{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T16:34:51Z","timestamp":1792427691832,"version":"4.5.0"},"reference-count":162,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,5,23]],"date-time":"2025-05-23T00:00:00Z","timestamp":1747958400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000774","name":"Newcastle University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000774","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","jacionline.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Allergy and Clinical Immunology"],"published-print":{"date-parts":[[2025,8]]},"DOI":"10.1016\/j.jaci.2025.05.010","type":"journal-article","created":{"date-parts":[[2025,5,26]],"date-time":"2025-05-26T01:56:56Z","timestamp":1748224616000},"page":"224-236","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":10,"title":["Atypical hemolytic uremic syndrome"],"prefix":"10.1016","volume":"156","author":[{"given":"Patrick R.","family":"Walsh","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4718-0072","authenticated-orcid":false,"given":"David","family":"Kavanagh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jaci.2025.05.010_bib1","doi-asserted-by":"crossref","first-page":"300","DOI":"10.2215\/CJN.00620117","article-title":"Thrombotic microangiopathy and the kidney","volume":"13","author":"Brocklebank","year":"2018","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib2","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1016\/j.kint.2024.05.021","article-title":"An expert discussion on the atypical hemolytic uremic syndrome nomenclature\u2014identifying a road map to precision: a report of a National Kidney Foundation Working Group","volume":"106","author":"Nester","year":"2024","journal-title":"Kidney Int"},{"key":"10.1016\/j.jaci.2025.05.010_bib3","doi-asserted-by":"crossref","first-page":"836","DOI":"10.1111\/j.1523-1755.1998.00824.x","article-title":"Genetic studies into inherited and sporadic hemolytic uremic syndrome","volume":"53","author":"Warwicker","year":"1998","journal-title":"Kidney Int"},{"key":"10.1016\/j.jaci.2025.05.010_bib4","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/S0065-2776(07)96004-6","volume":"96","author":"Richards","year":"2007","journal-title":"Adv Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib5","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1016\/j.molimm.2009.09.005","article-title":"The down-stream effects of mannan-induced lectin complement pathway activation depend quantitatively on alternative pathway amplification","volume":"47","author":"Harboe","year":"2009","journal-title":"Mol Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib10","doi-asserted-by":"crossref","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":"10.1016\/j.jaci.2025.05.010_bib64","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1007\/s00467-016-3399-0","article-title":"Renal thrombotic microangiopathy in patients with cblC defect: review of an under-recognized entity","volume":"32","author":"Beck","year":"2016","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib67","doi-asserted-by":"crossref","first-page":"1260","DOI":"10.1016\/j.kint.2020.01.045","article-title":"Long-term outcomes and response to treatment in DGKE nephropathy","volume":"97","author":"Brocklebank","year":"2020","journal-title":"Kidney Int"},{"key":"10.1016\/j.jaci.2025.05.010_bib70","doi-asserted-by":"crossref","first-page":"7604","DOI":"10.3390\/ijms25147604","article-title":"Mutations in genes encoding subunits of the RNA exosome as a potential novel cause of thrombotic microangiopathy","volume":"25","author":"Wijnsma","year":"2024","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.jaci.2025.05.010_bib124","doi-asserted-by":"crossref","first-page":"3066","DOI":"10.1681\/ASN.2017010031","article-title":"The phenotypic spectrum of nephropathies associated with mutations in diacylglycerol kinase \u03b5","volume":"28","author":"Azukaitis","year":"2017","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib125","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1016\/j.ymgme.2009.09.008","article-title":"Newborn screening and early biochemical follow-up in combined methylmalonic aciduria and homocystinuria, cblC type, and utility of methionine as a secondary screening analyte","volume":"99","author":"Weisfeld-Adams","year":"2010","journal-title":"Mol Genet Metab"},{"key":"10.1016\/j.jaci.2025.05.010_bib6","doi-asserted-by":"crossref","first-page":"1249","DOI":"10.1084\/jem.20070301","article-title":"Spontaneous hemolytic uremic syndrome triggered by complement factor H lacking surface recognition domains","volume":"204","author":"Pickering","year":"2007","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2025.05.010_bib7","doi-asserted-by":"crossref","first-page":"554","DOI":"10.2215\/CJN.04760512","article-title":"Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults","volume":"8","author":"Fremeaux-Bacchi","year":"2013","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib8","doi-asserted-by":"crossref","first-page":"852","DOI":"10.1136\/jmg.2005.030783","article-title":"The development of atypical haemolytic-uraemic syndrome is influenced by susceptibility factors in factor H and membrane cofactor protein: evidence from two independent cohorts","volume":"42","author":"Fremeaux-Bacchi","year":"2005","journal-title":"J Med Genet"},{"key":"10.1016\/j.jaci.2025.05.010_bib9","doi-asserted-by":"crossref","first-page":"1558","DOI":"10.1182\/blood.2020006931","article-title":"Familial risk of developing atypical hemolytic-uremic syndrome","volume":"136","author":"Arjona","year":"2020","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib11","doi-asserted-by":"crossref","first-page":"2610","DOI":"10.4049\/jimmunol.181.4.2610","article-title":"A new map of glycosaminoglycan and C3b binding sites on factor H","volume":"181","author":"Schmidt","year":"2008","journal-title":"J Immunol"},{"issue":"7367","key":"10.1016\/j.jaci.2025.05.010_bib12","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1038\/nature10449","article-title":"Complement factor H binds malondialdehyde epitopes and protects from oxidative stress","volume":"478","author":"Weismann","year":"2011","journal-title":"Nature"},{"key":"10.1016\/j.jaci.2025.05.010_bib13","doi-asserted-by":"crossref","first-page":"30451","DOI":"10.1074\/jbc.M803648200","article-title":"Complement factor H binds to denatured rather than to native pentameric