J Med Assoc Thai 2000; 83 (4):451

Views: 1,467 | Downloads: 22 | Responses: 0

PDF XML Respond to this article Print Alert & updates Request permissions Email to a friend


A Case Report : Alagille Syndrome
Ukarapol N Mail, Wongsawasdi L , Sittiwangkul R

Alagille 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.
Key word : Cholestasis, Arteriohepatic Dysplasia, Alagille

Download: PDF