HAE

Question 1

name 4 categories treatment for HAE and type

Answer 1

1. Replace C1-INH:
   LT prophylaxis - Cinryze (1000 IU q 3-4 days IV)
   LT prophylaxis: Haegarda (60 IU/kg q 3-4d subcutaneous)
   Acute Treatment - Berinert (20 IU/Kg IV)
   Acute treatment - Ruconest (50IU/kg (under 84kg), 4200 IU max dose) IV (not in canada)
2. Block Bradykinin 2 Receptor
   Acute Treatment – Icatibant (Firazyr) 30mg SC injection Q6H (max 3 doses/24 hrs)
3. Anti-fibrinoloytics
   Long term Prophylaxis –Tranexamic Acid (Effective in some patients but not ideal in children and should not be used in acute attacks)
4. Inhibit Plasma Kallikrein
   Acute - Ecallantide (Kalbitor) 30mg SC injection - Not licensed in CA- can get through SAP)
   Prophylaxis - Takhzyro (lanadelumab) 300mg q2w initially subcutaneous

Question 2

name 3 things inhibited by C1inh

Answer 2

12a (so cant allow prekalikiren to kallikrein) and also kallikrein to convert HK to brady kiniin
and facto 12 to activate classical complement pathway