" /> Alagille syndrome - CISMeF





Preferred Label : Alagille syndrome;

ICD-11 definition : Alagille syndrome is variably characterized by chronic cholestasis due to paucity of intrahepatic bile ducts, peripheral pulmonary artery stenosis, vertebrae segmentation anomalies, characteristic facies, posterior embryotoxon/anterior segment abnormalities, pigmentary retinopathy, and dysplastic kidneys.;

ICD-11 synonym : Alagille-Watson syndrome; Arteriohepatic dysplasia; Syndromic bile duct paucity;

ICD-11 inclusion : Alagille syndrome type 2; Alagille syndrome type 1;

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Alagille syndrome is variably characterized by chronic cholestasis due to paucity of intrahepatic bile ducts, peripheral pulmonary artery stenosis, vertebrae segmentation anomalies, characteristic facies, posterior embryotoxon/anterior segment abnormalities, pigmentary retinopathy, and dysplastic kidneys.

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20/05/2024


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