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.09 Idursulfase
Pharmacodynamics:Hydrolyses 2-sulfate esters, which are intermediate products of cleavage of glycosaminoglycans (dermatan sulfate and heparan sulfate) in lysosomes of various cell types, which leads to internalization of the enzyme targeted at intracellular lysosomes and subsequent catabolism of accumulating glycosaminoglycans.
Pharmacokinetics:After intravenous administration for 1 hour at a dose of 0.15 mg / kg, the maximum concentration in the blood plasma is 1.5 μg / ml.
The half-life is 44 minutes.
Indications:It is used for substitution fermentotherapy with Mucopolysaccharidosis type 2 (Hunter syndrome).
IV.E70-E90.E76 Disorders of glycosaminoglycan metabolism
Contraindications:Individual intolerance, children under 5 years.
Carefully:Age over 60 years.
Pregnancy and lactation:Recommendations for FDA - Category C. Contraindicated in pregnancy and lactation.
Dosing and Administration:Intravenously drip for 1 hour, 500 μg / kg body weight once a week.
The highest daily dose: 500 mcg / kg.
The highest single dose: 500 mcg / kg.
Side effects:Central and peripheral nervous system: headache.
Respiratory system: cough.
Digestive system: diarrhea, abdominal pain.
Musculoskeletal system: arthralgia.
Sense organs: otitis media.
Allergic reactions.
Overdose:During the infusion of bronchospasm, apnea, respiratory distress, hives on the face.
Treatment is symptomatic, discontinuation of infusion.
Interaction:Clinically significant interactions are not described.
Special instructions:Before the infusion of idursulfase, it is necessary to perform a premedication with antihistamines, the action of which prevents the risk of developing an anaphylactic reaction.