C-reactive protein","volume":"283","author":"Hakobyan","year":"2008","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2025.05.010_bib14","doi-asserted-by":"crossref","first-page":"1858","DOI":"10.4049\/jimmunol.1200357","article-title":"Atypical hemolytic uremic syndrome\u2013associated variants and autoantibodies impair binding of factor H and factor H\u2013related protein 1 to pentraxin 3","volume":"189","author":"Kopp","year":"2012","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib15","doi-asserted-by":"crossref","first-page":"10894","DOI":"10.1074\/jbc.M610256200","article-title":"The factor H variant associated with age-related macular degeneration (His-384) and the non\u2013disease-associated form bind differentially to C-reactive protein, fibromodulin, DNA, and necrotic cells","volume":"282","author":"Sjoberg","year":"2007","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2025.05.010_bib16","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1016\/j.semnephrol.2013.08.003","article-title":"Atypical hemolytic uremic syndrome","volume":"33","author":"Kavanagh","year":"2013","journal-title":"Semin Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib17","doi-asserted-by":"crossref","first-page":"7009","DOI":"10.4049\/jimmunol.0804031","article-title":"The binding of factor H to a complex of physiological polyanions and C3b on cells is impaired in atypical hemolytic uremic syndrome","volume":"182","author":"Ferreira","year":"2009","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib18","doi-asserted-by":"crossref","first-page":"2185","DOI":"10.1182\/blood.2021012037","article-title":"Functional characterization of 105 factor H variants associated with aHUS: lessons for variant classification","volume":"138","author":"Martin Merinero","year":"2021","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib19","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1182\/blood-2011-03-339903","article-title":"A novel hybrid CFH\/CFHR3 gene generated by a microhomology-mediated deletion in familial atypical hemolytic uremic syndrome","volume":"119","author":"Francis","year":"2012","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib20","doi-asserted-by":"crossref","first-page":"1617","DOI":"10.1681\/ASN.2015010100","article-title":"A de novo deletion in the regulators of complement activation cluster producing a hybrid complement factor H\/complement factor H\u2013related 3 gene in atypical hemolytic uremic syndrome","volume":"27","author":"Challis","year":"2016","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib21","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1016\/j.jmoldx.2022.02.006","article-title":"Unraveling structural rearrangements of the CFH gene cluster in atypical hemolytic uremic syndrome patients using molecular combing and long-fragment targeted sequencing","volume":"24","author":"Tschernoster","year":"2022","journal-title":"J Mol Diagn"},{"key":"10.1016\/j.jaci.2025.05.010_bib22","doi-asserted-by":"crossref","first-page":"5283","DOI":"10.1093\/hmg\/ddu226","article-title":"Whole-exome sequencing identifies rare, functional CFH variants in families with macular degeneration","volume":"23","author":"Yu","year":"2014","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.jaci.2025.05.010_bib23","doi-asserted-by":"crossref","first-page":"1366","DOI":"10.1038\/ng.2741","article-title":"Rare variants in CFI, C3 and C9 are associated with high risk of advanced age-related macular degeneration","volume":"45","author":"Seddon","year":"2013","journal-title":"Nat Genet"},{"key":"10.1016\/j.jaci.2025.05.010_bib24","doi-asserted-by":"crossref","first-page":"2425","DOI":"10.1681\/ASN.2013070732","article-title":"Characterization of a factor H mutation that perturbs the alternative pathway of complement in a family with membranoproliferative GN","volume":"25","author":"Wong","year":"2014","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib25","doi-asserted-by":"crossref","first-page":"2180","DOI":"10.1681\/ASN.2010030315","article-title":"Clinical features of anti\u2013factor H autoantibody\u2013associated hemolytic uremic syndrome","volume":"21","author":"Dragon-Durey","year":"2010","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib26","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1016\/j.kint.2017.04.028","article-title":"Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland","volume":"92","author":"Brocklebank","year":"2017","journal-title":"Kidney Int"},{"key":"10.1016\/j.jaci.2025.05.010_bib27","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.3389\/fimmu.2019.01282","article-title":"Clinical and immunological profile of anti\u2013factor H antibody associated atypical hemolytic uremic syndrome: a nationwide database","volume":"10","author":"Puraswani","year":"2019","journal-title":"Front Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib28","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1182\/blood-2009-05-221549","article-title":"Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome","volume":"115","author":"Moore","year":"2010","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib29","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.molimm.2006.07.004","article-title":"Implications of the initial mutations in membrane cofactor protein (MCP; CD46) leading to atypical hemolytic uremic syndrome","volume":"44","author":"Richards","year":"2007","journal-title":"Mol Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib30","doi-asserted-by":"crossref","first-page":"2017","DOI":"10.1681\/ASN.2005101051","article-title":"Genetic and functional analyses of membrane cofactor protein (CD46) mutations in atypical hemolytic uremic syndrome","volume":"17","author":"Fremeaux-Bacchi","year":"2006","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib31","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1182\/blood-2005-10-007252","article-title":"Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome","volume":"108","author":"Caprioli","year":"2006","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib32","doi-asserted-by":"crossref","DOI":"10.1016\/j.imbio.2023.152410","article-title":"Complement factor I: regulatory nexus, driver of immunopathology, and therapeutic","volume":"228","author":"Hallam","year":"2023","journal-title":"Immunobiology"},{"key":"10.1016\/j.jaci.2025.05.010_bib33","doi-asserted-by":"crossref","first-page":"2809","DOI":"10.1681\/ASN.2018070759","article-title":"Genetic