The natural history of children with joint hypermobility syndrome and Ehlers–Danlos hypermobility type: a longitudinal cohort study
- 18 April 2017
- journal article
- research article
- Published by Oxford University Press (OUP) in Rheumatology
- Vol. 56 (12), 2073-2083
- https://doi.org/10.1093/rheumatology/kex148
Abstract
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. 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. 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). 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.Funding Information
- NWO (023.002.094)
This publication has 32 references indexed in Scilit:
- The role of benign joint hypermobility in the pain experience in Juvenile Fibromyalgia: an observational studyPediatric Rheumatology, 2012
- Muscle mass, muscle strength, functional performance, and physical impairment in women with the hypermobility type of Ehlers‐Danlos syndromeArthritis Care & Research, 2012
- Muscle–tendon tissue properties in the hypermobility type of Ehlers‐Danlos syndromeArthritis Care & Research, 2012
- The PedsQL™ Multidimensional Fatigue Scale in pediatric obesity: Feasibility, reliability and validityPediatric Obesity, 2010
- Motor Competence and Physical Activity in 8-Year-Old School Children With Generalized Joint HypermobilityPEDIATRICS, 2009
- The lack of clinical distinction between the hypermobility type of Ehlers–Danlos syndrome and the joint hypermobility syndrome (a.k.a. hypermobility syndrome)American Journal of Medical Genetics Part A, 2009
- Six‐minute walk test in obese children and adolescents: Reproducibility and validityPhysiotherapy Research International, 2008
- Musculoskeletal reflex function in the joint hypermobility syndromeArthritis Care & Research, 2007
- Inter-examiner reproducibility of tests and criteria for generalized joint hypermobility and benign joint hypermobility syndromeRheumatology, 2007
- Normal scores for nine maneuvers of the Childhood Myositis Assessment ScaleArthritis Care & Research, 2004