Effect of Kasai Procedure on Hepatic Outcome in Alagille Syndrome

• Published in: Journal of pediatric gastroenterology and nutrition Vol. 51; no. 3; pp. 319 - 321
• Main Authors: Kaye, Adam J, Rand, Elizabeth B, Munoz, Pedro S, Spinner, Nancy B, Flake, Alan W, Kamath, Binita M
• Format: Journal Article
• Language: English
• Published: United States Copyright by ESPGHAN and NASPGHAN 01.09.2010
• Subjects: Alagille syndrome; Alagille Syndrome - complications; Alagille Syndrome - mortality; Alagille Syndrome - surgery; Bilirubin - blood; Calcium-Binding Proteins - genetics; Case-Control Studies; gamma-Glutamyltransferase - blood; Genotype; Heart Diseases; Humans; Infant; Intercellular Signaling Peptides and Proteins - genetics; Jagged-1 Protein; Jaundice - etiology; Kasai; Liver - enzymology; Liver - surgery; Liver Transplantation - statistics & numerical data; Membrane Proteins - genetics; Mutation; Phenotype; Portoenterostomy, Hepatic - methods; Portoenterostomy, Hepatic - mortality; Postoperative Complications - mortality; Retrospective Studies; Serrate-Jagged Proteins; transplant; Treatment Outcome

ABSTRACT Objectives: Alagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high γ‐glutamyl transpeptidase (GGT) cholestasis, most notably biliary atresia. As a result infants with AGS may undergo intraoperative cholangiogram and even Kasai procedure. The ai...