Weatherly M R, Palmer C G, Peters M E, Green C G, Fryback D, Langhough R, Farrell P M
University of Wisconsin, Madison.
Pediatrics. 1993 Feb;91(2):488-95.
A new clinical scoring system for patients with cystic fibrosis is needed because of recent advances in diagnosis and treatment which have changed the course of this disease. Chest radiograph scoring is the best objective measure of pulmonary disease for longitudinal studies beginning with infants; however, based on pilot studies, previous scoring systems are not sensitive enough in discriminating between degrees of mild lung disease. Therefore, a new radiographic scoring system was developed with the goal of achieving both sensitivity and reproducibility. This objective was pursued by applying multiattribute utility theory, using a panel of interpreters with expertise in cystic fibrosis radiology, and employing mathematical modeling techniques to weight the various components. The system was developed and validated in three phases including comparison to the Brasfield method of quantitative radiology. The data demonstrate that the new system can be applied reliably and conveniently to generate reproducible scores of pulmonary disease severity. Evaluation of the scores by four independent raters using chest radiographs from 61 patients at an average age of 8.37 years revealed good agreement with a .714 Kendall coefficient of concordance. Assessment of serial changes over time was performed using a group of 176 chest radiographs from 25 patients ranging from 4 weeks to 6 years old; this showed that the Wisconsin system generates score differences that are greater in magnitude with disease progression compared with the Brasfield method. Therefore, the new method is more sensitive to progression of mild disease and should be superior to prior radiographic scoring systems for evaluating therapies designed to modify the early course of disease. The Wisconsin system is designed to be useful in longitudinal clinical studies involving young children with cystic fibrosis and is capable to detecting progression from normality to mild lung disease.
由于近期诊断和治疗方面的进展改变了囊性纤维化疾病的进程，因此需要一种针对囊性纤维化患者的新临床评分系统。胸部X光片评分是对从婴儿期开始的纵向研究中肺部疾病的最佳客观测量方法；然而，根据初步研究，以前的评分系统在区分轻度肺部疾病程度方面不够敏感。因此，开发了一种新的X光片评分系统，目标是实现敏感性和可重复性。通过应用多属性效用理论、使用一组具有囊性纤维化放射学专业知识的解读人员，并采用数学建模技术对各个组成部分进行加权来实现这一目标。该系统分三个阶段开发和验证，包括与定量放射学的布拉斯菲尔德方法进行比较。数据表明，新系统可以可靠且方便地应用，以生成可重复的肺部疾病严重程度评分。四名独立评估人员使用61名平均年龄为8.37岁患者的胸部X光片对评分进行评估，结果显示肯德尔和谐系数为0.714，一致性良好。使用一组来自25名年龄在4周至6岁之间患者的176张胸部X光片对随时间的系列变化进行评估；结果表明，与布拉斯菲尔德方法相比，威斯康星系统生成的评分差异在疾病进展时幅度更大。因此，新方法对轻度疾病进展更敏感，在评估旨在改变疾病早期进程的治疗方法时应优于先前的X光片评分系统。威斯康星系统旨在用于涉及患有囊性纤维化幼儿的纵向临床研究，并且能够检测从正常到轻度肺部疾病的进展。
