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a 78-year - old caucasian woman presented with an ulcerated nodule in the right pretibial region ( figure 1 ) . the nodule measured 1 cm in diameter and was surrounded by an erythema . it had grown in a large pre - existing , congenital pigmented lesion that , according to the patient , existed since birth ( figure 2 ) . it extended over the right leg and the right side of the abdomen and the lower back , skipping the buttock and the back of the thigh , and crossed the midline ( figure 3 ) . close up it consisted of multiple dark - brown macules on a light brown , caf - au - lait - like background ( figure 4 ) . a punch biopsy of the nevus showed elongation of rete ridges and hyperpigmentation of basal keratinocytes but no substantial proliferation of melanocytes ( figure 5 ) . histopathology of the nodule showed a nodular melanoma with an invasion thickness of 4.5 mm ( figure 6 ) . on the basis of the clinical and pathologic findings we made the diagnosis of a melanoma arising in a giant nevus spilus maculosus malignant melanoma arising in association with a nevus spilus , also referred to as speckled lentiginous nevus , is rare . in german , kiebitzei - naevus , referring to the peculiar pattern of the nevus that resembles a lapwing s egg ( figure 7 ) . nevus spilus maculosus is flat and characterized by dark brown spots on a light brown background . in this type of nevus spilus the background pigmentation histopathologically corresponds to hyperpigmention of basal keratinocytes , and the dark spots are believed to be typified by junctional melanocytes arranged in nests . in the presented case a punch biopsy of the nevus did not show a proliferation of melanocytes , but the biopsy was relatively small and sampling error can not be excluded . papular nevus spilus , on the other hand , is characterized by dark brown papules or nodules on a light - brown background , although small dark spots may also be present . histopathologically , this type of nevus spilus shows the pattern of a superficial and deep congenital nevus with the presence of melanocytes in the papillary and reticular dermis . the malignant potential of the papular type appears to be lower than that of macular type of nevus spilus . it is possible that the two different types of nevus spilus originate from different mutations . this would also explain the dissimilar malignant potential of either type of nevus . as a possible differential diagnosis we also considered a partial unilateral lentiginosis ( pul ) , which seems to be a mosaic manifestation of type 1 neurofibromatosis . this rare condition was excluded on the basis of clinical findings because in pul the background is normal and not hyperpigmented and the body - midline is not crossed . in addition , our patient showed no signs of neurofibroma or caf - au - lait macules . phacomatosis spilorosea , a subtype of phacomatosis pigmentovascularis , could also be excluded because of the absence of telangiectasias . we excluded the speckled lentiginous nevus syndrome that is characterized by a speckled lentiginous nevus of the papular type and ipsilateral neurological abnormalities such as hyperhidrosis , muscular weakness and dysesthesia because such abnormalities were not present in our patient . this case of a melanoma arising in a nevus spilus maculosus suggests that this type of nevus should be regularly observed by a dermatologist and patients should be instructed to perform self - examinations to detect changes as early as possible .

melanoma arising in a nevus spilus is rare . there are two distinct types of nevus spilus characterized by macular or papular speckles , respectively . we report the case of a melanoma that arose in association with a giant nevus spilus maculosus .

, the reported rate of infection after disc surgery is between 1 % and 15 % ( 4 ) . infection can be disabling , it requires a long time treatment with bed rest and antibiotics , deformity , chronic pain , even death . it is well known that systemic antibiotics prophylaxis reduced the incidence of infection ( 5 ) . however , there is little evidence for the effect of the local irrigation of operative field with antibiotics at the end of procedure at the infection rate in herniated disc patients . this paper , based on the experience of a busy neurosurgical department is an attempt to contribute to the wide discussion on this matter . in this retrospective study we analyzed patients operated for lumbar discectomy , from 20092012 in our institution . during this period 604 patients were operated , including patients operated for the first time , those with recurrent disk disease . preparation for operation necessarily includes routine laboratory tests , ers and crp , lumbar radiography , spine ct and mri of the lumbar region . all of the patients , who were operated , did nt present with any signs of infectious focus elsewhere before surgery . a patient with epidural abscess after lumbar discectomy is excluded from our study because the resident has forgotten a part of the cotton in epidural space . we use amikacin sulfate injection diluted with saline solution for irrigation and systemic antibiotic cefazolin iv 2.0 , 30 min prior to surgery or 600 mg clindamycine in patients allergic to b - lactam antibiotics ( 15 patients ) . we stop the antibiotics after third dose in patients without drainage . in patients with drainage antibiotic the infection is judged by neurosurgeon during regular ambulatory visits , with laboratory analysis , white blood count , ers , crp , radiological imaging , mri for suspicious cases . a total of 604 patients , 285 ( 47.2% ) were females and 319 males ( 52.8% ) . gender of the patients in the study level of the herniation of the lumbar disc infection : 3 patients with infection of the wound , two male patients with superficial infection and 1 female diabetic patient with deep infection . all infected patients were smokers and did nt stop smoking even for short time . with the wound swab staphylococcus aureus is isolated to the three cases ; two patients had superficial wound infection and were treated with oral tablets , cefuroxime 500 mg 21 for 10 days , according to sensitivity with antibiograme , the patient with deep wound infection was treated with vankomycine amp . . the surgeon must be very vigilant to reduce their incidence as much as possible . according to the previous similar study in our institution with only systemic antibiotic prophylaxis , at that period the infection rate after lumbar discectomy was 2.7 % ( 1.6% discitis and 1.1% wound infections ) . results obtained in this study show us that the addition of local antibiotic is effective in reducing the rate of infection after lumbar discectomy compared with the rate of infection after prophylaxis with systemic antibiotics only . our results are similar to studies done worldwide regarding infections after lumbar discectomies 1 % -10 % ( 4 ) . the idea for choose amikacin sulfate was : local activity , effective against staphylococcus and gram negative bacteria , the most common agents of infections after these procedures . as in other studies diabetes and smoking was a risk factor for infection ( 8 , 9 ) , due to the impaired function of leukocyte , the angiopathy , and poor supply of oxygen to the tissues and poor penetration of the antibiotic in area . meticulous surgical work , good fascial closure , correct hemostasis , frequent space irrigation with saline , treatment of high blood sugar ( 8) , abstinence of smoking ( 9 ) , good nourishment ( 10 ) are very important factors which together with systemic and local antibiotic prophylaxis help to prevent infections after lumbar discectomies . our study showed that systemic antibiotic prophylaxis and addition of local antibiotic irrigation of space is effective in reducing the rate of infection after surgery for herniated lumbar disc compared with the rate of infection after prophylaxis with systemic antibiotics only .

introduction : reported rate of infections after lumbar discectomy is 1%15 % . this complication may result in disability or even the death.aimthe aim of the study is to assess the rate of infection associated with lumbar discectomies when combined systemic and local antibiotic prophylaxis was employed.patients and methods : in this retrospective study we analyzed all patients operated for herniated lumbar disc from 2009 -2012 in our institute . beside of receiving systemic prophylaxis with 2 g of cefazoline , all patients had their operative field irrigated at the end of operation with amikacin sulfate injection . wound was considered infected when local and systemic signs of infection were revealed and were associated with elevated esr , leukocytosis and elevated crp . assessment of infection is done by neurosurgeon during the hospitalization and later at outpatient s clinic along postoperative course of three months.results:a total of 604 patients were operated , of those 285 patients ( 47.2 % ) females and 319 males ( 52.8 % ) , 12 patients were operated on two levels ( 1.98 % ) . average patient age was 32.5 years ( range 2065 years ) localization of herniated disc was : in l/2-l/3 20 patients or 3.3 % , the l/3-l/4 level 42 patients or 7 % , the l/4 -l /5 262 patients or 43.3 % at the level l / v- s/1 280 patients or 46.3 % . three patients ( 0.49% ) developed wound infection , two of them superficial infection only with local signs : local pain , redness and leakage . they were treated with oral antibiotics . one with deep wound infection . he presented with local and systemic signs and treated with i.v antibiotics . all the cultures from wound swab revealed staphylococcus aureus.conclusion:prophylaxis with systemic antibiotic ( cefazoline 2.0 ) intravenous administration 30 minutes before the incision and irrigation of operative field with local antibiotic amikacine sulfate at the end of procedure reduces the infection rate in patients operated for herniated lumbar disc when compared with systemic antibiotic prophylaxis only .

since the advent of the hiv - aids pandemic across the globe , pneumocystis jiroveci pneumonia has been one of the most common defining illnesses of this condition . idiopathic cd4 lymphocytopenia is one such condition where the patient presents with such aids defining illnesses but with negative serological marker for this dreaded disease . here , we describe a case where a young man presented with pneumocystis infection but was having negative markers for hiv and thus the diagnosis of icl was concluded . a 32-year - old adequately nourished male was admitted for complaints of shortness of breath for 1 month which had exacerbated for the past 2 days . he used to work as a tailor with a mixed diet usually on his platter . examination revealed a well built and nourished male with a body mass index of 25.3 kg / m who was tachypneic with the presence of tachycardia and mild fever . oral thrush was present and scrapings were sent for fungal culture . there were no apparent risk factors for the presence of hiv or any other immunosuppressive states . chest x - ray revealed few scattered inspiratory crackles and the saturation monitor was showing a spo2 of 87% . high flow o2 was initiated and the patient was then shifted to the intensive care unit for further management . given the clinical picture of the patient , pneumocystis jiroveci pneumonia ( pjp ) was high on cards , given the apparent immunosuppressed state . empirical antibiotics , fluconazole and oral trimethoprim - sulphomethoxazole [ tmp - smx ] , was started along with steroids . u / l ( high ) and high - resolution computed tomography of the thorax revealed ground glass opacities in both lung fields , more so being perihilar . thus , the diagnosis of pjp was confirmed . to identify the cause of the immunosuppressed state , since the clinical suspicion of hiv was so high , a western blot and a hiv - rna polymerase chain reaction were ordered as well . cd4 and cd8 counts were ordered , as a suspicion for idiopathic cd4 lymphocytopenia ( icl ) was high . the cd8 counts were 312/mm ( normal ) with a cd4:cd8 ratio , inverted ( 0.35 ; normal : 1.41.7 ) . he denied any history of immunosuppressive drug intake , did not experience repeated infections in the childhood , his immunoglobulin and sugar levels were normal , and the titers for htlv1 and 2 were negative . thus , idiopathic cd4 lymphocytopenia was labeled as the cause of his immunodeficiency and was given a prophylaxis of tmp - smx and fluconazole post - treatment . the centre for disease control in the usa in 1993 defined a new entity in the ever expanding world of immunology , known as the idiopathic cd4 lymphocytopenia . the hiv - aids pandemic had just began to shape in those days and defining a disease , so close and yet so far from it , was a challenge that the centers for disease control and prevention had to face . they defined this entity as the presence of cd4 counts < 300/mm or < 20% of the total lymphocyte count on more than one occasion , at least 6 weeks apart , with the absence of hiv infection or any other condition that might cause cd4 cytopenia . icl is a heterogeneous condition which is usually diagnosed in middle age and has a slight male preponderance . the spectrum of the presence of opportunistic infections in icl is the same when compared to a patient with hiv infection . cryptococcosis is the most common infection associated with this disease , but candida , cytomegalovirus , nontuberculous , and tuberculous infections are common . icl appears to be due to a physiological response where there is an altered environment of cytokines and inflammation that leads to decreased production of t - cell precursors and clonogenic capacity of the bone marrow . this appears due to decreased interleukin-2 and increased tumor necrosis factor alpha in the internal mileu . in addition , decreased t - cell response and increased t - cell activation have been noted . one study had found that there is a profound defect in cxcr4 expression on cd4 cells and an abnormal intracellular accumulation of cxcr4 and its ligand cxcl12 . loss of this cxcr4 was postulated to cause disturbance in the priming of t - cells in the secondary lymphoid organs and its improper antigen reception and subsequent activation . barr virus , adenovirus , parvovirus b19 , and some other viral infections can cause lymphopenia in their acute stages , but it is highly unusual for them to cause an exclusive isolated cd4 cytopenia - like syndrome . there are not any published guidelines for managing these cases , and most centers advocate using a cd4 cell count level of below 200/mm to start antimicrobial prophylaxis . however , few case reports do suggest that icl may not be at an identical risk of infection as a patient with hiv at the same cd4 count , these data are too less to refute such claims and deny the patient of these measures . if the cd4 cytopenia is very low , i.e. below 100 and there is a presence of recalcitrant infections , then treatment with interleukin-2 is warranted ; however , this therapy has its own set of side effects and needs an informed written consent , depicting the investigational nature of the treatment protocol . hematopoietic stem cell transplant also has shown promise in some reports to be an effective mode of treatment for this condition . pjp is regarded as an aids - defining illness , but its presence in our patient without the serological evidence of hiv in any form leads us to investigate for this rare but important disorder . icl is a rare disease which warrants a high index of suspicion to be diagnosed . it can come with the same opportunistic infections as in a hiv patient , but the negative serology gives way the diagnosis .

idiopathic cd4 + lymphocytopenia ( icl ) is a rare disorder characterized by the presence of depleted cd4 cell line without the presence of hiv infection . slight male preponderance is noticed and is usually seen in the middle age group . opportunistic infections are the reason for their discovery and here we describe a case where a man was diagnosed as having pneumocystis jiroveci pneumonia and oral candidiasis .

total or partial sacrectomy is occasionally required to treat malignant tumors involving the sacrum , such as chordomas , osteosarcomas , and recurrent or invasive colorectal cancer . these radical surgical procedures result in a large pelvic defect that requires soft - tissue reconstruction . the defect is associated with a high risk of complications , such as perineal wound dehiscence , hernia , bowel obstruction , and abscess [ 2.3.4 ] . various techniques can be used to repair pelvic defect , including primary suture repair , myocutaneous flaps , and prosthetic mesh [ 1.5.6.7 ] . here , we report a case of chronic infection caused by small intestinal penetration 10 years after a sacrectomy and abdominosacral resection . we treated by performing subtotal removal of the mesh and reconstruction of the pelvic defect using the mesenteric leaf and omentum . she had undergone an s2-s5 sacrectomy and abdominosacral resection to treat sacral chordoma 10 years ago . at that time interim 10 years , she had occasionally suffered low - grade fever with an elevation of serum inflammatory markers . pelvic ct and mri showed fluid build - up , with free air around the dorsum of the mesh ( fig . 1 ) . this finding suggested an infectious process involving gas - producing bacteria , and/or a perforation of the intestine . the infection was not improved by administration of antibiotics ( sulbactam / cefoperazone ; 1 g / day for 1 day and meropenem ; 1.5 g / day for 3 days ) , and then , the operation was indicated . at the operation , we performed partial resection of small bowel and subtotal removal of the mesh , because of severe adhesion of a part of mesh with sacrum ( figs , 2 and 3 ) . post operatively , the patient s general condition is fair except for persistent infection of the pelvic cavity with a little plurent discharge from the surgical wound . enterobacteria , candida albicans , and methicillin - resistant staphylococcus aureus were detected in culture of the discharge . the patient was discharged 59 days after surgery , and returned to normal activity , although small amount of discharge from perineal wound was persisted for two years . sacrectomy is occasionally results in a large pelvic defect that requires soft - tissue reconstruction . the procedure can also injure the sacral nerves , resulting in bowel and bladder incontinence , weakness in the lower extremities and attenuation of the pelvic floor musculature , , , . to prevent such complications , many repair techniques have been reported , such as primary suture , repair using myocutaneous flap and prosthetic mesh reconstruction , , , , . in several studies , mesh reconstruction of the pelvic floor has been used to treat sacral hernia , , , , . importance is an onlay position of the mesh to avoid its direct contact with the small intestine , colon and rectum . in the present case , the patient had undergone an s2-s5 sacrectomy and abdominosacral resection to treat sacral chordoma . a polypropylene mesh ( marlex mesh ) was used to reconstruct the pelvic floor by peritoneal approach . a large defect in after the patient s first operation , interim 10 years , follow up mri showed fluid collection around the sacral defect . and she had occasionally suffered low - grade fever with an elevation of serum inflammatory markers . chronic inflammation of the surgical site ( including the mesh ) , was probably related to penetration of small intestine 10 years after the first operation . chronic mesh infections are difficult to treat using antibiotics with or without simple surgical drainage . in many cases , the infected mesh must be completely removed associated with debridment of the infected area , , . in our case , severe adhesion of a part of the mesh with sacrum resulted in incomplete removal of the mesh completely . , we used the mesenteric leaf and the omentum to repair the pelvic defect at the operation . this procedure has not been used as a usual procedure for pelvic reconstruction because of necessitates resection of the small intestine . but this procedure might be the best alternative , when we need to resect small intestine and the use of mesh have to be avoided like present case . to our best knowledge in english literature , this is the first report of operation for mesh infection after sacrectomy : the likely cause of infection was direct contact between the mesh and small intestine with resultant penetration . this case educates us , when the mesh and intestine are inevitably in contact , pelvic reconstructions should be performed using another procedure such as the myocutaneous flap or mesenteric leaf , not using a mesh . in conclusion , when a biomaterial mesh is used in surgery , surgeons should avoid direct contact between the mesh and abdominal organs such as the digestive tract . in this regard , mesentery leaf repair is a useful procedure by which pelvic defects can be reconstructed . written informed consent was obtained from the patients for publication of these case reports and accompanying images . this patient was properly informed and gave consent for her clinical information to be included in an elsevier publication . makoto koyama and yuta yamamoto and akira suzuki participated in preoperative , intraoperative , and postoperative care .

highlightsan 83-years old woman underwent sacrectomy combined with mesh reconstruction for chordoma suffered chronic mesh infection and bowel penetration 10 years after surgery.this is the first report of mesh infection after sacrectomy and its surgical strategy.we performed subtotal mesh resection and mesenteric leaf repair for pelvic reconstruction.mesentric leaf repair might be a useful procedure for pelvic reconstruction when the use of a mesh has to be avoided .

since 1938 , when the use of electro convulsive therapy ( ect ) was first described in the literature , it has played a major role in psychiatric medicine . also , since its introduction in the 1950 's , succinylcholine has remained the most common muscle relaxant used to modify the motor effects of ect . a coincidental association between neuroleptic malignant syndrome ( nms ) and malignant hyperthermia ( mh ) may render succinylcholine unsafe in some patients undergoing ect . a 69-year - old woman , of weight 50 kg , was admitted to the psychiatric service requiring anesthesia for ect to treat severe depression . she presented with a presumptive diagnosis of nms , parkinson 's disease , rheumatoid arthritis , celiac disease , and hypertension . she had an edentulous airway , with mallampatti score of ii , and had limited neck movement . magnetic resonance imaging ( mri ) showed moderate degenerative changes at c3/4 vertebrae and a normal odontoid peg . her heart rate was 80/min , blood pressure was 130/86 mm hg , and temperature was 36.5c . ect was administered using thymatron system iv ( somatics lake bluff , il , usa ) , with bitemporal electrode placement . induction of anesthesia was accomplished with propofol 80 mg and rocuronium 50 mg ( 1 mg / kg).the patient was hyperventilated by face mask with 100% o2 through a vapor - free datex - ohmeda anesthesia machine . three minutes after the administration of rocuronium and deep blockade confirmed with a neuromuscular monitor ( life -tech model ms iv ; mini stim ) , ect was performed . sugammadex 800 mg ( 16 mg/ kg ) was administered after ect approximately 5 min after the administration of rocuronium . recovery of train of four ( tof ) ratio to 0.9 was within 2 min and the time to first spontaneous breath was within 3 min from the administration of sugammadex . hr , bp , and temperature were measured before anesthesia induction , pre - seizure , post - seizure , and every minute for 10 min thereafter , and remained stable throughout the procedure . rocuronium and sugammadex were employed in all subsequent ects and found to be an excellent and safe alternative to succinylcholine in this patient . neuroleptic malignant syndrome is a relatively rare but potentially fatal complication of the use of neuroleptic drugs . nms has been associated with all dopamine blocking drugs such as antipsychotics ( phenothiazines , butyrophenones , thioxanthenes , benzamides , and recent drugs such as clozapine and risperidone ) and antiemetics ( metoclopramide , prochlorperazine , promethazine , and droperidol ) . nms pathophysiology is complex and probably involves an interplay between multiple central and systemic pathways and neurotransmitters . dopamine blockade in the hypothalamus is believed to contribute to thermoregulatory failure , and blockade in the nigrostriatal system may contribute to muscle rigidity and hypermetabolism . medial orbitofrontal circuit and the lateral orbitofrontal circuit likely contributes to changes in the mental status and catatonic features seen in nms . nms typically develops over a period of 24 - 78 hours following antipsychotic initiation , although the condition can occur at any time during treatment . the presence of all three major or two major and four minor manifestations indicates a high probability of nms . [ table 1 ] clinical criteria for diagnosis of nms nms is a self - limiting condition once the offending agent has been discontinued . general symptomatic treatment such as hydration , nutrition , reduction of fever , and treatment of secondary complications ( hypoxia , acidosis , renal failure ) are essential . muscle relaxants are often administered during ect to prevent myalgia and more serious musculoskeletal complications . succinylcholine remains the most commonly used muscle relaxant , but is not recommended in patients with a history of susceptibility to mh , nms , catatonic schizophrenia or organophosphate poisoning . there are clinical reports describing the use of other muscle relaxants in this high - risk group of patients . however , a dose - finding study reported that only a full intubating dose of mivacurium ( 0.2 mg / kg ) was associated with effective muscle relaxation during ect . at least two clinical studies have demonstrated that at higher doses , it causes clinically significant histamine release and hypotension . atracurium has been used as an alternative , but a dose of 0.5 mg / kg required more time to achieve a satisfactory tof ratio and recovery . rapacuronium at doses of 0.6 - 0.8 mg / kg provided effective muscle relaxation for ect and was readily reversible with edrophonium and atropine . frequent occurrence of bronchospasm led to its withdrawal from the market , so it is no longer a viable alternative . we submit that the combination of rocuronium and sugammadex offers a serious alternative to succinylcholine in patients with neuroleptic malignant syndrome for ect .

we report a case of presumptive neuroleptic malignant syndrome requiring muscle relaxation for electro - convulsive therapy . short acting muscle relaxation without the use of succinylcholine was achieved using rocvronivm reversed with the novel reversal agent sugammadex . we suggest that this combination is a safe and effective alternative to succinylcholine in such cases .

strongyloides stercoralis is a human intestinal nematode that is considered a common parasitic disease in tropical and subtropical areas . it is endemic in southeast asia , africa , west indies , south america , bangladesh , and pakistan . the ability to replicate in the human host permits autoinfection of s. stercoralis leading to chronic infection . hyperinfection occurs when the parasite load increases and the rhabditiform larvae penetrate the bowel mucosa and finally reaches the organs normally involved in pulmonary autoinfection cycle ( gi tract , peritoneum , and lungs ) . dissemination is defined as larva migrating to end organs not usually involving the normal life cycle of the parasite such as brain and skin . there are very few case reports of hyperinfection or disseminated s. stercoralis infection in immunocompetent patients . we present a case of hyperinfected s. stercoralis infection in an adult male patient presenting with respiratory symptoms . a 48-year - old farmer from eastern part of coastal india presented to the emergency department with complaints of low - grade fever for 15 days , cough with expectoration for 10 days , and gradually increasing breathlessness for 5 days . the patient was suffering from cough with expectoration and breathlessness on and off for the past 2 years . he was neither a diabetic nor a hypertensive . at the time of admission , the patient was confused , irritable , cyanosed , and tachypneic . his pulse rate was 120/min , blood pressure was 90/60 mm of hg , temperature was 101 f , respiratory rate was 30/min , and oxygen saturation was 78% . hb - 8.8 gm / dl , tlc - 10.8 10/cmm , dc - p 72% , l 22% , e 4% , and absolute eosinophil count - 480/cmm , tpc rbs was 96 mg / dl , bun - 28 mg / dl , serum creatinine - 1.2 , serum na - 128 mmol / l , serum k - 3.9 mmol / l . abg analysis revealed ph - 7.44 , paco2 32 mm hg , pao2 67 mm hg , bicarbonate 24 a provisional diagnosis of bronchopneumonia , and septic shock was made , and the patient was shifted to icu . blood and sputum were sent for aerobic culture along with sputum gram stain and zn stain before starting the antibiotic . chest x - ray at the time of admission revealed bilateral interstitial pattern of opacities [ figure 1 ] . on the 2 day , he developed ards and was put on mechanical ventilator . x - ray chest showing features of ards while examining the gram stain and zn stain smears of sputum , plenty of larvae were seen [ figure 2 ] . on wet mount , plenty of actively motile larvae of s. stercoralis were found [ figures 3 and 4 ; videos 1 and 2 ] . s. stercoralis rhabditiform larvae in sputum on zn staining s. stercoralis larvae on wet film observation of sputum sample motile rhabditiform larvae of s. stercoralis on wet film observation of sputum . rhabditiform larva of s. stercoralis in stool sample of patient the patient 's condition gradually deteriorated inspite of adding oral ivermectol and intravenous imipenam . s. stercoralis is a unique intestinal nematode causing chronic asymptomatic infection , hyperinfection , and dissemination due to its ability to produce autoinfection in the intestine and due to penetration of the perianal skin by the filariform larvae . hyperinfection and dissemination syndrome usually occur in immunocompromised patients includes those undergoing steroid therapy or chemotherapy , and those with hematologic malignancy , kidney and bone marrow transplant , hiv infection , and hypogammaglobulinemia . hyperinfection and dissemination syndrome has a very high mortality rate ( up to 87% ) . complications of hyperinfection and disseminated disease include bacterial and fungal infections , sepsis , and meningitis . secondary bacterial infection occurs because of the leakage of gut flora from an ulcerative bowel mucosa or as a result of bacteria carried on the surface of the larvae when they migrate into the host 's circulation . therefore , blood cultures commonly grow escherichia coli , klebsiella pneumoniae , proteus mirabilis , pseudomonas , and enterococcus fecalis . s. stercoralis hyperinfection has been documented in immunocompetent patients very rarely . a case has been documented from puducherry in an elderly male who presented with fever , cough , breathlessness , and pain abdomen who responded to oral ivermectol . a study from sgpgims documented 15 cases of strongyloidiasis ( 7 in immunocompromised and 8 in immunocompetent patients ) . but , eosinophilia is often absent in hyperinfection syndrome , as in our case . in conclusion , besides showing gastrointestinal symptoms , it may mimic acute exacerbation of underlying copd or new - onset asthma and be complicated with gram - negative bacteremia . therefore , stool and sputum examinations are important when the clinical picture is suspicious for s. stercoralis hyperinfection .

s. stercoralis infection is very common in south east asian countries including india . chronic infection is very common with symptoms of diarrhea , abdominal pain , nausea , vomiting , anemia , and cough . hyperinfection and dissemination usually occur in immunocompromised patients with symptoms mimicking asthma , copd , or aseptic meningitis . very few cases of hyperinfection and dissemination have been documented in immunocompetent patients . we report this case for its rarity and future references .

although approximately 200 cases of appendiceal intussusception have been reported in the literature , very few have ever been diagnosed preoperatively . we report a case of appendiceal intussusception secondary to endometriosis in an otherwise healthy female and review the literature regarding this rare finding . a 45-year - old female presented as an outpatient complaining of a several year history of rectal bleeding and intermittent right lower quadrant pain . she reported an association of her pain and rectal bleeding with her menstrual periods , and she stated that her symptoms were gradually worsening . an anorectal examination revealed external and internal hemorrhoids as well as a 1.5-cm pedunculated rectal polyp . a full colonoscopy was scheduled . at colonoscopy , 3 benign rectosigmoid polyps were removed . in the cecum , she had an appendiceal intussusception ( figure 1 ) . a computed tomograhic scan was ordered that did not reveal any evidence of intraabdominal disease . intraoperative findings showed an intussuscepted appendix ( figure 2 ) as well as multiple scattered small peritoneal and mesenteric deposits ( figure 3 ) consistent with endometriosis . final pathology of the specimen ( figure 4 ) showed extensive adherent endometriosis along the appendix and mesoappendix . the patient 's postoperative course was unremarkable , and 12 months after surgery , she is doing well and is without symptoms . . only tip of appendix was visualized secondary to intussusception into the cecum ( center ) . first reported in a 7-year - old boy in 1858 by mckidd , intussusception of the appendix is an extremely rare entity . approximately 200 cases of appendiceal intussusception have been reported in the surgical literature , but very few have been diagnosed preoperatively as in this case . in fact , less than 10 cases have been reported in which a preoperative diagnosis had been made ; ultrasound and barium enema were useful in diagnosing many of these , whereas diagnosis by colonoscopy has been noted in only a select few . although the cause of intussusception is unknown , the postulated mechanism for its occurrence is abnormal peristalsis provoked by local irritation . possible intrinsic causes of intussusception are varied and include foreign body , fecalith , polyp , carcinoid or other neoplasm , mucocele , crohn 's disease , parasites , lymphoid hyperplasia , or endometriosis . the appendix may be completely normal or may harbor a malignancy , endometriosis , or any of the other conditions listed . the appearance of intussusception may mimic that of acute appendicitis or , as in this case , may present as a constellation of vague and non - specific symptoms . radiologic studies may be helpful in diagnosis , but they are rarely diagnostic for intussusception , as supported by the majority of cases being diagnosed at laparotomy . spiral shell appearance of the appendix may be seen with abdominal ultrasound or contrast enema . computed tomography may also be useful in establishing a diagnosis by demonstrating a target appearance or concentric rings of intussuscepted appendix and cecum . absence of filling of the appendix with barium enema is suspicious but neither sensitive nor specific for intussusception . a classification scheme for appendiceal intussusception was proposed by mcswain and modified by langsam et al . a type i intussusception begins at the appendix tip that intussuscepts ( the intussusceptum ) into the more proximal appendix ( the intussuscipiens ) . in type 2 , type 3 intussusception occurs when the proximal appendix is the intussusceptum and invaginates into the distal portion of the appendix . a type 4 intussusception is a complete appendiceal inversion into the cecum with associated ileocecal intussusception . recognition of appendiceal intussusception is important in avoiding misdiagnosis and misguided attempts at endoscopic removal or inappropriate surgery . an appendiceal intussusception may be mistaken for a polyp or carcinoma , and failure to accurately diagnosis this condition has resulted in patients undergoing colonoscopic polypectomy with resultant perforation and peritonitis . alternatively , patients have also undergone unnecessary hemicolectomy when the intussuscepted appendix is regarded as a malignancy . although reduction of the intussuscepted appendix may occur via increased cecal luminal pressure from barium enema or colonoscopy , the definitive treatment requires surgical resection . as in this case , preoperative identification by colonoscopy facilitates selection of appropriate surgical management and ultimate treatment of this rare condition .

intussusception of the appendix is an extremely rare condition . although approximately 200 cases of appendiceal intussusception have been reported in the literature , very few have ever been diagnosed preoperatively . we report a case of appendiceal intussusception secondary to endometriosis in an otherwise healthy female . the case was diagnosed preoperatively by colonoscopy and treated surgically at laparoscopy . we review the literature of appendiceal intussusception and discuss the associated conditions , diagnosis , and a classification scheme for this unusual finding .

central neurogenic hyperventilation ( cnh ) is a rare condition and defined as a syndrome comprising normal or elevated arterial oxygen tension , decreased arterial carbon dioxide tension , and respiratory alkalosis with hyperventilation even during sleep . the diagnosis of cnh requires the exclusion of pulmonary , cardiac , and metabolic disorders that can result in hyperventilation . to date , 44 cases have been reported in the literature and majority of these cases were secondary to tumoral lesions involving the brainstem . we report here a 3-year - old male patient presented with asphyxia and diagnosed as cnh related to diffuse thalamic infarction . a 3-year - old boy , who was intubated , was admitted to the emergency care unit . his parents had found him unconscious and cyanotic in the garden and there was a mark of his rain - hood on his neck , which suggested suffocation . although there was no apparent lesion in the brain stem , bilateral diffuse thalamic , putaminal and globus palllideal lesions were detected on mri ( figures 1 and 2 ) . examination revealed tachypnea ( respiratory rate , 42/min ) , but other findings were normal . arterial blood gases ( abgs ) were ph , 7.52 ; paco2 , 29 mmhg ; and pao2 , 142 mmhg . laboratory studies disclosed the following values : hematocrit , 33.7% , white blood cell count , 10.610/l ; sodium , 140 meq / l ; potassium , 3.7 meq / l ; serum urea nitrogen ; 6 mg / dl ; creatinine , 0.21 mg/ dl ; and glucose , 110 mg / dl . his tachypnea could not be treated with analgesia ( fentanyl infusion ) and sedation ( midazolam infusion ) . on the 16th day of admission hyperventilation tracheostomy was performed and he was discharged from the intensive care unit in vegetative state with normal ventilation . it has been shown that in animal models , stimulation of the lateral parabrachial nucleus increased the respiratory rate . stimulation of respiratory control areas in the pons and medulla could explain cnh but it is not sufficient . it has been known that pontine respiratory group neurons modulate the respiratory rhythm . but animal models that disconnect the pontine respiratory group from the medulla have not resulted in cnh . elevated cns lactate levels could be a reason of hyperventilation by stimulation of medullary chemoreceptors . theoretically , hyperventilation could be seen in ictal - period during convulsions . nevertheless intermittent hyperventilation would be more likely explanation in these cases . no ictal or interictal discharges were determined on electroencephalogram of our case during hyperventilation . the thalamus plays a major role in regulating arousal , the level of awareness , and activity described chronic dyspnea and hyperventilation in a patient with subcortical infarcts in the medial thalamus bilaterally . these cases suggested a possible role for the thalamus in regulating ventilation in addition to the brainstem . based on these information s , we thought that thalamic lesion may be more likely causing to cnh in our patient . to date 44 cases were reported with cnh in the literature ( supplementary table s1 ) . thirty of 39 patients ( 77% ) had malignancies such as lymphoma , astrocytoma , glioblastoma and medulloblastoma . other three underlying pathologies of cnh were multiple sclerosis , encephalitis and anti n - metil - d - aspartat encephalitis . similar to our case one patient , who was 79 years old , had cnh due to thalamic infarction without brainstem involvement . described a male awake patient , who had chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids syndrome ( clippers ) and diagnosed cnh . in this report , a 3-year - old male patient , whom had bilateral diffuse thalamic lesion due to post - hypoxic damage resulted in cnh , was presented .

central neurogenic hyperventilation ( cnh ) is a rare clinical condition , whose mechanism is still unclear . here , we report a 3-year - old male patient , who had bilateral thalamic , putaminal and globus pallideal infarction resulted in cnh without brainstem involvement . this case may illustrate a possible role for the thalamus in regulating ventilation .

sacral neuromodulation ( snm ) is an accepted treatment for lower urinary tract dysfunction refractory to conservative treatment . it has also proven to be an effective treatment for fecal incontinence and functional bowel disorders . although no fda - approved indication exists , there are several reports showing a beneficial effect of snm treatment in chronic pelvic pain conditions , such as interstitial cystitis or vulvodynia [ 1 , 2 ] . the results seem promising , although the study populations are small and long - term success has not been defined yet . clitoral pain is an uncommon pain condition , and very little data exist about its prevalence . reported 21 cases of clitoral pain syndromes , including one patient with clitoral pain after a laparoscopic hysterectomy . their study showed that a wide variety of conditions are associated with clitoral pain syndromes ( trauma , surgery , neurological disease ) , although treatment was not discussed . we present a case of a patient with clitoral pain following hysterectomy , treated successfully with snm therapy . a 51-year - old woman presented to our urological clinic with persistent voiding symptoms , which developed shortly after an abdominal hysterectomy done 6 months earlier . the patient reported impaired bladder emptying requiring abdominal straining , followed by a sensation of incomplete emptying . she also complained of urinary incontinence occurring during day and night , without perceiving urgency . post - operatively , a hematoma of the vaginal apex developed , which was treated conservatively . in the acute stage , after 2 months , the pain became more prominent in the clitoral area , and paresthesia also developed . further relevant medical history noted two vaginal deliveries and a right - sided ovariectomy due to the presence of a mature teratoma of the ovary . there was no history of back injury or surgery , vaginal infections , sexually transmitted diseases , genital trauma , or sexual abuse . clinical neurological examination was unremarkable , including normal perineal sensation for touch and normal sacral reflexes . detrusor hypocontractility and inadequate sphincter relaxation was observed , with urethral pressures up to 130 cmh2o . post - micturition residual volume was 321 ml , with a voided volume of 65 ml . electrophysiological evaluation suggested the presence of a peripheral motor neuron lesion of the pudendal nerve . the bulbocavernosal reflex latency was increased to 54.8 ms ( normal range 2441 ms ) , and bladder bulbocavernosus reflex latency was 83.6 ms ( normal range 4779 ms ) . the latency of the pudendal somatosensory - evoked potential was normal with a normal shape of the evoked response . the total score of the hospital anxiety and depression scale was 16 , with scores of 10 on the anxiety subscale and 6 on the depression subscale . the initial treatment consisted of pelvic floor physiotherapy combined with clean intermittent self - catherization ( cic ) . this treatment facilitated voiding , resulting in reduced residual volumes below 100 ml , after which cic was discontinued . however , the complaints of urinary incontinence persisted , and therefore anticholinergics were added to the treatment . for the clitoral pain , amitriptylin was started but was only temporarily effective . additional treatment with pregabalin , lamotrigin , and carbamazepin gave partial relief of pain symptoms but was discontinued because of side effects . due to failure of conservative treatment of voiding symptoms , sacral neuromodulation was considered a possible treatment option . this procedure involved placement of a quadripolar lead into the third sacral foramen in order to stimulate the s3 nerve root . during the screening period of 3 weeks the test was considered successful if more than 50% was noted in any of the studied parameters . additionally , pain symptoms were recorded before ( baseline ) and during test stimulation using the visual analogue scale ( vas ) . evaluation of the voiding diaries showed less than 50% improvement in any of the relevant voiding diary parameters ( table 1 ) . the vas scores however showed a significant reduction in pain symptoms ( table 2 ) . although the test stimulation was considered unsuccessful regarding voiding symptoms , the marked decrease in pain as well as the high patient satisfaction served as guidance to offer the patient a permanent implantable neurostimulator . after extensive counseling and informed consent that snm is an off - label treatment for clitoral pain , she received an implant 3 weeks after the tined - lead test stimulation . table 1voiding diary parameters at baseline and during test stimulation . in the last column , the amount of improvement is shown as a percentage of the baseline dataparameterbaselinetest stimulationimprovement ( % ) voided volume / void ( ml)217263 + 36number of voids / day12.38.928number of leakages / day5.43.437number of pads / day1.21.011table 2overview of the vas pain scores filled out by the patient before and during snm treatmentvas score ( 0100)baselinetest stimulation6 months follow - uppain at the moment75150pain during last week70105pain in best period4000pain in worst period853510 voiding diary parameters at baseline and during test stimulation . in the last column , the amount of improvement is shown as a percentage of the baseline data overview of the vas pain scores filled out by the patient before and during snm treatment after 6 months , vas scores still showed a significant improvement ( table 2 ) in pain symptoms . the patient reported to be very satisfied with the treatment and had resumed her daily activities . no improvement in bladder function was noted : bladder emptying was still difficult and incontinence persisted . . repeated minor trauma of the pelvic floor and pudendal nerve may have accumulated due to the vaginal deliveries and pelvic surgery , leading to the measurable pudendal nerve lesion . snm shows to be beneficial in improving clitoral pain and the effect persists already for up to 6 months post - implantation . previous studies that evaluated the effect of snm on chronic idiopathic pelvic pain have shown positive results . siegel et al . reported a significant improvement in pain symptoms in six out of ten patients with pelvic pain , which persisted after a median follow - up of 19 months . yet , no previous studies evaluated the effect of snm treatment in clitoral pain . although snm was used as an off - label treatment for clitoral pain in our case , the results of this report suggest that the application of snm in patients with clitoral pain might be effective .

sacral neuromodulation ( snm ) may be beneficial in the treatment of patients with chronic pelvic pain , although it is not an fda - approved indication . we present a case of a 51-year - old patient that presented with symptoms of lower urinary tract dysfunction and clitoral pain after an abdominal hysterectomy . electrophysiological evaluation suggested a pudendal nerve lesion . after failure of conservative treatment , she was offered snm as a treatment for her voiding symptoms . during test stimulation , she experienced only moderate improvement in voiding symptoms , but a striking improvement in pain symptoms . she underwent a two - stage implantation of a neurostimulator with a successful outcome after 6 months follow - up . the results of this report suggest that snm may be effective in patients with neuropathic pelvic pain .

paget 's disease ( pd ) of the bone was first described , in 1877 , by sir james paget under the term osteitis deformans . it is characterized by rapid bone resorption and deposition , resulting in formation of numerous reversal lines , which give rise to a mosaic pattern in the lamellar bone with profuse local vascularity and fibrous tissue in the marrow . in the initial phase of pd however , both may occur simultaneously , resulting in osteoporosis and sclerotic bone . within the sclerotic bone there is reduced vascularity ( localized vascularity ) and the ability for normal healing is compromised , hence it can lead to osteonecrosis . therapeutic agents for pd include bisphosphonates like alendronate , risedronate , and zoledronic acid . a 70-year - old male patient presented with a 3-month history of perforation in the left maxillary region with drainage of oral fluids from the nasal cavity . extra - oral examination showed localized hyperthermia with enlarged cranium , zygoma , and incompetent lips [ figure 1 ] . intraoral examination showed enlarged maxilla , fistulous tract connecting posterior maxilla and maxillary sinus , and ill - fitting temporary partial denture in relation to left maxillary molars [ figure 2 ] . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . extra - oral clinical photograph shows enlarged cranium , zygoma , and incompetent lips ( arrows ) . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . pd of the bone is diagnosed primarily by radiographic examination . early in the course of the disease subsequently , areas of sclerosis develop leading to the characteristic appearance of mixed lytic and sclerotic areas , thickened trabeculae , bone expansion , cortical thickening , and deformity . generalized mixed radiolucent and radiopaque areas were seen in the maxilla and mandible with characteristic cotton wool appearance in the left maxilla [ figure 3 ] . paranasal sinus view revealed generalized increase in the fuzziness of all the facial bones with mixed radiolucencies in the frontal bones and marked haziness in the maxillary sinuses . lateral skull view showed generalized mixed radiolucent and radiopaque areas suggestive of pagetic calvaria [ figure 4 ] . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . panoramic radiograph shows generalized mixed radiolucent and radiopaque areas in the maxilla and mandible ( arrows ) with characteristic cotton wool appearance in the left maxilla . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . lateral skull view shows generalized mixed radiolucent and radiopaque areas ( arrows ) suggestive of pagetic calvaria . histopathology of the lesion showed increased osteoblasatic activity and hematoxyphilic reversal lines giving an appearance of the characteristic pagetoid bone . marrow spaces were filled with fibrous connective tissue , confirming osteitis deformans [ figure 5 ] . biochemical analysis showed abnormal increase ( 740 u / i ) in serum alkaline phosphatase enzyme level ( normal range is 40 - 125 u / l ) and urinary hydroxylproline ( 88 mg / day / m ) . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . hematoxylin and eosin stained biopsy tissue ( 100 ) shows immature bone either with osteoid and osteoblastic rimming , reversal lines , and enough fibrovascular stroma ( arrow ) . on correlating the clinical , radiographic , histopathological , and biochemical findings , it was finally diagnosed as a case of pd involving the maxilla and skull . an obturater was placed in the left maxillary ridge to prevent draining of oral fluids into the nasal cavity [ figure 6 ] . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . intraoral clinical photograph shows an obturator ( arrow ) placed in the left maxillary molar region . pd is a relatively common disease in western countries ; it is very rarely encountered in the indian population . it occurs mostly in the fifth to sixth decade of life , with a high preponderance in the male population . the etiology of pd is still not totally clear , but genetic and environmental factors may play a role . pd may affect the jaw bones , cementum of the teeth and pulp tissue , which lack cellular structure with manifestation of interglobular dentine . in our case altered size and shape of the maxilla , osteonecrosis that resulted in a fistulous tract , radiographically hypercementosis , displacement of the teeth , and cotton wool appearance in both the jaws were evident . non - healing extraction sites and exposed alveolar bone following the removal of maxillary teeth in a patient with advanced pd has been reported . a case of chronic osteomyelitis involving the maxilla following dental extractions in a pagetoid patient a total of 63 cases of bisphosphonate - induced osteonecrosis of the jaws were reported ; majority of the cases affected the maxilla . in the clinical scenario , the question posed is whether osteonecrosis in postoperative sites occurs as a result of pd , bisphosphonate therapy , or a combination of the two . in both , but , in our case there was no evidence of any non - healing socket and the patient had not used any medication to treat pd . the patient was referred to a physician for treatment of pd , who prescribed alendronate 40 mg / day for 6 months . drug induced osteonecrosis in paget 's disease is common . in the present case , osteonecrosis secondary to paget 's disease ( non - drug induced ) seen in a 70-year - old male patient shows characteristic radiographic and pathologic features of paget 's disease .

paget 's disease ( pd ) is a chronic progressive disease of the bone characterized by abnormal bone metabolism affecting either a single bone ( monostotic ) or many bones ( polyostotic ) with uncertain etiology . we report a case of pd in a 70-year - old male , which was initially identified as osteonecrosis of the maxilla . non - drug induced osteonecrosis in pd is rare and very few cases have been reported in the literature .

adenoid cystic carcinoma ( acc ) is a malignant tumor which develops in areas with secretory glands , most commonly in the head and neck region . the name cylindroma originated from the specific appearance of the tumor cells with cylindrical pseudolumina or pseudospaces . primary acc ( pacc ) of the skin is a rare tumor first described by boggio in 1975 , of which fewer than 70 cases have so far been studied in detail and reported in the english literature . pacc has a low potential for distant metastases but has a very aggressive infiltrative growth pattern , frequent perineural invasion , and a high risk of local recurrence after excision . the high risk of local recurrence after surgery suggests that postoperative radiotherapy should be administered . in 1991 , a 43-year - old woman presented to the dermatologist with a 10-year history of an enlarging nodule in the right axilla . in the previous couple of years , there were several episodes of inflammation . a wide excision was performed , and the histological result showed acc of the skin . macroscopically , the tumor nodule was solid and covered with connective tissue . in 1996 , radiotherapy was offered and performed with 44 gy administered . in 2000 , a local recurrence was suspected in the area of the previous surgical treatments , but postexcision histology showed connective tissue only . in 2006 and 2010 , excisions were made after local recurrences of tumor formation in the operative cicatrix , which were diagnosed as acc . all of the laboratory and imaging tests during the 24-year history of the disease were within normal ranges , and no metastatic disease was diagnosed . in march 2015 , once again , growth in the postoperative cicatrix in the right axilla appeared . 1 ) , with the tumor cells arranged in multiple variously sized lobules . they showed an overall cribriform appearance of the tumor ( fig . the chest ct scan showed no pulmonary metastases , and the patient was offered an active follow - up . pacc of the skin is a rare tumor with fewer than 70 cases reported in the english literature . it equally affects males and females , with a slight prevalence in females ( 54% ) [ 7 , 8 , 9 ] . in 44% of the cases , there was a documented local recurrence with a follow - up period of 58 months . metastases in other organs are rare and , in the event that they occur , the lung and lymph nodes are commonly affected sites . the reported patient presented with pacc of the skin , first diagnosed in 1991 , with four histologically confirmed local recurrences . there were no detailed case reports in the accessible literature on such series of local recurrences , only reports on a high risk of such recurrences . all four recurrences were surgically treated ; after the second one , a decision for radiotherapy was made , and the latter was administered at a dose of 44 gy . the last local recurrence we described was diagnosed in march 2015 and was treated only surgically . acc of the skin most often presents a treatment challenge due to the rarity of the lesion . surgical resection is a primary treatment , usually followed by radiotherapy ; chemotherapy is used in select cases . wide excision is the most common surgical technique , which was performed in the reported patient as well . xu et al . reported 7 cases treated with mohs micrographic surgery without local recurrences during the follow - up period . nevertheless , the authors admit that the limited number of cases and the insufficient follow - up period do not allow drawing a definitive conclusion on the superiority of mohs micrographic surgery over wide local excision . there are no studies in the literature indicating the best surgical approach , and there are no sufficient follow - up data in cases with multiple local recurrences either . again , because of the rarity of pacc of the skin , there are no studies in the literature about the efficacy of radiotherapy and chemotherapy . nevertheless , there are a few reports debating that radiotherapy in pacc of the skin can help in achieving locoregional control when given postoperatively . cisplatin - containing regimens ( cisplatin and 5-fluorouracil , cisplatin and anthracyclines , cisplatin and vinorelbine ) showed some response in isolated cases [ 12 , 13 , 14 ] . the reported patient presented with pacc of the skin , with multiple local recurrences treated with wide local excisions and radiotherapy , and without imaging evidence of metastatic disease . after the last local recurrence , the patient was offered an active follow - up , the decision being based on the long disease - free intervals between the local recurrences . surgery remains a possible future option for treatment , as the localization of the tumor allows further excision in case of recurrences . chemotherapy is not curative and could be reserved for metastatic disease , whereas radiotherapy could be used for future local control . the corresponding author acknowledges that he is responsible for complying with ethical requirements and declares that the patient was correctly informed and written informed consent was obtained ; the confidentiality of the patient was strictly preserved ; the patient was informed about the submission of the manuscript and will be acquainted when the article is published , and the principal of the complex cancer center , plovdiv , was informed and the manuscript was written according to the ethical codex of the institution .

primary adenoid cystic carcinoma ( pacc ) of the skin is a rare tumor with fewer than 70 cases studied in detail in the english literature . this type of tumor shows a prolonged course and a growth pattern usually manifested by multiple local recurrences and has a low potential for distant metastases . the most important modality for primary treatment is surgical resection followed by radiotherapy . we report a woman aged 43 years at the time of diagnosis , who presented with a slow - growing nodule in the right axilla without lymph node enlargement . a wide local excision was performed , and the histology revealed an adenoid cystic carcinoma . during the next 24 years , another four local recurrences were excised ( the last one in 2015 ) and confirmed histologically to be adenoid cystic carcinoma . the patient was given 44 gy of radiotherapy after the second surgery in 1996 . pacc of the skin is a rare tumor with insufficient data concerning the efficacy of the surgical technique and chemotherapy and radiotherapy treatment , even more so in the case of multiple recurrences . after the last recurrence , the patient was offered an active follow - up based on the long tumor - free intervals in the past and because the site of the primary tumor allowed further surgical excisions in future recurrences .

the ct perfusion ( ctp ) is an important modality in acute stroke imaging to delineate the infarct from ischemic penumbra especially when considering tissue plasminogen activators ( t - pa ) and endovascular intervention ( ei ) . it is a very sensitive measure of cerebral blood flow dynamics playing a vital role in diagnosing stroke mimics like seizures , migraine , tumors , abscess , hypoglycemia , and posterior reversible encephalopathy syndrome ( pres ) , when they present to emergency department as a stroke . even though intravenous t - pa is the standard of care in acute ischemic strokes , if given to nonstroke patients , they may contribute to morbidity . we present a case report where ct perfusion of the brain played a significant role in the diagnosis and management . he was seen by paramedics ( ems ) as obtunded , nonverbal , with left gaze deviation , and flaccid right extremity . there was no past medical history of seizures , but ems noticed an episode of seizure on the way to the hospital and intubated him for airway protection . the brain ct perfusion ( ctp ) showed [ figures 1 and 2 ] reduced time to peak ( ttp ) , increased cerebral blood flow ( cbf ) , and volume ( cbv ) . in ischemic stroke the core infarct would show increased time to peak ( ttp ) , reduced cerebral blood flow ( cbf ) , and volume ( cbv ) whereas penumbra would show increased time to peak ( ttp ) , reduced cerebral blood flow ( cbf ) but increased blood volume ( cbv ) [ table 1 ] . ct perfusion axial view at the level of the sylvian fissure demonstrate reduced ttp ( white arrows ) in the parietal - temporal lobe with corresponding increase in cerebral blood flow ( cbf ) ( white arrows ) and cerebral blood volume ( cbv ) ( white arrows ) suggestive of increased blood flow in these areas . mean transit time ( mtt ) , is a measure of the mean time for blood / contrast to perfuse a region of tissue . ct perfusion axial view at the cerebral high convexity level demonstrate reduced ttp ( white arrows ) in the parietal - temporal lobe with corresponding increase in cerebral blood flow ( cbf ) ( white arrows ) and cerebral blood volume ( cbv ) ( white arrows ) suggestive of increased blood flow in these regions . the ct perfusion is a quick , fast imaging modality which if incorporated in acute stroke imaging will shed light not only on the infarct size and penumbra but also delineate conditions mimicking stroke . when a patient presents with stroke - like symptoms and altered sensorium is one of the presenting complaints , the differential can be wide including intra - cerebral hemorrhage , large frontal infarcts , thalamic infarcts , infarcts involving the reticular activating system , hypoglycemia , postictal states , and other stroke mimics . acute post - stroke epilepsy is not a rare entity and can present with focal neurological deficits mimicking stroke . when the patient is obtunded , intubated , or in no position to participate in the clinical assessment , imaging plays a significant role . since the ictal focus has high metabolic demand due to excito - toxicity there will be hyperperfusion , which is manifested in the perfusion studies as above . plain ct head without the perfusion in the above scenario with the described neurological examination would have wrongly led to intravenous thrombolysis . however , limitations for ct perfusion studies are exposure to additional radiation ( also intravenous contrast ) , additional delay ( 15 minutes ) in care , and cost of the procedure . the brain ctp is a very sensitive imaging in acute strokes and can also play a pivotal role in diagnosis of stroke mimics . however , caution is advised in interpretation of perfusion images and needs to be done in conjunction with the clinical story rather than in isolation .

stroke mimics constitute 15 - -20% of all cases presenting to the emergency department , prominent among them being seizures , hypoglycemia , tumors , migraines , and posterior reversible leucoencephalopathy . they have also abnormal computerized tomography perfusion ( ctp ) findings . we report a case where ctp helped us to diagnose and treat a seizure when initial presentation was that of a stroke .

this case uniquely highlights the successful non - operative management of isolated traumatic gallbladder perforation in a patient that was considered to be a high risk operative candidate . a 64-year - old man with a background of alcoholic liver disease presented to our emergency department following high velocity blunt abdominal trauma . he had been driving at 60 miles / h when he lost control and car rolled - over . he was admitted under cardiology for further management of a fast narrow complex tachycardia , atrial fibrillation as this was the only outstanding issue . a transthoracic echocardiogram 2 days later revealed a right ventricular thrombus and he was therefore commenced on therapeutic low molecular weight heparin . on day eight post - trauma , this gentleman was referred to general surgeons for abdominal distension and mild pain . he was stable hemodynamically and clinically he was found to have a soft , distended abdomen , with localized upper peritonism . a repeat ct scan reported free fluid and a perforated gallbladder [ figure 1 ] . computed tomography showing gallbladder fundus perforation ( red arrow ) and free intraperitoneal fluid ( blue arrow ) in view of his comorbidities , after multidisciplinary discussion conservative management was agreed . an endoscopic retrograde cholangio - pancreatogram ( ercp ) with stent insertion was performed and a radiologically - guided intra - peritoneal drain inserted . endoscopic retrograde cholangio - pancreatogram showing common bile duct ( green arrow ) , cystic duct ( blue arrow ) and extravasation of contrast from perforated gallbladder ( red arrow ) over the next few days , the patient 's condition improved . the intra - peritoneal drain was removed at day 12 post ercp and the patient was discharged , uneventfully 35 days after the injury . a follow - up ercp 2 month later revealed a normal cholangiogram with no obvious leak and no filling defects . gallbladder injury secondary to blunt abdominal trauma is very rare , approximating 2% in the literature . the incidence of isolated gallbladder injury is even less as it tends to be protected anatomically by the liver , intestines , omentum , and rib cage . pre - disposing risk factors include recent alcohol consumption , a distended post - prandial gallbladder with a thin wall and malposition . traumatic gallbladder injuries usually present in three ways ; as an additional finding during the damage control surgery ( emergency laparotomy ) , with insidious onset of symptoms and signs a few days after the trauma and occasionally , a few weeks later with on - going non - specific abdominal complaints . laceration or perforation , which usually affect the gallbladder fundus , partial avulsions from the gallbladder fossa to near total traumatic cholecystectomy with or without disruption to the cystic duct and or cystic artery , and finally , contusions with an intramural hematoma contributing to wall necrosis and delayed perforation . losanoff and kjossev have formally previously categorized injuries to the gallbladder in an attempt to guide operative intervention and there is an evidence to suggest that this classification system has merit in dictating management . conservative measures such as cholecystostomy , percutaneous radiological intraperitoneal drainage or even cholecystorraphy have been reported , but found to be unsuccessful in the definitive control of gallbladder injury and invariably a cholecystectomy is required . others recommended an approach of diagnostic laparoscopy in equivocal trauma cases to identify gallbladder injury and once confirmed , proceed to laparoscopic cholecystectomy . furthermore , operative management is recommended in gallbladder injuries associated with additional varying degrees of extra - hepatic biliary ductal injuries ; either primary repair or duct to jejunum anastomosis with roux - en - y reconstruction . our patient was considered to be a high risk candidate for operation and we had to employ non - operative management , which was successful .

isolated gallbladder perforations following blunt abdominal trauma are very rare . they often present with insidious onset of symptoms a few days after the initial insult and an operative course of management ensues . this is in the form of a cholecystectomy and peritoneal lavage ; more often via laparotomy rather than laparoscopically . conservative management , in the form of cholecystostomy , percutaneous intraperitoneal drainage or cholecystorraphy has been described ; however , these cases have invariably resulted in cholecystectomy eventually . the case uniquely highlights the successful non - operative management of isolated traumatic gallbladder perforation .

a 5-year - old boy presented with a large 5 cm 5 cm cyst covering the left eye completely since birth [ fig . 1 ] . a written informed consent was taken from the father of the patient for publishing the clinical photographs . the visual acuity in the right eye was 20/20 with unremarkable anterior and posterior segment findings . the cyst was excised in toto and was sent for histopathological examination . during the surgery , the io muscle was found to have an abnormal origin [ fig . 2 ] . the patient having a cyst , measuring 5 cm 5 cm and covering the left eye completely since birth during surgery , the inferior oblique muscle was seen originating from the medial orbital wall , just posterior to the lacrimal bone and moving laterally , downward , and posterior from its origin the io muscle was originating from the medial orbital wall , 1012 mm behind the medial orbital margin , just posterior to the lacrimal bone and moving laterally , downward , and posteriorly from its origin making a more acute angle - around 20 to its site of origin [ fig . 2 ] . the muscle belly was grasped and pulled with toothed forceps to observe and confirm the primary action of io extortion . the insertion of the io to sclera was found to be at its normal site 12 mm behind the insertion of the lateral rectus near its inferior border . the ocular movements of the left eye were further tested 2 weeks after the surgery and were found to be normal in all directions . however , the child was slightly hypertrophic and amblyopic [ fig . 3 ] . the histopathological findings showed the orbital cyst to contain squamous epithelium , hair shafts , sebaceous glands , sweat glands , inflammation , respiratory epithelium , and intestinal epithelium . abnormal insertion of the io muscle has been reported previously . to the best of the authors knowledge , abnormal origin of the io muscle has not been reported in the literature to date . at 8.5 mm stage of the embryological development , a process arises from the ventrocaudal portion of the premandibular mesodermal condensation and develops into the inferior rectus , io , and medial rectus muscles . the io and inferior rectus muscles are separated from their point of junction by 12.5 mm stage and grow in two different directions to their point of origin and insertion . the cranioventral extremity of the io muscle reaches the orbital surface of maxilla whereas the caudodorsal extremity is inserted just medial to the insertion of lateral rectus muscle , shifting backward to its normal position later . the io muscle is the shortest extraocular muscle measuring 37 mm in length and has an orbital and an ocular surface . the muscle originates from the orbital floor formed by the orbital plate of the maxilla , 5 mm posterior to the inferior orbital rim . the point of origin coincides with a line extending from the supraorbital notch to the infraorbital foramen . the muscle moves backward , upward , and laterally to insert in the external and posterior aspect of sclera , under the inferior border of the lateral rectus muscle , through a short tendon ( 12 mm ) after passing between the floor of the orbit and inferior rectus muscle . these irregularities include congenital isolated absence , anterior and nasal transpositions , and abnormal insertions . in the current case , the io muscle originated from the orbital plate of the ethmoid bone instead of originating from the orbital plate of the maxilla [ fig . 2 ] . the authors propose that a mechanical obstruction ; the dermoid cyst in the current case at around 12.5 mm stage of the embryological development forced the io to alter its route toward its unusual site of origin as it separated from the inferior rectus muscle to proceed toward its usual site of origin and insertion . periorbital dermoids develop between 3 and 5 weeks of gestation along the zygomaticofrontal suture whereas nasal cysts develop around 8 weeks of gestation within the prenasal space as the nasal capsule ossifies . the authors believe that as the io muscle separated from the inferior rectus muscle to proceed toward its usual site of origin and insertion at 12.5 mm stage ( 7 week of gestation ) , the periorbital dermoid cyst , already started developing during the 3 and 5 week of gestation , in the current case , obstructed the normal course and forced the io to move toward the medial wall of the orbit instead of the orbital floor . the periorbital cyst in the current case was excised in toto . the histological contents of the dermoid cyst in the current case shared the contents of the orbital cysts reported by shields et al . and to the best of authors knowledge , this is the first case of orbital dermoid cyst presumably causing an extraocular muscle to change its site of origin during embryological development . developmental anomalies of extraocular muscles influence the ocular motility and may cause varying degrees of ocular deviation . however , in the current case , no limitation of ocular movements was observed after the removal of the dermoid cyst . this case report provides useful information not only to ophthalmologists but also to neurosurgeons , anatomists , plastic surgeons , and oral and maxillofacial surgeons by introducing a new landmark for the origin of io muscle . this new landmark must be kept in mind while operating in the orbital region to avoid inadvertent damage to the io muscle . the case report also invites research into possible role of mechanical factors such as dermoid cysts in etiopathogenesis of congenital anomalies in the topographic anatomy . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed .

