{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T04:40:43Z","timestamp":1780375243356,"version":"3.54.1"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,12,4]],"date-time":"2024-12-04T00:00:00Z","timestamp":1733270400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Paediatrics Child Health"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>The aim of this study was to describe long\u2010term outcomes of kidney replacement therapy (KRT) in Australians with prune belly syndrome in comparison to a control group of congenital kidney disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We identified all Australians treated with KRT between 1977 and 2021 with a diagnosis of PBS from the Australia and New Zealand Dialysis and Transplant Registry.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified 37 males (no females) who commenced KRT at a median age of 17\u2009years (range 1\u201345). At initiation of KRT treatment, 54% of patients were on haemodialysis, 30% on peritoneal dialysis and 16% received a pre\u2010emptive kidney transplant. Forty\u2010eight kidney transplants (35 first, 11\u2009second and 2 third grafts) occurred, of which 48% were from deceased donors. Median age at first transplant was 21\u2009years (range 2\u201347). Graft survival at 1, 5 and 10\u2009years for first grafts was 91%, 71% and 51%, respectively (range 6\u2009days to 36\u2009years). Three men reported parenthood at median age 35\u2009years. There were 10 deaths reported at a median age of 37\u2009years (range 17\u201349). Reported aetiology was cardiac death (50%), malignancy (20%), dialysis cessation (10%) and uncertain cause (20%). Compared to an age and gender\u2010matched control group of people with congenital kidney dysplasia, Australians with PBS had equivalent peritoneal dialysis technique survival, but slightly better transplant graft and overall survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Prune belly syndrome has marked variation in outcomes from KRT, but overall, these were equivalent or better than a matched control group with congenital kidney disease, including use of peritoneal dialysis (despite lack of abdominal wall musculature).<\/jats:p><\/jats:sec>","DOI":"10.1111\/jpc.16735","type":"journal-article","created":{"date-parts":[[2024,12,4]],"date-time":"2024-12-04T07:54:19Z","timestamp":1733298859000},"page":"191-195","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Long\u2010term outcomes of kidney replacement therapy in Australians with prune belly syndrome"],"prefix":"10.1111","volume":"61","author":[{"given":"Isabella","family":"Curran","sequence":"first","affiliation":[{"name":"School of Medicine University of Tasmania  Hobart Tasmania Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5121-6245","authenticated-orcid":false,"given":"Erin","family":"Jose","sequence":"additional","affiliation":[{"name":"Endocrinology and Diabetes Service Women's and Children's Hospital  Adelaide South Australia Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Burgess","sequence":"additional","affiliation":[{"name":"School of Medicine University of Tasmania  Hobart Tasmania Australia"},{"name":"Renal Unit Royal Hobart Hospital  Hobart Tasmania Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Cuthbertson","sequence":"additional","affiliation":[{"name":"School of Medicine University of Tasmania  Hobart Tasmania Australia"},{"name":"Department of Nephrology Royal Melbourne Hospital  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jenni","family":"English","sequence":"additional","affiliation":[{"name":"Renal Unit Royal Hobart Hospital  Hobart Tasmania Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew D","family":"Jose","sequence":"additional","affiliation":[{"name":"School of Medicine University of Tasmania  Hobart Tasmania Australia"},{"name":"Renal Unit Royal Hobart Hospital  Hobart Tasmania Australia"},{"name":"Menzies Institute for Medical Research University of Tasmania  Hobart Tasmania 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