LIPID-LEVELS IN ADULTS WITH CYSTIC-FIBROSIS

Objective To examine the hypothesis that an energy-dense, high-fat diet, which is necessary to maintain weight in adults with cystic fibrosis, does not result in high serum cholesterol levels. Design Dietary, anthropometric, and biochemical data were correlated. Setting A cystic fibrosis center in Pittsburgh, Pa. Subjects Thirty-one adults with cystic fibrosis, 50 obligate carriers of the cystic fibrosis gene, and 26 controls who did not have cystic fibrosis. Main outcome measures Adults with cystic fibrosis had a lower mean serum cholesterol level and higher mean intakes of energy and fat than controls. Statistical analyses performed Student's t test was used to determine the statistical significance between two means. Univariate correlation coefficients were determined to measure the relative intensity of association between two variables. Results Mean total serum cholesterol levels in men with cystic fibrosis was 3.1 mmol/L vs 4.7 mo\L in male controls (P<.001). Mean total serum cholesterol levels in women with cystic fibrosis was 3.2 mmol/L vs 4.3 mmol/L in female controls (P<.001). Three adults with cystic fibrosis and no signs of pancreatic insufficiency had serum cholesterol levels in the high normal range. Carriers had serum lipid levels in the same range as the controls. Conclusions/applications The findings indicate that a high-energy, high-fat diet does not raise serum lipid levels in those patients with cystic fibrosis and pancreatic insufficiency. However, those individuals with cystic fibrosis and normal pancreatic function may be at the same risk as the general population for developing high serum lipid levels. They should have their serum lipid levels monitored and be given appropriate dietary recommendations.