Abstract
A retrospective clinical and immunological survey was conducted in 60 patients with Chronic Granulomatous Disease. A prospective controlled non-randomized study of the efficacy of long-term IFNγ treatment was carried out. The mean age at the time of diagnosis was 4.4 years; mean duration of follow-up was 10.4 years. Lung and skin infections were the most frequent manifestations both prior to diagnosis and during follow-up. Aspergillus species was the first cause of infection and of death in our cohort. The mortality rate was 13%. Long term prophylaxis with IFNγ did not significantly change the rate of total infection per patient-year compared to controls (p = 0.07). Our data provide clear evidence that protocols of continuing intensive surveillance and monitoring of compliance with anti-infective regimens may significantly improve the quality of life and long-term survival in patients with CGD. No evidence justifying long-term prophylaxis with IFNγ was obtained.
Original language | English |
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Pages (from-to) | 155-164 |
Number of pages | 10 |
Journal | Clinical Immunology |
Volume | 126 |
Issue number | 2 |
DOIs | |
Publication status | Published - Feb 2008 |
Keywords
- Chronic Granulomatous Disease (CGD)
- Cotrimoxazole (CTX)
- Follow up
- Infections
- Interferon gamma (IFNγ)
- Itraconazole (ITRA)
ASJC Scopus subject areas
- Immunology
- Immunology and Allergy