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Benign recurrent intrahepatic cholestasis

Disease definition

Benign recurrent intrahepatic cholestasis (BRIC) is a hereditary liver disorder characterized by intermittent episodes of intrahepatic cholestasis, generally without progression to chronic liver damage. BRIC is now believed to belong to a clinical spectrum of intrahepatic cholestatic disorders that ranges from the mild intermittent attacks in BRIC to the severe, chronic and progressive cholestasis seen in progressive familial intrahepatic cholestasis (PFIC; see this term).

ORPHA:65682

Classification level: Disorder
  • Synonym(s):
    • BRIC
    • Summerskill-Walshe-Tygstrup syndrome
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant or Autosomal recessive 
  • Age of onset: All ages
  • ICD-10: K83.1
  • OMIM: 243300  605479
  • UMLS: C0149841
  • MeSH: -
  • GARD: 12185
  • MedDRA: -
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