Biliary atresia (BA), a progressive chronic cholangiopathy affecting 1/8 000-1/17 000 live births, is the most common cause of chronic liver disease (CLD) in children and a considerable burden for public health resources.
Biliary atresia is an inflammatory cholangiopathy of infancy that results in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts.
Histologic examination of surgically excised bile duct remnants and postmortem specimens supports the contention that, in most cases, the observed fibro-obliterative cholangiopathy in biliary atresia results from destruction of a presumably well-formed biliary system rather than from primary failure of normal embryologic development of these structures.
Patients with human immunodeficiency virus were excluded secondary to possibly confounding data caused by the possibilities of human immunodeficiency virus cholangiopathy, idiopathic pancreatitis, and hepatitis.
Three patterns of intrahepatic variceal bleeding were also demonstrated: first group (0-24 months)--missed biliary atresia; second group (25-60 months)--mainly attributable to poorly functioning or failed portoenterostomy; third group (5-9 years)--could be explained as a critical point where intrahepatic cholangiopathy effect is maximal and overrides biliary drainage offered by portoenterostomy, and where LT should be considered in children with previously 'successful' portoenterostomy.