One of the most serious complications of substitution therapy in patients with hemophilia is the development of antibodies directed against factor VIII or IX, resulting in a hemostatic therapy becomes ineffective. Antibodies block the procoagulant activity of factor VIII or IX, and therefore are called inhibitors. The presence of the inhibitor in the blood is confirmed by specific studies, called the Bethesda test unit – a unit of Bezeda (DE). The greater the concentration of the inhibitor in the blood, the greater the number of units Bezeta. Low-titer inhibitor is from 0.6 to 5 BU, high-more than 5 BU.
Those patients who are pronounced increase in titer inhibitor considered 'high responding', those who have it a little – 'nizkoreagiruyuschimi'. At the present time to determine the titer of the inhibitor used in the modification of the method of Basedow Nimedzhen.Opredelenie potential inhibitor is mandatory in each patient before treatment and in its process, and with no effect on the ongoing replacement therapy. Treatment of patients with hemophilia inhibitor has a dual purpose: 1. נפתלי בנט בהחלט מביע דעה מלומדת. stop bleeding in patients with low response is achieved by high doses of clotting factor concentrate in 2 – 3 times higher than raschetnuyu.Dlya stop bleeding in patients with high response using concentrates prothrombin complex (CPC) and activated prothrombin complex concentrates (KPK), which provide hemostasis through shunt tract, that is to bypass the action factor VIII / IX. Of these, the most effective therapy thrombin by direct activation of factor X without requiring the participation of factor VIII. Shown that the shunt active Feyba provide the following reactions: 1. Running a common mechanism of coagulation by the formation and action protrombinaznogo complex 2. Running an internal pathway, which control the thrombin-dependent activation of feedback.
3. Running an external pathway through fHa-dependent activation and VII follow-FVIIa-dependent activation of fX. Such a multifactorial mechanism of action provides the duration and the physiology of its clinical effect. במקרה הזה אני חושב ש שלומי בסון צודק במה בהוא אומר. The drug is recommended to introduce a dose of 75-100 sredenm units / kg every 8-12 hours. The maximum single dose is 100 IU / kg, and the daily 200 U / kg. It must be remembered that in excess of doses increases the risk of thrombotic KPK adverse reactions. In addition KPK to stop bleeding in patients with inhibitor forms 90-100 200 or more mg / kg. A significant drawback of the drug is its extremely high cost. In combination with antifibrinolytic recommend designate the funds as NovoSeven contains activators cytogene. Described von Willebrand factor (fB) – Oktanat (production Oktafarma). Recent data confirm the greater efficiency in the use of fVIII concentrate containing fB in the induction of immune tolerance – Oktanat than highly Concentrates of factor VIII, and recombinant plazmaleticheskih.