<abstract xmlns="http://www.w3.org/1999/xhtml"><p> Hereditary angioedema (HAE) is a rare autosomal dominant disease consisting of recurrent angioedema attacks, varying in severity, possibly life-threatening and with frequent involvement of the head and neck areas. The patophysiology of HAE differs from histamine-mediated allergic angioedema. Three types of reduced quality or quantity in various complement or coagulation factors, leading to massive release of bradykinin, increase vascular permeability and produce capilary leakage. Clinical manifestations of HAE include swelling located predominantly in the head and neck area, hands, feet and urogenital area and abdominal pain caused by edema of the gastrointestinal tract mucosa. Diagnosis requires laboratory tests for complement components and genetic tests. A timely and correct diagnosis in the emergency room is of utmost importance, the medical treatment of HAE being substantially different from that of allergic angioedema. Although new therapies are available and in development, airway intervention and surgery are still life-saving procedures and the ENT surgeon is an important part of the multidisciplinary team managing an HAE attack.</p></abstract>

Hereditary angioedema (HAE) is a rare autosomal dominant disease consisting of recurrent angioedema attacks, varying in severity, possibly life-threatening and with frequent involvement of the head and neck areas. The patophysiology of HAE differs from histamine-mediated allergic angioedema. Three types of reduced quality or quantity in various complement or coagulation factors, leading to massive release of bradykinin, increase vascular permeability and produce capilary leakage. Clinical manifestations of HAE include swelling located predominantly in the head and neck area, hands, feet and urogenital area and abdominal pain caused by edema of the gastrointestinal tract mucosa. Diagnosis requires laboratory tests for complement components and genetic tests. A timely and correct diagnosis in the emergency room is of utmost importance, the medical treatment of HAE being substantially different from that of allergic angioedema. Although new therapies are available and in development, airway intervention and surgery are still life-saving procedures and the ENT surgeon is an important part of the multidisciplinary team managing an HAE attack.

Hereditary angioedema - an otolaryngologist’s perspective

ENT&HNS Department, “Sfanta Maria” Hospital, 37-39 Ion Mihalache Blvd., District 1, Bucharest, Romania

“Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania

Romanian Journal of Rhinology

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

{"article-title":"Hereditary angioedema - an otolaryngologist\u2019s perspective"}

Hereditary angioedema (HAE) is a rare autosomal dominant disease consisting of recurrent angioedema attacks, varying in severity, possibly life-threatening and...

Hereditary angioedema - an otolaryngologist’s perspective

Online veröffentlicht: 04. Aug. 2016

Seitenbereich: 133 - 138

DOI: https://doi.org/10.1515/rjr-2016-0015

Schlüsselwörter
angioedema, hereditary angioedema, surgical airway, tracheostomy

© 2016

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Hereditary angioedema - an otolaryngologist’s perspective

Online veröffentlicht: 04. Aug. 2016

Seitenbereich: 133 - 138

DOI: https://doi.org/10.1515/rjr-2016-0015

Schlüsselwörterangioedema, hereditary angioedema, surgical airway, tracheostomy

© 2016

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Schlüsselwörter
angioedema, hereditary angioedema, surgical airway, tracheostomy