Department of Pediatrics, Jinling Hospital, Nanjing Medical University, Nanjing, China.
Department of Pediatrics, Jinling Hospital, The First School of Clinical Medicine, Southern Medical University, Nanjing, China.
J Nephrol. 2021 Apr;34(2):589-596. doi: 10.1007/s40620-020-00816-y. Epub 2020 Aug 8.
Primary membranous nephropathy (PMN) is a rare pathological finding in paediatric patients. Data on PMN in children have been restricted to studies with small samples and fairly short follow-up periods. Therefore, we conducted this single-centre study to evaluate the long-term renal survival and related risk factors for PMN in children, and the clinical and histological characteristics were also described.
Two hundred and seventeen children with PMN were enrolled from July 2008 to September 2017. Patients with follow-up durations < 12 months were excluded, except for patients who progressed to end-stage kidney disease (ESKD) or experienced a related death within 12 months. Long-term renal survival and related risk factors were analysed.
The sex ratio was 1.33:1 (male vs female), and the median age was 15.0 (14.0-17.0) years old. The most prominent clinical manifestation was nephrotic syndrome (130 59.9%), which was accompanied by various degrees of oedema (142 65.4%), hyperlipidaemia (151 69.6%), hypoalbuminemia (130 59.9%), and nephrotic proteinuria (135 62.2%). Hypertension occurred in 36.4% of children with PMN. After a median follow-up of 45.0 (23.5-74.0) months, 11 patients (5.1%) developed ESKD, and the cumulative kidney survival rates of ESKD at 5 and 10 years after renal biopsy were 95.3% and 67.8%, respectively. The cumulative kidney survival rates of the combined event of ESKD and/or 30% decline in estimated glomerular filtration rate (eGFR) at 5 and 10 years after renal biopsy were 92.6% and 59.5%, respectively. Cox multivariate regression and Kaplan-Meier analysis demonstrated that hypertension and proteinuria ≥ 50 mg/kg/day were associated with renal outcome.
In this study, the 5-year and 10-year cumulative renal survival rates of ESKD in children with PMN were reported for the first time as 95.3% and 67.8%, respectively. In addition, this is the first report to find that hypertension and proteinuria ≥ 50 mg/kg/day are associated with renal outcome in children with PMN.
原发性膜性肾病（PMN）在儿科患者中是一种罕见的病理发现。PMN 患儿的数据仅限于样本量较小且随访时间相当短的研究。因此，我们进行了这项单中心研究，以评估儿童 PMN 的长期肾脏存活率和相关风险因素，并描述了临床和组织学特征。
我们从 2008 年 7 月至 2017 年 9 月招募了 217 名 PMN 患儿。排除随访时间<12 个月的患者，但在 12 个月内进展为终末期肾病（ESKD）或发生相关死亡的患者除外。分析长期肾脏存活率和相关风险因素。
男女比例为 1.33:1（男/女），中位年龄为 15.0（14.0-17.0）岁。最突出的临床表现为肾病综合征（130 例，59.9%），伴有不同程度的水肿（142 例，65.4%）、高脂血症（151 例，69.6%）、低白蛋白血症（130 例，59.9%）和肾病蛋白尿（135 例，62.2%）。PMN 患儿中 36.4%发生高血压。在中位随访 45.0（23.5-74.0）个月后，11 例（5.1%）患者发生 ESKD，肾活检后 5 年和 10 年的 ESKD 累积肾脏存活率分别为 95.3%和 67.8%。肾活检后 5 年和 10 年时，ESKD 和/或估算肾小球滤过率（eGFR）下降 30%的复合事件的累积肾脏存活率分别为 92.6%和 59.5%。Cox 多变量回归和 Kaplan-Meier 分析表明，高血压和蛋白尿≥50mg/kg/天与肾脏结局相关。
在这项研究中，我们首次报道了儿童 PMN 的 5 年和 10 年累积 ESKD 肾脏存活率分别为 95.3%和 67.8%。此外，这是首次报道发现高血压和蛋白尿≥50mg/kg/天与儿童 PMN 的肾脏结局相关。
