Alglucosidase alpha (Alglucosidasum alpha)

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
  • Mayozaim®
    lyophilizate d / infusion 
    Genzyme Europe BV     Netherlands
    • Included in the list (Order of the Government of the Russian Federation No. 2782-r of 30.12.2014):

    VED

    АТХ:

    A.16.A.B.07   Alglucosidase alpha

    Pharmacodynamics:

    Human alglycosidase alpha is a lysosomal enzyme, the deficiency of which leads to the development of rare progressive metabolic myopathy - Pompe disease (second type glycogenosis). The drug alglucosidase alpha is a recombinant protein obtained in the culture of Chinese hamster ovary cells (cell line CHO). This drug compensates for the lack of the enzyme alglyukozidase alpha and normalizes the work of muscles by reducing the amount of glycogen.

    Pharmacokinetics:

    Enter intravenously, half-elimination - 2.75 hours. Through the blood-brain barrier does not penetrate.

    Indications:

    The drug is intended for long-term enzyme replacement therapy in patients with Pompe disease (adults and children).

    ICD-10

    IV.E70-E90.E74.0   Diseases of accumulation of glycogen

    Contraindications:

    Hypersensitivity.

    Carefully:

    With the repeated administration of the drug to patients who for the first time observed undesirable drug effects.

    You should observe the patient during infusion and within two hours after.

    Pregnancy and lactation:

    Category FDA - C. Alglucosidase alpha should not be used during pregnancy, if there is no absolute indication. The potential risk to humans is unknown. Studies on the use of alglucosidase alpha in pregnant women have not been conducted, but studies in animals have demonstrated reproductive toxicity.

    Alglucosidase alpha may be excreted in breast milk. When using the drug, it is recommended to stop breastfeeding, since there is no evidence of the effect of alglucosidase alpha through breast milk on newborns.

    Dosing and Administration:

    Treatment is mandatory under the supervision of a doctor. The recommended dosage regimen of alglucosidase alpha: 20 mg / kg body weight once every 2 weeks as an intravenous infusion.

    Infusions should be carried out with a gradual increase in the rate of administration of the drug, starting at 1 mg / kg / h, and gradually increase the dose by 2 mg / kg / h every 30 minutes, in the absence of infusion-related reactions, until a maximum rate of 7 mg / kg / h.

    Side effects:

    From the immune system: hypersensitivity, an allergic shock.

    From the side of the cardiovascular system: tachycardia, pallor, chest pain, cyanosis, hypertension, hot flashes, heart failure, cardiac arrest, bradycardia, hypotension, vascular spasm.

    From the nervous system: tremor, paresthesia, dizziness, headache.

    Mental disorders: agitation, anxiety.

    From the sense organs: conjunctivitis.

    From the respiratory system: rapid breathing, cough, apnea, a feeling of compression in the throat, respiratory distress syndrome, bronchospasm, wheezing, swelling of the pharynx, dyspnea, stridor, respiratory failure.

    From the digestive system: vomiting, abdominal pain, diarrhea.

    Co of the skin: erythema, rash, itching, hives, hyperhidrosis.

    From the side of the musculoskeletal system: arthralgia, muscle spasms, muscle twitching, myalgia.

    From the urinary system: nephrotoxic syndrome, proteinuria.

    Other disorders and complications at the site of administration: fever, chills, edema of the face, cold extremities, swelling, burning sensation, soreness in the infusion site.

    Overdose:

    There have been no cases of overdose, therefore, there is no information about the consequences.In clinical trials, the maximum applied doses were 40 mg / kg.

    Interaction:

    Studies on the drug interactions of alglucosidase alpha with other drugs have not been conducted, however, while taking it with other drugs, consultation and supervision of the doctor is necessary. As alglucosidase alpha is recombinant human protein, drug interactions by cytochrome are unlikely P450.

    Special instructions:

    Since taking the drug can lead to allergic reactions, the first time you must have immediate access to all means for first aid in the reaction of hypersensitivity. Patients who had any negative effects at the first administration should carefully monitor the doctor at the second injection to avoid the consequences of an allergic reaction to the drug.

    If the patient has cardiovascular disease, he needs additional advice and supervision of the doctor, since this drug can cause many side effects from the cardiovascular system.

    Influence on the ability to drive vehicles and control mechanisms: Studies on the effect of the drug on the ability to drive and use mechanisms have not been conducted. On the day of intravenous infusion, special care should be taken in connection with possible dizziness.

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