HEREDITARY ANGIOEDEMA - CLINICAL SYNDROME AND ITS MANAGEMENT
HEREDITARY ANGIOEDEMA - CLINICAL SYNDROME AND ITS MANAGEMENT
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DOI：
10.7326/0003-4819-84-5-580
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发表时间：
1976-01-01
影响因子：
39.2
通讯作者：
ATKINSON, JP
中科院分区：
文献类型：
--
作者：
FRANK, MM;GELFAND, JA;ATKINSON, JP
遗传性血管性水肿[人类]表现为四肢、面部、躯干气道或腹部内脏肿胀的发作，自发发生或继发于创伤。它是作为常染色体显性遗传性状遗传的，是由于激活的补体第一组分（C1）的抑制剂活性不足所致。临床诊断可以通过C4或C1酯酶抑制剂活性水平低或两者的结果来证实。治疗可分为长期预防发作、短期预防发作和治疗急性发作。抗纤溶药物和雄激素可实现长期预防。这些药物和血浆输注的短期预防是成功的。目前尚无针对急性发作的特异性治疗方法，但良好的支持性护理，加上对疾病过程的了解，可以预防气道阻塞引起的窒息。在治疗出现之前，据报道死亡率高达30%。
Hereditary angioedema [human] is manifested by attacks of swelling of the extremities, face, trunk airway, or abdominal viscera, occurring spontaneously or secondary to trauma. It is inherited as an autosomal dominant trait and is due to deficient activity of the inhibitor of the activated 1st component of complement (C1). The clinical diagnosis can be confirmed by the findings of low levels of C4 or C1 esterase inhibitor activity, or both. Therapy may be divided into long-term prophylaxis of attacks, short-term prophylaxis of attacks and treatment of acute attacks. Long-term prophylaxis may be achieved with antifibrinolytic agents and androgens. Short-term prophylaxis with these agents and plasma transfusions was successful. Specific therapy for acute attacks is not available, but good supportive care, together with a knowledge of the course of the disease can prevent asphyxiation from airway obstruction. Before the advent of therapy mortality was reported as high as 30%.