{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T02:37:46Z","timestamp":1792636666757,"version":"4.5.0"},"reference-count":54,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,10,22]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Atypical hemolytic uremic syndrome (aHUS) is a rare disorder that affects individuals of any age. It is caused by genetic abnormalities of the alternative complement pathway, arising from inherited or de novo mutations, or from acquired factors such as autoantibodies against complement proteins, including complement factor H. Clinically, aHUS is characterized by the classic triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. However, its clinical presentation may be difficult to distinguish from typical hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. aHUS is driven by uncontrolled activation of the alternative complement pathway, leading to endothelial injury and microvascular thrombosis.<\/jats:p>\n                  <jats:p>Atypical HUS may also occur after kidney transplantation, either as recurrence of the native disease or as de novo post-transplant thrombotic microangiopathy. The diagnosis of post-transplant aHUS relies on the presence of the above-mentioned classic triad and the exclusion of secondary causes such as thrombotic thrombocytopenic purpura and Shiga toxin\u2013associated HUS. The pathophysiology is similar, involving either genetic mutations affecting complement proteins or acquired dysregulation due to autoantibodies.<\/jats:p>\n                  <jats:p>Historically, plasma exchange was used to replace dysfunctional complement regulators and remove circulating autoantibodies, with variable success. After transplantation, the disorder may either recur or develop de novo. In the past, plasmapheresis was considered beneficial. More recently, the availability of eculizumab, an anti-C5 monoclonal antibody that inhibits terminal complement activation, has become the treatment of choice, either as monotherapy or with plasma exchange. However, uncertainties remain regarding the optimal duration and dosing of long-term therapy, particularly in transplant recipients.<\/jats:p>","DOI":"10.1093\/joneph\/aajag096","type":"journal-article","created":{"date-parts":[[2026,4,12]],"date-time":"2026-04-12T11:35:46Z","timestamp":1775993746000},"page":"1334-1341","source":"Crossref","is-referenced-by-count":0,"title":["Atypical hemolytic uremic syndrome in kidney transplantation"],"prefix":"10.1093","volume":"39","author":[{"given":"Claudio","family":"Ponticelli","sequence":"first","affiliation":[{"name":"Independent Researcher , via Ampere 126, Milan 20131 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriella","family":"Moroni","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, Humanitas University , Via Rita Levi Montalcini 4, 20072 Pieve Emanuele, Milan ,","place":["Italy"]},{"name":"Nephrology and Dialysis Division, IRCCS Humanitas Research Hospital , Via Manzoni 56, 20089 Rozzano, Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,9,15]]},"reference":[{"key":"2026102121374949200_aajag096-B1","doi-asserted-by":"publisher","first-page":"557","DOI":"10.2215\/CJN.11470918","article-title":"Etiology and outcomes of thrombotic microangiopathies","volume":"14","author":"Bayer","year":"2019","journal-title":"Clin J Am Soc Nephrol"},{"key":"2026102121374949200_aajag096-B2","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1111\/1744-9987.12763","article-title":"Atypical hemolytic- uremic syndrome: an update on pathophysiology, diagnosis, and treatment","volume":"23","author":"Raina","year":"2019","journal-title":"Ther Apher Dial"},{"key":"2026102121374949200_aajag096-B3","doi-asserted-by":"publisher","first-page":"1885","DOI":"10.1007\/s00467-023-06258-5","article-title":"Gastrointestinal involvement in STEC-associated hemolytic uremic syndrome: 10 years in a pediatric center","volume":"39","author":"Giordano","year":"2024","journal-title":"Pediatr Nephrol"},{"key":"2026102121374949200_aajag096-B4","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1016\/j.ekir.2020.10.009","article-title":"Complement in secondary thrombotic microangiopathy","volume":"6","author":"Palma","year":"2021","journal-title":"Kidney Int Rep"},{"key":"2026102121374949200_aajag096-B5","doi-asserted-by":"publisher","first-page":"1676","DOI":"10.1056\/NEJMra0902814","article-title":"Atypical hemolytic\u2013uremic syndrome","volume":"361","author":"Noris","year":"2009","journal-title":"N Engl J Med."