116例先天性巨结肠的几种免疫组化结果分析

Hirschsprung's disease: an immunohistochemical study of 116 cases

目的 探讨是否存在能准确识别先天性巨结肠病变肠段神经节细胞的特异性抗体。方法 结合HE染色、普通光镜下肠壁神经节细胞的形态特征 ,并应用免疫组化技术对 1 31例临床拟诊为HD的病变肠段行组织蛋白酶D(cathepsinD)、NSE、S 1 0 0蛋白染色 ,比较它们的染色结果和分布特点。结果 光镜下有 1 1 6例确诊为HD ,其肠壁神经丛cathepsinD表达阴性。余 1 5例肠壁神经丛内的个别细胞虽不具备神经节细胞的典型形态特征 ,但cahep sinD表达阳性。而NSE、S 1 0 0只在神经纤维或神经鞘膜 (施万 )细胞及周围细胞中表达阳性。结论 在肠壁神经丛中 ,cathepsinD只标记神经节细胞 ,具有较强的特异性和敏感性 ,故可作为一种辅助诊断方法 。

Objective To explore whether there was a specific marker for distinguishing ganglion cells from the aganglionic segment of the bowel in Hirschsprung's disease (HD). Methods Clinicopathologic data were reviewed and immunohistochemical staining was performed for cathepsin D, neuron specific enolase (NSE) and S 100 protein (S 100) using paraffin-embedded tissues from 131 cases of suspected HD patients. Results Total 116 cases of HD were diagnosed with histological confirmation and immunohistochemical staining showing cathepsin D negative. However, a few of ganglion cells were intensely positive for cathepsin D in other 15 cases of suspected HD. The nerve fibers, Schwann cells and perineural satellite cells showed immunoactivities for NSE and S 100. Conclusions Cathepsin D is a promising marker for ganglion cells and can be used in the diagnosis of HD, especially in some suspected cases.