The hemolytic uremic syndrome

Abstract

HUS is the most common cause of acute renal failure in infants and young children and follows a diarrheal prodrome about 90% of the time. Persuasive evidence shows that virtually all of postdiarrheal cases are caused by EHEC infections, and that the great majority of cases in the United States are caused by the EHEC serotype O157:H7. Mortality is approximately 5%, and approximately 10% of survivors are left with severe sequelae. A much larger number (30%-50%) experience mild chronic renal damage. Public health strategies, including zero tolerance for fecal contamination in slaughter houses and additional public education on proper food handling and cooking, does much to decrease the prevalence of the syndrome. Efforts to further dissect the postdiarrheal pathogenic cascade should continue, and an animal model needs to be developed. Only then will researchers be positioned to develop effective intervention strategies. Preventing life-threatening extrarenal complications, especially of the CNS, is a major challenge. Idiopathic nondiarrheal HUS accounts for approximately 10% of cases and comprises a poorly understood composite of HUS subsets. Research directed toward a better understanding of these mysterious variants also is a priority for the years ahead.