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a 60-year - old lady presented to our sister department of medical gastroenterology at a tertiary health care institute with a history of an increasing mass in the right upper abdomen of one and a half year duration . she also had a history of weight loss due to early satiety and decreasing appetite . she had been evaluated with abdominal cect 14 months before by some local doctor which had shown a mass lesion in the right lobe of the liver . all her systemic examinations were normal except a big nodular mass in the right hypochondrium merging with the liver . the department of medical gastroenterology considered her for a repeat workup , with abdominal mri , rbc trigged nuclear scan of the liver , serum tumor markers and routine base investigations . mri showed a nodular mass lesion arising from the right lobe of the liver , crossing towards the left lobe with involvement of the right portal vein , with areas of cystic changes and hemorrhage within the lesion ( fig . her tumor markers , including -fetoprotein , cea , ca 19 - 9 and hcg , were all within normal limits . she was also subjected to upper and lower gastrointestinal endoscopy to rule out any occult primary lesion in the gastrointestinal tract . routine laboratory investigations including liver function tests , hemogram , kidney function , chest x - ray and ecg were within fairly normal limits , her coagulogram revealing pt elevated by 3 s. serology was negative for hepatitis b and c. she was also subjected to usg guided fnac which was inconclusive and revealed only blood cells . after complete workup with a tentative diagnosis of fibrolamellar hepatocellular carcinoma the patient was referred to our department for surgical intervention . she was offered a diagnostic and if possible curative laparotomy after repeat imaging ( abdominal cect ) as she had again lapsed a period of 34 months after her workup . due to monitoring constraints , her attenders did not agree to get an abdominal ct but were ready for any possible surgical intervention with the minimum of a biopsy to plan further treatment . on exploration the patient was found to have nodular bilobular disease occupying almost all of the liver . there were areas of boggy swelling in between firm nodules predominantly on the inferior surface of liver suggestive of bleeding within the tumor on aspiration . the lesion involving both lobes of the liver was too big and multicentric for any resectional procedure and so a biopsy from the lesion was taken safely . after getting her histopathological report the patient was referred to a medical oncologist for further treatment . this is the first case of fibrosarcoma of the liver reported at our institute . on light microscopy the tumor revealed homogeneous , spindle - shaped cells with abundant collagen fibers showing a classic herringbone pattern ( fig . tissue samples were then sent to another referral cancer hospital for immunohistochemistry where immunoreactive vimentin was found in the tumor cells . electron microscopically , the tumor cells were rich in rough endoplasmic reticulum without a basement membrane and were surrounded by large amounts of collagen fibers . the purpose of this case report is to share information on a rare tumor of the liver , a primary fibrosarcoma , as up to the late seventies the literature about this entity was restricted to autopsy diagnosis . to our knowledge there are case reports from various parts of the world [ 2 , 3 ] , but there are no series of cases of primary fibrosarcoma of the liver . etiologically exposure to cadmium has been linked to carcinogenesis of this disease in animal models . described a series of 20 cases of sarcomatous tumors of the liver , of which angiosarcoma was the commonest , and concluded that primary treatment is surgery with survival depending on the degree of differentiation . from the available literature , primary treatment to be considered for fibrosarcoma of the liver is surgery with curative intent . in a recent article from sloan - kettering cancer center , between 1981 and 2004 they identified only 30 cases of primary liver sarcomas of which most cases ( 10 ) were hemangioendotheliomas and only 3 were diagnosed to be primary fibrosarcoma . only 11 of these patients underwent r0 resection and this group of resected tumors had a 5-year survival rate of 65% . unfortunately our patient presented quite late in the course of disease even if we consider her first ct scan done one and a half year back . there is a report of one patient with primary fibrosarcoma presenting with frequent episodes of hypoglycemia , where insulin - like growth factor ii was intensely stained in the golgi area of the tumor cells , suggesting its role in the mechanism of hypoglycemia . on histopathological examination these neoplasms show typical light microscopic features like that of malignant rhabdoid tumor with filamentous cytoplasmic inclusions ( fig . 4 ) , and on electron microscopic examination show staining for both cytokeratin and vimentin by immunohistochemistry . after making a diagnosis of primary fibrosarcoma of the liver at an inoperable stage , an extensive review of the literature regarding the role of other treatment modalities was done which was not encouraging [ 5 , 8 ] . though few reports recommend chemotherapy in liver metastasis of retroperitoneal fibrosarcoma consisting of cyclophosphamide , vincristine , farmorubicin , and dacarbazine , there is no definite role as treatment for primary lesion of liver . overall the prognosis reported by most of the authors is dismal [ 1 , 2 , 3 , 10 ] . we conclude by passing on the message that this rare entity does exist and is an area for workup to make guidelines for management of such tumors . this may be one of the few reported cases of primary hepatic fibrosarcoma confirmed by immunohistochemistry and electron microscopy .

a 60-year - old lady presented to us with a right upper abdominal mass . with a clinical diagnosis of liver tumor , she was evaluated with abdominal ct , mri , nuclear scan , tumor markers , usg guided fnac and other baseline investigations . on evaluation she had a massive right lobe tumor crossing the midline . in view of the ambiguous diagnosis she was subjected to laparotomy where the lesion was judged unresectable and a biopsy was taken . histopathological examination showed the rare pathology of primary fibrosarcoma of the liver with features of homogeneous , spindle - shaped cells with abundant collagen fibers showing a classic herringbone pattern . tissue samples were then sent to another referral cancer hospital for immunohistochemistry and immunoreactive vimentin was found in the tumor cells . electron microscopically , the tumor cells were rich in rough endoplasmic reticulum without a basement membrane , and were surrounded by large amounts of collagen fibers . the fibroblastic character of the tumor cells was suggested by light and electron microscopy .

congenital bochdalek hernia ( cbh ) develops as a result of the inappropriate fusion of the pleuroperitoneal fold and septum transversum at the site of the posterolateral lumbocostal trigone at the sixth week of gestation . this entity was first described by mccauley in 1754 and then by vincent alexander bochdalek in 1848 . this type of diaphragmatic hernia is considered a perinatal disease that presents a few hours after birth , or in early infancy , and it is associated with significant morbidity and mortality . cbh in adults is very rare ; it remains asymptomatic until adulthood and generally presents with thoracic and/or abdominal symptoms aggravated in supine position . we present a case of left - sided congenital bochdalek hernia that remained asymptomatic and was discovered incidentally during secondary cytoreductive surgery for epithelial ovarian cancer ; in this patient , a iatrogenic diaphragmatic defect was found , hyperthermic intraperitoneal chemotherapy ( hipec ) was performed and early postoperative bilateral anterior thalamic infarction developed . a 46-year - old female patient with a bmi of 25 was referred to our tertiary center ( cerrahpasa medical faculty , gynecological oncology unit ) in august 2016 with the diagnosis of platinum - resistant ovarian cancer . positron emission tomography and computed tomography ( ct ) revealed implants on the right diaphragmatic surface after first - line adjuvant chemotherapy . she has never had any thoracic or abdominal symptoms and no history of major trauma . initial preoperative evaluation of the patient revealed no significant sign of diaphragmatic hernia . her physical examination was normal , including chest auscultation sounds and chest x - ray . thoracic ct did not reveal any abnormal image that could be evaluated as a diaphragmatic hernia . she was placed in flank position , and , under general anesthesia , laparotomy was performed . an oval defect with the dimensions of 3 4 cm was seen in the left posterolateral site of the diaphragm ( fig . the most distal portion of the inferior lobe of the left lung was visible through the defect during inspiration and expiration movements . drainage chest tubes were inserted bilaterally via the 6th intercostal spaces to avoid leakage of the chemotherapeutic agents across repaired diaphragm and postoperative massive pleural effusion . the tissue around the congenital left - sided defect was excised circularly and the defect itself was repaired with interrupted nonabsorbable monofilament sutures . after complete cytoreduction had been achieved , hyperthermic intraperitoneal chemotherapy with doxorubicin 35 mg / m and paclitaxel 175 mg / m for 60 min at 42.5c was delivered . no leakage via drainage tubes from the thoracic cavity was noted during the procedure . during the surgery , the patient was managed by standard monitoring , which involved continuous monitoring of electrocardiography , invasive blood pressure , central venous pressure , pulse oximetry , body temperature and hourly urine output . arterial samples were drawn at regular periods for the determination of electrolytes , acid base status and hematocrit . finally , the operation lasted 4 h. at the end of the surgery , the patient remained intubated and was transferred to the intensive care unit for monitoring ongoing resuscitation along with fluid and electrolyte management . the patient was extubated 10 h after completion of surgery , and was transferred to the service on the 2nd postoperative day . prophylactic enoxaparin 4,000 iu was started 6 h after surgery . on the 3rd and 4th postoperative day , excessive sleepiness throughout the day and dyspraxia was noted . bilateral anterior thalamic infarction caused by emboli that block the thalamosubthalamic artery the dose of enoxaparin was changed from 4,000 iu once a day to 800 iu twice a day . follow - up at 4 months after surgery showed no evidence of recurrence and no symptom regarding thalamic infarct . cbh generally presents in newborns with severe respiratory failure or in infancy due to herniation and/or strangulation of the abdominal contents . one of the best studies concerning the incidence of the defect in adults was carried out by mullins et al . , who reviewed 13,138 ct scans . their study revealed an incidence of 0.17% , with 68% being right - sided defects and 77% of the patients being female . since the second study summarized symptomatic cases , demographics identified were different than those in asymptomatic ones . this difference showed that more cases of right - sided cbh in females may remain silent than left - sided cases . these data were supported by several case reports . nevertheless , in our case , the defect was noted on the left side . also , no herniation of intestinal loops was noted , and the patient has never had any thoracic or abdominal symptom that might be related to diaphragmatic hernia . diagnosis of the disease might be carried out by chest ct when there are clinically unexplained thoracic and abdominal symptoms . the chest ct of our patient did not reveal any of the above - mentioned patterns . there are several cases concerning surgical repair of the defect both laparoscopic , via laparotomy , and through the thoracic cavity . we performed upper midline incision for laparotomy , and there was no need for another access to carry out the repair of the diaphragmatic defect . repair of diaphragmatic defect can be performed by various techniques , using absorbable or nonabsorbable sutures in an interrupted or continuous fashion . from our point of view , surgeons may choose any of them provided that they are comfortable with the procedure . the main reason for using bilateral chest tubes was to prevent massive pleural effusion due to hipec . in addition , intra - abdominal pressure increases during hipec , which may subsequently cause a cephalad shift of the diaphragm to increase peak airway pressures and reduce residual capacity . chest drains which were inserted prior to hipec reduce the increase in peak airway pressure , and by this way , the length of the intubation period and hospital stay can be decreased . malignancy itself , hipec , central venous cannulation , the position of the patient 's neck , sudden blood loss and a long duration of the operation were noted as possible risk factors in our case . in conclusion , although congenital bochdalek hernia commonly occurs in the newborn and infancy period , it must not be excluded in the case of unexplained abdominal and/or thoracic symptoms in adults . despite the fact that this kind of rare condition presents with pain , obstruction symptoms or pulmonary symptoms , it can be silent until discovered incidentally . secondly , cerebral embolism should be suspected in the presence of any neurological symptom in postoperative patients .

congenital bochdalek hernia is a defect of the diaphragm and very rare in adults . only around 100 cases have been reported in the literature . herein , we present a case with a recurrent ovarian cancer who underwent secondary cytoreductive surgery and hyperthermic intraperitoneal chemotherapy . an oval defect with dimensions of 3 4 cm was seen in the left posterolateral site of the diaphragm during surgical exploration . in addition , a 6 3 cm iatrogenic right - sided diaphragmatic defect was found and repaired . in the early postoperative period , a bilateral thalamic infarction occurred .

a 78-year old male with past medical history significant for chronic shortness of breath ( for the last two years ) , chronic systolic heart failure secondary to non - ischemic dilated cardiomyopathy , pulmonary hypertension , essential hypertension , coal miner s pneumoconiosis , and chronic obstructive lung disease was found to have bilateral pulmonary nodules on pre - operative chest x - ray . the work - up for the pulmonary nodules was initially planned on an outpatient basis . however , two weeks later the patient was admitted to the hospital with progressive shortness of breath . upon admission , vital signs included temperature 35.8c , pulse 56 beats / min , respiratory rate 22 breaths / min , and blood pressure 110/54 mmhg . on review of systems he reported decreased appetite for the past three months and a 20 pound weight loss . he denied hematuria , rhinorrhea , rashes , sinus pain , muscle aches , ear ache , hearing loss or hemoptysis . blood urea nitrogen was 18 mg / dl , creatinine 1.2 mg / dl , glomerular filtration rate 59 ml / min . chest x - ray revealed bilateral hilar and right upper lobe areas of infiltrate increased from the prior imaging examination . computed tomography ( ct ) scan revealed multiple cavitary lesions , the largest on the right , with a large air fluid level suspicious for infection . the day after admission the patient underwent ct guided fine needle aspiration of the right lung . all blood cultures were the negative and so were cultures of the pleural fluid and the surgical specimen . however , the antibiotics were soon discontinued secondary to low suspicion for the presence of active infection . at that point the diagnosis of vasculitis was entertained and a vasculitis panel was significant for elevated sedimentation rate at 84 mm / h . anti - glomerular basement membrane antibody level was negative , but proteinase 3-anti - neutrophil cytoplasmic antibody ( pr3-anca ) level was elevated at 3.8 units . stool studies were positive for clostridium difficile for which the patient was started on flagyl . repeat urinalysis showed brown colored urine with ph 6 , specific gravity 1.023 , moderate occult blood , bile negative , small leukocyte esterase , nitrate negative , 9 - 10 red blood cells ( rbc)/high power field ( hpf ) , 9 - 10 white blood cells / hpf , rare squamous epithelial cells , rare hyaline casts , 30a protein . kidney biopsy was inadequate for diagnosis . during the course of his treatment he also completed 5 cycles of plasmapheresis . wegener s granulomatosis ( wg ) is anca associated vasculitis of small and medium sized vessels . the disease has variable presentation and diagnosis is made on the basis of clinical , laboratory , pathologic and imaging studies . usually patients present with upper respiratory tract symptoms such as nasal ulcer , nasal discharge , rhinorrhea and sinus pain . kidney involvement manifests as acute renal failure with red cells , red cell casts and proteinuria . although wg is mainly characterized by respiratory disease and nephritis , it can also affect the nervous system , heart , eyes , skin , joints and spleen . the diagnostic criteria published by the american college of rheumatology include nasal or oral inflammation with ulcers or purulent bloody discharge ; chest radiograph showing nodules , cavities or infiltrates ; urinary sediment with red cell casts or microscopic hematuria ; granulomatous inflammation on lung biopsy . infection could serve as a trigger for disease expression and is an established factor for disease relapse . under the effect of unknown antigen , neutrophils express cytoplasmic pr3 , which leads to the production of anti - pr3 antibodies . laboratory studies include leukocytosis , elevated erythrocyte sedimentation rate , normocytic anemia , positive anca and rheumatoid factor . potential biopsy sites include affected areas such as nasal sinus , muscle , temporal artery , kidney , lung . kidney biopsies are done in patients with urinary sediment abnormalities , proteinuria or change in renal function . on histologic examination wg is characterized by vasculitis , inflammatory infiltration of medium and small vessels , and necrotizing granuloma formation . the limited form of wegener s usually does not affect the function of vital organs . the systemic form of wg is more common and only 25% of patients will have limited wg . some authors state that the limited form may evolve into systemic disease , implying that the two forms are different stages of progression of the same disease . they further state that the lack of progression in some of the cases is due to improved treatment modalities . c - anca is present in 50% of patients with the limited form and 80% of patients with the systemic form of the disease . initially our patient was found to have bilateral pulmonary nodules on preoperative chest x - ray . differential diagnosis at that time included more common conditions such as lung metastasis , tuberculosis , sarcoidosis , rheumatoid nodules , lung infection ( bacterial or fungal ) . the presence of vasculitic process was investigated after the above mentioned conditions were ruled out . the patient did not present with upper respiratory findings or findings indicative of kidney involvement . due to the absence of such findings he later developed diarrhea and was diagnosed with clostridium difficile infection . at that time the patient developed systemic manifestations of wg including renal failure , vasculitic rash and hemoptysis . the degree of kidney involvement could not be assessed secondary to failure to obtain satisfactory biopsy specimen . the infection could have triggered the evolvement of his limited wg into systemic disease , supporting the theory that those two forms are different stages of the same disease entity . the patient s condition rapidly transformed from a limited form to a systemic form of wg in the course of two weeks . this highlights the importance of timely treatment in patients with limited wg in order to prevent disease progression . when left untreated , wg is usually fatal . wg that does not involve the kidneys or other vital organs can be treated with less toxic regimens such as methotrexate or azathioprine . limited wg may progress to involve kidneys and other organs and hence should be treated and closely monitored . however , patients do not always present with the triad and a high index of clinical suspicion is needed in order to make a prompt diagnosis . when patients present with limited disease , without renal involvement , diagnosis is delayed and immunosuppressive treatment is not recommended . there are no clear guidelines on how limited wg should be approached , followed up , or treated . our case demonstrates that limited wg can evolve into systemic disease secondary to environmental triggers such as infection . this supports the notion that the two forms represent different stages of the same disease .

wegener s granulomatosis is a granulomatous vasculitis that can present with a wide spectrum of clinical manifestations . this disease entity predominantly affects the respiratory tract and the kidneys . two forms of wegener s granulomatosis have been recognized : systemic and limited . it has not been established if the two forms represent separate disease entities or different stages of the same condition . in the limited form of wegener s granulomatosis there is no immediate threat to the function of vital organs and there is no evidence of glomerulonephritis . environmental factors that could serve as triggers for the activation of wegener s granulomatosis have not been clearly defined . we report a case of a 78-year old male who was found to have bilateral pulmonary nodules on pre - operative chest x - ray and was diagnosed with the limited form of wegener s granulomatosis . the patient developed clostridium difficile infection , and shortly after that active glomerulonephritis , a manifestation of systemic wegener s granulomatosis .

to determine the prevalence and clinical characteristics of hbov , we investigated the presence of this virus in children with respiratory tract infection in our region ( gipuzkoa , basque country , spain ) . among the first patients in whom hbov was detected in nasopharyngeal aspirates , we found two 12-month - old children with diarrhea in addition to respiratory symptoms . because animal parvoviruses are frequently associated with enteritis in young animals ( 6 ) , we investigated the presence of hbov in the diarrheal feces of both children . hbov was detected in both samples , and no other intestinal pathogens were identified . to rule out the possibility that this result could have been due to fecal contamination resulting from swallowing respiratory secretions , and to determine whether the gastrointestinal tract is affected by this new respiratory virus , we studied its presence in patient feces in 527 episodes of acute gastroenteritis , unrelated to respiratory infection , in children <3 years of age , mainly from nonhospital centers ( ambulatory clinics ) . viral dna and rna were obtained from nasopharyngeal aspirates and stool specimens with an automatic extractor biorobot m48 ( qiagen , hilden , germany ) by using the magattract virus mini m48 kit ( qiagen ) . cdna was obtained by using m - mulv reverse transcriptase ( promega , madison , wi , usa ) and random primers . aliquots of the dna and cdna were frozen at 40c until pcr for hbov detection was performed . respiratory samples were investigated for respiratory syncytial virus , influenza viruses a and b , parainfluenza virus types 14 , and adenovirus by cell culture and pcr . rhinovirus , coronavirus ( nl63 coronavirus included ) , and metapneumovirus were studied by pcr alone . yersinia enterocolitica , campylobacter spp . , and enteroinvasive escherichia coli o157 by standard culture methods . hbov detection was performed by pcr with primers derived from the np1 gene ( 1 ) . positive samples were retested and confirmed as positive by using a second pcr assay with primers derived from another location in the hbov genome ( vp1 gene ) ( 7 ) . amplified np1 and vp1 gene fragments ( 354 bp and 403 bp , respectively ) were sequenced and analyzed by using the blast software ( www.ncbi.nlm.nih.gov/blast ) . each pcr run included a negative control ( water ) that was treated as the clinical sample throughout , and pcr was performed with the usual precautions to avoid contamination . of the 527 stool samples analyzed from december 2005 through march 2006 , hbov was detected in 48 ( 9.1% ) . from a second group of 520 children <3 years of age who came to the pediatric emergency unit of our hospital with an episode of acute respiratory infection during the same period , a similar frequency of hbov detection was obtained ( 40/520 , 7.7% ) when nasopharyngeal aspirates were tested . analysis of np1 and vp1 partial gene sequences obtained from all fecal and respiratory hbov - positive samples showed a similarity of > 95% with previously published hbov sequences . of 40 hbov - positive respiratory samples , 25 ( 62.5% ) showed coinfection with other viruses ( respiratory syncytial virus in 13 , rhinovirus in 3 , influenza a in 3 , coronavirus oc43 in 2 , adenovirus in 1 , influenza b in 1 , respiratory syncytial virus and coronavirus oc43 in 1 , and influenza a and rhinovirus in 1 ) . of the 48 hbov - positive fecal samples , 28 ( 58.3% ) showed coinfection with another intestinal pathogen ( salmonella enteritidis in 1 , campylobacter jejuni in 5 , rotavirus in 14 , norovirus in 7 , and c. jejuni and norovirus in 1 ) . in this study , simultaneous detection of hbov and the incidence of coinfection in respiratory illness was similar to that observed in studies that were not limited to specimens that had already tested negative for other microorganisms and in which a wide number of agents were investigated ( 4 ) . adenoviruses have been associated with infection of the colon and the gut and are a cause of severe gastroenteritis in nonindustrialized countries . in this study , coinfection of adenovirus and hbov was detected in 1 respiratory specimen but these viruses together were not detected in any fecal sample . hbov and parvovirus b19 are the only 2 species of the parvoviridae family that have been associated with disease in humans . to date , hbov has only been detected in samples from the respiratory tract and has been associated with both upper and lower respiratory tract disease in infants and young children . the results of our study show that hbov is also present in the gastrointestinal tract in children with gastroenteritis with or without symptoms of respiratory infection . the fecal excretion adds new concern about the transmission of hbov . to our knowledge , this report is the first to document hbov in human feces . the high frequency of hbov detection in the feces of children with gastroenteritis and the absence of any other intestinal pathogen suggest that this new virus species is an enteric , as well as a respiratory , pathogen . further investigations to confirm this preliminary hypothesis and gain greater knowledge of the association between hbov and enteric disease are required .

in spain , human bocavirus ( hbov ) was detected in 48 ( 9.1% ) of 527 children with gastroenteritis at similar frequency as for children with respiratory illness ( 40/520 , 7.7% ) . fecal excretion adds new concern about the transmission of hbov . to our knowledge , this report is the first to document hbov in human feces .

a 45-year - old man with a lifelong history of atopic dermatitis had a year - long unremitting exacerbation for which he had started systemic therapy . after treatment with cyclosporine for several weeks , laboratory abnormalities and nonspecific neurologic signs prompted a switch to methotrexate . within 4 weeks , he was hospitalized ( in an overseas us military hospital ) for generalized umbilicated papulopustules accompanied by profound hypothermia , hypotension , and mental status changes . he had large pustules on his trunk , inner thighs , and upper extremities ( figure 1 , panel a ) . he was transferred to our intensive care unit with widespread umbilicated pustules and normal mental status . the pustules were deep seated , monomorphic , dome shaped , and firm and were distributed densely on the patient s forearms and abdomen ( figure 1 , panels b and c ) . clinical photographs of the patient . a ) patient with generalized pustules , which were deep seated , monomorphic , dome shaped , and firm and were distributed densely on forearms and abdomen . c ) umbilicated papulopustules in the same stage of evolution ; no herpetiform clusters or red areolae are seen around the lesions . at our hospital , his oral temperature fluctuated dramatically , from 89.3f to 101.3f , with rectal confirmation < 95f ( < 35c ) , indicating hypothermia ( 5 ) . we performed a tzanck preparation , which showed multinucleated giant keratinocytes with nuclear molding and margination ( appendix figure ) . a direct fluorescent antibody ( dfa ) test was positive for varicella zoster virus ( vzv ) . a biopsy specimen showed epithelial necrosis with cellular ballooning and multinucleated giant cells , plus intranuclear inclusion bodies ( figure 2 , panels a and b ) . subsequently , special immunohistochemical stains were positive for herpes simplex virus ( hsv ) ( figure 2 , panel c ) , and a viral culture grew hsv type 2 . his illness was diagnosed with disseminated hsv concurrent with underlying atopic dermatitis ( i.e. , eczema herpeticum ) . c ) positive immunohistochemical stain for herpes simplex virus . within minutes of the tzanck smear evaluation , our patient was given intravenous acyclovir . when cutaneous improvement was evident , he was switched to oral valacyclovir . within days , his skin lesions largely resolved without conspicuous crusting or scarring , but he remained intermittently hypothermic for several weeks . this patient was markedly ill on admission and had a distinctive varioliform eruption with lesions in a uniform stage of evolution . tzanck preparation promptly confirmed herpetic etiology , but we nevertheless used cdc s algorithm for evaluating agvpri , and our patient s illness stratified to high risk . cdc has 3 major diagnostic criteria to designate a case as high risk for smallpox ( table ) ( 6 ) . the first is febrile prodrome , which typically lasts 14 days before cutaneous lesions appear and must include > 1 of the following : prostration , headache , backache , chills , vomiting , or severe abdominal pain . although our patient s illness eventually met the fever criterion , his 101f temperature occurred only after he began antiviral treatment . prolonged hypothermia is associated with severe illness ( 7 ) and is equivalent to fever in determining critical illness ( 8) , which we believe satisfies cdc s first major criterion . the second criterion requires classic cutaneous lesions that are deep seated , firm , round , well - circumscribed vesicles or pustules that may become umbilicated or confluent . the third criterion requires the same stage for most cutaneous lesions on an affected area . our patient s illness met all 3 criteria ; however , laboratory tests confirmed herpesvirus infection . smallpox was declared eradicated by the world health organization in 1977 ; nevertheless , some health organizations consider this illness a bioterrorism threat . clinical smallpox typically starts with a prodrome of high fever , headache , myalgia , backache , nausea , vomiting , and diarrhea . an oropharyngeal enanthem is followed by cutaneous eruption of erythematous macules that quickly become papules . the papules evolve over days into vesicles and then pustules , often developing central umbilication . classic smallpox lesions occur in the same stage of evolution on a body segment , which differentiates it from varicella . smallpox pustules have been called pearls of pus to help distinguish them from the more delicate histopathologically , cutaneous smallpox lesions may resemble herpetic lesions except that smallpox has intracytoplasmic inclusions ( guarnieri bodies ) instead of intranuclear inclusions ( lipschutz bodies ) of herpetic lesions . also , multinucleated giant keratinocytes are uncharacteristic of smallpox ( 9 ) . eczema herpeticum , described by kaposi in 1887 , is most common in patients with atopic dermatitis but can occur in other conditions that disrupt epidermal integrity . in eczema herpeticum , lesions are typically monomorphic vesicles that evolve into pustules ( 10 ) . fever , malaise , lymphadenopathy , and tender skin may accompany cutaneous eruption ( 11 ) . fever is a well - recognized sign of infection ; however , hypothermia can also signal serious disease , including bacterial sepsis or viral encephalitis ( 12 ) , and may be more dire than fever in severely ill hospitalized patients ( 13 ) . we propose that our patient s hypothermic temperature dysregulation is equivalent to fever , thus serving as a major diagnostic criterion . when a patient with agvpri is evaluated for possible smallpox , rapid laboratory tests are necessary . viral culture does not yield results quickly enough to avert infection control measures expected with a smallpox case . indeed , cdc reports 7 incidents when patients with agvpri prompted emergency department diversions or hospital closures ( 1 ) . also , rapid confirmation of nonvariola etiology can help avert public panic , a potential problem in a suspected smallpox outbreak and a probable intended consequence of a terrorist attack . the tzanck smear must be performed by someone experienced in using the technique and interpreted by someone who can confidently and correctly distinguish herpesvivus nuclear inclusions from poxvirus cytoplasmic inclusions . dfa for hsv and vzv is relatively rapid , but in our case , the dfa result was positive for vzv , although viral culture and immunohistochemical staining later showed that the patient s infection was due to hsv-2 . had we been unable to confirm a nonvariola etiology , we would have proceeded to poxvirus testing . with no commercially available tests for smallpox , the algorithm advises close coordination among local , state , and federal public health authorities . some state and federal reference laboratories can provide confirmatory tests , including pcr , for orthopoxviruses such as smallpox and monkeypox . although not performed in this case , we recommend such testing if a simultaneous infection with an orthopoxvirus can not be ruled out .

eczema herpeticum can clinically resemble smallpox . on the basis of the algorithm for rapid evaluation of patients with an acute generalized vesiculopustular rash illness , our patient met criteria for high risk for smallpox . the tzanck preparation was critical for rapid diagnosis of herpetic infection and exclusion of smallpox .

mauriac syndrome is a rare syndrome associated with type 1 diabetes ( t1 dm ) in children . the incidence of this syndrome had decreased significantly with introduction of long - acting insulin and better control of blood sugar . recently , there has been re - emergence of this syndrome , especially with the use of premix insulin . a 15-year - old type 1 diabetic boy was referred to us for evaluation of short stature . he was diagnosed to have t1 dm , following an episode of diabetic ketoacidosis ( dka ) 8 years back , and was started on premix ( 30/70 ) insulin . examination showed that he was significantly short for his age , height 131 cms ( less than 3 percentile ) , weight 28 kg ( less than 3 percentile ) and body mass index of 16.31 . he had abdominal fat deposition [ figures 1 and 2 ] and liver was palpable clinically 5 cms below costal margin [ figure 3 ] with no splenomegaly or free - fluid . short stature , abdominal obesity short stature , abdominal obesity investigations showed haemoglobin of 13.5 gm / dl . ultrasound abdomen showed liver enlargement 15.5 cms with increased echotexture , with normal spleen and portal vein and no free - fluid . on evaluation for diabetic status , he had an hba1c of 10.3% , urine microalbumin 70 g / gm of creatinine , and fundus showing background diabetic retinopathy . he had a bone age of 10.2 years , ( tanner whitehouse 2 ) thyroid - stimulating hormone ( tsh ) of 2.4 miu / ml and t4 of 8.8 microgm / dl . his growth hormone ( gh ) stimulation test and overnight dexa methasone suppression test were normal ( after blood glucose control and testosterone priming ) . based on the clinical history and investigations , the final diagnosis of mauriac syndrome was made and the patient was advised tight control of sugars . he was switched over to basal bolus regime , with glargine in the night and three doses of short - acting insulin before meals . mauriac in 1930 , described growth failure and maturational delay with hepatomegaly and abdominal distension in children with t1 dm , who were treated with short - acting insulin . hepatomegaly was commonly observed in children in the earlier periods of diabetic treatment , when only short - acting insulin was available and aglycosuria was the objective of treatment . it was noticed that the hepatomegaly regressed when the children were given the newly introduced protamine zinc insulin , providing better sugar control . in the late 1930s joslin clinic reported a case series of 60 youngsters with hepatomegaly , growth failure , delayed sexual maturation , and severe uncontrollable diabetes . equal incidence is reported in males and females , with most of the cases occurring during adolescence . with better control of sugar , the incidence of this syndrome has reduced rapidly and in the current era this is a very rare syndrome . two different forms of mauriac syndrome have been described , based on the presence or absence of obesity . in first form , as classically described , treatment with regular insulin alone there is associated cushingoid obesity and documented - wide fluctuation between hyperglycemia and hypoglycemia , suggestive of a pattern of over - and under - insulinization , with secondary hyperadrenalism . periods of over - insulinization appear to be essential for the development of obesity , and for the induction of hyperadrenalism . recently , mauriac syndrome has been reported in patients who are not obese and are without a history of alternating hypoglycemia and ketoacidosis . this occurs in patients who have been given regular , under the dose insulin . inadequate glucose to the tissues , decreased insulin - like growth factor-1 and gh levels , hypercortisolism , resistant or defective hormone receptor action contribute to stunted growth and delay in puberty . the cause of hepatomegaly is thought to be due to the deposition of glycogen in the liver , and similar subcutaneous deposition gives rise to the round moon like facies . growth failure , delayed puberty and hepatomegaly in mauriac 's syndrome improves with glycemic control . aggressive glycemic control has been associated with worsening of retinopathy , which should be monitored . mauriac syndrome is a rare manifestation of poorly treated t1 dm . with aggressive glycemic control ,

mauriac syndrome is a rare syndrome associated with type 1 diabetes ( t1 dm ) in children presenting with growth retardation , hepatomegaly , and cushingoid features . recently , there has been re - emergence of this syndrome , especially with the use of premix insulin . a 15-year old type 1 diabetic boy , who was on premix insulin with erratic blood glucose , was referred to us for evaluation of short stature . he had significant short stature , hepatomegaly , and cushingoid features . his growth hormone ( gh ) stimulation was normal , and so was the overnight dexamethasone suppression test , based on which the diagnosis of mauriac syndrome was reported . he was made to switch over to basal bolus regime , and was advised to follow - up for 6 months . he had reduction in hepatomegaly and a height gain of 3 cms .

ultrasound ( eus)-guided celiac plexus neurolysis ( cpn ) has been reported to be effective in inducing pain relief . however , a 75-year - old man suffering from opioid refractory pain ( recorded as 9 according to visual analog scale ( vas ) ) due to an advanced stage iv histologically - proven pancreatic adenocarcinoma with liver metastases and celiac trunk infiltration not suitable for surgery , was referred to our center . eus confirmed the nodular hypoechoic lesion of 40 38 33 mm in the pancreas [ figure 1 ] . as the tumor stage was beyond any possibility of surgical radical care and in order to relieve patient 's symptoms , after informed consent was obtained , eus - guided cpn combined to eus - guided tumor ablation was arranged . under sedation with propofol , eus was conducted with a pentax fg-36ua ultrasound endoscope ( pentax europe ltd , hamburg , germany ) using a curved - array transducer . a 22 g needle ( cook medical inc , bloomington , indiana , usa ) was introduced though the endoscope 's working channel to inject 10 ml 2.0% lidocaine and 20 ml of ethylic alcohol 95% into the base of the celiac trunk at its origin from the aorta [ central approach , figure 2 ] . after performing cpn , 40 ml 75% of lesion volume of ethanol ( concentration 95% ) were directly injected into the tumor [ figure 3 ] . tumoral nodule in the body of the pancreas celiac plexus neurolysis performed via the central approach ethanol ablation of the pancreatic tumor no major complications were recorded during the procedure . in the days following the procedure , mild ( grade 2 ) diarrhea and fever were the only minor complications experienced by the patient . complete tumor devascularization was assessed by means of computed tomography ( ct ) 48 h after the procedure [ figure 4 ] . ca 19 - 9 dropped down to 56.84 u / ml at 2 weeks after eus . tumor response assessed by means of computed tomography ( ct ) 48 h after the procedure complete pain relief , namely vas 0 , was achieved 3 days after the endoscopic ablation . the patient remained pain - free without need of opioid , and was treated only with paracetamol for 20 weeks and afterwards experienced pain relief , ( defined as pain within 30% of baseline ) until death occurred 30 weeks after the procedure . advanced pancreatic cancer commonly induces severe refractory pain . in such patients , where opioids result often ineffective , eus - guided cpn has been reported to induce pain relief in 70 - 80% of cases . however , most patients achieve only suboptimal and transient relief , probably due to technical failure or further nociceptive impulses which can not be interrupted by the neurolysis . furthermore , definitive data on its efficacy in improving survival is still lacking . therefore , it was decided to try a combined approach directed both to the celiac plexus and the neoplastic mass , in order to delay tumor progression and lengthen pain - free survival as well as overall survival . the results were optimal in terms of pain relief and immediate tumor response ( assessed by means of ct and tumor marker levels ) . the present case demonstrates that the combined approach ( eus - guided ethanol ablation and cpn ) may be a valuable option aimed at improving both prognosis and pain control in patients with pancreatic cancer . randomized - controlled trials comparing eus - cpn alone to cpn associated to tumor ablation are needed to confirm this result .

a 75-year - old man suffering from opioid - refractory due to an advanced pancreatic adenocarcinoma was treated with endoscopic ultrasound ( eus)-guided celiac plexus neurolysis ( cpn ) combined to eus - guided tumor ablation . no major complications were recorded during the procedure . in the days following the procedure , mild diarrhea and fever were the only minor complications experienced by the patient . complete tumor devascularization was assessed by means of computed tomography ( ct ) 48 h after the procedure . the patient remained pain - free without need of opioid , and was treated only with paracetamol for 20 weeks . our results were optimal in terms of pain relief and immediate tumor response ( assessed by means of ct and tumor marker levels ) . the present case demonstrates that the combined approach ( eus - guided ethanol ablation and cpn ) may be a valuable option in patients with pancreatic cancer . randomized - controlled trials are needed to confirm this result .

most of the children with cerebral venous thrombosis have an underlying chronic disease and those who are previously well have an acute infectious illness . obstructive hydrocephalus as a complication of cerebral venous thrombosis is rare and few cases are described . a previously healthy 4-year - old boy presented with 5 days of headache and vomiting . magnetic resonance imaging of the patient revealed obstructive hydrocephalus and magnetic resonance venography revealed thrombosis of the right transverse sinus [ figure 1 ] . low molecular weight heparin was started at a dosage of 1 mg / kg twice a day . control magnetic resonance imaging and magnetic resonance venography 1 month after the first event revealed partial resolution of hydrocephalus along with recanalization of the affected sinus [ figure 2 ] . hydrocephalus and absent flow in the right transverse sinus showing acute thrombosis improvement of hydrocephalus and recanalization of the affected sinus after a month of heparin therapy with folic acid and vitamin b6 and b12 replacement , the follow - up of the patient was given up by the family and he did not receive any treatment for 5 months . six months after the first event , the patient was again admitted to hospital with a similar clinical picture along with new thrombosis at the sagittal sinus . the patient is still followed up with low molecular weight heparin treatment after the second thrombosis attack . cerebral venous system thrombosis is an unusual event in childhood and clinical presentations , complications , diagnostic procedures , etiological investigations , and treatment modalities were not well established . clinical findings of cerebral venous system thrombosis include seizures , headache , vomiting , drowsiness , lethargy , and confusion . the patient may show acute or subacute picture related to progression of the disease . the present case presented with acute symptoms secondary to increased intracranial pressure . increased intracranial pressure is a well - known complication of cerebral venous thrombosis , but accompanying hydrocephalus is rarely described . the mechanism of hydrocephalus is considered to be secondary to an increase in venous pressure resulting in the blockade of cerebrospinal fluid flow into the sinuses by arachnoid villi . most of the patients with cerebral venous thrombosis have an increased intracranial pressure without an increase in ventricular size . why some patients develop hydrocephalus is not clearly understood and only a few adult cases were described . the etiologies regarding cerebral venous thrombosis are diverse and include head and neck infections and chronic disorders such as collagen tissue disorders , cardiac disease , and hematological abnormalities . half of the patients have prothrombotic states which include anticardiolipin antibodies , deficiencies of protein c and s , antithrombin iii and prothrombotic gene mutations such as prothrombin 20210 , factor v leiden , and mthfr mutations . the most frequent two common mthfr polymorphisms are c677 t ( alanin to valin transition ) and a1298c ( glutamine to alanine transition ) . evaluation of nine case control studies with total 382 patients having cerebral venous thrombosis revealed that carrying mthfr was not a risk factor for cerebral venous thrombosis . on the contrary a canadian study revealed that 29% of patients with cerebral venous thrombosis had mthfr polymorphisms . our case had a prothrombotic state with mthfr 1298 homozygous polymorphism without any risk factors . occurrence of clinical findings after cessation of heparin treatment suggests that mthfr polymorphism may be an important risk factor for cerebral venous thrombosis . anticoagulation is not recommended for asymptomatic and hemorrhagic cases . when used , low molecular weight heparin is the standard anticoagulant treatment in children . treatment may last for 36 months or it may be continued until recanalization if risk factors are eliminated . our patient responded well to low molecular weight heparin with recanalization of sinuses and recovery of hydrocephalus in both the attacks . in conclusion mthfr 1298 polymorphism may be an important risk factor and low molecular weight heparin should be the choice of treatment in cases without hemorrhage .

cerebral venous thrombosis is a rare form of stroke in childhood . increased intracranial pressure is a well - defined complication of cerebral venous thrombosis but obstructive hydrocephalus as a presentation finding of cerebral venous thrombosis is rarely described . a child case of cerebral sinus thrombosis presenting with obstructive hydrocephalus and management of clinical condition is presented with discussion of reported cases and treatment recommendations .

although global gene content and detailed sequence similarity comparisons with the previously described megaviridae members clearly classify pgv within the family ( including the partially sequenced organic lake phycodnaviruses ( olpv ) , chrysochromulina erecina virus , and phaoecystis pouchetti virus ) , the finding of a pgv - associated virophage came as a surprise . complete virophage genomes have now been described , but only 3 correspond to identified and isolated the pgv virophage ( pgvv ) is the third , and presumably not the last , of a rapidly growing series . except for their genome size in the 20 kb range , these new types of satellites viruses have little in common in terms of gene content , although they all code for a major capsid protein and one dna primase . consistent with the fact that it was never observed in pgv - infected cultures , pgvv is the first example of a virophage lacking the information to make a capsid protein , the essential building block of a virus particle . once multiplied in the pgv virus factory , the pgvv genome is thus packaged ( in multiple copies ) alongside the pgv genome , and propagated through the pgv virion , either as an integrated or free viral plasmid . the precise molecular structure(s ) the finding of a virophage associated to pgv is already teaching us the important lesson that virophages are not solely associated to dna viruses with micron - sized particles and 1 mb genome sizes , but can be found with large dna viruses of more reasonable ( poxvirus - like ? ) proportion . it is thus likely that many of such associations have been overlooked in the past , and that virophages might have played a fundamental role in the evolution of many more viruses than just the megaviridae . if this is true , they could be ( and have been ) the main vehicle of gene transfers between eukaryotic viruses , and indirectly between these viruses and their hosts . they may also be responsible for the sporadic occurrences of mobiles elements such as self - splicing introns , inteins , and transpovirons . another lesson is that , like all parasites , the virophages are submitted to the irreversible phenomenon of reductive evolution , condemning them to disappear as individual biological entities , eventually saving some of their genes by integrating them into the genome of their companion virus , themselves undergoing a similar process vis - - vis their cellular host . the first published phylogenetic tree including mimivirus 2 and using a concatenation of 7 universally conserved protein sequences , already pointed out that : we could now build a tentative tree of life , within which mimivirus appears to define a new branch distinct from the three other domains . elsewhere in the same article , mimivirus was also shown to be part of the broad family of the nucleocytoplasmic large dna viruses ( ncldv ) , branching near the middle of the previously defined iridovirus , phycodnaviruses , poxviruses , and asfarviruses lineages . put together , these 2 results suggested in a subliminal way that all the large dna viruses were in fact defining a domain distinct from the 3 established cellular domains . as additional genomes of megaviridae became available , molecular phylogenies computed with an increasing number of universal proteins associated to basic functions ( dna clamp loaders , ribonucleotide reductases , aminoacyl - trna synthetases , dna polymerases ) kept clustering the megaviruses in their own clade , clearly separate from the cellular domains , thus contributing an additional domain rooted in between archaea and eukarya in the tree of life . despite receiving increasing support from various authors , others remain strongly opposed to this view . in agreement with the notion of a fourth domain of life anchored by the largest dna viruses , the dna polymerase of pgv nicely clusters with the other megaviridae homologs , exhibiting no affinity with any of the main cellular lineages ( fig . 1 ) . based on the presence of a vestigial protein translation system , i previously argued that the genome of today 's giant viruses derived from an ancestral cellular organism through the irreversible process of reductive evolution experienced by all parasites . dna viruses exhibiting a whole range of genomic complexity could have been generated through this continuous process : viruses would have lost translation first ( the ribosome ) , then transcription ( the rna polymerase ) , then dna replication ( the dna polymerase ) , following an evolutionary scenario whereby they become increasingly dependent from their host . the vast range of size and complexity among today 's dna viruses might thus be the results of differences in their evolution rates ( the largest ones experiencing the least evolutionary pressure ) . in this context , the lack of affinity of the giant viruses with any of today 's cellular domains , as well as the huge proportion of the viral genes without cellular homolog , would suggest that the lineage of the ancestral cellular organism that gave rise to giant viruses became extinct as a cellular life form , and only managed to survive as a parasitic fourth domain . the recently described giant pandoravirus might represent an independent instance of the same scenario . the tree was produced using the default option on the mafft server ( url : mafft.cbrc.jp ) from the multiple alignment of 25 dna polymerase b sequences ( 510 ungapped positions , excluding the inteins ) . branches with bootstrap values < 80 are collapsed . despite infecting eukaryotic hosts from vastly divergent phyla , the viruses do not show any phylogenetic affinity with a specific eukaryotic group , and cluster ( in red ) separately from the 3 cellular domains : eukarya ( green ) , archaea ( purple ) , and eubacteria ( blue ) . this strongly supported topology suggests that the common ancestor of the megaviridaelargely predated the radiation of the eukaryotes .

the viral nature of the first giant virus , mimivirus , was realized in 2003 , 10 y after its initial isolation from the water of a cooling tower in bradford , uk . soon after its genome was sequenced , the mining of the global ocean sampling environmental sequence database revealed that the closest relatives of mimivirus , only known to infect acanthamoeba , were to be found in the sea . these predicted marine mimivirus relatives remained elusive until 2010 , with the first genomic characterization of a virus infecting a heterotrophic unicellular eukaryote , the microflagellate grazer cafeteria roenbergensis . the genome analysis of a virus ( pgv ) infecting the common unicellular algae phaeocystis globosa now shows that it is a bona fide member of the mimivirus family ( i.e. , the megaviridae ) , extending the realm of these giant viruses to abundant blooming phytoplankton species . despite its smaller genome size ( 460 kb encoding 434 proteins ) , pgv exhibits the most intriguing feature of the previously characterized megaviridae : an associated virophage . however , the 19-kb virophage genome , devoid of a capsid gene , is packaged in the pgv particle and propagated as a viral plasmid , the first ever described . the pgv genome also exhibits the duplication of core genes , normally present as single copies and a putative new type of mobile element . in a dna polymerase phylogeny including representatives of the three cellular domains , pgv and the other megaviridae cluster into their own clade deeply branching between domains archaea and eukarya domains , thus exhibiting the topology of a fourth domain in the tree of life .

a 70-year - old man was admitted to the emergency department with a 1-month history of coughing and dyspnea . he developed a productive cough with purulent sputum , left chest wall pain , and 3 days prior to arrival ecchymosis developed on the left upper quadrant of the abdomen . he did not have a recent history of thoracic or abdominal trauma , and it was unknown if he had a history of thoracic surgery . the initial chest x - ray showed a hyperdensity in the left lower lung , an obliterated diaphragmatic border with an atelectasis , and a decreased lung volume . the scan film showed a protrusion of the lung through the left 7th intercostal space ( fig . therefore , he was diagnosed with a lung herniation , and a medical conservative treatment was performed . on hospital day 10 , after experiencing uncontrolled coughing , the patient complained of aggravated dyspnea and left chest wall pain . the subsequent chest x - ray showed increased hyperdensity with a gas - filled area above the left dome of the diaphragm . he also showed dullness to percussion and chest wall tenderness . on hospital day 12 , a ct scan with contrast of the chest revealed herniation of the bowl and omental fat in the anterior portion of the left hemithorax ( fig . , the patient was transferred to the department of thoracic surgery and underwent an emergency operation due to the incarceration of the bowl and a parapneumonic effusion due to passive atelectasis . the operation revealed a partial agenesis of intercostal muscle , costal cartilage around the 7th anterolateral intercostal space due to the lack of developed intercostal muscle ( fig . 2a ) , an 8 cm defect of the diaphragm , and a herniation of the small bowel located in the anterior portion of the left thoracic cavity ( fig . the primary repair of the diaphragm was performed and the direct approximation of the 7th intercostal space was determined . a lung herniation is defined as a protrusion of the lung beyond the normal confines of the thoracic cavity through an abnormal opening in the chest wall . a condition associated with increased intra - thoracic pressure or that weakens the thoracic wall may cause a lung herniation . according to a report by goverde et al . , lung herniation is such a rare disease , that only about 300 patients have been reported with the disease in the world literature , and most were single case reports . congenital lung hernias are especially rare and consist of approximately 18% of all reported lung hernias . most congenital lung hernias are found in the supraclavicular area , whereas a lung herniation in the intercostal area is rare . a morgagni 's hernia is caused by a failure of fusion between the fibrotendinous portions of the sternal and the costal parts of the diaphragm . a morgagni 's hernia is also a rare disease . in a report by berman et al . , only 15 infants and children with morgagni 's hernia were admitted over a 20 year period at the hospital for sick children in toronto . the majority of patients present with a morgagni 's hernia in the neonatal period , but the hernia may remain undiscovered until later in life . morgagni 's hernia is secondary to an incomplete development of the diaphragm in contributing to the increase of abdominal pressure ; other contributing factors include trauma , severe exertion , and obesity . we located the agenesis of the intercostal muscle and the costal cartilage , and the congenital morgagni 's hernia was located near the intercostal herniation . the discoveries serve as evidence that this case involved a congenital disorder . in conclusion , we believe that the cause of the intercostal hernia combined with the morgagni 's hernia might have been due to incomplete development of the chest wall and diaphragm . the increased abdominal pressure due to recurrent cough is what aggravated the symptoms .

a 70-year - old male visited urgent care due to coughing for 1 month and left chest pain . he had no history of trauma . the initial chest computed tomography ( ct ) showed the 7th left intercostal lung herniation . a follow - up ct showed an intercostal lung herniation combined with a bowl herniation , which had developed due to a morgagni 's hernia . an emergency operation was performed due to the incarceration of the bowl and lung . the primary repair of the diaphragm was performed and the direct approximation of the 7th intercostal space was determined . we concluded that the defect of the diaphragm and the intercostal muscle was a congenital lesion , and the recurrent coughing was the aggravating factor of herniation .

it is widely believed that the rate of mortality associated with anesthesia is steadily decreasing , and that anesthesia today is very safe indeed . however , this assertion has been questioned by lagasse , who argues that the definitions of anesthetic mortality used in different studies over the years have not been consistent . perhaps the best data on anesthesia mortality come from australia , where the state - based anesthetic mortality review committees have followed a relatively consistent approach with clear definitions ( table 1 ) for 20 years . case mix clearly makes a substantial difference to the risk of anesthesia , but the sequential triennial reports of these committees do suggest that anesthesia has become safer over time and that the risk of death definitely and entirely attributable to anesthesia today is close to 1 in 200,000 in patients who are essentially healthy and undergoing minor or moderate surgical procedures . it is disappointing , however , that even these data relate primarily to deaths within either 24 or 48 hours of anesthesia , whereas most people would probably be more interested in knowing their chances of actually going home from hospital and surviving for a reasonable period thereafter ( 30 days post - surgery , for example ) . there is very little information on this , although at least one european study suggests that these risks may be much higher than generally appreciated . this european study also confirms earlier evidence suggesting that anesthesia makes an important contribution to outcome after surgery . fundamental to any discussion of the safety of anesthesia is the matter of when mortality should be attributed to anesthesia . the australian mortality committees have provided definitions by which perioperative deaths can be attributed to one of eight categories , three of which relate to anesthesia ( table 1 ) . the critical words are : caused by the anesthesia or other factors under the control of the anesthetist . it is these other factors that have largely been overlooked in efforts to estimate the true incidence of mortality ( let alone morbidity ) attributable to anesthesia . perioperative myocardial ischemia is a case in point : clearly it may be attributable or amenable to factors under the control of the anesthetist ; its consequences often manifest several days postoperatively so are easily overlooked , particularly in studies that focus on the first 24 or 48 hours after surgery ; and it may contribute to death , although often only some time later . in a study by the study of perioperative ischemia research group , patients surviving a postoperative in - hospital myocardial infarction had a 28-fold increase in the rate of subsequent cardiac complications within 6 months following surgery , a 15-fold increase within 1 year , and a 14-fold increase within 2 years . are these extra deaths attributable to anesthesia ? we would argue that they are . in the perioperative ischemic evaluation ( poise ) study , a multicentre prospective blinded controlled trial involving 8,351 patients , the incidence of perioperative myocardial infarction in patients randomized to receive metoprolol was 4.2% compared with 5.7% in those receiving placebo ( confirming the value of beta - blockers in protecting the heart ) , but the overall mortality was 3.1% and 2.3% , respectively . in other words , the perioperative use of beta - adrenergic blocking drugs , a factor very much under the control of anesthetists , was associated with an excess mortality rate of 0.8% . the wider question of how and when to use beta - blockers during anesthesia , and of the other factors relevant to optimal management , is of course complex , which is exactly why anesthetists are rebranding themselves as experts in perioperative medicine . however , one can not have it both ways : the fact that the overall management of patients perioperatively can make a substantial difference to outcome is strong endorsement of the argument for a central role for well - resourced medically qualified practitioners in the provision of anesthesia , but it also makes a nonsense out of blanket claims that the risk of dying from an anesthetic is very low . there are many parts of the world in which anesthesia mortality rates are many multiples of those in high income countries such as the usa , europe , and australia [ 11 - 13 ] . clearly , many advances that have been made in the safety of anesthesia have not been realized everywhere . even worse , many patients do not receive desperately needed surgical and anesthetic services at all . the number of surgical operations undertaken around the world every year has only recently been estimated , and , at about 234 million , actually exceeds the number of births . unfortunately , these operations are very unevenly distributed , with only 3.5% being undertaken amongst those 34.8% of the global population who live in countries spending $ 100 or less per person on healthcare . it seems that about 11% of the global burden of disease measured in disability - adjusted life years arises from conditions amenable to surgery , so access to appropriate surgery is clearly essential , and obviously depends on equal access to safe anesthesia . even in high income countries there is substantial variance in access to services : more disturbingly , there is astonishing variance in practices over and above the variance attributable to resource differences . furthermore , there is ongoing evidence that even those patients who actually do receive appropriate healthcare ( including surgery and anesthesia ) are at unacceptable risk of harm from avoidable errors [ 17 - 20 ] . the safe surgery saves lives initiative of the world health organization has developed a surgical safety checklist to address some of these errors through a cost - effective tool applicable to surgery and anesthesia everywhere . in a study undertaken in eight pilot sites around the world , introduction of this checklist significantly and substantially reduced harm associated with surgery . the pilot study was not a randomized controlled trial , but it was prospective and large ( data from almost 4,000 patients collected at baseline were compared with data from a similar number after the introduction of the checklist ) . it will not eliminate errors , but it is highly likely to reduce them and , through enhanced teamwork , to improve outcomes more generally . there is now a considerable onus on senior clinicians to promote the use of the checklist in a meaningful manner involving the engaged participation of all members of the operative team . there is also considerable onus on the organizations associated with anesthesia to continue to support initiatives to improve the training and resources available for anesthesia in resource - limited regions of the world . the world federation of societies of anaesthesiologists has provided strong leadership in this regard and in recent years the support for anesthesia from the world health organization has increased considerably . as with so many things , the sophistication and accuracy of the way in which we measure outcome has increased in parallel with other advances in anesthesia . this has the effect of highlighting risks of anesthesia that , in previous years , may not have been appreciated at all . so is anesthesia becoming safer ? certainly - and this is self evident to anyone who has practiced for more than three decades . certainly not , especially in higher - risk patients , and in low - income regions of the world . we are making commendable progress , but there is a long way to go to achieve the goal that no patient shall be harmed by anaesthesia . the authors have financial interests in safer sleep llc , which produces a system to promote safety in anesthesia .

in well - resourced areas of the world anesthesia has become safer over the past decades , and anesthesia mortality does seem to be reducing . however , there is a lack of international agreement over definitions of anesthetic mortality and , therefore , difficulty in knowing exactly what the rate of anesthetic mortality is . avoidable harm from error is still a problem , and sophisticated analysis suggests that more deaths than generally appreciated may be attributable to factors under the control of anesthetists . mortality rates in low income areas of the world are unacceptably high . there is more to be done if anesthesia is to become truly safe for all patients .

psoriasis is a chronic immunologically - based inflammatory skin disease which affects approximately 2% of the general population [ 1 , 2 ] . the presence of an unknown antigen causes the generation of effector t cells that infiltrate the skin and initiate a pathogenetic process by producing different types of cytokines . tumor necrosis factor- ( tnf- ) is considered the most important cytokine involved in the pathogenesis of psoriasis [ 3 , 4 ] . indeed , tnf- plays an important physiological role in host defense , inflammation and cell differentiation , and a pathological role in many conditions such as fever , rheumatoid arthritis and some malignant neoplasms , e.g. b - chronic lymphocytic leukemia ( b - cll ) . b - cll is the most common form of leukemia in the western world , characterized by the slow and progressive accumulation of monoclonal cd5 + b lymphocytes in peripheral blood , bone marrow , lymph nodes and other organs . b - cll is the first example of an indolent cancer characterized by abnormally low apoptosis rather than increased mitosis . although multiple apoptosis inhibiting factors other than tnf- have been identified in b - cll , several data indicate that tnf- is a central mediator in apoptosis resistance of malignant lymphocytes in b - cll . a 41-year - old white woman , suffering from plaque psoriasis since she was 23 years old , was referred to our outpatient clinic . she was not affected by psoriatic arthropathy and was known to be in good health with no other illnesses , no history of major surgical procedures and was receiving no concomitant medications . she was administered two 8-month cycles of cyclosporine treatment with moderate results in 2005 and 2006 . in 2007 , she started phototherapy with ultraviolet b narrowband ; she did not experience good results because of a scarce compliance to this kind of treatment . in order to start a new therapy based on biologic drugs , all routine laboratory tests ( serum chemistry , hematology , and urinalysis ) were performed . the results were normal except for a moderate lymphocytosis ( 63% , normal range 2045% ; 6.32 10/l , normal range 1.23.5 10/l ) with a normal white blood cell count . afterwards , laboratory tests were repeated and moderate lymphocytosis was present again ; according to this evidence , a hematologic counseling was required , and the diagnosis of b - cll was made . since tnf- is reported as a common link between psoriasis and b - cll , two cycles of etanercept were administered ( 1 cycle = 12 weeks ) from january to june 2008 . lymphocyte levels remained stable ( 61% ; 6.1 10/l ) , indicating no progression of the leukemia , but after 24 weeks of treatment , the patient did not achieve psoriasis area and severity index ( pasi ) 50 ( a 50% reduction in pasi score ) . therefore , in december 2008 , she was started on a course of treatment with infliximab ( 5 mg / kg intravenous ) . after the 3rd infusion of infliximab , a significant improvement of clinical manifestations was observed ( fig . pasi and body surface area decreased from 12.4 to 5.5 and from 18.8 to 9.0% , respectively . after 18 months of infliximab treatment , lymphocyte levels remained stable and psoriatic manifestations were under control . here , we have described the case of a patient suffering from plaque psoriasis and b - cll . the patient had suffered from psoriasis for 18 years and , like many other subjects with moderate - to - severe psoriasis , had received a series of different treatments ( topical and systemic ) in an attempt to control symptoms . laboratory tests showed a moderate lymphocytosis so that a hematologic counseling was required and a diagnosis of b - cll was made . a t - helper 1 cytokine - mediated pathway is involved in these disorders in which tnf- plays a central role . tnf- involvement in psoriasis has been well validated by the clinical success of anti - tnf- therapy . on the other hand , tnf- is constitutively produced by b - cll cells , and it may act as an autocrine factor for their growth and survival . moreover , in b - cll patients , tnf- serum levels and soluble tnf- receptor ( stnfr ) levels are increased , and a correlation with disease progression has been demonstrated , associated with an adverse prognosis ; it has been hypothesized that high levels of tnf- and stnfr can predict outcome in lymphoma patients . confirmed the relationship between tnf- plasma levels and the severity of b - cll , suggesting that they should be monitored together with other disease markers . the first drug chosen was etanercept , a dimeric fusion protein consisting of the extracellular ligand - binding portions of two soluble tnf- receptors fused to a fc fragment of an immunoglobulin g1 molecule . two cycles of etanercept were administered ( 50 mg twice a week for 12 weeks followed by 50 mg every week until 24 weeks ) with poor effects on psoriatic disease and no b - cll progression . therefore , the patient was started on a course of treatment with infliximab ( 5 mg / kg intravenous ) . infliximab is a chimeric murine / human immunoglobulin g1 monoclonal antibody that binds specifically to human tnf-. in our case , psoriatic lesions improved and b - cll showed a lack of progression during infliximab therapy . during 3 years of anti - tnf- therapy , the patient did not show significant modifications of lymphocyte levels . on the basis of this evidence , tnf- could represent a therapeutic target in patients affected by both psoriasis and b - cll ; specific inhibition of tnf- , either with dimeric fusion protein etanercept or the monoclonal antibody infliximab , could potentially result in the control of proliferation of the leukemic clone . we report this case to highlight the possibility to administer anti tnf- treatment in psoriatic patients affected by concomitant b - cll .

