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AIM: To investigate whether the expression of plateletderived growth factor receptor-α-positive(PDGFRα~+)-cells is altered in Hirschsprung’s disease(HD).METHODS: HD tissue specimens(n = 10) were collected at the time of pull-through surgery, while colonic control samples were obtained at the time of colostomy closure in patients with imperforate anus(n = 10). Immunolabelling of PDGFRα~+-cells was visualized using confocal microscopy to assess the distribution of these cells, while Western blot analysis was undertaken to quantify PDGFRα protein expression.RESULTS: Confocal microscopy revealed PDGFRα+-cells within the mucosa, myenteric plexus and smooth muscle in normal controls, with a marked reduction in PDGFRα~+-cells in the HD specimens. Western blotting revealed high levels of PDGFRα protein expression in normal controls, while there was a striking decrease in PDGFRα protein expression in the HD colon.CONCLUSION: These findings suggest that the altered distribution of PDGFRα~+-cells in both the aganglionic and ganglionic HD bowel may contribute to the motility dysfunction in HD.
AIM: To investigate whether the expression of platelet derived growth factor receptor-α-positive (PDGFRα ~ +) - cells is altered in Hirschsprung’s disease (HD) .METHODS: HD tissue specimens (n = 10) through surgery, while colonic control samples were obtained at the time of colostomy closure in patients with imperforate anus (n = 10). Immunolabelling of PDGFRα ~ + -cells was visualized using confocal microscopy to assess the distribution of these cells, while Western blot analysis was undertaken to quantify PDGFRα protein expression .RESULTS: Confocal microscopy revealed PDGFRα + -cells within the mucosa, myenteric plexus and smooth muscle in normal controls, with a marked reduction in PDGFRα ~ + -cells in the HD specimens. Western blotting revealed high levels of PDGFRα protein expression in normal controls, while there was a striking decrease in PDGFRα protein expression in the HD colon. CONCLUSION: These findings suggest that the altered distribution of PDGFR ~ + -cells in both the aganglionic and ganglionic HD bowel may contribute to the motility dysfunction in HD.