Ursodeoxycholic acid for cystic fibrosis-related liver disease.

ABSTRACT:

Background

Abnormal biliary secretion leads to the thickening of bile and the formation of plugs within the bile ducts; the consequent obstruction and abnormal bile flow ultimately results in the development of cystic fibrosis-related liver disease. This condition peaks in adolescence with up to 20% of adolescents with cystic fibrosis developing chronic liver disease. Early changes in the liver may ultimately result in end-stage liver disease with people needing transplantation. One therapeutic option currently used is ursodeoxycholic acid. This is an update of a previous review.

Objectives

To analyse evidence that ursodeoxycholic acid improves indices of liver function, reduces the risk of developing chronic liver disease and improves outcomes in general in cystic fibrosis.

SUBMITTER: Cheng K 

PROVIDER: S-EPMC6483662 | biostudies-literature | 2017 Sep

REPOSITORIES: biostudies-literature