A rare disorder of blood coagulation

The problem of impaired hemostasis remains relevant even today. Rare bleeding disorders that cause life-threatening in patient are often overlooked by clinicians. blood coagulation a genetically determined group coagulopathies caused deficiency plasma proteins involved hemostasis, as well fibrinogen, prothrombin, factor V (FV), factors and VIII (FV+FVIII), VII (FVII), X (FX), XI (FXI), XII (FXII), XIII (FXIII), which clinically manifested bleeding. amount the determines not only nature bleeding, but also their severity prognosis for disease. In such patients, general hemostatic balance is important, since level each control can determine risk remain important. Purpose - to draw attention doctors various specialties clinical manifestations rare hereditary coagulation, be accompanied poses threat health life patient. Clinical case. A case presented illustrates course disorder children from one family, where comprehensive diagnostic search was conducted establish final diagnosis. Conclusions. pathology found population, symptoms have negative consequences person's life. Children with hemorrhagic syndrome need thorough examination specialized laboratories. Physicians related should look possible early late complications postoperative period or after medical manipulations. It necessary remember genesis this examine all family members. research carried out accordance principles Helsinki Declaration. informed consent obtained conducting studies. No conflict interests declared authors.