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Benign recurrent intrahepatic cholestasis

ORPHA:65682· ICD-10 K83.1

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).

Prevalence
Unknown
Inheritance
Autosomal dominant, Autosomal recessive
Age of onset
All ages