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Cystic fibrosis-related diabetes in children—gaps in the evidence?

Nature Reviews Endocrinology volume 6, pages 371–378 (2010)Cite this article

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Abstract

As the life span of patients with cystic fibrosis has increased, so has the prevalence of cystic fibrosis-related diabetes mellitus. However, screening practices for cystic fibrosis-related diabetes mellitus vary widely, which affects accurate estimates of the health burden of this comorbidity. The management of prediabetes and hyperglycemia is an increasingly important aspect of care in patients with cystic fibrosis, but few studies have specifically addressed the management of cystic fibrosis-related diabetes mellitus. Previous studies support the use of insulin for the treatment of patients with this disorder, but the evidence for its use in patients with cystic fibrosis and impaired glucose tolerance is poor. Nutritional management is currently guided by dietary recommendations for individuals with cystic fibrosis, with little evidence specific to the dietary management of patients with cystic fibrosis-related diabetes mellitus. Additionally, microvascular complications have become more frequent as a result of the rise in life expectancy of these patients, yet to date no intervention studies have addressed prevention or management of diabetic complications in patients with cystic fibrosis. A strong evidence base is needed to guide the management of patients with cystic fibrosis-related diabetes mellitus and its complications.

Key Points

  • The prevalence of cystic fibrosis-related diabetes mellitus has risen, as life expectancy of patients with cystic fibrosis has increased

  • The diagnosis of cystic fibrosis-related diabetes mellitus is currently made on the basis of consensus guidelines, and diagnostic criteria do not address cystic fibrosis-specific outcomes

  • Microvascular complications are becoming more prevalent in patients with cystic fibrosis, but evidence for their prevention and management is minimal

  • Strong evidence supports the use of insulin therapy in patients with cystic fibrosis-related diabetes mellitus; however, its use in those with impaired glucose tolerance is controversial

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Figure 1: Pathophysiology of cystic fibrosis-related diabetes mellitus.

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Acknowledgements

Charles P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. Department of Endocrinology and Diabetes, The Children's Hospital at Westmead, Locked Bag 4001, Westmead, 2145, NSW, Australia

    Malay Rana, Craig F. Munns, Kim C. Donaghue & Maria E. Craig

  2. Department of Respiratory Medicine, The Children's Hospital at Westmead, Locked Bag 4001, Westmead, 2145, NSW, Australia

    Hiran Selvadurai

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Correspondence to Maria E. Craig.

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Rana, M., Munns, C., Selvadurai, H. et al. Cystic fibrosis-related diabetes in children—gaps in the evidence?. Nat Rev Endocrinol 6, 371–378 (2010). https://doi.org/10.1038/nrendo.2010.85

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