acute in vegetable foreign body aspiration , especially in kids of age group 1 - 3 years , with new onset wheeze , breathlessness or fever . it tends to be less dramatic in non - organic foreign bodies unless there is lung collapse or involvement of trachea . occasionally non - organic fb can be retained for years with bizarre presentation , which can be mistaken for endobronchial tumors or bronchiectasis . such fb is a cause for a significant heartburn to both the patient and treating physician as unless it is removed it causes chronic symptoms and leads to irreversible damage . the case of interest is one such with the diagnosis missed for over 14 years . a 16-year - old male was diagnosed with bronchial asthma since childhood , had recurrent hospital admissions with exacerbations . patient reported a recent worsening of symptoms for the last year for which he was evaluated and chest radiology revealed left lower lobe collapse . he was taken up for a bronchoscopy , which revealed a web - like stenosis of distal left main stem bronchus with a residual lumen of about 4 mm [ figure 1 ] and in view of small residual lumen an ultrathin pediatric bronchoscope ( olympus bf type 3c160 ) was used . bronchoscope could be negotiated past the stenotic segment and to our surprise found a freely mobile object moving between upper and lower lobes with each breath [ figure 2 ] . patient was questioned after the procedure regarding history of aspiration and he denied recollecting any incident . whistle was blown when the patient 's father said that the patient had aspirated a whistle or part of one when he was about 2 years of age . he had a violent bout of cough for which he was evaluated and suspected whistle was removed . now with the diagnosis clear , patient was taken up for rigid bronchoscopy , intubated with size 6 ventilating tracheobronchosope and bevel was positioned at distal left main bronchus . flexible bronchoscope was introduced through this conduit and mercedes benz like peripheral cuts given using eletrocautery knife to the web like stenosis [ figure 3 ] . serial balloons introduced across the stenotic segment and gradually dilated up to 10 mm , bronchoscope negotiated beyond to visualize the foreign body . the fb was extracted using cryoprobe introduced through the channel of flexible bronchoscope [ figures 4 and 5 ] . the fb turned out to be a piece of plastic probably part of the whistle aspirated 14 years back [ figure 6 ] . stenosis at distal left main stem bronchus foreign body beyond stenosis electrocautery knife to make radial incisions cryo - extraction of foreign body from bronchus foreign body being taken into rigid barrel plastic foreign body after removal retained non - organic foreign bodies are great masqueraders presenting with non - specific symptoms and high propensity of being missed unless index of suspicion is high . the longest duration of retained fb in english literature is 39 years , a case of wooden chip reported from japan . history of fb is the first clue to making a diagnosis but is unfortunately forthcoming only in few of the cases that too in retrospect . evaluation should be undertaken even if fb was removed previously and patient has symptoms as part of it could have been retained as it was in our case . symptoms range from none to chronic cough , wheezing , hemoptysis and recurrent episodes of chest infections . in our country profile of symptoms are generally non specific but documented episodes restricted to one side or lobe warrant evaluation for endobronchial lesions . chest x - ray is the next step in assessing these patients ; it has a high sensitivity for detecting metallic foreign bodies and can also reveal subtle signs like lobar collapse or hyperinflation . chest x - ray has been shown to reveal the fb as opacity in only 14 - 20% among both adults and pediatric population . this has been the case in all reports of retained fb of over 10 years in english literature including ours . flexible bronchoscopy should be undertaken if radiology reveals or suspects fb ; also in cases of unexplained longstanding non - specific symptoms with negative radiology and no alternate diagnosis . luminal appearance of fb after years of residence is rarely clear , especially if one is unsure of what they are looking for . granulation tissue can be exuberant and mimic tumor in appearance , attempts to biopsy it can result in significant bleeding which can further compromise vision . stenosis of bronchus like the one seen in our patient is a relatively uncommon complication seen in about 8% of cases as against 62.9 - 76.5% incidence of granulations in two large series . even among patients with stenosis only minority require management of the same and in our case was required due to presence of tight stricture in the main stem bronchus . there is consensus regarding rigid bronchoscopy for management of fb in pediatric patients as these patients are sicker , hypoxemic , can not cooperate , have poor reserve and flexible bronchoscope lacks robust instruments for retrieval due to small working channel . further , flexible bronchoscopy is useful in only confirming the presence of fb in stable pediatric patients , thus avoiding a negative rigid bronchoscopy . flexible versus rigid bronchoscopy for management in adults is a long - standing argument with various operators having different points of view . rigid bronchoscopy is the preferred modality for either long - standing fb or in case of failed attempt with flexible bronchoscope . long - standing fb is also notorious for failure of rigid bronchoscopy due to excessive granulation , which then requires bronchotomy and/or lobectomy for retrieval . in our case rigid bronchoscopy was done in view of long - standing fb and presence of web - like stenosis that required dilatation . long - standing fb is a challenge both to diagnose and manage , needs a high index of suspicion and low threshold for detailed evaluation . management requires personnel with adequate experience and availability of appropriate instruments both flexible as well as rigid . there should be seamless switch over to rigid if flexible is not successful in recovery of fb .

16 years old boy with childhood history of chronic respiratory symptoms requiring several admissions presented with recent worsening of symptoms . chest x - ray showed left lower lobe collapse and flexible bronchoscopy revealed stenosis of left main bronchus . foreign body was seen beyond the stenosis , which was removed after dilatation of narrowed bronchus under general anesthesia using rigid bronchoscopy . patient 's father gave a history of aspiration of whistle 14 years ago , which had then been removed . patient is now free of symptoms after removal of foreign body .

the majority of patients diagnosed with incompetent cervix can be treated successfully with a transvaginal cerclage . a select group of patients who have failed the vaginal approach , or have extremely short cervices , anatomically deformed cervix , deeply lacerated cervices , or severely scarred from previous failed vaginal cerclages may benefit from the transabdominal approach . the patient is a 36-year - old african - american woman ( gravida 2 , para 2 , aborta 0 , last menstrual period 4/1/03 ) referred from our maternal fetal medicine division for consideration for laparoscopic cerclage . the patient had a previous laparoscopy at age 20 with normal findings . on physical examination , the cervix was normal in appearance , the vagina was normal , and on bimanual examination the uterus was midposition and normal size , shape and contour with some uterine descensus . the patient was taken to the operating room after extensive counseling as to possible laparotomy and bleeding and underwent laparoscopic placement of an abdominal cerclage using a 5-mm mersilene band as is the usual suture used by our maternal fetal medicine ( mfm ) division . this included placing the 5-mm mersilene band around the internal os of the cervix , tying the knots anteriorly , and suturing 2 0 silk suture to the cut mersilene to prevent any unraveling ( figure 1 ) . ( a ) placing suture ; ( b ) tying the suture ; ( c ) completed suture : mersilene and silk ; ( d ) showing posterior placement ; ( e ) end of case after irrigation . she had some first trimester bleeding at 8 weeks in which the vaginal ultrasound confirmed cardiac activity . the patient 's prenatal course was significant for chronic hypertension , an abnormal glucose tolerance test that was diet controlled with fastings less than 100 and 2-hour postprandials consistently less than 120 . she began kick counting at 28 weeks and non stress tests were begun at 33 weeks . an elevated creatinine level was noted at 33 weeks and decreasing creatinine clearance was noted , for a diagnosis of preeclampsia . the patient was brought to the medical university of south carolina for bedrest and evaluation at 34 weeks . she underwent a low transverse cesarean delivery after lung maturity was confirmed , that day delivering a 5 pound 12 ounce female infant , apgar 8/9 . the patient tolerated the surgery well , and the infant and mother went home on postoperative day 3 , doing well . certainly , the usual treatment for women at risk for cervical incompetence is a cerclage placed transvaginally . when this approach has failed , or when this approach is not possible because of anatomic deformities , a transabdominal cerclage is a viable option . data clearly show an improved fetal survival rate compared with fetal survival in untreated pregnancies . the advantage of the abdominal approach is placement of the suture at the internal os with decreased movement . the obvious disadvantage is the need for 2 or even 3 laparotomies ( although some skillful nonlaparoscopic surgeons have managed to remove these sutures through a colpotomy , most can not ) should the fetus succumb in the second trimester . one must always keep the patient and fetus ' health first , and if unable to succeed laparoscopically , must be prepared to perform the abdominal cerclage via laparotomy to complete the procedure .

abdominal cerclages are necessary when the standard transvaginal cerclages fail or anatomical abnormalities preclude the vaginal placement . the disadvantage of the transabdominal approach is that it requires at least 2 laparotomies with significant morbidity and hospital stays . we discuss a case of abdominal cerclage performed laparoscopically . we feel it offers less morbidity and in the proper hands eliminates or significantly shortens hospital stays .

aneurysms of the internal carotid artery ( ica ) in the middle ear are uncommon vascular anomalies and are difficult to detect and treat the causes of aneurysms are multiple and include atherosclerosis and dysplastic , traumatic , and infectious lesions . differential diagnosis should include a glomus tumor ( tympanic , jugular or both ) , or other vascular temporal bone lesions such as a dehiscent jugular bulb , cholesterol granuloma , petrous carotid aneurysm , pseudoaneurysm , arteriovenous malformations or hemangioma . otoscopy may reveal a normal tympanic membrane with a mass in the middle ear that , in some cases , is pulsating . imaging studies are fundamental for diagnosis and can define the nature and origin of these lesions . high resolution computed tomography angiography ( cta ) is the technique of choice and , in some cases , can be complemented with a magnetic resonance angiography ( mra ) . in this article we describe an asymptomatic case of aneurysm of ica treated successfully by middle ear surgery . a 50-year - old female was referred to the emergency room of ent department of cluj- napoca for massive left otorrhagia started a few hours before presentation . there was no medical history of tinnitus , vertigo , otalgia , or otorrhea , or any history of trauma or surgery of the head and neck . recent psychological trauma ( death of her father ) with high blood pressure of 210/100 mm\hg was revealed at presentation . fresh blood flowed out from the left ear during a short period and hemostasis was accomplished with difficulty using compressive cotton balls . release of the compress resulted in relapse of the bleeding . a micro - otoscopic examination of the left ear showed a perforation at the level of the antero - inferior quadrant of the tympanic membrane with a red pulsatile mass . an audiogram revealed a left - sided conductive hearing loss of up to 30 db , whereas tympanometry showed a type c tympanogram with a low amplitude . computed tomography angiography ( cta ) showed an aneurysm of the internal carotid artery protruding into the middle ear and a massive hematoma of the mastoid cells . after the patient had been given a thorough explanation about the risks of ica injury during surgery , she provided an informed consent , and middle ear surgery was performed . a partial mastoidectomy canal wall down with evacuation of the left middle ear hematoma was performed ( figure 1 ) . a complete package of the middle ear and the mastoid cavity with merocel , surgicel , gelfoam and temporalis muscle fascia and a left anterior and posterior nasal package was also performed during surgery . after three days the patient underwent another computed tomography angiography . a complete obstruction by a massive thrombus of the left internal carotid artery at 1.2 cm from the bifurcation to clinoid area was revealed without any focal neurological deficits or stroke ( figure 2 ) . the anterior and posterior nasal package was suppressed two days after surgery . the follow - up magnetic resonance angiography ( mra ) 6 months later revealed the thrombosis of the left ica with new collateral vascularization in the left cerebral territory of ica by circle of willis ( figures 3 and 4 ) . the preponderance of men is clear in the literature , with a male / female ratio of 2:1.3 . the different causes and especially the traumatic lesions explain the relatively young age of this population , 56 years old . aneurysms of the extracranial carotid artery can be partially or completely thrombosed , can cause distal embolization , or compression of adjacent structures , and can be ruptured . before the introduction of antibiotics , dysplastic lesions appeared to be the main cause of aneurysms of the ica . in the experience of lotina et al . depending on the size and location of the aneurysm , the direction of its growth , and the specific adjacent structures involved , patients may or may not present signs and symptoms . our patient direct compression or anterior and posterior nasal package may not be able to stop the bleeding . in that situation a patient may enter a critical condition following hemorrhagic shock or respiratory failure . in our case , the appropriate management strategy for these rare lesions is unclear . in the cases reported until now most patients were treated with revascularization of the carotid artery using vein bypass grafts to reduce the risk of acute ischemic complications , but there are also cases treated without vascularization . successful treatment usually involves selective aneurysm embolization or carotid closure with detachable balloons . in our case middle ear surgery was effective in resolving bleeding and did not cause any permanent neurological deficit . because of the high blood pressure and the thrombosis of the left ica after surgery an antihypertensive ( enalapril 10 mg ) and antithrombotic ( aspirin cardio 100 mg ) chronic medication was mandatory in this case . aneurysms of the ica in the middle ear are rare and difficult to treat because the anatomy of the region and life threatening complications after surgery . otologists should be aware that symptoms and signs such as pulsatile tinnitus , conductive hearing loss , and a pulsatile retrotympanic mass in the anteroinferior part may be related to an ica aneurysm . thus , ica aneurysm , which is asymptomatic most of the time , will be diagnosed during middle ear surgery or a routine otoscopy or complications like otorrhagia . misdiagnosis of the ica aneurysm may lead to serious morbidity because of bleeding or vascular occlusion . long - term follow - up is necessary to look out for delayed post treatment complications .

middle ear aneurysms are rare and difficult to treat . the case of a 50-year - old female who presented with left otorrhagia caused by an internal carotid aneurysm is reported . she had no medical history of tinnitus , vertigo , otalgia or otorrhea . middle ear surgery was effective in resolving bleeding and did not cause any permanent neurological deficit . high resolution computed tomography angiography is the technique of choice and , in some cases , can be complemented with a magnetic resonance angiography . misdiagnosis of the internal carotid artery aneurysm may lead to serious morbidity because of bleeding or vascular occlusion . the use of modern imaging techniques explain the current relative increase in frequency .

