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Infectious pathogens in pediatric patients with primary immunodeficiencies.

BACKGROUND AND PURPOSE
Primary immunodeficiency diseases (PIDs) are rare disorders. Unusual infections often guide the initial investigation for immunodeficiency.


METHODS
In order to ascertain the organisms that lead to a predisposition for PIDs, we reviewed the charts of 92 children diagnosed with PIDs at the National Taiwan University Hospital between March 1984 and March 2004.


RESULTS
Pneumonia was diagnosed in 92%, 81%, and 76.5% of patients with antibody, combined, and cellular deficiencies, respectively. Other major illnesses were similar in the 3 groups and included bronchiolitis, acute gastroenteritis, otitis media, and bacteremia. Skin abscess, pneumonia, and lymphadenitis (54.5%, 45%, and 27% of cases, respectively) were the most common infections in patients with phagocyte defects. Organisms were speciated in only 44.8% of infection episodes. Most viral infections were diagnosed by traditional and time-consuming viral culture. Prophylactic antibiotics were prescribed to 9 out of the 92 patients with PIDs.


CONCLUSIONS
Early recognition of PIDs requires that practitioners be aware of the infection characteristics, and subsequent reliable and rapid molecular diagnosis are needed in such immunocompromised patients.