【摘要】 目的观察先天性巨结肠(HD)不同节段肠壁肠神经系统(ENS)、平滑肌和肠间质细胞(ICCs)的病理改变及病变范围,探讨HD病理诊断依据及手术切除肠管范围。方法以20例手术切除HD标本为HD组,以11例死于与胃肠道或神经系统无关疾病患儿结肠标本为对照组,分别行肠壁S-100蛋白、α-平滑肌肌动蛋白(α-SMA)和c-kit蛋白免疫组织化学,观察2组ENS、平滑肌和ICCs的分布情况。结果 HD组狭窄段、移行段及扩张段0、2、4、6cm S-100、α-SMA和c-kit阳性表达与对照组相比均显著降低(P<0.05);至扩张段上述指标逐渐增加,在扩张段8、10cm处,三者总体趋于正常。结论 HD切除肠段ENS、平滑肌和ICCs均存在病变,至扩张段8cm处病变缓解,在患儿结肠长度允许的情况下,手术切除范围应达到或超过此范围。更多还原

【Abstract】 Objective To observe the pathological changes and lesion extent in enteric nervous system(ENS),smooth muscle and interstitial cells of Cajal(ICCs)in congenital Hirschsprung’s disease(HD),and to investigate the pathological diagnosis of HD and bowel resection range.Methods The expression of S-100,α-smooth muscle actin(α-SMA)and c-kit was detected by immunohistochemistry in surgically resected colon specimens obtained from 20children who had HD(HD group)and 11children who died of diseases unrelated to gastrointestinal tract or nervous system(control group).Lesion distribution in ENS,smooth muscle and ICCs were observed in both groups.Results Compared with control group,the expression of S-100,α-SMA and c-kit in narrow segment,transitional segment and expansion segment at 0,2,4and 6cm significantly decreased in HD group(P<0.05).However,the expression of S-100,α-SMA and c-kit gradually increased in expansion segment and became normal in expansion segment at 8and 10cm in HD group.Conclusion Lesions exist in ENS,smooth muscle and ICCs of the resected bowel in HD,but are relieved in expansion segment at 8cm.Therefore,the length of intestinal resection should be equal to or greater than 8cm in expansion segment if conditions can be met.更多还原