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Hereditary angioedema

Transmitted as an autosomal dominant trait, hereditary angioedema is secondary to the absence of an inhibitor of the first component of complement, C1 esterase inhibitor. Severe generalized urticaria can result.
Respiratory distress from upper respiratory tract involvement with urticaria is potentially fatal.
Edema of the GI tract can result in pain and diarrhea.

Treatment:

  • C1-INH concentrate
  • Epsilom – aminocaproic acid
  • Tranexamic acid
Hereditary angioedema

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