All issues > Volume 28(10); 1985

Case Report

J Korean Pediatr Soc. 1985;28(10):1047-1051. Published online October 31, 1985.

Two Case of Watson-Alagille Syndrome.

Heon Seok Han, Soo Heum Lim, Young Sun Kim, Jeong Kee Seo, Hyung Ro Moon

¹Department of Pediatrics, Seoul National University Hospital, Seoul, Korea

Abstract

Watson-Alagille syndrome (Arteriohepatic dysplasia, Syndromatic paucity of interlobular bile duct), a disease characterized by chronic liver disease, characteristic facies, cardiova scular abnormalities, and vertebral arch abnormalities has paucity of interlobular bile ducts on histologic examination of liver. We experienced two cases of Watson-Alagille syndrome in a 7-month-old male infant and a 12-month-old male infant who had the chief complaint of prolonged jaundice. Both of them^were diagnosed as neonatal hepatitis. During follow-up period, they showed characteristic facies. Vertebral roentgenogram revealed arch anormalies and echocardiogram showed pulmonary valvular stenosis and ventricular septal defect on each case. The histologic examination of liver showed paucity of interlobular bile ducts. A brief review of related literatures was made.

Keywords :Watson-Alagille Syndrome(Arteriohepatic dysplasia, syndromatic paucity of interlobular bile duct)