vAcquired ADAMTS-13 deficiency in pediatric patients with severe sepsis
vAcquired ADAMTS-13 deficiency in pediatric patients with severe sepsis
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DOI：
10.3324/haematol.10262
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发表时间：
2007-01-01
期刊：
影响因子：
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通讯作者：
Dong, Jing-fei
中科院分区：
文献类型：
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作者：
Nguyen, Trung C.;Liu, Anne;Dong, Jing-fei
我们研究了21例儿童严重脓毒症患者的超大型血管性血友病因子（ULVWF）蛋白水解状态，发现整个组患者的ADAMTS-13活性中度降低，但31%的患者存在严重的酶缺乏，严重缺乏与血管性血友病因子粘附活性、血小板减少的严重程度和血浆白细胞介素-6水平升高相关。它在临床上也与疾病的严重程度和器官功能障碍相关。这些结果表明，ULVWF蛋白水解在脓毒症患者中不足，在一个亚组患者中严重缺乏。这种缺乏可能导致与败血症相关的血小板减少症和缺血性器官衰竭。
We studied the state of ultralarge von Willebrand factor (ULVWF) proteolysis in 21 pediatric patients with severe sepsis and found that the overall group of patients had moderately reduced ADAMTS-13 activity, but 31% had severe enzymatic deficiency, The severe deficiency correlated with greater adhesion activity of von Willebrand factor, severity of thrombocytopenia and plasma levels of interleukin-6. It also correlated clinically with severity of illness and organ dysfunction. These results suggest that ULVWF proteolysis is insufficient in septic patients and severely deficient in a subgroup of patients. The deficiency may contribute to the development of thrombocytopenia and ischemic organ failure associated with sepsis.