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Table 1 On-demand and prophylaxis medications approved for HAE-C1-INH in the United States [9]

From: Considerations in the management of hereditary angioedema due to C1-INH deficiency in women of childbearing age

Brand name
(Generic)
Class Ages Dosage Route (self-administration) Adverse effects
On-demand therapy
 Berinert (C1-INH) [46] Plasma-derived C1-inhibitor Pediatric and adults of all ages 20 U/kg IV (yes) Allergic reaction, nausea, diarrhea
 Ruconest (C1-INH) [47] Recombinant C1-inhibitor Adolescents and adults 50 U/kg IV (yes) Headache, nausea, diarrhea
 Kalbitor (ecallantide) [25] Kallikrein inhibitor  ≥ 12 years 30 mg SQ (no) Possible anaphylaxis (uncommon)
 Firazyr (icatibant) [26] B2 bradykinin receptor antagonist  ≥ 18 years 30 mg SQ (yes) Redness, swelling, pain at the site of injection
Prophylaxis
 Danazol [39] Attenuated androgen  ≥ 18 years Variable, with maximum long-term recommended dosage of 200 mg/day Oral Weight gain, virilization, hirsutism, acne, voice changes (hoarseness/deepening), menstrual irregularities, pseudomenopause, vaginal burning, vaginal dryness or itching, hypercholesterolemia, hypertension, hepatotoxicity
 Cinryze (C1-INH) [48] Plasma-derived C1-inhibitor  ≥ 6 years 500 IU in children 6–11 years, 1000–2500 IU in adults, twice weekly IV (yes) Allergic reaction, nausea, diarrhea
 Haegarda (C1-INH) [49] Plasma-derived C1-inhibitor  ≥ 6 years 60 IU/kg twice weekly SQ (yes) Injection site reactions
 Takhzyro (lanadelumab-flyo) [41] Plasma kallikrein inhibitor  ≥ 12 years 300 mg every 2 weeks initially; may be given every 4 weeks based on clinical response SQ (yes) Injection site reactions, upper respiratory infections, headache
 Orladeyo (berotralstat) [42] Plasma kallikrein inhibitor  ≥ 12 years 150 mg daily Oral Abdominal pain, vomiting, diarrhea, back pain, gastroesophageal reflux