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Orthostatic intolerance & Chronic Fatigue Syndrome (CFS) associated with Ehlers-Danlos syndrome

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OBJECTIVE: To report chronic fatigue syndrome (CFS) associated

with both Ehlers-Danlos syndrome (EDS) and orthostatic


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


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.

Rowe PC, Barron DF, Calkins H, Maumenee IH, Tong PY, Geraghty MT

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