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the strategy for renal replacement therapy ( rrt ) in patients with acute kidney injury ( aki ) remains controversial . to date , crucial questions such as the optimal timing , treatment intensity , and modality for acute rrt have not found definitive answers from adequately powered prospective randomized controlled trials ( rcts ) . an area of particular uncertainty is the choice between hemodialysis , hemofiltration , or hemodiafiltration ( that is , between diffusive and convective treatment or a combination of the two ) . although all three modalities can easily be performed on a modern continuous rrt machine with a few simple modifications of the setup , some major technical differences do exist . with convection , the elimination of solutes across a membrane is carried by water flux , resulting in similar removal of small , middle - sized , and larger molecules for which the membrane is permeable . in contrast , diffusive clearance is highly efficient in removing small solutes but less so in eliminating larger compounds ( such as uremic ' middle molecules ' , myoglobin , or cytokines ) . however , convection requires large rates of ultrafiltration in order to be effective . if the necessary replacement fluid is administered post - filter , hemoconcentration within the hemofilter will result and patients potentially will be predisposed to clotting . on the other hand , if replacement fluid is added pre - filter , the treatment efficacy is reduced since solutes are diluted before elimination . whether and how these fundamental differences translate into clinical outcomes are unclear at present . in this issue of critical care , friedrich and colleagues present a systematic review and meta - analysis of clinical trials that compare hemofiltration and hemodialysis for the treatment of aki . the authors found no difference in mortality or other clinical outcomes such as rrt dependence in survivors , organ dysfunction , or vasopressor use . not unexpectedly , the data suggested an increased clearance of larger molecules with hemofiltration but also a shorter filter life . the essential conclusions from the meta - analysis are that we do not have a sufficient database at present to recommend one procedure over the other , a fact reflected in the current aki guidelines of the kdigo ( kidney disease improving global outcomes ) group and the uk renal association , and that we will require larger clinical trials before definitive recommendations can be made . however , the question is whether a ' definitive ' prospective rct in unselected populations with aki will actually help to resolve this issue . as with the choice of intermittent versus continuous versus ' hybrid ' rrt as the initial treatment for aki , one size more likely , future studies will have to address the question of whether there are specific subgroups of patients who might benefit from convective therapies ( for example , myoglobinuric or septic aki patients in whom the enhanced removal of myoglobin or cytokines by hemofiltration might help to improve clinical course and renal recovery ) . moreover , the question of rrt ' dose ' is inextricably linked with the choice of modality . if replacement fluid is added pre - filter in order to limit hemoconcentration and clotting risk , total treatment volumes this , together with a potentially reduced filter life , may lead to increased costs of convective therapies in comparison with continuous hemodialysis . in clinical practice , the intensivist will have to balance the desired intensity of treatment , in particular with regard to the removal of larger molecules , against clotting risk , filter life , and costs . as the current uk renal association guidelines put it , ' choice of rrt modality should be guided by the individual patient 's clinical status , medical and nursing expertise , and availability of modality ' . aki : acute kidney injury ; rct : randomized controlled trial ; rrt : renal replacement therapy .

the optimal choice of modality for acute renal replacement therapy is unclear at present . diffusive therapy ( hemodialysis ) removes small solutes mainly , whereas convective therapies ( hemofiltration and hemodiafiltration ) may also eliminate larger molecules such as myoglobin or cytokines . conversely , convective therapies might predispose patients to filter clotting and thus increased costs . a systematic review and meta - analysis of clinical trials could not find evidence for clinical benefits of either modality . thus , the decision on renal replacement therapy modality still is based on the clinical status of the individual patient , the expertise of the medical and nursing staff , and local circumstances and availability .

ventricular septal rupture ( vsr ) is a serious complication of acute myocardial infarction ( ami ) that generally causes progressive circulatory failure , rapid clinical state deterioration , and death , despite advances on the treatment of it . the incidence of vsr after ami was found to be about 0.71 % with using primary percutaneous coronary intervention ( ppci ) . although this decrease is promising , the mortality rate of this complication compared to ami without vsr has remained high . we present the case of a 59 year - old patient with acute vsr related to ami who underwent a surgical correction , and died shortly after the surgery . the patient was a 59-year - old caucasian female with no record of hypertension , diabetes , hypercholesterolemia , tobacco use or previously known coronary artery disease . the patient first presented to the emergency department of a hospital complaining of chest pain . she was diagnosed with acute anterior myocardial infarction and referred to our hospital after 2 hour of symptoms onset . she was admitted to the coronary care unit ( ccu ) and continued on her ordered medications . transthoracic echocardiography showed akinesia in the apex and apical septum and mid septum walls , with a calculated ejection fraction of 56% . a cag showed a 98 % stenosis in the proximal lad and pci was performed successfully without any complication ( figure 1 a , b ) . however , despite full anti ischemic therapies , the patient s condition became worse , with decline of the cognitive state , on the 7 day of ami . she had progressive hypotension and dyspnoea and there was a harsh grade iv / vi systolic murmur best heard at the apex , radiating to the axilla . ongoing tachycardia , patient s unstable condition and persistent st segment elevation and led to an urgent bedside echocardiogram which showed apex , apical septum and mid septum - walls akinesis , a large echodense area at the side of apical septum ( figure 2 ) , through which continuous wave - doppler examination suggested blood crossing between right and left ventricle ( figure 3 ) . an intra - aortic balloon pump ( iabp ) was inserted . the patient could not be weaned from the iabp because of severe hemodynamic compromise on the first postoperative day and inotropic drugs were increased to maximum dose . in spite of all efforts she died on the same day of the operation . figure 2a large echodense area at the side of apical septum . a large echodense area at the side of apical septum . figure 3continuous wave doppler recordings from the parasternal position of an infarct related ventricular septal defect . the peak systolic velocity is 2.4 cm / s , corresponding to a 24 mmhg pressure gradient between the lv and rv . lv , left ventricle , rv , right ventricle , vsd , ventricular septal defect . continuous wave doppler recordings from the parasternal position of an infarct related ventricular septal defect . the peak systolic velocity is 2.4 cm / s , corresponding to a 24 mmhg pressure gradient between the lv and rv . lv , left ventricle , rv , right ventricle , vsd , ventricular septal defect . the contemporary strategy of the treatment of acute coronary syndromes is now focused on primary pci of culprit lesion using balloon angioplasty and stent implantation , but vsr as the most feared complication of ami still caries extremely high mortality rate . clinical features associated with an increased risk of rupture of the interventricular septum include lack of development of a collateral network , advanced age , single vessel coronary artery disease ( cad ) , hypertension , anterior location of infarction , no current smoking , high killip class , lower body mass index ( bmi ) , and possibly thrombolysis . post - infarction vsd is usually located in the anterior or apical portion of the ventricular septum ( about 60% of cases ) as a result of an anterior mi . patients with vsd had relatively longer time from the ami onset to primary pci compared to the non - vsd group and female gender was the strongest independent predictor of ami related vsd . one hypothesis is based on a concept that , in women , the rupture is attributable to more susceptible collagen framework and to differences in the collagen matrix within the infarcted myocardium . our patient s demographic characteristics were consistent with these poor outcomes including an anterior infarct with single cad except for having the chance of early intervention which is about 2 hour from the onset of ami symptoms . although many surgeons recommend surgical vsd closure after a 34 week delay to allow scarring of the surrounding tissue to occur , which allows for firmer anchoring of suture and patch material , unfortunately , in this case we were not able to postpone the operation because the patient did express cardiogenic shock despite an iabp . in the ward , after three hours of operation , the patient s condition became worse and she died shortly after it . this case is an example of acute vsr causing catastrophic death , despite percutaneous coronary intervention after myocardial infarction and surgical correction . awareness of the clinical signs and early use of echocardiography may be life - saving , but the outcomes related to interventricular septum rupture even in the advanced interventional era are bad . more effective ways to predict , prevent , and treat this devastating complication are needed .

ventricular septal rupture ( vsr ) complicating acute myocardial infarction ( ami ) is a serious clinical problem with high mortality rate due to cardiogenic shock or prolonged hemodynamic compromise . despite multiple improvements in medical , interventional and surgical techniques , early and long - term prognosis after ami related vsr still remain unpromising . we report a patient in whom an acute vsr was diagnosed 7 days after an anterior myocardial infarction treated with early primary percutaneous coronary intervention ( ppci ) .

schwannomas are benign slow - growing mesenchymal neoplasms that arise from schwann cells with a low malignant potential . they are rare tumors , occurring most commonly intracranially in acoustic nerves or spinal nerves . gastrointestinal schwannoma ( gis ) represents only 26% of all submucosal tumors of the gastrointestinal tract , with the stomach being the most affected organ ( 6070% ) . the incidence of gis in the cecum , because of the rare occurrence , is not reported in the literature . gis is a distinctive neoplasm usually difficult to distinguish from others tumors of the gastrointestinal wall , such as gastrointestinal stromal tumors ( gist ) , lipomas , leiomyomas and leiomyossarcomas , despite the fact that gis has a well - defined immunohistochemical pattern that is different from the others tumors , showing positive s-100 protein and vimentin in tumor cells . here , we present a very rare case of schwannoma of the cecum and review the literature , focusing on the different aspects of diagnosis and treatment of this tumor . a 79-year - old woman presented to her physician with recurrent pain in the right upper abdomen following meals , associated with nausea and vomiting . she had also complained of hyporexia with a weight loss of 11 pounds over the prior 3 months . the patient had no history of previous lower abdominal pain , melena , hematochezia or surgical interventions . an abdominal ultrasound was ordered , which detected gallbladder stones and also a hyperechoic mass in right iliac fossa . further investigation with abdominal computed tomography ( ct ) showed a well - defined round mass of 10 cm in maximal diameter behind the cecum , with homogenous and minimal contrast enhancement ( fig . the intraoperative findings were a retroperitoneal mass with no cleavage plane with the cecum wall ( fig . 2 ) , and cholelitiasis . a frozen section of the retroperitoneal specimen was suggestive of gist . macroscopic examination of the resected segment showed a solid cystic lesion in the cecum wall , which measured 9.0 7.5 cm , with minimal margin of 3 cm . light microscopic examination of the specimen showed a mesenchymal neoplasm of the cecum wall , with spindle cells and mild atypia arranged in whorls , and a low mitotic count ( 2 per 50 high - power fields ) . the diagnosis was a low - grade mesenchymal spindle - cell neoplasm , and further investigation with immunohistochemical staining ( ihs ) was necessary for the differential diagnosis . ihs was strongly positive for vimentin and s-100 protein and negative for hmb-45 , cd-34 , cd-117 , compatible with schwannoma . the patient was discharged 7 days after the surgery and no further treatment was indicated in follow - up . schwannoma is a benign neurogenic neoplasm that originates from schwann cells ; it is slow growing with low malignant potential . it usually affects the peripheral nerves of limbs , the spinal cord and central nervous system . primary and isolated gis are not frequent , and occur most commonly in the stomach and jejunum , with the cecum rarely affected . because of the lack of uniformity in terminology of neurogenic tumors ( schwannoma , neurilemmoma , neurofibroma , plexiform neurofibromatosis ) , their incidence in the large bowel is not known , and only a few case have been reported to date . the symptoms depend on tumor size , although the disease can be totally asymptomatic , presenting as an incidental finding in imaging exams . ct and magnetic resonance imaging can establish the tumor site , its characteristics and relationships with other organs . the light microscopy with hematoxylin and eosin stain is not accurate to distinguish the neurogenic from the myogenic spindle - cell tumors , and ihs with broad - panel markers is mandatory for this differential diagnosis . schwannomas are neurogenic tumors and usually show s-100 protein and vimentin , while they are negative for desmin , keratin , glial fibrillary acidic protein , smooth muscle and muscle - specific actin , cd34 and ema 1 . in this case , ihs was strongly positive for s-100 protein and vimentin . intracellular pas - positive crystalloids could be a marker of cellular differentiation of schwann cells . the intraoperative frozen section analysis can help to diagnose the main tumor lineage , and eventually it can suggest the malignant behavior of the neoplasm . the prognosis of patients with schwannoma of the cecum is good after an adequate surgical resection , and no adjuvant therapy is necessary .

a 78-year - old woman presented with an abdominal mass diagnosed by ultrasound and computed tomography . the patient underwent a laparotomy , during which a retroperitoneal tumor adherent to the cecum wall was identified . microscopically , it showed spindle - cell proliferation in whorls , with low mitotic count ( 2 per 50 high - power fields ) and was strongly positive for s-100 protein and vimentin . the final diagnosis was benign schwannoma of the cecum and no further treatment was required . large intestine schwannomas are extremely rare tumors and only a few cases of schwannoma of the cecum have been reported to date .

a 23-year - old male presented with the complaint of decreased vision in the right eye since 3 months . he had been treated elsewhere for right eye choroiditis with intravenous methyl prednisone acetate ( ivmp ) 1 g / day for 3 days followed by oral steroids and tab azathioprine . at presentation , he was taking tab prednisone 50 mg / day and tab azathioprine 125 mg / day . best corrected visual acuities ( bcva ) were 20/200 and 20/20 in the right and left eyes , respectively . right eye revealed quiet anterior chamber and quiet vitreous cavity with a yellowish area of active choroiditis with geographic borders involving the macula in serpiginous pattern . he was diagnosed as a case of active macular serpiginous choroiditis . after obtaining physician 's clearance , we prescribed tab prednisone 60 mg / day ( 1 mg / kg body weight ) , tab azathioprine 50 mg thrice a day , tab cyclosporine 150 mg twice a day , with antacid and calcium supplements . quantiferon - tb gold test was found to be negative . on regular follow - up visits , the choroiditis lesions were noted to heal over a period of 3 months , and treatment was gradually tapered . after 3 months , patient complained of redness , pain and blurring of vision in the left eye . at this point of time , he was on tab prednisone 7.5 mg / day , tab azathioprine 50 mg / day and tab cyclosporine 200 mg / day . right eye examination revealed a quiet eye with healed lesions of macular serpiginous choroiditis [ fig . 1 ] . left eye had 2 + anterior chamber cells , severe vitritis , and an area of retinal whitening suggestive of necrotizing retinitis in the inferonasal periphery . tab cyclosporine and tab azathioprine were stopped , tab prednisone was hiked to 60 mg / day , and intravenous aciclovir 500 mg thrice a day was started under monitoring . serum elisa for human immunodeficiency virus ( hiv ) was negative . on regular examinations , vitreous haze decreased markedly and the lesion showed signs of healing [ fig . , intravenous aciclovir was stopped and the patient was prescribed tab valaciclovir 1 g thrice a day , and tab prednisone to be tapered at 10 mg / week . as on last visit after 2 months , anterior chamber was quiet and retinitis had resolved significantly . color fundus photograph of the right eye showing the healed lesions of macular serpiginous choroiditis color fundus photograph of the left eye showing presence of vitreous haze with white lesion of necrotizing retinitis ( acute retinal necrosis ) in the inferonasal periphery we report a patient who developed arn in the fellow eye while he was on tapering doses of immunosuppressive medications for unilateral serpiginous choroiditis . to the author 's knowledge this association can be explained by two possibilities ; it may represent a common viral etiology of the two diseases or development of arn due to general state of iatrogenic immunosuppression . gass et al . reported a case of serpiginous choroiditis following herpes zoster ophthalmicus and suggested a possible viral etiology in some patients with serpiginous chorioditis . suggested that herpes viruses might have a role in the pathogenesis of the disease in a subset of patients with serpiginous choroiditis . reported increased levels of antiviral ( herpes simplex ) antibodies in serum of two patients with serpigenous choroiditis . ampigenous choroiditis , a variant of serpiginous choroiditis , has been reported to occur following quadrivalent human papilloma virus vaccine . the treatment of serpiginous choroiditis with systemic antivirals has been recommended and practiced by some authors . in our case , the limitation is the lack of serological diagnosis to prove a common herpetic origin of the serpiginous choroiditis in the right eye and necrotizing retinitis in the left eye . however , most immunologic explanations of serpiginous choroiditis deal with hypersensitivity reactions and virus would not need to be present in the eye in order to explain the disease . likewise , though anterior chamber biopsy with polymerase chain reaction ( pcr ) testing for herpes virus would have strongly confirmed the diagnosis of arn in the left eye , arn is a clinical diagnosis and has an established viral etiology arn has been reported to occur in immunosuppressed patients , including iatrogenic immunosuppression and following intravitreal steroid injections . though our patient was not found to have an hiv infection , he had received a course of ivmp elsewhere and was on immunosuppressive medications for almost 9 months when he developed arn . the temporal relationship between serpiginous choroiditis and arn , reported in the present case , is intriguing . our case report also highlights the importance of a detailed evaluation of both the eyes on regular follow - up visits in the patients receiving iatrogenic immunosuppression .

we describe a rare association of serpiginous choroiditis with necrotizing retinitis having clinical features of acute retinal necrosis ( arn ) . a 23-year - old male developed arn in the fellow eye while he was on tapering doses of immunosuppressive medications for unilateral serpiginous choroiditis . the association may represent a common viral etiology of the two diseases or may be due to the development of arn due to general state of iatrogenic immunosuppression . this report also highlights the importance of a detailed evaluation of both the eyes on regular follow - up visits in the patients receiving iatrogenic immunosuppression .

familial mediterranean fever ( fmf ) is a genetic disease characterized by recurrent febrile attacks and inflammation of serous membranes . amyloidosis is frequent in untreated fmf patients and is also the most important complication of fmf . it is generally seen with renal , hepatic , gastrointestinal , spleen , testicular and thyroidal involvement . herein , we report a case with acute scrotum and hydrocele amyloidosis as a presenting finding in a child with fmf . although the acute scrotum and scrotal swelling are not characteristic clinical features of fmf , this genetic disease should not be forgotten in the differential diagnosis of acute scrotum in patients of mediterranean origins . familial mediterranean fever ( fmf ) is an autosomal recessive inherited disorder characterized by recurrent episodes and self limited attacks of fever , peritonitis , pleuritis , arthritis and erysipelas - like erythema . the most important complication of fmf is amyloidosis and is characterized by the generalized or localized extracellular deposition of amyloid , a proteinaceous fibrillar material , in different tissues and organs . orchitis and testicular amyloidosis are rarely reported due to fmf but can also occur with testicular amyloidosis typically being asymptomatic . here we present a case with acute scrotum and hydrocele amyloidosis as a presenting sign of fmf . a previously healthy 14-year - old turkish boy was admitted to the emergency room with swollen , painful and erythematous scrotum . at admission , he had no fever or abdominal pain . on physical examination , translumination was positive on the scrotum . 48 mm / h ( normal range : 015 ) and c - reactive protein 43 mg / l ( normal range : 05 ) . serum amyloid a level was 315 ng / ml ( normal range : < 10 ng / ml ) . scrotal and abdominal ultrasonographs showed epididymal enlargement , thickened scrotal skin , bilateral hydrocele and splenomegaly . the abdominal pain was diagnosed as a urinary tract infection and was treated with antibiotics . the histopathological examination of the removed hydrocele sac revealed amyloid deposition in the vessel walls . fmf can be seen at every part of the body , is characterized by fever , arthritis , abdominal and chest pain . patients ' clinical features are peritonitis ( 93.7% ) , fever ( 92.5% ) , arthritis ( 47.4% ) , pleuritis ( 31.2% ) , myalgia ( 39.6% ) and erysipelas - like erythema ( 20.9% ) . the symptoms of fmf are related to the inflammation of serosal membranes . an extension of peritoneal sac , tunica vaginalis , could be affected by fmf at the same time . there are few studies reporting scrotal swelling frequency in 4 - 8% of fmf patients[6 , 7 , 8 ] . many other articles on fmf from turkey did not mention acute scrotum or scrotal swelling . a complete recovery is expected without significant consequence[6 , 9 ] . a recent study from turkey indicated that fmf is the most common cause of amyloidosis in turkish patients and the highest frequency of fmf ( 1/123 ) abdominal pain can be very dull and yet is sometimes very serious and seems to be an acute abdomen . patients with typical phenotype and genetically confirmed mutations of fmf are defined as phenotype i , but phenotype ii patients develop amyloidosis without any previous attacks typical of fmf . in childhood , fever or abdominal pain could be attributed to infectious disease rather than fmf so diagnosis of fmf can be postponed to older ages . although our patient 's first two abdominal pain attacks were not clear and characteristic , it can still be attributed to fmf . these reports and findings were recognized as a clue for investigating fmf in this patient . other differential diagnosis of acute scrotum and scrotal swelling were ruled out by physical examination , laboratory and radiological test findings . although acute scrotum has been recognized as a feature of fmf in jewish and arab fmf patients , it can also be a clinical feature in turkish fmf patients . early diagnosis and colchicine prophylaxis are essential to prevent amyloidosis , which is the most significant , unique and lethal complication of fmf . around the age of 40 , these patients undergo unnecessary interventions and recurrences , which lead to risk of ischemic testicular necrosis[6 , 13 , 14 ] . although the acute scrotum and scrotal swelling are not characteristic clinical features of fmf , this genetic disease should not be forgotten in the differential diagnosis of acute scrotum in patients of mediterranean origin .

backgroundfamilial mediterranean fever ( fmf ) is a genetic disease characterized by recurrent febrile attacks and inflammation of serous membranes . amyloidosis is frequent in untreated fmf patients and is also the most important complication of fmf . it is generally seen with renal , hepatic , gastrointestinal , spleen , testicular and thyroidal involvement.case presentationherein , we report a case with acute scrotum and hydrocele amyloidosis as a presenting finding in a child with fmf.conclusionalthough the acute scrotum and scrotal swelling are not characteristic clinical features of fmf , this genetic disease should not be forgotten in the differential diagnosis of acute scrotum in patients of mediterranean origins .

parinaud oculoglandular syndrome is a rare eye disease caused by different etiologic agents , including bacteria , viruses and fungi . it is characterized by a granulomatous conjunctivitis , accompanied by adjacent preauricular lymphadenopathy which is almost always caused by local trauma and can bring consequences if not treated promptly . we present a case caused by the dimorphic fungus sporothrix schenckii from the sporotrichosis epidemic occurred in rio de janeiro , brazil . male , 21 years - old , single , student , resident in seropedica city / rio de janeiro state / brazil . refers to appearance of a finger injury two months after a sick cat scratch with subsequent involvement of the eye . the clinical examination presents erythematous crusted lesion of 0.3 cm in size , located on the index finger ( fig . 1a ) and in the right eye was observed a small rounded painful lesion with sessile base and soft consistency , located in the lower tarsal conjunctiva ( fig . 1c and d ) . itraconazole 100 mg day was administered and the patient is in regular monitoring in dermatology and ophthalmology departments . parinaud 's oculoglandular syndrome is a curious medical condition and it has been associated with several different infectious diseases , especially to cat scratch disease ( bartonella henselae ) , but sometimes it has been reported in granulomatous chronic infectious like tuberculosis , atypical mycobacteria and syphilis and even some viruses ( herpes simplex virus ) . fungal infections also have been associated to this syndrome especially sporotrichosis ( s. schenckii ) , blastomycosis ( blastomyces dermatitidis ) and coccidioidomycosis ( coccidioides immitis ) . sporotrichosis is a subacute or chronic mycosis that affect all ages , currently distributed throughout the world , especially in tropical and subtropical zone . infection almost always occurs by traumatic inoculation of soil , plants and organic matter contaminated with fungus and may be localized in the conjunctiva as a primary infection or may be secondary involving of the eyelid and face . numerous soft , yellow , granulomatous nodules , which may ulcerate , develop in the palpebral or bulbar conjunctiva of the involved eye . there is no evidence how the infectious agent has been disseminated throughout the rio de janeiro municipality and its outskirts , but it is beyond reasonable doubt that the close interaction with cats represents a key form of transmission of the fungus , , . felines have very close contact with contaminated soil and organic matter and constitute a reservoir of this agent . the gold standard for sporotrichosis diagnosis is culture and drug of choice to treat these patients has been oral itraconazole . ophthalmologists and dermatologists must be aware of its occurrence especially in endemic regions in order to avoid diagnostic pitfalls . suzana corte - real and ftima conceio - silva : laboratory diagnosis and paper preparation . the authors declare that a 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 .

the parinaud oculoglandular syndrome is a rare eye disease caused by different etiologic agents , including bacteria , viruses and fungi . it is characterized by a granulomatous conjunctivitis , accompanied by adjacent preauricular lymphadenopathy and can bring consequences if not treated promptly . we present a case of parinaud oculoglandular syndrome caused by sporothrix schenckii from the sporotrichosis epidemic in its zoonotic form occurring in rio de janeiro , brazil .

lung sonography is an essential component of lci ( lung , cardiac , inferior vena cava ) ultrasound exam . lung emergencies in the operating room such as pulmonary edema or pneumothorax are low - incidence events . we have developed models that simulate pulmonary sonopathology and allow supervised teaching of lung sonography . lung is a bi - compartmental ( air and tissue ) organ and can be modeled by a polyurethane foam . transverse sonograms of the metacarpal bones of the hand and images of intermetacarpal tissue resemble sonographic images of ribs and intercostal spaces . the hybrid simulation model of the lung ( foams with the attached pig rib cages wrapped in the ioban dressing ) . the rib cage elevated above the foam (= pneumothorax ) scanning of the palmar aspect of the hand ( m metacarpal bones ) . the hand on the surface of wet ( b and d ) and almost dry foams ( c and e ) fig . 1 a shows an intercostal sonogram of the foam with a single horizontal reverberation artifact ( a ) . to simulate pneumothorax similar images of multiple horizontal artifacts ( a - lines ) were obtained during scanning of the hand elevated above the foam ( fig . when the dorsum of hand was placed on the wet foam a - lines disappeared ( fig . they were well visualized when a high resolution ( 15 mhz ) transducer was used ( fig . we could demonstrate this sign by sliding the dorsum of hand back and forth on the surface of a pu foam or a metal plate . in this study this model consists of the experimenter 's hand placed on top of the water - filled container with a wet foam . metacarpal bones of the human hand simulate a rib cage and a wet foam simulates a diseased lung immersed in the pleural fluid ( fig . the experimenter 's hand is placed on top of the fluid - filled container with a wet foam ( left panel ) . a sonogram ( right panel ) of metacarpal bones ( m ) of the human hand which simulate a rib cage and a wet foam underneath simulates a diseased lung ( l ) that is immersed in the pleural fluid ( f ) ; n needle sonographic exam of the pathological lung requires interpretation of real images and artifacts such as a - lines ( beam reflections from the pleura ) and b - lines ( beam reflections between fluid - filled or air - filled alveoli ) . a - lines can be visualized in the normal lung but their number increases dramatically during pneumothorax . this quantitative difference can be confusing to the novice lung sonographer but can be easily taught in our model . the use of human hand to simulate a sonogram of a rib cage has been described before . the authors used sliding fingers across the dorsum of hand to simulate lung sliding . in this study we have combined the use of human hand with the use of dry or wet pu foams simulating normal or fluid overloaded lungs and lung sliding sign . both our models produced images that accurately depict sonopathology of the lung . several ultrasound guided procedures require , for safety reasons , initial training in animal models or phantoms . in - vitro models can facilitate learning of scanning techniques and hand - eye coordination skills . the elastomeric phantoms that are usually used for training lack tissue feedback , are expensive , rapidly deteriorate and become unusable due to needle tracks . the thoracentesis model presented in this study is simple , inexpensive and easy to assemble . metacarpal bones of the human hand convincingly simulate a rib cage , but at a minimal extra cost can be replaced by animal ribs with intercostal muscles placed on top of the water - filled container with a wet foam simulating a diseased lung . the author do not report any financial or personal links with other persons or organizations , which might negatively affect the content of this publication and claim authorship rights to this publication .

lung sonography allows rapid diagnosis of lung emergencies such as pulmonary edema , hemothorax or pneumothorax . the ability to timely diagnose an intraoperative pneumothorax is an important skill for the anesthesiologist . however , lung ultrasound exams require an interpretation of not only real images but also complex acoustic artifacts such as a - lines and b - lines . therefore , appropriate training to gain proficiency is important . simulated environment using ultrasound phantom models allows controlled , supervised learning . we have developed hybrid models that combine dry or wet polyurethane foams , porcine rib cages and human hand simulating a rib cage . these models simulate fairly accurately pulmonary sonopathology and allow supervised teaching of lung sonography with the immediate feedback . in - vitro models can also facilitate learning of procedural skills , improving transducer and needle positioning and movement , rapid recognition of thoracic anatomy and hand eye coordination skills . we described a new model to teach an ultrasound guided thoracentesis . this model consists of the experimenter 's hand placed on top of the water - filled container with a wet foam . metacarpal bones of the human hand simulate a rib cage and a wet foam simulates a diseased lung immersed in the pleural fluid . positive fluid flow offers users feedback when a simulated pleural effusion is accurately assessed .

the safe , effective recovery of organs with maximal transplant potential is of paramount importance particularly , in the context of acute shortage of donor organs . vascular anomalies at the time of operation can present significant challenges testing the technical and anatomical knowledge of the recovery surgeon . we highlight the case of a double inferior vena cava ( ivc ) encountered in a multi - visceral recovery operation , outline our operative approach and serve as a reminder of this rare anatomical variation . the donor was a 34-year - old brainstem dead male who had suffered a non - traumatic intracranial haemorrhage . there was no significant past medical history including any congenital defects . no previous or current abdominal cross sectional imaging abdominal and thoracic multi organ procurements were planned with heart , lungs , liver , kidneys and pancreas accepted for transplantation . intra - operative photograph of double ivc in a deceased multi organ donor laparotomy revealed the presence of a double ivc ( figure 1 ) . the two vena cava were approximately of equal caliber . at the level of the kidney , the left renal vein joined the left vena cava which then passed to the right and joined the right vena cava . the combined ivc trunk then traversed the normal retro hepatic route before passing through the diaphragm in to the right atrium in normal anatomical position . thoracic organs ( heart and lungs ) were procured first and no anatomical anomaly was reported . the supradiaphragmatic ivc was incised after start of aortic perfusion to ensure adequate flush of both right and left ivc . liver was removed after dividing supra and common infra hepatic ivc , portal vein and celiac trunk . the left renal vein was retrieved with the intact left ivc which was divided before its junction with the right ivc . similarly the right renal vein was retrieved with intact right ivc . duplication of the ivc is one of the most common anomalies affecting the inferior vena cava ( ivc ) , next to transposition ( left sided ) , and has been reported to occur in 0.2 - 3% of the population ( 3 ) . recognizing this anomaly is important in order to procure abdominal organs safely and also to recognize other anomalies that may co - exist with this condition . duplication of ivc in the context of organ procurement has not been previously described in literature . during the embryonic development the formation of the ivc is a complex multistep process involving three paired venous channels ( posterior cardinals , sub cardinals and supra - cardinals ) . the adult ivc is the result of , the formation of communicating channels between them and the eventual regression of specific segments before the 8 week of gestation ( 1 ) , ( 2 ) . duplication of ivc arises as a result of the persistence of the right and left supra cardinal and sub cardinal veins ( 2 ) . the left vena cava joins it in front or behind the aorta at the level of or just above the left renal vein ( figure 2a , b , c , d ) . the size of the left vena cava can vary significantly and drain directly into the left renal vein ( 4 ) . associated conditions of relevance to the abdominal procurement surgeon include anomalies of the renal veins ( circum aortic renal collar , and retro aortic renal vein ) ( 1 ) , right retro caval ureter ( 3 ) , horseshoe kidney , congenital right renal agenesis ( 4 ) and cloacal exstrophy ( 5 ) . lumbar vein aberrations , including agenesis and collaterals have also been associated with vena cava anomalies . ( 2a ) common variant , left ivc crossing in front of the aorta , ( 2b ) the left ivc passes behind the aorta , ( 2c ) transposition of ivc ( left ivc ) , ( 2d ) circum aortic left renal vein . a computerized tomogram ( ct ) or a magnetic resonance ( mr ) angiogram arethe best diagnostic modalities for identifying and mapping venous anomalies within the retro peritoneum . if preoperatively identified , this will allow for safe surgical planning and outcomes ( 6 ) . whilst , a rare condition one should be alert to potential caval abnormalities when reviewing abdominal imaging of potential donors , ( figure 3 ) as anomalous venous structures tend to be dilated and tortuous making injury more likely ( 7 ) . in deceased donor procurement setting , cross sectional imaging is usually not available at and a surgeon will have to rely on vigilance , awareness of potential anatomical variation and ensure adequate exposure of major vessels . the identification of a duplicated ivc in our donor did increase operative time , in order to define the true nature and extent of the anomaly and to plan the procurement of intra abdominal organs without compromising the utilization of procured organs for transplantation .

we highlight the case of a double inferior vena cava ( ivc ) encountered in a multi - visceral recovery operation on a deceased donor . in such cases pre - operative abdominal cross sectional imaging is usually not available hence the procurement surgeon needs to rely on vigilance and awareness of other potential anatomical variations that are associated with the condition . we outline our operative approach , summarize the embryological development of ivc and present the case as a reminder of this rare anatomical variation .

a 61 year old african american male currently receiving chemotherapy for multiple myeloma presented with a 3 month history of a non - healing wound on the finger that occurred after a puncture wound from a rose thorn while gardening . just proximal to the nail fold , was a tender 1.4 1.2 cm hyperpigmented , hyperkeratotic plaque with an area of shallow erosion . the patient was treated with itraconazole 200 mg po daily for presumed sporotrichosis infection . histology revealed full thickness epidermal keratinocyte atypia consistent with squamous cell carcinoma in - situ ( sccis ) and it resolved with excision ( fig . 1 , fig . candida parapsilosis has been reported as the predominant candidal species transmitted in hospitals from the hands of health care personnel , particularly those with onychomycosis , onycholysis , or artificial nails . like most species of candida , c. parapsilosis is a transient commensal of skin flora and its pathogenicity is limited to breaches in the skin barrier . disease is usually limited to superficial skin infections such as ulcers and most infections resolve spontaneously without treatment . however , infection can become severe in immunocompromised patients , patients receiving parenteral hyperalimentation , and patients in the icu . amphotericin b has been the traditional treatment for c. parasilosis candidemia , but there are no established treatment guidelines . owing to their more favorable side effect profile , fluconazole and other azole antifungals are utilized as alternatives to amphotericin . low level resistance to azoles has been reported , with fluconazole and itraconazole demonstrating slightly higher levels of resistance than voriconazole . however the rate of resistance remains lower than 5% and overall susceptibility still remains high . given our patient s immunosuppression and preceding rose thorn puncture wound , sporotrichosis was initially suspected . culture grew candida parapsilosis , which has been reported to mimic sporotrichosis infection following rose thorn injury . however , c. parapsilosis can colonize the skin and nails and our histology revealed squamous cell carcinoma in - situ . culture growing c. parapsilosis as the sole pathogen could be suggestive of infection over colonization , which may have led to a misdiagnosis and inappropriate treatment in our case . we present this case to highlight the importance of obtaining histological examination in addition to culture . his immunosuppression raised concern for infection , however it also confers an increased risk of skin cancer . it is well - known that iatrogenic immunosuppression can lead to an increased risk of secondary primary tumors , particularly scc . in kidney and heart transplant patients receiving immunosuppressive therapy , the degree of risk also has a linear correlation with the number and/or dosages of the medications used . for example , compared to kidney transplants , heart transplant recipients , who typically receive more medication for immunosuppression , were three times more likely to develop nonmelanoma skin cancer . furthermore , patients with multiple myeloma have nearly double the risk of developing skin cancer compared to age-,race- and gender - matched control subjects , with the most significant being scc . in another case of c. parapsilosis following rose thorn injury in an immunocompetent patient , cultures similarly grew solely c. parapsilosis and the wound healed following antifungal therapy with itraconazole . although our case draws many similarities , including a rose thorn injury and subsequent growth of c. parapsilosis from cultures , our biopsy of the lesion revealed sccis . this highlights the importance of biopsy since squamous cell carcinoma and fungal infection can not be distinguished on clinical grounds alone and tissue culture can be misleading in lesions that are secondarily colonized . 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 . both authors have seen and approved the manuscript , contributed significantly to the work , and also that the manuscript has not been previously published nor is not being considered for publication elsewhere . this research did not receive any specific grant from funding agencies in the public , commercial , or not - for - profit sectors .

a 61 year old immunosuppressed male presented with a non - healing wound following a rose thorn injury . initial cultures revealed candida parapsilosis , however histology confirmed squamous cell carcinoma in - situ ( sccis ) . this case emphasizes the importance of biopsy and cultures as fungal infection and sccis often have similar , non - specific clinical appearances .

electronic cigarettes ( i.e. , e - cigarettes ) are battery - powered devices that delivery nicotine in an aerosol to the user . the first electronic cigarette was created in 1963 when an american engineer named herbert a. gilbert filed a patent for a device that produced a nicotine - containing steam . however , this device was never commercialized . the modern electronic cigarette was invented in 2003 by a chinese pharmacist named hon lik for his father who was a heavy smoker with lung cancer . e - cigarettes were sold first in china in 2004 and later exported by the ruyan company and made available over the internet and more recently in retail establishments in europe and in the united states . e - cigarettes heat and vaporize a solution containing nicotine , and many are designed to look outwardly like traditional tobacco cigarettes ( figure 1 ) . thus , as a cigarette - like device that mimics both hand - to - mouth and oral - sensory experiences of a traditional cigarette , e - cigarettes have the potential to attract significant numbers of customers who might otherwise smoke cigarettes . there are no clinical guidelines that recommend the use of e - cigarettes for smoking cessation . electronic cigarettes have not been approved as a stop smoking treatment by the u.s . food and drug administration or any other government agency responsible for evaluating the safety and efficacy of drugs for smoking cessation . other countries , such as the united kingdom , are considering regulating e - cigarettes like medicinal nicotine products . although many smokers report using e - cigarettes to reduce or help them stop smoking , there is a paucity of reliable data on their efficacy for smoking cessation . although a recent study found that e - cigarettes with or without nicotine were about as effective as a nicotine patch in helping smokers abstain from using tobacco cigarettes , there are no published studies evaluating the safety and efficacy of e - cigarettes for smoking cessation in patients with chronic obstructive pulmonary disease ( copd ) or cancer . e - cigarettes deliver heated nicotine aerosol with a few other chemicals , so compared with smoking cigarettes there is exposure to many fewer inhaled chemicals . for patients with a serious lung ailment , it is reasonable to be cautious about recommending the use of any treatment that involves inhaling foreign material into the airways . it is likely to be many years before the harms ( if any ) associated with the acute and long - term exposure to e - cigarettes can be more completely ascertained . there needs to be an urgency to get cancer patients to stop smoking because the adverse effects of continued smoking can be immediate and severe . second , tell your patients that you are willing to work with them to overcome their nicotine dependence . the treatment guidelines for tobacco dependence are a good starting point to identify evidence - based options for smoking cessation , although you can acknowledge that current treatment approaches for nicotine dependence are only minimally effective . third , explain to your patient that the safety and effectiveness of e - cigarettes are not fully understood , nor is there any evidence to suggest that e - cigarettes are safer or more effective than existing government - approved stopping smoking medications . table 1 provides some clinical scenarios and suggestions on when e - cigarettes might or might not be considered as an adjunct to established nicotine dependence treatments . clinical scenarios and suggested responses for patients in summary , cancer patients deserve treatment guidance from their doctor . those who smoke should be advised to stop smoking and informed of the evidence - based treatment methods that have been shown to increase cessation outcomes compared to unassisted quitting . most smokers believe that they ought to be able to quit on their own without assistance and are skeptical about the value of current treatment approaches for nicotine dependence . however , given the importance of smoking cessation for cancer patients , oncologists should be insistent in their efforts to assist their patients to stop smoking including considering combination therapies . there are currently no evidence - based guidelines to support the recommendation of e - cigarettes . whereas evidence - based cessation strategies should be used wherever possible , clinicians should consider the strong need for cancer patients to stop smoking as soon as possible to promote the most effective outcomes of cancer therapy . in the absence of sufficient evidence that e - cigarettes are effective and safe for treating nicotine dependence in cancer patients , the international association for the study of lung cancer advises against recommending their use at this time . the international association for the study of lung cancer does recommend that research be done to evaluate the safety and efficacy of e - cigarettes as a cessation treatment in cancer patients to help guide clinical practice . for individual patients who are either using or planning to use e - cigarettes despite advice not to do so , they should be offered evidence - based stop smoking treatments while monitoring for any adverse effect of e - cigarette use .

the increasing popularity and availability of electronic cigarettes ( i.e. , e - cigarettes ) in many countries have promoted debate among health professionals as to what to recommend to their patients who might be struggling to stop smoking or asking about e - cigarettes . in the absence of evidence - based guidelines for using e - cigarettes for smoking cessation , some health professionals have urged caution about recommending them due to the limited evidence of their safety and efficacy , while others have argued that e - cigarettes are obviously a better alternative to continued cigarette smoking and should be encouraged . the leadership of the international association for the study of lung cancer asked the tobacco control and smoking cessation committee to formulate a statement on the use of e - cigarettes by cancer patients to help guide clinical practice . below is this statement , which we will update periodically as new evidence becomes available .

a 39-year - old female with bilateral development dysplasia of the hips , for which she had undergone bilateral hip replacements ( asr hip system , depuy orthopaedics , warsaw , in , usa ) 2 and 5 years ago , presented with a 3-week history of a paracentral scotoma in her left eye and bilateral ocular discomfort . she denied taking any medication and was not aware of any occupational exposure to toxic substances . the type of implant present in this patient has recently been recalled owing to wear - related toxic cobalt and chromium release and hazardous metallosis . on examination , . automated visual field testing ( humphrey 24 - 2 ) did not reveal any visual field defect . the patient proceeded to have a high - resolution optical coherence tomography ( oct ) scan , which showed several circumscribed lesions of the outer retina at the level of the retinal pigment epithelium ( rpe ) and the photoreceptors [ fig these patchy lesions were hypofluorescent on indocyanine green ( icg ) angiography [ fig . 2 ] but could not be demonstrated with fluorescein angiography . d. the oct shows discrete lesions with thinning of the photoreceptor - rpe complex ( arrows ) the icg angiography shows numerous hypofluorescent lesions in the affected eye electrophysiology testing was also performed . occipital pattern reversal visual evoked potentials were recorded for right and left eyes , monocularly using standard 35 checks and also with the very small 9 checks . cutaneous electroretinograms were recorded using a color dome ganzfeld stimulator and lower eyelid electrodes without pupil dilation . electroretinograms were well developed for both eyes with responses for the left were generally of slightly lower amplitudes in comparison to the right . pattern electroretinograms recorded using 140 check reversal stimulation ( 4/s ) showed equal and well developed responses for both eyes . the electro - oculograms were well developed with a well - defined dark trough and light peak . the arden index was satisfactory for the right eye ( 2.9 ) whereas borderline normal for the left eye ( 1.9 ) . laboratory testing showed elevated serum cobalt and chromium levels , 757 nmol / l ( 0 - 20 physician assessment did not reveal any signs of other system involvement and electrocardiograph and thyroid function tests were within normal limits . the diagnosis of toxic chorio - retinal degeneration presumably secondary to toxic serum levels of cobalt was made . given the lack of clinical progression , no intervention was considered necessary as yet . the patient continues to have regular follow - up with her family doctor , orthopedic surgeon , and ophthalmologist . the retina , one of the most metabolically active tissues in the body , requires a continuous oxygen supply to maintain optimal function . photoreceptor cells found in the outermost layers of the retina rely mainly on choroidal blood supply . hence , choroidal ischemia will lead to hypoxic injury of the outer retina and consequently photoreceptor degeneration . cobalt chloride is a hypoxia - mimicking compound , which exerts hypoxia - like effect on the local retina microenvironment . reported photoreceptor cell degeneration following intravitreal cobalt chloride injection in both rat and mouse retinas . . experimentally demonstrated choroidal vessel obliteration and alteration in the photoreceptor nucleus in rabbit retinas after intraperitoneal cobalt chloride injection . we postulate that toxic serum cobalt concentrations have a similar effect on the human retina and can cause retinal degeneration . in our patient , degenerative alterations of the photoreceptor - rpe complex were noted on oct . the icg angiography findings were suggestive of choroidal infarction . the distribution of the lesions noted on icg angiography corresponded with the oct findings and was consistent with patient 's paracentral scotomas . satisfactory visual evoked potentials and pattern electroretinograms indicated normal ganglion cells and optic nerve function . the electroretinograms showed a tendency toward slightly reduced amplitudes in left eye responses and the electro - oculograms findings suggested reduced rpe function in the left eye . mild rpe dysfunction in the left eye demonstrated by electrophysiology testings reinforced our clinical diagnosis . the asymmetric ocular manifestation may be explained by differences in choroidal perfusion , which can vary between eyes . we believe that this is the first case report of implant - related cobalt toxicity to the outer retina .

a 39-year - old female with elevated serum cobalt levels from her bilateral hip prostheses presented with a 3-week history of blurred vision in her left eye . optical coherence tomography revealed patchy degeneration of the photoreceptor - retinal pigment epithelium ( rpe ) complex . the lesions were hypofluorescent on indocyanine green angiography . we postulate that this is a case of implant - related chorio - retinal cobalt toxicity .

the method of ovarian cancer induction using lsl - k - raspten and lsl - k - rasptencd95 female mice was described previously 28 . briefly , mice were sedated , the right ovary was exposed and the ovarian bursa was injected with adcre ( 2.5 10 plaque - forming units ) ( university of iowa gene transfer vector core ) . mice were evaluated weekly for palpable tumour and all were sacrificed 8 or 14 weeks after the injection of the virus . at the time of sacrifice the primary tumour was excised , weighed , measured and the number of metastatic nodules and volume of ascites were recorded . all tissue was fixed in 10% formalin , embedded in paraffin , and stained with haematoxylin and eosin . , mice were anaesthetised with ketamine ( 100 mg / kg ) and xylazine ( 10 mg / kg ) i.p . , the liver was exposed through a midline incision , and the right and left lobes were sequentially ligated with a silk 4 - 0 suture , and resected . dna synthesis was measured by immunohistochemical staining of paraffin liver sections with anti - brdu antibody . the percentage of brdu positive hepatocyte nuclei among total hepatocyte nuclei was calculated using the cellular image analysis system ( acis , clarient , san juan capistrano , ca ) . 15 day old cd95 ( wt ) and cd95albumin - cre ( cd95 k.o . ) male mice were injected with a single dose of den ( 25 g / g body weight , i.p . ) . parameters of total liver weight , number of liver surface nodules and maximum nodule diameters were recorded . full methods and associated references are available in the online version of the paper at www.nature.com/nature . the method of ovarian cancer induction using lsl - k - raspten and lsl - k - rasptencd95 female mice was described previously 28 . briefly , mice were sedated , the right ovary was exposed and the ovarian bursa was injected with adcre ( 2.5 10 plaque - forming units ) ( university of iowa gene transfer vector core ) . mice were evaluated weekly for palpable tumour and all were sacrificed 8 or 14 weeks after the injection of the virus . at the time of sacrifice the primary tumour was excised , weighed , measured and the number of metastatic nodules and volume of ascites were recorded . all tissue was fixed in 10% formalin , embedded in paraffin , and stained with haematoxylin and eosin . , mice were anaesthetised with ketamine ( 100 mg / kg ) and xylazine ( 10 mg / kg ) i.p . , the liver was exposed through a midline incision , and the right and left lobes were sequentially ligated with a silk 4 - 0 suture , and resected . dna synthesis was measured by immunohistochemical staining of paraffin liver sections with anti - brdu antibody . the percentage of brdu positive hepatocyte nuclei among total hepatocyte nuclei was calculated using the cellular image analysis system ( acis , clarient , san juan capistrano , ca ) . 15 day old cd95 ( wt ) and cd95albumin - cre ( cd95 k.o . ) male mice were injected with a single dose of den ( 25 g / g body weight , i.p . ) . parameters of total liver weight , number of liver surface nodules and maximum nodule diameters were recorded . full methods and associated references are available in the online version of the paper at www.nature.com/nature .

cd95 ( also called fas and apo-1 ) is a prototypical death receptor that regulates tissue homeostasis mainly in the immune system through induction of apoptosis 1 - 3 . during cancer progression cd95 is frequently downregulated or cells are rendered apoptosis resistant 4,5 raising the possibility that loss of cd95 is part of a mechanism for tumour evasion . however , complete loss of cd95 is rarely seen in human cancers 4 and many cancer cells express large quantities of cd95 and are highly sensitive to cd95 mediated apoptosis in vitro . furthermore , cancer patients frequently have elevated levels of the physiological ligand for cd95 , cd95l 6 . these data raise the intriguing possibility that cd95 could actually promote the growth of tumours through its nonapoptotic activities 7 . here we show that cancer cells in general , regardless of their cd95 apoptosis sensitivity , depend on constitutive activity of cd95 , stimulated by cancer - produced cd95l , for optimal growth . consistently , loss of cd95 in mouse models of ovarian cancer and liver cancer reduces cancer incidence as well as the size of the tumours . the tumorigenic activity of cd95 is mediated by a pathway involving jnk and c - jun . these results demonstrate that cd95 plays a growth promoting role during tumorigenesis and suggest that efforts to inhibit its activity rather than to enhance its activation should be considered during cancer therapy .

