LAUSR

Cystic fibrosis (CF) is the most common lethal genetic autosomal recessive disease in Caucasians, with a worldwide prevalence of 1 in ~2500 live births. Cystic fibrosis related diabetes (CFRD) is the most common co-morbidity in CF. There are important pathophysiologic differences between CFRD and type 1 and type 2 diabetes Table 1, which necessitate a unique approach to diagnosis and management. Factors specific to CF which impact glucose metabolism include the loss of total islets leading to both insulin and glucagon deficiency, chronic and acute inflammation and infection which cause fluctuating insulin resistance, a requirement for high caloric intake because of increased energy expenditure and malabsorption, risk of life-threatening malnutrition, and gut abnormalities including delayed gastric emptying, altered intestinal motility, and liver disease. This article is protected by copyright. All rights reserved.