Key result
Fabry disease linked to ~12% thicker carotid IMT and impaired brachial FMD versus healthy controls.
Why the study?
Does Fabry disease affect peripheral arterial function and structure compared to healthy controls?
Case-Control (n=51)
Does Fabry disease affect peripheral arterial function and structure compared to healthy controls?
Absolute Event Rate: 0.64% vs 0.57%
p-value: p=<0.05
Fabry disease is associated with increased intima-media thickness and impaired flow-mediated dilatation in peripheral arteries, indicating disturbed endothelial function and structural changes.
Subclinical vascular changes may occur in Fabry disease; hypothesis-generating and should not yet change practice.
OBJECTIVE: Fabry disease is a lysosomal storage disorder due to deficient alpha-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.
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Kalliokoski et al. (2006) conducted a case-control in Fabry disease (n=51). Fabry disease vs. Healthy controls was evaluated on Carotid intima-media thickness (mm) (p=<0.05). Fabry disease was associated with significantly increased carotid intima-media thickness (0.64 vs 0.57 mm) and impaired brachial flow-mediated dilatation (6.3% vs 9.7%) compared to healthy controls.
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