Description of the active substance:
This information is a reference and it is not enough that the drug has been prescribed by a doctor ..
Recombinant antihemophilic factor. Increases the activity of clotting factor VIII in plasma, has the ability to temporarily correct the corresponding clotting disorders.
Moroktokog alpha is a glycoprotein with a molecular weight of 170 kDa, consisting of 1438 amino acids. Has a sequence of amino acids that is comparable to the 90 + 80 kDa form of coagulation factor VIII, and post-translational modifications, which are similar to those in a molecule derived from plasma. Moroktokog alpha has in vitro characteristics comparable to the endogenous coagulation factor VIII.
The activated clotting factor VIII acts as a cofactor to activate coagulation factor IX, which accelerates the formation of the coagulation factor X from coagulation factor X. The activated coagulation factor X converts prothrombin to thrombin, which then converts fibrinogen to fibrin, resulting in the formation of a fibrin clot.
In patients suffering from hemophilia A, the activity of coagulation factor VIII is significantly reduced, therefore, replacement therapy is necessary.
The effect of regular routine prophylaxis on long-term morbidity and mortality is not known.
Morocco-alpha does not contain vWF and therefore is not indicated for the treatment of von Willebrand disease.
T 1/2 of the coagulation factor VIII preparations both isolated from the blood plasma and recombinant, is about 12 hours.
Control and prevention of episodes of bleeding and to prevent bleeding during surgical interventions in patients with hemophilia A (hereditary deficiency of coagulation factor VIII or classical hemophilia).
Moroktokog alpha can be effective in the treatment of hemophilia A in some patients with coagulation factor inhibitors VIII.
Moroktokog alpha injected IV in the form of infusion.
The required dose is calculated by a special formula taking into account the patient's body weight and the desired level of coagulation factor VIII.
The dose and duration of treatment depend on the degree of severity of coagulation factor VIII deficiency, the localization and severity of bleeding, and the clinical condition of the patient. To determine the clinical response to treatment, a dose titration should be performed.
For short-term planned prophylaxis in order to reduce the incidence of spontaneous musculoskeletal bleeding in patients with haemophilia A, moroktokog alfa should be administered at least 2 times per week. In some cases, especially in children, shorter intervals between administrations or higher doses of morococcus alfa may be required. In clinical studies, the average dose for routine prophylaxis in patients aged 8-73 years was 29 В± 11 IU / kg, in patients aged less than 1 month to 52 months, 53 В± 22 IU / kg.
At episodes of a bleeding or at surgical interventions a dose establish individually, depending on localization and a bleeding degree, on volume of operation, from necessary level of coagulation factor VIII in a plasma.
Patients receiving a morococcus alpha require careful monitoring to detect clotting factor inhibitors VIII. If, with the appropriate dose of the morococcus alpha, it is not possible to achieve the expected activity of coagulation factor VIII in the plasma, or control bleeding, then an inhibition of coagulation factor VIII should be performed. If the content of the inhibitor corresponds to <10 units of Bethesda, the additional administration of morococcus alfa can neutralize the inhibitor. With an inhibitor content> 10 units Betezda, factor VIII therapy may be ineffective.
Infusion reactions: possibly - headache, fever, chills, nausea, vomiting, lethargy, manifestations of allergic reactions.
From the coagulation system: bleeding.
From the cardiovascular system: arterial hypotension, vasodilation.
On the part of the digestive system: nausea, vomiting, anorexia, diarrhea, bleeding from the digestive tract, increased activity of AST, ALT, increased bilirubin content in the blood.
Immunological reactions: the appearance of a coagulation factor inhibitor VIII, the appearance of antibodies to coagulation factor VIII, to Chinese hamster ovary cells, to murine IgG.
From the nervous system: dizziness, drowsiness.
From the respiratory system: dyspnoea, rhinitis.
Dermatological reactions: skin itching, hives, hyperhidrosis.
From the senses: a taste disorder.
From the musculoskeletal system: arthralgia, increased activity of CK in the blood.
From the urinary system: urinary tract infection.
On the part of the body as a whole: asthenia, fever, chills, headache.
Local reactions: pain at the injection site, abdominal pain, catheter infection, catheter thrombosis, catheter loss, edema, fever, pain, infusion site infusion.
Increased sensitivity to the morococcus alfa, to murine proteins, to the proteins of the Chinese hamster.
PREGNANCY AND LACTATION
Experimental and clinical studies of the safety of the use of morococcus alfa during pregnancy and lactation were not conducted. The use of moroktokoga alfa during pregnancy and lactation is possible only if there is a strong indication and after assessing the expected benefit and risk of therapy for the mother and fetus.
APPLICATION IN ELDERLY PATIENTS
In elderly patients, the dose of morococcus alfa should be selected individually.
Treatment for morococcus alfa is performed under the supervision of a physician experienced in the treatment of hemophilia A and under close control of blood coagulation (clotting factor VIII activity in plasma), especially in surgical intervention.
As with any protein preparation, with the introduction of moroktokoga alpha, it is possible to develop reactions of hypersensitivity, including local and generalized urticaria, heaviness in the chest, arterial hypotension, difficulty breathing, anaphylaxis.
With the development of any adverse reactions due to the introduction of morococcus alfa, treatment should be discontinued immediately.
Just as with the use of other coagulation factor preparations VIII, in the treatment of morococcus alpha, it is necessary to monitor the presence of inhibitors and determine their quantity in Bethesda units, using appropriate biological tests.
Moroktokog alfa contains trace amounts of mouse protein and Chinese hamster protein, therefore, during the treatment period, the possibility of developing allergic reactions to the corresponding mammalian proteins should be considered.
In micronuclear tests in mice, the absence of a mutagenic potential in morococcus alfa is shown. Other studies on mutagenicity, studies of carcinogenicity and effects on fertility have not been conducted.
In elderly patients, as in other age groups, the dose of morococcus alfa should be selected individually.