The children in this group had the following pre-transplant diagnoses: 37 had biliary atresia, 2 had cirrhosis, 2 had Alpha-1 antitrypsin deficiency, 1 had Alagille syndrome, 1 had glycogen storage disease Type IV, 1 had familial intrahepatic cholestasis, and 1 had cyst sclerosing cholangitis. The gene discussed is SERPINA1; the disease is glycogen storage disease due to glycogen branching enzyme deficiency.