Chronic inflammation of biliary atresia

Biliary atresia (BA) is the most common cholestatic liver disease in children, and is characterized by the progressive destruction and obliteration of the extrahepatic bile ducts and irreversible cholestatic liver injury within months of birth. However, the exact etiology and pathogenesis of BA remain unknown. Despite improved survival with the use of Kasai's procedure, more than half of BA patients develop cirrhosis necessitating liver transplantation. Infectious pathogens-particularly viruses-have long been suggested as the causative agents. However, none have been universally isolated or identified in BA cases. Here, we summarize the recent findings on the underlying immunological and inflammatory characteristics of this disease.