Frontiers in Pediatrics (Nov 2021)

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

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

DOI
https://doi.org/10.3389/fped.2021.746274
Journal volume & issue
Vol. 9

Abstract

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Objective: The aim of this study was to explore the development of enteric glial cells (EGCs) in different segments of Hirschsprung's disease (HSCR).Methods: 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.Results: 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 (p < 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.Conclusion: Aberrant development of myenteric EGCs was observed in the colon of HSCR, which may affect the survival of enteric neurons.

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