is there a formula for turning young people from diverse backgrounds into scientists ? as our country s demographics change , and as so - called minorities become majorities in many locations , it might be argued that our cultural differences make a one - size - fits - all educational strategy sound nave and even insensitive . yet there are some similarities among successful youth programs that might serve as good examples to follow . one program that is showing great promise in engaging urban teens of various ethnicities in meaningful science activities , including some citizen - science work , and steering them into science , technology , engineering , and mathematics ( stem ) careers is the science career continuum ( scc ) at the chicago botanic garden . the scc is an extracurricular program that strives to prepare a new generation of scientists , representing all ethnic backgrounds , and provide them with the education and training needed to address the environmental and conservation challenges of our time . for 20 years , this grant - funded program for students attending chicago public schools has offered an opportunity to explore science - related programs at the chicago botanic garden ( the garden ) . participant selection has targeted african - american and latin american youth who have expressed an interest in science and nature , with preference given to students from lower - income households and who are the first in their families to attend college . most of these students do not have family members who could help them make college selection and course choices for science majors because they are unfamiliar with the field . the scc includes three programs that a student may participate in for five or more years . it begins with science first , a four - week summer day camp for 40 students who are entering grades 8 , 9 , or 10 in the fall . students engage in the content and practices of science while also studying environmental issues in their lives such as the impact of climate change or the availability of quality grocery stores and health care services in urban neighborhoods . recent projects have included measuring the effectiveness of different insulating materials , testing and comparing the effectiveness of different weed - control practices , and studying the pollinator - attraction properties of flowers . twenty students in total are selected to continue with an eight - week program , college first . they may be those who showed the most enthusiasm and promise while participating in science first , similar qualified students from comparable programs at other institutions , or a mix of the two . selection is based on the work students contributed to the science first research project or city science fair , class participation during the summer , and other behaviors that demonstrate a commitment to returning to the garden for a summer of engagement with a summer science research project before their junior and senior years of high school . college first combines a college environmental field study course with an extended research project working alongside garden professionals . some students may perform dna tests and analyze results to assist researchers in measuring the genetic diversity of a jackfruit tree population in bangladesh , which is a concern of the agricultural community there , while other students may assist with ongoing research on the impact of the invasive asian buckthorn tree and its ability to alter soil and air conditions and harm native species . still other students might help their mentors assess the conditions of local ravines in order to recommend remedial work , or sprout seed samples from the seed bank collection in order to measure the viability of seeds exposed to long - term extreme cold storage . college first students also attend monthly meetings during the school year to learn about college selection , application , and the financial aid that they will need to pursue their passion for doing this kind of work . the third formal program in the scc is a ten - week research for undergraduates ( reu ) program . three students who have successfully completed college first , are declaring a science major , and have demonstrated academic achievement in college are selected for the program . students work with scientists and graduate students to do independent research on topics related to their mentors research , such as plant pathology , genetic diversity , or soil quality . garden educators have used a variety of methods and indicators to determine student progress and program effectiveness , including content knowledge assessment , attitude surveys , and science grades in school . 100% of scc students graduate high school , as compared with the chicago public school rate of 66% in 2014 ( 1 ) . furthermore , 94% of scc alumni enrolled in college within a year after high school graduation , with 66% choosing to major in a stem field . to understand what makes the scc and similar programs successful , the garden gathered together 32 institutions with stem - focused youth programs to compare successes and identify best practices for encouraging students to pursue stem careers . the hive chicago - funded pathways to stem success ( 2 ) wrapped up in early 2015 with a list of recommendations for successful recruitment , retention , and release of students from our programs . first , scc identifies individuals who demonstrate interest and aptitude in science while they are in middle school , when they are beginning to understand their own interests . students at this age may be influenced by peers who do not share the same interests and may discourage science - minded students from participating in science at school . the scc pools students from different schools into an affinity group of diverse students that will encourage each other s passion for the subject matter . students are introduced to stem careers early in the program so they have time to acclimate to the idea that this may be an exciting and attainable career choice . being able to do the work alongside science professionals and college students enables participants to imagine working as professionals in these fields one day . the scc makes science learning accessible by providing transportation and financial support so that there is no cost to the students . moreover , it conquers emotional barriers by establishing a safe community of like - minded students who come from different backgrounds and feel comfortable sharing their interests with each other . college first students get to work with scientists , professionals , and older students , participating in science research , horticulture , and interpretation programs that are happening every day at the garden . a second critical element in any stem pathway program is retention of students . the scc achieves continuity through the multi - year format and by providing challenging educational experiences with increased expectations each year . students receive mentoring from professional staff as well as students who are at the next level of the program their near peersreinforcing the continuity , accessibility , and nurturing aspects of the program . this support extends to the group of students within their own cadre . a recent youth program quality assessment ( ypqa ) the ypqa is a self - assessment tool used to measure a program s strengths and weaknesses based on a set of established metrics for successful youth programs . the ypqa examines student engagement , safety , rapport with instructors , personal interaction , and other factors that prove significant in youth programs . the study is used internally by the institution to improve the program and , as such , is not published . the scc ypqa revealed that students form a bond of friendship and stay in touch with each other outside of the program . this is despite coming from different ethnic backgrounds , attending different schools , and living in distant neighborhoods , and demonstrated for garden staff that the program is building support between program participants it is not enough to offer a program and turn students loose without any further support . best practices tell us that how we release students is as important to their success as how we recruit them . scc staff members remain in contact with alumni and help connect these former students to internships and other opportunities as appropriate . best practices also dictate that programs should be flexible to various outcomes of success for participants . scc staff members recognize that not all students will become scientists , but all of them leave the program more confident in their abilities , more scientifically literate , and motivated to pursue their interests . the scc has shown that when students from diverse backgrounds are given an opportunity to participate in real scientific research under the mentorship of a caring professional over multiple years , they are better able to envision themselves pursuing stem careers . by establishing a community of learners who share a passion for science and support each other s personal achievement , the scc expands participants notions of what is possible and brightens their futures , regardless of career choices they make .

the science career continuum at the chicago botanic garden is a model program for successfully encouraging youth from diverse backgrounds into stem careers . this program has shown that when students are given an opportunity to participate in real scientific research under the mentorship of a caring professional over multiple years , they are more likely to go to college and pursue stem careers than their peers . journal of microbiology & biology education

the truth is that most medical breakthroughs have ultimately come from basic research , and i think that we as scientists need to do a better job of telling these success stories . my favorite example is that antibiotics came out of finding a random contaminant on a plate . and remember that we have crispr [ clustered regularly interspaced short palindromic repeats ] tools only because people were studying some bizarro antipathogen mechanism in bacteria . it s a beautiful example of something entirely unanticipated coming out of left field . that s why it s important to maintain funding for a very diverse program of basic research ; history tells us you ca nt predict where the next advance will come from . right now it seems like funding agencies and the public have an engineering mentality , and they believe that , as long as we think hard enough about a problem , we can solve it . that s an admirable attitude , but in the life sciences it s not always the right path to discovery . i would argue that under some circumstances , studying yeast cells is a better idea than studying highly transformed human cells in a dish . for many years , we used to write in the significance section of grant applications that the reason that it is important to understand chromosome segregation is because , when it fails , cells become aneuploid and cancerous . why should aneuploidy cause cells to proliferate ? when you do yeast crosses and by chance get an aneuploid strain , invariably the cells do poorly . and one explanation is that building an organism is a complicated procedure , so if you change the gene dosage of certain genes , you mess everything up . that s probably true you could then further argue that , at the cellular level , maybe it does nt really matter how many chromosomes you have , as long as you have at least one of each . perhaps extra chromosomes are actually good for an individual cell and allow it to keep proliferating when they should not and this gives you cancer . however , we never thought this was very likely , given that aneuploid yeast often proliferate poorly . you can very agnostically ask : what happens to a cell if you change the chromosome number ? yeast was the right organism for this because we have genetic tricks and selection methods to generate aneuploids that are stable enough to study . because this allowed us to examine many different types of aneuploidies at the same time , we could look for broad patterns . we could nt have done it in any other organism . actually , we do all our discovery work in yeast . and when we find something interesting , we move it into the mouse or human cell lines and ask whether we see the same thing . sometimes the answer is no , but more often the answer is yes . it s just harder to discover fundamentally new things in very complex organisms where the repertoire of tools is limited . and i would argue that , under some circumstances , studying yeast cells is a better idea than studying highly transformed human cells in a dish . angelika amon s work over the past 15 years has guided all of us who think about mitotic and meiotic cell division . , we wondered whether there was a systematic way the cell avoids losing or gaining chromosomes . to be honest , i was hoping there would be some kind of cute mechanism that counts chromosomes . that turned out to be completely wrong . or at least we havent been able to find anything of this sort . once we realized that , we had to step back and let the genetics lead the way . we did a lot of phenotypic characterization and then developed hypotheses to explain the patterns we saw . if complex subunits are not expressed in the correct ratios , often unassembled subunits need chaperones to maintain their soluble state and eventually need to be degraded if they can not find a binding partner . cancer cells do nt only lose or gain whole chromosomes , they also have translocations and deletions and point mutations . we know that making and repairing dna requires a lot of nifty multi - subunit complexes , so our hypothesis was that whole - chromosome abnormalities cause stoichiometric imbalances in these complexes and this leads to defects in their formation and function . so we re testing the idea that aneuploidy contributes to cancer because it s a mutator , rather than because it s a promoter of growth and proliferation , and we re doing more and more mouse work to answer those kinds of questions . but we still use yeast to discover new properties of aneuploidy and to look at the cellular effects of changing the dosage of specific genes . we started a new project on mitochondria a few years ago that i m really excited about . one big question is : how did these two organisms start talking to each other ? did one learn english or the other learn french ? or did they resort to a more primitive sign language metabolites to talk to each other ? the other thing i m fascinated by is that a large fraction of uncharacterized yeast genes localize to mitochondria . that tells you there s a lot of biology in the mitochondria still to be learned . what i think is even more important is that many of these genes are not at all conserved , not even in fungi . so here are all these very new , very fast - evolving genes and nobody knows what they do ! usually when people hear that a gene is not conserved , they run ! arrgh ! it s something yeast specific ! but in this particular instance i think it s important and suggests mitochondria are still battling it out with the nucleus . one way to look at it is to say the nucleus and mitochondria are not really symbiotic . if mitochondria do nt get anything out of the relationship , they are really just slaves to the nucleus . so the mitochondria are trying to escape the reign of the nucleus and the nucleus is fighting back in an arms race . and if we can understand what all these nonconserved mitochondrial genes are doing , we can begin to find the frictions that still exist between the interests of the nucleus and the mitochondria . i m totally fascinated by understanding how these different entities interact with each other inside a single cell .

the genetics society of america medal is awarded to an individual for outstanding contributions to the field of genetics in the past 15 years . recipients of the gsa medal are recognized for elegant and highly meaningful contributions to modern genetics . the 2014 recipient , angelika b. amon , has uncovered key principles governing the cell cycle and was the first to demonstrate a connection between the physical completion of anaphase and the initiation of mitotic exit . more recently , her research has focused on the consequences of aneuploidy . genetics spoke with dr . amon about her approach to science and what is next on the horizon .

ventriculo - peritoneal ( vp ) shunting is the preferred and most successful method for managing congenital hydrocephalus . abdominal complications include peritonitis , ascites , bowel obstruction , bowel and abdominal wall perforation , and inguinal herniae . an uncommon but recognized complication is formation of an abdominal pseudocyst , with cerebrospinal fluid ( csf ) collecting and being poorly absorbed or not absorbed across the serosa . the continuous flow of csf within a confined space leads to increased pressure within the abdominal cavity , reducing forward pressure gradient and , eventually , shunt malfunction . these pseudocysts have traditionally been treated with surgical shunt externalization , antibiotics , and a second surgical procedure for shunt reinsertion . exploratory laparotomy with a partial excision pseudocyst and placement of a catheter in a quadrant of abdomen is also an option . we report a 20-month - old female child with a vp shunt and a gradual distension of abdomen since three months , respiratory distress , and bilious vomiting for two days . right - sided vp shunt insertion was done on the seventh day of life and meningomyelocele repair on the 15 day of life . at the age of three months , there was shunt malfunction for which the right - sided vp shunt was removed and a vp shunt was inserted on the left side . at the time of index admission , she presented with a history of gradual distension of abdomen for 3 months and respiratory distress and bilious vomiting for 2 days . , there was a large , 15 cm 12 cm , nontender , cystic intraperitoneal lump with a smooth surface and ill - defined margins , occupying almost the entire abdomen ; moreover , fingers could be insinuated between the costal margins and the lump . x - ray of the abdomen showed vp shunt in the left side of the abdominal cavity having ground glass opacity around the shunt , with the displacement of bowel loops to the periphery of the opacity [ figure 1 ] . abdominal ultrasonography ( usg ) revealed a large multiseptate pseudocyst with the presence of the shunt within . as the baby had considerable respiratory distress , an ultrasound - guided aspiration of 200 ml of a straw - colored fluid was done . however the fluid recollected immediately with increasing abdominal distension and the symptoms persisted ; hence , we decided to operate on the child . x - ray abdomen showing a giant psuedocyst on the left lower abdomen with a shunt in situ we went ahead with two - port laparoscopy ; one 5-mm primary epigastric camera port above the cyst placed under vision and one 5-mm port in the right iliac fossa . the placement of the second port was difficult , as there was a large csf pseudocyst predominantly on the left side of the abdomen , with multiple adhesions of bowel loops around the pseudocyst . gentle maneuvering and dissection with the scope itself helped identify a window in the right iliac fossa where the second port could be placed . a grasper was used to gently free all adhesions of the cyst wall to parietes . the cyst was incised with a hook cautery and 1,100 ml of the csf fluid was drained . the incision in the cyst wall was extended carefully so as to avoid injury to the adherent bowel loops , and the cyst was then deroofed . the shunt was repositioned in the right subdiaphragmatic space over the liver after confirming the patency of shunt with a free flow of the csf . . on sixmonth follow - up , the child is recovering well and is asymptomatic . insertion of a vp shunt is the preferred and most successful method for the management of congenital hydrocephalus . in the setting of a vp shunt , the time from the last shunt procedure to the development of the abdominal csf pseudocyst ranges from 3 weeks to 5 years . a chest and abdominal x - ray visualizes the position of the thoracoabdominal part of the vp shunt . pathi reported that ultrasound and computerized tomography features of the vp shunt floating in the thickened sac wall would allow early recognition of this complication . these pseudocysts have traditionally been treated with surgical shunt externalization , antibiotics , and a second surgical procedure for shunt reinsertion . moreover , exploratory laparotomy with partial excision and marsupialisation of pseudocyst and placement of catheter in a quadrant of abdomen has been done . an extensive review revealed increasing use of laparoscopic management of this complication . using the technique of laparoscopy , kim et al . excised a portion of the csf pseudocyst , removed the shunt catheter from the residual cavity , and repositioned it within the peritoneal cavity in a 12-year - old boy . ( 2003 ) found that the laparoscopic management of csf pseudocyst , accomplished through 3-mm to 5-mm ports with the help of delicate laparoscopic instruments , minimizes the risk of a laparotomy , and the formation of intraperitoneal adhesions . furthermore , laparoscopy allows visual confirmation of the adequate flow of the csf from the end of the catheter after it is repositioned . however , the greatest advantage of laparoscopy lies in its ability to assess the entire abdominal cavity for the presence of adhesions and undertake adhesiolysis when necessary . this allows placement of the catheter in the quadrant of the abdomen with the maximum absorptive surface . martin et al . performed laparoscopy in ventriculoperitoneal shunt revision for pseudocyst in two cases and reported the use of extensive adhesiolysis for decreasing the risk of future adhesive obstruction and extending shunt life and thus , recommended laparoscopy for vp shunt revisions . performed the laparoscopic drainage of a pseudocyst containing 2 liters of fluid and retrieved the catheter from the peritoneal cavity . esposito et al . recommended that laparoscopy is a safe procedure in patients with abdominal complications of vp shunts , especially in adhesions and pseudocyst formation . on extensive search and review of literature , we found that minimally invasive technique is a useful modality for the treatment of this complication . however , the management of this complication is generally done using three ports ( one camera port and two working ports ) . we recommend that even giant pseudocysts can be managed by the two - port laparoscopic technique as done in another child with giant csf pseudocyst as well . to summarize , laparoscopy is a useful modality for the treatment of intra - abdominal complications of vp shunt such as adhesions and pseudocyst . it is effective , avoids multiple laparotomies , and its attendant complications and future adhesions . it is feasible to manage this complication by using only the two - port laparoscopic technique .

insertion of a ventriculo - peritoneal ( vp ) shunt is the most common operative procedure for the treatment of hydrocephalus in children . of the several causes of shunt malfunction , cerebrospinal fluid ( csf ) pseudocyst is relatively uncommon . there are several modalities to treat a csf pseudocyst . laparotomy is required , at times , more than once . we managed a patient of csf pseudocyst with two - port laparoscopy , by deroofing the psuedocyst and repositioning of the shunt . this minimally invasive technique avoids morbidity associated with laparotomy and aids in early recovery .

