{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T10:11:25Z","timestamp":1773223885748,"version":"3.50.1"},"reference-count":11,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T00:00:00Z","timestamp":1773100800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100012112","name":"Swedish Orphan Biovitrum","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100012112","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Membranoproliferative glomerulonephritis (MPGN) is a rare glomerular disorder characterized by immune complex or complement-mediated injury, often leading to nephrotic syndrome, hypertension, and progressive renal dysfunction. Its management remains challenging, particularly in pediatric patients with coexisting renal pathologies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>\n                      We report a case of an 11-years-old girl who presented with nephrotic syndrome, severe hypertension, and impaired renal function. Renal biopsy confirmed immune complex-mediated MPGN (IC-MPGN), and genetic testing revealed a homozygous, pathogenic missense\n                      <jats:italic>PKHD1<\/jats:italic>\n                      variant (NM_138694.3:c.4870C &amp;gt; T; p.(Arg1624Trp), leading to a diagnosis of autosomal recessive polycystic kidney disease (ARPKD). Initial treatment with prednisolone and mycophenolate mofetil failed to halt disease progression, and the patient became dialysis dependent. Pegcetacoplan, a complement C3 inhibitor, was subsequently initiated. After 11 weeks of pegcetacoplan therapy, the patient achieved renal recovery and was successfully weaned from dialysis. Proteinuria decreased from nephrotic to sub-nephrotic levels without significant adverse effects.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>To our knowledge, this is the first pediatric case of IC-MPGN with genetically confirmed ARPKD successfully treated with pegcetacoplan. The case illustrates that renal recovery occurred following initiation of proximal complement inhibition with pegcetacoplan.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fmed.2026.1668375","type":"journal-article","created":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T13:08:27Z","timestamp":1773148107000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Pegcetacoplan-induced remission in pediatric immune-complex membranoproliferative glomerulonephritis with comorbid autosomal recessive polycystic kidney disease: a case report"],"prefix":"10.3389","volume":"13","author":[{"given":"Reem","family":"Alrasheed","sequence":"first","affiliation":[{"name":"Pediatric Nephrology Section, Children\u2019s Specialized Hospital, King Fahad Medical City","place":["Riyadh, Saudi Arabia"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abdulkarim","family":"Alanazi","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Section, Children\u2019s Specialized Hospital, King Fahad Medical City","place":["Riyadh, Saudi Arabia"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raghad","family":"Bukhari","sequence":"additional","affiliation":[{"name":"Pathology and Laboratory Medicine, King Fahad Medical City","place":["Riyadh, Saudi Arabia"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sawsan","family":"Albatati","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Section, Children\u2019s Specialized Hospital, King Fahad Medical City","place":["Riyadh, Saudi Arabia"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hassan","family":"Faqeehi","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Section, Children\u2019s Specialized Hospital, King Fahad Medical City","place":["Riyadh, Saudi Arabia"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saeed","family":"Alzabali","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Section, Children\u2019s Specialized Hospital, King Fahad Medical City","place":["Riyadh, Saudi Arabia"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,3,10]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"434","DOI":"10.1038\/ki.2011.399","article-title":"Membranoproliferative glomerulonephritis and C3 glomerulopathy: resolving the confusion.","volume":"81","author":"Sethi","year":"2012","journal-title":"Kidney Int."},{"key":"B2","doi-asserted-by":"publisher","first-page":"454","DOI":"10.1038\/ki.2012.63","article-title":"Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies.","volume":"82","author":"Servais","year":"2012","journal-title":"Kidney Int."},{"key":"B3","doi-asserted-by":"publisher","first-page":"521","DOI":"10.1007\/s00467-021-05088-7","article-title":"A clinical approach to children with C3 glomerulopathy.","volume":"37","author":"Vivarelli","year":"2022","journal-title":"Pediatr Nephrol."},{"key":"B4","doi-asserted-by":"publisher","first-page":"283","DOI":"10.1093\/ndt\/gfab281","article-title":"Membranoproliferative glomerulonephritis: no longer the same disease and may need very different treatment.","volume":"38","author":"Noris","year":"2023","journal-title":"Nephrol Dial Transplant."},{"key":"B5","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1681\/ASN.2024qdwvz5bg","article-title":"VALIANT: a randomized, multicenter, double-blind, placebo (PBO)-controlled, phase 3 trial of pegcetacoplan for patients with native or post-transplant recurrent glomerulopathy (C3G) or primary immune complex membranoproliferative glomerulonephritis (IC-MPGN).","volume":"35","author":"Nester","year":"2024","journal-title":"J Am Soc Nephrol."},{"key":"B6","doi-asserted-by":"publisher","first-page":"1374","DOI":"10.1681\/ASN.2007010125","article-title":"Genotype-phenotype correlations in autosomal dominant and autosomal recessive polycystic kidney disease.","volume":"18","author":"Rossetti","year":"2007","journal-title":"J Am Soc Nephrol"},{"key":"B7","doi-asserted-by":"publisher","first-page":"e833","DOI":"10.1542\/peds.2013-3646","article-title":"Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects.","volume":"134","author":"Hartung","year":"2014","journal-title":"Pediatrics."},{"key":"B8","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1186\/1752-1947-4-125","article-title":"Autosomal dominant polycystic kidney disease with diffuse proliferative glomerulonephritis - An unusual association: A case report and review of the literature.","volume":"4","author":"D\u2019Cruz","year":"2010","journal-title":"J Med Case Rep."},{"key":"B9","unstructured":"Li\n              X.\n            \n          \n          Polycystic Kidney Disease | Exon Publications.\n          \n          2015"},{"key":"B10","doi-asserted-by":"publisher","first-page":"e70001","DOI":"10.1111\/nep.70001","article-title":"Pegcetacoplan for the treatment of paediatric C3 glomerulonephritis: a case report.","volume":"30","author":"Guzman","year":"2025","journal-title":"Nephrology."},{"key":"B11","doi-asserted-by":"publisher","first-page":"1959","DOI":"10.1007\/s00467-025-06673-w","article-title":"Efficacy of complement inhibition with pegcetacoplan in children with C3 glomerulopathy.","volume":"40","author":"Mancuso","year":"2025","journal-title":"Pediatr Nephrol."}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2026.1668375\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T13:08:28Z","timestamp":1773148108000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2026.1668375\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,10]]},"references-count":11,"alternative-id":["10.3389\/fmed.2026.1668375"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2026.1668375","relation":{},"ISSN":["2296-858X"],"issn-type":[{"value":"2296-858X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,10]]},"article-number":"1668375"}}