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先天性肾病综合征肾移植后依赖类固醇的肾病综合征

Steroid-dependent nephrotic syndrome following renal transplantation for congenital nephrotic syndrome.

作者信息

Lane P H, Schnaper H W, Vernier R L, Bunchman T E

机构信息

Division of Pediatric Nephrology, University of Minnesota Hospitals, Minneapolis.

出版信息

Pediatr Nephrol. 1991 May;5(3):300-3. doi: 10.1007/BF00867484.

Abstract

A boy developed recurrent steroid-responsive nephrotic syndrome following renal transplantation for congenital nephrotic syndrome. The first episode was associated with mild tubulointerstitial rejection on kidney biopsy. Subsequent episodes showed normal histology by light microscopy and epithelial foot process fusion on electron microscopy, consistent with minimal change nephrotic syndrome. Serum analysis for soluble immune response suppressor was negative pre-nephrectomy, positive during each bout of nephrotic syndrome, and negative during each remission. This case represents de novo occurrence of steroid-sensitive minimal change nephrotic syndrome following renal transplantation for congenital nephrotic syndrome. We stress the need for histological examination of the renal allograft to diagnose rejection, recurrent disease, or de novo disease.

摘要

一名男孩在因先天性肾病综合征接受肾移植后出现复发性类固醇反应性肾病综合征。首次发作与肾活检显示的轻度肾小管间质排斥反应有关。随后的发作在光学显微镜下组织学正常,电子显微镜下显示上皮足突融合,符合微小病变型肾病综合征。术前血清可溶性免疫反应抑制因子分析为阴性,肾病综合征每次发作时为阳性,每次缓解期为阴性。该病例代表了先天性肾病综合征肾移植后类固醇敏感性微小病变型肾病综合征的新发情况。我们强调需要对同种异体肾移植物进行组织学检查,以诊断排斥反应、复发性疾病或新发疾病。

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