{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T03:18:18Z","timestamp":1761535098123,"version":"build-2065373602"},"reference-count":30,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2016,2,1]],"date-time":"2016-02-01T00:00:00Z","timestamp":1454284800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2016,2,1]],"date-time":"2016-02-01T00:00:00Z","timestamp":1454284800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2022,11,9]],"date-time":"2022-11-09T00:00:00Z","timestamp":1667952000000},"content-version":"vor","delay-in-days":2473,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"name":"Italo Monzino Foundation"},{"name":"Fondazione Fiera di Milano"},{"DOI":"10.13039\/501100004963","name":"Seventh Framework Programme","doi-asserted-by":"publisher","award":["305608"],"award-info":[{"award-number":["305608"]}],"id":[{"id":"10.13039\/501100004963","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Associazione per la Lotta alla DDD"}],"content-domain":{"domain":["jthjournal.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Thrombosis and Haemostasis"],"published-print":{"date-parts":[[2016,2]]},"DOI":"10.1111\/jth.13210","type":"journal-article","created":{"date-parts":[[2015,11,28]],"date-time":"2015-11-28T03:28:43Z","timestamp":1448681323000},"page":"340-345","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":3,"title":["Thrombotic microangiopathy without renal involvement: two novel mutations in complement\u2010regulator genes"],"prefix":"10.1016","volume":"14","author":[{"given":"F.","family":"Peyvandi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Rossio","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B.","family":"Ferrari","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.A.","family":"Lotta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Pontiggia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N.","family":"Ghiringhelli Borsa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Pizzuti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Donadelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Piras","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Cugno","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Noris","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1111\/jth.13210_bb0010","doi-asserted-by":"crossref","first-page":"878","DOI":"10.3324\/haematol.11196","article-title":"Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome: much progress and many remaining issues","volume":"92","author":"Mannucci","year":"2007","journal-title":"Haematologica"},{"key":"10.1111\/jth.13210_bb0015","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\u2010uremic syndrome","volume":"339","author":"Furlan","year":"1998","journal-title":"N Engl J Med"},{"key":"10.1111\/jth.13210_bb0020","doi-asserted-by":"crossref","first-page":"3889","DOI":"10.1016\/j.molimm.2007.06.003","article-title":"Where next with atypical hemolytic uremic syndrome?","volume":"44","author":"Jokiranta","year":"2007","journal-title":"Mol Immunol"},{"key":"10.1111\/jth.13210_bb0025","doi-asserted-by":"crossref","first-page":"778","DOI":"10.1182\/blood-2001-12-0166","article-title":"Thrombotic thrombocytopenic purpura\/hemolytic uremic syndrome","volume":"100","author":"Remuzzi","year":"2002","journal-title":"Blood"},{"key":"10.1111\/jth.13210_bb0030","doi-asserted-by":"crossref","first-page":"1555","DOI":"10.3324\/haematol.2009.019299","article-title":"Multiple domains of ADAMTS\u201013 are targeted by autoantibodies against ADAMTS\u201013 in patients with acquired idiopathic thrombotic thrombocytopenic purpura","volume":"95","author":"Zheng","year":"2010","journal-title":"Haematologica"},{"key":"10.1111\/jth.13210_bb0035","doi-asserted-by":"crossref","first-page":"1556","DOI":"10.1111\/j.1538-7836.2012.04808.x","article-title":"Peyvandi F ADAMTS\u201013 activity and autoantibodies classes and subclasses as prognostic predictors in acquired thrombotic thrombocytopenic purpura","volume":"10","author":"Bettoni","year":"2012","journal-title":"J Thromb Haemost"},{"key":"10.1111\/jth.13210_bb0040","doi-asserted-by":"crossref","first-page":"560","DOI":"10.1002\/ajh.23455","article-title":"Management and outcomes for patients with TTP: analysis