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Impaired Aerobic Function in Patients with Cystic Fibrosis during Ramp Exercise


Medicine & Science in Sports & Exercise: December 2014 - Volume 46 - Issue 12 - p 2271–2278
doi: 10.1249/MSS.0000000000000369
Applied Sciences

Purpose: This study aimed to document the matching of muscle O2 delivery to O2 use in young patients with cystic fibrosis (CF) from muscle deoxygenation (HHb) dynamics during ramp exercise.

Methods: Ten patients with stable, mild-to-moderate CF (12.7 ± 2.8 yr) and 10 healthy controls (CON, 12.8 ± 2.8 yr) completed a combined ramp and supramaximal cycling test to determine maximal O2 uptake (V˙O2max). Changes in gas exchange and ventilation, HR, and m. vastus lateralis HHb (near-infrared spectroscopy) were assessed. Δ[HHb]-work rate and Δ[HHb]-V˙O2 profiles were normalized and fit using a sigmoid function.

Results: Aerobic function was impaired in CF, indicated by very likely reduced fat-free mass-normalized V˙O2max (mean difference, ±90% confidence interval: −7.9 mL·kg−1·min−1, ±6.1), very likely lower V˙O2 gain (−1.44 mL·min−1·W−1, ±1.12), and a likely slower V˙O2 mean response time (11 s, ±13). An unclear effect was found upon the absolute and relative work rate (−14 W, ±44, and −0.7% peak power output, ±12.0, respectively) and the absolute and percentage (−0.10 L·min−1, ±0.43, and 3.3% V˙O2max, ±6.0) V˙O2 corresponding to 50% Δ[HHb] amplitude, respectively, between groups. However, arterial oxygen saturation (SpO2) was very likely lower in CF (−1%, ±1) and demonstrated moderate-to–very large relations with parameters of aerobic function.

Conclusions: Young patients with mild-to-moderate CF present with impaired aerobic function during ramp incremental cycling exercise. Because the rate of fractional O2 extraction during ramp cycling exercise was not altered by CF, yet SpO2 was lower, the present findings support the notion of centrally mediated oxygen delivery to principally limit the aerobic function of pediatric patients with CF during ramp incremental cycling exercise.

1Children’s Health and Exercise Research Centre, Sport and Health Sciences, University of Exeter, Exeter, Devon, UNITED KINGDOM; and 2Royal Devon and Exeter NHS Foundation Trust Hospital, Exeter, Devon, UNITED KINGDOM

Address for correspondence: Craig Anthony Williams, Ph.D., Children’s Health and Exercise Research Centre, Sport and Health Sciences, University of Exeter, St. Luke’s Campus, Heavitree Road, Exeter EX1 2LU, United Kingdom; E-mail:

Submitted for publication February 2014.

Accepted for publication April 2014.

© 2014 American College of Sports Medicine