不同年龄胆总管囊肿的临床与肝纤维化特点探讨

Clinical Manifestations and Hepatic Fibrosis of Choledochal Cyst in Children with Different Ages

目的 探讨不同年龄胆总管囊肿的临床与肝脏纤维化特点与相互关系。方法 分析12例婴儿及36例幼儿、儿童胆总管囊肿的临床资料;肝组织HE染色观察纤维化程度和炎性细胞浸润情况,免疫组化染色观察细胞角化蛋白(AE1/AE3)、人类组织相容性抗原DR(HLA—DR)表达,并与18例胆道闭锁作对照。结果 婴儿胆总管囊肿黄疸出现率12/12,显著高于幼儿儿童组8/36(P<0.01);肝纤维化程度、小叶周边AE1/AE3阳性细胞和HLA—DR在肝脏表达显著高于非婴儿组(P<0.01),低于胆道闭锁组(P<0.01)。肝纤维化程度与黄疸及肝小叶周边AE1/AE3表达分级呈正相关(P<0.05)。结论 婴儿胆总管囊肿以黄疸为主要临床表现,其肝纤维化重于幼儿及儿童组,胆道梗阻和胆小管增生可能是其发生肝纤维化的原因。

Objective To observe different characteristics of clinical manifestation and hepatic fibrosis in choledochal cyst between infants and older children. Methods Forty-eight children with choledochal cysts were divided into infants (n=12) and older children(n=36)groups, the clinical data were recorded. Semiquantitive scoring systems were used to assess the grade of liver fibrosis, degrees of inflammatory cells infiltration in HE sections, the expressions of cytokeratin (AE1/AE3), human histocompatibility antigen (HLA-DR) were observed by immunohistochemical staining technique, and compared with 18 cases biliary atresia(control group).Results the occurrence rate of jaundice in the infants(12/12) was significantly higher than that of older children(8/36(P< 0.01). The grade of liver fibrosis, proliferation of AE1/AE3 positive staining in the periportal biliary ductules and HLA-DR positive expression in the inflammatory cells in infants were obviously higher than those of older children (P<0.01), but lower than those in biliary atresia (P<0.01).The score of AE1/AE3 expression in periportal ductules had positive correlation with jaundice and the grade of hepatic fiberosis (P<0.05).Conclusions Infantile choledochal cyst could develop into hepatic fiberosis was more frequently than those of older children. Bile duct obstruction and proliferation of periportal biliary ductules may play an important role in liver fibrosis in these patients.