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Table 3 Treatments for HAE available in Canada that are supported by a high level of evidence [7, 16]

From: Angioedema

HAE-specific treatment

Product name

Mechanism of action

Approved indications in Canada

Dose and route of administration

Age indications

Plasma-derived C1-INH†

Berinert

Replaces C1-INH

Acute treatment

20 U/kg intravenous as needed

Pediatric and adult

Cinryze

Replaces C1-INH

Long-term prophylaxis

1000 U intravenous q 3–4 days

≥ 12 years

Haegarda

Replaces C1-INH

Long-term prophylaxis

60 U/kg body weight twice weekly subcutaneously

(every 3–4 days)

≥ 12 years

Icatibant

Firazyr

Synthetic selective and specific antagonist of bradykinin 2 receptor

Acute treatment

30 mg subcutaneous injection as needed; dose-adjusted for adolescents < 65 kg and children ≥ 2 years*

≥ 2 years

Lanadelumab

Takhzyro

Fully human monoclonal antibody that binds plasma kallikrein and inhibits its proteolytic activity

Long-term prophylaxis

300 mg subcutaneous injection every 2 weeks

A dosing interval of 300 mg every 4 weeks may be considered if the patient is well-controlled (e.g., attack free) for more than 6 months

≥ 12 years

Berotralstat

Orladeyo

Oral, selective inhibitor of plasma kallikrein that blocks enzymatic activity of plasma kallikrein in releasing bradykinin

Long-term prophylaxis

150 mg daily taken orally with food

≥ 12 years

  1. Treatment of choice during pregnancy, delivery and breastfeeding
  2. *12 kg to 25 kg: 10 mg (1.0 ml); 26 kg to 40 kg: 15 mg (1.5 ml); 41 kg to 50 kg: 20 mg (2.0 ml); 51 kg to 65 kg: 25 mg (2.5 ml); > 65 kg: 30 mg (3.0 ml)
  3. Adapted from: Betschel S, et al. Allergy Asthma Clin Immunol. 2019 [7]. Berotralstat product monograph 2022 [16]