Aerobic training in patients with anoctamin 5 myopathy and hyperckemia
Research output: Contribution to journal › Journal article › Research › peer-review
INTRODUCTION: Anoctamin 5 deficiency has recently been defined to cause limb-girdle muscular dystrophy type 2L (LGMD2L) with pronounced hyperCKemia. No treatment interventions have been made so far in this condition.
METHODS: In 6 patients with LGMD2L, we studied the effect of home-based, pulse-watch monitored, moderate-intensity exercise on a cycle ergometer for 30 minutes, 3 times weekly, for 10 weeks. Plasma creatine kinase (CK) was assessed before, during, and after the program as a marker of muscle damage. Primary outcome measures were maximum oxygen uptake (VO(2max)) and time in the 5-repetitions-sit-to-stand test (FRSTST).
RESULTS: Training resulted in improvements in VO(2max) (27 ± 7%; P = 0.0001) and FRSTST time (35 ± 12%; P = 0.007). Improvements in physiologic and functional muscle testing were accompanied by stable CK levels and no reports of adverse effects.
CONCLUSIONS: These findings suggest that supervised aerobic exercise training is safe and effective in improving oxidative capacity and muscle function in patients with anoctamin 5 deficiency.
Original language | English |
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Journal | Muscle & Nerve |
Volume | 50 |
Issue number | 1 |
Pages (from-to) | 119-123 |
Number of pages | 5 |
ISSN | 0148-639X |
DOIs | |
Publication status | Published - Jul 2014 |
- Adult, Anaerobic Threshold, Chloride Channels, Creatine Kinase, Exercise, Exercise Test, Female, Heart Rate, Humans, Male, Middle Aged, Mobility Limitation, Muscular Dystrophies, Limb-Girdle, Mutation, Neurologic Examination, Patient Compliance, Physical Education and Training, Treatment Outcome
Research areas
ID: 138776047