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Moderate-intensity aerobic exercise improves physical fitness in bethlem myopathy

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@article{8f8d020a50134262aef0790a7a43188d,
title = "Moderate-intensity aerobic exercise improves physical fitness in bethlem myopathy",
abstract = "INTRODUCTION: Bethlem myopathy is caused by dysfunctional collagen VI assembly, leading to varying degrees of hyperlaxity, contractures and muscle weakness. Previous studies demonstrate that cardiovascular training is safe and beneficial in patients with myopathies. However, exercise exacerbates the dystrophic phenotype in collagen VI-knockout mice.METHODS: Six men with Bethlem myopathy were included (4 training; 2 controls). After training, 2 patients detrained. Patients performed 10 weeks of home-based, moderate-intensity exercise monitored by a pulse-watch. The primary outcome was change in peak oxygen uptake (VO2peak ). Secondary outcomes were performances in functional tests.RESULTS: VO2peak improved in the training group (16{\%}, P = 0.017). Detraining led to regression of VO2peak toward baseline values (-8{\%}; P = 0.03). No change was seen in the control group (-7{\%}; P = 0.47). Performance in functional tests did not change significantly. Creatine kinase values were stable during the study.CONCLUSIONS: Moderate-intensity exercise seems to safely improve oxidative function in patients with Bethlem myopathy. Muscle Nerve 60: 183-188, 2019.",
author = "Vissing, {Christoffer Rasmus} and Gitte Hedermann and John Vissing",
note = "{\circledC} 2019 Wiley Periodicals, Inc.",
year = "2019",
month = "8",
doi = "10.1002/mus.26498",
language = "English",
volume = "60",
pages = "183--188",
journal = "Muscle and Nerve",
issn = "0148-639X",
publisher = "John/Wiley & Sons, Inc. John/Wiley & Sons Ltd",
number = "2",

}

RIS

TY - JOUR

T1 - Moderate-intensity aerobic exercise improves physical fitness in bethlem myopathy

AU - Vissing, Christoffer Rasmus

AU - Hedermann, Gitte

AU - Vissing, John

N1 - © 2019 Wiley Periodicals, Inc.

PY - 2019/8

Y1 - 2019/8

N2 - INTRODUCTION: Bethlem myopathy is caused by dysfunctional collagen VI assembly, leading to varying degrees of hyperlaxity, contractures and muscle weakness. Previous studies demonstrate that cardiovascular training is safe and beneficial in patients with myopathies. However, exercise exacerbates the dystrophic phenotype in collagen VI-knockout mice.METHODS: Six men with Bethlem myopathy were included (4 training; 2 controls). After training, 2 patients detrained. Patients performed 10 weeks of home-based, moderate-intensity exercise monitored by a pulse-watch. The primary outcome was change in peak oxygen uptake (VO2peak ). Secondary outcomes were performances in functional tests.RESULTS: VO2peak improved in the training group (16%, P = 0.017). Detraining led to regression of VO2peak toward baseline values (-8%; P = 0.03). No change was seen in the control group (-7%; P = 0.47). Performance in functional tests did not change significantly. Creatine kinase values were stable during the study.CONCLUSIONS: Moderate-intensity exercise seems to safely improve oxidative function in patients with Bethlem myopathy. Muscle Nerve 60: 183-188, 2019.

AB - INTRODUCTION: Bethlem myopathy is caused by dysfunctional collagen VI assembly, leading to varying degrees of hyperlaxity, contractures and muscle weakness. Previous studies demonstrate that cardiovascular training is safe and beneficial in patients with myopathies. However, exercise exacerbates the dystrophic phenotype in collagen VI-knockout mice.METHODS: Six men with Bethlem myopathy were included (4 training; 2 controls). After training, 2 patients detrained. Patients performed 10 weeks of home-based, moderate-intensity exercise monitored by a pulse-watch. The primary outcome was change in peak oxygen uptake (VO2peak ). Secondary outcomes were performances in functional tests.RESULTS: VO2peak improved in the training group (16%, P = 0.017). Detraining led to regression of VO2peak toward baseline values (-8%; P = 0.03). No change was seen in the control group (-7%; P = 0.47). Performance in functional tests did not change significantly. Creatine kinase values were stable during the study.CONCLUSIONS: Moderate-intensity exercise seems to safely improve oxidative function in patients with Bethlem myopathy. Muscle Nerve 60: 183-188, 2019.

U2 - 10.1002/mus.26498

DO - 10.1002/mus.26498

M3 - Journal article

VL - 60

SP - 183

EP - 188

JO - Muscle and Nerve

JF - Muscle and Nerve

SN - 0148-639X

IS - 2

ER -

ID: 58148971