Hereditary Angioedema Questions & Answers

Updated: Aug 30, 2018
  • Author: Michael M Frank, MD; Chief Editor: Michael A Kaliner, MD  more...
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Questions & Answers

Overview

What is hereditary angioedema (HAE)?

What are the signs and symptoms of hereditary angioedema (HAE)?

How is hereditary angioedema (HAE) diagnosed?

What is the role of imaging in the evaluation of hereditary angioedema (HAE)?

Which medications are used in the treatment of hereditary angioedema (HAE)?

What is included in prophylaxis for hereditary angioedema (HAE)?

What is hereditary angioedema (HAE)?

What are the types of hereditary angioedema (HAE)?

What is the mortality rate for hereditary angioedema (HAE)?

What is included in the treatment of hereditary angioedema (HAE)?

What is the pathophysiology of hereditary angioedema (HAE)?

What role of bradykinin in the pathogenesis of hereditary angioedema (HAE)?

What is the role of genetics in the pathogenesis of hereditary angioedema (HAE)?

Which genetic mutations cause type I hereditary angioedema (HAE)?

Which genetic mutations cause type II hereditary angioedema (HAE)?

What is the pathogenesis of hereditary angioedema (HAE) with normal C1 inhibitor?

What is the prevalence of hereditary angioedema (HAE)?

What are the racial and sexual predilections of hereditary angioedema (HAE)?

How does the incidence of hereditary angioedema (HAE) vary by age?

What is the prognosis of hereditary angioedema (HAE)?

What should be included in patient education about hereditary angioedema (HAE)?

Presentation

Which medical history is characteristic of hereditary angioedema (HAE)?

What are possible triggering events in hereditary angioedema (HAE)?

How frequently do attacks of hereditary angioedema (HAE) occur?

How does site of hereditary angioedema (HAE) affect the presentation?

How is swelling characterized in hereditary angioedema (HAE)?

What is the most common symptom of hereditary angioedema (HAE)?

What are the signs and symptoms of hereditary angioedema (HAE)?

What is the most serious complication of hereditary angioedema (HAE)?

What are less common presentation of hereditary angioedema (HAE)?

Which physical findings are characteristic of hereditary angioedema (HAE)?

What are less common physical findings suggestive of hereditary angioedema (HAE)?

DDX

What is acquired angioedema (AAE) and how is it differentiated from hereditary angioedema (HAE)?

Which conditions should be included in the differential diagnoses of hereditary angioedema (HAE)?

What are the differential diagnoses for Hereditary Angioedema?

Workup

What is the role of lab testing in the diagnosis of hereditary angioedema (HAE)?

Which tests are performed to differentiate the types of hereditary angioedema (HAE)?

What is the role of imaging studies in the diagnosis of hereditary angioedema (HAE)?

Which histologic findings suggest hereditary angioedema (HAE)?

Treatment

What is included in the treatment of hereditary angioedema (HAE)?

What are the World Allergy Organization (WAO) guidelines for prophylaxis and screening in hereditary angioedema (HAE)?

What are the treatment options for hereditary angioedema (HAE) types I and II?

How is pregnancy managed in patients with hereditary angioedema (HAE)?

What are the World Allergy Organization (WAO) guidelines for managing hereditary angioedema (HAE) types I and II?

Which medications are used in the treatment of acute hereditary angioedema (HAE)?

What is the role of ecallantide in the treatment of hereditary angioedema (HAE)?

What is the role of icatibant (Firazyr) in the treatment of hereditary angioedema (HAE)?

What is the role of prophylaxis in the treatment of hereditary angioedema (HAE)?

Which medications are used in the prophylactic treatment of hereditary angioedema (HAE)?

How are the underlying causes of hereditary angioedema (HAE) managed?

What is the role protease inhibitors in the treatment of hereditary angioedema (HAE)?

What is the role of diet and activity modifications in the treatment of hereditary angioedema (HAE)?

Which specialist consultations are needed for the treatment of hereditary angioedema (HAE)?

What long-term monitoring is required for hereditary angioedema (HAE)?

Medications

What are the goals of drug treatment for hereditary angioedema (HAE)?

What is the role of C1-INH in the treatment of hereditary angioedema (HAE)?

What is the role of recombinant C1 esterase inhibitor (Ruconest) in the treatment of hereditary angioedema (HAE)?

Which medications are used in the treatment of acute hereditary angioedema (HAE)?

Which medications in the drug class Antifibrinolytic Agents are used in the treatment of Hereditary Angioedema?

Which medications in the drug class Bradykinin Receptor Antagonists are used in the treatment of Hereditary Angioedema?

Which medications in the drug class Kallikrein Inhibitors are used in the treatment of Hereditary Angioedema?

Which medications in the drug class C1-Inhibitor Concentrates are used in the treatment of Hereditary Angioedema?

Which medications in the drug class Androgens and Androgen Derivatives are used in the treatment of Hereditary Angioedema?

Which medications in the drug class Blood Products are used in the treatment of Hereditary Angioedema?