INTRODUCTION CREST (calcinosis, Raynaud phenomenon, oesophageal dysmotility, sclerodactyly, and telangiectasia) syndrome comprising calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia and primary sclerosing cholangitis are both chronic fibrotic diseases but the association between them is extremely rare. While primary sclerosing cholangitis has been as...

Cholangitis is relatively uncommon (Boey & Way, 1980). Any condition that leads to stasis or obstruction of bile in the common bile duct (CBD), including benign or malignant stricture, parasitic infection, or extrinsic compression by the pancreas, can result in bacterial infection and cholangitis (Lam et al., 1978; Boey & Way, 1980). Choledocholithiasis is the most common cause of acute cholang...

conclusions in middle-aged subjects with psc, we found a high rate of non-vertebral fractures and a moderately decreased bmd in lumbar spine and hip. fragility fractures had an impact on physical and mental aspects of hrqol. results in 32% of patients, bmd measured in the hip or spine was below 1.0 standard deviation. a history of fragility fractures (distal forearm and ribs) was reported in si...

conclusions a comparison of our results with those obtained from other centers in both western and asian countries (which reported cca in 3.6% - 36.5% of patients with psc), shows that the incidence of cca in the patients we studied is intermediate. results of the 181 patients, 16 were found to have cca, two of whom had been diagnosed after pathologic study of the explanted livers. therefore it...

Chronic cholestasis often results in premature death from liver failure with fibrosis; however, the molecular mechanisms contributing to biliary cirrhosis are not demonstrated. In this article, we show that the death signal mediated by TNF-related apoptosis-inducing ligand (TRAIL) receptor 2/death receptor 5 (DR5) may be a key regulator of cholestatic liver injury. Agonistic anti-DR5 monoclonal...

The clinical features of 61 patients with sclerosing cholangitis were reviewed. This group included 23 patients with biliary tract calculi, commonly considered as excluding the diagnosis of primary sclerosing cholangitis. The aim of this study was to compare these 23 patients (group A) with 38 patients with sclerosing cholangitis free of calculi (group B). Both groups had the following features...

The purpose of this paper is to evaluate factors affecting the outcome of cholangitis after PTBD in jaundiced cancer patients. Twenty nine patients with neoplastic jaundice (male/female ratio 13/16, median age 55 years) with full clinical data, were treated by PTBD and developed cholangitis at a median of 9 days later. Four patients (14%) died of biliary sepsis a median of one month after PTBD ...

BACKGROUND & AIMS The NOD.c3c4 mouse model develops autoimmune biliary disease characterized by spontaneous granulomatous cholangitis, antimitochondrial antibodies and liver failure. This model for primary biliary cirrhosis (PBC) has evidence of biliary infection with mouse mammary tumour virus (MMTV), suggesting that the virus may have a role in cholangitis development and progression of liver...

Background/Aims Treatment for cholangitis without common bile duct (CBD) stones has not been established in patients with gallstones. We investigated the usefulness of endoscopic biliary drainage (EBD) without endoscopic sphincterotomy (EST) in patients diagnosed with gallstones and cholangitis without CBD stones by endoscopic retrograde cholangiopancreatography (ERCP) and intraductal ultrasono...

Background: Although bacterial cholangitis is frequently mentioned as a cause of secondary sclerosing cholangitis, it appears to be extremely rare, with only one documented case ever reported. Case presentation: A 48-year-old woman presented with an episode of acute biliary pancreatitis that was complicated by pancreatic abcess formation. After 3 months she had an episode of severe pyogenic (E....