Kobayashi H, Hirakawa H, Puri P
Children's Research Centre, Our Lady's Hospital for Sick Children, Dublin, Ireland.
J Pediatr Surg. 1995 Dec;30(12):1680-3. doi: 10.1016/0022-3468(95)90451-4.
The pathogenesis of Hirschsprung's disease (HD) is not fully understood. The authors studied expression of intercellular adhesion molecule-1 (ICAM-1) and major histocompatibility complex (MHC) class II antigen in the resected bowel specimens of 18 patients with HD who had no evidence of enterocolitis and in eight age- and site-matched controls, using indirect immunohistochemistry. There was strong expression of ICAM-1 and MHC class II antigen on hypertrophic nerve trunks, in both the submucous and myenteric plexuses of the aganglionic colon. The transition zone showed strong expression of ICAM-1 and MHC class II antigen on small ganglia in the myenteric and submucous plexuses. However, no staining of ganglia or nerve fibers was found in the submucous and myenteric plexuses of the colon from controls or in the ganglionic colon from patients with HD. The expression of both antigens on hypertrophic nerve trunks suggests the presence of an immunologic response in the pathogenesis of HD.
先天性巨结肠（HD）的发病机制尚未完全明确。作者采用间接免疫组织化学方法，研究了18例无小肠结肠炎证据的HD患者以及8例年龄和部位匹配的对照组患者的切除肠段标本中细胞间黏附分子-1（ICAM-1）和主要组织相容性复合体（MHC）II类抗原的表达情况。在无神经节结肠的黏膜下和肌间神经丛中，肥大的神经干上ICAM-1和MHC II类抗原呈强表达。过渡区在肌间和黏膜下神经丛的小神经节上ICAM-1和MHC II类抗原呈强表达。然而，在对照组结肠的黏膜下和肌间神经丛或HD患者的有神经节结肠中未发现神经节或神经纤维染色。两种抗原在肥大神经干上的表达提示在HD发病机制中存在免疫反应。
