Colonic dysmotility in postsurgical patients with Hirschsprung's disease. Potential significance of abnormalities in the interstitial cells of Cajal and the enteric nervous system
Colonic dysmotility in postsurgical patients with Hirschsprung's disease. Potential significance of abnormalities in the interstitial cells of Cajal and the enteric nervous system
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DOI：
10.1016/j.jpedsurg.2007.10.067
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发表时间：
2008-08-01
影响因子：
2.4
通讯作者：
Rubin, Steven
中科院分区：
文献类型：
--
作者：
Bettolli, Marcos;De Carli, Claudio;Rubin, Steven
目的：正常的肠肌功能依赖于肠神经系统（ENS）和Cajal间质细胞（ICC）的协调活动。巨结肠病（HD）一直被认为是一种纯粹的神经元缺陷，但最近的数据表明，近端神经节结HD结肠的ICC异常。我们检查了HD患者近端神经节结结肠中ICC和神经元细胞的标记，以确定ICC和ENS的异常是否可能与不良的临床结果相关。方法：采用免疫组织化学方法对HD患者的组织进行ICC和神经元鉴定，并与非HID患者的对照组织进行比较。对图像数据进行定量评估，并根据临床结果进行解释。结果：HD组神经节结结肠Cajal间质细胞与对照组无明显差异，但神经细胞/纤维减少40%。个别患者的神经纤维和ICC的成对减少与正常的肠功能有关。在神经支配正常但神经节结结肠ICC明显下降的患者中，观察到术后预后较差。结论：在HD患者中，近端神经节结结肠的神经纤维减少，但不伴有肠道运动障碍。只有神经支配正常且ICC明显降低的患者才会出现不良的临床结果。收集大量临床结果不佳的患者的数据对于确定ICC和神经支配失衡的意义是必要的。(C) 2008爱思唯尔公司版权所有。
Purpose: Normal gut muscular function depends on the coordinated activity of both the enteric nervous System (ENS) and the interstitial cells of Cajal (ICC). Hirschsprung's disease (HD) has long been considered a purely neuronal deficit but recent data point to abnormalities in ICC in the proximal ganglionated HD colon. We examined the labeling of ICC and neuronal cells in the proximal ganglionated colon in patients with HD to determine whether abnormalities of ICC and ENS might be associated with a poor clinical Outcome.Methods: Tissue from I I patients with HD was studied using immunohistochemistry for ICC and neuronal identification in comparison to control tissue from patients without HID. Image data were evaluated quantitatively and interpreted relative to clinical outcome.Results: Interstitial cells of Cajal in the ganglionated colon of the HD group did not differ from the control group, but nerve cells/fibers were decreased 40%. Paired decreases in both nerve fibers and ICC in individual patients were associated with normal bowel function. Poor postoperative outcome was observed in a patient with normal innervation but with a profound decrease in ICC in the ganglionated colon.Conclusions: Nerve fibers are decreased in the proximal ganglionated colon in patients with HD without associated gut dysmotility. Poor clinical outcome was noted only in a patient with normal innervation and markedly decreased ICC. Collection of data from a much larger number of patients with poor clinical outcome will be necessary to determine the significance of this imbalance of ICC and innervation. (C) 2008 Elsevier Inc. All rights reserved.