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a review of the literature and an extensive medline search revealed that this is the first case report of the use of guaifenesin to increase sperm motility . he reported an inability to conceive with his wife after 18 months of unprotected intercourse . a semen analysis was performed that included spermatozoa count , liquefaction , morphology , motility , viscosity and volume . two months after guaifenesin therapy the semen analysis was repeated that demonstrated marked improvement in both total sperm count and motility . evidence for the effectiveness of guaifenesin is almost entirely anecdotal . given the mechanism of action of guaifenesin , it is not clear from this case why the patient demonstrated such a large improvement in both sperm count and motility . additional studies of the effects of guaifenesin on male fertility could yield information of the medication s effect on men with normal or decreased total sperm counts . there are currently anecdotal reports and popular news media stories on the use of guaifenesin , particularly the brand name product robitussin ( pfizer , inc . , new york , ny ) , for use in treating both male and female infertility.14 guaifenesin is an expectorant medication sold over the counter and usually taken by mouth to assist expectoration of phlegm from the airways in acute respiratory tract infections . its mode of action in treating infertility is not well understood , but it appears to decrease mucus viscosity . a 32-year - old male patient presented to his primary care provider for an infertility evaluation . the patient is a nonsmoker , who consumes little or no alcohol with no known allergies . a recent screening exam for pulmonary tuberculosis was negative and the patient had recently undergone a required military service physical exam . he reported an inability to conceive with his wife after 18 months of unprotected , regular intercourse . as part of a routine infertility evaluation a semen analysis was performed that included spermatozoa count , liquefaction , morphology , motility , viscosity and volume ( cpt code 89320 ) . initial results of the semen analysis demonstrated low sperm count and motility ( table 1 ) . this sample , as well as the follow - up sample , were obtained through masturbation and provided to the lab within 30 minutes of collection . the patient s primary care provider offered treatment with guaifenesin 600 mg extended release tablets twice daily . the repeat semen analysis demonstrated marked improvement in both total sperm count and motility ( table 1 ) . the patient made no other significant lifestyle changes during the treatment course with guaifenesin . at the time of writing this case report describes the semen analysis laboratory results in a male patient who was given guaifenesin . guaifenesin is a mucolytic agent usually taken orally to assist the expectoration of phlegm from the airways in acute respiratory tract infections . scientific evidence for the effectiveness of guaifenesin is almost entirely anecdotal ; a review of medical literature revealed very limited data on use of guaifenesin for infertility.5,6 there appeared to be some improvement in a small study without controls of female infertility related to hostile cervical mucus.5 check regards guaifenesin as the simplest but least effective method of improving cervical mucus.7 given the proposed mechanism of action of guaifenesin , it is not clear from this case why the patient demonstrated such a large improvement in both sperm count and motility . additional study of the effects of guaifenesin on male fertility suggests the need to conduct a more rigorous placebo - controlled clinical trial that could yield information of the medication s effects on men with normal or decreased total sperm counts .
backgrounda review of the literature and an extensive medline search revealed that this is the first case report of the use of guaifenesin to increase sperm motility.casea 32-year - old male presented for an infertility evaluation . he reported an inability to conceive with his wife after 18 months of unprotected intercourse . a semen analysis was performed that included spermatozoa count , liquefaction , morphology , motility , viscosity and volume . initial results of the semen analysis demonstrated low sperm count and motility . the provider offered treatment with guaifenesin 600 mg extended release tablets twice daily . two months after guaifenesin therapy the semen analysis was repeated that demonstrated marked improvement in both total sperm count and motility.conclusionevidence for the effectiveness of guaifenesin is almost entirely anecdotal . given the mechanism of action of guaifenesin , it is not clear from this case why the patient demonstrated such a large improvement in both sperm count and motility . additional studies of the effects of guaifenesin on male fertility could yield information of the medication s effect on men with normal or decreased total sperm counts .
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a 60-year - old woman ( height , 162 cm ; weight , 61 kg ) visited pain clinic with tactile allodynia and electric shock - like pain in the left dorsal scapular area around the t3 dermatome , which had been diagnosed as phn about 1 month previously and attack of the herpes zoster was 1 year ago . the 100-mm visual analogue scale ( vas ) of allodynia and electric shock - like pain was rated between 70 and 80 mm on a scale from 0 ( no pain ) to 100 ( worst pain imaginable ) . the interlaminar epidural block was performed at the t3 - 4 space by the paramedian approach with 5 ml of 0.2% ropivacaine and 20 mg of triamcinolone acetate . pregabalin and morphine at doses of 150 mg and 10 mg , respectively , twice a day , amitriptyline at a dose of 10 mg before sleep and topical lidocaine patches were prescribed . dosages of all drugs were adjusted depending on the side effects during the follow - up period . epidural blocks were repeated twice with a 1-week interval and the continuous intravenous infusion of ketamine ( 60 mg ) was performed over a period of 1 hour twice a week under careful monitoring . after 1 month elapsed , electric shock - like pain was reduced to a vas score of 30/100 , but allodynia was not diminished ( vas score of 70/100 ) . after 4 months elapsed , we decided to administer magnesium sulfate via the intravenous route . and it was done with continuous intravenous infusion of 1,000 mg of magnesium sulfate in 50 ml of normal saline for 1 hour . before and after infusion , the serum magnesium levels were checked . after magnesium therapy , she felt very good about her pain and the vas of allodynia was reduced to 40 - 50 . at 1-week follow - up , she was very satisfied with the treatment and reported the reduction of allodynia on the dorsal scapular area of up to 50% ( vas 25 - 30/100 ) . however , the serum magnesium level had increased above normal range ( 2.3 meq / l to 2.9 although it was below the serum level reveals of the adverse effect , we decided to stop intravenous infusion of magnesium sulfate . for more accurate and safe delivery of magnesium to the target site , we applied magnesium using the transforaminal epidural injection technique . with the patient 's informed consent , left t3 tfemi identification of the t3 nerve root sheath and epidural space was performed using contrast media ( fig . 1 ) . then , 100 mg of magnesium sulphate and 1 ml of 0.2% ropivacaine ( total volume , 2 ml ) was carefully injected . tfemi was repeated twice with a 1-week interval ( total of three times ) and the degree of pain decreased gradually during the follow - up period . one week after the last procedure , the vas score of allodynia decreased to 15/100 and all medications except pregabalin were discontinued . the vas was 10/100 throughout 1-month follow - up , and pregabalin had also been tapered . to our knowledge , no previous report has described about the magnesium administration by the transforaminal epidural route in patients with neuropathic pain . here , in our report of phn patient , this treatment resulted in effective pain relief . previous studies have demonstrated the anti - allodynic effects of nmda receptor antagonists in neuropathic pain disorders . among the currently available nmda receptor antagonists , ketamine is the most widely used one for the treatment of neuropathic pain . however , ketamine is not always effective and psychomimetic side effects are frequent . magnesium can antagonize nmda receptor channels by blocking calcium influx in a voltage - gated manner . intravenous administration of magnesium is efficacious in the management of various conditions associated with neuropathic pain , including phn . demirkaya and colleagues revealed 1 g i.v . mg sulfate is effective in the treatment of migraine attacks and collins and colleagues reported that 70 mg / kg magnesium sulphate infusions in 4 hours for 5 days reduced pain in patients with complex regional pain syndrome . whether intravenous administration of magnesium can achieve a sufficient concentration in the cerebrospinal fluid to block nmda receptors is unclear and studies have reported on the limited efficacy of magnesium when administered via the intravenous route . furthermore , even if the dose of intravenously administered magnesium is not sufficient to present toxicity , patients are still at risk of magnesium overdose . neuraxial administration of magnesium is an " off - label " use , and the safety of this technique in human subjects is still undetermined . however , animal studies showed that intrathecally administered magnesium was free of neurotoxicity , and recent studies have demonstrated the safety of magnesium administration via the epidural or intrathecal route in humans . in fact , the exact site of action of epidurally administered magnesium ( i.e. , spinal or supraspinal ) remains unclear . however , comparison with previous reports regarding intravenous magnesium administration suggested that the low dose epidural magnesium used in our patient was unlikely to result in systemic effects . in conclusion , tfemi showed a favourable result in the treatment of intractable allodynia associated with phn . this study was performed in only a single case , and further investigations are required to determine the efficacy of tfemi in the management of allodynia in patients with phn .
although postherpetic neuralgia ( phn ) is a common chronic pain syndrome , the pathophysiology of this disorder is not well known and management is often very difficult . n - methyl - d - aspartate ( nmda ) receptor antagonists are known to be effective in phn , and magnesium , a physiological blocker of nmda receptors , is widely used to treat various chronic pain disorders . here , we present a case of the phn refractory to conventional treatment , which was treated successfully with transforaminal epidural injection of magnesium sulphate at the affected dermatome .
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the following section describes the treatment procedure for patients with deep infection following tka using modified static spacers . the original prosthesis was removed , followed by intensive irrigation and wide debridement of the infected soft tissue . a 36 fr - diameter straight thoracic catheter ( mallinckrodt medical , athlone , ireland ) and a steinmann pin measuring 3.0 mm in diameter and 22 cm in length vancomycin ( 2 g ) was then added to the gentamicin bone cement ( depuy , warsaw , in , usa ) . at the late liquid stage of the cement , the steinmann pin was inserted into the tube , and the prepared cement was delivered through the tube . during this procedure , the tube was removed from the cement rod using a surgical knife . finally , a cement rod measuring 9 mm in diameter and 22 cm in length was formed ( fig . an entry hole was created at the center of the distal femur and proximal tibia for the insertion of the cement rod . the rod was then inserted into the femur and tibia through this hole . during insertion , it was important to place the center of the cement rod on an imaginary joint line . the proximal medullary canal of the tibia was filled with antibiotic - impregnated cement up to the surface of the proximal tibia , while the surgical assistant maintained proper anatomic alignment and joint space . the space between the cement of the distal femur and the proximal tibia was filled with more antibiotic - impregnated cement . finally , the suprapatellar pouch and medial and lateral gutter space were filled with antibiotic - impregnated cement to reduce soft tissue adhesion ( fig . cylinder splint immobilization was required for three days after the operation , after which a fixed - angle knee brace was used and toe - touching ambulation was allowed until the reimplantation surgery . from april to november 2007 , the authors performed the static technique in four patients using the novel antibiotic - impregnated cement rod for the treatment of infected tka ( culture : staphylococcus in three cases and no bacteria in one case ) . follow - up laboratory studies , including erythrocyte sedimentation rate and c - reactive protein , culture study via knee aspiration and a frozen biopsy from the second - stage operation ( < 5 polymorpho - leukocytes / high power field ) were performed to confirm successful eradication of the infection . the second - stage reimplantation was performed when all the criteria for the validation of infection control were met ( fig . the second - stage reimplantation was performed using the rectus snipping approach , and more than 90 of flexion was obtained intra - operatively . in all four patients , no re - infection was evident after two and a half years of follow - up . the range of motion of the knee joints were respectively improved from 50 to 80 , 95 , and 100 , and the knee society scores were 70 , 86 , 65 , and 84 , respectively , in the last follow - up evaluation . fehring et al.8 ) emphasized the importance of resting the joint in septic joint conditions . others also reported that the static spacer technique provides more stability than the mobile spacer technique in patients with severe bone loss.5,8 ) the main advantage of this technique is the maintenance of a normally aligned lower limb during the interval period . this maintains knee stability in combination with early muscle strengthening exercises , including quadriceps setting exercise , and enables the patient to comfortably dress and manage him / herself during the intervening period . the cement rod and static spacer provide a stable gap between the femur and tibia , thereby minimizing soft tissue contracture and shortening of the lower limb . in addition , symmetric maintenance of the soft tissue of both medial and lateral gutters requires no additional soft tissue balancing at the second - stage reimplantation . the additional cost of a metal nail , however , and the difficulty in infection control due to biofilm formation around the metal nail can be troublesome . in addition , a metal nail can not be removed easily at the second stage reimplantation due to its hardness , and soft tissue adhesion of the femoral or tibial medullary canals . on the other hand , an antibiotic - impregnated cement rod enjoys advantages , such as inexpensive antibiotic delivery to the marrow spaces and easy removal by using a hercules cutter . in addition , a static spacer is anchored to the cement rod , which can prevent spacer migration and bone erosion . this feature is believed to generate less cement wear debris than with the conventional static spacer or mobile articulating spacer technique . during the conduct of the antibiotic - impregnated cement rod technique , antibiotic - impregnated cement was applied to the proximal tibia , distal femur , joint gap space , suprapatellar pouch , and both gutters in a stepwise manner . this technique enables easy removal of the cement and reduced soft tissue adhesion at second - stage reimplantation .
the two - stage exchange arthroplasty ( one- or two - stage ) is believed to be the gold standard for the management of infections following total knee arthroplasty . we herein report a novel two - stage exchange arthroplasty technique using an antibiotic - impregnated cement intramedullary nail , which can be easily prepared during surgery using a straight thoracic tube and a steinmann pin , and may provide additional stability to the knee to maintain normal mechanical axis . in addition , there is less pain between the period of prosthesis removal and subsequent reimplantation . less soft tissue contracture , less scar adhesion , easy removal of the cement intramedullary nail , and successful infection control are the advantages of this technique .
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ankle dorsiflexion passive range of motion ( df prom ) measurements are performed in the field of physical therapy to estimate ankle motion during functional activities1 and to prevent lower extremity injuries2 . although in the clinical setting , ankle df prom is frequently measured under non - weight - bearing ( non - wb ) conditions1 , 3 , 4 , many researchers have stated that the wb position is more appropriate for estimating the amount of ankle df motion during functional activities5 , 6 . therefore , wb ankle df prom should be measured during interventions focused on increasing ankle df prom . limited ankle df prom with knee extended may result from gastrocnemius tightness and insufficient posterior talar glide7 . thus , gastrocnemius stretching and talocrural joint mobilization have been performed as intervention strategies to increase ankle df prom3 , 8 , 9 . previous studies have reported a significant increase in ankle df prom after these interventions3 , 8 , 9 ; however , to our knowledge , no study has demonstrated the combined effect of both interventions on wb ankle df prom . therefore , the aim of the present study was to examine the influence of gastrocnemius stretching combined with joint mobilization on wb ankle df prom . in total , 11 male subjects with bilateral limited non - wb ankle df prom with knee extended ( mean age , 22.82 3.09 years ; mean height , 175.91 3.39 cm ; mean weight , 69.55 3.78 kg ; mean non - wb ankle df prom , 4.17 2.48 ) participated in this study . inclusion criteria were 1 ) ankle df prom with knee extension < 10 ; 2 ) ankle df prom with knee flexion > 10 ; and 3 ) > 5 difference in ankle df prom between knee extension and knee flexion conditions on bilateral sides in non - wb positions3 . subjects with a history of surgery on the lower extremity , fracture , or neurological diseases were excluded from this study . all participants signed an informed consent form approved by the institutional research review committee of inje university prior to participation in this study . the study protocol of this study complies with the ethical standard of the declaration of helsinki . wb ankle df prom with knee extended was measured following the procedures suggested by munteanu et al10 . subjects stood in front of a wall and placed the leg being tested behind the contralateral leg in a lunge posture . subjects were asked to place both hands on the wall and then lean forward without heel - off and knee flexion until maximum stretch was felt in the gastrocnemius on the tested leg . the force applied to the tested leg was maintained at 60 5% of the subject s weight using scales11 . an examiner determined the maximum tibial inclination using an inclinometer to measure the wb ankle df prom with knee extended . measurements of wb ankle df prom were repeated 3 times for each ankle under pre- and post - intervention conditions . the mean value of 3 trials was used for data analysis . for gastrocnemius stretching combined with joint mobilization , subjects leaned forward against the wall in the same lunge posture as that during measurement of wb ankle df prom with knee extended until the maximum gastrocnemius stretch was felt . subjects held the end - range posture while an examiner provided the talus of the tested leg with sustained anterior - to - posterior gliding force . an intervention trial was performed for 30 s , and 10 trials were repeated with 30-s rest periods for each ankle . the difference in wb ankle df prom with knee extended between pre- and post - intervention conditions was analyzed using a paired t - test . wb ankle df prom with knee extended was significantly increased in post - intervention compared with pre - intervention conditions ( 42.60 5.49 versus 38.24 4.69 , p < 0.001 ) . our findings demonstrate that gastrocnemius stretching combined with joint mobilization significantly increases wb ankle df prom with knee extended . stretching exercises increase tolerance , resulting in increased rom12 . additionally , increased displacement of the myotendinous junction ( mtj ) after gastrocnemius stretching for 5 min was found in a previous study13 . therefore , the change in tolerance and/or increase in mtj displacement might have influenced our findings . the addition of talocrural joint mobilization to gastrocnemius stretching is another possible explanation for our findings . previous research by dinh et al.3 showed a 4.25 increase in wb ankle df prom with knee extended after gastrocnemius stretching alone for 3 weeks . although gastrocnemius stretching combined with joint mobilization was applied for 5 min in the present study , the amount of increase in wb ankle df prom after intervention ( i.e. , 4.36 ) was similar to that found previously . considering this outcome , despite the relatively short period of intervention in the present study , it may be inferred that the addition of talocrural joint mobilization might maximize the effects of general gastrocnemius stretching . thus , we conclude that gastrocnemius stretching combined with joint mobilization might decrease gastrocnemius tightness and increase posterior talar gliding movement , which effectively increases wb ankle df prom with the knee extended . first , although non - wb ankle df prom was used as an inclusion criterion , changes in non - wb ankle df prom after intervention were not measured . however , we believe that wb ankle df prom is clinically more important because most functional activities are performed under the wb condition . second , our study included only male subjects , and the results can not be generalized to women .
[ purpose ] the purpose of this study was to investigate the effect of gastrocnemius stretching combined with talocrural joint mobilization on weight - bearing ankle dorsiflexion passive range of motion . [ subjects ] eleven male subjects with bilateral limited ankle dorsiflexion passive range of motion with knee extended participated in this study . [ methods ] all subjects received talocrural joint mobilization while performing gastrocnemius stretching . ankle dorsiflexion passive range of motion was measured using an inclinometer under weight - bearing conditions before and immediately after intervention . a paired t - test was used to analyze the difference between weight - bearing ankle dorsiflexion passive range of motion pre- and post - intervention . [ results ] a significant increase in weight - bearing ankle dorsiflexion passive range of motion was found post - intervention compared with pre - intervention . [ conclusion ] these findings demonstrate that gastrocnemius stretching combined with joint mobilization is effective for increasing weight - bearing ankle dorsiflexion passive range of motion .
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nathan , as an oncology fellow , knew well that white blood cells fought infections . this was an experiment repeated in front of us all the time , he says : chemotherapy lowered his patients ' white blood cells and increased their risk of infections . mackaness had shown that macrophage activation did not depend on direct contact with t cells ( 1 ) , suggesting the possibility of a secreted factor . when nathan tested the supernatant from activated t cells , he saw that it did indeed induce macrophage activation ( 2 ) . nathan got a rough idea of the molecular weight ( 3 ) , but that was the best anyone could do , he says . protein separation methods were primitive , and cloned proteins and monoclonal antibodies would only become available a decade later . henry murray , one of nathan 's collaborators , sums up the feeling of frustration : we were all nibbling at the edges of the same problem . nathan therefore changed tack to take a closer look at the activated macrophages . short - lived neutrophils were known to produce hydrogen peroxide , and nathan found the same was true of longer - lived activated macrophages ( 4 ) . unlike previous signs of macrophage activation increased spreading , phagocytosis , and glucose metabolism this so - called respiratory burst ifn had been on the cover of time magazine , and recombinant murine ifn was found to induce macrophages to kill tumor cells ( 5 ) . nathan , now a faculty member in zanvil cohn 's macrophage factory at rockefeller university ( new york , ny ) , thought ifn might also activate macrophages to kill intracellular parasites . consistent with this idea , ifn was made by antigen - stimulated t cells and was associated with defense from infection . now the respiratory burst gave nathan an assay , berish rubin ( down the street at the new york blood center ) supplied an ifn monoclonal antibody , and a phone call to genentech yielded recombinant ifn. in a seminal paper published in the journal of experimental medicine in 1983 , nathan was thus able to show that depleting ifn from unpurified t cell supernatants decreased the respiratory burst activity and the killing of intracellular protozoa in human macrophages . adding back recombinant ifn into this mix restored macrophage activation ( 6 ) . i had an assay , a hunch , a history of purifying proteins that did this , and the serendipity of meeting with people nearby who had the antibody . nathan next showed that ifn worked in people . injecting recombinant ifn directly into cutaneous lesions of lepromatous leprosy patients induced macrophage infiltration , hydrogen peroxide production , and killing of the causative pathogen , mycobacterium leprae ( 7 ) . in the 1990s , the macrophages of children with ifn receptor deficiencies were shown to be defective in killing mycobacteria ( 8) . tracing the pathway from t cells to macrophages to bacteria started , for nathan , in 1967 , and he says we still haven't finished making the molecular links .
t cells tell macrophages when to start making the toxic soup of lysosomal enzymes , reactive oxygen species , and nitric oxide that destroys intracellular pathogens . in 1983 , carl nathan proved that this start signal comes in the form of the secreted cytokine ifn.
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a glomangioma is a benign vascular tumor derived from the glomus body , a specialized neuromyoarterial structure involved in thermal regulation . it is a subtype of the more generalized category of glomus tumors and should not be confused with the head and neck paragangliomas , such as the glomus tympanicum or glomus jugulare . it should also be distinguished from the glomangiopericytoma , the sinonasal type hemangiopericytoma , which displays different ultrastructural and histochemical characteristics.1 glomangiomas are most commonly found subungually and are exceedingly rare in the head and neck.2 they account for only 0.6% of all nonepithelial tumors of the nasal cavity , nasopharynx , and paranasal sinuses.3 glomangiomas that induce paraneoplastic osteomalacia are even more uncommon , with only one case reported to date.4 while there are several documented cases of oncogenic osteomalacia ( oo ) caused by glomangiopericytomas,5 we present the second reported case of glomangioma - induced osteomalacia and the first case documented in english . a 42-year - old man with a history of unexplained hip , rib , scapula , and metatarsal fractures ; left foot pain with exercise ; and weakness presented to our clinic for evaluation of a paranasal sinus tumor . the endocrinology service initially diagnosed him with tumor - induced osteomalacia caused by fibroblast growth factor 23 ( fgf23 ) , which prompted an octreotide scan revealing a questionable area of enhancement in the pituitary . a subsequent magnetic resonance imaging ( mri ) showed an ethmoid mass extending through the cribriform intracranially , measuring 3.9 1.9 2.4 cm at its largest dimensions ( fig . the patient reported occasional sinus congestion , decreased sense of smell during the preceding 2 to 3 years and occasional blurry vision in the morning . his laboratory workup revealed a significantly decreased 1,25-vitamin d level of less than 8 ng / ml , a decreased phosphate level to 1.6 mg / dl , a low to normal calcium level of 8.7 mg / dl , and a significantly elevated alkaline phosphatase level at 65.3 g / l . preoperative mri , t1 with contrast , showing an ethmoid mass extending through the cribriform intracranially . the patient underwent a combined endoscopic endonasal approach to the anterior skull base with tumor resection . the cribriform defect and intraoperative cerebrospinal fluid ( csf ) leak pathology revealed a vascular neoplasm with a uniform cluster of ovoid cells arranged around the vessels and moderate focal nuclear enlargement ( fig . tumor cells stained positive for cd31 and smooth muscle actin ( sma ) but negative for cd34 , s100 , and pan - cytokeratin on immunohistochemistry ( fig . the patient reported near - complete resolution of bone pain and improvement in smell , and he had normalization of phosphate , alkaline phosphatase , vitamin d , and other laboratory values . repeat mri showed gross total resection of the mass and no detectable recurrence ( fig . 4 ) . postoperative mri , t1 with contrast , showing gross total resection of the mass with nasoseptal flap reconstruction of the skull base . osteomalacia is a disease of the bone characterized by defective mineralization of osteoid from decreased levels of available phosphate and calcium or increased bone resorption . it often presents with diffuse joint and bone pain , easy fracturing , difficulty walking , weakness , and other nonspecific symptoms . oo is a rare , disabling , and curable form of osteomalacia that affects both sexes equally and usually presents around 40 years of age.6 it is not well described in glomangiomas but has been detailed several times in relation to glomangiopericytomas and other soft tissue and bone tumors , with more than 300 reported cases7 since its debut in 1947.8 it predominantly occurs in the context of mesenchymal tumors and is thought to be due to neoplastic overexpression of fgf23 . this protein inactivates the sodium - phosphate pump in the proximal tubule ( prohibiting phosphate reabsorption and inducing renal phosphate wasting ) and reduces 1-hydroxylation of 25-hydroxy vitamin d.9 accordingly , common oo laboratory abnormalities include hypophosphatemia , normal or decreased calcium , decreased 1,25-dihydroxy vitamin d3 with resistance to vitamin d supplementation , and elevated alkaline phosphatase , which our patient exhibited . tumors causing oo tend to be small , occult , and slow growing , making diagnosis remarkably difficult . when other causes of osteomalacia have been ruled out and oo is suspected , clinicians should consider measuring a serum fgf23 level . elevated fgf23 in this setting should then prompt full body imaging ( including hands and feet ) to expose the lesion . the current imaging standard for investigation of oo is whole - body mri short tau inversion recovery ( stir).10 another commonly used modality is octreotide scintigraphy ( octreoscan ) , which utilizes a radiotracer that binds to somatostatin receptors overexpressed on the causative tumor.11 once the neoplasm is localized , surgical resection with wide margins is the definitive treatment . it is curative in essentially all cases and typically leads to rapid normalization of laboratory values and reversal of most clinical symptoms . the nonspecific presentation of osteomalacia , the obscure nature of the tumors that cause oo , and the rarity of glomangiomas in the head and neck make this patient 's diagnosis challenging . we believe this to be the second reported case of paranasal sinus glomangioma - induced osteomalacia and the first reported in english . many head and neck surgeons may not be aware of its existence or consider it in their differential diagnoses of sinus tumors . this report serves to increase the awareness of this uncommon pathology so that it may be considered and treated in future cases .
oncogenic osteomalacia ( oo ) is an uncommon but treatable cause of osteomalacia related to tumor production of fgf23 , usually caused by benign mesenchymal neoplasms . paranasal sinus glomangiomas are a rare cause of oo , with only one previously reported case . here we describe a second case ( first reported in english ) of paranasal sinus glomangioma - induced osteomalacia in a 42-year - old man . he presented with weakness and multiple spontaneous fractures , and was found to have an ethmoid sinus glomangioma with intracranial extension . the tumor was removed via endoscopic endonasal approach to the anterior skull base , which resulted in complete resolution of symptoms and no further evidence of disease 1 year postoperatively .
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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 .
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a 55 year - old man visited our emergency department because of increasing frequency of chest pain . he had undergone off - pump coronary artery bypass grafting ( cabg ) 10 years ago because of unstable angina associated with three vessel coronary artery disease . at the initial operation , the in situ right internal thoracic artery ( ita ) , in situ left ita and in situ right gastroepiploic artery ( rgea ) grafts were used to revascularize the left anterior descending coronary artery , two obtuse marginal coronary branches , and posterior descending coronary artery , respectively . an excess segment of the distal right ita was connected to the side of left ita as a y - composite graft and anastomosed to the first diagonal coronary artery . coronary angiography and myocardial single photon emission computed tomography ( spect ) were performed at 5 years after surgery as a follow - up study . the 5-year angiography showed all patent grafts and the myocardial spect demonstrated no perfusion decrease . exertional chest pain recurred at 7 years after surgery , and a repeated coronary angiography showed patent previous grafts including faint visualization of the in situ rgea graft associated with significant stenosis at the os of the celiac axis . the computed tomographic angiogram also demonstrated a 90% stenosis at the celiac os , which had been without stenosis on abdominal angiography taken before the surgery ( fig . redo off - pump cabg was performed 10 years after the initial surgery because of an increasing frequency of angina and an aggravated finding of the follow - up myocardial spect , which was a newly developed reversible perfusion decrease in the inferior wall ( fig . , the great saphenous vein was harvested from the lower leg and interposed between the middle part of in situ right ita and distal part of in situ rgea grafts used previously , to supply blood flow from the right ita graft to the posterior descending coronary artery . one year after redo surgery , the patient had no symptoms of angina and coronary angiogram was performed and revealed patent grafts , including an interposed saphenous vein graft ( fig . 3a ) . the myocardial spect test was also performed and demonstrated that there was no perfusion decrease including the inferior wall ( fig . reoperations for coronary artery disease have been increased due to the increased number of isolated cabg . the society of thoracic surgeons statistics indicated that nearly 5% of the current cabg procedures done in the us were repeat surgical revascularization . angiographic indications for reoperation are progression of native coronary atherosclerosis , previous graft failure or a combination of both . one previous study demonstrated that 4 out of 400 patients who underwent cabg using the rgea graft needed percutaneous interventions due to the rgea graft failure during postoperative follow - up of 2211 months . one of those 4 patients required an angioplasty for a newly developed stenosis of the celiac trunk . in the present case , an indication for reoperation the patient had been free of angina , and the angiographic and myocardial spect follow - up studies revealed no abnormal findings at postoperative 5 years . when the patient suffered from recurred angina at postoperative 7 years , coronary angiography showed a faint visualization of the in situ rgea graft associated with significant stenosis at the os of the celiac axis . the 10-year follow - up myocardial spect test demonstrated a newly developed reversible perfusion decrease in the inferior wall . the prevalence of celiac axis stenosis was 7.3% in a korean population although it was lower than the previously reported incidence of celiac axis stenosis in western populations ranged from 12.5% to 24% . in the present case , celiac artery stenting could be an alternative option in such a case . however , we performed a redo operation because celiac axis stenting was associated with a high incidence of late restenosis . the aorta or another in situ arterial graft could be chosen as a blood source . alternatively , patent in situ grafts used previously may be re - used as an inflow conduit . with regards to our patient , the 3 in situ arterial grafts had already been used . the saphenous vein graft was interposed between the middle part of right ita and distal part of in situ rgea grafts used previously .
we report a redo coronary artery bypass grafting ( cabg ) in a 55-year - old man . angina recurred 7 years after the initial surgery . coronary angiography showed all patent grafts except a faint visualization of the in situ right gastroepiploic artery ( rgea ) graft , which was anastomosed to the posterior descending coronary artery , associated with celiac axis stenosis . redo - cabg was performed at postoperative 10 years because of aggravated angina and decreased perfusion of the inferior wall in the myocardial single photon emission computed tomography . the saphenous vein graft was interposed between the 2 in situ grafts used previously ; the right internal thoracic artery and rgea grafts . angina was relieved and myocardial perfusion was improved .
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optimal treatment of cancers in children often requires combined - modality therapy , including : chemotherapy , surgery , and/or radiotherapy . chemotherapy is not always sufficient to achieve the cure of solid tumors in children ; either resection or radiation may be needed for local tumor control as well ( 1 ) . children with radiosensitive malignant tumors typically require radiation therapy for a number of sessions over a period of several weeks . although the procedure is painless , young children need to be sedated or anesthetised in order to provide a motionless state during the procedure . a short period of sedation , analgesia or general anaesthesia the patient and anaesthesia equipment are observed continuously by closed - circuit television , and monitors are mirrored to the remote observation site outside the treatment room . different anesthesia methods and anesthetics have been recommended to provide a safe and optimal situation of motionless with a short recovery period in children undergoing general anaesthesia or sedation for external beam irradiation ( 37 ) . this report describes the accidental detection of a missed complication by the anesthetist at the time of radiotherapy , from the previous chemotherapy , which was preliminarily and unduly attributed to anesthesia . a 2.5 year - old , 13-kg boy , asa ( american society of anesthesiologists ) class ii , with acute lymphoblastic leukaemia ( all ) , was scheduled for radiotherapy . anesthesia was planned by the anesthetist , and the physical examination was normal before radiotherapy . routine monitoring was established , and for peripheral pulse monitoring , the distal portion of the right upper limb of the child was rested out of the covers . after about 5 minutes , when the anesthetic effect of ketamine began , we noticed a lack of right radial pulse . anesthesia - related hemodynamic instability was expected , however no other manifestation of hypoxia or hypo - perfusion was detected . furthermore , carotid pulsation was normal , and examination of the left radial pulse and other peripheral pulses showed normal pulsation . the patient showed a scar and swelling on the right antecubital area . a more detailed history taken from the child s parents showed his history of chemotherapy during which extravasation of the chemotherapy drug had resulted in severe inflammation and edema at the site of injection . a colour doppler ultrasound of the antecubital area showed deep edema and chronic compression on the antecubital tissue and confirmed the diagnosis . permission was obtained from the patient s parents to use the patient s reports , however the patient s information was to remain confidential . the incidence range of extravasations of cytostatic drugs in cancer patients has been reported from 0.2 to 1.4% in a five - year study ( 8) . extravasations in cytostatic treatment may cause a wide range of symptoms , from patients discomfort to severe complications such as necrosis and amputation . ( 9 ) eccrine squamous syringometaplasia , while rare , has also occurred in patients who have received chemotherapy treatment ( 10 ) . yeung et al described a case of metastatic ovarian carcinoma with repeated thrombosis of the femoral arteries following intravenous carboplatin - based combination chemotherapy . persistent withdrawal occlusion ( pwo ) is frequently caused by fibrin sheath formation around venous access devices . small doses of thrombolytic drugs ( such as urokinase ) could manage pwo , but could also serious complicate chemotherapy drug extravasation ( 12 ) . keratolytic ointment was applied for old lesions , whereas in new lesions , multiple subcutaneous injections of hydrocortisone solution were used before the application of betamethasone ointment . application of conservative agents in radiotherapy - induced extravasation areas may avoid tissue necrosis and consequently , reconstructive surgery ( 13 ) . management of cytotoxic drug extravasation in humans is based on the experimental evidences and available case reports because of lack of randomized trials . for instance , topical dimethylsulfoxide ( dmso ) and cooling for extravasation of anthracyclines or mitomycin , local injection of hyaluronidase for extravasation of vinca alkaloids , and local injection of sodium thiosulfate ( sodium hyposulfite ) for extravasation of chlormethine ( mechlorethamine ; mustine ) should be empirically recommended . in the case of failed conservative treatment , history and physical examination before injection of anesthetic agents could be useful in preventing patient mismanagement . a weak pulse in a child can be a significant problem for the anesthesiologist and in this case , it was shown to be a critically misleading factor . increased emphasis is on clinical evaluation and pulse checking is necessary , especially in children with a history of chemotherapy . providing thorough pulse evaluation when we work on children and avoiding immediate aggressive intervention before being certain about the cause of weak pulse are additional issues of importance shown by this case report .
treatment of cancer in children often requires a combination of chemotherapy , surgery , and/or radiotherapy . radiotherapy and chemotherapy are not painful processes , but children undergoing these procedures must be made motionless through anesthesia or sedation . there are a few reports of complications during these procedures in relation to the procedures themselves or to the anesthesia given . this report describes an unexpected pulseless radial artery which was preliminarily and unduly attributed to anesthesia . a 2.5 year - old male pediatric patient with an acute lymphoblastic leukaemia was scheduled for radiotherapy . anesthesia with intramuscular ketamine was induced before starting radiotherapy . about 5 minutes after injection of ketamine we found the right radial pulse undetectable . there was no other manifestation of hypoxia or hypo - perfusion . carotid pulsation was normal . examination of the left radial pulse and other peripheral pulses showed normal pulsation . the procedure was continued uneventfully . the next follow - up after radiotherapy , showed a scar and swelling on the right antecubital area , caused by extravasation of chemotherapeutic agent in the prior period of chemotherapy . doppler ultrasonography of the antecubital vein confirmed the diagnosis . this case study therefore demonstrates that proper intravenous cannula establishment before chemotherapy is of great importance . furthermore , accurate history and physical examination before induction of anesthesia or sedation may be useful in preventing mismanagement in pediatric cancer procedures .
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care coordination is an important aspect of nursing care especially for elderly patients admitted to an acute care setting . in singapore care coordination and transitional care nursing is a new concept of care nevertheless important but unexplored . the objective of this paper is to explore the characteristics of elderly patients receiving care coordination , determine care gaps and intervention during home visit and telephonic review . a designed questionnaire was used to collect information on the patient s demography , social and clinical profile and determine post discharge activities using eric coleman s four pillars tool . the retrospective data from the patient s index admission from the last six months ( nov 08april 09 ) was analyzed using spss version 16 . majority , 69% were above 70 years old of which 57% female and 76% lives with their children . clinical information demonstrates that 53% had 36 co - morbidities and 58% were taking more than five medications . the abbreviated mental test score were 6.2 , 6% were depressed and delirium was present in 14% of patients . only 65 patients ( 0.1% ) had home visits and telephonic review done whilst 97% of the remaining had only telephonic review done . those who had both telephonic and home visit review , medications advice and compliance were checked only in 0.8% ( at one week ) and 1.6% ( at one month ) whilst during home visit this was done in 12.2% of patients as medication discrepancy were apparent at home . as for appointment compliance and compilation were done in 0.8% at one week and 51% at four weeks of telephonic review compared to during home visit only 4.8% . caregivers education was emphasized in 14% of patients at home visit , 2% at one week and 4% at one month of telephonic review . the result showed that home visit is effective in exploring medication compliance , advice and emphasizing caregiver education , managing appointments can be effectively done through telephone review . this study demonstrates the vital role of home visit for elderly patient to safely transit between hospitals to home .
introductioncare coordination is an important aspect of nursing care especially for elderly patients admitted to an acute care setting . in singapore care coordination and transitional care nursing is a new concept of care nevertheless important but unexplored.aim/objectivesthe objective of this paper is to explore the characteristics of elderly patients receiving care coordination , determine care gaps and intervention during home visit and telephonic review.research design and samplinga designed questionnaire was used to collect information on the patient s demography , social and clinical profile and determine post discharge activities using eric coleman s four pillars tool . a pilot study of ten questionnaires was conducted . the retrospective data from the patient s index admission from the last six months ( nov 08april 09 ) was analyzed using spss version 16.resulttotal of 517 patients were recruited from october 2008 to march 2009 . majority , 69% were above 70 years old of which 57% female and 76% lives with their children . clinical information demonstrates that 53% had 36 co - morbidities and 58% were taking more than five medications . the abbreviated mental test score were 6.2 , 6% were depressed and delirium was present in 14% of patients.only 65 patients ( 0.1% ) had home visits and telephonic review done whilst 97% of the remaining had only telephonic review done . those who had both telephonic and home visit review , medications advice and compliance were checked only in 0.8% ( at one week ) and 1.6% ( at one month ) whilst during home visit this was done in 12.2% of patients as medication discrepancy were apparent at home . as for appointment compliance and compilation were done in 0.8% at one week and 51% at four weeks of telephonic review compared to during home visit only 4.8% . caregivers education was emphasized in 14% of patients at home visit , 2% at one week and 4% at one month of telephonic review.discussionthe result showed that home visit is effective in exploring medication compliance , advice and emphasizing caregiver education , managing appointments can be effectively done through telephone review.conclusionthis study demonstrates the vital role of home visit for elderly patient to safely transit between hospitals to home .
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a 36-year - old woman complained of an insidious onset of generalized myoclonus that first became apparent at age 27 years . she had no perinatal problems and her development was normal in childhood and juvenile periods . adult - onset myoclonus had worsened progressively from right hand to four extremities , tremulous voice and gait disturbance developed after 3 years from disease onset , and she could not continue working as a nurse . she had no history of febrile convulsions or seizure , infectious disease in the central nervous system , exposure to toxic materials , or intake of herbal drugs . her younger brother aged 40 years old also had progressive generalized myoclonus , which was detected 6 years ago at age 34 years ( figure 1 ) . the patient was alert and oriented , and her mini - mental state examination score was 30 . she did not have gaze palsy , and her vision and hearing were normal ; however , her voice was tremulous and generalized positive myoclonus was observed at four extremities and body . negative myoclonus , dystonia , tremor , and rigidity were not detected , and both motor and sensory functions were intact . there was no evidence of cerebellar dysfunction , and she did not have an ataxic or parkinsonian gait , although she staggered slightly because of myoclonus . examination of the eyes revealed cherry - red spots ( figure 2 ) , but her electroencephalogram was normal . no white matter lesion or cerebellar atrophy was detected in an mri of her brain ( figure 3 ) . neuraminidase , hexosaminidase a , and -galactosidase activities in the leukocytes and cultured fibroblasts in patient and younger brother were normal . the cherry - red spot is a pale perifoveal ring that develops when large deposits of lipid , sphingolipid , or oligosaccharide material accumulate in the ganglionic cells at the macula.2 this is a characteristic finding in storage diseases , including the sialidoses , gm1 and gm2 gangliosidoses , neuronal ceroid lipofuscinosis , niemann - pick disease ( groups a through d ) , farber s lipogranulomatosis , and metachromatic leukodystrophy . interestingly , niemann - pick disease , farber s lipogranulomatosis , and metachromatic leukodystrophy are not associated with myoclonus . moreover , the patient in this report did not have typical findings of these 3 diseases such as the organomegaly , cognitive impairment , and gaze palsy seen in niemann - pick disease4 ; the hoarseness , arthritis , and subcutaneous nodules seen in farber s lipogranulomatosis ; or the abnormal brain mri findings in metachromatic leukodystrophy . in ceroid lipofuscinosis , sialidosis , gm1 and gm2 gangliosidoses , myoclonus , and maculopathy ( e.g. a cherry - red spot ) may coexist . although they are quite similar in appearance , the macular abnormality seen in patients with neuronal ceroid lipofuscinosis ( which has been described as bulls - eye maculopathy ) can be distinguished from the cherry - red spot by color and shape , as well as by the decreased visual acuity and visual - field restriction that are common in neuronal ceroid lipofuscinosis.5 moreover , adult - onset lipofuscinosis has an autosomal dominant inheritance , rather than the autosomal recessive pattern seen in this patient.6 gm1 gangliosidosis results from a deficiency of -galatosidase ; the adult form ( type 3 ) presents as a slowly progressive dementia with prominent parkinsonian features and extra - pyramidal dysfunction , particularly dystonia.7 gm2 gangliosidosis results from a deficiency of hexosaminidase a ; the late form ( with an onset during adolescence and young adulthood ) may be characterized by cognitive dysfunction , cerebellar dysfunction , upper and lower motor neuron involvement , and extrapyramidal dysfunction.8 because -galatosidase and hexosaminidase activity was normal in this patient , because she has a normal level of intelligence , and because no other prominent pyramidal or extrapyramidal dysfunction was detected , we might not diagnose this patient as gm1 and gm2 gangliosidoses . sialidosis is an inherited , autosomal recessive disease associated with a neuraminidase deficiency.9 it has 2 major clinical manifestations : type i ( late , adult onset ) and type ii ( early , infantile onset ) . type i sialidosis is typically found in patients aged 8 to 25 years and is characterized by cherry - red spot myoclonus , seizure , neuropathy , corneal clouding , and difficulty walking , but with normal vision and intelligence ( obrien , 1978 ) . type ii sialidosis is characterized by dysmorphism , myoclonus , mental retardation , ocular cherry - red spots , and hepatosplenomegaly . the patient in this report may present clinical evidence of type i sialidosis , but her laboratory findings do not support this diagnosis ; activities of the neuraminidase were normal . a similar case of progressive myoclonic epilepsy has been reported.10 differences from the previous report which described a patient with progressive myoclonic epilepsy , cherry - red spots and negative enzyme deficiency were theses ; 1 ) the onset - age was older than the previous report ( 27 versus 13 years , adult versus juvenile - onset ) ; 2 ) the patient had a sibling with same disease , which indicated she had inherited disease , but the patient in previous report did not have familial history ; 3 ) the patient in our report did not have a history of seizure , and it is different from the patient with myoclonic epilepsy . although the cause of cherry - red spot myoclonus is not clear , to our knowledge , this is the first report of adult - onset familial cherry - red spot myoclonus caused by an unknown type of lysosomal storage disease in korea .
we report a case of a 36-year - old woman with progressive generalized myoclonus that first became apparent 9 years ago . her younger brother had similar problems . examination of her eyes revealed cherry - red spots . hexosaminidase a , -galactosidase and neuraminidase activity were normal . although the laboratory findings were negative , cherry - red spots , progressive myoclonus and autosomal recessive inheritance pattern suggested that she had an unknown type of lysosomal storage disease .
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supported by nci 2p50 ca09825806 , nci u01 ca168394 , stand up to cancer / aacr dream team translational cancer research grant su2c - aacr - dt0209 , tcga gdac grant ( nih / nci u24 ca143883 ) to gbm ; mdacc uterine spore career development award ( nci p50ca098258 ) to lwt .
pik3r1 ( encoding the p85 subunit of phosphatidylinositol 3-kinase ) is the 11th most frequently mutated gene across tumors . we recently reported neomorphic p85 mutants that induce signaling cascades not predicted by the canonical functions of p85 , suggesting the need to functionally annotate specific mutations in cancer genes for effective genome - informed personalized therapy .
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brown - squard syndrome ( bss ) , which occurs due to dysfunction of the spinothalamic tract , typically reflects the hemisection of the spinal cord at the cervical or thoracic level . the syndrome mainly occurs as a result of penetrating trauma , syringomyelia , hematomyelia , tumor , severe discs , or blunt trauma . among the multiple etiologies , the most common cause is penetrating trauma , such as a gunshot7,8 ) . therefore , most management guidelines focus on penetrating cervical injuries and/or vertebral artery ( va ) injury12 ) . non - missile penetrating spinal cord and va injuries are rare because of the bony structures that protect the spinal cord and va14 ) . thus , the treatment approach for wounds caused in non - missile penetrating spinal injuries such as a knife , a power drill bit , or even a pen could be different from common missile penetrating injuries5,6,13,17).to our knowledge , there are few reports in the literature of complete obstruction of the va due to penetration of a foreign body through the neural foramen into the spinal canal . herein , the authors report on va dissection and bss caused by penetration of an electric screw driver bit . a 25-year - old machine operator was involved in a violent episode and was stabbed in his right neck with an electric screw driver bit that was thrown by the opponent . on arrival at the emergency department , the electric screw driver bit was placed in the right lateral aspect of the neck at zone i ( fig . the tip of the electric screw driver bit was located at the center of the vertebral canal of c3 ( fig . he was given high - dose methylprednisolone ( bolus dose of 30mg / kg followed by 5.4mg / kg / hour for 23 hours ) according to the protocol for spinal cord injury . an immediate interventional angiography was undertaken without general anesthesia due to the nature of the emergency . the angiography revealed a total occlusion with dissection of the right va at the level of c3 . immediate coil embolization at both proximal and distal ends of the injury site was performed ( fig . an attempt at manual extraction of the electric screw driver bit failed with great resistance . after the patient was moved to the operating room , the electric screw driver bit was removed manually with muscle dissection under general anesthesia . venous blood spilled out and was controlled easily by application of several pieces of gelatin sponge . no postoperative complications such as wound dehiscence , cerebrospinal fluid ( csf ) leakage , or infection were observed . the neurological motor function of the right upper and lower extremities recovered to 3/5 and 4/5 , respectively , with persistent decreased sensory function after one year . fortunately , the patient experienced no neck swelling , auscultation of a neck bruit , or delayed ischemic complications . penetrating injury is the third most frequent cause of spinal cord injury in adults , surpassed only by traffic accidents and falls3,18 ) . stab wounds are associated with lesser surrounding tissue injury than gunshot wounds because the former delivers less energy than missile injuries9 ) . although vascular injury is the most common sequel of penetrating neck trauma , va injury is rare because it is well protected by the transverse foramen4,10 ) . therefore , penetrating injury of the va is mostly caused by gunshot wounds which deliver large kinetic energy , depending upon the bullet 's mass and speed12 ) . in this article , we report a rare case of va penetration by an electric screw driver bit with spinal cord insult , consequently presenting as bss . moreover , surgical exploration of the va can cause additional damage to the spine and surrounding tissues . therefore , it may be reasonable to embolize an occluded artery , because the unilateral ligation of the va rarely results in brainstem ischemia11,16 ) . there are a few reports regarding the treatment of traumatic va injury such as the arteriovenous fistulas and pseudoaneurysms2 ) . emergent surgical exploration is necessary for patients with hard signs of vascular injury , such as hemodynamic instability , hemorrhage exsanguinations , or expanding hematoma15 ) . patients that are hemodynamically stable and who are without respiratory compromise should undergo further diagnostic imaging evaluation15 ) . as presented in this case , endovascular techniques were a safe and effective method of treatment and were not associated with significant morbidity or mortality1 ) . airway management , intubation methods , and surgical positions can be points of debate between anesthesiologists and surgeons9 ) . if a lacerated va can be successfully obliterated , a penetrating electric screw driver bit may be extracted without general anesthesia . nevertheless , the authors recommend that surgeons should be prepared for conversion to open surgery and extraction should be performed with the support of a surgical team . we initially tried to extract the electric screw driver bit manually without general anesthesia in the intervention theater after va embolization . however , the electric screw driver bit was positioned firmly in the neural foramen , and the patient complained of severe pain when the electric screw driver bit was being pulled out . in addition , there was more important rationale that justified surgical exploration for extraction of the electric screw driver bit . on extraction of the electric screw driver bit , the authors describe a rare case of penetrating cervical injury caused by an electric screw driver bit with accompanying va penetration and bss .
there are few reports in the literature of complete obstruction of the vertebral artery ( va ) due to an electric screw driver bit penetration through the neural foramen into the spinal canal with brown - squard syndrome ( bss ) . a 25-year - old man was admitted to the emergency department with a penetrated neck injury by an electric screw driver bit after a struggle . the patient presented the clinical features of bss . computed tomography scan revealed that the electric screw driver bit penetrated through the right neural foramen at the level of c3 - 4 , and it caused an injury to the right half of the spinal cord . emergent angiography revealed va dissection , which was managed by immediate coil embolization at both proximal and distal ends of the injury site . after occlusion of the va , the electric screw driver bit was extracted under general anesthesia . bleeding was minimal and controlled without difficulties . no postoperative complications , such as wound dehiscence , csf leakage , or infection , were noted . endovascular approaches for occlusion of vertebral artery lesions are safe and effective methods of treatment .