a 5-year - old boy presented with a large 5 cm 5 cm cyst covering the left eye completely since birth . the cyst was excised in toto and was sent for histopathological examination . during the surgery , the inferior oblique ( io ) muscle was seen originating from medial orbital wall , 1012 mm behind the medial orbital margin , just posterior to the lacrimal bone and moving laterally , downward , and posteriorly from its origin making a more acute angle - around 20 to its site of origin . the insertion of the io to sclera was at its normal site . the abnormal origin of io was confirmed later by magnetic resonance imaging . the ocular movements of the left eye were tested 2 weeks after the surgery and were found to be normal in all directions . however , the child was hypertrophic and amblyopic . the histopathological findings showed the orbital cyst to contain dermal elements , respiratory , and intestinal epithelium .

neuroleptic malignant syndrome ( nms ) is a potentially life - threatening adverse reaction to antipsychotic drugs . neuroleptic malignant syndrome is uncommon with the use of atypical antipsychotics like olanzapine . in the past few years there has been increased use of atypical antipsychotic drugs . a case of nms related to olanzapine therapy was reported in a young male ; thereby drawing the attention towards adverse events occurring with the use of atypical antipsychotics . an 18 year old male presented with four years duration of bipolar affective disorder ( bpad ) . his illness started with manic symptoms characterized by decreased need for sleep and rest , over activity , over cheerfulness , over planning and over spending . in the initial two years of his illness , he had multiple episodes of mania , each lasting for three weeks , after which he would recover spontaneously . in between he had a few depressive episodes when he would complain of poor sleep and appetite , lethargy , sad mood , and finding no interest in any activity . during the last one year of his illness , the patient sought treatment from various medical facilities but did not continue any long - term management . during his last episode of mania , the treating psychiatrist prescribed olanzapine 10 mg / day , aripiprazole 15 mg bd , and sertraline 100 mg / day . after one week , the patient reported tremors when aripiprazole was stopped and olanzapine was increased to 20 mg / day along with sertraline 100 mg / day . the patient reported increase in tremors after two days and in next week he developed rigidity in all the four limbs associated with mutism , at which time the patient stopped all medications and presented to us with altered consciousness and no responsiveness to questions or commands . the patient was diagnosed with neuroleptic malignant syndrome , and his serum cpk at 1622 iu / l along with the characteristic symptoms and signs confirmed the diagnosis . he was started with bromocriptine 7.5 mg / day in divided doses , which was later increased to 15 mg / day with tab lorazepam 4 mg / day . the patient improved only minimally , and levodopa 125 mg three times a day was added the rigidity also reduced markedly and he started walking . during routine chest x - ray , the patient was referred to chest and tuberculosis hospital . at the time of discharge he was ambulatory , had adequate oral intake and serum cpk had reduced to 106 he was discharged with an advice to continue levodopa 125 mg , three times a day , bromocriptine 5 mg / day and lorazepam 2 mg / day and was asked to follow up in the out - patient department after one week with a plan to start prophylaxis for bpad during follow up . however , this case illustrates that nms can also occur due to treatment with atypical antipsychotic medications ( like olanzapine ) . the patient also had tubercular infection of chest , but patient 's symptomatology could not be explained on the basis of this . nms is more common in affective illness and young male , which is also the risk factor in our case . a rapid loading of antipsychotics is considered to be the causal factor in the development of nms by causing a sudden and massive down - regulation of dopaminergic transmission . the same mechanism seems to have worked in our patient too as there was temporal co - relation between increased dose of olanzapine to 20 mg / day and onset of features suggestive of neuroleptic malignant syndrome . we have assessed the causality of this adverse reaction by the who - umc causality assessment system and naranjo 's assessment scale . in this case , there is clinical features and laboratory test abnormality ( serum cpk at 1622 the symptoms could not be explained on the basis of other disease or drugs . and considering the typical clinical picture and laboratory abnormalities of nms , rechallenge with the drug was not required . so as per the who - umc causality assessment system the adverse event is as the reaction was life threatening and led to prolonged hospitalisation and required intensive medical care , it is classified as severe. neuroleptic malignant syndrome can occur with atypical antipsychotics like olanzapine especially in the presence of risk factors . nms remains a dangerous condition and has also been described in non - psychiatric settings .

an 18 year old male diagnosed as a case of bipolar affective disorder ( bpad ) , developed neuroleptic malignant syndrome ( nms ) following treatment with olanzapine ( 20 mg per day ) , an atypical antipsychotic drug . nms is usually seen with typical antipsychotic drugs . the patient was diagnosed as a case of nms , offending agent was immediately withdrawn and prompt treatment with bromocriptine and levodopa produced a good recovery . the various features of the case are discussed in view of the potential mortality of the syndrome .

caud is classified into saccular variety and globular variety , the former being more common than the latter . the cause and effect relationship between anterior urethral diverticulum ( aud ) and anterior urethral valve ( auv ) has been extensively debated . some authors do not distinguish between aud and auv , while others believe these are different entity . the diverticulum that is associated with auv is not a true diverticulum because in aud an acute angle is formed between the proximal part of dilated portion and the ventral floor whereas this acute angle is not present in auv . the diverticulum and valve causes obstruction of male urethra and patient complaints of dysuria , dribbling of urine , recurrent urinary tract infection , or a fluctuant ventral penile swelling . the treatment options in aud include endoscopic division of distal lip of diverticulum , excision of diverticulum with primary repair , marsupialisation with staged urethroplasty , or even suprapubic diversion followed by definitive repair . in this paper , we are presenting our experience of two cases of aud who were managed by excision of diverticulum with primary repair . moreover , out of these two cases , in one patient , the site of diverticulum was unique because it was just proximal to corona which is a very rare site for its development . a 12-year - old male presented with complaints of poor urinary stream and a swelling on the ventral aspect of penile urethra ( figure 1 ) . swelling was a soft cystic , fluctuant , and compressible , and it collapsed completely on manual pressure with urine coming out per urethra . urine analysis , routine blood counts and blood urea , and serum creatinine were normal . micturating urethrogram was done , and it showed the presence of a wide - mouthed diverticulum in distal penile urethra and the proximal lip of diverticulum forming an acute angle with normal caliber proximal urethra and normal urinary bladder without any vesicoureteric reflux ( figure 2 ) . the diverticulum was opened by incision on the ventral aspect of penile shaft , and its distal lip was widened by abutting a triangular flap taken from one half of the diverticular wall , and the double breasting of urethral suture line was also done using the other half of diverticular wall after scraping its mucosa . postoperative recovery was uneventful . a six - month - male child presented with poor urinary stream and a cystic swelling at the penoscrotal junction ( figure 3 ) . on investigations , the patient was managed by open diverticulectomy along with the double breasting of urethral suture line . in the postoperative period , the patient had normal urination without any swelling in the penile urethra and is on regular followup . congenital anterior urethral diverticulum ( caud ) may be found all along the anterior urethra but is usually located between the bulbous and the midpenile part . it is rare for aud to be in distal urethra near the coronal level as in the present case1 of this paper . various proposed hypotheses include a development defect of corpus spongiosum , cystic dilatation of the urethral glands , and sequestration of an epithelial nest after closure of the urethral folds . with a lack of a corpus spongiosum , suter proposed the theory that a diverticulum of the urethra develops because of epidermal pockets communicating with the ventral urethral wall . as the anterior urethral tube forms , the urethral groove may leave behind epithelial cells that form a congenital cyst . cysts in this region developing a communication with the urethra could lead to diverticulum formation as a result of the spontaneous rupture of the cyst into the urethral lumen . most children with this condition present with difficulty in micturition , dribbling of urine , poor urinary stream , or urinary tract infection . a careful history will reveal that the child never had a good urinary stream since birth , and a tell - tale sign is a cystic swelling at the penile urethra . if it is uninfected and without complication , the mass is unattached to the overlying skin , nontender , and mobile laterally . on compression , urine will be seen dribbling out of the external meatus , and the swelling is seen to empty . mcug has the additional advantage of demonstrating proximal changes like megacystis , vur , or other associated anomaly . ultrasonography ( usg ) is complimentary to the contrast studies to diagnose the condition and offers the additional advantage of evaluating the upper tracts as well . moreover , voiding usg has been found to be alternative to the contrast studies in making a diagnosis of aud . a diverticulum typically appears as outpouching from the ventral wall of the urethra and has a proximal and distal rim . the primary differential diagnostic conditions of aud include anterior urethral valve ( auv ) , dilated cowper 's gland ducts , and posttraumatic diverticulum . the presence of a penile or penoscrotal mass clinically and the proximal lip radiologically which is seen as an arcuate filling defect should readily distinguish the diverticulum from the valve . in addition , the proximal lip forms an acute angle with the normal caliber proximal urethra in aud , whilst in auv , it forms an obtuse angle . in dilated cowper 's gland ducts , a tubular channel is seen in the ventral surface of the bulbar urethra which it parallels , and its termination is in the urogenital diaphragm . treatment of aud depends on the size of the diverticulum and the degree of obstruction . transurethral resection ( tur ) with a paediatric resectoscope is the treatment of choice for small , well - supported diverticula wherein the distal obstructing lip is resected . moreover , successful treatment of aud has also been reported by using a sachse knife . but in the large diverticula , as also in our cases , open diverticulectomy and primary repair is recommended . we have used the technique of making a triangular flap which is fitted into the distal lip and double breasting of the urethral suture line , as described in literature . some authors have also advocated the placation of redundant diverticular wall with good results . in situations where there are back - pressure changes of upper tracts with deranged renal function , urinary diversion either by marsupialisation of diverticulum or even suprapubic to summarise , in patients of aud with large diverticula without any back - pressure changes , as in the present paper , open diverticulectomy with primary repair is recommended as this procedure carries good results , and it takes care of the redundant diverticular wall .

congenital anterior urethral diverticulum ( caud ) is an uncommon condition in children . we describe 2 patients of caud who presented with ventral penile swelling ; in one , the site of swelling was just proximal to corona which is quite rare . the diagnosis was made on usg and mcu . both patients had normal renal function . open diverticulectomy and primary repair was done in both patients .

giant inguinal hernias ( gih ) are rarely encountered in high income countries ; however , in low resource setting such conditions present more frequently often do to a lack of patient knowledge , trust in the health system , or funds to pay for treatment . additionally , in low income countries , resources may preclude management that is the hallmark of more resourced environments where a pneumoperitoneum is used to increase the capacity of the abdominal cavity and a mesh is used for definitive repair ( 1,2 ) . in sierra leone , a small west - african country with a population of 6 million and only 10 surgeons ( 3 ) , the presentation of a gih is less of a surprise . a noted aphorism is that in africa there are only two types of hernias : above the knee and below the knee . the reality in clinical practice , however , where there are many cases of incarcerated and sliding hernias , is that true gih , defined as a sac with a capacity greater than 2000 ml , are fortunately rare . however , when such cases appear , surgeons in these locations are faced with the problems of how to safely treat a patient with their limited resources . a 55 year old male with a right gih was referred to a tertiary care hospital in sierra leone from a smaller district hospital . the patient was known to have had the hernia since childhood but it had grown large over the years with excoriation of the skin at the apex of the hernia resulting in a painful , foul smelling ulcer measuring 4 x 6 cm . the hernia reached to the patient s knee while in the upright position and his penis was invaginated into the enlarged scrotal area ( figure 1 ) . a smaller left inguinal hernia was also noted . pre - operative counseling was given regarding the planned three stage repair that would be accomplished over a number of months . stage i , a 2 hour operation under spinal anesthesia , resulted in the excision of the ulcerated skin and partial replacement of the hernia contents into the abdomen as much as possible without compromising the patients venous return and breathing ( figure 2 ) . two months later , after the wound had healed , stage ii , a second 2 hours operation under spinal anesthesia , reduced the hernia by an additional 10 cm , again careful not to cause any respiratory compromise or abdominal compartment syndrome and the excessive scrotal skin was removed to maintain the hitched up level of the hernia ( figure 3 ) . the patient was advised to rest as much as possible and not to undertake any weight - bearing activities , to allow the abdominal muscles to relax and accommodate well the additional abdominal contents . giant inguinal hernia after stage i : resection of ulcer and surrounding scrotal skin , partial reduction of hernia sac contents . giant inguinal hernia after stage ii : partial reduction of hernia sac contents and resection of scrotal skin five months later , stage iii lasted 7.5 hours and commenced under spinal anesthesia and ended with general anesthesia to fully relax the abdominal musculature . on exploration , bowel loops were adhered together and could not be easily separated from the sac and scrotal skin . this final procedure included a lysis of adhesions to separate the small bowel from the ascending colon followed by an en bloc resection of the colon and attached scrotal skin . the specimen included 15 cm of distal ileum and ascending and transverse colon to the splenic flexure . a primary end - to - end anastomosis of the ileum to the descending colon was carried out and a bassini repair was performed . a scrotal skin reduction allowed ex - vagination of the penis ( figure 4 ) giant inguinal hernia after stage iii : bowel resection , ileo - colic anastomosis , hernia repair and resection of scrotal skin ) after discharge from the hospital the patient was followed up four years later in 2009 . the operational site looked good , the hernia repair was intact and the patient did not have any complaint . the patient returned to his village and was able to ultimately gain employment as a hospital porter . repair of a gih needs careful planning as the hernia sac can contain urinary structures , omentum and large and small bowel ( 4 ) . if the contents of the sac remain unreduced for an extended period of time attempts at sudden reduction can lead to raised intra - abdominal pressure ( 5 ) . an abdominal compartment syndrome with its consequences of respiratory and circulatory collapse and multi - organ failure can be the results of such an attempt . furthermore the diaphragm will be displaced into the thoracic cavity , resulting in respiratory difficulty and compromised venous return . management options can be grouped into either enlarging the abdominal cavity or debulking the abdominal contents . the abdominal cavity can be enlarged by insufflation with co2 over time causing a pneumoperitoneum ( 1,2 ) , a phrenectomy , or by use of a large prostetic mesh to repair the defect ( 5,6 ) . splenectomy , omentectomy , small or large bowel resections can be optional if debulking of the abdominal contents are necessary . these procedures are often combined and contribute to long operative times and usually necessitate an icu admission and post - operative ventilation ( 7 ) . furthermore neither phrenectomy nor splenectomy is advisable for a patient who must earn a living performing difficult manual labor or where infectious diseases , such as malaria , are common . long intensive care unit stays and ventilators are also rarely available in a low - resource setting context . therefore this case in sierra leone was best managed in a multi - stage operation where the abdomen was gradually accustomed to more organs being returned and finally a debulking procedure for organs that could not be safely reduced . for this case a 3-stage procedure was needed , however , earlier experiences ( n=21 ) by the senior author ( ssd ) show that 2 stage procedures can be undertaken for less complicated gih . for surgeons working or visiting such a setting , it is important to be aware of the pitfalls of trying to repair such a hernia in a single stage , but also to understand that such cases are not infrequent and that safe techniques are available . time must also be taken to explain to the patient and family that this is a multi - stage procedure that will be undertaken over a protracted period of time .

in this report we discuss the clinical management of a giant inguinal hernia ( gih ) in sierra leone where surgical resources are severely limited . though possibly not appropriate for more heavily resourced areas , a three - stage repair proved to be effective and safe for the management of this condition.careful explanation , good planning and staged operations may be needed for the repair of gih in low resource settings and we therefore encourage that they be treated by experienced personnel who are able to secure follow up of the patient . the management of gih is complicated by the potential of abdominal compartment syndrome and respiratory distress if the hernia is reduced suddenly .

doxazosin , terazosin and prazosin induce apoptosis in prostatic epithelial and stromal cells in vitro . it was even stated that terazosin and doxazosin induce apoptosis within prostates of patients with benign prostatic hyperplasia , but the biochemical pathways of doxazosin action are still not defined [ 2 , 3 ] . this effect was never observed despite long - term treatment with 1-antagonists in a huge number of patients suffering from bph all over the world . few years ago it was suggested that each tissue including a malignant one has a progenitor layer or niche . progenitor cells , which residue within such a niche have a different properties from their proliferating and differentiating counterparts . resistance to many drugs is a characteristic feature of progenitors and stem cells . drewa et al and miki et al proposed cd133 as a marker of prostate epithelial progenitors [ 4 , 5 , 6 ] . toward to resolving the question , why 1-antagonists treatment does not decrease prostate volume in bph patients we compared incidence of apoptosis induced by doxazosin in progenitor and differentiated cells isolated from human prostate epithelium . tissue specimens were obtained from 10 patients suffering from bph and undergoing adenomectomy . local ethical committee agreement was obtained . establishment of human prostate epithelium primary cultures and cell incubation with doxazosin was previously described . released epithelial cells were labeled with cd133 microbeads and sorted using supermacs ii device ( miltenyi biotec ) . after 14 days both types of primary cultures were incubated for 12 h with 20 , 50 and 80 m concentrations of doxazosin as previously described . to detect apoptotic cells annexin v conjugated with fluorescein izothiocyanate ( annexin v - fitc , immunotech , usa ) and propidium iodide ( pi ) , ( immunotech , usa ) were used . analysis was performed using flow cytometry epics xl ( coulter ) with system 2 software version 1.0 . 90 primary co - cultures of cd133(+)/cd133(- ) cells and 41 primary cultures containing cd133(+ ) cells were established . 12 h incubation of cd133(+)/cd133(- ) co - cultures with doxazosin resulted in decreasing number of viable cells and significant increase of apoptotic cell number ( fig . 1 ) . high correlation ( r = 0.98 ) between cell number and doxazosin concentration was noticed in cd133(+)/cd133(- ) co - cultures group . correlation between apoptotic cell number and doxazosin concentration in cd133(+)/cd133(- ) co - cultures group was high ( r = 0.99 ) . there was no significant changes in living and apoptotic cell number in cd133(+ ) primary cultures after 12 h incubation with doxazosin ( fig . apoptotic and viable cells after incubation with increasing concentrations of doxazosin ( 20 , 50 and 80 m ) measured using flow cytometry ( annexin v - fitc and iodide propidium ip labeling ) . the cancer stem cell hypothesis suggests that these cells are a minority population that drives tumor growth and possess resistance mechanisms against widely used drugs . cytotoxic chemotherapy eliminates most cells in a tumor , but cancer stem cells probably survive . currently , there is a huge pressure on inquiring mediators of apoptotic signal , which is very important in prostate cancer management [ 911 ] . doxazosin is commonly used in bph treatment and induces apoptosis among prostate stroma smooth muscle and epithelial cells . in our previous study we observed decreasing number of viable cells in co - cultures of cd133 + /cd133 cells after doxazosin application . in presented study we showed that doxazosin induces apoptosis in co - cultures of progenitor and differentiated prostatic epithelial cells . moreover , we observed different effect on progenitor cells , which were not susceptible to apoptosis after doxazosin treatment . similar effect is noticed after treatment of acute myeloid leukemia ( aml ) , which often failed , because administered drugs do not target leukemic stem cells ( lcss ) [ 13 , 14 ] . although , drug resistance may differ among various cancer stem cells , there is a suspicion that differential influence of doxazosin on progenitor and differentiated cells can be partially responsible for lack of prostate volume decrease after 1-antagonist treatment . in spite of small number of cancer stem cells , their properties are probably sufficient for allowing tumor recurrence . the mechanism responsible for the resistance of cd133 + cells to conventional therapies remains to be elucidated . one of the explanation could be increased expression of bcrp1 , a putative drug resistance protein , which was observed in cd133 + hepatocellular carcinoma and glioblastoma cell lines [ 15 , 16 ] . the phosphorylation of akt and accumulation of anti - apoptotic signals in akt / pkb survival pathways have also been suggested to contribute the chemoresistance of cd133 + tumor cells . the survival of cancer cells could be also promoted by oct-4 , the homeobox protein . however , progenitor cells were not susceptible to apoptosis , what can be a reason of treatment failure in bph patients .

introductioninduction of apoptosis in prostatic epithelial cells by doxazosin , terazosin and prazosin has been well documented . however , the biochemical pathways of doxazosin action is still unclear . aforementioned drugs should lead to decrease of prostate volume , although this effect was never observed in patients suffering from bph after treatment with 1-antagonists . probably , it is connected with cancer stem cells resistance on chemotherapeutic agents . the aim of this study was to compare incidence of apoptosis induced by doxazosin in progenitor and differentiated cells isolated from human prostate epithelium.material and methodsfor this purpose tissue specimens were obtained from 10 patients suffering from bph , the primary cultures of prostate epithelium were established and cd133 microbeads sorting was prepared . both , cd133(+)/cd133(- ) co - cultures and cd133(+ ) cells were incubated with different concentration of doxazosin for 12 h. cell viability and apoptosis was estimated with annexin v-fitc.results12 h incubation of cd133(+)/cd133(- ) co - cultures with doxazosin resulted in increase of apoptotic cells , while in cd133(+ ) cultures no changes were observed . correlation between apoptotic cell number and doxazosin concentration in cd133(+)/ cd133(- ) co - cultures group was high ( r = 0.99).conclusiondoxazosin induced apoptosis in co - cultures of progenitor and differentiated epithelial cells . however , progenitor cells were not susceptible to apoptosis , what can be a reason of treatment failure in bph patients .

in the current issue senn and colleagues present their findings from a study aimed at validating a new version of the flotrac / vigileo monitor ( edward lifesciences , irvine , ca , usa ) , a system that measures cardiac output using the arterial pulse contour method . the method was first used commercially in the finapres ( ohmedia , englewood , co , usa ) , a device that used a pneumatic finger cuff to continuously measure blood pressure . by transforming the pressure wave into a flow wave , cardiac output was measured from the area under the flow curve . in the past few years a number of pulse contour methods based on the arterial line pressure trace have been developed . the most well known of these methods are the picco ( pulsion medical systems , munich , germany ) , the lidco ( lidco group plc , london , uk ) and the flotrac / vigileo . until recently these systems have all required prior calibration using a second method of cardiac output measurement , either thermodilution ( pulmonary artery or transpulmonary ) or lithium dye dilution . the flotrac / vigileo has recently developed a method of self - calibration based on imputing patient demographic data . the main concern with all pulse contour systems has been their failure to properly adjust to changes in circulatory dynamics , such as blood loss or peripheral vasoconstriction . this failure has lead to more favourable reviews when these systems have been validated in the relatively stable haemodynamic setting of postcardiac surgery , but to less favourable reviews when validated in septic or liver transplant patients , where the circulatory changes are more challenging . the points to note about senn and colleagues ' study are that updated software ( version 1.07 ) was evaluated , by comparing 25 patients using the old software ( version 1.03 ) and 25 patients using the new software ( version 1.07 ) . comparisons of the flotrac / vigileo were also made against pulse contour and transpulmonary cardiac outputs using the pulsion picco system . three serial changes in cardiac output were generated by head - up head - down tilting , and the trending ability was assessed . patients were kept haemodynamically stable throughout the study with propofol remifentanil sedation , a pacing wire to control the heart rate and noradrenaline infusion targeted at a mean arterial blood pressure of 70 mmhg . their data showed a marked improvement in bland and altman limits of agreement between set a data ( 37.5% ) and set b data ( 21.6% ) . there was also a 20% difference in the reference cardiac output between the sets following the head - down tilt , as the set a reference output was 6.1 l / min and the set b reference output was 5.0 l / min . the last observation is relevant because it suggests the two sets of patients were haemodynamically different in respect to tilting responses . differences in nursing management and the use of fluids , sedation and noradrenaline in the intensive care unit could have been responsible for this difference . the improvement in bland and altman limits of agreement from 37.5% to 21.6% is puzzling . judged against the 28.3% acceptable level , the new software version performed very reliably but so did the picco , with limits of 25.5% . the authors attributed this improvement to more frequent recalibration from 10 minutes to 1 minute . readings in the study were taken after 15 minutes of continuous tilt , however , so recalibration should have occurred with both software versions . furthermore , the basic algorithm to adjust for circulatory changes does not seem to have been modified . more recent software ( version 1.10 ) still did not cope with the marked changes in systemic vascular resistance seen in liver transplant patients . these impressive agreement statistics may reflect experimental design and a failure to generate sufficiently large changes in haemodynamics to properly test the device . in set b data the systemic vascular resistance only changed by 5% ( from 960 to 1,008 dynscm ) . head - up tilting to 30 should produce at least a 10% to 20% increase in peripheral resistance in healthy adults . senn and colleagues ' study does not in my opinion comprehensively test the flotrac / vigileo system as their use of tilting did not provoke sufficiently large changes in haemodynamics . little new information can therefore be gained about the reliability of the flotrac / vigileo , other than the new software only improving reliability by more frequent recalibration . the flotrac / vigileo system has yet to be shown to cope with more extreme circulatory conditions . new software versions need to be rigorously tested in the animal laboratory against a true reference standard , such as an aortic flow probe , and need to be shown to track changes in cardiac output reliably over a range of conditions . only then should human studies be performed against thermodilution , using the standard bland and altman approach . finally , one should not forget that the clinical utility of the device would still need to be shown in randomized , clinical controlled trials .

the present study was performed to test a new software version of the flotrac / vigileo using head - up head - down tilting in post - cardiac surgery patients . impressive improvements in bland and altman limits of agreement from 37.5% to 21.6% were recorded . the results , however could be attributed to a failure to produce a wide enough range of test circulatory conditions . a more rigorous test of performance is needed before any real conclusion concerning use of the flotrac / vigileo in clinical practice can be made .

newborn babies commonly develop mucocutaneous candidiasis , which is usually acquired during the process of birth . congenital cutaneous candidiasis presenting at birth up to 25% of pregnant women harbor candida albicans in their vaginal flora , and hence , mucocutaneous candidiasis is common in their babies . by contrast , congenital cutaneous candidiasis is very uncommon with only about 70 cases reported in the literature over the past 40 years and none in indian literature . a female child weighing 3.1 kg was born to a 30-year old gravida 3 , parity 2 , and vdrl negative mother by vaginal breech delivery . mother did not have any per vaginal leak , bleed , or premature rupture of membrane prior to onset of labor . on examination , maculopapular eruptions over the face trunk , back and extremities ( figure 1 ) . in many areas , her white blood cell count was 17,000/mm with 45% polymorphonuclear cells , 6% band cells , 30% lymphocytes . skin biopsy with periodic acid - schiff ( pas ) staining revealed the fungus within stratum corneum . no further complications were observed ; vesicles began drying by fourth day and desquamated completely by day 10 . , congenital cutaneous candidiasis ( ccc ) occurred from a combination of maternal vaginal candidiasis , prolonged rupture of membranes , and administration of antibiotics . although 20%25% of women harbor candida species in their vagina only a few ascending infections are noted . whether this relative infrequency of ccc is because of asymptomatic infection or resistance to uterine invasion by candida a factor associated with congenital candidiasis is a foreign body in the uterus or cervix , such as a retained iucd or sutures placed for cervical encirclage . it begins as erythematous macules that may evolve through papular , vesicular , and pustular stages . prematurity is generally accepted as a factor predisposing to disseminated disease because of their immature , compromised mucocutaneous barrier and systemic host defenses . those with burn - like dermatitis , respiratory distress , altered liver enzymes as well as laboratory evidence of sepsis should be considered systemically infected until proven otherwise . differential diagnosis includes listeriosis , syphilis , staphylococcal , and herpes simplex infection as well as erythema toxicum , transient neonatal pustular melanosis , malaria , and drug eruption . infection may be limited to the skin or may disseminate causing respiratory distress , sepsis , or deranged liver function tests . johnson et al . studied 31 case reports of newborns with ccc and showed that 15 responded to topical therapy alone . they suggested that the risk of dissemination of ccc is increased if : ( 1 ) there is an evidence of respiratory distress or other signs of sepsis in immediate neonatal period ; ( 2 ) birth weight is < 1500 gm ; ( 3 ) received treatment with broad spectrum antibiotics ; ( 4 ) extensive instrumentation during delivery and ( 5 ) positive blood , urine , or cerebrospinal fluid ( csf ) culture . blood , urine , and csf cultures should be obtained to rule out systemic involvement . treatment is in the form of topical antifungal therapy given the benign nature of the illness . oral nystatin suspension is often added to decrease the number of organisms in the gastrointestinal tract . systemic antifungal therapy is recommended for neonates with burn - like dermatitis attributable to candida species , or positive blood , urine , and/or cerebrospinal fluid cultures . systemic treatment also should be considered for all infants with ccc who have respiratory distress in the immediate neonatal period and/or laboratory signs of sepsis such as an elevated leukocyte count with an increase in immature forms or persistent hyperglycemia and glycosuria .amphotericin b ( 0.51 mg / kg / day ) is the first line agent for treatment of systemic disease . lipid - associated amphotericin b preparations ( 35 mg / kg / day ) are preferred in those cases with invasive candidiasis and severe preexisting renal insufficiency . fluconazole ( 6 - 12mg / kg / day ) can be used as alternative therapy to amphotericin b , if the candida species is identified and the susceptibility is shown or toxicity of amphotericin b is prohibiting its use . flucytosine ( 50100 mg / kg / day ) is used in combination with amphotericin b for central nervous system infection . there are no controlled clinical trials to provide the optimal length of therapy . the topical antifungal agent must be used until the lesions resolve , and the systemic therapy must be used a minimum of 2128 days . prompt initiation of antifungal therapy appears to be the most important associated with survival in systemic infection . we emphasize the self - limited character of this disease , although preterm infants may be at risk of systemic spread .

congenital cutaneous candidiasis ( ccc ) is an extremely rare disorder that presents within the first 6 days of life . the manifestations ranges from diffuse skin eruption without any systemic symptoms to respiratory distress , hepatosplenomegaly , sepsis , and death . we report a neonate who presented with generalized skin eruptions at birth , characterized by erythematous macules and papules . the eruption involved head , face , neck , trunk , and extremities . candida albicans was demonstrated on direct koh smear , skin biopsy . the disease implies a congenital intrauterine infection and is different from neonatal candidiasis , which manifests as thrush or diaper dermatitis . the infection is acquired from the maternal genital tract in an ascending fashion . clinical features , direct smear examination of specimen , and appropriate cultures are useful in differentiating the lesions from other more common dermatoses of the neonatal period . topical antifungal therapy is sufficient unless systemic candidiasis is present . prognosis for congenital cutaneous candidiasis is good .

this genus comprises of gram - negative , strictly - aerobic , non - fermenting , non - fastidious , non - motile , catalase - positive , oxidase - negative bacteria with dna g + c content of 39.0% to 47.0% . according to euzeby 's list of prokaryotic names with standing in nomenclature ( http://www.bacterio.cict.fr/a/acinetobacter.html ) acinetobacter guillouiae proposed by nemec et al . was isolated from sewage - containing gas - work effluent and shares characteristics corresponding to those of the genus acinetobacter . a. guillouiae strain msp 4 - 18 , isolated from a mangrove soil sample from parangipettai ( 1130n , 7947e ) , tamil nadu , india , was grown on tryptic soya agar medium ( tsa ; himedia ) at 30 c . genomic dna was extracted from 36 hour old culture using zr fungal / bacterial dna miniprep as per manufacturer 's instructions . amplification and sequencing of 16s rrna was performed as described by mayilraj et al . . identification was confirmed using 16s rrna sequencing . to determine the phylogenetic relationship of strain msp4 - 18 , the 16s rrna sequence consisting of 1502 bp was compared with those of type strains of species of related genera and identification of phylogenetic neighbors and the calculation of pairwise 16s rrna gene sequence similarities were achieved using the eztaxon server and aligned using mega version 5.0 . bootstrap analysis was performed to assess the confidence limits of the branching ( fig . 1 ) . the genome of a. guillouiae msp 4 - 18 was sequenced using the illumina - hiseq 1000 technology . sequencing resulted in 26,685,818 paired - end reads ( insert size of 350 bp ) with a length of 101 bp . a total of 26,465,246 high - quality reads with approximately 550 coverage were assembled with clcbio wb6 ( word size 40 and bubble size 60 ) to obtain 94 contigs ( n50 , 128,068 bp ) of 4,848,959 bp and average g + c content of 38.0% . the functional annotation was carried out by rast ( rapid annotation using subsystem technology ) , fig . 2 shows the subsystem distribution of a. guillouiae strain msp 4 - 18 , trna was predicted by trnascan - se 1.23 and rrna genes by rnammer 1.2 . the genome contains 3 rrna genes ( 5s-23s-16s ) and 69 aminoacyl - trna synthetase genes . a total of 4543 coding regions ( 2294 genes transcribed from the positive strand and 2249 from the negative strand ) were found in the genome , of which 3052 ( 67% ) could be functionally annotated . the genome coding density is 83% with an average gene length of 883 bp . the annotated genome has 106 genes responsible for resistance to antibiotic and toxic compounds including 13 genes for mdr efflux pumps . sixty five genes are involved in oxidative stress , 12 in osmotic stress , 15 for heat shock and several others for response to various other stresses , to make a total of 107 genes responsible for stress response in this organism . the functional comparison of the genome sequences available on the rast server revealed the closest neighbors of a. guillouiae msp 4 - 18 as acinetobacter baumanii ab0057 ( score 502 ) followed by a. baumanii aye ( score 500 ) , acinetobacter johnsonii sh046 ( score 494 ) and a. baumanii acicu ( score 494 ) . the a. guillouiae msp 4 - 18 whole genome shot gun ( wgs ) project which has been deposited at ddbj / embl / genbank under the project accession asqg00000000 of the project ( 01 ) has the accession number asqg01000000 and consists of sequences asqg01000001asqg01000094 . the authors declare that there is no conflict of interest on any work published in this paper .

the genus acinetobacter consists of 31 validly published species ubiquitously distributed in nature and primarily associated with nosocomial infection . we report the 4.8 mb genome of acinetobacter guillouiae msp 4 - 18 , isolated from a mangrove soil sample from parangipettai ( 1130n , 7947e ) , tamil nadu , india . the draft genome of a. guillouiae msp 4 - 18 has a g + c content of 38.0% and includes 3 rrna genes ( 5s , 23s , 16s ) and 69 aminoacyl - trna synthetase genes .

bony overgrowth involving the medial end of the clavicle and sternum along with periarticular soft - tissue calcification are typical features of this disease . this condition is strongly associated with palmoplantar pustulosis and seronegative spondyloarthropathy computed tomography ( ct ) scans of sternoclavicular joint ( scj ) are commonly used for the diagnosis of this disorder . a 42-year - old male attended our rheumatology outpatient clinic with a history of swelling and mild pain of right scj for about eight months . the patient had been diagnosed to have ankylosing spondylitis since ten years with predominantly axial involvement . ankylosing spondylitis disease activity was fairly controlled ( bath ankylosing spondylitis disease activity index : 2.5 . ultrasonography ( usg ) and doppler examination revealed calcifications around the joint with periarticular hypervascularity [ figures 1 and 2 ] . ct scan showed calcifications around the right sternoclavicular joint and adjacent right first rib [ figure 4 ] . the patient was treated with non - steroidal anti - inflammatory agent and advised rest , physiotherapy and follow - up . usg of the right sternoclavicular joint ( rt scj ) showing calcification around the medial end of the clavicle ( solidarrow ) and sternum ( dottedarrow ) . normal left scj ( lt scj ) for comparison color flow imaging of the right sternoclavicular joint ( rt scj ) showing periarticular hypervascularity ( solidarrow , dottedarrow - medial end of clavicle . arrowhead - sternum ) . the normal left side ( lt scj ) for comparison duplex study of right scj vessels ( arrow ) showing low - velocity flow with prolonged antegrade diastolic phase axial ct scans of the sternoclavicular joints showing soft - tissue calcification around the medial end of the right clavicle ( arrow ) scch is a chronic inflammatory disease which commonly presents with pain , swelling and redness of the affected joint . between 10 - 30% cases the disease characteristically starts as a non - specific inflammation of the sternoclavicular ligament and also involves periarticular soft tissues . the chronic inflammatory process ultimately leads to bony hyperostosis and localized soft - tissue calcification which may result in fusion of scj in longstanding disease . differential diagnosis of unilateral scj arthritis includes infective and inflammatory pathologies like sternal osteomyelitis , septic arthritis , rheumatoid arthritis and osteoarthritis of scj . ct scan detects the characteristic changes of scch earlier than conventional radiography and is therefore the preferred modality for diagnosis of scch . findings range from localized disease with mild ossification of costoclavicular ligament to the formation of a bone mass between the clavicle , sternum , and first or upper ribs . based on these features , sonozaki et al . , categorized the radiological findings into three groups . this was later modified by various other authors to include those without definite ossification or evidence of multiple rib or extrasternal involvement . radiography of the sternoclavicular joint and scintigraphy are other modalities used in the diagnosis of scch . scintigraphy shows increased radiotracer uptake in characteristic regions and is helpful in the diagnosis of scch , especially when radiographic changes are subtle . power doppler usg of hand joints has good sensitivity and accuracy in detecting active synovitis in rheumatoid arthritis . in rheumatoid arthritis of scj , in addition , synovial vascularity can be assessed by color doppler study giving some information on activity of the disease . infective conditions of scj like septic arthritis and osteomyelitis demonstrate joint effusion , irregularity of the bony margins and soft tissue thickening on usg . there is a paucity of data regarding the role of usg in the assessment of scj pathology ; neither is there any case report with usg description of scch . the capability to perform dynamic real - time imaging is a unique advantage of usg . ct scan is very useful for detection of articular and peri - articular changes seen in scch . abnormalities of the bony cortex , articular surface irregularities and soft - tissue calcifications are readily detected by ct scan but it is relatively expensive . in our case , further , the application of color flow imaging helped us to study the ongoing inflammatory process within the affected joint and thereby detection of scch at an earlier stage . this is very important from the prognostic viewpoint as early intervention may prevent scj fusion . usg and color doppler evaluation is useful in the diagnosis of scch as well as determining the disease activity . this cost - effective modality must be generously used in imaging of sternoclavicular joint pathologies .

sternocostoclavicular hyperostosis is a chronic inflammatory disorder affecting the sternoclavicular joint and upper ribs . there is a strong association with seronegative spondyloarthropathy in which bilateral involvement is common . ultrasonography and color doppler findings of this entity have not been described previously , to the best of our knowledge . we describe the findings in a patient of ankylosing spondylitis who was referred for unilateral sternoclavicular joint swelling .

lipoma is the most common soft tissue mesenchymal neoplasm in adults , which is located in any part of the body . the majority occurs in the upper - half of the body , particularly the trunk , head , and neck region , and it is rarely described in the oral cavity . conventional lipoma has been divided into three forms according to the origin of the localization : superficial lipoma ( arise within subcutaneous tissue ) , deep lipoma ( arise within deep soft tissue ) and parosteal lipoma ( arise within the surfaces of bone ) . the histological characteristics and types vary , which include angiolipoma , angiomyolipoma , myolipoma , osteolipoma and chondrolipoma . both osteolipomas and chondrolipomas are rare entities.[24 ] however , occurrence of osteolipomas or in other term lipomas with oseous and cartilagenous metaplasia are less common than other subtypes of lipomas . the term of osteochondrolipoma has been used for the lipomas containing both cartilage and bone which , are very rarely seen and generally associated with a paraosteal localization . to our knowledge , a 64 year old male patient with painless mass on his mandibulary symphysis region , was referred to the department of oral and maxillofacial surgery , faculty of dentistry on september , 2007 . the lesion had been first noticed 2 months prior of the presentation to the clinic with no significant enlargement . clinical examination of the oral cavity revealed a well demarcated , firm , round and exophitic lesion of approximately 2 cm in diameter at the symphysis region of the left mandible . the lesion was firmly attached to the bone in the clinical examination and the overlying mucosa was intact and of normal color . radiographically , at the site of the lesion some degree of radioopacity was observed [ figure 1 ] . under local anesthesia , the patient was taken to the operating room and the lesion was completely excised with an intraoral approach . during the surgical intervention , the encapsulated lesion was not adherent to the underlying periosteum and overlying mucosa , thus the lesion was dissected and removed easily [ figure 2 ] . the surgical specimen was histopathologically evaluated for final diagnosis at department of oral pathology , faculty of dentistry , gazi universitys . the 6-month postoperative follow - up showed healing of the oral mucosa without any recurrency . preoperative panoromic radigoraph exhibiting the dense radioopacity ( arrow ) in the symphysis region of the mandible intraoperative view of the lesion with encapsulation macroscopically , the gross specimen was a 2.7 1.8 1.4 cm encapsulated mass mostly comprising of adipose tissue with a few number of encapsulated nodular structures . on section , focal areas of hyaline chondroid structures and lamellar bone with fatty marrow were seen throughout the lesion . both islands of bone and cartilage were surrounded with well defined fibrous tissue [ figure 3 ] . there was no histological evidence of malignancy , such as mitosis , cellular atypia , necrosis , and hemorrhage . histochemistry with alcian blue periodic acid - schiff stain showed acid mucopolysaccaride presence in chondroid tissue ; hence , this was hyaline chondroid . on immunohistochemistry , adipocytes were positively stained with vimentin antibody , while both adipocytes and chondrocytes were positive for s-100 protein [ figure 4 ] . histopathological features of osteochondrolipoma showing mature adipose tissue containing cartilage islands and lamellar bone trabecules ( h and e , 40 ) chondrocytes and adipocytes demonstrating positivity for s 100 protein ( immunoperoxidase , 40 ) however , they are relatively uncommon in the oral cavity with incidence of 14% . variants of lipoma especially osteolipoma and chondrolipoma are very rare lesions which are located in the oral cavity . furthermore , intaoral lipoma with both osteoid and cartilaginous metaplasia has been reported just in two cases . we presented an osteochondrolipoma case that differed from the typical arrangement by two rare alterations . first , the lesion showed well - defined cartilaginous and osseous areas within lipoma that is also considered as osseous and cartilaginous metaplasia . in the a study of fregnani et al . , fibrolipoma was reported as the dominant subtype in 46 lipoma cases , in which neither osteolipoma nor chondrolipoma was found . two lipomas with cartilaginous or osseous metaplasia was reported out of 125 lipoma cases in another study , whereas none of the cases represented osteochondrolipoma . although , any case in the name of oral osteochondrolipoma has not been reported , lipoma with osteoid and cartilaginous metaplasia has been represented by two cases in the oral region . the osteochondrolipoma is the specific term of the lipoma with mature cartilaginous and osseous differentiation ; thus , both terms represents the same entity . some authors consider such type of lipoma as a benign mesenchymoma due to consisting of two or more mesenchymal elements such as lipocytes , chondrocytes , osteocytes , and fibroblasts . jones et al . stated that the term of benign mesenchymoma should be used strictly to describe an unencapsulated neoplasm composed of two or more mature mesenchymal tissues not ordinarily associated with each other , excluding fibrous connective tissue . in the present case , as the tumor is well demarcated and partially encapsulated and moreover the prominent component is mature adipose tissue , the tumor is considered as osteochondrolipoma . second alteration that took part in this case , was the parosteal localization of the lesion . the parosteal lipoma which is defined as lipoma exhibits a conguous relationship with the periosteum , usually demonstrating some form of attachment to periosteum with an underlying osseous reaction , is rarely seen in the oral cavity . various types of osseous cortical responses from a reactive overproduction of bone to cortical erosion can be seen underlying bone . the radiopacity in our case may reflect either the reactive bone production of underlying bone or the density of cartilagenous and ossous component of the tumor . multipotent cells of mesenchyme , different cell lines and metaplastic differentiation of the adipose tissue are three hypothesis regarding the origin of the tumor . however , considering its parosteal location , histological findings of well - formed cartilage and bone tissue within the adipose tissue neoplasm , it is decided to be a true neoplasm of mesenchymal tissue stem cells showing characteristics of three different tissues originating from the mesenchyme .

osteochondrolipoma is a rare benign soft tissue neoplasm . it is occasionally considered to be a variant of adipose tissue neoplasm lipoma showing multiple differentiation pathways of pluripotent stem cells . as with the lipomas they can be seen at any location and show cartilagenous and osteoid differentiation when located parosteally . we present a case of osteochondrolipoma located at the symphysis of the mandible . to our knowledge , this is the first reported case of an oral osteochondrolipoma associated with parosteal localization .

male bowerbirds create and decorate a structure called a bower which is used only for attracting and mating with females ; females then go off and make their own nests and raise their offspring by themselves . female bowerbirds choose which male to mate with on the basis of various bower and male characteristics ( 1 , references in ref . male great bowerbirds ( ptilonorhynchus nuchalis ) build a large ( 0.6 min long ) avenue of densely thatched sticks opening into two slightly larger flat areas or courts which are covered with colorless objects ( gesso ) , and decorated with colored objects . from within the avenue , females watch the male display his head and lilac - pink nuchal crest , and colored objects in his bill , over the gesso . great bowerbirds go further , and arrange the gesso objects in increasing size with distance from the avenue entrances . the combined effect of the geometric gesso pattern and the fact that the avenue creates a predictable direction and field of view of the court sets up perfect conditions for a geometric optical illusion known as forced perspective . the forced perspective has several possible consequences including creating a more regular gesso pattern as seen by the female within the avenue , altering the perceived sizes of court and displayed objects , and creating further illusions which attract the female 's attention . to decide whether or not what bowerbirds do is art , in principle all that one would have to do would be to compare it to the definition of art . unfortunately , the definition of art as a human activity is problematic ; no two dictionaries define it the same and controversy rages . here skill , esp . human skill as opposed to nature ; ( ability in ) skilled execution as an object in itself ; cunning ; imitative or imaginative skill applied to designand thing in which skill may be exercised . skill acquired by experience , study , or observation , and the conscious use of skill and creative imagination . the production or expression of what is beautiful ( especially visually ) , appealing , or of more than ordinary significance , and a method of doing a thing , especially if it is difficult . the process or product of deliberately arranging elements in a way to affect the senses or emotions , and fine art means that a skill is being used to express the artist 's creativity , or to engage the audience 's aesthetic sensibilities , or to draw the audience towards consideration of the finer things . the use of skill and imagination in the creation of aesthetic objects , environments , or experiences that can be shared with others . the conscious production or arrangement of sounds , colors , forms , movements , or other elements in a manner that affects the sense of beauty . note that some definitions depend upon other problematic definitions ( e.g. , production of beauty ) . not only is the definition of art variable and controversial , but to some merely defining it is thought to restrict artistic creativity . for a thorough discussion of definitions and their problems one way to minimize confusion is to simply create an operational definition which attempts to capture most of the ideas . for this reason i suggest that visual art can be defined as the creation of an external visual pattern by one individual in order to influence the behavior of others , and an artistic skill is the ability to create art . influencing behavior can range from attraction to and voluntary viewing of the art by others to viewers mating with the artist ( modified from the online supplemental material of reference 3 ) . this definition equates art with conventional signals and aligns it with signaling theory , including that in animals . art objects are conventional signals that are not part of the artist 's body but have the same function as signals originating from the body . by this definition both bowerbirds and humans produce and react to art , and the practitioners of art can be called artists . darwin and diamond first suggested that an aesthetic sense underlies sexual selection for bowerbird art , but most discussions of animal aesthetics seem to be anthropomorphic or use human - style standards ( e.g. , symmetry or beauty ' ) in tests , measure human neural correlates of beauty , or test animals with human paintings . although these papers are interesting , it is difficult to know how to interpret them , although putting aesthetics in a sexual selection context is a good first step . the general concept of aesthetics and the definition of aesthetic sense is at least as controversial as the definition and concept of art , and even more controversial when applied to animals . what does seem to be generally agreed upon by philosophers is that aesthetics involves judgement ; the assessor ( the one with an aesthetic sense ) must be able to judge and perhaps even rank the quality of different art objects or individuals . the darwinian approach suggests an operational definition : judgement is the active choice among different art objects or individuals leading to change of fitness in both artist and judge . these definitions implicitly include cases where both artist and judge benefit as well as cases of deception which lead to fitness gain in the artist but fitness loss in the judge . variation in judgement probably encourages variation in art , and this may well work in animals . it would be illuminating to examine the theoretical relationship between variation in judgement and variation in art and other signals to investigate whether one leads to variation in the other , and vice versa , and what processes jointly maintain variation in judgement and judged . the operational definitions of art , judgement and aesthetics suggest that great bowerbirds are artists and have an aesthetic sense . males create art because their created bower is voluntarily viewed by females , leading to changes in their behavior up to and including mating with the artist . males have an aesthetic sense in that they have to create the bowers and forced perspective , and appear to constantly maintaining and improving the bower geometry . individual males vary in the quality of the art ( forced perspective and associated geometric patterns ) that they produce . females judge the art and mate with those males which produce the best art , and both benefit by producing offspring . one complication for bowerbirds is that there is a possibility that females choose males producing the best perspective not because the particular perspective pattern is best for that particular male , but because a more even pattern of court objects makes his displayed objects more conspicuous or generates more attention - grabbing illusions . however , the quality , conspicuousness and attention - grabbing aspects of the bower are not mutually exclusive and all of these factors affect the female s judgement . as in humans , the target of aesthetics is not always straightforward or even unimodal . seeking single explanations for things other animals build and decorate structures , for examples sticklebacks , cichlids , chubb , sand gobies , raptors and owls . however , in these cases the structure ( a nest ) is also used for reproduction as well as signaling quality or breeding status so it has utility . so , for example , by that more restricted art definition the colorfully decorated stickleback nests are not art . however , by the operational definitions given here , these species do exhibit art , judgement and aesthetics . so far , only the great bowerbird exhibits art which has no direct function other than to modify the behavior of its viewer . many of these ideas need further clarification . the use of operational definitions of art , judgement and aesthetics should help to test hypotheses about these phenomena in other animals .

male bowerbirds create and decorate a structure called a bower which serves only to attract females for mating , and females visit and choose one among many bower owners before deciding which male to mate with . is what they do art , and do they have an aesthetic sense ? i propose operational definitions of art , judgement , and an aesthetic sense which depend upon communication theory which allow one to get explicit answers to this question . by these definitions great bowerbirds are artists , judge art , and therefore have an aesthetic sense .

traumatic arteriovenous fistula ( avf ) is a pathology characterized by arteriovenous shunting secondary to blunt or penetrating trauma of artery and vein running in parallel , but avf of the superficial temporal artery ( sta ) is rare . in this article , we report a case of histopathologically confirmed avf of sta , which was surgically treated 1 month after surgery for right acute epidural hematoma caused by a traffic accident . computed tomography scan had shown an acute epidural hematoma on the right surface of frontal lobe , which was emergently treated by surgical evacuation . one month later , he began to complain of pulsatile tinnitus in the left ear . a head magnetic resonance angiogram showed an extremely dilated vein in the left temporal region ( fig . 1 ) . an angiography revealed a direct shunt between the left parietal branch of the sta and the superficial temporal vein ( stv ) in the preauricular region as well as an aneurysm in the left frontal branch of the sta ( fig . 2 ) . histopathological examination demonstrated that the artery was connected to the vein through the thin - walled vessels lacking internal elastic lamina and muscularized media , suggesting that the connecting vessels were newly formed ( fig . figure 2:left external carotid arteriography showed a high - flow avf from the parietal branch of the sta ( thin arrow ) to the stv ( wide arrow ) , and an aneurysm on the frontal branch of the sta ( arrowhead ) . ( b ) the sta ( arrow ) was connected to the stv ( asterisk ) . figure 4:histopathological photomicrograph with elastica van gieson stain showed that a thin - walled vessel lacking in internal elastic membrane ( arrow ) connected the artery ( a ) and the vein ( v ) . left external carotid arteriography showed a high - flow avf from the parietal branch of the sta ( thin arrow ) to the stv ( wide arrow ) , and an aneurysm on the frontal branch of the sta ( arrowhead ) . ( b ) the sta ( arrow ) was connected to the stv ( asterisk ) . histopathological photomicrograph with elastica van gieson stain showed that a thin - walled vessel lacking in internal elastic membrane ( arrow ) connected the artery ( a ) and the vein ( v ) . blunt trauma to the sta may cause both aneurysm and avf , but the incidence of avf is lower than that of aneurysm , with a ratio of 23:131 , and only 36 cases have been reported as of 2007 . as in this case , the preauricular region is one of the most commonly affected areas due to its anatomy : sta and stv run in parallel with each other over the temporal bone . traumatic avf is treated with direct surgery or endovascular therapy . in direct surgery , since the block of the incoming blood vessel has a risk of recurrence , complete resection of the lesion is essential . endovascular treatments include percutaneous coil embolization , embolization with liquid embolic agents such as isobutyl l-2-cyanoacrylate or onyx , direct - puncture coil embolization , and injection of absolute alcohol , all of which have been reported with good outcomes . however , avf in the external carotid arterial system , which has potential complex collateral circulation , may be difficult to treat again if incomplete embolization ends in relapse .

traumatic arteriovenous fistula ( avf ) of the superficial temporal artery ( sta ) is rare . we report a case of surgically treated , histopathologically confirmed traumatic avf of the sta . a 24-year - old male was admitted to our hospital after a traffic accident . computed tomography scan on admission showed an acute epidural hematoma on the right surface of frontal lobe , which was emergently treated by surgical evacuation . one month later , he was aware of pulsatile tinnitus around the left ear . digital subtraction angiogram showed an avf , mainly fed by the left sta . after total removal of the fistula , his tinnitus immediately disappeared . histopathological examination demonstrated that the artery was connected to the vein through the thin - walled vessels lacking internal elastic lamina and muscularized media , suggesting that the connecting vessels were newly formed .

cutaneous metastases occur in 0.7~0.9% of all patients with cancer , and the most frequent origins of cutaneous metastases are the breast , lung and colon cancer1 ) . there are scant reports in the literature on the frequency of skin metastasis as the initial manifestation of a malignancy2 - 6 ) . the most frequent sites of metastasis for carcinoma of the pancreas are the lymph nodes , lung , liver , adrenal glands , kidney and bone7 ) . cutaneous metastases are rare , and they are generally situated in the periumbilical area8 ) . to our knowledge , very few patients have been reported on with a solitary lesion or multiple cutaneous lesions that ultimately disclose a pancreatic adenocarcinoma . there was only one case report about pancreatic cancer skin metastasis in korea , but the patient in that case complained of chest pain and dyspnea due to the malignant pleural effusion9 ) . we report here on a patient who presented with a metastatic skin lesion in an unusual location as the initial manifestation of pancreatic cancer . a 68-year - old korean man was seen in the dermatology department because he had a painless subcutaneous right forearm nodule for 3 months . two firm , 1.0 cm , subcutaneous nodules were noted on the physical examination ( figure 1 ) . the histology revealed neoplastic gland cords or nests in the desmoplastic stroma , and this was consistent with metastatic adenocarcinoma ( figure 2 ) . he was referred to the gastroenterology department to further investigate the source of the cutaneous lesion . he was totally asymptomatic except for the multiple subcutaneous nodules on his forearm , but he complained of a 5 kg weight loss during the last six months . his past medical history and family history were non - contributory . apart from the subcutaneous nodules , the remainder of the exam was normal , including both the rectal and abdominal examinations that showed no masses . no enlarged lymph nodes were present , and the prostate was normal in size and consistency . the routine blood chemistry tests revealed only a slightly elevated alkaline phosphatase of 238 u / l ( normal : 30~110 u / l ) , but there was a remarkable elevation in the tumor markers ca 19 - 9 to 486.9 u / ml ( normal : 0.139 u / ml ) . the gastroscopy and colonoscopy exams revealed no specific abnormality except for a hyperplastic gastric polyp . the abdominal computed tomography ( ct ) detected a low - density mass located in the tail of the pancreas along with multiple liver metastases ( figure 3 ) . the ct and ultrasonography findings of the abdomen were compatible with pancreatic cancer with multiple liver and cutaneous metastases . the immunohistochemical staining of the skin biopsy showed a positive reaction to monoclonal antibodies for ck 7 , ck 19 and thyroid transcription factor ( ttf ) , and these markers are normally present in adenocarcinoma of the lung ( figure 4 ) . we describe here an unusual case of pancreatic cancer presenting as subcutaneous nodules on the forearm and chest wall . looking et al.6 ) found that two out of 420 cases ( 0.47% ) of cutaneous metastatic lesions had originated from pancreatic cancers . the most common site of cutaneous metastasis from the pancreas is the umbilicus , and this is known as ' sister mary joseph 's nodule10 ) . our case is unique in that the unusual cutaneous metastasis of pancreatic cancer as the first clinical manifestation was on the forearm and back , in contrast to the umbilicus . also , the patient was asymptomatic from his pancreatic cancer on admission , and this was despite the neoplasm being located in the body and tail of the pancreas . miyahara et al.11 ) reported on four cases in which cutaneous metastases presented prior to the diagnosis of pancreatic cancer . cubilla et al.12 ) reported that 8 of 50 cases ( 16% ) of occult pancreatic cancer had metastatic skin lesions as the first manifestation of the malignancy . our case and the other cases noted above reinforce the notion that metastatic skin lesions may be the first manifestation of an occult pancreatic malignancy . takeuchi et al.13 ) revealed that the tail of the pancreas was the most common site of the primary tumor , and the gross appearance of the cutaneous tumors in that study was oval , firm , solid , non - painful nodules . although an excellent response to gemcitabine has been reported on in one case report14 ) , most of the patients died within 7 months . trying to determine the primary source of the cutaneous metastasis from occult tumor is very difficult . however , immunohistochemistry may enable the physician to precisely identify the primary neoplasm . ca 19 - 9 , which was initially found in colorectal adenocarcinoma , has the highest sensitivity as a tumor marker for adenocarcinoma of the pancreas . this antigen is also found in other tumors , as well as in non - cancerous diseases . according to ridwelski et al.15 ) , monoclonal antibodies against cytokeratin may be more specific and reliable than ca 19 - 9 at detecting disseminated tumor cells in lymph nodes . this is especially true because the false positivity or false negativity for ca 19 - 9 is related to the level of ca 19 - 9 . many different types of ck that are specific to various organs do exist . in the pancreas , ck 8 and ck 18 are produced by the exocrine acinar cells , the endocrine islets and the duct cells . pancreaticobiliary ductal adenocarcinomas , in general , have a cytokeratin immunophenotype identical to that of normal pancreatic ducts , including being positive for ck 7 , 8 , 18 and 1916 ) . approximately 90% of pancreaticobiliary adenocarcinomas stain duval et al18 ) have recently reported that the majority of pancreatic carcinomas and extrahepatic carcinomas were positive for ck 7 and negative for ck 20 . therefore , the findings concerning the immunohistochemical expression of cks are considered as being useful in the diagnosis of metastatic carcinomas . clinicians should be aware that metastatic cutaneous lesions could be the initial presenting sign for pancreatic cancer . also , the immunohistochemical staining for ck 7 and 19 may be helpful for the diagnosis of metastatic pancreatic adenocarcinoma .

