{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,6]],"date-time":"2026-04-06T09:02:45Z","timestamp":1775466165430,"version":"3.50.1"},"reference-count":103,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2010,7,1]],"date-time":"2010-07-01T00:00:00Z","timestamp":1277942400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Seminars in Nephrology"],"published-print":{"date-parts":[[2010,7]]},"DOI":"10.1016\/j.semnephrol.2010.06.006","type":"journal-article","created":{"date-parts":[[2010,9,1]],"date-time":"2010-09-01T09:32:06Z","timestamp":1283333526000},"page":"395-408","source":"Crossref","is-referenced-by-count":27,"title":["Genetics and Genetic Testing in Hemolytic Uremic Syndrome\/Thrombotic Thrombocytopenic Purpura"],"prefix":"10.1016","volume":"30","author":[{"given":"Marina","family":"Noris","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuseppe","family":"Remuzzi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.semnephrol.2010.06.006_bib1","series-title":"Therapy in nephrology & hypertension a companion to Brenner & Rector's the kidney","first-page":"294","article-title":"Thrombotic microangiopathies","author":"Ruggenenti","year":"2008"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib2","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1038\/ki.1987.206","article-title":"HUS and TTP: variable expression of a single entity","volume":"32","author":"Remuzzi","year":"1987","journal-title":"Kidney Int"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib3","doi-asserted-by":"crossref","first-page":"1676","DOI":"10.1056\/NEJMra0902814","article-title":"Atypical hemolytic-uremic syndrome","volume":"361","author":"Noris","year":"2009","journal-title":"N Engl J Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib4","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1038\/sj.ki.5001581","article-title":"A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders","volume":"70","author":"Besbas","year":"2006","journal-title":"Kidney Int"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib5","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1160\/TH08-05-0317","article-title":"Shiga toxins, glycosphingolipid diversity, and endothelial cell injury","volume":"101","author":"Muthing","year":"2009","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib6","doi-asserted-by":"crossref","first-page":"1460","DOI":"10.4049\/jimmunol.181.2.1460","article-title":"Fractalkine and CX3CR1 mediate leukocyte capture by endothelium in response to Shiga toxin","volume":"181","author":"Zanchi","year":"2008","journal-title":"J Immunol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib7","doi-asserted-by":"crossref","first-page":"1207","DOI":"10.1016\/S0140-6736(98)01267-7","article-title":"Escherichia coli O157:H7","volume":"352","author":"Mead","year":"1998","journal-title":"Lancet"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib8","doi-asserted-by":"crossref","first-page":"1063","DOI":"10.1086\/319269","article-title":"The United States National Prospective Hemolytic Uremic Syndrome Study: microbiologic, serologic, clinical, and epidemiologic findings","volume":"183","author":"Banatvala","year":"2001","journal-title":"J Infect Dis"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib9","doi-asserted-by":"crossref","first-page":"1360","DOI":"10.1001\/jama.290.10.1360","article-title":"Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression","volume":"290","author":"Garg","year":"2003","journal-title":"JAMA"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib10","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1053\/j.ajkd.2004.02.010","article-title":"Non-enteropathic hemolytic uremic syndrome: causes and short-term course","volume":"43","author":"Constantinescu","year":"2004","journal-title":"Am J Kidney Dis"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib11","doi-asserted-by":"crossref","first-page":"1951","DOI":"10.1007\/s00467-007-0518-y","article-title":"Streptococcus pneumoniae-associated hemolytic uremic syndrome","volume":"23","author":"Copelovitch","year":"2008","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib12","doi-asserted-by":"crossref","first-page":"1126","DOI":"10.1681\/ASN.V961126","article-title":"The pathogenesis and treatment of hemolytic uremic syndrome","volume":"9","author":"Kaplan","year":"1998","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib13","doi-asserted-by":"crossref","first-page":"654","DOI":"10.1136\/adc.40.214.654","article-title":"Fatal haemolytic uraemic syndrome and idiopathic hyperlipaemia in monozygotic twins","volume":"40","author":"Campbell","year":"1965","journal-title":"Arch Dis Child"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib14","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1007\/s004670050793","article-title":"Autosomal dominant hemolytic uremic syndrome: variable phenotypes and transplant results","volume":"14","author":"Kaplan","year":"2000","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib15","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1055\/s-2005-925474","article-title":"Drug-induced thrombotic microangiopathy","volume":"31","author":"Zakarija","year":"2005","journal-title":"Semin Thromb Hemost"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib16","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1046\/j.1523-1755.2002.00543.x","article-title":"Post-transplant hemolytic-uremic syndrome","volume":"62","author":"Ruggenenti","year":"2002","journal-title":"Kidney