a 29-year - old man presented with eight - year history of vague pain in the right knee that aggravated when squatting . , there were negative findings on the lachman and pivot - shift tests and no swelling or tenderness along the joint line . the range of motion was full , with no loss of extension , but painful in extreme range of flexion . coronal and sagittal t1-weighted images demonstrated that the soft tissue mass was isointense to muscle and obscured the acl . t2-weighted sagittal magnetic resonance images showed heterogenous , intermediate to low signal intensity , which was slightly higher than that of skeletal muscle ( fig . 1 ) . there were no palpable mass - like lesions on his whole body and no family history of hyperlipidemia or xanthomatosis . laboratory examinations , including platelet count , prothrombin time , bleeding time and total cholesterol , were all within normal limits . arthroscopic examination showed normal findings of both meniscus , posterior cruciate ligament and articular cartilage . we were able to access the lesions by switching the posterolateral and posteromedial portal alternatively . it was round in shape , measuring about 20 mm 11 mm in diameter with a reddish - brown color ( fig . . a repeat performance of the lachman test and pivot - shift test showed a stable knee . the basic cellular composition of the tumor was well - defined polygonal mononuclear cells with a scanty , faintly eosinophilic cytoplasm . foci of xanthoma cells with foamy cytoplasm and vacuoles were present , accompanied by branching capillaries . multinucleated giant cells had abundant eosinophilic cytoplasm and contained eight or more nuclei ( fig . the patient had no further complaints following the operation , and there was no recurrence on 55-months of follow - up . the many synonyms of tenosynovial giant cell tumor reflect its clinicopathologic heterogeneity and historical differences . the various terms used to describe the tumor are : giant cell tumor of tendon sheath , nodular tenosynovitis , pigmented villonodular synovitis , fibroxanthoma and fibrous histiocytoma.4 ) the four clinicopathologic variants of tenosynovial giant cell tumor are localized , diffuse extra - articular , diffuse intra - articular ( diffuse pigmented villonodular synovitis ) , and malignant.1,5 ) the localized type of tenosynovial giant cell tumor is defined as a circumscribed lesion that is microscopically not infiltrating into the fat or skeletal muscle . the tumor most commonly occurs in the fingers and may be extra - articular , intra - articular or both.5 ) less common is its occurrence in hands and wrists , toes , feet , ankles , knees and rarely in the hip . this tumor is usually diagnosed in young to middle - aged adults between the ages of twenty to fifty , with a female predominance . the tumor typically presents as a slowly growing , painless mass of size between 0.5 to 4.0 cm . lesions of the knee are usually intra - articular and may cause pain and joint effusion . following local excision , the digital tumors have some tendency to recur at 10%-20% , but the intra - articular tumors in the knee joint recur with a greater frequency.6 ) we have found in the literature only one case of localized tenosynovial giant cell tumor that arose from the acl of the knee.3 ) in this case report , we described a histopathologically proven giant cell tumor of the tendon sheath arising from the acl , which was diagnosed by magnetic resonance imaging . the patient had no further complaints following the operation , and there was no recurrence at 55-months of long - term follow - up .

the localized type of tenosynovial giant cell tumor usually occurs on the palmar side of fingers and toes . tenosynovial giant cell tumors of the tendon sheath are rarely intra - articular . we report a giant cell tumor of the tendon sheath arising from femoral attachment of the anterior cruciate ligament and its treatment with arthroscopy in a 28-year - old man .

to a dry 100 ml schlenk flask equipped with a stir bar was added pd(ch3cn)2(ots)2 ( 15.9 mg , 0.0300 mmol , 6.00 mol% ) , cu(otf)2 ( 5.43 mg , 0.0150 mmol , 3.0 mol% ) , ligand ( 12.3 mg , 0.0450 mmol , 9.0 mol% ) , 3 ms ( 75.0 mg , 150 mg / mmol ) , and dmf ( 8 ml ) . to this flask , a three - way adapter fitted with a balloon of o2 was added , and the flask was evacuated via house vacuum and refilled with o2 three times while stirring . the resulting mixture was stirred for 10 min . to this , a dmf solution ( 2 ml ) of the alkenyl alcohol ( 0.5 mmol ) and corresponding boronic acid ( 1.5 mmol , 3 equiv ) was added via syringe . the resulting mixture was stirred for 24 h at room temperature . the mixture was diluted with diethyl ether ( 200 ml ) and water ( 50 ml ) . the combined organic layers were washed with water ( 3 20 ml ) , brine ( 1 20 ml ) , and dried over sodium sulfate . the organic extracts were concentrated under reduced pressure , and the resulting residue was purified by silica gel flash chromatography using 210% etoac in hexanes containing 0.1% triethylamine to yield an aldehyde product . full experimental details and characterization of new compounds can be found in the supplementary information . to a dry 100 ml schlenk flask equipped with a stir bar was added pd(ch3cn)2(ots)2 ( 15.9 mg , 0.0300 mmol , 6.00 mol% ) , cu(otf)2 ( 5.43 mg , 0.0150 mmol , 3.0 mol% ) , ligand ( 12.3 mg , 0.0450 mmol , 9.0 mol% ) , 3 ms ( 75.0 mg , 150 mg / mmol ) , and dmf ( 8 ml ) . to this flask , a three - way adapter fitted with a balloon of o2 was added , and the flask was evacuated via house vacuum and refilled with o2 three times while stirring . the resulting mixture was stirred for 10 min . to this , a dmf solution ( 2 ml ) of the alkenyl alcohol ( 0.5 mmol ) and corresponding boronic acid ( 1.5 mmol , 3 equiv ) was added via syringe . the resulting mixture was stirred for 24 h at room temperature . the mixture was diluted with diethyl ether ( 200 ml ) and water ( 50 ml ) . the combined organic layers were washed with water ( 3 20 ml ) , brine ( 1 20 ml ) , and dried over sodium sulfate . the organic extracts were concentrated under reduced pressure , and the resulting residue was purified by silica gel flash chromatography using 210% etoac in hexanes containing 0.1% triethylamine to yield an aldehyde product . full experimental details and characterization of new compounds can be found in the supplementary information .

summarymolecules containing all - carbon quaternary stereocenters carbon atoms bonded to four distinct carbon substituents are prevalent in nature . however , the construction of such compounds in an enantioselective fashion remains a long - standing challenge to synthetic organic chemists . in particular , methods for forging quaternary stereocenters that are remote from other functional groups are underdeveloped . herein we report a catalytic and enantioselective intermolecular heck - type reaction of trisubstituted - alkenyl alcohols with aryl boronic acids . the reported method allows direct access to quaternary all - carbon - substituted - , - , - , - or aryl carbonyl compounds , as the unsaturation of the alkene is relayed to the alcohol resulting in the formation of a carbonyl group . the scope of the process also includes incorporation of pre - existing stereocenters along the alkyl chain , which links the alkene and the alcohol , wherein the stereocenter is preserved . the described method is flexible , allowing access to diverse building blocks containing an enantiomerically enriched , quaternary center .

in the previous issue of critical care , seligman and coworkers showed that copeptin , the c - terminal part of the vasopressin prohormone , increased continuously with the severity of sepsis . copeptin remained the only accurate prognostic marker for mortality in patients with ventilator - associated pneumonia . procalcitonin and the pao2/fio2 ratio , shown to be prognostic in ventilator - associated pneumonia , unfortunately were not included in the analysis . nevertheless , the authors conclude that copeptin could be useful in risk - stratifying patients with ventilator - associated pneumonia and may provide an early indication of treatment failure . the severity of a disease influences the consumption of costly healthcare resources , including the need for intensive care admission and the suitability for discharge , among others . an early and adequate prognosis and risk assessment facilitates an optimized care of patients with severe infections and other compromising diseases . in this context , there is a potential need for readily measurable biomarkers to predict disease severity and , finally , outcome . the advantage of biomarkers is that they are rapidly and easily available and are not investigator dependent . copeptin is cosynthesized with vasopressin , also known as antidiuretic hormone , thereby directly mirroring vasopressin levels but copeptin is more stable in plasma and serum . vasopressin not only has hemodynamic and osmoregulatory effects but also reflects the individual stress level . copeptin shows identical changes during disordered water states as previously shown for vasopressin , and directly correlates with plasma vasopressin levels in healthy volunteers and critically ill patients . in the past 2 years copeptin has been studied as a diagnostic marker and as a prognostic marker in different diseases . as a diagnostic marker , copeptin was evaluated in patients with diabetes insipidus for example , after pituitary surgery . in these patients copeptin had a superior diagnostic accuracy to detect an insufficient activity of the posterior pituitary , offering an alternative to the laborious and ambiguous water - deprivation test . as a prognostic marker , copeptin levels were independent predictors of survival in critically ill patients suffering from hemorrhagic and septic shock . in lower respiratory tract infections , the copeptin levels were significantly higher as compared with control individuals , with the highest levels in patients with community - acquired pneumonia . copeptin levels increased with increasing severity of pneumonia , as classified by the pneumonia severity index . similarly , in patients with acute exacerbations of chronic obstructive pulmonary disease , copeptin was shown to be predictive of long - term clinical failure independent of age , comorbidity , hypoxemia and lung functional impairment in multivariate analysis . copeptin levels also have prognostic implications in diseases other than infections . in patients with destabilized heart failure in postacute myocardial infarction cases , copeptin was elevated in patients who died compared with survivors . copeptin was thereby a significant independent predictor of death or heart failure within 60 days . vasopressin , together with corticotropin - releasing hormone , is the main secretagogue of the hypothalamo serum cortisol levels have been reported to be proportionate to the degree of stress and , by mirroring the individual stress level , to predict outcome in sepsis and pneumonia . importantly , copeptin levels seem to mirror even more subtly moderate levels of stress than cortisol levels . for the critical care clinician , this analysis could be particularly helpful in patients where knowledge of endogenous vasopressin , mirrored by copeptin concentrations , is crucial for therapy , such as in patients with prolonged hypotension and ongoing vasopressor drug requirements or in patients with electrolyte disturbances . of course , any biomarkers will always oversimplify the interpretation of important variables , and therefore biomarkers are meant to complement , rather than to supersede , the judgment of clinicians and/or validated clinical severity scores . conceptually , the likelihood of an adverse outcome should determine the medical indication , the length of hospitalization and the allocation of healthcare resources . it is time to perform intervention studies using biomarkers such as copeptin in specific settings to guide the allocation of hospital resources , including the need for intensive care admission and duration to ultimately prove their clinical usefulness and cost - efficiency .

the study conducted by seligman and coworkers included in the previous issue of critical care demonstrates that copeptin is a promising marker to predict outcome in patients with ventilator - associated pneumonia . in recent years , copeptin has emerged as a new prognostic marker in a variety of diseases , such as sepsis , community - acquired pneumonia , chronic obstructive pulmonary failure , heart failure and myocardial infarction . what is the pathophysiological basis for these findings ? copeptin together with vasopressin is co - secreted from the posterior pituitary and therefore mirrors the amount of vasopressin in the circulation . vaso - pressin is a main secretagogue of the hypothalamo pituitary adrenal axis , thereby mirroring the individual stress level . furthermore , vasopressin is an important hormone in salt and volume regulation . in this context , copeptin is also a diagnostic marker in patients with diabetes insipidus and in patients with disordered water states .

laser - assisted intrastromal keratomileusis ( lasik ) and photorefractive keratectomy ( prk ) may be used in accommodative esotropia either in adolescent or in adult patients . some authors have shown that lasik is safe , effective , and stable at 10 years follow - up post - surgery . a 14-year - old boy was admitted to our outpatient department on 08 - 01 - 2008 . together with his parents he asked for both eyes to be operated on by myopic prk . on september 5 2008 a myopic prk was performed on his right eye and on september 12 2008 on his left eye . a bausch & lomb z172 excimer laser was used to perform a planoscan procedure on both eyes . uncorrected visual acuity was 1.0 after both procedures . the patient did not suffer pain , haze or glare in postoperative follow - up . apart from the surgical technique , the author raises questions about the ethics of this procedure in a young boy . laser - assisted intrastromal keratomileusis ( lasik ) was introduced a few years later in 1999 . in this moment lindstrom wrote an editorial perspective on the 2009 october issue of ocular surgery news . in the same issue , a cover story raises questions about refractive surgery ( prk , the use of mitomycin c , lasik , lasek or phakic iols ) in paediatric patients . good vision correction results have also been shown with surgically implanted intraocular lenses , also called phakic iols . some authors have shown that paediatric lasik is safe , effective and stable at 10 years follow - up . lasik and prk may be used in refractive accommodative esotropia[612 ] either in adolescent or in adult patients . refractive surgery may be an alternative to contact lens wear and may reduce long - term costs . however , paediatric eyes may still be developing , and there is a theoretical risk of rapid myopic progression and shift in patients performing tasks that require near vision , such as schoolwork . another risk is to operate on eyes with unrecognized keratoconus . the aim of this report is to describe a case of a 14-year - old boy , operated on with prk on both eyes , and to evaluate this procedure both on surgical technique and on its ethical implications . on august 1 2008 , a 14 years old boy was admitted to the outpatient department . systemic and ophthalmological anamnesis was negative . on his ophthalmological assessment , re ucva 0.5 bcva 1.50 sf 1.0 p1 and le ucva 0.5 bcva cycloplegic refraction was exactly the same as the subjective refraction . on september 5 2008 , the boy 's corneal central pachymetry was 476 m ( right eye ) and 492 m ( left eye ) . keratometric indexes in both eyes a myopic prk was performed with planoscan . in the right eye , a 42 m ablation of the corneal stroma by 1157 pulses was performed using a bausch & lomb z172 excimer laser . after topical anaesthesia by lydocaine in the right eye , a corneal epithelium abrasion was performed by a smooth spatula . the patient did not have any pain , haze or glare , and received topical 1% omatropine , 0.3% of loxacin and nsaid drops as postoperative therapy . an ophthalmological assessment was performed at days 1 , 7 , 14 , 21 , 30 , 180 and 360 post - operation . one week after the procedure on the right eye , the same surgical procedure was performed on the left eye . post - operative medical therapy and follow - up were the same as in the right eye . no local or systemic adverse effects , apart from mydriasis due to 1% omatropine drops , were seen . this case report raises certain questions . from the surgical technique point of view , a skilled refractive surgeon is able to perform such a procedure . results were quite excellent both from a subjective and objective point of view . from the ethical point of view , we must ask if , even after written informed consent and central corneal thickness data , the chance of an increase in myopic shift during the next years of life indicates that this type of laser procedure should be performed . data from international literature stress that paediatric lasik is safe , effective and stable at 10 years post - procedure . some authors[911 ] stress that paediatric refractive surgery is useful in high unilateral myopia and/or anisometropic amblyopia . in any case i agree with lindstrom 's thought that we need a carefully planned clinical trial , approved by an institutional review board ( 1 ) . it is essential to know if the written informed consent , signed by both parents , will be sufficient for the surgeon in case of surgical failure .

context : refractive surgery has been performed for more than 10 years . laser - assisted intrastromal keratomileusis ( lasik ) and photorefractive keratectomy ( prk ) may be used in accommodative esotropia either in adolescent or in adult patients . some authors have shown that lasik is safe , effective , and stable at 10 years follow - up post-surgery.case report : a 14-year - old boy was admitted to our outpatient department on 08 - 01 - 2008 . together with his parents he asked for both eyes to be operated on by myopic prk . on september 5th 2008 a myopic prk was performed on his right eye and on september 12th 2008 on his left eye . a bausch & lomb z172 excimer laser was used to perform a planoscan procedure on both eyes . uncorrected visual acuity was 1.0 after both procedures . the patient did not suffer pain , haze or glare in postoperative follow-up.conclusion:apart from the surgical technique , the author raises questions about the ethics of this procedure in a young boy .

bladder - drained pancreatic recipients experience a relatively high incidence of metabolic and urologic complications resulting in conversion to enteric drainage in about 1530% of cases . spontaneous rupture of the bladder after enteric conversion of a bladder - drained pancreatic transplant has not been previously reported . a 44-year - old man with diabetes mellitus type 1 and end - stage renal failure receiving haemodialysis for four years received a sequential pancreatic transplant after renal transplant in march 2002 and july 2001 , respectively . the exocrine secretions of the transplanted pancreas drained into the urinary bladder . due to recurrent urinary tract infection and significant dysuria despite urinary alkalinisation , he underwent pancreatic duodenojejunostomy in june 2003 . in april 2006 , he presented to the emergency department with generalised abdominal pain associated with non - bilious vomits . the creatinine had increased to 286 mol / l from 170 mol / l and the white cell count was 20 10/l . empirical therapy with intravenous antibiotics and fluids was administered after an indwelling urinary catheter had been inserted at presentation . clinical and biochemical improvement once again occurred quickly and an outpatient colonoscopy and barium swallow returned normal findings . in december 2006 , he presented for a third occasion with abdominal pain and vomiting to the renal outpatients department . the previous evening , he had been lifting a sack of potatoes and experienced some discomfort in his perineum . on examination a diagnostic abdominal paracentesis was performed and the creatinine in the ascitic fluid proved to be very similar to serum ( 501 mol / l compared to 490 a urinary catheter was inserted into the bladder with a rapid improvement in the serum creatinine and a reduction in the volume of ascites . 1 ) revealed leakage of contrast into the peritoneal cavity . in the operating theatre , an omental patch was mobilised from the transverse mesocolon and placed over the dome of the bladder ( fig . a cystogram repeated two months later did not reveal any extravasation of contrast into the peritoneal cavity . bladder - drained pancreatic transplants have been associated with complications such as urinary infection and dysuria which can lead to a requirement for enteric drainage . however , bladder rupture after conversion from bladder to enteric drainage has so far not been reported in the literature . this occurred in our case three years after enteric conversion , and the diagnosis was not established until the third acute presentation when a urinary catheter was not initially placed . in our opinion , early urinary catheterisation with the first two presentations allowed the bladder to rest at low volume and resulted in a temporary repair . with the final presentation , the urinary effluent escaped from the bladder directly into the peritoneal cavity resulting in a reverse peritoneal dialysis with recycling or reabsorption of creatinine and a rapid increase in serum creatinine . urinary bladder rupture is a rare condition but one in which a pathological basis can be identified in most cases [ 2 , 3 ] . tarrass et al . identified 23 cases of renal failure following bladder rupture and these were mainly due to intrapelvic gynaecological operations , eosinophilic cystitis , blunt trauma to the abdomen , anatomical outflow obstruction and in association with indwelling catheters [ 5 , 6 , 7 , 8 , 9 , 10 ] . the clinical presentation can be variable but often includes oliguria , acute kidney injury and ascites with or without peritonitis . early diagnosis is important as the condition can be associated with high mortality . whilst routine imaging techniques allow a diagnosis of bladder rupture to be made in the majority of cases , its rare nature demands a high level of clinical suspicion where this constellation of clinical features exists . for a definitive diagnosis , conventional cystography is the gold standard ( 95% sensitivity ) although ct cystograms are considered of equal value . intravenous urography , duplex sonography , cystoscopy and laparoscopy have also led to the diagnosis in some cases . the management of bladder rupture depends on the site of the rupture . for extraperitoneal rupture , bladder catheterisation alone has been considered safe and effective , but for intraperitoneal bladder rupture , open repair with an omental patch is favoured and this is especially the case with recurrent bladder rupture .

complications associated with bladder - drained pancreatic transplant are not uncommon and include urinary tract infections and reflux pancreatitis . bladder rupture with peritoneal leak is a rare complication after pancreatic transplantation and can present as an acute abdomen with rapidly deteriorating renal function . we describe the first case of a urine leak into the peritoneal cavity occurring after conversion from bladder to enteric drainage . a high index of suspicion is required to diagnose such a complication .

alopecia areata ( aa ) is a chronic inflammatory disease that develops in hair follicles and rarely in the nail plate . studies suggesting a relationship between aa and psychological stress have been frequently reported ; these studies included both animal experiments and clinical research [ 2 , 3 , 4 ] . the autoimmune theory that aa is caused by t cells identifying hair follicles as autoantigens is considered highly plausible [ 5 , 6 ] . however , to date , no study has clinically proven this relationship . we treated the present pediatric case of aa that developed as a result of the divorce of the patient 's parents and the remarriage of her mother . we describe the transactional analysis approach to aa , which is a type of psychotherapy and appears to have completely cured aa in this patient . her parents divorced 6 years previously . since then , circular bald patches appeared on her head ; these patches exhibited repeated new growth and hair loss . she received treatment for this condition at her local hospital . since then , her hair loss became acutely exacerbated ; therefore , she was examined at her local hospital once again . she underwent treatment with externally and internally administered corticosteroids in addition to liquid nitrogen therapy . this treatment did not completely cure her condition , and she was thus examined at our hospital . peripheral blood and biochemical tests revealed no aberrations , and autoantibodies and thyroid hormones were within the reference ranges . the results of psychological testing were as follows : self - rating depression scale 47 points , state - trait anxiety inventory , state anxiety 48 points ( high ) , and state - trait anxiety inventory , trait anxiety 63 points ( extremely high ) . among the results of the self grow - up egogram ( sge ) , the score on the nurturing parent ( np ) subscale was the highest and that on the free child ( fc ) subscale was the lowest ( fig . these data suggest the basic interpersonal relationship stance of i am not ok , you are ok. unconditional positive affirmations , such as you are important to us even if you lose your hair , were repeatedly provided . in particular , when the patient underwent outpatient examinations , she was advised to speak about at least one interesting episode that had occurred in her daily life . six months later , new hair growth was evident when she gained the ability to proactively talk about herself . there has been no recurrence of any bald patches during 5 years since her initial examination ( fig . 3 ) . while speaking about her feelings during her illness , she said , when i was experiencing repeated hair loss , i could n't forgive myself for losing my hair . the severity of aa on initial examination is considered a strong long - term prognostic factor . the recovery rate of patients with an area of hair loss covering 50% of their head is 8% . cases of intractable aa , such as ours , are treated with systemic administration of steroids and regional immunotherapy , but treatment is often unsuccessful . one report shows that in a mouse model of aa caused by stress an antidepressant drug , tianeptine , is effective . our patient experienced negative life events ( divorce of her parents and her mother 's remarriage ) when aa onset and exacerbation occurred ; therefore , it is likely that psychosocial factors were involved . we believed that there would be serious risks involved in prescribing antidepressants to an adolescent and therefore decided to attempt the transactional analysis approach . transactional analysis is a psychological theory proposed by eric berne . according to this theory , each person is made up of three ego states ; parent , adult , and child . in the egogram , the parent alter ego is divided into the nurturing and critical elements , and the child alter ego is divided into the adapted and free elements , thus forming the five functional ego states : critical parent ( cp ) , np , adult ( a ) , fc and adapted child ( ac ) . sge was developed by the japanese researcher ( t. katsura ) as a standardized method for assessing personality . sge consists of 50 statements that can be categorized into the five functional ego states . respondents indicate the degree of their agreements with each statement using a three - point scale : agree , disagree and neither agree nor disagree , the scores for which are 2 , 0 and 1 , respectively . i am not ok , you are ok to i am ok , you are ok by raising the fc state . this change helped her to accept herself with her hair loss , and this acceptance appears to have reduced the stress . we believe that this method could also be easily used by dermatologists and may uncover a common language between the caregiver and the patient .

the patient was a 13-year - old female . six years previously , she developed alopecia areata when her parents divorced . one year after that , the bald area drastically expanded when her mother remarried . she was treated at her local hospital ; however , no improvement was observed . she then visited our hospital for examination . a bald patch was covering > 80% of her head . self grow - up egogram indicated the basic interpersonal relationship stance of i am not ok , you are ok. we therefore implemented a transactional analysis approach to increase the patient 's score on the free child subscale . new hair growth was observed after 6 months and the bald patch disappeared after 2 years . our results suggest that this method could also be easily applied in a clinical setting by dermatologists .

the previous issue of critical care contains a report associating more severe anaemia with worse outcome after intracerebral haemorrhage . these data are analogous to published reports in subarachnoid hemorrhage and traumatic brain injury that link more severe anaemia with worse outcomes . the traditional goal for packed red blood cell ( prbc ) transfusion was traditionally 10 g / dl , and revised down to < 7 g / dl with the exception of acute coronary syndromes or acute resuscitation . unfortunately , there have been no large , prospective trials of prbc transfusion in patients specifically with neurologic disease . anaemia is associated with worse outcomes in nontraumatic subarachnoid hemorrhage ( ruptured brain aneurysm ) . preventing brain hypoxia might be important to reduce the incidence and severity of cerebral infarction from vasospasm , and prbc transfusion in that setting leads to improved markers of brain tissue function on positron emission tomography . in patients with traumatic brain injury , brain oxygen monitors may show low brain oxygen tension that responds to prbc trans fusion ; the boost2 study is planned to assess if brain oxygen tension - guided therapy improves outcomes . a sub - study of the transfusion requirements in the critical care trial found no apparent effect of goal haemoglobin concentration on functional outcomes after neurotrauma . intracerebral haemorrhage does not lead to vasospasm , but cerebral infarction can be found on magnetic resonance imaging scans and this may impact outcomes . there is probably not hypoxia around the clot , but there may be altered metabolism for a period of several days . the nadir haemoglobin for patients with poor outcome ( 11.5 g / dl ) was above the usual trigger for transfusion , so these data are of limited usefulness in determining when a prbc transfusion should be given . what one should think of anaemia in the neurologically critically ill is likely to depend on one 's preconceived notions . if you are convinced that anaemia in the neuro - icu is linked to worse neuronal function , cerebral ischemia and poor outcome , you will probably ( successfully ) justify keeping your trigger for prbc transfusion at < 10 g / dl . if you are convinced these observational data simply show sicker patients have worse outcomes despite the statistical correction for older age and larger haemorrhage size , then you will probably ( successfully ) justify keeping your trigger for prbc transfusion at < 7 g / dl . if you have a specific physiologic trigger ( reduced brain oxygen tension , increased oxygen extraction fraction on positron emission tomography , and so on ) , few will argue with you . as benjamin franklin said , ' so convenient a thing it is to be a reasonable creature , since it enables one to find or make a reason for everything one has a mind to do . ' anaemia is generally associated with worse outcomes in neurologically critically ill patients . whether the outcome can be improved by more frequent use of prbc transfusion amn has received grant support for a prospective , randomized trial of goal haemoglobin in patients with subarachnoid hemorrhage from novonordisk and the neurocritical care society and the northwestern memorial foundation . amn has previously published on the topic of anaemia and subarachnoid hemorrhage , as cited in the article under discussion ; this may be perceived as a non - financial competing interest .

most healthy humans have a haemoglobin concentration of 12 to 15 g / dl and most intensivists now transfuse packed red blood cells for haemoglobin < 7 g / dl . higher haemoglobin is associated with improved intermediate and clinical outcomes after subarachnoid hemorrhage ( from ruptured brain aneurysm ) or neurotrauma . an observational study in a recent issue shows that higher haemoglobin was associated with better functional outcomes in patients with spontaneous intracerebral haemorrhage ; few patients received a packed red blood cell transfusion , so it is not known if that treatment is better than the disease . the mechanism of anaemia 's purported impact on outcome is unclear , although altered metabolism in brain tissue that is sensitive to reduced oxygen delivery is plausible . these data may intensify the differences of opinion between intensivists : whether neurologic patients are better served by higher haemoglobin and potentially by more packed red blood cell transfusion , or simply need to be studied more in prospective clinical trials , remains unclear .

five patients with b. capitatus infections were observed at 3 tertiary care hospitals in switzerland during june 2009june 2011 ( table 1 , table 2 ) . these hospitals provide services to a population of 3 million persons living in the temperate zone north of the alps in an area 5,000 km . a structured chart review was conducted for each patient , and further information was obtained from the involved infectious diseases specialist . the yeast was identified by using standard culture methods ( sabouraud dextrose agar with chloramphenicol and gentamicin and corn meal agar tween 80 ; both from oxoid , cambridge , uk ) and i d 32 c ( biomrieux , marcy letoile , france ) ( 13 ) . identification of isolates was confirmed by dna sequence analysis of the intergenic spacer ( its ) region ( primers its1 + its4 , genbank accession nos . hq014711.1 and hq014712.1 ) . for antifungal drug susceptibility testing , a microtiter broth dilution method based on the clinical and laboratory standards institute m27-a2 standard ( sensititre yeastone ; disk diffusion testing of fluconazole and voriconazole was performed as described ( 10 ) . a review of the microbiology laboratory records of the 5 swiss university centers yielded no additional isolations of b. capitatus in the past 5 years . * diagnosis is defined as the day of collection of first sample positive for b. capitatus . aml , acute myeloid leukemia ; capd , continuous ambulatory peritoneal dialysis ; cyca , cyclosporine a ; pdn , prednisone ; bal , bronchoalveolar lavage ; na , not applicable ; dta , deep tracheal aspiration ; l - amb , liposomal amphotericin b ; vrc , voriconazole ; flc , fluconazole ; amb , deoxycholate amphotericin b ; mof , multiorgan failure . * amb , deoxycholate amphotericin b ; 5-fc , 5-fluorocytosine ; flc , fluconazole ; itc , itraconazole ; vrc , voriconazole ; pos , posaconazole ; cas , caspofungin . by etest ( biomrieux , marcy letoile , france ) , the isolate had an amb mic of 3 g / ml . he became febrile , and pulmonary nodules and pustular skin lesions developed after induction chemotherapy . the patient was treated with amphotericin b and voriconazole , both of which had low mics . skin lesions improved within days , and pulmonary nodules resolved within weeks . eventually , relapsing leukemia proved refractory , and the patient died of multiorgan failure . patient 3 was a 58-year - old man receiving induction chemotherapy for acute myeloid leukemia , who was treated for polybacterial neutropenic sepsis . b. capitatus was isolated from blood cultures , urine , and tracheobronchial fluid while he was being treated . when a preliminary report indicated an amphotericin b mic > 2 g / ml ( etest ; biomrieux ) for the isolate , treatment was changed to fluconazole . autopsy findings confirmed extensive fungal pulmonary infiltrates with angioinvasion and multiple foci of b. capitatus infection in the liver , spleen , kidneys , bone marrow , myocardium , and brain . patient 4 was a 60-year - old man who was receiving continuous ambulatory peritoneal dialysis for renal failure 15 years after kidney transplantation for chronic glomerulonephritis ; he sought care for acute peritonitis . upon cultivation of yeasts in the dialysis fluid , two months later , b. capitatus was cultivated in urine while she was being treated empirically with caspofungin for possible fungal infection . emphysematous cystitis was detected by computed tomographic scan , and b. capitatus was cultured from urine collected from the right kidney . despite treatment with voriconazole , b. capitatus was cultured from urine for several weeks ; however , the patient recovered . the repeated isolation of b. capitatus in a temperate climate zone was unexpected because of the well - documented restriction of b. capitatus to areas with a mediterranean climate > 450 km south in italy , spain , and france . because of the high recovery rate of the organism from blood cultures of patients with disseminated infection and the ease of culturing b. capitatus on standard media , we believe it unlikely that a large number of cases could have been missed in the past . mean annual temperatures for the past 25 years were all above the mean for 19611990 and now exceed those of 1980 by > 1c . the rise in average temperatures was more pronounced during spring and summer months . since 1981 , average spring and summer temperatures in central switzerland have increased by 0.77c and 0.48c per decade , respectively ( 14 ) . the occurrence of 4 of the 5 infections reported here during the warm season indicates that the rising temperatures might have contributed to the expanded range of climatically restricted fungi to cooler areas and that the emergence of b. capitatus might be a consequence of the local effects of global warming ( 5,15 ) . alternatively , importation of the pathogen from areas to which it is endemic through increasing traffic of humans and goods might have led to establishment of a new endemic hotspot . our observation should alert clinicians caring for severely immunocompromised patients in temperate areas to consider infections caused by unfamiliar fungal pathogens , notably b. capitatus .

we report 5 cases of disseminated infection caused by blastoschizomyces capitatus yeast in central switzerland . the emergence of this yeast in an area in which it is not known to be endemic should alert clinicians caring for immunocompromised patients outside the mediterranean region to consider infections caused by unfamiliar fungal pathogens .

the prevalence of skin mycotic infections is changing with age , geographic location and climate ( 1 , 2 ) . despite regional characteristics and predispositions for dermatophyte infections , the epidemiology of dermatophyte infection has changed as a result of migration , lifestyle , drug therapy , and socioeconomic conditions ( 4 ) . the aim of our study was to analyze the appearance sites and frequency of the skin myocotic infections . we included patients visited in out- inpatient departments of the dermatology clinic of university clinical center of kosova during one calendar year . we studied the prevalence of mycosis involving 560 patients referred to the dermatology clinic of university clinical center of kosova during 2011 year . the study population included all ages with the youngest a newborn patient and the oldest patient over 75 years ; both genders , with slightly less presented males 46.5% . the study population included all ages with the youngest a newborn patient and the oldest patient over 75 years ; both genders , with slightly less presented males 46.5% . the mean age of our study group was 30.8217.69 with the oldest patient of 77 years . there was a predominance of women diagnosed with skin mycology changes 53.4% and also a predominance of patients from rural places 58.2% . basic characteristics of the study population . * except the average age and sd other values are presented by number and percentage the great percentage of referred patients had single site localization 94.8% whereas there were also those with multiple site of the disease localization , double ( 4.8% ) respectively triple ( 0.4% ) site . affected sites noticed at our study population we have analyzed also the sites that were affected by skin changes in particular in those who had more than one site and also whether the involvement of multiple sites were related to age , gender or living place . there was shown a very low correlation between age and single or multiple sites of localization while very low negative correlation was found between living place and single or multiple sites of localization too . the correlation between number of disease sites and some basic modalities the correlation between living place , age and sites of localization fungi are everywhere and no geographical area or any group of people is spared by these organisms ( 5 ) . however this study is very useful to determine the size of the problem , prevention of these infections and protocols of the treatment . in our study we found that the highest incidence of mycotic infections occur in females same as in other studies ( 6 , 7 ) . the increased prevalence of mycosis- skin manifestations as well as more frequent tinea pedis and tinea unguium deserves an improved approach toward these diseases . more frequent presence of skin mycotic infections in particular the multiple sites of their localization rises priorities of taking simple measures like education activities for whole population , special population categories and also activities for updating and upgrading health caregivers knowledge about diagnoses and treatment of these entities .

introduction : since the prevalence of skin mycotic infections is changing and is area depended we aimed to analyze the frequency of the skin myocotic infections and the appearance sites.material and methods : there were involved 560 patients referred to the dermatology clinic of university clinical center of kosova during a period of one year.results:the mean age of our study group was around thirties with a predominance of female and rural patients . although most of cases presented with single site disease localization , we observed the increase in number of cases with more than one site localization with age.conclusion:the increased prevalence skin mycotic infections , as well as more than one place of localization deserve a multidimensional approach .

the management of subfoveal wet armd with current modalities of treatment is an expensive deal . a few years back , the advent of photodynamic therapy with verteporfin initiated a fresh approach to the management of armd by stabilizing vision in selected cases ( mostly classic type , though indications were loosely expanded ) . the main advantage is that it is a noninvasive procedure ( besides the dye injection ) and though the cost seems exorbitant , several insurance companies and government agencies usually cover the costs required for treatment . despite reimbursement from various agencies , a large number of patients of wet armd are undergoing transpupillary thermotherapy ( ttt ) , often labeled as poor mans pdt ; its efficacy is questionable and it has lately been abandoned . in view of better understood angiogenesis , several anti - vegf drugs like pegaptanib sodium ( macugen ) , ranibizumab ( lucentis ) and bevacizumab ( avastin ) are available for use in the management of wet armd . due to their recent launch , limited data are available regarding their long - term outcomes and comparative studies are underway to determine the best treatment modality as monotherapy or in combination therapy . however , recent studies have shown promising outcomes11,12,13,14 and anti - vegf drugs may seem to be a popular treatment in the near future at least . secondly , multiple such treatments are required at four to six - week intervals for a period of two years . thirdly and most importantly , most of these drugs are too expensive for the general masses and are unaffordable in developing nations . the economic burden is huge due to the cumulative multiple injection costs , treatment for iatrogenic complications caused by these injections , hospital costs , surgeon visits , social care and rehabilitative services . two years of treatment with pegaptanib with approximately 20 six - weekly injections will cost rs . though both these drugs have shown promise and have been approved for treatment of armd , the high costs of total treatment limits their usage in the population at large . bevacizumab , an anti - vegf drug used in the treatment of metastatic colorectal cancer , is gaining popularity primarily due its comparable results and a cheap total cost of treatment . though it is still used " off - label " , a single dose of avastin would cost approximately rs . 2,000 and two years of treatment with avastin with approximately 20 six - weekly injections will cost only rs . the 60 + years age group is at risk for armd and constitutes 7.5% of the indian population ( 75 million).15 about one million of them will suffer from armd ( considering a 1.5% prevalence ) . wet armd will constitute about 10% of these cases ( 0.1 million ) and will require treatment . considering that about 18 to 22% of the indian population is below the poverty line , they can not afford these treatments . of the rest , indias per capita income is a mere $ 720 ( compared to $ 43740 of the united states)16 and most indians can not afford these treatments , unless costs are covered by insurance companies or sponsored by government agencies . considering that each dose of ranibizumab costs approximately 1200 , 10 doses in a year will cost 12000 and the total burden for an estimated 0.1 million patients of wet armd in india will be approximately 1.2 billion as drugs cost only . thus , such eyes often end up being treated with ttt , laser photocoagulation or no treatment at all leading to eventual blindness . research initiatives continue at a rapid pace by apex organizations and pharmaceutical companies worldwide to find a safe and effective treatment for armd . anti - vegf drugs have provided a ray of hope but involve the use of multiple intravitreal injections , which not only increase the risk of complications , but are expensive too . undergoing these expensive treatments in developing countries like though bevacizumab is still an off - label drug , promising results at a very cheap cost has prompted its use in a wide spectrum of ocular diseases.17,18 as research continues , very soon we may see newer and more effective agents offering treatment options for armd . the drug industry should not only review the entire pricing policy of these drugs in developing countries like india , but also look for affordable alternative compounds .

age - related macular degeneration ( armd ) is the most common cause for visual impairment in the elderly in western countries . recently several anti - vascular endothelial growth factor ( vegf ) drugs like pegaptanib sodium ( macugen ) , ranibizumab ( lucentis ) and bevacizumab ( avastin ) are available for use in the management of wet armd . a major limitation of these drugs is that they require multiple intravitreal injections , every 4 to 6 weeks interval for a period of 2 years . moreover , most of these drugs are too expensive for the general masses to afford in developing nations . avastin , though used " off - label " , offers a comparable result at affordable cost , however , long term results are awaited . the drug industry should review the entire pricing policy of these drugs in developing countries like india , and develop affordable alternative compounds . the article reviews the economic burden and affordability issues of these anti - vegf drugs in armd .

although it has been studied extensively , its pathogenesis remains largely unknown . in recent years , late preterm birth ( 3436 gestational weeks ) the placenta is a record of the fetal environment and its examination may provide information about fetal growth and subsequent development . i report the histopathology findings of a case of placental infarction associated with late preterm birth . the subject was a male neonate delivered at 34 weeks and 5 days of gestation with a birth weight of 2500 g. his mother had been diagnosed with threatened premature birth at 27 weeks of pregnancy . the child showed no apparent congenital abnormalities at birth and no noteworthy developmental problems appeared thereafter . the placenta was 19 cm in diameter and its weight was considered appropriate for gestational age ( no measurement data are available ) . , a white focal infarct was noted beneath the site of the umbilical cord insertion ( fig . there were small focal infarcts scattered within the marginal area , but these findings are generally not considered abnormal ( fig . 2a and b ) . figure 1:gross appearance of the formalin - fixed placenta , viewed from the fetal side ( a ) and the maternal side ( b ) . ( a and b ) focal infarct was detectable immediately beneath the site of umbilical cord insertion . gross appearance of the formalin - fixed placenta , viewed from the fetal side ( a ) and the maternal side ( b ) . ( a and b ) focal infarct was detectable immediately beneath the site of umbilical cord insertion . microscopy confirmed the presence of focal infarcts , but no massive fibrin depositions were seen ( fig . the placental villi were of appropriate maturity for gestational age and the villous vessels showed no structural abnormalities ( fig . no signs of funisitis or structural abnormalities were identified in the umbilical cord , which had two arteries and one vein ( fig . the villi were of appropriate maturity for gestational age and the villous vessels showed no structural abnormalities ( a ) . histopathology revealed no signs of chorioamnionitis ( b ) or funisitis ( c ) . the villi were of appropriate maturity for gestational age and the villous vessels showed no structural abnormalities ( a ) . a review of the available literature identified only a limited number of studies on placental histopathology findings related to threatened preterm birth . several studies found marginal insertion of the umbilical cord at the periphery of the placenta in association with intrauterine growth restriction and premature labor . the presence of infarcts regardless of size in the placenta of a preterm infant is an abnormal finding . therefore , the size of infarcts and the timing of their formation are likely to have important clinical implications . it is generally assumed that central infarcts are more important than peripheral ones , and there is an association between pregnancy - induced hypertension and multiple placental infarcts . in addition , microscopy revealed no particular vascular abnormalities in the placenta and no thrombus formation . overall , these findings suggest that one or more humeral factors might contribute to the pathogenesis of threatened preterm delivery . in particular , we speculate that some thrombogenic factors are involved in the formation of placental infarcts . the placenta reported here had focal infarct just beneath the umbilical cord insertion and this observation suggests that thrombosis possibly led to the premature labor through various unknown mechanisms . complement fraction c4d , on the other hand , is commonly referred to as an indicator of antibody - mediated tissue injury . the minimal expression of c4d in this case might give insight into placental infarction from the immunologic point of view . since not all placentas of preterm infants are investigated histopathologically , the presence of placental infarcts might go unnoticed for a considerable proportion of preterm children . all pathomorphologic changes ( pathomorphome ) in placentas including those of normal infants should be analyzed routinely . this case highlights the benefits of histopathology for postpartum placentas ( evidence of fetomaternal interface ) for both normal and preterm children to explore their possible causative significance in premature birth .

here the histopathology findings of placental infarction associated with late preterm birth are reported . the subject was a male neonate delivered at 34 weeks and 5 days of gestation with a birth weight of 2500 g. his mother had been diagnosed with a threatened premature birth at 27 weeks of pregnancy . the size and weight of the placenta was considered appropriate for gestational age . on the cut surface of the placenta , a white - colored focal infarct was noted beneath the site of the umbilical cord insertion . there were small focal infarcts scattered within the marginal area . there were no particular vascular abnormalities or apparent thrombi . the placental villi were of appropriate maturity for the gestational age and the villous vessels showed no structural abnormalities . this case highlights the benefits of examining the histopathology of postpartum placentas for preterm children to explore their significance in premature birth .

scintigraphic analysis of gastric motility is noninvasive , reproducible , simple to perform , accurate and quantitative . ever since the introduction of more than 30 years ago , gastric emptying scintigraphy has been significantly refined and optimized over the years . radionuclide gastric emptying studies are now well established as the standard method to evaluate gastric emptying . various methodologies have been used.1,2 meal composition , patient positioning , instrumentation , frequency of data acquisition , study length and quantitative methods all vary between institutions . in general , the patient is asked to fast overnight or for at least 4 hours prior to the study . diabetic patients should be studied in the morning , 20 - 30 minutes after their normal insulin dosage . a wide variety of drugs can affect gastric emptying and therefore most medication should be discontinued prior to a scintigraphic evaluation . generally , radiolabel egg albumen with tc-99 m sulfur colloid is used in most nuclear medicine laboratories . the eggs are mixed with 37 mbq tc-99 m sulfur colloid by frying or scrambling and the prepared egg product is then administered orally , often with toasted bread as an egg sandwich . labeling efficiency is approximately 85% . measuring simultaneous solid and liquid emptying is feasible using a dual isotope study by radiolabeling a liquid phase with in-111 dtpa ( indium-111 diethylenetriamine pentaacetic acid ) . a clear liquid study may be useful in a patient intolerant of solids but able to retain liquids.3,4 to evaluate liquid emptying , water is labeled with 3.7 mbq in-111 dtpa . the test meal should be ingested within 5 - 10 minutes , after which imaging commences . immediately after the completion of the meal , the patient is positioned in front of the camera . the study can be acquired on standing , sitting and supine . for accurate quantification , it is not important which method is used , except that the methodology should always be the same and normal values need to be derived and validated for that methodology . the stomach lies obliquely within the abdomen and as food moves from the relatively posterior fundus to the relatively anterior antrum , there is an apparent increase in anterior counts due to lesser depth . for accurate quantitation , images in both anterior and posterior projections are obtained with averaging while attenuation correction should be performed routinely . the standard method for attenuation correction is the geometric mean method ( square root of the product of anterior and posterior views ) . other work suggests that the use of a single left anterior oblique projection also minimizes geometric effects.5 in that projection , the stomach contents move roughly parallel to the head of gamma camera , thus minimizing the effect of attenuation . data acquisition is performed for 60 - 120 minutes either as continuous or intermittent imaging ( fig . 1 ) . in cases where there is prolonged retention of material within the stomach , even more delayed images up to 3 hours may be required . measurement of the half emptying time , or time required by the stomach to empty 50% of the ingested meal , is the simplest way to assess gastric transit . other protocols call for less frequent image acquisition at every 20 - 30 minutes for 2 - 3 hours ( fig . the normal values were 10% , 65% and 90% , at 1 , 2 and 4 hours , respectively.6 some data suggest that the longer imaging period of 4 hours provides a higher sensitivity for detection of abnormal emptying , compared to 2 hours.7 gastric emptying scintigraphy is the only satisfactory method of quantitatively measuring the rate of gastric emptying . but it is very important to establish normal ranges for the technique employed in each individual laboratory , as the results are highly dependent on the acquisition of parameters and the test meal used .

gastric emptying scintigraphy has long been the standard method for measuring gastric motility . various methodologies for this study have been used including meal composition , patient positioning , instrumentation , frequency of data acquisition , study length and quantitative method . for accurate quantification , it is not important which method is in use , except that the methodology should always be the same and normal values need to be derived and validated for that methodology .

bariatric surgery has become an integral part of morbid obesity treatment with well - defined indications [ 1 , 2 , 3 , 4 ] . it even appears as the sole treatment for long - term weight loss stability and regression of morbidities [ 5 , 6 , 7 , 8 , 9 , 10 , 11 ] . two types of bariatric surgery are available : restrictive ( laparoscopic adjustable gastric banding and laparoscopic sleeve gastrectomy ) and malabsorptive ( laparoscopic gastric bypass ) procedures . laparoscopic sleeve gastrectomy ( lsg ) is increasingly being recognised as a valid stand - alone procedure for the surgical management of morbid obesity . lsg is becoming more and more popular because this technique is more confortable and more efficient than laparoscopic adjustable gastric banding and causes less morbidity than bypass . during this intervention , two different tubes are used by the anaesthesiologists and introduced through the nose : a nasogastric tube and a calibration silastic tube . the calibration silastic tube , allowing the gastric section , is a full tube and is interesting for avoiding stapling of this tube or narrowing of the stomach tubulisation . some complications due to lsg procedure , specific or not , have recently been described [ 12 , 13 ] . we report a rare complication unpublished to date : a nasogastric section during great gastric curve stapling . a 44-year - old woman suffered from severe obesity ( bmi 36.6 kg / m2 , weight 88 kg , height 155 cm ) with failure of medical treatments for years . her obesity was accompanied by comorbidities , including diabetes mellitus type 2 and metabolic syndrome ncep - atp3 . she was thus eligible for bariatric surgery according to the has ( haute autorit de la sant ) guidelines . the gastric sleeve is created by using a linear stapler . according to already published technique [ 13 , 14 , 15 ] , the staples are applied alongside a 34-fr calibrating bougie positioned against the lesser gastric curve . the anaesthesiology team helps to correctly set up the bougie . before the insertion of the calibrating bougie , the pernasal nasogastric tube is partially removed ( 20 cm ) from the stomach and positioned in the cavum . once the bougie is withdrawn , a leak test is performed using intraoperative methylene blue dye through a pernasal nasogastric tube repositioned in the stomach . six hours postoperatively , when the patient had come back to the recovery room , the nurse removed the nasogastric tube according to the local protocol . in this case , the nasogastric tube was abnormally short and staples were present at its extremity ( fig . the patient did not present hyperthermia ( 37.7c ) , tachycardia ( 87 bpm ) , abdominal pain ( under analgesic drugs ) or dyspnoea , but she presented nausea according to the normal postoperative course . after discussion we decided , with the patient 's information , to perform an abdominal ct scan . the gastric wall was subsequently repaired by the surgeon and the methylene blue test was negative for staple line leak . lsg has recently emerged as a reproducible surgical technique effective for long - term weight loss stability with regression of most major associated comorbidities , such as diabetes type 2 . complications caused by this surgical technique vary from 0 to 10% , with a median estimate of 5.1% , according to studies . the major complication , by its frequency and its consequences , is gastric fistula , blind or draining into a nearby organ . this fistula is most often located at the upper end of the stapling line . to our knowledge , this is the first publication of nasogastric section during lsg . therefore we report this case and propose a solution to prevent its occurrence . during lsg , its extremity stays in the cavum and the calibrating bougie is introduced trough the mouth . therefore , in this case the calibrating bougie led the nasogastric tube to the stomach . to avoid this kind of accident , we now systematically insert the nasogastric tube by mouth through a guedel cannula . to insert the calibrating bougie when this situation occurs , surgery must be systematically associated with endoscopic removal of the remnant nasogastric tube : indeed , its ablation can create a staple line defect . the gastric wall is subsequently repaired by the surgeon and the methylene blue test is systematically performed in order to prevent staple line leak .

bariatric surgery has become an integral part of morbid obesity treatment with well - defined indications . some complications , specific or not , due to laparoscopic sleeve gastrectomy ( lsg ) procedure have recently been described . we report a rare complication unpublished to date : a nasogastric section during great gastric curve stapling . a 44-year - old woman suffered of severe obesity ( bmi 36.6 kg / m2 ) with failure of medical treatments for years . according to already published technique , a lsg was performed . six hours postoperatively , a nurse removed the nasogastric tube according to the local protocol and the nasogastric tube was abnormally short , with staples at its extremity . surgery was performed with peroperative endoscopy . in conclusion , this is the first publication of a nasogastric section during lsg . therefore we report this case and propose a solution to prevent its occurrence . to avoid this kind of accident , we now systematically insert the nasogastric tube by mouth through a guedel cannula . then , to insert the calibrating bougie , we entirely withdraw the nasogastric tube .

