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Ency. home > Disease > H > Hereditary angioedema

Hereditary angioedema   

Overview | Symptoms | Treatment | Prevention

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 and Risks

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.

Ency. home > Disease > H > Hereditary angioedema

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