Related Experiment Videos
Peripheral hemodynamics in patients with Fabry's disease
American Heart Journal
|May 1, 1983
Summary
Fabry's disease impairs peripheral circulation, showing higher vascular resistance and lower blood flow in extremities. This suggests glycolipid accumulation and sympathoadrenal activity contribute to the disease's vascular dysfunction.
Area of Science:
- Vascular Biology
- Cardiovascular Physiology
- Rare Genetic Disorders
Background:
- Fabry disease (angiokeratoma corporis diffusum universale) is characterized by altered vasomotor activity.
- Cardiovascular involvement is common in Fabry disease, but peripheral hemodynamic effects are poorly understood.
Purpose of the Study:
- To investigate peripheral hemodynamics in the extremities and digits of Fabry disease patients.
- To compare vascular function in Fabry disease patients with healthy controls.
Main Methods:
- Segmental and venous occlusion pneumoplethysmography were used to assess peripheral hemodynamics.
- Thermal probes measured digital temperature.
- Vasodilation procedures were employed to evaluate circulatory response.
Main Results:
- Fabry patients exhibited significantly higher forearm vascular resistance and lower venous capacitance compared to controls.
- Reduced resting and post-vasodilation finger and toe blood flow and pulse volume were observed in Fabry patients.
- Despite similar digital temperature increases after vasodilation, blood flow and pulse volume remained lower in Fabry patients.
Conclusions:
- Findings indicate a vasoconstrictive process in resistance and capacitance vessels within cutaneous and skeletal muscle beds in Fabry disease.
- A limited cutaneous circulation response to vasodilation suggests impaired vascular function.
- Glycolipid accumulation and enhanced sympathoadrenal discharge may play key roles in Fabry disease pathophysiology.