Abstract
Introduction: Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is expressed in human skeletal muscle cells and CFTR dysfunction may present an important determinant of aerobic exercise capacity in CF. Previous studies on the relationship between CFTR genotype and aerobic exercise capacity are scarce and contradictory. Aims and objectives: This study was designed to explore factors influencing aerobic exercise capacity, expressed as peak oxygen consumption (VO 2peak , primary outcome measure) with a specific focus on CFTR genotype in children and adults with CF. Methods: In an international, multicenter cross-sectional study we collected data on CFTR genotype and cardiopulmonary exercise tests (CPET) in patients with CF age 8 years and older. CFTR mutations were classified into functional classes I-V. Results: 513 patients (45% females) from 10 CF centers in North America and Europe had both valid maximal CPET and complete CFTR genotype data and were included in the analysis. Overall, patients had reduced VO 2peak (mean±SD, 81.5 ± 19.2% predicted), but values were comparable among different CFTR classes. Using multilevel mixed-effects models adjusted for study center and relevant confounders, lung function and body mass index were the main predictors of VO 2peak , independent of CFTR genotype. Conclusions: Lung disease severity and reduced nutritional status rather than CFTR genotype are the major determinants of maximal exercise capacity in CF patients.
Published Version
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