Early detection of hepatobiliary involvement in cystic fibrosis: Biomarkers, radiologic methods, and genetic influences.

Autor: Sankararaman S; Division of Pediatric Gastroenterology, Hepatology & Nutrition, UH Rainbow Babies & Children's Hospital, Cleveland, Ohio, USA.; Case Western Reserve University SOM, Cleveland, Ohio, USA., Freeman AJ; Division of Pediatric Gastroenterology, Hepatology & Nutrition, The Ohio State University College of Medicine, Columbus, Ohio, USA.; Nationwide Children's Hospital, Columbus, Ohio, USA.
Jazyk: angličtina
Zdroj: Pediatric pulmonology [Pediatr Pulmonol] 2024 Sep; Vol. 59 Suppl 1, pp. S107-S114.
DOI: 10.1002/ppul.26892
Abstrakt: Cystic fibrosis-related hepatobiliary involvement (CFHBI) is a term used to describe a spectrum of hepatobiliary involvement ranging from a transient 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. After respiratory/cardiorespiratory issues and transplant-related complications, aCFLD is now the 4th leading cause of mortality among PwCF. Additionally, aCFLD 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 the 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.
(© 2024 Wiley Periodicals LLC.)
Databáze: MEDLINE
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