CASE 10
Barry is 16 months of age and since his adoption at birth has been hospitalized on several occasions with multiple and severe gram-positive bacterial infections of the respiratory tract, skin, soft tissues, gastrointestinal tract, and even bones. On two occasions he has suffered from meningitis, and on at least one occasion he developed nearly overwhelming septicemia, with systemic spread of gram-negative bacteria. Laboratory analysis at the time of these various infections indicated that he had had infections with Mycobacterium avium, as well as with both gram-positive (Streptococcus pneumoniae, Staphylococcus aureus) and gram-negative (Haemophilus influenzae and Pseudomonas aeruginosa) organisms.
Interestingly, despite childhood immunization with H. influenzae, as well as infection with S. pneumoniae and H. influenzae, there was a dearth of specific antipolysaccharide antibodies, probably contributing to the susceptibility to encapsulated bacteria. IgM levels were elevated, with a slight diminution in IgG and IgA levels. Levels of T cells and B cells and macrophages/neutrophils were normal, as was the subset distribution of T cells and the surface expression of CD40L/CD154 (see Case 4). Analysis of phagocyte function (see Case 6) and proliferation of mitogen-stimulated T cells were at the low end of normal (see Case 2). NK cell levels were normal, with evidence for some decrease in killing function (˜threefold on a cell-for-cell basis compared with normal).
