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目的:探讨肠间质细胞(ICC)、神经生长因子受体(NGFR)及一氧化氮合酶(NOS)与先天性巨结肠(HD)发病的关系.方法:应用c-kit及NGFR免疫组化法和NADPH黄递酶组化法观察10例HD患者病变结肠及8例对照组结肠ICC,NGFR及NOS的分布.结果:ICC密布于对照组结肠环肌浅表层及肌间神经丛周围并相互交织成网状结构,肠壁内含丰富的NGFR及NOS阳性神经节细胞和神经纤维.HD病变肠壁内ICC数量减少、网状结构破坏,缺乏NGFR及NOS阳性神经节细胞和神经纤维.结论:ICC,NGFR及NOS的异常分布可能与HD无神经节细胞结肠的舒张功能障碍有关.
Objective: To investigate the relationship between intestinal interstitial cells (ICC), nerve growth factor receptor (NGFR) and nitric oxide synthase (NOS) and the pathogenesis of Hirschsprung’s disease.Methods: And NADPH diaphorase histochemistry were used to observe the distribution of ICC, NGFR and NOS in colonic mucosa of 10 cases of HD patients and 8 cases of control group.Results: The ICC was densely distributed in the superficial layers of the colon and the myenteric plexus of the control group Intertwined into a network structure, the intestine wall is rich in NGFR and NOS-positive ganglion cells and nerve fibers.HD lesions in the intestinal wall ICC number decreased, the destruction of the network structure, the lack of NGFR and NOS-positive ganglion cells and nerve fibers. Conclusion: The abnormal distribution of ICC, NGFR and NOS may be related to the diastolic dysfunction of colon of HD ganglion cell.