The role of plasma exchange in the treatment of severe forms of hemolytic-uremic syndrome in childhood.

Therapeutic plasma exchange (PE) or plasma-pheresis has been used in recent years in the treatment of severe hemolytic uremic syndrome (HUS) in children. We analyzed the benefit of PE and peritoneal dialysis (PD) in 9 children, 6 boys and 3 girls, aged 1-10 years, from 1983-1993. All children came from different geographical regions, and all had the sporadic form of the illness. Three patients had the gastrointestinal form, 5 had respiratory prodromes while 1 child developed HUS during the course of varicella. Seven children were hypertensive, but only in 3 was hypertension persistent. The child with varicella had a transient complement decrease. Five children were treated with PE. In 4 children, fresh frozen plasma (FFP) was used as replacement fluid, and human albumin was used in 1 child. Four children were treated with PD and infusions of FFP. Rapid recovery of renal function was observed in 5 patients whereas in 2 oliguric children the recovery of renal function ensued within 1 and 2 months, respectively. Two children developed terminal renal failure (TRF) (in 1 child the treatment was very delayed, and in other child HUS developed following varicella). Only 1 boy had relapses of the disease followed by impairment of renal function from which he gradually recovered. During the 3-10 year follow-up period, only the child with relapses was hypertensive while the others had normal clinical and laboratory parameters. We suggest that PE plays an important role in the early treatment of severe forms of HUS in children.