A Case of Alagille Syndrome with Atresia of the Hepatic Duct.
- Author:
Hyo Sun KIM
1
;
Hong KOH
;
Ki Sup CHUNG
;
Jung Tak OH
;
Young Nyun PARK
;
Myeung Jun KIM
Author Information
1. Department of Pediatrics, Yonsei UniversityCollege of Medicine, Severance Children's Hospital, Seoul, Korea. kschung58@yuhs.ac
- Publication Type:Case Report
- Keywords:
Neonatal cholestasis;
Alagille syndrome;
Intrahepatic bile duct atresia
- MeSH:
Alagille Syndrome;
Bile Ducts, Intrahepatic;
Biopsy;
Cholangiography;
Heart Diseases;
Hepatic Duct, Common;
Humans;
Hyperbilirubinemia, Neonatal;
Infant;
Liver
- From:Korean Journal of Pediatric Gastroenterology and Nutrition
2008;11(1):65-69
- CountryRepublic of Korea
- Language:Korean
-
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.
