Hemolytic uremic syndrome and kidney transplantation in uncontrolled donation after circulatory death (DCD): A two-case report.

Clinical Nephrology. Case Studies Pub Date : 2021-05-25 eCollection Date: 2021-01-01 DOI:10.5414/CNCS110434
Leonardo Caroti, Giuseppe Cestone, Lorenzo Di Maria, Marco Allinovi, Vicenzo Li Marzi, Sergio Serni, Calogero Lino Cirami
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引用次数: 1

Abstract

Background: Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolysis, thrombocytopenia, and renal involvement. Complement-mediated atypical HUS (aHUS) is a result of genetic defects in the alternative complement pathway components or regulators. The introduction of eculizumab has improved renal and overall survival of aHUS patients. Nowadays, given organ shortage, it is necessary to consider kidney transplantation (KT) even in protocols with a high risk of HUS recurrence, such as from donation after circulatory death (DCD) donors. Here, we describe two patients with HUS who underwent a KT from an uncontrolled DCD (uDCD).

Case summary: The first patient, affected by aHUS due to a heterozygous deletion in CFHR3-CFHR1 and a novel heterozygous variant in CFHR5 gene, underwent a KT with eculizumab prophylaxis. The patient did not experience a post-transplant aHUS recurrence. The second patient, who experienced an HUS episode characterized by a hypertensive crisis and with no underlying mutations in complement system genes, underwent a KT without eculizumab prophylaxis. At day 5, anti-complement treatment commenced due to hematological signs of thrombotic microangiopathy (TMA). After the introduction of eculizumab, we observed a stabilization of kidney function and hematological remission.

Conclusion: We present herein two different patients with HUS who both underwent successful KT from uDCD donation under the umbrella of eculizumab therapy. Taking into account the importance of increasing the number of organs available for transplantation, uDCD could represent an additional resource in this subset of HUS patients.

Abstract Image

Abstract Image

溶血性尿毒症综合征与循环性死亡(DCD)后无控制捐献肾移植:两例报告。
背景:溶血性尿毒症综合征(HUS)是一种罕见的疾病,以微血管性溶血、血小板减少和肾脏受累为特征。补体介导的非典型溶血性尿毒症(aHUS)是补体途径成分或调节因子存在遗传缺陷的结果。eculizumab的引入改善了aHUS患者的肾脏和总生存期。如今,由于器官短缺,即使在具有高复发风险的方案中,如循环性死亡(DCD)供者的捐赠,也有必要考虑肾移植(KT)。在这里,我们描述了两例溶血性尿毒综合征患者,他们从不受控制的DCD (uDCD)中接受了KT。病例总结:第一位患者因CFHR3-CFHR1基因杂合缺失和CFHR5基因新型杂合变异而感染aHUS,接受了eculizumab预防的KT治疗。该患者未经历移植后aHUS复发。第二例患者经历溶血性尿毒综合征发作,以高血压危象为特征,补体系统基因没有潜在突变,在没有eculizumab预防的情况下接受了KT。第5天,由于血栓性微血管病(TMA)的血液学体征,开始抗补体治疗。引入eculizumab后,我们观察到肾功能稳定和血液学缓解。结论:我们在此报告了两例不同的溶血性尿毒综合征患者,他们都在eculizumab治疗的保护伞下成功地从uDCD捐赠进行了KT。考虑到增加可用于移植的器官数量的重要性,uDCD可能是溶血性尿毒综合征患者这一亚群的额外资源。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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