Urinary excretion of bile alcohols in normal children and patients with α1‐antitrypsin deficiency during development of liver disease
Urinary excretion of bile alcohols in normal children and patients with α1‐antitrypsin deficiency during development of liver disease
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正常儿童和α1抗胰蛋白酶缺乏症患者肝病发展过程中胆汁醇的尿排泄
DOI：
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发表时间：
1982
影响因子：
5.5
通讯作者：
J. Sjövall
中科院分区：
文献类型：
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作者：
G. Karlaganis;A. Németh;B. Hammarskjöld;B. Strandvik;J. Sjövall
摘要。健康的婴儿和儿童被发现在尿液中排泄胆汁酒精葡萄糖醛酸盐。分离和水解后，用毛细管气液色谱法测定胆汁醇。健康婴儿和儿童每日尿中主要化合物27 - no - 5β -胆甾烷- 3α、7α、12α、24 - ξ、25 - ξ -戊醇（一种C26胆汁醇）的排泄量为每m2体表面积0.1 ~ 1.1 μmol/24 h。还研究了两组婴儿期和儿童期α1‐抗胰蛋白酶缺乏症（表型PiZ）患者，并比较了生化肝功能测试和肝脏形态学与胆醇排泄的关系。尿中C26胆汁醇排泄量最高的是α - 1 -抗胰蛋白酶缺乏和幼年肝硬化患者（2.1 - 8.4 μmol 24 h‐1 m‐2），与新生儿是否有胆汁淤积无关。α1‐抗胰蛋白酶缺乏、新生儿胆汁淤积和随后的纤维化或肝脏形态正常的患者在正常范围内排出的胆汁醇。在43份尿液样本中，C26胆醇平均占总胆醇的36%。这一比例在被研究的三组中大致相同。研究结果表明，测定尿胆醇可能是一种有价值的非侵入性诊断工具，用于肝硬化患者或有肝硬化风险的患者。
Abstract. 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‐5β‐cholestane‐3α,7α,12α,24ξ,25ξ‐pentol (a C26 bile alcohol), ranged from 0·1 to 1·1 μmol/24 h per m2 body surface area for healthy infants and children. Two groups of patients with α1‐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 α1‐antitrypsin deficiency and juvenile cirrhosis (2·1–8·4 μmol 24 h‐1 m‐2) regardless of preceding neonatal cholestasis. Patients with α1‐antitrypsin deficiency, neonatal cholestasis and subsequent fibrosis or normal liver morphology excreted bile alcohols within the normal range. The C26 bile alochol 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.