【摘要】 目的 观察S-100蛋白(S-100)和组织蛋白酶D(cathepsin D，CAD)在先天性巨结肠(Hirschsprung's disease，HD)中的表达。 方法 应用免疫组织化学染色观察25例HD患儿及10例对照组儿童结肠。 结果 (1)在正常对照组与HD扩张段组肠壁肌间和黏膜下层可见CAD只对神经丛中神经节细胞阳性表达，对神经纤维与神经胶质细胞均不表达。S-100染色与CAD染色相反，神经纤维与神经胶质细胞均阳性表达，神经节细胞阴性表达，表现为阳性神经丛中细胞状“空白区”。(2)在HD狭窄段组肠壁神经丛中缺乏CAD阳性表达。S-100染色神经丛中细胞状“空白区”未观察到;S-100强阳性表达的神经纤维束显著增生，扭曲呈波浪状。 结论 S-100和CAD的免疫组化染色对诊断HD具有重要意义。

    关键词 先天性巨结肠 组织蛋白酶D S-100蛋白

    Application of S-100protein and cathepsin D in diagnosis of Hirschsprung's disease

    Chen Weijian，Zhou Xiaoyu，Li Liping，et al.

    Hunan Provincial Children's Hospital，Changsha410007

    【Abstract】 Objective To observe the expression of S-100protein(S-100)and cathepsin D(CAD)in the colon of patients with Hirschsprung's disease(HD).Methods Colons from25children with HD and10controls were stained for S-100and CAD by immunohistochemistry.Results (1)In the controls and the dilated intestine segment of the HD，CAD-positive ganglionic cells were observed in myenteric and submucosal plexus.S-100positive nerve fibers and gliocyte stained were negative to CAD.The ganglionic cells-like blank area in the plexus stained were negative to S-100.(2)In the stenotic segment of HD ......