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Rarely, patients require liver transplant and typically the patient outcomes are excellent. With improved understanding of liver injury mechanisms, new strategies for treatment are now being explored, including siRNA technology, molecules to modulate secretion, and enhancers of proteolysis.The observation of bile duct paucity is an important diagnostic finding in children, occurring in roughly 11% of pediatric liver biopsies. Alagille syndrome (ALGS) is a well-defined syndromic form of intrahepatic bile duct paucity that is accompanied b