limy bile is a rare disorder in which the gallbladder ( gb ) is filled with a thick paste - like radiopaque material , which extends rarely into the common bile duct ( cbd).1,2 since churchman 's description of this syndrome in 1911 , more than 300 cases have been reported in the literature , including 7% with limy bile in both the gb and cbd.3 tsukamoto et al.4 reported 26 cases in which obstructive jaundice was accompanied by limy bile . in cases with biliary symptoms accompanying limy bile , laparoscopic cholecystectomy is a less invasive option than open surgery , and its safety has been established.5 nevertheless , patients still incur the risks associated with general anesthesia and longer hospital admission period . we experienced a case of limy bile with acute cholangitis and cholecystitis that was treated with endoscopy without surgical intervention . a 45-year - old female presented in the outpatient department with right upper quadrant pain , fever , and chills for 3 days duration . laboratory findings were as follows : white blood cell count , 13,200/mm ; total / direct bilirubin , 7.73/5.52 mg / dl ; gamma - glutamyl transferase , 797 iu / l ; alkaline phosphatase , 232 iu / l ; ast / alt , 348/759 iu / l ; and amylase / lipase , 49/32 iu / l . computed tomography showed high - density material in a dependent position of the gb and cbd . the patient was diagnosed with obstructive jaundice due to choledocholithiasis , which entails limy bile . a duodenoscopy revealed diffusely enlarged major papilla . an endoscopic retrograde cholangiography ( ercp ) revealed a 14-mm filling defect at the distal cbd . after an endoscopic sphincterectomy ( est ) , limy bile materials were washed out by sweeping with a balloon catheter ( fig . after then , clinical finding of the patient was improved dramatically , and surgical treatment such as a laparoscopic cholecystectomy was postponed . four days after the first ercp , we repeated the ercp and again found a filling defect in the distal cbd . after completely removing limy bile from the cbd , we inserted a guide wire into the gb through the cystic duct , and a 7 fr double - pigtail catheter ( length , 15 cm ) was introduced to the gb to evacuate limy bile ( fig . six months later , she was admitted for follow - up examination . during the follow up of six months , she was administered daily medication consisting of 600 mg of udca ( ursa ; daewoong , seoul , korea ) and 300 mg of a terpene preparation ( rowachol ; rowa pharma , cork , ireland ) . laboratory findings were within normal ranges , and follow - up studies , including a ct , showed the complete evacuation of limy bile from the biliary system and gb ( fig . limy bile is an uncommon condition in which the gallbladder and bile duct are filled with radiopaque material , readily noted on plain radiographs.1,2 since the first description of this syndrome in 1911 by churchman , more than 300 cases of limy bile have been reported.3 the presence of limy bile in the cbd is very rare , with only a few reported cases . thus , when obstructive jaundice occurs with limy bile syndrome , there are no definite treatment guidelines . the most frequently used treatment to date is cholecystectomy ; cholecystectomy is performed for limy bile in the gb , and cholecystectomy plus insertion of a t - tube into the cbd after removal of limy bile are performed for limy bile in the gb and the cbd.6 recently , laparoscopic cholecystectomy for focal limy bile in the gallbladder has been reported,5 and a combined application of est and laparoscopic cholecystectomy was used to treat limy bile in both the gb and cbd.7 in the present case , we performed est to treat the cholangitis caused by biliary lithiasis . in most cases of limy bile syndrome , however , in patients at increased surgical risk , endoscopic drainage such as endoscopic transpapillary gallbladder drainage ( etgd ) is an alternative treatment for acute cholecystitis.8,9 itoi et al.9 reported that among 36 acute cholecystitis patients who received etgd , 35 showed clinically favorable response without any serious procedure - related complications . given that limy bile is a movable precipitant of calcium carbonate combined with cholesterol,10 we postulated that etgd could be used to drain limy bile and performed etgd in this patient . six months after the procedure , we observed complete evacuation of limy bile from the gb . though surgery is the mainstay of the treatment for limy bile , endoscopic treatment such as est and/or etgd may be considered in selective patients , especially a high - risk candidate for operation .

limy bile is a relatively rare condition in which a radiopaque material is visible in the gallbladder , extending rarely into the bile duct , on plain radiography . acute cholangitis or cholecystitis caused by limy bile is a very rare condition . there are no definite treatment guidelines for limy bile , but in most cases with cholangitis or cholecystitis , laparoscopic cholecystectomy has been the preferred treatment . we report a case of limy bile with biliary symptoms that was treated only with an endoscopic procedure .

the first 2 modules discussed an introduction to family medicine and the general concepts of family medicine , followed by 4 modules on common problems in family medicine : chronic illnesses ; communicable diseases in adults and children ; neurological , musculoskeletal , and mental health diseases ; and gynecological diseases and obstetric care . 1 ) . the content and format were reviewed , edited , and approved by 23 family physicians . each module of the course consists of 5 lectures , 6 small group sessions , and 4 practice cases taught over 3 full days . the course was designed to give theoretical information and practical skills on some of the main concepts of family medicine , and included the definition and scope of family medicine , communication skills , consultation and referral skills , and the clinical practice of family medicine . each topic area starts with a lecture followed by group work , discussions , and other interactive learning activities . the small group learning sessions provided ample opportunity to give practical information that could be applied to patient care . the approximate division of the total learning time was as follows : 60% interactive small group sessions , 30% didactic lecturing , and 10% self - learning . the course was constructed to enable interactive learning with opportunities for peer learning , brainstorming , and small and large group discussions , as well as problem - based learning . the fame program was started in riyadh , the capital and the largest city in saudi arabia , and expanded to other areas with a vision to reach gps in all 13 regions of the kingdom of saudi arabia . during the course period , analyzing the pre - test and post - test scores for the first 53 physicians who completed the program in 2009 showed significant improvement in knowledge , with scores improving from a mean of 49% ( sd , 22% ) for the pre - test results to a mean of 89% ( sd , 13.5% ) for the post - test results on a paired t - test ( t=15.75 , p<0.001 ) . the teachers of the program attended focus group sessions in february 2012 to discuss the progress and future of the program . they discussed decentralizing the program ; investing in trainers and other resources such as support staff , librarians , system administrators , textbooks , up - to - date journals and internet access ; and implementing new teaching techniques with the conventional curriculum , such as practice - based small group learning and online learning . funding to modify the program according to changing needs , offering the program for a small nominal fee , and securing research funding to evaluate the program and to analyze system changes were stressed as important considerations for the sustainability of the program . cme has been shown not only to improve physician knowledge but also to result practice changes and patient outcomes . therefore , we believe that the impact of the program on patient and population health will be the more meaningful outcome , and are in the process of evaluating these factors . serious thought should be given to the creation of continuing medical education programs based on the health - care needs of the community to improve access to primary health care . we are hoping to measure the improvement of disease management and reduction of chronic disease complications , initially , to formulate measures of health and wellness for the community in the coming years .

the purpose of this study was to describe the development , implementation , and evaluation of an educational program in family medicine for general practitioners in saudi arabia from 2009 to 2011 . a continuing medical education program called family medicine education ( fame ) was developed with 7 modules each consisting of 12 - 14 hours of teaching to be delivered in 3 day blocks , over 45 days . twenty percent ( 2,761 ) of all general practitioners participated in the fame program . initial assessment of the program showed significant improvement of knowledge from scores of 49% on a pre - test to 89% on post - tests . fame program in saudi arabia facilitated primary care physicians knowledge .

idiopathic functional constipation is a clinical ailment that is difficult to manage . in general specific solutions for reducing idiopathic slow transit constipation ( istc ) include pharmacological therapy3 , biofeedback4 , and surgical treatment5 . although the effects of these treatments have been confirmed in previous studies , it may be difficult for most people to adhere to a regular pattern of medicine treatment and biofeedback exercise . surgery is also an unattractive option for most patients , and abdominal pain and bloating may continue6 . in some cases , the intervention of sacral nerve stimulation has been reported to be a successful way of treating urological function problems7 , 8 . to our knowledge , few studies have been conducted on the use of low - frequency current in managing constipation , and its efficacy is also controversial . recent studies on the effects of sacral nerve stimulation have claimed it is effective for patients with intractable constipation9 ; however , other studies failed to find evidence to support the effectiveness of the low - frequency current therapy in the treatment of constipation patients10 , 11 . therefore , the purpose of this study was to determine whether low - frequency current therapy can be used to reduce the symptoms of istc . this study was conducted in the city of andong , south korea , between may and july 2013 . fifteen patients ( ten male and five female ) were enrolled in the present study . the inclusion criteria required the subjects to be constipated with scores on the constipation assessment scale ( cas ) above 4 points12 , and an abnormal bowel frequency ( <3 bowel movements for a week)13 . exclusion criteria were : dementia , psychiatric problems , cancer , or scheduled for surgery . prior to the initiation of this study , all of the subjects were provided with a detailed description of the experimental procedures , and they signed a written and informed consent form in conformity with the ethical principles of the helsinki declaration . characteristics of participantssexage(years)weight(kg)exercise time(min / day)water amount(ml / day)meal frequency(times)dietexperiencelaxativeexperiencemale : 5female : 1021.602.5356.209.7926.3322.72570.84258.31two : 10three : 5yes : 8no : 7yes : 4no : 11 . low - frequency current was applied using transcutaneous electrical nerves stimulation through four electrodes ( tens ) ( chung woo , seoul , south korea ) . the current was set at a comfortable intensity , and a frequency of 50 hz with burst intervals of three seconds and six seconds were used . twenty minutes of electrical stimulation was given in each session , and electrical stimulation therapy was performed three times a week for six weeks . in this study , data were analyzed using spss 12.0 ( spss inc . the comparison before and after treatment was performed using the paired t - test . after the intervention the bowel movements per day and week , and the cst points were significantly improved compared to their respective values before the experiment ( table 2table 2 . comparison of constipation symptoms between pre- and post - testvariablepre test(0 weeks)post test(6 weeks)bowl movements per day*0.470.521.070.26bowl movements per week*2.470.645.271.44cas score*1.020.420.200.09*p<0.05 , meansd , cas : constipation assessment scale ) . this study was conducted to investigate whether the use of low - frequency current applied to the sacral dermatomes is helpful for istc . the findings of this study support the suggestion that low - frequency current therapy may be favorably used to diminish symptoms istc . electrical nerve stimulation of the sacral dermatomes has been demonstrated to increase the bowel activity of the recto - sigmoid colon , which is evidenced by the activation of the pelvic parasympathetic nerve originating from the sacral spinal cord14 . based on this , lee et al.15 suggested that magnetic simulation over the s2-s3 dermatomes of the buttocks would contribute to amelioration of istc . frost et al.14 also reported an increase in rectal contractile activities after electrical stimulation of the s2 dermatome . the results of our present study suggest that low - frequency current stimulation of the s2-s3 dermatome increases both defecation and cas scores , and they show that stimulation with low - frequency current of the sacral dermatomes may offer therapeutic benefits for a subset of patients with istc . however , this study had several limitations that should be addressed by further studies . a pre - post study is highly limited with regard to the inferences that can be drawn from it because of the nature of the design . further studies with randomized control trials are required , and the efficacy of the treatment needs to be established in a larger sample size .

[ purpose ] this study aimed to determine whether low - frequency current therapy can be used to reduce the symptoms of idiopathic slow transit constipation ( istc ) . [ subjects ] fifteen patients ( ten male and five female ) with idiopathic slow transit constipation were enrolled in the present study . [ results ] bowel movements per day , bowel movements per week , and constipation assessment scale scores significantly improved after low - frequency current simulation of s2-s3 . [ conclusion ] our results show that stimulation with low - frequency current of the sacral dermatomes may offer therapeutic benefits for a subject of patients with istc .

uniportal video - assisted thoracic surgery ( vats ) has become an important innovation in the field of thoracic surgery over the last decade . this may be secondary to the relatively low number of thoracic procedures performed in children , limited space in the thorax and the challenge of single - lung ventilation in this age group . we present a modification of the uniportal vats technique that uses a single incision with the thoracoscope in the superior intercostal space relative to the working instruments . a 14-year - old male with liver mass and bilateral lung nodules was referred to our institution . pulmonary lesions were concerning for metastatic disease and a lung nodule biopsy was required . following implementation of one - lung ventilation , the patient was placed in the lateral decubitus position . a 2.5 - 3 cm incision was made in the 5 intercostal space to accommodate the thoracoscopic instruments ; a 5 mm 30 endoscope was passed through the superior intercostal space relative to the working instruments [ figures 1 and 2 ] . a thoracostomy tube was then introduced through the superior muscular defect , where the endoscope had been and was brought out of the incision anteriorly . ( a ) thoracoscope ( 1 ) in the superior intercostal space with grasper ( 2 ) and stapler ( 3 ) in the inferior space . ( b ) diagram of the thoracoscope and instruments locations ( a ) long thoracoscope relative to working instruments avoids interaction between hands of the assistant and operating surgeon . single - port or uniportal vats was first described by lin et al . in 2002 for the treatment of palmar and axillary hyperhidrosis . in 2004 , rocco et al . , reported uniportal vats pulmonary wedge resection in 15 patients . in 2011 , gonzalez - rivas et al . , reported the first upper lobectomy performed by uniportal vats . this surgical group has since applied the uniportal vats technique to major resections , including complex cases and advanced tumours . based on their experience , several other groups have recently started their own programs using uniportal vats . while single - site laparoscopy in paediatric patients is becoming more common , with publications increasing in number , the same approach is not as well - established for the thorax . the first report of uniportal middle lobectomy in a paediatric patient , for the treatment of a pulmonary aspergilloma , was recently published . lack of experience with uniportal thoracic procedures in paediatric surgery is secondary to fewer pathologic processes requiring surgical intervention relative to those affecting adults , namely cancer . limited space in the thorax of these young patients makes uniportal vats difficult to perform . single lung ventilation in neonatal and paediatric patients is also challenging , which may represent an important barrier for the implementation of uniportal vats . advantages associated with uniportal vats include parallel instrumentation that mimics the manoeuvres performed during open surgery , less incisional pain and paraesthesia and improved cosmesis . we present a modification to uniportal vats , using a single skin incision , but placing the thoracoscope in the superior intercostal space relative to the working instruments . this allows increased instrument range of motion within a single intercostal space [ figure 1 ] . furthermore , use of a long thoracoscope allows the hands of an assistant to be remote relative to those of the operating surgeon [ figure 2 ] . the scope intercostal incision can be utilised as an exit site for the thoracostomy tube which permits a better closure of the larger intercostal incision . furthermore , tissue trauma may be greater , since multiple instruments are being passed through a limited space . , we describe a modification to uniportal vats that may help facilitate operations in children by increasing degrees of freedom with regard to the range of motion . further development of instruments scaled to size , such as endoscopic staplers , will expand opportunities for utilisation of uniportal vats in small children .

video - assisted thoracic surgery ( vats ) has been traditionally performed by a multi - port approach , but uniportal vats is gaining popularity among thoracic surgeons . the use of only one intercostal space may result in less pain , but competition among camera and operating instruments may be a disadvantage . in children , the limited space in the thorax makes the uniportal vats difficult to accomplish . we present a modification of the uniportal vats , using a single skin incision but placing the thoracoscope in the superior or inferior intercostal space relative to the working instruments to increase instrument range of motion within a single intercostal space .

pneumatosis cystoides intestinalis ( pci ) is a rare finding characterized by multiple air - filled cysts in the submucosa , subserosa and/or muscularis propria of the intestine . recent studies report that pci or pneumatosis intestinalis is more frequently found in the colon than the small bowel [ 1 , 2 ] . when located in the colon , it is specifically termed pneumatosis coli . symptoms associated with it are non - specific , commonly including abdominal pain and distension , diarrhea , bloody stool and constipation . the presence of pci can be an incidental finding of no clinical significance ; however , it can also be a sign of an underlying disease process such as bowel necrosis , mesenteric ischemia and bowel obstruction which can be life - threatening . when a serious underlying intra - abdominal pathology is suspected , urgent surgical intervention is warranted . we present a case of pneumatosis coli and pneumoperitoneum in an otherwise healthy male patient . a 54-year - old gentleman with 30 pack year history of smoking , chronic constipation and symptoms of irritable bowel syndrome presented to an outside hospital emergency department ( ed ) with 5 days of lower abdominal pain and cramping . the pain was initially very mild in nature , but suddenly became severe a few days after onset . he was afebrile with normal white blood cell count and c - reactive protein level . on examination computed tomography ( ct ) of the abdomen pelvis demonstrated findings concerning for colonic perforation ; there was a small amount of free intraperitoneal air scattered throughout the abdomen and pelvis and prominent cystic pneumatosis coli of the sigmoid colon ( fig . he presented to our clinic 2 days after discharge for further work - up complaining of persistent mild symptoms of abdominal pain . flexible sigmoidoscopy was performed showing approximately a 7 cm segment of sigmoid colon with numerous polypoid appearing lesions with grossly normal appearing overlying mucosa ( fig . notably , his last colonoscopy was approximately 10 months ago which demonstrated tubular adenomas , one in the descending colon and the other in the rectum . figure 2:flexible sigmoidoscopy : sigmoid colon . he was taken to the operating room electively for additional evaluation and potential therapeutic intervention . there were findings of chronic non - obstructing sigmoid volvulus with a twisted and inflamed sigmoid mesentery . extensive pci of the sigmoid colon was apparent externally and upon opening of the specimen on the back table ( fig . 3 ) . laparoscopic sigmoid colectomy with end - to - end colorectal anastomosis was performed . air pockets in the submucosa ranging from 0.3 to 1.5 cm in diameter were identified ( fig . 4 ) . microscopy revealed multiple intramural empty cysts lined by histiocytes and multinucleated giant cells , consistent with pci ( figs 5 and 6 ) . figure 4:multiple submucosal air pockets ( cysts ) ranging in size from 0.3 to 1.5 cm . figure 5:microphotograph : multiple cystic spaces in submucosa and muscularis propria , with mild hyperplastic mucosal changes . multiple submucosal air pockets ( cysts ) ranging in size from 0.3 to 1.5 cm . microphotograph : multiple cystic spaces in submucosa and muscularis propria , with mild hyperplastic mucosal changes . in this case report , we describe a patient who initially presented with symptomatic pneumatosis coli and pneumoperitoneum of undetermined etiology . despite the imaging findings , his entire clinical picture did not warrant emergent surgical intervention , and he was managed conservatively as an inpatient . he was discharged home and additional work - up was performed on an outpatient and elective basis , revealing multiple sigmoid hyperplastic polyps without evidence of transmural colonic perforation . pneumoperitoneum in association with pci can be secondary to viscus perforation or ruptured intramural cysts . when this patient initially presented to the ed , he had free air on ct concerning for colonic perforation . however , further work - up determined the source of pneumoperitoneum was likely from ruptured intramural cysts as there was no evidence of gross transmural perforation intraoperatively and on final surgical pathology of the sigmoid colon . sigmoid volvulus has been reported as both an etiology and a complication of pci . here , we favor chronic sigmoid volvulus as the etiology rather than a complication of pci . he had a redundant sigmoid colon , short mesenteric attachment and history of constipation , predisposing him to sigmoid volvulus . his presentation of recurrent abdominal pain with resolution may be due to recurrent torsion and spontaneous detorsion . this case describes chronic sigmoid volvulus as a potential etiology of pci and ruptured intramural cysts resulting in pneumoperitoneum .

this is a case of a 54-year - old gentleman who presented to an outside hospital emergency department with lower abdominal pain . computed tomography imaging showed a small amount of intraperitoneal free air and cystic pneumatosis coli . he was admitted , managed conservatively with intravenous antibiotics , and then discharged home after his symptoms improved . elective laparoscopic sigmoid colectomy was subsequently performed with intraoperative findings of partial sigmoid volvulus and extensive pneumatosis coli of the sigmoid colon . pneumoperitoneum was determined to be from ruptured intramural cysts . the etiology of pneumatosis coli was likely from chronic sigmoid volvulus .

a 40 year - old male smoker was presented with low back pain and mild epigastric discomfort . he had been taking non - steroidal antiinflammatory drugs for his back pain for two days . he came to the emergency room with 5 hours of epigastric discomfort early in the morning . he had dyspeptic symptoms with some certain foods for 2 years and had a heavy meal last night . he was using 2 different groups of antihypertensive drugs but he stopped taking them for 1 month . at presentation , his blood pressure was 200/100 mmhg at both arms and he had a mild epigastric discomfort pathologically . initial blood tests including cardiac enzymes , complete blood count , renal function tests , liver enzymes , amylase , lipase , and d - dimer were in normal ranges . intravenous proton pump inhibitor was started , and his blood pressure was lowered with short acting ace inhibitor orally . so a contrast enhanced thoracic and abdominal computed tomography ( ct ) scan was performed to rule out vascular emergencies . there was no dissection or aneurysm at the thoracic and abdominal aorta but isolated celiac artery dissection was observed . liver , spleen , bowel , and kidney perfusions were also normal ( figure 1 ) . upper abdominal contrast - enhanced ct scan revealed celiac artery dissection ten months later he was asymptomatic with good blood pressure control . dissection of an artery occurs with intimal layer disruption which allows blood to enter the media , and separates the intimal and adventitial layers . the dissecting column of blood forms a false lumen and may extend proximally , distally , or both . classical presenting symptom is sharp , stabbing pain radiating to back in the chest and upper abdomen.1 risk factors listed at previous reports include hypertension , cystic medial necrosis , abdominal aortic aneurysm , fibromuscular dysplasia , trauma , pregnancy , and connective tissue disorders . however , most of the patients in some series are generally healthy with no underlying disease . the definitive cause has not been well understood yet.56 for the diagnosis , conventional angiography , doppler ultrasonography , ct , and magnetic resonance imaging can be used . as in our case , contrast - enhanced ct is the advised technique because of its convenience , quickness , low invasiveness , and reliability.67 ct scan can provide high quality images of dissection site and also gives knowledge about the extension of the lesion , aneurysm formation , and intramural hematomas . the sma dissection alone and involvement of both sma and ca were reported much frequently than isolated cad . we have found 25 cases of isolated cad except our presenting case , at pubmed ( since 1966 ) , with the key words of celiac artery complications included ischemia , aneurysm formation , and rupture.2 as far as we know ; wang et al . published the last case of spontaneous isolated cad in the literature . the reported patient was a 44 year - old male presenting with chest and abdominal pain , and he was treated conservatively.8 the involvement of branch vessels is generally observed and patients showed various clinical signs and symptoms according to the involved branch vessel.9 our patient demonstrated normal flow at hepatic and renal arteries in ct scan . to our knowledge , there is only one spontaneous isolated cad case reported previously except our patient , without any branch vessel involvement or visceral damage , detected by ct scans taken on admission.8 traditional open surgery , endovascular surgery , and interventional radiological approach are invasive options of the splanchnic artery dissection treatment . indications for surgery in sma dissection include increasing the size of the aneurysm , intraluminal thrombosis , abnormal blood flow through the vessel , and the persistent symptoms despite antico - agulation.3 in case of cad , glehen et al . recommend surgical repair in the presence of complications like occlusive lesions , aneurysm formation , and arterial rupture , or extension of the cad into hepatic arteries.10 the recommended conservative treatments include anticoagulants , anti - platelets , and antihypertensives.4 we followed up our patient only with antihypertensive regimen . since there was normal flow at the visceral branches with intact organ perfusions , we did not use anticoagulants and anti - platelets . in conclusion spontaneous cad should be included in the differential diagnosis of refractory epigastric pain in the emergency room . it is a rare situation but may be underestimated in the past because of its probable benign nature . with continued improvement of the use of contrast enhanced ct scan in emergency room , the lesions may be diagnosed more commonly . we thought that uncomplicated cases with normal visceral perfusions can be managed only with strict control of blood pressure . but , for a definitive way of treatment large series of cases and prospective studies are needed .

dyspepsia with mild , stabbing epigastric discomfort without history of trauma is a very common symptom that emergency physicians see in their daily practice . vascular emergencies , mostly the aortic dissection and aneurysm , are always described in the differential diagnosis with persistent symptoms . isolated celiac artery dissection occurring spontaneously is a very rare diagnosis . the involvement of branch vessels is generally observed and patients show various clinical signs and symptoms according to the involved branch vessel . here we are presenting a case with spontaneous isolated celiac artery dissection , without any branch vessel involvement or visceral damage , detected by computed tomography scans taken on admission .

the posterior corneal surface was uneven , and there were opacities of dm in the corneal periphery . changes of the posterior corneal surface were documented with a specular microscope ( noncon robo pachy sp-9000 ; konan medical inc , tokyo , japan ) [ fig . 1 ] . by the age of 20 years , both his corneas had become edematous , and his best corrected visual acuity was reduced to 0.4 in both eyes . after signing an informed consent , specular microscopy images of the case with posterior polymorphous corneal dystrophy taken at the age of 13 years ; right eye ( a ) , left eye ( b ) the donor button was prepared from a cornea stored under hypothermic conditions at 4c in eusol - c ( alchimia s.r.l , padova , italy ) . donor age was 69 years , and the button consisted of a 6.0-mm diameter bare dm - endothelium lamella surrounded by a 1.0-mm wide posterior stromal rim ; the endothelial cell density was 3533 cells / mm . the exact surgical technique , including the preparation of the donor button , has been reported elsewhere . right eye 1 month after dmek - s ( note the scleral rim of the lamella ) the clinical outcome was satisfactory . at the last follow - up 2 years and 9 months after the surgery , the patient had no complaints , and the cornea was clear with an unaided visual acuity of 0.5 and best corrected visual acuity 1.0 with 1.25 diopter sphere . the central endothelial cell density measured with a topcon sp3000p noncontact autofocus specular microscope ( topcon corp , tokyo , japan ) 11 months after the surgery was 1089 cells / mm , and it was 1012 cells / mm at the last follow - up nearly 3 years after the procedure . this is the first report of a posterior lamellar keratoplasty performed in a cornea with ppcd , using lamellae with bare dm and endothelium in the optical axis . although the necessity for keratoplasty differs in various cohorts of ppcd patients ( it can be as high as 40% ) and a large majority of affected individuals are asymptomatic till an older age , many ppcd patients are younger at the time of grafting than patients with late - onset fuchs endothelial corneal dystrophy which is also treated with dmek and dmek - s . therefore , all possible benefits as well as disadvantages of various keratoplasty surgical techniques need to be very carefully evaluated . the reason why we chose dmek - s and not penetrating keratoplasty was that we considered it important to offer a young patient with an endothelial dystrophy the well - known benefits of posterior lamellar keratoplasty . compared to dsek , the lamellae used in dmek - s are thinner , allowing for an almost normal anatomic structure in the visual axis . although the technique requires the acquisition of additional surgical skills , the scleral rim makes the manipulation with the lamellae easier than in dmek , in our opinion . in addition , no instruments other than those used in routine penetrating keratoplasty and cataract surgery are needed in dmek - s , and the surgery can be performed repeatedly . the clear limitation of dmek - s is the endothelial cell loss , in the case presented here 69% at 11 months , and 71% at 2 years and 9 months after the surgery , which implies that the technique needs to be further improved . in the ppcd patient reported herein , dmek - s led to the rapid recovery of vision without postoperative complications ; however , the long - term survival of these grafts in the treatment of various conditions needs to be evaluated .

a 20-year - old patient , diagnosed with posterior polymorphous corneal dystrophy , developed corneal edema for which he underwent descemet membrane endothelial keratoplasty with a stromal rim ( dmek - s ) in the right eye . no intra- or postoperative complications were noted . at the last follow - up 2 years and 9 months after the procedure , the best corrected visual acuity was 1.0 and endothelial cell density declined from 3533 cells / mm2 to 1012 cells / mm2 . despite the endothelial cell loss , dmek - s appears to be a good alternative to other surgical techniques for the treatment of corneal endotheliopathies , and it may be of benefit to young patients .

different new technologies have become available in the recent years in the cardiac rhythm disease management . atrial sensor in single lead implantable cardioverter defibrillator ( icd ) and daily remote monitoring are examples of these innovations . new tools and technologies can drive a different approach in the management of patients , with efficiency improvements and better patient 's outcome . a 60-years - old man with ischemic cardiomiopathy ( lvef = 25% ) and two previous coronary artery bypass underwent single - lead icd ( iforia 5 dx , biotronik se & co. kg , berlin , germany ) implantation for primary prevention of sudden death in april 2014 . the dx icd is able of atrial sensing by the presence of a floating dipole mounted 150 mm from the tip and it is equipped with a daily remote monitoring system ( home monitoring , biotronik se & co. kg , berlin , germany ) . at the time of the implantation the patient had no history of atrial arrhythmias . in july 2014 a home monitoring ( hm ) alert of atrial monitoring episode detected ( longer than 8 h ) was received : the message arrived through fax during the night in the cure centre . the day after we contacted the patient the supraventricular arrhythmia had a low ventricular response , the mean heart rate was 83 bpm . we introduced in the patient 's therapy a new oral anticoagulant drug ( apixaban ) because he had cha2ds - vasc score of 3 . in the following days the persistence of the atrial arrhythymia was confirmed by the intracardiac electrogram ( iegm ) daily sent by the hm ( fig . 1 ) . we had a defined quantification of the arrhythmia with the daily atrial burden value ( percentage of the time in atrial arrhythymia ) . the synchronized atrial arrhythmia documented by hm was a typical atrial flutter ( afl ) , that was successfully ablated without any complications . in the following days the maintenance of the sinus rhythm was monitored : no arrhythmic alerts were receveid and the atrial burden was permanently 0% , as shown in fig . 1 . in october 2014 at the follow up visit , after one month from the ablation , we removed the oral anticoagulant drug in the patient 's therapy ; this decision was driven by the fact that any possible new arrhythmic episode would be notified in the cure centre with a hm alert . this case reports an early detection of asymptomatic atrial arrhythmia with consequent clinical interventions for its optimal management . this has been possible thanks to the concomitant utilization of two technologies : the atrial sensing in a single - lead icd and the daily remote monitoring of the device . an usual single - chamber icd would not have been able to detect this arrhythmia due to its low ventricular response , exactly as the patient had not been able for its asymptomatic nature . the dx icd with floating atrial dipole has been proven to provide reliable atrial sensing without the need to insert a second electrode which carries in itself a significant associated risk and expense . there is a substantial incidence of subclinical atrial tachyarrhythmias which are a leading preventable cause of recurrent stroke . the data become meaningful if they are early available for the physician to prevent arrhythmia - related severe adverse events . without remote monitoring any information is available only during in - hospital follow - up , usually scheduled every 6 or 12 months , significantly delaying any clinical reaction . in this case we had the opportunity to contact the patient the day after the arrhythmia occurrence and to have prompt reaction in terms of drugs therapy modification that may have avoided major clinical adverse events . early detection of atrial arrhythmia and prompt patient management may theoretically prevent stroke , ; however clinical evidence for stroke risk reduction by remote monitoring is still awaited . this case is also an example of hm clinical practice : in our centre there is a reference nurse for the transmissions who filters the significant notifications for the expert physicians . when an organizational model is implemented , the hm has been also demonstrated to safely reduce healthcare source consumption , in terms of patient visits , time required for patient follow - up , physician time , costs of patient transport , and hospital incurred costs . we managed this patient remotely ; just one ambulatory visit was performed one month after the ablation procedure . in this occasion nowadays telemedicine should be used in the management of arrhythmic patients and it may guide in some cases , as in the one here presented , the clinical decisions .

this case report describes how new tools and technologies can drive a different approach in the management of arrhythmic patients . an unknown and asymptomatic atrial flutter was detected by the atrial sensor mounted in a single lead implantable cardioverter defibrillator . moreover daily remote monitoring of the device allowed early notification and prompt clinical reaction . anticoagulant therapy onset , radiofrequency ablation and the following anticoagulant therapy removal were driven by the device data transmissions .

it is caused by filarial worms of the genus dirofilaria species , which is the natural parasites of dogs , cats , foxes , and wild mammals . dirofilaria species belongs to the filarial nematodes , causes zoonotic infections in man , occasionally . subcutaneous dirofilariasis is caused mainly by dirofilaria repens , which causes subcutaneous nodules in and around the eye . the mosquitoes culex , aedes , and even anopheles species form the vector for this nematode . a 28-year - old male hailing from cochin , south india presented with a subcutaneous nodule on the left cheek of duration of 2 weeks , which was gradually increasing . on clinical examination , he had a firm oval swelling on the left cheek measuring 1.5 cm 1 cm , which was non - tender and freely mobile . a clinical diagnosis of a subcutaneous swelling / sebaceous cyst was made , and the swelling was excised as an out - patient procedure under local anaesthesia . on excision two live thread like cylindrical worms , 0.5 mm thick measuring about 10 - 25 cm were found , coiled to each other . on macroscopic examination , of both the worms it was found that both the worms had a short and rounded tail [ figure 1 ] . the outer surface of the nematode 's cuticle was found to have fine transverse striations and prominent longitudinal ridges [ figure 2 ] . the longer worm had oesophagus anterior to the vulva and was identified female dirofilarial worms . on the basis of macroscopic and , microscopic examination , worm was thus identified as d. repens . two live dirofilarial worms microscopic picture of the worm showing cuticle and transverse striation the hemogram showed hemoglobin 13 g% , total leucocyte count 10,200/mm and differential count showed eosinophilia , with absolute eosinophil count 2060/mm . the patient did not review after the excision of the nodular swelling for follow - up . human subcutaneous dirofilariasis is a rare helminthic infection , caused by filarial worms dirofilaria species . the worm is a natural parasites of dogs , cats , foxes , and wild mammals . the clinical implication of human dirofilariasis is that , these subcutaneous lesions may be initially misidentified as malignant tumors , requiring invasive investigation and surgery before the correct diagnosis is made . the pathology of the condition results from the aberrant localization of immature worms intended for nonhuman hosts . among the documented cases of human dirofilariasis in india , most of them had ocular infections.[24 ] in india and the coastal kerala is endemic for dirofilariasis due to the presence of the suitable vector and climatic condition . most of the cases of dirofilariasis reported from the northern and western parts of india is caused by d. repens . the cause of endemicity in southern - india and sri lanka is mainly due to drastic changes in climatic conditions especially humidity and rainfall pattern , which favor the development of vector mosquitoes and the larval phase of the nematode in the mosquitoes . recently two more cases have been reported from the southern - india presenting as subcutaneous nodules . most cases are diagnosed retrospectively , when the histopathological sections of biopsy or excision material are viewed . thus , this case emphasizes that subcutaneous dirofilariasis should be considered as the differential diagnosis in case of a single subcutaneous swelling , especially , when the patient is coming from an endemic area .

human subcutaneous dirofilariasis is a rare helminthic infection . it is caused by filarial worms of the genus dirofilaria , which is the natural parasites of dogs , cats , foxes , and wild mammals . zoonotic filariasis caused by dirofilaria species is rarely seen in the region of dakshina kannada . most of them are seen in patients who are along the border of the district which shares its boundary with the state of kerala , where dirofilariasis is constantly reported to occur . here , we report a case of subcutaneous dirofilariasis , presenting as a subcutaneous nodule on the left cheek of a 28-year - old male from cochin ( ernakulam ) , kerala , south india . two live worms were recovered from the subcutaneous nodular swelling .

we present a case of a previously fit male presenting with a spontaneous upper - limb compartment syndrome and subsequent diagnosis of aplastic anaemia . a 17-year - old man presented with pain and swelling of his left forearm with his wrist and fingers held in a flexed position ( fig . 1 ) . prior to this , there was a 3-day history of progressive pain in the forearm , but no history of trauma . the patient had no previous medical history , was on no regular medications and had no history of previous infections . a clinical diagnosis of compartment syndrome was made and the patient was planned for urgent fasciotomies . routine blood examination demonstrated pancytopenia with a haemoglobin level of 3.9 g / dl , white cell count of 0.9 10/l and platelet count of 4 10/l . in addition , an urgent ultrasound scan was performed to rule out haematoma and possible tamponade of an arterial bleed . after transfusion of three units of cross - matched blood , two units of fresh frozen plasma and two units of platelets , an urgent fasciotomy and drainage of the collection under general anaesthesia was performed with a concurrent iliac crest bone marrow biopsy . figure 1:image of the left forearm with swelling and with the wrist and fingers held in a flexed position . image of the left forearm with swelling and with the wrist and fingers held in a flexed position . a modified henry 's approach and carpal tunnel decompression was performed and revealed a collection of pus was superficial and ulna to flexor digitorum superficialis ( fds ) muscle . the distal muscle of fds was ischaemic and swollen preventing finger extension due to the tight wrist and carpal tunnel fascias . the ischaemic muscle was debrided and a dusky flexor carpi ulnaris ( fcu ) was decompressed and left in situ for a review at second look surgery ( fig . 2 ) . staphylococcus aureus , sensitive to flucloxacillin , on which he was commenced after samples were taken . no further focus of infection was identified . in the following 12 days , the patient underwent four further surgical debridements , topical negative pressure wound therapy and delayed primary closure of the wound . only partial resection of fds and fcu was necessary ; therefore , all functional units were maintained ( fig . 2 ) . an immediate family member was found to be a match for a bone marrow transplant which has been carried out successfully . at latest review , 6 months following initial presentation , the patient had regained full extension of wrist and digits , full wrist flexion and full finger flexion apart from a mildly limited middle finger which comes within 1 cm of his palm . his median and ulna nerve sensation has resolved after being slightly reduced compared with the opposite side in the initial postoperative period . this is the first reported case in the literature of a compartment syndrome being the first presentation of aplastic anaemia . aplastic anaemia is a rare haemopoietic stem - cell disorder that is defined as pancytopenia with a hypocellular bone marrow . it has a variety of presenting symptoms , including anaemia , bleeding disturbances and a wide range of infections [ 3 , 4 ] . the diagnosis of aplastic anaemia was made following the routine blood results and subsequent bone marrow biopsy . compartment syndrome occurs when the pressure in an osteofacial envelope increases and prevents tissue perfusion , resulting in ischaemia and subsequent tissue necrosis . early diagnosis is vital to prevent permanent tissue necrosis and the formation of fibrotic tissue which leads to permanent disability . upper - limb compartment syndrome is a rare diagnosis and is most commonly caused by trauma resulting in forearm fractures , but haemorrhage , iatrogenic injuries and many atraumatic causes are reported [ 1 , 2 ] . these include post - thrombolysis , secondary to acquired factor viii inhibitor , following anticoagulation and secondary to insect or snake bites [ 1 , 2 , 58 ] . these were all unusual presentations , and therefore the clinician must have the diagnosis of compartment syndrome in their mind for unexplained pain in the forearm . the management of compartment syndrome is through fasciotomy which provides release of the high compartment pressures and allows tissue perfusion to be restored . there are numerous different reported techniques for the incision for a forearm fasciotomy [ 1 , 9 ] . patients with upper limb compartment syndrome have a varied outcome with up to a third suffering some long - term complications , and a fifth suffering some neurological deficit [ 1 , 9 ] . in conclusion , it is vital that the attending clinician considers the potential causes of atraumatic compartment syndrome and a multidisciplinary approach with early intervention can improve both short- and long - term outcomes .

this is a case of a previously fit and healthy 17-year - old man presenting with atraumatic forearm compartment syndrome . on his initial blood film , he was found to have a pancytopenia and was subsequently diagnosed with aplastic anaemia . his compartment syndrome was treated with urgent fasciotomy after correction of his pancytopenia . he made a good functional recovery with only a very minor deficit , and no recurrence of his compartment syndrome . he has undergone a successful bone marrow transplant for treatment of his aplastic anaemia .

it causes occlusion of the cerebral vasculature most frequently affecting the intracranial internal carotid arteries and proximal segments of the middle cerebral and anterior cerebral arteries . the occlusion of the major intracranial arteries is associated with appearance of a tuft of fine collateral vessels at the base of the brain . the clinical presentations include transient ischemic attacks , ischemic strokes in children and hemorrhage in adults . atypical presentations in the form of headaches , seizures , cognitive impairment , and dementia are also seen . the clinical presentation as an isolated initial manifestation with predominant movement disorders is extremely rare , especially in the pediatric population . an 11-year - old male child presented with a 6 month history of the progressive recurrent dystonic posturing of limbs and trunk . there was no history of visual loss , motor weakness , sensory deficit or any bladder and bowel disturbance . the general physical , cardiovascular , respiratory , and per abdominal examination were unremarkable . his biochemical examination including a complete hemogram , blood sugar level , renal , and hepatic function tests was normal . he was subjected to magnetic resonance imaging ( mri ) of brain examination which revealed multiple focal and confluent t2-weighted and fluid attenuated inversion recovery weighted hyperintense lesions in bilateral basal ganglia and periventricular regions [ figure 1a d ] . the precontrast magnetization prepared rapid acquisition gradient echo ( mprage ) images showed these areas as hypointensities [ figure 2a and b ] , with filling of contrast on postcontrast mprage images [ figure 2c and d ] . due to distribution of these lesions predominantly along basal ganglia vasculopathy was suspected and patient was subjected to magnetic resonance angiography next day using time - of - flight technique which showed abrupt occlusion of terminal parts of bilateral internal carotid arteries [ figure 3a and b ] , with attenuated anterior and posterior circulations arteries and multiple collaterals giving puff of smoke appearance [ figure 3c and d ] . axial t2-weighted ( a and b ) and fluid attenuated inversion recovery images ( c and d ) showing multiple focal and confluent hyperintensities ( white arrows ) axial magnetization prepared rapid acquisition gradient echo precontrast ( a and b ) images showing hypointense foci which on postcontrast images ( c and d ) show enhancement ( white arrows ) magnetic resonance angiography ( time - of - flight technique ) images show abrupt occlusion of terminal parts of bilateral internal carotid arteries yellow arrows in ( a and b ) , axial image show collaterals in posterior circulation white thick arrow in ( c ) , coronal image shows attenuated arteries with multiple collaterals giving puff of smoke appearance white arrow in ( d ) it is a progressive bilateral occlusive state of the distal internal carotid artery and the proximal segments of the middle cerebral as well as anterior cerebral arteries . the intracranial vascular changes are primary and truly idiopathic . in this rare form of vasculopathy , revealed multiple small ischemic watershed infarcts on mri in three patients of mmd who presented with movement disorders in the bilateral frontal subcortical white matter . suggested that movement disorders appear as a manifestation of mmd with an estimated frequency of 36% . the dystonic movements are self - limited and are usually no symptoms or signs of disease are noted in the interval between episodes . symptoms of movement disorders in these patients probably occur due to ischemia or changes in excitatory and inhibitory circuits interconnecting cerebral cortex and the basal ganglia . the basal ganglia receive cortical afferents from associative cortical areas such as prefrontal , temporal , posterior parietal , and preoccipital cortices . ischemic dysfunction of basal ganglia , the thalamus , cerebral cortex , and brainstem have also been implicated to stimulate simple or complex movement disorders . operated six patents of mmd presenting with involuntary movements and suggested that in all patients movement features improved completely within 3 months of bypass grafts secondary to improved cerebral perfusion . the early recognition of mmd as the etiology for movement disorders help in managing these patients with direct and indirect bypass surgery which offers an effective means of revascularization . we conclude that should be included in the differential diagnosis of pediatric patients presenting with recurrent involuntary movements , dystonia , chorea , and athetosis . we also suggest that pediatric patients of dystonia undergoing mri brain examination , should be supplemented with mra to rule out vasculopathy presenting as a rare cause of movement disorders .

moyamoya disease ( mmd ) is a rare cerebrovascular disease characterized by idiopathic occlusion of bilateral internal carotid arteries and the development of characteristic leptomeningeal collateral vessels along anterior or posterior circulation . we present an unusual case of mmd presenting with generalized dystonia as the predominant manifestation .

pilomatrixoma or pilomatricomais a relatively uncommon benign tumor that originates from the matrix cells at the base of the hair . the most common location of this tumor is the head and neck region , followed by the upper extremities and rarely in the lower extremities . it presents as a solitary , slow - growing dermal or subcutaneous nodule ; however , clinical diagnosis is often difficult or misdiagnosed . histologic features of this tumor are well recognized , but cytological features often produce diagnostic difficulties . a 35-year - old female presented with a nodular ulcerated swelling measuring 3 2.5 cm over the left lower leg since the past 5 years [ figure 1 ] . the patient attended fine - needle aspiration cytology ( fnac ) clinic with a clinical suspicion of malignancy . on physical examination , the lesion was not fixed to the underlying bone and no inguinal lymphadenopathy was detected . external clinical photograph . ulcerated nodular lesion in the lower leg fine - needle aspiration was performed using a 22-gauge needle . cytological smears were cellular , showing clusters of basaloid cells having round to oval nuclei , nucleated squamous cells , and anucleated squames . few fragments of shadow cells adherent to basaloid cells were also noted [ figure 2a and b ] . a cytological diagnosis of pilomatrixoma was made . fine - needle aspiration cytology showing ( a ) clusters of basaloid cells admixed with squamous cells and anucleated squames ( may - grnwald - giemsa 200 ) ; and ( b ) cluster of shadow cells adherent to basaloid cells ( mgg , 400 ) excision biopsy and histopathologic examination of the resected specimen showed typical features of pilomatrixoma with basaloid cells in the periphery , shadow cells toward the center , and a few foreign body - type giant cells [ figure 3a and b ] . sections showing ( a ) islands of basaloid cells and shadow cells with surrounding foreign body giant cells and inflammatory cells ( h and e , 100 ) ; inset : showing shadow cells in higher magnification ; and ( b ) basaloid cells in higher magnification ( h and e , 400 ) pilomatrixoma is a relatively uncommon benign tumor of hair matrix differentiation , first described by malherbe as benign calcifying epithelioma , as a tumor of the sebaceous gland . later on , the term was changed to pilomatrixoma because of its histogenesis from hair matrix cells . the most common sites involved in this tumor are the head and neck region , followed by the upper extremities , trunk , andthe lower extremities . this tumor usually occurs in children younger than 10 years of age and also in the second decade of life . clinically , this tumor presents as a soft to firm subcutaneous mass with intact overlying skin with average size 1.6 cm . correct clinical diagnosis is very rare and differential diagnosis includes epidermal cyst , dermoid cyst , cysticercosis , sebaceous cyst , sebaceous adenoma or carcinoma , capillary hemangioma , chalazion , rhabdomyosarcoma , and so on . fna of the lesion commonly reveals basaloid cells , squamous cells , anucleate squames , shadow cells , foreign - body giant cells , polymorphs , and calcification in variable frequencies . as all these findings are not present in a single case , cytological misdiagnosis is common and includes epidermal inclusion cyst , adnexal tumor , squamous cell carcinoma , basal cell carcinoma , mucoepidermoid carcinoma , or even round - cell tumor . when aspirates contain mainly nucleated squamous cells and squames , it leads to a wrong diagnosis of epidermal inclusion cyst . predominance of basaloid cells can give a misdiagnosis of basal cell carcinoma or even round - cell tumor . in aspirates of pilomatrixoma , the presence of foreign - body giant cells is also variable . moreover , they can be found in other lesions such as epidermal inclusion cysts , ruptured benign cysts , and panniculitis to squamous cell carcinoma . extreme calcification in pilomatrixoma may yield only calcified material and proper diagnosis may not possible . histopathologic examination provides the correct diagnosis and shows solid nests of basaloid cells with the formation of ghost cells with foreign - body giant - cell reaction , calcification , and ossification in some cases . our patient presented with an ulcerated nodule in the left lower leg . in our case , fnac yielded basaloid cells , squamous cells , anucleate squames , and shadow cells ; which helped us arrive at a correct preoperative diagnosis . histopathology also confirmed the diagnosis . in our case , the site of the lesion , which was very uncommon , and the presence of surface ulceration led to a clinical misdiagnosis . knowledge of the morphological spectrum of characteristic cellular components helps in correct preoperative diagnosis of pilomatrixoma by fnac and helps in proper patient management . lastly , we have presented this case because of the rare location of the tumor and the malignant clinical presentation . this case also highlights the role of fnac in arriving at a correct preoperative diagnosis .

pilomatrixoma or pilomatricoma is an uncommon benign tumor of hair matrix origin , which most commonly occurs in the head and neck region and upper extremities . in this study , we report a case of pilomatrixoma of a 35-year - old female presenting with left - leg swelling with surface ulceration , clinically thought to be malignant . fine - needle aspiration cytology ( fnac ) of the lesion showed clusters of basaloid cells with round to ovoid nuclei , nucleated squamous cells , and anucleated squames and clusters of shadow cells . acytological diagnosis of pilomatrixoma was made . subsequent surgical excision and histopathological examination confirmed the diagnosis . this case highlights a rare site of presentation and the role of fnac in preoperative diagnosis of this benign tumor for proper management .

