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a 9-year - old male child presented with decreased vision in the left eye of 3 months duration . visual acuity was 0.0 and 0.8 logmar in the right and left eye , respectively . general examination revealed multiple cafe - au - lait skin spots over the back and arms [ fig . 1 ] . fundus examination revealed bilateral mprap and chrrpe in the left eye [ figs . 2 and 3 ] . suspecting nf-2 , neuroimaging was done ; magnetic resonance imaging revealed bilateral acoustic neuroma [ fig . 4 ] . optical coherence tomography ( oct ) showed vitreoretinal traction in both eyes and disorganized inner retinal structures in the left eye [ fig . 5 ] . fundus fluorescein angiography of mprap showed central hypofluorescence ( blocked fluorescence ) with surrounding hyperfluorescence ( staining ) [ fig . systemic examination revealed multiple caf - au - lait spots over skin left eye fundus showed elevated , pigmented macular lesion with surface wrinkling and vascular tortuosity suggestive of combined hamartoma of retina and retinal epithelium . both eye fundi showed multiple presumed retinal astrocytic proliferations ( arrows ) presumed retinal astrocytic proliferation ( circle ) in superonasal quadrant of right eye ( a ) , and closer view of combined hamartoma of retina and retinal epithelium in left eye ( b ) bilateral acoustic neuroma on magnetic resonance imaging ( arrows ) optical coherence tomography showed vitreoretinal traction in both eyes ( arrows ) and disorganized inner retinal structures in the left eye fundus fluorescein angiography showed central hypofluorescence ( blocked fluorescence ) with surrounding hyperfluorescence ( staining ) presumed solitary circumscribed retinal astrocytic proliferation is a recently described entity that occurs in middle - aged to older patients . it appears as a small , solitary , well - defined opaque lesion confined to the retina . our case exhibits the defined clinical characteristics but manifests with multiple lesions bilaterally in a 9-year - old boy , associated with chrrpe that we describe as mprap . oct scan through the presumed retinal astrocytic proliferation in the right eye showed characteristics of retinal astrocytic hamartoma ( rah ) type ii , as described by pichi et al . hence , it is possible to have multiple retinal astrocytic proliferations in a case of nf-2 . the differential diagnosis of a well - circumscribed yellow - white lesion of the retina includes rah , acquired retinal astrocytoma , retinoblastoma , retinocytoma , myelinated retinal nerve fibers , granuloma , reactive gliosis , unifocal helioid choroiditis ( solitary idiopathic choroiditis ) , and solitary circumscribed retinal astrocytic proliferation . rah is typically diagnosed early in life and often associated with tuberous sclerosis . in such cases , lesions are often multiple or bilateral . our patient did not have any of the stigmata of tuberous sclerosis but presented with cafe - au - lait spots , chrrpe , and neuroimaging revealed bilateral acoustic neuroma - all suggestive of nf-2 . retinocytomas appear as gray translucent tumors with intralesional calcification , surrounding retinal pigment epithelium alterations , and intralesional cysts . our case had smaller lesions than what is typically seen with retinocytoma and did not have the characteristic features of that lesion . unifocal helioid choroiditis ( solitary idiopathic choroiditis ) also presents with a yellow - white circumscribed lesion in the posterior pole . our case differs because of its location within retina , rather than choroid . in summary these lesions are well - circumscribed , opaque intraretinal lesions without exudation or feeding vessels . these differ from other white or yellow - white lesions of the retina in important ways that enable the clinician to appropriately image the patient for possible features of nf-2 . hence , recognition of this rare finding as presenting feature of nf-2 can lead to earlier diagnosis which is vital to appropriate surveillance and possible surgical intervention .
neurofibromatosis type 2 ( nf-2 ) is characterized by multifocal proliferation of neural crest - derived cells . the characteristics finding of nf-2 is bilateral vestibular schwannomas . combined hamartoma of retina and retinal epithelium ( chrrpe ) is another associated finding . a 9 year - old - male child presented with left eye decreased vision for 3 months . visual acuity was 0.0 and 0.8 logmar in the right and left eye , respectively . left fundus showed an elevated , pigmented lesion with surface wrinkling and vascular tortuosity suggestive of chrrpe with multiple presumed retinal astrocytic proliferations in mid - periphery . he had multiple caf - au - lait spots . optical coherence tomography confirmed clinical findings . magnetic resonance imaging brain showed bilateral acoustic neuroma . recognition of this rare finding as presenting feature of nf-2 can lead to earlier diagnosis which is vital to appropriate surveillance and possible surgical intervention . it is recommended that children with chrrpe be screened for nf-2 .
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the commonest cause of external pancreatic fistulae is pancreatic surgery with an incidence reported between 3 and 30% ; 90% of cases are initially treated conservatively although 20% may ultimately require surgery . a 39-year - old type 2 diabetic ( fn ) presented with a 48-h history of central crampy abdominal pain , radiating into his back and lower abdomen . he had not opened his bowels or passed flatus in 36 h , although his appetite remained normal . he had generalized abdominal tenderness localizing to the right iliac fossa ( rif ) with guarding . blood tests showed raised c - reactive protein ( crp ) 94 mg / l , white cell count 21.79 10/l and neutrophils 19.04 10/l with normal liver function tests . his condition deteriorated later that day , he became anorexic with localized peritonism in the rif . a clinical diagnosis of appendicitis was made , and the patient submitted to open appendicectomy . at operation , purulent fluid was found in the rif along with evidence of inflammatory changes in retroperitoneal tissues and a mildly inflamed retrocaecal appendix . iv antibiotics were commenced prior to and following surgery . at this stage , it was unclear whether the appendix was responsible for the inflammatory process , the decision to operate was based on clinical findings ; however , subsequent histology showed no active inflammation . on the first post - operative day a decision was made to proceed to laparotomy . at the surgery , copious blood - stained fluid a consultant hepato - pancreato - biliary ( hpb ) surgeon was called for an opinion and found a severely swollen pancreas with areas of fat necrosis ; therefore , a diagnosis of acute haemorrhagic pancreatitis was made . gallstones were neither seen nor found later by ultrasound scanning . excess alcohol intake was the suggested cause for the pancreatitis . he remained unwell and 14 days later a computed tomography ( ct ) scan was performed , which confirmed the appearances of acute pancreatitis with poor perfusion and necrosis of most of the head area . there was good perfusion of the body and tail , which were considerably swollen . on the 16th day , the amylase level of this fluid was 18 240 iu / l , suggesting the presence of a pancreatic fistula emerging via the appendicectomy wound ( see fig . serial ct scans continued to confirm that necrosis was limited to the head of the pancreas , the body and tail enhanced healthily . fluid collections were seen especially in the right paracolic area compatible with a fistulous track and drains inserted into this area on several occasions . however , this never significantly affected the persistent fistulous output through the appendicectomy wound which remained between 150 and 250 ml per day . after 8 weeks , the patient improved enough to be managed with regular home leave ; however , he did remain significantly nauseated and had a low mood throughout this period . figure 1:a coronal ct image 12 weeks following his initial presentation with necrotizing pancreatitis ; there is atrophy of the pancreatic head and a fluid collection tracks caudally to the right iliac fossa wound . the pancreatic duct is visible and of normal calibre . a coronal ct image 12 weeks following his initial presentation with necrotizing pancreatitis ; there is atrophy of the pancreatic head and a fluid collection tracks caudally to the right iliac fossa wound . his case was discussed with several hpb surgeons and a decision was made that further conservative management was futile . on day 113 after his first laparotomy there was extensive fibrosis around the right colon , where the fistulous track was found . it ran close in front of the duodenum but was not possible to track safely into the pancreatic head itself . a 50 cm roux loop procured from the proximal jejunum was anastomosed widely to the track just below the pancreatic head in front of the third part of the duodenum . post - operatively , lanreotide injections were maintained , also parenteral nutrition for the first 2 weeks . the fistulous output continued for 4 weeks , then declined to cease after 5 weeks . a ct scan confirmed no further collections and healthy looking body and tail of pancreas . he did not become diabetic and was well at 6- and 24-month follow - up . we are not aware that a pancreatic fistula has previously been described through an appendicectomy incision . it seems likely that , in this case , the original diagnosis of acute pancreatitis was missed because of clinical suspicion of appendicitis and laboratory difficulty measuring serum amylase because of lipaemia . this might have been a clue to the diagnosis , which could have been confirmed by a ct scan and obviated the need for an unnecessary appendectomy . this can not have been the first patient to undergo appendectomy at first presentation of pancreatitis . first , pancreatitis can present in unusual ways and it is worthwhile sending peritoneal fluid for amylase if the appendix appears grossly normal . secondly , this case confirms the fact that pancreatic fistulae are difficult to manage and may require prolonged conservative management . it is interesting to note that the conservative approach to management of this case seemed ineffective . pancreatic fistulae are not uncommon , and their development is due , in part , to proteolytic secretions , which can make them refractory to conservative management . in this case , a surgical approach to provide internal drainage was successful and proved an important and useful technique .
we report an unusual case of a pancreatic fistula communicating with an appendicectomy wound . this occurred following an episode of acute haemorrhagic pancreatitis . the patient was initially admitted with signs and symptoms indicating appendicitis and went to theatre for an open appendicectomy . however , this did not resolve his symptoms and a laparotomy was performed the next day revealing haemorrhagic pancreatitis . he endured a stormy post - operative course , the cause of which was found to be an external pancreatic fistula with discharge of amylase - rich fluid from the lanz incision . a trial of conservative management failed despite multiple percutaneous drainage procedures and treatment with broad - spectrum antibiotics . after a second opinion was sought , it was decided to fit a roux loop anastomosis between the head of the pancreas and the duodenum to divert the fistulous fluid . this procedure was a success and the patient remains well 2 years later .
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nail polishes and nail polish removers may contain a variety of hydrocarbon - based solvents , while nail adhesives usually contain various acrylic ester monomers . the chemical n , n - dimethyl - p - toluidine is a chemical commonly found in artificial fingernail solutions . its oxidation metabolite phenyl hydroxylamine is presumably one of the most potent chemical producers of methemoglobin . accidentally drinking small amounts of acetone / nail polish remover although there are so many reported case of nail polish removal poisoning in the western literature , but so far no such case reported in asia . a 13-month - old male child presented to us with history of ingestion of small volume of nail polish remover 2 h back and followed by he has started vomiting and gradually becoming lethargic . after admission , the child was kept under observation and we observed that gradually he became cyanosed , restless , and more lethargic . he had no other significant history . on general physical examination , at the beginning though he had no cyanosis or any significant observation . but , after about 1 h he found to have cyanosis . on systemic examination , he had only lethargy and irritability without any sign of respiratory distress . we investigated the child and he had all the hematological and biochemical parameters within normal limit . but , he had low oxygen saturation ( 85 - 87% ) in pulse oxymeter and simultaneously taken arterial blood gas showing po2 : 157 mm of hg with mildly increased lactate ( 1.9 mmol / lit ) and the ph and other parameter was within normal limit . as there was low spo2 with increased po2 , so we suspected that he must be having methemoglobinemia and further investigated for serum methemoglobin level and serum g6pd level . the serum methemoglobin level came out to be 72% and g6pd found to be in the normal range . the child was discharged after 1 day with the final diagnosis of toxic methemoglobinemia due to nail polish remover poisoning . when an infant presents severe cyanosis which is not associated with respiratory distress , methemoglobinemia should always be suspected . blood from the heel sticks is chocolate - brown and does not become pink when exposed to room air . in the absence of cyanide level determinations , lethargy , vomiting , seizures , and the lack of normal venous blood hemoglobin desaturation are clues to cyanide toxicity . diagnosis can be confirmed by excluding other causes of cyanosis and by spectrophotometric analysis of blood for methemoglobin . when methemoglobin levels reach 70% or more , the patient will collapse and become comatose and may die . treatment with methylene blue can be complicated by the presence of underlying enzyme deficiencies , including glucose-6-phosphate dehydrogenase deficiency . health care providers should not confuse the potentially highly toxic acetonitrile - containing cosmetics , particularly false - fingernail removers , with less - toxic acetone - containing fingernail - polish removers . this potential confusion between acetone and acetonitrile poisoning is compounded by the initial similarity of their early features , including vomiting , lethargy , slurred speech , ataxia , stupor , coma , and respiratory depression . delayed vomiting , although not typically a major clinical indicator of most cases of cyanide poisoning , may be important in alerting health care providers to acetonitrile toxicity in exposed children . however , vomiting is common from many causes and is not sufficient by itself to dictate the administration of a cyanide antidote in the absence of other supporting evidence of cyanide toxicity from history and clinical laboratory studies . sudden death is possible in some nail polish poisoning cases . the health care provider will measure and monitor the patient 's vital signs , including temperature , pulse , breathing rate , and blood pressure . the patient may receive activated charcoal if the patient arrives immediately after swallowing the poison , gastric lavage , oxygen , if needed . we wish to draw attention to the potential toxicity of acetone containing nail polish removers and the need for appropriate labeling of product packaging .
children are most susceptible to accidental exposure of common household substances and one of the common household substances is nail polish remover . we are presenting a case of accidental ingestion of nail polish remover with lethal methemoglobinemia ( serum methemoglobin level-72% ) . this patient was treated successfully with injection methylene blue . however , even small amounts can be dangerous to children , so it is important to keep this and all household chemicals in a safe place
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maffucci 's syndrome is a rare , congenital , nonhereditary mesodermal dysplasia which consists of hemangiomas of the soft tissue and multiple enchondromas.[13 ] it was first described by maffucci in 1881 . approximately 170 cases have been reported in the literature.[35 ] maffucci 's syndrome is known to be associated with malignant and benign tumors.[35 ] the common benign tumors associated with maffucci 's syndrome include pituitary adenoma , adrenal cortical adenoma , parathyroid adenoma , and breast fibroadenomas . most benign tumous with maffucci 's syndrome are endocrine tumors and are often associated with more than one tumor . a 30-year - old female was referred to the department of dermatology for the evaluation of multiple soft cystic swellings over the dorsum of both hands , palms , and dorsum of both feet since childhood . she was admitted to the department of gastroenterology with recurrent episodes of acute pain in the abdomen . the patient gave history of having recurrent episodes of pain in the left lower limb associated with hard swellings about 10 years back , for which she was admitted to a hospital and which was subsequently diagnosed as enchondroma of the lower end of the femur and was treated with bone curettage and grafting . histopathological examination of the specimen showed hyaline cartilage with mononuclear cells with lacunae . at the periphery were the trabeculae of lamellated bone and marrow space showed islands of hematopoietic cells , suggestive of enchondromas . other histories included recurrent episodes of cholelithiasis for which she underwent cholecystectomy and a history of renal calculi due to which she developed grade ii renal parenchymal disease . bluish cystic swellings showed over the dorsal aspect of both hands , palms [ figure 1 ] , and also on the ankles and dorsal aspect of both feet [ figure 2 ] . the swellings were 25 cm in diameter , nontender , and were not attached to the underlying structures . routine investigations were normal except for anemia and raised fbs ( 180 mg / dl ) . x - ray of both hands showed multiple cystic lesions on the radial aspect of the phalanges [ figure 3 ] . x - ray of the skull showed diffuse loss of bone density [ figure 4 ] . x - ray left knee showed surgical defect in lower end of the femur [ figure 5 ] . ercp showed distal narrowing with proximal dilation of common bile duct diagnosed as pancreatitis with a ductal leak . ultrasonography of the abdomen showed features suggestive of chronic pancreatitis and bilateral renal parenchymal disease . a ct scan of the abdomen showed postcholecystectomy status , altered pancreatic parenchyma with peripancreatic edema . serum amylase was raised ( 349 u / l ) ; hiv i and ii and hbsag were negative . with these clinical and laboratory findings she was diagnosed as muffucci 's syndrome with hyperparathyroidism . bluish swellings on the dorsum of the left hand bluish soft swellings over the left foot radiograph showing surgical defect involving lower end of femur radiograph of the skull showing diffuse osteoporosis x- ray both hands showing multiple cystic lesions on the radial aspect of phalanges ollier 's disease was also considered in the differential diagnosis but was ruled out as hemangiomas are not a feature of ollier 's disease . maffucci 's syndrome is a rare disease characterized by hemangiomas , enchondromas , and bone deformities . the venous malformations present as bluish , compressible , nontender , subcutaneous nodules in various areas of the body and usually have an asymmetric distribution . our patient also presented with similar venous malformations ; however , these lesions were bilateral . venous malformations have been reported in leptomeninges , the eyes , the pharynx , the trachea , and the intestines . enchondromas may appear anywhere on the body but are most often found on the hands or feet or on long bones of limbs or on the ribs and the skull . the case under study also had enchondromas , which was treated with curettage and bone grafting . the bone deformities include shortening of long bones , resulting in unequal arms and legs . lewis and ketchum found various benign and malignant neoplasms in their extensive review of the world literature of 105 cases . the benign tumors include pituitary adenoma , adrenal cortical adenoma , parathyroid adenoma , and breast fibroadenoma . the malignant tumors associated are chondrosarcoma , astrocytoma , ovarian tumors , pancreatic cancer , hemangiosarcoma , and lymphangiosarcoma . have reported three different endocrine tumors with muffucci 's syndrome involving parathyroid , thyroid , and adrenal glands . kenji et al . have reported multiple endocrine tumors ( men ) type i with muffucci 's syndrome involving pituitary , adrenal , and parathyroid glands . adrian et al . have also reported maffucci 's syndrome with pituitary and parathyroid adenomas along with two other neoplasms . there are only four case reports of maffucci 's syndrome associated with parathyroid involvement resulting in hyperparathyroidism . although cystic lesions of the bone and ectopic calcification are common to both the diseases , pathogenesis is apparently unrelated . the association of parathyroid involvement with maffucci 's syndrome suggests the potential involvement of ectodermal structures in this mesenchymal dysplastic disorder .
maffucci 's syndrome is a rare , congenital , nonhereditary , mesodermal dysplastic disease characterized by venous malformations and benign cartilaginous tumors . the occurrence of endocrine tumors in maffucci 's syndrome is very rare . we report a case of maffucci 's syndrome associated with hyperparathyroidism and multinodular goiter .
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we examined a 33-year - old male with progressive painless decrease in vision and nystagmus since childhood and no history of night blindness . his best corrected visual acuity was 20/80 with 0.00 diopter sphere ( d sph ) / -4.00 diopter cylinder ( d cyl ) x 10 in the right eye and 20/60 with 0.00 d sph / -3.50 d cyl x 170 in the left eye respectively . a positive history of similar abnormality was obtained in his sibling ( elder brother had similar abnormality ) . fundus examination showed macular vascularization , macular hypoplasia and hypoplasic optic nerve head with diffuse retinal pigment epithelial atrophy and prominent choroidal vessels in both eyes . sd - oct ( copernicus , italy ) was done in both eyes using asterisk scan protocol ( 7 mm length scan , 6 b - scans with 2743 a - scans per b - scan ) . sd - oct was recorded with the eyes in the null zone , to overcome nystagmus . the scan location was assured by positioning the asterisk scan acquisition pattern over the foveal area and this location was assured as the scan passes through the fovea in the fundus image obtained in sd - oct . the scan revealed absence of foveal dip , preservation of the inner retinal layers with thickened fovea , 270 microns in the right eye and 245 microns in the left eye . the qualitative and quantitative characteristics of the retina were similar in macular and peripheral areas ( 2 and 7 respectively ) . the small elevation of the inner segment/ outer segment ( is / os ) junction was absent in the fovea which is otherwise present in normal eyes . microperimetry ( mp1 , nidek technologies , padova , italy ) using 33 stimuli in the central 20 of the macula and 4 - 2 threshold strategy revealed reduction of retinal sensitivity in the central retina . mean retinal sensitivity was 7.2 db in the right eye and 14.2 db in the left eye . fixation was relatively unstable in both eyes , with abnormal fixation location ( central fixation in the right eye and eccentric fixation in the left eye ) . average eye movements during the microperimetry were 1.27/sec in the right eye and 1.49/sec in the left eye . 1 and 2 describe the color fundus photography , microperimetry and sd - oct findings in our case . fundus autofluorescence ( faf ) imaging with confocal scanning laser ophthalmoscope ( heidelberg retina angiograph , hra ii , heidelberg engineering , heidelberg , germany ) did not show the typical foveal darkening in both eyes [ fig . 3 ] . ( a ) color fundus photography of the right eye reveals macular vascularization and disc hypoplasia . ( c ) fundus - reconstructed image from sd - oct showing the location of the scan . ( d ) sd - oct image shows absence of foveal depression and persistence of inner retinal layers at the fovea ( a ) color fundus photography of the left eye reveals macular vascularization and disc hypoplasia . ( c ) fundus - reconstructed image from sd - oct showing the location of scan . ( d ) sd - oct image shows absence of foveal depression and persistence of inner retinal layers at the fovea fundus autofluorescence of right eye and left eye shows reduced autofl uorescence at the macula foveal hypoplasia in oculocutaneous albinism is commonly associated with ocular features like decreased visual acuity , nystagmus , photophobia , strabismus , iris transillumination , macular transparency and neuronal abnormality . both eyes had high astigmatism with reasonably good visual acuity in contrast to previously reported studies . , ) in view of mild foveal hyporeflectivity , < 2 choroidal transillumination , absence of tram - tract sign and foveal depression . sd - oct showed absence of foveal depression , as reported in previous studies using time domain oct.[146 ] the foveal thickness in this case was higher due to the presence of inner retinal layers in the foveal area . this can be due to the absence of the chievitz layer which has an important role in the foveal maturity . in the normal fovea , the photoreceptor layer appears as a distinct hyper - reflective layer with high - resolution oct due to the difference in the optical properties of the photoreceptor segments , and the distance between the retinal pigment epithelium and the is / os junction of the photoreceptors increases significantly in the foveal region , consistent with the well - known increase in the length of the outer cone segments in this region . in case of foveal hypoplasia in our case we found persistent is / os junction of the photoreceptor layer in the foveal area in both the eyes but the central is / os junctional elevation was absent . this could be due to no difference in the size of the cones present in the foveal and peripheral retina . the retinal sensitivity was less in the central macular area compared to the peripheral area . abnormal cones in the central fovea cause low vision which affect the emmetropization process and can lead to nystagmus . in our case the patient had jerky nystagmus and the average eye movement measured with microperimetry was relatively higher compared to the normal age - matched control ( 0.18/sec , unpublished data ) . the fixation of this patient was relatively unstable in both the eyes due to nystagmus . faf of the central macula was reduced which may be due to the amount of macular pigment present . in the literature our case report shows foveal hypoplasia associated with ocular albinism in which sd - oct and microperimetry were used for the first time .
a case of foveal hypoplasia associated with ocular albinism with anatomic and functional changes by various techniques using spectral domain optical coherence tomography ( sd - oct ) , microperimeter and confocal scanning laser ophthalmoscope is described . this case highlights the importance of microperimeter in detecting the functional abnormalities of vision and sd - oct in identifying the retinal laminar abnormalities in foveal hypoplasia .
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a 47-year - old woman was a dmitted to o ur clinic for persistent purulent discharge from the left eye for a duration of six months . she had been diagnosed with chronic conjunctivitis at a private eye clinic . despite conservative treatment , the symptom was persistent , leading to referral of this patient to our clinic . her corrected visual acuity was 1.2 , and no specific findings were noted in her past medical history . slit lamp examination showed discharge via a fistula in the central part of the caruncle of the left eye , a red and swollen caruncle , and mild hyperemic conjunctiva ( fig . lacrimal drainage was patent to syringing and showed no reflux in the upper or lower canaliculus of the left eye . computed tomography ( ct ) scan and magnetic resonance imaging ( mri ) showed an elliptical hypo - dense lesion of about 1 cm in diameter in the medial portion of the left lower eyelid ( fig . intraoperative lacrimal probing and irrigation were performed to confirm that the abscess and canaliculus were not connected . bacteria were not identified in the culture , but biopsy of the lesion confirmed a benign purulent cyst with ' sulfur granules ' by histopathologic examination ( fig . postoperative oral and topical antibiotics were administered ; the lesion resolved with no evidence of recurrence within four months , and the symptom significantly improved . actinomycosis is a chronic disease that spreads to surrounding tissues with the formation of abscesses and granulomas that are typically accompanied by microcolonies known as ' sulfur granules ' . actinomyces is a genus of the actinobacteria , anaerobic gram - positive organisms that grow through the formation of hyphae . the diagnosis of actinomycosis is confirmed microbiologically by the identification of actinomyces in a sample obtained from the lesion and pathologically by observing sulfur granules from tissue or an abscess . actinomycosis is a relatively rare disease , and although some cases of orbital cellulitis and chronic endophthalmitis after cataract surgery were reported to generate cultured actinomyces [ 6 - 8 ] , most of the described cases have been canaliculitis . actinomyces canaliculitis is usually accompanied by a mucopurulent punctal discharge , epiphora , inflammation of the medial canthus , a thickened canaliculus , and a red pouting punctum . since the frequency of canaliculitis is low , it is often mistaken for refractory chronic or recurrent conjunctivitis , although differentiation is possible by confirming the presence of purulent discharge from the punctum . the case under discussion was originally mistaken for chronic conjunctivitis due to erythema , swelling , and purulent discharge from the pericaruncular area . after failing to respond to treatment , the patient was referred to us . although canaliculitis can show a similar pattern , the purulent discharge was observed to not originate from the punctum , and a ct scan and mri identified a lesion near the lower eyelid , which led to a preoperative diagnosis of a caruncular abscess . topical ciprofloxacin and oral penicillin antibiotics were administered postoperatively , and the erythema and swelling of the pericaruncular area gradually decreased ; purulent discharge was no longer observed . reports of caruncle infection a re d ifficult to find , although koo and chang reported a case of caruncular abscess with methicillin - resistant staphylococcus aureus , and pappalardo et al . reported granules of the caruncle caused by actinomycosis . to our knowledge , this is the first reported case of caruncular abscess with chronic purulent discharge caused by actinomycosis in korea .
the authors report a caruncular abscess caused by actinomycosis . a 47-year - old woman was admitted with persistent purulent discharge from the caruncle of the left eye for a duration of six months . excisional drainage was performed , and ' sulfur granules ' were observed , consistent with actinomyces infection . intraoperative lacrimal probing and irrigation were performed to confirm that the abscess and canaliculus were not connected . oral and topical antibiotics were administered postoperatively ; the lesion resolved with no evidence of recurrence , and the symptom improved .
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the most common cause of sellar calcification is craniopharyngioma but it can be seen very rarely in pituitary adenomas and rathke 's cleft cysts . incidence of calcification in pituitary adenoma on imaging varies from 0.8 to 8.0% , but usually it is < 2% . pituitary calcification has been reported most commonly in prolactinoma , somatotroph adenoma , thyrotroph adenoma and rarely in gonadotroph and corticotroph adenoma . here we report a case of pituitary calcification , which was earlier thought to have pituitary apoplexy because of findings on imaging . however , on follow up he had normal pituitary functions and findings on imaging remain unchanged and were suggestive of pituitary calcification . a 32-year - old male reported for review after three years of an episode of headache , vomiting , and confusional state when he was posted to high altitude area , when his computerized tomography ( ct ) scan of the sella showed a mildly enlarged sella with hyperdensity within the adenohypophysis [ figure 1a ] and magnetic resonance imaging ( mri ) revealed focal hyperintensity within the pituitary on t1 weighted imaging [ figure 1b ] which appeared hypointense on t2 weighted scans [ figure 1c ] . his hormonal evaluation showed normal thyroid ( ft4 - 1.5 ng / dl , tsh - 1.72 miu / l ) , adrenal ( basal cortisol - 10.6 g / dl , stimulated cortisol-29.12 g / dl ) , gonadal ( lh - 6.78 iu / l , fsh - 7.97 iu / l , testosterone - 4.08 ng / ml ) and prolactin levels ( 9.97 ng / ml ) . mri and ct done for the sella revealed unchanged findings compared to the previous imaging done in 2009 [ figure 2a - c ] . he was diagnosed as a case of non - functioning pituitary adenoma with pituitary calcification . in view of normal visual perimetry and pituitary function with no progression of lesion for three years ( a ) ct scan showing hyperdense lesion in sella ; ( b ) t1 weighted hyperintensity and ( c ) t2 weighted hypointensity in the sellar region in mri in 2009 ( a ) ct scan showing hyperdense lesion in sella ; ( b ) t1 weighted hyperintensity and ( c ) t2 weighted hypointensity in the sellar region in mri in 2012 surprisingly most of reports of pituitary calcifications are from japan and few from other countries in asia including india . though calcifications in pituitary adenomas are rare on imaging , a higher incidence of microscopically proven calcified adenomas has been recently reported . ncct and mri pituitary showed hyper - dense lesion and t1 weighted focal hyper - intensity within the pituitary respectively , suggestive of hemorrhage . hormonal evaluation revealed evidence of secondary adrenal insufficiency , hence was diagnosed as a case of pituitary apoplexy . horiuchi et al . , reported a case of male prolactinoma with pituitary calcification who also had features of hypopituitarism . , to explain pituitary stone in a case of acromegaly . in present case , sella is enlarged ; hence there is high probability of pituitary adenoma which is hormonally inactive . however , normal pituitary function makes pituitary apoplexy in the past unlikely but possibility can not be rules out . he had normal libido and had a child about 6-year old , which also indicate unlikelihood of long standing prolactinoma or hypopituitarism in the past . t1 weighted hyperintensity of the sellar region at mri is a common finding due to vasopressin storage in the posteriorly located neurohypophysis . it is also seen normally in newborns and pregnant or lactating women due to hyperactive hormone secretion in the anteriorpituitary . pathologically , t1 weighted hyperintensity can be caused by blood as in hemorrhagic pituitary adenoma , pituitary apoplexy or by the presence of a high concentration of protein in rathke 's cleft cyst , craniopharyngioma , or mucocele , by presence of fat in lipoma , dermoid cyst , and lipomatous meningioma , and due to calcificationas in craniopharyngioma , and sometimes due to presence of paramagnetic substance like manganese or melanin . in our case presence of calcification on firstly , calcifications in the intratumoral hematoma ; secondly , calcifications in the degenerative adenoma tissue ; and finally , scattered psammoma bodies between the adenoma cells . other types of calcification were thought to be the result from degenerative or hemorrhagic change of the adenoma . in our case surgical intervention was deferred in view of asymptomatic , non - progressive nature of lesion , hence histopathology of the lesion is not available .
pituitary calcification occurs commonly in lactotroph or somatotroph adenoma but rare in chomophobe or gonadotroph adenoma . on imaging , it can mimic hemorrhage , hence may masquerade pituitary apoplexy if patient present with neurological manifestations . we present a case of pituitary calcification which mimicked pituitary apoplexy .
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acral ( lentiginous ) melanoma ( alm ) is the most prevalent subtype in the asian and mexican mestizo populations . acral lentiginous melanoma is most often diagnosed at an advanced stage and associated with a poor outcome [ 24 ] . dermoscopy is a noninvasive diagnostic technique that allows early recognition and increases diagnostic accuracy of pigmented skin lesions on acral volar skin [ 18 ] . congenital melanocytic nevi ( cmn ) are defined as melanocytic nevi that are present at birth or become apparent shortly after . acral cmn tend to show a greater size and a greater variability in color and shape than acquired nevi . therefore , a histopathologic examination is sometimes required for differentiating acral cmn from alm , especially when the lesion has a large diameter and/or is clinical and dermoscopically atypical . according to previous studies , acral cmn show characteristic dermoscopic features such as a combination of the crista dotted and parallel furrow patterns dermoscopically , the parallel ridge pattern is most commonly associated with alm in situ showing a high sensitivity ( 86% ) and specificity ( 99% ) for its diagnosis . we , herein , present a case of acral cmn nevus with a parallel ridge dermoscopic pattern along with its histopathological correlates . a 37-year - old , male patient , skin phototype iv , while being hospitalized for the treatment of schizophrenia , was seen for a 12 mm , pigmented skin lesion on his plantar arch ( figures 1 and 2 ) . according to the patient s mother , the lesion had appeared shortly after birth and had been progressively enlarging in the course of the last years . under polarized dermoscopy , the melanocytic lesion was polychromatic with a central blue - grey area and dark and light brown colors in the periphery ; a central parallel ridge pattern blended with a peripheral lattice - like pattern associated with few asymmetric dots and globules . based on the large size and the atypical dermoscopic presentation , the lesion was excised . histologic examination revealed a very bland compound melanocytic neoplasm with clear - cut congenital - like features ( figure 4a ) . the central area of the lesion was mainly an intradermal combination of type c and dendritic ( blue nevus - like ) melanocytes ( figure 4b ) , with a striking acrosyringial and periductal distribution ( figure 4c ) ; the junctional component was mainly peripheral , with regularly arranged nests without cytologic atypia ( figure d ) and melanin columns under the surface furrows . this last histologic finding was also seen in the crista profunda intermedia ( figures 4d and 4e ) under the surface ridge in accordance with the dermoscopic appearance of the lesion . dermoscopy is a powerful , noninvasive , diagnostic tool , which helps the clinical differential diagnosis between benign and malignant melanocytic lesions on volar skin . it is commonly diagnosed at an advanced stage and associated with a poor outcome [ 24 ] . however , it has also been described to occur in acral cmn as a relatively rare finding . in the latter occurrence , anamnestic data and stable clinicodermoscopic features on digital follow - up support the diagnosis of benignity . in our case , the lesion was reported as congenital , but seemed to have been enlarging even in recent times . this was interpreted as an atypical feature , inasmuch as cmn on acral volar skin commonly tend to fade over years or , at least , do not commonly reveal a parallel ridge pattern . dermoscopically , the pattern was atypical also because it was characterized by pigmentation along the ridges . furthermore histopathologically , a dermal dendritic cell component was found as responsible for the central bluish area ; the striking growth of melanocytes surrounding the ductal compartment of the eccrine sweat glands might be considered as a further histopathological correlate for the central pigmentation along the ridges ( where acrosyringia emerge ) . it has been recently proposed by some authors that eccrine melanocytic precursor cells may be the source of acral melanomas . the junctional component was mainly peripheral and responsible for the lattice - like pattern , a quite common finding in melanocytic nevi of the plantar arch . recently , chuah et al . confirmed that acral congenital melanocytic nevi tend to be larger and more asymmetrical than acral aquired melanocytic nevi . furthermore , they tend to be polychromatic and approximately 50% have a blue - grey coloration in the central portion of the lesion , which may be associated with the intradermal component . other authors have also recorded a parallel ridge pattern in other benign conditions such as lentiginosis , racial melanosis , melanocytic nevi , drug - induced hyperpigmentation , subcorneal hemorrhage and dye - related pigmentation , particularly in darker skin phototypes . our case is an example of acral cmn with a parallel ridge pattern observed in an adult . acral cmn may be larger and more asymmetrical and polychromatic than acral acquired melanocytic nevi , but tend to fade or to become architecturally organized in adulthood . thus , if the patient is uncertain whether the lesion has recently changed / enlarged and/or if follow - up can not be completed , histopathological examination may be required . in the final dermoscopic - pathologic evaluation of the case , one must remember that the dermoscopic features of an acral melanocytic lesion are best evaluated at its periphery .
acral melanoma is the most frequent subtype in the asian and mexican mestizo populations . dermoscopy is a noninvasive diagnostic technique that helps the differential diagnosis of pigmented skin lesions on acral volar skin.we , herein , present a case of acral congenital melanocytic nevus with a parallel ridge dermoscopic pattern . since the parallel ridge pattern in a melanocytic lesion of the acral skin is classically ascribed to melanoma , the present case can be definitely labeled as atypical and worth of being elucidated in its histopathological correlates .
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the quest for a therapeutic to ameliorate ischemic and traumatic brain injury is certainly a noble ideal , but , thus far , a futile endeavor . in the previous issue of critical care , loetscher and colleagues provided further evidence that the inert , noble gases may have ameliorative properties in the setting of acute neuronal injury . stimulated by a shared interest in the neuroprotective properties of another noble gas , xenon [ 2 - 4 ] , they have shifted their focus to argon , a gas that is more abundant and cheaper to obtain . in their current investigation , they demonstrate that argon is neuroprotective when applied after an oxygen - glucose deprivation ( ogd ) or traumatic injury in organotypic hippocampal slice cultures in vitro . the models the authors employ are robust ; the cultured slices have intact synaptic networks , replicating the in vivo setting well ; ogd is a well - described simulation of ischemic brain injury ; similarly , the trauma model replicates the clinical situation . loetscher and colleagues report a dose - responsive neuroprotective effect , with 50% argon appearing to be the optimal concentration for neuroprotection . furthermore , argon was even neuroprotective when administered 3 hours after the injury . although this report used only in vitro models , it is a foundation on which to base further studies that may further reveal argon 's potential in a field largely bereft of interventions to improve neurological outcome from ischemic or traumatic brain injury . we recently reported that argon ( 75% ) prevented neuronal injury from ogd in vitro but that the protection afforded was inferior to that of xenon . xenon has been shown to be neuroprotective in multiple models and species and has now entered clinical trials for neonatal hypoxic - ischemic brain injury ( tobyxe ; nct00934700 ) . if argon is also to be exploited clinically , it too must undergo rigorous examination in different animal models , species , laboratories , and clinically relevant injury settings . while at this stage argon fulfills some criteria , it would be imprudent , in the absence of in vivo data , to hail argon as the elusive neuroprotective agent . why has there been a cascade of studies exploring the clinical utility of noble gases [ 1 - 5 , 7,8 ] ? helium , neon , argon , krypton and xenon , the first five noble gases in the periodic table , contain a full outer shell of electrons , precluding the formation of covalent bonds under biological conditions ; thus , they are chemically inert . due to the uncharged and non - polar nature of their chemical composition , these gases are able to easily partition into the brain and are able to fit snugly into amphiphilic binding cavities within proteins . depending on the properties of the surrounding electrons , some of the noble gases can create an instantaneous dipole in the atom from a charged binding site , thereby promoting a biological effect , including induction of anesthesia . neon and helium are thought to create an unfavorable balance between binding energies and repulsive forces and therefore do not produce anesthesia and other biological effects . in the case of xenon , there are several candidate molecules that may be capable of producing the cytoprotective properties , including the nmda ( n - methyld- aspartic acid ) subtype of the glutamate receptor , the atp - sensitive potassium channel , the two - pore potassium channel , and an as - yet - unidentified protein that is upstream of mtor ( mammalian target of rapamycin ) . a reduced ability to form induced dipoles with argon ( due to its smaller size ) may limit the number of available protein - binding sites when compared with xenon . indeed , there are important pharmacodynamic differences between xenon and argon ; in particular , xenon is an anesthetic at atmospheric pressure , argon is not . nonetheless , argon 's lack of sedative properties may actually be beneficial as it allows administration to patients with acute , focal neurological injury ( such as stroke ) , who would not necessarily benefit from sedation . xenon 's cost necessitates administration through cumbersome recirculating and recycling systems ; argon is substantially cheaper and thus may be feasibly administered through open circuits . however , a decade of investigation of the effects of xenon has led to a clinical trial that may yet change clinical care of perinatal asphyxia . the findings of loetscher and colleagues should encourage the pursuit of argon as a neuroprotective alternative / supplement to xenon . mm has received consultancy fees and funding from air products ( allentown , pa , usa ) and air liquide sant international ( paris , france ) concerning the development of clinical applications for medical gases , including xenon . rds has received consultancy fees from air liquide sant international concerning the development of clinical applications for xenon .
certain noble gases , though inert , exhibit remarkable biological properties . notably , xenon and argon provide neuroprotection in animal models of central nervous system injury . in the previous issue of critical care , loetscher and colleagues provided further evidence that argon may have therapeutic properties for neuronal toxicity by demonstrating protection against both traumatic and oxygen - glucose deprivation injury of organotypic hippocampal cultures in vitro . their data are of interest as argon is more abundant , and therefore cheaper , than xenon ( the latter of which is currently in clinical trials for perinatal hypoxic - ischemic brain injury ; tobyxe ; nct00934700 ) . we eagerly await in vivo data to complement the promising in vitro data hailing argon neuroprotection .
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in the previous issue of critical care , nakos and colleagues presented interesting experimental research in sheep , reporting beneficial effects of the prone position on the damage of mechanical ventilation ( mv ) on lung tissue and apoptosis in several vital organs . these observations are an interesting addition to a number of experimental and clinical studies showing that mv can initiate as well as exacerbate lung injury , and can worsen other vital organ function . ventilator - induced injury ( vili ) can thereby contribute to an unfavourable outcome . at least two different basic mechanisms are involved in vili and peripheral organ dysfunction : direct mechanical lung damage and enhancement of inflammatory changes in pulmonary tissue . as a result , subsequent pathophysiological pathways contribute to clinical symptoms and morbidity , including translocation of inflammatory mediators , endotoxins and bacteria from the lung to the systemic circulation . the clinical relevance of vili in the intensive care unit is confirmed by the beneficial effects on outcome of protective ventilatory techniques , including the use of lower tidal volumes and plateau pressures , as well as higher levels of positive end - expiratory pressure . the study of nakos and colleagues expands the findings of two recent publications on potentially beneficial effects of the prone position on vili and its systemic complications . in an experimental work on normal rats , valenza and colleagues observed a more homogeneous distribution of lung strain during mv in the prone position , assessed by computed tomography . these data suggest that a better distribution of alveolar ventilation in the prone position could be the cause of the delayed occurrence of vili compared with the supine position . in the other recent investigation , mentzelopoulos and colleagues examined the overall parenchymal lung stress and strain , estimated from the transpulmonary plateau pressure and the tidal volume to end - expiratory lung volume ratio , in 10 patients with severe ards . both of these indexes were reduced in the prone position compared with the semirecumbant position . this suggests that lung tissue damage by vili can be reduced by the prone position . in the aforementioned study of vili in normal sheep , nakos and colleagues it is noteworthy that the type of mv used ( tidal volume of 15 ml / kg body weight and positive end - expiratory pressure of 3 cmh2o ) for a duration of only 90 minutes did produce marked alterations in the lung and certain distal organs . the prone position made a significant difference only for the lung , the liver and the diaphragm . in contrast , apoptotic changes in the kidney , the brain and the intestine were no different between the supine and prone positions . how could these findings be explained ? first , the modifications of lung histology observed are in line with some earlier studies [ 7,9 - 12 ] and could be explained by differences in the distribution of ventilation , in tissular stress and strain as well as in changes of interactions between the weight of the heart and underlying lung tissue in the supine and prone positions . more novel approaches may be needed to explain the different intensities of apoptosis observed in different organs . although such observations have been reported previously , little is known about the causes of programmed cell death in this situation . one of the suggested mechanisms could be the increased systemic plasma levels of inflammatory mediators and proaptotic soluble factors such as fas ligand , but this does not explain the profound differences between some organs . other factors such as different sensibility for these circulating proteins and/or differences in organ perfusion between the supine and prone positions may explain the more protective effect of the prone position for the liver and the diaphragm than for the kidney and the intestine epithelial cells . these changes in cell biology induced by mv and the protective role of the body position seem an exciting area for further research . the optimal position in an intensive care unit patient in regard to vili remains to be defined , and it could be different from the sheep model studied by nakos and colleagues .
mechanical ventilation can cause structural and functional disturbances in the lung , as well as other vital organ dysfunctions . apoptosis is thought to be a histological sign of distant organ damage in ventilator - induced lung injury ( vili ) . nakos and colleagues observed a protective effect of prone positioning against vili in normal sheep . less alteration in the lung architecture and function and in liver transaminases , and lower indices for apoptosis in the liver , the diaphragm and the lung were noted in the prone position compared with the supine position . if confirmed , these data open a new hypothesis for pathogenesis and prevention of vili and its extrapulmonary complications .
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familial juvenile hyperuricemic nephropathy ( fjhn1 ; mim 162000 ) is an autosomal dominant condition characterized by defective urinary concentrating ability , gouty arthritis , interstitial nephritis , and chronic renal failure . it is a genetically heterogeneous condition , caused due to mutation in three genes : uromodulin ( umod ) ( 40% ) , renin ( 2.5% ) , and hepatocyte nuclear factor-1 beta ( 2.5% ) . biochemical hallmarks of the disease are hyperuricemia out of proportion to the degree of renal failure and reduced fractional uric acid excretion . here , we describe an indian family with multiple members affected with fjhn1 due to a novel heterozygous missense mutation in exon 5 of umod gene . a 17-year - old male presented with pain and swelling in small joints of hands , feet , knees , ankles , elbows , and wrists for 2 years along with the presence of nodules on the right little finger and the left ear lobe . on examination , swellings were present on second and third metacarpophalangeal joints and third proximal interphalangeal joints . there were tophi of size 23 cm with whitish discharge on the right little finger and of 0.5 cm on the left ear lobule [ figure 1 ] . the serum uric acid level was 13.9 mg / dl , and serum creatinine 1.4 mg / dl ( normal uric acid level in > 18 years old : 6.2 0.8 mg / dl ) . there were multiple family members affected with the same disorder ; some had only gouty arthropathy and others had both gouty arthropathy and renal disease [ tables 1 and 2 ] . his father had died at the age of 42 years due to chronic kidney disease and had gouty arthritis . one of his elder brothers and one paternal uncle 's son has gout along with kidney disease , and one brother has only gouty arthritis [ figure 2 ] . proband showing a nodule on left ear lobe and another on right little finger with whitish discharge clinical features of proband and his family members investigations of the proband and his brother family pedigree showing multiple affected members informed consent was obtained from the three affected individuals for deoxyribonucleic acid analysis , collection of clinical data , and publication of photographs . the 10 coding exons of umod gene were amplified by polymerase chain reaction and cycle sequenced by capillary electrophoresis using an abi 310 sequencer ( applied biosystems foster city , ca , usa ) . a heterozygous missense variant ( c. 949 t > g ) in exon 5 [ figure 3 ] was identified in the proband and two affected brothers causing substitution of cysteine by glycine at codon position 317 ( p.c317 g ) . it is a novel variant , not reported in 1000 genomes , clinvar , human genome mutation database , and human genome variation . the affected residue locates within the cysteine - rich 2 domain and is immediately preceded by a highly conserved cysteine residue . various bioinformatics tools such as mutation taster , polyphen and sorting intolerant from tolerant also predicted it to be pathogenic . based on the above evidence , we concluded that this novel variant is very likely the causal pathogenic mutation . g in exon 5 of uromodulin gene causing substitution of cysteine by glycine at position 317 umod also called as tamm - horsfall protein is a polymeric protein located on the tubular cells lining the thick ascending limb of the loop of henle and early distal convoluted tubules . from the apical membrane , it is secreted into the tubular lumen where it polymerizes into a water impermeable gel - like structure that modulates salt transport , urine concentration , and urate metabolism . common genetic variants in umod gene have been found to be associated with hypertension , reduced renal function , and increased risk of chronic renal failure . other disorders caused by mutation in umod are medullary cystic kidney disease type 2 ( mim 603860 ) and glomerulocystic kidney disease ( mim 609886 ) . until date , more than 70 mutations have been described in umod and most of them are clustered in exon 4 ( 83.8% ) , 5 ( 8% ) , and 8 ( 8% ) of umod gene . two - thirds of these mutations cause substitution of cysteine residues or highly conserved polar amino acids . in the present family , the mutation c. 949 t > substitution of cysteine residue can alter the disulfide bond formation that interferes with correct folding of the umod protein . the misfolded protein accumulates in the endoplasmic reticulum of tubular cells that elicits an adaptive response called as unfolded protein response causing cell death . have shown that substitution of cysteine by tyrosine ( cys317tyr ) at the same position leads to delay in export of umod to plasma membrane due to longer retention time in endoplasmic reticulum . there is marked interfamilial and intrafamilial variability that was also seen in the present family . hyperuricemia is present in 83.3% of the cases and gout in 45% of mutation positive cases . median age of onset of end - stage renal disease ( esrd ) was earlier in cases with mutation in epidermal growth factor ( egf)-2 and egf3 domains and later in cases with mutation in domain of 8 cysteine ( d8c ) and cysteine rich regions 1 and 2 . however , there is no correlation between the presence of hyperuricemia or gout and development of esrd . use of uricosuric drugs such as allopurinol has a definite role in decreasing hyperuricemia and features related to gout . however , the efficacy of these medications in slowing the progression of renal disease is still not clear .
familial juvenile hyperuricemic nephropathy ( fjhn ) , characterized by early - onset hyperuricemia , reduced fractional excretion of uric acid , and chronic renal failure is caused due to mutation in uromodulin ( umod ) gene . we identified a novel mutation in a family with multiple members affected with fjhn . ten coding exons of umod gene in three family members with clinical and biochemical features of fjhn and one unaffected family member were sequenced , and sequence variants were analyzed for the pathogenicity by bioinformatics studies . a heterozygous novel missense mutation ( c. 949 t > g ) in exon 5 leading to the replacement of cysteine by glycine at position 317 was identified in all three affected family members . this mutation has not been reported earlier in human gene mutation database , human genome variation , clinvar , and 1000 genome . the mutation lies in the cysteine - rich 2 domain of the protein , and the affected residue is evolutionary conserved in other species . to our knowledge , this is the first report of the identification of umod mutation in an indian family .
