title: Clinical grand rounds: atypical hemolytic uremic syndrome.
abstract: Atypical hemolytic uremic syndrome (aHUS) is a rare, lifethreatening, chronic, genetic disease of uncontrolled alternative pathway complement activation. The understanding of the pathophysiology and genetics of this disease has expanded over recent decades and promising new developments in the management of aHUS have emerged. Regardless of the cause of aHUS, with or without a demonstrated mutation or autoantibody, blockade of terminal complement activation through C5 is of high interest as a mechanism to ameliorate the disease. Eculizumab, an existing monoclonal antibody directed against C5 with high affinity, prevents the perpetuation of the downstream activation of the complement cascade and the damage caused by generation of the anaphylotoxin C5a and the membrane attack complex C5b-9, by blocking C5 cleavage. We report the successful use of eculizumab in a patient after kidney transplantation and discuss the disease aHUS.
authors.affiliation: Division of Kidney Diseases, Department of Pediatrics, Feinberg School of Medicine, Northwestern University, and Children's Memorial Hospital, Chicago, IL, USA.
authors.forename: Kavita S
authors.initials: KS
authors.lastname: Hodgkins
authors.forename: Amy E
authors.initials: AE
authors.lastname: Bobrowski
authors.forename: Jerome C
authors.initials: JC
authors.lastname: Lane
authors.forename: Craig B
authors.initials: CB
authors.lastname: Langman
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome; D015938:Complement Membrane Attack Complex / Q000187:drug effects / Q000276:immunology*; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007223:Infant; D016030:Kidney Transplantation / Q000009:adverse effects*; D016896:Treatment Outcome
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D015938:Complement Membrane Attack Complex; C481642:eculizumab