intriguingly , mtorc1 activity only regulates the expression of nedd41 but not of nedd42 , the closest homolog of nedd41 in the mammalian genome , but the molecular mechanism underlying this isoform preference is unknown . mtorc1 promotes global cap - dependent protein synthesis mainly by mediating the phosphorylation of eukaryotic initiation factor 4e binding protein 1 and 2 ( 4e - bp1 and 4e - bp2 ) . 4e - bps bind to eukaryotic initiation factor 4e ( elf4e ) to prevent the formation of protein initiation complex and protein translation . phosphorylation of 4e - bps leads to decreased affinity of 4e - bps to eif4e . this enables eif4e to be anchored to the 5-cap structure of mrnas , promotes the formation of protein initiation complex , and initiates protein synthesis . of note , the 5 untranslated region ( 5'utr ) of the nedd41 mrna ( genbank accession number : nm_010890 ) contains a pyrimidine - rich sequence stretch that is related to the 5 terminal oligopyrimidine ( 5top ) motif . this 5top motif is important for anchoring elf4e to the 5-cap structure of mrnas , to direct the assembly of the translation machinery , and to initiate protein synthesis . the pyrimidine - rich sequence in the 5utr of nedd41 mrna may play a similar role as the 5top motif in initiating the translation of nedd41 mrnas in an mtorc1 activity - dependent manner ( fig . further evidence supporting this model includes a study employing ribosome profiling to examine the translational efficiency of specific mrnas in the presence of an mtorc1 inhibitor , which showed that the translational efficiency of nedd41 mrna is reduced in non - neuronal cells upon mtorc1 inhibition . figure 1.model of the regulation of nedd41 translation by mtorc1 . in response to growth factor signaling , . elevated ptdinsp3 levels lead to the phosphorylation and activation of akt , which then activates mtorc1 . phosphorylation of 4e - bps by mtorc1 reduces the binding of 4e - bps to eif4e , enabling eif4e to bind to the 5'utr of target mrnas and thus initiate protein synthesis . the 5'utr of nedd41 mrna ( see sequence in the dialog box ) contains a pyrimidine - rich sequence stretch ( underlined ) after the putative transcriptional start site ( red ) , which is related to the 5 ' terminal oligopyrimidine ( 5'top ) motif . this pyrimidine - rich sequence may play a key role in starting the translation of nedd41 mrnas in an mtorc1 activity - dependent manner . nedd41 is a positive regulator of neurite growth and may have additional roles in regulating axon pathfinding , spine formation , and synaptic plasticity . in response to growth factor signaling , pi3k catalyzes the phosphorylation of ptdinsp2 to generate ptdinsp3 . pten converts ptdinsp3 back to ptdinp2 and thus antagonizes the effect of pi3k . elevated ptdinsp3 levels lead to the phosphorylation and activation of akt , which then activates mtorc1 . phosphorylation of 4e - bps by mtorc1 reduces the binding of 4e - bps to eif4e , enabling eif4e to bind to the 5'utr of target mrnas and thus initiate protein synthesis . the 5'utr of nedd41 mrna ( see sequence in the dialog box ) contains a pyrimidine - rich sequence stretch ( underlined ) after the putative transcriptional start site ( red ) , which is related to the 5 ' terminal oligopyrimidine ( 5'top ) motif . this pyrimidine - rich sequence may play a key role in starting the translation of nedd41 mrnas in an mtorc1 activity - dependent manner . nedd41 is a positive regulator of neurite growth and may have additional roles in regulating axon pathfinding , spine formation , and synaptic plasticity . accumulating evidence indicates crucial roles of local protein synthesis in various aspects of normal neuronal development and function , including axon guidance , spine formation , and synaptic plasticity . all of these processes are highly dynamic and require spatially and temporally well - regulated and accurate changes in local protein levels in response to various stimuli . locally regulated translation of mrnas provides an efficient way to spatially and temporally control local protein composition , which is of particular importance in distal neurites , where proteins synthesized at the soma may not be an economic source . the finding that nedd41 expression is regulated at the translational level is compatible with the notion that nedd41 mrnas are major targets of the local translational machinery in neurons . if so , it is very likely that nedd41 does not only regulate neurite growth regulation , as we reported previously , but may have important additional roles in axon guidance , spine formation , and synaptic plasticity . indeed , drosophila melanogaster nedd4 regulates commisureless , which is an essential protein regulator of slit / robo signaling during axon pathfinding and midline crossing . moreover , synaptic activation has been shown to cause a site - specific increase in nedd41 expression , which may mediate the ubiquitination and endocytosis of the ampa receptor subunit glua1 . in view of these findings , additional studies , ideally with higher spatiotemporal resolution , on how nedd41 expression is regulated in response to extracellular signals is very likely to provide further important insights into the physiological roles of nedd41 in neurons . this work has been supported by grants from the german research foundation ( spp1365/ka3423/11 ; to h.k . ) and the fritz thyssen foundation ( to h.k . ) .

we found recently that mtorc1 regulates the biosynthesis of the ubiquitin e3 ligase nedd41 , but not the close homolog nedd42 . this regulatory process plays a key role in promoting neurite growth in neurons of the mammalian central nervous system . the molecular mechanism underlying this rather specific regulation likely involves a pyrimidine - rich sequence stretch near the putative transcriptional start site within the 5 untranslated region of the nedd41 mrna , which may play a crucial role in directing the assembly of the protein translation machinery . we postulate that the nedd41 mrna is a major target of the local translation machinery within neurons that can be translated in a spatially and temporally controlled manner in response to various stimuli . based on this model , neuronal nedd41 may not only be involved in the regulation of neurite growth but also in axon guidance , spine formation , and synaptic plasticity .

hypothermia defined as a core body temperature < 35 can be a life - threatening emergency , especially in cold winter climates , but can be used therapeutically . therapeutic hypothermia ( th ) has been used to improve neurological outcome in comatose patients after cardiac arrest.1)2 ) the exact mechanism for neuroprotection is unclear ; potential mechanisms include attenuation of deleterious processes occurring after initial hypoxic tissue injury.3)4)5 ) generally , mild hypothermia is defined as a core body temperature between 32 and 34.2 ) this is thought to be appropriate temperature between neuroprotective effects and possible toxicities . the osborn wave ( also known as the j wave ) it is not pathognomonic of hypothermia , but usually occurs in patients with a core temperature < 32 and the magnitude of the j point elevation correlates inversely with the body temperature.6 ) several studies have suggested an arrhythmogenic potential of the osborn wave,7)8)9)10 ) but there is no solid evidence that th - induced osborn wave itself is associated with increased mortality.11)12 ) here we report a case of recurrent ventricular fibrillation ( vf ) after appearance of osborn wave during mild th . a 56-year - old man with no underlying disease was admitted to the emergency department with sudden cardiac arrest . while having breakfast , he suddenly pounded his chest . he received cardiopulmonary resuscitation ( cpr ) from his son for about 20 minutes until paramedics arrived with a defibrillator . 1 ) . defibrillation with 200 j was delivered once followed by 5 cycles of cpr . following restoration of cardiac rhythm , he was transferred to our hospital for further management . at our hospital , initial ecg ( fig . 2a ) showed sinus tachycardia with a heart rate of 114 beats per minute . on the first day of admission , echocardiography was done , which showed no regional wall motion abnormality of the left ventricle . the initial ph was 7.375 and the levels of electrolytes including sodium , potassium , calcium and magnesium were within the normal ranges . the target temperature was 33. soon after th was begun , osborn waves appeared in lead ii and v 4 , v 5 , and v 6 ( fig . two hours after th , vf occurred when the esophageal temperature was 34.9. defibrillation of 150 j was applied ( fig . 2c ) . not much later atrial fibrillation developed and the osborn waves were prominent in diffuse leads . three more defibrillations were delivered to terminate vf and the height of osborn waves increased over time ( fig . two hours after a warmer was applied to the patient , ecg revealed less prominent osborn wave and vf did not recur . he was diagnosed with idiopathic vf and discharged from our hospital after implantable cardioverter defibrillator insertion ( fig . it is generally accepted that th has the potential to improve the neurological outcome in comatose patients after cardiac arrest.1)2 ) following initial resuscitation , various mechanisms including ischemia , reperfusion injury , inflammatory response , and free radical production are involved in cerebral damage.1)3)5 ) th lowers the cerebral metabolic rate , blocks the apoptotic pathways , suppresses inflammatory responses , and attenuates production of free radicals.3)4)5 ) however , possible side effects , such as electrolyte imbalance , hemodynamic instability , infection , seizures , and arrhythmias , can rarely occur in patients undergoing th.1)2)5 ) electrocardiogram findings of hypothermia include osborn waves , atrial and ventricular dysrhythmias , and interval ( pr , qrs , qt ) prolongations.11)13)14 ) among these , the osborn wave is a positive deflection occurring at the qrs - st junction and may originate from a transmural voltage gradient during early repolarization due to a transient outward potassium current in the epicardium , but not in the endocardium.15 ) as the osborn wave can be seen in various conditions like acidosis , subarachnoid hemorrhage , and hypercalcemia , it is not pathognomonic of hypothermia . but , it has been reported that the height of j point elevation is related with the severity of hypothermia.6 ) several mechanisms have been suggested to explain the arrythmogenic potential of the osborn wave . the prominent action potential notch can lead to loss of the dome of action potential , which can heterogeneously cause a marked dispersion of repolarization . this eventually can cause extrasystolic activity by a mechanism called phase 2 reentry and vf.15)16 ) another proposed mechanism is triggered automaticity . intracellular ca overload during hypothermia is a precursor for early or delayed after depolarization , which is associated with triggered activity.17 ) however , other reports have not associated the osborn wave with vf in hypothermic patients.18 ) therefore , the arrythmogenic potential of the osborn wave requires further study . although various adverse events have been reported during th , th - induced arrhythmia seems not to be related with increased mortality.1)2 ) in one study of ecg changes in th , the osborn wave incidence during th was 30% and no association with unfavorable short - term outcome was evident.11 ) after rewarming , the osborn wave disappears in almost cases . so , on the basis of many studies , th is well tolerated and relatively safe . hypothermia is also proarrhythmic and can cause lethal arrhythmias , especially at low temperature and in the presence of low serum ph.19 ) however , in our case , vf developed in normal serum ph , electrolyte levels , and not markedly low body temperature . therefore , vf and osborn wave seem not always to be related to body temperature , and individual variations in response to hypothermia can be more important to determine the vulnerability to arrhythmias . physicians need to monitor patient 's vital signs including core temperature , ecg , and laboratory tests carefully during th .

therapeutic hypothermia ( th ) has been used to protect neurological functions in cardiac arrest patient . although osborn wave is not pathognomonic of hypothermia , it is a well - known electrocardiogram finding of hypothermic patients . the cellular and ionic mechanisms of the osborn wave have been suggested , and its relationship to tachyarrhythmias , such as ventricular tachycardia and ventricular fibrillation , is being explored . this case highlights the arrhythmogenic potential of osborn wave and individual difference in response of th .

angioma serpiginosum is a benign vascular nevoid disorder with proliferation and ectatic dilatation of capillaries in the papillary dermis . most cases are sporadic but familial cases suggesting an autosomal dominant inheritance have also been described . we report a case of late onset angioma serpiginosum localised to the breast , an unusual site . a 46-year - old female patient presented to the dermatology out - patient clinic with an asymptomatic , progressive red eruption on the right breast of 4 years duration . she did not provide a history of bleeding disorder , preceding trauma , or contact allergy prior to the eruption of the lesions . clinical examination revealed multiple punctate macules in a serpiginous pattern against a bluish background , grouped at places , located on the lateral half of the right breast . a 3 mm , red , soft papule was noted overlying these lesions at their lower extent [ figure 1 ] . multiple , punctate macules in a serpiginious pattern against a bluish background on the right breast with a red smooth papule at the inferior aspect the differentials considered were angioma serpiginosum , unilateral nevoid telangiectasia , pigmented purpuric dermatoses and telangiectasia macularis eruptiva perstans . epiluminescence microscopy with heine delta 20 dermatoscope ( heine optotechnik , herrsching , germany ) revealed multiple well demarcated oval to round red lagoons [ figure 2 ] . epiluminescence microscopy ( 20 ) revealing the well demarcated red lagoon appearance histopathological examination of the nonblanching punctate macules showed a normal to mildly orthohyperkeratotic epidermis with dilated thin walled capillaries in the papillary dermis . there was no evidence of extravasation of erythrocytes , inflammatory cell infiltrate or deposition of hemosiderin in the surrounding tissue [ figure 3 ] . periodic acid - schiff ( pas ) stain showed a thick cuff of amorphous acidophilic pas - positive diastase - resistant material surrounding the dilated vessels [ figure 4 ] . the red papule overlying the punctate macules revealed a well delineated papillary dermal lesion composed of closely placed ectatic thin walled capillaries engorged with erythrocytes that was consistent with a diagnosis of cherry angioma [ figure 5 ] . the clinical examination complemented by epiluminescence microscopy and she was advised ophthalmic examination , which was refused and no further treatment was sought . dilated thin walled capillaries ( arrow ) in the superficial papillary dermis with an unremarkable deep papillary and reticular dermis . there is no extravasation of erythrocytes , inflammatory cell infiltrate or deposition of hemosiderin in the surrounding tissue ( h and e , 200 ) cuff of periodic acid - schiff ( pas ) positive diastase - resistant material ( arrow ) surrounding the dilated vessels ( pas , 200 ) well delineated papillary dermal lesion composed of closely placed ectatic thin walled capillaries engorged with erythrocytes ( h and e , 100 ) first described by hutchinson in 1889 and named by radcliffe - crocker in 1893 , angioma serpiginosum consists of multiple red , minute , nonblanchable and grouped macules , resembling purpura , in a serpiginous or gyrate pattern with a background of erythema or violaceous hue . . the erythematous or violaceous background hue may be due to dilatation of the subpapillary venous plexus . the eruption usually affects teenage females and in 90% cases has its onset before the age of 16 years . it commonly affects the lower extremities and buttocks and is often asymmetric . any anatomic site with exceptions of the mucocutaneous junctions , palms and soles can be affected , though there has been a case report describing plantar involvement as well as reports of disseminated distribution . majority of cases occur in females and are of childhood onset . in view of female preponderance and progression of lesions in pregnancy , the role of hormonal stimuli has been refuted by the absence of estrogen - progesterone receptor stimulation . epiluminescence microscopy reveals demarcated red lagoon appearance due to the presence of increased and dilated vascular spaces in the papillary dermis and can help distinguish the condition from purpuric dermatoses on the basis of typical lagoon pattern . histopathological findings include a normal epidermis , dilated thin walled capillaries in the papillary dermis without an extravasation of red blood cells or deposition of hemosiderin in the surrounding tissue . the capillary walls may be thickened due to deposition of pas positive material around blood vessels . the absence of dermal mast cell increase , hemosiderin deposition and red blood cell extravasation in our case ruled out the differential diagnoses that were considered . it was first described by campbell de morgan in 1872 . generally developing after the third decade clinically , early lesions appear as flat , red macules that look like petechiae that later evolve into 1 - 5 mm red papules . these are known to be related to chemical exposures , with liver transplants , graft - versus - host disease , and secondary to cyclosporine treatment . in the absence of any known inciting factors , it is assumed that our patient had an incidental onset of solitary cherry angioma or as a result of chronological ageing . histological examination of cherry angiomas shows numerous , newly formed capillaries in a lobular pattern in the papillary dermis . there have reports of two female patients with chest wall involvement with the onset at 7 and 9 years of age . chest involvement has also been described as a part of disseminated lesions in three patients . two of these patients were beyond 50 years of age at presentation with the onset of their lesions in early childhood . angioma serpiginosum has been described to be associated with angiokeratoma of the vulva in contiguous areas . however , there has not been any report of cherry angioma in association with lesions of angioma serpiginosum or overlying the lesions . our patient presented with a solitary cherry angioma superimposed on the angioma serpiginosum with the onset beyond 40 years of age and on the breast that is an unusual site for the latter . to the best of our knowledge , there has been no reported case of this entity with late onset lesions in the english language literature . co - existence of cherry angioma is assumed to be a result of chronological ageing process or may be secondary to a common vascular origin of both these lesions .

angioma serpiginosum is a cutaneous vascular nevoid disorder that presents as red , nonblanchable and grouped macules distributed in a serpiginous pattern and resembles purpura . it usually begins in childhood and commonly affects the lower limbs and the buttocks . late onset of the lesions is unusual . we report a 46-year - old female who presented with progressively extending lesions on her breast since preceding four years . she also had a cherry angioma overlying the lesions , an observation hitherto not reported so far .

