There have been improved insights into the diagnosis, pathophysiology, and treatment of pediatric hepatobiliary disorders. As the longevity of patients with cystic fibrosis has increased, hepatobiliary complications have become more significant. Newer diagnostic methods and treatment modalities are discussed. Insights into the genetics, pathophysiology, and treatment of progressive familial intrahepatic cholestasis (Byler's syndrome) are presented. Parenteral nutrition-associated cholestasis continues to be a significant problem for the neonate. Potential causes and treatment modalities are discussed.
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