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Severe lymphatic microangiopathy in Fabry disease
Beatrice R Amann-Vesti1, Gabriele Gitzelmann, Urs Widmer
1University Hospital of Zurich, Zurich, Switzerland. beatrice.amann@usz.ch
Lymphatic Research and Biology
|December 31, 2004
Summary
Fabry disease patients exhibit significant structural and functional changes in skin lymphatic microvessels, potentially explaining lymphedema. This study investigated lymphatic morphology and function in Fabry disease.
Area of Science:
- Vascular Biology
- Genetics
- Dermatology
Background:
- Lymphedema is occasionally observed in Fabry disease patients, but its underlying cause remains unclear.
- Fabry disease is a rare genetic disorder affecting multiple organ systems.
Purpose of the Study:
- To investigate the morphology and function of lymphatic microvessels in individuals with Fabry disease.
- To explore the relationship between lymphatic abnormalities and lymphedema in Fabry disease.
Main Methods:
- In vivo fluorescence microlymphography was used to study skin lymphatic microvessels.
- Lymph capillary pressure was measured in male and female Fabry disease patients and healthy controls.
Main Results:
- Patients with Fabry disease showed increased dye spread in lymphatic networks compared to controls.
- All Fabry disease patients displayed fragmentation of the microlymphatic network, unlike controls.
- Microlymphatic hypertension was observed in Fabry disease patients with lymphedema.
Conclusions:
- Fabry disease is associated with severe structural and functional alterations in the initial lymphatic microvessels of the skin.
- These lymphatic changes likely contribute to the development of lymphedema in Fabry disease.