cutaneous metastases from pancreatic adenocarcinomas are rare lesions . the most common site of cutaneous metastasis is the umbilicus , and this is also known as the ' sister mary joseph ' nodule . a 68-year - old korean male , who was previously healthy and asymptomatic , was seen in the dermatology department for two subcutaneous nodules that he had on his right forearm and his back . histological examination of the right foreman nodule revealed metastatic adenocarcinoma . immunohistochemical staining for cytokeratin ( ck ) 7 and ck 19 were positive , and this strongly suggested the pancreatic duct as being the primary source of the cancer . the abdominal computed tomography findings were compatible with pancreatic cancer . clinicians should be aware that metastatic cutaneous lesions could be the initial presenting sign for pancreatic cancer . the immunohistochemical staining for ck 7 and 19 may also be helpful in the diagnosis of metastatic pancreatic adenocarcinoma .

an article recently published demonstrated the efficacy of eculizumab in the treatment of children with severe hematopoietic stem cell transplantation - associated thrombotic micro - angiopathy ( hsct - tma ) . we report the case of an adult with hsct - tma successfully treated with eculizumab . hsct - tma is a rare but very serious complication of allogeneic hematopoietic progenitor stem cell transplantation . several factors have been implicated in the endothelial damage which leads to hsct - tma : calcineurin inhibitors , acute graft versus host disease ( gvhd ) and cytomegalovirus ( cmv ) infection ; however , in recent years another mechanism has been described in which complement deregulation plays an important role . therefore complement - modulating therapies are beginning to gain ground in the treatment of this complication . we report the case of a 30-year - old man , diagnosed with very serious acquired bone marrow aplasia in july 2014 . he underwent progenitor stem cell transplantation of bone marrow from his hla - identical sister in july 2014 . the conditioning regimen consisted of cyclophosphamide ( 30 mg / kg / day , 7 to 4 ) , fludarabine ( 30 mg / m2/day , 5 to 2 ) and antithymocyte globulin ( 2.5 mg / kg / day , 3 to 1 ) . , the patient developed acute cutaneous and liver gvhd ( grade ii ) which initially responded to treatment with corticosteroids and etanercept . the patient was readmitted on post - transplant day 116 with diarrhea and hyperbilirubinemia ( 1.7 mg / dl , normal values 0.3 - 1.1 mg / dl ) and colonoscopy confirmed the existence of acute intestinal gvhd . after the diagnosis of acute grade iii gvhd , which was refractory to steroids , he sequentially received various lines of treatment ( corticosteroids , mesenchymal stromal cells and sirolimus ) without any response . on post - transplant day 189 , the patient developed severe bloody diarrhea ( up to 3000 ml / day ) followed by persistent rectal bleeding that required intense transfusional support and treatment with activated factor vii ( 5 mg/2 h 6 doses ) . a new colonoscopy was performed and the colonic mucosa biopsy confirmed worsening of the intestinal gvhd without histological evidence of hsct - tma ( figure 1 ) . iu / l ) , total bilirubin 0.7 mg / dl ( normal values 0.3 - 1.1 mg / dl ) , hemoglobin 8.5 g / dl , platelets 4210/l and normal coagulation tests . treatment was then initiated with one dose of pentostatin ( 4 mg / m2 iv ) and alemtuzumab ( 20 mg sc 3 times / week for 2 weeks ) . one week after the administration of pentostatin , and with persistent gastrointestinal bleeding , biochemistry showed hyperbilirubinemia ( total bilirubin 6.4 mg / dl , direct bilirubin 5.5 mg / dl , normal values 0.0 - 0.5 mg / dl ) and elevated ldh ( 2700 the blood count revealed profound anemia ( up to 6.8 g hb / dl ) , reticulocytosis ( 0.310/l ) , thrombocytopenia 3910/l and the presence of numerous schistocytes in blood smear ( 6% ) . other laboratory findings were : negative direct coombs test , undetectable haptoglobin , proteinuria ( 30 mg / dl ) , normal adamst13 activity ( 94% ) and normal complement proteins ( c3 and c4 ) . that the patient was diagnosed with hsct - tma , treatment was initiated with eculizumab 900 mg iv weekly for 4 doses followed by a single maintenance dose of 1200 mg 2 weeks later . after the first dose of eculizumab , the patient ceased to require transfusions and a progressive improvement in analytical parameters for microangiopathy was observed until their complete normalization after 7 weeks ( hb 11.4 g / dl , platelets 16410/l , no schistocytes , bilirubin 0.8 mg / dl and 450 ldh iu / l ) . ch50 determinations showed complement activity inhibition after each dose had been administered . coinciding with the improved of hsct - tma , the patient presented a clear response to his acute gvhd with disappearance of the diarrhea and bilirubin normalization ( figure 2 ) , although it has not been documented histologically . he was discharged eight weeks after the start of treatment ( post - transplant day 257 ) . unfortunately , one month later , the patient was readmitted by diarrhea ; a new colonoscopy showed intestinal gvhd relapse . the patient died two weeks after admission because of acute respiratory distress syndrome of unknown cause , with diffuse bilateral infiltrates , cardiomegaly and right pleural effusion in chest ct . plasma exchange , defibrotide , rituximab and basiliximab have been used with variable response rates . we started eculizumab instead of plasma exchange for the poor results of this treatment in hsct - tma cases associated with acute gvhd . in our case , the rapid clinical and analytical response to early treatment with eculizumab ( a humanized monoclonal antibody against complement fraction 5 ) supports the implication of the complement in its pathophysiology . the rapid response of a severe , refractory gvhd is noteworthy and suggests that the drug has a beneficial effect when used as coadjuvant therapy in this situation .

a 30-year - old man with acquired aplastic anemia underwent an hla - identical bone marrow transplant . he developed a grade iii acute graft versus host disease ( gvhd ) refractory to various lines of treatment . on post - transplant day 196 , he was diagnosed with stem cell transplantation - associated thrombotic micro - angiopathy ( hsct - tma ) and he received treatment with eculizumab 900 mg iv weekly for 4 doses followed by a single dose of 1200 mg 2 weeks later . after the first dose of eculizumab , the patient ceased to require transfusions and a progressive improvement in analytical parameters for microangiopathy was observed until their complete normalization . coinciding with the improved of hsct - tma , the patient presented a clear response to his acute gvhd with disappearance of the diarrhea and bilirubin normalization . he was discharged eight weeks after the start of treatment . unfortunately , one month later , the patient was readmitted for a gvhd relapse and he died two weeks later by an acute respiratory distress syndrome . in our case , the rapid clinical and analytical response to early treatment with eculizumab supports the implication of the complement in hsct - tma and suggests that the drug has a beneficial effect when used as coadjuvant therapy in acute gvhd .

pharmacovigilance programme of india ( pvpi ) launched in the country in the year 2010 to ensure the safety of medicine . indian pharmacopoeia commission functions as national coordination centre ( ncc ) for pvpi under the aegis of ministry of health and family welfare , government of india . currently 150 adverse drug reaction monitoring centres are recognized to monitor and report adverse drug reactions ( adrs ) in vigiflow , world health organization - uppsala monitoring centre ( who - umc ) web - based system to report adrs and participating in international drug monitoring program . the generated individual case safety reports ( icsrs ) are evaluated by ncc and submitted to umc . documentation grading and completeness of icsrs plays a vital role in quality of icsrs also quality icsrs facilitate the process of signal detection ncc plays a very important role in generating quality icsrs before submitted to umc . the present article describes about the documentation grading and completeness score of indian icsrs for the period of july 2011 to december 2014 . the icsrs received by ncc - pvpi for the period of july 2011 to september 2014 , were evaluated for their completeness by applying the factors such as age at onset , free text , gender , indication , outcome , primary source , report type , time to onset . after this preliminary screening at the ncc level , the reports were further assessed for documentation grading and completeness score of data by who - umc . who - umc uses the following formula [ equation 1 ] for calculating completeness score for icsrs . where , c = completeness score , j = drug - adr combination , i = is the field included in the score , wi = is the field weight and fi = is the field score , n = is total number of drug - adr combination . the factors ( fields ) were taken into consideration for documentation grading and completeness score with their weights is given in table 1 . the number of factor considered for completeness scoring by who - umc with their weight in equation 1 the documentation grading - completeness score for indian icsrs as per who - umc for the period of july 2011 to september 2014 is analyzed in this article . the progress of completeness scoring pattern of indian icsrs reported during the period of july 2011 to dec . the full score was given if the all the mandatory fields [ table 1 ] are addressed . in figure 1 the completeness score for each quarter starting from 3 quarter of 2011 to the 3 quarter of 2014 is depicted and the trend line of completeness score for each year given separately . it shows the growth of completeness score from 0.66 in september 2012 to 0.94 in september 2014 . completeness score progress in each quarter of year 20112014 completeness scoring by who - umc for indian icsrs . indian pharmacopoeia commission started function as national centre for pvpi since 15 april 2011 at that time major challenge was to initiate the culture of reporting rather than looking at the quality of reports . therefore the documentation grading and completeness score was around 0.6 during the period of year 2011 to 2012 . after 2012 ncc organized several awareness training programs on pharmacovigilance and subsequently the quantity and quality of reports were increased in 20122013 . during the year 20122013 ncc appointed technical associates to assist in the process of adr monitoring and assessing of adrs . these technical associates were trained on the concept of pharmacovigilance and to generate quality icsrs . therefore , the quality of reports was increased up to 0.94 out of 1 . since , to identify the signal from indian drug safety database , quality icsrs by addressing all required fields is essential .

the quality of individual case safety reports ( icsrs ) generated under pharmacovigilance programme of india ( pvpi ) plays a pivotal role in detecting a signal from indian drug safety data . currently , more than hundred thousand icsrs were generated under pvpi and reported to uppsala monitoring centre . the documentation grading and completeness score of indian icsrs were rapidly increasing , and the current score was 0.94 out of 1.0 . periodical training on emphasizing the quality icsrs is need of the hour .

the presence of adnexal masses during pregnancy ranges from 1 to 81 to 1 in 2,500 pregnancies , but only 3% of these masses are malignant . krukenberg tumor is an ovarian metastasis of a gastric tumor and accounts for 1%2% of all ovarian tumors . these tumors during pregnancy are even rarer , as the incidence of gastric cancer in women of reproductive age group is only 0.4%-0.5% . we present a case report of a woman 38 weeks pregnant with massive bilateral krukenberg tumors , which had an unfortunate outcome despite of treatment . a 30-year - old woman , gravida 3 para 2 , from a very remote community was referred at 38 weeks to our tertiary care center due to the presence a bilateral ovarian masses and shortness of breath . the patient complained of history of 1 year before the pregnancy with mild bloating and early satiety that was attributed to gastritis and gastroesophageal reflux , but despite the symptoms she never saught for medical attention . g / l ) , progesterone ( 22.29 nmol / l , normal range : 0.642.58 nmol / l ) , and 17-oh - progesterone ( 4.96 g / l , normal range : 0.401.02 g / l ) , and the tumor markers levels were 43.4 u / ml ( ca 199 ) , 1.7 ng / ml ( carcinoembrionic antigen ) 12.8 u / ml ( ca724 ) and 33.9 u / ml ( ca 125 ) , respectively . intraoperatively she was found to have two bilateral adnexal masses measuring 15 cm in its greatest diameter for the right ovary and 22 cm for the left ( figure 1 ) and also a 10 cm tumor in the major gastric curvature that resembled linitis plastica . she underwent an elective cesarean delivery and debulking surgery including bilateral oophorectomy omentectomy , and gastric tumor biopsy . histopathology of the gastric biopsy was consistent with the presence of poorly differentiated tumor cells corroborating the diagnosis of linitis plastica . since debulking does not improve the prognosis we decided against debulking . in the postoperative period the patient was started on chemotherapy with 6 cycles of chemotherapy using 5-fluorouracil ( 2000 mg / m ) and oxaliplatin ( 50 mg / m ) in a weekly schedule . despite all our efforts the patient succumbed to disease in 3 months . krukenberg tumor refers to gastrointestinal cancer metastatic to the ovaries , accounting for 1%-2% of all ovarian tumors . the eponym of this condition was given due by krukenberg initially described in 1896 and the criteria given were ( 1 ) the presence of a tumor in the ovary , ( 2 ) evidence of intracellular mucin secretion by the formation of signet cells , and ( 3 ) diffuse infiltration of stroma giving a sarcoma - like appearance . a search in english , spanish , and french literature revealed four reports of bilateral ovarian krukenberg tumors , with our case being the fifth in the literature . the cornerstone of the management of these tumors is the diagnosis of the gastrointestinal primary tumor , and also the prognosis worsens when the primary tumor is identified after the metastasis to the ovary is discovered . due to the rare nature of these tumors also due to the rarity of the condition it is not appropriate to comment on the effect of tumor on pregnancy and vice versa . there are only 3 cases of pregnancy - related krukenberg the tumors in the literature ; of these also 2 were detected postpartum , and this is the only tumor that is not associated to virilization . some authors are of the opinion that there might be an increased risk of acute abdomen secondary to tumor torsion or rupture . the role of debulking surgery and chemotherapy with platinum - based chemotherapy can be reasonable , and even relatively safe to be administered during pregnancy . but despite the interventions , usually the discovery of the presence of masses of the size that our patient exhibited the prognosis is poor as it generally represents an advanced stage disease , however , possible early detection with debulking surgery , possible hysterectomy with / without delivery , and platinum - based chemotherapy may improve the survival of these patients [ 5 , 6 ] .

objective . krukenberg tumors in pregnancy are very rare and their management can present a dilemma for the obstetrician gynecologist . case report . we present the case of a g3p2002 who presented to us and the 38 weeks gestation with bilateral massive krukenberg tumors . despite at surgery and chemotherapy she died 3 months postpartum . conclusion . early detection followed by surgery and chemotherapy could possibly result in a favorable outcome with such patients .

syncope is caused by transient diffuse cerebral hypoperfusion and is characterized by transient loss of consciousness with a rapid onset followed by spontaneous and complete recovery . clinical features of syncope may include myoclonic jerks which are often multifocal and asynchronous , convulsions , and urinary incontinence , making it difficult to differentiate from epileptic seizure by clinical features alone . significant fluctuations in cerebral perfusion pressure are prevented by autoregulation of cerebral circulation , but there may be conditions where such mechanism may not compensate adequately . cough syncope , a rare form of syncope , may be a result of transient failure of the cerebral autoregulatory mechanism to cope with sudden decrease in cerebral blood flow . we present an unusual case of recurrent cough syncope , which was initially diagnosed and treated as seizures , in the context of a left - sided glomus jugulare tumor , a benign paraganglioma . a 43-year - old right - handed woman with history of glomus jugulare tumor in the left jugular fossa with intracranial extension into the posterior cranial fossa was transferred from another hospital for recurrent seizure - like spells . she had a 90% surgical resection of the tumor done in 2011 followed by radiation therapy in september 2012 . her episodes occurred multiple times a day ( 7 per day on average ) during wakeful state . they were triggered by coughing ( usually a bout of cough ) and were characterized by staring and unresponsiveness as well as stiffening of the body with mild shaking of both upper extremities . she was diagnosed with epileptic seizures but continued to have episodes during treatment with the antiepileptic drugs ( aeds ) phenytoin , levetiracetam , and lamotrigine . escalation of aed therapy made her increasingly drowsy , and she was on all three aforementioned aeds at the time of presentation . her physical examination was remarkable for excessive drowsiness , mild dysarthria , right sixth cranial nerve palsy , mild hypertonia with hyperreflexia in the lower extremities ( left more than right ) , and bilateral ( left more than right ) ankle clonus . she had a lumbar puncture done at the outside hospital , and the opening cerebrospinal fluid ( csf ) pressure was reported to be 25 cm . blood work was also unremarkable except for mild anemia ( hemoglobin : 9.4 g / dl ) , mild hyponatremia ( 132 meq / l ) , and mild hypokalemia ( 3.1 meq / l ) . antiepileptic drug levels were within therapeutic range ( free phenytoin : 1.3 g / ml , levetiracetam : 5.9 g / ml , and lamotrigine : 2.3 g / ml ) . all started with a bout of cough when the patient was lying in bed ( in supine or in lateral position ) which was followed by brief ( less than a minute ) distal upper extremity tremor and subtle proximal upper extremity myoclonic jerks and prolonged unresponsiveness for up to 10 min . all of these episodes were associated with hypotension ( 7278/3147 mm of hg as revealed by continuous arterial pressure monitoring ) and bradycardia ( 5459 bpm ) . the eeg during the spells was characterized by generalized synchronous and asynchronous high amplitude 1- to 2-hz delta activity which progressed to generalized attenuation and then transitioned to generalized delta activity again with recovery ( fig . 1 ) . a head ct showed recurrence of the glomus jugulare tumor and communicating hydrocephalus . an external ventricular drain ( evd ) after placement of the evd , her drowsiness gradually started to improve , and episodes decreased in frequency to one per day . 3 showed an enhancing t2 hyperintense left skull base mass in the region of the left jugular foramen with extension into the posterior cranial fossa and below the base of the skull . brain imaging showed evidence of hydrocephalus that had increased compared with her previous brain imaging done 2 months back . her mental status continued to improve , and she had only one mild episode triggered by cough during the next two days before her discharge . repeat surgical resection of the tumor was recommended by the otolaryngology team , which the patient declined . based on the clinical features and eeg findings , the episodes observed in our patient are most consistent with cough syncope . the mechanism underlying cough syncope is not definitively established , but it is postulated that coughing increases intrathoracic and intraabdominal pressures leading to a transient increase in icp . increased icp , in turn , causes a decrease in cerebral perfusion pressure which , if it drops below a critical level , may result in global cerebral hypoperfusion leading to syncope . transient cerebral circulatory arrest has been demonstrated by transcranial doppler measurements during cough syncope . our patient also had a drop in blood pressure and heart rate but probably not sufficient to cause syncope by itself . cough syncope has been associated with posterior fossa mass lesions or tonsillar herniation and with hydrocephalus . it may be speculated that bouts of cough caused transient herniation of cerebellar tonsils obstructing csf flow that further contributed to the increase in icp during coughing . decrease in frequency of events following placement of evd to relieve icp lends support to this notion . paragangliomas are rare tumors of extraadrenal chromaffin cell origin that most commonly occur in the head and neck region . catecholamine - hypersecreting paraganglionomas are uncommon in the head and neck region , and most patients ( 95% ) with hypersecreting paraganglionomas have hypertension . hypotension accompanying syncope observed in our patient was not orthostasis - related ( the patient was always supine during spells ) and was most likely related to cough . identified a subset of patients with cough syncope who lacked a blood pressure overshoot ( expected response ) after the relief of straining during valsalva maneuver . the authors postulate that cough syncope in these patients might be the result of delayed recovery from hypotension that follows a paroxysm of cough , and this was likely contributing to global cerebral hypoperfusion in our patient . this case highlights the fact that cough syncope , a rare form of syncope , may be associated with intracranial mass lesions that indirectly exaggerate the increase in icp in response to cough . glomus caroticum tumor presenting as recurrent unexplained syncope and posterior fossa meningioma presenting as recurrent cough syncope have been described . recurrent cough syncope should trigger search for factors , including brain tumors , with the potential to cause transient elevation in icp . this case also illustrates an important role for ceeg monitoring with video in distinguishing syncope from seizures in cough syncope cases .

we present an unusual case of recurrent cough syncope in a 43-year - old woman , which was initially thought to be seizures . syncopal episodes were triggered by paroxysms of cough and were characterized by unresponsiveness and myoclonic jerks in her extremities . she had a left - sided glomus jugulare tumor that extended into the posterior cranial fossa with evidence of worsening communicating hydrocephalus on brain imaging . we postulate that bouts of cough produced increased intracranial pressure both by raising intrathoracic and intraabdominal pressures as well as by transient obstruction to cerebrospinal fluid flow secondary to intermittent tonsillar herniation during cough . this resulted in diffuse decrease in cerebral blood flow causing syncope . the patient 's syncopal episodes decreased in frequency once an external ventricular drain was placed followed by a ventriculoperitoneal shunt . search for factors that can increase intracranial pressure seems warranted in patients with recurrent cough syncope .

we performed a retrospective study among all patients who initiated individualized , community - based mdr - tb therapy from august 1 , 1996 , to march 1 , 2000 . the details of patient identification , enrollment , and treatment are described elsewhere ( 5 ) . . regimens generally included at least 5 drugs to which the infecting isolate was susceptible , and treatment duration was 1824 months . routine follow - up after completion of mdr - tb therapy included 1 ) routine smear microscopy and culture on sputum samples 1 month after completion of treatment and then every 6 months for 1 year ; 2 ) subsequent smear microscopy , culture , and clinical evaluation by a tb physician for any episode of potential tb symptoms , e.g. , a respiratory illness lasting > 14 days , hemoptysis , or weight loss of unclear cause ; and 3 ) continued contact with socios en salud ( the community - based organization working with the ministry of health on this mdr - tb treatment project ) through the network of health promoters , patient group therapy sessions , and a social assistance program . thus , loss to follow - up or undocumented medical attention for respiratory illness is rare . when reporting cohort outcomes , the mdr - tb working group recommends follow - up for 2 years from the time of treatment initiation when reporting cohort outcomes ( 6 ) ; however no international definition of long - term follow - up for mdr - tb cohorts exists . therefore , we defined long - term follow - up as twice the duration set forth by the mdr - tb working group . we conducted a chart review to determine tb - related symptoms and employment status of persons recorded at baseline by the intake physician before they received mdr - tb therapy . in addition to data obtained through routine patient follow - up as per program norms described above , socios en salud staff involved in the social assistance program , members of group therapy sessions , and health promoters were interviewed to obtain additional follow - up information about the patients , including income , employment status , and household information . a community health worker conducted home visits to interview all patients ; patients were questioned about current symptoms as well as their socioeconomic status . the study was reviewed and approved by the institutional review board at harvard medical school ; local institutional review was not required . among 120 persons enrolled in this study , 23 patients died during treatment , and two patients ( both of whom had defaulted from treatment ) were lost to follow - up . data are reported on the remaining 96 ( 80% ) patients who were alive at the time of stopping mdr - tb therapy . patients were followed for a median ( 95% confidence interval ) of 67 ( 4788 ) months after initiation of treatment and a median of 46 ( 384 ) months after completion of treatment . as summarized in the table , 86 ( 72% ) patients were considered cured , 9 ( 8% ) defaulted from treatment , and 1 ( 1% ) had failed treatment . among those who were considered cured at the time of treatment completion , 83 ( 97% ) are currently healthy . one patient relapsed 1 month after completion of treatment ; this patient refused retreatment and subsequently died of tb . two other cured ( culture - negative ) patients later died ( 1 of a narcotic overdose and the other of respiratory insufficiency ) . of the 9 patients who defaulted , 3 were culture - positive at the time they abandoned treatment . among the 9 defaulters , 5 died ( 4 from tb and 1 by suspected suicide ) , 2 are currently culture negative , and 2 were lost to follow - up since the time of treatment default . one patient was considered a treatment failure and , despite further retreatment regimens , subsequently died of tb . among 96 patients who were alive at treatment completion , 85 ( 89% ) thus , among the entire cohort of 120 patients enrolled during the study period , favorable long - term outcome was observed among 71% . four patients experienced long - term sequelae : hemoptysis caused by aspergilloma necessitating pulmonary resection ( 1 patient ) , bronchiectasis and recurrent respiratory infections ( 2 patients ) , and bronchopleural fistula after pneumonectomy ( 1 patient ) . of the 96 patients , 21 patients ( 22% ) received financial aid to pursue work or study , and 13 ( 14% ) have had children since they were cured . none of the persons employed before starting mdr - tb treatment have lost their jobs because of work disruption caused by their tb therapy . although mdr - tb presents a major challenge to tb control , effective treatment can result in cure . long - term follow - up is important for understanding the long - term efficacy of treatment and the overall impact of this disease on patients ' physical and socioeconomic well - being . first , unlike previous reports of high death rates associated with mdr - tb , most of our patients met the definition of cure upon completion of treatment . second , few patients who were cured at treatment completion had long - term sequelae or relapse . third , most patients were able to resume work or studies and participate in family roles as parents and caretakers . first , the cohort was small , and longer follow - up would be useful in determining if these indicators of physical and social recovery are sustained . second , several patients were lost to follow - up ; thus , the outcome of these patients is still not well characterized . finally , these results may not be applicable to other situations , where the socioeconomic situation determines , in large part , the ability of a patient to resume work and studies . with access to laboratory results to guide individualized therapy for persons with mdr - tb in the context of strong community - based support , the outcomes observed in peru are encouraging .

patients treated in peru for multidrug - resistant tuberculosis ( mdr - tb ) were followed - up for a median of 67 months . among 86 patients considered cured after completion of treatment , 97% remain healthy ; 1 patient relapsed . employment increased from 34% before treatment to 71% . we observed favorable long - term outcomes among mdr - tb patients .

initial laboratory values were : wbc 2900/l , anc 900/l , hemoglobin 12.0 g / dl , mcv 95 fl , and platelet count of 156,000/l . creatinine , ast , alt , bilirubin , tsh , iron , total iron binding capacity , ferritin , and vitamin b12 levels were normal . past medical history was significant for coronary artery disease , ulcerative colitis , and stage ii esophageal adenocarcinoma treated with neoadjuvant concomitant radiation plus carboplatin and paclitaxel followed by ivor - lewis esophagectomy . postoperative esophagocutaneous fistula formation led to long - term reliance on jejunal feeding for all caloric intake . his medications were lorazepam , aspirin , amiodarone , lisinopril , balsalazide , metoprolol , and vitamin b12 . amiodarone was discontinued . a repeat cbc after 1 month showed wbc 2600/l , anc 200/l , hemoglobin 12.7 g / dl , mcv 107 fl , and platelet count of 174,000/l . a second repeat cbc after 2 months following discontinuation of amiodarone showed : wbc 2900/l , anc 300/l , hemoglobin 11.0 g / dl , mcv 114 fl , and platelets 193,000/l . examination of a bone marrow aspirate and biopsy specimen revealed nor mocellular bone marrow with progressive trilineage hematopoiesis , mild erythroid hyperplasia , with megaloblastoid features , and vacuolated erythroid cells , highly suspicious for myelodysplastic syndrome ( figs1 and 2 ) . additional laboratory evaluation included rheumatoid factor 12 , esr 30 , crp 0.4 , serum zinc level 86 g / dl ( 60130 ) and serum copper < 10 g / dl ( 70140 ) . a cbc after 1 month revealed wbc 7600/l , anc 5400/l , hemoglobin 12.2 g / dl , mcv 120 fl , and platelet count 246,000/l . ceruloplasmin and cytochrome oxidase are both copper - containing enzymes essential for heme synthesis 3 . typical morphological findings in peripheral blood and bone marrow aspirate in copper deficiency can mimic myelodysplastic syndrome 1 . bone marrow aspirate in both conditions may show dysplasia in the erythroid precursors such as large size , nuclear multilobulation , and nuclear budding as well as the presence of ring sideroblasts . unlike myelodysplastic syndrome , the bone marrow aspirate in copper deficiency characteristically shows cytoplasmic vacuoles within erythroid and myeloid precursors 2 . furthermore , karyotyping in cases of copper deficiency does not reveal cytogenetic features characteristic of myelodysplastic syndrome . copper is present in common foods including meat , fish , nuts , seeds , and legumes , and therefore deficiency from dietary inadequacy is rare 4 . most copper absorption occurs in the stomach and proximal duodenum , although the more distal small intestine can also absorb copper . problems with copper absorption leading to deficiency should be considered in patients who have history of gastric bypass or gastrectomy , are receiving chronic parenteral nutrition or tube feeding , or on zinc supplementation and develop pancytopenia and/or myeloneuropathy . however , per the mayo clinic the daily recommended intake for adults is 1.52.5 mg / day . copper is available in both intravenous ( cupric chloride ) and tablet forms . in this patient , we hypothesize that the long - term use of jejunal feeding led to a nutritional deficiency of copper and consequent pancytopenia . currently , there is a national shortage of intravenous copper ; therefore we elected to attempt correction of his copper deficiency with higher doses of oral copper . copper deficiency represents a rare but treatable cause of anemia and neutropenia that may be mistaken for myelodysplastic syndrome . therefore , patients with a history of abdominal surgery who present with new found anemia and neutropenia with bone marrow biopsy showing dysplastic changes with no cytogenetic abnormalities should have copper levels checked . recognition of copper deficiency as the cause of these blood abnormalities is critical to appropriately treat this disorder .

key clinical messagecopper deficiency is a rare cause of pancytopenia that may be mistaken for myelodysplastic syndrome . cytoplasmic vacuolization in erythroid and myeloid precursors is found on bone marrow examination . patients with a history of abdominal surgery who present with anemia and neutropenia with dysplastic changes should have copper levels checked .

pulmonary veins ( pv ) were found to be important sources of ectopic beats for the initiation of atrial dysrrythmias.1 therefore , using diagnostic and therapeutic methods for assessing pv is recommendable . the present case demonstrates the feasibility of advancing a conventional electrophysiology ( ep ) catheter into the right upper pulmonary vein ( rupv ) via the retrograde aortic approach . a 13-year - old girl was admitted to our hospital in september 2008 with the electrocardiogram ( ecg ) manifestation of an ectopic atrial tachycardia ( at ) with a cycle length ( cl ) of 300 m sec . a p - wave morphology evaluation in standard 12-lead ecg revealed positive p - waves in d1 , inferior leads , and all precordial leads . echocardiography showed an ejection fraction of 35% and left atrium size of 27 mm with no evidence of valvular heart disease . electrophysiological study was performed using two quadripolar catheters in the right atrium and right ventricle : one decapolar in the coronary sinus and one halo catheter in the right atrium . bipolar electrograms were filtered at 30 to 500 hz . during at , the earliest right atrial activation was recorded at high posterior right atrium . at this site , radiofrequency ( rf ) ablation at this site could not terminate the tachycardia ; consequently , a 7 fr biosence - webster ( 34e67r ) ablating catheter was advanced into the left ventricle without the need for any long sheath via the retrograde aortic approach . the ablating catheter was then maneuvered to pass across the mitral valve , so that the left atrium was accessed . left atrial mapping was thereafter performed , which revealed that the area of interest was near the orifice of the rupv . the catheter was advanced into the rupv , and the recording of low amplitude fractionated electrical activity helped locate the focus of the arrhythmia . finally , the arrhythmia was ablated by applying some rf currents around the orifice of this vein ( outside the orifice ) and only one rf current ( 20 watt-60 c-40 seconds ) just at the origin of at . the total procedure time was 120 minutes , and the fluoroscopy time was 45 minutes . after ablation , the arrhythmia was not inducible with different stimulation protocols even during isoproterenol infusion . clinical follow - up showed no recurrence of the arrhythmia , there was no evidence of pulmonary vein stenosis , and ejection fraction had returned to normal . transseptal puncture is most often used for electrophysiological procedures in the left atrium.2 newer techniques and increased experience have improved the safety of this procedure and reduced some risk.3 although the success rate of transseptal puncture is usually above 95% , it occasionally fails 2 , 3 especially when repeat transseptal puncture is necessary . 4 in some patients , contraindication may also present . given the number of patients presenting for percutaneous left atrial ablation , it is important that second line , non - surgical approaches be found to access the left atrium in the cases where the transseptal approach is contraindicated . standard steerable catheters can be placed retrogradely on the atrial side of the mitral annulus for the ablation of accessory pathways , but to our knowledge there is no report about advancing the catheter into the rupv via the retrograde aortic approach . although there is previous a report on the ablation of focal left atrial tachycardia via a retrograde approach using remote magnetic navigation5 and another report on the cannulation of the pulmonary vein in five canines using a retrograde transaortic magnetic - enabled approach,6 we managed to establish an rupv access in this patient without resorting to the magnetically enabled catheter and successfully mapped the focal rupv tachycardia . this case demonstrates that rupv mapping is feasible via the retrograde aortic approach and it is also possible to ablate the arrhythmia using the same catheter and approach . this method should be tried only when there is an absolute contraindication for the transseptal approach by electrophysiologists who are experts in this method .

the use of diagnostic and therapeutic methods for assessing pulmonary vein due to its status as a main source of ectopic beats for the initiation of atrial dysrrythmias is strongly recommended . we report the case of a 13-year - old girl who was admitted to our hospital with the electrocardiogram manifestation of an ectopic atrial tachycardia . the focus of arrhythmia was inside the right upper pulmonary vein . the patient underwent successful ablation with a conventional electrophysiology catheter via the retrograde aortic approach . we showed that when the origin of atrial tachycardia is in the right upper pulmonary vein , it is possible to advance the catheter into this vein via the retrograde aortic approach and find the focus of arrhythmia . this case demonstrates that right upper pulmonary vein mapping is feasible through the retrograde aortic approach and it is also possible to ablate the arrhythmia using the same catheter and approach .

miller fisher syndrome ( mfs ) is characterized by the acute onset of external ophthalmoplegia , ataxia of cerebellar type , and the loss of tendon reflexes . it is considered a variant of guillain - barr syndrome ( gbs ) , because some patients who present with mfs progress to gbs . in contrast , patients who show drowsiness , brisk reflexes , extensor plantar responses and hemisensory disturbance are usually considered to have bickerstaff 's brainstem encephalitis ( bbe ) rather than mfs . the fact that bbe and mfs share a common autoantibody suggests that they are closely related . bickerstaff and cloake speculated that the etiology of bbe is similar to that of gbs because they found areflexia and cerebrospinal fluid albuminocytologic dissociation ; however , a case of overlapping mfs , gbs and bbe has not been fully reported . a 72-year - old woman developed diplopia and unsteady gait in the morning ( day 1 ) a week after an upper respiratory infection . she was admitted to our hospital due to weakness of both upper limbs on day 3 . on admission , the general physical examination was normal . on neurological examination , she had bilateral blepharoptosis . her eyeballs were fixed in the central position . she had weakness of both upper limbs . hematological investigation showed an elevated white blood cell count of 9,500/l ( segmented leukocytes , 86.5% ; lymphocytes , 10.5% ) , and her cerebrospinal fluid ( csf ) showed 2/l ( 100% mononuclear cells ) and 30 mg / dl protein . magnetic resonance imaging ( mri ) showed a normal brain . we diagnosed mfs on day 1 , and she developed gbs on day 3 . on day 5 , she became drowsy , with a worsened respiratory status and weakness in all four limbs . this condition was considered to be overlapping bbe . on day 12 , electroencephalography ( eeg ) showed background activity at 78 hz with no epileptiform discharges . on day 21 , a nerve conduction study of the right median nerve showed abnormal amplitude reductions in both the forearm and upper arm . motor nerve conduction velocity in the left median nerve was decreased ( 44.3 m / s ) . the sensory nerve action potential amplitude in both median nerves was reduced , and sensory nerve conduction velocity was normal . on day 51 , anti - gm1 igg , gd1a igg , gq1b igg , and gt1a igg antibodies were positive , and anti - gq1b igg antibody titer was the highest . she underwent intravenous immunoglobulin therapy from day 3 to 7 and seven sessions of immunoadsorption therapy from day 14 to 28 . on day 30 we added intravenous immunoglobulin therapy from day 34 to 38 . on day 40 , she began to open her eyelids and move both eyeballs . on day 54 , her consciousness became alert . on day 56 , she was taken off the respirator . on day 63 , she could stand up , but the ataxic gait remained . on day 90 , she changed hospital for rehabilitation ( fig . we report a case of overlapping mfs , gbs and bbe during the course of illness . neurologically , the responsible lesions were suggested as the pyramidal tract , nuclear or infranuclear of the ocular motor nerves , brainstem reticular formation , cerebellum and peripheral nerves of the limbs . we diagnosed her with mfs on day 1 , gbs on day 3 and overlapping bbe on day 5 . overlapping mfs / gbs and bbe / gbs syndromes have been reported [ 3 , 4 , 5 , 6 , 7 , 8 , 9 ] ; however , only two case reports have described overlapping mfs / gbs / bbe syndrome ( table 1 ) . arai et al . described a patient with bbe on day 1 , mfs on day 2 and gbs on day 5 in the presence of anti - gq1b antibody . stevenson et al . described a patient with bbe on day 1 , gbs on day 9 , and mfs on day 10 in the presence of anti - gm1 antibody . although these two patients underwent plasma exchange and intravenous immunoglobulin therapy , respectively , our patient required mechanical ventilation and underwent immunoadsorption therapy and two courses of intravenous immunoglobulin therapy . anti - gm1 igg , gd1a igg , gq1b igg , and gt1a igg antibodies were positive in our case . the abundant and synaptic - specific binding of anti - gq1b , -gt1a , and -gd1b ganglioside antibodies and the rich capillary supply in the human extraocular muscles may partly explain the selective paralysis of these muscles in mfs . some large neurons of the dorsal root ganglia were immunostained with anti - gq1b monoclonal antibody . gq1b also exists in nerve endings near the skeletal muscle spindle and anti - gq1b igg antibody may thus be associated with ataxia as well as ophthalmoplegia . our case supports a previous proposal by odaka et al . that bbe , mfs , gbs and acute ophthalmoparesis are all part of a continuous clinical spectrum , which is an antibody - mediated process . anti - gq1b antibody is detected in gbs , fs and bbe , so it is called anti - gq1b igg antibody syndrome , and is useful for understanding the etiological relationships among those illnesses . because of the similarities in the clinical presentation and autoimmune etiology of mfs and bbe , a terminology fisher - bickerstaff syndrome may be helpful for nosology . although the clinical picture , neurophysiology , and csf findings are usually sufficient to indicate therapies , the presence of antiganglioside antibodies is a useful guide to diagnose this group of conditions .

we report a 72-year - old woman with overlapping miller fisher syndrome ( mfs ) , guillain - barr syndrome ( gbs ) and bickerstaff 's brainstem encephalitis ( bbe ) . she developed diplopia and unsteady gait a week after an upper respiratory infection on day 1 . she had weakness of both upper limbs on day 3 and became drowsy , and her respiratory status worsened on day 5 . neurologic examination revealed ophthalmoplegia , ataxia , symmetrical weakness , areflexia , and consciousness disturbance . we diagnosed her with mfs on day 1 , gbs on day 3 and overlapping bbe on day 5 . she underwent immunoadsorption therapy and two courses of intravenous immunoglobulin therapy . ten months after onset , her symptoms had fully recovered . anti - gm1 igg , gd1a igg , gq1b igg , and gt1a igg antibodies were positive . our case supports the notion that mfs , gbs , and bbe are all part of a continuous clinical spectrum , which is an antibody - mediated process .

a 67-year - old caucasian woman presented with a lesion on her left sural region which consisted of an erythematous purplish round plaque , surrounded by a the patient had brown hair and was classified as skin phototype 3 according to fitzpatrick 's classification . she had been suffering from type 2 diabetes for 12 years and was on treatment with oral hypoglycemic agents ; moreover , she was paraplegic as a consequence of poliomyelitis . at clinical examination the epidermis was hyperplastic , hyperkeratotic and showed a typical column of parakeratosis corresponding to the cornoid lamella ; no granular layer was seen beneath the parakeratotic column . the papillary dermis showed an unspecific , mild lymphocytic infiltrate , in addition to dilated capillaries and incontinentia pigmenti . treatment consisted of oral tretinoin ( 0.5 mg / kg / day for 2 months ) . at the end of the treatment the lesion slightly improved ; therefore , the patient decided to stop the therapy , since the lesion had always been almost asymptomatic . a 49-year - old man presented with an oval plaque on the latero - dorsal side of the left foot ( fig . because of an eroded papula which had appeared within the lesion several weeks earlier , the general practitioner had hypothesized a squamous cell carcinoma , and therefore the patient had been referred to our clinic . at first observation , the characteristic ridge - like border oriented towards the diagnosis of porokeratotic eccrine ostial and dermal duct naevus . histopathological examination showed the cornoid lamella , but the pathognomonic dermal eccrine duct beneath the parakeratotic column was not detected . in accordance with the dermopathology team the plaque probably derived from the confluence of discrete papules in the final geographic appearance . the eroded area included in the plaque was diagnosed as a benign and probably trauma - induced lesion and treated with a 8% zinc oxide emulsion . its complete regression within about 3 months confirmed such a diagnosis . at four - year follow - up five clinical variants of porokeratosis are recognized : classic porokeratosis of mibelli , disseminated superficial actinic porokeratosis , porokeratosis palmaris et plantaris disseminata , linear porokeratosis , and punctate porokeratosis . the histological hallmark is the cornoid lamella , a thin column of hyperproliferative parakeratotic keratinocytes . the pathogenesis is unclear ; it has been postulated that it results from proliferation of an abnormal clone of keratinocytes . several risk factors of porokeratosis have been identified , including genetic inheritance , ultraviolet radiation , and immunosuppression . the lesions of porokeratosis characterized by a keratotic rim tend to expand slowly in a centrifugal fashion , leaving in their wake a zone of atrophy . at one moment in time , a malignancy can rarely occur at the lesion site ; in order of frequency , squamous cell carcinoma , bowen 's disease or basal cell carcinoma have been described in the literature . it is controversial whether this occurrence is just a coincidence of photo - induced , immunosuppression - induced damage or the expression of a familial genetic trait .

porokeratosis is a disorder of keratinisation whose pathogenesis is yet unclear . it has been postulated that it results from the proliferation of an abnormal clone of keratinocytes , triggered by several factors , such as immunosuppression or prolonged ultraviolet exposure . various clinical forms are recognized whose common denominator is a keratotic ring surrounding a central zone of atrophy . the histological hallmark is the cornoid lamella , a thin column of hyperproliferative abnormal keratinocytes . we describe two cases of porokeratosis . a 67-year - old woman with an erythematous purplish round plaque surrounded by a keratotic border that had appeared 6 years previously on the left sural region was diagnosed as giant porokeratosis . a 49-year - old man presented with small papules coalescent in an erythematous oval plaque on the lateral side of the left foot consistent with linear porokeratosis .

percutaneous thrombolysis of hemodialysis fistulas became a well - established and promising technique after the development of the pharmaco - mechanical technique of pulse - spray thrombolysis . all these years , the catheter - directed interventions have been widely used for thrombolysis . thrombolysis is done using infusion catheters placed inside the grafts / thrombosed vein , followed by balloon angioplasty or stenting if required . this new technique of needle - directed thrombolysis can be done as an opd procedure / bedside with usg guidance . it does not require the use of fluoroscopy or the infusion catheters and wires , thereby significantly reducing the cost of the procedure and hospital stay . after the initial usg examination ( ge logiq e , usa ) of the thrombosed segment , 5000 units of heparin was injected intravenously . under aseptic precautions and usg guidance , multiple 26 g needles were inserted into the thrombosed vein or graft and were connected to three - way stop cocks with short connectors [ figure 1 ] . the thrombolytic solution was prepared by dissolving 2.5 lakh units of urokinase ( u frag , bharat serum and vaccines ltd , india ) in 10 ml of heparinized saline ( 1000 u heparin ) as described by valji et al . manual pulse injections of 0.2 - 0.3 ml aliquots were applied to each needle about every 30 s with a 1 ml syringe . forceful and rapid injections were essential to adequately penetrate the thrombus and to achieve uniform thrombolysis . intermittent usg screening was done to assess the progress of the thrombolysis [ figures 2 and 3 ] . if the flow was not restored at the end of the injections , another 2.5 l urokinase was prepared and used . after recanalization , low - molecular - weight ( lmw ) heparin was administered for the next 2 - 3 days to prevent re - thrombosis due to the residual clot burden and also to manage the small risk of pulmonary embolism . the arteriovenous ( av ) fistula / graft was ready to be used for dialysis almost on the same day . photograph showing multiple 26 g needles inserted into the arteriovenous fistula ( avf ) graft in the arm . three - way stop cocks with connectors are connected to the needles doppler image showing hypoechoic thrombus ( horizontal arrow ) within the avf graft ( vertical arrows ) . no colour flow seen within the graft post procedure doppler showing restored flow ( horizontal arrow ) within the arteriovenous fistula graft with few residual clots ( vertical arrow ) in the periphery usg - guided needle - directed thrombolysis of av fistula / grafts is a promising alternative to the catheter - directed thrombolysis .

the incidence of end - stage renal disease is significantly increasing and most patients who require renal transplantation are undergoing hemodialysis through tunneled / non - tunneled dialysis catheters and arteriovenous fistulas . the greatest disadvantage of the hemodialysis access is the limited durability of the arteriovenous fistulas and grafts , which , on average , remain patent for <3 years , but are the lifeline for hemodialysis patients . catheter - directed interventions are successful in re - establishing flow in more than 80% of thrombosed hemodialysis fistulas and have become the treatment of choice for failing or thrombosed accesses . however , these interventions are expensive and require a tertiary center with well - equipped labs . the novel technique of needle - directed thrombolysis is less expensive and can be done on an opd basis in any primary healthcare setup . to the extent we have searched , no such technique has been published so far in the english literature .

intracranial subdural empyema ( isde ) is a life - threatening condition that is localized between the dura and arachnoid mater . it can stem from multiple etiologies , including sinusitis , mastoiditis , infected cranial procedures , or dental complications [ 13 ] . repeated studies have supported that prompt neurosurgical intervention is of utmost importance in patients with isde as a lack of treatment can progress to septic shock and death [ 1 , 4 ] . clinical manifestations of patients with isde can be subtle ; however , patients can also present with neurological symptoms such as headaches , seizures , vomiting and mass effect . this non - specificity in its clinical presentation often makes isdh difficult to diagnose . here , we present a rare case of isde mimicking a recurrent chronic subdural hematoma ( csdh ) emphasizing the significance of obtaining early magnetic resonance images ( mr ) of the brain for early diagnosis and treatment to achieve the optimal outcome . patient is a 59-year - old male without a significant medical history presenting with a worsening headache and the right lower extremity paresis for the past three weeks . computed tomography ( ct ) scans of the brain on admission showed bilateral subdural hematomas ( fig . a left subdural drain was placed , which led to the marked improvement of symptoms and the csdh ( fig . a day later , the drain was removed , and the patient with discharged with the resolution of his symptoms ; however , a week following the initial procedure , the patient was re - admitted to the hospital with concerns for increased drowsiness and word finding difficulty . the repeated ct of the head demonstrated what was thought to be a stable left csdh ( fig . the patient was discharged home the next day as the work - up at that time was insignificant and symptoms were considered secondary to the opioid use . figure 1:ct of the head without contrast demonstrated csdh , denoted by the asterisks , at the initial presentation ( a ) , after the procedure ( b ) , 7 days later ( c ) and 4 weeks later , when the csdh has enlarged ( d ) . ct of the head without contrast demonstrated csdh , denoted by the asterisks , at the initial presentation ( a ) , after the procedure ( b ) , 7 days later ( c ) and 4 weeks later , when the csdh has enlarged ( d ) . after 4 weeks of his initial procedure , patient again returned to the hospital with the right - sided hemiparesis and aphasia . patient underwent a left craniectomy for evacuation of what was thought to be a hematoma . unexpectedly , after opening of the dura , a large amount of purulent materials was encountered and the diagnosis of subdural empyema was made . he was later switched to intravenous nafcillin for the duration of 6 weeks , as operative cultures were positive for methicillin - sensitive staph aureus ( mssa ) . after the 4 months of empyema evacuation , patient underwent a left cranioplasty with an artificial implant to cover the craniectomy defect . isde is a deadly infection that accounts for up to 25% of all intracranial infections . the mortality rate from isde has decreased significantly from 40 to 10% in the last 60 years due to neurosurgical advances in treatment modalities , imaging and in improved antimicrobial treatment regimens . the treatment of the isde includes an evacuation of the empyema and antibiotics based on the sensitivity result from the culture . the most common organism isolated from isde has been streptococcus and staphylococcus species [ 1 , 5 ] . the most important aspect of managing an isde is the early diagnosis and treatment to prevent severe sequelae . one of the challenges that clinicians face in treating an isde is that patients , such as the patient in this report , can present with subtle clinical manifestations and without classical signs of infection such as fever . in reported studies , the so - called classic triad of fever , headache and vomiting , only accounts for less than 20% of patients with isde [ 1 , 5 ] . however , there are also reports of having a prior neurosurgical procedure as a risk of developing an isde [ 1 , 2 ] . the probability of developing an isde after multiple intracranial procedures was upwards of 50% in some studies , while it was only 0.441.8% after one neurosurgical procedure [ 2 , 6 ] . our patient had undergone a prior subdural hematoma evacuation four weeks prior to developing the isde . in fact , ryu et al . reported that patients developing isdes after a neurosurgical procedure often have subtle signs and symptoms with less than 35% of patients having a fever and only 15% having headaches from isdes . while the cranial ct is widely used in the initial evaluation , it has a false negative result of 6% for diagnosing an isde , which can be readily identified by the mr of the brain with a much higher sensitivity and specificity [ 2 , 4 , 5 ] . it is highly important to make an early diagnosis of isde as an undiagnosed and untreated isde can lead to severe complications such as seizures , septic sinus thrombosis , infarctions , septic shock and even death . given the lack of sensitive and specific clinical signs of isde , a low threshold to obtain an early mr of the brain is highly important to facilitate a prompt diagnosis for early treatments especially in patients who have undergone prior procedures and presented with findings on the ct of the brain suggesting of a recurrent chronic subdural hematoma .

intracranial subdural empyema ( isde ) is a life - threatening condition . the risk for isde increases in patients that have undergone prior intracranial procedures . the non - specificity in its clinical presentation often makes isde difficult to diagnose . here , we present a rare case of isde mimicking a recurrent chronic subdural hematoma , emphasizing the significance of obtaining early magnetic resonance images of the brain for early diagnosis and treatment to achieve the optimal outcome .

volvulus is a type of bowel obstruction involving the twisting of bowel loops around the axis of the mesentery , causing strangulation and eventual necrosis of the intestines . its etiologies are categorized as primary , without associated underlying anatomic abnormalities , and secondary , due to congenital malrotation of the gut , adhesions after prior surgery , and anatomic bands . among the pediatric population , one in 500 children are born with malrotation annually , 85% present within the first 2 weeks of life . in adults , malrotation is extremely uncommon . although there are reports that found many cases of adult malrotation without overt symptoms . it is estimated that 1 in 6000 individuals are affected and it is rarely the cause of volvulus ; rather , it is most often due to adhesive bands which form after abdominal surgery . the standard treatment of malrotation volvulus in the pediatric population is ladd 's procedure ; however , repair of incidental malrotation in adults remains controversial . there is very little published regarding progression of malrotation into volvulus after abdominal surgery for another etiology . this paper describes a case of adult malrotation which progressed to volvulus shortly after abdominal surgery . it also reviews clinical presentations and diagnostic tools , discusses the recommended management , and considerations when addressing of this rare malady . our patient was a 19-year - old male who presented to the emergency room with 1-day history of new onset right lower quadrant abdominal pain , denying fever , nausea , vomiting , change in appetite or bowel habits . his vital signs were stable and physical examination revealed right lower quadrant tenderness without rebound or guarding . scan diagnosed appendicitis with malrotation , the appendix was located deep to the umbilicus . due to the location of the appendix , the malrotation was not addressed because there are no definitive recommendation for repair of this finding incidentally in adults . additionally , as the surgeon was not a formally trained pediatric surgeon , the decision was made to proceed with appendectomy alone . the patient was discharged home without complication the following day . on post - operative day 5 , the patient returned with nausea , bilious vomiting and constipation . a ct scan revealed dilation of the stomach and proximal small bowel , indicating a malrotation volvulus . the patient 's post - operative course was unremarkable and he was discharged 5 days later . malrotation remains the most common etiology of volvulus in neonates , though a rare cause in adults . however , adults have less specific symptoms . some patients presented with acute or chronic abdominal pain , while our patient denied any pain at all . regardless , the differential diagnosis of an adult presenting with any combination of these symptoms is wide , making the diagnosis of malrotation volvulus particularly challenging . , however , abdominal x - ray is typically first obtained , though there is a significant rate of false negatives . a ct scan is commonly utilized revealing sequestration of the small bowel and colon to the right and left sides of the abdomen , respectively , and an inversion of the superior mesenteric vessels . the vast majority of malrotation is incidentally found on ct scan . in pediatric patients with asymptomatic or symptomatic malrotation , more specifically , there is a scarcity of information regarding acute progression of malrotation into volvulus after abdominal surgery for a separate cause . only a handful of other cases [ 36 ] describe patients to have appendicitis with an incidental malrotation . and , among those who underwent surgery , as diagnostic imaging improves and is utilized more , incidental diagnosis of malrotation in adults is becoming increasingly common , thus investigating treatment of incidental malrotation in adults will become increasingly significant . perhaps , this complication would have been avoided if a pediatric surgeon was consulted for the initial operation . or , perhaps , this was an unlikely complication , and correction should not have been considered at all . as more cases like ours are published , surgical considerations are likely to be affected .

among the pediatric population malrotation is the most common cause of volvulus . this paper describes a case of adult malrotation , which progressed to volvulus shortly after appendectomy . it also reviews clinical presentations and diagnostic tools , discusses the recommended management , and considerations when addressing of this rare malady .

cutaneous leishmaniasis ( cl ) is a protozoan disease caused by the protozoa leishmania subtypes and transmitted by the bite of an infected female sand - fly . it appears after a variable period of time ( from months to years ) in the same area as , or very close to the old scar of , a previous acute lesion healed apparently . herein , we present a case of a large and horrifying example of lrc of the lips mimicking granulomatous cheilitis . an 8-year - old , healthy , syrian boy was admitted to our outpatient clinic of dermatology with a swelling and disfigurement of his lips existing for the last 4 years . the initial lesion had started as asymptomatic papules , 0.5 - 1 cm in diameter on cheek and lip when he was two years old . after the treatment with intralesional meglumine antimoniate ( glucantime ) in syria at that time , the lesions disappeared ; however , cribriform scarring on the lip and cheek remained . two years later , new lesions began as papules , 1 cm in diameter around the cribriform scars on the lips and cheeks . dermatologic examination showed the presence of a severe swelling and purulent draining , crusty infiltrated granulomatous plaques on the lower and upper lips . a 5 cm cribriform scarring and 1 cm papule on this scar were seen on the left cheek [ figures 1a and b ] . laboratory tests revealed hb 10.3 g / dl ( 12.2 - 18.1 g / dl ) , hct 30.5% ( 37.7 - 53.7% ) , sedimentation rate 70 mm / h ( 0 - 12 mm / h ) , c reactive protein 42.4 mg / dl ( 0 - 5 mg / dl ) . abundant intra and extracellular leishmania amastigotes were determined in the smear prepared from the lesion with giemsa stain [ figure 2 ] . histopathology showed foamy histiocytes and leishmania parasites within the cytoplasm of macrophages in the epidermis and a dense dermal mixed type inflammatory cell infiltrate composed of lymphocytes and foamy histiocytes with multinucleated giant cells [ figure 3 ] . the patient was treated with 20 mg / kg / day systemic meglumine antimoniate intramuscularly , for 20 days . because the healing was slow , he was also prescribed oral fluconazole 5 mg / kg / day for 4 weeks . the lesion improved markedly at the first month of the treatment [ figures 1c and d ] . clinical pictures of the patient before treatment ( a and b ) and after 1 month of the treatment ( c and d ) abundant intra and extracellular leishmania amastigotes in the smear ( giemsa stain , 100 ) leishmania parasites ( black arrows ) in the cytoplasm of macrophages ( h and e , 400 ) in lrc , the actual cause of reactivation of the disease is unclear ; insufficient treatment or the use of non - effective drugs may be possible causes of recurrence , but the most identified theory is a defect in the t - lymphocyte activation by the protozoa and the inability of the macrophages to kill all amastigotes . clinically , differential diagnosis includes lupus vulgaris , bacterial infections , pseudolymphoma and squamous cell carcinoma and when the lesions found on the lips syphilitic chancre , granulomatous cheilitis , melkersson - rosenthal syndrome and foreign body granuloma should be considered . the treatment for lrc includes systemic therapy with pentavalent antimony , alone or in combination with allopurinol , and amphotericin b , and local therapy with intralesional antimonials , cryosurgery , or excision . in children to our knowledge , lips of lrc mimicking granulomatous cheilitis have not been reported so far . the difference of our case from other cases is that our patient is a child , he had a very giant lesion and lesion was located on the lip . we consider there is unusually abundant amastigotes because of unavailability of a sufficient therapy due to existing social situations . in summary , we want to report a case with a large and horrifying example of lrc of the lips mimicking granulomatous cheilitis . the absence of pcr examination is a limitation of our study . considering the different forms of the disease nature , any unusual skin lesion located on the lips in an endemic area should always be investigated for cl and thus , clinicians should have a high level of suspicion to make a diagnosis of lrc and an appropriate treatment . considering the different forms of the disease nature , any unusual skin lesion in an endemic area should always be investigated for cutaneous leishmaniasis.clinicians should have a high level of suspicion to make a diagnosis of leishmaniasis recidiva cutis and an appropriate treatment . considering the different forms of the disease nature , any unusual skin lesion in an endemic area should always be investigated for cutaneous leishmaniasis . clinicians should have a high level of suspicion to make a diagnosis of leishmaniasis recidiva cutis and an appropriate treatment .

leishmaniasis recidiva cutis ( lrc ) is an unusual form of acute cutaneous leishmaniasis . herein , we present a case of lrc of the lips mimicking granulomatous cheilitis . an 8-year - old , syrian child admitted with a swelling and disfigurement of his lips for 4 years . abundant intra and extracellular leishmania amastigotes were determined in the smear prepared from the lesion with giemsa stain . histopathology showed foamy histiocytes and leishmania parasites within the cytoplasm of macrophages in the epidermis and a dense dermal mixed type inflammatory cell infiltrate composed of lymphocytes , foamy histiocytes with multinucleated giant cells . on the basis of anamnestic data , the skin smears results , clinical and histopathologic findings , lrc was diagnosed . the patient was treated with meglumine antimoniate intramuscularly and fluconazole orally . cryotherapy was applied to the residual papular lesions . the lesion improved markedly at the first month of the treatment .

