AUTHOR=Zhou Tingting , Liu Wei , Yu Xiaofang , Cao Zengcai , Mu Weijing , Hou Peimin , Ren Chuantao , Li Aiwu 

TITLE=Aberrant Development of Enteric Glial Cells in the Colon of Hirschsprung's Disease

JOURNAL=Frontiers in Pediatrics

VOLUME=9

YEAR=2021

URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.746274

DOI=10.3389/fped.2021.746274

ISSN=2296-2360

ABSTRACT=<p><bold>Objective:</bold> The aim of this study was to explore the development of enteric glial cells (EGCs) in different segments of Hirschsprung's disease (HSCR).</p><p><bold>Methods:</bold> Colonic specimens from 35 children with HSCR were selected to analyze the relative expression of glial fibrillary acidic protein and S100 calcium-binding protein B using Western blotting and real-time fluorescence quantitative PCR. Immunofluorescence and immunohistochemical staining were performed to determine the distribution of myenteric EGCs and neuronal cells in different segments of HSCR.</p><p><bold>Results:</bold> There was a trend of diminished protein and mRNA expression of glial fibrillary acidic protein and S100 calcium-binding protein B from the proximal, dilated, and transitional segments to the aganglionic segment (<italic>p</italic> &lt; 0.05). Immunofluorescence and immunohistochemistry showed that the EGCs in the aganglionic, transitional, and dilated colonic muscles were morphologically abnormal, which was consistent with the dysplasia of myenteric neurons.</p><p><bold>Conclusion:</bold> Aberrant development of myenteric EGCs was observed in the colon of HSCR, which may affect the survival of enteric neurons.</p>