PDF(Pubmed)
Abstract：
The enteric nervous system (ENS) controls gastrointestinal (GI) motility, and defects in ENS development underlie pediatric GI motility disorders. In disorders such as Hirschsprung\'s disease (HSCR), pediatric intestinal pseudo-obstruction (PIPO), and intestinal neuronal dysplasia type B (INDB), ENS structure is altered with noted decreased neuronal density in HSCR and reports of increased neuronal density in PIPO and INDB. The developmental origin of these structural deficits is not fully understood. Here, we review the current understanding of ENS development and pediatric GI motility disorders incorporating new data on ENS structure. In particular, emerging evidence demonstrates that enteric neurons are patterned into circumferential stripes along the longitudinal axis of the intestine during mouse and human development. This novel understanding of ENS structure proposes new questions about the pathophysiology of pediatric GI motility disorders. If the ENS is organized into stripes, could the observed changes in enteric neuron density in HSCR, PIPO, and INDB represent differences in the distribution of enteric neuronal stripes? We review mechanisms of striped patterning from other biological systems and propose how defects in striped ENS patterning could explain structural deficits observed in pediatric GI motility disorders.
摘要：
肠神经系统（ENS）控制胃肠（GI）运动，ENS发育缺陷是儿科胃肠动力障碍的基础。在诸如先天性巨结肠病(HSCR)等疾病中，小儿假性肠梗阻（PIPO），和肠道神经元发育不良B型（INDB），ENS结构发生改变，注意到HSCR中神经元密度降低，PIPO和INDB中神经元密度增加。这些结构性缺陷的发展起源尚未完全了解。这里,我们结合有关ENS结构的新数据,综述了目前对ENS发育和小儿胃肠动力障碍的认识.特别是,新出现的证据表明，在小鼠和人类发育过程中，肠神经元沿着肠的纵轴被图案化为圆周条纹。这种对ENS结构的新理解提出了有关小儿胃肠动力障碍的病理生理学的新问题。如果ENS被组织成条纹，HSCR中观察到的肠神经元密度的变化，PIPO,和INDB代表肠神经元条纹分布的差异？这里，我们回顾了其他生物系统中条纹图案形成的机制,并提出了条纹ENS图案形成的缺陷如何解释儿科胃肠动力障碍中观察到的结构缺陷.
