The clinical and biochemical effectiveness and safety of cholic acid treatment for bile acid synthesis defects: a systematic review.

ABSTRACT:

Background

Bile acid synthesis defects (BASDs) can be severely disabling involving the liver and nervous system, potentially due to elevated levels of toxic C₂₇-bile acid intermediates. Cholic acid (CA) supplementation is hypothesized to decrease bile acid production, stimulate bile secretion and -flow, and slowing down disease progression. This systematic review assesses the clinical and biochemical effectiveness, and safety of CA in BASDs patients.

Methods

A systematic review of MEDLINE, Embase and clinical trial registries (ClinicalTrials.gov, ICTRP registry) using controlled MeSH- and Emtree terms.

Results

From 526 articles 70 publications were deemed eligible for inclusion based on title and abstract. 14 publications were included after full-text asses

SUBMITTER: Polak Y 

PROVIDER: S-EPMC11657003 | biostudies-literature | 2024 Dec

REPOSITORIES: biostudies-literature