caa a22 4500 467933715 CHVBK 20180406152950.0 cr unu---uuuuu 170328e20061001xx s 000 0 eng 10.1007/s10545-006-0340-x doi (NATIONALLICENCE)springer-10.1007/s10545-006-0340-x Structural and functional changes in peripheral vasculature of Fabry patients [Elektronische Daten] [Riikka Kalliokoski, Kari Kalliokoski, Maila Penttinen, Ilkka Kantola, Aila Leino, Jorma Viikari, Olli Simell, Pirjo Nuutila, Olli Raitakari] Summary: Objective: Fabry disease is a lysosomal storage disorder due to deficient α-galactosidase A activity, which leads to glycosphingolipid accumulation especially in vascular smooth-muscle and endothelial cells. Little is known about the effects of Fabry disease on peripheral artery function and structure. Therefore, we aimed to further characterize the peripheral vascular structural and functional changes in Fabry disease. Methods and results: We measured structural and functional vascular parameters, including intima-media thickness (IMT) of brachial and carotid arteries and abdominal aorta, carotid and aortic compliance, and brachial artery flow-mediated dilatation (FMD) in 17 Fabry patients and 34 healthy controls matched for age, sex and smoking. Carotid IMT (0.64 ± 0.15 vs 0.57 ± 0.12 mm), brachial IMT (1.02 ± 0.25 vs 0.74 ± 0.18 mm), and aortic IMT (0.31 ± 0.09 vs 0.26 ± 0.04 mm) were significantly increased, and brachial FMD was significantly impaired (6.3 ± 5.0 vs 9.7 ± 3.9%) in Fabry patients compared to healthy controls (p < 0.05 in all comparisons after adjustments for age, LDL-cholesterol, and systolic blood pressure). No differences were observed in arterial compliance between the groups. Conclusions: These data suggest that Fabry disease affects arterial function and structure by disturbing peripheral endothelial function and promoting intima-media thickening. SSIEM and Springer, 2006 Kalliokoski Riikka Turku PET Centre, University of Turku, Turku, Finland aut Kalliokoski Kari Turku PET Centre, University of Turku, Turku, Finland aut Penttinen Maila Clinical Genetics Unit, Department of Pediatrics, University of Turku, Turku, Finland aut Kantola Ilkka Department of Medicine, University of Turku, Turku, Finland aut Leino Aila Department of Clinical Chemistry, Turku University Central Hospital, Turku, Finland aut Viikari Jorma Department of Medicine, University of Turku, Turku, Finland aut Simell Olli Department of Pediatrics, University of Turku, Turku, Finland aut Nuutila Pirjo Turku PET Centre, University of Turku, Turku, Finland aut Raitakari Olli Department of Clinical Physiology, University of Turku, Turku, Finland aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/5(2006-10-01), 660-666 0141-8955 29:5<660 2006 29 10545 https://doi.org/10.1007/s10545-006-0340-x text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10545-006-0340-x text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kalliokoski Riikka Turku PET Centre, University of Turku, Turku, Finland aut NATIONALLICENCE 700 1- Kalliokoski Kari Turku PET Centre, University of Turku, Turku, Finland aut NATIONALLICENCE 700 1- Penttinen Maila Clinical Genetics Unit, Department of Pediatrics, University of Turku, Turku, Finland aut NATIONALLICENCE 700 1- Kantola Ilkka Department of Medicine, University of Turku, Turku, Finland aut NATIONALLICENCE 700 1- Leino Aila Department of Clinical Chemistry, Turku University Central Hospital, Turku, Finland aut NATIONALLICENCE 700 1- Viikari Jorma Department of Medicine, University of Turku, Turku, Finland aut NATIONALLICENCE 700 1- Simell Olli Department of Pediatrics, University of Turku, Turku, Finland aut NATIONALLICENCE 700 1- Nuutila Pirjo Turku PET Centre, University of Turku, Turku, Finland aut NATIONALLICENCE 700 1- Raitakari Olli Department of Clinical Physiology, University of Turku, Turku, Finland aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/5(2006-10-01), 660-666 0141-8955 29:5<660 2006 29 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer