Quality of life prediction in children with joint hypermobility syndrome
Abstract
Aims To assess the child- and parent-reported health-related quality of life (HRQOL) of children with joint hypermobility syndrome (JHS), to compare these with other chronic paediatric conditions and to determine whether symptoms experienced by children with JHS can predict their HRQOL. Methods Eighty-nine children with JHS and one of their parents completed the Pediatric Quality of Life Inventory 4.0 Generic Core Scale, the Multidimensional Fatigue Scale and the Pediatric Pain Questionnaire. Anthropometric measures and reported symptoms were recorded. Child-reported HRQOL scores were compared with parent report, and both child- and parent-reported HRQOL scores of children with JHS were compared with those of children with other chronic conditions. Stepwise multiple regression was undertaken to determine whether any combination of measures could predict HRQOL. Results Parent- and child-reported HRQOL scores were strongly correlated (r = 0.6-0.84, all P Conclusion Children with JHS experience poor HRQOL and disabling fatigue, with parent scores providing a good proxy. Pain, fatigue and the presence of stress incontinence symptoms have the greatest impact on their HRQOL.
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Constipation is the commonest GI symptom, followed by diarrhea and recurrent abdominal pain, in pediatric hypermobility.
“Constipation is the commonest symptom, followed by diarrhea and recurrent abdominal pain, with 54% of the Sydney cohort reporting any GI symptom and 17% reporting more than one [22].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
Functional gastrointestinal disorders (FGID) can present in pediatric patients with hypermobility.
“Functional gastrointestinal disorders (FGID) can present in pediatric patients with hypermobility.”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
In the Sydney cohort, patients commonly reported joint instability (67% overall) at the knee (predominantly patella) (35%), ankle (32%), shoulder (26%), hand (18%), and wrist (14%).
“In the Sydney cohort [22] patients commonly reported joint instability (67% overall) at the following sites knee (predominantly patella) (35%), ankle (32%), shoulder (26%), hand (18%), and wrist (14%).”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
In the Sydney clinical cohort of 89 patients aged 6-16 years with Beighton scores ≥ 4 at inception, the commonest complaint was joint pain, with 94% reporting pain in multiple joints (mean 6.4 joints, range 0-15).
“In the Sydney clinical cohort of 89 patients aged 6–16 years, with Beighton scores ≥ 4 at inception, the commonest complaint was joint pain with 94% reporting pain in multiple joints (mean 6.4 joints, range 0–15), at the knee 63%, foot 50%, ankle 48%, hand 36%, wrist 33%, back 27%, hip 26%, shoulder 22%, and elbow 21% [22].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
Several studies have identified an increased prevalence of urinary incontinence in hypermobile pediatric case-controlled studies.
“Several studies have identified an increased prevalence of urinary incontinence in hypermobile pediatric case-controlled studies [22, 48, 49].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
17% of the Sydney cohort reported more than one GI symptom.
“Constipation is the commonest symptom, followed by diarrhea and recurrent abdominal pain, with 54% of the Sydney cohort reporting any GI symptom and 17% reporting more than one [22].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
The Sydney cohort reported orthostatic intolerance symptoms in 39% of the group.
“The Sydney cohort reported orthostatic intolerance symptoms in 39% of the group [22].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
The prevalence of symptoms of stress incontinence in the Sydney cohort was 26% in hypermobile children.
“The prevalence of symptoms of stress incontinence in the Sydney cohort was 26% and this predicted reduced quality of life in hypermobile children [22].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
There is growing recognition of an association between autonomic dysfunction and hEDS/HSD.
“There is growing recognition of an association between autonomic dysfunction and hEDS/HSD and the group agreed it is an important comorbidity, commonly presenting as Postural Orthostatic Tachycardia Syndrome (POTS) in older children.”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
Stress incontinence symptoms predicted reduced quality of life in hypermobile children.
“The prevalence of symptoms of stress incontinence in the Sydney cohort was 26% and this predicted reduced quality of life in hypermobile children [22].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
There is increasing evidence for lower urinary tract dysfunction as part of the clinical presentation of symptomatic hypermobility.
“There is increasing evidence for the presence of lower urinary tract dysfunction, defined using the NDDIK criteria [39], as part of the clinical presentation of symptomatic hypermobility”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
54% of the Sydney cohort reported any GI symptom.
“Constipation is the commonest symptom, followed by diarrhea and recurrent abdominal pain, with 54% of the Sydney cohort reporting any GI symptom and 17% reporting more than one [22].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [22]
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- Authors
- Pacey, V., Tofts, L., Adams, R. D., Munns, C. F., & Nicholson, L. L.
- Affiliations
- The University of Sydney (AU)
- Corresponding author
- Pacey, Verity
- Venue
- Journal of Paediatrics and Child Health
- Publisher
- Wiley
- Published
- 2015-01-26
- Volume
- 51
- Issue
- 7
- Pages
- 689-695
- ISSN
- 1034-4810 (ISSN-L)
- 1440-1754
- OpenAlex
- W2144799769
- Cited by
- 77
- References
- 4
- Citations by year
- 2026: 3 · 2025: 6 · 2024: 5 · 2023: 8 · 2022: 15 · 2021: 7 · 2020: 9 · 2019: 7 · 2018: 8 · 2017: 6 · 2016: 2 · 2015: 1
- FWCI
- 3.88
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of Paediatrics and Child Health (publishedVersion)
- PubMed (publishedVersion)
- Funders
- The Menzies Foundation
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Pharmacology › Musculoskeletal pain and rehabilitation
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Pediatric Pain Management Techniques
- Keywords
- joint hypermobility syndrome, health-related quality of life, children, parent-reported outcomes, child-reported outcomes, fatigue, pain, stress urinary incontinence
- MeSH terms
- Adolescent, Child, Chronic Disease, Ehlers-Danlos Syndrome, Fatigue, Female, Health Status Indicators, Humans, Joint Instability, Male, Parents, Quality of Life, Case-Control Studies, Self Report, Chronic Pain
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