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Medicine General 27dae80c

All are features of Alagille syndrome, EXCEPT:

A
Posterior embryotoxon
B
Hepatocellular jaundice
C
Peripheral pulmonary stenosis
D
Butterfly veebrae
High-Yield Explanation
Syndromic paucity of interlobular bile ducts (Alagille syndrome) is the most common form of familial intrahepatic cholestasis. Chronic cholestasis affects 95% of patients. Peripheral pulmonic stenosis is observed in approximately 90%. Veebral arch defects are seen (e.g., butterfly veebrae, hemiveebrae, and a decrease in the interpedicular distance). Ophthalmologic examination may reveal posterior embryotoxon, retinal pigmentation, and iris strands. Ref: Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 9th Edition, Page 1061

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