wernicke 's encephalopathy is an acute neuropsychiatric disorder due to the lack of thiamine ( vitamin b1 ) , generally occurred in chronic alcoholics1 . in addition to alcoholism , other causes include continuous long term administration of glucose - only intravenous ( iv ) , high calorie administration by parenteral nutrition , hyperemesis gravidarum , starvation of long duration , hemodialysis , gastrointestinal surgery , and cancer2 . if a patient with wernicke 's encephalopathy remains untreated , severe amnesic deficits , korsakoff psychosis , and death could result from the acute disease4 . recently there are many studies showing that wernicke 's encephalopathy develops in other diseases , for example malnutrition , malabsorption , aids , and malignant tumors , which result in thiamine deficiency5 . this case shows that poor oral intake and malnutrition due to masticator space and a parapharyngeal space abscess resulted in thiamine deficiency which led to wernicke 's encephalopathy . we report the case with a literature review and discuss wernicke 's encephalopathy and the management of patients with eating difficulty . a 79-year - old female patient with hypertension was referred to the oral and maxillofacial surgery department of sanggye paik hospital because of severe pain and swelling of the left cheek and difficulty of opening the mouth that had been present since about 10 days prior to presentation . she had received treatment at a local clinic , but the patient 's symptoms did not improve . we confirmed that the patient had pain , swelling , and induration of the left cheek , including the left temporalis area . her vital signs were unstable ( systolic blood pressure , 70 mmhg ; diastolic blood pressure , 30 mmhg ; pulse rate , 110 beats / min ; respiratory rate , 28 breaths / min ; body temperature , 37 ) . the patient did not complain of dysphagia or dyspnea . on computed tomography with contrast enhancement , 1 ) the patient 's condition improved after incision , drainage , and antibiotic therapy . after 2 weeks her condition was improved after an additional incision and drainage of the palatal area . however , the patient was unable to eat meals normally . after 2 more weeks , we consulted with the department of neurology because the patient displayed sudden confusion and seizures . we suspect that prolonged malnutrition due to abscess formation resulted in thiamine deficiency , in turn inducing wernicke 's encephalopathy . if it is depleted , cerebral hypoperfusion and functional disorders in the blood brain barrier may occur within 2 weeks5 . the diagnosis of wernicke 's encephalopathy is based on clinical features and fast reversal of symptoms after the administration of thiamine6 . however , in clinical situations , acute wernicke 's encephalopathy is often difficult to detect6 . magnetic resonance imaging can be useful for diagnosis7 , as it shows t2 signal hyper - intensity in the pulvinar of the thalamus , hippocampus , and mammillary body , and in the periaqueductal area368 . bilateral paramedian thalamic infarction , cytomegalovirus encephalitis , primary cerebral lymphoma , multiple sclerosis , behcet 's disease , central pontine myelinolysis or extrapontine myelinolysis , lyme disease , mitochondrial disorders such as leigh 's disease , and variant creutzfeldt - jakob disease should be considered in the differential diagnosis7 . if the patient 's symptoms improve dramatically after supplementation with thiamine , his or her condition may be confirmed as wernicke 's encephalopathy6 . because wernicke 's encephalopathy can be avoided by the administration of thiamine supplements , lindberg and oyler9 recommended that all comatose patients with unknown cause of coma should receive parenteral thiamine supplementation before definite identification of thiamine deficiency . the administration of thiamine supplements , either orally or parenterally , is recommended in patients with alcoholism for the prophylaxis of wernicke 's encephalopathy . oral thiamine supplements are recommended for patients who are at low risk of wernicke 's encephalopathy10 . it is standard practice to assess electrolyte levels , but clinicians are likely to overlook the quantities of meals that patients actually ingest . the patient 's state of nutrition should be confirmed not only by lab findings , but also through interviews with both the patient and ( if relevant ) guardians . if patients have difficulty swallowing , additional treatment should be offered such as the placement of a levin tube or provision of total parenteral nutrition . our case illustrates that wernicke 's encephalopathy may result from malnutrition due to the presence of abscesses in the masticator space and parapharyngeal space . clinicians should keep in mind that patients who have difficulty eating due to conditions affecting the oral cavity are at risk of wernicke 's encephalopathy due to malnutrition .

wernicke 's encephalopathy is a fatal neurological disease caused by thiamine deficiency . many reports indicate that wernicke 's encephalopathy is caused by malnutrition . we report the case of a 79-year - old female patient who had a left masticator space and parapharyngeal space abscess who was diagnosed with wernicke 's encephalopathy . she reported problems while eating due to the presence of the abscess , but the true quantities of food she was ingesting were never assessed . clinicians have a responsibility to provide adequate nutritional support by ensuring that patients receive adequate nutrition . clinicians should also keep in mind that wernicke 's encephalopathy may occur in patients who experienced prolonged periods of malnutrition .

lung cancer is one of the absolute priorities in oncology because of its mortality , incidence , social and economic impact . the lack of clinical signs at the early stages and therapeutic inefficiency for advanced stages , lead to the evidence that early diagnosis should reduce lung cancer related mortality , as in early stages 70% of cases can be treated successfully with surgery . this justifies the need to study the impact on survival of a lung cancer screening program with stronger motivation than those used for assessing and spreading the standard protocols for breast , prostate and colonic cancer screening . opportunities for screening for lung cancer have been debated for years , but several studies have shown that conventional chest x - ray and sputum examination did not satisfy the primary criteria of a screening test [ 4 , 5 ] , until the value of low - dose spiral ct in detecting small parenchymal lesions was demonstrated by some reports [ 6 , 7 ] . the diagnostic imaging technique to be applied was stated in the first elcap report , which describes the ct tumour detection rate as four times higher than chest x - ray , and by studies demonstrating similar accuracy of low - dose spiral ct and conventional ct in detecting pulmonary nodules . while awaiting the new promising techniques of molecular biology , multidetector computed tomography [ 911 ] , with the use of low dose protocols , is nowadays widely recognized as the best technique for lung tumour screening . the high accuracy , together with the low biological and economical costs , justify the success of the protocols for the early detection of lung cancer by low - dose spiral ct . this is confirmed by the growing number of ongoing studies all over the world . although the various screening campaigns sometimes apply different inclusion criteria , the epidemiologic data identify the high risk population in asymptomatic subjects of both genders , over 50 years of age , smokers or ex - smokers ( within the previous 1015 years ) of at least 20 cigarettes a day for at least 20 years . in order to reduce the cost / benefit ratio of the test , lung cancer screening is based on the application of low - dose protocols to avoid exposure to excessive amounts of radiation . the low - dose technique ensures good spatial resolution and high contrast resolution , and guarantees high sensitivity for the detection of lung nodules up to 2 mm in diameter [ 12 , 13 ] . ct protocols differ according to different scanners : we report an example applied to lightspeed ct 16-rows ( general electric , milwaukee , wl , usa ) : thickness of acquisition 2.5 mm ; standard reconstruction filter ; 40 ma ; 140 kv ; rotation time 0.8 s ; speed 35 mm / rotation ; collimation 10 mm . the introduction of multidetector ct , with the use of thin sections , resulted in an increase in the number of lung nodules detected [ 10 , 11 ] , thus making the definition of diagnostic algorithms very relevant . the most important parameter to define the diagnostic work - up is the nodule diameter and its changes over time . although there is no clinical evidence , it is widely accepted that lung nodules less than 5 mm in size can be safely checked at 1 year intervals : this follow - up schedule allows identification of early stage malignancies , while avoiding unnecessary anxiety and useless radiation in the majority of those being screened [ 14 , 16 ] . the evaluation of the nodule characteristics ( solid , partially solid , non - solid ) , depending on the ability to obscure the lung parenchyma , and its morphologic appearance [ 1820 ] are important to determine the grade of suspicion . apart from follow - up to evaluate volume doubling time , diagnosis can be reached by pet or pet / ct , ct enhancement with contrast media via i.v . several authors have suggested different protocols for the management of lung nodules [ 2527 ] : we suggest the algorithm shown in fig . 1 . the preliminary results of observational studies show high sensitivity of the technique in detecting lung nodules . in the elcap report , 23% of patients had one or more non - calcified nodules ; the european institute of oncology study detected 284 nodules in 199/1035 subjects ( 19% ) . a large number of these nodules were benign and the prevalence of lung cancer was 2.7% and 1.1% in the two series . at the second year ct , the incidence of lung cancer was 0.59% and 1.1% [ 16 , 28 , 29 ] ( table 1 ) . the results from the observation of the stage at diagnosis are encouraging : 81% of patients had a stage i tumour , with a good prognosis ( survival rate at 5 years of 63% for stage ia and 46% for stage ib ) . epidemiologic data in the us show that the diagnosis at stage i is made in only 20% of cases not resulting from screening projects . the percentage of invasive procedures performed for benign lesions ranges from 21% to 55% . the high prevalence of benign nodules detected by ct and reported as false positive results is the major criticism against programs for early detection of lung cancer . the use of a dimensional cut - off should reduce the number of false positives and the recall rate with no major impact on the cancer detection rate . some authors state that the screening results are affected by an overdiagnosis bias , as ct reveals a significant percentage of non - aggressive tumours that would never cause the death of the patient , because of slow growth and other risk factors related to age and smoking habits . this could explain the increase of survival in cancer patients enrolled in screening campaigns , without a corresponding reduction of mortality . early diagnosis does not necessarily result in a reduction of mortality ; there are no definitive data on the outcome of the subjects enrolled in screening programs . at the end of their screening project at the mayo clinic , swenson and colleagues did not demonstrate any significant difference in mortality when comparing their results with those from a study performed in the 1970s by conventional chest x - ray . our data on 1035 volunteers , enrolled in a screening project in 20002001 , with only 30 subjects lost to follow - up at 5 years , show 42 lung cancers detected , 9 deaths from lung cancer , 11 for other cancers , 7 for cardiovascular diseases and 6 for other causes , resulting in a mortality rate of 3.2% in 5 years . the costs are extremely variable from one study to another , and data reported range from 2500 to 2,300,000$per year of saved life [ 3436 ] . to assess the real efficacy of ct in reducing lung cancer related mortality and to introduce this test into clinical practice , we are all waiting for the results of the randomized clinical studies recently started all over the world . prevalence , incidence and percentage of stage i tumours detected by low - dose ct in published lung cancer survey studies

the lethality of lung cancer is related to the advanced stage at diagnosis . initial studies have demonstrated that screening computed tomography ( ct ) is effective in diagnosing lung cancer at an earlier stage when compared with current clinical practice , however the best clinical approach for screening detected nodules has to be defined . the population to be identified as high risk should be over 50 years of age and should have smoked at least one pack / day for 20 years . ct protocols should use multidetector ct , low dose and a 2.5 reconstruction interval . diagnostic work - up on detected nodules should be designed according to size and consider ct at 3 or 12 months to evaluate doubling time , ct enhancement , pet / ct and/or fnab or vats . the prevalence of lung cancer in the screened population is 1.1%2.7% , and the incidence is 0.2%1.1% . eighty - one percent of cancers are diagnosed in stage i. the percentage of surgery performed for benign lesions ranges from 21% to 55% . in our series , the overall mortality rate was 3.2% in 5 years . the results of randomized clinical studies , when available , will assess the real efficacy of ct in reducing lung cancer related mortality .

a 31-year - old nonprofessional rugby player , with no medical history experienced acute substernal chest pain with dyspnea , shortly after a rugby game . lungs were clear to auscultation and cardiac examination was normal without murmurs , rubs or gallops . initial electrocardiogram ( ecg ) revealed sinus rate with segment ( st ) elevation in v2 to v6 leads with inferior mirror image . shortly after ecg recording , he presented a sudden cardiac arrest related to ventricular fibrillation , successfully treated by external defibrillation ( 200j then 300j ) . emergency cardiac catheterization showed a proximal left anterior descending ( lad ) artery dissection extending into mid - artery , with thrombolysis in myocardial infarction ( timi ) 1 flow . a cypher ( cordis corporation , warren , nj ) drug - eluting stent ( 3.5 mm 28 mm ) was successfully implanted at the proximal lad and timi 3 flow was immediately achieved . initial high sensitivity troponine t level was normal , but subsequently increased and peaked at 4700 iu / l ( normal < 14 ) with creatine phosphokinase ( cpk ) peak of 3550 iu ( normal 39 - 308 ) . echocardiography showed moderate hypokinesis of the septum and the anterior wall , with left ventricular ejection fraction ( lvef ) of 55% . he was discharged home 8 days later and remained event - free at 12 months follow - up . a 33-year - old amateur rugby player , without medical history , was admitted for dizziness , nausea with vomiting within 2 h following a rugby game . during this game , he had been struck sharply in the mid - sternum by an opponent 's shoulder . at initial examination initial ecg revealed sinus rate with st elevation in inferior leads with lateral mirror image [ figure 1 ] . emergency cardiac catheterization with radial access revealed a proximal right coronary artery ( rca ) dissection extending to mid - rca with timi 3 flow [ figure 2 ] . a promus element ( boston scientific corporation , natick , ma ) , drug - eluting stent ( 3.5 mm 38 mm ) was successfully placed in the artery . iu / l ( normal < 14 ) with cpk peak of 4750 iu ( normal 39308 ) . he was discharged home 7 days later and referred for enrollment in a cardiac rehabilitation program . inferior segment elevation myocardial infarction with antero - lateral mirror image right coronary artery dissection from proximal to mid - segment a 31-year - old nonprofessional rugby player , with no medical history experienced acute substernal chest pain with dyspnea , shortly after a rugby game . lungs were clear to auscultation and cardiac examination was normal without murmurs , rubs or gallops . initial electrocardiogram ( ecg ) revealed sinus rate with segment ( st ) elevation in v2 to v6 leads with inferior mirror image . shortly after ecg recording , he presented a sudden cardiac arrest related to ventricular fibrillation , successfully treated by external defibrillation ( 200j then 300j ) . emergency cardiac catheterization showed a proximal left anterior descending ( lad ) artery dissection extending into mid - artery , with thrombolysis in myocardial infarction ( timi ) 1 flow . a cypher ( cordis corporation , warren , nj ) drug - eluting stent ( 3.5 mm 28 mm ) was successfully implanted at the proximal lad and timi 3 flow was immediately achieved . initial high sensitivity troponine t level was normal , but subsequently increased and peaked at 4700 iu / l ( normal < 14 ) with creatine phosphokinase ( cpk ) peak of 3550 iu ( normal 39 - 308 ) . echocardiography showed moderate hypokinesis of the septum and the anterior wall , with left ventricular ejection fraction ( lvef ) of 55% . he was discharged home 8 days later and remained event - free at 12 months follow - up . a 33-year - old amateur rugby player , without medical history , was admitted for dizziness , nausea with vomiting within 2 h following a rugby game . during this game , he had been struck sharply in the mid - sternum by an opponent 's shoulder . at initial examination , initial ecg revealed sinus rate with st elevation in inferior leads with lateral mirror image [ figure 1 ] . emergency cardiac catheterization with radial access revealed a proximal right coronary artery ( rca ) dissection extending to mid - rca with timi 3 flow [ figure 2 ] . a promus element ( boston scientific corporation , natick , ma ) , drug - eluting stent ( 3.5 mm 38 mm ) was successfully placed in the artery . iu / l ( normal < 14 ) with cpk peak of 4750 iu ( normal 39308 ) . he was discharged home 7 days later and referred for enrollment in a cardiac rehabilitation program . inferior segment elevation myocardial infarction with antero - lateral mirror image right coronary artery dissection from proximal to mid - segment coronary artery injury resulting in myocardial infarction is an extremely rare complication of blunt chest trauma and is mostly observed in high - speed motor vehicle accidents . suspected mechanism of ca dissection is shearing of the coronary vessel wall , caused by dramatic acceleration / deceleration forces , producing intimal tearing . the lad ca is most commonly affected ( 76% ) , followed by the rca ( 12% ) and circumflex artery ( 6% ) . the higher incidence of lad ca involvement may relate to its proximity to the chest wall . the rca may be more prone to injury when blunt chest trauma occurs during ventricular systole , a time when this vessel lies in a more vulnerable anterior position . the recognition of traumatic ca dissection may be difficult since symptoms can be wrongly attributed to chest wall pain . blunt chest trauma with myocardial contusion may also produce ecg changings ( as st elevation or depression , q waves ) , myocardial enzyme elevation or wall motion abnormalities on echocardiography . moreover , a significant delay ( up to 7 days ) between blunt trauma and dissection can be observed and may result from gradual enlargement of the false lumen . cases of sports - related traumatic ca dissection are very unusual and have been reported following waterskiing , bicycle accidents and after collisions during basketball , soccer or rugby games . prognosis in survivors of ca dissection varies according to the amount of ischemic myocardium and the residual lvef . cases of sudden death in sports - related blunt chest trauma that are attributed to commotio cordis ( ventricular arrhythmia induced by blunt chest trauma ) may , in fact , represent cases of coronary dissection and myocardial infarction . as with any myocardial infarction , however , optimal management of traumatic ca dissection is uncertain , most likely because of the rare incidence of the condition . emergency transfer to the cath lab is pivotal to confirm ca and rule out myocardial contusion . management options include medical therapy with anticoagulation and antiplatelet agents , angioplasty ( with or without stenting ) and ca bypass surgery . thrombolytic therapy should be avoided in the setting of wall chest trauma because of potential catastrophic consequences . thrombolysis has also been associated with the extension of the dissection by promoting hemorrhage into the false lumen .

coronary artery ( ca ) dissection following blunt chest trauma is a life - threatening and rare event . its occurrence in the setting of a contact sport like rugby is even less common . we report on two cases of young adult presenting with segment elevation myocardial infarction related to ca dissection following rugby game . both were successfully treated with stent implantation . we discuss the mechanism , diagnosis , and optimal management of blunt chest trauma - induced ca dissection .

the most common site is in the infrascapular region beneath the muscular tissue . in magnetic resonance imaging ( mri ) , a preferable noninvasive investigation , elastofibromas typically appear as a solitary , poorly circumscribed , heterogeneous , soft - tissue mass . in the absence of a reliable preoperative diagnostic investigation , histopathological examination of biopsy a 46-year - old female presented with the complaint of diffuse soft - tissue swelling in the back . the swelling was present since the last 2 years and was gradually increasing in size . the mri was reported as showing focal ill - defined altered signal intensity lesion in right posterolateral chest wall deep to serratus anterior muscle [ figure 2a d ] . the patient underwent fine needle aspiration ( fna ) cytology examination that was reported as lipoma . grossly , the lesion was irregular , not capsulated , and measured 7 cm 5 cm in size . right inferior subscapular swelling ( a and b ) mri image showing focal ill - defined altered signal intensity lesion in the right posterolateral chest wall deep to serratus anterior muscle . the lesion is isointense to muscle in t1 and t2wi images ( axial ) ( c and d ) t1 and t2wi images ( sagittal ) of the lesion cut section of the mass with grayish - white areas microscopically , the lesion was poorly circumscribed and showed an admixture of collagenous tissue with mature adipocytes . additionally , there were many fragmented ones , which gave the appearance of beads on a string . there was no area of necrosis or increased mitotic activity [ figure 4a and b ] . the verhoeff 's elastic stain confirmed the elastic nature of the fibers [ figure 5a and b ] . ( a ) lesion with admixture of collagen fibers and adipose tissue with characteristic elastic fibers ( hematoxylin and eosin 10 ) ( b ) the rounded and beaded elastic fibers ( hematoxylin and eosin 40 ) ( a ) verhoeff 's elastic stain showing elastic fibers ( verhoeff 's elastic stain 10 ) ( b ) the rounded and beaded elastic fibers ( verhoeff 's elastic stain 40 ) based on the characteristic histopathological features and histochemical staining with verhoeff 's stain highlighting elastic fibers , the lesion was diagnosed as a case of elastofibroma . but there is geographic variation and elastofibroma is common in the japanese population . in indian medical literature , though , it has a marked predilection for elderly females , it is often reported in the age group ranging from 35 - 94 years . the typical location is infrascapular region , occurring predominantly on the right side , but , is bilateral in 30% of the cases . majority of the patients are asymptomatic . rarely , when symptoms are present , they are pain , scapular snapping , limitation of motion , clunking sensation in the shoulder adduction - abduction movement . the present case is a 46-year - old female who presented with a slow growing nontender swelling in the right subscapular region . elastofibroma dorsi ( ed ) is used as a synonym and it is the name given to those lesions in the periscapular region . some authors consider it to be a true neoplasm , whereas others presume it a result of reactive hyperproliferation of connective tissue exposed to repetitive minor trauma or friction between the lower scapula and the underlying chest wall . according to nagamine et al . , who reported a large series from japan , genetic factors are implicated in the pathogenesis . theories suggest that they may be caused by abnormal elastogenesis or by degeneration as a secondary process , or may be by a combination of both processes . plain radiographs do not show specific changes apart from a possible soft - tissue signal intensity or elevated scapula . mri is the investigation of choice and shows a heterogeneous fibrous mass of intermediate density with entrapped signals of higher intensity . computed tomography ( ct ) is less sensitive for visualizing the strands of fatty tissue . on positron emission tomography ( pet)-ct radiotracer accumulation of the hypermetabolic tumor has been described . due to its muscle - like appearance in nearly all the imaging procedures , diagnosis of elastofibroma the lesion was located on the right side only and no lesion clinically or radiologically was identified on the other side . it is difficult to distinguish elastofibroma from other benign and malignant soft - tissue tumors of thoracic wall due its rarity . fnac is not recommended as these lesions are hypocellular in nature and in frozen sections , the elastic fibers are difficult to detect . in the absence of a definitive preoperative diagnosis , there is a chance of inappropriate treatment in the form of radical surgery . the clues for the presumptive diagnosis of elastofibroma are advanced age of the patients , the typical localization , female gender , or bilateral manifestation . for a definitive diagnosis of elastofibroma , macroscopically , elastofibroma appear as an irregular , poorly defined , unencapsulated , fibroelastic mass with firm , rubbery consistency . histologically , they are dermal in location , unencapsulated , hypocellular masses composed of fibrocollagenous strands mixed with adipose tissue . immunohistochemically , elastofibromas stain positively for vimentin but negatively for smooth muscle actin , s-100 , desmin , and p53 . because the histopathology and verhoeff 's stain findings were classical cases of elastofibroma , we did not perform immunohistochemistry . ultrastructurally , the elastic fibers appear as granular or fibrillary aggregates of electron - dense material surrounded by an amorphous matrix with scattered microfibrils . the differential diagnoses include various benign and malignant soft - tissue tumors such as nuchal fibroma , fibrolipoma , fibroma , spindle cell lipoma , and pleomorphic lipoma , aggressive fibromatoses , sarcoma , and subcutaneous metastasis . however , the characteristic findings of elastofibroma such as the presence of elastic fibers in a collagenized fibrous tissue with entrapped adipose tissue helps in arriving at the correct diagnosis . these tumors rarely recur and malignant transformation has not been described so far ; therefore , in asymptomatic lesions there is no need for excision . the role of diagnostic biopsy is to exclude malignancy and to reassure the asymptomatic patient that no radical surgical treatment is necessary .

elastofibromas are rare benign , soft - tissue slow - growing tumors seen predominantly in elderly females . the most common location is the infrascapular region . these benign tumors require resection only in symptomatic cases . we present a case of elastofibroma in a 46-year - old female . she presented with gradually increasing soft - tissue swelling of 8 cm 6 cm in the right inferior subscapular region for the last 2 years . she underwent excisional biopsy and the histopathology was reported as elastofibroma . microscopically , the mass showed numerous characteristic eosinophilic , beaded elastic fibers . these fibers were highlighted by the verhoeff 's elastic stain . we present this uncommon case to emphasize the important role of histopathology in diagnosis . a definitive diagnosis helps to avoid unnecessary wide and radical resection .

clavicle stress fractures are particularly rare , with only 16 reported cases.[18 ] they have been described in patients following radical neck dissection , following coracoclavicular ligament reconstruction and few sports - related cases . stress fractures are injuries that typically occur in sport and are usually related to overuse . we report a case of a clavicle stress fracture non - union secondary to weight - training . this is the first reported case of a sports - related non union stress fracture of the clavicle . a 47-year - old male who had been performing regular weight training for more than ten years , had increased the intensity of training over a two - week period . he consulted with his family physician , who requested an x - ray investigation , which was reported as normal . with the pain not abating , he was referred for a ct scan that revealed a linear defect confirming a stress fracture over the lateral third of the clavicle [ figure 1 ] . ct scan of left clavicle delineating site of stress fracture ( arrowed ) he was treated symptomatically with oral analgesic and anti - inflammatory medication . after several weeks he was prescribed narcotic analgesics for the pain . four months after the onset of symptoms , despite reduced activity and the use of narcotic analgesics , he continued to have pain and was referred to the authors ' sports medicine clinic . the history confirmed the patient 's regular weight training program , usually five to six days a week , with increased intensity prior to the onset of symptoms . clinically there was a tender swelling over the lateral third of the clavicle [ figure 2 ] . a repeat x - ray confirmed the diagnosis of a hypertrophic non - union stress fracture of the clavicle [ figure 3 ] . photograph showing clinical prominence of non - union stress fracture of left clavicle radiograph of stress fracture indicating hyperdrophic non - union the patient was advised to have surgery , which he underwent . at surgery the hypertrophic bone and the non - union a seven - hole lcdc plate ( synthes usa ) was contoured and applied antero - superiorly across the fracture site [ figure 4 ] . allomatrix injectable putty ( wright medical technology , arlington , tennessee , usa ) , was applied around the fracture site . the post surgery x - rays revealed acceptable reduction and fixation [ figure 5 ] . post - operatively a sling was worn for four weeks and thereafter the patient underwent rehabilitation . the final follow - up was at 11 months from the non - union surgery . intraoperative photograph of an lcdc plate over fracture site postoperative radiograph of internal fixator over fracture range of movement and strength were clinically symmetrical at final follow - up . aerobic exercise ( running and cycling ) was permitted at four months following the non - union surgery . light , but progressive gym activities were permitted six weeks later , as the patient did not experience any adverse effect from the aerobic activity . the clinical outcome was therefore deemed favorable , with the patient recovering fully from the injury . the pectoralis major and deltoid muscles produce a downward force , whilst with the trapezius and sternocleidomastoid muscles will oppose this movement . repetitive activation of these muscles may result in enough strain to overcome remodeling and create micro architecture disruption , eventually leading to a stress fracture . lower limb stress fractures are more common than upper extremity stress fractures and may be diagnosed on history , physical examination and imaging ( including radiographs , bone scans , mri and ct scan ) . those stress fractures that do occur in the upper limb are usually related to upper - limb dominated activities , such as throwing sports , swimming , baseball pitching and tennis . clavicle stress fractures are particularly rare , with only 16 cases reported and only seven related to sporting activities [ table 1 ] . in patients diagnosed with clavicle fractures , the differential diagnosis should include traumatic or pathological fracture , neoplasm , infection , metabolic bone disease and bone dysplasia . sprots related clavicle stress fracture we report a case of clavicle stress fracture with non - union in a middle - aged male after accelerated weight training . this case is particularly unique in that not only is it one of a rare form of stress fracture , it is only the second reported case related to weight lifting and the first reported sports - related stress fracture with resultant non - union . clinicians treating athletes should have a high index of suspicion because although rare , clavicle stress fractures should feature in the differential diagnosis of shoulder or clavicle pain .

stress fractures are relatively uncommon sports injuries and when they do occur , are mostly found in the lower limb . stress fractures of the clavicle are particularly rare , having been described in a number of non - sport related pathologies , such as nervous tics and post radical neck dissection . in sport , there have only been seven cases reported in the literature . we report on a clavicle stress fracture in a 47-year - old male , partaking in recreational weight lifting activities . this is the first reported case of a non - union stress fracture of the clavicle . the patient underwent an open reduction and internal fixation and made a full recovery .

we monitored mers - cov specific antibody levels in 11 pairs of camel dams and their calves at monthly intervals over the course of 1 year postparturition . these animals were born and raised on a closed commercial camel dairy farm in the united arab emirates that had a strict animal health and biosecurity program . animals are kept in open paddocks and are grouped according to the age of their calves and production stage . despite high standards of hygienic husbandry and biosecurity , the transmission of pathogens within the farm can not be completely eliminated . calf pairs investigated in this study were kept in different fenced compartments within 100150 m from each other . however , all these animals were kept together with other dam nasal swab specimens were taken from all 11 mothers and calves , and serum samples were obtained through jugular vein puncture . blood cells were removed immediately after collection , and samples were stored at 20c until testing . serum samples and nasal swabs were taken at the day of parturition , at 1 week and 1 month postparturition , and then at monthly intervals until june 2015 . mers - cov rna was detected through amplification of gene targets as described previously ( 12 ) . specific igg and neutralizing antibodies were determined by elisa and microneutralization test as described previously ( 5,10 ) . in general , maternal igg antibodies in camels are not acquired via the transplacental route but through the intake of colostrum during the first 24 hours postparturition ( 13 ) . after 24 hours , antibody levels in the dam s milk decrease rapidly , and igg levels in calves serum cease to rise ( 13 ) . this pattern is reflected for mers - cov in this study . on the day of parturition , high levels of mers - cov specific antibodies were observed in all dams , whereas no antibodies were detected in calves ( figure , panel a ) . at day 7 postparturition , however , all 11 camel calves had high mers - cov specific antibody levels . these levels declined during the first 6 months postparturition , whereas igg in dams remained constantly at high levels . the neutralizing activity of igg was confirmed by microneutralization tests on serum samples from calves collected 23 months , 56 months , and 1 year after birth ( figure , panel b ) . after 56 months , serum from 6 of 11 calves had completely lost their neutralizing activity . the remaining 5 calves had low neutralizing titers , ranging from 1:20 to 1:40 ( figure , panel b ) . specific igg antibody levels in dromedary camel dam calf pairs , united arab emirates , 20142015 . a ) mers - cov spike protein s1-domain based elisa ratios of individual samples ( dots ) plus mean ( horizontal line ) and sd ( error bars ) over the course of 1 year for dams ( black dots ) and calves ( gray dots ) . ratios were calculated by dividing the elisa optical density at 450 nm of each sample by that of a calibrator to minimize interassay variation . dashed lines indicate cutoff values for positive ( ratio 1.1 ) and borderline ( ratio 0.8 ) samples . mers - cov infection indicates time points where mers - cov rna was detected in camels . b ) neutralizing titers of individual samples from camel calves at selected time points determined by microneutralization test ( dots ) . for comparison , elisa ratios for the selected time points are shown in parallel as a boxplot diagram ; box represents 50% of the complete dataset from the first to the third quartile , and whiskers are drawn according to the tukey method . mers - cov , middle east respiratory syndrome coronavirus ; nt , neutralization test . to examine possible correlations between antibodies and mers - cov infection , we examined shedding of mers - cov in nasal swab specimens . during the first 5 months postparturition , mers - cov rna was detected sporadically on days 7 and 30 ( table ) . infectious virus was isolated only from calves but not dams . at 6 months postparturition , when calves showed the lowest antibody titers , mers - cov rna was detected in 2 of 11 calves ( table ) , indicating active mers - cov infection . all calves seroconverted during the following weeks ( figure , panel a ) ; 1 calf had meanwhile been euthanized because of a congenital forelimb deformity . calf pair was excluded after month 6 because the calf was euthanized for an unrelated condition . 5 mers - cov positive specimens from adult camels were from 4 individual animals . 9 mers - cov positive specimens from camel calves were from 6 individual animals . this longitudinal study of natural mers - cov infections in camels confirms assumptions from preliminary cross - sectional studies in camels ( 4,5,11 ) . serum antibodies might not have been sufficient to mediate protective immunity in the respiratory tract because dams and calves were sporadically infected even as maternal antibodies peaked at day 7 postparturition . these findings are consistent with earlier reports of mers - cov reinfection in seropositive camels ( 4,11 ) . nevertheless , our findings of virus isolation from calves but not dams are in line with earlier observations of reduced viral load in seropositive camels on reinfection ( 11,14 ) , indicating that neutralizing antibodies might not provide sterile immunity but could still reduce the viral replication level . the predominance of infection in young animals is better explained by the absence of immunity than by other factors , such as social group density , because the number of newborn camels in our study was negligible compared with the overall size of the herd at the farm . moreover , young camels were not kept in a contiguous group but in small compartments , where they had more contact with their mothers than with other young animals . calves are likely to have been infected through fomites or through adult animals shedding low quantities of virus . our findings have important implications for the prevention of human infections through camel herd management and camel vaccination . camel breeding , even if involving a small number of newborn animals , should be classified as a risk for human acquisition of mers - cov . the greatest risk should be assumed for the time after the fourth month of life until the first wave of natural infections , which should occur during the first year of life in camels raised in mers - cov endemic regions . measures for the prevention of infection , such as personal protective equipment , hand hygiene , and environmental sanitation , as applied on the farm in our study , should be sufficient for protection , given that no human mers - cov illnesses occurred among staff and only 2 of 300 workers with regular contact with camels had detectable mers - cov specific igg antibodies . because persons with underlying disease and the elderly show the most severe outcomes of mers - cov infection , these groups should generally avoid farms where camel calves are being raised . our results also suggest that studies dealing with application and efficacy of mers - cov vaccines should be modified . a first study involving immunologically naive animals showed a sharp decline in virus secretion after vaccination ( 14 ) . however , future vaccination trials should also investigate the effect of preexisting maternal antibodies on vaccine efficacy ( 15 ) .

knowledge about immunity to middle east respiratory syndrome coronavirus ( mers - cov ) in dromedary camels is essential for infection control and vaccination . a longitudinal study of 11 dam calf pairs showed that calves lose maternal mers - cov antibodies 56 months postparturition and are left susceptible to infection , indicating a short window of opportunity for vaccination .

the widespread use of central venous catheters during the last few years has been reported to be associated with many complications , some of them lethal . this paper audits our immediate complications noted during the procedure of percutaneous central venous catheterization at a tertiary referral center . one hundred and three consecutive percutaneous central venous catheters inserted in 70 children within the age group of 012 years over a period of 18 months were studied . the common types of catheters used included leaderflex 22 g ( vygon , gurgaon , india ) , leadercath 20 g ( vygon ) and primicath ( vygon ) . the type of catheter inserted depended on the age of the patient and the indication of insertion . all lines were inserted by a single person ( resident in the department of paediatric medicine ) using the seldinger technique by a uniform protocol initially under supervision and later independently by the bedside . each catheterization was followed by an x - ray to confirm the position of the tip of the catheter . data collected included age , weight , sex , hemodynamic status , indication for placement , site of catheterization , number of attempts and immediate complications . percentages , test of proportion and odds ratio were applied to compare the two groups . of the 103 catheters , 65 ( 63.1% ) were inserted in neonates , 24 ( 23.3% ) in infants and 14 ( 13.6% ) in children between 1 and 12 years of age . there were 86 ( 83.5% ) males and 17 ( 16.5% ) females . among the neonates , 53.8% were term and catheterization was performed in neonates with a mean weight of 2.276 0.847 kg , the smallest being 0.5 kg , and the range of weight of the non - neonates was 3.06021.0 kg , with a mean weight of 7.315 4.144 kg . of the neonatal catheterizations , 53.8% were in hemodynamically unstable patients and 47.4% among the non - neonatal catheterizations were in unstable patients . also , 64.6% of the catheterizations were on neonates with ventilatory support while 42.1% of the non - neonates were on ventilator during catheterization . the most common indication was failure of peripheral venous access in both neonates and non - neonates ( 63.1% and 76.3% , respectively ) . routes of insertion included internal jugular ( 89.2% in neonates and 81.6% in non - neonates ) , femoral vein ( 4.6% in neonates and 18.4% in non - neonates ) and basilic vein ( 6.2% in neonates and none in non - neonates ) . indication for insertion of central venous access devices the complications related to the procedure of insertion and various factors influencing them are summarized in tables 2 and 3 . a single attempt at catheterization was successful in 70.7% of the neonates and in 79.3% of the non - neonates . the overall success rate of the procedure was 92.9% and on only five occasions ( four neonates and one non - neonate ) , percutaneous insertion failed and a venesection was required . success rate ( after one or multiple attempts ) was seen to be increasing with increasing age , with a 90.2% success rate in neonates and 100% in children in the age group of 512 years . the immediate complications , namely pneumothorax , hydrothorax and subcutaneous extravasation , were noticed only in neonates at a rate of 1.5% each . both pneumothorax and extravasation developed in the hemodynamically unstable patients ( complication rate of 2.9% ) while hydrothorax developed in the hemodynamically stable neonate ( complication rate of 3.3% ) . similarly , pneumothorax developed in a patient with ventilatory support ( complication rate of 2.4% ) while hydrothorax and extravasation developed in patients with spontaneous breathing ( complication rate of 4.3% ) . also , none of the cases of pneumothorax , hydrothorax and extravasation developed in neonates in whom catheterization was achieved in single attempt . in case of hydrothorax and extravasation ( complication rates of 8.3% ) , catheterization was achieved in two attempts while multiple attempts at catheterization were made in case of pneumothorax ( complication rate of 4.2% ) . line - related complications and influencing factors in total catheterizations distribution of immediate complications of pcvcs in neonates according to the influencing factors hemodynamic monitoring , fluid infusion , administering hypertonic solutions and also complex procedures like hemodialysis are the benefits of a central venous catheterization . cannulation can be carried out in major central veins like the internal jugular , external jugular , subclavian , basilic or femoral veins . of the 103 catheterizations in our study , 63.1% were carried out in neonates and 36.9% in non - neonates , majority in critically unstable patients and the most common indication being non - availability of suitable peripheral venous access followed by need for prolonged venous access . there were a total of 41 complications with a rate of 39.8% at the time of insertion . of the 41 complications , 38 were failed attempts at cannulation . of all complications , the majority ( 75.6% ) this was due to their smaller veins , resulting in an increase in the number of attempts taken to catheterize the vein . the success rate of percutaneous central venous catheterization was seen to be increasing with increasing age , which is similar to that seen by venkataraman et al . the rate of immediate complications in this group is comparable to other reported series.[357 ] eichelberger et al . and filston and grant have reported lower rates of pneumothorax in infants , but their studies did not include hemodynamically unstable patients . hemodynamically unstable patients had a slightly higher rate of immediate complications than those with normal blood pressure , mainly because of the greater number of attempts made to catheterize the vessel in these patients . the low incidence and absence of statistical significance none of the patients ( neonates / non - neonates ) in the present study developed hemothorax , air embolism , subclavian artery puncture , cardiac arrhythmias or cardiac perforation , which were seen in a few of the studies . no statistically significant conclusion could be drawn regarding the bearing of insertion site and type of catheter used on the occurrence of complications at catheter insertion as the numbers of catheters inserted by routes other than the internal jugular vein were few and so were the catheters other than polyurethane catheters . it was observed that complications were fewer with increasing familiarity with the procedure with two - third of the complications being seen in the first half of the cannulations and one - third in the later half .

objective : to study the incidence of immediate complications associated with percutaneous central venous catheterization.materials and methods : a total of 103 central venous catheters were inserted in 70 children over a period of 18 months , governed by a uniform protocol . sixty - three percent of the catheters were inserted in neonates , 23.3% in infants and 13.6% in children between 1 and 12 years of age.statistical analysis used : software spss version 15.results:there were a total of 41 insertion - related immediate complications , of which 75.6% were in neonates . neonatal age , hemodynamic instability and more number of attempts to catheterize the vein had a higher risk of insertion - related problems . there was no mortality directly as a result of the procedure.conclusion:in our practice , it was observed that complications were fewer with increasing familiarity with the procedure . hence , percutaneous central venous catheterization is a safe procedure when performed in experienced hands .

hemophagocytic lymphohistiocytosis ( hlh ) is a rare but lethal disease , which may be due to genetic defects , as in familial hlh caused by mutations in fhl1 hplh1 , fhl2 prf1 ( perforin ) , fhl3 unc13d ( munc13 - 4 ) , fhl4 stx11 ( syntaxin 11 ) , fhl5 stxbp2 ( syntaxin binding protein 2)/unc18 - 2 , or immunodeficiency syndromes as chediak - higashi , hermansky pudlak , griscelli or x - linked lymphoproliferative syndrome . on the other hand , acquired hlh is associated with malignancies , autoimmune diseases , and infections with the epstein barr virus ( ebv ) being the main trigger . a 22-month - old girl presented to us with 8-day fever ( 40c ) , and mild cough . on examination , she had hepatosplenomegaly . past medical history included failure to thrive , but normal neurological development . she was stunted , pale , and had non - tender hepatomegaly of 5 - 6 cm ; the spleen was palpable 4 cm below the left costal margin as confirmed by abdominal ultrasound . the wbc count was 5100/mm ( anc 1200/mm ) , hb 9.1 g / dl , and platelets 47.000/mm . ferritin was 1134 ng / ml . soluble il-2 receptor ( scd25 ) was 3183 ui / ml . epstein - barr virus ( ebv ) serology yielded seroreactivity ( positive igm and igg anti - vca ) with negative igg anti - ebna . the patient worsened clinically , with persistent malaise , progressive splenomegaly , and petechial rash on her chest and legs . subsequent investigations were wbc 4800/mm ( anc 800/mm3 ) , hb 6.8 g / dl and platelets 27000/mm , triglycerides 686 mg / dl , ferritin 3849 ng / ml , and scd25 3596 ui / ml with normal clotting tests as well as perforin expression by flow - cytometry . hair microscopy was not performed because clinical features of albinism were not present neither in our patient nor in her family . the following day , leishmania polymerase chain reaction was done where leishmanial dna was detected by nested pcr . ) this was confirmed on a bone marrow aspirate as well . standard therapy with liposomal amphotericin b ( 3 mg / kg / day iv on days 1 , 2 , 3 , 4 , 5 , 14 , and 21 ) was given . the patient completed hlh-2004 protocol , except for 2 doses of etoposide due to clinical and laboratory improvement without evidence of underlying primary hlh . at 30 month follow up , there is no evidence of relapse . as cd107a degranulation assay was not performed ( just normal intracytoplasmic pattern of perforin staining ) , hlh comprises a rare group of disorders caused by activation of cd8 + t cells and macrophages and high - level secretion of pro - inflammatory cytokines , characterized by persistent fever , cytopenia , hepatosplenomegaly , and hemophagocytosis in bone marrow , lymph nodes , liver or spleen . primary hlh as well as secondary hlh not controlled by treatment of underlying condition must be treated with hlh-2004 protocol , which includes dexamethasone , cyclosporine a , and etoposide . in ebv - hlh , it has been proposed a multi - step treatment , in an attempt to reduce long - term toxicity of etoposide . a case of ebv - leishmania co - infection in a 9-month old girl as a trigger for hlh has been reported . transient low - level viremia has been reported without significance in immunocompromised and non - immunocompromised host . in our patient , we found ebv and leishmania co - infection , an extremely rare association . in addition , ebv is the main trigger of primary hlh , what makes it difficult to distinguish between primary and secondary hlh . this is the main reason why investigation of leishmania by pcr should be done on bone marrow of patients with hlh and ebv infection ( specially in endemic areas ) , to rule out leishmania infection because its sensitivity and specificity are greater than microscopy or other microbiological test , in order to avoid toxicity derived from drugs contained in hlh-2004 protocol .

this is the report of an ebv + leishmanial co - infection . the patient developed hemophagocytic syndrome ( hlh ) and was treated with the standard hlh-2004 protocol . however , pcr in bone marrow discovered this secondary cause for hlh . in endemic countries , visceral leishmaniasis should be considered in the differential diagnosis even in ebv - related hlh , as chemotherapy toxicity may be avoided .

congenital hypothyroidism is the most common congenital endocrine disorder and occurs at rate of one in 3000 - 4000 births . as the clinical features of congenital hypothyroidism are often subtle , many newborns remain undiagnosed at birth . it is important for physicians to be aware of such presentations , especially , in areas where screening for congenital hypothyroidism is absent . we present an infant who had refractory cardiogenic shock as the presenting feature of congenital hypothyroidism . a five - month - old female infant presented with refusal to feed , lethargy and vomiting for three days . there was no history of fever , urinary complaints , recurrent respiratory tract infections or hospitalisation in the past . there was no history of prolonged neonatal jaundice , delayed passage of meconium or feeding difficulties after birth . the heart rate was 80/ min with poor peripheral pulses , respiratory rate was 38/min and blood pressure was 52/34 mmhg . there was excessive hair on forehead , macroglossia , distended abdomen and umbilical hernia [ figure 1 ] . generalised hypotonia with delayed relaxation of deep tendon reflexes was present . on cardiovascular system examination , clinical photo of the patient showing excessive hair on forehead , macroglossia , distended abdomen and umbilical hernia fluid resuscitation with 20 ml / kg normal saline was given on admission followed by dopamine infusion @10 g / kg / min . investigations revealed : hemoglobin 9.8 gm% , total leucocyte count 8900/cumm and platelet count 4.2 lac / cumm . serum calcium was 7.8 mg / dl and alkaline phosphatase was 910 iu . her blood culture and urine culture were normal . thyroid profile showed : serum t3 < 10 ng / dl ( n-105 - 245 ) , serum t4 1.20 /dl ( n 7.8 - 16.5 ) and tsh 221.67 miu/ ml ( n 0.8- 8.2 ) , suggestive of primary hypothyroidism . technetium 99 thyroid scan revealed absent thyroid gland in the neck with no evidence of functioning ectopic thyroid tissue . x - ray of lower limb revealed absent distal femoral epiphyses [ figure 2 ] . echocardiography revealed a left ventricular ejection fraction ( lvef ) of 35% with global hypokinesia . electrocardiogram ( ecg ) showed low voltage p waves with diminished amplitude of qrs complexes . the next day in view of persistent hypotension , dopamine infusion was increased to 15 / kg / min and dobutamine @10 g / kg / min was added which was increased to 15 g / kg / min . however , even on the third day of admission the hypotension persisted in spite of high dose dopamine and dobutamine infusion . the heart rate was 96/min with poor peripheral pulses , respiratory rate was 32/min and blood pressure was 54/30 mmhg . oral levothyroxine was started at 10 mcg / kg and was increased to 15 mcg / kg mg over 48 hours . after 48hrs , the heart rate was 106/min with good peripheral pulses and the blood pressure was 84/60 mmhg . the patient was discharged on levothyroxine after ten days of admission . a repeat echocardiography done after one month revealed normal left ventricular function with lvef of 60% . cardiovascular manifestations of hypothyroidism include pericardial effusion , weak arterial pulses , bradycardia , hypotension , non - pitting facial and peripheral edema muffled heart sound , and evidence of congestive heart failure such as ascitis , orthopnea and paroxysmal dyspnea . the chronotropic response and normal tension of the heart muscle in diastole depends on tri - iodothyronine . moreover , tri - iodothyronine expression in the cardiac muscle affects the number of b - adrenergic receptors and their sensitivity to catecholamines . hence , patients with profound hypothyroidism with cardiogenic shock have a poor response to catecholamines . severe hypothyroidism causes muscle relaxation and the isovolumetric relaxation and contraction time becomes prolonged and myocardial performance index ( mpi ) increases indicating systolic and diastolic dysfunction . the ecg changes seen in hypothyroidism include sinus bradycardia , low voltage qrs complexes , prolonged qt interval , low p wave amplitude , right bundle branch block and ventricular dysrhythmias , including torsades de pointes.[36 ] these cardiovascular effects are usually reversed after starting thyroxin . while cardiac dysfunction is well documented secondary to hypothyroidism , hypotension and cardiogenic shock is rare and is resistant to conventional therapy in the form of intravenous fluids and vasopressors . in such cases , urgent levothyroxine replacement can restore hemodynamic stability by reversing the systolic and diastolic dysfunction , as seen in our case . dharmasena et al and gupta et al , have described a 52 year old male and a 62-year - old female respectively with hypothyroidism . both the cases presented with cardiogenic shock and did not respond to intravenous fluids , vasopressors or steroids . congenital hypothyroidism and cardiogenic shock revealed only a single case described by drop et al . they have described a 6-week - old girl who presented with cardiogenic shock and had a partial thyroid hormone unresponsiveness of the pituitary due to congenital elevation of thyroid binding albumin . another unusual feature seen in our patient was the presence of hypertrichosis . . the stimulation of hair growth via adrenal androgens by tsh may be the probable pathophysiological mechanism . zaki et al . have suggested that full assessment of thyroid function be carried out in all cases of hypertrichosis or abnormal distribution of body hair .

thyroid dysfunction causes remarkable cardiovascular derangements . both systolic and diastolic dysfunction of the heart can occur in hypothyroidism leading to cardiac arrhythmia and congestive heart failure . refractory cardiogenic shock and hypotension in congenital hypothyroidism is rare . we describe a 5-month - old female infant with congenital hypothyroidism and refractory cardiogenic shock . cardiac function and hemodynamic stability were restored after starting levothyroxine therapy .

surgical resection remains the only chance of cure for patients with pancreatic adenocarcinoma and periampullary cancers . the most commonly performed procedure for lesions in or around the head of the pancreas is a pancreaticoduodenectomy ( whipple 's procedure ) . although mortality associated with pancreaticoduodenectomy has decreased over the years , morbidity rates remain high . the most common complication associated with a whipple 's procedure is failure of the pancreatic anastomosis . risk factors reported to increase the risk of pancreatic anastomotic failure include a soft pancreas and a small duct . one other factor that has rarely been discussed in the literature is the association between ischaemia of the remnant pancreatic margin and pancreatic anastomotic failure . that ensuring adequate blood supply to the remnant margin reduces the incidence of pancreatic anastomotic failure . here , we report a novel technique using indocyanine green dye ( icg ) to assess blood flow at the pancreatic remnant margin before construction of the pancreatic anastomosis ( pancreaticojejunostomy ) . investigations including endoscopy and computed tomography ( ct ) scans demonstrated an ampullary lesion in the duodenum . biopsies taken at endoscopy were reported as a tubular adenoma with high - grade dysplasia . the initial treatment with ampullectomy demonstrated an infiltrating ampullary adenocarcinoma with incomplete excision on histopathology . in the presence of an incomplete resection , the patient subsequently underwent a laparoscopic - assisted whipple 's procedure . before construction of the pancreaticojejunal anastomosis , the viability of the margin of the remnant pancreas was assessed with icg and a near - infrared ( nir ) laparoscopic camera ( olympus , hamburg , germany ) . briefly , once the surgeon is prepared to start the construction of the pancreaticojejunal anastomosis , the anaesthetist injects 2 ml ( 0.5 mg ) of infracyanine ( concentration was 0.25 mg / ml ) intravenously via a peripheral vein . the icg contrast can be visualized in the viable areas but not in the ischaemic areas ( fig . 1a ) . the ischaemic segment at the margin was further resected before anastomosis ( fig . the pancreatic resection margin appears healthy to the naked eye with a small ( <3 mm ) pancreatic duct . the infrared camera is then switched on , and the ischaemic segment of the pancreatic margin demonstrating no icg fluorescence can be visualized . ( b ) after resection of the ischaemic portion , the margin appears well perfused . the pancreatic resection margin appears healthy to the naked eye with a small ( <3 mm ) pancreatic duct . the infrared camera is then switched on , and the ischaemic segment of the pancreatic margin demonstrating no icg fluorescence can be visualized . ( b ) after resection of the ischaemic portion , the margin appears well perfused . the patient had no postoperative evidence of a pancreatic leak either clinically , on assessment of drain amylase or on ct imaging . the definition proposed by the international study group for pancreatic fistula has graded the fistulas into a , b and c with grade c accounting for significant morbidity and mortality . a recent review by denbo et al . reported an 18% incidence of pancreatic fistula with 15% of these being grade c and suggested ( rightfully so ) that the focus should be on reducing this incidence . risk factors commonly reported for the development of a pancreatic fistula are a soft pancreas , small pancreatic duct and a fatty pancreas [ 5 , 6 ] . while nothing can be done about the texture of the gland or the size of the duct , some studies have focussed on decreasing pancreatic exocrine function with the use of somatostatin analogues , using pancreatic stents , different forms of pancreatic anastomosis and gluing of the duct . the results are conflicting in the literature , but a recent review by lermite et al . however , little has been published on pancreatic resection margin ischaemia and its association with the development of pancreatic fistula . the pancreatic head is supplied by the vascular arcade formed by the anastomosis of the superior and inferior pancreatico - duodenal arteries , while the body and tail are supplied by the splenic artery . the neck of the pancreas has been described as a watershed area with blood supply coming from either the head or the body . consequently , transection of the pancreas at the neck may result in an area of relative ischaemia and may contribute to anastomotic failure . reported that the assessment of blood supply to the transected margin of the pancreas visually and with doppler ultrasound , with further transection if the blood supply is inadequate , resulted in decreased incidence of pancreatic fistula . acute pancreatic ischaemia can not be seen with the naked eye or with ultrasound , and fluorescence angiography is better than ultrasound in detecting tissue viability . the use of icg for nir fluorescence angiography to demonstrate ischaemic tissue has been reported in animal models . in addition , kudszus et al . demonstrated that nir angiography using icg and a detection video camera in colorectal surgery reduced the rate of anastomotic leaks . the injection of icg and assessment of its distribution with the nir detection device allow the display of ischaemic zones , which can then be resected further . the disadvantage of strasberg 's technique is that the surgeon has to be skilled in intraoperative ultrasound assessment of the pancreas . the advantage of our technique is that no such skill is required , and the technique is simple and easy to learn . however , compared with the morbidity and cost associated with pancreatic leaks in our opinion , this technique provides significant advantages . the identification of an ischaemic transection margin during a whipple 's procedure allows further resection to viable tissue . this will improve the blood supply to the pancreatic anastomosis and may potentially help to decrease the incidence of pancreatic fistulas . the nir camera is supplied by olympus ( tokyo , japan ) and is not yet available for commercial purchase .

risk factors for anastomotic leaks of pancreaticojejunostomy have been well described . we present a technique using indocyanine green dye ( icg ) and a near - infrared ( nir ) capable laparoscope to assess blood supply to the transected margin of the pancreas before pancreaticojejunal anastomosis . a 39-year - old female patient underwent a laparoscopic - assisted pancreaticoduodenectomy ( whipple 's procedure ) for an invasive ampullary adenocarcinoma . before construction of the pancreaticojejunal anastomosis , the viability of the margin of the remnant pancreas was assessed with infrared scanning of injected icg . the nir identified an ischaemic segment of the margin , which was further resected . the patient had no postoperative evidence of a pancreatic leak and was discharged home on postoperative day 18 . ischaemia of the remnant pancreas is a risk factor for pancreaticojejunostomy leak . infrared icg testing might help to identify these ischaemic segments , which can be excised before anastomosis , and reduce failure rates .

temporary henna tattoos or pseudotattoo have become increasingly widespread among children and adolescent , as a safe and economic alternative to permanent tattoos . it is well - known that allergic skin reactions to natural henna are rare , due to its extremely low rate of sensitization . in india , north of africa , china , and egypt , it is used in weddings and religious ceremonies ; in occident , it is used to dye hair and cosmetics . paraphenylenediamine ( ppd ) , a powerful allergen , is added to the henna tattoo mixtures ( black henna tattoo ) to decrease application time and intensify the color . we describe the case of a 7-year - old boy who reported erythematous papular bulls - eye shaped lesions and consolidated edema primarily in the upper and lower extremities [ figure 1 ] . he also showed an erythematous - eczematous lesion on his leg , shaped like a dolphin [ figure 2 ] , and lesions compatible with erythema multiforme - like reaction . erythematous papular lesions contact eczema in the tattoo area dolphin shaped patch tests were performed , and we observed a high sensitivity after 48 h and moderate after 96 h. we reported a positive reaction to ppd . henna has been used to paint the skin for adornment and religious reasons for 9000 years and in over 60 countries . christians , jews , muslims , hindus , and buddhists have used henna as part of their religious customs . the henna is a flowering plant native to northern africa , western and southern asia in semi - arid zones , used since antiquity to dye skin . it has a great affinity for keratinocytes , and it is used to create temporary tattoos , without it being necessary to puncture the skin . black henna contains an ingredient in addition to pure henna to achieve its ebony color . in most cases , this added ingredient is ppd , a powerful sensitizer that should not be directly applied to the skin as it may cause mild contact dermatitis . one of the most dangerous applications of this chemical is when it is added to henna because the dye is applied while the ppd is in its oxidation process , and its potential as allergen is increased . when added to henna , the concentration of ppd is often much higher than what is approved for use in hair dyes . the cause of the sensitivity to ppd is unknown ; it is believed that the mechanism involved in the pathogenesis may be a reaction mediated by type iii immune complexes and associated with type iv retarded hypersensitivity . various topicals allergens cause erythema multiforme , including topical drugs such as corticosteroids , nonsteroidal anti - inflammatory drugs , iodine povidone , imiquimod ; rubber gloves ; nickel and herbicides . three possible causes of the residual hypopigmentation have been described : a reduction in melanin synthesis , selective destruction of the melanocytes , or photoleukomelanodermitis due to pigment blocking . as henna tattoos are becoming increasingly popular , prevention requires the provision of information to consumers , especially young people and their parents . it is important for the population to be aware of this circumstance and the risk entailed by sensitization to ppd . to conclude , we believe that temporary black henna tattooing should be controlled by health authority legislation to minimize the appearance of new cases of reaction to ppd and the serious and permanent consequences we have presented . it is important for the population to be aware of the risk entailed by sensitization to ppd due to popular henna tattoos .

temporary henna tattoos or pseudotattoos have become increasingly widespread among children and adolescent . a generalized skin reaction , type erythema multiforme - like reaction is unusual , and rarely reported . we describe the case of a 7-year - old boy who reported erythematous papular bulls - eye shaped lesions and consolidated edema primarily in the upper and lower extremities . these lesions were compatibles with erythema multiforme - like reaction . he also showed an erythematous - eczematous lesion on his leg , shaped like a dolphin . in this area , a temporary henna tattoo was painted 1-month earlier . patch test was positive for paraphenylenediamine ( ppd ) . skin reactions due to henna are rare . most of the reactions are due to additives , especially ppd , an aniline derivative , which is added to speed up the process of skin dyeing and to give a darker brown to black color ( black henna ) . as henna tattoos are becoming increasingly popular , prevention requires the annual provision of information to consumers , especially young people and their parents .

the animal ethics committee and animal care and use committee of the national institute of animal health approved the study . five holstein calves 23 months of age were intracerebrally injected with 1 ml of 10% ( w / v ) brain homogenates prepared from the medulla oblongata of bse / jp24 . in our earlier report , experimentally challenged cattle appeared to display clinical signs indicative of bse at 11 months postinoculation ( mpi ) ( 9 ) . animals were sequentially euthanized before and after the onset of clinical signs ( cattle identification codes 8515 and 496 at 10 and 12 mpi , respectively ) and at the terminal stage of the disease ( cattle identification codes 528 , 1061 , and 5566 at 16 mpi ) . we provisionally categorized the adrenal gland as nerve tissue because of the presence of chromaffin cells in the medulla of the gland . western blot analysis for prpres was performed on obex tissue samples as described previously by using anti - prp monoclonal antibody t2 ( 9 ) . prpres was detectable in all obex samples obtained 10 , 12 , and 16 mpi , suggesting that transmission of l - type bse prions to these animals was successful . dilution of the protease - treated brain sample and analysis of western blot results showed that the detection threshold for prpres was 1.25 g of brain tissue equivalent ( data not shown ) . a variety of nerve and lymphoid tissue samples were investigated for accumulation of prpres by western blot analysis by using phosphotungstic acid precipitation , as described previously ( 10 ) ; examples of cattle tissue samples obtained 10 and 16 months mpi ( codes 8515 and 1061 , respectively ) are shown in figure 1 . in cattle at the preclinical stage , prpres was detectable in all tested ganglia and barely detectable in the vagus nerve and vagosympathic trunk . in cattle at the terminal stage , prpres was barely detectable in the forelimb nerves ( suprascapular nerve , brachial nerve plexus , median nerve , and radial nerve ) , whereas substantial amounts of prpres were present in other nerve tissues except for facial and hypoglossal nerves ( table ) . a broader nerve tissue distribution of prpres was observed in cattle at 16 mpi than at 10 and 12 mpi . contrary to what we found in nerve tissues , we detected no prpres from tests performed on lymphoid tissues obtained from any of the 5 cattle studied . western blot analysis of a protease - resistant form ( prpres ) of a normal cellular prion protein in nerve tissue samples obtained from cattle 10 ( a ) and 16 ( b ) months postinoculation ( cattle identification codes 8515 and 1061 , respectively ) . the nerve tissues tested are shown above the lanes : 1 , trigeminal ganglia ; 2 , pituitary gland ; 3 , anterior cervical ganglion ; 4 , facial nerve ; 5 , hypoglossal nerve ; 6 , cranial mesenteric ganglia ; 7 , vagus nerve ( cervical part ) ; 8 , stellate ganglia ; 9 , adrenal gland ; 10 , phrenic nerve ; 11 , vagus nerve ( pectoral part ) ; 12 , vagosympathic trunk ( pectoral part ) ; 13 , vagosympathetic trunk ( lumbar part ) ; 14 , accessory nerve ; 15 , suprascapular nerve ; 16 , brachial nerve plexus ; 17 , median nerve ; 18 , radial nerve ; 19 , sciatic nerve ; 20 , tibial nerve , 21 , middle cervical ganglion . molecular mass standards ( kda ) are indicated on the left of each panel . * prpres , prion protein resistant ; bse , bovine spongiform encephalopathy ; mpi , months postinoculation ; + , positive for prpres ; , negative for prpres . infectivity of selected nerve tissues ( including the obex , sciatic nerve , adrenal gland , brachial nerve plexus , and vagus nerve ) obtained from cattle euthanized at 10 , 12 , and 16 mpi ( codes 8515 , 498 , and 5566 , respectively ) was analyzed by intracerebral injection into mice transgenic for bovine prion protein , as described previously ( 11 ) . as a negative control , mice were injected with cells from the brainstem of a normal cow . the presence of prpres in the brains of all mice used in the experiment was determined by western blot analysis . infectivity was detected in all nerve tissues tested , regardless of the presence of detectable prpres ( figure 2 ) . inocula from selected tissues obex ( a ) , sciatic nerve ( b ) , adrenal gland ( c ) , branchial nerve plexus ( d ) , and vagus nerve cervical part ( e)were prepared from cattle euthanized at 10 ( code 8515 , circle ) , 12 ( code 498 , square ) , and 16 ( code 5566 , triangle ) months postinoculation were and inoculated intracerebrally into mice transgenic for bovine prion protein . ratios above graph indicate number of prion - diseased mice / number of inoculated mice at 500 d postinoculation . we report accumulation of l - type atypical bse prions in peripheral nerve tissues sampled from intracerebrally challenged cattle . our study demonstrated that almost all of the peripheral nerve tissues tested became prpres positive in a time - dependent manner , whereas no prpres was detectable in lymphoid tissues , even in cattle with fatal atypical bse . our results suggest the possibility that , like classical bse prions , l - type bse prions propagated in the central nervous system and were spread centrifugally by nerve pathways ( 11,12 ) . in italy , l - type bse prions have been characterized in detail by using cattle challenged intracerebrally . however , prpres was not detected in their peripheral tissues , including the peripheral nerves ( 13 ) . the reason for the discrepancy in prpres detection is unclear . in view of the similarities between the l - type and bse / jp24 prion characteristics ( 9 ) , this discrepancy may result from differences in the methods used for prpres detection . we detected infectivity in the nerve tissue samples ( including samples from the obex , sciatic nerve , adrenal gland , brachial nerve plexus , and vagus nerve ) obtained 10 , 12 , and 16 mpi . on the basis of the incubation time of 223 25 ( mean sd ) days in mice injected with a 1,000-fold dilution of the obex homogenate , infectious titers in peripheral nerve tissues appeared to be 1,000 lower than those estimated in the obex during endpoint titration of infectivity . our results demonstrate that l - type atypical bse prions can be distributed in the peripheral nerve tissues of intracerebrally challenged cattle . these findings are useful for understanding l - type bse pathogenesis and accurately assessing the risks associated with this disease . investigations of prion distribution in cattle that have been orally challenged with l - type bse prions are critical .

