摘要
目的观察缝隙连接蛋白43(Cx43)在先天性巨结肠(HD)肠壁中的分布,探讨Cx43与HD发病的关系。方法HD患儿42例。男33例,女9例;年龄2个月~10岁。均为散发病例,全部取材经组织病理学检测符合HD诊断,包括常见型30例,短段型12例;其中并回肠闭锁1例,并先天性肠扭转不良2例。同时取5例肠套叠患儿(30 d^8岁;男4例,女1例)作为对照组。采用免疫组织化学方法观察42例经病理诊断为HD的标本肠壁各层之间Cx43蛋白分布情况,取阳性细胞计数进行半定量分析。采用SPSS 11.5软件进行统计学分析。结果HD狭窄段(无神经节细胞肠段)肠壁肌层内Cx43的表达缺失,各层中几乎未见Cx43的表达,与HD扩张段(有神经节细胞肠段)及健康对照组间的差异有统计学意义(P<0.01)。HD移行段肠壁环肌层和环肌层与纵肌层交界处Cx43有中等强度表达,与扩张段、狭窄段之间有明显差异(P<0.01)。HD扩张段肠壁环肌层与纵肌层交界部位Cx43呈强阳性分布,黏膜下层和纵肌层未见或少见Cx43表达。HD扩张段和健康对照组肠壁环肌层和环肌层与纵肌层交界处Cx43有中至强度表达,二组肠壁肌层内Cx43表达无显著性差异(P>0.05)。结论Cx43表达的缺失或减少及缝隙连接结构的破坏,可能是使细胞间物质、电信号的传递障碍而导致HD发病的原因之一。
Objective To investigate the distribution of connexin 43 （Cx43）in Hirschsprung＇s disease （HD）bowels and explore its role in the pathogenesis of HD. Methods Forty - two cases （33 boys, 9 girls）aged from 2 months to 10 years of HD were included in this study by using immunohistochemistry method. All eases were sporadic. There were 30 cases of common type, 12 cases of short segment type of which there was 1 case of ileal atresia. And all cases were diagnosed as HD using histopathological method. The other 5 patients（ aged from 1 month to 8 years ;4 boys, 1 girl）with intussusception-were used as controls. SPSS 11.5 software was used to analyze the data. Results There was no immunoreaetivity in Cx43 in the muscle layers of aganglionic segment in HD ,which had significant difference in Cx43 expression in ganglionic segment of HD and normal bowels（ P 〈 0.01 ）. Moderate immunostainning of Cx43 was observed in the circular muscle and the region between the circular and longitudinal layer in migratory segment from patients with HD,whieh had significant difference in Cx43 expression in ganglionic and aganglionic segment of HD （P 〈 0.01 ）. Moderate to strong immunostainning of Cx43 was observed in 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 in Cx43 expression in ganglionic segment of HD and normal bowels （P 〉 0.05）. Conclusions The failure or decline of Cx43 expression in the aganglionie bowel of HD and the destruction of the gap - junction indicate that the impaired intercellular substances, electric signals may partly be responsible for the motility dysfunction in HD.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2008年第7期513-515,共3页
Journal of Applied Clinical Pediatrics
基金
陕西省自然科学基金项目资助(2003C2022)
