Pathogenesis and treatment opportunities for biliary atresia

Abstract

Biliary atresia is the single most common cause of chronic cholestasis in children and is the leading indication for liver transplantation worldwide. It results from an inflammatory and fibrosing obstruction of the extrahepatic bile ducts in the first few months of life. Early diagnosis and timely surgical portoenterostomy are necessary for improved biliary drainage, but the liver disease progresses to end-stage biliary cirrhosis in most patients. Although the pathogenesis of disease is largely unknown, recent patient- and animal-based experiments indicate interactions between infectious agents and inflammatory circuits may be important pathogenic mechanisms of disease.