先天性巨结肠神经肌肉连接免疫组织化学研究

Immunohistochemical study of the neuromuscular junction in birschsprung's disease

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 colonic neuromuscular ]unction in of the in patients with Hirschsprung＇ s disease. Methods The distribution of the neuromuscular junction in the muscle layers of bowel from 10 normal controls and 20 patients with Hirschsprung＇s disease were imrnunostained for synaptophysin（SY） and neurofilament（NF）： Anti-mouse synaptophysin and neurofilament serum were applied to identify the neuromuscular junction. Results In the control group, the immunoreactivities of SY and NF were widely distributed in the circular muscle layer. In the dilated colon with Hirschsprung＇s disease, the immunoreactivities of SY and NF were positive and moderately positive. In the aganglionic segment, the immunoreactivities of both SY and NF were absent. Conclusions Poor innervation of the colonic muscles in Hirschsprung＇s disease results in muscle spasm and poor peristalsis.