Medicinal Products

BETAFACT 100 IU / mL

Generic drug of the therapeutic class: Haemostasis and blood
active ingredients: Factor IX
laboratory: Lfb-Biomedicaments

Powder and solvent for solution for injection
Box of 1 Bottle of powder + 5 ml solvent bottle
All forms

Indication

Human coagulation factor IX is indicated for the treatment and prevention of hemorrhage in patients with hemophilia B (congenital factor IX deficiency).

Dosage BETAFACT 100 IU / mL Powder and solvent for solution for injection Box of 1 vial of powder + vial of solvent of 5 ml

Dosage

Treatment should be initiated under the supervision of a hemophilia specialist.

The dosage and duration of the substitution treatment depend on the severity of the factor IX deficiency, the location and intensity of the bleeding event, and the clinical condition of the patient.

The number of units of factor IX administered is expressed in International Units (IU), calculated against the WHO standard for factor IX. The plasma activity of factor IX is expressed either as a percentage (relative to normal human plasma) or as International Units (relative to the international standard for plasma factor IX).

One IU of human coagulation factor IX corresponds to the amount of factor IX contained in one ml of normal human plasma.

The calculation of the required dose is based on the observation that one IU of human coagulation factor IX per kg of body weight increases the normal factor IX plasma activity by 1.08%. This dose is determined using the following formula:

Number of units to be administered = body weight (kg) desired increase in factor IX (%) (IU / dl) 0.93

The dose and frequency of injections should always be adapted to each case based on clinical effectiveness. Human coagulation factor IX rarely requires more than one daily injection.

The plasma activity of factor IX must be maintained at rates and for a period depending on the bleeding events to be treated. The table below can be used to determine the dosage in the following hemorrhagic and surgical situations:

Intensity of bleeding

Type of surgical intervention

Factor IX plasma level to be achieved (%) (IU / dl)

Frequency of injections (hours)

Duration of treatment (days)

Haemorrhages:

Premature haemarthrosis, muscle bleeding or oral cavity

Extended haemarthrosis, muscle bleeding or hematoma

Life-threatening haemorrhages such as cerebral hemorrhage, bleeding from the neck, major abdominal bleeding

20 to 40

30 to 60

60 to 100

Repeat every 24 hours, for at least 1 day, until the pain or bleeding accident stops

Repeat the injection every 24 hours for 3 to 4 days or more until discomfort and pain disappear

Repeat the injection every 8 to 24 hours until the risk of bleeding disappears

Surgery:

Minor of which tooth extraction

major

30 to 60

80 to 100

(pre and postoperative)

Every 24 hours for at least 1 day until the bleeding stops

Repeat the injection every 8 to 24 hours until wound healing, then continue treatment for at least 7 additional days to maintain the factor IX level between 30% and 60% (IU / dl)

During treatment, it is recommended to control the level of factor IX to adjust the dosage and frequency of injections. In the case of major surgery in particular, substitution treatment requires special monitoring with control of coagulation (plasma activity of factor IX). The response to human coagulation factor IX treatment, including in vivo recovery rates and observed half-lives, may vary between individuals.

In long-term prophylaxis in patients with severe hemophilia B, doses of 20 to 40 IU of human coagulation factor IX per kg of body weight should be administered every 3 to
4 days. In some cases, especially in young people, it may be necessary to increase the dosage or to shorten the interval between two injections.

The appearance of a factor IX inhibitor should be monitored. If the desired plasma factor IX levels can not be achieved or the bleeding is not controlled after the appropriate dose, biological tests should be performed to detect the presence of an inhibitor.

If an inhibitor occurs at a level below 10 Bethesda Units (BU), the increased dose of injected human coagulation factor IX may be sufficient to neutralize the inhibitor. In patients with high titer inhibitors, factor IX therapy may be ineffective and other therapies should be considered. Such treatments should be conducted by physicians who are used to treating patients with hemophilia B.

In clinical trials, 11 children under 6 years of age were treated with BETAFACT at a dose appropriate to their body weight, identical to that of adults.

Method and route of administration

Reconstitute the preparation as described in 6.6 "Instructions for Use, Handling and Disposal". Inject intravenously.

It is recommended not to administer human coagulation factor IX at a rate greater than 4 ml / minute. BETAFACT must be injected intravenously, at once, immediately after reconstitution, without exceeding 400 IU / min (ie 4 ml / min).

Against indications

Hypersensitivity to the active substance or to any of the excipients, including heparin or its derivatives including LMWH.

History of Heparin-Induced Thrombocytopenia or Type II (HIT).

Betafact side effects

Hypersensitivity or allergic reactions (such as angioedema, burning and tingling at the injection site, chills, redness, generalized urticaria, headache, urticarial reactions, low blood pressure, lethargy, nausea, agitation, tachycardia, chest tightness, tingling), vomiting, asthmatic respiration) have been observed inconsistently in some patients treated with human coagulation factor IX preparations.

In some cases, these reactions have resulted in a severe anaphylactic reaction, associated with the simultaneous appearance of factor IX inhibitor (see Warnings and Precautions "Special warnings and special precautions for use" section). ). The required treatment depends on the nature and severity of the reaction.

The occurrence of nephrotic syndrome has been described following the induction of immune tolerance in hemophilia B patients with a factor IX inhibitor.

Rare cases of hyperthermia can be observed.

Patients with hemophilia B may develop anti-factor IX antibodies (inhibitors).

The appearance of such inhibitors results in an insufficient clinical response. In this case, it is recommended to contact a center specialized in the treatment of hemophilia.

In clinical studies in 11 previously untreated patients, no inhibitors were detected following the injection of BETAFACT.

Administration of factor IX includes a potential risk of thromboembolic complications, particularly with low purity preparations. The occurrence of myocardial infarction, disseminated intravascular coagulation, venous thrombosis and pulmonary embolism could be correlated with the use of low purity factor IX preparations. Administration of highly purified human coagulation factor IX, such as BETAFACT, is rarely associated with such adverse effects.

For safety regarding transmissible agents, see Warnings and precautions for use .

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