Background

Children with prune belly syndrome (PBS) are at higher risk of developing kidney dysfunction and requiring kidney replacement therapy (KRT). While studies have described surgical and survival outcomes in these populations, there has yet to be a focused synthesis of evidence regarding kidney outcomes in this population. Here, the focus of this scoping review was to highlight knowledge gaps and report standards on kidney outcomes in PBS of all ages.

Methods

Following scoping review methodology, EMBASE, MEDLINE, and Scopus were searched for peer-reviewed literature that describe kidney outcomes in PBS. All studies with a broad set of kidney outcomes (such as kidney function measures, chronic kidney disease (CKD), KRT and associated outcomes) were included. Findings were summarized and qualitatively synthesized.

Results

Of the 436 unique records identified, 25 were included for synthesis. A total of 17 studies (441 patients) reported on kidney insufficiency outcomes, with an estimated prevalence of CKD ranging from 8 to 66%. A total of 15 studies (314 patients) described KRT, primary kidney transplant, and outcomes. Of these, the age for KRT ranged from 4 to 21 years, and graft survival ranged from 22 to 87% by last follow-up (range 1.3–27 years).

Conclusions

There is significant variability in studies reporting kidney outcomes in PBS which limits meaningful synthesis. There is a need for future studies with comprehensive reporting of confounders and drivers for kidney insufficiency in PBS.

中文翻译：

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梅腹综合征的肾功能和移植：范围审查

背景

患有梅干腹综合征 (PBS) 的儿童出现肾功能障碍并需要肾脏替代治疗 (KRT) 的风险较高。虽然研究描述了这些人群的手术和生存结果，但尚未对有关该人群肾脏结果的证据进行集中综合。在这里，本次范围审查的重点是强调知识差距并报告所有年龄段 PBS 肾脏结果的标准。

方法

按照范围审查方法，在 EMBASE、MEDLINE 和 Scopus 中检索了描述 PBS 中肾脏结果的同行评审文献。所有具有广泛肾脏结局（例如肾功能测量、慢性肾脏病 (CKD)、KRT 和相关结局）的研究均包含在内。对调查结果进行了总结和定性综合。

结果

在已识别的 436 个独特记录中，有 25 个被纳入综合。共有 17 项研究（441 名患者）报告了肾功能不全的结果，估计 CKD 患病率为 8% 至 66%。总共 15 项研究（314 名患者）描述了 KRT、原发性肾移植和结果。其中，接受 KRT 的年龄为 4 至 21 岁，到最后一次随访时移植物存活率为 22% 至 87%（范围为 1.3-27 岁）。

结论

报告 PBS 肾脏结果的研究存在显着差异，这限制了有意义的合成。未来需要进行研究，全面报告 PBS 中肾功能不全的混杂因素和驱动因素。