A longitudinal assessment of non-invasive biomarkers to diagnose and predict cystic fibrosis-associated liver disease

https://doi.org/10.1016/j.jcf.2020.05.002Get rights and content
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Highlights

  • Certain non-invasive biomarkers can predict cystic fibrosis-associated liver disease (CFLD) with high sensitivity and specificity.

  • Gamma-glutamyl transferase to platelet ratio (GPR), gamma-glutamyl transferase (GGT), AST to Platelet Ratio Index (APRI), platelet count, and AST/ALT ratio were effective biomarkers.

  • Platelet count continuously decreased over time for patients eventually diagnosed with CFLD.

  • Cystic fibrosis-associated pulmonary disease was not distinguishable from “healthy” CF patients using APRI.

Abstract

Background & Aims

A practical, inexpensive, and non-invasive biomarker of liver fibrosis is needed as a reliable screening test for cystic fibrosis-associated liver disease (CFLD). Studies have shown the utility of AST to Platelet Ratio Index (APRI), fibrosis index based on 4 factors (FIB-4), and gamma-glutamyl transferase (GGT) as good biomarkers for identifying CFLD. The goal of the study was to evaluate the effectiveness of APRI, FIB-4, AST/ALT ratio, platelet count, GGT, and GGT platelet ratio (GPR) in predicting CFLD development.

Methods

Data was collected from CF Foundation Patient Registry for patients aged 3–21 years at Johns Hopkins from January 1, 2002 to December 31, 2014. Collected data included demographic characteristics, presence of splenomegaly, hepatomegaly, ascites, and variceal bleeding, AST, ALT, GGT, platelet count, and FEV1. The sensitivity and specificity of each biomarker were analyzed and reported by the area under receiver operating characteristic (AUROC) curve.

Results

By the end of the study, 144 “healthy” CF, 12 CFLD, 19 CF-associated pulmonary disease (CFPD), and 4 CFLD with CFPD cases were identified. APRI scores were higher in CFLD, 0.85 versus 0.28 in “healthy” CF and 0.23 in CFPD groups (p<0.001). GPR had the highest AUROC curve at 0.91.

Conclusions

GPR, GGT, APRI score, and platelet count were potentially useful biomarkers while FIB-4 did not predict CFLD development. Cost-effectiveness studies are needed to analyze the utility of these biomarkers in clinical practice.

Keywords

Liver cirrhosis
Blood chemical analysis
Transferases
Respiratory tract infections
Blood platelets

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