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Moderate-intensity aerobic exercise improves physical fitness in bethlem myopathy

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

DOI

  1. Hydroxylated Long-Chain Acylcarnitines are Biomarkers of Mitochondrial Myopathy

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  2. MSTO1 mutations cause mtDNA depletion, manifesting as muscular dystrophy with cerebellar involvement

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  3. Growth and differentiation factor 15 as a biomarker for mitochondrial myopathy

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  4. Fat oxidation is impaired during exercise in lipin-1 deficiency

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

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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.

OriginalsprogEngelsk
TidsskriftMuscle & Nerve
Vol/bind60
Udgave nummer2
Sider (fra-til)183-188
Antal sider6
ISSN0148-639X
DOI
StatusUdgivet - aug. 2019

Bibliografisk note

© 2019 Wiley Periodicals, Inc.

ID: 58148971