BACKGROUND: Hereditary angioedema (HAE), a rare autosomal dominant disorder, is characterized by repeated attacks of swelling of the skin, gastrointestinal tract, face, larynx, and other organs. In most cases it is caused by low levels of functional C1 esterase inhibitor (C1-INH), a serine protease inhibitor that plays important regulatory roles in the complement, contact, and fibrinolytic pathways.
METHODS: Lack of functional C1-INH results in excessive release of bradykinin, which triggers vasodilation, vascular permeability, and edema. Most attacks are mild and self-limiting, but untreated laryngeal attacks may cause rapid asphyxiation and death. Potential triggers of laryngeal attacks include trauma to or manipulation of the face, mouth, or upper airway. Therefore, before performing such a procedure in a patient with HAE, the otolaryngologist should consult with the patient, the physician managing the HAE, and the anesthesiologist and make appropriate preparations for prevention and/or treatment of an attack.
RESULTS: Current World Allergy Organization and European guidelines recommend the use of i.v. plasma-derived C1-INH replacement for short-term prophylaxis of angioedema attacks. Other effective options include danazol given for several days before and after the procedure and fresh-frozen plasma, but these may not be as effective as C1-INH and may be associated with a high rate of adverse events.
CONCLUSION: Acute attacks, which may occur many hours after a procedure, may be treated with C1-INH; icatibant, a bradykinin B2-receptor antagonist; or ecallantide, a kallikrein inhibitor, all of which have been shown to reduce the duration and severity of HAE attacks.
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