PATHOPHYSIOLOGY OF HEMOLYTIC UREMIC SYNDROME

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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.