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Rudolph's PediatricsRudolph's Pediatrics

Section 13. Immunologic Disorders > 

Chapter 188. Primary Immunodeficiencies

Talal A. Chatila
Topics Discussed: humoral immune defect; immunology; predominantly t-cell defect; primary immune deficiency disorder; t-lymphocyte deficiency.
Sections: Severe Combined Immunodeficiency, Primary T and Combined T and B Immunodeficiencies, Combined Immunodeficiencies Associated with Other Defects, Primary Antibody Deficiency Diseases, Disorders of Immune Regulation, References.
Excerpt:"Primary immunodeficiency diseases result from genetic defects affecting the development and/or the function of immune system components.1-5 They frequently manifest soon after birth, although some become evident later in life. Recurrent infections are the hallmark of immunodeficiency syndromes, with the types of infections reflecting the nature of the defect. Autoimmune disorders and certain malignancies occur more frequently in individuals with immunodeficiency diseases...."
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