Abstract

Background: Cystic fibrosis (CF) is a disease that occurs as a result of genetic mutations in cystic fibrosis transmembrane conductance regulator gene (CFTR). CFTR can be found in airways epithelial cells, intestine and cells with exocrine and exocrine function. CF is associated with variant complications, Cystic Fibrosis-Related Diabetes (CFRD) is considered as one of the most common complications of CF. Objective: In this review, we discussed the recent updates about Cystic Fibrosis-Related Diabetes (CFRD) diagnosis and management plan. Methods: PubMed database were used for articles selection. All relevant articles related to our review were chosen to cover the following topics: Cystic Fibrosis, Cystic Fibrosis Related Diabetes management, Cystic Fibrosis Related Diabetes diagnosis. We excluded other articles, which are not related to our objectives. Conclusion: CFRD can significantly worse the health condition of CF patients. However, with the recent advancement of management plans of CFRD, previous sex differences in mortality have disappeared and that the gap in mortality between CF patients with diabetes and CF patients without diabetes has considerably narrowed. Early diagnosis and early intervention also helped in improving the quality of CFRD patients and preventing the complications. This can be obtained by annual screening for DM among CF patients since 10 years of age using OGTT as recommended by ADA. The primary pathologic feature of CFRD is insulin insufficiency. It leads to increased breakdown of protein and fat. So, insulin replacement is the only recommended medical treatment and it has been shown to improve clinical outcomes. Oral diabetes agents are usually not recommended in CFRD.

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