loading page

Early Detection of Hepatobiliary Involvement in Cystic Fibrosis: Biomarkers, Elastography and Genetic Influences
  • Senthilkumar Sankararaman,
  • A. Jay Freeman
Senthilkumar Sankararaman
University Hospitals Rainbow Babies & Children's Hospital
Author Profile
A. Jay Freeman
Nationwide Children's Hospital

Corresponding Author:[email protected]

Author Profile


Cystic fibrosis-related hepatobiliary involvement (CFHBI) is a term used to describe a spectrum of hepatobiliary involvement ranging from benign elevation of transaminase levels to advanced cystic fibrosis associated liver disease (aCFLD). While CFHBI is common among people with cystic fibrosis (PwCF), aCFLD is rare impacting only approximately 5-10% of the CF population. Importantly though, aCFLD is the third leading cause of death among PwCF, is an independent predictor of all-cause mortality and is associated with significant morbidity. Despite this recognition, our ability to predict those patients at greatest risk for aCFLD, identify early aCFLD and monitor incremental progression of CFHBI is lacking. Here we review the strengths and weaknesses of the common biomarkers and imaging modalities used in the evaluation and monitoring of CFHBI, as well as the current understanding of genetic modifiers related to aCFLD.
17 Aug 2023Submitted to Pediatric Pulmonology
17 Aug 2023Review(s) Completed, Editorial Evaluation Pending
17 Aug 2023Assigned to Editor
17 Aug 2023Submission Checks Completed
21 Aug 2023Reviewer(s) Assigned
07 Oct 2023Editorial Decision: Revise Minor