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Review
. 2021 Feb 16;30(159):200293.
doi: 10.1183/16000617.0293-2020. Print 2021 Mar 31.

Cystic fibrosis-related diabetes and lung disease: an update

Bernadette J Prentice  1   2 , Adam Jaffe  3   2 , Shihab Hameed  2   4   5 , Charles F Verge  2   5 , Shafagh Waters  2   6 , John Widger  3   2
Affiliations
Review

Cystic fibrosis-related diabetes and lung disease: an update

Bernadette J Prentice et al. Eur Respir Rev. .

Abstract

The development of cystic fibrosis-related diabetes (CFRD) often leads to poorer outcomes in patients with cystic fibrosis including increases in pulmonary exacerbations, poorer lung function and early mortality. This review highlights the many factors contributing to the clinical decline seen in patients diagnosed with CFRD, highlighting the important role of nutrition, the direct effect of hyperglycaemia on the lungs, the immunomodulatory effects of high glucose levels and the potential role of genetic modifiers in CFRD.

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Conflict of interest statement

Conflict of interest: B.J. Prentice reports other funding from Vertex/TSANZ fellowship, NHMRC postgraduate scholarship, and the Australian Government Research Training Program scholarship, during the conduct of the study. Conflict of interest: A. Jaffe has nothing to disclose. Conflict of interest: S. Hameed reports grants from National Health and Medical Research Council of Australia, Australasian Cystic Fibrosis Research Trust, Regional Diabetes Support Scheme, Sydney Children's Hospital Foundation, and Australasian Pediatric Endocrine Care Grant from Pfizer, and non-financial support from Novo Nordisk, Medtronic and Abbott Diagnostics, during the conduct of the study. Conflict of interest: C.F. Verge reports grants from National Health and Medical Research Council of Australia, Australasian Cystic Fibrosis Research Trust, Regional Diabetes Support Scheme, Sydney Children's Hospital Foundation and Australasian Pediatric Endocrine Care Grant from Pfizer, and non-financial support from Novo Nordisk, Medtronic and Abbott Diagnostics, during the conduct of the study. Conflict of interest: S. Waters has nothing to disclose. Conflict of interest: J. Widger has nothing to disclose.

Figures

FIGURE 1
FIGURE 1
Schematic diagram of airway surface liquid (ASL) glucose homeostasis. a) In health, ASL has a tightly regulated glucose gradient in which minimal glucose (black triangles) is present. ASL hydration is supported by the CFTR protein which is situated on the apical plasma membrane and transports Cl− and HCO3− ions into the ASL. ASL glucose is transported to the site of intracellular glucose metabolism by GLUT transporters and there is minimal passive diffusion across tight paracellular junctions. b) In the setting of CFRD, there are changes in ASL with reduced and inflamed ASL, with an increased number of bacteria seen (crossed circles). Paracellular junctions leak glucose into the ASL. Despite initial upregulation of apical GLUT transporters, there is net increase in movement of glucose towards the ASL and an increased level of glucose is seen in the ASL. CFRD: cystic fibrosis-related diabetes; CFTR: cystic fibrosis transmembrane conductance regulator; GLUT: glucose transporter.
FIGURE 2
FIGURE 2
Graphical representation of potential mechanisms involved in the loss of lung function associated with the development of cystic fibrosis-related diabetes (CFRD). The lung function effects of CFRD are likely to be multifactorial with some mechanisms having greater influence in certain patients.
See this image and copyright information in PMC

References

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