Cajal间质细胞在先天性巨结肠分布的研究

目的：通过观察cajal间质细胞（interstitial cells of cajal，ICC）在正常结肠及先天性巨结肠先天性巨结肠（hirschsprung’s disease，HD）患者痉挛段、移行段、扩张段的分布，探讨HD的发病机制。方法：收集25例HD患儿标本，于术中分别选取扩张段、移行段、痉挛段肠壁的全层组织，另取6例手术患儿的正常结肠全层组织标本，常规固定石蜡包埋组织切片备用。对标本行c-Kit免疫组织化学染色。光镜观察ICC的分布，计数并进行统计学分析。结果：正常结肠ICC主要分布在环肌内侧面与黏膜下层之间即黏膜下ICC（submucosal ICC．ICC—SM）、环肌与纵肌之间的肌间神经从周围即肌间ICC（myenteric ICC，ICC—MY）以及环肌与纵肌内。HD患儿痉挛段ICC—SM、ICC—IM细胞数较扩张段和正常对照组明显减少（P〈0．01），且ICC的细胞突起的分支亦减少，彼此之间不能形成完整的细胞网络。而扩张段ICC与正常对照组比较无明显差异（P〉0．05）。结论：HD患儿结肠ICC的异常分布，可能是HD发病、肠管蠕动障碍以及排便异常的原因之一。

Immunohistochemical study on interstitial cells of cajal in Hirschsprung''s disease

Objective To investigate the role of interstitial cells of cajal （ICC） in the pathogenesis of HD through observing the distribution of the cells in normal colon and HD＇s different segments. Methods The aganglionic,transitional and dilated segments were collected in operations from 25 children with Hirschsprung＇s disease. Another 6 normal segmengts were also collected. Each sections was stained by c-Kit immunohistochemistry. The distribution of the interstitial cells of Cajal was observed by light microscope. Results The ICC in aganglionic segments of colon from Hirchsprung＇s disease were decreased significantly and statistically than that in dilated and normal colon. Moreover, whole mount preparations showed that, not only the number of ICC-IM was decreased down, but also the branches of ICC-IM and the cellular network were also broken down. Conclusion The pathogenesis of HD is related to the abnormal distribution of ICC, which lead to or aggravate the bowel spasm in HD.