Antibacterial prophylaxis in chronic granulomatous disease. A case report

The value of long-term prophylactic treatment of chronic granulomatous disease (CGD) in childhood cannot be established with certainty, as controlled studies are not available. We describe a boy, presently 15 years old, who suffered from CGD since early infancy. By the clinical, laboratory and genetic features, this case appeared to be a new variant of CGD, combining elements of the "childhood" type with others that characterize the "adult" type of the syndrome. For years, the patient had been almost continuously ill and needed frequent and prolonged hospitalizations because of severe bacterial infections. At age 13 years, long-term prophylactic treatment with trimethoprim-sulfamethoxazole (TMP-SMX) was instituted. With this regimen, the patient was maintained practically infection-free, relapsing only in those instances when he neglected to comply with the prophylactic regimen for 1 to 2 weeks. Thus, the patient served as his own control in demonstrating the efficacy of antimicrobial prophylaxis in CGD. The rationale for employing TMP-SMX for the prophylactic regimen is discussed.