Two brothers with benign recurrent
intrahepatic cholestasis were studied over a period of 6 years. During this period, 11 episodes of
cholestasis were observed, with a mean duration of 2.6 months (range: 2 weeks to 6 months). Once, both brothers developed
cholestasis simultaneously. There was a prevalence for episodes of
cholestasis in wintertime. The postprandial rise in serum sulfated
glycolithocholic acid was increased in the patients, and the
bile acid pool was enriched with secondary
bile acids. In periods prior to
cholestasis, the urinary 3 alpha
OH-
bile acid concentration was often elevated (greater than 50 mumoles per liter) without a clear correlation with the clinical prodromata. However, it could not be used as a predictor of
cholestasis. In contrast, the postprandial rise in serum 3 alpha
OH-
bile acids was always grossly elevated in periods just before
cholestasis. An increase both in fecal
bile acid excretion as well as secondary
bile acids in the
bile acid pool indicated an increased spillover of
bile acids into the large bowel.
Cholestyramine administered directly after the first signs of
cholestasis appeared to shorten an episode of
cholestasis. On the other hand, withdrawal of
cholestyramine in a
cholestasis-free period may have resulted in an episode of
cholestasis. Neither
taurine supplementation for 3 and 7 weeks nor
calcium phosphate, which binds sulfated
bile acids in vitro, for 3 weeks could prevent an episode of
cholestasis, although the latter normalized the
bile acid pool composition. There is a rationale for a
fat-restricted diet and
cholestyramine therapy only as maintenance treatment.(ABSTRACT TRUNCATED AT 250 WORDS)