Cystic fibrosis-related diabetes: an update

Abstract

Summary Cystic fibrosis (CF) is the most common life-threatening inherited condition in the Caucasian population, where mutations in the CF transmembrane conductance regulator gene result in a multifactorial syndrome, with pulmonary disease representing the largest contributor to morbidity and mortality. Life expectancy has improved and the recent development of disease-modifying CF transmembrane conductance regulator modulator therapies is likely to further improve survival. However, increasing life expectancy brings new challenges related to the complications of a chronic disease including an increasing prevalence of CF-related diabetes, itself associated with increased morbidity and early mortality. This review provides an update as regards the underlying mechanisms, investigation and management of CF-related diabetes.