Universal reference book for medicines
Product name: BERIATE ® (BERIATE)

Active substance: human coagulation factor VIII

Type: A drug of factors (VIII and Willebrand) coagulation

Manufacturer: CSL BEHRING (Germany)
Composition, form of production and packaging
Lyophilizate for the preparation of a solution for intravenous administration of
white or almost white;
prepared solution from colorless to light yellow color, transparent or slightly opalescent.
1 f.

clotting factor VIII 250 IU

Excipients: glycine, sodium chloride, D (+) sucrose, calcium chloride.

Solvent: water d / and (2.5 ml).

Vials (1) complete with a solvent (1 pc.) And a device for adding a solvent with a built-in filter - packs of cardboard *.

Lyophilizate for the preparation of a solution for intravenous administration of white or almost white;
prepared solution from colorless to light yellow, transparent or slightly opalescent.
1 f.

coagulation factor VIII 500 IU

Excipients: glycine, sodium chloride, D (+) sucrose, calcium chloride.

Solvent: water d / and (5 ml).

Vials (1) complete with a solvent (1 pc.) And a device for adding a solvent with a built-in filter - packs of cardboard *.

Lyophilizate for the preparation of a solution for intravenous administration of white or almost white;
prepared solution from colorless to light yellow color, transparent or slightly opalescent.
1 f.

coagulation factor VIII 1000 IU

Excipients: glycine, sodium chloride, D (+) sucrose, calcium chloride.

Solvent: water d / and (10 ml).

Vials (1) complete with a solvent (1 pc.) And a device for adding a solvent with a built-in filter - packs of cardboard *.

* - an additional kit for intravenous administration (disposable syringe (1), needle-butterfly (1), disinfecting napkins (2)) can be additionally added) - packs of cardboard.

INSTRUCTION FOR THE SPECIALIST.

Description of the drug approved by the manufacturer for the printed edition of 2011.

PHARMACHOLOGIC EFFECT

Hemostatic drug.
The complex of coagulation factor VIII / von Willebrand factor consists of two molecules performing various physiological functions.
When administered to a patient with hemophilia, coagulation factor VIII binds to von Willebrand factor in the patient's vascular bed.
The activated blood coagulation factor VIII acts as a co-factor for the activated coagulation factor IX, accelerating the conversion of the blood coagulation factor X into an activated coagulation factor X.
Activated coagulation factor X promotes the conversion of prothrombin to thrombin.
Thrombin, in turn, converts fibrinogen into fibrin and promotes the formation of a thrombus.
Hemophilia A is a hereditary, sex-linked disorder in the blood clotting system due to a decrease in the level of the coagulation factor VIII.
The disease manifests itself in the form of profuse hemorrhages in the joints, muscles and internal organs spontaneously or as a result of accidental or surgical trauma.
The substitution therapy of the deficiency of the coagulation factor VIII in the plasma allows temporarily normalizing the content of the factor, as well as the tendency to bleeding.

PHARMACOKINETICS

Distribution

After IV introduction, the activity of factor VIII is mono- or bi-exponentially reduced.

An increase in the activity of factor VIII after the introduction of factor VIII at a dose of 1 IU / kg (gradual recovery) averaged about 2% (1.5-3% depending on individual characteristics).
The average retention time was 17 h (standard deviation 5.5 h), the AUC estimated on the basis of extrapolation was 0.4 h? kg / ml (standard deviation 0.2).
Excretion

T 1/2 is from 5 hours to 22 hours, an average of 12 hours. The average clearance is 3 ml / h / kg (standard deviation is 1.5 ml / h / kg).

Pharmacokinetics in special clinical cases

There are data on the treatment of 16 children under the age of 6 years: the clinical efficacy and safety of the drug are similar to those in older patients.

INDICATIONS

- Treatment and prevention of bleeding in patients with hemophilia A (congenital deficiency of the coagulation factor VIII);

- treatment and prevention of bleeding in patients with acquired coagulation factor deficiency VIII.

Beryate ® does not contain vWF in therapeutic concentration and is therefore not indicated for the treatment of von Willebrand disease.

DOSING MODE

Treatment should be carried out by a doctor with experience in the treatment of hemophilia.

Doses and duration of substitution therapy depend on the severity of the deficiency of the coagulation factor VIII, the localization and severity of bleeding, and the clinical condition of the patient.

The number of units of factor VIII administered is measured in ME, which corresponds to the current WHO standard for preparations containing the coagulation factor VIII.
The activity of factor VIII in plasma is expressed as a percentage (relative to normal human plasma) or in ME (relative to the International Standard for Factor VIII Content in Plasma).
1 ME of factor VIII activity is equivalent to the amount of factor VIII in 1 ml of normal human plasma.

The calculation of the required dose of factor VIII is based on an empirically determined pattern according to which 1 IU / kg of body weight increases the activity of factor VIII by approximately 2% of normal activity (2 IU / dl).
The required dose is calculated using the following formula:
Necessary number of units = body weight (kg) x required increase in factor VIII (% or IU / dl)?
0.5.
The dose and frequency of administrations are always calculated taking into account clinical effectiveness in each individual case.

