Table I.
Characteristics of included studies (n = 12)
| Author, year (reference) Country Study design |
Sample size Total, IG and CG. Mean age (SD) or range And Participants functioning |
Exercise training intervention for the IG Frequency, Intensity, Time, Setting, Duration |
Intervention for the CG (Comparison) Frequency, Intensity, Time, Setting, Duration |
Outcome Measures |
|---|---|---|---|---|
| N = 24 | Arm cycling | UE ROM exercises | Functioning | |
| IG (n = 12) | F:3 days/week | F: 5 days/week | AERA. Standing from | |
| 9.5 (1.38) years IG (n = 12) 9.33 (1.37) years |
I:50% of max difficulty T:40 minutes |
I:5-10 reps depending on individual fatigue T:40 minutes |
supine. T-shirt donning/Removing NSAA |
|
| Alemdaruglo et al. 2015 (24) Turkey | All ambulant, able to sit 1 hour independently, steroid use for more than 6 months | S: Hospital, supervised by PT D: 8 weeks |
S:Home, supervised by family D: 8 weeks |
Quality-of-life NA |
| Strength | ||||
| RCT | Isometric strength by HDD UE. Grip Strength. | |||
| Endurance | ||||
| A6MCT | ||||
|
| ||||
| N = 19 | Gravity compensated | Functioning | ||
| IG (n = 9) 12.9 (2.8) years CG (n = 10) 12.6 (3.4) years |
UE training with use of 3D Sony PlayStation videogame F:5 days/week I:n/a |
Usual care | PUL, MFM ROM Abilhand-plus (questionnaire children/parents) |
|
| Heutinck et al. 2018 (26) Netherland | Ambulatory and wheelchair dependent, able to lift their hands to the head by use of elbow flexion or compensate. 100% in IG and 60% in CG used steroids. | T: 15 minutes S: Home, supervised at start and after 10 weeks, otherwise independently D: 30 weeks |
Quality-of-life Kidscreen 52 Global Health (Children and parents questionnaires) |
|
| RCT | ||||
| Strength Isometric strength by HDD UE. MVC Endurance A6MCT |
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|
| ||||
| N = 14 | Breathing exercises and PT | PT program | Functioning | |
| IG (n = 7) | program | FVC, FEV1, FEF25-75, PERF | ||
| 8.5-14.11 years | F: 5 days/week | F:5 days/week | ||
| CG (n = 7) 8.5-15.6 years |
I: 10 and 18 cm H20 CPAP during 12 deep insp, 4-5 cough cycles, 6 forced expirations | I: n/a T: n/a S: School, supervised by PT. |
Quality-of-life n/a |
|
| Houser et al. 1971 (27) | T: n/a | D: 12 weeks | Strength | |
| USA | All the participants were wheelchair dependent | S: School, supervised by PT. | n/a | |
| D: 12 weeks | Endurance | |||
| CCT | M | |||
|
| ||||
| N = 30 (29 analyzed) | Active assisted UE and LE cycling (KTP kinetic ergometer). | Usual care | Functioning | |
| IG (n = 17) 10.8 (2.4) years |
F: 5 days/week | MFM, ROM PEDI (self-care questionnaire). | ||
| Jansen et al. 2013 (8) Netherland RCT |
CG (n = 13) 10.5 (2.8) years Ambulatory (n = 18) and wheelchair dependent (n = 12). 23 of participants used steroids. All were able to lift both arms to the head, but unable to use wheel chair > 500 meters. |
I:65 revolutions per minute/< 6 OMNI scale) T: 15 minutes legs, 15 minutes arms. S: Home supervised by parents or PT. D: 24 weeks |
Quality-of-life n/a Strength MRC Endurance A6MCT |
|
|
| ||||
| N= 18 (17 analyzed, one died) | Respiratory strength and endurance training by use of circuit respiration device (flow limiting resistance). | Usual care | Functioning | |
| 14.2 (7-20) years IG (n = 9) CG (n = 9) |
F: 5 days/week I: Endurance; |
