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肾脏病与透析肾移植杂志 ›› 2025, Vol. 34 ›› Issue (2): 107-113.DOI: 10.3969/j.issn.1006-298X.2025.02.002

• 论著 • 上一篇    下一篇

移植肾C3肾小球肾炎的临床病理及预后特征

  

  • 出版日期:2025-04-28 发布日期:2025-05-07

Clinicopathological and prognostic features of C3 glomerulonephritis in renal allograft

  • Online:2025-04-28 Published:2025-05-07

摘要: 目的:分析移植肾C3肾小球肾炎(C3GN)患者的临床病理特征及预后。
方法:回顾性分析国家肾脏疾病临床医学研究中心2008年1月至2023年12月行移植肾活检并诊断为移植肾C3GN患者的临床病理及随访预后资料。
结果:共纳入移植肾C3GN患者8例。肾活检时距肾移植手术中位时间32(7, 135)月,年龄为39±7岁,尿蛋白定量3.89±2.10 g/24h,5例患者伴镜下血尿,血清肌酐162.66±70.72 μmol/L。5例患者检测了血清补体C3,且均为低补体C3血症。1例患者有C3GN家族史,1例患者行基因检测,提示具有补体I因子复合杂合突变。移植肾活检光镜表现为肾小球系膜增生性、膜增生性或毛细血管内增生性病变等;免疫荧光示C3沉积为主,伴或不伴极少量免疫球蛋白沉积。电镜示肾小球系膜区及毛细血管袢内皮下见电子致密物沉积。肾活检至随访中位时间为66(3, 118)月,3例患者出现移植肾失功。
结论:移植肾C3GN临床表现为低补体C3血症及慢性肾炎综合征,移植肾活检是确诊的重要手段,其预后欠佳。


关键词: 肾移植, 移植病理, C3肾小球肾炎

Abstract: Objective:To analyze the clinicopathological and prognostic characteristics of C3 glomerulonephritis (C3GN) in renal allograft.
Methodology:A retrospective analysis was conducted of clinical and pathological data from patients diagnosed with transplant kidney C3GN through renal biopsy at the National Clinical Research Center for Kidney Diseases between January 2008 and December 2023.
Results:A total of 8 patients with transplant kidney C3GN were included. The median time from kidney transplant to biopsy was 32(7, 135)months. At biopsy, the mean age was 39 ± 7 years, and mean urine protein quantification was 3.89 ± 2.10 g/24h. Microscopic hematuria was observed in 5 cases, and the average serum creatinine was 162.66±70.72 μmol/L. Serum complement 3 was measured in 5 patients, all showing hypocomplementemia. One patient had a family history of C3GN. One patient underwent genetic testing, which revealed a heterozygous mutation in complement factor I. The light microscopic presentation of the transplanted kidney biopsies shows mesangial proliferative, membranoproliferative, or endocapillary proliferative lesions. Immunofluorescence showed predominantly C3 deposition with or without minimal immunoglobulin deposition. Electron microscopy revealed dense deposits in the mesangial area and subendothelial space of the glomerular capillary loops. The median time from biopsy to follow-up was 66 (3, 118)months, and three patients developed graft renal failure.
Conclusion:Transplant kidney C3GN clinically presents with persistent hypocomplementemic C3 and nephritic syndrome. Transplant kidney biopsy is a crucial tool to confirm the diagnosis, and the prognosis is generally poor.


Key words: kidney transplantation, transplantation pathology, C3 glomerulonephritis