Reduction of urinary bile alcohol excretion and serum cholestanol in patients with cerebrotendinous xanthomatosis after oral administration of deoxycholic acid

Clin Chim Acta. 1990 Dec 14;193(3):113-8. doi: 10.1016/0009-8981(90)90242-k.

Abstract

Deoxycholic acid and chenodeoxycholic acid were administered alternately to four patients with cerebrotendinous xanthomatosis. During this oral therapy serum cholestanol and urinary bile alcohols were determined. Both showed a marked decrease after the start of the two different therapies. It can be concluded that not only chenodeoxycholic acid but also deoxycholic acid is able to suppress endogenous human bile acid synthesis, which is in accordance with other experiments describing the effect of feeding of various bile acids on endogenous bile acid synthesis.

MeSH terms

  • Adult
  • Bile Acids and Salts / biosynthesis
  • Cholestanol / blood*
  • Cholestanols / urine*
  • Deoxycholic Acid / therapeutic use*
  • Female
  • Humans
  • Lipid Metabolism, Inborn Errors / drug therapy*
  • Lipid Metabolism, Inborn Errors / metabolism
  • Male
  • Middle Aged
  • Xanthomatosis / drug therapy*
  • Xanthomatosis / metabolism

Substances

  • Bile Acids and Salts
  • Cholestanols
  • Deoxycholic Acid
  • Cholestanol