Synonyms: Iduronate-2-sulfatase; Idursulfasa; Idursulfasum; Idusulfase. αLIduronate sulfate sulfatase.
Cyrillic synonym: Идусульфас.
💊 Chemical information
CAS — 50936-59-9.
ATC — A16AB09.
ATC Vet — QA16AB09.
Idursulfase is recombinant human iduronate-2-sulfatase used as enzyme replacement therapy in the treatment of mucopolysaccharidosis II (Hunter syndrome), a lysosomal storage disorder that results in the accumulation of glycosaminoglycans in cells with consequent progressive damage. Idursulfase is given by intravenous infusion in a dose of 500 micrograms/kg once a week. Infusion reactions are common and treatment with antihistamines with or without corticosteroids, or a reduction in infusion rate may be necessary. Stopping the infusion should be considered in severe reactions. Anaphylactoid reactions have been reported, in some cases up to 24 hours after the infusion. Idursulfase should be diluted in 100 mL of sodium chloride 0.9% and infused over 1 to 3 hours. The initial infusion rate should be 8 mL/hour for the first 15 minutes, which may then be increased by 8 mL/hour every 15 minutes if well tolerated, up to a maximum rate of 100 mL/hour. If the infusion rate is decreased because of infusion reactions, the infusion time should not exceed 8 hours because of lack of preservative in the product.
Cz.: Elaprase; Port.: Elaprase; UK: Elaprase; USA: Elaprase.
Published May 08, 2019.