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 mere preoperatively determined by microanalysis using gas chromatography-mass spectrometry with negative ion chemical ionization detection. The hepatic content of total bile acids mas 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) Tvas observed in the liver tissue of these patients, although only trace amounts mere detected in their sera. The ratio of glycine-conjugated to taurine-conjugated bile acids mas 0.44+/-0.18 in liver tissue and 0.79+/-0.52 in serum and these values mere 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.