Epidemiology and burden of progressive familial intrahepatic cholestasis: a systematic review

Abstract Background Progressive familial intrahepatic cholestasis is a rare, heterogeneous group of liver disorders of autosomal recessive inheritance, characterised by an early onset of cholestasis with pruritus and malabsorption, which rapidly progresses, eventually culminating in liver failure. F...

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Bibliographic Details
Main Authors: Tracey Jones-Hughes, Jo Campbell, Louise Crathorne
Format: Article
Language:English
Published: BMC 2021-06-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Online Access:https://doi.org/10.1186/s13023-021-01884-4