{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T05:46:59Z","timestamp":1776491219185,"version":"3.51.2"},"reference-count":14,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T00:00:00Z","timestamp":1776470400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T00:00:00Z","timestamp":1776470400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Alexion Pharma GK","award":["Alexion Pharma GK"],"award-info":[{"award-number":["Alexion Pharma GK"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["CEN Case Rep"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>A 53-year-old woman with kidney failure and low-titer donor-specific antibody underwent a living-unrelated donor kidney transplant. Presentation of severe thrombotic microangiopathy (TMA) resulted in early graft loss at day 8 post-transplant and initiation of hemodialysis. Thorough examination of the medical data revealed the patient to have had atypical hemolytic uremic syndrome (aHUS). Hemolytic anemia persisted for several months, even after complete embolization of the kidney graft. The patient subsequently received prophylactic eculizumab treatment and underwent a successful second transplant from a familial donor. Withdrawal of eculizumab resulted in development of TMA in the second graft, while reintroduction of eculizumab reversed the TMA. We examined complement-related profiles over time, and serial measurement of factors such as factor Ba, soluble C5b-9, and C3, and of total complement activity (CH50), clearly reflected the underlying pathophysiological condition and treatment status of the patient before, during, and after transplant. Longitudinal observation of complement protein levels may therefore aid in identifying candidates for kidney transplant who are at risk of aHUS, and monitoring patient status during transplant and follow-up.<\/jats:p>","DOI":"10.1007\/s13730-026-01114-5","type":"journal-article","created":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T05:10:29Z","timestamp":1776489029000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Measurement of complement proteins may aid interpretation of pathophysiology in kidney transplant recipients with atypical hemolytic uremic syndrome"],"prefix":"10.1007","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8469-5326","authenticated-orcid":false,"given":"Masayoshi","family":"Miura","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Higashiyama","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takahiro","family":"Tsuji","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katsuki","family":"Ohtani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nobutaka","family":"Wakamiya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,4,18]]},"reference":[{"key":"1114_CR1","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1016\/j.jim.2018.07.006","volume":"461","author":"M Sridharan","year":"2018","unstructured":"Sridharan M, Go RS, Willrich MAV. Atypical hemolytic uremic syndrome: review of clinical presentation, diagnosis and management. J Immunol Methods. 2018;461:15\u201322.","journal-title":"J Immunol Methods"},{"issue":"2","key":"1114_CR2","doi-asserted-by":"publisher","first-page":"99","DOI":"10.5551\/jat.RV17026","volume":"26","author":"Y Yoshida","year":"2019","unstructured":"Yoshida Y, Kato H, Ikeda Y, Nangaku M. Pathogenesis of atypical hemolytic uremic syndrome. J Atheroscler Thromb. 2019;26(2):99\u2013110.","journal-title":"J Atheroscler Thromb"},{"issue":"10","key":"1114_CR3","doi-asserted-by":"publisher","first-page":"1844","DOI":"10.2215\/CJN.02210310","volume":"5","author":"M Noris","year":"2010","unstructured":"Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, Gamba S, Daina E, Fenili C, Castelletti F, Sorosina A, Piras R, Donadelli R, Maranta R, van der Meer I, Conway EM, Zipfel PF, Goodship TH, Remuzzi G. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol. 2010;5(10):1844\u201359.","journal-title":"Clin J Am Soc Nephrol"},{"issue":"2","key":"1114_CR4","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1097\/MOP.0b013e32835df48a","volume":"25","author":"C Joseph","year":"2013","unstructured":"Joseph C, Gattineni J. Complement disorders and hemolytic uremic syndrome. Curr Opin Pediatr. 2013;25(2):209\u201315.","journal-title":"Curr Opin Pediatr"},{"issue":"11","key":"1114_CR5","doi-asserted-by":"publisher","first-page":"1205","DOI":"10.1097\/TP.0000000000000200","volume":"98","author":"D Matar","year":"2014","unstructured":"Matar D, Naqvi F, Racusen LC, Carter-Monroe N, Montgomery RA, Alachkar N. Atypical hemolytic uremic syndrome recurrence after kidney transplantation. Transplantation. 