Cystic fibrosis-related diabetes (CFRD) is a major co-morbidity generally affecting patients over 15 years old and it is associated with increased morbidity and mortality. The pathophysiology includes exocrine tissue destruction, insulin deficiency and insulin resistance; the carbohydrate metabolism dysfunction begins with an altered kinetic in insulin secretion followed by a progressive insulin deficiency. Postprandial hyperglycemia is the first abnormality seen in CF patients and the classical symptoms of diabetes may not be recognized. The screening strategy proposed is annual random plasma glucose or fasting plasma glucose investigation, as well as the performance the oral glucose tolerance test (OGTT). Two categories of diabetes are related to CF: CFRD without fasting hyperglycemia (fasting glucose < 126 mg/dL and 2h OGTT glucose > 200 mg/dL) and CFRD with fasting hyperglycemia (fasting glucose > 126 mg/dL). Nutritional management and hyperglycemia control are the CFRD treatment goals. Insulin control is the standard medical therapy for CFRD with fasting hyperglycemia and the benefits of oral insulin secretagogue and sensitizing agents are still controversial.
Cystic Fibrosis-Related Diabetes; Pathophysiology; Diagnosis; Treatment
