Elexacaftor/tezacaftor/ivacaftor and inflammation in children and adolescents with cystic fibrosis: a retrospective dual-center cohort study.

ABSTRACT:

Background

Cystic fibrosis (CF) is characterized by chronic neutrophilic inflammation in the airways. Elexacaftor/tezacaftor/ivacaftor (ETI) therapy has demonstrably improved clinical outcomes and quality of life in people with CF (pwCF), but its effects on systemic inflammatory parameters remain unclear.

Objective

To evaluate the impact of ETI on systemic inflammation in children and adolescents with CF.

Design

Retrospective, dual-center observational, propensity score-matching study of pediatric pwCF on ETI.

Methods

PwCF aged ⩽ 18 years treated with ETI at two Italian reference centers were included in this study. Data on immunoglobulins (Ig) (A, G, and M), γ-globulin, leukocyte levels, percent predicted forced expiratory volume in the first second (ppFEV1),

SUBMITTER: Pepe A 

PROVIDER: S-EPMC11811973 | biostudies-literature | 2025 Jan-Dec

REPOSITORIES: biostudies-literature