title: Quality of life prediction in children with joint hypermobility syndrome.
abstract: OBJECTIVE
  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 < 0.001); however, parents of children with JHS perceived lower overall HRQOL (mean difference = 4.44, P = 0.001), physical (mean difference = 7.11, P < 0.0001) and emotional functioning (mean difference = 5.24, P = 0.011) than their children. When considered together with previously reported HRQOL scores for children with other chronic conditions, parent and child scores were similarly strongly correlated (r = 0.93, P = 0.001). Multiple regression revealed that 75% of the variance in child-reported HRQOL scores was accounted for by a child's level of pain and fatigue, and presence of stress incontinence symptoms (P < 0.0001).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Physiotherapy Department, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.|Kids Rehab, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.|Discipline of Biomedical Sciences, Sydney Medical School, The University of Sydney, Sydney, New South Wales, Australia.|Department of Health Professions, Macquarie University, Sydney, New South Wales, Australia.
authors.forename: Verity
authors.initials: V
authors.lastname: Pacey
authors.affiliation: Kids Rehab, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.|Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, Sydney, New South Wales, Australia.
authors.forename: Louise
authors.initials: L
authors.lastname: Tofts
authors.affiliation: Discipline of Physiotherapy, Faculty of Health Sciences, The University of Sydney, Sydney, New South Wales, Australia.
authors.forename: Roger D
authors.initials: RD
authors.lastname: Adams
authors.affiliation: Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, Sydney, New South Wales, Australia.|Endocrinology Department, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.
authors.forename: Craig F
authors.initials: CF
authors.lastname: Munns
authors.affiliation: Kids Rehab, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.|Discipline of Biomedical Sciences, Sydney Medical School, The University of Sydney, Sydney, New South Wales, Australia.
authors.forename: Leslie L
authors.initials: LL
authors.lastname: Nicholson
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002908:Chronic Disease; D059350:Chronic Pain / Q000175:diagnosis / Q000209:etiology; D004535:Ehlers-Danlos Syndrome / Q000150:complications / Q000503:physiopathology / Q000523:psychology; D005221:Fatigue / Q000175:diagnosis / Q000209:etiology; D005260:Female; D006305:Health Status Indicators; D006801:Humans; D007593:Joint Instability / Q000150:complications / Q000151:congenital* / Q000503:physiopathology / Q000523:psychology; D008297:Male; D010290:Parents; D011788:Quality of Life; D057566:Self Report
keywords: Ehlers-Danlos syndrome; fatigue; hypermobility; joint laxity; pain; quality of life