PEDIATRICS Vol. 91 No. 2 February 1993, pp. 488-495
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Weatherly, M. R.
Right arrow Articles by Farrell, P. M.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Weatherly, M. R.
Right arrow Articles by Farrell, P. M.

Wisconsin Cystic Fibrosis Chest Radiograph Scoring System

Mark R. Weatherly MD1, Christina G.S. Palmer MS1, Mary Ellen Peters MD1, Christopher G. Green MD1, Dennis Fryback PhD1, Rebecca Langhough MS1, and Philip M. Farrell MD, PhD1

1 From the University of Wisconsin, Madison

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.

Key Words: cystic fibrosis • chest radiograph • lung disease • multiattribute utility model • scoring system • severity index

Submitted on July 3, 1992
Accepted on October 15, 1992




This article has been cited by other articles:


Home page
 Eur Respir JHome page
S. W. J. Terheggen-Lagro, H. G. M. Arets, J. van der Laag, and C. K. van der Ent
Radiological and functional changes over 3 years in young children with cystic fibrosis
Eur. Respir. J., August 1, 2007; 30(2): 279 - 285.
[Abstract] [Full Text] [PDF]

Home page
 Arch. Dis. Child.Home page
S C Langton Hewer
Is limited computed tomography the future for imaging the lungs of children with cystic fibrosis?
Arch. Dis. Child., May 1, 2006; 91(5): 377 - 378.
[Full Text] [PDF]

Home page
 Am. J. Respir. Crit. Care Med.Home page
R. L. Gibson, J. L. Burns, and B. W. Ramsey
Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis
Am. J. Respir. Crit. Care Med., October 15, 2003; 168(8): 918 - 951.
[Abstract] [Full Text] [PDF]

Home page
 PediatricsHome page
R. J. Singleton, G. J. Redding, T. C. Lewis, P. Martinez, L. Bulkow, B. Morray, H. Peters, J. Gove, C. Jones, D. Stamey, et al.
Sequelae of Severe Respiratory Syncytial Virus Infection in Infancy and Early Childhood Among Alaska Native Children
Pediatrics, August 1, 2003; 112(2): 285 - 290.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Roentgenol.Home page
D. P. Frush, L. F. Donnelly, and H. G. Chotas
Contemporary Pediatric Thoracic Imaging
Am. J. Roentgenol., September 1, 2000; 175(3): 841 - 851.
[Full Text] [PDF]

Home page
 RadiologyHome page
L. F. Donnelly, J. R. MacFall, H. P. McAdams, J. M. Majure, J. Smith, D. P. Frush, P. Bogonad, H. C. Charles, and C. E. Ravin
Cystic Fibrosis: Combined Hyperpolarized 3He-enhanced and Conventional Proton MR Imaging in the Lung-Preliminary Observations
Radiology, September 1, 1999; 212(3): 885 - 889.
[Abstract] [Full Text]

Home page
 PediatricsHome page
F. Santamaria, G. Grillo, G. Guidi, A. Rotondo, V. Raia, G. de Ritis, P. Sarnelli, M. Caterino, and L. Greco
Cystic Fibrosis: When Should High-resolution Computed Tomography of the Chest Be Obtained?
Pediatrics, May 1, 1998; 101(5): 908 - 913.
[Abstract] [Full Text] [PDF]