VC Quality-of-life n/a |
||
| Martin et al. 1986 (28) | Wheelchair dependent (n = 17), ambulatory (n = 1) | Ventilate until exhaustion within 3 minutes, 20% over | Strength | |
| Australia | VC rage in one sequence. | PeMax and | ||
| Strength: Maximal inspiratory/expiratory manoeuvers in 3-5 seconds | PiMax | |||
| RCT cross-over | S: supervised at school D: 8 weeks |
Endurance Pe time and Pi time |
||
|
| ||||
| N = 22 (20 analyzed) | Inspiratory muscle training | Placebo | Functioning | |
| 11.6 (9-14) years | (Triflow II) | Forced expirations (Peak expiratory flow meter). | FVC, FEV1, PERF | |
| F: 5 days/week | ||||
| Radillo et al. 1989 (29) United Kingdom |
n/a | I: 20 inspirations with increased resistance flow T: n/a S: School, supervised by PT D: 18 days |
F: 5 days/week I: 10 expirations T: n/a S: School, supervised by PT D: 18 days |
Quality-of-life n/a Strength PiMax |
| RCT cross-over | Endurance n/a |
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|
| ||||
| N = 18 | LE exercise program and passive stretching. | Series of oral instructed free exercises for the LE and passive stretching. | Functioning | |
| Scott et al. 1989 (30) United Kingdom | 6.9 (1.17) years (5-9) years IG (n = 9) CG (n = 11) All were fully ambulatory, with anticipated compliance to the intervention. |
F: 7 days/week I: Manual resistance (n/a) T: 15 minutes S: Home, supervised by the parents D: 6 months |
F: 7 days/week I: n/a T: 15 minutes S: Home, supervised by the parents D: 6 months |
Locomotor ability, ROM ankle dorsiflexion. Vignos Scale, 8.4 and 45 meter timed test. Quality-of-life n/a |
| RCT | Strength MRC, Myometric and torque force output Endurance n/a |
|||
|
| ||||
| N = 18 (12 analyzed, four died). | Inspiratory muscle training | Usual care | Functioning | |
| 15 (range 10.4 – 23.4) years IG (n = 7) |
with flow resistance to play a video game with visual audio feedback. | FVC (% pred) | ||
| CG (n = 11) Ambulatory (n = 2) and wheelchair dependent (n = 2). | F: 5 days/week I: Exceed a pre-set level of resistance, 6.25, 4.76, 3.18 or 2.38 mm restrictors. |
Quality-of-life n/a |
||
| Stern et al. 1989 (31) Australia | S: School, supervised | Strength PeMax |
||
| D: 6 months IP, 12 months SP. | (% pred), PiMax (% pred) | |||
| RCT cross-over | Endurance | |||
| Endurance (mmHg) | ||||
|
| ||||
| N = 16 | Inspiratory resistive muscle training (Triflow) | Placebo | Functioning | |
| IG (n = 8) | F: 2times/5 days | Inspiratory muscle training (Triflow) | VC, FRC, TLC, FEV1, FEV1/ | |
| 14.7 (4.5) years | I: 30% of PiMax | FVC | ||
| Topin et al. 2002 (32) | CG (n = 8) | T: 10 minutes | F:2times/5 days | |
| France | 12.63 (1.8) years | S: Home, supervised by parents, D: 6 weeks | I: 5% of PiMax | Quality-of-life |
| T: 10 minutes | n/a | |||
| RCT | All were wheelchair dependent, clinically stable and free of medication and dyspnea | S: Home, supervised by parents | Strength | |
| D: 6 weeks | PiMax/MIP | |||
| Endurance | ||||
| Tlim | ||||
|
| ||||
| N = 28 | Resistance muscle training (active/active assisted) of LE, UE and abdominal muscles. | Usual care | Functioning | |
| Vignos et al. 1966 (33) | IG (n = 14) 7.4 years CG (n = 14) 7.7 years |
F: 7 days/week first 6 months, 3-5 days/week next 6 months | Timed tests by Stair climbing, rising from floor, rising from chair, 23 feet walking. | |
