Mononine 1000

/ Genmedix

Treatment should be initiated under the supervision of a physician experienced in the treatment of haemophilia Posology The dosage and duration of the substitution therapy depend on the severity of the factor IX deficiency, on the .location and extent of the bleeding and on the patient’s clinical condition The number of units of factor IX administered is expressed in International Units (IU), which are related to the current WHO standard for factor IX products. Factor IX activity in plasma is expressed either as a percentage relative to normal human plasma) or in International Units (relative to an international standard for factor IX in) .(plasma One International Unit (IU) of factor IX activity is equivalent to that quantity of factor IX in one ml of normal human plasma. The calculation of the required dosage of factor IX is based on the empirical finding that 1 IU factor IX per kg body weight raises the plasma factor IX activity by 1.0 % of normal activity. The required dosage is determined :using the following formula *Required units = body weight [kg] × desired factor IX rise [% or IU/dl] × 1.0 The amount to be administered, the method as well as the frequency of administration should always be oriented to the clinical effectiveness in the individual case. Factor IX products rarely require to be administered more than .once daily when given by bolus injection In the case of the following haemorrhagic events, the factor IX activity should not fall below the given plasma activity level (in % of normal or IU/dl) in the corresponding period.
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For the prevention and control of bleeding in factor IX deficiency, also known as hemophilia B or Christmas disease.

Hypersensitivity to the active substance or to any of the excipients.Known allergic reaction to mouse protein. High risk of thrombosis or disseminated intravascular coagulation. S
ee prescribing information for full details.

As with any intravenous protein product, allergic type hypersensitivity reactions are possible. Mononine 500 and Mononine 1000 contains traces of mouse protein (the murine monoclonal antibody used in its purification process). While the levels of mouse protein are extremely low (≤ 50 ng mouse protein/100 IU), infusion of such proteins might theoretically induce hypersensitivity responses.Patients should be informed of the early signs of hypersensitivity reactions including hives, generalised urticaria,tightness of the chest, wheezing, hypotension, and anaphylaxis. If these symptoms occur, they should be advisedto discontinue use of the product immediately and contact their physician.In case of shock, the current medical standards for shock-treatment should be observed.Mononine 500 contains up to 10.2 mg sodium per 500 IU and Mononine 1000 contains up to 20.3 mg sodium per1000 IU. To be taken into consideration by patients on a controlled sodium diet.
After repeated treatment with human coagulation factor IX products, patients should be monitored for the development of neutralising antibodies (inhibitors) that should be quantified in Bethesda Units (BU) using appropriate biological testing.There have been reports in the literature showing a correlation between the occurrence of a factor IX inhibitor and allergic reactions. Therefore, patients experiencing allergic reactions should be evaluated for the presence of an inhibitor. It should be noted that patients with factor IX inhibitors may be at an increased risk of anaphylaxis with subsequent challenge with factor IX. Because of the risk of allergic reactions with factor IX concentrates, the initial administrations of factor IX should, according to the treating physician’s judgement, be performed under medical observation where proper medical care for allergic reactions could be provided. Since the use of factor IX complex concentrates has historically been associated with the development of thromboembolic complications, the risk being higher in low purity preparations, the use of factor IX-containing products may be potentially hazardous in patients with signs of fibrinolysis and in patients with disseminated intravascular coagulation (DIC). Because of the potential risk of thrombotic complications, clinical surveillance for early signs of thrombotic and consumptive coagulopathy should be initiated with appropriate biological testingwhen administering this product to patients with liver disease, to patients post-operatively, to new-born infantsor to patients at risk of thrombotic phenomena or DIC. In each of these situations, the benefit of treatment withMononine 500 or Mononine 1000 should be weighed against the risk of these complications.There is no safety and efficacy data for continuous infusion application in children, particularlythe potential for development of inhibitors is unknown.
Virus safety : Standard measures to prevent infections resulting from the use of medicinal products prepared from human blood or plasma include selection of donors, screening of individual donations and plasma pools for specific markers of infection and the inclusion of effective manufacturing steps for the inactivation/removal of viruses. Despite this, when medicinal products prepared from human blood or plasma are administered, the possibility of transmitting infective agents cannot be totally excluded. This also applies to unknown or emerging viruses and other pathogens. The measures taken are considered effective for enveloped viruses such as HIV, HBV and HCV. The measures taken may be of limited value against non-enveloped viruses such as HAV and parvovirus B19.Parvovirus B19 infection may be serious for pregnant women (foetal infection) and for individuals with immunodeficiency or increased erythropoiesis (e.g. haemolytic anaemia). Appropriate vaccination (hepatitis A and B) should be generally considered for patients in regular/repeated receipt of human plasma-derived factor IX products. It is strongly recommended that every time that Mononine 500 or Mononine 1000 is administered to a patient, thename and batch number of the product are recorded in order to maintain a link between the patient and the batch of the product.
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Renal and urinary disorders: Nephrotic syndrome has been reported very rarely following attempted immune tolerance induction in haemophilia .B patients with factor IX inhibitors and a history of allergic reaction
Vascular disorders: There is a potential risk of thromboembolic episodes following the administration of factor IX products, with a higher risk for low purity preparations. The use of low purity factor IX products has been associated with instances. of myocardial infarction, disseminated intravascular coagulation, venous thrombosis and pulmonary embolism .The use of high purity factor IX is rarely associated with such side effects
General disorders and administration site conditions: Fever has been observed rarely
Immune system disorders: Hypersensitivity or allergic reactions (which may include angioedema, stinging, burning (irritation), or phlebitis at ,the injection/infusion site, chills, flushing, generalised urticaria, headache, hives, hypotension, lethargy, nausea restlessness, tachycardia, tightness of the chest, tingling, vomiting, wheezing) have been observed rarely in patients,treated with factor IX containing products. In some cases, these reactions have progressed to severe anaphylaxis. From post-marketing experience it has been reported that patients with haemophilia B may very rarely develop neutralising antibodies (inhibitors) to factor IX. If such inhibitors occur, the condition will manifest itself as an insufficient clinical response. In such cases, it is recommended that a specialised haemophilia centre be contacted. In a clinical study 2 of 51 (4 %) previously untreated patients (PUPs) developed inhibitors, and in one .of these patients, this was associated with an anaphylactoid reaction in two occasions.
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No interactions of human coagulation factor IX products with other medicinal products are known Little data are available regarding the use of ε-aminocaproic acid following an initial infusion of Mononine 500 or Mononine 1000 for the prevention or treatment of oral bleeding following trauma or dental procedures such as .extractions.
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Animal reproduction studies have not been conducted with factor IX. Based on the rare occurrence of haemophilia .B in women, experience regarding the use of factor IX during pregnancy and breast-feeding is not available.

No symptoms of overdose with human coagulationn factor IX have been reported.