Biomarkers for liver disease in urea cycle disorders.

ABSTRACT:

Background

Urea cycle disorders (UCDs) are among the most common inborn errors of liver metabolism. As therapies for hyperammonemia associated with urea cycle dysfunction have improved, chronic complications, such as liver disease, have become increasingly apparent in individuals with UCDs. Liver disease in UCDs may be associated with hepatic inflammation, hepatic fibrosis, portal hypertension, liver cancer and even liver failure. However, except for monitoring serum aminotransferases, there are no clear guidelines for screening and/or monitoring individuals with UCDs for liver disease. Thus, we systematically evaluated the potential utility of several non-invasive biomarkers for liver fibrosis in UCDs.

Methods

We evaluated grey-scale ultrasonography, liver stiffness obtaine

SUBMITTER: Nagamani SCS 

PROVIDER: S-EPMC8195846 | biostudies-literature | 2021 Jun

REPOSITORIES: biostudies-literature