Cholestasis in infancy and childhood.

Many disorders are capable of producing cholestasis in infancy. Primary hepatobiliary diseases and systemic infectious, toxic, and metabolic insults may present clinically as conjugated hyperbilirubinemia. A careful, organized diagnostic evaluation allows early identification of potentially treatable lesions. Recent success in the surgical management of biliary atresia, previously a uniformly fatal disorder, has emphasized the need for early diagnosis. Medical management of the complications of chronic cholestasis remains a major challenge. Liver transplantation currently offers the only chance for long-term survival for infants with progressive cirrhosis.