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囊性纤维化患者尿胆汁酸排泄与肝脏组织病理学的相关性

Urinary bile acid excretion in correlation to liver histopathology in cystic fibrosis.

作者信息

Arborgh B, Eklund A, Norman A, Strandvik B

出版信息

Scand J Gastroenterol. 1980;15(1):73-80. doi: 10.3109/00365528009181435.

DOI:10.3109/00365528009181435
PMID:7367826
Abstract

Urinary bile acid excretion and liver morphology were compared in 25 patients with cystic fibrosis (CF). None showed clinical signs of liver disease. Most of the patients had normal liver function tests. Bile acids were determined in 24-h samples by a modification of the method of Almé. All patients had increased urinary excretion of trihydroxy bile acids, mainly cholic, 3 beta, 7 beta, 12 alpha- and 3 alpha, 7 beta, 12 alpha-trihydroxy-5 beta-cholanoic acids. Lithocholic acid excretion was lower in CF than in normal children. The urinary excretion of 3 beta-hydroxy-5-cholenoic acid was not increased in CF. In three patients with cirrhosis the urinary excretion of chenodeoxycholic acid was increased. The ratio of cholic to 3 beta-hydroxy-5-cholenoic acids was increased in all but three patients, and the ratio of chenodeoxycholic to 3 beta-hydroxy-5-cholenoic acids was increased in those with cirrhosis. These ratios differed more between cirrhotic and non-cirrhotic CF patients in this series than the ratio of cholic to chenodeoxycholic acids.

摘要

对25例囊性纤维化(CF)患者的尿胆汁酸排泄情况和肝脏形态进行了比较。所有患者均无肝病临床体征。大多数患者肝功能检查正常。采用改良的阿尔梅方法测定24小时样本中的胆汁酸。所有患者三羟基胆汁酸的尿排泄量均增加,主要是胆酸、3β,7β,12α - 三羟基胆酸和3α,7β,12α - 三羟基 - 5β - 胆烷酸。CF患者中石胆酸的排泄量低于正常儿童。CF患者中3β - 羟基 - 5 - 胆烯酸的尿排泄量未增加。在3例肝硬化患者中,鹅去氧胆酸的尿排泄量增加。除3例患者外,所有患者胆酸与3β - 羟基 - 5 - 胆烯酸的比值均增加,肝硬化患者中鹅去氧胆酸与3β - 羟基 - 5 - 胆烯酸的比值增加。在本系列中,肝硬化和非肝硬化CF患者之间这些比值的差异大于胆酸与鹅去氧胆酸的比值。

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Current clinical management of hepatic problems in cystic fibrosis.囊性纤维化肝脏问题的当前临床管理
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