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Pediatric Practice: Infectious DiseasePediatric Practice: Infectious Disease

Section 16. Congenital Immunodeficiency Syndromes > 

Chapter 59. Evaluation of the Child with Suspected Immune Deficiency

Timothy R. La Pine, Harry R. Hill
Topics Discussed: immunologic deficiency syndromes; immunology; primary immune deficiency disorder.
Sections: Pathogenesis, Clinical Presentation, Differential Diagnosis, Diagnosis, Special Considerations: Functional Categories of Immunodeficiency, References.
Excerpt:"Primary immune deficiencies represent a class of disorders in which there is an intrinsic defect in the immune system. In contrast, acquired or secondary immune deficiency states occur as a consequence of external agents such as infection or chemotherapy. Over 150 distinct primary immune deficiency syndromes have been recognized. The true prevalence of primary immune deficiencies is not known as routine screening for these defects is not performed at birth or at any time during life. One well-conducted telephone survey of 10,000 households found that the population rate for the diagnosis of primary immune deficiency was 0.0863%, which translates into approximately 250,000 persons (95% confidence interval: 151,769 to 361,408) with primary immune deficiency in the United States.46..."
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