Pan-enteric neuropathy and dysmotility are present in a mouse model of short-segment Hirschsprung disease and may contribute to post-pullthrough morbidity.
Pan-enteric neuropathy and dysmotility are present in a mouse model of short-segment Hirschsprung disease and may contribute to post-pullthrough morbidity.
复制标题
全肠神经病变和动力障碍存在于短节段先天性巨结肠病小鼠模型中，并可能导致牵拉后发病率。
DOI：
10.1016/j.jpedsurg.2020.04.002
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发表时间：
2021-03
影响因子：
2.4
通讯作者：
Goldstein AM
中科院分区：
文献类型：
--
作者：
Bhave S;Arciero E;Baker C;Ho WL;Guyer RA;Hotta R;Goldstein AM
巨结肠病（HSCR）以远端肠神经节病为特征。虽然手术可以挽救生命，但胃肠道运动障碍仍然存在于许多患者中。我们的目的是确定肠神经系统（ENS）异常是否存在于胃肠道神经节区远近端的神经节部分，以及这些异常是否与胃肠道运动障碍有关。采用ednrb缺失小鼠（HSCR模型）进行免疫组化分析，定量评价近端结肠、小肠和胃的ENS结构。在体内测量胃排空和肠道运输，在体外通过时空制图评估小肠和大肠收缩性。HSCR小鼠近端结肠神经节较小，神经元纤维密度降低，迁移运动复合体明显减少。远端小肠神经节明显减少，神经元纤维密度下降，这与小肠转运时间延迟有关。最后，在HSCR小鼠胃中，肠道神经元堆积密度增加，胃排空速度加快。在ednrb缺陷小鼠中，整个胃肠道神经节部分存在ENS异常和运动缺陷。这也许可以解释HSCR拉通手术后经常发生的胃肠道疾病。
Hirschsprung disease (HSCR) is characterized by distal intestinal aganglionosis. While surgery is lifesaving, gastrointestinal (GI) motility disorders persist in many patients. Our objective was to determine whether enteric nervous system (ENS) abnormalities exist in the ganglionated portions of the GI tract far proximal to the aganglionic region and whether these are associated with GI dysmotility. Using Ednrb-null mice, a model of HSCR, immunohistochemical analysis was performed to evaluate quantitatively ENS structure in proximal colon, small intestine, and stomach. Gastric emptying and intestinal transit were measured in vivo and small and large bowel contractility were assessed by spatiotemporal mapping ex vivo. Proximal colon of HSCR mice had smaller ganglia and decreased neuronal fiber density, along with a marked reduction in migrating motor complexes. The distal small intestine exhibited significantly fewer ganglia and decreased neuronal fiber density, and this was associated with delayed small intestinal transit time. Finally, in the stomach of HSCR mice, enteric neuronal packing density was increased and gastric emptying was faster. ENS abnormalities and motility defects are present throughout the ganglionated portions of the GI tract in Ednrb-deficient mice. This may explain the GI morbidity that often occurs following pull-through surgery for HSCR.
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影响因子：
3.5
作者：
Zaitoun I;Erickson CS;Barlow AJ;Klein TR;Heneghan AF;Pierre JF;Epstein ML;Gosain A
通讯作者：
Gosain A
影响因子：
1.8
作者：
Kyrklund, Kristiina;Neuvonen, Malla I.;Rintala, Risto J.
通讯作者：
Rintala, Risto J.
在肠道中建立大脑：肠神经系统的发展。
影响因子：
3.5
作者：
Goldstein AM;Hofstra RM;Burns AJ
通讯作者：
Burns AJ
影响因子：
2.4
作者：
Widyasari A;Pavitasari WA;Dwihantoro A;Gunadi
通讯作者：
Gunadi
影响因子：
9
作者：
Neuvonen, Malla I.;Kyrklund, Kristiina;Pakarinen, Mikko P.
通讯作者：
Pakarinen, Mikko P.