Hemolytic uremic syndrome; pathogenesis, treatment, and outcome

Abstract

The hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and chronic morbidity. It is therefore relevant and appropriate that pediatricians remain familiar with the various subsets of the disease including its classification, management, and outcome. This review will focus on recent information relative to epidemiology, pathogenesis, treatment, and outcome. It will include some of the newer associations between HUS and a variety of infections, including, but not limited to E. coli 0157:H7 (Shiga toxin-mediated) HUS, as well as the ever-increasing number of associations between HUS and a variety of drugs. It will review some of the newer therapies for the more common subsets, but will acknowledge that choosing evidence-based therapies is often limited by our incomplete understanding of the various pathogenic cascades, and that with the possible exception of Shiga toxin-mediated HUS(D+HUS), long-term outcome information is often limited by small numbers and limited follow-up. This review should provide a framework for making the proper diagnosis, implementing appropriate treatment, and advising the family about anticipated outcome.