2014;98(11):1205\u201312.","journal-title":"Transplantation"},{"issue":"7","key":"1114_CR6","doi-asserted-by":"publisher","first-page":"919","DOI":"10.3390\/jcm8070919","volume":"8","author":"ML Gonzalez Suarez","year":"2019","unstructured":"Gonzalez Suarez ML, Thongprayoon C, Mao MA, Leeaphorn N, Bathini T, Cheungpasitporn W. Outcomes of kidney transplant patients with atypical hemolytic uremic syndrome treated with eculizumab: a systematic review and meta-analysis. J Clin Med. 2019;8(7):919.","journal-title":"J Clin Med"},{"issue":"3","key":"1114_CR7","doi-asserted-by":"publisher","first-page":"434","DOI":"10.1016\/j.ekir.2018.11.010","volume":"4","author":"AM Siedlecki","year":"2018","unstructured":"Siedlecki AM, Isbel N, Vande Walle J, James Eggleston J, Cohen DJ. Global aHUS registry. Eculizumab use for kidney transplantation in patients with a diagnosis of atypical hemolytic uremic syndrome. Kidney Int Rep. 2018;4(3):434\u201346.","journal-title":"Kidney Int Rep"},{"issue":"18","key":"1114_CR8","doi-asserted-by":"publisher","first-page":"2053","DOI":"10.1007\/s40265-013-0147-7","volume":"73","author":"GM Keating","year":"2013","unstructured":"Keating GM, Eculizumab. A review of its use in atypical haemolytic uraemic syndrome. Drugs. 2013;73(18):2053\u201366.","journal-title":"Drugs"},{"issue":"23","key":"1114_CR9","doi-asserted-by":"publisher","first-page":"2169","DOI":"10.1056\/NEJMoa1208981","volume":"368","author":"CM Legendre","year":"2013","unstructured":"Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, Bingham C, Cohen DJ, Delmas Y, Douglas K, Eitner F, Feldkamp T, Fouque D, Furman RR, Gaber O, Herthelius M, Hourmant M, Karpman D, Lebranchu Y, Mariat C, Menne J, Moulin B, N\u00fcrnberger J, Ogawa M, Remuzzi G, Richard T, Sberro-Soussan R, Severino B, Sheerin NS, Trivelli A, Zimmerhackl LB, Goodship T, Loirat C. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013;368(23):2169\u201381.","journal-title":"N Engl J Med"},{"issue":"4","key":"1114_CR10","doi-asserted-by":"publisher","first-page":"994","DOI":"10.1097\/TP.0000000000004355","volume":"107","author":"EK Glover","year":"2023","unstructured":"Glover EK, Smith-Jackson K, Brocklebank V, Wilson V, Walsh PR, Montgomery EK, Wong EKS, Johnson S, Malina M, Kavanagh D, Sheerin NS, UK aHUS Transplant Consortium. Assessing the impact of prophylactic eculizumab on renal graft survival in atypical hemolytic uremic syndrome. Transplantation. 2023;107(4):994\u20131003.","journal-title":"Transplantation"},{"issue":"1","key":"1114_CR11","doi-asserted-by":"publisher","first-page":"2231264","DOI":"10.1080\/0886022X.2023.2231264","volume":"45","author":"ZC Tang","year":"2023","unstructured":"Tang ZC, Hui H, Shi C, Chen X. New findings in preventing recurrence and improving renal function in AHUS patients after renal transplantation treated with eculizumab: a systemic review and meta-analyses. Ren Fail. 2023;45(1):2231264.","journal-title":"Ren Fail"},{"issue":"1","key":"1114_CR12","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1186\/s12882-020-01847-0","volume":"21","author":"S Kant","year":"2020","unstructured":"Kant S, Bhalla A, Alasfar S, Alachkar N. Ten-year outcome of eculizumab in kidney transplant recipients with atypical hemolytic uremic syndrome - A single center experience. 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Ex vivo C5b-9 deposition test to monitor complement activity in clinical and subclinical atypical hemolytic uremic syndrome and in transplantation-associated thrombotic microangiopathy. Kidney Int Rep. 2024;9(7):2227\u201339.","journal-title":"Kidney Int Rep"}],"container-title":["CEN Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s13730-026-01114-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s13730-026-01114-5","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s13730-026-01114-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T05:10:29Z","timestamp":1776489029000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s13730-026-01114-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,18]]},"references-count":14,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2026,6]]}},"alternative-id":["1114"],"URL":"https:\/\/doi.org\/10.1007\/s13730-026-01114-5","relation":{},"ISSN":["2192-4449"],"issn-type":[{"value":"2192-4449","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,18]]},"assertion":[{"value":"25 December 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 March 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 April 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The case described in this article was enrolled in a study conducted by the Japanese Association for Complement Research which was funded by Alexion Pharma GK. However, the case report in this study was not requested by Alexion; while Alexion provided a review of the data for scientific accuracy, the authors retained control and final authority over publication content and decisions, including the choice of journal. Authors\u2019 declarations: Research funding (Masayoshi Miura), Leadership\/fiduciary role: Nobutaka Wakamiya (councilor of the Japanese Association for Complement Research; councilor of the International Complement Society). The other authors have declared no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"Specific ethical approval was not required for this case publication. The authors obtained written informed consent from the patient to publish the medical data included in this case report. The authors confirm that the study was carried out in accordance with the Declaration of Istanbul.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}}],"article-number":"72"}}