acute cytomegalovirus ( cmv ) infection is common in both immunocompromised and immunocompetent patients worldwide.12 the infection is often asymptomatic . symptoms , when they occur , include fever , cervical lymphadenitis , and arthralgia.3 rarely , acute cmv infection may be complicated by pneumonia , colitis , myocarditis , pericarditis , or hemolytic anemia.4 vascular events caused by acute cmv infection , including thrombosis of the venous or arterial vascular system , are rarely reported in the english literature . we report a case of acute cmv colitis in an immunocompetent adult that was complicated by vascular thrombosis and pulmonary embolism . further , we present a review of the literature regarding the association between vascular events and cmv infection in immunocompetent patients . a 78-year - old man was referred to our emergency department with a 3-day history of diarrhea and fever . he had no recent travel history , did not use steroids , and had not experienced recent trauma . he used medication for hypertension and diabetes mellitus , both of which were well controlled . on admission , his vital signs were : body temperature , 38.8 ; blood pressure , 145/85 mmhg ; respiratory rate , 18/min ; and pulse rate , 90/min . on physical examination , his pulse was regular and he had no cardiac murmurs , his chest was clear without signs of respiratory distress , and his abdomen was soft with hyperactive bowel sounds . laboratory tests showed a white blood cell count of 8,270/mm ( normal : 3,99010,500/mm ) , a platelet count of 199,000/mm ( normal : 140,000450,000/mm ) , a prothrombin time of 14.10 seconds ( control : 11.16 seconds ) , an inr of 1.21 , an activated partial thromboplastin time of 26.0 seconds ( control : 27.4 seconds ) , and crp level of 16.7 mg / dl ( normal : < 0.8 mg / dl ) . a coagulation profile , including protein c ( 80% , normal : 70%140% ) , protein s ( 75% , normal : 60%130% ) and anti - thrombin iii ( 95% , normal : 75%125% ) , was normal . . colonoscopy demonstrated multiple giant ulcers with skip lesions in the distal colon , sparing the rectum ( fig . multiple forceps - biopsy tissue samples , taken from the coloniculcers , were sent for pathological examination . however , 5 days post - colonoscopy , the patient experienced dyspnea and severe hypoxemia , necessitating emergent endotracheal intubation and ventilation . a chest radiograph obtained post - intubation revealed an engorged main pulmonary trunk with an abrupt cutoff of pulmonary vascularity in the distal portions bilaterally , indicative of the " westermark sign " ( fig . subsequently , a ct scan of the chest was performed that showed several filling defects within the pulmonary trunk and main pulmonary arteries ( fig . histological evaluation of the colonic biopsy specimens revealed cytomegalic cells with densely staining nuclei and intranuclear inclusion bodies in the epithelium ( fig . these epithelial cells were positive for monoclonal anti - cmv antibody on immunohistochemical analysis , indicative of cmv infection ( fig . the patient had no obvious risk factors for thrombosis , and had no family history of coagulopathy . follow - up ct scan of the patient 's chest showed complete resolution of the pulmonary emboli . acute cmv infection in immunocompetent patients is common worldwide , with seroprevalence rates ranging from 40% to 100% , depending on country , socio - economic conditions , and age.1 colitis is the most common manifestation of severe cmv infection.2 patients with cmv colitis are generally asymptomatic ; if symptomatic , they may present with diarrhea , fever , bleeding , abdominal pain , and perforation . 4 vascular events are a very rare manifestation of severe cmv infection in immunocompetent individuals.2 these vascular events include thrombosis of the portal , superior mesenteric , or splenic vein ; cerebral venous thrombosis ; deep vein thrombosis ; and life - threatening pulmonary embolism . 567 vascular complications may be discovered only several weeks after the initial diagnosis of cmv infection . however , abgueguen et al . proposed that venous thrombosis in immunocompetent patients with cmv infection may not be as rare as previously reported.8 although the pathogenesis of cmv - induced vascular events is unknown , several mechanisms have been postulated , including direct damage to endothelial cells , increased levels of hemostatic indicators , induction of smooth muscle proliferation , enhanced platelet - derived growth factor production , and the presence of antiphospholipid antibodies.91011 vascular thrombosis in immunocompetent patients is usually associated with predisposing factors such as heterozygous factor v leiden mutation , presence of antiphospholipid antibodies , heterozygous prothrombin gene mutation , protein c and/or protein s deficiency , or oral contraceptive use.12 in contrast , this patient had no family history of coagulopathy , was not bed - ridden , and had no other risk - factors for thrombosis . it is , therefore , likely that cmv infection was the precipitating factor for pulmonary embolism . in the diagnosis of vascular events caused by cmv infection , doppler ultrasound and ct scanning are the most useful diagnostic modalities.6 treatment of cmv - associated pulmonary embolism includes immediate administration of antiviral agents and anticoagulants . advanced age , male gender , presence of immune - modulating comorbidities , and need for surgical intervention all negatively influence survival.13 although the cost - effectiveness of investigating for acute cmv infection in patients presenting with thrombosis has not yet been established , we believe that serum samples for cmv infection should be obtained and endoscopic examination should be considered in patients with idiopathic thrombosis . in conclusion , although pulmonary embolism caused by acute cmv colitis in immunocompetent patients is extremely rare , it should be considered in patients who present with severe respiratory symptoms . to the best of our knowledge , this is the first report of an immunocompetent patient with acute cmv colitis complicated by vascular thrombosis with pulmonary embolism in which no other obvious underlying predisposing factor for thrombosis was identified .

acute cytomegalovirus ( cmv ) infection occurs commonly in immunocompromised and immunocompetent patients , but is usually asymptomatic in the latter . vascular events associated with acute cmv infection have been described , but are rare . hence , such events are rarely reported in the literature . we report a case of pulmonary embolism secondary to acute cmv colitis in an immunocompetent 78-year - old man . the patient presented with fever and diarrhea . colonic ulcers were diagnosed based on colonoscopy findings , and cmv was the proven etiology on pathological examination . the patient subsequently experienced acute respiratory failure . pulmonary embolism was diagnosed based on the chest radiography and computed tomography findings . a diagnosis of acute cmv colitis complicated by pulmonary embolism was made . the patient was successfully treated with intravenous administration of unfractionated heparin and intravenous ganciclovir .

dyskeratosis congenita ( dc ) is a rare multisystem disorder first described in the early 20 century ( 1906 ) ; it is also known as zinsser - engman - cole syndrome.1 it is characterised by the classic triad of mococutaneous pigmentary disorder , nail dystrophy and oral leukoplakia seen in 89% , 80% and 45% of cases respectively.2 however abnormalities involving the digestive system ( oesophageal stricture , liver cirrhosis ) , ocular ( corneal ulcers , conjunctivitis , blepharitis , lacrimal duct stenosis with epiphoria ) , neurologic ( mental retardation , delayed developmental milestones ) have also been reported,3 and bone marrow failure occurs in about 75% of cases with x - linked form.4 bone marrow failure is the major cause of death , while other causes of mortality are pulmonary fibrosis and malignancy . there is scarcity of information on this disorder among nigerians to the best of our knowledge . therefore , the case of a 9-year - old boy who presented with recurrent febrile illness , repeated blood transfusion with dystrophic nails and hyperpigmentation of the skin in whom a diagnosis of dc was made is reported . a 9-year - old boy presented with complaints of easy fatigability , recurrent nose and gum bleeding with recurrent blood transfusion of 2 months duration ; he had four blood transfusions in the past 2 months . there was history of easy bruisability , but no history of prolonged bleeding following circumcision which he had at the age of 7 years . there was history of generalised darkening of the body , palms and soles with some whitish and dark patches on the tongue noticed around the age of 5 years . he was the 2 child in a monogamous non - consanguineous marriage setting of six children . the mother was a 30-year - old stay - at - home mother while the father was a 35-year - old self - employed trader . the patient had epistaxis from both nostrils ; he was pale with hyperpigmentation of the skin of the neck , chest [ figure 1 ] , arms and palms of the hands [ figure 2 ] ; and soles of the feet with scattered areas of hypopigmentation . he had some white and black plaques [ figure 3 ] on the tongue which were non - scrapable . there were dental caries , gum bleeds [ figure 4 ] , while the other mucosal surfaces of the oral cavity were normal . both eyes had epiphoria [ figure 5 ] , but more on the right eye . the nails of both fingers and toes were cracked , ridged and atrophic [ figures 6 and 7 ] . hyperpigmentation of the skin of the neck , chest with areas of hypopigmentation hyperpigmentation of the palms white and black plaques on the tongue dental caries with gum bleeds epiphoria from both eyes dystrophic finger nails the full blood count showed haemoglobin of 56 g / l white blood cells count of 2.0 10/l ( normal = 4 11 10/l ) , with an absolute neutrophil count of 1.0 10/l , platelet count of 23 10/l ( normal = 150 - 450 10/l ) , clotting profile was normal and bone marrow biopsy revealed a hypocellular marrow [ figure 8 ] ; therefore , he had a pancytopenia . based on the clinical and laboratory findings the diagnosis was dc . he was also referred for ophthalmologic evaluation which revealed lacrimal duct stenosis . despite being on anabolic steroid for 12 weeks the bone marrow failure persisted and due to financial constraints he could not afford haematopoietic cell transplantation ; he is currently on supportive care and is being followed up in the paediatric clinic . dc occurs worldwide with very few cases reported so far.56 although x - linked , autosomal recessive and dominant modes of inheritance have been reported , our case had no family history of same disease and was a product of a non - consanguineous marriage , which makes a sporadic relationship most likely . those with mild manifestations may have minimal physical features with normal bone marrow function , while those with severe manifestation may present with serve multisystemic disorders like revesz syndrome and hoyeraal hreidarsson syndrome7 presenting early in childhood . in classical dc , most of the somatic abnormalities are often absent at birth , but develops with increasing age ; therefore , the diagnosis of dc is often delayed until adulthood , but our patient had all the major clinical features which made the diagnosis easy even in the absence of genetic analysis . our patient had the classic triad of the disease , namely skin hyperpigmentation , nail dystrophy and oral leukoplakia which fulfilled the clinical diagnostic criteria . he also had a severe manifestation of the disease , with early onset of bone marrow failure . bone marrow failure is often delayed until the second decade of life , but our case presented early - at the age of 9-years - involving all the three major blood cell lines ( pancytopenia).5 dermatologic manifestation of dc can be reticular hyperpigmentation or hypopigmentation of the skin , especially affecting the neck and chest . the patient had hyperpigmentation of the skin of the neck , chest , arms and the palms and soles of the feet . white patches may affect the tongue , buccal mucosa or the palate , but the tongue is mostly affected . in our patient , the epiphoria was attributed to lacrimal stenosis , while the dental caries and repeated gum bleeds are due to deficiencies in the neutrophils and platelets respectively . the cause of dc has been linked to mutations in genes related to telomere maintenance ( telomerase ) and to date , eight genes have been implicated.89 mutations in a component of the shelterin complex are involved in autosomal dominant dc . the shelterin complex determines the structure of the telomeric terminus ; it also controls the synthesis of telomeric dna . therefore , without the shelterin complex telomeres are exposed to the dna damage - repair mechanisms , which may result in the ends of chromosome being incorrectly processed by the dna - repair pathways predisposing to fusion of chromosomal ends during replication.7 the fact that pancytopenia did not respond to oral androgen and the non - availability of facilities for haematopoietic stem cell transplant , which is the main stay of treatment,10 represent a big challenge in the management of this patient . moreover , even if the patient had responded to steroids , a long - term anabolic steroid regimen may be complicated by side effects considering the age of this patient . the case of a 9-year - old boy , who had all the major clinical features - hypo-/hyperpigmentation of the skin , nail dystrophy , leukoplakia and early bone marrow failure- which before now had not been documented in nigeria , is reported .

dyskeratosis congenita is a rare hereditary disease . it mainly affects males and manifest between 5 years and 12 years . its classic manifestation consists of skin pigmentary changes , nail dystrophy , oral leukoplakia , bone marrow failure and predisposition to malignany . we report the case of a 9-year - old boy who presented with hyperpigmentation of the skin , palms and soles , leukoplakia of the tongue , dystrophy of the nails , epiphoria and recurrent epistaxis with gum bleeding . full blood count showed pancytopenia and bone marrow biopsy showed hypocellular marrow with no abnormal cells . he was transfused with pack red blood cells , platelets concentrate and was commenced on co - trimoxazole prophylaxis and anabolic steroid . he is currently on follow - up in the paediatric clinic .

a 61-year - old woman was referred to department of thoracic and cardiovascular surgery with thrill on the right wrist . two years earlier , she had visited the emergency department of smg - snu boramae hospital with a recently aggravated chest pain . she underwent emergency coronary angiography ( cag ) via the right femoral artery , and percutaneous coronary intervention for the proximal right coronary artery , which was successfully completed . one year after the intervention , she underwent diagnostic cag via the right radial artery , and was discharged one day after the procedure . about 11 months after the day of the diagnostic cag , she complained of palpable thrill on the right wrist at the outpatient clinic . she also presented dilated superficial veins on the right forearm , which was very close to the previous puncture site for the diagnostic coronary angiography ( fig . 1 ) . suspecting radial arteriovenous fistula ( avf ) formation , local compressive dressing and elastic bands were applied on the right wrist , with the expectation of spontaneous closure , but there was no effect . a vascular ultrasound scan revealed a tract between the right radial artery and adjacent subcutaneous vein , which suggested radial arteriovenous fistula ( fig . 2 ) . computed tomography ( ct ) angiography of the upper extremity was performed , and it showed early visualization of dilated deep and superficial veins of the right forearm in the arterial phase . in addition , the right cephalic , brachial and axillary veins were visualized by contrast media in the arterial phase ( fig . the fistula tract between the radial artery and vein was ligated , and the arterial side fistula opening was closed primarily ( fig . transfemoral access is a widespread method for coronary diagnostic and interventional procedures , and it is rarely associated with access site vascular complications . the possible complications are major hematoma , avf , pseudoaneurysm , and arterial dissection , and surgical correction for these complications is very commonly recommended . transradial access for coronary procedures had become an increasingly popular technique since it was first introduced by campeau in 1989 because it significantly reduces access site vascular complications compared to transfemoral access . in particular , avfs after transradial access are an extremely rare complication ( 0.08% ) because of the absence of large veins near the radial artery . reported that there were several factors predisposing patients to iatrogenic femoral avfs : age over 60 , female gender , arterial hypertension , prolonged coumadin therapy , and high heparin dosage during the procedure . most of the predisposing factors were associated with delayed healing of the access sites of the punctured arteries . in this case , there was atheromatous plaque in the fistulous portion of the right radial artery , which might have resulted in a poor healing process around the puncture site of the radial artery , and this , in turn , might have contributed to the formation of radial avf . the clinical presentation of iatrogenic avf is various : a pulsatile mass , thrill , pain , edema , and dilated and varicose veins can occur . however , radial avf is usually asymptomatic and manifests as a thrill or bruit over the wrist . the iatrogenic radial avfs usually have smaller shunt volumes than other large fistulae , such as hemodialysis fistula , so that significant hemodynamic change is not frequent . several diagnostic tools , such as duplex ultrasonography , computed tomography , magnetic resonance imaging , and conventional angiography , can be used if there is clinical suspicion of iatrogenic avf . duplex ultrasonography has become the routine screening test for patients with suspected avfs , but the use of a ct scan has gradually increased because ct is minimally invasive , rapid , and operator independent . in addition , 3-dimensional ct angiography shows the exact spatial relationship between the radial artery , superficial veins , and adjacent structures around the avf , which was the reason we performed additional ct angiography after the ultrasonographic diagnosis of the avf in this case . three different therapeutic strategies have been reported so far for treating iatrogenic radial avf : conservative management , endovascular treatment , and surgical treatment . about one - third of iatrogenic avfs close spontaneously ; for this reason , conservative management is usually considered to be the first line of treatment . some have reported successful implantation of stents in radial avfs , and surgical repair also has frequently been recommended . in this case , we had applied local compression and elastic bands for a week , anticipating spontaneous closure of the radial avf . however , there was no effect ; thus , we performed surgical repair of avf for the patient . we report an extremely rare case of delayed radial arteriovenous fistula formation , one year after cag , using transradial access , which was treated successfully with surgical repair .

