new treatments addressing the pathophysiology of hereditary angioedema新的治疗方法解决遗传性血管性水肿的病理生理学.pdfVIP

Clinical and Molecular Allergy BioMed Central Review Open Access New treatments addressing the pathophysiology of hereditary angioedema Alvin E Davis III Address: Professor of Pediatrics, Harvard Medical School, Senior Investigator, Immune Disease Institute, 800 Huntington Avenue, Boston, MA 02114, USA Email: Alvin E Davis - aldavis@ Published: 14 April 2008 Received: 28 December 2007 Accepted: 14 April 2008 Clinical and Molecular Allergy 2008, 6:2 doi:10.1186/1476-7961-6-2 This article is available from: /content/6/1/2 © 2008 Davis; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract Hereditary angioedema is a serious medical condition caused by a deficiency of C1-inhibitor. The condition is the result of a defect in the gene controlling the synthesis of C1-inhibitor, which regulates the activity of a number of plasma cascade systems. Although the prevalence of hereditary angioedema is low – between 1:10,000 to 1:50,000 – the condition can result in considerable pain, debilitation, reduced quality of life, and even death in those afflicted. Hereditary angioedema presents clinically as cutaneous swelling of the extremities, face, genitals, and trunk, or painful swelling of the gastrointestinal