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Abstract
We retrospectively analyzed the frequency and nature of infections occurring in 48
patients with chronic granulomatous disease. The long-term use of trimethoprim-sulfamethoxazole
and ketoconazole as a preventive therapy for infections has also been evaluated. Lymphadenitis,
lung infections, dermatitis, enteral infections, and hepatic abscesses were the most
frequent infections. Staphylococcus aureus, Salmonella, and Aspergillus were the main
microorganisms encountered. Twelve patients died: five from lung aspergillosis, three
from hepatic abscesses, two from pneumonopathy of unknown origin, one from salmonellosis,
and one from another probable infection that could not be proved. The actuarial survival
rate was 50% at 10 years of age, with a prolonged plateau thereafter. There was no
difference in survival rates between patients with X-linked and those with autosomal
recessive chronic granulomatous disease. The 8-year actuarial survival rate was significantly
higher for patients born in 1978 or afterward than for patients born before 1978 (92.9%
vs 70.5%). A retrospective analysis of the occurrence of bacterial and fungal infections
in patients who received trimethoprim-sulfamethoxazole and ketoconazole as infection
prophylaxis indicated that the former was effective against bacterial infections but
that ketoconazole provided no protection against Aspergillus infections.