Generalized joint hypermobility (GJH) is highly prevalent among patients diagnosed with chronic pain. When GJH is accompanied by pain in $4 joints over a period $3 months in the absence of other conditions that cause chronic pain, the hypermobility syndrome (HMS) may be diagnosed. In addition, GJH is also a clinical sign that is frequently present in hereditary diseases of the connective tissue, such as the Marfan syndrome, osteogenesis imperfecta, and the Ehlers-Danlos syndrome. However, within the Ehlers-Danlos spectrum, a similar subcategory of patients having similar clinical features as HMS but lacking a specific genetic profile was identified: Ehlers-Danlos syndrome hypermobility type (EDS-HT). Researchers and clinicians have struggled for decades with the highly diverse clinical presentation within the HMS and EDS-HT phenotypes (Challenge 1) and the lack of understanding of the pathological mechanisms that underlie the development of pain and its persistence (Challenge 2). In addition, within the HMS/EDS-HT phenotype, there is a high prevalence of psychosocial factors, which again presents a difficult issue that needs to be addressed (Challenge 3). Despite recent scientific advances, many obstacles for clinical care and research still remain. To gain further insight into the phenotype of HMS/EDS-HT and its mechanisms, clearer descriptions of these populations should be made available. Future research and clinical care should revise and create consensus on the diagnostic criteria for HMS/EDS-HT (Solution 1), account for clinical heterogeneity by the classification of subtypes within the HMS/EDS-HT spectrum (Solution 2), and create a clinical core set (Solution 3).

Dancers with GJH seem more vulnerable to musculoskeletal and psychological complaints. In addition, GJH was also associated with lower physical fitness, despite training. Caregivers for professional dancers should monitor closely the physical capabilities and the amount of psychological strain.

BackgroundGeneralized Joint Hypermobility (GJH) has been found to be associated with musculoskeletal complaints and disability. For others GJH is seen as a prerequisite in order to excel in certain sports like dance. However, it remains unclear what the role is of GJH in human performance. Therefore, the purpose of the study was to establish the association between GJH and functional status and to explore the contribution of physical fitness and musculoskeletal complaints to this association.MethodsA total of 72 female participants (mean age (SD; range): 19.6 (2.2; 17-24)) were recruited among students from the Amsterdam School of Health Professions (ASHP) (n = 36) and the Amsterdam School of Arts (ASA), Academy for dance and theater (n = 36) in Amsterdam, The Netherlands. From each participant the following data was collected: Functional status performance (self-reported Physical activity level) and capacity (walking distance and jumping capacity: side hop (SH) and square hop (SQH)), presence of GJH (Beighton score ≥4), muscle strength, musculoskeletal complaints (pain and fatigue) and demographic characteristics (age and BMI).ResultsGJH was negatively associated with all capacity measures of functional status. Subjects with GJH had a reduced walking distance (B(SE):-75.5(10.5), p = <.0001) and jumping capacity (SH: B(SE):-10.10(5.0), p = .048, and SQH: B(SE):-11.2(5.1), p = .024) in comparison to subjects without GJH, when controlling for confounding: age, BMI and musculoskeletal complaints. In participants with GJH, functional status was not associated with performance measures.ConclusionGJH was independently associated with lower walking and jumping capacity, potentially due to the compromised structural integrity of connective tissue. However, pain, fatigue and muscle strength were also important contributors to functional status.

The Dutch version demonstrated good internal consistency and good construct validity in a population of adolescents with chronic musculoskeletal pain. Implications for rehabilitation The Fear of Pain Questionnaire-Child version was developed to measure fear of pain and avoidance in children and adolescents with chronic pain. Identification of fear of pain and activities that are being avoided are important during screening and assessment of the adolescent for chronic pain rehabilitation treatment. The presence of fear of pain and/or avoidance behavior is important information to shape and target multidisciplinary rehabilitation treatment.

The Dutch version can be used to assess pain-related disability in Dutch-speaking adolescents comparable to the study sample. Scores on both subscales provide insight into the severity of the pain-related disability in both daily routine and more physically vigorous activities. Implications for Rehabilitation Chronic pain is a disabling disorder which not only impacts physically but restricts quality of life. This study provides clinicians a questionnaire to measure pain-related disability and quantify the impact of pain on the daily living of adolescents. The advantage of the Dutch version of the Child Activity and Limitations Interview over other measurements is that it can distinguish limitations in daily activities from more physically vigorous activities.

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