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An ABC of the Warning Signs of Hereditary Angioedema.
- Source :
-
International archives of allergy and immunology [Int Arch Allergy Immunol] 2017; Vol. 174 (1), pp. 1-6. Date of Electronic Publication: 2017 Sep 27. - Publication Year :
- 2017
-
Abstract
- Hereditary angioedema (HAE) with C1 inhibitor deficiency is a genetic disorder that clinically manifests with attacks of angioedema in the subcutaneous and submucosal tissues, mainly in the extremities, abdomen, and upper airway. During attacks, vascular permeability is increased due to increased bradykinin (BK). This means that special therapies are needed for attacks that do not respond to traditional antiallergic therapies involving antihistamines, corticosteroids, and epinephrine. The recurring attacks may disable patients and lead to frequent visits to emergency rooms where misdiagnoses are common. HAE attacks may be fatal when upper-airway edema occurs, if proper treatment with a C1 inhibitor concentrate or BK receptor antagonist is not administered or an emergency tracheostomy is not performed. We propose a mnemonic method for the warning signs of HAE for the use as a diagnostic tool, i.e., the so-called "ABC" of the warning signs of HAE. The letters represent the following: A = Angioedema, B = Bradykinin, C = C1 inhibitor, D = Distress factors, E = Epinephrine nonresponsive, F = Family history, and G = Glottis/Gastrointestinal edema. To avoid fatalities, medical staff and patients, including family members, must be aware of HAE. An alphabetical mnemonic method has been developed and we hope it may benefit patients.<br /> (© 2017 S. Karger AG, Basel.)
- Subjects :
- Angioedemas, Hereditary pathology
Bradykinin metabolism
Bradykinin therapeutic use
Capillary Permeability genetics
Capillary Permeability physiology
Complement C1 Inactivator Proteins genetics
Complement C1 Inhibitor Protein
Genetic Predisposition to Disease genetics
Humans
Receptors, Bradykinin metabolism
Angioedemas, Hereditary diagnosis
Angioedemas, Hereditary drug therapy
Bradykinin analogs & derivatives
Bradykinin Receptor Antagonists therapeutic use
Complement C1 Inactivator Proteins deficiency
Subjects
Details
- Language :
- English
- ISSN :
- 1423-0097
- Volume :
- 174
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- International archives of allergy and immunology
- Publication Type :
- Academic Journal
- Accession number :
- 28950264
- Full Text :
- https://doi.org/10.1159/000479839