transradial access is a widely accepted method for percutaneous coronary diagnostic and interventional procedures , and it has dramatically reduced access site vascular complications compared to transfemoral access . arteriovenous fistula formation at the access site is an especially rare complication in transradial access . we report an extremely rare case of delayed radial arteriovenous fistula that developed one year after transradial coronary angiography , which was successfully treated by surgical repair .

brucellosis is a systemic infection that may affect any organ or system of the human body . usually , a slight increase in the liver function tests and mild hepatosplenomegaly occur , and sometimes , acute hepatitis develops , but hepatic abscess is a rare manifestation of that disease 1 , 2 , 3 . a 33yearold woman was admitted to our university hospital with the complaints of temperature up to 40c , headache , nausea , and weakness for 2 weeks . she had a history of raising livestock and lived in rural area . on physical examination the laboratory data were as follows : total leukocyte count , 5600/mm ( 400010,000/mm ) ; differential leukocyte count : neutrophils , 54% ; lymphocytes , 36% ; monocytes , 8% ; basophils , 0.3% ; eosinophils , 1.7% ; platelets , 285,000/mm ( normal range : 150,000450,000/mm ) ; hemoglobin , 12 g / dl ; hematocrit , 36.6% ; serum alanine transferase ( alt ) , 242 u / l ( rr 035 iu / l ) ; serum aspartate transferase ( ast ) , 162 u / l ( rr 032 iu / l ) ; serum glutamyl transpeptidase ( gtp ) , 60 u / l ( rr 040 iu / l ) ; alkaline phosphatase ( alp ) , 95 iu / ml ( rr 35114 iu / l ) ; total bilirubin , 0.4 mg / dl ( rr 01.2 mg / dl ) ; sedimentation rate , 17 mm / h ; and creactive protein , 11.3 mg / l ( rr 05 mg viral hepatitis markers ( the hbsag , antihbc igm , antihav igm , and antihcv tests ) were negative . abdominal ultrasonography showed multiple small echogenic foci are more prominent in the right lobe of the liver ( fig . 1).with these findings , the patient was diagnosed as having hepatic microabscesses due to brucellosis . doxycycline ( 2 100 mg / day p.o . ) and rifampicin ( 1 600 mg / day p.o . ) the patient received this treatment for 3 months . on the fiftyninth day of the treatment , the levels of serum alanine transferase level and serum aspartate transferase decreased to the reference range . brucellosis is a zoonosis that has been virtually eliminated from most developed countries , but it is still endemic in many regions of the world including mediterranean areas , in parts of south and central america , and east and western africa . the disease is transmitted to man mainly after consumption of contaminated unpasteurized milk and dairy products and less often after direct contact with infected animals 1 . hepatic involvement in brucellosis covers a wide spectrum , ranging from mild elevation of aminotransferases to hepatitis including granulomatous forms and to liver abscesses . increases in aminotransferases are noted in onefourth to onethird of brucellosis cases and are more frequent in the acute stages . hepatic involvement in brucellosis has been reported in the literature in around 23% of the cases . although b. abortus tends to establish a granulomatous form of hepatitis , b. melitensis may cause both diffuse and granulomatous lesions in the liver 1 , 2 , 3 , 4 . an abscess caused by brucella spp usually represents the chronic form of disease , but it can occur in acute or subacute brucellosis . routine laboratory findings in brucellosis are not usually diagnostic that may include leukopenia , anemia , thrombocytopenia , pancytopenia , and mildtomoderate elevation of liver function tests 2 , 5 , 6 . ultrasonography most commonly shows a single , hypoechoic lesion with 1 centrally located calcium deposits . computed tomography findings most commonly depict a hypodense area , and often one or more saccular , loculated , heterogeneous mass , and one or more calcifications 5 , 6 . the diagnosis of brucellosis can be established according to the isolation of brucella spp . in blood , bone marrow or any other body fluid or tissue sample , or the presence of a compatible clinical picture with the demonstration of specific antibodies at significant titers or seroconversion . significant titers are considered to be a standard agglutination test ( sat ) result 1/160 or a coombs antibrucella or immunocapture agglutination test result 1/320 1 , 6 . the best regimen for the treatment of localized lesions has not been clearly defined . the duration of treatment varies depending on the individual case and the response to treatment 1 . small , multifocal abscesses which can be detected in the acute forms of the disease respond very well to medical treatment . other types of abscesses with an indolent course have a much worse prognosis , which considered to be a true focal complications of the disease . however , no clear distinction is made of these groups 7 . in this case , the short duration of symptoms and the high titer of brucella agglutination test suggest that this case was acute . it was identified multiple millimetric foci were more prominent in the right lobe of the liver parenchyma to be significant in terms of microabscesses . our patient was successfully treated with a combination of doxycycline and rifampicin for 3 months . the diagnosis of brucellosis in our case was confirmed with clinical findings , livestock farming history , positive serological tests , and complete response to medical treatment . in conclusion , brucellosis is a systemic infectious disease and it is still an important public health problem in endemic areas of the world including turkey that can cause serious complications and significant morbidity . clinicians should be considered in the differential diagnosis of this unusual complication of brucellosis for those who live in or have visited endemic areas . ie , rka , oo , pod , and ss : performed the analysis of case data . all authors : contributed toward data analysis , drafting and critically revising the manuscript , and agree to be accountable for all aspects of the manuscript .

key clinical messagehepatic abscess due to brucella species is an extremely rare complication especially in acute illness . here , we report a case of hepatic microabscesses probably caused by brucella in a 33yearold woman with acute infection who was successfully treated with a combination of doxycycline and rifampicin for 3 months .

a 7month old baby girl was brought to our hospital with a 2 months history of an ill defined mass involving entire abdomen . contrast enhanced computed tomography ( cect ) of the abdomen and pelvis revealed a small left kidney with a dilated renal pelvis that continued as a hugely dilated and tortuous ureter ( figure 1 ) . on a diethylene triamine pentaacetic acid ( dtpa ) micturating cystourethrogram ( mcu ) showed no reflux , with the bladder pushed laterally , probably due to the mass effect of the dilated ureter ( figure 2 ) . on cystoscopy , hemitrigone was noticed , with absent left ureteric orifice . a diagnosis of left ectopic ureter with congenital giant hydroureter and poorly functioning left kidney was made and open nephroureterectomy was done ( figure 3 ) . ct scans with a small left kidney , an extremely dilated left ureter and a displaced bladder . this case has been presented to highlight the extent to which a ureter can dilate . in case of congenital giant hydroureter the ureter is dilated out of proportion to the kidney , which is usually small and atrophic . very few such cases have been reported in literature [ 2 , 3 , 4 ] and surgeons should be aware of this entity as one of the causes of abdominal mass in an infant .

herein we present a case of a 7month old baby girl , with left single system ectopic ureter , in whom the ureter was dilated to such an extent that it presented as an abdominal mass . diagnosis was established by means of computed tomography . work up was completed with renal scan and micturating cystourethrography . the patient was operated and left nephorureterectomy was performed . hydroureter has to be considered in differential diagnosis of abdominal mass in an infant .

levetiracetam ( lev ) is used to treat generalized and partial seizures of various etiologies . rhabdomyolysis postconvulsive seizure may limit kidney excretion of muscle breakdown products like creatine phosphokinase ( cpk ) . in this case report , a 19-year - old right - handed male presented following a complex partial seizure with secondary generalization in the setting of stress and sleep deprivation . a second convulsive event lasting 2 min occurred in the emergency room . the patient was given 2 mg iv lorazepam , 2000 mg iv lev , and 1200 mg oxcarbazepine ( oxc ) , and no further seizures occurred . epilepsy etiology was due to a right frontal arteriovenous malformation ( avm ) located in the left leg primary motor cortex ( fig . 1 ) . the avm had previously been treated with gamma knife radiosurgery ( 20 gy at 50% isodose to encompass a volume of 1.2 ml ) . outpatient anticonvulsant medications for this patient prior to the present hospitalization included oxc 600 mg in the morning and 900 mg in the afternoon or evening and lorazepam 0.5 mg prn for breakthrough seizures . of note , this patient is an avid weight lifter and very muscular ( fig . 2 ) . although an avid weight lifter , the patient denied using supplemental agents for muscle bulking such as anabolic steroids or protein powders . they occurred rarely in the daytime , and if they did , a warning of lightheadedness , dizziness , and a lost sense of balance would progress to bilateral muscle cramping , tonic this admission was complicated by a rapid rise in cpk and mild renal compromise , a new finding for the patient ( fig . maximum creatinine was 2.17 mg / dl and had normalized prior to cpk maximum . the patient 's cpk levels were slow to resolve despite appropriate iv hydration . during the entire period of cpk elevation , the patient 's urine remained clear / yellow ; trace hematuria was noted only on day two . his day nine serum myoglobin was 228 ng / ml ( normal range : 1092 ng / ml ) . an initial serum myoglobin level was not checked . with a marked drop of cpk on day 5 ( cpk : 2736 ) however , blood work postdischarge showed cpk levels again in the 29,000 range , suggesting that the day five cpk measurement was a lab error . levetiracetam was discontinued on day eight ; day nine blood work showed a halving of cpk levels from 29,136 to 14,918 and a normal level of creatinine . creatine phosphokinase levels continued to decline rapidly after lev discontinuation , with normalization occurring nearly a month later after resumption of a normal exercise regimen . two convulsive seizures and subsequent lev therapy led to a dramatic increase in cpk in this muscular male . although this could indicate a delayed rhabdomyolysis , the classic findings including marked elevations in myoglobin / myoglobinuria , decreased urine output , and muscle pain were not evident . additionally , following muscular injury , rhabdomyolysis - related cpk elevations typically occur maximally by day three and decline by day five . the cpk elevation in this patient was both delayed and persisted beyond the typical time frame of a case of seizure - induced rhabdomyolysis , suggesting ongoing issues of muscle breakdown despite cessation of seizure activities . creatine phosphokinase release occurs with insults to the sarcolemma , most notably failure of atp production or use . however , lev was the only new drug given , and its use even at a low maintenance dose and discontinuation closely mirror the trends in cpk levels in this patient . although we suspect that lev may have contributed to cpk elevations in this heavily muscled patient , those suspicions remain unproven . a retrial of lev with cpk and renal function checks , a muscle biopsy , and a repeat renal ultrasound are not planned , given that the patient 's clinical issues have resolved . this case revealed a marked and dangerous increase in cpk levels corresponding with modest lev use . although this scenario could indicate a delayed rhabdomyolysis , the rapid decline in cpk with lev discontinuation suggests that , in this patient , lev may have had effects on kidney filtration and/or muscle breakdown .

a 19-year - old muscular male with a history of epilepsy presented following two convulsive events . levetiracetam ( lev ) was given as an additional therapy , resulting in a marked boost in creatine phosphokinase ( cpk ) that could not easily be explained by renal dysfunction or rhabdomyolysis alone . levetiracetam discontinuation caused cpk levels to quickly normalize and should be considered in patients with persisting cpk elevations postconvulsive seizure .

corynebacterium urealyticum , a gram - positive bacillus , has been identified as the most frequent causative pathogen . urinary stasis is highly likely to contribute to stone formation , because large - scale spontaneous precipitation of crystals occurs when a critical concentration ( e.g. , supersaturation ) is exceeded . failed treatment of encrusted pyelitis in a transplanted kidney can cause graft failure or nephrectomy . to overcome urinary stasis with encrusted pyelitis in a transplanted kidney , we report surgical treatment by ileo - pelvic anastomosis without nephrectomy . in addition , in this case , augmentation cystoplasty was performed to strengthen the atrophied bladder following tuberculosis . a 51-year - old female patient received a kidney from her sister after a double nephrectomy owing to renal tuberculosis 12 years previously . at that time , the transplanted ureter was anastomosed to the ileum in the left lower abdomen with an ileal conduit on the opposite side . the patient experienced no problems for 12 years . at a routine checkup , she complained of abdominal pain , gross hematuria , and increased debris in the ileal conduit . her serum creatinine was elevated to 1.7 mg / dl ( normal , 0.6 to 1.1 mg / dl ) , and her transplanted kidney developed hydronephrosis with calcified debris observed by sonography ( fig . neither hydronephrosis nor calcified debris had been observed 6 months before in a routine outpatient checkup . abdominopelvic computed tomography revealed hydronephrosis with encrusted pyelitis and ureteritis in the transplanted kidney ( fig . a cystogram showed that the bladder was not refluxed but had a small capacity ( 50 ml ) . after 3 weeks , we operated to anastomose the transplanted renal pelvis to one end of the ileal conduit , performing augmentation cystoplasty with new ileum and to anastomosis the other end of the remnant ileum with the augmented bladder . augmentation cystoplasty is a reconstructive technique for creating a compliant , large - capacity urinary storage unit to protect the upper urinary tract . the operation was performed via a midline incision . because it was difficult to approach the major calices of the transplanted kidney in the left pelvic rim , we anastomosed one end of the ileal conduit to the pelvis of the transplanted kidney in an end - to - end fashion . the ileal conduit was lengthened by 30 cm to extend from the pelvis to the augmented bladder chimney . after exposure of the urinary bladder , a transverse wide v - shaped cystostomy incision was created , with the apex approaching the anterior bladder neck and the base extending posteriorly past the midcoronal plane of the bladder dome . a 20 cm segment of new ileum at least 15 cm proximal to the ileocecal junction was used for ileocystoplasty . for the small bowel , a v - shaped plate was created by a side - to - side anastomosis with 2 - 0 vicryl . a circumferential , continuous , full - thickness , single layer anastomosis of the bowel to the bladder was started posteriorly by using 2 - 0 braided absorbable sutures . the other end of the ileal conduit was anastomosed through small separate ileotomies ( 1.5 cm length ) in the limb of the augmentation cystoplasty . a follow - up cystogram showed increased bladder capacity to approximately 300 ml with well - preserved end - to - end anastomosis of the ileo - pelvic portion with the other end of one chimney of augmentation cystoplasty with the elongated ileal conduit ( figs . 3 , 4 ) . the most common causative agent in the formation of infection stones is a urease - positive urinary tract infection . less invasive methods are preferable to preserve the connection between the renal pelvis and the upper ureter . ileo - pelvic anastomosis must be adjusted for patients with infection stones in the upper urinary tract after renal transplantation . an alternative way to prevent these infection stones is to ensure a wider pathway through a ureter that has been narrowed in places by these stones . according to a report on the incidence of urolithiasis in cystectomy patients , 5 of 78 patients who had undergone diversion with an intestinal conduit developed urolithiasis , all in the upper tract . in contrast , 4 of 78 patients who had received a diversion had a renal stone at the time of presentation . refluxing urine may contribute to an increased risk of stone formation after urinary diversion , whereas pouch stasis may contribute to stone formation in the diversion group . a case of failed treatment of encrustation in a kidney transplant recipient was reported and graft removal was performed after 6 months of failed management owing to intractable febrile urinary tract infections that became life threatening . they excised renal parenchyma from the caudal portion of the inferior calyx , which was then anastomosed to the ileostomy . in their case , bladder capacity was within the normal limit ; thus , augmentation cystoplasty was not indicated . in conclusion , if urinary tract infection is controlled , we suggest that ileo - pelvic anastomosis combined with augmentation cystoplasty is an alternative for the treatment of encrusted pyelitis with atrophied bladder . in our patient , we performed ileo - pelvic anastomosis to prevent urinary stasis by elongating the ileal conduit . in addition , the augmentation cystoplasty technique is useful for increasing bladder capacity that has been diminished by renal tuberculosis ( fig .

