Stephen J. Schueler, M.D.

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Angioedema Hereditary Overview

Another name for Angioedema Hereditary is Hereditary Angioedema.

What is hereditary angioedema?
A person with hereditary angioedema has a rare, inherited illness that causes episodes of sudden swelling of the hands, feet, face, intestines, throat and air passageways. Severe attacks of hereditary angioedema can be life threatening, but most episodes of swelling resolve. The cause of hereditary angioedema is an abnormal gene that results in low levels of C1 inhibitor protein. C1 inhibitor protein prevents episodes of inflammation in the body. When levels of the protein are low, inflammation occurs much more easily in all tissues of the body.

What are the symptoms of hereditary angioedema?
Symptoms of hereditary angioedema include facial swelling, eye swelling, mouth swelling, or tongue swelling. Additional symptoms may include nausea, vomiting, difficulty breathing, difficulty swallowing, hoarse voice, wheezing, and cough.

How does the doctor treat hereditary angioedema?
Treatment for hereditary angioedema includes strict avoidance of the substance known to trigger swelling. Medications for hereditary angioedema include C1 inhibitor, epinephrine injections, antihistamines, corticosteroids, and leukotriene antagonist medications. Some cases of hereditary angioedema may be treated with immunotherapy.

Continue to Angioedema Hereditary Symptoms

Last Updated: Aug 19, 2010 References
Authors: Stephen J. Schueler, MD; John H. Beckett, MD; D. Scott Gettings, MD
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PubMed Angioedema Hereditary References
  1. Davis AE 3rd. The pathophysiology of hereditary angioedema. Clin Immunol. 2005 Jan;114(1):3-9. [1559640]
  2. Nzeako UC, Frigas E, Tremaine WJ. Hereditary angioedema: a broad review for clinicians. Arch Intern Med. 2001 Nov 12;161(20):2417-29. [11700154]
  3. Zuraw BL. Current and future therapy for hereditary angioedema. Clin Immunol. 2005 Jan;114(1):10-6. [15596404]
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