{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T08:23:04Z","timestamp":1780561384229,"version":"3.54.1"},"reference-count":17,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2024,7,2]],"date-time":"2024-07-02T00:00:00Z","timestamp":1719878400000},"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":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2024,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Children with WAGR (Wilms tumor, aniridia, genitourinary anomalies, and range of development delays) syndrome are predisposed to Wilms tumor (WT) and intrinsic kidney disease. Using the comprehensive International WAGR Syndrome Association (IWSA) survey of children with WAGR syndrome, we analyzed tumor characteristics, treatment and congenital risk factors, and kidney function in children with WAGR and WT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Descriptive statistics were utilized including demographics, treatment strategies, and patient outcomes. Comparisons were made between patients with WAGR and WT to those with WAGR alone. A multivariable logistic regression was completed for risk of developing WT and to identify predictors of chronic kidney disease (CKD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixty\u2010four of 145 children with WAGR developed WT (44.1%). Three relapsed and one died. CKD developed in five children with WAGR without WT (5\/81, 6.2%), and in 34 with WAGR and WT (34\/64, 28.3%). Children with WAGR and WT were younger (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.017), and had a greater association with CKD than WAGR children without WT (<jats:italic>p<\/jats:italic>\u00a0&lt;\u00a0.0001). Two children with WT required hemodialysis, and one underwent kidney transplantation. By univariate analysis, CKD at any stage was associated with complete nephrectomy for the WT surgery (<jats:italic>p<\/jats:italic>\u00a0&lt;\u00a0.0001), chemotherapy duration greater than 12\u00a0months, and three\u2010drug therapy. Upon multivariate analysis, prior nephrectomy was the only significant variable (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.0002).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Epidemiological analysis of children with WAGR demonstrated favorable oncologic outcomes, but high rate of early CKD in those who developed WT. Further study of the use of nephron\u2010sparing surgery in children with WAGR and strategies to delay or treat early CKD are needed.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.31172","type":"journal-article","created":{"date-parts":[[2024,7,3]],"date-time":"2024-07-03T04:34:44Z","timestamp":1719981284000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Wilms tumor characteristics, surgical management, outcomes, and chronic kidney disease in children with WAGR syndrome: A report from the International WAGR Syndrome Association survey"],"prefix":"10.1002","volume":"71","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3459-2772","authenticated-orcid":false,"given":"Elisabeth T.","family":"Tracy","sequence":"first","affiliation":[{"name":"Division of Pediatric Surgery, UNC Children's Hospital University of North Carolina at Chapel Hill  Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harold","family":"Leraas","sequence":"additional","affiliation":[{"name":"Department of Surgery Duke University Medical Center  Durham North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0803-0218","authenticated-orcid":false,"given":"Lindsay","family":"Olson","sequence":"additional","affiliation":[{"name":"Department of Surgery Duke University Medical Center  Durham North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert C.","family":"Shamberger","sequence":"additional","affiliation":[{"name":"Department of Surgery Boston Children's Hospital  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6161-1815","authenticated-orcid":false,"given":"Peter F.","family":"Ehrlich","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery University of Michigan, C.S. Mott Children's Hospital  Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,7,2]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2003.06.096"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.33304"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2006.041723"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.32958"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20891"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2003.06.096"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/SLA.0000000000002356"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2021.733018"},{"key":"e_1_2_8_10_1","unstructured":"Sanford Medical.The CORDS program (Coordination of Rare Diseases at Sanford).Sanford Medical;2022. 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