Korean J Pediatr Gastroenterol Nutr.
2002 Sep;5(2):192-198.
A Case of Alagille Syndrome
- Affiliations
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- 1Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea. kschung58@yumc.yonsei.ac.kr
- 2Department of Surgery, Yonsei University College of Medicine, Seoul, Korea.
- 3Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
Abstract
- Alagille syndrome is characterized by paucity of interlobular bile ducts, chronic cholestasis, characteristic facial abnormalities, cardiovascular abnormalities, posterior embryotoxon, vertebral arch defects, skeletal abnormalities, and glomerular renal involvement. We experienced a case of Alagille syndrome in a 10 month-old male presenting with jaundice. He had chronic cholestasis, characteristic face, cardiovascular abnormalities (aortic stenosis, dextrocardia, double chamber of left ventricle), and situs inversus. Histological examination of liver biopsy specimen revealed paucity of interlobular bile ducts with septal fibrosis, cirrhotic transformation and severe cholestasis. He underwent liver transplantation, but died of cardiopulmonary arrest associated with cardiac anomaly.
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