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Cystic fibrosis-related diabetes mellitus

Kim Donaghue, MB, BS, PhD, FRACP
Paul D Robinson, MBChB, MRCPCH, FRACP, PhD
Section Editors
Joseph I Wolfsdorf, MB, BCh
George B Mallory, MD
Deputy Editor
Alison G Hoppin, MD


Cystic fibrosis-related diabetes (CFRD) is a distinct form of diabetes mellitus that is an important complication of cystic fibrosis (CF). It is different from either type 1 or type 2 diabetes mellitus, but shares features of both [1]. The primary cause is a relative insulin deficiency related to destruction of pancreatic islets. Insulin resistance also may play a role, especially in association with acute exacerbations or chronic progression of pulmonary disease.

Development of CFRD is associated with worse lung function, poorer nutritional status, and more chest infections. In addition, longitudinal studies have demonstrated decreased survival (sixfold) in individuals with CFRD as compared with non-diabetic CF patients, with females at particularly high risk in some studies [2,3]. Importantly, insulin treatment improves lung function and nutritional status [4-6]. The addition of insulin treatment and glucose monitoring, however, adds substantially to the burden of treatment, and therapeutic targets may need to be modified to maximize benefit.

Several important management guidelines have been published. These provide recommendations on screening, diagnosis, and management. Available guidelines include American Diabetes Association and Cystic Fibrosis Foundation (CFF, 2010) [7,8]; International Society for Pediatric and Adolescent Diabetes (ISPAD, 2014) [9]; Australian standards of care for CFRD (2014) [10]; and United Kingdom Cystic Fibrosis Trust (2004) [11] (see 'Society guideline links' below). Key recommendations contained within these guidelines are discussed across various parts of this monograph.

The pathophysiology, diagnosis and treatment of CFRD is discussed in this topic review. Other aspects of CF are discussed separately:

(See "Cystic fibrosis: Clinical manifestations and diagnosis".)

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Literature review current through: Nov 2017. | This topic last updated: Jun 21, 2017.
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