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a biotage 5 ml microwave vial containing a stirring bar was charged with [ rh(acac)(co2 ) ] ( 2 mol % , 4.3 mg , 0.0166 mmol ) , ( r , r)-ph - bpe ( 3 mol % , 12.6 mg , 0.0249 mmol ) and paraformaldehyde ( 6 equiv , 150 mg ) . the vial was sealed with a crimp cap , purged with three vacuum / argon cycles and left under an argon atmosphere . alkene ( 150 mg , 0.832 mmol ) , toluene ( 3 ml ) and an internal standard ( 1 drop of cyclooctane ) were added to a schlenk flask under an inert atmosphere . the resulting solution was mixed , and a small sample was taken for a t0 nmr analysis . the solution was then added to the microwave vial and heated to 120 c using microwave radiation . after 45 min , the vial was cooled , and the positive pressure inside the vial was released by piercing the cap with a needle . a small sample was taken and analysed by h nmr spectroscopy to calculate the conversion to the resulting aldehyde . the aldehyde was reduced using nabh4 to the corresponding alcohol ( see the supporting information ) and isolated using flash chromatography on silica gel ( n - hexane / etoac 4:1 ) to give 2,3-diphenylpropan-1-ol as a white solid ( 126 mg , 0.60 mmol ) . the enantiomeric excess of the alcohol was determined by chiral hplc on a chiralcel od - h , 2504.6 mm , n - hexane/2-propanol 90:10 , 0.5 ml min , 254 nm , tr[()-(r ) , major]=18.3 min , tr[(+)-(s ) , minor]=20.5 min ; [ ]d 85.5 ( c 1.0 , chcl3 , ee 95 % ; lit : [ ]d 80.7 ( c 1.13 , chcl3 ) , ee 93 % ) see the supporting information for nmr data . caution ! ! ! this procedure gives enhanced convenience and requires far less complicated safety measures than conventional ahf , because the syngas is added to the reactor in solid form . using the amounts above , the highest pressure detected during an athf using 6 equiv of [ ch2o]n was 7 bar at reaction temperature ( 4 bar at rt ) . the highest pressure detected at 120 c when no athf was taking place , but decarbonylation did was 13 bar . in the 50 ml glass or steel pressure vessels , the highest pressure detected was 3 bar . similar to the majority of chemical reactions with volatile components , scale - up of these procedures needs careful consideration of the pressures possible and the maximum operating pressure of the vessel used . 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
as an alternative to conventional asymmetric hydroformylation ( ahf ) , asymmetric transfer hydroformylation ( athf ) by using formaldehyde as a surrogate for syngas is reported . a catalyst derived from commercially available [ rh(acac)(co)2 ] ( acac = acetylacetonate ) and 1,2-bis[(2s,5s)-2,5-diphenylphospholano]ethane(1,5-cyclooctadiene ) ( ph - bpe ) stands out in terms of both activity and enantioselectivity . remarkably , not only are high selectivities achievable , the reactions are very simple to perform , and higher enantioselectivity ( up to 96 % ee ) and/or turnover frequencies than those achievable by using the same catalyst ( or other leading catalysts ) can be obtained by using typical conditions for ahf .
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as the most common form of dementia , alzheimer disease is characterized by progressive loss of memory and deterioration of cognitive functions . it is predicted that about 75.63 million people would suffer from dementia by 2030 . accordingly , in the present study , the intended remedy was selected and an appropriate pharmacognostical and pharmaceutical evaluations were performed . by searching through the traditional pharmaceutical manuscripts such as qarabadeen - e - salehi , qarabadeen - e - azam , qarabadeen - e - ghaderi and canon of medicine , a simple but proven compound remedy ( frankincense and black pepper ) was selected . related pharmaceutical assessments such as weight variation , hardness , friability , and disintegration tests as well as pharmacognostical evaluations such as microscopic characterization , tlc , gc / ms , ft / ir fingerprints , and radical scavenging activity assessment ( dpph ) were performed . the resulting formulation , as a floating tablet , included 60% of frankincense gum and 15% of black pepper along with appropriate pharmaceutical ingredients ( weight variation : 0.2190.004 g , hardness : 6.500.67 , friability : 0.45% , disintegration time > 30 min ) . microscopic characterization demonstrated stone cells , calcium oxalate crystals , sclereids of endocarp and pitted cells of mesocarp of pepper fruits as well as oil drops of frankincense gum . gc / ms analysis revealed acetyl acetate and trans - caryophyllene as the main constituent . moderate radical scavenging activity ( ic50 > 100 g / ml ) was calculated for the methanol extract of tablets . carrying out and validating a gc method for standardization of the formulated tablet , and having the structure for the effectiveness of these medicinal herbs in alzheimer may be the horizon for a new alzheimer - targeted medicine .
background : as the most common form of dementia , alzheimer disease is characterized by progressive loss of memory and deterioration of cognitive functions . it is predicted that about 75.63 million people would suffer from dementia by 2030 . apart from conventional remedies , the application of herbal medicines is on the rise . there are numerous natural medicaments reported in the traditional manuscript of persian medicine . accordingly , in the present study , the intended remedy was selected and an appropriate pharmacognostical and pharmaceutical evaluations were performed.methods:by searching through the traditional pharmaceutical manuscripts such as qarabadeen - e - salehi , qarabadeen - e - azam , qarabadeen - e - ghaderi and canon of medicine , a simple but proven compound remedy ( frankincense and black pepper ) was selected . a floating tablet was designed and formulated from those herbal components . related pharmaceutical assessments such as weight variation , hardness , friability , and disintegration tests as well as pharmacognostical evaluations such as microscopic characterization , tlc , gc / ms , ft / ir fingerprints , and radical scavenging activity assessment ( dpph ) were performed.results:the resulting formulation , as a floating tablet , included 60% of frankincense gum and 15% of black pepper along with appropriate pharmaceutical ingredients ( weight variation : 0.2190.004 g , hardness : 6.500.67 , friability : 0.45% , disintegration time > 30 min ) . microscopic characterization demonstrated stone cells , calcium oxalate crystals , sclereids of endocarp and pitted cells of mesocarp of pepper fruits as well as oil drops of frankincense gum . tlc fingerprinting showed classes of secondary metabolites related to both components . gc / ms analysis revealed acetyl acetate and trans - caryophyllene as the main constituent . moderate radical scavenging activity ( ic50 > 100 g / ml ) was calculated for the methanol extract of tablets.conclusion:carrying out and validating a gc method for standardization of the formulated tablet , and having the structure for the effectiveness of these medicinal herbs in alzheimer may be the horizon for a new alzheimer - targeted medicine .
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minor bladder shift plus inguinal hernia is a relatively rare condition that occurs in only 13% of hernias and up to 10% of men over 50 years of age . . only 22 cases of neoplasm arising within a vesical hernia are cited in published reports . we present a case of a papillary urothelial bladder carcinoma in a direct inguinal hernia . a 79-year - old man presented to our department with a swelling in the left groin evolving over 10 years without any symptoms . the patient was operated using the transurethral resection of bladder tumor ( tur - bt ) technique 3 months before due to papillary urothelial carcinoma of the prostatic part of the urethra . the ultrasound examination was inconclusive showing only a left inguinal hernia while the intravenous pyelography revealed an abnormal position of the urinary bladder . 1 ) showed a left groin hernia with an irregular mass content which grows from the urinary bladder ( probably tumor of the bladder wall ) . the patient underwent open surgery via an inguinal approach ; the tumor was palpated in the herniated portion of the bladder ( fig . a partial cystectomy was done and the inguinal hernia was repaired without the use of mesh . pathologic examination of the specimen reported a tumor of 8 cm 5 cm 5 cm ( fig . 4 ) revealed a high - grade carcinoma with necrosis and characteristics of a papillary urothelial carcinoma with invasion of 1/3 of the muscularis propria ( t2a ) . one year after the operation the patient presented no recurrence of the tumor or hernia . some researchers have estimated the prevalence may be closer to 10% in men . according to the relationship with the peritoneum , the hernias are classified as follows : ( a ) paraperitoneal , which are common ( 60% ) in which the peritoneum covers the external aspect of the herniated bladder , can be direct or indirect , as it was in our case ; ( b ) extraperitoneal , in which the tumors do not cover the peritoneum and are usually small ; and intraperitoneal , in which the peritoneum covers the entire portion of herniated bladder . the most common etiologies of bladder hernias are : obesity , weakness of the pelvic wall , bladder outlet obstruction , and decreased tone of the bladder . in our case the patient had a history of hernia repair 53 years ago , however , whether a history of herniorrhaphy affects the occurrence of bladder hernia is uncertain . bladder hernias are usually asymptomatic but are often associated with intermittent swelling in the groin and significant lower urinary tract symptoms . in cases of large hernias the patients typically present with two - stage micturition , involving spontaneous bladder emptying with a second stage manual compression of the hernia . the differential diagnosis includes : bladder diverticulum , hernia of a mesenteric cyst , hydrocele and spermatic cord cyst . the radiological diagnosis can be established by cystography , ultrasonography , and intravenous pyelography or computed tomography . the numbers of cases of hernia which contain a tumor of the urinary bladder being published are low ( table 1 ) . the purpose of treatment is to remove the tumor , repair the hernia and correct the obstructive condition of any lower urinary tract . the patient survival is low due to the delay and difficulty in obtaining an accurate diagnosis . despite the overall poor prognosis inguinal bladder hernias are relatively uncommon , with few ( 22 ) reports of tumor in the herniated bladder have been published in the international literature ( medline / pubmed ) . the surgical management consists of removing the herniated bladder tissue containing the tumor with a marginal of safety , and repairing the hernia , with careful urological follow up . written informed consent was obtained from the patient of the publication of this case report and accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal of request . anastasios katsourakis participated in the design of the study and drafted the manuscript ; george noussios participated in the coordination and helped to draft the manuscript ; christos svoronos performed the literature review ; michael alatsakis helped to draft the manuscript ; efthimios chatzitheoklitos participated in the coordination .
introductioninguinal hernia containing bladder carcinoma is a very rare occurrence.presentation of casewe report a case of a male patient who presented with a left groin hernia containing an irregular mass . the hernia was repaired without the use of mesh and a partial cystectomy was done.discussiononly 13% of all inguinal hernias involve the bladder , with very few reports containing a carcinoma.conclusiontreatment consists of removing the tumor and repairing the hernia .
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intussusception is not a rare disease in children , however in adults it accounts for only 1% of bowel obstructions , and 5% of all intussusceptions . we herein report a preoperatively diagnosed case of adult intussusception caused by a small bowel lipoma . a 33-year - old man was admitted to our hospital with three weeks history of colicky epigastric pain . before admission the patient had been hospitalized at another hospital , but the cause of the repeated colicky epigastric pain had not been identified . the patient had no significant past medical history of illnesses nor any hospitalization otherwise . on physical examination , the abdomen was flat and no tumor was palpable . 1 ) . colonoscopy revealed ileocolic intussusception , but no obvious cause was identified ( fig . barium enema for reduction of the ileocolic intussusception showed a small bowel tumor in the ileum 15 cm proximal to the ileocecal valve ( fig . reduction of the intussusception under barium enema was succeesful , and the patient underwent laparotomy the next day . however , recurrent ileocolic intussusception was found , for which a manual reduction and a partial resection of the ileum encompassing the small bowel tumor in the ileum 15 cm proximal to the ileocecal valve was performed . the resected specimen showed a soft and yellowish - white submucosal tumor with a diameter of 40 25 mm . histological findings revealed fat cells proliferating in the submucosal layer and confirmed the diagnosis of lipoma of the small bowel . adult intussusception is rare and usually caused by a tumor acting as the apex of the intussusception . therefore , such a condition should indicate the possibility of the presence of malignancy in the bowel . however , it has been reported that 52 - 80% of cases of adult small bowel intussusception are caused by benign entities , such as lipoma , hamartomatous polyp , inflammatory polyp , hyperplastic polyp , meckel 's diverticulum or surgery - related lesions . because benign tumors of the small bowel are commonly polypoid - shaped , the intussusception usually develops before the tumor has grown to a size sufficient to obstruct the passage of the small intestinal contents . small intestinal tumors are rare , accounting for 1 - 2% of all gastrointestinal tract tumors . moreover , preoperative diagnosis is often difficult because of the lack of specific clinical symptoms and difficulty with the examination of the small intestine . computed tomography and ultrasonography of the abdomen . however , further examinations such as barium enema or endoscopic ultrasonography are useful in cases of difficult diagnosis . it is accepted that gentle preoperative or operative reduction can be attempted safely to avoid unnecessary operation . laparoscopic surgery for intussusception is increasing , and successful reduction of intussusception recently has been reported . although more technically difficult than laparotomy , with an increased risk of perforation and dissemination and metastasis in cases of malignancies or with intestinal dilatation , laparoscopic surgery seems to become a procedure of choice for intussusception caused by benign intestinal tumors in the future .
adult intussusception is rare , accounting for only 5% of all intussusceptions , for which preoperative diagnosis is difficult . we herein report a preoperatively diagnosed case of adult intussusception caused by a small bowel lipoma . a 33-year - old man was admitted to our hospital with three weeks history of colicky epigastric pain . computed tomography revealed thickening of the ileal wall suggestive of intussusception . colonoscopy revealed an ileocolic intussusception . barium enema for reduction of ileocolic intussusception demonstrated a small bowel tumor in the ileum 15 cm proximal to the ileocecal valve . the intussusception was reduced , and the patient underwent partial resection of the ileum encompassing the small bowel tumor . histological findings confirmed the diagnosis of lipoma of the small bowel . the patient made a satisfactory recovery and remains well .
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reticulate acropigmentation of kitamura ( rak ) and dowling - degos disease ( ddd ) are part of a spectrum of rare autosomal dominant genodermatosis , characterized by progressive , symmetric and asymptomatic reticulated pigmented macules affecting the dorsa of the hands , in the former , and the flexures , in the latter . hyperpigmented lesions in rak may also involve the flexor aspects of the wrists , neck , eyelids and periorbital areas . other features include palmoplantar pits , breaks in the epidermal ridge pattern and occasionally , plantar keratoderma and alopecia . rak 's onset is usually in childhood unlike ddd , where the reticulate pigmentation has a late onset , in early adult life . in addition , ddd also present comedo - like lesions and pitted acneiform scars in the face , without palmoplantar pitting . it is very often associated with epidermoid cysts , keratoacanthoma , squamous cells carcinoma , abscess , suppurative hidrosadenitis , seborrheic keratosis and pilonidalis cysts . the histopathology of the hyperpigmented lesions is similar and characteristic in rak and ddd , with the presence of digitated and filiform elongated rete ridges , with clumps of heavy melanin pigmentation at their tips , thinning of the epidermis and pseudo - cysts . this resembles the appearance of a solar lentigo , but with epidermal atrophy , melanin incontinence and perivascular lymphocytic infiltrate . accordingly to the similarity in clinical and histological features , many authors consider rak and ddd different phenotypes of a single disorder . in literature the authors found only few cases of overlap rak - ddd ( about a dozen ) , including family cases described until four generations . an otherwise healthy 45-year - old female presented since early childhood , with a progressive and asymptomatic reticulated acropigmentation with brown pigmented macules on the dorsa of the hands , forearms and feet ( figures 1 , 2 ) . hyperpigmented macules , ephelide - like , on both axillae , buttocks and perioral area , also appeared in adulthood ( figure 3 ) . the physical examination revealed as well palmar pits ( figure 4 ) but no facial pitted scars , breaks in dermatoglyphics , comedo - like papules or alterations in mucous membranes , hair , teeth and nails . hyperpigmented macules on the dorse of the feet . figure 3hyperpigmented , ephelide - like macules around the mouth . the patient reported similar lesions ( mainly the reticulated acropigmentation ) on two relatives ( mother and aunt ) apparently in an autosomal dominant pattern . the skin biopsy of a pigmented macule of the back of the hand revealed elongated rete ridges , with increased pigmentation of the basal layer and an increment in the number of melanocytes , characteristic of rak ( figure 5 ) . histopathology of an hyperpigmented lesion of the dorso of the hand . based on the history , clinical and pathologic findings , the authors believe that this case is one more example of the rare event of overlap rak - ddd , among the few non - asian cases described in literature . rebora and crovato first suggested in 1983 that the two entities , rak and ddd were different phenotypic expressions of the same genodermatosis . in similarity to the other rak - ddd overlap cases described in the literature , our patient had typical characteristics of both variants . concerning rak , it was possible to perceive the reticulated hyperpigmentation with acral distribution and palmar pits and ; in addition , the hyperpigmented lesions on the flexures were typical of ddd . the true knowledge of the relationship between rak and ddd will be possibly achieved , through the clarification of the genetic background of both diseases .
reticulate acropigmentation of kitamura ( rak ) and dowling - degos disease ( ddd ) are rare genodermatosis inherited as an autosomal dominant trait with variable penetrance . they are part of a spectrum of diseases with hyperpigmented macules coalescing in a reticular pattern , facial and palmoplantar pits , breaks in dermatoglyphics , comedo - like lesions and epidermoid cysts , and a unique histological picture of hyperpigmented digitate epidermal downgrowths . the authors describe the case of a 45-year - old female with reticulate acropigmentation of the dorsa of the hands and feet , hyperpigmented macules on the axilla and around the mouth , and palmar pitting . clinical and histological findings , together with a relevant family history , allowed the authors to consider this case an example of the rare event of an overlap rak - ddd .
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a medline search revealed a report of olanzapine - induced hyponatremia , and three such cases have been reported at a dutch pharmacovigilance centre . hyponatremia has also been reported with other typical and atypical antipsychotics . a systemic review by meulendijks et al . included four studies and 91 publications containing case reports and case series of antipsychotic - induced hyponatremia . they found that the number of case reports of hyponatremia involving typical and atypical antipsychotics was 58 and 10 respectively , from 1974 to 2003 . they also concluded that antipsychotic - induced hyponatremia did not seem to be associated with age or gender and was not dose dependent . a 63-year - old hindu male , smoker and a known case of prostate enlargement was diagnosed with recurrent depressive disorder , for which he was on escitalopram 5 mg / day for last two years . around two months back , he complained of unsatisfactory night sleep , feeling low , and experiencing referential ideas with elementary auditory hallucinations . the current exacerbation was categorized as severe depression with psychotic symptoms as per icd-10 ( dcr ) . the dose of escitalopram was increased to 10 mg / day and olanzapine 5 mg / day was added . after two weeks , his sleep pattern was restored , symptoms abated and patient was better for about a month . he then developed symptoms of mild anorexia , nausea , mild weakness , and occasional muscle cramps . after 10 days , abrupt exacerbation of excessive weakness , lethargy , muscle cramps , unsteady gait , fleeting disorientation , and urinary retention developed , and the patient was admitted . his physical examination revealed mild pallor , tachycardia , normal blood pressure , moderate dehydration , and disorientation of time and place . his respiratory system and abdominal examination was unremarkable and his glasgow coma scale was 11 . his blood investigations revealed serum sodium was 118 mmol / l , serum potassium was 3.5 mmol / l , and total leucocyte count was 12,100/cmm with 78% neutrophils . thyroid - stimulating hormone , hemoglobin , albumin , and bicarbonate levels ; liver and renal function ; and lipid profile were normal . he was treated with oral fluid restriction , stopping olanzapine and starting antibiotics . in view of lack of signs and symptoms of fluid overload , it was considered as probable case of normovolemic hyponatremia and 3% nacl was used initially as the correcting fluid . mmol / l / day . after 4 hours , the patient became alert and regained his sensorium . later , correction was done using free water restriction , normal saline , and oral salt supplement , and by serially measuring blood na regularly every 6 hours . his symptoms and general condition improved over the next two days and serum sodium level reached 138 mmol / l . patient was discharged on third day and he was told to continue escitalopram 10 mg . olanzapine or any other antipsychotic was not restarted as his mental status examination did not revealing any psychotic psychopathology at that point of time . the patient and guardians were educated about the risk of hyponatremia , importance of diet and fluid intake habits , and early warning signs , and about quitting smoking . on subsequent three follow ups , he was maintaining well , and his serum electrolyte estimation was within normal range . a possible causal relation between the drug and adverse event was established by the who - umc scale ( who ) and naranjo algorithm . written informed consent has been obtained from the patient for publication of this case report . the exact estimate about incidence of hyponatremia induced by antipsychotics is currently not available but many antipsychotics like chlorpromazine , fluphenazine , haloperidol , flupenthixol , trifluoperazine , thioridazine , amisulpride , and risperidone have been implicated . it has been suggested that the inhibitory effect of dopamine on release of anti - diuretic hormone ( adh ) is blocked by d2 receptor antagonism . this may be the possible mechanism for the causation of hyponatremia by all d2 receptor antagonists including olanzapine . the exception is clozapine , which has been found to have a beneficial effect on polydipsic behavior and development of hyponatremia , which may be attributed to its lower binding affinity to d2 receptors . other contributory factors like old age , diet , salt intake , smoking , original psychopathology like psychogenic polydipsia , diabetes , other comorbid conditions , and side effects of antipsychotics or concurrent medications such as dryness of mouth may also play a role . in this case , the temporal relationship suggests olanzapine as the causative molecule , but it is difficult to pinpoint the offending medication . all serotonin reuptake inhibitors including escitalopram are also known for causing hyponatremia , and the patient was on escitalopram for two years without any problems . with the worsening of the disease , the dose of escitalopram was increased and at the same time , olanzapine was also initiated . hence , the causality may be attributed to the combined effect to both of these drugs . however , escitalopram was restarted with no further episodes of hyponatremia suggesting a stronger possibility of causal relation with olanzapine . there may be involvement of other possible non - pharmacological factors like water and salt intake pattern and some infection as evidenced by leucocytosis . olanzapine may be responsible for hyponatremia in vulnerable people , which may be the product of a combination of factors . emphasis should also be laid on the education of patients and their family members regarding early identification of hyponatremia .
here we report a case of a 63-year - old male diagnosed with recurrent depressive disorder and current episode of severe depression with psychotic symptoms , developed hyponatremia soon after addition of olanzapine and increasing the dose of escitalopram . a possible causality association was established with olanzapine , and the possible etiological reasons of this clinically significant risk were discussed .
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cutaneous complications caused by human immunodeficiency virus ( hiv)/acquired immune deficiency syndrome have been reported , including , all kinds of opportunistic infections , psoriasis , seborrheic dermatitis , hairy leucoplakia , and granuloma annulare , among others . alopecia areata and vitiligo are generally frequent in patients , and their solitary associations with hiv are probably fortuitous in most cases . in the current paper , we report a case of an hiv - infected patient with these conditions . two years ago , several dispersed hair - loss patches appeared on the patient 's scalp without any subjective symptom . he previously visited a dermatologist and was diagnosed with alopecia areata as well as vitiligo . no member of his family had vitiligo or alopecia . dermatological examination revealed several depigmented macules measuring 2 cm to 5 cm with clear boundaries distributed on the face and neck . several smooth hair - loss patches measuring 1 - 2 cm were also distributed on the top of the head , and diffuse alopecia was observed around the head [ figure 1a , b , and c ] . ( a ) hair loss at his first visit to the clinic ; ( b ) and ( c ) status of vitiligo at his first visit ; ( d ) hair loss at his second visit a month later enzyme - linked immunosorbent assay and western blot revealed a positive result for serum anti - hiv antibody . flow cytometry revealed that the patient 's cd4 lymphocyte count and cd4/cd8 ratio were 20 cells/l ( 3.7% ) and 0.04 , respectively , which are considerably low . the thyrotropic - stimulating hormone and free thyroxine levels were 7.69 iu / ml and 10.06 pmol / l , respectively . serological test of syphilis showed a positive t. pallidum particle agglutination , but the toluidine red unheated serum test was negative . the patient did not accept any therapy for 1 month until his second visit to the clinic . his alopecia areata worsened , presented ophiasis pattern with 80% hair loss on his head , and even his brows [ figure 1d ] . the patient 's general state of health was still good , and manifestations of opportunistic infection and malignant tumor were not observed . one month after antiretroviral therapy ( art ) , the patient 's alopecia areata dramatically improved , but no evident improvement in his vitiligo was found [ figure 2 ] . his cd8 lymphocyte count had a more considerable increase , resulting in a cd4/cd8 ratio of 0.03 . although , his cd4 lymphocyte count was still very low , no other complaints were reported . the patient received benzathine penicillin injection for 3 times , but his vitiligo was not still improved . ( b ) status of alopecia areata dramatically improved ; ( c ) and ( d ) vitiligo did not have obvious improvements alopecia areata is one of the most common autoimmune diseases that can be considered a t - cell - mediated autoimmune disease , whereby the gradual loss of protection provided by the immunity of normal hair follicle plays an important role . alopecia areata has been shown as a cd8 + t - cell - dependent and organ - specific autoimmune disease in a rat model of alopecia areata , in which depleting cd8 + t - cells could restore hair growth . vitiligo is a common depigmentation disease characterized by the presence of circumscribed white macules in the skin caused by the destruction of melanocytes in the epidermis . skin biopsies of vitiligo patients show that inflammatory cells are prominent in the perilesional areas , consisting of cd8 + and cd4 + t - cells , often with an increased cd8+/cd4 + ratio . although , syphilis infection may also cause alopecia areata or vitiligo like lessions , the laboratory studies in this case only confirmed that the patient had been infected with syphilis . rawson et al . suggested an autoimmune mechanism that involves the release of protein fragments from dying cd4 + t - cells that , in turn , promote the formation of auto - reactive cd8 + t - cells in hiv infection . the massive level of death and destruction of lymphocytes in hiv infection breaks the tolerance for self - peptides and leads to the production of auto - reactive cytotoxic t - cells that respond to the cleavage products of apoptotic cells . this mechanism explains the successive occurrence of alopecia areata and vitiligo in the same patient with hiv . although , the patient 's cd4 lymphocyte count was only 20 cells/l , he had no other complaints , except for alopecia areata and vitiligo . after art , his cd4 lymphocyte count increased , simultaneously accompanied by improvement in his alopecia areata without anti - alopecia treatment . hence , the improvement of alopecia areata and increased cd4 lymphocyte count are positively correlated . the patient represents a very rare case of alopecia areata and vitiligo associated with hiv infection . self - reactive cd8 + t - cells are generated because the release of protein fragments from dying cd4 + t - cells breaks the tolerance for hidden antigens during progressive immune exhaustion . this mechanism explains the occurrence of some autoimmune diseases , including , alopecia areata and vitiligo , with hiv infection . written informed consent was obtained from the patient for the publication of this case report and any accompanying images . alopecia areata and vitiligo associated with hiv infection might be connected with the generation and maintenance of self - reactive cd8 + t - cells .
a 26-year - old chinese male consulted with the team regarding his alopecia areata and vitiligo for which previous treatment was ineffective . the patient , a homosexual man , denied having a history of drug abuse and of blood transfusion . no member of his family had vitiligo or alopecia . laboratory studies revealed that the serum for anti - human immunodeficiency virus ( hiv ) antibody was positive . the patient 's cd4 lymphocyte count and cd4/cd8 ratio were both strikingly low ( 20 cells / ml and 0.04 ) , but no other complaints or opportunistic infections were reported . one month after antiretroviral therapy , the patient 's alopecia areata dramatically improved , but no evident improvement in his vitiligo was found . this case is a very rare case of alopecia areata and vitiligo associated with hiv infection that might be attributed to the generation and maintenance of self - reactive cd8 + t - cells due to chronic immune activation with progressive immune exhaustion in hiv infection .
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current guidelines recommend implantable cardioverter defibrillator ( icd ) implantation for brugada syndrome ( brs ) patients with a history of ventricular fibrillation ( vf ) . however ; patients whose first presentation is vf are at high risk of recurrent vf episodes and/or electrical storm . here we report a case of brs whose first presentation was electrical storm , for which , primary ablation ( i.e. before icd implantation ) resulted in vf - free survival during a 48-month follow up . after resuscitation , he was admitted to a nearby hospital , where brugada - type electrocardiographic readings ( ecgs ) were recorded . these findings were associated with frequent monomorphic premature ventricular contractions ( pvcs ) that degenerated to ventricular fibrillation ( vf ) 3 times during the night ( fig . 1 ) . telemetry revealed that the qrs morphology of the first beat in all vf episodes was identical ( left bundle branch block and inferior axis configuration ) . the patient was then transferred to our hospital , where he was stable , with repetitive pvcs ( > 10 pvcs / min ) of the same initial morphology . because these pvcs had led to an electrical storm and continued with similar frequency the next day , emergency catheter ablation ( ca ) was performed 4 hours after admission . 2 ) with late potentials ( lp ) , scattered at the free wall of the rv outflow tract ( rvot ) with only few lp points at its posterior aspect . an optimal pacemap and early activation were observed at the rvot free wall , where ca completely eliminated the pvcs and rendered vf non - inducible . lp ablation and further energy applications in a broad area around the earliest activation site were performed . pilsicainide provocation and treadmill exercise tests were performed ; however , no triggering pvcs or vf occurred . the patient has been event free during a 48-month medication - free follow - up . in brugada syndrome ( brs ) , icd implantation is recommended for patients experiencing vf . however , up to 48% of such high - risk patients experience frequent icd shocks that result in significant sequelae such as depression , post - traumatic stress , and difficult vf termination due to a relatively high defibrillation threshold in some patients . pharmacologic therapy , typically an isoproterenol infusion , can be effective in suppressing vf , and deep sedation has occasionally yielded good results in isolated cases . however , because the patient was relatively stable on admission , suppressing his pvc / vf would have resulted in missing his culprit pvc because trigger pvcs are episodic , appear just before vf , and disappear within a narrow time window . local ablation of vf - triggering pvcs after icd implantation has proven effective as adjuvant therapy in a few patients with electrical storm ; however , such patients had already been exposed to the icd discharge sequelae . this is the first case to demonstrate the feasibility of ablation and implantation as an alternative approach in suppressing es during long - term follow - up . unlike the culprit lesion in myocardial infarction , the culprit pvc of vf in brs can not be targeted once the vf subsides , because brs patients rarely have frequent pvcs that can be mapped or have pvcs during holter monitoring . for this reason , this case suggests that careful monitoring and possible ablation of vf - triggering pvcs in the acute phase may be clinically significant in selected patients . because epicardial mapping was not performed in this case , we could not identify whether the lp origin was epicardial or endocardial . however , endocardial ablation could have an epicardial modification effect because of the thin - walled rvot . while this approach is not first - line therapy for brs , by taking advantage of reducing the icd burden in the modern era of new catheter designs and mapping technologies , our approach might be effective in selected cases , especially when the origin of the culprit pvc is easily accessible , such as the rvot .
placement of an implantable cardioverter defibrillator ( icd ) is the only powerful treatment modality for brugada syndrome in patients presenting with ventricular fibrillation ( vf ) . for those whose first presentation is an electrical storm , pharmacologic therapy is typically used to control vf followed by icd implantation . we report an alternative approach whereby , before icd implantation , emergency catheter ablation of the vf - triggering premature ventricular contraction ( pvc ) resulted in long - term vf - free survival . the results suggest that , because vf triggers appear in a narrow time window , ablation of the culprit pvcs that initiate vf before the index pvcs subside is a reasonable alternative approach .
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a diaphragmatic hernia presenting in the postoperative period is a rare complication of transhiatal oesophageal surgery with an incidence of 0.4 - 2% . it can be easily confused with pulmonary complications like aspiration , atelectasis , consolidation , pneumonia and anastomotic leak causing sepsis which are the leading cause for morbidity . our aim is to highlight the possibility of this complication in the presence of cardio - respiratory compromise . this will help in a prompt diagnosis and swift surgical intervention to get the best outcome . a 65 year old male diabetic patient underwent transhiatal oesophagectomy and gastric resection for adenocarcinoma of the gastro - oesophageal junction . on the 10 postoperative day , he developed progressive breathlessness which became severe by the next day . chest x - ray as in figure 1 revealed large bowel loops in the left hemithorax leading to a diagnosis of diaphragmatic hernia . chest x - ray showing herniated colon in the left hemithorax clinical examination revealed that the patient had severe respiratory distress with inability to lie down , nasal flaring , active accessory muscles , sweating and dilated neck veins . the pulse was thready with a rate of 180/min and blood pressure of 90/60 mmhg . he maintained oxygen saturation of 85 - 90% with supplemental oxygen of 6 l / min . thus , noradrenaline infusion was initiated at 0.05 - 0.1 g / kg / min . an immediate and dramatic improvement in the haemodynamic parameters and resolution of high airway pressures was observed following reduction of the herniated colon . postoperatively , the patient was transferred to the intensive care unit for ventilatory support in view of severe mixed acidosis . diaphragmatic hernia following transhiatal oesophagectomy is an uncommon but potentially life - threatening complication , especially , if the diagnosis is delayed . it can present in the early postoperative period or many years later . extended incision , lateral incision and partial resection of the diaphragm it is more common on the left side and the colon is most likely to herniate . once in the thoracic cavity , the hernia contents stay there due to transdiaphragmatic pressure gradient . it can be asymptomatic or present with abdominal pain , intestinal obstruction and cardio - respiratory distress . the large volume of the oedematous herniated colon caused an exponential rise in the intrathoracic pressure . this led to the collapse of the underlying lung , resulting in ventilation perfusion mismatch and hypoxaemia . the continued rise in the intrathoracic pressure led to an extrinsic compression of the heart . cardiac tamponade results in impaired venous return , increased intracardiac pressure and decreased cardiac output . it has a cascading effect on the patient 's condition which is likely to progress to shock , cardiovascular collapse and death . an early x - ray chest would have helped in early diagnosis and decreased morbidity . practicing defensive surgical measures like routine narrowing of hiatus , making an anterior incision in the diaphragm rather than lateral and checking diaphragmatic integrity at the end of surgery minimize the chances of postoperative herniation of abdominal contents through the diaphragm . in conclusion , cardio - respiratory compromise following oesophagectomy requires exclusion of diaphragmatic hernia as an underlying cause .
postoperative diaphragmatic hernia following transhiatal oesophagectomy is a rare but potentially life threatening complication . we describe a case of a 65 year old patient who developed diaphragmatic hernia following oesophagectomy and presented with cardio pulmonary compromise . during surgery , haemodynamic instability continued despite fluid resuscitation and noradrenaline infusion . an immediate improvement in the haemodynamics and reduction in airway pressure occurred on reduction of the herniated colon from the thoracic cavity . this can be explained by tension colothorax causing collapse of the underlying lung and cardiac tamponade . it is a surgical emergency requiring urgent decompression for resuscitation . the etiology , clinical presentation , pathophysiology and preventive measures are discussed .
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gyrate atrophy of the choroid and retina is a rare chorioretinal dystrophy inherited in an autosomal recessive pattern . patients typically report night blindness and/or loss of peripheral vision , usually in the second decade of life . more than 200 individuals with gyrate atrophy have been reported since it was first described in the late 19th century , with cases mainly reported from finland , the us , japan , and france.1 although there have been anecdotal reports of gyrate atrophy cases in australia , we could not identify a case reported in the literature . thus , to our knowledge , we report the first documented case of gyrate atrophy from australia and document the clinical findings with ultrawide - field fundus photography and angiography . a 56-year - old woman who reported being previously diagnosed with retinitis pigmentosa and open - angle glaucoma 20 years prior was referred for ongoing worsening night vision and peripheral vision in her right eye over several years . she reported being myopic since childhood and had used latanoprost at night into both eyes over a long period . she had a past medical history of hypertension , depression , and elevated cholesterol . on examination , best - corrected visual acuity was hand movements in the right eye and 6/12 in the left eye with refractive errors of -1.75 in each eye . ten years previously she had developed bilateral posterior subcapsular cataracts and was now bilaterally pseudophakic with patent posterior capsulotomies . only small islands of retina at each posterior pole still appeared to be clinically normal ( figure 1 ) . these islands were sharply demarcated from the atrophic areas by a pigmented border ( figure 1 ) . visual field testing showed almost total constriction of the right visual field and also severe peripheral constriction of the left visual field ( figure 2 ) . extensive visual electrodiagnostic testing , including electroretinography , pattern electroretinography , multifocal electroretinography , electro - oculography , visual evoked potential , average visual evoked potential , and farnsworth munsell 100 ( all performed to international society for clinical electrophysiology of vision standards ) , showed virtually abolished responses in scotopic and photopic conditions , confirming an advanced retinal dystrophy . on further questioning , the patient reported a history of consanguinity , with her parents being first cousins thus potentiating an autosomal recessive inheritance and clinching the diagnosis of gyrate atrophy . clinically , gyrate atrophy appears as well - circumscribed areas of atrophy of the choroidal vessels , retinal pigment epithelium , and photoreceptors in the midperipheral retina.2 typically , scalloped atrophic areas are well demarcated from the posterior pole , which although it appears clinically normal usually also has areas of photoreceptor cell loss.3 gyrate atrophy has been associated with serum hyperornithinemia due to a deficiency of the vitamin b6-dependent enzyme ornithine ketoacid aminotransferase ( oat ) , and the human oat gene has been localized to chromosome 10.4 our case is typical of other gyrate atrophy cases in terms of retinal findings , myopia , early cataract formation , serum hyperornithinemia , and an autosomal recessive inheritance pattern . we have commenced treating the patient with a low - arginine diet and pyridoxine ( vitamin b6 ) , and plan to follow her up with serial visual field testing , serum ornithine levels , and retinal photography / angiography . although the efficacy of these treatments has been variable,5 we are hopeful that we will preserve the patient s remaining visual function . in the future , diseases like gyrate atrophy with known molecular and genetic foundations hopefully may become targets for enzyme replacement treatments or gene therapy .
gyrate atrophy of the choroid and retina is a rare chorioretinal dystrophy inherited in an autosomal recessive pattern . we describe the first documented case of gyrate atrophy from australia in a 56-year - old woman with a history of previous diagnosis of retinitis pigmentosa and worsening night vision in her right eye over several years . she was myopic and bilaterally pseudophakic , and fundus examination revealed pale optic discs and extensive peripheral chorioretinal atrophy exposing bare sclera bilaterally with only small islands of normal - appearing retina at each posterior pole . visual field testing showed grossly constricted fields , blood testing showed hyperornithinemia , and further questioning revealed consanguinity between the patient s parents . we then used the patient s typical retinal findings of gyrate atrophy to demonstrate the potential use of ultrawide - field fundus photography and angiography in diagnosis and monitoring response in future treatment .
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trauma to the eye and retrobulbar injections can often lead to retrobulbar hemorrhaging that can result in an increased pressure in the retrobulbar space and an occlusion of the central retinal artery ( crao).1,2 the intraorbital pressure can be relieved by surgical drainage of the hematoma.15 however , a medline search did not extract any papers on cases of a blow - out fracture complicated by a crao , although one case of a crao following a blow - out fracture that was not immediately repaired has been reported.6 to the best of our knowledge , this is the first case of a blow - out fracture with a crao even after an early repair of the fracture . a 20-year - old woman was involved in a traffic accident while riding her motorcycle . she most likely hit her head on the handlebar , but did not remember anything about the accident . she had a severe decrease of vision in her right eye and was referred to our department of ophthalmology . her best - corrected visual acuity was no light perception ( nlp ) in the right eye and 1.2 in the left . movements of her right eye could not be detected because the eyeball was depressed downward . computed tomography demonstrated that her right eye was not perforated but had shifted down into the maxillary sinus through a blow - out fracture of the lower orbital wall ( figure 1 ) . the intraocular pressure of right eye appeared to be moderately high by palpation , and slit - lamp examination revealed corneal edema , mydriasis , and hyphema . surgery was immediately performed , and the blow - out fracture was repaired with replacement of the eyeball to its proper position ( figure 2 , left ) . after the surgery , her right fundus was not visible due to a hyphema , and she was treated with systemic corticosteroids for three days ( methylprednisolone sodium succinate 1000 mg / day ) . her vision slowly recovered to light perception , and on the fourth postoperative day , the right fundus could be seen and showed a cherry - red spot and milky - white edema ( figure 2 , right ) . the edema appeared to be caused by a complete crao , although embolic materials were not seen . fluorescein angiography revealed leakage on the optic disc , and a slightly delayed filling time in the right eye but an arterial filling defect was not noted . one month later , the optic disc appeared pale and atrophic , and optical coherence tomography demonstrated that the extrafoveal region of macula was thinner than normal ( figure 3 ) . a blow - out fracture of the orbital wall is not uncommon , and it often leads to diplopia due to dysfunctions of the extra - ocular muscles . however , it is rare to have a simultaneous blow - out fracture and a crao . earlier , it was reported that an 18-year - old young man was hit over the left eye with a baseball bat and his visual acuity was 1.0 in the left after the injury and before surgical intervention.6 the blow - out fracture was corrected surgically on the ninth day after injury , and several days later a crao developed and his vision in the end was nlp.6 although a spontaneous crao is usually caused by a thorombosis or sclerotic changes , a crao as a complication of trauma is thought to be caused by an elevation of the intraocular pressure , mechanical stress , or injury to the optic nerve . the exact cause of the crao was not determined in our case , however because the blow - out fracture was very severe and eyeball was completely dislocated , an extreme stretching of the optic nerve and compression from the fractured orbital wall may have caused the transient and complete crao . although the efficacy of decompression in cases of trauma is also uncertain,7 her visual outcome was poor even though surgery was performed on the day of the accident and the occlusion was considered to be relieved within several hours . our case demonstrates that a crao can be a complication of a blow - out fracture of the orbital wall and can lead to severe visual loss even with early surgical decompression .
a 20-year - old woman was involved in a traffic accident while riding a motorcycle . the vision in her right eye was severely reduced . at the first examination , the eyelids of her right eye were severely swollen , and the eye could barely be seen . the fundus was not visible . she had no light perception in her vision . computed tomography revealed a severe blow - out fracture in her right eye . surgery was immediately performed to correct the fracture and the eye globe was replaced in the orbit . on the fourth postoperative day , the right fundus was visible and a cherry - red spot and milky - white edema were seen . fluorescein angiography showed an arterial filling defect . four months later , her visual acuity was light perception . our case shows that a central retinal artery occlusion can be a complication of a blow - out fracture of the lower orbital wall and can lead to severe visual loss even with early surgical repair .
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in this issue of critical care , graf and colleagues describe a long - term cohort study of the costs and consequences of intensive care after resuscitation from cardiac arrest . we took particular interest in this study because health care costs in the us exceed those of any other nation . this study was a programmatic evaluation rather than an assessment of a specific intervention such as therapeutic hypothermia . thirty - one percent of the cohort that survived to be cared for in the intensive care setting were still alive 5 years after hospital discharge . the health - related quality of life of this group of 5-year survivors was similar to that of matched healthy controls . the cost per quality - adjusted life year ( qaly ) gained was 14,487 euros ( approximately us $ 22,900 at current rates ) . the cost per life year gained increased by 18% when it included the 6.4% of 5-year survivors who had severe neurological disability ( that is , glasgow coma scale score of less than 6 ) . how much to pay for a health intervention is a poignant question most societies have yet to answer formally . such decisions are complex and are predicated not only on the absolute and incremental cost of the intervention but also on the quantity and quality of effectiveness data related to the intervention . countries with a centralized planning process for health care may imply their answer when they approve or disapprove for national formulary a drug designed to extend life in a terminal disease . the uk 's national health service recently declined approval of bevacizumab ( avastin , with a cost of therapy per year of approximately $ 100,000 ) as first - line therapy for lung and breast cancer . in the us , there appears to be a general consensus that $ 50,000 to $ 100,000 per year of life gained is acceptable . an analysis based on economic principles suggested that we should be willing to spend up to twice the average annual income on health care . in this light , less than 15,000 euros per qaly for intensive care after resuscitation from cardiac arrest is similar to or less than the cost of other commonly used medical interventions . this study has some limitations relative to current standards for economic evaluation of health interventions . the application of post hoc subgroup analysis based on neurologic status tended to underestimate the costs and overestimate the cost - effectiveness of the program . restricting the analysis to consider a health care rather than a societal perspective underestimated costs and made it difficult to compare the results of this analysis with comprehensive economic evaluations of health care and other interventions . these are that quality of life after resuscitation from cardiac arrest is good and that the costs of care after resuscitation are acceptable . survival after out - of - hospital cardiac arrest ( ohca ) has been static over time , but a recent analysis suggests that outcomes are improving . therapeutic hypothermia is likely to be the first of several effective hospital - based interventions for cardiac arrest [ 10 - 12 ] . the perceived poor prognosis and expense of care of patients resuscitated from cardiac arrest are key barriers to the implementation of effective therapies such as cooling . we need to change the culture of resuscitation and recognize that cardiac arrest is a treatable condition that is associated with good quality of life after resuscitation as well as acceptable costs of care . in many countries , imminent death is not always predictable , and a persistent vegetative state is associated with poor quality of life . therefore , we require better methods of predicting who will recover and who will have disability after resuscitation from cardiac arrest , especially in the era of hypothermia . two hundred seventy thousand people experience ohca each year in the us ( g. nichol , unpublished data ) . about 450,000 do so in europe based on extrapolation from population - based incidence estimates . if we double survival after ohca , then 18,900 premature deaths in the us and 31,500 in europe would be averted each year . there are many ways to improve the chain of survival , including improved communications from citizens to emergency medical services , delivery of care to the patient , delivery of the patient to the hospital , and delivery of cardiac and critical care once there . ohca = out - of - hospital cardiac arrest ; qaly = quality - adjusted life year . saw is a member of the american heart association ( aha ) ( dallas , tx , usa ) national registry for cardiopulmonary resuscitation adult research task force . gn is a member of the aha advanced cardiac life support subcommittee , the scientific advisory board of the aha national registry for cardiopulmonary resuscitation , and the board of directors of the medic one foundation ( seattle , wa , usa ) . he has received grants from the national institutes of health ( bethesda , md , usa ) for the resuscitation outcomes consortium ( 20042009 ) , the laerdal foundation for acute medicine ( stavanger , norway ) for a randomized trial of a cpr training aid ( 2007 ) , and the canadian institutes of health research ( ottawa , on , canada ) and medtronic inc . ( minneapolis , mn , usa ) for a randomized trial of a resynchronization therapy ( 20052009 ) . he has received equipment , including mannequins ( laerdal medical , stavanger , norway ) and monitor / defibrillators ( physio - control inc . , a division of medtronic , redmond , wa , usa ) , donated to support overseas medical missions .
two hundred seventy thousand people in the us and 450,000 people in europe experience out - of - hospital cardiac arrest each year . perceived poor prognosis and expense of care of patients resuscitated from cardiac arrest remain barriers to implementation of effective therapies . in this issue of critical care , graf and colleagues have provided a programmatic evaluation of the costs and consequences of intensive care after resuscitation from cardiac arrest . thirty - one percent of the cohort that survived to be cared for in the intensive care setting were still alive 5 years after hospital discharge . the health - related quality of life of this group of 5-year survivors was similar to that of matched healthy controls , and the cost per quality - adjusted life year gained was similar to or less than the cost of other commonly used medical interventions . we need to change the culture of resuscitation and recognize that cardiac arrest is a treatable condition that is associated with acceptable quality of life and costs of care after resuscitation .
