Maintenance of Kidney Function Following Treatment With Eculizumab and Discontinuation of Plasma Exchange After a Third Kidney Transplant for Atypical Hemolytic Uremic Syndrome Associated With a CFH Mutation
Maintenance of Kidney Function Following Treatment With Eculizumab and Discontinuation of Plasma Exchange After a Third Kidney Transplant for Atypical Hemolytic Uremic Syndrome Associated With a CFH Mutation
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DOI：
10.1053/j.ajkd.2009.08.011
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发表时间：
2010-04-01
影响因子：
13.2
通讯作者：
Goodship, Tim
中科院分区：
文献类型：
--
作者：
Davin, Jean-Claude;Gracchi, Valentina;Goodship, Tim
非典型溶血性尿毒综合征（阿胡斯）患者的肾移植与疾病复发相关，尤其是已知有H因子突变的患者。长期的预防性血浆置换和肝肾联合移植可以预防复发引起的移植物丢失。然而，与肝移植相关的死亡率是不可忽略的，预防性血浆置换需要永久性血管通路和定期住院，并使患者暴露于对血浆的潜在过敏反应。依库珠单抗是一种高亲和力的人源化单克隆抗体，可与C5结合，从而防止C5 a和膜攻击复合物的产生。我们报告了一例17岁的阿胡斯患者，该患者与补体因子H（CFH; c.3572C > T，Ser 1191 Leu）基因突变相关，高度依赖血浆置换。由于第三次肾移植后对血浆的严重过敏反应，引入依库珠单抗代替血浆置换，没有问题。该报告和其他报告表明，补体抑制剂在阿胡斯管理中的前景即将实现。美国肾脏病杂志55：708-711。(C)2010年，美国国家肾脏基金会（National Kidney Foundation，Inc.）
Kidney transplant in patients with atypical hemolytic uremic syndrome (aHUS) is associated with a poor outcome because of recurrent disease, especially in patients known to have a factor H mutation. Long-term prophylactic plasma exchange and combined liver-kidney transplant have prevented graft loss caused by recurrence. However, the mortality associated with liver transplant is not negligible, and prophylactic plasma exchange requires permanent vascular access and regular hospitalization and exposes the patient to potential allergic reactions to plasma. Eculizumab is a high-affinity humanized monoclonal antibody that binds to C5 and thus prevents generation of C5a and the membrane attack complex. We report the case of a 17-year-old girl with aHUS associated with a mutation in the gene for complement factor H (CFH; c.3572C > T, Ser1191Leu) who was highly dependent on plasma exchange. Because of severe allergic reactions to plasma after the third renal graft, eculizumab was introduced in place of plasma exchange without problems. This and other reports suggest that the promise of complement inhibitors in the management of aHUS is going to be fulfilled. Am J Kidney Dis 55:708-711. (C) 2010 by the National Kidney Foundation, Inc.