To characterize the clinical, laboratory, imaging and pathological features of primary sclerosing cholangitis (PSC) and investigate the impact of ursodeoxycholic acid (UDCA) therapy on patient prognosis.The medical records of 22 patients diagnosed with PSC between 2002 and 2011 were retrospectively reviewed. The PSC diagnosis had been made in patients with suspect biochemical abnormalities following evaluation by magnetic resonance cholangiopancreatography (MRCP) and/or endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC). Fibrosis and inflammaton were assessed by immunohistochemical analyses of tissue biopsies. Outcome of patients treated with UDCA (13-15 mg/kg/day, oral) were compared to that of patients without UDCA treatment by the X2 or corrected X2 tests.Among the 22 PSC patients, the majority was male (n=15) and presented with fatigue, dark urine, and body weight loss (n=15). Four cases had ulcerative colitis. At admission, all 22 cases showed elevated levels of alkaline phosphatase[ALP: (348+/-184) U/L], 19 cases showed elevated alanine aminotransferase [ALT: (94.0+/-67.0) U/L] and aspartate aminotransferase [AST: (98.0+/-67.0) U/L], and 15 cases showed elevated levels of total bilirubin (99.0+/-115.0) mumol/L and direct bilirubin (74.4+/-92.4 mumol/L. ERCP examination showed segmental intrahepatic bile duct stenosis with expansion, and stiff and enlarged gallbladder bile ducts, but unclear findings for the common bile ducts and pancreatic ducts. MRCP showed beading of the intrahepatic bile duct, stiffness of the bile duct wall, and dilation of the common bile duct. Fibrosis and inflammation were observed in the bile ducts, along with hyperplasia and the typical features of "onion skin" fibrosis and fibrous obliterative cholangitis. Five of the 10 patients treated with UDCA improved, and seven of the 12 patients in the non-UDCA treatment group improved. There was no statistically significant difference in outcome between the groups (paired X2=0.333, corrected X2=0.083, P more than 0.05).PSC patients were predominantly male and the common clinical manifestations were fatigue, dark urine, and body weight loss. At admission, serum biochemical indicators of cholangitis were increased significantly and subsequent imaging studies confirmed the suspected diagnosis by showing obvious characteristic changes. UDCA treatment did not significantly improve patient prognosis.

A 69-year-old man with advanced heart failure treated with a continuous-flow left ventricular assist device presented for evaluation of dark urine and severe dysphagia. Because of evidence of ongoing intravascular hemolysis with device dysfunction, there was a clinical suspicion for pump thrombosis. He had progressive end-organ dysfunction and was therefore treated with tissue plasminogen activator with prompt resolution in hemolysis and dysphagia. Although symptoms of smooth muscle dystonia could represent worsening heart failure in the setting of device failure, the observation may also be related to intravascular hemolysis as described in the prototypic hemolytic disease, paroxysmal nocturnal hemoglobinuria.

An 18-year-old female with sickle cell disease presented with thigh pain, dark urine, and hematuria within 72 hours of receiving a blood transfusion. Her clinical picture was consistent with hemolysis. Subsequent laboratory workup, however, demonstrated reticulocytopenia without evidence of an antibody-mediated transfusion reaction. As her hemoglobin continued to decrease, she was treated with IVIG and steroids for presumed hyperhemolysis. Clinicians should have a high index of suspicion for hyperhemolysis in sickle cell patients with evidence of hemolysis after a recent transfusion. Differentiating hyperhemolysis from other hemolytic syndromes is critical; transfusions in a hyperhemolytic episode can accelerate hemolysis causing life-threatening anemia.

