Plasma-derived C1-INH, recombinant C1-INH (for children above 12 years) and icatibant
(for children above 2 years) are licensed for the acute treatment of pediatric patients.
Twelve of 40 patients had already received an on-demand treatment after diagnosis such as icatibant
, pdC1-INH, or fresh frozen plasma; 9 of these 12 patients had a family member who gave them access to drugs to treat attacks (Figure 2).
Randomized placebo-controlled trial of the bradykinin B2 receptor antagonist icatibant
for the treatment of acute attacks of hereditary angioedema: The FAST-3 trial.
Specific treatment with C1-INH concentrate, icatibant
, or fresh frozen plasma (FFP) was never received in the ED.
Stelter et al., "A randomized trial of icatibant
in ace-inhibitor-induced angioedema," New England Journal of Medicine, vol.
Abbreviations: ACE:, angiotensin-converting enzyme; ACTZ:, acetazolamide; ANOVA:, analysis of variance; BW:, body weight; [degrees]C:, degree Celsius; CID:, collision-induced dissociation; CG-MS:, chromatography-mass spectrometry; EDTA:, ethylene-diaminetetraacetic acid; El:, excretion load; ELISA:, enzyme linked immunosorbent assay; eV:, electron-volts; ES-EG:, ethanol soluble fraction obtained from Echinodorus grandiflorus: ETOH:, ethanol; FWHM:, full width at half maximum; HOE-140:, icatibant
acetate; HR:, heart rate; HCTZ:, hydrochlorothiazide; HUEM:, herbarium of the Universidade Estadual de Maringa; INDO:, indometacin; LC-UV-MS:,
Treatment of ACEI-related angioedema with icatibant
: a case series.
and ecallantide are targeted at the issue of bradykinin accumulation as the cause of angioedema.
An emergency treatment with the plasma-derived or recombinant C1-INH administered by intravenous infusion at a dose of 20 U/kg can induce an unusually dramatic regression of all symptoms in 30 to 60 minutes.8 Kallikrein inhibitor ecallantide (Kalbitor) and a bradykinin B2 receptor antagonist icatibant
(Firazyr) are also approved for the treatment of acute episodes in recent years.
This C1 esterase inhibitor complements Shire's FIRAZYR icatibant
injection for the treatment of acute HAE attacks.
In contrast, the animal data for icatibant
(Firazyr; C), the other hematologic agent that is indicated for the treatment of acute attacks of hereditary angioedema, suggest risk.