title: Generalized Hyperalgesia in Children and Adults Diagnosed With Hypermobility Syndrome and Ehlers-Danlos Syndrome Hypermobility Type: A Discriminative Analysis.
abstract: Lowered pressure-pain thresholds have been demonstrated in adults with Ehlers-Danlos syndrome hypermobility type (EDS-HT), but whether these findings are also present in children is unclear. Therefore, the objectives of the study were to determine whether generalized hyperalgesia is present in children with hypermobility syndrome (HMS)/EDS-HT, explore potential differences in pressure-pain thresholds between children and adults with HMS/EDS-HT, and determine the discriminative value of generalized hyperalgesia.
  
  
  
  Patients were classified in 1 of 3 groups: HMS/EDS-HT, hypermobile (Beighton score ≥4 of 9), and healthy controls. Descriptive data of age, sex, body mass index, Beighton score, skin laxity, and medication usage were collected. Generalized hyperalgesia was quantified by the average pressure-pain thresholds collected from 12 locations. Confounders collected were pain locations/intensity, fatigue, and psychological distress. Comparisons between children with HMS/EDS-HT and normative values, between children and adults with HMS/EDS-HT, and corrected confounders were analyzed with multivariate analysis of covariance. The discriminative value of generalized hyperalgesia employed to differentiate between HMS/EDS-HT, hypermobility, and controls was quantified with logistic regression.
  
  
  
  Significantly lower pressure-pain thresholds were found in children with HMS/EDS-HT compared to normative values (range -22.0% to -59.0%; P ≤ 0.05). When applying a threshold of 30.8 N/cm2for males and 29.0 N/cm2for females, the presence of generalized hyperalgesia discriminated between individuals with HMS/EDS-HT, hypermobility, and healthy controls (odds ratio 6.0).
  
  
  
  Children and adults with HMS/EDS-HT are characterized by hypermobility, chronic pain, and generalized hyperalgesia. The presence of generalized hyperalgesia may indicate involvement of the central nervous system in the development of chronic pain.
authors.affiliation: University of Applied Sciences and Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: M C
authors.initials: MC
authors.lastname: Scheper
authors.affiliation: The Children's Hospital at Westmead and Macquarie University, Sydney, New South Wales, Australia.
authors.forename: V
authors.initials: V
authors.lastname: Pacey
authors.affiliation: Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium.
authors.forename: L
authors.initials: L
authors.lastname: Rombaut
authors.affiliation: University of Sydney, Sydney, New South Wales, Australia.
authors.forename: R D
authors.initials: RD
authors.lastname: Adams
authors.affiliation: The Children's Hospital at Westmead and University of Sydney, Sydney, New South Wales, Australia.
authors.forename: L
authors.initials: L
authors.lastname: Tofts
authors.affiliation: Ghent University Ghent, Belgium.
authors.forename: P
authors.initials: P
authors.lastname: Calders
authors.affiliation: The Children's Hospital at Westmead and University of Sydney, Sydney, New South Wales, Australia.
authors.forename: L L
authors.initials: LL
authors.lastname: Nicholson
authors.affiliation: University of Applied Sciences and Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: R H H
authors.initials: RH
authors.lastname: Engelbert
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001530:Belgium; D001696:Biomechanical Phenomena; D016022:Case-Control Studies; D002648:Child; D059350:Chronic Pain / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D016002:Discriminant Analysis; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology / Q000523:psychology; D005260:Female; D006801:Humans; D006930:Hyperalgesia / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D007593:Joint Instability / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology / Q000523:psychology; D007596:Joints / Q000503:physiopathology*; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009426:Netherlands; D009517:New South Wales; D016017:Odds Ratio; D010147:Pain Measurement; D017288:Pain Threshold; D011237:Predictive Value of Tests; D012307:Risk Factors; D055815:Young Adult