title: Atypical aHUS: State of the art.
abstract: Tremendous advances in our understanding of the thrombotic microangiopathies (TMAs) have revealed distinct disease mechanisms within this heterogeneous group of diseases. As a direct result of this knowledge, both children and adults with complement-mediated TMA now enjoy higher expectations for long-term health. In this update on atypical hemolytic uremic syndrome, we review the clinical characteristics; the genetic and acquired drivers of disease; the broad spectrum of environmental triggers; and current diagnosis and treatment options. Many questions remain to be addressed if additional improvements in patient care and outcome are to be achieved in the coming decade.
authors.affiliation: Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, IA, USA; Division of Nephrology, Stead Family Department of Pediatrics, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
authors.forename: Carla M
authors.initials: CM
authors.lastname: Nester
authors.affiliation: Centre for Complement and Inflammation Research, Department of Medicine, Imperial College, London W12 0NN, UK.
authors.forename: Thomas
authors.initials: T
authors.lastname: Barbour
authors.affiliation: Centro Investigaciones Biológicas and Ciber de Enfermedades Raras, Ramiro de Maeztu 9, 28040 Madrid, Spain.
authors.forename: Santiago Rodriquez
authors.initials: SR
authors.lastname: de Cordoba
authors.affiliation: Assistance Publique - Hopitaux de Paris, Service d'Immunologie Biologique, Hopital Europeen Georges Pompidou, Paris, France.
authors.forename: Marie Agnes
authors.initials: MA
authors.lastname: Dragon-Durey
authors.affiliation: Assistance Publique - Hopitaux de Paris, Service d'Immunologie Biologique, Hopital Europeen Georges Pompidou, Paris, France.
authors.forename: Veronique
authors.initials: V
authors.lastname: Fremeaux-Bacchi
authors.affiliation: Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.
authors.forename: Tim H J
authors.initials: TH
authors.lastname: Goodship
authors.affiliation: Assistance Publique - Hopitaux de Paris, Service d'Immunologie Biologique, Hopital Europeen Georges Pompidou, Paris, France.
authors.forename: David
authors.initials: D
authors.lastname: Kavanagh
authors.affiliation: IRCCS - Istituto di Ricerche Farmacologiche "Mario Negri", Clinical Research Center for Rare Diseases "Aldo e Cele Daccò", Ranica, Bergamo, Italy.
authors.forename: Marina
authors.initials: M
authors.lastname: Noris
authors.affiliation: Centre for Complement and Inflammation Research, Department of Medicine, Imperial College, London W12 0NN, UK.
authors.forename: Matthew
authors.initials: M
authors.lastname: Pickering
authors.affiliation: Unidad de Investigación and Ciber de Enfermedades Raras, Hospital Universitario de La Paz_IdiPAZ, Paseo de la Castellana 261, 28046 Madrid, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Sanchez-Corral
authors.affiliation: Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany.
authors.forename: Christine
authors.initials: C
authors.lastname: Skerka
authors.affiliation: Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany; Friedrich Schiller University, Jena, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Zipfel
authors.affiliation: Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, IA, USA; Division of Nephrology, Stead Family Department of Pediatrics, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, IA, USA. Electronic address: richard-smith@uiowa.edu.
authors.forename: Richard J H
authors.initials: RJ
authors.lastname: Smith
mesh_terms: D000328:Adult; D065766:Atypical Hemolytic Uremic Syndrome / Q000209:etiology / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D001323:Autoantibodies / Q000096:biosynthesis; D002648:Child; D003141:Communicable Diseases / Q000150:complications / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D003167:Complement Activation; D003179:Complement C3b / Q000235:genetics / Q000276:immunology; D003180:Complement C3b Inactivator Proteins / Q000235:genetics / Q000276:immunology; D017242:Complement Factor H / Q000235:genetics / Q000276:immunology; D005786:Gene Expression Regulation / Q000276:immunology*; D020022:Genetic Predisposition to Disease; D006801:Humans; D007668:Kidney / Q000276:immunology / Q000473:pathology*; D007680:Kidney Neoplasms / Q000150:complications / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D016030:Kidney Transplantation
chemical_list: D001323:Autoantibodies; C493495:CFH protein, human; D003180:Complement C3b Inactivator Proteins; D003179:Complement C3b; D017242:Complement Factor H