Aerobic training in patients with anoctamin 5 myopathy and hyperckemia
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Aerobic training in patients with anoctamin 5 myopathy and hyperckemia. / Vissing, Christoffer R; Preisler, Nicolai; Husu, Edith; Prahm, Kira P; Vissing, John.
I: Muscle & Nerve, Bind 50, Nr. 1, 07.2014, s. 119-123.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Aerobic training in patients with anoctamin 5 myopathy and hyperckemia
AU - Vissing, Christoffer R
AU - Preisler, Nicolai
AU - Husu, Edith
AU - Prahm, Kira P
AU - Vissing, John
N1 - Copyright © 2013 Wiley Periodicals, Inc.
PY - 2014/7
Y1 - 2014/7
N2 - 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.
AB - 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.
KW - Adult
KW - Anaerobic Threshold
KW - Chloride Channels
KW - Creatine Kinase
KW - Exercise
KW - Exercise Test
KW - Female
KW - Heart Rate
KW - Humans
KW - Male
KW - Middle Aged
KW - Mobility Limitation
KW - Muscular Dystrophies, Limb-Girdle
KW - Mutation
KW - Neurologic Examination
KW - Patient Compliance
KW - Physical Education and Training
KW - Treatment Outcome
U2 - 10.1002/mus.24112
DO - 10.1002/mus.24112
M3 - Journal article
C2 - 24639367
VL - 50
SP - 119
EP - 123
JO - Muscle & Nerve
JF - Muscle & Nerve
SN - 0148-639X
IS - 1
ER -
ID: 138776047