Stasyshyn O. Molecular genetic mechanisms of pathogenesis and ways of optimization of diagnosis, treatment and prevention of inhibitors in hemophilia patients

Objective: To increase the effectiveness of providing medical care to hemophilia patients with inhibitors; improve diagnostics, treatment and prophylaxis programs in these patients. Subject of research: molecular genetic factors of predisposition to the development of an inhibitor; the characteristics of the inhibitor and the treatment of patients with acquired hemophilia A and B. On the basis of clinical and coagulation studies, the rate of inhibitors to FVIII(IX) in hemophiliacs in Ukraine is estimated to be 16.7% for hemophilia A and 3.9% for hemophilia B, presented characterization of types of inhibitors. On the basis of multiple logit regressions, the risk factors for the appearance of an inhibitors in hemophilia patients have been summarized, which made it possible to identify patients at high risk of developing inhibitors, to create a model to predict its occurrence and to propose ways of prevention. Clinical evaluation of hemostatic effectiveness of monotherapy with rFVІIa and aPCC in hemophilia patients with inhibitors during bleeds and hemarthrosis for a long period was performed on the basis of standardized criteria. The programs of the treatment of the bleeds and providing hemostasis during surgery in hemophilia patients with low titre of inhibitors and low responders are substantiated. Medicine.