analysis of 400 patients refines understanding and implicates a new gene in atypical hemolytic uremic syndrome","volume":"29","author":"Bu","year":"2018","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib34","doi-asserted-by":"crossref","first-page":"2464","DOI":"10.4049\/jimmunol.1701695","article-title":"Statistical validation of rare complement variants provides insights into the molecular basis of atypical hemolytic uremic syndrome and C3 glomerulopathy","volume":"200","author":"Osborne","year":"2018","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib35","doi-asserted-by":"crossref","first-page":"2678","DOI":"10.1093\/hmg\/ddac060","article-title":"Rare complement factor I variants associated with reduced macular thickness and age-related macular degeneration in the UK Biobank","volume":"31","author":"Tzoumas","year":"2022","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.jaci.2025.05.010_bib36","first-page":"3861","article-title":"Rare genetic variants in the CFI gene are associated with advanced age-related macular degeneration and commonly result in reduced serum factor I levels","volume":"24","author":"Kavanagh","year":"2015","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.jaci.2025.05.010_bib37","doi-asserted-by":"crossref","first-page":"417","DOI":"10.2215\/CJN.05750611","article-title":"Factor I autoantibodies in patients with atypical hemolytic uremic syndrome: disease-associated or an epiphenomenon?","volume":"7","author":"Kavanagh","year":"2012","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib38","doi-asserted-by":"crossref","DOI":"10.1016\/j.imbio.2020.152000","article-title":"Anti\u2013complement factor I antibody associated atypical hemolytic uremic syndrome\u2014a new insight for future perspective","volume":"225","author":"Govindarajan","year":"2020","journal-title":"Immunobiology"},{"key":"10.1016\/j.jaci.2025.05.010_bib39","doi-asserted-by":"crossref","first-page":"4948","DOI":"10.1182\/blood-2008-01-133702","article-title":"Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome","volume":"112","author":"Fremeaux-Bacchi","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib40","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1073\/pnas.0603420103","article-title":"Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome","volume":"104","author":"Goicoechea de Jorge","year":"2007","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2025.05.010_bib41","doi-asserted-by":"crossref","first-page":"1909","DOI":"10.1007\/s00467-024-06284-x","article-title":"Anti\u2013factor B antibodies in atypical hemolytic uremic syndrome","volume":"39","author":"Khandelwal","year":"2024","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib42","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1681\/ASN.2010050451","article-title":"The development of atypical hemolytic uremic syndrome depends on complement C5","volume":"22","author":"de Jorge","year":"2011","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib43","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.kint.2019.01.009","article-title":"Differential contribution of C5aR and C5b-9 pathways to renal thrombic microangiopathy and macrovascular thrombosis in mice carrying an atypical hemolytic syndrome\u2013related factor H mutation","volume":"96","author":"Ueda","year":"2019","journal-title":"Kidney Int"},{"key":"10.1016\/j.jaci.2025.05.010_bib44","doi-asserted-by":"crossref","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":"10.1016\/j.jaci.2025.05.010_bib45","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1016\/j.kint.2024.12.016","article-title":"The membrane attack complex drives thrombotic microangiopathy in complement mediated atypical hemolytic uremic syndrome","volume":"107","author":"Smith-Jackson","year":"2025","journal-title":"Kidney Int"},{"key":"10.1016\/j.jaci.2025.05.010_bib46","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.ekir.2016.08.015","article-title":"Complement activation induces neutrophil adhesion and neutrophil\u2013platelet aggregate formation on vascular endothelial cells","volume":"2","author":"Riedl","year":"2017","journal-title":"Kidney Int Rep"},{"key":"10.1016\/j.jaci.2025.05.010_bib47","doi-asserted-by":"crossref","first-page":"5307","DOI":"10.1182\/blood-2007-08-106153","article-title":"Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation","volume":"111","author":"St\u00e5hl","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib48","doi-asserted-by":"crossref","first-page":"1586","DOI":"10.3389\/fimmu.2017.01586","article-title":"Factor H C-terminal domains are critical for regulation of platelet\/granulocyte aggregate formation","volume":"8","author":"Blatt","year":"2017","journal-title":"Frontiers in Immunology"},{"key":"10.1016\/j.jaci.2025.05.010_bib49","doi-asserted-by":"crossref","first-page":"1619","DOI":"10.1084\/jem.185.9.1619","article-title":"The cytolytically inactive terminal complement complex activates endothelial cells to express adhesion molecules and tissue factor procoagulant activity","volume":"185","author":"Tedesco","year":"1997","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2025.05.010_bib50","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1182\/blood.V76.2.361.361","article-title":"Consecutive enzyme cascades: complement activation at the cell surface triggers increased tissue factor activity","volume":"76","author":"Carson","year":"1990","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib51","doi-asserted-by":"crossref","first-page":"1954","DOI":"10.1182\/blood.2020007206","article-title":"Complement and the prothrombotic state","volume":"139","author":"Schmidt","year":"2022","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib52","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1182\/blood-2013-03-489245","article-title":"Complement activation by heme as a secondary hit for atypical hemolytic uremic syndrome","volume":"122","author":"Frimat","year":"2013","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib53","doi-asserted-by":"crossref","first-page":"2169","DOI":"10.1056\/NEJMoa1208981","article-title":"Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome","volume":"368","author":"Legendre","year":"2013","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jaci.2025.05.010_bib54","doi-asserted-by":"crossref","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":"10.1016\/j.jaci.2025.05.010_bib55","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/j.ekir.2022.10.013","article-title":"Early