psoriasis is a chronic immunologically - based inflammatory skin disease . b - chronic lymphocytic leukemia ( b - cll ) is a form of leukemia characterized by the slow and progressive accumulation of monoclonal cd5 + b lymphocytes in peripheral blood , bone marrow , lymph nodes and other organs . a t - helper 1 cytokine - mediated pathway is involved in these disorders in which tumor necrosis factor- ( tnf- ) plays a central role . tnf- is involved in physiological phenomena , such as host defense , inflammation and cell differentiation , and in many pathological conditions , such as fever and some malignant neoplasms . tnf- involvement in psoriasis has been well validated by the clinical success of anti - tnf- therapy . tnf- has been well studied in the pathogenesis of b - cll , suggesting it as a target in b - cll therapy . we present the case of a patient suffering from plaque psoriasis and b - cll . since tnf- is reported as a common link between psoriasis and b - cll , the patient was treated with etanercept followed by infliximab , two anti - tnf- drugs . during 3 years of therapy , the patient did not show significant modifications of lymphocyte levels , indicating no progression of b - cll . we report this case to highlight the possibility to administer anti - tnf- treatment in psoriatic patients affected by concomitant b - cll .

experimental groups of 20 ( 4- to 6-week old ) c57bl/6 female mice ( charles river , l'arbresle , france ) were injected intracerebrally with 20 l of 10% ( weight / volume ) homogenates per mouse prepared from brain stem samples of 3 cattle tse isolates . two of the isolates were characterized , as previously described ( 7 ) , by a higher molecular mass of unglycosylated prp ( h - type isolates ) and labeling with p4 monoclonal antibody ( table ) . mice were housed and cared for in an appropriate biohazard prevention area ( a3 ) according to european ( directive 86/609/eec ) and french ethical committee ( decree 87848 ) guidelines . mice were checked at least weekly for neurologic clinical signs and were killed when they exhibited signs of distress or confirmed evolution of clinical signs . the whole brain of every second mouse was frozen and stored at 80c before western blot analysis . frozen mouse brain tissues and fixed brain tissues were examined by western blot analysis and immunohistochemical tests as previously described ( 12,13 ) . prp extracted from half of whole brain was detected with monoclonal antibodies sha31 ( 1:10 from tesee sheep / goat western blot , bio - rad , hercules , ca , usa ) ( 14 ) and 12b2 ( 340 ng / ml ) ( 15 ) . these antibodies are directed against the 144-wedryyre-151 and 88-wgqgg-92 murine amino acid prp sequences , respectively . antibody 12b2 , which has an n - terminal specificity similar to that of monoclonal antibody p4 , shows poor binding to bse - derived prp , but unlike p4 , binds with high affinity to prion protein from most mammalian species , including mice and cattle . bound antibodies were detected by using enhanced enzymatic chemiluminescence ( amersham , little chalfont , uk ) or supersignal ( pierce , rockford , il , usa ) and visualized either on film ( biomax , eastman kodak , rochester , ny , usa ) or directly in an image analysis system ( versadoc , bio - rad ) . molecular masses of prp glycoforms were determined as the average of the center positions of the bands from at least 3 repeated electrophoretic procedures , as measured by comparison with a biotinylated marker ( b2787 , sigma , saint louis , mo , usa ) included on each gel . immunologic reactivities of antibodies 12b2 and sha31 were compared in western blots run in parallel with the same samples with both antibodies . after intracerebral injection of cattle brain samples into c57bl/6 mice , disease was observed in mice with the 2 h - type isolates , as well as with the bse sample . survival periods of mice and results of prp detection among mice analyzed by western blot are shown in the table . western blot analysis of prp from h - type infected mouse brains in comparison with bse - infected mice is shown in figure 1 . this pattern showed higher molecular mass prp glycoforms in mice infected with h - type isolates than in mice infected with a typical bse agent ( 1.1- to 1.5-da difference in the unglycosylated prp ( figure 1a ) . studies of prp protease cleavage showed that only the prp of mice infected with h - type isolates was recognized by antibody 12b2 ( figure 1b ) . this finding is in contrast to the result obtained with monoclonal antibody sha31 directed against an epitope in the central region of the protein , which showed that the 12b2 epitope was preserved in h - type infected mice . thus , the molecular features of h - type cattle isolates , which are distinct from those of the bse agent , were maintained after development of disease in mice . western blot analysis of disease - associated prion protein ( prp ) from proteinase k treated brain homogenates of c57bl/6 mice infected with type h ( lanes 2 and 4 ) or bovine spongiform encephalopathy isolates ( lanes 3 and 5 ) . prp of mice infected with an experimental scrapie strain ( c506m3 ) ( 6 ) was used as a control ( lane 1 ) . monoclonal antibodies used for detection of prp were sha31 in panel a and 12b2 in panel b. lane m , molecular mass markers : 39.8 , 29 , 20.1 , and 14.3 kda . histopathologic analysis showed vacuolar lesions in the thalamus ( figure 2a ) that were absent from the hypothalamus , cochlear nucleus , and superior collicules . these 3 neuroanatomic sites were severely affected in c57bl/6 mice brain after primary passage of the bse agent as we and others have reported ( 1 ) . abnormal prp was detected only in amyloid plaques ( figure 2b ) , in contrast to what was reported after bse transmission in c57bl/6 mice ( 1 ) . a ) characteristic vacuolar lesions in the thalamus ( hematoxylin and eosin stained , scale bar = 60 m ) . b ) immunohistochemical analysis of prion protein with monoclonal antibody 12b2 ( diluted 1:200 ) shows the absence of granular deposition , but the presence of plaques in the thalamus . the inset shows that plaques are amyloids since they bind congo red and show birefringence in polarized light ( scale bar = 60 m , scale bar in inset = 16 m ) . our data show that the recently identified bovine h - type isolates involve an infectious agent that can induce development of a disease across a species barrier , while maintaining the specific associated prp molecular signature . this evidence in favor of a new bovine prion strain in cattle suggests that bse is not the only transmissible prion disease in cattle . these cases suggest either the existence of alternative origins of such diseases in cattle or phenotypic changes of prp after infection with the bse agent . however , based on analysis of molecular features of prion diseases in cattle , this situation is similar to that in humans ( 5 ) , in which different subtypes of sporadic creutzfeldt - jakob disease agents are found .

we previously reported that cattle were affected by a prion disorder that differed from bovine spongiform encephalopathy ( bse ) by showing distinct molecular features of disease - associated protease - resistant prion protein ( prpres ) . we show that intracerebral injection of such isolates into c57bl/6 mice produces a disease with preservation of prpres molecular features distinct from bse .

we conducted a preliminary screening of the 16s rrna methylase - producing bacilli on our gram - negative microbial stock of 2,877 strains isolated from japanese hospitals within the past several years . arbekacin , a semisynthetic aminoglycoside belonging to the kanamycin group , requires 2 modifications at the ( 6 ) aminogroup and the ( 2 ) hydroxyl group for inactivation , so this agent is not inactivated by known plasmid - mediated aminoglycoside - modifying enzymes . 512 mg / l ) was used as a marker for screening the 16s rrna methylase - producing strains . all arbekacin - resistant strains were subjected to polymerase chain reaction ( pcr ) analysis to detect rmta , rmtb , or arma , and all strains were pcr positive , except for a strain of acinetobacter demonstrating a very high level of resistance to arbekacin ( mic 1,024 mg / l ) . this strain was later shown to produce both aminoglycoside 6-acetyltransferase and 2-adenyltransferase ( 12 ) , so arbekacin was inactivated in this strain by both 6-acetylation and 2-adenylation . each pcr primer set was used to detect rmta and rmtb genes as in our previous reports ( 6,7 ) . the pcr primers for amplification of arma were newly designed ( forward : 5-agg ttg ttt cca ttt ctg ag-3 , reverse : 5-tct ctt cca ttc cct tct cc-3 ) , and the predicted size of the amplicon was 590 bp . these 3 sets of pcr primers were very reliable in detecting rmta , rmtb , and arma genes , respectively . each pcr amplicon was then subjected to sequencing analyses on both strands to confirm its nucleotide sequences for detecting mutations in the methylase genes . as reported in our previous study , rmta and rmtb genes had been found in p. aeruginosa isolates ( 6,10 ) and in 1 strain of s. marcescens ( 7 ) , respectively . as shown in the table , 5 p. aeruginosa strains isolated after our previous report ( 6 ) were rmta positive . the rmtb gene was additionally identified in 4 k. pneumoniae , 2 e. coli , and 1 k. oxytoca strains in japan . to our surprise , the arma gene , which had been found in various gram - negative microbial species belonging to the family enterobacteriaceae exclusively in europe as reported by galimand et al . ( 13 ) , was also identified in japan in 1 strain each of e. coli , s. marcescens , and acinetobacter sp . notably , the arma and rmtb genes were also recently identified in k. pneumoniae and e. coli in taiwan ( 9 ) . furthermore , the genetic environment of the arma gene found in c. freundii isolated in poland was similar to that of k. pneumoniae isolated in france . the genetic environments of the arma gene found in the 3 japanese microbial species , e. coli , s. marcescens , and acinetobacter sp . , ( genbank accession nos . ab116388 and ab117519 ) , were also similar to those found in europe ( genbank accession nos . af550415 and ay220558 ) . these findings suggest that the arma - producing gram - negative nosocomial microbes that harbor a very similar genetic environment carrying the arma gene have spread globally . as described previously , arbekacin still shows a very broad antimicrobial spectrum from gram - positive to gram - negative nosocomial microbes and has been approved solely to treat methicillin - resistant staphylococcus aureus ( mrsa ) infections in japan since 1990 to ensure the prudent use of this agent . the emergence and presence of the 16s rrna methylase - producing gram - negative bacilli , however , has not been well recognized in japan to date ; arbekacin has not been listed among the antimicrobial agents for daily antimicrobial susceptibility testing of gram - negative microbes . the use of semisynthetic aminoglycosides , including arbekacin , in japanese clinical settings for > 10 years may have promoted the emergence and dissemination of the 16s rrna methylase - producing gram - negative microbes in japan . the large amount of various aminoglycosides used in livestock - farming environments could have also been a selective pressure for the emergence and spread of pathogenic microbes that harbor genetic determinants for the newly identified 16s rrna methylases , as exemplified by recent isolation of arma - producing e. coli from swine in spain ( genbank accession no . since acquisition of multidrug resistance against clinically important antimicrobial agents such as carbapenems and fluoroquinolones has been developing rapidly worldwide , the acceleration of even greater aminoglycoside resistance among gram - negative bacilli promises to become an actual clinical concern in the near future , just as vancomycin - resistant enterococci ( vre ) did in the 1990s ( 14 ) . the emergence of gram - positive cocci including mrsa and vre that acquire the 16s rrna methylase could also be a grave clinical matter , although fortunately no such hazardous microbes have been identified . thus , steps must be taken to further block proliferation of these multidrug - resistant gram - negative super microbes , including p. aeruginosa , k. pneumoniae , and acinetobacter spp . , as well as multidrug - resistant cocci such as mrsa and vre , which have acquired an extraordinarily high level of resistance to various aminoglycosides through production of 16s rrna methylases , especially in clinical environments .

emergence of the newly identified 16s rrna methylases rmta , rmtb , and arma in pathogenic gram - negative bacilli has been a growing concern . arma , which had been identified exclusively in europe , was also found in several gram - negative pathogenic bacilli isolated in japan , suggesting global dissemination of hazardous multiple aminoglycoside resistance genes .

reinforced surveillance systems aimed at monitoring the introduction of chikv have been implemented in 6 departments in southeastern france , including the var department , where ae . albopictus has spread since its introduction in 2004 , presumably from northern italy ( 4 ) . on august 29 , 2010 , a 7-year - old girl ( patient 1 ) with acute febrile syndrome , headache , and abdominal pain sought treatment in the city of frjus ( var ) 1 day after she had returned from rajasthan , india . continuous chikv circulation in northern india districts has been reported during 20092010 ( www.promedmail.org ) . three weeks after the notification of patient 1 , another young girl ( patient 2 ) experienced clinical symptoms that began on september 18 with fever , arthralgia , backache , headache , and retro - orbital pain . patient 2 s physician reported that a young girl ( patient 3 ) , a close friend of her patient , showed clinical symptoms compatible with chikv infection at the same time . patient 3 , who lives near patient 1 , had invited patient 2 to spend the night of september 15 at her home . a serum sample from patient 3 was collected 1 week after onset of fever and monoclonal antibody capture elisa detected high titers of specific anti - chikv immunoglobulin m. the serum sample also showed a weak rt - pcr signal for chikv . given that patients 2 and 3 did not report any recent travel to areas endemic for chikv no complications were recorded , but all 3 patients had persistent weakness and joint pain 3 months after the acute phase . intensive mosquito control measures , including spraying for adult mosquitoes and destroying breeding sites , were undertaken around the patients residences and areas visited by confirmed case - patients . no further cases were found by the active case finding system ( a local physician and laboratories network ) implemented for 45 days after the declaration of the last autochthonous case . a molecular study of france/2010 chikv strains isolated in frjus obtained from patients 1 ( imported case ) and 2 ( autochthonous case ) was performed . viral genomic rna was extracted from chikv grown once in mosquito c6/36 cells and then subjected to rt - pcr amplification by using a set of primers targeting the structural genes of chikv ( 7 ) . paired sequence analysis of the e26k e1 junction showed that the 2 france/2010 chikv strains display a divergence rate < 0.05% at the nucleotide level , whereas 100% identity was observed at the amino acid level . phylogenetic analysis demonstrated that these viral strains belong to a cluster that is closely related to strains from india within the ecsa lineage ( figure ) . the france/2010 chikv isolate from patient 2 might be derived from an indian strain introduced by patient 1 ( index case ) . genotypes e2 - 211 t , e2 - 312 m , e2 - 386a , 6k-8i , and e1 - 284e that are found in the currently circulating strains belonging to the ecsa lineage were identified in france/2010 chikv isolates ( 2,3,7 ) . these isolates also display the genotype e1 - 211e specifically shared by viral strains belonging to the asian phylogenetic group ( table ) . the residue ala at position e2 - 264 has not been previously described in any chikv strains . phylogenetic relationships among chikungunya virus isolates from cases of chikungunya fever in france , based on complete e2 - 6k - e1 nucleotide sequence ( 2,771 nt ) analysis . phylogenetic analysis was inferred by using the maximum - likelihood method as implemented in mega version 5 software ( www.megasoftware.net ) . the sequence of the strains from france described in this study has been deposited in genbank ( accession number pending ) ; other sequences were retrieved from genbank . * molecular signatures were based on the analysis of complete amino acid sequence e2 - 6k - e1 ( 923 aa ) . the numbering of amino acid positions refers to the african isolate s27 ( genbank access no . letters in parentheses after strain names refer to east / central / south africa ( a ) , asia ( b ) and west africa ( c ) phylogroups . recent attention has focused on the predominant role of e1 and e2 proteins in successful chikv infection of the anthropophilic ae . vector competence experiments with la runion/2006 chikv isolates demonstrated the importance of the newly acquired e1-ala226val substitution for efficient transmission by ae . albopictus from northern italy and from southeastern france showed disseminated infection rates ranging from 75%90% for chikv strains with e1 - 226v ( 10 ) . the 2 france/2010 chikv strains isolated in frjus have ala at position e1 - 226 ( table ) . the presence of an asp residue at position e2 - 60 , found in most of the ecsa chikv strains , may in part counterbalance the less favorable transmission of e1 - 226a strain in ae . albopictus ( table ) . the thr residue at position e2 - 211 potentiates the infectivity of chikv in ae . the presence of e2 - 211 t in chikv isolates from france underlines the risk for emergence of a fully adapted viral variant if the e1 - 226v genotype was selected during continuous transmission within ae . the efficient chikv transmission in italy and southeastern france sheds new light on its dissemination potential in europe from 1 index case , regardless of the viral genetic background and mosquito species in the region of origin of the imported chikv ( 1,10,11 ) . in emerging regions , such as italy and runion island , where the seroprevalence in the population was < 50% however , > 2 years passed since the end of the epidemic in runion island before a local transmission of chikv was again detected . in europe where sylvatic cycles are absent , vertical transmission may participate in the maintenance and/or cyclic reemergences of chikv . albopictus that may have more efficient vertical transmission than mosquito populations in eastern italy and tropical regions ( 11,12 ) . in 2010 , southeastern france faced the concomitant emergences of dengue virus ( denv ) and chikv ( 13 ) . for each of these viruses , only 2 autochthonous infections were confirmed , which suggests that rapid detection and control measures implemented around imported and autochthonous cases have been efficient . a recent report mentioned the dual emergence of chikv and denv in southeastern france and urged the implementation of specific surveillance and response measures to reduce the risk for arbovirus emergence ( 14 ) . since 2006 , a specific chikungunya / dengue national preparation and response plan based on rapid detection and investigation of imported and suspected autochthonous cases , mosquito control measures , and efficiency evaluation in the treated areas has been activated from may through november and then modified after annual debriefing meetings involving all partners . in 2010 , this model proved to be well adapted to the early detection and control of chikv and denv . albopictus mosquitoes and the successful emergence of chikv in italy and france , reinforced surveillance and response to chikv and denv dissemination should become a higher priority in europe ( 15 ) .

in september 2010 , autochthonous transmission of chikungunya virus was recorded in southeastern france , where the aedes albopictus mosquito vector is present . sequence analysis of the viral genomes of imported and autochthonous isolates indicated new features for the potential emergence and spread of the virus in europe .

rhabdomyosarcoma ( rms ) was first described by weber in 1854 , is a malignant soft tissue neoplasm of the skeletal muscle origin . the most common sites of involvement of rms are head and neck , genitourinary tract , retroperitoneum , and extremities . clinically , the manifestations of rms may vary from a small cutaneous nodule on the face to an extensive fast - growing facial swelling , which may be painless or occasionally associated with pain , trismus , paresthesia , facial palsy , and nasal discharge . the histogenesis of rms is still unclear , but the most widely accepted hypothesis is that rms arises due to the proliferation of embryonic mesenchymal tissue . the survival rate of patients with this tumor ranged from 20% to 35% in reported series . the purpose of this article was to report a case of oral rms with mobile tooth present in upper left posterior maxillary ridge surrounded by large , firm , and tender swelling in a 1-year - old boy and discuss the clinical , radiological , histopathological , and immunohistochemical features . an 1-year - old boy was referred to our institution with painful swelling in his mouth . on history taking , his parents reported that the swelling was present for 67 months which was earlier small and painless but now has increased in size up to 5 cm 6 cm . initially , the patient was seen by a physician who prescribed antibiotic / anti - inflammatory therapy , however , the treatments were ineffective , and he was , therefore , referred to us . written informed consent was obtained from her parent for further investigations . a mobile tooth is present in upper left posterior maxillary ridge surrounded by large , firm , and tender swelling . intra - oral examination showed a 5 cm 6 cm , red , firm mass with grayish areas of central necrosis , covering the left side of the maxillary gingiva , from the canine to the second molar region [ figure 2 ] . extraoral photograph showing the lesion intraoral aspect showing extensive mass involving the maxillary alveolar mucosa computed tomography confirmed the presence of an extensive infiltrative lesion accompanied by severe bone destruction and displacement of adjacent structures [ figure 3 ] . histopathological analysis of the hematoxylin and eosin stained material showed clusters of small round cells with hyper chromatic nuclei and eosinophilic cytoplasm separated by fibrovascular septae [ figure 4 ] . the neoplastic cells were strongly positive for vimentin , desmin , myoglobin , and muscle - specific actin . a diagnosis of oral rms was established on the basis of the history , clinical , radiographic and histopathological findings . computed tomography scan showing extensive infiltrative lesion with displacement of adjacent structures photomicrograph showing a sheet of mesenchymal cells in a myxoid stroma ( h and e , 200 ) after performing the standard diagnostic workup , the tumor was diagnosed as alveolar rms . he was referred to the pediatric oncology department , and the proposed treatment plan was a combination of chemotherapy , including vincristine , actinomycine , cyclophosphamide , and dexamethasone radiotherapy . the tumor continued to increase in size , and the patient died from lung metastases 6 months after the treatment . the incidence of rms is the highest in children aged 14 years , lower in children aged 1014 years , and lowest in those aged 1519 years . these tumors exhibit a fast and aggressive growth , reaching large dimensions , and are generally painless associated with high rates of recurrence and generalized metastases through the hematogenic and/or lymphatic routes . the head and neck region is the most common site for rms , with the orbit being the most frequent primary site . the most common site of involvement in the oral cavity is the tongue followed by the soft palate , hard palate , and buccal mucosa . in our patient , the maxillary alveolar ridge and hard palate were involved . a careful histological examination is required to differentiate such lesions from other more frequent and aggressive lesions affecting the concerned site . in our case , the marked pleomorphism noted was critical for differentiating rms from ewing 's sarcoma . the presence of an alveolar pattern , pleomorphism , cohesive nature of the cells , and the absence of lymphadenopathy ruled out the diagnosis of lymphoma . in this respect , however , neuroblastoma , another small cell tumor characterized by a diffuse pattern of small round cells and the presence of rosettes / pseudorosettes with pale eosinophilic material is quite similar to the alveolar variant of rms . the frequently elevated level of urinary catecholamines in neuroblastoma aids in the differential diagnosis . the differential diagnosis also includes vascular malformations , within which the most common affecting the pediatric airway is the lymphatic or lymphatic - venous malformation . prognosis of rms is relatively poor compared to that of other oral soft tissue malignant lesions and depends on the clinical staging and the anatomic site of the tumor . unfortunately , in the present case , the lack of cooperation of the patient 's guardians and the lack of institution of adjuvant chemotherapy and/or radiotherapy at first admission may have favored the rapid progression of the tumor and subsequently aggravated the severity of the condition , resulting in the child 's death . patients with rms may present signs and symptoms such as pain , paresthesia , loss of teeth , and trismus as a result of factors such as advanced tumor stage , infiltrative growth , and tumor location . in the present case , involvement of teeth it consists of surgical removal of the tumor followed by multiagent chemotherapy with or without radiotherapy since rms tends to metastasize to bone marrow . finally , we conclude that in children , any swelling should be carefully examined , and treatment outcomes should be regularly followed up . high degree of suspicion , early diagnosis , and a multidisciplinary treatment approach would be of great importance in such cases .

rhabdomyosarcoma ( rms ) , a tumor of skeletal muscle origin , is the second most common soft tissue sarcoma encountered in childhood after osteosarcoma . the common sites of occurrence are the head and neck region , genitourinary tract , retroperitoneum , and to a lesser extent , the extremities . site predilections in the oral cavity are a soft palate , maxillary sinus and alveolus , posterior mandibular region , cheek and lip , and possibly tongue . rms is a highly malignant tumor with extensive local invasions and early hemorrhagic and lymphatic dissemination . despite aggressive approaches incorporating surgery , dose - intensive combination chemotherapy , and radiation therapy , the outcome for patients with metastatic disease remains poor . here , we report a case of oral rms in a 1-year - old child and describe the clinical , radiological , histopathological , and immunohistochemical findings .

neonatal intestinal perforation ( nip ) is an extremely rare complication of intestinal hemangioma ( ih ) . neonates younger than 30 days and having perforation include more common etiologies such as necrotizing enterocolitis , meconium ileus , and spontaneous idiopathic as well as gastric perforation.1 nip has been associated with mortality rates of 40% to 70%.2 this report presents a rare case of nip resulting from ih . a 27-day - old male neonate was admitted with low - grade fever , abdominal distension , and bilious vomiting associated with bouts of diarrhea of 2 days duration . baby was a product of full - term spontaneous vaginal delivery with normal prenatal follow - up . before coming to our hospital , the patient weighed 4.1 kg and was febrile , irritable , and moderately dehydrated . plain abdominal radiographs suggested bowel obstruction versus ileus , therefore a barium meal follow through was obtained , which showed persistently dilated jejunal and proximal ileal loops but normal flow of contrast . computed tomographic scan with intravenous contrast was not done because of family history of allergic reaction to the dye . on the basis of the diagnosis of intestinal obstruction , a decision for exploratory laparotomy was made . laparotomy revealed dense inflammatory adhesions surrounding a solitary hemangioma located at the antimesenteric border 30 cm from the ileocaecal valve and on close inspection a small perforation was seen on one side of the hemangioma , which was sealed by extensive adhesions ( fig . other areas of the bowels inspected were normal and resection with primary anastomosis was done . dilated capillary spaces lined by single layer of endothelium with proliferation of endothelial cells in between . hemangiomas of the gastrointestinal tract are rare and account for only 0.05% of all intestinal neoplasms.3 they are commonly found within the small bowel , jejunum being the commonest site and compose 7 to 10% of all benign tumors.3 4 they have a tendency toward multiplicity , with solitary tumors being extremely rare.3 5 microscopically , ih may be classified as cavernous , capillary , or mixed types , commonest being the cavernous.6 the usual presentation is intestinal bleeding usually insidious presenting as anemia7 or sometimes acute and potentially life threatening.4 other forms of rare presentation include intussusception , obstruction , and perforation.8 9 10 ileal perforation is a rare complication of ih in neonates , and so far , only one case has been reported in the literature , by mcgaughey et al,11 who discovered evidence of ileal perforation due to ih while operating on a neonate with intestinal obstruction . ours is the second reported case of nip due to ih , and what makes it unique is the fact that the hemangioma belonged to the rare capillary type whereas the previously reported case was of the commoner cavernous type . diagnosis in both reported cases , including ours , were made only at surgery for presumed acute intestinal obstruction . although various causes of nip including the rare ih have been reported , diagnosis can be difficult and exploratory laparotomy has often proved to be the final diagnostic tool . although a rare cause of intestinal perforation in neonates , ih should be considered in the differential diagnosis .

intestinal perforation is an extremely rare complication of hemangioma of the small intestine in the neonatal period . this is a case report of a 27-days - old male infant who presented with signs and symptoms of acute intestinal obstruction . exploratory laparotomy findings revealed intestinal perforation due to solitary hemangioma in the ileum , which led to obstruction from peritoneal reaction and adhesions . there are two reported cases in the literature presenting with ileal perforation in the pediatric age group , but only one previous report mentioned in the neonatal period . because there are other more common causes of perforation in the neonatal period , intestinal hemangioma in spite of its rarity should be included in the differential diagnosis .

delirium caused by somatic factors is a widespread and dangerous problem associated with the aging of the population . it has been reported that consciousness disorders are experienced by 10 - 15% of patients on general wards and up to 50% of patients admitted emergently to geriatric wards . the frequent occurrence of delirium stems from the commonness and number of risk factors for this complication , which include , among others : old age , organic injury of the central nervous system , alcoholism , malnutrition , chronic somatic diseases , chronic pain and analgesic treatment , long - term insomnia , electrolyte disorders , blood loss , dehydration , cognitive impairments , depression , metabolic disorders , use of psychoactive agents , immobility , urinary catheterization , and numerous factors associated ( directly or indirectly ) with surgical procedures [ 14 ] . one - year mortality of delirium in the population of intensive care unit patients is estimated to be around 40% [ 5 , 6 ] . one should keep in mind that , although qualitative disorders of consciousness are reversible and transient , they directly precede the patient 's death in 88% of cases . persisting symptoms of delirium result in growing motor agitation , disorganization , and anxiety , which increases the risk of injury or self - harm , necessitates parenteral nutrition and the maintenance of water balance , and impedes cooperation , thus worsening the course and prognosis of the underlying disease . consciousness disorders are believed to be among the most challenging diagnostic problems and the most difficult therapeutic problem among psychotic disorders . differential diagnosis typically considers dementia as well as maniacal , paranoid , and delusional syndromes . a particular diagnostic challenge is presented by hypoactive delirium , which is often confused with depression , depressive stupor , catatonia , or apathetic - abulic syndromes . retrospective studies state that this form of delirium with poor motor activity is observed in the decided majority of cases of delirium in old patients ; its frequency correlates with the deterioration of the patient 's general condition . these data indicate an obvious need for improving the diagnostics of consciousness disorders on somatic wards , especially those employing surgical procedures . in 1990 , a new diagnostic tool for mid - level medical personnel was developed ; the purpose of the confusion assessment method ( cam ) is the early detection of somatically induced delirium . numerous studies conducted over the period of 16 years established cam 's position as an effective standardized diagnostic tool ; its sensitivity was estimated at 94 - 100% , specificity : 90 - 95% , positive predictive value : 91 - 94% , and negative predictive value : 90 - 100% . therefore , the cam questionnaire had been translated into 10 languages by 2008 ; it is widely used in japan , germany , finland , denmark , france , italy , spain , portugal , turkey , china , and other countries . the aim of this article is to present the polish version of this screening diagnostic tool . the cam questionnaire consists of 9 points with questions verifying the presence of : acute changes in mental status , attention disorders , disorganized thinking , altered level of consciousness , disorientation , memory impairment , perceptual disturbance , psychomotor agitation or retardation , and sleep - wake cycle disturbances . the questions require the respondent to evaluate the severity and dynamics of the symptoms ; space is also provided for descriptive comments . most questions pertain to information that can be acquired during a single patient examination ; answers to the first and last question require information concerning the patient 's stay , which can be gathered from the patient 's records or the attending medical personnel . the preliminary diagnosis of delirium only requires answers to the first five questions , constituting the so - called short form of cam . in order to interpret the questionnaire 's results , it is necessary to be acquainted with the scoring manual , which has also been translated in this article . the aim of the authors was to provide a translation that would correspond to the intentions of the questionnaire 's original creators to the largest degree possible . therefore , the original authors were invited to actively cooperate on the project . after receiving approval from professor sharon k. inouye , the director of the aging brain center , the first stage of work on the translation began : two independent translation groups were formed , each consisting of three physicians , including one psychiatrist . the first group translated the original text into polish , while the second group translated the new polish text back into english without knowing the original version . subsequently , the return translation was sent to the authors of the original questionnaire . based on their concerns and suggestions , the final version was modified twice before receiving full approval of the original questionnaire 's authors . the new polish version of cam was accepted for publication among the other official translations on the help webpage : ( http://www.hospitalelderlifeprogram.org/uploads/disclaimers/cam_polish.pdf ) . apart from cam , other tools for consciousness disorder screening are used around the world , including the delirium symptom interview ( dsi ) , the neecham confusion scale , the delirium observation screening ( dos ) scale , the cognitive test for delirium ( ctd ) , and the descard tool used in poland . among these tools , evaluation using the cam questionnaire can be performed by any member of the medical staff based on the instructions contained within the cam training manual . the tool has already been translated into 10 languages and is being used in 10 non - english speaking countries . it can be suspected that introducing the cam scale into everyday practice in poland will also facilitate the diagnostics of consciousness disorders ; however , one should keep in mind that evaluation using a questionnaire is only a screening tool , and one should always strive to confirm its preliminary diagnosis with a clinical examination , preferably conducted by a consulting psychiatrist . the questionnaire has been translated by physicians with active support provided by staff members of the aging brain center , the creators of the original version . the polish translation still requires validation on somatic wards as well as further studies to determine its effectiveness and sensitivity in clinical conditions . the first obtained data indicate that the polish version of cam is characterized by good diagnostic accuracy , but its usefulness in everyday practice requires further studies with more precise validation of the questionnaire in clinical conditions .

confusion on a somatic basis is a dangerous problem mainly related with aging of the population . data says that consciousness disorders concern 10 - 15% of patients in general wards , and up to 50% of patients admitted to geriatric wards . the persistence of the symptoms of confusion results in increase of agitation , disorganization , fear , which increases the risk of self - injuries of patients , it causes the need for parenteral feeding and hydratation , as well as maintaining water balance , and also disturbs cooperation , worsening the course and prognosis of the primary disease . it is believed that consciousness disorders are one of the most difficult diagnostic problems and the most difficult therapy problem among psychotic disorders . so far in poland there have been no screening evaluation tools to assess the occurrence of confusion on the somatic basis . the purpose of this paper is to introduce the polish translation of the widely used scale to assess consciousness disorders , intended also for middle personnel of health care confusion assessment method ( cam ) . numerous studies over 16 years established the position of cam as an exceptionally effective standardized diagnostic test , specifying the sensitivity of 94 - 100% , specificity from 90 - 95% , positive predictive value of 91 - 94% , negative predictive value of 90 - 100% . the questionnaire and instructions of its interpretation have been translated by doctors with active help from the original creators of cam . further studies are required in order to validate and determine the effectiveness of the newly formed diagnostic tool .

fixed drug eruption ( fde ) is characterized by recurrent well - defined lesions in the same location each time the responsible drug is taken . we report here a rare case of fde induced by atenolol , a beta - adrenoreceptor - blocking agent . a 48-year - old tunisian woman was referred to our department for the appearance of five inflammatory plaques on both legs . she had been treated for hypertension with atenolol ( hypoten , al - hikma pharmaceuticals , jordan ) 100 mg once a day for six weeks . no other medications had been taken and she had not previously received beta - blocking agents . physical examination revealed five well - demarcated reddish and round plaques with an itching and burning sensation on both legs ( figure 1 ) . skin biopsy , taken from a lesion with no vesicular changes , showed focal necrosis of keratinocytes , hydropic degeneration of the basal cells , dermal edema and a perivascular lymphocytic infiltrate of the upper dermis . topical desonide ( locatop ) was applied twice a day and skin lesions resolved within two weeks with a residual pigmentation . 6 weeks after complete resolution , patch testing was carried out according to the international contact dermatitis research group ( jacobs et al 1999 ) recommendations with 10% atenolol in petrolatum on a previously affected site of the right leg ( figure 2 ) and on normal skin of the back . a positive reaction ( + + ) was seen at d2 and d3 on the left leg but no reaction was detected on the back . they mostly include nonsteroidal antiinflammatory drugs , nonopioid analgesics , sulphonamides , and tetracyclines ( savin 2001 ) . topical provocation testing has been reported to be useful and safe for the diagnosis of fde when applied on previously affected sites ( alanko et al 1987 ; alanko 1994 ; lee 1998 ; ozkaya - bayazit et al 1999 ) . different patch test methods ( open / occlusive ) and variations in their evaluation ( erythema of more than six hours duration / erythema and infiltration ) exist ( ozkaya 2008 ) . beta - blockers - induced fde are very rare ( palungwachira and palungwachira 1999 ; zaccaria et al 2006 ) . only two cases have been reported in the literature ( palungwachira and palungwachira 1999 ; zaccaria et al 2006 ) . they were induced by atenolol ( palungwachira and palungwachira 1999 ) and propranolol ( zaccaria et al 2006 ) . none of them was confirmed by patch testing or systemic provocation ( palungwachira and palungwachira 1999 ; zaccaria et al 2006 ) . thus , to the best of our knowledge , we report herein the first case of fde induced by atenolol and confirmed by a positive patch test on previously affected sites .

fixed drug eruption ( fde ) is characterized by recurrent well - defined lesions in the same location each time the responsible drug is taken . we report here a case of multiple fde induced by atenolol in a 48-year - old woman confirmed by positive patch test in previously affected sites . beta - blockers - induced fde are very rare . only two cases had been reported in the literature . to the best of our knowledge , this is the first case reported of atenolol - induced fde confirmed by a positive patch test .

transcatheter closure of patent ductus arteriosus ( pda ) by use of the amplatzer duct occluder shows good results in properly selected patients.1 the advantages of the amplatzer duct occluder device include a user - friendly delivery system , the ability to retrieve or reposition the device when required , and a high complete closure rate ( > 99% complete occlusion of pda within 6 months of implant).2 although embolization of an amplatzer duct occluder is rare , it can occur . we present a case of retrieval of an embolized amplatzer duct occluder by use of a percutaneous method in a young man . transthoracic two - dimensional echocardiography and computed tomography ( ct ) confirmed pda ( fig . , we deployed the amplatzer duct occluder ii ( 9-pda2 - 05 - 06 , aga med . , usa ) after confirmation of the proper position by repeated angiogram . however , 5 min after successful deployment , the amplatzer duct occluder device was suddenly embolized into the branch of the right pulmonary artery ( rpa ) ( fig . 2a ) . before surgical removal , we decided to remove the embolized device by use of a percutaneous method after obtaining consent from the patient 's relatives . multiple attempts at removal by use of guidewire twisting , a goose - neck snare , and a radiofrequency ablation catheter were unsuccessful . because the embolized device was floating in the branch of the rpa , it moved into the distal portion of the rpa after several attempts of retrieval with the snare and ablation catheter . in order to stabilize the embolized device , a 5.020 mm balloon catheter was placed just distal to the embolized device and inflated . endomyocardial biopsy forceps ( cordis corp . , usa ) were introduced from the left femoral vein through an 8-fr sheath . after multiple attempts , we caught the screw attachment tip of the embolized device and removed it into the 8-fr shuttle sheath ( fig . subsequently , an amplatzer duct occluder 8 - 6 ( 9-pda-005 ) was carefully deployed via a venous route . the device was confirmed to be properly positioned across the defect by echocardiography and the ct angiogram ( fig . neither clinical complications nor a residual shunt was observed during the 6-month follow - up . transcatheter pda closure is currently the standard of therapy and is performed with high success rates and few complications.3 although the rate of device embolization is rare ( < 0.5% ) , this remains a major complication requiring urgent surgical management.4 the most common cause of embolization is the use of an undersized device . therefore , exact pda sizing and the use of the proper size of device is essential to avoid this potential complication . careful attention must be paid to imperfect alignment or malposition of the device or excessive tension on the delivery cable ; performing the wiggle procedure may reduce the acute failure rate of the procedure.5 excessive tension on the delivery cable was the main cause of embolization in our patient . in this case , stabilizing the floating device by balloon inflation played a pivotal role in success . the use of a larger 8-fr sheath during retrieval could make easier device entry into the sheath after capture of the embolized device . in general , the following techniques are recommended to increase the chances of successful retrieval : the use of a sheath 2 fr sizes larger than the delivery sheath , the use of stiff sheaths to avoid kinking , and cutting a bevel or notch at the end of the sheath to facilitate the entrance of the embolized device.6 our case illustrates a practical transcatheter removal technique for an embolized amplatzer duct occluder . even in procedures performed by the most experienced operators , thus , physicians have to keep in mind both the possibility and the treatment of this severe complication during pda closure .

transcatheter closure of patent ductus arteriosus with the amplatzer duct occluder shows excellent results . however , there is a risk of device embolization after deployment . we report the case of a 33-year - old man in whom an embolized amplatzer device was retrieved from the right pulmonary artery by a percutaneous method . we also review this uncommon severe complication and offer technical tips for percutaneous removal .

a 52-year - old previously healthy male patient visited the emergency department of a local clinic due to dyspnea with the recent onset of chest pain . an emergency coronary angiogram ( cag ) , which was prompted by a 12 lead ekg study suggesting precordial lead st segment elevation myocardial infarction revealed a totally occluded distal left circumflex coronary artery . although percutaneous stenting of the culprit vessel was successfully performed , the symptoms of dyspnea had persisted . a subsequent trans - thoracic echocardiogram showed severe mitral regurgitation ( mr ) caused by a ruptured papillary muscle head . the patient was , therefore , subsequently transferred to our hospital for urgent surgery of the mitral valve . a preoperative echocardiogram showed severe prolapsing mitral regurgitation ( a proximal isovelocity surface area radius of 14 mm at an aliasing velocity of 40 cm / sec ) due to rupture of the posteromedial papillary muscle and a decreased left ventricular ejection fraction of 48% . mild to moderate pulmonary hypertension with a tricuspid regurgitation ( tr ) velocity of 3.3 m / sec was also noted . at surgery moderate hypothermic cardiopulmonary bypass at 27 esophageal temperature was performed through selective cannulation of the aorta , superior vena cava , and inferior vena cava . maintenance of cardioplegia was achieved by continuous retrograde coronary sinus infusion of cold blood cardioplegia . the mitral valve was exposed by transseptal incision extended to the left atrial roof , as exposure would otherwise have been extremely difficult through sondergaard 's groove in the relatively small - sized normal left atrium of this patient . when viewed directly , it became clear that the anterior mitral valve a3 prolapse was caused by the disruption of a subpapillary muscle head of the posteromedial papillary muscle . after careful examination to rule out any additional structural valvular pathology that could have complicated the planned simple repair , the disrupted papillary muscle tip was reattached to the papillary muscle base with three interrupted mattress sutures of 5 - 0 polytetrafluoroethylene buttressed by a pledget . the approximating sutures were passed through healthy tissue adjacent to the endocardial surface at the base , thereby avoiding suturing the friable , necrotic tissue of the detached margins . finally , a 26 mm cosgrove - edwards annuloplasty band ( edwards lifesciences , irvine , california , usa ) was used to perform the undersized mitral annuloplasty ( fig . two interrupted stitches of 5 - 0 polypropylene sutures were placed to approximate the lateral commissural portions of the anterior and posterior mitral leaflets to correct the residual trivial mr on saline testing . after an uneventful weaning of the cardiopulmonary bypass ( cpb ) , the proceeding trans - esophageal echocardiography showed no residual mitral stenosis or regurgitation . follow up echocardiography on the fifth postoperative day showed no mitral regurgitation but persistent mild left ventricular dysfunction with an ejection fraction of 42% . the patient remained in excellent clinical condition on the 4 postoperative month , which was the last follow up . severe mr occurs in about 3% of patients presenting with an acute myocardial infarction , and is associated with an in - hospital mortality of up to 70% . although progress in surgical management has led to improved outcomes over the decades , operative mortality remains high . the papillary muscle is a contiguous structure of the adjacent left ventricular wall that serves to preserve the contractile function and acts as an anchor for the chordae tendinae . while the anterolateral papillary muscle receives dual blood supply from the left anterior descending and circumflex coronary arteries , the posteromedial papillary muscle usually receives a single blood supply from either the right or left circumflex coronary artery . as a result , the posteromedial papillary muscle is relatively more vulnerable to rupture than the anterolateral papillary muscle , with a nearly three- to six - fold higher reported rate of disruption after acute myocardial infarction . repair is usually recommended over replacement for the treatment of functional ischemic mr , as it is generally conducive to superior survival outcomes . however , in acute postinfarction papillary muscle rupture , experience is limited and most surgeons prefer to replace rather than repair the mitral valve . this is mainly due to their uncertainty regarding the durability of a repair that inevitably requires suturing onto friable and infarcted myocardial tissue . in addition , the potential for a longer aortic cross clamping time , greater technical difficulties in achieving a successful repair , and the likelihood of ventricular remodeling progression with consequent recurrence of mitral regurgitation have also led to a greater preference for valve replacement in this subset of patients . in the present case , one of two papillary projections of the posteromedial papillary muscle was disrupted . the basal portion of the main posteromedial papillary muscle appeared to be non - necrotic . therefore , from the perspective of the papillary muscle mass involvement , the posteromedial papillary muscle was partially infarcted and thus considered to be amenable to repair by re - attaching the detached papillary muscle head to normal tissue . reported a modified papillary muscle re - implantation technique involving reattaching the ruptured papillary muscle tip to a healthy area of the papillary muscle and reinforcing the repair with additional sandwiched pericardial pledgetted ptfe sutures . we avoided approximating necrotic tissue directly with necrotic tissue by placing the sutures through healthier - appearing tissue that was closer to the ventricular endocardium at the papillary muscle base . a search of the literature published in korea revealed surgery for acute post infarction papillary muscle rupture performed in nine patients . of note , in all of them , therefore , to the best of our knowledge , this is the first reported case of a post infarction papillary muscle rupture that was treated with reimplantation of the papillary muscle in korea . although the repair was successfully performed , further studies are warranted to substantiate and validate the durability of this method .

a 53-year - old man presenting with dyspnea and chest pain was diagnosed with acute myocardial infarction secondary to occlusion of the left circumflex coronary artery . urgent revascularization by percutaneous stenting was successfully performed . however , the post - echocardiography revealed a ruptured papillary muscle that was causing severe mitral regurgitation and aggravation of congestive heart failure . the patient subsequently underwent mitral valve repair with papillary muscle re - implantation . postoperative echocardiography showed a competent mitral valve without residual stenosis or regurgitation . the patient was discharged from the hospital with an uneventful recovery and has been doing well on outpatient follow up .

ganglioside may influence growth and cell - to - cell interactions may be of importance in the development of malignancy . in the present study , we used a transplantable murine hepatoma as the experimental model , and treated the animals with 5-fluorouracil ( 5-fu ) as the active therapeutic drug to determine the membrane glycolipids content variety . kunming mice ( normal mice ) were obtained from the animal center at fu - dan university shanghai medical college . mice containing the ascites - form transplantable hepatoma were obtained from the cancer institute of jiangsu province . the ascites was drawn from the transplantable hepatoma mice by a sterilized injector and placed into bacteria - free 0.9% nacl for dilution ( 1/3 , v / v ) . subsequently , the normal mice were inoculated with 0.2 ml of diluted cells under the skin in the foreleg and armpit regions . the tumors were allowed to grow for 13 days . usually , solid tumors weighing 12 g were found afterwards . the animals were injected with 5-fu ( 0.2 mg/0.2 ml / day ) i.p . at 48 h after tumor cell transplant . in the control group the animals were injected with a sterilized solution of 0.2 ml of 0.9% nacl instead of 5-fu . a third group of animals , raised without hepatoma transplant or any other form of treatment , was used as the normal group . after the administration of 5-fu or saline , the animals were sacrificed , and tissue and blood samples were collected for examination . based on the method of sevenerholom , the contents of ganglioside , tsa lsa , and r - sa were determined . one - way anova was conducted to compare the biochemistry index of membrane glycolipids between the 2 groups . kunming mice ( normal mice ) were obtained from the animal center at fu - dan university shanghai medical college . mice containing the ascites - form transplantable hepatoma were obtained from the cancer institute of jiangsu province . the ascites was drawn from the transplantable hepatoma mice by a sterilized injector and placed into bacteria - free 0.9% nacl for dilution ( 1/3 , v / v ) . subsequently , the normal mice were inoculated with 0.2 ml of diluted cells under the skin in the foreleg and armpit regions . the tumors were allowed to grow for 13 days . usually , solid tumors weighing 12 g were found afterwards . the animals were injected with 5-fu ( 0.2 mg/0.2 ml / day ) i.p . at 48 h after tumor cell transplant . in the control group the animals were injected with a sterilized solution of 0.2 ml of 0.9% nacl instead of 5-fu . a third group of animals , raised without hepatoma transplant or any other form of treatment , was used as the normal group . after the administration of 5-fu or saline , the animals were sacrificed , and tissue and blood samples were collected for examination . based on the method of sevenerholom , the contents of ganglioside , tsa lsa , and r - sa were determined . one - way anova was conducted to compare the biochemistry index of membrane glycolipids between the 2 groups . in evaluating the ability of antimetabolic drug 5-fu to inhibit tumor growth , we found that the weight of tumors was significantly different between the 2 groups ( p<0.05 ) ( table 1 ) . ganglioside content in hepatoma tissue was significantly different among the 3 groups ( p<0.01 ) , and those in the therapeutic group were significantly lower than in the control group ( p<0.01 ) . in the plasma of the hepatoma mice , the tsa and lsa contents were higher in in the control group than in the normal group ( p<0.05 ) , but the tsa and lsa content in the therapeutic group was lower than in the control group ( table 3 ) . the tsa and lsa contents in the plasma of the hepatoma mice were higher in the control group than in the therapeutic and normal groups ( p<0.05 ) . no significant difference was found in the r - sa between the therapeutic and control groups . percent inhibition was calculated from the following equation : [ ( tumor weight of control group tumor weight of the therapeutic group)/tumor weight of the control group ] 100% . * * p<0.05 . to observe the ability of antimetabolism drug 5-fu in the inhibition of tumor growth are showed in table 4 , the transplanted hepatoma group weight of tumor was significance higher than that of normal control group ( p<0.05 ) . therapeutic group , control group and normal group membrane content variety the ganglioside content in hepatoma tissue from the control group was significantly higher than that in normal mouse liver ( p<0.01 ) , whereas those in the therapeutic group was significantly lower than in the control group ( p<0.01 ) . in the plasma of the hepatoma mouse , the tsa and lsa content was significantly higher in the control group than the normal group ( p<0.05 ) , and the therapeutic group was significantly lower than the control group ( table 6 ) . the ability to produce transplantable hepatoma and the effect of 5-fluorouracil ( 5-fu ) in the inhibition of tumor growth are depicted in table 4 . our data reconfirm the ability of 5-fu to inhibit the proliferation of tumor cells by competitive inhibition of the synthesis of thymidine monophosphate . the mechanism could involve sialic acid , a family of acylated derivatives of neuragmic acid , which usually occurs as a terminal component at the nonreducing end of carbohydrate chains of glycolipids . cell surface and membrane components play a vital role in neoplastic behavior . increasing concentrations of sialic acid are common on the tumor cell surfaces of neoplasms . the carbohydrate moiety may have an influence on growth , as well as cell - to - cell interaction and the development of malignancy . the biochemistry index of membrane glycolipids may be considered as an ancillary indicator for judging therapy effect in transplanted hepatomas in mice .

backgroundthe aim of this study was to investigate the variety of plasma contents of membrane glycolipids in 65 gastrointestinal tumors and 31 transplant hepatomas in mice.material/methodsthe experimental model was a transplantable murine hepatoma . experimental mice were divided into 3 groups.resultsthe lsa and tsa content in the 2 groups were significantly difference ( p<0.01 ) , and were significantly lower in the therapeutic group than in the control group ( p<0.01).conclusionsthese results indicate that membrane glycolipids index lsa and tsa are sensitive markers in gastrointestinal tumors . in the transplanted hepatomas in mice , they may be considered as ancillary indicators for judging the therapeutic effect of hepatoma .

most cases of spontaneous rupture of the renal pelvis and ureter are associated with ureteral obstruction by calculi . although uterine cervical cancer is an exceedingly rare cause of renal pelvic or ureteral rupture , several cases have been reported ( singh et al . , 2009 , mcclinton et al . , 1989 , spurlock et al . , 1987 ) . the mechanism by which uterine cervical cancer causes spontaneous rupture of the renal pelvis and ureter has been suggested to involve obstruction of the ureter by tumor or swollen lymph nodes . we report a case of spontaneous ureteral rupture that occurred during concurrent chemoradiotherapy in a woman who had uterine cervical cancer associated with mild hydroureter without obstruction of the urinary collecting system . a 66 year - old woman with a diagnosis of uterine cervical cancer was referred to the gynecologic department of our hospital . pelvic examination showed a 5-cm cervical tumor with bilateral parametrial involvement , which had infiltrated to bilateral pelvic walls . histopathological examination revealed squamous cell carcinoma of the cervix , and federation of gynecology and obstetrics ( figo ) stage iiib disease was diagnosed . contrast - enhanced computed tomography performed 10 days before starting concurrent chemoradiotherapy showed a normal renal pelvis and right mild hydroureter ( fig . 1 ) . the serum creatinine and hemoglobin levels were 0.68 mg / dl and 12.9 g / dl , respectively . uniform bilateral enhancement of the renal cortex was seen on contrast - enhanced ct , indicating normal functions of both kidneys . moreover , contrast - enhanced ct and ultrasonography showed no stone in the ureter or renal pelvis . during the first week of concurrent chemoradiotherapy , 40 mg / m of cisplatin and 3 l of infusion solution were administered as an intravenous drip infusion , followed by 2 l of infusion solution on the next day . the patient 's body weight increased by 2 kg as compared with before treatment , and 10 mg of furosemide was given as an intravenous bolus injection . the serum creatinine and hemoglobin levels were 0.80 mg / dl and 13.3 g / dl , respectively . first , rupture of the renal pelvis and ureter can occur during concurrent chemoradiotherapy in the presence of uterine cervical cancer with ureteral stenosis . second , contrast - enhanced ct was more useful than ultrasonography for the diagnosis of this condition . first , our experience showed that rupture of the renal pelvis or ureter can occur during concurrent chemoradiotherapy in patients who have uterine cervical cancer with hydroureter . this is most likely attributed to the fact that the lower ureter has three layers of myometrium , whereas the upper ureter has only two layers . although tumors rarely cause rupture of the renal pelvis or ureter , several previous studies have reported that obstruction of the ureter by tumor or swollen lymph nodes is a potential cause ( singh et al . , 2009 , mcclinton et al . , 1989 , spurlock et al . , 1987 we confirmed that the ureter was not obstructed when the double - j stent was inserted after ureteral rupture . in the presence of normal kidney function with partial obstruction of the ureter however , hydration does not lead to increased urine production in patients who have decreased renal function with complete obstruction of the ureter . the renal rupture in our patient was apparently caused by the rapid elevation of intrapelvic pressure due to hydration and treatment with furosemide . to our knowledge , this is the first reported case of ureteral rupture occurring during concurrent chemoradiotherapy in a patient who had uterine cervical cancer without obstruction of the urinary collecting system . our second important finding was that contrast - enhanced ct was more useful than ultrasonography for diagnosis immediately after ureteral rupture had occurred . several case reports have documented that renal rupture complicated by retroperitoneal abscess can lead to sepsis ( hadar and servadio , 1979 , lin et al . intravenous pyelography can confirm dilation of the ureter and renal pelvis , as well as perirenal extravasation of contrast medium . although it is easy to diagnose renal rupture when we see a hyperechoic lesion and urine around the kidney , most ruptures of the renal collecting system are not associated with a hyperechoic lesion or urinoma immediately after rupture has occurred ( hwang et al . , 2000 , koktener et al . , 2007 ) . we diagnosed ureteral rupture on detecting the extravasation of contrast medium from the ureteral pelvis on contrast - enhanced ct . therefore , if we encounter a patient with dilation of the renal pelvis with acute abdomen during concurrent chemoradiation , spontaneous ureteral rupture should be kept in mind . one report documented rupture of the renal pelvis in a patient with a ureteral stone who was being observed while receiving hydration and analgesics ( tas et al . , 2013 ) . another study reported on a woman at 19 weeks ' gestation in whom rupture of the renal pelvis occurred after she received a rapid intravenous infusion before cervical cerclage during spinal anesthesia ( huang et al . , both cases of renal pelvic rupture were caused by a hydration - induced increase in urine flow under the condition of ureteral stenosis . in conclusion , rupture of the renal pelvis and ureter can occur during concurrent chemoradiotherapy in the presence of uterine cervical cancer with ureteral stenosis . we consider contrast - enhanced ct to be more useful than ultrasonography for the diagnosis of rupture of the renal pelvis or ureter . the early diagnosis of renal pelvic and ureteral rupture can prevent the development of severe complications , such as abscess . in women who have uterine cervical cancer with ureteral stenosis and normal renal function , the placement of a double - j stent before concurrent chemoradiotherapy may help to prevent rupture of the renal pelvis and ureter .

spontaneous rupture of the renal pelvis and ureter is associated with obstruction of the urinary collecting system , but is rarely caused by tumors . we describe our experience with a patient who had uterine cervical cancer with mild hydroureter in whom spontaneous ureteral rupture occurred during concurrent chemoradiotherapy . the patient was a 66-year - old woman with stage iiib uterine cervical cancer and mild hydroureter who received concurrent chemoradiotherapy . the patient felt uncontrolled right - side abdominal pain caused by ureteral rupture after she was given hydration and an intravenous bolus injection of furosemide during the first week of chemoradiotherapy . contrast - enhanced computed tomography was more useful than ultrasonography for diagnosis of the ureteral rupture . the ureteral rupture in our patient was attributed to a rapid rise in the pressure of the urinary collecting system caused by hydration and the bolus injection of furosemide . placement of a double - j stent before starting concurrent chemoradiotherapy may help to prevent ureteral rupture in patients who have uterine cervical cancer with mild hydroureter .

infarct of the anterior spinal artery is the most common subtype of spinal cord infarct , and is characterized by bilateral motor deficits with spinothalamic sensory deficits . we experienced a case with atypical anterior - spinal - artery infarct that presented with bilateral hand weakness but without sensory deficits . a 29-year - old man presented with sudden neck pain and bilateral weakness of the hands . magnetic resonance imaging ( mri ) of the brain did not reveal any lesion . his motor symptoms improved rapidly except for mild weakness in his left wrist and fingers . magnetic resonance angiography showed proximal occlusion of the left vertebral artery ; a spine mri revealed left cervical cord infarction . bilateral or unilateral hand weakness can be the sole symptom of a cervical cord infarct . spinal cord infarction is much less frequent than cerebral infarction , accounting for only 1% of all strokes.1 the anterior spinal artery supplies the anterior two - thirds of the spinal cord via the sulcal ( central ) artery . this usually results in anterior spinal artery infarcts presenting with profound bilateral motor deficits , sensory disturbances , and spinothalamic sensory deficits.2,3 although the mechanisms are not completely understood , there are some case reports of restrictive clinical syndromes , such as the ' man - in - the - barrel syndrome ' and the sulcal artery syndrome.4,5 here we describe a patient who presented with bilateral hand weakness but without sensory deficits due to left vertebral artery occlusion . a healthy 29-year - old man without any vascular risk factors presented with sudden neck pain and bilateral hand weakness . after flexing his neck while tying his shoelaces , the patient experienced a sudden , severe pain ( with a maximum score on a visual analog scale ) that started in the posterior neck and spread rapidly across the entire head . bilateral hand weakness that followed the pain onset prevented the patient from tying his shoelaces . the patient 's vital signs were stable upon hospital admission , except for markedly elevated blood pressure ( 202/108 mm hg ) . the initial neurological examination revealed weakness in both flexion and extension of the wrists and fingers bilaterally , and during left leg extension [ medical research council ( mrc ) grade 4 ] . however , the deep tendon reflexes were normal and the patient denied hypoesthesia in any sensory modality including pain , temperature , proprioception , and vibration . laboratory findings were normal , and chest radiography did not reveal any pathology ; an electrocardiogram also produced no evidence of ischemia or arrhythmia . his motor symptoms improved rapidly within 1 day except for mild weakness of his left wrist ( mrc grade of flexion / extension iv / iv ) and fingers ( mrc grade of flexion / extension iv / iv ; slightly more severe in the 5th finger ) . brain computed tomography ( ct ) and brain magnetic resonance imaging ( mri ) did not reveal any signal abnormalities or mass lesions . however , ct aortography identified severe stenosis and occlusion of the left vertebral artery , and a pseudo lumen with mural thrombi was suspected on cervical mri ( fig . mri of the cervical segment produced high signal intensities in the left gray matter of spinal cord at the c3 , c4 , and c6 levels on diffusion - weighted images ( fig . magnetic resonance angiography performed 3 months after symptom onset showed persistent occlusion of the left vertebral artery . unique features of this spinal - cord - infarct patient were bilateral hand weakness , absence of sensory deficits , and rapid improvement of motor symptoms in the right hand and left leg . weakness of both arms has been reported previously in association with cervical cord infarcts , typically also with spinothalamic sensory deficit and vertebral artery occlusion.4,5,6,7 the mechanism that preserves leg strength in these cases is most likely collateral flow from the surrounding pial plexus.4 nevertheless , weakness of both hands without sensory deficits is rare in infarcts of the cervical spinal cord , and its mechanism remains to be determined.7,8 the anterior spinal artery forms at the level of the foramen magnum from the branches of the vertebral artery , and gives rise to the sulcal ( central ) arteries that penetrate the right and left sides of the spinal cord.3 unlike circumferential areas of the spinal cord , its interior has no anastomosis and the sulcal arteries are essentially end arteries . therefore , the weakness of both hands in the present patient suggests that the end - arteries zone or the zone bordering the sulcal artery and circumferential artery corresponds to a region where hand motor neurons are located bilaterally.8 this would include the ventrolateral to lateral regions within the gray matter of the lower cervical spinal cord ( fig . 3).9,10 alternatively , the hand motor areas may be more vulnerable than the more proximal arm motor areas . the hand motor area occupies a relatively large proportion of the cortical homunculus , so the hand motor area may occupy a larger area in the spinal gray matter , or require a larger blood supply.11,12 finally , the isolated unilateral hand weakness of this patient may be related to variation in the branches of the sulcal artery . successive sulcal arteries generally alternate in their distribution to the left or right side of the spinal cord , but not both ; this would make unilateral involvement or improvement possible.5 sensory deficit was absent in the present case . all subtypes of spinal cord infarcts are presumed to have sensory deficits , and therefore a pure motor presentation can result in misdiagnosis.2,3 anterolateral involvement could explain the present atypical presentation , and bilateral presentation without facial weakness may indicate a spinal cord infarct.5 transient ischemic attacks in the spinal cord reported occur primarily in the cervical cord , and recovery depends on the severity of the initial involvement and its collateral flow.1,2,6 cervical lesions are generally less vulnerable to ischemic insults than are thoracic or lumbar lesions due to the intensive vascularization from the vertebral and radicular arteries.3,6,13 the rapid improvement in the present case might therefore have resulted from initial distal involvement and collateral flow from the intact right vertebral artery . in conclusion , transient bilateral hand weakness can be the initial symptom of a spinal cord infarct , and unilateral hand weakness can be the sole symptom of a cervical cord infarct .

backgroundinfarct of the anterior spinal artery is the most common subtype of spinal cord infarct , and is characterized by bilateral motor deficits with spinothalamic sensory deficits . we experienced a case with atypical anterior - spinal - artery infarct that presented with bilateral hand weakness but without sensory deficits.case reporta 29-year - old man presented with sudden neck pain and bilateral weakness of the hands . magnetic resonance imaging ( mri ) of the brain did not reveal any lesion . his motor symptoms improved rapidly except for mild weakness in his left wrist and fingers . magnetic resonance angiography showed proximal occlusion of the left vertebral artery ; a spine mri revealed left cervical cord infarction.conclusionsbilateral or unilateral hand weakness can be the sole symptom of a cervical cord infarct .

