小儿先天性胆管扩张症血清胆汁酸谱的初步分析

The changes in the bile acids in the serum of children with congenital biliarv dilation

目的探讨小儿先天性胆管扩张症血清胆汁酸谱的特点及其意义。方法建立人类已知全部15种胆汁酸液相色谱串联质谱定量检测方法,对15例先天性胆管扩张症患儿术前及术后1周、10例术后1年的空腹血清进行胆汁酸定量检测。结果患儿术前血清总胆汁酸(TBA)、牛黄胆酸(TC)和脱氧胆酸(DC)浓度异常升高,而胆酸(CA)、鹅脱氧胆酸(CDC)和熊脱氧胆酸(UDC)浓度降低;术前血清牛黄型胆汁酸和DC的百分含量升高,牛黄型和甘氨型胆汁酸比值(T/G)升高,而UDC百分含量下降。肝纤维化较重组患儿TBA和DC浓度以及DC百分含量高于轻度组。这些异常在根治术后1年内逐渐恢复正常。结论先天性胆管扩张症患儿存在血清胆汁酸谱异常,可能与继发性肝脏损伤程度有关。根治术后这些异常的恢复需较长时间。

Objective To investigate the changes of the bile acids in the serum of children with congenital biliary dilation. Methods The serum of 15 patients with congenital biliary dilation was taken preoperatively and at 7 days post-operation. Ten was taken at 1 year post-operation. The samples were analyzed using a method introduced by us based on Liquid Chromatography-Electrospray Tandem Mass Spectrometry （LC-MS/MS）. Concentration of bile acids including all the 15 unconjugated, glycine and taurine-conjugated isomeric forms of bile acids were quantized. Results The concentration of total bile acids （TBA）, TC, and DC was significantly higher, but CA, CDC, and UDC were significantly lower than those of the normal controls （10 cases）. Proportion of preoperative taurine-conjugated bile acids,DC and ratio of T/G were increased markedly compared with normal controls. On the contrary, the proportion of UDC decreased significantly. The concentrations of TBA, TC, and DC were correlated to the severity of hepatic cirrhosis in these patients. Conclusions There is an abnormal spectrum of bile acids in CBD patients,which may be correlated to the severity of hepatic damage.