抄録
This study evaluated the urinary metabolism of ursodeoxycholic acid (UDCA) by measuring 7β-N-acetylglucosaminide and 3α-sulfate composition in neonates to adolescents using liquid chromatography electrospray ionization tandem mass spectrometry (LC/ESI-MS/MS). We obtained urine from 13 babies, corrected gestational age (CGA) 37–70 weeks, receiving UDCA to treat cholestasis due to total parenteral nutrition (TPN) and 8 patients, aged from 9 months to 15 years, who were treated with oral UDCA administration. The ratios of each UDCA conjugate to total UDCA were as follows: at CGA 37–42 weeks: non-amidated and glycine- or taurine- amidated ursodeoxycholic acid (UDCAs): 44.0±8.0% (mean±SD), UDCAs 3α-sulfate (UDCAs-3S): 37.8±10.1%, UDCAs 7β-N-acetylglucosaminide (UDCAs-7NAG): 18.1±14.9%; at 9 months-3years: UDCAs: 15.7±23.2%, UDCAs-3S: 37.2±8.8%, UDCAs-7NAG: 47.0±22.1%. The ratios of UDCAs-3S and UDCAs-7NAG increased gradually between the ages of 5 and 15 years, as follows: UDCAs, 2.2±1.3%; UDCAs-3S, 51.2±22.9%; and, UDCAs-7NAG, 46.6±22.6%. The ratio of 3-sulfooxy-7-N-acetylglucosaminyl-UDCAs (UDCAs-3S-7NAG) lowered by 0.2% per each age group. Urinary UDCAs-7NAG was not detectable in 2 of 21 patients, who were thus considered to have 7β-hydroxy bile acid N-acetylglucosaminyl transferase (UGT3A1) deficiency. The enzyme activity of N-acetylglucosaminyl transferase to UDCAs was at the same degree as sulfotransferase to UDCAs in early neonates, but reached the adult values by 3–5 years.
