tailieunhanh - Báo cáo y học: "Management of hereditary angioedema: 2010 Canadian approach"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học General Psychiatry cung cấp cho các bạn kiến thức về ngành y đề tài: Management of hereditary angioedema: 2010 Canadian approach. | Bowen et al. Allergy Asthma Clinical Immunology 2010 6 20 http content 6 1 20 ALLERGY ASTHMA CLINICAL IMMUNOLOGY RESEARCH Open Access Management of hereditary angioedema 2010 Canadian approach 1 13 14 15 Tom Bowen John Brosz Kristylea Brosz Jacques Hebert Bruce Ritchie Abstract C1-inhibitor C1-INH deficiency is a rare blood disorder resulting in angioedema attacks that are debilitating and may be life-threatening. Prophylaxis and therapy of events has changed since our first Canadian Consensus Conference on the diagnosis therapy and management of HAE. We have formed the Canadian Hereditary Angioe-dema Network CHAEN Réseau Canadien d Angioédème Héréditaire RCAH - http to advance care of patients with this disorder in Canada. We here present a review of management of HAE in Canada. Introduction C1 inhibitor C1-INH deficiency presents in congenital hereditary angioedema HAE or acquired forms. There are three variants of hereditary angioedema HAE HAE-C1-INH Type I with low C1-INH protein and function 85 of cases autosomal dominant HAE-C1-INH Type II with normal protein but low function 15 of cases autosomal dominant and HAE Type III hereditary angioedema with normal C1 inhibitor protein and function estrogen-dependent inherited form found mostly in females some with defects in coagulation factor XII HAE-FXII others of unknown defect HAE-Unknown 1 2 . Acquired angioedema AAE is most frequently associated with lymphoproliferative and autoimmune disorders and with some medications including ACE inhibitors and plasmin activators 3 4 . Patients with HAE may experience recurrent soft tissue swellings intestinal swellings and abdominal pains and may have life-threatening swellings of the airway. The incidence of HAE is estimated at 1 10 000 to 1 50 000. Risk of dying from airway obstruction is not clear but deaths from this complication if left untreated are not uncommon 1 5 . To learn from international experience in HAE the first .