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Klin Wochenschr. 1980 Jan 15;58(2):55-63. doi: 10.1007/BF01477189.
The metabolism of bile acids in man is disturbed under the conditions of cholestasis. Besides of the main bile acids atypical bile acids can be found, which are mainly eliminated by renal excretion as sulphate esters and glucuronides. The pattern of urinary bile acids up to now renders no conclusions with respect to the underlying disease, although intrahepatic cholestasis seems to be in some way connected with disturbances in the metabolism of 3 beta-hydroxy-5-cholenoic acid, a bile acid, which exerts cholestatic effects by itself. The metabolites to be found seem to reflect a derepression of a genotypical synthesis program, which is not phenotypically apparent in healthy adults, but which may have been active during prenatal developmental stages of the liver.
在胆汁淤积的情况下，人体胆汁酸的代谢会受到干扰。除了主要的胆汁酸外，还能发现非典型胆汁酸，它们主要以硫酸酯和葡糖醛酸苷的形式通过肾脏排泄而被清除。尽管肝内胆汁淤积似乎在某种程度上与一种胆汁酸——3β-羟基-5-胆烯酸的代谢紊乱有关，该胆汁酸自身具有致胆汁淤积作用，但迄今为止，尿胆汁酸的模式并不能为潜在疾病提供诊断依据。所发现的代谢产物似乎反映了一种基因型合成程序的去抑制，这种程序在健康成年人中没有表型上的体现，但可能在肝脏产前发育阶段是活跃的。
