American Journal of Epidemiology Vol. 153, No. 4 : 345-352
Copyright © 2001 by The Johns Hopkins University School of Hygiene and Public Health
ORIGINAL CONTRIBUTIONS |
Predictive 5-Year Survivorship Model of Cystic Fibrosis
1 Division of Respiratory, Critical Care and Occupational Pulmonary Medicine, Department of Internal Medicine, Health Sciences Center, University of Utah, Salt Lake City, UT.
2 The Intermountain Cystic Fibrosis Center, University of Utah, Salt Lake City, UT.
3 Department of Mathematics, University of Utah, Salt Lake City, UT.
4 Department of Biology, University of Utah, Salt Lake City, UT.
5 Cystic Fibrosis Foundation, Bethesda, MD.
6 Lung Transplantation Program, Health Sciences Center, University of Utah, Salt Lake City, Utah.
7 The New York State Department of Health, Albany, NY.
8 The Salt Lake Veteran's Administration Medical Center, Salt Lake City, UT.
9 Present address: FitzSimmons and Associates, Bethesda, MD.
The objective of this study was to create a 5-year survivorship model to identify key clinical features of cystic fibrosis. Such a model could help researchers and clinicians to evaluate therapies, improve the design of prospective studies, monitor practice patterns, counsel individual patients, and determine the best candidates for lung transplantation. The authors used information from the Cystic Fibrosis Foundation Patient Registry (CFFPR), which has collected longitudinal data on approximately 90% of cystic fibrosis patients diagnosed in the United States since 1986. They developed multivariate logistic regression models by using data on 5,820 patients randomly selected from 11,630 in the CFFPR in 1993. Models were tested for goodness of fit and were validated for the remaining 5,810 patients for 1993. The validated 5-year survivorship model included age, forced expiratory volume in 1 second as a percentage of predicted normal, gender, weight-for-age z score, pancreatic sufficiency, diabetes mellitus, Staphylococcus aureus infection, Burkerholderia cepacia infection, and annual number of acute pulmonary exacerbations. The model provides insights into the complex nature of cystic fibrosis and supplies a rigorous tool for clinical practice and research.
cystic fibrosis; logistic models; models; theoretical; multivariate analysis; proportional hazards models; survival analysis
Abbreviations: CFFPR, Cystic Fibrosis Foundation Patient Registry; FEV1, forced expiratory volume in 1 second; FEV1%, forced expiratory volume in 1 second as a percentage of predicted normal
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|
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|
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|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
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|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
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|
|
|
|
 |
|
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|
|
|
|
 |
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
P. G. Noone and T. M. Egan Cystic Fibrosis: When to Refer for Lung Transplantation-Is the Answer Clear? Am. J. Respir. Crit. Care Med., December 15, 2002; 166(12): 1531 - 1532. [Full Text] [PDF]
|
|
|
|
|
|
N. Mayer-Hamblett, M. Rosenfeld, J. Emerson, C. H. Goss, and M. L. Aitken Developing Cystic Fibrosis Lung Transplant Referral Criteria Using Predictors of 2-Year Mortality Am. J. Respir. Crit. Care Med., December 15, 2002; 166(12): 1550 - 1555. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. M. Nixon, J. A. Glazner, J. M. Martin, and S. M. Sawyer Urinary Incontinence in Female Adolescents With Cystic Fibrosis Pediatrics, August 1, 2002; 110(2): e22 - 22. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H.-C. Lai, M. R. Kosorok, A. Laxova, L. M. Makholm, and P. M. Farrell Delayed Diagnosis of US Females with Cystic Fibrosis Am. J. Epidemiol., July 15, 2002; 156(2): 165 - 173. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P Aurora, A Wade, M E Hodson, R Sharma, and S D Anker Wasting as predictor of survival in CF Thorax, May 1, 2002; 57(5): 468 - 468. [Full Text] [PDF]
|
|
|
|
|
|
T. G. Liou, F. R. Adler, B. C. Cahill, S. C. FitzSimmons, D. Huang, J. R. Hibbs, B. C. Marshall, A. B. Maish, J. R. Maurer, T. G. Liou, et al. Priorities for Lung Transplantation Among Patients With Cystic Fibrosis JAMA, March 27, 2002; 287(12): 1523 - 1525. [Full Text] [PDF]
|
|
|
|
|
|
T. G. Liou, F. R. Adler, B. C. Cahill, S. C. FitzSimmons, D. Huang, J. R. Hibbs, and B. C. Marshall Survival Effect of Lung Transplantation Among Patients With Cystic Fibrosis JAMA, December 5, 2001; 286(21): 2683 - 2689. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. R. Maurer Patient Selection for Lung Transplantation JAMA, December 5, 2001; 286(21): 2720 - 2721. [Full Text] [PDF]
|
|
|
|
|
|
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|
|