Preemptive Use of Eculizumab for Living - Donor Kidney Transplantation in a Child with Atypical Hemolytic Uremic Syndrome

نویسنده

  • Mario Negri
چکیده

INTRODUCTION Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy characterized by overactivation of the alternative complement pathway. This syndrome is associated with severe clinical manifestations, a tendency to recur, and, until recently, a poor long-term prognosis (Noris & Remuzzi, 2009). With conventional supportive therapy, the expected rate of graft failure after kidney transplantation due to recurrent aHUS among patients with a mutation in a complement gene ranges from 50% to 80% within the first five years post transplant (Le Quintrec et al., 2013; Zimmerhackl, Scheiring, Prufer, Taylor, & Loirat, 2007).

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تاریخ انتشار 2015