先天性巨结肠并发小肠结肠炎肠道免疫功能的变化

Mucosal Immune Defence in Enterocolitis Complicating with Hirschsprung's Disease

目的探讨ＨＤ并发小肠结肠炎的发病原因和发病机理。方法利用放射免疫技术及免疫组织化学方法（ＳＡＢＣ）检测ＨＤ患儿肠粘膜免疫功能。结果１３例ＨＤ患儿中，５例伴有小肠结肠炎患儿与８例无小肠结肠炎患儿相比肠腔内ＳＩｇＡ浓度明显降低，肠粘膜淋巴细胞转化能力减弱，固有层中含ＩｇＡ的浆细胞明显增多，ＣＤ４＋、ＣＤ８＋细胞分布异常。结论ＨＤ伴有小肠结肠炎患儿肠粘膜免疫功能受到抑制，多聚ＩｇＡ不能通过上皮细胞转运到肠粘膜表面形成ＳＩｇＡ，若远端肠管狭窄，粪便淤积，细菌和病毒可侵入肠粘膜大量繁殖，引起肠道炎症，进一步导致粘膜的破坏，粘膜免疫功能更加下降，形成恶性循环。

Objective To investigate the aetiology and the pathogenesis of enterocolitis complicated with Hirschsprung's disease.Methods Mucosal immune defence of colonic specimens from patients of Hirschsprung's disease was studied by immunohistochemical and radioimmunological methods.Results Compared to the 8 patients of Hirschsprung's disease without enterocolitis, five cases with Hirschsprung's disease and enterocolitis had markedly deficient transfer of IgA across distribution of CD4 and CD8 positive cells in lamina propria.Conclusions Patients of Hirschsprung's disease complicated with enterocolitis have compromised local mucosal immune defence. Stasis of fecal content,multiplication of bacteria and endotoxin production further damage the mucosa, leading to deterioration of mucosal immune mechanism. This may be a possible complex mechanism of Hirschsprung's disease and enterocolitis.