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Published ahead of print on August 15, 2002, doi:10.1164/rccm.200202-087OC

Am. J. Respir. Crit. Care Med., Volume 166, Number 12, December 2002, 1550-1555

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Submitted on February 8, 2002
Accepted on August 9, 2002

Developing Cystic Fibrosis Lung Transplant Referral Criteria Using Predictors of Two Year Mortality

Nicole Mayer-Hamblett1*, Margaret Rosenfeld2, Julia Emerson2, Christopher H Goss3, and Moira L Aitken3

1 Pediatrics, University of Washington, Seattle, WA, USA; Statistical Analysis Unit, CF Therapeutics Development Network Coordinating Center, Children's Hospital and Regional Medical Center, Seattle, WA, USA, 2 Pediatrics, University of Washington, Seattle, WA, USA, 3 Medicine, University of Washington, Seattle, WA, USA

* To whom correspondence should be addressed. E-mail: nhambl{at}chmc.org.

The first study objective was to develop a model identifying the best clinical predictors of 2-year mortality among cystic fibrosis patients, in order to assist in selection of appropriate candidates for lung transplantation. Using multivariate logistic regression, age, height, FEV1, respiratory microbiology, number of hospitalizations for pulmonary exacerbations, and number of home intravenous antibiotic courses were all found to be significant predictors of 2-year mortality among 14,572 patients in the Cystic Fibrosis Foundation National Patient Registry >=6 years of age in 1996. The second objective was to compare the diagnostic accuracy of our model when used to guide referral for lung transplant to that of the widely used criterion of an FEV1 <30% predicted. Surprisingly, this well-fitting model derived from the largest collection of cystic fibrosis patient data available provided no better diagnostic accuracy than the simpler FEV1 criterion. Both had high negative predictive values (98% and 97%, respectively), but only modest positive predictive values (33% and 28%, respectively). Transplant referral decisions based either on a multivariate logistic model or the criterion of an FEV1 <30% predicted are likely to result in high rates of premature referral. Better clinical predictors of short-term mortality among cystic fibrosis patients are needed.


Key words: Cystic Fibrosis, mortality, lung transplantation, logistic models.




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