Teitelbaum D H, Caniano D A, Qualman S J
Department of Surgery, Ohio State University College of Medicine, Columbus.
J Pediatr Surg. 1989 Dec;24(12):1271-7. doi: 10.1016/s0022-3468(89)80566-4.
To more clearly define the pathophysiology of Hirschsprung's-associated enterocolitis (HAEC), this study comprehensively evaluates the gastrointestinal tissue of a group of infants with clinical HAEC. A pathologic grading system that demonstrates a progressive sequence of histologic changes specific for HAEC is established. The grading system correlates closely with clinical enterocolitis and may prove to be a useful method for early detection of infants at risk for the development of clinical HAEC. A significant alteration of intestinal mucins with an increase in neutral mucins and a decrease in acidic-sulfomucins was identified in HAEC tissue specimens. Enterocyte-adherent organisms were present in 39% of HAEC tissue specimens. The pathogenesis of HAEC may result from an alteration in intestinal mucins that may allow for the subsequent adherence of enteropathogenic organisms to enterocytes. The enterocyte-adherent organisms have the potential to induce an enterocolitic process and may contribute to both the intestinal and systemic manifestations of HAEC.
为更清晰地界定先天性巨结肠相关小肠结肠炎（HAEC）的病理生理学，本研究全面评估了一组患有临床HAEC的婴儿的胃肠道组织。建立了一种病理分级系统，该系统显示出HAEC特有的组织学变化的渐进序列。该分级系统与临床小肠结肠炎密切相关，可能被证明是早期检测有发生临床HAEC风险婴儿的有用方法。在HAEC组织标本中发现肠道黏蛋白有显著改变，中性黏蛋白增加而酸性硫黏蛋白减少。39%的HAEC组织标本中存在肠细胞黏附性微生物。HAEC的发病机制可能源于肠道黏蛋白的改变，这可能使肠道致病微生物随后黏附于肠细胞。肠细胞黏附性微生物有可能引发小肠结肠炎症过程，并可能导致HAEC的肠道和全身表现。
