Intrinsic defect of the immune system in children with Down syndrome: a review

Abstract

SummaryDown syndrome (DS) is the most frequent cause of mental retardation in man. Immunological changes in DS have been observed since the 1970s. The neurological system appears to be ageing precociously, with early occurrence of Alzheimer disease; until now, the observed immunological differences have been interpreted in the same context. Looking back at past and present results of immunological studies in DS children in relation to the clinical consequences they suffer, we conclude that it is more likely that the DS immune system is intrinsically deficient from the very beginning.

Down Syndrome with End-Stage Renal Disease

“…and suppressor T cells) and NK cells that are thought to be related to the increased susceptibility to infection, autoimmune disorders, and malignancies [10]. It may predispose DS patients to post-infectious glomerulonephritis.…”

Decreased AIRE Expression and Global Thymic Hypofunction in Down Syndrome

“…DS is associated with several complex clinical features including immunological abnormalities (4)(5)(6)(7)(8), and it has long been noticed that patients present abnormal thymuses, characterized by lymphocyte depletion, cortical atrophy, and loss of corticomedullary demarcation (9,10). In addition to higher susceptibility to infections-as evidenced during the H1N1 2009 pandemic where the likelihood of death was 300 times greater for DS patients (11)-DS is characterized by increased frequency of organ-specific autoimmune disorders and of lymphoid and myeloid leukemias, contrasting with a decreased risk for allergic diseases, particularly asthma (12,13).…”

Research Article Polymorphisms of interleukin 6 in Down syndrome individuals: a case-control study.

“…Alteration in the immune system, such as functional and morphological thymus abnormalities (Bloemers et al, 2011;Karl et al, 2012), lymphocytopenia, and alteration in differentiation, maturation, and activation of the T lymphocyte (Guazzarotti et al, 2009;Lorenzo et al, 2013) is frequently observed in DS individuals (Kusters et al, 2009). It can be responsible for increased incidence of infections, mainly in the respiratory tract (Bloemers et al, 2010;Broers et al, 2012), and occurrence of autoimmune disease in DS (Gillespie et al, 2006;Pellegrini et al, 2012).…”

“…DS also present functional and morphological alterations of the thymus that is smaller (Kusters et al, 2009;Lorenzo et al, 2013), possibly as a result of the increase of cytokines as gamma-interferon (gamma-IFN), and tumor necrosis factor (alpha-TNF) (Murphy et al, 1992).…”

“…Individuals with DS present an impairment of adaptive immune system, with reduced level of lymphocytes (Kusters et al, 2009) and an increase in the susceptibility to infections and autoimmune diseases (Gillespie et al, 2006).…”