S-100蛋白及Cathepsin D在先天性巨结肠诊断中应用.docVIP

S-100蛋白及Cathepsin D在先天性巨结肠诊断中应用 　　作者：陈卫坚，周小渔，李梨平，贺新玉，范丽明【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（Hirschsprungs disease，HD）中的表达。; 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-100 protein and cathepsin D in diagnosis of Hirschsprungs disease 　　 　　【Abstract】; Objective; To observe the expression of S-100 protein(S-100) and cathepsin D(CAD) in the colon of patients with Hirschsprungs disease(HD).Methods; Colons from 25 children with HD and 10 controls were stained for S-100 and 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-100 positive nerve fibers and gliocyte stained were negative to CAD. ganglionic cells-like blank area in the plexus stained were negative to S-100. (2) In the stenotic segment of HD，neither CAD-positive neurons nor cell-like blank area in S-100 stained were found in the plexus.S-100 positive fibers were proliferated obviously; and wave-like in their appearance.Conclusion; The expression of S-100 and CAD immunohistochemistry-staining has an important significance to the confirmed diagnosis of HD. ; 【Key words】; Hirschsprung’s disease; cathepsin D; S-100 protein ; 先天性巨结肠（Hirschsprungs disease，HD）是小儿外科常见的消化道畸形，主要病理改变是远端病变段肠管神经节细胞缺如。在HD的病理诊断中，有部分病例，尤其是新生儿病例，由于肠壁神经系统发育不完全，神经节细胞欠成熟，形态不典型，与神经丛中神经胶质细胞难以鉴别。而且多数患儿并发肠道感染，肠壁内有较多炎细胞浸润，使HE切片中神经节细胞难以辨认。这些原因均可造成HD不易明确诊断。本应用免疫组织化学（免疫组化）方法，观察正常肠壁、HD扩张段和HD狭窄段肠壁神经组织中S-100蛋白（S-100）和组织蛋白酶D（cathepsin D，CAD）的表达结果，加以，以明确诊断HD。 ; 1; 资料与方法 ; 1.1; 一般资料; 回顾我院2002年6月～2004年6月临床拟诊断为HD的手术切除标本25例。男22例，女3例，年龄1个月～4岁，其中≤3个月10 例，gt;3个月15例，均经术后病理检查证实为HD，23例为常见型，2例为长段型。每例标本分别取近端扩张段切缘肠壁作为HD扩张段组，取远端狭窄段肠壁作为HD狭窄段组。10例横结肠或乙