eculizumab withdrawal in patients with atypical hemolytic uremic syndrome in native kidneys is safe and cost-effective: results of the CUREiHUS study","volume":"8","author":"Bouwmeester","year":"2023","journal-title":"Kidney Int Rep"},{"key":"10.1016\/j.jaci.2025.05.010_bib56","doi-asserted-by":"crossref","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":"10.1016\/j.jaci.2025.05.010_bib57","doi-asserted-by":"crossref","first-page":"1504","DOI":"10.1182\/bloodadvances.2020003175","article-title":"Outcomes of a clinician-directed protocol for discontinuation of complement inhibition therapy in atypical hemolytic uremic syndrome","volume":"5","author":"Chaturvedi","year":"2021","journal-title":"Blood Adv"},{"key":"10.1016\/j.jaci.2025.05.010_bib58","doi-asserted-by":"crossref","first-page":"1332","DOI":"10.1016\/j.ekir.2023.04.029","article-title":"Design and rationale of the APPELHUS phase 3 open-label study of factor B inhibitor iptacopan for atypical hemolytic uremic syndrome","volume":"8","author":"Kavanagh","year":"2023","journal-title":"Kidney Int Rep"},{"key":"10.1016\/j.jaci.2025.05.010_bib59","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1055\/s-0030-1262887","article-title":"Transplantation in atypical hemolytic uremic syndrome","volume":"36","author":"Kavanagh","year":"2010","journal-title":"Semin Thromb Hemost"},{"key":"10.1016\/j.jaci.2025.05.010_bib60","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1111\/ajt.12077","article-title":"Complement genes strongly predict recurrence and graft outcome in adult renal transplant recipients with atypical hemolytic and uremic syndrome","volume":"13","author":"Le Quintrec","year":"2013","journal-title":"Am J Transplant"},{"key":"10.1016\/j.jaci.2025.05.010_bib61","doi-asserted-by":"crossref","first-page":"994","DOI":"10.1097\/TP.0000000000004355","article-title":"Assessing the impact of prophylactic eculizumab on renal graft survival in atypical hemolytic uremic syndrome","volume":"107","author":"Glover","year":"2023","journal-title":"Transplantation"},{"key":"10.1016\/j.jaci.2025.05.010_bib62","doi-asserted-by":"crossref","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":"10.1016\/j.jaci.2025.05.010_bib63","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1097\/TP.0000000000005135","article-title":"Kidney transplantation in patients with aHUS: a comparison of eculizumab prophylaxis versus rescue therapy","volume":"109","author":"Duineveld","year":"2024","journal-title":"Transplantation"},{"key":"10.1016\/j.jaci.2025.05.010_bib65","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1136\/jmedgenet-2011-100286","article-title":"Novel inborn error of folate metabolism: identification by exome capture and sequencing of mutations in the MTHFD1 gene in a single proband","volume":"48","author":"Watkins","year":"2011","journal-title":"J Med Genet"},{"key":"10.1016\/j.jaci.2025.05.010_bib66","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1038\/ng.2590","article-title":"Recessive mutations in DGKE cause atypical hemolytic-uremic syndrome","volume":"45","author":"Lemaire","year":"2013","journal-title":"Nat Genet"},{"key":"10.1016\/j.jaci.2025.05.010_bib68","doi-asserted-by":"crossref","first-page":"6186","DOI":"10.1021\/cr1004106","article-title":"Regulation and functions of diacylglycerol kinases","volume":"111","author":"Shulga","year":"2011","journal-title":"Chem Rev"},{"key":"10.1016\/j.jaci.2025.05.010_bib69","doi-asserted-by":"crossref","DOI":"10.1172\/jci.insight.146959","article-title":"Loss of diacylglycerol kinase \u03b5 causes thrombotic microangiopathy by impairing endothelial VEGFA signaling","volume":"6","author":"Liu","year":"2021","journal-title":"JCI Insight"},{"key":"10.1016\/j.jaci.2025.05.010_bib71","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1016\/j.tibs.2013.06.013","article-title":"Threading the barrel of the RNA exosome","volume":"38","author":"Schneider","year":"2013","journal-title":"Trends Biochem Sci"},{"key":"10.1016\/j.jaci.2025.05.010_bib72","doi-asserted-by":"crossref","DOI":"10.1002\/wrna.1766","article-title":"Caught in the act\u2014visualizing ribonucleases during eukaryotic ribosome assembly","volume":"14","author":"Schneider","year":"2023","journal-title":"Wiley Interdiscip Rev RNA"},{"key":"10.1016\/j.jaci.2025.05.010_bib73","doi-asserted-by":"crossref","first-page":"9397","DOI":"10.1093\/nar\/gkad637","article-title":"The induction of p53 correlates with defects in the production, but not the levels, of the small ribosomal subunit and stalled large ribosomal subunit biogenesis","volume":"51","author":"Eastham","year":"2023","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.jaci.2025.05.010_bib74","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1111\/cge.14105","article-title":"A splice site mutation in the TSEN2 causes a new syndrome with craniofacial and central nervous system malformations, and atypical hemolytic uremic syndrome","volume":"101","author":"Canpolat","year":"2022","journal-title":"Clin Genet"},{"key":"10.1016\/j.jaci.2025.05.010_bib75","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1016\/j.tig.2020.08.017","article-title":"Genetic lesions of type I interferon signalling in human antiviral immunity","volume":"37","author":"Duncan","year":"2021","journal-title":"Trends Genet"},{"key":"10.1016\/j.jaci.2025.05.010_bib76","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1038\/nri3787","article-title":"Type I interferons in infectious disease","volume":"15","author":"McNab","year":"2015","journal-title":"Nat Rev Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib77","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1038\/s41577-021-00633-9","article-title":"The type I interferonopathies: 10 years on","volume":"22","author":"Crow","year":"2022","journal-title":"Nat Rev Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib78","doi-asserted-by":"crossref","DOI":"10.1126\/sciimmunol.aav7501","article-title":"Severe type I interferonopathy and unrestrained interferon signaling due to a homozygous germline mutation in STAT2","volume":"4","author":"Duncan","year":"2019","journal-title":"Sci Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib79","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1016\/j.jaci.2021.03.045","article-title":"Multisystem inflammation and susceptibility