Biliary atresia (BA) is the end result of a destructive, rhosis. Until recently, BA was uniformly fatal; liver inflammatory process that affects intra-and extrahetransplantation has altered the inevitability of that patic bile ducts, leading to fibrosis and obliteration of outcome, but at a ''cost'' to the patient, who must face the biliary tract with the development of biliary cirrhothe consequences of living with a transplanted organ, sis. It is the commonest cause of chronic cholestasis in and to society in real dollars. Our understanding of the infants and children, and therefore is the most frequent etiology and pathogenesis of BA has remained virtually indication for liver transplantation in this age group. unchanged for two decades. This disorder is of interest The disease occurs worldwide, affecting an estimated 1 to all individuals involved in basic and clinical studies in 8,000 to 12,000 live births. At present, there is no speof diseases of the liver; the rapidly progressive ficific therapy for BA; however, sequential surgical therbroobliterative process may represent a paradigm for apy begins with creation of a hepatoportoenterostomy (HPE); in those with end-stage liver disease, liver trans-other forms of hepatobiliary injury, perhaps reflecting plantation is indicated. Since most candidates are young an inter-relationship between genetic predisposition children of small size, there is a shortage of size-matched and environmental exposure. donors for liver transplantation. At present, an in-In September 1994, a symposium, sponsored by the creased awareness to ensure early diagnosis and devel-National Digestive Diseases Advisory Board, focused opment of methods to prevent progressive fibrosis are on BA to address the pathogenesis and the clinical chalneeded. These considerations are dependent on detailed lenges presented by this disorder, including the need studies of the pathogenesis of BA. Recent studies have for rapid and precise diagnosis and improved managefocused on normal and altered bile duct morphogenesis ment. The ultimate goal was to stimulate basic investiand the role of various factors (infectious or toxic agents gation of this enigmatic disease, addressing specific isand metabolic insults) in isolation or in combination with a genetic or immunologic susceptibility in the etiol-sues, such as: 1) the genetic, virologic, or immunologic