Hemolytic-uremic Syndrome: an Analysis of the Natural History and Prognostic Features

Sixty-seven children with hemolytic-uremic syndrome (HUS) were admitted between 1974 and 1981. Of these, 52 (78%) were aged less than 3 years. All children had acute renal failure and 48 (72%) required peritoneal dialysis. The etiology in twenty cases varied from bacterial and viral infections (7 and 5 cases, respectively) to renal irradiation with chemotherapy (2) and preexisting glomerulopathy (1). 5 (7%) children died during the acute phase of the illness. Long-term follow-up (mean 3 years 3 months) of 56 cases showed that 37 children (60%) had so far experienced no functional sequelae and 8 (13%) only mild sequelae while 3 (5%) were on iterative hemodialysis, 3 had severe chronic renal failure and high blood pressure (HBP) and 5 (8%) had HBP and normal kidney function. While the recovery rate was approximately 60% in all age groups, the mortality rate and serious after-effects were twice as frequent (42%) in children over 3 years of age as in those less than 3. Renal histology (total of 37) showed 12 cases of cortical necrosis, 22 of glomerular thrombotic microangiopathy (TMA) and 3 arterial TMA. Prognosis was poor for all cases of arterial TMA and 58% of those exhibiting cortical necrosis.