bowel endometriosis occurs in approximately 10% of all patients with endometriosis and usually arises from the rectum and sigmoid colon at a rate of approximately 80% . conservative treatment with hormonal therapy is usually performed . however , if the patient hopes to become pregnant , an alternative to long - term hormonal therapy must be found . we report a case of lower rectal endometriosis performed using laparoscopically assisted low anterior resection of the lower rectum . a 34-year - old woman presented with pain during menstruation and was diagnosed with endometriosis of the lower rectum . despite treatment with an lh - rh agonist , she was unable to become pregnant and surgical removal of her endometriosis was recommended . on admission , tumor markers such as cea , ca-125 and ca-19 - 9 were 1.7 ng / ml , 31.9 u / ml and < 2.0 u / ml , respectively . on lower gastrointestinal series , poor extensibility of the lower rectum with irregular elevation and mucosal irregularity was observed . the portion of the rectum involved with endometriosis was approximately 35 mm from the anal verge ( fig . colonoscopic findings showed tumor - like submucosal tumor in the anterior wall of the lower rectum ( fig . 2 ) . abdominal computed tomography findings revealed a 2-cm mass without contrast enhancement in the lower rectum . concerning abdominal magnetic resonance imaging ( mri ) , a mass which was slightly enhanced and demonstrated mass effect on the surrounding organs was shown on gadolinium - enhanced t1-weighted images ( fig . there were adhesions to the right lower abdominal cavity , and a small amount of ascites was found in the pouch of douglas . exfoliation was started from the presacral region to the right side of the rectum and the rectococcygeus ligament was cut . exfoliation was continued to the levator ani muscle and mobilization of the left side of the rectum was performed . in front of the rectum , exfoliation was performed from the posterior wall of the vagina , but because the layer of exfoliation was unclear and tissue was solid around the tumor , the exfoliation was difficult . since the left hypogastric nerve was involved by inflammation surrounding the tumor , the nerve was cut , and subsequently the left lateral ligament was cut . after the inferior border of the tumor was confirmed by colonoscopy , the rectum was cut 1 cm distal to the inferior border of the tumor . then a 3-cm skin incision was made in the lower abdomen , the specimen was pulled up and the rectum was cut 5 cm distal to the superior border of the tumor . postoperatively , a rectovaginal fistula occurred on the 9th postoperative day , and an ileostomy was performed . after that , the patient 's postoperative course was uneventful and she left the hospital on the 25th postoperative day ; 4 months later the ileostomy was closed . endometriosis affects approximately 15 - 20% of women of child - bearing age , and bowel endometriosis occurs in 5% of all endometriosis cases . athmanathan et al . reported that 80% of the cases of bowel endometriosis arise from the rectum and sigmoid colon . symptoms of rectal endometriosis include dysmenorrhea in 57 - 85.3% of patients , dyspareunia in 55 - 57% , rectal pain in 41.2% , rectal bleeding in 14% , and tenesmus in 8.8% . in our case , the patient had rectal pain during menstruation . physical examination , computed tomography , mri and endorectal sonographic evaluation are useful in making the diagnosis . however , the sensitivity of mri for the diagnosis of colorectal endometriosis is much higher , at between 80 and 92.6% with a positive predictive value of approximately 89% . endorectal sonographic evaluation is noninvasive and its sensitivity and positive predictive value are both 100% . endorectal sonography was indicated in our case due to its high feasibility and lower cost compared to mri . significant resistance to the surgical treatment of colorectal endometriosis currently exists because of major complications such as colo- or rectovaginal fistulae . reported that rectovaginal fistulae occurred in 10.3% of patients . on the other hand , fleisch et al . reported the utility of surgical treatment for patients with persistent pain and found that 21 ( 91.3% ) of 23 patients reported improvement in their symptoms . for patients who desire to become pregnant , long - term hormonal therapy for endometriosis is contraindicated ; therefore , surgery is needed . bailey et al . reported that the pregnancy rate following surgery was 49% , strongly supporting the surgical treatment of rectal endometriosis . reported that all the items on the sf-36 health status and quality of life scores were significantly improved after laparoscopic colorectal resection for endometriosis . reported that the probability of requiring further surgery was 36% in patients with deep endometriosis , and furthermore , 38.6% of patients had residual or recurrent endometriosis . reported that the incidence of adhesion - related obstruction after laparoscopic colorectal surgery was 1.3% , which was lower than for open laparotomy ; therefore , laparoscopic surgery for colorectal endometriosis is recommend if further surgery is needed . for patients whose lesions are located on the lower part of the rectum , exfoliation is needed up to the levator ani muscle ; therefore , the laparoscopically assisted approach is more useful in providing a good operative field compared to open laparotomy . in our case , a rectovaginal fistula occurred on the 9th postoperative day and ileostomy was required , but intraabdominal adhesions were mild and ileostomy was performed easily using a laparoscopic approach . in conclusion , for patients with lower rectal endometriosis for whom hormonal therapy is contraindicated , laparoscopically assisted low anterior resection is recommended for its visual amplification effect , minimal invasiveness and palliation of symptoms .

a 34-year - old woman presented with pain during menstruation and was diagnosed with endometriosis of the lower rectum . despite treatment with an lh - rh agonist , she was unable to become pregnant and surgical removal of her endometriosis was recommended . preoperative magnetic resonance imaging revealed endometriosis localized between the neck of the uterus and rectum with indentation and scuffing . laparoscopically assisted low anterior resection was performed . exfoliation was started from the right side of the rectum to the presacral and retrorectal space , and the rectococcygeus ligament was transected . exfoliation of the retrorectal space was continued to the levator ani muscle and mobilization of the right side of the rectum was performed . in front of the rectum , exfoliation was started posterior to the wall of the vagina , but layers became unclear near the tumor as the tissue was solid in this region . the left hypogastric nerve close to the tumor was inflamed and it was cut . the layer of the exfoliation was connected to the right side of the rectum , the tumor was isolated from the vagina , and the lower rectum was transected at a point 1 cm distal to the tumor with a 60-mm linear stapler . reconstruction with a 31-mm circular stapler was performed using the double stapling technique . operative time was 520 min with a blood loss of 320 ml . on the 9th post operative day , a rectovaginal fistula occurred , and ileostomy was performed . the patient was discharged from the hospital on the 25th postoperative day , and 4 months later , stoma closure was performed .

progressive neurologic deterioration or back and leg pain in children with a history of myelomeningocele ( mmc ) surgery may be due to cord tethering or growth of an intradural inclusion tumor . loss of motor function in the lower extremities , gait abnormality , back or leg pain , scoliosis , and foot deformity are common findings in postsurgical tethering . here , we report a child who developed progressive neurological deterioration and severe back and leg pain subsequent to large infected dermoid tumor extending underneath the skin to the spinal canal and intramedullary area . a 3-year - old boy was referred to neurosurgical department because of severe back and leg pain and motor regression . he was a known case of lumbar mmc and was operated at 8 months of age . he could sit at 11 months of age and stand at age of 2 years on his knees , but could not move the distal part of his legs at all . his back pain had started 3 months ago , which was severe at night and made him unable to sit . subsequently , he experienced severe leg pain , which prevented him from moving the proximal part of his lower extremities and his mother faced difficulty while cleaning him after urination or defecation . on physical examination , the child was afebrile , but very irritable , with good mental performance , and normal neurological examination of upper limbs . both lower extremities had lost any movement in proximal and distal parts ( 0/5 ) . a small tender bulging was identified in his back under the mmc repair incision for one week before admission , but no dermal sinus was found inside or around the incision . spinal magnetic resonance imaging ( mri ) was performed one month before surgery , which revealed cord tethering ( cord at l4- l5level ) . distal cord was attached to a mass , which was isointense in t1- and hypointense in t2-weighted images . it was extending from distal cord through the bony defect to the extraspinal canal space . the cord was dilated from the level of tethering to l1level with a lesion , which was isointense to hypointense in t1- and hyperintense in t2-weighted images , dissimilar to the intensity of cerebrospinal fluid [ figures 1 and 2 ] . notice the hypointense signal inside the expanded cord compatible with infected dermoid tumor found during surgery hypointense mass in t2-weighted image is extending from distal cord through the bone defect to the extraspinal canal space and the prior mmc surgery field the child underwent surgery with the suspicion of tethered cord associated with an inclusion tumor . in the prone position , the prior midline vertical incision was opened . during the dissection of subcutaneous tissue , dura mater , cord and dermoid tumor were attached to each other at the depth of previous surgical field , and the tumor was extending from the extraspinal area to the intramedullary space between l4 and l1 vertebral levels . microsurgical release of all intradural adhesions was performed to achieve circumferential untethering of spinal cord associated with gross total resection of intramedullary mass . complete excision of the dermoid cyst and capsule were performed , which was followed by primary closure of the dura . on histopathological examination , specimen had the characteristic features of a dermoid cyst , with simple squamous epithelium , hair follicles , and cholesterol - containing debris . he could move proximal part of his lower extremities with score of 1 - 2/5 , but was unable to stand on his knee as he could do before . children with mmc are born with a wide range of neurological deficits that will remain after primary repair of lesion . any new changes in the previous neurological , urological or orthopedics status warrant an extensive investigation to rule out the possible causes , especially tethered cord syndrome . tethered cord is a common complication following mmc surgery , which may be found in most patients when they are evaluated with spinal mri , but symptomatic tethering occurs only in 10 - 30% of them . tethered cord may present with subtle changes in prior neurophysical situation , which become progressive and disabling if neglected . spinal cord tethering due to dermoid cyst subsequent to mmc surgery has been reported so far , which can occur decades after closure.[58 ] all patients with symptomatic tethered cord have attachment of spinal cord to the prior closure site , which can be associated with inclusion tumors in 16% of instances . dermoid tumors following mmc surgery may result from inadequate excision of dermal elements during dissection of placode or dura mater from the skin or with implantation of dermal elements inside the repair site . , this tumor can be an associated abnormality with mmc , which was undiagnosed or neglected because of very small size at the time of primary repair , especially during surgery without magnification . cases of dermoid tumor in mmc children who were operated during the intrauterine period have been reported , suggesting a higher potential for rapid growth of this tumor in lesions that were closed in utero rather than after birth . dermal sinus tract as a rare congenital abnormality is accompanied by inclusion tumors in 43% of instances , wherein most tumors are dermoids . intramedullary spinal abscess without sinus tract is extremely rare , but a devastating condition in children.[1216 ] intramedullary abscess and dermoid tumor without dermal sinus have been reported before ; however , not following mmc repair and especially not associated with tethered cord . in order to explain infected dermoid tumor in this patient with prior mmc repair , we propose several explanations implantation of bacteria during closure of mmc sac can lead to abscess formation but occurence three years after the first surgery seems to be unusual . microscopic sinus tract through the preceding mmc surgery incision can be a way for bacteria to reach the subcutaneous space . hematogenous superinfection of the dermoid cyst during repeated urinary tract infections in mmc patient is another possiblity for abscess formation in this case . dermoid tumors , even infrequent , should be considered in the differential diagnosis of the causes of neurological deterioration in patients with a history of mmc repair . along with tethered cord syndrome , dermoid tumor can complicate the prior neurological and physical status of the child , and should be searched vigilantly during evaluation of these patients . because of rapid growth of dermoid tumors and associated infection , these can deteriorate the previous neurological status in a more aggressive and destructive manner as compared to tethered cord per se . awareness of such pathology in patients with previous history of myelomeningocele repair , early diagnosis and surgical intervention can provide better result with less disability . tethered cord syndrome should be investigated in any patient with a history of mmc repair if one finds neurological , orthopedics , or urological deterioration . dermoid tumor is considered as an important differential diagnosis during thorough evaluation of the causes of worsening . in order to minimize the risk of dermoid tumor development following mmc surgery , any residual epidermal or dermal elements the operative field should be explored accurately proximal and distal to the sac to find any associated anomaly or minute inclusion tumor and prevent late inclusion tumor formation and tethered cord .

intramedulary dermoid tumors are rare tumors mostly found associated with dermal sinus tracts . spinal dermoid tumor can occur after myelomeningocele repair . infected dermoid tumors are reported in patients with dermal sinus tracts as well , but have never been reported subsequent to myelomeningocele surgery . here , we report a rare association of infected dermoid tumor with tethered cord without dermal sinus tract in a child who had been operated for myelomeningocele during infancy .

sialolithiasis is the most common disease of salivary glands caused by the obstruction of a salivary gland or its excretory duct by a calculus . it may occur at any age but there is a peak incidence in fourth , fifth , and sixth decades . majority of salivary calculi ( 8095% ) occur in the submandibular gland , whereas only 520% are found in the parotid gland . sialoliths located in the duct are usually elongated , while those situated in the gland or hilus tend to be round or oval . the size of the salivary calculi may vary from < 1 mm to a few cms in largest diameter . most of the calculi ( 88% ) are < 10 mm in size , whereas only 7.6% are larger than 15 mm . they consist of mainly calcium phosphate with smaller amounts of carbonates in the form of hydroxyapatite , with smaller amounts of magnesium , potassium , and ammonia . a 45-year - old male reported to the outpatient department of vspm 's dcrc , nagpur , with the chief complaint of pain and swelling in the floor of mouth on the left side since 1 month . detailed history revealed that it has started as a small swelling which used to increase before meals 1 month back . intraoral examination revealed a well - defined elongated swelling of approximately 2.5 cm 1 cm in size in the floor of the mouth in relation to lower left incisors to first molar region . overlying mucosa was inflamed and the swelling was hard in consistency and tender on palpation [ figure 1 ] . radiographic evaluation included cross - sectional mandibular occlusal view which revealed a large well - defined elongated homogenous radio - opacity in the floor of the mouth on the left side in relation to lower left canine to first molar region . it was approximately 2.5 cm 1.5 cm in size [ figure 2 ] . swelling in the floor of mouth on the left side mandibular occlusal radiograph showing submandibular salivary duct calculus on the basis of clinical and radiological findings , a diagnosis of left submandibular duct sialolith was made . as it was a large sialolith , we elected to remove the sialolith surgically under local anesthesia [ figure 3 ] . the sialolith removed measured 25 mm ( i.e. 2.5 cm ) in length [ figure 4 ] . removal of the sialolith from wharton 's duct the removed sialolith measured 2.5 cm 1.5 cm in its largest dimension the sialolith was in the wharton 's duct and the patient had pain before and during meals . for stone formation it is likely that intermittent stasis produces a change in the mucoid element of saliva , which forms a gel . this gel produces the framework for deposition of salts and organic substances creating a stone . traditional theories suggest that the formation occurs in two phases : a central core and a layered periphery . the central core is formed by the precipitation of salts , which are bound by certain organic substances . another theory has proposed that an unknown metabolic phenomenon can increase the saliva bicarbonate content , which alters calcium phosphate solubility and leads to precipitation of calcium and phosphate ions . a retrograde theory for sialolithiasis has also been proposed . aliments , substances , or bacteria within the oral cavity might migrate into the salivary ducts and become the nidus for further calcification . salivary stagnation , increased alkalinity of saliva , infection or inflammation of the salivary duct or gland , and physical trauma to salivary duct or gland may predispose to calculus formation . submandibular sialolithiasis is more common as its saliva is ( i ) more alkaline , ( ii ) has an increased concentration of calcium and phosphate , and ( iii ) has a higher mucous content than saliva of the parotid and sublingual glands . in addition , the submandibular duct is longer and the gland has an antigravity flow . sialolithiasis typically causes pain and swelling of the involved salivary gland by obstructing the food - related surge of salivary secretion . calculi may cause stasis of saliva , leading to bacterial ascent into the parenchyma of the gland and therefore infection , pain , and swelling of the gland at meal time . bimanual palpation of the floor of the mouth , in a posterior to anterior direction , reveals a palpable stone in a large number of cases of submandibular calculi formation and a uniformly firm and hard gland suggests a hypofunctional or nonfunctional gland . other traditional diagnostic methods include sialography , ultrasound , computed tomography , and scintigraphy for sialoliths . as the lesions were observed clearly in occlusal radiographs , no further investigations were performed for diagnosis . the treatment objective for giant sialoliths , as for the standard - sized stones , is restoration of normal salivary secretion . there are three ways in which we can treat patients with salivary stones : removal through the oral cavity , interventional sialoendoscopy , and resection of the gland . our choice depends on the site , size , shape , number , and quality of the stones . the giant sialolith should be removed in a minimally invasive manner , via a transoral sialolithotomy , to avoid the morbidity associated with sialadenectomy . whenever the stone can be palpated intraorally , it is best to remove it through an intraoral approach . for giant sialoliths , transoralsialolithotomy with sialodochoplasty or sialadenectomy submandibular gland removal is indicated if ( 1 ) the gland has been damaged by recurrent infection and fibrosis , ( 2 ) there is a stone of substantial mass within the gland itself that is not surgically accessible intraorally , ( 3 ) there are small stones present in the vertical portion of wharton 's duct from the comma area to the hilum , ( 4 ) the size of an intraglandular stone reaches 12 mm or more as the success of lithotripsy may be < 20% in such cases . there are various methods available for the management of salivary stones , depending on the gland affected and stone location . transoral sialolithotomy remains mainstay of the treatment for giant sialolith in the duct of submandibular gland . also , patients should be followed up regularly as recurrence has been reported in the literature .

salivary gland calculi account for the most common disease of the salivary glands . most of the salivary calculi are small in size . some calculi that reach several centimeters are reported as megaliths or giant calculi in the literature . they may occur in any of the salivary gland ducts but are most common in wharton 's duct and in the submandibular gland . this report presents clinical and radiographical sign of an unusually large sialolith . a patient came with pain in the floor of mouth . there was a swelling on floor of mouth on the left side . radiographical examination revealed large irregular radio - opaque mass superimposed on left lateral incisor to molar areas . this case report describes a patient presenting with an unusually large submandibular gland duct sialolith , the subsequent patient management , the etiology , diagnosis , and its treatment .

pain in the upper trapezius ( ut ) region may be caused by the performance of repetitive tasks or continuous weight on the shoulder1 , 2 . brassieres may be a factor contributing to ut pain in women because the weight of the breast causes the brassiere strap to press on the ut muscle4 , 5 . from their teenage years on , women continuously wear brassieres on a daily basis6 , 7 . in most cases , the brassiere has parallel straps that go over the shoulders from front to back8 . the weight of the breasts causes the parallel straps to cut into the outer shoulder and lengthen the ut muscle , causing pain5 . excessive ut muscle activation resulting from continuous weight on the ut region generates myofascial trigger points that cause pressure pain9 . generally , clinicians concerned about upper trapezius region pain have overlooked whether or not wearing a brassiere leads to upper trapezius region pain . in addition , previous studies have suggested that wearing a brassiere may be a factor contributing to ut region pain due to the weight of the breasts4 , 5 , 9 . however , the influence of wearing a brassiere on the muscle activity of ut has not been scientifically proven . therefore , the purpose of the present study was to examine the effect of wearing a brassiere on ut region pain and muscle activity during arm elevation by women . fourteen female with ut region pain aged 25 to 47 years volunteered for this study . inclusion criteria were history of ut region pain for at least 6 weeks and a visual analog scale ( vas ) score > 5 ( severe pain ) at rest . exclusion criteria included past or present neurological pain , cervical spine fractures , radiating pain to an upper limb , and a history of unresolved cancer . the subject s mean age was 34.12 11.62 years , and their mean height and weight were 158.73 4.49 cm and 54.65 6.23 kg , respectively . all subjects read and signed an informed consent form approved by the inje university ethics committee for human investigations prior to their participation in this study . surface electromyography ( emg ) data were recorded using a delsys trigno wireless emg system ( delsys , inc . , emg data were collected from the right side ut muscle ( approximately half the distance between the seventh cervical spinal process and the acromion ) . sampling was performed at 1,000 hz , with a bandwidth of 20450 hz , and the root mean square was calculated using emg works 4.0 analysis software ( delsys , boston , ma , usa ) . each maximum isometric contraction maneuver was performed twice for 5 s , and the average muscle activity of the middle 3 s of the two trials was used to normalize the data . pressure pain in the ut region was measured using a baseline dolorimeter ( pain diagnosis and treatment , inc . , great neck , ny , usa ) . the instrument consists of a gauge attached to a hard rubber tip 1 cm in diameter . the dial gauge can be calibrated in kilograms ( kg ) or pounds ( lb ) , with a range of 130 kg or 160 lb . at intervals of 0.25 kg or 0.5 lb . inter - examiner reliability for the baseline dolorimeter is good to excellent ( interclass correlation coefficient= 0.750.89)11 . prior to testing , the subjects were instructed to indicate when the pressure point was painful . to measure the pressure pain in the ut , the subjects were instructed to sit upright on a chair with their feet on the floor looking straight ahead . the examiner stood and measured pressure pain in the middle of the muscle belly between c7 and the acromion . the right and left sides were each measured three times , and the average was calculated . the subjects were instructed to perform shoulder flexion with scaption . during upper limb elevation , the scaption plane was controlled using a vertical bar . subjects were asked to hold the right side shoulder at an angle of 120 degrees for 5 s. the middle 3 s of muscle activity averaged over three trials was used in the analysis . the pressure pain and emg activity were measured with and without wearing of a brassiere in a randomized order . ut muscle activity and pressure pain between with and without wearing brassiere were compared using the paired t - test . statistical analyses were performed using spss ( ver . 17.0 ; spss , chicago , il , usa ) . the emg signal amplitude ( % mvic ) of ut increased significantly ( mean sd , 50.87 8.92 compared to 39.79 7.08 ) when wearing a brassiere compared to the no brassiere condition in the females with ut region pain ( p < 0.05 ) . the ut pressure pain did not differ between two conditions ( p > 0.05 ) . previous studies have suggested that wearing a brassiere may be a factor contributing to ut region pain in women9 , 10 . however , no studies have reported the influence of wearing a brassiere on the emg activity of ut . to the best of our knowledge , this is the first study to demonstrate that a brassiere can increase ut muscle activity during shoulder scaption in women with upper trapezius region pain . our results shows there was a significant 21.7% increase in ut muscle activity when wearing a brassiere . a brassiere transfers the weight of the breast from the pectoral fascia to the ut region , generating a downward force on the shoulder12 . to counterbalance the downward force , greater ut contraction is required to elevate the shoulder , resulting in increased ut muscle activity moreover , shoulder scaption resulted in greater contraction of the ut muscle due to the increased leverage of the arm in this study . sustained and repeated ut muscle contraction would quickly generate ut muscle fatigue , resulting in ut tenderness10 . therefore , when wearing a brassiere , the ut sustains the small weight of the breast with ischemia induced by the brassiere , which would lower the pressure pain threshold of ut . a previous study suggested that brassiere removal during a day ( 24 hours ) was effective at reducing ut and pectoral girdle muscle pain12 . however , in this study , the pressure pain threshold showed no significant difference between the brassiere and no brassiere conditions , because subjects were asked to remove the brassiere for just one minute for no brassiere condition . further study is needed to investigate the effects of brassiere removal during the day on the ut muscle activity and pressure pain threshold of the ut region in women with upper trapezius region pain . the investigation and comparison of various designs of brassiere on the ut muscle activity and pain threshold is also required . a limitation of this study was that we did not measure the breast weight or size of the subjects ; different sizes of breast may affect the pain intensity of ut . also , we did not control for the fit of the brassiere that each participant wore . in conclusion , wearing a brassiere can increase ut muscle activity , so clinicians should consider recommending brassiere removal for as long as possible , or suggest wearing a well - fitting and supportive brassiere when managing women with upper trapezius region pain .

[ purpose ] this study examined the effect of wearing a brassiere on upper trapezius ( ut ) region pain and emg activity during arm elevation by women . [ subjects and methods ] fourteen healthy women were recruited . surface emg data were collected from the ut muscles during arm elevation . pressure pain in the ut region was measured using a baseline dolorimeter . [ results ] the emg activity of the ut increased significantly when a brassiere was worn compared to without . ut region pain showed no significant difference between with and without wearing a brassiere . [ conclusion ] this suggests that wearing a brassiere increases the muscle activation of the ut in women .

sodium valproate is a frequently used drug for the treatment of seizure disorders , bipolar disease and chronic pain . it is known to cause a serious complication , hyperammonemic encephalopathy which is characterized by acute onset of impaired consciousness , headache , vomiting , seizures , ataxia , generalized slowing on electroencephalography and rapid recovery after discontinuation . in this submission , we present a case of valproate - induced hyperammonemic encephalopathy where the major manifestation was restricted to bilateral cerebellar clinical features . a 19-year - old male presented with acute onset tremulousness , staggering gait and difficulty in walking and speech . he was a known case of idiopathic generalized epilepsy treated initially with carbamazepine ( 800 mg / day ) and recently switched over to sodium valproate ( 1000 mg / day ) before the onset of this acute complication . the patient showed bilateral cerebellar signs in the form of defective coordination in both upper and lower limbs with severe degree of gait ataxia and scanning speech . investigations including hemogram , liver function tests , renal function parameters , serum electrolytes and blood sugar showed normal results . thyroid function tests ( t3 , t4 , thyroid stimulating hormone ) and nutritional parameters including vitamin b12 levels were also found in normal range . however , serum ammonia level was raised to 106 mol / l ( normal range 12 - 47 mol / l ) . the levels of serum valproate and carbamazepine were found to be in the normal range ( serum valproate : 52 ug / ml [ range : 50 to 100 gm / ml ] and serum carbamazepine < 1.25 gm / ml [ range : 4 to 12 gm / ml ] ) . patient underwent magnetic resonance imaging of cranium , which turned out to be normal . with this background of clinical observations and laboratory assessment , a diagnosis of valproate - induced hyperammonemic encephalopathy the valproate was replaced by levetiracetam ( 500 mg ) two times a day along with carnitine supplementation . the patient showed rapid recovery and was able to walk without support after a week . his serum ammonia level normalized on repeat serum ammonia estimation ( 20 mol / l ) . valproic acid by virtue of its broad spectrum of action has application in various disorders like epilepsy , bipolar disorders , prophylaxis and treatment of migraine and neuropathic pain . although valproate - induced hepatic dysfunction leading to encephalopathy is a well - recognized entity , less commonly the drug can also produce an encephalopathy of non - hepatic origin by producing hyperammonemia , and is called as valproate - induced non - hepatic hyperammonemic encephalopathy ( vnhe ) . other causes of non - hepatic hyperammonemia include inborn errors of metabolism ( urea cycle enzyme defects , fatty acid oxidation defects ) , drugs like 5-fu , asparaginase , salicylate , halothane and enflurane , hematologic diseases like multiple myeloma and acute myeloblastic leukemia , hyperinsulinemia , hyperglycemia , distal renal tubular acidosis , parenteral nutrition and reye 's syndrome . the clinical presentation of hyperammonemic encephalopathy constitutes irritability , drowsiness , coma and occasionally these patients have paradoxical seizures . the presence of significant hyperammonemia with cerebellar signs and reversal of hyperammonemia , encephalopathy and disappearance of cerebellar signs and symptoms after discontinuation of sodium valproate confirmed the diagnosis of valproate - induced hyperammonemic cerebellar ataxia with metabolic encephalopathy . this view is also supported by generalized slowing in eeg suggestive of metabolic encephalopathy which also normalized after discontinuation of valproate . we estimated serum valproate and carbamazepine levels which were within normal limits although there is little correlation between valproate level and clinical effects because of the variable absorption rate and short half - life of the drug . in our study , there was decline in ammonia levels after withdrawal of valproate and subsequent disappearance of cerebellar symptoms and features of encephalopathy . there is scarcity of literature on unusual symptoms of valproate - induced hyperammonemic encephalopathy presenting as mainly cerebellar ataxia . cases have been reported where patients presented with acute onset of confusion , decline in cognitive abilities , and ataxia . we gave carnitine supplementation to our patient as valproate is known to enhance urinary excretion of l - carnitine , leading to depletion of its blood stores . thus we conclude that valproate - induced hyperammonemic encephalopathy can present mainly with cerebellar ataxia and a high index of suspicion is required for an early recognition , investigation , and treatment of this potentially life - threatening condition .

valproate - induced hyperammonemic encephalopathy is a rare event clinically characterized by impaired sensorium , vomiting , headache , seizures and focal neurological deficits . the pathogenesis of this dreadful complication is not well understood , although hyperammonemia has been implicated in causation of encephalopathy . in this submission , we have highlighted a case of valproate - induced encephalopathy who presented mainly with bilateral cerebellar features and generalized slowing on electroencephalogram . high index of suspicion of valproate - induced hyperammonemic encephalopathy is required if diffuse ataxia is present as it is a potentially reversible clinical disorder .

aneurysmal bone cysts ( abcs ) are rare entities which cause expansile and destructive bone lesions characterized by reactive proliferation of connective tissue1,5 ) . they are benign lesions and can occur in any part of the skeleton and the spine can be affected up to 30% of the cases . abcs can cause symptoms such as back and/or dorsal pain , neurological deficitis , and pathological fractures . although abcs mostly occur in the distal part of femur and/or proximal part of the tibia , some cases have been reported in pelvis and posterior elements2,4,5 ) . abcs can occur rarely in the sacrum and this location has some difficulties in treatment because of the relations with the sacral nerves . togetherness of abc and fibrous dysplasia , giant cell tumors and/or osteoblastomas are defined as secondary abc by some authors and this association was seen aproximately in 20 - 30% of cases . diagnosis can be verified by ct scans and mri . in this paper , authors reported two rare cases with thoracic and sacral aneurysmal bone cysts . a 14-year - old male admitted to neurosurgery department with suffering of being unable to walk for 15 days . thoracic mri images and ct scans demonstrated a mass which causes mass effect on spinal cord ( fig . 2 day he was mobilised and his neurological examination was enough for walking with arm holder on postoperative 7 day . on the 5 months control of the patient there were no suffering and no instable images on x - ray and mr images ( fig . an 8-year - old male pateint admitted to us with right hip and back pain . in his history , he had fallen down from 2 meters . spinal mri revealed abc on the right side of the 3 sacral vertebra with an enlargement of 2419mm(fig . the second case is under control and no surgical treatment is suggested because of the patient 's age and observing no neurological deficits at the patient . a 14-year - old male admitted to neurosurgery department with suffering of being unable to walk for 15 days . thoracic mri images and ct scans demonstrated a mass which causes mass effect on spinal cord ( fig . 2 day he was mobilised and his neurological examination was enough for walking with arm holder on postoperative 7 day . on the 5 months control of the patient there were no suffering and no instable images on x - ray and mr images ( fig . an 8-year - old male pateint admitted to us with right hip and back pain . in his history , he had fallen down from 2 meters . spinal mri revealed abc on the right side of the 3 sacral vertebra with an enlargement of 2419mm(fig . the second case is under control and no surgical treatment is suggested because of the patient 's age and observing no neurological deficits at the patient . known as a non - neoplastic expansile bone lesion , abc has a consistance of blood - filled spaces separated by connective tissue septa containing bone and osteoclastic giant cells . discussions about thoracic and sacral aneurysmal bone cysts are limited and only a few cases have been reported3,7 ) . papagelapoulos et al described 44 pelvis abcs and 12 of them were in the sacrum6 ) . it is well known that total extirpation of sabc is so difficult and surgical results in treatment of sabc are excellent . these clinical findings include leg weakness and numbness , bowl or bladder dysfunctions . in our cases up to 60 - 70% of abc cases that occured in spine present with neurological deficits . the clinical aspects are pain due to the destruction and neurological deficits due to the fractures and compression . in our case the diagnostic problems are because of the expeditious growth of abc and its expansive destruction of bone . the diagnosis becomes more complicated if there is an extra - osseous and soft - tissue tumour mass . it also helps to evaluate the fluidfluid level , which is characteristic for abc on mr images1 ) . our first case was evaluated as a pathological fracture , and the patient underwent urgent surgery because of the compression on spinal cannal . but the second case was so typical for abc on the radiological examinations as if in the literature . the primary option for treatment is surgery . enneking classified 3 surgical types : 1-intralesional ( curettage and bone grafting ) , 2-marginal ( en bloc ) resection , 3-wide resection ( segmental resection)3,4,5,6,7 ) . the most important factors in preoperative planning are the location and the growing pattern of the abc . if abc is growing superficially and besides if it is not involving the one - third of the bone , in this case it will be more advantageous . this is really a good intervention for abc and local recurrences occur less com- monly . besides this , if the abc is so large and located in pelvis or spine extraperitoneal excision and bone grafting could be more difficult and risky . in similar cases like this , curettage and bone grafting remain a choiceable surgical technique . low - dose radiotheraphy can be performed after curettage and bone grafting as an other option for treatment5 ) . age , location , size and number of mitotic figures have been suggested for recurrence treatment of abc is difficult and histological examination should be done to prevent overlooked of an underlying more aggressive neoplasm .

aneurysmal bone cysts are rare entities which causes expansile and destructive bone lesions characterized by reactive proliferation of connective tissue . they usually grow rapidly with hypervascularity . in clinical practice they can be easily misdiagnosed due to the rare occurance and having no such typical findings as radiologically . most cases have uncommon pain symptoms , but rarely , if fractures occur , neurological findings can be seen and the surgical treatment , if needed , could be difficult . we will discuss our evaluations to two cases that we experienced in our clinic in this report .

synchronous multiple primary lung cancers are reported rarely and account for about 8% ( 0.2% to 20% ) of all lung cancers [ 1 , 2 ] . patients with two or more lung tumours always require thorough diagnostics and an individually planned treatment procedure due to the nature of nodular lesions in the lung . two primary lung cancers are rarely reported , but triple primary lung cancers are almost unheard of , and there has been just a single report from england describing a patient with this condition ( 2011 ) . the authors successfully treated the patient surgically ( right upper lobectomy ) , and post - operative histopathological examination revealed squamous cell carcinoma , adenocarcinoma , and adenomatous hyperplasia . the 74-year - old patient with multiple primary lung cancers within the left lung was admitted to the thoracic , general and oncology surgical clinic in 2013 . on interview , the patient reported fatigue and about 8 kg loss of body weight within 6 months . the patient did not report any other ailments , but had been suffering from hypertension and copd , and reported smoking for 60 years , 20 cigarettes per day . the patient underwent bronchoscopy , but it did not confirm any pathology suggesting a neoplastic process in the bronchial tree . the first lesion , 41 21 mm in size , was located in segments 1 and 2 , and the second lesion , 31 19 mm in size , was found in segment 3 of the left lung ( figs . 1 a , b ) . tomographic imaging revealed lesions suspicious for cancer , group 5 lymph nodes up to 13 mm , and left hilum up to 12 mm . an image from the abdominal ultrasound scan revealed no significant abnormalities , apart from a left kidney cyst , 28 19 mm in size . the patient was referred for fdg - pet scanning ( positron emission tomography with f fluorodeoxyglucose ( fdg ) ) and for determination of the standard uptake value ( suv ) , providing indirect diagnosis of pulmonary and other neoplastic tumours . in the upper lobe of the left lung investigations revealed a metabolically active tumour between segments 1 and 2 , with a maximum diameter of 42 mm and suvmax fdg = 12.5 . the second metabolically active tumour was detected in segment 3 of the left lung and had a maximum diameter of 21 mm with suvmax fdg = 25.6 ( figs . similar to findings from computed tomography , metabolically active lymph nodes were detected with suvmax fdg = 6.6 . no abnormalities associated with elevated fdg metabolism were found in other scanned parts of the body . fine - needle aspiration ( fna ) biopsy indicated non - small cell lung cancer . the patient underwent left upper lobectomy plus lymphadenectomy under general anaesthesia and single - lung ventilation . intraoperative findings confirmed the presence of the previously described lesions within the upper lobe of the left lung , and small lymph node groups 5 , 6 , 10 and 11 l , which were resected . on the second and third day after surgery , drains were gradually removed from the pleural cavity and full lung expansion was achieved . on the fourth day after the surgery the patient was discharged and currently remains under the care of the thoracic surgery outpatient clinic affiliated with the hospital . histopathological findings confirmed a tumour 30 20 20 mm in size in segment 3 , with the histopathological structure of keratinizing squamous cell carcinoma g2 , and with immunohistochemical profiles of p63 ( + ) and ttf1 ( ) . the second nodular lesion , 45 35 20 mm in size , was described by histopathologists as two lung cancers . the first part of it ( 90% ) consisted of neuroendocrine carcinoma g2 cells , with the immunohistochemical profile cd56 ( + ) , ck7 ( ) , chromogranin ( ) , and synaptophysin ( ) . the second part of the described nodular lesion ( 10% ) consisted of acinar adenocarcinoma g2 cells , with the immunohistochemical profile p63 ( ) and ttf1 ( + ) . in total , 12 lymph nodes were resected ; of these , in 5 , 6 and 11 l groups no metastases were found , and one out of two resected lymph nodes from group 10 l was found to be metastatic . all the three identified lesions were synchronous primary lung cancers . computed tomography scan nodular lesions in the left lung positron emission tomography scan the synchronous occurrence of two primary lung cancers in the same lung was described for the first time by beyreuther in 1924 . in the literature there is a single case report , dated 2011 , describing the occurrence of triple synchronous primary lung cancers , i.e. squamous cell carcinoma , adenocarcinoma , and adenomatous hyperplasia . the researchers estimated that two primary lung cancers in the same lobe were diagnosed in 15% of all analysed patients ( n = 175 ) . the precise diagnosis of nodular lesions in lungs is crucial for the choice of further treatment method . procedures used in patients with multiple metastatic lung cancers differ from those used in patients with multiple primary lung cancers . positron emission tomography ( pet ) and computed tomography ( ct ) are very helpful diagnostic techniques for the preoperative assessment of patients . these techniques allow for the determination of the number , type and location of tumours within the body . multiple lung cancers are usually metastatic , but their status can be confirmed by pet / ct scans [ 6 , 7 ] . treatment options for patients with multiple and multifocal lung cancers remain controversial . in the described case examination results provided us with information on multiple metabolically active tumours within the same lung lobe . nevertheless , the final confirmation of the lesion type is still provided by histopathological examination . studies evaluating the survival of patients after procedural treatment for multiple lung cancers demonstrated much better outcomes in patients treated surgically for two synchronous primary lung cancers in the same lobe compared to patients with cancer at stage iiib or iv . the patient was classified as having stage t3n1m0 lung cancer ( stage iiia ) according to the latest , 7 edition of the tnm classification .

we present the case of a 74-year - old patient with three synchronous primary lung cancers within the same lung lobe . computed tomography and positron emission tomography investigations revealed two suspicious nodular lesions in the upper lobe of the left lung . fine - needle aspiration biopsy confirmed that one of the lesions was non - small cell lung cancer . the patient was qualified for surgical treatment , and left upper lobectomy plus lymphadenectomy was performed . histopathological examination confirmed the presence of three primary cancers in the left lung : keratinizing squamous cell carcinoma , neuroendocrine carcinoma , and acinar adenocarcinoma , localized within the same lung lobe . the patient was classified as having stage t3n1m0 lung cancer ( stage iiia ) according to the latest , 7th edition of the tnm classification .

we reviewed outbreak reports to identify outbreaks associated with an imported food from the inception of the surveillance system in 1973 through 2014 , the most recent year for which data were available . we obtained additional data for some outbreaks ( e.g. , country of origin ) from the us food and drug administration ( fda ) and the us department of agriculture food safety and inspection service . we categorized implicated foods by using the schema developed by the interagency food safety analytics collaboration ( 3 ) . we conducted a descriptive analysis of the number of outbreaks over time , by food category , and by region of origin . during 19962014 , a total of 195 outbreak investigations implicated an imported food , resulting in 10,685 illnesses , 1,017 hospitalizations , and 19 deaths . outbreaks associated with imported foods represented an increasing proportion of all foodborne disease outbreaks where a food was implicated and reported ( 1% during 19962000 vs. 5% during 20092014 ) . the number of outbreaks associated with an imported food increased from an average of 3 per year during 19962000 to an average of 18 per year during 20092014 ( figure ) . number of outbreaks caused by imported foods and total number of outbreaks with a food reported , united states , 19962014 . reporting practices changed over time ; 19731997 , imported foods anecdotally noted in report comments ; 19982008 , contaminated food imported into u.s . included as a location where food was prepared ; 20092014 , reporting jurisdictions could indicate whether each food is imported ( yes / no ) and the country of origin . the most common agents reported in outbreaks associated with imported foods were scombroid toxin and salmonella ; most illnesses were associated with salmonella and cyclospora ( table ) . aquatic animals were responsible for 55% of outbreaks and 11% of outbreak - associated illnesses . outbreaks attributed to produce had a median of 40 illnesses compared with a median of 3 in outbreaks attributed to aquatic animals . most of the salmonella outbreaks ( 77% ) were associated with produce , including fruits ( n = 14 ) , seeded vegetables ( n = 10 ) , sprouts ( n = 6 ) , nuts and seeds ( n = 5 ) , spices ( n = 4 ) , and herbs ( n = 1 ) . other agents implicated were tetrodotoxin ( 3 outbreaks ) and campylobacter , chaconine , paragonimus , other virus , sulfite , and trichinella ( 1 outbreak each ) . foods implicated were a chicken dish , crab cake , creampuff , beer , and a wheat snack ( 1 outbreak each ) . latin america and the caribbean was the most common region implicated , followed by asia ( technical appendix ) . thirty - one countries were implicated ; mexico was most frequently implicated ( 42 outbreaks ) . other countries associated with > 10 outbreaks were indonesia ( n = 17 ) and canada ( n = 11 ) . fish and shellfish originated from all regions except europe but were most commonly imported from asia ( 65% of outbreaks associated with fish or shellfish ) . produce originated from all regions but was most commonly imported from latin america and the caribbean ( 64% of outbreaks associated with produce ) . all but 1 outbreak associated with dairy products involved products imported from latin america and the caribbean . outbreaks in this analysis were reported from 31 states , most commonly california ( n = 30 ) , florida ( n = 25 ) , and new york ( n = 16 ) . the number of reported outbreaks associated with imported foods , although small , has increased as an absolute number and in proportion to the total number of outbreaks in which the implicated food was identified and reported . although many types of imported foods were associated with outbreaks , fish and produce were most common . many outbreaks , particularly outbreaks involving produce , were associated with foods imported from countries in latin america and the caribbean . because of their proximity , these countries are major sources of perishable items such as fresh fruits and vegetables ; mexico is the source of about one quarter of the total value of fruit and nut imports and 45%50% of vegetable imports , followed by chile and costa rica . similarly , our finding that many outbreaks were associated with fish from asia is consistent with data on the sources of fish imports ( 6 ) . one quarter of the outbreaks were multistate , reflecting the wide distribution of many imported foods . systems like pulsenet have helped to improve detection and investigation of multistate outbreaks , resulting in an increased number of multistate outbreaks ( 7,8 ) . the increasing number of outbreaks involving globally distributed foods underscores the need to strengthen regional and global networks for outbreak detection and information sharing . the importance of having standard protocols for molecular characterization of isolates and systems for rapid traceability of implicated foods to their source was illustrated during the investigation of a listeriosis outbreak linked to italian cheese imported into the united states in 2012 ( 9 ) . newer tools like whole genome sequencing can also help to generate hypothetical transmission networks and in some instances facilitate traceback of foods to their origin ( 10 ) . moreover , new tools that aid visualization of supplier networks facilitate the investigation of outbreaks involving the increasingly complex global economy ( 11 ) . only a small proportion of fda - regulated foods are inspected upon entry into the united states . new rules under the food safety modernization act of 2011 , including the preventive controls rule for human food , produce safety rule , foreign supplier verification program , and accreditation of third party auditors , will help to strengthen the safety of imported foods by granting fda enhanced authorities to require that imported foods meet the same safety standards as foods produced domestically ( 12 ) . although data collection has improved in recent years , these findings might underestimate the number of outbreaks associated with imported foods because the origin of only a small proportion of foods causing outbreaks is reported . similarly , because of how data are collected and reported , the relative safety of imported and domestically produced foods can not be compared . because of changes in surveillance and changing import patterns , changes over time should be interpreted cautiously . our findings reflect current patterns in food imports and provide information to help guide future outbreak investigations . prevention focused on the most common imported foods causing outbreaks , produce and seafood , could help prevent outbreaks . efforts to improve the safety of the food supply can include strengthening reporting by gathering better data on the origin of implicated food items , including whether imported and from what country . region and country of origin of imported foods implicated in outbreaks , by food category , united states , 19962014 .

the proportion of us food that is imported is increasing ; most seafood and half of fruits are imported . we identified a small but increasing number of foodborne disease outbreaks associated with imported foods , most commonly fish and produce . new outbreak investigation tools and federal regulatory authority are key to maintaining food safety .

this whole genome shotgun project has been deposited at ddbj / embl / genbank under the accession jpul02000000 ( http://www.ncbi.nlm.nih.gov/nuccore/jpul00000000.2 ) . the stramenopile blastocystis is a common anaerobic protist living in the digestive tract of several animal groups . its prevalence in human often exceeds 5% in industrialized countries and can reach 100% in developing countries . although the role of blastocystis as a human pathogen remains unclear , it has been associated with acute or chronic digestive disorders and some epidemiological surveys have suggested an association with irritable bowel syndrome ( ibs ) , . in patients with ibs , blastocystis seems to be associated with a decrease of the fecal microbiota protective bacteria , bifidobacterium sp . and faecalibacterium prausnitzii . the life cycle of the parasite is poorly documented . among the parasitic forms described in the literature , the vacuolar stage which is maintained in vitro in axenic culture , blastocystis exhibits an extensive genetic diversity and seventeen subtypes ( st1st17 ) have been identified based on the gene coding for the small - subunit ribosomal rna among which the first nine are found in humans . briefly , it consists of an 18.8 mbp nuclear genome with 6020 predicted genes and a circular genome of 29 kbp located within mitochondria - like organelles ( mlo ) . other mlo genomes with conserved gene synteny we report the sequencing of the blastocystis st4-wr1 genome from an isolate of a laboratory rodent and cultured axenically . genomic dna was isolated using a qiagen dneasy blood and tissue kit and sequencing was performed with the illumina hiseq 2000 system ( genoscreen , lille , france ) . a total of 43.855.085 of 100-bp high quality paired - end reads were generated and were de novo assembled using the idba - ud algorithm . the output was then scaffolded using sspace and gaps were filled by gapfiller software . in total , 1301 scaffolds from 494 bp to 133,271 bp were obtained , with a scaffold n50 of 29,931 bp . the draft genome sequence of blastocystis st4 has a deduced total length of 12.91 mbp and a g + c content of 39.7% . assembly also provided a circular dna molecule of 27,717 bp in size with a g + c content of 21.9% corresponding to the whole mlo genome sequence . the maker pipeline was set with the results of ab initio gene prediction algorithms augustus and snap , the 6020 protein - coding genes of blastocystis st7 , ests of both blastocystis st7 and st1 and 414 manually - designed genes of the st4-wr1 isolate . basic information about the assembled genome and predicted genes are shown in table 1 . gene functions were annotated by blast2go and blast analyses with ncbi ( http://www.ncbi.nlm.nih.gov/ ) . the preliminary annotation data revealed that blastocystis st4-wr1 nuclear genome harbors 5713 protein - coding genes . the presence of proteases was determined using blast against merops database , and secreted proteases were identified using signalp 3.0 and wolf psort . this comparative analysis revealed that the st4 genome contains less duplicated genes than st7 and that more than 30% of st4 genes have no ortholog in the st7 genome at an e value cutoff of 10 . this also led to the identification of new candidate genes , in particular some potential virulence factors , including 20 secreted proteases that may be involved in the physiopathology of this parasite . among these proteases , 7 seem to be specific to st4 as no ortholog has been found in the st7 genome . sequencing and annotation of additional st ( st1 , st2 , st3 and st8 ) genomes are under progress and should be helpful for a better understanding of the genetic diversity , pathogenesis , metabolic potential and genome evolution of this highly prevalent human parasite .

the intestinal protistan parasite blastocystis is characterized by an extensive genetic variability with 17 subtypes ( st1st17 ) described to date . only the whole genome of a human st7 isolate was previously sequenced . here we report the draft genome sequence of blastocystis st4-wr1 isolated from a laboratory rodent at singapore .

a 64-year - old man visited our hospital with the chief complaint of microscopic hematuria for the past 2 months . abdominal computed tomography ( ct ) revealed a homogeneous solid tumor with slightly low attenuation in the left renal sinus ( fig . altogether , these abdominal ct findings were suggestive of invasive urothelial carcinoma arising in the left pelvis with involvement of renal sinus soft tissue . nephroureterectomy was performed , and the cut surface of the renal sinus revealed a grayish - white solid tumor of rubbery consistency . the tumor was primarily located in the renal pelvis , but extensions to the renal calyces and proximal ureter were suspicious . the pelvic tumor mass measured 42.5 cm and exhibited peripelvic infiltrative features ( fig . 2 ) . however , the mucosal surfaces of the renal pelvis , ureter and calyces were smooth , and there was no evidence of urothelial malignancy . additionally , the mucosal layers of the renal pelvis , calyces and ureter were intact without abnormal urothelial changes . microscopically , the tumor occupying the renal sinus contained widely scattered , hyperplastic lymphoid follicles , characteristic of a lymphoproliferative lesion . the follicles varied in size and showed polarized germinal centers ; moreover , interfollicular spaces were markedly infiltrated by mature plasma cells , highlighted by cd138 immunostaining ( fig . 3 ) . immunostaining for and immunoglobulin light chains indicated that the plasma cells were polyclonal in origin . immunostaining for human herpes virus 8 ( hhv-8 ) was performed , the results of which were negative . these histological and immunohistochemical findings were compatible with a diagnosis of castleman 's disease of the plasma cell type . three months after the operation , there was no evidence of disease recurrence on follow - up abdominal ct . the occurrence of castleman 's disease in the renal sinus is exceedingly rare.1 to the best of our knowledge , only a few cases have been reported.1,3 - 6 the clinicopathological characteristics of the reported cases , involving six males and one female , are summarized in table 1 . although castleman 's disease generally shows no gender preference,2 cases involving the renal sinus appeared to show a male predominance . the median age for castleman 's disease is in the fourth decade.2 however , the mean age of the cases involving the renal sinus was 65 years . the main presenting symptoms included abdominal pain , weight loss , anorexia , and microscopic hematuria . five cases were of the plasma cell type , one of the hyaline vascular type and one of mixed type . the patient in the present case , a 64-year - old male , presented with symptoms of microscopic hematuria , and his disease was histologically classified as plasma cell type and clinically as unicentric type . although the exact pathophysiology of castleman 's disease is unknown , recent reports suggest that hhv-8 infection may stimulate b lymphocytes to induce interleukin 6 ( il-6 ) production in the mantle zone ; il-6 overproduction has been shown to be associated with the systemic manifestations of castleman 's disease , especially in cases of multicentric disease.7 in our case , immunostaining for hhv-8 was performed , but the result was negative . preoperative diagnostic imaging methods are not useful in differentiating castleman 's disease arising in the renal sinus from other diseases because of a lack of tumor - specific imaging features . previously , nishie et al.1 described the ct and magnetic resonance imaging features of three cases of castleman 's disease involving the renal sinus . they reported that the renal sinus lesions formed in castleman 's disease can appear as homogeneous masses with mild enhancement , such as is often observed in malignant lymphomas . thus , it is difficult , on the basis of radiological findings , to differentiate castleman 's disease from malignant lymphomas infiltrating the renal sinus . other differential diagnoses may include invasive urothelial carcinoma , granulomatous diseases , sarcomas , and metastases . consequently , pathological evaluation is currently the only method of identifying castleman 's disease of the renal sinus . here we have described an extremely rare case of unicentric castleman 's disease of the plasma cell type arising in the renal sinus . although preoperative diagnosis of castleman 's disease is difficult and accurate diagnosis is only possible by histopathologic evaluation , it should be considered in the differential diagnosis of renal sinus tumors .

castleman 's disease is a rare benign lymphoproliferative disorder that frequently affects lymph nodes of the mediastinal thorax and the neck . it very rarely affects the renal sinus . we report a case of castleman 's disease arising in the renal sinus in a 64-year - old man . the patient visited the hospital with the chief complaint of hematuria . abdominal computed tomography revealed a homogeneous mass in the sinus of the left kidney , radiologically interpreted as a malignant urothelial tumor . subsequently , nephroureterectomy was performed , after which microscopic examination of the specimen revealed a diffuse lymphoproliferative lesion with reactive lymphoid follicles of various sizes and prominent plasma cell infiltration of interfollicular spaces , highlighted by immunohistochemical staining for cd138 . the lesion was diagnosed as castleman 's disease of the plasma cell type . although preoperative diagnosis of castleman 's disease is difficult and the incidence is exceedingly rare , it should be considered in the differential diagnosis of renal sinus tumors .

leri - weill 's dyschondrosteosis ( lwd ) is anautosomal dominant condition with variable penetrance characterized by mesomelic short stature and with madelung deformity . it is due to deletion or mutation in short stature homeobox ( shox ) gene . here , there is deficient growth of a volar and ulnar aspect of distal radialphysis , triangulation of corpus with proximal and volar shift of the lunate . a 13-year - old girl referred to us with a history of deformity of bilateral forearm from the age of 5 to 6 years . there was no history of pain in the joint of hands , trauma to the wrist and no history of similar illness in the family . patient was moderately built and significantly short for her age [ figure 1 ] . with height of 130 cm ( less than 3 percentile ) . upper segment was 69 cm , lower segment was 61 cm , weight of 35 kg and body mass index of 20.71 kg / m . secondary sexual characteristic tanner - whitehouse staging - b4 ( breast ) , p4 ( pubic hair ) , a3 ( axillary hair ) her elder brother had normal height for age and no any hand deformities and so were her parents . investigation showed hemoglobin of 12.5 g / dl , liver function test and renal function test were normal ca= 9.0 mg / dl alkaline phosphates 260 iu and phosphorus = 3.5 mg / dl . x - ray bilateral wrist showed malformed medial radial epiphysis with dorsal and ulnar shift and with increased length of phalanges suggestive of madelung deformity [ figure 2 ] . her ultrasound abdomen showed no hepatomegaly or splenomegaly and had normal sized uterus and ovary x - ray spine and x - ray elbow were normal [ figure 3 ] . her luteinizing hormone = 1.1 miu / ml follicle stimulating hormone = 2.4 miu / ml and karyotype was normal as was urine mucopolysaccharide screening . x - ray bilateral wrist based on the history , clinical examination , and investigations findings , the final diagnosis of lwd as a cause of madelung deformity was made . it is the most common cause of mesomelic deformity and is inherited as an autosomal dominant trait with variable penetrance . it is often seen in girls and becomes apparent in late childhood or early adolescence . madelung deformity , beside is also found in turner 's syndrome , pseudohypoparathyroidism , mucopolysaccharidosis and achondroplasia of distal radial epiphysis . madelung deformity was first described by malgaigne in 1885 and later by madelung in 1878 as a spontaneous forward subluxation of the hand . madelung deformity is an ulnar and dorsal curvature of the distal radius due to deficient growth of the volar and ulnar aspect of distal radialphysis , increased inclination of the distal radial joint surface , triangulation of the corpus with proximal and volar migration of and a prominent dorsal subluxation of ulnar head . recently , two subtypes of madelung deformity have been described , one with short stature and mesomelia consistent with lwd and the other with severe involvement of the entire radius with limited range of motion of extremity , markedly bowed appearance of the forearm and conspicuous radiographic deformity of the forearm and distal radius . the pathogenesis of lwd is linked to deletion or mutation in shox gene , present in the pseudoautosomal region of the sex chromosomes - xp23 and yp11 . in early human embryos it is expressed in the developing limbs ( particular elbow , knee , distal radius / ulnar and wrist ) as well as first and second pharyngeal arches and plays an important role in bone growth as well as development . shox gene is also associated with short stature in turner 's syndrome and also some causes of growth retardation like lws and langers mesomelic dysplasia . management is usually conservative . persistent pain and or severe deformity call for orthopedic surgery involving radial osteotomy . in addition , ulnar shortening in skeletally immature patient or excision of distal ulnar head in skeletally mature patients are done . surgical prophylaxis by distal resection of the abnormal part of distal radial epiphysis and its replacement by autologous fat ( also known as physeolysis ) has recently been shown to restore growth and minimized deformity . growth hormone supplementation is found to increase final height in turner 's syndrome , lws but not in langers mesomelic dysplasia . hand deformity is corrected by orthopedic surgery and for shox gene related short stature food and drug administration has recently approved growth hormone therapy .

madelung deformity is a rare inherited disorder associated with endocrine disorders like turner 's syndrome , pseudohypoparathyroidism , but can be seen with short stature homeobox deficiency conditions such as leri - weill dyschondrosteosis ( lwd ) and langers mesomelic dysplasia . it has also been reported following trauma to the distal radius epiphysis neoplasia mucopolysaccharidosis ( mps ) and achondroplasia . madelung deformity is an abnormality of distal radial epiphysis where in progressive ulnar and volar tilt of the articular surface occurring in association with distal subluxation of ulna . a 13-year - old girl was referred to us for evaluation of bilateral deformity of wrist and short stature . there was ulnar deviation and dorsal tilt of bilateral hands without history of pain to the joint trauma and family history of similar illness . on x - ray , wrist showed malformed distal radial epiphysis with dorsal and ulnar shift and with increased length of phalanges suggestive of madelung deformity . x - ray spine was normal . ultrasound abdomen showed normal uterus and ovary and her follicle stimulating hormone . luteinizing hormone was normal and so was urine mps screening . based on the above points the diagnosis of lwd was made .

pityriasis rubra pilaris ( prp ) includes a spectrum of rare chronic , idiopathic inflammatory disorders with papulosquamous eruptions of unknown cause . prp shows consistent clinical heterogeneity ; consequently , it is hard to predict the outcome of treatment . a 29-year - old female presented for evaluation of a skin condition previously diagnosed as psoriasis vulgaris . the lesions first appeared at age six years with no preceding trauma or infection and were characterized by palmoplantar keratoderma and demarcated , hyperkeratotic plaques on the elbows and knees . the patient had been on many systemic treatments over the last two years without response , including cyclosporine ( 3 mg / kg / day for 3 months ) , methotrexate ( 15 mg / weekly for 4 months ) , adalimumab ( two subcutaneous injection of 40 mg at day 0 , a subcutaneous injection of 40 mg at day 7 and every 14 days thereafter for 16 weeks ) , etanercept ( 50 mg weekly subcutaneous injections for 12 weeks ) , infliximab ( 5 mg / kg given as an intravenous infusion at weeks 0 , 2 , 8) . physical examination showed a diffuse , orange - pink palmoplantar keratoderma [ figure 1 ] . well - defined keratotic follicular papules also involved the dorsal aspects of the hands and feet , elbows and knees [ figures 2 and 3 ] . a skin biopsy revealed alternating parakeratosis and orthokeratosis , pronounced irregular acanthosis , focal hypergranulosis and mild focal spongiosis . based on clinical and histologic findings , the patient was diagnosed having prp , type iv ( circumscribed juvenile ) . after voluntary , informed consent , ustekinumab 45 mg subcutaneously at weeks 0 and 4 , and quarterly thereafter ( patient 's weight = 55 kg ) was then started , the same posology as in psoriasis . keratoderma of the hands with a sharp demarcation of the borders diffuse transgrediens palmoplantar keratoderma on the dorsum of hands follicular hyperkeratosis and erythema on the elbows alternating parakeratosis and orthokeratosis , irregular acanthosis , focal hypergranulosis and a perivascular lymphocytic infiltration in the papillary dermis ( h and e 10 ) pityriasis rubra pilaris occurs equally in male and female patients , with a bimodal age distribution , peaking during the first and then the sixth decade . griffiths proposed a classification for prp in five subtypes , based upon age , duration , and type of cutaneous involvement . type i , or classic prp , is the commonest type ( 50% of cases ) and occurs in adults . the patient is usually erythrodermic with diffuse thickening of the palms and soles and possibly ectropion . 80% of patients experience clinical resolution within 3 years . on the basis of griffith 's classification clinical manifestations occurred in her prepubertal age and relapsed at age 27 , after a long - lasting remission . type iv prp develops in prepubertal children presenting with sharply - demarcated areas of follicular hyperkeratosis and erythema on the elbows and knees . a waxy , orange - red , diffuse , palmoplantar keratoderma is also commonly observed . a standard therapeutic approach does not exist as cases are few and treatment is protracted . an increasing number of reports document the effectiveness of tumor necrosis factor- ( tnf- ) blockers in recalcitrant prp . further , some case reports have documented favorable response of prp to ustekinumab , a fully human monoclonal antibody which binds to interleukin-12 ( il-12 ) and il-23 with high specificity and affinity . an upregulation of tnf mrna in lesional compared with nonlesional skin in two patients with type i prp has been demonstrated . a recent retrospective revision investigating treatment options showed a marked clinical in more than 50% of patients with type , a systematic review of reports of prp responding positively to tnf- blockers does not recommend them due to possible reporting bias and spontaneous remissions . in the literature , the patients who achieved remission with tnf- blockers or ustekinumab were all consistent with classical type 1 prp . our patient who presented a type iv prp was unresponsive to the tnf - blockers adalimumab , etanercept and infliximab . our clinical experience suggests that in patients with type iv prp , tnf and il-12/il-23 blockade may not be useful targets . since we observed a primary lack of response to all available tnf - blockers , we suggest that yet unmapped signaling pathways may be involved in type iv prp . there seems to be inadequate response of circumscribed variants of prp to standard therapies that are effective in classical prp .

