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BLOOD AND BLOOD FORMING ORGANS › OTHER HEMATOLOGICAL AGENTS › OTHER HEMATOLOGICAL AGENTS › Drugs used in hereditary angioedema
c1-inhibitor, plasma derived
B06AC01
Forms & Strengths
- Intravenous powder for reconstitution: 500 units, 1500 units
Adult Dosing
- Hereditary angioedema (HAE) acute attack: 20 units/kg IV
- HAE routine prevention: 1000 units IV every 3 to 4 days
Pediatric Dosing
- HAE acute attack (adolescents and children): 20 units/kg IV
- HAE routine prevention (adolescents and children): 1000 units IV every 3 to 4 days (or 20 units/kg based on clinical response)
Indications
- Treatment of acute angedema attacks in patients with hereditary angioedema (HAE)
- Routine prophylaxis against angioedema attacks in patients with HAE
Mechanism of Action
- Replaces endogenous C1 esterase inhibitor, which regulates the complement, contact, and fibrinolytic systems
- Inhibits C1r, C1s, kallikrein, and factor XIIa, preventing the excessive bradykinin generation responsible for HAE swelling
Contraindications
- History of life-threatening hypersensitivity reactions, including anaphylaxis, to C1 esterase inhibitor products
Adverse Reactions
- Headache
- Nausea
- Nasopharyngitis
- Dizziness
- Rash
- Diarrhea
- Infusion-site reactions
- Hypersensitivity including anaphylaxis
Drug Interactions
- No formal drug interaction studies have been conducted
- Avoid co-administration with tissue plasminogen activator (tPA) or other agents that increase bleeding risk or potentiate angioedema
Curated Content: Needs Vetting Before Put to Clinical Use