Alagille syndrome
Alagille syndrome
Definition
Alagille (AGS) 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.
Also known as Alagille syndrome, Alagille-Watson syndrome, Arteriohepatic dysplasia, syndromic bile duct paucity — per MONDO