Imiglucerase

Gross

C2532-H3843-N671-O711-S16

Characteristics

Rekombinantnaya macrofagnacelennaya бета-глюкоцереброзид��за (imigluzeraza), which is analogous to lizosomalna �ета-глюкоцереброзида зы rights. Treated imigluzeraza - monomeric glikoprotein, which is composed of 497 amino acid residues and oligosaharidnyi компонент.Лиофилизир��ванный white powder, or nearly white.

Of drugs

The drugs-metabolicescoe. COMPENSATION lack functional enzyme activity бета-глюкоцереброзид��зы. Catalyzes hydrolysis glikolipida glukoterebrosida to glucose and zeramida and warns accumulation glukoterebrosida in macrofagah, i.e. hinders Goche.ispolzovanie cells in the dose of 60 U / kg once every two weeks for six months to improve haematological parameters and reduce signs of viscerotropic disease. Continued treatment delays the progression of the disease and lessen the symptoms of the severity of the skeletal system. Using the dose of 2.5 U / kg three times a week or 15 U / kg once every two weeks by the improvement of haematological parameters erosion of the liver and spleen (unchanged symptomatic of the skeletal system). Details of the impact on the reproductive function, During pregnancy and the ability to infiltrate the breast milk Otsuttuyut.posle in / with the introduction in doses of 7.5, 15, 30 or 60 U / kg for 1 h stable enzyme activity is the 30th minute and quickly weakened after infusions. T_1/2 - 3,6-10,4 mines plasma Cl - 9,8-20,3 ml / min / kg, the volume of distribution 0,09-0,15 l / kg

Indications

Ферментозаместительн ая Therapy with a Goce I type with clinically significant manifestations (anemia, thrombocytopenia, gepato- and spleen, pathological changes in bone).

Restrictions on the use of

Гиперчувствительност ь or the presence of antibodies to agluzeraze in its previous appointment.

Pregnancy and lactation

Perhaps if the effect of therapy outweighs the potential risk to the fetus. With caution in the period of breast-feeding.

Contraindications

Гиперчувствительност ь.

Side-Effects

Rarely : nausea, diarrhea, vomiting, headache, dizziness, increased body temperature, skin rashes, the reactions гиперчувствительност и (3% of the cases) : generalizovanny itch, tides, angioneuroticeski swelling, shortness of breath, bronhospazm; feeling discomfort, itching, burning, swelling at the site venopounktion.

Specific guidance

Is not recommended to use in the four weeks doses greater than 120 U / kg, as examine their effectiveness has been done.

Patient interaction

Details on the interaction with other drugs and food are not available.

Precautions

Injecting a imigluzeraza a physician with experience in treating the disease Goce. During therapy should be periodically monitored education IgG antibodies to imigluzeraze. In the first year of therapy antibodies generated from the 15% of the patients (often appear in the first six months and seldom-after 12 months of treatment). Patients with antibodies to imigluzeraze recommended holding Then antihistamines and / or korticosteroidnami drugs reduce the frequency of infusions.

Dosing and Administration

An individual dosing regime. B / for a period of 1-2 hours at the rate of not more than 1 U / kg for 1 minute every two weeks.

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