先天性胆总管囊肿患儿胆汁成分分析及致石性研究

目的：探讨先天性胆总管囊肿患儿胆汁成分变化与结石形成关系。方法：对10例胆总管囊肿及9例非肝胆疾病手术患儿胆汁成分进行定量分析，包括总胆汁酸、4种初级胆汁酸、磷脂、胆汁蛋白、胆固醇及总脂含量等，并进行胆汁细菌培养。结果：①囊肿患儿胆汁中胆汁酸、磷酸、胆固醇、总蛋白及总脂浓度明显异常，以胆汁酸减少为显著。其肝胆汁、胆囊胆汁、囊肿胆汁中总胆汁酸浓度均明显降低，总脂浓度下降。易发生胆结石的主要原因之一；②囊肿患儿三种胆汁中四种初级结合型胆汁酸的含量及G／T，CA／CDCA均降低，胆汁酸组分比的变化，也是易产生结石的原因。囊肿中初级结合型胆汁酸的浓度相对降低，次级胆汁酸相对增加，可能对囊肿恶变发生的影响；③囊肿患儿肝细胞功能受损是导致CCC患儿胆汁成分发生改变的主要原因。结论：CCC患儿胆汁中主要固体成分存在明显异常，以胆汁酸减少及胆汁酸的组分比改变为著，是CCC患儿易发生结石的主要原因之一，导致CCC患儿胆汁成分改变的核心问题是肝功能受损所致。

Analysis of bile composition and gallstone formation in children with congenital choledochal cyst

Objective To study relationship between the bile composition and the gallstone formation in patients with congenital choledochal cyst (CCC). Methods In 10 children with choledochal cyst and 9 children with diseases outside the bile duct and liver, the total bile acid (TBA), cholesterol (CHO), phospholipid (PL), total lipid (TL), the concentration of bile protein were determined and biliary culture was performed for bacteria.Results (1) In children with CCC, the bile acids, PL, CHO, total protein and TL in bile were obviously abnormal, and this is especially true of bile acids. The concentration of bile acids in liver bile, gallbladder bile and cyst bile were all obviously lowered with the TL being decreased, which are believed to be responsible for the formation of biliary stones. The content of four conjugated bile acid, CA/CDCA, G/T were all lowered in the bile of children with CCC, and the decrease was responsible for the formation gall stones. (2) The primary conjugated bile in cyst was relatively lower and the secondary bile acid was relatively increased, which might contribute to the cancerous change of the cyst. (3) The impairment of liver cell function was the main reason for the change of bile composition in CCC patients.Conclusions The study demonstrate that children with CCC have a significant change of solid contents in bile with the reduction of TBA and change of composition of bile acids being the most obvious, which might predispose CCC patients to cholelithiasis. The critical cause for the bile changes is impairment of liver function.