title: Atypical hemolytic uremic syndrome responsive to steroids and intravenous immune globulin.
abstract: Atypical hemolytic uremic syndrome remains a challenge to diagnose and treat, with significant acute morbidity and risk for progression to end stage renal disease. Treatment strategies center on plasma exchange but do not necessarily affect the progression of disease. We report the case of a patient with atypical HUS resulting from a mutation in the complement pathway who responded to treatment with steroids and IVIG, therefore avoiding transfusion or plasma exchange.
authors.affiliation: Aflac Cancer Center and Blood Disorders Service, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
authors.forename: Tanya
authors.initials: T
authors.lastname: Watt
authors.forename: Barry
authors.initials: B
authors.lastname: Warshaw
authors.forename: Howard M
authors.initials: HM
authors.lastname: Katzenstein
mesh_terms: D001803:Blood Transfusion; D005938:Glucocorticoids / Q000627:therapeutic use*; D006463:Hemolytic-Uremic Syndrome / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use; D007223:Infant; D008297:Male; D051916:Membrane Cofactor Protein / Q000235:genetics; D008775:Methylprednisolone / Q000627:therapeutic use*; D009154:Mutation; D016896:Treatment Outcome
chemical_list: C497857:CD46 protein, human; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D051916:Membrane Cofactor Protein; D008775:Methylprednisolone