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. For children and their parents, PFIC is an extremely distressing disease. Significant pruritus can lead to severe cutaneous mutilation and may affect many activities of daily living through loss of sleep, irritability, poor attention, and impaired school performance.

Methods

Databases including MEDLINE and Embase were searched for publications on PFIC prevalence, incidence or natural history, and the economic burden or health-related quality of life of patients with PFIC. Preferred Reportin

SUBMITTER: Jones-Hughes T 

PROVIDER: S-EPMC8173883 | biostudies-literature | 2021 Jun

REPOSITORIES: biostudies-literature