Abstract
Children with Down syndrome (DS) have a high incidence of recurrent respiratory tract infections, leukaemia and autoimmune disorders, suggesting immune dysfunction. The present study evaluated the role of the CD19 complex and memory B cells in the pathogenesis of immunodeficiency in children with DS. The expression levels (median fluorescein intensity—MFI) of CD19, CD21 and CD81 molecules on the surface of B cells and memory B cell subsets were studied in 37 patients and 39 healthy controls. Twenty-nine of the DS group had congenital cardiac disease. The B cell count was significantly low in children with DS compared with healthy age-matched controls for all three age groups (under 2 years; 2–6 years and older than 6 years). The MFI of CD19 was reduced in all the age groups, whereas that of CD21 was increased in those older than 2 years with DS. The expression level of CD81 was significantly increased in those older than 6 years. Age-related changes were also detected in memory B cell subsets. The frequency of CD27+IgD+IgM+ natural effector B cells was reduced in children with DS who had needed hospitalisation admission due to infections. The observed intrinsic defects in B cells may be responsible for the increased susceptibility of children with DS to severe respiratory tract infections.
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Acknowledgements
We thank the children and their families for participating. In particular, we thank Fatih Kara, M.D., for the statistical analysis.
Funding
The study was supported by the Scientific Research Projects of Selcuk University (Project No: 14102013).
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Seckin, A.N., Ozdemir, H., Ceylan, A. et al. Age-related alterations of the CD19 complex and memory B cells in children with Down syndrome. Clin Exp Med 18, 125–131 (2018). https://doi.org/10.1007/s10238-017-0457-2
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DOI: https://doi.org/10.1007/s10238-017-0457-2