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1Deficiency of platelet-derived growth factor receptor-α-positive cells in Hirschsprung's disease colon显示文摘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.Anne-Marie O'Donnell David Coyle Prem Puri 2016World Journal of Gastroenterology2016,22,12:1
2Pediatric intestinal motility disorders显示文摘Pediatric intestinal motility disorders affect many children and thus not only impose a significant impact on pediatric health care in general but also on the quality of life of the affected patient.Furthermore,some of these conditions might also have implications for adulthood.Pediatric intestinal motility disorders frequently present as chronic constipation in toddler age children.Most of these conditions are functional,meaning that constipation does not have an organic etiology,but in 5% of the cases,an underlying,clearly organic disorder can be identified.Patients with organic causes for intestinal motility disorders usually present in early infancy or even right after birth.The most striking clinical feature of children with severe intestinal motility disorders is the delayed passage of meconium in the newborn period.This sign is highly indicative of the presence of Hirschsprung disease(HD),which is the most frequent congenital disorder of intestinal motility.HD is a rare but important congenital disease and the most significant entity of pediatric intestinal motility disorders.The etiology and pathogenesis of HD have been extensively studied over the last several decades.A defect in neural crest derived cell migration has been proven as an underlying cause of HD,leading to an aganglionic distal end of the gut.Numerous basic science and clinical research related studies have been conducted to better diagnose and treat HD.Resection of the aganglionic bowel remains the gold standard for treatment of HD.Most recent studies show,at least experimentally,the possibility of a stem cell based therapy for HD.This editorial also includes rare causes of pediatric intestinal motility disorders such as hypoganglionosis,dysganglionosis,chronic intestinal pseudo-obstruction and ganglioneuromatosis in multiple endocrine metaplasia.Underlying organic pathologies are rare in pediatric intestinal motility disorders but must be recognized as early as possible.Stefan Gfroerer Udo Rolle 2015World Journal of Gastroenterology2015,21,33:0
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