Cleveland R H, Neish A S, Zurakowski D, Nichols D P, Wohl M E, Colin A A
Department of Radiology, Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
AJR Am J Roentgenol. 1998 Apr;170(4):1067-72. doi: 10.2214/ajr.170.4.9530060.
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.
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.
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.
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.
本研究展示了一个基于X线摄影的数据库，该数据库记录了大量囊性纤维化患者随时间的变化情况。此数据库的目的是为接受实验性治疗的患者群体提供比较，以评估治疗效果。这些数据还可用于将个体与年龄匹配的囊性纤维化队列进行比较。
对230例患者的3038张胸部X线片采用布拉斯菲尔德系统进行评分。将所有患者X线片的评分按年龄分别绘制，并创建了一条基于年龄的严重程度曲线。将基于年龄的严重程度曲线与来自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。
我们发现观察者间和观察者内的可靠性都很高。观察者之间的差异平均为1.3个布拉斯菲尔德评分点，该评分范围最高可达25分。基于年龄的严重程度曲线表示为平均布拉斯菲尔德评分与年龄（出生至30岁以上）的关系，并绘制了95%置信区间；该曲线也以百分位数绘制。这条曲线的下降速率与该患者群体肺功能研究的下降速率相似。
基于年龄的曲线是一个结构解剖参数，与肺功能研究（功能性参数）不同。因此，基于年龄的严重程度曲线为组间和个体间的比较提供了一个额外的、独立的基础。它可用于肺部疾病的初始评估以及衡量和预测下降速率。该曲线可作为一个长期结果标准，用于评估囊性纤维化患者群体中的新治疗方法。