given the multiplicity of anti - inflammatory actions of activated protein c ( apc ) , it is perhaps not surprising that apc would be effective in reducing the severity of lung injury produced by the use of large tidal volumes during mechanical ventilation . in this issue of critical care , maniatis and colleagues provide data showing that inhaled apc is protective against ventilator - induced lung injury ( vili ) . the use of inhaled apc in these studies is an intriguing use of this agent and would be expected to target its effects to the airways and alveoli affected by vili while minimizing systemic effects . unfortunately , the present study does not provide any information about circulating coagulation parameters or about potential leakage of the inhaled apc into the systemic circulation , so it is unknown at present whether the beneficial effects found in these experiments are due purely to the pulmonary effects of apc or whether inhaled apc can gain access to the circulation , thereby exerting its effects on the lungs and other organs . in addition to its effects on coagulation cascades , apc has been shown to have a number of actions that may contribute to reducing inflammation in the setting of sepsis or acute lung injury . for example , apc has potent anti - apoptotic effects on endothelial cells that remain even when modified forms of apc that lack anti - coagulant properties are used . interaction between apc and the endothelial protein c receptor , which is actually a misnomer since this receptor is also found on other cell populations , including neutrophils and mononuclear cells , results in diminished migration toward inflammatory gradients . in human studies in which lipopolysaccharide ( lps ) was instilled into airways , there were decreased numbers of neutrophils in the airways among the volunteers randomly assigned to infusions of apc . recent data have shown that apc cleaves histones released by dying cells , diminishing the damage to endothelial cells and inflammatory responses initiated by exposure to histones . finally , interaction of apc with protease - activated receptor-1 can diminish the activation of macrophages and other cell populations [ 5 - 7 ] . while the authors of this article hypothesize that the beneficial actions of inhaled apc in vili result from inhibition of activation of the extracellular - regulated kinase ( erk ) pathway , this potential mechanism is not directly explored . rather , diminished erk activation is shown in lung homogenates and in cultured lung epithelial cells after treatment with apc . however , it remains unclear whether the decrease in erk activation is the cause of the beneficial effects of apc or rather is simply a reflection of the decrease in inflammation produced by apc treatment . additional experiments in which erk was specifically inhibited would be necessary to determine the importance of this signaling pathway in modulating the effects of apc in vili . several previous studies have shown that inhaled apc reduces lung injury and inflammation in models of lps - induced lung injury [ 8 - 10 ] . in those studies , despite decreases in histologic indices of lung injury , neutrophil numbers and concentrations of proinflammatory cytokines did not appear to be reduced after apc treatment . in contrast , in at least one study , inhaled apc did reduce the severity of coagulation abnormalities in the lungs . such results suggest that the beneficial effects of inhaled apc may result from reversing alterations in coagulation and fibrinolysis , which are almost ubiquitous findings in acute lung injury , or possibly from diminishing the enhanced apoptosis of epithelial and other cell populations which accompanies acute lung injury . in the present experiments , treatment with apc therefore , it remains unknown at present whether inhaled apc would be beneficial if initiated after the lungs have been exposed to large tidal volumes , an important clinical issue . additionally , low - tidal - volume ventilation has become the standard of care in critically ill patients because of studies showing that its use improves outcome , including diminishing mortality , in patients with acute lung injury and also is likely to prevent the development of lung injury when used as the initial mode of ventilation [ 11 - 14 ] . therefore , the clinically relevant questions now become whether inhaled apc can diminish the severity of lung injury when used in conjunction with low - tidal - volume ventilation in the presence of acute lung injury due to sepsis or other etiologies and whether inhaled apc can prevent or minimize primary or secondary lung damage when low tidal volumes are used for mechanical ventilation . apc : activated protein c ; erk : extracellular - regulated kinase ; lps : lipopolysaccharide ; vili : ventilator - induced lung injury . this work was supported , in part , by national institutes of health grants gm87748 and hl76206 .

systemic administration of activated protein c ( apc ) has been shown to reduce pulmonary inflammation in preclinical models of acute lung injury . however , there is only limited information concerning the effects of inhaled apc in modulating the severity of pulmonary inflammation . in a study reported in this issue of critical care , maniatis and colleagues show that pretreatment of mice with inhaled apc is protective against ventilator - induced lung injury . while the mechanisms responsible for this effect require additional elucidation , inhaled apc appears to be a potentially useful intervention in diminishing the severity of ventilator - induced lung injury and other forms of acute lung injury .

antihypertensive medication combination therapy in neonates should be monitored closely for hypotension.the interaction of a beta blocker and amlodipine therapy in the neonatal period needs to be studied further . neonatal hypertension secondary to renal , endocrine or other causes is commonly seen in sick newborn infants . its treatment may include use of beta blockers , vasodilators such as hydralazine , calcium channel blockers or alpha blockers . although amlodipine has been shown to be effective and safe when used in children , its use has not been fully studied in neonates nor in combination with beta blockers in that age group . however , amlodipine was listed in a recent review article as one of the treatment options for hypertension in neonates . in an infant born to a mother with grave s disease , we report the occurrence of severe hypotension , which developed after amlodipine was administered in addition to the beta blocker therapy already initiated for hypertension secondary to thyrotoxicosis . a male newborn infant , with a birth weight of 2,210 g , was born by caesarean section at 32 weeks of gestation to a 35-year - old mother with a 9-year history of grave s disease . thyrotoxicosis was confirmed by physical examination and laboratory investigations : his serum thyroxin ( t4 ) level was 59 ng / dl and his thyroid stimulating hormone ( tsh ) level was 0.013 iu / ml . oral treatment with carbimazole 500 g every 8 h and propranolol 2 mg / kg / day , divided into two doses , was started at 24 h after birth . on day 4 , the infant developed hypertension , with a mean blood pressure ranging from 90 to 100 mmhg . on the recommendation of a paediatric nephrologist , therapy with amlodipine 0.1 mg orally once daily was started . twenty hours after administration of the first dose of amlodipine , the child developed severe hypotension , with a mean blood pressure of 27 mmhg , and required supportive care with mechanical ventilation and intravenous inotropes ( dopamine and dobutamine at a rate of 20 g / kg / min ) . amlodipine was stopped , propranolol therapy was continued , a full septic work - up was performed and all cultures showed no bacterial growth . following the discontinuation of amlodipine , the blood pressure normalized , and the inotropes were stopped within 36 h. by day 8 , propranolol was discontinued , as the blood pressure and serum t4 level ( 14.7 g / dl ) were normal . this case report aims to describe the occurrence of severe hypotension following amlodipine administration for hypertension developing in a preterm infant with thyrotoxicosis who was already receiving beta blocker . this is similar to the findings in an animal study by ishizaka et al . , where more significant hypotension occurred when a beta blocker was added to amlodipine than when it was added to a newer calcium channel blocker . recent research supports the use of amlodipine , a calcium channel blocker , in young children . robinson et al . described its use in 33 children aged 1.316.9 years and showed that it was well tolerated when used for 6 months . lago rivero et al . reported its use in refractory hypertension in a 5-year - old girl with secondary hypertension . by contrast , there have been no good studies on the use of calcium channel blockers in neonates . one case report described the development of subcutaneous fat necrosis in a newborn whose mother had received a calcium channel blocker . a case report of death caused by an amlodipine overdose recommended caution when using it in young infants . the current report suggests that severe hypotension may result from combining amlodipine with beta blocker therapy in neonates . informed consent was obtained from both the infant s father and his mother for the publication of this report .

a 35-year - old woman with a 9-year history of grave s disease delivered a male infant weighing 2,210 g at 32 weeks of gestation by caesarean section . the neonate developed thyrotoxicosis and , at the age of 24 h , was treated with oral carbimazole ( 500 g every 8 h ) and propranolol ( 2 mg / kg / day in two divided doses ) . he subsequently developed hypertension on day 4 , which required therapy with amlodipine ( 0.1 mg once daily ) . severe hypotension developed within 24 h and required discontinuation of amlodipine , with initiation of intravenous inotropic support with dopamine and dobutamine ( at a rate of 20 g / kg / min ) . the blood pressure rapidly normalized , and both dopamine and dobutamine infusions were stopped within 36 h. a naranjo assessment score of 6 was calculated , indicating that the severe hypotension was a probable adverse drug reaction caused by the combination of amlodipine and propranolol therapy .

appropriate antibiotic therapy for bacterial sepsis is one of the most important therapeutic challenges , as mortality clearly depends on this issue . therefore , most therapeutic strategies rely on an initial appropriate and broad spectrum antibiotic use . resistance of numerous bacterial species evolved as a major clinical problem , especially in intensive care units ( icus ) . additionally , from an economic point of view , unnecessary antibiotic use is highly expensive overall . hence , unnecessary or inappropriate anti - microbial therapy should be avoided for medical and economic reasons . on the other hand , at the beginning of bacterial sepsis , clinicians are often forced to make decisions regarding the change of anti - microbial therapy , especially within the first hours . unfortunately , until today no concluding and approved clinical parameters are available to support those decisions . because of their dynamics c reactive protein ( crp ) and procalcitonin ( pct ) alone are not good enough to support treatment monitoring in these very early stages of sepsis . studies regarding pct guided anti - microbial therapy decisions for example , did not show improvement of patient outcomes . thus pct is regarded as investigational , concerning appropriateness or change of anti - microbial therapy in sepsis . therefore , even if the chosen anti - microbial strategy is appropriate , early therapy of bacterial sepsis is often guided by personal experiences and expert opinions rather than laboratory markers and evidence based approaches . in 2013 jekarl et al . were able to show that in serial measurements ( follow - up in the first 96 hours of sepsis ) interleukin-6 ( il-6 ) is a more reliable parameter than crp and pct to distinguish between survivors and non - survivors in bacterial sepsis . in their cohort , 86% of the survivors showed a decline in il-6 levels after 48 hours , in contrast to non - survivors . therefore the authors proposed that il-6 exhibits better dynamics for monitoring anti - microbial sepsis therapy . however , in their cohort they observed only 16 patients with severe sepsis or septic shock and serial pct , il-6 and crp measurements were available for only nine patients ( 6 non - survivors ; 3 survivors ) . therefore , they stated that additional data are needed to underline their observations and we intend to do so with the data we present in this brief report . we analyzed charts of patients who were admitted to our icu between 07/2012 to 08/2013 with the diagnosis of severe sepsis or septic shock according to the criteria of angus and van der poll , and for whom serial il-6 , crp , pct and white blood cell count ( wbc ) measurements were available . 20 patients met these criteria . for patient characteristics , detected microbes , anti - microbial therapy and baseline values of il-6 , crp , pct and wbc the summarized kinetics of il-6 , crp , pct and wbc are shown in figure 1 . to illustrate the biomarker kinetics data are depicted as relative fold change between mean baseline values and the mean values after 24 , 48 and 72 hours . in our study il-6 showed a rapid decrease in survivors whereas in the non - survivor - group there was a delayed decrease or even an increase in the first 24 - 48 hours . interestingly , crp and pct showed an initial decrease only after 48 hours in the survivor group , at a time when the relative il-6 decrease had already become significant . in contrast , wbc was still rising at 72 hours in our survivor cohort ( figure 1 ) . additionally , in the survivor group we were able to observe a significant increase of crp after 24 hours . therefore , in this setting il-6 seems to be the only predictor of appropriate ( anti - microbial ) therapy in the first 24 - 48 hours of severe bacterial sepsis and septic shock . in the non - survivor group none of the studied biomarkers declined during the observed time period ( figure 1 ) . regarding the absolute baseline values , only wbc was significantly higher in the non - survivor group compared to survivors ( table 1 ) . the other biomarkers showed no significant baseline value differences between the two groups ( table 1 ) . jekarl et al . made similar observations in their cohort and hypothesized that the observed il-6 decline might be related to the appropriateness of the chosen antibiotic therapy regimen , although they did not have data supporting their hypothesis . in our cohort we observed that the initially chosen antibiotic therapy was appropriate in 10/14 survivors ( 71% ) in whom etiological organisms could be detected e.g. by blood , urine and/or bronchial lavage cultures . for 4/14 survivors no microbes could be detected , but an infectious focus was confirmed e.g. by radiological imaging . in the non - survivor group 4/6 patients ( 67% ) were treated with appropriate therapy according to the detected microbes , but in most of these patients at least two different microbes were detected . therefore , these patients were challenged with a broader bacterial spectrum . for one patient of the non - survivor group the initial antibiotic therapy was not appropriate ( necrotizing pancreatitis ) and for another patient no etiological organism could be detected . our data confirm that a rapid decline of il-6 in the first 24 - 48 hours ( before microbial cultures are available ) could be a useful tool to identify patients in whom anti - microbial therapy will be successful . a prospective analysis of the outcome of an il-6-guided anti - microbial sepsis - therapy is necessary before this approach can be recommended for general use .

early appropriate anti - microbial therapy is necessary to improve outcomes of septic patients . we describe 20 case histories of patients with severe bacterial sepsis regarding kinetics of several biomarkers . we found that interleukin-6 is able to predict survival and might be able to evaluate appropriateness of anti - microbial therapy .

nonepithelial tumors account for 25% of all primary urinary bladder neoplasms . inflammatory myofibroblastic tumor ( imt ) imt has been described in major organs , including lungs , liver and skin , along with mesentery and retroperitoneum . lung is the most common site of occurrence for this tumor . in the genitourinary system , imt has been found most commonly in the urinary bladder but has also been reported in the kidneys , prostate , ureter , and epididymis . the first case of imt of the bladder was reported by roth in 1980 . the largest clinicopathologic study comprising of 42 cases of imt of the bladder , we report a case of imt of the urinary bladder and discuss its clinical presentation , diagnosis , and management . a 30-years - old man presented in the surgery outpatient department with painless gross hematuria for 2 weeks . there was no history of fever , trauma , bladder instrumentation , recurrent urinary tract infections , sexually transmitted disease 's or weight loss . initial abdominal ultrasound showed a 6 cm 4 cm 4 cm sized , broad - based polypoidal growth arising from the posterior wall of urinary bladder which was confirmed on computed tomography abdomen as having deep muscle invasion and nonuniform dye uptake . microscopically , the submitted material showed urothelium with underlying loose spindle cell proliferation with tissue culture appearance . the tumor was composed of plump spindle cells with abundant eosinophilic cytoplasm and elongated nuclei ( without nuclear atypia ) in a myxoid and inflammatory background of plasma cells and lymphocytes [ figure 1 ] . in addition , tumor cells surrounded by smooth muscle was also seen [ figure 2 ] . on immunohistochemistry , these spindle cells were strongly positive for ae1/ae3 and focally positive for -smooth muscle actin ( -sma ) . proliferation of plump spindle cells in a fibromyxoid background with lymphoplasmacytic infiltration ( h and e , hp ) tumor surrounding smooth muscle bundles ( h and e , mp ) inflammatory myofibroblastic tumor of bladder is an uncommon tumor of controversial nosology ; at the edge between benign and malignant tumors and has also been variously named as inflammatory pseudotumor , atypical myofibroblastic tumor , atypical fibromyxoid tumor and plasma cell granuloma . the term inflammatory fibrosarcoma has been proposed for the more aggressive tumors of this group . though any age can be affected , it is characterized by proliferation of plump , bland spindle cells arranged in a vaguely fascicular fashion in a fibromyxoid and inflammatory background of plasma cells , lymphocytes , and other inflammatory elements . anaplastic or pleomorphic features , as well as atypical or bizarre mitotic figures , are absent . postoperative spindle cell nodule is a histologically similar , reactive lesion that occurs weeks to months after transurethral resection ( tur ) of prostate or bladder lesions . pseudosarcomatous proliferation is another similar lesion , which shows higher cellularity , more prominent hyperchromasia , prominent nucleoli and nuclear pleomorphism ; is more infiltrative and shows strong , diffuse alk positivity . it is accompanied by fever , anemia and weight loss , all of which remit after tumor excision . imt shows immunohistochemical positivity for vimentin ( strong , diffuse ) , sma , muscle specific actin , calponin and alk . the pathogenesis of imt is still in doubt - some regard this entity as a reactive or inflammatory condition , while others believe that it represents a low - grade mesenchymal malignancy . recent evidence suggests that it is a neoplastic process of low - grade nature because of its aggressive behavior , deep infiltration , occasional coexistence with urothelial carcinoma and the demonstration of a nonrandom chromosomal translocation involving chromosome 2p23 and cytogenetic monoclonality . close follow - up is required and complete surgical resection is important to avoid local recurrence .

inflammatory myofibroblastic tumor ( imt ) is a rare , aggressive tumor of indeterminate malignant potential with myofibroblastic differentiation . though bladder location is very uncommon , it arises from the bladder submucosal stroma as a polypoidal growth and is easily mistaken for a malignant neoplasm- clinically , radiologically and histologically . essential criteria for the diagnosis of imt are : spindle myoepithelial cell proliferation and lymphoplasmacytic infiltrate . here we report the case of a 30 years old man who presented with painless gross haematuria for 2 weeks . the patient underwent open partial cystectomy and the final pathological diagnosis was imt of bladder .

in november 2007 , a 28-year - old female patient presented at our hospital with acute onset of hemoptysis , characterized by the daily expectoration of 50 ml of fresh blood . the patient was a non - smoker with no prior history of chronic lung disease . further , a physical examination performed upon admission revealed left lung basal crepitation with good air entry . in addition , the patient showed no signs of a fever and was hemodynamically stable . however , the patient developed another episode of massive hemoptysis , characterized by the expectoration of 100 ml of fresh blood a day later . an urgent bronchial arteriogram and embolization ( siemens , axiom artis dba , germany ) was arranged in view of the profuse hemorrhage . next , the catheterization of the left bronchial artery was performed with a 4.1f shk catheter ( bjaeverskov , denmark ) , and demonstrated hypertrophied arteries in the lower lobe of the left lung with an arterio - venous shunt ( fig . after excluding the spinal medullary artery supply , an embolization of the left bronchial artery was performed with two vials of 500 - 700 m crossed - linked tris - acryl microspheres ( embosphere ) ( guerbet biomedical ; louvres , france ) . special care was taken to prevent reflux by injecting the embospheres slowly , and under continuous fluoroscopic control . a computerized tomography ( ct ) scan of the abdomen ( general electric lightspeed vct ; waukesha , wi ) showed multiple micro - infarctions of the spleen and bilateral kidneys ( fig . the patient 's renal function deteriorated with a serum creatinine level of 157 mol / l ( reference range : 60 - 120 the treatment resulted in the complete resolution of abdominal pain and improved renal function ( serum creatinine level : 132 mol / l ) . since the patient was completely asymptomatic , she declined to undergo a follow - up ct scan of the abdomen . however , it has the disadvantage of causing the recanalization of the embolized artery , which may result in the recurrence of bleeding . coils are generally not used for baes because they tend to occlude more proximal vessels and may preclude further embolization if hemoptysis recurs . polyvinyl alcohol particles are a nonabsorbable embolic material with particles of 350 - 500 m in diameter that are frequently used as smaller particles which can pass through the bronchopulmonary anastomosis which result in pulmonary or systemic infarctions ( 2 ) . a more recent introduction of the crossed - linked trisacryl microspheres ( embospheres ) , have a more uniform size and better penetration than polyvinyl alcohol particles ( 3 ) . particle sizes of 300 - 500 and 500 - 700 m have been used for baes ( 4 ) . however , the better penetration characteristics of the crossed - linked tris - acryl microspheres may result in a greater tendency to cross bronchial artery - pulmonary artery shunts or bronchial artery - pulmonary vein shunts , which cause pulmonary infarctions or systemic infarcts as in our patient . to the best of our knowledge , only three other previous reports of systemic infarcts following baes with microspheres exist in the literature . the authors suggested that the embosphere particles had crossed the bronchiopulmonary shunt into the systemic circulation . two other similar cases were also reported by fitztgerald et al . ( 6 ) and sriram et al . the microinfarction of the kidneys and spleen were self - limited in our patient , which was consistent with sriram et al . , we have to be aware of this potential complication when using microspheres as the embolic material in baes . since the embosphere particles show better penetration than polyvinyl alcohol particles , choosing a size of embosphere based on experience with polyvinyl alcohol particles could result in significant distal embolization as a result of shunting through the lesion ( 3 ) . we propose that in the presence of bronchial artery - pulmonary artery shunts or bronchial artery - pulmonary vein shunts , the use of larger sized crossed - link tris - acryl microspheres ( 700 - 900 m ) may avoid this complication .

a bronchial artery embolization ( bae ) is an important therapeutic method used to control acute and chronic hemoptysis . we report a case of multiple micro - infarcts involving both the kidneys and spleen , following a bae with 500 - 700 m crossed - linked tris - acryl microspheres ( embospheres ) in a patient with bronchial artery pulmonary vein shunts . the superior penetration characteristics of the microspheres may have resulted in the greater tendency to cross the bronchial artery pulmonary vein shunts , which subsequently caused the systemic infarcts in our patient . we propose the use of larger sized microspheres ( 700 - 900 m ) , which may aid in avoiding this complication .

antithyroid medications are still widely used in younger patients with mild disease , and pregnant or lactating patients . it has several side effects ; the majority are mild and reversible such as allergic reaction , and upper gi disturbances . other rare side effects include agranulocytosis- and vasculitis - like reaction , particularly with propylthiouracil . hepatic toxicity is a rare , but serious side effect with both methimazole ( and its pro - drug , carbimazole ) and propylthiouracil ( ptu ) . the hepatic histopathological findings with ptu are toxic hepatitis with necrosis , whereas , they resemble cholestatic hepatitis with methimazole ( and carbimazole ) . we present a case of carbimazole - induced cholestatic hepatitis in a patient with graves disease . we describe the clinical and biochemical findings in this patient and review the relevant literature . a 30-year - old male patient presented to the outpatient clinic of the endocrinology department with chief complaints of a 6 kg weight loss over the last four months and bilateral prominent and bulging eyes . he was a known case of rheumatic heart disease with mitral stenosis under treatment with salt restriction , digoxin , and blockers . in the last four months he was admitted thrice , with episodes of atrial fibrillation and congestive heart failure . pulse rate was 120 beats / minute , regular , and blood pressure was 150 / 70 mmhg in the right arm supine position . there was diplopia on looking to both sides , indicating ophthalmoplegia amounting to stage iv nospecs classification . cardiovascular system examination revealed loud first heart sound and mid - diastolic murmur , with delayed peaking at the mitral area . his initial t4 was 22.4 g / dl and tsh < 0.01 uiu / ml . he was diagnosed with graves disease and started on propranolol with prednisolone for graves ophthalmopathy . carbimazole was also initiated at a dose of 30 mg per day , in two divided doses . he neither did a thyroid scan nor took i because of concern with iodine allergy and exacerbation of the eye manifestations . three weeks later , ft4 was normal and tsh was < 0.03 uiu / ml . he complained of nausea and vomiting , and the accompanying attendants complained of yellowish discoloration of his eyes . hepatitis serology was negative for hepatitis a , b , c , and e. as per the gastroenterologist 's advice , a liver biopsy was done , which revealed cholestatic hepatitis with a paucity of bile ducts [ figure 1 ] . he received radioactive iodine under the cover of prednisolone , to minimize the risk of eye sign deterioration . he developed post - radioiodine hypothyroidism and was started on thyroxine therapy . upon re - evaluation after four and six months his liver functions were still normal . all antithyroid drugs ( methimazole , carbimazole , and propylthiouracil ) can affect the liver on rare occasions . a cohort study on 50 patients by liaw found that subclinical liver injury is common during ptu treatment . thirty percent of the patients had a transient , asymptomatic rise in the aminotransferase level , with no elevation in bilirubin after two months of therapy . despite continuation of ptu , he was suggested cautious continuation of ptu in the absence of symptoms and hyperbilirubinemia . despite this carbimazole and methimazole , on the other hand have been typically associated with cholestatic jaundice ( mainly hyperbilirubinemia ) without evidence of hepatic necrosis on liver biopsy . nevertheless , there are occasional reports of severe and fatal cases with methimazole - induced liver disease . a review of the literature has revealed around 22 cases of cholestatic jaundice due to both methimazole and carbimazole . the proposed mechanism of carbimazole - induced cholestasis is not fully understood , but it is thought to be an allergic reaction . a cross - reaction between ptu and carbimazole is still a concern , when a switch from one to another is considered , in spite of some reports to the contrary . our patient developed significant hyperbilirubinemia within four weeks of starting carbimazole , despite normalization of the thyroxin level . it continued to rise for one more week after stopping the offending drug , until complete normalization over two months . the aminotransferase level was mildly elevated , reaching two - to - three times the normal . although the patient received steroids from the start for reasons discussed earlier , it did not affect the course of the liver disease . in fact , previous reports of using steroids in methimazole - induced hepatitis did not show any benefit . routine liver function tests during therapy are not cost - effective , but must be performed when this complication is suspected . the drug must be withdrawn immediately and alternative therapy for hyperthyroidism , such as radioiodine , must be considered .

antithyroid medications are one of the treatment options for graves disease . carbimazole is widely used as the drug of choice , except in pregnancy , where propythiouracil is preferred by many . it is generally well - tolerated . its side - effects include allergy , upper gastrointestinal upset , a rare occurrence of granulocytosis , and others . hepatitis is another rare , but serious side - effect . we report a healthy 30-year - old male patient with graves disease , who developed cholestatic jaundice after carbimazole therapy for four months . he made a full recovery after the drug was discontinued . an idiosyncratic mechanism seemed likely .

bartter syndrome is a rare autosomal recessive disorder , manifested in childhood or in the perinatal period with severe hypokalemia , metabolic alkalosis , and low - normal blood pressure . it is due to primary defect in sodium chloride reabsorption in the medullary thick ascending limb of the loop of henle . bartter syndrome is caused by inactivating mutation of each of its major transport proteins nkcc2 , romk , clc - kb , and bartin which are typed as bartter syndrome 1 , 2 , 3 , and 4 , respectively . gentamicin - induced renal tubular dysfunction may present as a acquired bartter - like syndrome ( bs ) and to the best of our knowledge , only a few cases are reported in literature . a 26-year - old male was admitted with 6-day history of high - grade fever followed by productive cough who was diagnosed as a case of the right - sided pneumonitis . investigations showed increased white cells count ( 15,100/cmm ) with normal serum electrolytes , renal profile , and liver function test . on the 7 day of admission , the patient complained of nausea and generalized weakness . in the evening he had one episode of generalized tonic - clonic seizure . on the initial evaluation , his vital signs were blood pressure 124/70 mmhg , pulse rate 104/min , temperature 98.6f , and respiratory rate 18/min . he was managed immediately with intravenous ( iv ) diazepam 10 mg bolus dose and iv phenytoin at a loading dose of 15mg / kg followed by 100 mg 8 hourly . the blood gas analysis revealed ph 7.54 , pco2 42 mmhg , po2 114 mmhg , so2 98.8% , sodium 112 mmol / l , potassium 2.2 mmol / l , bicarbonate 38 mmol / l , and calcium ( ca ) ( ionic ) 0.82 mmol / l . his ultrasonography revealed normal kidneys . computed tomography of the head detected no abnormality , and cerebrospinal fluid cerebrospinal fluid analysis was normal . we noted increase in urine output ( > 5 l/24 h ) after an episode of seizure . spot urine found k 13 ( 3.5 - 5.0 mmol / l ) and na 129 ( < 20 mmol / l ) . twenty - four hours urine collection revealed ca of 475 mg ( 50 - 300 ) . blood chemistry showed gross electrolytes abnormalities , suggestive of metabolic encephalopathy , and the seizure episode could be due to severe hypocalcemia and/or hyponatremia . these deranged parameters as a result of severe renal salt wasting were due to defective renal tubular concentrating ability . approximately , 8%26% of patients receiving an aminoglycoside for several days will develop mild renal impairment which is almost always reversible . gentamicin is excreted by glomerular filtration , and toxicity is due to the the accumulation of drug in porphyria cutanea tarda porphyria cutanea tarda cells . the drug interferes with cellular energetic , impares intracellular phospholipase , and induces oxidative stress which in turn results in cell destruction . however , mitochondrial dysfunction is hypothesized for underlying abnormalities in all parts of the renal tubule . thus , aminoglycoside - induced renal tubular dysfunction may present as fanconi - like syndrome , bs , and distal renal tubular acidosis . the exact pathways culminating in aminoglycoside - induced bs is not well known and may be similar to the hereditary variant of bartter 's disease which involves a transporter defect situated in the thick ascending loop of the renal tubule . it is hypothesised that gentamicin , a polyvalent cationic molecule , directly activates the ca sensing receptor in the thick ascending loop of henle and the distal tubule , which results in renal wasting of sodium , potassium , chloride , ca , and magnesium . the risk of renal tubular dysfunction is related to a prolonged and inappropriately high - dose aminoglycosides use . cases of transient bartter 's syndrome associated with aminoglycoside use have been described to recover after 26 weeks following cessation of the antibiotics . the presentation of our patient ( metabolic alkalosis , refractory hypokalemia , hypocalcemia hypomagnesemia , and hypercalceuria ) was suggestive of bs while other close differential diagnosis , gitelman syndrome was ruled out by the the presence of low serum ca level and documentation of hypercalciuria . patients on gentamicin therapy should be monitored . routinely for electrolyte imbalances to avoid potential morbidity and life - threatening condition .

aminoglycoside nephrotoxicity may manifest as nonoliguric renal failure or tubular dysfunction , such as fanconi - like syndrome , bartter - like syndrome ( bs ) , or distal renal tubular acidosis . we report a case who developed severe renal tubular dysfunction on the the 7th day of gentamicin therapy , resulting in metabolic alkalosis , refractory hypokalemia , hypocalcemia , hypomagnesemia , and polyuria . the patient was diagnosed as a case of transient bs associated with gentamicin exposure . the patient recovered with conservative management .

although the characteristic clinical appearance and the differences in distribution , it is often difficult to differentiate keratosis follicularis squamosa ( dohi ) from other keratotic disorders . here we reported in the first time that by using dermoscopy , atypical clinical appearance in dohi became more obvious . a 5-year - old japanese boy presented with a 1-year history of asymptomatic scaly eruptions symmetrically scattered on the trunk and thighs ( figure 1a ) . physical examination revealed small scattered patches whose margin is slightly detached from the underlying epidermis , but a typical brown - coloured follicular plug in the centre was not obvious ( figure 1b ) . however , dermoscopic observation without echo - jelly remarkably showed typical lotus leaves on the water appearance ( figure 2a ) . moreover with echo - jelly , even though lotus leaves on the water appearance can not observe , a typical brown - coloured follicular plug in the center and fatty hair which has larger diameter than normal area became obvious by dermascopy ( figure 2b ) . histopatholo - gy showed dilated follicles with keratotic plugs and orthohyperkeratosis ( figure 2c ) . treatment with 20% uric acid containing cream led to clinical disappearance of the lamellar scaling improved . b ) typical lotus leaves on the water appearance are seen , but a typical brown - coloured follicular plug in the centre was not obvious . b ) typical lotus leaves on the water appearance are seen , but a typical brown - coloured follicular plug in the centre was not obvious . a ) observation without echo - jelly remarkably represent typical lotus leaves on the water appearance . b ) observation with echo - jelly showed a typical brown - coloured follicular plug in the center and delicate pigment network around it . c ) pathological findings of the lesion . a dilated hair follicle with keratotic plugging and surrounding epidermis with orthohyperkeratosis ( haematoxylin and eosin , original magnification x100 ) . a ) observation without echo - jelly remarkably represent typical lotus leaves on the water appearance . b ) observation with echo - jelly showed a typical brown - coloured follicular plug in the center and delicate pigment network around it . c ) pathological findings of the lesion . a dilated hair follicle with keratotic plugging and surrounding epidermis with orthohyperkeratosis ( haematoxylin and eosin , original magnification x100 ) . dohi , characterized by 103 mm diameter scaly patches scattered on the trunk , is a keratinizing disorder first described by dohi and momose in 1903 . its asymptomatic scaly patches with a margin that is slightly raised from the underlying epidermis was originally described as lotus leaves on the water . a tiny pigmented papule is present in the center of the lesion . this is histopathologically a dilated hair follicle with a follicular plug surrounded by lamellar orthohyperkeratosis . dohi is , thus , well recognized in japan as a separate clinical entity from keratosis follicularis ( darier 's disease ) . but it often difficult to differentiate keratosis follicularis squamosa ( dohi ) from other keratotic disorders when the characteristic feature of cutaneous findings are not obvious .

although the characteristic clinical appearance and the differences in distribution , it is often difficult to differentiate keratosis follicularis squamosa ( dohi ) from other keratotic disorders . here , we describe the case of a 5-year - old boy with dohi in the first time that by using dermoscopy . dermoscpoy of the lesion showed typical lotus leaves on the water appearance and follicular plug in the center . we reported the usefulness of dermosocopy in dohi .

lipoleiomyoma is a very rare lesion of the uterus occurring primarily in obese perimenopausal and post menopausal patients . the tumor consists of long intersecting bundles of bland , smooth muscle cells admixed with nests of mature fat cells and fibrous tissue.[24 ] we report a case of lipoleiomyoma that arose in the uterus . a 50-year - old postmenopausal woman presented with increased frequency of per vaginal bleeding since 6 months and distension of abdomen since 15 days . the patient 's history revealed that she had attained menarche at the age of 14 years , had regular menstrual cycles of 45 days duration and moderate intensity at 28 days interval . gynecological examination revealed no abnormalities of the vulva , cylindrical vaginal portion of the cervix and no evident pathological change was detectable with clinical examination . findings of ultrasonography examination suggested bulky uterus with thickened endometrium of 6 mm and hyperechoic mass suggestive of myoma of posterior wall of uterus , measuring 3.5 cm in diameter . in addition , transvaginal sonography revealed hyperechoic lesion of 3.5 3.5 cm in the posterofundal region . both the ovaries showed follicular cysts measuring 3.3 3.3 cm each and tubes were normal in appearance . the patient underwent total abdominal hysterectomy with bilateral salpingo - oophorectomy because of multiple leiomyomas . on gross examination of the specimen , the uterus measured 8.5 6.1 5 cm and had three intramural and subserosal well - circumscribed round masses . the biggest nodule which was measuring 3.5 cm in diameter differed from a typical appearance of uterine leiomyoma by being pale yellow and having a somewhat softer consistency on its cut surface [ figure 1 ] . the other two leiomyomas , each of 0.5 cm diameter , showed a coarsely whorled pattern with grayish white appearance on their cut surface . sectioned surface of uterine lipoleiomyoma showing distinct pale yellow appearance histological examination of the biggest nodule showed a mixture of bland , spindle - shaped smooth muscle cells without nuclear atypia in a whorled pattern with admixed mature adipocytes . the nuclei of the smooth muscles were elongated and had finely dispersed chromatin and small nucleoli . between these muscle cells , sections from both the ovaries showed follicular cysts with the tubes being unremarkable histologically . well - circumscribed proliferation of bland , spindle - shaped smooth muscle cells without nuclear atypia in a whorled pattern with admixed mature adipocytes ( h and e , 10 ) smooth muscle cell proliferation admixed with mature adipocytes ( h and e , 40 ) these tumors showed characteristic histological findings , being composed of benign smooth muscle and mature adipose tissue . it is suggested that lipoleiomyomas result from fatty metamorphosis of uterine smooth muscle cells which can proceed to form localized or diffuse mature adipocyte tissue in leiomyoma or in the myometrium rather than fatty degeneration . the differential diagnosis of the lipomatous mass in the pelvis includes benign cystic teratoma , malignant degeneration of cystic teratoma , non - teratomatous lipomatous ovarian tumor , benign pelvic lipomas , liposarcomas and lipoblastic lymphadenopathy . association of lipomatous uterine tumors and endometrial carcinomas with lipoleiomyosarcoma arising in uterine lipoleiomyomas has been reported . immunocytochemical studies confirm the complex histogenesis of these tumors , which may arise from mesenchymal immature cells or from direct transformation of smooth muscle cells into adipocytes . a number of various lipid metabolic disorders or other associated conditions , which are associated with estrogen deficiency as occurs in peri or post menopausal period , possibly promote abnormal intracellular storage of lipids . so , it is important to differentiate these tumors from ovarian teratoma , which requires surgical excision . though imaging plays an important role in preoperative diagnosis and localization of the lipoleiomyoma , it is the final pathological examination that confirms the diagnosis .

lipoleiomyomas are uncommon benign neoplasms of uterus and are considered to be a variant of uterine myomas . their reported incidence varies from 0.03 to 0.2% . lipoleiomyoma consists of variable proportion of mature lipocytes and smooth muscle cells . these tumors generally occur in asymptomatic obese perimenopausal or menopausal women . we report this case of uterine lipoleiomyoma because of its rarity .

surgical aortic valve replacement ( avr ) remains the cornerstone of treatment for symptomatic critical aortic stenosis ( as ) . it is rewarding for most of the patients , but it can have several associated complications . it was first described by roberts and morrow in 1967.1 it is estimated to occur in about 15% of all avr procedures.26 the exact cause of coronary ostial stenosis is still undetermined , but few hypotheses have been proposed.5 symptoms are severe and generally appear within 16 months postoperatively . coronary artery bypass grafting ( cabg ) is the typical treatment , but percutaneous coronary intervention ( pci ) has also yielded favorable outcomes.711 we present a case of left main coronary ostial stenosis 11 months after avr was done for symptomatic critical as , which was successfully managed with primary stent placement . in june 2015 , a 67-year - old female with a long - standing history of asymptomatic critical as was referred for avr after new onset of shortness of breath . a preoperative transesophageal echocardiogram showed aortic valve area of 0.6 cm with 2 + mitral regurgitation . cardiac catheterization revealed no significant coronary artery disease and well - preserved left ventricular ejection fraction . repeat coronary angiography revealed 99% narrowing of left main coronary artery ( lmca ) ( fig . 4 ) . the left anterior descending artery , left circumflex , and right coronary artery ( rca ) did not reveal any discrete stenosis . after consultation with the cardiac surgeon and the family , the patient underwent urgent primary stent deployment of lmca ( fig . a successful placement of intra - aortic balloon pump was also done in the left femoral artery since this was a high - risk procedure . coronary ostial stenosis after avr occurs most commonly in the lmca , but rca can also be affected.35 exact pathophysiologic mechanism of coronary ostial stenosis has not been determined . roberts and morrow1 discovered avr fibrous thickening in the aortic root and proximal coronary artery in autopsy subjects . no evidence of atherosclerosis was seen on histological examination.5 intimal thickening and fibrosis near the aortic root have been proposed to be due to turbulent flow around the prosthetic valve leading to obstruction of the coronary ostia.1 there may be microinjury and local hyperplastic reaction due to cardioplegic fluid and over dilation of the vessel by the tip of the catheter during avr , and tukiji et al.12 have suggested immunologic reaction to the bioprosthesis.13 coronary ostial stenosis after avr generally presents as unstable angina , left ventricular heart failure , ventricular arrhythmias , or sudden death.7 high clinical suspicion is crucial . cabg may not always be a safe option , mainly due to the proximity of the graft site to the area of prior operation.7 high incidence of perioperative infarction , increased mortality , and poor long - term outcome have been reported with cabg.2,3 several reports have shown good early and late outcomes with pci and stent implantation.5,6,12,13 though rare , coronary ostial stenosis after avr should be suspected in patients presenting with new relevant symptoms . mostly , such cases have been reported to occur within 16 months after avr , but it can manifest even later , as evident by our case . thorough history and physical examination and timely coronary angiography should be performed if suspicion of coronary ostial stenosis is high . it can be fatal due to potential complications of acute coronary syndrome , left ventricular heart failure , or ventricular arrhythmias . immediate reperfusion strategies are crucial in management . although cabg is considered to be the typical treatment , pci has shown to have good short - term results .

coronary ostial stenosis is a rare but potentially life - threatening complication of aortic valve replacement ( avr ) . it can present potential complications such as acute coronary syndrome , left ventricular failure , ventricular arrhythmias , or sudden death . exact pathogenetic mechanism is not known , but a few hypotheses have been proposed . we present a case of left main coronary stenosis 11 months after avr was done for symptomatic critical aortic stenosis . high index of clinical suspicion and timely coronary angiography helped to determine the exact etiology . a percutaneous coronary intervention was done emergently . the patient has done very well so far .

cbct scanning was performed using a ray - scan symphony scanner ( ray medical system , seoul , korea ) in the department of oral and maxillofacial radiology of the yonsei university dental hospital . the sagittal and cross - sectional images were reconstructed from the volumetric cbct data using the imaging analysis software ondemand 3d ( cybermed , seoul , korea ) . a 39-year - old male visited the yonsei university dental hospital for extraction of the left mandibular third molar . on a sagittal cbct image , a curving branch of the mandibular canal with a recurrent path at the right retromolar area was found . the passage of rmc , which branches from the mandibular canal , ended in a round radiolucent foramen of the retromolar fossa ( rmf ) as it moved superiorly approaching the most posterior molar area ( fig . 1c ) . a 30-year - old male visited the yonsei university dental hospital with a chief complaint of inflammation in the right mandibular third molar area . routine panoramic radiography and cbct scans were performed for the extraction of the right third molar . it was observed that there was rmc branching from the mandibular canal that passed superiorly and in the reverse direction of the right retromolar area on sagittal images of cbct ( fig . two radiolucent canals surrounded by cortical bone were found on cross - sectional images perpendicular to the alveolar ridge ( fig . a 39-year - old male visited the yonsei university dental hospital for extraction of the left mandibular third molar . on a sagittal cbct image , a curving branch of the mandibular canal with a recurrent path at the right retromolar area was found . the passage of rmc , which branches from the mandibular canal , ended in a round radiolucent foramen of the retromolar fossa ( rmf ) as it moved superiorly approaching the most posterior molar area ( fig . a 30-year - old male visited the yonsei university dental hospital with a chief complaint of inflammation in the right mandibular third molar area . routine panoramic radiography and cbct scans were performed for the extraction of the right third molar . it was observed that there was rmc branching from the mandibular canal that passed superiorly and in the reverse direction of the right retromolar area on sagittal images of cbct ( fig . two radiolucent canals surrounded by cortical bone were found on cross - sectional images perpendicular to the alveolar ridge ( fig . analyses of the rmf using cadavers or dry mandibles have been inconsistent , with values ranging from 6.1% to 72.0%.1,11,15,16 the rmc is often classified as a subtype of the bifid mandibular canal in radiological studies.9,10,13,17 because a rmc is generally very narrow,14 conventional radiography is not reliable in detecting rmc . however , cbct can be used to confirm a number of anatomical variations of the mandibular canal that can not be assessed with conventional imaging.7,12,13 recent studies of the rmc using cbct showed higher occurrence rates of 14.6%-65.3%7,13,18,19 than did those using panoramic radiography . the incidence of rmcs has been found to vary among different populations.1,19 some authors have demonstrated that a neural branch to the mandibular molars arises from the mandibular canal,2,3,6 while the distal end of the rmc reaches the third mandibular molar and retromolar area . others have reported that an aberrant buccal nerve originating from the inferior alveolar nerve within the ramus of the mandible , which runs through the rmc , can end up in the rmf and pass anterosuperiorly to be distributed into the buccinator muscle.4,5 further studies are required to verify this buccal nerve connection . with regard to the courses of the rmc , 3 types of rmcs were recognized by ossenberg.1 type 1 is a vertically curved rmc that branches from the mandibular canal around the molars from a single mandibular foramen , type 2 was a horizontally curved rmc that branches from the mandibular canal just beyond the single mandibular foramen , and type 3 is an rmc with separate foramina in the mandibular ramus that runs independently of the main mandibular canal . some researchers7,18 have generally focused on the most common type of rmc ( type 1 ) proposed by ossenberg . in conclusion , therefore , the clinician must pay particular attention to the identification of a rmc by preoperative radiographic examination , and additional cbct scanning is recommended .

a retromolar canal is an anatomical variation in the mandible . as it includes the neurovascular bundle , local anesthetic insufficiency can occur , and an injury of the retromolar canal during dental surgery in the mandible may result in excessive bleeding , paresthesia , and traumatic neuroma . using imaging analysis software , we evaluated the cone - beam computed tomography ( ct ) images of two korean patients who presented with retromolar canals . retromolar canals were detectable on the sagittal and cross - sectional images of cone - beam ct , but not on the panoramic radiographs of the patients . therefore , the clinician should pay particular attention to the identification of retromolar canals by preoperative radiographic examination , and additional cone beam ct scanning would be recommended .

hirsutism is defined as excess growth of terminal hair in females , distributed in an androgen - dependent pattern . it is present in approximately 7% of women , and can lead to severe implications on their quality of life . while several causes have been implicated in hirsutism development , androgenic factors are the most prevalent , accounting for more than 80% of the cases , and polycystic ovary syndrome is the most common sole disorder for hirsutism . iatrogenic causes for hirsutism , including medications , are considered as an uncommon cause , although the exact incidence is not well defined . we present a case of severe hirsutism developing after treatment with isotretinoin , a widely prescribed drug in dermatology practice . her past medical history included thymoma that was treated with irradiation 10 years prior to her current consultation . the patient was diagnosed with papulopustular rosacea , and treatment was initiated with isotretinoin 20 mg daily for 1-month . following good clinical response and lack of side - effects , the dose was increased to 30 mg daily . two months later , the patient developed excess hair growth on her face , located mainly to the temples and the forehead . the patient denied other symptoms of virilization , including change of voice or growth of hair in other locations . androgen levels ( including testosterone , dehydroepiandrosterone sulfate and 4 androstenedione ) were within the normal limits . physical examination revealed the presence of coarse black hairs on her bilateral temples and sides of the forehead [ figure 1 ] . coarse black hair on the temples ( a ) and side of the forehead ( b ) in a patient treated with isotretinoin for rosacea isotretinoin is a widely prescribed medication in dermatology practice , mainly for the treatment of severe nodulocystic acne . while hirsutism is considered to be one of the possible side effects of isotretinoin therapy , its exact frequency is not defined . to the best of our knowledge , only one case has been reported before , and such occurrence has not been reported in the dermatology literature . our patient did not show additional features of hyperandrogenism , and a recent study also demonstrated that isotretinoin therapy does not seem to alter androgen levels in acne patients . interestingly , the distribution of excess facial hair is reminiscent of the distribution of hirsutism seen in females following the topical application of minoxidil as a treatment for androgenetic alopecia . furthermore in these cases , the most common sites for excessive hair growth are the forehead and sideburns . an additional patient that was observed by us , a 22-year - old female patient who was treated with isotretinoin for cystic acne , also developed facial hirsutism on her forehead , chin , and cheeks [ figure 2a ] . in this case , facial hirsutism resolved spontaneously several months after cessation of isotretinoin treatment [ figure 2b ] . facial hirsutism in a 22-year - old female patient treated with isotretinoin for cystic acne ( a ) , that resolved spontaneously several months after cessation of treatment ( b ) there are no conflicts of interest .

hirsutism is usually a manifestation of hyperandrogenism , and iatrogenic causes for excess hair growth are uncommon . here , we report on a 48-year - old female patient , who developed severe excess facial hair following treatment with isotretinoin for papulopustular rosacea . to the best of our knowledge , only one case has been reported before , and not in the dermatology literature . taking into consideration the fact that isotretinoin is a widely prescribed medication in the dermatology practice , information on its possible adverse effects is of major importance for the treating dermatologist .

acyclovir is a unique antiviral agent for the treatment of herpes virus infections including herpes simplex encephalitis . local adverse effects are common and typically consist of inflammation or phlebitis at the site of intravenous infusion . here we present a child with bullous eruptions away from infusion site caused by intravenous acyclovir treatment . bulla formation away from injection site is exceptionally rare , with only one adult case reported to date . a 15-year - old boy was admitted to pediatric intensive care unit for status epilepticus . since several combinations of antiepileptics failed to control the status , intravenous acyclovir infusion ( 30 mg / kg / day ) was initiated with the presumptive diagnosis of herpes encephalitis . at the fifteenth day of treatment , a necrotic and bullous eruption occurred on the left leg adjacent to the infusion site [ figure 1 ] . the infusion was suspended and the next dose of acyclovir was diluted and delivered to the vein on the right hand . nevertheless , bullous eruptions re - occurred on the right hand and on the right ear auricle [ figure 2 ] , which was far from the infusion site . the results of laboratory evaluation including total blood count , peripheral smear , erythrocyte sedimentation rate , c - reactive protein , biochemical tests , immunoglobulin levels , complement 3 and 4 levels , ana , anti dna and herpes simplex virus ( hsv ) antibodies were negative . histopathology of skin biopsy from the left leg revealed subcorneal bulla formation , necrotic keratinocytes in epidermis , perivascular and periadnexial lymphocytes , nuclear remnant , sparse neutrophils and eosinophils , squamous metaplasia in the sweat gland in the dermis . he was concomitantly receiving meropenem , vancomycin , variconazole , and ciprofloxacin for ventilator - associated pneumonia and levetiracetam , phenobarbital , haloperidol , clonazepam for the status condition . the patient had never received any acyclovir treatment ( topical , oral or intravenous ) before . further support for this causal relationship was obtained by naranjo 's algorithm which suggested a bulla and necrosis on patient 's left leg distant bullous eruptions on patient 's ear causality analysis by naranjo 's algorithm adverse cutaneous reactions have been reported with all routes of administration . local inflammation or phlebitis at the injection site are well - known dermatologic reactions of intravenous acyclovir therapy , but vesicular - bullous eruptions are rarely reported . although hypersensitivity , local drug toxicity , or damage associated with the extravasation of the alkaline solution were proposed to be responsible for the mechanism of these bullous eruptions , it still remains unclear . diluting the concentration of acyclovir did not prevent the development of new lesions , phlebitis and the distant bullous eruptions in our patient as reported by buck et al . armingaud et al . , has reported the only case of localized bullous eruption at and far from the acyclovir injection site in a 50-year - old man . they proposed an immunoallergic mechanism to explain the pathogenesis of the lesion instead of the toxic mechanism . we were not able to take a biopsy from the distant lesion on the right ear because the parents refused to give consent for the procedure . however , we suggest that an adverse reaction to acyclovir should be included in the differential diagnosis of vesicular eruptions in patients receiving acyclovir therapy . this may occur regardless of the route used or the concentration administered . distant bullous lesions should be considered as a potential adverse reaction caused by intravenous acyclovir treatment .

acyclovir is an antiviral agent against herpes virus . its local adverse effects are common and typically consist of inflammation or phlebitis at the site of intravenous infusion . here we present a child with bullous eruptions away from infusion site due to acyclovir administration . it is exceptionally rare with only one adult case has been reported to date .

