Pathogenetic mechanisms determining the origin and development of a hemolytic-uremic syndrome in children

Hemolytic uremic syndrome (HUS) induced by Shiga toxin-producing E. coli (STEC) is the most common causes of acute kidney injury in children. The therapy of the disease is symptomatic and the main factors leading to the development of severe course of a STEC-HUS are still unknown. In our study, we dealt with factors leading to development of a severe course of STEC-HUS in pediatric patients on both the host and pathogen side. Using retrospective analysis of the courses in children in the Czech Republic, we found that the most common cause of STEC-HUS was serotype O26 and HUS most often affected children under 3 years of age. 63,8 % required dialysis and mortality was 8.62 %. On the host side we focused on the relationship between the activation of the alternative complement pathway and the severity of the course of HUS. We found a significant difference in the level of the C3 part of complement in patients who required dialysis and patients for whom dialysis was not necessary. We also a cut-off value for the C3 part of complement and its reduction below 0.825 g / l was associated with the need for dialysis treatment and a higher incidence of extrarenal complications. Based not only on our results, it can be assumed that the therapeutic effect of complement could affect the severity of the disease...