{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T10:02:14Z","timestamp":1769162534629,"version":"3.49.0"},"reference-count":26,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,3,28]],"date-time":"2024-03-28T00:00:00Z","timestamp":1711584000000},"content-version":"vor","delay-in-days":87,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["K23HD104933"],"award-info":[{"award-number":["K23HD104933"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Case Reports in Pediatrics"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:p><jats:italic>Background<\/jats:italic>. Cytokine storm syndromes (CSS) are life\u2010threatening systemic inflammatory disorders caused by immune system dysregulation. They can lead to organ failure and are triggered by various factors, including infections, malignancy, inborn errors of immunity, and autoimmune conditions. Trisomy 21 (TS21), also known as Down syndrome, is a genetic disorder associated with immune dysfunction, increased infection susceptibility, and inflammation. While TS21 has been linked to infectious\u2010triggered hyperinflammation, its role as a primary cause of CSS has not been confirmed. <jats:italic>Case Presentation<\/jats:italic>. We present a case of a 16\u2010year\u2010old male with TS21 with fever, rash, joint pain, and abdominal symptoms. Extensive investigations ruled out infections, autoimmune conditions, malignancies, and inborn errors of immunity as triggers for a CSS. The patient\u2019s symptoms improved with treatment using IL\u20101 inhibition and corticosteroids. <jats:italic>Conclusions<\/jats:italic>. This case reinforces that TS21 is an immune dysregulation disorder and highlights the importance of considering CSS in TS21 patients, even when triggers are unclear. The positive response to IL\u20101 inhibition in this patient suggests that dysregulation of the IL\u20101 superfamily and the NLRP3 inflammasome may contribute to CSS in TS21. This finding raises the possibility of using IL\u20101 inhibition as a treatment approach for CSS in TS21 patients.<\/jats:p>","DOI":"10.1155\/2024\/9946401","type":"journal-article","created":{"date-parts":[[2024,3,29]],"date-time":"2024-03-29T00:20:06Z","timestamp":1711671606000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Cytokine Storm Syndrome Responsive to IL\u20101 Inhibition in Trisomy 21"],"prefix":"10.1155","volume":"2024","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7547-5906","authenticated-orcid":false,"given":"Aimee","family":"Magnarelli","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julia","family":"Shalen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria 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