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Median age at clinical diagnosis was 1.7\u00a0years (0\u00a0days-22\u00a0years) with follow-up for 21.7\u00a0months (0\u00a0days-17.3\u00a0years). Common evaluation reasons were cardiac features (30.6%), failure to thrive (15.3%), and neurological features (15.3%). Craniofacial dysmorphism (64\/66,\u00a097%), central nervous system findings (62\/72,\u00a086.1%), and congenital cardiovascular defect (43\/70,\u00a061.4%) were common. We noted lymphopenia (30\/72,\u00a041.7%) and low IgM (25\/69,\u00a036.2%). T helper cell counts were low in 49.3% (33\/67). T cytotoxic and NK cell counts were normal\/high in 80.6% (54\/67) and 97% (65\/67) of patients, respectively. 42.3% (11\/26) had low CD4\u2009+\u2009TEMRA, and 34.6% (9\/26) had low CD8\u2009+\u2009TEM percentages. None had low CD8\u2009+\u2009TEMRA. B cells were normal\/high (52\/67,\u00a077.6%). 30.8%(8\/26) had low switched-memory and 38.5% (10\/26) had low active B cell percentages. Low IgA levels were associated with decreased lymphocyte activation and recent thymic emigrant (RTE) cell percentages. Six(8.3%) patients with lymphopenia, three of whom had congenital athymia, died.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>CD4 lymphopenia was more common than CD8 lymphopenia. Normal\/high CD8\u2009+\u2009and NK cell counts were remarkable. Increased CD8+\u2009TEMRA cells seem to indicate peripheral homeostatic proliferation following viral infections. Low serum IgA correlated with low RTE% and impaired T-cell function. DGS severity markers include hypocalcemia, congenital cardiac anomaly, and T-cell lymphopenia.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Graphical Abstract<\/jats:title>\n                  <\/jats:sec>","DOI":"10.1007\/s10875-025-01884-0","type":"journal-article","created":{"date-parts":[[2025,6,3]],"date-time":"2025-06-03T15:58:05Z","timestamp":1748966285000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Clinical and Immunological Features of a Large DiGeorge Syndrome Cohort"],"prefix":"10.1007","volume":"45","author":[{"given":"Merve","family":"S\u00fcleyman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2213-4627","authenticated-orcid":false,"given":"Deniz","family":"Cagdas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pelin \u00d6zlem \u015eim\u015fek","family":"Kiper","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00fclen Eda","family":"\u00dctine","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Merve Ka\u015f\u0131kc\u0131","family":"\u00c7avdar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Feyzi \u0130lhan","family":"Tezcan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,6,3]]},"reference":[{"key":"1884_CR1","doi-asserted-by":"publisher","first-page":"247","DOI":"10.1007\/s10875-022-01418-y","volume":"43","author":"PJ Mustillo","year":"2023","unstructured":"Mustillo PJ, Sullivan KE, Chinn IK, Notarangelo LD, Haddad E, Davies EG, de la Morena MT, Hartog N, Yu JE, Hernandez-Trujillo VP, et al. 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