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在ADAMTS13活动中适度下降与STEC-HUS的严重程度相关.

Khadizha M Emirova1,2, Olga M Orlova1,2, Ekaterina M Chichuga3

  • 1Department of Pediatrics, A.I. Yevdokimov Moscow State University of Medicine and Dentistry, Moscow 127473, Russia.

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概括
此摘要是机器生成的。

适度下降的ADAMTS13活性与严重的Siga毒素诱导的血溶性尿素性综合征 (STEC-HUS) 在儿童中有关. 这种减少可以预测严重的并发症,如器官衰竭和急性损伤,需要透析.

关键词:
亚当斯13 亚当斯13斯蒂克 - 哈斯的牛排.孩子们的孩子们的孩子们的孩子们.血溶性尿素性综合征是什么血小板 血小板 是·维勒布兰德因素是什么

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科学领域:

  • 儿科脏病学 儿科脏病学
  • 血液学 血液学 血液学
  • 微生物学 微生物学

背景情况:

  • 非典型的血溶性尿性综合征 (HUS) 是由大肠杆菌的Shiga毒素引起的,导致血栓性微血管病变 (TMA).
  • TMA的发病包括ADAMTS13-von Willebrand因子 (vWF) -血小板系统失衡.
  • STEC-HUS是幼儿急性损伤 (AKI) 的主要原因.

研究的目的:

  • 调查中度降低的ADAMTS13活性在STEC-HUS病变发生中的作用.
  • 分析小儿STEC-HUS患者的ADAMTS13活性和疾病严重程度之间的相关性.

主要方法:

  • 在138名患有急性STEC-HUS.US的儿童中测量了ADAMTS13的活性和度.
  • 在一小部分患者中评估了vWF抗原和活性水平.
  • 分析了关于疾病过程和严重程度的临床数据.

主要成果:

  • 分别在79.8%和90.6%的患者中观察到ADAMTS13活性和度的降低.
  • 在48.6%和34.4%的患者中发现vWF抗原和活性升高.
  • 血小板缺血影响97.8%的儿童;减少ADAMTS13与严重结局风险增加相关.

结论:

  • 适度下降的ADAMTS13活性与儿童严重的STEC-HUS表现有关.
  • 减少ADAMTS13活性可能预示着多器官衰竭,大脑疾病,肺水和需要透析的AKI的发展.
  • 减少ADAMTS13活动可以作为STEC-HUS严重性的潜在生物标志物.