Alpha 1-Proteinase inhibitor (Alpha 1-proteinase inhibitor)

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

Enzymes and antiferments

Included in the formulation
  • Respicam®
    solution d / infusion 
    Kamada Co., Ltd.     Israel
    • АТХ:

    B.02.A.B   Inhibitors of plasma proteinases

    Pharmacodynamics:Substitution therapy with a deficit of alpha-1-antitrypsin - chronic hereditary, autosomal recessa clear disease characterized byI develop a severe, slowly progressing panacinous emphysema of the lungs.
    Pharmacokinetics:

    When infusion once a week at a dose of 60 mg / kg body weight, the average concentration alpha-1-antitrypsin was 126 mg / dl (before treatment 30 mg / dL). This concentration is sufficient to prevent the development of emphysema in patients with congenital deficiency alpha-1-antitrypsin.

    In the first hours after the infusion, the maximum plasma concentration is 280 mg / dL with a rapid decrease in the first 2 days, and a gradual subsequent decrease within 7 days. The half-life of the alpha 1-proteinase inhibitor was 4.5 days. During 2 years of the study, he did not change, which indicates the lack of antibody formation in response to the introduction alpha-1-antitrypsin.

    Indications:

    Congenital deficiency of the alpha 1-proteinase inhibitor in patients with a clinical picture of panacinose emphysema.

    For prolonged replacement therapy only in patients with phenotypes PiZZ, PiZ (-) or Pi (-) (-).

    ICD-10

    X.J40-J47.J43   Emphysema

    Contraindications:

    - selective deficiency of immunoglobulin A (IgA) in the presence of antibodies to IgA;

    - hypersensitivity to the components of the drug.

    Safety and effectiveness of the drug in children is not established. Use in children is contraindicated.

    Carefully:No data.
    Pregnancy and lactation:

    FDA category is not defined ..

    The alpha 1-proteinase inhibitor may be prescribed by a doctor during pregnancy and breastfeeding only in cases of acute necessity, when the expected benefit to the mother exceeds the possible risk to the fetus or the baby.

    Dosing and Administration:

    Intravenously.

    The recommended dose of 60 mg per kilogram of body weight once a week is intravenously dripped at a rate of 0.08 ml / kg / min or faster. The duration of the infusion should not exceed 30 minutes.

    Side effects:Headache, erythema and dysgeusia; hyperthermia, allergy, chills, the appearance of dyspnea, rash, tachycardia, lowering blood pressure, sometimes - short-term increase in blood pressure.
    Overdose:No cases of drug overdose have been reported. Antidote to the drug does not exist.
    Interaction:Alpha 1-proteinase inhibitor in the form of a solution for infusion should not be mixed with other drugs.
    Special instructions:

    The "threshold" concentration of the alpha 1-proteinase inhibitor in blood plasma, designed to provide sufficient anti-elastase action in the lungs of patients with a deficit of alpha 1-antitrypsin is 80 mg / dL.

    It is made from blood plasma of healthy people with the help of ion-exchange chromatography. In order to prevent the risk of transfer of pathogens of infectious diseases, each portion of human plasma used to produce the drug is tested for the content of the human immunodeficiency virus, antibodies to hepatitis C, hepatitis B surface antigen, and a serological response to the determination of the causative agent of syphilis.

    Despite the inactivation and / or elimination of viral pathogens during the manufacture of the drug, the presence of an unknown infectious agent in it can not be completely ruled out. In patients receiving infusions of blood or plasma preparations, signs and / or symptoms of a viral disease, for example hepatitis C, may develop. Before prescribing the drug, the doctor must correlate the risk and benefit.Possible increase in the volume of plasma after infusion, which should be taken into account in patients with an increased volume of circulating fluid.

    Do not re-apply the remnants of the drug after performing the infusion.

    Unused bottles of the drug that were taken from the refrigerator should be returned to the refrigerator for storage for 6 hours. If the vial with the drug remained at room temperature (25 ° C) for more than 48 hours, the drug is considered to be overdue and not allowed for further use.

    Impact on the ability to drive vehicles and manage mechanisms

    The effect of the drug on the performance of potentially hazardous activities requiring increased concentration of attention and speed of psychomotor reactions has not been established.

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