this genus comprises gram - negative , strictly aerobic , nonfermenting , nonfastidious , nonmotile , catalase - positive , oxidase - negative bacteria with a dna g + c content of 39% to 47% . according to euzeby 's list of prokaryotic names with standing in nomenclature ( http://www.bacterio.cict.fr/a/acinetobacter.html ) acinetobacter haemolyticus was proposed by bouvet and grimont 1986 ; it was isolated from human clinical specimens and environment , with characteristics corresponding to those of the genus acinetobacter . the organism in this study is acinetobacter haemolyticus strain mtcc 9819 equivalent to dsm 6962 (= atcc 17906 , a. haemolyticus strain mtcc 9819 was obtained from mtcc and grown on tryptic soya agar medium ( tsa ; himedia ) at 30 c . genomic dna was extracted from 36 hour old culture using zr fungal / bacterial dna miniprep as per manufacturer 's instructions . the 16s rrna sequence consisting of 1502 bp was compared with those of type strains of species of related genera and identification of phylogenetic neighbors and the calculation of pairwise 16s rrna gene sequence similarities were achieved using the eztaxon server and aligned using mega version 5.0 . bootstrap analysis was performed to assess the confidence limits of the branching ( fig . 1 . the genome of a. haemolyticus strain mtcc 9819 was sequenced using illumina - hiseq 1000 technology . sequencing resulted in 5,755,416 paired - end reads ( insert size of 350 bp ) of length 101 bp . a total of 5,580,067 high - quality reads with approximately 166 coverage were assembled with clcbio wb6 ( word size = 50 and bubble size = 100 ) and to obtain 182 contigs ( n50 , 57,666 bp ) of 3,384,229 bp with an average gc content of 40% . the functional annotation was carried out by rast ( rapid annotation using subsystem technology ) , fig . 2 shows the subsystem distribution of strain a. haemolyticus strain mtcc 9819 , and trna was predicted by trnascan - se 1.23 and rrna genes by rnammer 1.2 . the genome contains 3 rrna genes ( 5s , 23s , 16s ) and 65 aminoacyl - trna synthetase genes . a total of 3122 coding regions ( 1546 transcribed from the positive strand and 1576 from the negative strand ) were found in the genome , of which 2298 ( 74% ) could be functionally annotated . the genome coding density is 87% with an average gene length of 911 bp . the annotated genome has 65 genes responsible for resistance to antibiotic and toxic compounds including 13 genes for mdr efflux pumps . forty four genes are involved in response to oxidative stress , 10 for osmotic stress response and 16 genes for heat shock and many more stress responses , all summed up to 94 genes for stress response are present . the functional comparison of genome sequences available on the rast server revealed the closest neighbors of a. haemolyticus strain mtcc 9819 as acinetobacter junii sh205 ( score 503 ) followed by acinetobacter baumanii acicu ( score 489 ) , acinetobacter haemolyticus atcc 19194 ( score 476 ) and acinetobacter baumanii ab0057 ( score 453 ) . the a. haemolyticus strain mtcc 9819 whole genome shot gun ( wgs ) project has been deposited at ddbj / embl / genbank under the project accession asyx00000000 of project ( 01 ) which has the accession numbers asyx01000000 and consists of sequences asyx01000001 asyx01000182 . the authors declare that there is no conflict of interest on any work published in this paper .

the genus acinetobacter consists of 31 validly published species ubiquitously distributed in nature and primarily associated with nosocomial infection . we report the 3.4 mb genome of acinetobacter haemolyticus strain mtcc 9819 t . the genome has a g + c content of 40.0% and includes 3 rrna genes ( 5s , 23s , 16s ) and 65 aminoacyl - trna synthetase genes .

hemimegalencephaly is a rare congenital neuronal migration disorder characterized by unilateral hypertrophy of a cerebral hemisphere and ipsilateral ventriculomegaly . it can be an isolated abnormality , or it can be associated with neurocutaneous syndromes . clinical manifestations include intractable motor seizures , impaired psychomotor development , hemiparesis , and hemianopsia . the definition of the dysfunctional areas of dysplastic cortex may affect surgical management of these anomalies . evaluation of regional cerebral blood flow using technetium-99 m ethyl cysteinate dimer ( tc-99 m ecd ) single - photon emission computed tomography ( spect ) provides useful information in the patient of hemimegalencephaly because it clearly delineates the border of the dysplastic cortex by showing altered blood flow to the dysfunctional areas . an 11-year - old male child was referred to a tertiary care hospital with history of gradually increasing seizures frequency since infancy . seizures were mainly right sided , complex partial in nature and refractory to medical treatment over the past decade . the child was born out of non - consanguineous marriage , at term with a normal vaginal delivery . non - contrast computed tomography ( ct ) of head showed diffuse enlargement of left cerebral hemisphere with midline shift towards right while the right cerebral hemisphere was normal [ figure 1a ] . t2w magnetic resonance imaging ( mri ) images showed diffuse gyral thickening involving the left cerebral cortex causing midline shift and scalloping of inner table of left calvarium [ figure 1b ] . linear hyperintensities involving the subcortical u fibers of left fronto - parietal region were seen on the fluid attenuation inversion recovery image [ figure 1c ] . post - gadolinium t1w image in axial plane showed no abnormal enhancement [ figure 1d ] . tc-99 m ecd brain perfusion spect revealed diffusely decreased tracer uptake in the left cerebral hemisphere , with normal uptake in the right hemisphere suggestive of left cerebral hemihypoperfusion [ figure 2a - c ] . non - contrast computed tomography of head ( a ) axial shows diffuse enlargement of left cerebral hemisphere ( * ) with midline shift towards right while the right hemisphere is normal . non - contrast magnetic resonance imaging t2w ( b ) images ( axial ) show diffuse gyral thickening ( thin arrow ) in the left cerebrum causing midline shift and scalloping of inner table ( arrow head ) of left calvarium . linear hyperintensities involving the subcortical u fibers of left fronto - parietal region seen on fluid attenuation inversion recovery image ( c ) post - gadolinium t1w image in axial plane ( d ) shows no abnormal enhancement 99 m technetium - ethyl cysteinate dimer brain perfusion single - photon emission computed tomography transaxial , coronal and sagittal images reveal diffusely decreased tracer uptake in the left cerebral hemisphere ( arrow ) , with normal uptake in the right hemisphere . tc-99 m ecd is a perfusion agent similar to tc-99 m hexamethylpropyleneamine oxime ( hmpao ) . regional differences in perfusion within the affected hemisphere with relative interictal hypoperfusion and ictal hyperperfusion of the seizure focus in a neonate with hemimegalencephaly has been reported on tc-99 m hmpao brain spect imaging . ictal spect using tc-99 m hmpao is also useful in demonstrating the site of epileptogenesis and its spread . sometimes it has been found that brain spect shows increased radiotracer uptake in the pathological hemisphere in the early part of life , which gets reversed in the later stage with decreased radiotracer uptake in the pathological hemisphere . these serial changes of radiotracer uptake on spect probably reflect either changes in epileptic activity or maturational changes in cerebral perfusion in hemimegalencephaly . brain spect helps in excluding seizure foci or other perfusion abnormalities in the contralateral side before surgery . considering recent recommendations for hemispherectomy in these patients , tc-99 m ecd / hmpao spect scan should be used to complement electroencephalogram ( eeg ) as a method to define the extent of abnormality , which may be more relevant to long - term prognosis than eeg alone . also , while interpreting a brain spect , hemimegalencephaly must be considered as a differential in hemihypoperfusion , for which ct / mri correlation should be sought before reporting . the other differentials for hemispheric hypoperfusion are rasmussen 's encephalitis , sturge weber syndrome , aspergers syndrome , exanthema subitum and gliomatosis cerebri , which can even be mistaken for hemimegalencephaly .

hemimegalencephaly is a rare congenital neuronal migration disorder that can presents with the equally rare finding of hemihypoperfusion on brain perfusion single - photon emission computed tomography ( spect ) . it is an extremely rare cause of intractable epilepsy . technetium-99 m ethyl cysteinate dimer ( ecd ) brain perfusion spect is useful in excluding other foci of hypoperfusion in the contralateral since hemispherectomy has been suggested to be the treatment of choice . furthermore , hemimegalencephaly may present with hyper as well as hypoperfusion on ecd spect . we present the case of an 11-year - old male child with intractable seizures who showed hemihypoperfusion in the hemimegalecephalic hemisphere .

the majority of cases of obsessive compulsive disorder ( ocd ) begin in childhood and adolescence ; but in clinical practice , early onset ocd with slowness is rarely encountered . the obsessive slowness ( os ) was first described by rachman ( 1974 ) who documented ten cases of primary obsessive slowness . the literature on obsessive slowness is limited and primarily deals with the diagnostic criteria , neurobiology , along with some highlights on the management . we report a case of early onset ocd with obsessive slowness , which was satisfactorily treated with combined pharmacotherapy and behavioral therapy . m , a 15-year - old boy from middle socioeconomic status , hindu , nuclear family , student of eighth class , without any past medical and psychiatric illness , with anankastic traits in parents as rigidity and stubbornness , and with slow to warm premorbid temperament ; presented with 3 year history of insidious onset and progressively deteriorating course of symptoms characterized by repeated doubts ( regarding locking and unlocking the door knobs , turning switches on and off ) , repeated fears ( something bad would happen with him if somebody would touch him while performing almost all routine activities ) , magical thinking ( roof falling on him if he would not stand properly from toilet seat ) , mental compulsion ( counting ) with progressive slowness in self - care behavior ( brushing , bathing , changing clothes , walking , and eating ) , and avoidance of routine activities with academic decline . for 6 months prior to reporting to a tertiary level referral hospital in india , the patient started having anxious and low mood , loss of interest in all pleasurable activities , and stopped going to the school . he would remain confined to bed , would not speak and communicate mostly by gestures . he would spend 2 - 3 h in finishing his one meal , and similar time in bathing , changing clothes , etc . , the mental status examination revealed obsessive slowness , obsessive doubts and fears , magical thinking , yielding , and mental compulsions . yale - brown obsessive compulsive scale ( ybocs ) score was 40 at the time of initial assessment . he was diagnosed as a case of ocd with slowness following international classification of diseases and related health problems ( icd-10 ) . after detailed behavior analysis , it was found that he had slowness and repetition in all routine activities with score of 9 - 10 for distress on a scale of 1 - 10 . he would remain in bed throughout the day and would do all routine activities only on being coaxed by parents . prompting , pacing , and shaping of routine activities were carried out daily . also , fluoxetine was slowly titrated to 60 mg / day and was well tolerated by him . initially , it was difficult to get him involved in the treatment as he would take several minutes while coming to interview room , starting conversation , and following instructions . it was decided to make individual sessions longer because the slowness and resistance made it difficult to achieve the individual session goals . the duration of sessions was increased to 90 - 120 min instead of usual 45 - 50 min . also , psychoeducation for the family members was started . initially , the parents had difficulty in accepting the fact that their son 's problems were because of a psychiatric illness as they believed that m 's problems were due to maladaptive behavior and he did it on purpose to annoy them . however , later on with psychoeducation , his mother understood the nature of his illness . she agreed to be cotherapist and her increased involvement and dedication made the therapy more intense and helped the team to achieve more and more cooperation from the patient . the two major hurdles in managing the case were difficulty in establishing rapport with the patient and retaining him in the therapy . after beginning of the combined therapy , he started showing improvement in almost all the problem areas ( as tabulated below ) and after 3 months , ybocs score reduced to 25 with improvement in the time spent in getting out of bed , brushing , going to toilet , answering to questions , changing clothes , and eating . post discharge from the hospital , he came regularly for follow - ups and his mother continued to be a cotherapist and would practice therapy at home . in our case , ocd had started at the age of 12 years with obsessive doubts and fears , magical thinking , mental compulsions , and slowness in self - care behavior . slowness in his behaviors like brushing , getting up from bed , urinating and defecating , and responding to questions was not preceded by obsessive ideas , doubts or fears , or mental compulsions and was not accompanied by anxiety . the features like prominent slowness in self - care behavior , absence of an increase in anxiety , or discomfort either before or following the behavior corresponds to description of primary obsessive slowness by rachman ( 1974 ) . however , some researchers ( veale , 1993 ) have suggested that the slowness is secondary to the recognized phenomena of ocd or anakastic personality disorder . our patient responded to combined therapy of fluoxetine along with behavioral therapy in the form of prompting , pacing , shaping for symptoms related to slowness , and exposure - response prevention directed for the repetitive behavior he improved considerably . in most of the interventional studies available for obsessive slowness , the interventions sped up specific target behaviors without affecting other areas of ocd . in a case series of 11 down 's syndrome patients with obsessive slowness by charlot et al . , ( 2002 ) , good response to behavioral pacing and shaping was demonstrated . however , replicable details of the treatment were not provided by the authors . in our case , there was considerable improvement after 12 weeks of intervention ; most of the studies reported that effect occurs over 6 - 16 weeks . his case demonstrates a successful treatment of ocd with obsessive slowness and the challenges met during the course of treatment .

obsessive slowness is a rare entity and is conceptualized either as primary psychiatric illness or as part of obsessive compulsive disorder ( ocd ) . often its outcome is frustrating even with treatment . we report a case of early onset severe ocd with obsessive slowness which showed good response to combined pharmacotherapy and behavioral therapy .

a 52-year - old obese white man was referred to our hospital because of increasing fatigue and exertional dyspnea . he had been well until 5 months previously . the patient had a medical history of dyslipidemia and hypertension . his hypertension was poorly controlled despite a combination of antihypertensive agents ( beta - blocker and angiotensin receptor blocker ) . physical examination showed blood pressure 140/90 in both arms , a heart rate of 74 beats / minute and an apical gallop sound ( s4 ) . his echocardiogram showed bicuspid aortic valve with minimal regurgitation , segmental wall motion abnormalities and mild mitral insufficiency . a cardiac silhouette at the upper limits of normal and notching of the ribs were observed on the chest radiography . due to the significance of the cardiac dysfunction and his clinical presentation , the patient underwent a cardiac catheterization to evaluate his coronary artery disease . the left ventricular ejection fraction was significantly reduced ( ejection fraction : 30 - 35% ) . aortography showed a mildly dilated aortic root , minimal aortic valve insufficiency and a significant ring - like stenosis in the thoracic descending aorta ( figures 1 and 2 ) . since , the collaterals were well recognized before surgery , the procedure was achieved without major bleeding and any adverse event . furthermore , the patient was adult and any minor bleeding has not resulted in requirement of blood transfusion . the coarctated segment was resected totally and end to end anastomosis of thoracic aorta was performed in a standart fashion . the coarctated segment was short in our patient and it was not difficult to get the two ends together without tension on the anastomosis so that we do not considered an interposition graft . the cross clamp time was 23 minutes and because the collaterals were left intact , any malperfusion syndrome has not occurred . total hospital stay after procedure was only four days . after the 1-year follow - up visit , aortic coarctation is a congenital vascular lesion typically diagnosed in early life , accounting for 5 to 10% of all congenital cardiovascular malformations1 but may go undetected well until adulthood2 . it manifests as childhood hypertension , lower extremity fatigue or weakness , diminished lower extremity pulses and/or congestive heart failure . the latter include blood pressure difference between the upper and lower extremities , pulse delay and systolic murmur over the thoracic spine . other manifestations can include bicuspid aortic valve systolic ejection sound and/or murmur and neurological complaints . prognosis and survival depend on the disease severity and patient 's age at the time of correction . death in these patients is usually due to heart failure , coronary artery disease , aortic rupture / dissection , concomitant aortic valve disease , infective endarteritis / endocarditis , or cerebral hemorrhage4,5 . there are few reports of patients first diagnosed with uncorrected aortic coarctation at very late age2,6,7 , treatment consists of aggressive hypertension therapy , endocarditis prophylaxis and corrective treatment for coarctation lesions with a high gradient8 . in this case report , we present aortic coarctation with bicuspid aortic valve in a 52-year - old male . our patient was relatively asymptomatic until he presented with chest discomfort , fatigue and dyspnea in his fifth decade of life .

aortic coarctation is a congenital malformation of the aorta usually diagnosed and corrected early in life . long - term survival is exceptional in patients with untreated aortic coarctation . in this case report , we present a late diagnosis of aortic coarctation in a 52-year - old male . our patient was relatively asymptomatic until he presented with exertional dyspnea and fatigue in his fifth decade of life . the patient was managed by surgery of aorta . after the 1-year follow - up visit , the patient was in good clinical condition .

the majority of ingested foreign bodies can be retrieved or pass through the gastrointestinal tract uneventfully . less than 1% of patients with foreign body ingestion develop gastrointestinal perforation . in our case , the small bowel perforation was self - sealing , but the ingested fish bone also caused necrotising fasciitis of the abdominal wall . a 63-year - old lady with good past health presented to our hospital complaining of right - sided abdominal pain for 3 days . there were no associated symptoms . on physical examination , she was afebrile with stable vital signs . there was right abdominal patchy erythema from the level of the lower ribs down to , and including , the vulva . scan showed a gas - containing collection at the right abdominal wall with a linear hyperdensity visible in the abdomen ( fig . laparoscopy showed that there was a fish bone that had perforated through the small bowel and lodged in the abdominal wall causing an abscess to form ( fig . 2 ) . the small bowel perforation had spontaneously sealed off , and the infection had travelled down along the fascia down to the vulva . figure 1:transverse plane of ct scan showing right - sided abdominal wall collection with fish bone just visible as a hyperdense dot just anterior to the liver . transverse plane of ct scan showing right - sided abdominal wall collection with fish bone just visible as a hyperdense dot just anterior to the liver . the resulting abdominal wall defect was in excess of 50 20 cm ( fig . figure 3:abdominal wall defect 1 day after the third debridement and the wound healing after 20 weeks . abdominal wall defect 1 day after the third debridement and the wound healing after 20 weeks . between the debridements , she had persistent sepsis and developed cardiovascular complications ; takotsubo 's cardiomyopathy , supraventricular tachycardia and pulseless ventricular tachycardia ( vt ) requiring defibrillation , with return of spontaneous circulation . although it can occur anywhere , it is most common in the lower limbs , followed by the abdominal wall and perineum . early diagnosis and early treatment play a crucial role in saving lives of victims with necrotising fasciitis . the aetiology of abdominal wall necrotising fasciitis can be external or internal . external causes include trauma , which can be minor or major , ranging from mosquito bites to blunt trauma , and also surgical wounds . internal causes are mostly secondary to intra - abdominal sepsis such as perforated appendicitis , gangrenous cholecystitis and bowel perforation . necrotising fasciitis is infection of the superficial fascia . nutrient vessels in the hypodermis are thrombosed , leading to tissue ischaemia , worsened by oedema . in turn it has been proposed to divide necrotising fasciitis into four types according to their microbiology . type ii is monomicrobial infection with beta - haemolytic streptococcus a [ streptococcus pyogenes ] . type iii is monomicrobial infection with clostridium species or gram - negative bacteria such as vibrios species or aeromonas . the diagnosis of necrotising fasciitis can be difficult , and a high index of suspicion is required in the early phase before sepsis becomes overwhelming . early clinical signs and symptoms may only suggest local pain , erythema and swelling . as for investigations , blood tests are often non - specific . the finger test , involving a 2-cm incision down to the deep fascia followed by gentle probing of a finger , may yield dishwater pus , lack of bleeding and lack of tissue resistance to blunt finger dissection . ct and magnetic resonance imaging ( mri ) can help to establish the diagnosis by showing tissue infection , fascial swelling and inflammation . it can also demonstrate gas in the soft tissue , which is present in almost half of all patients . emergency surgical debridement of the affected tissues is the primary management modality for necrotising fasciitis . parallel or ventricle incision will result in bridges of skin , and skin islands will not usually survive . extension of the infection to involve the bowel or the peritoneum is not uncommon and may require laparotomy . postoperatively , abdominal wall wounds require serial dressing changes until the wound is free of infection . repair may require advanced flaps using an abdominoplasty technique , biological mesh or skin grafts . the second is the early diagnosis and early treatment ; the diagnosis was made within hours of admission , and the first debridement was performed soon afterwards , without delay . the third is the good outcome that the patient survived without morbidity and also without requiring abdominoplasty .

abdominal wall necrotising fasciitis secondary to fish bone ingestion is extremely rare . we present a case of ingested fish bone complicated with self - sealing small bowel perforation and abdominal wall necrotising fasciitis . following principles of necrotising fasciitis , a high index of suspicion led to early diagnosis and early treatment . the patient enjoyed a good recovery .

a 2 month old girl was referred for assessment of respiratory distress , murmur and failure to thrive . echocardiography confirmed dextroposition of the heart with dilated right atrium , right ventricle and pulmonary artery . there was anomalous pulmonary venous drainage with the right - sided pulmonary veins appearing to drain to coronary sinus . partial anomalous drainage of the right - sided veins pulmonary veins to the upper inferior vena cava was identified . the right - sided veins were successfully baffled to left atrium through the atrial septal defect . despite surgery she remained unwell with frequent respiratory infections and did not thrive . a ct angiogram was performed which demonstrated scimitar syndrome with an area of pulmonary sequestration in the lower lobe of the right lung ( figure 1 ) . coronal multiplanar reconstruction demonstrating the scimitar vein draining to the inferior vena cava just below the right hemidiaphragm . the right pulmonary artery was underdeveloped and there was a large collateral artery from the descending aorta supplying the right lower lobe . interesting , a complex racemose network of venous channels was noted connecting the inferior vena cava to the hepatic veins and the right atrium . the primitive hepatic venous plexus is seen draining to the infra - diaphragmatic inferior vena cava ; at the point of entry of these veins , the inferior vena cava is stenotic ( white arrow ) . the sequestrated segment lies posteriorly , immediately adjacent to the inferior vena cava ( grey accented arrow ) . the systemic arterial supply vessel arises from the descending aorta just above the origin of the coeliac trunk ( black accented arrow ) . the large collateral artery was successfully occluded during cardiac catheterisation with 14 micro coils , which greatly improved her symptoms . the patient made a good recovery from this procedure and continues to do well at follow - up . to date she has required no further intervention for the sequestered lobe of right lung ( figures 3 and 4 ) . scimitar syndrome , a rare congenital abnormality consisting of anomalous drainage of one or all of the right - sided pulmonary veins to the inferior vena cava producing a classic scimitar - like shadow on the chest x - ray , was first describedin 1960 . scimitar syndrome has a number of associations including absence or abnormalities of the right lung , dextroposition and congenital heart defects such as atrial septal defect . it has been described in association with anomalies of the systemic venous connections including , very rarely , underdevelopment of the inferior vena cava and persistence of the primitive hepatic venous plexus as seen here . the use of vascular plugs and coils has emerged as a successful , alternative method to occlude vascular connections . such large aorto - pulmonary collaterals carry high flows and often require multiple devices to achieve complete occlusion ( as described here ) . the primitive hepatic venous plexus may represent the embryological precursors to the hepatic veins and the hepatic segment of the inferior vena cava . it is usually benign , although its presence can have implications for patients undergoing palliation for complex congenital heart disease who may remain desaturated post operatively . there are no reports in the literature to date describing scimitar syndrome + /-pulmonary sequestration in conjunction with persistent hepatic venous plexus . the recognition of such associations , although rare , can prevent unnecessary investigations of the abdominal and hepatic vasculature and aid decision - making when planning surgical or other interventions for patients with complex congenital heart disease .

we report a case of scimitar syndrome with pulmonary sequestration , persistent primitive hepatic venous plexus and stenosis of the inferior vena cava in a child presenting with failure to thrive . such associations are rare but may have implications when planning interventions for patients with complex congenital heart disease

a 59-year - old man presented with progressive dysphagia and weight loss over a period of 6 months . the patient 's medical history did not reveal any granulomatous diseases such as sarcoidosis or tuberculosis . on upper gastrointestinal endoscopy , a tumor was detected in the distal esophagus and at the gastroesophageal junction ( siewert type i ) ; biopsies were taken . on endoscopic ultrasonography ( eus ) a tumor with a length of 8 cm was seen in the distal esophagus including the gastroesophageal junction . ct scanning confirmed the presence of the tumor in the distal esophagus and at the gastroesophageal junction ( fig . furthermore , it was noted on both eus and ct scanning that remarkably enlarged lymph nodes ( 20 and 17 mm , respectively ) were located near the celiac trunk , which appeared to be calcified ( fig . eus with fine - needle aspiration of these enlarged nodes was done and cytological examination showed tumor cells ( fig . hence , the tumor was classified as ct3n1m1a and was considered ineligible for primary surgical therapy because of the large peritruncal nodes . the patient was offered induction chemotherapy to downstage the tumor in order to facilitate radical resection . the patient followed a 6-course weekly treatment with carboplatin / paclitaxel , followed by another 3-weekly treatment with carboplatin / paclitaxel for 3 courses . response to induction chemotherapy was evaluated at 2 and 5 months after the diagnosis by means of ct scanning . a clear reduction in wall thickness of the primary tumor in the distal esophagus and at the gastroesophageal junction was seen after the patient had completed 9 cycles of chemotherapy ( fig . however , the calcified part of the lymph nodes near the celiac trunk had not decreased in size ( fig . nevertheless , given the patient 's age , good general condition and substantial response of the primary tumor , he was offered surgical resection of the tumor . six weeks after having finished chemotherapy the patient underwent transhiatal esophagectomy with peritruncal lymph node dissection , followed by gastric tube reconstruction with cervical anastomosis . the calcified lymph nodes could easily be identified intraoperatively and were resected en bloc together with the resection specimen . postoperative recovery was uneventful , and the patient was discharged home 15 days after the operation . histological examination of the resection specimen revealed a poorly differentiated adenocarcinoma with tumor - free resection margins ( pr0 ) . lymph node metastases were present in 3 locoregional nodes , out of 17 lymph nodes that were resected in total . the enlarged calcified lymph nodes that were located at the celiac trunk did not harbor vital tumor lesions anymore . calcified lymph nodes mostly involve thoracic and , less frequently , abdominal and pelvic nodes . nodal calcification is common in benign granulomatous diseases such as sarcoidosis and tuberculosis , in which half of the patients have been reported to have calcification of hilar and/or mediastinal nodes on ct scanning . in hematological malignancies it is well known that lymph nodes may calcify in response to chemo- and/or radiotherapy . likewise , nodal calcification has been reported in patients with metastatic prostate , ovary and colon carcinoma that have been treated with hormonal , chemo- and/or radiotherapy . moreover , calcification of lymph nodes can be identified by ct scanning as radiation - induced injury to normal tissue . these so - called de novo calcifications have been described in several case reports of patients with hodgkin 's lymphoma , papillary thyroid carcinoma , and adenocarcinomas of prostate , breast , colon , ovary and lung . metastatic nodal calcification can occur as a consequence of dystrophic calcium deposition in areas of necrosis . precipitation can occur as a reaction to tissue damage , when unresorbed necrotic material persists for prolonged periods . in our patient imaging suggested that the calcified part of the lymph nodes did not decrease in size and , consequently , did not respond to chemotherapy . however , on pathologic examination no vital tumor cells could be detected in these lymph nodes anymore . to our knowledge , no published data are available on response evaluation in tumor - positive calcified lymph nodes in esophageal cancer . we hypothesize that calcified lymph nodes can not decrease in size even in case of adequate destruction of tumor cells by chemotherapy . their incapacity to shrink should be kept in mind when evaluating response to neoadjuvant or induction chemotherapy . response measurement should preferably be directed at the change in size of the primary tumor in case calcified lymph nodes are present .

untreated malignant lymph nodes that are calcified are rare . publications on such calcifications are restricted to case reports . we present a case of calcified lymph nodes in a patient with adenocarcinoma of the gastroesophageal junction that seemed to be nonresponsive to induction chemotherapy , as they did not decrease in size . however , on pathological examination of the resected lymph nodes no vital tumor cells could be detected anymore . therefore , we hypothesize that a calcified lymph node is unable to shrink , even after adequate remission on induction chemotherapy . this should be taken into account when clinical decision - making depends on the change in size of an enlarged , calcified lymph node as a measure of treatment effect .

we present a case of 30-year - old female who presented with loss of consciousness , vomiting , fever and rash [ fig . 1 ] for 1 day . fundus examination revealed intraretinal hemorrhages , white retinal lesions . blood investigations such as coagulation profile , random blood sugar ( rbs ) , serum electrolytes , complete blood cell , and hiv were normal . . 2 and 3 ] . the patient also had choroidal neovascularization in the right eye which was confirmed with fluorescein angiography ( fa ) and optical coherence tomography ( oct ) . fa showed early phase shows hyperfluorescent area at macula which increases in intensity in transit phase ; it shows leakage during late phase . it also shows multiple areas of hyperfluorescence in both eyes which increase in intensity in late phase . oct showed retinal pigment epithelium disruption , overlying cystoid macular edema , minimal subretinal fluid suggestive of active cnv [ fig . 7 ] . optical coherence tomography ( od ) during initial presentation patient was given intravenous mannitol , intravenous antibiotics . once the patient was diagnosed with cnv , she was given 1.25 mg in 0.05 ml bevacizumab intravitreally every month for 3 months along with doxycycline 100 mg od for 15 days . the patient was followed up every month for first 6 months and then every 3 months . felix test was repeated after 2 months and showed decreased titers of ox2 indicating response to treatment . there was an improvement in the vision after the first dose of bevacizumab which was maintained after next two doses of bevacizumab . after 1 year vision was maintained and there was no activity in the cnv both on oct as well as on fa . once the patient was diagnosed with cnv , she was given 1.25 mg in 0.05 ml bevacizumab intravitreally every month for 3 months along with doxycycline 100 mg od for 15 days . the patient was followed up every month for first 6 months and then every 3 months . felix test was repeated after 2 months and showed decreased titers of ox2 indicating response to treatment . there was an improvement in the vision after the first dose of bevacizumab which was maintained after next two doses of bevacizumab . after 1 year vision was maintained and there was no activity in the cnv both on oct as well as on fa . multisystem involvement may be seen such as respiratory , gastrointestinal , renal , cns , and eye . ocular manifestations include mild vitreous inflammation , white retinal lesions , focal vascular sheathing , multiple arterial plaques , intraretinal hemorrhages , white - centered retinal hemorrhages , subretinal hemorrhages , serous retinal detachment , macular star , cystoid macular edema , optic disc edema , branch retinal artery occlusion , optic disc staining , retinal vascular leakage , delayed filling in a branch retinal vein , multiple hypofluorescent choroidal dots , and retinal neovascularization . it was confirmed serologically using elisa and weil felix test . as noted in earlier studies intravitreal bevacizumab vision was maintained in the patient even after 1 year , and there was no activity of cnv during the same period . in conclusion , cnv due to tick typhus occurred within first 4 weeks of onset of systemic and retinal manifestations . since the cnv was detected early , it responded well to the bevacizumab . the activity of the cnv did not increase during 1 year of follow - up . after observing this case , we conclude that early diagnosis and three , monthly doses of intravitreal bevacizumab helped to improve and maintain the vision of the patient in cnv due to indian tick typhus . 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 .

tick typhus causes hemorrhagic lesions over the skin . retina also shows hemorrhages and exudates . many cases have been reported in western literature about this condition . to our best of knowledge , this is the first case report of tick typhus in india which was also associated with inflammatory choroidal neovascularization .

non - or partial weight bearing is frequently the standard treatment after peri - articular lower extremity fractures . displaced talar neck fractures are severe injuries compromising vascularity of the corpus and consequently are at risk for non - union and avascular necrosis , the main reason to restrict weight bearing for up to three months according to most literature . we report a case of a 31-year old male with a high impact car accident . his pelvic ring and hawkins ii talar fracture were treated by open reduction and internal fixation . his fractures healed uneventfully and he was able to run freely , without any discomfort within 8 weeks . radiological evaluation of the talus showed complete bone healing without signs of avascular necrosis . at one year follow - up , we might consider changing the restricted or non - weight bearing protocol in surgically treated talar neck fractures at our centre and allow early weight bearing , based on body awareness and the creation of a safe environment during the rehabilitation phase . there is currently no consensus among surgeons worldwide with regard to early versus late weight bearing in fractures of the lower extremities . as an example displaced talar neck fractures requiring open reduction , internal fixation ( orif ) are usually rehabilitated with 10 - 15 kg ( partial ) weight bearing for at least 2 - 3 months after surgery . although talus fractures comprise approximately 0.1 to 0.85 % of all fractures , disturbed fracture healing frequently results in significant loss of motion and function . the incidence of avascular necrosis in surgically treated talar neck fractures is according to a recent systematic review up to 33% . early weight bearing after surgery in case of cartilage damage of the talus has been found to be beneficial on pain relief and ankle - hind - foot mobility [ 6 , 7 ] . we hypothesize that it might also lower the risk of avascular necrosis through increased revascularization . in this case report , we present a patient with a hawkins ii talus fracture treated by open reduction and early mobilization , leading to remarkable quick clinical improvement . a 31-year - old patient who had a high impact trauma ( car against a tree)was diagnosed with a pelvic ring fracture , a hawkins ii talar neck fracture of the right foot ( fig . 1 ) and a mtp [ 2 , 3 , 4 ] fracture of the left foot . the talus fracture was initially treated by casting ( 8 days ) , followed by an open reduction ( bilateral approach ) and internal fixation ( orif ) by plate and screw osteosynthesis and cannulated screws ( fig . after 10 days of hospitalization , the patient was signed up for intensive rehabilitation at a specialized unit . rehabilitation comprised of early weight bearing , based on body awareness technique , mobilizing according to pain , and hydrotherapy . the recovery was surprisingly fast and within 7 weeks postoperatively , the patient could mobilize completely unaided and was even able to run ( see video ) . at one year follow - up follow - up x - ray did not demonstrate any mechanical failure and an mri scan one year after surgery showed complete bone healing without signs of avascular necrosis . several biomechanical and human studies demonstrate that early weight bearing in talus fractures is beneficial on osteogenic healing , new vessel formation and on pain relief and ankle - hind foot mobility [ 6 , 7 , 8 ] . however , talus fracture protocols in the netherlands as well as most countries worldwide prescribe a non - weight bearing short - leg cast for six weeks with maximum weight bearing allowance of 10 - 15 kg , followed by gradual weight - bearing in a cast boot for two to four weeks , and finally a gradual return to full weight bearing after 3 months . the question one is faced with is if nonweight bearing enhances bone healing and/or revascularization of the subchondral bone and thereby improved cartilage recovery or might be even detrimental . this case is an interesting and unique illustration that early mobilization guided by body awareness may be beneficial not only on pain relief and increased mobility but also accelerates recovery and lower the risk of avascular necrosis after orif surgery patients with a talus fracture . important is to mention that this is an observation and certainly no policy recommendation until further evidence is provided to support this case . in an aftercare treatment scheme , both over and under - loading may lead to prolonged and complicated recovery . patients with surgically treated talar neck fractures might benefit from an early weight bearing protocol , based on body awareness and the creation of a safe environment during the rehabilitation phase . high - quality studies comparing weight - bearing protocols after talar neck fractures are not readily available . the exact timing and subgroups of patients that might benefit from early weight bearing therefore needs further clarification .

introduction : non - or partial weight bearing is frequently the standard treatment after peri - articular lower extremity fractures . displaced talar neck fractures are severe injuries compromising vascularity of the corpus and consequently are at risk for non - union and avascular necrosis , the main reason to restrict weight bearing for up to three months according to most literature.case presentation : we report a case of a 31-year old male with a high impact car accident . his pelvic ring and hawkins ii talar fracture were treated by open reduction and internal fixation . rehabilitation was based on permissive weight bearing following wound healing . his fractures healed uneventfully and he was able to run freely , without any discomfort within 8 weeks . radiological evaluation of the talus showed complete bone healing without signs of avascular necrosis . at one year follow - up , the patient is free of the symptoms.conclusion:we might consider changing the restricted or non - weight bearing protocol in surgically treated talar neck fractures at our centre and allow early weight bearing , based on body awareness and the creation of a safe environment during the rehabilitation phase .

a 69-year - old japanese man presented with pigmented skin lesions on the inguinal regions ( figure 1 ) . the lesions were first noticed three years earlier , and they subsequently spread to the chest ( figure 2 ) and back . the patient had no specific symptoms but reported that his father had had similar lesions . physical examination showed dark brown macules of up to 20 mm in diameter with partial confluence ( figure 3 ) . the macules on the chest tended to be smaller ( maximum diameter : 5 mm ) and lighter in color than those on the back . dermoscopy demonstrated central brown pigmentation with many blue - gray dots surrounded by a single hypopigmented band , namely the peripheral border of the white track showed light brown pigmentation , making the white track recognizable . there was no whitish linear structure corresponding to wickham s striae often seen in lichen planus . a biopsy was taken from the peripheral ridge of a brown macule on the right inguinal area . histopathological examination showed a parakeratotic column , the so - called cornoid lamella ( figure 5 ) . irregular arrangement of keratinocytes with pyknotic nuclei , in addition to perinuclear edema were noted in the epidermis underneath the parakeratotic column . five different forms can be distinguished , including the plaque type of mibelli , disseminated superficial actinic porokeratosis ( some lesions of this type are distributed mainly in areas not exposed to the sun or appear in patients on immunosuppressants ) , linear porokeratosis , porokeratosis plantaris palmaris et disseminata and punctuate porokeratosis . genital porokeratosis is extremely rare , but classical lesions have been reported on the penis and scrotum . chen et al reported 10 cases of genital porokeratosis in asian patients and the lesions in three of their patients were in genital and adjacent areas , with one patient presenting with inguinal lesions . the lesions in our patient appeared first in the inguinal area and subsequently spread to the trunk . since the lesions on the trunk were relatively subtle compared to those in the inguinal area , the patient only complained of the latter lesions on the initial examination , thus the differential diagnosis included lichen planus linearis . however , dermoscopic examination demonstrated typical features characteristic of porokeratosis , namely a white track or whitish - yellowish annular structure . as reported by chen et al , genital porokeratosis is extremely rare and some of their cases were clinically indistinguishable from extramammary paget s disease , bowen s disease or lichen planus . because genital or inguinal porokeratosis is very rare , though more common in asians and often mimics extramammary paget s disease , bowen s disease or lichen planus , dermoscopic examination is useful when the white track structure is detected at the periphery of the macule .

inguinal and genital porokeratosis are rare but seem to be more common in asians . we report a case of a 69-year - old japanese man with multiple lesions of porokeratosis in both inguinal regions . the lesions first appeared in the inguinal region and subsequently spread to the trunk . the patient reported that his father had had similar lesions . dermoscopy demonstrated central brown pigmentation and blue - gray dots surrounded by a single white track at the periphery . the exterior border of the white track also showed light brown pigmentation . genital or inguinal porokeratosis is uncommon and may be misdiagnosed as bowen s disease , lichen planus or extramammary paget s disease . however , awareness of this entity and the use of dermoscopy are helpful to establish a correct diagnosis .

montelukast sodium obtained as a gift sample from cedilla health care , moriya , chitosan ( medium . molecular weight ) , mannitol a. r. , propylene glycol a. r. , leucine , glacial acetic acid a. r. , distill water , ethanol , gluterladehyde . elemental analysis was used to determine the degree of deacetylation in raw chitosan sample with chns / o elemental analyzer ( model name perkinelmer pe 2400 series 2 ) . the chitosan - drug solution was spray dried by lab - ultima spray drier ( lu-222 advanced , mumbai , india ) with standard 0.7 mm nozzle . the spray drying conditions such as inlet temperature , outlet temperature , pump prate , pressure and aspirator setting were set as 130c , 70 - 80c , 3 ml / min , 2 kg / cm and 50 m / h , respectively . the prepared microspheres were characterized for loading efficiency , entrapment efficiency , morphological characterization by scanning electron microscope , swelling index determination and an in vitro mucoadhesive property of dry powder was performed by modified liquid falling film method . the chitosan - drug solution was spray dried by lab - ultima spray drier ( lu-222 advanced , mumbai , india ) with standard 0.7 mm nozzle . the spray drying conditions such as inlet temperature , outlet temperature , pump prate , pressure and aspirator setting were set as 130c , 70 - 80c , 3 ml / min , 2 kg / cm and 50 m / h , respectively . the prepared microspheres were characterized for loading efficiency , entrapment efficiency , morphological characterization by scanning electron microscope , swelling index determination and an in vitro mucoadhesive property of dry powder was performed by modified liquid falling film method . degree of deacetylation of chitosan was found to be 90% , the percent yield , mean particle size , drug loading efficiency and encapsulation efficiency was found to be 32 - 49% , 7 - 12 m , 48 - 53% and % 78 - 86% respectively . based on swelling index determination results , as the concentration of polymer increased , the swelling of microspheres were found to be increased because the polymer network increases with increasing in the concentration of polymer . in vitro drug release study revealed that the burst release of drug was observed in this study indicating approximately 50 - 60% of drug release within first 30 minutes . the sustained drug release was seen after two hours which followed zero order release pattern , more than 90% of drug released in 8 hours measure in u. v. at 285 nm . in vitro mucoadhesive study indicated that , more than 50% of microspheres were adhering to rat intestine that attributed with the increasing the mucoadhesion by increasing the drug : polymer ratio . the recent studies on spray dried chitosan microspheres demonstrated possibility of generating highly respirable powders that exhibits good aerosolization properties . it is possible to produce good spherical particles with size less than 10 m with spray drying technique . the applied processing conditions resulted in drug loaded chitosan microspheres having good surface properties and spherical shape suitable for inhalation .

the objective of present work was to prepare microspheres of montelukast sodium using a natural polymer- chitosan by spray drying method by using glutaraldehyde as a cross linking agent . the microspheres were characterized for size , shape , dissolution , swelling and mucoadhesion . it was observed that , all microspheres were spherical in shape with narrow size distribution . microspheres had mean particle size of 7 - 12 m , with % encapsulation efficiency of 78 - 86% . the % yield was 32 - 49% and drug load was 48 - 53% . with the increase in proportion of chitosan in formulation mucoadhesive strength was increase and also increased in particle size of microspheres . as the drug : polymer ratio increase drug loading was increase and % encapsulation efficiency was also increase .

all simulations were carried out using charmm , with the additive c36 or the polarizable drude-2013 ffs . each dna double helix was solvated with tip3p water and neutralizing na ions in a periodic octahedral unit cell . following energy minimization , each system was heated to 283.15 k , corresponding to the experimental conditions , over 20 ps with weak harmonic restraints on all dna heavy atoms . restraints were removed , and temperature and pressure were held constant at 283.15 k using a hoover thermostat and 1.0 atm with the langevin piston method , respectively , for an additional 80 ps . all covalent bonds involving hydrogen atoms were constrained with shake , allowing an integration time step of 2 fs for c36 simulations and 1 fs for drude-2013 simulations . electrostatic interactions were calculated with the particle mesh ewald method , with the real - space contribution truncated at 12 . following equilibration , 72 configurations for umbrella sampling were generated using the miscellaneous mean field potential ( mmfp ) module of charmm . the reaction coordinate was a pseudodihedral angle ( ) described previously , and an example is illustrated in figure s1 in the supporting information . in brief , the four groups of atoms constituting the pseudodihedral angle are defined as the heavy atoms of the flipped base , its sugar ring , the sugar ring of the adjacent 3-nucleotide , and the base pair to the 3-side of the flipped base . configurations were generated from 0 to 180 and from 0 to 180 in increments of 5 along the reaction coordinate , constituting flipping through both the minor and major grooves ( figure s1 in the supporting information ) , using a force constant of 5000 kcal mol rad . at this point , for the polarizable simulations , lone pairs and drude particles were added to the systems , a process that also converted tip3p water into the polarizable swm4-ndp model . for drude systems , an additional 50 ps of npt equilibration was performed , and the selections defining the pseudodihedral angle also included the drude particles associated with the non - hydrogen atoms . the simulation time in each window was 1 ns for c36 simulations and 1.5 ns for drude-2013 simulations , with all analysis performed over the final 500 ps . pmf curves were generated using wham , with a window dimension of 1 along the periodic reaction coordinate . coordinates were saved every 1 ps for analysis ; values of were output at each simulation step . convergence was assessed by calculating the pmf profiles and associated kop values over consecutive blocks of time during the simulations . simulations were stopped when 500 ps of continuous time existed , such that pmf profiles had stabilized . pmf profiles over different blocks of time are shown in figures s3 and s4 in the supporting information .

base flipping in dna is an important process involved in genomic repair and epigenetic control of gene expression . the driving forces for these processes are not fully understood , especially in the context of the underlying dynamics of the dna and solvent effects . we studied double - stranded dna oligomers that have been previously characterized by imino proton exchange nmr using both additive and polarizable force fields . our results highlight the importance of induced polarization on the base flipping process , yielding near - quantitative agreement with experimental measurements of the equilibrium between the base - paired and flipped states . further , these simulations allow us to quantify for the first time the energetic implications of polarization on the flipping pathway . free energy barriers to base flipping are reduced by changes in dipole moments of both the flipped bases that favor solvation of the bases in the open state and water molecules adjacent to the flipping base .

cheilitis granulomatosa manifests as edematous swelling of the lips and is considered an incomplete expression of melkersson - rosenthal syndrome , a triad of recurrent orofacial edema , recurrent facial nerve palsy and fissuring of the tongue . various treatments for cheilitis granulomatosa have been reported , but there is no defined treatment for cheilitis granulomatosa . here a 38-year - old male presented with a 3-month history of persistent lower lip swelling ( fig . previously , he had been treated with antihistamines , 2 weeks minocycline orally , topical corticosteroid and dapsone without success . patch testing showed positive reactions to 0.05% mercury chloride and amalgam ( both + + at d4 according to the icdrg recommendations ) . histopathological findings of a biopsy from the lower lip revealed epithelioid cell granuloma ( fig . the patient underwent dental treatment to replace his dental metals as he had positively reacted to mercury and amalgam ; however , we did not find any mercury in the removed metals . cheilitis granulomatosa is a rare disease that manifests as a diffuse and painless swelling of the lips . melkersson - rosenthal syndrome consists of the triad of recurrent orofacial swelling , relapsing facial paralysis and fissuring of the tongue . cheilitis granulomatosa is considered an incomplete expression of melkersson - rosenthal syndrome [ 1 , 2 ] . the etiology of cheilitis granulomatosa is unknown , but some cases have been associated with crohn 's disease and sarcoidosis [ 1 , 2 , 3 ] . various treatments for cheilitis granulomatosa have been reported , including antibiotics [ 4 , 5 ] , tranilast , oral and intralesional steroids [ 7 , 8 ] , and surgical resection . rapid improvement and/or complete resolution after dental treatment have been reported [ 1 , 10 , 11 , 12 ] . reported that elimination of the dental infectious foci resulted in regression or disappearance of swelling in 11 out of 16 patients . in our case , there was significant improvement of the lip swelling after treatment of the apical periodontitis and replacement of dental crowns . as the replaced dental crowns did not contain mercury , we believe that our case was associated with periodontitis . we suggest that examination and treatment of focal dental infections is necessary in the treatment of cheilitis granulomatosa . cases that do not improve after elimination of dental focal infection require further follow - up .

a 38-year - old male suffered from swelling of the lower lip for 3 months . neither facial nerve palsy nor fissuring of the tongue was present . histological examination of a biopsy taken from the lower lip revealed non - caseous epithelioid cell granulomas , suggestive of cheilitis granulomatosa . patch testing revealed positive reactions to mercury chloride and amalgam . his symptoms markedly improved 3 months after treatment of the apical periodontitis and replacement of dental crowns . as his dental crowns did not contain mercury , we believe that the cheilitis granulomatosa may have been related to the focal dental infection .

originally called bone - metastasizing renal tumor of childhood by marsden et al . , clear cell sarcoma of the kidney ( ccsk ) is a highly malignant neoplasm occurring in the same age range as wilms tumor . however , ccsk is resistant to conventional therapy , but often responsive to doxorubicin containing regimens . a 2-year - old boy presented with 2-week history of a mass in the right upper abdomen . clinical examination showed firm , non - tender swelling occupying the right hypochondrium and lumbar region . ultrasonography showed a 12.3 8.7 cm hetrogenous mass in the right kidney . with the provisional diagnosis of wilms tumor gross examination revealed a right nephrectomy specimen , measuring 12.5 10.5 7.5 cm , encased in capsule surrounded by perinephric fat . portion of lower pole of kidney was grossly identifiable , and there was a small segment of ureter measuring 0.75 cm located at the posteriomedial aspect along with vascular channels , embedded in the fat . the surface was grayish solid with areas of necrosis and hemorrhage [ figure 1 ] . perinephric fat was grossly free from the tumor and the outer capsular surface appeared smooth . the gray - tan tumor is large , soft focally cystic and necrotic sections showed highly cellular tumor consisting of proliferating monotonous undifferentiated malignant cells of small to medium size . tumor showed delicate vascular stroma , foci of cystic degeneration , and extensive areas of necrosis [ figure 2 ] . immunochemical markers vimentin , non specific esterase ( nse ) , and cytokeratin were used . the prominent and branching vascular patterns and cell nuclei with fine occasionally vesicular chromatin and inconspicuous nucleoli ( h and e , hp ) in view of the above morphological and immunochistochemial findings , diagnosis of ccsk was confirmed . the bone scan was done prior to chemoradiation , which showed disseminated bony metastasis ( stage 4 ) . the patient was treated in accordance with nwtsg-3 protocol and was kept on doxorubicin in combination with vincristine and dactinomycin , initially for a period of 24 weeks . the patient also received postoperatively , a total of 10.8 gy of radiation therapy in divided doses for the tumor bed . he responded to treatment as evident by clinical improvement at subsequent follow - ups till 4 months . the patient , however , died 2 months later at his home , and the cause of death remains unknown . non - wilms renal tumors account for less than 10% of primary renal neoplasm of childhood . it has a peak incidence during 3 - 5 years , with a male : female ratio of 2:1 . these are unilateral and unicentric tumors arising from the medullary region of the kidney with mucoid texture , foci of necrosis , and cyst formations . most of these have a classic pattern , with ( 91% ) as either a predominant or a secondary morphology . the variant patterns recognized are myxoid ( 50% ) , sclerosing ( 35% ) , cellular ( 26% ) , epithelioid ( trabecular or acinar type ) ( 13% ) , palisading verocay body ( 11% ) , spindle cell ( 7% ) , storiform ( 4% ) , anaplastic ( 2.6% ) , blended smoothly with classic pattern or with each other . in the classic pattern , the tumor cells appear monomorphic with cords or nests of 6 - 10 cells separated by dispersed in small , regularly spaced arborizing fibrovascular septa . while usually plump , the cells in the core uncommonly may assume a spindle shape . nuclei are overall uniform in shape , with fine dusty chromatin without prominent nucleoli or coarse condensations . tumor cells are not reactive for cytokeratin , ema , mic2 , s100 , synaptophysin , gfap , lca , and nse . distinction of ccsk from blastemal wilms tumor and primitive neuroectodermal tumor ( pnet ) , both having prominent vascular patterns , is important . these tumors are more aggresively invasive than ccsk , entrapping whole islands of native renal parenchyma , as opposed to single tubules entrapped by ccsk . wilms tumor has an early metastasis and classical spread to lymph nodes , lungs , and liver , but metastasis to bone is rare . nearly 10% of wilms tumor displays recognized congenital anomalies such as overgrowth syndrome , isolated aniridia and trisomy 18 , hypospadias , cryptorchidism , and renal fusion . it is of considerable therapeutic importance that clear cell sarcoma be not only correctly diagnosed but early too .

