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

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 and excessive uncontrolled bradykinin release. This manifests as angioedema, a vascular reactio...

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Bibliographic Details
Main Authors: Aravind Narayanan, Rohit R. Date, Sanathkumar Birur, Pradipta Bhakta, Sinnakirouchenane Srinivasan
Format: Article
Language:English
Published: Sultan Qaboos University 2013-08-01
Series:Sultan Qaboos University Medical Journal
Subjects:
Online Access:https://journals.squ.edu.om/index.php/squmj/article/view/1852
Description
Summary: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 and excessive 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.
ISSN:2075-051X
2075-0528