Department of Surgery, Division of Urology, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, Ontario, Canada.
Department of Chemical Engineering, McGill University, Montreal, Quebec, Canada.
J Pediatr Urol. 2021 Aug;17(4):556-565. doi: 10.1016/j.jpurol.2021.03.001. Epub 2021 Mar 5.
Abnormal renal development that results in lack of function or development of one of two kidneys is known as congenital solitary functioning kidney (CSFK). Two well characterized sub-categories of CFSK are unilateral renal agenesis (URA) and multicystic dysplastic kidney (MCDK). This systematic review sought to evaluate the change in renal function in children ≤18 years old with a CSFK as a result of URA or MCDK.
A literature search in MEDLINE and Embase was conducted (1946 to July 13, 2020). All relevant articles were retrieved and evaluated based on pre-selected criteria by two independent researchers. Data was then extracted from variables of interest and conflicts were resolved by a third researcher. The primary outcome was renal function, and the secondary outcomes were proteinuria and hypertension.
Forty-five studies were included, of which 49% (n = 22) were retrospective and/or 58% (n = 26) were cohort studies. A combined total of 2148 and 885 patients were diagnosed with MCDK or URA, respectively. The proportion of children with worsened renal function at follow-up was found to be 8.4% (95% CI: 5.2%-13.4%). Among the studies reporting renal function as a group mean or median at follow-up, 84% (21/25) had a GFR/CrCl above 90 (mL/min/1.73 m/ml/min). In terms of secondary outcomes, the proportion of children with proteinuria and hypertension was found to be 10.1% (95% CI: 6.9%-14.6%) and 7.4% (95% CI: 5.0%-10.9%), respectively.
The risk of developing proteinuria (10.1%), hypertension (7.4%), and/or worsened renal function (8.4%) for children with CFSK as a result of MCDK or URA is low. However, the level of evidence in the literature is weak. Further research is needed to identify the predisposing factors that may differentiate the small subset of children with CSFK at a higher risk of developing adverse renal outcomes.
导致单侧肾脏功能缺失或发育不全的异常肾脏发育被称为先天性孤立功能性肾脏（CSFK）。CSFK 的两种特征明确的亚类是单侧肾发育不全（URA）和多囊性发育不良肾（MCDK）。本系统综述旨在评估因 URA 或 MCDK 导致 CSFK 的≤18 岁儿童的肾功能变化。
对 MEDLINE 和 Embase 进行文献检索（1946 年至 2020 年 7 月 13 日）。由两位独立研究人员根据预先选定的标准检索所有相关文章并进行评估。然后从感兴趣的变量中提取数据，并由第三位研究人员解决冲突。主要结局是肾功能，次要结局是蛋白尿和高血压。
共纳入 45 项研究，其中 49%（n=22）为回顾性研究，58%（n=26）为队列研究。分别有 2148 例和 885 例患者被诊断为 MCDK 或 URA。随访时肾功能恶化的患儿比例为 8.4%（95%CI：5.2%-13.4%）。在报告随访时肾功能为组平均值或中位数的研究中，84%（21/25）的肾小球滤过率/肌酐清除率（GFR/CrCl）高于 90（mL/min/1.73 m/ml/min）。就次要结局而言，蛋白尿和高血压患儿的比例分别为 10.1%（95%CI：6.9%-14.6%）和 7.4%（95%CI：5.0%-10.9%）。
由于 MCDK 或 URA 导致 CSFK 的儿童发生蛋白尿（10.1%）、高血压（7.4%）和/或肾功能恶化（8.4%）的风险较低。然而，文献中的证据水平较弱。需要进一步的研究来确定可能使一小部分 CSFK 儿童处于更高风险的不良肾脏结局的易感因素。
