{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T19:52:33Z","timestamp":1791229953977,"version":"4.1.0"},"reference-count":55,"publisher":"Springer Science and Business Media LLC","issue":"10","license":[{"start":{"date-parts":[[2010,2,16]],"date-time":"2010-02-16T00:00:00Z","timestamp":1266278400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatr Nephrol"],"published-print":{"date-parts":[[2010,10]]},"DOI":"10.1007\/s00467-010-1446-9","type":"journal-article","created":{"date-parts":[[2010,2,15]],"date-time":"2010-02-15T06:49:29Z","timestamp":1266216569000},"page":"2009-2019","source":"Crossref","is-referenced-by-count":75,"title":["DEAP-HUS: Deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndrome"],"prefix":"10.1007","volume":"25","author":[{"name":"for the European DEAP-HUS Study Group","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter F.","family":"Zipfel","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Mache","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominik","family":"M\u00fcller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Licht","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianne","family":"Wigger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Skerka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2010,2,16]]},"reference":[{"key":"1446_CR1","doi-asserted-by":"crossref","first-page":"1512","DOI":"10.1182\/blood-2007-09-109876","volume":"111","author":"M Jozsi","year":"2008","unstructured":"Jozsi M, Licht C, Strobel S, Zipfel SL, Richter H, Heinen S, Zipfel PF, Skerka C (2008) Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1\/CFHR3 deficiency. Blood 111:1512\u20131514","journal-title":"Blood"},{"key":"1446_CR2","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1681\/ASN.2004050380","volume":"16","author":"MA Dragon-Durey","year":"2005","unstructured":"Dragon-Durey MA, Loirat C, Cloarec S, Macher MA, Blouin J, Nivet H, Weiss L, Fridman WH, Fremeaux-Bacchi V (2005) Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol 16:555\u2013563","journal-title":"J Am Soc Nephrol"},{"key":"1446_CR3","doi-asserted-by":"crossref","first-page":"e41","DOI":"10.1371\/journal.pgen.0030041","volume":"3","author":"PF Zipfel","year":"2007","unstructured":"Zipfel PF, Edey M, Heinen S, Jozsi M, Richter H, Misselwitz J, Hoppe B, Routledge D, Strain L, Hughes AE, Goodship JA, Licht C, Goodship TH, Skerka C (2007) Deletion of complement factor H-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome. PLoS Genet 3:e41","journal-title":"PLoS Genet"},{"key":"1446_CR4","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1160\/TH08-05-0322","volume":"101","author":"C Skerka","year":"2009","unstructured":"Skerka C, Jozsi M, Zipfel PF, Dragon-Durey MA, Fremeaux-Bacchi V (2009) Autoantibodies in haemolytic uraemic syndrome (HUS). Thromb Haemost 101:227\u2013232","journal-title":"Thromb Haemost"},{"key":"1446_CR5","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.1681\/ASN.2004100861","volume":"16","author":"M Noris","year":"2005","unstructured":"Noris M, Remuzzi G (2005) Hemolytic uremic syndrome. J Am Soc Nephrol 16:1035\u20131050","journal-title":"J Am Soc Nephrol"},{"key":"1446_CR6","doi-asserted-by":"crossref","first-page":"1957","DOI":"10.1007\/s00467-008-0872-4","volume":"23","author":"C Loirat","year":"2008","unstructured":"Loirat C, Noris M, Fremeaux-Bacchi V (2008) Complement and the atypical hemolytic uremic syndrome in children. Pediatr Nephrol 23:1957\u20131972","journal-title":"Pediatr Nephrol"},{"key":"1446_CR7","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1136\/jmg.2008.064766","volume":"46","author":"MA