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Alagille syndrome

Disease 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.

ORPHA:52

  • Synonym(s):
    • Alagille-Watson syndrome
    • Arteriohepatic dysplasia
    • Syndromic bile duct paucity
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: All ages
  • ICD-10: Q44.7
  • OMIM: 118450  610205
  • UMLS: C0085280
  • MeSH: D016738
  • GARD: 804
  • MedDRA: 10053870

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