{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,27]],"date-time":"2025-09-27T16:31:46Z","timestamp":1758990706933},"reference-count":39,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[2018,11,1]],"date-time":"2018-11-01T00:00:00Z","timestamp":1541030400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","clinicalkey.es","clinicalkey.com.au","clinicalkey.fr","amjmedsci.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["The American Journal of the Medical Sciences"],"published-print":{"date-parts":[[2018,11]]},"DOI":"10.1016\/j.amjms.2018.05.012","type":"journal-article","created":{"date-parts":[[2018,6,5]],"date-time":"2018-06-05T07:57:09Z","timestamp":1528185429000},"page":"492-498","update-policy":"http:\/\/dx.doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":5,"title":["Shiga Toxin as a Potential Trigger of CFHR1 Deletion-Associated Thrombotic Microangiopathy"],"prefix":"10.1016","volume":"356","author":[{"given":"Swarna Sri","family":"Nalluru","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meera","family":"Sridharan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronald S.","family":"Go","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Samar","family":"Said","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ariela L.","family":"Marshall","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.amjms.2018.05.012_bib0001","doi-asserted-by":"crossref","first-page":"654","DOI":"10.1056\/NEJMra1312353","article-title":"Syndromes of thrombotic microangiopathy","volume":"371","author":"George","year":"2014","journal-title":"NEngl J Med"},{"key":"10.1016\/j.amjms.2018.05.012_bib0002","doi-asserted-by":"crossref","first-page":"1189","DOI":"10.1016\/j.mayocp.2016.05.015","article-title":"Thrombotic microangiopathy care pathway: a consensus statement for the mayo clinic complement alternative pathway-thrombotic microangiopathy (CAP-TMA) disease-oriented group","volume":"91","author":"Go","year":"2016","journal-title":"Mayo Clin Proc"},{"key":"10.1016\/j.amjms.2018.05.012_bib0003","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1016\/j.ejim.2013.05.007","article-title":"Atypical hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: clinically differentiating the thrombotic microangiopathies","volume":"24","author":"Cataland","year":"2013","journal-title":"Eur J Intern Med"},{"key":"10.1016\/j.amjms.2018.05.012_bib0004","doi-asserted-by":"crossref","first-page":"1389","DOI":"10.1016\/S0161-5890(96)00078-8","article-title":"Inhibition of complement activity by humanized anti-C5 antibody and single-chain Fv","volume":"33","author":"Thomas","year":"1996","journal-title":"Mol Immunol"},{"key":"10.1016\/j.amjms.2018.05.012_bib0005","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1681\/ASN.2012090884","article-title":"Combined complement gene mutations in atypical hemolytic uremic syndrome influence clinical phenotype","volume":"24","author":"Bresin","year":"2013","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.amjms.2018.05.012_bib0006","doi-asserted-by":"crossref","first-page":"E1445","DOI":"10.1002\/humu.21256","article-title":"Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndrome","volume":"31","author":"Maga","year":"2010","journal-title":"Hum Mutat"},{"key":"10.1016\/j.amjms.2018.05.012_bib0007","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1681\/ASN.2005080868","article-title":"Factor H and atypical hemolytic uremic syndrome: mutations in the C-terminus cause structural changes and defective recognition functions","volume":"17","author":"Jozsi","year":"2006","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.amjms.2018.05.012_bib0008","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1016\/j.it.2015.04.008","article-title":"Factor H-related proteins determine complement-activating surfaces","volume":"36","author":"Jozsi","year":"2015","journal-title":"Trends Immunol"},{"key":"10.1016\/j.amjms.2018.05.012_bib0009","doi-asserted-by":"crossref","first-page":"e41","DOI":"10.1371\/journal.pgen.0030041","article-title":"Deletion of complement factor H-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome","volume":"3","author":"Zipfel","year":"2007","journal-title":"PLoS Genet"},{"key":"10.1016\/j.amjms.2018.05.012_bib0010","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.imlet.2014.01.014","article-title":"Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome","volume":"160","author":"Jozsi","year":"2014","journal-title":"Immunol Lett"},{"key":"10.1016\/j.amjms.2018.05.012_bib0011","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0166200","article-title":"Mapping the complement factor H-related protein 1 (CFHR1):C3b\/C3d interactions","volume":"11","author":"Hannan","year":"2016","journal-title":"PLoS