Dragon-Durey","year":"2009","unstructured":"Dragon-Durey MA, Blanc C, Marliot F, Loirat C, Blouin J, Sautes-Fridman C, Fridman WH, Fremeaux-Bacchi V (2009) The high frequency of complement factor H related CFHR1 gene deletion is restricted to specific subgroups of patients with atypical haemolytic uraemic syndrome. J Med Genet 46:447\u2013450","journal-title":"J Med Genet"},{"key":"1446_CR8","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1203\/PDR.0b013e3181b1bd4a","volume":"66","author":"BH Lee","year":"2009","unstructured":"Lee BH, Kwak SH, Shin JI, Lee SH, Choi HJ, Kang HG, Ha IS, Lee JS, Dragon-Durey MA, Choi Y, Cheong HI (2009) Atypical hemolytic uremic syndrome associated with complement factor H autoantibodies and CFHR1\/CFHR3 deficiency. Pediatr Res 66:336\u2013340","journal-title":"Pediatr Res"},{"key":"1446_CR9","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1182\/blood-2009-05-221549","volume":"115","author":"I Moore","year":"2010","unstructured":"Moore I, Strain L, Pappworth I, Kavanagh D, Barlow PN, Herbert AP, Schmidt CQ, Staniforth SJ, Holmes LV, Ward R, Morgan L, Goodship TH, Marchbank KJ (2010) Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4 and with mutations in CFH, CFI, CD46, and C3 in patients with atypical haemolytic uraemic syndrome. Blood 115:379\u2013387","journal-title":"Blood"},{"key":"1446_CR10","doi-asserted-by":"crossref","first-page":"4261","DOI":"10.1182\/blood-2009-05-223834","volume":"114","author":"C Abarrategui-Garrido","year":"2009","unstructured":"Abarrategui-Garrido C, Martinez-Barricarte R, Lopez-Trascasa M, Rodriguez de Cordoba S, Sanchez-Corral P (2009) Characterization of complement factor H-related (CFHR) proteins in plasma reveals novel genetic variations of CFHR1 associated with atypical hemolytic uremic syndrome. Blood 114:4261\u20134271","journal-title":"Blood"},{"key":"1446_CR11","doi-asserted-by":"crossref","first-page":"1516","DOI":"10.1182\/blood-2007-02-071472","volume":"110","author":"M Jozsi","year":"2007","unstructured":"Jozsi M, Strobel S, Dahse HM, Liu WS, Hoyer PF, Oppermann M, Skerka C, Zipfel PF (2007) Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome. Blood 110:1516\u20131518","journal-title":"Blood"},{"key":"1446_CR12","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1093\/ndt\/gfp388","volume":"25","author":"S Strobel","year":"2010","unstructured":"Strobel S, Hoyer PF, Mache C, Sulyok E, Liu W, Richter H, Oppermann M, Zipfel PF, Jozsi M (2010) Functional analyses indicate a pathogenic role of factor H autoantibodies in atypical haemolytic uraemic syndrome. Nephrol Dial Transplant 25:136\u2013144","journal-title":"Nephrol Dial Transplant"},{"key":"1446_CR13","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1111\/j.1365-2249.2006.03071.x","volume":"144","author":"M Oppermann","year":"2006","unstructured":"Oppermann M, Manuelian T, Jozsi M, Brandt E, Jokiranta TS, Heinen S, Meri S, Skerka C, Gotze O, Zipfel PF (2006) The C-terminus of complement regulator Factor H mediates target recognition: evidence for a compact conformation of the native protein. Clin Exp Immunol 144:342\u2013352","journal-title":"Clin Exp Immunol"},{"key":"1446_CR14","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1042\/bj3310041","volume":"331","author":"WM Prodinger","year":"1998","unstructured":"Prodinger WM, Hellwage J, Spruth M, Dierich MP, Zipfel PF (1998) The C-terminus of factor H: monoclonal antibodies inhibit heparin binding and identify epitopes common to factor H and factor H-related proteins. J Biochem 331:41\u201347","journal-title":"J Biochem"},{"key":"1446_CR15","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1681\/ASN.V122297","volume":"12","author":"J