Large amounts of 1 beta-hydroxylated bile acids in urine during taurine therapy.

The management of infants with cholestasis remains a difficult challenge. On the hypothesis that taurine is effective in treating neonatal cholestasis, taurine (1 g/day, per os) was administered to 2 patients with neonatal hepatitis and the bile acids were analyzed using gas chromatography-mass spectrometry (GC-MS). The serum levels of bilirubin and bile acids were significantly decreased by taurine. Before the treatment, cholic acid (CA) and chenodeoxycholic acid (CDCA) were predominant (79.2% in both patients) in the urine. There was a significant elevation of 1 beta-hydroxylated bile acids (1 beta BA), especially 1 beta, 3 alpha, 7 alpha, 12 alpha-tetrahydroxy-5 beta-cholan-24-oic acid (CA-1 beta-ol), in urine collected during the taurine therapy, and 1 beta BA became predominant (57.7-78.3%). Therefore, increased amounts of urine 1 beta BA were excreted during taurine administration. Taurine therapy is recommended, because it might be effective for treating neonatal cholestasis.