Korean J Pediatr Gastroenterol Nutr.
2008 Mar;11(1):65-69.
A Case of Alagille Syndrome with Atresia of the Hepatic Duct
- Affiliations
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- 1Department of Pediatrics, Yonsei UniversityCollege of Medicine, Severance Children's Hospital, Seoul, Korea. kschung58@yuhs.ac
- 2Department of Surgery, Yonsei UniversityCollege of Medicine, Severance Children's Hospital, Seoul, Korea.
- 3Department of Pathology, Yonsei UniversityCollege of Medicine, Severance Children's Hospital, Seoul, Korea.
- 4Department 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.
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