Symposium 15: Diabetes in cystic fibrosis

Abstract

Symposium 15: Other conditions and diabetes

Diabetes in cystic fibrosis

Cystic fibrosis is a very common autosomal recessive disorder characterized by severely altered or absent function of the CFTR (chloride and bicarbonate transmembrane regulatory channel). With advances in nutritional and pulmonary care, and the prolongation of survival into adulthood, CFRD has emerged as a common comorbidity primarily in subjects with pancreatic insufficiency. CFRD is the final stage of a slow and progressive spectrum of alterations in carbohydrate metabolism that is characterized by early insulinopenia, which produces nutritional deterioration and pulmonary decline.

For decades, cystic fibrosis patient care teams have diagnosed CFRD through OGTT with cut-off points for microvascular complications (such as in DM1 and DM2). Since the lung is the organ primarily at risk in CF, perhaps CFRD should be defined by the level of blood glucose at which pulmonary disease develops. It is likely that insulinopenia with consequent catabolism plays a fundamental role in pulmonary deterioration and that early postprandial hyperglycemia (high glycemic levels at intermediate points but normal at 2 hours) can be used as an indicator of the need to prescribe precision insulin therapy.