Int"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib17","doi-asserted-by":"crossref","first-page":"1289","DOI":"10.1046\/j.1600-6143.2003.00222.x","article-title":"Outcome of plasma exchange therapy in thrombotic microangiopathy after renal transplantation","volume":"3","author":"Karthikeyan","year":"2003","journal-title":"Am J Transplant"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib18","doi-asserted-by":"crossref","first-page":"2457","DOI":"10.1046\/j.1523-1755.1999.00492.x","article-title":"Cyclosporine-associated thrombotic microangiopathy in renal allografts","volume":"55","author":"Zarifian","year":"1999","journal-title":"Kidney Int"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib19","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1182\/blood-2005-10-007252","article-title":"Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome","volume":"108","author":"Caprioli","year":"2006","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib20","doi-asserted-by":"crossref","first-page":"1694","DOI":"10.1111\/j.1600-6143.2008.02297.x","article-title":"Complement mutation-associated de novo thrombotic microangiopathy following kidney transplantation","volume":"8","author":"Le Quintrec","year":"2008","journal-title":"Am J Transplant"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib21","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1016\/S0140-6736(74)90991-X","article-title":"Letter: haemolytic-uraemic syndrome: evidence for intravascular C3 activation","volume":"2","author":"Stuhlinger","year":"1974","journal-title":"Lancet"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib22","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1681\/ASN.V102281","article-title":"Hypocomplementemia discloses genetic predisposition to hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: role of factor H abnormalities","volume":"10","author":"Noris","year":"1999","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib23","first-page":"845","article-title":"Breakdown products of C3 and factor B in hemolytic-uremic syndrome","volume":"89","author":"Kim","year":"1977","journal-title":"J Lab Clin Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib24","first-page":"193","article-title":"Hemolytic uremic syndrome with hypocomplementemia and deposits of IgM and C3 in the involved renal tissue","volume":"16","author":"Gonzalo","year":"1981","journal-title":"Clin Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib25","doi-asserted-by":"crossref","first-page":"1058","DOI":"10.1056\/NEJM200104053441406","article-title":"Complement","volume":"344","author":"Walport","year":"2001","journal-title":"N Engl J Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib26","first-page":"110","article-title":"Hypocomplementaemia due to a genetic deficiency of beta 1H globulin","volume":"46","author":"Thompson","year":"1981","journal-title":"Clin Exp Immunol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib27","doi-asserted-by":"crossref","first-page":"2318","DOI":"10.1681\/ASN.V9122318","article-title":"Human complement factor H deficiency associated with hemolytic uremic syndrome","volume":"9","author":"Rougier","year":"1998","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib28","doi-asserted-by":"crossref","first-page":"836","DOI":"10.1111\/j.1523-1755.1998.00824.x","article-title":"Genetic studies into inherited and sporadic hemolytic uremic syndrome","volume":"53","author":"Warwicker","year":"1998","journal-title":"Kidney Int"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib29","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1086\/318203","article-title":"Factor H mutations in hemolytic uremic syndrome cluster in exons 18-20, a domain important for host cell recognition","volume":"68","author":"Richards","year":"2001","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib30","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1097\/01.ASN.0000115702.28859.A7","article-title":"Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases","volume":"15","author":"Dragon-Durey","year":"2004","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib31","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1681\/ASN.V122297","article-title":"The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20","volume":"12","author":"Caprioli","year":"2001","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib32","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1086\/318201","article-title":"Clustering of missense mutations in the C-terminal region of factor H in atypical hemolytic uremic syndrome","volume":"68","author":"Perez-Caballero","year":"2001","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib33","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/0167-5699(94)90155-4","article-title":"Complement factor H and related proteins: an expanding family of complement-regulatory proteins?","volume":"15","author":"Zipfel","year":"1994","journal-title":"Immunol Today"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib34","doi-asserted-by":"crossref","first-page":"1784","DOI":"10.1038\/sj.emboj.7601052","article-title":"Structure of complement factor H carboxyl-terminus reveals molecular basis of atypical haemolytic uremic syndrome","volume":"25","author":"Jokiranta","year":"2006","journal-title":"EMBO J"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib35","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1681\/ASN.2006091069","article-title":"Hemolytic