Table 3 Treatment summary of emergency care available in the US to patients experiencing acute attacks of hereditary angioedema [23, 32–34]
From: Emerging concepts in the diagnosis and treatment of patients with undifferentiated angioedema
Therapy and indication | Dosage | Monitoring tests |
|---|---|---|
C1 esterase inhibitor [human] (Berinert; CSL Behring) | 20 U/kg body weight IV at a rate of 4 ml/ minute | · Monitor patients with known risk factors for thrombotic events |
Indicated for the treatment of acute abdominal or facial attacks of HAE in adult and adolescent patients | ||
· Epinephrine should be immediately available to treat any acute severe hypersensitivity reactions following discontinuation of administration | ||
Plasma kallikrein inhibitor (Kalbitor [ecallantide]; Dyax Corp) | 30 mg (3 ml) SC in three 10-mg (1 ml) injections. If attack persists, additional dose of 30 mg (3 ml) may be administered within a 24-h period | · Given the similarity in hypersensitivity symptoms and acute HAE symptoms, monitor patients closely for hypersensitivity reactions |
Indicated for attacks at all anatomic sites | ||
· Administer in a setting equipped to manage anaphylaxis and HAE | ||
Fresh-frozen plasma | 2 U at 1 to 12 h before the event (only for use when C1-INH concentrate is not available) | · Baseline: liver function tests, hepatitis virology |
Bradykinin β2 receptor antagonist | 30 mg (3 ml) injected SC in the abdominal area. If attack persists, additional injections of 30 mg (3 ml) may be administered at intervals of ≥6 h. No more than 3 injections in 24 hours | For patients who never received Firazyr previously, the first treatment should be given in a medical institution or under the guidance of a physician |
(Firazyr [icatibant]; Shire Orphan Therapies) | ||
Indicated for attacks at all anatomic sites |