Abstract
The reproducibility of cardiopulmonary exercise testing (CPET) has not been established in young cystic fibrosis (CF) patients using a valid protocol. Thirteen 7-18 year olds completed three CPETs, separated by 48 h and 4-6 weeks. CPET involved a ramp-incremental cycling test with supramaximal verification. Maximal oxygen uptake was repeatedly determined with no learning effect and typical errors expressed as a coefficient of variation (TE(CV%)) of 9.3% (48 h) and 13.3% (4-6 weeks). The reproducibility of additional parameters of aerobic function [gas exchange threshold (TE(CV%): 11.2%, 16.8%); VO2 mean response time (TE(CV%): 37.8%, 89.4%); VO2 gain (TE(CV%): 17.4%, 24.5%)] and clinical utility [e.g. SaO2% (TE(CV%): 2.2%, 3.1%); ventilatory drive (V(E)/VCO2-slope) (TE(CV%): 7.8%, 17.7%)] was also established over the short- and the medium-term, respectively. These results establish limits of variability to determine meaningful changes over the short- and the medium-term for CPET outcomes in young CF patients.
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