uremic syndrome: a factor H mutation (E1172Stop) causes defective complement control at the surface of endothelial cells","volume":"18","author":"Heinen","year":"2007","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib36","doi-asserted-by":"crossref","first-page":"1181","DOI":"10.1172\/JCI16651","article-title":"Mutations in factor H reduce binding affinity to C3b and heparin and surface attachment to endothelial cells in hemolytic uremic syndrome","volume":"111","author":"Manuelian","year":"2003","journal-title":"J Clin Invest"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib37","doi-asserted-by":"crossref","first-page":"e431","DOI":"10.1371\/journal.pmed.0030431","article-title":"Atypical haemolytic uraemic syndrome associated with a hybrid complement gene","volume":"3","author":"Venables","year":"2006","journal-title":"PLoS Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib38","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1681\/ASN.2004050380","article-title":"Anti-factor H autoantibodies associated with atypical hemolytic uremic syndrome","volume":"16","author":"Dragon-Durey","year":"2005","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib39","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1160\/TH08-05-0322","article-title":"Autoantibodies in haemolytic uraemic syndrome (HUS)","volume":"101","author":"Skerka","year":"2009","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib40","doi-asserted-by":"crossref","first-page":"1516","DOI":"10.1182\/blood-2007-02-071472","article-title":"Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome","volume":"110","author":"Jozsi","year":"2007","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib41","doi-asserted-by":"crossref","first-page":"1512","DOI":"10.1182\/blood-2007-09-109876","article-title":"Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1\/CFHR3 deficiency","volume":"111","author":"Jozsi","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib42","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1136\/jmg.2008.064766","article-title":"The high frequency of Complement factor H-related CFHR1 gene deletion is restricted to specific subgroups of patients with atypical haemolytic uraemic syndrome","volume":"46","author":"Dragon-Durey","year":"2009","journal-title":"J Med Genet"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib43","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1182\/blood-2009-05-221549","article-title":"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","volume":"115","author":"Moore","year":"2009","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib44","doi-asserted-by":"crossref","first-page":"4261","DOI":"10.1182\/blood-2009-05-223834","article-title":"Characterization of complement factor H-related (CFHR) proteins in plasma reveals novel genetic variations of CFHR1 associated with atypical hemolytic uremic syndrome","volume":"114","author":"Abarrategui-Garrido","year":"2009","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib45","doi-asserted-by":"crossref","first-page":"1542","DOI":"10.1016\/S0140-6736(03)14742-3","article-title":"Familial haemolytic uraemic syndrome and an MCP mutation","volume":"362","author":"Noris","year":"2003","journal-title":"Lancet"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib46","doi-asserted-by":"crossref","first-page":"12966","DOI":"10.1073\/pnas.2135497100","article-title":"Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome","volume":"100","author":"Richards","year":"2003","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib47","doi-asserted-by":"crossref","first-page":"2017","DOI":"10.1681\/ASN.2005101051","article-title":"Genetic and functional analyses of membrane cofactor protein (CD46) mutations in atypical hemolytic uremic syndrome","volume":"17","author":"Fremeaux-Bacchi","year":"2006","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib48","doi-asserted-by":"crossref","first-page":"2150","DOI":"10.1681\/ASN.2005010103","article-title":"Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome","volume":"16","author":"Kavanagh","year":"2005","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib49","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/j.molimm.2007.05.004","article-title":"Characterization of mutations in complement factor I (CFI) associated with hemolytic uremic syndrome","volume":"45","author":"Kavanagh","year":"2008","journal-title":"Mol Immunol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib50","doi-asserted-by":"crossref","first-page":"e84","DOI":"10.1136\/jmg.2004.019083","article-title":"Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome","volume":"41","author":"Fremeaux-Bacchi","year":"2004","journal-title":"J Med Genet"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib51","doi-asserted-by":"crossref","first-page":"4948","DOI":"10.1182\/blood-2008-01-133702","article-title":"Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome","volume":"112","author":"Fremeaux-Bacchi","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib52","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1073\/pnas.0603420103","article-title":"Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome","volume":"104","author":"Goicoechea de Jorge","year":"2007","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib53","doi-asserted-by":"crossref","first-page":"2837","DOI":"10.1182\/blood-2009-01-197640","article-title":"Hyperfunctional