Cystic Fibrosis-Related Diabetes

Overview

Journal Front Endocrinol (Lausanne)

Specialty Endocrinology

Date 2018 Mar 9

PMID 29515516

Citations 38

Authors

Kayani Kayani

Raihan Mohammed

Hasan Mohiaddin

Affiliations

Soon will be listed here.

Abstract

Cystic fibrosis (CF) is the most common autosomal recessive disorder in Caucasian populations. Individuals with CF have seen significant increases in life expectancy in the last 60 years. As a result, previously rare complications are now coming to light. The most common of these is cystic fibrosis-related diabetes (CFRD), which affects 40-50% of CF adults. CFRD significantly impacts the pulmonary function and longevity of CF patients, yet a lack of consensus on the best methods to diagnose and treat CFRD remains. We begin by reviewing our understanding of the pathogenesis of CFRD, as emerging evidence shows the cystic fibrosis transmembrane conductance regulator (CFTR) also has important roles in the release of insulin and glucagon and in the protection of β cells from oxidative stress. We then discuss how current recommended methods of CFRD diagnosis are not appropriate, as continuous glucose monitoring becomes more effective, practical, and cost-effective. Finally, we evaluate emerging treatments which have narrowed the mortality gap within the CF patient group. In the future, pharmacological potentiators and correctors directly targeting CFTR show huge promise for both CFRD and the wider CF patient groups.

Citing Articles

Elevated glucose increases methicillin-resistant Staphylococcus aureus antibiotic tolerance in a cystic fibrosis airway epithelial cell infection model.

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Changes in immunofluorescence staining during islet regeneration in a cystic fibrosis-related diabetes (CFRD) ferret model.

Mohammed S, Bone R, Aquino J, Mirmira R, Evans-Molina C, Ismail H Islets. 2024; 16(1):2436696.

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Low-Dose Ketone Monoester Administration in Adults with Cystic Fibrosis: A Pilot and Feasibility Study.

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Evaluation of Oral Glucose Tolerance Test Results in Children with Cystic Fibrosis.

Bestas A, Unal E, Aktar Karakaya A, Beyazit N, Savas S, Sen V Sisli Etfal Hastan Tıp Bul. 2024; 58(3):389-394.

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Functional Consequences of CFTR Interactions in Cystic Fibrosis.

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