In the event of subsequent bleeding, the activity of factor VIII during the relevant period should not be below the level of plasma activity (in% of normal level or in IU / dL).

Table of calculating the doses of the drug for bleeding and in surgical practice.

Severity of bleeding / Type of surgical procedure Required level of factor VIII (% or IU / dL) Frequency of administration (hours) / Duration of therapy (days)

Bleeding

Early hemarthrosis, muscle or mouth bleeding 20-40 Repeated infusion of the drug every 12-24 hours. At least 1 day, until the bleeding stops (according to pain) or healing occurs.

Greater bleeding, muscle bleeding or bruising 30-60 Repeated infusion of the drug every 12-24 hours for 3-4 days or more until the pain or severe disability disappears.

Life-threatening bleeding 60-100 Repeated infusions every 8-24 hours until the threat to life is eliminated.

Surgery

Minor, including tooth extraction 30-60 Every 24 hours, at least 1 day, until healing occurs.

Greater 80-100 (before and after surgery) Repeated infusions every 8-24 h until adequate wound healing, then therapy for at least 7 days to maintain factor VIII activity at 30-60% (IU / dl).

During the course of treatment, it is recommended to determine the level of factor VIII in order to calculate the administered dose and the frequency of infusions.With extensive surgical intervention, monitoring of substitution therapy with coagulation analysis (factor VIII activity) is mandatory.
There is a significant individual variability in the response to treatment with factor VIII, in vivo there are different recovery rates and T 1/2 .
With the long-term prophylaxis of bleeding in patients with severe hemophilia A, factor VIII is normally administered at a dose of 20-40 IU / kg with an interval of 2-3 days.
In some cases, especially in young patients, shorter intervals and higher doses may be required.
Calculation of the doses of the drug is based on body weight, so when determining the doses in children the same calculation formulas are used as in adults.
When determining the frequency of drug administration, clinical effectiveness should be considered in each individual case. There is some experience in treating children younger than 6 years. The frequency of drug administration should be calculated on the basis of clinical efficacy in each individual case.
It is necessary to monitor the production of factor VIII inhibitors in patients.
If the expected level of factor VIII activity is not achieved with the preparation or if bleeding is stopped when the calculated dose is administered, an analysis should be made for the presence of factor VIII inhibitors. In patients with a high content of inhibitors, factor VIII therapy may not be effective, in which case alternative treatment should be considered. These patients should be administered by physicians with experience in the treatment of patients with hemophilia.
Rules for the preparation of solution and for / in the administration of the drug

The reconstituted lyophilizate solution varies from clear to slightly opalescent.
After filtration and before administration, it is recommended to visually inspect the reconstituted product for the presence of particles and discoloration. Do not use a cloudy solution or solution containing a precipitate (small particles).
The opening of the package and the reconstitution of the solution are carried out under aseptic conditions.

Remains of the drug and packaging after use should be disposed of in accordance with local requirements.

Solution recovery

Heat the solvent to room temperature.
Make sure that the caps from the vial with the solvent and the preparation are removed, the plugs are treated with an antiseptic solution and dried before opening the device for adding a solvent with a built-in filter. The device for adding a solvent with an integrated filter is intended for single use; Do not use the device in the event of damage to the packaging after the expiry date indicated on the paper part of the blister package as follows: "EXP.year-month."
1. Open the blister pack of the solvent addition device with the built-in filter.

2. Place the solvent bottle on a flat, clean surface and hold the bottle tightly.
Take a device for adding a solvent with a built-in filter, along with a blister pack and a sharp core of the blue part of the device, to pierce the solvent plug.
3. Carefully remove the blister pack from the solvent addition device.
Make sure that only the packaging is removed, not the device itself.
4. Place on the flat surface the vial with the preparation and turn over the vial with the solvent together with the device for adding the solvent attached to it, then pierce the tube of the vial with the lyophilizate with a sharp rod of the transparent part of the device.
The solvent will automatically move into the vial with lyophilizate.
5. Without disconnecting the two vials, gently twist the vial with the drug and make sure that the lyophilizate is completely dissolved.
Do not shake the bottle.
6. With one hand, grasp the device for adding the solvent from the side of the vial with the solution of the preparation, the other from the side of the vial with the solvent and, turning, to separate it.
Collect air with a sterile syringe. While holding the bottle in the upright position, attach the syringe to the device for adding the solvent with
built-in filter.
Insert air into the vial with the drug solution.
7. Then turn the vial with the syringe, and gently pull the syringe piston into the reconstituted solution.

8. After the reconstituted solution is put into the syringe, grab the syringe cylinder (holding the syringe down with the piston) and detach the device for adding the solvent from the syringe.

For the injection of Beriaté ® it is recommended to use disposable plastic syringes, since the solution can remain on the glass walls of the all-glass syringes.

