BILIARY ATRESIA - CURRENT CONCEPTS AND RESEARCH DIRECTIONS - SUMMARY OF A SYMPOSIUM

Biliary atresia (BA) is the end result of a destructive, inflammatory process that affects intra- and extrahepatic bile duets, leading to fi brosis and obliteration of the biliary tract with the development of b iliary cirrhosis. It is the commonest cause of chronic cholestasis in infants and children, and therefore is the most frequent indication fo r li ver transplantation in this age group. The disease occurs worldwi de, affecting an estimated 1 in 8,000 to 12,000 live births. At presen t, there is no specific therapy for BA; however, sequential surgical t herapy begins with creation of a hepatoportoenterostomy (HPE); in thos e with end-stage liver disease, liver transplantation is indicated. Si nce most candidates are young children of small size, there is a short age of size-matched donors for liver transplantation. At present, an i ncreased awareness to ensure early diagnosis and development of method s to prevent progressive fibrosis are needed. These considerations are dependent on detailed studies of the pathogenesis of BA Recent studie s have focused on normal and altered bite duct morphogenesis and the r ole of various factors (infectious or toxic agents and metabolic insul ts) in isolation or in combination with a genetic or immunologic susce ptibility in the etiology of BA.