Bile acids and bile alcohols in a child with hepatic 3 beta-hydroxy-delta 5-C27-steroid dehydrogenase deficiency: effects of chenodeoxycholic acid treatment
H Ichimiya, B Egestad, H Nazer, ES Baginski… - Journal of lipid …, 1991 - ASBMB
Duodenal bile, urine, plasma, and feces from a child with hepatic 3 beta-hydroxy-delta 5-
C27-steroid dehydrogenase deficiency were analyzed by fast atom bombardment mass
spectrometry and gas chromatography-mass spectrometry to investigate the formation and
excretion of abnormal bile acids and bile alcohols. The biliary bile salts consisted of
glycocholic acid (25%) and of sulfated and glycine conjugated di-and trihydroxycholenoic
acids (55%), two C27 bile acids, and eleven sulfated bile alcohols (mainly tetrols, 20%), all …
C27-steroid dehydrogenase deficiency were analyzed by fast atom bombardment mass
spectrometry and gas chromatography-mass spectrometry to investigate the formation and
excretion of abnormal bile acids and bile alcohols. The biliary bile salts consisted of
glycocholic acid (25%) and of sulfated and glycine conjugated di-and trihydroxycholenoic
acids (55%), two C27 bile acids, and eleven sulfated bile alcohols (mainly tetrols, 20%), all …