Aldurazyme 100 U/ML 25 X5ML

Description

Aldurazyme 100 U/ML 25 X5ML

About the preparation
Purified laronidase is a glycoprotein with a molecular weight of 83 Da.

Indications and dosage
Mucopolysaccharidosis type 1 (MPS l, α-L-iduronidase insufficiency), for the purpose of prolonged enzyme replacement therapy
Treatment of manifestations of a disease not related to the nervous system
Composition and properties
Laronidase

Form of issue
Concentrate for the preparation of solution for infusion.

pharmachologic effect
Laronidase consists of 628 amino acids, the molecule contains 6 N-combined oligosaccharides, which are modified. The disruption of the accumulation of mucopolysaccharides is due to the inadequacy of certain lysosomal enzymes required for the catabolism of glycosaminoglycans. Mucopolysaccharidosis (MPS) of type I is characterized by insufficiency of α-L-iduronidase, lysosomal hydrolysis, which catalyzes hydrolysis of α-L-iduronic residue, dermathenesulfate and heparan sulphate. The reduction or absence of α-L-iduronidase activity results in the accumulation of glycosaminoglycans, dermathenesulfate and heparan sulfate in many types of cells and tissues. The expediency of replacement enzyme therapy consists in restoring the level of enzymatic activity sufficient to hydrolyze the accumulated substrate and prevents its further accumulation

The use of Aldurasim should be performed under the supervision of a physician with experience in treating patients with type 1 MPS or with other hereditary metabolic diseases. The duration of therapy is determined by the doctor, depending on the tolerability and clinical effectiveness.

Introduction Aldurasim should be performed in the appropriate clinical unit with intensive care equipment, ready for use in the event of emergence of emergency conditions.

Overdose
Any information on cases of overdose is absent.

Side effects
The most serious side effects found in clinical and post-marketing studies are anaphylactic and allergic reactions.

In clinical studies with the use of laronidase, infections of the upper respiratory tract, rashes, and reactions at the injection site were most often noted.

In clinical studies, there were also reactions associated with the administration of the drug, among which tides, fever, rash and headache were most frequently observed, less often cough, bronchospasm, dyspnea, urticaria, angioedema, itching.

Contraindications
Contraindications not established

At present, there are no well-controlled and adequate studies on the use of laronidase in pregnant women.

It is not known whether laronidase is excreted in breast milk.

Interaction with other drugs and alcohol
Drug interaction is not established.

Storage conditions
At a temperature of 2-8 ° C (in the refrigerator). Diluted solution: from the point of view of microbiological safety the preparation should be used immediately after preparation. If the preparation has not been used immediately after preparation, it can be stored for no more than 24 hours at a temperature of 2-8 ° C, provided that the preparation has been diluted in controlled and validated aseptic conditions.