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Upper airway considerations in hereditary angioedema

Papadopoulou-Alataki, Efimia

Current Opinion in Allergy and Clinical Immunology: February 2010 - Volume 10 - Issue 1 - p 20–25
doi: 10.1097/ACI.0b013e328334f629
Upper airway disease: Edited by Ruby Pawankar and David P. Skoner

Purpose of review The purpose of the present review is to outline the clinical aspects and management of the upper airway involvement in the patients suffering from hereditary angioedema.

Recent findings Molecular mechanisms of hereditary angioedema reviewed in the literature conclude that it is an autosomal dominant disorder, characterized by the deficiency of C1 inhibitor due to mutations of its gene (SERPING). Hereditary angioedema manifests as episodes of localized swelling in any site of the body from skin, gastrointestinal tract to the upper airway, where it is severe and life-threatening. The age of onset, frequency of attacks and the factors triggering upper airway swelling in hereditary angioedema are variable among different patients. Acute laryngeal edema should be managed in emergency with monitoring of airway patency. To avoid airway obstruction, therapy should begin early either with current treatment (C1 inhibitor concentrate) or with new drugs developed recently. In patients with recurrent upper airway swelling attacks, long-term prophylaxis is recommended.

Conclusion The use of old and new treatment in acute attacks as well as in prophylaxis (long and short-term) has changed the outcome of patients with hereditary angioedema who present upper airway swelling.

4th Department of Pediatrics, Aristotle University of Thessaloniki, General Regional Hospital Papageorgiou, Thessaloniki, Greece

Correspondence to Efimia Papadopoulou-Alataki, MD, PhD, Lecturer in Pediatric/Immunology, Aristotle University of Thessaloniki, General Regional Hospital Papageorgiou, Ring Road, 56403 Thessaloniki, Greece Tel: +30 2310 320814, Tel: +30 2310 991594; fax: +30 2310 693918; e-mail:

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