title: Aerobic Training in Patients with Congenital Myopathy.
abstract: BACKGROUND
  Congenital myopathies (CM) often affect contractile proteins of the sarcomere, which could render patients susceptible to exercise-induced muscle damage. We investigated if exercise is safe and beneficial in patients with CM.
  
  
  METHODS
  Patients exercised on a stationary bike for 30 minutes, three times weekly, for 10 weeks at 70% of their maximal oxygen uptake (VO2max). Creatine kinase (CK) was monitored as a marker of muscle damage. VO2max, functional tests, and questionnaires evaluated efficacy.
  
  
  RESULTS
  Sixteen patients with CM were included in a controlled study. VO2max increased by 14% (range, 6-25%; 95% CI 7-20; p < 0.001) in the seven patients who completed training, and tended to decrease in a non-intervention group (n = 7; change -3.5%; range, -11-3%, p = 0.083). CK levels were normal and remained stable during training. Baseline Fatigue Severity Scale scores were high, 4.9 (SE 1.9), and tended to decrease (to 4.4 (SE 1.7); p = 0.08) with training. Nine patients dropped out of the training program. Fatigue was the major single reason.
  
  
  CONCLUSIONS
  Ten weeks of endurance training is safe and improves fitness in patients with congenital myopathies. The training did not cause sarcomeric injury, even though sarcomeric function is affected by the genetic abnormalities in most patients with CM. Severe fatigue, which characterizes patients with CM, is a limiting factor for initiating training in CM, but tends to improve in those who train.
  
  
  BACKGROUND
  The Regional Committee on Health Research Ethics of the Capital Region of Denmark H-2-2013-066 and ClinicalTrials.gov H2-2013-066.
authors.affiliation: Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Gitte
authors.initials: G
authors.lastname: Hedermann
authors.affiliation: Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Christoffer Rasmus
authors.initials: CR
authors.lastname: Vissing
authors.affiliation: Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Karen
authors.initials: K
authors.lastname: Heje
authors.affiliation: Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Nicolai
authors.initials: N
authors.lastname: Preisler
authors.affiliation: Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Nanna
authors.initials: N
authors.lastname: Witting
authors.affiliation: Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
authors.forename: John
authors.initials: J
authors.lastname: Vissing
mesh_terms: D000328:Adult; D003402:Creatine Kinase / Q000097:blood; D003710:Demography; D015444:Exercise; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D053580:Muscle Strength / Q000502:physiology; D009135:Muscular Diseases / Q000097:blood / Q000151:congenital* / Q000503:physiopathology*; D010084:Oxidation-Reduction; D010101:Oxygen Consumption / Q000502:physiology; D010349:Patient Compliance; D010352:Patient Dropouts; D011795:Surveys and Questionnaires; D014797:Vital Capacity / Q000502:physiology; D055815:Young Adult
chemical_list: D003402:Creatine Kinase