title: Intravascular platelet activation in the hemolytic uremic syndrome.
abstract: We studied intravascular platelet activation in patients with typical (epidemic) and atypical (sporadic) HUS and found defective aggregation, decreased platelet and increased plasma serotonin in both groups. The findings were present not only on admission during the thrombocytopenic stage of the disease, but persisted for several weeks after recovery of the platelet count. Reduced endothelial PGI2 production was significantly more common in plasma from atypical than typical cases. Plasma from both typical and atypical HUS patients induced aggregation of normal platelets, but this phenomenon was unrelated to both the presence of thrombocytopenia or the stage of the disease. Serum platelet aggregating activity was higher in the atypical HUS patients, and was not associated with immune complexes detectable by polyethylene glycol precipitation. The data indicate that intravascular platelet activation is a feature of both forms of HUS, and may be initiated by different mechanisms in the two subgroups. While abnormal PGI2 synthesis appears to be important in the atypical cases, neither defective PGI2 production nor platelet aggregation by plasma factors adequately accounts for platelet activation in the majority of typical cases.
authors.affiliation: Department of Paediatric Nephrology, Hospital for Sick Children, London, United Kingdom.
authors.forename: M D
authors.initials: MD
authors.lastname: Walters
authors.forename: M
authors.initials: M
authors.lastname: Levin
authors.forename: C
authors.initials: C
authors.lastname: Smith
authors.forename: T J
authors.initials: TJ
authors.lastname: Nokes
authors.forename: R M
authors.initials: RM
authors.lastname: Hardisty
authors.forename: M J
authors.initials: MJ
authors.lastname: Dillon
authors.forename: T M
authors.initials: TM
authors.lastname: Barratt
mesh_terms: D000293:Adolescent; D001792:Blood Platelets / Q000502:physiology*; D002648:Child; D002675:Child, Preschool; D011464:Epoprostenol / Q000096:biosynthesis; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000097:blood* / Q000145:classification / Q000209:etiology; D006801:Humans; D007223:Infant; D008297:Male; D010974:Platelet Aggregation; D012701:Serotonin / Q000097:blood
chemical_list: D012701:Serotonin; D011464:Epoprostenol