Cystic fibrosis-related diabetes

Cystic fibrosis-related diabetes (CFRD) is diabetes specifically caused by cystic fibrosis, a genetic condition. Cystic fibrosis related diabetes mellitus (CFRD) develops with age, and the median age at diagnosis is 21 years.[1]

For general diabetes discussion, see Diabetes.
Cystic fibrosis-related diabetes
SpecialtyEndocrinology

Presentation

CFRD shares features of both type 1 and type 2 diabetes. CFRD patients are typically young and are not obese, and lack metabolic syndrome features. On the other hand, the cause is not autoimmune, some insulin resistance is present, and ketosis is rare.[2]

Pathophysiology

The endocrine pancreatic function deterioration appears to be secondary to chronic pancreatitis and subsequent scarring associated with CF.[2]

Epidemiology

CFRD occurs in some 20% of adolescents and 40–50% of adults affected by CF. Though rare in children, it has been described in CF patients of all ages, including infants. Beginning in the teenage years, CFRD has an annual incidence of ~3%, and may be more common in females. It is associated with more severe CF gene mutation types.[2]

As survival of CF patients has steadily increased in past decades, CFRD is an increasingly common – and currently the most common – complication of CF.[3]

See also

References
  1. Lek N, Acerini CL (January 2010). "Cystic fibrosis related diabetes mellitus - diagnostic and management challenges". Current Diabetes Reviews. 6 (1): 9–16. doi:10.2174/157339910790442600. PMID 20034372.
  2. Moran A, Becker D, Casella SJ, Gottlieb PA, Kirkman MS, Marshall BC, Slovis B (December 2010). "Epidemiology, pathophysiology, and prognostic implications of cystic fibrosis-related diabetes: a technical review". Diabetes Care. 33 (12): 2677–83. doi:10.2337/dc10-1279. PMC 2992212. PMID 21115770.
  3. Kayani K, Mohammed R, Mohiaddin H (2018-02-20). "Cystic Fibrosis-Related Diabetes". Frontiers in Endocrinology. 9: 20. doi:10.3389/fendo.2018.00020. PMC 5826202. PMID 29515516.
Classification
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