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Hereditary Angioedema

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Table 2. HAE-Specific Agents Drug Name (Brand) FDA Indications Dosage Mechanism Anticipated Potential Side Effects Plasma-derived nanofiltered C1INH (Cinryze ® ) Long-term prophylaxis 1000 units IV q3-4d Inhibits plasma kallikrein, coagulation factors XIIa and XIa, C1s, C1r, MASP-1, MASP-2, and plasmin Rare: risk of anaphylaxis eoretical: transmission of infectious agent Plasma-derived nanofiltered C1INH (Berinert ® P) Acute attacks 20 units per kg IV Inhibits plasma kallikrein, coagulation factors XIIa and XIa, C1s, C1r, MASP-1, MASP-2, and plasmin Rare: risk of anaphylaxis eoretical: transmission of infectious agent Ecallantide (Kalbitor ® ) Acute attacks 30 mg subcuta- neous (administered as 3 injections of 1 mL each) Inhibits plasma kallikrein Uncommon: anti-drug anti- bodies, risk of anaphylaxis Icatibant (Firazyr ® ) Acute attacks 30 mg subcuta- neous Bradykinin B2 receptor antagonist Common: injection site reactions Recombinant- human C1INH (Rhucin ® ) Acute attacks (pending ) 50-100 units per kg IV Inhibits plasma kallikrein, coagulation factors XIIa and XIa, C1s, C1r, MASP-1, MASP-2, and plasmin Uncommon: risk of anaphy- laxis in rabbit- sensitized individuals Adapted from Zuraw, BL. N Engl J Med. 2008;359(10):1027-36.

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