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Báo cáo y học: "Pediatric hereditary angioedema due to C1-inhibitor deficiency"

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: Pediatric hereditary angioedema due to C1-inhibitor deficiency. | Farkas Allergy Asthma Clinical Immunology 2010 6 18 http www.aacijournal.eom content 6 1 18 ALLERGY ASTHMA CLINICAL IMMUNOLOGY REVIEW Open Access Pediatric hereditary angioedema due to C1-inhibitor deficiency Henriette Farkas Abstract Hereditary angioedema HAE resulting from the deficiency of the C1 inhibitor C1-INH is a rare life-threatening disorder. It is characterized by attacks of angioedema involving the skin and or the mucosa of the upper airways as well as the intestinal mucosa. In approximately 50 per cent of cases clinical manifestations may appear during childhood. The complex management of HAE in pediatric patients is in many respects different from the management of adults. Establishing the diagnosis early preferably before the onset of clinical symptoms is essential in cases with a positive family history. Complement studies usually afford accurate diagnosis whereas molecular genetics tests may prove helpful in uncertain cases. Appropriate therapy supported by counselling suitable modification of lifestyle and avoidance of triggering factors which primarily include mechanical trauma mental stress and airway infections in children may spare the patient unnecessary surgery and may prevent mortality. Prompt control of edematous attacks short-term prophylaxis and intermittent therapy are recommended as the primary means for the management of pediatric cases. Medicinal products currently used for the treatment of children with hereditary angioedema include antifibrinolytics attenuated androgens and C1-INH replacement therapy. Current guidelines favour antifibrinolytics for long-term prophylaxis because of their favorable safety profile but efficacy may be lacking. Attenuated androgens administered in the lowest effective dose are another option. C1-INH replacement therapy is also an effective and safe agent for children. Regular monitoring and follow-up of patients are necessary. 1. Introduction The deficiency of the C1 inhibitor C1-INH is inherited as an