Development of interstitial cells of Cajal in a full-term infant without an enteric nervous system
Development of interstitial cells of Cajal in a full-term infant without an enteric nervous system
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DOI：
10.1053/gast.2001.21200
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发表时间：
2001-02-01
期刊：
影响因子：
29.4
通讯作者：
Jacobson, K
中科院分区：
文献类型：
--
作者：
Huizinga, JD;Berezin, I;Jacobson, K
在一个表现为假性肠梗阻的足月婴儿中，研究了人小肠中肠神经系统发育与Cajal间质细胞（ICC）之间的关系。免疫组化显示肠神经和神经节的情况下，但丰富的c-Kit免疫反应与奥尔巴赫的神经丛（ICC-AP）。然而，c-Kit免疫反应与深肌丛（ICC-ICC）和肌间ICC缺席。电子显微镜显示ICC-AP具有正常的超微结构; ICC-AP可见但严重损伤，提示变性。离体肠肌记录显示慢波活动以及对胆碱能刺激的反应。小肠的X线透视检查显示了各种运动模式，包括节律性、传播性收缩。总之，肠神经的完全缺失与正常ICC-ESTA的缺失相关。然而，正常的肌肉组织，包括ICC-AP的网络，允许产生有节奏的，传播的收缩活动，表明存在功能性运动活动。
The relationship between the development of the enteric nervous system and interstitial cells of Cajal (ICC) in the human small intestine was investigated in a full-term infant who presented with intestinal pseudo-obstruction. Immunohistochemistry revealed absence of enteric nerves and ganglia but abundant c-Kit immunoreactivity associated with Auerbach's plexus (ICC-AP). However, c-Kit immunoreactivity associated with the deep muscular plexus (ICC-DMP) and intermuscular ICC was absent. Electron microscopy showed ICC-AP with a normal ultrastructure; ICC-DMP were seen but were severely injured, suggesting degeneration. In vitro recording of intestinal muscle showed slow wave activity as well as response to cholinergic stimulation. Fluoroscopic examination of the small bowel showed a variety of motor patterns, including rhythmic, propagating contractions. In conclusion, total absence of enteric nerves was associated with absence of normal ICC-DMP. However, a normal musculature, including a network of ICC-AP, allowed for generation of rhythmic, propagating contractile activity, suggesting the presence of functional motor activity.