Instruction for use: Antihemophilic factor IX

Trade name of the drug – Agemfil B, Aimafix, Bebulin TIM 4, Immunine, Mononain, Octanine, Octanine® F (filtered), Replenine-VF

Pharmacological group:

Coagulants (including coagulation factors), hemostatics

Model clinical-pharmacological article 1

Pharmacotherapy. The blood coagulation factor IX has a hemostatic effect; Increases the concentration of factor IX in plasma, restores hemostasis in patients with deficiency. The active form of factor IX-factor IXa-in combination with factor VIII activates factor X, which promotes the transition of prothrombin to thrombin and the formation of a fibrin clot. Increases plasma concentrations of vitamin K-dependent coagulation factors (II, VII, IX and X). With a decrease in plasma factor IX below 5% of the norm, the risk of spontaneous hemorrhages increases sharply, and a content above 20% of the norm ensures satisfactory hemostasis.

Pharmacokinetics. About 30-50% of factor IX is detected in the blood immediately after intravenous infusion; Then the haemostatic activity gradually decreases. T1 / 2 - 24 h.

Indication. Bleeding caused by factor IX deficiency (treatment, prevention); hemophilia; Bleeding caused by coumarin anticoagulants (before an emergency surgery, with trauma).

Contraindications. Hypersensitivity, DIC-syndrome, acute thrombosis, acute myocardial infarction, factor VII deficiency, acute renal failure, pregnancy, lactation.

Dosing. IV (by stream or drop infusion), no more than 2 ml / min. The dosage regimen is set individually for the control of factor IX in the blood. In emergency cases, it should be taken into account that the first dose of 1 IU / kg increases factor IX by 0.5-1%, and subsequent administration of the same dose by 1-1.5%. With the removal of teeth and small operations, the concentration of factor IX should not be below 30% of the norm, with gastrointestinal bleeding - below 30-50%, and with intracranial hemorrhages or extensive operations - below 60%. If the patient undergoes surgery or is injured, an additional amount of factor IX is required, maintaining a dose of 10-20 IU / kg / day.

For prolonged prevention of bleeding (severe hemophilia B) - 18-30 IU / kg 1 time per week or 9-15 IU / kg 2 times a week. To treat bleeding in patients with hemophilia A - 75 IU / kg; If necessary, a second dose is given after 12 hours. The I / O rate is 100 IU / min.

To calculate the number of units of factor IX activity that should be administered to the patient to achieve the required concentration of factor IX in the bloodstream, the following formula can be recommended: the required dose = F (%) x body weight (kg) × 0.8, where Ô (% ) Is the necessary increase in activity.

Side effect. Heat, chills, headache, nausea, tachycardia, tingling sensation in the body, back pain, postoperative thrombosis, allergic reactions (hives, anaphylactic reaction), decreased resistance to infectious diseases.

Interaction. Incompatible with other hemostatic drugs due to possible contact activation or inactivation.

Aminocapronic acid increases the risk of thrombosis.

Special instructions. The diluted drug should be used no later than 1 hour after preparation and not added to other transfused liquids (plasma, solutions).

It should be borne in mind that the antibodies to the factor IX can partially or completely inactivate the drug. In this case, an increase in the dose and the use of activated factors of the prothrombin complex and / or immunosuppressants are recommended.

With a significant increase in heart rate, the injection is slowed or stopped.