The drug should be heated to room temperature or body temperature before administration.

The drug is administered IV slowly with a speed that does not cause any discomfort to the patient, after making sure that the blood does not enter the syringe with the drug.
The rate of administration of the drug should not exceed 2 ml / min.
If a patient has a reaction that can be caused by the introduction of Beriathe ® , it is necessary to reduce the rate of drug administration or to stop the administration depending on the clinical condition of the patient.

The reconstituted solution remains chemically and physically stable for 8 hours at a temperature of 25 ° C.
However, from the point of view of biological stability, the reconstituted solution should be administered immediately after dilution. Storage of reconstituted solution should not exceed 8 hours at room temperature.
SIDE EFFECT

Determination of the frequency of adverse reactions: very often (? 1/10);
often (? 1/100, <1/10); sometimes (? 1/1000, <1/100); rarely (? 1/10 000, <1/1000); very rarely (<1/10 000, including individual cases).
Allergic reactions: very rarely - angioedema, burning and tingling at the site of infusion, chills, redness, generalized urticaria, severe anaphylactic reactions, including shock, hypersensitivity reactions (arterial hypotension, tachycardia, drowsiness, anxiety, nausea, vomiting, chest pain, tingling.In patients with hemophilia A, neutralizing antibodies (inhibitors) can be produced to factor VIII.The emergence of antibodies leads to clinical inefficiency of drug treatment.
In these cases specialized care is recommended in the center of hematology.
Since the clinical experience of the use of Beriathe in previously untreated patients is very limited, the frequency of cases of specific inhibitors with ineffective treatment is unknown.

Common reactions: very rarely - fever.

CONTRAINDICATIONS

- Hypersensitivity to the components of the drug.

PREGNANCY AND LACTATION

Due to the fact that hemophilia A is rare in women, there is no experience of using the drug during pregnancy during lactation.
Therefore, the use of the drug during pregnancy and lactation (breastfeeding) is possible only if there are indisputable indications.
SPECIAL INSTRUCTIONS

When applying Beriathe ® , as well as any preparation for intravenous administration containing protein, it is possible to develop hypersensitivity reactions of the allergic type.
Patients should be informed of early symptoms of hypersensitivity reactions such as rash, generalized urticaria, chest compressions, wheezing, arterial hypotension and anaphylaxis. When these symptoms appear, patients are advised to consult a doctor immediately, the drug should be discontinued.
It is necessary to follow standard methods of treatment of shock in case of development of shock state.

Beryate ® contains up to 28 mg of sodium per 1000 IU, which should be considered with the recommended salt-free diet.

Viral safety

Standard measures to prevent the transmission of infections due to the use of medicines made from human blood or plasma consist of the selection of donors, screening of individual, donor and plasma pools for the presence of markers, as well as the inactivated / deleted viruses included in the production process.
Despite this, with the use of drugs derived from human blood or plasma, the emergence of infectious diseases due to transmission of infectious agents can not be completely ruled out. This provision applies to unknown viruses and pathogens.
The measures taken are effective for enveloped viruses, such as HIV, hepatitis B and C viruses.

The measures taken may be less effective for viruses without envelope, such as hepatitis A virus and parvovirus B19.

Infection caused by parvovirus B19 can have serious consequences for pregnant women (infection of the fetus) and those with immunodeficiency or enhanced erythropoiesis (hemolytic anemia).

Patients who regularly / re-take Factor VIII preparations derived from human plasma are advised to receive appropriate vaccinations against hepatitis A and B.

The development of neutralizing antibodies (inhibitors) against factor VIII is a known complication in the treatment of patients with hemophilia A.

Inhibitors of the coagulation action of factor VIII are usually IgG immunoglobulins, measured in units of Bethezda (BE) in 1 ml of plasma.

The risk of producing inhibitors is related to the degree of exposure to anti-hemophilic factor VIII and is greatest during the first 20 days of use.

In rare cases, inhibitors can be produced within the first 100 days of the use of factor VIII.

Patients receiving a human blood coagulation factor VIII should be under close medical control to detect the production of antibodies to Factor VIII.

Each time the introduction of Beriathe ®, it is necessary to fix the serial number of the product used.

Impact on the ability to drive vehicles and manage mechanisms

The effect of the drug on the ability to drive vehicles or moving mechanisms is not noted.

OVERDOSE

Symptoms of an overdose of the human factor of blood coagulation VIII were not observed.

DRUG INTERACTION

There are no reports of cases of drug interaction of the coagulation factor VIII with other drugs.

Pharmaceutical incompatibility

Do not mix the drug with other medicinal products and solvents in one bottle.

TERMS OF RELEASE FROM PHARMACY

The drug is released by prescription.

TERMS AND CONDITIONS OF STORAGE

The drug should be stored out of reach of children, protected from light at a temperature of 2 ° to 8 ° C;
Do not freeze. Shelf life - 2 years.
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