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重温血管性血友病的谱系。

The spectrum of von Willebrand's disease revisited.

作者信息

Bowie E J, Fass D N, Olson J D, Owen C A

出版信息

Mayo Clin Proc. 1976 Jan;51(1):35-41.

PMID:765637
Abstract

We have examined nine patients with presumed von Willebrand's disease who present the spectrum of that disorder. Two had findings that would be accepted generally as diagnostic of von Willebrand's disease, and seven had variations of the usual pattern. The commonest variation was the combination of borderline and variable levels of coagulant Factor VIII, commensurate levels of Factor VIII-related antigen, and low levels of ristocetin-Willebrand factor.

摘要

我们检查了9名疑似患血管性血友病的患者,这些患者呈现出该病症的一系列症状。其中两名患者的检查结果通常可被认定为血管性血友病的诊断依据,另外七名患者则有不同于常见模式的情况。最常见的差异是凝血因子VIII水平处于临界值且不稳定、VIII相关抗原水平相当,以及瑞斯托菌素-血管性血友病因子水平较低。

相似文献

1
The spectrum of von Willebrand's disease revisited.重温血管性血友病的谱系。
Mayo Clin Proc. 1976 Jan;51(1):35-41.
2
[Von Willebrand's disease. Studies on platelet function, ristocetin induced platelet aggregation and factor VIII related antigen (author's transl)].[血管性血友病。血小板功能、瑞斯托霉素诱导的血小板聚集及 VIII 因子相关抗原的研究(作者译)]
Rinsho Ketsueki. 1975 Aug;16(8):775-84.
3
Diagnosis of von Willebrand's disease. A comparative study of diagnostic tests on nine families with von Willebrand's disease and its differential diagnosis from hemophilia and thrombocytopathy.血管性血友病的诊断。对九个血管性血友病家族的诊断试验及其与血友病和血小板病的鉴别诊断的比较研究。
Am J Med. 1976 Mar;60(3):344-56. doi: 10.1016/0002-9343(76)90750-6.
4
[Diagnosis of von Willebrand's disease].[血管性血友病的诊断]
Med Klin. 1978 Jun 2;73(22):833-8.
5
[Diagnosis of von Willebrand's disease].[血管性血友病的诊断]
Bilt Hematol Transfuz. 1977;5(3-4):41-61.
6
Re-evaluation of plasmas from patients previously diagnosed as having von Willebrand's disease with the factor VIII-related antigen and ristocetin cofactor assay.通过因子VIII相关抗原和瑞斯托霉素辅因子测定法对先前诊断为血管性血友病患者的血浆进行重新评估。
Am J Clin Pathol. 1979 Jan;71(1):26-30. doi: 10.1093/ajcp/71.1.26.
7
Quantitative assay of a plasma factor deficient in von Willebrand's disease that is necessary for platelet aggregation. Relationship to factor VIII procoagulant activity and antigen content.对血管性血友病中缺乏的、血小板聚集所必需的一种血浆因子进行定量测定。与凝血因子VIII促凝活性及抗原含量的关系。
J Clin Invest. 1973 Nov;52(11):2708-16. doi: 10.1172/JCI107465.
8
Platelet adhesion to collagen in subtypes of type I von Willebrand's disease is dependent on platelet von Willebrand factor.I型血管性血友病各亚型中血小板对胶原蛋白的黏附依赖于血小板血管性血友病因子。
Thromb Haemost. 1990 Oct 22;64(2):227-31.
9
Heightened interaction between platelets and factor VIII/von Willebrand factor in a new subtype of von Willebrand's disease.血管性血友病新亚型中血小板与凝血因子VIII/血管性血友病因子之间的相互作用增强。
N Engl J Med. 1980 May 8;302(19):1047-51. doi: 10.1056/NEJM198005083021902.
10
Stabilization of factor VIII in plasma by the von Willebrand factor. Studies on posttransfusion and dissociated factor VIII and in patients with von Willebrand's disease.血管性血友病因子对血浆中凝血因子VIII的稳定作用。关于输血后及解离的凝血因子VIII的研究以及血管性血友病患者的研究。
J Clin Invest. 1977 Aug;60(2):390-404. doi: 10.1172/JCI108788.