ANALYSIS OF THE BILE ACID PROFILES AND APPLICATION TO THE CONGENITAL LIVER DISORDER

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The establishment of the diagnosis of congenital liver disorder is urgently required for the remedy of liver disorder in early life. The aim of this research is the development of quantitative analysis of fetal bile acids by GC-MS and immunological assay, elucidation of the change of bile acid profiles with growth, and establishment of the precise diagnosis and masscreening of congenital biliary atresia.(1) The stereoisomeric C_<27>-bile acids and bile alcohols were chemically synthesized for the authentic specimens. The newly developed analysis of these intermediates was achieved by capillary gas chromatography.(2) Highly sensitive radioimmunoassay and enzyme immunoassay were developed. By the established method, the change of conjugated fetal bile acids in urine of pregnant women and newborns was examined.(3) The bile acid composition of neonatal periods changed with growth. In the case of preterm infants, the similar results were observed. The result of comparison of the ratio of 1beta-hydroxycholic acid to cholic acid indicated the activity of 1beta-hydroxylase decreased with growth.(4) 1. Diagnostic analysis of congenital biliary atresia was accomplished by enzyme immunoassay. The possibility of masscreening using dried blood spots was also examined. 2. From the urine of Zellweger syndrome, THCA and varanic acid were identified and determined by GLC. 3. In addition to 1beta-hydroxycholic acid, 1beta-hydroxylated C_<23>- and C_<22>-bile acids were found in the urine of patients with cerebrotendinous xantomatosis.