Angioedema of the head and neck or other internal organs is a medical emergency and must be treated immediately. For laryngeal edema, the indication for intensive care must be aggressive, as intubation or even coniotomy may become necessary. Cutaneous angioedema should be treated if it is painful, functionally impairing or progressive. The treatment of acute angioedema caused by a C1-INH defect is:

1) C1-INH concentrate (Berinert P):

Dose: Adults and children, 20 units per kg of body weight.
Application: Carefully dissolve 500 units in 10 ml of the included solvent and slowly administer intravenously.

Recommendations for preparing Berinert P

Berinert® P consists of two components: a vial containing protein concentrate (500 U human C1 esterase inhibitor) and a vial of solvent (10 ml of water for injection). The pack additionally contains all the medical devices which are required for preparing the solution for injection and for intravenous administration of Berinert® P. The storage temperature of the preparation must not exceed 25°C.
If the preparation has been stored in a refrigerator, both components should be adjusted to room temperature prior to preparation. The solution for injection is prepared using the enclosed needleless Mix2VialTM filter transfer set (follow instructions for use). Once the solvent has been transferred into the product vial, the protein quickly dissolves. To avoid foaming, do not shake the product vial. The protein solution should be clear to slightly opalescent and must not contain any clearly visible particles. Any waste should be properly disposed of after use.


2) Icatibant (Firazyr)

Dose: 30 mg Icatibant in 3 ml solution as ready-to-use syringe
Application: Inject s.c.

Antihistamines, corticosteroids, danazol or tranexamic acid are not effective or ineffective for an acute attack.