clear cell sarcoma of the kidney is an uncommon renal neoplasm of childhood . it represents one of the most common unfavorable tumors included in national wilms tumor study group clinical protocols . we came across this rare tumor in a 2-year - old male child . the case report is followed by discussion , stating the differentiating features between wilms and clear cell sarcoma , histological details , treatment , and prognostication .

crown fracture has been documented to account up to 92% of all traumatic injuries to the permanent dentition . the extent of the tissues involved in the traumatic injuries determines the management needs . for fracture restricted to the crown with pulp involvement in anterior teeth , immediate reattachment of a dental fragment a 24-year - old male patient reported to the department of conservative - dentistry and endodontics , m.m . college of dental sciences and research , mullana , haryana , india with the chief complaint of broken upper front tooth following trauma a day ago . initial examination revealed a green stick type of fracture with pulp exposure on the labial surface of maxillary right central incisor . the fracture was not evident palatally [ figure 1 ] . left maxillary central incisor showed mesio - angular incisal chipping . pre - operative photograph radiographic examination revealed an oblique fracture labio - palatally [ figure 2 ] . after routine history taking and examination , a treatment plan was formulated to immediately reattach the dental fragment of the teeth . local anesthesia was administered ( lignocaine 2%).the fracture segment was completely removed and preserved in physiological saline solution in order to prevent dehydration of the tooth fragment . a clean horizontal fracture , mesial to distal angulated incisally from labial to palatal was evident [ figure 3 ] . [ figure 3]the root canal was then prepared with parapost drill , a corresponding pre - fabricated fiber post was selected for coronal fixation.preparatory alignment was done , by placing selected fiber post in to the canal and access prepared in the coronal tooth fragment to fit the post . its bonding surface and pulp cavity was loaded with flowable composite to attach post , maintaining alignment with finger pressure until the composite set . [ figure 5]after obtaining single unit , dual cure resin was placed in the canal , single unit post , and coronal segment was placed in to the canal . [ figure 6]a radiograph was then recorded to confirm apposition of the two tooth portions . the fracture segment was completely removed and preserved in physiological saline solution in order to prevent dehydration of the tooth fragment . a clean horizontal fracture , mesial to distal angulated incisally from labial to palatal was evident [ figure 3 ] . [ figure 3 ] the root canal was then prepared with parapost drill , a corresponding pre - fabricated fiber post was selected for coronal fixation . preparatory alignment was done , by placing selected fiber post in to the canal and access prepared in the coronal tooth fragment to fit the post . its bonding surface and pulp cavity was loaded with flowable composite to attach post , maintaining alignment with finger pressure until the composite set . [ figure 5 ] after obtaining single unit , dual cure resin was placed in the canal , single unit post , and coronal segment was placed in to the canal . [ figure 6 ] a radiograph was then recorded to confirm apposition of the two tooth portions . radiograph of sectional obturation with reattached segment single unit post and crown segment post - operative photograph patient was recalled after 2 days . at the end of 2 days , the uncomplicated traumatic injuries involving tooth fracture can be treated by reattachment of the tooth fragment using an adhesive system to provide what is considered to be the most conservative of restorations . newer dentine bonding system work with such efficiency that they easily allow for normal masticatory forces . survival rates for such restoration have been shown to be good , with failure often only resulting from subsequent trauma . fiber - reinforced posts are fabricated to bond with most resin cements and resin - based composite core materials . successful bonding minimizes the wedging effect of post within the root canal , requires less dentin removal to accommodate a shorter and thinner post , and leads to lower susceptibility to tooth fracture . factors influencing the extent and feasibility of such repairs include the site of fracture , size of fracture remnants , periodontal status , pulpal involvement , maturity of root formation , biological width invasion , occlusion , time , and resource of the patient . post - placement in addition to bonding , serves to retain the coronal portion via a friction bond , and assist in preventing dislodgement non - axial forces . the composite reinforcement technique together with this light - transmitting post had been widely used to functionally and esthetically restore compromised root filled teeth . it is more flexible and has modulus of elasticity approximately of the same ( stiffness ) as dentin . the advantage of using original tooth fragment over all other materials includes color , morphology , translucency , physicochemical characteristics , patient acceptance , structurally conservative , and economical . root extraction and prosthetic replacement , i.e. , fixed , implant , removable.retention of the apical tooth portion and conventional conservation , e.g , periodontal correction if required , cast restoration.orthodontic extrusion , followed by restoration.surgical extrusion involving extraction then re - implantation and restoration . root extraction and prosthetic replacement , retention of the apical tooth portion and conventional conservation , e.g , periodontal correction if required , cast restoration . these may include multi - visit appointments , cost , stabilization , and be less conservative in nature when compared with this case report . in fracture involving two thirds of the crown extending subgingivally , invading the biological width , the repair of the fracture includes- flap surgery with a slight ostectomy and endodontic treatment . further , in some cases reattachment of a fractured tooth segment using dual cure resin allows the restoration of original tooth contour , colour and aesthetics . in this era of conservative , esthetic dentistry , the reattachment of fractured tooth segments has established itself as a realistic treatment option in the restoration of fractured teeth . it permits rapid restoration of original tooth contours and overall esthetics with greatly reduced chair time for both the patient and operator .

minimal intervention with maximum dentistry the immediate restorative technique resolving the acute problem of traumatic tooth fracture with pulpal involvement an immediate fracture fragment reattachment using pre - fabricated fiber post with dual cure cement a challenging , conservative , aesthetics , rehabilitating , functionally , and economically viable single visit procedure .

in october 2008 , a 32-year - old man with a history of type ii diabetes , hypertension , and obesity was admitted to a small community hospital ( hospital a ) in arizona . he had severe right knee pain and fever . on 3 occasions the week before being admitted , he had been evaluated in hospital a s emergency department for severe right knee pain . the patient denied cough , chest pain , and swelling and pain of other joints . over the course of 23 months , he lost weight and had exhaustion , intermittent right knee pain , and nightly fevers . the patient did not smoke , drink alcohol , or use illicit drugs and was in a monogamous heterosexual relationship . he worked at an automobile body shop preparing and painting cars and had previously worked as a motorcycle and all - terrain vehicle mechanic . he also had several dogs in the household but reported no unusual exposure to other animals . culture of the synovial fluid did not yield any bacterial growth , and evidence of infection or crystals was not apparent . a blood specimen was sent for culture and yielded what was initially identified as escherichia coli by an automated in - house instrument . chest radiograph and computerized axial tomography scan with contrast were unremarkable and did not demonstrate evidence of pneumonia . after 8 days in hospital a and no resolution of fever or knee pain , he was transferred to hospital b , a large regional hospital , with an initial diagnosis of persistent e. coli sepsis and possible vegetative valve lesions . blood cultures grew b. pseudomallei identified by an automated in - house instrument . because initial results were unexpected , blood samples were submitted to a reference laboratory and the arizona state public health laboratory ( phoenix , az , usa ) where results confirmed the presence of b. pseudomallei . the bacteria continued to grow in blood cultures for 16 days after initial hospitalization at hospital a on october 7 . cultures of the knee fluid grew b. pseudomallei for 7 days ; sputum cultures were positive for 6 days . all cultures were negative 2 weeks before the patient was discharged from hospital b on december 5 . the patient s hospital course was complicated by respiratory failure that required intubation and ventilation , acute renal failure , pneumothorax and pneumoperitoneum , and anemia and hypotension . fever resolved 21 days after admission to hospital a. knee swelling persisted for 6 weeks . antimicrobial therapy administered to the patient while he was an inpatient in hospital b included meropenem , moxifloxacin , vancomycin , ceftazidime , gentamicin , and trimethoprim / sulfamethoxazole . the patient was discharged with oral doxycycline and trimethoprim / sulfamethoxazole to a rehabilitation facility 7 weeks after his initial hospital admission . clinical isolates were analyzed to confirm b. pseudomallei infection and to determine the genetic origin of the isolate strain . specimens from hospital a had been destroyed by the time the patient s melioidosis was diagnosed , which precluded the possibility of determining whether the presumptive e. coli infection was actual or misdiagnosed . after receipt at the arizona state public health laboratory , an isolate was submitted to the centers for disease control and prevention ( atlanta , ga , usa ) for confirmation , and bacterial dna was extracted and sent to the translational genomics research institute ( phoenix , az , usa ) for genetic characterization . molecular analyses determined that the isolate strain originated from southeast asia , most likely malaysia , or a nearby country . serology testing performed 6 weeks post infection demonstrated a b. pseudomallei indirect hemagglutination assay titer of 160 ; any titer is considered positive in a person living in an area where the disease is nonendemic ( 2 ) . the patient and his family were interviewed to determine travel history and possible sources of exposure . no lifetime travel outside of the united states and only limited intrastate and interstate travel were established . the epidemiologic investigation , therefore , focused on the patient s home and work sites . possibilities for exposure included occupational exposure to imported vehicle parts , exposure to a person or object from a disease - endemic area , recreational exposure to imported soil or plants , or inoculation with contaminated medication . extensive investigation showed no evidence of exposure at the patient s worksite and no known exposure to any person or objects from a disease - endemic area . we conducted multiple on - site residential investigations , primarily focusing on the patient s self - reported history of exotic plant repotting . plant soil and root samples were collected in and around the patient s home 6 weeks after diagnosis ( winter ) , and 6 months later ( summer ) and were taken for analysis to the select agent laboratory of northern arizona university ( flagstaff , az , usa ) . infection may have occurred from exposure to contaminated medical products . because the patient was initially hospitalized with sepsis identified as e. coli , sepsis might have been the source of his knee pain , and he was subsequently inoculated with b. pseudomallei during knee arthrocentesis or from a contaminated oral or intravenous medication . despite extensive investigation , when , how , or where the patient was exposed to b. pseudomallei remains unclear . although travel to a disease - endemic area including southeast asia was ruled out , molecular analysis of the etiologic agent showed that it was consistent with southeast asian origin . this case demonstrates the difficulty in diagnosing a disease caused by a rare organism not endemic to the area and the complications that can ensue from delayed diagnosis . unfortunately , we could not identify the source of exposure despite an aggressive epidemiologic , environmental , and laboratory investigation . heightened awareness and surveillance by public health officials for this select agent is critical to learning more about the possible presence of b. pseudomallei in the united states .

burkholderia pseudomallei is a bacterium endemic to southeast asia and northern australia , but it has not been found to occur endemically in the united states . we report an ostensibly autochthonous case of melioidosis in the united states . despite an extensive investigation , the source of exposure was not identified .

hpmc e-15 , polyvinyl alcohol and propylene glycol ( pg ) and polyethylene glycol 400 ( peg400 ) were purchased from sigma chemicals . oral thin strip with varied mixture composition of hpmc e15 and pva were prepared by casting drug dispersion in water and subsequent evaporation of solvent in an open glass mould and dried at 40c for 9 hrs . propylene glycol ( 10% w / w ) was added as a plasticizer and citric acid as saliva stimutating agent , aspartame as sweetner . solvent was evaporated slowly at room temperature and controlled evaporation was assured by covering glass mould with an inverted funnel . after complete removal of the solvent , film were removed and kept in desiccators till further use . oral thin strip with varied mixture composition of hpmc e15 and pva were prepared by casting drug dispersion in water and subsequent evaporation of solvent in an open glass mould and dried at 40c for 9 hrs . propylene glycol ( 10% w / w ) was added as a plasticizer and citric acid as saliva stimutating agent , aspartame as sweetner . solvent was evaporated slowly at room temperature and controlled evaporation was assured by covering glass mould with an inverted funnel . after complete removal of the solvent , hence various cellulose derivatives namely hpmc , hpc , cmc , gelatin , pva , were used as primary film former . various trials were taken to formulate the quick dissolving films ( qdf ) where in all the polymers at different concentrations were assessed . film containing hpmc and pva were transparent where as hpc , gelatin and cmc films were opaque in their appearance . as all batches do not have uniform amount of ingredients in it , hence their weight and thickness were varied . levocetirizine dihydrochloride in combination with hpmc e-15 and pva produced smooth , flexible and transparent oral thin strip . prepared strips were evaluated for different parameter like weight uniformity , folding endurance , % drug content , % drug release . from the result , it was observed that all the formulation shows good weight uniformity and % drug content . as concentration of polymer increase , decrease in folding endurance . hpmc e 15 alone shows lesser folding endurance 1555.5 . while only polyvinyl alcohol containing film shows greater folding endurance 452 3.2 . as concentration of polymer increase , drug release was decreased . only hpmc e15 containing film shows 92% drug release in 3 min . while only pva containing film showed 93% drug release in 3 min . maximum drug release of 98.32% 0.35 in 30sec was shown by patch containing 2:1 ratio of pva : hpmc in combination . the fast dissolving films containing levocetirizine dihydrochloride were prepared with an aim to have rapid onset of action and increased bioavailability in allergic conditions . various cellulose derivatives were employed for their film forming properties of which hpmc and pva showed promising physicochemical properties as compare to all other grades therefore , it was selected for further studies . prepared films were transparent with smooth surface and acceptable mechanical properties . it can be concluded that oral thin strip of levocetirizine dihydrochloride can prepared using the polymer combinations of hpmc e15 : pva ( 1:2 ) . when the concentration of polymer increase drug release decreases .

the aim of present research was to develop a fast releasing oral polymeric film , with good mechanical properties , instant disintegration and dissolution , producing an acceptable taste when placed on tongue . solvent casting method was used to prepare oral films . levocetirizine dihydrochloride , an antihistaminic was incorporated to relieve the symptoms of allergic rhinitis . the polymers selected were hpmc e 15 and pva . propylene glycol was the plasticizers used . nine batches of films with drug were prepared using different combinations of polymers and plasticizer concentration . the resultant films were evaluated for weight variation , content uniformity , folding endurance , thickness , surface ph , in vitro disintegration and in vitro dissolution . the optimized films which disintegrated in less than 30 sec , releasing 85 - 98% of drug within 2 minutes . the percentage release was varying with concentration of plasticizer and polymer . the films made with hpmc : pva ( 1:2 ) released 96% of drug in 1 min , which was the best release amongst all .

mrs . s 55-year - old woman was first observed by her family approximately 1 years back when she started complaining of headache and developed the belief that people were after the life of her only daughter and her children . she proceeded to say that her things were being stolen and her own relatives were involved in it . she would substantiate her accusations by saying that she could hear them talking to her and would start abusing her relatives visiting her without provocation . , she began to see every relative and neighbor as a threat to her daughter and herself . s started having complaints of giddiness while walking , vomiting , urinary incontinence and increased frequency and intensity of the headache . there was no known past or family history of mental illness , substance abuse or suicide . on examination , her blood pressure was found to be 118/84 mmhg with a pulse rate of 80/min . her glasgow coma scale score at the time of admission was 15 ( eye movement-4 , verbal response-5 , and motor response-6 ) . the neurological examination revealed ataxia and her fundus examination was normal . on mental status examination , she was extensively worked up to rule out various etiologies known to contribute to organic psychosis . patient had normal blood counts , hemoglobin , blood sugar , lipid profile , serum electrolytes , kidney and liver function tests . brain magnetic resonance imaging ( mri ) showed prominent sulcal spaces , and dilated ventricular system suggestive of diffuse cerebral atrophy and mild cerebellar atrophy [ figure 1 ] . brain magnetic resonance imaging showing prominent sulcal spaces , and ventricular system dilated with diffuse cerebral atrophy she received olanzapine 5 mg initially , she developed tremors for which trihexyphenidyl 2 mg was added . she was started on betahistine 8 mg 3 times a day , antiemetic drug was added . according to international classification of diseases-10 , the diagnosis fulfills the criteria for f06.2 , odds ( schizophrenia like ) . objective evidence of cerebral disorder by history is present in the form of classical triad of nph , and it was confirmed by mri brain findings . symptoms of mental disorder predominantly delusions , presented with the neurological complaints and evidence for another mental disorder or family history was absent . her brain mri revealed hydrocephalus with pan - ventricular enlargement without any evidence of a mass lesion . it suggests a process affecting the subarachnoid spaces or csf absorptive surfaces which may be seen in conditions causing acute inflammation or delayed scarring ( i.e. , trauma , infection or hemorrhage ) as well as with leptomeningeal metastases from a systemic cancer . cognitive symptoms present in these patients can be both because of age - related changes and also , because of the illness but the presence of delusions and hallucinations in an elderly is not usual presentation of psychotic symptoms in this age group . depression is also common in the elderly population but it must be distinguished from the negative symptoms of psychosis as apathy , which is seen to be a common presentation of psychosis due to or comorbid with nph in the elderly population . the first - line treatment for delusions and hallucinations , even in the elderly population , consist of antipsychotics . typical antipsychotics such as haloperidol and chlorpromazine are effective in treating these symptoms , but they have significant side effects including extrapyramidal symptoms atypical antipsychotics in low dosages are seen to be devoid of such side effects and are effective as treatment of psychotic symptoms in elderly population , though they have their own side effects such as risperidone can cause urinary incontinence , which are not generally seen at low doses . risperidone in the dosages from 1 to 4 mg has been used in nph with psychosis , without any significant problem and seems to be a good option for the treatment . one should be clinically vigilant in such late onset psychotic patients , presenting with behavioral complaints , without significant past psychiatric history . it is the thorough assessment , by which these patients can be recognized at an early stage and treatment can be instituted to reverse troublesome symptoms and thereby reduce the morbidity associated with late onset psychosis .

normal pressure hydrocephalus ( nph ) is caused by aqueductal stenosis resulting in syndrome of gait disturbance , cognitive impairment , and urinary incontinence . late onset psychosis should be evaluated thoroughly to rule out any organic cause . we present the case of a female patient presenting with delusions , gait disturbances , urinary incontinence in her 50s .

an internal hernia is defined as the protrusion of a viscus through a normal or an abnormal opening within the boundaries of the peritoneal cavity . trans - mesenteric hernia is a type of internal hernia which is an unusual cause of bowel obstruction . less than 1% of the cause of small bowel obstruction is due to internal hernia [ 1 , 2 ] . paraduodenal hernia continues to be the most common type of internal hernia ( 50% ) in the abdomen without prior surgery [ 3 , 4 ] compared with trans - mesenteric hernia in which the incidence ranged from 8 to 10% [ 5 , 6 ] . most of the reports of congenital trans - mesenteric hernia involve the pediatric population but even in this group of patients , the trans - mesenteric defect is infrequent [ 7 , 8 ] . a 21-year - old woman who was otherwise healthy before presented with severe abdominal pain . she was also found to have multiple episodes of vomiting for a period of 24 h. otherwise , she had no fever , no history of previous abdominal surgery , no other co - morbids and no trauma . on examination she was drowsy , tachycardic with a pulse rate of 120 beats / min , bp 130/78 mmhg , temperature 37.0c , dehydrated and respiratory rate of 24 breaths / min . laboratory investigations results were normal , except for an elevated white blood cell count of 16 000/mm . in view of generalized peritonitis , the abdomen was explored through an emergency midline laparotomy after initial resuscitation . upon entering the peritoneal cavity , a loop of ileum had herniated through a small mesenteric defect . a total of 180 cm ileum noted to be gangrenous with loss of peristalsis and absent pulsations ( fig . 1 ) . the mesenteric defect measuring 3 3 cm located about 4 cm from the mesenteric edge of mid - jejunum . a limited right hemicolectomy with extensive gangrenous small bowel resection was performed and a stapled side - to - side functional anastomosis created . the mesenteric defect mentioned earlier she was allowed orally on day 3 post surgery ( figs 24 ) . congenital defects in the mesentery have been described as an uncommon pathology , presented mainly in the pediatric population and these are very rare in adults . however , in this case we are reporting the mesenteric defect to be congenital and the cause for the bowel strangulation . defects are most commonly acquired in adults as a result of either blunt abdominal trauma or surgical manipulation of the bowel and mesentery . most of the literature reports that the most common form of presentation is an acute intestinal obstruction which is also the clinical presentation of our patient . it could be an intermittent obstruction as well , which delays the diagnosis and treatment [ 8 , 10 ] . as it is an uncommon etiology for intestinal obstruction most of the congenital mesenteric hernia diagnoses most of the time clinical features such as peritonitis and acute intestinal obstruction warrant for an immediate surgical intervention . morbidity and mortality of the condition depend mainly on the time of onset , clinical severity , the patient 's co - morbidities and vascular compromise .

trans - mesenteric hernia is a form of internal hernia which is an extremely rare cause of intestinal obstruction in adults compared with the pediatric population . it often presents with complications such as acute intestinal obstruction and peritonitis requiring immediate surgical intervention . we report a case of a 21-year - old woman who presented to us with an acute abdomen and peritonitis that required an immediate exploratory laparotomy . she was found to have a small congenital mesenteric defect with strangulated segment of ileum . gangrenous portion of the ileum was resected and the congenital defect was closed . the patient made an uneventful recovery . the occurrence of trans - mesenteric hernia in adults is rare and difficult to be diagnosed clinically ; thus , the patient 's clinical features may lead to early surgical intervention in order to reduce morbidity and mortality .

initially , we screened 85 fecal samples collected during 2006 through mid-2012 from farmed mink by nested pcr ; a broad panel of hev variants was detected ( 6 ) . one sample was positive , and subsequent sequencing and phylogenetic analysis showed that this virus represented a new hev variant . to screen more samples for this new virus , we developed a specific real - time reverse transcription pcr ( rt - pcr ) ( technical appendix ) . the initially tested 85 fecal samples and an additional 233 fecal samples from farmed mink , together with liver and fecal samples from 89 wild mink , were tested with this new and more sensitive assay . in addition , screening with an hev real - time rt - pcr ( 9 ) specific for g1g4 found none positive . the hev - infected mink were all submitted for diagnostic examination ; all had histories of diarrhea in the herd . lipidosis , aleutian mink disease virus , and catarrhal enteritis also were diagnosed in the mink ( online technical appendix table ) . the 4 samples positive for the novel hev variant were collected during 20082011 from herds across jutland , denmark , with a minimum distance of 80 km between the herds . the 4 pcr products obtained by the nested pcr , covering a region of 261 bp of the rdrp gene , were cloned and sequenced ( genbank accession nos . the sequences were 98%100% identical , with only 1 nonsynonymous mutation , resulting in a neutral amino acid change from isoleucine to valine ( technical appendix figure ) . the high homology in this region is not surprisingbecause the gene encodes the rna polymerase . we initially tried to uncover a larger fragment by primer walking , but the limited amount of material prohibited this . on the basis of the 261-bp fragment , we analyzed the phylogenetic relationship of this novel mink hev variant to variants found in other animals ( figure ) . the mink hev variant clustered with hev variants found in ferrets and rats , which grouped in a separate branch that was clearly distinct from other previously described hev variants . at nucleotide level , the mink hev variant was 65% identical to the closest classical hev genotype ( g3 and g4 ) and 76% and 69% identical with ferret and rat hevs , respectively . at the amino acid level , the homologies were more pronounced , showing 87% and 78% identity with ferret and rat hev variants , respectively . the grouping of the hev reference sequences in the analysis was identical with results of previously performed phylogenetic analysis on full - length sequences ( 12 ) . phylogenetic tree showing the relationship between the novel mink hepatitis e virus ( hev ) , other hev variants , and the 4 known hev genotypes based on 261 bp of the rdrp gene . the clc main workbench software ( clc bio , aarhus , denmark ) was used for the phylogenetic analysis . alignments were made by using muscle algorithm ( www.drive5.com/muscle/ ) and phylogenetic tree was made by using distance - based method with the neighbor - joining algorithm and bootstrap value of 1,000 . we detected a variant of hev in 4 farmed mink from 4 geographically distinct locations in denmark during a 3-year period , which indicates that the virus has been circulating among mink . phylogenetic analysis showed that the virus was clearly distinct from , but closely related to , ferret and rat hev variants recently reported from germany and the united states ( 6,7,13 ) . it has not been possible to infect primates with rat or avian hev variants ( 13,14 ) . thus , because of the phylogenetic resemblance of mink hev with these nonzoonotic hev variants , there are no indications that mink hev can infect humans , although no human samples have been tested specifically for this virus . the zoonotic potential of hev has been documented only in the case of g3 and g4 , which were not found in mink . however , considering the relatively high hev seroprevalence in humans , the possibility of other variants being zoonotic and cross - reacting with hev g1g4 in serologic assays can not be ruled out . the mink in this study were from herds that had mink enteritis virus , hepatic lipidosis , aleutian mink disease virus , and catarrhal enteritis , all factors that could explain the conditions of the mink infected with hev ( 15 ) . however , it can not be ruled out that the mink hev variant contributed to the clinical signs of the mink hev - positive animals . to determine whether the virus is indeed capable of inducing clinical signs in mink , the animals need to be experimentally infectedd . however , the rat and ferret hev variants induced almost no histologic signs in rats after experimental infection , and the ferrets were described as not showing overt clinical signs ( 7,13 ) . so far , only chickens infected with ahev and humans infected with hev g1g4 have been described as being clinically affected by hev infections . the possibility exists that the hev variants recently reported in a variety of different species , including the 1 reported here , could evolve into disease - causing pathogens in animals and possibly also humans . development of a specific real - time reverse transcription pcr to screen samples for the novel hepatitis e virus ( hev ) variant ; characterization of the 4 hev - positive samples ; and alignment of the 4 mink positive for hev variant sequences obtained by the nested pcr .

hepatitis e virus ( hev ) is a zoonotic virus for which pigs are the primary animal reservoir . to investigate whether hev occurs in mink in denmark , we screened feces and tissues from domestic and wild mink . our finding of a novel hev variant supports previous findings of hev variants in a variety of species .

we report a woman with focal dermal hypoplasia ( fdh ) and blaschko linear enamel defects . fdh or goltz - gorlin respectively goltz syndrome is a rare genetic skin disorder within the large spectrum of ectodermal dysplasias . its protein plays a key role in the wnt pathway , which has an impact on embryonic development . the inheritance is x - linked dominant , therefore 90% of affected people are female . apart from the skin , common manifestations appear on the teeth , eyes , skeleton and face . a first dermatological examination at the age of 19 years showed pigmentary changes , atrophic skin areas and telangiectasias . hypopigmentation appeared as focal , pea - like , white spots on the thorax , back , forearms , knees , front thighs and secondary inguinal . furthermore syndactyly ( toes ii / iii ) and fat herniations with piezogenic papules were found . the dental changes included malposition of the lower incisors and vertical grooving of all teeth ( fig . in addition to the above - mentioned symptoms and findings , more typical features of the disease were detected . the case history also revealed that she has been suffering from reflux since the age of 20 . poikiloderma ( skin areas of hypo- and hyperpigmentation , telangiectasis and atrophy ) , particularly on the legs , was confirmed ( fig . the patient suffered from periocular scars ( status after several laser therapies ) and perioral papules with hypertrichosis . the patient has already had five abortions and has one son with astigmatism , myopia and hyperopia . her mother had several abortions too and also showed conspicuous symptoms : slight depigmentation on the legs , lipomas , brittle hair and teeth variations . the further procedure includes frequent dermatological examinations ( due to painful skin lesions ) , search for the mutation ( identified in the patient ) in the mother and physiotherapy to strengthen the musculoskeletal system . our patient shows typical signs of fdh . beside the atrophic skin areas and fat herniations , hypo- and hyperpigmentation along the blaschko lines the orthopedic ailments consist of syndactyly and scoliosis with its consequences ( e.g. pain ) . the clinical features are generally more pronounced on her left side , an interesting observation already described in other cases . the dental changes include malposition , fragile teeth , enamel hypoplasia with caries and generalized vertical enamel grooving , all distinctive signs for goltz syndrome . we hypothesize that , in the context of typical skin changes , visible blaschko lines on the teeth in the form of vertical grooves are almost pathognomonic for fdh . still , for a definite diagnosis , a detailed clinical investigation of the whole patient and a genetically proven mutation is required in any case . a possible differential diagnosis of dental analogies of blaschko lines with enamel impairment is x - linked amelogenesis imperfecta , though those patients normally do not show any other symptoms than caries , abnormal color and temperature hypersensitivity of the teeth . unfortunately her mother did not agree to genetic counseling and analysis , thus we do not know for sure whether it is a familial or a sporadic case of fdh . however , this case provides evidence for an inherited , familial case . mother and daughter furthermore the mother suffers from depigmentation of the skin , multiple lipomas , brittle hair and dental changes , without a doubt characteristic symptoms for fdh . however , without the genetic proof of mutation the disease can not be verified 100% . our patient 's son can not have inherited the mutation from his mother due to the fact that heterozygous male embryos are not viable . on suspicion of familial cases of goltz syndrome , genetic counseling and analysis is highly recommended . once the disease - causing gene in a family is identified , prenatal molecular genetic testing can be conducted in expectation of pregnancy . striated lines on the teeth in combination with skin changes and further meso-/ectodermal alterations point to the diagnosis of goltz syndrome ( fdh ) . especially the dental ridges , representing blaschko lines , are almost pathognomonic if suspicious features on the skin are found , the teeth should be examined and vice versa . in any case cooperation between dermatologists , dentists and other specialists genetic counseling is highly recommended , so that the exact location of the porcn gene mutation can be identified . if patients desire to have children , the identified mutation simplifies prenatal molecular genetic testing .

focal dermal hypoplasia ( fdh ) is a rare genetic skin disorder . the inheritance of fdh or goltz - gorlin syndrome is x - linked dominant and the disease is associated with a porcn gene mutation . this gene plays a key role in the wnt pathway , which has an impact on embryonic development . every tissue derived from meso- and ectoderm can be affected . patients suffer from cutaneous , ocular , osseous , oral and dental defects . the skin and dental alterations manifest along the blaschko lines . we present a woman ( born in 1962 ) suffering from fdh with congenital skin changes and blaschko linear enamel defects . typical symptoms ( e.g. fat herniations , scoliosis , syndactyly , microphthalmia , caries and alopecia ) plus vertical grooving of all teeth gave a first indication . molecular genetic testing confirmed the definitive diagnosis of fdh . we hypothesize that , in the context of typical skin changes , visible blaschko lines on the teeth in the form of vertical grooves are almost pathognomonic for fdh .

carcinoid tumors are neuroendocrine tumors originating from the enterochromaffin cells primarily of the gut and also occasionally from the pulmonary , renal or gonadal ( ovaries and testes ) tissues . they secrete various neurohumoral chemicals including serotonin , histamine and kallikerin which produce the clinical manifestations of carcinoid syndrome ( red hot flushing of the face , neck and upper chest , severe and debilitating diarrhea , abdominal pain , palpitations , bronchospasm ) . carcinoid tumors of the testes are uncommon with only 1 - 3% of them being associated with carcinoid syndrome and hence their preoperative clinical suspicion is extremely low . a 36-year - old man with no significant past medical history returned to the urology clinic with nocturia , dysuria , and persistent right testicular pain despite 1 month of analgesic therapy . an outpatient scrotal sonogram done 1 month before for this reason showed a 3-mm hypoechoic left testicular mass and some punctate calcifications but no masses or other pathology of the right testicle ( fig . furthermore , he was noted to have swelling and some tenderness over the right testicular area . a repeat scrotal sonogram was obtained and showed a stable left testicular mass with suspicion for malignancy and right testicular findings similar to those on the previous sonogram . in view of these findings , the patient underwent an elective left - sided orchiectomy , and pathology revealed a carcinoid tumor ( fig . the tumor was unifocal and no other concurrent pathology ( such as teratoma ) was seen . testicular carcinoids are rare neoplasms of the testes occurring commonly in the 40 - 60-year age group , though cases involving younger populations have been reported . only about 3% of these tumors generally , larger tumors and those associated with carcinoid syndrome are metastatic in origin . about a quarter of the primary testicular carcinoids are mixed with teratoma which then portends an indolent and benign course . also for this reason the common presentation is a painless or painful testicular mass though sometimes patients may present with a nonpalpable mass found incidentally on imaging . sonogram shows a hypoechoic mass with punctate calcifications within the testicle , and histology shows the typical nesting pattern of neoplastic cells . definitive diagnosis is done on biopsy post - orchiectomy . grossly , the tumors are solid , yellow or light brown to tan , well - circumscribed but unencapsulated masses . immunohistochemical analysis shows reactivity of the tumor cells to various neuroendocrine markers including chromogranin a , synaptophysin , neuron - specific enolase and cd117 . the prognosis is poor in case of metastatic carcinoids irrespective of the site of the primary tumor . delayed recurrence of up to 17 years has been documented making postoperative follow - up essential and which consists of whole - body scanning ( ct scan of the chest , abdomen and pelvis ) and measurement of urinary 5-hiaa or serum serotonin and chromogranin a levels . the atypical presentation of ipsilateral testicular carcinoid with contralateral testicular pain , nocturia and dysuria has not been previously reported . it is important to recognize this uncommon presentation to avoid missing or delaying the diagnosis of this rare but ominous clinical entity .

testicular carcinoid generally presents either with a palpable ( painful or painless ) or rarely a nonpalpable mass ( when it is diagnosed incidentally on scrotal imaging ) of the involved testis . an ipsilateral testicular carcinoid presenting exclusively with contralateral testicular symptoms has never been described in the literature to date . we report a case of nonpalpable left testicular carcinoid diagnosed incidentally on a sonogram done for right testicular pain and swelling , with conspicuous absence of left testicular symptoms . this case highlights the importance of recognizing such an atypical presentation of testicular carcinoid .

reagents like bromoethanol , edchcl , n - methylpiperazine , n - ethylpiperazine , n - acetylpiperazine , n - phenylpiperazine , potassium dihydrogen phosphate , 1-octanol . high performance liquid chromatography ( hplc ) method has been developed for the determination of various evaluation parameters such as aqueous solubility , partition coefficient etc . prodrugs should be designed in such a way that they should fulfill the ideal properties required for percutaneous drug delivery i.e. pka , molecular weight , log p etc . designed derivatives which fulfill thecriteria have been synthesized and characterized by spectral studies ( uv , ir , mass , and nmr ) for structural confirmation and further evaluated . aqueous solubility of the derivatives was determined at room temperature by dissolving excess of the compound in 0.5 - 1 ml of the phosphate buffer ( 0.20 m ) at both ph 5.0 - 5.5 and 7.4 . partition coefficients of the derivatives were determined at room temperature in 1-octanol and phosphate buffer ( 0.20 m ) at both ph 5.0 - 5.5 and 7.4 . before use , stability study of the derivatives was performed in phosphate buffer ( usp , 0.20 m ) at both ph 5.0 and 7.4 and also in human plasma . the receptor medium ( 0.05 m isotonic phosphate buffer solution of ph 7.4 ) was stirred and kept at 37c throughout the study . prodrugs should be designed in such a way that they should fulfill the ideal properties required for percutaneous drug delivery i.e. pka , molecular weight , log p etc . designed derivatives which fulfill thecriteria have been synthesized and characterized by spectral studies ( uv , ir , mass , and nmr ) for structural confirmation and further evaluated . aqueous solubility of the derivatives was determined at room temperature by dissolving excess of the compound in 0.5 - 1 ml of the phosphate buffer ( 0.20 m ) at both ph 5.0 - 5.5 and 7.4 . partition coefficients of the derivatives were determined at room temperature in 1-octanol and phosphate buffer ( 0.20 m ) at both ph 5.0 - 5.5 and 7.4 . before use , stability study of the derivatives was performed in phosphate buffer ( usp , 0.20 m ) at both ph 5.0 and 7.4 and also in human plasma . the receptor medium ( 0.05 m isotonic phosphate buffer solution of ph 7.4 ) was stirred and kept at 37c throughout the study . aqueous solubility of the derivatives was determined at room temperature by dissolving excess of the compound in 0.5 - 1 ml of the phosphate buffer ( 0.20 m ) at both ph 5.0 - 5.5 and 7.4 . partition coefficients of the derivatives were determined at room temperature in 1-octanol and phosphate buffer ( 0.20 m ) at both ph 5.0 - 5.5 and 7.4 . before use , stability study of the derivatives was performed in phosphate buffer ( usp , 0.20 m ) at both ph 5.0 and 7.4 and also in human plasma . the permeation studies were carried out using the franz - type diffusion cell . the receptor medium ( 0.05 m isotonic phosphate buffer solution of ph 7.4 ) was stirred and kept at 37c throughout the study . designed prodrugs were synthesized and their structures confirmed by various analytical techniques and their physicochemical properties like aqueous solubility and log p were also evaluated . derivatives 5a - b showed increased aqueous solubility in comparison to the parent nsaid but derivative 5c - d showed reduced aqueous solubility than the parent nsaid . stability data indicated that prodrugs are stable in phosphate buffer but got quickly hydrolyzed in plasma due to presence of ester group in prodrugs and released active drug in skin to show activity . from the skin permeability study it was concluded that derivatization of free carboxylic acid group by hydrophilic moiety increases skin permeation . the present study shows that the permeation of nsaid through rat skin can be significantly improved by using piperazinylalkyl prodrugs . the good skin permeation observed for these prodrugs can most likely be attributed to their simultaneously high aqueous solubility and lipophilicity , the combination of which is important for permeation across the skin.[13 ]

arthritis is a heterogeneous group of conditions that leads to joint symptoms and signs which are associated with defective integrity of articular cartilage . major classes of drugs which are widely used for the treatment of arthritis are non - steroidal anti - inflammatory drugs ( nsaids ) . development of an efficient means of percutaneous delivery can increase drug concentration in local soft - tissues and joints while reducing the systemic distribution of a drug and its side effects . the present work is aimed at synthesisand evaluation of prodrugs of some nsaids for percutaneous drug delivery for the treatment of arthritis .

the online version of this article ( doi:10.1007/s12020 - 013 - 0117 - 6 ) contains supplementary material , which is available to authorized users . a 30-year - old woman was referred for fine - needle aspiration biopsy ( fnab ) of a lesion located in an ectopic thyroid . at the moment the patient was born ( 1982 ) , neonatal screening for congenital hypothyroidism had not yet been introduced in poland . hence , the data on her tsh value at birth are not available . the ultrasound examination showed the absence of the thyroid gland in the typical localization but the scintiscan revealed an ectopic thyroid in the left submandibular region of the neck . levothyroxine substitution was initiated at the dose of 50 g daily and then continued with 75 g daily . her parents did not present features of hypothyroidism . however , they were not willing to undergo further diagnostic tests . [ tsh 1.71 iu / ml , ft4 12.91 pmol / l ( normal 11.521.0 ) , and ft3 4.76 pmol / l ( normal 3.937.70 ) ] , free of symptoms and had not been taking levothyroxine for at least six months . physical examination revealed a palpable painless lump in the left submandibular region of the neck . concentration of anti - thyroid peroxidase antibodies and anti - tsh receptor autoantibodies ( measured with the 2nd generation brahms trak human ria ; berlin , germany ) was increased ; 87 iu / ml ( normal < 34 ) and > 40 iu / l ( normal < 2 ) , respectively , while anti - thyroglobulin autoantibodies were 68 iu / ml ( normal 10115 ) . no signs of endocrine orbitopathy were noticed . the ultrasound examination revealed an absence of the orthotopic thyroid gland and the presence of an unilateral ectopic thyroid localized in the left submandibular region . the bilobed ectopic gland was 5.9 ml in volume and presented markedly decreased echogenicity as well as highly increased vascularization on color doppler examination . additionally , a focal lesion of 15 mm in size was observed in the left thyroid lobe . fnab of the lesion , thyroid tc-99 m scintiscan , and a mri of the neck were subsequently performed ( fig . 1 ) . a mri confirmed the presence of an ectopic thyroid located between the thyrohyoid and sternohyoid muscle on the left with a bilobed structure , with the right lobe located anteriorly toward the thyroid cartilage on the left and the left lobe placed higher , extending to the medial wall of the left submandibular salivary gland . a total thyroidectomy was performed and the result of the histopathological examination revealed the presence of chronic thyroiditis and fibrosis . the focal lesion in an ectopic thyroid gland was identified as a benign hyperplasic nodule with oxyphilic metaplasia.fig . 1 a t2-weighed mri stir ( short inversion time inversion recovery ) image in coronal plane . an arrow indicates an ectopic thyroid , b t2-weighed mri stir image in transverse plane . an arrow indicates a focal lesion in an ectopic thyroid a t2-weighed mri stir ( short inversion time inversion recovery ) image in coronal plane . an arrow indicates an ectopic thyroid , b t2-weighed mri stir image in transverse plane . an arrow indicates a focal lesion in an ectopic thyroid an ectopic thyroid is a rare thyroid developmental anomaly usually presenting with congenital hypothyroidism . our search of the english language literature revealed only three reports of patients presenting an ectopic thyroid with no thyroid tissue in the orthotopic localization and graves disease , two of them associated with orbitopathy [ 13 ] . to our knowledge , our patient is the first person described with an ectopic thyroid presenting with a nodular variant of graves disease and no signs of orbitopathy , who was initially hypothyroid and became euthyroid presumably because of the autoimmune thyroid disease .

a 15-year - old woman was diagnosed with hypothyroidism and unilateral ectopic thyroid . levothyroxine treatment was introduced ; however , the patient was non - compliant and took the medication irregularly . when she presented to an endocrinologist at the age of 30 , she had not been using levothyroxine for at least 6 months . surprisingly , she was clinically and biochemically euthyroid . due to decreased echogenicity on ultrasound examination , enhanced vascularization on color doppler examination and increased concentration of anti - tsh receptor autoantibodies , she was diagnosed with graves disease . eventually , she underwent total thyroidectomy due to diagnosis of follicular neoplasm in fine - needle aspiration biopsy of the focal lesion found in the thyroid gland . to our knowledge , our patient is the first described with ectopic thyroid , presenting a nodular variant of graves disease and no signs of orbitopathy , who was initially hypothyroid and became euthyroid in the course of autoimmune thyroid disease.electronic supplementary materialthe online version of this article ( doi:10.1007/s12020 - 013 - 0117 - 6 ) contains supplementary material , which is available to authorized users .

of all hernias encountered , incisional ( ventral ) hernia can be the most frustrating and difficult to treat . they usually occur as the result of a failure of fascial tissues to heal and close following laparotomy . the use of polypropylene mesh was initially reported by usher as a means of repairing incisional hernias . meanwhile , about 1 million meshes are implanted worldwide per year . the most common complication associated with polypropylene mesh fistula formation from a polypropylene mesh has been reported to occur particularly if the prosthetic mesh is placed adjacent to the stomach or in contact with the small or large intestine . a 60-year - old man presented to us with a chronic foul smelling discharge with a typical history of passage of gas from a subumbilical discharging sinus for 6 months , with a history of repair of an incisional hernia following an open truncal vagotomy and gastro - jejunostomy . the incisional hernia was repaired with laparoscopic polypropylene mesh hernioplasty and 1 month after the surgery , he had developed the chronic discharging sinus . he was treated conservatively for more than 6 months without any result before presenting to our hospital . therefore , he was planned for an exploratory laparotomy [ figures 1 and 2 ] . fistulogram showing dye entering to colon polypropylene mesh eroding colon the abdomen was opened through a mid - line incision following a no . 10 infant feeding tube placed in the fistula tract . as expected , a part of the culprit polypropylene mesh was found to have eroded the mid - transverse colon , causing fistula . primary resection and end - to - end anastomosis of the transverse colon were done along with the removal of the polypropylene mesh and the fistulous tract . post - operative recovery was uneventful , and the patient was discharged on the eighth post - operative day , with follow - up at regular intervals . 10 infant feeding tube placed in the fistula tract . as expected , a part of the culprit polypropylene mesh was found to have eroded the mid - transverse colon , causing fistula . primary resection and end - to - end anastomosis of the transverse colon post - operative recovery was uneventful , and the patient was discharged on the eighth post - operative day , with follow - up at regular intervals . it also helps to identify additional hernial defects ( swiss cheese ) in the anterior abdominal wall during repair . the goal of dissection is to provide a minimum of 5 cm overlap of the mesh beyond the edges of the hernia defect . in the present era , ventral hernias are repaired by the laparoscopic intraperitoneal onlay mesh ( ipom ) technique using tissue separating / dual meshes to reduce adhesive complications ; few surgeons are trying to use the laparoscopic ipom technique with polypropylene mesh by raising a flap such as tandem ph domain containing protein ( tapp ) , even though the cost factor is taken care of but the long - term results of this technique is not evaluated till date . protein absorption theory is the most acceptable theory regarding the incorporation of mesh into surrounding tissues ( by inducing foreign body reaction ) . intraperitoneal placement of a polypropylene mesh has a high propensity for complications such as seroma , wound infection , adhesions , recurrence , enterocutaneous fistula , etc . , and that is the reason why people are switching over to tissue separating meshes . general division 's nasdaq : genz ) and proceed ( cincinnati , usa ) a thin coating of bioabsorbable material layer on polypropylene mesh , gore - tex , physio etc are now the prosthesis of choice in laparoscopic ventral hernia repair by ipom technique . the prosthesis is fixed in place with either transfascial sutures or tacks ( pro tack / secure strap ) placed circumferentially in a double crown fashion . mesh erosion into caecum , ascending colon , rectum ( following mesh rectopexy ) , stomach , small intestines , and bladder is available in the literature , but erosion into transverse colon is rare . hence we conclude in the present era in a laparoscopic ventral / incisional hernia repair only tissue separating / dual meshes should be used , so that the inadvertent complications like the one described in this case can be minimised . some surgeons even though are using polypropylene mesh in the ipom technique to cut down on the cost of surgery , they should resist themselves from using polypropylene mesh in the ipom technique , rather if a surgeon contemplates to use a polypropylene mesh in laparoscopic ventral hernia repair , then he should create a preperitoneal flap for placement of mesh , though it still carries a risk for adhesion .

polypropylene mesh when used in laparoscopic ventral hernia repair can produce the worst complication such as enterocutaneous fistula . we report an interesting case of incisional hernia operated with laparoscopic polypropylene mesh hernioplasty who subsequently developed an enterocutaneous fistula 1 month after surgery . a fistulogram showed dye entering into the transverse colon . on exploration , the culprit polypropylene mesh was found to have eroded into the mid - transverse colon causing the fistula . resection and end - to - end anastomosis of the colon were done with the removal of the mesh . on literature review , polypropylene mesh erosion in to transverse colon is rare .

the unicuspid aortic valve ( uav ) is a rare form of congenital heart disease , mainly confused with bicuspid aortic valve and presents with aortic stenosis [ figures 14 ] . others may remain hemodynamically stable for several years before they require intervention , especially in the unicommissural form . here , we present a case of uav with moderate aortic stenosis detected by echocardiography in the infant . transthoracic echocardiography at parasternal short axis view shows posteriorly situated eccentric aortic valve orifice in systole transthoracic echocardiography at parasternal short axis view shows the one lateral commissural attachment to the aorta posteriorly in diastole parasternal long axis view shows dome - shaped configuration of aortic valve during systole parasternal long axis view shows dome - shaped configuration of aortic valve during diastole absence of failure to thrive and no signs of congestive heart failure on clinical evaluation . on general examination , the child was afebrile , pallor , no cyanosis or clubbing , and jugular venous pressure was not raised . transthoracic echocardiography revealed a posteriorly situated eccentric aortic valve orifice extending to the annulus in systole . it showed noncalcified , unicuspid unicommissural aortic valve with moderate aortic stenosis ( peak pressure gradient of 50 mmhg ) . the size of the aortic annulus ( 08 mm ) , aortic root ( 13.7 mm ) and ascending aorta ( 12.4 mm ) . no other associated congenital heart disease was noted [ video 12 ] . the aortic valve develops from three tubercles that are converted into thin valve cusps and sinuses of valsalva by a process of excavation . a uav is formed by the fusion of all the three cusps . the free edge of the single leaflet originates from the single attachment . uav may have a central opening ( no true commissure / acommissural ) or an eccentric orifice ( unicommissural ) as in our case . the acommissural type has no lateral attachment to the aorta with a central orifice , and the unicommissural type has one attachment with an eccentric orifice that could be linear ( like an exclamation mark ) , triangular , oval , or tear drop shape . the acommissural form usually causes severe stenosis and is highly symptomatic at a neonatal age . the uav is a rare congenital malformation seen in 0.019% of patients during the echocardiographic evaluation and in 5.59% of patients during aortic valve replacement . severe aortic stenosis or mixed stenosis and regurgitation are the predominant disorder that accompanies patients with a uav . it presents far more often in males than females , often in the third decade of life when aortic stenosis becomes clinically significant . the uav is prone to be associated with dilatation or dissection of the aorta , involving the aortic root , ascending aorta , or aortic arch , which typically requires surgical intervention . other associated disorders include aortic coarctation , an aberrant right subclavian artery , and a single coronary artery and ventricular septal defects ( vsds ) . the importance of an early diagnosis of uav lies in its risk of sudden cardiac death and association with other congenital anomalies , such as patent ductus arteriosus ( pda ) , vsd , anomalous coronary arteries , and coarctation of the aorta . recently , magnetic resonance imaging , cardiac computed tomography , and multislice tomography angiography used as diagnostic tool for uav . whereas , echocardiography remains a useful and reliable method for the diagnosis of a uav . systemically reviewed nine articles with 60 pediatric cases ( age < 15 years ) of uav . he reported the most common presentation of uav was congestive heart failure due to congenital aortic stenosis , and the most common lesion was isolated aortic stenosis , reported in 19 cases ( 32% ) . associated anomalies included 22 cases ( 37% ) of aortic coarctation , 7 ( 12% ) with vsd , 3 ( 5% ) with pda , and 3 ( 5% ) with aortic aneurysm . the preoperative diagnosis of uav was rare , and 33 cases ( 55% ) of pediatric uav were reported at autopsy or at the pathological examination of the surgically excised valves . uav are usually stenotic at birth requiring aortic valve replacement in third decade of life . others may remain hemodynamically stable for several years or decades before they require intervention for calcified stenosis or regurgitation . although stenotic unicuspid valves are treated by valvotomy , regurgitant uavs are commonly aortic valve replaced . aortic valve repair , including bicuspidisation , can be performed with low risk and excellent operative results . conclude that patients with uavs will present for cardiac surgery in their third decade of life . this case demonstrates incidental diagnosis of uav with moderate aortic stenosis in an infant who is asymptomatic and not accompanies any other congenital heart diseases . the surgical intervention had been deferred in this case and closely followed with echocardiography for aortic stenosis severity .

