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Disability in Adolescents and Adults Diagnosed With Hypermobility-Related Disorders: A Meta-Analysis

  • Mark C. Scheper
    Correspondence
    Corresponding author Mark C. Scheper, PT, MSc, Amsterdam Centre for Innovative Health Practice, Center for Applied Research, Faculty of Health, University of Applied Sciences Amsterdam, Tafelbergweg 51, 1105BD Amsterdam, The Netherlands.
    Affiliations
    Amsterdam Center for Innovative Health Practice, Center for Applied Research, Faculty of Health, University of Applied Sciences Amsterdam, Amsterdam, The Netherlands

    Department of Rehabilitation, Academic Medical Center, Amsterdam, The Netherlands
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  • Birgit Juul-Kristensen
    Affiliations
    Research Unit for Musculoskeletal Function and Physiotherapy, Institute of Sports Science and Clinical Biomechanics, University of Southern Denmark, Odense M, Denmark

    Institute of Occupational Therapy, Physiotherapy and Radiography, Bergen University College, Bergen, Norway
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  • Lies Rombaut
    Affiliations
    Department of Rehabilitation Sciences and Physiotherapy, Ghent University, Ghent, Belgium
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  • Eugene A. Rameckers
    Affiliations
    Department of Rehabilitation Medicine, Maastricht University Medical Center, CAPHRI School for Public Health and Primary Care, Maastricht, The Netherlands

    Adelante Center of Expertise in Rehabilitation and Audiology, Hoensbroek, The Netherlands

    Master of Pediatric Physical Therapy, University of Applied Sciences, AVANS+, Breda, The Netherlands
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  • Jeanine Verbunt
    Affiliations
    Department of Rehabilitation Medicine, Maastricht University Medical Center, CAPHRI School for Public Health and Primary Care, Maastricht, The Netherlands

    Adelante Center of Expertise in Rehabilitation and Audiology, Hoensbroek, The Netherlands
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  • Raoul H. Engelbert
    Affiliations
    Amsterdam Center for Innovative Health Practice, Center for Applied Research, Faculty of Health, University of Applied Sciences Amsterdam, Amsterdam, The Netherlands

    Department of Rehabilitation, Academic Medical Center, Amsterdam, The Netherlands
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Published:March 11, 2016DOI:https://doi.org/10.1016/j.apmr.2016.02.015

      Abstract

      Objective

      To (1) establish the association of the most common reported symptoms on disability; and (2) study the effectiveness of treatment on disability in patients with Ehlers-Danlos syndrome–hypermobility type (EDS-HT)/hypermobility syndrome (HMS).

      Data Sources

      An electronic search (Medical Subject Headings and free-text terms) was conducted in bibliographic databases CENTRAL/MEDLINE.

      Study Selection

      Comparative, cross-sectional, longitudinal cohort studies and (randomized) controlled trials including patients with HMS/EDS-HT aged ≥17 years were considered for inclusion. A class of symptoms was included when 5 publications were available. In regards to treatment (physical, cognitive interventions), only (randomized) controlled trials were considered. Surgical and medicinal interventions were excluded.

      Data Extraction

      Bias was assessed according to the methodological scoring tools of the Cochrane collaboration. Z-score transformations were applied to classify the extent of disability in comparison with healthy controls and to ensure comparability between studies.

      Data Synthesis

      Initially, the electronic search yielded 714 publications, and 21 articles remained for analysis after selection. The following symptoms were included for meta-analysis: pain (n=12), fatigue (n=6), and psychological distress (n=7). Pain (r=.64, P=.021), fatigue (r=.91, P=.011), and psychological distress (r=.86, P=.018) had a significant impact on disability. Regarding treatment, a significant pain reduction was achieved by a variety of physical and cognitive approaches. Treatment effectiveness on disability was not established.

      Conclusions

      Disability can affect patients with HMS/EDS-HT significantly and is highly correlated with both physical and psychological factors. Although evidence is available that physical and psychological treatment modalities can induce significant pain reduction, the evidence regarding disability reduction is lacking.

      Keywords

      List of abbreviations:

      EDS (Ehlers-Danlos syndrome), EDS-HT (Ehlers-Danlos syndrome–hypermobility type), GJH (generalized joint hypermobility), HDCT (hereditary diseases of connective tissue), HMS (hypermobility syndrome), ICF (International Classification of Functioning, Disability and Health), IQR (interquartile range), MeSH (Medical Subject Headings)
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