Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học Critical Care giúp cho các bạn có thêm kiến thức về ngành y học đề tài: New treatments addressing the pathophysiology of hereditary angioedema. | Clinical and Molecular Allergy BioMed Central Open Access Review 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 Clinical and Molecular Allergy 2008 6 2 doi 1476-7961-6-2 Accepted I4 April 2008 This article is available from http 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 http licenses by 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 mucosa. Angioedema of the upper airways is extremely serious and has resulted in death by asphyxiation. Subnormal levels of C1-inhibitor are associated with the inappropriate activation of a number of pathways - including in particular the complement and contact systems and to some extent the fibrinolysis and coagulation systems. Current findings indicate bradykinin a product of contact system activation as the .
