Cajal间质细胞和缝隙连接蛋白43在先天性巨结肠中的表达

目的本研究通过观察Cajal间质细胞（ICCs）和缝隙连接蛋白43（connexin43）在先天性巨结肠（HD）肠管中的分布情况，探讨HD的发病机制。方法采用兔抗人多克隆c-kit抗体和connexin 43抗体，通过SABC双标免疫组化染色方法观察35例HD患儿肠管c-kit^＋ICCs和connexin 43的分布情况。结果ICCs分布在环肌内和肌间神经丛周围，ICCs彼此及与平滑肌细胞形成缝隙连接。在HD病变肠管ICCs数目明显减少甚至消失，与HD正常肠管相比，差异十分显著（P〈0．01）。在HD病变肠管connexin 43表达明显减弱。结论ICCs和connexin 43分布异常导致了HD病变肠管慢波节律和兴奋传导异常，从而引起或加重HD发病。

Distribution of Interstitial Cells of Cajal and the Gap Junction Protein Connexin 43 in Hirschsprung's Disease

Objective To study the role of ICCs and the Gap junction protein connexin 43 in the pathogenesis of Hirschspreng's disease.Methods The distribution of ICCs and the expression of the Gap junction protein connexin 43 were studied by double immunostaining using anti c-kit and anti connexin 43 antibodies in 35 cases of HD aged from 5 months to 2.5 years.Results ICCs were located in the inner part of the circular muscle and around the myenteric plexus between the two muscle layers.There was gap junction between ICCs and ICCs,between ICCs and the smooth muscle cells.The number of c-kit+ ICCs was remarkably reduced or even disappeared in aganglionic segment of bowel in HD.Statistics showed the significant difference in ICCs between the aganglionic and ganglionic bowel segments(P < 0.01).In the aganglionic part of HD the connexin 43 staining was weak.Conclusion The lack of ICCs and connexin 43 may lead to the abnormal spontaneous electrical activity and conduction in aganglionic segments.