Serum bile acid composition in patients with cystic fibrosis

Abstract

Serum bile acid composition was examined in detail using capillary column gas chromatography and mass spectrometry in 10 children with cystic fibrosis (CF) and 4 healthy children. The mean total bile acid concentrátion in fasting serum of CF patients was 2.33 ± 0.84 / gmmol/l, slightly lower than but not statistically significantly different from healthy controls (mean 2.86 ± 0.98 μmol/l) and appeared to show no relationship to the degree of exocrine pancreatic insufficiency. Analysis of individual serum bile acids in these children showed that cholic acid represented less than 10% of the total bile acids. Chenodeoxycholic acid was the predominant serum bile acid; the mean concentration in CF patients was 0.98 ± 0.51 / gmmol/l, lower than for the healthy controls (1.69 ± 0.84 μmol/l). Concentrations of lithocholic acid, 3/gb-hydroxy-5-cholenoic, ursodeoxycholic and 3/gb,7α,12α-trihydroxy-5/gb-cholanoic acids in fasting serum samples of the CF patients were not significantly different from the healthy control sera but were higher than those normally found in adults. Measurements of fecal bile acid excretion indicated an increased loss of primary bile acids in patients with CF consistent with an impairment of the enterohepatic circulation of bile acids.