Abstract

HUS is an important cause of acute renal failure in children and its pathogenesis remains unknown. Recently it has been suggested that in both HUS and Thrombotic Thrombocytopenic Purpura there is a defect of an endogenous inhibitor of platelet aggregation, prostacyclin. The infusion of plasma has been reported to be an effective means of reversing this defect. Ten consecutive children with HUS, anuric at the time of diagnosis and who required peritoneal dialysis were treated with daily plasma (5 ml/kg) for a mean of 7.8 days. Their outcome was compared retrospectively with ten consecutive patients, seen over the previous two years, who were also anuric and required peritoneal dialysis, but did not receive plasma. Recovery from the microangiopathic process, as judged by a rising platelet count, occurred after 6.5 days in the plasma treated group and after 6.6 days in those not given plasma. There were no deaths in either group but one patient in the no plasma group has required long term dialysis and two patients in each group have mild chronic renal failure. Six patients in the plasma treated group required dialysis for more than 14 days. These results do not suggest any beneficial effect from plasma infusion in anuric HUS.

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