CAJ | 학술논문

目的检测肠神经在先天性胆总管囊肿(CCC)中的发育异常,探讨CCC发病机制.方法 2007年至2009年连续36例CCC患儿实施胆总管囊肿切除、肝管空肠吻合术,分别切取其远、近端囊壁为实验组；选取计划外怀孕,中晚期终止妊娠胎儿15例的肝外胆道为对照组.对比两组神经生长因子(NGF)及其受体(P75N6FR和TrkA)、神经细胞特异性烯醇化酶(NSE)和蛋白基因产物S-100、Cajal间质细胞(c-kit)和突触素(SY)的表达,分析各指标间以及其与囊肿直径的相关性,明确胆道神经发育与CCC发病关系.结果 CCC组各指标在囊肿远端表达均弱于近端表达(F=2.694、2.667、1.833、1.750、1.667、2.139、0.861,P＜0.05).囊肿远端与胎儿胆管间除SY外差异均有统计学意义(F=2.734、3.552、3.448、2.996、3.337、3.155,P＜0.05)；囊肿近端与胎儿胆管间除TrkA、S-100外,其余指标差异均无统计学意义(F=0.040、0.885、1.246、1.016、0.242,P＞0.05).囊肿直径与囊肿远端NGF、NSE、S-100、c－kit表达均呈负相关(rs=-0.739、-0.787、-0.577、-0.798,P＜0.05),囊肿远端NGF与P75NGFR、NSE、S-100、c－kit间,P75NGFR、NSE、S-100与c-kit间,NSE与S-100间均呈正相关(P＜0.05).结论胚胎期各种原因导致胆管远端肠神经细胞发育、成熟和分布受限,加之神经－肌肉的传导不良,可导致CCC发病；胆道远端肠神经细胞的发育、分布及神经传导与囊肿大小密切相关.
목적 검측장신경재선천성담총관낭종(CCC)중적발육이상,탐토CCC발병궤제.방법 2007년지2009년련속36례CCC환인실시담총관낭종절제、간관공장문합술,분별절취기원、근단낭벽위실험조；선취계화외부잉,중만기종지임신태인15례적간외담도위대조조.대비량조신경생장인자(NGF)급기수체(P75N6FR화TrkA)、신경세포특이성희순화매(NSE)화단백기인산물S-100、Cajal간질세포(c-kit)화돌촉소(SY)적표체,분석각지표간이급기여낭종직경적상관성,명학담도신경발육여CCC발병관계.결과 CCC조각지표재낭종원단표체균약우근단표체(F=2.694、2.667、1.833、1.750、1.667、2.139、0.861,P＜0.05).낭종원단여태인담관간제SY외차이균유통계학의의(F=2.734、3.552、3.448、2.996、3.337、3.155,P＜0.05)；낭종근단여태인담관간제TrkA、S-100외,기여지표차이균무통계학의의(F=0.040、0.885、1.246、1.016、0.242,P＞0.05).낭종직경여낭종원단NGF、NSE、S-100、c－kit표체균정부상관(rs=-0.739、-0.787、-0.577、-0.798,P＜0.05),낭종원단NGF여P75NGFR、NSE、S-100、c－kit간,P75NGFR、NSE、S-100여c-kit간,NSE여S-100간균정정상관(P＜0.05).결론 배태기각충원인도치담관원단장신경세포발육、성숙화분포수한,가지신경－기육적전도불량,가도치CCC발병；담도원단장신경세포적발육、분포급신경전도여낭종대소밀절상관.
Objective To explore the pathogenesis of congenital choledochal cysts (CCC) by detecting enteric neural abnormalities in CCC.Methods From 2007 to 2009,36 children with CCC underwent choledochal cyst excision and hepaticojejunostomy.Their proximal and distal cystic wall specimens were incised respectively and selected as the experimental group.At the same time,the extrahepatic bile ducts of 15 fetus who were late terminated because of unplanned pregnancy were selected as the control group.The expression of NGF and its receptors (p75NGFR and TrkA),NSE,S-100,Cajal interstitial cells and SY between two groups were compared and the correlation between each index and cysts' diameters was analyzed in order to understood the relationship between the pathogenesis of CCC and neural abnormalities.Results The expression levels of all indicators in the distal cysts were weaker than those in the proximal ones (F=2.694,2.667,1.833,1.750,1.667,2.139,0.861,P＜0.05).There was significant difference in the expression of indicators except SY between distal cysts and fetal bile duets ( F =2.734,3.552,3.448,2.996,3.337,3.155,P ＜0.05) ; There was no significant difference between the proximal cysts and fetal bile ducts except TrkA and S-100 ( F =0.040,0.885,1.246,1.016,0.242,P ＞0.05 ).The expression of NGF,NSE,S-100 and c-kit in distal cysts had a negative correlation with the diameter of cysts ( rs =- 0.739,- 0.787,- 0.577,- 0.798,P ＜ 0.05 ) ; There was a positive correction between the expression of NGF with p75NGGR,NSE,S-100 and c-kit in distal cysts ( P ＜ 0.05 ),between the expression of c-kit with p75NGFR,NSE,S-100,as well as between the expression of NSE with S-100.Conclusion The restriction of enteric nerve distribution and maturation in distal biliary tract,coupled with nerve-muscle transmission badness due to embryonic dysplasia may cause CCC.The size of cyst is closely related to the upgrowth,distribution and nerve conduction of enteric nerve cells in distal biliary tract.