小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.

Quantitative analysis of bile acid contents of choledochal cyst in children with liquid chromatography-elec-trospray tandem mass spectrometry

Objective To investigate the changes of bile acids in children suffering from thole-doehal cysts (CBD). Methods The bile from the choledochal cysts of 16 children was taken during op-eration. The samples were analyzed with Liquid Chromatography-Eleetrospray Tandem Mass Spec-trometry (LC-MS/MS). The levels of the bile acids including 15 unconjugated,glycine and taurine-con-jugated isomeric forms of bile acids were determined. Results The concentrations of total bile acids (TBA) declined significantly,and so did all forms of bile acids. No bile acid with carcinogenesis poten-tial was found to be elevated. The portion of glycoursodeoxycholic acid(GUDC) with CBD was de-creased,which had not been reported previously in children. The decrease of concentrations of TBA corre-lated with the severity of hepatic cirrhosis in these children. Conclusions The bile acids from CBD children was abnormal This may be related to the severity of hepatic cirrhosis and result in gallstone formation.