the unicuspid aortic valve ( uav ) is a very rare congenital anomaly , which usually presents as aortic stenosis , incompetence , or a combination of both . here , we present a case of uav with moderate aortic stenosis detected by transthoracic echocardiography in the infant .

congenital anomalies of the coronary arteries appear in coronary angiography with an approximate incidence of 0.61.3% . of these , 90% are abnormalities of the origin of the coronary arteries , and the rest are coronary artery fistulas [ 1 , 2 ] . we report a case of right coronary artery ( rca ) arising as a proximal branch of the left main coronary artery ( lmca ) near the left sinus of valsava , which was confirmed by coronary angiography and surgically repaired by rca reimplantation into the right sinus of valsava using saphenous vein patch ( svp ) . a 37-year female patient with no prior medical illnesses was admitted multiple times to the hospital complaining of palpitations , dyspnea on exertion and angina since june 2010 . the patient was a non - smoker , and carried no risk factor of ischemic heart disease . additional work - up included laboratory investigations ( cardiac enzymes , kidney function test and liver function test ) , electrocardiography , echocardiogram ( ejection fraction 65% ) and chest x - rays were all normal . cardiac catheterization , computed tomography angiography ( cta ) and constructional computed tomography ( figs 1 and 2 ) were done and revealed aberrant rca origin as a proximal branch from the lmca then adapts its position between the pulmonary artery and ascending aorta where it is slightly compressed during systole . figure 1:axial cta demonstrates an anomalous origin of rca ( arrow a ) which arises as a branch from lmca ( arrow b ) . figure 2:constructional ct demonstrates an anomalous origin of rca ( arrow a ) which arises as a branch from lmca ( arrow b ) . axial cta demonstrates an anomalous origin of rca ( arrow a ) which arises as a branch from lmca ( arrow b ) . constructional ct demonstrates an anomalous origin of rca ( arrow a ) which arises as a branch from lmca ( arrow b ) . the patient was counseled with the surgical correction , and the consent form was signed by her . a median sternotomy was performed , and mild hypothermic ( 35c ) cardiopulmonary bypass was started . after exposing the left artery and rca , we found that the origin of the rca originated from the left sinus of valsalva . the rca was dissected from its attachment to the left main at the left sinus of valsalva . the svp measuring 1.5 2 cm sewn to the ascending aorta , the rca reimplanted into the svp using 7 - 0 polypropylene continuous suture forming neo - ostium of the rca . the perioperative course went smoothly . over the past 3 years serial follow - up visits in the outpatient clinic , congenital coronary artery anomalies are present in ~0.61.3% of adults undergoing coronary angiography and rarely involve the rca . anomalous origin of rca from the lmca is an infrequently encountered condition . however , it is associated with variable clinical presentations , the extremes of which are no symptoms to sudden death , having in between easy fatigability , angina pectoris and myocardial infarction in the absence of atherosclerosis . this previously asymptomatic 37-year - old patient presented with continuous complaints of dyspnea on exertion associated with chest pain . making her candidate for cta which demonstrated the very rare anomalous origin of the rca as a proximal branch from the lmca that then continues between the pulmonary artery and ascending aorta where it is slightly compressed during systole . this finding provides us with the explanation of the presenting symptoms of such a case , which is supported by grollman et al . conservative treatment was proposed in japan as a main line of treating patients with anomalous origin of coronary arteries . this includes limitation of exercise and drug therapy ( such as nitrates , calcium channel blockers , b - adrenergic antagonists and antiarrhythmics ) showing an apparent improvement in 69.2% . surgical treatment should aim at restoring the normal anatomical position of rca to the original right ostium . literature review suggests several different approaches , including coronary artery bypass , coronary reimplantation and unroofing of the intramural segment ( also termed as osteoplasty ) [ 4 , 7 , 8 ] . in the 1980s , bett et al . used a single saphenous vein graft inserted from the aorta to the rca as it emerged from behind the pulmonary artery . reul et al . reported patients who underwent ligation of the anomalous coronary artery and bypass with a saphenous vein graft , and others where the right internal mammary artery graft had been used without ligation of the rca . the present case was treated by rca reimplantaion using the svp , measuring 1.5 2 cm , aiming at preventing future stenosis at the site of reimplementation . according to the literature , this exact use of saphenous patch was not used before in the surgical therapy of rca anomalous origin . however , this has been introduced in surgical treatment of other coronary artery diseases ; kuwaki et al . who used an svp for the reconstruction of left anterior descending coronary artery aneurysm , and raffa et al . reported a case in which an svp plasty was used in the surgical therapy of lmca ostial disease . in conclusion , long - term studies on the surgical repair techniques are needed , which will help in early identifying of such cases and decreasing the mortality of lately managed cases . this research received no specific grant from any funding agency in the public , commercial or not - for - profit sectors .

anomalous origin of the coronary arteries is a rare congenital heart defect that may lead to disturbed life style , myocardial infarction and sudden death . this report describes a young lady with the right coronary artery arising from the left main coronary artery , which was confirmed by coronary angiography and corrected surgically using saphenous vein patch .

continuous intrathecal baclofen infusion therapy ( ibt ) makes use of an implanted programmable pump to deliver baclofen , a derivative of gamma - aminobutyric acid ( gaba ) , directly to the cerebral spinal fluid . ibt has been demonstrated to be effective in controlling spasticity and muscle spasms following spinal cord injury [ 1 , 2 ] . patients with spinal cord injury may also suffer from dysphagia or insufficient oral intake , requiring long - term enteral tube nutrition in some cases . percutaneous endoscopic gastrostomy ( peg ) has been established as a minimally invasive procedure to achieve percutaneous tube feeding for long - term enteral nutrition [ 3 , 4 ] . although the presence of a peg tube has been reported as the greatest significant predictor of infection in patients undergoing ibt pump implantation , there is limited literature regarding the safety or rate of infection among patients with ibt undergoing peg . we herein report the successful peg tube placement in a 68-year - old man with a previously implanted ibt pump . a 68-year - old man was referred to our hospital to undergo peg for long - term enteral tube feeding . about one and a half years ago , he encountered a traffic accident which gave him a cerebral contusion and diffuse axonal injury . he was left with quadriplegia and dysphagia , being tube - fed via a nasogastric tube since the accident . the patient experienced severe muscle spasms from his spinal cord injury and about a year ago had an ibt pump implanted subcutaneously into his left inguinal area to help alleviate his symptoms . laboratory data were mostly within the normal range and abdominal ct evaluation indicated that peg was possible . the patient underwent peg using the introducer technique with a 20 fr size catheter kit ( intolief peg kit by create medic co. , ltd , yokohama , japan ) . after determining the puncture site , gastropexy procedure was repeated at least three times surrounding the intended puncture site in a triangular pattern before the puncture needle / trocar was inserted ( fig . 1c ) and a 20 fr size gastrostomy tube was inserted into the gastric lumen ( fig . ( a ) after determining the puncture site , local anesthesia was applied and gastropexy was performed using a gastropexy device . ( b ) gastropexy procedure was repeated at least three times surrounding the intended puncture site in a triangular pattern before the puncture needle / trocar was inserted . ( d ) a 20 fr size gastrostomy tube was inserted into the gastric lumen . postoperative clinical course was also uneventful and there was no peristomal infection at 1 week after the procedure . after achieving stable intermittent gastric feeding via the peg tube , the patient was discharged 10 days after the procedure without further complications . follow - up on day 30 after the procedure confirmed that there were no adverse events related to the ibt pump implant . an abdominal radiograph taken on day 2 after the procedure showed the position of the gastrostomy tube ( fig . 2 , red arrow ) . abdominal radiograph revealing the position of the peg catheter ( yellow arrow ) relative to the implanted ibt pump ( red arrow ) . our case demonstrated that peg is a procedure that can be safely performed in a patient with a previous ibt pump implantation . although previous peg tube placement seems to contribute to ibt pump - related infections , the route of infection remains unclear . however , it should be noted that the most common causative agent in ibt pump - related infection ( which may occur in up to 9% of implanted patients ) has been shown to be staphylococcus aureus [ 5 , 6 ] , which is common in skin and soft tissue infections . since peristomal infection has also been reported as the most common adverse event after peg , it is quite plausible that ibt pump - related infection after peg tube placement occurs mainly through the skin or connective tissue . the occurrence of implantation site infection is not something that can be taken lightly because it may develop to meningitis if not managed properly . although systemic antimicrobial prophylaxis has been shown to reduce the incidence of post - peg peristomal infection , it does not totally eliminate the risk of infection . peg using the introducer technique , in which the gastrostomy tube is inserted without passing through the oral cavity , has also been associated with lower peristomal infection rates compared to conventional pull / push techniques . nevertheless , this technique is not widely used because the size of the peg catheter employed is often small . recently , large - caliber tubes using this technique , such as in our case , can also be safely inserted without increasing the risk of peristomal infection . in our patient , peg performed after ibt implantation seems to be reasonably safe although there are no large studies we are aware of to confirm this . if infection does occur mainly via the skin and soft tissue , further measures to avoid ibt pump - related infection after peg tube placement such as the choice of tube insertion technique as well as systematic antibiotics administration should be strongly considered .

percutaneous endoscopic gastrostomy ( peg ) is the method of choice in patients requiring long - term enteral tube feeding . in patients with intrathecal baclofen infusion therapy ( ibt ) pump implantation , infection via the skin and soft tissue may be an issue of concern . the introducer technique for gastrostomy tube insertion may be useful in reducing the risk of peristomal infection . although the presence of a peg tube has been reported as a risk factor for implantation site infection ( for newly inserted ibt pumps ) , whether existing ibt pumps are at risk for infection during or after the placement of a peg tube is not clear . we report a case where peg was successfully performed using the introducer technique on a patient with an ibt pump implant .

we are presenting a case of a pregnant woman with a large vesical calculus which was diagnosed early in pregnancy . early diagnosis can help in preventing morbidity of pyelonephritis , recurrent urinary tract infections and obstructed labor . a high index of suspicion for renal calculus is needed in pregnant women when they present with recurrent urinary symptoms and infection . a 23-year - old primigravida at 13 weeks of gestation presented to the antenatal clinic of the urban health center with urgency , dysuria and increased frequency of urine . on examination a mobile hard mass a large bony mass was felt and a probable diagnosis of bladder stone complicating pregnancy was made . she was referred to the tertiary hospital where an ultrasound showed mild bilateral hydroureteronephrosis and a large vesical calculus measuring 8.3 cm causing dense acoustic shadowing obscuring the bladder base [ figure 1 ] . ultrasound also confirmed the presence of a single live intra uterine fetus corresponding to the gestational age . ultrasound image of vesical calculus she was referred to the urologist and underwent open cystolithotomy under spinal anesthesia . intra operative cystoscopy revealed bladder trabeculation with stone sitting in the anterior wall of trabeculation . post operatively she had continuous bladder drainage for 2 weeks and was treated with appropriate antibiotics . she delivered normally a live - term baby girl of apgar 9 and 10 and birth weight 2.42 kg at 38 weeks of gestation and is on regular follow up and doing well . renal stone disease in pregnancy is very rare ; the incidence being one in 2000 - 3300 of pregnancies . congenital and acquired diverticula of the urinary bladder may be a reason for stasis and development of calculus . foreign bodies like wire , needles and intra uterine devices migrating to the urinary bladder can result in stone formation . vesical calculus can be asymptomatic or can present with supra pubic pain , dysuria , frequency , hesitancy , terminal hematuria and sometimes urinary retention . there can be recurrent episodes of urinary tract infections in women with vesical calculus in pregnancy . persisting pyelonephritis despite appropriate and adequate antibiotic therapy should prompt the search for obstruction and stone disease in pregnancy . our patient presented with urinary symptoms and a clinical diagnosis of vesical calculus which was later confirmed by an ultrasonogram . vesical calculus can cause obstruction of labor and formation of vesicocutaneous fistula following bladder stone removal . although transvesical litholapexy is the preferred choice of treatment , open supra pubic cystotomy is indicated for removal of large vesical calculus . in case of large vesical calculus the potential danger of complications in labor outweigh the risk of intervention during pregnancy . a high index of suspicion for vesical calculus is needed in women who present with urinary symptoms and recurrent urinary tract infections in pregnancy . an early diagnosis of a large vesical calculus in pregnancy and its appropriate surgical management can help avoid serious complications . an important factor which favored this woman 's outcome of pregnancy was the well - developed link of the urban heath center with the tertiary unit and the continuity of care practiced in the urban health center . thus it is not only important to suspect and diagnose an aberration from the normal but also to develop good links with local tertiary units and co - ordinate care for the individual patient .

vesical calculus - complicating pregnancy is rare . this is a case report of a large vesical calculus - complicating pregnancy . the early diagnosis and appropriate surgical management of the large vesical calculus prevented complications like recurrent urinary tract infections and obstructed labor . it enabled the mother to have an uneventful vaginal delivery .

a 68-year - old man ( height 166 cm , weight 72 kg , bmi 26.1 ) underwent nephroureterectomy with cuff excision due to a left ureter carcinoma . the patient had a history of surgery for colon cancer and was on antihypertensive medication . preoperative physical examination and laboratory investigations showed normal findings . on arrival in the operating room , his baseline blood pressure was 150/88 mmhg and his heart rate was 68 beats / minute . the patient 's blood pressure and heart rate were maintained within a range of 20 percent of baseline during the procedure . findings from intraoperative arterial blood gas analyses performed every 2 hours were within the normal ranges . the estimated blood loss was 500 ml , with no need for a blood transfusion . he was then transferred to the postanaesthetic care unit ( pacu ) . in the pacu , intravenous ketolorac was administered for pain relief , and the patient was transferred to the general ward . blood chemistry tests that were performed 90 minutes after completion of the surgery revealed ast , 90 iu / l , and alt , 54 iu / l . the urine volume was maintained at 1.5 - 3.0 ml / kg / h with no darkening of color . serum levels of ast and alt increased markedly to 386 iu / l and 158 iu / l , respectively , on the first postoperative day ( day 1 ) and to 544 since the viral markers were all negative , ischemic liver injury or toxic hepatitis was presumed to have occurred during surgery . however , no hypotensive or hypoxic episodes occurred during surgery . the back pain did not respond to non - steroidal anti - inflammatory drugs , despite the fact that no tenderness or erythema was present in the thigh or back . rhabdomyolysis was suspected because the patient had intraoperative risk factors , such as being overweight , having a prolonged operation , and unusual positioning without repositioning . at that time , blood chemistry tests revealed creatine kinase ( ck ) , 26,276 iu / l ; serum myoglobin , 3,000 ng / ml ( normal , 5 - 60 ng / ml ) ; lactate dehydrogenase ( ld ) , 1,002 iu / l and albumin , 3.0 g / dl . no abnormalities were noted on alkaline phosphatase , prothrombin time , or -gt . under a diagnosis of rhabdomyolysis , the patient was sufficiently hydrated to maintain high urine output , but alkalinization was not performed because his urine ph was 7.0 . two weeks later , blood chemistry tests showed ck , 261 iu / l ; ld , 239 iu / l ; ast , 30 iu / l ; and alt , 42 rhabdomyolysis is caused by leakage of muscle enzymes , myoglobin and intracellular constituents from the dissolution of striated muscle fibers . although rhabdomyolysis can be diagnosed based on a 5-fold or more increase in the serum ck level , recognition of this disease entity is difficult when there are no remarkable clinical symptoms and signs such as gluteal and back pain , reddish brown urine , or oliguria . it is inferred that rhabdomyolysis may have resulted from a direct compressive injury to the striated muscles because the surgery was performed on an overweight patient for 9 hours in the lateral decubitus position without repositioning . even when the ck level increased to 26,276 iu / l and the myoglobin level increased to 3,000 ng / ml , the patient 's urine volume was well maintained and its color did not change . the classic finding of reddish - brown urine found in rhabdomyolysis was not seen until serum myoglobin reached 100 mg / dl . akmal and massry reported that reversible hepatic dysfunction appears to occur in 25% of patients with rhabdomyolysis . report the case of an obese patient who developed rhabdomyolysis with arf and hepatic dysfunction . have demonstrated that proteolytic activity increases in patients with acute renal failure and traumatic rhabdomyolysis . hepatic dysfunction in rhabdomyolysis may be attributed to liver damage caused by proteases released from injured muscle . in our patient , in addition , no events , such as hypotension or hypoxia during surgery , were present . serum alt level reached a peak on day 2 , and then decreased together with the serum ck level . transient liver dysfunction in our patient is believed to have been a concomitant phenomenon related to rhabdomyolysis . in conclusion , we present a case of rhabdomyolysis with liver dysfunction that occurred after a prolonged urologic procedure in the lateral decubitus position . we would like to emphasize that sufficient attention should be given , during the perioperative period , to patients with a higher risk of rhabdomyolysis .

the classic signs and symptoms of rhabdomyolysis are non - specific and not present in all cases , and mild cases might go unrecognized . we present a case of rhabdomyolysis recognized in a 68-year - old man after elevation of liver enzymes following prolonged urologic surgery . the patient 's postoperative course was concerned with elevated serum aspartate aminotransferase and alanine aminotransferase without any clinical manifestations on the first postoperative day . after examining the serum creatine kinase and myoglobin levels , the patient was diagnosed with rhabdomyolysis . after 16 days , he was discharged with fully recovered liver enzymes and creatine kinase . we suggest that sufficient perioperative attentions should be given to patients at a higher risk of rhabdomyolysis .

lupus eythematosus - lichen planus ( le - lp ) overlap syndrome is an uncommon disorder with about 50 cases being reported in literature . there is an overlap of clinical , histopathological , and immunofluoresecnce findings , which help in distinguishing the disorder . most of the cases reported were le - lp overlap and the association of subacute cutaneous lupus erythematosus ( scle ) with lp has been reported only once in literature . a 35-year - old female presented with multiple scaly pigmented plaques on the trunk and limbs of 2 years duration . the lesions initially started as scaly annular plaques on the lower limbs , which became ulcerated , crusted and healed with pigmentation and gradually spread to involve other parts of the body . there was no history of viral infection or drug intake prior to onset of lesions . on examination , few erosive and crusted plaques were seen in the forearms and lower legs [ figure 1 ] . erosive and crusted plaques in the forearms oral cavity showing palatal erosions with hyperpigmentation her complete blood count , liver and renal function tests were normal . few civatte bodies were seen in the epidermis and superficial dermis [ figure 3 ] . the superficial dermis was edematous and showed mucin deposits with perivascular lymhocytic infiltrate and melanophages . direct immunofluorescence showed linear deposits of fibrinogen at the basement membrane zone . with the history and investigations , a diagnosis of scle - lp overlap syndrome hyperkeratosis with basal cell degeneration , colloid bodies , and pigmentary incontinence ( h and e , 10 ) scle is a nonscarring nonatrophy - producing photosensitive dermatoses characterized by papulosquamous or annular lesions . scle occurs in genetically predisposed individuals , most often in patients with hla - b8 and hla - dr3 . the reaction is believed to be related to ultraviolet ( uv ) light modulation of autoantigens , epidermal cytokines and adhesion molecules , with resultant keratinocyte apoptosis . it can also be induced by drugs such as hydrochlorthiazide and terbinafine and complement ( c2 ) deficiency . the association of le with lp has been documented , most of them being systemic lupus erythematosus . a genetic , autoimmune , viral , or drug etiology has been postulated as a cause of le or lp , and it has also been considered as a spectrum of same disease caused by a viral infection in a genetically predisposed host the association of scle with lp is very rare with only a single case has been reported by grabbe et al.2they concluded that clinically and historically , scle - like lesions appeared to progress into lp - like lesions , supporting the concept of a common autoimmune pathophysiology in these disorders . in our case , the lesions started as scaly annular plaques and ultimately healed with pigmentation similar to the case reported by grabbe et al . the presence of pigmented plaques , oral pigmentation and positive immunofluorescence findings were suggestive of lichen planus , whereas the presence of scaly annular plaques , palatal erosions , histopathology findings and ana and anti - ro positivity were suggestive of scle . inaloz et al.1reported a case of le - lp overlap with positive immunofluorescence findings for lp and negative for le as seen in our case .

a 35-year - old female presented with scaly annular and pigmented lesions all over the body of 2 years duration . her clinical features , histopathology , immunofluorescence findings and positive ana and anti - ro antibodies were suggestive of subacute cutaneous lupus erythematosus - lichen planus . we report this case because of its clinical rarity .

a thirty - year - old male was undergoing evaluation of chronic left flank pain and intermittent fever . his ultrasonography his blood examination had revealed leukocytosis and urine examination had showed 1015 pus cells / hpf . he underwent dynamic renal scintigraphy for evaluation of hydronephrosis of left kidney detected on usg . the scintigraphy was performed with intravenous ( iv ) injection of 375 mbq of tc-99 m diethylenetriaminepentaacetic acid and f-0 injection of furosemide in the dose of 1 mg / kg . the dynamic imaging during perfusion phase revealed transit of bolus through aorta [ red arrow , figure 1 ] , vascular blush to right kidney ( blue arrow ) , and an intense vascular blush in region of left renal fossa at level of lower pole of kidney ( black arrow ) . the uptake phase of renal scintigraphy revealed the absence of cortical uptake in left kidney and normal tracer uptake of right kidney [ figure 2 ] . to evaluate cause of vascular blush in left renal fossa , further evaluation with contrast - enhanced computed tomography ( cect ) was performed . the scintigraphy was performed with intravenous injection of 375 mbq of tc-99 m diethylenetriaminepentaacetic acid and f-0 injection of furosemide in the dose of 1 mg / kg . the dynamic imaging during perfusion phase revealed transit of bolus through aorta ( red arrow , figure 1 ) , vascular blush to right kidney ( blue arrow ) and an intense vascular blush in region of left renal fossa at level of lower pole of kidney ( black arrow ) the uptake phase of renal scintigraphy revealed the absence of cortical uptake in left kidney and normal tracer uptake of right kidney cect was performed on a 64 slice scanner ( philips brilliance ) before and after iv injection of noniodinated contrast medium ( iohexol 350 ) . figure 3a precontrast coronal section shows large left renal staghorn calculus with enlarged , hydronephrotic kidney . figure 3c reveals arterial phase of cect , depicting enhancement in the lower pole of the left kidney . figure 3b shows axial section images of postcontrast delayed phase at the level of lower pole . persistent parenchymal enhancement , perinephric fat stranding , and thickening of gerota 's fascia was seen ( arrows ) . the cect scan findings suggested a diagnosis of acute pyelonephritis ( apn ) involving lower pole of left kidney . figure 3d sagittal cect section shows that the perinephric fat stranding is extending inferoposterior to the lower pole of left kidney . ( a ) precontrast coronal section shows large left renal staghorn calculus with enlarged , hydronephrotic kidney . ( c ) reveals arterial phase of contrast - enhanced computed tomography , depicting arterial enhancement in the lower pole cortex of left kidney . ( b ) axial section of postcontrast delayed phase at the level of lower pole cortex . persistent parenchymal enhancement , perinephric fat stranding , and thickening of gerota 's fascia was seen ( arrows ) . the contrast - enhanced computed tomography scan findings suggested a diagnosis of acute pyelonephritis involving lower pole of left kidney . ( d ) sagittal contrast - enhanced computed tomography section shows that the perinephric fat stranding is extending inferoposterior to lower pole of left kidney dynamic renal scintigraphy is an established procedure for the diagnostic work - up of upper urinary tract dilatation . it contributes to the management of patients with hydronephrosis by assessing both urinary flow and renal function . while performing dynamic renal scintigraphy , radiotracer temporally traverses and recirculates through abdominal aorta , visceral ( spleen and liver ) , and soft tissues before being extracted by renal parenchyma . therefore , any abnormality involving aorta , visceral organs , and soft tissues within the field of view is highlighted for a brief period and can be brought to notice of referring clinician for further investigations . a careful review of perfusion phase of dynamic renal scintigraphy can help in diagnosing various renal and extrarenal physiological and pathological processes in the field of view . the various causes of vascular blush in renal scintigraphy described in the literature are renal malignancies , other hypervascular tumors , metastatic bone lesions , oncocytoma , uterine vascularity , fetal uptake in gravid uterus , aortic aneurysm , liver abscess , infrarenal hematoma , and injection abscess .

a thirty - year - old male underwent tc-99 m diethylenetriaminepentaacetic acid renal scintigraphy for evaluation of gross hydronephrosis of left kidney . the perfusion phase revealed an intense vascular blush in left renal fossa . the uptake phase of scintigraphy revealed the absence of tracer uptake in left kidney . contrast - enhanced computed tomography ( cect ) was performed for evaluating the cause of vascular blush . cect demonstrated features suggestive of acute pyelonephritis ( apn ) involving lower pole of the hydronephrotic left kidney , corresponding to the site of vascular blush seen on renal scintigraphy . the postnephrectomy specimen confirmed the diagnosis of apn suggested on cect .

clinical view of the exophytic lesion with a smooth surface in the buccal region . the annual incidence and mortality rates vary considerably between different races , genders , and age groups . in the united states this is 7.7 per 100,000 . as with so many carcinomas , the risk of intra - oral cancer raises with increasing age especially for males.1 - 4 individuals with oral scc are almost aware of an alteration in an oral cancer site for 4 - 8 months before seeking professional help . there is minimal pain during the early growth phase and this may explain the delay in seeking professional care . if the health care professional does not have a high index of suspicion , an additional several weeks or months may elapse before a biopsy is performed . oral scc has various clinical presentations such as exophytic , endophytic , leukoplakic and erythroplakic , all showing visible changes in the surface.1 , 5 in the present paper , we report an unusual case of exophytic oral scc with a smooth surface . a 75-year - old female patient was admitted to the department of oral medicine , in mashhad university of medical sciences , in october 2005 , with the chief complaint of a painful mass in the left buccal mucosa which was first noticed by the patient two weeks earlier with a gradual increase in size . intra - oral examination revealed a normal - colored firm exophytic lesion with a smooth surface on the left buccal mucosa adjacent to premolar - molar region and a size of approximately 2.5 1.5 cm . small yellow papules were seen on the surface and the superior border of the lesion ( figure 1 ) . medical history indicated type ii diabetes mellitus . considering the smooth surface of the lesion and its location , salivary gland tumors and mesenchymal tumors the excised specimen was submitted for histopathological examination , which revealed a malignant neoplastic proliferation of stratified squamous epithelial cells as sheets or islands of cells with keratin pearl formation , invading to the connective tissue ( figure 2 ) . dyskeratosis , cellular pleomorphism and mitotic activity was observed in tumoral cells ( figure 3 ) . the patient was referred to the oncology department where an intra - oral excisional biopsy was performed . the patient , however , died three days after surgery because of poor management of diabetes . this is an unusual case of oral scc in a 75-year - old female patient presenting an exophytic lesion with a smooth and intact surface . invasion of malignant epithelial cells into the connective tissue with keratin pearl formation ( h&e staining ; 40 ) . invasion of malignant squamous islands into the connective tissue ( h&e staining ; 100 ) . considering the pathogenesis of scc , all presentations in this case are associated with changes in the surface as expected for epithelial lesions ; since , on rare occasions , squamous cell carcinoma may commence at a small location on the surface , burrow and undermine the subepithelial tissue in such a manner that the lesion appears mostly as a smooth surfaced exoplytic lesion . the diagnosis of the presented case emphasizes that even in smooth - surfaced rapid - growing oral lesions , scc should be considered in the differential diagnosis and this needs a careful examination and management by both medical and dental practitioners .

squamous cell carcinoma ( scc ) is the most com - mon malignant tumor of the oral cavity and one of the ten most common causes of death . it arises from dysplastic oral squamous epithelium . considering the pathogenesis of scc , a smooth and intact surface in this lesion is not a usual finding . in this paper , we report an extremely rare case of oral scc in buccal mucosa presenting as an exo - phytic lesion with smooth and intact surface , very unusual for oral scc .

in 1935 jaffe described a new bone lesion , which he named osteoid osteoma . this entity arose in spongy bone and was less than 2 cm in diameter . jaffe was the first to identify and describe osteoid osteoma of the spine . in 1956 osteoid osteoma and osteoblastoma are bone - producing lesions that are frequently localized in long bones and posterior elements of the vertebra . osteoblastoma and osteoid osteoma are histologically similar in many regards . the basic microscopic pattern in osteoblastoma and osteoid osteoma is bone - forming tumor containing numerous osteoblasts producing osteoid and woven bone . in comparison to osteoid osteoma , osteoblastoma has more aggressive characteristics and often forms extraskeletal bone in the soft tissue . there have been several large series reporting on osteoid osteoma and osteoblastoma of the spine [ 1 , 12 , 13 , 16 , 17 ] . however , the reports included information on patients who did not have surgery [ 12 , 16 , 17 ] or numerous patients who were treated before 1960 [ 1 , 13 ] . as several imaging techniques have been developed during the last 20 years , the diagnosis in patients can be made earlier in the clinical course and more exact excision possible . after the first descriptions of osteoid osteoma and osteoblastoma by jaffe [ 10 , 11 ] and lichtenstein , these rather vascular , osteoid , and bone - forming tumors caught the attention of different authors . jackson et al . , published a review of 860 cases of osteoid osteoma and 184 cases of osteoblastoma collected from the english literature . the spine has been the location of 10% of all osteoid osteoma [ 1 , 4 , 9 , 23 ] and 36% of osteoblastoma . these spine tumors arose in the posterior elements . histologically , osteoid osteoma is small , benign , and self - limited , containing osteoblasts that produce osteoid and woven bone . both of these tumor types tend to occur in the thoracic and lumbar spine [ 3 , 21 ] . osteoblastoma occur predominantly in patients younger than 20 years of age [ 5 , 10 , 15 ] . clinically , the pain of osteoblastoma is not as severe at night , nor is it relieved by aspirin , as is pain of osteoid osteoma . in contrast to osteoid osteoma , plain x - ray films are usually sufficient to diagnose osteoblastomas , although ct scans and mr images are of great value for both diagnostic and therapeutic considerations in the spine . preoperative ct scanning has been shown to be very helpful for precisely defining the location of the tumor and extent of osseous involvement . appearance of these tumors on mr images is generally non - specific , but this imaging modality is surely the best to reveal the effects of tumor on the spinal canal and cord , as well as the extensive intra- and extra - osseous reactive changes and the possible infiltration of adjacent soft tissues . the nidus usually has a low - to - intermediate signal intensity on t1-weighted and a low - to - high signal intensity on t2-weighted magnetic resonance ( mr ) images [ 2 , 6 , 18 ] . technetium bone scanning is now accepted as the most accurate means for localizing the tumor . this modality demonstrates an intense focal accumulation of the bone - seeking agent [ 14 , 19 ] . surgery is the most common treatment for this disease ( osteoid osteoma ) and the prognosis after total resection is favorable . the recommended treatment for osteoid osteoma causing disabling pain and spinal deformity is excision . osteoid osteomas are most frequently treated because of the persistent pain associated with them , whereas osteoblastomas are treated both for pain and the increase in size , which leads to destruction of bone . recently , percutaneous radiofrequency thermal ablation [ 1 , 7 , 20 , 22 , 24 ] and laser photocoagulation have been promoted as a minimally invasive treatment for osteoid osteoma located in the spine and extremities . these techniques are both based on thermal necrosis obtained by local temperatures ranging from 50 to 90c . in addition , heating the tip of needle up to 90c for 46 min in a nidus located in the posterior structures of the spine inevitably risks thermal damage to the neural structures . the use of these techniques for spinal osteoid osteoma has been reported previously [ 4 , 6 , 7 , 20 , 22 , 24 , 25 ] with conflicting results . recently reported two patients treated with radiofrequency ablation , of which one had incomplete resection necessitating reoperation . vanderschueren et al . reported recurrent symptoms in two of four cases with spinal osteoid osteoma in a series of 97 patients treated with thermocoagulation . in conclusion , we believe that with the development of modern imaging and treatment methods , exact surgical planning has become possible . as several imaging techniques have been developed during the last 20 years , the diagnosis in patients can be made earlier in the clinical course and more exact excision possible .

osteoid osteoma and osteoblastoma are rare primary bone tumors that usually do not arise in the spine . histologically , osteoid osteoma and osteoblastoma are similar , containing osteoblasts that produce osteoid and woven bone . osteoblastoma , however , is larger , tends to be more aggressive , and can undergo malignant transformation , whereas osteoid osteoma is small , benign , and self - limited . with the help of modern imaging modalities that aid in diagnosis and surgical planning , a complete removal and cure may be achieved for most of these rare tumors . we document a brief review of the literature .

mechanical prosthetic valve dysfunction may result from thrombosis or pannus formation and has a spectrum of clinical presentations that range from nonspecific symptoms to acute hemodynamic compromise leading to shock and death . although intermittent dysfunction of a mechanical prosthesis is usually attributed to thrombus formation , pannus formation rarely has been reported to cause intermittent , cyclic dysfunction of a mechanical aortic prosthesis.1 - 6 ) we describe the unusual case of intermittent , non cyclic severe mechanical aortic valve regurgitation due to pannus formation with thrombus . a 68-year - old man who had been performed aortic valve replacement with a bileaflet mechanical tekna valve ( 23 mm , baxter health care , santa ana , ca , usa ) 12 years ago presented to the emergency department with an exertional chest pain and short of breath for four days . on presentation , he was clinically stable with blood pressure of 120/70 mmhg , heart rate of 97 bpm and 2/6 systolic murmur , but no diastolic murmur . chest x - ray showed mild cardiomegaly and tortuous ascending aorta . troponin i was normal at 0.01 ng / ml ( normal range 0.00 - 0.05 ng / ml ) and international normalized ratio was suboptimal at 1.04 sec . transthoracic echocardiogram and transesophageal echocardiogram demonstrated intermittent non cyclic severe acute aortic valve regurgitation with normal systolic excursion of the occluding disk , a mean transprosthetic gradient of 14 - 23 mmhg ( fig . a small less mobile echogenic mass was seen in the outflow tract immediately above the prosthesis consistent with pannus formation on which possible thrombus superimposed ( fig . doppler echo showed increased left ventricular filling pressure ( e / e ' 19 ) and steep slope of aortic regurgitation ( pressure half time 120 msec ) . aortogram showed also non cyclic , intermittent incomplete closing of prosthesis with severe aortic regurgitation ( supplementary movie 1 ) . after short term intravenous heparin infusion , he underwent urgent surgical treatment . at surgery , inspection and visualization of the structure with a cardiac optical fiber device confirmed echocardiographic data showing a focal pannus formation above the aortic prosthesis . the prosthetic valve leaflets were not involved and the disk motion appeared unrestricted ( fig . histologic examination revealed a structure of fibroconnective tissue consistent with pannus formation and superimposed thrombus . the patient had an uncomplicated postoperative recovery and was discharged on his 7th postoperative day . after 2 years with meticulous anticoagulation therapy , the patient was stable and follow - up echocardiogram showed normal aortic prosthetic valve function with a mean transprosthetic gradient of 10 mmhg . prosthetic valve dysfunction as a result of pannus formation due to fibrous tissue ingrowth is an infrequent and usually produces a stenosis of the prosthesis due to obstruction of the left ventricular outflow tract or restriction on the movement of the opening of the discs of the prosthetic valve . unlike thrombus formation , pannus is not related to inadequate anticoagulation,7 ) and more common with prostheses in the aortic than mitral position and is often observed years after implantation of the prosthesis.8 ) a single tilting - disc prosthesis seems to be a significant risk factor for pannus formation and the need for reoperation.9 ) differentiating pannus formation from thrombus is often difficult ; however , the latter tends to be less video - dense and larger in size , and has increased mobility.7)8 ) there have been a few case reports of pannus causing intermittent , cyclic or non cyclic severe aortic regurgitation due to intermittent interference of disk closure.1 - 6 ) our case differs from previous reports in that the episodes of intermittent severe aortic regurgitation were non cyclic with random , short episodes of severe regurgitation lasting only several cardiac cycles , associated with brief angina pain and short of breath . incomplete closure of the occluding disk was identified on fluoroscopy and echocardiography , in the absence of restricted systolic excursion . we suspect that pannus ingrowth on the outflow tract aspect of the valve extended slightly beyond the inner aspect of the prosthesis ring , such that the pannus tissue interfered with disk closure and resulted in severe aortic regurgitation , without altering systolic excursion of the disk . marginal , especially around hinge contact of the occluding disk and pannus ingrowth and coexistence of thrombosis , in aorto - ventricular pressure differences might account for the intermittent nature of the dysfunction . we report an unusual case of intermittent , non cyclic mechanical aortic prosthesis dysfunction due to pannus formation with thrombus , that presented with intermittent severe aortic regurgitation . aortogram showed also non cyclic , intermittent incomplete closing of prosthesis with severe aortic regurgitation .

mechanical aortic prosthesis dysfunction can result from thrombosis or pannus formation . we describe an unusual case of intermittent , non cyclic mechanical aortic prosthesis dysfunction due to pannus formation with thrombus in the absence of systolic restriction of disk excursion , that presented with intermittent severe aortic regurgitation .

the muckle wells syndrome ( mws ) is assigned to the family of periodic fever syndromes and belongs to the sub - group of the cryopyrin - associated periodic syndromes ( caps ) . it causes recurrent fever attacks , urticarial rash , myalgia , arthralgia , headache , sensorineural deafness and severe fatigue syndrome . mws is an autosomal - dominant - inherited disease , which is caused by a mutation of the cias1 gene on chromosome 1q44 , leading to a permanent and excessive activation of the interleukin ( il)-1 receptor . so far , there have been > 50 heterozygous mutations reported leading to different phenotypes , i.e. different clinical manifestations of mws . the exact incidence of the caps is unknown . in the literature , there are currently 100 case reports . the prognosis of mws is determined by the development of systemic aa amyloidosis , especially renal amyloidosis with severe renal impairment up to end - stage renal disease . but , only little is known about the treatment of patients with mws and renal impairment . over decades there are few case reports about successful treatment with anakinra , an il-1 receptor antagonist , before and after renal transplantation [ 6 , 7 ] . in 2009 canakinumab , a human anti - il-1-monoclonal antibody was approved by the u.s . food and drug administration ( fda ) and european medicines agency ( ema ) for the treatment of mws . a 32-year - old woman with mws and systemic amyloidosis underwent renal transplantation in 2010 after 7 years of being on haemodialysis . in detail , the patient developed all of the typical symptoms of mws , starting in her earliest childhood . the diagnosis of mws was made by the detection of a mutation in the cias1 gene on chromosome 1q44 exon 3 with the variant r260w when the patient was 27 years old . her grandmother and mother died of sudden cardiac death due to systemic amyloidosis and end - stage renal disease . the brother , the aunt , three uncles and two cousins are also affected by mws , three of them with renal impairment as well . furthermore , a cardiac amyloidosis was found on mri as well as a biopsy - proven aa amyloidosis in the adipose tissue and thyroid gland . due to rapid progression of renal amyloidosis , the therapy with anakinra at a dosage of 100 mg subcutaneously every other day was started and showed a very good clinical and biochemical response . the important inflammatory markers c - reactive protein and serum amyloid decreased within 2 weeks to normal levels . thereafter , the specific therapy was switched to canakinumab 150 mg subcutaneously once every 8 weeks because of better pharmacokinetic features and persistent suppression of il-1 with a sustained remarkable response of clinical symptoms and inflammatory markers . in february 2010 the initial immunosuppressive therapy consisted of cyclosporine a , mycophenolate mofetil and prednisolone . immediately after renal transplantation the creatinine decreased to 121 mol / l and the creatinine clearance increased to 48 ml / min at the time of discharge from our hospital 1 month after kidney transplantation . the therapy with canakinumab has been continued with the same dosage after renal transplantation with no flares of mws despite improved renal clearance . no severe bacterial or viral infections or any other adverse events have occurred up to now . fortunately , we did not observe any pharmacological interactions between canakinumab and the immunosuppressive therapy . one and half years after renal transplantation , the patient has an excellent graft function with a creatinine of 86 the anti - il-1-monoclonal antibody canakinumab has been recently introduced as a specific treatment option in patients with mws with normal and impaired renal function . reported about the successful treatment with canakinumab once every 8 weeks in patients with caps . little is known about the feasibility of renal transplantation in mws patients and its impact on mws activity under a specific treatment . as shown in our patient , we believe that under the immunomodulatory effect of canakinumab with direct and highly specific blockade of il-1 , there is no interference with the immunosuppressive therapy after renal transplantation . so far , the immunological challenge after renal transplantation and the triple immunosuppressive therapy had no negative impact on activity of mws in our patient . it can be postulated that canakinumab can be used with a very good clinical response in patient with normal renal function as well as in patients with renal replacement therapy , either haemodialysis or renal transplantation . in the future , the growing experience with canakinumab in the treatment of patients with mws will demonstrate whether it is possible to prevent under the therapy the development of aa amyloidosis as a severe and life - threatening complication of mws with canakinumab treatment . in the case of our patient , we have had no recurrence of aa amyloidosis in the kidney transplant at the present time . the limitation is that the observation period of one and a half years is very short . further evaluations will be performed in order to confirm the positive effect of canakinumab . in our opinion , treatment with the anti - il-1-monoclonal antibody canakinumab in patients with mws and renal replacement therapy is feasible , safe and without severe side effects .

we report the first case of a 32-year - old woman with muckle wells syndrome and biopsy - proven systemic aa amyloidosis and end - stage renal disease . she was treated with canakinumab 150 mg subcutaneously every 8 weeks and underwent renal transplantation . fourteen months after renal transplanation , the patient had no flares of muckle wells syndrome and no evidence of amyloidosis in the renal transplant under an excellent graft function and therapy with canakinumab .

the correlation between the activity of neurons in the sensory cortex has long been the object of interest , as its measurement allows inferences about underlying anatomical connectivity . a peak in the cross - correlogram between the firing rates of two neurons can indicate a monosynaptic connection or common input . this technique , for instance , is in wide use to study functional connectivity within the primary visual cortex ( e.g. , to investigate horizontal connections or connections between simple and complex cells ) . knowing the correlation between neurons can also serve to constrain the possible schemes employed by the cortex to code and decode sensory stimuli [ 6 - 8 ] . intuitively , it may not seem advantageous to maintain millions of neurons if their activity is highly correlated . the impact of correlations on decoding accuracy , however , depends on the decoding scheme . for a decoder that performs simple averages , the appropriate methods for measuring correlation depend on what is being studied . under truly stationary conditions ( i.e. , in the absence of a stimulus and of global modulations of responsiveness ) a stimulus introduces a trivial correlation among neurons , which depends on their stimulus selectivity . stimulus correlation is usually removed by subtracting the shuffle predictor , which is the cross - correlogram of trial - averaged responses . what remains after subtraction is the noise correlation and is typically the quantity of interest . correlations due to changes in the global state activity , in turn , are harder to remove because such changes are typically not repeatable and are hard to identify . changes in global activity are therefore usually ignored , an approach fraught with peril because their impact on the measured correlation can be substantial . using these methods , most studies to date the exact value of the correlation depends heavily on a variety of factors , including the time scale over which it is computed , the firing rates of the neurons , the distance and difference in tuning between the neurons , the strength of the sensory stimulus , the past history of stimulation , and the behavioural condition in terms of arousal and attention . nonetheless , these and other studies have found that in most conditions the correlations are positive and substantial , with average values in the range of 10 - 40% . surprisingly , two recent studies reported that average correlations in the sensory cortex can be 10 times lower . ecker and colleagues measured average correlations in the primary visual cortex of awake macaques and found the correlation to be in the order of 1% . this is particularly surprising as the authors binned the data in long windows ( 500 ms ) , and such long windows , if anything , bias correlation toward higher values . extremely low correlations - again , in the range of 1% - were seen during activated states , which are devoid of up - down fluctuations . a remarkable aspect of these two new studies is that correlations between pairs of neurons were about as likely to be positive as to be negative ( thereby yielding an average of close to zero ) . this is in sharp contrast to most other studies [ 10,13 - 15,17 ] , which found predominantly positive correlations ( and thus positive averages ) . given that cortical neurons are likely to receive substantial common input , extremely low average correlations need explanation . this explanation may come from the theory of balanced networks , which was developed to explain how an interconnected network can inhabit regimes intermediate between complete silence and full - on epilepsy . a balanced network postulates connections ( typically random ) between and within two pools of excitatory and inhibitory cells . such tracking generates negative correlations in synaptic currents and these negative correlations cancel existing positive correlations , including those due to common input . thanks to this intuition , show that average correlations between neurons can be very small , not only in the sparsely connected networks studied previously , but also in densely connected networks with strong coupling and substantial common input . the authors show that , in such a network , the population - averaged correlation decreases as the inverse of the number of neurons . a network where correlations are extremely weak asynchronous state , but in neuroscience , synchrony usually refers only to time scales of milliseconds ( e.g. , ) . what are the key factors that explain these large differences in correlation relative to the previous studies ? these global modulations can have a large impact on measured correlations and are common not only under some forms of anaesthesia but also in awake animals [ 26 - 28 ] . these global modulations may perhaps have been lower in the animals in the study by ecker et al . . conversely , when the authors included data showing up - down fluctuations , the average correlation between neurons rose substantially . other factors that may contribute to the low correlations seen in the study of the visual cortex are the size and strength of the stimulus . ecker et al . used a high - contrast visual stimulus , and it is well established that such stimuli decrease neuronal correlation . it would be interesting to know to what extent the stimulus influenced the low values of correlation that were measured . very recently , a possible factor for the discrepancy in measured correlations was identified in the laminar structure of the cortex . smith and kohn measured spike count correlation between pairs of neurons at various depths in the primary visual cortex and found a striking dependence on cortical layer . correlation between neurons in the superficial or deep layers could be as high as in the previous studies ( in excess of 10% ) , but correlation between neurons in the intermediate layers was extremely low ( as low as 2% ) . knowledge of the layers in which the various studies have made recordings may explain the discrepancy between their measures of correlation . a future challenge will be to identify clearly the temporal and spatial scales of cortical correlation and to investigate their dependence on stimulus attributes such as contrast and on cognitive factors such as attention . more generally , the results of ecker et al . and renart et al . it may be more useful to develop generative models of neural responses which will include the effects of incoming stimuli , of common inputs , and of lateral connections . by predicting the spike trains of individual neurons accurately , these models would also predict their noise correlation ( or lack thereof ) and thereby provide insight into the underlying mechanisms .

the spike trains of nearby neurons in the sensory cortex are typically thought to be correlated due to mutual connections and common input . multiple studies have measured these correlations and found them to be substantial ( in the range of 10 - 40% ) . two recent papers , however , reported that average correlations can be an order of magnitude smaller . such low correlations could indicate an uncorrelated state for the cortex , where cortical neurons act independently even in the face of strong common input .

most common complications caused by a defunctionalized bladder are : pyocystitis , hemorrhage , pain , spasm , and bleeding . we report here a case of a carcinoma found in a defunctionalized bladder accompanied by selected literature . a 43-year - old non - smoker male was admitted to our department with a suspicion of a bladder tumor . the lesion was accidentally found in a nuclear magnetic resonance test performed due to a pain of the spinal column ( fig . one month before admission to the hospital the patient experienced an episode of pyocystitis that was accompanied by high fever . in childhood he underwent a bilateral ureterocutaneostomy due to a vesicoureteral reflux and was diagnosed with kidney failure . at the age of 28 he underwent a left nephrectomy due to a pyelonephritis and dialysis was started . cystoscopy performed before that operation revealed a decreased volume of the bladder , while no neoplasmatic changes were yet found . during the present hospitalization of the patient , microbiological examination of evacuated pus revealed the presence of e.coli with medium sensitivity to antibiotics . although the patient was offered a cystectomy , he refused the procedure due to a risk of a possible erectile dysfunction . therefore he was planned for a regular cystoscopic follow - up . check - up performed one month after turb procedure did not reveal any pathological lesion . further cystoscopies have been scheduled for the patient classified to a high - risk group ( based on guidelines of the european board of urology ) . aaronson et al . reported a case of squamous cell carcinoma ( sqcc ) in a bladder that was diverted due to persistent infections and incontinence . moloney et al . reported three cases of cancer in defunctionalized bladder : two male paraplegics were diagnosed with sqcc and a female with tcc . the authors claim that the variable duration of time from diversion to development of a tumor suggests that multiple factors apart from infections may be important in the pathogenesis of carcinoma . postulate that patients who require supravesical urinary diversion for benign bladder pathology should be offered primary cystectomy at the same time as the diversion unless there are comorbid factors that would significantly increase the risks of surgery . a rare case of a carcinoma in a remaining bladder diverted 25 years ago was described by asano et al . and is worth mentioning here since it occurred without the typical pyocystitis classified as a key feature suggesting the presence of a tumor . additionally , an extremely rare histopathological finding , an adenocarcinoma , in a leftover bladder was reported by djavan et al . . a retrospective study made by eigner et al . in which thirty adults after urinary diversion were observed ( mean time 3.5 years , max . similarly , no incidence of carcinoma was reported in a long - term follow - up of ninety - three patients after the supravesical diversion ( mean time 5 years ) conducted by singh et al . . the incidence of bladder problems in over half of these patients and upper urinary tract changes in over a third of them suggests however that indefinite follow - up is mandatory . they postulated that the same carcinogenic factors that give rise to tcc may also be responsible for sqcc on the condition that the epithelium had previously undergone squamous metaplasia . some authors remind that sexual function is better preserved with the bladder left in situ so this aspect should be taken into consideration before a decision of a cystectomy is made [ 1 , 10 ] . although cancer in the inactive bladder is a rare condition , it is advisable to follow - up these patients with a lifetime cystoscopy . the presence of recurrent intractable pyocystitis is a key feature to suggest unexpected cancer and this must be ruled out by all appropriate means , including extensive bladder biopsies . the decision about leaving the urinary bladder must be considered individually , based on a patient 's preferences , the will of maintaining sexual function and potential risk of developing neoplasm .

etiologic factors affecting bladder tumor have been well confirmed and it is widely recognized that carcinogenic substances in urine may play an important role in a pathogenesis . carcinoma developing in a defunctionalized bladder , although uncommon , does occur . we report a case of a transitional cell carcinoma ( tcc ) found in a remaining bladder of a male patient and a review of the most relevant literature .

