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Table 3 Therapies for HAE supported by high level of evidence

From: Canadian hereditary angioedema guideline

HAE specific treatment Product name and company Mechanism of Action Approved Indications In Canada Dose Adverse Events
C1-INH – Plasma Berinert® (CSL) Replaces CI-INH Acute treatment 20 IU/Kg intravenous Anaphylaxis/Thrombosis (rare);Transmission of infectious agents (theoretical)
  Cinryze® (Shire) Replaces CI-INH Long term Prophylaxis 1000 IU q3-4 days intravenous Anaphylaxis/Thrombosis (rare);Transmission of infectious agents (theoretical)
– Recombinant Rhucin® (Pharming) Replaces CI-INH Not licensed 50 U/Kg Intravenous Anaphylaxis (rare)
Ecallantide Kalbitor® (Dyax) Inhibits plasma kallikrein Not licensed 30 mg subcutaneous injection Anaphylaxis (uncommon)
Icatibant Firazyr® (Shire) Blocks bradykinin 2 receptor Acute treatment 30 mg subcutaneous injection Pain, swelling, pruritis at injection site (common)
      Exacerbation of coronary artery disease (theoretical)