BILIARY ATRESIA STUDY IN INFANTS AND CHILDREN (BASIC)

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This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The Biliary Atresia Research Consortium (BARC) is an NIH-funded network of ten clinical sites and a data coordinating center whose goal is to study biliary atresia, neonatal hepatitis and other cholestatic diseases in the infant. Biliary atresia (BA) is a progressive necroinflammatory process initially involving the extrahepatic biliary tree. As the disease progresses, there is loss of patency of the lumen and obstruction to bile flow. The result is cholestasis and chronic liver damage. With time, the intrahepatic biliary system becomes involved. BA occurs in one in 8-15,000 live births resulting in 250-400 new cases per year in the US. Untreated, the disease leads to complete biliary obstruction with cirrhosis, and is uniformly fatal. After a hepatoportoenterostomy (Kasai procedure), children have a variable disease progression with less than 20% surviving beyond the teen years without liver transplantation. Little is known about the factors that cause biliary atresia nor the factors that influence disease progression. A variety of genetic, autoimmune and environmental influences have been hypothesized to be important. Most studies to date have focused on the neonate and young child with BA, yet the older surviving child with BA can provide important information about genetics, as well as, natural history. This project proposes to develop a database that contains appropriate clinical information, genetic material and body fluid samples obtained from children and young adults with BA to address hypotheses aimed to identify genetic and clinical influences on etiology and outcome.