INTRODUCTION: To provide a state of the art on diagnostics, clinical characteristics, and treatment of paediatric generalised joint hypermobility (GJH) and joint hypermobility syndrome (JHS).METHOD: A narrative review was performed regarding diagnostics and clinical characteristics. Effectiveness of treatment was evaluated by systematic review. Searches of Medline and Central were performed and included nonsymptomatic and symptomatic forms of GJH (JHS, collagen diseases).RESULTS: In the last decade, scientific research has accumulated on all domains of the ICF. GJH/JHS can be considered as a clinical entity, which can have serious effects during all stages of life. However research regarding the pathological mechanism has resulted in new potential opportunities for treatment. When regarding the effectiveness of current treatments, the search identified 1318 studies, from which three were included (JHS: n = 2, Osteogenesis Imperfecta: n = 1). According to the best evidence synthesis, there was strong evidence that enhancing physical fitness is an effective treatment for children with JHS. However this was based on only two studies.CONCLUSION: Based on the sparsely available knowledge on intervention studies, future longitudinal studies should focus on the effect of physical activity, fitness, and joint stabilisation. In JHS and chronic pain, the effectiveness of a multidisciplinary approach should be investigated.
Background: To determine whether adolescents with generalized hypermobility spectrum disorder/hypermobile Ehlers-Danlos syndrome (G-HSD/hEDS) show changes in the level of disability, physical functioning, perceived harmfulness and pain intensity after completing multidisciplinary rehabilitation treatment.Methods: Pre-test post-test design. Fourteen adolescents with G-HSD/hEDS participated. The multi-disciplinary rehabilitation treatment consisted of a combination of physical training and exposure in vivo. Physical training aims to improve aerobic capacity, muscle strength and propriocepsis for compensating hypermobility. Exposure in vivo aims to decrease disability and pain-related fear. Pre- and post-treatment assessments were conducted to assess the level of disability, physical functioning (motor performance, muscle strength and physical activity level), perceived harmfulness and pain intensity.Results: After completing multidisciplinary rehabilitation treatment, the adolescents showed a significant and clinically relevant improvement (improvement of 67%, p < 0.01) in functional disability. Furthermore, significant improvements were found in motor performance (p < 0.01), muscle strength (p < 0.05), perceived harmfulness (p < 0.01) and pain intensity (p < 0.01) after completing multidisciplinary rehabilitation treatment.Conclusion: Multidisciplinary rehabilitation treatment leads to a significantly and clinically relevant improvement in the level of disability for adolescents with G-HSD/hEDS. Positive effects were also found in physical functioning, perceived harmfulness and pain intensity. Although the results of this multidisciplinary rehabilitation treatment for adolescents with G-HSD/hEDS are promising, further study is needed to confirm these findings in a randomized design.
OBJECTIVE: To reach consensus on the most important biopsychosocial factors that influence functional capacity results in patients with chronic nonspecific musculoskeletal pain, arranged in the framework of the International Classification of Functioning, Disability and Health.DESIGN: Three-round, internet-based Delphi survey.SETTING: Not applicable.PARTICIPANTS: Participants were scientists, clinicians, and patients familiar with functional capacity testing. Scientists were invited through purposive sampling based on the number of relevant publications in peer-reviewed journals. The scientists recruited clinicians and patients through snowball sampling.INTERVENTIONS: Not applicable.MAIN OUTCOME MEASURES: Consensus was reached if at least moderate influence (25%) was achieved and an interquartile range of no more than 1 point was reached.RESULTS: Thirty-three scientists, 21 clinicians, and 21 patients from 9 countries participated. Participants reached consensus on 6 factors that can influence the outcome of the lifting test, having a median of severe influence (50%-95%): catastrophic thoughts and fear, patient adherence to "doctor's orders," internal and external motivation, muscle power, chronic pain behavior, and avoidance behavior. Motivation, chronic pain behavior, and sensation of pain were the top 3 factors affecting postural tolerance and repetitive movement functional capacity tests. Furthermore, participants reported 28 factors having a median of moderate influence (25%-49%) that could influence the outcome of lifting, postural tolerance, and repetitive movement tests.CONCLUSIONS: Overall, chronic pain behavior, motivation, and sensation of pain are the main factors that can influence functional capacity results. We recommend that scientists and clinicians, respectively, consider the most important factors when planning future studies and when interpreting functional capacity test results.
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