Abstract：
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.
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.
摘要：
背景：患有修剪腹部综合征（PBS）的儿童发生肾功能障碍的风险较高，需要肾脏替代疗法（KRT）。虽然研究描述了这些人群的手术和生存结果，在这一人群中,关于肾脏结局的证据还没有集中的综合.这里,本范围审查的重点是突出知识差距,并报告所有年龄段的PBS肾脏结局标准.
方法：遵循范围审查方法，EMBASE,MEDLINE,和Scopus在PBS中检索了描述肾脏结局的同行评审文献。所有具有广泛肾脏结局的研究（例如肾功能指标，慢性肾脏病（CKD），包括KRT和相关结果）。对研究结果进行了总结和定性综合。
结果：在确定的436条唯一记录中，包括25个用于合成。共有17项研究（441例患者）报告了肾功能不全的结果，CKD的患病率估计为8％至66％。共有15项研究（314名患者）描述了KRT，初次肾移植,和结果。其中,KRT的年龄从4到21岁不等，到最后一次随访（1.3-27年），移植物生存率为22％至87％。
结论：在PBS中报告肾脏结局的研究中存在显著的差异，这限制了有意义的合成。未来的研究需要全面报告PBS中肾功能不全的混杂因素和驱动因素。
方法：遵循范围审查方法，EMBASE,MEDLINE,和Scopus在PBS中检索了描述肾脏结局的同行评审文献。所有具有广泛肾脏结局的研究（例如肾功能指标，慢性肾脏病（CKD），包括KRT和相关结果）。对研究结果进行了总结和定性综合。
结果：在确定的436条唯一记录中，包括25个用于合成。共有17项研究（441例患者）报告了肾功能不全的结果，CKD的患病率估计为8％至66％。共有15项研究（314名患者）描述了KRT，初次肾移植,和结果。其中,KRT的年龄从4到21岁不等，到最后一次随访（1.3-27年），移植物生存率为22％至87％。
结论：在PBS中报告肾脏结局的研究中存在显著的差异，这限制了有意义的合成。未来的研究需要全面报告PBS中肾功能不全的混杂因素和驱动因素。
