Atypical aHUS: State of the art

Carla M. Nester, Thomas Barbour, Santiago Rodriquez de Cordoba, Marie Agnes Dragon-Durey, Veronique Fremeaux-Bacchi, Tim H J Goodship, David Kavanagh, Marina Noris, Matthew Pickering, Pilar Sanchez-Corral, Christine Skerka, Peter Zipfel, Richard J H Smith

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)31-42
Number of pages12
JournalMolecular Immunology
Volume67
Issue number1
DOIs
Publication statusPublished - Sep 1 2015

ASJC Scopus subject areas

  • Molecular Biology
  • Immunology
  • Medicine(all)

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