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. 2019 Dec 6;2019(12):CD003907. doi: 10.1002/14651858.CD003907.pub5

Summary of findings 5. Aerobic exercise compared to no training for facioscapulohumeral muscular dystrophy.

Aerobic exercise compared to no training for facioscapulohumeral muscular dystrophy
Patient or population: people with facioscapulohumeral muscular dystrophy
 Setting: at home and in rehabilitation centres
 Intervention: aerobic exercise
 Comparison: without aerobic exercise training
Outcomes Mean (SD) without aerobic exercise Mean (SD) with aerobic exercise Difference (95% CI) Certainty of the evidence
 (GRADE) What happens
Muscle strength: maximum voluntary isometric knee extension strength
Assessed with Quantitative Muscle Assessment fixed myometry testing system
Follow‐up: mean 16 weeks
 52 participants (1 RCT)a
The mean change in maximum voluntary isometric knee extension strength without aerobic exercise was a decrease of 1.8 (1.4) kg The mean change in maximum voluntary isometric knee extension strength with aerobic exercise was a decrease of 1.7 (1.4) kg MD 0.1 kg higher
 (0.7 lower to 0.9 higher) ⊕⊕⊝⊝
 Lowb,c May have little or no effect on quadriceps strength
Aerobic capacity: VO2 peak
Assessed with submaximal cycling test
Follow‐up: mean 16 weeks
 38 participants (1 RCT)a
The mean change in VO2 peak without aerobic exercise was a decrease of 0.4 (0.8) L/min The mean change in VO2 peak with aerobic exercise was an increase of 0.7 (1.3) L/min MD 1.1 L/min higher (0.4 higher to 1.8 higher) ⊕⊕⊝⊝
 Lowb,c May increase aerobic capacity (VO2 peak) slightly
Time‐scored functional assessments of muscle performance: distance walked
Assessed with 6‐minute walk test
Follow‐up: mean 16 weeks
 52 participants (1 RCT)a
The mean change in distance walked in the 6‐min walk test without aerobic exercise was an increase of 0.0 (15.0) m The mean change in distance walked in the 6‐min walk test with aerobic exercise was an increase of 31.0 (27.0) m MD 31.0 higher (19.3 higher to 42.7 higher) ⊕⊕⊝⊝
 Lowb,c May improve distance walked in a 6‐min walk test
Quality of life
Assessed with Sickness Impact Profile
Scale from 0 to 572
Follow‐up: mean 16 weeks
 52 participants (1 RCT)a
The mean change in quality‐of‐life score without aerobic exercise was an increase of 8.0 (19.0) The mean change in quality‐of‐life score with aerobic exercise was a decrease of 2.0 (16.0) MD 10.0 lower (19.6 lower to 0.4 lower) ⊕⊕⊝⊝
 Lowb,c May improve quality of life slightly
Pain
Asssesed with a Visual Analogue Scale
Scale from 0 to 100
Follow‐up: mean 16 weeks
 52 participants (1 RCT)a
The mean change in pain score without aerobic exercise was an increase of 1.0 (2.8) The mean change in pain score with aerobic exercise was an increase of 0.0 (4.5) MD 1.0 lower (3.0 lower to 1.0 higher) ⊕⊕⊝⊝
 Lowb,c May have little or no effect on pain
Experienced fatigue
Assesed with Checklist Individual Strength
Scale from 7 to 56
Follow‐up: mean 16 weeks
 52 participants (1 RCT)a
The mean change in fatigue score without aerobic exercise was a decrease of 1.2 (1.0) The mean change in fatigue score with aerobic exercise was a decrease of 8.5 (2.0) MD 7.3 lower (8.1 lower to 6.5 lower) ⊕⊕⊝⊝
 Lowb,c May improve experienced fatigue
Adverse effects requiring withdrawal
Follow‐up: mean 16 weeks
52 participants (1 RCT)a
There were no adverse events leading to withdrawal
15 participants who had received aerobic exercise training reported 1 to 5 adverse events: 4 participants experienced knee pain, 9 saddle soreness, 7 neck and shoulder pain, and 6 back pain. All these complaints resolved spontaneously during the study period
⊕⊕⊝⊝
 Lowb,c May be no adverse effects requiring withdrawal
CI: confidence interval; MD: mean difference; RCT: randomised controlled trial; SD: standard deviation; VO2 peak: peak oxygen uptake
GRADE Working Group grades of evidenceHigh certainty: we are very confident that the true effect lies close to that of the estimate of the effect.
 Moderate certainty: we are moderately confident in the effect estimate: the true effect is likely to be close to the estimate of the effect, but there is a possibility that it is substantially different.
 Low certainty: our confidence in the effect estimate is limited: the true effect may be substantially different from the estimate of the effect.
 Very low certainty: we have very little confidence in the effect estimate: the true effect is likely to be substantially different from the estimate of effect.

aWe did not include data from two other small studies in this summary. These trials had 13 and 19 participants, respectively. Both were at high risk of bias due to their methods of randomisation, lack of allocation concealment, lack of blinding, and attrition.
 bDowngraded one level for imprecision: the sample size was 52 participants.
 cDowngraded one level for study limitations: blinding of participants and personnel was not possible.