Caprioli","year":"2001","unstructured":"Caprioli J, Bettinaglio P, Zipfel PF, Amadei B, Daina E, Gamba S, Skerka C, Marziliano N, Remuzzi G, Noris M (2001) The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20. J Am Soc Nephrol 12:297\u2013307","journal-title":"J Am Soc Nephrol"},{"key":"1446_CR16","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1002\/humu.20435","volume":"28","author":"RE Saunders","year":"2007","unstructured":"Saunders RE, Abarrategui-Garrido C, Fremeaux-Bacchi V, Goicoechea de Jorge E, Goodship TH, Lopez Trascasa M, Noris M, Ponce Castro IM, Remuzzi G, Rodriguez de Cordoba S, Sanchez-Corral P, Skerka C, Zipfel PF, Perkins SJ (2007) The interactive Factor H-atypical hemolytic uremic syndrome mutation database and website: update and integration of membrane cofactor protein and Factor I mutations with structural models. Hum Mutat 28:222\u2013234","journal-title":"Hum Mutat"},{"key":"1446_CR17","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1086\/318203","volume":"68","author":"A Richards","year":"2001","unstructured":"Richards A, Buddles MR, Donne RL, Kaplan BS, Kirk E, Venning MC, Tielemans CL, Goodship JA, Goodship TH (2001) Factor H mutations in hemolytic uremic syndrome cluster in exons 18\u201320, a domain important for host cell recognition. Am J Hum Genet 68:485\u2013490","journal-title":"Am J Hum Genet"},{"key":"1446_CR18","doi-asserted-by":"crossref","first-page":"1285","DOI":"10.1086\/344515","volume":"71","author":"P Sanchez-Corral","year":"2002","unstructured":"Sanchez-Corral P, Perez-Caballero D, Huarte O, Simckes AM, Goicoechea E, Lopez-Trascasa M, de Cordoba SR (2002) Structural and functional characterization of factor H mutations associated with atypical hemolytic uremic syndrome. Am J Hum Genet 71:1285\u20131295","journal-title":"Am J Hum Genet"},{"key":"1446_CR19","doi-asserted-by":"crossref","first-page":"1181","DOI":"10.1172\/JCI16651","volume":"111","author":"T Manuelian","year":"2003","unstructured":"Manuelian T, Hellwage J, Meri S, Caprioli J, Noris M, Heinen S, Jozsi M, Neumann HP, Remuzzi G, Zipfel PF (2003) Mutations in factor H reduce binding affinity to C3b and heparin and surface attachment to endothelial cells in hemolytic uremic syndrome. J Clin Invest 111:1181\u20131190","journal-title":"J Clin Invest"},{"key":"1446_CR20","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1681\/ASN.2005080868","volume":"17","author":"M Jozsi","year":"2006","unstructured":"Jozsi M, Heinen S, Hartmann A, Ostrowicz CW, Halbich S, Richter H, Kunert A, Licht C, Saunders RE, Perkins SJ, Zipfel PF, Skerka C (2006) Factor H and atypical hemolytic uremic syndrome: mutations in the C-terminus cause structural changes and defective recognition functions. J Am Soc Nephrol 17:170\u2013177","journal-title":"J Am Soc Nephrol"},{"key":"1446_CR21","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1681\/ASN.2006091069","volume":"18","author":"S Heinen","year":"2007","unstructured":"Heinen S, Jozsi M, Hartmann A, Noris M, Remuzzi G, Skerka C, Zipfel PF (2007) Hemolytic uremic syndrome: a factor H mutation (E1172Stop) causes defective complement control at the surface of endothelial cells. J Am Soc Nephrol 18:506\u2013514","journal-title":"J Am Soc Nephrol"},{"key":"1446_CR22","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1681\/ASN.2007080923","volume":"19","author":"R Martinez-Barricarte","year":"2008","unstructured":"Martinez-Barricarte R, Pianetti G, Gautard R, Misselwitz J, Strain L, Fremeaux-Bacchi V, Skerka C, Zipfel PF, Goodship T, Noris M, Remuzzi G, de Cordoba SR (2008) The