treatment of pityriasis rubra pilaris ( prp ) may be difficult since no standardized therapeutic approach has been established . recently , tumor necrosis factor- ( tnf- ) blockers have been demonstrated to be favorable in the management of recalcitrant prp . the authors report a case of a patient who presented a type iv prp or circumscribed , juvenile type . such a condition follows an unpredictable course , presenting with diffuse , palmoplantar keratoderma and sharply - demarcated areas of follicular hyperkeratosis on the elbows and knees . treatment with all available tnf- inhibitors and ustekinumab did not prove to be helpful . the authors suggest that circumscribed variants of prp could respond to therapy in ways different from classical prp .

teratoma is a special type of mixed tumor that contains recognizable mature or immature cells or tissues representative of more than one germ cell layer and sometimes all three . teratomas can develop in almost any area of the body , but usually occur in median sites . the most common sites are the sacrococcyx , anterior mediastinum , testicle , ovary , or retroperitoneum . 1 day old male baby was referred for respiratory distress secondary to the presence of an oropharyngeal mass . there no other abnormalities found in the head and neck region . under general anesthesia the lesion was excised . on the gross pathological examination , the excisional material was a polypoid lesion of 3 2 1.5 cm in size whose surface was covered with skin ( fig . histologically , the mass consisted of a core of lobules of mature adipose tissue admixed with fascicles of striated skeletal muscle and covered by keratinized squamous epithelium containing many hair follicles associated with sebaceous and eccrine glands ( fig . these findings confirmed the diagnosis of nasopharyngeal mature teratoma also known as congenital hairy polyp . . teratomas generally occur in 1 of 4000 births , but only 10% are found in the head and neck . they most commonly arise from the midline or lateral nasopharyngeal wall and some reports have described a slight female predilection , but jiang et al . found malignant teratoma to be more common in men . the majority of neonates with nasopharyngeal teratomas can be successfully intubated either via the oral or nasal routes , whereas the remainder usually require tracheostomy . in 6% of all cases , teratomas are associated with malformations such as cleft palate , bifid tongue , and bifid uvula . most of the congenital nasopharyngeal lesions present as an emergency respiratory problem and require immediate surgery . benign ones consist of mature tissue components , while those with malignant potential contain immature tissues ; benign teratomas may undergo malignant change with age . histopathological examination showed the mass in our case to be composed of multiple mature tissues with no juvenile cells , which confirmed a benign tumor . the main therapy of teratoma is complete surgical excision , which depends on the site of the tumor . patients with teratomas are more likely to require intensive airway management prior to surgical excision of the lesion . written informed consent was obtained from the patient 's parent for publication of this case report and accompanying images . a copy of the written consent is available for the review of the editor - of - chief of this journal on request .

highlightswe report a newborn with soft palatal mass.we excised the mass.on the gross pathological examination , it was polypoid lesion covered with skin . histologically , it consisted of a core of lobules of mature adipose tissue admixed with fascicles of striated skeletal muscle and covered by keratinized squamous epithelium containing many hair follicles associated with sebaceous and eccrine glands.these findings confirmed mature teratoma .

cavernous sinus thrombosis ( cst ) , which was first described by dease in 1778 , is a rare disease associated with high mortality and morbidity rates if not treated immediately1 . cst can result from infection of the paranasal sinus or any of the anatomic structures drained by the cavernous sinus , including the mid - face , orbit , and oral cavity2 . deep neck infection containing lateral pharyngeal , pterygomandibular and infratemporal space that patient are presenting symptom of the limited mouth opening can cause infection of the cavernous sinus from the pterygoid plexus3,4 . in this study , we report a patient with cst who initially presented with limited mouth opening . a 55-year - old male visited our department with a chief complaint of limited mouth opening , which began suddenly on august 13 , 2013 . on physical examination , mouth opening was limited to approximately 10 mm , with mild pain on opening . however , body temperature was normal and signs of infection including odynophagia , dyspnea , swelling and tenderness were absent . in the transcranial view of the temporomandibular joint , the translation of both temporomandibular joints was limited.(fig . 2 ) the provisional diagnosis was myospasm of the masticatory muscles ; we prescribed a muscle relaxant and analgesic with physiotherapy . three days later , on august 16 , 2013 , the patient was admitted to the department of ophthalmology complaining of orbital swelling and pain . clinical examination revealed ptosis , proptosis , moderate chemosis , diplopia , and complete ophthalmoplegia of right side.(fig . 3 ) peripheral sensation around the right orbit was good and cranial nerves vii through xii were intact . 4 ) computed tomography ( ct ) and magnetic resonance imaging ( mri ) revealed proptosis and engorgement of the right superior ophthalmic vein that was accompanied by enhancement of retrobulbar tissues and swelling of the lateral pterygoid muscle with pus formation . also , contrast enhancement and dilation indicative of inflammation of the cavernous sinus was noted.(fig . 5 ) the paranasal sinuses , including the ethmoid and maxillary sinus of the ipsilateral side , exhibited mucosal thickening and retention of purulent material.(fig . 6 ) initially , the patient was empirically treated with intravenous cephalosporin . on hospital day 3 , he was prepared for incision and drainage under general anesthesia . a large amount of pus was drained from the lateral pharyngeal , pterygomandibular and infratemporal spaces . the wound was irrigated with copious saline through silastic drains placed in the abscess cavity . repeated ct 10 days after surgery demonstrated improvement in the engorgement of the superior ophthalmic vein and dilation of the cavernous sinus.(fig . 7 ) over the following 11 months , his orbital symptoms resolved completely and his range of mouth opening returned to normal.(fig . the cavernous sinus , which is located to the rear of the optic canal and the superior orbital fissure , is an important structure containing the internal carotid artery and cranial nerve iii , iv , v , and vi . thus , if infection or thrombosis occurs in the cavernous sinus , various symptoms referred to as ' cavernous sinus syndrome ' can appear , including ophthalmoplegia , diplopia and ptosis due to impairment of cranial nerve iii , iv , and vi . involvement of the ophthalmic nerve and maxillary nerve can cause paresthesia around the orbital cavity5 . thus , proptosis and chemosis can cause drain blockages of ophthalmic veins to the cavernous sinus6 . there are also connections between the contralateral cavernous sinus , the intercavernous sinus , facial veins that have not valve , and the pterygoid plexus , which is located very close to the cavernous sinus . given the complex anastomosis of veins with the cavernous sinus , sometimes orbital symptoms appear on both sides , or on the opposite side7,8 . childs and courville11 reported that an odontogenic source was responsible in up to 10% of cases . pavlovich et al.2 presented two cases with different mechanisms : sinusitis and dental infection . in our case , chronic periodontitis of the maxillary posterior teeth and mucosal thickening of the right ethmoidal sinus were observed . because the initial clinical symptom was the limitation of mouth opening , however , the infection was assumed to be from an odontogenic source . mazzeo4 reported that mandibular infections may spread directly to the dural sinuses through the pterygoid plexus . in our case , the patient exhibited symptoms of the proptosis , ptosis , and ophthalmoplesia , accompanied by swelling of the face and neck due to what appeared to be a deep neck infection . the infection was thought to originate around the pterygoid muscle , since trismus preceded orbital symptoms . the route of infection to the cavernous sinus was deemed to be via the pterygoid plexus . numerous causes of superior orbital fissure syndrome including trauma , infection of the cavernous sinus , neoplasm , aneurysm of the internal carotid artery , carotid cavernous fistula or idiopathic etiology have been reported in the literature12,13 . imaging studies such as ct , mri , and magnetic resonance angiography should be employed in patients with findings consistent with a cst . if a vascular lesion of the cavernous sinus is suspected , cerebral angiography may be necessary5 . typical radiographic signs of cst include expansion of the cavernous sinus , convexity of the normally concave lateral wall , irregular filling defects , and asymmetry ; indirect signs include venous obstruction , dilation of the superior ophthalmic vein , proptosis , and thrombus in the veins14,15 . our patients underwent angiography with simultaneous ct and mri to distinguish the clinical signs from the vascular lesion . on angiography , abnormal blood vessels were not observed , but direct and indirect radiographic signs of cst were demonstrated on ct and mri . the treatment of cst includes the use of antibiotics directed at the causative organism and surgery to remove the primary source of infection . the role of steroid and anticoagulant therapy for the treatment of cst remains controversial14 . in summary , cst is a rare disease that can cause life threatening complications . infections of the pterygoid plexus from an odontogenic source can present as trismus , and clinicians should consider the potential for progression to cst . prompt diagnosis and appropriate treatment are essential for preventing serious complications .

in the department of oral and maxillofacial surgery , patients with trismus can be easily identified . if the cause of trismus is infection of the masticatory space near the pterygoid plexus , the possibility of cavernous sinus thrombosis should be considered . we report the case of a patient who presented with limited mouth opening and progressed to cavernous sinus thrombosis , along with a review of the relevant literature .

metastatic disease is rare and usually occurs within the first two years after initial diagnosis , and is extremely rare after that time period . however , metastases to forearm bones are very rare with only very few reports published till date . here we present a case of bilateral retinoblastoma with metastases to right forearm bones four years after the initial treatment . an 8-year - old boy treated four years back for bilateral retinoblastoma with enucleation of left eye along with adjuvant chemotherapy and radiotherapy presented with a right forearm swelling . an x - ray revealed a mass lesion in right forearm with destruction of lower one third of right ulna . bone scan was performed three hours after intravenous injection of 5 mci of 99tc mdp . whole body images showed increased tracer uptake in right forearm bones [ figure 1 ] . static images revealed increased tracer uptake in middle and lower one - third of right ulna consistent with x - ray findings . in addition , bone scan showed involvement of lower one - third of left radius . however , special stains excluded ewing 's sarcoma and other primitive neuroectodermal tumors ( pnets ) . involvement of radius picked up by bone scan had important implication in deciding the nature of disease as ewings tumor with multi - focal disease as second primary is very unlikely . tc99m - mdp bone scan ; ( a ) whole body anterior view ; ( b ) whole body posterior view showing increased tracer uptake in right forearm bones . static images of bilateral forearms ; ( c ) anterior ; ( d ) posterior revealing involvement of right ulna and radius osseous metastases from retinoblastoma are reported most commonly in the skull and long bones . the prognosis for most children diagnosed with retinoblastoma is good , with a 5-year survival of rate of more than 90% . complications include local recurrence , metastases , and the development of a second primary tumor . focal and diffuse involvement of the bones and bone marrow is known to occur , but metastases are usually seen within two years of initial diagnosis and later metastases are said to be extremely rare . these patients are also at risk of increased second primaries like osteosarcoma , leiomyosarcoma , spindle cell sarcoma , malignant fibrous histiocytoma , rhabdomyosarcoma , angiosarcoma , ewing sarcoma , and pnet . these typically develop after a latent period of 815 years.[68 ] involvement of multiple bones would point the diagnosis towards metastases rather than multifocal second primary . imaging modalities available for skeletal metastatic work up include x - ray , ct , mri and functional imaging modalities like bone scan . x - rays are in general less sensitive to pick up the metastases , whereas whole body mri is not widely available , so conventional wb bone scan is essential in the metastatic work up of these patients . in our case , x - ray picked up ulnar involvement but radius involvement was missed . however , bone scan revealed radius involvement suggesting a metastatic disease rather than second primary . later , histopathology revealed round blue cell tumour involving both radius and ulna and ihc was negative for cd99 excluding diagnosis of ewings sarcoma . skeletal scintigraphy remains an essential diagnostic procedure in evaluation of these patients and can have important implications in management .

osseous metastases from retinoblastoma , the most common ocular malignant neoplasm of childhood , are reported most commonly in the skull and long bones . however , metastases to forearm bones are very rare . here we present a case of bilateral retinoblastoma with metastases to right forearm bones four years after the initial treatment .

the occurrence of foreign bodies such as metal screws , staple pins , darning needles , pencil leads , beads and tooth picks lodged in the exposed pulp chambers of carious or traumatically injured deciduous and permanent teeth has been reported . most often , these cases are diagnosed accidentally on radiographic examination of the tooth which may be associated with infection , pain , swelling and recurrent abscesses as a sequelae to the pulpal exposure and lodgement of the foreign body . clinical and radiographic examinations are necessary to confirm the presence , size , location and the type of the foreign object . two cases of foreign objects found within the pulp chambers of the deciduous teeth with their management are presented here . a 10-year - old girl reported with a chief complaint of pain and pus discharge from the upper front tooth on the right side . patient gave a history of pain since 10 days and pus discharge from the tooth since last 2 days . on clinical examination , there was discoloration and open pulp chamber in relation to right maxillary deciduous canine ( 53 ) [ figure 1 ] . radiographic examination of the tooth revealed a radio - opaque object resembling twisted pieces of metal wire lodged within the pulp chamber and root canal of 53 [ figure 2 ] . the tooth was extracted under local anesthesia [ figure 3 ] . on removal of the foreign objects in relation to the tooth , it was found that there were two staple pins and a small piece of aluminium foil [ figure 4 ] . the child , who had previously denied placing inserting within the tooth , later confessed that she had placed the objects to remove food particles and to get relief from pain . photograph of discolored deciduous maxillary canine on the right side radiograph showing the foreign object in the tooth photograph showing the metallic object in the extracted tooth two staple pins and pieces of aluminum foil recovered from the tooth a 5-year - old male child was brought to the department by their parents with a complaint of pain in relation to decay in the upper front teeth . clinical examination revealed that the deciduous right maxillary central incisor ( 51 ) was grossly decayed with open pulp chamber [ figure 5 ] . intraoral periapical radiographs of the maxillary anterior region examination revealed the presence of a radio - opaque foreign object within the pulp chamber and root canal of 51 [ figure 6 ] . the tooth was extracted under local anesthesia and the object was retrieved from the canal , which was found to be the broken head of a sewing needle [ figure 7 ] . photograph showing decayed maxillary incisors radiograph showing the foreign object in the tooth resembling the head of sewing needle head of sewing needle recovered from the tooth a 10-year - old girl reported with a chief complaint of pain and pus discharge from the upper front tooth on the right side . patient gave a history of pain since 10 days and pus discharge from the tooth since last 2 days . on clinical examination , there was discoloration and open pulp chamber in relation to right maxillary deciduous canine ( 53 ) [ figure 1 ] . radiographic examination of the tooth revealed a radio - opaque object resembling twisted pieces of metal wire lodged within the pulp chamber and root canal of 53 [ figure 2 ] . the tooth was extracted under local anesthesia [ figure 3 ] . on removal of the foreign objects in relation to the tooth , it was found that there were two staple pins and a small piece of aluminium foil [ figure 4 ] . the child , who had previously denied placing inserting within the tooth , later confessed that she had placed the objects to remove food particles and to get relief from pain . photograph of discolored deciduous maxillary canine on the right side radiograph showing the foreign object in the tooth photograph showing the metallic object in the extracted tooth two staple pins and pieces of aluminum foil recovered from the tooth a 5-year - old male child was brought to the department by their parents with a complaint of pain in relation to decay in the upper front teeth . clinical examination revealed that the deciduous right maxillary central incisor ( 51 ) was grossly decayed with open pulp chamber [ figure 5 ] . intraoral periapical radiographs of the maxillary anterior region examination revealed the presence of a radio - opaque foreign object within the pulp chamber and root canal of 51 [ figure 6 ] . the tooth was extracted under local anesthesia and the object was retrieved from the canal , which was found to be the broken head of a sewing needle [ figure 7 ] . photograph showing decayed maxillary incisors radiograph showing the foreign object in the tooth resembling the head of sewing needle head of sewing needle recovered from the tooth as children often tend to have the habit of inserting foreign objects in the oral cavity , there are more chances of finding foreign objects in their teeth . sometimes , children do not reveal to their parents due to fear . grossman , gelfman , and harris reported retrieval of indelible ink pencil tips , brads , a tooth pick , adsorbent points , tomato seed , pins , wooden toothpick , a pencil tip , plastic objects , toothbrush bristles and crayons from the root canals of anterior teeth left open for drainage . mcauliffe has suggested various radiographic methods to be followed to localize a radio - opaque foreign object , such as parallax views , vertex occlusal views , triangulation techniques , stereo radiography and tomography . the steglitz forceps have also been described for use of removal of silver points from the root canal . there is a description of an assembly of a disposable injection needle and thin steel wire loop , formed by passing the wire through the needle being used . this assembly was used along with a mosquito hemostat to tighten the loop around the object . in the cases in the first case , the patient first denied of inserting anything in the tooth but on further questioning she admitted of inserting staple pins , foils and papers in the tooth . as the prognosis was poor , the tooth was extracted . on removal of the foreign objects from the extracted tooth , we found two staple pins and a piece of aluminum foil . in the second case , the child came with a complaint of pain in the primary central incisor . on routine radiographic examination , broken head of sewing needle was found . again here , the parents were not aware of the fact that child had inserted sewing needle in the tooth . but the parents were not willing to come again and were insisting for the removal of the tooth . though the presence of foreign objects retrieved from the root canals and pulp chambers of the permanent teeth have been reported , the presence of foreign objects found in the deciduous teeth is an uncommon situation . timely diagnosis and management of foreign object embedded in the tooth should be done to avoid further complication .

accidental foreign body ingestion or aspiration is a common problem in children . children often have a habit of inserting objects into their mouth . some of these objects can be accidentally ingested or even aspirated which can be frightening and a stressful experience . but the presence of foreign objects in the teeth are rare . the foreign objects in the teeth may act as a potential source of infection and pain . in most of the cases , children avoid informing their parents due to fear of punishment . this paper presents two cases of foreign objects embedded in the deciduous teeth . in both the cases , parents were not aware of foreign body ingestion by their children .

toxoplasmosis occurs in advanced stages of human immunodeficiency virus ( hiv ) infection and is the most common opportunistic infection of the central nervous system in patients with the acquired immunodeficiency syndrome ( aids ) , especially in those patients with a cd4 + count < 200 cells / mm . the most common clinical presentation of cerebral toxoplasmosis in patients with hiv infection is headache , focal neurological deficits and seizures . we present two cases with stroke - like presentation of cerebral toxoplasmosis from our centre . a 30-year - old right - handed nigerian woman was admitted to the university of calabar teaching hospital , nigeria , with a 1-week history of inability to move the left upper and lower limbs of acute onset . there was a history of headache , but no seizures , vomiting or loss of consciousness . she was not known to be hypertensive or diabetic , and she does not drink alcohol nor does she smoke . the patient was diagnosed to be hiv positive about 2 years prior to this admission and commenced on antiretroviral drugs ( arv ) but defaulted on therapy for about 1 year prior to the current presentation . her latest cd4 + count was not known . on neurologic examination , she showed left upper motor neuron facial nerve palsy and increased tone and brisk reflexes in both left upper and lower limbs . power was grade 0/5 and grade 1/5 in the left upper and lower limbs , respectively , on the medical research council scale , with a positive babinski sign on the left . a brain ct scan revealed a rounded ring - enhancing hypodense lesion in the parafalcine region of the right parietal cortex ( fig . a complete blood count revealed low haemoglobin ( 8 g / dl ) , esr 80 mm in the first hour , and cd4 + count 18 cells / mm . iu / l and toxoplasma igg was 202.0 iu / l , both markedly raised . a diagnosis of stroke - like syndrome due to cerebral toxoplasmosis in the background of hiv infection was made and the patient was commenced on pyrimethamine ( 200 mg at start , then 50 mg daily ) , clindamycin ( 600 mg every 6 h ) , folic acid ( 10 mg daily ) , septrin ( 960 mg b.i.d . ) , amoxicillin / clavulanic acid ( 625 mg t.d.s . ) , and haematinics . two weeks after treatment , the patient resumed arv therapy with truvada and lopinavir / ritonavir . power gradually improved to 4/5 in both limbs , and she started ambulating and was discharged to follow - up as an outpatient . a 35-year - old female was referred to the university of calabar teaching hospital , nigeria , with a 2-day history of left - sided weakness affecting her leg and arm and focal seizures involving her left upper limb . she had a history of hiv infection , diagnosed 4 years prior to the current presentation and treated with arv drugs . her last cd4 + count ( 6 months before ) was 28 cells / mm . on neurologic examination , there was left hemiparesis ( grade 3:5 on the medical research council scale for both left upper and lower limbs ) with increased tone and brisk reflexes and upgoing plantar reflexes ( babinski sign ) . an urgent brain ct showed a rounded marginally enhancing hyperdense lesion with perilesional oedema in the right thalamus ( fig . there was obliteration of the posterior horn of the ipsilateral lateral ventricle by the aforementioned mass . a similar but ill - defined lesion with more extensive perilesional oedema blood tests showed haemoglobin 10 g / dl , esr 106 mm in the first hour , cd4 + count 24 cells / mm and toxoplasma igg antibody 300 iu / l . based on these findings , a diagnosis of cerebral toxoplasmosis presenting with a stroke - like syndrome , on the background of hiv treatment failure , was made . the patient was discharged home to continue follow - up at the medical outpatient department .

toxoplasmosis is the most common opportunistic infection of the central nervous system in patients with acquired immunodeficiency syndrome ( aids ) . clinical presentation of cerebral toxoplasmosis in these patients includes headache , focal neurological deficits and seizures . prompt diagnosis and appropriate therapy results in rapid clinical and radiological improvement as well as good outcome for patients . in this article , we report two cases with stroke - like presentation of cerebral toxoplasmosis in the setting of hiv infection .

facioscapulohumeral muscular dystrophy ( fshd ) is a common inherited muscular dystrophy presented clinically with slowly progressive weakness and wasting of facial and limb muscles and rare bulbar muscle involvement . we present herein a known case of fshd presented with recent onset of severe bulbar symptoms and was found to have myasthenia gravis ( mg ) based on electrodiagnostic study , elevated level of acetylcholine receptor antibody and dramatic improvement with choline esterase inhibitor agents . clinical presentation , electrodiagnostic and pathologic findings of this patient are described . a 70-year - old man presented to our department with complaint of 15-day history of progressive difficulty in chewing and dysartheria . he had a 50-year history of slowly progressive asymmetrical weakness of proximal upper limb muscles . examinations revealed reduction in forces of bilateral orbicularis oculi muscles , weakness and wasting of bilateral triceps muscles especially on right side and bilateral winging of scapula more prominent on right side . the legs and pelvic girdle muscles had normal forces . edrophonium test was performed and dysartheria and chewing difficulty showed dramatic improvement but had no effect on limb weakness . muscle biopsy showed myopathic changes with invariability in muscle fiber size , intramuscular infiltration of chronic inflammatory cells , mostly lymphocytes , few hyaline fibers and prominent fat infiltration . our patient 's bulbar symptoms showed dramatic improvement following administration of choline - esterase inhibitor agents . a 70-year - old man presented to our department with complaint of 15-day history of progressive difficulty in chewing and dysartheria . he had a 50-year history of slowly progressive asymmetrical weakness of proximal upper limb muscles . examinations revealed reduction in forces of bilateral orbicularis oculi muscles , weakness and wasting of bilateral triceps muscles especially on right side and bilateral winging of scapula more prominent on right side . the legs and pelvic girdle muscles had normal forces . edrophonium test was performed and dysartheria and chewing difficulty showed dramatic improvement but had no effect on limb weakness . muscle biopsy showed myopathic changes with invariability in muscle fiber size , intramuscular infiltration of chronic inflammatory cells , mostly lymphocytes , few hyaline fibers and prominent fat infiltration . our patient 's bulbar symptoms showed dramatic improvement following administration of choline - esterase inhibitor agents . it presents clinically with slowly progressive weakness and wasting of facial and shoulder girdle muscles and sometimes involvement of lower extremities . the clinical severity is wide ranging from asymptomatic individuals to wheel - chair dependent patients . any unusual changes in course of disease or development of unusual symptoms should raise the possibility of concomitant disease . sansone et al . reported a 69-year - old known case of fshd who presented with sudden deterioration of limb weakness and development of bulbar symptoms and was found to have mg based on repetitive nerve stimulation , elevated level of acetylcholine receptor - binding antibody and dramatic improvement following immunomodulator administration . reported a 50-year - old man with a 35 year history of fshd who presented with acute progressive weakness of lower extremities three weeks prior to admission . the patient was found to have mg based on decrement response on repetitive nerve stimulation , elevated level of acetylcholine receptor - binding antibody and improvement after thymectomy and administration of corticosteroid . in another report , mcgonigal et al . presented a 56-year old newly diagnosed myasthenic patient who found to have 40-year history of progressive foot drop and although our case is a rare coexistence of fshd and mg , low prevalence of both diseases , may raise the possibility of the presence of other etiologies . theoretically , it is related to breaking of immune tolerance to achrs as a result of muscle fiber degeneration . patients with genetic myopathies may occasionally develop antibodies to achr . while these antibodies may not have pathogenic effects , their production is likely to be a consequence of sensitization to achr secondary to muscle fiber damage , rather than through an immune process in thymus . muscle histopathological examination in some cases of fshd shows inflammatory changes , disproportionate to muscle fiber necrosis but the presence of mononuclear infiltration does not affect disease progression and the patients do not benefit from prednisone treatment . to the best of our knowledge , there are limited reports of concomitant occurrence of mg and fshd in literatures . although the association of mg with fshd in our patient could be an incidental finding , it may raise the possibility of innate immune response , i.e. autoinflammation in development of achr antibodies in genetic myopathies or it may suggest immune mechanisms in pathogenesis of fshd . moreover , our experience may warrant concomitant neuromuscular disorder in patients with unusual symptoms of fshd .

facioscapulohumeral muscular dystrophy ( fshd ) is a common inherited muscular dystrophy presented clinically with slowly progressive weakness and wasting of facial and limb muscles and rare bulbar muscle involvement . we present herein a 70-year - old man who was a known case of fshd with complaint of 15-day history of progressive difficulty in chewing and dysarthria and was found to have myasthenia gravis . related literatures have been also reviewed .

digital radiograph or radiovisiography was first described in 1987 and was commercially introduced by trophy radiologie in collaboration with mouyen12 in 1993 . three systems are employed for radiovisiography : the ccd sensor ( charge - coupled device ) , cmos sensor ( complementary metallic oxide sensor ) , and psps ( psps phosphor storage plate optical plate ) ; the latter includes the digora , digident , denoptix and den ortix systems11 . however , some doubts remain in the use of radiovisiography based on the optical plate system as to the durability of the plate3 , image quality according to the processing delay1,2,14 , and storage conditions10 . no study has yet investigated the influence of utilization of opaque cases on the quality of digital image . this ex vivo study evaluated the quality of digital radiographic images obtained with the digora system according to the processing delay and maintenance of optical plates in either opaque ( supplied with the system ) or transparent protective plastic cases during this period . the study was conducted on a dry human mandible fixed on an acrylic resin base . a plastic tube was fixed frontally to the mandible to standardize the position of the x - ray source , thus standardizing the vertical and horizontal angles ( figure 1 ) . a groove was prepared on this resin base to standardize the position of the optical plate . radiographs were obtained from the mandibular molar region by utilization of optical plates with an x - ray unit ( gnatus model xr 6010 ; gnatus , ribeiro preto , sp , brazil ) set at 60 kvp and 10 ma , with open tube , total filtration of 1.5 mm al , exposure time of 0.3 s , and optical plate of the digora system ( soredex , orion corporation , finland ) . two groups were constituted for x - ray exposure : in group a , the optical plate was inserted and sealed in the opaque plastic case supplied by the manufacturer and kept as such until processing ; in group b , the optical plate was stored in a transparent plastic case ( figure 2 ) . the optical plates were processed in the digora scanner immediately and 5 , 60 and 120 min after x - ray exposure , and were transferred to the computer for analysis of digitized images . analysis was performed directly on the computer screen by three examiners using the digora for windows software . the images were analyzed as to brightness , contrast and resolution of tooth structures ( enamel , dentin , root and pulp cavity ) and periapical structures ( periodontal space , cortical bone , alveolar bone ) , which received scores 0 to 2 , as follows : 0- poor image quality ; 1- good image quality ; 2- excellent image quality . data were submitted to the kruskal - wallis non - parametric test , and comparison of results between opaque and transparent cases were submitted by the mann - whitney test . table 1 presents the scores assigned by examiners to the images , according to processing delays and utilization of opaque or transparent plastic cases . there was statistically significant difference ( p<0.05 ) regarding the processing delays , namely immediate , 5 and 60 min with utilization of opaque case ( table 2 ) . this difference was observed between the 120-min delay and the other periods ( table 3 ) . no statistically significant difference ( p>0.05 ) was observed among processing delays with utilization of transparent case ( table 4 ) . comparison of results between opaque and transparent cases did not reveal significant differences ( p>0.05 ) ( table 5 ) . 10.05882 ; chi - square with 3 degrees of freedom ; probability = 0.018072 ( significant ) u=5.00000000;exact probability = 0.150794 ; normal approximation : z=1.96396101 ; probability = 0.04953461 hc= 5.164999 ; chi - square with 3 degrees of freedom ; probability = 0 . digital radiography was introduced to dental practice with a view to replace the conventional radiography due to the shorter exposure to radiation , achievement of high - quality images , possibility of adjustments with aid of software , and comparable reliability to conventional radiography , for both endodontic diagnosis and treatment3,57 , the main disadvantage of the optical plate system is the need of additional time for processing and possible loss of quality when the sensitized optical plate is exposed to light . some authors have emphasized that , after exposure to x - ray , the optical plates should be processed within 1 h in order to avoid loss of image quality1,2 . other authors believe that the plates can be processed within 6 h if stored in appropriate cases10 . in the present study , a processing delay of 120 min caused a mild reduction in image quality , yet without compromising the quality of diagnosis ( table 1 ) . it should be highlighted that the images were not altered as to the brightness and contrast provided by the system , being analyzed as produced on the computer screen . together with the digora system , the manufacturer supplies opaque plastic cases that offer protection against contamination and the deleterious effect of light . images of optical plates maintained in the original opaque cases after exposure to x - ray maintained their image quality up to 120 min ( table 2 ) , with significant difference between the 120- min processing delay and the other periods ( table 3 ) . when the optical plates were maintained in transparent cases , there was also a progressive reduction of image quality ( table 1 ) , which was more accentuated at 120 min ( table 4 ) , though without statistical difference among periods . this is due to the fact that the optical plate protected with transparent cases is subjected to the continuous action of light , with progressive loss of image quality . however , images obtained with optical plates protected with both opaque and transparent cases and with processing delays of up to 120 min provided good quality for diagnosis . the findings of the present study revealed that the processing delay of 120 min for the digora system caused a reduction in image quality , yet without interfering with the quality of diagnosis . the opaque case supplied by the system 's manufacturer provided better protection to the optical plate than the transparent case .

this ex vivo study evaluated the quality of digital radiographic images obtained with the photostimulable phosphor plate system ( digora ) according to the processing delay and maintenance of optical plates in either opaque ( supplied with the system ) or transparent protective plastic cases during this period . five radiographs were obtained from the mandibular molar region of a dry human mandible using optical plates . these plates were placed in the protective plastic cases before obtaining the radiographs and were processed immediately or after processing delays of 5 , 60 and 120 min , when the case was removed . the results revealed a reduction in image quality when processing was delay 120 min compared to the other times . the opaque case provided better protection to the sensor than the transparent case . in conclusion , a 120-min processing delay for the digora system caused a reduction in image quality , yet without interfering with the quality of diagnosis . the opaque case supplied by the system 's manufacturer provided better protection to the optical plate than the transparent case .

five provinces in afghanistan were selected as a convenience sample ( accessibility ) that included both affected and unaffected areas . two accessible districts in each province were randomly selected by using a random number generator . random transects were used to select 10 households per village . to give an approximately equal male : female ratio , either the head of household , spouse ( woman ) , or the oldest person available at the time was selected . ethical approval was provided by the institutional review board , ministry of public health , afghanistan . a standardized , structured questionnaire collected information on demographic and socioeconomic measures , avian influenza information sources and knowledge of appropriate preventive measures , poultry and animal handling , food and generic hygiene , and human influenza knowledge and treatment seeking . the questions were ranked for importance in preventing avian influenza transmission in poultry or reducing human exposure and awarded 5 points , 3 points , or 1 point for correct answers . for each respondent , the sum of scores for correct answers divided by the sum of available points generated a percentage score . data were double - entered by using microsoft access ( microsoft , redmond , wa , usa ) and analyzed by using stata 8 software ( stata corporation , college station , tx , usa ) . percentage scores for each respondent were ranked and classified as above or below the median . the primary analysis was conducted to compare factors ( age , sex , socioeconomic status , provincial exposure to avian influenza iec campaigns , and poultry ownership ) associated with knowledge above the median . socioeconomic quintiles ( seqs ) were defined by principle components analysis using employment , education , and household assets as indicators ( 3 ) . factors independently associated by univariate regression at the 95% confidence level were included in a stepwise multivariate logistic regression model . to numerically evaluate kap levels , a secondary analysis assessed differences between mean percentage scores , stratified by factors identified by logistic regression analysis . data for 304 respondents were included in the analysis . of the 5 provinces , kabul and nangahar had had influenza outbreaks in poultry in 2007 . median age of respondents ( 38 years ) was high , but it reflected the age of heads of households and spouses . poultry ownership was reported by 65.2% of households ( > 95% backyard ownership ) and differed significantly between seqs ( poorest 53/62 [ 85.5% ] vs. least poor 20/55 [ 36.4% ] ; 30.0 , p<0.001 ) . provinces exposed to avian influenza and intensive information , education , and communication campaigns ( kabul , march 2007 , and nangahar , february 2007 ) . seq was positively associated with kap score above the median ( lowest vs. highest : adjusted odds ratio [ aor ] 14.3 , 95% confidence interval [ ci ] 5.239.9 ) , as was provincial exposure to avian influenza iec campaigns ( aor 9.5 , 95% ci 4.918.6 ) . backyard poultry ownership ( nonowners vs. owners : aor 0.3 , 95% ci 0.20.7 ) and older age group ( 1520 years vs. > 40 years : aor 0.3 , 95% ci 0.10.8 ) were both negatively associated . for secondary analysis , mean kap score differed between seq ( p<0.001 , by analysis of variance ) and was higher in provinces previously exposed to iec campaigns ( 50.2% vs. 40.1% ; p<0.001 , by t test ) . reporting of sick or dead poultry to authorities was less frequent among lowest seq ( 8/47 [ 13% ] ) than highest seq ( 20/49 [ 37% ] ; 6.6 , p = 0.02 ) where selling poultry in the event of a local outbreak was more commonly reported ( 21/66 [ 66% ] vs. 10/51 [ 18% ] ; 27.2 , p<0.001 ) . presence of coops was less frequent in lowest seq ( 9/49 [ 18.4% ] ) than in highest seq ( 21/46 [ 45.6% ] ; 8.2 , p = 0.004 ) . human cases of avian influenza ( h5n1 ) have resulted from contact between humans and infected backyard poultry ( 4 ) . risk to humans is also related to frequency of disease occurrence in the avian population ( 5 ) . recently , human - to - human transmission has been reported in the neighboring northwest frontier province of pakistan ( 2 ) . overall knowledge was low , although in provinces exposed to intensive iec campaigns , kap scores of the population were higher . the level of concern generated by the campaign , government response , media reports , and proximity to the outbreak are all likely to contribute to this association . despite this encouraging evidence , level of knowledge was far higher among persons with higher socioeconomic status . our results can be broadly generalized to the population , although we did not have access to unsafe districts ( most of the districts in southern and eastern afghanistan ) . this limitation may introduce selection bias , which would underestimate the effect of socioeconomic status because those living in inaccessible areas likely have a lower status than persons in accessible areas . preintervention and postintervention surveys would provide a more robust measure of effectiveness . in the immediacy of an outbreak , although there are limitations to the study design in concluding intervention effectiveness , the results provide evidence to support further intensive campaigns as a response to influenza outbreaks in poultry . several reports have examined kaps and behavior related to avian influenza ( h5n1 ) ( 69 ) . similar to the finding in the lao people s democratic republic ( 6 ) , our study suggests that conventional education and behavior change messages have a limited effect in populations with highest exposure . efforts to ensure that iec messages are suitable for lower socioeconomic groups should be adopted , specifically by improving the knowledge of community leaders , designing messages in a suitable format for the poor and illiterate , and ensuring that the most accessible channels are used . messages should carefully balance the risk for human disease against potential nutritional and economic consequences of high population concern ( e.g. , food scares ) . successfully promoting behavior change is a lengthy process and requires frequent reinforcement . the acuteness of avian influenza ( h5n1 ) outbreaks requires a concerted effort to enhance knowledge and change behavior among those most at risk in low - income countries .

from february through april 2007 , avian influenza ( h5n1 ) was confirmed in poultry in 4 of 34 afghan provinces . a survey conducted in 2 affected and 3 unaffected provinces found that greater knowledge about reducing exposure was associated with higher socioeconomic status , residence in affected provinces , and not owning backyard poultry .

linear psoriasis is a rare form of psoriasis , characterized by a linear distribution of the psoriatic lesions along blaschko 's lines . the pathogenesis of linear psoriasis is not clear , but it could be explained by the well - established concept of genetic mosaicism . a 56-year - old previously healthy woman presented with a 3-month history of multiple confluent erythematous papules and plaques covered by scales . the papules and plaques had well - defined and sharply demarcated boundaries , ranged from 3 to 10 mm in diameter , were limited to the right side of her body and were arranged along blaschko 's lines with a marked mid - line cutoff . the lesions were first noticed in the right scapular region and progressively extended within a few weeks to the lateral aspect of the right superior limb , the right side of the trunk , the abdomen and the anterior aspect of the inferior limb , especially pronounced in the proximal third of the thigh and the knee ( fig . the patient denied any episodes of infection preceding the dermatosis . no psoriasis - form nail or scalp lesions were observed , and there was no personal or family history of psoriasis . the histopathologic analysis of a skin biopsy revealed orthokeratosis , parakeratosis and spongiosis - form munro 's microabscesses , in association with a regular elongation of rete ridges , suprapapillary thinning , and an absence of granular cell layers ( fig . a slight improvement was noticed after 1 month of combined treatment with betamethasone valerate and calcipotriol ointment . after complete laboratory exams including blood cell count , renal , hepatic and lipid panels , hbv and hcv serologies , and a chest radiograph , the patient was started on methotrexate 10 mg weekly with excellent response . in the 26th week of treatment , as only residual lesions persisted , the methotrexate dose was reduced to 7.5 mg weekly and maintained until the present time ( cumulative dose of 465 mg ) with no clinical relapse . there are few cases reported in the literature [ 1 , 2 , 3 ] , and therefore there is no estimated prevalence . also because of its clinical and histological similarity to ilven , the main differential diagnosis is ilven , and the distinction between the two entities has been discussed in the literature [ 4 , 5 , 6 ] . a late onset of asymptomatic to slightly itchy lesions with a possible involvement of scalp and nails and a favorable response to antipsoriatic treatment favors the diagnosis of linear psoriasis [ 8 , 9 ] . in contrast , ilven lesions usually occur in the first months of life , are slowly progressive , very pruritic and highly refractory to antipsoriatic therapy . as for the histological features , ilven classically demonstrates hypergranulosis and parakeratosis alternating with hypogranulosis and orthokeratosis . on the other hand , the classic features of psoriasis are hyperkeratosis , parakeratosis , the absence of granular cell layers , an elongation of rete ridges , suprapapillary thinning and munro 's microabscesses , which can also be seen in ilven . immunohistopathologic studies may be helpful in distinguishing the two conditions , as done by vissers et al . : in ilven patients , the number of ki-67-positive nuclei tended to be reduced , whereas the number of keratin-10-positive cells was increased compared with psoriasis patients . involucrin expression , another marker , is absent in ilven patients but detectable in psoriasis . the pathogenesis of linear psoriasis is not clear , but it could be explained by the well - established concept of genetic mosaicism .

a 56-year - old woman presented with a 3-month history of erythematous plaques covered by scales and limited to the right side of her body . the plaques were arranged along blaschko 's lines with a marked mid - line cutoff . the histopathologic analysis of a skin biopsy in conjunction with the anamnesis allowed the diagnosis of linear psoriasis . our patient showed a good clinical response to antipsoriatic treatment .

malignant tumors which originated from mesenchymal tissue , has occurred very rarely in the breast . the metaplastic carcinomas which have characterized by a combination of mesenchymal and epithelial components would be uncommon malignancies of the breast . the chondrosarcoma has been a typical example of these rarest tumors of the mesenchymal tissue . a 65 years old man has presented in our department with a complaint of a right sided breast mass which has rapidly grown for 5 months . breast palpation has revealed a painless , less mobile , and firm to hard mass of 1010 cm with regular margins in area of right breast . contrast enhanced mri ( figures 1a and b ) of breasts has revealed huge mixed signal intensity multi lobulated cystic - solid extra - pulmonary mass lesion involving the right anterior chest wall extending from infra clavicular region to the level of xiphisternum measuring 10.4 cm ( si ) 10.3 cm ( ap ) 9.9 cm ( trans ) appearing predominantly hyper intense on t2w and hypo intense on t1w . heterogeneous predominantly peripheral solid tumoral enhancement has observed with multiple large loculated central areas of necrosis and intensely enhancing septae . histopathology has revealed the overall appearances of low - grade chondrosarcoma with a tumor composed of lobules of cartilage of varying size separated by fibrous tissue . immunohistochemical studies have performed by standard revealed that chondrosarcomatous elements were positive for s-100 and vimentin , but negative for cytokeratin and also for estrogen and progesterone receptors . this paper has discussed one of the rarest mesenchymal malignancies of the breast , primary chondrosarcoma . as a primary breast tumor , chondrosarcoma might occur in three different forms : as a pure neoplasm ( pure chondrosarcoma ) , as the stromal component of a histologically malignant phyllodes tumor , or as chondrosarcomatous differentiation in a metaplastic carcinoma . in this report prognosis of chondrosarcomatous breast tumors has not fully known , because many of the reported cases were difficult to analyze ( owing to lack of detailed clinical or morphologic information ) . these tumors were usually large - sized that have occurred in more than 40 year old woman . axillary lymph nodes have found in 14 - 29% of the cases , most of which were reactive hyperplasia . the present case substantiates the clinical findings of previously reported cases . to diagnose a primary chondrosarcoma of the breast , other types of pure sarcoma , such as spindle cell sarcoma , neuroectodermal tumor , angiosarcoma etc , have also been reported [ 6 , 7 ] . the history of the patient had an important feature as the mass had grown rapidly without systemic and deleterious effect on the patient s health status . these tumors have tended to grow rapidly and present themselves as a mass for a short duration [ 2 , 3 , 7 ] . a large multilobular mass with regular margins and without a sign of regional invasion has detected by palpation . previous reports have also pointed out that these large tumors have not generally invaded skin and regional lymph nodes despite their locally advanced nature [ 2 - 4 , 7 ] . a relatively circumscribed , well demarcated mass , as in our patient , was an imaging characteristic of a pure mesenchymal or a metaplastic carcinoma with sarcomatoid differentiation . it has been redported that imaging studies seldom led to the diagnosis of sarcoma in a suspected benign lesion . the mass was complex echoic on ultrasound and has shown round hyperdense opacity on mammography [ 4 , 7 , 8 ] . the very limited number of such cases has not permitted us to establish an appropriate therapeutic approach . chondrosarcoma in common locations has generally known as refractory to all types of conventional chemotherapy and radiotherapy . pure chondrosarcoma and metaplastic cancer of the breast has rarely invaded axillary lymph nodes and would be generally hormone receptor - negative [ 1 , 2 , 4 , 11 ] . this extremely rare tumor has tended to grow rapidly , and it was usually large at first physical examination . a large , hyper dense and complex echoic mass with regular margins has given the impression of a benign tumor on mammography and ultrasound . despite its large size , it has not invaded local and regional structures . regarding systemic management , there was no standard treatment protocol , and a large variety of chemotherapy protocols have been employed in treating this disease . systemic therapy principles have been derived from small retrospective case reviews of primary breast chondrosarcomas and extrapolated from studies of non - breast chondrosarcomas , since the clinical behavior and histology were similar . this fact has been supported the theory that adjuvant therapy with estrogen antagonists and other hormone manipulations had no role in treatment of mammary sarcomas . the adjuvant treatment could decrease the rates of local and systematic recurrences , but the results were not significant because of the rarity of this pathological entity and the small numbers of cases have reported , which made the evaluation of the role of the chemotherapy and the radiotherapy in the primary breast chondrosarcoma more difficult . it has seemed to be very important to identify the mammary primary sarcomas as entity separated from the carcinomas of the breast . the primitive chondrosarcoma has remained a rare pathology , among which the therapeutic modalities and the forecast were credibly identical to those of the sarcomas of the same type arising in the other localizations . it has seemed to be very important to identify the mammary primary sarcomas as entity separated from the carcinomas of the breast . the primitive chondrosarcoma has remained a rare pathology , among which the therapeutic modalities and the forecast were credibly identical to those of the sarcomas of the same type arising in the other localizations .

breast sarcomas have relatively been rare and accounted for 1% of all primary malignant tumors of the breast . pure and primary chondrosarcoma of the male breast would be an extremely rare tumor . it might arise either from the breast stroma itself , or from underlying bone or cartilage . a 65-year - old man has presented with a rapidly growing breast mass since 5 months . physical examination has established a large firm to hard mass with regular margins in the region of right breast . there was no axillary lymphadenopathy . contrast enhanced mri of breasts has shown a mixed - signal intensity multi lobulated cystic - solid mass ( 10.4 cm 10.3 cm 9.9 cm ) appearing predominantly hyper intense on t2w and hypo intense on t1w . the tumor has diagnosed as a low - grade chondrosarcoma of the breast by histopathological and immunohistochemistry analysis . right sided radical mastectomy with grafting has done . it has seemed to be very important to identify the mammary primary sarcomas as entity separated from the carcinomas of the breast .

in clinical practice , one may encounter female patients with infertility which may be attributed to primary or secondary causes . in the group of primary infertility , congenital anomalies form relatively small but significant group . mayer - rokitansky - kuster - hauser syndrome ( mrkh ) is characterized by congenital absence of the uterus and vagina . there is a failure of development of the mullerian derivative of the vagina and the portions of the mullerian ducts that form the uterus . the american fertility society classification , based on uterine anomalies is most commonly used to classify mullerian duct anomalies . mrkh syndrome is a class 1 mullerian duct anomaly , and it has been subdivided into typical or type a and atypical or type b. patients with type a syndrome have symmetric muscular buds , normal fallopian tubes . patients with type b have asymmetric muscular buds , abnormal fallopian tubes , and may be associated with other congenital anomalies such as renal , skeletal , hearing , cardiac , and ocular anomalies . out of few genes which are identified in causation , one candidate is a mutation of the wnt4 gene , a developmental gene which regulates growth and differentiation during embryogenesis . women with this syndrome are characterized by the presence of 46 xx karyotype , normal female secondary sex characters , normal ovarian functions , and underdeveloped vagina . the presence of leiomyoma in mrkh syndrome is very rare , and only a few cases have been reported in the literature . here , we report a case of mrkh syndrome ( type a ) with multiple leiomyomas originating from the rudimentary uterus . twenty - five years married female presented to the gynecology outpatient department with primary amenorrhea . secondary sexual characters revealed the presence of pubic hair and axillary hair ( tanner stage 5 ) [ figure 1a ] . ultrasonography examination showed agenesis of the uterus with right adnexal mass measuring 5 cm 4 cm 4 cm . differential diagnosis of leiomyoma of the rudimentary uterus in mrkh syndrome includes ovarian fibroma , a gastrointestinal stromal tumor of the intestine and extravesical leiomyoma of the urinary bladder . values were : serum follicle stimulating hormone 7.17 miu / ml ; serum leutenizing hormone 15.6 miu / ml ; serum prolactin 10.64 ng / ml ; and cancer antigen 125 4.5 iu / ml . with a provisional diagnosis of mrkh syndrome associated with right adnexal mass , she was taken for laparotomy . a rudimentary bicornuate uterus measuring 1.5 cm 1.5 cm with a small cervix measuring 1 cm 1 a total abdominal hysterectomy was performed in view of the presence of multiple nodules in a nonfunctional uterus . multiple myomertial nodules [ figure 1b ] were seen arising from the rudimentary right and the left horns of the uterus [ figure 1c ] . on cut section of the specimen , uterine horns were found noncommunicating , and they had a rudimentary endometrial cavity . all the nodules were well circumscribed , grayish white and had a characteristic whorled appearance . histological examination revealed features of leiomyoma [ figure 1d ] with rudimentary mullerian lining [ figure 1e ] . failure of fusion and development of mullerian ducts results in muscular thickening at the proximal end of each tube that are joined in the midline by a visible and palpable cord resembling hypoplastic bicornuate uterus without an endometrial lining . rarely , an active endometrium can exist with uterine analog , which becomes active in the presence of well - estrogenized state . in literature , it is mentioned that fibroids and adenomyosis rarely develop in the rudimentary nonfunctioning uterus . cytogenetic abnormalities in the form of spontaneous chromosomal rearrangements are known to occur in uterine leiomyomas . these chromosomal arrangements may be responsible for the initiation and progressive growth of the leiomyomas . the leiomyomas are found to have higher concentration of estrogen receptors as compared to normal myometrium . these might explain the propensity to develop leiomyoma in the presence of exogenous and endogenous estrogen . as the proximal ends of mullerian ducts have smooth muscles , the presence of leiomyoma in a case of mullerian agenesis is a theoretical possibility . however , the occurrence of multiple leiomyomas in a rudimentary uterine bulb has been rarely reported earlier . the possible reason for this uncommon occurrence could be a decreased concentration or sensitivity of the estrogen receptors or a lesser genetic predisposition for the clonal chromosomal abnormalities that are observed in women with normal uterus with leiomyomas . in our patient , we could not perform cytogenetic and receptor studies to point out the exact etiopathogenesis of this unique occurrence of multiple leiomyomas in the rudimentary uterine bulbs . hysterectomy was performed in our case in view of nonfunctioning uterus and risk of recurrence after myomectomy . treatment for patients of mrkh syndrome with leiomyoma is myomectomy or hysterectomy and vaginal reconstruction ( vaginoplasty ) . frank 's dilatation method , williams vaginoplasty , vecchietti procedure , abbe - mcindoe vaginoplasty and davydov technique . with advances in minimal access surgery the ovarian function is normal in these patients so that ovum can be retrieved and with the help of a surrogate mother , it is possible to have fertility potential .

mayer - rokitansky - kuster - hauser ( mrkh ) syndrome is a rare disorder described as aplasia or hypoplasia of uterus and vagina due to an early arrest in development of mullerian ducts . women with this syndrome are characterized by the presence of 46 xx karyotype , normal female secondary sex characters , normal ovarian functions , and underdeveloped vagina . the presence of leiomyoma in mrkh syndrome is very rare , and only few cases have been reported in the literature . here , we report a case of mrkh syndrome with multiple leiomyomas originating from the rudimentary horn of uterus in 25 years married , phenotypically female patient .

although traditionally malignant melanoma has been considered a tumor of the caucasians , recently , an increasing incidence is being noted in india . we present here a case of malignant melanoma , resembling a vascular tumor ; the first of its kind from the indian subcontinent . a 40-year - old gentleman presented to us with multiple asymptomatic , pigmented , nodular lesions , resembling cluster of grapes , distributed over the medial aspect of right lower limb . initially , there was a single nodular lesion , which rapidly increased in size over the next 3 months ; gradually new nodules arose in the vicinity which coalesced with each other to form a plaque . he also complained of heaviness due to a mass in his right groin . his past medical and surgical history including drug history were unremarkable . fever or other constitutional symptoms were absent except for the presence of significant weight loss and occasional episodes of bleeding from the lesions when subjected to trivial trauma ; he also complained of occasional breathlessness and two episodes of bloody expectoration following cough . on dermatological examination , multiple dome - shaped nodular lesions were found on the distal part of right lower limb ; ranging from 2 - 3 mm in diameter to plaque of 5 cm 7 cm with bosselated surface ; formed by the coalescence of individual nodules [ figure 1 ] . the color varied from light to dark mottled brown with some lesions showing atrophy and superficial ulceration . on palpation , they were firm , nontender nodules with the smooth , glossy surface , with woody hard induration of the underlying skin . general survey was normal except for the presence of pallor and a palpable mass in the right inguinal region sized about 7 cm 3 cm which was hard and immobile [ figure 2 ] . routine hematologic and biochemical investigations were within normal limits except for the presence of anemia ( hb - 8.4g / dl ) and an elevated erythrocyte sedimentation rate ( 55 mm / h ) . the patient was referred to the department of general medicine to evaluate his respiratory complaint and rule out any other evidence of systemic metastasis . ( a and b ) multiple brownish dome - shaped nodular lesions with a bosselated surface , right lower limb right inguinal lymphadenopathy on histopathological examination ( hpe ) , sections showed a proliferative growth centered in the upper dermis with marked thinning of the epidermis [ figure 3 ] . tumor cells were arranged in nests with focal infiltration of adnexa [ figure 4 ] . the cells were polygonal with hyperchromatic nuclei , perinuclear halo , and prominent nucleoli ; also inclusion bodies were found with conspicuous mitotic activity [ figure 5 ] . proliferative growth in the upper dermis with marked thinning of epidermis ( h and e , 40 ) lobulated mass showing closely packed pigmented cells ( h and e , 100 ) the mass showing clustered polygonal cells with hyperchromatic nuclei and perinuclear halo ( h and e , 400 ) clark et al . divided malignant melanoma into three main subsets , by using a combination of clinical and pathological features ; the superficial spreading melanoma , the nodular melanoma , and the lentigo maligna melanoma . the national institutes of health consensus conference on the diagnosis of melanoma has suggested the use of the asymmetry , border irregularity , color variegation , diameter > 6 mm checklist for the detection of melanocytic lesions . risk factors for the development of melanoma may be divided into three categories : phenotypic manifestations of gene / environment interactions , environmental factor , and genetic factors . the histological diagnosis of melanoma is based on the assessment of a constellation of findings , including both architectural and cytological features . cytological atypia , nuclear enlargement , nuclear pleomorphism , hyperchromasia of nuclei , nucleolar variability , and the presence of mitoses especially deep in the dermis , is considered necessary for a diagnosis of melanoma . there is a lack of maturation of nests of melanocytes with a descent into the dermis . pagetoid spread of large solitary epidermal melanocytes is usually considered diagnostic of melanoma . in our case , kaposi sarcoma is multifocal , endothelial proliferation predominantly involving the skin and other organs and associated with the formation of vascular channels and proliferation of the spindle - shaped cell . clinically , skin lesions vary from small violaceous papules to large plaques to ulcerated nodules . initially , the upper body is involved , often along skin lines in a pityriasis rosea - like pattern and at sites of local trauma . lesions develop on the face , in particular , the nose , and on oral mucosal surfaces , including the gums and hard palate . during tumor progression the color changes to brownish and the skin overlying the tumor becomes hyperkeratotic and , in particular on the lower extremities , may ulcerate . on hpe , tumor lobules are composed of bland , spindle - shaped cells with poorly defined , pink cytoplasm along with extensive vascular proliferation at all levels of the dermis . retiform hemangioendothelioma presents mainly in young adults , with no sex predilection , as a slowly growing , asymptomatic , dermal , and subcutaneous plaque or nodule . histology reveals arborizing , thin - walled , narrow , vascular channels with a striking resemblance to the rete testis . dusky blue or red nodules develop and grow rapidly , and fresh , discrete nodules appear nearby . in some cases , hemorrhagic blisters are a prominent feature . as the tumors grow , the edema may increase , and older lesions may ulcerate . on histology vascular channels infiltrate the normal structures in a disorganized fashion along with the characteristic dissection of collagen . in our case , none of the histological features of hemangioendothelioma or angiosarcoma was present . there have been reports of rare disseminated malignant melanoma presenting as multiple asymptomatic , nodular lesions on the trunk , extremities , and the face and a rare sporotrichoid pattern of malignant melanoma . however , our case is unique as it is malignant melanoma mimicking closely mimicking a vascular tumor ; where the final diagnosis could only be reached on hpe . very few reports have depicted an unilateral distribution of malignant melanoma ; in our case also the lesions are unilateral ; distributed only over the right lower limb ; this has further added to the uniqueness of our case . malignant melanoma is itself a rare disease in our set - up , further the unilateral distribution and clinical resemblance to vascular tumors has made our case even more unique ; all these features prompted the current case report . there are no conflicts of interest . a rare report of malignant melanoma from the indian subcontinent region , which clinically resembled a vascular tumor ; histopathological examination clinched the final diagnosis there are no conflicts of interest . a rare report of malignant melanoma from the indian subcontinent region , which clinically resembled a vascular tumor ; histopathological examination clinched the final diagnosis

malignant melanoma is an invasive neoplasm of the skin , whose incidence is reported to be rising among indians . we hereby present a unique case of unilateral , multiple , asymptomatic , pigmented , nodular lesions over the lower limb ; resembling vascular tumor , revealing itself as malignant melanoma only on histopathology . to the best of our knowledge , such a unique presentation of malignant melanoma has not yet been reported from the indian subcontinent .

the femoral approach is still the most commonly used route for diagnostic cardiac catheterizations and percutaneous coronary interventions . at the end of the procedure the femoral artery is usually compressed manually to control bleeding until hemostasis is achieved . local vascular complications such as bleeding and hematoma are not uncommon , ranging from less than 1% to 12% . many devices have been developed to aid in the closure of the femoral arteriotomy , including the vasoseal ( datascope inc . , jude medical devices , minneapolis , mn , usa ) , the starclose clip closure system ( abbott vascular , redwood city , ca , usa ) , and the perclose ( perclose , redwood city , ca , usa ) suture - mediated closure system . in two recent meta - analyses these devices have proven their efficacy in significantly reducing time to hemostasis while simultaneously improving patient comfort . vascular closure devices should be avoided or used with caution with non - common femoral artery sheath location , small femoral artery size , bleeding diathesis , morbid obesity , inflammatory disease , uncontrolled hypertension and in the presence of significant peripheral vascular disease , especially with calcification . in an attempt to reduce local complications , inflatable compression devices such as the femostop ( radi medical system ab , uppsala , sweden ) and the safeguard ( datascope corp . , fairfield , nj , usa ) ( figure 1 ) have thus been developed for compression of the femoral artery and are now in use in some centers because they are well accepted by the nursing staff . figure 1 the safeguard 24 cm pressure assisted dressing . the safeguard 24 cm pressure assisted dressing . we hereby report a clinical experience describing a significant complication occurring after safeguard use for femoral hemostasis . in this case , an extensive pressure ulcer developed in the groin of a very elderly patient in whom a safeguard had been applied overnight because of persistent oozing after diagnostic coronary angiography . a 95-year old diabetic female was referred to our catheterization laboratory for workup of percutaneous aortic valve replacement because of a severe calcified aortic stenosis and pronounced dyspnea on effort . the patient was anesthetized locally with 2% lidocaine and the femoral artery cannulation and placement of a 6 fr introducer sheath ( cordis , miami , fl , usa ) were performed by the usual technique without complications . angiography disclosed a significant stenosis of the mid tract of the right coronary artery which was treated with a 3.518 mm bare metal stent dilated at up to 20 atm , achieving a good final angiographic and clinical result . three hours after the procedure , when a check of the activating clotting time showed a value of 150 sec , the sheath was removed and manual compression was performed . after achieving hemostasis , a 24 cm safeguard pressure assisted device was positioned in the point of maximum femoral pulse and inflated with 40 ml of air , as recommended in the device package insert . no bleeding or oozing was found after manual compression or shortly after positioning the safeguard. two hours later the safeguard bulb was deflated but a persistent oozing was observed . similarly , 4 h after positioning the safeguard another deflation attempt was performed , but oozing was still present and the device was again inflated with 20 ml of air , and kept overnight . since the volume of inflated air was significantly lower than recommended , the nurse did not deflate the bulb every 2 h as suggested by the safeguard instructions . in the early morning of the following day nonetheless , a circular area of non - blanchable erythema was observed which developed into a pressure ulceration two days after ( figure 2 ) , with partial - thickness loss of dermis . the lesion was treated with frequent irrigations of saline solution , gentle manual debridement and hydrocolloid - based dressings ( 3 m tegasorb , minneapolis , mn , usa ) and resolved without any need for systemic antibiotics in a week , leaving , however , a minor scar . figure 2right groin 's extensive ( 129 cm ) pressure ulcer with loss of epidermis and dermis . right groin 's extensive ( 129 cm ) pressure ulcer with loss of epidermis and dermis . the 24 cm safeguard pressure - assisted device is a disposable device with a clear polyurethane bladder and a pressure sensitive self - adhesive peel backing . a luer valve enables a syringe to be connected to the device in order to inflate the central bladder with air to provide pressure to the puncture site . adverse effects reported by the manufacturer that may result from the use of this device include hematoma , local bleeding , pseudoa naurysm and arterio - venous fistula ; all complications associated with bleeding . to date , only a single prospective , non - randomized , multicenter study has been conducted in 101 low - risk patients to evaluate the safety and effectiveness of the safeguard space . the device proved to be effective in reducing active compression time without increasing risk in patients undergoing diagnostic and interventional procedures . the case report described here highlights how such a device , despite its inherently favorable features including user friendliness for staff , may seriously harm a patient if the instructions for use are not meticulously followed . given its inherent mechanical pressure properties , it is absolutely mandatory to deflate the bulb of the device every 2 hours to allow for capillary refill in accordance with the device instructions . even if long - term harm to the patient could be avoided , thus leading to an acceptable final cosmetic result , greater harm could have been caused , possibly with potentially dire medico - legal implications ( e.g. in the case of groin skin damage in a younger woman ) . vascular access complications are amongst the most common adverse events associated with coronary angiography and intervention . a number of devices are available to aid in achieving post - procedure homeostasis . information on vascular complications from access of the femoral artery , the methods of arterial closure , and post - procedural care instructions ( including avoidance of lenghty mechanical pressure with pneumatic devices ) can help physicians and nurses to provide safe and effective care to patients after cardiac catheterization .