},{"key":"2026102121374949200_aajag096-B6","doi-asserted-by":"publisher","first-page":"18807","DOI":"10.3390\/jcm13071880","article-title":"Hypertensive emergency with thrombotic microangiopathy or TTP? A case series and literature review","volume":"13","author":"Song","year":"2024","journal-title":"J Clin Med."},{"key":"2026102121374949200_aajag096-B7","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1186\/1750-1172-6-60","article-title":"Atypical hemolytic uremic syndrome","volume":"6","author":"Loirat","year":"2011","journal-title":"Orphanet J Rare Dis."},{"key":"2026102121374949200_aajag096-B8","doi-asserted-by":"publisher","first-page":"52","DOI":"10.3389\/fmed.2014.00052","article-title":"Atypical hemolytic uremic syndrome post-kidney transplantation: two case reports and review of the literature","volume":"1","author":"Alasfar","year":"2014","journal-title":"Front Med (Lausanne)"},{"key":"2026102121374949200_aajag096-B9","doi-asserted-by":"publisher","first-page":"128","DOI":"10.1053\/j.ackd.2019.11.003","article-title":"Peri- and post-operative evaluation and management of atypical hemolytic uremic syndrome (aHUS) in kidney transplantation","volume":"27","author":"Java","year":"2020","journal-title":"Adv Chronic Kidney Dis"},{"key":"2026102121374949200_aajag096-B10","doi-asserted-by":"publisher","first-page":"848","DOI":"10.1177\/0961203312439334","article-title":"Predictors of kidney biopsy complication among patients with systemic lupus erythematosus","volume":"21","author":"Chen","year":"2012","journal-title":"Lupus"},{"key":"2026102121374949200_aajag096-B11","doi-asserted-by":"publisher","first-page":"416","DOI":"10.1055\/s-0034-1375701","article-title":"Pathology of renal diseases associated with dysfunction of the alternative pathway of complement: c3 glomerulopathy and atypical hemolytic uremic syndrome (aHUS)","volume":"40","author":"Sethi","year":"2014","journal-title":"Semin Thromb Hemost"},{"key":"2026102121374949200_aajag096-B12","doi-asserted-by":"publisher","first-page":"S44","DOI":"10.1002\/ajh.26854","article-title":"Complement-driven hemolytic uremic syndrome","volume":"98","author":"Leon","year":"2023","journal-title":"Am J Hematol"},{"key":"2026102121374949200_aajag096-B13","doi-asserted-by":"publisher","first-page":"881","DOI":"10.2215\/CJN.0000000000000182","article-title":"Epidemiology, outcomes, and complement gene variants in secondary thrombotic microangiopathies","volume":"18","author":"Werion","year":"2023","journal-title":"Clin J Am Soc Nephrol"},{"key":"2026102121374949200_aajag096-B14","doi-asserted-by":"publisher","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":"2026102121374949200_aajag096-B15","doi-asserted-by":"publisher","first-page":"1844","DOI":"10.2215\/CJN.02210310","article-title":"Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype","volume":"5","author":"Noris","year":"2010","journal-title":"Clin J Am Soc Nephrol"},{"key":"2026102121374949200_aajag096-B16","doi-asserted-by":"publisher","first-page":"84","DOI":"10.1016\/j.thromres.2016.07.017","article-title":"Thrombomodulin enhances complement regulation through strong affinity interactions with factor H","volume":"145","author":"Heurich","year":"2016","journal-title":"Thromb Res"},{"key":"2026102121374949200_aajag096-B17","doi-asserted-by":"publisher","first-page":"345","DOI":"10.1056\/NEJMoa0810739","article-title":"Thrombomodulin mutations in atipical hemolytic-uremic syndrome","volume":"361","author":"Delvaeye","year":"2009","journal-title":"N Engl J