{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T15:22:38Z","timestamp":1772119358204,"version":"3.50.1"},"reference-count":5,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2025,11,10]],"date-time":"2025-11-10T00:00:00Z","timestamp":1762732800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,11,10]],"date-time":"2025-11-10T00:00:00Z","timestamp":1762732800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100004721","name":"The University of Tokyo","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100004721","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Nephrol"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Renal tubular dysgenesis (RTD) is a rare disorder characterized by impaired development of the proximal tubules and kidney dysfunction due to decreased fetal renal plasma flow and renin-angiotensin system (RAS) inhibition. Fetal anuria causes severe oligohydramnios and Potter sequence, and most patients die within days after birth due to refractory hypotension, anuria, and respiratory distress. While vasopressin and fludrocortisone therapy have been reported, the long-term efficacy remains unclear due to the rarity of survivors. We report a 10-year-old girl with autosomal recessive renal tubular dysgenesis (ARRTD), a genetic form of RTD caused by mutations in RAS-related genes. She initially experienced recurrent dehydration, electrolyte abnormalities, and kidney dysfunction due to polyuria but showed long-term improvement following fludrocortisone therapy.<\/jats:p>","DOI":"10.1007\/s00467-025-07051-2","type":"journal-article","created":{"date-parts":[[2025,11,10]],"date-time":"2025-11-10T10:52:17Z","timestamp":1762771937000},"page":"679-682","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Long-term kidney function stabilization with fludrocortisone in autosomal recessive renal tubular dysgenesis: a case report"],"prefix":"10.1007","volume":"41","author":[{"given":"Reina","family":"Sugita","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6674-1221","authenticated-orcid":false,"given":"Shoichiro","family":"Kanda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keiichi","family":"Takizawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuko","family":"Kajiho","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yutaka","family":"Harita","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,11,10]]},"reference":[{"key":"7051_CR1","doi-asserted-by":"publisher","first-page":"400","DOI":"10.1038\/ki.2009.423","volume":"77","author":"MC Gubler","year":"2010","unstructured":"Gubler MC, Antignac C (2010) Renin-angiotensin system in kidney development: renal tubular dysgenesis. Kidney Int 77:400\u2013406. https:\/\/doi.org\/10.1038\/ki.2009.423","journal-title":"Kidney Int"},{"key":"7051_CR2","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1093\/ndtplus\/sfr153","volume":"5","author":"SY Kim","year":"2012","unstructured":"Kim SY, Kang HG, Kim EK, Choi JH, Choi Y, Cheong HI (2012) Survival over 2 years of autosomal-recessive renal tubular dysgenesis. Clin Kidney J 5:56\u201358. https:\/\/doi.org\/10.1093\/ndtplus\/sfr153","journal-title":"Clin Kidney J"},{"key":"7051_CR3","doi-asserted-by":"publisher","first-page":"357","DOI":"10.1007\/s00467-014-3007-0","volume":"30","author":"S Hibino","year":"2015","unstructured":"Hibino S, Sasaki H, Abe Y, Hojo A, Uematsu M, Sekine T, Itabashi K (2015) Renal function in angiotensinogen gene-mutated renal tubular dysgenesis with glomerular cysts. Pediatr Nephrol 30:357\u2013360. https:\/\/doi.org\/10.1007\/s00467-014-3007-0","journal-title":"Pediatr Nephrol"},{"key":"7051_CR4","doi-asserted-by":"publisher","DOI":"10.3346\/jkms.2020.35.e283","volume":"35","author":"J Min","year":"2020","unstructured":"Min J, Cho MH, Bae SP, Shin SH, Ha IS, Cheong HI, Kang HG (2020) A premature baby with severe oligohydramnios and hypotension: a case report of renal tubular dysgenesis. J Korean Med Sci 35:e283. https:\/\/doi.org\/10.3346\/jkms.2020.35.e283","journal-title":"J Korean Med Sci"},{"issue":"12","key":"7051_CR5","doi-asserted-by":"publisher","first-page":"2531","DOI":"10.1007\/s00467-010-1584-0","volume":"25","author":"R Schreiber","year":"2010","unstructured":"Schreiber R, Gubler MC, Gribouval O, Shalev H, Landau D (2010) Inherited renal tubular dysgenesis may not be universally fatal. Pediatr Nephrol 25:2531\u20132534. https:\/\/doi.org\/10.1007\/s00467-010-1584-0","journal-title":"Pediatr Nephrol"}],"container-title":["Pediatric Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-025-07051-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00467-025-07051-2","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-025-07051-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T14:02:16Z","timestamp":1769608936000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00467-025-07051-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,10]]},"references-count":5,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,3]]}},"alternative-id":["7051"],"URL":"https:\/\/doi.org\/10.1007\/s00467-025-07051-2","relation":{"has-preprint":[{"id-type":"doi","id":"10.21203\/rs.3.rs-6586160\/v1","asserted-by":"object"}]},"ISSN":["0931-041X","1432-198X"],"issn-type":[{"value":"0931-041X","type":"print"},{"value":"1432-198X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,11,10]]},"assertion":[{"value":"3 May 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"24 June 2025","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"26 June 2025","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 November 2025","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This study was approved by the Ethics Committee of the University of Tokyo.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval"}},{"value":"Written informed consent was obtained from the patient\u2019s legal guardian for publication of this case report.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}