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High-Dose Intravenous Gamma Globulin Infusions in Hemolytic-Uremic Syndrome: A Preliminary Report

KUMUDCHANDRA J. SHETH, MB, MRCP; JOAN COX GILL, MD; HEINZ E. LEICHTER, MD
Am J Dis Child. 1990;144(3):268-270. doi:10.1001/archpedi.1990.02150270014009.
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Sir.—The classic form of hemolyticuremic syndrome (HUS) follows a prodrome of bloody diarrhea due to verotoxin-producing Escherichia coli infection.1 Verotoxin damages endothelial cells2 and releases factor VIII, von Willebrand factor (vWF) antigen, and large multimers of vWF.3 These large vWF multimers, in the presence of a platelet agglutinating factor, may facilitate platelet aggregation, resulting in microthrombi and thrombocytopenia. A platelet agglutinating factor has been reported in HUS4 and can be inhibited by IgG obtained from adults only.4,5 Anecdotal reports suggest a beneficial effect of intravenous infusions of human gamma globulins (IVGG) in thrombotic thrombocytopenic purpura.6 Because the inciting factors and clinical manifestations of thrombotic thrombocytopenic purpura and HUS are similar, it is likely that both represent a spectrum of disease with a common mechanism7 and both might improve with IVGG therapy.

In our preliminary study, eight consecutively treated children (three boys, five girls;

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