diagnostic cardiac catheterizations are predominantly performed using the femoral artery access . several devices have been developed to aid in the closure of femoral arteriotomy.safeguard is a new pneumatic compression device that has been developed for compression of the femoral artery after brief manual compression . we hereby report the case of an elderly patient who underwent a percutaneous coronary intervention via the femoral artery in whom a safeguard device , left overnight because of persistent oozing , provoked an extensive pressure ulcer . knowledge of this potential complication is important to minimize its occurance and provide appropriate treatment .

in the search for new agents to protect the lung from injury , kuklin and colleagues report that tezosentan , a non - selective endothelin-1 receptor antagonist , reduces pulmonary edema in endotoxemic sheep , in parallel with a prevention of protein kinase c- activation . acute lung injury ( ali ) and its more severe form , acute respiratory distress syndrome ( ards ) , are syndromes of acute respiratory failure secondary to permeability , non - cardiogenic , pulmonary edema . ali / ards is a major cause of morbidity , death and cost in intensive care units . alveolar edema accumulates in ali / ards mainly because the permeability of the capillary ( endothelium)alveolar ( epithelium ) barrier is increased . this mechanism allows edema formation at normal capillary pressures and greatly increases the rate of edema formation at elevated capillary pressures . in addition , pressure elevation in the pulmonary circulation and mechanical stresses applied to the lung ( during mechanical ventilation , for example ) may cause " stress failure " in lung capillaries and alveoli , as evident in the formation of breaks and discontinuities in the endothelial and epithelial membranes of the blood gas barrier . the american european consensus conference on ards defined ali / ards as a " syndrome of inflammation and increased permeability " . hence , it is now widely accepted that the pathophysiology of ali / ards is driven by an aggressive inflammatory reaction that damages the alveolocapillary unit . after rapid recruitment of leukocytes to the inflamed site , there is activation of mediator cascades including the production of cytokines , chemokines , acute phase proteins , free radicals , complement , coagulation pathway components and focal upregulation of adhesion molecule expression units . several cytokines , such as tumor necrosis factor alpha ( tnf- ) , interleukin ( il)-1 , il-6 and il-8 , have been found in bronchoalveolar lavage fluid and plasma of patients with ards . inflammatory mediators amplify endothelial injury directly or by recruiting inflammatory cells into the vascular , interstitial and alveolar spaces . in addition , some mediators may also alter endothelial permeability by disturbing intracellular signaling pathways . tnf- and -thrombin have been shown to activate protein kinase c - mediated mechanisms that participate in the pulmonary endothelial response to agents involved in lung injury . protein kinase c is a ubiquitously expressed family of kinases that have a key role in regulating multiple cellular activities . activation of specific protein kinase c isoforms , most probably protein kinase c- , may cause lung endothelial dysfunction through several mechanisms . endothelins are a family of 21-amino - acid isopeptides . initially described as strong vasoconstrictors , for example , transgenic mice overexpressing endothelin-1 release increased amounts of tnf- , interferon- , il-1 and il-6 . the endothelin system is activated in clinical lung injury and in various types of experimental lung injury , and several endothelin receptor antagonists have been reported to exert protective effects in some models of ali / ards . endothelins may thus play a pathophysiologic role in ali / ards and promote pulmonary edema by increasing the filtration pressure ( as postcapillary vasoconstrictors ) , and also by increasing capillary permeability ( as inflammatory mediators ) . the results of kuklin and colleagues are in accordance with this hypothesis and provide further insight into an intracellular effect of endothelin receptor antagonists ( i.e. prevention of protein kinase c- activation ) . endothelin receptor antagonists have been made available recently for clinical use in the treatment of pulmonary arterial hypertension . these drugs may therefore offer new and attractive opportunities for the management of patients with ali / ards , especially because , to date , no pharmacologic agent has been convincingly shown to improve the prognosis of these patients . the data of kuklin and colleagues suggest that endothelin receptor antagonists might benefit patients with ali / ards . as expected from the use of a receptor antagonist , the level of the ligand increased ; that is , the plasma concentration of endothelin-1 was elevated after tezosentan treatment . as also expected from the proinflammatory properties of endothelin-1 , plasma levels of tnf- and il-8 increased after tezosentan treatment . the clinical relevance of these observations is unknown , but production of these cytokines might counteract the benefits expected from endothelin blockade . in other experimental models of ali / ards , endothelin receptor antagonists did not decrease intrapulmonary shunt , lung lymph flow or the histologically evaluated degree of parenchymal injury , despite a reduction in pulmonary vascular resistance . the work of kuklin and colleagues provides important information on the complex effects of endothelin blockers on inflammatory processes . from a clinical point of view , given the prolific nature of the inflammatory cascade causing ali / ards and the angel / devil effects of endothelin receptor antagonists on inflammatory mechanisms , it is unlikely that these single agents could reverse or terminate such a complex process . ali = acute lung injury ; ards = acute respiratory distress syndrome ; il = interleukin ; tnf- = tumor necrosis factor alpha .

acute lung injury is a syndrome of inflammation and of increased permeability of the blood gas barrier . endothelins are thought to exert proinflammatory effects . kuklin and colleagues show that the endothelin receptor antagonist tezosentan reduces pulmonary edema in endotoxemic sheep , in parallel with a prevention of protein kinase c- activation . in turn , the level of some cytokines increased after tezosentan treatment . whether these contrasting effects of endothelin blockade on inflammatory mechanisms have clinical relevance and whether these agents might benefit patients with acute lung injury is unknown .

the number of patients undergoing chemotherapy grows year by year . despite their efficacy in controlling the cancer , chemotherapy drugs are not inert , and they cause damage to the human body regarding nail involvement , diverse clinical features can be observed , according to the structure affected and the severity of the damage caused . the most common chemotherapeutic agents implicated in nail changes are cyclophosphamide , doxorubicin and hydroxyurea [ 2 , 3 ] . in the majority of cases , the effects are fully reversible after a few months once the causative agent has been withdrawn [ 2 , 4 ] . a 25-year - old male patient was admitted to the hospital 's hematology ward with the diagnosis of acute lymphocytic leukemia . the hyper - cvad regimen delivers a combination of drugs in 2 cycles ( cyclophosphamide , vincristine , doxorubicin and dexamethasone in cycle a , methotrexate and cytarabine in cycle b ) , split into smaller doses and administered at more frequent intervals in order to minimize side effects . during hospitalization , a dermatology assessment was requested . the dermatological examination showed nail injury characterized by transverse , brown - gray hyperpigmentation affecting the proximal half of the nail plate , with a brown band delimiting the healthy nail plate on all fingers ( fig . 1 , fig chromonychia induced by chemotherapeutic agents has different forms , the most common being melanonychia [ 2 , 4 , 5 ] . the dark pigmentation in nails can be diffuse , transverse or longitudinal and can coexist with skin and mucosal pigmentation [ 2 , 4 , 6 ] . it has been suggested that chemotherapy drug regimens influence the incidence of changes that may result from toxicity in the nail matrix , nail bed , periungual tissue and digits vessels [ 2 , 6 ] . although several chemotherapeutic agents can cause these changes , those most associated with these side effects are cyclophosphamide , doxorubicin and hydroxyurea [ 2 , 3 ] . cyclophosphamide is an alkylating agent whose main target is the cell cycle ; thus , tissues with high proliferation rates are more susceptible . alkylating agents form cross - links with dna filaments and prevent dna replication , therefore entailing cytotoxicity . cyclophosphamide can cause nail changes such as diffuse , black pigmentation , longitudinal striae ranging in color from slate gray to black , and diffuse , dark gray pigmentation located proximally , with overlying transverse , black bands [ 2 , 4 ] . this drug - induced nail pigmentation starts from the proximal edge of the nail and proceeds towards the distal edge . upon withdrawal of the drug it takes approximately 46 months [ 2 , 3 ] . in 60% of patients , cyclophosphamide is also associated with pigmentation of the oral mucosa and tongue , which also regresses upon withdrawal of the drug [ 2 , 4 ] . its side effects include skin and nail hyperpigmentation , alopecia , skin rash , pruritus , photosensitivity , urticaria , acral erythema and palmoplantar erythrodysesthesia . nail changes such as transverse , dark brown bands alternating with white stripes [ 2 , 7 ] and dark brown diffuse pigmentation bands 45 mm wide , which affect two - thirds of the distal portion of the nail , also occur [ 2 , 5 ] . additionally , doxorubicin can cause subungual keratosis , onycholysis and thickening of the nail plate . hydroxyurea causes an immediate inhibition of dna synthesis by acting as an inhibitor of ribonucleotide reductase , without interfering in the synthesis of ribonucleic acid or protein . this pigmentation has a diffuse , dark brown color which may appear as single or double transverse bands [ 2 , 4 ] . ungual hyperpigmentation induced by chemotherapy is not uncommon , although its occurrence is underestimated and seldom reported , especially in patients with dark skin phototypes , such as our patient , in this case , the drugs most probably implicated are doxorubicin and cyclophosphamide because they produce ungual alterations very similar to the ones seen in our patient ; his chemotherapeutic scheme included both of them .

chemotherapy drugs can affect the skin and its appendages . several clinical presentations can be observed , depending on the affected structure . the most common dermatological side effect is chromonychia . the main causative agents are : ( 1 ) cyclophosphamide , which can provoke a diffuse , black pigmentation , longitudinal striae and dark grey pigmentation located proximally on the nails ; ( 2 ) doxorubicin , which promotes dark brown bands alternating with white striae and dark brown pigmentation in transverse bands , and ( 3 ) hydroxyurea , which produces a distal , diffuse , dark brown pigmentation . in the majority of cases , the effects are reversible after the suspension of the causative agent for a few months . we report a patient who developed chromonychia while undergoing treatment with cyclophosphamide , vincristine , doxorubicin , dexamethasone , methotrexate and cytarabine for acute lymphocytic leukemia .

granuloma annulare ( ga ) was first described in 1895 by colcott - fox who reported an 11-year - old girl with a ring eruption of the fingers . the condition is a benign , usually self - limited granulomatous disease of the dermis and subcutaneous tissue . clinically , the condition is characterized by asymptomatic , flesh - colored or erythematous - brown papules , frequently arranged in a ring or annular pattern on the distal extremities . it may be skin - colored erythematous or violaceous measuring 1 - 5 cm in diameter . several clinical variants of ga have been reported , namely localized , generalized or disseminated , targetoid , giant , subcutaneous and perforating ga . we report an 18-month - old male child with multiple , progressive ga over the lower extremities . an 18-month - old male child presented with multiple annular lesions over lower extremities and buttocks . initially it started as a small papule over the left thigh , substantially attaining the present size . there was no history of fever , pain , drug intake or recent vaccination . on examination , on cutaneous examination , closely set , skin - colored , firm , smooth papules 2 - 3 mm in size arranged in a ring - like fashion to form annular plaques measuring approximately 3 3 cm ( eight lesions ) were present over dorsal aspect of foot [ figures 1 and 2 ] , both legs and buttocks . granuloma annulare over the left foot granuloma annulare over the right ankle routine hematological , biochemical and urine examinations were normal . venereal disease research laboratory test , human immunodeficiency virus 1 and 2 , hepatitis surface antigen test were negative and a biopsy showed features consistent with ga . ga is a benign , relatively common cutaneous disease that classically presents as closely set , skin - colored , firm , smooth papules 2 - 3 mm in size arranged in a ring - like fashion to form annular lesions . most cases occur before the age of 30 years and it is approximately twice more common in females . it often favors sacral sites such as the dorsa of the hands , elbows and feet but involvement of the palms appears to be rare . occasional familial cases are described with the occurrence in twins , siblings , and members of successive generations . attempts to identify an associated hla sub - type have yielded disparate results in different population groups . linear ga , a follicular pustular form and popular umbilicated lesions in children have also been described . the condition manifests as numerous ( at least 10 , often hundreds to thousands ) small , asymptomatic , erythematous , violaceous , brown- or skin - colored papules . rarely , ga may be complicated by nerve involvement as a result of granulomatous inflammation surrounding cutaneous nerves and perineural infiltrates of histiocytes in the dermis . the differential diagnoses of localized ga include tineacorporis , pityriasis rosea , pityriasis rotunda , erythema annulare centrifugum , nummular eczema , discoid lupus erythematosus , psoriasis , necrobiosis lipoidica , morphea , hypertrophic lichen planus and erythema chronicum migrans . treatment options include topical or intralesional corticosteroids , imiquimod cream , topical calcineurin inhibitors ( tacrolimus , pimecrolimus ) , cryotherapy , and pulsed dye laser .

granuloma annulare is a benign , self - limiting , inflammatory and granulomatous disease of unknown etiology occurring in both adults and children . an 18-month - old male child had multiple progressive annular plaques over the lower extremities . clinical and histopathological features were consistent with granuloma annulare . localized granuloma annulare is the most common form in children . we report a young child with multiple , progressive granuloma annulare over the lower extremities .

a 39-year - old man visited our clinic with a 2-week history of whitening of the eyelashes of his left lower lid . the patient reported that this started with 1 to 2 lashes on the central part of his eyelid and that adjacent eyelashes subsequently became involved over the next few days . otherwise the patient was in excellent health with no significant medical history ; he had no known drug allergies and was on no chronic medications . there was no associated family history of vitiligo , poliosis , or autoimmune disorders such as vkh or sarcoidosis . on examination , a pigmented skin nevus , approximately 4 mm in diameter , was noted above a patch of white eyelashes on the central aspect of the left lower eyelid ( fig . 1 ) . according to the patient , the nevus had existed and remains unchanged since childhood . no other abnormalities of the eyelid were noted and there was no vitiligo or signs of periocular inflammation . his visual acuity was 20/20 in both eyes and there were no significant findings in the anterior segment or fundus . serological tests for autoimmunity ( antinuclear , antimitochondrial , and antismooth muscle antibodies ) were negative and human leukocyte antigen ( hla ) typing failed to demonstrate types hla - dr4 or dw53 excisional biopsy of the central eyelid , including the pigmented nevus and white eyelashes , was performed . on histopathologic examination , the nevus cells appeared unremarkable and showed no evidence of malignancy . marked deficiency of melanocytes in the hair follicles was visible as compared with the normal overlying epidermis ( fig . 2 ) . infiltration of inflammatory cells , including lymphocytes and macrophages , was observed in the dermis ( fig . 3 , the majorities of halo nevi are acquired compound melanocytic nevi and appear in childhood and early adolescence . the site of predilection is the trunk and the back , especially when there are multiple lesions . patients with halo melanocytic nevi usually seek medical attention when their pigmented lesions develop a rim of depigmentation . over time , i , the central nevus remains brown in color or its pigment can disappear leading to a pink - colored papule ( stage ii ) . then the central papule disappears , leading to a circular area of depigmentation ( stage iii ) . finally , the depigmented area may repigment ( stage iv ) , leaving no trace of its existence . the underlying pathophysiology of the halo phenomenon is not well understood , but may possibly be the result of an immune response leading to nevus cell destruction . some authors suggest an autoimmune response directed against antigenic changes of dysplastic nevi while others regard it as an autoimmune phenomenon against melanocytes , as occurs in vitiligo . however , cellular and antibody - mediated immune mechanisms seem to be heavily involved in both theories and it is now well accepted that the inflammatory response is predominantly lymphocytic in nature , often with the addition of a small number of melanophages . there are four histologic forms of halo nevi described : ( a ) inflammatory halo nevus , ( b ) noninflammatory nevus , ( c ) halo nevus without halo or halo nevus phenomenon , and ( d ) halo dermatitis around a melanocytic nevus ( mayerson 's nevus ) . a halo nevus without the halo phenomenon is very rare and can only be diagnosed by histology . . described spontaneous regression of congenital nevi without the development of the halo phenomenon . sotiriadis et al . reported one case of a halo nevus without the halo phenomenon which developed on the trunk of a 5-year - old girl . they reported pathologic findings of a thin epidermis with marked orthokeratotic hyperkeratosis and dense lymphocytic infiltration intermingled with melanocytes ; these findings were consistent with the diagnosis of a halo nevus . a t - cell - mediated autoimmune response leading to melanocyte destruction with resultant poliosis and associated halo nevi in vkh disease however , our case exhibited no evidence of autoimmune disease and no halo phenomenon or vitiligo . in our case only histopathologic findings , such as infiltration of lymphocytes and melanophages , suggested the regression phase of the melanocytic halo nevus . total loss of melanocytes in eyelash follicles may be an isolated sign of the halo phenomenon without depigmentation of the skin . despite an in - depth review of the literature . the only reports of halo nevi of the eyelids were accompanied by vitiligo , not poliosis . also , a case of poliosis with a conjunctival melanoma has been described . one possible , though very unlikely , explanation for the presence of poliosis without skin depigmentation occurring in this case may be a predilection of melanin - rich hair follicles for immunologic reactivity . it is not possible for us to state with certainty why this may have occurred in the setting of a halo nevus undergoing regression , although it is very likely that the inflammation resulted from an exaggerated cell - mediated immune response . in conclusion , we herein present a case of a halo nevus with poliosis of the eyelashes as the only sign of the halo phenomenon . further analysis is needed to elucidate the mechanism of spontaneous regression of a halo nevus without depigmentation .

a 39-year - old man with poliosis of his lower eyelid lashes visited our clinic . he reported that his symptoms began with a few central lashes and then spread along the adjacent lashes during the ensuing 2 weeks . a pigmented nevus , approximately 4 mm in diameter , was identified just above the white lashes without surrounding skin depigmentation . no specific findings were identified with regard to the patient 's general health or serologic and radiologic testing . excisional biopsy of the pigmented nevus was performed . on histopathologic examination , infiltration of the dermis by numerous lymphocytes and melanophages was observed . the poliosis was ultimately diagnosed as a presenting sign of the halo phenomenon in the regressive stage of a melanocytic nevus .

acute cholecystitis is an inflammation of the gallbladder due to obstruction of the neck or cystic duct , primarily caused by gallstones . acute cholecystitis is characterized as a combination of local and systemic inflammation , and its treatment is dependent on the severity , although it is often treated conservatively with antibiotics . when acute cholecystitis becomes severe , it is treated surgically , while for some patients , percutaneous transhepatic gallbladder drainage ( ptgbd ) is an alternative to surgery . an accurate assessment of the severity is critical for the appropriate management of acute cholecystitis . abdominal ultrasonography ( us ) is useful for the diagnosis of diseases of the abdominal cavity . with abdominal us , however , this condition can be treated more appropriately if the severity is evaluated , using diagnostic imaging prior to the occurrence of organ failure or worsening of blood test variables . with regard to diagnostic imaging , wall thickening is representative of edema and correlates with the severity of acute cholecystitis , while inflammation is indicated by both edema and increased blood flow . if blood flow is assessed using abdominal us , it is possible to more accurately evaluate the severity of acute cholecystitis . superb microvascular imaging ( smi ) is based on color doppler imaging of abdominal us and enables the visualization of very slow blood flow without requiring contrast medium . it is composed of color - coded ( csmi ) and monochrome smi ( msmi ) , where csmi visualizes pulsatory signals such as color doppler images and msmi enhances the blood flow signal by suppressing background signaling . , we present the changes observed using csmi and msmi over the course of an acute cholecystitis in 1 patient . an 84-year - old man was admitted to the national hospital organization shimoshizu hospital with right upper abdominal pain that persisted for 3 days . white blood cell count ( wbc ) and c - reactive protein ( crp ) levels were 11.4 10/l and 15.3 mg / dl , respectively . total bilirubin , alkaline phosphatase , aspartate aminotransferase , alanine aminotransferase , and -glutamyl transpeptidase levels were 1.5 mg / dl , 206 iu / l , 20 iu / l , 10 iu / l , and 10 the possibility of obstructive jaundice was low . to clarify the cause of inflammation , computed tomography ( ct ) scans ( somatom emotion 16 ; siemens , munich , germany ) were performed , which showed slight thickening of the gallbladder wall ( fig . 1a ) . to confirm the results of the ct scans , the patient was subjected to abdominal us ( applio 400 ; toshiba medical , ohtawara , japan ) with a curved array transducer ( pvt-375bt ) at 3.75 mhz ( toshiba medical ) . the severity was classified as mild as the patient showed no signs of organ failure . diagnostic imaging found no evidence of marked local inflammation such as pericholecystic or hepatic abscess . the patient was treated conservatively with antibiotics ( cefazolin sodium 3 g / day ) intravenously from the date of admission . after 2 days of treatment , right upper abdominal pain still persisted , and his body temperature rose to 38.7c . wbc and crp levels rose to 15.6 10/l and 19.6 mg / dl , respectively . these data indicated that the acute cholecystitis had worsened . to evaluate the acute cholecystitis morphologically , abdominal us showed that the wall thickness of the gallbladder had increased and a sonolucent area was visible ( fig . complications , such as liver abscess or peritonitis , were considered possible . to avoid complications and promote recovery , seven days after ptgbd , wbc and crp levels were 6.8 10/l and 1.2 mg / dl , respectively , also confirming recovery . , csmi showed a pulsatory signal on the wall of the gallbladder with the curved array transducer ( 2.0 cm / s ; pvt-375bt ) at 3.75 mhz ( fig . 2a ) , whereas no such signal was observed on the wall with msmi ( 2.2 cm / s ) ( fig . when his acute cholecystitis had worsened , this signal became more evident with csmi ( fig . after ptgbd , the gallbladder shrank due to drainage of the gall and the pulsatory signal was no longer visible with either csmi ( fig . abdominal us is recommended as a first - line diagnostic imaging tool for the diagnosis of acute cholecystitis ; however , false negatives are a known issue of this technique . in our case , a pulsatory signal was observed on the wall of the gallbladder on the day of admission , which suggested positive csmi , even when wall thickening was minimal . smi is expected to reduce the incidence of false negatives when diagnosing this condition . in the present case , the strength of the pulsatory signals correlated with the severity of the gallbladder inflammation , as illustrated using both csmi and msmi . the results suggest that the signal intensity of csmi and msmi is correlated with the severity of inflammation . our case shows that both csmi and msmi are useful for the diagnosis and evaluation of the severity of acute cholecystitis . contrast - enhanced abdominal us shows a strong enhancement that successfully differentiates acute cholecystitis from a healthy gallbladder and chronic cholecystitis . however , unlike contrast - enhanced abdominal us , smi does not require contrast medium and is therefore suitable for the management of acute cholecystitis . a primary limitation of the present study is that it is based on just 1 case of acute cholecystitis . in order to confirm the usefulness of smi for the evaluation of the severity of acute cholecystitis , more patients should be enrolled in larger studies and smi findings for acute and chronic cholecystitis compared . furthermore , signal intensity should be stratified against the severity of acute cholecystitis . in conclusion , a strong pulsatory signal correlated with the severity of acute cholecystitis with both csmi and msmi and may be suitable for the evaluation of the severity of this condition . this report was approved by the ethical committee of the national hospital organization of shimoshizu hospital . the study was considered an element of daily clinical practice as opposed to a clinical trial . written informed consent was obtained for the performance of contrast - enhanced ct and ptgbd . the patient 's records were anonymously and retrospectively analyzed , and written informed consent was obtained for their use in this report .

evaluation of the severity of acute cholecystitis is critical for the management of this condition . superb microvascular imaging ( smi ) enables the assessment of slow blood flow of small vessels without any contrast medium . an 84-year - old man visited our hospital with right upper abdominal pain . computed tomography and abdominal ultrasonography showed a slight thickening of the gallbladder . white blood cell count and c - reactive protein levels were elevated . he was diagnosed with acute cholecystitis and treated conservatively with antibiotics . two days later , his condition worsened and percutaneous transhepatic gallbladder drainage ( ptgbd ) was performed . the patient recovered and was discharged , and his drainage was withdrawn 7 days later . on admission , color - coded smi ( csmi ) showed pulsatory signals on the slightly thickened gallbladder wall . on the day of ptgbd , the intensity of the signal on csmi had increased . once the patient was cured , no further signal was observed on the gallbladder wall with either csmi or msmi . in conclusion , the strong pulsatory signal correlated with the severity of acute cholecystitis observed with csmi and msmi . illustrating the signal intensity is useful for the evaluation of the severity of acute cholecystitis .

the most common adverse effects are indigestion , bleeding disorders , rash , diarrhea , and , rarely , taste disorders . here , we report a case of clopidogrel - induced ageusia notified in sfax pharmacovigilance center on march 13 , 2009 . a 46-year - old man was admitted in the department of cardiology in sfax tunisia in january 2009 for coronary artery disease and underwent coronary artery stenting . clopidogrel ( 150 mg / day ) was initiated on january 8 , 2009 , with lysine acetylsalicylate ( one bag / day ) and molsidomine ( 60 mg / day ) . however , the sense of smell was not affected . the ear nose throat ( ent ) examination ruled out abnormality ( dryness , atrophy , or infection ) of buccal mucosa and cranial nerve lesion . it was considered that simvastatin could be responsible for this adverse effect and it was then stopped it is indicated for secondary prevention in patients with atherosclerosis , coronary artery disease , and cerebral event such as transient ischemic attack . the caprie study demonstrated that clopidogrel has a favorable efficacy / safety ratio and provides an additional 8.7% relative - risk reduction for thrombotic events over lysine acetylsalicylate and is safer than ticlopidine . in addition , several other clinical studies have shown that clopidogrel reduces the overall rate of thromboembolic events in patients with recent myocardial infarction , stroke , or peripheral arterial disease compared with aspirin . side effects of new medicines are often not revealed during the initial clinical trials but are discovered when their use becomes a standard of care and , therefore , widespread . indeed , in the present case , an enquiry of pharmacovigilance was done according to french imputation method . besides psychogenic , systemic , oral , and neurological pathologies , medicines are the most common cause of taste disturbance . the fact that a large variety of medications affect the sense of taste is evidence of the complexity of the gustatory systems . the reception , transduction , propagation , and perception of a chemical tasting or odorant require the effective operation of numerous mechanisms . . thus , drug - induced adverse taste effects can reflect the taste of the drug itself , destroy mitosis in a replicating receptor cell , block the apical ion channels on a taste bud as a diuretic , and lead to candidal overgrowth on the tongue surface as immunosuppressants and steroids . these can also inhibit cytochrome p450-dependent enzymes at the level of receptors as an antifungal , disturb neuronal impulse propagation , cause changes in the neurotransmitter function , as well as alter higher order cortical processing of taste - related sensory information , production , and chemical composition of saliva and mucosal elements . the time of appearance of this side effect in the present case was similar to that reported in literature between two to eight weeks . this observation reminds the dose - related mechanism that remit spontaneously after drug discontinuation , albeit sometimes weeks or months after discontinuing a drug . the present case confirms the hypothesis and the suspected causal drug clopidogrel in the genesis of ageusia . although not a life - threatening side effect , loss of taste can produce significant changes in quality of life of a patient . it can lead to loss of appetite , weight , and may require discontinuation of drug administration in already compromised patients .

clopidogrel is prescribed in cardiac and extracardiac vascular diseases . it is generally well tolerated ; however , few cases of taste disorders have been reported . we present a case of clopidogrel - induced ageusia notified in sfax pharmacovigilance center on march 13 , 2009 . a 46-year - old patient developed ageusia with decreased appetite five weeks after starting clopidogrel . other etiologies including ear nose throat ( ent ) examination were ruled out . five months after reduction of clopidogrel dose , ageusia partially decreased . clopidogrel was strongly suspected as a causal drug . according to the french imputation method , score of imputability was considered as plausible ( c2s2 ) i2 . physiopathology of this side effect is not yet understood . however , it seems to be a reversible and dose - related event . although it is not life - threatening , loss of taste can have significant effect on the quality of life of patients .

historically , presence of heterotopic gastric mucosa in the intestinal tract has never aroused particular interest among clinicians . apart from its occurrence in the meckel s diverticulum , the condition is regarded as clinically insignificant . gastric metaplasia in the duodenum has been described by several authors , however , the significance of its occurrence remains unknown . extensive use of gastroduodenal fibroscopy enabled the authors to obtain biopsies of the duodenal mucosa . this paper will describe the incidence of gastric metaplasia in duodenum in various gastroduodenal diseases and discuss the pathogenetic role of gastric metaplasia in peptic ulcer . the authors obtained two or three pieces of random biopsy specimens from apparently normal mucosa in the anterior wall of duodenal bulb using gastroduodenal fiberscope on 65 subjects . all biopsies were immediately fixed in 10% formalin solution , embedded in paraffin , and 5 mm thick sections were stained with haematoxylin and eosine and pas , as described by mark and drysdale for sharp distinction of gastric metaplasia . the authors attempted to locate the fundic gland in all specimens but failed . consequently , the possibility of gastric heterotopia was excluded . the surface epithelium of gastric mucosa stained strongly with pas reaction contrasting sharply against the duodenal epithelium , where only goblet cells and the brush border disclosed a positive reaction ( fig . duodenal ulcer and prepyloric ulcers are grouped together since they have the same pathogenetic mechanism as proposed by johnson . the gastric ulcer group consisted solely of patients with ulcers located at the body , angle , or proximal antrum : two of the subjects in this group were additionally diagnosed with duodenitis . the incidence of gastric metaplasia according to its underlying diseases gastric metaplasia was not observed in normal subjects with exception to one ( 5.2% ) . in patients with duodenal and/or prepyloric ulcer ( s ) , its incidence was 72.2% , which was higher than in normal controls ( 5.2% ) and those with gastric ulcer ( 36.4% ) , nevertheless the difference lacked statistical significance ( p<.25 ) . three of 7 patients with duodenitis ( 42.8% ) , and one of 8 patients with gastric erosions ( 12.5% ) , had gastric metaplasia . the incidence of gastric metaplasia in the patients with gastic ulcer seemed to be varied according to the location of ulcer . although the number of cases was too low to evaluate it more thoroughly , the incidence of metaplasia was higher ( 66.7% ) in the patients with ulcers located in lower portion of the stomach ( prepyloric and pyloric ) than ulcers located in lower portion of the stomach ( prepyloric and pyloric ) than ulcers located in upper portion of the stomach ( 57.1% in those with ulcers located in angle and proximal antrum : 0% in those with body ulcer ) . the gastric mucosa in the duodenum can be classified into congenital heterotopic gastric mucosa and acquired gastric metaplasia as shown in table 3 . until now , many authors have made the mistake of combining metaplasia and true heterotopic gastric mucosa . since taylor reported earlier ( in 1927 ) two cases of gastric heterotopia in the duodenum , many authors described heterotopic gastric mucosa in duodenum as slightly raised patches consisting of chief cells and parietal cells . as true gastric heterotopia is frequently seen in meckel s diverticulum , and occasionally in rectum and small intestine , it is usually regarded as congenital in origin . the gastric metaplasia , a term which was first suggested by lessels , is an incomplete form of gastric heterotopia , and is composed of foveolar and pyloric gland : it can be identified only with microscopy . the incidence of true gastric heterotopia is suggested to be present in approximately 2% of the population . however , microscopic gastric metaplasia is far more frequently seen than true heterotopia and it is especially common in duodenal ulcer patients . the incidence of gastric metaplasia in duodenal ulcer patients was reported to be 51.9% by johansen , 74.3% by urakami and 72.2% by these authors series , which was quite similar to previous reports . it is unknown whether the gastric metaplasia has a protective role or a harmful effect in the formation of duodenal ulcer . the presence of gastric epithelium in the duodenum could indicate a simple protective response to excessive acid secretion . urakami reported that the gastric metaplasia is less frequent in the active stage of duodenal ulcer , and its incidence increases in the ulcer s healing stage . jochi found that gastric metaplasia was frequently seen in the margin of the ulcer and appeared to occur with the healing process of the ulcer . suzuki divided the gastric metaplasia into three types : foveolar cell metaplasia , parietal cell metaplasia , fully developed fundic gland metaplasia : he reported that each incidence had been 79.2% , 10.4% . and this study was intended only to reveal the incidence of gastric metaplasia in various gastroduodenal disease patients . the study could be expanded to clarify possible relations between acid secretion , stage of peptic ulcer , and stomach cancer .

to investigate the incidence of duodenal gastric metaplasia and its underlying gastric or duodenal diseases , the authors obtained endoscopic biopsy specimens from the duodenal bulb at random sites during endoscopy from 19 normal subjects , 11 patients with gastric ulcer , 18 with duodenal and/or prepyloric ulcer ( s ) , 7 with duodenitis and 8 with gastric erosions . the biopsy specimens were assessed with pas staining to confirm gastric metaplasia.the incidence of duodenal gastric metaplasia was 72.2% in patients with duodenal and/or prepyloric ulcer ( s ) , which contrasted with the patients with gastric ulcers ( 36.4% ) , duodenitis ( 42.9% ) , gastric erosions ( 12.5% ) , and normal subjects ( 5.3%).in conclusion , the results suggest that gastric metaplasia seen predominantly in patients with duodenal ulcer , seems to be related to hyperacidity and it plays some role in the pathogenesis of peptic ulcer in duodenum .

paget 's disease ( pd ) of bone is a focal , progressive disorder of accelerated bone remodeling and can lead to bone pain and several complications , including deformities , fractures , and secondary osteoarthritis . although the pathophysiology of pd is very complicated and not well known , the primary lesion is the abnormally increased and activated osteoclast in pd . bisphosphonates , which is the most potent anti - resorptive drug , have been the most effective agents available for the treatment of pd . among the bisphosphonates for osteoporosis treatment , zoledronate is a nitrogen - bound intravenous bisphosphonate with highest potency and longest administration interval . we reported a patient with pd , who had inadequate responses to therapy with pamidronate , has had a clinical remission with intravenous zoledronate . in may , 2010 , a 49-year - old woman presented with 4 year history of both buttock pain . iu / l ( reference range , 30 to 115 iu / l ) . a bone scan , which undergone at outside hospital 4 year ago , confirmed pagetic changes in the left ilium ( fig . 1 ) . and , pelvis anterior - posterior radiograph obtained at our hospital revealed a typical mosaic pattern of pd in her pubic and left ilium ( fig . her weight was 61.4 kg , height was 162 cm , and body mass index was 23.4 kg / m . range of motion at left hip was decreased due to pain , and she walked with a mild limp . although the level of alp was within reference range , pamidronate treatment was started orally for 3 months , because she had a severe pain . however , her pain intensity level increased . iu / l , and bone scan show still hot uptake in her both ilium and lumbar vertebrae ( fig . subsequently , she received intravenous zoledronate ( 5 mg ) in a single dose at august , 2010 . after this treatment , we evaluated clinical and biochemical remission and improvement in bone scan . three months after administration zoledronate , alp decreased 26 iu / l , and a severe pain and limping gait disappeared . six months after intravenous zoledronate , alp decreased 21 u / l , and bone scan showed the improvement , which means a response of zoledronate ( fig . iu / l , and bone scan showed hot uptake in both ilium and lumbar vertebrae ( fig . we report a patient with pd of bone , resistant to pamidronate treatment in whom intravenous zoledronate produced clinical remission and an improvement in the bone scan during 3 years . in united states population over 55 years , the incidence of pd is estimated to approximately 2 - 5% . however , the incidence is very low , and had not been well - known in korea . serum alp is an indicator of osteoblast activity and the primary marker for assessment of pd . however , this patient had low alp at initial presentation , which can not allow physician to suspect pd by a screening laboratory profile . bone scan can be used as a complementary method to diagnose and monitor the activity of pd . recent treatment guidelines recommend an use of bisphosphonate for pd to prevent disease progression and associated - complications , even in asymptomatic patients , especially with critical anatomic location such as skull , spine , weight - bearing bones , or the acetabular regions . oral bisphosphonate has been traditionally used for this , but compliance of oral bisphosphonate is too low , because of complex ingesting methods and gastro - esophageal irritation . therefore , annually intravenous zoledronate could be alternative optional . a clinical trial reported that a single 5 mg zoledronic acid infusion normalized alp levels in 89% of patients , compared with 58% in the control group administered oral risedronate , 30 mg / day for 60 for 2 months . although advantages of zoledronate include the convenience of a 15 minute infusion , approximately 40% of patients experienced zoledronate - associated symptoms ( flu - like illness , pyrexia , myalgia , arthralgia , bone pain ) , typically within the first 3 days after the infusion , with most symptoms resolving within 3 days after onset . this case study demonstrated that a patient with pd who had been treated unsuccessfully with oral bisphosphonates was effectively treated with a 5 mg infusion of zoledronate . the ability of zoledronate to provide a sustained remission offers many advantages for patients with pd and may lead to considerable benefits for the long term in preventing or minimizing complications .

paget 's disease ( pd ) of bone is characterized by increase of bone resorption by atypical osteoclasts , followed by rapid new bone formation resulting in a disorganized mosaic bone . although the pathophysiology is not fully understood , bisphosphonate , which is a potent anti - resorptive agent for treatment of osteoporosis , have been the most effective agents available for the treatment of pd . we report a case of pd of bone in a 49-year - old woman patient , who was treated with intravenous zoledronate .

coronary anomalies are reported in 1 to 5% of coronary angiographic examinations ; they are usually asymptomatic , and are discovered accidentally.1 an anomalous origin of the left anterior descending artery ( lad ) from both right and left sinus of valsalva is considered one of the rarest anomalies.27 the following report describes a patient presenting with an acute coronary syndrome , whose coronary angiography revealed an unusual course of the lad from the right and left coronary arteries . as he stated , he had been suffering from pain without radiation for the past 30 minutes , accompanied by nausea , vomiting , and diaphoresis . he claimed that he had had a previous similar episode the day before his admission , but the pain had resolved spontaneously after about one hour . he had a history of cigarette smoking , hypertension , and respiratory disease , for which he had received no appropriate and regular medical care . no abnormal findings were detected on his physical examination ( blood pressure = 140/80 mmhg , heart rate = 60 bpm , and normal breathing and heart sounds ) . his initial electrocardiogram ( ecg ) showed a left - axis deviation and left anterior hemiblock with ischemic changes ( inverted t wave ) on its pericardial leads . he had elevated cardiac troponin , and no other significant abnormalities were detected in his blood tests . he underwent medical management for acute coronary syndrome , and his pain was reduced within his first hour of admission . echocardiography was performed for the patient and revealed a moderate left ventricular ( lv ) dysfunction with an lv ejection fraction of 40% and anterior and lateral wall hypokinesia . the patient was scheduled for coronary angiography on the following day . no chest pain or other symptoms the next morning , the ecg showed the evidence of a posterior ( lateral ) myocardial infarction ( mi ) . the selective angiography of the left coronary artery showed a critical and thrombotic stenosis on the midportion of the lad as well as a moderate lesion on the midportion of the left circumflex ( lcx ) artery ( figure 1 ) . mild stenoses were also detected at the proximal end of the lad and high obtuse marginatus . the lad ran through the interventricular groove but at the end of the midportion , it deviated from its usual course and transformed to a diagonal and supplied the anterolateral area of the lv . on the right side , the selective coronary artery injection revealed a dominant right coronary artery ( rca ) and also an anomalous coronary artery arising just after the origin the rca ( figure 2 ) . the accessory vessel ran into the left side and coursed into the mid and distal portions of the anterior interventricular groove and supplied the apex area . the culprit lesions on the lad were stented with a drug - eluting stent ( des ) , and the patient was discharged from the hospital with an acceptable condition on the fourth day of his hospitalization . different types of congenital coronary anomalies have been reported , most of them having been discovered incidentally through routine coronary angiography . a double lad is among the rarest anomalies and its incidence ranges from 0.01 to 0.03% in different studies.5 in their classification , spindola - franko described four types of double lad8 : type i iii arises from the left side , and the last type ( apparently the rarest ) emerges from the right coronary sinus . here , we present a patient whose coronary anatomy consisted of a double lad type iv . he was admitted to our department with acute coronary syndrome , and his ecg showed evidence of anterior and posterior ( lateral ) mi . on selective coronary angiography , the lad had a thrombotic lesion on its mid course and it turned toward the anterolateral region , which is usually supposed to be supplied by a diagonal . probably the lesion at the midportion of the lad temporarily blocked the blood flow in the posterolateral area and caused the mi . on the right side , the rca was dominant , and an anomalous branch arose from the origin of the rca and coursed to the left side through the distal interventricular groove . the septal arteries branching from the anomalous vessel and also its left - sided course proved its identity as the lad . as was mentioned before , the last type of a double lad has a very low incidence . more often than not , the anomaly is suspected by visualization of an avascular area in the distribution of the left coronary artery and the absence of collaterals.3 in the present report , the lad changed its usual destination and ran into the posterolateral area ; and the apex , which is usually supplied by the distal portion of the lad , was supplied by the anomalous branch : the second lad . the anatomical and physiological understandings of coronary artery anomalies have both clinical and therapeutical applications . the relationship between an anomalous coronary artery , aorta , and pulmonary trunk and the possibility of the compression of the aberrant vessel by the two great arteries could be a source of different clinical presentations ( i.e. , angina , syncope , mi , and sudden cardiac death ) and sometimes mandate surgical correction . apart from the interarterial course , recent studies have shown that an intramural course could be identified in some anomalous coronary artery emerging from the opposite valsalva sinus.9 the clinical impact of such entity remains unclear . fortunately , the course of the anomalous branch usually could be detected by conventional angiography . the operator should always pay attention to the avascular region and search for a possible anomalous circulation . reported , sometimes the aberrant vessel can not be found by coronary artery angiography and multi - detector - row computed tomography may be helpful.10 beside the clinical consequences of anomalous vessels , knowing the anatomical variation is crucial at the time of surgery . grave surgical complications may occur by cutting an aberrant vessel.3 in conclusion , we presented a rare anatomical variation of the lad , known as a double lad type iv . the lad arose aberrantly from the right sinus of valsalva and supplied the area abandoned by the main lad . operators should always be aware of an anomalous circulation and seek to define its anatomical and clinical roles .

a double left anterior descending ( lad ) coronary artery emerging from the left and right coronary arteries is classified among rare coronary anomalies . we herein report a 73-year - old man presenting with acute coronary syndrome ( posterolateral myocardial infarction ) . he was admitted with typical chest pain , and due to his progressive ischemic changes on electrocardiography ( ecg ) and elevated cardiac enzyme , he was candidated for cardiac catheterization . the coronary angiography revealed an anomalous lad from the right sinus of valsalva . the unusual coronary anatomy was perfectly matched with the distribution of ischemia and its clinical evidence on echocardiography and ecg . the culprit lesion was stented , and the patient was discharged in good physical condition from the hospital .

we report a case of a patient who presented with a complex open 3c gustilo anderson fracture who subsequently developed flap failure , failed internal fixation complicated with osteomyelitis of the talus . they vary in severity from minor metatarsal / phalangeal fractures to complicated midfoot and hindfoot injuries . open fractures are poorly reported since they are rare and usually treated in specialist trauma centres . reported amputation rates in open midfoot fractures are 30% highlighting their severity , coexisting soft tissue compromise , infection and difficulty covering midfoot injuries with flaps . with the midfoot playing a key role in foot function , soft tissue defects of the foot pose further challenges given the specially adapted architecture to facilitate tendon gliding . a 55-year - old woman presented with an open 3c gustilo anderson fracture . her medical history included rheumatoid arthritis , osteoarthritis , ischaemic heart disease , diabetes mellitus and asthma . soft tissues were closed using a free flap from the left thigh and a flap from the right lower limb . bony injury was treated with open reduction internal fixation with screw fixation of the first and third metatarsals into the talus with restoration of the medial and middle columns achieved using cannulated screws . additional stability was achieved by plate fixation of the medial and lateral columns ( fig . ( c and d ) radiographs illustrating open reduction internal fixation ( orif ) ( screw fixation of the first and third metatarsals into the talus ) . the orif using cannulated screws allowed restoration of the medial and middle columns of the foot . ( c and d ) radiographs illustrating open reduction internal fixation ( orif ) ( screw fixation of the first and third metatarsals into the talus ) . the orif using cannulated screws allowed restoration of the medial and middle columns of the foot . four months since the initial presentation , she was readmitted due to foot dorsal flap edge failure for which vacuum - assisted closure was unsuccessful ( fig . figure 2:flap failure on dorsal aspect of foot when patient presented after failed vacuum - assisted closure treatment . flap failure on dorsal aspect of foot when patient presented after failed vacuum - assisted closure treatment . primary limb shortening with a talectomy was conducted to reduce the soft tissue deficit and remove infected and necrotic bone , tibiocalcaneal arthrodesis for joint stability using external fixation , and dead space was treated with calcium sulphate mixed with vancomycin and systemic intravenous and oral antibiotics for osteomyelitis postoperatively ( figs 3 and 4 ) . the wound was closed after the external fixator was applied . wound swabs and bone samples revealed enterococcus faecalis and e. raffinosus and diphtheroids . the patient was treated with intravenous teicoplanin and meropenem with oral stepdown to linezolid and ciprofloxacin . ( b d ) radiographs showing external fixation in situ . figure 4:external fixation used to facilitate tibiocalcaneal arthrodesis post talectomy . eight months since the initial presentation , the patient was taken to the theatre where a large defect in the anterior dorsum of the foot , and the external fixator was adjusted in the foot to allow for exploration and debridement . the abductor hallucis was identified and the tendon divided distally and mobilized and rotated to fill in the soft tissue medial defect . calcium sulphate with vancomycin and gentamycin was inserted into the bony defect , and external fixation frame was adjusted back to its original position . the wound was closed using the abductor hallucis and skin graft from the right lateral leg . postoperatively the patient was put on intravenous teicoplanin and meropenem with oral stepdown to linezolid and ciprofloxacin . on discharge once the infection was controlled the patient s crp was 6.0 , white cell count 7.9 and neutrophils 4.0 . after having the external fixation for 8 months in total , it was removed when fusion was deemed adequate on computed tomography imaging ( fig . she was instructed to weight bear while in a plaster of paris cast for 14 days and then a moon boot for 14 days . two months after this , the patient was mobilizing without issues and was doing well . at 12 months , the patient stated that she is able to mobilize with any mild residual pain controlled with analgesia . ( a ) anterior posterior view ; ( b ) lateral view . computed tomography scans illustrating tibiocalcaneal fusion 1 year postoperatively . midfoot injuries with talar infections can often lead to below - knee amputation with the main goals of treatment being to successfully treat the infection and maintain a functional foot . in adults , most work focuses predominantly on internal fixation techniques or a combination of internal and external fixation . most reports of arthrodesis following talectomy are for patients with charcot deformities rather than traumatic injuries with osteomyelitis . to successfully treat patients with infected midfoot injuries , calcium sulphate with antibiotics was used intraoperatively in the fusion site with some evidence supporting its use in deep foot infections . following this , a long course of intravenous antibiotics was used according to the sensitivities of the microorganisms growing in the region . external fixation facilitates soft tissue healing , allows earlier weight bearing , closer monitoring of surgical incision sites and postoperative adjustments . fabian and colleagues concluded that tibiocalcaneal fusion in seven charcot joint patients using external fixation alone was unlikely to lead to fusion but the fibrous healing was sufficient . agarwala s group and dennison s group report full fusion in all of their patients undergoing arthrodesis using external fixation [ 8 , 9 ] . our results are in concordance with work of agarwala and dennison et al . in that external fixation there is a need for randomized controlled trials to investigate the most appropriate fixation technique for tibiocalcaneal arthrodesis . in conclusion , we report a good outcome treating an open 3c gustilo anderson fracture complicated by osteomyelitis , necrosis and inadequate soft tissue coverage . our management involved a single - staged talectomy to remove the infected bone and tackle the inadequate soft tissue coverage , external fixation for tibiocalcaneal arthrodesis to achieve joint stability and a combination of bone graft antibiotics and a course of intravenous and oral antibiotics postoperatively to deal with the underlying osteomyelitis .

foot fractures vary in severity with complex midfoot fractures having poor morbidity rates and high amputation rates . complex midfoot fractures are rarely reported since they are uncommon and only treated in specialist centres . given the important role of the midfoot in foot function , reconstruction is preferable . soft tissue management on the dorsal aspect of the foot poses further challenges to reconstructive surgeons . we report a case of a 55-year - old woman who sustained an open 3c gustilo anderson fracture that was initially treated with open reduction internal fixation and free flap . she subsequently developed flap and internal fixation failure with osteomyelitis of the talus . we report a good outcome using primary limb shortening with a talectomy , tibiocalcaneal arthrodesis using external fixation and a combination of vancomycin - loaded calcium sulphate and intravenous antibiotics in our patient .

pelvic fractures and in particular crescent fracture dislocations , which are characterised by disruption of the sacroiliac joint with extension proximally as a fracture of the posterior iliac wing , usually occur as a result of high velocity trauma ( 1,2 ) . osteopenia and the associated loss of bone trabeculae decreases the elastic resistance of the osseous elements of the pelvis , thus making the pelvis a common location for insufficiency fractures ( 3 ) . as the general population ages , the number of osteopenic specific pelvic fracture patterns are likely to increase . in cases where surgical fixation is required , less invasive techniques are beneficial in this older population ( 3 ) . the unique fracture pattern described in this report occurred as a result of the patient falling in such a way as to result in simultaneous sudden flexion of one hip with extension of the contra - lateral hip , into a position known in athletic terms as the front splits . the combination of the torque force produced by the fall and the patients reduced bone mineral density resulted in a unique , unstable pelvic fracture . to our knowledge this is the first reported case of bilateral crescent fractures , and the first reported case of pelvic fracture as a result of this mechanism of injury . the authors have obtained the patient 's informed written consent for print and electronic publication of this case report . a 57 year old woman slipped while shopping resulting in a fall , where - upon her right hip flexed to 90 degrees and her left hip extended to 90 degrees , to a point where her perineum came to rest on the floor . this particular fall resulted in a twisting or torque like force being transmitted through her pelvis as she fell . she attributed the increased pain that she was experiencing to aggravation of her long standing back complaint . prior to her fall she had attended the spinal clinic for her back pain and was scheduled for a bone scan with a view to progressing to facet joint blocks . on attendance at the spinal clinic following her scan it was noted that her low back pain had become more pronounced and she required walking aides in the form of crutches to mobilise . on reviewing the bone scan , further imaging , both plain film and computed tomography ( ct ) ( fig.1 & fig . 2 ) revealed extensive pelvic fractures with displaced bilateral superior and inferior pubic rami fractures and bilateral posterior ring crescent fractures . she had no previous fractures and prior to her fall worked as a care assistant . coronal computerised tomography ( ct ) image demonstrating bilateral posterior iliac wing fractures and sacro - iliac joint disruption three - dimensional ct reconstruction showing both the bilateral crescent and superior and inferior pubic rami fractures following admission , routine blood parameters ( including bone profile ) were all normal and a pelvic mri scan out - ruled any intra - pelvic soft tissue lesions . open reduction and internal fixation of her anterior pelvic ring was undertaken through a stoppa approach , both rami fractures were reduced and plated anteriorly and superiorly . at this time bone and soft tissue samples from the anterior ring were sent for analysis . following reduction and fixation of her anterior pelvis , intra - operative fluoroscopy revealed that her posterior injuries were in an acceptable position . a 3 cm long vertical incision was made over both anterior inferior iliac spines and supra - acetabular screws were placed from the anterior inferior iliac spines to the posterior inferior iliac spines to fix the bilateral iliac wing fractures . inlet radiograph at 16 months post surgery demonstrating solid fixation and bony healing post - operative recovery was uneventful . subsequent dual energy x - ray absorptiometry scanning revealed mild osteopenia for which she is being managed medically . final follow - up at 16 months demonstrated that the fractures had healed , and the patient was pain free and mobilising unaided ( fig . these injuries are rotationally unstable , there may also be some vertical displacement but this is limited by the sacrotuberous and sacrospinous ligaments , which typically remain intact ( 1,2 ) . our patient sustained bilateral crescent fractures and complete bony disruption of her anterior pelvic ring through inadvertently and suddenly performing what is known in athletic terms as the front splits , where one hip is flexed and the other extended to 90 degrees in neutral adduction / abduction . although this does not fit with the classic description of high energy trauma , the force imparted to the untrained individual s pelvis in performing such an act would be considerable . another contributing factor is our patients post - operative diagnosis of osteopenia , the associated loss of bone trabeculae decreases the pelvic elastic resistance making it a common location for insufficiency fractures in post - menopausal women ( 4 ) . day et al ( 5 ) have classified crescent fractures according to the extent of sacroiliac joint involvement , with type i fractures entering the anterior third of the sacroiliac joint , type ii fractures involving the middle third and type iii fractures limited to the posterior third of the sacroiliac joint . operative stabilisation of these injuries is recommended and aims to achieve accurate reduction of the sacroiliac joint and stabilisation of the associated pelvic ring fracture , thus facilitating early mobilisation and minimising disability due to post - traumatic malunion and osteoarthritis or instability of the sacroiliac joint ( 2,3,6 - 9 ) . however both reduced function and ongoing pain are commonly reported even after technically satisfactory surgery ( 2,7 ) . pelvic insufficiency fractures are relatively common and should be suspected in older female patients with unexplained hip , groin , buttock , low back pain and/or difficulty with ambulation , particularly if there is any recent history of trauma ( 10 ) . while the majority of these insufficiency fractures may be treated conservatively our patient required surgical fixation due to the unstable nature of her injury . to our knowledge this is the first reported case of bilateral crescent fractures and also of a pelvic fracture occurring as a result of this unique mechanism of injury .

a case is presented of a healthy 57 year old female who slipped and fell awkwardly into what is known in athletic terms as the front splits . as a result of her fall she sustained bilateral crescent and superior and inferior rami pelvic fractures . successful operative fixation was undertaken by a combination of open and percutaneous techniques . to our knowledge this is the first reported case of bilateral crescent fractures , and of a pelvic fracture as a result of this mechanism of injury .

children constitute approximately 40% of india 's population but information on adverse drug reactions occurring in them is limited . phenytoin sodium ( pht ) is one of the commonest antiepileptic drug ( aed ) used in children in india . the drug has wide pharmacokinetic variability and has a narrow therapeutic range that leads to toxicity . there is some evidence of the association of long - term use of pht and toxicity like cerebellar atrophy . such cerebellar changes have been reported even with the long - term use of nontoxic levels of phenytoin . , we report a case of reversible cerebellar atrophy induced by pht in a 10-year - old boy . after initiating therapy with pht 10 years back , he was seizure free for 2 years . patient was also occasionally prescribed clobazam , 5 mg , oral , as and when required . the episodes used to last for a brief period of over 6 - 7 days . seizures used to occur at every 3 - 4 months interval . the boy had history of birth asphyxia and delay in developmental milestones . after the patient recovered , he was evaluated again due to complaint of difficulty in walking . cns examination revealed normal mentation with cerebellar signs including gaze - evoked nystagamus , truncal , and appendicular ataxia . mri scan of the brain was advised and it showed cerebellar atrophy as shown in figure 1 . serum phenytoin level was high ( 30 mcg / ml ) and pht was withdrawn immediately . patient was started on valproic acid and followed up . however , a sizeable proportion of patients may develop drug induced adverse effects that include sedation , gum hypertrophy , lymphadenopathy , chorea , ataxia , etc . there are a few reports on cerebellar atrophy after long - term use of pth . these adverse effects are usually reported if the drug serum levels are above the therapeutic range . our patient presented with severe cerebellar disorder and he was on the drug for 10 years . hence , a regular monitoring for adverse drug reaction should be considered in patients who are on drugs on long term basis . these reactions could be idiosyncratic or dose - dependent which again may be acute or chronic in nature . since most of these effects are reversible , it is important to identify the clinical manifestations related to drug toxicity and to manage them appropriately . also , it warns the need for regular monitoring of plasma concentration , accurate dosing , and identification of adherence issues in patients on phenytoin .

epilepsy is an important health problem due to its high prevalence and potential for causing long - term morbidity . it is commonly treated in children with phenytoin sodium . it has wide pharmacokinetic variability and a narrow therapeutic range that leads to toxicity . here , we report a case of phenytoin - induced cerebellar atrophy in a 16-year - old epileptic boy who presented to the hospital with a viral infection .

the natural history for these hernias is progressive enlargement if left untreated and many of these patients undergo elective repair with synthetic mesh for symptomatic or cosmetic reasons . also , the sequelae of potential complications , such as bowel incarceration or strangulation , result in greater morbidity and mortality in cirrhotic patients . this case report discusses one of such patients , who presented with eviscerated bowel through a long - standing umbilical hernia and describes the surgical management employed . a 50-year - old caucasian male with a long - standing history of cirrhosis , secondary to chronic alcohol abuse and hepatitis c infection , presented to the emergency department with evisceration of his small bowel through an existing umbilical hernia ( fig . 1 ) . the patient had a complicated course of liver disease marked by recurrent ascites and hepatic encephalopathy . two years prior , the patient had his umbilical hernia primarily repaired ; however , he developed a recurrence of the umbilical hernia secondary to recurrent and refractory ascites . the patient stated that he suddenly noticed protrusion of bowel through his umbilical skin after a sudden coughing bout several hours prior to presentation . the patient also reported copious drainage of ascitic fluid through the hernia site following the evisceration . following his surgery , the patient spent 7 days in the icu after which he was transferred to a medical surgical floor and subsequently discharged with normal bowel function and in stable condition . patients with ascites in the setting of cirrhosis have an 20% chance of developing an umbilical hernia . in the presence of persistently increased intra - abdominal pressure , points of weakness in the abdominal wall are potential sites of herniation . the linea alba is discontinuous at the umbilicus and thus is one point of weakness . furthermore , this patient had an albumin level of 1.8 g / dl suggesting chronic nutritional deficits that could potentially lead to weakened abdominal fascia . evisceration of small bowel through an existing umbilical hernia is a rare and potentially fatal complication of umbilical hernia in the presence of recurrent ascites . there is a reported case of omental evisceration through an umbilical hernia in a patient with a similar history of cirrhosis and hepatitis c . similar to our patient , this occurred following an acute increase in intra - abdominal pressure . evisceration of abdominal contents puts the patient at risk for incarceration , infection and necrosis . a 2011 study performed by erasmus university researchers found that surgical repair of an umbilical hernia in a patient with ascites due to cirrhosis is preferable to conservative treatment . the complications of the umbilical hernia appear to be a greater cause of mortality and morbidity than the surgical repair . in summary , small bowel evisceration is a rare but serious and potentially fatal complication of umbilical hernia in cirrhotic patients with refractory ascites . this case is the first - in - literature depicting spontaneous evisceration of bowel in a cirrhotic patient with a recurrent umbilical hernia .

umbilical hernia in the cirrhotic patient is frequently seen in the setting of refractory ascites . this article reports a rare case of spontaneous rupture of a recurrent umbilical hernia in a patient with persistent ascites , following an acute increase in intra - abdominal pressure , leading to bowel evisceration . this case highlights a potentially fatal complication of umbilical hernia in the setting of chronic ascites , which was successfully managed with prompt surgical intervention .

the most frequent sites of bleeding in patients with haemophilia are the soft tissues , the joints , the urinary tract , but much more rarely the gastrointestinal tract . the complications of intramural bleeding are acute intestinal obstruction , but also rupture of the haematoma in the lumen or the peritoneal space . we present the case of a haemophiliac patient who was admitted as an emergency due to distended abdomen , nausea , vomiting and the clinical picture of ileus . the native abdomen in a standing position presented air fluid levels with moderate distension of the accompanying bowel loops . a nasal probe was inserted and the symptoms of ileus disappeared , but after taking food by mouth , the picture of ileus returned . ct of the abdomen and pelvis was performed , which showed circular , high density thickening of the walls in places in the area of the jejunum , indicating haemorrhage , but also the formation of haematoma in the wall structure . after administering factor viii , the symptoms of ileus ceased , and the patient recovered completely . this unusual presentation of haemophilia with bleeding in the wall of the small intestine is very rare and has only been seen in a few cases in the world . ct diagnosis defined the cause of the obstruction and saved the patient from an unnecessary surgical procedure . haemophilia is a congenital blood ailment characterized by the lack of coagulating factor viii ( haemophilia a ) or factor iv ( haemophilia b ) . the most frequent sites of bleeding are the soft tissue , the joints , the urinary tract , and much more rarely the gastrointestinal tract ( 1 ) . gastrointestinal haemorrhage has an incidence of 17.525% in haemophiliacs and causes death in 4% of this population overall ( 2 ) . the complications of intramural bleeding are acute intestinal obstruction , but also the rupture of the haematoma in the lumen or in the peritoneal space ( 3 ) . this case presents a patient with haemophiliac ileus of the small intestine , where ct diagnostics defined the cause of the obstruction and saved the patient unnecessary surgery . the patient was admitted in the evening as an emergency case , due to a distended abdomen , nausea , vomiting and the clinical picture of ileus . the native abdomen in a standing position presented air fluid levels with moderate distension of the relevant bowel loops ( figure 1 ) . a nasal probe was inserted and the symptoms of ileus disappeared , but after taking food by mouth , the picture of ileus returned . laboratory test results : wbc 11/00 , rbc 6.35 , hct 0.503 , hgb 166 . a - ptt 84.8 ( rv:26 - 28 s ) , antihaemophilic factor a ( ahg - a ) , f - viii < 0.05 ( rv:0.7 - 1.5 j ) . ct of the abdomen and pelvis was performed , which showed circular high density thickening of the walls in places in the area of the jejunum , indicating haemorrhage , but also the formation of haematoma in the wall structure . the presence in places of air fluid levels suggested disturbed passage through the bowels ( figures 2 and 3 ) . in the area of the jejunum circular high density thickening of the walls in places , indicating haemorrhage , but also the formation of haematoma in the wall structure . after administering factor bleeding in the gastrointestinal tract in haemophiliacs is not rare , and is usually manifest either in melena or per rectum . bleeding less often occurs in the intestinal wall , with the symptoms of intestinal obstruction . in this case , intramural bleeding had occurred in the small intestine ( figure 2 ) and the intramural haematoma was the cause of paralytic ileus and intestinal obstruction . the most probable physiopathology of intramural haematomas of the bowel is characterized by the spread of the terminal arterial vessels as they leave the mesentery and penetrate the muscular layer of the intestinal wall ( 3 ) . an intramural haematoma may also cause intussusception , as another form of ileus ( 4 ) . as bleeding is the most frequent cause of acute abdominal pain in haemophiliacs , an acute abdomen should be deemed to be bleeding , until proven otherwise by diagnostic methods ( ct ) . it is a very useful method in diagnosing intramural haematoma , defining the area of the haemorrhage and revealing possible complications . most intramural haematomas can be treated conservatively and spontaneous resolution occurs , where bleeding is treated by prompt compensation of factor viii . correct diagnosis and a multi - discipline approach are imperative in order to avoid unnecessary explorative procedures .

