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儿童肾移植后新发膜性肾病

Posttransplant de novo membranous nephropathy in childhood.

作者信息

Sebire N J, Bockenhauer D

机构信息

Department of Paediatric Histopathology, Great Ormond Street Hospital, London, United Kingdom.

出版信息

Fetal Pediatr Pathol. 2005 Mar-Apr;24(2):95-103. doi: 10.1080/15227950591008358.

Abstract

Posttransplant proteinuria is a recognized, but relatively uncommon, presentation of renal transplant dysfunction. Significant proteinuria occurs in around 10-15% of renal transplant recipients. We present a case of de novo posttransplant membranous nephropathy in childhood complicating renal transplantation for severe congenital obstructive uropathy and review the pathology, pathogenesis, and clinical implications of this condition. In the majority of cases, the cause of posttransplant proteinuria is either related to chronic allograft nephropathy or recurrence of the glomerulonephritis for which transplantation was indicated. In a minority, however, de novo posttransplant membranous nephropathy (DNPMN) is identified on biopsy. The histopathological findings in some cases may either be similar to those of classical membranous nephropathy, or may be more subtle, showing focal segmental variation in severity, often in conjuction with the features of chronic allograft nephropathy. The use of ancillary techniques including immunohistochemistry and electron microscopy may be required to confirm the diagnosis. The presence of posttransplant de novo membranous nephropathy may be associated with an increased risk of graft loss.

摘要

移植后蛋白尿是肾移植功能障碍一种已被认识但相对不常见的表现。约10% - 15%的肾移植受者会出现显著蛋白尿。我们报告一例儿童期肾移植后新发膜性肾病,该患儿因严重先天性梗阻性尿路病接受肾移植,并对这种疾病的病理、发病机制及临床意义进行综述。在大多数情况下,移植后蛋白尿的原因要么与慢性移植肾肾病有关,要么与移植所针对的肾小球肾炎复发有关。然而,少数情况下,活检可发现移植后新发膜性肾病(DNPMN)。某些病例的组织病理学表现可能与经典膜性肾病相似,也可能更不典型,表现为严重程度的局灶节段性差异,常与慢性移植肾肾病的特征同时存在。可能需要使用包括免疫组织化学和电子显微镜在内的辅助技术来确诊。移植后新发膜性肾病的存在可能与移植肾丢失风险增加有关。

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