{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T19:47:29Z","timestamp":1789069649864,"version":"build-2803163510"},"reference-count":81,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T00:00:00Z","timestamp":1621296000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T00:00:00Z","timestamp":1621296000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Nephrol"],"published-print":{"date-parts":[[2022,3]]},"DOI":"10.1007\/s00467-021-05088-7","type":"journal-article","created":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T04:04:03Z","timestamp":1621310643000},"page":"521-535","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["A clinical approach to children with C3 glomerulopathy"],"prefix":"10.1007","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2448-1959","authenticated-orcid":false,"given":"Marina","family":"Vivarelli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1990-1189","authenticated-orcid":false,"given":"Nicole","family":"van de Kar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raffaella","family":"Labbadia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Diomedi-Camassei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3476-9956","authenticated-orcid":false,"given":"Joshua M.","family":"Thurman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,5,18]]},"reference":[{"key":"5088_CR1","doi-asserted-by":"crossref","unstructured":"Bao L, Cunningham PN, Quigg RJ (2015) Complement in lupus nephritis: new perspectives. Kidney Dis (Basel) 1:91\u201399","DOI":"10.1159\/000431278"},{"key":"5088_CR2","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1038\/nrneph.2017.37","volume":"13","author":"M Chen","year":"2017","unstructured":"Chen M, Jayne DRW, Zhao M-H (2017) Complement in ANCA-associated vasculitis: mechanisms and implications for management. Nat Rev Nephrol 13:359\u2013367","journal-title":"Nat Rev Nephrol"},{"key":"5088_CR3","doi-asserted-by":"publisher","first-page":"572","DOI":"10.1016\/j.ekir.2020.02.1033","volume":"5","author":"V Brglez","year":"2020","unstructured":"Brglez V, Boyer-Suavet S, Seitz-Polski B (2020) Complement pathways in membranous nephropathy: complex and multifactorial. Kidney Int Rep 5:572\u2013574","journal-title":"Kidney Int Rep"},{"key":"5088_CR4","doi-asserted-by":"publisher","first-page":"1503","DOI":"10.1681\/ASN.2014101000","volume":"26","author":"N Maillard","year":"2015","unstructured":"Maillard N, Wyatt RJ, Julian BA, Kiryluk K, Gharavi A, Fremeaux-Bacchi V, Novak J (2015) Current understanding of the role of complement in IgA nephropathy. J Am Soc Nephrol 26:1503\u20131512","journal-title":"J Am Soc Nephrol"},{"key":"5088_CR5","doi-asserted-by":"publisher","first-page":"829","DOI":"10.1681\/ASN.2019080851","volume":"31","author":"S Chauvet","year":"2020","unstructured":"Chauvet S, Berthaud R, Devriese M, Mignotet M, Vieira Martins P, Robe-Rybkine T, Miteva MA, Gyulkhandanyan A, Ryckewaert A, Louillet F, Merieau E, Mestrallet G, Rousset-Rouviere C, Thervet E, Hogan J, Ulinski T, Villoutreix BO, Roumenina L, Boyer O, Fremeaux-Bacchi V (2020) Anti-factor B antibodies and acute postinfectious GN in children. J Am Soc Nephrol 31:829\u2013840","journal-title":"J Am Soc Nephrol"},{"key":"5088_CR6","doi-asserted-by":"publisher","first-page":"2166","DOI":"10.3389\/fimmu.2019.02166","volume":"10","author":"PF Zipfel","year":"2019","unstructured":"Zipfel PF, Wiech T, Rudnick R, Afonso S, Person F, Skerka C (2019) Complement inhibitors in clinical trials for glomerular diseases. Front Immunol 10:2166","journal-title":"Front Immunol"},{"key":"5088_CR7","doi-asserted-by":"publisher","first-page":"262","DOI":"10.3389\/fimmu.2015.00262","volume":"6","author":"NS Merle","year":"2015","unstructured":"Merle NS, Church SE, Fremeaux-Bacchi V, Roumenina LT (2015) Complement system part I \u2014 molecular mechanisms of activation and regulation. Front Immunol 6:262","journal-title":"Front Immunol"},{"key":"5088_CR8","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1111\/imr.12468","volume":"274","author":"P Garred","year":"2016","unstructured":"Garred P, Genster N, Pilely K, Bayarri-Olmos R, Rosbjerg A, Ma YJ, Skjoedt MO (2016) A journey through the lectin pathway of complement-MBL and beyond. Immunol Rev 274:74\u201397","journal-title":"Immunol Rev"},{"key":"5088_CR9","doi-asserted-by":"publisher","first-page":"1984","DOI":"10.1093\/ndt\/13.8.1984","volume":"13","author":"M Endo","year":"1998","unstructured":"Endo M, Ohi H, Ohsawa I, Fujita T, Matsushita M, Fujita T (1998) Glomerular deposition of mannose-binding lectin (MBL) indicates a novel mechanism of complement activation in IgA nephropathy. Nephrol Dial Transplant 13:1984\u20131990","journal-title":"Nephrol Dial Transplant"},{"key":"5088_CR10","doi-asserted-by":"publisher","first-page":"1724","DOI":"10.1681\/ASN.2005090923","volume":"17","author":"A