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. © 2007 Elsevier Inc. All rights reserved.
Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: An Italian multicenter study / 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.. - In: CLINICAL IMMUNOLOGY. - ISSN 1521-6616. - 126:2(2008), pp. 155-164. [10.1016/j.clim.2007.09.008]
Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: An Italian multicenter study
Locatelli F.;
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 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. © 2007 Elsevier Inc. All rights reserved.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
