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成人起病型微小病变性肾病:组织学慢性改变对临床表现和结局的意义。

Adult-onset minimal change disease: the significance of histological chronic changes for clinical presentation and outcome.

机构信息

Dr. Carol Davila Teaching Hospital of Nephrology, Romanian Renal Registry, Street Calea Grivitei, No. 4, 010731, Bucharest, Romania.

University of Medicine and Pharmacy Carol Davila, Bucharest, Romania.

出版信息

Clin Exp Nephrol. 2021 Mar;25(3):240-250. doi: 10.1007/s10157-020-01985-7. Epub 2020 Oct 22.

DOI:10.1007/s10157-020-01985-7
PMID:33090339
Abstract

INTRODUCTION

Data on pathologic features with prognostic utility in adults with minimal change disease (MCD) are limited. We assessed the relationship between histologic chronic changes and clinical presentation and outcomes.

METHODS

The consecutive records of 79 patients with MCD and minimum of 6 months follow-up were retrospectively reviewed. Kidney survival was the primary endpoint (doubling serum creatinine or dialysis initiation). Secondary endpoints were time to remission and relapse. Total chronicity score was the sum of glomerulosclerosis (0-3), interstitial fibrosis (0-3), tubular atrophy (0-3), and arteriolosclerosis (0/1).

RESULTS

The median renal chronicity score was 1; 77% had minimal (0-1), 18% mild (2-4), and 5% moderate (5-7) chronicity. Fifty percent had a null score; they were younger, had higher eGFR, similar proteinuria, better renal survival, and lower mortality. Mean kidney survival time was 5.7 (95% CI 5.2-6.2) years; 89% reached a form of remission at a median of 8 weeks; 31% relapsed at a mean of 26 months. Chronic changes severity predicted both relapses and kidney survival, each one-point increase in score raised with 27% the risk of relapse and with 31% the risk of dialysis initiation. Acute kidney injury (AKI) was present in 42% of the patients; they had more often mesangial proliferation, interstitial inflammation, tubular atrophy, arteriosclerosis, podocyte villous hypertrophy, and higher chronicity score.

CONCLUSION

Standardized grading of chronicity was a predictor of kidney survival and disease relapse and was related to AKI. Older patients with severe nephrotic syndrome and with increased chronicity score could represent a high-risk category.

摘要

介绍

成人微小病变性肾病(MCD)中具有预后意义的病理特征数据有限。我们评估了组织学慢性改变与临床表现和结局的关系。

方法

回顾性分析了 79 例 MCD 患者的连续记录,这些患者的随访时间至少为 6 个月。肾脏存活率是主要终点(血清肌酐加倍或开始透析)。次要终点是缓解和复发时间。总慢性评分是肾小球硬化(0-3)、间质纤维化(0-3)、肾小管萎缩(0-3)和小动脉硬化(0/1)的总和。

结果

中位数肾慢性评分 1 分;77%为轻度(0-1),18%为轻度(2-4),5%为中度(5-7)。50%为零分;他们更年轻,eGFR 更高,蛋白尿相似,肾脏存活率更好,死亡率更低。平均肾脏生存时间为 5.7 年(95%CI 5.2-6.2);89%在中位数 8 周时达到某种缓解;31%在平均 26 个月时复发。慢性改变的严重程度预测了复发和肾脏存活率,评分每增加 1 分,复发的风险增加 27%,开始透析的风险增加 31%。42%的患者存在急性肾损伤(AKI);他们更常出现系膜增殖、间质炎症、肾小管萎缩、小动脉硬化、足细胞绒毛样肥大和更高的慢性评分。

结论

慢性程度的标准化分级是肾脏存活率和疾病复发的预测因子,与 AKI 相关。伴有严重肾病综合征和慢性评分增加的老年患者可能代表高危人群。

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