Included in the formulation
Included in the list (Order of the Government of the Russian Federation No. 2782-r of 30.12.2014):VED
7 nosologies
АТХ:A.16.A.B.02 Imiglucerase
Pharmacodynamics:Macrophaglanded recombinant β-glucocerebrosidase is an analog of human lysosomal β-glucocerebrosidase. Imiglucerase purified - monomeric glycoprotein, which contains 487 amino acid residues and oligosaccharide. Enhances the processes of glycolipid hydrolysis of glucocerebroside to ceramide and glucose, preventing the accumulation of glucocerebroside in macrophages, preventing the formation of Gaucher cells.
Improves hematologic parameters and reduces the manifestations of lesions of internal organs, reduces the severity of symptoms from the side of the skeleton, inhibits the progression of the disease.
Pharmacokinetics:After intravenous administration, the maximum concentration in the blood plasma is reached after 30 minutes. Plasma clearance is 9.8-20.3 ml / min / kg, the volume of distribution: 0.09-0.15 l / kg.
The half-life is 3-10 minutes. Elimination by the kidneys.
Indications:Used for enzyme replacement therapy for Gaucher's disease of the first type with clinically significant manifestations: thrombocytopenia, anemia, hepato- and splenomegaly, pathological changes in bones.
IV.E70-E90.E75 Disorders of sphingolipids exchange and other diseases of lipid accumulation
IV.E70-E90.E75.2 Other sphingolypidosis
Contraindications:Individual intolerance.
Carefully:The presence of antibodies to imiglucerase after its previous use.
Pregnancy and lactation:Recommendations for FDA - Category C. It is used with caution in pregnancy and lactation, in cases where the expected effect of the drug exceeds the risk for the fetus and infant.
Dosing and Administration:Intravenously drip for 1-2 hours, 60 units / kg once every 2 weeks for 6-12 months. The dose is adjusted depending on the severity of the symptoms of the disease.
The highest daily dose: 120 units / kg for 4 weeks.
The highest single dose: 1 unit / kg for 1 minute 1 time in 2 weeks.
Side effects:Central and peripheral nervous system: dizziness, headache.
Respiratory system: shortness of breath, bronchospasm.
The cardiovascular system: tides.
Digestive system: nausea, diarrhea, vomiting.
Dermatological reactions: generalized skin itching, swelling and burning at the injection site.
Allergic reactions.
Overdose:Cases of overdose are not described.
Treatment is symptomatic.
Interaction:Not described.
Special instructions:For patients who have antibodies to imiglucerase, it is recommended that premedication be administered with corticosteroid or antihistamine drugs, as well as a reduction in infusion rates.