Four infants with Haemolytic-Uremic Syndrome (HUS) were investigated sequentially for F VIII R: Ag/F VIII: C ratio, F VIII: C (one-stage method)/ F VIII: C (two-stage method) ratio and beta-thromboglobulin levels. There is a close relationship between the patterns of FVIII R: Ag/F VIII: C ratio and beta-thromboglobulin, as well as between these parameters and the course of the disease. High values of F VIII R: Ag/F VIII: C ratio and beta-thromboglobulin levels were found in the acute phase of the disease and normal values during remission (after treatment with plasma and anti-aggregating agents). Moreover, normal values of F VIII: C (one-stage method)/F VIII: C (two-stage method) ratio (indicating enhanced thrombin production) were always found.These data confirm the prominent role of endothelial damage and subsequent platelets aggregation in the pathogenesis of HUS in children.
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