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Clinico-pharmacological groupBlood coagulation factor ix
Treatment and prevention of bleeding in patients with hemophilia A (congenital coagulation factor VIII deficiency) aged 12 years and older.
Note: Octofactor does not contain von Willebrand factor. therefore not indicated for the treatment of von Willebrand disease.
1 bottle of the drug contains:
active ingredient: moroctic alpha 2000 ME;
excipients: sodium chloride -36.0 mg, sucrose - 12.0 mg histidine - 6.0 mg, calcium chloride dihydrate (in terms of anhydrous substance) -1.0 mg, poloxamer 407 - 0.4 mg.
1 bottle of solvent contains: sodium chloride solution of 0.9% for injection - 5 ml.
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Dosage and Administration
Treatment should be carried out by a physician experienced in the treatment of hemophilia.
The dose and duration of replacement therapy depend on the degree of deficiency of factor IX, localization and severity of bleeding, as well as the clinical picture.
The amount of factor IX administered is expressed in ME, which correspond to the current WHO standard for preparations containing blood coagulation factor IX. The activity of factor IX in the blood plasma is expressed either as a percentage (corresponds to normal human blood plasma) or in IU (corresponds to the International Standard for factor IX in blood plasma).
1 ME of factor IX activity is equivalent to the same amount of factor IX in 1 ml of normal human plasma. The calculation of the required factor IX vines is based on the results of empirical studies, which showed that the administration of factor IX at a dose of 1 ME / kg body weight increases the activity of this factor in the blood plasma by 1% of its normal activity.
The required dose of the drug is calculated by the following formula:
Required dose of the drug (IU) = body weight (kg) × desired level of increased activity of factor IX (% or IU / dl) × 1.0 *
* - the reciprocal of the observed recovery.
When determining the amount of factor IX, which should be assigned, the choice of method of administration, as well as the frequency of administration of the drug, you should always focus on the therapeutic effect of the drug in each individual case.
When bolus administration of factor IX rarely requires its appointment more than 1 time / day.
In the case of the development of the following cases of bleeding, the activity of factor IX should not decrease below the plasma level of activity established for the corresponding period (in% of normal activity or IU / dl).
Table 1. Dosage regimen for short-term intravenous injection for bleeding episodes and surgical interventions.
|The severity of bleeding / Type of surgical procedure||Required factor IX level (% or IU / dl)||The frequency of drug administration (hours) / duration of treatment (days)|
|Early hemarthrosis, muscle bleeding or bleeding from the mouth||20-40||Repeated administration every 24 hours / For at least 1 day, until the bleeding stops (based on pain syndrome) or healing.|
|More extensive hemarthrosis, muscle bleeding or hematoma||30-60||Repeated infusions every 24 hours for 3-4 days or more until the pain disappears and the ability to work is restored.|
|Life threatening bleeding||60-100||Repeated infusions every 8-24 hours until the threat to life disappears.|
|Small surgical interventions, including tooth extraction||30-60||Every 24 h / for at least 1 day, until healing|
|Large surgery||80-100 (pre- and postoperative period)||Repeated infusions every 8-24 hours until a sufficient degree of wound healing is achieved, then treatment is continued for at least 7 days to maintain factor IX activity in the range from 30% to 60% (IU / dl).|
Table 2. Dosing regimen for prolonged infusion during surgical interventions.
|The desired levels of factor IX to ensure hemostasis||40-100% (or ME / dL)|
|To achieve the desired level of factor IX, a loading dose is initially administered.||A single bolus dose of 90 IU / kg (range 75-100 IU / kg) or dosing based on the pharmacokinetic parameters of the drug.|
|Frequency of drug administration||Continuous IV infusion, depending on clearance and established values of the level of factor IX.|
|Duration of treatment||Up to 5 days.
Depending on the type of surgery, a longer treatment may be required.
During the course of treatment in order to establish the necessary dose of the drug and the frequency of repeated infusions it is recommended to properly determine the levels of factor IX in the blood.In particular, with extensive surgical interventions, it is necessary to carefully monitor replacement therapy by means of blood coagulation analysis (factor IX activity in the blood plasma). In individual patients, the response to therapy with factor IX may differ, while they achieve different indicators of recovery of the drug activity in vivo, and there is a different duration of T1 / 2.
