Hepatic and serum bile acid compositions in patients with biliary atresia: a microanalysis using gas chromatography-mass spectrometry with negative ion chemical ionization detection.

Hepatic and serum bile acids in five patients with biliary atresia were preoperatively determined by microanalysis using gas chromatography-mass spectrometry with negative ion chemical ionization detection. The hepatic content of total bile acids was markedly elevated (3079+/-711 nmol/g protein), most of which were primary bile acids. Accumulation of unconjugated bile acids (2.93% to 4.62% of the total) was observed in the liver tissue of these patients, although only trace amounts were detected in their sera. The ratio of glycine-conjugated to taurine-conjugated bile acids was 0.44+/-0.18 in liver tissue and 0.79+/-0.52 in serum and these values were significantly lower than those of controls. This study has shown that the composition of bile acids in serum does not reflect that in liver tissue faithfully. The accumulation of these hydrophobic bile acids may contribute to initiating or exacerbating liver injury in infants with cholestatic liver diseases.