外周蛋白和组织蛋白酶D在先天性巨结肠症中的表达

目的:观察外周蛋白(peripherin，PR)和组织蛋白酶D(cathepsin D，CD)在Hirschsprung症(HD)肠壁中的表达并探讨其诊断价值。方法：应用免疫组织化学染色观察9例HD及5例对照组患儿结肠。结果：在正常结肠肠壁内有CD或PR阳性神经节细胞，肠壁各层均有PR阳性神经纤维，但无CD阳性神经纤维染色。在无神经节细胞肠段的肠壁内缺乏CD或PR阳性神经元，亦无CD阳性神经纤维染色，肌层和黏膜下层内PR阳性神经纤维明显减少或缺如。结论：CD和PR是肠神经节细胞特异性较高的标志物，二者的免疫组织化学染色将有助于HD及其同源病的诊断。

Expression of peripherin and cathepsin D and its clinical significance in Hirschsprung's disease

Objective To observe the expression of peripherin (PR) and cathepsin D (CD) in the colons of patients with Hirschsprung's disease (HD) and to evaluate their diagnostic values in HD.Methods Colons from 9 children with HD and 5 controls were stained for PR and CD by immunohistochemistry.Results In ganglionic colon,CD or PR-positive ganglionic cells were observed in the myenteric and submucosal plexuses. The PR-positive nerves were abundant in all layers while the CD-positive nerves were not evident. In aganglionic specimens,neither the CD and PR-positive neurons nor the CD-positive nerves were noted. The PR-positive fibers were remarkably decreased or absent in the myenteric and submucosal plexuses.Conclusions Both CD and PR are relatively specific markers for intestinal ganglionic cells,the changes of their immunoreactive staining might be helpful in the diagnosis of HD and HD allied diseases.