Jafry Nazarul Hassan, Sarwar Sarfraz, Waqar Tajammul, Mubarak Muhammed
Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi 74200, Sindh, Pakistan.
Department of Histopathology, Sindh Institute of Urology and Transplantation, Karachi 74200, Sindh, Pakistan.
World J Nephrol. 2024 Dec 25;13(4):98932. doi: 10.5527/wjn.v13.i4.98932.
Kidney function loss or renal insufficiency indicated by elevated creatinine levels and/or an estimated glomerular filtration rate (eGFR) < 60 mL/minute/1.73 m² at presentation in patients with primary focal segmental glomerulosclerosis (FSGS) is commonly seen as a poor prognostic marker for kidney survival. However, a pre>vious study from our center suggested this may be due to hemodynamic factors.
To observe the clinical and biochemical parameters, treatment response, kidney survival, and overall outcomes of adult patients with primary FSGS presenting with kidney function insufficiency.
This retrospective observational study was conducted at the Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan, from January 1995 to December 2017. During this period, 401 biopsy-proven primary FSGS patients were identified, of which 98 (24.4%) presented with kidney function loss or renal insufficiency defined as eGFR < 60 mL/minute/1.73 m² at presentation and were studied in detail.
Among the 98 patients with renal function loss on presentation, the mean age was 30.9 years ± 13.6 years with a male-to-female ratio of 2.5:1. The mean serum creatinine level was 2.2 mg/dL ± 1.3 mg/dL and mean eGFR 37.1 mL/minute/1.73 m ± 12.8 mL/minute/1.73 m. The mean 24-hour urinary protein excretion was 5.9 g/day ± 4.0 g/day, and the mean serum albumin was 2.1 g/dL ± 1.0 g/dL (median: 1.5 g/dL). The mean systolic blood pressure (BP) was 132.7 mmHg ± 19.8 mmHg, and the mean diastolic BP was 87.4 mmHg ± 12.7 mmHg. Steroid treatment was given to 81 (82.6%) of 98 patients for an average duration of 19.9 weeks ± 14.4 weeks, with a mean total steroid dose of 4.4 g ± 1.5 g. Treatment response showed that 20 (24.6%) patients achieved complete remission, 9 (11.1%) achieved partial remission, and 52 (64.1%) did not respond. The baseline eGFR was significantly lower in the non-responsive group ( = 0.006). The distribution of FSGS variants was also significantly different among steroid-responsive and non-responsive groups ( = 0.012).
Renal function loss in FSGS patients at presentation does not necessarily indicate irreversible kidney function loss and a significant number of patients respond to appropriate treatment of the underlying disease.
在原发性局灶节段性肾小球硬化（FSGS）患者中，肌酐水平升高和/或估算肾小球滤过率（eGFR）<60毫升/分钟/1.73平方米所提示的肾功能丧失或肾功能不全，通常被视为肾脏存活的不良预后指标。然而，我们中心之前的一项研究表明，这可能是由于血流动力学因素所致。
观察出现肾功能不全的原发性FSGS成年患者的临床和生化参数、治疗反应、肾脏存活情况及总体结局。
这项回顾性观察性研究于1995年1月至2017年12月在巴基斯坦卡拉奇信德泌尿与移植研究所肾脏病科进行。在此期间，共确定了401例经活检证实的原发性FSGS患者，其中98例（24.4%）出现肾功能丧失或肾功能不全，定义为就诊时eGFR<60毫升/分钟/1.73平方米，并对其进行了详细研究。
在98例就诊时出现肾功能丧失的患者中，平均年龄为30.9岁±13.6岁，男女比例为2.5:1。平均血清肌酐水平为2.2毫克/分升±1.3毫克/分升，平均eGFR为37.1毫升/分钟/1.73平方米±12.8毫升/分钟/1.73平方米。平均24小时尿蛋白排泄量为5.9克/天±4.0克/天，平均血清白蛋白为2.1克/分升±1.0克/分升（中位数：1.5克/分升）。平均收缩压（BP）为132.7毫米汞柱±19.8毫米汞柱，平均舒张压为87.4毫米汞柱±12.7毫米汞柱。98例患者中有81例（82.6%）接受了类固醇治疗，平均疗程为19.9周±14.4周，平均总类固醇剂量为4.4克±1.5克。治疗反应显示，20例（24.6%）患者实现完全缓解，9例（11.1%）实现部分缓解，52例（64.1%）无反应。无反应组的基线eGFR显著更低（P = 0.006）。FSGS变异型的分布在类固醇反应组和无反应组之间也有显著差异（P = 0.012）。
FSGS患者就诊时的肾功能丧失不一定表明肾功能不可逆转丧失，相当一部分患者对基础疾病的适当治疗有反应。