infection stones are more likely to form after urinary diversion as the result of urinary stasis . to prevent urinary stasis due to encrusted pyelitis in a transplanted kidney , we describe an alternative a surgical treatment : ileo - pelvic anastomosis . in our patient with a transplanted kidney , the ileal conduit had previously been anastomosed end - to - side owing to renal tuberculosis with an atrophied bladder ; the transplanted ureter was anastomosed to the ileum in the left lower abdomen with an ileal conduit on the opposite side . routine check - up revealed hydronephrosis with infected pyelitis and ureteritis in the transplanted kidney . we performed ileo - pelvic end - to - end anastomosis to prevent urinary stasis by lengthening the ileal conduit and performed augmentation cystoplasty to support the atrophied bladder following tuberculosis . we suggest that this approach may be useful in similar cases .

a 65-year - old male patient presented to us with diminution of vision of one - day duration in the right eye . the best - corrected - visual - acuity ( bcva ) in the right eye was 10/200 . fundus of the eye showed an smh along with a large pigmentary epithelial detachment ( ped ) [ fig . leakage of the dye on indocyanine green angiography ( icg ) and variable reflectivity on optical coherence tomography ( oct ) suggested a subfoveal cnvm [ fig . 2 ] . red free image of right eye showing submacular hemorrhage ( blue arrow ) along with pigmentary epithelial detachment ( red arrow ) optical coherence tomography showing variable reflectivity of the retinal pigmentary epithelium ( blue arrow ) suggestive of choroidal neovascular membrane with subretinal blood and large pigmentary epithelial detachment ( red arrow ) a 62-year - old male presented with sudden diminution of vision in the right eye since one week . oct showed variable reflectivity of the retinal pigment epithelium ( rpe ) along with an increase in the retinal thickness suggestive of subfoveal cnvm with macular edema . a 35-year - old female presented to us with diminution of vision in the left eye since seven days . patient had undergone refractive surgery for myopia 10 years back . left eye fundus showed a subretinal hemorrhage and oct showed hyper - reflectivity in the rpe suggestive of cnvm . a 65-year - old female came to us with complaint of diminution of vision in the left eye since one week . . left eye fundus showed smh along with chorio - retinal atrophy at the edge . ffa showed blocked fluorescence in the area of hemorrhage with late hyperfluorescence of the chorioretinal atrophy . all patients were given an intravitreal injection of 1.25 mg or 0.05 ml bevacizumab ( one vial of bevacizumab contains 100 mg in 4 ml ) along with pneumatic displacement of the smh by 0.4 - 0.5 ml of sf6 [ micro sf , micromed s.r.l , italy ] . intravitreal gas was injected using 30 g needles ( pricon , iscon surgicals ltd . , a 65-year - old male patient presented to us with diminution of vision of one - day duration in the right eye . the best - corrected - visual - acuity ( bcva ) in the right eye was 10/200 . fundus of the eye showed an smh along with a large pigmentary epithelial detachment ( ped ) [ fig . leakage of the dye on indocyanine green angiography ( icg ) and variable reflectivity on optical coherence tomography ( oct ) suggested a subfoveal cnvm [ fig . 2 ] . red free image of right eye showing submacular hemorrhage ( blue arrow ) along with pigmentary epithelial detachment ( red arrow ) optical coherence tomography showing variable reflectivity of the retinal pigmentary epithelium ( blue arrow ) suggestive of choroidal neovascular membrane with subretinal blood and large pigmentary epithelial detachment ( red arrow ) a 62-year - old male presented with sudden diminution of vision in the right eye since one week . oct showed variable reflectivity of the retinal pigment epithelium ( rpe ) along with an increase in the retinal thickness suggestive of subfoveal cnvm with macular edema . a 35-year - old female presented to us with diminution of vision in the left eye since seven days . patient had undergone refractive surgery for myopia 10 years back . left eye fundus showed a subretinal hemorrhage and oct showed hyper - reflectivity in the rpe suggestive of cnvm . a 65-year - old female came to us with complaint of diminution of vision in the left eye since one week . . left eye fundus showed smh along with chorio - retinal atrophy at the edge . ffa showed blocked fluorescence in the area of hemorrhage with late hyperfluorescence of the chorioretinal atrophy . all patients were given an intravitreal injection of 1.25 mg or 0.05 ml bevacizumab ( one vial of bevacizumab contains 100 mg in 4 ml ) along with pneumatic displacement of the smh by 0.4 - 0.5 ml of sf6 [ micro sf , micromed s.r.l , italy ] . intravitreal gas was injected using 30 g needles ( pricon , iscon surgicals ltd . , treatment , if delayed , leads to poor recovery due to the delay in diagnosis and treatment of the primary pathology as well as due to the harmful effect of persistent subretinal blood on photoreceptors . treatment options have included cnvm and hemorrhage removal with forceps , pneumatic displacement with/ without intravitreal tissue plasminogen activator ( tpa ) , injection of subretinal tpa with displacement by perfluorocarbon liquid , injection of subretinal tpa followed by hemorrhage evacuation , vitrectomy with intravitreal/ subretinal injection of tpa with an intraocular gas bubble to help displace the hemorrhage inferiorly . previously , pneumatic displacement was the treatment of choice in patients of smh in cnvm , at our center . subsequent management of the underlying pathology used to be done in a second sitting . with the widespread use of intravitreal bevacizumab in treatment of wet amd , we decided to combine the two approaches for a faster visual recovery . intravitreal injection of sf6 displaces the hemorrhage from the macular area , allowing for increase in visual acuity as well as better visualization of the primary pathology . india being a developing country with limited availability and high cost of tpa , bevacizumab seemed a good alternative . this resulted in a better definitive delineation of the primary pathology on subsequent ffa and oct [ figs . 3 and 4 ] . red free image of the right eye showing clearing of submacular hemorrhage after intra - vitreal bevacizumab and sf6 injection ( red arrow ) optical coherence tomography of the same patient showing normal reflectivity of the retinal pigmentary epithelium after the procedure with normal foveal contour ( red arrow ) gopalakrishan et al . , have reported a series of 20 cases of smh who were treated with pneumatic displacement out of which five had amd . in these patients in contrast , all the four cases in our series had good recovery of visual acuity . this is an observation with very few patients but seems a promising technique in patients of amd with smh . since earlier studies have had poor results with the other techniques available , more patients need to undergo this treatment for a better analysis of outcome .

choroidal neovascular membrane ( cnvm ) is one of the most common causes of submacular hemorrhage ( smh ) . conventional treatment involves management of the smh with pneumatic displacement with or without tissue plasminogen activator ( tpa ) followed by intravitreal injection of bevacizumab in a second sitting . we decided to assess the efficacy of treating smh secondary to cnvm with pneumatic displacement using sulphur hexafluoride ( sf6 ) gas and intravitreal bevacizumab . four patients with smh secondary to cnvm were included in this study . intravitreal bevacizumab , 0.05 ml , along with 0.5 ml of sf6 was injected through the pars plana into the vitreous cavity . postoperative best corrected visual acuity improved in all eyes with complete or partial displacement of smh out of the foveal area .

klippel - trenaunay syndrome ( kts ) is characterized by the triad of port wine stain , venous and lymphatic malformation , and soft tissue hypertrophy of the affected extremity . depending on the type of vessel involved and its flow characteristics kts is classified as a slow flow complex combined capillary venous or capillary venous lymphatic malformation . the localized variants are solitary angiokeratoma , fordyce 's angiokeratoma , angiokeratoma of mibelli and angiokeratoma circumscriptum naeviforme ( acn ) . the systemic form , angiokeratoma corporis diffusum , is usually associated with an inborn error of metabolism . among all the types , acn is the rarer and the only congenital variant of angiokeratoma . the lesions of acn are bluish red , well defined and are classically seen on the lower extremity in a unilateral distribution . a 4-year - old male child presented with complaints of a linear eruption on his right leg since birth . his parents gave history of pain and several episodes of bleeding from the lesion after trauma . , there were multiple hyperkeratotic discrete and closely aggregated papules and plaques of varying size on an erythematous base arranged linearly along the lateral aspect of the right thigh extending up to the knee joint [ figures 1 and 2 ] . right lower limb arteriovenous ( av ) doppler revealed a hypoplastic right lower limb deep venous system with compensatory dilatation of the superficial venous system . the superficial femoral vein , popliteal vein , anterior tibial and posterior tibial vein on right side were hypoplastic along with thickening of subcutaneous tissue and associated dilatation and malformation of the superficial venous system [ figures 5 and 6 ] . histological examination revealed numerous dilated thin walled , congested capillaries mainly in the papillary dermis and very few in reticular dermis . overlying epidermis showed compact hyperkeratosis , irregular acanthosis with elongated rete ridges consistent with diagnosis of angiokeratoma [ figures 7 and 8 ] . on the basis of clinical , histological and radiological findings dilated superficial venous system verrucous plaque with soft tissue hypertrophy prominent superficial veins on dorsum of foot plain radiograph showing soft tissue hypertrophy magnetic resonance imaging angiography showing dilated superficial veins on the right side dilated superficial venous system hyperkeratotic epidermis with congested capillaries in papillary dermis with normal deep dermis and subcutaneous tissue hyperkeratotic epidermis with dilated congested capillaries in papillary dermis in 1900 , two french physicians maurice klippel and paul trenaunay described two patients with hemangiomatous lesions of the skin with associated bone and soft tissue hypertrophy and coined the term naevus variqueux osteohypertrophique . in 1907 , parkes weber reported the association of kts with av fistula and called it hemangiectatic hypertrophy . in 1965 , lindenauer proposed that the syndrome originally described by klippel and trenaunay without av malformation be considered as a specific entity the kts and the one associated with av fistula be designated as parkes weber syndrome . in a study of 252 patients with kts at the mayo clinic , 63% had all three features of kts . port wine stains were found in 98% of patients , venous malformations in 72% and limb hypertrophy in 67% patients . the port wine stain is apparent at birth and usually involves the affected limb , often stopping at the midline with a sharp linear border . the nevus may involve the whole of one side of the body and may sometimes be present on the contralateral limb . varicose veins may be obvious at birth , but frequently become evident after walking starts . the venous abnormalities of the deep venous system that occur in kts include aneurysmal dilatation , duplication , aplasia , and hypoplasia . patients with at least two of the three cardinal features have been classified as having an incomplete form of kts . it is the only congenital variant and is more common in women and is usually unassociated with systemic disease . the histopathological finding is same irrespective of the type of angiokeratoma and consist of numerous thin walled congested capillaries mainly in the papillary dermis underlying an epidermis that shows variable degree of acanthosis with elongation of rete ridges and hyperkeratosis . unusual variants of acn described in literature are along the lines of blaschko , and a systematized band like pattern . verrucous hemangioma clinically resembles angiokeratoma but they can be differentiated histopathologically as the former involves the dermis and the subcutaneous fat and the latter involves only the dermis . our case showed typical clinical features of acn , which was confirmed by histopathologic examination . associated soft tissue hypertrophy and deep venous malformation on mri are classical features of kts . there are very few case reports of association of kts with acn in world literature . schimpf and wehberg in their study have reported three cases of acn on the lower limb associated with soft tissue hypertrophy . bone hypertrophy was present in two cases , varicose veins in one case and only one case had associated port wine stain on the back . the case reported by odeh had acn of the right leg associated with soft tissue hypertrophy and bone hypoplasia of the right half of pelvis . they preferred the term hemangiectatic hypertrophy as there was no associated bony and venous abnormalities of kts . to the best of our knowledge , this is the first case report of this rare association from india .

klippel - trenaunay syndrome ( kts ) is a cutaneous capillary malformation on a limb in association with soft tissue swelling with or without bony hypertrophy and atypical varicosity . the capillary malformation associated with kts is port wine stain . angiokeratoma circumscriptum naeviforme ( acn ) is a congenital variant of angiokeratoma commonly present on the lower limb as a hyperkeratotic plaque . acn is rarely associated with kts . we report a case of acn with soft tissue hypertrophy and deep venous malformation ( possibly a variant of klippel - trenaunay ) in a 4-year - old male child .

traumatic gallbladder rupture occurs rarely and the incidence is reported to amount to approximately 2% of all blunt abdominal injuries . the main reason for the low incidence is that the gallbladder is protected by the surrounding organs , including the liver , intestines , omentum , and ribs . preoperative diagnosis of gallbladder rupture is generally difficult because no specific symptoms are observed and other concomitant organ injuries are common . gallbladder rupture is usually diagnosed at laparotomy because emergency operation is necessary due to other organ injuries . however , even in the isolated gallbladder rupture which does not need an emergency operation , prompt diagnosis of gallbladder rupture is quite challenging due to the lack of specific symptoms . it is very difficult to detect a defect in the gallbladder wall even when using imaging such as ultrasonography , computed tomography ( ct ) , and magnetic resonance imaging ( mri ) . however , such images can detect the accumulation of intraperitoneal fluid including hemorrhage and bile . if accumulated fluids contain bile , endoscopic cholangiography is useful not only to diagnose the gallbladder injury but also to determine the therapeutic strategy . we present the details of a patient who underwent laparoscopic cholecystectomy after an exact preoperative diagnosis of traumatic gallbladder rupture . a 43-year - old man fell off a tractor and was subsequently run over by the tractor . on admission to our emergency department , his vital signs were stable and he was alert . complete blood count revealed a white blood cell count of 21,800/mm and a hemoglobin level of 14.0 g / dl . enhanced abdominal ct suggested a mild liver laceration in hepatic segment 5 and a small amount of hemorrhagic ascites around the liver ( fig . the tentative diagnosis was liver injury with edward 's classification grade i , and conservative treatment was initiated . the day after admission , the right upper quadrant pain and ascites increased and a muscular defense sign appeared . percutaneous drainage of the ascites was performed , and the aspirated fluid was bloody and almost pure bile . although his abdominal pain improved after continuous drainage , the amount of biliary discharge reached 800 ml / day . mri and ultrasonography revealed little about the source of the bile leakage ; therefore , on the 10th day after abdominal blunt injury , endoscopic retrograde cholangiography was performed , and a diagnosis of gallbladder rupture was made ( fig . 2 ) . laparoscopic cholecystectomy was subsequently performed and revealed biliary ascites accumulated in the upper right abdomen with the gallbladder covered by edematous greater omentum . there were no injuries in other abdominal organs and no obvious injury in segment 5 of the liver . after isolating the gallbladder , a perforation was seen in the gallbladder body , as suggested by endoscopic retrograde cholangiography ( fig . the perforation in the gallbladder wall was sutured closed , and then standard cholecystectomy was performed . blunt traumatic gallbladder injury is rare , and the most common causes are accidents associated with motor vehicles , falls , blows , kicks , or industrial work . blunt gallbladder injury is classified into three types : contusion , avulsion , and laceration ( rupture or perforation ) , and lacerations are the most common after blunt injury . the gallbladder injury in our case was categorized as a laceration with an exact preoperative diagnosis . ct scan is the most useful examination when diagnosing abdominal injuries , and avulsion gallbladder injuries can easily be diagnosed by ct because the gallbladder is torn from the liver and hemorrhage is obvious . however , no specific signs are revealed on ct for laceration gallbladder injuries . despite the substantial leakage of bile into the peritoneal cavity in our case , we could not determine whether it was caused by a gallbladder injury or liver laceration , and further examinations and treatments were required because of the persistent high - volume bile leakage . as magnetic resonance cholangiopancreatography revealed little about the origin of the bile leakage , endoscopic retrograde cholangiopancreatography was planned . because gallbladder rupture is often associated with multiple organ injuries leading to peritonitis , laparotomy is recommended as soon as possible after the diagnosis . however , ruptured gallbladder alone has no associated hemodynamic disorders after successful abdominal drainage ; therefore , emergent laparotomy is not required , and patients are good candidates for laparoscopic surgery . also , the final diagnosis of gallbladder rupture can be confirmed laparoscopically and simultaneous minimally invasive treatment can then be performed . in conclusion , management of gallbladder injuries varies with the presence of other concomitant organ injuries and the degree and type of gallbladder injury . in cases with gallbladder rupture simply diagnosed by direct cholangiography ,

abstractgallbladder rupture due to blunt abdominal injury is rare . there are few reports of traumatic gallbladder injury , and it is commonly associated with other concomitant visceral injuries . therefore , it is difficult to diagnose traumatic gallbladder rupture preoperatively when it is caused by blunt abdominal injury . we report a patient who underwent laparoscopic cholecystectomy after an exact preoperative diagnosis of traumatic gallbladder rupture . a 43-year - old man was admitted to our hospital due to blunt abdominal trauma . the day after admission , abdominal pain and ascites increased and a muscular defense sign appeared . percutaneous drainage of the ascites was performed , and the aspirated fluid was bloody and almost pure bile . he was diagnosed with gallbladder rupture by the cholangiography using the endoscopic retrograde cholangiopancreatography technique . laparoscopic cholecystectomy was performed safely , and he promptly recovered . if accumulated fluids contain bile , endoscopic cholangiography is useful not only to diagnose gallbladder injury but also to determine the therapeutic strategy .