Plasmacytomas are malignant tumors characterized by abnormal monoclonal proliferation of plasma cells. They originate in either bone--solitary osseous plasmacytoma, or in soft tissue--extramedullary plasmacytoma (EMP). EMP represents less than 1% of all head and neck malignancies.We presented a case of EMP of the nasal septum in a 44-year-old male who had progressive difficulty in breathing through the nose and frequent heavy epistaxis on the right side. Nasal endoscopy showed dark red, soft, polypoid tumor in the last third of the right nasal cavity arising from the nasal septum. The biopsy showed that it was plasmacytoma. Bence Jones protein in the urine, serum electrophoresis, bone marrow biopsy, skeletal survey and other screening tests failed to detect multiple myeloma. This confirmed the diagnosis of EMP. The mass was completely removed via an endoscopic approach, and then, 4 week later, radiotherapy was conducted with a radiation dose of 50 Gray. No recurrence was noted in a 3-year follow-up period.EMP of the nasal cavity, being rare and having long natural history, represents a diagnostic and therapeutic challenge for any ear, nose and throat surgeon. Depending on the resectability of the lesion, a combined therapy is the accepted treatment.

A 26-year-old male presented with three weeks of jaundice after the self-initiation of the injectable anabolic steroid, Mastabol [Dromastanolone Di-Propionate (17 beta-Hydroxy-2alpha-methyl-5alpha-androstan-3-one propionate)]. He reported dark urine, light stools, and pruritus. He denied abdominal pain, intravenous drug use, intranasal cocaine, blood transfusions, newly placed tattoos, or sexually transmitted diseases. He used alcohol sparingly. Physical exam revealed jaundice with deep scleral icterus. The liver was palpable 2 cm below the right costal margin with no ascites. The peak bilirubin was 23.6 mg/dL, alkaline phosphatase was 441 units/L, and aspartate aminotransferase/alanine aminotransferase were 70 units/L and 117 units/L respectively. A working diagnosis of acute intrahepatic cholestasis was made. Liver biopsy revealed a centrilobular insult with neutrophilic infiltrates and Ito cell hyperplasia consistent with acute drug induced cholestasis. The patient's clinical symptoms resolved and his liver enzymes, bilirubin, and alkaline phosphatase normalized. Anabolic steroids with 17 alpha carbon substitutions have been associated with a bland variety of cholestatic injury with little hepatocellular injury. Cholestasis, under these circumstances, may be secondary to the binding of drugs to canalicular membrane transporters, accumulation of toxic bile acids from canalicular pump failure, or genetic defects in canalicular transport proteins. Mastabol is an injectable, 17 beta hydroxyl compound with no alpha alkyl groups at the 17 carbon position. As such, it has been reported to have little potential toxic effects on the liver. This is the first known reported case of Mastabol-induced cholestatic liver injury. It highlights the need for physicians to consider such widely available substances when faced with hepatic injury of unclear etiology.

In 2012, there were 402 incident episodes of rhabdomyolysis likely due to physical exertion and/or heat stress ("exertional rhabdomyolysis") among U.S. service members. The annual rates of exertional rhabdomyolysis increased 30 percent from 2008 to 2012. Th e highest incidence rates occurred in males, black, non-Hispanic service members, service members younger than 20 years of age, members of the Army and Marine Corps, recruit trainees, and those in combat-specific occupations. Incidence rates were higher among service members with homes of record from the Northeast compared to other regions of the U.S. Most cases were diagnosed at installations that support basic combat/recruit training or major Army or Marine Corps ground combat units. Medical care providers should consider exertional rhabdomyolysis in the differential diagnosis when service members - particularly recruits - present with muscular pain and swelling, limited range of motion, and/or the excretion of dark urine (e.g., myoglobinuria) after strenuous physical activity, particularly in hot, humid weather.

We report two cases of biliary fascioliasis in patients from the highlands of Peru; with several months of symptoms characterized for biliary-type pain, dark urine and jaundice in one case. Both of them referred to the department of Gastroenterology of the Hospital Nacional Guillermo Almenara Irigoyen for suspicious of choledocolithiasis. Laboratory tests were performed which showed variable high levels of functional liver tests (cholestasis) and marked eosinophilia in one case. Magnetic resonance cholangiography (CRMN) was performed and then informed as an apparent image of biliary stone in choledocus in both cases. Endoscopic retrograde cholangiopancreatography (ERCP) was performed and after sphincterotomy, one flattened, lanceolate parasite, with active mobility morphologically compatible with Fasciola hepatica was extracted. After the ERCP antiparasitic treatment was given with triclabendazole. Clinical outcome was favourable.Key words: Fasciola hepatica; Fascioliasis; Cholangiopancreatography, endoscopic retrograde (source: Me SH NLM).

