Congenital angioedema type I and II (HAE) - treatment and pre-treatment of acute conditions.
Composition:
1 vial contains 500 IU or 1500 IU C1-esterase inhibitor. After reconstitution, the medicine contains 50 IU / ml or 500 IU / ml C1-esterase inhibitor. The medicine contains sodium: 486 mg / 100 ml.
Action:
The C1-esterase inhibitor belongs to the human plasma serine protease inhibitor (serpin) and acts as other proteins of this group, such as: antithrombin III, α-2-antiplasmin, α-1 antitrypsin and others. Under physiological conditions, the C1-esterase inhibitor blocks the classical pathway of the complement system by inactivating the enzymatically active components C1s and C1r. The active enzyme forms a complex with an inhibitor in a stoichiometric ratio of 1: 1. In addition, the C1-esterase inhibitor is the most important inhibitor of coagulation factor activators by inhibiting factor XIIa and fragments thereof. In addition to α-2-macroglobulin, it is a major inhibitor of plasma kallikrein. The therapeutic effect in congenital angioedema is based on the substitution of the lacking C1-esterase inhibitor activity. The drug immediately achieves a dose-dependent concentration in plasma. The maximum concentration of C1-esterase inhibitor occurs within 0.8 h after administration (without significant differences between individual groups of patients). Medium T0,5 is 36.1 hours and is slightly shorter in children compared to adults (32.9 vs. 36.1 hours) and in patients with severe attacks compared to patients with mild attacks (30.9 vs. 37.0) h).
Contraindications:
Hypersensitivity to the active substance or to any of the excipients.
Precautions:
Patients with a proven allergy prophylaxis should be treated with antihistamines and corticosteroids prophylactically. If allergic symptoms or anaphylactic reaction occur, immediately discontinue treatment and start treatment, depending on the type and severity of side effects. Anti-shock treatment should be included in line with current standards. Patients with laryngeal edema require special observation to secure immediate intensive treatment in the hospital. Use not in accordance with registered indications or in the treatment of capillary leak syndrome is not recommended. Despite the use of inactivation methods of infectious agents, the transfer of known and unknown pathogens can not be completely ruled out. The precautionary measures taken are considered effective against enveloped viruses such as HIV, HBV and HCV and HAV and parvovirus B 19 non-enveloped viruses. Patients who regularly or repeatedly receive products derived from human plasma should be additionally considered for vaccination against hepatitis A and B. The sodium content of the preparation - up to 486 mg of sodium (about 21 mmol) / 100 ml should be taken in patients who are in control of sodium intake in the diet.
Pregnancy and lactation:
Pregnant should only be used if there are strict indications. The degree of penetration of the drug into breast milk is unknown, however, considering the high molecular weight, the probability of excretion with breast milk is very low. Breast-feeding in women with congenital angioedema is not recommended. Taking into account the advantages of breastfeeding for the child and the benefits of therapy for the mother, it should be considered whether to recommend discontinuation of breastfeeding or discontinue treatment with the product.
Side effects:
Rare: increased temperature and / or injection site reactions, allergic or anaphylactic reactions (eg tachycardia, hyper- or hypotension, redness, urticaria, dyspnoea, headache and dizziness, nausea). Very rare: shock. It is possible to transfer infectious agents. In attempts to treat high doses in prophylaxis and therapy of capillary leak syndrome, before, during or after cardiac surgery using extracorporeal circulation (indication and unregistered dose), rare (thrombosis) development of thrombosis was observed, in individual cases fatal.
Dosage:
Intravenously.Adults. Treatment of acute angioedema attack: 20 IU / kg; Pre-operative prevention of angioedema: 1000 IU less than 6 hours before a medical, dental or surgical procedure.Children and youth. Treatment of acute angioedema attack: 20 IU / kg; Pre-operative prevention of angioedema: 15-30 IU / kg less than 6 hours before a medical, dental or surgical procedure. The dose should be selected taking into account clinical circumstances (eg type of surgery and severity of the disease).Way of giving. The drug must be reconstituted. Reconstituted solution is given slowly by intravenous injection, Berinert 500 can be infused (4 ml / min). There are limited data on home remedies or self-administration. The decision regarding the use of home therapy for an individual patient should be made by the attending physician, who should ensure proper training of the patient and verification of home therapy at intervals.