Related Experiment Video
Updated: May 1, 2026
Murine Renal Transplantation Procedure
Published on: July 10, 2009
RUBACE profile of pediatric patients with prune belly syndrome undergoing renal transplantation: Findings from a case
Priscila Cardoso Braz Ascar1, Marcelo Costamilan Rombaldi2, Camila Penteado Genzani2
1Department of Pediatric Kidney Transplantation, Hospital Samaritano Higienópolis de São Paulo, Rua Conselheiro Brotero, 1486 - Higienópolis, São Paulo, SP, CEP: 01232-010, Brazil; Department of Pediatrics, Federal University of São Paulo, UNIFESP, Rua Botucatu, 740 - Vila Clementino, São Paulo, SP, CEP: 04023-062, Brazil.
Insights
Prune Belly Syndrome patients undergoing renal transplantation have higher RUBACE scores associated with mortality. The RUBACE score may help identify high-risk pediatric patients for better outcomes.
Area of Science:
- Pediatric Nephrology
- Transplantation Immunology
- Medical Genetics
Background:
- Prune Belly Syndrome (PBS) involves complex urological and multisystemic issues.
- Severe PBS manifestations can necessitate renal transplantation.
- The RUBACE score comprehensively categorizes PBS multisystem involvement.
Purpose of the Study:
- To investigate the association between the RUBACE score and renal transplantation outcomes in pediatric PBS patients.
- To determine if higher RUBACE scores correlate with increased graft loss or mortality.
Main Methods:
- Retrospective review of 22 pediatric renal transplantations for PBS (2008-2021).
- RUBACE scores assigned pre-transplant based on clinical assessment.
- Statistical analysis using R 4.4.3 with permutation tests (100,000 simulations).
Main Results:
- 50% of patients experienced febrile urinary tract infections; 14% had graft loss; 9% died.
- Higher RUBACE scores were observed in patients who died (p=0.03).
- A trend of higher RUBACE scores in graft loss patients was noted but not statistically significant (p=0.16).
Conclusions:
- Multisystemic manifestations in PBS significantly impact quality of life and survival.
- The RUBACE score shows potential for risk stratification in pediatric renal transplant recipients with PBS.
- Multidisciplinary care is crucial for managing PBS complications and optimizing transplant outcomes.
Introduction:
Prune Belly Syndrome extends beyond the classical triad of deficient abdominal wall musculature, urinary tract anomalies, and cryptorchidism. Urological abnormalities may be severe enough to require renal transplantation during their lifetime. The RUBACE score is currently the most comprehensive tool for categorising the multisystem manifestations of the syndrome. We hypothesized that higher RUBACE scores would be associated with poorer post renal transplantation outcomes.
Patients And Methods:
We conducted a retrospective review of 500 paediatric renal transplantations performed between April 2008 and October 2021, identifying all patients with Prune Belly Syndrome undergoing their first transplant. Demographic variables were collected, and RUBACE scores were assigned based on clinical assessment and physical examination prior to renal transplantation. Descriptive analyses were performed using the software R 4.4.3. Qualitative variables were expressed as percentages, and numeric variables as medians and interquartile (IQR 25-75). Associations between RUBACE scores graft loss or mortality were evaluated using a permutation test with 100.000 simulations, adopting a level of significance of 5%.
Results:
In the period of the study, 22 patients with Prune Belly Syndrome underwent their first renal transplantation. During follow-up, 11 patients (50%) experienced at least one febrile urinary tract infection, 3 (14%) had graft loss, and 2 (9%) died. Those with graft loss had higher RUBACE scores than those without, although this difference was not statistically significant (p = 0.16). RUBACE scores were higher among patients who died (p = 0.03).
Discussion:
Intestinal, osteoarticular, neurologic, pulmonary and cardiac manifestations impact the quality of life and survival of patients with Prune Belly Syndrome and should be considered in multidisciplinary care when preparing these patients for renal transplantation. Many of the patients in the present study needed procedures to allow adequate bladder emptying and to reduce complications such as recurrent urinary tract infections. Until now, no previous study has evaluated RUBACE score with renal outcomes in paediatric Prune Belly patients that received renal transplantation.
Conclusions:
Renal transplantation in children with Prune Belly Syndrome presents unique clinical challenges. RUBACE score may assist in risk stratification, helping to identify patients at higher risk of graft loss and mortality after transplantation.
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