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明显血管性血友病(VWD)病例的止血特征详述:一项横断面研究。

Hemostatic profile detailing in apparent VWD cases: A cross sectional study.

作者信息

Alqahtany Fatmah S, ALBackr Hanan B, Aldakhil Lateefa O, Alharbi Abdullah A, Alqahtani Nawaf Abdullah, Algahtani Farjah H

机构信息

Hematopathology Unit, Department of Pathology, College of Medicine, King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia.

Department of Cardiac Sciences, King Fahad Cardiac Center, College of Medicine, King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia.

出版信息

Saudi J Biol Sci. 2021 Dec;28(12):6701-6704. doi: 10.1016/j.sjbs.2021.07.044. Epub 2021 Jul 19.

Abstract

The von willebrand disease (vWD) accounts to be one of the most common hereditary bleeding ailment that amounts its incidence to almost 1.5% of normal population. It is mostly associated with a defect in primary hemostasis as well as secondary defect in coagulation factor VIII as diagnosis of vwd happened to be challenging with earlier diagnostic criteria's. Testing Vwd in menorrhagia patients was not at ease. A cross-sectional study was conducted in female patients who have visited obstetrics and gynecology clinic at King Saud University Medical City (KSUMC), Riyadh, Saudi Arabia. The inclusion criteria consist of adult female patients between 16 and 45 years old with menorrhagia. A sample of 45 patients were screened and selected for the above-mentioned study. The SPSS Statistical analysis package was performed to analyze the data's. The fisher's exact test was conducted to compare the demographic variables. The independent samples -test was conducted to compare the means of subjects. The P value of ≤0.05 considered as statistically significant. The cases manifested with a history of bleeding during periods stretching from 7 to 90 days. The vWD was reported in 6.6 % (n = 3) women out of the total 45 patients. The vWF: Ac mean ± SD (51.4 ± 6.3) and vWF: Ag Mean ± SD (93 ± 67) were significantly lesser in vWD patients with that of non-vWD (98.7 ± 22.6) vs (116 ± 42.4) (p = 0.027) (p = 0.032) respectively. WBC, ESR, MCV, MCH, Hemoglobin, PLT count, INR, PT, APTT and FVIII showed no significant difference among the groups (p > 0.05).

摘要

血管性血友病(vWD)是最常见的遗传性出血性疾病之一,其发病率几乎占正常人群的1.5%。它主要与初级止血缺陷以及凝血因子VIII的继发性缺陷有关,因为根据早期诊断标准,vWD的诊断具有挑战性。在月经过多的患者中检测vWD并不容易。在沙特阿拉伯利雅得国王沙特大学医学城(KSUMC)妇产科诊所就诊的女性患者中进行了一项横断面研究。纳入标准包括年龄在16至45岁之间、患有月经过多的成年女性患者。筛选并选择了45名患者作为上述研究的样本。使用SPSS统计分析软件包对数据进行分析。采用Fisher精确检验比较人口统计学变量。采用独立样本t检验比较受试者的均值。P值≤0.05被认为具有统计学意义。这些病例表现为经期出血史,持续时间从7天到90天不等。在45名患者中,有6.6%(n = 3)的女性被报告患有vWD。vWD患者的血管性血友病因子活性(vWF:Ac)均值±标准差(51.4±6.3)和血管性血友病因子抗原(vWF:Ag)均值±标准差(93±67)明显低于非vWD患者(分别为98.7±22.6和116±42.4)(p = 0.027)(p = 0.032)。白细胞、红细胞沉降率、平均红细胞体积、平均红细胞血红蛋白含量、血红蛋白、血小板计数、国际标准化比值、凝血酶原时间、活化部分凝血活酶时间和凝血因子VIII在各组之间无显著差异(p>0.05)。

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New advances in the diagnosis of von Willebrand disease.血管性血友病的诊断新进展。
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J Atheroscler Thromb. 2019 Apr 1;26(4):303-314. doi: 10.5551/jat.RV17031. Epub 2019 Mar 12.
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von Willebrand factor regulation of blood vessel formation.血管生成中 von Willebrand 因子的调节。
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Von Willebrand's disease: case report and review of literature.血管性血友病:病例报告及文献综述
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