Alagille syndrome is a genetic disorder that affects the liver, heart,kidney, and other systems of the body. Problems associated with the disorder generally become evident in infancy or early childhood. The disorder is inherited in an autosomal dominant pattern, and the estimated prevalence of Alagille syndrome is 1 in every 100,000 live births.
This child had in addition to biliary atresia also situs inversus incompletus (spleen is on right side and heart remains on left side).
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