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despite advancements in modern medical science and the health management industry , the incidence of cerebral palsy ( cp ) continues to rise1 . the most common features of cp are decreased muscle strength and abnormal muscle tone2 . cp sufferers lack theability to generate enough force to maintain antigravity postural control , which result in abnormal postures3 . the development of movement and posture may be altered by non - progressive damage to the brain and subsequent neurological impairments ( spasticity , muscle weakness , co - contractions and visual impairment)5 . studies indicate that children and adults with both mild and severe forms of cp have postural impairments6,7,8 . the emergence of sitting postural control in early infancy changes the way infants interact with the world . from the sitting position , looking , reaching , and interacting become functional and allow exploration that supports learning and further development of motor skills . therefore , independent sitting , defined as not needing support from a caregiver or pillow while sitting , is one of the first developmental goals for every child . individual differences are present between children , and characteristic signs of developmental disorders during infancy are relatively unspecific . therefore , why a specific child is not able to achieve sitting postural control is not always clear . one method of examining postural control in adults and children is to measure the center of pressure ( cop ) at the base of support using a force platform during the task of remaining upright . cop has frequently been used to investigate postural control during standing by young children who are healthy or have cp9 , 10 . the purpose of this study was to investigate the differences of the pressure distributions of the sitting postures of typical developmental ( td ) children and children with cp . twelve cp childrens were recruited from an outpatient rehabilitation clinic . they were hemiparetic , had an mmse - k score above 2411 , could maintain an independent sitting posture without support . two of the cp children were subsequently excluded because they refused to participate in this experiment . all parents of the enrolled participants provided their written informed consent to their children s participation prior to this experiment , in accordance with the ethical principles established in the declaration of helsinki . as a result of the exclusion , this study used two school chairs mounted on a force platform to assess the quiet - sitting pressure distribution of the subjects . fsa seating assessment ( canada ) the acquisition frequency was set at 5 hz . the stated working range of the device is 0200 mmhg , with a resolution of 1 mmhg . the system was also calibrated to assign absolute pressure values to the digital output from an a / d converter connected to the sensing pad . this was done by applying a pressure distribution as similar to actual conditions as possible . then thus subjects sat on one of two school chairs according to their height . the chairs were those generally used in school . for research purposes , this study used two basic school chair because students spend a long time of day - to - day sitting on them . one chair had a 40 cm floor to seat height , a 35 cm seat depth , and a 32 cm seat width and is designed for 122.4133.5 cm height of subjects . the another had a 35 cm floor to seat height , a 38 cm seat depth , and a 35 cm seat width , and is designed for 133.6152.7 cm height of subjects . it has been used for the posture symmetry in other study13 statistical analyses were performed using pasw 18.0 . descriptive statistics were calculated ( frequency , mean , standard deviation , range ) . the mann - whitney u - tests and wilcoxon s signed rank tests were used to analyze differences between the groups and differences in lesion side , respectively . the si of the age matched td group was employed as the normal criteria . table 1table 1.general characteristics of the subjectscerebral palsygroup ( n=10)typicaldevelopmentalgroup ( n=10)age ( years)8.040.827.840.94gender ( male / female)5/54/6lesion side ( right / left)4/6symmetry index ( meansd)5.041.342.302.76 shows the general characteristics of the participants . the si of the td group was significantly closer to zero than that of the cp group of children . cp and right hemiparetic cp were respectively 4.96 ( 2.24 ) and 5.12 ( 0.83 ) with no significant difference between the hemiparetic sides . the purpose of the present study was to investigate the differences in sitting posture of children with cp while they sat on school chairs . it is known that dynamic postural control during sitting can be reliably assessed using cop data of infants who are developing typically or infants with or at risk of cp14 . the principal finding of this study is that cp children s sitting posture is asymmetrical , leaning to the less - paretic side . we thought that infants who are developing typically develop the ability to sit by exhibiting an optimal range of movement variability , whereas cp may present either too much or too little variability leading to a very rigid and narrow or unpredictable set of movement solutions to achieve independent sitting . the finding of this study is agreement with previous studies that have the dissimilarities of the cop patterns of infants with cp and td have been obviously demonstrated14 . the results can not be generalized to all cp children because the sample was limited to ten children and they were at the one stage of the developmental process . future studies should assess the relationship between symmetry of sitting posture and functional activities .
[ purpose ] the purpose of this study was to investigate the differences in symmetry of sitting posture between typical developmental ( td ) children and hemi - cerebral palsy ( cp ) children . [ subjects and methods ] a school chair mounted on a force platform was used to assess the quiet - sitting pressure distribution of 10 td and 10 cp children . [ results ] the symmetry index of the td children was significantly closer to zero than that of the cp children irrespective of the latter group s hemiparetic side . [ conclusions ] sitting posture on school chairs of cp children was more asymmetrical than that of td children .
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the laparoscopic removal of a cervical stump following a supra cervical ( subtotal ) hysterectomy was first described by nezhat et al , and they concluded that the cervical stump could be removed laparoscopically by an experienced surgeon . the advantages of the laparoscopic approach included possible stump adhesiolysis , providing adequate postoperative vault support , and assessment of the pelvic lymph nodes . the 43-year - old , presented with a history of persistent p v discharge and occasional post - coital bleeding . she had undergone subtotal hysterectomy in 1994 , due to postpartum hemorrhage following a normal delivery . a colposcopic biopsy done in january 2009 , reported severe dysplasia of the cervix , with a human papillomavirus ( hpv ) effect and crypt extension . there was a strong family history of cancer of the cervix , as her mother had succumbed to the disease . on general examination she was in fair general condition , well - built and well - nourished , with adequate hydration . the hemoglobin was 13.3 g / dl , blood sugar was 5.3 mmols / l , urea and electrolytes were normal . an initial diagnosis of abnormal pap smear was entertained and the patient opted for a laparoscopic trachelectomy , with the option of a laparotomy , after discussing all her options . there were dense adhesions in the pouch of douglas involving the bowel and the cervical stump . the pelvic lymph nodes were clearly visualized ( after intracervical methylene blue injection ) and did not appear to be enlarged . gentle adhesiolysis was undertaken using sharp dissection , bipolar cautery , and a harmonic scalpel . the vaginal vault was subsequently opened over the ceramic cup of a clermont ferrand elevator . a cystoscopy with retrograde ureteral catheterization , to confirm the integrity of the bladder and ureters , was undertaken . the cervical stump after laparoscopic trachelectomy at one week of follow - up the patient was well . a postoperative intravenous urogram ( ivu ) confirmed that both the ureters and bladder were intact . subtotal hysterectomy was developed as a procedure in the 1990s , and is regarded as a safe option to total abdominal hysterectomy in the management of benign uterine conditions and in obstetrics , due to severe postpartum hemorrhage . okaro et al , in an assessment of the long - term outcomes of laparoscopic supracervical hysterectomy analyzed the case records of 70 consecutive women undergoing the procedure . of these , 24.3% ( 17 cases ) reported symptoms related to the cervical stump , within 14 months of the original surgery . in his series 14 of these patients underwent laparoscopic trachelectomy , one had only laparoscopic adhesiolysis and two underwent a laparotomy with trachelectomy due to dense bowel adhesions on the cervical stump . histologically the stumps showed endometriosis ( 23.5% ) and mild dysplasia in 7.6% of the patients . in this case our patient presented with persistent p v discharge and occasional post - coital bleeding . the subsequent pap smears were abnormal . in a retrospective of 41 patients undergoing laparoscopic subtotal hysterectomy , van der stege et al , noted that 98% of the patients were satisfied with their procedure , with 10% of them having monthly spotting . they concluded that although laparoscopic hysterectomy for benign diseases was a satisfactory procedure , special attention should be paid to careful management of the cervical stump . hilger et al , reviewed the indications of 310 trachelectomies performed at the mayo clinic from 1974 to 2003 . they included stump prolapse ( 4% ) , fibroid mass ( 1% ) , cervical dysplasia ( 6% ) , carcinoma in situ ( 5% ) , irregular bleeding ( 2% ) , and cervicitis ( 53% ) . the complications following vaginal trachelectomies were encountered in 80% of the procedures against 37% in the abdominal procedure . in our report the cervical stump confirmed carcinoma in situ .
a 43-year - old , who underwent a subtotal hysterectomy for postpartum hemorrhage following a normal delivery , 10 years ago , presented with a history of persistent vaginal discharge and post - coital bleeding . a pap smear reported moderate dysplasia , and a subsequent colposcopic biopsy reported severe dysplasia with crypt extension . the patient underwent a laparoscopic trachelectomy , and histology of the stump reported cervical squamous carcinoma in situ , with no microinvasion .
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established in 2003 , erste foundation has a history stretching back 190 years to the year 1819 when it was founded as the erste sterreichische spar casse in vienna . rooted in our history as a social enterprise and financial service provider , erste foundation recognises that contemporary society faces huge challenges and that for a new and united europe to work , integration is crucial and that means uniting the economic , cultural and social capital of our region . we develop ideas and concepts to increase social participation and to ensure that no - one is left out , whatever their circumstances which in turn creates stable , effective and fairer societies . we want to work against prejudice and nationalism , to integrate thinking and living across borders , and make these experiences accessible , particularly to the young generation . we intend to play an active role in giving people opportunities to increase their understanding of each other . therefore , erste foundation has supported research projects that concern themselves with the effects of societal transformation processes : in particular we have been looking at the effects that demographic changes will have on the long - term care needs of elderly people in central and eastern europe . from this , we intend to develop policy recommendations for decision makers in civil society , economy and politics .
introductionestablished in 2003 , erste foundation has a history stretching back 190 years to the year 1819 when it was founded as the erste sterreichische spar casse in vienna . rooted in our history as a social enterprise and financial service provider , erste foundation recognises that contemporary society faces huge challenges and that for a new and united europe to work , integration is crucial and that means uniting the economic , cultural and social capital of our region.descriptionwe develop ideas and concepts to increase social participation and to ensure that no - one is left out , whatever their circumstances which in turn creates stable , effective and fairer societies.european integration is important to us . we want to work against prejudice and nationalism , to integrate thinking and living across borders , and make these experiences accessible , particularly to the young generation.projectswe intend to play an active role in giving people opportunities to increase their understanding of each other . therefore , erste foundation has supported research projects that concern themselves with the effects of societal transformation processes : in particular we have been looking at the effects that demographic changes will have on the long - term care needs of elderly people in central and eastern europe . from this , we intend to develop policy recommendations for decision makers in civil society , economy and politics .
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acute generalized exanthematous pustulosis ( agep ) is a rare acute reaction that is drug - induced in 90% of the cases , characterized by a widespread , sterile pustular rash . cefepime is a fourth generation cephalosporin antibiotic used to treat febrile neutropenia , severe infections related to the urinary tract , skin , nosocomial pneumonia , brain abscess , and intra - abdominal and septic lateral / cavernous sinus thrombosis . a 67-year - old man with renal failure who had been on dialysis during the last 2 years and with an 8-year history of cardiac insufficiency was admitted to the hospital complaining of 6 days of diarrhea . the patient was taken to the semi - intensive care unit and treated with ciprofloxacin . as a consequence , his long - term medications had not been changed and consisted of acetylsalicylic acid , furosemide , captopril , carvedilol and clonazepam . on the seventh day , the patient became dyspneic and his chest radiograph showed a left lower lobe opacity . treatment for nosocomial pneumonia was promptly initiated with cefepime ( 1 g / day ) . five days later , he presented with a pruritic , erythematous , maculopapular eruption affecting the abdomen , neck and skin folds . one day later , he developed disseminated pustular lesions ( fig . 1 ) and his temperature was 37c . laboratory exams evidenced c - reactive protein 136 mg / l , white blood cells 14,700 cells/l ( normal 3,50010,500 cells/l ) with 11,995 cells/l neutrophils ( normal 1,7008,000 cells/l ) . histology showed a toxic pustuloderma with spongiform subcorneal pustules , edema in the papillary dermis and perivascular inflammatory infiltrate consisting of neutrophils ( fig . after withdrawal of cefepime and introduction of imipenem , the disseminated skin nonfollicular pustules cleared within 4 days following a desquamation . the patient denied previous adverse reaction to other drugs and no personal or family history of psoriasis was evident . agep is a disease characterized by the rapid onset of many sterile , nonfollicular pustules usually arising on an edematous erythema and frequently accompanied by leukocytosis and fever . skin symptoms usually arise rapidly after an insult and resolve spontaneously ( within a few days ) . agep often starts predominantly in intertriginous areas or on the face , spreading rapidly to the trunk and lower limbs . the mean duration of the pustules is 9.7 days , and an annular desquamation typically follows for a few days . complications are rare [ 1 , 3 ] . the agep validation score of the euroscar study group has been used to establish the diagnosis . a score between 8 and 12 for agep is a definitive diagnosis ( table 1 ) . the case score was 11 , according to the validation score of the euroscar study group ( table 2 ) . the main differential diagnosis of agep is pustular psoriasis . because the pustules clinically and histologically resemble the lesions of pustular psoriasis and because in a number of reports patients had a history of plaque psoriasis , some authors assume that agep is nothing more than an acute exacerbation of psoriasis caused by a variety of exogenous triggers however , many studies strongly suggest that agep is not associated with psoriasis [ 1 , 5 ] . up to now agep has been attributed to a variety of causes such as viral infections , chlamydia pneumoniae infection or hypersensitivity to mercury , but the skin reaction is primarily an adverse response to drugs . antibiotics , other than cefepime , have been implicated as the causative agents in 80% of individuals . in this group , the present case of agep has well defined criteria , and because correct diagnosis generally leads to spontaneous resolution once the causative drug is withdrawn , clinicians should keep the possibility of this cutaneous drug reaction in mind .
acute generalized exanthematous pustulosis ( agep ) is a rare cutaneous rash characterized by widespread sterile nonfollicular pustules . cefepime is a fourth generation cephalosporin , used to treat severe infections . a 67-year - old man was admitted with acute gastroenterocolitis . on the seventh day , the patient developed a nosocomial pneumonia and cefepime was initiated . on the fourth day of cephalosporin treatment , he presented with a maculopapular , pruritic eruption affecting the face , neck , abdomen and limbs . one day later he developed disseminated pustular lesions and his temperature was 37c . laboratory analysis evidenced leukocytosis and skin biopsy showed subcorneal pustule , edema in the papillary dermis , perivascular inflammatory infiltrate consisting of neutrophils , leukocytoclasia and red cell extravasation in the epidermis . cefepime was suspended and within 4 days the non - follicular pustules cleared following a desquamation . agep is a disease attributed to a variety of causes , but in 90% of the cases it is due to an adverse drug reaction . antibiotics are implicated in 80% of these cases , mostly penicillins and macrolides . there are few cases associated with cephalosporins . it is very important to consider agep in cases of acute pustular rashes and drugs should be investigated as causative agents .
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canaliculitis is a common encounter in ophthalmic practice but supernumerary puncta and canaliculi ( spc ) are rare congenital disorders . in a large series a 59-year - old gentleman presented with painful swelling of the left lower lid for a week , which was associated with epiphora . the swelling was confined to the nasal aspect of the left lower lid ( 0.50.5 mm ) with inflamed overlying skin ( figure 1a ) . eversion of the lower eyelid revealed two puncta , 0.5 mm apart ( figure 1b ) . the outer punctum was situated at the normal anatomical position ; whereas the inner punctum in the caruncle . gentle pressure did not result in any regurgitation from the both puncta . the patient was treated with oral cloxacillin 500 mg , 6 hourly for 5 days . the outer punctum had a soft stop with regurgitation of fluid from the same punctum . the outer punctum - canaliculus system was a cul - de - sac ( figure 1c ) . c ) dacryocystography showed pooling of dye in the cul - de - sac ( white arrow ) . c ) dacryocystography showed pooling of dye in the cul - de - sac ( white arrow ) . most spcs ( 78% ) present with epiphora . among the 23 patients reported by satchi et al . , none presented with canaliculitis . sequestration of tear and debris in the cul - de - sac served as nidus for infection . the resultant canaliculitis with its surrounding edema caused obstruction of the lacrimal drainage ; hence epiphora . epiphora however , may develop despite patent lacrimal drainage system . the 2-compartment model for lacrimal canalicular drainage of kakizaki et al . , suggested that the muscle of duverney - horner may deviate normal flow within the accessory canaliculus and thence transport tears back to the lacrimal tear lake , leading to epiphora . a solid epithelial cord forms in the region of the medial lower eyelid ( figure 2a ) and sends projections to form the canaliculi and the nasolacrimal duct ( figure 2b ) . spc is due to extra out - budding of the solid epithelial cord ( figure 2c ) . canalization begins at 4 months of gestation with disintegration of the central ectodermal core , forming lacrimal drainage outflow system . in this case , the extra inner canalicular epithelial bud ( nearer to the main epithelial cord ) underwent complete canalization and remained connected to the main epithelial cord . the outer canalicular epithelial bud , although its punctum is located at the normal anatomical position , was separated from the main epithelial cord ; forming a cul - de - sac ( figure 2d ) . c ) extra out - budding of the solid epithelial cord in supernumerary puncta and canaliculi . d ) the outer canalicular epithelial cord was separated from the main epithelial cord , forming a cul - de - sac . c ) extra out - budding of the solid epithelial cord in supernumerary puncta and canaliculi . d ) the outer canalicular epithelial cord was separated from the main epithelial cord , forming a cul - de - sac .
we report the first case of supernumerary puncta and canaliculi presented with canaliculitis . a-59 year - old gentleman presented with painful swelling of the left lower lid for a week , which was associated with epiphora . the swelling was confined to the nasal aspect of the left lower lid ( 0.50.5 mm ) with inflamed overlying skin . two puncta ( 0.5 mm apart ) were noted . the outer punctum at the normal anatomical position was a cul - de - sac while the inner punctum it the caruncle was patent . we described the embryology leading to supernumerary puncta and canaliculi to explain the paradoxical patency of the abnormally located punctum as well as the pathomechanism leading to canaliculitis . the patient was treated with oral cloxacillin 500 mg , 6 hourly for 5 days ; the cellulitis subsided after three days .
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we are grateful for financial support from the industrial source technology development program of the ministry of knowledge economy ( 10044909 ) , the next - generation biogreen 21 program ( ssac grant # pj01111803 ) , rda , and the kribb initiative program of south korea .
abstractbacterial volatiles protect plants either by directly inhibiting a pathogenic fungus or by improving the defense capabilities of plants . the effect of bacterial volatiles on fungal growth was dose - dependent . a low dosage did not have a noticeable effect on botrytis cinerea growth and development , but was sufficient to elicit induced resistance in arabidopsis thaliana . bacterial volatiles displayed negative effects on biofilm formation on a polystyrene surface and in in planta leaf colonization of b. cinerea . however , bacterial volatile - mediated induced resistance was the major mechanism mediating protection of plants from b. cinerea . it was responsible for more than 90% of plant protection in comparison with direct fungal inhibition . our results broaden our knowledge of the role of bacterial volatiles in plant protection .
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endocrine disorders may complicate , cause or mimic otolaryngologic disorders , some of which may be anatomical , due to an enlargement of the thyroid gland , while others are physiological , resulting from increased or decreased glandular activity.1 hypothyroidism is characterized by the slowing of mental and motor activity , depression , constipation , cold intolerance menorrhagia , stiff muscles , carpal tunnel syndrome , sleep apnea , dry hair and skin , weight gain , snoring and a hoarse voice.1 less common symptoms involve the heart , muscles , joints , and blood.2 dysarthria as the presenting symptom of hypothyroidism has only been reported once before.3 here , we present an unusual case of hypo - thyroidism presenting with dysarthria . a 39-year - old female presented to the clinic with dysarthria of six months duration . , it was revealed that there had been episodes of dysphonea , snoring , sleep apnea , dysphagia and choking during eating or drinking . her past medical history was normal apart from one occasion of delayed recovery from anaesthesia during surgery for a fractured femur the year before . clinical examination , including the central nervous system , was normal apart from a slightly puffy face . there was no abnormality in the movement of the tongue or pharygeal / palate muscles . pmol / l ( normal 9.2 - 23.9 pmol / l ) , a thyroid stimulating hormone of 82.7 miu / l ( normal 0.32 - 5.00 miu / l ) , antithyroglobulin antibodies were 1:320 u / ml ( normal 1:40 u / ml ) , antiperoxidase autoantibodies of 1:1600 u / ml ( normal 1:40 u / ml ) . a complete blood count film was consistent with iron deficiency , a haemoglobin of 7grams ( normal 11 - 16 grams ) , low serum iron of 2 umol / l ( normal 10 - 28 umol / l ) , increased red cell distribution width ( rdw ) of 17.2 ( normal 11.6 - 13.7% ) . other biochemical abnormalities were high serum cholesterol of 6.9 mmol / l ( normal 3.6 - 6.8 , hypo - thyroidism was diagnosed . in the light of the patient 's diagnosis , a second history was taken which showed that she suffered from other symptoms of hypothyroidism such as , dry skin , generalised weakness , excessive sleeping , hoarse voice , and menorrhagia . two months after the initiation of therapy , the patient had no more dysarthria or other associated symptoms . dysarthria is a disturbance of articulation that may be caused by a neuromuscular lesion , or an abnormality of the vocal cords . the first may result from damage to the central or peripheral nervous system such as head trauma , brain stem infarction , bulbar palsy , motor neuron disease , peripheral neuropathy , huntington 's chorea , parkinson 's disease , multiple sclerosis , myasthenia gravis , or muscle disease.4 the second may be attributable to congenital , traumatic , inflammatory , tumors , or post - operative lesions of the vocal cords . these causes were unlikely in this patient , because she showed no associated clinical features of these diseases besides the normal neurological examination and investigations . other causes such as congenital or aquired storage disorders such as amyloidosis , and such endocrine disorders as acromegaly or hypo - thyroidism,3 as in the presented case , may lead to an enlargement of one or more of the components of the vocal cords.56 the most likely cause for the dysarthria in this patient was hypothyroidism . this was supported by the abnormal thyroid functions and the response of the dysarthria to thyroxin . dysarthria due to hypothyroidism had been reported only once previous to this case.3 the pathophysiology of dysarthria in hypothyroidism can be explained by edematous swelling of laryngeal and hypopharyngeal structures in combination with macroglossia.3 it has been shown that macroglossia in hypothyroidism is caused by a thickening of the epithelial tissue.6 these changes can also explain the choking and the dysphagia which this patient experienced . there have been a few reports of hypothyroidism responsible for secondary dysphagia.1012 her sleep apnea may also be a manifestation of hypothyroidism , most likely caused by edema and myopathy.7 sleep apnea attributable to hypothyroidism is reversible with thyroxin replacement therapy.8 the episodic hoarseness of voice can also be explained by hypothyroidism,1 as well as the delayed recovery from anaethesia the year before , most probably the result of undiagnosed hypothyroidism.91314 unfortunately , thyroid function tests had not been performed on our patient at that time . iron deficiency anemia in this patient was due to menorrhagia , which is one of the characteristic features of the disease.1 hyperlipidaemia may also be due to hypothyroidism , a known association.1 clinical implication of the presented patient was that dysarthria may be the presenting symptom of hypothyroidism , even if other symptoms had been present for a long time . hypothyroidism as the cause of dysarthria was confirmed with the discovery of additional symptoms in the patient 's history . otolaryngeal symptoms should therefore be considered possible symptoms of hypothyroidism.15 prompt recovery of dysarthria is expected after hormone replacement therapy .
hypothyroidism is a common endocrine disorder with characteristic clinical symptoms and signs . typical symptoms of hypothyroidism are lethargy , cold intolerance , slowing of intellectual and motor activity , decreased appetite , weight gain , and dry skin . a 39-year - old female presented to the clinic with dysarthria as the chief symptom . subsequent questions revealed that other symptoms were confined to the otolaryngeal region , which were episodes of mild dysphonia , dysphagia , sleep apnea , and snoring . laboratory data revealed marked hypothyroidism and positive tests for antithyroglobulin and antimicrosomal antibodies . after administration of thyroxin , the dysarthria and the other symptoms rapidly disappeared . dysarthria may be the presenting symptom of hypothyroidism and can be resolved after hormone replacement therapy .
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progressive nodular histiocytosis ( pnh ) is a rare normolipemic macrophage disorder first described by taunton et al . , in 1978 . it belongs to a subgroup of non - langerhans cell histiocytosis ( lchs ) , the juvenile xanthogranuloma ( jxg ) family , which includes xanthoma disseminatum ( xd ) , benign cephalic histiocytosis ( bch ) , multiple adult xanthogranuloma ( maxg ) and generalized eruptive histiocytosis ( geh ) . according to weitzman and jaffee , they present positive reaction to factor xiiia , cd68 , cd14 and negative reactions to cd1a and s-100 proteins . these disorders are difficult to categorize , primarily because of the diverse pathological findings and due to overlapping clinical and microscopic features . a 30-year - old gentleman presented to us with complaints of asymptomatic nodular skin lesions all over the body of 10 years duration . at the age of 20 years , he started developing few small skin - colored lesions on the bilateral peri - orbital areas . these lesions continued to increase in size over time and few lesions became pedunculated especially over the calves . along with the appearance of the cutaneous lesions , he also noticed a gradually progressive , bilateral painless loss of vision . he did not complain of itching , watering , photophobia or discharge from the eyes . he was diagnosed to have eale 's disease and underwent laser photocoagulation for the same . he developed premature cataracts of the eyes and was treated with phacoemulsification and posterior capsular intraocular lens implantation in both eyes . prior to presentation to us , he underwent intra - lesional steroid therapy using triamcinolone acetonide in the peri - orbital lesions with good response seen in smaller lesions but no effect on the larger lesions . the lesions at other sites were removed using various modalities like cryotherapy , radiosurgery and surgical excision of the larger , troublesome lesions . cutaneous examination revealed multiple , approximately 30 to 40 , yellowish to skin - colored , firm , non - tender papules and nodules ranging in diameter from 5 to 40 mm , over the face [ figure 1 ] , trunk , extremities , elbows , hands , palms and soles [ figure 2 ] in decreasing order of frequency . these lesions were located almost symmetrically over the peri - orbital area , mostly over the outer canthi , but also over the lower eyelids , leading to ectropion . the smaller lesions on the face were mounted on the skin , while the larger lesions on the calf area were pedunculated . papular and nodular lesions on the face and eyelid large nodules seen over the elbows , hand and sole visual acuity at the time of presentation was 6/18(r ) and 6/60(l ) . the clinical differential diagnoses considered at the time of presentation to us were multiple eruptive dermatofibromas , xanthoma disseminatum and pnh . blood examination revealed hemoglobin of 10.5 gm / dl with a microcytic hypochromic blood picture , total leukocyte count of 7200/mm and a normal differential leukocyte count . fasting lipid profile , renal , total serum proteins and albumin were normal with no reversal of the a : g ratio . a cellular tumor was seen centered in the dermis , composed of sheets of oval to spindle - shaped cells arranged haphazardly , in short fascicles and in a storiform pattern at places [ figures 3a - c ] . the spindle cells had oval to elongated benign looking nuclei with vesicular chromatin and small nucleoli . on immunohistochemistry the cells were positive for cd68 [ figure 4 ] but negative for s-100 , cd34 , cd21 , cd35 and hmb45 supporting a diagnosis of spindle cell histiocytic tumor . dab chromogen 100 based on the history , clinical examination and histopathological correlation , his dermatological disorder was diagnosed as pnh . he was started on tab imatinib 400 mg od and some of the lesions developed encrustation but it was stopped after 5 months as he continued to develop new lesions while on this drug . second - line treatment with pazopanib ( 800 mg od ) was started after stopping tab imatinib , in lines of soft tissue sarcoma . based at the age of the onset of the lesions , areas of the body involved , progression of disease , lab abnormality ( serum lipids ) , it is possible to differentiate pnh from other forms in the jxg family . benign cephalic histiocytosis and jxg usually occur in early childhood , but jxg has also been reported in adults and the lesions have a tendency to resolve spontaneously over a period of time . multiple adult xanthogranuloma is another important differential diagnosis of pnh which has similar features to jxg but occurs in adults . xanthoma disseminatum was ruled out based on the absence of mucosal and systemic involvement and with normal lipid profile as it usually presents with hyperchylomicronemia and hypertriglyceridemia and diabetes insipidus . since the accurate diagnosis of a specific disorder within the jxg subgroup is quite difficult , we made the diagnosis of pnh based on the history , presence of the typical skin lesions , progressive course and histopathological demonstration of spindle cells that were positive on immunohistochemistry for histiocyte marker , cd68 , but negative for s-100 . non - langerhan 's cell histiocytosis disorders are difficult to diagnose and our case presents another clinical expression of this variable and confusing disorder . to our knowledge this is the first case report of progressive nodular histiocytosis associated with eale 's disease .
progressive nodular histiocytosis ( pnh ) is a rare normolipemic macrophage disorder and belongs to a subgroup of non - langerhans cell histiocytosis ( lchs ) which is characterized by a progressive course with no sign of spontaneous resolution but without systemic involvement . we report a 30-year - old gentleman who presented with skin lesions all over the body associated with gradual bilateral painless loss of vision . on examination , approximately 30 to 40 , skin - colored , firm , non - tender papules and nodules were noted over the body especially on the face and trunk . a skin biopsy revealed a cellular tumor in the dermis composed of oval to spindle - shaped cells , positive for cd68 but negative for s-100 , cd34 , cd21 , cd35 and hmb45 , supporting a diagnosis of spindle cell histiocytic tumor . ophthalmic examination revealed a generalized arteriolar attenuation in both eyes . he received tab imatinib 400 mg od for 5 months followed by tab pazopanib 800 mg od for 4 months and both the drugs were stopped due to lack of any response in the skin lesions . we report this case due to its rarity , characteristic clinical presentation , and its association with eale 's disease . primary treatment remains surgical excision of bothersome lesions and optimal systemic treatment is still unknown .
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the potential relevance of endothelial activation biomarkers to sepsis has been raised in both this journal and others [ 1 - 3 ] . biomarkers for sepsis associated with the endothelial glycocalyx remain relatively unknown , however , and this commentary attempts to reverse this omission . the term glycocalyx ( sweet husk ) was introduced 50 years ago to describe an extracellular polysaccharide coating of cells . whilst electron microscopy revealed that the luminal surface of the endothelium expressed this structure , it was thought to be of little consequence or functional significance . what has become increasingly evident , however , is that the glycocalyx - now estimated to extend up to 1 m from the endothelial cell membrane - represents a substantial intravascular compartment contributing significantly to vascular wall homeostasis . specifically , roles of the glycocalyx include maintenance of the vascular permeability barrier , mediation of shear - stress - dependent nitric oxide production , and housing vascular protective enzymes ( for example , superoxide dismutase ) and a wide array of coagulation inhibition factors such as antithrombin , the protein c system and tissue factor pathway inhibitor . the glycocalyx also modulates the inflammatory response by preventing leukocyte adhesion and binding numerous ligands , including chemokines , cytokines and growth factors [ 4 - 6 ] . negatively charged and with a mesh - like structure , the endothelial glycocalyx is comprised of glycoproteins , proteoglycans , glycosaminoglycans ( gags ) and associated plasma proteins including albumin . proteoglycans consisting of a core membrane - bound protein of the syndecan or glypican families with attached heparan or chondroitin sulphate gag side chains are a prominent feature . hyaluronan - a nonsulphated , uncharged gag with water - retaining properties - is attached or adsorbed onto other cell - surface anchored proteins ( for example , cd44 ) and helps to stabilise the glycocalyx structure . alteration in the composition of the glycocalyx following exposure to an inflammatory insult is one of the earliest features of endothelial activation . it is now accepted that tnf , oxidised lipoproteins , lipopolysaccharide , thrombin , ischaemia / reperfusion , hyperglycaemia and growth factors all cause glycocalyx disruption via the action of proteases - leading either to partial degradation with release of gag side chains , or to more severe damage characterised by shedding of core proteins . several studies have evaluated circulating levels of syndecan-1 and gags in patients with sepsis [ 10 - 13 ] . plasma gag levels were higher in patients with septic shock than in matched controls , and were significantly higher in nonsurvivors . in the same study , syndecan-1 levels were also increased and correlated with the sequential organ failure assessment score . in an additional study of 150 patients either with severe sepsis or septic shock or post - abdominal surgery without the systemic inflammatory response syndrome and healthy volunteers , significant increases in plasma syndecan-1 and heparan sulphate were observed in the sepsis and surgery groups . the highest syndecan-1 levels were detected in patients with sepsis and correlated with those of il-6 . a further study showed greater syndecan-1 levels in patients with septic shock compared with healthy controls , together with a positive correlation with vascular adhesion protein-1 and with day 1 sequential organ failure assessment scores . finally , hyaluronan levels , in addition to those of syndecan-1 and heparan sulphate , have been shown to increase with severity of sepsis . whilst the care of patients with sepsis has improved over the last decade the failure of two promising drugs , eritoran tetrasodium and drotrecogin alfa , to confer significant reduction in mortality suggests that novel approaches to sepsis research are required . given the fundamental , but perhaps relatively overlooked , role of the endothelial glycocalyx in regulating vascular integrity and functions central to the pathophysiology of sepsis , identifying interventions aimed at protecting or repairing it might prove a promising therapeutic target . some clinically established therapies used for the treatment of sepsis ( such as glucose control and steroid administration ) and also approaches used in experimental studies ( such as tnf inhibition , antithrombin iii , infusion of albumin and avoidance of natriuretic peptide release ) are known to reduce glycocalyx disruption . however , drugs that might specifically increase the synthesis of glycocalyx components , refurbish the glycocalyx or selectively prevent protease degradation are not currently available . future endeavours in the field of sepsis research , which are urgently required , should not only include components of the endothelial glycocalyx in the list of biomarkers , but also consider their potential as therapeutic targets for the development of new therapies .
sepsis is the third largest cause of death in industrialised countries , but treatment remains largely supportive and effective therapeutic interventions are urgently needed . disruption and dysfunction of the microvascular endothelium leading directly or indirectly to multiple organ failure are now recognised to underpin the pathophysiology of sepsis . biomarkers of endothelial activation may therefore assume an important role in guiding future research efforts . we suggest that integral to this approach is the investigation and evaluation of endothelial glycocalyx biomarkers , not only as indicators of the pathogenic process but also to inform the development of pharmacological and other therapies .
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esophageal achalasia is a rare motility disorder of the esophagus involving the smooth muscle layer and the lower esophagus sphincter ( les ) , with its incomplete relaxation and increased tone . this pathology is characterized by difficulty in swallowing , regurgitation , and sometimes chest pain . specific tests for diagnosis of esophageal achalasia are barium swallow and esophageal manometry . esophago - gastro - duodenoscopy with or without endoscopic ultrasound can be also performed to rule out the probability of cancer . for management , dilation or stretching of the esophagus , surgery and injection of muscle relaxing substances ( botulin toxin ) in the esophagus were foreseen . we present a case of esophagus achalasia diagnosed for intense rest and effort dyspnea , persistent cough , arterial hypotension , and chest discomfort . 12-leads ecg showed sinus rhythm with pulse rate at 95 beats / min . left axial deviation and diffuse disorders of repolarization were also seen . chest x - ray revealed massively dilated esophagus along the right cardiac border [ figure 1 ] . ct of the chest showed esophageal body dilatation filled with food remaining that compressed the left atrium [ figure 2 ] . the esophageal manometry evidenced body esophageal a - peristalsis , with low amplitude of esophageal body contraction and failed relaxation of les after water swallow . left atrial compression induced by an extrinsic structure was seen at two - dimensional - trans - thoracic echocardiography ( 2d - tte ) . this structure has an elongate form and was filled of liquid drinking to differentiate esophagus from any cardiac formation [ figure 3 ] . diastolic mitral inflow pattern showed an e / a waves ratio = 1.1 ; dt measured 210 msec . ; ivrt was 87 msec . three dimensional echocardiography ( 3d - tte ) pointed out the extracardiac roundish esophageal cavity compressing left atrium , clearly separated from the heart structures [ figure 4 ] . the same evaluation performed in parasternal approach ( at level of aortic root ) consented to identify the pulmonary trunk and its subdivision in right and left pulmonary arteries [ figure 5 ] . antero - posterior chest x - ray that shows poorly defined borders at the median and lower right lobe and at the lung base ct of the chest pointed out extrinsic compression at level of the left atrium by dilated esophagus ( arrow ) two - dimensional echocardiography recorded in apical 4 chambers view showing an extrinsic compression on the left atrium due to a dilated and lengthened formation evidenced after drinking a liquid ( arrow ) ( a ) three - dimensional echocardiography performed in apical 2-chambers view . evidence of a round structure ( arrow ) compressing left atrium ; ( b ) three - dimensional echocardiography in the same approach . more evident dilated esophagus ( arrow ) located below to the cardiac plane and separated from the cardiac structures three - dimensional echocardiography performed from the parasternal approach intermediate between the long and short - axis view , at level of aortic root . clear evidence of dilated esophagus ( arrow ) compressing the lower segment of the pulmonary trunk the symptoms are a consequence of the left atrial compression that reduces its volume causing an impairment of left ventricular diastolic filling . in addition , as a consequence of increased left atrial pressure , pulmonary pressure also rises causing an intense dyspnea leading to pulmonary edema . esophageal achalasia is usually diagnosed by chest x - ray , ct , mri , and esophageal manometry . functional magnetic resonance imaging ( fmri ) has been recently proposed for the evaluation of the esophagus motility . but , the test of choice for diagnosing its extrinsic compression of left atrium by esophageal achalasia is two - dimensional echocardiography ( 2d - tte ) . at 2d - tte , the achalasia moves asynchronously with the atria in contrast to intrinsic atrial structures . in our case , 2d - echocardiography performed in apical long - axis view evidenced the compression of left atrium by an extracardiac structure corresponding to the dilated esophagus evidenced by the liquid drink . nevertheless , 2d - tte is limited to the cases with acceptable sonographic window . in the presence of a poor sonographic space , trans - esophageal echocardiography three - dimensional trans - thoracic echocardiography ( 3d - tte ) was also performed in our patient . this was firstly carried out in an individual with esophageal achalasia . in our patient , 3d - tte records consented to better appreciate the esophagus compressing the left atrium and the lower part of the pulmonary trunk . even though 3d - tte is not explanatory than 2d - tte , it consented to better evaluate the dilated esophagus separated from the left atrium and compressing this same and some neighboring structures without the liquid drink too
esophageal achalasia is a motility disorder characterized by impaired relaxation of the lower esophageal sphincter and dilatation of the distal two - thirds of the esophagus . this condition may be a non - frequent reason of extrinsic compression of left atrium . in turn , this can be a cause of some hemodynamic changes such as chest discomfort , dyspnea or reduced exercise tolerance , systemic hypotension and tachycardia . we describe a case of a patient with esophagus achalasia compressing the left atrium and inducing hemodynamic compromise . the diagnostic methods , as chest x - ray , computed tomography ( ct ) , manometry , and 2d - trans - thoracic echocardiography ( tte ) demonstrated the esophagus dilation , the impaired relaxation of the lower esophageal sphincter , and its compression on the left atrium . three - d trans - thoracic echocardiography ( 3d - tte ) was firstly performed also . this last examination pointed out better than 2d - tte the extrinsic compression of the left atrium due to the esophagus dilatation . therefore , 3d - tte is a true improvement for the echocardiographic diagnosis of the left atrial compression induced by esophageal achalasia .
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the adenoma is solitary in 8590% of patients , while others have multiple adenomas or parathyroid hyperplasia . accurate preoperative localization is essential for good surgical outcome , and inability to locate the adenoma in an ectopic gland may delay the diagnosis . nuclear imaging accurately localizes the tumor in more than 90% of cases , obviating the need for advanced imaging modalities . rarely , patients present with localization failure posing a great challenge to the treating endocrinologist and operating surgeon . we report the use of a novel imaging method leading to successful outcome in a patient of primary hyperparathyroidism with failed first surgery . a 54-year - old lady presented with body pains and muscle aches for 1-year duration to a peripheral hospital . investigations revealed high serum calcium ( 11.6 mg / dl ) , low phosphorus ( 2.6 mg / dl ) , elevated alkaline phosphatase ( 677 u / l ) and intact parathyroid hormone ( ipth ) of 116 pg / ml ( normal 10 - 65 pg / ml ) . sestamibi scan revealed right inferior parathyroid adenoma , and she was diagnosed as a case of primary hyperparathyroidism . she underwent adenomectomy along with thyroidectomy and showed no features of hungry bone syndrome postoperatively . there was no confirmation of parathyroid adenomectomy by using intraoperative pth levels or by frozen section of the removed tissue . her clinical symptoms persisted after surgery and histopathological examination of the specimen removed showed thyroid tissue with no evidence of parathyroid adenoma . she reported to us after 6 months of initial surgery with persisting complaints of body aches and myalgia . her clinical examination was unremarkable , with a normotensive blood pressure , and well - healed scar in the neck . serum biochemistry revealed elevated calcium ( 10.8 mg / dl ) , low phosphorus ( 2.8 mg / dl ) and elevated alkaline phosphatase ( 280 bone mineral density estimation revealed a t - score of -2.2 at hip joint and z - score of -2.3 . serum 25 hydroxy vitamin d level was 22 ng / l and parathyroid hormone was elevated ( ipth-140 pg / ml ) . localization with sestamibi scan revealed right inferior parathyroid adenoma with no tracer uptake in thyroid bed [ figure 1 ] . abdominal sonography showed normal renal parenchyma and ultrasonography neck and plain ct neck did not show parathyroid adenoma . tc 99 m sestamibi scan showing right inferior parathyroid adenoma in view of past history of failed surgery , tc 99 m sestamibi single photon emission computed tomography ct ( spect ) was done for precise localization of the adenoma prior to re - exploration . it revealed an ectopic parathyroid adenoma , located suprasternally in the pretracheal region on right side [ figure 2 ] . histopathological examination of the specimen confirmed the parathyroid adenoma . during last follow - up , 1 year after second surgery the patient is free of all symptoms and had normal serum calcium , phosphorus and alkaline phosphatase values . sestamibi emission computed tomography ct showing parathyroid adenoma pretracheal in location ( coronal and sagittal views ) the disease is detected during asymptomatic stage in developed countries , while we encounter the advanced spectrum of the disease with severe metabolic bone disease . precise localization is important to prevent further delay in definitive therapy after biochemical confirmation of the diagnosis . parathyroid glands are derived from pharyngeal pouches ( superior parathyroid glands from 4 and inferior from 3 pouch ) with subsequent caudal migration . the modalities available for precise localization of a parathyroid adenoma are palpation , ultrasonography ( usg ) , ct , mri , nuclear scintigraphy , and combination of these tests . ultrasonography is useful for its wide availability , convenience , cost and a guiding tool for the surgeon before surgery . however , the sensitivity and specificity of usg reported was 73% and 100% , respectively . ct and mri scans provide excellent spatial resolution but often miss a small parathyroid adenoma . this is recommended mostly in cases of failed surgery , recurrent disease and when planned for a limited surgical exploration . immediate imaging reveals the tracer uptake in both thyroid and parathyroid gland along with adenoma but the adenomatous tissue shows retention of the tracer in delayed images . spect scan is an advance in radionuclide studies with a three - dimensional ( 3-d ) reconstruction , further increasing the sensitivity for adenoma localization . spect scan , with its 3-d capability , combined with ct images , is very helpful in directing the surgeon particularly in recurrent or residual hyperparathyroidism . recent reports suggest that spect / ct is superior to spect scan alone for localization of parathyroid adenoma with nodular goiter , distorted neck anatomy and those with ectopic parathyroid glands . to conclude , our patient had an ectopic parathyroid adenoma resulting in failed initial surgery . use of a novel imaging modality like spect helped in accurate localization of the adenoma prior to repeat surgical exploration .
primary hyperparathyroidism often presents with protean manifestations , resulting in delayed diagnosis . at times , aberrant development and migration of the gland leads to ectopic location leading to problems in localization . judicious use of combination methods of localization is recommended in treatment failure or recurrent disease . we report the use of single photon emission computed tomography - ct in precise localization of parathyroid adenoma in a patient with failed initial surgery .
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ala - al - din abu al - hassan ali ibn abi - hazm al - qarshi al - dimashqi , known as ibn al - nafis ( 1210 - 1288 ad ) , was a muslim syrian physician primarily famous for being the first to describe the pulmonary circulation of the blood . the most voluminous of his books is alshamel fi sanaat tebbiat , which is a comprehensive medical encyclopedia . the aim of this review article , as a tribute to ibn al - nafis , was to introduce his valuable but neglected encyclopedia of materia medica . ibn al - nafis traditional approach in his alshamel fi sanaat tebbiat book is studied in the present article . alshamel fi sanaat tebbiat covers three branches of knowledge . the first category is devoted to theoretical traditional medicine . the third category is on materia medica covering the aspect of unani medicine , from which only 28 volumes of the comprehensive book on the traditional medicine have been found so far . the latter , introduces mono - ingredient medications in alphabetical order . each chapter , in several parts , is dedicated to the botanical characteristics and nature of each mono - ingredient medication . in addition , this book explains traditional pharmacokinetic of every single medication for each human body organs . based on pharmaco - mechanistic perspective on alshamel fi sanaat tebbiat , it could be considered as the main reference book on traditional medicine and pharmacy , worthy of revival .
background : ala - al - din abu al - hassan ali ibn abi - hazm al - qarshi al - dimashqi , known as ibn al - nafis ( 1210 - 1288 ad ) , was a muslim syrian physician primarily famous for being the first to describe the pulmonary circulation of the blood . the most voluminous of his books is alshamel fi sanaat tebbiat , which is a comprehensive medical encyclopedia . it comprised 300 volumes of notes , from which only 80 volumes are published . his writings are cataloged in many libraries around the world . the aim of this review article , as a tribute to ibn al - nafis , was to introduce his valuable but neglected encyclopedia of materia medica.methods:ibn al - nafis traditional approach in his alshamel fi sanaat tebbiat book is studied in the present article.results:alshamel fi sanaat tebbiat covers three branches of knowledge . the first category is devoted to theoretical traditional medicine . the second is in four sections where much of it is not available yet . the third category is on materia medica covering the aspect of unani medicine , from which only 28 volumes of the comprehensive book on the traditional medicine have been found so far . the latter , introduces mono - ingredient medications in alphabetical order . each chapter , in several parts , is dedicated to the botanical characteristics and nature of each mono - ingredient medication . in addition , this book explains traditional pharmacokinetic of every single medication for each human body organs.conclusion:based on pharmaco - mechanistic perspective on alshamel fi sanaat tebbiat , it could be considered as the main reference book on traditional medicine and pharmacy , worthy of revival .
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fixed drug eruption ( fde ) is a distinctive variant of drug induced dermatoses characterized by sharply demarcated , erythematous patches with / without blistering that develop within hours of administration of the causative drug and heals with postinflammatory residual hyperpigmentation . it usually recurs at the same site of the skin or mucous membrane upon subsequent exposure to the same / similar group of drugs . fluoroquinolones are widely used antimicrobials , which cause cutaneous adverse drug reactions in about 1 - 2% of patients . however , bullous fde is rarely reported . herein we report a rare case of fde induced by ciprofloxacin followed by ofloxacin administration . a 37-year - old male presented to the outpatient dermatology department of our hospital , puducherry with a history of multiple fluid filled blisters over both hands and feet [ figures 1 and 2 ] . he stated that the lesions appeared within 5 h of taking a single dose of oral ofloxacin , which was obtained as over the counter drug for fever from a local private medical shop . history of itching over both hands and feet followed by a burning sensation and the subsequent development of multiple fluid filled lesions were present . there was no previous history of any medical conditions such as allergy or atopic dermatitis . on further inquiry , he recalled a history of a similar episode about 1 year back for ciprofloxacin , which has been prescribed for fever . at that time physical examination revealed multiple flaccid bullous lesions with intact roof of the blister in an erythematous base were seen over proximal metacarpophalangeal joint of left thumb , left instep of sole , right dorsal big toe and little toe of left foot . diagnosis of fde caused by ofloxacin was made taking into account of previous history of fde induced by ciprofloxacin and clinical signs . patch test was not done as the patient did not give consent for the same . the causative drug ofloxacin was discontinued and the patient was treated with antihistaminics and topical emollients . the lesions and symptoms improved gradually within a week leaving behind residual hyperpigmentation and the patient was advised not to take fluoroquinolones in future . well - defined bullous lesion in the instep of left foot bullous lesions in the right dorsal toe fluoroquinolones are commonly used antimicrobials ( effective for both gram negative and gram positive bacteria ) in the treatment of various bacterial infections and are generally well tolerated . common side - effects include gastrointestinal effects ( nausea , vomiting and diarrhea ) and neuropsychiatric symptoms ( headache and insomnia ) . photosensitivity and morbilliform rash have been reported with fluoroquinolones , but fde is quite uncommon . a large number of drugs have been reported to elicit fdes such as trimethoprim - sulfamethoxazole , tetracyclines , penicillin , erythromycin , nonsteroidal antiinflammatory drugs , barbiturates , valproate , phenytoin , phenolphthalein , and nitroimidazoles . even though , the pathogenesis of fde is not known , certain serum factors , antibodies , and cell mediated immunity have been attributed as causative factors . localized tissue damage results when intra - epidermal cd t - cells are activated to kill surrounding keratinocytes and release cytokines such as interferon - gamma into the microenvironment . quinolones can cause both delayed type and ige - mediated hypersensitivity reactions . in this case , the following criteria were considered : there were previous conclusion reports on this reaction ( + 1 ) ; the adverse event appeared after ofloxacin was administered ( + 2 ) ; adverse event improved when ofloxacin was discontinued ( + 1 ) ; adverse event reappeared when ofloxacin was re - administered ( 0 ) ; alternate causes that could solely have caused the reaction ( + 2 ) ; the reaction reappeared when a placebo was given ( 0 ) ; drug detected in the blood ( or other fluids ) in a concentration known to be toxic ( 0 ) ; the reaction was more severe when the dose was increased or less severe when the dose was decreased ( 0 ) ; the patient had a similar reaction to ciprofloxacin in the previous exposure ( + 1 ) ; the adverse event confirmed by objective evidence ( + 1 ) . probable reaction to ofloxacin administration . according to who - uppsala monitoring centre causality assessment system patient had fde to ciprofloxacin 1 year back followed by similar reaction to ofloxacin in the current admission . cross - reaction between quinolone families , clinically manifested as fde , has been rarely reported in the literature . to the best of our knowledge , only one case of cross reactivity between ciprofloxacin and ofloxacin has been reported so far which proposed the probable mechanism would be a complex of quinolone and piperazine residue as the antigenic determinant for both ciprofloxacin and ofloxacin . bullous fde due to fluoroquinolones should be included in the differential diagnosis when fde is suspected . our case described the cross sensitivity between two fluoroquinolones ciprofloxacin and ofloxacin used within 1 year interval time . hence , health care providers should be aware of the diagnosis and proper management of fde . patients should be warned against the use of anti - microbials without the physician 's advice .
fixed drug eruptions ( fde ) are the common dermatological adverse drug reaction accounts for 1621% of all cutaneous drug reactions in india . drugs most frequently implicated in fde are antimicrobials , anticonvulsants , and nonsteroidal antiinflammatory drugs . here , we report a rare case of bullous fde due to ciprofloxacin followed by ofloxacin administration .