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since the first case of transcatheter closure of patent ductus arteriosus ( pda ) by porstmann in 1967,1 device closure has become a mainstream form of intervention for this lesion with a wide variety of devices.2 the amplatzer pda occluder is a self - expanding nitinol double - disk device and consists of two disks of varying sizes with the larger disk positioned at the aortic end of the duct . since its introduction , this device remains , to date , the device of choice for the transcatheter occlusion of large ( > 3 mm ) pdas,3 and the exact technique for device deployment has been previously described.4 we report one child who required device closure of pda with two amplatzer pda devices on two separate occasions . our patient was born in march 2001 and a murmur was noted shortly after birth . echocardiography eventually showed a large pda , and followup did not reveal signs of pulmonary hypertension . an amplatzer pda device ( 8 by 6 mm ) was implanted at almost 3 years of age ( figure 1 ) . repeat cardiac catheterization showed a significant residual shunt ( figures 2 and 3 ) and a second device ( 10 by 8 mm ) was implanted at 4 years and 3 months of age , 1 years after the first device was implanted ( figures 4 and 5 ) , with little residual shunting . the amplatzer ductal occluder is a safe device and has been utilized extensively with few complications in competent hands.5 closure rates of > 99% have been documented,5 and follow - up has not revealed any episodes of delayed device migration , endocarditis , thromboembolism , or wire fracture / device disruption.6 our patient is unusual in that despite standard placement of an appropriately sized amplatzer device in the usual position , significant residual shunting necessitated the placement of a second device .
it is accepted practice to close large patent arterial ducts ( pda ) with amplatzer duct occluder devices , with extremely low rates of residual pda . we report a child who required device closure of pda with two amplatzer pda devices on two separate occasions , despite the first device deployment being a standard placement of an appropriately sized amplatzer device in the usual position .
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since the technology introducing video capsule endoscopy was for the first time presented during digestive disease week in may 2000 and the first brief communication was published in nature , video capsule endoscopy has been widely used in clinical practice . to date more than 600,000 capsules have been deployed worldwide . video capsule endoscopy is a useful tool for evaluating small bowel disease , but appropriate indications and rates of detection , completion , and retention vary . video capsule endoscopy is based on gastrointestinal motility allowing the swallowed capsule to record the mucosa of the gastrointestinal mucosa as the capsule travels , usually uneventfully , down the gastrointestinal tract . in this case , the patient felt discomfort in the cervical portion shortly after swallowing the capsule . the equipped real - time viewer continued to show the unchanged image that was different from an ordinary image of the mucosa of the esophagus or stomach . using upper endoscopy video capsule retention in a zenker diverticulum was clarified and the capsule was safely removed endoscopically . this report describes the case of a video capsule endoscope lodged within a zenker diverticulum , including the usefulness of the equipped real - time viewer and hood - assisted upper endoscope . however , a slight iron deficiency anemia was found as follows : red blood cell count 4.47 106 ( normal 4.1 - 5.3 106 ) , hemoglobin 12.1 g / dl ( normal 14.0 - 18.0 g / dl ) , hematocrit 38.7% ( normal 39.0 - 52.0% ) , serum iron 36 g / dl ( normal 80 - 200 g / dl ) , total iron binding capacity 349 g / dl ( normal 271 - 469 g / dl ) , and ferritin 6.4 ng / ml ( normal 27.0 - 211.0 ng / ml ) . all other standard laboratory tests were within normal ranges . upper endoscopy and colonoscopy did not show any potential cause . immediately after swallowing a capsule endoscope ( olympus , tokyo , japan ) the patient complained of discomfort in the cervical region . the system was equipped with a real - time viewer that showed an unchanging image different from the expected images of the mucosa of the esophagus or stomach . the presence of discomfort in the cervical portion and the image of the capsule endoscopy led to the suspicion that the capsule might be lodged within a cervical diverticulum . upper endoscopy ( gif - xq260 , olympus , tokyo , japan ) under propofol sedation confirmed the retention of the capsule along with food within a zenker diverticulum ( fig . 2 ) . a hood - fitted upper endoscope was used to examine the zenker diverticulum and upper esophagus ( fig . there are several reports of capsule retention in various types of duodenal , small bowel and large bowel diverticula [ 3 , 4 ] . not very many reports exist describing retention in a zenker diverticulum , hopefully because the capsule is placed with endoscopic guidance in these patients . zenker diverticulum was first described by ludlow in 1769 . however , it was friedrich von zenker who recognized zenker diverticulum results from increased intrapharyngeal pressure . zenker diverticulum is located proximal to the upper esophageal sphincter usually on the posterior hypopharyngeal wall and is thought to result from increased intrapharyngeal pressure . zenker diverticulum usually occurs between the seventh and eighth decades of life , and rarely before the age of 40 years . the prevalence of zenker diverticulum among the general population is believed to be between 0.01 and 0.11% . the incidence varies based on region , but it has been described more frequently in the us , canada and australia than in japan and indonesia . in the uk , however , the true incidence of zenker diverticulum is difficult to establish since the number of asymptomatic patients is unknown . in this case , retention of the capsule endoscope was the first suggestion of the existence of a heretofore asymptomatic zenker diverticulum , although the patient did have an initial upper endoscopy which should have noted the presence of the diverticulum . the presence of the real - time viewer proved useful for quickly allowing retention of the capsule within a diverticulum to be considered . whenever a zenker diverticulum is suspected , the capsule should probably be placed using some nonendoscopic method where it is attached to a holder that could be used , hopefully because the capsule is being placed with endoscopic guidance in these patients [ 5 , 13 ] .
we report the case of a video capsule endoscope lodged within a zenker diverticulum . the system that was equipped with a real - time viewer showed an unchanging image unlike esophageal or gastric mucosa , suggesting that the capsule was elsewhere . the presence of cervical discomfort suggested video capsule retention in a zenker diverticulum . the capsule was removed endoscopically and reinserted using a hood - assisted endoscope and the procedure was completed .
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brown tumor is one of the lesions that develop in patients with hyperparathyroidism and it affects the jaw bones occasionally . histologically it is difficult to differentiate from other giant cell tumors , so clinical diagnosis is made with the findings of hyperparathyroidism . although initially associated with primary hyperthyroidism , they are being seen with greater frequency of secondary hyperparathyroidism . we report a rare case of browns tumor of maxilla , mandible , and left knee joint in a 21-year - old female patient . a 21-year - old female reported to the department of oral and maxillofacial surgery with complaint of a painless swelling in the mandible and posterior maxilla bilaterally [ figures 13 ] , left lateral wall of the nose and left knee joint for the past three years , which was growing slowly ; swelling started in the mandible first then in maxilla and later in lateral wall of the nose and knee joint . clinical examination and ct scan showed a diffused swelling in the mandible measuring 6 7 cm and diffused swelling in the posterior maxilla at the tuberosity region ; on the right side it measures 4 2 cm and 2 2 cm on the left side , and lateral wall of the nose measures 1 2 cm and left knee measures 10 6 cm [ figures 47 ] . skin over the swelling was normal and pinchable , mouth opening was normal , teeth involved were mobile . preoperative picture showing left maxillary tumor preoperative picture showing right maxillary tumor preoperative picture showing mandibular tumor ct scan showing tumor at mandibular region and right lateral wall of the nose ct scan showing tumor at mandibular region ct scan showing tumor at right maxillary region x - ray showing tumor at left knee joint ct scan was evident for a large non - homogenously enhancing mixed density lesion . an incisional biopsy showed numerous osteoclastic giant cells with fibroblastic proliferation and areas of hemorrhage were seen with no evidence of malignancy . routine blood investigation like hb , bt , ct , esr , total wbc count , platelet count , and biochemical examination like thyroid profile , serum calcium , parathormone levels , fbs , and urine examination for deposits and albumin was done . the blood and urine investigation showed normal values except for the hemoglobin , which was below normal value and wide increased in the parathormone levels [ table 1 ] . two units of pre - surgical blood transfusion and one unit of transfusion postoperatively were given to maintain the hemoglobin levels . surgery was performed under general anesthesia ; the mandibular tumor site was approached extraorally through bilateral submandibular incision along with chin sparing lip split technique . the mandibular mass was removed by segmental mandibulectomy , and bilateral maxillary mass was approached through the mandibular resected site and posterior maxillectomy done bilaterally till the clear pterygoid plates were seen and lateral nasal lesion was approached intra orally [ figures 8 and 9 ] . reconstruction plate was used to maintain the contour of the mandible [ figure 10 ] ; primary closure was achieved in the mandible [ figure 11 ] and lateral wall of the nose , but posterior maxilla was left to heal by secondary intention . the resected specimen was sent for histopathological examination ; the histopathological slide with eosin and hematoxylin section with 40 magnification diagnosed as brown tumor [ figure 12 ] . the case was further referred to endocrinologist for further management of hyperparathyroidism and was reviewed for three years , which showed no signs of recurrence . biochemical investigation chart resected mandibular segment resected maxillary site reconstruction plate in position postoperative extraoral picture many osteoclasts like multinucleated giant cells and fi broblast with hemosiderin deposits ostitis fibrosa cystica as a manifestation of primary hyperparathyroidism was initially described by von recklinghausen in 1891 . it results from direct effect of parathyroid hormone on bone , causing the conversion of potentially osteogenic cell from osteoblast to osteoclast , with bone resorption exceeding the formation of new osseous tissue . an imbalance of osteoclastic and osteoblastic activity causes bone resorption with fibrous replacement of the marrow and thinning of the cortex . widespread use of dialysis has led to a larger number of patients with secondary hyperparathyroidism . histologically there is a dense fibroblastic stroma , focal areas of osteoid , cystic degeneration , hemorrhage , osteoclastic multinucleated giant cells . brown tumors is difficult to distinguish histologically or radiologically from other giant cell tumors because of its infiltrative nature . persistent or large tumors can be removed by surgical method . the destructive nature and tumor progression necessitated surgical removal and was stabilized with external fixation . it 's been reported that brown tumor could be treated by local radiotherapy or curettage . it is also said that excision of the brown tumor may be required in case of the large tumor with the tissue destruction . many methods that are used to reconstruct mandible are alloplastic implants such as bone plates and screws , autogenous bone graft , fibular free flap , scapular free flap , iliac crest , radial forearm free flap , double - flap reconstruction , osteointegrated dental implant . in our case
brown tumor is a focal lesion differentiated from other giant cell tumor by the presence of hyperparathyroidism . these lesions are non - neoplastic and they appear as a mass with partly cystic and partly solid areas . clinically they are slow growing lesions that can be locally destructive resulting in variety of symptoms such as significant bone swelling , pain and pathological fracture . here is a female patient of 26 years with brown tumor involving mandible , maxilla and left knee joint .
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to describe the activities and time spent by casemanagers for home - dwelling people with dementia and their family caregivers in a western region of the netherlands . people with dementia and their family caregivers go through several transitions in disease , care and social roles . integrated care for this group is important . in the dutch guideline for integrated dementia care ( 2008 ) , the casemanager is put forward as the vital link in the health - care supply chain for demented people and their families . during six months , six casemanagers registered the frequency and content of their contacts with a total of 40 home - dwelling elderly and their families . on average , casemanagers had contact with their clients once every five weeks , mostly with the family caregiver alone ( by phone ) or with the client - caregiver dyad ( home visit ) . the most frequent interventions offered by the casemanager are : monitoring ( keeping track ) , listening , and giving moral support , practical advice and information about services and procedures . casemanagers spend most of their working time linking health care services with the client - caregiver 's needs . they spend about 56 hours per month at non - client related activities , including education / studying , participation in dementia support groups and networking . case management includes monitoring , counselling and linking of care to stimulate tailor - made care that meets the needs of the client - caregiver dyad .
purposeto describe the activities and time spent by casemanagers for home - dwelling people with dementia and their family caregivers in a western region of the netherlands.theorypeople with dementia and their family caregivers go through several transitions in disease , care and social roles . integrated care for this group is important . in the dutch guideline for integrated dementia care ( 2008 ) , the casemanager is put forward as the vital link in the health - care supply chain for demented people and their families.methodsduring six months , six casemanagers registered the frequency and content of their contacts with a total of 40 home - dwelling elderly and their families.results and conclusionson average , casemanagers had contact with their clients once every five weeks , mostly with the family caregiver alone ( by phone ) or with the client - caregiver dyad ( home visit ) . the mean contact time was about 50 minutes . the most frequent interventions offered by the casemanager are : monitoring ( keeping track ) , listening , and giving moral support , practical advice and information about services and procedures . casemanagers spend most of their working time linking health care services with the client - caregiver 's needs . they spend about 56 hours per month at non - client related activities , including education / studying , participation in dementia support groups and networking.discussioncase management includes monitoring , counselling and linking of care to stimulate tailor - made care that meets the needs of the client - caregiver dyad . a caseload of around 50 clients per fte casemanager seems reasonable .
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we report the case of a 25-year - old caucasian male who developed an acute right scrotum secondary to bile within peritoneal fluid that entered the scrotum through a previously undiagnosed communicating hydrocele . to the best of our knowledge , approximately 10 ml to 15 ml of bile was spilled during the procedure after inadvertent entry into the gallbladder during its dissection from the gallbladder fossa . after the gallbladder was removed , the abdomen was irrigated until aspirated fluid was clear . the right cremaster muscles were noted to be in spasm . a urologic consultation was obtained . the patient 's complaints and examination were concerning for testicular torsion , and right scrotal exploration was performed 3 hours after completion of the laparoscopic cholecystectomy . opening of the tunica vaginalis revealed 10ml of bile - stained fluid and a normal right testicle . the tunica vaginalis and right testicle were irrigated with saline , and a communicating hydrocele was identified and ligated . postoperatively , the patient noted immediate relief of pain , and examination revealed minimal tenderness in the right scrotum . the total bilirubin value of the fluid drained from the scrotum was 10 mg / dl . the patients ranged in age from 7 to 20 years of age and presented with symptoms between postoperative day 1 and 10 . acute postoperative scrotum due to early hernia recurrence has also been described in an infant after an open inguinal herniorrhaphy . yasumoto et al reported a case of a 10-year - old male who underwent open appendectomy for perforated appendicitis on postoperative day 1 following incision and drainage of a left scrotal abscess . infarction of the upper pole of the right testicle causing acute scrotal pain has been reported after a laparoscopic total extraperitoneal inguinal herniorrhapy . bile causes peritoneal signs on examination in some patients with cystic duct stump leaks after cholecystectomy . although the mechanism of irritation is not fully understood , bile salt concentration and bacteria are thought to be possible causes of bile - induced abdominal pain . the cause of acute scrotal pain in this case was due to bile within peritoneal fluid that entered the right scrotum through a communicating hydrocele . the fluid entered the right scrotum after spillage occurred while the patient was in a reverse trendelenberg position with increased intraperitoneal pressure due to carbon dioxide insufflation . the possibility of bile causing the patient 's pain was included in our differential diagnosis ; however , with no prior cases reported in the literature and our concern for testicular torsion , we felt urgent exploration was indicated . future management of a similar patient may include the option of percutaneous aspiration of the hydrocele with laboratory examination to determine bile concentration . if symptoms persist following aspiration , urgent scrotal exploration would be indicated to rule out testicular torsion . the surgical literature is scattered with only a handful of reported cases of an acute scrotum developing after laparoscopic procedures . an acute suppurative process associated with laparoscopic appendectomy is the most common cause described to date . the patient 's symptoms completely resolved after urgent scrotal exploration with drainage of bilious fluid that entered via a communicating hydrocele . to the best of our knowledge , this is the first case of an acute scrotum due to bile after laparoscopic cholecystectomy . as the volume of minimally invasive procedures performed increases , so does the number of unusual complications that develop . reporting of such uncommon disorders developing after minimally invasive procedures provides a reference that may potentially allow earlier recognition and treatment of similar complications by fellow surgeons in the community .
we report our experience with a patient that developed an acute right hemiscrotum immediately after undergoing an uncomplicated laparoscopic cholecystectomy for gallbladder dyskinesia . the etiology of the acute scrotal pain was due to bile which was spilled into the peritoneum after entry into the gallbladder during dissection . the bile obtained access to the right hemiscrotum via a communicating hydrocele . to the best of our knowledge this is the first report of bile causing an acute scrotum following laparoscopic surgery . a review of the current literature on the topic of the postoperative acute scrotum follows our case presentation .
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hydrometrocolpos is a rare congenital anomaly formed by cystic dilatation of vagina and uterus due to conditions like imperforated hymen , distal vaginal atresia or transverse vaginal septum . ca 19 - 9 is widely used as a tumor marker for cancers of the pancreas , stomach , colon , cholangial duct , ovaries , endometrium , and lung ( adenocarcinoma ) . however , several benign conditions are also known to increase serum ca 19 - 9 levels . ca 125 is an antigenic determinant widely used for screening of ovarian , pancreatic , breast , colon and lung cancers . it also increases in some benign and physiological conditions like pregnancy , menstruation and endometriosis . the baby was born to 28 years old healthy mother in the 38th gestational week by cesarean section with an apgar score at 1 and 5 min were 8 and 9 , respectively . the birthweight , height and head circumference were 3940 g ( > 90p ) , 50 cm ( 5075p ) and 34.8 cm ( 5075p ) , respectively . in the prenatal period the newborn was diagnosed with giant cystic mass that fills the all the abdominal cavity and amniotic fluid volume was normal . in preliminary diagnosis bilateral polycystic kidneys was reported . after the delivery , the patient was intubated because of respiratory failure . in the follow - up of patient requiring mechanical ventilation support with low pressure and there is good ventilation on chest radiograph we did not accept pulmonary hypoplasia . the main cause of intubation accepted as the compression of abdominal mass . laboratory data ( white cell count , c - reactive protein , biochemical tests ) were normal . abdominal ultrasound showed a cystic structure posterior to the urinary bladder measuring 61 46 77 mm , with a thick wall structure and anechoic fluid level , and dilatation of ureters ( fig . abdominal computed tomography confirmed the presence of a cystic structure in the uterine compartment ( fig . the urinary outflow decreased in the postnatal 12th hour , which was thought to be compression by the mass and thus 400 ml of fluid was aspirated underwent ultrasound guided percutaneous drainage . biochemical , cytological and microbiological analysis of the drainage fluid revealed no pathology . among the tests performed for differential diagnosis , levels of alpha - fetoprotein and beta - hcg were normal but serum ca 19 - 9 level was 110.1 u / ml ( reference value : < 27 u / ml ) and ca 125 level was 278.7 u / ml ( reference value : < 35 u / ml ) . the urinary system was found to be normal but distal vaginal atresia was found with cystoscopy . the drainage level decreased and ceased on the 7th day and abdominal distention did not recur . on the 8th day the levels of ca 19.9 and ca 125 turned back to normal ( 17 u / ml and 21 u / ml respectively ) . in the follow - up of patient requiring mechanical ventilation support with low pressure was discontinued on the eighth day . hydrometrocolpos is a rare congenital anomaly with an estimated incidence of 0.13.8 % ( 3 ) . during the newborn period the condition occurs as a result of accumulation of cervical and vaginal secretions due to the obstruction of vaginal outlet . most cases present with abdominal mass , recurrent urinary tract infections or primary amenorrhea in the pubertal period . in the neonatal period it may present with abdominal mass , recurrent urinary tract infection , sepsis , obstructive uropathy and respiratory failure . ca 19 - 9 is widely used as a tumor marker for pancreatic , colon , ovarian , endometrial and lung carcinoma . however several benign conditions such as inflammatory or proliferative diseases ( cholangitis , pancreatitis , bronchial cyst , bronchiectasis , pulmonary fibrosis , endometriosis , pregnancy and ovarian cysts ) , ductal obstructions ( pancreatic and choledochal ) and chronic diseases ( hepatitis , glomerulonephritis , diabetes mellitus , hemodialysis , peritoneal dialysis ) also increase serum ca 19 - 9 levels . ca 125 is also used as a tumor marker for ovarian , pancreatic , breast , colon and lung carcinomas . also some other physiologic conditions such as pregnancy and menstruation can cause elevated serum ca 125 levels . in a study which evaluated patients with ca 125 levels over 1000 u / ml ; 37% of patients had non - malignant gynecological disease ( 43.9% endometriosis , 12.2% adenomyosis , 9.7% pelvic inflammatory disease , 7.3% uterine leiomyoma , 2.4% imperforated hymen ) . however serum ca 125 levels in non - malignant gynecological diseases were significantly lower than those in malignant diseases . there is two case report with elevated levels of ca 19 - 9 and ca 125 in hydrometrocolpos due to imperforated hymen which is a benign condition . both reported cases were in premenarcheal age group and the levels were both higher than our case . this is the first incident reported in literature in the neonatal period presenting with hydrometrocolpos and elevated levels of ca 19 - 9 and ca 125 . hydrometrocolpos is a rare benign condition which may be kept in mind in girl newborn baby with abdominal mass and associated with high ca 19 - 9 and ca 125 levels .
highlightshydrometrocolpos is a rare congenital malformation of the female in neonate.ca 19 - 9 and ca 125 are widely used as tumor markers , however several benign conditions are also known to increase levels.here we report a newborn with hydrometrocolpos due to distal vaginal atresia and elevated serum ca 19 - 9 and ca 125 levels .
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since 2004 , the province of quebec has devoted significant efforts to unify the governance of the main health and social care organizations of its various territories . notwithstanding the uniformity of the national plan s prescription , the territorial integration modalities greatly vary across the province . this research is based upon a conceptual model of integration that comprises six components : inter - organizational partnership , case management , standardized assessment , a single entry point , a standardized service planning tool and a shared clinical file . we conducted an embedded case study in six contrasted sites in terms of their level of integration . interestingly however , no link seems to exist between the quality of local prescriptions and the level of integration achieved in each site . this finding leads us to hypothesize that the variable quality of the operational accompaniment offered to implement these prescriptions is a variable in play .
purposeservices integration comprises organizational , normative , economic , informational and clinical dimensions . since 2004 , the province of quebec has devoted significant efforts to unify the governance of the main health and social care organizations of its various territories . notwithstanding the uniformity of the national plan s prescription , the territorial integration modalities greatly vary across the province.theorythis research is based upon a conceptual model of integration that comprises six components : inter - organizational partnership , case management , standardized assessment , a single entry point , a standardized service planning tool and a shared clinical file.methodswe conducted an embedded case study in six contrasted sites in terms of their level of integration . all documents prescribing the implementation of integration were retrieved and analyzed.results and conclusionsthe analyzed documents demonstrate a growing local appropriation of the current integrative reform . interestingly however , no link seems to exist between the quality of local prescriptions and the level of integration achieved in each site . this finding leads us to hypothesize that the variable quality of the operational accompaniment offered to implement these prescriptions is a variable in play .
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recently , a covered self - expandable metal stent ( sems ) is being used . the advantages of the covered sems is prolonged patency , lower failure , few complications , and more easiness in its removal.1 however , a rare complication of stent fracture is reported due to the possibility of metal fatigue , tearing of polyurethane stent cover , or application of laser , electrocoagulation.2 proximal or distal migration is another complication after stent insertion . so far , just a few cases of proximal migration of sems have been reported . this report describes that the complication of sems fracture during endoscopic retrieval of the stent and proximal migration of the remnant stent in patient with chronic pancreatitis . a 62-year - old man with benign biliary stricture due to chronic pancreatitis underwent 10-mm diameter , 60-mm lengthened covered biliary sems ( shim - hanaro ; mi tech , seoul , korea ) ( fig . 1 ) . after 5 months , removal of sems was attempted by using forcep to grasp the extraductal portion of the distal sems . during the procedure , 2 ) , and remnant sems moved proximally into the common bile duct ( fig . 3a ) . despite multiple trials were attempted to retrieve the remnant sems by using various grasping accessories or balloon catheter , methods failed . endoscopic papillary balloon dilatation with maximal diameter of 12 mm diameter ( cre wire - guided dilator ; boston scientific , marlboro , ma , usa ) was performed below the remnant sems to make a space between the outer surface of sems and the inner surface of common bile duct ( fig . 3b ) . snare was advanced through the space , then the stent was grasped and retrieved without any complication ( fig . the frequency of sems using is gradually increasing in alternative treatment for benign biliary strictures.3 it is difficult to remove uncovered sems coated with epithelial hyperplasia . in comparison , it is easier to remove covered sems.1,4 therefore , recently , the frequency of covered sems using is increasing . complications related to sems are failure of stent release , malpositioning of the stent , stent occlusion with epithelial hyperplasia , duodenal bleeding or perforation due to trauma to duodenal wall , stent fracture and stent migration . the causes of fracture were not only therapeutic thermal overstrain during the electrocoagulation but spontaneity.2,5,6 the fracture might be related to bile induced corrosion or metal fatigue from constant and repeated bending in the duodenum.5 - 7 the anatomic sites of fracture were mostly around the ampulla,5 - 7 and a literature7 reported that 75% ( 3/4 ) of the fracture sites were at the ampulla with disconnection of the distal part of the stent in patients with periampullary malignant biliary obstruction . the cause of fracture of sems in this case was unclear and might be multifactorial . in case of distal biliary stricture , it could be suggested that there is a possibility of the structural characteristics of weak point of the sems at the ampulla as a causative factor . to the best of our knowledge , fracture of sems during the endoscopic removal has of been previously reported . in our case , the fracture of distal part of stent at the level of ampulla occurred during the retrieval of the normally situated sems . we presume that the event related to shearing force by pulling the stent through the grasp device at the damageable site of unseen stress fracture . biliary stent migration can reduce the function of stent and make injury of duodenal wall , perforation , or bleeding.8 if stent migration occurred , the stent should be removed . in the case of retrieval of distally migrated sems , snare or forcep can be used to grasp the free end of sems.3 however , it is difficult to remove proximally migrated biliary sems , and methods to remove proximally migrated sems are stent cannulation and subsequent balloon retrieval , balloon placement parallel to the stent with traction retrieval , and use of a wire basket , snare , or forceps.9 - 11 in our case , we successfully removed the distal fragment of the fractured sems at the beginning , but we failed to remove proximally migrated fragment of the fractured sems with use by several tools such as the forcep , snare , balloon , or retriever . therefore , to grasp firmly remained stent , we allocated spaces between the sems and inner part of bile duct through the balloon , then successfully we removed the stent . in our case , we realized that the endoscopist must be careful to inspect the complication such as the fracture and migration of stent which can occur during the endoscopic removal of sems .
the endoscopic insertion of the self - expandable metal stent ( sems ) in benign biliary stricture has become an alternative to surgery . fracture or migration of sems can occur rarely as complications . we report a case of fracture of sems during endoscopic retrieval in patients with chronic pancreatitis . in this case , broken stent was successfully removed with endoscopic ballooning of bile duct and with a snare device .
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lower gastrointestinal ( gi ) bleed due to hemangioma in rectum is an uncommon problem . a hemangioma generally has a feeding and draining vessel and the collection of contrast in hemangioma helps in its identification during computed tomography ( ct ) scan or magnetic resonance imaging ( mri ) . a 19-year - old female patient presented with history of recurrent episodes of lower gi bleeding 1 - 2 times / month for last 3 years . at the time of hospitalization colonoscopy showed bluish reddish elevated nodular lesions limited to distal rectum [ figure 1 and video 1 ] . mri of rectum showed hyper intense signals in the anterior wall [ figure 2 ] . the radial ultrasound was able to demonstrate vascular signal in the submucosa of anterior wall of rectum [ figure 3 ] . real time eus imaging was able to trace an outflowing vessel through the left lateral wall of rectum for a distance of about 3 cm [ video 2 ] . application of pulse doppler confirmed the venous nature of the outflowing vessel [ figure 4 ] . linear eus showed a submucosal vascular lesion in the anterior wall of rectum supplied by an inflowing artery [ figure 5 and video 3 ] . a biopsy of the lesion showed numerous dilated vascular spaces within lamina propria and submucosa [ figure 6 ] . the lesion was seen in anterior wall magnetic resonance imaging of rectum showed hyper intense signals in the anterior wall ( green arrow ) the radial ultrasound of rectum showed vascular signal in the submucosa of anterior wall of rectum application of pulse doppler confirmed the venous nature of the outflowing vessel in the anterior wall of rectum linear endoscopic ultrasound showed a submucosal vascular lesion in the anterior wall of rectum supplied by an inflowing artery a biopsy of the lesion showed numerous dilated vascular spaces within lamina propria and submucosa cavernous hemangioma is an uncommon entity responsible for < 1% of lower gi bleed . diagnosis is best established by endoscopic visualization of a blood filled hemangioma that has appearance of plum red nodules or vascular congestion . ct scan and mri can also be used for diagnosis and evaluation of the extent . in this case the diagnosis was suspected by endoscopic appearance and mri and continuous color doppler eus of the lesion provided additional information of presence of a vascular lesion [ figures 35 , videos 2 and 3 ] .
lower gastrointestinal ( gi ) bleed due to hemangioma in rectum is an uncommon problem . a 19-year - old female patient presented with history of recurrent episodes of lower gi bleeding 1 - 2 times / month for last 3 years . at the time of hospitalization her vital signs were normal and rectal examination revealed frank blood . investigations revealed a hemoglobin level of 8.9 g / dl and normal coagulation parameters . colonoscopy showed bluish reddish elevated nodular lesions limited to distal rectum . magnetic resonance imaging and endoscopic ultrasound showed cavernous hemangioma .
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rhegmatogenous retinal detachment ( rd ) is caused by liquefied vitreous passing through a retinal break into the subretinal space , separating the neurosensory retina from the retinal pigment epithelium . low myopes [ 0.75 to 2.75 diopters ( dpt ) ] show an odds ratio of 3.14 for rd , and the odds ratio was shown to rise steeply with increasing myopic refractive errors in the population . refractive surgeries , like laser in situ keratomileusis ( lasik ) and laser - assisted subepithelial keratomileusis , have been popularized for correction of low to moderate myopia . vision - threatening posterior segment complications may occur after refractive surgeries , including macular hemorrhages , macular holes and rhegmatogenous rd . the reported incidence of rhegmatogenous rd in lasik patients is not high , ranging from 0.033 to 0.25% [ 9 , 131415 ] . however , many have regarded a suction ring application during lasik to be a potential risk factor for rhegmatogenous rd , because this procedure may induce vitreous traction and detachment resulting from sudden decompression of the eye [ 16 , 17 ] . it has been reported that retinal breaks were more commonly located in the inferotemporal quadrant in rhegmatogenous rd after lasik . here , we report on a case with bilateral simultaneous rhegmatogenous rd following lasik surgery . she was wearing a correction for myopia with a prescription of 6.5 dpt in the right eye and 7.00 dpt in the left eye . her past medical history was unremarkable . on initial examination , visual acuity was counting fingers at 1 m on the right and 10/10 on the left . dilated fundus examination revealed a total rd secondary to a retinal tear at approximately 11 o'clock in the right eye . there was a localized rd secondary to retinal tear at approximately 13 o'clock in the left eye , and the posterior pole was attached ( fig . the patient underwent pars plana vitrectomy surgery combined with endolaser photocoagulation and silicone oil tamponade in the right eye . a week later , pneumatic retinopexy was done in the left eye . as the retinal tear did not seal , a 360 scleral buckling surgery was performed and the retina was attached . on the last visit , risk of rd is 10-fold higher in eyes over 3.0 dpt and is 0.075% in eyes exceeding 10 dpt . there are several reports on post - lasik rd and most of these cases have high myopia ( > 10 dpt ) [ 21 , 22 ] . increased vitreous liquefaction , earlier posterior vitreous detachment and higher incidence of vitreoretinal degeneration , such as lattice degeneration , are thought to be attributable to the higher prevalence in rhegmatogenous rd in myopes . in our case , there was bilateral rhegmatogenous rd in both eyes at the same time . as far as we know , no such case has been reported previously . ozdamar et al . reported a bilateral rd with giant retinal tear following lasik surgery . reviglio et al . presented a case with high myopia ( 13.00 + 3.00 15 dpt in the right eye and 13.00 + 3.00 170 dpt in the left eye ) who developed rd 14 h after lasik surgery . arevalo et al . evaluated 1,1594 lasik patients with myopia in a 10-year follow - up . they found rd in 22 eyes of 19 patients during the course ( ranging between 1 month and 10 years ) . therefore , lasik surgery could not be the only causative factor for rd in these eyes . there is a positive correlation between rd and the amount of myopia . in a study by ogawa and tanaka , patients with > 15 qin et al . reported 6 rd cases in 9,598 lasik patients ( 0.033% ) . as known , myopia increases the risk of rd ( from 0.70 to 6% ) . the difference may be due to detailed fundus examination and preventive interventions prior to lasik surgery . it was in accordance with two previous large series which reported 16.3 and 27.3 months , respectively [ 22 , 27 ] . all the previously reported cases in the literature were either unilateral rd or bilateral rd presenting at separate times following lasik . simultaneous rd patients , as a subgroup of bilateral rd , may have more severe retinal weakness . younger patients with myopia and coexisting retinal degenerations may be predisposed to simultaneous rd as in this case . to conclude , although lasik surgery is still an effective and safe method used to correct myopia , bilateral simultaneous rd should be included in the postoperative lasik complications . a thorough and careful retinal examination should be done before surgery , particularly in young myopic patients .
a 21-year - old woman developed simultaneous rhegmatogenous retinal detachment after laser in situ keratomileusis ( lasik ) in both eyes . she underwent pars plana vitrectomy surgery combined with endolaser photocoagulation and silicone oil tamponade in the right eye . a week later , pneumatic retinopexy was done in the left eye . as the retinal tear did not seal , 360 scleral buckling surgery was performed and retina was attached . bilateral simultaneous rhegmatogenous retinal detachment after lasik for correction of myopia can be a serious complication . patients should be informed about the possibility of this complication .
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pentacene was deposited on a muscovite mica surface by physical vapor deposition from a knudsen cell in an ultrahigh vacuum chamber . the cell temperatures were adjusted between 450 and 490 k , in order to realize deposition rates between 0.01 ml / min and 4.5 ml / min , respectively . the mica samples ( 10 10 0.01 mm ) were attached to a steel plate via tantalum wires . the steel plate was heated resistively , and its temperature was controlled by a ni , the temperature of the mica sample could be varied between 100 and 1000 k. typically , the sample holder was cooled during the experiments for a better residual gas pressure , but the sample was hold at 300 k during deposition by proper sample heating . for a quantitative determination of the deposited material a quartz microbalance was used , which was located next to the mica substrate . the mica substrate was cleaved with adhesive tape prior to installation into the vacuum chamber and subsequently gently sputtered by argon ions . ten minutes of sputtering with 500 ev ar+ ions at an argon pressure of 5 10 mbar were sufficient to change the 5a film morphology from needle like islands , composed of lying molecules , to compact islands composed of standing molecules . the surface chemical composition was analyzed by auger electron spectroscopy and x - ray photoelectron spectroscopy . thermal desorption spectroscopy was applied to determine the thermal stability of the pentacene film and the sticking coefficient . ex - situ atomic force microscopy ( nanosurf , easyscan2 ) was used to analyze the film morphology .
it is known in thin - film deposition that the density of nucleated clusters n varies with the deposition rate f as a power law , n f. the exponent is a function of the critical nucleus size i in a way that changes with the aggregation limiting process . we extend here the derivation of the analytical capture - zone distribution function p(s ) = asexp(-bs2 ) of pimpinelli and einstein to generic aggregation - limiting processes . we show that the parameter is generally related to the critical nucleus size i and to the exponent by the equality = i , in the case of compact islands . this remarkable result allows one to measure i with no a priori knowledge of the actual aggregation mechanism . we apply this equality to measuring the critical nucleus size for pentacene deposition on mica . this system shows a crossover from diffusion - limited to attachment - limited aggregation with increasing deposition rates .
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subarachnoid hemorrhage ( sah ) is one of the most common radiological features of traumatic brain injury ( tbi ) occurring in 30%40% of moderate severe head injuries . vasospasm and resulting cerebral ischemia as well as cerebral hyperemia can cause delayed neurological recovery and poor outcome . a 56-year - old male suffered a moderate head injury following an alleged road traffic accident and presented to the neuro intensive care unit with a glasgow coma scale ( gcs ) score of 11/15 . the computerized tomography ( ct ) scan of brain showed bilateral frontal and right temporal contusion with left sylvian and temporal sah . he was treated with antiedema measures , namely , mannitol 1 g / kg and hypertonic saline ( 3% ) at 30 ml / h infusion . ct scan was repeated when gcs did not improve after 48 h. it showed a relative decrease in cerebral edema compared to the earlier scan . in view of this , antiedema measures were continued for further 2 days . however , there was no improvement in gcs . a transcranial doppler ( tcd ) done at this time was suggestive of cerebral hyperemia [ figure 1 ] with a lindegaard ratio ( lr ) of 4 . the possibility of vasospasm in view of traumatic sah was ruled out with a normal digital subtraction angiography ( dsa ) [ figure 2 ] done on the 5 posttrauma day . conservative management was continued and the patient started improving clinically to gcs score of 13/15 on day 7 . transcranial doppler flow with hyperemia of brain angiographic image of bilateral internal carotid arteries ruling out vasospasm transcranial doppler flow when hyperemia reduced tcd can be an important bedside tool in the management of head injury . in patients with unanticipated delay in improvement of gcs , tcd can play a vital role . the lr calculated as the ratio of mean flow of middle cerebral artery ( mca ) and mean flow of extracranial internal carotid artery helps diagnose or rule out vasospasm as a cause . the mean flow velocities of mca coupled with lr differentiates vasospasm from hyperemia [ table 1 ] . transcranial doppler grading criteria for middle cerebral artery vasospasm as in our case with lr of 4 , a definitive dsa was needed to rule out vasospasm . the significance of hyperemia on the management and clinical outcome of tbi is still unknown . the effect of hyperemia on intracranial pressure ( icp ) is a key to predicting clinical outcome . found hyperemia associated with raised icp to be a predictor of poor outcome and needing aggressive treatment while mild hyperemia without raised icp was linked to favorable outcome . in our case , also , mild moderate hyperemia suggested by lr of 4 and pulsatility index of 0.8 relating to low icp had a good outcome . the doppler waveform suggestive of hyperemia was described by chan et al . as an absent diastolic notch similar to our recording . the clinical implications of the above - mentioned findings are of uncertain significance . however , as observed in our patient , the resolution of hyperemia coincided with clinical improvement and may just be the temporal profile of the injury . the popularity of tcd as a bedside tool is hampered by the limitation that it is extremely operator dependent . the angle of insonation and detailed knowledge of cerebral vascular anatomy and its variant limits the interpretation . this case report highlights the importance of evaluating with tcd for an unexplained persistent low gcs . cerebral hyperemia may be one of the causes of nonimproving gcs in the early phases of tbi . once diagnosed , this entity may not need any further treatment other than close neurological observation .
subarachnoid hemorrhage is a common manifestation of traumatic brain injury . a clinical deterioration in glasgow coma scale score without an accompanying radiological worsening is suggestive of vasospasm . however , hyperemia could be another possibility which can easily be considered with corroborating transcranial doppler ( tcd ) features . this case report reiterates the value of tcd in such instances .
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myasthenia gravis ( mg ) , an autoimmune disease , impedes the postsynaptic acetylcholine receptors at the neuromuscular junction.1 ocular mg is a representative manifestation of this entity in which blepharoptosis and/or eye movement disturbances are often encountered.2 in general , up to 85% of all myasthenic patients show the anticholinesterase receptor antibody in serum , but only about 50% of patients demonstrate ocular muscle weakness.3 although cholinesterase inhibitors with or without steroids are commonly used as a treatment modality , they are , occasionally , less effective for ocular symptoms.4 in such a case , especially for ptosis without any eye movement disorders , ptosis surgery is often performed . this paper presents an ocular mg case with eyelid dysfunction , in which cholinesterase inhibitors and steroids did not work sufficiently , but surgical treatment successfully improved the symptom . a 39-year - old woman was diagnosed with mg when she was 8 years old . with a positive edrophonium test and detectable serum acetylcholine receptor antibody , the diagnosis of ocular mg was confirmed in addition to the unresponsiveness to treatment with pyridostigmine and steroids at 31 years of age . despite long - term treatment with steroids , she had difficulty opening both eyes , which caused stiff shoulders and headaches . as she showed bilateral 3 mm levator function ( figure 1a ) without any eye movement disturbances , bilateral frontalis sling procedures were performed with an autologous fascia lata . the skin incision was made 6 mm from the eyelid margin , and the central area of the upper brow margin was also incised ( figure 2a ) . a tunnel was made from the brow incision through the suborbicularis oculi layer , and reached the pretarsal area . the branched fascia lata was sutured with 60 nylon ( sigma , tokyo , japan ) on the tarsal plate and then the upper eyelid curvature was confirmed by pulling the fascia through the suborbicularis tunnel ( figure 2b ) . after the upper eyelid height was adjusted appropriately with a trial suture at the brow incision , the fascia was fixed at the subcutaneous tissue of the brow . one year after the operation , the upper eyelids showed symmetrically appropriate heights ( figure 1b ) . the patient did not demonstrate exposure keratitis , wound infection , lagophthalmos , or ptosis in the 6 months following the operation . the frontalis sling surgery for ptosis by ocular mg accomplished functionally and cosmetically good outcomes . as the levator function of the patient was bilaterally 3 mm , in general , ptosis with less than 4 mm levator function needs a sling procedure,5 but with more levator function , levator advancement surgery is applied.6 as an excessive advancement of levator often leads to an eyelid - eyeball dissociation , causing dry eyes , a sling procedure should be used in such a case . in the past , numerous materials have been used for slings like silicone rods , gore - tex , and autologous tissue graft.5,79 the autologous graft from fascia lata was chosen rather than the artificial material in terms of its histocompatibility . although the patient did not show any eye movement disturbances , mg patients often show eye movement disturbances simultaneously with ptosis.10 bilateral ptosis surgery should be avoided in such a case to prevent postoperative diplopia . in this situation , many treatment modalities had been given to the patient , but her condition did not show definite improvement . generally in mg , anticholinesterases ( cholinesterase inhibitors ) although steroids are of great short - term benefit in most patients with ocular mg , the side effects associated with steroids may prevent long - term use.10 therapeutic effect of thymectomy is controversial for ocular mg . therefore , a patient refractory to any medical treatments is a good candidate for ptosis surgery . in conclusion , this paper reports an ocular mg case with eyelid dysfunction , in which cholinesterase inhibitors and steroids did not work sufficiently , but surgical treatment successfully improved the symptom . a sling procedure with an autologous fascia was suitable for correcting poor levator function of an ocular mg case .
a 39-year - old woman was diagnosed with myasthenia gravis when she was 8 years old . although many treatments such as cholinesterase inhibitors and steroids had been given to the patient , her condition did not improve sufficiently . as she demonstrated bilateral 3 mm levator function without any eye movement disturbances , bilateral frontalis sling procedures were performed with an autologous fascia lata . one year after the operation , the operated upper eyelids showed symmetrically appropriate heights with good functional outcome . a sling procedure with an autologous fascia lata was suitable for correcting poor levator function of an ocular myasthenia gravis case .
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metachromatic leukodystrophy ( mld ) ( scholz 's disease ) is an autosomal recessive lysosomal storage disease caused by the lack of arylsulfatase a ( asa ) . this enzyme is essential for the normal metabolism of sulfatides which are vital elements of the myelin sheath . in mld , sulfatides accrue in many organs comprising brain , peripheral nerves , kidneys , liver , and gallbladder . sulfatide collects in white matter of the central nervous system and peripheral nerves and causes progressive demyelination and lethal neurological symptoms . mld is diagnosed biochemically by finding low levels of arylsulfatase in peripheral white blood cells and urine . mld is classified into three main clinical forms on the basis of the age of onset . the most common and lethal form is the late - infantile form , which begins before 4 years of age typically presenting between 12 and 18 months of age , and patients die by the end of the first decade . the juvenile form of mld comprises age onset between 4 and 16 years , whereas symptoms of adult mld begin after puberty . the patients usually present with signs and symptoms of peripheral neuropathy and alterations in intelligence , speech , and coordination . the disorder is progressive with gait disturbance , quadriplegia , decerebration , and mortality by the age of 6 months to 4 years . magnetic resonance ( mr ) imaging findings of the brain , especially t2-weighted imaging findings in the form of symmetric t2 hyperintense signal in the periventricular white matter , have been commonly reported in previous reports . however , diffusion - weighted imaging ( dwi ) findings have been sparsely reported and we present mr imaging , especially dwi findings in a 12-month - old patient of mld . a 12-month - old male presented with regression of milestones and progressive spasticity . mr imaging examination was performed and t2-weighted images revealed symmetrical hyperintensities , predominantly involving the deep white matter , corpus callosum with sparing of subcortical u - fibers [ figure 1 ] . the tigroid and leopard skin patterns of demyelination , which imply sparing of the perivascular white matter , were evident in the periventricular white matter and centrum semiovale [ figure 2 ] . the corpus callosum ( genu and splenium ) was also involved . using the transverse single - shot echo planar diffusion - weighted mr imaging , diffusion mr images were acquired . on b = 1000 mm / s images ( heavily dwis ) , hyperintensities were apparent in the deep white matter and corpus callosum with a signal intensity pattern the same as that of cytotoxic edema . on apparent diffusion coefficient ( adc ) maps , low levels of arylsulfatase in peripheral white blood cells and urine confirmed the diagnosis of mld . ( a and b ) bilateral symmetrical butterfly - shaped t2 hyperintensities of the periventricular and deep white matter with relative sparing of the subcortical u - fibers sagittal t2-weighted image showing tigroid pattern of due to hypointense linear bands against the background of hyperintense white matter the corresponding lesions showing restricted diffusion with high diffusion - weighted ( a and b ) and low apparent diffusion coefficient signal ( c and d ) symmetric confluent areas of t2 hyperintense signal in the periventricular white matter with sparing of the subcortical u - fibers and with no enhancement in postcontrast images are the most frequent reported mr imaging findings of mld . the tigroid and leopard skin patterns of demyelination are basically due to sparing of perivascular white matter and are visualized as dark spots or dark linear areas against a background of hyperintense white matter , giving the appearance of the skin of a leopard . the disease process also commonly affects the corpus callosum , internal capsule , and corticospinal tracts . the cerebellar white matter may also be affected and appears bright on t2-weighted images . in the advanced stage of mld , it has been reported that the diminished activity of asa leads to dysmyelination ( failure of myelin breakdown and reutilization ) . the corresponding distribution of the lesions on t2-weighted image and dwi in the present case strongly implied that the lesions on dwi were directly linked to the disease process . restricted diffusion ( cytotoxic edema ) was seen on the echo planar sequence as hyperintensities on heavily dwis ( b = 1000 s / mm ) and low adc values on adc maps [ figure 3 ] . sener reported a similar diffusion mr imaging pattern on echo planar diffusion images of a patient with mld with unchanged pattern on 6-month follow - up . phillips et al . also reported a similar pattern of restricted diffusion in patients of phenylketonuria which was hypothesized to be due to impaired myelination leading to reduced mobility of protons . hence , in the present case of mld , the restricted diffusion pattern perhaps was consistent with some breakdown of the white matter secondary to impaired myelination , which results in restricted mobility of protons leading to a diffusion mr imaging pattern similar to that of cytotoxic edema . to conclude , we report diffusion mr imaging of mld done using the echo planar sequence which has been sparsely reported . more diffusion mr imaging studies of dysmyelinating disorders might improve our interpretation of the imaging features of these diseases .
metachromatic leukodystrophy ( mld ) is caused by insufficiency of arylsulfatase a resulting in impaired myelination . diffusion magnetic resonance ( mr ) imaging features of this disease have been rarely reported . we report diffusion mr imaging of mld in a 12-month - old male who presented with regression of milestones and progressive spasticity .
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to report a case of radiation - induced macular ischemia where vision and macular perfusion improved after hyperbaric oxygen ( hbo ) therapy . a 62-year - old male patient developed radiation - induced macular ischemia after he was treated with radiation for brain glioma . the patient presented with best spectacle - corrected visual acuity ( bscva ) acuity of 20/400 in his right eye . the patient s vision improved from 20/400 to 20/100 after focal laser and intravitreal triamcinolone . his central macular thickness improved from 468 m to 132 m . after receiving hbo therapy ,
purpose : to report a case of radiation - induced macular ischemia where vision and macular perfusion improved after hyperbaric oxygen ( hbo ) therapy.methods:a 62-year - old male patient developed radiation - induced macular ischemia after he was treated with radiation for brain glioma . the patient presented with best spectacle - corrected visual acuity ( bscva ) acuity of 20/400 in his right eye . optical coherence tomography ( oct ) showed central macular thickness of 468 m . the patient received focal laser , intravitreal triamcinolone , and hbo therapy.results:the patient s vision improved from 20/400 to 20/100 after focal laser and intravitreal triamcinolone . his central macular thickness improved from 468 m to 132 m . after receiving hbo therapy , his va improved to 20/50 and fluorescein angiography showed improvement in macular perfusion.conclusion:hbo therapy improves macular perfusion in patients with radiation - induced macular ischemia .
