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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

Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: An Italian multicenter study,10.1

Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: An Italian multicenter study   (Citations: 32)
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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. Pietrograndehttp://academic.research.microsoft.com/io.ashx?type=5&id=29497129&selfId1=0&selfId2=0&maxNumber=12&query=
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.
Journal: Clinical Immunology - CLIN IMMUNOL , vol. 126, no. 2, pp. 155-164, 2008
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    • ...Aspergillus spp., Serratia spp., and Staphylococcus aureus were reported as pathogens causing osteomyelitis in patients with CGD [6, 7]. This is the first case report of osteomyelitis caused by trimethoprim/sulfamethoxazole-resistant E. tarda infection in a patient with X-linked chronic granulomatous disease (X-CGD)...

    T. Kawaiet al. Osteomyelitis due to trimethoprim/sulfamethoxazole-resistant Edwardsie...

    • ...Patients diagnosed before the advent of antifungal azole agents had a very different trajectory of disease, as reflected by the very poor survival of patients into their 30s and 40s in those series [9, 11]...
    • ...This is easily appreciated in the examination of survival rates in several of the large cohorts reported [11, 13, 14]...
    • ...Several large studies have shown a relatively similar rate of infection of around 0.3/year [11, 13]...
    • ...to have a significantly better prognosis than X-linked disease [9, 11]...
    • ...However, a retrospective Italian study detected no benefit to the addition of IFN-gamma beyond that attributed to antibacterial and antifungals alone [11]...

    Steven M. Holland. Chronic Granulomatous Disease

    • ...The frequency of oral complications in CGD is variable and can range from few cases in some large series [1,15,16], to as high as 35% in other studies [17]...
    • ...This is among the highest reported frequency [17]...
    • ...The rate and severity of oral disease varies across different studies; one study [17] found rates of 35% while several other large studies [1,15,16,26,28,29] found low rates ranging from 0 to 26%...
    • ...Today, this observation is still valid as most studies in CGD still refer to the oral complications with the same non specific terminology such as “gingivostomatitis” [17], “ulcerative stomatits”, “gingivitis” ,a nd“periodentitis” [1,28,36] making it difficult both to make accurate comparisons between studies, and to describe the spectrum of the oral lesions...

    Najla S Dar-Odehet al. Orofacial findings in chronic granulomatous disease: report of twelve ...

    • ...The most frequent severe infections were in lung (four), liver (three), bone (four), chest (four), and brain (one; Table I). Lung and liver infections seem to be the most frequent severe infections in CGD patients [43, 44]...
    • ...However, based on a large patient cohort, Martire et al. recently demonstrated that long-term prophylaxis with interferon γ was not justified [44]...
    • ...Ten patients showed failure to thrive, nine patients suffered from skin or neck infections, five presented lymphadenitis (Table I). According to recent clinical data, skin infections are the most frequent mild infection, as we described [44]...

    Faris G. Bakriet al. First Report of Clinical, Functional, and Molecular Investigation of C...

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