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Immune system defects in DiGeorge syndrome and association with clinical course

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dc.contributor.authorÖZEN, AHMET OĞUZHAN
dc.contributor.authorsNain, Ercan; Kiykim, Ayca; Ogulur, Ismail; Kasap, Nurhan; Karakoc-Aydiner, Elif; Ozen, Ahmet; Baris, Safa
dc.date.accessioned2022-03-14T09:11:10Z
dc.date.available2022-03-14T09:11:10Z
dc.date.issued2019-11
dc.description.abstractWe evaluated 18 DiGeorge syndrome (DGS) patients and aimed to investigate the immunological changes in this population. DGS patients with low naive CD4(+)T and CD8(+)T cells were defined as high-risk (HR) patients, whereas patients with normal numbers of naive CD4(+) and CD8(+)T cells were defined as standard risk (SR) patients. Level of serum IgM, CD3(+) T cell counts and percentages of class-switched 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-switched B cells had earlier onset of infection, lower blood IgM, lower CD4(+) and CD8(+)T counts than patients with normal class-switched memory B cells. Decreased levels of IgM were associated with low numbers of naive CD4(+) and recent thymic emigrants T cells. Monitoring the immune changes of patients with DGS would be useful to predict the severe phenotype of disease.
dc.identifier.doi10.1111/sji.12809
dc.identifier.eissn1365-3083
dc.identifier.issn0300-9475
dc.identifier.pubmed31322747
dc.identifier.urihttps://hdl.handle.net/11424/242727
dc.identifier.wosWOS:000486091700001
dc.language.isoeng
dc.publisherWILEY
dc.relation.ispartofSCANDINAVIAN JOURNAL OF IMMUNOLOGY
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectantibodies
dc.subjectimmunoglobulins
dc.subjectB cells
dc.subjectDiGeorge syndrome
dc.subjectimmunodeficiency
dc.subjectT cells
dc.subject22Q11.2 DELETION SYNDROME
dc.subjectREFERENCE VALUES
dc.subjectCHILDREN
dc.subjectAUTOIMMUNITY
dc.subjectPREVALENCE
dc.subjectDEFICIENCY
dc.subjectPHENOTYPE
dc.subjectFEATURES
dc.titleImmune system defects in DiGeorge syndrome and association with clinical course
dc.typearticle
dspace.entity.typePublication
local.avesis.ide4c93650-3c87-4354-8bd7-caa4b3fce907
local.import.packageSS16
local.indexed.atWOS
local.indexed.atSCOPUS
local.indexed.atPUBMED
local.journal.articlenumbere12809
local.journal.numberofpages8
local.journal.quartileQ3
oaire.citation.issue5
oaire.citation.titleSCANDINAVIAN JOURNAL OF IMMUNOLOGY
oaire.citation.volume90
relation.isAuthorOfPublication3e9c297b-e636-4836-8f61-dc9c8b7c29cf
relation.isAuthorOfPublication.latestForDiscovery3e9c297b-e636-4836-8f61-dc9c8b7c29cf

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