3.4 A PRESSURE-INDEPENDENT ARTERIAL REMODELLING AND AORTA DILATATION IN TREATED PATIENTS WITH FABRY DISEASE
Purpose: Fabry disease is a deficiency of lysosomal enzyme α-galactosidase A leading to accumulation of glycosphyngolipids in cardiac and vascular tissues. After long-term enzyme replacement treatment, we described a continuous vascular hypertrophy whereas the aortic stiffness was paradoxically decr...
Main Authors: | , , , , , , , |
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Format: | Article |
Language: | English |
Published: |
Atlantis Press
2009-12-01
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Series: | Artery Research |
Online Access: | https://www.atlantis-press.com/article/125927364/view |