Atypical hemolytic-uremic syndrome: a comparison with postdiarrheal disease

To compare the epidemiologic, laboratory, clinical, and outcome variables of atypical (nondiarrheal) hemolytic-uremic syndrome with those of classic postdiarrheal disease.

A 24-year retrospective review of 28 episodes of atypical HUS that occurred in 22 children compared with 266 episodes of typical postdiarrheal disease in 265 children treated during the same period.

Of the 294 episodes of HUS, 9.5% were atypical (nondiarrheal), and 18% of the patients in the atypical disease group had recurrences. Prodromal features (other than the presence or absence of diarrhea) were similar between the groups. White blood cell count and serum creatinine concentration on admission to the hospital and most abnormal blood urea nitrogen values during hospitalization were significantly lower (p = 0.02) in the patients with atypical HUS. Oliguria, anuria, and the need for dialysis were also less common (p = 0.02) in the atypical disease group. There were no deaths in the subset of patients with atypical disease; 3.4% of the patients in the typical disease group died. Although there were no statistically significant differences in the incidence of end-stage renal disease between the atypical and typical disease groups, two of the four patients with atypical disease who had recurrences also had end-stage renal disease. There were no significant differences in chronic renal sequelae between the groups one or more years after HUS.

In contrast to reports from most other regions, patients with atypical disease in our area of the western United States have milder acute nephropathy and, with the exception of those with recurrence, do not experience worse outcomes.