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Introduction
The central purpose of the immune system is to recognize danger and protect us from infections and cancers. The immune system is an integral part of many other organ systems, including the skin, gastrointestinal tract, and respiratory tract, with roles in wound healing, mucosal immunity, and protection against inhaled pathogens. A complex network of cells, cytokines, antibodies, and proteins are intricately orchestrated in the development of an immune response against infection and in the resolution of the immune response. Due to the complexity of the immune system, there are often “backup” defense networks in place, such that minor defects of the immune system may not cause a great susceptibility to infections. However, defects in critical genes cause significantly increased susceptibility to recurrent, severe, or unusual infections, often accompanied by immune dysregulation or autoimmunity. The recognition of primary immunodeficiency...
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References
Bonilla FA, Khan DA, Ballas ZK, Chinen J, Frank MM, Hsu JT, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015;136(5):1186–205.e1–78. https://doi.org/10.1016/j.jaci.2015.04.049
Bousfiha A, Jeddane L, Al-Herz W, Ailal F, Casanova JL, Chatila T, et al. The 2015 IUIS phenotypic classification for primary Immunodeficiencies. J Clin Immunol. 2015;35:727–38.
Kuruvilla M, de la Morena MT. Antibiotic prophylaxis in primary immune deficiency disorders. J Allergy Clin Immunol Pract. 2013;1(6):573–82.
Ochs HD, Hitzig WH. History of primary immunodeficiency diseases. Curr Opin Allergy Clin Immunol. 2012;12(6):577–87.
Picard C, Al-Herz W, Bousfiha A, Casanova L, Chatila C, Conley ME, et al. Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for primary immunodeficiency 2015. J Clin Immunol. 2015;35(8):696–726.
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Perez, E.E. (2020). Clinical Presentation of Immunodeficiency, Primary Immunodeficiency. In: Orange, J.S., Chinen, J. (eds) Encyclopedia of Medical Immunology. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8678-7_69
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DOI: https://doi.org/10.1007/978-1-4614-8678-7_69
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