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右逆行性肾内手术(RIRS)后发生非典型溶血性综合征的罕见病例。

A Rare Case of Atypical Haemolytic Syndrome Following Right Retrograde Intrarenal Surgery (RIRS).

作者信息

Cretì Antonio, Pinto Francesco, Sighinolfi Maria Chiara, Rocco Bernardo Maria Cesare, Nigro Domenico, Ragonese Mauro

机构信息

Department of Urology, Policlinico Universitario Fondazione Agostino Gemelli, Istituto di Ricovero e Cura a Carattere Scientifico (IRCSS), Rome, ITA.

出版信息

Cureus. 2025 Jun 23;17(6):e86585. doi: 10.7759/cureus.86585. eCollection 2025 Jun.

Abstract

Renal failure, non-immune hemolytic anemia, and thrombocytopenia are the hallmarks of the uncommon but potentially fatal complement system-related illness known as atypical hemolytic uraemic syndrome (aHUS). We describe the case of a 46-year-old woman who underwent right retrograde intrarenal surgery (RIRS) for a 1.5 cm urinary stone in the renal pelvis and experienced septic shock on postoperative day 1 caused by aHUS. The patient received continuous venovenous hemodiafiltration (CVVHDF) for six days, eculizumab, a monoclonal antibody that blocks terminal complement activation, and three plasma exchanges with fresh frozen plasma, resulting in a progressive normalization of hemolytic parameters. Two months after the RIRS, the patient underwent percutaneous nephrolithotripsy to remove residual kidney stones. Three days before the surgery, she received a dose of eculizumab. The procedure went smoothly, and there were no postoperative complications. To the best of our knowledge, this is the report that mentions aHUS as a postoperative complication following endourology procedures. This report focuses on multidisciplinary diagnosis, treatment, and follow-up strategies for this unique complication in urology.

摘要

肾衰竭、非免疫性溶血性贫血和血小板减少症是一种罕见但可能致命的与补体系统相关疾病——非典型溶血性尿毒症综合征(aHUS)的特征。我们描述了一名46岁女性的病例,她因肾盂内1.5厘米的尿路结石接受了右逆行肾内手术(RIRS),术后第1天因aHUS发生感染性休克。患者接受了为期6天的连续性静脉-静脉血液透析滤过(CVVHDF)、阻断终末补体激活的单克隆抗体依库珠单抗以及三次新鲜冰冻血浆置换,溶血参数逐渐恢复正常。RIRS术后两个月,患者接受了经皮肾镜碎石术以清除残留肾结石。手术前三天,她接受了一剂依库珠单抗。手术过程顺利,术后无并发症。据我们所知,这是一篇提及aHUS作为腔内泌尿外科手术后并发症的报告。本报告重点关注泌尿外科这一独特并发症的多学科诊断、治疗及随访策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa3e/12285547/430a9df1896d/cureus-0017-00000086585-i01.jpg

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