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. ? 2006 Journal of Microbiology, Immunology and Infection.