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though both minor and major spontaneous or post - operative bleeding is the most common presentation of this rare disorder , there are several case reports of thrombotic complications also . there are few reports of myocardial infarction ( mi ) in the literature in patients of afibrinogenemia . a 33-year - old man , who was a confirmed case of congenital afibrinogenemia and was diagnosed six years back when he had excessive bleeding following trauma over face and persisted even after suturing that area , presenting to us with severe retro sternal chest pain of 10 h duration . he had a past history of myocardial infarction ( mi ) two years back and was advised dual antiplatelet therapy . he was born of second degree consanguineous marriage with history of sibling death after birth . on admission , electrocardiogram showed 2 mm st segment elevation in leads ii , iii , avf and st depression in leads i and avl [ figure 1 ] . troponin t obtained at admission was strongly positive with 1.24 ng / ml ( normal- < 0.1 ng / ml ) . coagulation profile was sent after admission and tests revealed absent fibrinogen using the clauss method , markedly reduced fibrinogen antigen level , normal platelet count and bleeding time , infinitely prolonged activated partial thromboplastin time ( aptt ) , prothrombin time ( pt ) and thrombin time . prominent q wave , st segment elevation and t wave inversion in lead ii , iii and avf with st segment depression seen in lead i and avl . right sided chest leads ( v4r - v6r ) showed < 1 mm st segment elevation as this patient had high risk for bleeding , thrombolysis or primary percutaneous transluminal coronary angioplasty ( ptca ) was not advised though he had ongoing chest pain . he was treated with dual antiplatelet therapy ( aspirin plus clopidogrel ) , statins , betablocker , angiotensin converting enzyme inhibitors and injection nitroglycerin ( ntg ) . after few hours of treatment , the chest pain subsided and st segment showed evolving changes . his admission lipid profile was normal ( low density lipoprotein 112 mg / dl , triglyceride 128 mg / dl , high density lipoprotein 40 mg / dl ) . the patient did not experience a recurrence of angina and was discharged three days after admission with dual antiplatelet therapy . fibrinogen is the major coagulation protein in blood by mass : normal fibrinogen levels vary between 1.5 and 3.5 bleeding , which usually manifests already in the neonatal period ( 85% of cases presenting umbilical cord bleeding ) , is the main complication of afibrinogenemia . paradoxically , both arterial and venous thromboembolic complications have also been reported in afibrinogenemic patients . these complications can occur in the presence of concomitant risk factors such as a co - inherited thrombophilic risk factor or after replacement therapy . first , even in the absence of fibrinogen , platelet aggregation is possible due to the action of von willebrand factor and , in contrast to patients with hemophilia , afibrinogenemic patients are able to generate thrombin , both in the initial phase of limited production and also in the secondary burst of thrombin generation . second , the increase of prothrombin activation fragments or thrombin - antithrombin complexes have been observed , reflecting enhanced thrombin generation . so , antithrombin role has also been attributed to fibrinogen because in its absence , clearance of thrombin is impaired . though there are several reports of both arterial and venous thrombosis in afibrinogenemia , only a few cases have been reported where these patients developed mi . with recurrent mi , treatment of mi in the presence of a bleeding disorder like afibrinogenemia is difficult as administration of thrombolysis and anticoagulant will increase bleeding . so , we treated with both aspirin and clopidogrel in our case . as patient stopped taking dual antiplatelet therapy he had recurrence of mi . chest pain subsided after starting injection of ntg and the area of myocardial involvement was also small , we managed the patient conservatively , and discharged him on dual antiplatelet therapy . further study is needed on this aspect to determine the best treatment that we can provide to them . until then dual antiplatelet therapyshould be recommended to all these patient with hereditary bleeding disorder with close supervision of bleeding diathesis since without this treatment they may have recurrences .
afibrinogenemia is a rare autosomal recessive bleeding disorder with an estimated prevalence of 1:1,000,000 . usual presentation of this disorder is spontaneous bleeding , bleeding after minor trauma and excessive bleeding during interventional procedures . paradoxically , few patients with afibrinogenemia may also suffer from severe thromboembolic complications . the management of these patients is particularly challenging because they are not only at risk of thrombosis but also of bleeding . we are presenting a case of 33-year - old male patient of congenital afibrinogenemia who had two episodes myocardial infarction in a span of two years . the patient was managed conservatively with antiplatelet therapy and thrombolytic therapy was not given due to high risk for bleeding .
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mesenteric pseudocyst is a term used to describe abdominal cystic mass without the origin of abdominal organ.(1 ) this has been classified according to embryologic , ehiologic , histologic , and ther data , causing considerable confusion . it was considered the term mesenteric cyst as merely descriptive , and apply a histologic classification such as lymphangioma , pseudocyst , enteric duplication cyst , enteric cyst , and mesothelial cyst.(2 ) we presented a case of mesenteric pseudocyst of the small bowel in a 70-year - old man . a 70-year - old man was referred to our hospital for operation of gastric cancer with a 1-month history of progressively worsening epigastric and intermittent peri - umbilical discomfort . he had no specific previous medical or surgical history including cancer . on physical examination , esophago - gastro - duodenoscopy ( egd ) showed a 3.5 cm sized excavated lesion on the posterior wall of angle . endocopic biopsy confirmed a histologic diagnosis of poorly differentiated adenocarcinoma including signet ring cell component . endoscopic ultrasonography revealed invasion of caner to the proper muscle layer . abdominal computed tomography ( ct ) scan showed a focal mucosal enhancement in posterior wall of angle of stomach , a 2.4 cm sized enhancing mass on distal small bowel loop without distant metastases or ascites in rectovesical pouch , and multiple gallbladder stones ( fig . 1 ) . these physical , laboratory , and radiological findings prompted us to diagnose early gastric cancer , and gastrointestinal stromal tumor of small bowel . laboratory testing revealed alfa - fetoprotein level of 2.88 ( normal range , 0 to 9 ng / ml ) , carcino - embryonic antigen level of 1.45 ng / ml ( normal range , 0 to 5 ng / ml ) , carbohydrate antigen ( ca ) 19 - 9 level of 6.5 u / ml ( normal range , 1 to 35 u / ml ) , and ca 72 - 4 level of 4.8 u / ml ( normal range , 0 to 4 u / ml ) . other laboratory test results were within normal limit . the patient underwent subtotal gastrectomy with gastroduodenostomy , segmental resection of small bowel , and cholecystectomy . mesenteric mass was adhered severely with greater omentum at the mesenteric side of small bowel , and mesenteric fat tissues . small bowel , mesentery , and mesenteric mass were resected en - bloc methods , and end to end anastomosis was performed . after fixation of the surgical specimen , macroscopic examination revealed a uni - locular cyst measuring 332 cm in size . pathological examination revealed 3 cm sized fibrous cystic wall without endothelial or epithelial lining and foam cell collection ( fig . 2 , 3 ) . pathologic stage of gastric cancer was t1bn1m0 ( 6th international union against cancer tnm staging system ) ; invasion to submusosa , metastases to 4 perigastric lymph nodes out of 16 retrieved nodes , and negative resection margin . mesenteric pseudocysts are very rare intraabdominal mass with an incidence of about 1 case per 100,000 hospital admissions.(3 ) ros et al.(2 ) first used the term " pseudocyst " in the classification of mesenteric cyst . mesenteric pseudocyst could be located in the small bowel , large bowel mesentery and even retroperitoneum.(1,4 ) most reports were pseudocyst of large bowel or retroperitoneum.(1 ) although most mesenteric pseudocysts are asymptomatic , symptomatic mesenteric cysts could be associated with cyst size , cyst location , and complications , including infection , rupture , hemorrhage , and intestinal obstruction.(5 ) in our patient , there was no specific symptom associated with mesenteric pseudocyst except for intermittent vague periumbilical discomfort . if egd and ct scan were not performed in this patient presenting non - specific abdominal pain , the diagnosis of mesenteric pseudocyst would be delayed . to the best of our knowledge , this is the first case report describing incidentally detected mesenteric pseudocyst of small bowel in gastric cancer patients . when clinician performed staging work up for gastric cancer , should be aware the possibility of associated intraabdominal lesions .
mesenteric pseudocyst is rare . this term is used to describe the abdominal cystic mass , without the origin of abdominal organ . we presented a case of mesenteric pseudocyst of the small bowel in a 70-year - old man . esophago - gastro - duodenoscopy showed a 3.5 cm sized excavated lesion on the posterior wall of angle . endocopic biopsy confirmed a histologic diagnosis of the poorly differentiated adenocarcinoma , which includes the signet ring cell component . abdominal computed tomography scan showed a focal mucosal enhancement in the posterior wall of angle of the stomach , a 2.4 cm sized enhancing mass on the distal small bowel loop , without distant metastases or ascites in rectal shelf , and multiple gallbladder stones . the patient underwent subtotal gastrectomy with gastroduodenostomy , segmental resection of the small bowel , and cholecystectomy . the final pathological diagnosis was mesenteric pseudocyst . this is the first case report describing incidentally detected mesenteric pseudocyst of the small bowel in gastric cancer patients .
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we used the lrn as a conduit to maintain the confidentiality and anonymity of the variola testing sites . a convenience sample of 45 laboratory workers completed an online survey developed by researchers at the university of nebraska medical center ( omaha , ne , usa ) . nonidentifying demographic information was collected , in addition to any adverse effects after vaccination and perceived barriers to revaccination . to determine a significant difference existed regarding the success ( presence or absence of a take after vaccination ) of the vaccine based on intervals between vaccines , we measured the mean interval ( in years ) between vaccinations . respondents mean age was 46 years ; they had worked a mean of 20.5 years in the laboratory setting . eighty - four percent of respondents reported that the only adverse events from vaccination were related to the skin irritation caused by the occlusive dressings worn over the vaccination lesion . sixty - seven percent listed a medical condition in themselves or a close household contact as the barrier to revaccination . the mean interval from first to second vaccination was 4.8 years for vaccinees who had a successful vaccine and 6.0 for those who did not . statistical analysis demonstrated no significant difference ( p = 0.149 ) between the number of years between first and second vaccinations and the take rates . sixty - two percent of respondents indicated they did not work with non highly attenuated orthopoxviruses . ( i.e. , developed lesions ) regardless of number of years since previous vaccination , suggesting that immunity might have waned . therefore , our data do not provide evidence to suggest that the acip recommended interval for revaccination be prolonged . although most respondents reported having no adverse effects from the vaccine , for some this vaccination caused discomfort . many reported symptoms related to the occlusive dressing worn as a precautionary measure to ensure that the lesion site was properly covered during work hours . other measures to ensure the lesion is covered appropriately , such as nonocclusive dressings and long sleeves , may be considered given that laboratory workers do not have direct contact with patients . although the lrn asks this small group of laboratory workers to comply with the acip recommendations , the question remains whether this requirement should include laboratory workers who do not handle orthopoxviruses . revaccination of most laboratory workers at variola testing sites every 3 years would be expected to be sufficient to provide an initial immunologic response , whereas laboratory workers who do not handle orthopoxviruses could be vaccinated in the same fashion as other health care and public health workers who have at least 1 recent ( since 2003 ) documented successful vaccination ( 5 ) . this recommendation is based on the same premise as using the vaccine as prophylaxis for documented exposure to a smallpox - infected person . this practice was used regularly during the smallpox eradication program . because the average incubation period for vaccinia is 34 days shorter than the incubation period for smallpox , a person exposed to smallpox would have a 34 day window in which to be vaccinated with and immunologically respond to vaccinia , which also confers immunity to smallpox ( 6 ) compromised immune systems or cardiac risk factors that make vaccinees ineligible for vaccination are more likely to develop as they age ( 7 ) . most barriers to revaccination were related to medical conditions ( compromised immunity and/or exfoliative skin disorders ) that place vaccinees at high risk for adverse events to the currently licensed smallpox vaccine . the conditions are an added challenge for the aging pool of laboratory workers assigned to national variola testing sites ( 8) . currently unlicensed third - generation smallpox vaccines may be considered ( pending licensure ) as replacements to acam2000 ( sanofi pasteur biologics , lyon , france ) , the currently licensed vaccinia vaccine , for laboratory workers at national variola testing sites or perhaps an even broader population of laboratory workers throughout the united states . third - generation vaccines are nonreplicating and safer in populations that might have contraindications to traditional vaccines ( 911 ) . the risk to the us population from a release of smallpox this reduced risk stems not from a lower threat from terrorism but from the existence of a stockpile of the new acam2000 smallpox vaccine , in addition to a cadre of health care and public health professionals who could be revaccinated quickly and mobilized accordingly ( 12 ) . more research on the immunogenicity of smallpox vaccine is needed but is challenged by the absence of smallpox disease to test the efficacy of vaccination . researchers now appreciate that the complex mechanism of the immune response to vaccinia and/or smallpox infection might lead to better treatment options for infectious and autoimmune diseases ( 7 ) . future opportunities may arise to challenge the vaccine with the actual virus to measure vaccine efficacy and provide sound recommendations to protect all public health and health care responders against smallpox ( 13 ) . in the meantime , ensuring that recommendations created to protect some populations are properly interpreted and applied is important to protecting the most vulnerable persons without exposing others to unnecessary harm .
to evaluate the need to revaccinate laboratory workers against smallpox , we assessed regular revaccination at the us laboratory response network s variola testing sites by examining barriers to revaccination and the potential for persistence of immunity . our data do not provide evidence to suggest prolonging the recommended interval for revaccination .
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compromised renal functions and previous central nervous system ( cns ) disease have been shown to predispose to this neurotoxicity . we describe a case of acute transient encephalopathy in a patient treated with ceftriaxonefor enteric fever infection . the present case illustrates the diagnostic challenges and management of this rare but potentially severe side effect of one of the most commonly prescribed parenteral antibiotics . an eight - year - old male child presented with a history of diarrhea and high - grade fever . the child was conscious , cooperative , well oriented to time , place and persons . the patient was hospitalized and started on ceftriaxone ( 1 g iv daily ) and intravenous fluids . after three days of treatment with iv ceftriaxone , child became afebrile but showed altered mental status with progressive apathy and somnolence . the patient was referred to the dyanand medical college , ludhiana ( punjab ) . in the emergency department , the patient was not in acute distress , had no fever , was hemodynamically stable , but dehydrated . hb 12 g / dl [ normal range 12 to 15 g / dl ] , hct 38% [ normal range 35.0 to 49.0% ] , tlc 6 10/l l [ normal range 5 to 12 10/l l ] , dlc - n 62 [ normal range 6070% ] , l 27% [ normal range 2040% ] , plt 274 10/l l [ normal range 100 to 300 10/l ] , urea 14 mg / dl [ normal range 825 mg / dl ] , cr 0.6 mg / dl [ normal range 0.51.7 mg / dl ] , na / k 139/4 [ normal range 135147/ 3.55 meq / dl ] , urinalysis revealed no bacteriuria and pyuria , tsb / dsb 0.77/0 [ normal range 0.11.0/ < 0.2 mg / dl ] , sgot / pt 44/23 [ normal range 1147/ 753 iu / l ] , stoolr / e , 2d mri scan of brain did not reveal acute stroke . the patient 's neurological status improved and three days later he was again alert and oriented . the proposed mechanisms include a decrease in -amino butyric acid ( gaba)-mediated inhibition and cephalosporin - mediated release of cytokines . in fact , cephalosporins may decrease gaba release from nerve terminals , increase excitatory amino acid release , and exert a competitive antagonism with gaba . alternatively , cephalosporin treatment has been proposed to induce endotoxin release , which generates cytokines liberation , such as tumor necrosis factor- , a proinflammatory cytokine implicated in septic encephalopathy . pre - existing cns abnormalities have been indicated as a risk factor for -lactams encephalopathy . in this was not the case in our patient , who presented with enteric fever and dehydration corrected with intravenous fluids . in fact , the temporal association of the encephalopathy induction and resolution with ceftriaxone administration and withdrawal makes this antibiotic highly likely to be responsible for the encephalopathy . moreover , the temporal pattern is in accordance with previous publications reporting cephalosporin neurotoxicity , with a latency of one to ten days after drug initiation and regression of all neurological symptoms within two to seven days following ceftriaxone treatment suspension . we could establish a probable causal relationship between ceftriaxone and the encephalopathy ( naranjo score 6 ) . the severity assessment revealed the adr to be moderate , suggesting that required therapeutic intervention and hospitalization prolonged by 1 day but resolved in 24 h or change in drug therapy or specific treatment to prevent a further outcome . since this patient did not have a history of any such reaction due to ceftriaxone , this adverse drug reaction was unpreventable . we describe a case of ceftriaxone - induced acute reversible encephalopathy in a patient treated for enteric fever infection . early recognition of this complication is particularly relevant as discontinuation of ceftriaxone reverts the neurological syndrome .
ceftriaxone is a commonly used , third - generation cephalosporin . encephalopathy is a rare side effect of third- and fourth - generation cephalosporins . renal failure and previous disease of the central nervous system predispose to this neurotoxicity . we describe a case of acute transient encephalopathy in a patient treated with ceftriaxone for enteric fever infection . early detection of this complication is relevant given that stopping the drug usually reverts the neurological syndrome .
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a 2-year - old asian indian female presented to us with mild fever and swelling of the right upper lid of 10 days duration . there was no history of preceding viral illness or significant medical history necessitating treatment with antibiotics . cutaneous anthrax was unlikely as there was no history of unexplained cattle death in her environment . on examination , the child had low - grade fever and there were no other skin lesions . ophthalmological examination revealed right upper lid edema with a large black necrotic area of the lid which was adherent to the underlying tissues . the child was examined by a pediatrician to rule out any other focus of infection . microscopic examination of the skin biopsy revealed staphylococci and hence cutaneous anthrax was ruled out . the child was started on intravenous cefotaxime for a week with resolution of fever and the necrotic area turned to a well - defined eschar with no edema and induration . after 2 weeks , the child underwent escharotomy with wound debridement and full thickness skin graft from the groin [ fig . 2 ] . under general anesthesia , the groin area was cleaned and draped . the eschar on the lid was found to be partial thickness , was excised in toto , and the wound margins were debrided . the harvested skin was placed over the lid defect and sutured with 6 - 0 prolene . clinical photograph of the child showing large black necrotic area of the right upper lid adherent to the underlying tissues with surrounding erythema and edema and no discharge immediate postoperative clinical photograph showing full thickness skin graft from the groin postoperative photograph at 1 week showing healthy well - taken graft bacterial invasion of the arteries in the dermis and subcutaneous tissues produces a necrotizing vasculitis . the characteristic clinical appearance of eg is a red macule that progresses to a nodular or ulcerative lesion with central area of necrosis surrounded by erythema . bullae develop subsequently and become filled with mucopurulent or serosanguinous fluid . in the end stage , the lesions become hemorrhagic and slough off , leading to a necrotic eschar . progression through these stages is rapid , typically occurring within 1224 h. there are few reports of this condition developing in healthy individuals without any predisposing factors . usually , eg is associated with bacteremia , but can also occur in the absence of it . classic eg rarely involves the periocular tissues and to our knowledge , only a few such cases have been described in the literature . maccheron et al . presented a case of eg that led to orbital cellulitis and panophthalmitis . inamadar et al . described a diabetic individual who developed severe periorbital eg after suffering a laceration to the forehead . ghosheh and kathuria reported a case of bilateral periorbital eg in a diabetic male with renal failure . the mortality rate in nonsepticemic cases varies between 0% and 15% compared with 2096% for those associated with septicemia . the closest differential diagnosis in our case was necrotizing fasciitis , but on the basis of clinical features and negative blood cultures , a diagnosis of eg was entertained in this case . the diagnosis of necrotizing fasciitis depends on clinical features , blood cultures , and gram stain to identify causative organisms and these patients usually have septicemia with positive blood cultures . the eschar formed following antibiotic administration was a full thickness eschar adherent to surrounding tissues and the lesion caused ectropion and mechanical ptosis , which blocked the pupil . considering the possible complications of scarring including entropion or ectropion , trichiasis , corneal exposure , and amblyopia in the child , surgical intervention was indicated . to the best of our knowledge , there are no reports of skin grafting being done as a treatment modality for eg . our patient was atypical in that eg was due to methicillin - resistant staphylococcal infection in contrast to all the four reports where there was pseudomonas infection . the case also highlights the need of early surgical intervention in such circumstances so as the probable sequelae of scarring of upper eye lid , resulting in mechanical ptosis which can result in stimulus deprivation amblyopia can be prevented . 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 .
ecthyma gangrenosum ( eg ) is a cutaneous infection which usually occurs in immunocompromised patients . we report a case of eg of the eyelid treated with escharotomy and skin grafting , highlighting the importance of surgical management . a 2-year - old asian indian female presented to us with right upper lid edema with a large necrotic area . the child received intravenous cefotaxime for a week and the necrotic area turned to a well - defined eschar . escharotomy with wound debridement and skin grafting was done . the present case highlights the importance of surgical intervention to prevent the sequelae of scarring of upper lid .
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epilepsy is associated with a two- to three - fold increase in mortality among patients compared with the general population . sudden unexpected death in epilepsy ( sudep ) is one of the most frequent causes of death among patients with epilepsy . there is strong evidence suggesting that sudden unexpected death in epilepsy ( sudep ) is a seizure - related phenomenon , , , . the first description of this phenomenon was introduced by russell in 1906 . since then , several cases have been reported in the literature presenting with a drop in heart rate or asystole during the seizure . bradycardia and asystole resulted from increased parasympathetic flow through the vagus nerve , which originates in the nucleus ambiguous and dorsal nucleus of the vagus in the medulla . on the basis of one study , the incidence of sudep ranges from 1:1000 and 1:2000 person - years to 1:200 person - years , . according to a recent revised definition , sudep consists of sudden , unexpected , witnessed or unwitnessed , nontraumatic and nondrowning death in patients with epilepsy , with or without evidence of a seizure , excluding documented status epilepticus ( seizure duration > 30 min or seizures without recovery in between ) and asphyxia ; if postmortem examination does not reveal a cause of death , the diagnosis is definite sudep , and if there is a preexisting condition before or after autopsy , which could have contributed to the death , it is classified as sudep plus . strong risk factors for sudep include young age , early onset of seizures , the presence of generalized tonic clonic seizures , male sex , and bedtime occurrence . less significant risk factors for sudep include the prone position , one or more subtherapeutic blood levels , sleep occurrence , and a structural brain lesion . the underlying pathophysiologic mechanisms for sudep are not completely understood , but autonomic dysfunction ; ictal arrhythmias , ictal bradyarrhythmia , and asystole , ; neurogenic pulmonary edema ; and ictal central or obstructive apnea , , were introduced in the literature . in this report , we describe two patients with seizure - associated asystole monitored by simultaneous video electroencephalography electrocardiography . a thirteen - year - old right - handed male with seizure disorder was admitted for a presurgical assessment . there was a history of neonatal hypoglycemia during an apparently normal vaginal delivery . he was started on antiepileptic drugs , but the second seizure appeared six months later . the seizures typically consisted of blurred vision and upward gaze followed by a loss of consciousness . his mri showed near symmetrical signal abnormality at parietooccipital regions bilaterally ( parasagittal aspect ) accompanied by mild gliosis and volume loss ( fig . , there was bilateral rhythmic activity maximum to the left associated with right - side clonic jerk and head and eye deviation to the right that secondarily generalized and was followed by 16 s of asystole at the end of the seizure ( fig . 1 , fig . 2 , fig . 3 ) . interictal abnormality consisted of bilateral spike - and - wave and bilateral slow activity maximum in the right posterior head region . at the end of monitoring , an anticonvulsant drug regimen of valproic acid and levetiracetam was started , and cardiology consultation was suggested . a 42-year - old right - handed male with seizure disorder was admitted for presurgical assessment . his epilepsy was due to penetrating head trauma in the left frontal lobe from a shell injury . since then , he had been experiencing episodes of intense fear followed by generalized tonic clonic movements . neurologic examination included mild paresis in the right upper extremity and in the distal part of the right lower extremity in the range of 12/5 . the very first clinical manifestation was after the initial eeg changes and consisted of a generalized tonic afterwards , the sa arrest took place and lasted about 1 min ( see fig . 5 , fig . 6 , the very first eeg change started with 5-hz spike slow waves over the left parasagittal area with the maximum amplitude on c3 and f3 . the interictal abnormality consisted of delta waves seen at p3 , c3 , and f3 . considering his cardiac arrest during the seizure , a cardiac consultation was done , and a pacemaker was implanted . during 24 months of antiepileptic regimen , the frequency of seizures was reduced remarkably . theoretically , these asystoles could have a role in the incidence of sudep , meaning that the presence of ictal bradycardia is a risk factor for sudep . furthermore , in cases of epileptic cardiac dysrhythmia , isolated eeg or ecg recording may prove insufficient , and prolonged simultaneous eeg / ecg monitoring may be required . only simultaneous eeg and ecg recording will reveal a possible cerebral origin of arrhythmias in these patients who are , typically , young . attaining the correct diagnosis is essential because appropriate treatment may prevent cardiogenic sudep , which is thought to be related to potentially lethal arrhythmias , such as asystole induced by epileptic seizures , and to prevent the cardiac side effects of specific antiepileptic drugs . in conclusion , cardiological investigation should be included in epilepsy management to search for abnormalities of hr or ischemic events . in addition , it can provide an appropriate guideline in pharmacotherapy since certain types of drugs including carbamazepine , phenytoin , benzodiazepine , and barbiturates should only be used with caution by patients with epilepsy who have cardiac dysfunction . increasing knowledge about sudep risk factors can have a significant preventive role . moreover , strategies such as taking a detailed cardiovascular history to get the comprehensive clinical picture including a detailed history of symptoms , risk factors , and prior cardiac findings should be undertaken .
ictal asystole is a rare , probably underestimated manifestation of epileptic seizures whose pathophysiology is still debated . this report describes two patients who had cardiac asystole at the end of their seizure . the first patient was a 13-year - old boy with complex partial seizures .. his mri showed symmetrical signal abnormality in the bilateral parietooccipital lobe accompanied by mild gliosis and volume loss . during a 3-day long - term video - eeg monitoring , he had cardiac arrest at the end of one of his seizures that was secondarily generalized . the second one was a 42-year - old veteran with penetrating head trauma in the left frontal lobe due to shell injury . during long - term video - eeg monitoring , he had one generalized tonic clonic seizure accompanied by bradycardia and cardiac asystole . asystoles could have a role in the incidence of sudden unexpected death in epilepsy ( sudep ) , meaning that the presence of ictal bradycardia is a risk factor for sudep . in cases of epileptic cardiac dysrhythmia , prolonged simultaneous eeg / ecg monitoring may be required . cardiological investigation should be included in epilepsy management .
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oral infections of geotrichum candidum are clinically similar to candidiasis and commonly associated with diabetes mellitus and hiv infection , . cases of dissemination and fungemia are reported in patients with chronic and acute myeloid leukemia , , , , , , . old women post - partum with isolated renal calculi and renal fungal bezoar attributed to geotrichum candidum and to illustrate the diagnostic dilemmas . old women presented with history of left flank pain and intermittent fever since 15 days . she was evaluated elsewhere with contrast enhanced computerized tomography ( ct ) scan which revealed contracted left kidney with 2 calculi in the lower and middle calyx of 89 mm each with intrapelvic mass and multiple air pockets in the renal pelvis ( fig . 2 ) . she had undergone cytoscopy and left dj stenting elsewhere but continued to have fever and flank pain when she was presented to us . after routine investigation , patient was started on 3rd generation cephalosporin and she underwent left percutaneous nephrolithotripsy ( pcnl ) which revealed brownish gray material with 2 calculi . gross specimen consists of multiple irregular gray brown tissue bits , largest measuring 0.5 cm0.5 cm and cut portion showed gray brown areas . section showed fungal ball containing aggregates of macerated , distorted fungal hyphae with some showing acute angle branching surrounded by cell debris and neutrophils . both urine and biopsy material sent to mycology laboratory for culture investigation were inoculated on sabourauds dextrose agar ( hi - media laboratories ltd . , mumbai ) and incubated at 37 c and 28 c which grew a rapidly growing fungus with flat , white to creamy having a smooth texture later becoming hairy consistent with geotrichum candidum ( fig . geotrichum candidum was morphologically identified by the presence of true hyphae , hyaline smooth , one - celled , subglobose to cylindrical , slimy arthroconidia and the lack of blastoconidia . the arthroconidia vary in size and germinate at one end giving a hockey stick appearance ( fig . 4 ) . biochemical identification was carried out in the mycology laboratory , kasturba medical college , manipal using both conventional and api 20c yeast identification system ( biomerieux inc . ) . it was further differentiated from trichosporon by the absence of urea utilization and inability to assimilate carbohydrate ; maltose , sucrose , lactose , cellobiose , inositol raffinose and trehalose . antifungal susceptibility testing for the isolate was performed according to the clinical and laboratory standards institute ( clsi ) document m38-a2 . the mic90 ( minimum inhibitory concentration ) for amphotericin , fluconazole , itraconazole and voriconazole were found to be 0.125 g / ml , 16 g / ml , 4 g / ml and 0.25 g / ml respectively . patient continued to have fever in the post - operative period and responded only after starting intravenous itraconazole 200 mg bd for 2 days followed by oral itraconazole 200 mg bd and continued for 6 weeks . during the follow up , a repeat ct done showed complete clearance of the fungal material from the left kidney . the saprophytic colonization of a preformed cavity by conglomerate of fungal mycelia without invasion of adjacent tissue is termed as fungal ball or fungal bezoar . renal colic can be caused by passage of fungal ball that obstruct the collecting system . genitourinary tract is rarely a site of primary fungal infection with exception of candida species , however it may be involved as a result or part of systemic infection . candidal infection can cause pyelonephritis , abscess , papillary necrosis and obstruction with fever and flank pain , . genitourinary fungal infections are usually encountered as a part of disseminated disease in immunocompromised host ( aids , corticosteroids , malignancy , neutropenia ) , , , , , , . fungal balls also called and fungal bezoars or accretions are known to cause ureteral and uretro - pelvic junction obstruction . diagnosis is established by identification the of fungi in urine and imaging studies using ( computerized tomography , ultra sonography , intravenous urography ) that document obstructive uropathy and soft tissue density with in renal collecting system . have successfully managed the removal of bilateral renal pelvis mycotic bezoars using a mechanical thrombectomy device followed by antifungal renal pelvis irrigation . percutaneous nephrostomy , tract dilation and fluoroscopically guided extraction of renal fungal ball under epidural anesthesia is described by doemeny et al . . the outcome of geotrichum infections depend on the degree of tissue invasion by the organism and the immune status of the host . sheehy et al . suggested that geotrichum lack virulence and ability to colonize renal tubules based on the rarity of disseminated disease , lack of tissue invasion and their rapid clearance in most of the case reported . its incidence may be under reported since it can be misdiagnosed histopathologically as candida , aspergillus or trichosporon . this possibility of misinterpretation highlights the importance of obtaining repeated fungal cultures in addition to histopathological examination . we hereby reiterate the pathogenic potential of geotrichum candidum and report its role in causing renal fungal ball .
geotrichum candidum is yeast like fungi that cause infections in immunocompromised patients . we report a case of renal fungal ball with geotrichum candidum in a 27 yr . old women post - partum . this case to our knowledge is the first case of renal fungal bezoar due to geotrichum candidum reported in india .
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many different chemotherapy regimens were therefore developed.13 however , little is known on the feasibility and efficiency of chemotherapy for these cancer types in patients with severe renal failure.46 only case reports on the pharmacology of irinotecan in patients with colon or rectal cancer are available at present.711 we present the first case of combination chemotherapy in metastatic gastroesophageal cancer in a dialysis patient . a 73-year - old patient with a longstanding history of ischemic heart disease had been on dialysis for two years for vascular renal insufficiency . in september 2004 he was admitted for gastrointestinal blood loss . ca 19.9 was very high : 24925 u / ml ( nl < 37 u / ml ) . the patient was started on chemotherapy , the regimen consisting of l - leukovorin 250 mg / m , irinotecan 50 mg / m followed by 5-fluorouracil ( 5 fu ) 2 g / m/24 h , six weeks out of eight.1213 there was neither significant nausea nor diarrhea . dialysis was continued three times a week , ( the patient was on a monday wednesday friday schedule of dialysis ) and chemotherapy was given on the monday , just after dialysis . after four weeks of chemotherapy he also underwent a right carotid endarterectomy for an intercurrent transient ischemic attack in the right carotid region . six months after starting chemotherapy the ct scan of the liver showed a complete response of the numerous metastases . ( table 1 ) nine months after initiation of treatment , however , the liver metastasis and tumor marker were progressive again . doses of chemotherapy were based on a number of case reports on paclitaxel for ovarian cancer in dialysis patients.14,15 ct scan after two months showed further progressive disease . the increase in solid tumors in a patient undergoing dialysis poses specific problems,16 especially in the choice and pharmacology of anticancer drugs , bearing in mind that all of these drugs were developed in patients with normal liver and kidney function . for gastric cancer , 5fu has always been the backbone of treatment.1,2 in chronic hemodialysis , there are some data on dose reductions with 5fu weekly.17,18 for gastric cancer , combination chemotherapy is , however , necessary to obtain prolonged disease control and even for prolonging overall survival.1,2 combinations of 5fu + cisplatin and either docetaxel or epirubicin have therefore become standard chemotherapy regimens in gastric cancer.19,20 besides the aforementioned regimens , irinotecan - based combinations were shown to be active in first21 and second line gastric cancer.22 its equivalence ( in combination with 5fu ) in first - line metastatic gastric cancer was recently established in two studies , both comparing this regimen with a combination chemotherapy with cisplatin and 5fu.23,24 irinotecan is metabolized in the liver to its active metabolite sn-38 , followed by biliary excretion.6 there is no significant renal elimination . the drug was evaluated in patients with serum creatinin between 1.6 and 5 mg / dl and no unexpected toxicities were seen.25 there are a number of case reports on the use of irinotecan during hemodialysis , all of which are on patients with metastatic colon cancer . a first report mentions the use of irinotecan at a dose of 50 mg / m without significant toxicity.7 in two other case reports on dialysis patients , both patients were started with irinotecan at 50 mg / m . both reports mention that by increasing the dose , prohibitive diarrhea was the consequence.8,11 the worst outcome in higher irinotecan doses ( above 125 mg / m ) was demonstrated in two other dialysis patients , where these dosages led to extreme gi toxicities and even death.9 it can be concluded that irinotecan in terminal renal insufficiency should not be given at a dose above 50 mg / m . korean authors have made pharmacologic evaluations on the use of irinotecan in small - cell lung cancer patients during dialysis . they noted however that these doses were only feasible in patients of korean descent.26 there is a very recent case report on the combination of irinotecan at a dose of 50 mg / m weekly combined with fu1600 mg / m/24 h / week , leading to disease stabilization at six months in a dialysis patients with diffuse bone , cerebral and liver metastases of colon cancer.10 our case report builds on this knowledge of the use of irinotecan in metastatic colorectal cancer during dialysis . this case report discusses both the weekly dose of irinotecan and the 24-hour administration of 5fu in a gastroesophageal cancer patient . this is the first report on the efficacy of irinotecan- and fluorouracil - based chemotherapy in a dialysis patient with liver metastases of a gastroesophageal carcinoma . combination chemotherapy of irinotecan and fu was extremely well tolerated , without significant delays in administration . it produced radiographically complete remission of the liver metastases , and a normalization of ca 19 - 9 tumor marker , leading to a remarkable overall survival .
we present the first case report of a complete response of metastatic gastroesophageal cancer in a chronic hemodialysis patient with irinotecan - based chemotherapy . an elderly dialysis patient presented with diffuse liver metastases by a gastroesophageal adenocarcinoma . he received combination chemotherapy with 5 fluorouracil and irinotecan . after six months of chemotherapy , liver scans show complete remission . the principles , practice , and experience of chemotherapy with irinotecan during dialysis are discussed .
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it is a major social and economic scourge in tropics and subtropics of africa , asia , western pacific and parts of america affecting over 120 million people in 80 countries . the disease is endemic all over india especially in uttar pradesh , bihar , jharkhand , andhra pradesh , orissa , tamil nadu , kerala and gujarat . there are at least six million attacks of acute filarial disease per year and 45 million persons are currently having one or more chronic filarial lesions . . conventional mode of diagnosis of filariasis is by demonstration of microfilaria in peripheral blood smear . despite high incidence , it is infrequent to find microfilariae in fine needle aspiration cytology ( fnac ) smears and body fluids . the literature contains a few reports of microfilariae found in various locations including thyroid nodule , skin and soft tissue swelling , epididymis , breast,[268 ] salivary gland , cervicovaginal smear , ovarian cyst , urine , lymph node , and effusion fluids . the aim of present study was to assess the role of fnac in diagnosis of filariasis in asymptomatic patients having superficial lumps . the study was conducted at the department of pathology in collaboration with the departments of medicine and surgery at b.r.d . medical college , gorakhpur during a period of two years i.e. 2006 - 2007 . a total of 250 cases , with age ranging from 15 - 80 years , having swellings at various sites were included in the present study . aspiration was made by technique of martin and ellis . in case of cystic lesions , cyst content was aspirated and smears prepared from cyst fluid after cytocentrifugation were studied along with the aspiration performed from cyst wall . these smears were wet fixed immediately in 95% alcohol and stained by hematoxylin and eosin and papanicolaou stain . this study was conducted on 24 cases of filariasis diagnosed on routine fnac material from various sites . out of these 24 cases , maximum cases of filariasis were reported in breast swelling ( eight cases ) , followed by lymph nodes ( six cases ) , scrotal swellings ( four cases ) , thyroid swellings ( three cases ) , soft tissue swellings ( two cases ) and ascitic fluid ( one case ) . clinical presentations of these cases were variable which included swelling , pain , fever and erythema [ table 1 ] . showing clinical profile , cytological findings and associated conditions of 24 cases smears revealed sheathed microfilariae , tails of which were free from nuclei and many had graceful curves . thick and thin blood smear examination of nocturnal venous blood revealed microfilariae of wuchereria bancrofti in three out of 24 cases . microscopic examination of breast swellings showed sheathed microfilaria along with few groups of benign ductal epithelial cells , myoepithelial cells , bare nuclei , few fragments of fibrofatty tissue and inflammatory cells comprising of eosinophils and neutrophils [ figure 1 ] . two cases of breast lumps showed epithelioid non - necrotising granuloma without giant cells and plasma cells . fnac smear of breast swelling showing sheathed microfilaria along with inflammatory cells thyroid aspirates revealed few groups of follicular cells in the background of colloid . in between follicular groups , aspirate from scrotal swelling showed numerous coiled and uncoiled sheathed microfilariae along with neutrophils , eosinophils and few lymphocytes [ figure 2 ] . fnac smear of scrotal swelling showing sheathed microfilariae along with polymorphs , macrophages and eosinophils lymph node aspirates showed sheathed microfilariae in the background of mixed population of lymphoid cells comprising of mature lymphocytes , centrocytes , centroblasts , dendritic cells and few eosinophils . cytological findings of soft tissue swellings showed microfilariae along with neutrophils , eosinophils and granular debris . cell adherence of inflammatory cells and macrophages to microfilariae was seen in three out of 24 cases . filariasis is a major public health problem in tropical countries , including india . in endemic areas like eastern part of uttar pradesh , a majority of infected individuals in filarial endemic communities were asymptomatic . in the present study maximum cases ( eight out of 24 cases ) many authors have reported microfilariae in breast lumps by fnac smears.[2468 ] aspirates from lymph nodes ( five out of 24 ) , demonstrated microfilariae in a background of reactive lymphoid cells . the lymphatic vessels of spermatic cord appear to be common and perhaps the principal site of adult wuchereria bancrofti in men with asymptomatic microfilaremia . occurrence of living w bancrofti in scrotal area of men was demonstrated by noroes et al . two cases of soft tissue swellings and one case of ascitic fluid showed microfilaria along with inflammatory cell including eosinophils , lymphocytes and macrophages . out of 24 cases showing microfilariae in fnac smear examination , blood eosinophilia was present in eight cases , of which microfilaremia in nocturnal venous blood smear examination was observed in three cases only . findings are consistent with observation made by others , who reported that filariasis can exist without microfilaremia . significant adherence of inflammatory cells and macrophages to microfilariae was present in three out of 24 cases . despite high incidence of filariasis , microfilaria in fine needle aspiration cytology is not a very common finding . careful screening of fnac smears undoubtedly the demonstration of parasite , in aspirate , play a significant role in recognition of disease and institution of specific treatment , thus obviate the severe manifestations of lymphatic filariasis .
background : filariasis is a major health problem in tropical countries including india . fine needle aspiration cytology plays an important role in prompt recognition of disease.aim:to assess the role of fine needle aspiration cytology ( fnac ) in diagnosis of filariasis at all possible sites.materials and methods : total 250 cases of superficial swellings at various sites were subjected to fine needle aspiration cytology.results:out of 250 cases , 24 cases of filariasis were detected which include breast lumps ( 8 cases ) , lymph nodes ( 6 cases ) , scrotal swellings ( 4 cases ) , thyroid swellings ( 3 cases ) , soft tissue swellings ( 2 cases ) and ascitic fluid ( 1 case ) . eosinophilia was present in 8 out of 24 cases with a percentage ranging from 12 - 24% . significant adherence of inflammatory cells and macrophages to microfilariae was present in 3 out of 24 cases.conclusions:in endemic areas , it should be considered one of the differential diagnoses of a superficial swelling . careful screening of fnac smears help in detecting microfilaria even in asymptomatic patients and thus plays a significant role in recognition of the disease and institution of specific treatment .
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anti--tubulin mouse monoclonal antibody ( sigma - aldrich corp ) , anti - arf6 mouse monoclonal antibody ( santa cruz biotechnology , inc ) , anti - rab11 rabbit polyclonal antibody , anti - tfr mouse monoclonal antibody ( invitrogen corp ) , anti - eea-1 mouse monoclonal antibody and anti - gm130 mouse monoclonal antibody ( bd biosciences ) were obtained commercially . s2 was performed with a fluorophore - conjugated anti - tfr antibody that was generated by using the zenon antibody labeling kit ( invitrogen corp ) . using this antibody slightly enhanced the tfr signals at the plasma membrane ( figs . s1 and s2 ) . all of the procedures used to perform cell culture , immunoblotting and the immunofluorescence analyses have been described elsewhere . anti--tubulin mouse monoclonal antibody ( sigma - aldrich corp ) , anti - arf6 mouse monoclonal antibody ( santa cruz biotechnology , inc ) , anti - rab11 rabbit polyclonal antibody , anti - tfr mouse monoclonal antibody ( invitrogen corp ) , anti - eea-1 mouse monoclonal antibody and anti - gm130 mouse monoclonal antibody ( bd biosciences ) were obtained commercially . s2 was performed with a fluorophore - conjugated anti - tfr antibody that was generated by using the zenon antibody labeling kit ( invitrogen corp ) . using this antibody slightly enhanced the tfr signals at the plasma membrane ( figs . s1 and s2 ) . all of the procedures used to perform cell culture , immunoblotting and the immunofluorescence analyses have been described elsewhere .
recycling endosomes are key platforms for endocytic recycling that return internalized molecules back to the plasma membrane . to determine how recycling endosomes perform their functions , searching for proteins and lipids that specifically localized at recycling endosomes has often been performed by colocalization analyses between candidate molecules and conventional recycling endosome markers . however , it remains unclear whether all the conventional markers have identical localizations . here we report finding that three well - known recycling endosome markers , i.e. , arf6 , rab11 and transferrin receptor ( tfr ) , have different intracellular localizations in pc12 cells . the results of immunofluorescence analyses showed that the signals of endogenous arf6 , rab11 and tfr in nerve growth factor - stimulated pc12 cells generally differed , although there was some overlapping . our findings provide new information about recycling endosome markers , and they highlight the heterogeneity of recycling endosomes .
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bspp : bisulfite padlock probe ; charm : comprehensive high - throughput arrays for relative methylation ; cimp : cpg island methylator phenotype ; esc : embryonic stem cell ; fda : food and drug administration ; help : hpaii tiny fragment enrichment by ligation - mediated pcr ; mca : methylated cpg island amplification ; mcam : methylated cpg island amplification microarray ; medip : methylated dna immunoprecipitation ; mira : methylated - cpg island recovery assay ; mscc : methyl - sensitive cut counting ; pcr : polymerase chain reaction ; rrbs : reduced representation bisulfite sequencing . the authors are supported by the leukemia specialized program of research excellence grant p50 ca100632 .
dna methylation of promoter cpg islands is strongly associated with gene silencing and is known as a frequent cause of loss of expression of tumor suppressor genes , as well as other genes involved in tumor formation . dna methylation of driver genes is very likely outnumbered by the number of methylated passenger genes , though these can be useful as tumor markers . much of what is known about the importance of dna methylation in cancer was gained through small- and moderate - scale analysis of gene promoters and tumor samples . a much better understanding of the role of dna methylation in cancer , either as a marker of disease or as an active driver of tumorigenesis , will likely be gained from genome - wide studies of this modification in normal and malignant cells . this goal has become more attainable with the recent introduction of large - scale genome analysis methodologies and these have been modified to allow for investigation of dna methylation . several research groups have been formed to coordinate efforts and apply these methodologies to decipher the methylome of healthy and diseased tissues . in this article we review technological advances in genome - wide methylation profiling .
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as a service to our authors and readers , this journal provides supporting information supplied by the authors . such materials are peer reviewed and may be reorganized for online delivery , but are not copyedited or typeset . technical support issues arising from supporting information ( other than missing files ) should be addressed to the authors .
abstractmacrocyclization can be used to constrain peptides in their bioactive conformations , thereby supporting target affinity and bioactivity . in particular , for the targeting of challenging protein protein interactions , macrocyclic peptides have proven to be very useful . available approaches focus on the stabilization of helices , which limits their general applicability . here we report for the first time on the use of ringclosing alkyne metathesis for the stabilization of an irregular peptide secondary structure . a small library of alkynecrosslinked peptides provided a number of derivatives with improved target affinity relative to the linear parent peptide . in addition , we report the crystal structure of the highestaffinity derivative in a complex with its protein target 1433. it can be expected that the alkynebased macrocyclization of irregular binding epitopes should give rise to new scaffolds suitable for targeting of currently intractable proteins .
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in 2009 , a 52-year - old woman presented with a single lesion on her nose , which started as a papule , referred to sedighe tahereh clinic , isfahan , iran . the lesion had existed for a period of 14 months and was slowly increasing in size , enlarging to a plaque . the diagnosis of leishmaniasis was confirmed with a positive smear of the lesion showing leishmania bodies about 1 year before . all five members of her family had had a history of proven leishmaniasis . in the past medical history , the patient was a renal failure case since 11 years before and received a renal transplant 4 years after the diagnosis of renal failure . she was receiving oral mycophenolate mofetil ( 2 g daily ) and cyclosporine ( 100 mg daily ) . a 33 cm indurated ulcer with elevated borders was present on the tip of her nose ( figure 1 ) . her therapeutic plan was intralesional glucantime injection ( approximately 1 ml of 1.5 g vial per week , intralesional injection ) . after completing a therapeutic course of 20 sessions receiving intralesional glucantime injections , she was considered as glucantime therapy resistant . the occurrence of malignant neoplasms in sites of scars is an infrequent but well - known phenomenon.5 although the coexistence of cutaneous leishmaniasis and bcc may have been coincidental , some studies suggest that an association between these two entities does exist.6 leishmaniasis can directly or indirectly alter the diagnosis and course of different malignancies.7 there are reports of bcc in chronic leg ulcers.8 cases of bcc developing in a leishmania scar have also been documented,9 but to our knowledge , cases of both leishmaniasis and bcc in the same site and the same lesion are rare.10 however , in this case , solid organ transplantation and long term immuno suppressive therapy should be considered as risk factors for malignancy . advances in effective immuno suppression after organ transplantation have led to increased risk of malignancies , particularly skin cancers11 including squamous cell carcinoma , basal bcc and malignant melanoma.12 thus , malignancies should be considered in the differential diagnosis of leishmaniasis lesions difficult to treat . the possible role of cutaneous leishmaniasis , as a predisposing factor for skin cancer , should also be kept in mind . aa was the main therapeutic physician and helped write the manuscript . i m and pk contributed in writing the manuscript .
leishmaniasis is a protozoan infection due to organisms of the genus leishmania . the differential diagnosis of cutaneous leishmaniasis includes arthropod bites , basal cell carcinoma ( bcc ) and other malignancies . bcc is the most common form of skin cancer . we present a case of cutaneous leishmaniasis resistant to standard intralesional glucantime injection in an immunocompromised patient , which was proved to be bcc after surgical excision .
