Beltran Andres, Jaramillo Arturo P, Vallejo Maria P, Acosta Luis, Barberan Parraga Gabriela Carolina, Guanín Cabrera Carlos Luis, Gaibor Victor G, Cueva Maria G
General Practice, Universidad Católica de Santiago de Guayaquil, Guayaquil, ECU.
General Practice, Universidad Estatal de Guayaquil, Machala, ECU.
Cureus. 2023 Aug 29;15(8):e44310. doi: 10.7759/cureus.44310. eCollection 2023 Aug.
Von Willebrand disease (VWD) and hemophilia A are the most common inherited bleeding disorders. Quantitative or qualitative von Willebrand factor (VWF) anomalies cause this disorder in men and women. VWF, a plasma glycoprotein, relies on platelets for primary hemostasis. It also carries and stabilizes factor VIII in the blood. VWD has several categories. Types 1 and 3 have partial or total VWF quantitative deficiencies. However, type 2 and its subtypes have VWF quality issues. The major treatment is desmopressin (DDAVP), which replaces endogenous VWF and factor VIII (FVIII). Plasma-derived VWF/FVIII products may also be substituted exogenously. Treatment with plasma-derived or recombinant VWF concentrates without FVIII is also possible. The purpose of this retrospective, single-center research was to evaluate DDAVP's efficacy in treating VWD based on many criteria established in the current literature. We looked at the results on Google Scholar, the Cochrane Library, and PubMed/Medline. There were a total of 10 papers found, evaluated, and accepted for inclusion in this study. A comprehensive analysis of DDVAP's role in VWD was compiled from the aforementioned papers. Various aspects of DDVAP were captured by including an analysis of complementary treatments used in surgical and clinical settings. We also describe the treatment's intended impact on the different variations of the disease. Given these results, further investigation is required to determine the most effective method for managing VWD so that it may be included in standard clinical practice.
血管性血友病（VWD）和血友病A是最常见的遗传性出血性疾病。血管性血友病因子（VWF）的数量或质量异常会导致男性和女性出现这种疾病。VWF是一种血浆糖蛋白，在初级止血过程中依赖血小板。它还在血液中携带并稳定因子VIII。VWD有几种类型。1型和3型存在部分或完全的VWF数量缺陷。然而，2型及其亚型存在VWF质量问题。主要治疗方法是去氨加压素（DDAVP），它可替代内源性VWF和因子VIII（FVIII）。也可以外源性替代血浆来源的VWF/FVIII产品。也可以使用不含FVIII的血浆来源或重组VWF浓缩物进行治疗。这项回顾性单中心研究的目的是根据当前文献中确立的多项标准评估DDAVP治疗VWD的疗效。我们在谷歌学术、考克兰图书馆和PubMed/Medline上查找结果。总共发现、评估并纳入了10篇论文用于本研究。从上述论文中汇编了对DDAVP在VWD中作用的全面分析。通过纳入对手术和临床环境中使用的辅助治疗的分析，捕捉了DDAVP的各个方面。我们还描述了该治疗对疾病不同变体的预期影响。鉴于这些结果，需要进一步研究以确定治疗VWD的最有效方法，以便将其纳入标准临床实践。
