Clinical features of anti-factor H autoantibody-associated hemolytic uremic syndrome.

  title={Clinical features of anti-factor H autoantibody-associated hemolytic uremic syndrome.},
  author={Marie-Agn{\`e}s Dragon-Durey and Sidharth Kumar Sethi and Arvind Bagga and Caroline Blanc and Jacques Blouin and Bruno Ranchin and J. -L. Andre and Nobuaki Takagi and Hae Il Cheong and Pankaj Hari and Moglie Le Quintrec and Patrick Niaudet and Chantal Loirat and Wolf Herman Fridman and V{\'e}ronique Fr{\'e}meaux-Bacchi},
  journal={Journal of the American Society of Nephrology : JASN},
  volume={21 12},
Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy that associates, in 70% of cases, with genetic or acquired disorders leading to dysregulation of the alternative pathway of complement. Autoantibody directed against Factor H causes at least 6% to 10% of aHUS cases, but only a few clinical reports are available. Here, we describe the clinical, biologic, genetic features, treatment, and outcome of 45 patients who presented with aHUS associated with anti-FH… CONTINUE READING