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a 60-year - old man was admitted because of visual disturbances and ocular pain following penetrating keratoplasty in the right eye for a corneal chemical burn . on slit lamp examination , we observed a feather - like corneal opacity , corneal infiltrations , and epithelial defects . subconjuctival amphotericin b ( 1 mg ) , topical amphotericin b 0.125% , and levofloxaxin 0.5% were subsequently administered every hour for 16 weeks . the patient was restarted on and maintained on a topical amphotericin b treatment ( 0.125% twice daily ) for 1 year . in addition , levofloxaxin 0.5% ( three times daily ) and fluorometholone 0.1% ( twice daily ) were administered for 1 year . the lesion did not progress , but there was no change in corneal infiltration . on examination one year later , an elevated corneal lesion and increased corneal infiltration with a yellow color were observed despite the topical amphotericin b treatment . seven days later anterior chamber hypopyon and vitreous opacity on a b - scan were observed ( fig . we immediately performed an intravitreal injection of amphotericin b 5 g/0.1ml and vancomycin 1 mg/0.1ml . intravitreal voriconazole 80 g/0.1ml and ceftazidime 2 mg/0.1ml were injected and topical voriconazole 1% was administered every 2 hours . the patient discontinued the oral voriconazole because of abdominal pain , dry mouth , and scaling of the oral mucosa . the patient was treated topically for 6 weeks , after which the corneal lesion and endophthalmitis had improved . the patient subsequently underwent another penetrating keratoplasty and demonstrated a visual acuity of 0.1 at 10 months follow - up with no signs of recurrence . a 60-year - old man was referred to us because of a lack of improvement after a month of empiric treatment with topical amphotericin b for fungal keratitis . on our initial examination after 10 days the culture revealed no growth and the corneal epithelium appeared healed ; however , there was no change in corneal infiltration ( fig . because of a lack of improvement in the corneal lesion a month later , a new treatment regimen was initiated with topical voriconazole 1% administered every hour . six weeks later we observed a decreasing density of the infiltrate and healing of the corneal epithelium ; the topical voriconazole was decreased to twice daily . following completion of the therapy , complete healing of the corneal epithelium and resolution of the corneal infiltrate were observed ; however , the corneal opacity persisted ( fig . 2c ) voriconazole , a derivative of fluconazole , is a new triazole antifungal agent.4 like other triazoles , this voriconazole inhibits cytochrome p450 demethylase , which is essential for the synthesis of ergosterol . it is hypothesized that this adversely affects the permeability of the fungal cell membrane.7 voriconazole has excellent oral bioavailability and a broad spectrum of activity . therapeutic aqueous and vitreous levels are achieved after oral administration of voriconazole.8 voriconazole showed lower mics compared to other antifungal agents when tested against five corneal isolates of scedosporium apiospermum,2 and it had the best in vitro susceptibility profile for 34 common fungal pathogens compared to other antifungal agents.9 gao et al.10 reported that direct intravitreal voriconazole injections of 25 g / ml ( equivalent to 100 mg per injection in a human eye ) caused no electroretinographic or histopathologic abnormalities in rodent retinas . kramer et al.11 reported that intravitreal injection of voriconazole ( 100 g/0.1ml ) with pars plana vitrectomy was an effective therapy for aspergillus endophthalmitis . lee et al.7 reported a case of drug - resistant penicillium endophthalmitis that was treated with intravitreal voriconazole injection ( 50 g/ 0.1ml ) . in these cases , voriconazole has been shown to be highly effective against filamentous organisms and is more potent in invasive aspergillosis than amphotericin b.12,13 according to a report by jang et al.,5 voriconazole is also effective against candida chorioretinitis . ozbek et al.14 reported one case of alternaria keratitis that showed improvement with 1% topical voriconazole . in contrast , giaconi et al.15 reported two cases of fungal keratitis caused by fusarium oxysporum and colletotrichum dematium that did not respond to treatment with 1% topical voriconazole . our report indicates that 1% voriconazole is an effective treatment for candida spp . and unknown fungal keratitis . in conclusion , voriconazole is a new , promising therapy for fungal keratitis refractory to standard antifungal agents . nevertheless , more clinical trials will be necessary to investigate the effectiveness of systemic voriconazole and corneal transplantation .
we describe two patients with fungal keratitis refractory to standard antifungal therapy whose conditions were managed with voriconazole.the first case is a patient with endophthalmitis and corneal ulcer due to candida parapsilosis after receiving a corneal transplant . the patient was treated with amphotericin but showed no signs of improvement . topical voriconazole , oral voriconazole , and intravitreal voriconazole yielded signs of improvement . the second case is a 63-year - old male who underwent a month of empiric treatment with 0.2% topical amphotericin for fungal keratitis but showed no signs of improvement . treatment was then provided with 1% voriconazole . both cases showed effective treatment with voriconazole.voriconazole may be considered as a new method to treat fungal keratitis refractory to standard antifungal therapy .
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we attempted to estimate the perceived degree of urgency of the visit and to identify reasons for seeking non - urgent care in the ped by patients and parents . a prospective survey was completed by parents ( for children 17 and younger ) and patients ( 18 - 21 ) presenting to a suburban academic ped that sees approximately 15,000 patients per year . three hundred and five of 334 surveys were completed ( 91% response rate ) over a 3-month period . twenty - four percent of the chief complaints were perceived by those surveyed as emergent or possibly life - threatening , 23% were felt to be very urgent , and 52% were deemed somewhat urgent or minor . twenty - five percent of those with minor or somewhat urgent complaints arrived by ambulance . overall , 79% of those surveyed identified a primary care provider ( pcp ) for themselves or their child . of those , 54% had attempted to contact the pcp prior to coming to the ped . six percent of those who attempted to reach their primary care providers were able to contact them and 52% were told to come to the ped . more than half of patients and parents presenting to the ped believed they had minor or somewhat urgent complaints . while the majority of patients have a regular provider , limited access to timely primary care and convenience may make the ped a more attractive care option than primary care for many parents and patients .
objectives : pediatric emergency department ( ped ) patients often present with non - urgent complaints . we attempted to estimate the perceived degree of urgency of the visit and to identify reasons for seeking non - urgent care in the ped by patients and parents.methods:a prospective survey was completed by parents ( for children 17 and younger ) and patients ( 18 - 21 ) presenting to a suburban academic ped that sees approximately 15,000 patients per year . a convenience sample of participants was enrolled.results:three hundred and five of 334 surveys were completed ( 91% response rate ) over a 3-month period . twenty - four percent of the chief complaints were perceived by those surveyed as emergent or possibly life - threatening , 23% were felt to be very urgent , and 52% were deemed somewhat urgent or minor . twenty - five percent of those with minor or somewhat urgent complaints arrived by ambulance . weekend visits and minority race correlated with a lower degree of perceived urgency . overall , 79% of those surveyed identified a primary care provider ( pcp ) for themselves or their child . of those , 54% had attempted to contact the pcp prior to coming to the ped . six percent of those who attempted to reach their primary care providers were able to contact them and 52% were told to come to the ped.conclusions:more than half of patients and parents presenting to the ped believed they had minor or somewhat urgent complaints . while the majority of patients have a regular provider , limited access to timely primary care and convenience may make the ped a more attractive care option than primary care for many parents and patients .
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ocular cicatricial pemphigoid ( ocp ) is an uncommon , chronic autoimmune disease that affects mucous membranes , particularly the conjunctiva.1 the disease typically results in chronic conjunctivitis and causes conjunctival and corneal scarring , which can result in limbal stem cell deficiency and blindness.2 adequate control of ocular inflammation usually requires systemic as well as topical immunosuppressants . commonly used systemic medications include prednisone , methotrexate , mycophenolate mofetil ( mmf ) , and cyclophosphamide.3 the porphyrias are metabolic disorders caused by defective enzymes within the heme synthetic pathway.4 these defective enzymes cause an accumulation of intermediates from the heme synthetic pathway that results in various clinical manifestations.4,5 porphyria cutanea tarda is the most common of the porphyrias and results from a deficiency in uroporphyrin decarboxylase , which is the fifth enzyme in the heme synthetic pathway.6 this deficiency results in the accumulation of porphyrins in the liver and plasma . on exposure to light with a wavelength near 400 nm , the porphyrins enter an excited state that can lead to the damage of proteins , lipids , and basement membranes.6 this process results in blisters , fibrosis , and scarring of the skin in areas of the body exposed to sunlight.6 park et al7 have reported a case of a 31-year - old with cicatricial conjunctivitis who was biopsy negative for ocp and was later diagnosed with porphyria cutanea tarda ; in this case the patient s clinical symptoms significantly improved after initiating phlebotomy treatments . the present authors report a similar case , in which pathology and direct immunofluorescence confirmed a diagnosis of ocp and where the patient s clinical condition also improved significantly upon the diagnosis and treatment of porphyria cutanea tarda . a 64-year - old caucasian male complaining of redness and tearing for 3 years in both eyes was referred for evaluation of cicatricial conjunctivitis . he had been treated with tobramycin and dexamethasone ophthalmic ointment in both eyes as needed and doxycycline 100 mg by mouth daily with no improvement in his symptoms . on slit lamp examination the patient had subconjunctival fibrosis , symblepharon , forniceal foreshortening , and trichiasis in both eyes ( figure 1 ) . examination of the corneas revealed multiple punctate epithelial erosions . a schirmer s test was performed without anesthesia , showing 22 mm of wetting in the right eye and 13 mm in the left after 5 minutes . direct immunofluorescence studies of the conjunctival biopsy specimen revealed immunoglobulin g4 deposits in the basement membrane zone of the junctional area ( figure 2 ) , consistent with ocp . given the findings of subconjunctival fibrosis and symblepharon formation in both eyes , the patient was diagnosed with bilateral stage iii pemphigoid . the patient was started on methotrexate 15 mg by mouth weekly and prednisone 20 mg by mouth daily . the patient demonstrated gradual improvement in the conjunctival inflammation after starting the methotrexate and prednisone . the patient was subsequently tapered off the prednisone , while the methotrexate 15 mg by mouth weekly was continued . after 4 months of treatment with the methotrexate , the patient s conjunctival inflammation began to worsen and his regimen was subsequently changed from methotrexate to mmf 1000 mg by mouth twice daily . approximately 3 months after initiating mmf treatment , the patient was diagnosed with porphyria cutanea tarda . the patient s conjunctival inflammation appeared stable following initiation of the phlebotomy treatments and the mmf was subsequently discontinued . approximately 6 weeks following discontinuation of the mmf , the patient returned with mildly increased conjunctival injection and trichiasis in both eyes . epilation was performed and the patient was started on 1% prednisolone acetate ( one drop in both eyes twice daily ) . the patient s conjunctival inflammation stabilized and he was tapered down to one drop of 1% prednisolone acetate in both eyes once daily . since initiation of the phlebotomy treatments , the patient s conjunctival inflammation and subconjunctival fibrosis has remained quiescent for 4 months without requiring mmf ( figure 3 ) . ocp is believed to be an autoimmune disease of genetic predisposition , and it is likely that a second - hit environmental trigger is required to initiate onset of the disease.1 it has been thought this could include chemical exposure or microbial environmental triggers . the present case suggests that the patient s porphyria could be a causal factor associated with the ocp and could even have been the environmental trigger that stimulated the disease to occur . the porphyrins are present in plasma and therefore they would be present in the ocular surface vasculature.5 the ocular surface is constantly exposed to light . exposure to ultraviolet light would lead the porphyrins to enter into an excited state , resulting in inflammation and damage to the ocular surface . this process could be the trigger to either initiate or exacerbate ocp . in the present case , the patient s conjunctival inflammation was observed to significantly improve following initiation of treatment for his porphyria . a similar response was observed in the aforementioned case reported by park et al,7 although that particular patient was biopsy negative for ocp and was positive for hepatitis c virus infection ; in addition , the patient remained on methotrexate . the patient in the present case was unique in that he was biopsy positive for ocp and his clinical improvement was significant enough after starting phlebotomy treatments that his mmf was discontinued . the authors consider that this case , as well as the case reported by park et al,7 sheds new light on the search for the etiology of ocp and the subsequent treatment options for patients with this disease .
a 64-year - old caucasian male complaining of redness and tearing for 3 years in both eyes was referred for evaluation of cicatricial conjunctivitis . ocular cicatricial pemphigoid was suspected and this diagnosis was confirmed through biopsy . the patient s condition showed moderate improvement following treatment with methotrexate and mycophenolate mofetil . the patient was later diagnosed with porphyria cutanea tarda and phlebotomy treatments were subsequently initiated . the patient s ocular symptoms improved further after he began receiving these phlebotomy treatments , and conventional treatment was discontinued . the authors hypothesize that circulating porphyrins activated by ultraviolet light could be the cause of the ocular cicatricial pemphigoid in this patient .
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to identify the possible barriers to the implementation of pharmaceutical care among community and hospital pharmacists in enugu state using nsukka and enugu metropolis as a case study . the questionnaires were distributed to community and hospital pharmacists from designated areas during one of their quarterly meeting and their practice sites in 2009 . eighty completed questionnaires were collected with 22.8% from community pharmacists , and 77.2% from hospital pharmacists . the important barriers identified were lack of space , enough personnel in pharmacy to handle routine technical tasks , time , need for too much effort , and need for payment for services . the opinions on barriers to pharmaceutical care of pharmacists from community and hospital practice areas in these two metropolises of enugu state are majorly lack of time , space and routine technical task personnel .
objective : to identify the possible barriers to the implementation of pharmaceutical care among community and hospital pharmacists in enugu state using nsukka and enugu metropolis as a case study.method:a semi structured questionnaire was designed to carry out a cross sectional descriptive study . the questionnaires were distributed to community and hospital pharmacists from designated areas during one of their quarterly meeting and their practice sites in 2009.results:eighty completed questionnaires were collected with 22.8% from community pharmacists , and 77.2% from hospital pharmacists . the important barriers identified were lack of space , enough personnel in pharmacy to handle routine technical tasks , time , need for too much effort , and need for payment for services.conclusion:the opinions on barriers to pharmaceutical care of pharmacists from community and hospital practice areas in these two metropolises of enugu state are majorly lack of time , space and routine technical task personnel .
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in the previous issue of critical care , chase and coworkers reported on their implementation into clinical practice and evaluation of the specialized relative insulin nutrition table ( sprint ) . this is an improved protocol in the form of a wheel - based system to control blood glucose levels and nutritional intakes in intensive care patients , which was developed a few years ago . blood glucose has become a key biological parameter in critical care since publication of the study conducted by van den berghe and colleagues , who demonstrated decreased mortality in surgical intensive care patients in association with tight glycaemic control ( tgc ) , based on intensive insulin therapy . however , two negative studies were recently reported , which were interrupted early because of high rates of severe hypoglycaemia , namely the visep study and the as yet unpublished glucontrol trial . hence , there is currently much debate regarding the actual benefits of such a strategy in intensive care patients in terms of outcomes . it is also uncertain whether the results of the ongoing multicentre , open label , randomized controlled trial nice sugar of the effects of blood glucose management on 90-day all - cause mortality in a heterogeneous population of intensive care unit ( icu ) patients will resolve remaining concerns about tgc in the icu . included among these concerns is the key issue of what is the most appropriate algorithm to achieve the desired blood glucose range . the major focus of the study conducted by chase and colleagues was on the method to achieve a predetermined blood glucose range by modulating both insulin infusion rate and nutritional inputs . as with the other reported studies comparing protocols , efficacy was evaluated by comparison with historical control patients . however , although the study reported by chase and coworkers was conducted with great care and rigour , it is but another case - control retrospective comparative study . nevertheless , there is a clear need to introduce efficient tools that will help clinicians and nursing staff to control blood glucose levels in icu patients , because hyperglycaemia superior to 10 mmol studies are required to provide clinicians with recommendations on the evaluation and comparison of the various protocols currently in use or that are soon to become available . benchmarking of tgc protocols must take in account all the dimensions of efficiency : performance , risk for severe hypoglycaemia , practical aspects ( ease of use , training time and required materials prior to implementation , error rate ) , integrated continuous monitoring , nursing workload ( evaluated on the mean time between controls ) . furthermore the best way to compare performance is controversial : is it the time with glucose within a common target range , the hyperglycaemia index , the recently described glycaemic penalty index , or the variability that would be associated with outcome ? this raises the question of whether the efficacy results from instructions regarding nutritional intake , allowing insulin infusion rates to be limited to a level lower than usual , or from the intrinsic quality of the algorithm used , which is based on the glucose - insulin regulatory system model ( capturing insulin utilization rate , insulin losses and saturation dynamics ) . also , sprint is apparently associated with few severe hypoglycemia events , which contrasts with the high rate of severe hypoglycaemic episodes reported in the second leuven study . finally , sprint should be relatively simple to implement in numerous icus as a paper - based protocol , presented in an original form using a wheel , without need for computational resources . weaknesses of sprint rest in its inability to monitor parameters related to the quality of glucose control as sprint is a paper - based protocol . most importantly , despite the favourable subjective opinions of care givers , sprint may not reduce workload because it requires measurements every hour or 2 hours . ultimately , evaluation of any tgc protocol must also include an assessment of its ability to be implemented easily and safely in another icu that did not participate in its development . the monocentric study of chase and coworkers may not ensure the ' exportability ' of their tgc protocol . the debate continues about the real benefits of tgc , with numerous questions being asked . what is the optimal target range ? which patients will benefit the most ? when during the icu stay should tgc be applied and to derive which benefits ? which is the best method to control glucose level intensive insulin therapy , and/or limitation of nutritional intakes during acute phase , and/or antidiabetic drugs ? however , the competition to develop the ideal tool with which to control blood glucose levels in the icu and perhaps throughout the hospital stay has begun , involving multidisciplinary teams of physicians and engineers who have specialized in control systems ( feedback control or model predictive control ) . icu = intensive care unit ; sprint = specialized relative insulin nutrition table ; tgc = tight glycaemic control . pk declares that he holds shares of lk2 ( saint - avertin , france ) .
the report by chase and coworkers in the previous issue of critical care describes the implementation into clinical practice of the specialized relative insulin nutrition table ( sprint ) for tight glycaemic control in critically ill patients . sprint is a simple , wheel - based system that modulates both insulin rate and nutritional inputs . it achieved a better glycaemic control in a severely ill critical cohort than their previous method for glycaemic control in a matched historical cohort . reductions in mortality were also observed .
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repair of anorectal malformation ( arm ) with rectourethral ( ru ) fistula involves the separation of two systems . this involves risk of injury to the urethra , ureters , seminal vesicles , bladder , and important nerves responsible for urinary control and sexual function . group a included patients of arm with ru fistula who had undergone posterior sagittal anorectoplasty ( psarp ) without closure of ru fistula , from february 2006 to january 2010 . the rest of the psarp procedure was the same as conventionally done , the only difference being that we did not close the ru fistula after separating it from the rectum . we just separated the rectum from the urethra and left the urethral fistula as it is without closing it . group b included 34 previous successive patients who had undergone psarp before january 2006 in whom the ru fistula was closed using interrupted sutures . all the patients in both the groups had undergone staged repair of arm and not primary psarp . micturating cystourethrogram ( mcu ) and distal colostogram was done in all these patients prior to psarp . all the patients were evaluated during follow - up both clinically and with investigations like mcu and cystoscopy . the patients were studied for parameters like urinary stream , urinary dribbling , urinary tract infections , and recurrent ru fistula . moreover , all patients had undergone urethrocystoscopy three months after psarp to check for the status of the urethra and bladder . patients who had sacral agenesis were excluded from the study group because such congenital sacral defects can lead to a neurogenic bladder . the following were the observations in group a ( a ) in the immediate postoperative period , there was no urinary leakage , urinary retention , or any other complication ; thus all these patients had an uneventful recovery . ( b ) urinary stream was normal ; there was no evidence of urinary dribbling or retention , urinary tract infection , and recurrent rectourinary fistula during follow - up . mcu showed normal urethra , without any evidence of stenosis or stricture , urethro - ejaculatory duct / vasal reflux , or diverticulum in any of the cases . however , in group b , complications like urethral stenosis , urethral diverticulum , and neurogenic dysfunction were seen . a comparative analysis of the two groups was done and overall complications were listed [ table 1 ] . during psarp , urological injuries in male patients are known complications.[13 ] excessive traction on the urethra during dissection leads to transection or injury to the urethra . it is extremely important for the surgeon to bear in mind that in arm with ru fistula , the rectum is intimately attached to the urethra and that meticulous dissection and separation are necessary . urethral stenosis can occur due to traction on the ru fistula , that is , indirect traction on the urethra during separation and closure of fistula . urethral stenosis can be avoided by applying less traction on the fistula during separation and avoiding the closure of fistula ; we have seen in our series that urethral stenosis was not seen in any of the cases of group a , where the ru fistula was not closed . closing another point to note is that if we separate the rectum from the urethra very near the urethral wall and use interrupted sutures for its closure , it also increases the chances for urethral stenosis . urethral stenosis due to ligation or closure of the ru fistula may result in recurrent epididymo - orchitis . urethral diverticulum is the result of a segment of the rectum left attached to the urethra and the separated end closed . such patients usually present with recurrent urinary tract infections , stone formations in diverticulum again leading to dysuria , urinary tract infections , and so on . this complication can be avoided by separating the rectum away from the urethra without leaving any segment of the rectum attached and leaving the fistula as it is without closing it , so that nothing like a pouch / diverticulum is formed . neurogenic dysfunction after psarp has been reported in the form of neurogenic bladder , impotence , or loss of ejaculation . postoperatively , a neurogenic bladder may reflect a poor surgical technique with denervation of bladder and bladder neck during repair . by avoiding the closure of the ru fistula , we can avoid excessive traction on the fistula and hence on the urethra , and also prevent the excessive dissection during fistula closure and minimize the chance of neurogenic dysfunction . damage to the external vesical sphincter has also been reported during ligation or closure of the fistula . thus by avoiding closure of the fistula , we avoid this complication also and , hence , neurovesical dysfunction . thus we have seen that by not doing something , that is , by not closing the ru fistula during psarp , we can avoid many complications ; so , not doing something is preferable here .
aim : to study the effect of nonclosure of rectourethral ( ru ) fistula and to do a comparative analysis of the complications with and without nonclosure of ru fistula during posterior sagittal anorectoplasty ( psarp ) in anorectal malformation cases ( arm).materials and methods : a total of 68 cases of arm were included in the study group , of which 34 cases were those in whom ru fistula was not closed ( group a ) during psarp . another 34 successive cases were included in study group b in whom the ru fistula was closed as is conventionally done by using interrupted sutures.results:comparatively , group a had none or minimum urological complications as compared to group b.conclusion:ru fistula closure is not mandatory during psarp and nonclosure avoids urological complications . it especially avoids urethral complications , which are 100% preventable .
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spondylolisthesis is defined as an anterior migration of a vertebral body in relation to the vertebra located immediately caudal . in 1930 junghanns was the first to describe anterior translation of a lumbar vertebra without any defect in the neural arch following this the term degenerative spondylolisthesis ( ds ) was introduced by newman in 1955 . five types of spondylolisthesis have been described including dysplastic , isthmic , traumatic , pathologic , and degenerative . there are many predisposing factors like sagittally - placed facet joint , a high iliac crest [ 4 - 6 ] , etc . ds which is characterized by an intact vertebral ring is presumed to result from degeneration of facet joints and intervertebral discs with aging and thus has traditionally been considered to represent instability of the vertebral segment . a 66-year - old gentleman , farmer by occupation , came with complaints of lower backache for 2 years which was insidious in onset , gradually progressive and non - radiating . he also complained of acute retention of urine since 15 days for which he was catheterized . he also had a history of neurogenic claudication at a distance of 100 m. there was no history of any trauma . a detailed systemic and neurological examination revealed power of flexor hallucis longus(fhl ) and flexor digitalis longus(fdl ) as 4/5 , ankle jerks were absent , sensory deficits in s1-s2 dermatome and per - rectal examination revealed decreased perianal sensations and anal tone with absent anal wink . plain radiographs of lumbo - sacral spine revealed spondylolisthesis of s1-s2 ( meyerding s grade 1 ) ( fig.1 ) . magnetic resonance imaging and computed tomography scan of the spine revealed lumbarization of s1 with spondylolisthesis of s1 over s2 , facetal hypertrophy at l5-s1 and canal stenosis at s1-s2 ( figs . 2 , and 3 ) . anteroposterior ( a ) and lateral ( b ) radiographs showing grade 1 spondylolisthesis ats1-s2b . the patient underwent posterior spine surgery where decompression was done with laminotomy of s1 bilaterally and then pedicular screw fixation was done bilaterally at l5 , s1 , and s2 ( fig.4 ) . s1 and post - operative radiographs - anteroposterior ( a ) and lateral ( b ) view . the bladder symptoms disappeared after 3 weeks and the power of fhl / fdl improved from 4/5 to 5/5 . the patient underwent posterior spine surgery where decompression was done with laminotomy of s1 bilaterally and then pedicular screw fixation was done bilaterally at l5 , s1 , and s2 ( fig.4 ) . s1 and post - operative radiographs - anteroposterior ( a ) and lateral ( b ) view . the bladder symptoms disappeared after 3 weeks and the power of fhl / fdl improved from 4/5 to 5/5 . the patient underwent posterior spine surgery where decompression was done with laminotomy of s1 bilaterally and then pedicular screw fixation was done bilaterally at l5 , s1 , and s2 ( fig.4 ) . s1 and post - operative radiographs - anteroposterior ( a ) and lateral ( b ) view . the bladder symptoms disappeared after 3 weeks and the power of fhl / fdl improved from 4/5 to 5/5 . the deformity occurs at l4 - 5 6 times more often than at other lumbar levels and four times more often above a sacralized l5 . the lumbosacral junction and middle lumbar spine are most often involved , but the lesion is also found in cervical or rarely the thoracic vertebra . to the best of our knowledge , ds of sacral vertebrae has not been reported in the available english literature till now . the prevalence of complete lumbarization is 1.8% and to get a spondylolisthesis is even rarer . there have been many publications in the literature mentioning incidence of spondylolisthesis with sacralization but hardly any on spondylolisthesis with lumbarization . further case series or longitudinal studies of such cases may help understand better the pathomechanics related to spondylolisthesis at this level . ds of s1-s2 is a very rare entity and further case reports will help us to explore the biomechanics at this level .
introduction : degenerative spondylolisthesis ( ds ) is usually seen at l4-l5 level and less frequently at l5-s1 level . this is a rare case report of spondylolisthesis of s1 over s2 with lumbarization of s1 . lumbarization of s1 is seen in just 1 - 2% of the population and to have spondylolisthesis in this segment is even rarer . the purpose is to report a rare case of ds at s1-s2 level.case report : this is a single case report of a 66-year - old gentleman who presented with complains of lower backache for 2 years and acute retention of urine to the emergency department . detailed clinical and radiological evaluation of the spine was done which revealed lumbarization of s1 with spondylolisthesis at s1-s2 and facetal hypertrophy at l5 , s1 , and s2 . he underwent decompression and stabilization at l5 , s1 , and s2 along with placement of autologous bone graft . the bladder symptoms disappeared after 3 weeks . at 1-year follow - up , patient s clinical symptoms were relieved , and he improved clinically.conclusion:to the best of our knowledge , this is probably the first case of ds of sacral vertebrae to be reported in english literature . the prevalence of complete lumbarization is around 1.8% and to get spondylolisthesis in this segment is even rarer , hence the lack of literature in this regard . since this is the first of its kind of case , further case series or longitudinal studies of such cases may help understand better the pathomechanics related to spondylolisthesis at this level .
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ductal adenocarcinoma of the prostate was first reported by melicow and pachter in 1967 as an endometrial carcinoma prostatic utricle . since then , ductal adenocarcinoma of the prostate has been found to account for 0.27.5% of all prostate carcinomas . a 73-year - old man was referred to our hospital due to an elevated prostate - specific antigen ( psa ) level of 23.4 ng / ml . he had no remarkable medical history . the hematological and biochemical data showed no abnormal findings aside from the elevated psa levels . in february 2016 , a prostate needle biopsy detected gleason score 4 + 4 adenocarcinoma in his left prostate . computed tomography ( ct ) and magnetic resonance imaging ( mri ) showed a higher density on his left peripheral zone ( fig 1a , b ) . in may 2016 , radical prostatectomy with lymph node resection histologically , there were many large , clear - edged cells and cancer cells with low differentiation forming a circular shape . based on these findings , ductal adenocarcinoma and gleason score 4 + 4 = 8 acinar adenocarcinoma with positive surgical margin were diagnosed . the patient has not experienced recurrence or biochemical recurrence in the 10 months since radical prostatectomy . histologically , there were many large , clear - edged cells and cancer cells with low differentiation forming a circular shape . based on these findings , ductal adenocarcinoma and gleason score 4 + 4 = 8 acinar adenocarcinoma with positive surgical margin were diagnosed . no adverse perioperative events were observed . the patient has not experienced recurrence or biochemical recurrence in the 10 months since radical prostatectomy . ductal adenocarcinoma of the prostate was first reported as endometrial carcinoma of the prostatic utricle in 1967 . recent studies have suggested that ductal adenocarcinoma of the prostate developed from the ductal epithelium , based on findings from immunohistochemical and electron microscope analyses . histologically , ductal adenocarcinoma of the prostate is characterized by high cylindrical epithelium collate papillary or etat cribriform . the histological differences between ductal adenocarcinoma and acinar adenocarcinoma are thought to be clear . in this case , although the prostate needle biopsy showed acinar adenocarcinoma , the surgical specimens showed ductal adenocarcinoma . the first is a mixed type with acinar adenocarcinoma and accounts for < 75% of ductal prostate specimens . mixed - type ductal prostate adenocarcinomas account for 5.06.6% of all prostate cancer cases , and pure - type ductal prostate adenocarcinomas account for 0.40.8% of all prostate cancer cases . because ductal carcinomas account for 90% of all prostatic carcinoma cases , our case was assumed to be pure type . because of its extension toward the urethra , the tumor was not palpable on a digital rectal examination and showed a low psa level . ductal adenocarcinoma of the prostate usually extends toward the urethra and shows macrohematuria and urinary symptoms at an early stage . reported that ductal adenocarcinoma of the prostate showed a significantly poorer prognosis than acinar prostate adenocarcinoma in nonmetastatic cases . however , in metastatic cases , there were no prognostic differences between these 2 groups . other reports have found no marked differences in the 5-year survival rate between ductal adenocarcinoma and gleason score 810 acinar adenocarcinoma . reported therapies of ductal adenocarcinoma of the prostate are also the same as for acinar adenocarcinoma , including radical prostatectomy , androgen deprivation therapy , and radiation therapy or a combination of these therapies . reported that pure ductal adenocarcinoma tended to extend into the submucosal urethra ; as such , pure ductal adenocarcinoma carries a higher risk of a positive surgical margin in the urethra . although we are not performing adjuvant therapy in this patient at present , careful observation including ct , mri , or positron emission tomography - ct should be performed , as psa does not always accurately represent cancer progression .
ductal adenocarcinoma is an unusual variant of adenocarcinoma of the prostate . a 73-year - old male was referred to our hospital for the further examination of an elevated prostate - specific antigen level of 23.4 ng / ml . radical prostatectomy ( rp ) was performed based on the diagnosis obtained by a prostate needle biopsy . the rp specimen revealed ductal adenocarcinoma of the prostate with positive capsular penetration . we herein report a rare case of ductal adenocarcinoma of the prostate .
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incidental detection of small renal masses is increasing . this has led to an increase in biopsy of small renal masses , a proportion of which needle biopsy of small renal masses is controversial owing to the risk of seeding malignant cells along the needle tract . needle tract seeding is a rare event ; the incidence is estimated to be less than 1 in 10,000 cases of all biopsies . eight other cases of needle tract seeding in a renal mass biopsy have been described in the medical literature , two as recently as 2013 ( table 1 ) . we report our experience of a man with renal cell carcinoma ( rcc ) seeding along a biopsy tract and compare the circumstances and biopsy techniques with reported cases in the literature . a 66-year - old man was incidentally found to have a 32-mm right lower pole renal mass on a computed tomography ( ct ) scan ( fig . two samples were obtained by use of a 16-gauge temno core biopsy needle ( carefusion , san diego , ca , usa ) and a 22-gauge francine needle . histopathology revealed a well - circumscribed 30-mm clear cell rcc , predominantly fuhrman grade 2 with focal areas of grade 3 . there was an area where the capsule was interrupted that corresponded to a hemorrhagic area on the cortical surface ( fig . a tumor deposit was also noted in the perinephric fat . these features suggested that the tumor deposit in the fat was likely due to tumor seeding rather than a metastasis and that the tumor seeding could have resulted from the needle biopsy . his tnm staging was pt3a nx mx , at least stage 3 disease ( american joint committee on cancer , 7th edition , 2010 ) and his leibovich score was 5 ( intermediate risk ) . six months after the operation , there was no radiological evidence of tumour recurrence on a ct scan . aside from the potential for false - negative results , a key risk of renal mass biopsy is seeding of the biopsy tract with malignant cells . several factors in theory could affect the risk of biopsy tract seeding , such as needle size , the number of needle passes , and the use of a coaxial needle . biopsy tract seeding has been reported in renal mass biopsies using needles as fine as 23-gauge and as large as 14-gauge . theoretically , a larger - bore needle would increase the risk of seeding owing to an increased area of defect on the surface of the tumor and an increased circumference or surface area of the needle . however , because of the scarcity of cases , it is difficult at this stage to accurately determine a relationship between needle size and the risk of seeding . it is also difficult because of underreporting to associate the risk of needle tract seeding with the number of needle passes through a tumor . use of a coaxial needle allows multiple passes through the renal mass with only one pass through the surrounding normal tissue . this theoretically reduces the risk of needle tract seeding into normal tissue and potentially reduces patient discomfort as well . although it is interesting to note that a coaxial needle was not used in any of the currently reported cases of needle tract seeding after renal mass biopsy ( table 1 ) , there are just too few cases to establish a firm relationship between the risk of biopsy tract seeding and the use of a coaxial needle . visualization of larger coaxial needles on ultrasound or ct may be easier than with smaller biopsy needles , and this may improve accuracy . histological evidence of biopsy tract seeding may not always be found after definitive surgery to remove the renal mass . seeding into excised perinephric tissues can be found soon after surgery but seeding into surrounding muscle , fascia , and skin may only be apparent months , or even years , after surgery . as was seen with this case , the biopsy needle traversed skin , subcutaneous tissue , multiple muscle and fascia layers , and perinephric fat before reaching the renal lesion ( fig . thus , the tumor could theoretically seed into one or more of these tissues ; seeding as superficial as the subcutaneous tissue has been reported ( table 1 ) . this delayed presentation may increase the risk of adverse outcomes such as further metastasis and poorer prognosis . time to presentation or diagnosis of tumor seeding after renal mass biopsy has ranged from 24 days to 84 months in previously reported cases where tumor seeding was not found on the initial histopathological analysis ( table 1 ) . in conclusion , a common feature in all reported cases of needle tract seeding from a renal mass biopsy is that a coaxial needle was not used . however , because of the paucity of cases , there is currently no satisfactory association between the risk of needle tract seeding and needle size or the number of needle passes . it is important to consider that histopathological evidence of needle tract seeding may not be apparent in all cases , especially if seeding occurred beyond the excised tissues .
a 66-year - old man underwent computed tomography - guided needle biopsy of a suspicious renal mass . two months later he underwent partial nephrectomy . histology revealed a 30-mm clear cell renal cell carcinoma , up to fuhrman grade 3 . an area of the capsule was interrupted , which corresponded to a hemorrhagic area on the cortical surface . under microscopy , this area showed a tongue of tumor tissue protruding through the renal capsule . a tumor deposit was found in the perinephric fat . these features suggest that tumor seeding may have occurred during the needle biopsy .
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chest pa showed superior mediastinal widening and bulging of the right paraspinal interface ( fig . 1 ) . increased opacity of the infrahilar window and thickening of the posterior wall of the bronchus intermedius were seen on the lateral chest x - ray . an oval - shaped mass measuring 9.73.86.0 cm was seen in the left superior mediastinum . a bizarre - shaped vascular structure that was connected to left innominate vein was seen in central area of the mass ( fig . the mass showed insinuating appearance without significant compression of adjacent structures . only the thymus was slightly compressed by the mass . another similarly natured mass was seen in the right paraspinal area , which measured about 4.23.06.6 cm . this mass also contained a bizarre - shaped vascular structure that communicated with the left atrium directly through an anomalous vascular structure ( fig . 2 ) . multiplanar reconstruction ( mpr ) and 3d reformation showed that the contrast directly entered the vascular components of the masses via an abnormal vein originating from the left innominate vein ( fig . the mediastinal window setting of contrast - enhanced ct showed additional multiple small cystic lesions in the middle mediastinum . a two - stage operation was planned due to the fact that the tumors were located bilaterally . first , for the left side tumor resection , posterolateral thoracotomy was performed and a large cystic mass was found in the anterosuperior mediastinum . the content of the cyst was bloody and a large communicating vein to left innominate vein and few small communicating veins to left intercostal veins were found , which were then ligated and divided . the second operation was performed for the right mediastinal mass 45 days after the first surgery . the right paraspinal mass was successfully resected and an anomalous communicating vein to left atrium was identified and divided . the patient recovered completely without any complications such as chylothorax or hemothorax . on gross pathologic examination , the cut surface of the tumor revealed numerous cystic spaces and a cavernous sinus containing bloody contents ( fig . histology of the mass showed combined proliferation of venous vascular channels ( ve ) and lymphatic channels ( ly ) that exhibited infiltration into mediastinal fat pad ( fig . mediastinal lymphangiohemangioma is a very rare venolymphatic malformation containing both vascular and lymphatic elements . direct vascular communication between the lesion and the vena cava system ct images of the mediastinal lymphangiohemangioma are very rarely found in the english literature [ 1 - 4 ] . moreover , neither a case with multiple masses and more than one vascular communication nor a case directly connected to the heart chamber through an anomalous vessel was found in the literature . we report a case of surgicopathologically confirmed mediastinal lymphangiohemangioma that communicated with the left innominate vein and left atrium directly via anomalous vascular structures ( fig . vascular malformations are subcategorized as lymphatic , capillary , venous , arteriovenous , and mixed malformations on the basis of their histologic nature . . therefore , our case can be categorized as low - flow vascular malformations with mixed lymphatic and venous components . slow - flowing blood appears as high signal intensity on t2-weighted images and as intermediate signal intensity on t1-weighted images . most lesions present early in childhood and the most common locations for lymphatic malformations are the neck and axilla . a less common location is the mediastinum , and the anterior mediastinum is the most commonly found mediastinal site . ct scan , the mass showed diffuse low attenuation without calcification , although previous reports showed scattered phleboliths in the mass , which represent calcified emboli in the venous channels . the anomalous vascular structures were enhanced at the same intensity as the systemic venous system and was connected directly to the left innominate vein and left atrium . the peripheral portion of the lesion was not enhanced on all phases of dynamic ct scan , which may be related to the lymphatic components or fibroconnective tissues of the mass . a previous report postulated that the anomalous vein acts not only as a draining vessel but also as a feeding vessel . in our case , only the proximal part of the vascular structure was enhanced on the pulmonary arterial phase , whereas all of the large bizarre - shaped vascular spaces were enhanced on the systemic venous phase ( fig . 2 ) . a delayed ct scan may show delayed clearance of contrast within the anomalous venous channels because of the lack of smooth muscle in these channels . lymphangiohemangiomas are not considered an congenital anomaly but are frequently associated with other vascular malformations , most commonly in the left superior vena cava . in our case , the anomalous vessels connected to the left innominate vein may be considered a left superior vena cava remnant . a lymphangiohemangioma should be included in the list of differential diagnoses of an incidentally found low - attenuating mediastinal mass , especially in young patients . another therapeutic option is percutaneous sclerotherapy using agents such as absolute ethanol , bleomycin , cyclophosphamide , doxycycline , alcohol solution of zein , or ok-432 . recognizing that the lesion is a low - flow vascular malformation is more important than determining whether the lesion is predominantly venous or lymphatic when making treatment decisions . we planned surgical excision of the lesions and successfully excised them without any complications such as hemothorax or chylothorax . for successful surgery , the anomalous vascular pedicle of the mass communicating with the systemic vein or heart chamber should be carefully identified and divided to prevent massive hemothorax .
lymphangiohemangiomas of the mediastinum are exceedingly rare and few cases have been published in the english literature . this report may be the only reported case in which lymphangiohemangiomas were found bilaterally . we report a case of a 7-year - old boy with an incidental finding of an abnormal mediastinal shadow on a chest x - ray . the chest ct showed a large mass in the left superior mediastinum and another in the right posterior mediastinum . the left mass had anomalous venous channels connected to the left innominate vein , and the right mass to the left atrium . we performed an excision of the mass in the left side first and then the right side one month later . anomalous venous channels were dissected carefully and ligated . there were no complications and no signs of recurrence 30 months after the operation .
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a male neonate born to g2 p1l1 mother at term by spontaneous vaginal delivery to iii degree consanguineous marriage was found to have proximal shortening of both upper and lower limbs [ figure 1 ] . the antenatal period was uneventful and antenatal ultrasound was reportedly not done during pregnancy and the mother was referred to our hospital after the onset of labor . apart from rhizomelic shortening , the neonate also had coronal clefts of thoracic vertebrae and stippled epiphysis of femur tibia and humerus on skeletal survey radiograph [ figure 2 ] . based on the above features a provisional diagnosis of rhizomelic chondro - dysplasia punctata ( rcdp ) was made and the prognosis was explained to the parents . the baby developed progressively severe respiratory distress and was discharged at request on day 3 of life as the parents were unable to come to terms with the diagnosis . rhizomelic shortening of upper limb punctate calcification and epiphyseal abnormalities chondrodysplasia punctata is a radiological diagnosis characterized by punctate or stippled calcifications in epiphyseal cartilage and seen in peroxisomal disorders such as zellweger syndrome , neonatal adrenoleukodystrophy , and infantile refsum disease . it may also be inherited as x - linked dominant , x - linked recessive , and autosomal recessive forms . it is classically associated with pex7 gene ( peroxin family of genes ) mutation and has been reported in indian patients too . rcdp is characterized by proximal shortening of the humerus and to a lesser degree the femur , punctate calcifications in cartilage with epiphyseal and metaphyseal abnormalities , radiolucent defects ( coronal clefts ) of the vertebral bodies which represents cartilage that are not ossified , cataracts , contractures , microcephaly , characteristic skin changes of icthyosis , facial dysmorphism ( depressed nasal bridge , hypertelorism , hypoplastic midface , anteverted nostrils , full cheeks ) , and developmental impairment . this condition is considered to be lethal and most of the affected fetuses die in utero or soon after birth . only few of them survive beyond infancy with severe physical disability and profound mental retardation in whom , death usually occurs in the first decade of life . diagnosis of rcdp is based on clinical findings and confirmed by clinically available biochemical or molecular genetic testing which includes biochemical tests of peroxisomal function like red cell plasmologen concentration , plasma phytanic acid , and very long chain fatty acid estimation . this case is presented due to its rarity and failure to detect such an abnormality in utero resulting in a wasted pregnancy . the lack of resources ( both money and manpower ) is probably responsible for this tragedy to the parents which could have been prevented by early diagnosis and appropriate counseling . establishing regional genetic labs which are connected with district level hospitals can be of immense help in reducing the burden of genetic diseases by appropriate prenatal diagnosis and counseling .
a male neonate was born with rhizomelic shortening of limbs . skeletal radiograph showed punctate calcification of epiphysis of humerus , femur , and tibia . the diagnosis and a brief review of literature pertaining to the condition with emphasis on antenatal diagnosis and counseling are being reported .
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a 56-year - old female presented with best corrected visual acuity ( bcva ) of 20/120 and nuclear sclerosis ( nuclear opacity 3 , nuclear color 2 using lens opacification classification system iii ) in the left eye ( le ) . the surgery was performed using proparacaine drops ( paracain ophthalmic solution 0.5% , sunways pvt . ltd . , a foldable iol of + 22.5 d of the sensar ar40e variety [ abbott medical optics inc ( amo ) , 1700 e. st . andrew place , santa ana , ca 92705 usa ] was loaded into the emerald c cartridge ( lot ch00841 of amo inc . , usa ) by the first assistant outside the field of the operating microscope to save the surgical time . the cartridge was inserted in the injector and the loaded injector was handed to the surgeon . the tip of the cartridge was just inserted snugly by slight rotatory motion into the anterior chamber through the 2.8-mm incision . however , while removing the injector system , it was noticed that there was a gross downward beaking of the bevelled anterior end of the cartridge [ fig . immediately , the incision site was inspected under the operating microscope and a descemet 's tear was detected with a rolled out flap of about 2 mm in length . the viscoelastic was meticulously washed out and the main incision wound and the two side port entries were carefully hydrated and an air bubble was injected into the anterior chamber . downward beaking of the beveled anterior end of the emerald c cartridge ( lot ch00841 of advanced medical optics , inc . , usa ) on the first postoperative day , the patient 's le had an uncorrected va of 20/60 with a small rolled out descemet 's flap and adjacent descemet 's striae at the site of the main clear corneal incision ( temporally ) [ fig . a corneal opacity remained at the deeper corneal layers with mild surrounding edema , even at the time of last check - up at 4 weeks post - op , with a bcva of 20/30 [ fig . a small rolled out descemet 's flap and adjacent descemet 's striae ( left eye of the reported case on the first postoperative day ) corneal opacity at the deeper corneal layers with mild surrounding edema ( left eye of the reported case at 4 weeks post - op ) damage to iols as a consequence of passage through various injector systems includes marks or scratches , stress fractures , cracks and tear lines . damage to descemet 's membrane can occur due to various factors during cataract surgery , including engaging of descemet 's membrane by the leading haptic during iol implantation or with the irrigation / aspiration device ( when mistaken as an anterior capsular remnant ) or due to inadvertent injection of viscoelastic between descemet 's membrane and corneal stroma . repair techniques include manual repositioning , repositioning with viscoelastic or air , suturing of descemet 's membrane to the peripheral cornea or use of sf6 or c3f8 . our case report describes injury to the corneal endothelium and descemet 's membrane intraoperatively due to frayed and beaked tip of the amo emerald c cartridge . the case has been followed up for a period of about 4 weeks as on the day of reporting , and as the visual axis was not completely involved , the vision is maintained , although the descemet 's stria and mild surrounding edema remains . we have reported only a single case ; however , a damaged amo emerald c cartridge has been found in a few subsequent cases in our institute . in cases where a back - up cartridge was not available , the size of the clear corneal incision was increased to 3.2 mm at least to protect the entry wound architecture and the corneal endothelium , while injecting the iol using the damaged cartridge . the cause of the damaged nature of the amo emerald c cartridges appears to be a manufacturer 's oversight which has been duly informed to the concerned authorities , who have assured speedy correction of the defect . these cartridges are delivered in sterile transparent cases and the tip can be easily examined under the slit lamp beforehand without opening the casing . this should be made a routine practice as this will allow any damaged cartridge to be replaced before starting the surgery by ordering a fresh one from the manufacturer . we suggest loading of the foldable iol should be done by the surgeon himself under the operating microscope . the speed of the surgery should never compromise the quality of the surgery and/or the final visual outcome . with the advent of newer techniques like the microincision cataract surgery ( mics ) , surgeons also can not compromise on the incision size or wound integrity and architecture . the onus is on the various manufacturing companies to provide surgeons with precision instruments that are both safe and durable for the patient 's eyes . and more importantly , every instrument entering the patient 's eye should undergo careful preoperative microscopic inspection by the operating surgeon himself so that a microscopic manufacturing defect can be identified and immediate rectification of the situation can be done .
foldable intraocular lens ( iol ) implantation using an injector system through 2.8-mm clear corneal incision following phacoemulsification provides excellent speedy postoperative recovery . in our reported case , a sensar ar40e iol ( abbott medical optics , usa ) was loaded into emerald c cartridge , outside the view of the operating microscope , by the first assistant . the surgeon proceeded with the iol injection through a 2.8-mm clear corneal incision after uneventful phacoemulsification , immediately following which he noted a descemet 's tear with a rolled out flap of about 2 mm near the incision site . gross downward beaking of the bevelled anterior end of the cartridge was subsequently noticed upon examination under the microscope . we suggest careful preoperative microscopic inspection of all instruments and devices entering the patient 's eyes to ensure maximum safety to the patient .
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when the gallbladder is not visualized in its normal location , the possibility of its ectopic location should be considered . a case of incidentally detected anomalous position of gall bladder causing confounding problem in interpretation of pet - ct is described . a 70-year - old man , with h / o chronic liver disease and suspected of hepatocellular carcinoma [ serum alpha - fetoprotein ( afp ) 5024 ng / ml ] was subjected to fluorine-18 fluorodeoxyglucose positron emission tomography ( f-18 fdg pet)/computed tomography ( ct ) imaging . rest of the liver revealed non - fdg avid lesions in segments iii and viii . on viewing the fused pet / ct images , the radiotracer accumulation was localized to the anomalously placed suprahepatic gallbladder . magnetic resonance ( mr ) images of the same patient confirmed the presence of the suprahepatic gallbladder [ figure 1 ] . ( a ) transaxial view of pet image showing suprahepatic subdiaphragmatic gallbladder with tracer uptake ; ( b ) coronal view of pet image ; ( c ) sagittal view of pet image ; ( d ) transaxial view of ct image ; ( e ) coronal view of ct image ; ( f ) sagittal view of ct image ; ( g ) transaxial view of post - contrast t1-weighted mri image ; ( h ) coronal view of post - contrast t1-weighted mri image ; ( i ) transaxial view of t2 fat saturated mri image shows gallbladder as a bright structure routine imaging of the gallbladder demonstrates a wide array of imaging variants , including anomalies in location , number , and configuration . an awareness of these normal variants would prevent misdiagnosis and aid in the assessment of differential diagnostic possibilities . normally , the gallbladder is situated adjacent to the inferior surface of the liver , in the plane of the interlobar fissure , with the gallbladder neck maintaining a constant relationship to porta hepatis . the gallbladder is generally found in the right upper quadrant , but may be seen in other parts of the abdomen . while anomalous positions are rare , the most common of these are ( 1 ) under the left hepatic lobe , ( 2 ) intrahepatic , ( 3 ) transverse , and ( 4 ) retroplaced ( retrohepatic or retroperitoneal ) . the lesser common of these are ( 1 ) supradiaphragmatic and ( 2 ) suprahepatic . gallbladder is intrahepatic during the embryonic period and becomes extrahepatic only later . an intrahepatic gallbladder ( usually a congenital anomaly ) this poses a problem for scintigraphy , as an intrahepatic gallbladder can cause a focal defect ( pseudo space - occupying lesion ) ; ultrasonography can be helpful in these cases . the suprahepatic region is among the rarest sites , and very few reports have appeared in either the surgical or radiological literature.[35 ] of the very few reports on the suprahepatic gallbladder , one refers to a normally inserted organ that rotated 180 upward to an intrathoracic position after eventration of the diaphragm . in two other cases , an abnormally mobile gallbladder was found trapped between the chest wall and the upper border of the liver ; this became symptomatic and caught the attention of the clinician and the imageologist . faintuch et al . reported three cases of suprahepatic gallbladder with hypoplasia of the right hepatic lobe and upward migration of the gallbladder . gansbeke reported a case of suprahepatic gallbladder which was associated with hepatomegaly due to macronodular cirrhosis complicating existing hepatitis . kabaroudis reported a case of floating gallbladder associated with hypoplasia of the right hepatic lobe , whereas maeda had reported a similar case associated with hypoplasia of left hepatic lobe . pet - ct is found to be useful in diagnosing this rare anatomical variant of ectopically located gall bladder and predicting its functional implication .
the purpose of this study was to appraise the imageologists of a possible mislocalization of tracer accumulation to anomalously placed gallbladder during positron emission tomography - computed tomography ( pet / ct ) examination . pet / ct is increasingly playing an important role in staging and restaging of the disease process in cancer patients . with the advent of fusion imaging , the tracer accumulation can be correctly localized to a structure or lesion on ct . we did a staging pet / ct scan of a patient with hepatocellular carcinoma for liver transplant evaluation . fluorine-18 fluorodeoxyglucose ( f-18 fdg ) was used as a tracer and the scan was performed on seimens biograph - mct pet / ct machine . we noted the tracer accumulation at the superior surface of liver , which was localized to the anomalously placed gallbladder in suprahepatic subdiaphragmatic location . the anomalously placed gallbladder can create localization confusion . keeping the possibility of ectopically placed gallbladder in mind , the imageologist can better localize the tracer uptake .