complement factor H R1210C mutation is associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol 19:639\u2013646","journal-title":"J Am Soc Nephrol"},{"key":"1446_CR23","doi-asserted-by":"crossref","first-page":"12015","DOI":"10.1016\/S0021-9258(18)99058-7","volume":"266","author":"C Skerka","year":"1991","unstructured":"Skerka C, Horstmann RD, Zipfel PF (1991) Molecular cloning of a human serum protein structurally related to complement factor H. J Biol Chem 266:12015\u201312020","journal-title":"J Biol Chem"},{"key":"1446_CR24","first-page":"19","volume":"26S","author":"CZ Skerka","year":"2008","unstructured":"Skerka CZ, Zipfel PF (2008) Complement factor H related proteins in immune diseases. Vaccines 26S:19\u2013114","journal-title":"Vaccines"},{"key":"1446_CR25","doi-asserted-by":"crossref","first-page":"e431","DOI":"10.1371\/journal.pmed.0030431","volume":"3","author":"JP Venables","year":"2006","unstructured":"Venables JP, Strain L, Routledge D, Bourn D, Powell HM, Warwicker P, Diaz-Torres ML, Sampson A, Mead P, Webb M, Pirson Y, Jackson MS, Hughes A, Wood KM, Goodship JA, Goodship TH (2006) Atypical haemolytic uraemic syndrome associated with a hybrid complement gene. PLoS Med 3:e431","journal-title":"PLoS Med"},{"key":"1446_CR26","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1016\/j.it.2008.04.008","volume":"29","author":"M Jozsi","year":"2008","unstructured":"Jozsi M, Zipfel PF (2008) Factor H family proteins and human diseases. Trends Immunol 29:380\u2013387","journal-title":"Trends Immunol"},{"key":"1446_CR27","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1038\/nri2620","volume":"9","author":"PF Zipfel","year":"2009","unstructured":"Zipfel PF, Skerka C (2009) Complement regulators and inhibitory proteins. Nat Rev Immunol 9:729\u2013740","journal-title":"Nat Rev Immunol"},{"key":"1446_CR28","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1016\/j.molimm.2004.02.005","volume":"41","author":"S Rodriguez de Cordoba","year":"2004","unstructured":"Rodriguez de Cordoba S, Esparza-Gordillo J, Goicoechea de Jorge E, Lopez-Trascasa M, Sanchez-Corral P (2004) The human complement factor H: functional roles, genetic variations and disease associations. Mol Immunol 41:355\u2013367","journal-title":"Mol Immunol"},{"key":"1446_CR29","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/S0167-5699(98)01432-7","volume":"20","author":"PF Zipfel","year":"1999","unstructured":"Zipfel PF, Skerka C (1999) FHL-1\/reconectin: a human complement and immune regulator with cell-adhesive function. Immunol Today 20:135\u2013140","journal-title":"Immunol Today"},{"key":"1446_CR30","doi-asserted-by":"crossref","first-page":"2904","DOI":"10.1016\/S0021-9258(18)53859-X","volume":"268","author":"C Skerka","year":"1993","unstructured":"Skerka C, Kuhn S, Gunther K, Lingelbach K, Zipfel PF (1993) A novel short consensus repeat-containing molecule is related to human complement factor H. J Biol Chem 268:2904\u20132908","journal-title":"J Biol Chem"},{"key":"1446_CR31","doi-asserted-by":"crossref","first-page":"5627","DOI":"10.1074\/jbc.272.9.5627","volume":"272","author":"C Skerka","year":"1997","unstructured":"Skerka C, Hellwage J, Weber W, Tilkorn A, Buck F, Marti T, Kampen E, Beisiegel U, Zipfel PF (1997) The human factor H-related protein 4 (FHR-4). A novel short consensus repeat-containing protein is associated with human triglyceride-rich lipoproteins. J Biol Chem 272:5627\u20135634","journal-title":"J Biol Chem"},{"key":"1446_CR32","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1038\/sj.ejhg.5201324","volume":"13","author":"M Jozsi","year":"2005","unstructured":"Jozsi