TY - JOUR
T1 - Orthostatic intolerance and chronic fatigue syndrome associated with Ehlers-Danlos syndrome
AU - Rowe, Peter C.
AU - Barron, Diana F.
AU - Calkins, Hugh
AU - Maumenee, Irene H.
AU - Tong, Patrick Y.
AU - Geraghty, Michael T.
N1 - Funding Information:
Supported in part by generous donations from the Boies family, Jacqueline Wolfe, and the friends of Polly Kelly and by grants from the Sunshine Foundation and the National Institutes of Health (Dr Rowe, AI 39500).
PY - 1999
Y1 - 1999
N2 - Objective: To report chronic fatigue syndrome (CFS) associated with both Ehlers-Danlos syndrome (EDS) and orthostatic intolerance. Study design: 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.
AB - Objective: To report chronic fatigue syndrome (CFS) associated with both Ehlers-Danlos syndrome (EDS) and orthostatic intolerance. Study design: 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.
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U2 - 10.1016/S0022-3476(99)70173-3
DO - 10.1016/S0022-3476(99)70173-3
M3 - Article
C2 - 10518084
AN - SCOPUS:0033497847
SN - 0022-3476
VL - 135
SP - 494
EP - 499
JO - Journal of Pediatrics
JF - Journal of Pediatrics
IS - 4
ER -