IMIGLUTSERAZA (Imiglucerase)

     Synonym : Tserezim, Cerezyme

     Description : Rekombinantnaya makrofagnatselennaya beta glyukotserebrozidaza (imiglutseraza) - analogue beta lizosomalnoy glyukotserebrozidazy rights. Treated imiglutseraza - monomeric glikoprotein, composed of 497 amino acid residues and oligosaharidny component.

Dried powder white or nearly white.

     Pharmacology : The drugs-metabolic effects.

Compensation functional deficiency of enzyme activity of beta glyukotserebrozidazy. Catalyzes hydrolysis glikolipida glyukotserebrozida to glucose and tseramida and prevents the accumulation of macrophages glyukotserebrozida, ie prevent the formation of cells Gosche.

Using the dose of 60 U / kg 1 every 2 weeks for 6 months to improve haematological parameters and reduce the signs of destruction of property. Continued treatment delays the progression of disease and reduces the severity of symptoms of bone. Using the dose of 2.5 U / kg three times a week or 15 ED / 1 kg every 2 weeks by improving haematological parameters, erosion of the liver and spleen (unchanged symptomatic of the bone system).

Details of the impact on the reproductive function during pregnancy and the ability to enter breast milk available.

After the on / in a dose of 7.5, 15, 30 or 60 ED / kg for 1 hour and sustainable enzyme activity reached by 30 th minute and quickly weakened after Infusion. T1 / 2-3,6-10,4 mines plasma Cl - 9,8-20,3 ml / min / kg, the volume of distribution-0,09-0,15 l / kg.

     Application : Fermentozamestitelnaya therapy with disease Gosche I type with clinically significant manifestations (anemia, thrombocytopenia, gepato- and an enlarged spleen, bone lesions).

     Contraindications : Giperchuvstvitelnost.

     Restrictions : To Giperchuvstvitelnost or an antibody to aglyutseraze in her previous assignment.

     Application of pregnancy and breastfeeding : Perhaps if the expected effect of treatment outweighs the potential risk to the fetus. A caution during breastfeeding.

     Side Effects : Seldom : nausea, diarrhea, vomiting, headache, dizziness, increased body temperature, skin deterioration, hypersensitivity reaction (3% of cases) : generalized itch, tides, angionevrotichesky swelling, dyspnoea, bronchospasm; Feeling discomfort, itching, burning, swelling in place venopunktsii.

     Interaction : Details of interaction with other drugs and foods available.

     Dosing and Administration : A unique dosing regime. In / in for 1-2 h at a rate of not more than one ED / kg for 1 minute 1 every 2 weeks.

     Precautions : W / imiglutserazy assigned to a doctor with experience in treating the disease Gosche. During the therapy should be periodically monitored education IgG antibodies to imiglyutseraze. In the first year of therapy antibodies are formed from 15% of patients (often appear in the first six months and seldom-after 12 months of treatment). Patients with antibodies to imiglutseraze recommended for sedation antihistamines and / or corticosteroid drugs, reduce the frequency of infusions.

     Special instructions : Do not use during the 4-week dose of more than 120 ED / kg, since the study their effectiveness has been done.

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