M, Richter H, Loschmann I, Skerka C, Buck F, Beisiegel U, Erdei A, Zipfel PF (2005) FHR-4A: a new factor H-related protein is encoded by the human FHR-4 gene. Eur J Hum Genet 13:321\u2013329","journal-title":"Eur J Hum Genet"},{"key":"1446_CR33","doi-asserted-by":"crossref","first-page":"3313","DOI":"10.4049\/jimmunol.148.10.3313","volume":"148","author":"C Skerka","year":"1992","unstructured":"Skerka C, Timmann C, Horstmann RD, Zipfel PF (1992) Two additional human serum proteins structurally related to complement factor H. Evidence for a family of factor H-related genes. J Immunol 148:3313\u20133318","journal-title":"J Immunol"},{"key":"1446_CR34","doi-asserted-by":"crossref","first-page":"6250","DOI":"10.4049\/jimmunol.174.10.6250","volume":"174","author":"JL McRae","year":"2005","unstructured":"McRae JL, Duthy TG, Griggs KM, Ormsby RJ, Cowan PJ, Cromer BA, McKinstry WJ, Parker MW, Murphy BF, Gordon DL (2005) Human factor H-related protein 5 has cofactor activity, inhibits C3 convertase activity, binds heparin and C-reactive protein, and associates with lipoprotein. J Immunol 174:6250\u20136256","journal-title":"J Immunol"},{"key":"1446_CR35","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1007\/BF00355378","volume":"28","author":"S Rodriguez de Cordoba","year":"1988","unstructured":"Rodriguez de Cordoba S, Rey-Campos J, Dykes DD, McAlpine PJ, Wong P, Rubinstein P (1988) Coagulation factor XIII B subunit is encoded by a gene linked to the regulator of complement activation (RCA) gene cluster in man. Immunogenet 28:452\u2013454","journal-title":"Immunogenet"},{"key":"1446_CR36","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1016\/S0161-5890(99)00100-5","volume":"36","author":"S Rodriguez de Cordoba","year":"1999","unstructured":"Rodriguez de Cordoba S, Diaz-Guillen MA, Heine-Suner D (1999) An integrated map of the human regulator of complement activation (RCA) gene cluster on 1q32. Mol Immunol 36:803\u2013808","journal-title":"Mol Immunol"},{"key":"1446_CR37","doi-asserted-by":"crossref","first-page":"592","DOI":"10.1080\/07853890601097030","volume":"38","author":"GS Hageman","year":"2006","unstructured":"Hageman GS, Hancox LS, Taiber AJ, Gehrs KM, Anderson DH, Johnson LV, Radeke MJ, Kavanagh D, Richards A, Atkinson J, Meri S, Bergeron J, Zernant J, Merriam J, Gold B, Allikmets R, Dean M (2006) Extended haplotypes in the complement factor H (CFH) and CFH-related (CFHR) family of genes protect against age-related macular degeneration: characterization, ethnic distribution and evolutionary implications. Ann Med 38:592\u2013604","journal-title":"Ann Med"},{"key":"1446_CR38","doi-asserted-by":"crossref","first-page":"2439","DOI":"10.1182\/blood-2009-02-205641","volume":"114","author":"S Heinen","year":"2009","unstructured":"Heinen S, Hartmann A, Lauer N, Wiehl U, Dahse H-M, Schirmer S, Gropp K, Enghardt T, Wallich R, H\u00e4lbich S, Mihlan M, Sch\u00f6tzer-Schrehardt U, Zipfel PF, Skerka C (2009) Factor H related protein 1 (CFHR-1) inhibits complement C5 convertase activity and terminal complex formation. Blood 114:2439\u20132447","journal-title":"Blood"},{"key":"1446_CR39","doi-asserted-by":"crossref","first-page":"2236","DOI":"10.1016\/j.molimm.2009.04.012","volume":"46","author":"AL Stahl","year":"2009","unstructured":"Stahl AL, Kristoffersson A, Olin AI, Olsson ML, Roodhooft AM, Proesmans W, Karpman D (2009) A novel mutation in the complement regulator clusterin in recurrent hemolytic uremic syndrome. Mol Immunol 46:2236\u20132243","journal-title":"Mol Immunol"},{"key":"1446_CR40","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1086\/518509","volume":"196","author":"K