Inspiratory Muscle Strength and Endurance in Children and Adolescents with Cystic Fibrosis.
BACKGROUND
Pulmonary changes that occur in cystic fibrosis may influence inspiratory muscle strength and endurance. We evaluated inspiratory muscle strength and endurance in children and adolescents with cystic fibrosis in comparison with healthy subjects.


METHODS
This is a cross-sectional observational study with subjects with cystic fibrosis and paired healthy individuals, age 6-18 y. Spirometry, impulse oscillometry, plethysmography, manovacuometry, and a protocol of inspiratory muscle endurance were performed.


RESULTS
Subjects with cystic fibrosis (n = 34) had higher maximum percent-of-predicted inspiratory pressure (PImax) than healthy (n = 68) subjects (118.5 ± 25.8% vs 105.8 ± 18.0%) and no significant difference in endurance (60.9 ± 13.3% vs 65.3 ± 12.3%). When restricting the analysis to subjects without Pseudomonas aeruginosa colonization and with FEV1 > 80%, PImax values were significantly higher, and inspiratory muscle endurance was lower, in comparison with the control group. PImax correlated significantly with FVC (r = 0.44, P = .02) and FEV1 (r = 0.41, P = .02), whereas endurance correlated better with total airway resistance (r = 0.35, P = .045) and with central airway resistance (r = 0.48, P = .004).


CONCLUSIONS
Children and adolescents with cystic fibrosis with no colonization by P. aeruginosa and normal lung function present increased inspiratory muscle strength and decreased endurance compared with healthy individuals, indicating that changes in the respiratory muscle function seem to be distinctly associated with pulmonary involvement. Strength was related to pulmonary function parameters, whereas endurance was associated with airway resistance.