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

  [ 46 votes ]   [ Discuss This Article ]
www.ProHealth.com • October 15, 1999


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.

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




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