Familial intrahepatic cholestasis (FIC) comprises a group of rare cholestatic liver diseases associated with canalicular transport defects resulting predominantly from mutations in ATP8B1, ABCB11 and ABCB4. Phenotypes range from benign recurrent intrahepatic cholestasis (BRIC), associated with recurrent cholestatic attacks, to progressive FIC (PFIC). Patients often suffer from severe pruritus a...

EXPERIMENTAL evaluation of ampicillin has proved that in animals this drug behaves like penicillin G in being rapidly excreted in high concentrations in the bile after oral, intramuscular and intravenous administration (Table I). The ratio of bile to blood (or serum) concentration varies with the species of animal, route of administration, dose, time and technique of sampling. However these res...

We herein report a rare case of microscopic polyangiitis with primary biliary cirrhosis (PBC) and a literature review of six previously reported cases of PBC complicated by anti-neutrophil cytoplasmic antibody-associated vasculitis. Due to the scarcity of similar reports, it was not possible to establish a true overlap syndrome or casual association. When the biliary enzyme levels are elevated ...

BACKGROUND Intraoperative assessment of biliary tract anatomy is relevant for a number of benign and malignant hepatobiliary diseases. During biliary reconstruction, drainage of all relevant bile ducts is imperative to prevent atrophy of undrained segment, cholangitis and secondary biliary cirrhosis. Intraoperative cholangiography, though widely used for intraoperative imaging of the biliary tr...

Biliary innate immunity is involved in the pathogenesis of cholangiopathies in patients with various biliary diseases. Biliary epithelial cells possess an innate immune system consisting of the Toll-like receptor (TLR) family and recognize pathogen-associated molecular patterns (PAMPs). Recently, regulatory mechanisms by intracellular negative regulators including peroxisome proliferator-activa...

INTRODUCTION Magnetic resonance cholangio-pancreatography (MRCP) is a valuable method for the evaluation of biliary and pancreatic diseases and a valuable alternative to endoscopic retrograde cholangiopancreatography (ERCP). It is noninvasive and does not require the use of contrast material or ionizing radiation. Since its introduction in 1991, this technique has significantly improved in spat...

BACKGROUND Immunoglobulin G4 (IgG4)-associated diseases mostly involve the pancreaticobiliary tree and pancreatic parenchyma. This disease complex is characterized by marked response to corticosteroid therapy and response to steroids is incorporated in the diagnostic algorithm of IgG4 associated diseases. However, there is much unknown about the sequences and duration of healing during the cort...

BACKGROUND Cholangiocarcinomas (CC) as well as gallbladder cancers are relatively rare and intractable diseases. Clinical, pathological, and epidemiological studies on these tumors have been under investigation. The current status and/or topics on biliary tract cancers have been reported in the East West Association of Liver Tumor (EWALT), held in Milano, Italy in 2015. SUMMARY All the author...

The incidence of autoimmune diseases appears to be increasing in virtually every population. This is well illustrated by primary biliary cirrhosis, a disease characterized by an immune response to small bile ducts and an incidence increasing with age. The etiology of primary biliary cirrhosis remains enigmatic, although there is clearly an interplay of genetics and environment with a possible r...