A 3-year prognostic score for adults with cystic fibrosis.
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 3years 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.


CONCLUSIONS
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.
Laboratoire Modélisation, Epidémiologie et Surveillance des Risques Sanitaires, Conservatoire National des Arts et Métiers, Paris, France. Electronic address: lionelle.nkam@cnam.fr.
L
L
Nkam
Hôpital Saint-Louis, Service de Biostatistique et Information Médicale, Assistance Publique-Hôpitaux de Paris, Paris, France.
J
J
Lambert
Centre d'Etudes et de Recherche en Informatique et Communications, Conservatoire National des Arts et Métiers, Paris, France.
A
A
Latouche
Institut National d'Etudes Démographiques, Paris, France.
G
G
Bellis
Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Service de Pneumologie, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France.
P R
PR
Burgel
Laboratoire Modélisation, Epidémiologie et Surveillance des Risques Sanitaires, Conservatoire National des Arts et Métiers, Paris, France.
M N
MN
Hocine
D000328:Adult; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D005260:Female; D005541:Forced Expiratory Volume; D005602:France / Q000453:epidemiology; D006801:Humans; D016040:Lung Transplantation / Q000379:methods / Q000706:statistics & numerical data; D008297:Male; D018579:Patient Selection; D011379:Prognosis; D012042:Registries / Q000706:statistics & numerical data; D012107:Research Design; D018570:Risk Assessment / Q000379:methods / Q000706:statistics & numerical data; D016019:Survival Analysis; D061665:Time-to-Treatment
Cystic fibrosis; Logistic model; Prognostic factors; Prognostic score; Registry data