Imiglucerase

When ATH:
A16AB02

Characteristic.

Recombinant beta-glucocerebrosidase makrofagnatselennaya (imiglucerase) — аналог лизосомальной бета-глюкоцереброзидазы человека. Очищенная имиглуцераза — мономерный glycoprotein, which comprises 497 amino acid residues and oligosaccharide component.

Dried powder white or nearly white.

Pharmacological action.
Metabolic.

Application.

Enzyme replacement therapy for Gaucher disease type I with clinically significant manifestations (anemia, thrombocytopenia, scarp- and splenomegaly, pathological changes in bones).

Contraindications.

Hypersensitivity.

Restrictions apply.

Hypersensitivity or the presence of antibodies to aglyutseraze in its previous appointment.

Pregnancy and breast-feeding.

Maybe, if the effect of therapy outweighs the potential risk to the fetus.

Category actions result in FDA - C. (The study of reproduction in animals has revealed adverse effects on the fetus, and adequate and well-controlled studies in pregnant women have not held, However, the potential benefits, associated with drugs in pregnant, may justify its use, in spite of the possible risk.)

Precautions during breastfeeding.

Side effects.

Rarely: nausea, diarrhea, vomiting, headache, dizziness, fever, skin rashes, hypersensitivity reactions (3% cases): generalized itching, tides, angioedema, breathlessness, bronchospasm; discomfort, itch, burning, swelling at the site of the venipuncture.

Cooperation.

For information about the interaction with other drugs and food are absent.

Dosing and Administration.

Requires dosing regime. В/в в течение 1–2 ч из расчета не более 1 ЕД/кг за 1 m 1 once every 2 Sun.

Precautions.

In / in the introduction imiglutserazy appointed doctor, have sufficient experience in the treatment of Gaucher disease. During therapy should periodically monitor the formation of IgG antibodies to imiglyutseraze. In the first year of therapy the antibodies are produced in 15% patients (often appear in the first 6 мес и редко — после 12 months of treatment). Patients, having antibodies to imiglutseraze, recommended that a premedication with antihistamines and / or corticosteroids, reduce the frequency of infusions.

Cautions.

Not recommended for 4 weeks dose, exceeding 120 U / kg, tk. the study of their effectiveness has not been.