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a pregnant 23-year - old female with a six - year history of type 1 diabetes presented with a complaint of increased blur in both eyes for the previous two months . best corrected visual acuity was 20/40 ou . slit - lamp examination was entirely unremarkable in both eyes . dilated funduscopic examination was significant for flame - shaped , dot , and blot hemorrhages in the posterior segment , with associated macular edema and exudates bilaterally ( figures 1a and 1b ) . optical coherence tomography ( oct ) revealed a foveal thickness of 578 5 microns and 667 8 microns in the right and left eye , respectively ( figures 2a and 2b ) . all oct scans were performed with the stratus optical coherence tomograph ( zeiss - humphrey inc , dublin , ca ) . examination findings were consistent with bilateral , nonproliferative diabetic retinopathy and clinically significant macular edema . after careful deliberation with the patient and her obstetrician , a decision was made to treat the bilateral macular edema with intra - vitreal triamcinolone acetonide injection . the patient received 0.05 ml of triamcinolone acetonide 40 mg / ml in the left eye initially and in the right eye one week later . best - corrected visual acuity was 20/20 and 20/25 in the right and left eye , respectively . repeat oct revealed a foveal thickness of 159 5 microns and 202 6 microns in the right and left eye , respectively ( figures 3a and 3b ) . slit - lamp examination and goldman applanation tonometry did not reveal any intraocular hypertension or significant lenticular changes at this visit or at any point after triamcinolone injection . the patient delivered a full - term , healthy baby boy weeks prior to this examination . progression of diabetic retinopathy during pregnancy has been described previously by several authors.1,2 visual impairment in these cases can result from both proliferative ( eg , vitreous hemorrhage , retinal detachment ) and nonproliferative etiologies ( eg , retinal hemorrhage , papillopathy , macular edema).1 although macular edema may regress in some cases after delivery , in other cases edema can persist and can be associated with severe and persistent visual dysfunction.1 data from large , randomized clinical trials have established the benefits of argon laser photocoagulation for clinically significant macular edema.3 focal laser photocoagulation of actively leaking blood vessels or grid laser for areas of diffuse permeability can decrease clinically significant edema . however , laser photocoagulation in close proximity to the fovea increases the risk of inducing iatrogenic central scotoma as a result of thermal injury to the tissues , or subsequent glial proliferation . there are many reports of off - label use of intravitreal triamcinolone in cases of persistent and refractory diabetic macular edema.4 improved visual acuity and decreased foveal thickness have been documented by serial oct after a single injection . however , these effects do not appear to persist beyond 34 months without repeated administration.5 a medline search using keywords pregnancy revealed no case reports or case series documenting the treatment of clinically significant macular edema with intravitreal corticosteroids in a pregnant patient . side effects of systemic corticosteroid administration are well known . major ophthalmic complications of intravitreal corticosteroid injection include , but are not limited to , cataract formation and increased intraocular pressure . although corticosteroid equivalents can be measured in the aqueous three months after a single intravitreal injection , it is not known how much corticosteroid is released into the systemic circulation after a single intravitreal injection.6 thus , it is hard to estimate the systemic effects of intravitreal corticosteroid therapy . to date , there are no reports of teratogenic outcomes with systemic corticosteroid use in pregnant human females . however , teratogenic effects have been observed in many species receiving equivalent systemic human doses.7 many authors have noted maxillofacial deformity and , in particular , cleft palate in mice which have received corticosteroids early in gestation . the area of greatest thickening was within the fovea of both the right and left eyes , with little edema observed in the extrafoveal area . laser photocoagulation to this area carried a significant risk of inducing a permanent central scotoma . given the patient s late stage of pregnancy , we felt that intravitreal steroid posed little risk to either mother or fetus . we were able to achieve resolution of macular edema and improved visual acuity with a single intravitreal dose to each eye . in conclusion , we propose that intravitreal triamcinolone injection may be a viable treatment modality for management of clinically significant macular edema in pregnant patients . the safety profile with administration of this medication is enhanced if the steroid is administered after the first trimester . we suggest a multidisciplinary approach and consultation with an obstetrician whenever using corticosteroid therapy in pregnant patients .
we present a case of diabetic macular edema in a pregnant patient treated with a single intravitreal injection of triamcinolone acetonomide . initial presentation and serial examinations after treatment included visual acuity , slit - lamp examination , indirect ophthalmoscopy , and optical coherence tomography . resolution of visual acuity and macular edema were present six weeks after injection and persisted throughout the duration of the pregnancy without further intervention . no adverse outcomes for either mother or fetus were noted . to our knowledge , this is the first report of intravitreal triamcinolone administration in this patient population to be published in the medical literature .
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idiopathic gingival fibromatosis ( igf ) is an uncommon , benign , hereditary , slowly progressive , nonhemorrhagic fibrous enlargement of keratinized gingiva . it usually begins at the time of eruption of permanent teeth , but can develop with the eruption of deciduous dentition and rarely present at birth . the hyperplastic gingival tissue is pale - pink , firm ; has leathery consistency and presents a characteristic pebbled surface . the enlarged tissues may partially or totally cover the dental crowns , can cause diastemas , delay or impede tooth eruption and periodontitis . in severe cases , it may lead to mastication and speech impediments or lip closure difficulties . an 18-year - old male , accompanied by his father reported to the department with the complaint of enlarged gums in upper and lower arches , which caused difficulties in speech , mastication and complete closure of lips , thereby leading to esthetic impairment . the patient presented with gradual and progressive enlargement of both upper and lower gingival tissues from the age of 6 years . the patient exhibited no signs of hypertrichosis , mental retardation , epilepsy or intake of medication known to cause gingival overgrowth . on examination , patient had bilaterally symmetrical face with incompetent lips with the bimaxillary protrusion . the gingiva was pink in color with melanin pigmentation , and consistency was firm and fibrous . the enlarged gingiva covered the crowns of all the teeth till the incisal or occlusal third region [ figure 1 ] . dentition revealed all permanent teeth except mandibular premolar ( 35 ) and an over retained deciduous molar ( 75 ) . preoperative photographs showing generalized gingival enlargement investigations included orthopantomograph , in which no significant alveolar bone loss was observed [ figure 2 ] . laboratory investigations which included complete hemogram , thyroid tests ( t3 , t4 , and thyroid stimulating hormone ) , parathyroid test , calcium and alkaline phosphatase levels were made . all the reports except the levels of alkaline phosphatase ( which was slightly increased ) were within the physiological limits . panoramic radiograph showing presence of retained deciduous molar in mandibular left region the treatment plan consisted of sextant wise surgical excision of the enlarged gingiva under local anesthesia . the treatment procedure was explained to the patient and parent , and written consent was obtained . ledge and wedge procedure was done to remove gingival overgrowth over the palatal aspect of the maxillary posterior region and mandibular posterior region [ figure 3 ] . intrasurgical photographs histopathology showed parakeratinized stratified epithelium , which was acanthotic at places and elongated rete pegs . the bulk of connective tissue was composed of dense fibrous connective tissue and numerous fibroblasts . histologic section shows hyperparakeratinized hyperplastic squamous epithelium with dense fibrocollagenous tissue the patient was recalled every week for 6 weeks when the periodontal pack was removed , and the next sextant was operated upon . the patient was then placed on a schedule of periodic recall visits for maintenance care . no recurrence of gingival enlargement was observed 6 months after the surgery [ figure 5 ] . gingival fibromatosis may occur as an inherited condition known as hereditary gingival fibromatosis , or it may be associated with inflammation , leukemic infiltration , and medications . according to gorlin igf is most commonly associated with hypertrichosis , also occasionally associated with mental retardation and epilepsy . it occurs either as an isolated disease or combined with some rare syndromes like zimmerman - laband syndrome ( defects of bone , nail , ear , nose and splenomegaly ) , murray - puretic - drescher syndrome ( multiple dental hyaline tumors ) , rutherford syndrome ( corneal dystrophy ) , cowden syndrome ( multiple hamartomas ) , and cross syndrome ( hypopigmentation with athetosis ) . in the present case , the patient exhibited no signs of hypertrichosis , mental retardation and epilepsy or intake of medication known to cause enlargement . idiopathic gingival fibromatosis affects the attached gingiva as well as gingival margin and interdental papillae . the cause is unknown , and thus the condition is designated as idiopathic . some cases have a hereditary basis , but the genetic mechanisms involved are not well understood . a study of several families found the mode of inheritance to be autosomal recessive in some cases and autosomal dominant in others . histologically , the gingival hyperplasia is mainly due to an increase and thickening of collagen bundles in connective tissue stroma . igf keratinocytes seem to have an important role in pathogenesis by inducing extracellular matrix accumulation by fibroblasts . furthermore , it has been reported that increased proliferation and elevated production of extracellular matrix molecules , fibronectin and type i collagen could lead to an increased bulk of gingiva . in the present case , the history revealed that the enlargement started in mixed dentition period . emerson recommended that the best time for the excision of gingival enlargement is when all the permanent teeth have erupted . among the suggested treatment protocols , ledge and wedge technique along with internal bevel gingivectomy was selected as it helped in the placement of primary incisions as opposed to the conventional external bevel gingivectomy procedure . moreover , this procedure does not leave a large external bevel and , therefore , result in less postoperative pain and bleeding . after surgery , recurrence is expected within a few months after surgery and is most commonly seen in children and teenagers rather than adults . maintenance of good oral hygiene with professional cleaning and home care maintenance is necessary to prevent the recurrence .
idiopathic gingival fibromatosis ( igf ) is a rare hereditary condition characterized by slowly progressive , nonhemorrhagic , fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements , mostly associated with some syndrome . this case report describes a case of nonsyndromic generalized igf in an 18-year - old male patient who presented with generalized gingival enlargement . the enlarged tissue was surgically removed by internal bevel gingivectomy and ledge and wedge procedure . the patient was regularly monitored clinically for improvement in his periodontal condition as well as for any recurrence of gingival overgrowth .
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a recent study demonstrated that a pest - like sequence present at the nh2 terminus of llo and absent in pfo is responsible for the rapid degradation of llo in the host cell cytosol ( decatur and portnoy , 2000 ) . pest - like sequences are thought to target eukaryotic proteins for phosphorylation and degradation , and deletion or specific amino acid substitution of this sequence in llo led to increased cytotoxicity and lower virulence in a mouse model . when the sequence was introduced in pfo and the chimeric toxin expressed in l. monocytogenes , bacteria were less toxic than those expressing wild - type pfo and were able to multiply intracellularly in j774 macrophages . thus , introduction of a pest motif in llo is a strategy used by l. monocytogenes to restrict the activity of this powerful toxin to the host cell vacuole , thereby preserving the intracellular niche for bacterial multiplication . intriguingly , another report ( lety et al . , 2001 ) has also shown that l. monocytogenes mutants expressing a pest - deleted hly allele , although fully hemolytic , are strongly impaired in virulence in a mouse model . however , in contrast to the previous report , in which j774 nonbactericidal macrophages were used , the authors of this second report used bone marrow derived macrophages and showed that pest - deleted or -substituted mutants were unable to escape from the phagocytic vacuole raising the interesting possibility that the pest motif may play different roles in different cells . direct evidence for a link between the optimum activity at low ph and compartment - specific pore - forming activity of llo has been provided by a recent study using a ph - sensitive and membrane - impermeant fluorophore 8-hydroxy - pyrene-1,3,6-trisulfonic acid ( hpts ) . it was shown that l. monocytogenes containing phagosomes ( average ph 5.9 ) rapidly acidify after bacterial uptake , followed by an increase in ph and dye release from the vacuole . perforation of the vacuole was inhibited by lysosomotropic agents , such as ammonium chloride and bafilomycin a1 ( beauregard et al . , 1997 ) . bafilomycin a1 was also shown to inhibit l. monocytogenes escape from the primary vacuole in epithelial cells ( conte et al . , 1996 ) . these experiments indicate that llo activity is maximal in the phagosome lumen , requires this low ph for activity and leads to membrane disruption , increasing ph , and inactivation of llo activity , an auto - switch process . in their recent study , glomski et al . ( 2002 ) , by swapping dissimilar residues from the ph - insensitive ortholog perfringolysin o into llo , identified leucine 461 of llo as a key residue responsible for the low optimum ph activity of llo . changing this residue to threonine results in a molecule highly active at ph 7 . if one assumes that llo shares with pfo , a general structure similarity , the l461 t mutation is located in the outer loop of the fourth domain ( fig . 1 ) . that a single amino acid residue is sufficient to increase the activity at ph 7.0 is quite astonishing , although it is important to note that activity at ph 5.0 is also increased in the mutant . how this single amino acid change llo l461 t affects ph sensitivity thus awaits further biochemical analysis . interestingly , the mutation that altered the optimum ph activity of pfo l462f described above was located in the conserved undecapeptide , which is very close to the region of the corresponding l461 t mutation ( jones et al . , 1996 ) . it is particularly worth noting that while the llo l461 t mutant can efficiently permeabilize the host cell membrane from the cytosolic compartment , it only promotes efficient escape from the vacuolar compartment if this latter has been acidified , suggesting that additional bacterial or host factors activated by low ph are needed to act in concert with llo to mediate escape from the phagosomal compartment . these findings could also reflect the difference in lipid composition of the internal versus external leaflets of the plasma membrane lipid bilayer , resulting in a difference in the behavior of llo depending on the nature of the first leaflet with which it is in contact . while it is clear that llo is largely responsible for mediating escape from the vacuole in most cell lines , two l. monocytogenes plcs also play a role . mutants lacking both the broad - range plc ( plcb ) and a phosphatidylinositol - specific plc ( plca ) escape from a macrophage vacuole at 50% of the efficiency of the wild - type . plcb can even replace llo in some cell lines , such as the human epithelial cell lines hela and henle 407 ( vazquez - boland et al . optimal pore formation occurs between ph 5.5 and 6.0 , the ph of an early endosome . one consequence of the pore formation is an elevation of the vacuolar ph , which may prevent vacuolar maturation , thus allowing the l. monocytogenes plcs , perhaps in concert with additional host factors , to mediate vacuolar dissolution . llo activity is then progressively switched off and degraded and bacterial multiplication can take place in the host cytosol . meanwhile , llo may have induced a series of signaling events since it is now well established that in addition to its role in escape from the phagosomal vacuole , llo is one of the most potent l. monocytogenes signal - inducing molecules ( vazquez - boland et al . , 2001 ) .
cholesterol - dependent cytolysins ( cdcs ) * are produced by a large number of pathogenic gram positive bacteria . a member of this family , listeriolysin o ( llo ) , is produced by the intracellular pathogen listeria monocytogenes . a unique feature of llo is its low optimal ph activity ( 6 ) which permits escape of the bacterium from the phagosome into the host cell cytosol without damaging the plasma membrane of the infected cell . in a recent study ( glomski et al . , 2002 , this issue ) , portnoy 's group has addressed the molecular mechanism underlying the ph sensitivity of llo . unexpectedly , a single amino acid substitution in llo l461 t results in a molecule more active at neutral ph and promoting premature permeabilization of the infected cells , leading to attenuated virulence . this finding highlights how subtle changes in proteins can be exploited by bacterial pathogens to establish and maintain the integrity of their specific niches .
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pseudoaneurysms and arteriovenous fistulas ( avf ) associated with the internal pudendal artery are unusual . we report the first case of recurrent haematuria in postoperative period caused by internal pudendal artery pseudoaneurysm with avf secondary to transvesical prostatic adenomectomy ( tpa ) . there were no intraoperative complications . on the 5 postoperative day , after removal of the foley catheter , the patient presented recurrent episodes of gross hematuria with a bladder full of clots and anemia that required blood transfusion . cystoscopy for clot evacuation showed a haematoma caused by arterial bleeding from the left side of the prostatic fossa . the patient underwent computed tomography ( ct ) , which revealed a 2.2 1.5 cm hypervascular lesion matching the density of the adjacent iliac vessels on the left side of the residual prostate gland [ figure 1 ] . a 4 french ( fr ) angiographic catheter was inserted via the left femoral artery and left internal iliac arteriography showed a pseudoaneurysm approximately 2 cm in size with venous filling during the early arterial phase , located at the distal portion of the left internal pudendal artery suggesting an avf [ figure 2a ] . the left internal pudendal artery was catheterized super selectively with a 2.7 fr microcatheter and embolization was carried out using two micro coils 3 mm 40 mm [ figure 2b ] . after the procedure , the patient had no hematuria and was discharged after 3 days . at 6 months pelvic computed tomography sagittal image with contrast shows a 2.2 cm 1.5 cm hypervascular component matching the density of the adjacent iliac vessels ( yellow arrow ) near the left residual prostate gland ( a ) selective left internal iliac artery injection revealed a pseudoaneurysm ( yellow arrow ) associated with arteriovenous fistulas ( venous filling in the early arterial phase ) ; ( b ) the left internal pudendal artery was catheterized ( 2.7 french microcatheter ) and embolized with microcoils ( yellow arrow ) a pseudoaneurysm is a single layer of fibrous tissue shaped like a sac containing a turbulent blood flow . arterial pseudoaneurysms are formed after disruption in the vascular wall continuity and may be caused by trauma , inflammation , cancers , and iatrogenic causes . the main iatrogenic causes are access for endovascular procedures , surgical procedures , and biopsies . the major complications of pseudoaneurysms are rupture , embolization of a thrombus , infection , pain , and compression of adjacent structures . we identified 6 published cases in the literature describing the management of this rare complication after radical prostatectomy or transurethral resection of the prostate . however , angiography is the reference standard for diagnosis and has the advantage of allowing simultanneosu therapeutic intervention . this case report confirms that trans - arterial embolization is an efficient way of managing postoperative bleeding due to prostatic surgery , preventing further surgical exploration .
pseudoaneurysms associated with the internal pudendal artery is rare and may occur as a complication of prostatic surgery or or pelvic trauma . we present images of the first case in literature of an isolated pseudoaneurysm secondary to transvesical prostatic adenomectomy , which was successfully treated by transarterial coil embolization . this complication can be difficult to diagnose , manage , and cause significant postoperative bleeding . management requires as a multidisciplinary approach .
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scientific literature has grown tremendously in past five decades in volume as well as content . this communication enlightens young dentist and scientist about availability of scientific literature in digital format and on the web since it has become impossible to maintain literature in printed format due to sheer volume and content . this article was primarily written to create awareness among young dentists and orthodontists the availability of varied orthodontic literature and contributors and journals in disseminating knowledge and opportunities . this article was primarily written to create awareness among young dentists and orthodontists the availability of varied orthodontic literature and contributors and journals in disseminating knowledge and opportunities . to quote dr . thomas m. graber , who in his preface of the orthodontic book said , this labor of love by our outstanding panel of world - class orthodontists is strictly that love of our wonderful dental specialty and the desire to help others attain that achievable optimum with maximum health and stability . read , enjoy , and profit yourself by rendering the best possible patient care !
background : scientific literature has grown tremendously in past five decades in volume as well as content . this communication enlightens young dentist and scientist about availability of scientific literature in digital format and on the web since it has become impossible to maintain literature in printed format due to sheer volume and content.materials and methods : the key words were searched in www.google.com.result:out of 3500 results relevant web pages was selected by the authors unanimously.conclusion:this article was primarily written to create awareness among young dentists and orthodontists the availability of varied orthodontic literature and contributors and journals in disseminating knowledge and opportunities .
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dacron graft is a successful and commonly used vascular conduit for many arterial diseases . despite its proven durability and long - term performance , structural abnormality secondary to mechanical defect or pressure dynamics of blood have been reported resulting in graft aneurysm and failure . however , a dacron graft aneurysm with dissection has not been reported . therefore , we present an abdominal aortic bypass graft failure with unusual presentation of aneurysm with dissection . a 30-year - old female , with history of takayasu arteritis underwent aortic bypass graft surgery . computed tomography ( ct ) abdominal aortic angiogram revealed the following findings : circumferential wall thickening and stenosis of suprarenal abdominal aorta with celiac trunk occlusion [ figure 1 ] . similarly , stenosis of infrarenal abdominal aorta demonstrated with superior mesenteric artery occlusion [ figure 3 ] . distal superior mesenteric artery was reformed by dilated , tortuous arc of riolan [ figure 4 ] . dacron bypass graft visualized anastomosing supraceliac and suprainferior mesenteric abdominal aorta [ figure 4 ] . 31 34 mm aneurysmal dilatation of distal aortic graft noted with incomplete flap , creating true and false lumen [ figure 5 ] . in addition , a pedunculated aneurysm measuring 17 14 mm from the proximal graft noted [ figure 4 ] . left renal artery stenosis , revascularized by great saphenous vein from the dacron graft . distal abdominal aorta and iliac braches appear normal . computed tomography angioram showing circumferential wall thickening of abdominal aorta causing narrowing of the lumen with occlusion of the celiac trunk ( arrow ) . satisfactory opacification of aortic bypass graft seen ( arrowhead ) coronal reformatted maximum intensity projection showing arc of buhler collateral ( red arrow ) anastomosing superior mesenteric artery ( green arrow ) and celiac artery ( blue arrow ) computed tomography angiogram showing occlusion of superior mesenteric artery ( arrow ) three - dimensional volume rendered image showing dilated , tortous arc of riolan ( white arrow ) anastomosing superior ( arrowhead ) and inferior mesenteric artery . aneurysmal dacron graft ( red arrow ) with pedunculated aneurysm from the proximal dacron graft ( yellow arrow ) computed tomography angiogram showing abdominal aorta ( arrowhead ) and aneurysmal dacron graft with flap ( arrow ) dacron aortic graft is the most widely used and reliable substitute for arterial replacement . dacron graft ( polyethylene terephthalate ) modern day dacron grafts are of woven type , which has high tensile strength , resistance to stretching , and degradation to abrasion and chemicals . however , there are few reports indicating dilatation and expansion of dacron graft after implantation . in our patient , an incomplete flap demonstrated within the aneurysmal segment , consistent with dissection within the layers of wall of the graft . this could be attributed to mechanical property changes such as fiber stretching , structural damage to the integrity of the graft due to instrument handling , and distortion of graft from hydrostatic pressure . to the best of our knowledge after extensive literature search , none reported with this kind of presentation . pedunculated aneurysm of the graft can be explained as a result of localized areas of microscopic structural damage due to manufacturing errors , inappropriate storage conditions , material fatigue , and biodegradation . arc of riolan , also known as meandering mesenteric artery , is a collateral channel between superior ( sma ) and inferior mesenteric artery ( i m a ) in cases of stenosis of occlusion of either of the arteries . in our case , the occluded sma is reformed by arc of riolan from i m a , which is dilated presumably due to hyperdynamic flow . the celiac artery is reformed by the arc of buhler , which is a collateral channel between the celiac artery and sma . in conclusion , we recommend long - term cross - sectional imaging for the follow - up of dacron graft recipients to monitor the integrity of the graft and guide vascular surgeons to intervene , if necessary to avoid lethal complications . ct angiography with multiplanar reconstruction , maximum intensity projections , and volume rendering aids in easy identification of aneurysm , vascular stenosis , and collateral channels . all procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee . this article does not contain any studies with animals performed by any of the authors . all procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee . this article does not contain any studies with animals performed by any of the authors .
dacron grafts have been used as a conduit for large caliber arteries for many years successfully . however , these grafts can undergo complications such as aneurysm formation , rupture , and failure . evaluation of these complications are of paramount importance because of its tendency to rupture and cause death . imaging plays an important role in identifying and monitoring of these complications , and also provides a road map to the vascular surgeons for early intervention and revascularization .
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green nail syndrome ( gns ) is characterized by greenish chromonychia caused by pyocyanin , a metabolite produced by p. aeruginosa . predisposing factors are onychomycoses , nail diseases , working in wet conditions , diabetes mellitus , paronychia and immunosuppression , among others [ 1 , 4 , 5 ] . p. aeruginosa can be transmitted from gns to wounds or surgical sites [ 6 , 7 ] and thus lead to local and possibly systemic complications in immunocompromised individuals . reported treatment options are systemic fluoroquinolones , topical octenidine 0.1% , topical aminoglycoside and nail extraction [ 1 , 8 ] . moreover , three patients with gns successfully treated with topical nadifloxacin have been reported [ 3 , 4 ] . nadifloxacin is a topical fluoroquinolone approved for the treatment of acne vulgaris in some countries ( germany , italy , spain , china , japan and india , among others ) . it has a broad - spectrum activity against gram - positive ( including methicillin - resistant staphylococcus aureus ) , gram - negative and anaerobic bacteria . hence in some countries ( e.g. india , japan ) it is also approved for the treatment of skin infections . a 64-year - old otherwise healthy female developed an asymptomatic greenish discoloration of the nail plate of the right middle finger within 2 months . treatment with topical acetic acid and ethyl lactate during 1 month showed no effect . at first visit a greenish discoloration and mild onychodystrophy of the entire nail plate with distal onycholysis exclusively on the right middle finger koh preparation ( direct mycological microscopy ) of nail scrapings was negative , but the culture was positive for candida parapsilosis . nadifloxacin ( nadixa cream ) was applied once daily on the nail plate for 6 weeks . the coinfection with c. parapsilosis was treated with itraconazole 100 mg twice daily on weeks 1 and 5 . the nail plate was cured within 6 months after the initiation of treatment ( fig . 2 ) . a 49-year - old female with bulimia suffered from recurrent onycholysis of the big toes due to tight shoes . moreover a yellow - greenish discoloration persisted for several weeks on the right big toe . lateral yellow - greenish discoloration , distal onychodystrophy and onycholysis exclusively on the right big toe without signs of paronychia ( fig . nadifloxacin ( nadixa cream ) was applied once daily on the nail plate for 6 weeks . the nail plate was cured within 4 months after the initiation of treatment ( fig . a 64-year - old otherwise healthy female developed an asymptomatic greenish discoloration of the nail plate of the right middle finger within 2 months . treatment with topical acetic acid and ethyl lactate during 1 month showed no effect . at first visit a greenish discoloration and mild onychodystrophy of the entire nail plate with distal onycholysis exclusively on the right middle finger koh preparation ( direct mycological microscopy ) of nail scrapings was negative , but the culture was positive for candida parapsilosis . nadifloxacin ( nadixa cream ) was applied once daily on the nail plate for 6 weeks . the coinfection with c. parapsilosis was treated with itraconazole 100 mg twice daily on weeks 1 and 5 . the nail plate was cured within 6 months after the initiation of treatment ( fig . a 49-year - old female with bulimia suffered from recurrent onycholysis of the big toes due to tight shoes . moreover a yellow - greenish discoloration persisted for several weeks on the right big toe . lateral yellow - greenish discoloration , distal onychodystrophy and onycholysis exclusively on the right big toe without signs of paronychia ( fig . nadifloxacin ( nadixa cream ) was applied once daily on the nail plate for 6 weeks . the nail plate was cured within 4 months after the initiation of treatment ( fig . the clinical presentation of these two cases with p. aeruginosa nail infection in only one nail is typical . in both cases a predisposing factor was prevalent ( onychomycosis in case 1 , nail trauma in case 2 ) . the observation of successful treatment with nadifloxacin is in line with previous reports [ 2 , 3 , 4 ] . easy - to - handle , effective , nail - preserving and low - priced treatment option of gns . as nadifloxacin is not approved for this indication , insurance confirmation of coverage should be provided . pseudomonas is not sensitive to itraconazole , hence the treatment effect on gns in case 1 can be attributed to nadifloxacin . however , the treatment of the coinfection ( c. parapsilosis ) with itraconazole in this case contributed to the cure of the nail . thus , screening for fungal coinfections and if needed antifungal treatment in patients with gns is crucial .
green nail syndrome ( gns ) caused by pseudomonas aeruginosa is the most common bacterial nail infection . the treatment of gns is challenging in many cases and recommendations based on clinical trials are lacking . we report two cases with gns successfully treated with off - label use of topical nadifloxacin , a fluoroquinolone approved for acne and bacterial skin infections in some countries .
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/ a22c / w184a / m185a or p17c / r18l / t19c / w184a / m185a mutations ) were prepared by sequential dialysis of purified protein . crystals were obtained by the sitting - drop vapor diffusion method in tris - buffered precipitant solutions containing polyethylene glycol and sodium iodide . molecular replacement phasing , model building , and crystallographic refinement were performed with the programs molrep , coot , and phenix . the capsid model was built by manual rigid - body docking of the high - resolution structures of the 5-fold symmetric ntd ring ( pdb code 3p05 ) , the 6-fold symmetric ntd ring ( 3h47 ) , and 2-fold symmetric ctd dimers ( 2kod and 1a43 ) into a geometric fullerene cone model .
the mature capsids of human immunodeficiency virus type 1 ( hiv-1 ) and other retroviruses are fullerene shells , composed of the viral ca protein , that enclose the viral genome and facilitate its delivery into new host cells1 . retroviral ca proteins contain independently - folded n - terminal and c - terminal domains ( ntd and ctd ) that are connected by a flexible linker24 . the ntd forms either hexameric or pentameric rings , whereas the ctd forms symmetric homodimers that connect the rings into a hexagonal lattice3,513 . we previously used a disulfide crosslinking strategy to enable isolation and crystallization of soluble hiv-1 ca hexamers11,14 . by the same approach , we have now determined the x - ray structure of the hiv-1 ca pentamer at 2.5 resolution . two mutant ca proteins with engineered disulfides at different positions ( p17c / t19c and n21c / a22c ) converged onto the same quaternary structure , indicating that the disulfide - crosslinked proteins recapitulate the structure of the native pentamer . assembly of the quasi - equivalent hexamers and pentamers requires remarkably subtle rearrangements in subunit interactions , and appears to be controlled by an electrostatic switch that favors hexamers over pentamers . this study completes the gallery of sub - structures describing the components of the hiv-1 capsid and enables atomic level modeling of the complete capsid . rigid - body rotations around two assembly interfaces appear sufficient to generate the full range of continuously varying lattice curvature in the fullerene cone .
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fasting during the light phase reduced oxygen consumption in female mice fed either a high - fat or standard diet ( sd ) for 3 days ( fig . 1b ) , but during the dark phase , fasting only reduced oxygen consumption when mice were fed a background high - fat diet ( fig . 1a ) . in both the dark and light phases , fasted mice fed a high - fat diet ( hfd ) had reduced oxygen consumption when compared to fasted mice fed sd ( fig . 1 ) . fasting significantly reduced the respiratory exchange ratio ( rer ) in mice fed either background diet , and consuming a hfd resulted in a lower non - fasted rer than consuming a sd , regardless of the phase of study ( fig . 2 ) . fasted mice fed a sd had increased activity during the dark cycle compared to non - fasted mice , although these data do not show a similar increase in activity during the dark cycle with fasting when mice are given a background hfd ( fig . these data show no significant differences between dietary or fasting / non - fasting groups with regards to activity ( fig . all animal procedures were approved by the university of waterloo animal care committee and were in accordance with the guidelines of the canadian council on animal care . group - housed mice were maintained on a reverse light - dark cycle and fed a defined standard diet ( sd ) ( cat # d12450h ( 10 kcal% fat , 70 kcal% carbohydrate , 20 kcal% protein ) from research diets , new jersey , usa ) for 1 week , prior to randomization to one of two groups . mice in the hfd group were instead provided with a diet containing 45 kcal% fat , 35 kcal% carbohydrate , and 20 kcal% protein ( cat#d12451 from research diets , new jersey , usa ) . soybean oil and lard provided the fat sources for the diets , while corn starch , maltodextrin and sucrose provided the carbohydrate source , and casein provided the protein source . complete diet compositional data are available from the manufacturer . on the second day of feeding , mice were placed into individual cages in the oxymax comprehensive laboratory animal monitoring system ( clams , columbus instruments ) for an additional 24 h. fasted mice had food withdrawn for ~12 h , from midway through the light cycle to midway through the dark cycle , and data shown are from these time periods for both fasted and non - fasted mice . the oxymax system is an open - circuit indirect calorimeter for lab animal research allowing the measurement of oxygen consumption ( vo2 ) , respiratory exchange ratio ( rer ) and activity levels of mice . oxygen consumption ( vo2 ) is a measure of the volume of oxygen used to convert energy substrate into atp . respiratory exchange ratio ( rer ) is the ratio of carbon dioxide production ( vco2 ) divided by oxygen consumption , and can be used to estimate the fuel source for energy production based on the difference in the number of oxygen molecules required for the oxidation of glucose versus fatty acids . an rer of 0.7 indicates that fatty acids are the primary substrate for oxidative metabolism , while an rer of 1.0 indicates that carbohydrate is the primary energy substrate . activity was calculated by summing the x - axis movement counts associated with horizontal movement . all animal procedures were approved by the university of waterloo animal care committee and were in accordance with the guidelines of the canadian council on animal care . group - housed mice were maintained on a reverse light - dark cycle and fed a defined standard diet ( sd ) ( cat # d12450h ( 10 kcal% fat , 70 kcal% carbohydrate , 20 kcal% protein ) from research diets , new jersey , usa ) for 1 week , prior to randomization to one of two groups . mice in the hfd group were instead provided with a diet containing 45 kcal% fat , 35 kcal% carbohydrate , and 20 kcal% protein ( cat#d12451 from research diets , new jersey , usa ) . soybean oil and lard provided the fat sources for the diets , while corn starch , maltodextrin and sucrose provided the carbohydrate source , and casein provided the protein source . complete diet compositional data are available from the manufacturer . on the second day of feeding , mice were placed into individual cages in the oxymax comprehensive laboratory animal monitoring system ( clams , columbus instruments ) for an additional 24 h. fasted mice had food withdrawn for ~12 h , from midway through the light cycle to midway through the dark cycle , and data shown are from these time periods for both fasted and non - fasted mice . the oxymax system is an open - circuit indirect calorimeter for lab animal research allowing the measurement of oxygen consumption ( vo2 ) , respiratory exchange ratio ( rer ) and activity levels of mice . oxygen consumption ( vo2 ) is a measure of the volume of oxygen used to convert energy substrate into atp . respiratory exchange ratio ( rer ) is the ratio of carbon dioxide production ( vco2 ) divided by oxygen consumption , and can be used to estimate the fuel source for energy production based on the difference in the number of oxygen molecules required for the oxidation of glucose versus fatty acids . an rer of 0.7 indicates that fatty acids are the primary substrate for oxidative metabolism , while an rer of 1.0 indicates that carbohydrate is the primary energy substrate . activity was calculated by summing the x - axis movement counts associated with horizontal movement .
whole animal physiological measures were assessed following three days of either standard diet or high fat diet , in either the fasted or non - fasted states . our data shows that acute 3-day high fat feeding increases whole body lipid oxidation . when this feeding protocol is followed by an overnight fast , oxygen consumption ( vo2 ) in the light phase is reduced in both dietary groups , but oxygen consumption in the dark phase is only reduced in mice fed the high - fat diet . furthermore , the fasting - induced rise in dark cycle activity level observed in mice maintained on a standard diet is abolished when mice are fed a high - fat diet .
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with an increasing number of laparoscopic cholecystectomy performed , studies have reported gallbladder perforation of up to 40% of cases . we present our case and describe laparoscopic drainage as our choice of management for chronic abscess caused by these spilled stones . mr m is a 75-year - old male who required repeated admission for right upper quadrant pain post - laparoscopic cholecystectomy . during his initial operation , a chronically inflamed gallbladder was encountered . multiple small gallstones in the gallbladder were present with iatrogenic perforation and spillage of stones . an endo - bag was used to remove the gallbladder . a further washout he had a raised white cells count of 15 and an elevated c - reactive protein ( crp ) of 110 . a computer tomography ( ct ) abdomen ( fig . 1 ) was performed , which showed numerous calcific densities that were surrounded by soft tissue thickening and stranding . he was admitted for 2 days on intravenous ( iv ) antibiotics then discharged . he then represented again 2 weeks later with ongoing pain and a raised white cells count of 16 and crp of 48 . a repeat ct was performed , which found a number of retained calculi , now lying at the postero he was admitted for 5 days of iv antibiotics . on discharge , he required regular visits to his general practitioner for antibiotics . he was seen at the clinic as a follow - up for his ongoing right upper quadrant pain . a magnetic resonance imaging ( mri ) abdomen ( fig . 2 ) was performed , which showed small , oval - shaped , thick wall collection anterior to the capsule of the liver . therefore , 6 months after his initial laparoscopic cholecystectomy , he proceeded to an exploratory laparoscopy . we found an abscess cavity measuring 3 4 cm over the anterior abdominal wall ( fig . he was discharged the day after with 1 week of oral antibiotics . figure 3:laparoscopic view of the chronic abscess . complications caused by stone spillage post - cholecystectomy are rare with a rate of 1.7 complications per 1000 cases . zehetner reviewed all reported complications from stone spillage and found that abdominal wall abscess and intra - abdominal abscesses were the most common complications . our patient presented multiple times with abdominal pain secondary to an abscess from spilled stones . other potential stone - related complications include adhesions , fistula , obstruction , intestinal perforation and pleural empyema . management of an abscess due to spilled gallstones includes draining the abscess and intravenous antibiotics . laparoscopic approach is optimal as the abscess cavity can be clearly identified , stones visualized and removed under direct vision . every attempt should be made to avoid spillage of gallstones during the initial surgery including careful dissection and identification of the correct planes . extra care taken at this stage of the operation can save many minutes attempting to retrieve spilled stones later . the surgeon should attempt to remove all visible stones at the time of surgery and use copious irrigation . it is also advisable that we should include this complication on our list of differential diagnoses when an unusual abdominal wall or cavity abscess develops in a post - cholecystectomy patient . if sizeable stones are seen on ct during the initial presentation , it should have been dealt with fairly early as the symptomatic stones are highly unlikely to resolve on its own . management with laparoscopic drainage and retrieval of stones is feasible for some of these patients .
we present a case on abdominal wall abscess from spilled stones post - cholecystectomy and describe laparoscopic drainage as our choice of management . mr m is a 75-year - old male who presented on multiple occasions to the hospital with right upper quadrant pain and fever post - laparoscopic cholecystectomy . he also required multiple courses of antibiotics . subsequent computed tomography and magnetic resonance imaging scan confirmed a number of retained stone with signs of chronic inflammation . hence , 6 months after his initial laparoscopic cholecystectomy , he proceeded to an exploratory laparoscopy . we found an abscess cavity measuring 3 4 cm over the anterior abdominal wall . the cavity was de - roofed , drained and washed out . the tissue culture grew klebsiella pneumoniae . laparoscopic approach is optimal as the abscess cavity can be clearly identified , stones visualized and removed under direct vision . patient does not require a laparotomy .
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congenital adrenal hyperplasia ( cah ) refers to a group of autosomal recessive disorders caused by an enzyme deficiency which leads to defects in biosynthesis of steroid precursors . depending on the severity and degree of 21 hydroxylase deficiency , the clinical spectrum may vary from mild form of non classical cah to classic cah . however , the non classical cah variant is more common with a prevalence rate of 1 in 1000 . it also helps in maintaining normal levels of precursors by suppressing adreno cortico trophic hormone ( acth ) . during childhood , the management is largely focused on achieving normal growth and attaining appropriate final adult height . johns medical college hospital , bangalore by the department of endocrinology on patients diagnosed to have cah and seen in the outpatient clinic between january 2012 and october 2012 . during this period data regarding demography , clinical presentation at time of diagnosis , treatment details , height sds and bmi were collected . all patients underwent biochemical testing for 17 hydroxy progesterone ( 17 ohp ) levels for assessment of adequacy of therapy . bone age assessment with left hand and wrist bmi was calculated for all patients and obesity was defined using who charts as values above 95th percentile . 17 ohp levels between 1 ng / ml and 12 ng / ml were considered appropriate ; values below 1 ng / ml suggested suppression and over treatment and values above 12 ng / ml suggested under treatment . 29 patients were included in the study of which 22 were females ( 76% ) and 7 were males ( 24% ) . based on the cross sectional data collected , 11 patients were adults ( age > 18 ) and 18 patients were children ( 62% ) . among the males , one child was identified at birth via a neonatal screening program , one child presented with early pubarche , the other 5 infants presented between their 3rd and 6th week of life with features suggestive of adrenal crisis - poor feeding , vomiting and failure to gain weight . one of the male patients incidentally also had a penoscrotal hypospadias which was surgically corrected . among the females , 9 infants were identified at birth due to presence of genital ambiguity ( 40% ) , 1 presented with symptoms of adrenal crisis at 4 weeks of life , 4 patients presented in the pre pubertal period due to early onset adrenarche ( 18% ) , 5 patients presented in the late adolescent period with marked virilization ( 23% ) and 3 patients presented with features of poly cystic ovarian disease ( pcos ) . all the five patients who presented in the late adolescent period had obvious genital ambiguity from birth , however , they sought medical attention only much later due to marked virilization and failure to attain menarche . one among them had actually been evaluated and even underwent a clitoroplasty at a young age , but unfortunately , the diagnosis of cah was missed as she was mistakenly categorized as probable ovo testicular dsd due to presence of mullerian structures with a phallic length of around 6 cm . all these 5 women had a masculine built at presentation with poor breast development , severe hirsutism , muscular body habitus , temporal balding and varying degrees of deepening of voice . the non classical cah ( nccah ) patients presented in adulthood with complaints of irregular cycles and hirsutism ; they had no features of virilisation . simple virilizing was the most common sub class seen among the women ( 81% ) whereas the salt wasting type was predominant among boys ( 85% ) . around 35% of patients had short stature as defined by height sds < -2.0 ; 2 patients who presented with adrenarche had increase in height sds , however , their bone age was also correspondingly advanced . all the children were treated with hydrocortisone ; almost all adult patients were on dexamethasone . none of the nccah patients were treated with glucocorticoids ; they were on anti - androgen therapy along with oral contraceptive pills . most adult classic cah patients with complaints of hirsutism were treated with either spironolactone or finasteride . pubertal induction with ethinyl estradiol was initiated for three of the adult patients who sought medical care late . analysis of 17 ohp levels revealed that 32% were suppressed with levels less than 1ng / ml ; appropriate in 47% and inadequate in 21% of patients . we report the phenotypic features of a cohort of patients with cah being evaluated and managed in a tertiary centre in southern india . in this series , the typical presentation of male babies were with adrenal crisis between the 3 and 6 week of life . around 20% of female patients with classic cah were identified and appropriately treated only after late adolescence even when genital ambiguity was present since birth . short stature was seen in one third of patients on therapy and the average final adult height among patients with classic cah was 142.37 , which was significantly lower than the mean adult height for females in our population . the mean final adult height of 157.5 cm for those with nccah was better than their classic cah counterpart . obesity and hypertension were not found to be significantly higher in this cohort of cah patients . one third of patients had suppressed 17 ohp levels suggestive of over replacement therapy which may also contribute to the reduction in the final adult stature .
congenital adrenal hyperplasia is a group of autosomal recessive disorders caused by enzyme deficiency which leads to defects in biosynthesis of steroid precursors . most common is 21 hydroxylase deficiency . clinical spectrum varies from non - classical cah to classic cah , and it may be simple virilising form or salt - wastinfg type . 29 patients were included in our study from january 2012 to october 2012 . 76% were females . male babies typically presented with adrenal crisis between 3rd to 6th week of life . around 20% of females were identified and appropriately treated only after late adolescence . short stature was seen in 1/3rd of patients . 1/3rd of patients had suppressed 17 ohp levels suggestive of over - replacement therapy which may contribute to final reduction in adult height .
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the femoral head often leads to healing complications , while the more predictable prosthetic replacements are associated with poorer function and significant complications . the treatment of these fractures depends on the age of the patient , fracture displacement , bone quality , timing of surgery and activity level of the patient . displaced fractures in healthy , active patients are best treated by reduction and internal fixation . there are however , complications unique to femoral neck fractures which are almost impossible to predict . non - union and avascular necrosis non - union usually can definitely diagnosed within a year of fracture fixation with the same being achieved within 3 months at times . after non - union has been established , intervention is inevitable . the decision to proceed in the management of failed fixation is based on the careful consideration of various factors . in young patients revision internal fixation with cancellous or muscle pedicle bone grafting ( vascularised bone graft ) or an osteotomy results in useful outcome . we report a case of non - union of the fracture of the neck of the femur , who refused additional procedures after his non - union had been established . the study conforms to the declaration of helsinki and was approved by the institutional ethical board . a 38-year old male businessman reported to the out door department of our hospital with a history of a fall from height . clinical and radiological examination revealed a displaced fracture of the neck of the femur which was graded as garden type 4 ( fig . the patient was operated within 24 h. intraoperatively the garden alignment index was used as a yardstick of acceptable reduction . the fracture was fixed with 3 ao 6.5 mm cannulated screws in an inverted triangle . onward the patient complained of persistent but mild groin pain on ambulation which was assisted . the patients conservative line was continued till 1 year when his symptoms continued and radiologically there was no progress . at this point twenty - six months into the post operative followup the patients pain subsided and radiographs started showing signs of union . a radiograph taken 32 months after the fixation showed full union . non - union after femoral neck fracture can be defined as a lack of radiographic evidence of union 6 months after the fracture . delayed or non - union often manifests as continued pain with weight bearing beyond 3 months post fixation . incidence of non - union of femoral neck fractures has been reported to be between 2 and 22% and generally becomes apparent within 1 year [ 69 ] . 3radiograph at 32 months depicting union garden 4 type fracture of the neck of the femur showing persistent non - union at 2 years radiograph at 32 months depicting union anatomical reduction and rigid internal fixation are the two important surgeon controlled factors that may contribute to outcome . the preferred treatment for symptomatic non - unions in the elderly is prosthetic replacement . in the young active patients , if failure is due to technical errors , revision open reduction and internal fixation may be adequate . our case demonstrates that as long as the implant is holding and the patient is regularly followed up good results might be expected in cases as far as 32 months into the post fixation period .
fracture of the femoral neck continues to be a vexing clinical and therapeutic challenge for the orthopedic surgeon . the fracture has a propensity for non - union and avascular necrosis . it is a challenge for the orthopedic surgeon to decide when to intervene in a case with non - union where the implant continues to be in place . we present a case with persistent clinical and radiological non - union signs where the fracture eventually united after 32 months . the case bolsters the view that a continued conservative regime might entail good results in such situations .
