title: Orthostatic intolerance and chronic fatigue syndrome associated with Ehlers-Danlos syndrome.
abstract: OBJECTIVE
  To report chronic fatigue syndrome (CFS) associated with both Ehlers-Danlos syndrome (EDS) and orthostatic intolerance.
  
  
  METHODS
  Case series of adolescents referred to a tertiary clinic for the evaluation of CFS. All subjects had 2-dimensional echocardiography, tests of orthostatic tolerance, and examinations by both a geneticist and an ophthalmologist.
  
  
  RESULTS
  Twelve patients (11 female), median age 15.5 years, met diagnostic criteria for CFS and EDS, and all had either postural tachycardia or neurally mediated hypotension in response to orthostatic stress. Six had classical-type EDS and 6 had hypermobile-type EDS.
  
  
  CONCLUSIONS
  Among patients with CFS and orthostatic intolerance, a subset also has EDS. We propose that the occurrence of these syndromes together can be attributed to the abnormal connective tissue in dependent blood vessels of those with EDS, which permits veins to distend excessively in response to ordinary hydrostatic pressures. This in turn leads to increased venous pooling and its hemodynamic and symptomatic consequences. These observations suggest that a careful search for hypermobility and connective tissue abnormalities should be part of the evaluation of patients with CFS and orthostatic intolerance syndromes.
authors.affiliation: Department of Pediatrics, Center for Hereditary Eye Diseases, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: P C
authors.initials: PC
authors.lastname: Rowe
authors.forename: D F
authors.initials: DF
authors.lastname: Barron
authors.forename: H
authors.initials: H
authors.lastname: Calkins
authors.forename: I H
authors.initials: IH
authors.lastname: Maumenee
authors.forename: P Y
authors.initials: PY
authors.lastname: Tong
authors.forename: M T
authors.initials: MT
authors.lastname: Geraghty
mesh_terms: D000293:Adolescent; D000328:Adult; D001794:Blood Pressure; D002648:Child; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis; D015673:Fatigue Syndrome, Chronic / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006339:Heart Rate; D006801:Humans; D007024:Hypotension, Orthostatic / Q000150:complications* / Q000175:diagnosis; D008297:Male