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Antibodies


Hereditary angioedema

Definition:

Hereditary angioedema is an inherited abnormality of the immune system that causes swelling, particularly of the face, and abdominal cramping. Also see angioedema.



Causes, incidence, and risk factors:

Hereditary angioedema is an inherited disorder. It is caused by low levels or improper function of a protein called C1 inhibitor. This, in turn, affects blood vessels. People with hereditary angioedema can develop rapid swelling of the hands, feet, limbs, face, intestinal tract, or airway (larynx or trachea).

Unlike an allergic reaction, there is usually no itching or hives. Swelling of the gut can cause intestinal cramping. If swelling closes the airway, it can be fatal. Attacks of swelling can become more severe in late childhood and adolescence.

There is usually a family history of the condition, but this may only reported to descendants as an unexpected, sudden, and premature death of a parent, aunt, uncle, or grandparent.




Review Date: 10/18/2004
Reviewed By: Updates to specific sections made by Jacqueline A. Hart, M.D., Senior Medical Editor, A.D.A.M., Inc. Previously reviewed by Donald Accetta, MD, MPH, President, Allergy & Asthma Care, PC, Taunton, MA. Review provided by VeriMed Healthcare Network (4/27/2004).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2004 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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