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anterior cervical spine fusion and stabilization is a well established procedure for cervical myelopathy , radiculopathy , neoplasms , and cervical trauma2 ) . although injuries to the pharynx and esophagus are known complications of anterior cervical spine surgery , delayed pharyngeal or esophageal perforation is rare7,9,10 ) . here , we describe a rare but potentially life - threatening complication after anterior cervical spine fusion and plating . the authors highlight this issue by presenting this case , which had no associated morbidity , and include a review of the relevant literature . a 43-year - old man was admitted to our institute with a 3-month history of dysphagia and neck pain with swelling . he was paraplegic due to a c6 - 7 fracture and dislocation and has been operated on 8 years previously . initial surgical treatment included anterior corpectomy of c7 and anterior iliac crest graft placement using a plate and screws . hematological studies including erythrocyte sedimentation rate ( esr ) and c - reactive protein ( crp ) were normal . a simple lateral radiograph and a computed tomography scan showed partial anterior migration of the lower screw . a hydro - soluble contrast swallow image confirmed esophageal perforation ( fig . the loose screw was removed and esophageal perforation was found during surgery and repaired directly by a cardiovascular team(fig . the patient was fed using a nasogastric tube for 3 weeks and subsequently oral feeding was gradually resumed . further progress was favorable , and a contrast study performed at 3 weeks postoperatively showed no evidence of fistula . anterior cervical fusion and plate fixation is an effective procedure for the treatment of cervical myelopathy or radiculopathy and cervical spine trauma . plating has been reported to achieve a fusion rate of up 98% , and to result in early mobilization , reduced graft - related complications ( especially for multilevel fusion ) , and to avoid late deterioration of the cervical spine alignment obtained at surgery1,4 ) . the complication rate after anterior cervical plating is generally low and decreases with surgeon 's experience . according to zeidmann14 ) , the overall complication rate associated with anterior cervical spinal fusion is approximately 5% , and pharyngo - esophageal perforation is uncommon , but nevertheless of the utmost importance because of the possibility of graft infection leading to osteomyelitis , mediastinitis , sepsis , and death6 ) . acute injury can be caused iatrogenically during surgical approach due to inappropriate placement or dislodgement of sharp - toothed retractor blades in the esophagus . retraction is particularly dangerous when a nasogastric tube is positioned because the wall of the hypopharynx or esophagus may be " trapped " by a high - pressure claw between the retractor and the tube , causing ischemic injury and secondary perforation8 ) . delayed esophageal injuries are due to chronic compression or contact and subsequent necrosis , abscess formation , and perforation due to graft dislodgement or screw migration with or without plate failure4,7 ) . screw dislodgement often follows a benign course and is completely asymptomatic , due to the small diameters of the screws used and slow migration from the external to the internal mucosa , which permits spontaneous tissue repair of the defect caused repetitive friction between the retropharyngo - esophageal wall and the plating system(normally positioned with adhesion ) , traction - type pseudodiverticulum , and perforation are other causes of delayed injury11 ) . the complications of esophageal perforation range from asymptomatic with local infection to mediastinitis and death . the clinical course depends on the etiology , location , and timing of the perforation . asymptomatic perforation has as well been reported as incidental oral extrusion of screw even years after anterior cervical spine stabilization5 ) . patients generally present with swallowing difficulty , regional swelling , neck pain , dysphagia , weight loss , dysphonia , subcutaneous emphysema , and fever ; our patient presented with dysphagia and neck pain with regional swelling12,13 ) . conservative treatment may be preferred for small , contained defects of less than 1 cm , and consists of the elimination of oral feeding , tube feeding to restore fluid and nutritional balance , and intravenous antibiotics . some cases need surgical repair , such as , perforation closure with a primary suture or sternocleidomastoid or pectoralis major flap repair13 ) . we operated on our patient to remove the offending screw due to evident fistula confirmed by esophagography and esophagoscopy . direct repair was effective in achieving a successful perforation repair of the esophageal perforation with an early return to oral feeding . we report a rare case of delayed esophageal perforation caused by screw displacement after anterior cervical spine plating . careful periodic follow - up is necessary , and when encountered , early surgical closure following removal of the offending screw is mandatory .
although anterior approaches to the cervical spine are popular and safe , they cause some of complications . esophageal perforation after anterior spinal fusion is a rare but potentially life - threatening complication . we present a rare case of delayed esophageal perforation caused by a cervical screw placed via the anterior approach . a 43-year - old man , who had undergone surgery for complete cord injury at another orthopedic department 8 years previously , was admitted to our institute due to painful neck swelling and dysphagia . radiological studies revealed a protruding screw and esophageal perforation . the perforation was found during surgery and was successfully repaired . this case emphasizes the need for careful long - term follow - up to check for delayed esophageal perforation in patients that have undergone anterior cervical spine plating .
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this work was supported , in whole or in part , by the one hundred person project of sun yat - sen university ( xz ) , national natural science foundation 81302262 ( xz ) , the basser research center for brca ( lz ) , the national institutes of health r01ca142776 , r01ca190415 , p50ca083638 ( lz ) , the ovarian cancer research fund ( lz and xh ) , the breast cancer alliance ( lz ) , department of defense ( lz ) , and the marsha rivkin center for ovarian cancer research ( lz ) .
long non - coding rnas ( lncrnas ) are defined as rna transcripts larger than 200 nucleotides that do not appear to have protein - coding potential . accumulating evidence indicates that lncrnas are involved in tumorigenesis . our work reveals that lncrna fal1 ( focally amplified lncrna on chromosome 1 ) is frequently and focally amplified in human cancers and mediates oncogenic functions .
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additional supporting information may be found in the online version of this article at the publisher 's web site .
this report describes that a regular positive electrospray ionization mass spectrometry ( ms ) analysis of terpendoles often causes unexpected oxygen additions to form [ m + h + o]+ and [ m + h + 2o]+ , which might be a troublesome in the characterization of new natural analogues . the intensities of [ m + h + o]+ and [ m + h + 2o]+ among terpendoles were unpredictable and fluctuated largely . simple electrochemical oxidation in electrospray ionization was insufficient to explain the phenomenon . so we studied factors to form [ m + h + o]+ and [ m + h + 2o]+ using terpendole e and natural terpendoles together with some model indole alkaloids . similar oxygen addition was observed for 1,2,3,4-tetrahydrocyclopent[b]indole , which is corresponding to the substructure of terpendole e. in tandem ms experiments , a major fragment ion at m / z 130 from protonated terpendole e was assigned to the substructure containing indole . when the [ m + h + o]+ was selected as a precursor ion , the ion shifted to m / z 146 . the same 16 da shift of fragments was also observed for 1,2,3,4-tetrahydrocyclopent[b]indole , indicating that the oxygen addition of terpendole e took place at the indole portion . however , the oxygen addition was absent for some terpendoles , even whose structure resembles terpendole e. the breakdown curves characterized the tandem ms features of terpendoles . preferential dissociation into m / z 130 suggested the protonation tendency at the indole site . terpendoles that are preferentially protonated at indole tend to form oxygen addition peaks , suggesting that the protonation feature contributes to the oxygen additions in some degrees . 2014 the authors . journal of mass spectrometry published by john wiley & sons , ltd .
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a dna sequence contains six potential open reading frames ( orfs ) , three on one strand and three on the reverse strand . however , typically only one of the six is actually expressed because it is associated with appropriate genetic signals that specify the dna strand and the reading frame to be transcribed and translate . exceptions occur in which more than one open reading frame is translated into a protein , as has long been observed in the case of viral genes , where it was suggested that this property permitted a high packing density of information ( 1 ) . however , analysis of the coding potential of 481 prokaryotic genomes revealed the surprisingly high frequency of alternate orfs of annotated genes especially in high g + c rich genomes , where almost every annotated orf exhibits an alternative orf that could potentially encode a protein of 100 amino acids or more ( 2 ) . the frequency of alternate open reading frames in high g + c genomes gives rise to the possibility that this property could be exploited to evolve novel genetic information and it is important to be able to detect this potential . however , this high frequency also provokes serious problems of gene annotation , where the incorrect orf may inadvertently be mis - annotated as the coding sequence . this potential for error is especially problematic when automatic gene prediction programs are used to annotate genomes , but errors can also slip by human annotators . the problem is exacerbated if an alternative orf is mis - annotated and the error is propagated in subsequent genome annotations . alterorf provides a searchable database of all possible alternative orfs in sequenced prokaryotic genomes that are potentially capable of encoding proteins of 100 amino acids or more . the objectives are 2-fold : to improve genome annotation by indicating possible errors in orf identification and , perhaps more important in the long term , to predict instances of genes that potentially could give rise to more than one protein . annotated protein coding genes were extracted from completely sequenced prokaryotic genomes in the genome database of ncbi . all alternative orfs , potentially encoding 100 amino acids or more , were extracted from each gene sequence using perl scripts and the bioperl application programming interface ( api ) ( 3 ) . using the standard genetic code , the in silico translated amino acid sequence of each alternative orf was searched for similarity in completely sequenced prokaryotic genomes ( 4 ) and for conserved domains and motifs using cdd ( 5 ) , pfam ( 6 ) , cog ( 7 ) , kog ( 8) , smart ( 9 ) and uniprot . ( 10 ) . hierarchical clustering using the software hcluster_sg developed as part of the treefam project ( 11 ) was used to build sequence families with the alternate orfs . blast e - values were normalized from 0 to 100 ( with 100 corresponding to e - value 0.0 ) . the resulting information was stored in a relational database built with microsoft sql server 2005 . release 1.0 ( september 2007 ) contains approximately 1.5 million annotated genes from 481 organisms and about 3 million alternate orfs . of these 942 856 ( 33% ) occur in frame 1 , 621 306 ( 21% ) in frame 2 , 322 284 ( 11% ) in frame 3 , 350 805 ( 12% ) in frame + 2 and 675 525 ( 23% ) in frame + 3 . the following are provided for each alternate orf sequence : ( i ) conserved domains and motifs including cdd ( 5 ) , pfam ( 6 ) , cog ( 7 ) , kog ( 8) , smart ( 9 ) and uniprot . ( 10 ) and ( ii ) blast results with annotated sequences in completely sequenced prokaryotic genomes and alternate orfs identified in alterorf . the cross genera conservation of some alternate orfs suggests that they might represent new protein families or domains and hierarchical clustering ( 11 ) was used to build sequence families from conserved alternate orfs . the alterorf database can be accessed through a simple and easy to use web interface at www.alterorf.cl . the database can be searched by protein i d ( derived from ncbi ) , by organism and by sequence using a sequence search service . in addition , an option is provided to analyze complete genome sequences not present in the database . searching by protein i d : a protein i d can be used to recover the original annotated gene that appeared in the database ( e.g. genbank ) , and also any alternate orf(s ) associated with that gene . if alternate orfs are detected , tables providing information regarding domains , motifs and protein family are displayed with links to further information . searching by organism : the user can select an organism from a pulldown menu or index for a pre - analyzed list of annotated protein coding genes with alternate orfs . searching by protein sequence : a search using a protein sequence can be carried out against all sequences stored in alterorf using wu - blast ( blast.wustl.edu/ ) . downloading data : all data in the alterorf database can be freely downloaded by ftp . additional information on the use of alterorf can be found in the faqs and tutorial sections .
alterorf is a searchable database that contains information regarding alternate open reading frames ( orfs ) for over 1.5 million genes in 481 prokaryotic genomes . the objective of the database is to provide a platform for improving genome annotation and to serve as an aid for the identification of prokaryotic genes that potentially encode proteins in more than one reading frame . the alterorf database can be accessed through a web interface at www.alterorf.cl
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the epidermal growth factor receptor ( egfr ) is over expressed in various solid malignancies including non small cell lung cancer ( nsclc ) . however , they are associated with a dermatologic side effects , which can occasionally be responsible for discontinuation of the egfr inhibitors . hence , we report a case of metastatic adenocarcinoma of lung who developed skin ulceration with gefitinib and responded to interruption of the drug and early intervention . the present case report is about a 50-year - old female patient who had been diagnosed as having lung adenocarcinoma with multiple bone metastases was initiated on gefitinib therapy at an oral dose of 250 mg / d . after 2 weeks of initiating therapy , the patient presented with ulcer over the palm [ figure 1 ] . the ulcers improved with stopping gefitinib for 2 weeks and also with the addition of topical steroids and antibiotics . non - small - cell lung cancer ( nsclc ) with sensitive mutations of the egfr is highly responsive to gefitinib . gefitinib is a small molecule tyrosine kinase inhibitor ( tki ) of egfr . since 2004 , it was clear that a substantial proportion of nsclc obtaining objective response when treated with gefitinib harboring activating mutations in the egfr gene . the occurrence of skin disorders ( dry skin and acneiform rash ) is explained by the fact that egfr is also expressed in the basal layer of the skin ; inhibition of the receptor will disturb normal biology and result in skin rash . skin rash is notorious as an adverse event of egfr - tki and is noted in up to two - thirds of patients receiving any of these agents although severe in only 5 - 10% who can develop pyogenic granuloma like lesions . very rarely the cutaneous inflammation is so pronounced that skin necrosis with black eschar formation and ulceration is seen . the cutaneous side - effects are treated with topical steroids and antibiotics with interruption of treatment for 2 - 4 weeks as in our case .
we report a case of gefitinib - induced skin ulceration in a 50-year - old female with metastatic adenocarcinoma of lung who developed this adverse effect 2 weeks following initiation of gefitinib at a dose of 250 mg / day . the ulcer improved with stopping gefitinib for 2 weeks and also addition of topical steroids and antibiotics . we are reporting this case to create awareness among treating oncologists of this adverse effect and also prompt interruption of therapy and topical steroids / antibiotics is useful to treat this adverse event .
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a 28-year - old man who was known to have fhi in the right eye was referred for secondary iol implantation . six years before referral , his right eye had undergone cataract surgery which was complicated by the capsular rupture and vitreous prolapse , for which the patient received complete anterior vitrectomy with removal of all capsular remnants . the patient was left aphakic and was prescribed with aphakic contact lens ; however , he developed contact lens intolerance over time . on presentation , his uncorrected visual acuity was 20/20 in the left eye and counting finger in the right eye which could be corrected to 20/20 with aphakic correction . slit - lamp examination of the right eye revealed diffuse fine keratic precipitates over the entire corneal endothelium and mild iris stromal atrophy with notable heterochromia . advantages and unknown risks of the surgery were thoroughly explained for the patient and he consented to have secondary iol implantation . under general anesthesia , an iris - claw iol ( artisan , ophtec , groningen , the netherlands ) was implanted in right eye through a limbal incision followed by a superior peripheral iridectomy . enclavation of the iol haptics was easily performed ; no intraoperative complication including hyphema was noted . the latter was prescribed as 0.1% betamethasone every 2 hours while awake for 1 week and then four times a day which was tapered within 6 weeks . postoperative follow - up examinations were performed at 1 , 2 , 3 , 5 , and 7 days , then weekly for 1 month , monthly for 3 months , and every 23 months thereafter until 1 year . postoperative course was uneventful with no significant anterior chamber inflammation ( more than 1 + cellular reaction ) or fibrin formation . on the first postoperative day , the examination showed 1 + cellular reaction and pigments in the anterior chamber which disappeared within 2 weeks . no subsequent exacerbation of the intraocular inflammation was observed during 12 months of postoperative follow - up ; therefore , no additional course of steroid was required . occasional cells in the anterior chamber were seen at some visits which were left untreated . one month after surgery , the patient achieved a best - corrected visual acuity of 20/20 in the right eye which was maintained for 12 months of follow - up . the iol remained stable with no subsequent iris atrophy at the enclavation sites , subluxation , or pupil ovalization . furthermore , the patient did not develop any anterior or posterior segment complication including glaucoma , vitreous inflammation , or clinical cystoid macular edema . secondary implantation of iris - claw artisan intraocular lens ( iol ) in an eye with fuchs heterochromic iridocyclitis . during 12 months of postoperative follow - up , no remarkable anterior chamber inflammation was observed in the right eye ( a ) and there were only few deposits on the iol surface ( b ) . the heterochromia in the involved eye is most obvious compared with the normal left eye ( c ) although secondary iol implantation in the ciliary sulcus has been reported to be safe in fhi , angle- and iris - supported iols have been feared because of the possible risk of postoperative uveitis , glaucoma , and hyphema . to the best of our knowledge , there has been no previous report of implantation of iris - claw artisan iols in eyes with fhi . even though our patient only received topical steroids , he did not show any significant postoperative inflammation or fibrinous reaction neither at the early postoperative period nor during 12 months of follow - up . therefore , it may suggest that in eyes with fhi the uveal irritation by iris - claw artisan iols is less than expected and the iol is more tolerable , even though recurrent or chronic anterior chamber inflammations has previously been reported in some eyes with these iols without preexisting uveitis . however , this lack of exacerbated postoperative inflammation in fhi may not be extrapolated to eyes with other more severe forms of uveitis . on the other hand , although fhi - associated iris atrophy in severe cases may theoretically make enclavation more difficult or compromise the long - term stability of an iris - claw iol , neither did develop in our case . therefore , it seems iris - claw iols , which have been shown to be safe in aphakic eyes without uveitis , may be an option in aphakic patients with fhi who do not have capsular support . however , studies on large number of patients with long - term follow up are required to determine the safety of these iols in eyes with uveitis including fhi .
implantation of iris - claw artisan intraocular lens ( iol ) is a surgical option for correction of aphakia ; however , these iols have not been used in eyes with uveitis including fuchs heterochromic iridocyclitis ( fhi ) due to possible risk of severe postoperative intraocular inflammation . in the case reported here , we secondarily implanted an artisan iol in a 28-year - old man with fhi who had aphakia with no capsular support due to a previous complicated cataract surgery . enclavation was easily performed and no intraoperative complication was noted . postoperative course was uneventful with no significant anterior chamber inflammation during 12 months of follow - up . although there were few deposits on the iol surface , the patient achieved a best - corrected visual acuity of 20/20 without developing glaucoma or other complications . therefore , artisan iol may be considered for correction of aphakia in patients with fhi . however , studies on large number of patients are required to evaluate safety of the procedure .
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all forms of mercury viz . , organic , elemental , and mercury salts are toxic and manifestations depend on nature , intensity and the chemical form of mercury . most human exposure results from fish consumption ( organic mercury ) or dental amalgam ( metallic mercury ) . kidneys are the prime target of mercury toxicity as it is primarily excreted through them . here , we describe a patient who consumed mercuric chloride with suicidal intent , and presented with typical manifestation such as acute kidney injury ( aki ) and gastrointestinal erosion . in addition , he had disseminated intravascular coagulation ( dic ) , a rare complication of mercury poisoning . a 36-year - old male was admitted with 2 days history of oliguria progressing to anuria , facial puffiness , edema legs , bleeding gums , hematochezia , and fever . he gave a history of consumption of unknown substance ( around 500 mg ) a week back used for folk remedies and rituals . his heart rate was 92/min , respiratory rate was 15/min , and blood pressure was 130/90 mm of hg . laboratory investigations showed hemoglobin 11.2 g / dl ; total count 6000/mm ; platelet count 100,000/mm ; blood urea 124 mg / dl ; serum creatinine 6.7 mg / dl ; sodium 135 meq / l ; and potassium 6.1 meq / l ; arterial blood gas analysis showed high anion gap metabolic acidosis . his coagulation profile showed prothrombin time 18 s , inr 1.6 ; activated partial thromboplastin time 60 s ; fibrin degradation products 10 mg / ml ; d - dimer levels 1 mg / ml ; and serum fibrinogen 250 mg / dl . diagnosis of overt dic was made as per criteria proposed by the international society of thrombosis and hemostasis with total score of five at admission . he received eight sessions of hemodialysis over 2 weeks after which his urine output started improving . renal biopsy done 2 weeks after admission showed markedly dilated tubules with sloughed off epithelium and cell debris within lumen [ figure 1 ] , interstitial edema , and mild inflammatory infiltrate in the interstitium consistent with acute tubular necrosis . consumed substance brought by the patient 3 weeks later was white colored powder , called . toxicological analysis of the compound revealed it to be mercuric chloride . at the end of 2 months , there are three classes of mercury : metallic elemental mercury , inorganic mercurial salts ( mercurous and mercuric salts ) , and organic mercurials . acute poisoning of this leads to corrosive bronchitis , pulmonary edema / fibrosis , diarrhea , renal dysfunction , visual and neuropsychiatric disturbances , and in severe cases , death due to respiratory failure . organic mercury compounds are absorbed completely from the intestine , converted to inorganic forms , and possess similar toxic properties . mercuric chloride is still used as wood preservative , photographic intensifier , disinfectants and also in indigenous drug formulation , and folk remedies in asian countries . once ingested , mechanism of mercury toxicity include ( a ) mercuric ions precipitate proteins that cause direct necrosis of tissues . about 8590% of mercury in the body accumulate in the kidneys causing acute renal failure due to necrosis of the proximal tubular epithelium . ( b ) inorganic mercury complexes sulfhydryl groups and causes metabolic acidosis , vasodilatation , and shock . though acute tubular necrosis is the most common lesion , tubulointerstitial nephritis and immune - mediated glomerular damage can also occur . rarely , it can present as hypertensive encephalopathy especially in children , nephrotic syndrome , chronic tubulointerstitial nephritis , or with isolated tubular dysfunction . our patient consumed mercuric chloride with suicidal intention and developed gastrointestinal erosion , anuric renal failure , and dic . franco et al . reported one patient with mercuric chloride poisoning who developed two consecutive episodes of acute renal failure by two different mechanisms , one toxic and the other immunological . renal biopsy done in that patient , showed acute tubular necrosis initially and granulomatous interstitial nephritis in the second biopsy . the international society of thrombosis and hemostasis criteria , which has 91% sensitivity and 97% specificity , was used for the diagnosis of overt dic . a score of five or higher is compatible with dic while a score below five is suggestive of dic . the possible explanation for dic was the lowered fibrinolytic activity due to inhibition of plasma plasminogen activator or the inhibition of plasminogen activation reaction catalyzed by this enzyme as demonstrated in experimental rat models . measurement of mercury levels in blood ( > 3.6 mg / dl ) and urine ( > 15 mg / dl ) may be helpful in diagnosis . chelation therapy should be considered for any symptomatic patient with a history of acute elemental mercury exposure . chelating agents include dimercaprol ( bal ) , 2,3-dimercaptopropane-1-sulfonate ( dmps ) , dimercaptosuccinic acid , and penicillamine . hemodialysis is not effective in removing mercury , but can enhance the removal of the dimercaprol - mercury complexes . the outcome depends on the form of the mercury compound and severity of the exposure . we did not do urine mercury levels , and the patient had not received any chelating agents as toxicological analysis was done much later . we conclude that mercury poisoning should be considered in case of aki and dic though it is a rare complication . prompt treatment with chelating agents guided by measurement of mercury levels will have an impact on the favorable clinical outcome .
mercury is a toxic heavy metal and occurs in organic and inorganic forms . inorganic mercury includes elemental mercury and mercury salts . mercury salts are usually white powder or crystals , and widely used in indigenous medicines and folk remedies in asia . inorganic mercury poisoning causes acute kidney injury ( aki ) and gastrointestinal manifestations and can be life - threatening . we describe a case with unknown substance poisoning who developed aki and disseminated intravascular coagulation ( dic ) . renal biopsy showed acute tubular necrosis . later , the consumed substance was proven to be mercuric chloride . his renal failure improved over time , and his creatinine normalized after 2 months .
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frontometaphyseal dysplasia ( fmd ) , also called gorlin cohen syndrome , is a hereditary x - linked dominant syndrome described in 1969 with less than 30 cases described in the literature . this case report of a child with fmd is presented owing to the rarity of the syndrome and the anticipated difficult airway , which was successfully managed by using a combination of dexmedetomidine and ketamine while preserving spontaneous ventilation . a 2-year - old female child , a known case of fmd , presented for open reduction of the left hip with osteotomy of femur . physical examination revealed a slender undernourished girl of 8 kg with prominent supraorbital ridges , ocular hypertelorism , low set ears and a wide bridge nose with prominent eyes . airway examination revealed a mallampatti score of iii with significant retrognathia , high arched palate with malocclusion of teeth . in addition , she had dorsolumbar scoliosis , pectus carinatum , bowing of long bones with distal phalangeal hypoplasia and multiple joint dislocations [ figure 1 ] . pre - operative blood investigations , echocardiography and chest x - ray were within physiological limits . on arrival to the operation theatre , monitors were connected and child pre - oxygenated for 5 minutes . injection dexmedetomidine 1 g / kg was administered for 10 min and then a continuous infusion at 1 g / kg / h was set for the duration of the remaining procedure . ketamine was administered in increments of 5 mg up to 12 mg until there was no response to jaw thrust while ensuring spontaneous respiration . just before direct laryngoscopy intravenous lignocaine rigid laryngoscopy with miller 1 straight blade offered a grade iv cormack and lehane view . after optimal external laryngeal manipulation , the visible glottic chink was sprayed with topical lignocaine and tracheal intubation was successfully performed using an uncuffed 4 sized endotracheal tube . anesthesia was continued with n2o in 40% o2 along with a continuous dexmedetomidine and atracurium infusion . adequate padding was provided at pressure points and extreme caution was exercised during positioning . at the end of fmd belongs to the otopalatodigital spectrum syndromes that includes four phenotypically related conditions , otopalatodigital syndrome types 1 and 2 , fmd and melnick - needles syndrome . the most common manifestations include supraorbital hyperostosis , hypertelorism , down - slanting palpebral fissures , broad nasal bridge and micrognathia with anomalies of teeth and generalized skeletal dysplasia . congenital heart disease , subglottic tracheal narrowing and genitourinary anomalies , muscular hypotonia . micrognathia , microstomia and malocclusion of teeth may make direct laryngoscopy impossible ; therefore , a well - planned airway strategy is mandatory . ketamine was preferred in our case of anticipated difficult airway due to it 's inherent sympathomimetic actions devoid of respiratory depression alongwith provision of excellent analgesia and amnesia . dexmedetomidine a specific and selective 2-adrenoceptor agonist known for its sedative , anxiolytic , analgesic properties was used to complement ketamine . at the same time dexmedetomidine offsets the sympathomimetic effects of ketamine , this unique pharmacological combination in the present case preserved the respiratory drive , allowed maintenance of a patent airway and provided sufficient sedation , analgesia and anesthesia to allow successful airway control . in addition , topical lignocaine was used as per recommendation of aroni et al . which states that ketamine does not depress coughing or swallowing reflexes . available literature describes the use of combination of both these drugs in children during procedural anesthesia and not as a complete anesthesia protocol in a challenging case . the present experience of using this combination successfully paves the way to emerging new solutions for management of a difficult pediatric airway . hence safety profile , rapid onset of action with adequate sedation and analgesia provided by the ketamine and dexmedetomidine make them a distinctive drug combination in the pediatric difficult airway situation in a child with fmd .
frontometaphyseal dysplasia ( fmd ) , also called gorlin - cohen syndrome , is a rare hereditary x - linked dominant craniotubular bone disorder . the presentation describes the airway management of a 2-year - old child suffering from fmd with significant retrognathia , posted for major long bone corrective osteotomy . induction with a combination of dexmedetomidine and ketamine preceded a successful endotracheal intubation under spontaneous ventilation .
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multiple sclerosis ( ms ) is a chronic , autoimmune , demyelinating disease of the central nervous system ( cns ) . presenting symptoms can vary greatly , but most commonly involve weakness , paresthesia , gait difficulty , or visual deficits . virtually any area of the cns white matter can be involved , though this most classically involves the periventricular white matter . brain stem involvement is common , though isolated cranial nerve palsies are rare signs in ms . previous studies have suggested that isolated cranial nerve palsies in ms are more commonly found as presenting symptoms than as relapsing symptoms . among isolated cranial nerve palsies in ms , the fifth nerve is most commonly involved ( 4.8% ) , followed by the seventh nerve ( 3.7% ) , and the sixth nerve ( 1.0% ) . thus , abducens palsy is a rare isolated ms finding , either as a presenting sign or during disease exacerbation . patients with abducens palsy typically present with diplopia upon horizontal gaze , and examination can reveal a slow ipsilateral lateral rectus movement [ 4 , 5 ] . ms has been implicated as the cause of unilateral abducens palsy in 49% of cases , though mri may not detect brain stem lesions in all cases . one 2002 study investigating nontraumatic causes of sixth nerve palsies in patients 2050 years of age found ms to be the cause in 24% of cases . in addition to ms , the differential diagnosis for an abducens nerve palsy includes mass lesions , lyme disease , viral infection , syphilis , sarcoidosis , and vascular disease . here , we report a patient who presented with a unilateral isolated abducens palsy as the initial sign of ms . a 28-year - old man with a past medical history of hypertension and obesity presented to the emergency department with a 1-day history of double vision , most prominent upon left lateral gaze . the double vision resolved with covering either eye . in addition , he had a 12-week history of paresthesia of the distal right hand and forearm , and the fifth digit of the right foot . initial laboratory findings revealed only a mild leukocytosis ( 11.3 10/l ) and no other hematologic or electrolyte abnormalities . several focal areas of increased t2 signal intensity were noted within the periventricular white matter of the frontal and parietal lobes , subcortical white matter of the left temporal lobe , and the left pons . these findings likely represented multiple lesions distributed in both time and space , and , along with the patient 's clinical history and examination , were suggestive of a diagnosis of ms . the patient was admitted and started on intravenous methylprednisolone . further workup to rule out other etiological causes of illness revealed an elevated esr of 25 mm / h , negative viral and lyme serologies , nmo igg negative and a negative ana . he was treated with methylprednisolone 500 mg intravenously for 3 days and then discharged home . he was re - evaluated in the office 2 days after hospital discharge and had complete resolution of the cn vi palsy . isolated nerve palsies occur in only 10.4% of patients with ms , with abducens palsy as the third most common isolated nerve palsy , occurring in 1.0% of patients with ms , behind trigeminal ( 4.8% ) and facial ( 3.7% ) . these palsies occur most commonly at disease onset , though they can also occur during the course of the disease . the lesion in the medial pons was enhancing and thus consistent with an active lesion , and the location was consistent with his cn vi palsy . the sixth nerve nucleus is located in the pontine tegmentum , and a compact fiber tract bundle containing motor neurons from this nucleus runs medially towards the ventral region , where the cranial nerve exits the pons [ 3 , 8 ] . our patient 's pontine lesion appeared to be located along the fiber tract emerging from the sixth nerve nucleus , and correlates with his diplopia . while an mri lesion was detected clearly in this case , it should be noted that previous authors have found that mri does not always detect brain stem lesions accounting for these palsies in ms patients [ 4 , 6 ] . in any patient presenting with isolated cranial nerve palsies , ms must be considered within the differential diagnosis . in patients younger than 50 years of age ( as with our patient ) , infectious causes ( lyme disease , viral infections , syphilis ) , sarcoidosis , and autoimmune vasculitis should also be considered on the differential diagnosis , whereas for older patients small vessel vascular disease should be considered as well [ 7 , 8 ] . the presence of multiple presenting neurological deficits including cranial nerve palsies is suspicious for ms and should be investigated with mri . though mri may not always detect brain stem lesions responsible for cranial nerve palsies , it can reveal other white matter lesions of the cns that can aid in the diagnosis of ms , as was the case with our patient .
while brain stem involvement in multiple sclerosis ( ms ) is relatively common , isolated cranial nerve palsies are rare , especially when they represent the initial presenting sign of a new diagnosis of ms . this report describes a patient with no prior history of ms whose sole presenting sign was an isolated abducens palsy . an enhancing pontine lesion was found on mri which correlated with his abducens palsy , and additional nonactive lesions on mri led to a diagnosis of ms . this case demonstrates the importance of considering ms as part of the differential diagnosis of patients with isolated cranial nerve palsies .
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non - hodgkin lymphomas ( nhls ) account for approximately 60% of all lymphomas in children and adolescents . childhood nhls are subdivided into burkitt 's lymphoma , diffuse large b - cell lymphoma ( dlbcl ) , lymphoblastic lymphoma , and anaplastic large - cell lymphoma . dlbcl is characterized by relatively more frequent extranodal presentation , seen in upto 40% of the cases . primary involvement of the lymphoma of the middle ear is rare , with only about 18 cases being reported in literature so far . here , we report a case of dlbcl , presented with features of facial palsy and otitis , who received initial symptomatic treatment and later chemotherapy after diagnosing dlbcl . a 2 years 8 months old boy visited our tertiary care hospital with complaints of ear ache ( left side ) and left facial palsy of 4 weeks duration . after initial 2 weeks of these symptoms , there was whitish serous discharge from the left ear . before referral to our hospital , he was treated for otitis media with antibiotics , details of which were not available . his complete blood picture and biochemistry investigations done in our hospital were within normal limits . as the symptoms persisted for 4 weeks , computed tomography of head and neck was done which was suggestive of solid mass lesion of 2 cm 2 cm size in the left mastoid with destruction of mastoid bone [ figure 1 ] . the disease was in stage 1 ( as per murphy 's staging ) and was completely resected . histopathological examination showed large cells of lymphoid cell proliferation immune histochemistry was positive for cd20 , bcl-2 and negative for cd3 , with low mib-1 , which confirmed dlbcl . his positron emission tomography for staging , bone marrow and cerebrospinal fluid revealed no abnormality . computed tomography head and neck suggestive of solid mass lesion of 2 cm 2 cm size in the left mastoid with destruction of mastoid bone computed tomography head and neck of right side showing no abnormality his initial clinical symptoms of earache and facial nerve palsy followed by ear discharge resolved after starting chemotherapy as per b - cell lymphoma protocol for 6 months . currently , he is 37 months off treatment , and no disease recurrence is seen clinically as well as radiologically . it is an aggressive form of lymphoma , usually curable with appropriate treatment and has high survival rate . rapid disease progression of dlbcl calls for an early , accurate diagnosis and appropriate treatment . however , unusual presentation can mislead the physician resulting in wrong diagnosis , which delays the treatment , thus promoting disease progression . extranodal presentations of childhood dlbcl are relatively uncommon in clinical practice , and much rarer are those primarily involving middle ear and mastoid . involvement of middle ear and mastoid can resemble the features of otitis media and unusual facial palsy mimicking mastoiditis . although these initial symptoms were suggestive of middle ear infection , the distinguishing factor was unresponsiveness to antibiotics . there have been very few reports of nhl with facial nerve involvement ; ogawa et al . mccabe et al . , reported a case of 2-year - old , an immunocompetent boy with spontaneous regression of an epstein - barr - virus - associated monoclonal lymphoid proliferation who presented with acute otitis media and facial palsy . have described a case of dlbcl with features of otitis media , mastoiditis , and facial palsy . as symptoms persisted even after 4 weeks of treatment , the patient was started on chemotherapy , to which he promptly responded . extranodal nhls of middle ear which is not a common clinical presentation may present as facial palsy , and misdiagnosed as otomastoiditis . there should be a high index of suspicion for primary neoplasms of the middle ear in patients with chronic otomastoiditis refractory to appropriate initial antibiotic therapy ; early diagnosis and appropriate treatment results in good therapeutic outcome and minimizes further complications .
extra nodal presentation of non hodgkins lymphoma ( nhl ) is a rare entity , and data available about the nhl that primarily involves of middle ear and mastoid is limited . we report a case of diffuse large b cell lymphoma ( dlbcl ) , in a 2 year 8 month old boy , who developed otalgia and facial palsy . computed tomography revealed a mass in the left mastoid . mastoid exploration and histopathological examination revealed dlbcl . this case highlights the importance of considering malignant lymphoma as one of the differential diagnosis in persistent otitis media and / facial palsy .
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acute pancreatitis due to antipsychotic treatment is rare but sometimes causes a fatal adverse effect . some atypical antipsychotic agents , including clozapine , olanzapine , quetiapine , and risperidone , are associated with acute pancreatitis.1,2 ) among them , acute pancreatitis caused by risperidone is the rarest.3,4 ) although most cases of acute pancreatitis due to atypical antipsychotic agents occur within 6 months of starting antipsychotic administration,1 ) we experienced a schizophrenic patient suffering from pancreatitis after more than 6 months of risperidone therapy . a 69-year - old japanese woman was diagnosed with schizophrenia at the age of 30 years and received outpatient care at another mental hospital . her positive symptoms were not prominent , but her cognitive level was so impaired that she could not regulate her appetite and consumed about 2,000 kcal / day in addition to three ordinary meals . she had never smoked , did not drink alcohol , and did not take any illegal drugs . blood tests ( table 1 ) , abdominal ultrasonography , and a computed tomography ( ct ) scan were performed . clinical features were accompanied by laboratory findings of hyperamylasemia ( amylase , 1,191 u / l ) , hyperlipasemia ( lipase , 1,514 u / l ) , and mild liver enzyme elevations . results of the abdominal ultrasonography were positive for gallstones in the gallbladder and distention of the common bile duct . subsequently , the amylase and lipase titers remained high ( 461 u / l and 804 u / l , respectively ) , although alanine and aspartate aminotransferases decreased gradually to normal levels . at this point , we felt that it was safe for her to start taking the risperidone again . two days after starting the risperidone , serum lipase and amylase increased again to 1,275 u / l and 745 u / l , respectively , and ck also increased ( 766 u / l ) . we decided to suspend the risperidone and introduced 10 mg intravenous haloperidol injections once per day . two days after discontinuing the risperidone , the serum amylase decreased ( 605 u / l ) , but the serum lipase level remained elevated ( 1,654 u / l ) . one week after discontinuing the risperidone , the levels of amylase and lipase decreased gradually ( 309 u / l and 542 u / l , respectively ) , and ck dropped to the normal range . as her general clinical condition and biochemical markers were stable , we changed the haloperidol injection to an oral solution of 6 mg / day aripiprazole because her mental condition worsened after stopping the risperidone treatment . her mental status improved with the aripiprazole treatment , and she was discharged without positive laboratory findings . the patient 's monthly blood tests continue to be normal , including amylase , lipase , and blood cell counts . although atypical antipsychotic - induced pancreatitis has been reported in conjunction with hyperglycemia,5 ) the pathophysiological mechanism of these adverse events remains unclear . most antipsychotic - induced pancreatitis occurs within 6 months after administration1 ) ; however , our case developed pancreatitis more than 6 months after the start of risperidone treatment . risperidone is a 5-ht2a antagonist and ameliorates diet - induced necrotic pancreatitis in mice,6 ) and reduced serum pancreatic amylase levels is observed after endoscopic retrograde cholangiopancreatography.7 ) however , there is no evidence of an association between risperidone treatment and acute pancreatitis . a thorough evaluation for pancreatitis , such as alcohol , tumor , and autoimmune causes , gallstones were present , which were due to an adverse effect of risperidone because the two separate risperidone administrations elevated serum amylase and lipase independently . aripiprazole is currently used in such cases , as aripiprazole is thought to have fewer effects on metabolism , including saccharometabolism , than other atypical antipsychotic agents . lifestyle was also a risk factor in this case . thus , it is necessary to monitor pancreatic function in addition to hyperglycemia in such cases .
acute pancreatitis with antipsychotic treatment is rare but sometimes causes a fatal adverse effect . most cases of acute pancreatitis due to atypical antipsychotic agents are reported to occur within six months of starting antipsychotic administration . acute pancreatitis caused by risperidone is rare . the patient had a high fever , stomachache and vomiting . the results of the abdominal computed tomograhpy scan were negative . the results of the abdominal ultrasonography were positive for gallstones in gallbladder and distention of the common bile duct . she had been fasting and received antibiotic intravenous injections . amylase and lipase titers were high . after risperidone discontinuation , both the levels of the amylase and the lipase were gradually decreased . three months later , the patient still maintains a good clinical balance . although atypical antipsychotic - induced pancreatitis has been reported in conjunction with hyperglycemia , the pathophysiologic mechanism of these adverse events remains unclear . this case got pancreatitis 6 month after risperidone treatment . using the antipsychotic agents , it is necessary to monitor pancreas function .
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in recent years , drug - eluting stents ( des ) have been demonstrated to dramatically reduce the rate of restenosis and the need for repeat revascularization.1 - 3 ) despite these promising results , stent thrombosis seems to occur more frequently with des and often seems to be associated with clopidogrel resistance.4 ) we report a case of recurrent stent thrombosis associated with clopidogrel resistance in a patient with acute myocardial infarction . a 63-year - old female was transferred to the emergency department complaining of squeezing chest pain that had increased over the past twelve hours . she underwent emergent coronary angiography ( cag ) , which revealed critical stenosis in the proximal and middle left anterior descending coronary artery ( lad ) . she was successfully treated with two paclitaxeleluting stents ( 3.012 mm and 2.528 mm taxus stents , boston scientific , reading , pa , usa ) in the proximal and middle lad ( fig . she was treated with triple antiplatelet therapy ( aspirin 100 mg , clopidogrel 75 mg , and cilostazol 200 mg daily ) because she was found to be hyporesponsive to clopidogrel when tested for adenosine diphosphase ( adp)-induced platelet aggregation utilizing the verifynow p2y12 point - of - care assay ( 181/0 p2y12 reaction unit/% ) . three days after discharge , she again developed chest pain and presented to the emergency department with mental confusion associated with acute pulmonary edema . kinase - mb 34.0 u / l , troponin - i 67.01 ng / ml , troponin - t 5.5 ng / ml ) . emergency cag , after intubation due to hypoxemia , showed total occlusion of the mid - lad due to stent thrombosis ( fig . 4 ) . after intravenous administration of a glycoprotein iib / iiia receptor blocker ( reopro ) , balloon angioplasty was carried out multiple times using a 3.0 mm balloon at 10 - 12 atm because of recurrent , immediate thrombus formation and coronary occlusion . the next day , however , she complained of severe chest pain again , and the ecg showed marked st - segment elevation in v1-v6 , i , and avl ; and new - onset complete right bundle branch block with left anterior fascicular block ( fig . she was successfully treated with balloon angioplasty and a final angiogram revealed improved flow over stented lad without intraluminal filling defect . despite the cilostazol medication , adp - induced platelet aggregation showed that she was still hyporesponsive to clopidogrel ( 171/0 p2y12 reaction unit/% ) . she was discharged after uneventful recovery with triple anti - platelet therapy using an increased dose of aspirin ( aspirin 200 mg , clopidogrel 75 mg , cilostazol 200 mg daily ) . we report this case to draw more attention to stent thrombosis associated with clopidogrel resistance after des implantation . this case shows that a des patient with clopidogrel resistance can be vulnerable to stent thrombosis even if treated with triple anti - platelet therapy , which in recent studies has been shown to be more effective in preventing stent thrombosis than conventional dual anti - platelet therapy . stent thrombosis is an uncommon but serious complication of coronary artery stents that often presents as myocardial infarction ( mi ) or death . over several trials , the incidence of stent thrombosis was 0.58 - 1.3% in des.5)6 ) several factors have been associated with stent thrombosis , including older age , black race , diabetes mellitus , bifurcation lesion , in - stent restenosis lesion , procedure - related factors such as stent malposition , greater stent length , postprocedure acute renal failure , non - compliance to anti - platelet agent and anti - platelet resistance.7 - 9 ) anti - platelet resistance as an independent predictor of stent thrombosis , even several years after implantation of des , increases the risk of stent thrombosis . in this patient , stent thrombosis may have been caused by several risk factors , especially anti - platelet resistance . several therapeutic approaches ( the addition of cilostazol or a glycoprotein iib / iiia inhibitor , increased dosage of clopidogrel and aspirin ) might be taken for a patient with anti - platelet resistance . in our patient , although cilostazol ( 200 mg daily ) was added to conventional dual anti - platelet therapy , recurrent stent thrombosis occurred . subsequently , the daily dose of aspirin was increased from 100 mg to 200 mg . in the des era , stent thrombosis is a fatal complication and anti - platelet therapy has been shown to be very important in preventing stent thrombosis . thus , assessment of the patient 's responsiveness to anti - platelet agents may be a crucial factor in monitoring these drugs ' therapeutic efficacy and improving clinical outcomes after implantation of des . recent studies have shown that adequate anti - platelet effects are not achieved in 5% to 45% of the patients taking aspirin and in 4% to 30% of patients taking clopidogrel10)11 ) and therefore suggest that many patients are resistant or only partially responsive to the anti - platelet agents . currently , however , routine screening for anti - platelet resistance remains a persistent , unresolved issue and further evidence is necessary before it will be possible to recommend this testing as part of standard assessment of pci candidates . in addition , further prospective studies are needed to set guidelines for optimal treatment of patients with antiplatelet resistance who are at increased risk of stent thrombosis , a catastrophic complication of des implantation .
drug - eluting stents ( des ) are considered the treatment of choice for most patients with obstructive coronary artery disease when percutaneous intervention ( pci ) is feasible . however , stent thrombosis seems to occur more frequently with des and occasionally is associated with resistance to anti - platelet drugs . we have experienced a case of recurrent stent thrombosis in a patient with clopidogrel resistance . a 63-year - old female patient suffered from acute myocardial infarction and underwent successful pci of the left anterior descending coronary artery ( lad ) with two dess . she was found to be hyporesponsive to clopidogrel and was treated with triple anti - platelet therapy ( aspirin 100 mg , clopidogrel 75 mg , and cilostazol 200 mg daily ) . three days after discharge , she developed chest pain and was again taken to the cardiac catheterization laboratory , where coronary angiography ( cag ) showed total occlusion of the mid - lad where the stent had been placed . after intravenous administration of a glycoprotein iib / iiia inhibitor , balloon angioplasty was performed , resulting in thrombolysis in myocardial infarction ( timi ) iii antegrade flow . the next day , however , she complained of severe chest pain , and the electrocardiogram showed marked st - segment elevation in v1-v6 , i , and avl with complete right bundle branch block . emergent cag revealed total occlusion of the proximal lad due to stent thrombosis . she was successfully treated with balloon angioplasty and was discharged with triple anti - platelet therapy .
