Universal reference book for medicines
Product name: FABRAZYME

Active substance: agalsidase beta

Type: The drug for the treatment of hereditary enzymatic insufficiency

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

PHARMACHOLOGIC EFFECT
A remedy for enzyme replacement therapy.
Agalsidase beta - recombinant human alpha-galactosidase A - an enzyme in which the amino acid sequence is the same as in the endogenous enzyme. Obtained using recombinant DNA technology on Chinese hamster cells.
Agalsidase beta is effective in Fabry disease, which is a consequence of a genetically determined (on the X-chromosome) metabolic disorder of glycosphingolipids.Deficiency of the lysosomal enzyme alpha-galactosidase A leads to a progressive accumulation of glycosphingolipids, mainly globotriasylceramide (GL-3), in the tissues of the body.
Clinical manifestations of Fabry's disease are renal failure, cardiomyopathy and cerebrovascular disorders. The accumulation of GL-3 in renal endothelial cells plays a role in the development of renal failure.
The results of preclinical and clinical studies show that agalsidase beta catalyzes the hydrolysis of glycosphingolipids, including GL-3, which increases the formation of alpha-galactosidase A in patients with Fabry disease.
Agalsidase beta reduces the content of GL-3 in the capillary endothelium of the vessels of the kidneys, heart and skin.
PHARMACOKINETICS
AUC and clearance of agalsidase beta do not increase in proportion to the increase in dose, which indicates the non-linearity of the pharmacokinetics of the enzyme.Pharmacokinetic parameters are characterized by variability.

INDICATIONS
Fabry's disease.

DOSING MODE
The recommended dose is 1 mg / kg in the form of intravenous infusion once every 2 weeks.
The rate of infusion depends on the patient's body weight. The duration of treatment is determined individually.
SIDE EFFECT
The most serious reactions are allergic and anaphylactic reactions.

General reactions :?
5% - chills, fever, heat or cold, hot flushes, pain in the extremities, chest pain, sensation of a lump in the throat, peripheral swelling, back pain, pallor, face swelling, myalgia, muscle spasms.
Infections: upper respiratory tract infections, lower respiratory tract infections, sinusitis, pharyngitis, fungal infections, viral infections, local infections.

From the respiratory system :?
5% - shortness of breath, nasal congestion, congestion in the respiratory system.
From the digestive system :?
5% - nausea, vomiting, abdominal pain, diarrhea, toothache, dry mouth.
From the side of the central nervous system and peripheral nervous system :?
5% - headache, paresthesia, weakness, dizziness, drowsiness, burning sensation.
From the cardiovascular system :?
5% - arterial hypertension, tachycardia, arterial hypotension, bradycardia, thinning of the ventricular wall.
Dermatological reactions :?
5% - itching, urticaria, rash.
From the sense organs: noise in the ears, hypoacusia.

Other: increased incidence of injuries.

CONTRAINDICATIONS
High risk of development of severe reactions of hypersensitivity to agalsidase beta.

PREGNANCY AND LACTATION
Adequate and strictly controlled clinical studies on the safety of agalsidase beta during pregnancy have not been conducted.
In pregnancy, use is only possible in cases of extreme necessity, when the expected benefit of therapy for the mother exceeds the potential risk to the fetus.
It is not known whether agalsiadza beta is excreted in human breast milk.
If it is necessary to use during lactation, the expected benefit of therapy for the mother and the potential risk for the infant should be carefully weighed.
APPLICATION FOR CHILDREN
Safety of use in children is not established.

APPLICATION IN ELDERLY PATIENTS
In patients with impaired cardiac activity, a predisposition to a higher risk of developing severe complications of infusion reactions is possible.

SPECIAL INSTRUCTIONS
Infusion should be made in conditions that allow for emergency therapy.

With the development of allergic and anaphylactic reactions, immediately stop the infusion and begin emergency therapy.

When re-used in patients with anaphylactic or severe allergic reactions to the introduction of agalsidase beta in the history, the expected benefit and potential risk should be weighed.
In such cases, the introduction should be carried out with extreme caution.
If there is a risk of developing infusion reactions, premedication should be performed with antipyretic and antihistamine.

With the development of infusion reactions, the rate of infusion should be reduced, the infusion temporarily suspended and / or the additional administration of antipyretic, antihistamines and / or corticosteroids commenced.
If the infusion reactions persist, stop the infusion and immediately begin the appropriate therapy. In case of a risk of developing infusion reactions, repeated administration of agalsidase beta should be performed with caution.
In patients with impaired cardiac activity, a predisposition to a higher risk of developing severe complications of infusion reactions is possible.

Patients who are predisposed to developing allergic reactions should be tested for the presence of antibodies to IgE and carefully weigh the risk and benefit of re-administration of agalcidase beta in the presence of specific IgE antibodies agalsidase beta.
If it is necessary to apply in such cases, the introduction should be carried out under the direct supervision of qualified personnel.
Safety of use in children is not established.

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