Abstract: FR-PO1088
The Long-Term Kidney Outcomes of Prune Belly Syndrome in Australia
Session Information
- Pediatric Hypertension, AKI, Urologic Disorders
November 08, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pediatric Nephrology
- 1700 Pediatric Nephrology
Authors
- Jose, Erin, Royal Adelaide Hospital, Adelaide, South Australia, Australia
- Skeat, Lee, Royal Hobart Hospital, Hobart, Tasmania, Australia
- English, Jennifer C., Royal Hobart Hospital, Hobart, Tasmania, Australia
- Jeffs, Lisa S., Royal Hobart Hospital, Hobart, Tasmania, Australia
- Jose, Matthew D., University of Tasmania, Hobart, Tasmania, Australia
Background
Prune Belly Syndrome (PBS) a rare congenital disorder consisting of the triad of: absence or incomplete abdominal wall muscle development, bilateral cryptorchidism and urinary tract anomalies including hydronephrosis, kidney dysplasia and dilated ureters, urethra or bladder. PBS varies considerably in clinical severity, with prognosis primarily being influenced by the degree of chronic kidney disease. The aim of this study was to describe the long-term kidney outcomes of people with Prune Belly Syndrome in Australia.
Methods
We identified all Australians treated with renal replacement therapy (RRT) who had a diagnosis of PBS (as determined by the treating unit) from the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA). ANZDATA is a clinical quality registry containing information on all people receiving dialysis or a kidney transplant since 1977.
Results
We identified 37 males (no females) with a diagnosis of PBS who received RRT in Australia and were recorded in ANZDATA. Commencement of RRT was at a median age of 17yrs (mean 19yrs, range 1-45yrs) when median creatinine was 720umol/L. RRT at first treatment was haemodialysis in 54%, peritoneal dialysis 30% and pre-emptive kidney transplant in 16%. Twenty percent of patients were late referrals to the dialysis unit (referral <3 months prior to starting dialysis). Comorbidities of diabetes, heart disease or vascular disease were not present at commencement of RRT. One man had chronic lung disease.
Forty-five kidney transplants (including 33 first, 10 second and 2 third grafts) occurred, of which 47% were from deceased donors. Mean age at first transplant was 21yrs (range 2-47yrs). Graft survival at 1, 5 & 10 years for first grafts was 94%, 67% and 48% respectively (range 6 days to 36 years). Parenthood was reported for 3 men at a median age of 35yrs.
There were 10 deaths reported at a median age of 37yrs (range 17-49yrs) due to cardiac death (50%), malignancy (20%), dialysis cessation (10%) and uncertain cause (20%).
Conclusion
Prune Belly Syndrome has marked variation in the severity of kidney disease. For those who receive RRT, kidney transplant is the predominant treatment, but peritoneal dialysis has been used successfully. Infertility is not universal. There is early cardiovascular mortality associated with this syndrome.