to viral infections in human ZNFX1 deficiency","volume":"148","author":"Vavassori","year":"2021","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib80","doi-asserted-by":"crossref","first-page":"1270","DOI":"10.1056\/NEJMc1316118","article-title":"Thrombotic microangiopathy associated with interferon beta","volume":"370","author":"Hunt","year":"2014","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jaci.2025.05.010_bib81","doi-asserted-by":"crossref","first-page":"2824","DOI":"10.1182\/blood-2016-05-715987","article-title":"Type I interferon causes thrombotic microangiopathy by a dose-dependent toxic effect on the microvasculature","volume":"128","author":"Kavanagh","year":"2016","journal-title":"Blood"},{"issue":"9464","key":"10.1016\/j.jaci.2025.05.010_bib82","first-page":"1073","article-title":"Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome","volume":"365","author":"Tarr","year":"2005","journal-title":"Lancet"},{"key":"10.1016\/j.jaci.2025.05.010_bib83","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0006990","article-title":"Shiga toxin and lipopolysaccharide induce platelet\u2013leukocyte aggregates and tissue factor release, a thrombotic mechanism in hemolytic uremic syndrome","volume":"4","author":"St\u00e5hl","year":"2009","journal-title":"PLoS One"},{"key":"10.1016\/j.jaci.2025.05.010_bib84","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1128\/JCM.44.2.313-317.2006","article-title":"Shiga toxins present in the gut and in the polymorphonuclear leukocytes circulating in the blood of children with hemolytic-uremic syndrome","volume":"44","author":"Brigotti","year":"2006","journal-title":"J Clin Microbiol"},{"key":"10.1016\/j.jaci.2025.05.010_bib85","doi-asserted-by":"crossref","first-page":"2769","DOI":"10.3390\/toxins2122769","article-title":"Escherichia coli Shiga toxin mechanisms of action in renal disease","volume":"2","author":"Obrig","year":"2010","journal-title":"Toxins (Basel)"},{"key":"10.1016\/j.jaci.2025.05.010_bib86","doi-asserted-by":"crossref","first-page":"2239","DOI":"10.1097\/01.ASN.0000027873.85792.52","article-title":"Molecular basis for high renal cell sensitivity to the cytotoxic effects of shigatoxin-1: upregulation of globotriaosylceramide expression","volume":"13","author":"Hughes","year":"2002","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib87","doi-asserted-by":"crossref","first-page":"364","DOI":"10.2215\/CJN.05830518","article-title":"Complement gene variants and Shiga toxin\u2013producing Escherichia coli\u2013associated hemolytic uremic syndrome: retrospective genetic and clinical study","volume":"14","author":"Fr\u00e9meaux-Bacchi","year":"2019","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib88","doi-asserted-by":"crossref","first-page":"1561","DOI":"10.1681\/ASN.0000000000000182","article-title":"Efficacy and safety of eculizumab in pediatric patients affected by Shiga toxin\u2013related hemolytic and uremic syndrome: a randomized, placebo-controlled trial","volume":"34","author":"Garnier","year":"2023","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib89","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1097\/INF.0b013e3182191c58","article-title":"Streptococcus pneumoniae\u2013associated hemolytic uremic syndrome among children in North America","volume":"30","author":"Banerjee","year":"2011","journal-title":"Pediatr Infect Dis J"},{"issue":"8046","key":"10.1016\/j.jaci.2025.05.010_bib90","doi-asserted-by":"crossref","first-page":"1024","DOI":"10.1016\/S0140-6736(77)92915-4","article-title":"Thomsen-Friedenreich antigen in haemolytic-uraemic syndrome","volume":"2","author":"Klein","year":"1977","journal-title":"Lancet"},{"key":"10.1016\/j.jaci.2025.05.010_bib91","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1038\/nchembio.1696","article-title":"Structural basis for sialic acid\u2013mediated self-recognition by complement factor H","volume":"11","author":"Blaum","year":"2015","journal-title":"Nat Chem Biol"},{"key":"10.1016\/j.jaci.2025.05.010_bib92","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1093\/bmb\/ldl004","article-title":"Atypical haemolytic uraemic syndrome","volume":"77-78","author":"Kavanagh","year":"2006","journal-title":"Br Med Bull"},{"key":"10.1016\/j.jaci.2025.05.010_bib93","doi-asserted-by":"crossref","first-page":"753","DOI":"10.3109\/0886022X.2010.486491","article-title":"Triggering of atypical hemolytic uremic syndrome by influenza A (H1N1)","volume":"32","author":"Bento","year":"2010","journal-title":"Ren Fail"},{"key":"10.1016\/j.jaci.2025.05.010_bib94","doi-asserted-by":"crossref","first-page":"1777","DOI":"10.1182\/blood.2021012752","article-title":"COVID-19 as a potential trigger of complement-mediated atypical HUS","volume":"138","author":"El Sissy","year":"2021","journal-title":"Blood"},{"issue":"suppl 5","key":"10.1016\/j.jaci.2025.05.010_bib95","doi-asserted-by":"crossref","first-page":"S267","DOI":"10.1086\/422363","article-title":"HIV-associated thrombotic microangiopathy in the era of highly active antiretroviral therapy: an observational study","volume":"39","author":"Becker","year":"2004","journal-title":"Clin Infect Dis"},{"key":"10.1016\/j.jaci.2025.05.010_bib96","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1056\/NEJMra020528","article-title":"Thrombotic microangiopathies","volume":"347","author":"Moake","year":"2002","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jaci.2025.05.010_bib97","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1111\/jth.13189","article-title":"The role of ADAMTS-13 activity and complement mutational analysis in differentiating acute thrombotic microangiopathies","volume":"14","author":"Phillips","year":"2016","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jaci.2025.05.010_bib98","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1056\/NEJMoa1806311","article-title":"Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura","volume":"380","author":"Scully","year":"2019","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jaci.2025.05.010_bib99","doi-asserted-by":"crossref","first-page":"1855","DOI":"10.1182\/blood.2020009150","article-title":"Redefining outcomes in immune TTP: an international working group consensus report","volume":"137","author":"Cuker","year":"2021","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib100","doi-asserted-by":"crossref","DOI":"10.1172\/JCI173510","article-title":"Thrombotic microangiopathy following