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KMID : 0816120020050020192
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Korean Journal of Pediatric Gastroenterolology and Nutrition
2002 Volume.5 No. 2 p.192 ~ p.198
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A Case of Alagille Syndrome
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Kim Eun-Soo
Lee Dong-Woo
Chung Ki-Sup
Kim Soon-Il
Park Young-Nyun
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Abstract
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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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KEYWORD
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Alagille syndrome, Paucity of interlobular bile duct, Liver transplantation, Children
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