For long-term prophylaxis of bleeding in patients with severe hemophilia B, the usual dose of the drug is 20-40 IU / kg with an interval of 3-4 days. In some cases, especially in young patients, it may be necessary to reduce the duration of the intervals between administration of the drug or use in higher doses.
Patients should be monitored for the formation of factor IX inhibitors. If it is not possible to achieve the expected level of activity of factor IX in the blood plasma or in the absence of control over bleeding during the use of the drug in recommended doses, then an analysis should be performed to determine inhibitors of factor IX. In patients with a high concentration of factor IX inhibitor, treatment may not be effective, and in this case, the use of other treatments should be considered.
The safety and effectiveness of Mononayn in children with prolonged infusion has not been studied. In this regard, the possibility of using long-term infusion of Mononayn in children and adolescents should be considered only if, before performing surgery, data on the pharmacokinetics of the drug (ie, increasing recovery of activity and clearance) to determine its dose are obtained, and careful control of drug concentration in the blood during the postoperative period.
Rules for the preparation of solutions for injection and infusion and drug administration
Recovery of the preparation and removal of the prepared solution from the vial should be performed under aseptic conditions.
As a rule, the solution is clear or slightly opalescent. After filtering or extracting the solution, as well as prior to administration, the reconstituted preparation should be inspected visually for the presence of solid particles and discoloration. Do not use turbid solutions or solutions containing precipitate (precipitate / particles).
Bring the solvent to room temperature. Remove caps from the bottle with the concentrate and the bottle with the solvent; process tubes aseptic solution. It is necessary to wait for the corks to dry before opening the package with the solvent addition device with a built-in filter *.
1. Removing the cover, open the blister pack with a device with a built-in filter to add solvent.
2. Carefully, without touching the tip of both ends of the double-pointed needle, remove the device from the package. Put the vial with solvent on a flat and clean surface and squeeze tightly in your hand. Thrust the vial with the solvent into the blue end of the device.
3. Put the vial with the lyophilisate on a flat surface. Invert the vial with the solvent with a device added to it to add the solvent, and then pierce the stopper of the vial with the lyophilisate with a rod on the transparent part of the device. The solvent will automatically flow into the vial with the lyophilisate.
4. Without separating the vials, gently rotate the lyophilized vial to make sure that the lyophilisate is completely dissolved. Do not shake the vial.
5. Unscrew the solvent addition device with the built-in filter into two parts, squeezing the end of the system in one hand, attached to the lyophilized vial, and in the other hand - the end of the system attached to the solvent bottle. Bleed air into an empty sterile syringe. Holding the vial in a vertical position with the stopper up, attach the syringe to the device to add solvent.Inject air into the vial with the reconstituted solution of the drug.
6. Keeping the syringe plunger pressed, turn the system over and, slowly pulling the plunger, dial the reconstituted solution of the drug into the syringe.
7. After the reconstituted solution of the drug is collected in the syringe, squeeze the syringe cylinder tightly (with the piston down) and disconnect the device with a built-in filter from the syringe to add a solvent.
* - solvent addition device with built-in filter is intended for single use; Do not use the device in case of damage to the package and after the expiration date, which is indicated on the paper part of the blister packaging as follows: "EXP. year-month"
Introduction Before administration, Mononine should be heated to room temperature. Mononine should be administered IV slowly to identify immediate type allergic reactions. If any possible drug-related reaction occurs during the administration process, the infusion rate should be reduced until the administration is discontinued, depending on the clinical picture.
In the case of short-term administration, the drug is administered intravenously slowly with a speed comfortable for the patient (maximum injection rate is 2 ml / min).
With prolonged infusion restoration of the lyophilisate is carried out as described above. After recovery, Mononayn can be used as a long-term infusion undiluted using an infusion pump or perfuser (syringe pump for long-term infusions).
Undiluted reconstituted mononine activity is approximately 100 IU / ml.
To obtain a dilute solution, dilute the reconstituted filtered solution by transferring the appropriate amount of the preparation to an isotonic solution of the desired volume under aseptic conditions.