Roos","year":"2006","unstructured":"Roos A, Rastaldi MP, Calvaresi N, Oortwijn BD, Schlagwein N, van Gijlswijk-Janssen DJ, Stahl GL, Matsushita M, Fujita T, van Kooten C, Daha MR (2006) Glomerular activation of the lectin pathway of complement in IgA nephropathy is associated with more severe renal disease. J Am Soc Nephrol 17:1724\u20131734","journal-title":"J Am Soc Nephrol"},{"key":"5088_CR11","doi-asserted-by":"publisher","first-page":"531","DOI":"10.1016\/j.semnephrol.2013.08.004","volume":"33","author":"H Ma","year":"2013","unstructured":"Ma H, Sandor DG, Beck LH Jr (2013) The role of complement in membranous nephropathy. Semin Nephrol 33:531\u2013542","journal-title":"Semin Nephrol"},{"key":"5088_CR12","doi-asserted-by":"publisher","first-page":"774","DOI":"10.1016\/j.kint.2016.05.011","volume":"90","author":"M Ozaki","year":"2016","unstructured":"Ozaki M, Kang Y, Tan YS, Pavlov VI, Liu B, Boyle DC, Kushak RI, Skjoedt MO, Grabowski EF, Taira Y, Stahl GL (2016) Human mannose-binding lectin inhibitor prevents Shiga toxin-induced renal injury. Kidney Int 90:774\u2013782","journal-title":"Kidney Int"},{"key":"5088_CR13","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1038\/s41581-018-0107-2","volume":"15","author":"RJH Smith","year":"2019","unstructured":"Smith RJH, Appel GB, Blom AM, Cook HT, D'Agati VD, Fakhouri F, Fremeaux-Bacchi V, Jozsi M, Kavanagh D, Lambris JD, Noris M, Pickering MC, Remuzzi G, de Cordoba SR, Sethi S, Van der Vlag J, Zipfel PF, Nester CM (2019) C3 glomerulopathy - understanding a rare complement-driven renal disease. Nat Rev Nephrol 15:129\u2013143","journal-title":"Nat Rev Nephrol"},{"key":"5088_CR14","doi-asserted-by":"publisher","first-page":"539","DOI":"10.1016\/j.kint.2016.10.005","volume":"91","author":"TH Goodship","year":"2017","unstructured":"Goodship TH, Cook HT, Fakhouri F, Fervenza FC, Fremeaux-Bacchi V, Kavanagh D, Nester CM, Noris M, Pickering MC, Rodriguez de Cordoba S, Roumenina LT, Sethi S, Smith RJ, Conference P (2017) Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a \"Kidney Disease: Improving Global Outcomes\" (KDIGO) Controversies Conference. Kidney Int 91:539\u2013551","journal-title":"Kidney Int"},{"key":"5088_CR15","doi-asserted-by":"publisher","first-page":"3539","DOI":"10.1681\/ASN.2015101155","volume":"27","author":"S Bally","year":"2016","unstructured":"Bally S, Debiec H, Ponard D, Dijoud F, Rendu J, Faure J, Ronco P, Dumestre-Perard C (2016) Phospholipase A2 receptor-related membranous nephropathy and mannan-binding lectin deficiency. J Am Soc Nephrol 27:3539\u20133544","journal-title":"J Am Soc Nephrol"},{"key":"5088_CR16","doi-asserted-by":"publisher","first-page":"1884","DOI":"10.2215\/CJN.02790313","volume":"8","author":"SJ Gou","year":"2013","unstructured":"Gou SJ, Yuan J, Wang C, Zhao MH, Chen M (2013) Alternative complement pathway activation products in urine and kidneys of patients with ANCA-associated GN. Clin J Am Soc Nephrol 8:1884\u20131891","journal-title":"Clin J Am Soc Nephrol"},{"key":"5088_CR17","doi-asserted-by":"publisher","first-page":"52","DOI":"10.2353\/ajpath.2007.060573","volume":"170","author":"H Xiao","year":"2007","unstructured":"Xiao H, Schreiber A, Heeringa P, Falk RJ, Jennette JC (2007) Alternative complement pathway in the pathogenesis of disease mediated by anti-neutrophil cytoplasmic autoantibodies. Am J Pathol 170:52\u201364","journal-title":"Am J Pathol"},{"key":"5088_CR18","doi-asserted-by":"publisher","first-page":"1517","DOI":"10.4049\/jimmunol.170.3.1517","volume":"170","author":"JM Thurman","year":"2003","unstructured":"Thurman JM, Ljubanovic D, Edelstein CL, Gilkeson GS, Holers VM (2003) Lack of a functional alternative complement pathway ameliorates ischemic acute renal failure in mice. J Immunol 170:1517\u20131523","journal-title":"J Immunol"},{"key":"5088_CR19","doi-asserted-by":"publisher","first-page":"162","DOI":"10.1038\/ni.2499","volume":"14","author":"MG Strainic","year":"2013","unstructured":"Strainic MG, Shevach EM, An F, Lin F, Medof ME (2013) Absence of signaling into CD4(+) cells via C3aR and C5aR enables autoinductive TGF-beta1 signaling and induction of Foxp3(+) regulatory T cells. Nat Immunol 14:162\u2013171","journal-title":"Nat Immunol"},{"key":"5088_CR20","doi-asserted-by":"publisher","first-page":"2756","DOI":"10.1681\/ASN.2016111179","volume":"28","author":"DRW Jayne","year":"2017","unstructured":"Jayne DRW, Bruchfeld AN, Harper L, Schaier M, Venning MC, Hamilton P, Burst V, Grundmann F, Jadoul M, Szombati I, Tesar V, Segelmark M, Potarca A, Schall TJ, Bekker P, CLEAR Study Group (2017) Randomized trial of C5a receptor inhibitor avacopan in ANCA-associated vasculitis. J Am Soc Nephrol 28:2756\u20132767","journal-title":"J Am Soc Nephrol"},{"key":"5088_CR21","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1056\/NEJMoa2023386","volume":"384","author":"DRW