Long-Term exercise intervention in patients with McArdle disease: clinical and aerobic fitness benefits
Introduction: The long-term effects of exercise in patients with McArdle disease—the paradigm of ‘exercise intolerance’—are unknown. This is an important question as the severity of the disease frequently increases with time. Purpose: To study the effects of a long-term exercise intervention on clin...
| Autores: | , , , , , , , , , , , , |
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| Tipo de recurso: | artículo |
| Fecha de publicación: | 2022 |
| País: | España |
| Institución: | Universidad de Castilla-La Mancha |
| Repositorio: | RUIdeRA. Repositorio Institucional de la UCLM |
| OAI Identifier: | oai:ruidera.uclm.es:10578/29633 |
| Acceso en línea: | http://hdl.handle.net/10578/29633 |
| Access Level: | acceso abierto |
| Palabra clave: | Myophosphorylase Exercise is medicine Glycogenosys type 5 Glycogen storage disease |
| Sumario: | Introduction: The long-term effects of exercise in patients with McArdle disease—the paradigm of ‘exercise intolerance’—are unknown. This is an important question as the severity of the disease frequently increases with time. Purpose: To study the effects of a long-term exercise intervention on clinical and fitness-related outcomes in McArdle patients. Methods: Seventeen patients (exercise group: N=10, 6 male, 38±18yrs; control: N=7, 4 male, 38±18yrs) participated in a twoyear unsupervised intervention including moderate-intensity aerobic (cycle-ergometer exercise for 1h) and resistance (high load-low repetition circuit) training on 5 and 2-3 days/week, respectively. Patients were assessed at baseline and postintervention. Besides safety, outcomes included clinical severity (e.g., exercise intolerance features) on a 0-3 scale (primary outcome), and aerobic fitness, gross muscle efficiency, and body composition (total/regional fat, muscle, and bone mass) (secondary outcomes). Results: The exercise program was safe and resulted in a reduction of one point (-1.0, 95% confidence interval -1.6—-0.5, p=0.025) in clinical severity vs. the control group, with 60% of participants in the exercise group becoming virtually asymptomatic and with no functional limitation in daily life activities. Compared with controls, the intervention induced significant and large benefits (all p<0.05) in the workload eliciting the ventilatory threshold (both in absolute (watts, +37%) and relative units (watts·kg-1 of total body mass or of lower-limb muscle mass, +44%)), peak oxygen uptake (ml·kg-1 ·min-1 , +28%) and peak workload (absolute (+27%) and relative units (+33%)). However, no significant changes were found for muscle efficiency nor for any measure of body composition. Conclusions: A two-year unsupervised intervention including aerobic and resistance exercise is safe and induces major benefits in the clinical course and aerobic fitness of patients with McArdle disease. |
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