缝隙连接蛋白43在先天性巨结肠肠壁中的分布

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。

Immunolocalization of the gap junction protein connexin 43 in the bowels of Hirschsprung''''s disease

Objective To investigate the expression of Connexin 43(Cx43) in Hirschsprung's disease(HD) bowels and to explore its role in the pathogenesis of HD.Methods Thirty cases(male 26,female 4) aging from 1 month to 8 years of HD were included in this study.Of which,short segment type was in 1 case,common type in 20,long segment in 5 and total colonic type in 4.The expression of Cx43 between the muscle layers of bowel from HD patients and controls(30 cases) was visualized by Cx43 immunohistochemistry.The other 3 fresh tissues were used to investigate the distribution of gap junctions in HD bowels by electromicroscopy.Results There was no immunoreactivity of Cx43 in the muscle layers of aganglionic segment in HD.Moderate to strong immunostainning of Cx43 was observed at the circular muscle and the region between the circular and longitudinal layer in ganglionic segment from patients with HD and in normal bowels.There was no significant difference of Cx43 expression in ganglionic segment of HD and normal bowels.Under electromicroscopic examination,gap junction was absent or less in the aganglionic part of HD,but it could be observed in ganglionic part of HD.Conclusions The lack expression of Cx43 in the aganglionic bowel of HD indicates that the impaired intercellular communication between cells might partly be responsible for the motility dysfunction in HD.