Genetic modifiers play a substantial role in diabetes complicating cystic fibrosis.

The Journal of Clinical Endocrinology and Metabolism
Scott M BlackmanGarry R Cutting

Abstract

Insulin-requiring diabetes affects 7-15% of teens and young adults, and more than 25% of older adults with cystic fibrosis (CF). Pancreatic exocrine disease caused by CF transmembrane conductance regulator (CFTR) dysfunction underlies the high rate of diabetes in CF patients; however, only a subset develops this complication, indicating that other factors are necessary. Our objective was to estimate the relative contribution of genetic and nongenetic modifiers to the development of diabetes in CF. This was a twin and sibling study involving 1366 individuals at 109 centers in the CF Twin and Sibling Study, from which were derived 68 monozygous twin pairs, 23 dizygous twin pairs, and 588 sibling pairs, all with CF. Chronic, insulin-requiring diabetes in the setting of CF, as established using longitudinal clinical and biochemical data, was studied. About 9% of this predominantly pediatric population (mean age = 15.8 yr) had diabetes. Key independent risk factors identified by regression modeling included having a twin or sibling with CF and diabetes, increasing age, pancreatic exocrine insufficiency or two mutations causing severe CFTR dysfunction, decreased lung function or decreased body mass index, and longer duration of gluco...Continue Reading

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