Universal reference book for medicines

Active substance: idursulfase

Type: The drug for the treatment of hereditary enzymatic insufficiency

Manufacturer: Shire Human Genetic Therapies (USA)
Description of the active substance:
This information is a reference and it is not enough that the drug has been prescribed by a doctor ..

A remedy for enzyme replacement therapy.
It is a human lysosomal enzyme idoronate-2-sulfatase, which is produced using recombinant DNA technology on the human cell line.
Idur sulphase is an enzyme that hydrolyses 2-sulfate esters, which are intermediate products of cleavage of hypocarcinoglycans-dermatan sulfate and heparan sulfate in lysosomes of various cell types.

It is shown that idursulfase improves walking ability in patients with type 2 mucopolysaccharidosis (Hunter syndrome).

With a single infusion for 1 hour, the AUC value of idursulfase is directly proportional to the dose at a dose range of 0.15 mg / kg to 1.5 mg / kg.
C max - 1.5 μg / ml, T 1/2 - 44 min, clearance - 3 ml / min / kg.
Mucopolysaccharidosis type 2 (Hunter syndrome).

It is used as an IV infusion.
The recommended dose is 500 μg / kg body weight 1 time / week.
Most often: > 30% hyperpyrexia, headache, arthralgia.

Very often: > 10% - pain in the extremities, itching, hypertension, general malaise, shortness of breath, visual disturbances, abscess, musculoskeletal system disorders, pain in the pectoral muscles, urticaria, abrasions on the skin, anxiety, irritability, indigestion, swelling at the injection site.
The formation of antibodies to idursulfase was observed, but their effect on the efficacy of treatment was not established.
When used according to the indications in the recommended doses, there are no contraindications.

Safety of use in pregnancy has not been studied.
It is not known whether idursulfase is excreted in human milk.
The safety and effectiveness of the use of idursulfase in children under 5 years of age have not been studied.

During the administration of idursulfas, there is a high risk of life-threatening anaphylactic reactions, so infusion should be performed under conditions that allow for immediate therapy and clinical monitoring.

After arresting the anaphylactic reaction during or immediately after the infusion, it is possible to re-develop the anaphylactic reaction 24 hours after the infusion.Therefore, patients who have had initial infusion reactions require longer medical control.

With the development of infusion reactions in connection with the first infusion, before or during subsequent infusions should be premedication with antihistamines.

The safety and effectiveness of the use of idursulfase in children under 5 years of age have not been studied.

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