we report the case of a 19-year - old female football spectator who suffered a duodenal rupture as a result of being hit in the abdomen by a football . a 19-year - old girl presented to the emergency department at 1 am after having a football kicked from a distance of 510 m into her abdomen 7 h previously whilst watching a game . her observations were stable ; however , on examination , her abdomen was diffusely tender and there was evidence of generalized peritonism . she had a white cell count of 18.8 10/l , but the rest of her blood tests , including amylase , was normal . a computed tomographic ( ct ) scan of her abdomen and pelvis was arranged , which showed abnormal areas of low attenuation and multiple pockets of air in the right flank , surrounding the right kidney and in the right sub - hepatic space , consistent with a perforation of either the ascending colon or the duodenum ( fig . 1 ) . a laparotomy was performed and a 0.5 cm perforation was seen in the antero - lateral border of the duodenum at the junction of the first and second parts of the duodenum following kocherization . figure 1:ct of abdomen showing free air in right para - renal space ( white arrow ) . ct of abdomen showing free air in right para - renal space ( white arrow ) . the duodenum is a c-shaped organ primarily situated in the retroperitoneum and is anatomically divided into four sections ( d1d4 ) . it is vulnerable to damage by shearing or compression forces , as d1 and d4 are relatively mobile in comparison with d2 and d3 , which are fixed . commonly injuries occur at the junction between d3 and d4 and at the junction between d1 and d2 , as seen in this case [ 1 , 2 ] . the retroperitoneal nature of the duodenum may also result in delays in the diagnosis of duodenal rupture as patients may not present with frank peritonism initially . it is therefore important to consider both mechanism of injury and also other clinical signs such as tachycardia and raised white cell count as delays in diagnosis and subsequent management have been shown to adversely affect morbidity and mortality [ 18 ] . however , in this case , due to the positioning of the perforation , duodenal contents entered the peritoneal cavity directly from the point of rupture , thus causing peritoneal irritation . duodenal injuries secondary to blunt trauma can range in severity from an intramural haematoma to a complete transection and devascularization of the duodenum , and are graded 15 by the american association for surgery of trauma [ 2 , 5 , 6 ] . ct scanning is a useful adjunct to in the diagnosis of duodenal rupture , and can aid in the differentiation between full thickness rupture that requires surgical intervention and a haematoma which can be managed conservatively . the finding of fluid in the right anterior para - renal space on a ct scan may be seen in the presence of a duodenal haematoma , whereas if air is seen in this area duodenal rupture is likely to have occurred [ 14 , 6 , 9 ] . in this case , ct scanning was useful in delineating the possible area where a perforation may be found , especially as the patient presented with generalized abdominal pain . there are multiple ways to repair a duodenal rupture and these are obviously dependant on the severity of the injury . the literature suggests that kocherization ( mobilization ) of the duodenum should be performed to allow full examination of the duodenum to rule out multiple perforations [ 1 , 2 , 4 , 7 , 9 ] . duodenal perforations are uncommon secondary to blunt trauma , comparative to penetrating trauma . a retrospective study analysing a trauma database of 103,864 patients showed that only 0.2% ( 208 patients ) had a duodenal injury related to blunt trauma and of these only 30 patients had full thickness duodenal rupture . the most common mechanism of injury was involvement in a road traffic accident [ 1 , 3 ] . duodenal rupture as a result of a sporting injury is very rare and there are no reports of duodenal injury from the specific mechanism described in this report [ 8 , 10 ] . this case demonstrates that even if the mechanism of injury seems trivial , a high level of suspicion should be maintained in the presence of suggestive clinical findings . it also shows the benefit of ct scanning in the diagnosis and management of duodenal rupture .

duodenal rupture secondary to blunt trauma is a relatively uncommon event and is usually a result of a road traffic accident . as the duodenum is a retroperitoneal organ , delays in diagnosis can occur , as the patient may present with vague abdominal symptoms and other non - specific signs . computed tomographic scanning is therefore a useful tool in the diagnosis of this condition . we present a 19-year - old girl who was hit in the abdomen with a football and subsequently had a duodenal rupture .

bisphosphonates have an important role in the prophylaxis and treatment of postmenopausal and corticosteroid osteoporosis . this case report presents a patient who presented to the ent department with sinus symptoms from oroantral fistulae following bisphosphonate use requiring surgery . this case report aims to raise awareness among ent surgeons to these patients on bisphosphonates that could present to them with sinus disease from oroantral fistulae . a 66 year old lady presented with facial pain , chronic purulent discharge into the mouth and signs of an oroantral communication . her medical history included type 2 diabetes , rheumatoid arthritis on prednisolone , hypertension and chronic pain syndrome . she had been on oral alendronic acid ( one of the bisphosphonates ) weekly for at least 9 months . , there was purulent material discharging from a sinus in the upper left fifth area in the oral cavity with a tender left maxilla . ct scans ( fig . 1 & 2 ) showed opacity and thickening of the left maxillary antrum with dehiscence of the medio - inferior and anterior walls of the sinus and a maxillo - oral fistula . there was destruction of bone in the floor of the left maxillary sinus consistent with an area of osteonecrosis secondary to bisphosphonate . coronal ct scan of the head showing osseo - sclerotic changes / oroantral fistula floor of left maxillary sinus axial ct scan of the head showing osseo - sclerotic changes and defect in left maxillary sinus she underwent exploration of her left maxilla with debridement of large necrotic sequestrum from the left maxillary alveolus . there was a large fistula into the maxillary sinus which was repaired with an advancement of buccal flap . bisphosphonates like alendronic acid , disodium etidronate , and risedronate are effective for preventing postmenopausal and corticosteroid induced osteoporosis . they are also useful in the treatment of paget 's disease , hypercalcaemia of malignancy and in bony metastases . osteonecrosis of the jaw ( onj ) has been increasingly reported following intravenous bisphosphonate use and rarely in those taking them orally . the vast majority of onj reported in the english language literature were seen following administration of intravenous pamidronate and zoledronate . of the only 28 reported cases following oral bisphosphonate use , onj frequently affects the mandible followed by the maxilla and in a number of cases , is preceded by a tooth extraction . patients can present with non - healing ulcers of the mandible or maxilla and can progress to pan - sinusitis with presentation to otolaryngologists ( 2 ) . in a series of 15 patients with onj , some had background breast cancer , osteoporosis , multiple myeloma and prostate cancer ( 3 ) . osteonecrosis of the jaw has also been shown in association with cancer chemotherapy in 10 patients who received bisphosphonates as treatment for their malignant bone disease ( 4 ) . the most common radiologic finding in bisphosphonate - associated osteonecrosis has been shown to be osseous sclerosis ( 3 ) . the spectrum varied from thickening of the lamina dura and alveolar crest ( subtle ) to attenuated osteopetrosis - like sclerosis . management will include the use of antibiotics , chlorhexidine mouthrinses and in cases of oroantral fistula , sequestrectomy , and surgical debridement . the aim of surgery is to try and remove all evidence of necrotic bone and to obtain mucosal coverage . there is an on - going 2 year audit by oral and maxillofacial surgeons in the uk in conjunction with the faculty of general dental practice , documenting the emerging trend of oroantral fistulae from bisphosphonate use ( bronj ) ( 5 ) . this case report highlights the need for awareness among ent surgeons to these patients on bisphosphonates who could present to them with sinus disease . with the millions of patients receiving various oral bisphosphonates for osteopenia and osteoporosis , health care practitioners should be aware of the potential for the onset of osteonecrosis and familiar with its management ( 1 ) .

bisphosphonates like alendronic acid , disodium etidronate , and risedronate are effective for preventing postmenopausal and corticosteroid induced osteoporosis . they are also useful in the treatment of paget 's disease , hypercalcaemia of malignancy and in bony metastases . however osteonecrosis of the jaw has been reported following intravenous bisphosphonate use and rarely in those taking them orally.increasingly , oroantral fistulae have been shown to occur as sequelae of bisphosphonate - induced osteonecrosis of the jaw and this case report highlights a patient that presented to our ent department and required sinus surgery in collaboration with maxillofacial surgeons.this case report aims to raise awareness among ent surgeons to these patients on bisphosphonates that could present to them with sinus disease from oroantral fistulae . there is an on - going audit in the maxillofacial community on this emerging trend .

dengue is the most rapidly spreading mosquito - borne viral disease in the world and in the last 50 years , incidence has increased 30-fold . in pakistan ( world health organization [ who ] eastern mediterranean region ) , the first confirmed outbreak of dengue hemorrhagic fever occurred in 1994 and since then dengue infections have been reported with increasing frequency and severity from large cities in pakistan . as the incidence of dengue is rising among adults more cases of dengue fever are being reported during pregnancy . dengue infection in pregnancy increases the risk of hemorrhage for both the mother and the newborn , especially if the mode of delivery is operative . we present the successful management of hemorrhage and unanticipated complications of severe dengue in a young primigravida admitted to the intensive care unit ( icu ) after an emergency cesarean section . an 18-year - old primigravida with no known co - morbids presented to one of the secondary setups of a university hospital at 35 weeks of gestation , with a history of fever for 5 days and threatened preterm labor . initial blood reports [ table 1 ] were normal along with normal liver function tests and negative screening for hepatitis , typhoid , and malaria . a baby girl of 2.8 kg was delivered by emergency lower segment cesarean section as tocolysis failed to delay labor . excessive bleeding per vaginum and hematuria was observed in the postanesthesia care unit , but she remained hemodynamically stable . oxytocin infusion was continued , four units packed cells and four units fresh frozen plasma ( ffp ) were transfused , and she was referred to the tertiary setup in anticipation of need for admission to the surgical icu ( sicu ) . on admission to the sicu , she was hypotensive ( blood pressure of 104/64 ) and tachycardic ( heart rate of 140/min ) but fully conscious . blood counts are shown in table 1 , and dengue serology was reported as positive . pharmacological measures ( oxytocin infusion 40 iu in 500 ml normal saline at 100 ml / h and misoprostol 800 g ) and balloon tamponade failed to control heavy per vaginal bleeding , and there was no structural or vascular injury , but the uterus was found to be atonic . b - lynch sutures were applied , and bilateral internal iliac artery ligation and vaginal tamponade was done to control the generalized ooze . in the icu , the vaginal bleeding continued , and she became hemodynamically unstable despite adequate volume replacement and transfusion of six units packed cells , 10 units of platelets , and 10 units of cryoprecipitate . two doses of activated factor vii were given to avoid hysterectomy in this young primigravida . however , the bleeding continued , and supracervical hysterectomy with ovarian conservation was performed as a lifesaving procedure . she was shifted back to the icu for hemodynamic monitoring , vasopressor support , mechanical ventilation , and transfusion of blood products . during her 16-day icu stay , she received mechanical ventilation for 9 days and noninvasive ventilation for another 5 days along with deep venous thrombosis prophylaxis , stress ulcer prophylaxis , and enteral nutrition via a nasogastric tube . the postoperative course was further complicated by an ileus , wound infection leading to wound dehiscence , persistent thrombocytopenia [ table 1 ] leading to hematuria , and ongoing fever treated with broad - spectrum antibiotics . a chest computed tomography after discharge from icu showed pulmonary embolism which was initially managed by heparin anticoagulation and later by warfarin . she also developed right sided leg weakness and pain which was diagnosed as acute right plexopathy on nerve conduction studies . she remained in the special care ward for another 6 days and was discharged home when she was afebrile , tolerated a regular diet , and mobilized with support . in addition , to the obstetrics and gynecology , she required follow - up by general surgery for wound management , chronic pain service for leg pain , and hematology for anticoagulation surveillance . severe dengue should be considered if the patient is from an area of dengue risk presenting with fever of 27 days and evidence of plasma leakage , significant bleeding , and severe gi involvement , altered level of consciousness , or severe organ impairment . diagnosis of dengue is difficult during pregnancy because the physiologic changes of pregnancy mask the pathognomonic features of severe dengue such as increased hematocrit , thrombocytopenia , and leucopenia . this patient had a normal white cell count , platelet count and hematocrit on presentation and thrombocytopenia was observed later [ table 1 ] . immunoglobulin m ( igm ) ( elisa ) is a widely used , simple and rapid method to detect dengue specific igm antibodies and should have been part of the initial workup of fever in this patient . conservative obstetrical management is preferred in women with dengue infections , but the incidence of cesarean deliveries is between 44% ( case reports ) and 20.4% ( case series ) . there are case reports of cesarean deliveries complicated by hemorrhage secondary to uterine atony and thrombocytopenia with variable outcome and the variable need for blood product transfusion . our patient received a transfusion of 36 units packed cells ( red cell concentrate ) , 44 units ffp , 49 units of platelets , nine single donor platelet mega units , and 20 units of cryoprecipitate during her hospital stay . she also received two doses of activated factor vii , as a last resort before hysterectomy was considered . the potential for thrombotic complications is known with the use of factor vii and this patient was diagnosed with pulmonary embolism after discharge from icu and may have resulted from activated factor vii use . who recommends that all patients with severe dengue should be admitted to a hospital with access to intensive care facilities and blood transfusion . icu admission may be required to manage thrombocytopenia ( < 20,000/mm ) , systemic inflammatory response or severe sepsis , severe bleeding , respiratory distress , hepatitis or jaundice , altered sensorium , shock , severe dehydration , or decreased urine output , and the associated mortality reported is 6.1% . hemodynamic monitoring and support , massive transfusion of various blood products , need for mechanical ventilation , and management of unanticipated problems required an icu stay of 16 days in this patient . as the incidence of dengue is rising among adults more cases of dengue fever are being reported during pregnancy and dengue serology should be part of the workup of unexplained fever in pregnancy in the endemic areas . surgical intervention in patients with suspected severe dengue should be undertaken cautiously , and early referral to healthcare centers where technical , transfusion and intensive care facilities are available may prove lifesaving .

as the incidence of dengue is rising among adults more cases of dengue fever are being reported during pregnancy . physiological changes of pregnancy mask the pathognomonic features of severe dengue such as increased hematocrit , thrombocytopenia , and leukopenia and a high index of suspicion are required in endemic areas . massive hemorrhage may complicate operative deliveries in unsuspected patients . world health organization recommends that all patients with severe dengue should be admitted to a hospital with access to intensive care facilities and blood transfusion . we present the successful management of hemorrhage and unanticipated complications of severe dengue in a young primigravida admitted to the intensive care unit after an emergency cesarean section .

