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Title: Hypogammaglobulinemia in children: a warning sign to look deeply?
Authors: de Melo, Karina Mescouto [UNIFESP]
de Moraes-Pinto, Maria Isabel [UNIFESP]
Andrade, Luis E. C. [UNIFESP]
Salomao, Reinaldo [UNIFESP]
Brunialti, Milena K. C. [UNIFESP]
Ferreira, Vanessa S. [UNIFESP]
Costa-Carvalho, Beatriz T. [UNIFESP]
Keywords: Hypogammaglobulinemia
primary immunodeficiency
Issue Date: 2017
Publisher: Wiley
Citation: Apmis. Hoboken, v. 125, n. 10, p. 902-909, 2017.
Abstract: This study investigated phenotypic and functional characteristics of lymphocytes in children with common variable immunodeficiency (CVID) and unclassified hypogammaglobulinemia (UH), as well as B-cell subsets in non-consanguineous parents. Blood samples of 30 children, CVID (n = 9), UH (n = 9), healthy donors HD (n = 12), and 19 adults (parents and controls) were labeled by a combination of surface markers to identify CD4, CD8 T-cell and B-cell sub-populations. T-cell cytokine production in children was analyzed in vitro after stimulation with phytohemagglutinin (PHA) and tetanus toxoid. We observed low percentages of switched memory B cells in children with CVID, increase in total CD4(+) T-cell counts, and high percentages of transitional B cells only in UH group. Analysis of T-cell immunity showed that CVID children had decreased percentages of CD8(+) IFN-gamma-producing cells after stimulation with PHA and tetanus toxoid. Parent of children with CVID had low percentages of naive B cell and increased percentages of memory B cells in comparison with controls. These results suggest that (i) early combined immune defect in children with CVID and (ii) a possible familial B-cell disturbance in pediatric CVID.
ISSN: 0903-4641
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