Pediatr Surg Int (1991) 6:309-312 Pediatric Eur gely International © Springer-Vedag 1991 Original article Fetal bile acids in congenital biliary atresia - production in the liver and clinical significance Y. Hata 1 , J. Uchino 1, E Sasaki 1, T. Abe 1, Y. Koike 1, S. Alam 1, K. Kakuya 2, S. Nagano 2, R. Mahara3, and M. Tohma 3 1First Departmentof Surgery,HokkaidoUniversitySchool of Medicine, 2Departmentof Pediatrics, HokkaidoUniversitySchool of Medicine, and 3 Facultyof PharmaceuticalSciences,Higashi-Nippon-Gakuen University,Sapporo,Japan Accepted 14 January 1991 Abstract. The l~-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 opera- tion 113,3c~,70~,12cz-tetrahydroxy-513-cholan-24-oic acid (CA-I[3-ol) and 113,3c~,Tc~-trihydroxy-513-cholan-24-oic acid (CDCA- 113-ol) were 0.46 + 34 gg/ml and 0.72-+0.45 gg/ml, respectively, which was significantly higher than in the control group. The percentage CA- 1~-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- 1[~-ol and CDCA- 113-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 secre- tion even after Kasai's procedure. These results suggest that these hydroxylases are reactivated in CBA patients. Key words: Congenital biliary atresia - l[3-hydroxy bile acids - Fetal bile acids Introduction The presence of several unusual bile acids in the serum of congenital biliary atresia (CBA) patients has been reported, but their significance has not yet been completely clarified. Large amounts of 1[~-hydroxy bile acids have recently been detected in the meconium and bile of neonates, but none can be found in healthy adults. Therefore, these substances have been termed fetal bile acids [5]. Offprint requests to: Y. Hata, First Department of Surgery, Hokkaido University School of Medicine, N-15 W-7, S apporo, Japan In this study the fetal bile acids were measured in patients with CBA; the production of these bile acids in the livers of CBA patients and their clinical significance were evaluated. Patients and methods The experimentalgroup consisted of eight CBA patients (52-143 days old, mean 98.1+33.7) with porta hepatis obstruction. Five patients without liver disease, three inguinal hernias, one umbilical hernia, and one anterioranus 30-78 days old, mean 46.2+-- 19.5) were chosen as the control group. The serum of these patients was collected and frozen at -20 °C. Four l[~-hydroxy bile acids: 1 ~,30~-dihydroxy-513-cholan-24-oic acid (LCA-I~3-ol); l[3,3c~,12c~-trihydroxy-5~-cholan-24-oic acid (DCA-I~- ol); l[~,3c~,Tcq12(z-tetrahydroxy-5~-cholan-24-oic acid (CA-I~-ol); and l~,3cz,7~z-trihydroxy-5~-cholan-24-oic acid (CDCA-I~-ol) were mea- sured using gas chromatography-massspectrometry [9]. These l~-hy- droxy serum bile acids were evaluated on the basis of several factors, e.g., age of the patients at operation, histological grade of hepatic fibrosis accordingto the classificationof Suruga et al. [8], and status of bile flow following Kasai's operation. Differences between values (mean _+ standard deviation) were tested for significance using Stu- dent's t-test. Results Of the four ll~-hydroxy bile acids, DCA-I[3-ol and LCA- 113-ol were not detected in any patient and were accord- ingly eliminated from our study. The serum level of CA-I~3-ol was 0.46+0.34 btg/ml, the percentage being 0.72_+0.45% of total bile acids, in CBA patients, and was 0.04 _+0.03 gg/ml, 1.60 _+ 1.48% of total bile acids, in the control group. The serum level of CDCA- 1~3-ol was 0.72 _+0.45 btg/ml, 1.13 _+0.56% of total bile acids, in CBA patients and 0.05+0.04gg/ml, 2.06_+ 1.88% of total bile acids, in the control group (Table 1). The levels of 113-hydroxy bile acids were signif- icantly elevated in CBA patients compared with the control group, but there was no significant difference in percentage of total bile acids between the two groups (Fig. 1).