远端消化道（包括肛门括约肌）内神经节细胞的先天性缺乏所引起的功能紊乱是先天性巨结肠一系列临床病理表现的症结所在，然而神经节细胞的缺乏如何导致受累区肠断的持续收缩，其可能机制尚未被普遍接受。过去10年里由于药物学、电生理学和免疫组织化学的发展，人们发现除肾上 Dysfunction caused by congenital deficiency of ganglion cells in distal digestive tract (including the anal sphincter) is the crux of a series of clinicopathological manifestations of Hirschsprung’s disease, however, how the lack of ganglion cells leads to a sustained contraction of the bowel in the affected area , Its possible mechanism has not been generally accepted. In the past 10 years because of the development of pharmacology, electrophysiology and immunohistochemistry, it was found that in addition to the kidney