C3 convertase leads to complement deposition on endothelial cells and contributes to atypical hemolytic uremic syndrome","volume":"114","author":"Roumenina","year":"2009","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib54","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1056\/NEJMoa0810739","article-title":"Thrombomodulin mutations in atypical hemolytic-uremic syndrome","volume":"361","author":"Delvaeye","year":"2009","journal-title":"N Engl J Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib55","doi-asserted-by":"crossref","first-page":"1957","DOI":"10.1007\/s00467-008-0872-4","article-title":"Complement and the atypical hemolytic uremic syndrome in children","volume":"23","author":"Loirat","year":"2008","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib56","doi-asserted-by":"crossref","first-page":"1356","DOI":"10.2215\/CJN.06281208","article-title":"A large family with a gain-of-function mutation of complement C3 predisposing to atypical hemolytic uremic syndrome, microhematuria, hypertension and chronic renal failure","volume":"4","author":"Lhotta","year":"2009","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib57","doi-asserted-by":"crossref","first-page":"1676","DOI":"10.1056\/NEJMra0902814","article-title":"Atypical hemolytic uremic syndrome","volume":"361","author":"Noris","year":"2009","journal-title":"N Engl J Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib58","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1053\/j.ajkd.2006.10.022","article-title":"Unusual clinical severity of complement membrane cofactor protein-associated hemolytic-uremic syndrome and uniparental isodisomy","volume":"49","author":"Fremeaux-Bacchi","year":"2007","journal-title":"Am J Kidney Dis"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib59","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1007\/s00467-007-0438-x","article-title":"Complete factor H deficiency-associated atypical hemolytic uremic syndrome in a neonate","volume":"22","author":"Cho","year":"2007","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib60","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1053\/j.ajkd.2004.10.018","article-title":"Successful plasma therapy for atypical hemolytic uremic syndrome caused by factor H deficiency owing to a novel mutation in the complement cofactor protein domain 15","volume":"45","author":"Licht","year":"2005","journal-title":"Am J Kidney Dis"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib61","doi-asserted-by":"crossref","first-page":"1769","DOI":"10.1007\/s00467-006-0237-9","article-title":"Secondary failure of plasma therapy in factor H deficiency","volume":"21","author":"Nathanson","year":"2006","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib62","doi-asserted-by":"crossref","first-page":"1517","DOI":"10.1007\/s00467-008-0833-y","article-title":"Plasma therapy in atypical haemolytic uremic syndrome: lessons from a family with a factor H mutation","volume":"23","author":"Davin","year":"2008","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib63","doi-asserted-by":"crossref","first-page":"1363","DOI":"10.1007\/s00467-008-0803-4","article-title":"Efficacy of plasma therapy in atypical hemolytic uremic syndrome with complement factor H mutations","volume":"23","author":"Lapeyraque","year":"2008","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib64","doi-asserted-by":"crossref","first-page":"2088","DOI":"10.1093\/ndt\/gfn063","article-title":"Successful pre-transplant management of a patient with anti-factor H autoantibodies-associated haemolytic uraemic syndrome","volume":"23","author":"Kwon","year":"2008","journal-title":"Nephrol Dial Transplant"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib65","doi-asserted-by":"crossref","first-page":"2392","DOI":"10.1681\/ASN.2006080811","article-title":"Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome","volume":"18","author":"Sellier-Leclerc","year":"2007","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib66","doi-asserted-by":"crossref","first-page":"1757","DOI":"10.1007\/s00467-009-1188-8","article-title":"Prophylactic plasma exchange in CD46-associated atypical haemolytic uremic syndrome","volume":"24","author":"Davin","year":"2009","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib67","doi-asserted-by":"crossref","first-page":"88","DOI":"10.2215\/CJN.00050505","article-title":"Outcome of renal transplantation in patients with non-Shiga toxin-associated haemolytic uremic syndrome: prognostic significance of genetic background","volume":"1","author":"Bresin","year":"2006","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib68","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1111\/j.1399-3046.2008.00910.x","article-title":"Hemolytic uremic syndrome recurrence after renal transplantation","volume":"12","author":"Loirat","year":"2008","journal-title":"Pediatr Transplant"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib69","doi-asserted-by":"crossref","first-page":"3548","DOI":"10.1093\/ndt\/gfp377","article-title":"Haemolytic uraemic syndrome caused by factor H mutation: is single kidney transplantation under intensive plasmatherapy an option?","volume":"24","author":"Hirt-Minkowski","year":"2009","journal-title":"Nephrol Dial Transplant"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib70","doi-asserted-by":"crossref","first-page":"1173","DOI":"10.1007\/s00467-004-1565-2","article-title":"Atypical