Background
Methods
Definition of Beighton Score
Search Strategy
1. Paediatric* OR Pediatric* | |
2. Child* OR Juvenile* OR Adolescent* | |
3. #1 OR #2 | |
4. Measure* OR Therap* OR Outcome* OR Hypermob* | |
5. #3 AND #4 | |
6. Elhers* OR Double-Join* OR Brighton OR Beighton | |
7. # 5 AND #6 |
Eligibility criteria
Study selection
Data extraction
Risk of bias assessment
Data analysis
Results
Selection strategy and methodological appraisal
Items | Criteria | Bale (2019) | Hsieh (2018) | Revivo (2018) | Pacey (2013) | Kemp (2010) | Scheper (2017) |
---|---|---|---|---|---|---|---|
REPORTING | |||||||
1
| Study hypothesis/aim/objective clearly described | 1 | 1 | 1 | 1 | 1 | 0 |
2
| Main outcomes in Introduction or Methods section | 1 | 1 | 1 | 1 | 1 | 1 |
3
| Patient characteristics clearly described | 1 | 1 | 1 | 1 | 1 | 1 |
4
| Relevant interventions including controls clearly described | 1 | 1 | 1 | 1 | 1 | NA |
5
| Distributions of principal confounders clearly described | 0 | 0 | 1 | 0 | 0 | 2 |
6
| Main findings (including outcomes) clearly described | 1 | 1 | 1 | 1 | 1 | 1 |
7
| Estimates of random variability in data for the main outcomes provided | 1 | 1 | 1 | 1 | 1 | 1 |
8
| All important adverse events related to intervention(s) reported | 0 | 0 | 0 | 1 | 0 | NA |
9
| Patient characteristics lost to follow-up described | 1 | 1 | 1 | 1 | 0 | 0 |
10
| Actual probability values for main outcomes reported | 1 | 1 | 1 | 1 | 1 | 1 |
EXTERNAL VALIDITY | |||||||
11
| Subjects asked to participate were representative of target populations | 1 | 1 | 1 | 1 | 1 | 1 |
12
| Subjects prepared to participate were representative of target populations | 1 | 1 | 1 | 1 | 1 | 1 |
13
| Treatment facilities and delivery were representative of target populations | 1 | 1 | 1 | 1 | 1 | 1 |
INTERNAL VALIDITY – bias | |||||||
14
| Study participants blinded to intervention administered | 0 | 0 | 0 | 1 | 0 | NA |
15
| Investigators blinded to assessment of main intervention outcomes | 1 | 1 | 0 | 1 | 1 | NA |
16
| Any data dredging was made clear at onset of study | 0 | 0 | 1 | 1 | 1 | 0 |
17
| Analyses adjust for different lengths of follow-up of participants | 1 | 0 | 1 | 1 | 0 | 1 |
18
| Statistical tests to assess the main outcomes were appropriate | 1 | 1 | 1 | 1 | 1 | 1 |
19
| Reliability of compliance with intervention(s) | 1 | 1 | 1 | 1 | 0 | NA |
20
| Main outcome measures used accurate in terms of validity and reliability. | 1 | 1 | 1 | 1 | 1 | 1 |
INTERNAL VALIDITY - confounding (selection bias) | |||||||
21
| All participants were recruited from the same target population | 1 | 1 | 1 | 1 | 1 | 1 |
22
| All participants were recruited over the same period of time | 1 | 1 | 0 | 1 | 1 | 1 |
23
| Participants were randomised to intervention group(s) | 1 | 1 | 0 | 1 | 1 | NA |
24
| Randomised intervention assignment was concealed from both participants and investigators | 0 | 0 | 0 | 1 | 0 | NA |
25
| Adequate adjustment for confounding | 0 | 0 | 0 | 0 | 0 | 1 |
26
| Lost to follow-up considered | 1 | 0 | 1 | 1 | 0 | 0 |
27
| Statistical power- clinical meaningful effect or power calculation reported b | 1~ | 1 | 1 | 1 | 1 | 1 |
Characteristics of the eligible studies
Study (year) Country | Study Design | Participant characteristics | Outcome assessment | ||||||
---|---|---|---|---|---|---|---|---|---|
Participants (n) | Drop out (%) | Age in years Mean (SD) % Female | Beighton scorea Mean (SD) | Recruitment site | Treatment or intervention group | Control group | Duration (Follow-up b) | ||
Bale et al. (2019) [39] UK | Randomised controlled trial | 119 baseline 111 At 3months 105 At 12 months | 7% 12% | 9.4 (3.2) 55% | 5.7 (1.4) | Children’s department at tertiary Hospital | therapy intervention (Tertiary PT and OT x5 sessions) | Standard care (medical assessment and allied health referrals) | 2 months (1, 10 months) |
