{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,6]],"date-time":"2026-07-06T11:17:38Z","timestamp":1783336658992,"version":"3.54.6"},"reference-count":9,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2021,7,18]],"date-time":"2021-07-18T00:00:00Z","timestamp":1626566400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Hereditary diseases of the glomerular filtration barrier are characterized by a more vulnerable glomerular basement membrane and dysfunctional podocytes. Recent clinical trials have demonstrated the nephroprotective effect of sodium-glucose cotransporter-2 inhibitors (SGLT2i) in chronic kidney disease (CKD). SGLT2-mediated afferent arteriole vasoconstriction is hypothesized to correct the hemodynamic overload of the glomerular filtration barrier in hereditary podocytopathies. To test this hypothesis, we report data in a case series of patients with Alport syndrome and focal segmental glomerulosclerosis (FSGS) with respect of the early effect of SGLT2i on the kidney function. Mean duration of treatment was 4.5 (\u00b12.9) months. Mean serum creatinine before and after SGLT-2i initiation was 1.46 (\u00b10.42) and 1.58 (\u00b10.55) mg\/dL, respectively, with a median estimated glomerular filtration rate of 64 (\u00b127) before and 64 (\u00b132) mL\/min\/1.73 m2 after initiation of SGLT2i. Mean urinary albumin-creatinine ratio in mg\/g creatinine before SGLT-2i initiation was 1827 (\u00b11560) and decreased by almost 40% to 1127 (\u00b1854) after SGLT2i initiation. To our knowledge, this is the first case series on the effect and safety of SGLT2i in patients with hereditary podocytopathies. Specific large-scale trials in podocytopathies are needed to confirm our findings in this population with a tremendous unmet medical need for more effective, early on, and safe nephroprotective therapies.<\/jats:p>","DOI":"10.3390\/cells10071815","type":"journal-article","created":{"date-parts":[[2021,7,18]],"date-time":"2021-07-18T21:16:48Z","timestamp":1626643008000},"page":"1815","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":39,"title":["Sodium-Glucose Cotransporter-2 Inhibitors in Patients with Hereditary Podocytopathies, Alport Syndrome, and FSGS: A Case Series to Better Plan a Large-Scale Study"],"prefix":"10.3390","volume":"10","author":[{"given":"Jan","family":"Boeckhaus","sequence":"first","affiliation":[{"name":"Clinic for Nephrology and Rheumatology, University Medical Center G\u00f6ttingen, 37075 G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8390-8852","authenticated-orcid":false,"given":"Oliver","family":"Gross","sequence":"additional","affiliation":[{"name":"Clinic for Nephrology and Rheumatology, University Medical Center G\u00f6ttingen, 37075 G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,7,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1437","DOI":"10.1056\/NEJMra1808786","article-title":"Insights into glomerular filtration and albuminuria","volume":"384","author":"Benzing","year":"2021","journal-title":"N. Engl. J. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1038\/nrneph.2012.259","article-title":"Alport syndrome\u2014Insights from basic and clinical research","volume":"9","author":"Kruegel","year":"2013","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1159\/000508099","article-title":"Focal segmental glomerulosclerosis: State-of-the-art and clinical perspective","volume":"144","author":"Shabaka","year":"2020","journal-title":"Nephron"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"494","DOI":"10.1038\/ki.2011.407","article-title":"Early angiotensin-converting enzyme inhibition in Alport syndrome delays renal failure and improves life expectancy","volume":"81","author":"Gross","year":"2012","journal-title":"Kidney Int."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1275","DOI":"10.1016\/j.kint.2019.12.015","article-title":"A multicenter, randomized, placebo-controlled, double-blind phase 3 trial with open-arm comparison indicates safety and efficacy of nephroprotective therapy with ramipril in children with Alport\u2019s syndrome","volume":"97","author":"Gross","year":"2020","journal-title":"Kidney Int."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1436","DOI":"10.1056\/NEJMoa2024816","article-title":"Dapagliflozin in patients with chronic kidney disease","volume":"383","author":"Heerspink","year":"2020","journal-title":"N. Engl. J. Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1042\/CS20191276","article-title":"SGLT2 inhibitors\u2014A potential treatment for Alport syndrome","volume":"134","author":"Mabillard","year":"2020","journal-title":"Clin. Sci."},{"key":"ref_8","doi-asserted-by":"crossref","unstructured":"Heerspink, H.J.L., and Cherney, D.Z.I. (2021). Clinical implications of an acute dip in eGFR after SGLT2 inhibitor initiation. Clin. J. Am. Soc. Nephrol.","DOI":"10.2215\/CJN.02480221"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1007\/s00467-020-04819-6","article-title":"Clinical practice recommendations for the diagnosis and management of Alport syndrome in children, adolescents, and young adults\u2014An update for 2020","volume":"36","author":"Kashtan","year":"2021","journal-title":"Pediatr. Nephrol."}],"container-title":["Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4409\/10\/7\/1815\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T06:31:32Z","timestamp":1760164292000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4409\/10\/7\/1815"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,7,18]]},"references-count":9,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2021,7]]}},"alternative-id":["cells10071815"],"URL":"https:\/\/doi.org\/10.3390\/cells10071815","relation":{},"ISSN":["2073-4409"],"issn-type":[{"value":"2073-4409","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,7,18]]}}}