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C1 Human Esterase Inhibitor
Aka: C1 Human Esterase Inhibitor, C1 Antagonist, C1 Inhibitor, Berinert, Plasma-Derived C1 Esterase Inhibitor
- See Also
- Angioedema
- Bradykinin Receptor Antagonist
- Indications
- Severe, airway compromising Angioedema
- Hereditary Angioedema (prophylaxis and treatment of choice)
- Not found effective in ACE InhibitorAngioedema
- Background
- Human plasma-derived C1-INH concentrate
- Pasteurized and nanofiltered plasma sample
- In Hereditary Angioedema, C1 esterase inhibitor is deficient or non-functional
- C1 Esterase Inhibitor suppresses the Classical Complement Pathway (C1 to C3 activation)
- C1 Esterase inhibitor also suppresses blood Coagulation Factors (Factor XII and plasma Kallikrein)
- Deficiency results in increased bradykinin (vasoactivity) and increased tissue edema
- Dose: Hereditary Angioedema Prophylaxis
- Adults and Teens: 60 IU/kg SQ twice weekly (every 3-4 days)
- Reduces episodes of Angioedema by 95%
- Dose: Acute Management
- Berinert 20 IU/kg IV for 1 dose
- Cost: $3000 per dose
- Adverse Effects
- Headache (common)
- Nausea (common)
- Dizziness
- Dysgeusia
- Fever
- Local skin reaction and Rash
- Flu-like symptoms
- Hypersensitivity Reaction (uncommon)
- Thrombotic event, especially at doses higher than recommended (uncommon)
- References
- (2015) Lexicomp, accessed online 4/9/2015
- Lovecchio (2021) Crit Dec Emerg Med 31(10): 24
- Farkas (2007) J Allergy Clin Immunol 120(4):941-7 +PMID:17761272 [PubMed]