A Case Report : Alagille Syndrome

NUTHAPONG UKARAPOL, M.D.*, LUMDUAN WONGSAWASDI,M.D.*, REKWAN SITTIWANGKUL, M.D.*

Affiliation : *Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand.

AbstractAlagille syndrome consists of 5 major features comprising paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects and peripheral pulmonic stenosis. The female patient in this report met 4 of the 5 major features except ocular abnormality. The first clinical presentations were prolonged jaundice and generalized ecchymoses. She was treated by plasma replacement and vitamin supplement, particularly vitamin K I, which produced clinical improvement. This report also reviews the literature of Alagille syndrome.

Keywords : Cholestasis, Arteriohepatic Dysplasia, Alagille

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