Sebelipase alfa (Sebelipase alfa)

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
  • Kanuma
    concentrate d / infusion 
    Alexion Europe CAC     France
    • АТХ:

    A.16.A.B.14   Sebelipase alfa

    Pharmacodynamics:

    The recombinant human lysosomal acid lipase, binds to receptors on cell lysosomes, catalyzes the hydrolysis of esters of cholesterol and triglycerides to glycerol, free cholesterol and fatty acids. Increases amount aminotransferase, amplifies malabsorption, prevents the development of dyslipidemia, atherosclerosis, liver pathologies: jaundice, hepatomegaly, fibrosis, steatosis, cirrhosis.

    Pharmacokinetics:

    After intravenous administration, the maximum concentration in the blood plasma is achieved through 1.1-1.3 hours.

    It is metabolized by peptide hydrolysis.

    Decrease of the level alanine transaminases observed after 2 weeks, triglycerides and Low-density lipoprotein cholesterol - 8 weeks after the start of treatment.

    The half-life is 6 minutes.

    Indications:

    It is used as a replacement therapy for a deficiency of lysosomal acid lipase.

    ICD-10

    XVIII.R83-R89.R89.0   Abnormal enzyme content

    Contraindications:

    Pregnancy and lactation, individual intolerance to chicken protein.

    Carefully:

    Hypersensitivity.

    Pregnancy and lactation:

    Recommendations for FDA - category is not defined.Contraindicated in pregnancy and lactation.

    Dosing and Administration:Infants from 1 to 6 months of life: intravenously drip for 2 hours, 1 mg / kg body weight once a week.

    Adults: intravenously drip for 2 hours, 2 mg / kg of weight 1 every 2 weeks. If necessary, the dose can be increased to 3 mg / kg once a week.

    The highest daily dose: 3 mg / kg.

    The highest single dose: 3 mg / kg.

    Side effects:

    Central and peripheral nervous system: headache, dizziness, irritability, insomnia.

    Respiratory system: shortness of breath, nasal congestion, sneezing, cough.

    Hematopoietic system: anemia.

    The cardiovascular system: hypotension, tachycardia.

    Digestive system: dry mouth, diarrhea, abdominal pain.

    Musculoskeletal system: arthralgia.

    Dermatological reactions: a rash.

    Sense organs: blurred vision, otitis media.

    urinary system: infection of the urinary system.

    Reproductive system: Menorrhagia.

    Allergic reactions.

    Overdose:

    During the infusion of bronchospasm, apnea, respiratory distress, hives on the face.

    Treatment is symptomatic.

    Interaction:

    Clinically significant interactions are not described.

    Special instructions:

    Before the infusion of amylipase alpha, a premedication with antihistamine drugs is necessary.

    When taking the drug is not recommended driving and working with moving machinery.

    Instructions
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