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The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update 1 Introduction 2 Methods 3 Selection of key questions, wording of recommendations 3 Literature search and review 3 Consensus procedures 4 Presentation of recommendations 4 Definitions, nomenclature, and classification 4 The pathophysiology of HAE 5 The pathophysiology of HAE-1 and HAE-2 5 The pathophysiology of HAE with normal C1 inhibitor 6 The diagnosis of HAE 6 The differential diagnosis of HAE 8 On-demand treatment of HAE attacks 9 On-demand treatment with C1 inhibitor 9 On-demand treatment with the kallikrein-inhibitor ecallantide 10 On-demand treatment with the bradykinin B2 receptor antagonist icatibant 10 Providing HAE patients with on-demand medication 11 Short-term prophylactic treatment of HAE 11 Long-term prophylactic treatment of HAE 12 Long-term prophylaxis with plasma-derived C1–INH 13 Long-term prophylaxis with lanadelumab 13 Long-term prophylaxis with berotralstat 14 Long-term prophylaxis with androgens 14 Long-term prophylaxis with antifibrinolytics 15 Monitoring of long-term prophylaxis 15 Management of HAE-1/2 in children 16 Course and clinical pictur
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