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Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: An Italian multicenter study
Open accessAbstract
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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Cited by
Chronic Granulomatous Disease: The European Experience
van den Berg, J. M., van Koppen, E., Åhlin, A., Belohradsky, B. H., Bernatowska, E., Corbeel, L., Español, T., Fischer, A., Kurenko-Deptuch, M., Mouy, R., et al. (2009) · PLoS ONE · doi:10.1371/journal.pone.0005234 · Cited by 687
Holland, S. M. (2010) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-009-8136-z · Cited by 328
Roos, D. (2016) · British Medical Bulletin · doi:10.1093/bmb/ldw009 · Cited by 212
Chronic Granulomatous Disease: a Comprehensive Review
Yu, H.-H., Yang, Y.-H., & Chiang, B.-L. (2021) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-020-08800-x · Cited by 197
Metadata
- Authors
- 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.
- Venue
- Clinical Immunology
- Publisher
- Elsevier BV
- Published
- 2008-02
- Volume
- 126
- Issue
- 2
- Pages
- 155-164
- ISSN
- 1521-6616 (ISSN-L)
- OpenAlex
- W1998805394
- Cited by
- 328
- References
- 0
- FWCI
- 5.27
- Type
- journal article
- Language
- en
- Open access
- green
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- The paper
- 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., . . . De Mattia, D. (2008). Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: An Italian multicenter study. Clinical Immunology. https://doi.org/10.1016/j.clim.2007.09.008
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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