The hemolytic-uremic syndrome (HUS) in childhood

HUS in children is caused in about 90% of cases by Shiga-toxin-producing E. coli (STEC), especially O157:H7, and therefore called typical or D+ HUS. T he most important reservoir for STEC is the intestine of cattle. The transm ission takes place by contaminated beef products or from person-to-person. The infection manifests itself with bloody diarrhea. 10% of cases develop H US, especially young children. After absorption into circulation Shiga-toxi ns bind to specific glycolipid-receptors (Gb3) to endothelial cells of the microvasculature of the kidney, colon and central nervous system. Various p athomechanisms finally lead to thrombotic microangiopathy with hemolytic an emia, thrombocytopenia and renal failure. Therapy consists of supportive me asures. Two thirds will need dialysis. Acute mortality is caused above all by severe extrarenal complications. It has decreased drastically over the l ast decades and now amounts to only 5% of cases. A further 5% develop prima ry end-stage renal disease (ESDR). About 30% sustain less severe chronic pr oblems (proteinuria, hypertension, decreased GFR) with the risk of developi ng ESDR. Until now there have been no proven prophylactic measures except h ygienic ones. The early administration of substances for binding of Shiga-t oxins in the intestine is under clinical investigation. Improvement of prog nosis is possible, however, only through early diagnosis of HUS, treatment in pediatric nephrology centers and long-term follow-up.