{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T14:28:17Z","timestamp":1782570497674,"version":"3.54.5"},"reference-count":29,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2026,2,6]],"date-time":"2026-02-06T00:00:00Z","timestamp":1770336000000},"content-version":"vor","delay-in-days":101,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T00:00:00Z","timestamp":1761609600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T00:00:00Z","timestamp":1761609600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Alexion Pharma GK"},{"name":"Alexion Pharma GK"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,12,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Atypical haemolytic uraemic syndrome (aHUS) leads to acute kidney injury, necessitating dialysis in about half of patients. A certain proportion of patients treated with C5 inhibitors discontinue dialysis; however, little is known about the patient characteristics and clinical courses relating to discontinuation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We compared the characteristics and clinical courses of patients with aHUS on dialysis at the initiation of eculizumab during post-marketing surveillance\u00a0in Japan, stratified by those who did (Group A) and did not (Group B) discontinue dialysis within 26\u00a0weeks of eculizumab treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of 38 included patients, 21 (55.3%) and 17 (44.7%) were placed in Groups A and B, respectively. No patient re-started dialysis. Hypertension was less frequent in Group A than in Group B (6\/21 [28.6%] vs. 11\/17 [64.7%], p\u2009=\u20090.022). Both the duration of dialysis before eculizumab initiation (6 vs. 17\u00a0days, p\u2009=\u20090.011) and the time from thrombotic microangiopathy onset to eculizumab initiation (9 vs. 25\u00a0days, p\u2009=\u20090.008) were shorter in Group A. A duration of less than 15\u00a0days from thrombotic microangiopathy onset to eculizumab initiation was associated with dialysis discontinuation. Kidney function improvement and normalisation of platelet count and lactate dehydrogenase levels were achieved earlier in Group A than in Group B (p\u2009=\u20090.050, 0.014, and\u2009&amp;lt;\u20090.001, respectively). Five (29.4%) of 17 patients in Group B discontinued dialysis after 27\u00a0weeks of eculizumab treatment, including one patient who underwent kidney transplantation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Early initiation of eculizumab was significantly correlated with dialysis discontinuation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Graphical Abstract<\/jats:title>\n                    <jats:p\/>\n                  <\/jats:sec>","DOI":"10.1007\/s40620-025-02433-z","type":"journal-article","created":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T10:34:32Z","timestamp":1761647672000},"page":"2663-2671","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Characteristics and clinical courses of patients with atypical haemolytic uraemic syndrome on dialysis withdrawal after eculizumab treatment: sub-analysis of post-marketing surveillance in Japan"],"prefix":"10.1093","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5242-8987","authenticated-orcid":false,"given":"Shuichi","family":"Ito","sequence":"first","affiliation":[{"name":"https:\/\/ror.org\/0135d1r83 grid.268441.d 0000 0001 1033 6139 Department of Pediatrics, Graduate School of Medicine Yokohama City University Kanagawa Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masanori","family":"Matsumoto","sequence":"additional","affiliation":[{"name":"https:\/\/ror.org\/045ysha14 grid.410814.8 0000 0004 0372 782X Department of Hematology and Blood Transfusion Medicine Nara Medical University Nara Japan 2"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akihiko","family":"Shimono","sequence":"additional","affiliation":[{"name":"Medical Affairs Division Alexion Pharma GK Tokyo Japan 3"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirofumi","family":"Teranishi","sequence":"additional","affiliation":[{"name":"Medical Affairs Division Alexion Pharma GK Tokyo Japan 3"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shoichi","family":"Maruyama","sequence":"additional","affiliation":[{"name":"https:\/\/ror.org\/04chrp450 grid.27476.30 0000 0001 0943 978X Department of Nephrology Nagoya University Graduate School of Medicine Aichi Japan 4"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,10,28]]},"reference":[{"key":"2026020606020203900_CR1","doi-asserted-by":"publisher","first-page":"99","DOI":"10.5551\/jat.RV17026","article-title":"Pathogenesis of atypical hemolytic uremic syndrome","volume":"26","author":"Yoshida","year":"2019","journal-title":"J Atheroscler Thromb"},{"key":"2026020606020203900_CR2","first-page":"216","article-title":"Atypical hemolytic uremic syndrome: differential diagnosis from TTP\/HUS and management. Atipik Hemolitik \u00dcremik Sendrom: Ttp\/H\u00fcs ile Ay\u0131r\u0131c\u0131 Tan\u0131s\u0131 ve Tedavisi","volume":"31","author":"Yenerel","year":"2019","journal-title":"Turk J Haematol"},{"key":"2026020606020203900_CR3","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1016\/j.molimm.2015.03.246","article-title":"Atypical aHUS: state of the art","volume":"67","author":"Nester","year":"2015","journal-title":"Mol