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目的采用免疫组织化学方法,对先天性巨结肠神经肌肉连接进行研究,并探讨其与先天性巨结肠发病机制之间的关系。方法应用免疫组化技术对20例先天性巨结肠病变肠段及10例正常结肠组织标本行突触素和神经丝神经肌肉连接标记,光镜下观察其免疫反应表达。结果对照组的结肠突触素和神经细丝的免疫反应呈强阳性表达,先天性巨结肠组扩张肠段突触素和神经细丝的免疫反应呈阳性或弱阳性表达,狭窄段肠壁突触素和神经细丝的免疫反应呈阴性表达。结论先天性巨结肠病变肠段同时缺乏内源性神经支配和外源性神经支配,处于完全失神经支配状态,Cannon定律是先天性巨结肠的病理生理最简单的解释。
Objective To study the relationship between neuromuscular junction in Hirschsprung’s disease and its pathogenesis with Hirschsprung’s disease by immunohistochemistry. Methods Immunohistochemical technique was used to detect the expression of synaptophysin and neurofibrillary neuromuscular junctions in 20 cases of Hirschsprung ’s disease and 10 cases of normal colon tissues. The expression of immunoreactivity was observed under light microscope. Results The immunoreactivity of the synaptophysin and nerve filaments in the control group was strongly positive. The immunoreactivity of synaptophysin and nerve filaments in the segment of bowel in congenital megacolon group was positive or weakly positive. The immunoreactivity of synovial cells and nerve filaments was negatively expressed. Conclusions The intestinal segments of Hirschsprung’s disease lack both endogenous and exogenous innervation and are completely denervated. Cannon’s law is the simplest explanation for the pathophysiology of Hirschsprung’s disease.