Abstract
Hereditary angioedema (HAE) is characterized by recurrent episodes of angioedema, without urticaria or pruritus
HAE often affects the skin and mucosal tissues of the upper respiratory and gastrointestinal tracts
Laryngeal attack is a common life-threatening complication and may cause fatal asphyxiation
Worldwide incidence of HAE estimated from 1:10,000 to 1:50,000
HAE has an autosomal dominant inheritance, while up to 20–25% patients may have new spontaneous mutations
Decrease activity or absence of C1-INH results leads to excessive bradykinin production during angioedema attacks
C4 and C1-INH level and function, are the key laboratory values in the diagnosis of hereditary angioedema
Patients with hereditary angioedema have poor response to treatment with antihistamines and steroids
First-line therapies for HAE are human plasma-derived C1 inhibitor concentrate or recombinant human C1 inhibitor, which are available only in the European Union, or Icatibant, a bradykinin B2 receptor antagonist
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Volokha, A. (2019). Laryngeal Edema. In: Rezaei, N. (eds) Pediatric Immunology. Springer, Cham. https://doi.org/10.1007/978-3-030-21262-9_120
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DOI: https://doi.org/10.1007/978-3-030-21262-9_120
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