Related Experiment Video
Updated: Jul 11, 2025
Using a Chemical Biopsy for Graft Quality Assessment
Published on: June 17, 2020
Kidney function and transplants in prune belly syndrome: a scoping review
Adree Khondker1,2,3, Ihtisham Ahmad1, Kellie Kim1
1Temerty Faculty of Medicine, University of Toronto, Toronto, ON, Canada.
Insights
Children with prune belly syndrome (PBS) face significant kidney issues, including chronic kidney disease (CKD) and kidney replacement therapy (KRT). This review highlights variability in reporting and calls for standardized data collection for better patient outcomes.
Area of Science:
- Nephrology
- Pediatric Urology
- Medical Research Synthesis
Background:
- Prune belly syndrome (PBS) significantly increases the risk of kidney dysfunction and kidney replacement therapy (KRT) in children.
- Existing research primarily focuses on surgical and survival outcomes, with limited synthesis of kidney-specific outcomes.
- A comprehensive understanding of kidney outcomes in PBS across all age groups is lacking.
Purpose of the Study:
- To conduct a scoping review of existing literature on kidney outcomes in patients with prune belly syndrome.
- To identify knowledge gaps and assess reporting standards for kidney outcomes in PBS.
- To synthesize current evidence on kidney function, chronic kidney disease (CKD), and KRT in PBS.
Main Methods:
- A systematic scoping review methodology was employed.
- Searches were conducted across EMBASE, MEDLINE, and Scopus for relevant peer-reviewed literature.
- Studies reporting on a wide range of kidney outcomes, including kidney function, CKD, and KRT, were included and qualitatively synthesized.
Main Results:
- Twenty-five studies involving 441 patients reported on kidney insufficiency, with CKD prevalence ranging from 8% to 66%.
- Fifteen studies detailed kidney replacement therapy (KRT) in 314 patients, with KRT initiation between ages 4 and 21 years.
- Kidney transplant graft survival varied widely, from 22% to 87% over follow-up periods of 1.3 to 27 years.
Conclusions:
- Significant heterogeneity exists in the reporting of kidney outcomes for PBS, hindering meaningful synthesis.
- There is a critical need for future research employing standardized reporting of confounders and etiological factors for kidney insufficiency in PBS.
- Improved data collection standards are essential for advancing the understanding and management of kidney complications in PBS.
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.
Related Concept Videos
01:28Kidney Transplant I: Introduction
01:26Kidney Transplant II: Surgical Procedure
01:16Kidney Transplant III: Nursing Management
01:28Chronic Kidney Disease III: Interprofessional Care
01:25Chronic Kidney Disease I: Introduction
01:30Acute Kidney Injury IV: Diagnostic Studies and Prevention