Hemolytic uremic syndrome - clinical aspects and outcome of an outbreak: Report of 28 cases.

Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. There are two main subgroups: the typical form of HUS follows a diarrheal prodrome (D+HUS) and the atypical form is without the diarrheal prodrome (D-HUS). We have studied 28 children with HUS over a period of 15 months between 1992 and 1993. The median age was 2.2 years (range from six months to six years). All children had prodromal diarrhea. Hypertension was present in 71% and neurological complications in 39%. All the patients had oliguria or anuria (16 oliguric and 12 anuric). The mean duration of anuria was 16 days (range seven to 42 days). Serum creatinine on admission ranged between 112 and 1064 Amicromol/L (mean 453 Amicromol/L). The lowest hemoglobin level and platelet count during hospitalization ranged between 38 and 87 g/L and 7 to 147x109/L respectively. Leukocytosis on admission was present in 22 patients, low C3 was documented in 11 patients (34%), and four patients had low C4. All patients received fresh frozen plasma transfusion, a total of 25 patients received dialysis therapy, 19 patients were treated with peritoneal dialysis (PD), one patient had hemodialysis (HD), and five patients had both HD and PD. The mean duration of dialysis was 18 days (range three to 56 days). Only one patient died (mortality rate 3%). The median duration of hospital stay was 28 days (range eight to 90 days). We conclude that HUS is emerging as an important clinical and public health problem and that early comprehensive management including dialysis therapy, aggressive management of hypertension, fresh frozen plasma transfusion, and nutritional support all improve the outcome and decrease the mortality and morbidity in patients with HUS.