Diabetes is a frequent complication seen in cystic fibrosis patients as they reach adulthood. Cystic fibrosis related diabetes (CFRD) is distinguished as a separate entity with features that include progressive loss of islet beta cell mass and insulin deficiency, as well as insulin resistance. Abnormalities in glucose tolerance may be detectable for many years prior to the development of overt diabetes. Therefore oral glucose tolerance testing is the preferred screening method for the identification of those patients at the highest risk for progression to diabetes. Progression to diabetes has been linked to poor outcomes in CF including loss of pulmonary function and increased mortality among females. Given the role that insulin deficiency plays in CFRD, insulin replacement therapy remains the only recommended intervention. In the absence of definitive supportive data, the use of oral antidiabetic agents is not considered standard therapy and needs further study. As with other forms of diabetes, CFRD patients also experience microvascular complications and should be periodically evaluated for manifestations.
ABSTRACT