Cystic fibrosis: A system for assessing and predicting progression

Cystic fibrosis: A system for assessing and predicting progression
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DOI:
10.2214/ajr.170.4.9530060
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发表时间:
1998-04-01
影响因子:
5
通讯作者:
Colin, AA
Colin, AA
中科院分区:
医学2区
文献类型:
--
作者:
Cleveland, RH;Neish, AS;Colin, AA

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目标。本研究在大量囊性纤维化患者中提出了一种基于放射学的数据库评分随时间的变化。该数据库的目的是为接受实验性治疗的患者组提供比较,以评估治疗效果。这些数据也可用于比较患有囊性纤维化的个体与其年龄匹配的队列。材料和方法。从230名患者中,使用Brasfield系统对3038张胸片进行评分。所有患者的x线片评分分别按年龄绘制,并创建了一个基于年龄的严重程度曲线。将基于年龄的严重程度曲线与同一患者群体子集的肺功能研究得出的类似曲线进行比较。我们发现观察者之间和观察者内部的高可靠性。观察者之间的差异平均为1.3布拉斯菲尔德分,其范围可达25分。基于年龄的严重程度曲线以平均Brasfield评分与年龄(出生至30岁)的关系绘制,具有95%的置信限;曲线也以百分位数绘制。该曲线的下降速率与该患者群体肺功能的下降速率相似。基于年龄的曲线是一种结构解剖参数,与肺功能研究不同,肺功能研究是功能性的。因此,基于年龄的严重程度曲线为群体和个人之间的比较提供了额外的、独立的基础。它可用于肺部疾病的初步评估和衡量和预测下降的速度。该曲线可作为评价囊性纤维化患者群体新治疗方法的远期预后标准。
OBJECTIVE. This study presents a radiography-based database scoring changes over time in a large population of patients with cystic fibrosis. The purpose of this database is to provide comparison for groups of patients undergoing experimental treatment to assess effect of the treatment. The data may also be used to compare individuals with their age-matched cohorts with cystic fibrosis.MATERIALS AND METHODS. From 230 patients, 3038 chest radiographs were scored using the Brasfield system. The scores from radiographs from all the patients were individually plotted for age, and a single age-based severity curve was created. The age-based severity curve was compared with similar curves derived from pulmonary function studies of a subset of the same patient population.RESULTS. We found high inter-and intraobserver reliability. The difference between the observers averaged 1.3 Brasfield points, the scale of which ranges up to 25 points. The age-based severity curve was presented as mean Brasfield scores versus age (birth to >30 years) plotted with 95% confidence limits; the curve was also plotted in percentiles. The rate of decline of this curve was similar to the decline of pulmonary function studies in this patient population.CONCLUSION. The age-based curve, a structural anatomic parameter, differs from pulmonary function studies, which are functional. Thus the age-based severity curve provides an additional, independent basis for comparison between groups and individuals. It may be used for the initial assessment of lung disease and for gauging and predicting the rate of decline. The curve may be used as a long-range outcome criterion to evaluate new treatments in groups of patients with cystic fibrosis.