title: Typical Hus: Evidence of Acute Phase Complement Activation from a Daycare Outbreak.
abstract: The clinical manifestations of typical hemolytic uremic syndrome (HUS) encompass a wide spectrum. Despite the potentially severe sequelae from this syndrome, treatment approaches remain supportive. We present the clinical course of a child who contracted Shiga toxin-positiveE. coli(STEC) from a daycare center during an outbreak. Utilizing the modified Ham test which is a rapid, serum-based functional assay used to detect activation of the alternative pathway of complement as observed in atypical HUS, patient sera revealed evidence of increased complement activation in the acute phase of the syndrome but not after resolution. Further, this complement activation was attenuated by eculizumabin vitro, an effect that was replicatedin vitroutilizing Shiga toxin as a stimulus of complement activation in normal serum. Our report suggests that complement blockade may be effective in the treatment of STEC-HUS when initiated early in the disease. Given the epidemic nature of the disease that limits the feasibility of randomized clinical trials, further studies are needed to determine the value of early eculizumab treatment in STEC-HUS.
authors.affiliation: Division of Pediatric Nephrology, Johns Hopkins University School of Medicine, USA.
authors.forename: Tammy M
authors.initials: TM
authors.lastname: Brady
authors.affiliation: Division of Pediatric Nephrology, Johns Hopkins University School of Medicine, USA.
authors.forename: Cozumel
authors.initials: C
authors.lastname: Pruette
authors.affiliation: Division of Pediatric Nephrology, Johns Hopkins University School of Medicine, USA.
authors.forename: Lauren F
authors.initials: LF
authors.lastname: Loeffler
authors.affiliation: Division of Pediatric Nephrology, Children's Mercy Hospital, Kansas City, MO, USA.
authors.forename: Darcy
authors.initials: D
authors.lastname: Weidemann
authors.affiliation: Division of Pediatric Hematology Johns Hopkins University School of Medicine, USA; Division of Hematology, Johns Hopkins University School of Medicine, USA.
authors.forename: John J
authors.initials: JJ
authors.lastname: Strouse
authors.affiliation: Division of Hematology, Johns Hopkins University School of Medicine, USA.
authors.forename: Eleni
authors.initials: E
authors.lastname: Gavriilaki
authors.affiliation: Division of Hematology, Johns Hopkins University School of Medicine, USA.
authors.forename: Robert A
authors.initials: RA
authors.lastname: Brodsky
keywords: Hemolytic anemia; Hemolytic uremic syndrome; White blood cell