{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T01:03:13Z","timestamp":1772931793797,"version":"3.50.1"},"reference-count":17,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2023,7,26]],"date-time":"2023-07-26T00:00:00Z","timestamp":1690329600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,7,26]],"date-time":"2023-07-26T00:00:00Z","timestamp":1690329600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100012306","name":"Universit\u00e0 degli Studi di Trieste","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100012306","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Nephrol"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>C3-glomerulopathy (C3G) is a rare pediatric kidney disease characterised by dysregulation of the alternative complement pathway, with glomerular deposition of C3. C3G may often present as a steroid-resistant nephrotic syndrome (SRNS), and there is no established effective therapy: the usual treatment involves corticosteroids and immunosuppressive drugs. Pioglitazone, a PPAR-\u03b3 agonist with a protective action on podocytes, was reported in a few cases as helpful in reducing proteinuria when combined with steroids.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case-Diagnosis\/Treatment<\/jats:title>\n                <jats:p>We report the case of a 13-year-old girl with silent past medical history who presented with SRNS. A kidney biopsy showed findings indicative of C3G. A low sodium diet and angiotensin-converting enzyme inhibitor were started; immunosuppressive treatment with mycophenolate mofetil (MMF) was administered due to the cortico-resistance. Because of poor response to the immunosuppressant, a trial with eculizumab was attempted without significant response and persistence of proteinuria in the nephrotic range. A further therapeutic trial was performed with tacrolimus with no disease remission. Due to a severe deterioration in her condition, the girl was hospitalized and treated with high-dose steroid bolus. A daily dose of oral prednisone and MMF were re-started without benefit with persistent levels of nephrotic range proteinuria. The administration of pioglitazone consistently lowered proteinuria levels for the first time since the onset of the disease, with a maintenance of the effect and normalization (&lt;\u20090.15\u00a0g\/24\u00a0h) at the 10-month follow-up.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>In this patient affected by C3G, pioglitazone proved effective in reducing proteinuria levels.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00467-023-06088-5","type":"journal-article","created":{"date-parts":[[2023,7,26]],"date-time":"2023-07-26T10:01:33Z","timestamp":1690365693000},"page":"309-314","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Pioglitazone, a PPAR-y agonist, as one of the new therapeutic candidates for C3 glomerulopathy"],"prefix":"10.1007","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5492-0166","authenticated-orcid":false,"given":"Elia","family":"Balestra","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6343-846X","authenticated-orcid":false,"given":"Egidio","family":"Barbi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7832-9349","authenticated-orcid":false,"given":"Viola","family":"Ceconi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9511-6318","authenticated-orcid":false,"given":"Vittorio","family":"Di Maso","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4317-8231","authenticated-orcid":false,"given":"Ester","family":"Conversano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5207-6232","authenticated-orcid":false,"given":"Marco","family":"Pennesi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2023,7,26]]},"reference":[{"key":"6088_CR1","doi-asserted-by":"publisher","first-page":"629","DOI":"10.1016\/S0140-6736(03)14184-0","volume":"362","author":"AA Eddy","year":"2003","unstructured":"Eddy AA, Symons JM (2003) Nephrotic syndrome in childhood. 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