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thrombocytosis is commonly seen in clinical practice and often the cases reported are incidental . the differential diagnosis for thrombocytosis is broad and the diagnostic process can be challenging . in general , causes of thrombocytosis can be described as spurious , reactive , or clonal in nature . a number of population studies have examined the degree of thrombocytosis as well as the frequency of various etiologies of thrombocytosis when it occurs . reactive causes are by far the most common etiology of thrombocytosis in these population studies , comprising 8897% of cases in adults in two large case series and 100% of pediatric cases in a single case series . extreme thrombocytosis , defined as a platelet count > 1,000 10/l is quite rare , as only 25.8% of patients demonstrate this degree of thrombocytosis upon presentation . although often thought to be more common in clonal processes , extreme thrombocytosis can also be due to reactive causes , with 82% of cases of extreme thrombocytosis in one series being reactive in nature . a 26-year - old male patient was scheduled for surgery of right indirect inguinal hernia with right sided encysted hydrocele of the cord . the preoperative laboratory tests reported normal coagulation and biochemical parameters , haemoglobin values of 15.1 g% , no leukocytosis and a platelet count of 2.60 lakhs / cumm . patient was operated , right sided mesh hernioplasty with excision of hydrocele of the cord was done . there were no adverse incidents during surgery , the patient was smoothly extubated in the surgery room and transferred to the post anaesthesia care unit . a routine postoperative analytical blood control was performed , showing high platelets count ( 1,100,000/cumm ) , hemoglobin 11.6 mg / dl and leukocytes 18,400/cumm , with normal values of coagulation and biochemistry tests . another blood analysis was extracted to verify these data , which again returned a high platelet count ( 1,300,000/cumm ) . we suggested monitoring platelet count throughout the postoperative period by serial analytical , with introduction of thromboembolic prophylaxis and control of bleeding in the face of the probability of some degree of platelet dysfunction . the immediate period after surgery was uneventful and the patient was discharged to the ward with hemodynamic stability . later , on the next postoperative day , patient developed huge scrotal haematoma with ecchymosis around the incision line . scrotal haematoma was explored , clots were drained , but no obvious bleeder could be identified . postoperative antithrombotic prophylaxis and bleeding monitoring were maintained until the patient was discharged after 2 weeks of admission , to follow up with outpatient treatment . analytical blood controls showed that the platelet count was down to normal in 20 days . thrombocytosis is an incidental finding in 3550% of cases and determination of the cause creates a diagnostic challenge . the causes of thrombocytosis can be described as spurious , reactive , or clonal in nature as shown in table 1 . causes of thrombocytosis the reactive thrombocytosis , also called secondary thrombocytosis is the most common type and appears after acute inflammatory , infectious , neoplastic and stress processes . in these scenarios the levels of thrombopoietin , interleukin-6 and catecholamines are very high , and are thought to be responsible for the increased number of platelets . it is always necessary to distinguish between clonal ( also known as primary or essential thrombocytosis ) and reactive ( or secondary ) thrombocytosis , because their treatment and prognosis differ . distinguishing clinical features for primary and secondary thrombocytosis although the diagnostic tests to differentiate essential and reactive thrombocytosis are not easy to perform , laboratory tests that show increased acute phase reactants such as c - reactive protein , fibrinogen , erythrocyte sedimentation rate and interleukin-6 may be useful in the diagnosis of reactive thrombocytosis . it is accepted that upto 1,000,000 l platelets level are a benign condition , although it remains unclear if these findings are associated with an increased postoperative thromboembolic or haemorrhagic risk . prophylactic treatment with platelet inhibitors in these situations is controversial , although some authors do consider management of low - dose acetylsalicylic acid appropriate . reactive thrombocytosis is generally felt thought to be a self - limited process which resolves with resolution of the underlying disorder when possible . the risk of thrombotic complications with reactive thrombocytosis is felt to be low , as 1.6% of patients with reactive thrombocytosis had thrombotic complications in one large case series . all of these thrombotic events were venous in location and occurred in patients with other risk factors ( postoperative setting or underlying malignancy ) . even in cases of extreme reactive thrombocytosis the appearance of thrombocytosis after surgery needs to be diagnosed to establish the type of thrombocytosis ( clonal or reactive ) , as treatment and prognosis are quite different between them . and at all times , the surgeon must remain vigilant due to the possible risk of bleeding or thromboembolic complications . the work described in this paper was supported jointly by department of pathology and department of surgery , himsr , new delhi . the authors would like to thank hamdard institute of medical sciences for the administrative support . the authors also thank the editor and the reviewers for their valuable comments and suggestions that have led to the substantial improvement of the paper . the work described in this paper was supported jointly by department of pathology and department of surgery , himsr , new delhi . the authors would like to thank hamdard institute of medical sciences for the administrative support . the authors also thank the editor and the reviewers for their valuable comments and suggestions that have led to the substantial improvement of the paper .
thrombocytosis is often an incidental finding seen in 3550% of cases and the cause determination creates a diagnostic challenge . extreme thrombocytosis is rare and seen in 25.8% patients only . among the various causes of increased platelet count , surgical procedures have attracted much attention in both experimental and clinical domain . the appearance of thrombocytosis after surgery needs to be diagnosed to establish the type of thrombocytosis ( clonal or reactive ) , as treatment and prognosis are quite different between them . this case report is vital because of two reasons : first , the increase in platelet count is difficult to rationalize than many of the other thrombocytoses , such as those related to primary augmentation of the function of the bone marrow ; second , the association of platelets with the clotting process has led to the belief that their increase after a surgical procedure is connected with the occurrence of postoperative thrombosis . this case presents an interesting finding from a patient who has undergone major abdominal surgery and has shown an unexpected perpetual increase in platelet count .
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1 . he had a history of bilateral brow suspension surgery 10 years ago at another institution . the patient had a 30 cm 20 cm pelvic mass located in the presacral area with boundaries between both parailiac regions displacing the rectum and bladder . it was completely excised with a pathology report of myelolipoma . in physical examination , he had hypertrichosis , finger clubbing , and radiologically cortical thickening of the bones fig . the patient presented with bilateral blepharoptosis with coarse skin folds the patient had cortical thickening of the bones and clubbing his best - corrected visual acuity was 7/10 in the right eye ( re ) and 5/10 in the left eye ( le ) . horizontal length of the upper lids of re and le was 46 mm and 41 mm , respectively . vertical fissure heights were 6 mm and 4 mm with margin - reflex distances of 0.5 mm and 0.5 mm , respectively . vertical eyelid contour of the re showed an inverse v shape for the re and a smooth curve for the le . the eyelids were floppy and easily everted . to correct these clinical findings , a surgery which combines vertical tarsal shortening accompanied with horizontal whole eyelid wedge resection and brown suspension 10 mg/0.25 ml triamcinolone acetate was injected into each supratarsal space to decrease the thickness of the tarsus . horizontal wedge resection which is not a part of routine ptosis surgery was performed in this operation with the aim of normalizing the obvious horizontal length of the lids . pathological evaluation revealed epidermal hyperplasia , severe inflammatory changes in subepidermal level , hyperplastic sebaceous glands , and collagen tissue derangement fig . reconstruction of each eyelid was achieved by vertical tarsectomy , horizontal full thickness lid resection , and supratarsal steroid injection . ptosis was assessed with silicone rod frontalis suspension , and resection of excessive skin was performed as in blepharoplasty skin biopsy showing thickening of the dermis with increased collagen content and lymphocytic infiltration ( h and e , 10 ) the postoperative course was uneventful and patient satisfaction is good within the 1 year of follow - up fig . it is characterized by skin thickening , clubbing , hyperhidrosis , and periosteal reaction in the long bones . primary hoa is predominantly a male disease that at least 90% of patients are men . although symptoms may be seen in childhood , it manifests mostly during the fifth decade of life . described the largest number of primary hoa series in the literature that the authors reported that family history was positive in all studied patients . the pathogenesis of hoa is unclear and some studies explained the role of several growth factors in the evolution of the disease . although clinical findings are similar , secondary hoa differs from primary hoa with absence of family history . secondary hoa is an acquired form that is associated with usually lung disease but also heart , liver , and intestines . the presented patient had a huge pelvic mass which was completely excised with a pathology report of myelolipoma . hence , our diagnosis was secondary hoa due to the paraneoplastic manifestations of pelvic malignancy which causes secretion of many growth factors . blepharoptosis may develop secondary to sebaceous gland hyperplasia , thickening of the dermis with increased collagen content , mucin deposition , and lymphocytic infiltration or due to additionally marked scarring . our patient was a 52-years - old man with negative family history which let us exclude the diagnosis of primary hoa . the differential diagnosis includes several diseases as acromegaly , syphilitic periostitis , and thyroid acropachy . acromegaly is characterized by enlargement of facial bones that are not present in our case . serology , radiology , and clinical findings did not support a diagnosis of syphilis or thyroid disease . hoa may develop secondary to pulmonary or congenital cyanotic cardiac diseases those were not present in our patient . the remaining possible etiological factor for secondary hoa in our case was the huge pelvic malignancy which was excised previously . the surgery for these kinds of pathologies may be planned as single or staged procedures . blepharoplasty with excessive skin excision is usually required both for better cosmesis and to reduce the tissue bulk . no complications were encountered during surgery , but bleeding was more than any other lid surgery . the profound inflammatory reaction in the tissue might be the cause of this excessive bleeding . hoa may not be always primary , particularly in patients with negative family history . in cases of findings with abnormal fibroproliferative and inflammatory changes , detailed systemic examination and investigations both using serologic and imaging modalities 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 52-year - old male patient presented to our hospital with a history of secondary hypertrophic osteoarthropathy ( hoa ) associated with an abdominal neoplasia and blepharoptosis . he had finger clubbing , hyperhidrosis , and hypertrichosis . he also had a recent history of extensive abdominal surgery with a pathology report of myelolipoma . routine blood work was unremarkable . upper eyelid reconstruction with blepharoplasty , upper eyelid wedge resection , and brow suspension was performed to address his eyelid concerns . by this case report , we would like to attract notice that the eyelid involvement may be a part of hoa and to emphasize the importance of systemic and pathologic evaluation in failed blepharoptosis surgery .
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a 46-year - old married saudi woman presented at a primary health care center with left upper abdominal pain , with no other associated symptoms . five months later , she presented to the surgery clinic because of increased abdominal pain with no response to medication ; her physical examination showed fullness of the left upper abdomen . her routine laboratory investigation as well as chest and abdominal x - rays were not remarkable . ultrasound of the abdomen showed a solid mass at the left hypochondrium separated from the spleen and just below the left hemidiaphragm . computed tomography ( ct ) scan of the abdomen ( figure 1 ) showed encapsulated hypodense mass at the left upper abdomen which was interposed but clearly separated from left liver lobe , spleen , stomach and left hemidiaphragm . magnetic resonance imaging ( mri ) of the abdomen ( figure 2 ) confirmed the location of the mass and attachment to the inner aspect of the anterior abdominal wall but separated from internal abdominal organs . the mass was isosignal intensity to muscles in t1- weighted images and heterogeneous hyperintense signal in t2 weighted images . in post ( a ) axial non - enhanced ct scan of the abdomen showing a well defined hypodense mass at the left hypchondrium , interposed between left liver lobe , left hemidiaphragm and stomach . ( a ) axial t1w1 : an oval shape mass with signal intensity parallel to the muscle . ( c ) axial post contrast t1w1 showing avid contrast enhancement of the mass . at surgery , a firm , rounded mass apparently encapsulated at the left sub - diaphragmatic area was found attached to the extra - peritoneal fascia of the anterior abdominal wall . histopathology showed a biphasic tumor composed of spindle cell and epithelial cell elements , with mild anaplasia & infrequent mitosis . the final diagnosis was low grade synovial sarcoma involving the fascial aponeurosis of the anterior abdominal wall . synovial sarcoma ( ss ) is an uncommon soft - tissue malignant tumor that is common in the extremities of middle - aged patients , close to large joints particularly the knee in the popliteal fossa.12 despite its name , the lesion does not commonly arise in an intraarticular location but usually near joints . the tumor arises from pleuripotential mesenchyme , in close association with joint capsules , tendon sheaths , bursae and fascial structures . it is generally accepted that synovial sarcoma is derived from primitive mesenchymal cells , not synovial cells.2 synovial sarcoma is the fourth most common type of soft tissue sarcoma following malignant fibrous histiocytoma , liposarcoma and rhabdomyosarcoma . synovial sarcoma accounts for 5 - 10% of all soft tissue sarcomas.3 about 85 - 90% of ss occur in the extremities.4 reported examples arising in the anterior abdominal wall are rare . synovial sarcoma in the abdominal wall tends to occur with a much greater frequency in females in contrast to such tumors in the extremities or the neck which tend to occur with a much greater frequency in males.5 radiological findings of ss are not pathognomonic . however , findings of a soft - tissue mass , particularly if calcified ( 30% ) , near but not in a joint of a young patient , are very suggestive of the diagnosis . cross - sectional imaging features are essential for staging extent of the tumor and the planning of surgical resection.6 the most common ct appearance of ss is that of a heterogeneous soft - tissue mass with attenuation similar to or slightly lower than that of muscle areas of lower attenuation representing necrosis or hemorrhage are also common with heterogeneous contrast enhancement.7 on mri , ss typically appears as a prominently heterogeneous multilobulated soft - tissue mass with signal intensity similar to or slightly higher than that of muscle on t1-weighted mr images . prominent heterogeneity with predominant high signal intensity is also a feature of ss on t2-weighted mr images . the presence of multilobulation and marked heterogeneity are highly suggestive of a diagnosis of ss . ct and mri are useful in defining the extent of the disease and in follow up response to chemotherapy.7 the presence of extensive calcification suggests a more favorable prognosis.8 similar ct and mri appearances are seen in our case . despite its name there are three main histologic subtypes of ss : biphasic , monophasic , and poorly differentiated types . poorly differentiated ss is generally epithelioid in morphology and has high mitotic activity.78 in the present case , histopathology showed a biphasic pattern of ss , the coexistence of spindle cells in a wavy pattern , and the pseudoglandular formation . both biphasic and monophasic synovial sarcomas are usually intermediate grade ( grade 2/3 ) ; however , both types can be high grade ( grade 3/3 ) . poorly differentiated synovial sarcomas are high - grade tumors.7 the specific chromosomal aberration in synovial sarcoma has recently been reported . the hallmarks for synovial sarcoma are the ( x ; 18 ) translocation and syt - ssx gene fusion products . is the molecular diagnosis in synovial sarcoma , as well as immunohistochemical study , especially in cases in which histological diagnosis is difficult.79 surgical excision is the treatment of choice , and the recurrence rate range from 28% to 36% even with adequate surgical and adjunctive therapies.4 however , the multimodality treatment approach has improved the prognosis of synovial sarcomas . tumor invasiveness , histologic grade and tumor size significantly correlate with the survival period , with a more preferable prognosis of synovial sarcomas encountered in childhood . chemotherapy has been used to treat metastatic or residual disease.710 primary synovial sarcoma of the anterior abdominal wall is a rare extra - articular tumor site . in cases of anterior abdominal wall masses ,
synovial sarcoma is a malignant mesenchymal neoplasm which commonly occurs in the extremities of adults , in close association with joint capsules , tendon sheaths , bursae and fascial structures . only a few cases of synovial sarcoma occurring in the abdominal wall have been reported . a case of a primary synovial sarcoma arising from the anterior abdominal wall fascial aponeurosis is presented .
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retinal artery macroaneurysms ( rams ) are rare , acquired dilatations of the retinal arterial vasculature , usually within the first three branches of the arteriolar tree.1 the incidence is estimated to be approximately 1 in 4,500 people over the age of 40.2 hypertension and older age lead to hyaline degeneration of the vascular walls , loss of autoregulatory tone and elastic recoil , and arterial dilatation.3 approximately 8%25% of rams will undergo spontaneous involution . even with submacular hemorrhage , up to 37% of patients will have better than 20/40 visual acuity without treatment.4 however one third of patients will develop vascular leakage and retinal edema and may need treatment.5 an 84-year - old female with a past medical history of hypertension and dyslipidemia was referred for progressively decreasing visual acuity in the left eye . she had no history of diabetes . on initial examination , dilated fundus examination and fluorescein angiography of the left eye revealed a superotemporal retinal arterial macroaneurysm , with surrounding circinate exudates involving the fovea ( figures 1 and 2 ) . the patient was observed initially , but the edema increased and her vision deteriorated to 20/60 . the patient was observed for 3 months , but the vision worsened again to 20/60 , with increased edema and exudates on optical coherence tomography . she was observed for 3 months , but due to recurrent leakage , she ultimately received a total of six injections of intravitreal bevacizumab every 1 month to 3 months . argon laser at a setting of 400 mw and 200 m spot size was applied around the ram . the ram occluded , and on follow - up examination 6 months later , the patient s vision has remained at 20/30 . the first report of laser photocoagulation to treat exudative retinal artery macroaneurysm was published in 1976 by dr donald gass and colleagues.6 in a study of 27 patients with symptomatic ram , half of the patients underwent argon laser therapy and the remaining half was observed . the vision in both groups improved , but the laser group experienced a greater gain.7 approximately 16%27% of rams occlude with lasers , but there is a risk of vascular occlusion , early increase in exudates from selective reabsorption of fluid , arteriovenous shunts , macular pucker , and scotomas.7,8 other treatment options for rams include yellow dye laser and indocyanine green dye - enhanced photocoagulation.9,10 anti - vascular endothelial growth factor ( vegf ) therapy has recently been reported in the treatment of patients with exudative or hemorrhagic rams . chanana and azad11 published the first case report in 2009 , and subsequent case reports have shown encouraging results ( table 1).1219 cho et al18 described 23 patients with rams who were either observed or received intravitreal bevacizumab . both groups experienced statistically significant improvements in visual acuity and central macular thickness , but the bevacizumab group regained vision faster . a larger , prospective study of 38 eyes with hemorrhagic and exudative rams underwent three monthly injections of bevacizumab , with the vision and retinal thickness improving in both groups.19 the role of vegf and the mechanism of action of anti - vegf therapy in rams are not fully understood . in a study that compared the vegf levels in 500 l vitreous samples from patients with vitreous hemorrhage from nondiabetic etiologies ( including four patients with ram ) with those from patients with proliferative diabetic retinopathy , the vegf levels were significantly lower in the former than in the latter groups ( 2.75 pg / ml vs 821 pg / ml , respectively).20 however , these were small vitreous samples obtained up to 3 weeks after the initial hemorrhage and may not have been representative of the vitreous and microenvironment around the ram . the role of vegf in intracranial aneurysms and other biological systems has been more clearly elucidated . in intracranial aneurysms , vegf levels were found to be significantly higher than the levels in controls.20 in particular , patients with intracranial aneurysms had higher expressions of vegf receptor 2 ( vegfr2 ) and lower levels of vegf receptor 1 ( vegfr1 ) . vegfr1 is associated with angiogenesis , while vegfr2 is associated with thrombosis.21 bevacizumab has been shown to decrease vegfr1 levels and to increase vegfr2 levels to normal levels in cancer cells.22 in the circulatory system , platelets produce vegf , which stimulates nitrous oxide production , resulting in vasodilation.21 inhibition of vegf may decrease leakage of fluid and exudates through the endothelium . in mice studies , bevacizumab decreases bleeding time and increases coagulation.23 thus , vegf inhibition may block angiogenesis , decrease binding of the prothrombotic vegfr2 , and decrease vascular permeability . in conclusion , the majority of rams can be observed , but intervention may be necessary in cases of persistent or recurrent macular edema from exudative or hemorrhagic rams . our patient s ram was initially observed , but continued leakage prompted further therapy . anti - vegf injections have shown promise in improving visual acuity and hastening resolution of macular edema in ram;5 however , most of the previously published cases had relatively short follow - up . our case has one of the longest follow - ups with the most number of anti - vegf treatments , but the patient continued to have leakage into the macula . the potential benefits of anti - vegf treatment must be carefully weighed against the potential risk of infection from serial injections . laser photocoagulation remains a viable treatment option in cases of macular edema secondary to ram .
an 84-year - old female with a history of hypertension and dyslipidemia was referred for a retinal artery macroaneurysm with exudation that had extended into the macula . she underwent a total of six injections of bevacizumab , with some improvement in visual acuity and retinal thickness . due to persistent macular edema , focal laser photocoagulation was performed around the macroaneurysm . the vision remained at 20/30 during 20 months of follow up . although anti - vascular endothelial growth factor therapy may improve vision and decrease retinal thickness in retinal artery macroaneurysm , recalcitrant cases may be treated with laser photocoagulation to seal the leaking vessel .
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bat guano , an excrement of the cave - dwelling bats forms the basis of the ecology inside the cave by acting as a food source for detritivorous microbes . it contains high content of organic carbon , nitrogen , phosphate , and potassium , . bacteria present in bat guano were reported to be involved in nitrification process and were also known as potential chitinase producer . a clone library based study in bat guano samples has revealed the presence of group 1.1a and 1.1b crenarchaeota , an efficient ammonia oxidizer , . analyzing bat guano is also important since they often harbor various pathogens which can be thread for speleologists , and tourists . although the microbial communities in diverse cave ecosystems have been studied , little is known about the microbial communities of bat guano heaps , and there has been no studies using high throughput sequencing technology . meghalaya is known to possess the largest and most diverse karst caves in the world . pnahkyndeng cave located in ri - bhoi district of meghalaya , india is a home of various bats and offering an ideal environment for studying the bat guano microbiota without any anthropological influence . samples were collected on february 2014 from the bat guano of pnahkyndeng cave ( 255722.70n , 915543.10e ) , nongpoh , ri - bhoi district , india . ten composite guano samples were collected from different places of the cave floor and the soil community dna was extracted separately using the fast dna spin kit for soils ( mp biomedical , solon , oh , usa ) . the freshly extracted dna was purified twice using 0.5% low melting point agarose gel and mixed to prepare a composite sample . final dna concentrations were quantified by the using a microplate reader ( bmg labtech , jena , germany ) . the v4 region of the 16s rrna gene was amplified using f515/r806 primer combination ( 5-gtgccagcmgccgcggtaa-3 ; 5-ggactachvgggtwtctaat-3 ) . amplicon was extracted from 2% agarose gels and purified using the qia quick gel extraction kit ( qiagen , valencia , ca , usa ) according to the manufacturer 's instructions . quality filtering on raw sequences was performed according to base quality score distributions , average base content per read and gc distribution in the reads . singletons , the unique otu that did not cluster with other sequences , were removed as it might be a result of sequencing errors and can result in spurious otus . chimeras were also removed using uchime and pre - processed consensus v4 sequences were grouped into operational taxonomic units ( otus ) using the clustering program uclust at a similarity threshold of 0.97 , . all the pre - processed reads were used to identify the otus using qiime program for constructing a representative sequence for each otus . the representative sequence was finally aligned to the greengenes core set reference databases using pynast program , . , 403,529 reads were classified at the phylum , 282,350 at the order , 188,406 at the family and 2926 sequences were classified at the species levels . classified otus belonged to 18 different phyla dominated by chloroflexi , crenarchaeota , actinobacteria , bacteroidetes , proteobacteria , and planctomycetes ( fig . 1 ) . analysis of bacterial communities revealed the two most dominant bacteria 's chloroflexi ( 29.97% ) and actinobacteria ( 22.55% ) , which are known to be a common inhabitant of cave microflora . other identified phyla include crenarchaeota ( 16.96% ) , planctomycetes ( 12.41% ) and proteobacteria ( 12.03% ) . chloroflexi was divided into 11 classes thermomicrobia , planctomycetia , gitt - gs-136 , ktedonobacteria , anaerolineae , tk10 , tk17 , s085 , chloroflexi , ellin6529 , and gitt - gs-136 . the most dominant otu within this phyla was denovo 317 , classified under the class thermomicrobia ( 40.52% ) followed by denovo 710 under the thermomicrobia ( 7.61% ) , denovo 235 under the genus thermogemmatisporaceae ( 7.18% ) and denovo 3 under the genus gemmataceae ( 2.98% ) . the dominant otu within this phyla was denovo 74 under the genus mycobacterium ( 29.39% ) followed by denovo 993 ( 8.27% ) and denovo 372 ( 5.27% ) classified under the genus acidimicrobiales and actinomycetales , respectively . only five otus were classified up to the species level ( mycobacterium llatzerense and mycobacterium celatum ) . a third dominant phylum in this sample was identified as planctomycetes comprising of 91 otus and 46,063 reads . seventeen archeal otus were classified under the order nrp - j , methanomicrobiales and methanosarcinales . four of them were identified at the genus level ( methanosarcina , haloquadratum , methanosaeta and methanocorpusculum ) . the phylogenetic tree based on the genus level relationships is provided as supplementary fig . 1 . previous study on archaeal communities present in bat guano identified many ammonia oxidizing bacteria but it was limited with a few number of clones . our analysis provides in - depth and high throughput identification of the bacterial communities present in bat guano . in the present study , we identified 18 bacterial phyla and most of the bacterial genus identified was known to be involved in nitrogen cycling as seen in previous study . a significant portion of otus still remains unclassified which indicates the possibility for the presence of novel species in pnahkyndeng cave . further studies like whole metagenome sequencing or functional metagenomics can illustrate the detailed information of this bacterial community .
v4 hypervariable region of 16s rdna was analyzed for identifying the bacterial communities present in bat guano from the unexplored cave pnahkyndeng , meghalaya , northeast india . metagenome comprised of 585,434 raw illumina sequences with a 59.59% g+c content . a total of 416,490 preprocessed reads were clustered into 1282 otus ( operational taxonomical units ) comprising of 18 bacterial phyla . the taxonomic profile showed that the guano bacterial community is dominated by chloroflexi , actinobacteria and crenarchaeota which account for 70.73% of all sequence reads and 43.83% of all otus . metagenome sequence data are available at ncbi under the accession no . srp051094 . this study is the first to characterize bat guano bacterial community using next - generation sequencing approach .
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an atlanto - axial synovial cyst is very rare . since the first report of this lesion by onofrio and mih1 ) in 1988 , to our knowledge . however , the current report described a patient with a large hemorrhagic cystic mass which was seen around prevertebral space of the atlantoaxial joint on the left side and the obstruction of the nasopharyngeal cavity on cervical magnetic resonance image ( mri ) . we report a symptomatic case associated with rheumatoid atlanto - axial subluxation , which regressed after the surgical management . a 72-year - old woman presented with sudden severe headache in her left occipital area with dyspnea . a laboratory examination of the patient 's blood revealed no remarkable abnormality indicative of inflammation , but high - titer of rheumatoid factor ( 95.9 iu / ml ) . seven months ago before visiting to our department , she checked brain mri due to sudden attack of severe headache on left occipital area , which revealed non - specific lesion except rheumatoid pannus with small prevertebral cyst of c1 - 2 junction ( fig . , there was a large hemorrhagic cystic mass around prevertebral space of the atlanto - axial joint on the left side , obstructing the nasopharyngeal cavity on cervical mri ( fig . the cystic mass was connected to atlanto - axial joint capsule on axial view of computed tomography ( ct ) . multiple bony erosion , rheumatoid atlanto - axial instability including left tilted c1 - 2 subluxation and cranial settling were demonstrated . and there were loss of lordosis and retrolisthesis in c 3 - 4 , 4 - 5 ( fig . 3 ) . in the first operation stage , considering patient 's dyspnea , aspiration of the cystic lesion was performed via transoral approach with otolaryngology surgeon ( fig . after 0.5 cm - sized mucosal incision on left side oropharyngeal wall , residual material of the cyst was squeezed by forceps and removed by suction tools . in the second operation stage , there were rheumatoid atlanto - axial instability and retrolisthesis in c 3 - 4 , 4 - 5 , so it was followed by posterior occipito - cervical fusion that connected from occiput to c5 , using vertex screw & rod system and iliac bone graft ( fig . 5 ) . the patient was tolerable on her postoperative course and showed good respiration and relieved headache . cervical synovial cysts are rare and located at the c1 - 2 junction or lower cervical spine . only 24 cases of synovial cysts of the c1 - 2 junction have been reported in the literature5 ) . moreover , the pathogenesis of spinal synovial cysts remains unclear but is thought to be attributable to degenerative changes of the facet joints or excessive joint motion2,3,5,7 ) . it revealed multiple bony erosions and a large pannus formation of c1 - 2 junction on this case . this patient also showed atlanto - axial instability caused by rheumatoid arthritis , such as left tilted antlanto - axial subluxation and basilar impression . the atlantoaxial articulation is a true synovial joint and is responsible for a large proportion of normal cervical mobility . the etiology of articular cysts is unclear , but they are assumed to be degenerative because minor chronic damage to articular surfaces produces a reactive proliferation of synovium or fibrocartilage that includes loculated collections of mucinous fluid . so , we hypothesized that hemorrhagic event was developed as a result of microtrauma caused by rheumatoid atlantoaxial instability . it is known that the higher titer of rheumatoid factor , the more destructive manifestations of joint occur1 ) . the patient was in the controlled state in serologic inflammatory marker , such as esr(erythrocyte sedimentation rate ) and crp ( c - reactive protein ) . however , the high titer of rheumatoid factor , in spite of long standing medication of rheumatology , was related factor of progressive destruction of joints . we report a rare case of large hemorrhagic cyst on prevertebral space of left side c1 - 2 area associated with rheumatoid arthritis causing airway obstruction and left occipital pain , successfully managed anterior and posterior approach . we suggest repeated microtrauma due to atlanto - axial subluxation associated with rheumatoid arthritis as a main cause of hemorrhagic event on the cyst .
synovial cyst on prevertebral space of c1 - 2 joint is rare but may be associated hemorrhagic event . we describe a case of a 72-year - old woman who presented with sudden severe headache in her left occipital area with dyspnea . she had rheumatoid arthritis for 14-years . large hemorrhagic cystic mass was seen around prevertebral space of the atlantoaxial joint on the left side on cervical mri ( magnetic resonance image ) and it obstructed the nasopharyngeal cavity . aspiration of the cystic lesion was performed via transoral approach , followed by posterior occipito - cervical fusion . the specimen was xanthochromic , suggesting old hemorrhage . the patient was tolerable on her postoperative course and showed good respiration and relieved headache . we suggest that repeated microtrauma due to atalantoaxial subluxation associated with rheumatoid arthritis as a main cause of hemorrhagic event on the cyst .
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colchicine is a widely used drug for treatment of familial mediterranean fever ( fmf ) . clinical manifestations of colchicine intoxication include abdominal cramps , diarrhea , myotoxicity , hemolytic anemia and ( pan)cytopenia . a 9-years - old female patient , receiving colchicine for four months with a dose of 1 mg / day for fmf , was admitted to a hospital with gastrointestinal disturbance four days ago . she was referred to our hospital with liver and kidney dysfunction . at the initial physical examination her laboratory results revealed : hemoglobin 13.6 g / dl ; leukocyte , 9.610/l ; thrombocyte 5710/l ; fibrinogen 157 mg / dl ( 230 - 500 ) ; pt 26.1 sec ; inr 2.3 ; aptt 43.8 sec ; ldh 5329 iu / l ; ferritin 2320 g / l ( 10 - 55 ) ; triglyceride 7.1 mmol / l ( 0.32 - 1.46 ) . the patient was taken to intensive care unit ; vitamin k and fresh frozen plasma were administered . on the second day of her hospitalization hemoglobin was 9.6 g / dl ; leukocyte was 2.310/l ; neutrophil was 0.7210/l and thrombocyte was 2410/l . on peripheral blood smear , loss of lobulation in neutrophils was detected ( pelger - hut anomaly ) . laboratory tests revealed ferritin 54,632 g / l , triglyceride 7.4 mmol / l , and fibrinogen 63 mg / dl , serum creatinine 3.2 mg / dl ( 0.4 - 1.4 ) , serum blood urea nitrogen 43 mg / dl ( 6 - 21 ) , serum sodium 131 meq / l ( 134 - 148 ) , potassium 5.2 meq / l ( 3 - 4.8 ) , calcium 8.1 mg / dl ( 7.9 - 9.9 ) , and inorganic phosphate 2.1 mg / dl ( 2.4 - 4.7 ) . at the bone marrow aspiration , many pelger - hut cells were observed . soluble cd25 level was 2840 u / ml ( 220 - 710 ) , and creatine kinase level was 18,959 when the history of colchicine intoxication was detailed , during fmf attack - free period of five days prior to admission to the hospital , it is learned that the patient was upset over a quarrel between her parents , and that she had taken approximately 30 pills of her drug ( total of 15 mg ; 0.5 mg / kg body weight ) , at which vomiting and diarrhea complaints were started . the patient was administered hlh-2004 protocol and plasma exchange is applied twice . on the second day of therapy , severe hypotension , renal failure and av complete block occurred . the patient was resistant to all therapies and had died at the fourth day of her hospitalization . in the colchicine intoxication , the most affected organs are those that have a rapid cell turnover . liver damage , renal failure , arrhythmias , neuromuscular disturbances , and bone marrow depression can be detected in the later stage . the worst predicted doses were classified regarding the risk of toxicity as sub - toxic ( < 0.5 mg / kg ) , toxic ( 0.5 - 0.8 mg / kg ) , and lethal ( > 0.8 mg / kg ) doses . however , cases with cardiogenic shock and death were also reported with doses less than 0.5 mg / kg . although our patient had taken a dose of 0.5 mg / kg body weight colchicine , the patient died due to severe myelosupression , hlh and multi - organ failure . pelger - hut anomaly is a state of limitation in segmentation of lobes in neutrophils . a pelger - hut - like change in granulocyte morphology may occur as an acquired condition in several diseases . this same finding may be produced by certain drugs such as colchicine . on the peripheral blood smear of our patient , pelger - hut cells were detected in myeloid series , and as the history of colchicine use was detailed , it is learned that the patient had taken lots of colchicine pills . on the examination of concurrently taken bone marrow , pelger - hut cells were also detected in the myeloid series as well as hemophagocytosis . for this reason , we suggest that hlh was not due to her disease . to our knowledge , this is the first reported case of a patient with hlh associated with colchicine intoxication . in conclusion , if patients with colchicine intoxication present with pelger - hut anomaly in addition with unexplained fever , cytopenia , organomegaly , and biochemical changes , hlh should be investigated for prompt diagnosis and treatment .
colchicine is frequently used in the treatment of familial mediterranean fever ( fmf ) . first symptoms of colchicine intoxication are gastrointestinal disturbances , such as abdominal cramps , diarrhea , pancytopenia and so on . herein , we report a female fmf patient with pancytopenia and hemophagocytic lymphohitiocytosis ( hlh ) , following colchicine intoxication for committing suicide . to our knowledge , this is the first reported case of a patient with hlh associated with colchicine intoxication .
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to investigate the anti - ulcerogenic properties of the chloroform extract of flemingia strobilifera root in rats . other anti - ulcer related activities of the extract such as the effects on free radicals and antimicrobial activity were also evaluated . chloroform extract of flemingia strobilifera root was found to be safe up to 300 mg / kg body weight when administrated orally in female wistar rats . pretreatment with chloroform extract of flemingia strobilifera root reduced the characteristic lesions in a dose dependent manner ( p<0.001 ) when compared with the control . pretreatment with chloroform extract of flemingia strobilifera root at a dose of 15 and 30 mg / kg body wt . increased the gastric mucosal glutathione level , total protein content significantly ( p<0.001 ) as compared to control group . whereas there is significant ( p<0.05 , p<0.001 ) reduction in gastric mucosal malonaldehyde levels when compared to control . free radical scavenging activity of chloroform extract of flemingia strobilifera root was observed in the concentration range tested , the ic50 value was calculated . antimicrobial activity of the chloroform extract of flemingia strobilifera root exhibited activity against both gram positive and negative bacteria at concentration of 10 mg / ml . the root extract of flemingia strobilifera possess antiulcerogenic properties could justify folklore uses of the plant in peptic ulcer diseases .
purpose : to investigate the anti - ulcerogenic properties of the chloroform extract of flemingia strobilifera root in rats.methods:anti-ulcer effect was evaluated by water immersion induced ulcer in rats . other anti - ulcer related activities of the extract such as the effects on free radicals and antimicrobial activity were also evaluated.results:chloroform extract of flemingia strobilifera root was found to be safe up to 300 mg / kg body weight when administrated orally in female wistar rats . water immersion stress produced characteristic lesions in the glandular portion of the rat stomach . pretreatment with chloroform extract of flemingia strobilifera root reduced the characteristic lesions in a dose dependent manner ( p<0.001 ) when compared with the control . pretreatment with chloroform extract of flemingia strobilifera root at a dose of 15 and 30 mg / kg body wt . increased the gastric mucosal glutathione level , total protein content significantly ( p<0.001 ) as compared to control group . whereas there is significant ( p<0.05 , p<0.001 ) reduction in gastric mucosal malonaldehyde levels when compared to control . free radical scavenging activity of chloroform extract of flemingia strobilifera root was observed in the concentration range tested , the ic50 value was calculated . antimicrobial activity of the chloroform extract of flemingia strobilifera root exhibited activity against both gram positive and negative bacteria at concentration of 10 mg / ml.conclusion : the root extract of flemingia strobilifera possess antiulcerogenic properties could justify folklore uses of the plant in peptic ulcer diseases .
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ocular cysticercosis is caused by the growth of the larval form of taenia solium within ocular tissues.1,2 the cysts may be located in descending order of frequency in the subretinal space ( 35% ) , vitreous ( 22% ) , conjunctiva ( 22% ) , anterior segment ( 5% ) and orbit ( 1%).3 intraocular cysticercosis usually presents with reduced vision and ocular inflammation . it is believed that the larva reaches the subretinal space through the posterior ciliary arteries.3 - 6 as the cyst develops , it may cause exudative retinal detachment.6 as long as the cyst remains viable , it evokes little or no inflammatory response . once the cyst starts degenerating , an antigen , which may be a metabolic by - product or toxin , leaks from the cyst and induces an inflammatory reaction , manifesting as vitritis , uveitis and sometimes endophthalmitis.3 - 6 most often , the characteristic intraocular cyst can be visualized by indirect ophthalmoscopy or detected by b - scan ultrasound in the presence of hazy media.3 b - scan ultrasonography will show a curvilinear echo corresponding to the cyst wall together with an eccentric hyperechoic dot suggestive of the scolex . a - scan analysis reveals two high amplitude echoes representing the anterior and posterior walls of the cyst . mahendradas et al7 highlighted the features of intraocular cysticercus cyst employing spectral domain oct ; they clearly delineated the hyper - reflective wall of the subretinal cyst with a more hyper - reflective portion within its wall suggestive of the scolex / larva . cns involvement can be observed in approximately 90% of patients with ocular cysticercosis and mri is superior to computed tomography ( ct ) in detecting lesions of neurocysticercosis ( ncc).8 in addition , mri better illustrates cystic lesions in the base of the brain , cerebrospinal fluid ( csf ) spaces as in ventricular ncc and cisternal ncc , and also with intramedullary lesions.9 the scolex may be more readily apparent on mri than on ct . on mri , contents of live cysts ( vesicular stage ) are isointense relative to csf on t1- and t2-weighted images.10 treatment of ocular cysticercosis is mandatory since it has been reported that 80% of untreated cases result in severe ocular damage.3 antihelminthic drugs such as praziquantel or albendazole are effective in central nervous system and skin cysticercosis . . surgical removal of the cyst can also be performed through transretinal or trans - scleral routes.11 systemic corticosteroid coverage is required before and after surgical removal of the cysticercus.3
a 31-year - old man of asian descent presented with loss of vision in his right eye from 6 months earlier . best corrected visual acuity ( bcva ) was limited to light perception in the right eye and was 6/6 , n6 in the left one . slit lamp examination revealed normal anterior segments bilaterally . intraocular pressure was 8 and 14 mmhg in the right and left eyes , respectively . fundus examination in the right eye showed a large subretinal cysticercus cyst , accompanied by total retinal detachment ( rd ) , severe extensive subretinal fibrosis and membranes ( fig . 1a).b - scan ultrasound ( alcon ultrascan , alcon laboratories , fort worth , texas , usa ) images illustrated total rd with a hyperechoic area within the cystic cavity suggestive of cysticercus scolex ( fig . 1b ) . spectral domain optical coherence tomography ( oct ) ( topcon 3d oct-2000 , topcon medical systems , oakland , new jersey , usa ) demonstrated a highly reflective cyst wall and a more hyper - reflective dome - shaped structure within the wall suggestive of the scolex ( fig . 1c ) . the posterior extent of the cyst could not be visualized due to its large size . t1-weighted contrast - enhanced magnetic resonance imaging ( mri ) of the brain demonstrated a ring - shaped enhancing lesion in the left cerebellar hemisphere with perilesional brain edema suggestive of neurocysticercosis ( fig . 1d ) . the patient was referred to a neurophysician and received a three month course of oral albendazole and steroids . eventually , he was recommended for follow - up care .
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a 60-year - old gentleman presented to our institution with pain and dimness of vision in both eyes . on evaluation , his best corrected visual acuity ( bcva ) was 20/80 in right eye ( re ) and 20/120 in left eye ( le ) . his cup - to - disc ratio was 0.6 re and 0.85 le with loss of the neural rim . he was prescribed betoxolol 0.5% in both eyes ( be ) and recommended elective cataract extraction for the le . keratometry measurements were 43.75 diopter ( d ) 180 and 40.00 90 os with 3.75 d of against the rule astigmatism . the calculated intraocular lens ( iol ) power was 23.50 d. the patient had used topical ofloxacin 3 mg / ml six times , 1 day prior to surgery , and thrice on the day of surgery at 1-hour intervals . povidone iodine 5% was used half an hour before surgery and just before the commencement of surgery . ocular adnexa was cleaned using 10% povidone iodine , the patient was draped and speculum was applied . a manual sutureless cataract extraction ( mscs ) with lri was planned for the le . through a 6-mm temporal sclerocorneal tunnel , a nasal 6-mm lri was performed using a 550-m steel knife prior to wound construction . on the first postoperative day , his bcva le was 20/60 , and the anterior segment was unremarkable except for mild iritis . on the 15 postoperative day , he presented emergently with pain and redness in the operated eye . on examination , anterior segment examination revealed circumcorneal congestion , iris nodule , a 1.5-mm hypopyon , and a corneal exudate at the site of the prior lri [ fig . 1 ] . b - scan ultrasonography revealed minimal vitreous exudates . on presentation at 15 post - op day with endothelial nodule at the site of limbal relaxing incision , hypopyon and iris nodule vitreous was aspirated and sent for analysis . intravitreal vancomycin 1 mg / ml and amikacin 400 g/0.1ml were administered . based on the clinical picture of corneal exudate , iris nodules , and hypopyon with minimal posterior segment involvement , a clinical diagnosis of nocardial endophthalmitis was made . the patient was treated with topical gatifloxacin and amikacin hourly , prednisolone acetate every 6 hours , and oral gatifloxacin . the isolate was sensitive to amikacin , gatifloxacin , moxifloxacin , ciprofloxacin , ofloxacin , and chloramphenicol . the inflammation worsened , exudates from the iris extended into the capsular bag and fresh exudates appeared at the sclerocorneal wound site . a subsequent ultrasound revealed increased vitreous opacities . on the 18th postoperative day , the patient underwent core vitrectomy with anterior chamber washout and capsular bag removal with iol explantation . his symptoms improved , iris and endothelial nodules resolved , hypopyon decreased and exudate at the lri site resolved [ fig . 2 ] . he was discharged after a 1-week course on a regimen of oral gatifloxacin and topical amikacin , ciprofloxacin , and a cycloplegic . four weeks later , the patient 's vision was 20/2000 and his examination revealed 2 + white blood cells ( wbcs ) in the anterior chamber , no hypopyon , and vitreous opacities . lri preserves the perfect optical qualities of the cornea and is an excellent option for low - to - moderate degrees of astigmatism in a planned single bioptic procedure . lri produces lesser effect than corneal relaxing incision ( cri ) , thus precise alignment of the axis is not as critical . have shown that lri performed during phacoemulsification surgery is a safe , effective , and stable procedure to reduce pre - existing corneal astigmatism . we are unaware of any prior report of a postoperative endophthalmitis associated with an lri performed at the time of cataract surgery . in this patient , as the lri was of partial thickness and cornea was not perforated , it appears that bacteria may be able to penetrate the cornea at the site of the lri . this patient presented with iris nodules and hypopyon , a classic presentation of nocardial endophthalmitis . the endothelial exudates were at the lri site while the wound was clear , thereby indicating the lri as the probable site of entry for the organism . the rate of postoperative endophthalmitis after cataract surgery in this population is 0.064% and norcardial infections account for 16.4% of these cases . norcardia endophthalmitis is more evident in emaciated and immunocompromised individuals and associated with poor prognosis . mscs is practiced widely in india and constitutes about 62% of the total 69,479 cataract procedures done at our institute in 2008 . the endophthalmitis rate with the mscs group was 0.03% and was lesser than the 0.05% infection rate with the phacoemulsification group ( unpublished data ) . have proved that poorly constructed wounds with a short corneal valve have a higher risk of developing endophthalmitis . in this patient , the scleral and corneal valves were 2 mm and 1.5 mm , respectively . in spite of making a larger incision in the mscs group , the infection rates are probably comparable due to the wider sclerocorneal tunnel covered well with conjunctiva at the end of surgery . lalitha et al . from the same population reported nil endophthalmitis in the 4275 cases who underwent mscs technique in the total group of 36,072 cataract procedures where the overall endophthalmitis rate was 0.05% . in our experience , the mscs technique per se has not been found to be a significant risk factor for endophthalmitis compared to phacoemulsification . in summary
limbal relaxing incisions ( lris ) are considered a relatively safe procedure with rapid stabilization and absence of infectious complications . do we need to readdress this last impression ? we report a case of nocardia endophthalmitis associated with an exudate at the site of an lri in a patient who underwent routine cataract surgery . this case , to the best of our knowledge , is the first report of its kind , stressing the need for a cautious approach to the adoption of this method of astigmatic correction .
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he had suffered from intermittent headache for 2 months and had taken benzodiazepines and analgesics under the diagnosis of somatoform disorder at a local clinic . he had a 5-day history of diplopia and a 3-day history of blurred vision that progressed to blindness during the preceding 24 hours . on admission his blood pressure was 130/70 mmhg , pulse rate was 82/min , respiratory rate was 20/min , and body temperature was 36.3. on neurologic examination the patient was alert and exhibited no meningeal signs . only light perception was possible in both eyes , but no other neurological abnormality was noted . lumbar puncture showed the opening pressure of 160 mm h2o with clear cerebrospinal ( csf ) fluid . csf evaluation showed 30 red blood cells / mm , 80 white blood cells / mm , 80% lymphocytes , 41 mg / dl glucose , and 114 mg / dl protein . his body temperature increased to 38.8 1 day after admission , but cbc revealed no leukocytosis . brain mri showed no meningeal enhancement , parenchymal lesion , or intracranial vascular abnormality supplying the optic nerve , optic chiasm , or optic tract . the results of fundoscopic examinations were unremarkable , and visual evoked potential showed no wave formation on bilateral pathways ( fig . an india ink smear of the csf demonstrated encapsulated yeast , and cryptococcus neoformans was cultured ( fig . he was found to be seropositive for hiv with a high antibody titer ( 53.87 s / co ) . he was treated with both antiretroviraland antifungal agents , and his visual acuity had improved upon discharge . neuro - ophthalmic lesions are present in 6% of patients with hiv infection during the course of the disease , with most of them being are attributable to cryptococcal meningitis.3 cryptococcus neoformans , the cause of cryptococcal meningitis , is the fourth most common source of life - threatening infection in aids patients after infections of cytomegalovirus , pneumocystis carinii , and mycobacterium avium intracellulare.1 it is present in pigeon droppings and infects by inhalation of contaminated soil . cryptococcal meningitis is fatal in hiv - infected patients if not treated , and hence early diagnosis is very important . the signs and symptoms of cryptococcal meningitis include headache ( 80~92% of cases ) , meningeal signs ( 50~80% ) , nausea / vomiting ( 40~80% ) , fever ( 36~67% ) , and visual disturbances ( 33~47%).1 our patient presented with acute blindness without other definite clinical symptoms at the time of admission . possible mechanisms for binocular blindness due to cryptococcal meningitis include direct fungal infiltration of the optic nerve , optic chiasm , or optic tracts , adhesive arachnoiditis , cerebral vasculitis , and intracranial hypertension.4 it has been suggested that rapid - onset visual loss is caused by infiltration of the optic nerve or optic chiasm , while slow - onset visual loss is due to increased csf pressure.4 a csf opening pressure exceeding 200 mmh2o and papilledema reflect intracranial hypertension , but our patient showed a normal csf opening pressure and unremarkable fundoscopic examination findings . moreover , his visual symptoms developed very early in the course of the disease . thus , the sudden visual loss might have been due to retrobulbar fungal infiltration . whereas the prevalence of cryptococcosis is decreasing because of the widespread availability of antiretroviral therapy , cryptococcal meningitis is still a fatal complication of hiv infection . thus , both early diagnosis of cryptococcal meningitis and detection of the underlying causes are important . in our opinion , unexpected sudden binocular blindness should be considered as a possible initial manifestation of cryptococcal meningitis related to hiv infection .
ocular complications of hiv - related cryptococcal meningitis are reasonably common , but complete binocular blindness as the first manifestation of hiv is extremely rare . a 58-year - old man presented with binocular blindness . he experienced blurred vision for 3 days before the blindness . mild pleocytosis was present in the cerebrospinal fluid , from which cryptococcus neoformans was cultured . serology revealed positivity for hiv antibody . he was treated with antifungal and antiretroviral therapy . this case indicates that hiv - related cryptococcal meningitis should be taken into consideration when determining the cause of unexpected sudden binocular blindness .
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most common cause of lower gastrointestinal hemorrhage ( lgib ) in elderly patient is diverticular disease and mortality rate is very low 24% . a massive lgib from primary ilio - rectal fistula is rare and difficult to diagnosis but more common from secondary after previous vascular surgery a variety of treatment options are available , such as in situ graft , closure of defect alone , endovascular stent graft , extra - anatomical bypass and embolic coiling . exploration using laparotomy for ligation internal iliac aneurysm and rectal resection is a simple operative procedure which can be applied when emergency option is needed to save a life . we describe successful emergency surgery in primary ilio - rectal fistula when patient presents a massive lgib and hypovolemic shock . an 88-year - old man , his underlying diseases are hypertension , dyslipidemia , moderate aortic stenosis and mitral valve stenosis , chronic kidney disease and prostate cancer . at emergency department , physical exam showed hypotension blood pressure 70/50 mmhg and per rectal exam showed blood clot with enlarged prostate . after resuscitation until stable vital sign , he was sent for colonoscopy but can not be evaluated due to a lot of blood clots in the rectum . he was admitted for observation and we planned to repeat colonoscopy again following day because he was stable and bleeding was stopped . following day after admitting , he had massive lower gastrointestinal bleeding again fresh blood ~1000 ml from his anus . but the patient had a problem vascular access site and then send to computed tomography angiography ( cta ) for evaluate access site . we found 3 cm aneurysm of right internal iliac artery with partial thrombus and attached to rectosigmoid colon with leakage of contrast into sigmoid lumen ( figs 12 ) . he was sent to operating room during transferring we gave blood transfusion and then emergency explore laparotomy was performed , the operation performed by partial aneurysmectomy , ligated right internal iliac , wedge resection rectum wall and temporary abdominal closure with swab packing due to bowel distension and abdominal pressure was high . he was sent to operating room for change swab packing on day 3 and permanent abdominal closure on day 5 . during postoperative period primary aorto / ilio - enteric fistula ( paef ) is very rare , but secondary fistula is more common . sir astley cooper was the first to describe a paef as a condition caused by spontaneous erosion of the aorta into the gastrointestinal tract . etiology of paef are several causes , most causes are due to aneurysm , infection , tumor , radiotherapy and foreign body ingestion [ 59 ] . the most common site of fistula located in duodenum 54% , esophagus 28% , small bowel and large bowel 15% . the classical triad of symptoms associated with a fistula is gastrointestinal hemorrhage , abdominal pain and a pulsating abdominal mass . most common sign and symptoms of fistula is gastrointestinal bleeding , but only 11% presented with classical triad . the diagnosis of paef is difficult to make . because most of patients were send to endoscope due to gastrointestinal bleeding symptoms . several diagnosis investigations were used when patients are clinically stable such as cta , angiography , colonoscopy , and red blood cell scan . all of these do not constitute specific investigation . however , ct scan can diagnose specifically aorto / ilio - enteric fistula ( for example , air within aortic wall or contrast fill in bowel lumen ) that detection rate 61% when failure diagnosis from endoscopy . such as , in situ graft , closure of defect alone , endovascular stent graft , extra - anatomical bypass and embolic coiling . optional treatment depends on surgeon s experience , hospital facilities and the type of paef . this case was send to the operating room after diagnosis right internal ilio - rectal fistula for emergency exploration with laparotomy because the patient s active bleeding and vital signs were not stable preventing adequate preparation time for intervention or stent graft in hospital . we decided to ligate right internal iliac artery , wedge resection rectum with suture repair because this is a simple operation and can save patient s life . other options could be done by endovascular therapy or embolic coiling if we had time to prepare and the patient was already stabilized in this hospital . in conclusion , paef is very rare . the patient who has massive lower gastrointestinal bleeding with failure of endoscope may need to consider imaging to rule out paef . there are several treatment options for paef but no conclusive specific treatment it depends on patient s situation and physician s experience . although endovascular or embolization is minimally invasive surgery and good for the patient who is a high operative risk but an emergency simple operation is an effective procedure not only saves patient s life but also can be done by a general surgeon ( fig . figure 1:axial view iliac aneurysm with partially thrombose contact rectosigmoid colon and contrast leak.figure 2:coronal view iliac aneurysm.figure 3:cta after 1 year .
abstractmassive lower gastrointestinal from primary isolated ilio - rectal fistula is a rare condition and difficult to diagnosis and emergency surgery for this situation has a high mortality rate . this report describes a successful operation in an 88-year - old man at present with massive lower gastrointestinal hemorrhage from ilio - rectal fistula and hypovolemic shock . underlying diseases are prostate cancer , hypertension , dyslipidemia , aortic stenosis , mitral valve stenosis and chronic renal disease . operative treatment is to explore laparotomy with internal iliac artery ligation and rectal resection . during postoperative period patient developed acute cholecystitis and treated by cholecystectomy 1 month after operation the patient went home without morbidity and 1-year follow - up he had not any complications .
