Nonacog Alpha (Nonacogum alfa)

Pharmacodynamics Pharmacokinetics Indications Carefully Contraindications Dosing and Administration Side effects
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Clinical and pharmacological group: & nbsp

Coagulants (including clotting factors), hemostatics

Included in the formulation
  • Benefiks®
    lyophilizate in / in 
    WAYET, LLC     Russia
  • InnovaFactor
    lyophilizate in / in 
    GENERIUM, CJSC     Russia
    • Included in the list (Order of the Government of the Russian Federation No. 2782-r of 30.12.2014):

    VED

    АТХ:

    B.02.B.D.09   Nonacog Alpha

    Pharmacodynamics:

    The drug is a recombinant vitamin K-dependent clotting factor IX. It is an essential component of the blood clotting process. The recombinant coagulation factor IX is activated by the coagulation factor VII complex and the tissue thromboplastin by the external clotting pathway, as well as the factor XIa by the internal clotting pathway. The activated clotting factor IX in combination with the activated clotting factor VIII activates the clotting factor X, which in turn leads to the conversion of prothrombin to thrombin. Then thrombin converts fibrinogen into fibrin and makes it possible to form a clot.

    Pharmacokinetics:

    The half-life is 11 to 36 hours.

    Indications:

    Prophylaxis / treatment of hemorrhagic complications caused by a deficiency of the factor of blood coagulability IX (hemophilia B) in patients 18 years of age and older.

    ICD-10

    III.D65-D69.D67   Hereditary deficiency of factor IX

    Contraindications:

    Age to 12 years, hypersensitivity.

    Carefully:

    Age before 6 years.

    Pregnancy and lactation:

    Not classified FDA. Use only if there are strong indications.

    Due to the rare incidence of women with hemophilia B, there is no experience with the use of nonacqua alfa during pregnancy and during breastfeeding. The use is possible only if there are strong indications and provided that the expected benefit to the mother exceeds the potential risk to the fetus or the baby.

    Dosing and Administration:

    The dosage regimen is individual and depends on the degree of insufficiency of the factor of blood coagulability IX.

    Intravenously injected slowly.

    Dose selection is based on the fact that in patients aged 12 years and over 1 unit of coagulation factor IX per 1 kg of body weight increases the concentration of circulating coagulation factor IX by an average of 0.8 IU / dL (0.3 to 1.4 IU / dl ).

    The required dose is determined by a special formula.

    To prevent bleeding, the average dose is 40 IU / kg (13 to 78 IU / kg) at intervals of 3-4 days. In younger patients, shorter intervals between administrations or higher doses are possible.

    Side effects:

    From the nervous system: dizziness, headache, impaired consciousness, inhibition, anxiety.

    On the part of the digestive system: increased activity of liver enzymes, dyspepsia, increased bilirubin concentration in the blood, changes in taste, diarrhea.

    Local reactions: pain, phlegmon, pain, phlebitis.

    On the part of the respiratory system: dry cough, respiratory distress syndrome.

    From the cardiovascular system: tachycardia, lowering blood pressure.

    Allergic reactions: hyperthermia, urticaria, laryngospasm, angioedema, allergic rhinitis, sneezing, bronchospasm, anaphylaxis.

    Other: skin rashes, the formation of neutralizing antibodies, thromboses, a burning sensation in the area of ​​the lower jaw and skull, a rush of blood to the face, photosensitivity reactions.

    Overdose:

    Not described.

    Interaction:

    Not described.

    Special instructions:

    Treatment under the strict supervision of a doctor who has experience in the treatment of hemophilia B.

    In case of allergic or anaphylactic reactions, the introduction of nonacarpal alpha should be stopped immediately and adequate treatment started.

    The experience of clinical use of complex concentrated plasma preparations of clotting factors II, VII, IX and X showed the risk of thromboembolic complications. When using nonacarpal alpha, the potential risk of developing thrombosis and DIC syndrome should be considered. Therefore, it is clinically and laboriously to detect early signs of thrombosis and coagulopathy of consumption in the appointment of nonacarpal alpha to patients with impaired hepatic function, postoperative patients, newborns or patients at risk of thrombotic disorders.

    Patients receiving drugs containing coagulation factor IX sometimes develop antibodies that neutralize the activity of factor IX, therefore, when using nonacarpal alpha, the presence of neutralizing antibodies should be monitored regularly.

    In patients who have high concentrations of inhibitory antibodies to the coagulation factor IX, therapy may not be effective. In this case, consideration should be given to the designation of alternative therapies.

    In the presence of neutralizing antibodies to coagulation Factor IX there is an increased risk of anaphylaxis with subsequent injections of coagulation factor IX.Therefore, all patients with indications of allergic reactions should be examined for the presence of neutralizing antibodies to coagulation factor IX.

    Impact on the ability to drive vehicles and manage mechanisms

    Studies to identify the effect of the drug on the ability to drive and work with mechanisms that require increased concentration of attention were not conducted.

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