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

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 secreti...

Full description

Saved in:
Bibliographic Details
Main Authors: Yasmin Polak, Laura van Dussen, E. Marleen Kemper, Frédéric M. Vaz, Femke C. C. Klouwer, Marc Engelen, Carla E. M. Hollak
Format: Article
Language:English
Published: BMC 2024-12-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Bile acid synthesis defects
Cholic acid
Toxic bile acid intermediates
Single enzyme defects
Zellweger spectrum disorders
Online Access:https://doi.org/10.1186/s13023-024-03449-7
Tags: Add Tag
No Tags, Be the first to tag this record!

Internet

https://doi.org/10.1186/s13023-024-03449-7

Similar Items