Immunol"},{"key":"2026020606020203900_CR4","doi-asserted-by":"publisher","first-page":"2233","DOI":"10.2147\/JMDH.S245620","article-title":"Atypical hemolytic-uremic syndrome: genetic basis, clinical manifestations, and a multidisciplinary approach to management","volume":"16","author":"Yerigeri","year":"2023","journal-title":"J Multidiscip Healthc"},{"key":"2026020606020203900_CR5","doi-asserted-by":"publisher","first-page":"e0177894","DOI":"10.1371\/journal.pone.0177894","article-title":"Predictive features of chronic kidney disease in atypical haemolytic uremic syndrome","volume":"12","author":"Jamme","year":"2017","journal-title":"PLoS ONE"},{"key":"2026020606020203900_CR6","doi-asserted-by":"publisher","first-page":"2169","DOI":"10.1056\/NEJMoa1208981","article-title":"Terminal complement inhibitor eculizumab in atypical haemolytic-uremic syndrome","volume":"368","author":"Legendre","year":"2013","journal-title":"N Engl J Med"},{"key":"2026020606020203900_CR7","doi-asserted-by":"publisher","first-page":"1287","DOI":"10.1016\/j.kint.2020.01.035","article-title":"The long-acting C5 inhibitor, ravulizumab, is effective and safe in adult patients with atypical hemolytic uremic syndrome na\u00efve to complement inhibitor treatment","volume":"97","author":"Rondeau","year":"2020","journal-title":"Kidney Int"},{"key":"2026020606020203900_CR8","doi-asserted-by":"publisher","first-page":"225","DOI":"10.1016\/j.kint.2020.10.046","article-title":"The long-acting C5 inhibitor, ravulizumab, is effective and safe in pediatric patients with atypical hemolytic uremic syndrome naive to complement inhibitor treatment","volume":"100","author":"Ariceta","year":"2021","journal-title":"Kidney Int"},{"key":"2026020606020203900_CR9","doi-asserted-by":"publisher","first-page":"1061","DOI":"10.1038\/ki.2014.423","article-title":"Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies","volume":"87","author":"Licht","year":"2015","journal-title":"Kidney Int"},{"key":"2026020606020203900_CR10","doi-asserted-by":"publisher","first-page":"701","DOI":"10.1016\/j.kint.2015.11.026","article-title":"Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndrome","volume":"89","author":"Greenbaum","year":"2016","journal-title":"Kidney Int"},{"key":"2026020606020203900_CR11","doi-asserted-by":"publisher","first-page":"84","DOI":"10.1053\/j.ajkd.2015.12.034","article-title":"Terminal complement inhibitor eculizumab in adult patients with atypical hemolytic uremic syndrome: a single-arm, open-label trial","volume":"68","author":"Fakhouri","year":"2016","journal-title":"Am J Kidney Dis"},{"key":"2026020606020203900_CR12","doi-asserted-by":"publisher","first-page":"1371","DOI":"10.1182\/blood.2022018833","article-title":"Atypical hemolytic uremic syndrome in the era of terminal complement inhibition: an observational cohort study","volume":"142","author":"Brocklebank","year":"2023","journal-title":"Blood"},{"key":"2026020606020203900_CR13","doi-asserted-by":"publisher","first-page":"bcr2015213928","DOI":"10.1136\/bcr-2015-213928","article-title":"Recovery of renal function after long-term dialysis and resolution of cardiomyopathy in a patient with aHUS receiving eculizumab","volume":"2016","author":"Emirova","year":"2016","journal-title":"BMJ Case Rep"},{"key":"2026020606020203900_CR14","doi-asserted-by":"publisher","first-page":"1603","DOI":"10.1016\/j.ekir.2021.03.884","article-title":"Long-term efficacy and safety of the long-acting complement C5 inhibitor ravulizumab for the treatment of atypical hemolytic uremic syndrome in adults","volume":"6","author":"Barbour","year":"2021","journal-title":"Kidney Int Rep"},{"key":"2026020606020203900_CR15","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1007\/s40620-016-0288-3","article-title":"Improved renal recovery in patients with atypical hemolytic uremic syndrome following rapid initiation of eculizumab treatment","volume":"30","author":"Walle","year":"2017","journal-title":"J Nephrol"},{"key":"2026020606020203900_CR16","doi-asserted-by":"publisher","first-page":"2181","DOI":"10.1007\/s40620-024-01921-y","article-title":"Eculizumab for adult patients with atypical haemolytic-uremic syndrome: full dataset analysis of Japanese post-marketing surveillance","volume":"37","author":"Maruyama","year":"2024","journal-title":"J Nephrol"},{"key":"2026020606020203900_CR17","doi-asserted-by":"publisher","first-page":"112","DOI":"10.1007\/s10157-018-1610-2","article-title":"Safety and effectiveness of eculizumab for pediatric patients with atypical hemolytic-uremic syndrome in Japan: interim analysis of post-marketing surveillance","volume":"23","author":"Ito","year":"2019","journal-title":"Clin Exp Nephrol"},{"key":"2026020606020203900_CR18","doi-asserted-by":"publisher","first-page":"414","DOI":"10.1093\/ndt\/gfac150","article-title":"Eculizumab for paediatric patients with atypical haemolytic uraemic syndrome: full dataset analysis of post-marketing surveillance in Japan","volume":"38","author":"Ito","year":"2023","journal-title":"Nephrol Dial Transplant"},{"key":"2026020606020203900_CR19","doi-asserted-by":"publisher","first-page":"65","DOI":"10.1007\/s10157-018-1609-8","article-title":"Safety and effectiveness of eculizumab for adult patients with atypical hemolytic-uremic syndrome in Japan: interim analysis of post-marketing surveillance","volume":"23","author":"Kato","year":"2019","journal-title":"Clin Exp