Med"},{"key":"2026102121374949200_aajag096-B18","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":"2026102121374949200_aajag096-B19","doi-asserted-by":"publisher","first-page":"1061","DOI":"10.1172\/JCI99296","article-title":"Hyperfunctional complement C3 promotes C5-dependent atypical hemolytic uremic syndrome in mice","volume":"129","author":"Smith-Jackson","year":"2019","journal-title":"J Clin Invest"},{"key":"2026102121374949200_aajag096-B20","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1016\/j.kint.2019.02.038","article-title":"Terminal complement effectors in atypical hemolytic uremic syndrome: c5a, C5b-9, or a bit of both?","volume":"96","author":"Noris","year":"2019","journal-title":"Kidney Int"},{"key":"2026102121374949200_aajag096-B21","doi-asserted-by":"publisher","first-page":"622","DOI":"10.1038\/nrneph.2012.195","article-title":"STEC-HUS, atypical HUS and TTP are all diseases of complement activation","volume":"8","author":"Noris","year":"2012","journal-title":"Nat Rev Nephrol"},{"key":"2026102121374949200_aajag096-B22","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1007\/s00467-010-1556-4","article-title":"aHUS caused by complement dysregulation: new therapies on the horizon","volume":"26","author":"Waters","year":"2011","journal-title":"Pediatr Nephrol"},{"key":"2026102121374949200_aajag096-B23","doi-asserted-by":"publisher","first-page":"563","DOI":"10.1038\/nrneph.2016.99","article-title":"Anti-complement-factor H-associated glomerulopathies","volume":"12","author":"Durey","year":"2016","journal-title":"Nat Rev Nephrol"},{"key":"2026102121374949200_aajag096-B24","doi-asserted-by":"publisher","first-page":"6904858","DOI":"10.1155\/2021\/6904858","article-title":"Anti-factor H antibodies in Egyptian children with hemolytic uremic syndrome","volume":"2021","author":"Shawky","year":"2021","journal-title":"Int J Nephrol"},{"key":"2026102121374949200_aajag096-B25","doi-asserted-by":"publisher","first-page":"931210","DOI":"10.3389\/fimmu.2022.931210","article-title":"Anti-factor H antibody and its role in atypical hemolytic uremic syndrome","volume":"13","author":"Raina","year":"2022","journal-title":"Front Immunol"},{"key":"2026102121374949200_aajag096-B26","doi-asserted-by":"publisher","first-page":"336","DOI":"10.1203\/PDR.0b013e3181b1bd4a","article-title":"Atypical hemolytic uremic syndrome associated with complement factor H autoantibodies and CFHR1\/CFHR3 deficiency","volume":"66","author":"Lee","year":"2009","journal-title":"Pediatr Res"},{"key":"2026102121374949200_aajag096-B27","doi-asserted-by":"publisher","first-page":"1909","DOI":"10.1007\/s00467-024-06284-x","article-title":"Anti-factor B antibodies in atypical hemolytic uremic syndrome","volume":"39","author":"Khandelwal","year":"2024","journal-title":"Pediatr Nephrol"},{"key":"2026102121374949200_aajag096-B28","doi-asserted-by":"publisher","first-page":"911","DOI":"10.5301\/jn.5000146","article-title":"Posttransplant recurrence of atypical hemolytic uremic syndrome","volume":"25","author":"Valoti","year":"2012","journal-title":"J Nephrol"},{"key":"2026102121374949200_aajag096-B29","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1111\/nep.12776","article-title":"Prevention and treatment of atypical haemolytic uremic syndrome after kidney transplantation","volume":"21","author":"Okumi","year":"2016","journal-title":"Nephrology (Carlton)"},{"key":"2026102121374949200_aajag096-B30","doi-asserted-by":"publisher","first-page":"2527","DOI":"10.3390\/jcm14072527","article-title":"Atypical hemolytic uremic syndrome: a review of complement dysregulation, genetic susceptibility and multiorgan involvement","volume":"14","author":"Bogdan","year":"2025","journal-title":"J Clin Med"},{"key":"2026102121374949200_aajag096-B31","doi-asserted-by":"publisher","first-page":"1517","DOI":"10.1007\/s00467-008-0833-y","article-title":"Plasma therapy in atypical haemolytic uremic syndrome: lessons from a family with a factor H mutation","volume":"23","author":"Davin","year":"2008","journal-title":"Pediatr