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EPIDEMIOLOGICHESKOE THE FIRST OBSERVATIONAL STUDY TO DETERMINE THE PREVALENCE OF VON WILLEBRAND DISEASE IN RUSSIA

Zelenova O. V.1, Oscow Y. I.1, Abramov S. I.1, Aslanyan, K. S.2, Shamray V. S.3, Ilyasov R. K.4, Ptushkin V. V.5, Babaeva, E. G.6, Kirillova E. G.7, Menshikova S. N.8, Kosinova M. V.9
1. "Central scientific – research Institute of organization and Informatization of health" Ministry of health of Russia, Moscow
2. Regional children's clinical hospital of Rostov-on-don, Rostov-on-don
3. State budgetary institution of the Rostov region "Rostov regional clinical hospital", Rostov
4. Crimean Republican oncological clinical dispensary named after V. M. Efetov, Sevastopol
5. MBUZ "GKB them. S. P. Botkina DZM", Moscow
6. Federal state budgetary educational institution of higher professional education "Novosibirsk state medical University" Ministry of healthcare of the Russian Federation, Novosibirsk
7. Budgetary healthcare institution of the Omsk region " Regional Clinical Hospital", Omsk
8. State budgetary healthcare institution of the Tver region "Regional clinical hospital", Tver
9. GAU KO "Kemerovo regional clinical hospital named after S. V. Belyaev", Kemerovo
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Summary:
Relevance. Willebrand disease (BV) is the most common hereditary coagulopathy caused by a decrease in the amount or impaired function of Willebrand factor (vWF). Approximately 70% of patients with BV have a mild clinical course, while the rest have moderate or severe clinical manifestations of hemorrhagic syndrome. According to the clinical recommendations of the society of hematologists, developed in 2018 and approved by the Ministry of health of the Russian Federation, a phased laboratory coagulological study is recommended to clarify the diagnosis [4]. Obtaining the maximum amount of data helps to verify the diagnosis and exclude acquired coagulopathies. The first phase of laboratory diagnostics recommended koagulologicescoe screening. To verify the diagnosis of BV, it is recommended to conduct the second stage of diagnosis-specific coagulological studies:the ratio of vWF:RSO/vWF: Ag (required); FVIII:C (required), the level of RIPA in two concentrations of ristomycin (to clarify the type of BV); analysis of VWF multimers (in unclear cases). Purpose of research. To conduct an observational epidemiological study in some regions of the Russian Federation and compare the data of epidemiological surveillance for Willebrand disease with data obtained from regional MIAC. Research problem. Develop a questionnaire that allows you to get data on the forms of the disease, conduct a survey of regional freelance GPS on the routine practice of diagnosis and treatment of patients with Willebrand's disease. Materials and methods. A survey of 25 regional freelance chief Hematology specialists was conducted based on the developed questionnaire. In parallel, a request was made to the regional MIAC to obtain information about the number of patients with hemophilia, D66 - D68. The Willebrand disease questionnaire included the following questions: the number of patients by disease type and without type determination, the number of patients on therapy or under observation, the number of patients on FVIII+vWF therapy in the "prevention" mode and in the "on-demand"mode. Results. Data were obtained from 13 regions where 2,774 patients with hemophilia were observed, including 1,396 patients with hemophilia A, including 155 patients with the inhibitory form of the disease, which is considered the most unfavorable and difficult to stop. Willebrand disease was diagnosed in 1,074 patients. Conclusions. Registration of patients with Willebrand's disease in Russia has several restrictions, as well as laboratory diagnostics, which is necessary for diagnosis, is not present in all regions. As a result of a survey of chief freelance specialists from 6 subjects, we were able to collect data on 292 patients with Willebrand's disease. Out of 292 patients, only 78 are men and 214 are women, 190 patients are observed without determining the type of disease, 63 patients have type 1, type 2 in 19 patients and 20 patients are diagnosed with type 3 disease. No subject conducts diagnostics based on subtypes of the second type of disease, which will soon be enriched with a pathogenetic type of drug therapy.
Keywords von Willebrand disease, morbidity, survey

Bibliographic reference:
Zelenova O. V., Oscow Y. I., Abramov S. I., Aslanyan, K. S., Shamray V. S., Ilyasov R. K., Ptushkin V. V., Babaeva, E. G., Kirillova E. G., Menshikova S. N., Kosinova M. V., EPIDEMIOLOGICHESKOE THE FIRST OBSERVATIONAL STUDY TO DETERMINE THE PREVALENCE OF VON WILLEBRAND DISEASE IN RUSSIA // Scientific journal «Current problems of health care and medical statistics». - 2020. - №4;
URL: http://healthproblem.ru/magazines?textEn=547 (date of access: 19.04.2024).

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