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granuloma faciale ( gf ) is an uncommon , benign , inflammatory skin disorder of unknown etiology . it is characterized by single or multiple , grey - brown or violaceous nodules or plaques primarily occurring on the face and occasionally at extra - facial sites . the disease is notoriously resistant to therapies and often tends to relapse when treatment is discontinued . we present a patient with multiple lesions of gf and its response to topical tacrolimus . a 35-year - old female presented to our department with a 5 year history of single , asymptomatic , grey - brown pigmented , nodule over the left cheek [ figure 1 ] . it started as a pin head sized papule which gradually increased to 2.5 cm 1.5 cm in size . two years later similar lesions appeared on the forehead , both arms and upper back . past and personal history was unremarkable . before treatment single , grey - brown nodule with prominent follicular orifices over left cheek . after treatment residual lesion after three months of tacrolimus application general physical and systemic examination was normal . cutaneous examination revealed multiple , well - defined , grey - brown , indurated , non - tender plaques , varying in size from 0.5 cm 0.5 cm to 1.5 cm 2.5 cm , present on the left cheek , left forehead , both arms and upper back . multiple grey - brown plaques over upper back routine hematological and biochemical investigations were normal . skin biopsy ( 4 mm ) from plaque revealed normal epidermis with clear sub epidermal grenz zone and pan dermal dense infiltrate comprising of neutrophils , lymphocytes , histiocytes and plasma cells . small dermal vessels showed infiltration of neutrophils in the vessel wall along with peri - appendageal and peri - neural infiltrate in subcutaneous fat [ figure 3 ] . ( h and e , 100 ) skin biopsy with normal epidermis and dense , mixed inflammatoey infiltrate beneath a narrow grenz zone in the dermis . infiltrate is composed of mononuclear cells with neutrophils and eosinophils she was started on intralesional triamcilone acetonide 10 mg / ml injection monthly with tab . six sessions of cryotherapy were performed once monthly after which she developed erythema and itching over the plaques and discontinued treatment . the lesions showed 40 - 50% improvement after 3 months of therapy [ figure 1 ] . classically , red - brown or violaceous nodules or plaques with associated telangiectasia and follicular accentuation are seen on the face over sun - exposed sites . differential diagnosis includes lupus pernio , lupus vulgaris , lymphoma , discoid lupus erythematosus and deep mycotic infection . skin biopsy is characterized by a mixed inflammatory infiltrate with a predominance of neutrophils and eosinophils in the dermis , in conjunction with small vessel vasculitis . there is a grenz zone that separates the infiltrate from the epidermis and pilosebaceous units . the disease is notoriously resistant to therapies and often tends to relapse once the treatment is discontinued . several medical and surgical modalities like topical and intralesional corticosteroids , cryotherapy , pulsed dye laser , puva , systemic corticosteroids , dapsone and antimalarials have been tried with variable success rates . carbon dioxide laser has also been used in a case of recurrent gf . surgical excision has been performed with often unsatisfactory results . ablative procedures may leave residual pigmentation and scarring , whereas long - term application of corticosteroids is associated with skin atrophy , telangiectasia and other possible adverse effects . in recent years successes with topical calcineurin inhibitors has been reported . several authors have reported complete or near - complete resolution of lesions after application of topical tacrolimus 0.1% ointment.[48 ] treatment regimens , duration and time to resolution of lesions have varied in these case reports [ table 1 ] . others have found time to resolution to be between 4 and 6 months . in our patient , treatment with tacrolimus 0.1% ointment twice daily for 3 months has resulted in improvement . reported cases of successful treatment of granuloma faciale ( gf ) with topical tacrolimus tacrolimus inhibits t - cell proliferation , production and release of several pro - inflammatory cytokines like interleukin-2 ( il-2 ) , il-4 , tumor necrosis factor - alpha , and interferon - gamma ( ifn - gamma ) . although the pathogenesis is still unknown , it has been suggested that gf may be an ifn - gamma mediated disease . in addition , an increased production of il-5 , probably induced by the clonal expansion of a locally recruited t - cell population may enhance the attraction of eosinophils into the lesions of gf . therefore , a possible mechanism of action of topical tacrolimus in this condition may be the inhibition of ifn - gamma and il-5 production and release , induced by the down - regulation of the t - cell activity , primarily involving the calcineurin binding and inactivation . however , we did observe eosinophils in skin biopsy in our case , probably since the biopsy was taken after one year of oral dapsone . our patient experienced a relevant improvement within 3 months of treatment with tacrolimus ointment after no response with intra lesional steroids , dapsone and cryo therapy . in conclusion , the previous reports and our observation suggest that topical tacrolimus may be a well - tolerated , efficacious therapy for gf .
granuloma faciale ( gf ) is a chronic condition characterized by red - brown plaques with follicular accentuation present usually on the face . we present a case of 35-year - old female with 5 year history of plaques over cheek and extra facial sites consistent with gf and its response to topical tacrolimus . this case supports previous reports of successful treatment of gf with topical tacrolimus .
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in a recent issue of critical care , we presented a series of 13 patients undergoing post - cardiotomy extracorporeal membrane oxygenation ( ecmo ) with bivalirudin - based anticoagulation . after this series , we satisfactorily continued this experience , reaching about 20 patients treated . however , we think that a word of caution should be added to our recently published experience . owing to its pharmacological profile , bivalirudin is rapidly cleaved by proteolytic enzymes , and its half - life is about 25 minutes when the renal function is normal . the use of bivalirudin for cardiopulmonary bypass ( cpb ) and ecmo is a feasible option . however , it is recommended that , during cpb , blood stagnation in the circuit be avoided because the rapid cleavage of bivalirudin may result in thrombosis . the ecmo circuit is devoid of a reservoir , and therefore blood stagnation is usually not a circuit- related problem . conversely , under some circumstances , the cardiac chambers may act as a ' natural reservoir ' , which entails blood stagnation and the risk for spontaneous intracardiac thrombosis . this is particularly true in the case of a very large right or left atrium with insufficient venous drainage or the case of very poor ventricular systolic function with intraventricular blood stagnation . to avoid this condition , we think that maintaining a partial ecmo support , leaving a minimal degree of intracardiac blood flow , may be a reasonable choice . this may be checked by echocardiographic view of heart valve movement or simply by the observation of some degree of pulsatile arterial blood pressure . we think that , in the event of echocardiographic evidence of a ' smoke effect ' within one or more cardiac chambers , bivalirudin should be replaced by standard heparin anticoagulation . intracardiac thrombus formation during ecmo with heparin anticoagulation has been described as well , but the pharmacokinetic properties of heparin may limit the risk of thrombus formation due to blood stagnation .
bivalirudin has been proposed as the sole anticoagulant in patients under extracorporeal membrane oxygenation ( ecmo ) or cardiopulmonary bypass . owing to the pharmacodynamic properties of bivalirudin , areas of blood stagnation should be carefully avoided in order to limit the risk of thrombosis . the ecmo circuit has no reservoir and is usually devoid of blood stagnation areas . conversely , under some circumstances , intracardiac blood stagnation areas may exist . in this case , there is a potential risk for the spontaneous formation of an intracardiac thrombus . we suggest that , under bivalirudin anticoagulation , a minimal degree of intracardiac blood flow with left heart valve movement is allowed .
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tuberculosis ( tb ) is a major health issue in developing countries with over two billion people being infected with tuberculosis bacilli world wide ( 1 ) . although tb was recently regarded primarily as a disease of the developing countries it is now also on the increase in developed countries such as the united kingdom , mainly as a result of globalisation as well as an increase in immunosuppressed patients . figures by the health protection agency ( hpa ) show that the cases of tb in the uk increased by 5.5% in 2009 when compared to those in 2008 ( 2 ) . this caucasian patient presented to the emergency department with sudden pain localised to the ulnar aspect of her wrist . x - ray of the forearm was normal and she was treated conservatively but was lost to follow up . she presented again at the orthopaedic clinic 26 months later with continuing swelling and pain . ultrasound showed a synovial tumour on the dorso - medial aspect of the wrist ( figure 1 ) . ultrasound showing a synovial tumour on the dorsal medial aspect of the wrist however , special stains for fungi and acid fast bacilli were negative . subsequently the patient developed a sinus and continued to have a chronic discharge from her left wrist . x ray this time showed destruction of the ulnar styloid consistent with a bony infection ( figure 2 ) . mri showed destruction localised around the ulnar head with oedematous changes extending up the shaft of the ulna ( figure 3 ) . ap xray of the wrist showing destruction of the ulnar styloid consistent with a bony infection . soft tissue swelling can also be noted over the dorsum of the wrist mri showing localised destruction around the ulnar head . the mri also shows thickening around the extensor carpi ulnaris tendon in keeping with marked tenosynovitis at this site . the patient agreed to a repeat biopsy and excision of the necrotic bone ( figure 4 ) . microscopy of the specimen again showed a florid granulomatous inflammatory process with areas of fibrinoid necrosis . post - operative xray of the wrist showing excision of the ulnar styloid further enquiry revealed that our patient had been experiencing lethargy but no other features to suggest tuberculosis . following the diagnosis of tb , the patient recalled that in her former post as a nursing assistant , when she was much younger , she was exposed to patients with tb . although improvements in its management and treatment are continuously occurring an estimated 1.8 million people died of tb in 2008 ( 1 ) . extra - spinal tuberculous osteomyelitis is rare and comprises about 2 - 3% of all cases of osteoarticular tuberculosis , with the hip and knee joints being the most common following spinal involvement ( 3 ) . tuberculous involvement of the ulna is uncommon and tends to be more common in the diaphysis . a high index of suspicion of tb is important to obtain the correct diagnosis . in chronic soft tissue and bony infection the diagnosis of tb may not be evident from the history and examination and indeed no obvious risk factors may be present as in our case . in our patient the infection could have been contracted at a much younger age , when she was caring for patients infected with tuberculosis , and remained latent . however no other risk factors were evident that could explain why the disease was activated after such a long period of latency . it is also interesting to note that the actual distal radio - ulnar joint was not involved , most of the destruction being situated around the ulnar process . although localisation of the tuberculous infection in the bone without joint involvement can occur , this is rare ( 3 ) .
tuberculosis ( tb ) is a major health issue in both developing and developed countries such as the uk . healthy individuals who contract the infection have only 5 - 10% chance of converting to the active disease over their lifetime . we present the clinical history of a 76 year old female who attended the emergency department complaining of wrist pain , and was only diagnosed with tb after three years . this case report emphasises the importance of including tb in the differential diagnosis when treating patients with an infection resistant to conventional antibiotics , even though risk factors for tb may not be evident or special stains for tb are negative .
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gossypiboma ( retained surgical sponge ) describes a mass of cotton or sponge that is left behind in the body cavity during a surgical operation . these foreign bodies can often mimic tumors or abscesses when detected clinically or with radiation . in fact , surgical sponges without opaque markers are the leading cause for the difficulty of a correct diagnosis . here , we present a case in which a foreign body , gauze without radiopaque markers , was left behind during a procedure that involved the removal of an intrapelvic tumor . after a computed tomography ( ct ) scan had revealed information leading to an inaccurate diagnosis , an accurate diagnosis of gossypiboma was successfully made using endoscopic ultrasound - fine - needle aspiration ( eus - fna ) . a 60-year - old female patient was referred with an unexpected detection of an intrapelvic tumor during a routine ct scan . the patient 's medical history included a hysterectomy and a left side ovariectomy at the age of 27 . the ct scan revealed a 45 mm tumor in the patient 's pelvis [ figure 1a and b ] , which was diagnosed as a gastrointestinal stromal tumor ( gist ) , a chronic expanding hematoma or leiomyoma . a forward - view eus ( tgf - uc260j , olympus optical co. , tokyo , japan ) identified a low echoic lesion when scanned from the sigmoid colon . fna was performed with a 22-gauge needle ( ezshot2 , olympus optical co. , tokyo , japan ) by using the forward - view eus [ figure 2 ] . examination of the fna specimen revealed a foreign body ( string of gauze ) displaying macrophage cells and necrotic tissue [ figure 3 ; hematoxylin and eosin , original magnification 40 ] . during the surgery , histological examination of the tissue adjacent to the foreign body revealed granuloma formations with fragmented silken threads [ figure 4 ; hematoxylin and eosin , original magnification , 100 and 200 ] . a : sagittal section ( black arrow ) ; b : horizontal section ( black arrow ) . endoscopic ultrasound ( eus)-fine - needle aspiration using forward - view eus a foreign body ( string of gauze ) obtained using the 22-gauge needle , with macrophage cells and necrotic tissue granuloma formations with fragmented silken threads gossypiboma describes a mass of cotton or a sponge that is retained in the body after a surgery . gossypiboma causes foreign body reactions of the surrounding tissue since they are inert and show no specific decomposition . in this case , radiopaque sponges were not used , and there were no adverse symptoms in the 30 years postsurgery . the typical appearance of gossypiboma shows a spongiform pattern with gas bubbles . in typical cases , however , it can be difficult to diagnose the disorder with ct scans in cases without gas bubbles . in the case described in this paper , radiologists using findings from a ct scan , initially and incorrectly diagnosed the lesion as gist with fibrosis . however , the disorder was correctly diagnosed by a team of gastroenterologists using eus - fna . the eus - fna procedure revealed a segment of string in a fixed foreign body in the pelvis . the application of eus using curved linear array ( cla ) endoscopes in the lower gi tract has been limited to the rectum and distal sigmoid colon because of the oblique - viewing optics . there were case reports using oblique - viewing cla echoendoscopes to evaluate lesions proximal to the sigmoid colon , but advancement of the cla echoendoscope beyond the sigmoid required the use of an overtube or a guide wire previously placed with a colonoscope . binmoeller et al . have reported the feasibility and safety of the front - view forward - array echoendoscope for evaluating right colon lesions . thus , we used a front - view forward - array echoendoscope to safely evaluate the intrapelvic tumor and sample the tissue . a correct diagnosis was obtained using this method . in summary , this is the first known case report diagnosing gossypiboma by eus - fna .
gossypiboma is a term used to describe a foreign mass within the body that is composed of a cotton matrix . gossypiboma can cause infection or abscess formation from an early stage , or it can remain clinically silent for many years . gossypiboma can be diagnosed using several methods including abdominal x - ray films , us of the abdomen , contrast - enhanced computed tomography of the abdomen , and during surgery . however , the variable appearance of gossypiboma can lead to diagnostic misinterpretations . here , we report a successful diagnosis of gossypiboma using a novel method , that is , endoscopic ultrasound - fine - needle aspiration .
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to demonstrate the usefulness of enhanced depth imaging optical coherence tomography ( edi - oct ) in investigating choroidal lesions inaccessible to ultrasound sonography . in a 60-year - old woman with an asymptomatic choroidal nevus , normal oct was used to observe the macula and edi - oct to image the choroidal nevus that was inaccessible to ultrasound . the exact location of the lesion in the choroid and the dimensions of the nevus were measured . the lesion was located in the superior macula , and the nevus was homogeneous in its reflectivity . we observed a thickened choroid delineated by the shadow cone behind it , measuring 1,376 325 m in the larger vertical cut and 1,220 325 m in the larger horizontal cut in an image with a 1:1 pixel mapping and automatic zoom . edi - oct appears to be an excellent technique for measuring choroidal nevi and all choroidal lesions accessible to oct imaging by depicting their exact location in the choroid , their dimensions , and their demarcation from the surrounding healthy tissue , thus allowing for a more efficient and accurate follow - up . choroidal nevus is the most common tumor of the ocular fundus , present in 7% of the white population [ 1 , 2 ] . despite its benign nature , it presents a risk of visual loss and visual field loss , and can rarely transform into malignant melanoma . optical coherence tomography ( oct ) and ultrasonography have been used for imaging choroidal nevi . recently , enhanced depth imaging oct ( edi - oct ) has been used in order to measure choroidal thickness in normal and pathologic eyes [ 3 , 4 ] . here , we report a case of a flat choroidal nevus inaccessible to ultrasound sonography that was evaluated by edi - oct . a 60-year - old woman was referred to our department with an asymptomatic choroidal nevus in her left eye . her best - corrected visual acuity was 20/20 in both eyes . slit lamp and fundus examinations of the right eye were normal . in the left eye , the anterior segment was normal ; however , on fundoscopy a choroidal nevus located in the superior macula was observed ( fig . spectral - domain ( sd ) oct ( heidelberg engineering , heidelberg , germany ) revealed normal foveal thickness ( fig . 2 ) . the flat nevus was inaccessible to ultrasound sonography , and edi - oct was used to image its posterior surface ( fig . edi - oct is a new technique that consists of positioning a heidelberg sd - oct close enough to the eye in order to obtain an inverted image , averaged for 100 scans . the advantage of this technique is that the sensitivity of the imaging in deeper tissue layers is increased , and thus the obtained measurements are more accurate . we employed this technique to image the location of the nevus in the choroid and obtain its dimensions as a reference measure for the follow - up examination ( fig . 3 ) . heidelberg sd - oct provides an exact correlation between the oct image and the infrared photograph . as infrared light is absorbed by melanin , the nevus appears brighter . in the edi - oct image , the lesion appears homogeneous and hyperreflective at the level of the large choroidal vessels , masking the underlying choroidal vasculature . the choroidal thickness at the level of the nevus appears greater than the neighbor choroid ( fig . the diameter of the nevus was calculated based on the presence of a shadow cone created by the lesion 's lateral borders . the choroidal thickness was measured based on the hyperreflectivity between the bruch 's membrane and the beginning of the shadow cone ( fig . the choroid measured 1,220 325 m in the larger horizontal cut and 1,376 325 m in the larger vertical cut in an image with a 1:1 pixel mapping and automatic zoom ( fig . edi - oct appears to be an excellent technique for evaluating flat choroidal nevi that are inaccessible to ultrasound sonography and accessible to oct imaging , displaying simultaneously the lesion and the retinal changes . it is a reproducible examination that allows depiction of the exact location of the nevi in the choroid , their dimensions and their demarcation from the surrounding healthy tissue . to date , the software available for sd - oct does not allow for objective measures . the subjective measures obtained are useful upon comparison with the follow - up images . in order to limit the possibility of bias , these follow - up images must be obtained and compared with the same sd - oct machine . we believe that this technique is clinically useful and , together with color fundus photography , can provide a more efficient and accurate follow - up of choroidal nevi .
purposeto demonstrate the usefulness of enhanced depth imaging optical coherence tomography ( edi - oct ) in investigating choroidal lesions inaccessible to ultrasound sonography.methodsin a 60-year - old woman with an asymptomatic choroidal nevus , normal oct was used to observe the macula and edi - oct to image the choroidal nevus that was inaccessible to ultrasound . the exact location of the lesion in the choroid and the dimensions of the nevus were measured.resultsthe lesion was located in the superior macula , and the nevus was homogeneous in its reflectivity . we observed a thickened choroid delineated by the shadow cone behind it , measuring 1,376 325 m in the larger vertical cut and 1,220 325 m in the larger horizontal cut in an image with a 1:1 pixel mapping and automatic zoom . the macular profile and thickness were both normal.conclusionsedi-oct appears to be an excellent technique for measuring choroidal nevi and all choroidal lesions accessible to oct imaging by depicting their exact location in the choroid , their dimensions , and their demarcation from the surrounding healthy tissue , thus allowing for a more efficient and accurate follow - up .
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for the first time , the presented data suggest that a variant in the retn gene plays a significant role in acne pathogenesis . we also demonstrate an excess transmission of mutant allele g at retn-420 from 130 trio families using tdt analysis . therefore , we suggest that inherited variation in retn at -420 appears to be associated with heritable acne vulgaris .
resistin ( retn ) , recently found to be relevant to inflammation and inflammatory disorders . we , therefore , aimed to investigate the potential role of retn gene polymorphism in pathogenesis of acne vulgaris with familial history . we investigated the retn-420c / g polymorphism in 180 patients with acne vulgaris and 180 healthy individuals in a case - control association analysis . in this study , we also investigated the heritability of the retn susceptible allele from 140 trio families with acne affected offspring . the genotyping was performed by polymerase chain reaction and direct dna sequencing . the retn-420c / g polymorphism was significantly associated with acne in patients compared with healthy controls ( p=0.014 ) . the minor allele g at -420 was more prevalent in cases vs. controls ( p=0.002 ) . the retn-420c / g polymorphism was significantly associated with severity of acne vulgaris in patients ( p=0.0097 ) . the results of a transmission disequilibrium test revealed a significant association between the retn-420c / g polymorphism and acne vulgaris ( p<0.001 ) . for the first time in the literature , to our knowledge , we demonstrate a significant association of the retn-420c / g functional polymorphism with familial acne vulgaris .
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six a17 bac and one fosmid library were used to create mt3.5 ( table s1 ) . most were processed by sanger paired - end sequencing of 3 - 6 kb shotgun libraries . sequences were downloaded in february / march 2009 with scaffolding performed by aligning all bac and fosmid ends against contigs and then anchored and ordered primarily by optical mapping . separately , 25 gb of illumina sequence was generated using short ( 375 nt ) inserts plus 2.1 gb from a 5 kb mate - pair library , then assembled using clcbio ( www.clcbio.com ) and soap ( http://soap.genomics.org.cn/ ) . five tissues were used for rna - seq analysis with ~10 million illumina 36 bp reads per library ( table s12 ) . three tissues were used for small rna analysis with ~3 million reads per illumina library ( figures s17-s18 , table s16 , datafile s9 ) . six a17 bac and one fosmid library were used to create mt3.5 ( table s1 ) . most were processed by sanger paired - end sequencing of 3 - 6 kb shotgun libraries . sequences were downloaded in february / march 2009 with scaffolding performed by aligning all bac and fosmid ends against contigs and then anchored and ordered primarily by optical mapping . separately , 25 gb of illumina sequence was generated using short ( 375 nt ) inserts plus 2.1 gb from a 5 kb mate - pair library , then assembled using clcbio ( www.clcbio.com ) and soap ( http://soap.genomics.org.cn/ ) . five tissues were used for rna - seq analysis with ~10 million illumina 36 bp reads per library ( table s12 ) . three tissues were used for small rna analysis with ~3 million reads per illumina library ( figures s17-s18 , table s16 , datafile s9 ) .
legumes ( fabaceae or leguminosae ) are unique among cultivated plants for their ability to carry out endosymbiotic nitrogen fixation with rhizobial bacteria , a process that takes place in a specialized structure known as the nodule . legumes belong to one of the two main groups of eurosids , the fabidae , which includes most species capable of endosymbiotic nitrogen fixation 1 . legumes comprise several evolutionary lineages derived from a common ancestor 60 million years ago ( mya ) . papilionoids are the largest clade , dating nearly to the origin of legumes and containing most cultivated species 2 . medicago truncatula ( mt ) is a long - established model for the study of legume biology . here we describe the draft sequence of the mt euchromatin based on a recently completed bac - assembly supplemented with illumina - shotgun sequence , together capturing ~94% of all mt genes . a whole - genome duplication ( wgd ) approximately 58 mya played a major role in shaping the mt genome and thereby contributed to the evolution of endosymbiotic nitrogen fixation . subsequent to the wgd , the mt genome experienced higher levels of rearrangement than two other sequenced legumes , glycine max ( gm ) and lotus japonicus ( lj ) . mt is a close relative of alfalfa ( m. sativa ) , a widely cultivated crop with limited genomics tools and complex autotetraploid genetics . as such , the mt genome sequence provides significant opportunities to expand alfalfa s genomic toolbox .
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neurofibromatosis ( nf ) is an autosomal dominant disease , discovered in 1882 , by the german pathologist friedrich daniel von recklinghausen , characterized by disordered growth of ectodermal tissues , and is part of a group of disorders called phakomatoses ( neurocutaneous syndrome ) . neurofibromatosis type - i ( nf-1 ) , also known as von recklinghausen syndrome , is caused by the mutation of a gene on the long arm of chromosome 17 , which encodes a protein known as neurofibromin . it is characterized by spots of increased skin pigmentation ( caf au lait spots ) , combined with peripheral nerve tumors and a variety of other dysplastic abnormalities of the skin , nervous system , bones , endocrine organs , and blood vessels . the localized form of neurofibromatosis type - i , first described by gammel in 1931 , is very rare . crowe et al . proposed the term sectorial neurofibromatosis for this localized form of neurofibromatosis , and miller and sparkes modified the nomenclature to segmental neurofibromatosis ( sn ) the current term for neurofibromas of segmental distribution . the commonly affected sites for sn are the thorax and abdomen ( 55% ) , upper extremities ( 20% ) , and lower limb and face ( 10% each ) . only few cases ( less than 10 ) of segmental neurofibromatosis over the face have been described so far . a 26-year - old female patient reported to the dental department complaining about the unesthetic appearance of her face , since eight years [ figure 1 ] . history revealed that eight years back , the patient underwent surgery in the neck region for a cyst and one to two days after surgery the patient got a swelling on the face . the swelling was of the same size since then and there was no pain associated with the swelling . the unesthetic appearance of the face on examination , the face of the patient appeared asymmetrical , bilaterally , with a growth on the right side of the face . the skin over the right side of her face was thickened and hyperpigmented , with a soft and loose hanging overgrowth . there were numerous small and large , sessile and pedunculated nodular growths localized on the right side of her neck , with dark - colored underlying skin . the skin over the right cheek and chin area showed coffee - colored pigmentation ( caf - au - lait spots ) [ figure 2 ] . nodular growths localized on the right side of the neck with dark colored underlying skin there was a soft , compressible , nontender growth on the lower lip . the right ear showed disfigurement and hypertrophy , with thickened cartilage and overlying hyperpigmented skin [ figure 3 ] . disfigurement of the ear and hypertrophy with thickened cartilage and overlying hyperpigmented skin intraorally there were numerous soft tissue growths with a sessile base seen on the right buccal mucosa [ figure 4 ] and tongue [ figure 5 ] . soft tissue growths with sessile bases on right buccal mucosa soft tissue growths with sessile bases on the tongue laboratory findings revealed a raised erythrocyte sedimentation rate ( esr ) ( 70 mm in the first hour ) , a decreased hemoglobin level ( 7.5 gm% ) , and the enzyme - linked immunosorbent assay ( elisa ) was found to be nonreactive . a panoramic radiograph disclosed asymmetric enlargement of the right side of the mandible , with loss of cortication of the lower border in the midline area , expansion of the body of the mandible causing displacement of the teeth , seen on right side , expansion of the right inferior alveolar nerve canal , enlarged mental foramen , resorption of the roots of the mandibular right teeth , and loss of the right antegonial notch . a deficiency was seen on the right side of the maxilla , with crowding of the posterior teeth [ figure 6 ] . a panoramic radiograph the right lateral oblique radiograph revealed a deep sigmoid notch , loss of the antegonial notch , an enlarged and rounded coronoid process , with resorption of the mandibular right teeth [ figure 7 ] . a right lateral oblique radiograph subsequently , biopsy of one of the nodules was taken and sent for histopathological examination . the histopathological slide revealed a non - encapsulated tumor of the dermis with a normal overlying epidermis . the tumor consisted of loosely spaced spindle cells and wavy collagenous strands in a clear matrix [ figure 8 ] . a non - encapsulated tumor of the dermis , with a normal overlying epidermis these tumors are composed of schwann cells , fibroblasts , mast cells , and vascular components . the caf au lait spots are irregularly - shaped , evenly pigmented , brown macules . most individuals with neurofibromatosis have six or more spots that are 1.5 cm or greater in diameter . in young children , five or more caf au lait macules , greater than 0.5 cm in diameter , are suggestive of neurofibromatosis . less than 1% of the healthy children have three or more such spots , although one or two caf au lait macules are commonly encountered in healthy individuals without disease . bone involvement includes pseudoarthrosis of the tibia , bowing of the long bones , and orbital defects . treatment of neurofibromatosis is predominantly surgical . when neurofibromas increase in size or cause pain , malignant transformation must be suspected , and excision or biopsy must be performed . acoustic neuromas and tumors that cause tinnitus and vertigo must be excised with great caution . plastic surgeons may be included in the correction of deformities , especially those of the face . considering the autosomal dominant inheritance pattern of neurofibromatosis , genetic counseling
segmental neurofibromatosis is a rare disorder , characterized by neurofibromas or caf-au - lait macules limited to one region of the body . its occurrence on the face is extremely rare and only few cases of segmental neurofibromatosis over the face have been described so far . we present a case of segmental neurofibromatosis involving the buccal mucosa , tongue , cheek , ear , and neck on the right side of the face .
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patient aged 64 was admitted to the county hospital urology ward in september 2010 and diagnosed with tumor of the left kidney . ultrasonographic exam revealed the structure with a diameter of 45 mm in the central field of the left kidney . ct showed a solid focal lesion of the left kidney ( 30 x 40 x 32 mm ) located below the renal stalk and covering the renal pelvis and the paranephric section of the left ureter , apart from that no changes were observed ( figure 1 ) . radiological image of the chest did not reveal any pathological changes . in order to make the results of pre operational diagnosis sided ureterorenoscopy was conducted and a urine sample was taken for cytology to exclude epithelial nature of the tumor . after undergoing the above mentioned test , the patient was qualified for transperitoneal radical nephrectomy . with general anesthesia applied , the left kidney was removed through an incision below the left costal margin . during the operation , a significant peripheral infiltration was discovered which hindered access to the renal stalk of the left kidney . operative course passed without complications and the patient was discharged in good general condition 8 days after the surgery . left kidney tumor in ct exam . on pathological examination ( samples no 12399241239928 ) in all samples after detailed hematological work up which did not show any other tumors , the patient was qualified for radiation treatment and currently remains under permanent oncological supervision . medical records and literature mention several cases of malignant kidney lymphoma [ 1 , 2 ] . some authors claim that this entity accounts for 3% of all solid renal tumors among adults . lymphomatous renal involvement can have three different causes : most commonly it appears along with the generalized disease and enlarged lymph nodes . renal involvement develops without any symptoms then.it may be related to organ transplantation and infection with epstein such cases are rare and difficult to diagnose which may lead to mistakes in treatment . such cases are rare and difficult to diagnose which may lead to mistakes in treatment . the case presented in the report illustrates diagnostic and therapeutic difficulties that a urologist might encounter during treatment of patients with possible renal tumor . results provided by clinical imaging of focal lesions are inconsistent . what must always be taken into account is an atypical tumorous process , autoimmunological disorders as well as inflammatory condition . in this particular case control appointments in the department of hematology have been scheduled to take place every four months and the prognosis for the patient has been described as good . they develop from lymphocytes and , depending on the variety of b and t lymphocytes , those processes can have different clinical outcome . some of them are located solely within the lymph nodes or tissues and internal organs , while others are accompanied by leukemia . it might seem that when it comes to lymphomas , the pathogens initiating the course of the disease could be both , the epstein barr viruses which attack the b lymphocytes , and by htlv1 viruses dwelling in t lymphocytes and causing a subacute form of leukemia among adults . there can also be some co factors of environmental character : chloro organic compounds , ionization energy , benzene like substances , states of immunosuppression and autoimmunological diseases . the malignant lymphoma morbidity rate can also be increased by extensive exposition to herbicides , and is even 35 times higher among patients with a kidney transplant . it usually dwells within the lymphoid tissue of waldeyer 's ring , spleen and thymus . extranodal lymphomas can most often be found in the gastrointestinal tract , other types can be located in skin , bones , eye kidney might be at the stage of involvement which spreads through lymphatic system from the nodes in retro peritoneum . if the renal involvement occurs at an advanced stage of the disease , as yet another location of it , it is relatively easy to diagnose . once a patient is diagnosed with lymphoma of kidney , further examinations are required in order to rule out a systemic disease . the necessary test in such cases include : blood tests , bone marrow biopsy , radiological examination , pathomorphological evaluation and histo immunological evaluation of enlarged lymph nodes . the malignant types , however , build up rapidly , are resistant to treatment and show no signs of improvement within a short period of time . how long the patient will live depends on the histopathological diagnosis of the tumor and its stage of development . non hodgkin lymphomas are treated with chemotherapy , radiation , and , in some cases , surgeries . low malignant , originally extranodal , tumors are usually surgically removed and treated with radiotherapy . cell lymphoma associated with chronic inflammation arising in a renal pseudocyst with barely noticeable clinical symptoms . publications in english present several cases of spontaneous rupture of the ureter as primary symptom of malignant lymphoma . risk of their occurrence increases from 1.2% in 5 years after the operation , to 6.8% in 20 years after the transplantation , which is related to epstein barr virus infections . some authors also report on cases of co existence of inflammatory intestines disease , including the crohn disease , and malignant lymphomas but then changes can most frequently be located in the vicinity of intestines or other adjacent sections of the digestive tract . the presented case leads to a conclusion that each focal lesion of unclear etiology found on imagining studies should be treated as a possible malignant lymphoma and ought to be histopathologically verified as quickly as possible .
we present a 64year old patient suffering from extranodal malignant lymphoma . the patient was admitted to the county hospital urology ward with suspicion of the left kidney tumor . as part of pre operational diagnosis , ultrasonographic examination and computed tomography of kidneys were conducted . the results confirmed the initial diagnosis . after undergoing radical surgical treatment , the patient is currently under the care of hematology ward of oncology centre .
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craniofacial defects are rare , disfiguring facial anomalies , with an incidence of about 1.4 to 4.9 cases per 100,000 live births . craniofacial clefts may be caused by failure of the maxillary processes to fuse , external pressure , amniotic bands , oligohydramnios , central disorganization of the neural crest , and hematomas . in 1973 , the median cleft of the upper lip , or tessier 0 class , has a variety of presentations , ranging from a minimal notching of the lip , vermilion , and nose to a wide cleft that divides craniofacial structures . tessier 0 clefts result from failure of the two medial nasal processes to fuse at midline . median clefts are broadly classified into true and false ; false clefts are due to agenesis of the medial nasal process , while true clefts are due to failure of the medial nasal process to fuse . treatment of median clefts depends on the clinical presentation and may vary from simple alignment of orbicularis oris and vermilion , to reconstruction of the cupid 's bow and philtrum for true median clefts , and complex craniofacial procedures in case of false median clefts . various techniques have been described to repair mild or moderate true median cleft lips . here we report using a pfeifer incision to correct a moderate true median cleft of the upper lip . pfeifer incision includes wavy lines , which elongate the incised tissues as the waves are straightened to close in a straight line , and also provide extra tissue for a tension free closure . a five - year - old boy with a facial cleft presented to our clinic . on examination , there was a median cleft of the upper lip involving the white roll , with no bony involvements . the highest- ( points a , a ) , and the deepest - points ( points b , b ) of the white roll were marked on both sides . subsequently , a wavy incision line was made starting from the deepest point and extending over the philtrum just above the cleft ( fig . a diamond incision was made over the vermilion and the labial mucosa , intra - orally extending just beyond the cleft margin . incision was made and the mucosal tissue covering the area medial to the incision site ( sterile zone ) was removed . undermining of the orbicularis oris muscle was performed and it was approximated using 4 - 0 vicryl . the cupid 's bow was properly aligned , with equal height on both sides ( fig . vermilion form was satisfactory and the fullness and continuity of the orbicularis oris were maintained . incision marking for pfeifer technique ( a , a : highest points on white roll ; b , b : deepest points on white roll ) postoperative result after suture removal a median cleft can also be called midline cleft or vertical cleft through the centre of the upper lip , and is a rare anomaly , the exact developmental origin of which is not clear . tessier 0 clefts occur during the third week of gestation due to failure of the two medial nasal processes to close in midline . it can occur in isolation or be a part of a syndrome such as orofacial - digital syndrome . false clefts are associated with abnormalities of the forebrain and are categorized under the category of holoprosencephaly . in 1937 , veau categorized median clefts to notch of the lip , median cleft extending to the columella , and defects due to atrophy of the midline facial structures . median cleft face syndrome , frontonasal dysplasia , and tessier 0 clefts are various terms used to describe abnormalities associated with true median clefts that are not accompanied by forebrain abnormalities . the tessier 0 anomaly may present as a small notch in the soft tissue , or in association with hypertelorism , midline craniofacial osseous defects , and hairline abnormalities . various treatment options are present for mild deformities which do not involve the white roll . when developing the treatment plan , reconstruction of the cupid 's bow , labial philtrum , vermillion , and buccal mucosa should be kept in mind . urata and kawamoto described using a v - y flap , while weimer et al , used a diamond incision to repair these anomalies . da silva frietas and colleagues described a mucosal z transposition technique to treat mild cases . for moderate defects involving the philtrum , these waves are subsequently approximated in a straight line , which help expanding the length and width of the tissue . this incision has previously been used to correct other tessier clefts with a high success rate . the above - mentioned studies prove the versatility of the pfeifer incision . in our case we were able to achieve adequate symmetry of the philtrum and the cupid 's bow , which is important in cases like this . the postoperative results were excellent and proper approximation of the orbicularis oris muscle and the vermilion was achieved with a symmetrical cupid 's bow and philtrum . the only disadvantage of this technique is that the final closure line is placed directly over the philtrum . since the presentation of midline cleft deformities varies widely , each case should be individually considered and treated . pfeifer incisions can successfully provide mucosal length , vermilion fullness , and lip symmetry in patients with moderate median cleft lip .
median cleft is the midline cleft of the lip . it develops due to incomplete or failed fusion of the median nasal prominence . it can present with minimal deformities such as involvement of the vermilion border , or complex clefting of the midline structures and brain . median clefts are broadly classified as true and false clefts . this case report describes a rare case of median cleft of the upper lip involving the white roll , which was not associated with any other deformities . treatment included reconstruction of the philtrum and the cupid 's bow while maintaining vermilion fullness and continuity , and minimizing scar formation . various techniques have been advocated for treatment of this type of median upper lip cleft . here we describe a technique using pfeifer incision to correct our patient 's defect . pfeifer incision consists of wavy lines and its use has been advocated for correction of various craniofacial abnormalities .
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the prevalence of syphilis in the normal population has decreased significantly since 1977 in korea ( 1 , 2 ) . however , in western countries the incidence of syphilis has risen , mainly in the 20 - 24 age group and its clinical manifestations have been various ( 3 ) . sexual activity has become more acceptable in our culture recently , initiating various forms of sex , and as a result , many different clinical conditions of sexually transmitted disease ( std ) have appeared . it is usually asymptomatic and less frequently presents as proctitis , ulcer and pseudotumors ( 3 , 4 ) . therefore , it is difficult to diagnose and physicians occasionally might prescribe inappropriate treatments ( 5 , 6 ) . in this paper we describe a case of primary rectal syphilis which was suspected to be rectal cancer . he complained of bowel habit change with tenesmus , mucous discharge , anal pain and intermittent presence of blood in stool . he had a 2 cm sized , firm , non - tender and indurated nodule on the left side of the inguinal area and multiple condyloma acuminatum around the anus . sigmoidoscopy in our gastrointestinal endoscopy unit showed two indurated masses of 1.5 to 2 cm on the posterior wall of the middle and lower rectum ( fig . the masses had a coin like appearance , and each of them had a slightly depressed and ulcerated surface . the vdrl quantitative test was 1:64 and the fta - abs igm and igg test was positive . a human immunodeficiency virus ( hiv ) the patient was treated with intramuscular penicillin g benzathine ( 2,400,000 iu im/1 week , thrice ) . follow - up sigmoidoscopy after 1 month showed nearly complete regression of the chancre ( fig . a repeat biopsy was done and the histologic result showed focal lymphoid hyperplasia . a vdrl quantitative test after 3 months was 1:1 . most physicians have a tendency to consider rectal ulceration as a neoplasm ( 7 , 8) . similarly in our case symptoms and signs suggested rectal neoplasia and the clinical history did not reveal homosexuality . however , after the vdrl test the patient confessed that he had been raped by a homosexual male . therefore , when rectal syphilis is suspected ( i.e. , perianal condyloma acuminatum associated with inguinal lymphadenopathy ) the history of sexual intercourse , especially rectal intercourse , has to be taken . a dark - field examination is recommended by many studies ( 3 ) . in our case , however , a dark - field examination was not performed , because rectal chancre was highly suggested by the clinical history , anorectal lesions , pathologic findings and positive serologic test . additionally , like other syphilis cases benzathine penicillin therapy induced a rapid regression of the rectal lesions . we believe when a positive serologic syphilis test is associated with ulcerative lesion , additional laboratory tests are not necessary . inguinal lymphadenopathy need not require an initial biopsy when evidence of regional infection is present . usually after antibiotic therapy of at least 4 weeks , most physicians might decide whether a biopsy is necessary . in this case , however , the physician did not have experience recognizing rectal syphilis , so he immediately obtained a biopsy specimen from the inguinal lesion . . in western countries the incidence of rectal syphilis is rising , especially in groups of active homosexual males . bassi et al . emphasized in his case report that endoscopists should keep in mind the rising incidence of syphilis ( 3 ) . therefore an initial physician should encourage his / her patient to inform him / her of the patient 's sexual history .
a 30-yr - old man was referred for suspicious rectal cancer because of ulcerated lesions in the rectum and a palpable mass in left inguinal area . sigmoidoscopy showed two indurated masses and histologic evaluation of biopsy revealed obliterative endarteritis with heavy plasma cell infiltration . both venereal disease research laboratories ( vdrl ) and fluorescent treponemal antibody absorption ( fta - abs ) tests were positive . after injection of penicillin g benzathine for 3 weeks , the rectal chancre and the palpable mass disappeared .
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kcm is itself very rare and distinct type of keratoacanthoma which usually occurs over extremities and scalp is unusual site for development of lesion . treatment is difficult as lesion of kcm present with large raised , rolled borders with peripheral extension . keratoacanthoma ( ka ) is a rapidly evolving tumor , composed of keratinizing squamous cells originating in pilosebaceous follicles and resolving spontaneously if left untreated . it is relatively common , especially in whites occurring in middle age while being uncommon in dark - skinned . . it presents as firm , rounded , flesh - colored or reddish papule ; with a rapid growth phase becoming 10 - 20 mm and then spontaneous healing taking place over three months . there are three rare clinical variants of solitary ka , namely giant ka , keratoacanthoma centrifugum marginatum ( kcm ) and subungual ka . in kcm , lesions are large , reaching upto 20 cms . the most common locations are dorsa of hands and legs , lesions on scalp being rare . we report a rare case of kcm occurring on the scalp which is an unusual site . a 62-year - old male , watchman by occupation presented with asymptomatic raised lesion on scalp since one year . lesion had developed de novo , as a pea sized lesion with gradual increase to cover entire vertex . cutaneous examination revealed a single , irregular , 12 15 cm , yellowish plaque on vertex of scalp with nodular surface and a central crater [ figure 1 ] . on palpation , the plaque was firm , non - tender , non - indurated and not attached to underlying structure . ultrasonography of lesion showed ill defined hypo echoic mass involving epidermis , dermis and subcutaneous tissue . a single , irregular , 12 15 cm , yellowish plaque on vertex of scalp with nodular surface and a central crater . deep punch biopsy revealed exoendophytic , globular , well circumscribed central cup shaped crater , epidermal invagination with marked hyperkeratosis and horn cyst with rich keratin filled crater imparting glassy appearance and well demarcated regular base . . marked epidermal proliferation with crater formation with horn pearls and inflammatory infiltrate at the base of the lesion was suggestive of a fully developed lesion of kcm . complete surgical excision with grafting was done successfully with dramatic improvement with no recurrence at 9 months [ figure 5 ] . exoendophytic , globular , well circumscribed central cup shaped crater , epidermal invagination with marked hyperkeratosis and horn cyst with rich keratin filled crater imparting glassy appearance and well demarcated regular base epidermis forming buttress over the pseudhorn cyst dense nodular lymphocytic infiltrate in the upper dermis surgical excision with grafting the etiology is multifactorial that includes chronic ultraviolet ray exposure , smoking , contact with chemical carcinogens like pitch , mineral oil , tar , trauma and vaccination . the role of human papilloma virus remains inconclusive but in one study , hpv type 6 and 11 were detected within the lesion . it can be localized to any region of the body but is more frequently seen on dorsum of hands and legs . progressive peripheral extension with a raised rolled - out margin and atrophy at the centre is a characteristic feature of kcm . the margin of the lesion showing multiple comedonal orifices giving rise to a cribriform pattern may represent a unique phenomenon of kcm . it is hypothesized that this typical appearance may arise as a result of sequential involvement of multiple adjacent hair follicles in a centrifugal fashion . kcm does not show tendency for spontaneous regression , a feature also seen in giant ka , which grows rapidly reaching a size of 5 cm or more and occurring commonly on nose and eyelids . kcm is differentiated from giant ka by absence of downward vertical spread and destruction of underlying tissue . nevertheless , in giant ka spontaneous involution takes place after several months , often accompanied by detachment of a large keratotic plaque . the other differential diagnoses include squamous cell carcinoma , lupus vulgaris , botryomycosis , blastomycosis - like pyoderma and pseudoepitheliomatous hyperplasia , hypertrophic lupus erythematosus , atypical mycobacterial infections , or deep fungal infections . there are reports that kcm has been treated successfully with oral retinoids ( acitretin , etretinate , or isotretinoin 0.5 - 1 mg / kg / day ) which should be given until complete clearance of lesion . surgical intervention is a preferred mode of therapy keeping in mind that a wide excision is performed to prevent subsequent recurrence . other treatment modalities successfully used include topical 5-fluorouracil , intralesional injections of interferon alpha , methotrexate , or bleomycin , or mohs micrographic surgery . in kcm , lesions are usually too large for excisional biopsy for diagnosis , but certain characteristic features like clinical behavior , appearance of lesions , histological features ( hyperkeratosis and hyperplasia ) , and cytological features ( tumor cells with eosinophilic and glassy cytoplasm , few mitoses ) help clinch the correct diagnosis . first time from india we are reporting case of kcm presenting over scalp which is successfully treated with surgical excision without any recurrence .
keratoacanthoma ( ka ) is a rapidly evolving tumor , composed of keratinizing squamous cells originating in pilosebaceous follicles and resolving spontaneously if left untreated . it is relatively uncommon in dark - skinned and occurs in middle aged individuals . males are three times more affected than females . it presents as firm , rounded , flesh - colored or reddish papule ; with a rapid growth phase followed by spontaneous healing over three months . two types of ka exist i.e. , solitary and multiple . there are three rare clinical variants of solitary ka , namely giant ka , keratoacanthoma centrifugum marginatum ( kcm ) and subungual ka . in kcm , lesions are large , reaching upto 20cms . there is peripheral extension with raised , rolled border and atrophy in the center . there is no tendency toward spontaneous involution . the most common locations are dorsa of hands and legs , lesions on scalp being rare . a rare case of kcm occurring on scalp which is an unusual site is reported .
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they are found most commonly in the cranial and peripheral nerves , and occurrence in the omentum is very rare . however , there have been some cases reported to develop serious complications and , if there was malignancy , to cause metastasis or recurrence . we presented a case of schwannoma originating from the great omentum , including histological and immunohistological studies . a 55-year - old man was referred to our department for the treatment of a tumor detected close to the stomach by ultrasound . medical examination including ultrasound had been regularly performed to follow up his gallbladder stone for the past 6 years . although he had no complaints and symptoms , the tumor had been increasing in size over one year . abdominal and endoscopic ultrasound showed a 2.0 1.3 cm cystic mass lateral to the wall of the stomach . its component included an 11.8 5.7 mm elevated lesion ( fig . 1 , fig computed tomography scan of the abdomen and pelvis showed a 2.6 1.9 cm cystic mass , which was slightly enhanced , and a gallbladder stone ( fig . 3 ) . magnetic resonance imaging demonstrated a hyperintense mass on t2-weighted image . a distance from the stomach wall laboratory tests , including tumor makers , were normal . under a perioperative diagnosis of cystic tumor in the abdomen and cholelithiasis , we performed laparoscopic resection with vessel sealing system , which revealed a 2.0 cm mass arising from the great omentum , not adherent to other organs . grossly , the tumor was configured by a well - encapsulated round mass measuring 30 18 15 mm in diameter ( fig . histologically , the spindle - shaped cells were arranged in interlacing bundles and fascicles , together with varying numbers of tumor cells containing various amounts of light brown or grayish pigment ( fig . these alternate with looser antoni b tissue , which is comprised of cells showing clear , vacuolated cytoplasm due to lipid accumulation . immunohistochemically , most of the neoplastic cells reacted moderately to nse , ncam and s-100 protein ( fig . schwannomas are classified as one kind of the peripheral nerve sheath tumors , of which schwannnoma and neurofibroma are the most frequent . currently , the most precise determination of the tumor 's cell type is established by its immunohistochemical profile , ultrastructural features , or both . a tumor composed of cells with distinctly schwannian characteristics schwannomas are known to mainly arise from the peripheral and caranial nerves , the extremities and the retroperitoneum . they are seldom found in the abdomen , especially the extragastrointestinal tract , of which solitaly schwannoma of the great omentum is an extremely rare tumor . in 303 schwannomas reported by das gupta , one case originated from the retroperitoneum , others from the central nervous system and peripheral nerve . in another review of the literature , stout and of these , 37 were in the stomach , 3 in the small bowel , and none in the omentum and the abdominal cavity . previously published cases of schwannoma in the omentum were collected from a computerized medical literature search ( pubmed ) . only 6 cases of schwannoma from the great omentum have been observed ( table 1 ) . we can see that more cases of schwannoma from the lesser omentum have been recorded than from the great omentum . it is mentioned that the lesser omentum contains a small amount of nerves in almost equal distribution , the great omentum has a paucity of nervous tissue and anatomically consists of fat and lymphatic tissue . we found a case of malignant schwannoma arising from the omentum which demonstrated peritoneal metastasis , and another reported case of the small intestine emphasized poor prognosis since only 2 of 24 patients survived for more than 5 years . benign schwannomas are also reported to increase in size and to eventually cause complications by compressing other organs or by causing bleeding in or outside the gastrointestinal tract . pigmented schwannoma is another type of schwannoma , usually arising from the sympathetic nervous system . additionally abdominal schwannomas cause diagnostic problems because clinical symptoms are uncharacteristic or misleading even if the tumor is large . ct imaging typically showed a low attenuation mass , peripheral enhancement and cystic degeneration pattern . mri disclosed schwannoma of low signal intensity on t1-weighted image and high signal intensity on t2-weighted image . in spite of these characteristic , it is difficult to exclude other abdominal tumors , leiomyomas , lymphomas and unspecified sarcomas etc . while previous cases confirm that the tumor may attain a considerable size , bigger than 5 cm or producing symptoms , our case of a schwannoma which was small and asymptomatic is hardly diagnosed . however this tumor showing progression in size is thought to have potential to behave very aggressively despite benign histological features , which stresses the need of more information on this type of tumor and diagnosis . consequently , histological analysis of the surgical specimen is necessary for a correct diagnosis , and common treatment for schwannoma is surgery . therefore laparoscopic resection seemed to be the most adequate method to diagnose and rule out malignant tumor as a minimally invasive surgery . the majority of tumors can be safely resected , the surgeon being careful of the dividing feeder vessels because schwannomas are well known as hypervascular tumors . to our knowledge there are no case reports documenting other treatments , including chemotherapy for schwannomas in the abdominal cavity .
schwannoma in the abdomen is an uncommon neoplasm that occurs most frequently in the cranial and peripheral nerves ; it is extremely rare in the great omentum and only 6 cases of schwannoma of the great omentum have been observed previously . we report the case of a schwannoma found in the great omentum of a 55-year - old man who was treated with laparoscopic surgery . though it was difficult to diagnose preoperatively , the tumor showed malignant potential by rapidly increasing in size . histologically it was configured by a well - encapsulated round mass measuring 30 18 15 mm in diameter . immunohistochemically most of the neoplastic cells reacted moderately to nse , ncam and s-100 protein . we document the clinicopathological study of a schwannoma of the great omentum , followed by a review of the literature .
