In this large predominantly francophone cohort, individuals with compoundheterozygous F508del/L206W or F508del/A455E CFTR variants 1) tended to have lessfrequent exocrine pancreatic insufficiency; 2) had preserved pulmonary function; 3)better clinical and nutritional status; and 4) better glucose homeostasis indiceswhich tended to translate into a lower risk of developing CFRD. This evidence concerns the gene CFTR and cystic fibrosis-related diabetes.