Included in the formulation
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 Enzyme preparations
A.16.A.B.05 Laronidase
Pharmacodynamics:Obtained by recombinant DNA technology, human alpha-L-iduronidase is a lysosomal hydrolase. After intravenous administration in the general blood stream laronidase penetrates into cellular lysosomes, where it hydrolyzes acid residues of glycosaminoglycans: heparin sulfate and dermatan sulfate.
Pharmacokinetics:Not studied.
Indications:Used for enzyme replacement therapy in type 1 mucopolysaccharidosis.
IV.E70-E90.E76.0 Mucopolysaccharidosis, type I
Contraindications:Individual intolerance.
Carefully:Patients with a history of allergic reactions to the introduction of protein preparations.
Pregnancy and lactation:Recommendations for FDA - category is not defined. There are data on the ingestion of the drug in breast milk. During pregnancy is used for life indications. During treatment with laronidase, breastfeeding should be discontinued.
Dosing and Administration:Intravenous infusions of 100 U / kg weekly, with an initial infusion rate of 2 U / kg / h, with a gradual acceleration of up to 43 U / kg / h.The total duration of infusion is not less than 3-4 hours.
The highest daily dose: 100 units / kg.
The highest single dose: 100 units / kg.
Side effects:Central and peripheral nervous system: headache.
The cardiovascular system: tachycardia.
Digestive system: rarely - pain in the abdominal cavity.
Musculoskeletal system: arthropathy, arthralgia.
Allergic reactions.
Overdose:Cases of overdose are not described.
Interaction:With simultaneous use with procaine and chloroquine, intracellular absorption of laronidase is possible.
Special instructions:Infusion of the drug should be carried out only in a hospital with equipment for continuous monitoring of vital functions and emergency medical care.
Before each infusion of laronidase, a premedication with antihistamine and antipyretic drugs is necessary.