In order to investigatde the pathogenesis of Hirschsprung disease(HD)and Hirschsprung associated enterocolitis(HAEC),the human antisera IgG immunofluorscent reaction and the gross changes of mucosa of 11 HD cases were observed.The specimens of 10 cases taken from bowel resections and one autopsy case that had died of HAEC soon after admission.The sections were chosen immediately from the middle part of the proximal,transitional and distal segment.The results demonstrate that:(1)the lamina propria of transitional and narrow segment,infiltrated with inflammation cells,showed an increase of IgG contained cells,(2)at the transitional part,the IgG immunofluorscence obviously attached to both sides of the submucosal collagen fibrils,muscularis mucosa,longitudinal and circular muscles.Even the humman antisera was diluted from 1∶16 to 1∶60,the flucorscence background still kept quite clear,(3)in accordance with the distribution of the myenteric plexuses,the mucosal fold patterns at the proximal and transitional part changed from circuit to oblique.and localized to the narrow segment like a funnel while the arrangement of the folds varied from regularity to disorder simultaneously.The authors consider the possibility of an immunological mechanism underlying the pathogenesis of HD and HEAC should be intensively investigated.These mucosal fold features are important to the pathological,clinical and fibercolon scope examination.%目前国内外对先天性巨结肠的病理生理、组织化学及电镜等方面的研究多,但导致其肠壁肌间神经丛缺如及肠结肠炎的原因尚不清楚,为此,我院于1989年11月起对11例先天性巨结肠婴儿的结肠粘膜病变,及抗人体IgG免疫萤光进行观察.
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