Nephrol"},{"key":"2026102121374949200_aajag096-B32","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1093\/bmb\/ldl004","article-title":"Atypical haemolytic uraemic syndrome","volume":"77\u201378","author":"Kavanagh","year":"2006","journal-title":"Br Med Bull"},{"key":"2026102121374949200_aajag096-B33","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1186\/s12882-019-1314-1","article-title":"Outcomes in patients with atypical hemolytic uremic syndrome treated with eculizumab in a long-term observational study","volume":"20","author":"Menne","year":"2019","journal-title":"BMC Nephrol"},{"key":"2026102121374949200_aajag096-B34","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":"Volume 16","author":"Yerigeri","year":"2023","journal-title":"J Multidiscip Healthc"},{"key":"2026102121374949200_aajag096-B35","doi-asserted-by":"publisher","first-page":"681","DOI":"10.1016\/S0140-6736(17)30062-4","article-title":"Haemolytic uraemic syndrome","volume":"390","author":"Fakhouri","year":"2017","journal-title":"Lancet"},{"key":"2026102121374949200_aajag096-B36","doi-asserted-by":"publisher","first-page":"643","DOI":"10.1038\/nrneph.2012.214","article-title":"Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies","volume":"8","author":"Zuber","year":"2012","journal-title":"Nat Rev Nephrol"},{"key":"2026102121374949200_aajag096-B37","doi-asserted-by":"publisher","first-page":"366","DOI":"10.1016\/j.jfma.2022.10.006","article-title":"Atypical hemolytic uremic syndrome: consensus of diagnosis and treatment in Taiwan","volume":"122","author":"Tseng","year":"2023","journal-title":"J FormosMed Assoc"},{"key":"2026102121374949200_aajag096-B38","doi-asserted-by":"publisher","first-page":"14389","DOI":"10.3390\/ijms241814389","article-title":"Immunosuppressive therapy of antibody-mediated aHUS and TTP","volume":"24","author":"Kelen","year":"2023","journal-title":"Int J Mol Sci"},{"key":"2026102121374949200_aajag096-B39","doi-asserted-by":"publisher","first-page":"1173","DOI":"10.1007\/s00467-004-1565-2","article-title":"Atypical relapse of hemolytic uremic syndrome after transplantation","volume":"19","author":"Olie","year":"2004","journal-title":"Pediatr Nephrol"},{"key":"2026102121374949200_aajag096-B40","doi-asserted-by":"publisher","first-page":"421","DOI":"10.1016\/j.nefro.2015.07.005","article-title":"An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document","volume":"35","author":"Campistol","year":"2015","journal-title":"Nefrologia (Engl Ed)."},{"key":"2026102121374949200_aajag096-B41","doi-asserted-by":"publisher","first-page":"104134","DOI":"10.1016\/j.drudis.2024.104134","article-title":"The efficacy and safety of eculizumab in patients and the role of C5 polymorphisms","volume":"29","author":"Bouwman","year":"2024","journal-title":"Drug Discov Today"},{"key":"2026102121374949200_aajag096-B42","doi-asserted-by":"publisher","first-page":"1658","DOI":"10.1016\/j.ekir.2019.08.020","article-title":"Use of eculizumab during pregnancy in kidney transplant recipients with atypical HUS","volume":"4","author":"Cheung","year":"2019","journal-title":"Kidney Int Rep"},{"key":"2026102121374949200_aajag096-B43","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1159\/000468528","article-title":"Hemolytic uremic syndrome and kidney transplantation: a case series and review of the literature","volume":"136","author":"Milan Manani","year":"2017","journal-title":"Nephron"},{"key":"2026102121374949200_aajag096-B44","doi-asserted-by":"publisher","first-page":"919","DOI":"10.3390\/jcm8070919","article-title":"Outcomes of kidney transplant patients with atypical hemolytic uremic syndrome treated with eculizumab: a systematic review and meta-analysis","volume":"8","author":"Gonzalez Suarez","year":"2019","journal-title":"J Clin Med"},{"key":"2026102121374949200_aajag096-B45","doi-asserted-by":"publisher","first-page":"e0258319","DOI":"10.1371\/journal.pone.0258319","article-title":"Thrombotic microangiopathy after kidney transplantation: analysis