EMOKLOT DI.

Active material: Blood coagulation factor VIII in complex with von Willebrand factor
When ATH: B02BD02
CCF: The drug factors (VIII and Willebrand) clotting
ICD-10 codes (testimony): D66, D68.0
At KFU: 20.01.06
Manufacturer: KEDRION S.p.A. (Italy)

Pharmaceutical form, composition and packaging

Valium for solution for infusion white or light-yellow; prepared solution is clear or slightly opalescent, colorless or light yellow.

Excipients: sodium citrate, sodium chloride, glycine, calcium chloride.

Solvent: water d / and – 5 ml.

Glass Bottles (1) together with the solvent (fl. 1 PC.) and a system for the on / in the (syringe 10 or 20 ml, igla-“butterfly” catheter, needle adapter, Needle filter, adhesive plaster) – cardboard boxes.

Valium for solution for infusion white or light-yellow; prepared solution is clear or slightly opalescent, colorless or light yellow.

Excipients: sodium citrate, sodium chloride, glycine, calcium chloride.

Solvent: water d / and – 10 ml.

Glass Bottles (1) together with the solvent (fl. 1 PC.) and a system for the on / in the (syringe 10 or 20 ml, igla-“butterfly” catheter, needle adapter, Needle filter, adhesive plaster) – cardboard boxes.

Valium for solution for infusion white or light-yellow; prepared solution is clear or slightly opalescent, colorless or light yellow.

Excipients: sodium citrate, sodium chloride, glycine, potassium chloride.

Solvent: water d / and – 10 ml.

Glass Bottles (1) together with the solvent (fl. 1 PC.) and a system for the on / in the (syringe 10 or 20 ml, igla-“butterfly” catheter, needle adapter, Needle filter, adhesive plaster) – cardboard boxes.

* Specific activity of the final product; protein – no less 80 IU / mg.

Pharmacological action

The complex of factor VIII (фVIII) and vWF consists of two molecules with different physiological functions. Activated FVIII acts as a cofactor, activating blood clotting factor IX, accelerating the conversion of clotting factor X to the active form. Activated blood coagulation factor X converts prothrombin to thrombin. Then thrombin converts fibrinogen into fibrin and the clot is formed.

Reduction of FVIII leads to the development of hemophilia A. Replacement therapy increases plasma FVIII, temporarily corrects a deficiency of factor in the blood plasma and prevents bleeding.

VWF stabilizes the FVIII molecule, promotes adhesion of platelets to the site of vascular injury, participates in platelet aggregation and needs replacement therapy of patients with von Willebrand's disease.

Pharmacokinetics

After introduction of FVIII activity in plasma reaches 80-120% required (settlement) the level of activity of this factor.

FVIII activity in plasma decreases biphasic exponential curve.

In the initial phase, distribution between vascular beds and tissues takes place with T_1/2 plasma 3-6 no; about 2/3-3/4 FVIII remain in the bloodstream.

In the subsequent slower phase (which probably reflects the consumption of FVIII) T_1/2 It varies between 8 to 20 no (average 12 no). This reflects the true biological T_1/2.

Testimony

- Treatment and prevention of bleeding, hereditary and caused by an acute shortage of coagulation factor VIII (Hemophilia A, hemophilia A with inhibitor of coagulation factor VIII, an acute shortage of coagulation factor VIII due to the spontaneous appearance of an inhibitor factor);

- Von Willebrand disease.

Dosage regimen

Treatment should be under strict medical supervision, with experience in treatment of hemophilia. Dose and duration of substitution therapy depend on the severity of deficiency of coagulation factor VIII , the localization and extent of bleeding and severity of the clinical condition of the patient.

The calculation of the required dose of factor VIII (фVIII) based on empirical data: 1IU FVIII / kg body weight increases the activity of coagulation factor in the plasma 1.5-2%. Given the initial activity of factor VIII in the patient's plasma, Calculate the required dose according to the formula:

The required dose (ME фVIII) = Patient weight (kg) x desired increase in the activity of FVIII (%) x 0.5.

The total dose and frequency of administration should always be correlated with clinical efficacy in each case.

The following table reflects the level of FVIII, necessary to stop bleeding or prevent bleeding in surgical interventions, as well as the duration of the maintenance required FVIII activity in patient plasma.

In some cases, there may be need large, rather than calculated, quantities of preparation, especially early in treatment.