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the other types of internal hernia that have been described include transmesenteric , supra- and/or perivesical , intersigmoid , foramen of winslow , and rarely , omental hernias . paraduodenal hernia is usually a congenital anomaly due to non - fusion of the mesocolon with the parietal wall that leaves a potential space . it manifests commonly in adults in the age group of 5060 years and usually presents as intestinal obstruction . internal hernias in todays era of advanced laparoscopic surgery are usually due to rents or defects in the mesentery . we present a case of intestinal obstruction due to internal herniation following laparoscopic donor nephrectomy , followed by a brief discussion . a 47-year - old lady presented with a history of vomiting and constipation for the past 5 days . upper gastrointestinal ( gi ) endoscopy revealed large amount of bile stained fluid in the stomach . a cect suggested dilatation of the stomach , duodenum and proximal jejunum with features of bowel obstruction in the mid jejunum , with the loops lying in left renal fossa [ figure 1 ] . patient was explored laparoscopically , through the same port sites that were made earlier for the nephrectomy ( two 10 mm ports , one at the level of umbilicus on the left side lateral to rectus muscle and another in the midclavicular line just above the left anterior superior iliac spine ; two 5-mm ports , one subcostal in the midclavicular line and another in the anterior axillary line in the lumbar region ) . herniation of small bowel was noted in the left paraduodenal space [ figure 2 ] . the herniation had occurred through a defect in the transverse mesocolon which probably would have occurred during the donor nephrectomy procedure . the loop of bowel was densely adhered to the tail of the pancreas and had to be dissected of it and reduced [ figure 3 ] . patient had an uneventful recovery and was discharged from hospital on the 5 postoperative day . cect scan showing obstructed bowel loops in the left renal fossa image showing herniated bowel loops image showing obstructed bowel loops being separated from the tail of pancreas image showing the end result following the closure of the mesocolic defect the increased use of laparoscopy for colonic resections , bariatric surgery , specifically the procedure of gastric bypass and laparoscopic nephrectomies have resulted in an increase in the postoperative complication of intestinal obstruction due to internal hernias . internal hernias have also been reported after liver transplantation specifically where the biliary continuity is restored through a roux - en - y choledochoenteric anastomosis . these hernias usually occur through the mesenteric or mesocolic defects that are either deliberately or inadvertently made during the above mentioned procedures and are not subsequently closed . in view of the fact that internal herniation of small bowel occurs through the transmesenteric or transmesocolic defects , it is generally recommended that these rents or openings should be specifically sutured and closed to prevent postoperative intestinal obstruction . in a retrospective review of 1,064 cases of laparoscopic gastric bypass , comeau and colleagues strongly recommended complete closure of all mesenteric defects to avoid internal hernias and their associated complications . however , following laparoscopic right colectomy for neoplasia in 530 consecutive patients , over a 7-year period , cabot et al . the incidence of intestinal obstruction was not statistically significant in their series when these transmesenteric defects were not sutured . they , however , stressed the need for more extended long - term follow - up in these series . most publications dealing with internal hernias following laparoscopic procedures in todays age do insist on closing mesenteric defects.[58 ] the diagnosis of intestinal obstruction due to internal herniation is usually difficult to be made by clinical signs and symptoms . plain radiographs as well as contrast - enhanced ct may sometimes suggest upper small gut obstruction . cect usually shows the jejunum massed in the left renal fossa as was reported in our case . though our case was not a true congenital paraduodenal hernia , the internal herniation had occurred into the left paraduodenal space through the mesocolic defect . surgical management of internal hernias has been traditionally performed by laparotomy , reduction of the hernia contents and closure of the defect . subsequently , laparoscopic repairs , using mesh as well as meshless have been performed by many groups . intestinal obstruction due to internal hernias following laparoscopic radical nephrectomy and laparoscopic donor nephrectomy , though few , have been reported in the literature . management in most of these cases has been exploration through either a laparotomy or laparoscopy . if the entrapped bowel is viable and not gangrenous , it can be usually reduced laparoscopically and the mesenteric or mesocolic rent sutured subsequently , as was done in our case . laparoscopic donor nephrectomy is the procedure of choice today , for procurements of kidneys across many centres , worldwide . small bowel obstruction due to internal hernia following this procedure though rare can lead to significant morbidity in an otherwise normal and healthy person . we do stress the need to be vigilant while mobilizing the colon and to suture any mesenteric or mesocolic defect deliberately or inadvertently made during the procedure . the other aspect to be highlighted is that in the current era , laparoscopy can be the modality of management especially in those patients who are haemodynamically stable and there is no suggestion of bowel necrosis or gangrene .
internal hernias are a rare cause of small bowel obstruction . following laparoscopic bariatric surgery , specifically gastric bypass and laparoscopic colonic resections , there has been an increase in the incidence of internal hernias . this has been due to either a mesenteric or mesocolic defect being not closed or completely missed . small bowel loops usually herniate through these defects and present as intestinal obstruction . internal hernia following laparoscopic donor nephrectomy is a rare complication . the need for presenting this case is the rarity of its occurrence , to stress the fact that following major abdominal laparoscopic surgery the mesenteric or mesocolic defects should be closed , and that this complication was managed laparoscopically , through the same port sites as used earlier for the donor nephrectomy .
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methods and any associated references are available in the online version of the paper at http://www.nature.com / naturemethods/. difference in optimal collision energies for identification and quantification supplementary note real - time filtering supplementary table 1 summary of quantification and identification results supplementary protocol implementing quantmode on commercial mass spectrometers
we describe a mass spectrometry method , quantmode , which improves the accuracy of isobaric tag based quantification by alleviating the pervasive problem of precursor interference co - isolation of impurities through gas - phase purification . quantmode analysis of a yeast sample contaminated with interfering human peptides showed substantially improved quantitative accuracy compared to a standard scan , with a small loss of spectral identifications . this technique will allow large - scale , multiplexed quantitative proteomics analyses using isobaric tagging .
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the majority of swallowed indigestible foreign bodies pass through the gastrointestinal tract without complications [ 1 , 2 , 3 , 4 ] . however , there are three physiological narrowings involving the pylorus , duodenal c - loop and ileocecal valve . foreign bodies longer than 10 cm , such as a toothbrush , can not negotiate the duodenal c - loop due to its fixed retroperitoneal position . these objects should be endoscopically removed as soon as possible to avoid pressure necrosis and gastrointestinal perforation [ 5 , 6 , 7 ] . if endoscopic removal fails or there is evidence of obstruction or perforation , laparoscopic gastrotomy should be performed . an 18-year - old caucasian woman with no previous history of related medical problems was admitted to the department of internal medicine , division of gastroenterology , clinical hospital split because she had accidentally swallowed a toothbrush . the patient admitted she had been using the toothbrush to induce emesis . on presentation , 2 h after ingestion , she was asymptomatic and her vital signs were within normal limits . a plain abdominal x - ray study confirmed the presence of the foreign body in the left upper abdominal quadrant ( fig . informed written consent for upper gastrointestinal endoscopy was obtained from the patient and her parents . esophagogastroduodenoscopy revealed the toothbrush in the stomach with its head positioned against the gastric fundus . the extracted toothbrush was 20 cm long . repeated upper gastrointestinal endoscopy was performed 4 h later and showed no evidence of mucosal lesion to the stomach or the esophagus . the patient was discharged home in excellent clinical condition after being observed for 6 hours . foreign bodies in the stomach will pass uneventfully through the gastrointestinal tract in 8090% of cases [ 1 , 2 , 3 ] . however , foreign objects longer than 10 cm , such as a toothbrush , can not negotiate the duodenal c - loop due to its fixed retroperitoneal position . in such cases , these objects should be removed as soon as possible to avoid pressure necrosis and gastric perforation [ 2 , 3 , 4 , 5 ] . removal of long foreign bodies from the stomach is influenced by the patient 's clinical condition and technical abilities of the endoscopist [ 2 , 3 , 4 , 5 ] . if endoscopic removal fails or there is evidence of obstruction or perforation , surgical gastrotomy should be performed . we had no need for conscious sedation since the patient was actively participating during the procedure . special attention has to be paid during the extraction of the toothbrush to its alongside alignment with the esophagus . otherwise , this most critical and demanding part of the extraction procedure may easily result in mucosal damage or foreign body impaction . the second important phase of the extraction procedure is when the foreign body reaches the oropharynx . the patient has to extend his head backwards and the endoscopist has to reach for the toothbrush with his hand and pull it out . this case report describes a rare case in whom a toothbrush was safely extracted from the stomach endoscopically by snare extraction . the procedure is brief , does not require conscious sedation , and the patient can be discharged from hospital after a few hours . early endoscopic retrieval of the toothbrush is critical for reducing morbidity and mortality . in cases when endoscopic removal fails
most ingested foreign bodies will pass uneventfully through the gastrointestinal tract . nevertheless , long and rigid foreign bodies are associated with an increased risk of gastrointestinal impaction , perforation and bleeding . moreover , there has been no case of spontaneous passage of a toothbrush reported . therefore , the prompt removal of such ingested foreign objects is recommended before complications develop . this case report describes a case of an 18-year - old woman who accidentally swallowed her toothbrush . the toothbrush was successfully removed via flexible endoscopy using a polypectomy snare . a swallowed toothbrush is a special clinical challenge . early endoscopic retrieval of the toothbrush is critical for reducing morbidity and mortality . in cases when endoscopic removal fails , a laparoscopic surgical approach may be an alternative .
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a 55-year - old man presented at our emergency department with sudden - onset sharp chest pain that had started two hours previously . he also complained of progressive swelling and bruising on his neck , and the development of dysphagia and dyspnea over the previous two hours . he had a history of admission with spontaneous hemothorax seven years previously , and at that time , despite several work - ups for hemothorax , its origin was not found . other than this incident , he had hypertension but no history of gastrointestinal or bleeding disorders . upon arrival at the emergency department extensive neck swelling was observed , as well as bruising on the neck and upper chest . during the examination , he suddenly vomited bright red blood with food material three times , and approximately a quarter - cup of blood was generated on each occasion . other laboratory tests , including cardiac markers and his coagulation profile , were within their normal ranges . 1b ) revealed extensive non - enhancing soft tissue lesions in the posterior mediastinum and neck , which were suspected to be hematomas . emergency gastroscopy showed many blood clots but no definite focus of gastrointestinal bleeding , such as an ulcer , mass , or perforation . instead , gastroscopy showed extrinsic esophageal compression . since the patient was hemodynamically stable , conservative management was adopted in the intensive care unit . followup ct on the third day post - admission showed a focal aneurysm in the right bronchointercostal trunk ( fig . subsequent selective bronchial arteriography revealed a 1-cm aneurysm in the right bronchial artery ( fig . since the aneurysm was considered to be the cause of the hematemesis and mediastinal hemorrhage , we decided to perform embolization to prevent recurrence . the right bronchial artery was subsequently embolized with four microcoils ( vortx-18 ; boston scientific co. , marlborough , ma , usa ) and 33% glue ( fig . 2c ) , and repeat ct showed that the mediastinal hematoma had nearly disappeared ( fig . the patient was discharged without complications . over the course of one year of follow - up spontaneous mediastinal hemorrhage can develop as a result of trauma , aortic dissection , the valsalva maneuver , or iatrogenic procedures . the rupture of a bronchial artery aneurysm ( baa ) is also known to result in mediastinal hemorrhage . when a baa ruptures into the mediastinum , most patients present with chest pain , hemothorax , or hemomediastinum . furthermore , if hematemesis is a prominent symptom of baa rupture , it can be confused with boerhaave s syndrome , variceal disease , or a perforated ulcer . to the best of our knowledge , only three cases of baa presenting with hematemesis have been reported in the literature [ 46 ] . in one case , a pinhole connection between the aneurysm and esophagus was found during an endoscopic examination , but in the present case , no evidence of communication was found between the aneurysm and the esophagus . we first suspected that the mediastinal hemorrhage and hematemesis were caused by the perforation of an esophageal ulcer , but repeated gastroscopy showed a normal esophagus . the only abnormal finding was the finding of a baa without evidence of extravasation three days after the onset of symptoms . after ruling out the possibility of mediastinal hematoma , the baa was the only remaining possible source of the mediastinal hemorrhage . since the baa was regarded as the source of the mediastinal hemorrhage and hematemesis , it was embolized to avoid recurrence . the treatment of a ruptured baa depends on the patient s hemodynamic status and the presence of cardiorespiratory compromise . if the patient is hemodynamically stable , endovascular embolization is considered to be the first - line management strategy , and surgery should only be considered when embolization is contraindicated , as in patients allergic to contrast medium or when a medullary artery is involved . in patients presenting at an emergency department with hematemesis and mediastinal hemorrhage ,
hematemesis is a rare manifestation of a ruptured bronchial artery aneurysm ( baa ) in the mediastinum . it is difficult to diagnose a ruptured baa presenting as hematemesis , because it can be confused with other diseases , such as boerhaave s syndrome , variceal disease , or a perforated ulcer . in this report , we describe a case of baa resulting in hematemesis and mediastinal hemorrhage .
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in the previous issue of critical care , kmpers and colleagues demonstrated a direct correlation between increased peripheral blood levels of the vascular growth factor , angiopoietin ( angpt)-2 , and mortality in 43 critically ill adults with sepsis . endothelial injury is one of the main hallmarks of sepsis , leading to capillary leak , microcirculatory dysfunction , organ failure , and eventual death in many critically ill patients . angpt-1 and angpt-2 are two of the best - characterized members of a family of endothelial - derived vascular growth factors necessary for both normal and pathologic angiogenesis and vasculogenesis . both angpt-1 and angpt-2 appear to bind to the tyrosine kinase receptor , tie-2 , found primarily on the luminal surface of endothelial cells . recent studies have also shown that the tie-2 receptor may be found on certain populations of peripheral blood monocytes , although the function and role of the tie-2 receptor in the host innate immune response remain relatively unexplored . angpt-1 is a tie-2 agonist and promotes endothelial stabilization and quiescence , whereas angpt-2 is a tie-2 antagonist and promotes endothelial activation , destabilization , and inflammation . as such , the relative balance between angpt-2 and angpt-1 at the tie-2 receptor may be more relevant to the pathobiology of sepsis than the absolute levels of the individual growth factors . several studies have demonstrated increased peripheral blood levels of angpt-2 in critically ill patients with sepsis [ 5,7 - 9 ] , multiple trauma , acute lung injury ( ali ) , and cardiopulmonary bypass when compared with healthy controls . more importantly , increased angpt-2 levels appear to be associated with adverse outcomes [ 5,6,9 - 12 ] . for example , the study of kmpers and colleagues showed that increased peripheral blood angpt-2 levels correlated with surrogate markers of tissue hypoxia , disease severity , and mortality in 43 critically ill adults with sepsis . also of note , consistent with the opposing roles of angpt-2 and angpt-1 on the tie-2 receptor , peripheral blood levels of angpt-1 were significantly lower in the patients with sepsis compared with healthy controls . unfortunately , in the study of kmpers and colleagues , similar to the aforementioned studies , the temporal kinetics of angpt-1 and angpt-2 were not assessed as blood samples were collected upon the first day of admission to the intensive care unit only . angpt-2 is stored in the weibel - palade bodies within endothelial cells in a more or less prepackaged form . it is therefore not surprising that angpt-2 levels are increased early in response to endothelial activation or injury . whether angpt-2 levels remain increased in critically ill patients with sepsis has not been directly addressed and is a question for future investigation . it is certainly tempting to speculate that peripheral blood angpt-2 levels would be an ideal biomarker of early endothelial activation and injury . similarly , whether angpt-1 levels remain decreased in critically ill patients who eventually succumb to their illness is an interesting question . angpt-1 may be a biomarker of endothelial recovery ; however , given its purported anti - inflammatory role , angpt-1 would appear to be an attractive therapeutic target as well . to this end , several studies have suggested that manipulating the ratio of angpt-2 to angpt-1 by augmenting angpt-1 levels may represent an ideal therapeutic strategy for patients with sepsis and ali . important translational laboratory studies are necessary to show that increased angpt-2 levels in critically ill patients are more than just an epiphenomenon . the role of angpt-2 in the pathobiology of sepsis and ali needs to be further elucidated by using in vitro cell - based studies and animal models of critical illness . similarly , the presence of the tie-2 receptor on certain subpopulations of peripheral blood monocytes suggests a larger role for angpt-2 in the host innate immune response . finally , manipulation of the angpt / tie-2 system may be a rational therapeutic strategy for the management of critically ill patients with sepsis and ali . all of these questions remain an active focus in several laboratories , including our own . the authors ' research is funded by the national institutes of health ( bethesda , md , usa ) ( grant numbers 5ko8gm077432 and 1r03hd058246 ) .
the early recognition and management of sepsis remain the greatest challenges in the field of critical care medicine . endothelial injury is one of the hallmarks of sepsis , leading to capillary leak , microcirculatory dysfunction , organ failure , and eventual death in many critically ill patients . the angiogenic growth factors , angiopoietin ( angpt)-1 and angpt-2 , act upon the tie-2 receptor in opposing roles . angpt-2 has been found in abundance in septic patients when compared with healthy controls . in the study by kmpers and colleagues in the previous issue of critical care , angpt-2 levels correlated with markers of tissue hypoxia , disease severity , and mortality in septic adults . however , the temporal kinetics of the angiopoietins were not assessed . it remains to be seen whether angpt-2 levels will function solely as an early marker of sepsis or whether the manipulation of the angpt / tie-2 system will become a rational therapeutic target for the management of sepsis .
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plx4032 was synthesized using the general procedures previously described.6 expression and purification of b - raf , structure determination , protein kinase activity measurements , and xenograft studies were carried out as previously described.6 clinical methods have also been recently described.5 melanoma patients were selected for study using previously described taqman methodology.8 semi - quantitative immunohistochemistry for perk and ki67 was performed on 5 m - thick formalin - fixed paraffin - embedded tumor biopsies following h&e staining to determine pathologic diagnosis and tissue morphology and integrity . the degree of phospho - erk staining in the nucleus and cytoplasm was interpreted semiquantitatively by assessing the intensity and extent of staining on the slides . for ki67 staining ,
b - raf is the most frequently mutated protein kinase in human cancers.1 the finding that oncogenic mutations in braf are common in melanoma2 followed by the demonstration that these tumors are dependent on the raf / mek / erk pathway3 offered hope that inhibition of b - raf kinase activity could benefit melanoma patients . herein , we describe the structure - guided discovery of plx4032 ( rg7204 ) , a potent inhibitor of oncogenic b - raf kinase activity . preclinical experiments demonstrated that plx4032 selectively blocked the raf / mek / erk pathway in braf mutant cells and caused regression of braf mutant xenografts.4 toxicology studies confirmed a wide safety margin consistent with the high degree of selectivity , enabling phase 1 clinical trials using a crystalline formulation of plx4032.5 in a subset of melanoma patients , pathway inhibition was monitored in paired biopsy specimens collected before treatment initiation and following two weeks of treatment . this analysis revealed substantial inhibition of erk phosphorylation , yet clinical evaluation did not show tumor regressions . at higher drug exposures afforded by a new amorphous drug formulation,4,5 greater than 80% inhibition of erk phosphorylation in the tumors of patients correlated with clinical response . indeed , the phase 1 clinical data revealed a remarkably high 81% response rate in metastatic melanoma patients treated at an oral dose of 960 mg twice daily.5 these data demonstrate that braf - mutant melanomas are highly dependent on b - raf kinase activity .
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pentalogy of cantrell is a rare multiple congenital malformation which occurs worldwide with a reported incidence of 5.5 per million live births.1 the exact cause is not known but is mainly thought to be sporadic , though its being associated with some chromosomal disorders like trisomy 1823 and deletion on locus at xq25 - 26 has been described in some cases.4 it was first described by cantrell et al . , in 19585 with the pentad consisting of a giant omphalocoele and a supra - umbilical anterior abdominal wall midline defect , sternal cleft , ectopia cordis , intracardiac congenital malformations like ventricular septal defect , atrial septal defect and tetralogy of fallot , ventral midline diaphragmatic defect with defect of the diaphragmatic pericardim.5 other associated defects include cranial and facial anomalies , clubfeet , malrotation of the colon , hydrocephalus and anencephaly.67 pentalogy of cantrell often have a poor outcome which is dependent on the severity of the malformations.8 though some cases of pentalogy of cantrell had been reported in nigeria , its true prevalence is not known and none of those reported had been proven to completely fulfill the five main components of the pentad.91011 we , therefore , report a case of pentalogy of cantrell in a 9-month - old boy who completely fulfilled the diagnostic criteria . a 9-month - old boy was first seen at the age of 5 months being delivered with multiple congenital malformations involving the anterior abdominal and chest walls . the child was delivered at term gestation and at home ; the pregnancy was not adversely eventful and she had no antenatal care . however , the child has defect on the anterior abdominal wall with a huge swelling extending from the lower anterior abdomen to the lower anterior chest wall , which was pulsatile and covered by a thin membrane which was initially reddish at birth but later became thickened and darker following daily cleaning and dressing . there was no history of vomiting or constipation , child sucked directly from the breast , though interrupted occasionally to catch his breath , there was no history of difficulty with breathing and no darkening of the lips or mucous membrane . the mother was a 25-year - old stay at home mother with four other children in a non - consanguineous marriage ; she neither smoked cigarette nor drank alcohol and had no adverse medical record . , there was a big pendulous and pulsatile mass extending from slightly above the symphysis pubis to the epigastrum measuring 12 10 cm [ figure 1 ] with an epithelised membranous covering ; it had visible peristaltic waves , it was pundunculated , with cardiac pulsation felt in the epigastric region [ figure 2 ] . bowel and heart sounds were heard over the mass below the level of the diaphragm . he was not tachypneic or tachycardic and had first and second heart sounds with a systolic murmur . his chest x - ray showed a midline heart with inferior displacement [ figure 3 ] , abdominal ultrasound showed the swelling to contain loops of bowl and echocardiography revealed a 6 mm secundum atrial septal defect . the child is currently being followed up awaiting staged repairs of the defects . a bulge in the region of the epigastrium and lower sternum x - ray showing loops of bowl in the swelling with displacement of cardiac shadow pentalogy of cantrell is commoner in boys and less than 60 cases have been documented so far worldwide as at 2007.8 the exact mechanism is not completely understood , however , the proposed pathogenesis involves a defect in embryogenesis between 14 and 18 days after conception due to failure of the in folding of the lateral mesoderm . while most affected children die shortly after birth due to the severity of their defect ( especially the associated intra - cardiac defect and risk of infection in open defects ) , it has been reported in a 32-year - old - man.12 our patient was 9-month - old and remained active despite the associated atrial septal defect and has better prospect with adequately staged surgical intervention . our case had all the major features of the pentad , though with a relatively favourable lesion because the membrane of the giant omphalocoele was completely epithelised [ figure 4 ] and the peduncle covered with skin , which helped in preventing infection ; furthermore , the atrial septal defect was of moderate size and the child had remained stable , though he stands the risk of trauma to the barely covered heart , but with proper counselling the child has survived up till this age . the mass covered by thickened , hyperpigmented keratinised membrane pentalogy of cantrell can be diagnosed prenatally but that has not been the case in those reported from nigeria so far because lack of accessibility to a proper antenatal healthcare and for the fact that most of those diagnostic tools are not readily accessible to these low income parents . patients with complete expression of pentalogy of cantrell are rare and few may survive to older age depending on the severity of the associated lesions , more especially the intracardiac defect . furthermore , we advised that patients with giant omphalocoele should be thoroughly evaluated , because they may have an incomplete expression of the syndrome .
pentalogy of cantrell is a rare congenital malformation whose exact cause is not completely understood ; it is characterized by defects in the anterior abdominal and thoracic walls consisting of omphalocoele , diaphragmatic defect , ectopia cordis , intracardiac defects and sternal clefts . the complex has variable clinical expression with complete and incomplete expressions reported . we , therefore , report a case of complete manifestation of the pentad in a 9-month - old boy .
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we report involvement of unusual site in ilio - acetabular region with breach in articular cartilage . intra - operatively breach in acetabular roof was seen which was missed in the scan . this case report emphasizes about the rarity of the lesion in this location and the rare chances of breach in articular cartilage of the joint . bone desmoid tumor was an unknown entity until when jaffe hl reported a case in 1958 . it is a locally aggressive benign tumor reported commonly in mandible and meta - diaphyseal region of long bones . we report a case of desmoid tumor of ilio - acetabular region in a 40 year old female . a 40 year old female came with complaint of pain in the left hip region which aggravated on squatting and climbing stairs . radiological evaluation showed a lytic lesion in the left ilio - acetabular region , surrounded by a sclerotic margin superiorly . mri scan was done which showed a well defined homogenous lytic lesion with no break in the cortex and no soft tissue involvement ( fig . plain radiograph of pelvis with both the hips which shows a well defined lytic lesion over the left ilio - acetabular region . there appears no obvious breach in the cortex or the articular margin a ct scan of the pelvis was taken to rule out any breach in the cortex . there appears no obvious discontinuity through an ilio - inguinal incision the lesion was approached through inner table of ilium . a defect on the roof of the acetabulum was found which was missed by the scan ( fig . thorough curettage was done and the roof defect was reconstructed with a cortical graft harvested from the inner table of the ilium . there appears a hollow curetted lesion within which a breach in the roof of the acetabulum is seen histopathological finding showed multiple spindle shaped fibroblast cells with small and elongated nuclei in the background of dense collagen fibres . histopathology specimen picture showing spindle shaped fibroblast cells with small and elongated nuclei over a background of collagen fibers . there appears no nuclear atypia or mitotic activity patient was kept non weight bearing for 3 months with gradual return to full weight bearing walking . at follow up of 20 months patient is symptom free and xray showing no evidence of lytic lesion ( fig 5 ) . however , on ct scan there appears a persistent lytic area which we suspect to be a recurrence ( fig 6 ) . hence patient is kept on regular follow - up . a 20 month post operative x ray picture showing well maintained articular margin and the joint space . the lytic lesion seems filled up ct scan of the hip taken at 20 month post op , however shows lytic lesion anteriorly which seems to be a recurrence few cases have been reported since then and common occurrence being in mandible and meta - diaphyseal region of the long bone . recognition of desmoplastic fibroma is important because on radiology and histology , the lesion may be mistaken for an indolent , benign fibrous lesion or more aggressive spindle - cell sarcomas . it commonly occurs in the age group between the adolescent to 40 years with no specific sex predominance . patients commonly come with complaint of pain ; however there are few reported cases of pathological fracture especially those involving tibia and femur . the lesions are well defined with no sclerosis or periosteal reaction except if there is associated pathological fracture . if a soft tissue lesion is invading onto the bone , they are eccentric in position with sclerosis due to endosteal bone formation . significant t2 shortening of a non - sclerotic fibro - osseous lesion should place desmoplastic fibroma high among the diagnostic considerations . radiologically they have to be differentiated from giant cell tumor , fibrous dysplasia ( long bone lesions ) , fibrosarcoma , brown tumors , and chondromyxoid fibroma and hence a tissue diagnoses is must . the differentiation from a low grade fibrosarcoma is difficult though . as these are rare tumors , there is no standard protocol for treatment of these lesions . all kinds of procedures like intralesional , marginal or wide resection are reported as a treatment . but if wide resection would result a major functional deficit , an attempt of intralesional curettage with bone grafting seems warranted . desmoid fibroma of ilio - acetabular region is very rare hence a great sense of suspicion is required for the appropriate management . this case report emphasizes about the rarity of the lesion in this location and the chances of breach in articular cartilage of the joint . desmoid tumor , though rare , should be kept as one of the important differentials for the lytic lesion around pelvis . one should also define whether there is any associated cortical or articular margin breach ( as seen in our case report ) and then decide further management regarding reconstruction .
introduction : desmoid tumor of bone is a rare benign tumor . it is reported commonly in mandibular and meta - diaphyseal region of long bones . we report involvement of unusual site in ilio - acetabular region with breach in articular cartilage.case report : a 40 year old female presented with pain in the left hip . radiologically , a lytic lesion at ilio - acetabular region was seen . intra - operatively breach in acetabular roof was seen which was missed in the scan . curettage and defect reconstruction was done . histopathology reported as desmoid tumor . 20 months post - operatively patient was symptom free.conclusion:desmoid tumor is a rare bone tumor . this case report emphasizes about the rarity of the lesion in this location and the rare chances of breach in articular cartilage of the joint . the chances of recurrences are high with intralesional curettage .
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a 32-year male patient presented to the ed with severe dyspnea and agitation at 2:00 a.m. the patient was placed in orthopnea position and oxygen was administered . blood pressure was 165/50 mmhg , heart rate was 113 bpm , body temperature was 36.5 c , 25 breaths of respiratory rate per minute and oxygen saturation with pulse oximetry was 78% with nasal cannula . physical examination revealed rales as far as upper zones of both of lungs and at base of heart , and a diastolic grade 2/6 murmur of aortic regurgitation was heard . medical history included hypertension treated with angiotensin receptor blocker ( arb ) and smoking habit . electrocardiography ( ecg ) showed sinus tachycardia with t - wave inversion in leads di and vl . the working diagnosis was pulmonary edema , and use of bronchodilator inhaler was initiated , as well as intravenous nitrates and intravenous diuretic therapy . while taking arterial blood gas sample , patient s condition deteriorated and abdominal respiration pattern became obvious . elective tracheal intubation of patient was performed , and patient was admitted to coronary intensive care unit ( cicu ) with pulmonary edema and possible acute valvular insufficiency . transthoracic echocardiogram ( tte ) showed mild to moderate aortic regurgitation with 3.3 cm of sinus of valsalva . systolic function of ventricle was normal and left ventricular ( lv ) cavity widened to 5.1 cm at end - diastolic volume . arterial blood gas before intubation showed hypoxia with 76% arterial oxygen saturation and hypocarbia with metabolic acidosis of 7.13 ph . contrast - enhanced computed tomography ( cect ) of thorax was used to refine the diagnosis . as a team , the cardiologist , the cardiovascular surgeon , and the radiologist checked the computed tomography ( ct ) images . sinus of valsalva was 3.5 cm and no flap was found in ascending or descending aortas . as lab test results showed coronary ischemia with positive troponin i levels ( 1.016 ng / dl ) , it was decided that a diagnostic coronary angiography ( cag ) would be performed . in the catheterization laboratory , intubation of right coronary artery ( rca ) and left main coronary artery ( lmca ) was difficult , and the test required 1 hour to complete . pulmonary embolism and coronary artery disease as cause of acute dyspnea were ruled out , but the cause of dissection of aorta was still unclear because of possibility of a false negative ct scan . transesophageal echocardiography ( tee ) was selected as the next diagnostic tool due to high pretest probability of aortic dissection . tee showed a stanford type a dissection flap closing lmca ostia from beat to beat and compromising the aortic valve with moderate aortic regurgitation . determining the appropriate diagnosis for acute heart failure was very challenging , but at 6.00 a.m. , surgery to correct a stanford type a dissection localized in the valve and coronary ostia was performed . upon further careful review of ct scan images , a very tiny flap was observed at the ostium of lmca ( figure 1 ) . transverse cut view of contrast enhenced enhanced computed tomography shows a tiny flap at the ostium of lmca that was initially misdiagnosed . acute aortic dissection is a life - threatening medical emergency that can quickly lead to death . incidence is estimated to be 3 in a 1000 cases according to international registry of aortic dissection ( irad ) . if left untreated , 33% of individuals will die within 24 hours of presentation , and 50% die in the initial 48 hours . although diagnostic tests have improved , the condition remains undiagnosed in about half of patients because of variable symptoms and negative laboratory tests . after first admission tests of physical examination , vital signs , and electrocardiogram ( ecg ) ; the most frequently performed tests to diagnose aortic dissection are ct , tee and magnetic resonance imaging ( mri ) . a recent meta - analysis by shiga et al . reviewed published studies of diagnosis of aortic dissection by tee , helical ct and mri showed that these tests have equal and reliable diagnostic value . tee had 99% sensitivity and 95% specificity , helical ct had 100% sensitivity and 98% specificity , and mri had 98% sensitivity and 98% specificity [ 4 , 5 ] . in the present case , first admission physical examination and laboratory tests were supportive of aortic dissection . after a misdiagnosis was made by the radiologist , the cardiologist , and the surgeon based on ct of chest , cag was performed to clarify coronary ischemia , which is contraindicated in aortic dissection . correct diagnosis of aortic dissection was made with tee , and the patient was taken to surgery . further examination of ct scan images then revealed a tiny flap at the ostium of lmca ( figure 1 ) . aortic dissection may occur in a small part of aorta and not be seen in ct scan or laboratory test results . if suspicion of aortic dissection is high , diagnosis can be made with tte and tee in intensive care units ( icus ) . dyspnea is the major symptom of acute heart failure and an etiologic assessment must be made for every patient who presents with acute dyspnea . in the present case , aortic dissection compromised aortic valve and coronary ostium , increasing left ventricle end - diastolic pressure , which led to pulmonary edema and acute dyspnea . in episodes of acute dyspnea though a diagnostic tool like ct scan may have 100% sensitivity and 98% specificity , it may also contribute to a misdiagnosed aortic dissection . if high pretest probability is present and ct scan does not support the diagnosis , tee and mri may be used next to determine the correct diagnosis .
acute dyspnea is a major complaint of patients admitted to cardiology and emergency departments ( ed ) . acute dyspnea can be life - threatening , and is seen in cases of asthma , pulmonary embolism , acute heart failure and myocardial infarction . the present case is that of a 32-year - old man admitted to the ed with orthopnea position and agitation . physical examination , electrocardiogram ( ecg ) , transthoracic echocardiogram ( tte ) , contrast - enhanced computed tomography ( cect ) of thorax and coronary angiography ( cag ) helped to rule out chest disease pathologies such as pneuomo - thorax , pulmonary embolism and coronary artery disease , but were not enough to make an appropriate diagnosis in this case . because of high pretest probability of aortic dissection , transesophageal echocardiography ( tee ) was performed and a diagnosis of stanford type a dissection closing left main coronary artery ( lmca ) ostia from beat to beat was made .
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renal cell carcinoma ( rcc ) may metastasize to any site of the body , but clinically evident metastatic intestinal involvement by rcc is extremely rare . to our knowledge , simultaneous duodenal and colonic metastases have not been reported in the english literature . we report a case of pathologically proven simultaneous duodenal and ascending colonic metastases about four years after a left nephrectomy for rcc . a 76-year - old female patient who had undergone a left radical nephrectomy 4 years previously for rcc ( mixed clear and granular cell type , tnm stage iii ) presented with a 1-month history of dyspepsia , lethargy and pain in the right upper abdomen . her blood pressure was 110/70 mmhg , pulse rate 80/min , respiration rate 22/min and body temperature 36.8c . abdominal examination revealed slight tenderness but normal peristalsis ; however , a movable mass was palpated in the right upper abdomen . the hematocrit was 28.1% and the white cell count was 9700/mm with 77% polymorphonuclear cells and 14% lymphocytes . serum sodium was 134 meq / l , potassium 3.5 meq / l , chloride 101 meq / l and calcium 9.5 mg / l . the result of liver function tests were as follows : total protein 6.5 g / dl , albumin 3.5 g / dl , cholesterol 165 mg / l , bilirubin 0.6 mg / dl , alkaline phosphatase 105 iu / l , ast 28 iu / l and alt 21 iu / l . tumor marker levels , such as carcinoembryonic antigen ( cea ) , carbohydrate antigen 19 - 9 ( ca19 - 9 ) and -fetoprotein ( afp ) , were within normal limits . an abdominopelvic ct scan showed circumferential wall thickening with high enhancement at the second portion of the duodenum and additional enhancement of an irregular protruding mass into the lumen of the ascending colon with multiple lymphadenopathy in the aortocaval area ( figure 1 ) . a gastroscopy showed a large , irregular multi - lobed , partially necrotic and ulcerative protruding mass nearly obstructing the second portion of the duodenum . a colonoscopy revealed a polypoid , nodular mass in the ascending colon , which was purplish in color , had sharp margins and was pliable ( figure 2 ) . microscopic findings of biopsy specimens showed features identical to those of the renal cell carcinoma which was resected 4 years earlier in this patient and immunohistochemical stainings for vimentin was positive ( figure 3 ) . interferon was given at a dose of 2 million iu per square meter three times a week . however , interferon therapy was discontinued after three months due to anemia , anorexia and general weakness . the clinical course of the disease ranges from months to several decades and even spontaneous regression has been documented . approximately 25% of patients with rcc have metastatic disease at the time of diagnosis . the delayed occurrence ( as late as 31 years after a nephrectomy ) of metastatic rcc is well known . solitary metachronous metastases from rcc are rare ; however , they can occur very late in the course of the disease . renal cell carcinoma may metastasize to almost every organ of the body , but 95% of the metastatic lesions involve the lung , lymph nodes , liver , bone , adrenal glands and the opposite kidney . however , there are scattered reports in the literature of clinically evident intestinal metastasis from rcc . to the best of our knowledge , a secondary tumor involving the intestinal tract may be caused by direct extension , lymphatic spread , peritoneal or hematogenous dissemination . in the present case , duodenal and colonic metastases are not generalized carcinomatosis because any other metastasis is not occurred in common metastatic sites and duodenal and ascending colonic involvement are remote from a primary cancer of the left kidney . hypervascularity of the present tumor , as suggested by a ct scan , gastroscopy and colonoscopy , was compatible with the characteristics of metastatic renal cell carcinoma . also , positive immuno - histochemical stain for vimentin elucidated that the duodenal and colonic masses are compatible with carcinoma rather than adenocarcinoma . surgical excision of the local recurrence is the best procedure for therapy , but this can be radical only when the recurrence can be completely excised . chemotherapies , including hormonal and interferon therapies , are effective in some patients with metastatic renal cell carcinoma . in our case although interferon treatment was used to treat metastatic rcc , it is not easy to evaluate its effectiveness because of premature discontinuance of the treatment . in our opinion , endoscopists should consider the possibility of intestinal metastasis of rcc when endoscopic and ct studies show a hypervascular mass in a patient with a previous history of rcc .
we report a case of pathologically proven simultaneous duodenal and colonic metastases about four years after nephrectomy for mixed clear and granular cell type renal cell carcinoma ( rcc ) . a 76-year - old female patient who had undergone a left radical nephrectomy 4 years previously for rcc presented with a 1-month history of dyspepsia and pain in the right upper abdomen . an abdominopelvic ct scan showed circumferential wall thickening with high enhancement at the second portion of the duodenum and additional enhancement of an irregular protruding mass into the lumen of the ascending colon . a gastroscopy showed a large and ulcerative protruding mass nearly obstructing the second portion of the duodenum . a colonoscopy revealed a polypoid , nodular and purplish mass in the ascending colon . microscopy of the biopsy specimen showed the features identical to those of the rcc which was resected 4 years earlier in this patient . we believe this to be the first case illustrating a metastatic renal cell carcinoma as simultaneous duodenal and colon masses .
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few similar cases are reported in literature but none of them is associated with mirizzi syndrome . radiological images could help the surgeon to choose the surgical strategy and to evaluate the presence of associated diseases . in our case laparoscopic surgery showed to be an excellent approach for both the cholecystectomy and the repair of the defect of the abdominal wall . the patient was an 85 year - old man with history of hypertension , previous surgery for perforated diverticular disease ( hartmann procedure followed by reversal of colostomy ) . the physical examination showed a 5 cm mass in the right upper quadrant tender to palpation . ultrasound and ct confirmed the presence of a lithiasic gallbladder herniated through the abdominal wall ( fig.1 ) , with a dilatation of the common bile duct that measured 11 mm , due to a 17 mm obstructive infundibular stone as it happens in mirizzi syndrome type i ( fig.2 ) . lithiasic gallbladder herniated through the abdominal wall the routine complete blood test , including bilirubin level , was unremarkable . therefore the patient underwent a laparoscopic cholecystectomy and a repair of the hernia . during surgery , multiple adhesions were found and released carefully to identify the gallbladder that was fully included in the right upper quadrant abdominal wall covered by peritoneum . the gallbladder was completely released and the dissection of the pedicle showed a short cystic duct and a normal common bile duct . most of the cases reported in literature are internal hernias through the winslow foramen ( 1 ) . the herniation of the gallbladder through acquired defects of the abdominal wall ( incisional hernia ) has been much less reported . ( 2 ) presented the case of a gallbladder herniated through a fascial defect of a subcostal incision . garcia reported a patient who presented a gallbladder hernia through a parastomal defect ( 3 ) . more recently , the case of a gallbladder strangulation through an abdominal wall defect on the site of a previous colostomy was described ( 4 ) . in this case , the most remarkable thing is that herniation does not occur through a natural orifice or an acquired defect , such as respectively for the winslow foramen or for an incisional hernia , but directly into the abdominal wall . to our knowledge , there are only 3 published cases of spontaneous herniation of the gallbladder through the abdominal wall ( 5,6,7 ) . another interesting aspect of this case is the presence of a chronically distended gallbladder ( gallbladder hydrops ) , associated with extrinsic compression of the common hepatic duct by an impacted stone in the infundibulum ( mirizzi syndrome type i ) ( 8) . it is probably the gallbladder dilatation that plays an important role in the development of the hernia pressing constantly the gallbladder against the abdominal wall . usually the clinical picture is represented by a right upper quadrant pain , associated with variable degrees of a compromised general condition . in our experience the preoperative study based on computed tomography is essential for the diagnosis of the gallbladder hernia , and it provides also additional information on the abdominal wall defect . the management of this type of hernia consists in reducing the content and repairing the abdominal wall defect . in this case we suggest the laparoscopy as surgical approach of first choice because it allows to solve three problems at the same time : first , the reduction of the gallbladder s incarcerated hernia ; second , it enables to perform a cholecystectomy that was indicated for the presence of gallstones and hydrops ; third , it allows to repair the hernia defect preferably with a mesh , if the local conditions are favorable . in our experience , preoperative imaging has been proved to detect the gallbladder disease and the morphology of the fascial defect . mesh repair is the gold standard for hernias ; however , acute cholecystitis still remains a contraindication for mesh repair due to the high risk of infection .
a gallbladder incarcerated hernia associated with mirizzi syndrome is a very rare entity and to our knowledge this is the first case ever described in literature . an 85-year - old man presented at the emergency department with a tender right upper quadrant mass . computed tomography ( ct ) revealed the presence of a gallbladder lithiasis with signs of acute cholecystitis , herniated through the abdominal wall with an associated mirizzi syndrome . laparoscopic cholecystectomy and repair of the abdominal wall defect were performed . the patient recovered very well and the postoperative period was uneventful .
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congenital coarctation of the aorta is a narrowing of the descending aorta which typically is located at the ligamentum arteriosum just distal to the left subclavian artery . this condition may be undiagnosed until adult life , when the clinical presentation most often is high blood pressure ( bp ) in both or more seldom in only one of the upper extremities . other typical clinical manifestations may include headache , fatigue on exertion , and bilateral lower limb claudication . coarctation of the aorta occurs in 5 - 8% of cases of congenital heart defects . this condition may occur along with ventricular septal defect and other related heart defects , or may occur isolated . in rare cases , severe trauma and injury may lead to coarctation of the aorta . in extremely rare cases , severe atherosclerosis or inflammatory diseases of the aorta may cause narrowing of the artery leading to aortic coarctation . a 57-year - old patient was referred to our outpatient clinic by his primary care physician because the 12-lead ecg demonstrated left ventricular ( lv ) hypertrophy . twenty - two years earlier , the patient had been referred for cardiological examination due to a cardiac systolic murmur . at that time , his bp was 98/50 mmhg , and simultaneous and equal radial and femoral pulses were described . no medical or cardiovascular history or cardiovascular risk factors were present , and the patient had no signs of genetic disorders . at the present consultation , the patient confirmed the absence of any cardiovascular symptoms . transthoracic echocardiography showed a non - dilated , hypertrophic left ventricle [ figure 1a and b ] with end - diastolic interventricular septal thickness of 21 mm , end - diastolic lv posterior wall thickness of 12 mm , and an estimated lv mass of 449 g ( lv mass index 214 g / m ) . the lv ejection fraction was 50% . except for a mild aortic regurgitation ( in a normally shaped tricuspid aortic valve ) and a dilatation of the ascending aorta of 40 mm a continuous wave doppler examination from the suprasternal notch showed a peak systolic pressure gradient in the thoracic descending aorta of 80 mmhg without diastolic run - off [ figure 1c and d ] , indicating a severe obstruction at the classical site of a coarctation . multislice computed tomographic ( ct ) angiography confirmed the finding of severe coarctation of the aorta . the ct scan demonstrated that both subclavian arteries originated distal to the severe coarctation , explaining the normal bp in both arms [ figure 2 ] . moreover , a ct scan of the cerebrum revealed the vessels in the circle of willis giving rise to numerous collaterals in the brain circulation . 57-year - old male was referred to our outpatient clinic because the 12-lead ecg demonstrated left ventricular ( lv ) hypertrophy that was later diagnosed as due to congenital coarctation of the aorta . transthoracic echocardiography ( a ) apical four - chamber view and ( b ) m - mode show left ventricle hypertrophy ( arrows ) ; ( c and d ) suprasternal views show the narrowing in the thoracic descending aorta ( arrow ) and the continuous wave doppler curve without diastolic run - off ( arrow ) . 57-year - old male was referred to our outpatient clinic because the 12-lead ecg demonstrated left ventricular ( lv ) hypertrophy that was later diagnosed as due to congenital coarctation of the aorta . we report an uncommon case of congenital coarctation in a 57-year - old man without the clinical signs of coarctation . because of the uncommon location of the aortic narrowing with both the right and left subclavian arteries originating distal to the area of coarctation , the bp was equally low in both upper extremities . the present case shows that a normal brachial bp does not rule out severe coarctation and should be considered in apparently normotensive patients presenting with a systolic murmur or target organ damage , in this case severe lv hypertrophy . uncorrected coarctation of the aorta in adults predisposes to congestive heart failure , aortic dissection and rupture , stroke , cerebral hemorrhage , and infective endocarditis . therefore , an early diagnosis is important , and the present case emphasizes the use of suprasternal view as a part of a standard diagnostic echocardiography . treatment options include surgical repair or balloon angioplasty with or without stent implantation . taking the atypical location , extent , and complexity of the lesion into account ,
the present case shows that a normal brachial blood pressure ( bp ) does not exclude severe coarctation and should be considered in normotensive patients presenting with a systolic murmur and/or unexplained severe left ventricular hypertrophy . congenital coarctation of the aorta is a narrowing of the descending aorta , usually located distal to the origin of the subclavian artery , causing hypertension in the upper part of the body . this condition may be undiagnosed until adult life where the clinical presentation most often is high bp in the upper extremities . a 57-year - old patient with severe aortic coarctation and left ventricular hypertrophy presented with normal brachial bp . however , standard suprasternal view by echocardiography indicated coarctation . multislice computed tomographic ( ct ) angiography revealed an uncommon location of the aortic narrowing with the right and left subclavian arteries originating below the area of coarctation , explaining the equally low bp in both upper extremities .
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multistep skin carcinogenesis assays were performed with mice with keratinocyte - specific deletion of the cnb1 gene ( cnb1xk5-crepr1)3 in parallel with cre - negative controls ( cnb1 ) . detailed conditions for these assays as well as chromatin immunoprecipitation , immunoblotting , immunofluorescence , senescence -galactosidase staining , biotinylated dna pull down assays , and sorting can be found in the method section and supplementary figure legends .
calcineurin inhibitors such as cyclosporin a ( csa ) are the mainstay of immunosuppressive treatment for organ transplant recipients . squamous cell carcinoma ( scc ) of the skin is a major complication of treatment with these drugs , with a 65100 fold higher risk than in the normal population1 . by contrast , the incidence of basal cell carcinoma ( bcc ) , the other major keratinocyte - derived tumour of the skin , of melanoma and of internal malignancies increases to a significantly lesser extent 1 . here we report that genetic and pharmacological suppression of calcineurin / nfat function promotes tumour formation in mouse skin and in xenografts , in immune compromised mice , of h - rasv12 expressing primary human keratinocytes or keratinocyte - derived scc cells . calcineurin / nfat inhibition counteracts p53-dependent cancer cell senescence thereby increasing tumourigenic potential . atf3 , a member of the enlarged ap-1 family , is selectively induced by calcineurin / nfat inhibition , both under experimental conditions and in clinically occurring tumours , and increased atf3 expression accounts for suppression of p53-dependent senescence and enhanced tumourigenic potential . thus , intact calcineurin / nfat signalling is critically required for p53 and senescence - associated mechanisms that protect against skin squamous cancer development .
