Hereditary angioedema

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Hereditary angioedema

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Hereditary angioedema p. 676

complement disorder and p. 105

Summary

Hereditary angioedema (HAE) is an autosomal dominant genetic disorder in which there is a C1-esterase inhibitor deficiency. This can lead to uncontrolled activation of kallikrein and increased bradykinin, resulting in episodes of swelling, most commonly of the face and extremities. Attacks can also affect the airway, causing difficulty breathing, and the gastrointestinal tract, causing abdominal pain.

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