Andersen 2017.
| Methods | Parallel‐group RCT | |
| Participants |
Sample size: 6 adults with FSHD1 randomised to intervention group, 7 adults with FSHD1 randomised to control group Inclusion criteria Age 18‐70 years and genetically verified FSHD1 Exclusion criteria Inability to cycle, regular cardio‐exercise (1 h/week), or factors that potentially could confound the results (pregnancy, breastfeeding, disabilities other than FSHD1, participation in other studies) Baseline demographics Mean age participants control group: 46 years, mean age participants training group: 53 years. FSHD clinical score participants control group: 7.5, FSHD clinical score participants control group: 6.3. Female/male ratio control group: 1/5, female/male ratio training group: 2/4 |
|
| Interventions | High‐intensity aerobic exercise training vs no training Type of training and exercise Cycling exercises on an ergometer Intensity Each min of HIT was performed at 3 different work intensities: 30 s of easy pedaling, 20 s of hard work and 10 s of all‐out, maximal intensity. Frequency 3 times/week Setting At the Copenhagen Neuromuscular Center (Denmark) and at home Duration Session: 21 min including an 8‐min standardised warm‐up and two sets of 5‐min HIT separated by a 3‐min break at very low intensity. Programme: 8 weeks Supervision 1 weekly session was performed in the clinic. All participants received live training instructions and a recorded training guide for home use. |
|
| Outcomes |
Primary Fitness as VO2 max and during an exhaustion test on a cycle ergometer Secondary Maximal workload (Wmax), meters walked in a 6‐min walk test, 5‐times sit‐to‐stand‐test, static muscle strength of hip and knee flexion and extension Time‐points measured Before and after 8 weeks of control or training period |
|
| Dates | Study dates not reported | |
| Funding/ declarations of interest | Study authors declare that they have no competing interests "We thank Aase and Einar Danielsens Foundation, Augustinus Foundation, and AP Moeller Foundation for financial support." |
|
| Notes | ‐ | |
| Risk of bias | ||
| Bias | Authors' judgement | Support for judgement |
| Random sequence generation (selection bias) | High risk | Quote: "According to the zip‐code, patients living close to our clinic were randomised to supervised HIT." |
| Allocation concealment (selection bias) | High risk | Quote: "According to the zip‐code, patients living close to our clinic were randomised to supervised HIT." |
| Blinding of participants and personnel (performance bias) All outcomes | High risk | Comment: neither participants nor investigators were blinded to the exercise intervention. |
| Blinding of outcome assessment (detection bias) All outcomes | High risk | Comment: neither participants nor investigators were blinded to the exercise intervention. |
| Incomplete outcome data (attrition bias) All outcomes | High risk | Quote: "12 of the 13 patients completed the controlled part of the study." Comment: analysis was not done by ITT |
| Selective reporting (reporting bias) | Low risk | Comment: no evidence found for selective reporting |
| Other bias | Low risk | Comment: no risk of bias from other sources detected |
1RM: one repetition maximum; ADL: activities of daily living; AMPS: Assessment of Motor and Process Skills; BMI: body‐mass index; CIS: Checklist Individual Strength; CK: creatine kinase; COPM: Canadian Occupational Performance Measure; DM: dermatomyositis; DM1: myotonic dystrophy type 1; DMD: Duchenne muscular dystrophy; FEV: forced expiratory volume; FI: functional index; FSHD: facioscapulohumeral muscular dystrophy; FSS: fatigue severity scale; FVC: forced vital capacity; HIT: high‐intensity training; ITT: intention‐to‐treat; MAP: maximal aerobic power; MFM: motor function measure; MIRS: muscular impairment rating scale; MM: mitochondrial myopathy; MRC: Medical Research Council; MVC: maximum voluntary contractions; MVIC: maximum voluntary isometric strength; N: Newton; NHP: Nottingham Health Profile; PM: polymyositis; PEDI: Pediatric Evaluation of Disability Inventory; RCT: randomised controlled trial; RM: repetition maximum; ROM: range of motion; SD: standard deviation; SF‐36: short form health survey; VAS: visual analogue scale; VO2 max: maximal oxygen uptake