CAJ | 학술논문

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、6 cm 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、10 cm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8 cm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。
목적：관찰선천성거결장（HD）불동절단장벽장신경계통（ENS）、평활기화장간질세포（ICCs）적병리개변급병변범위，탐토HD병리진단의거급수술절제장관범위。방법이20례수술절제HD표본위HD조，이11례사우여위장도혹신경계통무관질병환인결장표본위대조조，분별행장벽 S-100단백、α-평활기기동단백（α-SMA）화c-kit단백면역조직화학，관찰2조 ENS、평활기화 ICCs적분포정황。결과 HD조협착단、이행단급확장단0、2、4、6 cm S-100、α-SMA화c-kit양성표체여대조조상비균현저강저（P＜0．05）；지확장단상술지표축점증가，재확장단8、10 cm처，삼자총체추우정상。결론 HD절제장단 ENS、평활기화 ICCs균존재병변，지확장단8 cm처병변완해，재환인결장장도윤허적정황하，수술절제범위응체도혹초과차범위。
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 im-munohistochemistry in surgically resected colon specimens obtained from 20 children who had HD (HD group)and 1 1 children 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 0,2,4 and 6 cm significantly de-creased in HD group(P<0.05).However,the expression of S-100,α-SMA and c-kit gradually in-creased in expansion segment and became normal in expansion segment at 8 and 10 cm 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 8 cm.Therefore,the length of intestinal resection should be equal to or greater than 8 cm in expansion segment if conditions can be met.