One"},{"key":"10.1016\/j.amjms.2018.05.012_bib0012","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1016\/j.molimm.2013.06.001","article-title":"Complement factor H related proteins (CFHRs)","volume":"56","author":"Skerka","year":"2013","journal-title":"Mol Immunol"},{"key":"10.1016\/j.amjms.2018.05.012_bib0013","doi-asserted-by":"crossref","first-page":"2439","DOI":"10.1182\/blood-2009-02-205641","article-title":"Factor H-related protein 1 (CFHR-1) inhibits complement C5 convertase activity and terminal complex formation","volume":"114","author":"Heinen","year":"2009","journal-title":"Blood"},{"key":"10.1016\/j.amjms.2018.05.012_bib0014","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.amjms.2018.05.012_bib0015","doi-asserted-by":"crossref","first-page":"2847","DOI":"10.1182\/blood-2016-11-709865","article-title":"HUS and atypical HUS","volume":"129","author":"Jokiranta","year":"2017","journal-title":"Blood"},{"key":"10.1016\/j.amjms.2018.05.012_bib0016","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1128\/CMR.11.3.450","article-title":"Pathogenesis and diagnosis of Shiga toxin-producing Escherichia coli infections","volume":"11","author":"Paton","year":"1998","journal-title":"Clin Microbiol Rev"},{"key":"10.1016\/j.amjms.2018.05.012_bib0017","doi-asserted-by":"crossref","first-page":"1021","DOI":"10.1093\/infdis\/174.5.1021","article-title":"Vero cytotoxigenic Escherichia coli infection in dairy farm families","volume":"174","author":"Wilson","year":"1996","journal-title":"J Infect Dis"},{"key":"10.1016\/j.amjms.2018.05.012_bib0018","doi-asserted-by":"crossref","first-page":"1487","DOI":"10.1182\/blood-2013-03-492421","article-title":"Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome","volume":"122","author":"Feng","year":"2013","journal-title":"Blood"},{"key":"10.1016\/j.amjms.2018.05.012_bib0019","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1080\/08998280.2017.11929576","article-title":"Acquired thrombotic thrombocytopenic purpura and atypical hemolytic uremic syndrome successfully treated with eculizumab","volume":"30","author":"Sasapu","year":"2017","journal-title":"Proc (Bayl Univ Med Cent)"},{"key":"10.1016\/j.amjms.2018.05.012_bib0020","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1007\/s00467-015-3207-2","article-title":"CFH gene mutation in a case of Shiga toxin-associated hemolytic uremic syndrome (STEC-HUS)","volume":"31","author":"Caillaud","year":"2016","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.amjms.2018.05.012_bib0021","doi-asserted-by":"crossref","first-page":"2201","DOI":"10.1111\/ajt.12297","article-title":"Two patients with history of STEC-HUS, posttransplant recurrence and complement gene mutations","volume":"13","author":"Alberti","year":"2013","journal-title":"Am J Transplant"},{"key":"10.1016\/j.amjms.2018.05.012_bib0022","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1093\/ckj\/sfx030","article-title":"Rare genetic variants in Shiga toxin-associated haemolytic uraemic syndrome: genetic analysis prior to transplantation is essential","volume":"10","author":"Dowen","year":"2017","journal-title":"Clin Kidney J"},{"key":"10.1016\/j.amjms.2018.05.012_bib0023","first-page":"232","article-title":"Atypical hemolytic-uremic syndrome related to abnormalities within the complement system","volume":"32","author":"Fremeaux-Bacchi","year":"2011","journal-title":"Rev Med Intern"},{"key":"10.1016\/j.amjms.2018.05.012_bib0024","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1681\/ASN.2009070706","article-title":"Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations","volume":"21","author":"Fakhouri","year":"2010","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.amjms.2018.05.012_bib0025","doi-asserted-by":"crossref","first-page":"6394","DOI":"10.4049\/jimmunol.0900151","article-title":"Shiga toxin activates complement and binds factor H: evidence for an active role of complement in hemolytic uremic syndrome","volume":"182","author":"Orth","year":"2009","journal-title":"J Immunol"},{"key":"10.1016\/j.amjms.2018.05.012_bib0026","doi-asserted-by":"crossref","DOI":"10.21767\/2472-5056.100011","article-title":"Typical hus: evidence of acute phase complement activation from a daycare outbreak","volume":"1","author":"Brady","year":"2016","journal-title":"J Clin Exp Nephrol"},{"key":"10.1016\/j.amjms.2018.05.012_bib0027","doi-asserted-by":"crossref","first-page":"2561","DOI":"10.1056\/NEJMc1100859","article-title":"Eculizumab in severe Shiga-toxin-associated HUS","volume":"364","author":"Lapeyraque","year":"2011","journal-title":"N Engl J Med"},{"key":"10.1016\/j.amjms.2018.05.012_bib0028","doi-asserted-by":"crossref","first-page":"3807","DOI":"10.1093\/ndt\/gfs394","article-title":"Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the GermanSTEC-HUS registry","volume":"27","author":"Kielstein","year":"2012","journal-title":"Nephrol Dial