Purpose: To determine exercise response during cardiopulmonary exercise testing in children and adolescents with dystrophinopathies. Methods: Exercise response on the cardiopulmonary exercise test (CPET) was compared with a standard care test protocol. Results: Nine boys (aged 10.8 ± 4.7 years) with Becker muscular dystrophy (n = 6) and Duchenne muscular dystrophy (n = 3) were included. The feasibility of the CPET was similar to a standard care test protocol, and no serious adverse events occurred. In 67% of the subjects with normal or only mildly impaired functional capacity, the CPET could be used to detect moderate to severe cardiopulmonary exercise limitations. Conclusions: The CPET seems to be a promising outcome measure for cardiopulmonary exercise limitations in youth with mild functional limitations. Further research with larger samples is warranted to confirm current findings and investigate the additional value of the CPET to longitudinal follow-up of cardiomyopathy and the development of safe exercise programs for youth with dystrophinopathies.
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In this thesis we analyzed clinimetric measurement properties of physical fitness tests in wheelchair-using youth with SB. Furthermore, the amount of physical behavior in wheelchair-using youth with SB was quantified and associations with age, gender, VO2peak and Hoffer classification were evaluated. Finally, we described the factors associated with physical behavior in youth with SB and youth with physical disabilities, after which the evidence of interventions to improve physical behavior in youth with physical disabilities was analyzed. This last chapter presents the theoretical and clinical implications. At the end, methodological considerations and directions for further research will be discussed after which the overall conclusion is presented.
