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Patients with TMA who do not exhibit a severe ADAMTS\u201013 deficiency (defined as a disintegrin\u2010like and metalloprotease with thrombospondin type 1 motif no. 13 activity \u226510%: TMA\u201013n) continue to experience elevated mortality rates. This study explores the prognostic indicators for augmented mortality risk or necessitating chronic renal replacement therapy (composite outcome: CO) in TMA\u201013n patients. We included 42 TMA\u201013n patients from January 2008 to May 2018. Median age of 41\u2009years and 60% were female. At presentation, 62% required dialysis, and 57% warranted intensive care unit admission. CO was observed in 45% of patients, including a 9\u2010patient mortality subset. Multivariate logistic regression revealed three independent prognostic factors for CO: early administration of eculizumab (median time from hospitalization to eculizumab initiation: 5\u2009days, range 0\u201319\u2009days; odds ratio [OR], 0.14; 95% confidence interval [CI], 0.02\u20130.94), presence of neuroradiological lesions (OR, 6.67; 95% CI, 1.12\u201339.80), and a PLASMIC score\u2009\u22644 (OR, 7.39; 95% CI, 1.18\u201346.11). In conclusion, TMA\u201013n patients exhibit a heightened risk of CO in the presence of low PLASMIC scores and neuroradiological lesions, while early eculizumab therapy was the only protective factor.<\/jats:p>","DOI":"10.1111\/ejh.14261","type":"journal-article","created":{"date-parts":[[2024,7,3]],"date-time":"2024-07-03T02:44:03Z","timestamp":1719974643000},"page":"510-520","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Risk factors of death or chronic renal replacement therapy requirements in patients with thrombotic microangiopathies without <scp>ADAMTS\u201013<\/scp> deficiency"],"prefix":"10.1111","volume":"113","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1762-4226","authenticated-orcid":false,"given":"Miguel G.","family":"Uriol\u2010Rivera","sequence":"first","affiliation":[{"name":"Nephrology Department Hospital Universitario Son Espases  Palma de Mallorca Balearic Islands 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