relapse of hemolytic uremic syndrome after transplantation","volume":"19","author":"Olie","year":"2004","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib71","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1053\/j.ajkd.2008.06.027","article-title":"Recurrent atypical hemolytic uremic syndrome associated with factor I mutation in a living related renal transplant recipient","volume":"53","author":"Chan","year":"2009","journal-title":"Am J Kidney Dis"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib72","doi-asserted-by":"crossref","first-page":"E22","DOI":"10.1053\/ajkd.2002.36938","article-title":"Recurrence of hemolytic uremic syndrome after live related renal transplantation associated with subsequent de novo disease in the donor","volume":"40","author":"Donne","year":"2002","journal-title":"Am J Kidney Dis"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib73","doi-asserted-by":"crossref","first-page":"1671","DOI":"10.1016\/S0140-6736(02)08560-4","article-title":"Combined kidney and liver transplantation for familial haemolytic uraemic syndrome","volume":"359","author":"Remuzzi","year":"2002","journal-title":"Lancet"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib74","doi-asserted-by":"crossref","first-page":"1146","DOI":"10.1111\/j.1600-6143.2005.00783.x","article-title":"Hemolytic uremic syndrome: a fatal outcome after kidney and liver transplantation performed to correct factor h gene mutation","volume":"5","author":"Remuzzi","year":"2005","journal-title":"Am J Transplant"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib75","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1111\/j.1600-6143.2007.02029.x","article-title":"Successful liver-kidney transplantation in two children with aHUS caused by a mutation in complement factor H","volume":"8","author":"Jalanko","year":"2008","journal-title":"Am J Transplant"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib76","doi-asserted-by":"crossref","first-page":"940","DOI":"10.1681\/ASN.2008080906","article-title":"Liver-kidney transplantation to cure atypical hemolytic uremic syndrome","volume":"20","author":"Saland","year":"2009","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib77","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1055\/s-2006-939763","article-title":"Thrombotic thrombocytopenic purpura\u2014then and now","volume":"32","author":"Galbusera","year":"2006","journal-title":"Semin Thromb Hemost"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib78","doi-asserted-by":"crossref","first-page":"1927","DOI":"10.1056\/NEJMcp053024","article-title":"Clinical practice","volume":"354","author":"George","year":"2006","journal-title":"N Engl J Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib79","first-page":"352","article-title":"An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries: an undescribed disease","volume":"70","author":"Moschcowitz","year":"2003","journal-title":"Mt Sinai J Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib80","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1182\/blood-2008-02-078170","article-title":"Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura","volume":"112","author":"Sadler","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib81","doi-asserted-by":"crossref","first-page":"4223","DOI":"10.1182\/blood.V87.10.4223.bloodjournal87104223","article-title":"Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis","volume":"87","author":"Furlan","year":"1996","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib82","doi-asserted-by":"crossref","first-page":"1585","DOI":"10.1056\/NEJM199811263392203","article-title":"Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura","volume":"339","author":"Tsai","year":"1998","journal-title":"N Engl J Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib83","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1038\/35097008","article-title":"Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura","volume":"413","author":"Levy","year":"2001","journal-title":"Nature"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib84","doi-asserted-by":"crossref","first-page":"1578","DOI":"10.1056\/NEJM199811263392202","article-title":"von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome","volume":"339","author":"Furlan","year":"1998","journal-title":"N Engl J Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib85","doi-asserted-by":"crossref","first-page":"1765","DOI":"10.1182\/blood.V98.6.1765","article-title":"Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases","volume":"98","author":"Veyradier","year":"2001","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib86","doi-asserted-by":"crossref","first-page":"2815","DOI":"10.1182\/blood-2006-02-006064","article-title":"Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity","volume":"109","author":"Ferrari","year":"2007","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib87","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1146\/annurev.pathmechdis.3.121806.154311","article-title":"Pathogenesis of thrombotic microangiopathies","volume":"3","author":"Zheng","year":"2008","journal-title":"Annu Rev Pathol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib88","doi-asserted-by":"crossref","first-page":"3232","DOI":"10.1182\/blood-2003-03-0908","article-title":"ADAMTS-13 cysteine-rich\/spacer domains are functionally essential for von Willebrand factor