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Hereditary Angioedema.

R Gentry Wilkerson1, Joseph J Moellman2

  • 1Department of Emergency Medicine, University of Maryland School of Medicine, 110 South Paca Street, 6th Floor, Suite 200, Baltimore, MD 21201, USA.

Emergency Medicine Clinics of North America
|November 16, 2021
PubMed
Summary

Hereditary angioedema (HAE) is a rare genetic disorder causing swelling attacks. Prompt airway assessment and understanding HAE pathophysiology are crucial for emergency physicians managing acute HAE episodes.

Keywords:
BradykininC1-inhibitorComplementDifficult airwayHereditary angioedemaQuincke disease

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Area of Science:

  • Genetics
  • Immunology
  • Emergency Medicine

Background:

  • Hereditary angioedema (HAE) is a rare autosomal dominant disorder.
  • It stems from reduced functional C1-INH levels, leading to swelling attacks.
  • Swelling affects subcutaneous and submucosal tissues, including respiratory and gastrointestinal tracts.

Purpose of the Study:

  • To outline the role of emergency physicians in managing hereditary angioedema.
  • To emphasize the importance of airway assessment in acute HAE attacks.
  • To guide management decisions based on HAE pathophysiology.

Main Methods:

  • Review of HAE pathophysiology and clinical manifestations.
  • Discussion of diagnostic limitations during acute attacks.
  • Emphasis on immediate airway assessment and intervention strategies.

Main Results:

  • Laboratory and radiographic studies have limited utility in acute HAE evaluation.
  • Airway assessment is the critical first step in managing acute HAE.
  • Understanding HAE pathophysiology informs critical management decisions.

Conclusions:

  • Emergency physicians must recognize HAE and prioritize airway safety.
  • Management hinges on prompt assessment and knowledge of HAE's underlying mechanisms.
  • Early intervention based on pathophysiology can improve patient outcomes.