Cystic Fibrosis Related Diabetes - An Update

Cystic fibrosis (CF) is the most common life-threatening inherited condition in the Caucasian population, where mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) 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 CFTR 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 cystic fibrosis related diabetes (CFRD), itself associated with increased morbidity and early mortality. This review provides an update as regards the underlying mechanisms, investigation and management of CFRD.