Arterial wall properties and womersley flow in fabry disease

被引：24

作者：

Moore D.F. ^{[1
]}

Altarescu G. ^{[1
]}

Pursley R. ^{[2
]}

Campia U. ^{[4
]}

Panza J.A. ^{[4
]}

Dimitriadis E. ^{[3
]}

Schiffmann R. ^{[1
]}

机构：

[1] Devmtl./Metabolic Neurology Branch, Natl. Inst. of Neurological Disorder, National Institutes of Health, Bethesda, MD

[2] Center for Information Technology, National Institutes of Health, Bethesda, MD

[3] Div. of Bioeng. and Physical Science, Office for Research Services, National Institutes of Health, Bethesda, MD

[4] Cardiovascular Research Institute, Washington Hospital Center, Washington, DC

来源：

BMC Cardiovascular Disorders | / 2卷 / 1期

关键词：

Radial Artery; Fabry Disease; Sodium Nitroprusside; Fabry Patient; Arterial Wall Thickness;

D O I：

10.1186/1471-2261-2-1

中图分类号：

学科分类号：

摘要：

Background: Fabry disease is an X-linked recessive lysosomal storage disease resulting in the cellular accumulation of globotriaosylceramide particularly globotriaosylceramide. The disease is characterized by a dilated vasculopathy with arterial ectasia in muscular arteries and arterioles. Previous venous plethysomographic studies suggest enhanced endothelium-dependent vasodilation in Fabry disease indicating a functional abnormality of resistance vessels. Methods: We examined the mechanical properties of the radial artery in Fabry disease, a typical fibro-muscular artery. Eight control subjects and seven patients with Fabry disease had a right brachial arterial line placed allowing real time recording of intra-arterial blood pressure. Real time B-mode ultrasound recordings of the right radial artery were obtained simultaneously allowing calculation of the vessel wall internal and external diameter, the incremental Young's modulus and arterial wall thickness. By simultaneously measurement of the distal index finger-pulse oximetry the pulse wave speed was calculated. From the wave speed and the internal radial artery diameter the volume flow was calculated by Womersley analysis following truncation of the late diastolic phase. Results: No significant difference was found between Fabry patients and controls for internal or external arterial diameters, the incremental Young's modulus, the arterial wall thickness, the pulse wave speed and the basal radial artery blood flow. Further, no significant difference was found for the radial artery blood flow in response to intra-arterial acetylcholine or sodium nitroprusside. Both drugs however, elevated the mean arterial flow. Conclusions: The current study suggests that no structural or mechanical abnormality exists in the vessel wall of fibro-muscular arteries in Fabry disease. This may indicate that a functional abnormality downstream to the conductance vessels is the dominant feature in development Fabry vasculopathy. © 2002 Moore et al; licensee BioMed Central Ltd.

引用

页数：6

共 9 条

[1]

Brady R., Gal A.E., Bradley R.M., Martensson E., Warshaw A.L., Laster L., Enzymatic defect in Fabry disease: Ceramide trihexosidase deficiency, N. Engl. J. Med., 276, pp. 1163-1167, (1967)

[2]

Desnick R., Ioannou Y.A., Eng C.M., α-Galactosidase A Deficiency: Fabry Disease, The Metabolic and Molecular Bases of Inherited Disease, pp. 3733-3774, (2001)

[3]

Mitsias P., Levine S.R., Cerebrovascular complications of Fabry's disease, Ann. Neurol., 40, pp. 8-17, (1996)

[4]

Nichols W.W., O'Rourke M.F., McDonald's Blood Flow in Arteries Theoretical, Experimental and Clinical Principles, (1998)

[5]

Womersley J.R., Oscillatory motion of a viscous liquid in a thinwalled elastic tube: I. The linear approximation for long waves, Philosophical Magazine, 46, pp. 199-221, (1955)

[6]

Furchotts R.F., Zawadzki J.V., The obligatory role of endothelial cells in the relaxation of arterial smooth muscle by acetylcholine, Nature, 288, pp. 373-376, (1980)

[7]

Moore D.F., Pursley R., Altarescu G., Schiffmann R., Dimitriadis E., Real Time Peripheral Arterial Flow and Wall Properties derived by Pulse Waveform Analysis and B-mode Ultrasound Imaging, 14th IEEE Conference on Computer-Based Medical Systems, pp. 216-221, (2001)

[8]

Boutouyrie P., Laurent S., Laloux B., Lidove O., Grunfeld J.P., Germain D.P., Non-invasive evaluation of arterial involvement in patients affected with Fabry disease, J. Med. Genet., 38, pp. 629-631, (2001)

[9]

Altarescu G., Moore D.F., Pursley R., Campia U., Goldstein S., Bryant M., Panza J.A., Schiffmann R., Enhanced endothelium-dependent vasodilation in Fabry disease, Stroke, 32, pp. 1559-1562, (2001)

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