先天性巨结肠与巨结肠同源病的Ret蛋白免疫组织化学研究

目的了解Ret蛋白在先天性巨结肠和具结肠同源病肠组织中的表达,并进一步研究Ret蛋白在先天性巨结肠和巨结肠同源病发病中的作用。方法采用鼠抗人Ret单克隆抗体通过免疫组化SABC法对15例先天性巨结肠,11例巨结肠同源病肠组织Ret蛋白的表达进行研究,10例正常结肠组织作对照。结果Ret蛋白在先天性巨结肠和巨结肠同源病的扩张段,对照组均呈现阳性反应（P〉0．05）,在先天性巨结肠狭窄段大多数表现为阴性反应,极少数出现阳性反应,而巨结肠同源病狭窄段可见Ret免疫反应蛋白阳性细胞,而且偶见巨大的阳性细胞;先天性巨结肠狭窄段分别与巨结肠同源病狭窄段和对照组比较均有统计学意义（P〈0．001）。结论Ret蛋白对先天性巨结肠的发生有重要作用,而与巨结肠同源病的发生无明确关系。

Immunohistochemical study of RET protein in Hirschsprung's disease and allied Hirschsprung's disorder

OBJECTIVE: Hirschsprung's Disease (HD) and allied Hirschsprung's disorder (HAD) have very similar clinical manifestations, but there are many different theories about the two diseases. The present study was designed to understand the expression of Ret protein in HD and HAD, and to explore the role of Ret protein in the pathogenesis of HD and HAD. METHODS: Colon specimens from patients with confirmed HD and HAD, including 15 cases of HD (male 12, female 3) and 11 cases of HAD (male 8, female 3) were collected for this study. At the same time normal colon specimens from 10 individuals with other diseases were used as control. The expression of Ret protein in the intestinal tissue was detected by using immunohistochemical SABC technique with mouse anti-Ret monoclonal antibody. RESULTS: In the colon specimens from normal controls and the dilated segments of colon from HD and HAD patients, moderate to large number of Ret-positive cells were observed among the ganglion cells of myenteric plexuses and submucosal plexuses (P > 0.05). On the contrary, Ret-positive cells were not seen in the stenotic segment of colon from HD patients. But there was positive staining in the stenotic segment of the colon from HAD. Moreover, giant ganglion cells showing strongly positive staining could be seen. There were also displastic cells, small cells, and cells with irregular shape. Statistical analysis showed significant differences in Ret cells positivity between the stenotic segment of colon of HD and the normal control (P < 0.001) as well as between HD and HAD (P < 0.001). CONCLUSION: Ret protein may play an important role in the pathogenesis of HD and could not have definite relationship with HAD.