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目的观察缝隙连接蛋白43(Cx43)在先天性巨结肠症(HD)肠壁中的分布,探讨Cx43与HD发病的关系。方法运用免疫组化法观察30例HD患儿及30例对照组肠壁中Cx43的分布情况。患儿男26例,女4例,年龄1个月~8岁。其中短段型1例,普通型20例,长段型5例,全结肠型4例。另取3例新鲜组织在电镜下观察缝隙连接的分布情况。结果HD无神经节细胞肠段肠壁内Cx43的表达消失,与HD有神经节细胞肠段及对照组相比,具有显著性差异(P<0.01)。HD有神经节细胞肠段肠壁环肌层及肌间层有阳性至强阳性的Cx43表达,纵肌层偶有表达或表达缺失,与对照组无显著差异(P>0.05)。电镜下在HD无神经节细胞肠段未见缝隙连接的超微结构,而HD有神经节细胞肠段可见其存在。结论Cx43的表达缺失或减少,及缝隙连接结构的破坏可能与HD的肠动力障碍的发病机制有关。
Objective To observe the distribution of connexin 43 (Cx43) in the intestinal wall of Hirschsprung disease (HD) and to explore the relationship between Cx43 and HD. Methods The distribution of Cx43 in the intestinal wall of 30 cases of HD children and 30 cases of control group were observed by immunohistochemistry. 26 cases of children with males and 4 females, aged 1 month to 8 years old. One short segment in 1 case, 20 cases of general type, long segment in 5 cases, 4 cases of colon-type. Another 3 cases of fresh tissue under the electron microscope to observe the distribution of gap junction. Results The expression of Cx43 in the gut wall of HD ganglion cells disappeared, which was significantly different from that of ganglion cells in HD ganglion cells and the control group (P <0.01). The expression of Cx43 was positive and strongly positive in the muscularis propria of the ganglion cells in the intestine segment of HD. There was no significant difference (P> 0.05) between the expression of Cx43 and the control. There was no ultrastructure of gap junctions in the gut segment of HD without ganglion cells under electron microscope, while the presence of ganglion cells in HD was observed in HD. Conclusion The loss or decrease of the expression of Cx43 and the destruction of the gap junctions may be related to the pathogenesis of intestinal motility disorders in HD.