Devresse Arnaud, de Meyer Martine, Aydin Selda, Dahan Karin, Kanaan Nada
Division of Nephrology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.
Division of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.
Case Rep Nephrol. 2018 Mar 14;2018:1727986. doi: 10.1155/2018/1727986. eCollection 2018.
De novo thrombotic microangiopathy (TMA) can occur after kidney transplantation. An abnormality of the alternative pathway of complement must be suspected and searched for, even in presence of a secondary cause. We report the case of a 23-year-old female patient who was transplanted with a kidney from her mother for end-stage renal disease secondary to Hinman syndrome. Early after transplantation, she presented with 2 episodes of severe pyelonephritis, associated with acute kidney dysfunction and biological and histological features of TMA. Investigations of the alternative pathway of the complement system revealed atypical haemolytic uremic syndrome secondary to complement factor I mutation, associated with mutations in CD46 and complement factor H related protein genes. Plasma exchanges followed by eculizumab injections allowed improvement of kidney function without, however, normalization of creatinine.
新发血栓性微血管病（TMA）可发生于肾移植术后。即使存在继发原因，也必须怀疑并查找补体替代途径的异常。我们报告了一例23岁女性患者，因欣曼综合征继发终末期肾病，接受了来自其母亲的肾脏移植。移植后早期，她出现了2次严重肾盂肾炎发作，伴有急性肾功能不全以及TMA的生物学和组织学特征。补体系统替代途径的检查发现，继发于补体因子I突变的非典型溶血尿毒综合征，与CD46和补体因子H相关蛋白基因突变有关。血浆置换后注射依库珠单抗使肾功能有所改善，但肌酐未恢复正常。
