All issues > Volume 41(3); 1998

Case Report

J Korean Pediatr Soc. 1998;41(3):410-414. Published online March 15, 1998.

A Case of Alagille Syndrome

Eun Kyung EK Hwang¹, Gwang Hoon GH Lee¹, Eell E Ryoo¹, Kang Ho KH Cho¹, Gil Hyun GH Kim¹, Hak Soo HS Lee¹, Ji Hye JH Kim¹, Sung Hae SH Park², Hee Sup HS Kim³

¹Deprartment of Pediatrics, Chung-ang Gil Hospital, Inchon, Korea
²Deprartment of Clinical Pathology, Chung-ang Gil H ospital, Inchon, Korea
³Department of Pediatrics, Red Cross Hospital, Seoul, Korea

Correspondence Eun Kyung EK Hwang ,Email: 1

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.

Keywords :Alagille syndrome