Cystic Fibrosis–Related Diabetes: Pathophysiology and Therapeutic Challenges

Ryan Kelsey, Fiona N. Manderson Koivula, Neville H. McClenaghan, Catriona Kelly

Research output: Contribution to journalReview articlepeer-review

35 Citations (Scopus)

Abstract

Cystic fibrosis–related diabetes (CFRD) is among the most common extrapulmonary co-morbidity associated with cystic fibrosis (CF), affecting an estimated 50% of adults with the condition. Cystic fibrosis is prevalent in 1 in every 2500 Caucasian live births and is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Mutated CFTR leads to dehydrated epithelial surfaces and a build-up of mucus in a variety of tissues including the lungs and pancreas. The leading cause of mortality in CF is repeated respiratory bacterial infections, which prompts a decline in lung function. Co-morbid diabetes promotes bacterial colonisation of the airways and exacerbates the deterioration in respiratory health. Cystic fibrosis–related diabetes is associated with a 6-fold higher mortality rate compared with those with CF alone. The management of CFRD adds a further burden for the patient and creates new therapeutic challenges for the clinical team. Several proposed hypotheses on how CFRD develops have emerged, including exocrine-driven fibrosis and destruction of the entire pancreas and contrasting theories on the direct or indirect impact of CFTR mutation on islet function. The current review outlines recent data on the impact of CFTR on endocrine pancreatic function and discusses the use of conventional diabetic therapies and new CFTR-correcting drugs on the treatment of CFRD.

Original languageEnglish
JournalClinical Medicine Insights: Endocrinology and Diabetes
Volume12
DOIs
Publication statusPublished - 2019
Externally publishedYes

Keywords

  • CFTR
  • cystic fibrosis
  • cystic fibrosis–related diabetes
  • exocrine pancreas
  • islet

Fingerprint

Dive into the research topics of 'Cystic Fibrosis–Related Diabetes: Pathophysiology and Therapeutic Challenges'. Together they form a unique fingerprint.

Cite this