| USA | Fully ambulating with good functional status | I: 10 reps with maximal resistance/lowest degree of assistance by antigravity pulley. | Quality-of-life n/a |
|
| CCT | T: 30 minutes S: Home, initial supervised by PT. D: 12 months |
Strength Overall muscle strength %-of normal (MRC). Weight lifted in each exercise. Endurance n/a |
||
|
| ||||
| N = 30 (22 analyzed) IG (n = 15) 13.6 (4.5) years CG (n = 15) 14.5 (3.8) years |
Inspiratory muscle training with special constructed training device. F: 2 times/day 5 days/week |
Usual care | Functioning VC, FEV1, 12 s MVV Quality-of-life n/a |
|
| Wanke et al. 1994 (34) Australia | Both ambulatory (n = 7) and wheelchair depended (n = 23). | I: Endurance; 10 cycles of 1-minute duration with variable resistance, 20 second rest. |
Strength PesMax, Pdi | |
| Strength; | ||||
| 10 maximal inspirations. | Endurance | |||
| RCT | Endurance time (Te) | |||
| T:n/a | ||||
| S: Home, supervised by clinicians or parents | ||||
| D: 6 months | ||||
|
| ||||
| N = 45 | Breathing exercises and a PT program as CG. | PT program | Functioning | |
| IG (n=24) | F: 2 times/day | VC, FVC, FEV1, Mobility of thorax (circumference measured at maximal inspiration (FVC level), neutral (functional residual capacity- level) and maximal expiration (residual volume-level) at three defined anatomical reference points | ||
| Zileili et al. 1999 (35) Turkey | 12.08 (1.79) years CG (n = 21) 23.43 (2.04) years |
F: 3 times/day 7 days/week I: 10 reps a) isolated chest breathing b) respiratory exercise combined with other exercises c) Breathing cycles with Triflow device T: n/a S: Home, supervised by parents |
7 days/week I: 10 reps active or active assisted UE and LE exercises isotonic exercise for abdominal muscles. Passive stretching of LE (hip, flexors, hamstrings, tensor facia latae, gastro soleus, lumbar extensors) T: n/a S: Home, supervised and help from parents |
Quality-of-life n/a |
| CCT | Participants with early scoliosis, able to cooperate and without affected respiration and use of respiratory assistive devices | D: 4 weeks | D: 4 weeks | |
| Strength | ||||
| n/a | ||||
| Endurance | ||||
| n/a | ||||
IG : Intervention group; CG: Control Group; SD : Standard deviation; F: Frequency; I: Intensity; T: time; S : Setting; D: Duration (intervention period); RCT: Randomized Controlled Trial; HHD: Hand Held Dynamometer; MMDT: Minnesota Manual Dexterity Test; AREA: Arm elevation assessment; NSAA: North Star Ambulatory Assessment; A6MCT: Assisted 6 Minutes Cycling Test; PUL: Performance Of Upper Limb; MFM: Motor Function Measure; ROM : Range Of Motion; HRQoL: Health Related Quality of Life; CCT : Clinical Controlled Trial; MVV: Maximal Voluntary Ventilation; FVC: Forced Vital Capasity; FEV1 : Forced Expiratory Volume first second; FEF: Forced Expiratory Flow; PEFR: Peak Expiratory Flow Rate; MRC: Medical Research Council (scale); PEDI: Pediatric Evaluation of Disability Inventory; MEP: Maximal Expiratory Pressure; MIP and Pi Max : Maximal Inspiratory Pressure; Pe and Pi time: Expiratory or Inspiratory Pressure sustained over time; Tlim: Time limit, maximal time a subject was able to sustain breathing against a predetermined inspiratory load without fatigue; VC: Vital Capacity; TLC: Total Lung Capacity; Pesmax: Maximal Sniff assessed Esophageal; Pdi: Trans diaphragmatic Pressure; n/a: not available