Abstract
Immune system homeostasis is tightly maintained by regulatory mechanisms that allow for the destruction of pathologic microorganisms while preventing pathologic inflammation. Disruption of these regulatory mechanisms can lead to immunodeficiency and uncontrolled inflammation as seen in hyper-IgE syndrome and in immunodysregulation, polyendocrinopathy, enteropathy, X-linked syndrome. The discovery of monogenic defects in these disorders has led to a greater understanding of these complex disorders and of the mechanisms underlying immune system regulation.
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© 2009 Humana Press, a part of Springer Science+Business Media, LLC
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Marks, A.L., Tcheurekdjian, H. (2009). Disorders of Immune Regulation. In: Mahmoudi, M. (eds) Challenging Cases in Allergy and Immunology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60327-443-2_17
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DOI: https://doi.org/10.1007/978-1-60327-443-2_17
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