Risk factors for more rapid progression of severe liver fibrosis in children with cystic fibrosis-related liver disease: A multi-center study validated by liver biopsy.

Autor: Sakhuja S; Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA.; Division of Pediatric Gastroenterology, Hepatology, Nutrition, Texas Children's Hospital, Houston, Texas, USA., Staples HM; Department of Pediatrics, University of South Carolina School of Medicine, Columbia, South Carolina, USA.; Division of Pediatric Pulmonology, Prisma Health Children's Hospital-Midlands, Columbia, South Carolina, USA., Minard CG; Institute for Clinical and Translational Research, Baylor College of Medicine, Houston, Texas, USA., Ramm LE; Hepatic Fibrosis Group, QIMR Berghofer Medical Research Institute, Brisbane, Queensland, Australia., Lewindon PJ; Department of Gastroenterology, Queensland Children's Hospital, Brisbane, Queensland, Australia.; Faculty of Medicine, The University of Queensland, Brisbane, Queensland, Australia., Ramm GA; Hepatic Fibrosis Group, QIMR Berghofer Medical Research Institute, Brisbane, Queensland, Australia.; Faculty of Medicine, The University of Queensland, Brisbane, Queensland, Australia., Leung DH; Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA.; Division of Pediatric Gastroenterology, Hepatology, Nutrition, Texas Children's Hospital, Houston, Texas, USA.
Jazyk: angličtina
Zdroj: Liver international : official journal of the International Association for the Study of the Liver [Liver Int] 2023 Jun; Vol. 43 (6), pp. 1277-1286. Date of Electronic Publication: 2023 Apr 10.
DOI: 10.1111/liv.15572
Abstrakt: Background and Aims: Early identification of risk factors for the development of severe fibrosis in children with cystic fibrosis-related liver disease (CFLD) is crucial as promising therapies emerge.
Methods: This multi-center cohort study of children with a priori defined CFLD from 1999 to 2016, was designed to evaluate the clinical utility of CF-specific characteristics and liver biomarkers assessed years prior to liver biopsy-proven CFLD to predict risk of developing severe fibrosis (F3-4) over time. Fibrosis was staged by Metavir classification.
Results: The overall study cohort of 42 patients (F0-2 (n = 22) and F3-4 (n = 20)) was 57% male (n = 24) with median age of 7.6 years at baseline visit versus 10.3 years at biopsy. Median FEV 1 % predicted was lower in F3-4 participants at baseline versus F0-2 (59% vs. 85%; p = .002), while baseline FIB-4, APRI and GGT were higher in F3-4. Median splits for FIB-4 (≥.13), APRI (≥.36), GPR (≥.09), GGT (≥25.5), and FEV 1 % (<64%) were associated with more rapid progression to F3-4 (p < .01 for all). Using a combination of change/year in FIB-4, APRI, and GPR to predict F3-4, the AUROC was .81 (95% CI, .66, .96; p < .0001). For up to 5.8 years prior, thresholds for GPR were met 6.5-fold more rapidly, and those for APRI and FIB-4 were met 2.5-fold more rapidly, in those who progressed to F3-4 than those that did not.
Conclusions: This study suggests mild-moderate pulmonary dysfunction and higher liver biomarker indices at baseline may be associated with faster progression of CFLD.
(© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
Databáze: MEDLINE
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