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Laboratory Approaches for Assessing Contact System Activation

机译:评估联系系统激活的实验室方法

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Hereditary angioedema (HAE) is a rare autosomal dominant disease clinically characterized by recurrent, often unpredictable attacks of subcutaneous and mucosal swelling. Acute episodes are debilitating, painful, disfiguring, and potentially fatal. HAE type I and type II result from a deficiency in the plasma level of functional C1 inhibitor. HAE with normal levels of C1 inhibitor has been recognized. There is evidence that contact activation underlies the recurrent attacks of swelling. This article reviews laboratory parameters to detect contact system activation and implications for diagnosis of HAE and other forms of bradykinin-mediated angioedema.
机译:遗传性血统(HAE)是一种稀有的常染色体显性疾病,其特征在于经常性,通常是不可预测的皮下和粘膜肿胀的攻击。 急性发作是衰弱,痛苦,毁容和可能致命的。 Hae I类和II型从功能性C1抑制剂的血浆水平缺乏造成的。 已经认识到具有正常C1抑制剂的HAE。 有证据表明联系激活下潜过肿胀的经常性攻击。 本文审查了检测联系系统激活和对HAE和其他形式的Bradykinin介导的血管模型的影响的实验室参数。

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