Dynamics of complement activation in aHUS and how to monitor eculizumab therapy

  • Marina Noris
    IRCCS - Istituto di Ricerche Farmacologiche “Mario Negri,” Clinical Research Center for Rare Diseases “Aldo e Cele Daccò,” Ranica, Bergamo, Italy and “Centro Anna Maria Astori” Science and Technology Park Kilometro Rosso, Bergamo, Italy;
  • Miriam Galbusera
    IRCCS - Istituto di Ricerche Farmacologiche “Mario Negri,” Clinical Research Center for Rare Diseases “Aldo e Cele Daccò,” Ranica, Bergamo, Italy and “Centro Anna Maria Astori” Science and Technology Park Kilometro Rosso, Bergamo, Italy;
  • Sara Gastoldi
    IRCCS - Istituto di Ricerche Farmacologiche “Mario Negri,” Clinical Research Center for Rare Diseases “Aldo e Cele Daccò,” Ranica, Bergamo, Italy and “Centro Anna Maria Astori” Science and Technology Park Kilometro Rosso, Bergamo, Italy;
  • Paolo Macor
    Department of Life Sciences, University of Trieste, Trieste, Italy;
  • Federica Banterla
    IRCCS - Istituto di Ricerche Farmacologiche “Mario Negri,” Clinical Research Center for Rare Diseases “Aldo e Cele Daccò,” Ranica, Bergamo, Italy and “Centro Anna Maria Astori” Science and Technology Park Kilometro Rosso, Bergamo, Italy;
  • Elena Bresin
    IRCCS - Istituto di Ricerche Farmacologiche “Mario Negri,” Clinical Research Center for Rare Diseases “Aldo e Cele Daccò,” Ranica, Bergamo, Italy and “Centro Anna Maria Astori” Science and Technology Park Kilometro Rosso, Bergamo, Italy;
  • Claudio Tripodo
    Tumor Immunology Unit, Human Pathology Section, Department of Health Sciences, University of Palermo, Palermo, Italy;
  • Serena Bettoni
    IRCCS - Istituto di Ricerche Farmacologiche “Mario Negri,” Clinical Research Center for Rare Diseases “Aldo e Cele Daccò,” Ranica, Bergamo, Italy and “Centro Anna Maria Astori” Science and Technology Park Kilometro Rosso, Bergamo, Italy;
  • Roberta Donadelli
    IRCCS - Istituto di Ricerche Farmacologiche “Mario Negri,” Clinical Research Center for Rare Diseases “Aldo e Cele Daccò,” Ranica, Bergamo, Italy and “Centro Anna Maria Astori” Science and Technology Park Kilometro Rosso, Bergamo, Italy;
  • Elisabetta Valoti
    IRCCS - Istituto di Ricerche Farmacologiche “Mario Negri,” Clinical Research Center for Rare Diseases “Aldo e Cele Daccò,” Ranica, Bergamo, Italy and “Centro Anna Maria Astori” Science and Technology Park Kilometro Rosso, Bergamo, Italy;
  • Francesco Tedesco
    IRCCS, Istituto Auxologico Italiano, Milan, Italy;
  • Alessandro Amore
    Unit of Nephrology, Dialysis and Transplantation, Regina Margherita University Hospital, Turin, Italy; and
  • Rosanna Coppo
    Unit of Nephrology, Dialysis and Transplantation, Regina Margherita University Hospital, Turin, Italy; and
  • Piero Ruggenenti
    Unit of Nephrology and Dialysis, Azienda Ospedaliera Papa Giovanni XXIII, Bergamo, Italy
  • Eliana Gotti
    Unit of Nephrology and Dialysis, Azienda Ospedaliera Papa Giovanni XXIII, Bergamo, Italy
  • Giuseppe Remuzzi
    IRCCS - Istituto di Ricerche Farmacologiche “Mario Negri,” Clinical Research Center for Rare Diseases “Aldo e Cele Daccò,” Ranica, Bergamo, Italy and “Centro Anna Maria Astori” Science and Technology Park Kilometro Rosso, Bergamo, Italy;

抄録

<jats:title>Key Points</jats:title> <jats:p>Endothelial-restricted complement activation occurs in aHUS, and clinical remission relies on efficient endothelial complement inhibition. Ex vivo serum-induced endothelial C5b-9 deposits are a sensitive tool to monitor complement activation and eculizumab effectiveness in aHUS.</jats:p>

収録刊行物

  • Blood

    Blood 124 (11), 1715-1726, 2014-09-11

    American Society of Hematology

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