Immunology of Down syndrome: a review.

作者: Alberto G. Ugazio , Rita Maccario , Luigi D. Notarangelo , G. Roberto Burgio

DOI: 10.1002/AJMG.1320370742

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摘要: Subjects with Down syndrome (DS) have a high mortality due to infections and risk of developing malignancies. These observations, together the demonstration frequent occurrence HBsAg carrier state autoantibodies, prompted investigations immune function in DS. Thymic morphological functional abnormalities been demonstrated. Peripheral blood mononuclear cells DS subjects shown include number T lymphocytes low avidity for sheep erythrocytes very percentage an NK phenotype. However, activity is Production some important cytokines, such as IL-2, depressed, thus contributing T-cell derangement. Abnormalities B-cell compartment were also demonstrated, tendency towards IgG IgM serum levels. Controversial results obtained regard antigen-specific antibody response. Also phagocytes display characteristic impairments, chemotactic ability reduced production oxygen radicals. Despite clearly established rather detailed evidence derangements, therapeutic trials anecdotal resulted marginal effects. HBV vaccination highly advisable because becoming chronic carriers once infected.

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