All the Forced Expiratory Volume in 1 s (FEV1) measurements made between 2010 and 2015 have been extracted from the medical records of the cohort’s patients. FEV1 values (in L) have been converted in Z-score adjusted for age, sex and height by non-linear regression following the Global Lung Initiative recommendations. These international guidelines also advocate to adjust this Z-score according to ethnicity, but the French legislation does not allow to collect this information. Considering the greatest prevalence of CF in Caucasians, we therefore considered all our patients as of Caucasian origin in this calculation [56].

Patients have been stratified according to both the level and the temporal dynamics of all FEV1 records during the 2010–2015 follow-up period. Mean FEV1 was classified as high if above the cohort average, low if not. Long-term dynamic of the FEV1 was defined as in decline if the slope of the linear regression of the FEV1 over 5 years was significantly below 0, not declining if not. Short-term dynamic was defined as unstable if the standard deviation of the residuals around the linear regression of the FEV1 over time was above 40%, stable if not. One indicator of each Mean FEV1, Long-term dynamic and Short-term dynamic have been used to describe each patient’s clinical state over the study period.

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