PROFILE OF FETAL BILE ACID IN FETUS AND NEONATE,AND DIAGNOSIS OF NEONATAL HEPATOBILIALY DISEASES

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The metabolism of bile acids in fetus and neonate is thought to be different from that in adults. A highly sensitive analytical metod of bile acids by GC-MS has been required to elucidate the composition of fetal bile acids in serum for the diagnosis of neonatal hepatobiliary diseases.(1) Symthesis of 4beta- and 6alpha-hydroxy bile acids were efficiently carried out for the identification of these acid in urine and serum from neonates by the comparison with the standards.(2) A higly sensitive GC-MS determination of the fetal bile acid in neonatal serum and dry blood spot disc has been developed by capillary GC-MS using selected ion monitoring with negative ion chemical ionization.(3) The fetal bile acids are usualy biosynthesized thrugh the hydroxylation at 1beta- or 6alpha-psition of the correspnding usual bile acids. The 1beta-hydroxylated C_<27>-bile acid were detected in the incubation mixture of the C_<27>-intermediates with rat liver microsome as a new biosynthetic route.(4) Diagnostic analysis of neonatal liver diseases was accomplished by GC-MS.The 3-oxo unsaturated bile acids were identified and determined in the urine of patients with sevear cholestasis from hypertyrosinemia.