Children with Generalised Joint Hypermobility and Musculoskeletal Complaints: State of the Art on Diagnostics, Clinical Characteristics, and Treatment

Abstract

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.

The association between muscle strength and activity limitations in patients with the hypermobility type of Ehlers–Danlos syndrome: the impact of proprioception

“…Poor proprioception has frequently been reported in EDS-HT patients and has been postulated to be an important factor in activity limitations. [17,24,41] Our results indicate that poor proprioception, especially during activities that require controlling discrete joint motion (knee flexion), has an influence on muscle strength. However, the generalizability to other joints within the functional chain is unknown.…”

“…However, the evidence to support this rationality is scarce. [10,[15][16][17] When developing effective treatment it is essential to know which factors are associated with activity limitations. Muscle strength might be an important determinant of activity limitations, however, a direct relationship between muscle strength and activity limitations in EDS-HT has never been demonstrated.…”

Survey on joint hypermobility in university students aged 18–25 years old

“…It has been recognized as a phenomenon frequently observed in healthy people, acrobats, gymnasts, and ballerinas [1][2][3][4][5]. Hypermobility is also part of the syndromic presentation of certain genetic diseases, [6] such as Ehler-Danlos Syndrome, Marfan Syndrome, Down Syndrome, Osteogenesis imperfecta, and Stickler Syndrome, among others.…”

“…These maneuvers are individually scored on each side of the body and spine, to a total of 9 points. Scores greater than or equal to 4 are classified as generalized joint hypermobility, and scores 1-3 are classified as localized joint hypermobility [1,4,27].…”

Influência Do Hábito De Exercício Na Infância E Adolescência Na Flexibilidade De Adultos Sedentários

“…Valores muitos elevados, expressos por uma hipermobilidade, podem estar associados a uma maior predisposição a lesões musculares, tendíneas e articulares e a algumas outras doenças específicas do tecido conjuntivo ou a determinadas enfermidades clínicas 29 . Por outro lado, quando presente em valores reduzidos pode representar um risco maior de quedas nos idosos 14 , diminuindo assim a qualidade de vida dos mesmos e prejudicando a execução de atividades rotineiras, tal como sentar e levantar do solo 30 .…”