Endocrine and exocrine pancreatic function and the ΔF508 mutation in cystic fibrosis

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Abstract
The relationship between the cystic fibrosis (CF) genotype and endocrine and exocrine pancreatic function was studied in 215 CF patients. In the 211 patients with the ΔF508 mutation, endocrine pancreatic function (oral glucose tolerance; WHO criteria) was normal in 72.5%, impaired in 12.3%, and diabetic in 15.2% of the patients, with no difference between CF patients homozygous (N=163, median age 15 years, range 2–40) or heterozygous (N=48, 18 years, 3–40; age difference not significant) for the ΔF508 mutation. Exocrine pancreatic sufficiency (no need for pancreatic enzyme substitution) was found in 0.6% of the patients homozygous for the ΔF508 mutation and in 10.4% of the heterozygotes (pΔF508 mutation had normal glucose tolerance but exocrine pancreatic insufficiency. In conclusion, the major mutation genotype in CF (ΔF508) affects the severity of the exocrine pancreatic insufficiency, whereas endocrine pancreatic function is unrelated to this genotype.