Haupt","year":"2007","unstructured":"Haupt K, Kraiczy P, Wallich R, Brade V, Skerka C, Zipfel PF (2007) Binding of human factor H-related protein 1 to serum-resistant Borrelia burgdorferi is mediated by borrelial complement regulator-acquiring surface proteins. J Infect Dis 196:124\u2013133","journal-title":"J Infect Dis"},{"key":"1446_CR41","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1016\/S0014-5793(99)01554-9","volume":"462","author":"J Hellwage","year":"1999","unstructured":"Hellwage J, Jokiranta TS, Koistinen V, Vaarala O, Meri S, Zipfel PF (1999) Functional properties of complement factor H-related proteins FHR-3 and FHR-4: binding to the C3d region of C3b and differential regulation by heparin. FEBS Lett 462:345\u2013352","journal-title":"FEBS Lett"},{"key":"1446_CR42","doi-asserted-by":"crossref","first-page":"2752","DOI":"10.1093\/ndt\/gfp166","volume":"24","author":"T Kwon","year":"2009","unstructured":"Kwon T, Belot A, Ranchin B, Baudouin V, Fremeaux-Bacchi V, Dragon-Durey MA, Cochat P, Loirat C (2009) Varicella as a trigger of atypical haemolytic uraemic syndrome associated with complement dysfunction: two cases. Nephrol Dial Transplant 24:2752\u20132754","journal-title":"Nephrol Dial Transplant"},{"key":"1446_CR43","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1007\/s00467-008-0964-1","volume":"24","author":"G Ariceta","year":"2009","unstructured":"Ariceta G, Besbas N, Johnson S, Karpman D, Landau D, Licht C, Loirat C, Pecoraro C, Taylor CM, Van de Kar N, Vandewalle J, Zimmerhackl LB (2009) Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol 24:687\u2013696","journal-title":"Pediatr Nephrol"},{"key":"1446_CR44","author":"CM Taylor","year":"2010","unstructured":"Taylor CM, Machin S, Wigmore SJ, Goodship TH (2010) Clinical practice guidelines for the management of atypical haemolytic uraemic syndrome in the United Kingdom. Br J Haematol 148:37\u201347","journal-title":"Br J Haematol"},{"key":"1446_CR45","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1160\/TH07-12-0753","volume":"101","author":"E Bresin","year":"2009","unstructured":"Bresin E, Gastoldi S, Daina E, Belotti D, Pogliani E, Perseghin P, Scalzulli PR, Paolini R, Marceno R, Remuzzi G, Galbusera M (2009) Rituximab as pre-emptive treatment in patients with thrombotic thrombocytopenic purpura and evidence of anti-ADAMTS13 autoantibodies. Thromb Haemost 101:233\u2013238","journal-title":"Thromb Haemost"},{"key":"1446_CR46","doi-asserted-by":"crossref","first-page":"1262","DOI":"10.1182\/blood-2004-11-4490","volume":"106","author":"M Rieger","year":"2005","unstructured":"Rieger M, Mannucci PM, Kremer Hovinga JA, Herzog A, Gerstenbauer G, Konetschny C, Zimmermann K, Scharrer I, Peyvandi F, Galbusera M, Remuzzi G, Bohm M, Plaimauer B, Lammle B, Scheiflinger F (2005) ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood 106:1262\u20131267","journal-title":"Blood"},{"key":"1446_CR47","doi-asserted-by":"crossref","first-page":"3823","DOI":"10.1016\/j.molimm.2007.06.151","volume":"44","author":"MJ Glennie","year":"2007","unstructured":"Glennie MJ, French RR, Cragg MS, Taylor RP (2007) Mechanisms of killing by anti-CD20 monoclonal antibodies. Mol Immunol 44:3823\u20133837","journal-title":"Mol Immunol"},{"key":"1446_CR48","doi-asserted-by":"crossref","unstructured":"Hillmen P (2008) The role of complement inhibition in PNH hematology. Am Soc Hematol Educ Program, pp 116\u2013123","DOI":"10.1182\/asheducation-2008.1.116"},{"key":"1446_CR49","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1056\/NEJMc0809959","volume":"360","author":"RA Gruppo","year":"2009","unstructured":"Gruppo RA, Rother RP (2009) Eculizumab for congenital atypical hemolytic-uremic syndrome. N Engl J Med 360:544\u2013546","journal-title":"N Engl J Med"},{"key":"1446_CR50","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1056\/NEJMc0808527","volume":"360","author":"J Nurnberger","year":"2009","unstructured":"Nurnberger J, Philipp T, Witzke O, Opazo Saez A, Vester U, Baba HA, Kribben A, Zimmerhackl LB, Janecke AR, Nagel M, Kirschfink M (2009) Eculizumab for atypical hemolytic-uremic syndrome. N Engl J Med 360:542\u2013544","journal-title":"N Engl J Med"},{"key":"1446_CR51","doi-asserted-by":"crossref","first-page":"1312","DOI":"10.2215\/CJN.01090209","volume":"4","author":"CJ Mache","year":"2009","unstructured":"Mache CJ, Acham-Roschitz B, Fremeaux-Bacchi V, Kirschfink M, Zipfel PF, Roedl S, Vester U, Ring E (2009) Complement inhibitor eculizumab in atypical hemolytic uremic syndrome. Clin J Am Soc Nephrol 4:1312\u20131316","journal-title":"Clin J Am Soc Nephrol"},{"key":"1446_CR52","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.1111\/j.1600-6143.2009.02586.x","volume":"9","author":"M Quintrec Le","year":"2009","unstructured":"Le Quintrec M, Zuber J, Noel LH, Thervet E, Fremeaux-Bacchi V, Niaudet P, Fridman WH, Legendre C, Dragon-Durey MA (2009) Anti-Factor H autoantibodies in a fifth renal transplant recipient with atypical hemolytic and uremic syndrome. Am J Transplant 9:1223\u20131229","journal-title":"Am J Transplant"},{"key":"1446_CR53","doi-asserted-by":"crossref","first-page":"2088","DOI":"10.1093\/ndt\/gfn063","volume":"23","author":"T Kwon","year":"2008","unstructured":"Kwon T, Dragon-Durey MA, Macher MA, Baudouin V, Maisin A, Peuchmaur M, Fremeaux-Bacchi V, Loirat C (2008) Successful pre-transplant management of a patient with anti-factor H autoantibodies-associated haemolytic uraemic syndrome. Nephrol Dial Transplant 23:2088\u20132090","journal-title":"Nephrol Dial Transplant"},{"key":"1446_CR54","doi-asserted-by":"crossref","first-page":"2801","DOI":"10.1016\/j.molimm.2009.05.018","volume":"46","author":"C Skerka","year":"2009","unstructured":"Skerka C, Licht C, Mengel M, Uzonyi B, Strobel S, Zipfel PF, Jozsi M (2009) Autoimmune forms of thrombotic micorangiopathy and membranoproliferative glomerulonephritis: Indications for a disease spectrum and common pathogenic principles. Mol Immunol 46:2801\u20132807","journal-title":"Mol Immunol"},{"key":"1446_CR55","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1056\/NEJMoa0810739","volume":"361","author":"M Delvaeye","year":"2009","unstructured":"Delvaeye M, Noris M, De Vriese A, Esmon CT, Esmon NL, Ferrell G, Del-Favero J, Plaisance S, Claes B, Lambrechts D, Zoja C, Remuzzi G, Conway EM (2009) Thrombomodulin mutations in atypical hemolytic-uremic syndrome. N Engl J Med 361:345\u2013357","journal-title":"N Engl J Med"}],"container-title":["Pediatric Nephrology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-010-1446-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00467-010-1446-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-010-1446-9","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,30]],"date-time":"2023-05-30T07:18:59Z","timestamp":1685431139000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00467-010-1446-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,2,16]]},"references-count":55,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2010,10]]}},"alternative-id":["1446"],"URL":"https:\/\/doi.org\/10.1007\/s00467-010-1446-9","relation":{},"ISSN":["0931-041X","1432-198X"],"issn-type":[{"value":"0931-041X","type":"print"},{"value":"1432-198X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,2,16]]}}}