肠间质细胞和神经生长因子受体及一氧化氮合酶在先天性巨结肠的分布及意义

Distributions of interstitial cells of Cajal,nerve growth factor receptor and nitric oxide synthase in Hirschsprung's disease and their significance

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.

AIM： To investigate the possible roles of interstitial cells of Cajal （ICC）, nerve growth factor receptor （NGFR） and nitric oxide synthase （NOS） in Hirschsprang＇s disease （HD）. METHODS： Immunohistochemistry using the specific polyclonal antibody against c-kit and monoclonal antibody to human NGFR and NADPH diaphorase histochemistry were used to study the distributions of ICC, NGFR and NOS in 10 cases of HD and 8 control cases. RESULTS： ICC was intensively distributed in the surface of circular musculature and around the myenteric plexus and formed a network in normal control colon. NGFR and NOS were abundantly present in the myenteric plexus and in the nerve fibers. In contrast, ICC was scarcely distributed and the network was disrupted, NGFR and NOS were reduced or absent in the myenteric plexus and in the nerve fibers in the againglionic colon of HD. CONCLUSION： These findings suggest the involvement of ICC, NGFR and NOS in the pathophysiology of HD.