hemangiopericytoma ( hpc ) is a rare mesenchymal tumor thought to originate from the pericytes , first identified by zimmermann.1 other investigators believe that hpcs arise from pluripotential perivascular cells,2 so they can occur anywhere capillaries are found . the most frequent locations are the extremities , pelvis , retroperitoneum , head , neck , and meninges . hpcs are usually deeply located within the muscle tissue , yet dermal and subcutaneous masses have also been described . hpcs are classified as benign , borderline , and malignant , depending on their clinical and histopathologic features . not all hpcs are typical , and some present overlapping features with other vascular and mesenchymal tumors . immunohistochemistry helps in excluding other differential diagnoses , but some cases with unusual findings can pose a challenge to general pathologists . a 45-year - old man presented with a palpable mass in the medial aspect of the right lower lid ( figure 1 ) . the patient had noticed a rapid growth of the lesion during the previous 2 months . the clinical examination revealed a firm mass , with smooth edges , without pain , tenderness , or pulsation . given that the mass was superficial , the surgical approach was an anterior right orbitotomy , with complete excision of the tumor mass . macroscopic examination of the specimen revealed an oval white rubbery mass measuring 11 9 7 mm . the interior of the lesion was solid , white , and homogeneous , without calcifications , cystic spaces , or hemorrhages . hematoxylin and eosin sections showed a tumor with a lobular pattern composed of highly cellular areas separated by dense fibrous tissue . under high magnification , the lobules were predominantly vascular , with the presence of small and large vessels lined by normal endothelium , in a pattern resembling the staghorn pattern typical of hpc . immunohistochemistry was positive for vascular markers such as factor viii , cd34 , and ulex ( figure 2 ) . d2 - 40 , epithelial membrane antigen , s-100 , and glial fibrillary acidic protein were all negative , excluding other differential diagnoses . ki-67 staining returned an average of six positive cells per field ( 40 ) . despite the high proliferative index , it is believed that hpcs arise from pluripotential perivascular mesenchymal cells , and can occur anywhere in the body . the orbit is a rare location for this particular tumor , and corresponds to 0.8% to 3% of all primary orbital tumors.3 hpc generally affects adults ( median age 45 years ) , with an equal sex distribution.46 it presents as a slow - growing , firm mass causing a painless proptosis , without tenderness or pulsation . intermittent swelling and ecchymosis can also be present as the tumor grows . on computerized tomography , it generally appears as a well - circumscribed mass , which dramatically enhances after injection of contrast material . on magnetic resonance images , it is isointense in t1 , while in t2 it can show slightly high intensity . there are no clinical or radiological signs pathognomonic of hpc , so the diagnosis is established after careful histopathological examination . often , the diagnosis of an hpc is made after excluding other tumors that can demonstrate hpc - like features . grossly , it presents as a solitary , well - circumscribed , often lobulated mass with a gray to red - brown surface . microscopically , the lesions are composed of ovoid to spindle - shaped cells separated by sinusoidal spaces , the so - called staghorn pattern . the case described herein presented significant areas of fibrosis , which is unusual for this particular entity . the tumor also had a high proliferative index , which is in keeping with the fast growth seen clinically . in approximately 12%45% of cases , orbital hpcs metastasize.7 the most common sites of metastases are lung , mediastinum , liver , and bone . unfortunately , the aggressive behavior of hpc is unpredictable , as it happens that apparently benign tumors eventually spread systemically . nevertheless , some criteria have been used to classify a tumor as malignant : large diameter ( > 5 cm ) , increased mitotic rate ( four or more figures per ten high - power fields ) , a high degree of cellularity , immature and pleomorphic neoplastic cells , foci of necrosis , and hemorrhage.8,9 the case described herein did not meet all criteria , and was thus considered benign . however , due to the unexpected and potentially aggressive behavior of the tumor , complete excision is the recommended treatment , with postoperative follow - up to detect early recurrences . a common problem is local recurrence after incomplete excision , with rates varying from 13% to 40% . in cases of local recurrence , adjuvant another topic that has raised some debate is the distinction between hpc and solitary fibrous tumor ( sft ) . sft was first recognized in the pleura , but soon after it was observed in other sites . both lesions display varying features of pericytic , fibroblastic , and/or myofibroblastic differentiation , and have similar immunohistochemical profiles . to settle this controversy , a proposal to consider hpc as a cellular variant of sft we emphasize the importance of thorough histopathological examination of hpcs so that differential diagnoses , which could benefit from specific therapies , can be excluded .

hemangiopericytoma is a rare vascular tumor that originates from pericytes . the orbit is a rare location for this particular tumor , and corresponds to 0.8% to 3% of all primary orbital tumors . we report a case of a hemangiopericytoma in a 45-year - old man that had an unusual presentation , as a rapidly growing mass in the anterior right inferior orbit . given that there are no clinical or radiological signs pathognomonic of this tumor , a careful histopathological examination is necessary to confirm the diagnosis . in our case , it presented also with unusual histopathological findings . the clinical features , radiological findings , differential diagnosis and treatment of this challenging entity are reviewed in this case report .

an uncommon condition known as pyometra occurs when the natural drainage of the uterine cavity is comprised and pus accumulates within the cavity . we report a patient that was admitted to our hospital for diffuse peritonitis caused by spontaneously perforated pyometra who was successfully treated by surgery . a 63-year - old iranian woman was admitted to our hospital in december 2005 with the chief complaint of abdominal pain , vomiting , and fever that had developed one day earlier . she was a diabetic patient and she had no history of sexually transmitted disease . she had 3 pregnancies and all of her deliveries were normal vaginal deliveries . on physical examination , she looked acutely ill : her body temperature was 38.1c , pulse rate was 126 beats / min , and blood pressure was 140/70 mmhg . the results of laboratory studies on admission were as follows : white blood cell count : 18700/ml ; red blood cell count : 3.4 106 /ml ; hemoglobin : 10 g / dl ; albumin : 2.4 mg / dl . ultrasonography demonstrated a large amount of ascites in morison pouch . computed tomography ( ct ) with an intravenous injection of contrast material visualized a calcified uterine body and fluid and ascites without free air . after obtaining adequate informed consent , laparotomy was performed on the basis of a diagnosis of perforation of the gastrointestinal tract but revealed a perforated pyometra . at laparotomy , about 900 ml of pus was found in the peritoneal cavity . there were no abnormal findings in the alimentary tract , liver , or gallbladder . pyometra is common in postmenopausal women , and more than 50% of all patients of nonruptured pyometra are asymptomatic . the incidence of pyometra becomes much higher with age and decline in activity ; incontinence is also a significant risk factor . postmenopausal bleeding , vaginal discharge , uterine enlargement , and cramping pain are said to be the classic symptoms of pyometra , only bleeding and discharge were commonly reported in these cases but this patient had no vaginal bleeding or discharge . once ruptured , the symptoms become severe and acute abdomen often develops ; it is therefore important to make a differential diagnosis from other causes of acute surgical abdomens . in conclusion , the diagnosis of spontaneous perforation of pyometra is rarely made preoperatively and the possibility of a perforated pyometra should therefore be considered when elderly women suffer from acute abdominal pain . their management is often difficult , and hysterectomy and bilateral salpingo - oophorectomy may be the best choice of procedure in these patients .

spontaneous perforation of the uterus is rare , its incidence being about 0.01% 0.05% . we report a rare case of diffuse peritonitis caused by spontaneously perforated pyometra . a 63-year - old woman with severe abdominal pain was admitted to our hospital . laparotomy was performed because of the suspicion of gastrointestinal perforation with generalized peritonitis . at laparotomy , about 900 ml of pus was found in the peritoneal cavity . there were no abnormal findings in the alimentary tract , liver , or gallbladder . a total abdominal hysterectomy with bilateral salpingo - oophorectomy was performed . pathological investigation of the surgical specimen revealed endometritis and myometritis of the uterus ; but there was no evidence of malignancy , and the cervical canal was patent . although spontaneously perforated pyometra is rare , a perforated pyometra should therefore also be considered when elderly women present with acute abdominal pain .

hemospermia is defined as the macroscopic presence of blood in semen and is often considered idiopathic . the number of cases where hemospermia is considered idiopathic is decreasing due to new diagnostic methods . a 49-year - old man presented to his general practitioner with a history of 4 episodes of monosymptomatic hemospermia in a period of 2 months . on clinical examination , the general practitioner found a palpable tumor at the upper pole of the right testis . microscopy showed a 9-mm pt2 seminoma limited to the testis with vascular invasion but no invasion of the tunica albuginea , epididymis or funiculus . tumor cells stained positive for plap , d240 and cd117 , and stained negative for hcg , afp and cd30 . 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 . current recommendations state that most patients presenting with hemospermia can be treated with minimal investigation and simple reassurance . a prospective study concluded that an investigation following the discovery of hemospermia should include a focused history , clinical examination including a digital rectal examination , serum psa , urine cultures and screening for sexually transmitted diseases . ultrasonography of the scrotum in that study was not performed as an investigational method for hemospermia . previously , kumar et al . suggested an algorithm for hemospermia investigation that did not include scrotal ultrasound for patients below 40 years of age with recurrent hemospermia . an algorithm for hemospermia by the american academy of family physicians recommends scrotal examination for patients below 40 years of age presenting with hemospermia to rule out infection and testicular cancer . the first case of testicular cancer presenting with hemospermia was reported in 2001 , and 2 other cases were reported in 2002 . in all 3 cases , scrotal examination and ultrasonography were performed . our patient had no history of prostatic disease , infections or systemic diseases which could explain the hemospermia , making the seminoma the most likely cause of the bleeding . testicular cancer is most common in young men and can , in many cases , be diagnosed by scrotal examination alone . as the procedure is noninvasive and therapeutic options exist for the above - mentioned causes , we recommend scrotal examination in patients presenting with hemospermia to exclude testicular tumors or infections of the scrotal contents . we further recommend ultrasound investigation of the scrotal contents if testicular tumors are found on clinical examination , as recommended in the current guidelines . hemospermia is often considered to be a symptom of little significance , and its etiology is poorly understood . this case of monosymptomatic hemospermia in a man with seminoma underscores the importance of scrotal examination and eventually ultrasonography in such patients . the discovery of seminoma in this case may only be incidental , but scrotal examination is easy , noninvasive and should be performed in all patients presenting with hemospermia .

hemospermia is often considered idiopathic . we report a case of a patient who presented with hemospermia . scrotal examination and ultrasonography found a testis tumor . this case underscores the importance of scrotal examination and eventually ultrasound in patients presenting with hemospermia .

although articular manifestations due to human immunodeficiency virus ( hiv ) infection are relatively uncommon , many rheumatic manifestations have been reported ranging from 4% to 71.3% in their prevalence . in a prospective study of 74 consecutive hiv - positive patients , arthralgias were found in ( 45% ) , arthritis in ( 10% ) , and reiter 's syndrome in ( 8% ) . the data on the occurrence of hiv arthropathy in the pediatric age group is sparse , the first case in india being reported in 2003 . the various causes of the hiv arthropathy include arthralgia , spondyloarthropathies ( spa ) , reactive arthritis ( reiter 's syndrome ) , psoriatic arthritis , hiv - associated arthritis , and septic arthritis . a 7-year - old girl presented with back pain 6 months ago that responded to some medication within 15 days . she was hospitalized for same , and ultrasound of left knee showed small loculated pocket of the fluid collection on medial and lateral aspect of left knee joint . she was detected to be hiv infected by elisa and was referred for further management . on examination , she was malnourished ( weight = 13 kg ) , had pallor , oral thrush , and left knee effusion with bilateral ankle joint swelling with arthralgia . she was started on antiretroviral therapy ( art ) consisting zidovudine ( azt ) , lamivudine ( 3tc ) , and efavirenz along with ibuprofen to which she responded in 2 months and was on regular follow - up . after 1 year of art , her cd4 count was 673 cells / cumm ( 45.9% ) with cd4:cd8 of 0.92 . usual signs and symptoms of pediatric hiv infection include recurrent and severe occurrences of bacterial , viral , and fungal infections such as pneumonia , candidiasis , disseminated herpes simplex , and tuberculosis . furthermore , observed are growth failure , cachexia , failure to attain typical milestones , behavioral abnormalities such as loss of concentration and memory . in studies conducted in a prospective design with patients examined by rheumatologists , a conclusively high prevalence ( 70% ) of articular involvement associated with hiv the pathophysiology of arthritis in hiv - infected patients is not yet completely understood , but a direct role of the hiv on the initiation of synovitis is suspected in some of them . arthralgia is a common manifestation of hiv infection , and it may occur at any stage of the hiv infection and frequently oligoarticular . spas have been reported as one of the commonest manifestations of hiv infection and include reactive arthritis / reiter 's syndrome , psoriatic arthritis , and undifferentiated spa . studies among caucasians have been associated with a positive human leukocyte antigen ( hla ) b27 in 7080% of patients while the hla b27 is usually negative in studies from africa . our patient had hiv - associated arthritis as the child had all negative autoimmune markers and the child responded to art . the major risk factor for the development of septic arthritis in hiv - positive patients was related to the intravenous drug abuse , rather than the hiv infection itself . infections with fungal species and atypical mycobacteria usually occurred late in the course of the disease when the cd4 count was < 100/mm . most of these conditions respond to symptomatic treatment with analgesics and nonsteroidal anti - inflammatory drugs and usually , settle with the use of art ( as seen in our patient ) . musculoskeletal manifestations although reported in the adult age group have hardly ever been reported in the pediatric age group with reiter 's syndrome being the commonest presentation . physical and rehabilitative therapy is needed to maintain the range of motion and strengthen muscle function in addition to administration of drugs in the management of hiv - associated polyarthropathy .

articular manifestations are a frequent but often underdiagnosed manifestation in patients infected with the human immunodeficiency virus ( hiv ) . we present a 7-year - old hiv - infected malnourished girl who presented with recurrent joint pain and effusion in the left knee joint . her antistreptolysin o , dsdna , antinuclear antibody and rheumatoid arthritis factor were negative . she responded to antiretroviral therapy .

lipedema was first described in 1940 by allen and hines , when it was defined as an abnormal deposition of adipose tissue in the lower limbs , without involvement of the feet , which usually affects women with a family history of the disease . the characteristics of lipedema are an excessive deposit of fat in the lower limbs with the legs being classically described as having an egyptian column shape , presence of edema , skin hypothermia , changes in plantar support and a negative stemmer sign [ 2 , 3 ] . it affects women more often than men with the initial diagnosis usually being made at puberty ; often the disease is aggravated by pregnancy and late - onset cases can occur during menopause [ 3 , 4 ] . histological analysis of the tissue shows proliferation of progenitor cells derived from adipose tissue and necrosing adipocytes . it is caused by an increase in adipogenesis leading to necrosis of adipocytes and hypoxia . studies evaluating changes in lipedema showed that in lipo - lymphedema the lymphatic vessels are dilated and with obstructive characteristics and dermal reflux and in lipedema they are dilated but without signs of reflux . the objective of this study is to demonstrate the reduction in the leg volumes ( edema ) in patients with lipo - lymphedema using manual and mechanical lymph drainage associated with short - strech compression stockings . the patient had been gaining weight with an increase in fatty tissue in the legs and arms . at the age of 24 years she started taking oral contraceptives and noted worse swelling and pain in the lower limbs . she was advised to suspend the use of the contraceptives and to start using a transdermal lymphatic system drug ( topical ) and to do physical exercise which partially improved the symptoms . three years ago she noted that the swelling was increasing without improvement at rest and sought a physician who raised the hypothesis of lymphedema and referred her to a specialized center . lipedema and lymphedema ( lipo - lymphedema ) were diagnosed in the physical examination , lymphedema was grade i. a three - day intensive treatment program ( 8 h daily ) was started for lymphedema which included manual and mechanical lymph drainage associated with short - strech ( < 50 mm hg ) compression stockings custom made using a cotton - polyester fabric . volumetry and perimetry were performed before starting and after the treatment and the legs were photographed ( fig . , there was volumetric reduction of more than 400 ml below the right knee with the greatest perimetric reductions being around 5 cm both above and below the knees ( table 1 , table 2 ) . the current study reports on perimetric and volumetric reductions of the legs of a lipedema patient . the results of the intensive lymph drainage employed in this case suggest that there is pathophysiological involvement of this system in the increased leg volume . however , the characteristic of these patients is an accumulation in fat in the extremities . with the passage of time , the main physiopathological change is necrosis of adipocytes which leads to cutaneous nodules [ 4 , 5 ] . however , changes in the lymphatic system showed that there was dilatation of the lymphatic collectors , which is suggestive of lymphatic stasis . in addition , obesity is common in individuals where the accumulation of fatty tissue occurs mainly in the legs . thus , changes in the lymphatic system may lead to increases in the volume of the legs . in this case the therapeutic approach led to reductions in volume confirming that the lymphatic system is responsible for this increase in volume . these patients may benefit from reduced leg size , but will continue with the characteristics of lipedema as the accumulation of adipose tissue is a characteristic of this disease . hence , the only possible conduct is a physiopathological intervention with lymph drainage and weight loss . studies in the literature using pressure therapy showed reductions in leg size also suggesting the involvement of the lymphatic system . the hypothesis that the lymphatic system is involved in lipedema seems to be related to cutaneous lymphostasis , where an obstructive pattern is not usually detected . other interventions , such as liposculpture , do not interfere in the pathophysiology but in the shape of the limb and the removal of fatty tissue . the present study supports the idea that this therapeutic approach interferes in the physiopathology , thus aggravating this physiological condition ( lipedema ) . stimulation of the lymphatic system can be used to reduce leg volumes in patients with lipedema , thus suggesting the regional involvement of cutaneous lymphostasis in this disease .

a 54-year - old female patient reported that a characteristic of her family was fat legs with postural edema since adolescence . over the years the patient had been gaining weight with an increase in fatty tissue in the legs and arms . at the age of 24 years she started taking oral contraceptives and noted worse swelling and pain in the lower limbs . she was advised to suspend the use of the contraceptives and to start using a transdermal lymphatic system drug and physical exercise which partially improved the symptoms . three years ago she noted that the swelling was increasing without improvement and sought a physician who raised the hypothesis of lymphedema and referred her to a specialized center . lipedema and lymphedema was diagnosed in the physical examination . a 3-day intensive treatment program ( 8 h daily ) was started for lymphedema which included manual and mechanical lymph drainage associated with short - strech ( < 50 mm hg ) compression stockings custom made using a cotton - polyester fabric . volumetry and perimetry were performed before starting and after the treatment and the legs were photographed . volumetric and perimetric reductions were obtained suggesting the involvement of regional cutaneous lymphostasis in this disease .

the causes of persistent submacular detachment after successful rhegmatogenous retinal detachment ( rrd ) surgery remain unknown . its presence is associated with poor postoperative visual acuity , but due to its spontaneous resolution no additional therapeutic or diagnostic procedure is recommended . a case of central serous chorioretinopathy ( csc ) that simulated persistent subfoveal fluid after rrd surgery is presented . to the authors knowledge , no other case of visual impairment after successful retinal detachment surgery due to csc has been reported in the pubmed database . in view of this report , csc should be considered in the differential diagnosis of persistent subretinal fluid after successful retinal detachment surgery . persistent submacular fluid after successful rhegmatogenous retinal detachment ( rrd ) surgery is associated with poor initial visual outcome.1 its spontaneous resolution may take several months and no additional procedure is recommended to accelerate its natural course as it does not seem to influence the final visual acuity.2,3 the causes of persistent foveal detachment are unclear ; however , its incidence is more often significantly reduced after vitrectomy and gas surgery than after scleral buckle procedure.1,2 central serous chorioretinopathy ( csc ) is an idiopathic serous detachment of the macula related to increased adrenergic and steroidal stimulation state and has been reported secondary to different surgical procedures.46 a case of csc misdiagnosed as persistent subretinal fluid after successful retinal detachment surgery , with no spontaneous improvement over an 18-month period , is reported here . a 48-year - old phakic man underwent successful pars plana vitrectomy and encircling buckle for a 360 macula - off rrd in his right eye . the patient was started postoperatively on prednisolone drops five times per day in a decreasing dosage for the following 5 weeks . at the 5-month visit , best corrected visual acuity was 0.4 in his right eye and persistent submacular fluid was identified on spectral - domain optical coherence tomography ( figure 1a ) with an increased epimacular signal that was believed to be a thickened internal limiting membrane . to relieve tangential macular tractions , which might have accounted for the persistent foveal detachment , the patient underwent a second vitrectomy with internal limiting membrane peeling and long - lasting gas . one month later , an increase in subretinal fluid was noted ( figure 1b ) . fundus fluorescein angiography revealed two retinal pigment epithelium leaking points in the juxtafoveolar area , which led to the diagnosis of csc ( figure 2 ) . the presence of submacular fluid after successful retinal detachment surgery is associated with poor postoperative visual acuity . no additional therapeutic procedure is recommended due to its spontaneous resolution , which can take up to 18 months and is accompanied by slow visual improvement . optical coherence tomography is useful in detecting persistent subretinal fluid , which is otherwise difficult to identify on clinical examination.2,7 the causes of persistent submacular detachment after successful retinal detachment surgery remain unknown.13 its incidence seems to be higher after scleral buckling surgery than after vitrectomy surgery ( 55% versus 15%).1,2 complete drainage of subretinal fluid during pars plana vitrectomy plus the displacement of fluid by a large gas bubble have been proposed to explain such differences.1 however , no definitive mechanism has been established . in the patient discussed , csc simulated persistent subfoveal fluid after rrd surgery . fundus fluorescein angiography , which is not routinely performed in this setting , revealed retinal pigment epithelium leaking points underlying the detached fovea . it has been proposed that csc is more likely in patients having type a personality and when its occurrence is preceded by a variety of stressful events in a patient s life.46 csc in the patient discussed here could have worsened after the second vitrectomy since subretinal fluid increased after internal limiting membrane peeling . another explanation for the clinical course of this patient could be the persistence of subretinal fluid after rrd repair and the developing of csc following macular surgery . to the authors knowledge , no other case of visual impairment after successful retinal detachment surgery due to csc has been reported in the pubmed database . in view of this report , csc should be considered in the differential diagnosis of persistent subretinal fluid after successful retinal detachment surgery . this disease is associated with increased levels of catecholamines ( stress ) and glucocorticoids ( steroidal medication ) , both related to ocular surgery for rrd .

backgroundthe causes of persistent submacular detachment after successful rhegmatogenous retinal detachment ( rrd ) surgery remain unknown . its presence is associated with poor postoperative visual acuity , but due to its spontaneous resolution no additional therapeutic or diagnostic procedure is recommended.case reporta case of central serous chorioretinopathy ( csc ) that simulated persistent subfoveal fluid after rrd surgery is presented.conclusionto the authors knowledge , no other case of visual impairment after successful retinal detachment surgery due to csc has been reported in the pubmed database . in view of this report , csc should be considered in the differential diagnosis of persistent subretinal fluid after successful retinal detachment surgery .

blastic plasmacytoid dendritic cell neoplasm ( bpdcn ) is recognized as an independent entity in the world health organization ( who ) 2008 classification for tumors of hematopoietic and lymphoid tissues and is thought to be derived from the precursors of plasmacytoid dendritic cells . recently , several reports suggested that , in patients with bpdcn , the skin is the first organ to become clinically involved . indeed , various clinical features of the skin area have been reported [ 2 , 3 , 4 ] . typically , the involved skin areas of bpdcn are described as showing solitary or multifocal bruise - like papules , nodules or plaques . the disease consistently runs an aggressive course and most patients die within the first year after the diagnosis is established . because previous reports indicate that early chemotherapy might show a temporary effect in some rare patients and even prolong the survival rate , the accurate and rapid diagnosis for this disease is indispensable [ 3 , 4 ] . a 75-year - old japanese woman visited us with a one - month history of nodules developing on her scalp . on her first visit to our hospital , physical examination disclosed multiple areas of disseminated , elastic hard nodules with multicentric purpura on her scalp ( fig . histopathologically , there were atypical large lymphocytes densely infiltrated through the upper dermis to subcutaneous tissue with minimal involvement of the overlying epidermis ( fig . immunohistochemical staining revealed that these infiltrated lymphocytes were cd4 , cd56 ( fig . 2c , d ) and vimentin positive , cd3 , cd5 , cd7 , cd8 and cd20 negative . flow cytometry of the bone marrow lymphocytes revealed cellular characteristics as follows : cd4 + , cd56 + , cd1 , cd2 , cd3 , cd5 , cd7 , cd8 , cd10 , cd14 , cd19 , cd20 , cd33 , cd34. from the above data , we diagnosed this patient as having blastic plasmacytoid dendritic cell neoplasm . we first administered thp - cop therapy ( pirarubicin , cyclophosphamide , oncovin and prednisone ) . after this intensive chemotherapy , the nodules on the scalp regressed temporarily ; however , two weeks later , the tumors were rapidly increasing . thus , we selected a - triplev therapy ( cytarabine , etoposide , vincristine and vinblastine ) and devic therapy ( carboplatin , ifosfamide , etoposide , dexamethasone ) but there was no effect on the tumor growth . four months after the administration of intensive chemotherapy , the patient died before restarting chemotherapy . cutaneous involvement is often the first manifestation of this disorder , and the lesions have specific features , presenting as solitary or multifocal bruise - like nodules and plaques . it was formally known as blastic natural killer ( nk ) lymphoma , or agranular cd4+cd56 + hematodermic neoplasm , and accounts for about 0.7% of all skin and primary malignant lymphoma . the main clinical features of bpdcn are infiltration of the skin and subsequent involvement of bone marrow and lymph nodes . its typical pathologic characteristics are uniformly distributed , dense , medium - sized lymphocytes in the dermis layer . tumor cells are similar in appearance , with a medium - sized , round , or irregular nucleus , and the nucleolus is not obvious [ 6 , 7 ] . typical immunohistochemistry results include positive for cd4 , cd56 , negative for cd3 , cd20 , myeloperoxidase , and cd33 . this type of lymphoma is highly aggressive and most patients with bpdcn die within a short period of time after diagnosis [ 4 , 8 ] . although early chemotherapy may show a temporary effect in some rare patients , the disease often recurs soon afterward . however , the chance of post - treatment recurrence is much higher than with other malignant lymphomas . in our present case , systemic spread of the tumor thus , even though we administered intensive chemotherapy soon after the diagnosis , the patient died 5 months after her first visit to our clinic . therefore , dermatologists need to be familiar with the clinical features of bpdcn to diagnose this disease immediately .

we describe a 75-year - old japanese woman with blastic plasmacytoid dendritic cell neoplasm . on her first visit , there were multiple areas of disseminated , elastic hard nodules with multicentric purpura on her scalp . histopathologically , there were atypical large lymphocytes densely infiltrated through the upper dermis to subcutaneous tissue . immunohistochemical staining revealed that these infiltrated lymphocytes were cd4 , cd56 and vimentin positive . although we administered several courses of intensive chemotherapy soon after the diagnosis , the patient died 5 months after the first visit .

a 60-year - old male presented to the ophthalmology clinic with a 5-year history of epiphora and a 1-month history of bloody discharge from the right lower punctum . the patient did not complain of marked medial canthal swelling or tenderness . on examination , best corrected visual acuity was 20/20 bilaterally . there was mucoid discharge with a blood clot found in the right lower punctum [ fig . pressure over the lacrimal sacs produced bloody discharge , suggesting a primary lacrimal sac tumor [ fig . syringing and dacryocystography demonstrated a complete obstruction at the level of the lacrimal sac [ fig . ( a ) mucoid discharge with blood clot of the right lower punctum was observed . ( b ) pressure over the lacrimal sacs resulted in expression of bloody discharge complete obstruction of lacrimal drainage at the level of lacrimal sac shown on dacryocystography . dacryocystography revealed a complete obstruction of lacrimal drainage at the level of the common canaliculus the patient then underwent a computed tomography ( ct ) scan which demonstrated a solid mass , measuring 1.2 cm 1.2 cm 1.6 cm , centered on the enlarged right lacrimal sac fossa [ fig . the patient was advised to undergo an open dcr with excisional biopsy of the mass , lacrimal sac and the proximal portion of the nasolacrimal duct . a solid , well - circumscribed , 1.2 cm 1.2 cm 1.6 cm mass centered on the enlarged right lacrimal sac fossa . ( a ) computed tomography revealed a solid , well - circumscribed mass centered on the enlarged right lacrimal sac fossa . ( b ) the excised mass was oval - shaped and measured 1.2 cm 1.2 cm 1.6 cm a relatively well - circumscribed 1.2 cm 1.2 cm 1.6 cm solid mass [ fig . the specimen was subsequently evaluated for sma , s-100 , p63 , ck5 , ck6 , and alcian blue . the specimen was made up of variable patterns of epithelial and spindle cells in a hyalinized stroma with apocrine differentiation , similar to the histologic features of a pleomorphic adenoma of the lacrimal gland [ fig . histology of the tissue showed variable patterns of epithelial cells and spindle cells in a hyalinized stroma with apocrine differentiation ( h&e , 40 ) one month after excision of the mass , the patient underwent a repeat ct , and no evidence of recurrence was found . the patient complained of epiphora in the right eye after surgery and underwent canaliculo - rhinostomy with silicone tube intubation . neoplasms of the lacrimal drainage system are uncommon , but are potentially life - threatening and are often difficult to diagnose . among primary lacrimal sac tumors , the benign mixed tumor , also known as a pleomorphic adenoma , is histologically classified as a benign epithelial tumor . pleomorphic adenomas are common benign salivary gland tumors and are the most common tumors of the parotid gland . histologically , they are characterized by an admixture of polygonal epithelial elements with ductal or acinar arrangement and spindle - shaped myoepithelial elements in a variable background stroma that may be mucoid , myxoid , cartilaginous or hyaline . ophthalmologically , benign mixed tumors usually arise from the lacrimal gland , but are an extremely rare form of a primary lacrimal sac tumor . furthermore , the origin of these glandular structures in the walls of the lacrimal sac and nasolacrimal duct is not completely understood , because the embryology of the human lacrimal system is not clearly defined in the literature . benign epithelial lacrimal sac tumors are often managed by external dcr and simple excision of the mass . the most common symptoms indicating dysfunction of the lacrimal drainage system include punctual discharge and epiphora . bloody discharge and mass above the medial canthal ligament imply tumorous condition of lacrimal sac . in addition to a full ophthalmic examination ( ocular surface , eyelid , assessment of the lacrimal drainage system , syringing and probing and digital expression of lacrimal sac contents ) , imaging studies including dacryocyctography and ct should reserve for selected patients with tumorous condition . in the case of suspicious of lacrimal sac tumor , external dcr is regarded as the gold standard in terms of surgical success . the external approach allows an excellent possibility for inspection of the lacrimal sac and for biopsy of abnormal appearing findings . our patient had a benign mixed tumor with no evidence of the other tumor foci or systemic spread . the patient underwent open dcr with excisional biopsy of the mass , lacrimal sac , and the proximal portion of the nasolacrimal duct and after 1-month , canaliculo - rhinostomy with silicone tube intubation for the treatment of epiphora . although benign mixed tumors of the lacrimal sac are very uncommon , this case provides a rationale for including this condition in the differential diagnosis of lacrimal sac masses .

neoplasms of the lacrimal drainage system are uncommon , but potentially life - threatening and are often difficult to diagnose . among primary lacrimal sac tumors , benign mixed tumors are extremely rare . histologically , benign mixed tumors have been classified as a type of benign epithelial tumor . here we report a case of benign mixed tumor of the lacrimal sac .

in the previous issue of critical care , jacques and colleagues report that respiratory variations in stroke volume ( svv ) and in arterial pulse pressure ( ppv ) remain reliable indices of fluid responsiveness in a porcine model of intra - abdominal hypertension ( iah ) . threshold values , however , are higher than during normal intra - abdominal pressure ( iap ) , so that a ' supra - normal ' svv or ppv does not necessarily mean fluid responsiveness during iah dynamic indices such as ppv and svv are accurate predictors of volume responsiveness in critically ill patients under controlled positive pressure ventilation , with an accuracy greater than that of traditional static indices of cardiac preload . the mean threshold values allowing optimal discrimination between fluid responders and non - responders were 12.5 1.6% for ppv and 11.6 1.9% for svv in a recent systematic review of the literature . these results , however , did not include patients where conditions prevented correct measurement of these indices ( such as cardiac arrhythmias or spontaneous ventilation ) or may have been associated with reduced accuracy ( especially small tidal volumes or acute corpulmonale ) . a third category of circumstances , where dynamic indices remain robust indicators of fluid responsiveness if ( and only if ) used with different thresholds , may also be identified and constitute an additional refinement in ppv / svv interpretation . it was suspected early on that the magnitude of tidal volume would influence the threshold value of functional hemodynamics . more recently , biais and coworkers showed that prone position ( for scoliosis surgery ) does not alter the ability of both ppv and svv to predict fluid responsiveness , but induces a significant increase in ppv and svv , probably related to a decrease in static compliance of the respiratory system . accordingly , the ' optimal ' threshold value for ppv in this study was 11% in the supine position , and 15% in the prone position . in recent years cardio - vascular dysfunction and failure are commonly encountered in the patient with iah , and one of the bases of management is optimization of systemic perfusion and organ function , which includes accurate assessment of preload and preload responsiveness . however , static indices of preload are difficult to interpret in patients with iah . assessing fluid responsiveness with the passive leg raising maneuver in these patients results in false negative cases . experimental studies have shown that iah increases dynamic variables and , accordingly , 25% of patients with iah may be non - responsive to volume infusion despite a ppv > 12% . recently , renner and colleagues found in a porcine model that ppv , but not svv ( derived from pulse contour analysis ) , remained a sensitive and specific predictor of fluid responsiveness , although the threshold value for ppv increased from 11.5% ( mean iap = 7 mmhg ) up to 20.5% ( mean iap = 26 mmhg ) . thus , what does the study by jacques and colleagues add to previous knowledge ? first , they confirm former results with a protocol that elegantly combines , for the first time , changes in iap , blood withdrawal , and fluid loading . second , they show that svv , when directly measured using an ultrasound transit - time flow probe placed around the aortic root , is also predictive of fluid responsiveness in iah . this contrasts with the results reported by renner and colleagues , who acquired svv via pulse contour monitoring , suggesting that the accuracy of the latter may be altered during iah . third , their data strongly suggest that , at least in their experimental conditions , the ' non - responsive ' part of the increase in dynamic indices during iah is due to an increase in right ventricular afterload . these new data should , however , be extrapolated to critically ill patients with caution . in this study , baseline ppv and svv were much higher than in humans or in other experimental studies . high tidal volume as used in the study ( 13 1 ml / kg in the presence of severe reduction in chest wall compliance ) may explain , in part , these findings as well as the significant increase in right ventricular afterload , a result not found by renner and colleagues . in addition , the high iap level used in the study ( 30 mmhg ) corresponds to the more severe level ( grade iv , iah > 25 mmhg ) of iah in patients . finally , a high but discriminative threshold could be identified by the authors because a precise and controlled value of iap was induced in all animals , which would not be the case in clinical practice . thus , what is demonstrated in this study is qualitative ( there is an increase in dynamic indices in the presence of iap , with one part responsive to fluid infusion ) rather than quantitative . the consequences for clinical practice are thus that iap must be measured in critically ill patients , and dynamic indices such as ppv should be used , but , in order to avoid excess of fluids , higher than classical ( 10 to 13% ) thresholds should be considered when iap is increased . as a gradual increase of threshold values with iap is very likely , no precise value can be recommended so far . in a given patient with increased ppv , a fluid challenge may be performed and the resulting change in ppv / svv quantified since only the ' preload - dependent part ' of ppv is likely to be reduced with volume infusion . whether the ppv value obtained at completion of such fluid load in such a patient may be used as the ' operational ' threshold for the following hours of management , provided that other major determinants of ppv ( iap and tidal volume ) are kept constant , iah : intra - abdominal hypertension ; iap : intra - abdominal pressure ; ppv : respiratory variation in arterial pulse pressure ; svv : respiratory variation in stroke volume .

dynamic variables of fluid responsiveness are useful guides for fluid management in patients under controlled positive pressure ventilation . in the previous issue of critical care , jacques and colleagues show that these variables remain reliable predictors of fluid responsiveness in a porcine model of intra - abdominal hypertension , but threshold values are higher than during normal intra - abdominal pressure . their threshold values , however , can not be applied to clinical practice . this study suggests that intra - abdominal pressure must be measured in critically ill patients , and ' supranormal ' values of dynamic variables should be analyzed with caution . the ' fluid responsive part ' of an increased dynamic variable in such patients may be estimated by measuring its change during a fluid challenge .

the term diabetic papillopathy ( dp ) is used for unilateral or bilateral optic disc swelling in which the patient has either type 1 or type 2 diabetes mellitus.1 this entity is diagnosed by excluding the other causes of disc swelling.2 the exact pathogenesis is not clearly understood but some authors consider dp as a form of anterior ischemic optic neuropathy ( aion).3 unlike aion ; dp has a benign course without significant sequela.1 although there is no accepted treatment paradigm ; in some recent case reports , treatment with intravitreal injection of different agents was effective in reducing disc swelling.46 in this study we report a case of dp treated with intravitreal ranibizumab injection and demonstrate resolution of optic disc swelling . written informed consent has been provided by the patient to have the case details and accompanying images published . a 53-year - old male presented with acute painless decreased vision in his right eye . the patient was on a regimen of oral hypoglycemic agents and had no other known medical disease . best - corrected visual acuity ( bcva ) was 1/10 in the right eye and 10/10 in the left eye . dilated fundus examination showed significant bilateral non - proliferative diabetic retinopathy and a swollen optic disc with telangiectatic vessels in the right eye ( figure 1a and b ) . fundus fluorescein angiography ( ffa ) and optical coherence tomography were performed . in the right eye there was dye leakage from the optic disc and a significant increase in the retinal nerve fiberlayer thickness without macular edema ( figure 2a and b ) . visual field examination of the right eye showed diffuse depression whereas the left eye was normal . blood pressure measurements , complete blood count , sedimentation rate and electrolytes were within normal limits . after complete examination of the patient , he was diagnosed with dp and informed about expected effects and possible complications of intravitreal ranibizumab injection . after the patient was informed about the expected effects , the right eye was prepared and draped in a sterile manner and topical anesthetic was instilled in the eye . subsequently , 0.5 mg/0.05 ml ranibizumab was injected into the vitreous cavity . within 2 weeks , three months following the injection , bcva was 6/10 , iop was normal , and the optic disc was mildly pale with complete resolution of swelling in the right eye ( figure 4a c ) . acute , painless visual loss is a clinical sign of optic disc swelling that is caused by a variety of reasons . dp is one of these reasons and it has been published by many investigators since 1971.7,8 although it is thought to be a mild form of non - arteritic aion by some researchers,3 dp can be distinguished from aion by certain clinical findings . in contrast to patients with dp , aion patients suffer from profound loss of visual acuity . after the resolution of optic neuropathy visual field defect and visual acuity loss persists in patients with aion . on the other hand ; in dp patients visual field defects are transient and visual acuity improves to 4/10 or more.1,2 these two conditions can also be distinguished by ffa . early disc hypofluorescence due to hypoperfusion with late leakage around the affected segment occurs in aion . but in patients with dp , ffa shows a very early hyperfluorescence , most likely due to telangiectasia of the optic disc that increases throughout the study.9 the patient was diagnosed as having dp because of the following : painless visual loss , no other clinical disorder except uncontrolled diabetes mellitus , early disc hyperfluo - rescence on ffa and good visual outcome post - treatment . anti - vegf agents have recently been used for some reported dp cases with visual improvement and reduced optic disc swelling.4,5 periocular and intravitreal corticosteroid injection was also found to be effective in patients with dp.6 these effective treatment options indicate that inflammatory elements could be involved in the pathogenesis of this disorder . so , if an optic nerve head is ischemic there will be an increased amount of vegf leading to vasogenic disc edema.10 but still the exact pathogenesis of dp and the mechanism of action of anti - vegf in patients with dp is not well understood . after 2 weeks , disc swelling had largely resolved and visual acuity had improved from 1/10 to 6/10 . there were no complications at all and dp had not reappeared at the 3-month follow - up . two weeks after intravitreal ranibizumab injection , there was an increase in visual acuity in our patient . vegf may play a role in the pathogenesis of dp and so the administered intravitreal ranibizumab could have helped the patient to recover . however , the efficacy and safety of this management of dp needs to be proven through further larger clinical studies .

in this report , we present a case of diabetic papillopathy that resolved after a single dose of intravitreal ranibizumab injection . a 50-year - old male presented with painless visual loss in his right eye . his visual acuity was 1/10 in the right eye and 10/10 in the left eye . anterior segment examination of both eyes was unremarkable . posterior segment of the right eye showed nonproliferative diabetic retinopathy with a swollen optic disc . fluorescein angiography and optical coherence tomography were performed . there was dye leakage from the right optic disc . optical coherence tomography revealed a significant increase in retinal nerve fiber layer thickness . magnetic resonance imaging of the brain and orbit were normal . the patient received a single intravitreal ranibizumab ( 0.5 mg ) injection . two weeks after the injection , there was a marked regression of the disc swelling . three months after the injection the optic disc was pallor and visual acuity was 6/10 .

posterior reversible encephalopathy syndrome ( pres ) was initially described in 1996 by hinchey and co - workers , as reversible posterior leukoencephalopathy syndrome.1 it is characterized by variable associations of seizure activity , consciousness impairment , headaches , visual abnormalities , nausea / vomiting , and focal neurological signs.2 the term pres describes a potentially reversible imaging appearance and may occur in diverse situations . while most cases are due to systemic hypertension ( htn ) , other conditions and entities have been identified as etiologic or risk factors in the absence of htn , such as immunosuppressant drugs use , nephrotic state , sepsis , and systemic lupus erythematosus ( sle).24 the findings on neuroimaging in pres are often symmetric and predominate edema in the white matter of the brain areas perfused by the posterior brain circulation , which is reversible when the underlying cause is treated . the treatment is based in the management or withdrawal of the triggering factor.5 there were cases of pres related with electrolyte disturbance . however , there was no reported case of pres associated with hyponatremia in korea . the patient is a 63-year old female who visited our emergency department with altered mental status . two weeks ago , she admitted local medical center for left knee pain and underwent arthroscopic surgery of cartilage resection . after discharge , she continuously suffered from nausea and vomited more than 20 times a day , leading to dehydration and weight loss . she had no history of hypertension , hematologic disorders , or other systemic disease such as diabetes mellitus . on admission , her vital signs were stable . brainstem sign was intact and deep tendon reflexes were normoactive bilaterally . in our emergency room she showed continuously drowsy mentality and complained of severe headache . in laboratory test , she showed hyponatremia , which is 124 mmol / l . cerebrospinal fluid study revealed normal , which was performed to exclude central nervous system infection . brain magnetic resonance imaging ( mri ) showed bilaterally diffuse abnormal signal intensities in the parieto - occipital lobe and superior frontal sulcus , which involved predominantly the deep white matter ( fig . her clinical symptoms and brain mri findings suggested the possibility of pres . during admission , after three days , her sodium level was normal and she did not complain of lethargy or somnolence . after one month , follow - up brain mri revealed that abnormal signals involving both parieto - occipital cortex and superior frontal sulcus were much improved ( fig . most of the previously described pres had associated extremely high blood pressure or renal insufficiency.13 other etiologies are known such as pregnancy , immunosuppressant drugs use , nephrotic state , sepsis , autoimmune diseases and cns infection.5 various previous studies reported other miscellaneous associations such as hypomagnesemia , hypercalcemia , tumor lysis syndrome , rapid correction of anemia , hiv infection and esrd.5,6 numerous aspects regarding the pathogenesis of this entity are yet to be elucidated . although there are many proposed mechanisms for the patho - physiology of pres , the most widely accepted is a temporary failure of autoregulatory capabilities of the cerebral vessels , leading to hyperperfusion , a breakdown of the blood brain barrier and consequent vasogenic edema with some component of endothelial dysfunction.5,6 in our case , she had no other etiology such as high blood pressure , but she showed only hyponatremia . despite the importance of blood vessels function for the homeostasis of the brain , little is known about the influence of hyponatremia on cerebrovascular regulation.7 one previous study demonstrated that the dilatation of the cerebral vessel in response to acetylcholine , which is endothelium and nitric oxide dependent , was severely impaired in hyponatremia.8 cerebral hypoxia due to vasoconstriction probably makes impairment of brain adaptation due to dysfunction of na+k+atpase , which is primary importance for the extrusion of ions from the glial cell.9 also , vasopressin elevated in cases of hyponatremia . vasopressin leads to decreased cerebral oxygen utilization and facilitates direct movement of water into brain cells independent of the effects of hyponatremia.10 therefore , these mechanisms in hyponatremia may have an important impact on the brain edema and could explain our case . to our knowledge , this is the first case of pres associated with hyponatremia in korea . we suggest that a rapid change of sodium level may cause disruption of cerebral autoregulation , which result in pres . while pres is usually reversible , the early recognition and treatment of this syndrome is important to prevent permanent neurological sequelae . further investigation is needed to elucidate the association between sodium level and the development of pres .

posterior reversible encephalopathy syndrome ( pres ) is characterized by variable associations of seizure activity , consciousness impairment , headaches , visual abnormalities , nausea / vomiting , and focal neurological signs . the pres may occur in diverse situations . the findings on neuroimaging in pres are often symmetric and predominate edema in the white matter of the brain areas perfused by the posterior brain circulation , which is reversible when the underlying cause is treated . we report the case of pres in normotensive patient with hyponatremia .

isotopic response was first described by wolf et al . in 1985 and hence called wolf 's isotopic response . it refers to the occurrence of a new skin disorder at the site of another , unrelated , healed skin disease . diseases such as lichen planus , granuloma annulare , morphea , and scar sarcoidosis can occur over healed lesions of herpes virus infection . we present here a hitherto unreported occurrence of secondary syphilitic lesions developing over healed lesions of varicella . a 28-year - old housewife presented to us with a generalized , asymptomatic , reddish eruption , which had developed over the previous couple of weeks . the patient had varicella 2 months ago , which was diagnosed and treated by her family physician . her current lesions occurred precisely over the sites of healed lesions of varicella on the face , trunk , limbs , as well as the palms and soles . there was no history of any major illness , drug allergy , or genital ulcers . the patient denied any history of extramarital sexual exposure but her spouse had a history of sexual exposures with commercial sex workers on several occasions . on examination , she had multiple , erythematous , mildly scaly papules and plaques almost all over her body [ figure 1 ] . several small slightly depressed scars and hyperpigmented macules were visible over her face and limbs . there were sharply defined , hyperpigmented , scaly , annular lesions over the palms and soles [ figure 2 ] . a few mobile , nontender , widely scattered , erythematous , mildly scaly papules and plaques sharply defined , hyperpigmented , scaly , annular lesions over palms and soles erosions over hard palate venereal disease research laboratory ( vdrl ) test was reactive in 1:16 dilutions . her partner was treponema pallidum hemagluttination assay ( tpha ) positive but vdrl non - reactive . enzyme - linked immunosorbent assay for human immunodeficiency virus ( hiv - elisa ) of both was nonreactive . there was dermal edema , profuse pandermal infiltration of chronic inflammatory cells , chiefly plasma cells and lymphocytes [ figure 4 ] . she was given injection benzathine penicillin ( 2.4 million units ) intramuscularly , which resulted in rapid clinical improvement within a few days . intense upper dermal infiltrate composed primarily of plasma cells ( h and e , 100 ) the most common primary disease in an isotopic response is herpes zoster as compared with other herpes virus infections such as herpes simplex and varicella . the second unrelated diseases that may occur on the healed primary lesions include lichen planus , granulomatous dermatitis , granuloma annulare , morphea , rosacea , scar sarcoidosis , acne , kaposi 's sarcoma , erythema annulare centrifugum , xanthomas , psoriasis , and cutaneous malignancies . some authors have suggested immunologic and vascular changes following neural alteration by herpes virus , as causative factors for the localization . the role of neuropeptides , including vasoactive intestinal peptide , neuropeptide y , calcitonin - gene - related peptide , and substance p , was also implicated to influence the immune system . thus , a disturbed nervous system due to herpes virus infection may alter the immunologic system and then consequently the skin that leads to the localization of the second disease . the secondary stage of syphilis , the great imitator , can present with a myriad of clinical and histological features . lesions called syphilids may develop in 80 - 95% of the cases , usually 3 - 12 weeks after the appearance of a chancre , which may be unnoticed , especially in women . it is characterized by a maculopapular rash , symmetrically distributed , involving the palms and soles , associated with flu - like prodrome and lymphadenopathy . papular syphilids are observed in approximately 12% of the cases and may be of different patterns , such as papulosquamous , follicular , lenticular , corymbose , lichenoid , nodular , or annular . serological tests such as vdrl , rpr , tpha , and histologic features of psoriasiform or lichenoid epidermal hyperplasia , a dermal , predominantly plasma cell infiltrate corroborate the diagnosis of secondary syphilis . we considered guttate psoriasis , lepromatous leprosy , sarcoidosis , and pityriasis lichenoides et varioliformis acuta in the differential diagnosis . the presence of risk factors , absence of anesthesia or pruritus , positive serology , corroborative histopathology , and dramatic response to penicillin helped us to establish the diagnosis of secondary syphilis . in the literature , several cases of isotopic response and many atypical varieties of secondary syphilis have been documented . however , to the best of our knowledge , secondary syphilis occurring as second disease over healed lesions of varicella as wolf 's isotopic response has not been reported earlier . secondary syphilid can be considered as the newest among those diseases that can develop over healed lesions of herpes virus infection as isotopic response .

isotopic response is a distinctive phenomenon in which a new skin disorder occurs at the site of another , unrelated , and already healed skin disease . most of the cases documented in the literature were associated with herpes zoster as primary disease while the list of second diseases is quite long . we report here a hitherto unreported occurrence of isotopic response in which secondary syphilis occurred on the healed lesions of varicella .