LIPID-LEVELS IN ADULTS WITH CYSTIC-FIBROSIS

Objective To examine the hypothesis that an energy-dense, high-fat die t, which is necessary to maintain weight in adults with cystic fibrosi s, does not result in high serum cholesterol levels. Design Dietary, a nthropometric, and biochemical data were correlated. Setting A cystic fibrosis center in Pittsburgh, Pa. Subjects Thirty-one adults with cys tic fibrosis, 50 obligate carriers of the cystic fibrosis gene, and 26 controls who did not have cystic fibrosis. Main outcome measures Adul ts with cystic fibrosis had a lower mean serum cholesterol level and h igher mean intakes of energy and fat than controls. Statistical analys es performed Student's t test was used to determine the statistical si gnificance between two means. Univariate correlation coefficients were determined to measure the relative intensity of association between t wo 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 wit h cystic fibrosis and no signs of pancreatic insufficiency had serum c holesterol levels in the high normal range. Carriers had serum lipid l evels in the same range as the controls. Conclusions/applications The findings indicate that a high-energy, high-fat diet does not raise ser um lipid levels in those patients with cystic fibrosis and pancreatic insufficiency. However, those individuals with cystic fibrosis and nor mal pancreatic function may be at the same risk as the general populat ion for developing high serum lipid levels. They should have their ser um lipid levels monitored and be given appropriate dietary recommendat ions.