of 100 cases at a single institution","volume":"88","author":"Chaturvedi","year":"2013","journal-title":"Am J Hematol"},{"key":"10.1111\/jth.13210_bb0045","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1016\/j.thromres.2013.01.024","article-title":"ADAMTS\u201013 may not predict disease or outcome in patients with Thrombotic Thrombocytopenic Purpura","volume":"131","author":"Rock","year":"2013","journal-title":"Thromb Res"},{"key":"10.1111\/jth.13210_bb0050","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1111\/j.1538-7836.2012.04674.x","article-title":"Complement activation in thrombotic thrombocytopenic purpura","volume":"10","author":"R\u00e9ti","year":"2012","journal-title":"J Thromb Haemost"},{"key":"10.1111\/jth.13210_bb0055","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1160\/TH04-07-0450","article-title":"Complement activation: the missing link between ADAMTS\u201013 deficiency and microvascular thrombosis of thrombotic microangiopathies","volume":"93","author":"Ruiz\u2010Torres","year":"2005","journal-title":"Thromb Haemost"},{"key":"10.1111\/jth.13210_bb0060","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1038\/nrneph.2012.195","article-title":"STEC\u2010HUS, atypical HUS and TTP are all diseases of complement activation","volume":"8","author":"Noris","year":"2012","journal-title":"Nat Rev Nephrol"},{"key":"10.1111\/jth.13210_bb0065","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1111\/bjh.12112","article-title":"Complement activation in thrombotic microangiopathies","volume":"160","author":"Feng","year":"2013","journal-title":"Br J Haematol"},{"key":"10.1111\/jth.13210_bb0070","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1182\/blood-2009-11-252627","article-title":"aHUS: a disorder with many risk factors","volume":"115","author":"Rodriguez de C\u00f3rdoba","year":"2010","journal-title":"Blood"},{"key":"10.1111\/jth.13210_bb0075","doi-asserted-by":"crossref","first-page":"1177","DOI":"10.1681\/ASN.2005010086","article-title":"Complement factor H mutation in familial thrombotic thrombocytopenic purpura with ADAMTS\u201013 deficiency and renal involvement","volume":"16","author":"Noris","year":"2005","journal-title":"J Am Soc Nephrol"},{"key":"10.1111\/jth.13210_bb0080","doi-asserted-by":"crossref","first-page":"4012","DOI":"10.1182\/blood-2013-03-487694","article-title":"Complement factor H mutations are present in ADAMTS\u201013\u2010deficient, ticlopidine\u2010associated thrombotic microangiopathies","volume":"121","author":"Chapin","year":"2013","journal-title":"Blood"},{"key":"10.1111\/jth.13210_bb0085","doi-asserted-by":"crossref","first-page":"1439","DOI":"10.1111\/j.1538-7836.2012.04744.x","article-title":"Measurement of anti\u2010ADAMTS\u201013 neutralizing autoantibodies: a comparison between CBA and FRET assays","volume":"10","author":"Mancini","year":"2012","journal-title":"J Thromb Haemost"},{"key":"10.1111\/jth.13210_bb0090","doi-asserted-by":"crossref","first-page":"232","DOI":"10.3324\/haematol.11739","article-title":"ADAMTS\u201013 and anti\u2010ADAMTS\u201013 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission","volume":"93","author":"Peyvandi","year":"2008","journal-title":"Haematologica"},{"key":"10.1111\/jth.13210_bb0095","doi-asserted-by":"crossref","first-page":"1744","DOI":"10.1111\/j.1538-7836.2011.04428.x","article-title":"Platelet reactive conformation and multimeric pattern of von Willebrand factor in acquired thrombotic thrombocytopenic purpura during acute disease and remission","volume":"9","author":"Lotta","year":"2011","journal-title":"J Thromb Haemost"},{"key":"10.1111\/jth.13210_bb0100","doi-asserted-by":"crossref","first-page":"1440","DOI":"10.1111\/jth.12615","article-title":"Complement functional tests for monitoring eculizumab treatment in patients with atypical hemolytic uremic syndrome","volume":"12","author":"Cugno","year":"2014","journal-title":"J Thromb Haemost"},{"key":"10.1111\/jth.13210_bb0105","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1038\/ng.2590","article-title":"Recessive mutations in DGKE cause atipical hemolytic\u2010uremic syndrome","volume":"45","author":"Lemaire","year":"2013","journal-title":"Nat