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Kim, Koh, Chung, Oh, Park, and Kim: A Case of Alagille Syndrome with Atresia of the Hepatic Duct
Case Report

Korean Journal of Pediatric Gastroenterology and Nutrition 2008; 11(1): 65-69.

Published online: 31 March 2008

DOI: https://doi.org/10.5223/kjpgn.2008.11.1.65

A Case of Alagille Syndrome with Atresia of the Hepatic Duct

Hyo Sun Kim, Hong Koh, Ki Sup Chung, Jung Tak Oh*, Young Nyun Park†, Myeung Jun Kim‡

Department of Pediatrics, Yonsei UniversityCollege of Medicine, Severance Children's Hospital, Seoul, Korea.

*Department of Surgery, Yonsei UniversityCollege of Medicine, Severance Children's Hospital, Seoul, Korea.

†Department of Pathology, Yonsei UniversityCollege of Medicine, Severance Children's Hospital, Seoul, Korea.

‡Department of Radiology, Yonsei UniversityCollege of Medicine, Severance Children's Hospital, Seoul, Korea.

kschung58@yuhs.ac

Copyright © 2008 The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition

Abstract

A two-month-old baby had acholic stool, neonatal hyperbilirubinemia and congenital heart disease. Atresia of the hepatic duct was confirmed by open cholangiography, which showed a non-opacified intrahepatic bile duct. Liver biopsy and the Kasai operation were performed. Because the liver biopsy pathology revealed a paucity of intrahepatic bile ducts, the patient was diagnosed with the Alagille syndrome. We report the case of an infant diagnosed with the Alagille syndrome with atresia of the hepatic duct.

Keywords: Neonatal cholestasis, Alagille syndrome, Intrahepatic bile duct atresia

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