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In Europe, haemolytic uraemic syndrome (HUS) is the most frequent cause of acute renal insufficiency during childhood. In the Netherlands about 20 children per year are seriously affected. Following a prodromal phase of mostly bloody diarrhoea, the main symptoms become evident, namely, haemolytic anaemia, thrombocytopenia and renal failure. The syndrome is caused by a verocytotoxin producing Escherichia coli, which damages the endothelium of the arterioles and glomeruli in the kidney. In humans, the most important cause of infection is the consumption of infected food-stuffs. After transport through the intestinal epithelium, the toxin becomes attached to the neutrophils, which transport it through the circulatory system, mainly to the kidneys where it has a toxic effect. Why the endothelial cells in these organs are the main targets for this toxin is as yet unknown. A symptomatic treatment is given to the 10% of infected patients who develop HUS. Increasing knowledge about the pathogenesis has resulted in new forms of treatment that will be studied during the next few years.