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Development of renal failure in children with the prune belly syndrome
Y Reinberg1, J C Manivel, G Pettinato
1Department of Laboratory Medicine, University of Minnesota Hospital and Clinics, Minneapolis.
Insights
Prune belly syndrome patients often experience renal failure. Perinatal cases stem from renal dysplasia, while later-life failure is due to pyelonephritis and obstruction, highlighting the need for careful urinary tract management.
Area of Science:
- Pediatric Nephrology
- Congenital Abnormalities
Background:
- Prune belly syndrome (PBS) is a rare congenital disorder.
- It affects males predominantly and is characterized by abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism.
- Renal involvement is a major determinant of morbidity and mortality in PBS.
Purpose of the Study:
- To evaluate the causes of renal failure in patients with prune belly syndrome.
- To differentiate between perinatal and later-onset renal failure etiologies.
- To inform management strategies for improving renal outcomes in PBS patients.
Main Methods:
- Retrospective review of 32 patients with prune belly syndrome diagnosed between 1970 and 1987.
- Analysis of autopsy findings, renal biopsies, and clinical data.
- Correlation of renal pathology with patient age and clinical course.
Main Results:
- 11 of 32 patients died in infancy; 6 had severe renal dysplasia.
- In surviving patients, renal insufficiency developed, often requiring nephrectomy or biopsy.
- Renal dysplasia was present in most specimens, but pyelonephritis and obstruction were the primary causes of renal failure in older patients.
Conclusions:
- Perinatal renal failure in prune belly syndrome is primarily due to severe renal dysplasia.
- In surviving patients, renal failure is mainly caused by secondary factors like pyelonephritis and urinary tract obstruction.
- Aggressive management of urinary tract infections, reflux, and obstruction is crucial for preserving renal function in prune belly syndrome survivors.
Abstract:
From 1970 to 1987, 32 male and female patients with the prune belly syndrome were evaluated. Of these patients 11 died as neonates or infants, and autopsy in 9 revealed 6 cases of diffuse and severe renal dysplasia. In the 10 surviving patients renal insufficiency developed and a total of 13 nephrectomies and renal biopsies were performed. Renal dysplasia was noted in 9 specimens, but unlike the infant kidneys, the dysplastic changes involved less than 25% of the parenchyma in most cases. Renal failure in these 10 patients was caused by pyelonephritis and obstruction. In our estimation perinatal renal failure in patients with the prune belly syndrome results from renal dysplasia caused by an in utero insult, while in older patients pyelonephritis and obstruction are the causes. Careful treatment of reflux, obstruction and urinary tract infections may decrease the incidence of renal failure in patients with the prune belly syndrome who survive infancy.