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).
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Young professional dancers find themselves in a demanding environment. GJH within dancers is often seen as aesthetically beneficial and a sign of talent but was found to be potentially disabling. Moreover, high-performing adolescents and young adults (HPAA), in this specific lifespan, might be even more vulnerable to anxiety-related disability. Therefore, we examined the development of the association between the presence of Generalized Joint Hypermobility (GJH) and anxiety within HPAA with a one-year follow-up. In 52.3% of the HPAA, anxiety did not change significantly over time, whereas GJH was present in 28.7%. Fatigue increased significantly in all HPAA at one year follow-up (respectively, females MD (SD) 18(19), p < 0.001 and males MD (SD) 9(19), p < 0.05). A significantly lower odds ratio (ß (95% CI) 0.4 (0.2–0.9); p-value 0.039) for participating in the second assessment was present in HPAA with GJH and anxiety with a 55% dropout rate after one year. This confirms the segregation between GJH combined with anxiety and GJH alone. The fatigue levels of all HPAA increased significantly over time to a serious risk for sick leave and work disability. This study confirms the association between GJH and anxiety but especially emphasizes the disabling role of anxiety. Screening for anxiety is relevant in HPAA with GJH and might influence tailored interventions.
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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.
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