C1酯酶抑制剂(人)治疗急性遗传性血管性水肿

IF 3 Q2 Medicine
S. Valle, A. T. França, R. Campos, A. Grumach
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引用次数: 1

摘要

遗传性血管性水肿(HAE)是一种相对罕见的疾病,其特征是急性发作性肿胀。这些肿胀可能会毁容、疼痛并危及生命。由于症状出现在不同的部位,而且大多数患者的诊断出现延误,导致不必要的痛苦和危险情况。HAE可对生活质量产生巨大影响。这种疾病的主要遗传缺陷是缺乏或无功能的C1INH,它调节补体、纤溶蛋白、钾化酶和纤溶蛋白途径。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
C1 Esterase Inhibitor (Human) for the Treatment of Acute Hereditary Angioedema
Hereditary angioedema (HAE) is a relatively rare disease characterized by acute episodes of swelling. These swellings can be disfiguring, painful and life-threatening. Since the symptoms occur in different areas and most patients experience a delay in their diagnosis, resulting in unnecessary suffering and dangerous situations. HAE can have a tremendous impact on the quality of life. The major genetic deficiency in this disorder is either an absent or nonfunctional C1INH which regulates the complement, fibrinolitic, kalikrein and plasmin pathways.
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来源期刊
CiteScore
3.70
自引率
0.00%
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