The natural history of children with joint hypermobility syndrome and Ehlers–Danlos hypermobility type: a longitudinal cohort study

Scheper, Mark; Nicholson, Lesley L.; Adams, Roger; Tofts, Louise; Pacey, Verity

doi:10.1093/rheumatology/kex148

Cited by 47 publications

(68 citation statements)

References 32 publications

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“…This is also confirmed in a recent longitudinal study in children with G-HSD/hEDS, in which multi-systemic dysfunction (e.g. skin, cardiovascular, and gastrointestinal involvement), high levels of pain and fatigue and loss of postural control were found to be the most important constructs for functional impairment in terms of performance and capacity (Scheper et al, 2017). However, given that the findings of the current study are based on a small number of adolescents with asymptomatic GJH, this interpretation should be treated with caution.…”

Section: Discussionsupporting

confidence: 77%

“…A lower level of physical functioning in adolescents with G-HSD/hEDS is not solely caused by GJH. It is therefore important that, in clinical practice, treatment of adolescents with G-HSD/hEDS should also focus on other contributing factors such as pain, fatigue, multi-systemic dysfunction, loss of postural control and pain-related fear (Leeuw et al, 2007;Scheper et al, 2017;Vlaeyen and Linton, 2000).…”

Section: Discussionmentioning

confidence: 99%

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Generalized joint hypermobility and perceived harmfulness in healthy adolescents; impact on muscle strength, motor performance and physical activity level

Meulenbroek

Huijnen

Stappers

et al. 2020

Physiotherapy Theory and Practice

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Objective: The first aim of this study was to determine whether adolescents with asymptomatic Generalized Joint Hypermobility (GJH) have a lower level of physical functioning (physical activity level, muscle strength and performance) compared to non-hypermobile controls. Secondly, to evaluate whether the negative impact of perceived harmfulness on physical functioning was more pronounced in adolescents with asymptomatic GJH. Methods: Cross-sectional study. Sixty-two healthy adolescents (mean age 16.8, range 12-21) participated. Hypermobility (Beighton score), perceived harmfulness (PHODA-youth) and muscle strength (dynamometry), motor performance (Single-Leg-Hop-for-Distance) and physical activity level (PAL) (accelerometry) were measured. Hierarchical regression analyses were used to study differences in physical functioning and perceived harmfulness between asymptomatic GJH and non-hypermobile controls. Results: Asymptomatic GJH was associated with increased knee extensor muscle strength (peak torque/body weight; PT/BW), controlled for age and gender (dominant leg; ß = 0.29; p = .02). No other associations between asymptomatic GJH and muscle strength, motor performance and PAL were found. Perceived harmfulness was not more pronounced in adolescents with asymptomatic GJH. Conclusions: Adolescents with asymptomatic GJH had increased knee extensor muscle strength compared to non-hypermobile controls. No other differences in the level of physical functioning was found and the negative impact of perceived harmfulness was not more pronounced in adolescents with asymptomatic GJH. ARTICLE HISTORY

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Section: Discussionsupporting

confidence: 77%

Section: Discussionmentioning

confidence: 99%

Generalized joint hypermobility and perceived harmfulness in healthy adolescents; impact on muscle strength, motor performance and physical activity level

Meulenbroek

Huijnen

Stappers

et al. 2020

Physiotherapy Theory and Practice

View full text Add to dashboard Cite

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“…A case‐controlled study by Mastoroude et al found the prevalence of UI in women with bJHS to be significantly greater than controls (73% vs 48%). In a prospective longitudinal cohort study of children with hEDS or joint hypermobility syndrome (JHS), Scheper et al found UI in 23% overall and 40% among a subset more severely affected by EDS or JHS.…”

Section: Resultsmentioning

confidence: 99%

Urogenital and pelvic complications in the Ehlers‐Danlos syndromes and associated hypermobility spectrum disorders: A scoping review

