Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease an Italian multicenter study

Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease : an Italian multicenter study

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 IFNgamma treatment was carried out. The mean age at the time of diagnosis was 4.4 years; mean duration of foll...

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Veröffentlicht in:Clinical immunology (Orlando, Fla.). - 1999. - 126(2008), 2 vom: 08. Feb., Seite 155-64
1. Verfasser: Martire, Baldassarre (VerfasserIn)
Weitere Verfasser: Rondelli, Roberto, Soresina, Annarosa, Pignata, Claudio, Broccoletti, Teresa, Finocchi, Andrea, Rossi, Paolo, Gattorno, Marco, Rabusin, Marco, Azzari, Chiara, Dellepiane, Rosa M, Pietrogrande, Maria C, Trizzino, Antonino, Di Bartolomeo, Paolo, Martino, Silvana, Carpino, Luigi, Cossu, Fausto, Locatelli, Franco, Maccario, Rita, Pierani, Paolo, Putti, Maria C, Stabile, Achille, Notarangelo, Luigi D, Ugazio, Alberto G, Plebani, Alessandro, De Mattia, Domenico, IPINET
Format: Aufsatz
Sprache:English
Veröffentlicht: 2008
Zugriff auf das übergeordnete Werk:Clinical immunology (Orlando, Fla.)
Schlagworte:Journal Article Multicenter Study Anti-Infective Agents Antifungal Agents Antiviral Agents Itraconazole 304NUG5GF4 Trimethoprim, Sulfamethoxazole Drug Combination 8064-90-2 Interferon-gamma 82115-62-6
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520 |a 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 IFNgamma 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 IFNgamma 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 IFNgamma was obtained 
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700 1 |a Rondelli, Roberto  |e verfasserin  |4 aut 
700 1 |a Soresina, Annarosa  |e verfasserin  |4 aut 
700 1 |a Pignata, Claudio  |e verfasserin  |4 aut 
700 1 |a Broccoletti, Teresa  |e verfasserin  |4 aut 
700 1 |a Finocchi, Andrea  |e verfasserin  |4 aut 
700 1 |a Rossi, Paolo  |e verfasserin  |4 aut 
700 1 |a Gattorno, Marco  |e verfasserin  |4 aut 
700 1 |a Rabusin, Marco  |e verfasserin  |4 aut 
700 1 |a Azzari, Chiara  |e verfasserin  |4 aut 
700 1 |a Dellepiane, Rosa M  |e verfasserin  |4 aut 
700 1 |a Pietrogrande, Maria C  |e verfasserin  |4 aut 
700 1 |a Trizzino, Antonino  |e verfasserin  |4 aut 
700 1 |a Di Bartolomeo, Paolo  |e verfasserin  |4 aut 
700 1 |a Martino, Silvana  |e verfasserin  |4 aut 
700 1 |a Carpino, Luigi  |e verfasserin  |4 aut 
700 1 |a Cossu, Fausto  |e verfasserin  |4 aut 
700 1 |a Locatelli, Franco  |e verfasserin  |4 aut 
700 1 |a Maccario, Rita  |e verfasserin  |4 aut 
700 1 |a Pierani, Paolo  |e verfasserin  |4 aut 
700 1 |a Putti, Maria C  |e verfasserin  |4 aut 
700 1 |a Stabile, Achille  |e verfasserin  |4 aut 
700 1 |a Notarangelo, Luigi D  |e verfasserin  |4 aut 
700 1 |a Ugazio, Alberto G  |e verfasserin  |4 aut 
700 1 |a Plebani, Alessandro  |e verfasserin  |4 aut 
700 1 |a De Mattia, Domenico  |e verfasserin  |4 aut 
700 0 |a IPINET  |e verfasserin  |4 aut 
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