PATHOPHYSIOLOGY OF HEMOLYTIC UREMIC SYNDROME

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目及 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 溶血性尿毒综合征（HUS）是大肠杆菌O 157：H7感染的结果。 大约10%的感染儿童会出现这种并发症。 HUS发生在受感染儿童腹泻发作后约7或8天。 太平洋西北部是这种感染的流行地区。 为什么有些孩子会患上HUS，而大多数孩子不会，原因尚不清楚。 同样，急性感染后导致溶血尿毒综合征的一连串异常也没有得到很好的描述。 感染E.大肠杆菌O 157：H7感染可增加发生溶血尿毒综合征的风险。 我们已经证明，在HUS之前，存在严重的凝血异常。 与其在血栓性血小板减少性紫癜（TTP）的演变中至关重要不同，冯·维勒布兰德因子似乎也不发挥病理生理作用。 内皮细胞被认为是由HUS产生的毒素的关键靶标，因为HUS首先是血栓性疾病。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Hemolytic uremic syndrome (HUS) is a consequence of Escherichia coli O157:H7 infections. Approximately 10 percent of infected children develop this complication. HUS occurs approximately 7 or 8 days after the onset of diarrhea in infected children. The Pacific Northwest is an endemic area for this infection. The reasons why some children develop HUS and most do not is unknown. Similarly, the cascade of abnormalities leading to HUS following acute infection has also been poorly delineated. Antibiotics when administered to children infected with E. coli O157:H7 increase the risk of developing HUS. We have demonstrated that in advance of HUS, there are profound coagulation abnormalities. Von Willebrand Factor also does not appear to play a pathophysiologic role, unlike its critical importance in the evolution of thrombotic thrombocytopenic purpura (TTP). The endothelial cell is believed to be the critical target of the toxins elaborated by HUS because HUS is above all a thrombotic disorder.