Journal List > Korean J Pediatr Gastroenterol Nutr > v.11(1) > 1110141

Korean J Pediatr Gastroenterol Nutr. 2008 Mar;11(1):65-69. Korean.
Published online Mar 31, 2008.  https://doi.org/10.5223/kjpgn.2008.11.1.65
Copyright © 2008 The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition
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, and 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.
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