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DGS patients with low naive CD4<jats:sup>+<\/jats:sup>T and CD8<jats:sup>+<\/jats:sup>T cells were defined as high\u2010risk (HR) patients, whereas patients with normal numbers of naive CD4<jats:sup>+<\/jats:sup> and CD8<jats:sup>+<\/jats:sup>T cells were defined as standard risk (SR) patients. Level of serum IgM, CD3<jats:sup>+<\/jats:sup> T cell counts and percentages of class\u2010switched memory B cells were significantly low in HR group compared to SR ones. Severe infections and persistent hypoparathyroidism were detected significantly higher in HR group. Patients with reduced percentages of class\u2010switched B cells had earlier onset of infection, lower blood IgM, lower CD4<jats:sup>+<\/jats:sup> and CD8<jats:sup>+<\/jats:sup>T counts than patients with normal class\u2010switched memory B cells. Decreased levels of IgM were associated with low numbers of naive CD4<jats:sup>+<\/jats:sup> and recent thymic emigrants T cells. Monitoring the immune changes of patients with DGS would be useful to predict the severe phenotype of disease.<\/jats:p>","DOI":"10.1111\/sji.12809","type":"journal-article","created":{"date-parts":[[2019,7,19]],"date-time":"2019-07-19T14:33:12Z","timestamp":1563546792000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Immune system defects in DiGeorge syndrome and association with clinical course"],"prefix":"10.1111","volume":"90","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2560-9210","authenticated-orcid":false,"given":"Ercan","family":"Nain","sequence":"first","affiliation":[{"name":"Faculty of Medicine, Pediatric Allergy and Immunology Marmara University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5821-3963","authenticated-orcid":false,"given":"Ayca","family":"Kiykim","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, 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