BACKGROUND Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease and one of the most common indications for liver transplantation in adults. There are conflicting data regarding characteristic findings of PSC disease on endoscopic retrograde cholangiography (ERCP). We undertook this study to clarify whether there is a specific pattern of involvement of the biliary tract in...

One hundred and four adult patients with cystic fibrosis were evaluated for the presence of liver disease as defined by abnormal liver function tests of six months' duration, histological evidence of fibrosis or cirrhosis, or the presence of portal hypertension, or both. Twenty patients fulfilled these criteria and were evaluated further for the presence of biliary tract abnormalities with bili...

Autosomal recessive polycystic kidney disease (ARPKD) is a rare but devastating inherited disease in humans. Various strains of mice that are homozygous for the cpk gene display renal pathology similar to that seen in human ARPKD. The PKD progresses to renal insufficiency, azotemia, and ultimately a uremic death by approximately 3 wk of age. This study characterizes PKD in mice that are homozyg...

The bile duct system is a pathway of bile transportation from the liver to the intestine and plays a role of exocrine function of the liver. It consists of two different types of epithelial cells, hepatocytes and cholangiocytes. Anatomically as well as developmentally, the bile duct could be divided into intrahepatic bile duct (IHBD) and extrahepatic bile duct (EHBD; extrahepatic hepatic duct, ...

A 71-year-old-man was admitted to our hospital with a fever over 39°C. He had undergone pancreaticoduodenectomy for cholangiocarcinoma and suffered from recurrent cholangitis for a year after the surgery. Although laboratory data showed an elevated white blood cell count (13,100/mL) and C-reactive protein (6.53 mg/dL), no marked elevation was noted in the hepatobiliary enzymes. However, abdomin...

Biliary complications after living donor liver transplantation (LDLT) continue to be problematic. For reducing the biliary complications, the authors applied an intrahepatic Glissonian approach to the recipient hepatectomy. We called this Glissonian dissection technique at the high hilar level high hilar dissection (HHD). In this study, we introduced this HHD technique and evaluated its outcome...

During liver development, hepatoblasts differentiate into hepatocytes or biliary epithelial cells (BEC). The BEC delineate the intrahepatic and extrahepatic bile ducts, and the gallbladder. The transcription factors that control the development of the biliary tract are unknown. Previous work has shown that the onecut transcription factor HNF6 is expressed in hepatoblasts and in the gallbladder ...

The biliary tree consists of intrahepatic and extrahepatic bile ducts and is lined by biliary epithelial cells (or cholangiocytes). There are also peribiliary glands around the intrahepatic large bile ducts and extrahepatic bile ducts. The biliary tree is a conduit of bile secreted by hepatocytes and biliary epithelial cells and also of the peribiliary glands and has several physiological roles...

When cholestatic liver disease is present, liver ultrasound should be performed to ascertain if cholestasis is extrahepatic or intrahepatic. If bile ducts appear dilated and the probability of interventional treatment is high, endoscopic retrograde cholagio-pancreatography (ERCP) or trans-hepatic cholangiography (THC) should be the next step. If the probability of interventional therapeutics is...