Long-Term Cholic Acid Treatment in a Patient with Zellweger Spectrum Disorder

Zellweger spectrum disorders (ZSDs) are a subgroup of peroxisomal biogenesis disorders with a generalized defect in peroxisome function. Liver disease in ZSDs has been associated with the lack of peroxisomal β-oxidation of C27-bile acid intermediates to form primary C24-bile acids, which prevents no...

Full description

Bibliographic Details
Main Authors: James E. Heubi, Warren P. Bishop
Format: Article
Language:English
Published: Karger Publishers 2018-11-01
Series:Case Reports in Gastroenterology
Subjects:
Online Access:https://www.karger.com/Article/FullText/494555