Arterial wall properties and Womersley flow in Fabry disease.
Arterial wall properties and Womersley flow in Fabry disease.
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DOI：
10.1186/1471-2261-2-1
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发表时间：
2002-01-01
影响因子：
2.1
通讯作者：
Schiffmann, Raphael
中科院分区：
文献类型：
--
作者：
Moore, David F;Altarescu, Gheona;Schiffmann, Raphael
背景技术背景：法布里病是一种X-连锁隐性溶酶体贮积病，导致神经酰胺三己糖苷（尤其是神经酰胺三己糖苷）的细胞蓄积。这种疾病的特征是扩张性血管病变，伴有肌肉动脉和小动脉的动脉扩张。以前的静脉体积描记研究表明，增强内皮依赖性血管舒张法布雷病表明阻力vessels.METHODS的功能异常：我们研究了机械性能的桡动脉法布雷病，一个典型的纤维肌动脉。8名对照受试者和7名法布里病患者放置了右肱动脉管路，允许真实的时间记录动脉内血压。同时获得右桡动脉的真实的实时B型超声记录，从而计算血管壁内径和外径、增量杨氏模量和动脉壁厚度。通过同时测量远端食指脉搏血氧饱和度，计算脉搏波速度。从波的速度和内径桡动脉的体积流量计算Womersley分析截断后的舒张晚期phase.Results：法布里病患者和对照组之间没有显着差异，发现内部或外部的动脉直径，增量杨氏模量，动脉壁厚度，脉搏波速度和基底桡动脉血流量。此外，桡动脉血流对动脉内乙酰胆碱或硝普钠的反应无显著差异。然而，这两种药物，提高了平均动脉flow.CONCLUSIONS：目前的研究表明，没有结构或机械异常存在于法布里病的纤维肌动脉血管壁。这可能表明传导血管下游的功能异常是法布里病血管病变发展的主要特征。
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.