Altered T-Cell Subsets and Defective T-Cell Function in Young Children with Down Syndrome (Trisomy-21)

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Abstract
Children with Down syndrome (DS) suffer an increased incidence of severe viral and bacterial infections particularly during the first 5 years of life. Unfortunately, few studies have been performed on the immune systems of young children with Down syndrome. Peripheral blood mononuclear cells (PBMC) from a group of non-institutionalized children less than 6 years of age were studied and compared with PBMC from age-matched controls. The children with DS had reduced numbers of circulating OKT4+ (helper/inducer) T cells and a significantly depressed ratio of OKT4+ to OKT8+ (suppressor/cytotoxic) T cells. PBMC from the DS subjects exhibited reduced proliferative responses to phytohemagglutinin and to an optimal concentration of concanavalin A (Con A), but normal responses to suboptimal doses of Con A and pokeweek mitogen. PBMC from young children with DS appeared to produce normal levels of interleukin-2 (IL-2). These findings provide evidence that the primary immune defect in DS is in part a depressed number and function of helper T cells. They also indicate that IL-2 production may not be defective in DS, but rather that the mechanism for response to IL-2 may be faulty.