title: Effect of cholestyramine on bile acid kinetics in patients with portal cirrhosis of the liver. Evidence of a selective defect in the formation of cholic acid.
abstract: The kinetics of cholic acid (C) and chenodeoxycholic acid (CD) were studied in six patients with portal liver cirrhosis. The studies were conducted both before and after 5-6 weeks of treatment with cholestyramine (12 g/day). In keeping with previous observations, the pool size and formation of C showed subnormal values during the pretreatment period, while the production of CD was within normal limits. The pool sizes of C and CD did not change upon treatment with cholestyramine, but the mean total bile acid formation increased by a factor of about 2.5. The ratio between the amounts of C and CD synthesized remained essentially unchanged. Considering the therapeutic response previously observed in normal subjects and in patients with hyper-beta-lipoproteinemia, the present results suggest a selective impairment of the biosynthesis of C in patients with portal liver cirrhosis. It is suggested that the primary defect may reside in the 12alpha-hydroxylase enzyme system.
mesh_terms: D000368:Aged; D002635:Chenodeoxycholic Acid / Q000096:biosynthesis / Q000378:metabolism; D002792:Cholestyramine Resin / Q000627:therapeutic use*; D002793:Cholic Acids / Q000096:biosynthesis* / Q000378:metabolism; D005260:Female; D006801:Humans; D006900:Hydroxylation; D007700:Kinetics; D008099:Liver / Q000201:enzymology / Q000378:metabolism*; D008103:Liver Cirrhosis / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D006899:Mixed Function Oxygenases / Q000172:deficiency
chemical_list: D002793:Cholic Acids; D002635:Chenodeoxycholic Acid; D002792:Cholestyramine Resin; D006899:Mixed Function Oxygenases