Cystic fibrosis-related diabetes

Abstract

Cystic fibrosis (CF) is an autosomal recessive disorder. It is caused by mutations in CFTR gene that lead to chloride channel dysfunction and consequent changes in composition and properties of the secrets. The clinical presentation is dominated by the involvement of the lungs and pancreas. Thanks to novel therapies, the life expectancy of CF patients has improved significantly within the past years; anyway, the complication rate has also increased. One of them is cystic fibrosis-related diabetes (CFRD). After the age of 30 years old, up to 50 % of patients are currently developing impaired glucose tolerance or overt CFRD. Therefore regular screening is provided using oGTT, and new technologies such as continuous glucose monitoring (sensor) will also be employed in the near future. According to current guidelines, early insulin treatment is recommended. In advanced stages, insulin pump treatment linked with a sensor (hybrid closed loop system) is helpful. The CFRD diet differs from diets in other types of diabetes. Neither energy nor fat intake is reduced. Carbohydrate consumption is only partially restricted, especially the pop drinks.