introduction : the most frequent sites of bleeding in patients with haemophilia are the soft tissues , the joints , the urinary tract , but much more rarely the gastrointestinal tract . the complications of intramural bleeding are acute intestinal obstruction , but also rupture of the haematoma in the lumen or the peritoneal space.case report : we present the case of a haemophiliac patient who was admitted as an emergency due to distended abdomen , nausea , vomiting and the clinical picture of ileus . the native abdomen in a standing position presented air fluid levels with moderate distension of the accompanying bowel loops . a nasal probe was inserted and the symptoms of ileus disappeared , but after taking food by mouth , the picture of ileus returned . ct of the abdomen and pelvis was performed , which showed circular , high density thickening of the walls in places in the area of the jejunum , indicating haemorrhage , but also the formation of haematoma in the wall structure . after administering factor viii , the symptoms of ileus ceased , and the patient recovered completely.conclusion:this unusual presentation of haemophilia with bleeding in the wall of the small intestine is very rare and has only been seen in a few cases in the world . ct diagnosis defined the cause of the obstruction and saved the patient from an unnecessary surgical procedure .

quadricuspid aortic valve ( qav ) is a rare congenital anomaly with an incidence of 0.010.04% . it is far less frequent as compared to bicuspid ( 12% ) or unicuspid aortic valve anomaly . in recent decades , there has been increased awareness of qav because of better imaging techniques such as transthoracic , transesophageal echocardiography ( tee ) , and cardiac magnetic resonance imaging ( mri ) . this increased awareness has contributed to our knowledge of classification , clinical course , and management of this rare congenital condition . more than half of the patients with qav will require surgical intervention at some point in their life to treat the aortic insufficiency . a 39-year - old hispanic male was seen in his primary care physician 's office for follow - up of his hypertension . patient denied any exertional shortness of breath , chest pain , palpitations , orthopnea , or paroxysmal nocturnal dyspnea . cardiac auscultation revealed a diastolic murmur of grade 2/4 in the second right intercostal space . transthoracic echocardiogram was done for the evaluation of this new diastolic murmur , which showed normal left ventricular chamber size with an ejection fraction of 55% . the aortic valve was poorly visualized and showed evidence of mild to moderate aortic insufficiency with valve anatomy suspicious of qav . the aortic valve was found to be quadricuspid [ figure 1 and video 1 ] with aortic root measuring 2.40 cm with vena contracta measuring 4.55 mm . there was evidence of moderate aortic insufficiency [ figure 2 and video 2 ] . since the patient was asymptomatic , he was counseled regarding the condition and need for regular follow - up with the cardiologist . 39-year - old hispanic male was examined by his primary care physician for follow - up of his hypertension . transesophageal echocardiography image shows quadricuspid aortic valve in short axis view , cusp 1 , 2 , and 3 are equal in size whereas cusp 4 is the accessory cusp and is smaller in size . rvot : right ventricle outflow tract ; la : left atrium ; ra : right atrium . 39-year - old hispanic male was examined by his primary care physician for follow - up of his hypertension . qav is a rare congenital anomaly , which is usually diagnosed in the fifth or sixth decade of life when it causes aortic regurgitation . several different anatomical variations of qav have been described , hurwitz and roberts classified them into seven groups : type a - four equal cusps ; type b - three equal cusps and one smaller cusp ; type c - two equal larger cusps and two equal smaller cusps ; type d - one large , two intermediate , and one small cusp ; type e - three equal cusps and one larger cusp ; type f - two equal larger cusps and two unequal smaller cusps ; and type g - four unequal cusps . our patient had type b quadricuspid with three equal cusps and one smaller cusp as shown in figure 1 . this is the most common type and is often associated with regurgitation due to the unequal shear stress . one of them is abnormal septation of the embryologic truncus arteriosus . in general , after septation of the arterial trunk , three mesenchymal swellings develop into semilunar leaflets of the aortic and pulmonary trunks . in qav , however , the fourth cusp arises during the early stage of truncal septation , resulting in either a different number of primordial aortic leaflets or abnormal cusp proliferation . it usually appears as an isolated congenital anomaly but may also be associated with other malformations , the most common being coronary artery anomalies . development of the aortic valve leaflets occurs just after the development of the coronary artery origins from the sinuses of valsalva and it is possible that these two groups of anomaly may therefore be embryologically related . it is very important to diagnose these associated anomalies before repair or replacement of aortic valve to avoid ostial obstruction . aortic dilatation and other structural cardiac abnormalities were relatively common among patients with qav . in our patient , the physiopathology of the valve dysfunction is poorly understood : anatomical abnormalities of the cusps could induce unequal shear stress leading to fibrosis and incomplete coaptation . the characteristic echocardiographic finding in short - axis view is an x - shaped commissure pattern during diastole and a rectangular appearance during systole . however , transthoracic echocardiography may be suboptimal for recognizing qav and associated malformations due to the poor acoustic window . tee provides reliable imaging of heart valves and other structures even in complex congenital heart diseases . recently , cardiac mri has also been used for the diagnosis of qav and other associated congenital cardiac anomalies . with more liberal use of tee , computed tomography , and mri the importance of diagnosing this congenital anomaly in asymptomatic adults lies in the fact that more than 50% of these people will require valve repair / replacement in the fifth or sixth decade of life because of worsening aortic regurgitation or stenosis . hence , patients with qav should have regular follow - up and have aortic valve repair / replacement at an appropriate time before left ventricle decompensation to avoid morbidity , mortality , and complications . the importance of diagnosing qav lies in the fact that majority of these patients will require surgery for aortic insufficiency . once diagnosed patients should be closely followed so that aortic valve replacement / repair is done before the left ventricular decompensation occurs . 39-year - old hispanic male was examined by his primary care physician for follow - up of his hypertension . transesophageal echocardiography shows quadricuspid aortic valve in short axis view 39-year - old hispanic male was examined by his primary care physician for follow - up of his hypertension .

quadricuspid aortic valve ( qav ) is a rare congenital cardiac anomaly causing aortic regurgitation usually in the fifth to sixth decade of life . earlier , the diagnosis was mostly during postmortem or intraoperative , but now with the advent of better imaging techniques such as transthoracic echocardiography , transesophageal echocardiography ( tee ) , and cardiac magnetic resonance imaging , more cases are being diagnosed in asymptomatic patients . we present a case of a 39-year - old male who was found to have qav , with the help of tee , while undergoing evaluation for a diastolic murmur . the patient was found to have type b qav with moderate aortic regurgitation . we also present a brief review of classification , pathophysiology , and embryological basis of this rare congenital anomaly . the importance of diagnosing qav lies in the fact that majority of these patients will require surgery for aortic regurgitation and close follow - up so that aortic valve replacement / repair is done before the left ventricular decompensation occurs .

the online version of this article ( doi:10.1007/s13555 - 015 - 0084 - 3 ) contains supplementary material , which is available to authorized users . finasteride 1 mg is used successfully all over the world for the treatment of androgenetic alopecia in male pattern baldness . various clinical trials have established finasteride s efficacy in adult men with predominant vertex , anterior and midscalp region [ 2 , 3 ] . on average , common side effects are loss of libido , erectile dysfunction and decreased ejaculate volume . these side effects were found in less than 0.5% of patients . to assess the huge difference [ 2 , 11 ] in reporting the side effects , we conducted a 2-year randomized , questionnaire - based research study on patients receiving finasteride 1 mg tablets for androgenetic alopecia . for this , we used a questionnaire based on the international index of erectile function ( iief ) ( supplementary table 1 ) . this checks five domains : erectile function , orgasmic function , sexual desire , intercourse satisfaction and overall sexual satisfaction . a higher iief score indicates better sexual function and low score ( below 25 ) indicates erectile dysfunction . the aim of the present study was to evaluate an age - matched comparison and evaluation of sexual function in patients treated by finasteride 1 mg for early androgenetic alopecia by using iief . inclusion criteria were age 1840 years , who came for an outpatient consultation for male pattern androgenetic alopecia , which was diagnosed using the norwood hamilton s grading of male pattern alopecia with grade 3 to 5 on treatment with finasteride 1 mg , with good physical health . exclusion criteria were patients being treated for various other causes of hair loss other than androgenetic alopecia . those selected patients were briefed about the iief questionnaire and , with their consent , the questionnaire was provided for completion . a mean duration of approximately 16 weeks of medication was consumed by the patients at the time of answering the questionnaire . our controls were 586 patients who attended outpatient consultation for various other non - hair related diseases such as psoriasis vulgaris , lichen planus , allergic contact dermatitis , irritant contact dermatitis , nail disorders , etc . the resultant scores of erectile function domain and iief domain of both the study group taking finasteride 1 mg and their age - wise control group were analyzed with the ibm software package ( spss statistics , ny , usa ) . all procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation ( institutional and national ) and with the helsinki declaration of 1964 , as revised in 2013 . both the study group and controls were asked to answer the iief questionnaire anonymously to avoid any identity on sexual weakness . the results were compared in an age - wise manner . comparing the age - wise finasteride - consuming patients and the control group , there was no statistical difference between the groups regarding their sexual function in total iief score or in any of the five domains mentioned . 1 ) show the scores in the erectile function domain ( a ) and international index of erectile function domain ( b ) in patients on finasteride and their controls.fig . 1age controlled 3d graphs showing the scores of domain in erectile function ( a ) and international index of erectile function domain ( b ) in patients on finasteride and their controls with age and scores both depicted in y axis age controlled 3d graphs showing the scores of domain in erectile function ( a ) and international index of erectile function domain ( b ) in patients on finasteride and their controls with age and scores both depicted in y axis the main mechanism in androgenetic alopecia is miniaturization of hair follicles from terminal to vellus hair due to the effect of androgen , especially dihydrotestosterone . testosterone is converted by 5 alpha reductase to 5 alpha dihydrotestosterone , which is five times more potent than testosterone . this dihydrotestosterone binds to the androgen receptors of the genetically marked hair follicle and leads to miniaturization of that hair follicle . finasteride is an azasteroid type 2 isoenzyme , 5 alpha reductase inhibitor , which inhibits dihydrotestosterone conversion from testosterone . this type 2 isoenzyme is present in the hair follicles and its activity is important in controlling the end organ hyper - reactivity causing androgenetic alopecia . observation of eunuchs and prepubertal boys highlighted the role of testosterone in the induction of androgenetic alopecia . therefore , it is evident that finasteride should not cause any side effects , relating to sexual health , as there will be no decrease in the quantity or quality of the testosterone level . however , clinical trials conducted by leyden et al . and kaufman et al . resulted in 2% of patients experiencing sexual side effects such as the loss of libido , erectile dysfunction and decreased ejaculate volume . in this study , patients taking finasteride 1 mg for androgenetic alopecia and the control group were provided with the iief questionnaire and their responses analyzed . the results indicated that erectile and sexual function was not reduced compared to the controls . so , not only theoretically , finasteride was found to be a comparatively safe drug in this indian male population . this study was conducted in the indian context on the basis of the iief and the european study by tosti et al . . further studies should be conducted in a multi - center , double - blind manner . in both our study and the study conducted by tosti et al . , sexual and erectile function of those subjects who were orally treated with finasteride was not reduced compared with their age - related controls . supplementary material 1 ( pdf 301 kb ) supplementary material 2 ( docx 19 kb ) all procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation ( institutional and national ) and with the helsinki declaration of 1964 , as revised in 2013 . this article is distributed under the terms of the creative commons attribution - noncommercial 4.0 international license ( http://creativecommons.org/licenses/by-nc/4.0/ ) , which permits any noncommercial use , distribution , and reproduction in any medium , provided you give appropriate credit to the original author(s ) and the source , provide a link to the creative commons license , and indicate if changes were made .

introductionfinasteride is one of the most common drugs used in androgenetic alopecia . the literature discusses the sexual side effects of the drug ; however , in practice there is little evidence to support this . the aim of the present study was to investigate the sexual dysfunction in patients taking finasteride 1 mg for androgenetic alopecia.methodsa questionnaire , based on the international index of erectile function , was given to approximately 586 patients with androgenetic alopecia who were being treated with finasteride 1 mg for an average of 16 weeks . these patients were compared to an age - related control group who were attending the dermatology out patients department for various other skin ailments not related to hair disorders.resultsstatistical analysis of the results showed no significant difference in the scores between patients taking finasteride and the control group.conclusionanalysis showed no significant difference in sex - related problems with that of patients taking finasteride and age matched controls , suggesting that sex - related issues are not a side effect of finasteride.electronic supplementary materialthe online version of this article ( doi:10.1007/s13555 - 015 - 0084 - 3 ) contains supplementary material , which is available to authorized users .

cirrhosis is characterized by circulatory changes corresponding to a hyperkinetic state with arterial vasodilatation , decreased systemic vascular resistance , and increased cardiac output . hyperkinetic state and vasodilatation worsen in parallel with the deterioration in liver function . in patients with end - stage cirrhosis , arteriolar vasodilatation is no longer compensated by an increase in cardiac output , resulting in a state of arterial underfilling . arterial underfilling , in turn , results in the activation of vasoconstrictor systems , which causes intense intrarenal vasoconstriction and a marked decrease in glomerular filtration rate ( gfr ) and eventually leads to hepatorenal syndrome ( hrs ) . the increase in creatinine can be abrupt ( type i hrs ) or follow a more progressive course ( type ii hrs ) . a first set of criteria had been proposed in the mid-'90s to better differentiate hrs from other causes of acute renal failure in cirrhosis , the absence of improvement with volume expansion being central in the definition of hrs . however , these criteria proved to be too restrictive . for instance , ongoing bacterial infection was considered an exclusion criterion although experience shows that hrs frequently is triggered by infections . the diagnostic criteria were revisited in 2007 in order to be less restrictive . however , these criteria still have some limitations . firstly , it has been shown that a substantial proportion of patients who are undergoing transjugular renal biopsy and who have a clinical diagnosis of hrs do have intrinsic kidney changes , especially tubulointerstitial injury resulting from either comorbidities or chronic ischemia . secondly , the limit of a creatinine level of more than 1.5 mg / dl to define hrs may be inappropriate . indeed , owing to muscle wasting , some cirrhotic patients with a serum creatinine level of below 1 mg / dl may already have a marked decrease in true gfr . in the previous issue of critical care , a group of experts , including not only hepatologists but also nephrologists and intensivists , reported the results of a workshop aimed at reviewing current knowledge in the field of hrs and proposing new classifications , based on the widely used rifle ( risk , injury , failure , loss , and end - stage kidney disease ) criteria , of all forms of renal impairment in cirrhosis . the group proposed the term ' hepatorenal disorder ' to define any kind of kidney disease that is functional or structural in nature in patients with cirrhosis . acute kidney injury is considered at an earlier stage in patients with a creatinine increase of at least 0.3 mg / dl within 48 hours and/or a 1.5 or more increase in creatinine from baseline . this new classification may help initiate specific therapy at an earlier stage and increase the chance for recovery . this classification is also more appropriate to identify any kind of kidney injury within a wide spectrum of causes of acute , chronic , and acute - on - chronic kidney diseases in cirrhosis . however , even if the patients are identified at an earlier stage , the revised classification does not clearly differentiate those who may benefit from medical therapies of hrs ( that is , terlipressin or noradrenaline ) from those who may not . in addition , this classification does not help address the important issue of reversibility after liver transplantation . in most western countries , the model for end - stage liver disease ( meld ) score determines priority for allocation in liver transplantation . since creatinine is one of the three components of the meld score ( along with bilirubin and international normalized ratio ) , the proportion of patients undergoing liver transplantation with renal insufficiency has significantly increased in the meld era . the rate of post - transplant chronic renal failure has also increased with a 5-year cumulative incidence that may exceed 20% . finally , the number of combined liver and kidney transplantations has also increased with declining outcomes , raising the issue of overuse and misuse in combined transplantation . overall , circulatory changes in cirrhosis necessarily have a deleterious impact on the course of any chronic kidney disease , regardless of the cause and mechanisms . a challenging issue is to determine more accurately the potential for improvement with liver transplantation alone , by reversing hyperkinetic state , in order to better identify those who need a combined liver and kidney transplantation and those who do not . in theory , hrs is a functional disorder that is fully reversible with liver transplantation . however , as discussed above , a number of patients with hrs have intrinsic kidney changes that may not improve after transplantation . therefore , another issue is to better identify the subgroup of hrs patients who are likely to rapidly develop end - stage renal failure after transplantation . unfortunately , in patients who have end - stage cirrhosis complicated by hrs and who are not candidates for transplantation , therapies such as terlipressin or albumin dialysis provide only a modest survival benefit . in the majority gfr : glomerular filtration rate ; hrs : hepatorenal syndrome ; meld : model for end - stage liver disease .

hepatorenal syndrome ( hrs ) is a pre - renal azotemia - like acute renal failure occurring in patients with end - stage cirrhosis . hrs results from arteriolar vasodilatation , arteriolar underfilling , and intense renal vasoconstriction . by definition , it is not responsive to volume expansion , and the prognosis is especially poor even with the use of terlipressin or albumin dialysis or both . it may be difficult , on the basis of the current criteria , to clearly differentiate hrs from other causes of acute renal failure in cirrhosis . in addition , patients with hrs frequently have underlying chronic kidney changes that may not be reversible after transplantation . in the previous issue of critical care , a group of experts proposed a new classification of acute , acute - on - chronic , or chronic renal impairment in cirrhosis on the basis of the rifle ( risk , injury , failure , loss , and end - stage kidney disease ) criteria . the group proposed the term ' hepatorenal disorder ' to define patients with advanced cirrhosis and kidney dysfunction at an earlier stage , regardless of the mechanisms . as stated by the authors , more data are needed to clearly identify , by non - invasive means , those with a potential for improvement with liver transplantation and those who can undergo a combined liver and kidney transplantation .

primary cysts have no clear aetiology and tend to arise spontaneously , while secondary cysts result from a known aetiology such as trauma , surgery or medications [ 1 , 2 ] . primary iris cysts may arise from either the iris pigment epithelium ( ipe ) or the iris stroma [ 2 , 3 ] . in rare instances , an ipe cyst may dislodge and pass from the posterior chamber through the pupil , resulting in a free - floating cyst in the anterior chamber . we describe such a case , where a free - floating iris cyst within the anterior chamber was observed in the setting of recurrent iritis . we also discuss the complications , significance of iritis and management of free - floating iris cysts . a 39-year - old male with a 5-year history of mild recurrent iritis ( 6 episodes ) affecting the right eye was found to have a free - floating iris cyst of approximately 0.5 by 0.8 mm within his right anterior chamber ( fig . mobilisation of the cyst occurred with head tilt but never caused pain or visual compromise ( fig . there were small fluctuations in the size of the iris cyst but no gross or rapid enlargement . his past medical history included reactive arthritis , and he tested positive for hla - b27 . there was no past history of ocular surgery or trauma , and no family history of ocular disease . all episodes of mild iritis affected the anterior segment only and were treated effectively with dexamethasone ophthalmic drops for 26 weeks . he did not have keratic precipitates on his corneal endothelium , anterior or posterior synechiae . the differential diagnosis includes iris stromal cyst , iris or ciliary body melanoma , adenoma of the ipe and medulloepithelioma . ipe cysts have been associated with ocular complications such as angle closure glaucoma , plateau iris syndrome , and secondary pigment dispersion syndrome . however , these complications are uncommon . a case series of 234 patients found that none of the ipe cysts grew larger after a mean follow - up period of 35 months . in particular , no visual or ocular complications were reported in patients with free - floating iris cysts . a study of 62 patients sought to determine current or prior ocular disorders associated with iris cysts [ 1 , 7 ] . four patients had a history of iritis ; however , ocular inflammation was either mild or in remission in 3 of the 4 patients [ 1 , 7 ] . only 1 patient presented with severe iritis and a large iris cyst , which regressed in size upon resolution of the iritis . in another study of 14 patients with iritis , 3 patients showed evidence of new iris cyst formation . this suggests that ocular inflammatory disorders may contribute to the development of iris cysts or cause pre - existing cysts to enlarge . however , the paucity of further evidence means that the full extent of this relationship remains theoretical . in our patient we could not find an association between the small fluctuations in the size of the iris cyst and his episodes of iritis . asymptomatic non - progressive iris cysts are best left alone , and the indications for surgical removal include significant visual disturbances , rapid increase in cyst size over a short period of time , evidence of recurrent iridocyclitis , corneal endothelial touch by the cyst or secondary glaucoma [ 3 , 5 ] . the use of neodymium - doped yttrium aluminium garnet laser to collapse the iris cyst has also shown favourable results in symptomatic patients [ 3 , 9 , 10 ] . our patient remained asymptomatic ; thus , surgical removal or laser treatment was not warranted . few have been implicated in causing visual disturbance or ocular complications , warranting their removal . the paucity of available evidence means that the association between recurrent iritis and the development or progression of free - floating iris cysts remains inconclusive .

purposewe describe an unusual clinical finding of a free - floating iris cyst in a patient with recurrent iritis.methodthe clinical finding of a free - floating iris cyst was recorded using slit - lamp photography.resultsa 39-year - old male with a 5-year history of recurrent right iritis was found to have a small mobile iris cyst within his right anterior chamber , first identified 3 years ago . the patient did not experience any discomfort or visual symptoms resulting from the cyst.conclusionsurgical removal is not indicated for asymptomatic non - progressive free - floating iris cysts . the significance of a free - floating iris cyst in the setting of recurrent iritis remains unknown .

some other causes can be due to postoperative adhesions , duplication cyst , meconium ileus , and internal hernia . according to our knowledge , this is the first report of intrauterine volvulus of terminal ileum without malrotation that was diagnosed by ultrasound . our case deals with terminal ileum torsion around its axis with twisting of mesenteric vessels . therefore , it is of medical importance to recognize the radiological signs of bowel torsion as soon as possible . we present a case of volvulus of terminal ileum which was diagnosed and treated surgically in a neonate just 15 h after delivery . a female baby was born preterm at 35-weeks , with parameters appropriate for her gestational age , weighing 2666 g , with apgar 9 - 10 at 1 and 10 min , respectively . maternal history provided a previous missed abortion , otherwise the mother was a healthy woman who had a normal pregnancy and normal fetal screening follow - up findings . on physical examination of the preterm baby investigational work - up was started including , chest x - ray which was normal , arterial blood gases showed mild metabolic acidosis , blood and urine culture were obtained , and wide spectrum intravenous antibiotics were started . the baby was referred to our radiological department for plain abdominal x - ray at age of 2 h. the x - ray showed a distended and gasless abdomen , except for a small amount of air in the stomach [ figure 1 ] . female baby at 2 h after birth with distended abdomen diagnosed with intrauterine volvulus of terminal ileum without malrotation . x - ray of abdomen shows distended and gasless abdomen except a small amount of air in the stomach ( black arrow ) . this radiological finding might be normal at this age , but since the baby was crying it was assumed to have more air in the intestine . thus , we started an upper gastrointestinal study to rule out any cause of intestinal obstruction . the barium study ruled out a malrotation showing a normal gastric , duodenal , and normal location of the treitz ligament [ figure 2 ] . two hours after the start of the barium study , we noticed lack of propagation of the barium at the level of right lower quadrant of the abdomen . this led us to suspect ileal atresia , which is the most common cause of obstruction after malrotation . sign with filling defect and a round structure around this end loop [ figure 3 ] . this radiological finding suggested ileal obstruction due to volvulus with presumed duplication cyst as the leading point . female baby at 2 h after birth with distended abdomen diagnosed with intrauterine volvulus of terminal ileum without malrotation . female baby at 13 h after birth with distended abdomen diagnosed with intrauterine volvulus of terminal ileum without malrotation . at 13 h , the barium study shows stopage of barium with a beak - like shape of the pre - obstructed bowel ( black arrow ) with filling defect(white arrow ) and a structure containing air around the end loop ( arrowheads ) immediate ultrasonographic examination of the abdomen was done to confirm the diagnosis . ultrasound scan showed a target lesion consistent of two loops of bowel , pneumatosis of the bowel wall , and twisting of vessels [ figure 4 ] , suggesting a diagnosis of ileal volvulus with intestinal ischemia . intraoperative findings showed volvulus of terminal ileum with intestinal ischemia and perforation with free meconium in the abdomen . we think that meconium in this case was the main predisposing factor for the volvulus of this baby . female baby at 13 h after birth with distended abdomen diagnosed with intrauterine volvulus of terminal ileum without malrotation . ultrasonographic examination of the abdomen shows a target lesion consistent with two loops of bowel , pneumatosis of the bowel wall ( arrowheads ) and twisting of vessels ( white arrow ) . according to our knowledge , this is the first case of intrauterine volvulus of terminal ileum without malrotation , that was diagnosed definitively by ultrasound and operated on within a few hours after birth thus saving the life of the baby . neonatal volvulus is secondary to malrotation , and involves twisting of bowel loop with its feeding vessels that leads to ischemia , necrosis , and perforation with fatal complication , if not recognized and treated in time . however , other rare predisposing causes are meckel 's diverticulum , duplication cyst , and meconium plug . diagnosis of volvulus in any suspected neonatal intestinal obstruction by plain film and barium study is extremely difficult especially when malrotation is ruled out . the most common causes of these cases of intestinal obstruction are intestinal atresia that is fatal in the first hours of life if not treated immediately . based on our case , we suggest : in any case of suspected intestinal obstruction , first we have to rule out intestinal malrotation with an upper gastrointestinal barium study . once malrotation is ruled out , we suggest immediately performing an ultrasound examination of the abdomen to rule out an ileal volvulus . thus , ultrasound examination that shows a target lesion bird beak sign of the involved bowel allows to quickly identify this morbid entity without loss of precious time .

neonatal terminal ileum volvulus in the absence of malrotation has never been reported before in english literature . however , another similar rare entity known as neonatal primary segmental volvulus without malrotation has been reported before . volvulus , in general , is an extreme emergency and cases not diagnosed in time lead to death . the main diagnosis is based on radiological features seen on imaging . we present a case of volvulus of terminal ileum that was diagnosed and surgically treated at age of 15 h ensuring the newborn survived . the definitive diagnosis was based mainly on ultrasonographic findings .

ant was developed by bruno latour , michel callon and john law in the field of science and technology studies during the 1980s . although it carries theory in its name , ant is better understood as a range of methods for conducting research which aims to describe the connections that link humans and non - humans ( for example , objects , technologies , policies and ideas ) . in particular , ant seeks to describe how these connections come to be formed , what holds them together and what they produce . researchers using ant are interested in connections between humans and non - humans because they subscribe to the notion that everything that exists in the world is the outcome of an interaction between two or more human and/or non - human entities . using ant means ascribing equal agency to people and things . while it is true that people use data and do things with them , it is equally true that data make people do things , i.e. they influence their work , they structure organizational practices , make organizations take certain decisions rather than others and produce particular effects and affects . this system of mutual influence between people and objects is what ant calls an actor - network . in other words , ant states that actors ( for example , nurses in charge of collecting patient experience data ) act in the way they do and are able to produce effects only through their interactions with other human and non - human entities ( for example , the technical devices used to collect data , the protocols that regulate their work , the chains of authority they are accountable to , the targets they need to meet ) . from this perspective , data are what latour has called actants , entities that are endowed with the potential to produce change in , and in turn to be transformed by , the course of action of other actors . using ant to approach patient experience data has the potential to make two interrelated contributions to existing debates . firstly , it emphasizes the performative nature of quality improvement , bringing to the fore the ways in which quality improvement emerges or fails to emerge as a result of a contingent series of interactions between various human ( individual , institutional ) and non - human actors ( bureaucratic documents , policies , technologies , targets , etc . ) . in the case of patient experience , exploring such performativity of data would mean moving beyond dominant perspectives which see data as inert , open to infinite technical refinement in the service of quality improvement . rather , it would require recognizing that data collected by health care providers produce effects as a result of specific series of interactions with other actors , e.g. more or less competent hospital staff , sympathetic or indifferent policy makers , efficient or faulty technological devices used for their collection and analysis , etc . thus , it may be the case that the interaction of data with a particular presentational and analytical technology ( e.g. meridian or powerpoint ) , makes data more or less compelling to nurse managers , enabling or hampering its journeys to other hospital documents and meetings . secondly , tracing the movement of patient experience data through a health care organization by focusing on its performativity may also lead to important insights into the structure of an organization . as data travel , and translate into reports , narratives and interventions , they make and reveal alternative organizational relations to those which are officially recognized . for example , whereas hospitals are formally hierarchical institutions with a wide range of fixed roles and responsibilities , the contingent interactions in which data get embedded may reveal alternative decision - making processes , and may bring to the fore the role of certain actors ( such as health care assistants or receptionists ) who are conventionally marginal , but who nevertheless often come to play an unexpectedly central role in ensuring the quality of care . a flattened perspective such as this , which treats actors as equally important regardless of their assumed place in an institution , is key to more faithfully account for how quality improvement emerges in practice . by moving away from taken - for - granted organizational and institutional structures through which data are supposed to be gathered , analysed and deployed , such an approach requires that better attention is paid to alternative organizational arrangements as well as to forms of agency which would otherwise go undetected , including non - human agency . thus , in addition to allowing for the role unexpectedly played by certain people within hospitals , the flatness promoted by ant also requires us to pay attention to how quality improvement can be produced or hampered by the agency of specific non - human actors . this would , for instance , mean examining the unorthodox use of certain technologies , or of types of knowledge that are not usually labelled data. casting a panoramic view over the vast web of relations that practically shape quality improvement is essential to move forward the current debates on patient experience data and their role in health care . as mentioned at the beginning of the paper , it is widely recognized that a discrepancy exists between the proliferation of forms of data collection and the limited ways in which such data are used to inform quality improvement . with its flat approach to the mutually influencing relations between actors , ant holds out the promise of bringing to the surface the processes of interaction and negotiation between actors ; it keeps the messy , everyday mechanics of improvement centre stage . exploring these processes is key to helping organizations learn what data are and how they can be best put to use . if we understand data as not only the product of health care organizational structures but also as involved in creating and sustaining them , we may better show how such data shape quality improvement activities . recognizing this potentially creative character of data may offer alternative paths to improvements in patient care which would otherwise go undetected . the author(s ) declared no potential conflicts of interest with respect to the research , authorship , and/or publication of this article . the views and opinions expressed therein are those of the authors and do not necessarily reflect those of the health services and delivery research programme , nihr , nhs or the department of health . the author(s ) disclosed receipt of the following financial support for the research , authorship , and/or publication of this article : this work was funded by the national institute for health research health services and delivery research programme ( hs&dr 14/156/08 ) .

hospitals are awash with patient experience data , much of it collected with the ostensible purpose of improving the quality of patient care . however , there has been comparatively little consideration of the nature and capacities of data itself . using insights from actor - network theory , we propose that paying attention to patient experience data as having agency in particular hospital interactions allows us to better trace how and in what circumstances data lead ( or fail to lead ) to quality improvement .

children with new - onset type 1 diabetes were recruited for a randomized , placebo - controlled trial of low - dose oral interferon- for preservation of -cell function ( the primary outcome of which will be published in a separate report ) . patients had a mean age of 9.4 4.6 years , were enrolled within 6 weeks of diagnosis , and were free of other significant illness ( n = 23 , 30% female , 91% caucasian ) . study approval was obtained from the institutional review board at the national institute of diabetes and digestive and kidney diseases ( niddk ) at the national institutes of health ( nih ) . basal rates were continued but morning bolus injections were withheld . for patients on multiple daily injections of insulin , morning doses were withheld . the meal consisted of boost high protein , 7 ml / kg ( maximum 400 ml ) , ingested over 5 min . blood samples were drawn at 10 , 0 , 30 , 60 , 90 , and 120 min . ng / l , with an interassay coefficient of variation ( cv ) of 18% and an intra - assay cv of 15% . c - peptide concentrations were determined with a competitive chemiluminescence assay at the nih clinical center laboratory . nmol / l , with an intra - assay cv of 3.4% ( at 1.45 nmol / l ) and inter - assay cvs of 7.7 and 8.3% at 0.37 and 1.98 nmol / l , respectively area under the curve ( auc ) for glucagon and c - peptide were calculated using the trapezoidal method . the mean of values obtained at 10 and 0 min was used as the baseline value . net glucagon and c - peptide secretion following the mixed meal were expressed as auc . statistical analyses were performed using sas version 9.1 ( sas institute , cary , nc ) . results were described using frequency distributions , pearson 's correlation coefficients , and repeated - measures anova using proc mixed for testing change over time . opposing change ( i.e. , positive for glucagon vs. negative for c - peptide , or vice versa ) was assessed using a sign test based on the sign of the product of the slopes over time for glucagon and c - peptide . all tests were two - sided , with statistical significance defined as p 0.05 . there were no differences between treatment groups ( interferon vs. placebo ) for glucagon or c - peptide auc ; therefore , all 23 subjects were combined for subsequent analyses . subjects showed an increase in glucagon secretion following the mixed meal , with peak glucagon levels at 30 min ( fig . glucagon auc progressively increased over the 12-month observation period ( p = 0.0008 ) , whereas c - peptide auc progressively declined ( p < 0.0001 ) ( fig . the magnitude of change was similar for both hormones ( 37% rise in glucagon vs. 45% decline in c - peptide auc over 12 months ) . of the 23 subjects , 18 ( 78% ) showed opposing changes in glucagon and c - peptide auc over time ( p = 0.01 ) . the percent rise in meal - stimulated c - peptide [ 100 ( peak fasting)/fasting ] declined over time , from 147 22% at month 0 to 85 12% at month 12 . in contrast , the percent rise in meal - stimulated glucagon increased over time , from 63 12% at month 0 to 82 9% at month 12 . fasting glucagon rose from 40 3 ng / l at month 0 to 46 3 ng / l at month 12 . no significant correlations were found between glucagon auc and a1c , insulin dose , or insulin dose - adjusted a1c ( data not shown ) . the phenomenon of elevated glucagon in response to oral glucose or mixed meal testing has been described cross - sectionally in patients with type 1 and type 2 diabetes , generally of several years duration ( 25 ) . more recently , porksen et al . ( 6 ) reported a 17% rise in post mixed meal glucagon ( measured as a single value at 90 min ) from 1 to 12 months after diagnosis of type 1 diabetes . in the present study , we demonstrate a progressive , 37% rise in meal - stimulated glucagon secretion over 12 months after diagnosis of type 1 diabetes , paralleled by a 45% decline in c - peptide secretion . fasting glucagon rose only 15% over 12 months and remained within the normal range of the assay , suggesting that hyperglucagonemia may not be recognized if only fasting blood samples are obtained . as seen in previous studies ( 7,8 ) , the ability of -cells to secrete insulin in response to a stimulus declined over time in our patients with type 1 diabetes . in contrast , the ability of -cells to secrete glucagon in response to a stimulus increased over time . this suggests that -cell secretory reserve is not diminished by the ongoing autoimmune process in type 1 diabetes . although the pathophysiology of glucagon excess during hyperglycemia in type 1 diabetes is not well understood , the opposing directions of abnormal glucagon and c - peptide secretion over time support the link between dysregulated glucagon secretion and declining -cell function . these data represent the most detailed time course to date of the progression of abnormal glucagon secretion in type 1 diabetes . we did not observe a significant correlation between glucagon auc and a1c or insulin dose ; however , this may be related to small sample size . nevertheless , the timing and rapid progression shown here suggest that excess glucagon secretion may represent a distinct therapeutic target early in the course of type 1 diabetes .

objective to determine the time course of changes in glucagon and insulin secretion in children with recently diagnosed type 1 diabetes.research design and methods glucagon and c - peptide concentrations were determined in response to standard mixed meals in 23 patients with type 1 diabetes aged 9.4 4.6 years , beginning within 6 weeks of diagnosis , and every 3 months thereafter for 1 year.resultsglucagon secretion in response to a physiologic stimulus ( mixed meal ) increased by 37% over 12 months , while c - peptide secretion declined by 45% . fasting glucagon concentrations remained within the normal ( nondiabetic ) reference range.conclusionspostprandial hyperglucagonemia worsens significantly during the first year after diagnosis of type 1 diabetes and may represent a distinct therapeutic target . fasting glucagon values may underestimate the severity of hyperglucagonemia . the opposing directions of abnormal glucagon and c - peptide secretion over time support the link between dysregulated glucagon secretion and declining -cell function .

pelvic digits are rare congenital anomalies that develop in soft tissue.(1 - 4 ) there are less than twenty reported cases in the literature . the majority of reported pelvic digits are asymptomatic and found incidentally during radiography.(2 ) to date , there has never been a report describing a patient with a pelvic digit who presents with a chief complaint of dyspareunia . to the best of our knowledge , this is the third reported case of a symptomatic pelvic digit and emphasizes the importance of considering lesions of the bony pelvis in the differential diagnosis of dyspareunia . a 57-year - old caucasian female presented with a chief complaint of more than one year of dyspareunia . her past medical history includes chronic bilateral knee , hip and ankle pain and had a right total hip arthroplasty that required multiple revisions . she had no pain with hip range of motion and her motor and sensory examinations were intact . review of her radiographs demonstrated a well - positioned right total hip arthroplasty without any evidence of loosening or wear . however , there was an osseous growth projecting from the inferior aspect of left ischium toward the perineal region . additional imaging with computed tomography ( ct ) and non contrast magnetic resonance ( mr ) were used to further define the lesion . representative images , including : a plain radiograph ( figure 1 ) , 3d reconstructed computed tomography ( figue 2 ) and t1 coronal magnetic resonance imaging ( figure 3 ) indicated a bony excrescence from the inferior aspect of the left ischium measuring 1.7 x 1.1 cm . review of a previous ct scan from 2 years prior to the most recent study , demonstrated a stable lesion . plain x - ray : ap pelvis demonstrating pelvic digit arising from the left ischium 3d reconstructed computed tomography demonstrating the pelvic digit magnetic resonance imaging demonstrating a pelvic digit the above studies were suggestive of a pelvic digit . the patient s chief complaint was painful intercourse and she elected to proceed with a resection . pathology found no evidence of malignancy and the patient was discharged without pain and able to ambulate without support of a walker . the removed pelvic digit had two bony segments that formed a pseudoarticulartion or joint , visible in images a and b. follow up via a telephone communication indicated that her dyspareunia had resolved . literature reports have suggested various origins of the pelvic digit , including congenital formation , myositis ossificans , or traumatic avulsion.(1 - 3,5,6 ) in this case , the defining features were that it was well corticated with a pseudoarticulation between bony segments , resembling a digit . typically this would occur prior to the sixth week of fetal life in the mesynchymal stage.(6,8 ) in most reported cases the pelvic digit is an incidental finding on radiography . pandey et al . and maegele report the only other cases of symptomatic pelvic digits in a 35 and a 40 year old man , respectively.(6,9 ) these patients both experienced chronic hip pain , tenderness to palpation of the affected region and limited range of motion.(6,9 ) surgical excision was curative for chronic pain and increasing range of motion in both instances.(6,9 ) in summary , pelvic digits are typically incidental findings on imaging studies . however , in order to facilitate the diagnosis and treatment of pelvic conditions , physicians should be aware that this anomaly can be a source of pain and disability .

a pelvic digit is a rare congenital anomaly and often an incidental radiographic finding . here we present the first reported case of a 57-year - old caucasian female with a pelvic digit and a chief complaint of dyspareunia . radiographic studies and magnetic resonance imaging confirmed a stable bony excrescence from the inferior left ischium impinging on the pelvic floor . the patient underwent surgical removal of the pelvic digit without complications . accurate diagnosis of pelvic lesions is necessary to guide treatment .

mucormycosis is a rare but aggressive opportunistic fungal infection that is commonly caused by members of the family mucoraceae that include rhizopus , rhizomucor , mucor and absidia . mucormycosis can cause severe , sometimes fatal disease in susceptible individuals with uncontrolled diabetic ketoacidosis , neutropenia , chronic glucocorticoid use , hematological malignancy , chronic malnutrition and burn patients.13 rhino - orbito - cerebral mucormycosis ( rocm ) is an uncommon infection in immunocompetent hosts . rocm invades vessels , soft tissue , nerves , bone and cartilage producing tissue infarction and ultimately leading to tissue necrosis and vessel thrombosis.4 we present a case of an immunocompetent patient who survived rocm due to saksenaea vasiformis , and review the literature of this fatal condition . a 40-year - old healthy male presented with painful swelling in the left eye for two weeks . there was no history of eye trauma , dental carries , recent surgery , sinusitis or skin infection . the visual acuity was 6/60 in the left eye with 2-mm axial proptosis , with conjunctival congestion and limited ocular motility . the pupils were reactive bilaterally , with no evidence of a relative afferent pupillary defect in either eye . the patient was diagnosed with orbital cellulitis in the left eye . however , the primary cause was undetermined . broad spectrum antibiotic therapy was initiated that included intravenous cloxacillin 500 mg every 6 h , cefuroxime 750 mg every 8 h and metronidazole 500 mg every 8 h. there was minimal clinical improvement with the above regime after one week of hospitalization . the patient presented two weeks later to a tertiary hospital with severe pain and persistent purulent discharge and no light perception in the left eye . both upper and lower lids were swollen with discharging fistula proximal to the lateral canthus [ figure 1 ] . general examination revealed an alert , afebrile and well - oriented patient with no neurologic deficit . complete loss of globe structure and fistula formation on presentation plasma glucose , blood urea , full blood count and liver function test were normal . computed tomography ( ct ) of the brain and orbit showed destruction of the left eye , with an ill - defined mass and ring enhancement involving left pterygopalatine , inferotemporal fossa and adjacent bony destruction . it extended posteriorly to the orbital apex , causing a widening of the optic nerve canal and the superior orbital fissure . the cavernous sinus showed signs of thrombosis and presence of areas of infiltrate at the left maxillary , frontal and sphenoidal sinuses [ figure 2 ] . ( a ) ct scan of the orbit showing proptosis and distortion of the left globe ( b ) ct scan of brain ( post contrast ) showing an enhancement of the cavernous sinus suggestive of thrombosis a presumptive clinical diagnosis of rocm was made . intravenous amphotericin b 1.0 mg / kg / day was initiated after a test dose . the patient had no significant side effects due to intravenous amphotericin b. the patient consented to exenteration of left eye and extensive sinus debridement surgery . histopathology revealed broad , aseptate , long and right angled branching hyphae consistent with mucormycosis [ figure 3 ] . the intravenous amphotericin b stopped after 4 weeks of therapy and he was discharged with instructions to instill topical amphotericin b for another four weeks . photomicrograph showing aseptate , long broad , slender right angled branching hyphae , which is pathognomonic of mucormycosis postoperatively , the patient refused to repeat imaging and implantation of eye prosthesis due to poor family support and financial constraints . to date , the patient has been followed up for two and a half years with recurrence of rocm . rocm is usually associated with a fatal outcome especially in immunocompromised patients . for the last decade we reviewed 11 cases of rocm in immunocompetent hosts published in pubmed from 2000 to 2011 . table 1 summarizes age , gender , presentations , risk factor , causative organism and final outcome . summary of previous literature on rhino - orbito - cerebral mucormycosis in immunocompetent patients published in pubmed from 2000 to 2011 the presenting age ranged between 16 and 59 years and both genders were equally affected . nine out of 11 published cases were due to apophysomyces elegans.59 there was only one reported case of rocm caused by saksenaea vasiformis.11 apophysomyces elegans is an emerging pathogen that causes rocm via inhalation . whereas , saksenaea vasiformis is a soil saprophytic fungus and the only species in the genus saksenaea . in the previously reported cases , 7 immunocompetent patients who survived were infected by apophysomyces elegans.58 among three patients who died , two were infected by apophysomyces elegans and one with saksenaea vasiformis.8911 two of these three patients were above 50 years of age and died due to extensive brain involvement and severe cerebral vascular insult.811 this finding is similar to the findings of hargrove et al.12 that patients older than 46 years with concurrent frontal sinus involvement and fever were less likely to survive orbital mucormycosis . our patient was very fortunate to survive this fatal condition despite delayed diagnosis and treatment . this is likely due to his relatively young age and the lack of a pre - existing chronic medical illness . to the best of our knowledge , this is the first reported case of an immunocompetent patient with rocm due to saksenaea vasiformis who survived . our patient and other immunocompetent patients who survived rocm were treated with a combination of systemic amphotericin b , orbital exenteration and adjacent tissue debridement.58 this is consistent with roden et al.,1 who reported a survival rate of 70% for cases treated with antifungal and surgery . in contrast , only 61% of cases treated with amphotericin b deoxycholate only , 57% of cases that underwent only surgery and only 3% of untreated patients survived.1 in conclusion , diagnosis of rocm in immunocompetent patients is always misleading and possibly causes delay in treatment . it is essential to alert the managing ophthalmologist of the emergence of this rare disease among healthy individuals .

rhino - orbito - cerebral mucormycosis is a fungal infection that can be fatal especially in immunocompromised patients . it is extremely rare in immunocompetent individuals . we describe here an immunocompetent patient who survived rhino - orbito - cerebral mucormycosis due to saksenaea vasiformis , and provide a literature review of this rare entity .

a 37-year - old nigerian trailer mechanic presented to the eye clinic of the university college hospital , ibadan , with gradual blurring of vision and difficulty reading and identifying peoples faces in both eyes over 10 years . ocular examination showed unaided visual acuity of 6/36 , n48 in both eyes ( ou ) . dilated fundoscopy showed macular atrophy and a beaten - bronze appearance with surrounding yellow white flecks in both eyes ( figure 1 ) . the patient s sister , oa , a 43-year - old , presented with similar history . ocular findings included a visual acuity of 6/36 and similar ophthalmoscopic findings ( figure 1 ) . stargardt s macular degeneration was first reported in 1901 by german ophthalmologist karl stargardt , from whom the disease gets its name.1 the degeneration starts in childhood , but the symptoms may not be apparent until age 3040 years . progressive central visual loss occurs due to photoreceptor damage,2 and color - vision defects are associated with this disease . the inheritance of stargardt s disease can be autosomal recessive or dominant.3 the most common form of stargardt s disease is the recessive form caused by mutations in the abca4 ( formerly abcr ) gene on chromosome 1p21-p13 . the mutations cause the production of a dysfunctional protein ( the adenosine triphosphate - binding cassette transporter ) , producing defective transport of n - retinylidene - phosphatidylethanolamine from the disk space to the cytoplasm of rods and cones . this leads to a buildup of a toxic metabolite lipofuscin , which then causes the photoreceptor and retinal pigment epithelial cells to degenerate by way of membrane permeability , lysosomal dysfunction , and the detachment of proapoptotic proteins , leading to a cell - death pathway.4 central vision loss occurs , while peripheral vision usually is retained.5 symptoms include wavy vision , blind spots , blurriness , impaired color vision , and difficulty adapting to dim lighting . children first notice difficulty in reading , complaining of gray , black , or hazy spots in the center of their vision . they report that a longer length of time is needed to adjust between light and dark environments . onset of symptoms usually is between 10 and 20 years of age.6 our patients presented with poor central vision dating back 10 years the classical oval , snail - slime , or beaten - bronze foveal appearance may be surrounded by yellow - white flecks , which is suggestive of stargardt s disease . in some cases , a geographic atrophy with bull s eye configuration may be seen.7 the two patients presented with the classical findings , as shown in figure 1 . one needs to bear in mind the phenotypic variability in the abca4 retinopathy , as described by burke et al.8 investigations supporting the diagnosis include fundus photo / autofluorescence showing loss of posterior pole autofluorescence and fluorescein angiography with a dark choroid from blockage of choroidal fluorescence by deposits of lipofuscin.9 the focal or pattern electroretinogram is preferred to a full - field electroretinogram . studies showed normal full - field electroretinogram in early stages of the disease.10 the visual field will show a central scotoma . treatment includes supportive measures , such as low - vision devices , photochromic lenses , and genetic counseling . embryonic stem cell therapy is undergoing consideration.11 our patients were reviewed and given low - vision devices . the inheritance of stargardt s disease can be autosomal recessive or dominant.3 the most common form of stargardt s disease is the recessive form caused by mutations in the abca4 ( formerly abcr ) gene on chromosome 1p21-p13 . the mutations cause the production of a dysfunctional protein ( the adenosine triphosphate - binding cassette transporter ) , producing defective transport of n - retinylidene - phosphatidylethanolamine from the disk space to the cytoplasm of rods and cones . this leads to a buildup of a toxic metabolite lipofuscin , which then causes the photoreceptor and retinal pigment epithelial cells to degenerate by way of membrane permeability , lysosomal dysfunction , and the detachment of proapoptotic proteins , leading to a cell - death pathway.4 central vision loss occurs , while peripheral vision usually is retained.5 symptoms include wavy vision , blind spots , blurriness , impaired color vision , and difficulty adapting to dim lighting . children first notice difficulty in reading , complaining of gray , black , or hazy spots in the center of their vision . they report that a longer length of time is needed to adjust between light and dark environments . onset of symptoms usually is between 10 and 20 years of age.6 our patients presented with poor central vision dating back 10 years . beaten - bronze foveal appearance may be surrounded by yellow - white flecks , which is suggestive of stargardt s disease . in some cases , a geographic atrophy with bull s eye configuration may be seen.7 the two patients presented with the classical findings , as shown in figure 1 . one needs to bear in mind the phenotypic variability in the abca4 retinopathy , as described by burke et al.8 investigations supporting the diagnosis include fundus photo / autofluorescence showing loss of posterior pole autofluorescence and fluorescein angiography with a dark choroid from blockage of choroidal fluorescence by deposits of lipofuscin.9 the focal or pattern electroretinogram is preferred to a full - field electroretinogram . studies showed normal full - field electroretinogram in early stages of the disease.10 the visual field will show a central scotoma . treatment includes supportive measures , such as low - vision devices , photochromic lenses , and genetic counseling . embryonic stem cell therapy is undergoing consideration.11 our patients were reviewed and given low - vision devices . families of patients need adequate education and regular exams , especially the younger age - groups . the so - called rare diseases may be common in sub - saharan africa , and detailed retinal examination is important in patients presenting with poor central vision .

stargardt s disease is an inherited macular dystrophy that is transmitted in an autosomal recessive or dominant pattern . the disorder is typically characterized by impairment of central vision , with onset around the first 1020 years of life . stargardt s disease is rare in sub - saharan africa . this is probably the first reported case in the subregion . we present two siblings with the disease . presentation , pathophysiology , and management modalities are discussed .

secundum type atrial septal defect ( asd ) is one of the most common congenital heart defects that occur in adults.1 ) in the past , asd was closed surgically . percutaneous device closure of asd has been developed as an alternative treatment to surgery.2 ) the first percutaneous device closure of asd was performed in 1974.3 ) percutaneous device closure has several advantages over surgery , including less surgical morbidity , avoidance of a scar and reduced hospitalization duration.4 ) however , this method of closure is associated with rare early and late complications . we report a rare complication of silent and late device embolization of the asd occluder device into the right pulmonary artery , three months after implantation . a 16 year - old patient presented with dyspnea . clinical examination revealed normal vital parameters with a fixed split second heart sound and a 2/6 systolic ejection murmur heard best at the left upper sternal border . the patient was initially evaluated by transthoracic echocardiography ( tte ) and had a typical ostium secundum type asd . transesophageal echocardiography ( tee ) confirmed the presence of a moderately large sized secundum asd that measured 20 mm . the aortic rim was almost absent ( 2 - 3 mm ) with other surrounding rims > 7 mm ( not thin ) . the length of the interatrial septum was 45 mm in the longitudinal plane and 48 mm in the shortaxis view . the 30 mm sizing balloon was positioned across the defect and measured by both quantitative angiography and tee at 21 mm . a 24-mm asd septal occluder device ( occlutech figulla asd occluder n , international occlutech ab , helsingborg , sweden ) was deployed with fluoroscopic and transesophageal echocardiographic guidance . before releasing the device , fluoroscopy and the " minnesota tug technique " cessation of flow across the interatrial septum was confirmed on tee prior to final deployment of device . tte was performed 24 hours , seven days and one month after the implantation and confirmed adequate device position with no significant residual shunting . at the third month physical examination , a fixed split second heart sound and a systolic ejection murmur chest roentgenogram showed the asd closure device in the area of the right pulmonary artery ( fig . echocardiography showed device migration into the right pulmonary artery without any significant obstruction to forward flow into the right pulmonary artery ( fig . when the patient was interviewed again , he had complained of mild chest discomfort for a short duration during lifting of a heavy object fifteen days prior to being seen . right ventriculography and fluoroscopy also showed embolization of the device into the right pulmonary artery ( fig . 3 ) . percutaneous removal of the device was not considered because of the position of the device in the right pulmonary artery . the patient was referred for surgical retrieval of the device and closure of the defect , and underwent median sternotomy under general anesthesia , while cardiopulmonary bypass was performed by aorta - bicaval cannulation . asd closure device was removed out of the right pulmonary artery through arteriotomy performed on the main pulmonary artery , and secundum type asd primary closure operation was performed through the right atriotomy . following surgical retrieval , the device was macroscopically intact ( fig . there were no problems in the postoperative follow - ups ; additionally there was no leakage from atrial septum in the follow - up echocardiography . surgical closure of an asd is the gold standard treatment regardless of the size and number of defects present in any clinical case.2)5 ) percutaneous transcatheter closure of asd is an established alternative treatment to surgical closure as it has lesser morbidity , shorter hospital stay , lack of a scar , and comparable rates of complications.4 - 6 ) however , percutaneous device closure is associated with rare early and late complications such as migration or embolization of the device , pericardial effusion , arrhythmias , thrombus formation on the device , and mitral regurgitation and vascular injury.4)7)8 ) the most frequent complication of percutaneous transcatheter closure of asd is device embolization with incidence ranging from 4% to 21%.9 ) embolization usually occurs within 24 hours and after that , it is rarely seen . factors relating to device embolization are associated with the type of device used , larger size of defect , thin rim of atrial tissue , mobility of device postimplantation , use of undersized device and deficiency or absence of aortic rim.2)4)7)8 ) the aortic rim is very important and a margin < 5 mm may predispose to both early and late device embolization.2 ) our patient had a moderately large secundum asd and very small aortic rim ( 2 - 3 mm ) , the combination of which may have resulted in embolization of the device at a late period . another potential cause of late device embolism is acute change in intracardiac pressure due to physical strain . a sudden increase in afterload to the left heart in conjunction with diminished right heart filling ( valsalva ) may have favored the migration of the device to the right and subsequently to the pulmonary artery.10 ) ten weeks after the procedure , when the patient was lifting a heavy object , he had complained of mild chest discomfort for a short duration of time . the history of our patient suggests that an acute change in intracardiac pressure due to physical strain may have dislodged the device . therefore , we believed that both physical strain and small aortic rim were possible causes for device embolism in our patent . we routinely advise to avoid heavy lifting for 3 months in all asd patients who are treated with percutaneous device closure . stricter and longer duration of avoidance of heavy lifting should have been recommended for patients with increased risk of device embolism , as in our case . mashman et al.10 ) also recommended 6 months of avoidance from strenuous exercise for decreasing embolic risk . devices usually embolize in the main pulmonary artery.7 ) if the device embolizes to the pulmonary circulation and impedes pulmonary flow , it may lead to both acute volume and pressure overload of the right ventricular . according to the degree of pulmonary flow impairment , constitutional symptoms usually developed.2 ) in our patient , it was embolized to the right pulmonary artery . it is fortunate that the device position in the right pulmonary artery was in the longitudinal axis in our patient . because the pulmonary flow was not influenced by the embolized device , our patient was asymptomatic . if the device is embolized , percutaneous or surgical removal of the device is indicated.7 ) in our case , percutaneous treatment was not considered because of the orientation of the device in the right pulmonary artery , which precluded snaring the device and the chronicity of the implantation . device embolism , which is the most frequent complication of percutaneous transcatheter closure of asd , may also occur during late periods of post - implantation . in addition , longer duration of avoidance from physical straining must be recommended for patients who have high risk of device embolization .

percutaneous device closure of atrial septal defect ( asd ) is an alternative treatment to surgery . the main advantages of the percutaneous approach include avoidance of surgery , short procedure time and hospital length , in addition to comparable rates of complications . however , percutaneous device closure is associated with infrequent early and late complications including device embolization , air embolism , cardiac tamponade and thrombotic complications . we report a rare complication of silent and late device embolization of the asd occluder device into the right pulmonary artery , three months after implantation .

gastric cancer is the second most frequent cause of cancer - related death in the world and almost two - thirds of the cases occur in asian countries , especially china and japan . although current major therapies , including surgery and chemotherapy , have been widely used , the prognosis of gastric cancer is generally rather poor , with 5-year relative survival below 30% in most countries . however , the main cause of treatment failure in gastric cancer is the development of multidrug resistance to cytotoxic chemotherapies , which is at least in part related to the anti - apoptosis effect . it is well known that the resistance of cell death is one of the hallmarks of cancer cells . although the relationship between autophagy and cancer is still unclear , especially in the regulation of cancer development and progression , there has been much important progress in our understanding that autophagy may have important roles in the treatment of gastric cancer cells . autophagy is an evolutionarily conserved catabolic process by which damaged or long - lived cellular proteins and organelles are degraded . in the cancer cells it is still unclear if autophagy represents a survival mechanism or is involved in type ii programmed cell death ( pcd ) , which is termed autophagic cell death . autophagy is up - regulated when cells need to generate intracellular nutrients and energy , for example , during starvation , growth factor withdrawal , or high bioenergetic demands . moreover , basal autophagy can serve as an important homeostatic cellular recycling mechanism responsible for degrading unnecessary or dysfunctional cellular organelles and proteins in all living cells ; thus , autophagy can be viewed as a potent cytoprotective survival pathway in normal and cancer cells . recently , accumulating evidence has indicated that autophagy is particularly activated during metabolic stress such as nutrient depletion and hypoxia , and has a special homeostatic role mediating removal of old or dysfunctional proteins and organelles , and is particularly important for cell survival during conditions of metabolic stress . moreover , autophagy is the only mechanism for degrading large structures such as protein aggregates and damaged organelles . although autophagy has been induced in many different cancer cell lines , including gastric cancer cells using different agents such as chemotherapeutics , the role of autophagy in tumor cell death or survival is still unclear . autophagy has several adaptive roles in diverse human pathologies , including cancer and other diseases , and can act as a cytoprotective survival pathway . thus , under many conditions , autophagy is induced in response to many different forms of stress , including nutrient and growth factor deprivation and chemotherapeutics , and is utilized as a protective mechanism against cell death in the hostile environment . in fact , more and more results have suggested that autophagy can provide a survival advantage to tumors treated with chemotherapeutic agents . in human hepatocellular carcinoma and colorectal carcinoma cells , moreover , inhibition of autophagy by 3-methyladenine ( 3-ma ) can potentiate cisplatin - induced apoptosis in esophageal squamous cell carcinoma cells . in addition , many studies have revealed that inhibition of autophagy can augment cancer cell death through apoptosis , which indicates that autophagy may act as a protector in tumor cell survival . furthermore , other studies have proved that induction of autophagy can enhance tumor resistance in different tumor cell lines . hypoxia - induced autophagy can decrease hepatoma cell sensitization to chemotherapeutic agents that affect their apoptotic potential . in gastric cancer cells thus , results of many studies suggest that induction of autophagy can protect tumor cells against cancer treatment . previous studies have shown that inhibition of autophagy can potentiate the cell death induced by anticancer drugs in gastric cancer cells [ 1921 ] . therefore , we speculate that inhibition of autophagy may be a strategy for overcoming gastric cancer resistance to therapy . in recent years , the role of autophagy in cancer cells has been investigated extensively . although the impact of autophagy on tumor cells is still unclear and more studies are needed to prove the effects of autophagy in gastric cancer , recent research results suggest that autophagy may be a new target for combatting resistance of gastric cancer to therapy .

