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目的通过免疫组织化学染色研究不同神经标志物在全结肠型无神经节细胞症(TCA)和常见型巨结肠(HD)肠壁内表达的差异。方法复旦大学附属儿科医院1996年1月～2005年12月间收治TCA确诊病例18例,收集结肠、回肠全层标本。对照组为常见型HD及肛门直肠畸形各10例。采用免疫组织化学染色技术对肠壁内神经标记物:S-100蛋白、外周蛋白、蛋白基因产物9.5(PGP9.5)、神经元特异性烯醇化酶(NSE)的不同程度的表达进行比较。数据采用t检验分析。结果PGP9.5、S- 100蛋白、NSE和外周蛋白在对照组和TCA组、HD组近端肠壁内表达差异无统计学意义(P>0.05);移行段内可见少数成熟和不成熟神经节细胞,神经标志物表达低于HD组(P<0.01);18例TCA远端肠壁内均无神经节细胞,粗大的神经干较HD少见,神经标志物表达明显低于HD组(P<0.01),以PGP9.5最明显。结论研究表明TCA和常见型HD病变肠段中存在明显的神经标记物阳性表达差异,提示全结肠巨结肠肠壁内神经支配异常不同与常见型巨结肠。
OBJECTIVE: To investigate the differences in the expression of different neural markers in the whole colon of ganglion cell disorder (TCA) and common type of giant colon (HD) intestinal wall by immunohistochemical staining. Methods Fetal pediatric hospital of Fudan University from January 1996 to December 2005 were diagnosed in 18 cases of TCA, collected colon, ileum full-thickness specimens. The control group of common HD and anorectal malformation in 10 cases. Immunohistochemical staining was used to compare the expression of neuronal markers S-100, peripherin, protein gene product 9.5 (PGP9.5) and neuron-specific enolase (NSE) in intestinal wall. Data were analyzed using t-test. Results There was no significant difference in the expressions of PGP9.5, S-100 protein, NSE and periplasmic proteins between the control group and TCA group and HD group (P> 0.05). A few mature and immature nerves (P <0.01). There were no ganglion cells in the distal intestinal wall of 18 patients with TCA. The thicker neural stem cells were rarer than HD and the expression of neural markers was significantly lower than that of HD group (P <0.01) <0.01), with PGP9.5 the most obvious. Conclusions The study shows that there is a significant difference in the positive expression of neurological markers in the intestinal segments of TCA and common HD lesions, suggesting that there is a difference in neural innervation between the colon and colon of the common colon and the common type of megacolon.