Thrombocytopenia in cirrhotic patients is usually attributed to splenic pooling whereas in idiopathic thrombocytopenic purpura it is related to platelet bound immunoglobulin (PA-IgG). Since primary biliary cirrhosis (PBC) is an autoimmune disorder we have undertaken a prospective study to assess the frequency and possible relationship of PA-IgG to thrombocytopenia in this condition. Sixty-two p...

BACKGROUND ABCB4/MDR3 gene variants are mostly associated with a peculiar form of cholelithiasis in European adults, currently referred to as low phospholipid-associated cholelithiasis (LPAC) syndrome. METHODS LPAC syndrome is a rare genetic disorder, characterised by the following clinical features: biliary symptoms before the age of 40, recurrence of the symptoms after cholecystectomy, and ...

conclusion the involved liver with hht typically shows vascular shunting and biliary diseases. also, arteriovenous shunts may be vulnerable to biliary diseases. results (i) three types of shunting were found in the livers, including arteriovenous (hepatic artery to hepatic vein) in 6 cases, arterioportal (hepatic artery to portal vein) in 2 cases, and portal venous (portal vein to hepatic vein)...

background: our knowledge regarding the epidemiology of pancreatobiliary cancers in iran is limited. this study presents the first population-based report on pancreatobiliary cancers performed in five provinces of iran. materials andmethods: data used in this study were retrieved from population-based cancer registries in five provinces of iran (gilan, mazandaran, golestan, ardabil and kerman) ...

postcholecystectomy clip migration is rare and can lead to complications such as clip-related biliary stones. most of such incidents have been reported as case reports. this study reviews a case of postcholecystectomy clip migration. it can occur at any time but typically occur at a median of 2 years after cholecystectomy. clinical presentations are similar to those with primary or secondary ch...

introduction: early differentiation of biliary atresia from neonatal hepatitis is of utmost importance, since on time surgery of biliary atresia significantly improves the outcome. hepatobiliary scintigraphy is an integral part of diagnosis work-up of these patients; however its specificity for diagnosis of biliary atresia is suboptimal. in this study we evaluated the value of ursodeoxycholic a...

BACKGROUND The paucity of controlled data for the treatment of most biliary sphincter of Oddi disorder (SOD) types and the incomplete response to therapy seen in clinical practice and several trials has generated controversy as to the best course of management of these patients. In this observational study we aimed to assess the outcome of patients with biliary SOD managed without sphincter of ...

Wilson disease (WD) was a once progressive and uniformly fatal inherited disorder of copper metabolism. Medical therapy to arrest progression or prevent complications of WD was developed in the 1950s with the introduction of parenterally administered BAL [1], and over the next two decades by oral therapy with d-penicillamine, trientine, and zinc [2]. Effective therapy for WD requires life-long ...

North American Indian Childhood Cirrhosis (NAIC) is a rare, autosomal recessive, progressive cholestatic disease of infancy affecting the Cree-Ojibway first Nations of Quebec. All NAIC patients are homozygous for a missense mutation (R565W) in CIRH1A, the human homolog of the yeast nucleolar protein Utp4. Utp4 is part of the t-Utp subcomplex of the small subunit (SSU) processome, a ribonucleopr...