gastric cancer is the second most frequent cause of cancer - related death in the world and also causes much morbidity . the acquired resistance of cancer cells to drug reagents is becoming a major obstacle for successful cancer therapy . recently , many studies have revealed that macroautophagy ( here referred to as autophagy ) may be a prosurvival factor and protect the cancer cell from the development of drug - induced death . thus , we propose that autophagy may play an important role in the resistance of gastric cancer to therapy . although the exact role of autophagy in tumor cells is still unclear and further studies are needed to prove the role of autophagy in gastric cancer , recent research findings suggest a new direction in investigating the mechanism underlying resistance of gastric cancer to therapy .

while the term textiloma is used to describe a mass lesion consisting of surgical sponge , the term gossybipoma is reserved for both the mass of sponge and the foreign body reaction around it . these pathologies can mimic other common spinal mass lesions such as hematoma , abscess , soft tissue tumor , etc . their presentation is well known but varies with each case due to different kinds of reactions of body . in literature , 46 cases of gossybipoma involving the spine have been reported;[13419 ] however , it is thought to be more than this number because of medico - legal issues . in this report , we present a case of paravertebral gossybipoma , with a short review of the clinical presentation , radiologic findings and differential diagnosis of these lesions . a 40-year - old woman presented with a history of spinal operation for l4l5 lumbar disk herniation before 8 months and got admitted with non - purulent serous leakage from a small ( 5 mm ) detachment in the surgical wound . the wound was minimal erythematous at the detachment site , but there was no tenderness , swelling or fluctuation . there was no fever , and routine laboratory tests including complete blood count , erythrocyte sedimentation rate , c - reactive protein and blood biochemistry were all normal . microbiologic investigations of the serous leakage revealed no pathogens . while waiting for the microbiological results , treatment with first - generation cephalosporin was started and continued for 4 days , and the serous leakage stopped immediately with secondary healing of the wound in a 1-week period . however , the patient 's same complaints recurred , and thus she got admitted again to our clinic . a computed tomography ( ct ) of the lumbar vertebrae revealed a hyperdense mass lesion located in the left side of the previous operation site . magnetic resonance imaging ( mri ) showed a mass lesion in the left paravertebral area , which was hypointense on t1- and t2-weighted images , with peripheral hyperintense ring in t2-weighted images . mri post - contrast images showed ring enhancement of the lesion [ figure 1 ] . lumbar imaging of our case revealed a paravertebral mass lesion located in the left side of the previous operation site ( arrows ) . ( a ) axial ct scan , ( b ) axial post - contrast enhanced mri , ( c ) sagittal t2-weighted mri the patient was operated by lumbar midline reincision , and the exploration of the left paravertebral area revealed a retained sponge . the sponge was found adherent to the surrounding soft tissue by the new formed fibrotic tissue , which required individual dissection of these fibrotic attachments . histopathologic examination revealed mononuclear cell infiltration and fibrosis formation around the retained sponge [ figure 2 ] . the patient 's complaints showed improvement with no neurologic deficits , and the patient was discharged on postoperative day 3 without complications . a photomicrograph shows the mononuclear cell infi ltration and fi brosis formation around the retained sponge ( h and e , 200 ) many different kinds of hemostatic agents absorbable and non - absorbable are used to control intraoperative bleeding in neurosurgical operations . non - absorbable materials include various forms of cotton pledgets and synthetic hemostats , which should be removed before surgical closure . retained surgical sponges can be found following abdominal , gynecologic , urologic , thoracic , orthopedic or neurosurgical procedures . they are encountered in abdominal and thoracic cavities more commonly but they are also reported after extremity and spinal surgeries . a retained surgical sponge is thought to be a common entity ; however , due to the medico - legal issues , only a few cases in the literature have been reported . while the term textiloma is used to describe a mass lesion consisting of surgical sponge , the term gossybipoma is reserved for both the mass of sponge and the foreign body reaction around it . in the literature , there are 46 reported cases of gossybipoma after spinal surgery since 1965.[13419 ] in these reports , patients had presented mostly with complaints of back pain , common motor weakness and/or sensory deficits in neurologic examination , with no infectious findings , at which the placement of surgical sponge occurred at least a couple years ago before the admission.[151719 ] on the other hand , some cases admitted with fluid leakage after only a short time of the first operation . in our case , the patient presented with sterile serous leakage from the operation wound , 8 months after the previous operation . after surgery , the body gives two types of foreign body reactions against retained sponges : ( 1 ) the exudative type tissue reaction , which leads to acute abscess formation , with a tendency to form fistulas through the skin and ( 2 ) aseptic fibrous tissue reaction , which involves slow adhesion formation , such as encapsulation and granuloma formation . while the time interval to clinical presentation is short with the exudative type tissue reaction , it ranges to even decades after surgery with aseptic fibrous tissue reaction . surgical sponges with radiopaque markers are used now in most of the medical centers . due to this imaging characteristic of the gossybipomas , plain radiographs and/or had reported 13 patients with gossybipomas in thorax and abdomen , and remarked that the radiopaque marker inside the gossybipoma was seen in only nine patients ( 69.2% ) and even then did not always lead to diagnosis . in our case , the gossybipoma was revealed in ct scans as a hyperdense mass lesion , although it was not diagnostic for this lesion . because the differential diagnosis of paraspinal lesions in patients with history of spinal operations include hematomas , abscess or residual / recurrent tumors , mri with intravenous contrast enhancement is known to be the best radiologic investigation modality in these situations [ table 1 ] . kim et al . stated that mri usually shows a well - defined mass with a fibrous capsule that exhibits low signal intensity on t1-weighted images compared with the signal intensity of the paravertebral back muscles , high signal intensity in the center with hypointense rim on t2-weighted images , and strong peripheral enhancement in post - contrast images . on the other hand , mri of our case demonstrated a mass lesion , which was hypointense on both t1- and t2-weighted images , with peripheral hyperintense ring in t2-weighted images and peripheral enhancement in post - contrast images . accordingly , we believe that despite the importance of the mri in the diagnosis of gossybipoma lesions , the definitive diagnosis must be mainly aided by the high suspicion profile of the physician and the intraoperative findings . in patients with the history of spinal operation , gossybipomas should always have a place in the differential diagnosis of newly found lesions , as it is believed that they are much more common than they are reported . however , no pathognomic radiologic characteristics are defined for these lesions . for this reason , the definitive diagnosis must be mainly aided by the high suspicion profile of the physician and the intraoperative findings . thus , it must be remembered that careful inspection of the surgical field before closure is still an important basic rule in surgery .

spinal or paraspinal retained surgical sponges ( gossybipoma or textiloma ) are rare incidents and mostly asymptomatic in chronic cases , but can be confused with other masses such as a hematoma , an abscess or a tumor . in chronic cases , the presentation can be as late as decades after the initial surgery ; however , some gossybipomas cause infection or abscess formation in the early stages . the authors report a 40-year - old woman with a history of operation for lumbar disk herniation before 8 months , and got admitted with a complaint of serous fluid leakage from the operation wound . in this report , the authors discuss the clinical presentation , the radiologic findings and the differential diagnosis of gossybipoma .

in the early 2000s , the grading of recommendations assessment , development and evaluation ( grade ) working group developed a framework in which the certainty in evidence was based on numerous factors and not solely on study design which challenges the pyramid concept.8 study design alone appears to be insufficient on its own as a surrogate for risk of bias . certain methodological limitations of a study , imprecision , inconsistency and indirectness , were factors independent from study design and can affect the quality of evidence derived from any study design . for example , a meta - analysis of rcts evaluating intensive glycaemic control in non - critically ill hospitalised patients showed a non - significant reduction in mortality ( relative risk of 0.95 ( 95% ci 0.72 to 1.25)9 ) . the quality of this evidence is rated down due to the methodological imitations of the trials and imprecision ( wide ci that includes substantial benefit and harm ) . hence , despite the fact of having five rcts , such evidence should not be rated high in any pyramid . the quality of evidence can also be rated up . for example , we are quite certain about the benefits of hip replacement in a patient with disabling hip osteoarthritis . although not tested in rcts , the quality of this evidence is rated up despite the study design ( non - randomised observational studies).10 therefore , the first modification to the pyramid is to change the straight lines separating study designs in the pyramid to wavy lines ( going up and down to reflect the grade approach of rating up and down based on the various domains of the quality of evidence ) . another challenge to the notion of having systematic reviews on the top of the evidence pyramid relates to the framework presented in the journal of the american medical association user 's guide on systematic reviews and meta - analysis . the guide presented a two - step approach in which the credibility of the process of a systematic review is evaluated first ( comprehensive literature search , rigorous study selection process , etc ) . if the systematic review was deemed sufficiently credible , then a second step takes place in which we evaluate the certainty in evidence based on the grade approach.11 in other words , a meta - analysis of well - conducted rcts at low risk of bias can not be equated with a meta - analysis of observational studies at higher risk of bias . for example , a meta - analysis of 112 surgical case series showed that in patients with thoracic aortic transection , the mortality rate was significantly lower in patients who underwent endovascular repair , followed by open repair and non - operative management ( 9% , 19% and 46% , respectively , p<0.01 ) . clearly , this meta - analysis should not be on top of the pyramid similar to a meta - analysis of rcts . after all , the evidence remains consistent of non - randomised studies and likely subject to numerous confounders . therefore , the second modification to the pyramid is to remove systematic reviews from the top of the pyramid and use them as a lens through which other types of studies should be seen ( ie , appraised and applied ) . the systematic review ( the process of selecting the studies ) and meta - analysis ( the statistical aggregation that produces a single effect size ) are tools to consume and apply the evidence by stakeholders . changing how systematic reviews and meta - analyses are perceived by stakeholders ( patients , clinicians and stakeholders ) has important implications . for example , the american heart association considers evidence derived from meta - analyses to have a level a ( ie , warrants the most confidence ) . a evidence could have been high , moderate , low or of very low quality.12 the quality of evidence drives the strength of recommendation , which is one of the last translational steps of research , most proximal to patient care . one of the limitations of all pyramids and depictions of evidence hierarchy relates to the underpinning of such schemas . the construct of internal validity may have varying definitions , or be understood differently among evidence consumers . a limitation of considering systematic review and meta - analyses as tools to consume evidence may undermine their role in new discovery ( eg , identifying a new side effect that was not demonstrated in individual studies13 ) . ebm teachers can compare it to the existing pyramids to explain how certainty in the evidence ( also called quality of evidence ) is evaluated . it can be used to teach how evidence - based practitioners can appraise and apply systematic reviews in practice , and to demonstrate the evolution in ebm thinking and the modern understanding of certainty in evidence .

a pyramid has expressed the idea of hierarchy of medical evidence for so long , that not all evidence is the same . systematic reviews and meta - analyses have been placed at the top of this pyramid for several good reasons . however , there are several counterarguments to this placement . we suggest another way of looking at the evidence - based medicine pyramid and explain how systematic reviews and meta - analyses are tools for consuming evidence that is , appraising , synthesising and applying evidence .

surgical excision of abnormal parathyroid tissue whether adenoma , carcinoma , or hyperplasia is considered as the definitive treatment of hyperparathyroidism . surgery is the standard of care due to potential long - term skeletal , cardiovascular , and neuropsychiatric effects of even mild hyperparathyroidism . nuclear medicine imaging techniques have played an important role in the detection and localization of parathyroid adenomas , with 99mtc - methoxy - isobutyl - isonitrile ( mibi ) scan currently being the standard test used . the addition of single photon emission computed tomography / computed tomography ( spect / ct ) has had an incremental benefit in exact localization of the adenomas . although f - choline has been used as a photon emission tomography ( pet ) tracer for some years now , its utility in the detection of parathyroid adenomas has only recently been explored . we are currently studying the role of this modality vis - a - vis 99mtc - mibi scan with early spect / ct in patients with primary hyperparathyroidism . a 45-year - old female patient presented with complaints of bone pain and difficulty in walking for last 3 months . a plain radiograph of bilateral hip joints ordered by a local practitioner showed multiple lytic areas in the pelvis and femora . she was referred to a tertiary care institute for a fluorodeoxyglucose ( fdg ) pet / ct , in which multiple poorly fdg avid skeletal lesions were reported with no evidence of primary malignant pathology . the patient was referred to our institution for further management . a review of the fdg pet / ct revealed a rounded lesion measuring 2.2 cm with mild fdg uptake superior to the upper pole of the left lobe of thyroid just inferior to the left submandibular gland lateral to and abutting the left lamina of thyroid cartilage . her renal function was deranged for at least last 1 year , and last serum creatinine was 1.9 mg% . , we ordered a parathyroid hormone ( pth ) assay which showed a value of more than 3000 pg / ml from two different laboratories . her serum calcium was 10.4 mg / dl ( laboratory normal 8.8010.60 ) and serum phosphorus was 3.60 mg / dl ( 2.404.40 ) , and vitamin d levels were consistently in deficient range . we proceeded with a 99mtc - mibi scan and early , delayed and early spect / ct images were taken which revealed high uptake in the above lesion . this was interpreted as an ectopic left superior parathyroid adenoma [ figure 1a and b ] . as part of our ongoing study , she underwent a f - choline pet / ct study post 3 mci intravenous injection of the tracer , which showed intense uptake in the above lesion [ figure 2a and b ] . we found the lesion to thyroid ratio much higher than that seen in the mibi scan , making the images easier to interpret . a diagnosis of ectopic left superior parathyroid adenoma arising in the probable setting of tertiary hyperparathyroidism was made . one month after surgery , the patient reported a significant improvement in her symptoms and pth levels have returned to near normal . ( a ) planar early and delayed images of 99mtc - methoxy - isobutyl - isonitrile scan showing a focus of increased tracer uptake superior to the left lobe of thyroid ( black arrow ) . ( b ) single photon emission computed tomography / computed tomography done after the early image reveals the same lesion ( white arrow ) measuring 2.2 cm 1.5 cm 2.3 cm inferior to the left submandibular gland , closely abutting the left lamina of thyroid cartilage ( a and b ) maximum intensity projection ( posterior view ) and transaxial images of f - choline photon emission tomography / computed tomography image showing the ectopic left superior parathyroid adenoma with intense uptake ( white arrow ) in the era of pet / ct , various tracers have been tried for accurate localization of parathyroid adenomas . however , the efficacy of fdg , fluorodopa , and c - methionine has not been established so far . a search for a pet - based tracer is ongoing that can be as good as mibi scan or better . f - choline has recently been reported as a possible novel pet agent for parathyroid localization preoperatively . the possible mechanism for choline uptake is its conversion into phosphatidylcholine by the enzyme choline kinase and subsequent incorporation into the cell membrane . a recent study by lezaic et al . in 24 patients revealed that the sensitivity and specificity of f - choline pet / ct were 92% and 100% , and sensitivity was much higher than any of the conventional scintigraphic techniques . cazaentre et al . reported incidental uptake of f - choline in parathyroid hyperplasia in a case of prostate cancer . in a study of 12 patients by michaud et al . , the authors found a detection rate of 92% for f - choline pet / ct and it also solved discrepant results between mibi scan and ultrasound . reported a case where f - choline pet / ct enabled a minimally invasive surgery for parathyroid adenoma in a patient with negative ultrasound and mibi scan . no larger studies have been performed in this regard yet . at our institution , we do not have c - choline , and synthesis of f - choline has only recently started . in this particular case , the diagnosis was reliably made on mibi scan , but f - choline pet / ct proved to be as good as mibi scan . furthermore , unlike mibi scan , the choline pet / ct in our case showed a very high lesion to thyroid ratio . thus , it may be easier to report smaller lesions very close to the thyroid with this modality . it appears to provide additional hope specially in cases of low gland mass and multi - gland involvement due to better resolution of pet / ct as compared to spect / ct coupled with good image contrast . we feel that f - choline is , therefore , a promising tracer requiring further investigation .

recently , the role of 18f - choline in the detection of parathyroid adenomas has been reported . at our institution , we are currently studying the role of this tracer in comparison to the standard methoxy - isobutyl - isonitrile.(mibi ) scan with single photon emission tomography / computed tomography . our initial results show that 18f - choline is at least as good as 99mtc - mibi scan . we present here a representative case of a 45-year - old woman with multiple skeletal lytic lesions and a high parathyroid hormone.(pth ) who underwent both these imaging techniques with concordant results , further confirmed by histopathology and postoperative fall in serum pth levels .

extragonadal germ cell tumors ( eggcts ) have the same histological components as their gonadal counterparts , but a gonadal mass is not detectable by palpation or high - resolution ultrasonography ) here , we report a case of primary mediastinal seminoma with complete obstruction of the superior vena cava and no recurrence of the residual mass after intensive chemotherapy . his bilateral jugular veins were distended , and swollen lymph nodes were palpable in the right subclavicular region . a chest x - ray showed a mediastinal mass . computed tomography ( ct ) of the chest revealed a solid soft tissue mass ( 95 75 112 mm ) [ in diameter ] in the upper mediastinum , as well as a leftward displacement of the trachea and complete obstruction of the superior vena cava ( figure 1figure 1pretreatment ct shows a solid soft tissue mass ( 95 75 112 mm ) in the upper mediastinum , with leftward displacement of the trachea and complete obstruction of the superior vena cava . ) . based on the suspicion of a mediastinal tumor with lymph node metastasis , mediastinal radiotherapy and methylprednisolone were immediately started . biopsy of the right subclavicular lymph nodes revealed metastatic seminoma ( figure 2 figure 2biopsy specimen from a right subclavicular lymph node . well - defined tumor cells with large nuclei and clear nucleoli and a large clear reticulum are formed as map - like alveolar tumor cells . cobblestone growth can be seen with lymph node involvement surrounding the alveolar tissue . ) , and the patient was referred for chemotherapy . pretreatment ct shows a solid soft tissue mass ( 95 75 112 mm ) in the upper mediastinum , with leftward displacement of the trachea and complete obstruction of the superior vena cava . well - defined tumor cells with large nuclei and clear nucleoli and a large clear reticulum are formed as map - like alveolar tumor cells . lactate dehydrogenase , -human chorionic gonadotropin and alpha - fetoprotein were all in the normal ranges . ultrasound revealed a uniform , echogenic structure of the bilateral testicles , without any findings suggestive of malignancy . radiotherapy and methylprednisolone were discontinued , and systemic chemotherapy was simultaneously started with a combination of cisplatin , bleomycin and etoposide ( bep ) . since lung function test values deteriorated after one cycle of bep , bleomycin was excluded from subsequent chemotherapy , and the regimen continued with etoposide plus cisplatin ( ep ) . a partial response was observed after completing 3 cycles , but no further shrinkage was observed on subsequent examinations . salvage surgery was then considered for the residual mass because the superior vena cava was still completely obstructed by the tumor . although implantation of a prosthetic vessel was required , the procedure was not performed because it was considered to be a high risk surgery . instead , the patient received 2 courses of vinblastine , ifosfamide and cisplatin ( veip ) as salvage chemotherapy . on completion of the chemotherapy , the evaluable lesions were considered to represent a " stable disease " ( figure 3figure 3posttreatment ct shows less enhancement of a smaller soft tissue mass ( 30 18 36 mm ) . ) . as of december 2010 , there is no evidence of residual tumor growth , new masses or metastatic foci . the patient is being closely followed up on an outpatient basis and has been free of recurrence for 32 months after intensive treatment . posttreatment ct shows less enhancement of a smaller soft tissue mass ( 30 18 36 mm ) . our patient was first diagnosed as having an egct arising from the mediastinum when he presented with chronic cough and complete obstruction of the superior vena cava . since few patients with early germ cell tumors arising in the mediastinum have any symptoms , these tumors are often quite large by the time they are diagnosed and the mean maximum diameter of such tumors is reportedly 4.6 cm ) in 2008 , kesler et al . reported that 10 surgical deaths occurred among 158 patients who underwent additional resection of residual primary mediastinal nonseminomatous tumors ) like primary testicular seminoma , primary mediastinal seminoma has a good prognosis , and the rate of necrosis of the residual mass is as high as 92% when partial remission is obtained after chemotherapy )

a 20-year - old man was admitted to our hospital with persistent cough and dyspnea . he had bilateral distention of the jugular veins , and swollen lymph nodes were palpable in the right subclavicular region . plain x - ray and computed tomography ( ct ) of the chest showed a solid soft tissue mass in the upper mediastinum , with leftward displacement of the trachea and complete obstruction of the superior vena cava . mediastinal radiotherapy ( 1.8 gy / day ) and methylprednisolone ( 100 mg / day ) were started immediately . biopsy of the right subclavicular lymph nodes revealed metastatic seminoma . the patient was referred for chemotherapy , which was performed with a combination of cisplatin , bleomycin and etoposide ( bep ) . a partial response was observed after completion of 3 cycles of chemotherapy , but there was no further tumor shrinkage after additional salvage chemotherapy . the patient is being followed up on an outpatient basis and has been free of recurrence for 32 months after intensive treatment .

aplasia cutis congenita ( acc ) is a rare congenital malformation characterized by noninflammatory , well - demarcated defects of all skin layers , subcutaneous tissue , with possible co - defects in muscles , periosteum , bone and dura . it manifests usually as a solitary defect on the scalp , but sometimes may occur on the face , trunk or limbs . acc is most often a benign isolated defect , but can be associated with other physical anomalies or malformation syndromes . on the other hand , a split cord malformation ( scm ) , also called a diastematomyelia , is a rare spinal anomaly and refers to a sagittal division of the spinal cord into two symmetrical or asymmetrical hemicords . variants of this malformation associated with a split of the spinal column , spinal bony spurs , myeloceles , myelomeningoceles , lipomas and dermal sinuses has been previously reported in the literature . however , the association of acc with scm is considered very rare variant and only two reports of this condition are available . a full - term girl , the first child of a 24-year - old mother was born with large skin , muscle , bone and dural defect in the lumbo - sacral area . the patient weighed 2800 g. the patient was evaluated by the pediatric clinic and no other congenital malformations were found . the lesion showed 8 5 cm skin defect , covered with transparent arachnoid membrane . underneath nerve tissue of the spinal cord was split by a perpendicular bony spur and connected from its tip to the upper lamina [ figure 1a and b ] . dura matter was seen on both sides of the lesion extending laterally over the paraspinal muscles . brain computed tomography ( ct ) scan showed mild ventriculomegaly without signs of increased intracranial pressure . spinal x - rays showed a bony spur on the l2 vertebral column and laminar defect in the lumbo - sacral area [ figure 1c ] . under general anesthesia and prone position , the arachnoid layer was removed and the split spinal cord was dissected from the bony spur . there was no neural placode and the spinal cord was found with full neurulation . the spur was seen extending from the posterior border of the vertebral body and penetrating the anterior dura [ figure 2a ] . however , dural folds around the base of the spur invaginating between the two hemicords were seen . fibrous attachment between the bony spur and the upper lamina was resected [ figure 2b ] . the bony spur is then resected from its base to expose the opened anterior dura of the spine [ figure 3a ] . the anterior dura was sutured [ figure 3b ] , and the spinal roots were demonstrated [ figure 3c ] . posterior dura was dissected from both the sides from the subcutaneous tissue and reverted to midline and closure was done in a water tight fashion . skin defect was large and direct approximation of the lips of the skin was not possible . a wide subcutaneous undermining was done to allow a z - plasty - type flap closure . however , the post - operative follow - up showed necrosis in the lips of the flap . debridement of the necrotic lips was done and wound was allowed to heal by secondary closure with regular wet dressing and boric acid powder . ( c ) p - a spinal x - ray demonstrating the laminae defects in the lumbo - sacral region ( 1 : bony spur , 2 : fibrous band , 3 : split spinal cord ) ( a ) split spinal cord is dissected from the bony spur . ( b ) fibrous band is cut from the attachment site in the upper lamina ( a ) bony spur is resected from its base to expose the opening of the anterior dura . although the etiology is still uncertain , a variety of possible causes such as genetic syndromes , teratogens , intrauterine infection with varicella zoster or herpes simplex viruses , fetal exposure to cocaine , heroin , alcohol or antithyroid drugs , infarction of the placenta , and amniotic pathologies are described in the literature . the incidence is 1 to 3/2000 to 10,000 and 25% of the reported cases are familial with a vast majority ( 69% ) showing an autosomal dominant inheritance or part of a syndrome . lesions can be multiple and on different surfaces of the body , but most of the cases have solitary scalp lesions ( 70% ) . it can also be associated with other physical anomalies such as defects of eyes , extremities , limbs , gastrointestinal system , genito - urinary system , and central nervous system . in our case , the defect was seen in the lumbo - sacral area which is besides considered very rare . scm is an unusual congenital anomaly characterized by sagittal clefting of the spinal cord or filum terminale . pang et al . proposed a unified theory of embryogenesis in which all double cord malformations result from a basic ontogenetic error occurring around the time when the primitive neuroenteric canal closes . this basic error is the key step in the formation of an abnormal ( accessory ) neuroenteric canal ( fistula ) through the midline embryonic disc that maintains communication between the yolk sac , which is of endodermic origin , and the amnion , which is ectodermic in origin . an endomesenchymal tract condenses around this accessory canal , splitting the developing notochord and causing two hemineural plates to form . scms are classified as one of 2 types according to the unified theory . in type i scm , the hemicords are always invested with individual dural sacs and the medial walls of the sacs always ensheath a rigid ( bony or cartilaginous ) midline spur . in type ii scm , hemicords are always within a single dural sac and the midline septum is composed of non - rigid fibrous or fibrovascular tissues . according to this classification , our case can be diagnosed as type i scm with completely separated dural sacs and a bony spur . cutaneous stigmata are more common in cases of scms than in other spinal dysraphism , with an incidence rate of 20 to 55% ; hypertrichosis is the most frequent skin manifestation . the spinal cord was split by a perpendicular bony spur and connected from its tip to the upper lamina . the spur was seen extending from the posterior border of the vertebral body and penetrating the anterior dura . there was no neural placode and the hemicords were found with full neurulation reflected with the fully intact motor power in the lower limbs and excluding the diagnosis of myeloschisis . this association of both pathologies may be explained by an arrest during embryologic development of the skin and subcutaneous tissue formation ( as known for acc ) and of the regression of the neurenteric canal or the break between the ectoderm and endoderm forming the fistula due to common pathologic process , such as ischemia . in our case , the mother was previously healthy and there was no drug intake history which could be considered as teratogenic . recent studies demonstrated that magnetic resonance imaging ( mri ) and 3d ct are useful for the diagnosis of scm . in this case , however , we approached this variant as an open dysraphism without preoperative mri as the lesion was totally exposed and identifiable . careful intraoperative exploration was sufficient for the identification of the anatomy and the management of this variant .

a full - term newborn girl born with large skin , muscle , bone and dural defect in the lumbo - sacral area . the lesion included a split spinal cord by a perpendicular bony spur and connected from its tip to the upper lamina . patient was diagnosed with aplasia cutis congenita ( acc ) associated with type i split cord malformation ( scm ) . neurological examination of the lower extremities was normal . spinal x - rays showed a bony spur on the l2 vertebral column and laminar defect in the lumbo - sacral area . lesion was operated and closed according to anatomic layers . clinical and intraoperative findings of this extremely rare case are discussed .

pilomatricoma also known as benign calcifying epithelioma of malherbe is an uncommon slow growing benign adnexal skin tumor , which differentiates towards the hair matrix . , we report a case of pilomatricoma with bullous appearance in a young female which is rare , and also review the literature on bullous pilomatricoma . a 17-year - old girl presented with a solitary reddish semi - transparent blister over her right upper arm since 3 months . initially , the lesion started as a solid pin - head sized papule , which gradually progressed to form a semi - transparent blister with a hard nodule inside . no history of similar lesions in the past or in family members . on examination , single 2 cm sized erythematous semi - transparent bullae with a white nodule was seen on the medial aspect of the right upper arm [ figure 1a and b ] . on palpation , the bullae had a jelly like consistency and the nodule underneath was hard . on puncturing the lesion , it oozed a jelly like material . ( a and b ) 2 cm sized erythematous semi - transparent bullae with a white nodule inside photograph of a bouncy ball routine investigations were normal and excision biopsy was carried out [ figure 3 ] . histopathology revealed islands of basaloid cells with central abrupt keratinization and dilated lymphatics with dermal edema [ figures 4 and 5a ] . ghost cells ( shadow cells ) [ figure 5b ] , foreign body giant cell reaction [ figure 5c ] , and small foci of calcification were also seen [ figure 5d ] . with these findings , gross specimen of excised material histopathological features of excision biopsy showing islands of basaloid cells with central abrupt keratinization , shadow ( ghost ) cells , and dilated lymphatics with dermal edema ( h and e , 10 ) ( a ) basaloid cells undergoing abrupt keratinization ; ( b ) ghost cells / shadow cells ; ( c ) foreign body giant cell reaction ; ( d ) small foci of calcification ( h and e , 40 ) pilomatricoma is a benign tumor considered to be a hamartoma of the hair matrix composed of cells resembling those of the hair matrix , cortex , and inner root sheath . it makes up around 20% of all hair follicle - related tumors and is the commonest hair follicle tumor . majority of patients are under 20 years of age , and females are affected more often than males . it is usually a solitary , deep dermal or subcutaneous tumor 3 - 30 mm in diameter situated on the head , neck or upper extremities . clinical variants include , large ( giant ) , extruding or perforating lesions , ulcerative lesions , multiple eruptive cases , familial cases , superficial pilomatricoma presenting as a cutaneous horn and bullous pilomatricoma . association with myotonic dystrophy , gardner 's syndrome , turner 's syndrome , and rubinstein taybi syndrome have been reported with pilomatricoma . however , no such associations with bullous pilomatricoma has been reported . reports on bullous variant of pilomatricoma is sparse , and only 17 cases have been reported worldwide until date out of which only two cases are reported from india . bullous pilomatricoma has a predilection for occurring on the shoulder and upper arm regions , predominantly in females . it can also be found on the neck , trunk , eyelid , and scalp . the peak age of presentation is 10 - 20 years , and mostly of sizes 1 - 3 cm . usually presents as a solitary , flaccid , thick - walled red bullae with an underlying palpable hard nodule . our present case is consistent with these reports . since our case resembled a bouncy ball , we would like to coin this as the bouncy ball sign in bullous pilomatricoma . histopathological hallmarks of pilomatricoma are the tumor nests of basophilic cells and eosinophilic shadow cells . bullous variant in addition to these findings , show dilated lymphatic vessels , giant cell reaction , lymphoedema , disruption of collagen fibers , dilated blood vessels , fibrous capsule , calcification , nests of transitional cells , and necrosis . our case is consistent with these findings which showed islands of basaloid cells with central abrupt keratinization , shadow cells , dilated lymphatics with dermal edema , foreign body giant cell reaction and small foci of calcification . it could be due to mechanical irritation , or it could be a pseudoblister since the bullae - like spaces are filled with lymphatic fluid . another theory , suggests that the tumor cells or infiltrating inflammatory cells produce elastinolytic enzymes which disrupt the collagen fibers and destruct and dilate the lymphatic vessels , which lead to accumulation of lymph fluid in the dermis causing a bullae . however , the widely accepted theory is that obstruction of lymphatic vessels and congestion of lymphatic fluid caused by the growth of tumor nodule causes dilatation of lymphatic vessels , leakage of lymphatic fluid , and edema in the dermis surrounding the tumor resulting in the bullous appearance . malignant change ( pilomatricarcinoma ) is recorded in several cases , and appears to arise chiefly in large pilomatricomas that have been present for a long time . , dermatologists should be aware of this rare variant of pilomatricoma and excision biopsy is the norm for diagnosis and treatment of this condition .

pilomatricoma ( pilomatrixoma ) or calcifying epithelioma of malherbe , is a tumor with differentiation toward hair cells , particularly hair cortex cells . it frequently presents as a firm , deep - seated nodule that is covered by normal skin . bullous pilomatricoma is an unusual clinical variant . reports on bullous variant of pilomatricoma is sparse , and only 17 cases have been reported world - wide until date out of which only two cases are reported from india . we present a report of a 17-year - old female with a solitary reddish semi - transparent blister over her right upper arm since 3 months resembling a bouncy ball . histopathology revealed tumor nests of basophilic cells and eosinophilic shadow cells , which are consistent with pilomatricoma .

national medicaid outpatient drug expenditure and utilization data are available from two sources : cms ' medicaid statistical information system ( msis ) , and the medicaid drug rebate program data files . since 1999 , all states are required to submit electronically certain fields of claims data from their medicaid claims processing systems to cms ' msis . data files containing utilization and expenditure data on prescribed drugs from the drug rebate program beginning in federal fiscal year ( ffy ) 1996 can be accessed at internet address : http://www.cms.hhs.gov/medicaid/msis/msis99sr.asp . outpatient drug expenditure , but not utilization data , are also available through the quarterly medicaid statement of expenditures ( cms-64 ) . figures 1 and 2 display trends expenditure data on medicaid prescribed drugs under ffs payment arrangements . between ffys 1985 and 2001 , medicaid ffs expenditures for prescribed drugs increased from $ 2.3 to $ 24.7 billion , approximately a 10-fold increase . during the same period , medicaid ffs expenditures for all service types increased from $ 37.5 to $ 216.2 billion , slightly less than a 6-fold increase . between ffys 1997 and 2001 , medicaid ffs prescribed drug expenditures nearly doubled from $ 12.4 to $ 24.7 billion . during the same period , total medicaid expenditures increased from $ 160.3 to $ 216.2 billion , a growth of 35 percent . in ffy 1985 , prescribed drug expenditures in ffs comprised about 6 percent of total ffs medicaid expenditures . by ffy 2001 , in that same year , the medicaid ffs program provided pharmaceuticals to over 20 million beneficiaries . twenty - two percent of the increase in total medicaid expenditures between ffys 1997 and 2001 was accounted for by increases in expenditures for ffs prescribed drugs . figure 3 displays ffs medicaid prescribed drug expenditures by age in ffy 2000 , and figure 4 shows the percent change by age in ffs medicaid drug expenditures between ffys 1999 and 2000 . beneficiaries age 65 or over accounted for the largest share of total medicaid ffs prescribed drug expenditures in ffy 2000 ( 32 percent of the total ) . beneficiaries age 45 - 64 accounted for 30 percent of the total medicaid ffs prescribed drug expenditures followed by beneficiaries age 21 - 44 ( 26 percent of total ) , and beneficiaries age 0 - 20 ( 12 percent of the total ) . between ffys 1999 and 2000 , medicaid ffs expenditures for prescribed drugs increased by 20 percent for the age group 65 or over . age group 45 - 64 beneficiaries experienced the greatest increase in medicaid ffs prescription drug expenditures between ffys 1999 and 2000 ( 26 percent ) followed by the age group 0 - 20 ( 18 percent ) , and the age group 21 - 44 ( 18 percent ) . the following information relates to the medicaid ffs population for ffy 2001 ( october 1 , 2000-september 30 , 2001 ) , unless otherwise stated ( figure 5 ) . this section looks at the 10 states with the highest ffs prescribed drug expenditures in ffy 2001 . prescription payment data in medicaid ffs includes services to individuals in primary care case management . the composition and characteristics of the beneficiaries vary by state , in large part because the proportion of beneficiaries in capitated m+c varies by state . because most beneficiaries in capitated m+c are children and non - elderly adults , states with predominantly ffs populations will have a disproportionate number of elderly medicaid beneficiaries included in the data reported . there were $ 14.1 billion in ffs pharmaceutical expenditures for the top 10 states in ffy 2001 . the $ 14.1 billion figure represented 57 percent of the total ffs medicaid prescribed drug expenditures for that year . california had the greatest amount of prescribed drug expenditures at $ 3.1 billion followed by new york at $ 2.9 billion . florida was third in expenditures at $ 1.5 billion , slightly more than one - half of that of california . missouri was tenth in expenditures at $ 0.5 billion , less than one - fifth that of the top two states , california and new york . figure 6 shows the relationship between percent of the medicaid population who were ( age 65 or over ) by state , and the average number of prescriptions per medicaid beneficiary . figure 7 demonstrates the average number of claims per ffs beneficiary for the top 10 states . the number of pharmacy beneficiaries listed represents medicaid enrollees who received at least 1 prescription through a state 's ffs program . new jersey had the highest average number of prescriptions per beneficiary in 2001 at 33 , followed by ohio at 28 , and massachusetts at 23 . there appears to be a relationship between percent aged in the medicaid population and ffs pharmacy utilization ( figure 8) ; however this relationship is not necessarily a simple linear one . medicaid ffs beneficiaries ( age 65 or over ) accounted for 14 percent of the population and 33 percent of the expenditures . new jersey had both the highest average number of prescriptions per beneficiary and the highest percent aged in the medicaid population among the top 10 states . texas was lowest in average claims per beneficiary and third lowest in percent aged among the top 10 states . the medicaid drug rebate program operates according to the statutory requirements found in section 1927 of the social security act ( social security administration , 2003 ) . states invoice pharmaceutical manufacturers for both innovator or brand - name drugs , as well as non - innovator or generic drugs . while other states have established state supplemental rebate programs , california is the only state that showed an appreciable collection in ffy 2001 . during that year , the state of california collected additional state rebate amounts that totaled 7 percent of their entire pharmacy expenditure ( figure 8) . in ffy 2001 , nationwide , the federal rebate program collected approximately $ 4.9 billion . the average payment per prescription nationwide was roughly $ 55 without the rebate . when the federal rebate program is taken into consideration , the average cost per ffs medicaid prescription nationwide was reduced by approximately 20 percent to $ 44 .

the findings show that with the development of a multitude of safe and effective drugs during the later part of the 20th century and the beginning of the 21st century , drugs are , as never before , a true mainstay of medical treatment . cms continues to monitor drug utilization and expenditures in all of its programs in order to ensure that care is rendered in a cost - effective manner that is beneficial to the patient . this highlight summarizes trends in drug expenditures and utilization in the medicaid program .

in october 2004 , a 3-month - old kitten ( felis domesticus ) was adopted from the society of the prevention of cruelty to animals ( east london , eastern cape province , south africa ) and lived with its owner on a farm 23 km outside the city . it had been neutered and had been vaccinated at 10 months of age with an adjuvanted inactivated vaccine against rabies ( rabisin ; merial , lyon , france ) , but no subsequent vaccinations were given . the cat spent most of the day indoors , but went out at night and returned in the morning . it appeared dull and physically unbalanced and its pupils were dilated but it was not aggressive . the cat was humanely killed , and its brain was sent to the onderstepoort veterinary institute for rabies testing . on june 17 , 2005 , a 6-month - old puppy ( canis familiaris ) was brought by its owner to a veterinarian in the rural town of nkomazi ( mpumalanga province , south africa ) . after symptoms were treated , the dog was discharged , but it was brought back 11 days later because it was paralyzed , dehydrated , and had a fixed stare . the dog was humanely killed , and its brain was sent to the onderstepoort veterinary institute for rabies testing . direct immunofluorescent antibody test with an anti - rabies conjugate cross - reactive with african lyssaviruses showed numerous and strongly stained inclusion bodies in every field of impression smears of both brain samples . isolation of virus was attempted by suckling mouse brain passage and cell culture ( neuroblastoma cells ; diagnostic hybrids , athens , oh , usa ) ; both methods were successful for the cat sample . however , neither method yielded an isolate from the dog sample , despite a lyssavirus - specific reaction in the original brain sample by direct immunofluorescent antibody test . subsequently , antigenic characterization was performed with a panel of 16 monoclonal antibodies to the nucleocapsid protein of rabies virus ( canadian food inspection agency , nepean , ontario , canada ) . gt , genotype ; + , positive reactivity ; var , reactivity with some regional variants ; , negative reactivity . final confirmation of mokv in both case samples was obtained by reverse transcription pcr , nucleotide sequencing , and phylogenetic analysis as described ( 12 ) . phylogenetic analysis ( figure ) showed that the virus isolated from the cat sample ( designated mokv173/06 ) belonged to the same lineage of mokv isolates that were recovered from cats in the same region of south africa ( 12 ) . however , the virus detected in the dog sample ( designated mokv404/05 ) appeared to represent a different south african mokv lineage that was phylogenetically positioned between known south african and zimbabwean lineages . this mokv had nucleotide similarities of 88.1%90.4% and 85.3%88.5% with viruses from zimbabwe and south africa , respectively . phylogenetic tree based on 267 nt of partial nucleoprotein gene sequences of moloka virus ( mokv ) identified with the n1-n2 primer set as described ( 12 ) . the tree shows phylogenetic positions of 2 recently identified cases of mokv infection from south africa ( mokv173/06 from a cat and mokv404/05 from a dog ) ( in boldface ) relative to previously characterized mokv isolates from south africa ( sa ) and zimbabwe ( zim ) and lagos bat virus ( lbv ) as the outgroup . infections with mokv are rare ; only 23 isolates are known . during the past 2 decades , because these viruses are not exclusive to south africa ( 212 ) , lack of isolates from other regions of africa indicates a lack of active surveillance and limited diagnostic capabilities in many african laboratories . to our knowledge , the 2 cases of infection with mokv we report are the first in 8 years from south africa . clinical signs in the dog and cat , including general neurologic manifestations with a lack of aggression , are often signs that warrant submitting samples for rabies testing . whether these variations are caused by neutral genetic drift or reflect different epidemiologic features is not known . phylogenetically , divergence of these viruses into different lineages indicates active cycles and evolutionary changes that occur independently , but in close proximity ( a few hundred kilometers apart ) . although the epidemiology of mokv is incomplete , the case for a reservoir host(s ) among small terrestrial animals of limited range together with recent isolations of rabies - related lyssaviruses in a human ( 13 ) and wild animals ( 14,15 ) , these reports emphasize the endemicity of these lyssaviruses in south africa . public health implications of african rabies - related lyssaviruses should be recognized by laboratory workers , researchers , veterinarians , wildlife personnel , gamekeepers , and pet owners . a better understanding of the epidemiology of these viruses is vital and can only be achieved by improved surveillance and awareness .

we recently identified 2 mokola viruses from domestic mammals ( a dog and a cat ) in south africa . these cases occurred 8 years after the last reported case of infection with this virus . our findings emphasize the endemicity of rabies - related lyssaviruses in south africa and the need to better understand the epidemiology of mokola viruses .

phyllodes tumor ( pt ) is a rare fibroepithelial neoplasm accounting for less than 1% of all breast tumors . benign pt ( bpt ) has hypercellular stromal fragments organized in leaflike patterns around benign epithelial / myoepithelial lined spaces . standardized cytologic diagnostic criteria of bpt includes increased stroma over epithelium , highly cellular stromal fragments , presence of phyllodes fragments ( i.e. , abundant leaflike stromal fragments ) , myxoid stroma , and numerous single bare nuclei of spindle fibroblastic type . diagnostic pitfalls of pt in fine - needle aspiration cytology ( fnac ) may be due to sampling error , high cellularity , ductal hyperplasia , paucity of stromal component , and occasional dissociation of epithelial cells . occasionally , only epithelial components of pt is represented in smears and may be misdiagnosed as epithelial neoplasm , particularly tubular adenoma and carcinoma . here , we present a case of bpt diagnosed by excisional biopsy , but its fnac features were misleading due to predominance of the epithelial component showing a tubular adenoma - like pattern . a 19-year - old female presented with an ill - defined mass in the left breast about ( 2.5 2 ) cm above the areola in the breast tissue . on fnac , smear was cellular comprising of the predominance of benign ductal epithelial cells showing prominent tubular adenoma - like arrangement [ figure 1 ] and resetting arrangement [ figure 2a ] . large branching sheets [ figure 2b ] and papillary clusters of ductal cells without a wavy or folded shape were seen as well . because of diversity in cytological findings , various differentials were thought such as tubular adenoma , adenosis , papilloma , and fibroadenoma . finally , it was cytologically reported as benign proliferative breast lesion with atypical / indeterminate features ( c3 category ) as per the cytologic categorization by the national coordinating committee for breast screening and the uk national breast screening program . as c3 category is a grey area in breast fnac diagnosis , further investigation such as excision biopsy duct epithelial cell clusters showing tubular adenoma - like pattern in fnac ( h&e stain , 200 ) ( a ) duct epithelial cells in sheets showing rosette - like arrangement in fnac ( h&e stain , 200 ) ( b ) large branching sheets of bland epithelial cells ( h&e stain , 200 ) ( c ) duct epithelial cells showing focal apocrine metaplasia ( h&e stain , 400 ) excision biopsy specimen grossly consisted of two grey white soft tissue masses measuring 3.5 cm 3 cm 1 cm and 2 cm 2 cm 1 cm . cut section showed grey white areas . on histology , a well - circumscribed biphasic neoplasm containing leaf - like , epithelial - lined papillary - like projections penetrating into cystic spaces with cellular stroma was seen . though , cytological features of pt have been well characterized , the cytologic diagnosis of pt remains difficult . this tumor is said to be underdiagnosed by the pathologists and undertreated by the surgeons . thus , the preoperative diagnosis of this tumor becomes very important to allow correct surgical planning and to avoid reoperation ( wide local excision with at least 1 cm margin is currently the treatment of choice for bpt ) . pt occurs in women aged 40 - 50 years , however , in asian countries pts may occur at a younger age ( average age , 25 - 30 years ) . cytologic distinction of bpt from fibroadenoma is possible by noting large epithelial clusters longer than 1 mm , with a wavy or folded shape , in contrast to the small or medium - sized clusters with tubular , blunt - branching , or monolayered contours of fibroadenoma . krishnamurthy et al . have observed that hypercellular stromal fragments with the presence of a significant proportion of long spindle nuclei ( > 30% ) is strongly indicative of pt , whereas the stromal cell nuclei in fibroadenoma tends to be short and oval and long spindle nuclei accounts for 10 - 30% . however , in our case , all the abovementioned features were absent , which made cytologic diagnosis difficult . standardized criteria for the diagnosis of bpt include at least two large stromal fragments , hypercellular fragments and moderate - to - large number of dissociated stromal cells . predominance of epithelial elements over stromal elements and monotonous population of cells with tubular adenoma - like and rosetting pattern as seen in this case can mimic epithelial neoplasm . large branching sheets of bland epithelial cells and focal apocrine metaplasia , seen here as well , closely mimics fibroadenoma . presence of papillary - like clusters and acinar pattern could be the reasons to consider papilloma and adenosis as other differential diagnosis . we conclude that if there is significant epithelial proliferation in bpt , a false diagnosis of epithelial neoplasm in fnac is possible and it is an important diagnostic pitfall . so , very careful strict criteria should be used such as advised repeat fnac , cell block preparation , biopsy , or correlation with clinical and radiologic findings to improve preoperative diagnosis of bpt . this will help in correct planning of surgical treatment , i.e. , wide excision with 1 cm margin .

benign phyllodes tumor ( bpt ) is a biphasic neoplasm composed of bland stromal and epithelial elements . cytologic diagnostic criteria of bpt , though documented in the literature , diagnostic pitfalls in fine - needle aspiration cytology ( fnac ) may occur due to sampling error , high cellularity , ductal hyperplasia , paucity of stromal component , and occasional dissociation of epithelial cells . here , we describe a case of bpt diagnosed by histology in a 19-year - old female , where fnac features were inconclusive due to paucity of stromal component , predominance of tubular adenoma - like epithelial component , and due to the presence of other overlapping features with fibroadenoma .

we report a case , carbon dioxide ( co2 ) embolism a potentially devastating complication of laparoscopic surgery , during dissection phase and was successfully resuscitated . a 35-years - old female , weighing 42 kg was posted for transperitoneal laparoscopic boari 's ureteric reimplantation . her , physical examination and routine investigations were unremarkable . with routine monitoring , balanced general anesthesia was given . co2 pneumoperitoneum was created uneventfully using open insufflation technique and sustained with pressure of 14 mm hg . after 2 h , when the surgeon was dissecting the ureters , there was a sudden drop in etco2 ( 8 mm hg ) , tachycardia ( 130/min ) followed by multiple ventricular premature beats ( vpbs ) with blood pressure ( bp ) 110/72 mm of hg and sao2 - 98% . surgical bleeding and endotracheal tube blockage were ruled out , and gas embolism was suspected . injection lignocaine 80 mg intravenously was given for vpbs , and the regular rhythm was restored with the heart rate of 42/min . at this point , the sao2 decreased from 98% to 77% while the breath sounds were normal with no adventitious sounds . since there was no response to these measures , injection dopamine 20 ug / kg / min and nor - adrenaline 0.36 ug / kg / min were infused . multiorifice central venous catheter with its tip at the superior vena cava -right atrium ( svc - ra ) junction was inserted via right internal jugular vein but air could not be aspirated . after 20 min , resuscitative efforts were successful , patient had pulse rate of 120/min , bp 100/54 mm / hg , sao2 100% , etco2 30 mm / hg and ecg showed sinus rhythm with right ventricular strain pattern.at this time the cvp was found to be 10 mm of hg . two - dimension echocardiography ( 2-d echo ) showed no patent ductus arteriosus or any other congenital defects . she was shifted to intensive care unit with dopamine 5 ug / kg / min . which was tapered and stopped after h. the patient did not receive hyperbaric oxygen as we did not have the facility . co2 embolism can occur either during peritoneal insufflationor during the surgical dissection . in this case , possible causes of sudden cardiovascular collapse like hypovolemia , hemorrhage , vaso - vagal reflex , pneumothorax and endotracheal tube blockage were ruled out before considering gas embolism the adhesions caused by previous surgery might have led to vascular injury during dissection and the operating site was above the level of the heart ( trendelenburg position ) creating a pressure gradient thus the open venous channels , at lower pressure were open to gaseous medium at a higher pressure leading to gas embolism . either , slow infusion of air ( 0.01 - 2.00 ml / kg / min ) or bolus of gas ( 25 - 200 ml or 1 - 13 ml / kg ) , creates a gas paradoxical air embolism was ruled out by a 2-d echo that showed no intra - cardiac channels . any kind of pulmonary embolism leads to sudden deterioration of the hemodynamic status associated with decreased etco2 levels . since co2 is a highly soluble gas ( 54 ml / dl ) , prompt recovery of the vital signs is usual . aspiration of gas through central venous catheter is the definitive means of treating gas emboli . in our case , failure to aspirate the gas may suggest that it might have been trapped in the pulmonary vasculature . tee is the gold standard for detection of pulmonary air embolism , however , it is not indicated routinely for intra - operative monitoring . sudden decrease in etco2 is an early sensitive but nonspecific sign of venous air embolismprecordial stethoscope enhances specificity of etco2 and mean pulmonary artery pressure values . immediate treatment of gas embolism requires deflation of pneumoperitonium , placing the patient in durant 's position , hyperventilation and administration of 100% oxygen . aggressive cardiopulmonary resuscitation should be continued till the acute effects of the embolism abate and one should maintain positive circulating volume to prevent further entrainment of the gas . thus , in laparoscopic surgeries early detection of gas embolism with prompt resuscitation can result in a favorable post - operative outcome .

carbon dioxide ( co2 ) embolism is a rare but potentially fatal complication of laparoscopic surgery . we report a case of presumed co2 embolism in a 35-year - old female during laparoscopic ureteric reconstructive surgery . after 2 h of operating time , a sudden decrease in end - tidal carbon dioxide and deterioration of hemodynamic status followed by cardiac arrest with pulseless electrical activity suggested gas embolism . immediate cardiopulmonary resuscitation and inotropic support resulted in successful outcome . thus , early recognition of the complication and prompt treatment can avoid catastrophy .

vascular leiomyoma ( vl ) is an uncommon benign tumor composed of smooth muscle cell and vascular endothelium.1 intracranial involvement is quite rare : in fact , only two reports in the literature described an angioleiomyoma involving the cerebellopontine angle ( cpa ) and internal auditory canal ( iac).23 we present the case of a patient with vl localized within the geniculate ganglion and the initial labyrinthine segment of the facial nerve . to our knowledge , no other vl has been previously described in these sites . in august 2012 , a 45-year - old man came to our department complaining of a grade iii facial paralysis ( house - brackmann scale ) that had arisen 5 months earlier . otoscopy , as well as pure - tone audiometry , was normal with no tinnitus and no vestibular symptoms . magnetic resonance imaging ( mri ) , coronal t1-weighted images revealed a hyperintense , 8-to-10-mm enhancing lesion with contrast englobing the geniculate ganglion without any alteration of the other cranial nerves . preoperative magnetic resonance imaging , coronal t1-weighted images ; the arrowhead indicates 8 to 10 mm hyperintense enhancing lesion with contrast , englobing the geniculate ganglion . we planned an intact canal wall tympanomastoidectomy ( icwt ) with eventual facial nerve grafting . we were able to remove the entire lesion , identifying a cleavage plane with the geniculate ganglion and , thus , preserving the anatomical integrity of the nerve ( fig . postoperatively , the patient had a mild conductive hearing loss ( pta 25 ) . intact canal wall tympanomastoidectomy , tumor arising from the geniculate ganglion and the labyrinthine segment of the facial nerve ( arrowheads ) . intact canal wall tympanomastoidectomy , complete removal of the lesion preserving the integrity of the nerve ; the arrowhead indicates the imprint of the tumor on the geniculate ganglion . postoperative histology showed cells of medium size with an elongated nucleus and irregular eosinophilic cytoplasm , organized in small bundles . in the context of this lesion , numerous vascular spaces were evident with flat endothelium that were compressed by the lesion . immunohistochemical studies showed abundant positivity of tumor cells for smooth muscle actin and negativity for s100 . 4 ) . geniculate ganglion tumor ; the arrowheads indicate the presence of cells with elongated nucleus , eosinophilic cytoplasm organized in small bundles ; positivity for smooth muscle actin ( immunohistochemical , 10 ) . at 3-month follow - up the patient was tumor - free with a recovery of the facial palsy to grade ii . an mri performed at this time confirmed the absence of any expansive lesions involving the geniculate ganglion . vl is a benign tumor , with vascular and smooth muscle components , first reported by stout et al ( 11 cases ) in 1937.1 vl is rare in the head and neck , wang et al4 in a study published in 2004 analyzed 160 patients with vl affecting these sites ; only 21 had a tumor localization in this area . usually , vl develops in the oral or sinonasal cavities , the parotid , submandibular region , carotid sheath , and retropharynx . karagama et al2 and pepper et al3 were the first to describe two cases of vl of the iac and the cpa . to our knowledge , our patient is the first case of vl arising within the geniculate ganglion involving the mastoid and tympanic segment of the facial nerve . morimoto et al5 proposed a classification system that divides vl into three histological subtypes : capillary or solid , cavernous , and venous . some studies have suggested that the solid subtype is the most frequent in the head and neck region.4 microscopically , vl is generally characterized by a circumscribed nodule with well - organized smooth muscle bundles that extend in fingerlike projections from the smooth muscle in the vessel walls . the cells show eosinophilic cytoplasm and elongated basophilic nuclei with tapered endings ( cigar - shaped nuclei).145 vascular spaces , which are lined by a single layer of endothelial cells , are a constant feature in vl and have an intimate association with neoplastic cells . immunohistochemical studies were positive for muscle - specific actin , desmin , and vimentin and negative for s100 protein , cd34 , and cd31.3 histological differential diagnoses include peripheral nerve sheath tumor ( neurofibroma and schwannoma ) and solitary fibrous tumor.13 computed tomography ( ct ) or mri have no specific findings for preoperative diagnosis of vl,6 mass enhancement subsequent to contrast administration , owing to the vascular nature of the lesion , might be characteristic . therefore , only surgical removal and histological examination make it possible to establish a diagnosis with certainty . the best treatment strategy for lesions involving the facial nerve has not yet been fully established . a conservative strategy is often adopted in the presence of normal facial nerve function or minimal preoperative dysfunction.7 in contrast , others proposed not delaying surgery at all , pointing out the increased risk for hearing loss and the better results of facial reconstruction in those patients having no facial palsy.8 when surgical management is selected , the decision regarding the surgical approach to use depends on tumor size and site . when the tumor extends from the geniculate ganglion to the tympanic segment of the nerve , as in our case , the transmastoid extralabyrinthine approach allows access to both these portions and to the beginning of the labyrinthine portion.9 unlike our case , facial nerve integrity may be spared on rare occasions ; more frequently , nerve reconstruction is required . falcioni et al,7 in a retrospective case review analyzing 28 patients undergoing surgery for primary facial nerve tumors , preserved the anatomic integrity of the nerve in 4 cases . all the others required a nerve interruption followed by immediate reconstruction using a sural nerve graft . also , yamaki et al,10 in three cases of geniculate neurinoma after removal of the lesion , achieved facial nerve reconstruction with intratemporal anastomosis of the great auricular nerve . they preferred to use the greater auricular nerve because it can be readily obtained from the neighboring anatomical field . to conclude our study showed that every effort should be made to preserve the anatomic and functional integrity of the facial nerve .

objectives discussion of a rare case of angioleiomyoma involving the geniculate ganglion and the intratemporal facial nerve segment and its surgical treatment.design case report.setting presence of an expansive lesion englobing the geniculate ganglion without any lesion to the cerebellopontine angle.participants a 45-year - old man with a grade iii facial paralysis according to the house - brackmann scale of evaluation.main outcomes measure surgical pathology , radiologic appearance , histological features , and postoperative facial function.results removal of the entire lesion was achieved , preserving the anatomic integrity of the nerve ; no nerve graft was necessary . postoperative histology and immunohistochemical studies revealed features indicative of solid vascular leiomyoma.conclusion angioleiomyoma should be considered in the differential diagnosis of geniculate ganglion lesions . optimal postoperative facial function is possible only by preserving the anatomical and functional integrity of the facial nerve .

we report the first french case of r. aeria ie complicated by brain haemorrhage and femoral mycotic aneurysms , in an immunocompetent patient . a 57-year - old man born in nepal was hospitalized in january 2016 for fever reaching 39.4c , chills , diarrhoea and headache . he had a previous history , in 2008 , of right sub - thalamic ischaemic stroke and severe mitral insufficiency without ventricular dysfunction for dystrophic mitral valve . he did not have recent dental care and had no history of intravenous drug injections . at admission the patient presented with an already known 4/6 meso - systolic rough mitral murmur without cardiac failure and a painful erythema of the right hand ( fig . two aerobically incubated blood cultures performed at admission were positive with gram - positive branching filamentous bacilli ( fig . 1b , c ) within 41 and 86 h , as were the seven other blood culture sets . colonies were identified as r. aeria using matrix - assisted laser desorption - ionization time - of - flight mass spectrometry ( see fig . trans - oesophageal echocardiography revealed mitral ie with several nearby vegetations of the ring base , flail p1 without heart dysfunction or abscess ( fig . pre - operative screening for systemic embolism showed a left frontal subarachnoid haemorrhage on brain magnetic resonance imaging and a hypermetabolic focus in the femoral artery compatible with a mycotic aneurysm on positron emission tomodensitometry ( fig . empiric antibiotherapy was introduced according to european guidelines with intravenous amoxicillin 2 g every 4 h and gentamicin 3 mg / kg once a day . surgical mitral replacement by bio - prosthesis with annular reconstruction was performed according to the risk of cerebral embolism and cardiac abscess at day 10 after initiation of antibiotics . blood culture negativity was obtained at day 3 after antibiotics initiation and valvular culture was negative . 16s rrna gene sequence analysis was performed on the mitral sample and confirmed the identification of r. aeria ( genbank accession sequence kx270977 ) with a maximal identity of 100% for r. aeria type strain a1 - 17b ( genbank accession number ab071952 ) . antibiotic combination was maintained for 2 weeks , then amoxicillin alone for a further 4 weeks . subarachnoid haemorrhage regressed and trans - oesophageal echocardiography showed a good functioning of the prosthesis and the absence of added elements . initially r. aeria was known as rothia dentocariosa genomovar ii and could be mistaken for nocardia spp . except for its antibiotic sensitivity . infective endocardites due to r. aeria ( n = 7 ) and r. dentocariosa ( n = 25 ) , are reported to be associated with central nervous system involvement in 13 of the 32 cases ( 40% ) published , , . antibiotic susceptibility of the r. aeria strains isolated in all case reports showed sensitivity for ampicillin and gentamicin . we report the first infective ie due to r. aeria described in france and confirm that r. aeria ie is serious and associated with mycotic aneurysms and central nervous system involvement . fast and aggressive management with an antibiotic combination including penicillin for 46 weeks , and valve replacement when necessary , is required and could limit the occurrence of complications and death .

rothia aeria is a rothia species from the micrococcaceae family . we report here the first french r. aeria endocarditis complicated by brain haemorrhage and femoral mycotic aneurysms . altogether , severity and antimicrobial susceptibility should make us consider the management of r. aeria endocarditis as staphylococcus aureus methicillin - susceptible endocarditis .

