{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T00:50:00Z","timestamp":1776905400904,"version":"3.51.2"},"reference-count":15,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T00:00:00Z","timestamp":1774396800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T00:00:00Z","timestamp":1774396800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Nephrology"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Hereditary tubulointerstitial kidney diseases typically manifest as slowly progressive chronic kidney disease. Rapidly progressive kidney failure with non\u2010cystic nephromegaly is an exceptionally rare presentation posing significant diagnostic challenges. We describe a 64\u2010year\u2010old man presenting with rapidly progressive kidney failure and bilateral non\u2010cystic kidney enlargement. Initial imaging, including computed tomography (CT) and magnetic resonance imaging (MRI), revealed no cysts. Kidney biopsy showed tubular ectasia and basement membrane abnormalities consistent with tubulointerstitial disease, and genetic analysis identified a heterozygous\n                    <jats:italic>NPHP1<\/jats:italic>\n                    variant. Although liver biopsy supported a diagnosis of nephronophthisis\u2010related ciliopathy, the aggressive clinical course and absence of cysts were atypical. Notably, 5\u2009years after initiating haemodialysis, follow\u2010up MRI revealed numerous medullary cysts that were previously undetectable. Crucially, these cysts were clearly visible on MRI but remained indistinguishable on concurrent CT. This case illustrates a deceptive \u2018pre\u2010cystic\u2019 phase characterised by rapid progression and inflammatory nephromegaly. It underscores that initial cyst absence does not exclude the diagnosis and establishes the critical superiority of MRI over CT for detecting delayed medullary cysts to determine the aetiology in end\u2010stage kidneys.\n                  <\/jats:p>","DOI":"10.1111\/nep.70198","type":"journal-article","created":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T08:31:24Z","timestamp":1774427484000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Rapidly Progressive Kidney Failure With Transient Non\u2010Cystic Kidney Enlargement: A Case Report Highlighting Delayed Medullary Cyst Formation"],"prefix":"10.1111","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1239-6551","authenticated-orcid":false,"given":"Hiroki","family":"Ito","sequence":"first","affiliation":[{"name":"Division of Nephrology and Hypertension Faculty of Medicine, Tohoku Medical and Pharmaceutical University  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shigeto","family":"Mashiko","sequence":"additional","affiliation":[{"name":"Division of Geriatric and Community Medicine Faculty of Medicine, Tohoku Medical and Pharmaceutical University  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shinichi","family":"Mizuno","sequence":"additional","affiliation":[{"name":"Department of Nephrology Japan Community Health Care Organization Sendai Hospital  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshitaka","family":"Kanomata","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension Faculty of Medicine, Tohoku Medical and Pharmaceutical University  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kentaro","family":"Yano","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension Faculty of Medicine, Tohoku Medical and Pharmaceutical University  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuya","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension Faculty of Medicine, Tohoku Medical and Pharmaceutical University  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fumiya","family":"Sato","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension Faculty of Medicine, Tohoku Medical and Pharmaceutical University  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryo","family":"Tajima","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension Faculty of Medicine, Tohoku Medical and Pharmaceutical University  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Go","family":"Anan","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension Faculty of Medicine, Tohoku Medical and Pharmaceutical University  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takuo","family":"Hirose","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension Faculty of Medicine, Tohoku Medical and Pharmaceutical University  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katsuya","family":"Ishiyama","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension Faculty of Medicine, Tohoku Medical and Pharmaceutical University  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takefumi","family":"Mori","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension Faculty of Medicine, Tohoku Medical and Pharmaceutical University  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,3,25]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2020.03.031"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.06380613"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/cge.13249"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.01220211"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1177\/20543581231203054"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.xkme.2024.100956"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1159\/000526841"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00859626"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/ki.2011.134"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.55.6882"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/12.6.1242"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1097-149"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2007020243"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2013060646"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00467\u2010010\u20101585\u2010z"}],"container-title":["Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/nep.70198","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/nep.70198","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/nep.70198","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T00:07:58Z","timestamp":1776902878000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/nep.70198"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,25]]},"references-count":15,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,4]]}},"alternative-id":["10.1111\/nep.70198"],"URL":"https:\/\/doi.org\/10.1111\/nep.70198","archive":["Portico"],"relation":{},"ISSN":["1320-5358","1440-1797"],"issn-type":[{"value":"1320-5358","type":"print"},{"value":"1440-1797","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,25]]},"assertion":[{"value":"2026-01-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-03-18","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-03-25","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70198"}}