Validation of the French 3-year prognostic score using the Canadian Cystic Fibrosis registry.

Studies of large CF populations using registry data are important to identify people at high risk for death. Nkam et al. published a prognostic score developed on French CF registry data to predict death or lung transplantation (LT) over a 3-year period in the adult CF population. The goal of our study was to validate the proposed tool using the Canadian CF registry. Using data between 2011 and 2014, a total of 2043 adult CF patients were included. We found that the French prognostic score was a good predictor of death or LT in the Canadian CF population (OR for each unit increase: 3.12, 95% CI: 2.74-3.55; p value < 0.001). The proposed prognostic score accurately categorizes patients when applied to an external dataset. This score provides an important tool for early identification of patients at high risk for death or LT, in whom specific therapeutic intervention can be proposed.