Is it possible to influence the mortality in children with hemolytic uremic syndrome?

THE CURRENT STATE: Hemolytic uremic syndrome (HUS) is the most frequent cause of acute renal failure in children. In our geographic conditions D(+)HUS prevails, in its etiology E. coli O157:H7 is represented most. In the course of HUS there can occur extrarenal damage to some organs, the manifestation of multiple organ failure is then possible. Delayed diagnosis of HUS, its complicated course and therapeutical strategy influence greatly the mortality rate of affected children.

The optimal therapeutical procedure was elaborated on the base of evaluating the causes of death in children with HUS, and, simultaneously, there were revealed further reserves that can decrease the mortality rate.

A retrospective analysis of mortality within 1982-2000 was carried out in one of three centres dealing with the therapy for HUS in children in the Czech Republic. The total number of HUS children was 69 (40 girls, 29 boys, mean age 4.5 years). 9 patients out of the analyzed group died.

Out of 9 children (4 girls, 5 boys) died, D(+)HUS was present in 7 (77.8%, 4 girls, 3 boys, mean age 2.1 years), D(-)HUS occurred in 2 subjects (22.2%, boys, mean age 7.5 years). Dialysis therapy was needed in all 9 children (peritoneal dialysis and/or hemodialysis). The mortality rate in our group of children was 13%. An autopsy carried out in dead children showed dominant severe affection of kidneys/brain/heart.

In spite of marked decrease in the number of HUS children died particularly in developed countries, permanent attention must be paid to this disease. Besides early diagnosis, corresponding therapy is necessary. That should be performed at a specialized centre. The elaborated algorithm can be used in the therapy for HUS. This is one of the ways for further decrease of both mortality and chronic morbidity in children suffering from HUS. (Tab. 3, Fig. 1, Ref. 15.).