of the Brazilian atypical hemolytic uremic syndrome cohort","volume":"16","author":"Nga","year":"2021","journal-title":"PLoS One"},{"key":"2026102121374949200_aajag096-B46","doi-asserted-by":"publisher","first-page":"715","DOI":"10.1016\/j.ekir.2023.01.016","article-title":"Dutch aHUS working group. Eculizumab rescue therapy in patients with recurrent atypical hemolytic uremic syndrome after kidney transplantation","volume":"8","author":"Duineveld","year":"2023","journal-title":"Kidney Int Rep"},{"key":"2026102121374949200_aajag096-B47","doi-asserted-by":"publisher","first-page":"146","DOI":"10.1016\/j.transproceed.2019.10.015","article-title":"Atypical hemolytic uremic syndrome after kidney transplantation: lessons learned from the good, the bad, and the ugly. A case series with literature review","volume":"52","author":"Fayek","year":"2020","journal-title":"Transplant Proc"},{"key":"2026102121374949200_aajag096-B48","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1016\/j.transproceed.2016.10.013","article-title":"Efficacy of eculizumab therapy for atypical hemolytic uremic syndrome recurrence and antibody-mediated rejection progress after renal transplantation with preformed donor-specific antibodies: case report","volume":"49","author":"Yamamoto","year":"2017","journal-title":"Transplant Proc"},{"key":"2026102121374949200_aajag096-B49","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1186\/s12882-020-01847-0","article-title":"Ten-year outcome of eculizumab in kidney transplant recipients with atypical hemolytic uremic syndrome- a single center experience","volume":"21","author":"Kant","year":"2020","journal-title":"BMC Nephrol"},{"key":"2026102121374949200_aajag096-B50","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1111\/nep.12931","article-title":"Subclinical atypical haemolytic uremic syndrome relapse following discontinuation of eculizumab","volume":"22","author":"Choo","year":"2017","journal-title":"Nephro\u00adlogy (Carlton)"},{"key":"2026102121374949200_aajag096-B51","doi-asserted-by":"publisher","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":"2026102121374949200_aajag096-B52","doi-asserted-by":"publisher","first-page":"2438","DOI":"10.1182\/blood.2020009280","article-title":"Eculizumab discontinuation in children and adults with atypical hemolytic-uremic syndrome: a prospective multicenter study","volume":"137","author":"Fakhouri","year":"2021","journal-title":"Blood"},{"key":"2026102121374949200_aajag096-B53","doi-asserted-by":"publisher","first-page":"633","DOI":"10.1053\/j.ajkd.2014.01.434","article-title":"Discontinuation of eculizumab maintenance treatment for atypical hemolytic uremic syndrome: a report of 10 cases","volume":"64","author":"Ardissino","year":"2014","journal-title":"Am J Kidney Dis"},{"key":"2026102121374949200_aajag096-B54","doi-asserted-by":"publisher","first-page":"1223","DOI":"10.1111\/j.1600-6143.2009.02586.x","article-title":"Anti-factor H autoantibodies in a fifth renal transplant recipient with atypical hemolytic and uremic syndrome","volume":"9","author":"Le Quintrec","year":"2009","journal-title":"Am J Transplant"}],"container-title":["Journal of Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/joneph\/article-pdf\/39\/7\/1334\/71088239\/aajag096.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/joneph\/article-pdf\/39\/7\/1334\/71088239\/aajag096.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T01:37:53Z","timestamp":1792633073000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/joneph\/article\/39\/7\/1334\/8795937"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9]]},"references-count":54,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2026,9,15]]},"published-print":{"date-parts":[[2026,10,22]]}},"URL":"https:\/\/doi.org\/10.1093\/joneph\/aajag096","relation":{},"ISSN":["1121-8428","1724-6059"],"issn-type":[{"value":"1121-8428","type":"print"},{"value":"1724-6059","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,9]]},"published":{"date-parts":[[2026,9]]}}}