Jayne","year":"2021","unstructured":"Jayne DRW, Merkel PA, Schall TJ, Bekker P, ADVOCATE Study (2021) Avacopan for the treatment of ANCA-associated vasculitis. N Engl J Med 384:599\u2013609","journal-title":"N Engl J Med"},{"key":"5088_CR22","first-page":"185","volume":"135","author":"PJ Baker","year":"1989","unstructured":"Baker PJ, Ochi RF, Schulze M, Johnson RJ, Campbell C, Couser WG (1989) Depletion of C6 prevents development of proteinuria in experimental membranous nephropathy in rats. Am J Pathol 135:185\u2013194","journal-title":"Am J Pathol"},{"key":"5088_CR23","doi-asserted-by":"publisher","first-page":"F841","DOI":"10.1152\/ajprenal.2001.281.5.F841","volume":"281","author":"T Takano","year":"2001","unstructured":"Takano T, Cybulsky AV, Yang X, Aoudjit L (2001) Complement C5b-9 induces cyclooxygenase-2 gene transcription in glomerular epithelial cells. Am J Physiol Ren Physiol 281:F841\u2013F850","journal-title":"Am J Physiol Ren Physiol"},{"key":"5088_CR24","doi-asserted-by":"publisher","first-page":"424","DOI":"10.1038\/ng912","volume":"31","author":"MC Pickering","year":"2002","unstructured":"Pickering MC, Cook HT, Warren J, Bygrave AE, Moss J, Walport MJ, Botto M (2002) Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H. Nat Genet 31:424\u2013428","journal-title":"Nat Genet"},{"key":"5088_CR25","doi-asserted-by":"publisher","first-page":"1436","DOI":"10.2215\/CJN.09541010","volume":"6","author":"Y Athanasiou","year":"2011","unstructured":"Athanasiou Y, Voskarides K, Gale DP, Damianou L, Patsias C, Zavros M, Maxwell PH, Cook HT, Demosthenous P, Hadjisavvas A, Kyriacou K, Zouvani I, Pierides A, Deltas C (2011) Familial C3 glomerulopathy associated with CFHR5 mutations: clinical characteristics of 91 patients in 16 pedigrees. Clin J Am Soc Nephrol 6:1436\u20131446","journal-title":"Clin J Am Soc Nephrol"},{"key":"5088_CR26","doi-asserted-by":"publisher","first-page":"1245","DOI":"10.1681\/ASN.2015040385","volume":"27","author":"F Bu","year":"2015","unstructured":"Bu F, Borsa NG, Jones MB, Takanami E, Nishimura C, Hauer JJ, Azaiez H, Black-Ziegelbein EA, Meyer NC, Kolbe DL, Li Y, Frees K, Schnieders MJ, Thomas C, Nester C, Smith RJH (2015) High-Throughput genetic testing for thrombotic microangiopathies and C3 glomerulopathies. J Am Soc Nephrol 27:1245\u20131253","journal-title":"J Am Soc Nephrol"},{"key":"5088_CR27","doi-asserted-by":"publisher","first-page":"145","DOI":"10.1172\/JCI71866","volume":"124","author":"Q Chen","year":"2014","unstructured":"Chen Q, Wiesener M, Eberhardt HU, Hartmann A, Uzonyi B, Kirschfink M, Amann K, Buettner M, Goodship T, Hugo C, Skerka C, Zipfel PF (2014) Complement factor H-related hybrid protein deregulates complement in dense deposit disease. J Clin Invest 124:145\u2013155","journal-title":"J Clin Invest"},{"key":"5088_CR28","doi-asserted-by":"publisher","first-page":"794","DOI":"10.1016\/S0140-6736(10)60670-8","volume":"376","author":"DP Gale","year":"2010","unstructured":"Gale DP, de Jorge EG, Cook HT, Martinez-Barricarte R, Hadjisavvas A, McLean AG, Pusey CD, Pierides A, Kyriacou K, Athanasiou Y, Voskarides K, Deltas C, Palmer A, Fremeaux-Bacchi V, de Cordoba SR, Maxwell PH, Pickering MC (2010) Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis. Lancet 376:794\u2013801","journal-title":"Lancet"},{"key":"5088_CR29","doi-asserted-by":"publisher","first-page":"1155","DOI":"10.1681\/ASN.2012020166","volume":"23","author":"TH Malik","year":"2012","unstructured":"Malik TH, Lavin PJ, Goicoechea de Jorge E, Vernon KA, Rose KL, Patel MP, de Leeuw M, Neary JJ, Conlon PJ, Winn MP, Pickering MC (2012) A hybrid CFHR3-1 gene causes familial C3 glomerulopathy. J Am Soc Nephrol 23:1155\u20131160","journal-title":"J Am Soc Nephrol"},{"key":"5088_CR30","doi-asserted-by":"publisher","first-page":"933","DOI":"10.1038\/ki.2013.348","volume":"85","author":"N Medjeral-Thomas","year":"2014","unstructured":"Medjeral-Thomas N, Malik TH, Patel MP, Toth T, Cook HT, Tomson C, Pickering MC (2014) A novel CFHR5 fusion protein causes C3 glomerulopathy in a family without Cypriot ancestry. Kidney Int 85:933\u2013937","journal-title":"Kidney Int"},{"key":"5088_CR31","doi-asserted-by":"publisher","first-page":"2434","DOI":"10.1172\/JCI68280","volume":"123","author":"A Tortajada","year":"2013","unstructured":"Tortajada A, Yebenes H, Abarrategui-Garrido C, Anter J, Garcia-Fernandez JM, Martinez-Barricarte R, Alba-Dominguez M, Malik TH, Bedoya R, Cabrera Perez R, Lopez Trascasa M, Pickering MC, Harris CL, Sanchez-Corral P, Llorca O, Rodriguez de Cordoba S (2013) C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. J Clin Invest 123:2434\u20132446","journal-title":"J Clin Invest"},{"key":"5088_CR32","doi-asserted-by":"publisher","first-page":"886","DOI":"10.3389\/fimmu.2019.00886","volume":"10","author":"F Corvillo","year":"2019","unstructured":"Corvillo F, Okroj M, Nozal