{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T03:06:17Z","timestamp":1787972777634,"version":"build-2784847793"},"reference-count":42,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2018,9,19]],"date-time":"2018-09-19T00:00:00Z","timestamp":1537315200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 DK105068"],"award-info":[{"award-number":["R01 DK105068"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bjui-journals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["BJU International"],"published-print":{"date-parts":[[2019,1]]},"abstract":"\n Objective<\/jats:title>\n \n To design a novel system of scoring prune belly syndrome (\n PBS<\/jats:styled-content>\n ) phenotypic severity at any presenting age and apply it to a large pilot cohort.\n <\/jats:p>\n <\/jats:sec>\n \n Patients and Methods<\/jats:title>\n \n From 2000 to 2017, patients with\n PBS<\/jats:styled-content>\n were recruited to our prospective\n PBS<\/jats:styled-content>\n study and medical records were cross\u2010sectionally analysed, generating individualised\n RUBACE<\/jats:styled-content>\n scores. We designed the pragmatic\n RUBACE<\/jats:styled-content>\n \u2010scoring system based on six sub\u2010scores (R: renal, U: ureter, B: bladder\/outlet, A: abdominal wall, C: cryptorchidism, E: extra\u2010genitourinary, generating the acronym\n RUBACE<\/jats:styled-content>\n ), yielding a potential summed score of 0\u201331. The \u2018E\u2019 score was used to segregate syndromic\n PBS<\/jats:styled-content>\n and\n PBS<\/jats:styled-content>\n \u2010plus variants. The cohort was scored per classic Woodard criteria and\n RUBACE<\/jats:styled-content>\n scores compared to Woodard category.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n In all, 48 males and two females had a mean (range)\n RUBACE<\/jats:styled-content>\n score of 13.8 (8\u201325) at a mean age of 7.3\u00a0years. Segregated by phenotypic categories, there were 39 isolated\n PBS<\/jats:styled-content>\n (76%), six syndromic\n PBS<\/jats:styled-content>\n (12%) and five\n PBS<\/jats:styled-content>\n \u2010plus (10%) cases. The mean\n RUBACE<\/jats:styled-content>\n scores for Woodard categories 1, 2, and 3 were 20.5 (eight patients), 13.8 (25), and 10.6 (17), respectively (\n P\u00a0<\/jats:italic>\n <\u00a00.001).\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n RUBACE<\/jats:styled-content>\n is a practical, organ\/system level, phenotyping tool designed to grade\n PBS<\/jats:styled-content>\n severity and categorise patients into isolated\n PBS<\/jats:styled-content>\n , syndromic\n PBS<\/jats:styled-content>\n , and\n PBS<\/jats:styled-content>\n \u2010plus groups. This standardised system will facilitate genotype\u2013phenotype correlations and future prospective multicentre studies assessing medical and surgical treatment outcomes.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/bju.14524","type":"journal-article","created":{"date-parts":[[2018,8,16]],"date-time":"2018-08-16T10:15:02Z","timestamp":1534414502000},"page":"130-139","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Phenotypic severity scoring system and categorisation for prune belly syndrome: application to a pilot cohort of 50 living patients"],"prefix":"10.1111","volume":"123","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6627-9409","authenticated-orcid":false,"given":"Daniel G.","family":"Wong","sequence":"first","affiliation":[{"name":"Department of Urology University of Texas Southwestern Medical Center Dallas TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6938-7352","authenticated-orcid":false,"given":"Michelle K.","family":"Arevalo","sequence":"additional","affiliation":[{"name":"Department of Urology University of Texas Southwestern Medical Center Dallas TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niccolo Maria","family":"Passoni","sequence":"additional","affiliation":[{"name":"Department of Urology University of Texas Southwestern Medical Center Dallas TX USA"},{"name":"Pediatric Urology Children's Health Dallas Dallas TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nida S.","family":"Iqbal","sequence":"additional","affiliation":[{"name":"Department of Urology University of Texas Southwestern Medical Center Dallas TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Jascur","sequence":"additional","affiliation":[{"name":"Department of Urology University of Texas Southwestern Medical Center Dallas TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam J.","family":"Kern","sequence":"additional","affiliation":[{"name":"Department of Urology University of Texas Southwestern Medical Center Dallas TX USA"},{"name":"Pediatric Urology Children's Health Dallas Dallas TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma J.","family":"Sanchez","sequence":"additional","affiliation":[{"name":"Department of Urology University of Texas Southwestern Medical Center Dallas TX USA"},{"name":"Pediatric Urology Children's Health Dallas Dallas TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arthi","family":"Satyanarayan","sequence":"additional","affiliation":[{"name":"Department of Urology University of Texas Southwestern Medical Center Dallas TX USA"},{"name":"Pediatric Urology Children's Health Dallas Dallas TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jyothsna","family":"Gattineni","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Children's Health Dallas Dallas TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8272-4886","authenticated-orcid":false,"given":"Linda A.","family":"Baker","sequence":"additional","affiliation":[{"name":"Department of Urology University of Texas Southwestern Medical Center Dallas TX USA"},{"name":"Pediatric Urology Children's Health Dallas Dallas TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,9,19]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.urology.2009.12.072"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5347(17)38895-X"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5347(17)43880-8"},{"key":"e_1_2_9_5_1","unstructured":"North American Pediatric Renal Trials and Collaborative Studies.NAPRTCS 2014 Annual Transplant Report 2014. 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