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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: an Italian multicenter study.
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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.

机译：意大利多中心研究显示，一大批慢性肉芽肿病患者的临床特征，长期随访和结果。

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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.
机译：对60例慢性肉芽肿病患者进行了回顾性临床和免疫学调查。进行了长期IFNgamma治疗疗效的前瞻性对照非随机研究。诊断时的平均年龄为4.4岁;平均随访时间为10.4年。在诊断之前和随访期间，肺部和皮肤感染是最常见的表现。在我们的队列研究中，曲霉菌是引起感染和死亡的首要原因。死亡率为13％。与对照组相比，长期用IFNγ预防并没有显着改变每患者年的总感染率（p = 0.07）。我们的数据提供了明确的证据，表明持续进行严格的监测和监测抗感染方案的依从性可以显着改善CGD患者的生活质量和长期生存。没有证据证明长期预防性使用IFNγ。

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