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Báo cáo y học: "anagement of upper airway edema caused by hereditary angioedema"

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 upper airway edema caused by hereditary angioedema. | Farkas Allergy Asthma Clinical Immunology 2010 6 19 http www.aacijournal.eom content 6 1 19 ALLERGY ASTHMA CLINICAL IMMUNOLOGY REVIEW Open Access Management of upper airway edema caused by hereditary angioedema Henriette Farkas Abstract Hereditary angioedema is a rare disorder with a genetic background involving mutations in the genes encoding C1-INH and of factor XII. Its etiology is unknown in a proportion of cases. Recurrent edema formation may involve the subcutis and the submucosa - the latter can produce obstruction in the upper airways and thereby lead to lifethreatening asphyxia. This is the reason for the high 30-to 50-per-cent mortality of undiagnosed or improperly managed cases. Airway obstruction can be prevented through early diagnosis meaningful patient information timely recognition of initial symptoms state-of-the-art emergency therapy and close monitoring of the patient. Prophylaxis can substantially mitigate the risk of upper airway edema and also improve the patients quality of life. Notwithstanding the foregoing any form of upper airway edema should be regarded as a potentially life-threatening condition. None of the currently available prophylactic modalities is capable of preventing UAE with absolute certainty. Introduction Hereditary angioedema HAE is a rare disorder of autosomal dominant inheritance. Its genetic background involves mutation of the gene encoding the C1-inhibitor C1-INH or the factor XII protein FXII 1 2 . Diverse mutations in the C1-INH gene may result in either inadequately low C1-INH protein HAE-C1-INH-Type I or a dysfunctional protein in normal or even excessive amounts HAE-C1-INH-Type II 3 4 . This impairment of C1-INH function leads to the activation of all several plasma enzyme cascade complement fibrinolytic coagulation and kinin systems controlled by the C1-INH protein and leading to the release of bradykinin. Bradykinin a vasoactive mediator enhances capillary permeability and results in the extravasation of plasma .