Hsieh et al. (2018) [40] Taiwan | Randomised controlled trial | 52 Baseline 50 At 3 months | 4% | 6.6 (0.6) 46% | 7.5 (1.6) | Outpatient rehabilitation center – teaching hospital | Physical therapy & orthotics with customised insoles | Physical therapy & podiatry without customised insoles | 3 months |
Kemp et al. (2010) [41] UK | Randomised controlled trial | 57 Baseline 32 At 3 months | 44% | 10.9 (2.5) 33% | 5.8 (1.6) | Rheumatology Outpatient department | Psychosocial & physical therapy targeted to improve functional stability of symptomatic joints | Generalised therapy to improve muscle strength & fitness | 2 months (3months follow-up) |
Pacey et a1. (2013) [42] Australia | Randomised controlled trial | 29 Baseline 26 randomised 25 2 months | 14% | 12.1(2.9) 66% | 7.1 (1.2) | Physiotherapy department in a teaching hospital | Physical therapy: Muscle strength & motion control performed into full range of knee hyperextension | Physical therapy: Muscle strength & motion control performedinto knee extension neutral range | 2 week baseline without treatment followed by 8 treatment sessions and home exercises over 2 months |
Revivo et al. (2019) [43] UK | Pre-Post retrospective | 30 Baseline 26 2 months | 13% | 14.0 (2.8) 90% | >4 | Hospital Outpatient multidisciplinary pain management clinic | Physical therapy, occupational therapy, psychology counselling, & weekly paediatric rehabilitation follow-up | None | 1.5-2 months |
Scheper et al. (2017) [44] Australia | Observational longitudinal | 101 Baseline 81 3 years | 20% | 11.5 ± 3.1 55% | 7 ± 1.6 | Tertiary hospital Outpatients clinics | No restrictions on treatment of participants | None | 3 years |
Outcome measures
Outcome measures | Follow-up Timeframe d | Baseline Mean (SD) | Mean change in outcome at follow-up a | 95% CI | ||
---|---|---|---|---|---|---|
Scale | Test details | Type | ||||
PAIN (Intensity) | ||||||
(Visual Analogue scale) | 0-100 0 = no pain 100 = worst pain | PRO | 2 months [42] | Neutral treatment group: 40.0 (16.6) | -19.9 | NR |
Hypermobility treatment group: 38.6 (16.9) | -9.19 | NR | ||||
Combined groups: 39.4 (14.2) | -14.5 | -5.2, -23.8 | ||||
5 months e [41] | Targeted Physiotherapy: 55.5 (21.3) | -21.2 | -38, -4.5 | |||
General Physiotherapy: 62.1 (24.1) | -30.6 | -50.16, -11.0 | ||||
Combined groups: 57.6 (20.1) | -25.8 | -38.5, -13.1 | ||||
(Wong-baker faces pain scale) | 0-5 0 = no pain 5 = worst pain | PRO | 12 months [39] | Intervention: 2.2 (1.4) | -1.6 | -2.1, -1.1 |
Control: 2.5 (1.6) | -1.6 | -2.0, -1.2 | ||||
PAIN (Intensity) | ||||||
VAS-P [49] (Visual Analogue scale-Parental) | 0-100 0 = no pain 100 = worst pain | PRO b | 5 months [41] | Targeted Physiotherapy: 45.1 (23.0) | -21.6 | -33.2, -10.0 |
General Physiotherapy: 48.4 (22.9) | -12. | -23.3, 0.9 | ||||
Combined groups: 46.7 (22.7) | -17.2 | -25.3, -9.1 | ||||
12 months [39] | Intervention: 33.8 (24.8) | -6.8 | -14.3, 0.7 | |||
Control: 40.6 (27.5) | -7.3 | -15.4, 0.8 | ||||
FUNCTION | ||||||
CHAQ [50] (Childhood Health Assessment Questionnaire) | 0-3 0 = Without any difficulty 1 = With some difficulty 2 = With much difficulty 3 = Unable to do | PRO b | 2 months [42] | Neutral treatment group: -0.13 (0.44) | 0.12 | NR |
Hypermobility treatment group: 0.04 (0.71) | 0.02 | NR | ||||
Combined groups: -0.5 (0.6) | 0.07 | -0.1, 0.2 | ||||
5 months [41] | Targeted Physiotherapy: 0.62 (0.65) | -0.15 | -0.3, -0.02 | |||
General Physiotherapy: 0.76 (0.68) | -0.16 ( | -0.4, 0.1 | ||||
Combined groups: 0.69 (0.66) | -0.15 | -0.3, -0.02 | ||||
