Bethell George, Wilkinson David, Fawkner-Corbett David, Mesa Angelica, Shukla Rajeev, Edgar David, Kenny Simon
University of Liverpool Institute of Translational Medicine, Liverpool, United Kingdom; Department of Paediatric Surgery, Alder Hey Children's Hospital NHS Foundation Trust, Liverpool, United Kingdom.
University of Liverpool Institute of Translational Medicine, Liverpool, United Kingdom.
J Pediatr Surg. 2016 Oct;51(10):1581-4. doi: 10.1016/j.jpedsurg.2016.06.006. Epub 2016 Jun 16.
BACKGROUND/PURPOSE: Despite current treatments patients with Hirschsprung's disease (HSCR) suffer significant long-term morbidity. Therefore, there is increasing interest in adjunctive therapies, such as using enteric nervous system stem cells (ENSSC), isolated from typical aganglionic bowel. The source of these cells is unclear however it is hypothesized that they are present in the thickened nerve trunks in aganglionic short and long segment HSCR gut. These cells should therefore be absent in total colonic and pan intestinal HSCR where these thickened fibers are absent.
Cells were isolated from samples of short segment HSCR gut (n=18) and total colonic and total intestinal HSCR gut (n=2). Acetylcholinesterase histochemistry confirmed the presence/absence of thickened nerve trunks. P75 immunofluorescence highlighted ENSSC at isolation and after 10days in culture in both groups.
ENSSC were not isolated or cultured from total colonic and total intestinal HSCR gut where thickened nerve trunks were absent. In contrast 10.0% (+/-1.9 SEM) of cells from short segment HSCR gut were ENSSC at isolation rising to 22.7% (+/-2.9 SEM) after 10days in culture.
These results associate ENSCC with thickened nerve trunks and also suggest that the aganglionic bowel segment in total colonic and intestinal HSCR cannot be used as a source of ENSCC for adjunctive therapy.
背景/目的：尽管有当前的治疗方法，先天性巨结肠症（HSCR）患者仍遭受严重的长期发病。因此，人们对辅助疗法的兴趣与日俱增，例如使用从典型无神经节肠段分离出的肠神经系统干细胞（ENSSC）。然而，这些细胞的来源尚不清楚，不过据推测，它们存在于无神经节短段和长段HSCR肠道的增厚神经干中。因此，在全结肠和全肠道HSCR中，由于不存在这些增厚的纤维，这些细胞应该是缺失的。
从短段HSCR肠道样本（n = 18）以及全结肠和全肠道HSCR肠道样本（n = 2）中分离细胞。乙酰胆碱酯酶组织化学证实增厚神经干的存在与否。P75免疫荧光在两组分离时以及培养10天后突出显示ENSSC。
在不存在增厚神经干的全结肠和全肠道HSCR肠道中未分离或培养出ENSSC。相比之下，短段HSCR肠道分离时10.0%（±1.9 SEM）的细胞为ENSSC，培养10天后升至22.7%（±2.9 SEM）。
这些结果将ENSSC与增厚神经干联系起来，也表明全结肠和全肠道HSCR中的无神经节肠段不能用作辅助治疗的ENSSC来源。
