{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T18:42:17Z","timestamp":1793472137419,"version":"4.5.0"},"reference-count":551,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,1,16]],"date-time":"2024-01-16T00:00:00Z","timestamp":1705363200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Immunological Reviews"],"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>The thymus is the primary site of T\u2010cell development, enabling generation, and selection of a diverse repertoire of T cells that recognize non\u2010self, whilst remaining tolerant to self\u2010 antigens. Severe congenital disorders of thymic development (athymia) can be fatal if left untreated due to infections, and thymic tissue implantation is the only cure. While newborn screening for severe combined immune deficiency has allowed improved detection at birth of congenital athymia, thymic disorders acquired later in life are still underrecognized and assessing the quality of thymic function in such conditions remains a challenge. The thymus is sensitive to injury elicited from a variety of endogenous and exogenous factors, and its self\u2010renewal capacity decreases with age. Secondary and age\u2010related forms of thymic dysfunction may lead to an increased risk of infections, malignancy, and autoimmunity. Promising results have been obtained in preclinical models and clinical trials upon administration of soluble factors promoting thymic regeneration, but to date no therapy is approved for clinical use. In this review we provide a background on thymus development, function, and age\u2010related involution. We discuss disease mechanisms, diagnostic, and therapeutic approaches for primary and secondary thymic defects.<\/jats:p>","DOI":"10.1111\/imr.13306","type":"journal-article","created":{"date-parts":[[2024,1,16]],"date-time":"2024-01-16T21:38:10Z","timestamp":1705441090000},"page":"178-211","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Primary and secondary defects of the thymus"],"prefix":"10.1111","volume":"322","author":[{"given":"Sarah S.","family":"Dinges","sequence":"first","affiliation":[{"name":"Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases National Institutes of Health  Bethesda Maryland USA"},{"name":"Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu Berlin  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kayla","family":"Amini","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8335-0262","authenticated-orcid":false,"given":"Luigi D.","family":"Notarangelo","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4772-0799","authenticated-orcid":false,"given":"Ottavia M.","family":"Delmonte","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and 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