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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)