The prevalence of rare bleeding disorders among children in the Russian Federation

Rare bleeding disorders account for about 3–5% of all inherited disorders. Due to the rarity and complexity diagnosing these disorders, their prevalence estimates vary greatly. There is currently no national registry rare across country has not been studied yet. Aim: estimate coagulation among Russian children. For this multicenter study, we used retrospective anonymous patient data collected during clinical practice, so approval ethics committee was required. We analyzed completed questionnaires containing number patients with aged from 0 18 years 72 subjects Federation. The survey had conducted April June 2022. Our analysis included deficiencies factor I, II, V, VII, X, XI or XIII, as well combined unspecified hemorrhagic conditions. According reported data, total children 398. most common disorder deficiency VII (52%, n = 210); it followed by fibrinogen (16%, 63) X (12%, 48). Deficiencies factors XI, V XIII 9% ( 35), 5% 20), 4.5% 18) cases, respectively. Combined diagnosed in 1.7% 7) II detected only 1% 4). In order determine actual incidence manifestations identify need concentrates, necessary establish a following example hemophilia registry.