Duodenal duplication is a rare congenital malformation and has been reported as a rare cause of recurrent acute pancreatitis. Hemorrhagic ascites has been reported in only one case of duodenal duplication.An 11-year-old Chinese girl presented with abdominal pain, hematemesis and dark stools. On admission, an abdominal examination revealed a moderately distended abdomen with diffuse tenderness. Biochemical investigations showed increased serum levels of amylase, lipase, and urine amylase. An abdominal computed tomography scan and magnetic resonance imaging scan revealed an enlarged and heterogeneous pancreas with poorly delineated borders. There was a cystic lesion measuring 25mm × 48mm × 28mm, located between the descending portion of her duodenum and the head of her pancreas. There were massive effusion signals in her abdominal cavity. An exploratory laparotomy was performed. A tubular cyst measuring 32mm × 52mm × 30mm was found in the second part of the duodenum, next to the head of her pancreas. The anterior wall of the duplication cyst was resected and anastomosis of the remaining cyst to the duodenum was performed for drainage. Histopathological examination of the excised cyst wall showed duodenal mucosa, submucosa and muscle coats, indicative of a duodenal duplication.It is important to be aware of duodenal duplication when evaluating a patient with recurrent acute pancreatitis accompanied by massive hemorrhagic ascites.

Dengue is endemic in Sri Lanka and the physician should be aware of different and unusual presentation of the illness. Rhabdomyolysis is a well-known complication following many viral and bacterial infections; however, only a few cases have been reported with dengue viral infections. Further occurrence of coinfection by dengue and bacteria such as methicillin-resistant (MRSA) has been underestimated, and few reports have been published so far. This case describes a 17-year-old boy who presented with prolonged severe myalgia, dark red urine, and a febrile illness that was diagnosed as having dengue viral myositis, dark red urine, and a febrile illness that was diagnosed as having dengue viral myositis complicated with rhabdomyolysis and superinfection of MRSA. Despite intensive care management, he died due to multiorgan failure. Autopsy and serological studies confirmed the diagnosis. This case stresses that red-coloured urine in dengue patients is not always due to haematuria, and if a patient's vital signs do not respond to appropriate fluid management in DHF, sepsis from a secondary pathogen including MRSA should be suspected.

Endocrinology is a useful tool for conservation biologists and animal managers, and measuring glucocorticoids can help understand biological mechanisms associated with species decline and animal welfare. The current study describes the development and optimization of a glucocorticoid enzyme immunoassay (EIA) to non-invasively assess adrenal activity in a variety of taxa. The antiserum (CJM006) was raised in rabbits to a corticosterone-3-CMO-BSA immunogen and used in a standard competitive EIA system. However, the EIA initially produced results with unacceptably high inter-assay variation, attributed to consistent patterns observed within the optical density of developing plates. To determine the cause of this variability, a number of factors were examined using synthetic corticosterone standard and endogenous faecal extract, including: plate type (Nunc MaxiSorp® II versus Immulon IB plates); the use of non-specific secondary antibody; type (artificial versus natural) and presence (light versus dark) of light during incubation; plate loading temperature (4°C versus room temperature); and substrate reagent temperature (4°C versus room temperature). Results indicated that variability was associated with plate location effects, which were not initially detected because control samples were always run in the same positions across plates. Light and temperature were the two major factors that affected EIA reliability. For this assay, the standard protocol required slight modification, with the optimal protocol using Nunc MaxiSorp® plates, room temperature substrate reagents and dark incubation conditions. Following optimization, this EIA was then validated biochemically for 38 species, through parallel displacement curves and interference assessment tests of faecal and urine samples. Additionally, biological validation was performed opportunistically in a subset of species, with use of this EIA demonstrating significant elevations in faecal glucocorticoid metabolites following potentially challenging events. In summary, this glucocorticoid EIA cross-reacts with excreted glucocorticoid metabolites across a wide range of taxa, including ungulates, primates, felids, birds, rodents and amphibians. We conclude that when used with optimal reagent and incubation conditions, this EIA will be useful for non-invasive monitoring of adrenal activity in a wide range of wildlife species.