12 months [39] | Intervention: 0.84 (0.62) | 0.04 | 0.1, 0.2 | |||
Control: 0.86 (0.72) | −0.02 | -0.12, 0.08 | ||||
Dynamometry [51] Measurement of strength | Grip strength. Units: kilopascals | CRO | 12 months [39] | Intervention: 57.0 (25.0) | 4.7 | 0.1, 9.3 |
Control: 59.4 (31.7) | 7.3 | 2.9, 11.7 | ||||
Knee flexor and extensor strength. Units: Newtons | 2 months [42] | Neutral treatment group: 4.0 (1.7) | 0.88 | NR | ||
Hypermobility treatment group: 4.4 (2.4) | 1.21 | NR | ||||
Combined groups: 4.2 (2.0) | 1.1 | 0.4, 1.7 | ||||
FUNCTION | ||||||
M-ABC2 [52] (Movement Assessment Battery for Children, 2nd Edition) | Measures coordination Raw scores converted to centiles (0 – 100) with higher centiles indicating better performance compared to peers | CRO | 12 months [39] | Intervention: 33.4 (26.7) | 3.8 | -1.7, 9.3 |
Control: 35.6 (30.1) | 10.8 | 5.4, 16.2 | ||||
(Pediatric Outcomes Data Collection Instrument) | 0-100 100= highest functioning | PRO b | 3 months [40] | Transfer and basic mobility domain Intervention: 82.1 (14.8) | 11.8 | 0.30, 1.43 |
Control: 94.2 (14.8) | 1.2 | -0.46, 0.62 | ||||
6 MWT [55] (6 min walk test) | Maximum distance walked in 6 minutes (meters/leg length) | CRO | 36 months [44] | Able/moderate: 7.3 (1.6) c | -1.5 | -1.3, -4.5 |
Severe: 5.3 (1.6) | -2.3 | -2.4, -2.7 | ||||
No. of flights of stairs climbed in 2 min [56] | Assesses functional ability in stairclimbing | CRO | 2 months [42] | Neutral treatment group: 16.3 (5.0) | 3.8 | NR |
Hypermobility treatment group: 20.9 (6.7) | -0.33 | NR | ||||
Combined groups: 18.6 (5.7) | 1.7 | -0.5, 3.9 | ||||
Quality of Life | ||||||
CHU9D [57] (Child Health Utility 9D) | 0-4 higher scores indicate poorer HRQoL | PRO | 12 months [39] | Intervention: 0.85 (0.10) | 0.02 (0.09) | -0.004, 0.04 |
Control: 0.85 (0.12) | 0.00 (0.12) | -0.03, 0.03 | ||||
CHQ-PF50 [58] (Child Health Questionnaire) | 0-100 0 = worst QoL 100 = Best QoL | PRO b | 2 months [42] |
Physical summary score
| ||
Neutral treatment group: 32.0 (11.9) | 10.1 | NR | ||||
Hypermobility treatment group: 41.6 (15.0) | 2.3 | NR | ||||
Combined groups: 38.0 (12.6) | 5.3 | 1.7, 8.9 | ||||
Psychological summary score
| ||||||
Neutral treatment group: 46.4 (12.3) | -0.9 | NR | ||||
Hypermobility treatment group: 46.3 (9.0) | 8.1 | NR | ||||
Combined groups: 48 (10.3) | 2.7 | -0.3, 5.8 | ||||
Quality of Life | ||||||
(Patient global impression of change) | 1-7 1= very much improved 7= very much worse | PRO | 2 months [42] | Neutral treatment group: 0.3 (1.1) | 1.4 | NR |
Hypermobility treatment group: 0.2 (0.9) | 1.6 | NR | ||||
Combined groups: 0.2 (1.0) | 1.5 | 1.0, 2.0 | ||||
PODCI [53] (Pediatric Outcomes Data Collection Instrument) Pain comfort Happiness | 0-100 Higher score means higher health-related quality | PRO b | 3 months [40] |
1. Pain/comfort:
| ||
Intervention: 83.9 (16.2) | 4.9 | -0.22, 0.87 | ||||
Control: 84.4 (17) | -1.2 | -0.61, 0.47 | ||||
2. Happiness:
| ||||||
Intervention: 79.5 (18.7) | -0.2 | -0.55, 0.53 | ||||
Control: 80.7 (15.9) | -0.9 | -0.60, 0.48 | ||||
0-100 Lower score indicates lower quality of life | PRO b | 3 months [40] |
1. Physical
| |||
Intervention: 62.3 (19.9) | 3.9 | -0.35, 0.73 | ||||
Control: 79.2 (20.1) | -8.1 | -0.95, 0.14 | ||||
2. Psychosocial
| ||||||
Intervention: 65.6 (16.3) | 0.9 | -0.49, 0.59 | ||||
Control: 73.8 (18.8) | 0.3 | -0.52, 0.56 | ||||
Quality of Life | ||||||
Global-VAS (parent’s global assessment) | 0-100 0 = no impact of hypermobility 100 = high impact of hypermobility | PRO b | 5 months [41] | Targeted Physiotherapy: 36.1 (26.4) General | -17.6 | -31.1, -4.1 |
Physiotherapy: 37.2 (25.3) | 3.7 | -7.8, 15.3 | ||||
Combined groups: 36.6 (25.7) n = 32 | -7.6 | -17.2, -2.0 |