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the clavicle is a rare site for bone tumours . according to the world health organisation , the giant cell tumor is an aggressive potentially malignant lesion which means that its evolution based on histological features is unpredictable . sites commonly affected by giant cell tumour are proximal tibia , distal femur and distal radius . the oncologic patterns of clavicle resemble that of flat bones and not other long bones . among tumors of clavicle , a 60 year old man presented to our department with pain and swelling over lateral end of left clavicle ( fig . 1 , fig . the pain was insidious in onset , non radiating and had no diurnal variations and was aggravated on shoulder movements and relieved on taking medications . we got a plain radiograph which revealed which an expansile radiolucent lesion arising from lateral end of left clavicle ( fig . 3 ) . the swelling demonstrated geographic type destruction without any soft tissue component or periosteal reaction . 4 ) . to aid in the diagnosis fine needle aspiration cytology was done which revealed a predominantly cellular lesion having sheets of plump , oval mononuclear cells with mild pleomorphism . the cells had moderate cytoplasm , oval to elongated nucleus with moderate anisokaryocytosis with irregular nuclear membrane . amongst these cells , many multinucleated giant cells were also which were distributed evenly . the differential diagnosis which were kept in mind are aneurysmal bone cyst , non ossifying fibroma , eosinophilic granuloma and tuberculous osteomyelitis . since the clavicle does not necessary require reconstruction and the patient was a retired school teacher , not engaged in any physical work so surgical resection of the tumor was planned . after proper investigations and pre anaesthetic clearance , a wide excision of the mass along with 3 cm of the healthy tissue was done ( fig . the excised mass was sent for histopathological examination which also confirmed it to be a giant cell tumor . the range of motion of the left shoulder was normal and post operatively there was no neurovascular deficict . the patient was happy with the surgical outcome and at 1 year follow up there was no evidence of recurrence or metastasis . . primary bone tumors of the clavicle are more likely to be malignant than benign , and amongst these tumors which occur in clavicle , giant cell tumor is a rare entity . the differential diagnosis of giant cell tumor of clavicle which pose a diagnostic challenge both for the surgeon and the histopathologist are primary aneurysmal bone cyst , non ossifying fibroma.eosiniphilic granuloma and tuberculous osteomyelitis . giant cell tumor is basically a cellular lesion made up of sheets of plump mononuclear cells with mild pleomorphism . the cells have moderate amount of cytoplasm , oval to elongated nuceuswith moderate anisokaryosis with irregular nuclear membrane and 01 nucleolus . amongst these cells are multinucleated giant cells distributed in a regular fashion.no collagen formation , no new bone formation or no necrosis is usually seen . giant cells in abc are smaller as compared to giant cell tumor and their arrangement is loose with collagen formation . histologically it has histiocytes loaded with lipid and hemosiderin and spindle cells arranged in storiform or whorled pattern and there is presence of collagen fibres . eosinophilic granuloma has diagnostic langerhans cells and also it has large number of leucocytes , fibroblasts , plasma cells and lymphocytes . curettage remains the main stay of treatment for giant cell tumors but for giant cell tumors occurring in expendable bones like distal ulna , iliac wing or proximal fibula , en bloc resection is performed without any reconstruction . after extensive search of literature it was found that there are very few case reports describing giant cell tumors of clavicle , , . due to the paucity of the available literature no definite treatment guidelines are available on the management of giant cell tumor of clavicle . some authors reported not so favourable outcomes after total claviculectomy due to pain , loss of muscle strength and dropping of shoulder . while some authors established that total or subtotal excision of clavicle was rarely associated with loss of function . based on their reports we also performed partial claviculectomy and at one year follow up , patient was well satisfied with the clavicle is a rare site for bone tumors and shares its oncological behavior with that of flat bones rather than long bones . we have reported this case to emphasize the fact any expansile lytic lesion occurring in the lateral end of clavicle should be taken seriously and the diagnosis can be easily missed both clinically and radiographically if the clinician is not aware of the wide array of differential diagnosis which range from an infectious etiology like tuberulous osteomyelitis to a neoplastic etiology like giant cell tumour . there seems to be a difference in opinion regarding functional outcome after claviculectomy for tumour like lesions of clavicle and our case report further highlights the fact the claviculectomy without any reconstruction seems to be a good option with no disability noted in long term . since it is a single case report involving a single subject and we are not reporting the first case of this type in the literature , hence no approval was taken from the relevant ethics committee but written informed consent was taken from the patient to publish his details and clinical photographs . written informed consent was obtained from the patient for publication of this case report and accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal on request . ak , kk and js contributed to the development of protocol and edited the manuscript .
highlightsgiant cell tumour of clavicle is a rare entity.the differntial diagnosis of giant cell tumour of clavicle which pose a diagnostic challenege gor both surgeon and histopathologist are aneurysmal bone cyst , non ossifying fibroma , tubercular osteomyelitis and eosniophilic granuloma.claviculecomy is a good option with no disability noted at long term .
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the pylorus , duodenal c - loop , and ileocecal valve are the three physiological narrowings in the gastrointestinal tract , and most of the swallowed indigestible foreign bodies pass through it without complications . however , foreign bodies such as a toothbrush can not pass out of the stomach , and the gastrointestinal tract should get rid of these objects as soon as possible to avoid pressure necrosis and gastrointestinal perforation . although these objects are extracted either by endoscopy or laparoscopic gastrostomy , we devised an innovative technique by using pneumatic gastric insufflation and extracted the toothbrush by a tiny gastrotomy under local anesthesia . a 35-year - old male presented in our hospital at m.m . institute of medical sciences and research ( mmimsr ) , mullana , ambala , haryana , india in may 2013 ; he had accidentally swallowed a toothbrush two months back and there was a history of epigastric discomfort especially after meals . however , the vital signs were within normal limits and the abdomen was soft and nontender . x - ray of the abdomen suggested the presence of a foreign body and a computed tomography ( ct ) scan was done which confirmed a toothbrush lying in the stomach [ figures 1 , 2 ] . an upper gastrointestinal endoscopy was done which revealed the toothbrush in the stomach with its head toward the gastroduodenal junction . biopsy forceps were used to deliver the toothbrush by holding its bristles [ figure 3 ] . the endoscope was kept inside to insufflate and distend the stomach and a minilaparotomy with gastrotomy of 1.5 - 2 cm was performed through the midline under local anesthesia and the toothbrush was successfully removed . x - ray of the abdomen suggesting the presence of the toothbrush in the abdomen computed tomography ( ct ) scan of the abdomen showing the presence of the toothbrush in the tomach endoscopic picture of toothbrush with biopsy forceps in situ to retrieve it from the stomach in the stomach , 80 - 90% of foreign bodies pass uneventfully through the gastrointestinal tract without complications . however , objects longer than 10 cm like a toothbrush can not negotiate the duodenal c - loop due to its fixed position in the retroperitoneum , and these must be removed as soon as possible to avoid pressure necrosis and gastric perforation . more than 40 cases of toothbrush ingestion have been reported in the literature till date . however , extreme caution and experience of the endoscopist is required for such procedures . in failed cases of endoscopic removal , however , we devised a simple technique of minilaparotomy and gastrotomy under local anesthesia for removing such foreign bodies . in this technique , the stomach is distended with the help of air insufflation through the endoscope and a small incision is made in the midepigasrium under local anesthesia and the foreign object can be removed directly under the vision of the endoscope . a swallowed toothbrush is a rare occurrence and it never passes through the gastrointestinal tract spontaneously . early removal of the toothbrush is critical for reducing morbidity and mortality . in cases where endoscopic removal fails , endoscopy still remains an aid in performing surgical gastrotomy for delivering such complex foreign bodies under local anesthesia .
most of the ingested foreign bodies pass uneventfully through the gastrointestinal tract . however , long and rigid foreign bodies are associated with an increased risk of gastrointestinal impaction , perforation , and bleeding . spontaneous passage of a toothbrush has not been reported till date and the technique of its removal is a curiosity for surgeons . endoscopy is a recommended technique for the removal of such complex foreign bodies . however , if it fails , the foreign body can be removed successfully with a laparoscopic gastrotomy . we devised an innovative technique by using pneumatic gastric insufflation and extracted the toothbrush by a tiny gastrotomy under local anesthesia .
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adenomyoepithelioma has been described as a rare benign neoplasm that occurs almost exclusively in the breast . in the breast it is defined as a neoplasm composed of two structures , namely , tubules limited by an inner epithelial layer of duct - like cells and an outer layer with mostly clear myoepithelial cells . apart from the breast , adenomyoepitheliomas have been described in the salivary glands and in the lung . in the skin , these neoplasms seem to be exceedingly rare , with only few reports of adenomyoepitheliomas published to date [ 47 ] . we present the case of a 53-year - old woman with a clinically benign nodular cutaneous lesion that revealed histopathologic and immunohistochemical features of adenomyoepithelioma . a 53-year - old woman presented to her dermatologist with a 3 cm asymptomatic nodule on the left forearm . the biopsy specimen consisted of a 2.5 1.9 1.5 cm tan ellipse of skin and contained a hard yellow nodule measuring 1.5 cm in greatest dimension . examination of the sections at scanning magnification revealed beneath an intact epidermis a zone of fibrosis within the upper part of the dermis ( figure 1 a , b ) . beneath the scar , there was a large , lobulated neoplasm , which for the most part was surrounded by a compressed fibrous pseudocapsule . each of the aggregates of neoplastic cells varied in sizes and shapes and some were large and nodular ( figure 1a c ) . at higher magnification , most of the cells that constituted the lesion displayed myoepithelial differentiation with polygonal and plasmacytoid features ( figure 2 ) . the cells presented sometimes as solid sheets , but also as cords and solitary units . in places glandular and ductal structures were evident ; in some areas apocrine - type secretion within glandular structures was found ( figure 2 a c ) . f ) . in some areas cells were present within a myxoid stroma ( figure 2 g foci of cells with pleomorphic nuclei and mitotic figures were identified ( figure 2 k the myoepithelial cellular component of the neoplasm stained for s100 protein and was negative for cytokeratin and carcinoembryonic antigen ( cea ) expression . we describe a cutaneous neoplasm composed of myoepithelial cells and a focal epithelial and glandular component . the s100 positivity indicates myoepithelial cells , while epithelial - glandular cells are negative for s100 protein expression . based on findings by conventional microscopy and immunohistochemistry , this neoplasm shows myoepithelial differentiation and focal epithelial lined tubules with features of apocrine secretion , findings that are consistent with the diagnosis of primary adenomyoepithelioma of the skin . while various adnexal neoplasms with a myoepithelial cellular component have been described in the skin , adenomyoepithelioma of the skin seems to be extremely rare . what are the criteria that distinguish adenomyoepithelioma from other benign cutaneous neoplasms with myoepithelial differentiation ? while myoepithelioma is defined as a benign neoplasm consisting exclusively of myoepithelial cells embedded in a myxoid stroma , adenomyoepithelioma shows in addition to myoepithelial cells a second component displaying various degrees of epithelial - ductal differentiation . chondroid syringoma , at the other end of the spectrum is a benign adnexal neoplasm that , in addition to apocrine epithelium , manifests various degrees of follicular and/or sebaceous differentiation . clinically , myoepitheliomas occur usually in children and young adults and are located on the extremities , while mixed apocrine tumors affect older individuals and are usually found on the face . the few cases of cutaneous adenomyoepitheliomas that have been described to date , including the present case , were described in older patients and were located on the extremities and on the trunk . pleomorphic adenoma in the breast is regarded as the analogue to mixed apocrine tumor in the skin , while adenomyoepithelioma of the breast is defined as a neoplasm with nodular aggregations of clear myoepithelial cells that surround epithelial lined tubules . occasionally , the myoepithelial component predominates and loses the close association with epithelial structures . in the skin , the predominance of myoepithelial cells in relation to ductal epithelial structures lacking features of follicular and/or sebaceous differentiation separates this neoplasm from mixed apocrine tumor . therefore , myoepithelioma , adenomyoepithelioma and mixed apocrine tumor lie in the spectrum of neoplasms with pure myoepithelial differentiation at one end and apocrine - sebaceous - follicular differentiation at the other . the most important question for the patient is the biologic potential of such a lesion . while the chronic course in our patient and the majority of the histopathologic features suggest a benign neoplasm , foci of cells having pleomorphic nuclei and mitotic figures were present . both findings could indicate the potential for locally aggressive behavior and/or metastasis , as has been rarely described in adenomyoepitheliomas of the breast . therefore , adenomyoepitheliomas of the skin should be completely excised , as has been recommended in the reported case .
analogous to adenomyoepitheliomas of the breast , cutaneous adenomyoepithelioma is composed of two components , one being myoepithelial , the other ductal epithelial , and it lies in the spectrum of neoplasms having a pure myoepithelioma at one end and a mixed apocrine tumor at the other . we present the case of a 53-year - old woman with a 3 cm nodular lesion on her left forearm that had been present for many years . histopathologic examination revealed a large lobulated neoplasm surrounded by a compressed fibrous pseudocapsule . most of the cells that constituted the lesion displayed myoepithelial differentiation arranged in solid sheets , cords , and solitary units . glandular and ductal structures with features of apocrine differentiation composed the second part of the neoplasm . the myoepithelial cellular component of the neoplasm stained for s100 protein and was negative for cytokeratin and carcinoembryonic antigen ( cea ) . based on findings by conventional microscopy and immunohistochemistry , the neoplasm was classified as primary adenomyoepithelioma of the skin .
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beyond its role of cellular energy currency and phosphate donor , atp plays a potent signaling role through its extracellular release and activation of cell surface purinergic receptors . fast responses to atp are mediated through activation of p2x receptors , a family ( p2x1p2x7 ) of atp - activated ligand - gated ion channels and metabotropic effects through the activation of p2y receptors ( p2y12 , p2y4 , p2y6 , p2y1114 ) which couple to heterotrimeric g proteins . human leukocytes including monocytes , mast cells , neutrophils and central microglia express a diverse repertoire of p2y receptors though p2x1 , p2x4 and p2x7 are the dominant p2x subtypes present . activation of purinergic receptors in leukocytes is coupled to the production and secretion of cytokines and other pro - inflammatory molecules including prostaglandin e2 . purinergic receptors are associated with inflammation , with some receptors inhibited either directly ( p2y12 ) or indirectly through the activity of anti - thrombotic , in the case of platelet p2y12 , or anti - inflammatory agents , in the case of the action of statins on monocyte p2x4 . atp can act as a non - peptide damage - associated molecular pattern ( damp ) release from injured cells and tissues . in this fashion the release of cellular atp is unregulated and released due to cell lysis or puncture . cell surface purinergic receptors are activated by this atp damp signal which serves to initiate an inflammatory response and promote wound healing . however , atp can be released from cells physiologically and act as a critical signal for painful , inflammatory processes . do release other nucleotides including utp and udp - sugars but our focus here is atp release . mechanisms of atp release during physiological processes remain diverse and controversial . investigation into how cellular stress stimulates atp release in non - leukocytes suggests roles for connexin and pannexin hemichannels , maxi- and volume - regulated anion channels and efflux through the p2x7 receptor , though release routes and signal transduction mechanism underlying atp release in leukocytes remain poorly defined . in neutrophils atp is released in response to activation of fmlp receptor by bacterially derived n - formylmethionine . the precise release mechanism is unclear but occurs at the leading edge of migrating neutrophils . released atp and its subsequent metabolism to adenosine at the cell surface activate p2y2 and a3 receptors serving to direct cell orientation and promote migration in response to chemotactic signals . bacterially derived lipopolysaccharide can stimulate central microglia to secrete atp which in - turn activates neighboring astrocytes and modulates excitatory neurotransmission . this raises the possibility that atp can act in a feed forward loop possibly amplifying responses to itself or other external cues which couple to atp secretion . furthermore , such constitutive secretion leads to activation of cell surface gq - coupled p2y receptors which elevate intracellular calcium levels through release of calcium . this mode of constitutive secretion generates a constant pericellular atp cloud or halo which appears to be important in regulating intracellular calcium homeostasis following p2y receptor activation . in cells of hematopoietic lineage such as monocyte / macrophage , nk killer and mast cells , secretory lysosomes have evolved as bifunctional organelles which combine classical degradative properties with secretion . the mechanism of lysosomal atp transport remains undefined though a nucleotide transporter ( v - nut ) has been characterized for other atp containing vesicles . the signal transduction coupling external cues such as cytokines , chemokines and bacterially derived molecules to atp release is poorly defined . what are the release machines in leukocytes and do they differ from other cell types ? it is expected that therapeutic intervention in agonist stimulated atp release is a potentially novel route to pharmacological modulation of innate immune responses but also in chronic inflammatory disease where normal inflammatory responses are heighten and act deleteriously .
release and reception of extracellular atp by leukocytes plays a critical role in immune responses to infection , injury and cardiovascular disease . leukocytes of both the innate , adaptive immune and central nervous system express a repertoire of cell surface receptors for atp ( p2x and p2y receptors ) and its metabolites . atp acts as a damage - associated molecule pattern ( damp ) released by injured or dying cells . detection of released atp by neighboring leukocytes initiates inflammation and wound healing . however , recent evidence from our group and others suggests atp release by leukocytes themselves serves to regulate homeostatic mechanisms and coordinate responses to external pro - inflammatory cues . examples include the homeostatic control of intracellular calcium and regulation of migratory guidance during chemotactic response to external cues . though there has been some progress in elucidating atp release mechanisms of some mammalian cells types , release conduits and coupling signal transduction machinery remain larger elusive for leukocytes . our recent studies suggest a role for secretory lysosomes in releasing atp in monocytes . though poorly defined , targeting atp release mechanisms in leukocytes have great anti - inflammatory potential .
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most pituitary adenomas are clinically inactive . in patients with long - standing compression of the optic chiasm , ganglion cells may undergo axonal degeneration . spectral domain optical coherence tomography ( sd - oct ) is able to identify retinal nerve fiber layer ( rnfl ) and ganglion cell loss in the retina . we present a case in which sd - oct was used to diagnose an asymptomatic pituitary macroadenoma . sd - oct identified atrophy of the ganglion cell and nerve layers , with preservation of outer layers bilaterally . as macroadenomas enlarge , they can induce uncrossed axon loss , resulting in nasal field defects and reduced visual acuity . in these cases , there is atrophy of the nasal and temporal portions of the optic disc , thus occupying a horizontal band across the disc . sd - oct is able to identify rnfl loss in eyes with band atrophy of the optic nerve , which correlates with visual field defects found in perimetry . sd - oct is a useful tool to assess the structural and functional damage of ganglion cells . in our case the sd - oct demonstrated a symmetrical loss of the rnfl and the ganglion cell layer in both eyes , indicating important optic nerve damage . pituitary adenoma is the most common cause of the chiasmal syndrome.1 the tumor is classified based on size as microadenoma , smaller than 10 mm , or as macroadenoma when it exceeds 10 mm in diameter.2 some tumors secrete one or more hormones in excess , so - called secretory pituitary adenomas , but most are clinically inactive.3 classically , the nonsecretory tumors present with vision loss,4 whereas patients with secretory tumors are usually referred to ophthalmologists for evaluation due to hormonal imbalances that affect bodily functions.5 in patients with long - standing compression of the optic chiasm , ganglion cells may undergo axonal degeneration4 . optical coherence tomography ( oct ) is able to identify retinal nerve fiber layer ( rnfl ) and ganglion cell loss in retina.6,7 we present a case in which the oct ( 3d oct-2000 spectral domain oct , topcon corporation , tokyo , japan ) was used to diagnose an asymptomatic pituitary macroadenoma . the authors adhere to the international standards developed in the 2 conference on research integrity in singapore ( 2010 ) , while performing this study . the patient agreed to have their case published , and all information presented is non - identifiable.8 a 48-year - old , caucasian female presented with progressive vision loss in both eyes for several months . her past ocular , medical , and family history was non - contributory with no fatigue , loss of libido , no mood change , and no weight change . on examination , best - corrected visual acuity was hand motion in the right eye and 2/3 in the left eye . spectral domain optical coherence tomography ( sd - oct ) demonstrated diffuse atrophy of the ganglion cell and nerve layers , with preservation of outer layers bilaterally ( figure 1 and figure 2 ) . magnetic resonance imaging of the brain was performed and a pituitary macroadenoma with suprasellar extension was observed ( figure 3 ) . pituitary adenomas are common benign tumors of the pituitary gland that account for 12% of all intracranial tumors.9 people can develop pituitary adenomas at any age . some adenomas secrete hormones in excess . of the secretory tumors , the most common are prolactinomas.5 they may be relatively small when detected . the most common symptoms include headaches , menstrual changes in females , sexual dysfunction in males , vision problems , and behavioral changes.5 diagnostic imaging may include a high - resolution , t1 weighted , gadolinium enhanced magnetic resonance imaging.10 other secretory tumors may secrete corticotropin , growth hormone , gonadotropins , or thyroid - stimulating hormone.11 adenomas remain confined to the pituitary gland , but when a pituitary adenoma is 10 mm it is called a macroadenoma . macroadenomas can compress the rest of the pituitary gland and surrounding structures , such as the crossing retinal ganglion cell axons in the optic chiasm , resulting in bitemporal visual field loss.4 as the tumor enlarges , it has been shown to induce uncrossed axons loss , resulting in nasal field defects and reduced visual acuity . in these cases , there is atrophy of the nasal and temporal portions of the optic disc with relative sparing of the superior and inferior parts where the majority of temporal fibers enter . the optic atrophy occupies a horizontal band across the disc , called bowtie or band atrophy.12,13 oct is a noninvasive technique that allows cross - sectional imaging of the retina and quantifies the thickness of the rnfl around the optic nerve head . a number of studies have demonstrated that oct is able to identify rnfl loss in eyes with band atrophy of the optic nerve.6,7 the degree of rnfl thickness reduction has been shown to correlate with that of visual field defects using goldmann perimetry4 and automated static perimetry.14,15 this fact gives oct prognostic value regarding the visual outcome , as demonstrated in different studies.16,17 oct is a useful tool to assess the structural and functional damage of ganglion cells , objectively and quantitatively.4 in our case the sd - oct demonstrates a symmetrical loss of the rnfl and the ganglion cell layer in both eyes , indicating important optic nerve damage . sd - oct can help in the diagnosis of adenomas in equivocal situations in which there is only subtle optic nerve pallor in the setting of nonspecific visual complaints or visual field defects , or when patients are referred to screen for subclinical compressive optic neuropathies .
introductionmost pituitary adenomas are clinically inactive . in patients with long - standing compression of the optic chiasm , ganglion cells may undergo axonal degeneration . spectral domain optical coherence tomography ( sd - oct ) is able to identify retinal nerve fiber layer ( rnfl ) and ganglion cell loss in the retina . we present a case in which sd - oct was used to diagnose an asymptomatic pituitary macroadenoma.clinical casea 48-year - old female presented with progressive vision loss in both eyes . sd - oct identified atrophy of the ganglion cell and nerve layers , with preservation of outer layers bilaterally . magnetic resonance imaging of the brain showed a pituitary macroadenoma . the pathological diagnosis was nonfunctioning adenoma.discussionas macroadenomas enlarge , they can induce uncrossed axon loss , resulting in nasal field defects and reduced visual acuity . in these cases , there is atrophy of the nasal and temporal portions of the optic disc , thus occupying a horizontal band across the disc . sd - oct is able to identify rnfl loss in eyes with band atrophy of the optic nerve , which correlates with visual field defects found in perimetry . sd - oct is a useful tool to assess the structural and functional damage of ganglion cells . in our case the sd - oct demonstrated a symmetrical loss of the rnfl and the ganglion cell layer in both eyes , indicating important optic nerve damage .
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percutaneous nephrolithotomy ( pcnl ) is a widely accepted treatment for urinary calculi > 2 cm or resistant to other modalities . complications of this procedure are well reported ; however , to our knowledge no mention has occurred in the literature of a retained nephrostomy tube after pcnl . thus , we will present the management of a retained council tip catheter after pcnl . a 76-year - old gentleman underwent a pcnl for a 2.2x1.4-cm lower pole calculus via an uneventful upper pole puncture . incomplete stone fragmentation was obtained using the ultrasonic lithotripter ; therefore , a 24-french council tip nephrostomy tube was placed , the balloon was inflated with 3 ml of sterile water , and the patient was scheduled for a second look nephroscopy when a holmium laser was available . one week later at the second look nephroscopy , attempts to deflate the catheter balloon were unsuccessful . after transection of the balloon port valve , attempted passage of a 0.038 movable core bentson guidewire down the balloon port was unsuccessful . an antegrade nephrostogram outlined the catheter balloon within the renal pelvis ( figure 1 ) . with the c - arm in a vertical orientation , an 18-gauge chiba needle ( cook , bloomington in ) was advanced through a puncture site directly over the balloon under fluoroscopic guidance . the balloon was successfully punctured by the chiba needle with clear efflux through the needle . contrast was then instilled through the chiba needle to evaluate the integrity of the balloon and exclude the possibility of retained balloon fragments from a ruptured balloon ( figure 2b ) . flexible nephroscopy was performed to evaluate for balloon fragments , which were not identified , and to remove the lower pole stone . ( a ) chiba needle ( white arrow ) has punctured and drained the catheter balloon . ( b ) contrast injected via chiba needle has filled the catheter balloon ( dark arrow ) and confirmed its integrity . a retained urethral catheter due to balloon malfunction is a well - recognized urologic complication . many solutions to this problem have been attempted , including overinflation until rupture , puncture of the balloon via a wire through the balloon port , and puncture of the balloon under ultrasound guidance via a suprapubic or transvaginal route . given this infrequent but known complication of balloon - type cystostomy catheters , it is not surprising that a similar complication occurred in our patient . our familiarity with the use of the chiba needle and 3-dimensional fluoroscopy for percutaneous renal access facilitated percutaneous puncture of the catheter balloon . if equipment or experience limits the urologist 's ability to perform this technique , collaboration with an interventional radiologist using conventional c - arm or ultrasound for guidance should be considered . prevention of a retained catheter due to a balloon - port malfunction requires use of sterile water instead of saline and limiting the time of catheter placement . malecot tubes have been utilized for nephrostomy drainage , but these tubes are not without difficulties . tissue bridge formation over flanges of the tube has occurred , requiring endoscopic removal . tubeless pcnl would avoid these issues entirely ; however , the tubeless technique is contraindicated in the face of significant postoperative bleeding , urinary extravasation , ureteral obstruction , or anticipated need for a second - look nephroscopy . pigtail catheters following pcnl may represent a good alternative for drainage and access , while minimizing the risk of catheter malfunction or retention .
percutaneous nephrolithotomy is a widely accepted treatment for urinary calculi , but it is not without complications . we present the case of a 76-year - old male with a retained council tip catheter after percutaneous nephrolithotomy . fluoroscopic guidance was used to perform percutaneous puncture of the catheter balloon , and the catheter was removed without complication . advantages of various nephrostomy tube designs and additional measures to prevent this type of complication are discussed .
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a seventy five year old libyan man was seen in the urology department of tripoli medical centre , tripoli , libya with six month history of left loin pain . the patient noted a mass in the left loin two days before he was assessed in the hospital . the urine analysis revealed pus cells , protein , and no sugar , but urine culture yielded no bacterial growth . liver function tests showed slightly elevated alkaline phosphatase at 160 iu / l ( 44 to 147 iu / l ) , and lactate dehydrogenase at 152 iu / l ( 105 - 333 full blood count revealed haemoglobin of 8.3g% , ( 1315g% ) , white cell count of 14.7103/mm3 ( 411103/ mm3 ) , and platelets of 3.4 105/ mm3 ( 1.54.5 105/ mm3 ) . the patient 's vomiting subsided with proton pump inhibitors , but the loin pain showed partial response to analgesic . abdominal ct - scan showed an irregular mass in the upper pole of left kidney . the patient was posted for partial nephrectomy with the provisional diagnosis of renal tuberculosis or renal infarction involving upper pole . pathologically , the kidney was noted to have greyish white soft tissue mass , firm in consistency measuring 6x4x2 cms , with adjacent dilated calyces . the renal resection margin and external surface appeared free of tumour ( figure 2 ) . microscopically , the tumour consisted of pleomorphic squamoid cells arranged in nests and sheets with few foci of epithelial pearl formation and keratinisation . areas of necrosis and frequent mitosis were noted . the malignant cells nore were seen to infiltrate the adjacent renal parenchyma as nests of cells and also focally renal capsule ( figures 3 & 4 ) . the dilated calysis showed areas of squamous metaplasia and severe dysplasia with focal invasion of basement membrane into renal parenchyma to form the tumour ( figure 5 ) . photomicrograph revealing calyseal subepithelial invasion of malignant cells ( left ) and invasion of renal parenchyma adjacent to glomerulus with epithelial pearl formation ( right ) . postoperative chest x - ray ( pre - operative chest x - ray was normal ) revealed massive pleural effusion in the left lower lobe ( figure 6 ) . however , no histological typing is available as tissue showed only necrotic material with scanty malignant cells . he was put on cisplatin and sunitinib on the suspicion of primary lung tumour and showed no response . the patient was suspected clinically to have renal tuberculosis and was later found to have renal squamous cell carcinoma with possible lung secondaries . the common renal malignancy in adults is of clear cell type , followed by papillary carcinoma and chromophobe cell carcinoma [ 1 , 2 ] . primary neoplasms of the renal collecting system are rare , accounting for less than 5% of urothelial tumours in urinary system [ 4 , 5 ] . the transitional cell type is the most frequent ( 85%95% ) , followed by squamous cell carcinoma ( 6%15% ) and adenocarcinoma ( 7% ) . usually , renal squamous carcinoma is highly aggressive and of a high grade at presentation . haematuria , the classical presenting complaint of renal cell carcinoma , is not common in this entity as in this case . the incidence of co - existing stone was reported in a wide range of 18% to 100% . the present case demonstrates the transition from calyseal urothelium to squamous metaplasia , dysplasia and invasive squamous malignancy . there is a well known association between chronic pyelonephritis , renal pelvic stones , phanecetin ingestion and radiotherapy with squamous cell carcinoma . the case was clinically suspected as renal tuberculosis because of sterile pyuria and past history of cough with fever . the renal malignancy was not considered due to loin pain and absence of haematuria as the renal cell carcinoma classically presents as painless haematuria . the tumour can be difficult to diagnose by imaging modalities as the usual features are the presence of calculi and hydronephrotic changes with ureteral obstruction . filling defects or obstructive lesions in the renal pelvis by intravenous / retrograde urography or detection of a solid mass by ultrasonography can be the signs of the tumour . central renal squamous cell carcinoma presents more with intraluminal components and is usually associated with lymph node metastasis whereas peripheral renal squamous cell carcinoma presents with prominent renal parenchymal thickening and might invade the perirenal fat tissue before lymph node or distant metastasis could be identified . the survival of patients with central renal squamous cell carcinoma was reported to be significantly shorter than those with peripheral renal squamous cell carcinoma . nativ and colleagues reported that patients with locally invasive renal squamous cell carcinomas had 1 and 2 yearsurvival rates of 33% and 22% , respectively . if metastasis develops , adjuvant chemotherapy or irradiation has little effect on the unfavourable prognosis . our patient underwent partial nephrectomy because of suspicion of renal tuberculosis and was later found have pulmonary metastasis . in conclusion , this patient presented with lion pain and a mass without haematuria and was found to have renal squamous cell carcinoma with lung metastasis . as renal calyseal tumours could present with atypical features , a proper preoperative workup may help in better management though outcome is short lived .
a seventy five year old gentleman with the clinical diagnosis of renal tuberculosis was found to have renal squamous cell carcinoma . the clinical presentation and management are being discussed .
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it is one of the most commonly used medicines in the treatment of rheumatoid arthritis or psoriatic arthritis . we report the case of an adult woman with psoriatic arthritis who developed acute severe hepatitis following long - term treatment with mtx . the periodic biological monitoring of liver enzymes : alanine aminotransferase ( alat ) , aspartate aminotransferase ( asat ) , gamma - glutamyl transpeptidase ( gamma - gt ) performed every 6 months did not reveal any abnormalities . mtx was stopped in the year 2005 due to remission of the disease . in 2009 , it was reintroduced at a dose of 15 mg / week due to decompensation of the disease . monitoring of liver enzymes performed every 6 months showed an elevation < 2 times the normal levels on average of asat and alat since 2010 . assessing the risk - benefit ratio and given the severe decompensation of the disease , mtx was continued . in may 2013 , patient presented with jaundice following the development of asthenia and weight loss . laboratory investigation showed the following results : prothrombin time ( 71% ) , asat 580 u / l , alat 620 u / l , serum gamma - gt 146 iu / l , serum alkaline phosphatase 170 iu / l and total serum bilirubin 12 mg / dl . antimitochondrial antibodies ( titer > 1/40 ) and antinuclear antibodies ( titer of 1/640 ) were detected in serum . routine laboratory investigations for infection with hepatitis a , b , and c viruses and serology for cytomegalovirus and epstein - barr virus were negative . methotrexate was stopped , and therapy with oral methylprednisolone ( 80 mg / day ) was initiated . a significant improvement in the following parameters was observed a week later : prothrombin time ( 88% ) , asat 270 u / l , alat 340 u / l , s. gamma - gt 122 iu / l , and s. alkaline phosphatase 173 iu / l . after 4 months , all laboratory parameters had normalized , and serum was negative for antimitochondrial and antinuclear antibodies . patients taking mtx are more likely to discontinue therapy because of adverse drug effects rather than because of lack of efficacy . adverse effects of mtx are , usually , mild and self - limiting , but may include serious adverse drug reactions such as hematopoietic suppression , pulmonary , and hepatotoxicity . hepatic fibrosis is a usual adverse reaction reported with long - term use of mtx therapy in psoriatic patients . causality assessment of the adverse drug event ( ade ) was carried out using who - umc criteria , naranjo 's scale , and roussel uclaf causality assessment method ( rucam ) scale . in this case , patient improved on withdrawal of the drug , and there were no other confounding factors that could have caused this adverse effect . hence , the ade was probably caused by mtx ( who - umc criteria : probable ; naranjo 's score : 7 , probable ; and rucam scale : 5 ) . in addition , sufficient and continuous exposure to the drug , lack of previous evidence of autoimmune disease and complete resolution of the condition within 4 months of discontinuation of the suspected drug confirmed the diagnostic criteria of drug - induced autoimmunity . the first case of autoimmune hepatitis associated with mtx was described in 2011 in a 57-year - old man after 11 years of treatment with mtx . a complete normalization of laboratory parameters was obtained 5 months after stopping this drug . to the best of our knowledge , it , usually , occurs at higher doses and also positively correlates with the cumulative dose of drugs . other risk factors for the development of elevated liver enzymes and changes in liver biopsy include obesity , higher alcohol intake prior to commencement of mtx and persistent hepatitis b or c infection . in the present case , appearance of autoantibodies and transitory autoimmune disease associated with infliximab in a patient with psoriatic arthritis has also been documented and the preexistent serological signs of autoimmunity are believed to be a risk factor for the development of similar autoimmune reactions . this report confirms the need to monitor liver enzymes carefully in patients with psoriatic arthritis using long - term treatment with mtx . clinicians must collaborate with the pharmacovigilance center to detect and notify serious adverse effects of mtx . further research is needed to identify the exact mechanism and establish , if possible , primary means of prevention of this serious adverse effect .
methotrexate ( mtx ) is one of the most commonly used medicines in the treatment of psoriatic arthritis . the drug can produce steatosis and cirrhosis . autoimmune hepatitis is a rare and serious adverse effect . we describe the case of a 53-year - old woman who developed autoimmune hepatitis after a long - term use of mtx for psoriatic arthritis . hepatitis was completely resolved 4 months after stopping this drug . the pathophysiologic mechanisms of a drug - induced autoimmunity are unclear and complex . this report confirms the need to monitor liver enzymes carefully in patients using long - term treatment with mtx for psoriasis or rheumatoid arthritis .
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blunt traumatic infrarenal aortic injuries are rare , with a few case reports in the literature . a more common occurrence is an intimal flap which may form after blunt injury to the aorta , and most of these will resolve with anticoagulation alone [ 1 , 2 ] . blunt aortic injury which initially presents as an intimal flap ( grade i ) may progress and evolve into an intramural hematoma ( grade ii ) or a pseudoaneurysm ( grade iii ) . aortic intimal flap progressing to dissection or pseudoaneurysm is an occurrence identified in 2% or less of the patients , and the majority of these occur in the thoracic aorta [ 1 , 2 ] . this article presents the case of a young woman found to have an intimal flap in the infrarenal abdominal aorta after a car accident , and the injury progressed to a pseudoaneurysm over 3 months . a 47-year - old woman with a history of hypertension and smoking was involved in a high - speed motor vehicle accident and presented to the emergency department with back pain . computed tomographic ( ct ) scans were obtained of her abdomen and pelvis . on a single image from the abdominal scan , what appeared to be either an intimal flap or a small amount of contrast extravasation outside of the infrarenal aorta could be seen . her care providers had differing opinions as to what was being seen on the image . since the abnormality was only seen on a single image , the exact etiology could not be delineated ( fig . the patient was observed in the hospital overnight , had minimal complaints the next day , and was discharged on aspirin . figure 1:initial ct scan shows enhancing vascular abnormality on the right side of the aorta , shown by the black arrow . initial ct scan shows enhancing vascular abnormality on the right side of the aorta , shown by the black arrow . a follow - up ct scan was obtained 3 months later . at the location of the previous aortic abnormality , there was a 3 1 cm pseudoaneurysm that had formed and could now be clearly seen ( figs 2 and 3 ) . figure 2:pseudoaneurysm now clearly seen 3 months later . figure 3:black arrow pointing to pseudoaneurysm with enhancing flow outside of the aortic lumen . operative repair was recommended , but the patient s aorta measured only 18 mm at the aortic bifurcation and was too small for even the smallest endovascular stent graft available at our institution . therefore , an open replacement of her infrarenal aorta was performed using a dacron 22 mm graft . in the operating room , after the aorta was clamped and opened , a 4 mm tear in the intima had formed a mature channel into the pseudoaneurysm . the patient recovered very well and was discharged from the hospital without complications . at her 2-year follow - up , she was in good health and had no further problems , but continues to smoke . blunt abdominal aortic trauma occurs in only 0.040.1% of all nonpenetrating traumas [ 3 , 4 ] . and the vast majority of these ( 92% ) are associated with multiple other injuries [ 3 , 5 ] . our patient had no other injuries , and there was no retroperitoneal hematoma or other signs of vascular injury in the area , as is usually seen on ct when the aorta is injured [ 35 ] . the lack of other radiographic traumatic findings added to the initial confusion as to whether there was a true injury to the aorta , or if the single image was showing artifact or an enlarged vertebral vessel . since the majority of intimal flaps heal with anticoagulation alone ( 55% ) or remain stable ( 40% ) , a conservative plan including daily aspirin and re - imaging in 3 months was chosen . when repeat imaging showed a 3 cm pseudoaneurysm , operative repair was necessary . endovascular repair was initially chosen for this case , but after measuring the patient s aorta , femoral vessels and the degree of taper at the bifurcation , there was not an appropriate endovascular prosthesis available in such a small size . only one study has compared conventional open repair with endovascular repair for blunt injuries of the abdominal aorta and found similar results in both groups . endovascular repair is generally favored since the injuries tend to be focal and discrete , making them quite amenable to a short graft . further , the patients usually have other significant injuries that would complicate a long , and potentially morbid , open operation on the aorta . although all blunt infrarenal aortic injuries are unusual , most of them consist of intimal flaps or dissections [ 3 , 58 ] . a post - traumatic pseudoaneurysm , as this patient had , is even more unusual after blunt trauma . one prior publication did document the delayed occurrence of a post - traumatic infrarenal aortic pseudoaneurysm that was diagnosed 3 years after injury . endovascular repair for pseudoaneurysms must include post - procedure angiography to document no evidence of a type i or type ii endoleak , which could allow filling of the aneurysm sac and later rupture . this case report highlights the need for delayed imaging for even minor intimal flaps or small aortic injuries after trauma , since progression does occur . while the vast majority of these injuries improve over time or remain stable , a small percentage will worsen and require intervention .
blunt traumatic infrarenal aortic injuries are unusual , and the formation of a delayed pseudoaneurysm of the aorta is even more rare . in this report , a young woman developed a small intimal flap of the infrarenal aorta after a motor vehicle accident which progressed into a 3 cm pseudoaneurysm after 3 months . operative repair was successful and the patient recovered . this case illustrates the importance of repeat imaging of small blunt aortic injuries since progression can occur .
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strains carrying proa - tev - rpt1 were grown in 6 l of sd medium to od600 0.8 to 1.2 . cells were harvested , washed once with ice - cold water and drop - frozen in liquid nitrogen . frozen yeast samples were then ground using an mm301 grinding mill ( restch ) under liquid nitrogen following manufacturer s instructions , or using a mortar and a pestle as previously described24 . ground powder was hydrated in proteasome buffer ( 50 mm tris - hcl [ ph7.5 ] , 5 mm mgcl2 , 1 mm edta , and 10% glycerol ) supplemented with 2 mm atp , protease inhibitor tablets ( complete , roche ) , 2 mm pmsf , 1 mm benzamidine , 10 g / ml pepstatin a , and 1 g / ml antipain . cell extracts were cleared at 30,000 g for 30 min at 4c , and the supernatants were mixed with rabbit igg resin ( cappel , mp biomedicals ) for 90 min at 4c . resins were collected at 900 g for 2 min at 4c and washed with proteasome buffer containing 50 mm nacl three times , followed by a final wash with proteasome buffer alone . bp1 was then released from the resin by incubating with actev protease ( invitrogen ) at 2.5 unit / l culture in proteasome buffer containing 2 mm atp and protease inhibitors for 1 hr at 30c . eluates were concentrated using ultrafree-0.5 centrifugal filter device with 30 kda nmwl ( millipore ) . strains carrying proa - tev - rpt1 were grown in 6 l of sd medium to od600 0.8 to 1.2 . cells were harvested , washed once with ice - cold water and drop - frozen in liquid nitrogen . frozen yeast samples were then ground using an mm301 grinding mill ( restch ) under liquid nitrogen following manufacturer s instructions , or using a mortar and a pestle as previously described24 . ground powder was hydrated in proteasome buffer ( 50 mm tris - hcl [ ph7.5 ] , 5 mm mgcl2 , 1 mm edta , and 10% glycerol ) supplemented with 2 mm atp , protease inhibitor tablets ( complete , roche ) , 2 mm pmsf , 1 mm benzamidine , 10 g / ml pepstatin a , and 1 g / ml antipain . cell extracts were cleared at 30,000 g for 30 min at 4c , and the supernatants were mixed with rabbit igg resin ( cappel , mp biomedicals ) for 90 min at 4c . resins were collected at 900 g for 2 min at 4c and washed with proteasome buffer containing 50 mm nacl three times , followed by a final wash with proteasome buffer alone . bp1 was then released from the resin by incubating with actev protease ( invitrogen ) at 2.5 unit / l culture in proteasome buffer containing 2 mm atp and protease inhibitors for 1 hr at 30c . eluates were concentrated using ultrafree-0.5 centrifugal filter device with 30 kda nmwl ( millipore ) .
substrates of the proteasome are recognized and unfolded by the regulatory particle ( rp ) , then translocated into the core particle ( cp ) to be degraded1 . a hetero - hexameric atpase ring , containing subunits rpt1-rpt6 , is situated within the base subassembly of the rp1 . the atpase ring sits atop the cp , with the rpt c - termini inserted into pockets in the cp26 . we have identified a novel function of the rpt proteins in proteasome biogenesis through deleting the c - terminal residue from each rpt . our results indicate that assembly of the hexameric atpase ring is templated on the cp . we have also identified an apparent intermediate in base assembly , bp1 , which contains rpn1 , three rpts , and hsm3 , a chaperone for base assembly . the rpt proteins with the strongest assembly phenotypes , rpt4 and rpt6 , were absent from bp1 . we propose that rpt4 and rpt6 form a nucleating complex to initiate base assembly , and that this complex is subsequently joined by bp1 to complete the rpt ring . our studies show that assembly of the proteasome base is a rapid yet highly orchestrated process .
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kyphosis is a progressive curvature of the thoracic spine , which results in rounding or bowing of the back . a curved spine accompanied by restricted neck motion and poor lung reserve present challenges for an ophthalmic surgeon , who is used to patients neck and head resting flat on the operating table and the eye in the horizontal position . intraoperative difficulties include poor view , inability to maneuver surgical instruments properly and constant threat of elevated vitreous pressure . several innovative solutions have been published , including the maximal reverse trendelenburg position and pillows under the head and neck , use of a donut and wedge - shaped head positioner , allowing patients to remain in the upright sitting position while their head is positioned horizontally in a modified waiting room chair , performing surgery while standing [ 5 , 6 ] , using a 4-section orthopedic operating table , or laying the patients on their sides . however a 49-year - old obese male with controlled secondary glaucoma needed cataract surgery in the right eye . the past ocular history was positive for recurrent uveitis and glaucoma shunt surgery in the right eye . cataract density was + 4 , and the best - corrected visual acuity was 20/200 . the anterior chamber was shallow and the glaucoma shunt tube was visible at the 11 o'clock position . the patient was planned for pupillary dilation using iris retraction hooks , lens capsule staining with trypan blue and phacoemulsification with intraocular implantation under local anesthesia . the anesthesiologist was reluctant to use general anesthesia because of the patient 's disability and poor health . in the operating room , the patient could only recline to about 40 from the vertical axis despite maximal reverse trendelenburg position and a number of pillows under his back , neck , head and legs ( fig . 2 ) . while trying to focus the microscope , it became evident that by grasping the eye at the inferior limbus with a fine forceps and pulling it upwards , it could be moved to a more desirable horizontal position . a single 7/0 vicryl ( polyglactin ) corneal retraction suture was placed at the limbus at the 6 o'clock position to achieve the desired view ( fig . the assistant surgeon was able to rotate the eye to the desired positions during the whole procedure by relaxing or pulling the suture . patients with kyphosis may present several unique challenges to an eye surgeon , the foremost being the inability to lay the head and neck in the ideal horizontal position . the resulting poor view makes focusing and manipulation of tissues and instruments difficult . tilting the head end of the operating table down and raising the patient 's legs may compromise venous return from the orbit , causing venous engorgement and elevated posterior vitreous pressure during surgery . in addition , the patient 's poor pulmonary reserve itself may further impede orbital / jugular venous return , resulting in increased posterior vitreous pressure . other features of kyphosis that may adversely affect intraocular surgery are tenderness and stiffness of the back and difficulty in breathing . several practical solutions have been described by very experienced surgeons but complications may still occur . a wedge - shaped pillow with a built - in donut for the positioning of the head has been used by some surgeons . livingston and mackool recommended using more than one of these positioners in severe cases . another approach suggested performing cataract surgery while standing , using loupes and making an inferotemporal scleral tunnel [ 5 , 6 ] . the surgeons were unable to remove the subincisional cortex because of a shallow anterior chamber secondary to elevated posterior vitreous pressure . prasad et al . recommended using a 4-section orthopedic operating table to enable an extreme reverse trendelenburg position for cataract and glaucoma surgery . however , because of a blurred vision , these authors were unable to perform curvilinear capsulorhexis and had to resort to can - opener capsulotomy . in short , there is no single solution for the different groups of difficult patients . we have found that a single retraction suture at the inferior limbus can rotate the eye to any desired position . this may allow both the patient and surgeon to be in a comfortable position without compromising good view . it may be tried practically in all patients with kyphosis or other conditions that prevent them from reclining such as torticollis , chronic obstructive pulmonary disease , chronic congestive heart failure or morbid obesity . the surgeon can still sit on the preferred temporal side of the patient and perform intraocular surgery of choice . with this maneuver , it may be unnecessary to resort to extreme and uncomfortable tilting of the patient and avoid complications such as those described above . the authors have no financial or proprietary interest in the materials described in the article .
we describe a 49-year - old man with advanced kyphosis and dense cataract , who could only recline to about 40 from the vertical axis despite a maximal reverse trendelenburg position and pillows under the head , neck , shoulders and knees . with a single corneal retraction suture at 6 o'clock , the eye could be rotated horizontally , which enabled the surgeon to perform a complex cataract surgery despite prior glaucoma shunt , posterior synechiae , a small pupil and the need to stain the capsule . as the eye can be brought into any desired position with a retraction suture , patients with kyphosis or other conditions that prevent them from assuming a supine position can still have safe intraocular procedures . this maneuver reduces the need to tilt patients to an uncomfortable position that may cause pain , increased breathing difficulty and elevated posterior vitreous pressure .
