II. Indications
- Severe, airway compromising Angioedema
- Hereditary Angioedema (prophylaxis and treatment of choice)
- Not found effective in ACE InhibitorAngioedema
III. 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
IV. Dose: Hereditary Angioedema Prophylaxis
- Adults and Teens: 60 IU/kg SQ twice weekly (every 3-4 days)
- Reduces episodes of Angioedema by 95%
V. Dose: Acute Management
- Berinert 20 IU/kg IV for 1 dose
- Cost: $3000 per dose
VI. Adverse Effects
VII. 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]
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Related Studies
| Concepts | Amino Acid, Peptide, or Protein (T116) , Pharmacologic Substance (T121) |
| MSH | C469952 |
| English | C1 INHIBITOR (HUMAN), C1 INHIBITOR, C1 inhibitor (Human), HUMAN C1-ESTERASE INHIBITOR, C1-Esterase Inhibitor, Human, Plasma Protease C1 Inhibitor, C1-Inhibiting Factor, Human C1-Esterase Inhibitor, C1 Inhibitor (Human), C1s inhibitor, human, C1s-INH protein, human, complement C1s inhibitor, human |
Ontology: Berinert (C2723788)
| Concepts | Amino Acid, Peptide, or Protein (T116) , Pharmacologic Substance (T121) |
| English | Berinert |
