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目的应用两种神经肌肉接头的重要标记物———突触导素(SY)和神经细胞粘附分子(NCAM),对肠神经元发育异常症神经肌肉接头分布模式进行免疫组化的研究。方法分别取30例先天性无神经节细胞症(HD)、3例肠神经节发育不良(NID)、2例肠神经节细胞减少症(HYPG)及1例神经节细胞未发育(IMG)的病变肠壁全层作为病变组,取其正常的结肠壁全层作为对照组,采用免疫组化的方法对这两组肠壁的SY及NCAM分布方式进行光镜检查。结果正常组粘膜下神经丛和肌间神经丛有大量SY和少量NCAM染色的神经纤维,粘膜肌层、环肌和纵肌层有丰富的突触。HD病人组粘膜肌层、环肌和纵肌层SY染色的神经纤维及突触数量在狭窄段则明显稀少,NCAM染色的神经纤维数量明显增多;3例NID中,1例的粘膜肌层、环肌和纵肌层SY染色神经纤维数量减少明显,1例纵肌层和1例粘膜肌层无SY染色活性;在神经节细胞减少症组的肌间丛和肌层中只有少数神经肌肉接头。结论神经肌肉接头在肠神经元发育异常症中呈异常分布可能是肠神经发育异常症重要的发病机制,并可能是此类疾病肠运动障碍的原因之一。
OBJECTIVE: To study the distribution of neuromuscular junctions in intestinal neuronal dysplasia using immunohistochemistry, an important marker of neuromuscular junction, synaptic hormone (SY) and neural cell adhesion molecule (NCAM). Methods Thirty cases of congenital ganglion cell disorder (HD), three cases of idiopathic ganglia dysplasia (NID), two cases of enteric ganglion cell hypopnosis (HYPG) and one case of unmethylated ganglion cells (IMG) Lesions of the full-thickness as the lesion group, take the normal colon wall as the control group, using immunohistochemical methods of these two groups of intestinal wall SY and NCAM distribution of light microscopy. Results In normal group, submucosal plexus and myenteric plexus had abundant SY and few NCAM-stained nerve fibers. The mucosal muscular layer, the muscular ring of the mucosa, and the longitudinal muscle layer had abundant synapses. The number of SY staining nerve fibers and synapses in mucosal muscle, ring muscle and longitudinal muscle layer of HD patients was significantly less in the stenotic segment and the number of NCAM-stained nerve fibers was significantly increased. In 3 of the NIDs, 1 mucosal muscular layer, There was no significant difference in the numbers of SY stained nerve fibers in the ring muscle and the longitudinal muscle. There was no SY staining activity in one of the longitudinal and muscular layers of the muscular layer and only a few neuromuscular junctions in the myenteric plexus and muscular layer of the gangliosia group . Conclusion The abnormal distribution of neuromuscular junction in the development of intestinal neuronal dysplasia may be an important pathogenesis of intestinal neurodevelopmental dysfunction and may be one of the causes of intestinal motility disorders of such diseases.