During the course of treatment is recommended to determine the activity of FVIII for the correction of the dose and frequency of administration. In the case of major surgical interventions precise monitoring is particularly necessary replacement therapy, which involves determining FVIII activity in plasma. Pharmacokinetic parameters (FVIII release rate in vivo and T_1/2) different patients may be different.

Patients should be examined for the appearance of FVIII inhibitors. If the expected increase in activity of the factor in the blood plasma is not reached, or bleeding is not controlled corresponding dose, it is necessary to conduct a study on the presence of inhibitors of FVIII. If the inhibitor is present in an amount less 10 WAS / ml, the appointment of an additional amount of human coagulation factor VIII may neutralize the inhibitor. Patients with levels of inhibitor more 10 BU or high response recommended the appointment of a history of prothrombin complex concentrate.

Patients with von Willebrand's disease replacement therapy is empirically and depends on the level of FVIII in plasma, which is measured on a daily basis (before and after administration of the drug Emoklot DI). Monitoring of bleeding time is required in cases of bleeding from mucous or continuing, in spite of the adequate level of FVIII: C in blood plasma. In these patients, the dose Emoklot D. AND. necessary to increase the.

Terms of preparation and administration of the solution for infusion

Infusion solution should be prepared immediately before administration. Do not use if solution therein flake and other mechanical admixtures. The drug is administered in / for 3-5 min controlled by the patient's pulse.

To prepare the solution for infusion is necessary to add a solvent to the vial with lyophilisate through the twin needle and rotate the vial until complete dissolution. The lyophilisate can be dissolved more quickly, If the solvent is heated in a water bath to a temperature not higher than 37 ° C.. Incomplete dissolution of the drug will experience difficulty in filtering through the filter needle. To avoid foaming, slowly pour the solvent on the wall of the vial of lyophilized drug. After preparing the solution you need to dial it in a syringe, using a filter needle, then replace the needle and, using injection equipment from the supplied kit, enter the drug in / from said speed.

Side effect

Rarely: hypersensitivity reactions, fever.

Patients with hemophilia A may appear antibody (inhibitors) a blood coagulation factor VIII, which is manifested in the form of ineffective clinical response. In this case, it is recommended to contact a specialized center hemophilic.

Contraindications

- Hypersensitivity to the drug.

Pregnancy and lactation

In applying the drug in pregnant women during the clinical trials found no complications. Experimental studies animals are insufficient to assess the effects on reproduction, development of an embryo or fetus, during pregnancy, peri- and postnatal development of the child.

Therefore, the drug can be used in pregnancy and lactation only, if the expected benefit to women than the potential risk to the fetus and infant.

Cautions

In case of hypersensitivity reactions when using the drug Emoklot DI, administration of the drug should be discontinued.

Time solution for infusion should not exceed 3 m. After preparation of the solution must be used immediately. The contents of the vial should be used fully in one administration. Solution, the remaining unused, to be destroyed in the prescribed manner.

Emoklot DI. produced from human plasma. When using a plasma or products, prepared from human plasma, it is impossible to completely eliminate the transmission of infectious agents, incl. not yet known. To reduce the risk of transmission of a thorough selection of donors and plasma harvested by special testing (control of each portion of the plasma, used to produce the drug, in the absence of HIV type 1 and type 2, HbsAg , antibodies to HCV and ALT). Besides, are tested each pool of plasma RNA by polymerase chain reaction to detect HCV genome. In addition to testing in the manufacturing process uses a double viral inactivation: Solvent-detergent method, and heat treatment at 100 ° C for 30 m.

FVIII, derived from human plasma, It is a common component of human blood plasma and acts as endogenous factor. Toxicity single injection is not possible, while higher doses lead to overdosing. Determination of toxicity of repeated doses in animals is not possible due to the appearance of antibodies to the foreign protein. Clinical studies have shown no carcinogenic and mutagenic effects of FVIII, obtained from human plasma, Experimental studies in this case inexpedient.

Effects on ability to drive vehicles and management mechanisms

The drug has no effect on the ability to manage road and mechanisms.

Overdose

There is currently no data on overdose symptoms of human coagulation factor VIII.

Drug Interactions

It is not known about the interaction of the human clotting factor VIII with other drugs.

For the introduction, you can use only proven sets of injection kit supplied.

Conditions of supply of pharmacies

The drug is released under the prescription.

Conditions and terms

The drug should be stored out of reach of children, dark place at a temperature of 2 ° to 8 ° C. Shelf life – 2 year (if all the conditions of storage in the sealed containers).