Clinical Studies of Protein C

Clinical Studies of Protein C
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蛋白 C 的临床研究

DOI:
10.1055/s-2007-1004419
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发表时间:
1984
影响因子:
5.7
通讯作者:
J. Griffin
J. Griffin
中科院分区:
医学2区
文献类型:
--
作者:
J. Griffin

文献摘要

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血浆蛋白C的主要临床重要性是由遗传性蛋白C缺乏一半正常水平和复发性静脉血栓栓塞性疾病之间的强相关性证明的。纯合子蛋白C缺乏个体在没有持续治疗干预的情况下不能存活超过婴儿期。蛋白C缺乏症的范围越来越广,包括分子异常和功能活性分子半正常水平的患者。很少,少数患有血栓形成的年轻人被确定蛋白C水平低于25%。蛋白C活性的研究一直受到阻碍,直到最近血浆蛋白C功能测定的发展。将这些检测应用于涉及血栓并发症的各种临床情况才刚刚开始,可能会导致新数据的爆炸性增长,这些数据应该被证明是最吸引人的,并进一步深入了解蛋白C在血栓形成调控中的作用。
The major clinical importance of plasma protein C is attested to by the strong association between inherited protein C deficiencies of half normal levels and recurrent venous thromboembolic disease. Homozygous protein C deficient individuals do not survive beyond infancy without continuous therapeutic intervention. The spectrum of protein C deficiency is becoming broader and includes patients with both abnormal molecules and half normal levels of functionally active molecules. Rarely, a few young adults with thrombosis have been identified with protein C levels below 25%. Studies of protein C activity have been hampered until the very recent developments of functional assays of plasma protein C. Application of these assays to a wide variety of clinical situations involving thrombotic complications is just beginning and may lead to an explosive proliferation of new data that should prove most fascinating and give much further insight into the contributions of protein C in the regulation of thrombosis.