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成人微小病变病:巴基斯坦某单一中心的临床病理特征、治疗反应及预后

Adult minimal change disease: Clinicopathologic characteristics, treatment response and outcome at a single center in Pakistan.

作者信息

Shakeel Shaheera, Rashid Rahma, Jafry Nazarul H, Mubarak Muhammed

机构信息

Department of Histopathology, Sindh Institute of Urology and Transplantation, Karachi 74200, Sindh, Pakistan.

Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi 74200, Sindh, Pakistan.

出版信息

World J Nephrol. 2024 Dec 25;13(4):99643. doi: 10.5527/wjn.v13.i4.99643.

Abstract

BACKGROUND

Minimal change disease (MCD) is a significant cause of idiopathic nephrotic syndrome (INS) in adults, representing approximately 10%-15% of INS cases. The data is scanty on clinicopathological features, treatment responses, and long-term outcomes of MCD in adults.

AIM

To determine the clinicopathologic characteristics, treatment responses, and medium-term outcomes of adult patients with MCD in Pakistan.

METHODS

This retrospective cohort study included all adult patients with biopsy-proven MCD treated at the adult nephrology clinic, Sindh institute of urology and transplantation, between January 2010 and December 2020. The data was retrieved from the original renal biopsy request forms in the histopathology archives and the case files. Data on demographics, clinical presentation, laboratory findings, treatment regimens, and outcomes were collected and analyzed. Complete remission (CR), partial remission (PR), relapse, and steroid resistance were defined according to standard criteria. Statistical analyses were performed using statistical product and service solutions, Version 22.

RESULTS

The study cohort included 23 adults [15 (65.2% males), mean age 26.34 ± 10.28 years]. Hypertension was found in 7 (30.4%) and microscopic hematuria in 10 (43.4%) of participants. Laboratory findings revealed a mean serum creatinine of 1.03 ± 1.00 mg/dL, mean serum albumin of 1.94 ± 0.90 g/dL and mean 24-hour urinary proteins of 4.53 ± 2.43 g. The mean follow-up time was 38.09 ± 22.3 months. Treatment with steroids was effective in 16/18 (88.8%) of patients, with 10/16 (62.5%) achieving CR and 6/16 (37.5%) achieving PR. Two patients were resistant to steroids and required second-line immunosuppressive therapy. Relapse occurred in 4/20 (19.04%) of patients, with a mean time to first relapse of 6.5 ± 3.31 months. At the last follow-up, 18/20 (85.7%) of patients were in remission, and 16/20 (76.1%) maintained normal renal function. No patients progressed to end-stage renal disease or died.

CONCLUSION

MCD in adults shows a favorable response to steroid therapy, with a majority achieving remission. However, relapses are common, necessitating second-line immunosuppressive treatments in some cases. The study highlights the need for standardized treatment guidelines for adult MCD to optimize outcomes.

摘要

背景

微小病变性肾病(MCD)是成人特发性肾病综合征(INS)的重要病因,约占INS病例的10%-15%。关于成人MCD的临床病理特征、治疗反应及长期预后的数据较少。

目的

确定巴基斯坦成人MCD患者的临床病理特征、治疗反应及中期预后。

方法

这项回顾性队列研究纳入了2010年1月至2020年12月期间在信德泌尿与移植研究所成人肾脏病门诊接受活检证实为MCD的所有成年患者。数据从组织病理学档案中的原始肾活检申请表和病例档案中获取。收集并分析了人口统计学、临床表现、实验室检查结果、治疗方案及预后等数据。完全缓解(CR)、部分缓解(PR)、复发及激素抵抗根据标准标准进行定义。使用统计产品与服务解决方案22版进行统计分析。

结果

研究队列包括23名成年人[15名(65.2%为男性),平均年龄26.34±10.28岁]。7名(30.4%)患者有高血压,10名(43.4%)患者有镜下血尿。实验室检查结果显示平均血清肌酐为1.03±1.00mg/dL,平均血清白蛋白为1.94±0.90g/dL,平均24小时尿蛋白为4.53±2.43g。平均随访时间为38.09±22.3个月。16/18(88.8%)的患者使用激素治疗有效,其中10/16(62.5%)达到CR,6/16(37.5%)达到PR。2名患者对激素耐药,需要二线免疫抑制治疗。4/20(19.04%)的患者复发,首次复发的平均时间为6.5±3.31个月。在最后一次随访时,18/20(85.7%)的患者处于缓解状态,16/20(76.1%)的患者肾功能正常。无患者进展为终末期肾病或死亡。

结论

成人MCD对激素治疗反应良好,大多数患者可实现缓解。然而,复发很常见,在某些情况下需要二线免疫抑制治疗。该研究强调需要针对成人MCD制定标准化治疗指南以优化预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0995/11572659/85bc103a26b7/99643-g001.jpg

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