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Anaesthesia Management of a Patient with Hereditary Angioedema with Prophylactic Administration of C1 Esterase Inhibitor; Case report and literature review

Author(s): Aravind Narayanan | Rohit R. Date | Sanathkumar Birur | Pradipta Bhakta | Sinnakirouchenane Srinivasan

Journal: Sultan Qaboos University Medical Journal : SQUMJ
ISSN 2075-051X

Volume: 13;
Issue: 3;
Start page: 467;
Date: 2013;
Original page

Keywords: Anesthesia | Hereditary angioedema | C1 esterase inhibitor deficiency | Case Report | Oman

Hereditary angioedema (HAE) is a rare disorder caused by a deficiency of C1 esterase inhibitor. Minor trauma and emotional stress are the most common initiating events leading to contact system activation andexcessive uncontrolled bradykinin release. This manifests as angioedema, a vascular reaction of the deeper layers of the skin and mucous membranes, with vasodilatation and increased permeability resulting in tissue swelling. Severe angioedema can occur in the perioperative period, leading to fatal airway obstruction. We describe the anaesthetic management of a child with HAE for dental rehabilitation and provide an review of the relevant literature.
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