PURPOSE OF REVIEW: To provide an updated literature review highlighting important aspects of cystic fibrosis-related diabetes (CFRD) including epidemiology, pathogenesis, complications, screening, and management. RECENT FINDINGS: Although CFRD continues to be associated with increased rates of mortality in the cystic fibrosis (CF) population, this has improved over the past several years, and the previous sex difference is no longer present. Recent studies support that CFRD is primarily caused by insulin deficiency due to loss of beta cells, which may occur via a number of mechanisms including oxidative stress. Aggressive screening programs with oral-glucose tolerance testing and early treatment with insulin for patients with CFRD with or without fasting hyperglycemia have led to improvements in nutritional states and lung function. Oral agents do not appear to be effective in CFRD. SUMMARY: CFRD is the most common comorbidity in the CF population and is associated with microvascular complications and protein catabolism leading to worse health outcomes. Recognition of glycemic abnormalities through aggressive screening has led to improvements in nutritional status, pulmonary function, and mortality rates.
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