Relationship between intrahepatic bile duct development and hepatic fibrosis in children with biliary dysplasia

Q4 Medicine

中华小儿外科杂志 Pub Date : 2019-12-15 DOI:10.3760/CMA.J.ISSN.0253-3006.2019.12.008

Xin Li, Chen Yu, Wei Gao, Xiao-li Hu, J. Zhan

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引用次数: 1

Abstract

Objective To explore the pathological changes of intrahepatic bile duct development and liver fibrosis in children with biliary dysplasia at different ages and to explore the effect of intrahepatic bile duct development on liver fibrosis. Methods From January 2015 to January 2019, a total of 16 hospitalized children with biliary dysplasia at Municipal Children's Hospital and First Central Municipal Hospital were recruited and divided into two groups according to age, i. e. <180 days (G1, n=8) and ≥180 days (G2, n=8). Liver biopsy specimens were harvested intraoperatively for routine hematoxylin & eosin and immunohistochemical staining (CK19 & EMA). The major histopathological changes of liver, such as ratio of interlobular bile duct to portal area, diameter of interlobular bile duct, ductal proliferation, liver fibrosis, cholestasis, bile plugs in ductules and an infiltration of inflammatory cells in portal region, were analyzed. Results The pathological changes of G1 and G2 groups showed that the degree of liver fibrosis in G2 group was higher than that in G1 group [0.5(0, 1) vs. 4(2.25, 4)]. The inter-group difference was statistically significant (Z=3.467, P<0.001); The ratio of interlobular bile duct to portal area was lower in G2 group than that in G1 group [0.25(0.16, 0.34) vs. 0]. The inter-group difference was statistically significant (Z=2.663, P<0.05); The degree of ductal proliferation showed that G2 group was significantly higher than G1 group [1(0.25, 2) vs. 2.5(2, 3.75)]. The inter-group difference was statistically significant (Z=2.867, P<0.05). The expression of EMA was significantly lower in G2 group than that in G1 group [(0.034±0.012) vs. (0.082±0.009)]. The inter-group difference was statistically significant (t=9.432, P<0.001); The expression of CK19 was significantly higher in G2 group than that in G1 group [(0.201±0.016) vs. (0.135±0.010)]. There was significant inter-group difference (t=9.615, P<0.001). Correlation analysis of intrahepatic bile duct development revealed: ratio of interlobular bile duct to portal area and age of children (rs=-0.714, P=0.002), ductal proliferation (rs=-0.610, P=0.012) and liver fibrosis (rs=-0.533, P<0.05) showed a significant negative correlation. Conclusions With the aging of the patients, interlobular bile duct of children with biliary dysplasia decreases gradually. And the degree of hepatic fibrosis continues aggravating with a poor prognosis. Key words: Hepatic fibrosis; Interlobular bile duct; Deficiency symptom; Bile duct; Hyperplasia

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胆道发育不良患儿肝内胆管发育与肝纤维化的关系

目的探讨不同年龄胆道发育不良患儿肝内胆管发育及肝纤维化的病理变化，探讨肝内胆管发育对肝纤维化的影响。方法选取2015年1月~ 2019年1月在市儿童医院和市第一中心医院住院的胆道发育不良患儿16例，按年龄分为<180天组(G1, n=8)和≥180天组(G2, n=8)。术中取肝活检标本进行常规苏木精和伊红染色及免疫组织化学染色(CK19和EMA)。分析肝脏的主要组织病理学改变，如小叶间胆管与门静脉区比值、小叶间胆管直径、胆管增生、肝纤维化、胆汁淤积、胆管内胆塞及门静脉区炎症细胞浸润。结果G1组和G2组病理变化显示，G2组肝纤维化程度高于G1组[0.5(0,1)比4(2.25,4)]。组间差异有统计学意义(Z=3.467, P<0.001);G2组小叶间胆管与门静脉面积之比低于G1组[0.25(0.16,0.34)比0]。组间差异有统计学意义(Z=2.663, P<0.05);导管增生程度G2组明显高于G1组[1(0.25,2)vs. 2.5(2,3.75)]。组间差异有统计学意义(Z=2.867, P<0.05)。G2组EMA表达明显低于G1组[(0.034±0.012)∶(0.082±0.009)]。组间差异有统计学意义(t=9.432, P<0.001);CK19在G2组的表达明显高于G1组[(0.201±0.016)比(0.135±0.010)]。组间差异有统计学意义(t=9.615, P<0.001)。肝内胆管发育相关分析显示:小叶间胆管与门静脉面积之比与儿童年龄(rs=-0.714, P=0.002)、胆管增生(rs=-0.610, P=0.012)、肝纤维化(rs=-0.533, P<0.05)呈显著负相关。结论随着患者年龄的增长，胆道发育不良患儿小叶间胆管逐渐减少。肝纤维化程度继续加重，预后不良。关键词:肝纤维化;小叶间胆管;缺乏症状;胆管;增生

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来源期刊

中华小儿外科杂志 Medicine-Pediatrics, Perinatology and Child Health

CiteScore

0.40

自引率

0.00%

发文量

8707

期刊介绍： Chinese Journal of Pediatric Surgery is an academic journal sponsored by the Chinese Medical Association. It mainly publishes original research papers, reviews and comments in this field. The journal was founded in 1980 and is included in well-known databases such as Peking University Journal (Chinese Journal of Humanities and Social Sciences) and CSCD Chinese Science Citation Database Source Journal (including extended version). It is one of the national key academic journals under the supervision of the China Association for Science and Technology. Chinese Journal of Pediatric Surgery enjoys a high reputation and influence in the academic community. The articles published in this journal have a high academic level and practical value, providing readers with a large number of practical cases and industry information, and have received widespread attention and citations from readers.