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Laryngeal Edema

  • Alla Volokha
Chapter

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

Keywords

Hereditary angioedema Icatibant C1 esterase inhibitor Bradykinin 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Alla Volokha
    • 1
    • 2
  1. 1.Shupyk National Medical Academy for Postgraduate Education, Department of Pediatric Infectious Diseases and Pediatric ImmunologyKievUkraine
  2. 2.Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN)KievUkraine

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