Preemptive Use of Eculizumab for Living - Donor Kidney Transplantation in a Child with Atypical Hemolytic Uremic Syndrome
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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).
منابع مشابه
Prophylactic eculizumab after renal transplantation in atypical hemolytic-uremic syndrome.
To the Editor: Atypical hemolytic–uremic syndrome is a rare microangiopathic hemolytic condition characterized by thrombocytopenia and acute renal failure.1 The prognosis for patients with atypical hemolytic–uremic syndrome with a factor H mutation is poor; 60% have end-stage renal disease or die within a year.2 The expected rate of graft failure due to recurrent atypical hemolytic–uremic syndr...
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Atypical hemolytic uremic syndrome (aHUS) is a rare disorder characterized by over-activation and dysregulation of the alternative complement pathway. Its estimated prevalence is 1-2 per million. The disease is characterized by thrombotic microangiopathy, which causes anemia, thrombocytopenia, and acute renal failure. aHUS has more severe course compared to typical (infection-induced) HUS and i...
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تاریخ انتشار 2015