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Fabrazim is a medicinal preparation of international importance. Fabrazim is used in dermatology to treat Fabry's disease. We suggest you to consider the features of the drug, the dosage form, the effectiveness in treatment and the effect on the body.

Fabrazim has an international non-proprietary name - Agalzida Beta. Drug form of the drug - concentrate for solution. The drug is used for infusion, that is, for intravenous and intra-arterial administration. The composition of the pharabzyme includes such active substances as agalzide beta, mannitol, sodium hydrophosphate heptahydrate and sodium dihydrogen phosphate monohydrate.

The preparation is a dense powder of white color. After dissolution, the preparation becomes colorless, the solution should be obtained without foreign inclusions.

Indications Fabrazim

The main indication for the use of Fabrazim is Fabry's disease. Also, the drug is prescribed for prolonged enzyme-replacement therapy for patients with the above-described disease.

The drug is prescribed for patients who have a deficiency of α-galactosidase A in the body. The drug can be taken by adults and children over the age of eight.

Release form

The form of the drug - bottles of 5 and 35 mg. The vials are made of colorless glass and clogged with rubber stoppers with a snap-on lid. Each pack contains one bottle with instructions for use in the state and Russian language.

To preparation Fabrazim there is also a preparation lyophilizate for preparation of a concentrate. That is, this drug is used to prepare a solution for infusions from the factory. This drug is admixed with 10 bottles in one package.


Pharmacodynamics Fabrazim allows you to find out the biochemical effects and the effect of the drug on the body. The drug Fabrazim is prescribed for patients with the disease of Farbi. In this case, it is a preparation for the treatment of a polysystemic and heterogeneous disease. With the introduction of the drug, the body receives the missing amount of α-galactosidase - lysosomal hydralase. This substance catalyzes the hydrolysis of glycosphingolipids.

The drug is perfectly absorbed into the bloodstream and quickly reaches the desired effect. But, despite the high efficiency of the factory, there are a number of precautions for the use of this drug.


Pharmacokinetics Fabrazim - is the work of the drug in the body, that is, the process of digestion, metabolism and excretion. After the administration of the dose of the drug, the fibrazim is concentrated in the blood plasma and thus functions in the body.

Fabrazyme is a protein, so the drug removal process is a metabolic destruction by peptide hydrolysis. But this elimination process can cause kidney dysfunction, which has a negative effect on the pharmacokinetics of Fabrazyme. Removing the drug through the kidneys is considered to be an unimportant and safe way of removing Fabrazin from the blood.

Use Fabrazim during pregnancy

The use of Fabrazym during pregnancy is undesirable, although the effect of the drug on the female body has not been reliably studied. Despite the fact that the potential risk is not known, the drug should not be used by pregnant women unless absolutely necessary. As a rule, the use of the drug is provided only with clear indications and an analysis of the risk-benefit relationship for the mother and the future baby.

The drug is not allowed to use in the lactation period, since the substance is excreted with milk. If it is impossible to stop treatment, then it is necessary to stop breastfeeding so that the drug does not get to the baby.


The main contraindications to the use of Fabrazyme are caused by the individual intolerance of the drug and the active substances that are part of the drug.

At some patients at the first reception of a preparation there can be a giddiness, an unconscious condition, a drowsiness. That is why, when taking the drug is recommended to comply with the regime of inpatient treatment and to refuse to drive the machine and work with automatic equipment.

Side effects Fabrazim

The main side effects of Fabrazyme are caused by individual sensitivity to the components of the drug. Common side effects:

  • Pain in the back and lower back.
  • Dermatological reactions (discoloration, redness of the skin, rash or urticaria).
  • Problems with vision, lacrimation, swelling, severe itching.
  • Puffiness of the extremities, pharynx, face and other parts of the body.
  • Muscle pain, joint pain, muscle spasms, musculoskeletal stiffness.
  • Problems of cardiovascular nature (bradycardia and tachycardia).
  • Diarrhea, stomach pain, abdominal pain.
  • Pain in the chest with breathing, inflammation of the nasopharynx, cough, nasal congestion, tinnitus.
  • Increased fever, fainting, decreased sensitivity to pain.
  • Chills, nausea, vomiting, tingling in the extremities.

Dosing and administration

The method of administration and dose of the drug is prescribed by a doctor. Since the drug is prescribed for the treatment of Fabry disease, the drug is taken under medical supervision. This allows you to monitor the metabolic reactions of the body to the drug.

The recommended dose of Fabrazyme is 1 mg per kg of body weight. The drug is administered to the patient every two weeks in the form of an infusion or intravenous injection. At the same time, the initial infusion rate should not exceed 0.25 mg / min. After a while, the speed of administration of the drug can be increased, but gradually.

If the drug is prescribed to patients with renal insufficiency, then, if side effects occur, the dose of the drug is not subject to correction. The drug is very rarely prescribed to children, since the study of the effect of pharabzyme on children has not been conducted, so the drug is taken after the doctor's permission and under his supervision.


An overdose of the drug happens very rarely. An overdose can be caused by large doses of the drug and the use of pharazim not according to the doctor's prescription. In case of an overdose, you must stop using Fabrazyme and seek medical help.

Very often, with an overdose of the drug, a gastric lavage procedure is performed and infusions are made to remove the drug from the blood and body.

Interactions with other drugs

The interaction of Fabrazyme with other drugs is allowed in the event that a complex treatment is prescribed. But in pharmacology and medicine, no studies have been conducted that would confirm the possibility of interaction of pharabzyme with other drugs.

It is not recommended to take Fabrazym simultaneously with such drugs as: amiodarone, chloroquine, gentamicin, benohin. Since there is a high risk of a decrease in the activity of the active substance of the phrabrima-agalsidase beta. Particular attention should be paid to the fact that the drug is categorically prohibited from mixing with other drugs in one infusion.

Storage conditions

Storage conditions Fabrazim provide storage of the drug in a cool place with a temperature of 2-8 ° C (in the refrigerator). The drug should be kept away from sunlight and children.

If the rules for storage of the drug are not observed, Fabrazym should be disposed of. Since the storage conditions, provided spoilage of the drug, and therefore reduced its medicinal functions to zero.

Shelf life

Shelf life Fabrazim is 36 months, that is three years from the production date indicated on the package. After the expiry date, the drug must be disposed of. It is strictly forbidden to use the drug at the expiration date, as this can cause serious side effects of irreversible nature.

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