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Hereditary angioedema: Prophylaxis prior to procedures (short-term prophylaxis)

Authors
Marco Cicardi, MD
Bruce Zuraw, MD
Section Editor
Sarbjit Saini, MD
Deputy Editor
Anna M Feldweg, MD

INTRODUCTION

Hereditary angioedema (HAE or inherited C1 inhibitor [C1INH] deficiency) is a rare genetic disorder resulting from deficiency (type I) or dysfunction (type II) of C1INH. There is another form of HAE, in which patients have normal C1INH. This disorder affects mainly women, some of whom have defects in factor XII. It is exacerbated by estrogens in many patients. All forms of HAE are characterized by recurrent episodes of angioedema without pruritus or urticaria, which most often affect the skin or mucosal tissues of the upper respiratory and gastrointestinal tracts. Although swelling resolves spontaneously in two to four days in the absence of treatment, angioedema can often be temporarily debilitating and laryngeal angioedema may cause fatal asphyxiation.

Dental and medical procedures can trigger episodes of angioedema. The goal of short-term prophylaxis is to prevent these episodes with premedications. The various prophylactic therapies that are used prior to dental and medical procedures to prevent attacks will be reviewed here. Long-term prophylaxis to reduce the frequency and severity of angioedema episodes and other issues in the diagnosis and management of HAE are discussed elsewhere:

(See "Hereditary angioedema: General care and long-term prophylaxis".)

(See "Hereditary angioedema: Treatment of acute attacks".)

(See "Hereditary angioedema: Epidemiology, clinical manifestations, exacerbating factors, and prognosis".)

                            

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Literature review current through: Nov 2016. | This topic last updated: Mon Aug 03 00:00:00 GMT 2015.
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