P, Melgosa M, Sanchez-Corral P, Lopez-Trascasa M (2019) Nephritic factors: an overview of classification, diagnostic tools and clinical associations. Front Immunol 10:886","journal-title":"Front Immunol"},{"key":"5088_CR33","doi-asserted-by":"publisher","first-page":"1","DOI":"10.4049\/jimmunol.116.1.1","volume":"116","author":"MR Daha","year":"1976","unstructured":"Daha MR, Fearon DT, Austen KF (1976) C3 nephritic factor (C3NeF): stabilization of fluid phase and cell-bound alternative pathway convertase. J Immunol 116:1\u20137","journal-title":"J Immunol"},{"key":"5088_CR34","doi-asserted-by":"publisher","first-page":"2","DOI":"10.1007\/s00467-006-0299-8","volume":"22","author":"C Licht","year":"2007","unstructured":"Licht C, Schlotzer-Schrehardt U, Kirschfink M, Zipfel PF, Hoppe B (2007) MPGN II--genetically determined by defective complement regulation? Pediatr Nephrol 22:2\u20139","journal-title":"Pediatr Nephrol"},{"key":"5088_CR35","doi-asserted-by":"publisher","first-page":"605","DOI":"10.1038\/modpathol.3800773","volume":"20","author":"PD Walker","year":"2007","unstructured":"Walker PD, Ferrario F, Joh K, Bonsib SM (2007) Dense deposit disease is not a membranoproliferative glomerulonephritis. Mod Pathol 20:605\u2013616","journal-title":"Mod Pathol"},{"key":"5088_CR36","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1136\/jmg.2006.045328","volume":"44","author":"A Servais","year":"2007","unstructured":"Servais A, Fremeaux-Bacchi V, Lequintrec M, Salomon R, Blouin J, Knebelmann B, Grunfeld JP, Lesavre P, Noel LH, Fakhouri F (2007) Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome. J Med Genet 44:193\u2013199","journal-title":"J Med Genet"},{"key":"5088_CR37","doi-asserted-by":"publisher","first-page":"952","DOI":"10.1038\/ki.2008.657","volume":"75","author":"S Sethi","year":"2009","unstructured":"Sethi S, Gamez JD, Vrana JA, Theis JD, Bergen HR 3rd, Zipfel PF, Dogan A, Smith RJ (2009) Glomeruli of Dense Deposit Disease contain components of the alternative and terminal complement pathway. Kidney Int 75:952\u2013960","journal-title":"Kidney Int"},{"key":"5088_CR38","doi-asserted-by":"publisher","first-page":"1079","DOI":"10.1038\/ki.2013.377","volume":"84","author":"MC Pickering","year":"2013","unstructured":"Pickering MC, D'Agati VD, Nester CM, Smith RJ, Haas M, Appel GB, Alpers CE, Bajema IM, Bedrosian C, Braun M, Doyle M, Fakhouri F, Fervenza FC, Fogo AB, Fremeaux-Bacchi V, Gale DP, Goicoechea de Jorge E, Griffin G, Harris CL, Holers VM, Johnson S, Lavin PJ, Medjeral-Thomas N, Paul Morgan B, Nast CC, Noel LH, Peters DK, Rodriguez de Cordoba S, Servais A, Sethi S, Song WC, Tamburini P, Thurman JM, Zavros M, Cook HT (2013) C3 glomerulopathy: consensus report. Kidney Int 84:1079\u20131089","journal-title":"Kidney Int"},{"key":"5088_CR39","unstructured":"Martin B, Smith RJH (2007) C3 glomerulopathy. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Mirzaa G, Amemiya A (eds) GeneReviews((R)), Seattle (WA), pp 1993-2020"},{"key":"5088_CR40","doi-asserted-by":"publisher","first-page":"1195","DOI":"10.1007\/s00467-020-04799-7","volume":"36","author":"AS P\u0131narba\u015f\u0131","year":"2020","unstructured":"P\u0131narba\u015f\u0131 AS, Dursun I, Gokce I, \u00c7omak E, Sayg\u0131l\u0131 S, Bayram MT, Donmez O, Melek E, Tekcan D, \u00c7i\u00e7ek N, Y\u0131lmaz D, Tabel Y, Y\u0131ld\u0131r\u0131m ZY, Bahat E, Koyun M, Soylu A, Canpolat N, Aksu B, \u00c7elak\u0131l ME, Ta\u015fdemir M, Benzer M, \u00d6z\u00e7elik G, Bakkalo\u011flu SA, D\u00fc\u015f\u00fcnsel R (2020) Predictors of poor kidney outcome in children with C3 glomerulopathy. Pediatr Nephrol 36:1195\u20131205","journal-title":"Pediatr Nephrol"},{"key":"5088_CR41","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1007\/s00467-020-04736-8","volume":"36","author":"P Khandelwal","year":"2021","unstructured":"Khandelwal P, Bhardwaj S, Singh G, Sinha A, Hari P, Bagga A (2021) Therapy and outcomes of C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis. Pediatr Nephrol 36:591\u2013600","journal-title":"Pediatr Nephrol"},{"key":"5088_CR42","doi-asserted-by":"publisher","first-page":"131","DOI":"10.1016\/j.molimm.2016.01.010","volume":"71","author":"P Iatropoulos","year":"2016","unstructured":"Iatropoulos P, Noris M, Mele C, Piras R, Valoti E, Bresin E, Curreri M, Mondo E, Zito A, Gamba S, Bettoni S, Murer L, Fremeaux-Bacchi V, Vivarelli M, Emma F, Daina E, Remuzzi G (2016) Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome. Mol Immunol 71:131\u2013142","journal-title":"Mol Immunol"},{"key":"5088_CR43","doi-asserted-by":"publisher","first-page":"316","DOI":"10.1053\/j.ajkd.2018.09.002","volume":"73","author":"R Regunathan-Shenk","year":"2019","unstructured":"Regunathan-Shenk R, Avasare RS, Ahn W, Canetta PA, Cohen DJ, Appel GB, Bomback AS (2019) Kidney transplantation in C3 glomerulopathy: a case series. Am J Kidney Dis 