1. Long-Term Exercise Intervention in Patients with McArdle Disease: Clinical and Aerobic Fitness Benefits.
- Author
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SANTALLA, ALFREDO, VALENZUELA, PEDRO L., RODRIGUEZ-LOPEZ, CARLOS, RODRÍGUEZ-GÓMEZ, IRENE, NOGALES-GADEA, GISELA, PINÓS, TOMÀS, ARENAS, JOAQUÍN, MARTÍN, MIGUEL A., SANTOS-LOZANO, ALEJANDRO, MORÁN, MARÍA, FIUZA-LUCES, CARMEN, ARA, IGNACIO, and LUCIA, ALEJANDRO
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RESISTANCE training , *BODY composition , *AEROBIC exercises , *EXERCISE tolerance , *CONFIDENCE intervals , *CLINICAL trials , *OXYGEN consumption , *EXERCISE physiology , *ACTIVITIES of daily living , *PHYSICAL fitness , *TREATMENT effectiveness , *INBORN errors of carbohydrate metabolism , *EXERCISE intensity , *EMPLOYEES' workload , *ERGOMETRY , *EXERCISE therapy , *PATIENT safety - Abstract
Introduction: The long-term effects of exercise in patients with McArdle disease—the paradigm of "exercise intolerance"—are unknown. This is an important question because the severity of the disease frequently increases with time. Purpose: This study aimed 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 ± 18 yr; control: n = 7, 4 male, 38 ± 18 yr) participated in a 2-yr unsupervised intervention including moderate-intensity aerobic (cycle-ergometer exercise for 1 h) and resistance (high load–low repetition circuit) training on 5 and 2–3 d·wk−1, 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 1 point (−1.0; 95% confidence interval, −1.6 to −0.5; P = 0.025) in clinical severity versus 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 per kilogram of total body mass or of lower-limb muscle mass, +44%)), peak oxygen uptake (in milliliters per kilogram per minute, +28%), and peak workload (in absolute (+27%) and relative units (+33%)). However, no significant changes were found for muscle efficiency or for any measure of body composition. Conclusions: A 2-yr 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. [ABSTRACT FROM AUTHOR]
- Published
- 2022
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