Genet"},{"key":"10.1111\/jth.13210_bb0110","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1056\/NEJMoa0810739","article-title":"Thrombomodulin mutations in atypical hemolytic\u2013uremic syndrome","volume":"361","author":"Delvaeye","year":"2009","journal-title":"N Engl J Med"},{"key":"10.1111\/jth.13210_bb0115","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1055\/s-0030-1262886","article-title":"Atypical hemolytic uremic syndrome associated with mutations in complement regulator genes","volume":"36","author":"Le Quintrec","year":"2010","journal-title":"Semin Thromb Hemost"},{"key":"10.1111\/jth.13210_bb0120","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":"10.1111\/jth.13210_bb0125","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1016\/j.semnephrol.2010.06.006","article-title":"Genetics and genetic testing in hemolityc uremic syndrome\/thrombotic thrombocitopenic purpura","volume":"30","author":"Noris","year":"2010","journal-title":"Semin Nephrol"},{"key":"10.1111\/jth.13210_bb0130","doi-asserted-by":"crossref","first-page":"1835","DOI":"10.1016\/j.molimm.2006.10.005","article-title":"A mutation in factor I that is associated with atypical hemolytic uremic syndrome does not affect the function of factor I in complement regulation","volume":"44","author":"Nilsson","year":"2007","journal-title":"Mol Immunol"},{"key":"10.1111\/jth.13210_bb0135","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1007\/s00281-011-0282-8","article-title":"Thrombomodulin and its role in inflammation","volume":"34","author":"Conway","year":"2012","journal-title":"Semin Immunopathol"},{"key":"10.1111\/jth.13210_bb0140","doi-asserted-by":"crossref","first-page":"96","DOI":"10.5482\/HAMO-12-12-0025","article-title":"Complement activation in thrombotic microangiopathy","volume":"33","author":"Karpman","year":"2013","journal-title":"Hamostaseologie"},{"key":"10.1111\/jth.13210_bb0145","doi-asserted-by":"crossref","first-page":"1147","DOI":"10.1172\/JCI117430","article-title":"C5a\u2010induced expression of P\u2010selectin in endothelial cells","volume":"94","author":"Foreman","year":"1994","journal-title":"J Clin Invest"},{"key":"10.1111\/jth.13210_bb0150","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1016\/j.thromres.2011.10.031","article-title":"Thrombin activatable fibrinolysis inhibitor: at the nexus of fibrinolysis and inflammation","volume":"129","author":"Colucci","year":"2012","journal-title":"Thromb Res"},{"key":"10.1111\/jth.13210_bb0155","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1182\/blood-2004-01-0107","article-title":"Effects of inflammatory cytokines on the release and cleavage of the endothelial cell\u2010derived ultralarge von Willebrand factor multimers under flow","volume":"104","author":"Bernardo","year":"2004","journal-title":"Blood"}],"container-title":["Journal of Thrombosis and Haemostasis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjth.13210","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjth.13210","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S153878362205036X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S153878362205036X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/onlinelibrary.wiley.com\/wol1\/doi\/10.1111\/jth.13210\/fullpdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T03:14:29Z","timestamp":1761534869000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S153878362205036X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,2]]},"references-count":30,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2016,2]]}},"alternative-id":["S153878362205036X"],"URL":"https:\/\/doi.org\/10.1111\/jth.13210","relation":{},"ISSN":["1538-7836"],"issn-type":[{"type":"print","value":"1538-7836"}],"subject":[],"published":{"date-parts":[[2016,2]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Thrombotic microangiopathy without renal involvement: two novel mutations in complement\u2010regulator genes","name":"articletitle","label":"Article Title"},{"value":"Journal of Thrombosis and Haemostasis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1111\/jth.13210","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2015 International Society on Thrombosis and Haemostasis. Published by Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}