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 complica
tions have become more significant. Newer diagnostic methods and treat
ment modalities are discussed. Insights into the genetics, pathophysio
logy, and treatment of progressive familial intrahepatic cholestasis (
Byler's syndrome) are presented. Parenteral nutrition-associated chole
stasis continues to be a significant problem for the neonate. Potentia
l causes and treatment modalities are discussed.