Pharmacotherapeutic group: C1-inhibitor, plasma derived
ATC code: B06AC01
C1-esterase inhibitor is a plasma glycoprotein with a molecular weight of 105 kD and a carbohydrate moiety of 40 %. Its concentration in human plasma ranges around 240 mg/l. Besides its occurrence in human plasma, also the placenta, the liver cells, monocytes and platelets contain C1-esterase inhibitor.
C1-esterase inhibitor belongs to the serine-protease-inhibitor-(serpin)-system of human plasma as do also other proteins like antithrombin III, alpha-2-antiplasmin, alpha-1-antitrypsin and others.
Under physiological conditions C1-esterase inhibitor blocks the classical pathway of the complement system by inactivating the enzymatic active components C1s and C1r. The active enzyme forms a complex with the inhibitor in a stoichiometry of 1:1.
Furthermore, C1-esterase inhibitor represents the most important inhibitor of the contact activation of coagulation by inhibiting factor XIIa and its fragments. In addition, it serves, besides alpha-2-macroglobulin, as the main inhibitor of plasma kallikrein.
The therapeutic effect of Berinert in hereditary angioedema is induced by the substitution of the deficient C1-esterase inhibitor activity.
⚠️ Warnings
Any unused medicinal product or waste material should be disposed of in accordance with local requirements.
Method of administration
General instructions
- The solution should be colourless and clear to slightly opalescent.
- After filtering/withdrawal (see below) reconstituted product should be inspected visually for particulate matter and discoloration prior to administration.
- Do not use solutions that are cloudy or have deposits.
- Reconstitution and withdrawal must be carried out under aseptic conditions. Use the syringe provided with the product.
Reconstitution
Bring the solvent to room temperature. Ensure product and solvent vial flip caps are removed and the stoppers are treated with an antiseptic solution and allowed to dry prior to opening the Mix2Vial package.
1. Open the Mix2Vial package by peeling off the lid. Do
not
remove the Mix2Vial from the blister package!
2. Place the solvent vial on an even, clean surface and hold the vial tight. Take the Mix2Vial together with the blister package and push the spike of the blue adapter end
straight down
through the solvent vial stopper.
3. Carefully remove the blister package from the Mix2Vial set by holding at the rim, and pulling
vertically
upwards. Make sure that you only pull away the blister package and not the Mix2Vial set.
4. Place the product vial on an even and firm surface. Invert the solvent vial with the Mix2Vial set attached and push the spike of the transparent adapter end
straight down
through the product vial stopper. The solvent will automatically flow into the product vial.
5. With one hand grasp the product-side of the Mix2Vial set, and with the other hand grasp the solvent-side and unscrew the set carefully into two pieces.
Discard the solvent vial with the blue Mix2Vial adapter attached.
6. Gently swirl the product vial with the transparent adapter attached until the substance is fully dissolved. Do not shake.
7. Draw air into an empty, sterile syringe. Use the syringe provided with the product. While the product vial is upright, connect the syringe to the Mix2Vial's Luer Lock fitting. Inject air into the product vial.
Withdrawal and application
8. While keeping the syringe plunger pressed, invert the system upside down and draw the solution into the syringe by pulling the plunger back slowly.
9. Now that the solution has been transferred into the syringe, firmly hold on to the barrel of the syringe (keeping the syringe plunger facing down) and disconnect the transparent Mix2Vial adapter from the syringe.
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