Nephrol"},{"key":"2026020606020203900_CR20","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1007\/s10157-013-0911-8","article-title":"Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the Joint Committee of the Japanese Society of Nephrology and the Japan Pediatric Society","volume":"18","author":"Sawai","year":"2014","journal-title":"Clin Exp Nephrol"},{"key":"2026020606020203900_CR21","doi-asserted-by":"publisher","first-page":"536","DOI":"10.1007\/s10157-016-1276-6","article-title":"Clinical guides for atypical hemolytic uremic syndrome in Japan","volume":"20","author":"Kato","year":"2016","journal-title":"Clin Exp Nephrol"},{"key":"2026020606020203900_CR22","doi-asserted-by":"publisher","first-page":"979","DOI":"10.1007\/s10157-017-1524-4","article-title":"Controversies of the classification of TMA and the terminology of aHUS","volume":"22","author":"Kato","year":"2018","journal-title":"Clin Exp Nephrol"},{"key":"2026020606020203900_CR23","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-024-68736-6","article-title":"Annual trends in atypical haemolytic uremic syndrome management in Japan and factors influencing early diagnosis and treatment: a retrospective study","volume":"14","author":"Tatematsu","year":"2024","journal-title":"Sci Rep"},{"key":"2026020606020203900_CR24","doi-asserted-by":"publisher","DOI":"10.1016\/j.xkme.2024.100855","article-title":"Ravulizumab in atypical hemolytic uremic syndrome: an analysis of 2-year efficacy and safety outcomes in 2 phase 3 trials","volume":"6","author":"Dixon","year":"2024","journal-title":"Kidney Med"},{"key":"2026020606020203900_CR25","doi-asserted-by":"publisher","first-page":"539","DOI":"10.1016\/j.kint.2016.10.005","article-title":"Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a \u201cKidney Disease: Improving Global Outcomes\u201d (KDIGO) Controversies Conference","volume":"91","author":"Goodship","year":"2017","journal-title":"Kidney Int"},{"key":"2026020606020203900_CR26","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2023.1264310","article-title":"Recommendations for the individualised management of atypical hemolytic uremic syndrome in adults","volume":"10","author":"\u00c1vila","year":"2023","journal-title":"Front Med (Lausanne)"},{"key":"2026020606020203900_CR27","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1038\/ki.2012.130","article-title":"Hemodialysis effect on platelet count and function and hemodialysis-associated thrombocytopenia","volume":"82","author":"Daugirdas","year":"2012","journal-title":"Kidney Int"},{"key":"2026020606020203900_CR28","doi-asserted-by":"publisher","first-page":"1583","DOI":"10.1681\/ASN.2020101440","article-title":"Platelet function in CKD: a systematic review and meta-analysis","volume":"32","author":"Baaten","year":"2021","journal-title":"J Am Soc Nephrol"},{"key":"2026020606020203900_CR29","doi-asserted-by":"publisher","DOI":"10.1186\/s12959-024-00652-1","article-title":"Correlation between a 2-week change in platelet count and clinical outcomes after the initiation of ravulizumab treatment in adult patients with atypical hemolytic uremic syndrome: post-hoc analysis of the phase III trial","volume":"22","author":"Matsumoto","year":"2024","journal-title":"Thromb J"}],"container-title":["Journal of Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s40620-025-02433-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s40620-025-02433-z\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/academic.oup.com\/joneph\/article-pdf\/38\/9\/2663\/66794174\/joneph_38_9_2433.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s40620-025-02433-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/academic.oup.com\/joneph\/article-pdf\/38\/9\/2663\/66794174\/joneph_38_9_2433.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,6]],"date-time":"2026-02-06T11:02:09Z","timestamp":1770375729000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/joneph\/article\/38\/9\/2663\/8462665"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,28]]},"references-count":29,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2025,10,28]]},"published-print":{"date-parts":[[2025,12,1]]}},"URL":"https:\/\/doi.org\/10.1007\/s40620-025-02433-z","relation":{},"ISSN":["1121-8428","1724-6059"],"issn-type":[{"value":"1121-8428","type":"print"},{"value":"1724-6059","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2025,12,1]]},"published":{"date-parts":[[2025,10,28]]},"assertion":[{"value":"20 February 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 September 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 October 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"SI, MM, and SM received consulting fees, honoraria for lectures and participation on advisory board from Alexion Pharma GK. AS and HT are employees of Alexion Pharma GK and shareholders of AstraZeneca.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"Requirements of ethical approval were waived because of the nature of the surveillance.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"No animals were involved in this research so a statement on their welfare does not apply.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Human and animal rights"}},{"value":"Requirements of obtaining informed consent from individual patients were waived because of the nature of the surveillance.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent to participate"}}]}}