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assisted / supported mechanical ventilation is designed to interact with patient muscle activity and ' share ' the work of breathing . if properly done , assisted / , the ventilatory 's flow and pressure delivery must synchronize with patient effort during all three phases of breath delivery : breath initiation , flow delivery , and breath termination ( cycling ) . dys - synchronous interactions can overload ventilatory muscles ( ' imposed ' loads ) , compromise alveolar ventilation , overdistend alveolar units , disrupt sleep patterns , and cause patient discomfort , prompting additional sedation . carlucci and colleagues have recently studied patient - ventilator interactions in 69 acutely ill patients receiving non - invasive ventilation ( niv ) for a variety of reasons . they grouped these patients according to obstructive or restrictive pathophysiologies and carefully measured respiratory system mechanics ( including esophageal pressure - a surrogate for pleural pressure ) . the patients were then initiated on niv according to a standard protocol and were assessed for triggering dys - synchronies defined as missed triggers , delayed triggers , or double triggers . importantly , they found that these dys - synchronies were common ( 58% of patients experienced them with missed triggers being the most frequent ) . trigger dys - synchronies were associated with niv intolerance and their prevalence was similar among the different patho - physiologic patterns . this study is important as it underscores that patient - ventilator trigger dys - synchrony is common and can be a major barrier to niv effectiveness . this study also underscores the fact that the causes of trigger dys - synchrony are complex and affect all types of patients requiring assisted / supported mechanical ventilation [ 3 - 5 ] . like invasive mechanical ventilation , niv trigger dys - synchrony can stem from insensitive / poorly responsive breath triggering mechanisms . however , other factors also involved likely include ventilatory muscle loading patterns and their effects on the ventilatory controller , ventilatory muscle function , lung volumes at end - expiration ( that is , intrinsic positive end - expiratory pressure ( peep ) ) , gas exchange effectiveness , and cortical inputs to the patient 's ventilatory control center such as pain [ 3 - 5 ] . in addition , the triggering process with niv can be further hampered by the mask system with its inherent leaks and claustrophobia exaggerating dyspnea and neural drive . importantly , this study did not address issues of flow synchrony or cycle synchrony , two other forms of patient ventilator interactions likely to impact niv tolerance but not well studied in the current literature . flow synchrony is defined as the ability of the ventilator to provide flow in accordance with patient demand as manifest by the ventilatory muscle contraction pattern . if flow is synchronous with that contraction pattern , the inspiratory muscle pressure time profile conceptually should resemble a near normal pattern . instead it means providing flow to ' re - shape ' the inspiratory muscle 's pressure - time or pressure - volume profile to a more physiologic shape . in general , the variable flow features of pressure targeted breaths tend to flow synchronize easier with patient effort than clinician set flow - volume targeted breaths . an assisted / supported mechanical breath termination shorter than the neural inspiratory time ( machine ti < neural ti ) can lead to muscle activity beyond the machine 's flow delivery phase , which can lead to high muscle loading , excessive tidal volumes and/or triggering of a second breath . in contrast , when mechanical breath cycling terminates after the inspiratory effort has ended ( machine ti > neural ti ) , dyspnea and expiratory muscle recruitment may occur in an effort to terminate the breath . importantly , it is worth noting that dys - synchronous interactions often result in anxiety and dyspnea , which can stimulate overall ventilatory drive . thus , improving synchrony in one area ( for example , triggering ) can help facilitate achieving synchrony in other areas ( for example , flow demand ) . in summary , patient - ventilator interactions are complex and are important to recognize and manage during both invasive and non - invasive mechanical ventilation . carlucci and colleagues have demonstrated how common the trigger dys - synchronies are during niv and that understanding them requires assessment not only of respiratory system mechanics , but also the complex interactions of neural drive , gas exchange , muscle function , and muscle loading . much research is needed in this area as dys - synchronies of all types can lead to overloaded muscles and dyspneic patients requiring sedation - two factors that clearly can lead to prolonged mechanical ventilation days and poor outcomes .
patient - ventilator trigger dys - synchronies are common with the use of assisted forms of mechanical ventilatory support , including non - invasive mechanical ventilatory support ( niv ) . future system designs need to address this in order to improve the effectiveness of niv .
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postanginal septicaemia in the early 20 century ; originally associated with a mortality reaching 90% it involves a classic triad of oropahryngeal sepsis , internal jugular vein ( ijv ) thrombophlebitis and metastatic abscess formation ( 1 ) . clinical findings can therefore vary considerably from neck pain and swelling associated with thrombophlebitis to pleuritic chest pain , haemoptysis , dyspnoea or arthralgia as a result of septic thromboemboli . we report a case of predominantly external jugular vein ( ejv ) involvement in an adolescent male with negative blood culture results . a twelve year old boy presented to the children s assessment unit with a 24 hour history of unilateral face and neck swelling on a background of seven days progressive sore throat and dysphagia . minor systemic upset was also noted including headache , vomiting and non specific abdominal pain . a provisional diagnosis of tonsillitis had been made five days earlier by the local gp and a course of erythromycin and penicillinv commenced . examination revealed tender swellings over the left temporal area , angle of the mandible and upper third of the sternocleidomastoid extending into the submandibular region . examination of the oral cavity was complicated by trismus , however no obvious tonsillar enlargement , erythema or exudate was recorded . initial blood results showed an elevated c - reactive protein of 155mg / l and white cell count of 16.6(10 * 9l ) . initial imaging of the neck comprised an ultrasound scan ; this demonstrated normal right sided vasculature but abnormal left ejv and retromandibular veins . these veins appeared dilated with increased wall vascularity and showed no recordable signal on doppler imaging , an appearance in keeping with an inflammatory thrombophlebitis . to note an mri brain with venography ( mrv ) sequences provided greater anatomical delineation ( figure 1 ) . despite a coincidental finding of a smaller calibre left venous sinus ; thrombus was not seen to extend intracranially . lastly an echocardiogram was sought confirming a normal heart with no septal defects , vegetations or thrombus . mrv brain images demonstrate bilateral sagittal , straight , transverse and sigmoid sinuses with no evidence of thrombus extension . both right and left ijv as well as right ejv are clearly patent . the left ejv could not be demonstrated . treatment was started promptly on clinical suspicion of ls and consisted of intravenous ( iv ) metronidazole , benzyl penicillin and dexamethasone ; anticoagulation was not given . the patient remained in hospital on iv antibiotics for a total of 14 days , the noted swellings slowly decreased in size , blood results normalized and the trismus resolved . he remained afebrile throughout , developed no subsequent complications and was discharged home to complete seven weeks of oral antibiotics . despite ijv thrombophlebitis forming a main diagnostic criterion for ls , we report a case in which the primary vascular involvement is that of the ejv , with a corresponding normal ijv . studies have previously described ls with thrombosis of the carotid artery , intracranial venous sinus and abdominopelvic vessels ( 1 ) ; only three have ever described involvement of the ejv . this unusual finding helps to highlight the variable nature of this condition and the diagnostic confusion it may herald . to note there are reported cases of ls in which there was no demonstrable vascular involvement . numerous theories have tried to explain the spread of organisms from oropharynx to the surrounding vein ; haematogenous via the tonsillar vein , secondary to lymphangitis or via direct spread through deep neck spaces . ( 3 ) the incidence of ls within developed countries is now thought to lie around one per million per year , significantly reduced from the pre antibiotic era , however a resurgence over the last decade has recently been proposed . mortality has been recorded at 6.9% however morbidity and complications remain high as the early diagnosis can often be missed and treatment delayed . ( 3 ) although classically described as following an oropharyngeal infection ls has been reported developing from other primary infective sites including the middle ear , sinuses , mastoid and teeth ( 1 ) . the development of sepsis in ls is often delayed , presenting three to seven days from signs of the primary infection , which may have all but resolved ( 4 ) , making accurate history taking and a high index of clinical suspicion ever more important . ls is classically but not exclusively associated with an obligate oropharyngeal gram negative bacillus ; fusobacterium necrophorum . other causative pathogens described in the literature include streptococcus , staphylococci and bacteroides species ( 5 ) . it has however been reported that in almost 13% of cases no pathogen is isolated on culture ( 6 ) . ls can also be associated with thrombophillia ( thought to be endotoxin induced ) ( 5 ) . the role of anticoagulation however remains controversial with no conclusive literature evidence ; the decision should therefore be made on clinical grounds ; assessing severity of symptoms , poor treatment response and degree of thrombus extension . a recent history of a sore throat in conjunction with sepsis and or a suggestive and often declining clinical picture should always prompt early antibiotic therapy whilst a diagnosis of ls is sought ; hopefully preventing the development of late embolic complications . differential diagnoses to consider include infectious mononucleosis , parapharyngeal abscess , lymphoma , pneumonia ( including atypical forms ) and other embolic sources ( e.g. infective endocarditis ) .
oropharyngeal infections are routinely encountered within general practice and accident and emergency departments . most settle with simple analgesia and antibiotics ; occasionally such patients may develop lemierre s syndrome ( ls ) a rare and potentially fatal sequela that can be easily overlooked . we aim to highlight the main symptoms , pathology , investigations and management .
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with an incidence rate of 0.92% , maternal puerperal lower extremity nerve injuries are rare . lateral femoral cutaneous neuropathy ( meralgia paraesthetica ) is the most common , followed by femoral neuropathy . nerve injuries are more likely to occur in nulliparae , in cases of prolonged stages of labour and assisted vaginal deliveries . the presented case involved a 32-year - old woman with bilateral heel numbness due to bilateral neuropathy of the medial calcaneal nerve due to external compression during vaginal delivery . a 32-year - old woman presented to our neurology outpatient clinic with tingling and numbness in both heels . she continuously complained about these sensations ever since she had given vaginal birth to her first child 3 months earlier in a hospital . spinal analgesia ( lumbar level , bupivacaine / sufentanyl bolus , followed by a continuous administration of 8 ml / h ) was used during labour . the first stage of labour was prolonged , and because of fetal compromise during the expulsion phase , a vacuum delivery system ( palmpump kiwi ) , together with episiotomy , was used for fetal extraction . after delivery , the epidural analgesia was continued during the closure of the episiotomy with sutures . the patient did not use any other medication and had no history of neurological disorders or risk factors for neuropathy ( diabetes , hypothyroidism or hereditary liability to nerve pressure palsy ) . on examination , she had hypoaesthesia in the area of both heels . the strength of both the leg and foot muscles was normal , and so were the deep tendon reflexes . , a near - nerve needle conduction study of the medial calcaneal nerve was not performed . a diagnosis of bilateral neuropathy of the medial calcaneal nerve , most likely due to longstanding pressure on both heels with epidural analgesia as a predisposing factor , was made . three months after presentation ( 6 months after delivery ) , her numbness had diminished , but had not completely disappeared . bilateral neuropathy of the medial calcaneal nerve due to external compression during delivery using epidural analgesia is very rare . only 1 patient with numb heels has been reported in a large study , describing 2,615 women who received epidural anaesthesia during delivery . however , no details about delivery or analgesia in that patient were mentioned . in epidural analgesia during delivery , a motor block is unwanted , and thus voluntary muscle movements are possible , which makes longstanding external compression of nerves uncommon . in our patient , prolonged labour with spinal analgesia and a continuation of analgesia during episiotomy probably masked signs of external compression on both heels . the medial calcaneal nerve arises from the tibial nerve at the medial side of the ankle , perforates the laciniate ligament , travels downwards , passing the bony projection below on the inner side of the ankle , and supplies the skin over the medial aspect of the heel . patients with risk factors , such as diabetes , are at a greater risk of developing neuropathy during epidural anaesthesia . however , also without any risk factors , the clinician should be aware of the absence of the normal reaction to longstanding pressure on a nerve during epidural analgesia . preventative measures like soft gel pads or intermittent posture changes could possibly prevent neuropathy of the medial calcaneal nerve in long - lasting epidural analgesia . bilateral medial calcaneal nerve neuropathy as a result of external compression is a rare complication of epidural obstetric analgesia .
we describe the case of a 32-year - old woman who developed bilateral heel numbness after obstetric epidural analgesia . we diagnosed her with bilateral neuropathy of the medial calcaneal nerve , most likely due to longstanding pressure on both heels . risk factors for the development of this neuropathy were prolonged labour with spinal analgesia and a continuation of analgesia during episiotomy . padded footrests decrease pressure and can possibly prevent this neuropathy .
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symptomatic supravesical obstructive uropathy in a patient with dual functioning kidneys is classically characterized by bilateral hydroureters / hydronephrosis and an empty urinary bladder . this obstruction may be secondary to metastatic abdomino - pelvic and retroperitoneal malignancies , ureteric calculi and retroperitoneal fibrosis [ 25 ] . the evidence for obstruction may be partially or totally absent on ultrasound or computerized tomography , in one or both kidneys [ 68 ] . our recent experience with a uraemic 56-year - old caucasian showing only unilateral moderate right - sided hydronephrosis is presented . a 56-year - old caucasian male patient with a past medical history that included hypertension and coronary artery disease and a recent serum creatinine of 88.4 mol / l , was diagnosed 10 days earlier with urinary tract infection ( uti ) . two days before presentation to us , he developed worsening anorexia , nausea with vomiting and oliguria . his father had prostate cancer and died at age 75 years from colon cancer ; a sister had breast cancer and a maternal uncle had throat cancer . physical examination revealed a blood pressure of 165/89 mmhg , pulse rate of 56/min and respiratory rate of 16/min . he was not dehydrated nor orthostatic . except for trace ankle oedema and bilateral costo - vertebral angle punch tenderness , mol / l , co2 14 mmol / l and potassium 5.5 mmol / l . urinalysis showed 510 wbc per high - power field ( phf ) and 25 rbc phf . alanine aminotransferase ( alt ) , aspartate aminotransferase ( ast ) and total creatine kinase ( ck ) levels were normal . a post - void urinary bladder scan had demonstrated only 60 ml of residual urine . a renal ultrasound showed unilateral moderate right - sided hydronephrosis with suspected mass effect on the inferior urinary bladder . the left kidney appeared normal , measuring 16.2 cm 8.1 cm , with preserved cortical thickness . the next day , with increasing vomiting , serum creatinine 894.6 mol / l , phosphorus 2.6 mmol / l , potassium 6.2 mmol / l and oliguria ( table 1 ) , he started daily haemodialysis . a non - contrast computerized tomography examination , on day 2 , again showed unilateral right - sided hydronephrosis , bilateral nephric stranding and urinary bladder wall thickening suspicious for transitional cell cancer ( figure 1 ) . cystoscopy , on day 3 , revealed a sessile urinary bladder tumour , which was resected . the right ureter was successfully cannulated and a right ureteric stent was placed with prompt urine drainage . the next day , with a strong push from nephrology , the patient consented to a percutaneous left nephrostomy procedure despite the normal appearing left kidney . a percutaneous left nephrostogram revealed a previously unrecognized mild hydronephrosis / hydroureter with obstruction at the ureterovesical junction . the pathology report revealed high - grade urothelial carcinoma , grade 3 of 3 , with invasion of the muscularis propria . a patient with dual functioning kidneys presenting with uraemic symptoms and suspected to have obstructive uropathy must be presumed to necessarily have bilateral renal obstruction [ 610 ] . this is without prejudice to the findings on conventional renal imaging with ultrasound or computerized tomography [ 3,610 ] . there are false negative tests with these imaging modalities , the so - called syndrome of non - dilated obstructive uropathy or non - dilated obstructive nephropathy [ 610 ] . clinical conditions associated with the absence of hydronephrosis on ultrasound and computerized tomography despite obstructed kidney(s ) include acute early obstruction , the presence of retroperitoneal fibrosis or infiltrative metastatic abdomino - pelvic cancers , dehydration or septic shock and severe oliguria [ 210 ] . our patient was not dehydrated and was not hypotensive but was severely oliguric ( table 1 ) . we note that we did not rule out the presence of retroperitoneal fibrosis in our patient . the classic picture of bilateral hydronephrosis with hydroureters and an empty urinary bladder , in symptomatic uraemia following supravesical obstruction , in patients with dual functioning kidneys , is well acknowledged . however , the presentation of new - onset symptomatic uraemia concurrent with only unilateral hydronephrosis / hydroureter on conventional imaging ( ultrasound , computerized tomography ) should raise the plausibility of non - apparent obstruction of the contra lateral kidney . in such instances , the more sensitive albeit invasive percutaneous nephrostogram of the apparently normal appearing kidney is therapeutic and will lead to greater renal salvage [ 68 ] . we would like to remind practicing providers that symptomatic uraemia presenting in the setting of suspected obstructive uropathy must be assumed to imply bilateral renal obstruction , regardless of the results / interpretations of any form of conventional renal imaging . therefore , necessarily , every attempt to decompress both kidneys must be the rule . this approach would result in early and improved renal salvage . left undiagnosed and therefore untreated , this potentially reversible cause of renal failure can lead to irreversible renal failure if bilateral , , or to significant residual loss of renal function if missed on one side only . we note that we were not able to carry out any split renal functional testing after recovery as the patient 's primary attention at this point was to find out treatment options for his cancer .
bilateral hydronephrosis is classic for supravesical obstructive uropathy causing uraemia with dual functioning kidneys . recently , a patient presented with uraemia and metastatic urinary bladder carcinoma but only unilateral right - sided hydronephrosis . a right ureteral stent was placed retrograde and no further intervention was planned since the left kidney appeared normal , and since the left ureteric orifice was not visualized . we insisted on a left percutaneous nephrostomy which was successful with prompt urine return . a left nephrostogram revealed unrecognized hydroureter / hydronephrosis . following haemodialysis , kidney function normalized at 3 weeks . for symptomatic uraemia from obstruction , an antegrade and/or a retrograde decompression must be attempted bilaterally to improve renal salvage .
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musculoskeletal stiffness and limited range of motion ( rom ) can restrict functional joint movement , particularly during ankle supination following neurological diseases1 . ankle dorsiflexion rom restrictions impair dynamic balance and gait , which may contribute to secondary injuries ; reduced ankle rom and tight calf muscles can cause poor gait , inefficient energy use , impaired balance , and an increased risk of falls2 . musculoskeletal transformations manifesting as abnormal joint stiffness and limited rom result in numerous restrictions in the function and joint movements of patients3 . limited passive ankle dorsiflexion rom during knee extension can alter foot positioning and result in compensatory foot movements , leading to an abnormal gait , which can result in ankle sprains and lower extremity overuse injuries4 . studies have reported that balance - training programs that alter the somatosensory input , such as the use of an unstable surface to induce equalized weight distribution , can be helpful in hemiplegic patients5 . kim et al . used an ankle board with a slope in their center to achieve efficient ankle stretching and increased dorsiflexion6 . they suggested that stretching exercises using their device resulted in a greater increase in foot pressure compared to that with stretching exercises using existing inclined boards during ankle dorsiflexion stretching6 . therefore , we investigated the effects of an unstable inclined board on the active and passive ankle rom in patients with ankle stiffness . the study included 10 female patients ( mean age , 22.8 5.3 years ; height , 158.9 3.0 cm ; weight , 61.2 4.7 kg ) with ankle stiffness caused by diseases or medical conditions . the patients were selected based on ankle stiffness scores obtained using a symptom checker tool . the patients were assigned randomly to two groups ( both n = 5 ) and their height and weight were measured . the patients were not cognitively impaired and were capable of independent walking for 20 m without difficulty . patients were excluded if they had a history of or current neurological conditions . before participating , the purpose and methods of the study were explained to the patients , and all provided informed consent , according to the principles of the declaration of helsinki . active and passive ankle dorsiflexion were measured using a standard 31.75 4.45 cm clear plastic goniometer , with one increment , a 360 scale , and a bubble level at each end . group 1 performed the ankle dorsiflexion stretching exercise using a wooden inclined board , and group 2 performed ankle dorsiflexion stretching exercises using an air - cushioned inclined board that provided an unstable surface . rubber sheets were attached to the inclined boards to prevent falls due to sliding . the patients performed ankle dorsiflexion stretching exercises for 5 min , with a 10-min rest between each set , five times a day for 1 week . the active and passive ankle dorsiflexion angles were measured bilaterally using the goniometer before and after the study . independent and paired t - tests were used to assess within- and between - group differences in ankle dorsiflexion , with spss for windows ( spss , chicago , il , usa ) . the amount of active and passive dorsiflexion did not differ between the two groups before the exercises , with active dorsiflexion angles of 18.2 6.4 and 19.3 4.2 and passive dorsiflexion angles of 23.4 5.5 and 22.6 5.7 in groups 1 and 2 , respectively ( both p > 0.05 ) . the stretching exercises significantly ( p < 0.05 ) increased the active and passive ankle dorsiflexion angles in both groups compared to those at baseline . the active dorsiflexion angle was significantly increased ( p < 0.05 ) in group 2 compared to that in group 1 after the exercises . the active dorsiflexion angles before and after the exercises were 18.2 6.4 and 22.1 5.0 in group 1 and 19.3 4.2 and 28.1 6.9 in group 2 , respectively . the passive dorsiflexion angles did not differ significantly between the groups after the exercises ( p>0.05 ) and were 23.4 5.5 and 32.2 5.3 in group 1 and 22.6 5.7 and 35.1 6.0 in group 2 before and after exercising , respectively . the air - cushioned inclined board provided an unstable surface for performing ankle dorsiflexion stretching exercises . the results showed that ankle stretching exercises using the unstable inclined board significantly increased the active dorsiflexion angle compared to that with exercises using a wooden inclined board . we believe that the use of the air - cushioned inclined board provides somatosensory input and subsequently stimulates active ankle dorsiflexion by promoting automatic postural control . sensory proprioception of the ankle and sensory compression of the plantar are important in the control of sway7 . an increase in the number of somatosensory inputs affects the muscles , and the use of an unstable support surface result in many different somatosensory inputs that limit compensatory action7 . performing cognitive tasks in a standing posture reduces internal focus and induces external focus , which promotes automatic postural control8 . stretching improve the flexibility of tendons , and changes in the mechanical condition of tendons depend on the type of stretching performed9 . , the passive dorsiflexion angles did not differ significantly between the two groups after ankle stretching exercises . however , stretching exercises significantly increased the active and passive ankle dorsiflexion angles in both groups compared to those at baseline . these results suggest that passive ankle exercises using a wooden inclined board only stretch muscle and tissue , while exercises using an unstable inclined board stretch muscle and tissue , and stimulate activation of the ankle dorsiflexor . active ankle dorsiflexion was more effectively improved with ankle stretching exercises using an unstable inclined board than with exercises using a wooden inclined board .
[ purpose ] the present study assessed the effects of using an unstable inclined board on the active and passive ankle range of motion in patients with ankle stiffness . [ subjects ] the study included 10 young female patients with ankle stiffness . [ methods ] the patients were divided into the following two groups : a group that performed ankle dorsiflexion stretching exercises using a wooden inclined board and a group that performed stretching exercises using an air - cushioned inclined board ( unstable inclined board ) . active and passive ankle dorsiflexion angles were measured bilaterally using a goniometer . [ results ] both inclined boards significantly increased active and passive ankle dorsiflexion . after performing ankle stretching exercises , active dorsiflexion significantly increased the unstable inclined board compared to that using the wooden inclined board . however , the passive dorsiflexion angles did not differ significantly between the two groups after ankle stretching exercises . [ conclusion ] the use of an unstable inclined board might stimulate activation of the ankle dorsiflexors in addition to stretching muscle or tissue . active ankle dorsiflexion was more effectively improved with stretching exercises using an unstable inclined board than with exercises using a wooden inclined board .
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renal tubular involvement in sjgren 's syndrome ( ss ) usually manifests with fanconi syndrome , distal ( type 1 ) renal tubular acidosis ( rta ) , nephrogenic diabetes insipidus , and hypokalemia . , we describe a case a young female with ss who presented with features of gitelman syndrome and hypokalemic paralysis . a 29-year - old female presented with weakness of both upper and lower limb weakness since 15 days . she had a history of dry eyes and dry mouth for the past 6 months . on examination , laboratory evaluation showed hemoglobin - 9.5 g / dl , platelet 2.95 lakh / mm . blood urea nitrogen was 7 mg / dl , serum creatinine was 0.65 mg / dl , serum sodium was 134 meq / l , serum potassium was 1.81 meq / l and serum chloride was 84.3 urine chloride ( spot ) was 36.7 meq / l , urine creatinine ( spot ) 23.5 mg / dl , and urine calcium ( spot ) 1.23 mg / dl . this patient satisfied the american european consensus criteria for diagnosis of primary sjgren 's syndrome ( ss ) . ten days after admission , she was discharged on spironolactone and prednisolone . on follow - up this patient presented with muscle weakness and her laboratory reports showed hypokalemia and metabolic alkalosis with normal blood pressure . her urinary chloride levels were above 20 meq / l , which ruled out extra - renal causes of metabolic alkalosis . the presence of hypomagnesemia and absence of hypercalciuria ( urinary calcium creatinine ratio < 0.2 ) ruled out bartter syndrome . hypokalemic alkalosis , high urinary chloride , hypomagnesemia , absence of hypercalciuria in background of no history of diuretic use favored the diagnosis of gitelman syndrome . tubular involvement in ss is usually distal tubular dysfunction , type i ( distal ) renal tubular acidosis and nephrogenic diabetes insipidus . proximal tubular abnormalities are less frequent , and rarely fanconi 's syndrome has been reported in patients with ss . acquired gitelman syndrome is relatively rare . to the best of our knowledge , only five cases of acquired gitelman syndrome have been reported in english literature so far . among these five cases , four had ss , one was a case of chronic sialoadenitis . acquired gitelman syndrome secondary to ss presenting with hypokalemic weakness is very rare . only two cases have been reported so far . though gitelman syndrome is an inherited disorder , given the paucity of reports , we believe that ss presenting as acquired gitelman syndrome may be relatively rare . acquired gitelman syndrome should be considered in differential diagnosis of renal involvement in patients with ss .
we report a case of a young female patient who presented with weakness of upper and lower limbs . on evaluation , she had hypokalemia , hypomagnesemia , metabolic alkalosis and hypocalciuria . anti - ro ( ssa ) antibody was positive . she had an acquired gitelman syndrome due to primary sjgren 's syndrome ( ss ) . ss presenting with features of gitelman syndrome is very rare .
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however , proper amount of daily water intake in a healthy individual is controversial . there is a prevalent thought that we all have to drink eight glasses of water daily , we try to clarify this topic from the perspective of avicenna and traditional persian medicine ( tpm ) . the most important traditional persian medical encyclopedia , al - qanun fil - tibb ( canon of medicine ) , was reviewed . furthermore , medline , embase , scopus , iranmedex , and science iranian database ( sid ) focusing on the keywords traditional medicine , water intake , fluid , and complementary and alternative medicine were reviewed to find relevant information . avicenna believed that the demand for daily water is not the same in different individuals . the determinant variables mentioned in his book , the canon of medicine , comprises mizaj ( temperament ) , health status , age , sex , season , place , habits , occupation , etc . he believed that water in extra amount quenches the hararat - e - ghariezi , which is the basal internal heat to convey normal homeostasis and metabolism in the body . several factors determine the actual need of any person to drink water . consequently , recommending a specific amount of daily water intake for all is illogical . moreover , important recommendations of tpm sages on an appropriate amount of water intake should be considered to prevent associated disorders .
background : fluid intake is an essential factor in having a normal homeostasis . however , proper amount of daily water intake in a healthy individual is controversial . there is a prevalent thought that we all have to drink eight glasses of water daily , however , it lacks enough supportive evidence at least for healthy individuals . here , we try to clarify this topic from the perspective of avicenna and traditional persian medicine ( tpm).methods : the most important traditional persian medical encyclopedia , al - qanun fil - tibb ( canon of medicine ) , was reviewed . furthermore , medline , embase , scopus , iranmedex , and science iranian database ( sid ) focusing on the keywords traditional medicine , water intake , fluid , and complementary and alternative medicine were reviewed to find relevant information.results:avicenna believed that the demand for daily water is not the same in different individuals . the determinant variables mentioned in his book , the canon of medicine , comprises mizaj ( temperament ) , health status , age , sex , season , place , habits , occupation , etc . he believed that water in extra amount quenches the hararat - e - ghariezi , which is the basal internal heat to convey normal homeostasis and metabolism in the body.conclusion:several factors determine the actual need of any person to drink water . consequently , recommending a specific amount of daily water intake for all is illogical . moreover , important recommendations of tpm sages on an appropriate amount of water intake should be considered to prevent associated disorders .
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we report 2 patients infected with panton - valentine leukocidin ( pvl)positive mrsa t034 . each patient had a medical history typical of that reported for community - acquired mrsa of other lineages , which in most cases are pvl positive ( 8) . the first patient , a previously healthy 36-year - old male physiotherapist , sought medical care in march 2006 for a small abscess in his axilla . culture of the abscess grew mrsa . presence of meca gene was confirmed by pcr ( 9 ) . during the next 2 months , his youngest child , adopted from china , had been found to be mrsa positive ( throat , perineum , and a small wound ) a month earlier during routine screening for adopted children . during subsequent screening of the family , the older sister , adopted from south korea , both parents were negative for mrsa at that time , which suggests that the father was newly infected when his abscess developed and that he had not acquired the strain abroad . also , spa typing indicated that the children carried different strains from that of the father and from each other ( t286 , t1434 ) ( 10 ) . subsequent screening of family members for mrsa on several occasions found only the father to be repeatedly positive . the second patient , a 43-year - old male clerk , also previously healthy , sought medical attention during the summer of 2007 for a mrsa - infected elbow wound . follow - up examination determined that he carried mrsa also in the perineum and in a chronic external otitis eczema . the patients lived in geographically distinct areas in the western part of sweden and had no connection to each other . no animal contact ( e.g. , pets , farming ) was reported by the 2 patients , their family members , or other close contacts . both patient strains carried pvl , confirmed by identification of the luks - lukf genes ( 11 ) , and were resistant to digestion with restriction endonuclease smai when typing by pfge was attempted . they produced -hemolysin according to phenotypic detection methods that used rabbit blood agar with hot cold analysis , which further indicated their animal origin ( 12 ) . their drug - susceptibility profiles differed ; 1 was resistant to doxycycline and the other was resistant to ciprofloxacin , erythromycin , and clindamycin . these strains carry pvl , a toxin partly responsible for the increased virulence of several of the mrsa clones in the community ( 8) . despite several recent publications concerning st398 mrsa , few have reported pvl in this lineage , which is believed to be of animal origin ( 2,4,6,13 ) . most previous reports have described asymptomatic carriage in persons exposed to occupational hazards ( e.g. , veterinary personal and pig farmers ) ( 1,2,7 ) . however , severe clinical infections have been described ( 46 ) . in our patients these strains caused repeated infections that needed medical attention , even hospitalization . since neither patient had even a remote connection to animals and we found no common source of infection , these strains may already be more common in our region than we had thought . these case reports suggest that strains of this lineage may impose a threat in the community , even to patients with no obvious animal contact .
panton - valentine leukocidin ( pvl)positive methicillin - resistant staphylococcus aureus ( mrsa ) , sequence type 398 is believed to be of animal origin . we report 2 cases of infection due to pvl positive mrsa , spa type t034 , in patients in sweden who had had no animal contact .
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the selective melatonin receptor agonism effect of ramelteon is useful for insomnia.1 ) here we wanted to present a refractory chronic migraine case , who had significant improvements in migraine after using ramelteon . the precipitating factors were the stress , increase in physical activities and sudden cold weather . the moderate or severe pain intensity was aggravated by or causing avoidance of increased physical activity . the residual symptom between the migraine episodes were the head tense feeling , but not achieveing headache severity . the computed tomography revealed no significant findings of other causes for headache , such as tumor or hemorrhage . she ever received many kinds of medications , such as aspirin , nonsteroidal anti - inflammatory drugs , triptans , ergots , anticonvulsants and glucocorticoids . however , the severity of migraine still remained moderate ( migraine disability assessment test [ midas ] scores , 19 ) and the associated symptoms were moderate headache , dizziness , nausea , and sensitivity to sound . the migraine also exacerbated her insomnia problems , with fragmented sleep and inadequate sleep duration as 12 hours ( insomnia severity index [ isi ] score , 23 ) . she did nt have any comorbid mental disorder except insomnia . due to the fear of abuse potential related to hypnotics after 2 weeks treatment of ramelteon 8 mg / day , her sleep duration prolonged to 45 hours with less fragmentation . in addition , her migraine severity started to decline ( midas scores , 11 ) . after 6 months of ramelteon 8 mg / day treatment , the migraine severity continued to relieve ( midas scores , 6 ) with stable sleeping quality ( isi score , 14 ) . in this case , we found significant improvements in migraine severity and insomnia after the use of ramelteon . since the patient did nt have insomnia before the first onset of migraine . therefore we could speculate that the ramelteon had significant effects in migraine in such mild prolongation of sleep duration . the melatonin might relieve the headache via the following possible mechanisms , such as anti - inflammatory effect , free radical scavenging , reduction of pro - inflammatory cytokine , membrane stabilization , nitric oxide synthase activity and dopamine release inhibition , gaba and opioid analgesia potentitation , glutamate neurotoxicity protection , neurovascular regulation,2 ) cytoprotection and antiallodynic action.3 ) the pineal gland , the primary source of serotonin and melatonin , might also play a significant role in the analgesic effect.4 ) however , the clinical trial of melatonin administration showed no significant improvements in migraine severity,5 ) which suggested the modulation of melatonin receptor might be a better option for the analgesic effects . the modulation of serotonin system by the ramelteon would also relieve the pain sensation.2 ) in addition , the chemical structure of ramelteon was similar with that of indomethacin , which is a kind of non - steroidal anti - inflammatory medicine.2 ) in the study of animal model , the antinociceptive and antiallodynic actions of melatonin have been observed in different kinds of pathways , such as intrathecally or intracerebroventricular routes . the use of ramelteon , a kind of mt1 and mt2 agonist , could also relieve the pain even lack of free radical scavenger effects.3 ) the state - of - art treatment of refractory chronic migraine also includes the ramelteon due to selective m1 receptor agonism.6 ) from the successful experience in this case , we can consider the use of ramelteon for refractory chronic migraine with insomnia .
the selective melatonin receptor agonism effect of ramelteon is useful for insomnia . here we wanted to present a refractory chronic migraine case , who had significant improvements in migraine after using ramelteon . the possible mechanism for the ramelteon in the migraine relief might be related to melatonin effects .
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the patient was a 41-week - gestational - age infant girl with a birth weight of 3300 g. after an uneventful pregnancy , the patient was delivered spontaneously and healthy until this episode . at 35 days of age , the patient was admitted to our hospital with a one - day history of anorexia , vomiting and recurrent diarrhea . on admission , conscious level was glasgow coma scale e2 v2 m2 , and blood pressure was 104/flat . physical examination revealed a distended and tender abdomen . laboratory values were significant for a c- reactive protein ( crp ) of 1.7 mg / dl ( normal range was less than 0.3 mg / dl ) . the patient was suspected intestinal obstruction due to enteritis , antibiotics were started ( abpc / mcipc and amk ) . 4 hours after admission , she was pale with a temperature of 39.2 c and 220 per minutes pulse . blood pressure was low , and the fevers , vomiting , and diarrhea persisted and worsened despite antibiotics . she failed to respond to medical managements and died 27 hours after admission due to multiple organ failures . the cyst was unilocular , contained watery fluid , did not communicate with the adjacent intestinal lumen ( fig . microscopic finding of the ileocecal duplication cyst had an undefined mucosal lining though it resembled pyrolic glands or duodenal glands . duplications of the alimentary tract can be located anywhere between the mouth and the anus.4 they are most commonly found in the ileum ( 30% ) , ileocecal valve ( 30% ) , duodenum ( 10% ) , stomach ( 8% ) , jejunum ( 8% ) , colon ( 7% ) and rectum ( 5% ) with the half of the all lesions may contain ectopic gastric mucosa.5,6 most alimentary tract duplications are cystic ( 80%90% ) , with the remainder being tubular.7 they have three common features , hollow structure that has a wall of smooth muscle often shared with the contiguous intestinal tract , lined by mucous membrane similar to some part of the alimentary canal , and usually attached to some part of the alimentary tube.8 the clinical symptoms are highly variable from minor digestive problems to intestinal obstruction , gastrointestinal bleeding , or perforation.9 most patients present before the age of 2 years with obstructive symptoms , pain , or an abdominal mass . the presence of gastric or pancreatic mucosa can lead to peptic ulceration , perforation , and hemorrhage . early diagnosis and treatment reduces the risk of dangerous complications , such as bowel perforation , bleeding , obstruction , and malignant alteration . resection of duplication alone is the treatment of choice , and in uncomplicated cases laparoscopic resection is advised . surgically treated intestinal duplications have good prognosis , however , such duplications can have a fatal consequence if not properly treated . possible complications like bowel perforation , bleeding , obstruction , and malignant alterations are the reason why all duplications should be surgically treated at the time of diagnosis.10 alimentary tract duplications can be easily mis - diagnosed as other disorders.5 pyloric and duodenal duplications can mimic hypertrophic pyloric stenosis or choledochal cyst.11,12 ovarian cyst should be considered in female patients , and adolescent patients can sometimes have a presumptive diagnosis of crohn s disease.5 yousefzadeh et al13 showed that duplication of the colon and rectum clinically presented as abdominal pain , vomiting and chronic constipation , a significant number of patients were thought to have hirschsprung s disease . in our fatal case , there are two reasons for this diagnostic difficulty : first , fevers , vomiting , and recurrent diarrhea persisted and worsened despite antibiotics . the patient was suspected intestinal obstruction due to enteritis , however , actually the ileocecal duplication cyst led to intestinal obstruction and necrotizing ileum ; second , the patient died in an instant due to multiple organ failures . if this patient was considered for exploratory laparotomy , she might be able to be alive . however , the patient was getting worse very quickly due to multiple organ failures and showed intractable psvt . exploratory laparotomy was impossible . it is demonstrated a high association of other anomalies in these patients including skeletal and urologic abnormalities,13,14 however , in this case , there were no other congenital anomalies . sudden unexpected death of infants associated with duplication cysts is extremely rare with only 4 cases reported in medical literature to date ( table 1 ) . puligandla ps et al5 and byard rw15 reported the patients presented with group b streptococcal sepsis and clostridium perfringens sepsis , respectively . kibayashi et al16 reported the patient died at home after being diagnosed as having gastroenteritis . in present case , though blood , stool and cyst cultures were negative , the patient had high fever and crp level elevated . infection is suspected to be a risk factor for sudden and unexpected death in infants with duplication cysts . in all cases , this potential diagnosis should be borne in mind for a patient who complains of abdominal symptoms with an unknown cause , and duplication cyst should be recognized as a fatal cause in infant .
duplications of the alimentary tract are very rare . a one - month - old female presented with symptoms of anorexia , vomiting and continuous watery diarrhea . the plain abdominal radiograph showed thickened intestinal wall and signs of small bowel obstruction . the fevers , vomiting , and continuous wartery diarrhea persisted despite antibiotics , and worsened . the patient failed to respond to medical managements , 27 hours after admission , the patient died due to multiple organ failures . the autopsy was performed , small bowel obstruction due to an ileocecal duplication cyst ( 3 3 cm ) was recognized . the ileocecal duplication cyst was attached to the ileum which was changed edematous and necrotic . this potential diagnosis should be borne in mind for a patient who complains of abdominal symptoms with an unknown cause , and duplication cyst should be recognized as a fatal cause in infant .
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enteric duplications ( eds ) are uncommon anomalies that can occur at any point of the gastrointestinal tract . the small intestine is the most common location ; retroperitoneum is an extremely rare site . in general , diagnosed in the neonatal period or during infancy , they are increasingly diagnosed prenatally ; early prenatal detection is possible . , there have been seven reported cases of retroperitoneal ed cyst in the english literature . a female newborn , vaginally born at 39 weeks of gestation from a 32-year - old mother , gravid 3 , para 3 . prenatal ultrasound at 22 weeks of gestation objectified an abdominal cystic mass located in the left upper abdominal quadrant , associated with fetal pyelectasis . birth weight was 4000 g , length was 51 cm , and head circumference was 35 cm . postnatal ultrasound found a retroperitoneal para - aortic liquid - filled mass measuring 60 mm 33 mm 22 mm . magnetic resonance imaging ( mri ) confirmed the presence of a retroperitoneal cyst occupying the upper left retroperitoneal space ; with mass effect displacing the left kidney down [ figure 1 ] . peroperative finding was a retroperitoneal cyst above the left adrenal , displacing the left kidney down , measuring 70 mm 30 mm ; with no communication with any portion of the alimentary tract . histopathologic examination revealed an ed cyst lined by small intestinal epithelium , with no ectopic gastric mucosa . the postoperative period was uneventful ; the patient was discharged on the 5 postoperative day . magnetic resonance imaging showing retroperitoneal cyst occupying the upper left retroperitoneal space ; with mass effect displacing the left kidney intraabdominal duplications account for two - thirds of localization , among them , jejunoileal duplications account for 65% . eds occur early in intrauterine life ; their pathogenesis remains unclear ; several hypotheses have been proposed . reported 38 cases of ed cysts ; only one was retroperitoneal communicating with a nonfunctioning right kidney . eds are a part of fetal intraabdominal cysts which evoke several differential diagnoses including ovarian cysts , renal cysts , choledochal cysts , hepatic cysts , and mesenteric cysts . marchitelli et al . found high concordance between prenatal and postnatal findings in fetal intra - abdominal cystic lesions ( 90.4% ) . double - wall sign and peristaltism are suggestive of ed and help to differentiate it of differential diagnosis . postnatal ultrasound helps diagnosis showing an anechoic fluid - filled mass , rarely an echogenic mass is found due to complication ( hemorrhage , necrosis ) . more serious complications may occur : ulceration , perforation , severe hemorrhage , and malignant changes associated with the presence of ectopic gastric mucosa .
retroperitoneum is a very uncommon site of enteric duplication ( ed ) . we report a new case of retroperitoneal ed cyst suspected in utero . prenatal ultrasound showed an abdominal cystic mass . noncommunicating retroperitoneal ed cyst measuring 70 mm 30 mm was resected . histopathologic examination confirmed the diagnosis .
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, neither buccolingual width nor angulation can be properly visualized on the most traditional radiographs . in the following case report , an innovative simplified method without radiation is used to fabricate a three - dimensional model to assess the available bone for implant placement in the mandible . even a minor variation in comparison to ideal placement causes difficulties in fabrication of final prostheses . the surgical guide is essential to establish a logical continuity among diagnosis , prosthetic planning , and surgical phase . a patient of age 27 years reported to the department of prosthodontics for the replacement of missing left first molar . following steps were performed to achieve a three - dimensional model : intraoral impression was recorded with addition silicone putty and light body [ figure 1]fabrication of acrylic assembly for aligning both intraoral and extraoral impressiona rectangular block of acrylic was made to which impression tray was pressed to make indentations . after applying separating medium , the counter block of acrylic was made to which extraoral impression was attached [ figure 2 ] . in prototype , [ figures 24 ] plastic rod was used for supporting the extraoral impressionreplacing the plastic rod with metal plate and key system the plastic rod was replaced by a metal plate in the first generation [ figure 5 ] for better stability and better customization . a key system was incorporated to adjust the length and angulation according to each patient 's mandible . on the top of the metal plate , addition silicone putty placed on the acrylic plate can be customized each time to record the extraoral impression . the extraoral impression should record the lower border of the mandible to the medial extent as much as possible [ figure 6]fabrication of three - dimensional model the intraoral impression was poured first to get a cast . after the initial setting was over , the entire assembly was poured , and a three dimensional model of the mandible was fabricated [ figure 7 ] . intraoral impression was recorded with addition silicone putty and light body [ figure 1 ] fabrication of acrylic assembly for aligning both intraoral and extraoral impression a rectangular block of acrylic was made to which impression tray was pressed to make indentations . after applying separating medium , the counter block of acrylic was made to which extraoral impression was attached [ figure 2 ] . in prototype , [ figures 24 ] plastic rod was used for supporting the extraoral impression replacing the plastic rod with metal plate and key system the plastic rod was replaced by a metal plate in the first generation [ figure 5 ] for better stability and better customization . a key system was incorporated to adjust the length and angulation according to each patient 's mandible . on the top of the metal plate , acrylic addition silicone putty placed on the acrylic plate can be customized each time to record the extraoral impression . the extraoral impression should record the lower border of the mandible to the medial extent as much as possible [ figure 6 ] fabrication of three - dimensional model the intraoral impression was poured first to get a cast . after the initial setting was over , the entire assembly was poured , and a three dimensional model of the mandible was fabricated [ figure 7 ] . intraoral impression with acrylic assembly prototype ( extraoral impression ) prototype - extraoral and intraoral impression together with acrylic assembly first generation metal plate assembled metal frame three - dimensional model the accuracy of this method was verified with computed tomography ( ct ) scan . the cross section where the mandibular premolar teeth were disappearing from the ct image was taken ( 14 mm depth ) as a reference and measured to get the available bone width buccolingually ( 8.7 mm ) . this measurement was same when compared with the available bone in the sectioned cast at the premolar area , at a depth of 14 mm ( premolar root length ) . on the three - dimensional model , the surgical guide was fabricated [ figure 8 ] . this surgical template can dictate the implant body placement that offers the best combination of support for the repetitive forces of occlusion and esthetics . three - dimensional model with surgical guide this article had won the best table clinic award in 35 ips conference conducted in new delhi 2007 by army dental corps .
the primary factor that determines the success of implant is the amount of available bone in the edentulous site . diagnostic imaging and techniques help to develop and implement a cohesive and comprehensive treatment plan according to the available bone . in the following case report an innovative simplified method ( poor man ct ) is used to fabricate a three dimensional model to assess the available bone for implant placement in mandible .
