溶血性尿毒症症候群(Hemolytic Uremic Syndrome:HUS)(<特集>血小板減少症とアフェレシス)

概要

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Hemolytic uremic syndrome (HUS) is diagnosed when patients present with the simultaneous features of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. HUS comprises two major clinical categories : diarrheal-associated, typical HUS (D+HUS) and the diarrhea-negative atypical form. In developed countries, over 90% of cases are subsequent to gastrointestinal infection with enterohemorrhagic Escherichia coli (EHEC). The disease process is initiated and perpetuated by interactions between the pathogen or its virulence factors (e.g. Shiga toxin-1 (Stx1) or Shiga toxin-2 (Stx2)) and host cells. Recently, it has been clarified that Stx activates a complement in human serum through an alternative pathway by binding to the cell-binding domains of complement factor H and inhibiting its regulatory function. Typical HUS is treated using supportive measures including dialysis, transfusion of PRBC, and so on. In addition, newer treatments for typical HUS are currently being examined, including Shiga toxin-neutralizing antibodies, multibranched Stx receptor analogs, and therapies targetting the terminal complement cascade. This review summarizes current knowledge regarding the pathophysiology of typical HUS, including the interactions of the pathogen and its virulence factors with cells in the intestine and kidney, and the available treatment options.
2012-02-29