Shaw P A
Department of Histopathology, Leicester Royal Infirmary, UK.
Histopathology. 1990 Aug;17(2):117-21. doi: 10.1111/j.1365-2559.1990.tb00681.x.
There is little published information on changes in the appendix in Hirschsprung's disease despite the presence within the mucosa of nerves and neuroendocrine cells together forming a 'neuroendocrine ganglion'. The innervation and neuroendocrine cell population of 11 appendices (two from patients with total colonic aganglionosis, six from patients with short segment Hirschsprung's disease and three normal controls) were examined using H & E, a standard Grimelius silver stain and immunocytochemistry for S-100 protein, neuron-specific enolase, neurofilament and synaptophysin. Compared with both normal and short segment Hirschsprung's disease controls, the total colonic aganglionosis appendices showed decreased numbers of nerve bundles around the muscle layers and within the submucosa, markedly decreased numbers of smaller fibres within the circular muscle and absence of the finer fibres within the lamina propria. There was no change in the overall number of epithelial neuroendocrine cells but subepithelial neuroendocrine cells, normally found in association with mucosal nerve fibres, were totally absent. The concomitant loss of nerve fibres and neuroendocrine cells from the lamina propria in total colonic aganglionosis suggests a different embryological origin for epithelial and sub-epithelial neuroendocrine cells.
尽管在先天性巨结肠症患者的黏膜内存在共同形成“神经内分泌神经节”的神经和神经内分泌细胞，但关于该疾病中阑尾变化的已发表信息却很少。使用苏木精和伊红染色（H&E）、标准的格里米利乌斯银染色以及针对S-100蛋白、神经元特异性烯醇化酶、神经丝和突触素的免疫细胞化学方法，对11个阑尾（2个来自全结肠无神经节细胞症患者，6个来自短段先天性巨结肠症患者，3个为正常对照）的神经支配和神经内分泌细胞群进行了检查。与正常和短段先天性巨结肠症对照相比，全结肠无神经节细胞症的阑尾显示肌层周围和黏膜下层的神经束数量减少，环行肌内较细纤维数量明显减少，固有层内无细纤维。上皮神经内分泌细胞总数没有变化，但通常与黏膜神经纤维相关的上皮下神经内分泌细胞完全缺失。全结肠无神经节细胞症中固有层神经纤维和神经内分泌细胞的同时缺失表明上皮和上皮下神经内分泌细胞有不同的胚胎起源。
