{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T23:28:06Z","timestamp":1791070086919,"version":"4.1.0"},"reference-count":24,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2013,9,30]],"date-time":"2013-09-30T00:00:00Z","timestamp":1380499200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2013,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>HUS is characterized by hemolytic anemia, thrombocytopenia, and acute renal failure. While \u201ctypical\u201d HUS is usually associated with <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>higa toxin\u2010producing <jats:italic><jats:styled-content style=\"fixed-case\">E<\/jats:styled-content>scherichia coli<\/jats:italic> infections and recovers in the majority of cases, a<jats:styled-content style=\"fixed-case\">HUS<\/jats:styled-content> is caused by mutations of complement components or antibodies against <jats:styled-content style=\"fixed-case\">CFH<\/jats:styled-content> leading to uncontrolled activation of alternative complement pathway and often to <jats:styled-content style=\"fixed-case\">ESRD<\/jats:styled-content>. Recently, <jats:italic><jats:styled-content style=\"fixed-case\">THBD<\/jats:styled-content><\/jats:italic> gene mutations have been reported in a<jats:styled-content style=\"fixed-case\">HUS<\/jats:styled-content>. Theoretically, the risk of disease recurrence after renal transplantation should be low because THBD is primarily a membrane\u2010bound protein expressed by endothelial cells; however, a small proportion of THBD is present as a soluble form in plasma. We report the case of a 19\u2010yr\u2010old man with a<jats:styled-content style=\"fixed-case\">HUS<\/jats:styled-content> secondary to a <jats:italic><jats:styled-content style=\"fixed-case\">THBD<\/jats:styled-content><\/jats:italic> mutation that relapsed twice after two renal transplantations performed 12\u00a0yr apart. Despite successful control of <jats:styled-content style=\"fixed-case\">HUS<\/jats:styled-content> with plasma exchange and eculizumab after the second transplantation, the graft was ultimately lost due to severe steroid\u2010resistant cellular rejection. The present report suggests that <jats:italic><jats:styled-content style=\"fixed-case\">THBD<\/jats:styled-content><\/jats:italic> mutations may favor\u2010relapse of a<jats:styled-content style=\"fixed-case\">HUS<\/jats:styled-content> after renal transplantation.<\/jats:p>","DOI":"10.1111\/petr.12151","type":"journal-article","created":{"date-parts":[[2013,10,12]],"date-time":"2013-10-12T01:27:36Z","timestamp":1381541256000},"source":"Crossref","is-referenced-by-count":24,"title":["Post\u2010transplant recurrence of atypical hemolytic uremic syndrome in a patient with thrombomodulin mutation"],"prefix":"10.1111","volume":"17","author":[{"given":"Serena","family":"Sinibaldi","sequence":"first","affiliation":[{"name":"Department of Nephrology and Urology Bambino Ges\u00f9 Children's Hospital and Research Institute Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabella","family":"Guzzo","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Urology Bambino Ges\u00f9 Children's Hospital and Research Institute Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rossella","family":"Piras","sequence":"additional","affiliation":[{"name":"Clinical Research Center for Rare Diseases Aldo e Cele Dacc\u00f2 IRCCS \u2013 Istituto di Ricerche Farmacologiche Mario Negri Ranica Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Bresin","sequence":"additional","affiliation":[{"name":"Clinical Research Center for Rare Diseases Aldo e Cele Dacc\u00f2 IRCCS \u2013 Istituto di Ricerche Farmacologiche Mario Negri Ranica Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Emma","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Urology Bambino Ges\u00f9 Children's Hospital and Research Institute Rome 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