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首页> 外文期刊>Thrombosis Research: An International Journal on Vascular Obstruction, Hemorrhage and Hemostasis >Sulfatide-binding assay for von Willebrand factor. Detection of von Willebrand's disease without discrimination of vWD subtypes.
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Sulfatide-binding assay for von Willebrand factor. Detection of von Willebrand's disease without discrimination of vWD subtypes.

机译：von Willebrand因子的硫酸盐结合测定。在不区分vWD亚型的情况下检测von Willebrand病。

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The appropriate diagnosis of von Willebrand's disease requires a panel of laboratory assays. Subclassification of a patient's von Willebrand's disease is also important as it will influence management. The minimal test panel should comprise an assay for factor VIII coagulant, assessment of von Willebrand factor antigen and at least one functional von Willebrand factor assay (collagen-binding assay and/or Ristocetin cofactor assay). This panel will provisionally determine whether von Willebrand's disease is present and whether the von Willebrand's disease provisionally detected is a qualitative (Type 2) or quantitative (Types 1 or 3) defect, and results will help guide further investigation and the need for further testing. In view of reports indicating that sulfatides bind to von Willebrand factor by a specific high-affinity mechanism distinct from collagen, and with affinity reportedly dependent on von Willebrand factor size, the author has devised and tested a novel enzyme linked immunosorbant assay (ELISA)-based von Willebrand factor sulfatide-binding assay for its potential use in von Willebrand's disease diagnosis and von Willebrand's disease subtype discrimination. Results obtained using this assay were compared with those using a standard von Willebrand factor antigen assay (by ELISA), a Ristocetin cofactor assay for von Willebrand factor (by platelet agglutination procedure), and a collagen-binding assay for von Willebrand factor (by ELISA). All four von Willebrand factor assays were capable of detecting von Willebrand factor in a dose-dependent manner (i.e., were von Willebrand factor quantitative), and all were capable of detecting deficiencies in von Willebrand factor associated with von Willebrand's disease (i.e., all could detect the presence of von Willebrand's disease; Type 1 and Type 2 von Willebrand's disease). Linear regression analysis showed good correlation of results of sulfatide-binding assay for von Willebrand factor with results of the von Willebrand factor antigen, Ristocetin cofactor assay for von Willebrand factor, and collagen-binding assay for von Willebrand factor. However, only the collagen-binding assay for von Willebrand factor and Ristocetin cofactor assay for von Willebrand factor could detect the presence of von Willebrand factor functional discordance associated with Type 2 von Willebrand's disease. That is, in the current study, the sulfatide-binding assay for von Willebrand factor yielded laboratory data for von Willebrand's disease patients that was essentially similar to that obtained using the von Willebrand factor antigen assay, and which therefore could not be used to discriminate between von Willebrand's disease subtypes.
机译：von Willebrand病的适当诊断需要一组实验室测定。患者的von Willebrand病的亚分类也很重要，因为它将影响管理。最低限度的测试应包括凝血因子VIII凝血测定，von Willebrand因子抗原评估和至少一种功能性Will Willebrand因子测定（胶原结合测定和/或Ristocetin辅助因子测定）。该小组将临时确定是否存在von Willebrand病，以及临时检测到的von Willebrand病是定性（2型）还是定量（1型或3型）缺陷，其结果将有助于指导进一步的研究和进一步测试的必要性。鉴于有报道指出，硫化物通过不同于胶原蛋白的特定高亲和力机制与von Willebrand因子结合，并且据报道亲和力取决于von Willebrand因子大小，因此作者设计并测试了一种新型的酶联免疫吸附测定（ELISA）-基于von Willebrand因子硫酸盐结合试验，可用于von Willebrand的疾病诊断和von Willebrand的疾病亚型鉴别。将使用此测定法获得的结果与使用标准von Willebrand因子抗原测定法（通过ELISA），使用Ristocetin辅酶测定von Willebrand因子测定的结果（通过血小板凝集法）和胶原结合测定von Willebrand因子测定的结果（通过ELISA）进行比较）。所有四种von Willebrand因子测定均能够以剂量依赖的方式检测von Willebrand因子（即von Willebrand因子定量），并且均能够检测与von Willebrand病相关的von Willebrand因子缺乏症（即，所有检测von Willebrand病; 1型和2型von Willebrand病的存在）。线性回归分析显示，von Willebrand因子的硫酸盐结合测定结果与von Willebrand因子抗原的抗原测定结果，von Willebrand因子的Ristocetin辅因子测定结果和von Willebrand因子的胶原结合测定结果具有良好的相关性。但是，只有针对von Willebrand因子的胶原蛋白结合测定和针对von Willebrand因子的利斯托菌素辅助因子测定才能检测到与2型von Willebrand病相关的von Willebrand因子功能失调。就是说，在当前的研究中，von Willebrand因子的硫酸盐结合测定产生了von Willebrand病患者的实验室数据，该数据与使用von Willebrand因子抗原测定获得的数据基本相似，因此不能用于区分von Willebrand病亚型。

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