Role of polymorphonuclear leukocytes in the pathophysiology of typical hemolytic uremic syndrome.
Thrombotic microangiopathy and acute renal failure are cardinal features of post-diarrheal hemolytic uremic syndrome (HUS). These conditions are related to endothelial and epithelial cell damage induced by Shiga toxin (Stx), through an interaction with its globotriaosylceramide (Gb3) receptor. Although, Stx is the main pathogenic factor and necessary for HUS development, clinical and experimental evidence suggest that the inflammatory response is able to potentiate Stx toxicity. Lipopolysaccharides (LPS) and neutrophils (PMN) represent two central components of inflammation during a Gram-negative infection. In this regard, patients with high peripheral PMN counts at presentation have a poor prognosis. In the present review, we discuss the contribution of experimental models and patient's studies in an attempt to elucidate the pathogenic mechanisms of HUS.
Exeni
Ramón A
RA
Departamento de Nefrología, Hospital Municipal del Niño, San Justo, Provincia de Buenos Aires, Argentina. rexeni@pccp.com.ar
Fernández
Gabriela C
GC
Palermo
Marina S
MS
D000818:Animals; D004195:Disease Models, Animal; D006463:Hemolytic-Uremic Syndrome / Q000276:immunology / Q000378:metabolism / Q000503:physiopathology*; D006801:Humans; D008070:Lipopolysaccharides / Q000276:immunology / Q000378:metabolism; D008954:Models, Biological; D009504:Neutrophils / Q000276:immunology / Q000378:metabolism / Q000502:physiology*
D008070:Lipopolysaccharides