{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,2]],"date-time":"2025-12-02T15:21:55Z","timestamp":1764688915275},"reference-count":8,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2013,2,17]],"date-time":"2013-02-17T00:00:00Z","timestamp":1361059200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Nephrol"],"published-print":{"date-parts":[[2013,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:sec>\n            <jats:title>Background<\/jats:title>\n            <jats:p>Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder. In most cases, ADPKD similarly affects bilateral kidneys.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Case presentation<\/jats:title>\n            <jats:p>Among the 605 ADPKD patients that were followed up by our center, we identified two male patients with unilateral ADPKD. The cases were remarkable because the patients also had ectopia and multicystic dysplasia in the contralateral kidney, which are generally sporadic disease conditions. Both patients tested positive for polycystic kidney disease 1 mutation, but negative for hepatocyte nuclear factor 1 beta mutation. Moreover, the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings. Both patients had started a long-term hemodialysis in their 40s.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusion<\/jats:title>\n            <jats:p>Anatomical and genetic abnormality in patients with ADPKD may be more frequent and complex than previously believed. The compensatory capacity in patients with ADPKD is fragile, and missing one kidney could accelerate the deterioration of renal function.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1186\/1471-2369-14-38","type":"journal-article","created":{"date-parts":[[2013,2,18]],"date-time":"2013-02-18T19:23:06Z","timestamp":1361215386000},"update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Autosomal dominant polycystic kidney disease with ectopic unilateral multicystic dysplastic kidney"],"prefix":"10.1186","volume":"14","author":[{"given":"Jing","family":"Xu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dong-Ping","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhi-Guo","family":"Mao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"He-Feng","family":"Huang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chen-Ming","family":"Xu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cong-Rong","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wei-Ping","family":"Jia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chang-Lin","family":"Mei","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2013,2,17]]},"reference":[{"key":"479_CR1","doi-asserted-by":"publisher","first-page":"164","DOI":"10.1053\/j.ackd.2009.12.006","volume":"17","author":"A Alam","year":"2010","unstructured":"Alam A, Perrone RD: Management of ESRD in patients with autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis. 2010, 17: 164-172. 10.1053\/j.ackd.2009.12.006.","journal-title":"Adv Chronic Kidney Dis"},{"key":"479_CR2","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1053\/j.ajkd.2009.03.020","volume":"54","author":"D Poster","year":"2009","unstructured":"Poster D, Kistler AD, Krauer F, Blumenfeld JD, Rennert H, Weishaupt D, W\u00fcthrich RP, Serra AL: Kidney function and volume progression in unilateral autosomal dominant polycystic kidney disease with contralateral renal agenesis or hypoplasia: a case series. 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Pediatr Nephrol. 2010, 25: 1073-1079. 10.1007\/s00467-010-1454-9.","journal-title":"Pediatr Nephrol"},{"key":"479_CR7","doi-asserted-by":"publisher","first-page":"2864","DOI":"10.1681\/ASN.2006030277","volume":"17","author":"S Weber","year":"2006","unstructured":"Weber S, Moriniere V, Kn\u00fcppel T, Charbit M, Dusek J, Ghiggeri GM, Jankauskien\u00e9 A, Mir S, Montini G, Peco-Antic A, W\u00fchl E, Zurowska AM, Mehls O, Antignac C, Schaefer F, Salomon R: Prevalence of mutations in renal developmental genes in children with renal hypodysplasia: results of the ESCAPE study. J Am Soc Nephrol. 2006, 17: 2864-2870. 10.1681\/ASN.2006030277.","journal-title":"J Am Soc Nephrol"},{"key":"479_CR8","doi-asserted-by":"publisher","first-page":"233","DOI":"10.1007\/s00467-008-0828-8","volume":"24","author":"DS Hains","year":"2009","unstructured":"Hains DS, Bates CM, Ingraham S, Schwaderer AL: Management and etiology of the unilateral multicystic dysplastic kidney: a review. 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