we recently reported the intraspecies transmission of l - type atypical bovine spongiform encephalopathy ( bse ) . to clarify the peripheral pathogenesis of l - type bse , we studied prion distribution in nerve and lymphoid tissues obtained from experimentally challenged cattle . as with classical bse prions , l - type bse prions accumulated in central and peripheral nerve tissues .

to report an effect of the full tendon transposition augmented with posterior intermuscular suture and recession - resection surgery , for the patient with monocular elevation deficiency ( med ) and large exotropia . full tendon transposition augmented with posterior intermuscular suture and recession - resection surgery was performed for a 26-year - old male patient had monocular elevation deficiency ( med ) and large exotropia . preoperative angle of deviation was 56 prism diopters ( pd ) hypotropia and 45 pd right exotropia , compared with 18 pd left hypertropia and 10 pd right esotropia postoperatively . essotropia persisted after 2.5 years , however , and so the right medial rectus was recessed after removal of the previous posterior intermuscular suture . at a three - year follow - up after the second surgery , alignment was straight in the primary position at near and far distances . full tendon transposition augmented with posterior intermuscular suture and recession - resection surgery was effective for a patient with med associated with significant horizontal deviation , and a second operation was easily performed when overcorrection occurred . a 26-year - old male patient had med and large exotropia from birth , and a marked limitation of supraduction in the affected eye . he had no significant medical or ophthalmic history of prior trauma . an examination revealed best corrected visual acuities of 20/40 in his right eye and 20/20 in his left eye . preoperative angle of deviation was 56 prism diopters ( pd ) hypotropia and 45 pd exotropia of the right eye ( fig . the full tendon transposition of horizontal recti muscles to superior rectus muscle parallel to the spiral of tillaux was performed after lateral rectus muscle recession 9.0 mm and medial rectus muscle resection 5.0 mm ( fig . the transposed horizontal recti muscles were augmented with a posterior intermuscular suture as we previously described.4 briefly , the posterior augmentation consisted of a 5 - 0 non - absorbable polyester suture with a t-5 spatulated needle ( dacron ; ethicon ) that was placed through 25% of the belly of each transposed and paralytic rectus muscle at 7 mm posterior from the original muscle insertion . postoperative angle of deviation improved to 18 pd hypertropia of the left eye and 10 pd esotropia of the right eye ( fig . binocular single visual fields improved to 65 from 0. 10 pd esotropia of the right eye persisted after 2.5 years , leading the patient to complain about the cosmetic problem . accordingly , the right medial rectus was recessed 5.0 mm from the spiral of tillaux after removal of the posterior intermuscular suture . at a three - year follow - up , alignment was straight in the primary position at near and far distances . there were no complications such as anterior segment ischemia during the surgery and postoperative follow - up . surgery is indicated in med if there is vertical deviation in the primary gaze , deviation causing suppression and amblyopia , diplopia in the primary gaze , and contracted binocular fields . in patients with med associated with a significant horizontal deviation , multiple extraocular muscle surgery or normal fellow eye surgery are generally necessary.1 - 3 most patients or their parents , however , do not allow surgery in the normal fellow eye , and surgery on the multiple extraocular muscles may cause anterior segment ischemia . full tendon transposition augmented with posterior intermuscular suture and recession - resection surgery can effectively correct patients with med associated with significant horizontal deviation , while reducing the risk of anterior segment ischemia and avoiding an operation on the normal eye . additionally , the intermuscular suture can be easily removed even when a second operation is needed . in this case report , full tendon transposition augmented with posterior intermuscular suture and recession - resection surgery was an effective and safe procedure in a patient with med associated with significant horizontal deviation .

purposeto report an effect of the full tendon transposition augmented with posterior intermuscular suture and recession - resection surgery , for the patient with monocular elevation deficiency ( med ) and large exotropia.methodsinterventional case report . full tendon transposition augmented with posterior intermuscular suture and recession - resection surgery was performed for a 26-year - old male patient had monocular elevation deficiency ( med ) and large exotropia.resultspreoperative angle of deviation was 56 prism diopters ( pd ) hypotropia and 45 pd right exotropia , compared with 18 pd left hypertropia and 10 pd right esotropia postoperatively . essotropia persisted after 2.5 years , however , and so the right medial rectus was recessed after removal of the previous posterior intermuscular suture . at a three - year follow - up after the second surgery , alignment was straight in the primary position at near and far distances.conclusionsfull tendon transposition augmented with posterior intermuscular suture and recession - resection surgery was effective for a patient with med associated with significant horizontal deviation , and a second operation was easily performed when overcorrection occurred .

infection still remains a major cause of morbidity and mortality in systemic lupus erythematosus ( sle ) . patients with sle are well known to have increased risk for not only bacterial but other infections such as viral , fungal , and protozoa ( 1 , 2 ) . susceptibility to infection may be due to intrinsic abnormalities in the components constituting the immune system such as complements , mannose binding lectin , phagocytic cells , and t cells . the use of immuosuppressive agents , particularly steroids and cyclophosphamide , are the strongest extrinsic risk factors contributing to infection ( 3 ) . cryptococcal meningitis is a common opportunistic infection in acquired immunodeficiency syndrome ( aids ) patients and occurs not only in patients with other forms of immunosuppression but also in immunocompetent individuals ( 4 ) . the mortality rate remains high in both human immunodeficiency virus ( hiv)-associated cases and in non - hiv associated cases ( 4 , 5 ) . we describe a case of cryptococcal meningitis that presented with isolated sixth cranial nerve palsy in a male patient with sle , which was successfully treated with antifungal agents . most reports of ophthalmoplegia in patients with sle were related to the disease manifestations of sle ( 7 - 10 ) . to our knowledge , isolated sixth cranial nerve palsy as a presenting sign of crytococcal meningitis in an sle patient has not been reported . mary 's hospital in september 2006 with complaints of double vision and mild headache that had persisted for three days . in 1993 , he had been diagnosed with sle based on the presence of malar rash , photosensitivity , thrombocytopenia , positive antinuclear antibody , and positive anti - double stranded dna antibody . in november 1994 , he was diagnosed with lupus nephritis ( who classification iv ) and underwent a series of intravenous cyclophosphamide pulse therapies ( total 13 times ; total cumulative dose , 9,750 mg ) . he was treated with 24 mg of deflazacort and 1,000 mg of mycophenolate mofetil during the last one year before admission . on admission , his body temperature was 37.7. blood pressure , pulse rate , and respiratory rate were normal . the remaining general examination was unremarkable , and there were no signs indicative of meningeal irritation . his mental status was alert and no abnormalities were detected on neurologic or fundoscopic examination . on ophthalmic examination , he was found to have right sixth cranial nerve palsy without retinal abnormalities . initial laboratory findings revealed a white blood cell ( wbc ) count of 10,350/l ( neutrophil : 82.2% ) , a platelet count of 123,000/l , a serum creatinine level of 2.66 mg / dl , and slight hypocomplementemia ( c3 : 77.7 mg / dl ) . plain radiography of the chest was normal . computed tomography and magnetic resonance imaging of the brain showed no abnormal findings . cerebrospinal fluid ( csf ) analysis showed a pressure of 21 mmhg , a protein level of 179 mg / dl , and a glucose level of 73 mg / dl ( serum glucose , 91 mg / dl ) . mycophenolate mofetil was discontinued and empirical antibiotics ( ceftriaxone ) were administered without lowering the steroid dosage due to difficulties in differentiation between infection and lupus disease activity at the time of admission . on the fourth hospital day csf and blood cultures performed on the first hospital day revealed growth of cryptococcus neoformans . subsequently , amphotericin b and flucytosine were administered immediately , and lumbar puncture for a second csf analysis was performed . the second csf analysis done on the fourth hospital day revealed markedly elevated pressures of 42 mmhg and increased pleocytosis of 280/l . both crytococcal antigen test and indian ink stain were positive . the second brain mri done on the same day showed accentuation of leptomeningeal contrast enhancement along the sulci of both cerebral hemisphere , findings suggestive of meningitis ( fig . currently , he has recovered completely from esotropia that had been related to right sixth cranial nerve palsy . cryptococcal meningitis is a common opportunistic infection in patients with late stage hiv infection ( 4 ) . in parts of sub - saharan africa where hiv infections are prevalent , cryptococcal meningitis is now the leading cause of communityacquired meningitis , ahead of streptococcus pneumoniae and neisseria meningitides ( 11 , 12 ) . it also occurs in other immunocompromised patients and in apparently immunocompetent individuals ( 4 ) . since clinical features are often non - specific without neurologic deficit , cryptococcal meningitis should be included in the differential diagnosis of chronic or subacute meningoencephalitis ( 4 , 6 ) . there have been various reports that describe cryptococcal meningitis in sle patients ( 6 , 13 - 16 ) . in 1992 , zimmermann et al . described 2 cases and reviewed 24 previous cases of cryptococcal meningitis occurring in sle patients ( 6 ) . the outcome without antifungal agents was poor . on the contrary , among 20 patients treated with amphotericin b , 8 patients died . they demonstrated that the nonspecific neurologic findings associated with this infection are often a cause of misdiagnosis as a neuropsychiatric manifestation of lupus , emphasizing the importance of early diagnosis and implementation of effective antifungal therapy to ultimately improve the prognosis of cryptococcal meningitis in sle patients . our case also showed that cryptococcal meningitis could not be fully differentiated from central nervous system ( cns ) lupus at the time of initial presentation . in 2005 , hung et al . reported 17 cases of cns infections occurring in patients with sle ( 13 ) . among the 17 cases , 10 cases were due to cryptococcal meningitis , indicating a major role of cryptococcal meningitis in cns infection in patients with sle albeit being rare . in our case , common causes in young adults are a cns mass , demyelinating disease , or idiopathic causes ( 17 ) . cryptococcal meningitis is an uncommon cause of sixth cranial nerve palsy , and only a few cases of sixth cranial nerve palsy associated with cryptococcal meningitis have been reported irrespective of sle ( 18 ) . moreover , there have been a few cases of ophthalmoplegia occurring in sle patients ( 7 - 10 ) . however , all of them were associated with the disease manifestations of sle . in summary , we report a case of cryptococcal meningitis presenting with unilateral sixth cranial nerve palsy in a male patient with sle , which was successfully treated with antifungal agents . we recommend a clinical awareness for the possibility of crytococcal meningitis in sle patients whose presenting sign is isolated cranial nerve palsy without other neurologic manifestations . this is , to the best of our knowledge , the first case of cryptococcal meningitis presenting with unilateral sixth cranial nerve palsy in a patient with sle .

cryptococcal meningitis is a rare complication of systemic lupus erythematosus ( sle ) . the nonspecific neurologic findings associated with this infection delays accurate diagnosis because initial neuropsychiatric manifestations of sle are in instances indistinguishable from that of crytococcal meningitis . we report a case of cryptococcal meningitis presenting with unilateral sixth cranial nerve palsy in a male patient with sle , which was successfully treated with antifungal agents .

children with type 1 diabetes mellitus ( t1 dm ) have to deal with a complex and demanding daily treatment regime , which can have a negative impact on their quality of life ( qol ) as well as pose an economic and psychological burden for them . disease - specific qol problems , including a negative impact of diabetes on daily functioning , and diabetes - related worries have been reported . below is a case of t1 dm in an underprivileged child named suraj from the changing diabetes in children ( cdic ) program which will help to understand the life of children with t1 dm . he lives in a village . in order to reach the cdic center he has to travel 60 km via public transport . his mother , who works on a sugarcane farm , accompanies the child to the center as he can not travel alone . thus , diabetes management in such a child is no small task as t1 dm is not just about taking insulin . it is a difficult and different situation from daily life , as whenever a child is diagnosed with a disorder , which is not curable , there is a need to learn many new things for survival and most of the learning needs to be continuous . children with different ethnicities , culture and with different food habits visit the center and their main concern is about what they should eat . suraj told the helpers at the center that his parents can not read and write . furthermore , as they are children , they are more interested in playing than taking lessons on how to manage diabetes . suraj 's grandmother said that there is no doctor available in the village who knows about diabetes and its control . a surprise visit to his village it was discovered that the insulin was stored in an earthen pot ( matka ) . the ultimate goal of cdic is to make the child and his / her family capable of accepting and managing diabetes . multiple aspects ( physical , mental , psychosocial ) of a child need to be dealt with while managing diabetes . the challenges which actually cdic faces is to reach out to the children with diabetes as per their age , cultural background , level of understanding , language of understanding and economic status . so while providing insulin , glucometers and strips , cdic also included , along the way , complete care which involves various methods of educating children , parents , healthcare workers as well as doctors . thus , diabetes education is an important bridge between people with diabetes and good health . various innovative and creative diabetes educational materials have been developed which makes learning fun and includes mishti story book : story of a little girl with diabetes who shares her journey with diabetes ; novo nordisk teaches to take insulin toy : a soft toy which demonstrates insulin site selection and rotation ; snake and ladder game : a board game to help the child learn do 's and donts of diabetes ; make a healthy change folder : this in an input consisting of healthy recipes , lifestyle tips on diet , exercise , insulin and monitoring as well as an insulin site rotation aid ; hypo kit : help the child prevent hypo 's and consists of a snack box and water bottle to help them carry something to eat and water to drink ; and a glycated hemoglobin ( hba1c ) convertor : this calculator helps the child and their parents convert hba1c readings in to average blood glucose . most of these materials ( videos , leaflets etc . ) are in 10 languages . a child with diabetes needs empathy not sympathy and group education sessions go a long way in achieving it . when a child learns in groups they are more confident and thus such sessions are encouraged at all the centers of the cdic . the focus in these camps is on three basic components which are essential for children in managing diabetes and making their lives better : diabetes education , experience sharing and fun activities . experience sharing is the most important component of such camps as it helps in boosting the confidence of children as they listen to the stories of those children who have successfully survived diabetes . furthermore , a particular curriculum is made in year 2014 to make education sessions more effective . more than 275 camps have been conducted all over india since 2011 . in 2013 , 4795 children attended the camps , but cdic was able to reach out to about 2223 children . in 2014 , till now 3192 children have attended the camps , with cdic reaching to 1736 children . there are many challenges faced by cdic in managing t1 dm as it requires self - care round - the clock . moreover , even despite best efforts in managing diabetes , results are not good every time . it 's a financial liability as it is not only related to insulin , strips , other medications , but also time away from business , while taking child to doctor , transport expenses , expenses related to providing nutritional food and coping with child 's trauma , e.g. suraj 's mother has to miss her job for a day in order to accompany her son during the visit to cdic center and that leads to an expenditure of rs . thus the major challenges faced by cdic are : to cater to the needs of most deserving children with t1 dm throughout the country , to provide comprehensive care including self - sufficiency , to serve children for as long as possible and to ultimately have better outcomes for all children with t1 dm . thus the vision of cdic is to initiate and strive for comprehensive diabetes care for the economically underprivileged children with t1 dm . changing diabetes in children comprises of 21 centers and 27 satellite centers across india with leading doctors taking care of 4,063 children . there are about 3,00,000 insulin vials , 4,000 glucometers and 21,00,000 glucose strips for monitoring available . test facilities for measuring hba1c , complete blood count , microalbumin , fundus and thyroid stimulating hormone are present . till date along with medical knowledge , how to provide social and emotional support to children with t1 dm are also discussed during these workshops . the scientific committee is working on collating the tangible benefits observed in the qol among the participating children and sharing their learnings . all the children who are already registered in the program can get the benefit till they are 21 years . although not documented , but smiling faces on these children with t1 dm is testimony to the success of cdic . these minute efforts of cdic in improving the qol of children with diabetes should not be underestimated as small steps mark the beginning of big success . the main aim of the cdic program is to make the child more positive , secure and hopeful and the way forward is to have better outcomes for all children with t1 dm in all prospects so that they can grow into healthy adults .

the changing diabetes in children ( cdic ) program is a unique program aimed at children suffering from type 1 diabetes . the whole focus of cdic is to provide comprehensive care including diabetes education . various innovative and creative diabetes educational materials have been developed , which makes learning fun . lot of diabetes camps are held at cdic , focusing on diabetes education , experience sharing and fun activities . cdic faces many challenges in an effort to cater to the needs of most deserving children with type 1 diabetes mellitus ( t1 dm ) throughout the country , to provide comprehensive care including self - sufficiency , to serve children for as long as possible and to ultimately have better outcomes for all children with t1 dm . the cdic program aims to make the child more positive , secure and hopeful and initiate and strive for comprehensive diabetes care for the economically underprivileged children with t1 dm .

a 51-year - old female had palpitations and radiating back pain and underwent whole - aorta angio 3d computed tomography ( ct ) . the ct revealed a horizontal axis right kidney with a 4.2 cm , lobulating contour multilocular cystic mass at the lower pole . there were multiple irregular , enhancing septations with an enhancing solid portion and central calcification in the mass . 1 ) . she was diagnosed as having cystic rcc in the horizontal axis right kidney and underwent hand - assisted laparoscopic radical nephrectomy in september 2010 . under general anesthesia , a 7 cm laparoscopic hand - assist device was placed in the right lower quadrant with the placement of two additional 10 mm ports directly cephalad along the lateral rectus border , allowing 8 cm of separation between the ports and the hand - assist device . following dissection into the retroperitoneal space , the kidney was separated from the psoas muscle , adjacent bowel , and liver . there was a 4 cm cystic mass in the horizontal axis right kidney ( fig . the renal artery and renal vein were adhered together , so we used an endo - gia universal stapler ( covidien surgical , dublin , ireland ) and divided them , thereby isolating the entire right kidney . the specimen was entrapped in a laparoscopic sac and extracted . the procedure was completed successfully with a surgical time of 1 hour and an estimated blood loss of 100 ml . pathology revealed the tumor to be a clear cell carcinoma ( fuhrman grade 3 ) with the tumor limited to the kidney ( t1a ) and with negative resection margins . in december 2010 , follow - on ct revealed no recurrence of the disease . the adult kidney , as it assumes its final position in the ' renal ' fossa , orients itself so that the calyces point laterally and the pelvis faces medially . when this alignment is not exact , the condition is known as malrotation . most often , the inappropriate orientation is found in conjunction with another renal anomaly , such as ectopia with or without fusion or a horseshoe kidney . it is thought that medial rotation of the collecting system occurs simultaneously with renal migration . the kidney starts to turn during the 6th week , just as it is leaving the true pelvis , and it completes this process , having rotated 90 degrees toward the midline , by the time ascent is complete at the end of the 9th week of gestation . it has been postulated that rotation is actually the result of unequal branching of successive orders of the budding ureteral tree , with two branches extending ventrally and one dorsally during each generation or division . each ureteral branch then induces differentiation of the metanephrogenic tissue surrounding it to encase it as a cap . weyrauch accepted this theory of renal rotation to be the result of excessive ventral versus dorsal branching of the ureteral tree and concluded that the fault of malrotation lies entirely with the ureter . a late - appearing ureteral bud may insert into an atypical portion of the renal blastema , leading to a lessened propensity for the developing nephric tissue to shift . late appearance of the ureteral bud is almost always associated with an aberrant origin from the wolffian duct ; this translates into ureteral ectopia at the level of the lower urinary tract . the renal blood supply does not appear to be the cause or a limiting factor in malrotation but rather follows the course of renal hyporotation , hyper - rotation , or reverse rotation . there are two divergent opinions in the anatomical literature concerning the definite position of the kidneys . according to the first , the kidney ascends in the retroperitoneal space during precocious ontogenetic development . the renal rudiment occurs in the pelvic region , at the level of the l2 to l3 vertebrae , with the dorsal convex border and the ventral hilum touching the abdominal wall . to place itself in the definite position , the kidney undergoes a process of ascension and rotation . between the 6th and the 9th week the second opinion states that the kidney undergoes a pseudoascension caused by the fast development of the caudal extremity of the fetus [ 2 - 4 ] . in most malrotation cases , malrotation occurs ventrally , ventromedially , dorsally , or laterally . in our case , however , the renal pelvis faced the medial side , but the long axis of the kidney was lying ventral to dorsal . the upper pole and lower pole of the kidney were indistinctive . therefore , in this case , the process of malrotation likely did not follow the ordinary fashion . it is likely that the kidney rotated cranially or caudally , not medially or laterally . the ectopic kidney can be located in the pelvis , in the abdomen , or , rarely , in the thorax ( 0.3% ) and can be unilateral or bilateral . the most frequent cases of renal ectopia described in the literature of the field occur in male patients and are located on the right side of the pelvis [ 5 - 7 ] . generally , an ectopic kidney is smaller , is of irregular shape and variable rotation , and is vascularized by multiple arteries with various levels of origin . in some cases of ectopic kidney , there is often a bizarre rotation of the kidney as in our case [ 8 - 10 ] . however , the malrotated kidney was located at a similar level to the contralateral kidney and was also located in the retroperitoneal space . therefore , it was not an ectopic kidney . the process by which this malrotation occurred is not clear , but we can assume that the malrotation occurred during the ascension process or that the axis developed horizontally and proceeded ascension . several cases of renal stones in an ectopic kidney or malrotated kidney have been reported , but no cases of rcc have been reported in an abnormal kidney . the malrotated , ectopic kidney has clinical significance owing to its atypical location and malrotation . we are the first to report a horizontal axis kidney located in the retroperitoneal space . it is unknown whether the malrotated kidney is vulnerable to rcc development , but we are the first to report rcc in a case of kidney malrotation

the authors report a case of renal cell carcinoma in a right malrotated ( horizontal axis ) kidney . the patient was treated by hand - assisted laparoscopic radical nephrectomy . this is the first report of a horizontal axis malrotated kidney with renal cell carcinoma .

in the present issue of critical care , kinasewitz and colleagues report on the measurement of various markers of coagulation activation in patients with severe sepsis that were included in the prowess trial , the pivotal phase iii clinical trial of recombinant activated protein c . kinasewitz and colleagues underscore the notion that severe infection or sepsis is almost invariably associated with activation of coagulation . this coagulation activation spans a wide spectrum , from a subtle prohemostatic response only detectable with sensitive molecular markers of coagulation activation , to full - blown disseminated intravascular coagulation ( dic ) . there are several lines of evidence supporting an important pathogenetic role of dic in the development of sepsis - associated organ failure . histological studies of tissues from septic patients may show diffuse bleeding at various sites , hemorraghic necrosis of tissue , microthrombi in small blood vessels and thrombi in mid - size and larger arteries and veins . for example , experimental bacteremia or endotoxemia causes intravascular and extravascular fibrin deposition in the kidneys , the lungs , the liver , the brain and various other organs . amelioration of the hemostatic defect by various interventions in these experimental models appears to improve organ failure and , in most cases , to reduce mortality . finally , clinical studies support the notion of coagulation as an important denominator of clinical outcome . dic has been shown to be a strong and independent predictor of mortality in patients with sepsis and severe trauma . clinical studies aimed at intervening in the coagulation cascade in patients with severe sepsis have recently been completed . in particular , the administration of recombinant human activated protein c has been demonstrated to be effective in reducing 28-day mortality . the fact that other interventions such as antithrombin concentrate or recombinant tissue factor pathway inhibitor were less successful , however , may suggest that the beneficial effect of recombinant activated protein c may also rely on factors beyond coagulation . in fact , a marked interplay between coagulation and inflammatory activation at the level of the protein c system exists , and activated protein c has been shown to affect inflammation in vitro and in several in vivo models . on the contrary , the optimal clinical effect of activated protein c was calibrated using a coagulation parameter ( i.e. d - dimer ) , and patients with overt dic ( according to newly developed international criteria ) have a relatively larger benefit of activated protein c treatment . taken together , coagulation ( in conjunction with inflammation ) may well be at the basis of the pathogenesis of severe sepsis . kinasewitz and colleagues , in the present issue of critical care , show plasma levels of several biomarkers of coagulation activation , anticoagulant pathways , fibrinolysis , inflammation and endothelial cell injury in patients with severe sepsis . they indeed show that virtually all patients had activated coagulation , as reflected by increased levels of d - dimer and the thrombin in addition , the vast majority of these patients had coagulation activation that was detectable with less sensitive and more routinely available coagulation assays , such as the prothrombin time , and by measurement of antithrombin and protein c. also , a depression of fibrinolytic activity was noted in about 50% of patients . the changes in coagulation parameters were strongly related to inflammatory activity and to markers of endothelial cell injury . the data confirm previous studies that nonsurviving patients have a more marked activation of inflammation and coagulation , and a more severely depressed anticoagulant defense . most textbooks state that severe activation of coagulation or dic is most commonly associated with infection with gram - negative bacteria . however , systematic surveys show that gram - positive bacteria may cause fulminant coagulation activation as often as do gram - negative bacteria . other microorganisms , including viruses and parasites ( such as malaria ) , may also cause dic , which can dominate the clinical picture or at least play a significant role in the pathogenesis of the severe infection . the report by kinasewitz and colleagues also indicates that activation of coagulation in patients with severe sepsis occurs regardless of the causative microorganism . the only exception in the article by kinasewitz and colleagues may be represented by the patients with a fungal infection . however , when corrected for disease severity , it is probable that this group would be comparable with patients with bacterial infections . it is known that specific microorganisms may have a specific impact on the coagulation system . viruses , such as cytomegalovirus , may cause microangiopathic thrombosis and vasculitis on top of their ability to cause activation of coagulation . gram - positive bacteria , such as certain strains of streptococcus pyogenes , may cause a marked activation of the contact system . a significant difference in the ability to induce inflammatory and coagulation activation between endotoxin preparations of various microorganisms was also noted . nevertheless , in a mixed group of patients with severe sepsis , as was admitted to the prowess study , in which more than 20 different causative microorganisms were isolated , no major differences in biomarkers reflecting activation of coagulation and of inflammation between groups of microorganisms were observed . this result seems to support the hypothesis that the intensity of the inflammatory or coagulation response is dependent on other factors , such as host - dependent circumstances or the site of the infection , rather than on the causative microorganism . in conclusion , activation of coagulation , impairment of physiological anticoagulant pathways and depression of fibrinolysis , along with inflammatory activation , are seen in the vast majority of patients with severe sepsis . the severity of the inflammatory coagulative response does not seem to have a relationship with the causative microorganism . this once again underscores the notion that the host response rather than the underlying infection is responsible for the clinical manifestation of severe sepsis .

sepsis almost invariably leads to hemostatic abnormalities , ranging from insignificant laboratory changes to severe disseminated intravascular coagulation . there is compelling evidence from clinical and experimental studies that disseminated intravascular coagulation is involved in the pathogenesis of microvascular dysfunction and contributes to organ failure . data from the prowess phase iii clinical trial of recombinant activated protein c in patients with severe sepsis confirm this notion and demonstrate that the vast majority of patients with severe sepsis have increased markers for systemic coagulation activation , decreased physiological anticoagulant proteins and depressed fibrinolysis . there is no correlation between the type of microorganism that has caused the infection and the presence or severity of the coagulation disorder .

aneurysmal bone cyst ( abc ) is a benign tumor like condition of bone , which consists of blood filled cystic cavities and is locally destructive . we report an aneurysmal bone cyst of talus in a 20-year - old male that was managed by extended intralesional curettage and autologous iliac crest bone grafting . a 20-year - old male , student by occupation , presented to the outpatient department of government medical college , jammu , india in december 2009 with complaints of insidious onset , intermittent dull aching pain in the left ankle , since last 7 months . he denied history of swelling , fever , chills , sweats , weight loss or similar complaints in other joints of the body . the family , drug , occupational , sexual and recreational histories were non - contributory . , there was no visible or palpable swelling , local temperature was normal and tenderness to deep palpation was noted on the dorsal aspect of ankle . anteroposterior ( ap ) and lateral radiographs of the ankle [ figure 1 ] and a computed tomography ( ct ) scan [ figure 2 ] revealed a radiolucent , expansile , lytic lesion with lobulated borders occupying the head and neck of the left talus . gadolinium enhanced magnetic resonance imaging ( mri ) of the foot and ankle revealed an eccentric , cystic lesion in the left talus , which was hypo - intense on t1 weighted sequences and hyperintense on t2 weighted sequences and showed localized cortical erosion . there was no extension into the subtalar joint or adjacent soft tissues [ figure 3 ] . lateral radiograph of the ankle showing a lytic lesion in the talar head and neck ct scan of the talar lesion magnetic resonance imaging scan ( a ) t1 weighted saggital section showing an eccentric , cystic , hypointense lesion occupying the talar head and neck with localized cortical erosion . there is no soft tissue extension , ( b ) the lesion shows subtle enhancement with gadolinium ct guided fine needle aspiration from the lesion revealed numerous osteoclast giant cells in an hemorrhagic background . on the basis of the clinical findings , imaging studies and cytological examination a provisional diagnosis of giant cell lesion of bone was made . we considered giant cell tumor , aneurysmal bone cyst and simple bone cyst amongst the diagnostic differentials . however , we decided to plan the treatment of the lesion along the lines of giant cell tumor . the nature of the lesion , the diagnostic differentials , different treatment options and possible outcomes were discussed at length with the patient prior to obtaining an informed and written consent . the patient was taken up for extended intra - lesional curettage with phenol as an adjuvant and autologous iliac crest bone grafting . per - operatively , cortical erosion on the lateral aspect of the talar head and neck could be identified . a window was made on the lateral surface of the talar neck to facilitate curettage . thorough curettage was performed and the cavity was enlarged in all directions using a high speed burr and phenol was used as an adjuvant . post - operatively , the foot was immobilized in a non weight bearing below knee cast for 12 weeks . histopathological examination of the curettage material revealed presence of multiple blood filled cavities enclosed by fibrous septae without an endothelial lining [ figure 4 ] . numerous osteoclast giant cells arranged irregularly in clusters , as well as scattered individually were also identified . sections from the curettage material showing multiple blood filled cavities enclosed by fibrous septae without an endothelial lining . numerous osteoclast giant cells arranged irregularly in clusters , as well as scattered individually are also seen . ( h and e , 400 ) radiographs at 12 weeks showed good incorporation of the bone graft after which patient was permitted to bear weight [ figure 5 ] . aneurysmal bone cyst ( abc ) is a benign tumor like condition of the bone . many authors believe it to be a result of local circulatory disturbances and therefore do not consider it as a true neoplasm . most cases occur between the ages of 10 - 20 years and show a slight female preponderance . the favored site of presentation is the vertebrae , flat bones and metaphysis of long bones . talus is an unusual site for abc , in fact the most common tumors of talus are intra - osseous ganglion cysts . it has been said that abc is associated with distinctive 17p13 translocations that result in up - regulation of usp6 , a deubiquitinating enzyme . secondary. secondary abcs may be seen with fibrous dysplasia , osteoblastoma , chondromyxoid fibroma , nonossifying fibroma , chondroblastoma , osteosarcoma , chondrosarcoma , unicameral bone cyst , hemangioendothelioma , and metastatic carcinoma . therefore , a diagnosis of abc merits a thorough search any associated pathology , which if present , would dictate the line of management . other differentials include simple bone cyst ( sbc ) and giant cell tumor ( gct ) . the presence of blood filled cavities surrounded by proliferating fibroblasts and osteoclast giant cells differentiates abc from a sbc . giant cell tumor ( gct ) tends to occur in the skeletally mature population and involves the epiphyses of long bones . gcts have been reported to occur in the talus and can sometimes present with a secondary abc . however , the presence of mononuclear stromal and regular distribution of giant cells favors the diagnosis of gct . in addition , the giant cells in gct tend to be larger and contain more nuclei . many authors have described excellent results with intralesional curettage and bone grafting for lytic lesions that were well localized within the talus . partial or total talectomy along with tibiocalcaneal arthrodesis has also been described for lesions that show extensive destruction of the talus and soft tissue or subtalar extension . luna et al . , have described the use of external fixation in place of a traditional cast after curettage and bone grafting for abc of talus . in conclusion , the possibility of an aneurysmal bone cyst must not be forgotten when a patient presents with a lytic lesion in talus . since , it may be difficult to differentiate talar abcs from gcts on the basis of imaging studies or fine needle aspiration alone , it is prudent to follow the principles of treatment of gct viz even though the histological picture may be diagnostic of an abc , a meticulous search should be made to rule out the secondary nature of such a lesion . with primary abcs

aneurysmal bone cyst ( abc ) of the talus is an extremely rare lesion ; less than 20 cases have been reported in pubmed till 2012 . we report a primary abc of the talus in a 20-year - old male that was managed by extended intralesional curettage with phenol as an adjuvant and autologous cancellous iliac crest bone grafting . the patient had excellent functional outcome and there was no recurrence at 2 years of follow - up .

we conducted a population - based retrospective cohort study using administrative databases from ontario , canada , that included hospital discharge abstracts , physician service claims , and demographic data . the ontario diabetes database is a validated registry of physician - diagnosed nongestational diabetes that is identified using these administrative data ( 7 ) . individuals are linked between all data sources via a unique health card number , which is reproducibly encrypted in all of these data sources . women aged 2049 years who had a hospitalization record indicating a live birth between april 1994 and march 1997 were selected . for women who had more than one birth during this period , those who had pregestational diabetes or a cvd event ( as defined below ) in the prior 3 years were excluded . baseline characteristics were age at delivery , region of residence , and socioeconomic status ( measured as the neighborhood income quintile ) . women were defined as having gdm using an algorithm analogous to that used by the validated registry to exclude gdm : one hospitalization record or two ambulatory physician claims bearing the diagnosis of diabetes or gdm between 120 days before and 180 days after delivery . the primary outcome ( cvd events ) was defined as a hospitalization for acute myocardial infarction , stroke , coronary artery bypass , coronary angioplasty , or carotid endarterectomy . the prespecified secondary outcome ( coronary artery disease [ cad ] events ) was hospitalization for acute myocardial infarction , coronary artery bypass , or coronary angioplasty . although the registry does not distinguish between types , the majority of women developing diabetes in this group would have type 2 diabetes . cox proportional hazards regression was used to model the association of gdm with each outcome , accounting for the matched design of the study . for each outcome , an unadjusted model and a model adjusting for subsequent diagnosis of diabetes as a time - dependent covariate were built . the assumption of proportionality was verified by plotting log(log[survival ] ) versus log(time ) to assess parallelism . however , 3,127 were excluded because of preexisting diabetes , and 43 were excluded because of previous cvd . a further 2,036 were excluded due to missing data , mostly socioeconomic status . of the remaining 351,685 subjects , 8,194 ( 2.3% ) had gdm during the index pregnancy . diabetes developed during follow - up in 2,214 ( 27.0% ) of the women with gdm and 2,596 ( 3.2% ) of the women without gdm . significant associations were found between gdm and both outcomes , but these associations were attenuated following adjustment for subsequent diabetes . our study is the first of its kind to show that young women with gdm have a substantially increased risk for cvd relative to women without gdm . the subsequent development of type 2 diabetes accounts for much of this increased risk , which reinforces the vital need for diabetes prevention strategies in this high - risk population . our findings are consistent with a cross - sectional study conducted by carr et al . ( 5 ) , which reported that women with a history of gdm had odds ratios for cvd and cad similar to those reported here ( 1.85 and 1.58 , respectively ) . however , this study was cross - sectional and relied on retrospective self - report to ascertain exposures and outcomes . in contrast , our cohort study used a more rigorous end point assessment and followed a much larger population of women over many years . our study used administrative data where clinical information , such as cardiovascular risk factors , was unavailable . women with gdm exhibit chronic insulin resistance ( 8) , which is associated with a clustering of risk factors that are in the causal pathway to cvd . therefore , women with gdm likely have very different risk factor profiles than those without gdm , and adjusting for these differences might obscure a clinically important association between gdm and cvd . in summary , women with gdm are at increased risk for cvd events compared with women without gdm , and much of this risk is attributable to the subsequent development of type 2 diabetes . as diabetes prevention interventions in women with a history of gdm have also been shown to slow progression of atherosclerosis ( 9 ) , this study highlights the importance of diabetes prevention for this high - risk population .

objective to determine whether women with gestational diabetes mellitus ( gdm ) have an increased risk of cardiovascular disease ( cvd ) following pregnancy.research design and methods all women aged 2049 years with live births between april 1994 and march 1997 in ontario , canada , were identified . women with gdm were matched with 10 women without gdm and were followed for cvd.resultsthe matched cohorts included 8,191 women with gdm and 81,262 women without gdm . mean age at entry was 31 years , and median follow - up was 11.5 years . the hazard ratio for cvd events was 1.71 ( 95% ci 1.082.69 ) . after adjustment for subsequent type 2 diabetes , the hazard ratio was attenuated ( 1.13 [ 95% ci 0.671.89]).conclusions young women with gdm had a substantially increased risk for cvd compared with women without gdm . much of this increased risk was attributable to subsequent development of type 2 diabetes .

photographic documentation of nail diseases is a useful part of objective evaluation of response to treatment . good photographic documentation is also essential for proper dissemination of scientific knowledge in the form of presentations or publications . some of the important points to remember are as follows : taking patient consentproper framing keep the area of interest centred and crop out blank space around the lesion as much as possiblestandardisation of images ( especially for pre- and post - photographs ) use a tripod whenever possible , use uniform distances , angles and lighting for serial imagestake multiple images . you can always delete the poor imagesoptimum lighting this is probably the most crucial element of photography in generaluniform , non - reflective background , preferably with a darker colour . keep the area of interest centred and crop out blank space around the lesion as much as possible standardisation of images ( especially for pre- and post - photographs ) use a tripod whenever possible , use uniform distances , angles and lighting for serial images take multiple images . you can always delete the poor images optimum lighting this is probably the most crucial element of photography in general uniform , non - reflective background , preferably with a darker colour . a single - lens reflex camera with a dedicated macro lens would produce the best images . a ring light will be a useful additional tool as it would help to avoid strong reflections from the nail surface . the best way to capture the image would be to go back a bit and zoom out to capture the nails . this would help prevent the shape distortion which is associated with shooting from too close a distance . the same can be done even with a point - and - shoot camera with sufficient optical zoom ( also make it a point to use the macro mode on the point and shoot ) . unless you are familiar with manual settings , it is always better to use automatic settings with the flash on . a strong reflection from the nail surface can be avoided by using a ring flash , reflectors or dulling the flash by covering the flash surface with a dedicated flash diffuser or tissue paper [ figures 1 and 2 ] . this is very important as the nail plate tends to produce strong reflections which may whiten out important morphological features . effect of flash on nails ( a ) without flash diffusion ( b ) after flash correction using dedicated ring flashes and macro lenses for nail photography the background needs to be a uniform , non - reflective surface . however , to ensure good contrast between the nail and background , it is best to shoot the nails with a gap ( about 2 m has been suggested ) between the nails and the background , with the camera in the macro mode with flash on ( black background technique ) . we have found good results with a gap of even 510 cm between the nails and the background [ figures 3 and 4 ] . imaging of the nails with a dark non - reflective background with a gap between the nail and the background difference in the contrast illustrated between the images ( a ) nail placed directly on the background and ( b ) with a gap between the nail and the background when the condition involves multiple nails , there are different techniques mentioned to include all nails in a single frame ( or in minimum frames ) . ashique and kaliyadan described a technique , in which two sets of images are obtained ; one including all fingernails except the thumb and a second image including only the thumb . this provides an image in which the nails are in the same plane , symmetrical and also looks aesthetically pleasing . gupta and gupta suggested a modification of this method where the frame could be made less broad by placing one hand over the other [ figure 6 ] . inamadar and palit suggested a technique of imaging the nails in a position where the palmar aspects of both hands are kept side by side . the thumbs are flexed across the palm and brought closer to the tips of other fingers in a way that the thumbnails face upwards [ figure 7 ] . the advantage of this technique is that all the nails can be included in a single frame . it is , however , difficult to standardise this technique and it may also pose difficulties in patients having joint problems , making flexion of the small joints difficult . technique for placing all finger nails in a frame technique for placing all finger nails in a frame other than the thumb technique for placing all finger nails in a frame onychoscopy ( dermoscopy of the nails ) is being increasingly used in enhancing the diagnosis of nail diseases . both non - polarised , contact dermoscopy and polarised light dermoscopy can be used for the nails . however , we would like to highlight that documentation of dermoscopic images of the nails is sometimes difficult because of the curved convex surface of the nail . when using normal fluid contact dermoscopy , using a sufficiently thick amount of relatively firmer dermoscopic medium such as ultrasound gel is essential to fill the convex surface to get the best images with minimal peripheral blurring . transillumination can sometimes be a useful tool in assessing the extent of subungual lesions such as wart . since external light sources such as the camera flash will whiten out the transillumination , the image will have to be taken with the flash off [ figure 8 ] . using a tripod would be essential to prevent camera shake as even a mild shake will blur details in the case . general principles of clinical photography apply to nail photography toothe black - background or dark - background technique , where the nails are photographed with a gap between it and a dark non - reflective background , gives the best resultsthe flash must be controlled to avoid excess reflectionsdifferent standardised frames can be considered when all nails have to be placed in a single imageconsider using accessories such as macro lenses and ring flashes for the best results . general principles of clinical photography apply to nail photography too the black - background or dark - background technique , where the nails are photographed with a gap between it and a dark non - reflective background , gives the best results the flash must be controlled to avoid excess reflections different standardised frames can be considered when all nails have to be placed in a single image consider using accessories such as macro lenses and ring flashes for the best results .

photographic documentation of the nails is important in the objective evaluation of response to treatment and in disseminating scientific information related to nail diseases . the key to a good image of the nail is proper framing and achieving a sharp focused image with good contrast with the background , at the same time avoiding strong reflections from the nail surface . while the general principles of clinical photography apply to nail imaging also , this article attempts to highlight some tips which can be specifically used to improve the quality of nail images .

the annular pancreas is a rarely reported congenital anomaly that forms a ring of pancreatic tissue leading to partial or complete encirclement of the descending duodenum . 12 although it is usually found in neonates , the annular pancreas in an adult is reported infrequently.1 neonatal patients present with specific symptoms caused by duodenal obstruction , and adult patients present with abdominal pain , nausea , vomiting , weight loss , and jaundice . bypass surgery is the treatment of the annular pancreas in order to relieve duodenal obstruction . a 33-year - old male patient was admitted to the department of surgery of chung - ang university hospital complaining of recurrent upper abdominal discomfort and vomiting that had developed 12 months prior . five months before the admission , he visited our hospital presenting with upper abdominal pain . the computed tomography ( ct ) findings at that time suggested the possibility of the annular pancreas , but he was discharged without further evaluation , because his symptoms improved . at his second visit , ct showed a mass encircling the second portion of the duodenum with stenosis and food residue , suggesting annular pancreas ( fig . an upper gastrointestinal ( ugi ) series and magnetic resonance cholangiopancreatography ( mrcp ) were performed ( fig . 2 , 3 ) . a ugi series demonstrated an approximately 2.4 cm long luminal narrowing of the descending duodenum . the patient underwent laparoscopic surgery . a laparoscopic gastrojejunostomy with vagotomy was performed . under general anesthesia , the patient was placed supine in the reverse trendelenburg position . an 11 mm - sized camera port was inserted in the supra - umbilicus by the hasson method . three 5 mm - sized trocars were placed on the right upper and left lower abdominal wall and below the xyphoid process . a 12 mm - sized port was inserted in the right lower abdomen for the surgeon 's dominant hand and a laparoscopic linear stapler . then , the greater curvature of the stomach from 10 cm above the pylorus to proximal 10 cm was cleared in order to perform a gastrojejunostomy . a loop of the proximal jejunum , 50 cm distal to the ligament of treitz , was utilized for construction of the antecolic gastrojejunal anastomosis . then , a 60 mm linear stapler ( autosuture endo gia , covidien , mansfield , ma , usa ) was used to fashion the side - to - side gastrojejunal anastomosis , as it was fired between the opposing walls of the viscera . the annular pancreas is a rare congenital anomaly that affects males more commonly than females and occurs at a frequency of 1 : 20,000 births . of annular pancreas cases , 25% form a complete pancreatic ring , and 75% have a partial ring.3 although the etiology of the annular pancreas has not been fully elucidated , multiple developmental factors have been implicated in the pathogenesis of the annular pancreas.4 other congenital anomalies , including down syndrome , intestinal atresia , pancreatico - biliary malrotation , and pancreas divisum , are associated with the annular pancreas.5 the annular pancreas can present itself in a wide range of clinical severities according to the degree of duodenal obstruction . in adults , annular pancreas occurs most commonly in the decades of 20s to 50s . since these conditions in adults are usually asymptomatic , a number of diagnoses can be made by chance . the symptoms of adult annular pancreas include abdominal pain , nausea , vomiting , weight loss , jaundice , and abdominal fullness . when obstructive jaundice occurs , the possibility of a carcinoma of the ampulla of vater should be considered.6 the patient in this report presented with typical symptoms of the annular pancreas . a ' double bubble ' sign on the simple radiograph can help to make a diagnosis of the annular pancreas . however , this has limited diagnostic value , because it is neither specific nor sensitive.7 in adult patients , ugi series have been considered the study of choice.2 there are some findings that are typical , including annular defect on the second portion of the duodenum , dilatation of the proximal lumen , and retrograde intestinal movement from the proximal part to obstruction.5 stenosis of the second portion of the duodenum was found in this study . an endoscopic retrograde cholangiopancreatography ( ercp ) can make a specific diagnosis when the pancreatic duct is outlined . however , it is difficult to use ercp to diagnose patients with annular pancreatic ducts joining the accessory pancreatic duct or with duodenal obstruction.3 in addition , it is invasive and can cause iatrogenic pancreatitis . in addition , it is the most useful method to study abnormal pancreatic duct progression.28 abdominal ct and endoscopic ultrasonography are helpful to diagnose the annular pancreas . the definitive treatment of the annular pancreas is surgery , which can resolve a duodenal obstruction that causes symptoms . bypass is superior to local resection of the annular pancreas , which may be complicated with postoperative pancreatitis , pancreatic fistula , or recurrent duodenal stenosis.9 since vidal first reported successful gastrojejunostomy in a 3-year - old patient with annular pancreas in 1905 , zyromski et al.10 reported that duodenoduodenostomy or duodenojejunostomy caused less anastomotic site ulcers than gastrojejunostomy.10 although duodenoduodenostomy is a routine procedure in neonates and children , duodenojejunostomy or gastrojejunostomy is recommended in adult patients due to less mobility of the duodenum . the minimally invasive procedures are generally performed due to less postoperative complications and a short recovery time . it has been reported that laparoscopic duodenoduodenostomy and laparoscopic gastrojejunostomy are successfully performed in pediatric patients and in adult patients , respectively.510 we performed vagotomy in order to avoid an ulcer of the anastomotic site , which is one of the complications of gastrojejunostomy . we suggest that laparoscopic gastrojejunostomy with vagotomy is a good treatment option for adult patients with annular pancreas .

the annular pancreas in adults is a rare congenital anomaly that is detected after development of complications , such as gastric outlet obstruction , recurrent pancreatitis , and peptic ulcer . duodenal bypass is the procedure of choice for treating duodenal obstruction caused by the annular pancreas in both children and adults . duodenoduodenostomy is routinely performed in neonates and children . in adults , duodenojejunostomy or gastrojejunostomy are recommended , because the duodenum is less mobile . we report a case of annular pancreas in a 33-year - old male that was successfully treated with laparoscopic gastrojejunostomy .

bd is a multisystemic disease that may affect any organ in different combinations ? characterized by recurrent oral aphthous ulcers , genital ulcers , uveitis , and skin lesions . involvement of the heart is called cardio - bd , which includes pericarditis , myocarditis , endocarditis , endomyocardial fibrosis , atrial fibrillation , ventricular arrhythmias , coronary arteritis , acute myocardial infarction , and dilated cardiomyopathy ( 1 ) . we report a rare case of this syndrome , associated with aneurysm of the non - coronary sinuses of valsalva , resulting in symptomatic aortic valve regurgitation . a 38-year - old man was referred for investigation of a 5-day history of paroxysmal nocturnal dyspnea in may 2008 . he had a three - year history of recurrent genital ulceration initially diagnosed as genital herpes , but never confirmed on culture or nucleic acid amplification testing ; sequential treatment with aciclovir over a four - month period did not alleviate his genital symptoms . and he had no history of cardiac or respiratory disease , nor any cardiac risk factors . clinical examination revealed aphthous stomatitis , genital ulceration , erythema nodosum bilaterally and a grade 3/6 systolic murmur , audible at the 2nd intercostal space on the left sternal border . hematologic examination showed that high sensitive c - reactive protein ( hs - crp ) was 105 mgl and erythrocyte sedimentation rate ( esr ) is 70 mmhr . anti nuclear antibody ( ana ) , anti double strand dna antibody ( anti ds dna ) and muscle biopsy specimens for connective tissue disease were negative . chest x - ray revealed cardiomegaly with a cardiothoracic ratio of 55% and a slightly widened mediastinum . 1 ) . the left ventricular ( lv ) end - diastolic was 58 mm , and the lv ejection fraction was 53% . based on these findings , the diagnosis of aneurysm of the sinus of valsalva associated with bd was made . because the patient 's condition was in the active phase , prednisolone was administered over one month until the inflammatory signs ( hscrp and esr ) became negative . considered that our patient presented a competent valve and only one sinus involved , surgeon repaired the affected sinus only . he was discharged without any complications on postoperative day 15 , and was doing well at five - year follow - up after the operation . showing non - coronary - sinus of valsalva aneurysm aneurysms of the sinus of valsalva 's etiologies include atherosclerosis , infection such as syphilis , congenital disease such as marfan 's syndrome , trauma , and autoimmune disease such as bd . bd is a chronic , relapsing multisystem vasculitis with predominant involvement of the oral and genital mucosa ( 1 , 2 ) . bd has a worldwide distribution but is prevalent in japan , the middle east , and some mediterranean countries , and it often affects younger adults and is more common in men than women . james et al . demonstrated that valsalva sinus aneurysm was the leading causes of death in patients with bd ( 3 ) . in our patient , echocardiography revealed aneurysm formation of the sinus of valsalva , and should bd is the etiology ? the patient had a history of recurrent genital ulceration , on admission , clinical examination revealed aphthous stomatitis , genital ulceration , and erythema nodosum bilaterally , which could provide clues to differentiate diagnosis . if neglected these symptoms , it maybe at a loss of initial diagnosis of genital herpes . in addition to recurrent genital ulceration , aphthous ulcers and skin lesion , a clinical diagnosis of bd should be made , because other examination showed that high crp and esr , and ana , anti dsdna and muscle biopsy specimens for connective tissue disease were negative . aneurysms of the sinus of valsalva may incur complications if untreated , such as right ventricular outflow tract obstruction , coronary artery occlusion , aortic regurgitation and congestive heart failure , so surgical treatment should be considered ( 4 , 5 ) . but to aim directly at etiology is the most of all , and continuous steroid and immunosuppressive agent therapy are most important to maintain the integrity to prohibit recurrence of bd . our patient was doing well at five - year follow - up after the operation . it also highlights the importance of recognizing systemic disease and maintaining a holistic approach when treating patients .

abstracta 38-year - old man with a history of recurrent genital ulceration initially diagnosed as genital herpes was admitted after presenting with paroxysmal nocturnal dyspnea . echocardiography revealed aneurysm formation of the sinus of valsalva . on diagnosis of an aneurysm of the sinus of valsalva associated with behet 's disease ( bd ) , surgeon repaired the affected sinus only , and continuous steroid therapy maintained the integrity .

angiotensin converting enzyme ( ace , ec 3.4.15.1 ) inhibitors work by inhibiting the action of angiotensin converting enzymes , thus preventing the conversion of angiotensin to angiotensin - ii . ace inhibitors are widely used in the treatment of hypertension as well as heart failure [ 1 , 2 ] . the search for ace inhibitors that lacked the sulfhydryl group leads to the investigation of phosphorus containing compounds . the phosphinic acid is capable of binding to ace in a manner similar to enalapril . the interaction of the zinc atom with the phosphinic acid is similar to that seen with sulfhydryl groups [ 3 , 4 ] . additionally , this compound is capable of forming the ionic , hydrogen , and hydrophobic bonds . a feature unique to this compound is the ability of the phosphinic acid to more truly mimic the ionized , tetrahedral intermediate of peptide hydrolysis . structural modifications to investigate more hydrophobic , c - terminal ring systems , lead to a 4-cyclohexylproline analog of the original phosphinic acid . this compound , fosinoprilat , was more potent than captopril but less potent than enalaprilat . fosinoprilat proved to have the same problem as enalaprilat and other carboxylate - containing ace inhibitors like poor bioavailability . the addition of a hydrophobic side chain to modulate the ionization characteristics of the molecule thus fosinopril was developed . fosinopril is administered as a prodrug and is converted in vivo to the active form fosinoprilat ( scheme 1 ) . fosinopril containing analogs of trans-4-phenyl - l - proline ( a1 ) , 1,2,3,4-tetrahydro isoquinoline-3-carboxylic acid ( a2 ) , trans-4-cyclohexyl - l - proline ( a3 ) , trans-4-cyclohexyl - l - proline benzyl ester ( a4 ) , and l - proline ( a5 and a6 ) were used for this particular study . this method is based on the cleavage of the substrate hippuryl - glycyl - glycine by ace and subsequent reaction with trinitrobenzenesulfonic acid to form 2,4,6-trinitrophenyl - glycyl - glycine . the assay system consisted of 200 l of substrate solution , water , and drug solution in a volume of 0.25 ml . after 30 minutes of incubation at 37c , the reaction was stopped by adding 2 ml of stop solution ( 1.195 g hepes and 0.0465 g of edta were dissolved in 40 ml distilled water . the ph was adjusted to 9.0 with 1 mol / lit sodium hydroxide and diluted to 50 ml with distilled water ) . then 0.5 ml distilled water and 1.0 ml colour reagent were added , mixed vigorously , left for 5 min , and centrifuged at 30004000 rpm for 10 minutes at room temperature to remove any precipitate . the corresponding enzyme inhibitors blanks were assayed in the same manner except for adding the stop solution before the lung extract . the absorbance of the yellow product of the reaction between liberated hippurate and cyanuric chloride was measured spectrophotometrically at 405 nm against distilled water as blank .controls without drug were run simultaneously in all cases . the results of this study are given in tables 1 , 2 , and 3 and figures 1 , 2 , and 3 and the ic50 values are given in table 4 and figure 4 . one inhibitory unit is the amount of inhibitor that suppressed the ace activity by one unit . docking study was done by grip batch docking method with the help of vlife mds 3.0 software . the crystal structure of ace in complex with an irreversible inhibitor ( pdb i d : 1r4l ) for antihypertensive docking studies was obtained from the protein data bank . the parameter fixed for docking simulation was like number of placements : 30 , rotation angle : 30 , and exhaustive method . the ligand forming most stable drug - receptor complex is the one which is having minimum dock score . after docking simulation , the best docked conformer of each ligand was checked for various interactions with receptor like hydrogen bonding , hydrophobic bonding , van der waals , and charge interaction . compound a2 was found with highest negative dock score ( table 5 ) , meaning that it forms most stable drug - receptor complex amongst all the compounds . it was found to form hydrogen bonding ( figure 5 ) , hydrophobic bonding ( figure 6 ) , and van der waals interaction . amongst all the analogs , a3 was not found to exhibit hydrogen bonding with the receptor through o of oh of phosphoramidon moiety . analogs a1 , a2 , a4 , a5 , a6 , and fosinopril form hydrogen bonding with residues arg273 and thr371 by o atom of cooh group of proline moiety and o oh group of phosphoramidon moiety . some of the residues involved in this type of interaction are leu144 , asp269 , met270 , pro346 , thr347 , his345 , ala348 , thr360 , leu370 , and thr445 . thr347 and thr371 were common residues for hydrophobic bonding of all analogs including fosinopril . all the analogs including fosinopril were found to show van der waals ' interaction with common residues arg273 , thr347 , thr371 , tyr510 , and tyr515 . only a2 was found to show charge interaction ( figure 7 ) by interaction of o atom of cooh group of tetrahydroisoquinoline-3-carboxylic acid with three the ligand plot of all analogs and fosinopril is shown in figure 8 using vlife mds 3.0 . in vitro studies revealed that the analog a2 exhibited highest ace inhibition activity than other analogs including fosinopril . the percent inhibition of ace activity of different analogs is as follows : analog a2 showed highest activity followed by fosinopril , a1 , a4 , a3 , a5 , and a6 , respectively . in docking study analog a2 was found to have minimum dock score indicating highest binding with the receptor and in vitro studies also showed the same analog having highest ace inhibitory activity . hence we can conclude that analog a2 can be considered as a better molecule than fosinopril and should be investigated further .

the purpose of the present study was to determine the angiotensin - i converting enzyme inhibitory activity of few novel fosinopril derivatives which were predicted to possess better ace inhibitory activity and lesser side effects than the existing drug molecule . in vitro study was carried out to determine ace inhibitory activity of six different fosinopril analogs by spectrophotometric assay procedure . analog a2 showed the highest activity compared to other analogs and as well as fosinopril itself . docking studies of the compounds were done with the help of vlife mds 3.0 software using grip batch docking method to find out which derivative had a better docking with ace . the compounds which showed the highest negative score in docking have also exhibited good ace inhibitory activity .

