Systematic Review: The Epidemiology, Natural History, and Burden of Alagille Syndrome.

ABSTRACT:

Background and aim

Alagille syndrome (ALGS) is an inherited multisystem disorder typically manifesting as cholestasis, and potentially leading to end-stage liver disease and death. The aim of the study was to perform the first systematic review of the epidemiology, natural history, and burden of ALGS with a focus on the liver component.

Methods

Electronic databases and proceedings from key congresses were searched in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2009 guidelines. This analysis included publications reporting epidemiology, natural history, economic burden or health-related quality of life (HRQoL) outcomes in patients with ALGS.

Results

Of 525 screened publications, 20 met the inclusion criteria. Liver-relate

SUBMITTER: Kamath BM 

PROVIDER: S-EPMC6110620 | biostudies-literature | 2018 Aug

REPOSITORIES: biostudies-literature