In diluted solutions in a ratio of 1:10 (concentration of factor IX 10 ME / ml) the activity of factor IX remains stable up to 24 hours.
At higher dilutions, the activity of factor IX may decrease. To maintain the required level of factor IX in the blood should be monitored for its activity.
Example for dilution of 500 IU of reconstituted Mononine solution
|The target activity of the diluted solution||10 IU / ml||20 IU / ml|
|Volume of restored Mononayn||5 ml||5 ml|
|The required volume of isotonic solution||45 ml||20 ml|
|The resulting breeding||1:10||1:5|
Example for diluting 1000 IU of reconstituted Mononine solution
|The target activity of the diluted solution||10 IU / ml||20 IU / ml|
|Volume of restored Mononayn||10 ml||10 ml|
|The required volume of isotonic solution||90 ml||40 ml|
|The resulting breeding||1:10||1:5|
For the on / in the introduction of the drug is recommended to use infusion bags and tubes made of PVC.
The infusion solution is thoroughly mixed and checked to see if the infusion package is leaking. It is recommended to replace infusion packages every 12-24 hours with a freshly diluted solution of Mononayn.
The recommended rate of mononayne administration during prolonged infusion in order to maintain factor IX levels at a stable level (approximately 80%) is 4 IU / kg / h, but may depend on the pharmacokinetic profile of the drug in a patient and the desired target level of activity. For patients in whom clearance of factor IX is known, the rate of infusion can be calculated individually.
Speed (IU / kg / h) = Clearance (ml / h / kg) × required increase in factor IX activity (IU / ml).
After reconstitution, the solution should be injected immediately, taking into account microbiological safety. However, the reconstituted solution is physically and chemically stable for 24 hours at a temperature not exceeding 25 ° C.
The reconstituted solution of Mononain 500 ME after dilution of 1:10 is stable for 24 hours.
Determination of the frequency of adverse reactions: very often (≥ 1/10); often (≥ 1/100,
On the part of the urinary system: very rarely - nephrotic syndrome after unsuccessful induction of immunological tolerance in patients with hemophilia B, in the blood of which factor IX inhibitors have been determined and in which an allergic reaction has been observed in history.
Since the appointment of factor IX drugs, there is a potential risk of thromboembolic complications, and the risk is higher when using low-purity drugs. When using low-clearance factor IX preparations, cases of myocardial infarction, DIC, venous thrombosis, and pulmonary embolism were observed. When using highly purified factor IX, such side effects are rarely observed.
Allergic reactions: in patients who received preparations containing factor IX, rarely - angioedema, burning sensation (irritation) and heat, or phlebitis at the injection / infusion site, chills, skin flushing, generalized urticaria; severe anaphylactic reactions occurred in close temporal association with the formation of factor IX inhibitors. In the framework of post-marketing use it is rare - the formation of neutralizing antibodies (inhibitors) to factor IX. The appearance of such inhibitors will manifest itself in the form of an insufficient clinical response to the drug.
In such cases, it is recommended to contact a specialized center for the treatment of hemophilia. In one clinical study, 2 out of 51 (4%) patients who had not previously received factor IX treatment had observed the formation of inhibitors to the drug, in one of these patients the formation of these inhibitors was associated with an anaphylactoid reaction in 2 cases.
General reactions: rarely fever.
- known allergic reaction to mouse protein;
- high risk of thrombosis or disseminated intravascular coagulation;
- Hypersensitivity to the drug.
No interaction between human coagulation factor IX and other drugs has been observed.
There are limited data on the use of ε-aminocaproic acid after the initial infusion of Mononayn, conducted with the aim of preventing or treating bleeding from the oral cavity due to trauma or dental procedures (for example, tooth extraction).
Mononayn should not be mixed with other drugs, with the exception of isotonic solution.
Pregnancy and Lactation
Studies on animals of the effect of coagulation factor VIII on the reproductive system were not conducted. Given the rare occurrence of hemophilia A in women, there are no data on the use of drugs of coagulation factor VIII during pregnancy and lactation. In this regard, the drug Octofactor should be used only in the presence of strict indications.
