Journal of Allergy and Clinical Immunology
Chapter 15Primary immunodeficiencies
Section snippets
CIDs
CIDs comprise a heterogeneous group of disorders with impaired development, function, or both of T lymphocytes associated with a defective antibody response.2 The latter might result from intrinsic defects in B lymphocytes or might reflect inadequate TH cell activity. In the most severe forms of CID (also known as severe combined immunodeficiency [SCID]), there is a virtual lack of functional peripheral T cells, whereas residual number, function, or both of T lymphocytes are present in other
Diagnostic approach to PIDs
The main forms of PID (CIDs, antibody deficiencies, and defects of innate immunity) are characterized by different susceptibilities to pathogens (Table I). Accordingly, medical history (with particular regard to type, location, age at onset, and severity of infections) might provide important insights into the possible underlying mechanisms of immunodeficiency. Additional aspects of past medical history might also help. Certainly a history of HIV infection is very important in the differential
Treatment
Infants with a suspicion of SCID or other CIDs require prompt intervention with use of cotrimoxazole to prevent Pneumocystis infection, prophylactic use of antifungal drugs, immunoglobulin replacement therapy, and aggressive treatment of any infectious episodes. Nutritional support is often necessary. Immune suppression helps in controlling the inflammatory reactions associated with Omenn syndrome. Only irradiated and filtered blood products should be used in patients with SCID because of the
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Disclosure of potential conflict of interest: L. D. Notarangelo has received research support from the Manton Foundation and the National Institutes of Health.