Biliary and urinary
bile alcohol and
bile acid composition has been determined by high performance liquid chromatography in patients with
cerebrotendinous xanthomatosis before and
after treatment with
chenodeoxycholic acid. Most of the
bile acids and
bile alcohols in the bile and urine were separated in less than 30 min using a radial pack C18 muBondapak 5 micron particle size column with a mobile phase of
acetonitrile-water-
methanol-
acetic acid 70:70:20:1 (v/v/v/v) at a flow rate of 2 ml/min, and a refractive index detector. Before treatment,
cholic acid (49%) and 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 25-tetrol (27%) were the major biliary
bile acid and
bile alcohol, respectively, but were not detected in the urine of five patients. 5 beta-Cholestane-pentols were, instead, the major urinary
bile alcohols with 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 23 xi, 25-pentol (56%) predominating. Whereas 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 24S,25-pentol was not detected in the bile, it was isolated in the urine of all patients (27%). The only urinary
bile acid isolated by high performance liquid chromatography was nor-
cholic acid. After 1 month of treatment with
chenodeoxycholic acid, 0.75 g/day,
chenodeoxycholic acid became the major
bile acid in the bile of all patients (71%) along with its metabolite,
ursodeoxycholic acid (21%).
Cholic acid and 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 25-tetrol were drastically reduced and were only 3% each. The excretion of 5 beta-cholestane-pentols in the urine was also drastically reduced from 130 mg/day to 15 mg/day.