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Hereditary Angioedema

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Treatment Î All patients with HAE should have access to an effective, on-demand HAE-specific agent. Evidence from double-blind, placebo-controlled randomized clinical trials demonstrates the efficacy and safety of treatment of HAE attacks with C1INH concentrates, a plasma kallikrein inhibitor, or a bradykinin B2 receptor antagonist (A) Î Management of HAE attacks may involve symptomatic treatment based on the region of body swelling. (C) Î Fresh frozen plasma is often effective in abrogating HAE attacks, but fresh frozen plasma may acutely exacerbate some attacks. For this reason caution is required. (D) Î Neither anabolic androgens nor antifibrinolytic drugs provide reliably effective treatment for acute attacks of angioedema. (D) Î Epinephrine, corticosteroids and antihistamines are NOT efficacious and are NOT recommended for treatment of HAE. (C) Figure 2. HAE Treatment Algorithm Acute attack? On-demand treatment Predictable upcoming stressor? YES NO Short-term prophylaxis Known HAE YES Is AE well controlled? Minimize exacerbating factors NO Start long-term prophylactic treatment NO YES Continue on-demand treatment Still not well controlled Abbreviations: AE, angioedema; HAE, hereditary angioedema

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