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;
Origin ID : 1249656206;
Automatic exact mappings (from CISMeF team)
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.