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Case ReportOpen Access
A Case Report : Alagille Syndrome
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
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
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