Progressive impairment of insulin secretion in cystic fibrosis

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With the increased life expectancy of people affected by cystic fibrosis (CF), impaired glucose tolerance (IGT) and diabetes mellitus (CFRD) have emerged as significant clinical problems. In this issue of The Journal, Bismuth et al report their longitudinal study of glucose tolerance, insulin secretion, morbidity, and mortality in 237 French children with CF. They observed very high rates of IGT and CFRD – 82% and 70%, respectively, at 30 years. Impaired tolerance was associated with increased mortality, worse pulmonary function, decreased height, and impaired nutrition. Although earlier studies have reported insulin resistance in CF, it did not seem to play a major role in the current study where impaired insulin secretion appeared to be the major abnormality.