most patients with hodgkin 's disease and spinal epidural involvement either present with concurrent neurological and nodal disease or develop neurological disease after being diagnosed with hodgkin 's disease . a case of a 30-year - old male patient with primary thoracic epidural lymphoma who presented with back pain is presented in this paper . the severity of pain was associated with the inability to perform the activities of daily living . the patient did not have any lymphoma - related b - type symptoms , including body weight loss , fever and sweat at night . a magnetic resonance imaging ( mri ) scan of the thoracic spine demonstrated an epidural tumor at the t911 level ( figs 13 ) . histological examination revealed the polymorphous cellular infiltration by histiocytes , large mononuclear cells and lacunar reed sternberg cells with folded multi - lobed nuclei and small nucleoli ( fig . 4 ) . immunohistochemical staining was positive for cd15 and cd30 and negative for cd3 , cd20 , cd79a or cd45ro . these features were most frequently observed in the mixed cellularity type of hodgkin 's lymphoma . the results of all other examinations ( f-18 fluorodeoxyglucose positron emission tomography ( f-18 fdg pet / ct ) , bone marrow biopsy and computed tomography ( ct ) of the chest , abdomen and pelvis ) were negative for an occult disease . six courses of chemotheraphy containing abvd regimen ( adriamycin , bleomycin , vinblastine and dacarbazine ) were given to the patient . postoperative mri scan did not reveal any evidence of hodgkin 's disease ( fig . 5 ) , f-18 fdg pet / ct , ct of the chest , abdomen and pelvis were obtained in 24 months and did not reveal any evidence of hodgkin 's disease . figure 1:t2 weighted mri revealed a mass at the t9 - 11 level . figure 2:t1 weighted mri revealed a mass at the t9 - 11 level . figure 3:axial t1 weighted mri revealed at the epidural space . figure 4:histological examination revealed mixed cellularity type of hodgkin 's lymphoma . figure 5:postoperative mri scan did not reveal any evidence of hodgkin 's disease . t2 weighted mri revealed a mass at the t9 - 11 level . patients with hodgkin 's disease develop spinal cord compression caused by epidural hodgkin 's lymphoma and usually observed in the presence of an advanced disease [ 1 , 2 ] . there are six case reports in the literature that describe patients with primary spinal epidural hodgkin 's lymphoma [ 16 ] ( table 1 ) . samadian et al . reported a case of primary and isolated spinal epidural hodgkin 's lymphoma at l1l3 level . al - khayat et al . reported a hodgkin 's lymphoma case with epidural involvement at c7t1 level . reported a case with primary extranodal hodgkin 's disease who underwent decompressive surgery accompanied with stabilization and radiotheraphy . reported cases with primary spinal epidural hodgkin 's lymphoma ( s : surgery , rt : radiotheraphy , ct : chemotheraphy e : epidural , v : vertebral , p : paraspinal ) moridaira et al . rao et al . published a case with primary spinal epidural hodgkin 's lymphoma . our case is apparently the seventh case to be diagnosed with hodgkin 's disease who presented with spinal cord compression due to epidural space without lymphoma elsewhere . the abnormal mri marrow signal of the t9 and t10 vertebral bodies can be seen in fig . maybe the tumor in fact originated within the abnormal t9 or t10 bone marrow and spread to the epidural space via the epidural venous plexus . surgery is the first therapeutic approach in malignancies compressing the spinal cord . because hodgkin 's lymphoma is a very chemosensitive and radiosensitive tumor , the indications for surgery were reduced and limited to laminectomy or even biopsy only , leaving the major role to chemotheraphy and radiotheraphy . the combination of chemotheraphy and involved - field radiotheraphy is the most common treatment strategy ; two to four cycles of abvd are considered as the international gold standard for early - stage hodgkin 's lymphoma in combination with 2030 gy of involved - field radiotheraphy [ 7 , 8 ] . we chose the first therapeutic approach for this patient who underwent gross total resection of the tumor , because the tumor type was unknown at the time of initial presentation . the present case demonstrated that physicians should include primary spinal epidural hodgkin 's lymphoma in the differential diagnosis of spinal tumors . treatment of hodgkin 's lymphoma will almost always include in the acute phase a form of emergency decompressive surgery , with or without resection , followed by chemotheraphy and/or radiotheraphy .

primary spinal epidural hodgkin 's lymphoma is very rare . we will discuss the clinical features and treatment of primary spinal epidural hodgkin 's lymphoma . in this paper , a 30-year - old male patient who presented with spinal epidural tumor at the t911 level is reported . subtotal resection of the tumor was performed and the histological examination of the tumor specimen revealed hodgkin 's lymphoma . all other examinations were negative for an occult disease . six courses of chemotheraphy containing adriamycin , bleomycin , vinblastine and dacarbazine were given to the patient . surgery is the first therapeutic approach in malignancies compressing the spinal cord . hodgkin 's lymphoma is a very chemo- and radio - sensitive tumor . the indications for surgery were reduced and limited to laminectomy or even biopsy only , leaving the major role to chemo- and radiotheraphy .

leiomyoma is a benign tumor , which is made up of smooth muscle cells and located mostly in uterus , small bowel , and esophagus . herein , a prostatic leiomyoma that was pathologically reported after a robot - assisted radical prostatectomy ( rarp ) procedure is presented . a 70-year - old male patient with a serum prostate specific antigen value of 9.8 ng / ml underwent a transrectal ultrasound ( trus ) guided prostate biopsy . pathology revealed prostatic adenocarcinoma with 3 + 3 gleason score in 1 core on the left side . the patient had a history of irritable bowel syndrome and had bilateral inguinal herniorrhaphy operation performed 8 years ago . a transperitoneal rarp procedure with bilateral pelvic lymph node dissection was performed ( by dr . briefly , an incision was made on the anterior peritoneal covering of the douglas ' pouch , 1 cm proximal to its reflection on the rectum . seminal vesicles ( svs ) and vasa deferentia were dissected . at this stage , a 3 3 cm sized posterior mass lesion protruding from the prostate between svs was identified and dissected ( fig . figure 2 shows the removed specimen with a mass lesion protruding from the prostate located between svs . postoperative follow - up was uneventful , abdominal drain was removed at the postoperative second day , and patient was discharged on the third day . transurethral catheter was removed on postoperative day-10 followed by obtaining a cystography that demonstrated no leakage . pathology was reported as prostate adenocarcinoma , gleason score 3 + 3 , tumor size of 5 4 3 mm , left apex , and single focus with clear surgical margins . posteriorly located mass pathology was reported as a leiomyoma with a size of 3.5 3 2.8 cm . prostate had a weight of 210 g. microscopy of this mass lesion revealed a fascicular pattern of smooth muscle bundles separated by well - vascularized connective tissue ( fig . smooth muscle cells were elongated with eosinophilic or occasional fibrillar cytoplasm and distinct cell membranes . one to 2 mitotic figures per 10 high power fields in most mitotically active area were observed , and there was no significant atypia ( fig . also a single focus of prostate adenocarcinoma with gleason score 3 + 3 , size of 5 4 3 mm , located on left apex , with clear surgical margins was reported . a mass lesion , made up of smooth muscle bundles , is well demarcated from prostate tissue on the left ( h&e 4 ) . smooth muscle cells with bland cytology form fascicules ( h&e 40 ) . immunohistochemically smooth muscle actin is positive ( inset 40 ) . at postoperative ninth - month follow - up , patient is fully urinary continent with no safety pad usage . he is able to have penile erection for sexual intercourse with use of phosphodiesterase type 5 inhibitors . although pure prostatic leiomyoma is rarely seen , it was reported initially in 1876 in the autopsy material by lebel et al . in 1951 , kaufman and berneike defined prostatic leiomyomas . in 2016 , kristensen et al . stated that prostatic leiomyomas were reported in < 100 cases since it was defined . in addition , the origin of prostatic leiomyoma may reveal as wastes of mllerian duct . however , a certain mechanism of formation of prostatic leiomyoma is still not clearly presented . robotic surgery for the surgical management of a leiomyoma was previously performed by aoun et al . however , in our case the whole prostate with the posteriorly located leiomyoma was removed with the rarp procedure . the console surgeon in our case was challenged with the identification of a large mass lesion located between the svs as this area has already a limited space . therefore , it was surgically not difficult to dissect and isolate this mass lesion . however , the presence of this mass lesion made it challenging to open the denonvilliers ' fascia due to its close proximity with rectum . at this stage , intra - abdominal pressure was elevated as much as 18 mmhg temporarily to better see the area and a 4 close up magnification was used with the da vinci xi surgical robot . fourth arm of the robot holding a prograsp forceps was effectively used to lift up the prostate by holding from the svs in an attempt to make the console surgeon to see the area below the mass lesion . it is important to keep in mind that this maneuver might lead to tearing off the svs if too much force is applied as there is no tactile sensation in robotic surgery . a further trick that might also be applied could be using a 30 up lens to better see the area below the mass lesion . leiomyoma is a benign tumor and has excellent prognosis when complete resection is applied and atypia is not detected . on our case , vandoros et al . stated that negative surgical margins and absence of metastatic disease at presentation were the only factors predictive of long - term survival . therefore , prostatic leiomyosarcomas should also be kept in mind in the differential diagnosis . in conclusion , prostatic leiomyomas that are benign mesenchymal smooth muscle tumors might present as a posteriorly located mass lesion between svs that could challenge the surgeon during surgery , which should be kept in mind .

abstractbackground : prostatic leiomyoma is a benign and rare condition of the prostate . robotic surgery is increasingly being applied in the surgical management of prostate cancer.case presentation : herein , a mass lesion that was located in the posterior part of the prostate between seminal vesicles that was identified during robotic surgery is presented . this lesion further challenged the console surgeon during performing a robotic radical prostatectomy procedure for a 200 g large prostate with prostate cancer.conclusion : prostatic leiomyomas that are benign mesenchymal smooth muscle tumors might present as a posteriorly located mass lesion between seminal vesicles that could challenge the surgeon during surgery , which should be kept in mind .

as a professor in a clinical department , i am privileged to work with many clinician - investigator trainees . recently , a new clinical research fellow told me that he had been reading many papers in order to decide which research project he should take . the more he reads , the more confused he feels . in fact , this challenge is not only to new fellows , but also to experienced researchers . in this issue of critical care , dr li and dr quinn and their colleagues published a research article exploring the molecular mechanisms of ventilator - induced lung injury ( vili ) . i would like to use this interesting article as an example , to lead readers who are not experts in this field through a translational process . mechanical force - induced signal transduction ( mechano - transduction ) is responsible for many physiological processes in lung development , in maintaining lung functions , and in pathological conditions related to lung diseases , such as asthma , chronic obstructive pulmonary disease ( copd ) , and acute respiratory distress syndrome ( ards ) , especially related to vili . however , very much like the routes in a city , both " good boys " and " bad boys " drive on the same streets . neutrophil recruitment and activation is an important mechanism for lung tissue injury , which is mediated by a group of small molecules , namely chemokines , especially a subgroup of c - x - c chemokines . interleukin-8 ( il-8 ) is the best example , which has been shown to be up - regulated by mechanical forces in human lung cells . rodents do not have the il-8 gene , but produce macrophage inflammatory protein-2 ( mip-2 ) and other c - x - c chemokines . they further questioned that neutrophil migration is mediated by a signal pathway activated by akt ( also called protein kinase b ) . they also used a chemical inhibitor to prevent the activation of akt in mice , prior to their exposure to high volume ventilation and/or hyperoxia . however , it is worth mentioning that the akt pathway is also critically important for proliferation , survival and migration of cell types other than neutrophiles . based on their recent studies , these researchers suspected that endothelial nitric oxide synthase ( enos ) activation is also part of the mechanisms responsible for vili . they demonstrated increased phosphorylation of enos and also demonstrated that nitric oxide synthase ( nos ) inhibitor could attenuate vili . increased nos expression and/or activity have been shown in multiple lung injury models and clinical samples . the outcome of no related clinical studies , however , is controversial , which warns us for further investigation . as a clinician you may ask why 30 ml / kg of tidal volume was used in this study and you may note that oxygen was used for ventilation . first of all , as experimental biologists , we always try to create a condition that can give definite answers . for example , after serum starvation , add a growth factor into the culture medium to initiate a signal transduction , or start mechanical stretch on statically cultured cells . these protocols help us to reveal the potential of a biological stimulus on a particular biological process . this strategy has also been adapted to animal studies . on the other hand , this may also explain why many experimental findings did not translate to good clinical practices . as a bench scientist , one should try to simulate clinical conditions as much as possible . recently , clinically relevant models have been emphasized . after going through this interesting paper critically , what have we learnt ? first , good literature review may lead to proposal of a testable hypothesis in vili . secondly , the mechanical force - induced signal transduction by injurious ventilation ( high tidal volume and high oxygen tension ) could activate pathways that are important for normal functions . last but not the least , awareness of the significance and limitation of experimental data are crucial for our knowledge translation . ards = acute respiratory distress syndrome ; copd = chronic obstructive pulmonary disease ; enos = endothelial nitric oxide synthase ; il-8 = interleukin-8 ; mip-2 = macrophage inflammatory protein-2 ; no = nitric oxide ; nos = nitric oxide synthase ; vili = ventilator - induced lung injury .

mechanotransduction holds the underlying mechanisms of ventilator - induced lung injury . research on this subject , however , could be difficult for clinicians , especially when results are controversial . a recent study by li and co - workers is used as an example , to explain how to critically read literatures related to basic science and how to understand the limitation of experimental studies .

the missouri department of health and senior services conducted this investigation in response to the hospital s identification of an increased number of tracheal aspirates that were positive for b. cereus collected from newborns who were on ventilators during march may , 2011 . all tracheal aspirate culture results obtained in the neonatal intensive care unit ( nicu ) during january 2010june 2011 were reviewed . nicu data was also searched for positive b. cereus culture from other specimens , such as blood , body fluids , or tissues . investigators thoroughly evaluated respiratory management practices in the unit by direct observation , respiratory records review , and an interview with the respiratory therapist . several environmental cultures were obtained from the flow sensors of the unit s ventilators over the 1-month period . b. cereus isolates were forwarded to the centers for disease control and prevention to be molecularly characterized by using multilocus sequence typing ( mlst ) ( 10 ) . the dna was used as a template in pcrs with the primers described on the bacillus cereus mlst web site ( www.pubmlst.org/bcereus ) for the 7 loci which define the mlst scheme . the sequences for the loci glpf , gmk , ilvd , pta , pur , pyca , and tpi were then assigned allele designations . a greater number of alleles that match between strains indicates a higher level of relatedness ( 10 ) . prevalence of b. cereus positive specimens was compared by using the mann - whitney u test . retrospective analysis of tracheal aspirate culture results showed significant increase ( p = 0.039 ) in b. cereus isolation between march and may , 2011 ( figure 1 ) . no bacillus spp . were isolated from blood , other body fluids , or tissues during the study period . the chart review of the case - patients comprising the cluster of b. cereus colonization revealed that none received a diagnosis of clinical b. cereus infection . all patients were treated with vancomycin or tobramycin , or both , for indications not related to b. cereus in tracheal aspirate . one case - patient died 108 days later without evidence that b. cereus contributed to the outcome . epidemiologic curve of bacillus spp.positive tracheal aspirates from newborns on ventilators , january 2010january 2012 . investigation of the ventilation procedures in the nicu revealed that most equipment used for respiratory care was disposable , designated for single - patient use . telford , pa , usa ; www.draeger.us/sites/enus_us/pages/hospital/evita-xl.aspx ) was used for mechanical ventilation of infants who were intubated to treat severe respiratory compromise . the draeger evita v500 is a microprocessor controlled ventilator offering both mandatory and spontaneous ventilation modes for adult , pediatric , and neonatal patients . heated and humidified gas flows from the ventilator unit , through the inspiratory circuit and neoflow air flow sensor to the patient through an endotracheal tube . upon exhalation , gas flows back through the air flow sensor into the expiratory circuit and returns to the ventilator through the expiratory flow sensor and exhalation valve . in addition to the ventilator , reusable respiratory equipment comprised a proximal air flow sensor , expiratory flow sensor , exhalation valve , and circuit temperature probe . the sensor closest to the newborn s mouth was an air flow sensor located inside the disposable ventilation circuit ( figure 2 ) . from 9 environmental cultures obtained from 9 air flow sensors , 1 was positive for bacillus spp . , and was later confirmed as b. cereus by the state public health laboratory . mlst was performed for 8 b. cereus isolates from case - patients and for 1 environmental isolate from the air flow sensor . one locus for the remaining 5 strains did not yield an amplicon for sequencing after repeated attempts and , thus , could not be assigned a sequence type . the isolates that included sequence type ( st ) 73 and st94 were closely related to each other because they differed by merely 1 locus , gmk . the strains that were not fully typed because of the inability to obtain sequences for locus pta were also closely related to st73 or st94 because the other loci matched . there was 1 match between strains isolated from 1 case - patient and the air flow sensor , which was st73 . the contaminated air flow sensor was then sterilized by using a steam autoclave . a repeat culture of this sensor after sterilization was negative . we found that air flow sensors were routinely disinfected by placing them in a container with 70% alcohol solution for 60 minutes . after discovery of the air flow sensor contaminated with b. cereus , the disinfection policy was changed . all air flow sensors were first soaked in enzol enzymatic detergent ( asp , irvine , ca , usa ; www.aspjj.com/us/products/enzol ) solution and then sent for steam autoclave sterilization at 134c ( 273.2f ) . after implementation of new disinfection and sterilization procedures , no new cases of b. cereus tracheal colonization were identified in the nursery . in this cluster , contaminated proximal air flow sensors were the likely source of tracheal colonization with b. cereus in newborn infants , supported by a genetic match by mlst between a strain isolated from 1 case - patient and the contaminated air flow sensor . b. cereus transmission from contaminated respiratory equipment has been reported in other geographic areas . in the netherlands , an outbreak of b. cereus infections in a pediatric intensive care unit caused by contaminated reusable ventilator air flow sensors switching to disposable air flow sensors stopped colonization with b. cereus in that unit . in canada , an outbreak of b. cereus infections among patients in an adult icu was linked to colonized ventilator circuitry ( 8) . in the united kingdom , reusable ventilator circuits were also identified as the cause of a b. cereus outbreak among intubated nicu patients ( 9 ) . our investigation underscores the necessity of close monitoring of occurrences of bacillus spp . in tracheal aspirates since clustering of such cases , b. cereus isolates were either st73 , st94 , or closely related to those sequence types . st73 and st94 are associated with strains previously described as having caused illness in elderly persons . strains with st73 were implicated in cases of septicemia ( 12 ) , and of sepsis and pneumonia ( 13 ) . strains with st 94 were recovered from patients with pneumonia ( 14 ) . b. cereus strains harboring b. anthracis plasmids such as pxo1 , have also been associated with severe and fatal respiratory infections ( 15 ) . all case - patients in our investigation were considered to be colonized with b. cereus without clinical implications . since all of them received intravenous antimicrobial drugs effective against b. cereus , it is conceivable that the clinical course of those patients could have been different without such treatment . should not be routinely viewed as clinically insignificant and further testing to determine exact strain should be considered under appropriate clinical and epidemiologic circumstances . proper disinfection of the entire ventilator circuit as recommended by the equipment manufacturer is crucial in avoiding potentially lethal b. cereus infections .

we investigated bacillus cereus positive tracheal aspirates from infants on ventilators in a neonatal intensive care unit . multilocus sequence typing determined a genetic match between strains isolated from samples from a case - patient and from the air flow sensor in the ventilator . changing the sterilization method for sensors to steam autoclaving stopped transmission .

iridodialysis commonly occurs secondary to blunt ocular trauma,1 penetrating ocular trauma , and intraocular surgical procedures . in the cases of small iridodialysis the upper eyelid covers iridodialysis superiorly and prevents symptoms . however , temporal iridodialysis is usually symptomatic . in addition to visual problems such as diplopia , glare , and photophobia , cosmetic problems such as polycoria and ectopic pupil might occur due to large iridodialysis . a number of surgical techniques have been described for the repair of iridodialysis.245678 we describe a novel cobbler 's technique for repairing iridodialysis in the right eye of an 18-year - old patient . following peribulbar anesthesia , a fornix - based localized conjunctival peritomy ( 611 oclock ) was performed in the right eye of a patient with traumatic iridodialysis extending from 7 oclock to 10 oclock position in the inferotemporal quadrant [ figure 1 ] . a partial thickness scleral tunnel was created 1.5 mm from the limbus along the extent of the iridodialysis . a limbal paracentesis was created with a stiletto knife at the 2 oclock position , and intracameral pilocarpine was injected into the anterior chamber to place the iris tissue on the maximal stretch . the 26-gauge needle was passed through the paracentesis and the 10 oclock end of the scleral groove engaging the root of the iris . the needle was withdrawn into the anterior chamber and taken out at the 9.30 oclock position engaging the root of the iris again . the prolene suture was pulled out forming a loop through which the free end of the suture was passed to lock the loop . this step was repeated multiple times until multiple loops laid over the scleral bed [ figure 2 ] . these loops were then tied [ figure 3 ] and the conjuctival peritomy was closed using 8 - 0 vicryl sutures . preoperative photograph showing a corneal scar inferiorly and a large iridodialysis from 7 oclock to 10 oclock position illustration of passage of the 10 - 0 polypropylene suture from iris to sclera with multiple suture loops lying over the scleral bed illustration of suture tied at 7 oclock position first postoperative day photograph of the same eye after iridodialysis repair postoperative photograph 3 weeks following surgery functional symptoms , such as glare and monocular diplopia , result from the polycoria of a pseudo - pupil created by the iridodialysis . typically , these complaints are most commonly seen when iridodialysis is located in the nasal , temporal , or inferior quadrants , as was seen in our patient . cosmetic deformities associated with iridodialysis can affect self - esteem adversely and thus have a major psychosocial impact on the patient.910 various methods for surgical repair of iridodialysis2345678 have been described in literature . most of these procedures require more surgical maneuvers and anterior chamber is often not stable while repairing large iridodialysis . the advantages of cobbler 's technique for repairing iridodialysis as described above include : ( 1 ) it is performed through a small paracentesis wound ; ( 2 ) the anterior chamber remains stable throughout ; ( 3 ) multiple sutures can be passed without withdrawing the needle from anterior chamber ; ( 4 ) large dialysis can be repaired easily ; ( 5 ) the shape of the iris can be controlled ; ( 6 ) only one suture knot is required at the end of the incision which can be easily be buried within the sclera groove minimizing the likelihood of late suture erosion . our novel cobbler 's technique for iridodialysis repair allows a maximally functional and cosmetic result . this technique was named cobbler 's technique since it bears great similarity to the way a cobbler repairs shoes .

we describe a novel cobbler 's technique for iridodialysis repair in the right eye of a patient aged 18 years , with a traumatic iridodialysis secondary to open globe injury with an iron rod . our technique is simple with easy surgical maneuvers , that is , effective for repairing iridodialysis . the cobbler 's technique allows a maximally functional and cosmetic result for iridodialysis .

adult onset retinoblastoma is a rare intraocular malignancy that has been previously reported in the literature.12 many of the reported cases have been treated with enucleation due to the advanced nature of the disease . focal therapy and chemotherapy have also been attempted , but with poor outcomes.34 here we report a case of adult onset retinoblastoma , which initially responded very well to chemotherapy ; however , massive recurrence of the tumor necessitated enucleation of the eye . a 30-year - old female diagnosed elsewhere with choroidal melanoma with retinal detachment , presented to our emergency department with complaints of seeing black spot in front of the right eye accompanied with intermittent flashes for 20 days . on examination , the best - corrected visual acuity ( bcva ) was 6/9 in the right eye and 6/5 in the left eye . intraocular pressure measured with goldmann applanation tonometry was 11 and 15 mmhg in the right and left eye , respectively . fundus examination of the right eye revealed a large endophytic yellowish white , well - demarcated mass , with surface vascularity , located in the temporal quadrant extending up to the macula along with the presence of multiple subretinal yellowish infiltrates inferiorly [ figure 1a ] . there was overlying exudative retinal detachment involving the inferior portion of the retina reaching the macula . a provisional diagnosis of adult onset retinoblastoma with subretinal seeding and exudative retinal detachment was made . the patient underwent ocular ultrasound examination of the right eye which revealed a dome - shaped mass with homogenous internal structure , high surface reflectivity , and variable low to medium internal reflective echoes , with a basal circumference of 11.1 mm and height of 5.8 mm and the presence of an exudative detachment reaching up to the macula . magnetic resonance imaging ( mri ) of brain also revealed a retinoblastoma in the right eye with no evidence of extraocular or optic nerve invasion . the patient subsequently underwent six cycles of chemotherapy ( carboplatin , vincristine , and etoposide ) combined with transpupillary thermotherapy over a period of 6 months with regular monthly follow - up . after the third cycle , the tumor mass started showing excellent regression [ figure 1b ] . as some residual activity was present at the end of six cycles , she underwent two more cycles of chemotherapy and by the end of 8 months , the tumor had regressed completely leaving a flat scar [ figure 1c ] . the lesion was quiescent for a period of about 10 months . on follow - up , 10 months after the last treatment , bcva was 6/9 in the right eye and fundus examination revealed a regressed tumor with the presence of multiple small new tumor foci in the periphery [ figure 1d ] which were treated with laser using indirect ophthalmoscopy and transconjunctival cryotherapy . she was followed - up for a period of 2 years and in the event of new tumor foci [ figure 2a ] , she was given focal therapy ( either transpupillary thermotherapy or cryotherapy ) subsequently resulting in tumor regression [ figure 2b ] . after 2 years , at a follow - up visit , the bcva had decreased to 6/60 in the right eye and fundus examination revealed significant media haze due to massive vitreous seeding [ figure 2c ] and the presence of complicated cataract along with a substantial increase in the tumor size [ figure 2d ] . as the tumor showed further progression over the next 2 - 3 months , the patient was advised to undergo enucleation with ball implant with the aim of preventing further tumor spread and she agreed . histopathologic examination of the enucleated globe revealed a moderately differentiated retinoblastoma with choroidal invasion of more than 3 mm and presence of tumor cells in the anterior fibers of the sclera . , she was advised 6 cycles of adjuvant chemotherapy ; however , patient was not keen to undergo treatment and underwent just one cycle of chemotherapy and then discontinued treatment . on her last follow - up visit , 6 months later , the socket was healthy and the left eye remains normal . ( a ) presence of a large endophytic lesion with surface vascularity and overlying exudative retinal detachment and numerous subretinal infiltrates inferiorly . ( b ) significant resolution of the exudative retinal detachment with marked reduction in the tumor size , the vascularity and the inferior subretinal infiltrates following three cycles of chemotherapy . ( c ) complete regression of the tumor resulting in a scar at the end of eight cycles of chemotherapy . ( d ) presence of the scarred tumor along with small active tumor foci in the inferior and nasal periphery at 10-month follow - up after tumor regression color fundus photos of the right eye showing . ( a ) presence of larger peripheral active tumors along the old inactive scarred tumors . ( c ) significant media haze due to vitreous seeding and complicated cataract , with hazy view of the disc and the tumor . ( d ) large tumor masses in inferior periphery with extensive surface vascularity and exudative retinal detachment presence of calcification on ultrasonography and computerised tomography ( ct ) scan is not mandatory for diagnosis of retinoblastoma in adults as seen in the present case and also in other reports.4 the behavior of adult onset retinoblastoma is very similar to the childhood disease . enucleation is the primary modality of treatment in adults,15 mainly due to the large tumor size and the late presentation . though there have been reports of spontaneous regression6 as well as tumors showing initial response when treated with brachytherapy and or cryotherapy , they may recur34 eventually leading to enucleation . what was unique to our case was that it showed excellent response initially and had near normal vision and then failed therapy with reactivation necessitating enucleation . whether it is the genetic make - up of the adult onset retinoblastoma that makes it difficult to salvage the eye , attempts to salvage the globe in adult onset retinoblastoma with chemoreduction and focal therapy may be possible ; however , regular long - term follow - up is needed for potential recurrence , which mandate timely intervention .

adult onset retinoblastoma is a rare intraocular malignancy . the majority of the cases are treated with enucleation , due to late presentation and advanced - stage tumors . here we report a case of a 30-year - old female who presented with an intraocular mass with exudative retinal detachment in her right eye . b - scan ultrasound and magnetic resonance imaging ( mri ) confirmed the diagnosis of retinoblastoma . in an attempt to salvage the globe , she was treated with chemotherapy , which resulted in excellent regression of the tumor mass by the end of 8 months follow - up . the patient was followed - up regularly with focal treatment whenever necessary . two years later , she developed a massive recurrence necessitating enucleation . histopathologic examination revealed a moderately differentiated retinoblastoma with choroidal invasion . attempt to salvage the globe in adult onset retinoblastoma with chemoreduction and focal therapy may be possible ; however , regular long - term follow - up is necessary for recurrence which warrants timely intervention .

bidirectional ventricular tachycardia ( bvt ) is defined as a tachycardia showing beat - to - beat alternation in the qrs axis . the rate is typically between 140 and 180 bpm , with a frontal plane axis varying between 20 and 110. the most common causes of bvt include catecholaminergic polymorphic ventricular tachycardia and cardiac glycoside toxicity . other previously described etiologies include myocarditis , long qt syndrome type 7 , congenital cardiomyopathies , cardiac tumors , and acute cardiac allograft rejection . cardiac sarcoidosis is characterized by myocardial inflammation and interstitial fibrosis that can lead to slowed conduction and macro re - entrant arrhythmias . we report a case of bvt in a patient with cardiac sarcoidosis and briefly discuss the proposed mechanisms underlying bvt . a 73-year - old man with history of chronic pulmonary sarcoidosis was seen for an annual checkup , during which ventricular bigeminy was identified on a 12-lead electrocardiogram . subsequent holter monitor assessment showed multiple premature ventricular beats and several short runs of non - sustained ventricular tachycardia ( vt ) , with two different qrs morphologies ( fig . 1 ) . a cardiac magnetic resonance tomography with delayed gadolinium showed a curvilinear region of patchy mid - myocardial enhancement within the inferolateral left ventricular myocardium near the base , consistent with cardiac sarcoidosis ( fig . 2 ) . the left and right ventricular ejection fractions were 47% and 37% , respectively . a nuclear myocardial perfusion study using single - photon emission computed tomography showed no myocardial perfusion defects on stress or rest imaging , ruling out ischemia . a fasting 18-fluorodeoxyglucose positron emission tomography ( pet ) demonstrated increased uptake in the same area of the myocardium that had shown late gadolinium enhancement , consistent with active cardiac sarcoid ( fig . the patient was elected to have an implantable cardioverter defibrillator ( icd ) placed for prevention of sudden cardiac death . he had not experienced any vt episodes during the one - year follow - up . one of the proposed mechanisms of bvt include elevated intracellular calcium , causing delay after depolarization in anatomically separate parts of the conducting system . proposed that two separate foci , with different rate thresholds for delayed after depolarization induced ventricular bigeminy , were present in a rabbit model . this would effectively double the heart rate , increasing the overall ventricular rate above the second threshold . once this had developed , the two competing sites would simply alternate on a beat - to - beat basis . this is likely the mechanism underlying bvt observed with digitalis toxicity and catecholaminergic polymorphic vt . the other proposed mechanisms for bvt include an alternating bundle branch block related to bifocal automaticity and inscribed in opposite directions , or scar - mediated reentry around a circuit with two alternating exit sites . described a mechanism including retrograde conduction over the mid septal fascicular pathway , with alternating block in the left anterior or posterior fascicles , to explain polymorphic fascicular vt patterns . scarring is typically patchy , with a predilection for the basal septum , anterior wall , and perivalvular regions of the left ventricle . it may also be confluent , affecting the right ventricular epicardium or endocardium . in sarcoidosis , it is plausible that multiform or bidirectional premature ventricular contractions ( pvcs ) are due to multiple exits from the areas of inflammation and/or scarring . conduction disturbances in cardiac sarcoidosis are not uncommon , and often affect the his - purkinje system . in our case , the patient had an intraventricular conduction delay at baseline , with prolonged qrs complexes at relatively low atrial rates ( fig . the majority of the retrospective data suggest that immunosuppression reduces the burden of arrhythmias , especially in the early phases of the disease . previous case series have reported various success rates with vt ablation in patients with cardiac sarcoidosis , likely due to the small number of patients in each series , with varying degrees of disease burden . the most common circuit for vt in one report was reentry in the peritricuspid area , which can be safely ablated . in another study , abolishing all inducible tachycardias was not always feasible because of septal intramural circuits , extensive right ventricular scarring , or sites of origin in close proximity to the left anterior descending , the ramus intermedius arteries , or the para - hisian region , which prohibit safe ablation . in the current case , it is likely that the presence of a septal focus leads to alternate exits into the right or left ventricle which shows the observed electrocardiogram pattern . although the septal involvement was not visible by imaging until a year after diagnosis , it might have been present initially , but was microscopic in nature . this is consistent with previous autopsy studies that have shown a heterogeneous distribution of sarcoid granulomas in the myocardium . the recent expert consensus document is an excellent resource for management and risk stratification for cardiac sarcoidosis patients . icd implantation for primary prevention is commonly performed due to the high burden of vt events ( estimated incidence rate of 15% per year ) in patients with cardiac sarcoidosis . in the current case , icd implantation was recommended due to the high burden of nonsustained vt episodes , left and right ventricular dysfunction , and the presence of fibrosis and active inflammation by imaging studies .

a 73-year - old man with history of pulmonary sarcoidosis was found to have runs of non - sustained bidirectional ventricular tachycardia ( bvt ) with two different qrs morphologies on a holter monitor . cardiac magnetic resonance delayed gadolinium imaging revealed a region of patchy mid - myocardial enhancement within the left ventricular basal inferolateral myocardium . an 18-fluorodeoxyglucose positron emission tomography ( fdg - pet ) showed increased uptake in the same area , consistent with active sarcoid , with no septal involvement . follow - up fdg - pet one year later showed disease progression with new septal involvement . cardiac sarcoidosis , characterized by myocardial inflammation and interstitial fibrosis that can lead to conduction system disturbance and macro re - entrant arrhythmias , should be considered in differential diagnosis of bvt . bvt may indicate septal involvement with sarcoidosis before the lesions are large enough to be detected radiologically .

a 45year old woman was admitted to our hospital and surgery was planned for right staghorn calculus ( figure 1 ) . ultrasound showed multiple calculi in the right kidney ; 38 mm in the pelvis , 21 mm in the lower pole and 16 mm in the upper pole with moderate hydronephrosis . intravenous urography showed right renal staghorn calculus with delayed excretion suggestive of impaired right renal function . ray showing the burden of calculi in the right kidney . under general anesthesia , initial ureteric the procedure was carried out with the patient in prone position using a c arm and bull 's eye technique . lower pole puncture was done and the stones in the lower calyx , middle calyx and pelvis were cleared . an upper pole puncture was done under c arm guidance and bile was observed gushing out of the needle when the stiletto was removed . a 20 fr foley catheter was kept as a percutaneous nephrostomy tube and the patient was put on antibiotics according to urine culture and sensitivity along with metronidazole . in the immediate post operative period , the patient was closely monitored with parameters such as pulse rate , blood pressure , abdominal girth monitoring , water balance and nasogastric tube aspirate . the patient had two episodes of vomiting in the immediate post operative period and mild distension of the abdomen . an ultrasound was performed twenty four hours after surgery and revealed minimal collection in the gall bladder fossa ( < 10 ml ) with pelvic collection ( < 50 ml ) . a 72 hour scan was done which showed resolution of the gall bladder fossa collection . the patient had no signs of peritonitis and responded well to conservative line of management . she was discharged home on post operative day five and was followed up after two weeks with renal function test and liver function tests which were within normal limits . according to clavien dindo grading system for surgical complications this case fits into grade 2 . percutaneous nephrolithotomy is the treatment of choice for large ( > 2 cm ) renal staghorn calculi [ 1 , 2 ] . it constitutes a small number of all complicated visceral injuries during pcnl . till date , only six cases have been documented in literature . other organ injuries such as spleen , liver and colon have been documented , with gall bladder injuries being the least common . a well distended gall bladder is in close proximity to the right kidney and medial right sided percutaneous renal access may increase the risk of gallbladder injury . it is significant to bear in mind that gall bladder injury is not a common complication but is still commonly encountered in thin individuals . most of the cases in the literature have undergone immediate cholecystectomy . in our case , pcnl was not abandoned and the patient did not undergo cholecystectomy ; our case was instead managed conservatively . in the post operative period , close monitoring is required , both clinical and by means of ultrasound . if there is deterioration in the clinical scenario , then immediate cholecystectomy has to be done . in our case , the leak subsided and spontaneous closure of perforation occurred , most probably due to the use of a two piece diamond tip needle , which is supposed to be less traumatic as compared to other conventional needles .

percutaneous nephrolithotomy ( pcnl ) has been an excellent option for the management of kidney stones . there have been many complications in regards to solid organ injury during pcnl . here we discuss an interesting case of 45-year - old woman , who underwent pcnl for right renal staghorn calculus , and had an accidental puncture of the gall bladder . post operatively , the patient was conservatively managed and recovered well . a small number of cases has been reported until now in literature .

crohn 's disease , first described in 1922 , is characterized by segmental granulomatous inflammation of the intestinal tract that frequently involves the cutaneous tissues as well . cutaneous crohn 's disease ( ccd ) is synonymous with metastatic crohn 's disease ( mcd ) . a 20-year - old woman , mother of a 1.5-year - old child , presented with a swelling in the genitalia of 6 months duration . cutaneous examination revealed a single , non - tender , pedunculated , polypoid growth , about 6 7 cm , hanging from the left labium minus [ figure 1 ] . however , many scars were seen on the medial aspect of both the thighs , which were attributed to pruritic ulcers in childhood , details of which were unavailable . systemic examination , including the eyes and oral mucosa , did not reveal any abnormality . filariasis being endemic in cuddalore district of tamil nadu , elephantiasis of external genitalia due to filariasis , besides , lymphogranuloma venereum and granuloma inguinale were also considered . ( a ) single , non - tender , pedunculated , polypoid growth , about 6 7 cm , hanging from the left labium minus ( p ) and minimal swelling of the left labium majus , ( b ) multiple knife - cut ulcers on the external genitalia , in the inguino - crural fold , and in the interlabial creases a clinical diagnosis of soft fibroma was entertained and an excision biopsy of the nodule was undertaken . histolopathological examintion ( hpe ) revealed multiple non - caseating granulomas , edema , and dense lymphocytic infiltration in the dermis . multiple non - caseating granulomas in the dermis ( g ) ( a ) h and e , 20 ; ( b ) h and e , 40 a week later , at the time of suture removal , the patient presented with multiple typical knife - cut ulcers on the external genitalia , in the inguino - crural fold , interlabial creases , and natal cleft , leaving no doubt in the diagnosis of ccd [ figure 3 ] . biopsy from knife cut ulcers could not be carried out since the patient was not willing to undergo the procedure . she did not have any intestinal symptoms or pain abdomen at any time in the past or present . ulcers on the external genitalia in the inguino - crural fold , ( a ) interlabial creases , and ( b ) natal cleft on investigation , she was found to have hypochromic microcytic anemia ( hb : 10.5 gm / dl ) and mild neutrophilic leucocytosis . other relevant investigations including mantoux test , ultrasonogram of the abdomen , and chest radiograph were not contributory . both enzyme - linked immunoassay ( elisa ) test for hiv and vdrl test were negative . the patient was referred to a gastroenterologist for further work up , and no gastrointestinal involvement was reported . with this background , ciprofloxacin 500 mg bid . for 10 days in addition to oral prednisolone at 30 mg / day , which was gradually tapered over a period of 6 weeks . she reported after 2 weeks with ulcers showing signs of healing , and by 6 weeks , they resolved . however , she reappeared 2 years later with massive swellings of both the labia majora , more marked on the left labium majus . she gave a history of having had a full - term normal child , 3 months earlier , delivered by an elective lower segment caesarean section ( lscs ) in view of the edema of the labia majora [ figure 4 ] . ccd is relatively rare with fewer than 100 cases reported in the literature . two - thirds of the patients were women with a mean age of onset at 34.5 years , though our patient had lesions much earlier at 20 years of age . vulvar involvement in cd may be by virtue of contiguity as a direct extension of intestinal involvement or non - contiguous ( metastatic ) in which there is no connection between the vulva and the bowel . in a review by andreani et al . , 25% of vulvar cd did not have any intestinal involvement at the time of vulvar lesion . it is in these cases that making a correct diagnosis becomes difficult , as was the experience of the present authors , who also missed the correct clinical diagnosis initially . mucocutaneous manifestations occur in up to 44% of patients , but they do not necessarily mirror intestinal involvement akin to our patient , who did not have any intestinal symptoms at any time . no intestinal involvement was found in the present case , similar to that in other reports . skin manifestations in cd can be categorized as either histologically specific or non - specific ( reactive ) . specific lesions are characterized by non - caseating granulomas , similar to those seen in the intestinal lesions . nonspecific or reactive skin manifestations include entities like pyoderma gangrenosum , erythema nodosum , and oral aphthae . however , such non - specific manifestations were not observed in the present case . among patients with ccd , chronic edema leads to firm coalescing papules and fibrotic nodules , which could be mistaken for lymphatic obstruction . tuberculosis and filariasis , more common conditions in this part of south india , were also considered initially , before the biopsy report was available . the differential diagnosis of granulomatous vulvar lesions includes conditions like sarcoidosis , foreign body implantation , hidradenitis suppurativa , mycobacterial , or deep fungal infections and granuloma inguinale that were ruled out by the hpe and other relevant investigations . treatment of cd requires systemic administration of various antibiotics , immunosuppressive agents , and anti - inflammatory agents such as corticosteroids , azathioprene , sulfasalazine , or 6-mercaptopurine . infliximab and adalimumab , cyclosporine , and mycophenolate mofetil have been found beneficial by various authors in addition to surgical intervention for fistulae . she was advised frequent reviews by the gastroenterologist to help in early identification of gastrointestinal involvement and complications and its management , but she was lost to follow - up ; however , she resurfaced 2 years later with another child of 3 months age and no specific complications except for the massive swelling of the labia majora .

crohn 's disease , first described in 1922 , is characterized by segmental granulomatous inflammation of the intestinal tract and frequently involves the cutaneous tissues as well . cutaneous crohn 's disease ( ccd ) is synonymous with metastatic crohn 's disease ( msd ) . a case of ccd , without any gastrointestinal involvement is reported for its rarity .

nephrogenic adenoma is a rare lesion of the urinary bladder that may arise and induced by many inflammatory insults such as recurrent infections , recurrent renal stone , intravesical therapy , bladder diverticula , renal transplantation , foreign bodies , chemical agents , radiation therapy , and other chronic irritative factors . in 1954 , mostofi reported that the urinary bladder epithelium had the ability to transform into several morphologic types under appropriate stimulation and suggested that squamous and glandular metaplaisa of the urothelium is seen frequently in association with chronic infection . nephrogenic adenoma ( nephrogenic metaplasia ) shows a male predominance with a male to female ratio of 2:1 , and occurs over a wide age range ( 4 - 81 years ) . although most common in adults , approximately 10% of nephrogenic adenomas have been observed in children . in 1950 friedman and kuhlenbeck described eight such cases as nephrogenic adenoma resembling aberrant tubules of the kidney . the origin of the tumor is uncertain , and many believed that it might originate from embryonic mesonephroid tissue . the majority of reports indicate that this type of lesion is due to urothelial injury as a result of previous surgery or long - term inflammation . immunosuppressive therapy as in renal transplantation and intravesical drug ( bcg ) infusion are suspected causes in nephrogenic adenoma . the clinical and cystoscopic characteristics of nephrogenic adenoma are not diagnostic so cytomorphology , and immunohistochemistery study are needed to differentiate nephrogenic adenoma from malignant lesions , and to avoid erroneous therapeutic approach . nephrogenic adenoma is typically positive for cytokeratin 7 ( ck7 ) , -methylacyl coa racemase ( amacr ) ( p504s ) , pax2 and epithelial membrane antigen ( ema ) , and are usually negative for p63 , cytokeratin 20 ( ck20 ) , and prostatic specific antigen ( psa ) . a 55-year - old female was admitted to shahid faghihi hospital , shiraz , iran with chief complaint of irritative lower urinary tract symptoms and intermittent gross hematuria . four years ago she had developed renal stone , and had been subjected to percutaneous nephrolithotomy . her urine analysis showed red urine containing a large number of red and white blood cells , and few bacteria . cystoscopic examination revealed a sessile 33 centimeters lesion in the left lateral wall of bladder . microscopic examinations of hematoxilin eosin - stained slides showed irregular proliferation of small tubules , which were lined by single - layer low cuboidal epithelium in myxoid and inflammatory background in the lamina propria ( figure 1 ) . there were also some cord - like structures and single cell proliferation . the tissue sections deparaffinized and treated with 3% hydrogen peroxide and antigen retrieval was done . the slides were then stained with a polymer - based detection system ( dako`s envision system ) . immunohistochmistery study showed positive reactivity for p504 , cd10 , ema and ck7 ( figures 2 , 3 , 4 ) , but negative reactivity for psa , p63 and ck20 ( figures 5 , 6 ) . after five months follow up the patient showed decreased complaints , but she did not completely recovered and she did not come back for treatment either . nephrogenic adenoma is a rare bladder lesion presented with well - defined mass located mostly beneath the epithelium . in the past , it was believed that nephrogenic adenoma represented metaplasia of the urinary epithelium in response to inflammatory process . however , it has been demonstrated to result from urothelial shedding , and implant in injured area . adenocarcinoma of the bladder was reported to occur two year after nephrogenic adenoma in a 25-year - old man . few reports have examined the use of immunohistochemical findings in the diagnosis of nephrogenic adenoma . alsanjary et al . studied the morphological and immunohistochemical features for differential diagnosis of nephrogenic adenoma from clear cell adenocarcinoma . immunohistochemical study can differentiate nephrogenic adenoma from malignant process , and define the origin of adenoma . immunohistochmistery studies have shown that pax2 was positive only in remnant of fetal renal tubules and nephrogenic adenoma , and negative in malignant process such as prostatic adenocarcinoma . cytoplasmic staining for ck7 and absence of staining for psa is in favor of nephrogenic adenomaalpha - methylacyl - coa racemase ( amacr , p504s ) , which is the most useful marker for the diagnosis of prostatic adenocarcinoma , is detected in nephrogenic adenoma of urinary bladder . there are some problems in the differential diagnosis of nephrogenic adenoma from clear cell carcinoma of bladder , because it shows foci with tubular , cystic and papillary configuration , but no dysplastic changes . olivia and young reviewed 80 cases of nephrogenic adenoma , which showed a trend of male predominance . jalpota reported an extensive involvement of bladder by nephrogenic adenoma in patient with renal allograft transplant . nephrogenic adenoma is a benign metaplastic response to urothelial injury , and may mimic malignant process . in the present case bladder biopsy was done with high suspicion for malignant lesion . however , immunohisthochmical examination of the biopsy revealed positive findings for ck7 , ema , cd10 and amacr , and negative findings for psa , p63 and ck20 . the clinical and cystoscopic characteristics of nephrogenic adenoma are not diagnostic so cytomorphology , and immunohistochemistery study are needed to differentiate nephrogenic adenoma from malignant lesions and to avoid erroneous therapeutic approach . some cases of nephrogenic adenoma are associated with diagnostic difficulty using certain histologic features , since they may mimic some features of malignant lesions .

nephrogenic adenoma is a rare benign lesion of bladder that may be confused with malignant lesions . there is a strong relation with urinary tract irritation and intravesicle instrumentations . nephrogenic adenoma was initially thought to originate from urothelial metaplasia ; however , no solid proof is available . we present a case of 55-year - old lady with urinary problem . cystocopic examination showed a sessile mass , and biopsy revealed circumscribed proliferation of tubules , cysts , and papillae that were lined by low cuboidal to columnar epithelial cells . nephrogenic adenoma can be a significant diagnostic pitfall due to the presence of certain histological features such as the presence of enlarged nuclei with prominent nucleoli . immunohistochemistery study was strongly positive for ck7 , p504s , cd10 , and ema , but negative for ck20 , psa , and p63 .

gastrointestinal stromal tumors ( gists ) are the most common mesenchymal , nonepithelial tumors of the gastrointestinal tract in adults between 40 and 50 years of age [ 1 , 2 ] . as per seer analysis , only 1% of 1,458 gist cases are esophageal in origin , with an incidence of 5170% in the stomach ; 2536% in the small intestine ; 57% in the colon , rectum and appendix , and 13% in the esophagus [ 1 , 2 , 3 ] . they are generally small and asymptomatic , but occasionally can be large and produce dysphagia . a review of the literature revealed a case of a large esophageal gist presenting with dyspnea . however , here we report a rare case of a small esophageal gist in a patient with dyspnea who presented with a mediastinal mass on chest x - ray and was initially diagnosed by a pathologist as having leiomyosarcoma and referred for chemotherapy . 1 ) . a ct scan of the chest with contrast revealed a hypodense , soft tissue mass / lymphadenopathy in the posterior mediastinum measuring 2.0 3.0 cm , contiguous with the esophagus ( fig . 2 ) , suggestive of neoplastic etiology . further imaging with pet revealed a mediastinal soft tissue mass / lymph node and a standardized uptake value ( suvmax ) of 5.1 . the patient underwent a bronchoscopy and mediastinoscopy with fine - needle aspiration of the mass . pathology showed increased cellularity with moderate nuclear atypia and pleomorphism with a mitotic count of 23 mitoses/10 high - power fields . the ki67 index was 35% and , finally , the sample was reported as spindle cell well - differentiated leiomyosarcoma ( fig . the patient was referred to us for further management . given the possibility of a gist , thoracotomy findings showed the mass in the posterior mediastinum , which was dissected free from the trachea and inferior aspect of the aorta . six weeks after surgery , a repeated pet - ct scan showed no recurrence . on further follow - up gists are rare , accounting for 0.13.0% of all gastrointestinal neoplasms and 5.7% of sarcomas . initially , gist applied to neoplasms displaying only c - kit ( cd117 ) , but the diagnosis is based on histopathology and immunohistochemistry . a total of 95% of gists express kit or dog 1 and have mutations in kit or platelet - derived growth factor receptor , polypeptide . the kit - positive cells in abdominal soft tissues include mast cells in the wall of the gastrointestinal tract ; the interstitial cells of cajal ( intestinal pacemakers ) around the myenteric plexus were thought to be the origin of gists . gists typically present in adults 4050 years of age and predominantly in the stomach and intestine . a comprehensive review of 11 case reports ( table 1 ) and case series with esophageal gists showed that only 1 patient presented with dyspnea and a large posterior mediastinal mass ( 27 cm ) ; 3 further masses were detected on routine chest x - ray [ 3 , 4 ] . our patient presented with vague , progressive shortness of breath , with the unusual finding of a small mediastinal mass on chest x - ray . esophageal gists commonly present with dysphagia but bleeding , perforation , back pain , anorexia , regurgitation and weight loss have been reported . dyspnea with the finding of a small tumor on chest x - ray is rare . further , pet - ct can help to differentiate gists from sarcoma , but our case showed an suvmax of 5.1 which can misclassify it as sarcoma [ 3 , 8 ] . initial biopsy and testing created a diagnostic dilemma because immunohistochemistry did not include cd117 immunostaining and diagnostic imaging was inconclusive . we present this case to raise physician awareness of such a rare presentation , so that the possibility of gists is considered in these situations , and cd117 testing be done if sarcoma histology is obtained . surgery is the mainstay of treatment of localized gists ; targeted therapies like imatinib have shown overall survival benefit in high - risk patients after surgery [ 9 , 10 ] . and in unresectable and metastatic disease , it has been approved as primary treatment . the pdgfra mutation d842v , sporadic wild - type gists , mutations with succinate dehydrogenase or braf - mutated gists are unlikely to respond to imatinib . ongoing trials involve sorafenib , nilotinib , pazopanib , regorafenib and cediranib for advanced gists [ 11 , 12 , 13 , 14 , 15 ] . future trials with combined or sequential use of tyrosine kinase inhibitors with other medications and personalized therapy after tumor molecular subtyping are promising in the management of gists . our case report highlights the consideration of gists in the differential diagnosis of posterior mediastinal masses and emphasis on the necessity of cd117 staining in those situations which can alter the therapeutic and prognostic implication for the patient .

gastrointestinal stromal tumors ( gists ) are the most common mesenchymal tumors of the gastrointestinal tract and are predominant in the stomach and intestine but rare in the esophagus . here , we report a case of esophageal gist which presented as a mediastinal mass on chest x - ray and dyspnea . the case was initially diagnosed as leiomyosarcoma , which could create a diagnostic dilemma . therefore , recognizing this uncommon presentation as a mediastinal mass with esophageal gist is important in the differential diagnosis .

cutaneous hyperpigmentation is a recognized adverse effect of chronic minocycline use occurring in up to 50% of patients , . in a recent study performed at the mayo clinic , 54% of 291 patients receiving long - term minocycline suppression for orthopedic infections developed some degree of hyperpigmentation after a mean follow - up of 4.8 years . in this cohort , factors associated with minocycline - induced cutaneous hyperpigmentation ( mich ) include a history of vitamin d deficiency , presence of a shoulder prosthesis , noncirrhotic liver pathology , and use of a concurrent medication ( e.g. , calcium channel blocker ) also known to cause hyperpigmentation . mich is not associated with adverse clinical effects , and it is mostly cosmetic in nature . we herein present a rare case of extensive skin hyperpigmentation involving both lower extremities in a patient receiving long term minocycline . in june 2016 , a 76-year - old male with a past medical history significant for nephrolithiasis and diverticulitis , presented to the authors institution with extensive hyperpigmentation involving both lower extremities . he underwent a left total knee arthroplasty in 1988 at an outside institution and underwent revision surgery in 1992 . in 2001 , he underwent a second revision for fractured patella that was complicated by an infection with a coagulase - negative staphylococcus . he received six weeks of treatment with parenteral vancomycin followed by oral trimethoprim - sulfamethoxazole . operative cultures from the knee grew enterococcus sp . , prevotella sp , viridans group streptococcus , as well as candida parapsilosis . after completing antimicrobial treatment with vancomycin , ertapenem and fluconazole , he underwent reimplantation using a rotating hinged knee arthroplasty in september 2005 ( fig . since that time , the patient was maintained on oral minocycline chronic suppression . in 2010 he uses a brace as well as a cane to ambulate . at a follow - up in february 2012 when he was seen again in june 2016 , there was extensive blue - gray pigmentation in both cheeks and in the lower extremities ( fig . 2 ) , as well as sub - ungual blue - gray pigmentation in both hands ( fig . there are three types of pigmentation patterns that can result from taking minocycline for long periods of time . type i is a blue - gray pigmentation occurring around areas that were previously inflamed . type ii has the same appearance as type i and covers areas of normal skin such as the anterior shins , arms , and ankles . type iii is a muddy brown pigmentation usually occurring on areas of the skin that are exposed to the sun . the blue - gray pigmentation is due to the deposition of iron within the dermal macrophages . the diffuse hyperpigmentation seen in the patient presented in this case report includes both types i and ii . blue - gray pigmentation is clearly seen around the scar on the left knee from his knee replacement surgery ( type i ) . hyperpigmentation is also distinctly seen on the shins of both legs and around the ankles of the patient ( type ii ) . hyperpigmentation is predominantly seen with minocycline and less likely to occur with other tetracyclines such as doxycycline . minocycline is five times more lipophilic than doxycycline ; hence , central nervous system adverse events are more common with minocycline . although doxycycline may have a higher incidence of gastrointestinal upset and photosensitivity , minocycline has an increased likelihood of severe and permanent cosmetic adverse events and central nervous system adverse events . in the study of orthopedic patients on long - term minocycline suppression , these side effects are an addition to gastrointestinal adverse effects that can also occur with minocycline . in the absence of data to show that minocycline is superior to doxycycline for long - term suppression of infections including orthopedic infections , the authors propose that doxycycline be looked upon favorably when chronic use is indicated .

cutaneous hyperpigmentation is a recognized adverse effect of chronic minocycline use occurring in up to 50% of patients . in this report we present a rare case of extensive skin hyperpigmentation involving both lower extremities in a patient receiving long term minocycline . the patient was receiving minocycline as suppression for chronic prosthetic joint infection . risk factors associated with minocycline - induced cutaneous pigmentation ( mich ) will be reviewed .

the spanish reference laboratory for meningococci routinely receives meningococci isolated from sterile sites for serogrouping , serotyping , and serosubtyping . from january 1995 to november 2000 ( just before the new c conjugate vaccine was routinely introduced ) , the laboratory received 2,975 meningococcal strains to be characterized by serotyping and serosubtyping with monoclonal antibodies ( 8) . the b:2b : p1.2,5 and b:2b : p1.2 antigenic combinations were found in 18 isolates ( table 1 ) . all these strains were suspected of belonging to the a4 lineage and were fully characterized by pfge and mlst as described previously ( 2,11 ) ; results were compared with those obtained among the c:2b : p1.2,5 epidemic strains . two additional strains characterized as b:4:p1.2,5 were also included to determine if these antigenic combinations might be caused by similar genetics events . nr , pulse types not related to those found in c:2b : p1.2,5 strains . fifteen ( 83.3% ) meningococci showed sequence types identified as representative of the a4 clonal lineage ; this lineage also represents the genotype of the c:2b : p1.2,5 epidemic strain . the proportions of isolates belonging to the a4 clonal lineage were 75% and 85% , respectively , in both b:2b : p1.2 and b:2b : p1.2,5 strains . seven of these 15 meningococci characterized as serogroup b belonging to the a4 lineage were isolated from patients who had never been immunized with the a+c polysaccharide vaccine . three group b strains that were suspected by antigenic characterization of belonging to the a4 complex showed nonrelated sequence types ( table 1 ) . in a different study ( data not shown ) , most of the c:2b : p1.2,5 epidemic strains grouped in two closely related pattern profiles by pfge : pt7 and pt8 . table 1 shows the pfge pattern profiles of the b:2b : p1.2,5 strains . some strains showed minor pattern profiles already present among c:2b : p1.2,5 isolates ( pt1 , pt4 , and pt38 , all of them closely related to pt7 and pt8 ) . those strains showing pfge pattern profiles that we did not find among the c:2b : p1.2,5 epidemic strain belonged to lineages different than a4 ( table 1 ) . on the other hand , the b:4:p1.2,5 strains showed the same sequence type , st33 , associated with et5 . the frequency of the c:2b : p1.2,5 and b:2b : p1.2,5 meningococci of the a4 lineage is shown in table 2 . recombinant strains expressing serogroup b or c have been previously described as resulting from a capsule - switching genetic mechanism ( 9,10 ) . a similar event with w135 isolates has been recently described ( 12 ) . however , the relevance of this phenomenon has not been fully described . in our surveillance analysis , the group b strains of the a4 lineage appeared before the vaccination campaigns ; this finding differs from results of an analysis conducted in canada after a similar surveillance ( 10 ) . our findings show that those genetic variants are being produced in the meningococcal population at random and that a variant s appearance is not necessarily related to mass immunization campaigns . in fact , these group b meningococci belonging to the a4 lineage were also isolated in some of the regions that used the vaccine on a small scale ( 13 ) . however , the increased number of these b:2b : p1.2,5 strains from the a4 lineage during the study period might indicate a positive selection caused by mass immunization campaigns during 1996 and 1997 ( table 2 ) . seven ( 50% ) of these strains were isolated from patients who did not receive a+c vaccine , indicating that the individual immune status should not be a critical factor for developing meningococcal disease with these serogroup b strains of the a4 lineage rather than other clonal lineages . nasopharingeal competition might be an important factor in the spread of these group b strains , as has been suggested to explain the spread of serogroup b meningococci belonging to the et15 lineage ( 10 ) . theoretically , however , the c:2b strains of the a4 lineage and the b:2b meningococci also belonging to the a4 lineage should have a very similar genetic background with the exception of a locus in the capsular operon ( 9 ) . in fact , both types of strains showed not only the same or similar sequence type by mlst but also a very similar genetic profile by pfge ( data not shown ) . nevertheless , those strains with a group c polysaccharide capsule maintained a major epidemic even after a mass immunization campaign ( table 2 ) and even when these group c strains were not common in the carrier population ( 14 ) . whether a similar observation can be made for the serogroup b strains belonging to the a4 clonal lineage is not clear ; the small increase in these serogroup b strains does not appear to be linked with increased epidemic potential . thus , the capsular polysaccharide appears to be the only differing factor between these two types of meningococci . once again , the high number of serogroup b strains in asymptomatic carrier population ( 14 ) associated with a natural immunity , might partially explain the different epidemic potential of the two genotypes . however , the nature of the group c polysaccharide alone does not explain why c:2b : p1.2,5 strains were responsible for an important epidemic wave in spain in 19961997 . some other factors , such as the amount of polysaccharide , might explain differences in virulence ( 15 ) . two meningococci characterized as b:4:p1.2,5 were included to analyze if some other antigenic combinations might appear as result of different genetic events . this possibility was not confirmed in our study but should be more accurately analyzed in the future . in 2000 , a new increase in cases of group c meningococcal disease was detected in some regions of spain ( 6 ) . because of these data , spanish health authorities made the decision to include a new group c conjugate vaccine in the routine infant immunization schedule beginning in autumn 2000 . how the two different vaccines against group c meningococci ( polysaccharide and conjugate ) influence the selection of serogroup b belonging to the a4 lineage strains in spain that have had a capsular - switching event would be an interesting future topic of research .

a mass immunization campaign for 18-month to 19-year - olds was undertaken in spain in 19961997 because of an epidemic of serogroup c meningococcal disease associated with a c:2b : p1.2,5 strain belonging to the a4 lineage . surveillance for the capsule - switching phenomenon producing b:2b : p1.2,5 isolates was undertaken . of 2,975 meningococci characterized , b:2b : p1.2,5 and b:2b : p1.2 antigenic combinations were found in 18 isolates ; 15 meningococci were defined as serogroup b belonging to the a4 lineage .