73:316\u2013323","journal-title":"Am J Kidney Dis"},{"key":"5088_CR44","doi-asserted-by":"publisher","first-page":"283","DOI":"10.1681\/ASN.2017030258","volume":"29","author":"P Iatropoulos","year":"2018","unstructured":"Iatropoulos P, Daina E, Curreri M, Piras R, Valoti E, Mele C, Bresin E, Gamba S, Alberti M, Breno M, Perna A, Bettoni S, Sabadini E, Murer L, Vivarelli M, Noris M, Remuzzi G, Registry of Membranoproliferative Glomerulonephritis \/C3 Glomerulopathy, Nastasi (2018) Cluster analysis identifies distinct pathogenetic patterns in C3 glomerulopathies\/immune complex-mediated membranoproliferative GN. J Am Soc Nephrol 29:283\u2013294","journal-title":"J Am Soc Nephrol"},{"key":"5088_CR45","doi-asserted-by":"publisher","first-page":"454","DOI":"10.1038\/ki.2012.63","volume":"82","author":"A Servais","year":"2012","unstructured":"Servais A, Noel LH, Roumenina LT, Le Quintrec M, Ngo S, Dragon-Durey MA, Macher MA, Zuber J, Karras A, Provot F, Moulin B, Grunfeld JP, Niaudet P, Lesavre P, Fremeaux-Bacchi V (2012) Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int 82:454\u2013464","journal-title":"Kidney Int"},{"key":"5088_CR46","doi-asserted-by":"publisher","first-page":"2852","DOI":"10.1681\/ASN.2014040406","volume":"26","author":"S Sethi","year":"2015","unstructured":"Sethi S, Nasr SH, De Vriese AS, Fervenza FC (2015) C4d as a diagnostic tool in proliferative GN. J Am Soc Nephrol 26:2852\u20132859","journal-title":"J Am Soc Nephrol"},{"key":"5088_CR47","doi-asserted-by":"publisher","first-page":"601","DOI":"10.1111\/his.13385","volume":"72","author":"Y Bouatou","year":"2018","unstructured":"Bouatou Y, Kers J, Chevalier-Florquin MSN, Claessen N, Nguyen TQ, Damman J, Proenca H, Roelofs J, Florquin S (2018) Diagnostic accuracy of immunofluorescence versus immunoperoxidase staining to distinguish immune complex-mediated glomerulonephritis and C3 dominant glomerulopathy. Histopathology 72:601\u2013608","journal-title":"Histopathology"},{"key":"5088_CR48","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1016\/j.nefro.2016.05.011","volume":"37","author":"N Gupta","year":"2017","unstructured":"Gupta N, Wakefield DN, Clapp WL, Garin EH (2017) Use of C4d as a diagnostic tool to classify membranoproliferative glomerulonephritis. Nefrologia 37:78\u201386","journal-title":"Nefrologia"},{"key":"5088_CR49","doi-asserted-by":"publisher","first-page":"698","DOI":"10.1016\/j.ekir.2019.02.006","volume":"4","author":"G Singh","year":"2019","unstructured":"Singh G, Singh SK, Nalwa A, Singh L, Pradeep I, Barwad A, Sinha A, Hari P, Bagga A, Bagchi S, Agarwal SK, Dinda AK (2019) Glomerular C4d staining does not exclude a C3 glomerulopathy. Kidney Int Rep 4:698\u2013709","journal-title":"Kidney Int Rep"},{"key":"5088_CR50","doi-asserted-by":"publisher","first-page":"46","DOI":"10.2215\/CJN.04700513","volume":"9","author":"NR Medjeral-Thomas","year":"2014","unstructured":"Medjeral-Thomas NR, O'Shaughnessy MM, O'Regan JA, Traynor C, Flanagan M, Wong L, Teoh CW, Awan A, Waldron M, Cairns T, O'Kelly P, Dorman AM, Pickering MC, Conlon PJ, Cook HT (2014) C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol 9:46\u201353","journal-title":"Clin J Am Soc Nephrol"},{"key":"5088_CR51","doi-asserted-by":"publisher","first-page":"1876","DOI":"10.2215\/CJN.01820214","volume":"9","author":"Y Zhang","year":"2014","unstructured":"Zhang Y, Nester CM, Martin B, Skjoedt MO, Meyer NC, Shao D, Borsa N, Palarasah Y, Smith RJ (2014) Defining the complement biomarker profile of c3 glomerulopathy. Clin J Am Soc Nephrol 9:1876\u20131882","journal-title":"Clin J Am Soc Nephrol"},{"key":"5088_CR52","doi-asserted-by":"publisher","first-page":"1311","DOI":"10.1007\/s00467-018-3989-0","volume":"34","author":"M Noris","year":"2019","unstructured":"Noris M, Donadelli R, Remuzzi G (2019) Autoimmune abnormalities of the alternative complement pathway in membranoproliferative glomerulonephritis and C3 glomerulopathy. Pediatr Nephrol 34:1311\u20131323","journal-title":"Pediatr Nephrol"},{"key":"5088_CR53","doi-asserted-by":"publisher","first-page":"247","DOI":"10.1186\/s13023-019-1237-8","volume":"14","author":"N Garam","year":"2019","unstructured":"Garam N, Prohaszka Z, Szilagyi A, Aigner C, Schmidt A, Gaggl M, Sunder-Plassmann G, Bajcsi D, Brunner J, Dumfarth A, Cejka D, Flaschberger S, Flogelova H, Haris A, Hartmann A, Heilos A, Mueller T, Rusai K, Arbeiter K, Hofer J, Jakab D, Sinko M, Szigeti E, Bereczki C, Janko V, Kelen K, Reusz GS, Szabo AJ, Klenk N, Kobor K, Kojc N, Knechtelsdorfer M, Laganovic M, Lungu AC, Meglic A, Rus R, Kersnik-Levart T, Macioniene E, Miglinas M, Pawlowska A, Stompor T, Podracka L, Rudnicki M, Mayer G, Romana R, Reiterova J, Saraga M, Tomas S, Zieg J, Sladkova E, Szabo T, Capitanescu A, Stancu S, Tisljar M, Galesic K, Tisler A, Vainumae I, Windpessl M, Zaoral T, Zlatanova G, Csuka D (2019) C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy. Orphanet J Rare Dis 14:247","journal-title":"Orphanet J Rare Dis"},{"key":"5088_CR54","doi-asserted-by":"publisher","first-page":"1232","DOI":"10.1016\/j.kint.2017.04.017","volume":"92","author":"MC Marinozzi","year":"2017","unstructured":"Marinozzi MC, Chauvet S, Le Quintrec M, Mignotet M, Petitprez F, Legendre C, Cailliez M, Deschenes G, Fischbach M, Karras A, Nobili F, Pietrement C, Dragon-Durey MA, Fakhouri F, Roumenina LT, Fremeaux-Bacchi V (2017) C5 nephritic factors drive the biological phenotype of C3 glomerulopathies. Kidney Int 92:1232\u20131241","journal-title":"Kidney Int"},{"key":"5088_CR55","doi-asserted-by":"publisher","first-page":"5129","DOI":"10.4049\/jimmunol.1402770","volume":"194","author":"C Blanc","year":"2015","unstructured":"Blanc C, Togarsimalemath SK, Chauvet S, Le Quintrec M, Moulin B, Buchler M, Jokiranta TS, Roumenina LT, Fremeaux-Bacchi V, Dragon-Durey MA (2015) Anti-factor H autoantibodies in C3 glomerulopathies and in atypical hemolytic uremic syndrome: one target, two diseases. J Immunol 194:5129\u20135138","journal-title":"J Immunol"},{"key":"5088_CR56","doi-asserted-by":"publisher","first-page":"200","DOI":"10.1016\/j.molimm.2012.05.009","volume":"52","author":"TH Goodship","year":"2012","unstructured":"Goodship TH, Pappworth IY, Toth T, Denton M, Houlberg K, McCormick F, Warland D, Moore I, Hunze EM, Staniforth SJ, Hayes C, Cavalcante DP, Kavanagh D, Strain L, Herbert AP, Schmidt CQ, Barlow PN, Harris CL, Marchbank KJ (2012) Factor H autoantibodies in membranoproliferative glomerulonephritis. Mol Immunol 52:200\u2013206","journal-title":"Mol Immunol"},{"key":"5088_CR57","doi-asserted-by":"publisher","first-page":"2340","DOI":"10.1056\/NEJMc1107484","volume":"365","author":"Q Chen","year":"2011","unstructured":"Chen Q, Muller D, Rudolph B, Hartmann A, Kuwertz-Broking E, Wu K, Kirschfink M, Skerka C, Zipfel PF (2011) Combined C3b and factor B autoantibodies and MPGN type II. N Engl J Med 365:2340\u20132342","journal-title":"N Engl J Med"},{"key":"5088_CR58","doi-asserted-by":"publisher","first-page":"1247","DOI":"10.1016\/j.imbio.2016.06.008","volume":"221","author":"Z Prohaszka","year":"2016","unstructured":"Prohaszka Z, Nilsson B, Frazer-Abel A, Kirschfink M (2016) Complement analysis 2016: Clinical indications, laboratory diagnostics and quality control. Immunobiology 221:1247\u20131258","journal-title":"Immunobiology"},{"key":"5088_CR59","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1038\/sj.ki.5000269","volume":"70","author":"C Licht","year":"2006","unstructured":"Licht C, Heinen S, Jozsi M, Loschmann I, Saunders RE, Perkins SJ, Waldherr R, Skerka C, Kirschfink M, Hoppe B, Zipfel PF (2006) Deletion of Lys224 in regulatory domain 4 of Factor H reveals a novel pathomechanism for dense deposit disease (MPGN II). Kidney Int 70:42\u201350","journal-title":"Kidney Int"},{"key":"5088_CR60","doi-asserted-by":"publisher","first-page":"862","DOI":"10.1093\/ndt\/gfv054","volume":"30","author":"H Imamura","year":"2015","unstructured":"Imamura H, Konomoto T, Tanaka E, Hisano S, Yoshida Y, Fujimura Y, Miyata T, Nunoi H (2015) Familial C3 glomerulonephritis associated with mutations in the gene for complement factor B. Nephrol Dial Transplant 30:862\u2013864","journal-title":"Nephrol Dial Transplant"},{"key":"5088_CR61","doi-asserted-by":"publisher","first-page":"3702","DOI":"10.1172\/JCI43343","volume":"120","author":"R Martinez-Barricarte","year":"2010","unstructured":"Martinez-Barricarte R, Heurich M, Valdes-Canedo F, Vazquez-Martul E, Torreira E, Montes T, Tortajada A, Pinto S, Lopez-Trascasa M, Morgan BP, Llorca O, Harris CL, Rodriguez de Cordoba S (2010) Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation. J Clin Invest 120:3702\u20133712","journal-title":"J Clin Invest"},{"key":"5088_CR62","doi-asserted-by":"publisher","first-page":"1153","DOI":"10.1038\/ki.2015.227","volume":"88","author":"C Rabasco","year":"2015","unstructured":"Rabasco C, Cavero T, Roman E, Rojas-Rivera J, Olea T, Espinosa M, Cabello V, Fernandez-Juarez G, Gonzalez F, Avila A, Baltar JM, Diaz M, Alegre R, Elias S, Anton M, Frutos MA, Pobes A, Blasco M, Martin F, Bernis C, Macias M, Barroso S, de Lorenzo A, Ariceta G, Lopez-Mendoza M, Rivas B, Lopez-Revuelta K, Campistol JM, Mendizabal S, de Cordoba SR, Praga M, Spanish Group for the Study of Glomerular Disease (2015) Effectiveness of mycophenolate mofetil in C3 glomerulonephritis. Kidney Int 88:1153\u20131160","journal-title":"Kidney Int"},{"key":"5088_CR63","doi-asserted-by":"crossref","unstructured":"Levine AP, Chan MMY, Sadeghi-Alavijeh O, Wong EKS, Cook HT, Ashford S, Carss K, Christian MT, Hall M, Harris CL, McAlinden P, Marchbank KJ, Marks SD, Maxwell H, Megy K, Penkett CJ, Mozere M, Stirrups KE, Tuna S, Wessels J, Whitehorn D, MPGN\/DDD\/C3 Glomerulopathy Rare Disease Group;\u00a0NIHR