the patient is a 58 years old iraqi male that referred to our center three months after an unsuccessful operation for removing a subhepatic mass , probably adrenal mass . he experienced general weakness , fatigue , and weight loss in the last several months . a scar of previous surgery in the right subcostal area ( subcostal incision ) with near 15 cm length was noted . in hematological examination , hb was 11 g / dl . in serum biochemistry , renal function tests , electrolytes , and serum levels of cortisole and aldosterone were normal . in ct scan that was done with intravenous and oral contrast ( figure 1 ) , a large mass located in the subhepatic area and intervening with the upper pole of the right kidney was noted . in the report of previous operation that was done in iraq , tumorectomy was failed due to severe adhesions of the mass to the peripheral tissues . the patient scheduled for right adrenalectomy with the midline laparotomy incision . after opening the layers , the mass was located in the lodge of upper pole of the right kidney . it was severely adherent to the peripheral tissues , due to severe adhesion of the mass to the subhepatic area , its differentiation from the kidney or adrenal was difficult . due to severe bleeding from the subhepatic bed subcapsular resection of the mass with the adherent kidney performed . its total size was 14 cm 10 cm and it weighted 2250 grams . the final histopathological examination of the mass revealed , renal cell carcinoma , clear cell type , fuhrman nuclear grading 2 ( figure 2 ) with invasion to gerotas fascia and involvement of ipsilateral adrenal which originated from the upper pole of the kidney as exophytic growth pattern ( t3n0m0 ) . the postoperative course was uneventful and the patient discharged home at fourth postoperative day uneventfully . certain anatomical structures and extra - adrenal pathological conditions may produce ct images suggesting adrenal pathology where none actually exists . disorders of the adrenal gland result in classic endocrine syndromes such as cushing syndrome , hyperaldosteronism , and pheochromocytoma . in addition , tumors of the adrenals may present with abdominal pain or as an abdominal mass . the diagnosis of these disorders requires careful endocrine evaluation , and in many patients adrenal imaging studies are required to define adrenal anatomy . in our patient , pulse , blood pressure , renal function tests , serum cortisole , electrolytes , and urinary catecholamines were all normal . however , the tumor located below the right adrenal gland during operation and the diagnosis of renal cell carcinoma ( rcc ) depended on the histopathological examination . a mass arising in the upper pole of the kidney may be difficult to differentiate from an adrenal mass [ 1 , 2 ] . additional examinations are usually necessary to confirm the nature of the lesion and to exclude the presence of life - threatening malignant disease . only 21 of 1000 specimens submitted for frozen section diagnosis were of genitourinary origin , which reflects the relatively low incidence of kidney lesions that necessitate surgery and the limited role of frozen section in the surgical management of these lesions . with the advancements in the diagnosis and treatment of renal tumors , f - fluro-2-deoxy - d - glucose positron emission tomography ( fdg - pet ) has proved to be valuable in the diagnosis and management of a variety of malignancies , but is still limited in providing detailed anatomical information . according to the literature , an adrenal incidentaloma with high fdg uptake usually indicates malignancy and requires further investigation . however , accurate localization of the adrenal gland in fdg - pet is difficult without the presence of surrounding well - visualized organs , such as the kidney or liver . if these organs have a congenital anomaly or are altered due to a previous operation , misdiagnosis can occur . a rare diagnosis when faced with a renal mass is that of an ectopic adrenal adenoma . distinguishing this entity from renal cell carcinoma has proven to be extremely difficult due to similarities in clinical presentation and radiologic findings typically , the diagnosis of an ectopic adrenal adenoma is only reached after surgical excision and careful pathologic examination . an ipsilateral adrenal mass in a patient with rcc is not uncommon and usually is a primary adrenal lesion rather than metastatic rcc . on the basis of takeda et al . study , i-131 cholesterol adrenocortical scintigrapgy is useful in the differentiation of adrenal and renal tumors when a large tumor occupies the upper pole of the kidney or adrenal gland . in fdg - pet , a normal right adrenal gland is barely visualized , whereas an adrenal lesion with intense fdg uptake usually represents malignancy [ 810 ] . finally , possible causes of the adrenal pseudotumors are as follows ; exophytic upper pole renal mass , abundant suprarenal fat , retroperitoneal tumor localized in the area of adrenal gland , prominent lobation of the hepatic lobe , or hepatic tumor , gastric diverticulum and redundant gastric fundus , fluid filled duodenum / colon , splenic lobulation , tortuous or dilated splenic arteries and veins , pancreatic tail in an unusual location or pancreatic tail mass , and focal thickening of adjacent diaphragmatic crus . although some of the imaging modalities like meticulous ct technique , mri ( with emphasis on the usefulness of three - dimensional reconstructions ) or intraoperative frozen section can help in the differentiation between large masses of adrenal and/or renal tumors , but the final diagnosis depends on the histopathological examination by skilled pathologists . in these instances , diagnostic pitfalls did not affect patient care , because these cases generally are candidates for adrenalectomy or radical nephrectomy .

there are a variety of causes of adrenal pseudotumors on computerized tomography ( ct ) scan , including upper - pole renal mass , gastric diverticulum , prominent splenic lobulation , pancreatic mass , hepatic mass , and periadrenal varices . we present a case of a large subhepatic mass that discrimination of its origin from neighborhood organs was difficult preoperatively . our patient was a 58 years old man , that three months after an unsuccessful operation in another center for a pseudoadrenal mass underwent a very difficult subcapsular tumorectomy in our center .

to ascertain the etiologic agent causing an outbreak of febrile illness with symptoms similar to chikungunya fever , we selected 3 sites in chiapas state , mexico , for sampling : tapachula , la libertad , and ciudad hidalgo ( figure 1 , panel a ) . after patients informed consent was obtained , blood samples were collected from persons whose condition met the following case definition for possible chikungunya fever : acute onset of fever > 38.5c , accompanied by severe arthralgia not explained by other medical conditions ( 6 ) . samples from tapachula were collected from patients who sought treatment at the centro regional de investigacion en salud publica , whereas in la libertad and ciudad hidalgo , researchers surveyed houses to identify potential case - patients . in total , 119 blood samples were collected , and serum was isolated by centrifugation . six samples were stored in magnapure lc buffer ( roche , nutley , nj , usa ) , which inactivates virus particles but preserves the genomic rna . map of mexico showing the 3 sites where serum samples were obtained to test for chikungunya virus in chiapas , mexico , 2014 : tapachula , la libertad , and ciudad hidalgo . viral rna was extracted from serum samples using the zr-96 viral rna kit ( zymo research , orange , ca , usa ) according to the manufacturer s protocol . one - step quantitative reverse transcription pcr ( qrt - pcr ) ( 7 ) was performed by using the taqman rna - to - ct 1-step kit ( applied biosystems , san francisco , ca , usa ) . standard plaque assays were performed for the samples positive by qrt - pcr with vero cells . anti - chikv igm - capture enzyme - linked immunosorbent assays ( elisas ) ( 8) were performed by using a chimeric eilat - chikv ( 9 ) that contained the nonstructural proteins from eilat virus and structural proteins from chikv , resulting in a structure indistinguishable from that of chikv . a sample was considered to be chikv negative if the sample was not positive by qrt - pcr or igm elisa . viral rna from 5 serum samples was sent for illumina deep sequencing and assembled by using the virus - specific hive - hexagon algorithm ( 10 ) and the ngen module in lasergene suite version 10 ( bioinformatics pioneer dnastar , inc . , madison , wi , usa ) . single nucleotide polymorphisms were analyzed by using the sequencing profiling tool in the hive suite of programs ( 10 ) . sequences were aligned in seaview ( 11 ) by using translated proteins for the open reading frames and using nucleotides for the untranslated genome regions , and all gaps were removed . bayesian phylogenetic inference was performed by using the general time reversible plus invariant sites plus gamma distribution 4 model in mrbayes ( 12 ) with 1.5 million iterations to reach congruence . partial genome sequencing of the e2 and e1 glycoproteins was performed by using traditional sanger methods on pcr amplicons on an additional 8 samples . over 100 serum samples were obtained from persons seeking treatment for chikungunya fever like illness during october december 2014 in 3 locations in chiapas , mexico ( tapachula , la libertad , ciudad hidalgo ) ( figure 1 ) . these samples were analyzed by qrt - pcr and igm - capture elisa to detect viremia and igm , respectively . no overlap occurred between the samples that were positive for chikv by qrt - pcr or those positive by igm , demonstrating the importance of the humoral response to viral clearance . with few exceptions , viremia was detectable up to 3 days after fever onset ( figure 2 , panel a ) , after which most samples were igm positive . all age groups were equally likely to be infected , as expected during infectious disease outbreaks involving a naive population ( 13 ) . a ) number of serum samples positive for chikungunya virus ( chikv ) by reverse transcription quantitative pcr ( qrt - pcr ) , for chikv igm by elisa , and negative for chikv by both methods , arranged according to day after fever onset . b ) phylogenetic tree generated by bayesian analysis showing the relationship of the complete genomic sequences of 5 chikungunya virus isolates from mexico and representative sequences from the genbank library . all nodes showed posterior probabilities of > 0.9 , except those indicated with a star . plaque assays were performed to determine serum virus titers ( table ) ; 3 qrt - pcr samples from tapachula could not be assayed because of sample limitations . mean viremia level was similar among the 3 sampled locations and ranged from < 2 log10 to 4.2 log10 pfu / ml . * chikv , chikungunya virus ; qrt - pcr , quantitative reverse transcription pcr . six patient samples that were negative for chikv by qrt - pcr were unable to be used for plaque assays or elisas because they were stored in magnapure buffer ( roche , nutley , nj , usa ) . five serum samples from diverse locations and collection dates were selected for illumina sequencing ( genbank accession nos . the strains circulating in chiapas were most closely related to asian lineage strains first detected in the caribbean ( represented by a british virgin islands isolate ) and now presumed to be circulating in much of latin america ( figure 2 , panel b ) . curiously , no novel mutations appear to have been fixed in the year since the british virgin islands isolate was collected . although the genomic sequences confirmed that the circulating virus in chiapas belonged to the asian lineage , which is primarily transmitted by a. aegypti mosquitoes ( 1 ) , we nevertheless examined the sequences for mutations known to adapt chikv for transmission by a. albopictus mosquitoes . because both mosquito species are found in chiapas ( 14 ) , adaptation of the chikv strain circulating in mexico to a. albopictus mosquitoes could place temperate regions of the eastern united states and millions of naive persons at risk for infection . none of the e2 or e1 substitutions previously reported to increase fitness in a. albopictus mosquitoes was observed in 8 additional samples analyzed by sanger sequencing ( genbank accession nos . one sample had a nonsynonymous mutation , in comparison to the january 2014 british virgin islands isolate ( genbank accession no . we found that 79% of febrile illness cases with polyarthralgia in chiapas state during late 2014 were caused by chikv . our sequencing of chikv genomes confirmed spread of an asian lineage strain from the caribbean and suggested that although chikv has circulated in the americas since 2013 , no adaptive mutations have occurred . however , continued screening for vector - adaptive mutations will be critical , especially now that strains of the ecsa lineage , which gave rise to the indian ocean lineage , have been introduced into brazil ( 4 ) .

since chikungunya virus ( chikv ) was introduced into the americas in 2013 , its geographic distribution has rapidly expanded . of 119 serum samples collected in 2014 from febrile patients in southern mexico , 79% were positive for chikv or igm against chikv . sequencing results confirmed chikv strains closely related to caribbean isolates .

gastrointestinal stromal tumors ( gists ) represent the most common mesenchymal malignancy of the gastrointestinal tract . fdg - pet / ct is therefore a common diagnostic tool : it can be valuable in interpreting ambiguous ct or mri results and allows early assessment of treatment response . especially in cases in which biopsy remains inconclusive and radical surgery seems difficult , pet / ct can be an important measure , helping to direct the management of the patient . here we present two cases of gist with unusually low / negative fdg uptake at the time of diagnosis . an 82-year - old patient had an abdominal ultrasound in order to rule out a postrenal cause of a urinary tract infection . a ct scan showed a 14 7 8 cm tumor of the anterior wall of the stomach . 1 ) confirmed a partly cystic , partly solid tumor with a maximum fdg uptake standardized uptake value ( suv ) of 2.2 . a second mass in the right rectus abdominis muscle with a maximum suv of 2.9 had disappeared in a ct scan 3 months later and was probably the equivalent of a postinterventional hematoma . 2a , b ) showed a typical gist with a mitotic count of 2/50 high power fields ( hpf ) . because of the intermediate risk profile [ size > 10 cm , mitotic count < 5/50 hpf , location of the tumor ( stomach favorable compared to small intestine 3 ) ] , no adjuvant therapy with imatinib was given . one year after surgery , there was no evidence of relapse . a 66-year - old woman presented with epigastric and right - sided chest pain . 3 ) showed a 4-cm paragastric tumor , with a locoregional suspicious lymph node of 6 mm in diameter . the tumor was fdg negative , except for a central area of the tumor with a maximum suv of 4.2 . the patient initially refused the intended surgery because of fear of perioperative risks due to other medical conditions . after 3 months , pet / ct showed a slightly larger tumor with a lower fdg uptake with a suv of maximum 3.0 . consequently , the patient agreed to be operated on . a distal gastrectomy was performed . histology revealed a typical gist of 7 cm in diameter with a mitotic count of < 5/50 hpf . because of the low - risk profile ( low mitotic count , location of the tumor ) , the patient received no adjuvant therapy with imatinib . an 82-year - old patient had an abdominal ultrasound in order to rule out a postrenal cause of a urinary tract infection . a ct scan showed a 14 7 8 cm tumor of the anterior wall of the stomach . 1 ) confirmed a partly cystic , partly solid tumor with a maximum fdg uptake standardized uptake value ( suv ) of 2.2 . a second mass in the right rectus abdominis muscle with a maximum suv of 2.9 had disappeared in a ct scan 3 months later and was probably the equivalent of a postinterventional hematoma . 2a , b ) showed a typical gist with a mitotic count of 2/50 high power fields ( hpf ) . because of the intermediate risk profile [ size > 10 cm , mitotic count < 5/50 hpf , location of the tumor ( stomach favorable compared to small intestine 3 ) ] , no adjuvant therapy with imatinib was given . 3 ) showed a 4-cm paragastric tumor , with a locoregional suspicious lymph node of 6 mm in diameter . the tumor was fdg negative , except for a central area of the tumor with a maximum suv of 4.2 . the patient initially refused the intended surgery because of fear of perioperative risks due to other medical conditions . after 3 months , pet / ct showed a slightly larger tumor with a lower fdg uptake with a suv of maximum 3.0 . histology revealed a typical gist of 7 cm in diameter with a mitotic count of < 5/50 hpf . because of the low - risk profile ( low mitotic count , location of the tumor ) , the patient received no adjuvant therapy with imatinib . a great majority of gists occur in the stomach ( 6070% ) and the small intestine ( 2535% ) , with a rare occurrence in the colon and rectum ( 5% ) , the esophagus ( < 2% ) and the appendix . approximately 50% of patients have developed distant metastasis at the time of diagnosis , mostly of the liver or the peritoneum . pet / ct is frequently used for staging purposes and is particularly indicated in ambiguous ct or mri results . the sensitivity and positive predictive value for the detection of gists by pet / ct have been described as 86 and 98% , respectively , and false - negative pet / cts were mostly related to small lesions . this and the fact that these are relatively rare tumors might lead to the impression that gists are always fdg positive . however , pet / cts of our 2 patients showed very low fdg uptake , although their tumors were large , leaving us uncertain about the diagnosis until we obtained histological proof of gist . the malignant potential of gists is difficult to predict preoperatively as risk stratification is assessed by pathological factors such as tumor size , mitotic count and lesion site . pet / ct has been described as a potential predictor for malignant potential of gists with low fdg uptake , indicating low - risk gists [ 5 , 7 ] . both of our patients had gists with low histological risk profiles , which could possibly explain the low fdg uptake . pet / ct is a sensitive and specific method to assess early response to imatinib treatment [ 8 , 9 ] as tumor size alone is unreliable for assessing the response in early imatinib treatment . consequently , when neoadjuvant imatinib therapy is considered , we believe that a baseline pet / ct is compulsory . pet / ct can not be used for therapy monitoring in patients whose baseline fdg - pet results are negative . however , in tumors with typical morphological criteria , a gist should always be considered even when fdg uptake is negative or low .

gastrointestinal stromal tumors ( gists ) are the most common mesenchymal malignancy of the gastrointestinal tract . pet / ct is a common diagnostic tool and is also used for therapy monitoring . gists typically show strong 18f - fluorodeoxyglucose ( fdg ) uptake . here we present two cases of gist with unusually low / negative fdg uptake . fdg negativity does not preclude the diagnosis of a gist .

archaea and bacteria were also believed to synthesize lysine through diaminopimelate until it was reported that the extremely thermophilic bacterium thermus thermophilus synthesizes lysine through -aminoadipate [ 58 ] . during lysine biosynthesis in the budding yeast saccharomyces cerevisiae , -aminoadipate is synthesized from 2-oxoglutarate and acetyl - coa by the enzymes lys20 or lys21 ( homocitrate synthase [ hcs ] ) , lys4 ( homoaconitase ) , lys12 ( homoisocitrate dehydrogenase ) , and -aminoadipate aminotransferase . lysine is synthesized from -aminoadipate by the enzymes lys2 ( aminoadipate reductase ) , lys5 ( phosphopantetheinyl transferase which posttranslationally modifies lys2 ) , lys9 ( saccharopine dehydrogenase , glutamate forming ) , and lys1 ( saccharopine dehydrogenase , lysine forming ) [ 1 , 4 ] . it has been unclear why s. cerevisiae has 2 hcss ( lys20 and lys21 ) . for example , homocitrate is mainly synthesized through lys21 during growth on ethanol , while under fermentative metabolism , lys20 and lys21 play redundant roles . it was recently reported that lys20 of s. cerevisiae functions in nuclear activities involving chromatin regulation that are distinct from its previously established role in lysine synthesis . lys20 of s. cerevisiae is linked to the dna damage repair process via the histone acetyltransferase esa1 and the h2a.z histone variant . i selected 71 hcss ( 31 from saccharomycotina species , 30 from pezizomycotina species , 2 from taphrinomycotina species , and 8 from basidiomycota species ) based on blastp results in the fungal genome database at ncbi ( http://www.ncbi.nlm.nih.gov/projects/genome/guide/fungi/ ) . multiple alignments were generated with clustal w. a maximum likelihood tree was reconstructed using mega version 5 . the -distributed rate was considered , and the number of discrete gamma categories was 3 . the nucleosome position profiles were compared between the promoter ( 1000 bases upstream of the translational start site ) and coding regions ( between the translational start and end site ) of the hcs genes , according to a previously described method . similarity between the two nucleosome position profiles was estimated using the spearman 's rank correlation coefficient . the hcs phylogenetic tree ( figure 1 ) indicates that the hcs gene has been duplicated multiple times in the course of ascomycete evolution . the 31 hcss of the saccharomycotina species ( ascomycetous yeasts ) are encoded in 17 organisms . in contrast , the 30 hcss of the pezizomycotina species ( filamentous ascomycetes ) are encoded in 28 organisms . thus , 14 of the 17 saccharomycotina species and 2 of the 28 pezizomycotina species have 2 hcss ( figure 1 ) . gene duplication is not found in lys1 , lys2 , lys5 , lys9 , and their homologues . in addition , no duplication was found in lys4 , lys12 , and their homologues ( data not shown ) . therefore , among the fungal lysine biosynthesis - related genes , only the hcs gene has been duplicated . phylogenetic analysis of hcss in ascomycetous yeasts showed that the s. cerevisiae hcss ( lys20 and lys21 ) are most closely related to each other ( figure 1 ) , suggesting that hcs gene duplication occurred during evolution of the genus saccharomyces . on the other hand , all saccharomycotina species except ashbya gossypii , vanderwaltozyma polyspora , and yarrowia lipolytica have duplicated hcs genes ( figure 1 ) . thus , hcs gene duplication may be related to genome duplication events in saccharomycotina [ 1618 ] . in addition to the phylogenetic analysis based on hcs amino acid sequences , i compared the nucleosome positioning of lys20 and lys21 . interestingly , nucleosomes were mapped to the hcs gene promoters more often than to the coding regions ( figure 2 ) . nucleosome position profiles in the coding regions were highly correlated ( spearman 's rank correlation coefficient = 0.833 ) between lys20 and lys21 . on the other hand , those in the gene promoter regions were poorly correlated ( spearman 's rank correlation coefficient = 0.396 ) . this result suggests that these 2 hcs genes have different regulatory systems . on the other hand , lys20 expression is most similar to lys21 expression , and lys21 is most similar to lys20 expression , based on the spell version 2.0.2 . in addition , recent comparative analyses of orthologous genes in evolutionarily close yeasts indicated that divergence of nucleosome positioning is not correlated with divergence of gene expression [ 20 , 21 ] . although hcs ( lys20 and lys21 ) is located in the nucleus of s. cerevisiae , hcs is located in the cytoplasm of penicillium chrysogenum [ 23 , 24 ] . the phylogenetic tree ( figure 1 ) showed that gene duplication is not found in basidiomycota and taphrinomycotina . in addition , gene duplication has occurred rarely in pezizomycotina , suggesting that a common ancestor of the dikarya lacked the nuclear function of chromatin regulation . considering that duplication of the hcs gene occurred in a limited number of ascomycetes , it may not be an essential event in the evolution of dikarya . i hypothesize that after divergence of the phyla ascomycota and basidiomycota , s. cerevisiae obtained hcs bifunctionality .

most ascomycetous yeasts have 2 homocitrate synthases ( hcss ) . among the fungal lysine biosynthesis - related genes , only the hcs gene was duplicated in the course of evolution . it was recently reported that hcs of saccharomyces cerevisiae has an additional function in nuclear activities involving chromatin regulation related to dna damage repair , which is not related to lysine biosynthesis . thus , it is possible that the bifunctionality is associated with hcs gene duplication . phylogenetic analysis showed that duplication has occurred multiple times during evolution of the ascomycetous yeasts . it is likely that the hcs gene duplication in s. cerevisiae occurred in the course of saccharomyces evolution . although the nucleosome position profiles of the two s. cerevisiae hcs genes were similar in the coding regions , they were different in the promoter regions , suggesting that they are subject to different regulatory controls . s. cerevisiae has maintained hcs activity for lysine biosynthesis and has obtained bifunctionality .

the patient was a 16-year - old female who presented to khatam eye center , complaining of decreased vision bilaterally . the best spectacle corrected visual acuity was 20/200 and the refraction was 28.00 diopters sphere in both the eyes . intraocular pressure ( iop ) measurements were 20 mm hg in the right eye and 35 mm hg in the left eye . slit lamp examination demonstrated microspherophakia with dislocated lenses inferonasally with phakodonesis bilaterally . in the left eye , the anterior chamber was divided by a clear membrane which was attached to the cornea superiorly and inferiorly without any visible communications [ figs . 1 and 2 ] . dilated fundus examination revealed near complete cupping of both optic nerves [ figs . 3 and 4 ] . central corneal thickness was 470 m in the right eye and 555 m in the left eye . oculus - pentacam measured anterior chamber depth of 3.20 mm in the right eye and 2.32 mm in the left eye with anterior distance to membrane of 1.64 mm . lens thickness was 4.81 and 5.54 mm in the right and left eyes , respectively . anterior chamber angle was 45 in the right eye and 2835 in the left eye ( by oculus - pentacam ) . this measurement was inaccurate in the left eye due to the pseudo angle ( between cornea and the membrane ) . gonioscopy of the left eye was inconclusive due to the membrane 's position [ figs 6 and 7 ] . chamber volume was 309 mm in the right eye and 240 mm in the left eye . endothelial cell count was 2336 cells / mm in the right eye and 1036 cells / mm in the left eye with endothelial cells on the posterior cornea of the left eye . although clinical findings were suggestive of descemet 's membrane detachment , there was no corneal edema identified in the left eye [ fig . 1 ] . the patient underwent trabeculectomy because of poorly controlled iop in her left eye , 4 months later . good iop control was obtained after surgery and has been maintained for the past 4 years . during follow - up , no cataract formation was observed . slit lamp photograph from the left eye photograph of anterior chamber of both the eyes with pentacam . left eye anterior chamber is divided by a clear membrane into anterior and posterior parts , and it was attached to cornea superiorly and inferiorly fundus photograph of the right eye showing near complete cupping of the optic nerve fundus photograph of the left eye showing near complete cupping of the optic nerve gonioscopic photograph of the right eye showing an open iridocorneal angle gonioscopic photograph of the left eye gonioscopic photograph of the left eye showing part of the membrane three years later , the patient 's sister presented to khatam eye center with a similar clinical appearance . the sister 's visual acuity in the left eye was no light perception ( nlp ) and refraction was 19.00 diopters sphere in both eyes . other clinical findings were bilateral microspherophakia , phakodonesis , and increased iop , without double anterior chamber . the similar findings in two patients were suggestive of a genetic background to their disease . microspherophakia is a rare abnormality that can be isolated or associated with other ocular or systemic disorders such as weill - marchesani syndrome , marfan syndrome , homocystinemia , alport syndrome or kleinfelter syndrome . the mechanism of glaucoma in microspherophakia is pupillary block or irritation of ciliary body by ectopic lens . chronic angle closure from anteriorly dislocated microspherophakic lenses can cause irreversible changes to the trabecular meshwork , resulting in decreased aqueous outflow . some surgeons suggest lensectomy for angle closure glaucoma and trabeculectomy for open angle glaucoma in microspherophakia patients . in our patient , trabeculectomy has resulted in a well - controlled iop for the past 4 years . medication management sufficed for controlling iop in the right eye as well as for the patient 's sister . although there were no systemic features such as short stature or joint abnormalities in the two patients discussed in this case report , both exhibited ocular findings similar to weill - marchesani syndrome . ocular findings in this syndrome are microspherophakia , ectopic lens , high myopia and glaucoma . on the other hand , these ocular abnormalities coupled with the absence of systemic disorders may suggest one of the many conditions that comprise the wide spectrum of anterior segment dysgenesis . these disorders are the result of impaired migration or development of mesenchymal cells that play a critical role in the formation of the cornea , iris and trabecular meshwork . since the lens plays an important role in anterior segment development , these abnormalities may be present with any primary disorder of the lens . management of descemet 's membrane detachment depends on the extent and location of the detachment . clinical findings include corneal edema overlying the area of descemet 's membrane detachment along with a visible retrocorneal membrane . although our patients presented with clinical features consistent with this condition , the presence of endothelial cells on the posterior cornea and the absence of corneal edema argues for an alternative explanation [ fig . 8 ] . evaluation of endothelial cells in center of cornea in both eyes with specular microscope . in the left eye there is an abnormality in the shape and density of endothelial cells double or pseudo - anterior chamber ( a space between the corneal donor and descemet 's membrane ) is one of the most dramatic complications of deep anterior lamellar keratoplasty . we present a case of unilateral primary double anterior chamber in a patient with microspherophakia without a history of surgery or ocular trauma . to our knowledge , this is the first reported case of this condition in association with microspherophakia .

we report the case of a 16-year - old woman with microspherophakia and secondary open angle glaucoma . the patient presented with a membrane dividing the anterior chamber into two segments without edema or descemet 's membrane detachment . slit lamp biomicroscopy , pentacam , and specular microscopy images were obtained . double anterior chamber is primarily found in patients with anterior chamber anomalies when there is no history of surgery or trauma .

a 76-year - old male underwent a left upper lobectomy with en bloc wedge resection of the superior segment of the left lower lobe using video - assisted thoracoscopic surgery ( vats ) for non - small - cell lung cancer of the left upper lobe . chest radiography on the second postoperative day revealed atelectasis at the remaining left lower lobe , which was not resolved despite vigorous chest physiotherapy ( fig . in addition , a high - sensitivity c - reactive protein ( crp ) test revealed an elevated crp level . fiberoptic bronchoscopy on the fourth postoperative day revealed narrowing of the left lower bronchus with purulent secretion ( fig . computed tomography showed an obstruction of the left lower lobar bronchus associated with acute angulation at the distal main bronchus . the left lower lobar artery showed right - angle angulation , and the left inferior pulmonary vein was stretched upward ( fig . the intraoperative findings showed an inferiorly kinked left lower lobar bronchus accompanied by an excessively upwardly displaced left lower lobe ( fig . in addition , there was no supportive structure around the main bronchus and lower lobar bronchus due to extensive lymph node dissection . thus , bronchopexy was performed to the pericardium with two interrupted monofilament sutures to prevent recurrence of bronchial kinking ( fig . postoperative follow - up bronchoscopy revealed an elliptical bronchial lumen without obstruction , and chest radiography showed no atelectasis of the left lung thereafter . the patient underwent a mechanical ventilation treatment for 1 month and was discharged on the 80th postoperative day with a tracheostomy . bronchial kinking after an upper lobectomy has rarely been reported and has not been well described because of the difficulty of diagnosis . however , it was recently reported that bronchial kinking occasionally develops after an upper lobectomy and subsequently exacerbates shortness of breath . bronchial kinking is commonly caused by the upward displacement of the remaining lower lobe and can be diagnosed by computed tomography . usually , bronchial kinking after an upper lobectomy is a chronic manifestation and has an upward v - shape . in the present case , however , the patient showed an acute course and downward bronchial kinking ( reverse v - shape ) . bronchial kinking caused by a downward kink of the left lobar bronchus after a left upper lobectomy has not been reported thus far . the first potential cause is an extensive dissection of lymph nodes around the lower lobar bronchus and the inferior pulmonary vein , which results in a skeletonized long segment of the bronchus . in such circumstances , the bronchus would be more susceptible to kinking because of the lack of supporting and anchoring structures . the second possibility is the wedge resection of the superior segment and the displacement of the left lower lobar bronchus . as the hilum of the lower lobe ascends excessively and nears the main hilum , the excessively long bronchus can become folded due to a pushing force . in the present case , the left lower lobar bronchus ascended higher than the left upper bronchial stump , while the remaining left lower lobe rotated counterclockwise . this condition might have exacerbated the upward displacement of the remaining lung after wedge resection . in addition , the right - angled upper lobar stump against the main and lower lobar bronchus might have facilitated the kinking . if the ligament is not detached , excessive upward displacement of the lower lobe can be prevented . shortness of breath , leukocytosis , and elevated inflammatory markers may trigger a suspicion of bronchial kinking . however , it is difficult to differentiate bronchial obstruction from pneumonia on the basis of clinical symptoms and signs ; therefore , it is essential to diagnose bronchial obstruction by bronchoscopy . computed tomography is helpful for the differentiation of bronchial kinking from lung torsion in a patient with bronchial obstruction . the final diagnosis can be made during the operation by an inspection of the lung parenchyma . in a patient with severe congestion or definitive gangrene early diagnosis is the most important for the treatment of bronchial kinking or lung torsion . thus , bronchoscopy should be performed as soon as possible , whenever there are clinical signs of bronchial obstruction to rule out bronchial kinking or lung torsion . however , more serious complications such as bronchial wall perforation , stent migration , granuloma formation , and bacterial colonization may occur . surgical correction is the definitive treatment , and surgical diagnosis is one of the important roles of surgery . when obstructive pneumonia is combined , protection of the contralateral lung while positioning the patient in a lateral decubitus position is warranted during surgery . in the present case , we decided on vats exploration and surgical correction because an intraoperative diagnosis of congestion or gangrene formation was necessary . repositioning of the lower lobe was necessary and effective in relieving bronchial kinking . by repositioning , we could also restore the pulmonary artery and vein to a more neutral position . bronchopexy was also effective in maintaining a normal shape of the bronchus after resolving the kinking . reattachment of the pulmonary ligament was done to prevent excessive displacement of the lower lobe . to prevent bronchial kinking during the initial operation , reattachment of the pulmonary ligament after pulmonary ligament lymph node dissection less extensive dissection of lymph nodes around the lower lobar bronchus may prevent acute reverse v - shaped bronchial kinking . in conclusion , given that bronchial kinking can occur after an upper lobectomy , bronchial kinking should be one of the differential diagnoses in patients with signs of bronchial obstruction .

a 76-year - old male underwent a left upper lobectomy with wedge resection of the superior segment of the left lower lobe using video - assisted thoracoscopic surgery ( vats ) for non - small - cell lung cancer of the left upper lobe . he presented with shortness of breath , fever , and leukocytosis . chest radiography showed atelectasis at the remaining left lower lobe . bronchoscopy revealed narrowing of the left lower bronchus with purulent secretion , and computed tomography showed downward kinking of the left lower lobar bronchus . he underwent exploratory vats , and intraoperative findings showed an inferiorly kinked left lower lobar bronchus with upward displacement of the left lower lobe . after adhesiolysis , the kinked bronchus was straightened , and bronchopexy was performed to the pericardium to prevent the recurrence of bronchial kinking . also , the inferior pulmonary ligament was reattached to prevent upward displacement . postoperative follow - up bronchoscopy revealed no evidence of residual bronchial obstruction , and chest radiography showed no atelectasis thereafter .

a 17-year - old boy presented with severe headache of 2 weeks duration and sudden decrease in vision in both eyes for 10 days , right eye followed by left within 5 days . on examination , right eye had no perception of light and left eye had of counting fingers close to face vision . pupil was fixed dilated and nonreacting to light in right eye and left eye pupil was normal in size , but had ill - sustained reaction to light . fundus examination revealed subtle temporal pallor of optic disc in both eyes [ fig . 1 ] . he had undergone computed tomography of the brain and orbit which revealed a lesion in the spheno - ethmoid sinus which was slightly hyperdense . bone windows showed erosion of the roof of ethmoidal sinus [ fig . 2 ] . mri of the brain revealed a lesion in the spheno - ethmoid sinuses and located between the two internal carotid arteries which was hyperintense on t1w images and iso- to hyperintense on t2w images [ fig . picture of the optic nerve head right ( a ) and left ( b ) preoperative ct scan of the brain and paranasal sinuses coronal section ( a ) with bone cuts ( b ) shows lesion in the spheno - ethmoid sinus which is slightly hyperdense and erosion of bone in the roof of the ethmoidal sinus preoperative mri of the brain t1w ( a and b ) : axial sections showing hyperintense lesion in the spheno - ethmoid sinuses and located between the two internal carotid arteries . t2w ( c and d ) : axial and coronal sections showing iso- to hyperintense lesion patient was immediately referred to neurosurgeon for surgical intervention , but there was a delay of 2 days by the patient . when he presented to the neurosurgeon , he had developed right oculomotor nerve paresis in addition to the sixth nerve paresis . right trans - ethmo - sphenoidal approach and total evacuation of the mucocele under general anesthesia . one week after surgery , the right eye condition was same , but left eye showed vision improvement to counting fingers at 4 m ; pupil was sluggishly reacting to light . one month after surgery the right eye showed only abducent nerve paresis , while oculomotor nerve paresis was completely resolved ; pupil showed relative afferent pupillary defect . left eye showed improvement in vision of 6/6 , n6 , and pupillary reaction was normal . on 2 months follow - up , both eyes had full range of ocular movements [ fig . 4 ] . postoperative clinical picture of the patient showing full range of ocular movements postoperative ct scan of the brain and paranasal sinuses axial section ( a ) with bone window ( b ) showing complete excision of the lesion mucoceles are cyst - like lesions lined with respiratory epithelium that most commonly produce bone destruction within the paranasal sinuses . approximately two - thirds of all mucoceles involve the frontal sinuses , and the majority of the remainder involve the ethmoidal sinus . sphenoidal mucoceles occur rarely and have an incidence of 1% of all paranasal sinus mucoceles . anterior clinoid mucoceles causing optic neuropathy and cranial nerve palsies are reported in the literature , but sphenoid mucoceles causing both optic neuropathy and ophthalmoplegia are very rare and we could not find any similar report in the literature . the most common is headache ( frontal or retroorbital ; 70% of patient ) and second is visual disturbance ( 65% of patients ) . other visual symptoms include diplopia , ocular muscle paresis , exophthalmus , and complete visual loss . the slow and silent expansion of a mucocele may be unsuspected until bone is eroded and it impinges on adjacent structures . proposed theories for development of mucocele include chronic infection , allergic sinonasal disease , trauma , previous surgery , and in some cases the cause remains uncertain . lund and milroy proposed that the obstruction to sinus outflow in combination with superimposed infection caused the release of cytokines from lymphocytes and monocytes . the cytokine release would stimulate fibroblasts to secrete prostoglandins and collagenases , which in turn could stimulate bone resorption leading to expansion of the mucocele . a large mucocele produces a classic radiographic appearance of an enlarged distorted sinus with a bony defect representing a breakthrough into the adjacent structures . the ct features include a homogeneous iso- to hyperdense expansile mass , which completely fills the sinus cavity and surrounding structures . on contrast administration , there is no increase in the density of mucocele due to its avascular mucoid content . differential diagnosis includes hypophyseal , tumors , craniopharyngioma , meningioma , chordoma , dysgerminoma , cholesteatoma , and tumors arising from nasopharynx , sinuses , and base of the skull . surgical treatment options consists of trans - nasal endoscopic , trans - ethmoidal , trans - antral , trans - palatal , and trans - septal . the degree of improvement in visual acuity after an operation depends on visual acuity before the operation , the mode of development of the mucocele , and the time from onset of the disease until surgery . have described that if surgery is delayed for more than 610 days after the start of visual loss and if there is optic atrophy , the visual prognosis seems to be poor . the above presented case had symptoms in the right eye for 15 days and in the left eye for 10 days . although there was no optic atrophy in the right eye , the vision did not improve . poor recovery of the right eye could be due to comparative delay in presentation and intervention . mucoceles , though rare , have to be considered in the differential of visual deterioration . early surgery of these benign lesions will lead to complete recovery even in patients with poor vision .

sphenoid sinus mucocele comprises only 2% of all paranasal sinus mucoceles . in literature , there is a case report on sphenoidal mucocele causing bilateral optic neuropathy , with unilateral partial recovery and cranial nerve palsy , but we did not come across any literature with bilateral optic neuropathy and ophthalmoplegia together caused by spheno - ethmoidal mucocele . we present such a rare case of spheno - ethmoidal mucocele causing bilateral optic neuropathy and unilateral sixth nerve palsy who had postsurgery , unilateral good vision recovery , and complete resolution of sixth nerve palsy .

a 14-year - old siamese neutered male cat was evaluated for anorexia and a left periorbital mass . skull radiographic findings showed a well - defined lesion resembling new compact bone formation without destruction . the tumor was removed intact with a normal tissue margin of at least 1 cm . there were no postsurgical complications . periorbital tumors are infrequently diagnosed in companion animals and most are malignant . in this case , the diagnosis was orbital osteoma . the most commonly affected bone for osteoma in cats is the mandibular bone ; few cases have been identified in orbital bones . orbital surgery has the potential to be challenging owing to complex anatomy , difficult exposure and the tendency to bleed . surgical complications are common . in this case , although the disease was advanced , subtotal orbitectomy was successfully performed . periorbital tumors are infrequently diagnosed in companion animals and most are malignant . diagnosis is based on clinical signs , radiographic features and histological diagnosis . orbital surgery has the potential to be challenging owing to complex anatomy , difficult exposure and a tendency to bleed . the aim of this study is to report a successful subtotal orbitectomy in a cat with orbital osteoma . a 14-year - old siamese neutered male cat was evaluated for anorexia and a left periorbital mass . the left eye could not be visualized . a complete blood count ( cbc ) and the results from the cbc did not reveal any abnormality , and the only abnormal value on the biochemical panel was a mildly elevated creatinine level ( 2.5 mg / dl ; reference interval 0.91.8 mg / dl ) . skull radiographic findings showed a well - defined lesion resembling new compact bone formation without destruction ( figure 2 ) . in thoracic the cat was premedicated intramuscularly with acepromazine ( 0.03 mg / kg ) and methadone ( 0.2 mg / kg ) by subcutaneous injection 15 mins prior to induction . anesthesia was induced with propofol ( 4 mg / kg ) by intravenous injection and maintained with isoflurane . methadone was given intramuscularly q6h for 24 h. the cat was positioned in ventral recumbency . the tumor was removed intact and surrounded by at least a 1 cm margin of normal tissue . it was a circular , well - defined mineral mass measuring 3.2 cm in diameter at the rostral aspect of the left orbit . an osteotomy of frontal bone combined with an osteotomy of maxilla and zygomatic bone were performed using an osteotome and mallet . bony margins of the orbit were removed en bloc with the eye and structures within the orbit . the surgical defect was closed by two single - pedicle advancement flaps ( dorsum of head and cheek ) and apposition of subcutaneous tissues and skin . microscopically , the lump was ovoid and well - delineated from surrounding dermal connective tissue . the lesion was composed of numerous trabeculae consisting of both woven and lamellar bone , osteocytes of normal appearance being present in lacunae within the osseous matrix . amoxicillin ( amoxicilin ; medley ) 15 mg / kg orally q12h for 10 days ; dipyrone 25 mg / kg orally q12h for 3 days ; and tramadol ( dorless v ; agener ) 2 mg / kg orally q12h for 7 days , were prescribed , and an elizabethan collar was recommended . six percent of all primary bone tumors commonly affect the skull of dogs and cats . few cases of osteoma have been described in cats and they are rarely identified in the orbit . the most commonly affected bone is the mandible . diagnosis for orbital osteoma is based on clinical signs ( usually not painful on palpation ) , radiographic features ( well - circumscribed , dense bony projections ) and histological diagnosis ( similar to reactive bone ) . it is a compact bone formation without destruction and with continuous growth at a slow rate . orbitectomy is a painful surgical procedure.effective pain management generally involves a balanced or multimodal strategy using several classes of pain - modifying medications . a fentanyl patch was the better option for this case ; however , it was not available to be used in this cat . fentanyl patches provide long - lasting analgesia and can be placed prior to surgery in order to be effective postoperatively . non - steroidal anti - inflammatory drugs ( nsaids ) are a mainstay for the management of chronic pain , as well as for perioperative use . nsaids are avoided in cats with renal dysfunction because an important side effect associated with nsaids is nephrotoxicity . the cat had a mildly elevated creatinine level , so nsaids were avoided . because animals are non - verbal and can not self - report the presence of pain , appropriate pain management is required and includes several classes of pain - modifying medications . if the tumor remains static and asymptomatic it may be possible to consider no treatment but monitor it closely . orbital surgery has the potential to be challenging owing to the complex anatomy , difficult exposure and a tendency to bleed . in this case , the osteoma was removed without difficulty and complication . surgical complications like blindness in the contralateral eye , neurological signs , cardiopulmonary arrest , facial swelling , hemorrhage and dehiscence may develop after orbitectomy . in this case , no postsurgical signs were observed . the osteoma was completely removed without complications and the cat s quality of life was subjectively better than before surgery . early intervention is recommended for cats with osteoma , as surgical excision of the mass may be curative . subtotal orbitectomy was a successful treatment in this case , even though the mass was advanced ( 3.2 cm in diameter ) .

case summarya 14-year - old siamese neutered male cat was evaluated for anorexia and a left periorbital mass . skull radiographic findings showed a well - defined lesion resembling new compact bone formation without destruction . a subtotal orbitectomy was indicated . the tumor was removed intact with a normal tissue margin of at least 1 cm . there were no postsurgical complications . histopathologic examination revealed an osteoma . the cat returned to normal appetite and activity 15 days after surgery . six months after surgery , there were no gross signs of recurrence.relevance and novel informationperiorbital tumors are infrequently diagnosed in companion animals and most are malignant . in this case , the diagnosis was orbital osteoma . the most commonly affected bone for osteoma in cats is the mandibular bone ; few cases have been identified in orbital bones . orbital surgery has the potential to be challenging owing to complex anatomy , difficult exposure and the tendency to bleed . surgical complications are common . in this case , although the disease was advanced , subtotal orbitectomy was successfully performed .

. the cyst may either be congenital ( developmental ) or acquired . to a large extent , the differential diagnosis depends on its location in the mediastinum ( anterior , middle or posterior compartments ) . the cysts of non - infective etiology may be complicated by secondary infection and certain primary infectious conditions such as hydatid disease can also present with mediastinal cysts . a 14-year old boy presented with a history of dry cough and progressively increasing breathlessness for a duration of three months . this was associated with dysphagia and stridulous noises on lying down ( both supine and prone ) . a chest radiograph was performed , which revealed a widening of the superior mediastinum [ figure 1 ] . computed tomography ( ct ) of the chest revealed the presence of a multiloculated cystic mass located posterior to the trachea , starting at the level of the thoracic inlet and extending to the level of the carina [ figure 2 ] . laboratory investigations revealed anemia ( hemoglobin 10.2 g / dl ) , with normal leucocytes ( 5,800/l ) and platelet counts ( 232,000/l ) . he had deranged liver functions ( bilirubin 0.3 mg / dl , aspartate transaminase 54 u / l [ n , 0 - 35 ] , alanine transaminase 103 u / l [ n , 0 - 35 ] , and alkaline phosphatase 212 u / l [ n , 0 - 130 ] ) . chest radiograph showing mediastinal widening in the superior part ( a and b ) computed tomography ( ct ) of the chest depicting the cystic lesion posterior to the trachea the following diagnoses were considered : ( a ) secondarily infected foregut duplication cyst , ( b ) bronchogenic cyst , ( c ) hydatid cyst , and ( d ) cystic schwannoma with secondary infection . however , it was deemed unlikely , as the patient did not have any fever or back pain , and the chest tomogram did not show any vertebral involvement . ultrasound - guided needle aspiration from the mass revealed well - defined epithelioid cell granulomas and multinucleated giant cells , with necrotic material in the background . the patient was started on antituberculosis treatment with rifampicin , isoniazid , pyrazinamide , and ethambutol in doses according to his weight . his symptoms improved and he became completely asymptomatic in a month . a ct chest repeated at three months showed complete disappearance of the mass [ figure 3 ] . ( a and b ) computed tomography ( ct ) of the chest showing complete resolution of the lesion after treatment mediastinal involvement with tuberculosis usually occurs in the form of enlarged lymph nodes . on a contrast - enhanced ct , tuberculous mediastinal lymph nodes show a central hypodense area , which is suggestive of central necrosis . on an endobronchial ultrasound , this occurs either as a result of extensive necrosis in the conglomerate tuberculous lymph nodes or as a result of a direct extension from a cervical cold abscess . the location of tuberculous mediastinal abscess is usually in the anterior or middle mediastinum , secondary to extensive involvement of the lymph nodes . the appearance of a cystic lesion in the retrotracheal and paravertebral area without involvement of the spine is unusual . the index patient did not have back pain and there was no spinal involvement , as seen on the chest ct . we did not perform any specific imaging of the spine , as the diagnosis was already established and the clinical suspicion of spinal tuberculosis was low in view of lack of symptoms and no spinal involvement on ct . in conclusion , tuberculosis may present as a cystic lesion in the posterior mediastinum without any vertebral involvement . it may respond completely to anti - tuberculosis treatment without the need for surgical drainage .

cystic lesions of the mediastinum may be congenital or acquired . the differential diagnosis depends on their location in the mediastinum . cysts in the posterior mediastinum are generally developmental cysts and are neurogenic or of foregut origin . we report the case of a 14-year - old boy , who presented with dry cough and progressively increasing breathlessness , and was found to have a cystic lesion in the posterior mediastinum . fine needle aspiration from the cyst helped make a diagnosis of tuberculosis .

arteriovenous fistulas ( avfs ) are quite rare in the head and neck region and most of them are acquired , resulting from blunt or penetrating trauma . to the best of our knowledge , only 21 cases of congenital external carotid - external jugular avf have been reported in the literature ( 1 ) . nowadays , with the advent of computed tomography ( ct ) , it has become an integral part of evaluation in a wide spectrum of vascular diseases and among them avf can be confidently diagnosed on obtained state - of - the - art images . we report a rare case of congenital external carotid - external jugular avf with its characteristic imaging features on contrast - enhanced ct in an adult patient who was referred for the assessment of cardiac valvular disease . a 42-year - old woman presented with exacerbations of palpitation and dyspnea that she had been suffering from since her childhood . she had no history of trauma . upon admission , the patient s blood pressure was normal and an apical systolic murmur was heard during cardiac auscultation . also detected was a thrill at the left side of the neck and an audible bruit over the left mandibular angle . electrocardiography showed evidence of left ventricular hypertrophy and the chest x - ray revealed mild cardiac enlargement . transesophageal echocardiography ( tee ) demonstrated severe left ventricular enlargement , mild right ventricular enlargement , severe mitral regurgitation , moderate tricuspid regurgitation and moderate pulmonary hypertension . with respect to chronic symptoms , attributable to valvular problems , mitral valve replacement was planned . furthermore , abnormally dilated vascular structures with turbulent flow on the left side of the neck were also visualized on tee . presence of bruit in the physical exam as well as echocardiographic findings raised strong suspicion of avf ; therefore , the patient underwent ct - angiography of the cervical vasculature . consequently , an avf was identified between the distal part of the left external carotid artery and the left external jugular vein . significant dilatation of the left common carotid artery , 20 mm in diameter , and the left external carotid artery were seen , accompanied by dilatation and tortuosity of the left external jugular vein ; however , the major branches of the left external carotid artery had normal size and appearance ( figure 1 ) . an incidental finding was made of hypoplasia of the mid and distal portions of the ipsilateral internal jugular vein . thereafter , the patient was scheduled for ligation of the external carotid - external jugular avf before mitral valve replacement . as a definition , any direct communication between an artery and a vein is defined as avf . at first , the artery may have an increased number of abnormal branches and it may become dilated . in addition , it is commonly seen that the major draining vein is widely dilated , and it may become tortuous in appearance . congenital avf formation , neoplasms and degenerative vascular disease are among other etiologies of this entity . furthermore , generalized connective tissue disorders , e.g. ehlers - danlos syndrome or type i neurofibromatosis , have also been associated with spontaneous avf ( 2 , 3 ) . the most common symptoms are a pulsatile mass , swelling , and pain and untreated fistula may lead to cardiac failure , fistula rupture , or emboli ( 4 - 7 ) . in the literature , three patients with avf between the external carotid artery and the external jugular vein presented with vertigo ( 8) . our patient ; however , had no neurological symptoms and no evidence of cardiac failure had developed yet . diagnosis of avf is usually not difficult , but the vessels involved should be precisely identified . ct - angiography has been shown to be a robust noninvasive alternative to conventional angiography for the initial assessment of vascular anomalies and it markedly enhances the diagnostic accuracy in avfs . furthermore , with the advent of multi - detector row ct and three - dimensional post processing , vascular abnormalities can be more accurately depicted after a single intravenous bolus of contrast agent administration ( figure 2 ) . a delicate ct - angiography of the surrounding anatomy and vascular structures yields more detailed information about the feeding arterial supply , vascular drainage , collateral circulation , and possible fistula - unrelated vascular malformations ; thus , it provides a road map for the possible surgical procedures ( 9 ) . ct - angiography of our patient showed avf between the left external carotid and the external jugular vein associated with significant dilatation and tortuosity of these vessels . the mentioned fistula was confidently diagnosed with the use of ct - angiography , and it was also confirmed after open surgery of the neck .

arteriovenous fistula ( avf ) between the external carotid artery and external jugular vein is extremely rare , with only few cases reported in the literature so far . most of these avfs have been either iatrogenic or secondary to previous trauma.herein , we report a 42-year - old woman with congenital avf between the external carotid artery and external jugular vein , presenting with palpitation and dyspnea . the patient was suffering from mitral and tricuspid regurgitation . on physical examination , a thrill on the left side of the neck and an audible bruit over the left mandibular angle were detected.the possibility of abnormal avf was considered and it was confirmed on contrast - enhanced computed tomography ( ct ) , inferring that this modality is not only fast and non - invasive , but also accurate in detecting vascular abnormalities .

megalocornea is a nonprogressive bilateral congenital enlargement of the anterior segment in the absence of raised intraocular pressure.1 it can occur in three forms simple megalocornea unassociated with other ocular abnormalities ; anterior megalophthalmos with megalocornea ; and iris and angle abnormalities as well as buphthalmos in infantile glaucoma . anterior megalophthalmos is a rare hereditary disorder characterized by presence of megalocornea ( horizontal corneal diameter more than 13 mm ) in association with enlarged lens iris diaphragm and ciliary ring.2 secondary complications include presence of iridodonesis , miosis , atrophy of iris stroma , and cataractous lens . marfan 's syndrome , apert syndrome , down syndrome , and mucolipidosis type 2 are some systemic associations seen with this condition . premature development of cataract is the most common cause for visual impairment in this condition . cataract extraction in these patients is prone to complications due to presence of weak zonules and ciliary ring enlargement . one of the major challenges in the management of cataract is placing the correct size of intraocular lens ( iol ) in the capsular bag to prevent iol decentration . possible options would include use of a large custom - made posterior chamber ( pc ) iol or use of anterior chamber iris sutured iol.34 this case report describes the use of standard posterior chamber iol of larger diameter in the management of cataract in anterior megalophthalmos . a 40-year - old healthy woman presented with gradual progressive painless diminution of vision in both eyes for 1 year duration her best corrected visual acuity ( bcva ) od was 6/18 and light perception with projection , os . anterior segment examination in both eyes revealed presence of megalocornea [ figure 1 ] and clear cornea with mild pigment on endothelium . anterior chamber were deep bilaterally . there was grade i nuclear sclerosis ( by lens opacity classification system ii ) od and total cortical cataract with phacodonesis os . gonioscopy showed open angles with broad pigmented trabecular meshwork and anterior embryotoxon [ figure 2 ] . the central corneal thickness ( cct ) using ultrasound pachymetry was 0.445 mm od and 0.465 mm os . horizontal corneal diameter measured by using a castroviejo calipers biometric data of a patient with anterior megalophthalmos gonioscopy photograph showing presence of broad pigmented portion of trabecular meshwork manual small incision cataract surgery with use of capsular tension ring ( ctr ) with peribulbar block was planned for the left eye . a single piece rigid poly methyl methaacrylate posterior chamber iol of + 15.00 d power , with 6.5 mm diameter optic and an overall diameter of 13.5 mm ( aurolab , tamil nadu , india ) was implanted [ figure 3 ] . follow up was performed at 1 , 3 , 4 , 6 , 12 weeks postoperatively . at 3 months postoperatively , bcva in the left eye was 6/9 with + 2.00 d sphere and + 1.0 d cylinder at 90 and the pciol was well centered . per operative photograph anterior megalophthalmos is a rare inherited condition that is characterized by the presence of megalocornea , ciliary ring enlargement and secondary effects of iridodonesis , miosis , atrophy of iris stroma , and occurrence of cataract.5 x - linked genetic transmission is found in 50% of cases of anterior megalophthalmos ( possibly located on xq21.3-q22 ) , autosomal transmission in 40% , and sporadic transmission in the remaining 10% . it is commonly seen in males ( 90% ) because x - linked inheritance is common . our case being a female , with a negative family history probably suggests a sporadic occurrence . she had megalocornea , deep anterior chamber , hypoplasia of iris stroma as pupils failed to fully dilate pharmacologically as well as anterior embryotoxon with presenile cataract fitting into the diagnosis of anterior megalophthalmos . the lens in anterior megalophthalmos is of normal size,6 but the ciliary ring is enlarged , which is responsible for weakened zonules and subluxation of lens . one of the major challenges in the management of cataract in patients with anterior megalophthalmos is prevention of iol decentration . various techniques have been recommended including placement of iris clip iol in the posterior chamber7 and custom iol.8 javadi et al.9 have reported safe implantation of standard iol ( 13.4 mm in length with 7 mm optic ) in six eyes without complications . tsai et al.10 have also reported the use of standard iols ( 13 mm in length and 6 mm optic ) in a case of anterior megalophthalmos who underwent phacoemulsification . in our case we were able to implant a standard iol ( 13.5 mm overall length with 6.5 mm optic ) successfully . introduced in 1991 , ctrs have become indispensable in stabilizing the capsular bag in the presence of generalized weakness or dehiscence of less than 150.11 hence , placement of conventional ctrs are not limited to only zonular dialysis and can be used in generalized weakness of zonules . even though there was phacodonesis , zonular dialysis was not evident and iol implantation was possible in our case without the use of ctr . zare et al.12 have suggested preoperative use of ultrasound biomicroscopy ( ubm ) in anterior megalophthalmos to measure the actual size of the capsular bag . zare et al.12 were able to implant a standard foldable iol as the capsular bag diameter in their case was found to be normal by ultrasound biomicroscopy . the hyperopic shift could be explained with greater posterior shift of the iol due to long zonules . in our case it was not possible to assess the capsular bag diameter , we assume that it would have been of normal size as there was no iol decentration for the entire follow - up period . however , there is relative paucity of female cases ( such as the current case ) in the literature . however , good visual rehabilitation can be achieved with standard iol in these anatomically challenged eyes , even in centers with limited or no access to investigative techniques such as ubm .

we report a case of 40-year - old female who presented with diminution of vision in both eyes . ocular evaluation showed presence of bilateral megalocornea with deep anterior chamber , iridodonesis , cataract , and anterior embryotoxon . she was diagnosed with bilateral anterior megalophthalmos . she underwent an uneventful cataract extraction with standard posterior chamber intraocular lens implantation of overall large diameter in the left eye . zonular dialysis was not evident intraoperatively despite the presence of iridodonesis . postoperatively the intraocular lens was well centered throughout follow up . this case report reviews this rare disorder and highlights successful visual rehabilitation .

periodontal disease affects adolescents and young adults in varying degrees worldwide.[14 ] dental students are representative of the educated , urbanized , influential , and motivated class of individuals . the effect of joining a dental profession and its relationship with the personal level of oral health has not shown any correlation . the younger individuals are more concerned with their major task at hand education and establishing in a job or profession ; in this process the individuals have limited concern for their health and least for dental health . the dental students are expected to be well conversant with issues of health and disease . it is important that the health professional must be well motivated and knowledgeable to impart appropriate attitudes in their community . the dental professionals are faced with the dilemma of diagnosing conditions relating to periodontal tissues as there are conflicting perceptions of disease , progress , and severity in young adults . the integrity and position of the junctional epithelium shall be the major criteria for determining the status of periodontium . any breach in the attachment or apical migration of the junctional epithelium marks the initiation of periodontal disease and can be used for its detection . attachment loss of 1 mm or more can be a predictor for ensuing bone loss . an epidemiological study was planned to establish levels of periodontal conditions in dental students , and to assess the knowledge and understanding of management among affected individuals . three hundred and twenty - nine dental students of first to fourth year were screened for the presence of periodontitis using the criteria of clinical signs : presence of bleeding on probing and attachment loss of 2 mm . all four surfaces ( mesiofacial , midfacial , distofacial , and lingual ) of all posterior teeth were examined . the oral hygiene level of affected students was assessed by using oral hygiene index simplified ( ohi - s ) . the presence of proximal caries and mottled enamel in relation to involved teeth was examined . bitewing radiographs of the affected individuals were evaluated and the cemento - enamel junction ( cej ) to the crest of bone distance was measured . the subjects were questioned for the awareness of disease , and their understanding of management of disease . the mean and standard deviation ( sd ) the sample consisted of 329 dental students in the age group 1722 years [ table 1 ] . sixty - three ( 19.1% ) students showed clinical signs of disease [ figure 1 ] . twenty - one ( 6.3% ) students had cej to the bone crest distance of 2 mm on bitewing radiographs . eighteen ( 28.6% ) students out of 63 clinically affected students were aware of the presence of disease . distribution of sample distribution of clinically affected students observations of various parameters on students affected with periodontitis various studies on loss of attachment of 2 mm have reported the prevalence of 2.84.9% in sweden , 24.5% in usa , 88.7% in new mexico , and 014% in england in 12- to 19-year - old individuals . the bone loss of > 2 mm on bitewing radiographs was reported as 13.5% in sweden , 3.311.3% in norway , 28% in brazil , and 89.2% in new mexico . the cej to bone distance of 2 mm was observed in 6.3% individuals in our study . a small number of individuals ( 0.9% ) showed cej to bone margin distance of 4 mm or more . on radiographic evaluation , the mesial surface of the mandibular first molar was the most affected site in the present study whereas in some other studies , the mesial aspect of the maxillary first molar appeared to be the most frequently affected site.[1113 ] one case ( 0.3% ) showed characteristic arc - like bone loss of localized aggressive periodontitis . the attachment loss of 2 mm was seen in 22.3% of preclinical students and 16% of clinical students . males ( 27.3% ) were affected more than females ( 16.8% ) in our clinical assessment . this is in accordance with other studies . on evaluating bitewing radiographs , males ( 8.21% ) were affected more than females ( 5.8% ) in our study similar to observations made in other studies . ohi - s scores did not necessarily show any association with the presentation of disease . the proximal caries ( nine cases ) and mottled enamel with a roughened surface ( six cases ) had an association with the attachment loss of 2 mm . the prevalence of periodontal disease had no relationship with the year of study , i.e. preclinical or clinical . the study of prevalence of disease in dental students has shown similar trends in other study . the study has brought forward the information regarding the prevalence of disease in young adults and association with caries and mottled enamel . the clinical attachment loss of 1 mm or more precedes the changes in the crestal bone level ; highlighting such information is important for careful examination and appropriate diagnosis . since the disease progresses silently , the risk assessment of young individuals is paramount to the health . a study of prevalence of periodontal disease , related factors , and knowledge and understanding of management was conducted on 329 dental students . a total of 63 ( 19.1% ) students showed the clinical signs of disease , and 21 ( 6.3% ) had a threshold of 2 mm cej to the bone crest distance . the presence of proximal caries was associated with attachment loss in nine cases . the mottled enamel with surface roughness had an association with the attachment loss in six cases . the need for careful diagnostics is emphasized .