2019

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The Ehlers-Danlos syndromes (EDS) and associated hypermobility spectrum disorders (HSD) are a heterogenous group of connective tissue disorders associated with significant morbidity. The urogenital aspects of these disorders are understudied and there is little guidance on the prevalence, types, or outcomes of urogenital complications in EDS/HSD. Our objective was to perform a scoping review to characterize and synthesize the literature reporting urogenital and pelvic complications in EDS/HSD patients. We performed a systematic search of three databases (Medline, CINAHL, Embase) to January 2019. English language, full-text articles reporting on urogenital or pelvic complications in EDS/HSD were included. A total of 105 studies were included (62 case reports/series, 43 observational) involving patients with hypermobile (23%), vascular (20%), classical (12%) EDS, and HSD (24%). Some studies looked at multiple subtypes (11%) or did not report subtype (33%). Reported complications included urinary (41%), gynecological (36%), obstetrical (25%), renal (9%), and men's health problems (7%), with some studies reporting on multiple areas. Urinary and gynecological complications were most prevalent in patients with HSD, while a broad range of complications were reported in EDS. While further research is required, results suggest a higher index of suspicion for urogenital problems is probably warranted in this population.

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“…In comparison with healthy controls, children with hEDS/HSD (previously overlapping with JHS) have been previously reported to have overall decreased QOL scores (Fatoye, Palmer, MacMillan, Rowe, & Van Der Linden, ; Pacey et al, ), with possibly contributing factors including increased musculoskeletal injury (Rombaut, Malfait, Cools, De Paepe, & Calders, ; Stern et al, ), pain intensity and duration (Fatoye et al, ; Stern et al, ), increased fatigue (Scheper, Nicholson, Adams, Tofts, & Pacey, ), and higher risk for multisystem complaints including orthostatic intolerance and headaches (Rombaut et al, ; Scheper et al, ). Moreover, these children had decreased physical activity and participation in daily life activities (Schubert‐Hjalmarsson, Öhman, Kyllerman, & Beckung, ).…”

Section: Discussionmentioning

confidence: 99%

Factors affecting quality of life in children and adolescents with hypermobile Ehlers‐Danlos syndrome/hypermobility spectrum disorders

Muriello

Clemens

et al. 2019

American J of Med Genetics Pt A

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Hypermobile Ehlers‐Danlos syndrome (hEDS) is a hereditary disorder of connective tissue, often presenting with complex symptoms can include chronic pain, fatigue, and dysautonomia. Factors influencing functional disability in the pediatric hEDS population are incompletely studied. This study's aims were to assess factors that affect quality of life in children and adolescents with hEDS. Individuals with hEDS between the ages 12–20 years and matched parents were recruited through retrospective chart review at two genetics clinics. Participants completed a questionnaire that included the Pediatric Quality of Life Inventory (PedsQL™), PedsQL Multidimentional Fatigue Scale, Functional Disability Inventory, Pain‐Frequency‐Severity‐Duration Scale, the Brief Illness Perception Questionnaire, measures of anxiety and depression, and helpful interventions. Survey responses were completed for 47 children and adolescents with hEDS/hypermobility spectrum disorder (81% female, mean age 16 years), some by the affected individual, some by their parent, and some by both. Clinical data derived from chart review were compared statistically to survey responses. All outcomes correlated moderately to strongly with each other. Using multiple regression, general fatigue and pain scores were the best predictors of the PedsQL total score. Additionally, presence of any psychiatric diagnosis was correlated with a lower PedsQL score. Current management guidelines recommend early intervention to prevent disability from deconditioning; these results may help identify target interventions in this vulnerable population.

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The natural history of children with joint hypermobility syndrome and Ehlers–Danlos hypermobility type: a longitudinal cohort study

Cited by 47 publications

References 32 publications

Generalized joint hypermobility and perceived harmfulness in healthy adolescents; impact on muscle strength, motor performance and physical activity level

Generalized joint hypermobility and perceived harmfulness in healthy adolescents; impact on muscle strength, motor performance and physical activity level

Urogenital and pelvic complications in the Ehlers‐Danlos syndromes and associated hypermobility spectrum disorders: A scoping review

Factors affecting quality of life in children and adolescents with hypermobile Ehlers‐Danlos syndrome/hypermobility spectrum disorders

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