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Alagille syndrome

What is Alagille syndrome?

A rare syndrome 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.

Other condition names

  • Alagille-Watson syndrome
  • Arteriohepatic dysplasia
  • Syndromic bile duct paucity

Inheritance type

Autosomal dominant

Age of Onset

  • All ages
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.