Abstract
The 1β-hydroxy bile acids have been said to be fetal bile acids. These bile acids were evaluated in eight patients with congenital biliary atresia (CBA) using gas chromatography-mass spectrometry. At the time of operation 1β,3α,7α,12α-tetrahydroxy-5β-cholan-24-oic acid (CA-1β-ol) and 1β,3α,7α-trihydroxy-5β-cholan-24-oic acid (CDCA-1β-ol) were 0.46 ± 34 μg/ml and 0.72 ± 0.45 μg/ml, respectively, which was significantly higher than in the control group. The percentage CA-1β-ol of total bile acids showed a tendency to decrease as age advanced. The grade of hepatic fibrosis ranged from F2 to F3 and the values and percentages of CA-1β-ol and CDCA-1β-ol were relatively higher in F2 than F3 patients. The percentage of total bile acids gradually increased in patients without sufficient bile flow but fell sharply after Kasai's procedure in the patients with sufficient bile flow. It appears that fetal bile acids are produced in the livers of CBA patients in the same way as in fetal liver, and that production continues in patients without good bile secretion even after Kasai's procedure. These results suggest that these hydroxylases are reactivated in CBA patients.
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Hata, Y., Uchino, J., Sasaki, F. et al. Fetal bile acids in congenital biliary atresia —production in the liver and clinical significance. Pediatr Surg Int 6, 309–312 (1991). https://doi.org/10.1007/BF00178645
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DOI: https://doi.org/10.1007/BF00178645