When using Mononayn, as well as any protein preparation intended for IV injection, hypersensitivity reactions may occur.
Mononayne contains traces of mouse protein (mouse monoclonal antibody, which is used in the process of purification of the drug). Despite the fact that the concentration of mouse protein is extremely low (≤ 50 kg mouse protein / 100 ME), infusion of a preparation containing such proteins can theoretically cause hypersensitivity reactions.
It is necessary to inform the patient about early signs of hypersensitivity reactions, including rash, generalized urticaria, chest tightness, wheezing, arterial hypotension, anaphylaxis. If these symptoms occur, patients should be advised to immediately discontinue use of the drug and consult their physician.
In the event of shock, treatment should be carried out in accordance with modern medical standards for the treatment of shock, conditions.
Mononine 500 contains up to 10.2 mg of sodium per 500 ME, and Mononine 1000 contains up to 20.3 mg of sodium per 1000 ME. This should be considered when using the drug in patients who are on a diet with a controlled sodium content.
After re-use of human coagulation factor IX drugs, the patient’s condition should be carefully monitored for the formation of neutralizing antibodies (inhibitors), the concentration of which should be determined in Bethesda units (CU) using an appropriate biological test.
There are published data that show the relationship between the formation of an inhibitor of factor IX and allergic reactions. In this regard, patients who have experienced allergic reactions should be examined for the presence of an inhibitor of factor IX. It should be borne in mind that patients who have factor IX inhibitors in their blood may have an increased risk of anaphylaxis on subsequent contact with factor IX.
Due to the risk of allergic reactions when using factor IX concentrates, the initial administration of the drug should be carried out as prescribed by the attending physician, under medical supervision, in conditions that allow for the urgent treatment of allergic reactions.
Due to the fact that the use of concentrates of factor IX complex was previously accompanied by the development of thromboembolic complications, and the risk of their occurrence was higher with the use of low-clearance drugs, the use of factor IX drugs may pose a potential danger for patients who have symptoms of fibrinolysis, and for patients with DIC syndrome.
Due to the possible risk of thrombosis, as a complication of treatment with factor IX, when prescribing the drug for patients with liver disease, patients in the postoperative period, newborns or patients with an increased risk of thrombotic events or DIC, the condition is monitored to identify early signs of blood clots and coagulopathy consumption with the performance of the corresponding analysis. In each of these situations, the expected benefits of mononine therapy and the risk of developing these complications should be weighed.
Use in Pediatrics
There are no data regarding the safety and efficacy of long-term infusions of factor IX (in particular, the formation of inhibitors to factor IX) in children.
Standard measures to prevent patients from becoming infected as a result of using medical preparations prepared from human blood or plasma include donor selection, screening of individual donor plasma samples and plasma pools for specific markers of infectious diseases and introducing effective production steps to inactivate / remove viruses. Despite the measures taken in the case of the use of preparations prepared from human blood or plasma, the likelihood of transmitting pathogens of infectious diseases cannot be completely excluded. The possibility of infection also applies to unknown viruses or pathogens.
It is believed that the measures taken are effective against viruses that have a coating like HIV, hepatitis B virus and hepatitis C.
The measures taken may have limited effectiveness against non-enveloped viruses, such as the hepatitis A virus and parvovirus B19. Infection caused by parvovirus B19 can have serious consequences for pregnant women (fetal infection), for people with immunodeficiency or increased erythropoiesis (for example, hemolytic anemia).
Patients who regularly and / or repeatedly receive blood clotting factor IX preparations obtained from human plasma should be given appropriate vaccination (hepatitis A and B).
Each time a patient is prescribed Mononine, the name and serial number of the drug should be recorded.
Within the specified expiration date, it is possible to store the drug at room temperature (not above 25 ° C) for 1 month, after which the drug is not subject to use. Dates of the beginning and end of storage of the drug at room temperature is recorded on a carton box. Do not place in the refrigerator after storage at room temperature.
Influence on ability to drive motor transport and control mechanisms
Influences on ability to steer vehicles or moving mechanisms are not marked.
No cases of overdose of human coagulation factor IX have been reported.
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