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A 3-year prognostic score for adults with cystic fibrosis
Authors:L Nkam  J Lambert  A Latouche  G Bellis  PR Burgel  MN Hocine
Institution:1. Laboratoire Modélisation, Epidémiologie et Surveillance des Risques Sanitaires, Conservatoire National des Arts et Métiers, Paris, France;2. Hôpital Saint-Louis, Service de Biostatistique et Information Médicale, Assistance Publique-Hôpitaux de Paris, Paris, France;3. Centre d''Etudes et de Recherche en Informatique et Communications, Conservatoire National des Arts et Métiers, Paris, France;4. Institut National d''Etudes Démographiques, Paris, France;5. Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Service de Pneumologie, Paris, France;6. Université Paris Descartes, Sorbonne Paris Cité, Paris, France
Abstract:

Background

Therapeutic progress in patients with cystic fibrosis (CF) has resulted in improved prognosis over the past decades. We aim to reevaluate prognostic factors of CF and provide a prognostic score to predict the risk of death or lung transplantation (LT) within a 3-year period in adult patients.

Methods

We developed a logistic model using data from the French CF Registry and combined the coefficients into a prognostic score. The discriminative abilities of the model and the prognostic score were assessed by c-statistic. The prognostic score was validated using a 10-fold cross-validation.

Results

The risk of death or LT within 3 years was related to eight characteristics. The development and the validation provided excellent results for the prognostic score; the c-statistic was 0.91 and 0.90 respectively.

Conclusion

The score developed to predict 3-year death or LT in adults with CF might be useful for clinicians to identify patients requiring specialized evaluation for LT.
Keywords:Cystic fibrosis  Prognostic factors  Registry data  Logistic model  Prognostic score
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