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[Chronic septic granulomatosis. Clinical and therapeutic aspects]
Abstract:
Chronic granulomatous disease of childhood is characterized clinically by the occurrence of severe and recurrent bacterial and fungal infections. The underlying biologic anomaly is defective microbicidal capacity of phagocytic cells with an abnormal oxidative response during phagocytosis. In most instances, inheritance is recessive and X-linked. Purulent adenitis and skin infections are the most common manifestations, but the overwhelming majority of deaths are caused by pulmonary aspergillosis, abscesses of the liver, and Salmonella infections. The most frequently recovered microorganisms include staphylococci, Aspergillus, Salmonellae, and Gram-negative rods. Bacterial infections seem to be considerably less frequent in patients under long-term prophylactic treatment with the trimethoprim-sulfamethoxazole combination. However, no oral antifungal agent is as yet available for the prevention of pulmonary aspergillosis, as ketoconazole has proved ineffective.
Insights
Chronic granulomatous disease (CGD) involves severe infections due to phagocyte dysfunction. Trimethoprim-sulfamethoxazole reduces bacterial infections, but effective antifungal prophylaxis for aspergillosis remains elusive.
Area of Science:
- Immunology
- Genetics
- Pediatrics
Context:
- Chronic granulomatous disease (CGD) is a primary immunodeficiency.
- Characterized by recurrent severe bacterial and fungal infections.
- Caused by defective phagocytic cell oxidative burst.
Purpose:
- To summarize the clinical and microbiological features of CGD.
- To discuss current treatment strategies and their limitations.
Summary:
- CGD presents with recurrent infections, primarily from Staphylococcus, Aspergillus, Salmonella, and Gram-negative rods.
- X-linked recessive inheritance is most common.
- Pulmonary aspergillosis, liver abscesses, and Salmonella infections are leading causes of mortality.
- Long-term trimethoprim-sulfamethoxazole prophylaxis reduces bacterial infections.
- No effective oral antifungal prophylaxis for invasive aspergillosis is currently available.
Impact:
- Highlights the need for improved antifungal strategies in CGD.
- Informs clinical management of patients with CGD.
- Emphasizes the importance of early diagnosis and prophylactic measures.