systemic AAV administration is dependent on anti-capsid antibodies","volume":"134","author":"Salabarria","year":"2024","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jaci.2025.05.010_bib126","doi-asserted-by":"crossref","first-page":"3059","DOI":"10.1002\/1097-0142(19941201)74:11<3059::AID-CNCR2820741125>3.0.CO;2-Z","article-title":"Cisplatin-associated hemolytic uremic syndrome","volume":"74","author":"Canpolat","year":"1994","journal-title":"Cancer"},{"key":"10.1016\/j.jaci.2025.05.010_bib127","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1097\/MCG.0b013e31802da22e","article-title":"Hemolytic-uremic syndrome associated with gemcitabine treatment for metastatic pancreatic cancer","volume":"42","author":"Boeck","year":"2008","journal-title":"J Clin Gastroenterol"},{"key":"10.1016\/j.jaci.2025.05.010_bib128","first-page":"175","article-title":"Thrombotic thrombocytopenic purpura caused by ticlopidine, successfully treated by plasmapheresis","volume":"54","author":"Ariyoshi","year":"1997","journal-title":"Am J Hematol"},{"key":"10.1016\/j.jaci.2025.05.010_bib129","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1002\/(SICI)1096-8652(199908)61:4<268::AID-AJH9>3.0.CO;2-O","article-title":"Thrombotic thrombocytopenic purpura\u2013hemolytic uremic syndrome (TTP-HUS) following treatment with deoxycoformycin in a patient with cutaneous T-cell lymphoma (Sezary syndrome): a case report","volume":"61","author":"Leach","year":"1999","journal-title":"Am J Hematol"},{"key":"10.1016\/j.jaci.2025.05.010_bib130","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1002\/ajh.23960","article-title":"Drug-induced thrombotic microangiopathy: experience of the Oklahoma Registry and the BloodCenter of Wisconsin","volume":"90","author":"Reese","year":"2015","journal-title":"Am J Hematol"},{"key":"10.1016\/j.jaci.2025.05.010_bib131","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1097\/00043426-200009000-00024","article-title":"Hemolytic uremic syndrome secondary to the treatment of acute lymphoblastic leukemia","volume":"22","author":"Kanchi","year":"2000","journal-title":"J Pediatr Hematol Oncol"},{"key":"10.1016\/j.jaci.2025.05.010_bib132","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1007\/s12185-008-0140-1","article-title":"Thrombotic microangiopathy after treatment with bortezomib and dexamethasone in a patient with multiple myeloma","volume":"88","author":"Morita","year":"2008","journal-title":"Int J Hematol"},{"key":"10.1016\/j.jaci.2025.05.010_bib133","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1182\/blood-2012-10-459883","article-title":"Carfilzomib","volume":"121","author":"Kortuem","year":"2013","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib134","doi-asserted-by":"crossref","first-page":"E53","DOI":"10.1002\/ajh.24662","article-title":"Ixazomib-induced thrombotic microangiopathy","volume":"92","author":"Yui","year":"2017","journal-title":"Am J Hematol"},{"key":"10.1016\/j.jaci.2025.05.010_bib135","doi-asserted-by":"crossref","first-page":"211","DOI":"10.4143\/crt.2008.40.4.211","article-title":"TTP-HUS associated with sunitinib","volume":"40","author":"Choi","year":"2008","journal-title":"Cancer Res Treat"},{"key":"10.1016\/j.jaci.2025.05.010_bib136","doi-asserted-by":"crossref","first-page":"e57","DOI":"10.1136\/hrt.2004.039214","article-title":"Thrombotic thrombocytopenic purpura\/haemolytic uraemic syndrome associated with clopidogrel: report of two new cases","volume":"90","author":"Andersohn","year":"2004","journal-title":"Heart"},{"key":"10.1016\/j.jaci.2025.05.010_bib137","doi-asserted-by":"crossref","first-page":"S20","DOI":"10.1038\/ki.2008.613","article-title":"Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989-2008)","volume":"112","author":"Zakarija","year":"2009","journal-title":"Kidney Int Suppl"},{"key":"10.1016\/j.jaci.2025.05.010_bib138","doi-asserted-by":"crossref","first-page":"243","DOI":"10.7326\/0003-4819-119-3-199308010-00012","article-title":"Quinine sensitivity: a new cause of the hemolytic uremic syndrome","volume":"119","author":"Aster","year":"1993","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.jaci.2025.05.010_bib139","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1097\/00000441-200312000-00015","article-title":"Quinine induced HUS-TTP: an unusual presentation","volume":"326","author":"Baliga","year":"2003","journal-title":"Am J Med Sci"},{"key":"10.1016\/j.jaci.2025.05.010_bib140","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1002\/ajh.2830470407","article-title":"Quinine-induced immune thrombocytopenia with hemolytic uremic syndrome: clinical and serological findings in nine patients and review of literature","volume":"47","author":"Gottschall","year":"1994","journal-title":"Am J Hematol"},{"key":"10.1016\/j.jaci.2025.05.010_bib141","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1007\/s13730-020-00536-z","article-title":"Quetiapine-induced thrombotic microangiopathy in a patient on maintenance dialysis","volume":"10","author":"Takahashi","year":"2021","journal-title":"CEN Case Rep"},{"key":"10.1016\/j.jaci.2025.05.010_bib142","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1080\/1042819021000055066","article-title":"Vascular events associated with alpha interferon therapy","volume":"44","author":"Al-Zahrani","year":"2003","journal-title":"Leuk Lymphoma"},{"key":"10.1016\/j.jaci.2025.05.010_bib143","doi-asserted-by":"crossref","first-page":"97","DOI":"10.2147\/IJNRD.S30194","article-title":"Thrombotic microangiopathy associated with use of interferon-beta","volume":"5","author":"Olea","year":"2012","journal-title":"Int J Nephrol Renovasc Dis"},{"key":"10.1016\/j.jaci.2025.05.010_bib144","doi-asserted-by":"crossref","first-page":"396","DOI":"10.5414\/CN106523","article-title":"Thrombotic microangiopathy induced by long-term interferon-beta therapy for multiple sclerosis: a case report","volume":"76","author":"Broughton","year":"2011","journal-title":"Clin Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib145","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1159\/000045147","article-title":"Hemolytic uremic syndrome associated with beta-interferon therapy for chronic hepatitis C","volume":"80","author":"Ubara","year":"1998","journal-title":"Nephron"},{"key":"10.1016\/j.jaci.2025.05.010_bib146","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1093\/oxfordjournals.ndt.a090823","article-title":"Renal thrombotic microangiopathy in