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the clinical manifestation is typical and it is characterized by abduction of the affected arm , flexion of the elbow and pronation of the forearm . bilateral luxatio erecta represents an extremely rare condition , and just a few cases were reported in medical literature . because of a high incidence of related complications such as neurovascular damages early recognition and treatment of this type of dislocation is required by physicians . in this article we describe a case of a bilateral luxatio erecta that was successfully treated without long - term complications . a 70-year - old female was accepted in our emergency department because of a bilateral shoulder pain that onsets after a sudden fall happened in her house . the anamnestic research showed that the patient tumbled downstairs ; while clinging to both lateral banisters , she slipped down banging her face and chest to the ground . at physical examination both shoulders were abducted and elevated approximately 90100 from the horizontal plane , with flexed elbows and with hands below the head . the patient was unable to adduct and lower the arms , and there was immediate pain when attempting passive movements . no vascular deficit was found , and the patient complained of slight paresthesias on both arms , with superficial and deep sensation deficit on both arms . an x - ray examination was performed showing a bilateral inferior dislocation of humeral heads in relation with the glenoid fossa ; no fracture was associated ( fig . 1 ) . 1bilateral luxatio erecta bilateral luxatio erecta close reduction under sedation was achieved without difficulty by pushing the humeral head superiorly with an axial traction - countertraction maneuver on both shoulders . x - ray control revealed a correct anatomic reduction of dislocations and after the reduction no vascular damage were found while paresthesias were still present on the left arm ( fig . 2 ) . both arms were immobilized with bilateral slings in total adduction , intrarotation with the supinated arms . 2post - reduction x - ray the progressive mobilization of the shoulders started after 2 weeks with passive movements and pendulum codman s exercises . after 3 weeks the patient gave up complaining paresthesias on the left arm and active movements were allowed . after 8 weeks she recovered the complete function of both shoulders and after a one - year follow - up treatment the patient showed a complete range of motion of both arms . inferior dislocations are rare ( 0.5% of all shoulder dislocations ) and bilateral inferior shoulder dislocations are exceptional : only 10 cases have been described so far in the literature . according to the literature , luxatio erecta is due to an indirect mechanism such as a violent abduction force on abducted limb resulting in impingement of the proximal shaft of the humerus against acromion . direct axial loading from superior direction is another different mechanism , though less common . in our case the first mechanism has to be taken into consideration ; in fact , while the patient was sliding in a sitting position downstairs , her hands were holding onto both banisters and the arms were abduced . when she reached the ground floor the hands were blocked around the end of the banisters and her head and body were thrown forward by gravity . the clinical presentation of an inferior shoulder dislocation is typical : the extremities are held over the head or abduced in a fixed position with the elbow flexed . any passive movement is possible , and the humeral head is palpable on the chest wall . x - ray shows the humeral head located below the rim of the glenoid and the humeral shaft is parallel to the scapular spine . in order to reduce inferior glenohumeral dislocation , it is recommended a traction counter - traction maneuver under sedation followed by immobilization in a desault bandage for at least 2 weeks . there is a high incidence of associate tears of the rotator cuff ( 12% of patients ) and a slap lesion has been described by schai and hintermann after a post - reduction arthroscopic assessment view . fracture of the acromion , clavicle , inferior glenoid fossa and greater tuberosity represent a complication that should need a surgical treatment . neurovascular damages are also commonly associated with inferior shoulder dislocation [ 5 , 7 ] ; garcia et al . described a case of bilateral luxatio erecta complicated by an axillary vein thrombosis . in general , neurologic injuries ( 60% of cases ) of brachial plexus , axillar , radial and ulnar nerves are more common than vascular injuries but they tend to resolve after reduction with an excellent prognosis , suggesting the neuropraxia as the main mechanism of the injury [ 5 , 9 , 10 ] .
luxatio erecta is an uncommon form of glenohumeral dislocation . cases of bilateral inferior shoulder dislocation ( luxatio erecta ) are very rare , and only ten cases have been described in literature . we describe a case of a woman with bilateral luxatio erecta of the shoulders treated with close reduction , immobilization and rehabilitation . the patient had fallen downstairs while clinging to both lateral banisters .
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spider nevus ( also known as spider angioma or vascular spider ) is a common benign vascular anomaly that may appear as solitary or multiple lesions . spider nevi are represented by telangiectases that consist of a large arteriole from which radiate numerous small vessels that resemble spider 's legs . they are found in the distribution of the superior vena cava , that is , on the face , neck , arms , and upper trunk . spider nevi are usually < 2 cm in diameter and rarely grow to assume large size more than 2 cm . we hereby report a case of very large spider nevus in a patient with hepatitis c virus ( hcv ) related liver cirrhosis . a 48-year - old - female presented with a history of abdominal distension and jaundice of 3-month duration . physical examination revealed pallor , icterus , pedal edema , hepatospleenomegaly and tense ascites . she had multiple classical spider nevi over the chest . also , she had a large spider nevi situated on the forehead , measuring 8 cm in largest dimension with palpable dilated vessels and venous hum [ figure 1 ] . deep compression on the central area of the lesion produced complete disappearance with blanching and when released refilling of vessels occurred . laboratory tests revealed a hemoglobin level of 9.8 g / dl , a white - cell count of 4240/mm , and a platelet count of 77,000/mm . anti - hcv antibody for hcv was positive with hcv rna of 1.6 10 iu / ml . an ultrasound abdomen was suggestive of liver cirrhosis with portal hypertension ( portal vein diameter of 14 mm ) , ascites and splenomegaly . thus , a diagnosis of cirrhosis liver with portal hypertension was made ( ctp-9 , class b , and meld 11 ) . she was put on diuretics for the control of ascites but developed hepatic encephalopathy and renal dysfunction . diuretics were stopped and in view of refractory tense ascites , she was taken up for a transjugular intrahepatic portosystemic shunt . however , during the procedure she had massive intrahepatic bleed and in spite of intensive resuscitative measures she died 48 hours later . spider nevi can be seen in pregnancy , in patients with thyrotoxicosis , rheumatoid arthritis , oral contraceptive use and most commonly , liver cirrhosis . these frequently appear in alcoholic cirrhotic or when liver function deteriorates in patients of the chronic liver disease . found elevated levels of vascular endothelial growth factor and basic fibroblast growth factor in patients with liver cirrhosis , especially in those with spider nevi . in patients with nonalcoholic cirrhosis , the levels of substance p are elevated which may play an important role in the pathogenesis of spider nevi by causing vasodilatation . most remain small in size and rarely do they assume the size as seen in our patient . the present case is unique because of its very large size , rare presentation over the forehead and palpable mass like vascular lesion in a patient with nonalcoholic cirrhosis . electrodesiccation and laser treatments under local anesthesia are effective therapeutic procedures for facial spider nevi . spider nevi , however , usually do not require any treatment in cirrhotic patients due to a high risk of bleeding from these vascular lesions .
spider nevi are benign vascular lesions mostly seen in patients with decompensated liver cirrhosis . mostly , these are seen in the superior vena cava distribution and are small with pinhead size central vessel . giant spider nevus is rarely seen and hence this report .
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a 22 years old female patient was admitted to our outpatient clinic with a complaint of dyspnea for the previous three years . a right bundle branch block was detected on her electrocardiogram . in the transthorasic echocardiography ( tte ) , a 22 mm secundum type atrial septal defect ( asd ) , right heart dilatation and pulmonary arterial hypertension ( pap 45 mmhg ) were detected . transesophageal echocardiography ( tee ) showed that the defect was 25 mm , the superior rim was 16 mm and the aortic rim was 8 mm in size . the defect was closed through a right femoral route with a 30 mm asd occluder after balloon sizing with tte . in the cineangiography and tte early after the procedure , the device was observed to be in the correct position ( fig . the tte revealed that the device was not in the defect and it had embolized to the pulmonary artery . the patient was referred to the cardiovascular surgery unit and underwent surgery promptly . under general anaesthesia the pulmonary artery was incised along its vertical axis and the occluder device was removed ( fig . the right atrium was opened and the defect was then closed with a pericardial patch . secundum asd is the most common type , which accounts for 50% to 70% of all asds and located in the fossa ovalis . sinus venosus type asd is located at the upper portion of the interatrial septum where the vena cava superior opens . a shunt between atria leads to chronic right ventricular volume overload , which in turn causes lower filling rates of left ventricle . surgical or percutaneous closure in asymptomatic patients is indicated in the case of right ventricular volume overload and if the pulmonary to systemic flow ratio increases beyond 1.5 . in adult patients , the defect should be repaired as soon as possible after it is detected because patients who undergo repair after 25 years of age have a shorter life span than a control population . the most important factor for success in percutaneous closure is the correct patient selection . in patients with secundum asd , percutaneous closure can be performed safely with careful evaluation of anatomical factors like defect size and sufficiency of the rims . in a previous report , it was shown that atrial defects up to 43 mm in diameter can be closed percutaneously . a sufficient anterior rim is not necessary to bring the device into a stable position . conversely , deploying the device in patients with deficient cranial rims can be difficult and may lead to an unstable position of the device and increased risk of device embolization . the most common type of complications in percutaneous closure is device embolization , with a rate of 0.5% to 1 % . in a study with 450 patients , seven cases of device embolization into pulmonary artery in the first 12 hours one hour after intervention we detected device embolization into the pulmonary artery after performing echocardiography since the patient had dyspnea . after that , emergency surgery was performed within one hour with a median sternotomy and under cardiopulmonary bypass . , snares can be used to take out the device , but most of the time , surgical extraction of the device and asd patch closure is needed . percutaneous asd closure is a widely used and has been performed successfully throughout the world in recent years . however , it should be kept in mind that improper patient and device selection may result in deadly complications like device embolization , cardiac perforation and tamponade .
an atrial septal defect is the most common type of congenital heart disease among adults . surgical repair or percutaneous closure of the defect is the treatment options . even though percutaneous closure seems to be less risky than surgical repair , it may result in fatal complications like device embolism , cardiac perforation and tamponade . herein we report a case of the embolism of a device into the pulmonary artery after one hour of percutaneous closure in which the embolized device was surgically removed and the defect was closed with a pericardial patch .
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the work is self - sponsored by the first author who conceptualized and designed and wrote the manuscript . the second author assisted in the surgery provided some literature review and made necessary correction which lead to the final manuscript . the ethical committee of first choice specialist hospital where the authors carried out the procedure approved the work . he understood that part of his images will be published and also understood that due to the rarity of the case , that it is worthwhile publishing it for education and for health care delivery . the second author assisted during the surgery , provided immediate care , provided some literature review and made necessary correction which lead to the final manuscript .
highlightsremoval of an entrapped penile ring where possible should be done in a simple non - invasive way.milking the oedematous penis through the thick metallic ring in a slippery field complemented by levering with a blunt end paediatric bone lever , was the technique used to remove the ring.the ring was extricated without any morbidity and the patient went home on the same day .
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a number of skin disorders have been described in patients with acquired immunodeficiency syndrome ( aids ) . among them , secondary infections are common , but their incidence has decreased considerably following the introduction of combined therapies targeted to the human immunodeficiency virus ( hiv ) . some clinical presentations may be puzzling , particularly in secondary syphilis exhibiting lesions showing a marked tendency to polymorphism . a 29-year - old woman with a three - year history of aids presented with polymorphic papules on the face and abdomen . the skin lesions were asymptomatic but the patient complained of discrete malaise , stiff neck , myalgia headache , and mild fever . a biopsy specimen was taken from a papule on the abdomen . a series of 5-m thick sections were cut from the formalin - fixed paraffin - embedded biopsy . an immunohistochemical assessment was performed using a rabbit polyclonal antibody directed to treponema pallidum ( 1:200 biocare medical , walnut creek , ca , usa ) . the envision ( dakopatts , glostrup , denmark ) polymer - based revelation system and fast red ( dakopatts ) staining were used . negative immunohistochemical controls were performed by omitting or substituting the primary and the secondary antibodies in the laboratory procedure . the dermoepidermal junction contained a band - like infiltrate composed mostly of lymphocytes , histiocytes , and plasma cells . a deeper cell infiltrate of similar composition extended along the microvasculature , hair follicles , and sweat glands . the typical spiral , corkscrew , and threadlike spirochetes were highlighted by the red chromogen , and the contrast with the clear background was striking ( figure 1a , b , c ) . their presence inside the lichenoid infiltrate was associated with a dense superficial and deep perivascular cuff of spirochetes . the latter slender spirochetes were clustered in the dermal stroma ( figure 1a ) and in rims confined to the perivascular areas ( figure 1b , c ) . in addition , some t. pallidum were evident in the cytoplasm of cells , particularly endothelial cells ( figure 1c ) . dermal homing of t. pallidum on immunohistochemistry : ( a ) multiple interstitial clumps of spirochetes ( 200 ) ; ( b ) vascular trapping of spirochetes ( 400 ) ; ( c ) prominent accumulation of spirochetes in the microvasculature wall ( 400 ) . early syphilis . dermal homing of t. pallidum on immunohistochemistry : ( a ) multiple interstitial clumps of spirochetes ( 200 ) ; ( b ) vascular trapping of spirochetes ( 400 ) ; ( c ) prominent accumulation of spirochetes in the microvasculature wall ( 400 ) . in the present case , skin immunohistochemistry shed some light on the diagnosis of syphilis in an aids patient and showed the dermal homing and the microvascular tropism of t. pallidum . it is acknowledged that during a five - year period after inoculation , t. pallidum spreads to every organ . a long time later , it commonly re - emerges as a chronic and deadly illness . at any stage in its evolution , syphilis may mimic a number of other unrelated diseases . during the past decade , a sizable proportion of the population with syphilis corresponded to gay men coinfected with hiv . when the clinical diagnosis of syphilis is not established , a skin biopsy sometimes is submitted to the dermatopathologist without any relevant information . at the conventional histological examination , the diagnostic clues for syphilis are not always obvious because the disease presentation depends on both the host immunological response to the infection and the diverse angioinvasive propensity of the t. pallidum strains indeed , the histochemical silver stain may be difficult to interpret owing to heavy background staining . immunohistochemistry using an antibody directed to t. pallidum was reported to improve the histological diagnostic accuracy of syphilis . the present finding was assumed to illustrate the migration of t. pallidum toward the microvasculature during early syphilis . in summary , t. pallidum were abundant and heavily clustered in some specific portions of the skin . the peculiar homing of t. pallidum in the skin appears quite specific as it has not been reported for any other infectious microorganism .
during the past decade or so , the incidence of syphilis has increased in most parts of the world . in some urban regions , a coinfection with human immunodeficiency virus is disclosed in nearly 50% of the cases . owing to the polymorphism of the lesions , the clinical diagnosis may be puzzling . the homing patterns and migration paths of treponema pallidum in the skin during early syphilis represent the preliminary steps preceding dissemination to other organs . immunohistochemistry directed to t. pallidum is a convenient means for reaching the diagnosis and for exploring the dissemination process . the present case illustrates the dermal clustering and the vascular spread of t. pallidum in a woman with acquired immunodeficiency syndrome .
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the roots of zapoteca portricensis is a common remedy in the treatment gastrointestinal disorders used by tradomedical practitioners in eastern nigeria . this study was aimed at evaluating the possible antiulcer activity of the root of this plant in experimental rats . different groups of albino rats of male sex were given three doses ( 50 , 100 , 200 mg / kg ) of the extract . the ethanol model produced an average ulceration in rats with reduction of ulcer ( 50% , 75% and 90% ) seen in all the extract treatment . a dose dependent inhibition of ulcer was seen in all doses of the extract with doses 100 and 200 mg / kg produced a significant reduction compared with control . in the indomethacin model , inhibition of ulcer ( 57.1% , 65.7% and 80.0% ) was seen in the treatments with the extracts in a dose dependent manner . this study has shown that roots of this plant ( zapoteca portoricensis ) possess potent antiulcer activity .
background : the roots of zapoteca portricensis is a common remedy in the treatment gastrointestinal disorders used by tradomedical practitioners in eastern nigeria.aim:this study was aimed at evaluating the possible antiulcer activity of the root of this plant in experimental rats.methods:a methanolic root extract was prepared by cold maceration . antiulcer activity was tested using absolute ethanol and indomethacin induced ulcer models . sucralfate ( 100 mg / kg oral ) was used as the reference drug . different groups of albino rats of male sex were given three doses ( 50 , 100 , 200 mg / kg ) of the extract . phytochemical analysis of the freshly dried roots was also done.results:phytochemical results revealed presence of alkaloids , terpenoids , glycosides and flavonoids . the ethanol model produced an average ulceration in rats with reduction of ulcer ( 50% , 75% and 90% ) seen in all the extract treatment . a dose dependent inhibition of ulcer was seen in all doses of the extract with doses 100 and 200 mg / kg produced a significant reduction compared with control . in the indomethacin model , an absolute ulceration was produced in all the animals . inhibition of ulcer ( 57.1% , 65.7% and 80.0% ) was seen in the treatments with the extracts in a dose dependent manner . all the three doses of the extracts produced significant ulcer protection compared with control.conclusion:this study has shown that roots of this plant ( zapoteca portoricensis ) possess potent antiulcer activity .
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macroscopically , renal cell carcinomas ( rccs ) generally present as either solid or cystic mass . among cystic rccs , multilocular cystic rcc represents a rare entity that was recognized in 1982 , with a reported incidence of 1 - 4% of all rccs . renal leiomyomas are benign tumors of the kidney originating from smooth muscle cells of the renal capsule , pelvis , calices , or blood vessels . although small renal leiomyomas can be found in about 5% of autopsy specimens , clinical incidence of these lesions is much lower . in this report , we discuss the first case of simultaneous occurrence of a multilocular cystic rcc and a leiomyoma in the same kidney . a 38-year - old man was referred for treatment of a right renal cystic mass found incidentally . physical examination on admission revealed no palpable mass , and laboratory data were negative . computed tomography of the abdomen showed a about 5.4 4.6 cm poorly enhancing cystic mass with enhancing septa in the polar area of the right kidney ( fig . since multilocular cystic rcc could not be excluded , the patient underwent right laparoscopic radical nephrectomy . macroscopically , in the mid pole , a well - circumscribed mass showing a multicystic feature with thin fibrous septa and without expansile tumor nodules was noted . rccs show cystic changes on imaging studies in 4 - 15% of cases [ 4 , 5 ] . since it is often difficult to differentiate cystic rccs from benign cystic lesions , a definite diagnosis can in most cases only be established by histopathologic examination . these include : ( 1 ) intrinsic multiloculated growth ; ( 2 ) intrinsic unilocular growth ; ( 3 ) cystic necrosis , and ( 4 ) origin from the epithelial lining in a pre - existing cyst . because multilocular cystic rcc are more likely to be discovered incidentally , this feature may contribute to their excellent prognosis compared to that of conventional rccs . since the majority of rccs originate from the proximal tubules , the characteristics and/or environment of the proximal tubules are considered to play an important role in the pathogenesis of rcc . recently , imura et al . carried out a detailed immunohistochemical analysis of multilocular cystic rcc cases . they found that in a high proportion of cases , multilocular cystic rcc reacted strongly with the distal nephron markers , but none reacted preferentially with proximal nephron markers . these results illustrate that multilocular cystic rcc originates from the distal nephron , although the precise pathogenesis of cystic formation in multilocular cystic rcc has not been elucidated clearly . renal leiomyomas are benign tumors arising from the mesenchymal ( or connective ) tissue of the kidney . tumors may be subcapsular ( 53% ) , capsular ( 37% ) , or located in the renal pelvis ( 10% ) . a variety of structural patterns have been described at imaging : solid , cystic , and both cystic and solid [ 9 , 10 ] . in 1990 , steiner et al . the first group comprises small cortical or subcortical neoplasms that are usually asymptomatic , less than 2 cm in size , and are often detected incidentally during autopsy or surgery . the second group consists of larger neoplasms that arise from the renal capsule or blood vessels and may be symptomatic . unfortunately , it is almost impossible to clinically distinguish renal leiomyomas from their malignant counterparts . because of their small size ( usually less than 2 cm ) , these smooth muscle - containing tumors are commonly diagnosed during autopsy , and clinical manifestations become apparent only when they grow larger , hemorrhage , or undergo cystic or sarcomatous degeneration . since the finding of primary synchronous renal neoplasms is very uncommon , especially when they have a different histogenesis , up to now , only a few primary , synchronous rccs with different histotypes have been reported . the coexistence of multilocular cystic rcc and leiomyoma has not been documented . this present case is the first report of synchronous renal neoplasms with different histogenesis ( multilocular cystic rcc and leiomyoma ) in a patient without any symptoms . the significance of the relationship between multilocular cystic rccs and leiomyomas is not well understood and needs further exploration .
we present an unusual case of concurrent occurrence of a multilocular cystic renal cell carcinoma and a leiomyoma in the same kidney of a patient with no evident clinical symptoms . a 38-year - old man was found incidentally to have a cystic right renal mass on computed tomography . laparoscopic radical nephrectomy was performed under a preoperative diagnosis of cystic renal cell carcinoma . histology revealed a multilocular cystic renal cell carcinoma and a leiomyoma . this is the first report of this kind of presentation .
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pregnancy course was regular , except for maternal consumption of mebendazol at 5 weeks ' gestation to treat a pinworm infection . maternal history was negative for consumption of any known teratogenic class drug during the whole pregnancy . clinical examination at birth was normal , except for complete congenital anonychia of left middle finger ( fig.1 ) . x - ray was performed , showing an absence of the left middle finger distal phalanx ( fig.2).the rest of the nails on the fingers and toes were normal . parents were not consanguineous and have normal fingernails and toenails . the baby was discharged on the third day of life , and was found healthy and thriving at three - month follow - up visit . final diagnosis was that of a sporadic co. x - ray showing absence of left middle finger distal phalanx . congenital onychodystrophy ( co ) , also called as iso kikuchi syndrome , was first described by iso in 1969 1 and later by kikuchi in 1974 2 as a clinical syndrome involving dysplasia / absence of fingernails with underlying bone abnormalities . since these first observations , clinical criteria have been expanded to include a number of additional associated conditions derived from small series , case reports , and retrospective reviews over the next 30 years . co clinical criteria are the following : unilateral or bilateral hypoplasia of the index fingernails and/or other fingers including toenails 3 ( up to total anonychia of hands and feet ) ; radiographic abnormalities of the distal bony phalanx of the affected fingers ; congenital occurrence , which can be both sporadic or hereditary 4,5 . unilateral or bilateral hypoplasia of the index fingernails and/or other fingers including toenails 3 ( up to total anonychia of hands and feet ) ; radiographic abnormalities of the distal bony phalanx of the affected fingers ; congenital occurrence , which can be both sporadic or hereditary 4,5 . genetic loci responsible for the condition are still under investigation : linkage to the known keratin gene clusters on 12q12 and 17q21 has been excluded by krebsova et al . in 2000 ; 7 a putative isolated congenital nail dysplasia locus , designated ndic , has been identified on 17p13 , although the identified region harbors no genes known to be involved in skin or nail abnormalities 7 . several acquired isolated nail disorders which may represent differential diagnoses of co are presented in table1 8 . differently from all these conditions , co presents as a congenital hypoplasia , dysplasia , or absence of one or more fingernails , and is typically accompanied by underlying phalanx bone disease . acquired isolated nail disorders 8 several heterogeneous multisystem pathologies may also come with ungueal abnormalities ; these are summarized in table2 813 . 813 triangular nail lunula fingernails and toenails hypoplasia 8 absent or hypoplastic patellae elbow dysplasia , often involving posterior subluxation of the radial head iliac horns dysplasia arrested or reduced nail growth thickened and over - curved nails with absence of cuticle differently from the above - mentioned conditions , classic co does not come with involvement of systems other than nails and relative phalanges . kikuchi originally suggested fetal grip as a causative mechanism of nail and phalanx ischemia , leading to dysplasia or even complete resorption of such structures 14 . this theory , however , is not consistent with the recent acquisitions on developmental biology stating that limbs and bones develop early in fetal life , prior to fetus ' ability to exert a significant grip . more recently , several pathogenetic mechanisms have been proposed for this condition : selective abnormal fetal vascular supply from palmar digital artery causing in utero ischemic injury : the ischemic damage would mainly be seen on the radial side of the affected finger due to the smaller caliber of the artery on that side 15 , which should be more protected than the ulnar one;16 in utero dysplastic change in the crescent - shaped cap of the distal phalanx , a theory that would account for the frequent y shape of the dysplastic distal phalanx , when present;17 genetic mutations causing impairment of the wnt signaling pathway , an evolutionarily conserved signal transduction pathway that plays a pivotal role in embryonic development , growth regulation of multiple tissues , and cancer development;18 fetal exposure to teratogens , particularly antiepilectic drugs : phenytoin 19 , valproate , and carbamazepine20 selective abnormal fetal vascular supply from palmar digital artery causing in utero ischemic injury : the ischemic damage would mainly be seen on the radial side of the affected finger due to the smaller caliber of the artery on that side 15 , which should be more protected than the ulnar one;16 in utero dysplastic change in the crescent - shaped cap of the distal phalanx , a theory that would account for the frequent y shape of the dysplastic distal phalanx , when present;17 genetic mutations causing impairment of the wnt signaling pathway , an evolutionarily conserved signal transduction pathway that plays a pivotal role in embryonic development , growth regulation of multiple tissues , and cancer development;18 fetal exposure to teratogens , particularly antiepilectic drugs : phenytoin 19 , valproate , and carbamazepine20 a literature research showed no proven association between the use of mebendazole during pregnancy and co. conversely , helminth infection during pregnancy is associated with poor cognitive and gross motor outcomes in infants , so that measures to prevent helminth infection during pregnancy should be reinforced;21 anthelminthic therapy is actually recommended in infected pregnant women , and it has been associated to a decreased rate of maternal anemia and low birth weight 22 . nevertheless , clinical follow - up is needed in order to exclude other conditions associated with multisystem pathology ; a complete family history may also be warranted to determine sporadic or hereditary transmission of such a condition .
key clinical messageclassic co ( also called iso kikuchi syndrome ) represents a benign , isolated condition associated with normal patient outcome . nevertheless , clinical follow - up and/or further clinically - based tests are needed to exclude other nail diseases associated with multisystem pathology ; complete family history is also important to determine sporadic or hereditary transmission of such condition .
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port - wine stains ( pwss ) are congenital vascular malformations comprised of ectatic dermal capillaries and affecting 0.3 - 1% of newborn infants . acquired pwss are an uncommon entity developing later in life , but morphologically and histologically similar to the congenital lesions . we hereby report a case of 41-year - old male patient who acquired pws about a year ago . a 41-year - old male presented to the outpatient department of dermatology of our tertiary care hospital with complaint of asymptomatic reddish patches involving the lower part of right leg for 10 months . the patch started from near the ankle and gradually spread to involve whole of the medial aspect of right leg over a period of 10 months . we could not elicit any history of antecedent mechanical or thermal trauma , drug intake , topical application or excessive ultraviolet exposure . dermatological examination revealed a diffuse partially blanchable erythema extending from the right ankle and involving chiefly the medial aspect of right leg up to the knee [ figure 1 ] . there were also a few discrete irregularly shaped partially blanchable reddish - purple macules present on the anterior aspect of right leg [ figure 2 ] . a diffuse partially blanchable erythema extending from the right ankle and involving the medial aspect of right leg up to the knee discrete irregularly shaped partially blanchable reddish - purple macules present on the anterior aspect of right leg on the basis of the clinical examination , a provisional diagnosis of acquired pws was made and the patient investigated . the histopathological examination revealed multiple dilated , variably sized capillaries lined by a single layer of endothelial cells throughout the dermis showing lobular arrangement at places [ figure 3 ] . color doppler of the limb did not reveal any abnormality of the underlying arteries and veins . ( a ) multiple dilated variably sized capillaries in the dermis ( h and e , 40 , arrows ) . ( b ) high power showing groups of capillaries lined by a single layer of endothelial cells ( h and e , 250 , arrow ) laser therapy was discussed with the patient , but he declined treatment for the lesion . pwss are congenital vascular malformations usually presenting at birth in the form of pink - red to purple macules , which become darker , raised , and nodular as the person ages . the most accepted hypothesis is a defective embryological maturation of the sympathetic fibers , resulting in a loss of normal sympathetic control of the cutaneous vessels which leads to ectasia . various factors have been proposed for the occurrence of acquired pwss . of these , trauma has been found to be the most important causative factor in majority of the cases . trauma - induced pws was first described by fegeler in 1949 and hence called fegeler syndrome . in a review of 59 patients with acquired pws , trauma was found to be a causative factor in 17 ( 29% ) cases . it has been proposed that injury may result in loss of a previously effective sympathetic regulation of the cutaneous blood flow , leading to development of the lesions . reports of acquired port - wine stain few cases of acquired pws have also been proposed secondary to oral medications like isotretinoin , oral contraceptive pills , simvastatin , and metformin . isotretinoin causes skin fragility and frictional trauma , while simvastain and metformin have been shown to promote angiogenesis by upregulation of vascular endothelial growth factor . isolated cases of acquired pws following frostbite injury , obstruction of the peritoneovenous shunt , herpes zoster infection , cerebral arteriovenous malformation , spinal root compression , and solid brain tumor have also been reported . no definite cause was found for the development of lesion in our patient . in such cases , a likely possibility of having a latent vascular anomaly which manifests clinically after unnoticed trauma an unusual finding in our patient was the presence of a lobular arrangement of dilated vessels in the dermis which has not been observed in previous cases of congenital or acquired pws . interestingly , there are multiple reports of development of pyogenic granulomas ( pg ) within pws which also shows a lobular pattern of dilated vessels . it has been proposed that the pg probably results from an underlying arteriovenous anastamosis frequently associated with the pws . though , there was no clinically appreciable pyogenic granuloma like lesion , the distinct histological features seen in our patient might be attributed to a similar pathogenetic mechanism . as regards therapy , it includes treatment with krypton , argon , argon - pumped , tunable dye , neodymium : yttrium - aluminum garnet , copper vapor , and pulsed dye lasers . acquired pws tends to have a quicker and better response to pulsed dye therapy than congenital pws . the case report demonstrates a rare occurrence of a vascular malformation in an adult patient . although no definite cause could be appreciated for the development of lesion in our patient , further reports and future insights into the pathogenesis of such cases would broaden our knowledge regarding this infrequently reported phenomenon . this is the first report of an acquired port - wine stain from india and previous reports of such cases have also been reviewed .
port - wine stains ( pwss ) are congenital vascular lesions caused by progressive ectasia of blood vessels located in the vascular plexus of the dermis . acquired pwss develop later in life but are identical in morphology and histology to the congenital pwss . less than 75 cases of acquired pwss have been reported in the published literature , of which there has not been a single report from india so far . various factors have been proposed for its pathogenesis like trauma , actinic exposure , drugs , tumors , and herpes zoster infection . we report an acquired port - wine stain in a 41-year - old male . the causative factors , treatment , and previous reports of this uncommon entity have also been reviewed .
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angiomyolipoma is a benign tumor that is histologically composed of groups of mature adipose tissue intermixed with convoluted thick - walled blood vessels , interlacing bundles , and irregularly arranged sheets of smooth muscle , with the kidney as the most frequent site of involvement . extrarenal angiomyolipoma can occur in organs such as the liver , lung , uterus , and skin . only two instances of this tumor arising in the tongue have previously been reported [ 1 , 2 ] . a 61-year - old woman was referred to our hospital with a mass in the left proglossis that was painless , but had enlarged slowly for 5 years . the mass measured 20 mm 20 mm and was soft and dome shaped with a dark violet - colored surface ( figure 1 ) . the mass was well - demarcated and easily dissected with an ultrasonic surgical aspirator ( figure 2 ) . histopathological examination showed an encapsulated lesion composed of a proliferation of an intricate mixture of mature adipose tissue , blood vessels , and smooth muscle ( figure 3(a ) ) . the patient 's postoperative course was uneventful , and there were no signs of recurrence at followup at 18 months ( figure 4 ) . koizumi et al . reported a case of angiomyolipoma in the centre of the tongue . described a case of a angiomyolipomatous hamartoma arising in the left lateral border of the tongue . in the present case , angiomyolipoma arose in the proglossis and it had a diagnosis of hemangioma because of its hardness and color . because the three principal components in angiomyolipoma , regardless of its location , vary greatly in proportion and distribution , its heterogeneity may cause diagnostic confusion . the differential diagnosis includes lipomatous or myolipomatous tumours , angiomyoma , angiolipoma , hemangioma , fibroma , and fibrolipomatous hyperplasia . angiomyolipoma could be considered a hamartoma , but there is no consensus that these lesions are a single entity . ide et al . reported that the present lesion may not be a classic oral angiomyolipoma because it was poorly circumscribed and not encapsulated . they suggested the term angiomyolipomatous hamartoma to designate this lesion . in agreement with ide et al . , we diagnosed the present case as an angiomyolipoma because the mass had enlarged gradually with active proliferation and was well - encapsulated . in particular , tongue cancer and hemangioma are tumors with many blood vessels in the proposed area of surgical excision . for preservation of the nervous system and avoidance of bleeding , an ultrasonic surgical aspirator is effective in the resection of these tongue tumors [ 35 ] . in our case ,
a 61-year - old woman was referred to our hospital with a mass in the left proglossis . the lesion was excised with a provisional diagnosis of hemangioma . the mass was well - demarcated and easily dissected with an ultrasonic surgical aspirator . the histopathologic diagnosis was angiomyolipoma arising in the tongue . there were no signs of recurrence at followup at 18 months .
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it is an unusual " atypical " antipsychotic , as the " atypical " profile of the new antipsychotics clozapine , olanzapine , quetiapine , and risperidone has been linked to combined antagonism of serotonin 2 ( 5-ht2 ) and dopamine 2 ( d2 ) receptors , whereas amisulpride has negligible affinity for 5 ht2 receptors and is specific for dopamine d2 and d3 receptors in the limbic rather than striatal structures . there are reports of amisulpride being used for the management of bipolar disorders . however , there are very few reports of mania induced by amisulpride . here we describe a young male who developed mania while on amisulpride , a newer antipsychotic drug introduced in india . a 18-year - old unmarried hindu male presented with a history suggestive of schizophrenia for the last 2 years . the illness had an acute onset with complaints of suspiciousness , hearing voices not heard by others , violent abusive behavior , disturbed biological functions , and decreased self care . he was started on risperidone 4 mg along with trihexyphenidyl 2 mg and lorazepam 6 mg per day . gradually , over a period of 1 month , he showed improvement in the symptoms . subsequently , trihexyphenidyl and lorazepam were tapered and he was maintained on risperidone 4 mg per day for the next 1 and a half year . however , he would still be lethargic , would prefer to remain alone and would be inattentive at class . he would not interact with others and not take active part in various household functions . he was prescribed amisulpride for the negative symptoms along with risperidone 4 mg that was continued . it was initiated at 50 mg per day and was increased to 100 mg per day after 4 days of initiation it . after 10 days of 100 mg dose , the patient developed a manic episode , characterized by decreased need for sleep , over talkativeness , hyperactivity , persistent elevated mood , disinhibited behavior , over - grooming , and distractibility . amisulpride was stopped and he was prescribed lorazepam 4 mg on as and when required basis . the patient was followed up after 1 week and during this time there was substantial improvement in his manic symptoms with ymrs score of 17 . to the best of our knowledge of mania due to first amisulpride was reported by murphy in 2003 . however , in the case described by murphy , the patient was also on citalopram , an antidepressant , though its discontinuation did not led to improvement in manic symptoms . also , the patient was initiated on olanzapine immediately which would also probably have antimanic effects . in our case , use of the naranjo adverse drug reaction probability scale and edward 's criteria both indicate a probable relationship between the manic episode and short - term exposure to amisulpride therapy . the manic symptoms with amisulpride can be postulated to be due to the fact that amisulpride in low doses ( < 10 mg / kg ) , preferentially blocks presynaptic d2/d3 receptors , resulting in enhanced dopamine transmission . increased dopamine metabolites and increased dopamine transmission this case report highlights the fact that close monitoring of patients on amisulpiride should be done for manic symptoms . this is also important because of the fact that atypical antipsychotic drugs ( including amisulpride ) are used for the treatment for bipolar disorder . open label studies have shown that amisulpride may be useful for patients with bipolar disorder . also , as low doses enhance dopamine transmission , and high doses reduce dopamine transmission , it might be prudent to start amisulpride at the recommended target dose at the initiation of therapy .
amisulpride is an atypical antipsychotic used for the management of schizophrenia and other conditions like dysthymia . it has also been used for the management of bipolar disorders as an add on therapy . here , we report a patient of schizophrenia who developed a manic episode while on amisulpride .
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acoh ( 3.00 mmol ) was added to a solution of galactoside 20 or 13 ( 1.57 mmol ) in acetone ( 3.5 ml ) and meoh ( 4.7 ml ) at 0 c . after 8 h , the reaction was quenched by the addition of nahco3 ( 5.95 mmol ) and then filtered . after concentration of the filtrate under reduced pressure , the resulting oil was purified by flash column chromatography ( etoac in hexane ) to afford alcohol 21 ( from 20 ) or 26 ( from 13 ) as a colorless oil . pph3 ( 1.86 mmol ) , diad ( 1.86 mmol ) , and dppa ( 1.86 mmol ) were added sequentially to a cooled solution of alcohol 21 or 26 ( 0.90 mmol ) in thf ( 20 ml ) . concentration under reduced pressure followed by purification of the residue by flash column chromatography ( etoac in hexane ) afforded azide 22 ( from 21 ) or 25 ( from 26 ) as a colorless oil . tfa ( 0.50 ml , 6.6 mmol ) was added dropwise over 5 min to a solution of azide 22 ( 200 mg , 0.19 mmol ) in ch2cl2 ( 5 ml ) at rt . after 30 min , the reaction mixture was concentrated under reduced pressure to afford pentaol 9 as a colorless oil ( 115 mg , quant . ) : [ ]d + 12.4 ( c 0.5 , cdcl3:cd3od , 2:1 ) ; max(film ) ( cm ) 3282s br ( oh ) , 2114s ( n3 ) , 1696 m ( c = o ) ; h nmr ( 300 mhz , cdcl3:cd3od , 2:1 ) 0.85 ( t , j = 6.0 hz , 3h ) , 1.181.39 ( stack , 22h ) , 1.401.71 ( stack , 4h ) , 1.44 ( s , 9h ) , 3.26 ( a of abx , jab = 12.6 hz , jax = 4.9 hz , 1h ) , 3.513.64 ( stack , 3h ) , 3.653.98 ( stack , 7h ) , 4.89 ( d , j = 3.3 hz , 1h ) ; c nmr ( 100 mhz , cdcl3:cd3od , 2:1 ) 14.3 ( ch3 ) , 23.2 ( ch2 ) , 26.4 ( ch2 ) , 28.6 ( ch3 ) , [ 29.9 , 30.2 , 32.4 , 32.8 ( ch2 , resonance overlap ) ] , 51.7 ( ch ) , 51.8 ( ch2 ) , 68.3 ( ch2 ) , 69.3 ( ch ) , 70.4 ( ch ) , 70.6 ( 2 ch , resonance overlap ) , 72.5 ( ch ) , 75.3 ( ch ) , 80.1 ( c ) , 156.8 ( c ) ; ms ( tof es+ ) m / z 627.3 ( [ m + na ] , 100% ) ; hrms ( tof es+ ) calcd for c29h56n4o9na [ m + na ] 627.3945 , found 627.3956 . tfa ( 1.0 ml , 13.2 mmol ) was added dropwise over 5 min to azide 22 ( 400 mg , 0.38 mmol ) at rt . the resulting colorless oil was used in the next step without further purification ( 192 mg , quant . ) . tfa ( 0.50 ml , 6.6 mmol ) was added dropwise over 5 min to azide 9 ( 114 mg , 0.19 mmol ) at rt . the resulting colorless oil was used in the next step without further purification ( 96 mg , quant . ) .
a concise route to the 6-azido-6-deoxy--galactosyl - phytosphingosine derivative 9 is reported . orthogonal protection of the two amino groups allows elaboration of 9 into a range of 6-n - derivatized -galactosyl ceramides by late - stage introduction of the acyl chain of the ceramide and the 6-n - group in the sugar headgroup . biologically active glycolipids 6 and 8 have been synthesized to illustrate the applicability of the approach .
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does it have any bearing on outcome in the icu or during the post - icu period ? although not evidence based in any prospective , controlled , randomized clinical trials , it is unlikely that anyone would dispute that under - feeding eventually leads to death . this parallels the lack of controlled studies on the use of parachutes when jumping out of aeroplanes at high altitude . nevertheless , a short period of starvation appears to be a part of clinical treatment in many icus . this is highlighted by a report from colleagues in the netherlands presented in this issue of critical care . intensive care medicine is a comparatively young speciality , and within our arsenal of treatments the amount of evidence - based knowledge is often embarrassingly low . an illustration of this is seen in the guidelines for the surviving sepsis campaign , in which the evidence for different treatment modalities was evaluated in a formalized way . when we come to icu nutrition , there is little evidence at hand in terms of prospective , randomized , controlled clinical trials ; this was highlighted in recently published canadian guidelines . . some authors even call it ' poisonous nutrition ' and ban it from use in the icu . most investigators and authorities in the field would advocate enteral nutrition before parenteral , merely on the grounds that it is the natural way to feed and is cheaper . the evidence in favour of enteral over parenteral nutrition , or the other way around , is weak ; furthermore , it is obsolete because it was generated more than 10 years ago , with obscure indications for parenteral nutrition and with no blood sugar control . in studies comparing enteral and parenteral nutrition in patients whose attending physician is unsure regarding which modality will be optimal , the results demonstrate a very low feeding success rate with enteral nutrition and that complications are related to the duration of nutritional treatment , regardless of the mode of administration . in general terms it has repeatedly been shown that when patients are given only enteral nutrition , the success of feeding is below 70% . successful feeding may be defined in terms of the percentage of prescribed calories , number of days with at least 80% of prescribed calories delivered , or any other measure . the study from the netherlands presented in this issue adds further evidence to a number of publications that demonstrate that clinical practice is equivalent to delivering as little as 50% of the prescribed kilocalories . does this make any difference ? probably , a very large study population would be required to demonstrate that patients do worse when they are administered only 50% of the prescribed dose of antibiotics . the safety margin for bacterial kill when administrating antibiotics is such that reducing doses to half would require 500010,000 patients to show a difference in a prospective study . still no - one would advocate such a regimen . however , the side effects of antibiotics create renal insufficiency , liver insufficiency and drug fever , and predispose to development of multi - resistant bacteria . nevertheless , no - one would deliberately give only 50% of the prescribed dose , stating that it does not matter whether 50% or 100% of the dose is administered . when it comes to nutrition , however , many of us do this . in the study presented in this issue , the post hoc analysis shows that any action that reflects an interest in nutrition , such as placing the tube in any other position than the stomach , using some type of enhanced formula , or having a percutaneous feeding device , increases success rates with nutrition . these specially treated patient groups were small , and findings in these groups should perhaps not be used as a basis for clinical recommendations , but it is clear that an enhanced interest in nutrition and delivery of nutrition will increase success rates . so , the main reason for the systematic under - feeding that is practiced in icus is probably a lack of interest by the attending physician . binnekade and coworkers do not give any information on success rates of feeding in relation to outcome . this is a difficult area , and studies that try to link nutritional practice to outcome must be designed carefully . there are examples in the literature of investigators jumping to conclusions based on insufficient information . nevertheless , in any patient in whom a huge energy deficit is built up , resulting in malnutrition , increased risk for complications can be predicted . in addition , evaluation of nutritional protocols in terms of success rate , nutrition related complications and outcome must be encouraged . systematic under - feeding of icu patients may be used as a marker of suboptimal care . wherever it is considered a human right not be hungry , the burden of producing proper evidence should be imposed on anyone who suggests that half the feed is good enough .
systematic undernutrition of intensive care unit patients is common and neglected . is this inevitable or can better routines and protocols make a difference ? the necessity of feeding may be regarded as self - evident , but more evidence is obviously needed to strengthen this issue . in rich countries it should be a human right not to be hungry .
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the treatment of chronic hepatitis c ( chc ) has evolved in the last 15 years from monotherapy with interferon alpha ( ifnalpha ) to the combination treatment with pegylated ifn ( peg - ifn ) plus ribavirin for 24 - 48 weeks . up to now , several viral , host and drug - related reactions in response to ifnalpha - based therapy have been identified . recent studies suggest that liver inflammation in chc is controlled by several mechanisms , including host regulatory immune responses and viral polypeptides interacting with cells involved in innate and adaptive immunity . it is well known that cutaneous side effects of treatment with ifnalpha alone or ifnalpha plus ribavirin in patients with chc have been widely reported , beyond the fact that the virus itself can cause skin lesions . however , the cutaneous side effects during therapy of chc are of inflammatory type with local erythema , edema and , much less frequently , necrosis at the injection skin sites . by contrast , skin side effects of such drugs have few data available in the literature , although the number of reports has been increasing in the last years , including cosmetic filler site injections . roughly one half of these patients had cutaneous findings , either alone or with systemic involvement . more recently there have been reported dermatological diseases in patients receiving the combination of ifn / ribavirin or ifn / ribavirin / amantadine for the treatment of chc . a 44-year - old man with chc genotype 1a and a viral load of hcv > 800,000 iu / ml , a2-f1 on liver biopsy according to metavir criteria , was treated for the hepatic disease . peg - ifnalpha-2b 1.5 g / kg weekly and 1,000 mg of ribavirin daily was introduced . in the 40th week the patient reported sudden intense pain , pruritus , erythema and skin hypertrophy in the left deltoid area over the seahorse tattoo and in a scar on his face ( fig . 1 ) . a skin biopsy on the scar and tattoo showed granulomatous dermatitis ( fig . chest x - ray was normal and the level of angiotensin enzyme converter was 50 iu / ml ( range 18 - 55 iu / ml ) . we intend to call attention especially to dermatologists to the possibility of a granulomatous tattoo reaction as a side effect during chc treatment with peg - ifnalpha-2b plus ribavirin . until now , eight cases of sarcoidosis as a complication of ifn therapy in chc have been reported in the english language literature . the most relevant topic in this case is the occurrence of disease on a tattoo area . six months later retreatment using peg - ifnalpha-2a 180 g once a week plus ribavirin 1,000 mg daily was tried . in the 6th week , cutaneous symptoms relapsed in an unbearable way . the patient did not accept to continue therapy , since hydrocortisone cream was prescribed again but no satisfactory pruritus and pain improvement was noticed . a prompt regression of skin reaction occurred after stopping therapy . sarcoidosis is a granulomatous disorder of unknown etiology and whose epidemiology suggest a genetic tendency face to infectious agents being supposed to result of immune system deregulation leading to non - caseating granulomas as an immune reaction to an unknown persistent antigenic stimulus . ifn has been linked to pulmonary macrophage activation , a characteristic feature of sarcoidosis which has been assumed an exaggerated t helper 1 ( th-1 ) immune response to a variety of exogenous antigens . it seems very likely that a potent immunoregulatory protein for th-1 response such as ifn may induce the disease . in january 2003 the eighth case of ifn - related sarcoidosis was reported with a review of the literature . chest x - ray revealed hilar lymphadenopathy in three patients , with reticulonodular shadows in another three , while the patient with only cutaneous involvement had a normal chest x - ray . the case we report presented with cutaneous sarcoidosis during chc therapy and spontaneous regression of the lesions was noted with treatment discontinuation . we intend to emphasize , especially to dermatologists , the risks of a granulomatous tattoo and other cutaneous sites during chc treatment with peg - ifnalpha-2b plus ribavirin . thalidomide has been also shown to have specific activity to the inflammatory mediators of sarcoidosis and to be an alternative beneficial therapy .
the treatment of chronic hepatitis c ( chc ) has evolved in the past 15 years and combination of pegylated interferon plus ribavirin is its current standard therapy . however , several side effects are commonly observed and frequently lead to transient or definitive interruption of treatment . although sarcoidosis in its systemic or cutaneous form is a very rare side effect in such circumstances , some cases have been reported even with conventional interferon . this brief review of the literature and description of a case of sarcoidosis occurring in a tattoo and a scar patient 's face , during treatment with pegylated interferon alpha-2b plus ribavirin , is an educative report directed in special to dermatologists . the lesion improved after drug interruption and recurred after retreatment with pegylated interferon alpha-2a . we conclude that this side effect must call the attention of doctors to seek for the diagnosis and therapy as soon as possible in such circumstances . no differences were noticed neither with alpha-2a nor alpha-2b pegylated interferon employment .
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