cleavage","volume":"102","author":"Soejima","year":"2003","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib89","doi-asserted-by":"crossref","first-page":"4514","DOI":"10.1182\/blood-2003-12-4165","article-title":"Epitope mapping of ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura","volume":"103","author":"Klaus","year":"2004","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib90","doi-asserted-by":"crossref","first-page":"166","DOI":"10.3324\/haematol.2008.002493","article-title":"Inherited thrombotic thrombocytopenic purpura","volume":"94","author":"Galbusera","year":"2009","journal-title":"Haematologica"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib91","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1160\/TH06-05-0236","article-title":"In-vitro and in-vivo consequences of mutations in the von Willebrand factor cleaving protease ADAMTS13 in thrombotic thrombocytopenic purpura","volume":"96","author":"Donadelli","year":"2006","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib92","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1182\/asheducation-2007.1.121","article-title":"TTP and ADAMTS13: when is testing appropriate?","author":"Mannucci","year":"2007","journal-title":"Hematology Am Soc Hematol Educ Program"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib93","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1111\/j.1537-2995.2008.02060.x","article-title":"ADAMTS13: what it does, how it works, and why it's important","volume":"49","author":"George","year":"2009","journal-title":"Transfusion"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib94","doi-asserted-by":"crossref","first-page":"778","DOI":"10.1182\/blood-2001-12-0166","article-title":"von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome","volume":"100","author":"Remuzzi","year":"2002","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib95","doi-asserted-by":"crossref","first-page":"1931","DOI":"10.1111\/j.1538-7836.2006.02098.x","article-title":"Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura","volume":"4","author":"Tao","year":"2006","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib96","doi-asserted-by":"crossref","first-page":"1932","DOI":"10.1182\/blood-2005-03-0848","article-title":"Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases","volume":"106","author":"Fakhouri","year":"2005","journal-title":"Blood"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib97","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1160\/TH07-12-0753","article-title":"Rituximab as pre-emptive treatment in patients with thrombotic thrombocytopenic purpura and evidence of anti-ADAMTS13 autoantibodies","volume":"101","author":"Bresin","year":"2009","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib98","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1056\/NEJMc0809959","article-title":"Eculizumab for congenital atypical hemolytic-uremic syndrome","volume":"360","author":"Gruppo","year":"2009","journal-title":"N Engl J Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib99","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1056\/NEJMc0808527","article-title":"Eculizumab for atypical hemolytic-uremic syndrome","volume":"360","author":"Nurnberger","year":"2009","journal-title":"N Engl J Med"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib100","doi-asserted-by":"crossref","first-page":"1312","DOI":"10.2215\/CJN.01090209","article-title":"Complement inhibitor eculizumab in atypical hemolytic uremic syndrome","volume":"4","author":"Mache","year":"2009","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib101","doi-asserted-by":"crossref","first-page":"2644","DOI":"10.1111\/j.1600-6143.2009.02817.x","article-title":"Safety and long-term efficacy of eculizumab in a renal transplant patient with recurrent atypical hemolytic-uremic syndrome","volume":"9","author":"Chatelet","year":"2009","journal-title":"Am J Transplant"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib102","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1053\/j.ajkd.2009.08.011","article-title":"Maintenance of kidney function following treatment with eculizumab and discontinuation of plasma exchange after a third kidney transplant for atypical hemolytic uremic syndrome associated with a CFH mutation","volume":"55","author":"Davin","year":"2010","journal-title":"Am J Kidney Dis"},{"key":"10.1016\/j.semnephrol.2010.06.006_bib103","doi-asserted-by":"crossref","first-page":"4094","DOI":"10.1182\/blood-2008-11-189944","article-title":"Complement fraction 3 binding on erythrocytes as additional mechanism of disease in paroxysmal nocturnal hemoglobinuria patients treated by eculizumab","volume":"113","author":"Risitano","year":"2009","journal-title":"Blood"}],"container-title":["Seminars in Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0270929510000987?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0270929510000987?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2023,6,3]],"date-time":"2023-06-03T02:48:51Z","timestamp":1685760531000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0270929510000987"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,7]]},"references-count":103,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2010,7]]}},"alternative-id":["S0270929510000987"],"URL":"https:\/\/doi.org\/10.1016\/j.semnephrol.2010.06.006","relation":{},"ISSN":["0270-9295"],"issn-type":[{"value":"0270-9295","type":"print"}],"subject":[],"published":{"date-parts":[[2010,7]]}}}