An expert discussion on the atypical hemolytic uremic syndrome nomenclature—identifying a road map to precision: a report of a National Kidney Foundation Working Group

Carla M. Nester, David L. Feldman, Richard Burwick, Spero Cataland, Shruti Chaturvedi, H. Terence Cook, Adam Cuker, Bradley P. Dixon, Fadi Fakhouri, Sangeeta R. Hingorani, Anuja Java, Nicole C.A.J. van de Kar, David Kavanagh, Nelson Leung, Christoph Licht, Marina Noris, Michelle M. O'Shaughnessy, Samir V. Parikh, Flora Peyandi, Giuseppe RemuzziRichard J.H. Smith, C. John Sperati, Meryl Waldman, Patrick Walker, Marina Vivarelli

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

The term atypical hemolytic uremic syndrome has been in use since the mid-1970s. It was initially used to describe the familial or sporadic form of hemolytic uremic syndrome as opposed to the epidemic, typical form of the disease. Over time, the atypical hemolytic uremic syndrome term has evolved into being used to refer to anything that is not Shiga toxin–associated hemolytic uremic syndrome. The term describes a heterogeneous group of diseases of disparate causes, a circumstance that makes defining disease-specific natural history and/or targeted treatment approaches challenging. A working group of specialty-specific experts in the thrombotic microangiopathies was convened to review the validity of this broad term in an era of swiftly advancing science and targeted therapeutics. A Delphi approach was used to define and interrogate some of the key issues related to the atypical hemolytic uremic syndrome nomenclature.

Original languageEnglish
Pages (from-to)326-336
Number of pages11
JournalKidney International
Volume106
Issue number3
DOIs
StatePublished - Sep 2024

Keywords

  • complement
  • hemolytic uremic syndrome
  • nomenclature
  • thrombotic microangiopathy

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