Universal reference book for medicines

Active substance: imiglucerase

Type: Enzyme preparation

Manufacturer: GENZYME (United Kingdom)
Description of the active substance:
This information is a reference and it is not enough that the drug has been prescribed by a doctor ..

Recombinant macrophage-targeted beta-glucocerebrosidase is an analog of human lysosomal beta-glucocerebrosidase.
Purified imiglucerase is a monomeric glycoprotein, consisting of 497 amino acid residues and an oligosaccharide component. Gaucher's disease is characterized by a functional deficiency of the enzymatic activity of beta-glucocerebrosidase and leading to the accumulation of lipid glucocerebroside in macrophages that become overcrowded and are called "Gaucher cells." Gaucher cells are usually found in the liver, spleen, bone marrow, sometimes in the lungs, kidneys and intestines. Secondary hematologic manifestations include severe anemia and thrombocytopenia, which are combined with characteristic progressive hepatosplenomegaly. Bone changes are common and often the most debilitating and disabling effects of Gaucher disease. These include: osteonecrosis, osteopenia with secondary pathological fractures, insufficient remodeling, osteosclerosis. Imiglucerase compensates for the functional insufficiency of the enzymatic activity of beta-glucocerebrosidase: catalyzes the hydrolysis of lipid-glucocerebroside, resulting in the formation of glucose and ceramide (in accordance with the normal pathway of fat degradation).
After intravenous administration of 4 doses (7.5, 15, 30, 60 U / kg) of imiglucerase for 1 hour, a steady increase in enzyme activity is achieved by 30 minutes.
After completion of the infusion, the enzymatic activity of the plasma is rapidly reduced.
V d is 0.09-0.15 l / kg.
T 1/2 - from 3.6 to 10.4 minutes. The clearance varies from 9.8 to 20.3 ml / min / kg.
Gaucher's disease of type I (for prolonged enzyme replacement therapy in patients with a confirmed diagnosis having clinically significant manifestations of the disease: anemia (after exclusion of other causes), thrombocytopenia, pathological changes in bones (after exclusion of other causes such as vitamin D deficiency, hepato- or splenomegaly).

Given the heterogeneity and multisystem nature of Gaucher disease, the dose is set individually after a clinical evaluation of all manifestations of the disease.

Enter into / in the drip.

The initial dose is 60 units / kg once every 2 weeks.
(an average of 6 months, there is an improvement in hematological signs and symptoms from the internal organs).Continuation of treatment contributes to the suspension of the progression of the disease and a decrease in the severity of symptoms from the bone system. Use in a dose of 2.5 units / kg 3 times per week. or 15 units / kg once every 2 weeks. contributes to the improvement of hematological parameters, the decrease in the size of the liver and spleen, but does not affect the symptoms of the osseous system.
The control examination of patients is recommended to be performed once every 6-12 months.

The dose should be adjusted (increased or decreased) depending on the severity of the symptoms.

On the part of the digestive system: 10-16% - nausea, diarrhea, vomiting.

From the side of the central nervous system: 10-16% - headache, dizziness.

Dermatological reactions: 10-16% - skin rashes.

Allergic reactions: 3% - generalized itching, angioedema, dyspnea, bronchospasm.

Local reactions: discomfort, itching and burning of the skin, edema in the place of venepuncture.

Other: 10-16% - increase in body temperature, "tides" of blood to the face.

Hypersensitivity to the active substance.

Use with caution in pregnancy and lactation.

Use with caution in children.

Periodic monitoring of the formation of IgG antibodies to imiglucerase is necessary.
In the first year of IgG therapy, antibodies to imiglucerase are formed in approximately 15% of patients. The appearance of IgG antibodies most often occurs in the first 6 months of treatment and rarely after 12 months of therapy.
During treatment every 2-3 months, the activity of acidic phosphatase and ACE should be monitored (the duration of treatment should decrease their activity).
The effectiveness of therapy can also be controlled by the level of activity of ALT, AST every 6-12 months (a decrease in their activity also indicates a positive clinical effect). Every 6-12 months, control of the concentration of bilirubin, Ca2 +, creatinine, electrolytes and phosphorus is necessary.
To assess the effectiveness of therapy, monthly monitoring of Hb and platelet counts (an increase in their number indicates the effectiveness of therapy).
Every 6 months, you should control the size of the liver and spleen (should decrease).
Every 1 to 2 years the patient is shown MRI of long bones (with a positive clinical effect, their characteristics should be improved).
Patients with antibodies to imiglucerase are more likely to develop hypersensitivity reactions. In such cases, premedication with antihistamines and / or SCS is recommended, as well as a reduction in the frequency of infusions.
Use with caution in children.

Pharmaceutically incompatible with other medicines.

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