Takikawa H, Beppu T, Seyama Y, Obinata K, Nittono H
Biochem Med. 1985 Jun;33(3):381-6. doi: 10.1016/0006-2944(85)90014-6.
Serum concentrations of nonglucuronidated-nonsulfated, glucuronidated, and sulfated bile acids in 9 control children and 16 children with cholestasis were quantitated by mass fragmentography. Total bile acid levels in control children were 19.55 +/- 2.78 mumol/liter (mean +/- SEM), and glucuronidated and sulfated bile acids comprised 2.6 +/- 0.5 and 17 +/- 3.1%, respectively. In 9 patients with congenital biliary atrasia, total bile acid levels were 167.34 +/- 11.18 mumole/liter of which 2.1 +/- 0.3% were glucuronidated and 15 +/- 1.4% were sulfated. Lithocholic and 3 beta-hydroxy-5-cholenoic acids, which have hepatotoxic effects, were presented in only small amounts in cholestatic children, and they were almost all glucuronidated or sulfated. The percentages of glucuronidated bile acids in control and cholestatic children were lower than in healthy and cholestatic adults, which may be explained by the lower activity of UDP-glucuronyltransferase in neonatal liver.
采用质量碎片分析法对9名对照儿童和16名胆汁淤积儿童的非葡萄糖醛酸化-非硫酸化、葡萄糖醛酸化和硫酸化胆汁酸的血清浓度进行了定量分析。对照儿童的总胆汁酸水平为19.55±2.78μmol/升（平均值±标准误），葡萄糖醛酸化和硫酸化胆汁酸分别占2.6±0.5%和17±3.1%。在9例先天性胆道闭锁患者中，总胆汁酸水平为167.34±11.18μmol/升，其中葡萄糖醛酸化的占2.1±0.3%，硫酸化的占15±1.4%。具有肝毒性作用的石胆酸和3β-羟基-5-胆烯酸在胆汁淤积儿童中仅少量存在，且几乎全部为葡萄糖醛酸化或硫酸化。对照儿童和胆汁淤积儿童中葡萄糖醛酸化胆汁酸的百分比低于健康和胆汁淤积成人，这可能是由于新生儿肝脏中UDP-葡萄糖醛酸基转移酶活性较低所致。
