The pathogenesis of hemolytic uremic syndrome (D+ HUS) is characterized by endothelial damage of glomeruli and tubules within the kidney. In several other diseases in which glomerular endothelial damage occurs, elevated serum levels of vascular endothelial growth factor (VEGF) have been reported. VEGF is involved in angiogenesis, permeabilization of blood vessel endothelium, and wound repair. In this study we evaluated VEGF levels in the serum of 40 D+ HUS patients in the acute phase and during the course of the disease. VEGF levels were measured using a double-sandwich ELISA. Indirect immunohistochemistry was performed for the detection of VEGF in renal biopsy material of 3 HUS patients. Significantly elevated VEGF levels were found in HUS patients compared with controls in both serum (P0.001) and plasma (P0.05). A significant relationship was found between VEGF levels and severity of the disease according to the classification of Gianantonio (P0.05). Levels of VEGF in blood increased during the 2nd and 3rd week after HUS was diagnosed. Immunohistochemistry of renal biopsy material showed increased levels of the receptors for VEGF in the glomeruli. During the course of HUS, plasma VEGF levels increase and the increase is dependent on the severity of the disease. This is probably associated with the repair process.