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成人肾移植受者复发性溶血尿毒综合征:当前概念与管理

Recurrent hemolytic uremic syndrome in an adult renal allograft recipient: current concepts and management.

作者信息

Agarwal A, Mauer S M, Matas A J, Nath K A

机构信息

Department of Medicine, University of Minnesota Medical School, Minneapolis, USA.

出版信息

J Am Soc Nephrol. 1995 Oct;6(4):1160-9. doi: 10.1681/ASN.V641160.

Abstract

Acute renal insufficiency in the setting of hemolysis and thrombocytopenia, a triad that constitutes adult or pediatric hemolytic uremic syndrome, can be associated with or triggered by diverse conditions such as verocytotoxin-producing Escherichia coli, viral infections, pregnancy, malignant hypertension, scleroderma, renal radiation, allograft rejection, lupus erythematosus, and assorted medications such as mitomycin C, cyclosporine, and oral contraceptives. Recurrent and de novo hemolytic uremic syndrome occur after renal transplantation. Relapses are also common and probably reflect incomplete resolution of the initial episode. The major differential diagnoses of hemolytic uremic syndrome in the renal allograft include acute vascular rejection, cyclosporine, FK506 or antilymphocyte antibody nephrotoxicity, and malignant hypertension, all of which may display overlapping clinical and histologic features with primary hemolytic uremic syndrome; in such instances, the exact diagnosis may be quite difficult. It is possible that the risk of recurrence may be reduced by proper timing of transplantation and suitable choice of immunosuppressive agents. Intensive plasmapheresis in conjunction with fresh frozen plasma and supportive management of renal failure may lessen mortality and morbidity even in recurrent hemolytic uremic syndrome after transplantation.

摘要

溶血和血小板减少背景下的急性肾功能不全,这一构成成人或儿童溶血尿毒综合征的三联征,可能与多种情况相关或由其引发,如产志贺毒素大肠杆菌、病毒感染、妊娠、恶性高血压、硬皮病、肾脏放疗、同种异体移植排斥、红斑狼疮以及多种药物,如丝裂霉素C、环孢素和口服避孕药。肾移植后会出现复发性和新发溶血尿毒综合征。复发也很常见,可能反映了初始发作未完全缓解。肾移植中溶血尿毒综合征的主要鉴别诊断包括急性血管排斥、环孢素、他克莫司(FK506)或抗淋巴细胞抗体肾毒性以及恶性高血压,所有这些在临床和组织学特征上可能与原发性溶血尿毒综合征有重叠;在这种情况下,准确诊断可能相当困难。通过合适的移植时机和免疫抑制剂的恰当选择,有可能降低复发风险。即使在移植后复发性溶血尿毒综合征中,强化血浆置换联合新鲜冰冻血浆以及肾衰竭的支持性治疗也可能降低死亡率和发病率。

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