The manifestations of immune deficiencies that affect multiple cell types range from profound to mild; these conditions can present with severe infection, recurrent infections, unusual infections, or autoimmunity. The most profound disorder is severe combined immunodeficiency. Other combined immunodeficiencies include…
Defects in cellular immunity, historically referred to T-cell defects, comprise a large number of distinct immune deficiencies. The manifestations usually include prolonged viral infections, opportunistic fungal or mycobacterial infections, and a predisposition to autoimmunity. To facilitate conceptualization of this large…
Of the primary immunodeficiency diseases, those affecting antibody production are the most prevalent. Selective absence of IgA is the most common defect, with rates ranging from 1 in 333 to 1 in 18,000 persons among different races and ethnicities. Patients…
Defense against infectious agents is secured through a combination of anatomic physical barriers, including the skin, mucous membranes, mucous blanket, and ciliated epithelial cells, and the components of the immune system. The immune system of vertebrates integrates 2 fundamental response…
Primary care physicians must have a high index of suspicion to diagnose immune system defects early enough to institute appropriate treatment before irreversible damage develops. Diagnosis can be difficult because most affected patients do not have abnormal physical features. The…
Acknowledgement Substantial material from Dr. Michael Frank’s chapter in Principles and Practice of Pediatric Infectious Diseases, fifth edition, has been used and he is therefore granted appropriate authorship. Dr. Frank was a giant in the field of complement and a…