Endothelin in the Urine of Children with the Hemolytic Uremic Syndrome

Abstract

The pathogenic cascade leading to acute renal failure and hypertension in classic childhood hemolytic uremic syndrome (HUS) presumably is initiated by injury to the renal vascular endothelial cells. Multisystem involvement often occurs, apparently as a consequence of widespread endothelial cell perturbation. There is evidence that endothelial cell injury can be caused by circulating Shiga-like toxins (verotoxins) produced by certain diarrhea-producing enteric organisms (eg, Escherichia coli 0157:H7). Platelet and coagulation cascade activation probably occur secondarily with subsequent platelet-fibrin deposition within the renal microvasculature. Kidney biopsy specimens, however, often fail to show structural damage sufficient to account for the severe oliguric renal failure that often occurs in the syndrome.