patients with chronic myelogenous leukaemia treated with interferon-alpha 2b","volume":"10","author":"Jadoul","year":"1995","journal-title":"Nephrol Dial Transplant"},{"key":"10.1016\/j.jaci.2025.05.010_bib147","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1093\/ndt\/16.4.846","article-title":"Renal thrombotic microangiopathy induced by interferon-alpha","volume":"16","author":"Badid","year":"2001","journal-title":"Nephrol Dial Transplant"},{"key":"10.1016\/j.jaci.2025.05.010_bib148","doi-asserted-by":"crossref","first-page":"2111","DOI":"10.1093\/ndt\/16.10.2111-a","article-title":"Renal thrombotic microangiopathy induced by interferon-alpha","volume":"16","author":"Magee","year":"2001","journal-title":"Nephrol Dial Transplant"},{"key":"10.1016\/j.jaci.2025.05.010_bib149","doi-asserted-by":"crossref","DOI":"10.1136\/bmj.e3838","article-title":"Beware renal adverse effects of anti\u2013vascular endothelial growth factor treatment","volume":"344","author":"Keir","year":"2012","journal-title":"BMJ"},{"key":"10.1016\/j.jaci.2025.05.010_bib150","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1111\/j.1432-2277.2006.00445.x","article-title":"Hemolytic uremic syndrome following Campath-1H induction","volume":"20","author":"Bonatti","year":"2007","journal-title":"Transpl Int"},{"key":"10.1016\/j.jaci.2025.05.010_bib151","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1111\/j.1432-2277.2000.tb01023.x","article-title":"Hemolytic-uremic syndrome in association with both cyclosporine and tacrolimus","volume":"13","author":"Abraham","year":"2000","journal-title":"Transpl Int"},{"issue":"8415","key":"10.1016\/j.jaci.2025.05.010_bib152","doi-asserted-by":"crossref","first-page":"1337","DOI":"10.1016\/S0140-6736(84)90842-0","article-title":"Cyclosporin-induced haemolytic uraemic syndrome in liver allograft recipient","volume":"2","author":"Bonser","year":"1984","journal-title":"Lancet"},{"key":"10.1016\/j.jaci.2025.05.010_bib153","doi-asserted-by":"crossref","first-page":"2441","DOI":"10.1111\/j.1600-6143.2005.01047.x","article-title":"Sirolimus-induced thrombotic microangiopathy is associated with decreased expression of vascular endothelial growth factor in kidneys","volume":"5","author":"Sartelet","year":"2005","journal-title":"Am J Transplant"},{"key":"10.1016\/j.jaci.2025.05.010_bib154","article-title":"Everolimus-associated thrombotic microangiopathy following renal transplant: a case report","volume":"16","author":"Leong","year":"2024","journal-title":"Cureus"},{"key":"10.1016\/j.jaci.2025.05.010_bib155","doi-asserted-by":"crossref","first-page":"829","DOI":"10.2215\/CJN.03930907","article-title":"Higher levels of leflunomide are associated with hemolysis and are not superior to lower levels for BK virus clearance in renal transplant patients","volume":"3","author":"Leca","year":"2008","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib156","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1182\/blood-2014-11-611335","article-title":"Drug-induced thrombotic microangiopathy: a systematic review of published reports","volume":"125","author":"Al-Nouri","year":"2015","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib157","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.1345\/aph.1A350","article-title":"Ciprofloxacin-associated hemolytic-uremic syndrome","volume":"36","author":"Allan","year":"2002","journal-title":"Ann Pharmacother"},{"key":"10.1016\/j.jaci.2025.05.010_bib158","article-title":"Drug-induced thrombotic microangiopathy: an updated review of causative drugs, pathophysiology, and management","volume":"13","author":"Mazzierli","year":"2022","journal-title":"Front Pharmacol"},{"key":"10.1016\/j.jaci.2025.05.010_bib159","first-page":"1324","article-title":"[Hemolytic-uremic syndrome in a young woman following the use of ovulation inhibitors]","volume":"105","author":"Blumberg","year":"1975","journal-title":"Schweiz Med Wochenschr"},{"key":"10.1016\/j.jaci.2025.05.010_bib160","first-page":"212","article-title":"Hemolytic uremic syndrome in two postmenopausal women taking a conjugated estrogen preparation","volume":"17","author":"Ashouri","year":"1982","journal-title":"Clin Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib161","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1002\/ajh.10162","article-title":"A post-menopausal woman with anuria and uterus bulk: the spectrum of estrogen-induced TTP\/HUS","volume":"71","author":"Au","year":"2002","journal-title":"Am J Hematol"},{"key":"10.1016\/j.jaci.2025.05.010_bib162","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1111\/nep.13690","article-title":"Cocaine-associated atypical haemolytic uraemic syndrome in a genetically susceptible individual","volume":"25","author":"Bongetti","year":"2020","journal-title":"Nephrology"},{"key":"10.1016\/j.jaci.2025.05.010_bib101","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1182\/blood.V77.2.306.306","article-title":"Quinine-induced immune thrombocytopenia associated with hemolytic uremic syndrome: a new clinical entity","volume":"77","author":"Gottschall","year":"1991","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib102","doi-asserted-by":"crossref","first-page":"1129","DOI":"10.1056\/NEJMoa0707330","article-title":"VEGF inhibition and renal thrombotic microangiopathy","volume":"358","author":"Eremina","year":"2008","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jaci.2025.05.010_bib103","first-page":"908","article-title":"Systemic malignancies as a cause of unexpected microangiopathic hemolytic anemia and thrombocytopenia","volume":"25","author":"George","year":"2011","journal-title":"Oncology (Williston Park)"},{"key":"10.1016\/j.jaci.2025.05.010_bib104","doi-asserted-by":"crossref","first-page":"1420","DOI":"10.1016\/j.kint.2016.12.009","article-title":"Patients with hypertension-associated thrombotic microangiopathy may present with complement abnormalities","volume":"91","author":"Timmermans","year":"2017","journal-title":"Kidney Int"},{"key":"10.1016\/j.jaci.2025.05.010_bib105","doi-asserted-by":"crossref","first-page":"1237","DOI":"10.2215\/CJN.00280117","article-title":"Hemolytic uremic syndrome in pregnancy and postpartum","volume":"12","author":"Bruel","year":"2017","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib106","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1182\/asheducation-2015.1.644","article-title":"Syndromes of thrombotic microangiopathy associated with