C1-Esterase Inhibitor (Human)
Class: Complement Inhibitors
Chemical Name: Human plasma protease C1 inhibitor (C1 esterase inhibitor) (N,O-glycosylated recombinant protein expressed in the mammary gland of transgenic rabbits), glycoform α
Molecular Formula: C2355H3745N613O728S17
CAS Number: 80295-38-1
Brands: Berinert, Cinryze
Introduction
Preparation of complement 1 (C1)-esterase inhibitor derived from pooled human plasma.
C1-esterase inhibitor is a naturally occurring inhibitor of certain serine proteases (e.g., C1 complement, kallikrein, coagulation factor XIIa, plasmin) involved in the complement, coagulation (contact), and fibrinolytic systems.
Uses for C1-Esterase Inhibitor (Human)
Hereditary Angioedema: Prevention of Angioedema Attacks
Routine prophylaxis against angioedema attacks in adults and adolescents with hereditary angioedema (HAE). Designated an orphan drug by FDA for this use.
One of several agents that may be considered for long-term prophylaxis of HAE attacks; other drugs include attenuated androgens (e.g., danazol) and antifibrinolytic agents (e.g., tranexamic acid).
Consider long-term prophylaxis in patients with HAE who have frequent or severe attacks of angioedema; individualize need for prophylactic therapy based on factors such as severity of disease, frequency of attacks, patient's quality of life, availability of resources, and patient response to on-demand therapy.
Hereditary Angioedema: Treatment of Acute Angioedema Attacks
Treatment of acute abdominal, facial, or laryngeal attacks of HAE in adults and adolescents. Designated an orphan drug by FDA for this use.
Recommended by US and international consensus guidelines as one of several options for treatment of acute HAE attacks; other drugs include ecallantide (plasma kallikrein inhibitor) and icatibant (bradykinin B2-receptor antagonist).
C1-Esterase Inhibitor (Human) Dosage and Administration
Administration
IV Administration
Administer by slow IV injection.
May be self-administered after appropriate training is provided. (See Advice to Patients.)
Do not mix with any other drug or solution. Administer via dedicated IV line.
Vials are for single use only; discard any unused portions.
Reconstitution of Cinryze
Allow vials of drug and diluent (sterile water for injection) to reach room temperature prior to reconstitution.
Reconstitute vial of Cinryzecontaining 500 units of C1-esterase inhibitor (human) with 5 mL of sterile water for injection using transfer set provided by manufacturer or another commercially available double-ended needle; vacuum will draw in the diluent. Do not use vials that lack a vacuum. Gently swirl until powder is completely dissolved. Resultant solution contains 100 units/mL and should be colorless to slightly blue, and free from visible particles. Reconstitute 2 vials to obtain a single 1000-unit dose.
Manufacturer recom...