a cross - sectional study was conducted on dental students at bahadurgarh , haryana , for the assessment of periodontal health and possible associated factors . a total of 329 students were examined clinically by periodontists . students with bleeding on probing and attachment loss 2 mm were assessed further for extent of bone loss radiographically . the awareness of disease and knowledge of management was also assessed . sixty - three ( 19.1% ) students showed clinical signs of disease and 21 ( 6.3% ) had cemento - enamel junction ( cej ) to the bone crest distance of 2 mm . proximal carious lesions were observed in nine cases of attachment loss . mottled enamel with a roughened surface had association with attachment loss . the clinical students showed reasonable knowledge of disease and management . the need to strengthen the diagnostics for early detection of periodontal disease in young adults is suggested .

restrictive cardiomyopathies are defined as restrictive ventricular physiology in the presence of normal or reduced diastolic volumes ( of one or both ventricles ) , normal or reduced systolic volumes , and normal ventricular wall thickness . usually , patients develop severe symptoms of heart failure over a short period , and the majority die within a few years following diagnosis unless they receive a cardiac transplant . genetic investigations have shown that the condition may be caused by mutations in sarcomeric disease genes or genes for desmin and even may coexist with hypertrophic cardiomyopathy in the same family as seen in our case . a 25-year - old woman , gravida 4 , para 1 , living 1 , abortion 2 , presented to us at 34 weeks of gestation with antepartum hemorrhage . her past pregnancies were uneventful . on examination , pulse was 112/min and blood pressure was 120/80 mm hg . respiratory system showed bilaterally equal , normal vesicular breath sounds with no rhonchus or crepitations . obstetric scan showed a single live intrauterine gestation without any evidence of placenta previa / abruption . her electrocardiogram ( ecg ) showed biatrial enlargement and partial right bundle branch block pattern . echocardiography showed dilated left and right atria , concentric left ventricular hypertrophy with mild mitral and tricuspid regurgitation , normal left ventricle systolic function , ejection fraction 60% , pulmonary artery hypertension ( pulmonary artery systolic pressure-45 mmhg ) with small pericardial effusion [ figure 1 ] . doppler showed increased early diastolic filling ( e ) to atrial filling ( a ) ( e / a ) ratio ( 2.4 ) of the mitral inflow signal , mitral flow ( e-0.85 , a-0.35 ) , and pulmonary flow ( 0.90 m / s ) [ figure 2 ] . in light of these results , genetic counseling of the family was done and genetic testing was advised , but the patient declined testing . connective tissue disorders were ruled out ( antinuclear antibody and antiphospholipid antibody were negative ) . metabolic disorders were ruled out by serum creatine phosphokinase ( cpk ) ( 112.8 u / l ) and serum lactate ( 1.4 mmol / l ) which were found to be normal . she went into spontaneous labor 10 days later , and she had a preterm vaginal delivery of a live female baby weighing 2 kg . the patient is on diuretics and she continues to be on regular clinical follow - up . echocardiography reveals biatrial enlargement with normal systolic function doppler shows increased early diastolic filling to atrial filling ratio ( 1.6 ) common etiologies of rcm include infiltrative ( amyloidosis , sarcoidosis ) , storage ( hemochromatosis ) , noninfiltrative ( scleroderma , idiopathic ) , and endomyocardial processes ( endomyocardial fibrosis , hypereosinophilic syndrome , carcinoid , radiation , malignancy , anthracycline toxicity ) . feld and caspi reported a cardiomyopathy with variable hypertrophic and restrictive features that affected three generations of a family with a shared human leukocyte antigens haplotype . in our case , the sibling had hypertrophic cardiomyopathy . depending on the stage of the disease , the fourth heart sound or third heart sound may be present . in our patient , ecg may show progressive atrioventricular ( av ) conduction delay due to disease of the av node , or his - purkinje system is common in many genetic diseases that affect the myocardium , and in this case , ecg showed biatrial enlargement and partial right bundle branch block pattern . certain signs and symptoms that could raise the suspicion of specific diagnoses grouped according to the main echocardiographic phenotype such as learning difficulties and mental retardation ( noonan syndrome ) , paresthesia / sensory abnormalities / neuropathic pain with bilateral carpal tunnel syndrome ( amyloidosis ) and muscle weakness generally distal progressing to proximal ( desminopathies ) . in our patient the classical doppler finding is the restrictive pattern - mitral inflow pattern e / a > 1.5 as seen in our case . increased creatine kinase is seen in desminopathies , and increased proteinuria is seen in amyloidosis . in our case , rectal biopsy was done and it did not show any deposits of amyloidosis and cpk was normal . cardiac transplantation can be considered in patients with refractory symptoms in idiopathic or familial restrictive cardiomyopathies . the present case report shows the importance of careful evaluation of patients so that risk stratification can be done to avoid adverse disease complications and diminish the rate of sudden death .

restrictive cardiomyopathy ( rcm ) is a relatively rare form of cardiomyopathy with high mortality which is characterized by impaired filling of the ventricles in the presence of normal wall thickness and systolic function . rcm is generally seen in association with infiltrative , storage , or systemic diseases . we present a rare case of familial rcm with pregnancy which was successfully managed .

hydatid cyst disease is a zoonotic infection that results from tissue infestation with the larval stage of the parasite echinococcus granulosus . the definitive hosts of the parasite are dogs , whereas the intermediate hosts are sheep and other ruminants . it is an endemic disease that particularly affects people who live in rural areas in intimate contact with cattle . the primary location is mostly in the liver ( 75% ) and lungs ( 15% ) , with only 10% occurring in other parts of the body . primary hydatid disease of the breast is extremely rare even in endemic areas , accounting for 0.27% of all cases . hydatid cyst of the breast is rarely kept as a differential diagnosis of breast lump owing to the rarity of the disease . we describe a rare case of isolated hydatid cyst of the breast in a breastfeeding woman in whom a preoperative diagnosis was made on cytological examination of the cyst fluid . a 30-year - old breastfeeding female patient presented to our surgical outpatient department with complaints of a painless lump along with decreased milk secretion from her left breast . on examination , the right breast was normal but there was a 4 cm 4 cm well - defined soft lump present in the retroareolar region of her left breast . on ultrasonic mammography , heterogeneous predominantly hypoechoic lesions in the central quadrant of the left breast along with few anechoic cysts at the peripheral aspect of the lesion were seen , suggestive of hydatid cyst or breast abscess [ figure 1 ] . fine needle aspiration cytology ( fnac ) of the lump was performed , which yielded creamish white nonmilky fluid . microscopic examination of hematoxylin and eosin - stained smears showed hydatid scolices , hooklets foamy macrophages and granular debris consistent with the diagnosis of hydatid cyst of the breast [ figure 2a and b ] . the patient was further investigated by ultrasonography ( usg ) of the abdomen and chest radiograph , which were normal . preoperative chemotherapy with albendazole was started at a dose of 10 mg / kg / day for 1 month , after which the size of the cyst decreased . aspiration of the cyst was performed after injecting 20% saline and the surrounding tissue was packed with mops soaked in 3% saline . complete surgical resection of the cystic mass from the surrounding breast tissue was performed but the cyst ruptured accidentally ; however , there was no anaphylactic reaction [ figure 3 ] . she was discharged in a satisfactory condition on the fifth postoperative day on albendazole therapy for another 28 days to prevent recurrence . owing to the rarity of the site and such rare clinical presentation of hydatid disease , this case is being reported here . hydatid disease of the breast is a very rare entity ( 0.27% ) even in endemic areas ; it can be the only primary site or part of disseminated hydatidosis . it generally affects women between 30 and 50 years of age , although ages from 20 to 74 years have also been reported . when secondary infection occurs , hydatid cyst of the breast can not be distinguished from breast abscess , clinically or by mammography . triple assessment , i.e. clinical assessment , usg and fnac , is used for the diagnosis of breast lumps . in the present case , clinical assessment was suggestive of galactocele but fnac and usg were diagnostic . serological tests such as enzyme - linked immunosorbent assay for echinococcus can also be used for the preoperative diagnosis of hydatid disease . preoperative diagnosis is difficult due to the disease mimicking a variety of conditions in old and young women . hence , it should also be kept in the differential diagnosis of breast lump and proper evaluation should be performed preoperatively to rule out the same .

we report a rare case of a 30-year - old breastfeeding woman who presented to our surgical outpatient department with complaints of a gradually enlarging lump in her left breast for the last 2 months . she also complained of difficulty in breastfeeding for 1 week . on examination , the right breast was normal but there was a 4 cm 4 cm retroareolar soft lump in her left breast . examination of the bilateral axilla was normal . a provisional diagnosis of galactocele was made and the patient was subjected to ultrasonic mammography and fine needle aspiration cytology ( fnac ) . ultrasonic mammography of the left breast showed a heterogeneous , hypoechoic lesion with thick septations and internal echoes suggestive of a complex cystic lesion , leading to a differential diagnosis of either hydatid cyst or breast abscess . on fnac , the aspirate was not milky and cytological examination was performed , which revealed hydatid scolices , hooklets , foamy macrophages and granular debris , leading to the final diagnosis of hydatid cyst of the breast . the patient was investigated further but there were no cysts at any other site . chemotherapy with albendazole was started and surgery was performed after 1 month . histopathology further confirmed the diagnosis . owing to the rarity of presentation , this case is being reported here .

phaeohyphomycosis is a group of mycotic infections caused by dematiaceous fungi ( pigmented ) that contain melanin in their cell walls . melanin is a known virulence factor in these fungi , and it acts by scavenging free radicals produced by phagocytic cells in the oxidative process , which would kill most organisms , and also prevents their action on the plasma membrane by binding to hydrolytic enzymes . the formation of fungal appressorium , which helps in fungal penetration into the host cell is also influenced by the melanin . all these actions explain the pathogenic potential of dematiaceous fungi even in an immunocompetent host . the fungus is present in host tissues as brownish hyphae , pseudohyphae , yeast cells , or combination of these forms . these organisms are widespread in the environment , being found in soil , wood , and decomposing plant debris . the major etiologic agents of phaeohyphomycosis are species of bipolaris , exophiala , curvularia , chaetomium , phoma , exserohilum , and wangiella . it is a rare infection mostly involving the skin and subcutis , and rarely involving the paranasal sinuses , eyes , central nervous system , lymph nodes , and bone . the subcutaneous infections typically occur on the exposed areas of the body , especially the extremities , fingers , wrists , knees , or ankles , resulting from traumatic inoculation . we report this case for its uncommon clinical presentation with multiple cystic swellings on both the feet and right hand in an immunocompetent individual . a 35-year - old male agriculturist presented with multiple asymptomatic swellings on the dorsum of right hand , both feet , and ankle region of 6 months duration . there was no history of trauma , fever or intake of medication , and any other underlying systemic disease . cutaneous examination revealed multiple skin - colored , nontender , firm - to - soft , cystic swellings approximately 3 cm in diameter present on the ankle region and feet . multiple swellings were coalesced to form a large fluctuant mass measuring 810 cm , present on the dorsum of right hand [ figure 1 ] . a differential diagnosis of multiple abscesses , calcinosis cutis , and deep fungal infection were considered . 8 10 cm confluent nodular swelling over the dorsum of the hand routine investigations were normal . while performing an excisional biopsy , there was purulent discharge from the biopsy site , and hence all the lesions were incised [ figure 2 ] . cyst material was sent for hpe gram stain was negative for bacteria and on culture with sabouraud 's dextrose agar , pigmented fungi were identified and lactophenol cotton blue showed exophiala jeanselmi species [ figure 3 ] . cyst material for hpe showed multiple granulomas and grocott 's methanamine silver ( gms ) stain showed broad brownish filaments [ figures 4 and 5 ] . multiple cystic swellings over the feet pigmented fungi on sabouraud 's dextrose agar ( a ) ( h and e , 10 ) granuloma in the dermis composed of epithelioid cells , lymphocytes , and fibroblasts . ( b ) ( h and e , 100 ) closer view ( a ) granulomas with pigmented fungal growth ( scanner view gms ) ( b ) pigmented fungal filaments ( high power view gms ) the patient was treated by surgical excision of the lesions without any antifungal therapy . the infections caused by dematiaceous ( melanized ) fungi are grouped into three classes that include phaeohyphomycosis , chromoblastomycois , and eumycotic mycetoma . phaeohyphomycosis represents a broad range of primary and opportunistic mycoses classified into four clinical forms , which includes superficial , cutaneous , subcutaneous , systemic , and disseminated forms ( brain , eye , central nervous system , peritoneum , bones ) . subcutaneous infections mostly occur on the limbs , fingers , wrist , knees , or ankles . the immune status of the host plays a major role in the clinical presentation which could range from papulonodules , verrucous , hyperkeratotic , or ulcerated plaques , cysts , abscesses , pyogranuloma , nonhealing ulcers , or sinuses . subcutaneous phaeohyphomycosis is a rare infection , however , the number of cases appears to be increasing in recent years as the number of immunocompromised patients also have increased . however , our case showed that immunocompromised state is not a necessary prerequisite for phaeohyphomycosis . infection usually occurs through traumatic inoculation of the skin and subcutaneous tissue with contaminated matter , with majority of lesions occurring on the feet and legs of outdoor workers , as observed in this patient . the most common etiological agents of subcutaneous phaeohyphomycosis are e. jeanselmi followed by e. dermatitidis . the genus exophiala is widely distributed in the environment and may cause infections in both immunocompromised ( human immunodeficiency virus patients , transplant recipients , debilitating chronic diseases , diabetes , and immunosuppressive therapy ) and rarely , in immunocompetent persons . e. jeanselmi usually causes mild cutaneous and subcutaneous infections which are often localized and solitary ( phaeohypomycotic cyst ) . even in severely immunosuppressed patients common clinical presentation is solitary subcutaneous cyst or abscess , firm to fluctuant , usually sparing the overlying skin . the common site of involvement is upper and lower limbs over the fingers , wrist , knees , or ankles , and less frequently on the face , neck , and scalp . all dematiaceous fungi are similar in morphology and can be differentiated only by culture . according to sharma et al . who reviewed the literature on phaeohyphomycosis , 23 patients with subcutaneous phaeohyphomycosis have been reported from india predominantly involving the extremities and very few cases with dissemination . in a study by murayama , 54 cases of phaeohyphomycosis due to e. jeanselmi were reviewed , however , they failed to identify any underlying disease in 31 cases . our patient also showed that there was no underlying disease predisposing to phaeohyphomycosis , as reported by murayama et al . our case report highlights the fact that any subcutaneous soft swellings must also be considered under differential diagnosis for fungal infections as they may be mistaken for lipoma , fibroma , epidermal cyst , or foreign body reaction . surgical excision of the lesion with or without antifungal agents ( itraconazole , ketaconazole , amphotericin b ) has been widely used in treatment of subcutaneous phaeohyphomycosis . the present case presented with multiple lesions , and all the lesions were excised . surgical excision of lesions showed no relapse even after 6 months without any antifungal therapy .

phaeohyphomycosis is a rare mycotic infection caused by various heterogenous groups of phaeoid ( dematiaceous ) fungi involving the skin and subcutaneous tissue . common clinical manifestations are subcutaneous abscesses or cystic swellings . here , we report a case of subcutaneous phaeohyphomycosis presenting as multiple asymptomatic cystic swellings over the hands and feet without any predisposing factors . histopathology showed granulomatous inflammation and special stain with grocott 's methanamine silver stain revealed broad pigmented hyphae . culture showed black - colored colonies identified as exophiala jeanselmi . the patient was treated with surgical excision of the lesions .

epidermoid cyst of the breast is uncommon . it is commonly encountered over the face and back either by a dermatologist or general surgeon in day to day practice . a 48 years old male presented with swelling over the right side of breast that underlies nipple areola complex ( nac ) since last 6 years . swelling was gradually increasing in size . no other significant history was available . on examination swelling was 1010 cm cm in size with visible punctum , well defined margin and indentation was positive ( figure 1 ) . no evidence of malignancy was found in hpe and diagnosis was consistent with epidermoid cyst . epidermoid cysts are the most commonly encountered small , spherical , slightly compressible , dome shaped cyst of the skin . common age of presentation is young adult male and common site are scalp , face , and back . the source of epidermis is usually the infundibulum of hairfollicle , as the lining of the two structures is identical . cyst wall is composed of true stratified squamous epithelium and keritinocyte shed from the wall results in collection of white cheesy material with unpleasant smell . important diagnostic feature , they are attached to the skin but are mobile over underlying structure . epidermoid cysts are usually asymptomatic and slowly growing , but they may become inflamed or secondarily infected , resulting in pain and tenderness . spontaneous rupture of the cyst wall leads to discharge of soft , yellow , foul smelling material in to the dermis . punctum is a portal of entry for various skin commensals as well as pathological organism . epidermoid cysts are benign cyst , rarely squamous cell carcinoma ( scc ) , basal cell carcinoma , mycosis fungoides , and melanoma have developed in epidermoid cysts . some syndrome like gardner syndrome and basal cell naevus syndrome are associated with epidermoid cyst occasionally . if cyst is infected , it should be incised and drained first followed by complete excision once inflammation subsides . it is important to excise the cyst completely as failure to do so results in recurrence . epidermoid cysts on the breast are uncommon , long standing cyst may become giant due to neglect on the part of patients .

epidermoid cyst is commonly known as sebaceous cyst . it is the most commonly encountered cyst of the skin . epidermoid cyst over the breast is uncommon . punctum is the hallmark for clinical diagnosis . local excision with primary closure is the treatment of choice . biopsy is mandatory in giant cyst to exclude malignancy .

sebaceous cysts often known as epidermal cysts result from folding of the squamous epithelium beneath the epidermis . etiology of vulval sebaceous cyst is unknown , however , they are known to occur due to obstruction of the pilo sebaceous ducts and gland or secondary to trauma like episiotomy , perineal tear or female genital mutilation ( fgm ) . they appear as firm , mobile , nodular swellings over the vulva or perineum filled with white or yellow semi - solid caseous material containing sebum and dead skin cells . mostly asymptomatic and need no therapy but symptomatic infected cysts require complete excision of the cyst wall and its contents as incomplete excision leads to scarring and recurrence . the minimal excision technique for epidermal cyst removal is less invasive than complete surgical excision . a 35-year - old female presented to sri aurobindo institute of medical sciences ( saims ) outpatient department with a painless swelling over the private parts since 10 years [ figure 1 ] . there was no history of trauma in recent past or present except for an episiotomy during delivery about 12 years back . at present there was no history of fever , pain , bleeding , or discharge from the swelling . there was a large 10 8 cm soft , nontender , well - defined swelling over the vulva on the right side . the skin over the swelling was free and there was no evidence of any discharge or ulceration over the swelling . patient was then planned for incision and drainage under anesthesia with proper antibiotic coverage . during surgery an incision was given over the most prominent part and around 100 - 150 cc of oily , grease - like material suggestive of sebaceous cyst was obtained [ figures 2 and 3 ] . digital compression was used to express out the cyst contents and later the cyst wall was extracted using an artery forceps but it came out in pieces . reexploration was needed after 3 - 4 days due to incomplete excision of cyst wall leading to infection at operative site . pre - operative image of a huge vulval swelling oily , grease - like material suggestive of sebaceous cyst bartholin cyst , skene duct cyst , lipoma , fibroma , leiomyoma , syringoma , acrochordon , hidradenoma papilliferum and epidermoid cyst can be considered in differentials of vulval swelling . sebaceous glands are known to occur throughout the skin except in the skin overlying palms and soles . the principle function of the sebaceous gland is to secrete sebum , hence any obstruction to the pilo - sebaceous gland and duct or any trauma to the skin during episiotomy or fgm results in formation of sebaceous cyst also known as epidermoid cyst , epithelial cyst , and keratin cyst . epidermoid cyst are mostly firm , fluctuant , mobile , painless slow growing dome shaped swellings with a black punctum over the upper back , face , neck , ears , and private parts of male as well as female . they contain soft , yellow , oily and greasy material called sebum that contains lipid and keratin . minimal excision technique is , however , more acceptable than complete excision as it is easy and less time consuming . the minimal excision technique involves expression of the oily sebum by vigorous digital compression through a small 2 - 3 mm incision given over the vulvar cyst . the continuous compression loosens the cyst wall from the surrounding tissues to facilitate removal of the sac . the need to close such a small incision with sutures is negligible and this has an edge over other techniques . routine incision and drainage leads to recurrence and infection due to incomplete excision of cyst wall like in our case . care should be taken to avoid spraying of oily contents during surgery by use of gauze or splatter - shield . complications of the surgery includes incomplete excision of the cyst wall , spraying of cyst contents , hematoma , and recurrence . malignant transformation is rare ; however , any abnormality like a hard ulcerative growth or repeated recurrence of the swelling needs confirmation by histo - pathological correlation . very few case reports / short communication are reported on vulval epidermoid cystshuge vulval epidermoid cysts like in our case have never been reported till dateminimal excision technique is the most acceptable surgical technique . very few case reports / short communication are reported on vulval epidermoid cysts huge vulval epidermoid cysts like in our case have never been reported till date minimal excision technique is the most acceptable surgical technique .

vulval swellings have always caused dilemmas in diagnosis and more so when they are huge in size . sebaceous cysts are known to occur as a result of blocked pilo - sebaceous gland and duct or as a result of any injury to the skin . face , neck , chest , back , scalp , and ears are known sites , however , they also occur over private parts . they are mostly asymptomatic but cause intense pain and discomfort if infected . symptomatic cysts warrant removal .

when such tumors arise from extraadrenal chromaffin cells , they are called as paraganglioma ( pgl ) . adrenal medulla is the major source of catecholamine which manifests as hypertension in patients with pcc . in children , hypertension is detected in about 90% of cases and tends to be more severe and sustained than adults . the purpose of reporting this case of pcc is because of its two unusual findings , namely very young age at presentation and childhood case without hypertension ; each accounts for 10% of cases of pcc thus being categorized as rule of 10s or a 14-month - old female child presented to us for swelling of abdomen for last 1 month . abdominal examination revealed a mass in the right hypochondrium and lumbar area which was nontender , a firm with an irregular surface . ultrasonography suggested heterogeneous mixed echogenic solid lesions with the minimal cystic part within size 9 cm 7 cm seen in the right hypochondrium and lumbar region . computed tomography ( ct ) scan revealed 7.3 cm 8.0 cm 9.1 cm heterogeneous enhancing lesion in the upper pole of right kidney with internal calcific foci [ figure 1 ] . abdominal computed tomography scan images showing 7.3 cm 8.0 cm 9.1 cm heterogeneously enhancing right - sided adrenal tumor intraoperatively , the tumor was found adherent to liver , inferior vena cava , and portal vein . histopathological examination showed encapsulated tumor mass measuring 9 cm 8 cm 7 cm , cut section grayish yellow , soft with areas of hemorrhage and necrosis [ figure 2 ] . the mass was totally separate from the upper pole of kidney and grossly identified as adrenal mass , the adrenal cortex seen compressed superiorly . individual tumor cells show marked variation in size , shape along with spindle cells and finely granular basophilic to amphophilic cytoplasm with intracytoplasmic hyaline globules . nuclei were round to be oval with prominent nucleoli containing intracytoplasmic inclusions with salt and pepper chromatin at places [ figure 3 ] . some of the tumor cells were bizarre and pleomorphic , sparse mitosis with areas of hemorrhage , necrosis , and cystic changes . histopathological diagnosis of pcc was documented with advice for urinary vanillylmandelic acid ( vma ) estimation . the child is on follow - up with imaging studies , asymptomatic , and gaining weight . a largxe adrenal medullary mass . ( b ) cut section : grayish yellow hemorrhagic mass compressing adrenal cortex superiorly and kidney lie on the either sides photomicrograph ( a ) zellballen nest pattern of tumor cells with hemorrhage and cystic change ( h and e , 100 ) ( b ) bizarrely pleomorphic tumor cells with prominent nucleoli and intranuclear inclusions and amphophilic granular cytoplasm ( h and e , 400 ) pheo is the term used for catecholamine - secreting tumors that occur in the adrenal medulla , the most common location , whereas pgl is extraadrenal tumors that arise from both sympathetic and parasympathetic paraganglia . in a retrospective study of 36 years , ein et al . published 14 children ( 10 boys and 4 girls ) and found youngest child was 8 years of age . another series published from turkey reported 16 children ( 12 boys and 4 girls ) and the mean age at diagnosis was 10.7 years . a japanese series on pcc published in 2015 revealed the mean age at diagnosis in children as 13.2 years . our patient was only 14 months of age at diagnosis , and she was symptomatic for the last 1 month . hypertension is present and sustained in about 90% of affected children at the time of diagnosis . hence , pcc without hypertension in a child is very uncommon as in our case . the classic triad of symptoms a headache , palpitation , and diaphoresis recorded in many cases was also not found in our case . the diagnosis of adrenal gland phaeochromocytoma is documented here with following features : ( 1 ) characterized by zellballen nests of tumor cells separated by delicate fibrovascular stroma , ( 2 ) varying sized and shaped tumor cells with granular cytoplasm and with hyaline globules , ( 3 ) nuclear atypia with inclusions , and ( 4 ) though marked pleomorphism , necrosis were evident . the evidence of malignancy is not considered in this endocrine tumor as lymph nodes did not show any metastatic deposits . indication for urinary vma estimation was to exclude the multifocality of the tumor in extraadrenal chromaffin sites which is commonly seen in childhood pccs . clinically , another childhood tumor of adrenal medulla named as anaplastic neuroblastoma may come as a differential diagnosis of this tumor because both the tumors show bizarre appearance of the tumor cells and zellballen pattern . germ - line mutation of either of six known genes : ret ( men 2a , 2b ) , nf1 ( von recklinghausen neurofibromatosis type 1 ) , von hippel lindau syndrome , sdhd , sdhc , and sdhb ( mitochondrial succinate dehydrogenase gene mutations associated with pgl ) are associated with the familial occurrence of the tumor and those present at young age group as in this case . these familial cases are recently recognized as 25% occurrence instead of traditional 10% rule of the past . this type of pcc are more often bilateral , but our case has presented unilaterally . family history is not suggestive in this case , hence follow - up is continuing . pcc is very rarely diagnosed tumor during childhood and being detected in a 14-month - old baby is extremely uncommon . early diagnosis and complete surgical excision are essential for successful management of these rare children . pcc is known for its 10% bilaterality , 10% extra - adrenal , 10% malignant , 10% without hypertension , and 10% occurrence in children . pcc unassociated with hypertension and childhood presentation both the features as we observed in our case contribute to the rule of 10s .

pheochromocytoma ( pcc ) is a neuroendocrine tumor originating from chromaffin tissue in adrenal medulla . its diagnosis and treatment are well defined in adults , but experience in children is limited . children constitute only 10% of reported cases , the average age at presentation being 11 years . the most common presentation is sustained hypertension , which is absent in only 10% of children . we managed a 14-month - old female child with pcc , but she was not hypertensive . we report two unusual features , in this case , an extremely young age at presentation and a childhood case of nonhypertensive pcc contributing for rule of 10s .

a 46-year - old man was admitted to the internal medicine department for evaluation of an incidental finding of leukocytosis and hypereosinophilia . he complained of a pruritic skin rash on his lower extremities for the past eight months . no changes in bowel habits , arthralgias / myalgias or any other complaint were present . he denied any travel abroad in the past year or any exposures that may determine etiology of the eosinophilia . leukocytosis of 23,000/l with eosinophil count of 9430/l was observed , but platelet and hemoglobin levels were normal . no blastocysts were found in the peripheral blood . repeated stool samples for parasites and ova were negative . the patient underwent a computed tomography ( ct ) scan of the chest , abdomen and pelvis , with no pathology found , except for a left inguinal hernia . a skin biopsy from his right leg revealed hyperkeratotic skin with mild angiocentric inflammatory changes , and no signs of vasculitis or malignancy . his bone marrow biopsy revealed normocellular bone marrow with increased number of eosinophils , which may be compatible with reactive changes . bcr - abl and janus kinase-2 mutations were negative , as were tests for fip1l1/pdg - fra and fip1l1/pdg - frb mutations , ruling out myeloproliferative variant he s ( m - hes ) . the patient was offered an empirical trial of albendazole ( 400 mg twice a day for three days ) . a healthy , 23-year - old nonsmoker was admitted to the internal medicine department with abdominal pain and a three - week history of intermittent watery diarrhea . he did not experience vomiting , fever or night sweats , but complained of dyspareunia and approximately 2 kg of weight loss . stool samples collected for three consecutive mornings , which were tested for parasites using wet mount examinations and concentration procedures , found no signs of helminthic infection . ct scans of the chest and abdomen exhibited increased wall thickness of the large intestine ; however , gastroscopy and colonoscopy revealed normal mucosa appearance with no eosinophilic infiltration or parasites on biopsies . bone marrow biopsy found an increased number of myeloid eosinophils with no signs of myeloproliferative disorder . empirical therapy with albendazole ( 400 mg twice per day for three days ) was initiated , followed by a decrease in eosinophil count in consecutive blood tests . a 32-year - old man , with a history of epilepsy for several years and with no history of travel or known exposure to helminths , was hospitalized in the internal medicine department after a short episode of unconsciousness without convulsions . two days before his admission , he experienced right abdominal pain , without any fever or vomiting . bcr - abl and janus kinase-2 mutations were negative , as well as fip1l1/pdg - fra and fip1l1/pdg - frb mutations for detection of m - hes . gastroscopy with mucosal biopsy displayed signs of gastritis and helicobacter pylori infection , but no signs of eosinophilic infiltration . empirical therapy with albendazole ( 400 mg twice per day for three days ) was initiated , followed by a rapid decline in eosinophil count . normal eosinophil count is usually approximately 400/l ( range 100/l to 600/l ) . when elevated , eosinophil counts are arbitrarily classified as mildly elevated ( blood eosinophil count between 600/l and 1500/l ) , moderately elevated ( 1500/l to 5000/l ) and severely elevated ( > 5000/l ) . the finding of hypereosinophilia in any patient warrants clinical attention , especially when no apparent cause is found and counts are moderately or severely elevated . secondary causes are the most commonly encountered , including helminthic infection , allergic disorders , medications , toxins , autoimmune diseases and endocrine disorders such as addison disease . rarely , neoplastic diseases , including varied adenocarcinomas , some forms of hodgkin disease , t cell lymphoma and mastocytosis may be associated with paraneoplastic eosinophilia . clues from the patient s history and physical examination can often guide toward a diagnosis . primary hypereosinophilia is a clonal expansion of eosinophils , occurring in the context of hematological malignancy , such as acute leukemias and chronic myeloid disorders . hes are a group of clinical disorders characterized by hypeosinophilia , accompanied by apparent organ damage / dysfunction , unexplained by secondary causes . although once considered idiopathic , currently with modern diagnostic and management facilities , and recent advances in pathogenic mechanisms , criteria for hes are being revised ( 3,4 ) . a practical approach to managing a patient with hypereosinophilia has been discussed in the literature ( 1,2 ) . diagnosis of a patient with hypereosinophilia usually includes a thorough history ( including drug history and travel history ) and physical examination , complete blood counts and blood smears , routine blood chemistry and several other laboratory examinations based on clinical suspicion . these include bone marrow aspirate and biopsy , karyotype and cytogenetics , parasite serology and stool samples for parasites , immunoglobulin e level , antineutrophil cytoplasmic antibody , troponin and vitamin b12 levels , electrocardiogram and echocardiography , pulmonary function tests , and ct scans of the chest and abdomen . he s should be sought when previous tests have failed to detect the underlying cause . tests for fip1l1/pdg - fra and fip1l1/pdg - frb mutations are currently recommended for the diagnosis of newly recognized m - hes ( 3 ) . when no abnormality is found , wait - and - see is the common recommended approach . in the present report , we described three cases of hypereosinophilia in which a thorough history ( including travel and drug history ) , physical examination and laboratory , as well as imaging investigations were unable to detect any abnormality . although our patient in case 2 had recently traveled to south america , repeated stool cultures tested for parasites were completely normal . our investigation included the most recently recommended tests for detection of m - hes . however , helminthic serologies and polymerase chain reaction testing for helminth parasites were not performed at our hospital . all three cases were treated empirically with 400 mg of albendazole twice per day for three days , with complete normalization of eosinophil count thereafter . because albendazole has been reported to be effective in cases of idiopathic hypereosinophilia in tropical countries and countries in the far east ( where helminthic infection is more common ) ( 5 ) , our report demonstrated its efficacy in cases in the western world . therefore , we strongly suggest taking into consideration an empirical treatment using albendazole in cases in which thorough evaluation of hypereosinophilia has failed to detect the underlying cause .

hypereosinophilia can lead to life - threatening organ damage when associated with eosinophilic infiltration of tissues . although it is associated with a broad variety of diseases , sometimes no other abnormalities are detected . the authors present a novel approach to the treatment of hypereosinophilia , especially when an underlying cause fails to be detected .

xenopus na , k pumps were made insensitive to ouabain and extracellular mts reagents by the mutation c113y ( ref . xenopus oocytes were injected with a 50 nl mixture of 5 ng of xenopus 3 and 15 ng of mutated xenopus 1 crnas , and incubated at 18 c for 1 - 3 days . currents were recorded in outside - out excised patches at 22 - 24 c as described5,18 . internal ( pipette ) solution contained ( in mm ) : 125 naoh , 100 sulphamic acid , 20 hcl , 10 hepes , 1 egta , 1 mgcl2 and 5 mgatp ( ph 7.4 ) . external solution contained ( mm ) : 125 naoh or tma - oh , 125 sulphamic acid , 10 hepes , 5 bacl2 , 0.5 cacl2 and 1 mgcl2 ( ph 7.6 ) , plus 100 m ouabain . palytoxin ( wako pure chemical industries ltd . , osaka , japan ) was added ( from 100 m aqueous stock solution ) at 50 nm , with 0.001% bovine serum albumin and 1 mm na - borate . mts reagents ( toronto research biochemicals , north york , on , canada ) were added from ice - cold 100 mm aqueous stock solutions immediately before use , and were refreshed at 1.5-min intervals to maintain reactivity during prolonged ( 2 min ) applications4 . alteration of palytoxin - induced current by mts reagents was calculated as : % inhibition of ipalytoxin = 100 * ( 1 - iafter / ibefore ) , where iafter represents steady palytoxin - induced current at -50 mv after mts reagent application , and ibefore that just before . the xenopus na , k - atpase 1 subunit homology model was built from the ca - atpase e2bef3 structure ( ref . 12 ; pdb code 3b9b ) using swiss - model ( http://swissmodel.expasy.org ) as described5 . xenopus na , k pumps were made insensitive to ouabain and extracellular mts reagents by the mutation c113y ( ref . xenopus oocytes were injected with a 50 nl mixture of 5 ng of xenopus 3 and 15 ng of mutated xenopus 1 crnas , and incubated at 18 c for 1 - 3 days . currents were recorded in outside - out excised patches at 22 - 24 c as described5,18 . internal ( pipette ) solution contained ( in mm ) : 125 naoh , 100 sulphamic acid , 20 hcl , 10 hepes , 1 egta , 1 mgcl2 and 5 mgatp ( ph 7.4 ) . external solution contained ( mm ) : 125 naoh or tma - oh , 125 sulphamic acid , 10 hepes , 5 bacl2 , 0.5 cacl2 and 1 mgcl2 ( ph 7.6 ) , plus 100 m ouabain . palytoxin ( wako pure chemical industries ltd . , osaka , japan ) was added ( from 100 m aqueous stock solution ) at 50 nm , with 0.001% bovine serum albumin and 1 mm na - borate . mts reagents ( toronto research biochemicals , north york , on , canada ) were added from ice - cold 100 mm aqueous stock solutions immediately before use , and were refreshed at 1.5-min intervals to maintain reactivity during prolonged ( 2 min ) applications4 . alteration of palytoxin - induced current by mts reagents was calculated as : % inhibition of ipalytoxin = 100 * ( 1 - iafter / ibefore ) , where iafter represents steady palytoxin - induced current at -50 mv after mts reagent application , and ibefore that just before . the xenopus na , k - atpase 1 subunit homology model was built from the ca - atpase e2bef3 structure ( ref . 12 ; pdb code 3b9b ) using swiss - model ( http://swissmodel.expasy.org ) as described5 .

p - type atpases pump ions across membranes , generating steep electrochemical gradients that are essential for the function of all cells . access to the ion - binding sites within the pumps alternates between the two sides of the membrane1 to avoid the dissipation of the gradients that would occur during simultaneous access . in na+,k+-atpase pumps treated with the marine agent palytoxin , this strict alternation is disrupted and binding sites are sometimes simultaneously accessible from both membrane sides , transforming the pumps into ion channels ( e.g. , refs 2,3 ) . current recordings in these channels can monitor accessibility of introduced cysteine residues to water - soluble sulphydryl - specific reagents4 . we found previously5 that na+,k+ pump - channels open to the extracellular surface through a deep and wide vestibule that emanates from a narrower pathway between transmembrane helices tm4 and tm6 . here we report that cysteine scans from tm1 through tm6 reveal a single unbroken cation pathway that traverses palytoxin - bound na+,k+ pump - channels from one side of the membrane to the other . this pathway comprises residues from tm1 , tm2 , tm4 , and tm6 , passes through ion - binding site ii , and is likely conserved in structurally and evolutionarily related p - type pumps , such as serca- and h+,k+-atpases .

an 8-year - old female child presented in the pediatrics outpatient department with a history of fever since 7 days , headache , vomiting , and irritability . on examination , she was irritable . her vital parameters revealed a pulse rate of 96 beats per min , respiratory rate 28/min , blood pressure ( bp ) 82/53 mmhg , and spo2 98% on room air . cerebrospinal fluid examination including cartridge - based nucleic acid amplification test ( cbnaat ) confirmed diagnosis of tubercular meningitis after 12 days . hence , antibiotics were continued till then , and following which she was started on category 1 antitubercular therapy ( att ) as per revised national tuberculosis program ( rifampicin , isoniazid , pyrazinamide , and ethambutol along with oral prednisolone ) . as child received 12 days of antibiotics , it was planned to give vitamin k in view of presumed antibiotic - induced hypoprothrombinemia . she was apparently well before giving vitamin k. a slow intravenous vitamin k ( 2 mg diluted in 2 ml normal saline ) injection was given , immediately following which , she screamed , developed respiratory arrest , central cyanosis and became unresponsive . cardiopulmonary resuscitation ( cpr ) was started as per the protocol and she was revived within 2 min . pulse volume became normal , saturation with 40% o2 was 100% , and bp was 80/56 mmhg . she also received 20 ml / kg of normal saline bolus and vitals were monitored for 24 h postevent . complete blood count showed hemoglobin 10.6 mg / dl , total leukocyte count 10,500/mm , neutrophils 70% , lymphocytes 26% , eosinophils 2% , and platelet count 2.55 lacs / mm . renal function test showed urea 30 mg / dl , creatinine 0.3 mg / dl , sodium 135 mmol / l , potassium 4.2 mmol / l , calcium 8.8 mg / dl , and magnesium 3.8 mmol / l . this reaction was reported to adverse drug reaction ( adr ) monitoring center of our hospital ( having worldwide vigiflow number 2015 - 29603 ) . antibiotic - induced hypoprothrombinemia is a known phenomenon , especially in patients receiving prolong n - methylthiotetrazole ( nmtt ) group of antibiotics such as cefoperazone , cefmetazole , cefamandole , moxalactam , cefotetan , and cefmenoxime . two indian studies in children had mentioned incidence of 15% and 33% in two different settings and normalization of coagulation profile with vitamin k administration . the determinants for such phenomena are mostly related to nutritional status of the child and nmtt group of antibiotics . in our case , we have to give antibiotics initially because cbnaat report was available on the 12 day of treatment . serious reaction are rare , although the exact incidence of serious side effects such as anaphylactic , anaphylactoid reactions are not known and especially in pediatric age group they are less reported . in their 5-year retrospective study , riegert - johnson and volcheck reported the incidence of adverse reaction due to vitamin k to be 3 per 10,000 doses ( 0.0411 per 10,000 doses ) . the common reported symptoms range from facial flushing , cyanosis , dyspnea , chest pain , loss of consciousness , hypotension to cardiorespiratory arrest . some of the reactions got reversal with supportive treatments such as saline bolus , intravenous ( iv ) adrenaline , steroid , and oxygen whereas few were fatal . in the index case causality assessment was done by institutional adr monitoring center and was attributed to anaphylactoid reaction to vitamin k. this was because as there was no previous exposure of vitamin k to patient and the reaction happened immediately after injection , as well as reaction could not be explained by disease or other drugs in the stable patient . assessment as per naranjo 's scale had score of six which comes under category of probable adr . moreover , it required only supportive measure oxygen and cpr for treatment and there was no further recurrence . the preventability analysis for above case was done by adr center as per the schumock and thornton criteria and was determined to be not preventable . it was thought that dispersant used , i.e. polyethoxylated castor oil , might be responsible for the same because there were reports of similar anaphylactic reactions from other anesthetic and antineoplastic agents which used the same solvents for preparations and later withdrawn . therefore , a new formulation of vitamin k1 in mixed micelles of lecithin and glycocholic acid ( mm form ) was introduced to decrease these adverse events . havel et al . in their study compared both older and newer preparation and found no advantage of newer preparations as they reported one anaphylactoid reaction with mm form . however , pereira and williams in their postmarketing surveillance of vitamin k reported mm form to be safer than castor oil preparation . these reactions have also been linked to the route and the rate of administration , more common with iv route although reported with intramuscular ( i m ) and oral route as well . rapid iv bolus injections are more commonly associated with such reactions but the slow infusions are also not completely safe . rich and drage have reported incidents of anaphylactoid reaction following slow bolus of vitamin k and recommended bolus rate slower than 1 mg / min whereas songy and layon reported hypotension and collapse in two adult patients in midway of slow vitamin k infusion . reported anaphylactic shock in a newborn secondary to i m injection of vitamin k. these support that no single route is completely safe . vitamin k is an important factor for maintaining coagulation , commonly indicated for bleeding secondary to coumadin agents , underlying liver disorders , sepsis - induced coagulopathy , and also used as prophylaxis for prevention of hemorrhagic disease of newborn . adverse reactions are common but fatal ones are rare . no route and preparations are completely safe though using mixed micelles formulation through i m route or slow iv infusion is comparatively safer if administered under monitoring .

vitamin k is commonly indicated in pediatric patients with coagulopathies . its commercial preparations are available in the market since long . minor complications are very well - known , but life - threatening events are rarely reported in children . we present a case of 8-year - old child who developed life - threatening event following intravenous vitamin k injection . she was survived after cardiopulmonary resuscitation . the reaction was most probably due to anaphylactoid reaction to vitamin k.

we developed a combined diabetes and depression management program by adding a depression care manager to an existing diabetes management team . project dulce is a culturally competent diabetes case management program that includes peer - led self - management training . we felt that impact could be its sister in depression care management because of its focus on screening , measurement , and personalized , evidence - based guideline care . consistent with the philosophy of project dulce , the depression care manager was bilingual and bicultural and aware of the cultural issues involved in providing treatment for depression to a primarily spanish - speaking latino population . the impact model was made more flexible with regard to responding to cultural norms and beliefs , low literacy , socioeconomic barriers , and social stigma . those identified with clinically significant depressive symptoms ( defined as a phq-9 score 10 ) had a visit scheduled with the depression care manager , who conducted a psychosocial history , reviewed educational materials , and discussed patient preferences for depression treatment with antidepressant medications and/or structured psychotherapy using problem - solving treatment in primary care . all patients received education about depression and behavioral activation . as appropriate , the depression care manager consulted with the diabetes case manager and the patient 's primary care physician . new patients and patients needing treatment plan adjustments were discussed in weekly caseload review meetings with a consulting psychiatrist . we measured depressive symptoms using the phq-9 at baseline and at a 6-month follow - up . we calculated cohen 's d statistics from self - reported diabetes self - care activities related to nutrition , exercise , and glucose monitoring / medication adherence ( a summary of the diabetes self - care activities form , in spanish and in english , is available in an online appendix at http://dx.doi.org/10.2337/dc08-0307 ) . we screened 499 participants in project dulce for depression using the phq-9 : 464 ( 93% ) were eligible to participate in project dulce plus impact ; 154 ( 33% ) scored as having clinically significant depression , and 99 ( 64% ) completed the study . among those completing the study , mean sd age was 53 9 years ; 84% of subjects were female and 74% were latino , and 71% noted spanish as their primary language . only one demographic characteristic was related to study participation : among those eligible to participate in the study , women were more likely to be identified as having symptoms of major depression than men ( odds ratio 1.9 ; p < 0.001 ) . clients averaged 6.7 visits with the depression care manager during the study period ( one initial visit and 5.7 follow - up visits ) with 31.5 26.6 days as the average time between visits . sixty - nine percent received problem - solving treatment , and 35% received medication management . phq-9 scores declined by an average of 7.5 points throughout the study period , from 14.8 to 7.3 ( sd 6.5 , sem 0.66 ; p < 0.001 ; fig . diabetes self - care activities related to nutrition increased ( cohen 's d = 0.26 ; p = 0.0187 ) . the depression case manager spent 20 h per week in direct patient care ( face - to - face meetings ) and another 10 h per week on care coordination , scheduling , and administration . each participant required , on average , a 30-min consultation with the diabetes nurse case manager , 10 min with a consulting physician , and 20 min with the consulting psychiatrist . using current wages , benefits , and rates for administration / overhead , we estimate that depression care management cost $ 512 per participant completing the study . the combined diabetes and depression care management program tested in this pilot study was both feasible and highly effective in reducing depressive symptoms in a low - income , predominantly spanish - speaking latino population . because of the high rate of comorbid diabetes and depression in latinos , a combined program may be more attractive to primary care clinics than a freestanding depression care program like impact . our pilot study is limited by the lack of a randomly assigned control group and a relatively small sample size . this limits its statistical power to examine smaller but clinically important effects on health behaviors but provides strong support for further research on this combined intervention approach . although the impact model has previously been shown to be effective in older populations with diabetes ( 8) , this is the first study to examine a fully integrated program . it is also the first study of impact depression care management in a population of largely spanish - speaking latinos . the cost of providing depression care management in this setting is modest and consistent with earlier cost estimates for the impact program ( 7 ) . recent data from impact ( 8) suggest that better depression care may be associated with lower total health care costs than associated with care as usual .

objective to assess the feasibility and cost of integrating diabetes and depression care management in three community clinics serving a low - income and predominately spanish - speaking latino population.research design and methods we screened diabetes patients for depression , and for those with depressive symptoms , we provided depression care management . we assessed changes in depressive symptoms using the patient health questionnaire-9 ( phq-9 ) , diabetes self - care activities ( nutrition , exercise , and medication adherence ) , and costs.resultsthirty-three percent of patients with diabetes had symptoms of major depression . among 99 patients completing the study , phq-9 scores declined by an average of 7.5 points from 14.8 to 7.3 ( p < 0.001 ) . clients averaged 6.7 visits with the care manager during the study period . costs of depression care management were estimated to be $ 512 per participant.conclusionsadding a depression care manager to an existing diabetes management team was effective at reducing depressive symptoms at a reasonable cost .

traumatic spinal cord transection is uncommon . in a study of 62 patients with spinal cord injuring who underwent magnetic resonance imaging ( mri ) , 7 had cord transections ( 1 ) . in contrast to children in who closed spinal cord trauma may occur in the absence of skeletal injury , most cord injuries in adults are associated with major musculoskeletal injuries , such as vertebral fractures and/or dislocations ( 2 - 6 ) . in the subaxial cervical spine , hyperextension injuries without facet dislocations are well known as central cord syndromes , but cervical cord transection has not been described in the literature ( 7 - 10 ) . we report a patient with a cervical cord transection in the absence of a vertebral dislocation secondary to air bag deployment without the use of a seat belt and discuss the mechanism of this type of injury . a 33-yr - old male was admitted through the emergency room with neck pain , and loss of sensation and inability to move all four extremities . he had crashed his car into the post of a traffic signal , was not wearing a seat belt , and the front air bag deployed . on neurologic examination , the patient had flaccid paralysis of all four extremities , a sensory level at c4 , no sphincter control or rectal tone , an absent anal wink , no sacral sparing , and a positive bulbocavernosus reflex . he was fully alert with mild hypotension ( systolic arterial pressure < 100 mm hg ) . plain radiographic evaluation of his cervical spine showed c5 , c6 wedge compression fractures and minimal c5 retrolisthesis on c6 ( fig . a computed tomographic ( ct ) scan also demonstrated c5 , c6 vertebral body vertical fractures with laminar fractures , and a c3 spinous process fracture ( fig . the fractured c5 lamina was depressed into the spinal canal through the lamina - facet junctions . a mri revealed a linear area of abnormal signal intensity running horizontally through the spinal cord at the c5 - 6 disc space level , which was thought to represent a spinal cord transaction ( fig . the patient had no brain , thoracic , or abdominal abnormalities on plain films and ct scans . the patient underwent surgery via a posterior approach 5 days after his injury . a c5 laminectomy with a posterior fusion and lateral mass screw fixation at the c4-c7 levels 4 ) . during this procedure , disruption of the c4 - 5 supraspinous and interspinous ligaments were noted , but the facet joint capsules were intact at the c4-c7 levels in the operative field . he was transferred to the rehabilitation department 1 month post - operatively and no neurologic improvement had been observed 4 months after the injury . there have been no reports of traumatic spinal cord transections in adults other than fracture - dislocation injuries in the subaxial spine . although berlot et al . ( 11 ) described a delayed post - traumatic cervical cord transection in a spinal cord injury without radiologic abnormalities ( sciwora ) , the initial mri revealed only focal swelling of the spinal cord and several patch areas of cord contusions ; indeed , an initial incomplete lesion may have progressed to transection in the absence of fixation . our patient 's injury appeared to have occurred when his torso overrode the air bag , which resulted in acute hyperextension of the cervical spine . this potential mechanism has been shown in crash simulations using human cadavers and dummies positioned opposite airbags when unbelted ( 12 ) . but in such simulations , the velocities were between 32 and 48 km / hr . the velocity in our patient was more than 60 km / hr , so a more extreme hyperextension injury may have occurred . during the episode of hyperextension , rupture of the anterior longitudinal ligament and the disc caused marked backward displacement of the c5 vertebral body against the cord ( 13 ) . also , our patient had a c5 laminar fracture , which may have exacerbated the hyperextension and posterior displacement . although there was no facet disruption , this circumstance could result in a c5/6 spinal cord transection . because the spine returned to its normal position by the elastic recoil of the vertebral muscles when the head is in the neutral position or in flexion ( 14 ) , subtle c5 retrolisthesis on c6 . the above mechanism of injury could explain the cord transection and injuries involving the posterior vertebral column . however , c5 , c6 vertebral body fractures are unresolved . according to allen 's classification of subaxial cervical fractures and dislocations ( 15 ) , our patent 's injury was a distractive extension stage 2 lesion . in this type of injury , however , there was no co - existing vertical fracture centrum . therefore , we propose the hypothesis that during the initial short moment of the collision , the airbag was just in contact with the torso , but not with the face of the patient . as a result , compressive flexion force interacted at the cervical spine , resulting in c5 , c6 vertebral body fractures . 5 . the course of treatment in this patient is of less interest than the proposed mechanism of injury . distractive extension stage 2 lesions must be surgically stabilized , usually with an anterior approach . however , in the presence of cord involvement by laminar fragments with preservation of lordotic sagittal alignment , a posterior approach is also useful ( 16 ) . in this case report four months after the operation , no cervical instability was observed while the patient ambulated in a wheelchair . although there is a constant search for ways and means to enhance recovery of spinal cord injury , this patient 's prognosis is very poor . according to data of the model spinal cord injury systems ( 17 ) , 94.4% of patients with neurological complete spinal cord injuries remained so at the 5-yr post - injury evaluation . nevertheless , kirshblum et al . reported approximately 20% showed some improvement in motor power and neurologic level of injury from year 1 to year 5 ( 18 ) . therefore medical rehabilitation service should be provided sufficiently and continuously for the sake of this patient 's recovery . although this report describes just a single case of spinal cord transection by motor vehicle accident , it emphasizes the importance of proper use of seat belts especially in the circumstance of airbag deployment . the authors ' proposed hyperflexion - hyperextension mechanism can result in significant neurologic deficits , but subtle radiographic abnormalities might be seen on plain films . so clinicians in emergency centers should have the possibility of cord transection in the acutely injured patient in mind because it is important for both prognostic and therapeutic reasons .

a patient is presented with a cervical spinal cord transection which occurred after a motor vehicle accident in which the air bag deployed and the seat belt was not in use . the patient had complete quadriplegia below the c5 level and his imaging study showed cervical cord transection at the level of the c5/6 disc space with c5 , c6 vertebral bodies and laminar fractures . he underwent a c5 laminectomy and a c4 - 7 posterior fusion with lateral mass screw fixation . previous reports have described central cord syndromes occurring in hyperextension injuries , but in adults , acute spinal cord transections have only developed after fracture - dislocations of the spine . a case involving a post - traumatic spinal cord transection without any evidence of radiologic facet dislocations is reported . also , we propose a combined hyperflexion - hyperextension mechanism to explain this type of injury .

percutaneous endoscopic gastrostomy ( peg ) tube placement has been the minimally invasive procedure of choice for enteral access , or decompression , since the early 1980s . herniation through gastrostomy site is considered an extremely rare complication with only four cases reported . we present two unusual cases of gastrostomy site herniation , the surgical management thereof , and a corresponding review of the literature . the first patient is a 65 year old caucasian male who presented to the surgery clinic after a prolonged hospitalization for congestive heart failure exacerbation resulting in the need for gastrostomy tube placement . the patient had the peg tube in place for approximately a month and then it was removed by traction technique after no longer in use . the patient noticed an enlarging defect about 3 weeks after removal . at time of presentation he complained of epigastric pain and a bulge at his previous gastrostomy tube incision site . the hernia was easily reduced with gentle traction , and a gastro - cutaneous attachment resected ( fig . a polyester composite mesh was then used to repair the gastrostomy site ventral wall defect ( fig . 2 ) . the second patient is a 66 year old obese caucasian male who was hospitalized in the intensive care unit for lithium overdose . he required prolonged enteral feeding , and for that purpose he received a percutaneous endoscopic gastrostomy . subsequently he recovered well and had the tube removed by simple traction in the office . five months later he complained of continued pain in the midepigastric region around his gastrostomy site scar . on physical exam he was taken to the operating room and his hernia was repaired by open surgery with a composite mesh . both patients had non - complicated reducible hernias at presentation and no extra imaging or specific diagnostic modalities were required . authordatediagnosisremoval methodinterventionoutcomechuang 2003leakage from around peg tube , bulge with coughingtractionremoval of peg tube via traction method , plan for surgical interventionpneumonia , respiratory failure , and death before surgeryboldo - roda 2005leakage from around peg tube , bulge with physical activitytractionn / aunkkaplan 2006leakage from around peg tube , bulge with physical activitytractionreferral for surgical interventionunkozutemiz 2007bulgingtractionn / aunk peg site herniation has been described previously but without description of operative intervention received . with the increasing numbers of peg tubes being placed every year discussion of preventing this complication was presented by boldo - roda et al . and included avoidance of placement of peg tube through linea alba , as this is an area of potential weakness ; and possibly using cut and push technique rather than traction . it is possible that vigorous traction during removal may create a more permanent cavity than expected . however cut and push technique carries its own innate risks and clinicians should keep this in mind when deciding on the removal technique of choice . while gastrostomy site hernia is an exceedingly rare complication with only four other cases reported in the literature , it is likely , given the number of gastrostomy tubes placed per year , that it is simply underreported . clinicians should stay vigilant when performing physical exams or additional workup in patients with ongoing leakage , bulge or pain at the gastrostomy site ; as this is potentially a surgically correctable entity , and can be safely managed via laparoscopic or open techniques . written informed consent was obtained from the patients for publication of these two case reports and accompanying images . a copy of the written consents is available for review by the editor - in - chief of this journal on request .

highlightstow rare cases of herniation through gastrostomy site.percutaneous endoscopic gastrostomy is the minimally invasive procedure of choice for enteral access or decompression.while it s exceedingly rare , herniation through gastrostomy site is a possible complication that we should be aware of.in our two cases , epigastric pain around the site of the previous gastrostomy or the gastrostomy scar was the main complaint at presentation .

most cystic fibrosis ( cf ) patients have chronic pulmonary infections and episodic exacerbations requiring intravenous antibiotic therapy . early in the disease , there are only a few case reports showing isolation of nocardia from cf patients , but it did not appear to be pathogenic in this population . we report a case of a pediatric patient with cf harboring nocardia transvalensis that did appear to be pathogenic in this patient . a 19-year - old caucasian male patient with cf presented with an acute exacerbation of his respiratory symptoms . his past medical history was significant for pansinusitis ( requiring multiple surgeries ) , pancreatic exocrine insufficiency as well as numerous upper and lower respiratory tract infections . sputum specimens in his prior admissions had yielded mucoid pseudomonas aeruginosa , klebsiella pneumoniae , aspergillus and candida species . he reported good compliance with his medications , which included inhaled corticosteroids , inhaled dornase alfa , inhaled bronchodilators , inhaled tobramycin , pancreatic enzymes , along with cotrimoxazole and azithromycin prophylaxis . physical exam revealed a poorly nourished young male with a body mass index of 19 kg / m , clubbing and bilateral coarse breath sounds on chest auscultation . chest x - ray showed bilateral bronchiectasis consistent with cf , but no acute cardiopulmonary process . he was empirically initiated on anti - pseudomonal therapy , which included intravenous ceftazidime and tobramycin . despite 2 weeks of intervention he required supplemental oxygen in view of dyspnea and drop in oxygen saturation into the 80s . a modified ziehl - neelsen acid fast stain showed an intermittent staining pattern [ figure 1 ] . the isolate was subsequently sent to the mycobacterial / nocardia research laboratory of the university of texas in tyler , texas , for polymerase chain reaction ( pcr ) and antibiotic susceptibility . the organism was finally identified as n. transvalensis , which was sensitive to cotrimoxazole and linezolid and resistant to amikacin . a modified ziehl - neelsen acid fast stain showed an intermittent staining pattern sputum isolate of nocardia could be either colonization or infection . given the poor response to the usual antipseudomonal therapy , he was presumptively commenced on a trial of intravenous cotrimoxazole ( 20 mg / kg / day ) and linezolid ( 40 mg / kg / day ) . over the next 2 weeks of intravenous therapy , he was discharged home on 4 weeks of oral cotrimoxazole and 1 week of oral linezolid . two follow - up sputum cultures did not yield nocardia species , and the infiltrates resolved on the chest x - ray after 6 weeks of completion of treatment . most nocardia pulmonary infections are primary , but it can spread to the lungs from other sites . pulmonary nocardiosis may manifest as an acute or chronic infection , and the most frequent predisposing factors are chronic obstructive pulmonary disease , bronchiectasis , pulmonary fibrosis , emphysema , asthma , neoplastic disease , organ transplant , human immunodeficiency virus infection and long - term corticosteroid therapy . a study in japan concludes that the most common predisposing factors for nocardial infection were therapy by immunosuppressive agents ( 22.4% ) , cancer ( 6.6% ) , diabetes ( 3.6% ) , tuberculosis ( 3.3% ) and acquired immunodeficiency syndrome ( 2% ) . our patient had received long - term inhalational corticosteroids and , although the dosage was very low , this may have contributed to culture positivity of nocardia . the diagnosis of nocardiosis is mainly by isolation of the organism and identification of species from sputum or bronchoalveolar lavage fluid . petersen et al . first reported a case of an 8-year - old child with cf harboring n. farcinica in bronchoalveolar lavage fluid . radiographic findings are variable and include infiltrates , consolidation , lung masses , single or multiple nodules , pleural effusions and subpleural plaques . in our case , the patient 's sputum isolated pseudomonas aeruginosa and partially acid fast bacteria on admission , and he was on antipseudomonal treatment . after 2 weeks of therapy , our patient had worsening of clinical symptoms like cough , chest pain and dyspnea . chest x - ray showed new infiltrate in the right lower lobe and sputum isolated n. transvalenis . sputum isolation of nocardia from cf patients does not necessarily imply disease , but it may represent colonization ; hence , the need for treatment should be assessed on an individual basis . rosett and hodges et al , have recommended criteria to categorize colonization versus disease . according to the criteria studies have assessed that cotrimoxazole prophylaxis is not a protective factor for preventing breakthrough nocardiosis in immunocompromised patients . similarly , our patient , even though on cotrimoxazole prophylaxis , was diagnosed as having nocardiosis . a unique feature of n. transvalenis is it being resistant to amikacin and other aminoglycosides , while all other nocardia species , like n. farcinica , n. nova and n. asteroides are typically susceptible . n. transvalenis is highly susceptible to cotrimoxazole , third - generation cephalosporin , imipenem and linezolid . hence , we escalated the cotrimoxazole from prophylactic dose to higher dose ( 20 mg / kg / day ) and added linezolid . after 2 weeks of intravenous therapy , significant improvement in clinical symptoms was noticed and he was switched to oral therapy for 4 weeks . repeat sputum specimen after 6 weeks of treatment did not yield n. transvalensis . using a specific rt n . farcinica pcr , bittar et al . demonstrated that nocardia sp , despite treatment , were detected and cultured during the long follow - up in their patient . most case reports and infectious disease specialists suggest to treat with , initially , two susceptible drugs intravenously and to continue with a prolonged period of oral antibiotics . although there are no pathognomonic signs or symptoms of nocardiosis in a patient with cf , a high suspicion is necessary to make a timely diagnosis and treatment , which is associated with improved clinical outcomes . presumptive diagnosis can be made if partially acid fast filamentous branching rods are seen either in sputum or bronchoalveolar lavage fluid .