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in rare conditions , bacteria from carbuncles can spread into the bloodstream and migrate to other areas of the body , causing serious complications such as septicemia and infections in the liver , bones , joints , heart , and central nervous system . we describe an original case of forehead carbuncle with intractable headache , later confirmed as a subgaleal abscess . a 74-year - old female visited our hospital due to a 2 cm , painful , erythematous nodule with a small necrotic plug on her forehead , which had been resident for 2 days . she had suffered for 5 years from diabetes mellitus , which was not aggressively controlled . one day later , the patient visited our emergency room again due to progressive erythema and worsening headache . she had mild fever ( 37.7c ) and bilateral periorbital soft tissue swelling with local heat . laboratory data showed elevated white blood cell count ( 10.521,000 cells/l ) and c - reactive protein levels ( 78.90 preliminary diagnosis suspected cellulitis ; she was admitted to kaohsiung municipal ta - tung hospital , kaohsiung , for further management . however , her headache kept worsening and interfered with her sleep over the next 2 days . she described the headache as severe , persistent aching all over her head , which was not associated with postural change . nonsteroidal anti - inflammatory drugs and tramadol hydrochloride were administered but completely in vain . in the fear of central nervous system involvement however , brain computed tomography ( ct ) was still arranged due to intractable headache . brain ct showed no intracranial lesion , but subgaleal emphysema and abscess were suspected ( figure 1 ) . a neurosurgeon was then consulted , and operative debridement was promptly arranged . during the operation , widespread subgaleal abscess was noted and drained . as previously described in the case history section , this 74-year - old female had intractable headache , which developed in close relationship with subgaleal abscess . in addition , her headache resolved within 3 months after successful treatment of subgaleal abscess with operative debridement and antibiotics . according to the international classification of headache disorders ( ichd-2 ) criteria , headache disorders attributed to extracranial infection of the head ( such as ear , eye , and sinus infection ) are coded as subtypes 11 . headache or facial pain can be attributed to a disorder of the cranium , neck , eyes , ears , nose , sinuses , teeth , mouth , or other facial or cranial structures.1 carbuncle is a common dermatologic disease , and staphylococcus aureus is the pathogen responsible in most cases . though some authors advocate that systemic antimicrobial treatment is not needed for simple furuncles and carbuncles,2 we agree that incision and drainage with ancillary antimicrobial therapy is recommended for patients with immunosuppression or comorbidities , extremes of age ; rapid progression to cellulitis and the lack of an adequate response to incision and drainage are also indications for such treatment.3 however , empiric antibiotics for carbuncle , even vancomycin , did not work for the unusual bacterial culture of klebsiella in this patient . although klebsiella is strongly associated with infections in patients with diabetes , skin and soft tissue infections from klebsiella are still uncommon.4 this case reminds us that empiric , systemic antibiotics for carbuncles and cellulitis may respond poorly in situations as described in the case history section . the most common cause of subgaleal abscess is direct inoculation of microbes into the subgaleal space following scalp trauma . however , subgaleal abscess may result from hematogenous infection or contiguous spread , and the diagnosis may not be initially obvious.5 carbuncles may progress to cellulitis and cause redness of the skin , swelling , and pain . however , carbuncles located on the middle of the face may raise concern , especially when the patient is elderly and immune - compromised . intractable headache that is unresponsive to the standard medications and therapies utilized in the treatment of headaches also indicates the need for further survey . head ct is often needed in the diagnosis of subgaleal abscess , and if operative debridement is not promptly performed , subgaleal abscess may further progress to life - threatening septicemia , osteomyelitis , and even subdural or brain abscess or meningitis.6 there are no focal neurological signs or specific symptoms in the early stages of subgaleal abscess . thus , when encountering patients with intractable headaches , unusual causes should be kept in mind , which may include moyamoya syndrome,7 headache after botulinum a exotoxin injections,8 acquired immunodeficiency syndrome - related lymphoma confined to bone,9 migraine in obese individuals,10,11 and children with both migraine and periodic limb movement disorders in sleep.12 in conclusion , this case highlights that special attention should be paid to elderly and immune - compromised patients with carbuncles located on the middle of the face , especially when accompanied by intractable headache .
although carbuncles are commonly seen and may heal on their own or respond well to treatment , in rare conditions , bacteria from carbuncles can spread into the bloodstream and migrate to other areas of the body . herein , we report on an elderly female who suffered from forehead carbuncle with intractable headache , later confirmed as having subgaleal abscess . physicians should pay special attention to elderly and immune - compromised patients with carbuncles located on the middle of the face , especially when accompanied by intractable headache , to avoid poor outcome .
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initially , they were considered as metastasis from an occult primary tumor in gonads , but now it is known that they arise either as a consequence of abnormal migration of germ cells during embryogenesis or from a different histogenetic origin . choriocarcinoma of liver without a detectable primary tumor in gonads , retroperitoneum or mediastinum is termed as primary hepatic choriocarcinoma . due to varied clinical presentations and extreme rarity of the tumor , diagnosis on small needle biopsy is difficult . we present a case of adult male diagnosed to have primary hepatic choriocarcinoma on histopathology and immunohistochemical studies . a 40-year - old male was referred to us with history of sudden onset pain in abdomen . he was admitted in private hospital where computed tomography ( ct ) scan of abdomen was done which was suggestive of ruptured haemangioma in left lobe of liver ( figure 1 ) . patient was resuscitated and given adequate blood transfusions and was referred to our centre . on admission abdominal exploration revealed about 1.5 to 2 l of haemoperitoneum and multiple nodular lesions in both lobes of liver with large ruptured lesions in left lobe of liver with active bleeding . after achieving adequate inflow and outflow control , left lateral segmentectomy was done in view of active bleeding ( figure 2 ) . histopathology showed atypical trophoblastic cells predominantly cytotrophoblast and few syncitiotrophoblast lying in sheets as well as in clusters with large number of mitotic figures along with the normal hepatocytes suggestive of high grade malignant tumor ( figure 3 ) . immunohistochemistry was positive for beta human chorionic gonadotrophin ( figure 4 ) and negative for ck , ema , cd-30 , afp , cd-31 and cd-34 suggestive of choriocarcinoma . his serum hcg levels were significantly raised however serum levels of afp , cea and ca 19 - 9 were normal . patient died on postoperative day 10 due to sudden cardiopulmonary arrest . on autopsy , multiple nodular lesions were present on the remaining liver . bilateral testes were grossly normal which on subsequent serial sectioning and histological examination did not show any evidence of pathologic features associated with germ cell tumor regression or a scar . based on histopathological report and autopsy findings figure 1computed tomography scan of abdomen showing ruptured haemangioma in left lobe of liver with haemoperitoneum . computed tomography scan of abdomen showing ruptured haemangioma in left lobe of liver with haemoperitoneum . figure 3atypical trophoblastic cells predominantly cytotrophoblast and few syncitiotrophoblast lying in sheets as well as in clusters along with the normal hepatocytes . atypical trophoblastic cells predominantly cytotrophoblast and few syncitiotrophoblast lying in sheets as well as in clusters along with the normal hepatocytes . majority of them are infantile type , may represent metastasis from an occult placental choriocarcinoma . primary hepatic choriocarcinoma in adults are known to arise from abnormal migration of germ cells during embryogenesis or different histogenetic origin . clinically , patient may present with right upper abdominal pain and/or abdominal lump or distension . those with advanced disease may present with symptoms due to metastasis to various organs like brain and lungs . infertility , gynaecomastia and features of thyrotoxicosis may present in some patients , attributed to over production of hcg by tumour cells . very rarely , it may rupture spontaneously producing haemoperitoneum and may present as an acute abdomen , as in our case . major issue in diagnosing primary hepatic choriocarcinoma is to exclude metastasis from an occult primary in gonads by serial sectioning and histological examination , as these tumors may be small or undergo spontaneous regression at the time of metastasis . diagnosis on small needle biopsy is difficult due to rarity of the tumor in liver . immunoshistochemical staining for beta hcg , plap , hpl , hepar-1 , may aid the diagnosis and helps to differentiate from the other tumours which mimic choriocarcinoma like giant cell variant of poorly differentiated hepatocellular carcinoma , or tumor with trophoblast like giant cells . finally imaging modalities like ultrasonography and ct scan may be useful to assess the extent of primary tumour and metastasis to various organs . treatment consists of complete surgical resection of tumor if localized to liver and without ascites followed by chemotherapy . for advanced or metastatic disease chemotherapy is given . are etoposide , methotrexate , actinomycin - d , cyclophosphamide . prognosis of primary hepatic choriocarcinoma is distinctly poor as compared to its testicular counterpart owing to lack of restrictive effect of tunica albugenia as in the testes ; hence they attain a large size and often invade adjacent vital structures by the time diagnosis is made . average survival being 2 to 8 months as reported in literature , hence further studies are needed for early diagnosis and better treatment to improve survival .
choricarcinoma is a beta human chorionic gonadotrophin secreting neoplasm pertinent to uterus and pregnancy mostly . it occurs primarily in gonads but rarely in extragonadal sites . primary hepatic choriocarcinoma is an extremely rare tumor . most of the reported cases are seen in infants representing metastasis from an occult placental choriocarcinoma . till date , only 7 cases of primary hepatic choriocarcinoma in adults have been reported in literature . we present a case of a 40-yearold male presenting as haemoperitoneum due to ruptured hepatic tumor . he underwent emergency left lateral segmentectomy . he died on 10th postoperative day . the surgical specimen and autopsy findings confirmed it to be primary hepatic choriocarcinoma . this is the first case report from indian subcontinent . a brief case report and review of literature is presented .
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placental polyp is a somewhat pedunculated remnant of chorionic tissue retained in the uterine cavity for an indefinite time . it may result in abnormal uterine bleeding and slightly elevated detectable titers of serum -human chorionic gonadotropin ( hcg ) . these pedunculated masses of villi are often found within days to weeks following abortion or delivery of a term placenta . since trophoblastic neoplasms especially placental site trophoblastic tumor may have similar symptoms and signs , it is important to consider placental polyp in differential diagnosis in such situations . a 34-year - old g4l3ab1 woman came with abnormal uterine bleeding since her last normal vaginal delivery 3 months ago . serum -hcg level was slightly elevated ranging from 86 to 103 iu / ml during diagnostic investigations . ultrasonography revealed enlarged uterus with an echolucent intracavitary uterine mass measuring 73 mm 55 mm 24 mm . computerized topography confirmed the presence of the mass and showed no abnormality in thorax . clinical , laboratory , and imaging findings raised the suspicion of gestational trophoblastic tumors especially those arising from intermediate trophoblastic cells . macroscopically , the uterus showed slight global enlargement resulting from the presence of a polypoid mass within the endometrial cavity . the cut surface was diffusely red with some fine streaks of a gray colored tissue . it was attached to the uterine wall in the fundal region without any macroscopic permeation into the myometrium [ figure 1 ] . microscopic study showed largely necrotic villi in a network of fibrin deposition [ figures 2 and 3 ] . a large polypoid mass with smooth outer surface has completely filled the endometrial cavity necrotic chorionic villi are seen in the background of fibrin deposition ( 40 ) nuclear debris are seen in the stroma of necrotic chorionic villi ( 400 ) placental polyp is a fragment of retained placental tissue in the uterus that has undergone neovascularization after resolution of gestation . chronic uterine inversion due to placental polyp has also been reported . a case of placental these pedunculated masses of villi are often found within days to weeks following abortion or delivery of a term placenta . rarely , they persist for months or even years after pregnancy . abnormal uterine bleeding due to placental polyp has been attributed to preserved villi , clusters of destructive villi , and isolated viable cotyledons . preservation of the brush border of syncytiotrophoblastic cells and the presence of placental phosphatase maintain the anticoagulative properties of villi . thromboplastic properties of the preserved villi play an important role in the pathogenesis of uterine bleeding when necrotic villi with epithelial remnants are prevalent . computed tomographic angiography is also useful in diagnosis and management of placental polyp with neovascularization . magnetic resonance imaging may also be used in diagnosis and follow - up of placental polyps . . a hypervascular placental polyp may lead to severe hemorrhage that requires blood transfusions , interventional radiology procedures , hysteroscopic resection , and even hysterectomy to control bleeding . evaluation of neovascularization by multimodal imaging is potentially useful in management of placental polyp in women who wish to preserve fertility . successful treatment with the use of iliac artery occlusion catheters and concomitant hysteroscopic resection has been reported . intraoperative injection of prostaglandin f2 followed by hysteroscopic resection has been successful in management of these cases . serum hcg fell to undetectable level following surgery . although the patient had completed her family and did not have any desire to preserve her fertility , a proper preoperative diagnosis with accurate interpretation of imaging findings and satisfactory curettage would have prevented hysterectomy in this patient . placental polyp should be considered in any case of parous woman with unexplained abnormal uterine bleeding and slightly elevated serum hcg level . this does not exclude the possibility of the presence of a placental polyp as the source of abnormal bleeding . all authors have contributed in designing and preparation of the first draft of the manuscript . they have read and approved the content of the manuscript and confirmed the accuracy or integrity of any part of the work .
placental polyp is retained placental tissue within the endometrial cavity , which forms a nidus for inflammation and bleeding . there are very few reported cases of the clinical placental polyp . here , we report a case of 34-year - old g4l3ab1 woman with the chief complaint of intermittent vaginal bleeding since her last normal vaginal delivery 3 months ago . serum human chorionic gonadotropin ( hcg ) titer was slightly elevated . a polypoid mass was detected within the endometrial cavity by imaging studies . history of the patient , mass lesion within the endometrial cavity and slightly elevated serum hcg titer raised the suspicion of trophoblastic neoplasms . endometrial curettage yielded unsatisfactory specimen containing only fibrin deposition and was followed by total hysterectomy . the uterus showed slight global enlargement resulting from the presence of a polypoid mass within the endometrial cavity . the red - colored mass had a smooth outer surface and fragile consistency without any permeation into the myometrium . pathology reported it as the placental polyp . although very rare , placental polyp should be kept in mind as one of the reasons of abnormal uterine bleeding in parous women . definite diagnosis is made by pathology examination .
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many organs can be herniated into the scrotum such as small intestine , appendix , colon , and ovaries . ureteral herniation is extremely rare , usually asymptomatic and is reported as isolated case report or small series . we report the case of an 88-year - old man treated for inguinoscrotal hernia where the left ureter was incidentally found in the herniated retroperitoneal fat . presenting symptoms , diagnostic evaluation , and surgical management an 88-year - old man was admitted for a left moderately sized inguinoscrotal hernia . his medical history included hypertension and benign prostatic hyperplasia . the herniated parts were dislocated from the scrotum , as well as the testicle , and cord strictures . the cord was separated from the herniated parts and was partially covered from preperitoneal fat . a large amount of retroperitoneal fat surrounded from a sac - like formation was found adjacent to the cordis [ figure 1a and b ] . these adhesions , as well as a part of this fat , were excised because of the irreducibility of the mass . during the excision , 24 h later , an abdominal ultrasound was performed to rule out any structural abnormality , which may have been missed preoperatively . ( a and b ) the left extraperitoneal inguinoscrotal hernia the ureter identified into the herniated retroperitoneal fat ureteral herniation is rare , and approximately 140 cases have been reported in the literature , mainly as isolated case reports or small series . ureteric hernia is also reported as spontaneous , postoperative or as a complication of renal transplantation . ureteral herniation presents as a groin mass usually asymptomatic although many cases describe association with dysuria , hematuria , and hydronephrosis . the hernia has the classical aspect of an indirect hernia ; it is formed by the sac anteromedially that contain viscera that make up the wall of the sac , and the ureter lying posterolaterally . the ureter slides into the canal drawn by the posterior peritoneum that follows the herniated viscera . paraperitoneal hernias are more common in men , usually located on the right side , are often large in size , usually reducible and rarely symptomatic . there is not a clear association with kidney or ureteric abnormalities . on the other hand , the hernia is often accompanied by the large amount of retroperitoneal fat , is often nonreducible , usually small and commonly associated with urinary symptoms . authors consider this type of hernia congenital and related to developmental abnormalities of differentiation of the ureter from the wolffian duct . the ureter slides along with the testis into the scrotum ; a process also favored by adhesions between the ureter and genitoinguinal ligaments . many extraperitoneal hernias have a congenital association to renal or ureteral malformation such as crossed renal ectopia or nephroptosis . however , in our case the hernia was sizable , partially reducible and was no associated with urinary symptoms . the diagnosis of ureteral hernia is often missed due to lack of urinary symptoms or signs or symptoms that could lead doctors to apply an extended preoperative work up . although computed tomography may determine the type and the contents of hernias and the intravenous urography may determine abnormalities before surgery , they are not justifiable on every inguinal hernia repair . considering the fact that ureteric injuries are serious complications and require additional surgical approaches , we emphasize the high index of suspicion needed by surgeons when repairing hernias identifying gross amount of sliding fat and resecting fat trying to reduce hernia into the abdomen .
an inguinoscrotal hernia is a common disorder that usually contains intraperitoneal organs ( small intestine , colon , appendix , ovaries ) . extraperitoneal ureteral herniation into an inguinoscrotal hernia is a rare condition and often associated with congenital abnormalities or postoperative anatomic changes . a high index of suspicion is needed in order to avoid intraoperative ureteric injuries . we herein report the case of a ureteric herniation into an inguinoscrotal hernia incidentally found during a scheduled hernia repair .
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pancreatic heterotopia is defined as the presence , outside its usual location , of pancreatic tissue which lacks anatomical and vascular continuity with the pancreas proper ( 1 ) . the heterotopic pancreas ( hp ) is a relatively uncommon congenital anomaly , with an incidence between 0.55% and 13.7% in autopsy series and mean frequency between 1 and 2% . hp has been found in all age groups , predominantly in the sixth decade of life ( 2 ) . the usual locations of hp are in the stomach in 25 - 38% cases , the duodenum in 17 - 36% and the jejunum in 15 - 22% of cases . it is usually silent but it may become clinically evident when complicated by inflammation , bleeding , obstruction or malignant transformation ( 3 ) . symptomatic patients require surgical exploration in order to obtain a definitive diagnosis and to exclude malignancy . a 12 years old male child presented with severe abdominal pain and intermittent vomiting , not relieved with medications . usg showed telescopy of gut loop along with its mesentry into other infraumblical region at the level of anterior superior iliac spine suggestive of ileoileal intesusception . an ileal polyp was found to be the cause of intussusceptions which was removed along with small segment of adjacent bowel and sent for histopathological examination . surgical specimen of resected ileal polyp with adjacent bowel loop ( 1a ) cut surface of which was pale yellow ( 1b ) . histological examination revealed presence of pancreatic tissue in muscularis propria of ileum ( 1c ) ( h&e , x40 ) with overlying mucosa showing congestion and metaplasia ( 1d ) ( h&e , x40 ) on gross examination , the polyp was brown , oval sessile mass with a broad base measuring 63.52 cm ( fig . the histological examination revealed the presence of pancreatic tissue in the muscularis propria of ileum ( fig . 1c ) made up of pancreatic acini and dialated ducts interspersed by smooth muscle bundles ( fig . photomicrograph showing pancreatic acini and ducts ( h&e , x100 ) the patient had an uneventful recovery and remains asymptomatic postoperatively . as stated by hunt and bonesteel ( 5 ) the first case of heterotopic pancreas was reported by schultz in 1729 , and klob provided its histological confirmation in 1859 ( 6 ) . the reported incidence in autopsy studies is 0.5 - 13% ( 3 ) . in adults it is found mainly in the stomach , duodenum and jejunum , in much smaller proportions in the ileum and meckel s diverticulum , and it is rarely found in the esophagus , liver , gallbladder , omentum , lungs , mediastinum , fallopian tubes and umbilicus ( 2 ) . in adults the incidence is higher in males , while in pediatric patients the female gender prevails . the proposed theory is that during rotation of foregut in a fetus and fusion of dorsal and ventral parts of pancreas , small islands of pancreas are carried away and continue to develop at its aberrant location ( 2 ) . most patients with ectopic pancreas are asymptomatic and diagnosis is usually performed during radiological examination or endoscopy of the digestive tract or during surgical explorations motivated by other diseases ( 2 ) . when symptomatic , about 30% of total mimic clinical symptoms similar to diseases that affect the organ in which the heterotopia is located ( 3 ) . usually they present in the form of small yellowish nodules , ranging from 1 mm to 5 cm , typically covered by intact mucosa , and often exhibit a central hole representing exteriorization of the rudimentary pancreatic duct . however , lesions smaller than 1.5 cm do not usually show such an orifice ( 8) . the ectopic pancreatic tissue is detected more frequently in the submucosa and muscularis propria layers of the gastrointestinal tract and may be observed in the sub - serosa or even in the serosa of the affected segment ( 2 ) . the heinrich classification system is frequently used to classify heterotopic pancreas : type 1 ( containing acini , islets and ducts ) , type 2 ( acini and ducts , no islets ) and type 3 ( ducts alone ) ( 9 ) . the preoperative imaging studies ( ultrasonography , endoscopic ultrasonography and computerized tomography ) are not very specific ( 3 ) . hence , in the majority of cases , the diagnosis is made by histological evaluation following resection of a symptomatic or suspicious lesion ( 10 ) . the management of asymptomatic , histologically verified heterotopic pancreas or those found incidentally during other surgery is under debate . although , in the majority of the cases of heterotopic pancreas reported surgical resections were done ; endoscopic mucosal removal can be an attractive , less invasive option for the resection of accessible lesions ( 3 ) . although there have been studies describing clinicopathological analysis of patients presenting with heterotopic pancreas(2,10 ) , ileal heterotopic pancreas has been rarely reported in children . heterotopic pancreas is a rare congenital lesion , often diagnosed incidentally on histopathological examination and should be considered in the differential diagnosis of intestinal mass lesions .
heterotopic , aberrant or ectopic pancreas is defined as the presence of pancreatic tissue in topographic anomaly , with no anatomical , neural or vascular connection to the normal pancreas . it is a rare condition found mainly in stomach , duodenum and jejunum . ileal heterotopic pancreas is an uncommon condition and has been rarely reported in children so far . hereby we report a case of heterotopic pancreas presenting as ileal poyp leading to ileoileal intussusception in a 12 year child .
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primary neuroendocrine carcinoma ( nec ) of the breast is a rare distinct clinicopathological entity , comprising 0.5 - 2% of breast carcinomas world - wide . in 2003 , primary nec of the breast was identified as a distinct entity by the world health organization ( who ) classification of tumors . the who classification defines primary nec of the breast as tumors that express 50% or more of ne markers there are no previous reports of f-18 fluorodeoxyglucose ( fdg ) positron emission tomography / computed tomography ( pet / ct ) in patients with primary nec of breast with liver and bone metastasis . here we describe a case of a 45-year - old female patient who presented with jaundice and was evaluated to have multiple liver lesions and biopsy from liver showed metastatic neuroendocrine tumor . her chromogranin level was 886 ng / ml ( normal < 78 ng / ml ) . she was referred for the whole body pet / ct for detection of the primary site . pet / ct showed an intense uptake in the soft- tissue necrotic lesion in the inner quadrant of the left breast [ figure 1a and b ] with the same charecteristic feature of multiple hypodense liver lesions [ figure 1c ] . furthermore uptake noted in a lytic lesion in the d4 vertebra [ figure 1d and e ] . patient underwent fine - needle aspiration of the breast lesion , which confirmed neuroendocrine origin [ figure 2a and b ] . a diagnosis of primary nec of the left breast with metastasis was made and she was treated with peptide receptor radionuclide therapy and is on follow - up now . whole body fluorodeoxyglucose - positron emission tomography / computed tomography ( pet / ct ) maximum intensity projection image ( a ) , axial fused pet / ct showed a intense uptake in the soft - tissue lesion in the left breast ( b ) , liver lesions ( c ) and bone lesion ( d ) , axial ct showing lytic bone lesion in d4 vertebra ( e ) immunohistochemistry staining showing positive for chromogranin ( a ) and synaptophysin ( b ) primary nec of the breast is extremely rare with the first reported case in 1983 . the most frequent reported age varies from 40 to 70 years , with a higher incidence in women greater than 60 years . as metastatic neuroendocrine tumors of the breast are more common than that of primary neuroendocrine tumors of the breast , it is , therefore , important to differentiate primary breast neuroendocrine tumor from metastatic disease to the breast because of the differences in treatment focus . primary nec of the breast can be diagnosed if the presence of a non - mammary primary site can be clinically ruled out or if an in situ component is histologically detected or both . however , findings of certain studies have revealed that ne - differentiated tumors of the breast present as dense round or irregular masses with spiculated or lobular margins on the mammogram . definitive diagnosis is made with core needle biopsy , allowing for the immunohistochemical evaluation of the specimen for the ne markers . although the use of pet for the evaluation of ne tumors has been limited , tumors with moderate or high proliferative activity can be identified by fdg pet . there are reports of fdg pet / ct in a case of neuroendocrine differentiated breast carcinoma with pleural metastases using indium-111 octreotide . there are case reports of synchronous metastases to the liver and pancreas from a primary nec of the breast . our case is the first demonstrates that 18f - fdg pet / ct provides the most significant additional information related to the accurate detection of primary nec of breast and bone metastasis and guiding treatment .
cases of primary neuroendocrine carcinoma ( nec ) of the breast have been reported , though rare . we report the case of a 45-year - old woman presented with jaundice and evaluated to have liver metastasis from neuroendocrine origin . she underwent whole body positron emission tomography / computed tomography , which showed left breast lesion and bone metastasis . fine - needle aspiration ( fna ) of breast revealed a nec . a diagnosis of a primary nec of the breast was rendered with hepatic and bone metastasis . she was treated with peptide receptor radionuclide therapy and is on follow - up .
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soft tissue sarcomas are very rare tumors ; however , there are many histologic subtypes . among those subtypes , furthermore , among sarcomas originating within the retroperitoneum , which constitute 1015% of all soft tissue sarcomas , liposarcomas are the most common histologic type , accounting for 41% of these tumors [ 2 , 3 ] . liposarcomas are generally located in the head , neck , trunk , mediastinum , upper and lower extremities , gastrointestinal tract , and retroperitoneum . they commonly occur in patients aged 4060 years , and men and women are equally affected . the dimensions and weight of liposarcomas are variable ; those over 20 kg are called herein , we report a giant retroperitoneal liposarcoma weighing 25.0 kg , encasing the entire left kidney and adherent to adjacent structures which was successfully removed with kidney and aorta preserving . the patient had not experienced any symptoms , such as abdominal pain , nausea , vomiting , constipation , dyspepsia , or dyspnea . the patient was admitted to the hospital and underwent contrast - enhanced computed tomography ( ct ) of the abdomen . the scan revealed a huge fatty mass originating from the retroperitoneum probably indicative of retroperitoneal liposarcoma . the spleen was pushed anteriorly and the small bowel was deviated to the right side of the intra - abdominal space by the mass ( fig . 1 ) . because the patient was old , we decided to attempt organ - preserving surgery for the removal of the tumor to minimize the morbidity . adherence of the mass to the diaphragm , stomach , spleen , pancreas , and aorta could be observed . the greatest difficulty was that the tumor was encasing the entire left kidney and adherent to the aorta . although the entire left kidney was encased with the huge tumor , neither the renal parenchyma nor the ureter was invaded . we successfully performed a salvage of the left kidney by wide excision and separated the tumor from the aorta by shaving it away , thus preserving both kidney and the aorta . the specimen measured 45.0 30.0 11.0 cm and weighted 25.0 kg ( fig . 2 ) . microscopic examination showed a combined type of liposarcoma ( tumor component : well - differentiated liposarcoma , more than 95% , myxoid liposarcoma , less than 5% ) . an average of 12 mitotic figures were noted per high - power field . according to the grading system of the french federation of cancer centers sarcoma group , she underwent regular follow - up examinations for 16 months after the operation . at 16 months , a follow - up ct scan revealed a newly defined low - density soft tissue mass in the aortocaval and preaortocaval area , measuring 3.5 1.8 4.3 cm , suggesting a locally recurrent tumor in the retroperitoneum . we performed positron emission tomography ( pet ) , which revealed tumor recurrence in the retroperitoneum . fluorescence in situ hybridization showed mdm2 amplification , which was consistent with a diagnosis of well - differentiated liposarcoma . after the second surgery , the patient underwent regular follow - up ct scans for approximately 12 months , and to date , there has been no evidence of tumor recurrence liposarcoma is one of the most common soft tissue sarcomas , constituting approximately 20% of cancers within that group . between 10 and 15% of soft tissue sarcomas originate within the retroperitoneal space , and the most common type among these is liposarcoma . however , liposarcoma is very rare overall , accounting for 0.20.3% of all malignancies . multiple factors , such as site and depth of origin , margin involvement after resection , and histologic grade affect survival rates for patients with liposarcoma . if the size of the tumor is less than 2.5 cm , the rate of metastasis at 5 years is approximately 3% . on the other hand , the rate of metastasis at 5 years is between 55 and 60% in cases of tumors larger than 20 cm . this is because the retroperitoneal space allows the tumor to grow to a large size before the appearance of clinical signs and symptoms . therefore , the tumor is often diagnosed at the advanced stage . resection margin involvement also affects prognosis . the five recognized histologic types are the well - differentiated , myxoid , round cell , pleomorphic , and dedifferentiated type . the well - differentiated type has good prognosis , with 5-year survival rates of approximately 90% . however , the toxic effects of radiation therapy limit this option by primary treatment modality . research has documented little benefit from adjuvant chemotherapy in well - differentiated low - grade tumors , and partial responses in high - grade diseases in up to 50% of patients , with increased overall survival . as a result , complete surgical resection is the gold standard treatment , which might be curative . in many cases , combined resection of involved organs and vasculatures therefore , the most commonly sacrificed organ is the kidney , followed by the colon , pancreas , major vasculature , and spleen . in this study , we reported on a giant retroperitoneal liposarcoma encasing the entire left kidney and adherent to adjacent structures . furthermore , we described successful organ - preserving surgical removal and discussed the prognosis . although there has been no evidence of recurrence to date , we will continue to observe our patient closely for recurrence , as in other previously published reports .
retroperitoneal liposarcoma is a rare tumor . the dimension and weight of liposarcoma are variable ; those over 20 kg are called giant liposarcoma. herein , we report giant retroperitoneal liposarcoma measuring 45 cm in diameter and 25 kg in weight encasing the entire left kidney and adherent to adjacent structures . a 71-year - old woman presented for a regular checkup . image study revealed a huge mass probably indicative of retroperitoneal liposarcoma encasing the entire left kidney and adherent to adjacent structures . we performed an organ - preserving surgical removal . the pathologic report was liposarcoma . at postoperative month 16 , a follow - up ct revealed a locally recurrent tumor . the patient underwent surgical removal of the newly discovered mass . after the second surgery , the patient underwent regular follow - up ct for approximately 12 months , and to date , there has been no evidence of tumor recurrence . high - grade liposarcoma shows sensitivity to radiation therapy . however , the toxic effect of radiation therapy limits this option by treatment modality . the use of chemotherapy is also controversial . as a result , complete resection is the gold standard treatment . here , we report a giant retroperitoneal liposarcoma encasing the entire left kidney and adherent to adjacent structures , describe successful organ - preserving surgical removal and discuss prognosis .
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ameloblastoma is seen to have a benign appearance on histology , irrespective of its variable clinical behavior . microscopically various patterns have been described ( follicular , plexiform , acanthomatous , papilliferous - keratotic , granular cell type , desmoplastic , vascular , and dentinoameloblastoma).[13 ] majority of patients present in the fourth decade . men are affected slightly more often than female , with a particularly elevated incidence in eastern africa . more than 80% of ameloblastomas arise in the mandible ( mostly angle or ramus ) . they are either primary or secondary soft tissue tumors , the latter appearing after operations . radiologically , ameloblastomas present as unilocular or multilocular translucencies . malignancy in the ameloblastoma has been divided into two distant lesions . a malignant ( metastasizing ) ameloblastoma is diagnosed when a seemingly histologically benign ameloblastoma produces a metastasis resembling the original lesion . ameloblastic carcinoma is an odontogenic tumor having the overall microscopic architectural features of ameloblastoma but in addition having malignant cytological features such as marked nuclear atypia and numerous mitotic figures . in the year 1965 , tsukada et al . two years later , a case of granular cell ameloblastoma with metastasis to cervical vertebrae was reported . we reviewed the literature for any cases of granular cell ameloblastoma with metastasis , which might have been reported from year 1967 until now . to the best of our knowledge a 40-year - old female first reported to the surgery department of safdarjung hospital , new delhi , india . she presented with a disfiguring swelling on the right side of neck of approximately two years duration [ figure 1 ] . roentgenogram of the mandible showed swollen translucent cystic structures reaching from corpus to ramus of the right mandible . excision of zygomatic arch and surrounding soft tissue , masseter muscle , and temporalis muscle along with right parotid , was done . reconstruction was performed using right pectoralis major myofacial flap for buccal mucosal reconstruction and delto pectoral flap with split skin graft for skin defect . two years following surgery , patient 's follow up with x - rays , have not revealed any new lesions . patient with a large abnormal swelling on the right side of face gross examination of the excised specimen showed a well circumscribed large pink tan growth measuring 7.5 5 4 cm in the region of the mandible . tumor cells formed nests supported by fibrous connective tissue stroma [ figure 2 ] . at the periphery of the nests , the inner cell mass showed round to polygonal cells with abundant granular cytoplasm and small pyknotic nuclei [ figure 4 ] . one lymph node showed tumor metastasis characterized by the presence of granular cells [ figure 5 ] . ( h and e , 200 ) cells displaying cytoplasmic granularity with pyknotic nuclei , some of which are eccentric . ( h and e , 400 ) metastasis in lymph node showing tumor cells with granular cytoplasm . gross examination of the excised specimen showed a well circumscribed large pink tan growth measuring 7.5 5 4 cm in the region of the mandible . tumor cells formed nests supported by fibrous connective tissue stroma [ figure 2 ] . at the periphery of the nests , the inner cell mass showed round to polygonal cells with abundant granular cytoplasm and small pyknotic nuclei [ figure 4 ] . one lymph node showed tumor metastasis characterized by the presence of granular cells [ figure 5 ] . ( h and e , 200 ) cells displaying cytoplasmic granularity with pyknotic nuclei , some of which are eccentric . ( h and e , 400 ) metastasis in lymph node showing tumor cells with granular cytoplasm . the tumor is made up of proliferating odontogenic epithelium especially of enamel organ - type tissue that has not undergone differentiation to the point of hard tissue formation . it has been postulated that the epithelium of origin is derived from one of the following sources : ( 1 ) epithelial lining of odontogenic cyst , ( 2 ) dental lamina or enamel organ , ( 3 ) disturbances of developing enamel organ , ( 4 ) basal cells of surface epithelium , or ( 5 ) heterotopic epithelium of other parts of the body . our patient presented with a metastasis of granular cell ameloblastoma at the first instance , giving a brief history of 2 years . most common sites of metastasis are lung ( 76.7% ) , followed by regional lymphnodes ( 37.8% ) , pleura ( 16.2% ) , vertebrae ( 13.5% ) , skull ( 10.8% ) , diaphragm ( 8.1% ) , liver and parotid ( 5.4% ) and even more rarely , the spleen and kidney . granular cells of granular cell ameloblastoma are clearly of epithelial origin staining exclusively for cytokeratin . the term granular cell ameloblastic fibroma is a misnomer , as a number of these cases are probably central odontogenic fibromas exhibiting granular cell change . ultrastructurally it has been revealed that it is the lysosomal overload in these cells that imparts the characteristic granularity . whether granular cell change in ameloblastoma is a degenerative process or a harbinger of a more aggressive course is a matter of debate . all three cases of granular cell ameloblastoma ( our case included ) have eventually produced metastasis . add to this , our patient gave a short history of duration before tumor metastasis . although the exact implication of a granular cell change in ameloblastoma can not be ascertained due to paucity of cases documented , yet reported cases must be followed closely in anticipation of metastasis .
ameloblastoma is a slow growing odontogenic epithelial tumor of jaw . it accounts for 1% of all tumors and cysts arising in maxilla and mandible . although it is locally invasive and has a marked tendency to recur , metastasis is rare . of the various histological patterns of ameloblastoma , the granular cell type is extremely rare accounting for 4% of ameloblastomas . we report a case of granular cell ameloblastoma with metastasis to the cervical lymph node presenting in a 40-year - old indian female .
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mesenteric and omental mesothelial cysts are responsible for only 1 in 100,000 of adult hospital admissions , and are thus left low on the list of differential diagnoses for abdominal pain . further evaluation of such masses is first directed at determining a tissue origin if possible . lymphangioma accounts for the most commonly reported of such lesions , followed by enteric cyst , enteric duplication cyst , non - pancreatic pseudocyst , and mesothelial cyst . gastrointestinal leiomyomas ( cystic spindle cell tumors ) in particular have been reported to undergo central liquefactive necrosis and hemorrhage , appearing as cystic mesenteric or omental lesions on imaging . the pathogenesis of a mesothelial cyst involves failed coalescence of mesothelial - lined surfaces , typically involving the small bowel , mesentery , or mesocolon . imaging usually demonstrates a fluid - filled cavity without a readily identifiable wall . unlike lymphangiomas , however , given the overlapping features of such cystic intra - abdominal masses on imaging , definitive diagnosis is made on thorough histopathologic analysis of the resected specimen . a 41 year - old male presented to our emergency department with periumbilical abdominal pain and associated intermittent nausea and vomiting of several days duration . medical history was significant only for hypertension , and he was noted to have had prior appendectomy and posterior spinal fusion . physical examination revealed an obese abdomen with localized tenderness to palpation over the mid - abdomen without peritoneal signs . initial emergency department laboratory workup included cbc and cmp , which revealed a hypokalemic hypochloremic metabolic alkalosis . this patient was admitted to the general surgery service and treated conservatively with iv rehydration and empiric antibiotics . accounting for his chronic diarrhea , chief differential diagnosis at that time included sclerosing mesenteritis , small bowel diverticulitis , and small bowel carcinoid tumor . 5-hiaa , octreotide scan , and chromogranin a were within normal limits , lowering suspicion for neuroendocrine tumor . with conservative management , he initially reported improvement in his abdominal pain ; however , his pain never completely resolved and he eventually reported postprandial exacerbation . on hospital day 13 , the decision was made to perform diagnostic laparoscopy with potential mass and/or small bowel resection . the mass present on ct was identified intraoperatively in the mid - jejunal mesentery after an adhesion between two adjacent loops of jejunum was separated ( fig . the mass was resected along with a 15 cm segment of the involved jejunum , followed by primary anastomosis . his postoperative course was uneventful , and he was discharged shortly thereafter with resolution of his pain . on clinic follow - up , he has remained without pain . h&e staining of the specimen demonstrated spindle cells and signs of coagulation necrosis with ghost cells showing prominent eosinophilic staining fig . cd117 and dog1 staining were negative ( not shown ) , excluding gastrointestinal stromal tumor . h&e staining of the specimen demonstrated spindle cells and signs of coagulation necrosis with ghost cells showing prominent eosinophilic staining fig . cd117 and dog1 staining were negative ( not shown ) , excluding gastrointestinal stromal tumor . mesenteric and omental cystic masses are uncommon , representing only 1 in 100,000 acute adult hospital admissions , although it is possible they occur with greater frequency but are missed or identified incidentally as most remain asymptomatic . further hindering their rapid diagnosis when symptomatic is their very nonspecific presentation including but not limited to abdominal pain which is often chronic in nature , mass , or distension . this patient presented with the additional complaint of nausea and vomiting , and it is likely that local mass effect was involved , as concluded by other authors reporting on similar intra - abdominal cystic masses . the exact etiology underlying the necrosis of this patient s mesothelial cyst and its contribution to his abdominal pain is unclear . it is likely that this patient s cyst simply outgrew its own perfusion , although a possible contribution by some physiologic stressor is worth consideration . the mainstay of treatment for symptomatic lesions is complete surgical excision , , as recurrence of incompletely excised cysts has been noted by other authors . fortunately for this patient , surgery seems to have been curative as he remains well on follow - up . rare etiologies of gastrointestinal upset and abdominal pain , while reasonably left low on the differential list , ought not to be excluded from it completely . unfortunately however , it appears that mesothelial cyst and other intraabdominal cystic masses often fail to reveal themselves on physical examination and leave no characteristic clues in recent patient history , only becoming apparent nonspecifically on advanced imaging . the routine practice of ct imaging and the low threshold employed at some institutions for its use is a matter of controversy outside the scope of this case report ; however in the case of our patient , it was diagnostically important , appropriately directed treatment towards surgical management , and led to a therapeutic outcome . routine histopathological examination of resected tissue specimens is also of value in excluding malignancy , arriving at a specific diagnosis and confirming total resection of lesions that are likely to recur if incompletely excised . walter beversdorf , ms-4 : writing of case report , editing , proofing , submitting . this research did not receive any specific grant from funding agencies in the public , commercial , or not - for - profit sectors . written informed consent was obtained from the patient for publication of this case report and accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal on request .
highlightsmesenteric cystic masses account for a very small fraction of abdominal pain cases.symptoms arise likely as a result of mass effect , even when these lesions are small.on detection of these lesions by imaging , it is important to exclude neuroendocrine tumors.complete surgical excision is curative of symptoms caused by local mass effect.histopathological examination of specimens is the definitive means to differentiate between such lesions , that appear similarly on imaging .
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sarcoidosis is a rare condition with a prevalence of 4.46.3 patients per 100,000 personyears in australia , a figure similar to that of the united state 1 , 2 . it is a multisystem granulomatous disorder of unknown etiology predominantly affecting young and middleaged adults 3 , 4 . diagnostic criteria are based on a compatible clinical and radiological features supported by the histological evidence of noncaseating granulomas in the affected tissues , and the exclusion of other known causes of granulomatous inflammation 3 , 4 . pulmonary infiltration and hilar lymphadenopathy are the most common findings in more than 90% of cases , and transbronchial lung biopsy has been the recommended diagnostic procedure 3 , 4 . other organs involvement includes skin , eyes , liver , spleen , heart , musculoskeletal system , gastrointestinal tract , bone marrow , and the central nervous system . anemia has been reported in cases of sarcoidosis with the frequency ranging from 3.4% to 31% with a variety of explanations . these include hypersplenism , burden of chronic disease , autoimmunity , and bone marrow infiltration 5 . histological evaluation of extrapulmonary sarcoidosis depends on the site of involvement , but its diagnosis is rarely made via bone marrow biopsy 6 . features which may suggest bone marrow infiltration in sarcoidosis include extrapulmonary involvement and varying degree of cytopenia 7 . the characteristic granuloma in sarcoidosis is a noncaseating focal aggregation of macrophages or epithelioid cells , with or without multinucleated giant cells . its presence confirmed the diagnosis of a nonnecrotizing granuloma , in particular sarcoidosis 8 , 9 . a 40yearold caucasian woman presented with a threemonth history of polyuria , malaise , and weight loss . she had been receiving interferon ( ifn ) 1 for multiple sclerosis for the previous 12 years . investigations revealed moderate normocytic anemia , hypercalcemia , hyperglobulinemia , and acute kidney injury ( table 1 ) . her initial chest radiograph was normal , but further investigation with computed tomography showed diffuse lymphadenopathy in her chest and abdomen . a bone marrow trephine biopsy demonstrated noncaseating granulomas ( figs 1 and 2 ) and asteroid bodies ( black arrows in fig . her ifn treatment was discontinued , and she was commenced on prednisolone ( 60 mg daily ) with a slow tapering regimen over 6 months . her renal function and hypercalcemia recovered promptly , and her hemoglobin normalized at 12th week . summary of laboratory investigation results egfr , estimated glomerular filtration rate ; pth , parathyroid hormone ; k / l , kappa / lambda light chain ; ace , angiotensin converting enzyme ; si , systme international . histopathological section of a trephine bone marrow biopsy revealing a noncaseating granuloma composed of aggregation of multinucleated giant cells containing asteroid bodies ( arrowed ) . the noncaseating granuloma in the center ( black arrow ) is surrounded by pale pink amorphous material with scattered mononuclear cells interspersed throughout with some giant cells . there is also a surrounding cuff composed of reactive cells such as eosinophils and neutrophils . a second granuloma is visible in the periphery containing a langhans giant cell ( white arrow ) . a cardinal feature of sarcoidosis is the presence of cd+ t cells which display unregulated interaction with the antigenpresenting cells to initiate the formation and maintenance of noncaseating granulomas 10 . oligoclonal tcell repertoire observed in sarcoidosis suggests that the triggering antigens favor the progressive accumulation and activation of selective cd4 tcell clones 11 . this in turn leads to the preferential differentiation of the th1 helper cells which predominantly secretes interleukins ( il)2 and il12 12 . these ils have been found in higher quantity in the bronchoalveolar lavage fluid of patients with sarcoidosis and subsequently stimulate increased production of ifn and macrophage activation . it has a different structure and binds to a different receptor than the type i ifn ( and ) , and its gene is located on a different chromosome 12 . although endogenous ifn has been implicated in the pathogenesis of sarcoidosis , there is little evidence for other types of ifns 11 . the incidence of sarcoidosis is increasing with the use of type i ifn ( ifn , ifn ) for various conditions such as hepatitis b , hepatitis c , lymphoproliferative malignancy , and multiple sclerosis 13 , 14 , 15 . to date , 60 cases of sarcoidosis have been reported in the english literature in association with the use of ifn , in comparison with only six cases which were associated with the use of ifn 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 . the mean onset of development of sarcoidosis was 11.4 months after initiation of ifn therapy ( ranged 160 months ) , which is much earlier than our patient 13 . the pattern of system involvement is similar to that of the idiopathic form of sarcoidosis . only one case of the six is proven to have bone marrow involvement with a bone marrow biopsy 16 . ifninduced sarcoidosis exhibits a relatively mild disease course that usually resolves with cessation of ifn treatment . a small proportion of patients required systemic corticosteroid 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 . it is a diagnosis that should be considered in patients receiving ifn therapy of any type who present with anemia and multiorgan dysfunction . the overall pattern of organs involvement in sarcoidosis associated with ifn is similar to that of the idiopathic form .
key clinical messagesarcoidosis is a diagnosis that should be considered in patients receiving interferon therapy , who present with anemia and multiorgan dysfunction regardless of the duration of their treatment . when sarcoidosis is suspected , bone marrow biopsy should be considered especially for cases predominant by extrapulmonary features .
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thermal spring 's microbiomes have been in attention of the scientific community since past few decades . these springs , being hub of diverse microflora , increase the probability of vast gene pool of uncultured microbiota . metagenomics have helped the microbiologist to reveal the genome of the rest of the 99% of non - cultivable microbes which further helped to better understand the global microbial ecology and also helped in meeting the current demand for novel enzymes . thus , with the advances in metagenomics all the hidden facts of the microbial ecology have been faced off . odisha being rich in bio - diversity due to seasonal / climatic variations possesses a variety of hot - springs located in different geographical locations and vary both in physio - chemical and microbial ecology parameters . mainly four major hot - springs have been reported in odisha i.e. deulajhari hot spring in angul , taptapani hot spring in ganjam , atri hot spring in khurda and tarabalo in nayagarh . deulajhari hot spring is located at about 6 km from athamallik and between 203 north latitude and 8449 east longitude . in our study , sediment sample from deulajhari hot spring ( latitude 20.74199 n , longitude 84.49206 e ) was collected from the hot spring having 69 c temperature . the v3v4 region of the 16s rrna was amplified using primers 341f , 5-cctacgggaggcagcag-3 and 518r , 5-attaccgcggctgctgg-3 with 50 ng of metagenomic dna . the amplified pcr product was purified by gel elution using minelute column ( qiagen , india ) and further leads to 150 nucleotide paired end multiplex sequencing using illumina gaiix sequencer at genotypic technology pvt . krona tool was used for plotting krona graph as depicted in fig . 1 . 2,073,312 high quality reads obtained from the hot spring with temperature of 69 c were used for further analysis . out of the total , proteobacteria ( 88.12% ) , bacteriodetes ( 10.76% ) , firmicutes ( 0.35% ) , spirochetes ( 0.18% ) , thermi ( 0.13% ) and chloroflexi ( 0.11% ) were identified at the phylum level . of the total 216 genera in this deulajhari hot spring only 53.7% were identified and 46.29% were unidentified at the genus level . since all the phylotypes , retrieved through the sequencing , do not contribute to all the taxonomic groups known till date , it indicates the significance of the diversity of deulajhari hot spring explored .
insights about the distribution of the microbial community prove to be the major goal of understanding microbial ecology which remains to be fully deciphered . hot springs being hub for the thermophilic microbiota attract the attention of the microbiologists . deulajhari hot spring cluster is located in the angul district of odisha . covered within a wooded area , deulajhari hot spring is also fed by the plant litter resulting in a relatively high amount of total organic content ( toc ) . for the first time , illumina sequencing based biodiversity analysis of microbial composition is studied through amplicon metagenome sequencing of 16s rrna targeting v3v4 region using metagenomic dna from the hot spring sediment . over 28 phyla were detected through the amplicon metagenome sequencing of which the most dominating phyla at the existing physiochemical parameters like ; temperature 69 c , ph 8.09 , electroconductivity 0.025 dsm 1 and total organic carbon 0.356% , were proteobacteria ( 88.12% ) , bacteriodetes ( 10.76% ) , firmicutes ( 0.35% ) , spirochetes ( 0.18% ) and chloroflexi ( 0.11% ) . approximately 713 species were observed at the above physiochemical parameters . the analysis of the metagenome provides the quantitative insights into microbial populations based on the sequence data in deulajhari hot spring . metagenome sequence is deposited to sra database which is available at ncbi with accession no . srx1459736 .
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