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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.
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Overview, Causes, & Risk Factors
Hereditary angioedema is an inherited disorder. It is caused by the absence of a protein called C1 inhibitor (or a defect in this protein), which in turn effects 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.
A family history is usually noted, at times only reported to descendants as an unexpected, sudden, and premature death of a parent, aunt/uncle, or grandparent.
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• Definition
• Overview, Causes, & Risk Factors
• Symptoms & Signs
• Prevention
• Diagnosis & Tests
• Treatment
• Prognosis
• Complications
• Calling Your Health Care Provider
• Pictures & Images
Topics that might be of interest to you Diseases & Conditions
• Angioedema
• Hives
Tests & Exams
• C1 Inhibiting Factor
• C4 Level
• Complement
Other Topics
• Allergic Reactions
• Breathing Difficulty
• Itching
• Swelling
Review Date : 1/17/2002
Reviewed By : Frederic F. Little, M.D., Department of Allergy and Pulmonary/Critical Care Medicine, Boston University School of Medicine, Boston, MA and Julie A. Miller, M.D., Department of Surgery, Royal Melbourne Hospital, Melbourne, Australia. Review provided by VeriMed Healthcare Network.
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