nocardia species is rarely encountered in cystic fibrosis ( cf ) patients . its isolation usually implies colonization . of all other nocardia species , nocardia transvalensis is very unusual and is clinically distinguishable because of its resistance to aminoglycosides , a standard antinocardial therapy . we report a case of n. transvalensis pulmonary infection in a cf patient .

we have reported a 78 year old man with complaint of abdominal pain and vomiting . in patients preoperative evaluation for surgical management of an intra - abdominal mass , a large intracardiac mass has found incidentally during the echocardiography . pathologic biopsy of right atrial mass that has removed by open heart surgery shown : non hodgkin - b cell lymphoma . the patient has treated by chop chemotherapy regiment successfully and after completion of treatment , there was complete response . gross tumor formation in any of the cardiac chambers has been rare , particularly at the time of presentation and diagnosis of lymphoma ( 2 ) . symptoms were usually very subtle and non - specific , particularly in the setting of co - existing comorbidities ( 3 ) . as imaging modalities and treatment options for lymphoma have improved , more unusual disease presentation might be observed more frequently . in this article we have reported a 78-year - old man with an incidental large cardiac mass whose symptoms was abdominal pain and vomiting in the setting of pre - operative evaluation for surgical management of intra - abdominal mass . a 78-year - old man has affected by abdominal pain , vomiting and weakness and has evaluated at june 2013 . studying his medical history , he had hip joint replacement surgery , then in initial physical examination has shown no significant finding , except a few tenderness on the middle abdominal quadrant . in laboratory assessment , complete blood count , renal function tests - alkaline phosphatase and lactate dehydrogenase were normal , but hemoglobin and transaminases were abnormal as follows : hb = 9.1 mg / dl , ast = 71 u / lit , alt = 656 u / lit . in abdominal ct scan , there was a 71 61 mm mass at the aortic bifurcation in favor of tumoral lesion or adenopathy . ecg had no significant and specific changes and in echocardiography , a large mass with diameter of 74 60 mm has seen in right atrium ( figure 1 ) and lv ejection fraction was 65% . also , in thoracic sections of ct scan , an intra - cardiac mass has seen ( figure 2 ) . bases on these new findings management of the patient has changed to cardiac surgery and open heart surgery has recommended and performed at august 2013 . pathologic examination of removed cardiac mass has reported as below after immunohistochemistry study : hmb 45 and ck : negative ; cd20 : positive . compatible with non - hodgkin lymphoma in favor of b - cell origin ( figures 3 and 4 ) . he has referred to oncologist after recovery of heart surgery ( september 2013 ) . in evaluation of medical history and further physical examination , he had no history of fever and sweating , but weight loss of 2 - 3 kg during recent weeks . karnowsky performance status was 80% . at physical examination , he had an adenopathy of 2 1 cm at left jugulodigastric chain , and the another of 3 1 cm at left supraclavicular area and physical exam was normal otherwise . in bone marrow biopsy , the patient has planned to treat by r - chop chemotherapy regimen ( rituximab - cyclophosphamide - doxorubicin - vincristine - prednisolone ) . he could not provide rituximab due to economic problems , therefor he has received only chop regimen . after first cycle of treatment , cervical adenopathies have disappeared and at the end of seventh cycle , imaging of the neck , chest and abdominopelvic cavity by ct scan had no positive finding of disease but the patient has not satisfied to undergo bm biopsy for second time . patient treatment has completed after eight cycles of chop chemotherapy regimen at february 2014 , but he was in good condition , without any evidences of disease after six months of follow up . cardiac masses have been arising from the heart or pericardium , were potentially lethal whether defined as benign or malignant . metastatic deposits have represented the vast majority of cardiac malignancies ; the common primary malignancies sources have included cancers of lung , esophagus and breast as well as lymphoma , leukemia and melanoma ( 5 ) . cardiac involvement as an initial presentation of malignant lymphoma was a rare occurrence ( 2 ) . secondary involvement of the heart has seen in 8.7 - 27.2% of documented clinical case of lymphoma ( 2 , 6 , 7 ) . despite its life - threatening nature ( 8) , the cardiac manifestations of lymphomatous involvement of the heart these symptoms and signs might include arrhythmias , pericardial effusion or tamponade , tumor embolization and obstruction of blood flow and valvular dysfunction . these symptoms have related on tumor location , size , growth rate , degree of invasion and friability . in the present case and many other reports the majority of intracavital tumors have occurred on the right site of the heart , the reason for which has yet been to be found ( 1 , 11 ) . plain chest radiographs lack sensitivity and specificity as an initial diagnostic tool but could demonstrate cardiomegaly or specific chamber enlargement . echocardiography was the first non - invasive study for examining the chambers of the heart and pericardium ( 12 ) but trans esophageal echocardiography ( tee ) was a more sensitive technique for assessing patients ( 13 ) . ct scan and mri with gadolinium contrast injection were useful tools for demonstrating morphology , location , extension of disease , blood flow and cardiac function ( 14 , 15 ) . fdg - pet imaging has recently reported to reveal previously unsuspected cardiac involvement ( 16 , 17 ) . although traditionally this has required a thoracostomy , less invasive procedures has recently been available such as tee - guided biopsy , endomyocardial biopsy , or percutaneous intracardiac biopsy with combined fluoroscopy and tee or pericardial fluid sampling ( 18 , 19 ) . the available literature suggested systemic chemotherapy was the only effective therapy ( 18 ) and the majority of cases have treated with combination chemotherapy with varying results ( 20 , 21 ) . there was an improvement in response and survival rates by adding of monoclonal therapies to chemotherapy ( 22 ) . radiation therapy has indicated in cardiac mass that progresses despite chemotherapy and its adverse side effects have been pericarditis , cardiomyopathy , diastolic dysfunction , conduction defects and coronary artery disease . cardiac involvement was rare as an initial presentation of malignant lymphoma and has often been subclinical . these tumors have seen more common in the right site of the heart and tee , ct scan and mri with gd were effective tools in assessment of patients . pathologic diagnosis would be essential to management of cardiac masses and the only effective treatment has been chemotherapy .

introduction : cardiac involvement as an initial presentation of malignant lymphoma has been a rare occurrence.case presentation : we have reported a 78 year old man with complaint of abdominal pain and vomiting . in patients preoperative evaluation for surgical management of an intra - abdominal mass , a large intracardiac mass has found incidentally during the echocardiography . pathologic biopsy of right atrial mass that has removed by open heart surgery shown : non hodgkin - b cell lymphoma . bone marrow biopsy was taken and was positive for lymphomatous involvement.conclusions:the patient has treated by chop chemotherapy regiment successfully and after completion of treatment , there was complete response .

spinal epidural abscess ( sea ) is a very rare disease and occurs at an incidence rate of 0.2 to 2.8 cases per 10000 hospital admissions ( 13 ) . the most important factors involved are spinal surgery , recent trauma , immunosuppression , a distal site of infection , intravenous drug use , diabetes mellitus and alcoholism ( 46 ) . however pain and fever are two common complaints in the emergency department that are always missed ( 7 ) . sea represents a spinal emergency condition and a potentially life - threatening disease that can cause severe neurological deficits due to the compressive effect of abscess on the spinal co rd or nerve roots or ischemia ( 7 ) . we will present a case of 15 year old boy with spontaneous pyogenic epidural abscess . a 15 year old boy presented to orthopedic clinic with a 2 week history of urinary retention , back pain , bilateral leg numbness and weakness , lumbar pain exacerbated by movement , sweat and fever . he had received conservative medical treatment with a diagnosis of low back pain but there was no response to analgesic and medical treatment . his lower back pain was exacerbated and severe neurological deficit was also added to his clinical picture . in physical examination , he presented with blood pressure of 100/80 mmhg , pulse rate of 100 pulse / minute and a body temperature of 38.5 c. hematologic examination revealed a white blood cell count of 18600 /l ( neutrophil 70% ) , an erythrocyte sedimentation rate ( esr ) of 68 mm / hr and a c reactive protein 3-plus positive . on physical examination , there was a tenderness over the left side of lumbar vertebrae and on the left and right sides of para - lumbar region . neurological examination showed hyperactive deep tendon reflexes , muscle weakness ( 3/5 ) , and decreased pin prick sensation in the right leg . scan that were taken at the time of admission there were abnormal signal intensity at right paraspinal muscles of l2 and l3 level . the lesion was extended to the posterior aspect of thecal sac and its extra - dural mass effect caused canal stenosis and cauda equina compression to anterior wall of the canal . radiologist recommended emergent laminectomy and debulking of the canal ( figs . 2 , 3 ) . magnetic resonance imaging ( mri ) of lumbar vertebra detected an intensity in paralumbar of l2 and l3 ( arrow ) . intravenous antibiotic ( cefazolin 1gr ) was administered for two postoperative weeks then oral antibiotic ( cephalexin 500 mg every 6 hours ) were continued for 4 weeks . after 6 weeks from the time of operation , white blood cell ( wbc ) counts , esr and crp were all found to be normal . on a three month follow - up period there was no recurrence and the patient was returned to his normal life . its diagnosis must be made promptly because delay in treatment can result in irreversible neurologic damage or even death ( 3 ) . paralysis and death occur in 16% and 15% of patients respectively ( 8) . progression of neurological deficit can vary from few hours to several months ( 3 , 8) back pain in initial stages can lead to a missed diagnosis in spontaneous cases ( 9 ) . radiological modalities are most common diagnostic devices used for detection of abscess within spinal vertebra , but radiographic imaging is not much helpful . magnetic resonance imaging with a sensitivity of 91% is now considered the diagnostic gold standard ( 9).we found staphylococcus aureus on culture , similar to previous reports ( 8) . early recognition and high clinical suspicion before development of symptoms will yield to excellent results . endoscopy - assisted surgery and percutaneous drainage are also reported to be successful treatment modalities ( 2 ) . according to our report , spinal epidural abscess is one of the most important differential diagnosis of acute back pain in patients without a positive history .

epidural abscess of the spinal column is a rare condition that can be fatal if left untreated . it promptly progresses and can cause neurologic paralysis , urinary retention or cauda equina syndrome . compromised immune system that occurs in patients with diabetes mellitus , aids , chronic renal failure , alcoholism , or cancer is a predisposing factor . it mostly occurs in adults . here we would like to report a case of spontaneous pyogenic lumbar epidural abscess with neurological deficit diagnosed in a 15 year old boy . we treated this case successfully with surgical microscopic decompression and drainage .

double - j ( dj ) stents are used to drain urine from the kidney to the bladder and are effective in managing and preventing upper urinary tract ( ut ) obstruction in various urological conditions ( 1 ) . the most common indication of stenting is to prevent and relieve upper ut obstruction ( 2 , 3 ) . the known complications of dj stent include encrustation , fragmentation , proximal and distal migration , dysuria , and frequency ( 1 ) . most of these complications are self - limited and can be managed with suitable urologic interventions and supportive medical management ( 2 - 4 ) . we reported a rare and serious case of vascular migration of a stent to the pulmonary artery , with a long - time remained . the patient was a 47-year - old man with a history of long - lasting renal colic from four months before his admission . the surgeon had difficulty passing the ureteroscope through the intramural ureter and , with frequent manipulation , a 0.035 guidewire was passed through the intramural ureter , after which a dj stent of 4.8f-30 cm was inserted over the guidewire . kub was performed after the procedure for control of the location of the dj , but the resident did not diagnose abnormal pathway of the stent . he then developed gross hematuria for two days and , after passing a large clot in urine , the hematuria stopped , and he had no further urinary symptoms . after two weeks , he came to the clinic , and the surgeon planned to remove the dj stent in the operating room by cystoscopy . the cystoscope was passed into the bladder , but the stent could not be visualized there . migration of the stent to another anatomical position , such as the ureter or retroperitoneal , or vascular migration was suggested . the patient was referred to the radiology department , where kub and chest x - ray ( cxr ) was performed . the stent had an abnormal pathway , probably entering into the inferior vena cava ( figure 1 ) . cxr showed abnormal density that could have been the shadow of the stent ( figure 2 ) . the x - ray revealed an abnormal pathway of the double - j stent that probably entered into the inferior vena cava . the arrow shows the position of the ureteral stone , 8 6 mm in size , at the ureterovesical junction . the patient was referred to the angiography department . in fluoroscopy , during pulmonary angiography , the dj stent was seen completely in the pulmonary artery . the tip of the stent was fixed , which may have been due to wedging in the pulmonary artery branch , thrombus formation in this site , or other causes ( figure 3 ) . other laboratory data including cbc , bun , esr , crp , and creatinine were normal . blood culture was requested , and prophylactic antibiotic and anticoagulant therapies were performed for the patient . there are two methods to remove endovascular foreign bodies : open surgery and intravascular retrieval ( 5 - 9 ) , and it was planned to remove the stent percutaneously . 10 french right femoral sheath was used , and pulmonary angiography was performed ( figure 4 ) . a multipurpose 7f catheter was used to try to release the fixed left tip of the stent , but frequent manipulation was not successful . the right distal tip of the stent was captured by peripheral snare , and the stent was retrieved successfully without any complication . when the peripheral snare was introduced percutaneously from the femoral sheath , and the tip of the stent was captured by it . the common symptoms and probable complications following dj stent insertion include lower ut symptoms , loin pain , hematuria , ut infection , vesicoureteral reflux ( vur ) , stent malposition , luminal obstruction , ureteral erosion or necrosis , stone formation , encrustation , fracture , and migration proximally and distally in the urinary system ( 4 , 10 ) . stent placements can be prone to vessel erosion , and in rare situations , intravascular migration of the dj stent has been reported ( 6 , 10 ) . michalopoulos et al . reported pulmonary thromboembolism immediately after surgery from migration of a stent into the heart and left pulmonary arterial system after pyelolithotomy and antegrade insertion of a pigtail ureteral stent . the stent was removed percutaneously by using the femoral vein as an access site ( 7 ) . percutaneous removal of the migrated stent was performed under fluoroscopic guide ( 5 ) . however , in two other case reports , the stent was removed successfully via cardiopulmonary bypass ( 6 , 8) . although placement of a ureteral stent is easy , serious complications may occur during or after such a procedure ( 5 - 9 ) . in this case , the surgeon blindly passed a 0.035 guidewire through the intramural ureter , when it probably passed into the submucosa and then the iliac vessel . second , use of hydrophilic wire can prevent passing the wire into the submucosal layer . moreover , dj stent insertion under fluoroscopic guide can reduce these complications . in the absence of fluoroscopy if abnormal position of the stent is suspected , other diagnostic methods , such as ct with iv contrast , can confirm or disprove the diagnosis . finally , clinicians should pay attention to warning symptoms of the patient , such as significant hematuria and severe abdominal pain . in this case , the stent was completely in the pulmonary arteries . multiple treatment options can be used for this situation , such as percutaneous removal by snare catheter , when possible , and open surgery . because of the good condition of the patient and available expertise for endovascular intervention , it was decided to remove the stent by the endovascular method with femoral access . if expertise for endovascular intervention is not available , open surgical removal is an alternative , safe method . intravascular migration of dj stents is a rare but serious complication . when there is doubt , complementary diagnostic procedures and early intervention are recommended . percutaneous removal of a migrated stent under fluoroscopic guidance by snare catheter from femoral access is a safe and effective treatment .

double - j ( dj ) stents are the main tools used in urological practice for prevention and treatment of obstruction . stenting is also mandatory after complicated ureteroscopy or tul ( transureteral lithotripsy ) . known complications are upper migration of dj stents into the kidney and lower migration to the bladder . in a man with an impacted right lower ureteral stone , a dj stent was placed because the ureteroscope was not passed from an intramural ureter . we reported a very rare complication of dj ureteral stent placement with intravascular migration to the pulmonary arteries , which was removed percutaneously through the right femoral vein under fluoroscopic guidance .

in the previous issue of critical care , vermeulen windsant and colleagues demonstrate that transfusion of packed red cells is associated with a transient increase in plasma free haemoglobin and scavenging of nitric oxide ( no ) in vitro . they also demonstrate that older units of blood have more free haemoglobin in their supernatants . the implication is that the possible but unproven adverse effects of older transfused blood may be due to the inadvertent administration of free haemoglobin , enhanced no scavenging , and modified microvascular flow . however , it is still unclear whether allogenic blood stored for longer periods of time has adverse effects for the recipient . observational data demonstrate that the use of older blood in patients who have undergone cardiac surgery is associated with an increased mortality . by contrast , others have demonstrated variable effects on physiological indices that are surrogates for organ perfusion . these contrasting data and widespread equipoise from clinicians have fuelled several clinical trials : the age of blood evaluation study ( iscrtn 44878718 ) , the red cell storage duration study ( nct00991341 ) , and red cell storage duration and outcomes in cardiac surgery ( nct00458783 ) . these studies differ in their control groups and patient populations , with the first recruiting a wide group of critically ill patients and the latter two just examining those that have undergone cardiac surgery . the implications for the blood transfusion services , who typically issue the oldest blood to conserve precious and limited stocks , are immense . the limitation of the present study is that only one mechanism of many is investigated and the link to adverse outcomes in patients is not considered . indeed , it is possible that several mechanisms exist , such as the administration of older more fragile erythrocytes , depletion of adenosine triphosphate and 2,3-diphosphoglycerate , or the presence of cytokines , histamine , and complement in supernatants . furthermore , free haemoglobin has other effects such as renal tubular toxicity and decompartmentalisation of iron , which may influence the redox balance , microbial killing , or intracellular signalling . free haemoglobin and no scavenging may thus be events that occur in parallel with other phenomena . nevertheless , nonselective inhibition of the no pathway has been associated with increased mortality in sepsis and the authors describe a very plausible mechanism . the physiological and biochemical readout from the aforementioned clinical trials will be key in our understanding and the current study will inform such analyses . important also is the authors ' observation that higher pre - transfusion haptoglobin levels appear to be protective in terms of a rise in free haemoglobin and no scavenging . the implication is that the administration of haptoglobin may mitigate against any adverse effects of free haemoglobin , particularly in the setting of significant haemorrhage and cardiac surgery when levels are likely to be low . this is supported by the fact that free haemoglobin appears to stay primarily in the blood compartment in conjunction with any administered haptoglobin . quantitatively , the rise in plasma free haemoglobin following a 2-unit transfusion suggests that the free haemoglobin administered is diluted about 13-fold and that the volume of distribution is of the order of 7 l , similar to an adult 's total blood volume . haemolysis may also be a significant contributor to organ dysfunction in other scenarios encountered in the critically ill : haemoglobinopathies , severe infection ( for example , malaria , escherichia coli ) , cardiac valvular disease , and the use of extracorporeal therapies such as renal replacement therapy , cardiopulmonary bypass , and extracorporeal membrane oxygenation . mechanisms are important . this study generates the hypothesis that free haemoglobin present in older blood may be detrimental by modulating the no pathway , which merits consideration when clinical trials are evaluated .

in the previous issue of critical care , vermeulen windsant and colleagues demonstrate that transfusion of packed red cells is associated with a transient increase in plasma free haemoglobin and scavenging of nitric oxide in vitro . they also demonstrate that older units of blood have more free haemoglobin in their supernatants . whether the administration of older stored blood results in adverse clinical outcomes is a topical clinical question . the present study proposes a mechanism for transfusion - related harm but also has implications for patients who have other sources of free haemoglobin in their circulation .

cysticercosis , caused by encysted larva of taenia solium , is the most common parasitic infestation of the central nervous system , but spinal cysticercal infection is relatively rare with the incidence reported as only 0.75.85% . majority of the reported cases of intramedullary cysticercosis ranged in age between 20 and 45 years and the condition is very rare in the pediatric age group . although surgery is the mainstay of treatment , magnetic resonance imaging ( mri ) can help in preoperative diagnosis , thus avoiding the high morbidity and mortality associated with the surgery . we hereby report a case of a 10-year - old female patient with isolated intramedullary cysticercosis , who responded dramatically to medical treatment . a 10-year - old female patient presented with gradual weakness in both lower limbs and numbness from lower thorax to foot for 3 months . neurological examination revealed spastic paraplegia ( motor power 3/5 ) , brisk b / l deep tendon reflexes with extensor plantar and loss of sensations to pinprick bilaterally below t5 dermatomes . contrast - enhanced mri of the spine revealed an isolated , well - defined , intramedullary cystic lesion measuring 1.8 1.0 cm at the level of d2 vertebral body . on t1-weighted imaging , the lesion was hyperintense on t2-weighted image with an isointense mural nodule [ figure 1b and c ] . csf examination done revealed elevated protein and normal glucose levels with increased lymphocytes , and few eosinophils and positive enzyme - linked immunosorbent assay ( elisa ) for anti - t . ( b ) sagittal and ( c ) axial t2-weighted images showing a well - defined cystic hyperintense lesion with isointense mural nodule ( scolex ) opposite d2 vertebrae associated with adjacent cord edema . ( d ) contrast - enhanced mr images showing peripheral enhancement of lesion keeping the provisional diagnosis of cysticercosis in mind , empirical treatment with antihelminthic along with steroids was started . marked clinical improvement was seen within a few days in the form of increase in limb power and return of tendon reflexes . cysticercosis is endemic in most of the developing countries like brazil , mexico , korea and south asian countries . intramedullary cysticercosis is rare even in endemic areas , with an incidence reported between 0.7% and 5.85% . four forms of spinal neurocysticercosis have been identified : vertebral , extradural , intradural extramedullary and intramedullary , the commonest being intradural extramedullary region . extramedullary form occurs due to csf dissemination ( ventriculo ependymal migration ) of focus in brain , whereas intramedullary forms result due to hematogeneous spread of the parasitic larvae . intramedullary cysticercosis is most common in the thoracic spine due to high vascularity of this spinal segment . neurological manifestations can be produced by the parasite itself causing mechanical compression and obstruction , surrounding inflammation , cord edema and residual scarring , pachymeningitis or syrinx formation . usual mr findings include a cystic lesion with signal intensity similar to csf on t1-weighted and t2-weighted mri . a mural nodule , which represents the scolex , appears isointense or hyperintense on t1-weighted mri and reverse on t2-weighted mri . the thickened capsule is hyperintense on t1 wi and hypointense on t2 wi.[57 ] the role of mri in the diagnosis of neurocysticercosis is well documented . it helps not only in the diagnosis but also in the preoperative localization of the lesion and the extent of involvement . mri findings in neurocysticercosis can be non - specific , especially when scolex is not seen and the differentials for an intramedullary cystic lesion include hydatid cyst , dermoid cyst , post - traumatic syrinx , metastases and granulomatous diseases . but the presence of scolex and history of origin from endemic area enables differentiation from other disease processes . csf examination in patients with neurocysticercosis usually reveals moderate lymphocytic pleocytosis , variable eosinophilic pleocytosis , elevated protein , and low / normal glucose levels . surgery can eliminate the compressive element , especially in patients presenting with acute onset of symptoms , and in cases wherein the diagnosis is doubtful , surgical excision should be done . however , operative mortality is reported to be 15% and the morbidity is reported to be 85% , but excellent results can be obtained with microneurosurgery . based on mri findings , some authors now recommend the use of antihelminthic drugs along with steroids without resorting to surgery . treated their patients with drug therapy for 75 days and found that intramedullary cysticercosis resolved completely in a patient who was on albendazole treatment for cerebral neurocysticercosis . found that out of four patients in his series , two reported complete resolution of the cyst on mri , and marked clinical improvement was seen in all the four patients . ahmad et al . reported successful treatment of a case of intramedullary cysticercosis with a 2-week course of medical therapy . ahmad et al . reported achievement of cure within 2 weeks ; however , some authors suggest 48 weeks treatment . our patient was treated with albendazole and steroids for 4 weeks and showed marked clinical improvement . isolated cysticercosis of spine is difficult to suspect ; however , correlation of clinical findings , history of endemic area origin and typical mri findings can help in making the diagnosis , thus obviating the need for surgery which is associated with high morbidity and mortality .

neurocysticercosis is the most common parasitic infection of the central nervous system caused by larvae of taenia solium . spinal cysticercosis is an uncommon site of cysticercal infection , and isolated intramedullary involvement is even rarer . we present a case of 10-year - old girl who presented with gradual onset paraparesis with sensory loss and bowel and bladder incontinence . magnetic resonance imaging ( mri ) of spine revealed a cystic lesion with mural nodule ( scolex ) which was diagnostic for cysticercosis . patient was treated with antihelminthic , which led to marked clinico - radiological improvement .

replica exchange molecular dynamics simulations of d(cgcgaattcgcg)2 and d(cgcccgcgggcg)2 dodecamers were performed to enhance the sampling by accelerating barrier crossings at elevated temperatures . the generalized born with molecular volume ( gbmv ) implicit solvation model was used with dielectric constants 20 , 40 , and 80 . the initial d(cgcgaattcgcg)2 structure was obtained from x - ray analysis ( pdb i d code : 1bna ) and the initial d(cgcccgcgggcg)2 structure was obtained by mutating the base sequence in the x - ray structure of 1bna . replica exchange simulations were performed using eight replicas between temperatures 300 and 400 k. although the replicas visit higher temperatures than 300 k , only the sampling at 300 k is considered and reported here . langevin dynamics was performed using a friction coefficient of 50 ps to control the temperature of the system . simulations were carried out for 50 ns for each replica with a total simulation time of 400 ns and replica exchange was attempted every 10 ps . the first 6 ns of each replica was excluded during the analysis of the simulations . nonbonded interactions were cut off at 18 with a switching function becoming effective at 16 and a cutoff at 20 was used for the nonbonded list . all simulations were performed using the charmm program package ( v c37a2 ) with charmm36 force field for nucleic acids . replica exchange simulations were carried out using the multiscale modeling tools for structural biology ( mmtsb ) in combination with charmm . cluster analysis of the conformations was carried out with the kclust program in mmtsb . helicoidal and backbone parameters of dodecamers were analyzed by using the 3dna program package .

the effect of reduced dielectric environments on the conformational sampling of dna was examined through molecular dynamics simulations . different dielectric environments were used to model one aspect of cellular environments . implicit solvent based on the generalized born methodology was used to reflect different dielectric environments in the simulations . the simulation results show a tendency of dna structures to favor noncanonical a - like conformations rather than canonical a- and b - forms as a result of the reduced dielectric environments . the results suggest that the reduced dielectric response in cellular environments may be sufficient to enhance the sampling of a - like dna structures compared to dilute solvent conditions .

sinus histiocytosis with massive lymphadenopathy , also known as rosai - dorfman disease , is a very rare inflammatory entity . it can be accompanied by fever , neutrophilia , polyclonal hypergammaglobulinemia and an elevated erythrocyte sedimentation rate . sinus histicytosis is part of the non - langerhans cell histiocytosis or cutaneous non - histiocytosis x , in which proliferation of macrophages always lack langherhans granules . this group of disorders is usually nonaggressive , self - healing and can affect both children and adults [ 27 ] . we report a very rare case of sinus histiocytosis , which presented with only a solitary cutaneous nodule and no other clinical manifestations or lymphadenopathy . a 75-year - old man was referred to the dermatology department with a six - month history of an asymptomatic solitary nodule on the trunk . his general practitioner had diagnosed a possible pyogenic granuloma , but could not exclude a malignant lesion . examination revealed a 1.2 cm purple , soft , shiny nodule with a violaceous peripheral rim in the interscapular area ( figure 1 ) . the lesion was excised immediately with the clinical suspicion of a probable amelanotic melanoma , squamous cell carcinoma , or merkel cell carcinoma in mind . the pathology reported a dermal lesion composed of an infiltrate of cells expanding and filling the papillary dermis and spreading into the deep reticular dermis ( figure 2a ) . the infiltrate included prominent macrophages with pale , vacuolated cytoplasm , some multinucleate macrophages of similar staining characteristics , other small , untransformed macrophages and a large number of lymphoid cells . lymphocytes were predominant , but a modest number of plasma cells with a smaller number of other inflammatory cells were seen . lymphophagocytosis or emperipolesis ( the presence of an intact cell within the cytoplasm of another cell ) was noticed and the diagnosis of sinus histiocytosis established ( figure 2b ) . with this surprise diagnosis the laboratory investigations ( full blood count , c reactive protein , erythrocyte sedimentation rate , coagulation screen , electrolytes , renal and liver function tests ) were all normal . levels of immunoglobulins g , a , m and serum protein electrophoresis were also normal . approximately 365 cases of sinus histiocytosis with massive lymphadenopathy , also known as rosai - dorfman disease , have been described in the literature . sometimes the cutaneous manifestations may be the initial and sole presenting feature of this disease . massive bilateral cervical lymphadenopathy is usually the hallmark of this condition ; however , our patient presented with only a solitary cutaneous nodule with no lymph node enlargement , making the diagnosis more difficult . cutaneous lesions are reported to be polymorphic with yellowish patches and macules , reddish - brown papules , plaques and nodules that can become eroded or ulcerated . the aetiology of the disease remains unclear , but a possible disturbance of cell - mediated immunity or a primary infection hypothesis with epstein - barr virus have been suspected . the differential diagnosis is very broad and includes both proliferative and inflammatory diseases . in our case most of the sinus histiocytoses are self - healing with spontaneous regression over a period of months to years . very rarely when an internal organ is involved , for example , liver , spleen , upper respiratory tract , then glucocorticoids or other chemotherapeutic regimens may be required . our patient is undergoing close clinical follow - up , as it is possible that the solitary cutaneous nodule is the precursor to more extensive sinus histiocytosis ( rosai - dorfman disease ) . however , even after a long follow - up of 12 months , there are no signs of disease progression .

sinus histiocytosis is a rare inflammatory disease mainly affecting the cervical lymph nodes , presenting with skin lesions in 10% of cases . our patient had a solitary nodule on the trunk without any other clinical signs . the histology reported a dermal neoplasm composed mainly of macrophages and lymphocytes . macrophages were aggregated in clusters resembling lymph node sinuses . lymphophagocytosis or emperipolesis ( the presence of an intact cell within the cytoplasm of another cell ) was noted and the diagnosis of sinus histiocytosis established .

lung cancer is one of the most common types of cancer worldwide , it is highly aggressive and has a high rate of distant metastasis ( 1 ) . subcutaneous metastasis from lung cancer has been well described ( 2 ) ; however , reports of subcutaneous metastasis of lung cancer after three surgeries for recurrent brain metastasis are scarce . we herein report a rare case of subcutaneous metastasis from pulmonary adenocarcinoma and provide a brief review of the relevant literature . a 49-year - old female patient was admitted to our hospital due to a fast - growing mass in the left temporal scalp at a craniotomy site ( fig . a routine magnetic resonance imaging ( mri ) revealed recurrent lesions in the left temporal and parietal lobes and a separate large nodular mass in the subcutaneous tissue coinciding with the site of the previous craniotomy ( fig . a lung computed tomography ( ct ) scan at another hospital revealed a 2.4-cm mass in the upper lobe of the left lung . the patient did not undergo surgery due to enlarged mediastinal lymph nodes , but was treated with radiosurgery ( 5 years ) and chemotherapy ( 2 years ) . the subsequent ct scan demonstrated no residual or recurrent adenocarcinoma in the lung . in march , 2013 , a routine mri scan at another hospital revealed brain metastases in the left temporal and parietal lobes . the patient underwent subtotal resection of the tumors and decompressive craniectomy at the first hospital of jilin university ( changchun , china ) . in may , 2014 and august , 2015 the patient suffered recurrent brain metastases in the left temporal lobe , which were resected . , the patient was admitted to our hospital due to a fast - growing mass in the left temporal scalp at the craniotomy site . the patient 's medical history was unremarkable , apart from her father having been a heavy smoker for 30 years . the laboratory test results for tumor markers were as follows : carcinoembryonic antigen , 191.6 ng / ml ( normal , < 3.4 ng / ml ) and carbohydrate antigen 153 , 38.03 u / ml ( normal , < 25.0 u / ml ) . peripheral blood cell counts , liver and renal function tests and hormonal levels were within the normal range . as the location of the mass corresponded to the site of the craniotomy , seeding was considered . total excision of the lesions in the left temporal and parietal lobes and the mass in the subcutaneous tissue was performed ( fig . 4 ) , the masses diffusely expressed thyroid transcription factor-1 ( ttf-1 ) and cytokeratin ( ck)7 , the ki-67 index was 25% , whereas villin , ck20 and wilms tumor-1 were negative . cutaneous metastasis is caused by primary cancer - derived cells that grow in the skin ( 3 ) . according to the published literature , the overall incidence of cutaneous metastasis is 2.95.3% ( 4 ) , and 112% in lung cancer ( 2,58 ) . cutaneous metastasis usually presents as solitary or multiple nodules sized 5 mm-10 cm that are firm , immobile and covered with normal skin . in the present case , the nodule was firm , relatively immobile , and of normal yellow color . when the primary tumor is an adenocarcinoma , other diagnoses such as melanoma and hematopoietic malignancies should be considered ( 9 ) . the patient in the present study had undergone multiple surgeries with subsequent complications associated with the surgical wounds , which may have contributed to the scalp metastasis . certain cases of subcutaneous metastasis of lung cancer may manifest as purple or bright red masses that are ulcerated or cauliflower - like , accompanied by bleeding ( 3,10 ) . the patient in the present case did not experience bleeding , as the mass was discovered early . the scalp accounts for 46.9% of all cutaneous metastases and it is a relatively frequent metastatic site , possibly due to the abundant blood supply and immobility ( 4 ) . skin metastases from internal malignancies tend to occur at a site near the primary tumor ( 11,12 ) through various routes , including lymphatic spread , hematogenous spread and direct implanting . the patient underwent three surgeries for brain metastasis and the mass was located at the surgical site . therefore , the mass was considered to be an implantation metastasis from the previous brain metastases . our patient reported swelling and pain ; therefore , she underwent surgical resection in an attempt to provide local palliation ( 13 ) . during surgery surgical seeding of tumor cells is a known complication of lung cancer surgery ( 14 ) . we considered that subcutaneous spreading of the intracranial tumor may have occurred , rather than metastasis from the lung . the localization of a metastasis along a surgical incision site indicates tumor seeding as the likely mechanism , particularly when a metastasis is apparent on imaging soon after surgery , as in this case . we consider that scalp metastases due to intraoperative seeding may occur in all histopathological grades of lung cancer . the patient in the present case was at high risk for extracranial spreading of intracranial tumors , as she had undergone multiple surgeries . surgical bone defects may compromise the natural barriers to dissemination of intracranial tumors , providing access to the lymphatic system , blood vessels and connective tissues . vascular invasion of the extracranial tissues , migration of neoplastic cells and further growth may potentiate extracranial metastases . however , differentiation between implantation of tumor cells and metastasis from the lung may be difficult . prevention of extracranial spreading of intracranial tumors following resection may be achieved by appropriate closure of the dura mater , changing surgical instruments and gloves for wound closure after the intracranial phase of the surgery , and copious saline irrigation of the wound prior to closure . in our patient , the histology of cutaneous metastasis from lung cancer most commonly reveals adenocarcinoma , followed by squamous / small - cell carcinoma , and large - cell carcinoma ( 8) . immunohistochemistry , particularly ck7ck20 ( 15 ) and ttf-1 is useful for identification of adenocarcinoma . in the present case , ttf-1 , a tissue - specific transcription factor expressed in epithelial cells of the thyroid gland and lung ( 16 ) , confirmed that the primary origin of the adenocarcinoma was the lung . following three surgeries for brain metastases and one surgery for cutaneous metastasis , the patient recovered well and was able to care for herself . the presence of cutaneous metastases in lung cancer is associated with a poor prognosis , as this is an indication that the primary cancer is advanced . the patient in the present case was followed up for > 4 months and continues to show good recovery . the present study was approved by the ethics committee of the first hospital of jilin university . informed patient consent was obtained regarding the publication of the case details and accompanying images .

lung cancer is a common malignancy that is frequently associated with distant subcutaneous metastasis . however , reports of subcutaneous metastasis of lung cancer after three surgeries for recurrent brain metastasis are scarce . the present study describes the case of a 49-year - old female patient who was admitted to our hospital with a cutaneous mass . the patient had a history of lung cancer and had undergone three surgeries for brain metastases . the subcutaneous mass was considered to be an implantation metastasis from the previous brain metastases . the cutaneous mass was grossly resected and histopathological examination revealed adenocarcinoma . this case highlights the need to perform a comprehensive analysis for suspected subcutaneous masses in lung cancer patients . furthermore , pathological examination is crucial for accurate diagnosis and timely treatment .

persistent left superior vena cava ( plsvc ) is one of the most common venous anormaly of the thorax . however , the incidence is in less than 0.5% in the general population but occurs in approximately 4% of patients with congenital heart disease.1 ) sinus venosus atrial septal defect ( svd ) accounts for 4 - 11% of all atrial septal defect ( asd ) and partial anomalous pulmonary venous connection ( papvc ) is present in about 90% of patients with svd . anomalies of systemic venous connection are usually found incidentally in asymptomatic patients , but their identification is sometimes crucial especially when cardiac or abdominal aorta surgery is planned . we present a case of a complex anomaly of systemic and pulmonary venous return associated with svd which was detected by computed tomography ( ct ) pulmonary angiography and echocardiography . modern imaging techniques are not only useful for detecting congenital cardiac defects but also for detecting systemic and pulmonary venous connection anomalies . this case demonstrates the importance of knowledge of the anatomical basis of venous connection and identification of one lesion should prompt an appropriate investigation of other potential anomalies that may be less obvious . a 41-year - old man was admitted to the hospital because of palpitation and exertional dyspnea . on auscultation , fixed splitting of the second heart sound and a systolic murmur over the pulmonary valve area were heard . transthoracic echocardiography ( tte ) showed enlargement of the right ventricle and an extremely dilated coronary sinus with normal function of both ventricles ( fig . 1a ) . on the second day of admission , we performed a ct pulmonary angiography . ct also revealed an asd with anomalous return of the right superior pulmonary vein to the right superior vena cava ( svc ) ( fig . a right arm peripheral vein was injected with agitated saline , and normal opacification was noted in the right atrium , followed by opacification of the right ventricle ( fig . next , the left arm peripheral vein was injected with agitated saline and abnormal opacification of the coronary sinus was first noted , followed by opacification of the right atrium and then right ventricle ( fig . coronary ct angiography with 3-dimensional reconstruction demonstrated a more detailed structure of plsvc drainage into the right atrium via a dilated coronary sinus ( fig . five months later , follow - up transthoracic echocardiography showed near - normalization of the right ventricular and coronary sinus size . plsvc occurs in approximately 0.3 - 0.5% of the general population and characteristically drains into the coronary sinus . during and after embryonic development of svc , svc develops on the right side from a portion of the right anterior cardinal vein . on the left side , part of the left anterior cardinal vein undergo normal regression to form the ligament of the left vena cava.1 ) plsvc results from the persistence of the left anterior cardinal vein . usually , plsvc is asymptomatic and discovered incidentally during imaging study and pacemaker implantation or central catheterization but sometimes their elucidation is crucial especially during cardiovascular surgery.2 ) plsvc should be considered whenever a dilated coronary sinus is identified at echocardiography and the diagnosis could be confirmed by saline contrast echocardiography.3 ) other modern imaging modalities such as ct or magnetic resonance imaging ( mri ) can be used to confirm the diagnosis . in our case , we could not consider the presence of plsvc before performing ct pulmonary angiography just because of the focus on volume overload of right - sided heart chambers . we performed a contrast echocardiography based on the information obtained from ct pulmonary angiography . in our case , it is estimated that 10 - 15% of patients have an asd and approximately 85% of papvc are associated with svd.4)5 ) usually , the diagnosis of papvc can be made by echocardiography , and cardiac catheterization along with angiography is often performed for confirmation of the diagnosis . nowadays , ct , mri and tee with contrast examination are considered as sensitive methods for the detection of papvc.6 ) in our case , the diagnosis of papvc was missed on routine tte , and a definitive diagnosis of papvc could be made on ct pulmonary angiography . some authors have previously reported a combined anomaly of systemic and pulmonary venous return associated with svd.7)8 ) these authors especially emphasized the importance of new imaging modalities in diagnosing complex anomaly of systemic and pulmonary venous return associated with svd . also in our case , ct made a definitive diagnosis of the anomaly and provided more detailed structural information . however , this case report also illustrated that careful echocardiographic examination should be performed using several windows and even contrast for diagnosing the anomalies of systemic and pulmonary venous return combined with congenital heart disease . and consideration is required when the case has accompanying cardiac abnormalities besides plsvc such as an asd , when coronary sinus is extremely dilated and it is coexisting with enlargement of right ventricle .

we present a case of persistent left superior vena cava , anomalous right pulmonary venous connection to the right - sided superior vena cava and sinus venosus atrial septal defect detected by computed tomography ( ct ) pulmonary angiography and echocardiography . these defects were surgically corrected using a double - patch technique . in fact , ct can provide anatomical information about a complex anomaly in the systemic and pulmonary vasculatures . though modern imaging techniques are useful for detecting complex cardiovascular disease , careful echocardiographic examination should be performed to diagnose complex cardiac anomalies .

a 55-year - old woman presented with diplopia that had developed 12 days prior . the diplopia was preceded five days earlier by malaise and two days earlier by tingling sensation and pain in the right shoulder . three days after the occurrence of paresthesia , the patient developed multiple painful vesicular eruptions on the right shoulder . after three more days , the tingling sensation involved the right lower leg . on presentation , she was found to have 35 prism diopters of esotropia , which worsened on left gaze at distance and near in the primary position . visual acuity , the margin - reflex distance , levator function of the eyelid , anterior segments and fundi were normal in both eyes . sensation was also decreased in the right c3 to c5 dermatomes . although mental status was intact and brain magnetic resonance imaging ( mri ) was normal , an analysis of cerebrospinal fluid ( csf ) showed pleocytosis of 26 cells / mm and immunoglobulin ( ig ) g varicella zoster virus ( vzv ) antibody . the skin lesions and shoulder weakness improved within one week . on the 14th day after admission , the marginal reflex distance 1 was 1.5 mm in the right eye and 3.5 mm in the left eye . pupils were reactive in both eyes , however were anisocoric with the pupil size of 3 mm in the right eye and 4 mm in the left eye in light . anisocoria increased in the darkness to 3 mm in the right eye and to 4.5 mm in the left eye ( fig . there was no change in the abduction limitation of the left eye . by the 22nd day , when she returned to clinic one month after discharge , she demonstrated improvement of the abduction limitation in the left eye ( fig . when horner 's syndrome is associated with ipsilateral sixth nerve palsy , the condition strongly implies a posterior cavernous sinus lesion . however , the present case developed a contralateral abducens nerve palsy associated with horner 's syndrome , which has not been previously reported . unlike the ipsilateral condition , it is not easy to explain the coexistence of horner 's syndrome and a contralateral abducens nerve palsy with a single localized lesion because the pathways of the oculosympathetic nerve and the contralateral abducens nerve are distant from each other . the present case had additional multiple neuronal involvements including the sensory and motor systems , as well as meninges . furthermore , the sensory symptoms presented in multiple dermatomes of both the upper and lower extremities . thus , it is difficult to confine the lesion . among diseases causing multiple neurological disorders the clinical manifestations of herpes zoster such as painful eruptions , paresthesia , and arm weakness were helpful clues . diagnosis was based on csf pleocytosis and the presence of igg vzv antibodies in csf . when a patient presents with ophthalmoplegia , facial paralysis , and motor dysfunction , peripheral neuromuscular diseases such as guillain - barre syndrome , particularly miller fisher syndrome , should be ruled out . firstly , paresis of extraocular muscles in miller fisher syndrome is typically bilateral and accompanied by ataxia and areflexia . however , our patient did not demonstrate areflexia or ataxia , and only the abduction was limited unilaterally . secondly , she developed right arm weakness accompanied by vesicular skin eruptions which also favors zoster meningitis rather than miller fisher syndrome . thirdly , ataxia in miller fisher syndrome consists of a lack of voluntary coordination of muscle movements , but the arm weakness in our patient was muscular paralysis . lastly , csf study revealed pleocytosis commonly seen in viral meningitis , not albuminocytological dissociation in miller fisher syndrome . the presence of the zoster meningitis explains the mechanism of multiple neuronal involvements . in zoster meningoencephalitis , vzv can reach various areas of the central nervous system through meninges and causes diffuse neuronal damage . vzv may lead to clinical manifestations such as headache , malaise , pyrexia , nerve palsies , paresthesia , hemiplegia , and even horner 's syndrome . generally accepted pathogenesis of vzv includes a direct cytopathic effect on neural tissue , an allergic reaction of the nerve to the virus , and an occlusive vasculitis induced by the virus . in conclusion , we suggest that viral infection should be considered with a high priority in the diagnosis of multiple neuronal dysfunctions . in particular , if neurological signs or symptoms manifest in both eyes , systemic as well as central nervous lesions should be ruled out . the clinical findings of the present case may be helpful in diagnosing patients who present with several neuronal involvements .

a 55-year - old woman presented with diplopia following painful skin eruptions on the right upper extremity . on presentation , she was found to have 35 prism diopters of esotropia and an abduction limitation in the left eye . two weeks later , she developed blepharoptosis and anisocoria with a smaller pupil in the right eye , which increased in the darkness . cerebrospinal fluid analysis showed pleocytosis and a positive result for immunoglobulin g antibody to varicella zoster virus . she was diagnosed to have zoster meningitis with horner 's syndrome and contralateral abducens nerve palsy . after intravenous antiviral and steroid treatments , the vesicular eruptions and abducens nerve palsy improved . horner 's syndrome and diplopia resolved after six months . here we present the first report of horner 's syndrome and contralateral abducens nerve palsy associated with zoster meningitis .

sharp objects such as a toothpick sometimes fail to pass and may cause a perforation . the possible complications reported in the literature include obstruction , perforation , haemorrhage , fistula formation and sepsis . toothpick injury to the gut is a rare condition for which a low intellectual quotient and extremes of age have been described as predisposing factors . diagnosis is made difficult by the fact that few patients remember swallowing a toothpick , the delay before onset of symptoms is extremely variable , the physical and laboratory findings are unspecific and the injury is frequently caused by a wooden toothpick which is radiolucent . the most frequently reported locations for toothpick - related perforation of the gut include the caecum , sigmoid colon , duodenum and stomach . we present the case of a patient who sustained a perforation of the splenic flexure of the colon by a wooden toothpick accidentally ingested . a 23-year - old otherwise healthy student is brought by his mother to the emergency department for a left - sided burning abdominal sensation 6 days after accidentally ingesting a wooden toothpick . he initially consulted in a health centre immediately after the incident with no symptoms and was advised to go back home . his mother insists on telling us that she thinks the toothpick is the cause of the problem . physical examination reveals normal vital signs and tenderness on deep palpation of the left flank . full blood count elicits a leucocytosis ( 11 400/mm , 86% of neutrophils ) , and abdominal ultrasound reveals an agglutination of bowel around the splenic flexure of the colon , but no foreign body is identified . a mini - laparotomy performed 18 h after admission shows localized peritonitis in the left hypochondrium with the greater omentum and a small bowel contributing to circumscribe the infectious process . a wooden toothpick is discovered , partially in the peritoneal cavity , perforating the anti - mesenteric border of the splenic flexure of the colon ( fig . 1 ) . the toothpick is removed ( fig . 2 ) and the perforation of the colon closed with an absorbable polyglactin suture and protected with an omentum patch . figure 1:a peroperative view showing the colonic perforation ( tip of the forceps ) . figure 2:the wooden toothpick immediately after removal . a peroperative view showing the colonic perforation ( tip of the forceps ) . the wooden toothpick immediately after removal . the incidence of this type of lesion has been reported to be 0.2 per 100 000 people every year in the usa . our patient displayed none of the previously described related factors such as dementia , young or old age or carriage of denture . it is likely that this young man continued to play with the toothpick after use . longer delays between ingestion of the toothpick and diagnosis have been described previously [ 2 , 3 ] . according to li and ender , the delay ranges between 1 day and 15 years ! swallowed toothpicks are likely to cause a variety of lesions including perforation , bleeding , obstruction and haemorrhage [ 2 , 3 ] . septic complications have also been described , sometimes related to the migration of the toothpick out of the digestive tract . all the portions of the gut could be concerned by the perforation [ 1 , 58 ] , but they more frequently occur in areas controlled by sphincters , physiological narrowing and acute flexures . the most commonly described locations include the caecum , the duodenum , the sigmoid and the stomach [ 3 , 68 ] . the diagnosis is not always obvious ; it has been reported that the toothpick could be unnoticed in > 80% of cases . in our case , it is the toothpick! most patients consult for an abdominal pain , usually non - specific and often misleading [ 2 , 3 ] . standard x - ray studies usually fail in identifying the toothpick . as in our patient , many cases are diagnosed or confirmed only during laparotomy or endoscopy [ 2 , 3 , 7 , 8 ] . once this condition is diagnosed , the treatment is usually easy and the recovery uneventful [ 2 , 7 , 8 ] . this case indicates that there is need to increase awareness of healthcare providers and populations on the potential dangers of an ingested toothpick . each time this incident occur , it is advisable to follow - up such patients until the foreign body is properly traced as it may cause damage even months or years after .

we present the case of a 23-year - old man who consulted in the emergency department 6 days after ingesting a wooden toothpick with features of a localized peritonitis in the left upper quadrant of the abdomen . surgical exploration revealed a perforation of the splenic flexure of the colon by the toothpick during its migration . this rare case is analysed in the light of the current literature . there is need for a greater awareness about the dangers of a swallowed toothpick . it is advisable to always trace the toothpick until it is found or expelled .

tardive dyskinesia ( td ) is one of the most significant side effects of antipsychotic medications . the average prevalence rate has been estimated to be around 30% for individuals taking antipsychotic medications.1 ) risk factors associated with td include elderly patients , females , use of typical antipsychotics , higher dose of antipsychotics for prolonged period , extrapyramidal symptoms , cognitive deficits , structural brain damage.2,3 ) diabetes mellitus ( dm ) has been reported to be a risk factor for development of td.2,4,5 ) although there remains some uncertainty about the causal mechanisms of this link,6 ) studies have suggested an independent association between dyskinesia and diabetes.7 ) mukherjee et al.8,9 ) suggested a possible association between td and impaired glucose metabolism . other studies report a higher incidence of dm in relatives of patients with td , suggesting a genetic link between dopamine mechanisms and glucose regulation.10 ) schultz et al.11 ) suggested that hyperinsulinemia and hyperglycemia associated with insulin resistance contribute to td pathogenesis . but the use of clozapine is probably best supported for management of td.12 ) quetiapine,13 ) another weak striatal dopamine antagonist and olanzapine14 ) are also effective . but in patients of schizophrenia with td with comorbid type 2 dm clozapine and olanzapine are not preferred , as they have been strongly linked to hyperglycaemia and impaired glucose tolerance . we present a case of 35-year - old male with a diagnosis of schizophrenia and type 2 dm with td , who was successfully treated with quetiapine and clonazepam . a 35-year - old male patient who was premorbidly well - adjusted and without past and family history of neurological and psychiatric illness presented with complaints of suspiciousness , fearfulness , decreased sleep , irritability and muttering to self for last 5 years . the onset of illness was insidious and course was fluctuating . from last 6 months his psychotic symptoms and perioral tremors increased in intensity . for his illness he received treatment from a psychiatrist with various antipsychotics like olanzapine 20 mg per day , haloperidol 10 mg per day , trifluperazine 10 mg per day , risperidone 6 mg per day in various combinations from last 3 years . he was also diagnosed with type 2 dm and receiving treatment ( tablet glimepiride 1 mg+metformin 500 mg per day ) from last 2 years . informed consent has been taken from the patient and on mental status examination , he has delusion of persecution , auditory hallucination of commenting type . on physical examination , he was having perioral tremors with involuntary , repetitive , and irregular movements of tongue . other than this no other abnormal finding was observed in physical examination . on positive and negative syndrome scale ( panss ) for schizophrenia15 ) and abnormal involuntary movement scale ( aims ) for td,16 ) the patient scored 102 and 16 respectively . hematological and biochemical indices ( including blood sugar level ) were normal as was computed tomography of the brain and electroencephalogram . risperidone ( 6 mg / day ) , haloperidol ( 10 mg / day ) , trihexyphenidyl ( 4 mg / day ) were tapered to half the previously prescribed dose and stopped completely after a period of 10 days . he was started on tablet quetiapine 100 mg per day and increased to 300 mg per day . along with this clonazepam 1 mg per day his abnormal movements of face and psychotic symptoms improved significantly . his blood sugar levels remained within normal limits during this period of treatment . after 4 months of treatment , on panss for schizophrenia and aims for td the patient scored 54 and 8 , respectively . though clozapine is best supported for management of td in schizophrenia , but in our patient due to presence of type 2 dm it was not prescribed . diabetogenic potential of quetiapine is less than clozapine and olanzapine.17 ) due to its receptor profile , quetiapine is the atypical antipsychotic that is most similar to clozapine , which leads us to consider it for the treatment of td . quetiapine s low affinity and fast dissociation from post - synaptic dopamine d2 receptors should give the least risk of producing the symptoms of td.1820 ) with the use of quetiapine our patient s psychotic symptoms improved significantly over the next 8 weeks . his blood sugar levels were also remained within normal limits . as there are evidences for the use of clonazepam in the treatment of td21 ) and the sleep of our patient was decreased , so clonazepam 1 mg per day was prescribed . td is associated with more severe psychopathology and higher mortality.22 ) antipsychotic treated chronic schizophrenia patients with dm are more likely to develop abnormal movements than are patients with no dm . so we support the use of quetiapine and clonazepam in the treatment of schizophrenia patients with dm and td .

tardive dyskinesia is one of the most significant side effects of antipsychotic medications . antipsychotic treated schizophrenia patients with diabetes mellitus are more likely to develop tardive dyskinesia than those without diabetes . clozapine is probably best supported for management of tardive dyskinesia . but clozapine has been strongly linked to hyperglycaemia and impaired glucose tolerance , so it is not preferred in patients with diabetes mellitus . we present a case of 35-year - old male with a diagnosis of schizophrenia and type 2 diabetes mellitus with tardive dyskinesia , who was successfully treated with quetiapine and clonazepam .

there had been no pain or dysphonia but a bothersome sensation related to progressive surface roughness that prompted her to seek care . she was otherwise healthy ; a small congenital melanocytic nevus affecting the right inner thigh had been excised during early childhood . on examination , there was no palpable cervical lymphadenopathy . a well - demarcated , circular plaque ( 1.21.10.3 cm ) with a slight pebbled texture affected the left posterior hard palate . while predominantly erythematous , there was scattered light brown pigmentation ( figure 1 ) . figure 1initial presentation of a non - homogenous brown - pigmented palatal plaque . initial presentation of a non - homogenous brown - pigmented palatal plaque . the underlying stroma contained sheets of nevus cells ( figure 2 ) exhibiting maturation and extending deep into the lamina propria and around minor salivary ducts ( figure 3 ) , nerves and blood vessels . cells diffusely stained with melana ( figure 5 ) and ki-67 suggested a low proliferative index . only the superficial cells stained with hmb-45 ( figure 6 ) . figure 2papillomatous oral squamous epithelium and sheets of nevus cells extending deep within the lamina propria ( h+e , 4 ) . papillomatous oral squamous epithelium and sheets of nevus cells extending deep within the lamina propria ( h+e , 4 ) . figure 4scant melanin pigmentation was seen only in superficial nevus cells ( h+e , 20 ) . scant melanin pigmentation was seen only in superficial nevus cells ( h+e , 20 ) . figure 5diffuse staining of nevus cells with melana . diffuse staining of nevus cells with melana . melanocytic nevi are separated into acquired and congenital subtypes with the latter identified at birth . the palate is not easily visualized , however , which may explain delayed identification . while it is recognized that parental history may not be accurate , there was certainty of identification during early infancy . the larger size was suggestive of a congenital nevus as acquired nevi are usually less than 6 mm in diameter . congenital nevi may have a papular , pebbly or verrucous surface as well as hypopigmented areas . histologically , a congenital pattern was characterized by a diffuse infiltration of nevus cells extending around blood vessels , nerves , salivary ducts as well as between collagen bundles . nevus cells may exist and proliferation in diverse anatomic locations as evidenced by identification of aggregates in the parenchyma of lymph nodes . oral melanocytic nevi are uncommon , and to the best of these authors knowledge , only three well - documented cases of intraoral congenital melanocytic nevi have been reported in the english literature , ( table 1 ) . other mucosal sites are also uncommonly affected with rare reports of conjunctival or genital congenital nevi . characteristics of reported oral congenital melanocytic nevi classification of congenital melanocytic nevi is by size : small ( < 1.5 cm ) , medium ( 1.5 to 19.9 cm ) and large or giant ( > 20 cm ) . the primary concern is malignant transformation and there is general agreement that the risk increases with the size . while a clear risk exists for larger lesions , such an association is controversial with small congenital nevi . identified histologic features of congenital nevi in 8.1% of melanoma specimens . in another study , a melanoma risk of 2.64.9% was estimated for persons with small congenital nevi and it was concluded that small congenital nevi may represent precursors for at least some cases of cutaneous melanoma . there is general agreement that the larger the congenital nevi the more likely the need for excision . for small congenital nevi , management may be either excision or observation but there exists no clear consensus or guidelines . excision seems reasonable as in most cases one would expect minimal surgical morbidity and excision facilitates comprehensive histologic sampling to exclude melanoma , and presumably , may prevent malignant transformation . from the patient 's perspective , as was the case with this patient failing to attend post - surgical assessment , excision may potentially mitigate the necessity for long - term observation . ultimately , decisions require individualization with consideration of the size , anatomic restrictions as well as patient factors such as co - morbidities and ability to attend observation .

oral pigmented lesions are uncommon and congenital melanocytic nevi are especially rare . we report a case of a patient with multiple congenital melanocytic nevi including a palatal lesion . this is reported to add to the scant literature that exists on this subject . prognosis and management are discussed .