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Abnormal urinary bile acids in a patient suffering from cerebrotendinous xanthomatosis during oral administration of ursodeoxycholic acid.

Abstract
The urinary bile acid profile, obtained by capillary gas chromatography, of a patient suffering from cerebrotendinous xanthomatosis and treated with ursodeoxycholic acid demonstrated, besides the occurrence of 23-norcholic acid and (23R)-hydroxycholic acid (as a consequence of this disease), six additional unknown bile acids and three known bile acids, viz. ursodeoxycholic acid, hyocholic acid and omega-muricholic acid. The structure of two of the unknown bile acids were elucidated and proven by organic syntheses. These were 23-norursodeoxycholic acid and 3 beta-ursodeoxycholic acid. The structures of three bile acids were tentatively elucidated as being 1 beta-hydroxyursodeoxycholic acid, 21-hydroxyursodeoxycholic acid and 22-hydroxyursodeoxycholic acid, and the possibility that the structure of the remaining bile acid is that of 5-hydroxyursodeoxycholic acid is discussed. Two of these bile acids (1 beta-hydroxyursodeoxycholic acid and 5-hydroxyursodeoxycholic acid) also occurred in urine of a healthy individual during oral ursodeoxycholic acid treatment, whereas 23-norcholic acid, 23-norursodeoxycholic acid, (23R)-hydroxycholic acid, 21-hydroxyursodeoxycholic acid and 22-hydroxyursodeoxycholic acid were only present in urine of the patient suffering from cerebrotendinous xanthomatosis. The metabolism of ursodeoxycholic acid, both in the normal state and in the cerebrotendinous xanthomatosis, is discussed.
AuthorsB J Koopman, B G Wolthers, J C van der Molen, G T Nagel, W Kruizinga
JournalBiochimica et biophysica acta (Biochim Biophys Acta) Vol. 917 Issue 2 Pg. 238-46 (Feb 14 1987) ISSN: 0006-3002 [Print] Netherlands
PMID3801500 (Publication Type: Journal Article)
Chemical References
  • Bile Acids and Salts
  • Deoxycholic Acid
  • Ursodeoxycholic Acid
Topics
  • Adult
  • Bile Acids and Salts (urine)
  • Brain Diseases, Metabolic (drug therapy, urine)
  • Deoxycholic Acid (analogs & derivatives)
  • Female
  • Gas Chromatography-Mass Spectrometry
  • Humans
  • Middle Aged
  • Ursodeoxycholic Acid (therapeutic use)
  • Xanthomatosis (drug therapy, urine)

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