Langer J C, Berezin I, Daniel E E
Department of Surgery, McMaster University, Hamilton, Ontario.
J Pediatr Surg. 1995 Nov;30(11):1535-43. doi: 10.1016/0022-3468(95)90151-5.
Dysfunction of pyloric inhibition has been implicated in the pathophysiology of hypertrophic pyloric stenosis. Normal inhibition likely is mediated by peptidergic enteric nerves and also may involve interstitial cells of Cajal (ICC). The authors used electron microscopy to qualitatively assess these structures in infants with pyloric stenosis and in normal controls. Pyloric muscle strips from five infants with hypertrophic pyloric stenosis, from three normal pediatric organ donors, and from three adults were examined. The following observations were made. (1) Muscle cells were primarily in a proliferative phase in pyloric stenosis and exhibited very few gap junctions between smooth muscle cells or ICC compared with the control specimens. (2) The circular muscle layer in pyloric stenosis was characterized by near absence of large granular vesicle-containing nerve fibers compared with the control specimens. (3) There were fewer nerve cell bodies in the myenteric plexus in pyloric stenosis, and the total number of ganglia was lower than that in control samples. (4) Interstitial cells of Cajal were almost completely absent in patients with hypertrophic pyloric stenosis, but there was a group of cells resembling ICC that was termed ICC-like cells. These cells may represent a failure or delay in the maturation process of the ICC. These findings show that there are significant structural abnormalities of the inhibitory nervous system in hypertrophic pyloric stenosis. The ontogenic origins and functional significance of these results require further investigation.
幽门抑制功能障碍与肥厚性幽门狭窄的病理生理学有关。正常的抑制作用可能由肽能肠神经介导，也可能涉及 Cajal 间质细胞（ICC）。作者使用电子显微镜对肥厚性幽门狭窄婴儿和正常对照者的这些结构进行了定性评估。检查了来自 5 例肥厚性幽门狭窄婴儿、3 例正常儿科器官捐献者和 3 例成年人的幽门肌条。得出以下观察结果。（1）在幽门狭窄中，肌细胞主要处于增殖期，与对照标本相比，平滑肌细胞或 ICC 之间的缝隙连接极少。（2）与对照标本相比，幽门狭窄中的环肌层特征是几乎没有含大颗粒囊泡的神经纤维。（3）幽门狭窄中肌间神经丛中的神经细胞体较少，神经节总数低于对照样本。（4）肥厚性幽门狭窄患者中 Cajal 间质细胞几乎完全缺失，但有一组类似 ICC 的细胞，称为 ICC 样细胞。这些细胞可能代表 ICC 成熟过程的失败或延迟。这些发现表明，肥厚性幽门狭窄中抑制性神经系统存在明显的结构异常。这些结果的发生学起源和功能意义需要进一步研究。
