Treatment And Incidence Of Von Willebrand's Disease In The Republic Of Uzbekistan
DOI:
https://doi.org/10.62480/tjms.2022.vol12.pp52-55Keywords:
von Willebrand disease, menorrhagia, petechiae, hemorrhagiaAbstract
Von Willebrand disease (VW) is the most common coagulopathy caused by quantitative and qualitative von Willebrand factor (vWF) deficiency. BV affects about 1% of the population. However, most patients with VWD usually have no symptoms. The number of patients with BV who need ongoing treatment is comparable to that of hemophilia. Approximately 70% of patients with VWD have a mild disease, while the remaining 30% of patients have a moderate or severe disease [1]. The most characteristic and specific symptom in von Willebrand disease is bleeding from the mucous membranes of the mouth, nose, and internal organs. Symptoms of bleeding vary from moderately severe to extremely severe, proceeding mainly according to the microcirculatory type. Patients with severe factor VIII deficiency experience profuse and prolonged bleeding (nasal, gingival, uterine), as well as hemorrhages in muscles and joints. In addition, prolonged bleeding may occur with injuries, tooth extractions, and operations. In childhood, there are often bleeding from the mucous membranes of the oral cavity, nosebleeds, bruises on the skin. A more severe course of hemorrhagic diathesis is observed during or shortly after infectious diseases. The most likely trigger for bleeding in the presence of an infection is a violation of vascular permeability. The most likely trigger for bleeding in the presence of an infection is a violation of vascular permeability. As a result, spontaneous bleeding of the diapedetic type appears.
References
Bowman M., Tuttle A., Notley C. et al. The genetics of Canadian type 3 von Willebrand disease: further
evidence for co-dominant inheritance of mutant alleles.JThromb Haemost. 2013; 11(3): 512-
https://doi.org/10.1111/jth.12130.
Casonato A., Galletta E., Sarolo L., Daidone V. Type 2N von Willebrand disease: Characterization and
diagnostic diffi culties. Haemophilia. 2018; 24(1): 134-40. https://doi.org/10.1111/hae.13366
Flood V.H. New insights into genotype and phenotype of VWD. Hematology. 2014; (1): 531-
https://doi.org/10.1182/asheducation-2014.1.531
Goodeve A.C. The genetic bases of von Willebrand disease. Blood Reviews. 2010; 24: 123-
https://doi.org/10.1016/j.blre.2010.03.003.
Jokela V., Lassila R., Szanto T. et al. Phenotypic and genotypic characterization of 10 Finnish patients
with von Willebrand disease type 3: discovery of two main mutations. Haemophilia. 2013; 19(6): 344-
https://doi.org/10.1111/hae.12225.
Likhacheva E.A., Polyanskaya T.Yu., Zorenko V.Yu. i dr. Klinicheskie rekomendatsii po diagnostike i
lecheniyu bolezni Villebranda. M.: Natsional'noe gematologicheskoe obshchestvo, 2014.
Scottish Intercollegiate Guidelines Network (SIGN). SIGN 50: a guideline developer’s handbook.
Edinburgh: SIGN; 2014. (SIGN publication no. 50). [October 2014]. Available from URL:
Downloads
Published
Issue
Section
License

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
User Rights
Under the Creative Commons Attribution-NonCommercial 4.0 International (CC-BY-NC), the author (s) and users are free to share (copy, distribute and transmit the contribution).
Rights of Authors
Authors retain the following rights:
1. Copyright and other proprietary rights relating to the article, such as patent rights,
2. the right to use the substance of the article in future works, including lectures and books,
3. the right to reproduce the article for own purposes, provided the copies are not offered for sale,
4. the right to self-archive the article.