Cystic fibrosis-related diabetes: a distinct condition

Endocrinol Nutr. 2015 Jan;62(1):38-44. doi: 10.1016/j.endonu.2014.06.010. Epub 2014 Aug 21.
[Article in English, Spanish]

Abstract

Cystic fibrosis is the most common fatal inherited autosomal recessive disease in Caucasians, affecting approximately one out of every 2,000 births. Survival of patients with cystic fibrosis has significantly improved due to advances in respiratory and nutritional care, and their current average life expectancy is 30-40 years. Development of cystic fibrosis-related diabetes is a comorbidity that increases with age and may reach a prevalence up to 50% in adults. Its development is associated to impaired lung function and nutritional status, and early diagnosis and treatment are therefore essential to improve quality of life and performance status. Insulin therapy for diabetes and other early carbohydrate metabolism disorders may improve lung function and nutritional status of patients with cystic fibrosis.

Keywords: Cystic fibrosis; Cystic fibrosis-related diabetes; Diabetes relacionada con la fibrosis quística; Fibrosis quística; Función pulmonar; Pulmonary function.

Publication types

  • Review

MeSH terms

  • Adult
  • Comorbidity
  • Cystic Fibrosis / epidemiology*
  • Diabetes Complications / diagnosis
  • Diabetes Mellitus / diagnosis
  • Diabetes Mellitus / drug therapy*
  • Diabetes Mellitus / epidemiology*
  • Glucose Intolerance / diagnosis*
  • Glucose Tolerance Test
  • Glycated Hemoglobin / analysis
  • Humans
  • Hypoglycemic Agents / therapeutic use*
  • Insulin / therapeutic use*
  • Nutritional Status / drug effects
  • Prevalence
  • Pulmonary Ventilation / drug effects
  • Quality of Life

Substances

  • Glycated Hemoglobin A
  • Hypoglycemic Agents
  • Insulin
  • hemoglobin A1c protein, human