Predictors of fatality in postdiarrheal hemolytic uremic syndrome

OBJECTIVES. Describe the cause of deaths among patients with postdiarrheal hemolytic uremic syndrome (HUS) and identify predictors of death at the time of hospital admission. METHODS. Case-control study of 17 deaths among patients with HUS identified from the Intermountain HUS Patient Registry (1970-2003) compared against all nonfatal cases. RESULTS. Of the 17 total deaths, 15 died during the acute phase of disease. Two died because treatment was withdrawn based on their preexisting conditions, and 1 died because of iatrogenic cardiac tamponade; they were excluded from analysis. Brain involvement was the most common cause of death ( 8 of 12); congestive heart failure, pulmonary hemorrhage, and hyperkalemia were infrequent causes. Presence of prodromal lethargy, oligoanuria, or seizures and white blood cell count (WBC) > 20 x 10(9)/L or hematocrit > 23% on admission were predictive of death. In multivariate analysis, elevated WBC and elevated hematocrit were independent predictors. The combination of prodromal dehydration, oliguria, and lethargy and admission WBC values > 20 x 10(9)/L and hematocrit > 23% appeared in 7 of the 12 acute-phase deaths. CONCLUSIONS. Diarrheal HUS patients presenting with oligoanuria, dehydration, WBC > 20 x 10(9)/L, and hematocrit > 23% are at substantial risk for fatal hemolytic uremic syndrome. Such individuals should be referred to pediatric tertiary care centers.