成人微小病变:巴基斯坦一个中心的临床病理特征、治疗反应和结果。

Shaheera Shakeel, Rahma Rashid, Nazarul H Jafry, Muhammed Mubarak
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引用次数: 0

摘要

背景:微小改变病(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、治疗反应和长期结果的数据很少。目的:确定巴基斯坦成年MCD患者的临床病理特征、治疗反应和中期预后。方法:这项回顾性队列研究纳入了2010年1月至2020年12月在信德省泌尿外科和移植研究所成人肾脏病诊所接受活检证实的MCD治疗的所有成年患者。数据从组织病理学档案和病例档案中的原始肾活检申请表中检索。收集和分析了人口统计学、临床表现、实验室结果、治疗方案和结果的数据。完全缓解(CR)、部分缓解(PR)、复发和类固醇抵抗根据标准标准进行定义。使用统计产品和服务解决方案Version 22进行统计分析。结果:研究队列包括23名成年人[15名(65.2%)男性,平均年龄26.34±10.28岁]。高血压7例(30.4%),显微镜下血尿10例(43.4%)。实验室结果显示平均血清肌酐为1.03±1.00 mg/dL,平均血清白蛋白为1.94±0.90 g/dL,平均24小时尿蛋白为4.53±2.43 g。平均随访时间38.09±22.3个月。类固醇治疗在16/18(88.8%)的患者中有效,10/16(62.5%)达到CR, 6/16(37.5%)达到PR。2例患者对类固醇耐药,需要二线免疫抑制治疗。4/20(19.04%)患者复发,平均首次复发时间为6.5±3.31个月。末次随访时,18/20(85.7%)患者缓解,16/20(76.1%)患者肾功能维持正常。没有患者进展为终末期肾病或死亡。结论:成人MCD对类固醇治疗有良好的反应,大多数患者获得缓解。然而,复发是常见的,在某些情况下需要二线免疫抑制治疗。该研究强调了为成人MCD制定标准化治疗指南以优化治疗结果的必要性。
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Adult minimal change disease: Clinicopathologic characteristics, treatment response and outcome at a single center in Pakistan.

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.

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Adult minimal change disease: Clinicopathologic characteristics, treatment response and outcome at a single center in Pakistan. Clinical course and outcome of adult patients with primary focal segmental glomerulosclerosis with kidney function loss on presentation. Detection of decline in estimated glomerular filtration rate in patients with type 2 diabetes by cystatin C-based equations. Hypertension and associated complications in pregnant women with chronic kidney disease. Neutrophil gelatinase-associated lipocalin, kidney injury molecule-1, and periostin: Novel urinary biomarkers in diabetic nephropathy.
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