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Доброякісний рецидивуючий внутрішньопечінковий холестаз

ORPHA:65682· ICD-10 K83.1· Benign recurrent intrahepatic cholestasis

Визначення(English summary)

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).

Поширеність
Unknown
Успадкування
Autosomal dominant, Autosomal recessive
Вік початку
All ages