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Incidence, severity, and prevention of infections in chronic granulomatous disease
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.
Insights
Chronic granulomatous disease (CGD) patients frequently experience infections like lymphadenitis and lung infections. Trimethoprim-sulfamethoxazole effectively prevents bacterial infections in CGD, but ketoconazole does not prevent fungal infections.
Area of Science:
- Immunology
- Infectious Diseases
- Pediatrics
Background:
- Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by impaired phagocyte oxidative burst, leading to recurrent severe infections.
- Understanding infection patterns and treatment efficacy is crucial for managing CGD patients.
Purpose of the Study:
- To retrospectively analyze infection frequency and types in CGD patients.
- To evaluate the long-term efficacy of trimethoprim-sulfamethoxazole and ketoconazole prophylaxis.
- To assess survival rates and factors influencing them in CGD.
Main Methods:
- Retrospective analysis of 48 CGD patients' medical records.
- Evaluation of infection types, causative microorganisms, and mortality.
- Assessment of prophylactic trimethoprim-sulfamethoxazole and ketoconazole use.
- Actuarial survival rate calculation based on birth year.
Main Results:
- Common infections included lymphadenitis, lung infections, dermatitis, enteral infections, and hepatic abscesses.
- Staphylococcus aureus, Salmonella, and Aspergillus were the predominant pathogens.
- Trimethoprim-sulfamethoxazole showed efficacy against bacterial infections; ketoconazole did not prevent Aspergillus infections.
- Actuarial survival at 10 years was 50%; survival improved significantly for patients born after 1978.
Conclusions:
- CGD patients face significant infectious morbidity and mortality, particularly from bacterial and fungal pathogens.
- Prophylactic trimethoprim-sulfamethoxazole is effective for bacterial infections, while ketoconazole offers no protection against Aspergillus.
- Improved survival in recent birth cohorts suggests advancements in management or prophylaxis strategies.