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Rheumatology (Oxford). 2017 Dec 1;56(12):2073-2083. doi: 10.1093/rheumatology/kex148.

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

Author information

1
ACHIEVE, Center for Applied Research, Faculty of Health, University of Applied Sciences Amsterdam.
2
Department of Rehabilitation, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
3
Kids Rehab, The Children's Hospital at Westmead.
4
Discipline of Biomedical Sciences.
5
Discipline of Physiotherapy, University of Sydney.
6
Narrabeen Sports Medicine Centre, Sydney Academy of Sport.
7
Discipline of Paediatrics and Child Health, University of Sydney.
8
Department of Health Professions, Macquarie University.
9
Department of Endocrinology, The Children's Hospital at Westmead, Sydney, NSW, Australia.

Abstract

Objectives:

The objective of the manuscript was to describe the natural history of complaints and disability in children diagnosed with joint hypermobility syndrome (JHS)/Ehlers-Danlos-hypermobility type (EDS-HT) and to identify the constructs that underlie functional decline.

Methods:

One hundred and one JHS/EDS-HT children were observed over 3 years and assessed at three time points on the following: functional impairments, quality of life, connective tissue laxity, muscle function, postural control and musculoskeletal and multi-systemic complaints. Cluster analysis was performed to identify subgroups in severity. Clinical profiles were determined for these subgroups, and differences were assessed by multivariate analysis of covariance. Mixed linear regression models were used to determine the subsequent trajectories. Finally, an exploratory factor analysis was used to uncover the underlying constructs of functional impairment.

Results:

Three clusters of children were identified in terms of functional impairment: mild, moderately and severely affected. Functional impairment at baseline was predictive of worsening trajectories in terms of reduced walking distance and decreased quality of life (P ⩽ 0.05) over 3 years. Multiple interactions between the secondary outcomes were observed, with four underlying constructs identified. All four constructs (multi-systemic effects, pain, fatigue and loss of postural control) contributed significantly to disability (P ⩽ 0.046).

Conclusion:

Children diagnosed with JHS/EDS-HT who have a high incidence of multi-systemic complaints (particularly, orthostatic intolerance, urinary incontinence and diarrhoea) and poor postural control in addition to high levels of pain and fatigue at baseline are most likely to have a deteriorating trajectory of functional impairment and, accordingly, warrant clinical prioritization.

KEYWORDS:

Ehlers–Danlos (hypermobility type); disability; fatigue; hypermobility syndrome; multi-systemic dysfunction; natural course; pain

PMID:
28431150
DOI:
10.1093/rheumatology/kex148
[Indexed for MEDLINE]

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