Abstract
The bile alcohol glucuronides in urine of 12 patients with primary biliary cirrhosis (PBC), 10 patients with chronic active hepatitis (CAH), and 6 healthy volunteers were analyzed by capillary gas-liquid chromatography-mass spectrometry. In all subjects studied, the major urinary bile alcohol was found to be 27-nor-5 beta-cholestane-3 alpha,7 alpha,12 alpha,24,25-pentol (C26 pentol). In PBC patients, the excretion of C26 pentol (main isomer) was significantly increased above values observed in healthy volunteers (mean +/- SD = 5.2 +/- 3.5 mumol/24 h, range 1.0-13.4; versus 0.6 +/- 0.3, range 0.4-1.0). In addition, PBC patients excreted increased amounts of other bile alcohols such as isomers of C26 pentol, pentahydroxylated C27 bile alcohols (5 beta-cholestane-3 alpha,7 alpha,12 alpha,24,25-pentol) and 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25,26-pentol) and a hexahydroxylated C26 bile alcohol (27-nor-5 beta-cholestane-3 alpha,7 alpha,12 alpha,24,25,26-hexol). In CAH patients, the excretion of the C26 pentol main isomer ranged from 0.3 to 2.0 mumol/24 h (mean +/- SD = 0.7 +/- 0.5) and did not significantly differ from that in healthy volunteers. Moreover, the bile alcohol profile was comparable to those found in healthy volunteers and PBC patients. These findings show that total urinary bile alcohol glucuronide excretion is significantly increased in primary biliary cirrhosis. A PBC-specific urinary bile alcohol profile, however, does not exist.
Highlights
IntroductionIncreased urinary excretion of bile alcohol glucuronides in patients with primary biliary cirrhosis
To investigate to what extent the bile alcohol profile is specific for primary biliary cirrhosis (PBC), urinary bile alcohol glucuronide excretion was determined in 10 patients with chronic active hepatitis (CAH) and in 6 healthy volunteers
Other compounds in the profile of patients with PBC were pentahydroxylated C27 bile alcohols with two hydroxyl groups in the side chain at C-24,25 (CZ7-24,25-pentol)or C-25,26 (C2,25,26-pentol) and a hexahydroxylated c 2 6 bile alcohol, named c 2 6 hexol
Summary
Increased urinary excretion of bile alcohol glucuronides in patients with primary biliary cirrhosis. It was demonstrated in children that bile alcohols may be excreted as glucuronide conjugates in urine; a glucuronide of the major bile alcohol 27-nor-5/3-cholestane3a,7a,12a, 24,25-pentol was excreted by healthy children and in increased amounts by children with al-antitrypsin deficiency and subsequent cirrhosis [3, 4] This bile alcohol glucuronide was found in urine of a child with neonatal cholestasis and cirrhosis [5] and an adult patient with cirrhosis and Wilson's disease [6]. To investigate to what extent the bile alcohol profile is specific for PBC, urinary bile alcohol glucuronide excretion was determined in 10 patients with chronic active hepatitis (CAH) and in 6 healthy volunteers
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