Angioedema, hereditary type I and II (AEH).
Treatment and prevention before acute relapses.

Treatment should be initiated under the supervision of a physician experienced in the treatment of the deficiency of the C1 inhibitor esterase.
Dosage

Adults

Treatment of seizures, acute angioedema

20 IU per kilogram of body weight (20 IU / kg bw).
Prevention of angioedema attacks before a procedure

1000 IU less than 6 hours before a medical, dental or surgical intervention

Population, pediatric

Treatment of seizures, acute angioedema

20 IU per kilogram of body weight (20 IU / kg bw).
Prevention of angioedema attacks before an intervention

15 to 30 IU per kilogram body weight (15 to 30 IU / kg bw) less than 6 hours before a medical, dental or surgical intervention. The dose should be selected taking into account the clinical context (eg the type of intervention and the severity of the disease)

Mode of administration

Berinert must be reconstituted under the heading Instructions for Use, Handling and Disposal. The reconstituted solution should be administered intravenously or as a slow infusion (4 ml / min).
Powder (white lyophilisate).

Pharmacotherapeutic group: C1 inhibitor, derived from plasma,

ATC code: B06AC01

The C1 esterase inhibitor is a plasma glycoprotein with a molecular weight of 105 kD and a 40% carbohydrate portion. Its concentration in human plasma is about 240 mg / l. Human plasma, placenta, hepatic cells, monocytes and platelets also contain the inhibitor of C1 esterase.

The inhibitor of C1 esterase belongs to the serine (serpin) protease inhibitor system of human plasma, as well as other proteins, antithrombin III, alpha-2 antiplasmin, alpha-1-antitrypsin, Other

Under physiological conditions, the inhibitor of C1, esterase inhibits the classical pathway of the complement system by inactivating the enzymatic components C1s and C1r. The active enzyme forms a 1: 1 stoichiometric complex with the inhibitor.

In addition, the C1-esterase inhibitor represents the most important inhibitor of coagulation contact activation by inhibiting factor XIIa and its fragments. In addition, it is used with macroglobulin, alpha-2 main inhibitor of plasma kallikrein.

The therapeutic effect of Berinert in hereditary angioedema is induced by the substitution of the deficient activity of the C1-esterase inhibitor.
No studies on the ability to drive or use machines have been carried out.
Allergy to any of the components of the medication

For patients with a known allergic history, antihistamines and corticosteroids should be administered as prophylaxis

In the event of an allergic or anaphylactic reaction, immediate administration of BERINERT (eg, injection or infusion) should be discontinued and treatment should be initiated. Therapeutic measures depend on gender and severity, adverse effects. Standard shock treatments must be applied.

Patients with laryngeal edema require monitoring, particularly intensive in a department with emergency treatment.

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BERINERT contains up to 486 mg of sodium (approximately 21 mmol) per 100 ml of solution. This should be taken into account for patients following a hyposodic diet.
Home treatment and self-administration

Data on the use of this drug at home or in self-administration are limited. The potential risks associated with home treatment are related to the administration itself and to the treatment of adverse effects, particularly hypersensitivity. The decision to treat a patient at home must be made by the attending physician, who must ensure the proper training of the patient and the revision of the use at regular intervals.
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Security, viral

The usual measures of prevention of the risk of transmission of infectious agents by drugs prepared from blood or human plasma include clinical selection, donors, search for specific markers, infection on each donation and Plasma mixtures, and the use in the manufacturing process of effective steps for viral inactivation / elimination. However, when drugs prepared from blood or human plasma are administered, the risk of transmission of infectious agents can not be totally excluded. This also applies to unknown or emerging viruses or other types of infectious agents.

The measures taken are considered to be effective against enveloped viruses such as HIV, HBV and HCV, and against unwrapped viruses such as HAV and Parvovirus B19.

Appropriate vaccination (hepatitis A and B) of patients receiving coagulation factors is recommended.

None, study of interaction has been carried out

Berinert should not be mixed with other medicines and diluents in the syringe / infusion set.
No cases of overdose have been reported.
Pregnancy

Little data relating to the increased risk resulting from the use of Berinert in pregnant women are available. Berinert is a physiological component of human plasma. For this reason, no reproductive and developmental toxicity studies have been carried out, and in animals and no adverse effects on fertility, pre- and postnatal development, man. Accordingly, Berinert should only be used during pregnancy in cases of absolute necessity.


There is no data on the passage of Berinert into breast milk, but because of its high molecular weight, this seems unlikely. However, breastfeeding remains uncertain in women suffering from hereditary angioedema. A decision must be made as to whether to discontinue breast-feeding or discontinue Berinert's treatment, taking into account the benefit of breast-feeding, and for the child and the treatment for the woman. >

The following adverse reactions are based on the post-marketing experience as well as on the scientific literature. The following standard frequency categories are used

Very common> 1/10

Common:> 1/100 and 1/1000 and 1/10 000 and