san francisco , ca ) is a monoclonal antibody against vascular endothelial growth factor ( vegf ) which was approved by the united states food and drug administration for the treatment of metastatic colorectal cancer.1 ophthalmologists have used intravitreal injection of avastin off - label , for a number of indications including age - related macular degeneration , diabetic macular edema , cystoid macular edema and retinal vascular accidents.2 acute endophthalmitis is a rare but devastating complication of intravitreal bevacizumab ( ivb ) injection . the incidence of endophthalmitis varies in the literature from 1:1000 to 1:5233 per injection.35 potentially , bilateral intravitreal injection of bevacizumab can lead to bilateral endophthalmitis . our literature review during the research for this case report did not yield any reports of bilateral endophthalmitis following ivb injection . here we describe the first two cases in peer review literature of bilateral endophthalmitis after ivb . a 76-year - old female was referred to our emergency clinic with complaints of bilateral ocular pain and decreased vision 1 day after receiving bilateral ivb injection for diabetic macular edema . on examination , her visual acuity was hand movement and light perception in right and left eyes , respectively . significant anterior chamber reaction with a 0.7-mm hypopyon and severe vitritis were present in the left eye . due a high index of suspicion for bilateral postoperative endophthalmitis , the patient underwent diagnostic and therapeutic vitreous tap and intravitreal injection of 2.25 mg/0.1 ml ceftazidime and 1 mg/0.1 ml vancomycin . two days after antibiotic injection , visual acuity improved to 20/400 in both eyes and there was a significant improvement in the symptoms and signs of inflammation . a 52-year - old female with a history of bilateral ivb injection for diabetic macular edema , presented with bilateral ocular pain and photophobia 2 days after ivb injections . on examination , the patient underwent diagnostic and therapeutic vitreous tap and intravitreal injection of 2.25 mg/0.1 ml ceftazidime and 1 mg/0.1 ml vancomycin . two days later despite intravitreal antibiotics therapy , the signs and symptoms progressed and pars plana vitrectomy and intravitreal antibiotic injection was performed in both eyes . during vitrectomy , five days after vitrectomy , the signs and symptoms of endophthalmitis resolved . however , there was no improvement in visual acuity due to the retinal necrosis . a 76-year - old female was referred to our emergency clinic with complaints of bilateral ocular pain and decreased vision 1 day after receiving bilateral ivb injection for diabetic macular edema . on examination , her visual acuity was hand movement and light perception in right and left eyes , respectively . significant anterior chamber reaction with a 0.7-mm hypopyon and severe vitritis were present in the left eye . due a high index of suspicion for bilateral postoperative endophthalmitis , the patient underwent diagnostic and therapeutic vitreous tap and intravitreal injection of 2.25 mg/0.1 ml ceftazidime and 1 mg/0.1 ml vancomycin . two days after antibiotic injection , visual acuity improved to 20/400 in both eyes and there was a significant improvement in the symptoms and signs of inflammation . a 52-year - old female with a history of bilateral ivb injection for diabetic macular edema , presented with bilateral ocular pain and photophobia 2 days after ivb injections . on examination , the patient underwent diagnostic and therapeutic vitreous tap and intravitreal injection of 2.25 mg/0.1 ml ceftazidime and 1 mg/0.1 ml vancomycin . two days later despite intravitreal antibiotics therapy , the signs and symptoms progressed and pars plana vitrectomy and intravitreal antibiotic injection was performed in both eyes . during vitrectomy , five days after vitrectomy , the signs and symptoms of endophthalmitis resolved . however , there was no improvement in visual acuity due to the retinal necrosis . similar to our cases , most reports of endophthalmitis document decreased vision , ocular pain and redness , soon after ivb injection.36 recently , some standardization has been advocated to minimize the risk of postoperative endophthalmitis after ivb injection . these measures include preoperative cleansing of eyelids and conjunctiva with a 5% povidone - iodine solution , isolation of lids and lashes from the surgical field and treatment of high - risk patients with topical antibiotics . sterile and infectious endophthalmitis after intravitreal injection presents with similar sign and symptoms such as a rapid decrease in visual acuity . signs of infectious endophthalmitis include , inflammation , pain , fibrin , sudden and significant loss of vision within days of ivb . s. epidermidis is the most common pathogen isolated from the vitreous samples.78 the cause of infectious endophthalmitis after ivb remains contentious . some studies have implicated the needle used during ivb as it contacts the ocular surface and inoculates the vitreous.8 others suggest pharmacological compounding during preparation of bevacizumab for ophthalmic use as the cause of infection.9 prophylactic measures are particularly important for bilateral procedures . in the current cases , we recommend performing ivb injection in diabetics or immunocompromised patients in separate sessions for each eye . moreover , it is imperative to adhere to all prophylactic measures for each eye in all patients undergoing bilateral injection . we believe separate , surgical grade instruments ( including speculum , drug vial and calipers ) should be used for each eye in a bilateral procedure .

the clinical presentation and management of two patients who presented with acute bilateral endophthalmitis following bilateral intravitreal bevacizumab injection . both cases were diagnosed clinically and subsequent to a vitreous sample , intravitreal ceftazidime ( 2.25 mg/0.1ml ) and vancomycin ( 1 mg/0.1ml ) were injected . one patient had a significant improvement in signs and symptoms after intravitreal antibiotics . however , there were was no improvement in the other patient and pars plana vitrectomy was performed bilaterally . vitreous cultures were positive in both cases for staphylococcus epidermidis .

one hundred twenty - one thoroughbred mares ( 222 years old ) in hokkaido , japan housed under natural conditions were used for measuring the levels of circulating estradiol . for determining the correlation between the results for whole blood and serum , five pregnant mares , 819 years of age , were used . whole blood samples were collected weekly from the jugular vein into commercially supplied plastic tubes containing heparin sodium as an anticoagulant during day 233 of pregnancy and the day of delivery . for serum sample collection , whole blood was drawn from the jugular vein of the same mares into plain blood collection tubes . the concentrations of estradiol in all samples were measured by pathfast . for the experiments determining the correlation between the results of pathfast and time - resolved fia , two pregnant mares , 4 and 15 years old , were used . serum samples were collected monthly from the jugular vein into commercially supplied plastic tubes at the day of copulation and the day of delivery . the concentrations of estradiol in all samples were measured by pathfast and fia . for evaluating the pattern of circulating estradiol during gestation in mares , fifty - nine pregnant mares , ranging from 422 years of age , were used . serum samples were collected during gestation from the jugular vein into commercially supplied plastic tubes . all procedures were carried out in accordance with the guidelines established by the tokyo university of agriculture and technology for the care and use of horses in research . pathfast : the concentrations of estradiol in whole blood and serum samples were determined using the pathfast analyzer with the pathfast reagent kit for estradiol as described previously . in brief , estradiol measurement by pathfast was performed using a single reagent cartridge containing 100 l of whole blood and serum samples without extraction . in the competitive assays , estradiol in the samples was inhibited from forming an immunocomplex by an alkaline phosphatase - conjugated antigen . following immunoreaction for 5 min , separation of bound and free hormones was performed using the magtration technology . after addition of the chemiluminescent substrate to the immune complex , the amount of chemiluminescence was measured . the intra -assay coefficients of variance were 7.012.4% for whole blood , and 6.312.9% for serum samples . fia : serum estradiol concentrations were determined by a time - resolved fluoroimmunoassay using dissociation - enhanced fia systems ( perkinelmer , waltham , ma , usa ) as described previously [ 19 , 20 ] . the intra- and inter - assay coefficients of variation were 5.0% and 5.1% , respectively . pearson s r was calculated to determine the correlation between variables using graphpad prism software version 5 for windows ( graph pad software , san diego , ca , usa ) . one hundred twenty - one thoroughbred mares ( 222 years old ) in hokkaido , japan housed under natural conditions were used for measuring the levels of circulating estradiol . for determining the correlation between the results for whole blood and serum , five pregnant mares , 819 years of age , were used . whole blood samples were collected weekly from the jugular vein into commercially supplied plastic tubes containing heparin sodium as an anticoagulant during day 233 of pregnancy and the day of delivery . for serum sample collection , whole blood was drawn from the jugular vein of the same mares into plain blood collection tubes . the concentrations of estradiol in all samples were measured by pathfast . for the experiments determining the correlation between the results of pathfast and time - resolved fia , two pregnant mares , 4 and 15 years old , were used . serum samples were collected monthly from the jugular vein into commercially supplied plastic tubes at the day of copulation and the day of delivery . the concentrations of estradiol in all samples were measured by pathfast and fia . for evaluating the pattern of circulating estradiol during gestation in mares , fifty - nine pregnant mares , ranging from 422 years of age , were used . serum samples were collected during gestation from the jugular vein into commercially supplied plastic tubes . all procedures were carried out in accordance with the guidelines established by the tokyo university of agriculture and technology for the care and use of horses in research . pathfast : the concentrations of estradiol in whole blood and serum samples were determined using the pathfast analyzer with the pathfast reagent kit for estradiol as described previously . in brief , estradiol measurement by pathfast was performed using a single reagent cartridge containing 100 l of whole blood and serum samples without extraction . in the competitive assays , estradiol in the samples was inhibited from forming an immunocomplex by an alkaline phosphatase - conjugated antigen . following immunoreaction for 5 min , separation of bound and free hormones was performed using the magtration technology . after addition of the chemiluminescent substrate to the immune complex , the amount of chemiluminescence was measured . the assay range of pathfast was 202000 pg / ml . the intra -assay coefficients of variance were 7.012.4% for whole blood , and 6.312.9% for serum samples . fia : serum estradiol concentrations were determined by a time - resolved fluoroimmunoassay using dissociation - enhanced fia systems ( perkinelmer , waltham , ma , usa ) as described previously [ 19 , 20 ] . the intra- and inter - assay coefficients of variation were 5.0% and 5.1% , respectively . pearson s r was calculated to determine the correlation between variables using graphpad prism software version 5 for windows ( graph pad software , san diego , ca , usa ) .

a novel chemiluminescent enzyme immunoassay system , pathfast , for the measurement of estradiol in horses was evaluated . the concentrations of estradiol in the whole blood and serum of mares were measured using pathfast and the estradiol concentrations measured by pathfast were compared with those measured by a time - resolved fluoro - immunoassay ( fia ) . to monitor physiological changes , serum estradiol concentrations in mares were measured using pathfast throughout the gestation period . the serum estradiol concentrations correlated highly with those in whole blood samples . the serum concentrations of estradiol measured by pathfast also correlated well with fia . circulating estradiol increased during mid - gestation and high levels of serum estradiol were maintained in late gestation , followed by an abrupt decline to term . these results demonstrate the utility of pathfast in equine clinics as an accurate diagnostic tool for the rapid assay of estradiol within 26 min using unextracted whole blood .

kidney diseases are common in hiv - infected patients.17 the most common one is hiv - associated nephropathy ( hivan ) , as well as drug toxicities of various kinds . interstitial nephritis is a possible complication of hiv infection.8,9 it is usually caused by drugs such as indinavir , foscarnet , abacavir , and co - trimoxazole ; mycobacterial infections ; infections by other viruses ; or dysimmune syndromes such as immune reconstitution inflammatory syndrome and diffuse infiltrative lymphocytosis syndrome ( dils ; table 1).10,11 interstitial nephritis might occur as a direct consequence of hiv infection , but cases demonstrating this through exclusion of other etiologies are rare . herein , we present a case of interstitial nephritis that was likely caused directly by hiv infection and not by other etiologies . a 34-year - old african man was referred to our hospital because of microscopic hematuria identified at an annual health checkup at his workplace . the patient had no significant past medical history and was not taking any medications . the patient gave written informed consent to be included in this case report . upon initial routine workup , the patient s serum creatinine level was 0.86 mg / dl , with blood urea nitrogen of 10.1 mg / dl . urinalysis showed red blood cell ( rbc ) 3 + and urinary sediment showed dysmorphic rbcs ( > 100/high power field ) with rbc casts and absence of white blood cells . urine 2-microglobulin was 913 g / l , urine n - acetyl - beta - d - glucosaminidase was 14.9 the patient was subsequently diagnosed with pulmonary tuberculosis and was treated with a standard regimen including four drugs for 2 months , followed by isoniazid and rifampin for 4 months . because of persistent hematuria , the patient was hospitalized to undergo renal biopsy . the histopathological analysis revealed focal interstitial infiltration of lymphocytes and plasma cells in the renal cortex as well as in the corticomedullary junction , accompanied by mild tubulitis without microcysts ( figure 1 ) . no tubular necrosis was observed , with erythrocytic casts and flattened tubular epithelium ( figure 2 ) . analysis of glomeruli showed no evidence of podocyte hypertrophy , glomerular collapse , or endocapillary hypercellularity ( figure 2 ) . neelsen staining of the biopsied specimens was negative , and there were no pathological findings suggestive of tuberculosis . two weeks after the initiation of treatment for tuberculosis , antiretroviral therapy ( art ) , including lamivudine , abacavir , and dolutegravir , was started . eight months after the initiation of art , urinary levels of 2-microglobulin and n - acetyl - beta - d - glucosaminidase normalized and microscopic hematuria resolved completely . the onset and diagnosis of interstitial nephritis occurred prior to initiation of art or any other medications , eliminating the possibility of it being drug induced . inflammatory disorders such as immune reconstitution inflammatory syndrome and dils could not have been the cause of interstitial nephritis in the present case because art was started only after the onset of renal disease . moreover , other infections were unlikely to be causes of interstitial nephritis , as there were no signs of infectious diseases other than hiv and tuberculosis . hivan is the most common cause of renal dysfunction in patients with hiv , and only up to 10% of renal dysfunction is caused by interstitial nephritis.4,5 hivan is characterized by proteinuria , with histopathological changes such as focal and segmental glomerulosclerosis or collapsing or noncollapsing nephropathy . however , diagnostic confirmation by kidney biopsy is often important , particularly when the typical proteinuria is not observed , and other diagnoses such as those related to diabetes or hypertension can be confirmed frequently by kidney biopsy.12,13 hivan frequently accompanies interstitial inflammation of the kidney,1417 but the lack of pathognomonic findings in glomeruli in the present case made hivan unlikely . the lack of other glomerular changes such as podocyte hypertrophy and hyperplasia also contributed to excluding the diagnosis of hivan . the pathogenesis of hiv - associated renal diseases , including hivan , has been thoroughly investigated , and the majority of current knowledge was gained from studies using animal models . hiv-1 can infect renal epithelial cells through infected cd4 + lymphocytes , and viral proteins such as nef and vpr may have a synergistic role in inducing podocyte dysfunction . this also leads to renal tubular epithelial cell apoptosis and tubulointerstitial inflammation , which results in one of unique histopathological changes of hivan.15,18,19 through survey of the literature , we were unable to find studies on the pathogenesis of interstitial nephritis associated with hiv . whether similar pathophysiological observations related to tubulointerstitial inflammation observed in patients with hivan applies to our case remains unknown . the optimal therapy for hiv - associated interstitial nephritis is unknown,4 but art is likely to be effective , as shown in the present case . an early study suggested that corticosteroids may be beneficial for improving the inflammatory lesions of hivan,20 but this treatment regimen is not commonly prescribed in the era of art . additionally , we can not confirm that the application of corticosteroids would be beneficial for hiv - associated pure interstitial nephritis . the exact incidence and morbidity of interstitial nephritis caused by hiv physicians should be aware of the possibility , and kidney biopsies should be performed for differential diagnosis of interstitial nephritis . we identified interstitial nephritis without glomerular lesions in a treatment - naive hiv - infected patient , which was considered to be a complication of hiv infection . to our knowledge , the occurrence of interstitial nephritis in hiv - infected patients without other etiologies is rare , given the lack of similar reports . the findings presented herein are rare and should be further investigated to understand the potential role of interstitial nephritis on the prognosis of hiv infection .

interstitial nephritis is a common cause of renal dysfunction . it is primarily caused by drugs , infections , or autoimmune disorders . patients with human immunodeficiency virus ( hiv ) infection can develop interstitial nephritis , although it typically occurs because of the aforementioned etiologies and not as a direct consequence of hiv infection . interstitial lesions may occur in patients with hiv - associated nephropathy ( hivan ) . however , interstitial nephritis without the glomerular injuries characteristic of hivan , and without the risk factors described earlier , is very rare . here , we describe a rare case of interstitial nephritis that was likely caused directly by hiv infection and not by other etiologies .