{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T13:55:27Z","timestamp":1788270927479,"version":"build-2803163510"},"reference-count":11,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,1,22]],"date-time":"2024-01-22T00:00:00Z","timestamp":1705881600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,1,22]],"date-time":"2024-01-22T00:00:00Z","timestamp":1705881600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["19K17733"],"award-info":[{"award-number":["19K17733"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["19H03672"],"award-info":[{"award-number":["19H03672"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["22H03085"],"award-info":[{"award-number":["22H03085"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Nephrol"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Fibronectin (FN) glomerulopathy (FNG), a rare autosomal hereditary renal disease, is characterized by proteinuria resulting from the massive accumulation of FN in the glomeruli. It typically affects individuals aged 10\u201350\u00a0years. In this report, we describe the case of a 57-year-old man who was diagnosed with FNG through genetic analysis and histological examination that revealed membranoproliferative glomerulonephritis. Despite treatment with prednisolone, the therapeutic response was unsatisfactory. Prednisolone was subsequently tapered and discontinued because the patient had pulmonary thromboembolism. Subsequent comprehensive genetic testing, which was initially not conducted because the patient\u2019s parents did not have a history of kidney disease, identified a known disease-causing variant in the\n                    <jats:italic>FN1<\/jats:italic>\n                    gene, indicating a de novo variant. FNG was further confirmed by positive staining of glomeruli with FN using an IST-4 antibody. Although corticosteroid therapy is commonly employed as the initial treatment for MPGN, its appropriateness depends on the underlying etiology. Thus, clinicians must be aware of potential rare genetic causes underlying MPGN.\n                  <\/jats:p>","DOI":"10.1186\/s12882-024-03456-7","type":"journal-article","created":{"date-parts":[[2024,1,22]],"date-time":"2024-01-22T04:02:23Z","timestamp":1705896143000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["A case of unexpected diagnosis of fibronectin glomerulopathy with histological features of membranoproliferative glomerulonephritis"],"prefix":"10.1186","volume":"25","author":[{"given":"Misa","family":"Hata","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takayasu","family":"Mori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yurika","family":"Hirose","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuriko","family":"Nishida","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shintaro","family":"Mandai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fumiaki","family":"Ando","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koichiro","family":"Susa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Soichiro","family":"Iimori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shotaro","family":"Naito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eisei","family":"Sohara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatemitsu","family":"Rai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Towako","family":"Taguchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shohei","family":"Tomii","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenichi","family":"Ohashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinichi","family":"Uchida","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,1,22]]},"reference":[{"issue":"5","key":"3456_CR1","doi-asserted-by":"publisher","first-page":"668","DOI":"10.1016\/S0272-6386(96)90247-4","volume":"28","author":"O Gemperle","year":"1996","unstructured":"Gemperle O, Neuweiler J, Reutter FW, Hildebrandt F, Krapf R. Familial glomerulopathy with giant fibrillar (fibronectin-positive) deposits: 15-year follow up in a large kindred. Am J Kidney Dis. 1996;28(5):668\u201375.","journal-title":"Am J Kidney Dis"},{"issue":"15","key":"3456_CR2","doi-asserted-by":"publisher","first-page":"1715","DOI":"10.2169\/internalmedicine.52.0046","volume":"52","author":"Y Masabumi","year":"2013","unstructured":"Masabumi Y, Naoto M, Takahiro O, Keisuke S, Wataru K, Kazuhiro N, et al. Clinicopathological analysis of glomerulopathy with fibronectin deposits (GFND): a case of sporadic, elderly-onset GFND with codeposition of IgA, C1q, and fibrinogen. Intern Med. 2013;52(15):1715\u201320.","journal-title":"Intern Med"},{"issue":"1","key":"3456_CR3","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1016\/j.nefroe.2021.02.007","volume":"41","author":"T Wang","year":"2021","unstructured":"Wang T, Bw H. Fibronectin glomerulopathy: a case report and literature review. Nefrogia (Engl Ed). 2021;41(1):74\u20136.","journal-title":"Nefrogia (Engl Ed)"},{"issue":"1","key":"3456_CR4","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1007\/s10157-016-1252-1","volume":"21","author":"T Mori","year":"2017","unstructured":"Mori T, Kazuyoshi H, Motoko C, Shintaro M, Hirofumi N, Eisei S, et al. Comprehensive genetic testing approach for major inherited kidney diseases, using next-generation sequencing with a custom panel. Clin Exp Nephrol. 2017;21(1):63\u201375.","journal-title":"Clin Exp Nephrol"},{"issue":"9","key":"3456_CR5","doi-asserted-by":"publisher","first-page":"1459","DOI":"10.1007\/s00467-016-3368-7","volume":"31","author":"H Ohtsubo","year":"2016","unstructured":"Ohtsubo H, Taro O, Kandai N, Yutaka T, Akemi S, Katsuhiro A, et al. Identification of mutations in FN1 leading to glomerulopathy with fibronectin deposits. Pediatr Nephrol. 2016;31(9):1459\u201367.","journal-title":"Pediatr Nephrol"},{"issue":"7","key":"3456_CR6","doi-asserted-by":"publisher","first-page":"2538","DOI":"10.1073\/pnas.0707730105","volume":"105","author":"F Castelletti","year":"2008","unstructured":"Castelletti F, Donadelli R, Banterla F, Hildebrandt F, Zipfel PF, Bresin E, et al. Mutations in FN1 cause glomerulopathy with fibronectin deposits. Proc Natl Acad Sci USA. 2008;105(7):2538\u201343.","journal-title":"Proc Natl Acad Sci USA"},{"issue":"8","key":"3456_CR7","doi-asserted-by":"publisher","first-page":"315","DOI":"10.4052\/tigg.8.315","volume":"43","author":"C Wu","year":"1996","unstructured":"Wu C. Integrin mediated fibronectin matrix assembly. 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Thromb Res. 2006;118(2):247\u201352.","journal-title":"Thromb Res"}],"container-title":["BMC Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12882-024-03456-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12882-024-03456-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12882-024-03456-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,22]],"date-time":"2024-01-22T04:04:34Z","timestamp":1705896274000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcnephrol.biomedcentral.com\/articles\/10.1186\/s12882-024-03456-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1,22]]},"references-count":11,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["3456"],"URL":"https:\/\/doi.org\/10.1186\/s12882-024-03456-7","relation":{"has-preprint":[{"id-type":"doi","id":"10.21203\/rs.3.rs-3150527\/v1","asserted-by":"object"}]},"ISSN":["1471-2369"],"issn-type":[{"value":"1471-2369","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,1,22]]},"assertion":[{"value":"8 July 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 January 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 January 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"All participants provided written informed consent and agreed that their clinical information and DNA could be used in studies to identify genetic risk variants for kidney function. The Institutional Review Board of the Tokyo Medical and Dental University approved this study (Approval no. G2000-080).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for the publication of this case report.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"25"}}