Altered immune response in organic acidemia

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dc.authoridZeybek, Ayse Cigdem Aktuglu/0000-0001-7256-0750
dc.authoridKIYKIM, Ertugrul/0000-0001-5170-5594
dc.authoridcokugras, Haluk Cezmi/0000-0002-0086-3936
dc.authoridKiykim, Ayca/0000-0001-5821-3963
dc.authoridHOPURCUOGLU, Duhan/0000-0001-8599-8284
dc.authoridTopcu, Birol/0000-0003-0771-2505
dc.authoridZubarioglu, Tanyel/0000-0002-7159-4008
dc.authorscopusid57221539199
dc.authorscopusid55537270400
dc.authorscopusid56451085500
dc.authorscopusid57756396600
dc.authorscopusid57204543961
dc.authorscopusid37058172000
dc.authorscopusid8283704400
dc.authorwosidZeybek, Ayse Cigdem Aktuglu/C-9812-2019
dc.authorwosidKIYKIM, Ertugrul/ABA-9964-2020
dc.authorwosidKiykim, Ayca/J-6400-2017
dc.contributor.authorAltun, İlayda
dc.contributor.authorKıykım, Ayça
dc.contributor.authorZubarioğlu, Tanyel
dc.contributor.authorBürtecene, Nihan
dc.contributor.authorHopurcuoglu, Duhan
dc.contributor.authorTopcu, Birol
dc.contributor.authorZeybek, Ayşe Çiğdem Aktuğlu
dc.date.accessioned2023-04-20T08:01:12Z
dc.date.available2023-04-20T08:01:12Z
dc.date.issued2022
dc.departmentFakülteler, Tıp Fakültesi, Temel Tıp Bilimleri Bölümü, Biyoistatistik Ana Bilim Dalı
dc.description.abstractBackground Most patients with organic acidemia suffer from recurrent infections. Although neutropenia has been reported in multiple studies, other components of the immune system have not been evaluated thoroughly. This study was conducted to assess the immune status of patients with organic acidemia (OA). Methods Thirty-three patients with OA who were followed up in Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Nutrition and Metabolism Department and a total of 32 age- and sex-matched healthy controls were enrolled to the study. The demographic and clinical data were recorded retrospectively from patient files. Complete blood counts, immunoglobulins, and lymphocyte immunophenotyping were recorded prospectively in a symptom- (infection-) free period. Results Of the 33 patients enrolled to the study, 21 (88%) were diagnosed with methylmalonic acidemia, 10 (33%) with propionic acidemia, and two (6.6%) with isovaleric acidemia. The mean age of the patients with OA and healthy subjects were 5.89 +/- 4.11 years and 5.34 +/- 4.36, respectively (P = 0.602). Twenty-nine (88%) of the patients had experienced frequent hospital admission, 13 (39%) were admitted to pediatric intensive care unit, and 18 (55%) suffered from sepsis. Naive helper T cells and recent thymic emigrants were significantly lower in OAs (P < 0.001). Various defects in humoral immunity have also been documented including memory B cells and immunoglobulins. Conclusions Patients with OAs may show adaptive immune defects rendering them susceptible to infections. Metabolic reprogramming based on nutritional modifications may be a promising therapeutic option in the future.
dc.description.sponsorshipResearch Fund of Istanbul University-Cerrahpasa [TSA-2019-33755]; Turkish Pediatric Association [14.02.2019/18]
dc.description.sponsorshipThis work was supported by the Research Fund of Istanbul University-Cerrahpasa (Project number: TSA-2019-33755) and Turkish Pediatric Association (Project number: 14.02.2019/18).
dc.identifier.doi10.1111/ped.15082
dc.identifier.issn1328-8067
dc.identifier.issn1442-200X
dc.identifier.issue1en_US
dc.identifier.pmid34861062
dc.identifier.scopus2-s2.0-85132451704
dc.identifier.scopusqualityQ3
dc.identifier.urihttps://doi.org/10.1111/ped.15082
dc.identifier.urihttps://hdl.handle.net/20.500.11776/10791
dc.identifier.volume64
dc.identifier.wosWOS:000811136200001
dc.identifier.wosqualityQ4
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.institutionauthorTopcu, Birol
dc.language.isoen
dc.publisherWiley
dc.relation.ispartofPediatrics International
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccess
dc.subjectImmune Deficiency
dc.subjectLymphocyte Metabolism
dc.subjectLymphocyte Subset
dc.subjectOrganic Acidemia
dc.subjectRecent Thymic Emigrant
dc.subjectMethylmalonic Aciduria
dc.subjectIn-Vitro
dc.subjectPatient
dc.subjectInhibition
dc.titleAltered immune response in organic acidemia
dc.typeArticle

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