A Retrospective Study of Biliary Atresia Patients up to 2 Years of Age

The Biliary Atresia Research Consortium (BARC) is an NIH-funded network of 9 clinical sites and a data coordinating center whose goal is to study biliary atresia, neonatal hepatitis and other cholestatic diseases in the infant. BARC is preparing a prospective observational study of infants with cholestatic disease and clinical trials related to modalities of treatment.

Biliary atresia is the leading cause of morbidity and mortality from pediatric liver disease, and is the most common indication for pediatric liver transplantation. Despite its clinical importance, little is known of a wide range of fundamental issues regarding biliary atresia and optimal approaches to the medical management are unknown. No prospective randomized trials of important aspects of the care of children with biliary atresia have been performed. The majority of published data regarding biliary atresia is anecdotal and based upon a single center experience. Comprehensive registries of the management and outcome of biliary atresia do not exist.

This retrospective study will be unique and powerful because it analyzes the results of a large cohort of children in the United States from a diverse group of institutions with expertise in the management of children with liver disease. The proposed retrospective study may provide novel information and may lead to insights that can be used in the design of a subsequent 5 year prospective study.