BioResource; Johnson SA, Gale DP (2020) Large-scale whole-genome sequencing reveals the genetic architecture of primary membranoproliferative GN and C3 glomerulopathy. J Am Soc Nephrol 31:365\u2013373","DOI":"10.1681\/ASN.2019040433"},{"key":"5088_CR64","doi-asserted-by":"publisher","first-page":"1287","DOI":"10.2215\/CJN.15241219","volume":"15","author":"F Caravaca-Fontan","year":"2020","unstructured":"Caravaca-Fontan F, Diaz-Encarnacion MM, Lucientes L, Cavero T, Cabello V, Ariceta G, Quintana LF, Marco H, Barros X, Ramos N, Rodriguez-Mendiola N, Cruz S, Fernandez-Juarez G, Rodriguez A, Perez de Jose A, Rabasco C, Rodado R, Fernandez L, Perez Gomez V, Avila AI, Bravo L, Lumbreras J, Allende N, Sanchez de la Nieta MD, Rodriguez E, Olea T, Melgosa M, Huerta A, Miquel R, Mon C, Fraga G, de Lorenzo A, Draibe J, Cano-Megias M, Gonzalez F, Shabaka A, Lopez-Rubio ME, Fenollosa MA, Martin-Penagos L, Da Silva I, Alonso Titos J, Rodriguez de Cordoba S, Goicoechea de Jorge E, Praga M, Spanish Group for the Study of Glomerular Diseases GLOSEN (2020) Mycophenolate mofetil in C3 glomerulopathy and pathogenic drivers of the disease. Clin J Am Soc Nephrol 15:1287\u20131298","journal-title":"Clin J Am Soc Nephrol"},{"key":"5088_CR65","doi-asserted-by":"publisher","first-page":"748","DOI":"10.2215\/CJN.12901211","volume":"7","author":"AS Bomback","year":"2012","unstructured":"Bomback AS, Smith RJ, Barile GR, Zhang Y, Heher EC, Herlitz L, Stokes MB, Markowitz GS, D'Agati VD, Canetta PA, Radhakrishnan J, Appel GB (2012) Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol 7:748\u2013756","journal-title":"Clin J Am Soc Nephrol"},{"key":"5088_CR66","doi-asserted-by":"publisher","first-page":"1975","DOI":"10.1007\/s00467-013-2503-y","volume":"28","author":"S Gurkan","year":"2013","unstructured":"Gurkan S, Fyfe B, Weiss L, Xiao X, Zhang Y, Smith RJ (2013) Eculizumab and recurrent C3 glomerulonephritis. Pediatr Nephrol 28:1975\u20131981","journal-title":"Pediatr Nephrol"},{"key":"5088_CR67","doi-asserted-by":"publisher","first-page":"484","DOI":"10.1053\/j.ajkd.2014.09.025","volume":"65","author":"M Le Quintrec","year":"2015","unstructured":"Le Quintrec M, Lionet A, Kandel C, Bourdon F, Gnemmi V, Colombat M, Goujon JM, Fremeaux-Bacchi V, Fakhouri F (2015) Eculizumab for treatment of rapidly progressive C3 glomerulopathy. Am J Kidney Dis 65:484\u2013489","journal-title":"Am J Kidney Dis"},{"key":"5088_CR68","doi-asserted-by":"publisher","first-page":"84","DOI":"10.1053\/j.ajkd.2017.11.019","volume":"72","author":"M Le Quintrec","year":"2018","unstructured":"Le Quintrec M, Lapeyraque AL, Lionet A, Sellier-Leclerc AL, Delmas Y, Baudouin V, Daugas E, Decramer S, Tricot L, Cailliez M, Dubot P, Servais A, Mourey-Epron C, Pourcine F, Loirat C, Fremeaux-Bacchi V, Fakhouri F (2018) Patterns of clinical response to eculizumab in patients with C3 glomerulopathy. Am J Kidney Dis 72:84\u201392","journal-title":"Am J Kidney Dis"},{"key":"5088_CR69","doi-asserted-by":"publisher","first-page":"1773","DOI":"10.2215\/CJN.01360215","volume":"10","author":"MJS Oosterveld","year":"2015","unstructured":"Oosterveld MJS, Garrelfs MR, Hoppe B, Florquin S, Roelofs JJTH, van den Heuvel LP, Amann K, Davin J-C, Bouts AHM, Schriemer PJ, Groothoff JW (2015) Eculizumab in pediatric dense deposit disease. Clin J Am Soc Nephrol 10:1773\u20131782","journal-title":"Clin J Am Soc Nephrol"},{"key":"5088_CR70","doi-asserted-by":"publisher","first-page":"224","DOI":"10.1053\/j.ajkd.2018.12.046","volume":"74","author":"P Ruggenenti","year":"2019","unstructured":"Ruggenenti P, Daina E, Gennarini A, Carrara C, Gamba S, Noris M, Rubis N, Peraro F, Gaspari F, Pasini A, Rigotti A, Lerchner RM, Santoro D, Pisani A, Pasi A, Remuzzi G, EAGLE Study Group (2019) C5 convertase blockade in membranoproliferative glomerulonephritis: a single-arm clinical trial. Am J Kidney Dis 74:224\u2013238","journal-title":"Am J Kidney Dis"},{"key":"5088_CR71","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1186\/s12882-017-0802-4","volume":"19","author":"T Welte","year":"2018","unstructured":"Welte T, Arnold F, Kappes J, Seidl M, Haffner K, Bergmann C, Walz G, Neumann-Haefelin E (2018) Treating C3 glomerulopathy with eculizumab. BMC Nephrol 19:7","journal-title":"BMC Nephrol"},{"key":"5088_CR72","doi-asserted-by":"publisher","first-page":"891","DOI":"10.1182\/blood-2017-05-781450","volume":"130","author":"M Konar","year":"2017","unstructured":"Konar M, Granoff DM (2017) Eculizumab treatment and impaired opsonophagocytic killing of meningococci by whole blood from immunized adults. Blood 130:891\u2013899","journal-title":"Blood"},{"key":"5088_CR73","doi-asserted-by":"publisher","first-page":"524","DOI":"10.1111\/j.1523-1755.2005.67109.x","volume":"67","author":"JM Thurman","year":"2005","unstructured":"Thurman JM, Lucia MS, Ljubanovic D, Holers VM (2005) Acute tubular