Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies
Bibliographic Data
Objective : The objective of this study was to analyze respiratory muscle function in children and adolescents with cystic fibrosis (CF) treated with Elexacaftor/Tezacaftor/Ivacaftor (ETI) compared to healthy individuals, based on the hypothesis that CFTR modulators may improve respiratory muscle strength. Methods : A descriptive, observational, cross-sectional study was conducted with patients with CF treated with ETI aged 6-18 years. Lung function, maximal expiratory and inspiratory pressures (MIP and MEP), diet quality (KIDMED), and physical activity levels (PAQ) were assessed. The student's t -test or the Mann-Whitney U-test was used to compare differences between groups. The effect size was calculated with Cohen's d. Significance level was set as a p -value Results : A total of 48 children and adolescents (60.4% male) were analyzed in this study (24 healthy and 24 with CF). The participants with CF had mild pulmonary involvement. No significant differences were found in respiratory muscle strength between groups (MEP max p = 0.440, MIP max p = 0.206). Patients with CF showed lower KIDMED ( p = 0.022) and PAQ ( p = 0.010) scores. However, the MIP and MEP values observed in CF participants were higher than those reported in previous studies conducted before the introduction of ETI modulators. Conclusions : Children and adolescents with CF treated with ETI showed respiratory muscle strength comparable to that of healthy controls. Despite differences in lifestyle factors, these findings may reflect a positive impact of CFTR modulators on respiratory muscle function, although further longitudinal and controlled studies are needed
Cystic fibrosis · Cystic fibrosis transmembrane conductance regulator · Ivacaftor · Lung · Lung function · Observational study · Physical therapy · Pulmonary function testing · Respiratory system · Cystic Fibrosis Research Advances · Medicine · Pediatric health and respiratory diseases · Tracheal and airway disorders · Gastroenterology · Internal Medicine
Multi-ethnic reference values for spirometry for the 3–95-yr age range
The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) Statement
Interpretative strategies for lung function tests
Standardization of Spirometry 2019 Update. An Official American Thoracic Society and European Respiratory Society Technical Statement
Food, youth and the Mediterranean diet in Spain. Development of Kidmed, Mediterranean Diet Quality Index in children and adolescents
| Citation velocity | historical |
|---|---|
| Highly cited | No |