Year 2023 / Volume 115 / Number 6
Original
Cystic fibrosis with liver involvement in adults has a benign course. Results from a tertiary referral center cohort

301-305

DOI: 10.17235/reed.2022.9289/2022

Almudena Marinero Martínez-Lázaro, Rosa María Girón Moreno, Fernando Casals Seoane, Óscar Cano-Valderrama, Luisa García-Buey,

Abstract
Background: Cystic Fibrosis Liver Disease is a poorly understood entity, especially in adults, in terms of its real prevalence, natural history and diagnostic criteria, despite being the most important extrapulmonary cause of mortality. The aim was to evaluate the prevalence, characteristics and potential risk factors of liver disease in adults with cystic fibrosis, according to two diagnostic criteria accepted in the scientific literature. Methods: Patients were recruited in a tertiary referral hospital, and laboratory, ultrasound, non-invasive liver fibrosis tests (AST to Platelet Ratio Index; Fibrosis-4 Index) and transient elastography (Fibroscan) were performed. The proportion of patients with liver disease according to the Debray and Koh criteria were evaluated. Results: 95 patients were included, 48 (50.5%) females, with a mean age of 30.4 (28.6-32.2) years. According to the Debray criteria, 6 (6.3%) patients presented liver disease. According to the Koh criteria, prevalence increased up to 8.4%, being statistically different from the 25% value described in other published series (p = 0.005). Seven (7.5%) presented ultrasonographic chronic liver disease. Eleven (13%) presented liver fibrosis according to the APRI score; 95 (100%) had a normal FIB-4 value. Mean liver stiffness value was 4.4 (4.1-4.7) kPa. FEV1 (OR=0.16, p 0.05), meconium ileus (OR=14.16, p 0.002), platelets (Pearson coefficient -0.25, p 0.05) and younger age (Pearson coefficient -0.19, p 0.05) were risk factors. Conclusions: Prevalence and severity of liver disease in adult cystic fibrosis patients were lower than expected. Meconium ileus, platelets, age and respiratory function were confirmed as risk factors associated to cystic fibrosis liver disease.
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Marinero Martínez-Lázaro A, Girón Moreno R, Casals Seoane F, Cano-Valderrama Ó, García-Buey L. Cystic fibrosis with liver involvement in adults has a benign course. Results from a tertiary referral center cohort. 9289/2022


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Publication history

Received: 18/10/2022

Accepted: 27/10/2022

Online First: 10/11/2022

Published: 06/06/2023

Article Online First time: 23 days

Article editing time: 231 days


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