BACKGROUND Abernethy malformation is a rare congenital anomaly characterised by the partial or complete absence of the portal vein and the subsequent development of an extrahepatic portosystemic shunt. Caroli's disease is a rare congenital condition characterised by non-obstructive saccular intrahepatic bile duct dilation. Caroli's disease combined with congenital hepatic fibrosis and/or renal ...

aim : in this study, to clarify the smad4blocking impact on fibrosis process, we investigated its down-regulation by shrna on activated human lx-2 cell, in vitro.   background : liver fibrosis is a critical consequence of chronic damage to the liver that can progress toward advanced diseases, liver cirrhosis and hepatocellular carcinoma (hcc).  different smad proteins play as major mediators in...

Caroli's disease is characterized by multifocal segmental dilatation of the intrahepatic bile ducts. It is a rare congenital condition, which appears to be autosomal recessively inherited in most cases. There are two forms of disease, one associated with congenital hepatic fibrosis and a simple form occurring alone. Recent reports suggest that the simple form may be as common as that with conge...

A 1-year-old girl had pancreaticobiliary maljunction, a choledochal cyst, and polycystic kidney. At the age of 4 years, she was treated by resection of the choledochal cyst and Roux-en-Y reconstruction because of the cyst's risk of cancer. She was diagnosed as having congenital hepatic fibrosis based on the histological findings. Postoperatively, she suffered recurrent fever of unknown origin, ...

background hepatic fibrosis is a typical complication of chronic liver diseases resulting in cirrhosis that remains a major public health problem worldwide. liver biopsy is currently the gold standard for diagnosing and staging hepatic fibrosis. percutaneous liver biopsy; however, is an invasive procedure with risks of complications. therefore, there is need for alternative non-invasive techniq...

Congenital hepatic fibrosis (CHF) is a rare autosomal recessive disease that primarily affects the hepatobiliary and renal systems. It is characterized by hepatic fibrosis, portal hypertension, and renal cystic disease. Firm or hard hepatomegaly is present nearly in all patients, often with a prominent left lobe, and this is usually one of the presenting signs. The haematological manifestations...

OBJECTIVE To report clinical presentation, perioperative outcome, and long-term results of surgical management of congenital intrahepatic bile duct (IHBD) dilatations (including Caroli disease) in a multi-institutional setting. SUMMARY BACKGROUND DATA Congenital IHBD dilatations are a rare congenital disorder predisposing to intrahepatic stones, cholangitis, and cholangiocarcinoma. The manage...

background: the renin-angiotensin system has an important role in hepatic inflammation and fibrosis. renin-angiotensin system blockade by angiotensinconverting enzyme (ace) inhibitors provides some protective effects against hepatic fibrogenesis. captopril as an ace inhibitor can decrease inflammatory mediators and attenuate hepatic fibrosis in the livers of bile duct ligated (bdl) rats. object...

The necropsy findings in a 5-year-old girl with congenital hepatic fibrosis are described. She had been followed since the age of 18 months and finally developed hepatocellular failure. Extensive haemorrhagic necrosis was found at necropsy and was probably related to the terminal illness. Other unusual findings were areas of typical post-necrotic cirrhosis and marked centrilobular and diffuse e...