pregnancy","volume":"2015","author":"George","year":"2015","journal-title":"Hematology Am Soc Hematol Educ Program"},{"key":"10.1016\/j.jaci.2025.05.010_bib107","doi-asserted-by":"crossref","first-page":"2103","DOI":"10.1182\/blood.2020005221","article-title":"Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group","volume":"136","author":"Fakhouri","year":"2020","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib108","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pmed.1001013","article-title":"Mutations in complement regulatory proteins predispose to preeclampsia: a genetic analysis of the PROMISSE cohort","volume":"8","author":"Salmon","year":"2011","journal-title":"PLoS Med"},{"issue":"9741","key":"10.1016\/j.jaci.2025.05.010_bib109","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1016\/S0140-6736(10)60279-6","article-title":"Pre-eclampsia","volume":"376","author":"Steegers","year":"2010","journal-title":"Lancet"},{"key":"10.1016\/j.jaci.2025.05.010_bib110","doi-asserted-by":"crossref","first-page":"1084","DOI":"10.1681\/ASN.2015101189","article-title":"Thrombotic microangiopathy in inverted formin 2\u2013mediated renal disease","volume":"28","author":"Challis","year":"2017","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib111","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1186\/s12882-017-0643-1","article-title":"Hemolytic uremic syndrome as the presenting manifestation of WT1 mutation and Denys-Drash syndrome: a case report","volume":"18","author":"Alge","year":"2017","journal-title":"BMC Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib112","doi-asserted-by":"crossref","first-page":"2246","DOI":"10.1093\/ndt\/gft220","article-title":"Atypical haemolytic uraemic syndrome with underlying glomerulopathies. A case series and a review of the literature","volume":"28","author":"Manenti","year":"2013","journal-title":"Nephrol Dial Transplant"},{"key":"10.1016\/j.jaci.2025.05.010_bib113","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1053\/j.ajkd.2017.11.019","article-title":"Patterns of clinical response to eculizumab in patients with C3 glomerulopathy","volume":"72","author":"Le Quintrec","year":"2018","journal-title":"Am J Kidney Dis"},{"key":"10.1016\/j.jaci.2025.05.010_bib114","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2025.1537974","article-title":"Factor B as a therapeutic target for the treatment of complement-mediated diseases","volume":"16","author":"Kavanagh","year":"2025","journal-title":"Front Immunol"},{"key":"10.1016\/j.jaci.2025.05.010_bib115","doi-asserted-by":"crossref","first-page":"2754","DOI":"10.1016\/j.ekir.2023.09.017","article-title":"Efficacy and safety of iptacopan in patients with C3 glomerulopathy","volume":"8","author":"Wong","year":"2023","journal-title":"Kidney Int Rep"},{"key":"10.1016\/j.jaci.2025.05.010_bib117","doi-asserted-by":"crossref","first-page":"S136","DOI":"10.1016\/j.ekir.2024.11.300","article-title":"WCN25-2041. Efficacy and safety of iptacopan in patients with C3 glomerulopathy: 12-month results from the phase 3 APPEAR-C3G study","volume":"10","author":"Nester","year":"2025","journal-title":"Kidney Int Rep"},{"key":"10.1016\/j.jaci.2025.05.010_bib116","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.ekir.2024.09.030","article-title":"Efficacy and safety of pegcetacoplan in kidney transplant recipients with recurrent complement 3 glomerulopathy or primary immune complex membranoproliferative glomerulonephritis","volume":"10","author":"Bomback","year":"2025","journal-title":"Kidney Int Rep"},{"issue":"10 suppl","key":"10.1016\/j.jaci.2025.05.010_bib118","volume":"35","author":"Nester","year":"2024","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.jaci.2025.05.010_bib119","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1093\/ckj\/sfx081","article-title":"Complement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea","volume":"10","author":"Brocklebank","year":"2017","journal-title":"Clin Kidney J"},{"key":"10.1016\/j.jaci.2025.05.010_bib120","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1038\/bmt.2016.253","article-title":"Is complement blockade an acceptable therapeutic strategy for hematopoietic cell transplant\u2013associated thrombotic microangiopathy?","volume":"52","author":"Dhakal","year":"2017","journal-title":"Bone Marrow Transplant"},{"key":"10.1016\/j.jaci.2025.05.010_bib121","doi-asserted-by":"crossref","first-page":"2418","DOI":"10.1182\/bloodadvances.2019001315","article-title":"New insights into risk factors for transplant-associated thrombotic microangiopathy in pediatric HSCT","volume":"4","author":"Elfeky","year":"2020","journal-title":"Blood Adv"},{"key":"10.1016\/j.jaci.2025.05.010_bib122","doi-asserted-by":"crossref","first-page":"1112","DOI":"10.1182\/blood.2023022526","article-title":"A prospective multi-institutional study of eculizumab to treat high-risk stem cell transplantation-associated TMA","volume":"143","author":"Jodele","year":"2024","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2025.05.010_bib123","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1038\/nrneph.2016.124","article-title":"Scleroderma renal crisis and renal involvement in systemic sclerosis","volume":"12","author":"Woodworth","year":"2016","journal-title":"Nat Rev Nephrol"}],"container-title":["Journal of Allergy and Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674925005743?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674925005743?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T08:34:38Z","timestamp":1778142878000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0091674925005743"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,8]]},"references-count":162,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2025,8]]}},"alternative-id":["S0091674925005743"],"URL":"https:\/\/doi.org\/10.1016\/j.jaci.2025.05.010","relation":{},"ISSN":["0091-6749"],"issn-type":[{"value":"0091-6749","type":"print"}],"subject":[],"published":{"date-parts":[[2025,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Atypical hemolytic uremic syndrome","name":"articletitle","label":"Article Title"},{"value":"Journal of Allergy and Clinical Immunology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jaci.2025.05.010","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 The Authors. Published by Elsevier Inc. on behalf of the American Academy of Allergy, Asthma & Immunology.","name":"copyright","label":"Copyright"}]}}