Cajal间质细胞在先天性巨结肠和巨结肠同源病结肠中的分布研究

目的研究先天性巨结肠（Hirschsprung’s disease，HD）和巨结肠同源病（allied Hirschsprung’s disease，AHD）肠壁内Cajal间质细胞（interstitial cells of Cajal，ICCs）的分布状态，探讨HD和AHD的发病机制。方法选择确诊为HD和AHD的患者各20例，取巨结肠根治术吻合口远端的全层肠壁作为实验组，另取16例正常结肠标本作为对照组。用鼠抗人c—kit单克隆抗体（CD117）标记ICCs，Image Pro-Plus图像分析系统检测ICCs。结果对照组中大量ICCs分布在肌间神经丛周围和环纵肌层内，ICCs包绕神经丛周围，肌层间ICCs连续分布；HD组远端肠管中肌层间和各肌层内ICCs明显减少甚至缺如，与对照组比较差异有统计学意义，P〈0．01；AHD组远端肠管中神经丛大小不一，ICCs分布差异大，大多数神经丛区ICCs减少，环肌层内ICCs明显减少，与AHD组和对照组比较差异有统计学意义，P〈0.01。HD组远端结肠中ICCs减少比AHD组显著，差异有统计学意义，P〈0．01。结论HD、AHD病变肠管中除了神经节细胞的异常外，同时存在ICCs异常；ICCs在HD和AHD的分布不同可能与两者临床症状差异有关；肠管中ICCs数量可能与临床症状及预后有一定关系。

A study on the distribution of interstitial cells of Cajal in colon Hirschsprung''''s disease and allied Hirschsprung''''s disease

Objective This study was to examine the distribution of interstitial cells of Cajal (ICCs) in the colon of patients with Hirschsprung's disease and with allied Hirschsprung's disease,and to investigate the role of ICCs in the pathogenesis of Hirschsprung's disease (HD) and allied Hirschsprung's disease (AHD).Method Twenty HD patients,20 AHD cases and 16 healthy controls were studied.The whole layer of colon distal to the anastomosis was collected.ICCs were indentified with a monoclonal antibody to c-kit(CD117).The area occupied by ICCs was calculated with Image Pro-Plus analysis software. The result was analysed by SPSS10.0 software.Results ICCs were abundant and located in myenteric plexus(MP),circle muscle and longitudinal muscle.In HD aganglionic colon,ICCs were very scarce. There was a significant difference between the distal colon of HD and that of control (P<0.001).In AHD bowel,ICCs cells were scarce and mainly located in the area of MP.The distribution of ICCs in AHD distal colon was significantly different compared with control (P<0.01).The distribution difference of ICCs reached statistically significance among the three groups (P<0.01). Conclusion This study reveals the relation between the abnormal distribution of ICCs and the pathogenesis of HD,AHD.The distribution difference of ICCs in AHD and HD may be related with their varied signs,symptoms and prognosis.