A Practical Approach to Glucose Abnormalities in Cystic Fibrosis

Abstract

Cystic fibrosis is a common genetic condition and abnormal glucose handling leading to cystic fibrosis-related diabetes (CFRD) is a frequent comorbidity. CFRD is mainly thought to be the result of progressive pancreatic damage resulting in beta cell dysfunction and loss of insulin secretion. Whilst Oral Glucose Tolerance Testing is still recommended for diagnosing CFRD, the relationship between glucose abnormalities and adverse outcomes in CF is complex and occurs at stages of dysglycaemia occurring prior to diagnosis of diabetes by World Health Organisation criteria. Insulin remains the mainstay of treatment of CF-related glucose abnormalities but the timing of insulin commencement, optimum insulin regime and targets of glycaemic control are not clear. These complexities are compounded by common issues with nutritional status, need for enteral feeding, steroid use and high disease burden on CF patients.

Keywords: Cystic fibrosis; Cystic fibrosis-related diabetes; Diabetes; Hypoglycaemia.