Joint hypermobility syndrome subclassification in paediatrics: a factor analytic approach
Abstract
Objective: To determine if exploratory factor analysis can identify subtypes comprising recognisable clinical patterns of the presenting signs and symptoms of children with joint hypermobility syndrome (JHS). Patients: Eighty-nine children with JHS aged 6–16 years. Methods: Twelve tests comprising anthropometric, musculoskeletal and functional assessments were conducted. Signs, symptoms and family history were recorded. Exploratory factor analysis was performed, factor scores generated, and correlations calculated to identify associations. Results: Sixty-six percent of the variance in the score set could be accounted for by five JHS subtypes (Eigenvalues >1). Factor 1, ‘joint affected’ JHS, had loadings on multiple joint pain, recurrent joint instability and postural orthostatic hypotension symptoms, and factor scores were associated with worse pain (r=0.48, p<0.01), fatigue (r=−0.54, p<0.01) and reduced health-related quality of life (HRQOL) (r=−0.5, p<0.01). Factor 2, ‘athletic’ JHS, loaded on muscle endurance, balance and motor skill proficiency, and scores were associated with less fatigue (r=0.3, p<0.01) and better HRQOL (r=0.44, p<0.01). Factor 3, ‘systemic’ JHS, loaded on skin involvement, incontinence symptoms, bowel involvement and recurrent joint instability, and was associated with reduced HRQOL (r=−0.24, p=0.03). Factor 4, ‘soft tissue affected’ JHS, loaded on recurrent soft tissue injuries and reduced muscle length, and was associated with greater fatigue (r=−0.43, p<0.01) and reduced HRQOL (r=−0.44, p<0.0001). Factor 5, ‘high BMI’ JHS, had high loadings on body mass index (BMI) for age, muscle endurance and no gastrointestinal involvement, and was associated with higher pain (r=0.33, p<0.01). Conclusions: The presenting signs and symptoms of children with JHS can be summarised in five clinically recognisable subtypes.
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Children and adolescents with hypermobility reported headache and recurrent abdominal pain.
“Children and adolescents in these studies also reported headache [42], and recurrent abdominal pain [43].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [43]
Worse fatigue correlated with worse quality of life at inception.
“Worse fatigue correlated with more functional impairment at 3 years in the prospective Sydney cohort [17] and worse quality of life at inception [43].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [43]
An exploratory study of 89 JHS children identified five subphenotypes: joint affected, athletic, systemic, soft tissue affected, and high BMI.
“An exploratory study on 89 JHS children identified five subphenotypes, namely joint affected, athletic, systemic, soft tissue affected and high BMI [11].”
From Severity classes in adults with hypermobile Ehlers–Danlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients (2019) · cites this paper as [11]
Inclusion of adult patients only did not allow exploration of the impact of the developmental trajectories proposed by others on the resulting phenotype.
“Inclusion of adult patients only did not allow us to explore the impact of the developmental trajectories proposed by others [11,12] on the resulting phenotype.”
From Severity classes in adults with hypermobile Ehlers–Danlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients (2019) · cites this paper as [11]
Those subphenotypes were recognized by the prevalence of selected items such as BMI by age and number of painful joints.
“These categories were recognized by the prevalence of selected items, such as BMI by age and number of painful joints.”
From Severity classes in adults with hypermobile Ehlers–Danlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients (2019) · cites this paper as [11]
In that study, patients with the high BMI subphenotype experienced more pain, while those with the soft tissue affected subphenotype reported more fatigue.
“Some correlations with selected QoL determinants also emerged, as patients with the high BMI subphenotype experienced more pain while those with the soft tissue affected subphenotype reported more fatigue [11].”
From Severity classes in adults with hypermobile Ehlers–Danlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients (2019) · cites this paper as [11]
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Self-citation
- Authors
- Pacey, V., Adams, R. D., Tofts, L., Munns, C. F., & Nicholson, L. L.
- Affiliations
- The University of Sydney (AU)
- Corresponding author
- Pacey, Verity
- Venue
- Archives of Disease in Childhood
- Publisher
- BMJ
- Published
- 2014-05-26
- Volume
- 100
- Issue
- 1
- Pages
- 8-13
- ISSN
- 0003-9888 (ISSN-L)
- 1468-2044
- OpenAlex
- W2110358685
- Cited by
- 40
- References
- 44
- Citations by year
- 2024: 1 · 2023: 3 · 2022: 2 · 2021: 3 · 2020: 4 · 2019: 6 · 2018: 3 · 2017: 8 · 2016: 5 · 2015: 4 · 2014: 1
- FWCI
- 3.93
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Archives of Disease in Childhood (publishedVersion)
- PubMed (publishedVersion)
- HighWire Press Open Archive (submittedVersion)
- Funders
- Menzies Foundation
- University of Sydney
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Pharmacology › Musculoskeletal pain and rehabilitation
- Health Sciences › Health Professions › Pharmacy › Oral and gingival health research
- Keywords
- joint hypermobility syndrome, pediatric patients, clinical subtypes, joint pain, joint instability, health-related quality of life, fatigue, soft tissue injury, skin involvement, body mass index, exploratory factor analysis
- MeSH terms
- Adolescent, Child, Female, Humans, Joint Instability, Male, Quality of Life, Severity of Illness Index
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