Aerobic training in patients with anoctamin 5 myopathy and hyperckemia

Research output: Contribution to journal › Journal article › Research › peer-review

Standard

Aerobic training in patients with anoctamin 5 myopathy and hyperckemia. / Vissing, Christoffer R; Preisler, Nicolai; Husu, Edith; Prahm, Kira P; Vissing, John.

In: Muscle & Nerve, Vol. 50, No. 1, 07.2014, p. 119-123.

Research output: Contribution to journal › Journal article › Research › peer-review

Harvard

Vissing, CR, Preisler, N, Husu, E, Prahm, KP & Vissing, J 2014, 'Aerobic training in patients with anoctamin 5 myopathy and hyperckemia', Muscle & Nerve, vol. 50, no. 1, pp. 119-123. https://doi.org/10.1002/mus.24112

APA

Vissing, C. R., Preisler, N., Husu, E., Prahm, K. P., & Vissing, J. (2014). Aerobic training in patients with anoctamin 5 myopathy and hyperckemia. Muscle & Nerve, 50(1), 119-123. https://doi.org/10.1002/mus.24112

Vancouver

Vissing CR, Preisler N, Husu E, Prahm KP, Vissing J. Aerobic training in patients with anoctamin 5 myopathy and hyperckemia. Muscle & Nerve. 2014 Jul;50(1):119-123. https://doi.org/10.1002/mus.24112

Author

Vissing, Christoffer R ; Preisler, Nicolai ; Husu, Edith ; Prahm, Kira P ; Vissing, John. / Aerobic training in patients with anoctamin 5 myopathy and hyperckemia. In: Muscle & Nerve. 2014 ; Vol. 50, No. 1. pp. 119-123.

Bibtex

@article{b2a5b9b8d1294a2194573da33f272a17,
title = "Aerobic training in patients with anoctamin 5 myopathy and hyperckemia",
abstract = "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.",
keywords = "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",
author = "Vissing, {Christoffer R} and Nicolai Preisler and Edith Husu and Prahm, {Kira P} and John Vissing",
note = "Copyright {\textcopyright} 2013 Wiley Periodicals, Inc.",
year = "2014",
month = jul,
doi = "10.1002/mus.24112",
language = "English",
volume = "50",
pages = "119--123",
journal = "Muscle & Nerve",
issn = "0148-639X",
publisher = "JohnWiley & Sons, Inc.",
number = "1",

}

RIS

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