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 IFN gamma 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 IFN gamma 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 tong-term survival in patients with CGD. No evidence justifying Long-term prophylaxis with IFN gamma was obtained. (c) 2007 Elsevier Inc. All rights reserved.
Martire, B., Rondelli, R., Soresina, A., Pignata, C., Broccoletti, T., Finocchi, A., Rossi, P., Gattorno, M., Rabusin, M., Azzari, C., Dellepiane, R. M., Pietrogrande, M. C., Trizzino, A., Di Bartolomeo, P., Martino, S., Carpino, L., Cossu, F., Locatelli, F., Maccario, R., Pierani, P., Putti, M. C., Stabile, A., Notarangelo, L. D., Ugazio, A. G., Plebani, A., De Mattia, D., Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: an Italian multicenter study, <<CLINICAL IMMUNOLOGY>>, 2008; 126 (8): 155-164. [doi:10.1016/j.clim.2007.09.008] [https://hdl.handle.net/10807/258447]
Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: an Italian multicenter study
Gattorno, Marco;Locatelli, Franco;
2008
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 IFN gamma 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 IFN gamma 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 tong-term survival in patients with CGD. No evidence justifying Long-term prophylaxis with IFN gamma was obtained. (c) 2007 Elsevier Inc. All rights reserved.
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