Abstract:

 The  term  atypical  hemolytic  uremic  syndrome  (aHUS)  has  been  used  since  the  mid⁃1970s.  It  was originally used  to  describe  the  familial  or  sporadic  cases,  as  opposed  to  the  typical  hemolytic  uremic  syndrome  with epidemiological characteristics. Over time, the term aHUS has evolved to include any form of hemolytic uremic syndrome that is not associated with Shiga toxin. Diseases with distinct etiologies and high heterogeneity are uniformly referred to as aHUS, which impedes the  study  of  specific  diseases  and  the  provision  of  targeted  therapies.  Recently,  the  United  States  National Kidney Foundation (NKF)  working group established a specialty⁃specific expert  group in the thrombotic microangiopathies, utilizing the Delphi approach to define and discuss several key issues related to the nomenclature of aHUS.

Key words:

hemolytic uremic syndrome   , thrombotic microangiopathy   , complement   , nomenclature