Transplant"},{"key":"10.1016\/j.amjms.2018.05.012_bib0029","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1093\/ndt\/gft470","article-title":"Outbreak of Escherichia coli O104:H4 haemolytic uraemic syndrome in France: outcome with eculizumab","volume":"29","author":"Delmas","year":"2014","journal-title":"Nephrol Dial Transplant"},{"key":"10.1016\/j.amjms.2018.05.012_bib0030","doi-asserted-by":"crossref","first-page":"e1000","DOI":"10.1097\/MD.0000000000001000","article-title":"Eculizumab in typical hemolytic uremic syndrome (HUS) With neurological involvement","volume":"94","author":"Pape","year":"2015","journal-title":"Medicine"},{"key":"10.1016\/j.amjms.2018.05.012_bib0031","doi-asserted-by":"crossref","first-page":"117","DOI":"10.2146\/ajhp140134","article-title":"Safe and effective use of eculizumab in the treatment of severe Shiga toxin Escherichia coli-associated hemolytic uremic syndrome","volume":"72","author":"Dinh","year":"2015","journal-title":"Am J Health Syst Pharm"},{"key":"10.1016\/j.amjms.2018.05.012_bib0032","doi-asserted-by":"crossref","first-page":"1249","DOI":"10.1084\/jem.20070301","article-title":"Spontaneous hemolytic uremic syndrome triggered by complement factor H lacking surface recognition domains","volume":"204","author":"Pickering","year":"2007","journal-title":"J Exp Med"},{"key":"10.1016\/j.amjms.2018.05.012_bib0033","doi-asserted-by":"crossref","first-page":"2678","DOI":"10.1128\/IAI.01079-12","article-title":"Shiga toxin 2 reduces complement inhibitor CD59 expression on human renal tubular epithelial and glomerular endothelial cells","volume":"81","author":"Ehrlenbach","year":"2013","journal-title":"Infect Immun"},{"key":"10.1016\/j.amjms.2018.05.012_bib0034","doi-asserted-by":"crossref","first-page":"1786","DOI":"10.1681\/ASN.2013050450","article-title":"Shiga toxin promotes podocyte injury in experimental hemolytic uremic syndrome via activation of the alternative pathway of complement","volume":"25","author":"Locatelli","year":"2014","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.amjms.2018.05.012_bib0035","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1001\/jama.2012.264","article-title":"Association between azithromycin therapyand duration of bacterial shedding among patients with Shiga toxin-producing enteroaggregative Escherichia coli O104:H4","volume":"307","author":"Nitschke","year":"2012","journal-title":"JAMA"},{"key":"10.1016\/j.amjms.2018.05.012_bib0036","doi-asserted-by":"crossref","first-page":"496","DOI":"10.1016\/j.clinbiochem.2013.11.020","article-title":"Overexpression of complement C5 in endometriosis","volume":"47","author":"Aslan","year":"2014","journal-title":"Clin Biochem"},{"key":"10.1016\/j.amjms.2018.05.012_bib0037","doi-asserted-by":"crossref","first-page":"50","DOI":"10.2215\/CJN.06440616","article-title":"Pathogenic variants in complement genes and risk of atypical hemolytic uremic syndrome relapse after eculizumab discontinuation","volume":"12","author":"Fakhouri","year":"2017","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1016\/j.amjms.2018.05.012_bib0038","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1182\/blood-2017-02-770214","article-title":"Eculizumab cessation in atypical hemolytic uremic syndrome","volume":"130","author":"Merrill","year":"2017","journal-title":"Blood"},{"issue":"3","key":"10.1016\/j.amjms.2018.05.012_bib0039","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1007\/s00467-017-3813-2","article-title":"Complement functional tests for monitoring eculizumab treatment in patients with atypical hemolytic uremic syndrome: an update","volume":"33","author":"Ardissino","year":"2018","journal-title":"Pediatr Nephrol"}],"container-title":["The American Journal of the Medical Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0002962918302106?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0002962918302106?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,10,29]],"date-time":"2018-10-29T10:47:51Z","timestamp":1540810071000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0002962918302106"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,11]]},"references-count":39,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2018,11]]}},"alternative-id":["S0002962918302106"],"URL":"https:\/\/doi.org\/10.1016\/j.amjms.2018.05.012","relation":{},"ISSN":["0002-9629"],"issn-type":[{"value":"0002-9629","type":"print"}],"subject":[],"published":{"date-parts":[[2018,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Shiga Toxin as a Potential Trigger of CFHR1 Deletion-Associated Thrombotic Microangiopathy","name":"articletitle","label":"Article Title"},{"value":"The American Journal of the Medical Sciences","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.amjms.2018.05.012","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}