Format

Send to

Choose Destination
Mol Immunol. 2015 Sep;67(1):31-42. doi: 10.1016/j.molimm.2015.03.246. Epub 2015 Apr 3.

Atypical aHUS: State of the art.

Author information

1
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.
2
Centre for Complement and Inflammation Research, Department of Medicine, Imperial College, London W12 0NN, UK.
3
Centro Investigaciones Biológicas and Ciber de Enfermedades Raras, Ramiro de Maeztu 9, 28040 Madrid, Spain.
4
Assistance Publique - Hopitaux de Paris, Service d'Immunologie Biologique, Hopital Europeen Georges Pompidou, Paris, France.
5
Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.
6
IRCCS - Istituto di Ricerche Farmacologiche "Mario Negri", Clinical Research Center for Rare Diseases "Aldo e Cele Daccò", Ranica, Bergamo, Italy.
7
Unidad de Investigación and Ciber de Enfermedades Raras, Hospital Universitario de La Paz_IdiPAZ, Paseo de la Castellana 261, 28046 Madrid, Spain.
8
Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany.
9
Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany; Friedrich Schiller University, Jena, Germany.
10
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.

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.

PMID:
25843230
DOI:
10.1016/j.molimm.2015.03.246
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center