Karlaganis G, Nemeth A, Hammarskjöld B, Strandvik B, Sjövall J
Eur J Clin Invest. 1982 Oct;12(5):399-405. doi: 10.1111/j.1365-2362.1982.tb00687.x.
Healthy infants and children were found to excrete bile alcohol glucuronides in urine. Following isolation and hydrolysis, the bile alcohols were estimated by capillary gas-liquid chromatography. The daily urinary excretion of the major compound, 27-nor-5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 24 xi, 25 xi-pentol (a C26 bile alcohol), ranged from 0.1 to 1.1 mumol/24 h per m2 body surface area for healthy infants and children. Two groups of patients with alpha 1-antitrypsin deficiency (phenotype PiZ) were also studied during infancy and childhood, and biochemical liver function tests and liver morphology were compared to the excretion of bile alcohols. The highest excretion of the C26 bile alcohol in urine was found in patients with alpha 1-antitrypsin deficiency and juvenile cirrhosis (2.1-8.4 mumol 24 h-1 m-2) regardless of preceding neonatal cholestasis. Patients with alpha 1-antitrypsin deficiency, neonatal cholestasis and subsequent fibrosis or normal liver morphology excreted bile alcohols within the normal range. The C26 bile alcohol constituted an average of 36% of the total bile alcohols in forty-three urine samples. This percentage was about the same in the three groups studied. The findings suggest that determination of urinary bile alcohols may be a valuable non-invasive diagnostic tool for patients with or at risk of developing liver cirrhosis.
健康婴幼儿的尿液中可检测出胆汁醇葡糖醛酸苷。分离并水解后，通过毛细管气液色谱法对胆汁醇进行定量分析。健康婴幼儿和儿童的主要化合物27-降-5β-胆甾烷-3α，7α，12α，24ξ，25ξ-戊醇（一种C26胆汁醇）的每日尿排泄量为每平方米体表面积0.1至1.1 μmol/24小时。对两组α1-抗胰蛋白酶缺乏症（PiZ表型）的患者在婴幼儿期和儿童期进行了研究，并将其生化肝功能检查和肝脏形态与胆汁醇排泄情况进行了比较。无论之前是否有新生儿胆汁淤积，α1-抗胰蛋白酶缺乏症合并青少年肝硬化患者尿液中C26胆汁醇的排泄量最高（2.1-8.4 μmol 24 h-1 m-2）。α1-抗胰蛋白酶缺乏症、新生儿胆汁淤积以及随后出现纤维化或肝脏形态正常的患者胆汁醇排泄量在正常范围内。在43份尿液样本中，C26胆汁醇平均占总胆汁醇的36%。在研究的三组中，这一比例大致相同。研究结果表明，测定尿胆汁醇可能是一种对肝硬化患者或有肝硬化风险的患者有价值的非侵入性诊断工具。