necrosis is characterized by activation of the alternative pathway of complement. Kidney Int 67:524\u2013530","journal-title":"Kidney Int"},{"key":"5088_CR74","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1016\/S0161-5890(03)00105-6","volume":"40","author":"LA Trouw","year":"2003","unstructured":"Trouw LA, Seelen MA, Daha MR (2003) Complement and renal disease. Mol Immunol 40:125\u2013134","journal-title":"Mol Immunol"},{"key":"5088_CR75","doi-asserted-by":"publisher","first-page":"1052","DOI":"10.1126\/science.279.5353.1052","volume":"279","author":"R Clynes","year":"1998","unstructured":"Clynes R, Dumitru C, Ravetch JV (1998) Uncoupling of immune complex formation and kidney damage in autoimmune glomerulonephritis. Science 279:1052\u20131054","journal-title":"Science"},{"key":"5088_CR76","doi-asserted-by":"publisher","first-page":"415","DOI":"10.1016\/j.immuni.2005.02.006","volume":"22","author":"H Hawlisch","year":"2005","unstructured":"Hawlisch H, Belkaid Y, Baelder R, Hildeman D, Gerard C, Kohl J (2005) C5a negatively regulates toll-like receptor 4-induced immune responses. Immunity 22:415\u2013426","journal-title":"Immunity"},{"key":"5088_CR77","doi-asserted-by":"publisher","first-page":"228","DOI":"10.1182\/blood-2006-12-063636","volume":"110","author":"X Zhang","year":"2007","unstructured":"Zhang X, Kimura Y, Fang C, Zhou L, Sfyroera G, Lambris JD, Wetsel RA, Miwa T, Song WC (2007) Regulation of Toll-like receptor-mediated inflammatory response by complement in vivo. Blood 110:228\u2013236","journal-title":"Blood"},{"key":"5088_CR78","doi-asserted-by":"publisher","first-page":"1755","DOI":"10.4049\/jimmunol.143.6.1755","volume":"143","author":"RH Carter","year":"1989","unstructured":"Carter RH, Fearon DT (1989) Polymeric C3dg primes human B lymphocytes for proliferation induced by anti-IgM. J Immunol 143:1755\u20131760","journal-title":"J Immunol"},{"key":"5088_CR79","doi-asserted-by":"publisher","first-page":"745","DOI":"10.1189\/jlb.3MR0214-109R","volume":"96","author":"EV Clarke","year":"2014","unstructured":"Clarke EV, Tenner AJ (2014) Complement modulation of T cell immune responses during homeostasis and disease. J Leukoc Biol 96:745\u2013756","journal-title":"J Leukoc Biol"},{"key":"5088_CR80","doi-asserted-by":"publisher","first-page":"504","DOI":"10.3389\/fimmu.2019.00504","volume":"10","author":"DV Rizk","year":"2019","unstructured":"Rizk DV, Maillard N, Julian BA, Knoppova B, Green TJ, Novak J, Wyatt RJ (2019) The Emerging role of complement proteins as a target for therapy of IgA nephropathy. Front Immunol 10:504","journal-title":"Front Immunol"},{"key":"5088_CR81","doi-asserted-by":"publisher","first-page":"118","DOI":"10.1111\/cei.12754","volume":"184","author":"F Corvillo","year":"2016","unstructured":"Corvillo F, Bravo Garcia-Morato M, Nozal P, Garrido S, Tortajada A, Rodriguez de Cordoba S, Lopez-Trascasa M (2016) Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy. Clin Exp Immunol 184:118\u2013125","journal-title":"Clin Exp Immunol"}],"container-title":["Pediatric Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-021-05088-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00467-021-05088-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-021-05088-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,2,2]],"date-time":"2023-02-02T06:07:30Z","timestamp":1675318050000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00467-021-05088-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,5,18]]},"references-count":81,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2022,3]]}},"alternative-id":["5088"],"URL":"https:\/\/doi.org\/10.1007\/s00467-021-05088-7","relation":{},"ISSN":["0931-041X","1432-198X"],"issn-type":[{"value":"0931-041X","type":"print"},{"value":"1432-198X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,5,18]]},"assertion":[{"value":"24 January 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 March 2021","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 April 2021","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 May 2021","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"MV received funding from the Associazione per la cura del Bambino Nefropatico ONLUS and has served on advisory boards for Novartis, Apellis, Roche, Achillion, Retrophin and participated in the C3G clinical trials by Chemocentrix and Achillion. NK is member of the advisory board of Roche and participant in the clinical trials in C3G by Chemocentryx and Achillion. RL and FD: no funding or conflict of interest to report. JMT receives royalties from Alexion Pharmaceuticals, Inc. and is a consultant for Q32 Bio, Inc., a company developing complement inhibitors. He also holds stock and will receive royalty income from Q32 Bio, Inc. This does not influence the content of this manuscript.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}]}}