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

Baldassarre Martire; Roberto Rondelli; Annarosa Soresina; Claudio Pignata; Teresa Broccoletti; Andrea Finocchi; Paolo Rossi; Marco Gattorno; Marco Rabusin; Chiara Azzari; Rosa M. Dellepiane; Maria C. Pietrogrande; Antonino Trizzino; Paolo Di Bartolomeo; Silvana Martino; Luigi Carpino; Fausto Cossu; Franco Locatelli; Rita Maccario; Paolo Pierani; Maria C. Putti; Achille Stabile; Luigi D. Notarangelo; Alberto G. Ugazio; Alessandro Plebani; Domenico De Mattia

doi: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

Baldassarre Martire, Roberto Rondelli, Annarosa Soresina, Claudio Pignata, Teresa Broccoletti, Andrea Finocchi, Paolo Rossi, Marco Gattorno, Marco Rabusin, Chiara Azzari, Rosa M. Dellepiane, Maria C. Pietrogrande, Antonino Trizzino, Paolo Di Bartolomeo, Silvana Martino, Luigi Carpino, Fausto Cossu, Franco Locatelli, Rita Maccario, Paolo PieraniMaria C. Putti, Achille Stabile, Luigi D. Notarangelo, Alberto G. Ugazio, Alessandro Plebani, Domenico De Mattia

Risultato della ricerca: Contributo in rivista › Articolo in rivista

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.

Lingua originale	English
pagine (da-a)	155-164
Numero di pagine	7
Rivista	Clinical Immunology
Volume	126
DOI	https://doi.org/10.1016/j.clim.2007.09.008
Stato di pubblicazione	Pubblicato - 2008

Keywords

chronic granulomatous disease (CGD)
interferon gamma (IFN gamma)
follow up
itraconazote (ITRA)
infections
cotrimoxazoLe (CTX)

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10.1016/j.clim.2007.09.008

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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, 126, 155-164. https://doi.org/10.1016/j.clim.2007.09.008

@article{37120850bd6d49cb8f05e994b738e243,

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

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.",

keywords = "chronic granulomatous disease (CGD), interferon gamma (IFN gamma), follow up, itraconazote (ITRA), infections, cotrimoxazoLe (CTX), chronic granulomatous disease (CGD), interferon gamma (IFN gamma), follow up, itraconazote (ITRA), infections, cotrimoxazoLe (CTX)",

author = "Baldassarre Martire and Roberto Rondelli and Annarosa Soresina and Claudio Pignata and Teresa Broccoletti and Andrea Finocchi and Paolo Rossi and Marco Gattorno and Marco Rabusin and Chiara Azzari and Dellepiane, {Rosa M.} and Pietrogrande, {Maria C.} and Antonino Trizzino and {Di Bartolomeo}, Paolo and Silvana Martino and Luigi Carpino and Fausto Cossu and Franco Locatelli and Rita Maccario and Paolo Pierani and Putti, {Maria C.} and Achille Stabile and Notarangelo, {Luigi D.} and Ugazio, {Alberto G.} and Alessandro Plebani and {De Mattia}, Domenico",

year = "2008",

doi = "10.1016/j.clim.2007.09.008",

language = "English",

volume = "126",

pages = "155--164",

journal = "Clinical Immunology",

issn = "1521-6616",

publisher = "Academic Press Inc.",

}

Martire, B, Rondelli, R, Soresina, A, Pignata, C, Broccoletti, T, Finocchi, A, Rossi, P, Gattorno, M, Rabusin, M, Azzari, C, Dellepiane, RM, Pietrogrande, MC, Trizzino, A, Di Bartolomeo, P, Martino, S, Carpino, L, Cossu, F, Locatelli, F, Maccario, R, Pierani, P, Putti, MC, Stabile, A, Notarangelo, LD, Ugazio, AG, Plebani, A & 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, vol. 126, pagg. 155-164. https://doi.org/10.1016/j.clim.2007.09.008

TY - JOUR

T1 - Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: an Italian multicenter study

AU - Martire, Baldassarre

AU - Rondelli, Roberto

AU - Soresina, Annarosa

AU - Pignata, Claudio

AU - Broccoletti, Teresa

AU - Finocchi, Andrea

AU - Rossi, Paolo

AU - Gattorno, Marco

AU - Rabusin, Marco

AU - Azzari, Chiara

AU - Dellepiane, Rosa M.

AU - Pietrogrande, Maria C.

AU - Trizzino, Antonino

AU - Di Bartolomeo, Paolo

AU - Martino, Silvana

AU - Carpino, Luigi

AU - Cossu, Fausto

AU - Locatelli, Franco

AU - Maccario, Rita

AU - Pierani, Paolo

AU - Putti, Maria C.

AU - Stabile, Achille

AU - Notarangelo, Luigi D.

AU - Ugazio, Alberto G.

AU - Plebani, Alessandro

AU - De Mattia, Domenico

PY - 2008

Y1 - 2008

N2 - 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.

AB - 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.

KW - chronic granulomatous disease (CGD)

KW - interferon gamma (IFN gamma)

KW - follow up

KW - itraconazote (ITRA)

KW - infections

KW - cotrimoxazoLe (CTX)

KW - chronic granulomatous disease (CGD)

KW - interferon gamma (IFN gamma)

KW - follow up

KW - itraconazote (ITRA)

KW - infections

KW - cotrimoxazoLe (CTX)

UR - http://hdl.handle.net/10807/258447

U2 - 10.1016/j.clim.2007.09.008

DO - 10.1016/j.clim.2007.09.008

M3 - Article

SN - 1521-6616

VL - 126

SP - 155

EP - 164

JO - Clinical Immunology

JF - Clinical Immunology

ER -

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

Abstract

Keywords

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