Journal of the Korean Pediatric Society  1998;41(3):410-414

A Case of Alagille Syndrome.

Eun Kyung HWANG 1 ; Gwang Hoon LEE ; Eell RYOO ; Kang Ho CHO ; Gil Hyun KIM ; Hak Soo LEE ; Ji Hye KIM ; Sung Hae PARK ; Hee Sup KIM

Affiliations

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Keywords

Alagille syndrome

Country

Republic of Korea

Language

Korean

Abstract

Alagille syndrome is characterized by chronic cholestasis, posterior embryotoxon, skeletal abnormalities, cardiovascular abnormalities, and a typical face with prominent forehead and pointed chin. Its histological feature includes paucity of interlobular bile ducts. We experienced a 49-day-old female infant presenting with frequent upper respiratory tract infection and persistent jaundice. She had a typical face and chronic cholestasis. Echocardiograms revealed peripheral pulmonary stenosis. The histological examination of liver revealed paucity of interlobular bile ducts.