tailieunhanh - Báo cáo y học: "HAE therapies: past present and future"

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: HAE therapies: past present and future. | Zuraw Allergy Asthma Clinical Immunology 2010 6 23 http content 6 1 23 ALLERGY ASTHMA CLINICAL IMMUNOLOGY REVIEW Open Access HAE therapies past present and future Bruce L Zuraw Abstract Advances in understanding the pathophysiology and mechanism of swelling in hereditary angioedema HAE has resulted in the development of multiple new drugs for the acute and prophylactic treatment of patients with HAE. This review will recap the past treatment options review the new current treatment options and discuss potential future treatment options for patients with HAE. Introduction Hereditary angioedema with reduced C1 inhibitor function HAE is an autosomal dominant disease characterized by recurrent episodes of potentially life-threatening angioedema. The pathophysiology of HAE as well as the molecular mechanisms underlying attacks of swelling in HAE have been gradually dissected over the past 50 years 1-3 . These advances have led to a rapidly changing set of therapeutic options for patients with HAE. HAE patients typically begin to swell in childhood and often suffer increased symptoms about the time of puberty and continue to experience recurrent attacks of angioedema throughout the remainder of their lives 4 . Attacks of angioedema in HAE can be severe and prolonged typically lasting 3-5 days before the patient is well again. Abdominal attacks may result in hospitalization and all to often lead to inappropriate intra-abdom-inal surgery while oro-pharyngeal-laryngeal attacks can be life-threatening 4-6 . Despite striking advances in medical knowledge HAE patients continue to die from laryngeal attacks 7 8 . The disease thus imposes an enormous burden on patients as well as their families often preventing them from leading a productive life. Because of the significant morbidity and mortality associated with HAE careful management of these patients is essential. The management of HAE required attention to three areas treatment of acute episodes of .