{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T15:23:15Z","timestamp":1786980195251,"version":"build-2736575974"},"reference-count":21,"publisher":"Springer Science and Business Media LLC","issue":"6","license":[{"start":{"date-parts":[[2025,5,8]],"date-time":"2025-05-08T00:00:00Z","timestamp":1746662400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,5,8]],"date-time":"2025-05-08T00:00:00Z","timestamp":1746662400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100003500","name":"Universit\u00e0 degli Studi di Padova","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100003500","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Eur J Pediatr"],"abstract":"Abstract<\/jats:title>\n \n Hemolytic-uremic syndrome (HUS) is defined by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI) and is caused, in 90% of pediatric cases, by Shiga toxin-producing\n Escherichia coli<\/jats:italic>\n (STEC-HUS) infection. While targeting complement component C5 using eculizumab has shown benefit in atypical HUS, its effect on STEC-HUS, especially on neurological outcome, remains unclear. This systematic review and meta-analysis aimed to evaluate the impact of eculizumab on neurological prognosis in pediatric STEC-HUS. The review was conducted in accordance with PRISMA guidelines and was registered in PROSPERO (CRD42024496489). A comprehensive literature search was performed in Embase, MEDLINE, Cochrane Library, CINAHL, clinicaltrial.gov, and grey literature sources up to February 28, 2025. Original studies involving pediatric patients (0\u201318\u00a0years) with STEC-HUS and neurological complications, treated with eculizumab, were eligible. Two independent reviewers screened studies and extracted data. Seven studies were included, totaling 529 patients, of whom 135 (25.5%) developed neurological complications. Among these, 44 patients (32.5%) had received eculizumab. Meta-analysis showed a higher likelihood of receiving eculizumab therapy in patients with neurological involvement compared to those without (OR 13.03, 95% CI 4.40\u201338.75). However, in patients with neurological involvement, no clinical benefit was observed compared to those treated with standard therapies (OR 0.32, 95% CI 0.09\u20131.22,\n p<\/jats:italic>\n \u2009=\u20090.10).\n Conclusion<\/jats:italic>\n : Our data did not demonstrate a significant improvement in neurological outcomes for STEC-HUS patients treated with eculizumab. Findings are limited by retrospective designs and potential confounding by indication; therefore, further studies are needed.\n \n \n \n \n \n What is Known<\/jats:bold>\n :\n <\/jats:td>\n <\/jats:tr>\n \n \n \u2022\n Neurological involvement is a major contributor to morbidity in HUS, particularly in STEC-associated forms<\/jats:italic>\n .\n <\/jats:td>\n <\/jats:tr>\n \n \n \u2022\n Eculizumab is sometimes used off-label in severe cases, although its effectiveness in this setting remains uncertain<\/jats:italic>\n .\n <\/jats:td>\n <\/jats:tr>\n \n \n What is New<\/jats:bold>\n :\n <\/jats:td>\n <\/jats:tr>\n \n \n \u2022\n This is the first systematic review and meta-analysis specifically addressing neurological prognosis in STEC-HUS<\/jats:italic>\n .\n <\/jats:td>\n <\/jats:tr>\n \n \n \u2022\n Current evidence does not demonstrate a clear neurological benefit of eculizumab over standard therapy, highlighting the need for further studies<\/jats:italic>\n .\n <\/jats:td>\n <\/jats:tr>\n <\/jats:tbody>\n <\/jats:table>\n <\/jats:table-wrap>\n <\/jats:p>","DOI":"10.1007\/s00431-025-06160-2","type":"journal-article","created":{"date-parts":[[2025,5,8]],"date-time":"2025-05-08T11:02:44Z","timestamp":1746702164000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Eculizumab in severe pediatric STEC-HUS and its impact on neurological prognosis\u2014a systematic review and meta-analysis"],"prefix":"10.1007","volume":"184","author":[{"given":"Rachele","family":"Spagnol","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Alfisi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Moi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilaria","family":"Bonvecchio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicola","family":"Bertazza Partigiani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enrico","family":"Vidal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,5,8]]},"reference":[{"issue":"3","key":"6160_CR1","doi-asserted-by":"publisher","first-page":"285","DOI":"10.1136\/archdischild-2016-311377","volume":"103","author":"PR Walsh","year":"2018","unstructured":"Walsh PR, Johnson S (2018) Treatment and management of children with haemolytic uraemic syndrome. 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