Biliary Lipid Composition in Patients with Cystic Fibrosis

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Abstract
Lipid composition of gallbladder bile was determined in 20 patients with cystic fibrosis (CF) (9 females and 11 males, ranging in age from 3 to 18 years). The results were compared with 47 normal subjects matched for age, sex, and pubertal stage. In patients with CF, bile was undersaturated with cholesterol before puberty in both sexes and no differences with normal controls could be observed. After puberty, a similar increase in cholesterol saturation was noted in females with CF (85 .+-. 15% vs. 130 .+-. 38%, p < 0.01) and normal control (82 .+-. 11% vs. 138 .+-. 31%, p < 0.01). No change in cholesterol saturation could be observed in male patients and controls after puberty. Molar percentage of chenodeoxycholic acid (CDCA) was lower (p < 0.05) in postpubertal females (31 .+-. 9%) and males (36 .+-. 7%) with CF compared to controls (42 .+-. 8% and 40 .+-. 5%, respectively), while cholic acid (CA) was higher in all patients with CF. In females with CF, lithocholic acid (LCA) increased after puberty (2.2 .+-. 0.8% vs. 5.3 .+-. 2.6%, p < 0.05) and was higher compared to controls (2.2 .+-. 0.8%, p < 0.001). An increase was also noted for deoxycholic acid (DCA) in postpubertal females with CF (1.7 .+-. 2.6% vs. 10.8 .+-. 7%, p < 0.05), but it was lower in both sexes after puberty than in respective controls. The present results suggest that cholesterol saturation of bile in patients with CF is not different from respective controls. Thus, the higher incidence of gallstone disease in patients with CF reported in the literature might be related to other hepatobiliary disorders known in this disease.