摘要
目的 观察先天性巨结肠症 (HD)患者肠壁中细胞外基质成分———Ⅳ型胶原 (ColⅣ )及其降解酶 基质金属蛋白酶 2 (MMP 2 )的分布 ,探讨肠壁微环境改变与HD发病机制间的关系。方法 采用SABC免疫组化法 ,分别观察 37例HD患者的无神经节细胞、有神经节细胞及近端正常肠组织中ColⅣ和MMP 2的分布。结果 无神经节细胞肠壁ColⅣ的含量高于有神经节细胞段和正常段肠组织 ,差异有显著性意义 ,χ2 分别为 13.2 1和 14 .96 ,P值均为 0 .0 0 0 ;无神经节细胞肠壁中MMP 2含量也显著高于其他节段 ,χ2 分别为 6 .2 9和 5 .0 9,P值为 0 .0 12和 0 .0 2 4 ;有神经节细胞段与正常段ColⅣ和MMP 2的分布基本一致 ,没有明显差别。结论 肠壁微环境的异常在HD发病中充当了重要角色 ,其中细胞外基质成分ColⅣ及其降解酶MMP 2在无神经节细胞肠段中的异常增多提示异常的微环境可能影响了神经嵴母细胞的肠内迁移、定位及其后的分化和成熟 ,从而导致HD。
Objective To observe the distribution of extracellular matrix component, collagen(Col Ⅳ) and its proteinase, matrix metalloproteinase 2 (MMP 2) in Hirschsprung's disease (HD), and explore the relationship between microenvironmental change of intestinal wall and pathogenesis of HD. Methods SABC complex immunohistochemical method was utilized to visualize the distribution of collagen and MMP 2 in aganglionic, ganglionic and proximal segments from 37 HD patients. Results Col immunoreactivity was markedly increased in the aganglionic segments as compared with the ganglionic and normal segments ( χ 2 = 13.211 and 14.957 respectively, P = 0.000 ). Staining intensity of MMP 2 in aganglionic segments was also stronger than those in the other two segments ( χ 2 = 6.288 and 5.089 , P = 0.012 and 0.024 respectively). But the distribution of Col and MMP 2 in ganglionic and normal bowel segments of HD patients were approximately the same. Conclusions Anomalies of microenvironment in bowel wall play an important role in the development of HD. The remarkable increase of collagen (a component of extracellular matrix) and its proteinase, MMP 2 in aganglionic bowel segments indicate that the microenvironmental anomalies may cause HD by affecting migration, colonization, differentiation and maturation of the neural crest derived neuroblasts.
出处
《中华小儿外科杂志》
CSCD
北大核心
2003年第2期113-115,共3页
Chinese Journal of Pediatric Surgery
关键词
先天性巨结肠
免疫组织化学
细胞外基质
胶原
金属蛋白酶类
神经节细胞学
Hirschsprung's disease,congenital
Immunohistochemistry, methods
Extracellular matrix
Collagen
Metalloproteinases
Ganglia, cytology