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首页> 外文期刊>Revista Paulista de Pediatria >A first-year dornase alfa treatment impact on clinical parameters of patients with cystic fibrosis: the Brazilian cystic fibrosis multicenter study
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A first-year dornase alfa treatment impact on clinical parameters of patients with cystic fibrosis: the Brazilian cystic fibrosis multicenter study

机译：催眠阿尔法治疗的第一年对囊性纤维化患者的临床指标的影响：巴西囊性纤维化多中心研究

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OBJECTIVE: To describe the clinical impact of the first year treatment with dornase alfa, according to age groups, in a cohort of Brazilian Cystic Fibrosis (CF) patients. METHODS: The data on 152 eligible patients, from 16 CF reference centers, that answered the medical questionnaires and performed laboratory tests at baseline (T0), and at six (T2) and 12 (T4) months after dornase alfa initiation, were analyzed. Three age groups were assessed: six to 11, 12 to 13, and 14 years. Pulmonary tests, airway microbiology, emergency room visits, hospitalizations, emergency and routine treatments were evaluated. Student's t-test, chi-square test and analysis of variance were used when appropriated. RESULTS: Routine treatments were based on respiratory physical therapy, regular exercises, pancreatic enzymes, vitamins, bronchodilators, corticosteroids, and antibiotics. In the six months prior the study (T0 phase), hospitalizations for pulmonary exacerbations occurred in 38.0, 10.0 and 61.4% in the three age groups, respectively. After one year of intervention, there was a significant reduction in the number of emergency room visits in the six to 11 years group. There were no significant changes in forced expiratory volume in one second (VEF1), in forced vital capacity (FVC), in oxygen saturation (SpO2), and in Tiffenau index for all age groups. A significant improvement in Shwachman-Kulczychi score was observed in the older group. In the last six months of therapy, chronic or intermittent colonization by P. aeruginosa was detected in 75.0, 71.4 and 62.5% of the studied groups, respectively, while S. aureus colonization was identified in 68.6, 66.6 and 41.9% of the cases. CONCLUSIONS: The treatment with dornase alfa promoted the maintenance of pulmonary function parameters and was associated with a significant reduction of emergency room visits due to pulmonary exacerbations in the six to 11 years age group, with better clinical scores in the 14 age group, one year after the intervention.
机译：目的：描述按年龄分组对巴西囊性纤维化（CF）患者群进行多奈糖酶α治疗的第一年的临床效果。方法：分析了来自16个CF参考中心的152名合格患者的数据，这些患者回答了医疗问卷并在基线（T0）以及在dornase alfa启动后六个月（T2）和12（T4）进行了实验室测试。评估了三个年龄段：6至11、12至13和> 14岁。评估了肺部检查，气道微生物学，急诊室就诊，住院，急诊和常规治疗。适当时使用学生t检验，卡方检验和方差分析。结果：常规治疗以呼吸道物理治疗，定期运动，胰酶，维生素，支气管扩张药，皮质类固醇和抗生素为基础。在研究之前的六个月（T0期）中，三个年龄组的肺病急性发作住院率分别为38.0％，10.0％和61.4％。经过一年的干预，在6至11年组中急诊室就诊的次数大大减少了。在所有年龄组中，一秒钟的强制呼气量（VEF1），强制肺活量（FVC），氧饱和度（SpO2）和Tiffenau指数均无显着变化。在老年组中，Shwachman-Kulczychi得分明显改善。在治疗的最后六个月中，分别在75.0％，71.4％和62.5％的研究组中发现了铜绿假单胞菌的慢性或间歇性定植，而在68.6％，66.6和41.9％的病例中发现了金黄色葡萄球菌的定植。结论：在6至11岁年龄组中，多奈酶阿尔法治疗可促进肺功能参数的维持，并显着减少因肺病加重而导致急诊就诊的机会，在14岁以上年龄组中，临床评分更高，其中一项干预后一年。

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