Abstract
Cystic fibrosis is a rare autosomal recessive disorder. Mutations in CFTR gene lead to dysfunction of chloride channel.
This results to a change in the composition and properties of secretions, in the clinical picture dominated mainly damage of lung and pancreas. In recent years has greatly improved the median survival, but also are increasing complications.
One of them is cystic fibrosis related diabetes (CFRD). A problem with impaired glucose tolerance or CFRD develops after the 30th year nearly 50% of patients.
Annual screening for CFRD should be performed using the OGTT by the age of 10 years to recognize early stage of CFRD. According to current guidelines, early insulin treatment is recommended.
Treatment should be initiated even in patients with nevysvětlitelnýdeteriorations of lung function and nutritional status. Diet for CFRD is different from other types of diabetes we do not restrict calories or fat intake.
The consumption of carbohydrates is limited only partially, restriction applies to free sugars and sweet drinks.