先天性巨结肠免疫组织化学研究

为观察神经标志物PGP9．5和S－100蛋白在先天性巨结肠（HD）中的表达,采用一抗为抗PGP9．5和抗S－100蛋白的PAP免疫组织化学方法,探讨其在临床诊断中的意义。结果：（1）在对照组结肠壁神经丛中可见染色深浅不一的PGP9．5免疫反应阳性神经节细胞,神经纤维均匀分布在肠壁各层;神经节细胞胞体不表达S-100蛋白,表现为细胞状“空白区”。（2）HD结肠壁神经发育异常,PGP9．5和S－100蛋白免疫反应性神经纤维明显增生,分布紊乱,未见有PGP9．5阳性神经节细胞;在神经丛中,增生的S－100蛋白阳性神经纤维中偶见有细胞状的“空白”区。提示结肠壁神经发育异常是HD的主要病理生理变化,神经丛中PGP9．5阳性反应的细胞团块和S－100蛋白染色的神经丛中细胞状“空白”区,可特征性地提示神经节细胞的存在,用于HD的临床诊断敏感度高。

Immunohistochemistry study on Hirschsprung' s disease (HD) observations on the expression of PGP 9.5 and S-100 protein as well as their significance in the clinical diagnosis

To observe the expression of PGP 9.5 and S-100 protein (the neurogenic markers) in the patients with Hirschsprung's disease and their significance in clinical diagnosis , anti-PGP 9.5 and anti-S-100 protein were used as the first antibody to do the PAP immunohistochemical staining. The results showed as following: (1) The PGP 9.5 immunohisto-chemical positive staining neuroganglion cells with heterogeneous color could be found in the neuroplexus of the colon wall in the control group with neurofiber distributed uniformly in the different layers of the colon wall. As the cell body of the neuroganglion cells did not express S-100 protein. They left a "cell-form blank zone". (2) In patients with HD , the abnormal neuron development in the colon wall could be observed with PGP 9. 5 and S-100 protein positive fibers proliferated markedly and disturbance in distribution. But, it is hard to see any PGP 9.5 positive neuroganglion cells with the cell-form blank zone occasionally seen in the proliferated S-100 protein neurofibers of the neuroplexus, showing the abnormal neuron development in the colon wall is the main pathophysiologic changes in patients with HD.The PGP 9.5 positive cell mass and S-100 protein staining cell-form blank zone in the neuroplexus existed with charasteristic of suggesting the existence of the neuroganlion ells indicating this method has high degree of sensitivity in the clinical diagnosis of the patients with HD.