Modern Aspects of the Study and Treatment of Hemophilia B in the Republic of Uzbekistan

Juraeva Nodira  Tukhtapulatovna; Mahmudova Aziza  Dzhumanovna; Ismatova Gulira  Nazimovna; Mamatkulova Dilrux  Fayzullaevna

Authors

Juraeva Nodira Tukhtapulatovna Doctoral student, Republican Specialized Scientific and Practical Medical Center of Hematology, Tashkent, Uzbekistan
Mahmudova Aziza Dzhumanovna Doctor of Medical Sciences, Republican Specialized Scientific and Practical Medical Center of Hematology, Tashkent, Uzbekistan
Ismatova Gulira Nazimovna Republican Specialized Scientific and Practical Medical Center of Hematology, Tashkent, Uzbekistan
Mamatkulova Dilrux Fayzullaevna Candidate of Medical Sciences, Republican Specialized Scientific and Practical Medical Center of Hematology, Tashkent, Uzbekistan

Keywords:

hemarthrosis, coagulopathy, hemorrhage, blood coagulation

Abstract

Hemophilia B is the hereditary disease of the blood coagulant system linked to X-chromosome, which is characterized by decreasing or molecular anomalies of the IX factor of coagulation. The coagulation factor IX gene is localized on the X chromosome (Xq27.1-q27.2), it is characterized by X-linked recessive inheritance. The relevance of studying this subject is that at untimely diagnostics and late begun treatment hemophilia can lead to serious consequences. In the treatment of patients with hemophilia, the main component is timely adequate lifelong replacement therapy, which makes it possible to replenish the level of the deficient factor in the plasma. The main goal of such hemostatic therapy is to increase the content of the deficient factor in the patient's blood to an effective level. The aim of this work is to study the level of factor IX in patients with hemophilia during replacement therapy with plasma drugs of blood coagulation factors.