{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,4,24]],"date-time":"2025-04-24T04:24:30Z","timestamp":1745468670308,"version":"3.40.4"},"reference-count":7,"publisher":"S. Karger AG","issue":"4","license":[{"start":{"date-parts":[[2021,12,20]],"date-time":"2021-12-20T00:00:00Z","timestamp":1639958400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2021,12,20]],"date-time":"2021-12-20T00:00:00Z","timestamp":1639958400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nephron"],"published-print":{"date-parts":[[2022]]},"abstract":"<b><i>Purpose:<\/i><\/b> We aimed to evaluate therapeutic response and long-term renal outcomes of childhood idiopathic steroid-resistant nephrotic syndrome (iSRNS). <b><i>Methods:<\/i><\/b> We retrospectively reviewed treatment regimens, especially calcineurin inhibitor (CNI), pathologic diagnoses, and long-term renal outcomes of iSRNS patients for 30 years. <b><i>Results:<\/i><\/b> Of 516 patients with idiopathic NS, 52 (10.1%) had iSRNS. Renal biopsies from 48 patients showed minimal change disease (MCD) in 23 (47.9%), focal segmental glomerulosclerosis in 24 (50.0%), and mesangioproliferative glomerulonephritis in 1 (2.1%). The median follow-up period was 66.5 (range, 4\u2013275) months, and 90.4% of them were treated with a CNI. CNI induced remission in 70.2% within 50.4 \u00b1 43.5 days. Of the patients with MCD and focal segmental glomerular sclerosis (FSGS), 86.4% (19\/22) and 45.0% (9\/20) (<i>p =<\/i> 0.005) responded to CNI, respectively. Mean time until remission after using CNI was longer with FSGS (90.4 \u00b1 54.0 days) than with MCD (29.6 \u00b1 26.3 days) (<i>p =<\/i> 0.010). CNI-responsive patients with FSGS or MCD showed preserved renal function, and CNI nonresponsive MCD patients also showed preserved renal function during follow-up. However, end-stage renal disease (ESRD) progressed in 8 out of 11 patients with FSGS nonresponsive to the CNI for an average of 44.9 \u00b1 18.4 months after diagnosis. <b><i>Conclusion:<\/i><\/b> Different response rates and times for remission were achieved with the CNI according to the pathology of iSRNS. All MCD patients regardless of CNI response and all CNI-responsive patients with FSGS showed excellent renal outcomes, while almost all FSGS patients nonresponsive to CNI eventually progressed to ESRD. <\/jats:p>","DOI":"10.1159\/000520362","type":"journal-article","created":{"date-parts":[[2021,12,20]],"date-time":"2021-12-20T22:00:39Z","timestamp":1640037639000},"page":"327-334","source":"Crossref","is-referenced-by-count":1,"title":["Therapeutic Response and Long-Term Renal Outcomes in Childhood Idiopathic Steroid-Resistant Nephrotic Syndrome: A Single-Center Study"],"prefix":"10.1159","volume":"146","author":[{"given":"Jiwon","family":"Jung","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joo Hoon","family":"Lee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Young Seo","family":"Park","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"127","published-online":{"date-parts":[[2021,12,20]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.105.6.1242"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2015.00104"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-018-4093-1"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1007\/BF00862503"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.V7156"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-010-1471-8"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1007\/s004670100639"}],"container-title":["Nephron"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/520362","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/520362","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T15:28:45Z","timestamp":1745422125000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000520362"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12,20]]},"references-count":7,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2022,7,7]]},"published-print":{"date-parts":[[2022,7,12]]}},"URL":"https:\/\/doi.org\/10.1159\/000520362","archive":["Portico"],"relation":{},"ISSN":["1660-8151","2235-3186"],"issn-type":[{"type":"print","value":"1660-8151"},{"type":"electronic","value":"2235-3186"}],"subject":[],"published":{"date-parts":[[2021,12,20]]}}}