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| 1 | Decreased expression of hyperpolarisation-activated cyclic nucleotide-gated channel 3 in Hirschsprung's disease显示文摘AIM: To determine if hyperpolarisation-activated nucleotide-gated(HCN) channels exist in human colon, and to investigate the expression of HCN channels in Hirschsprung's disease.METHODS:We investigated HCN1,HCN2,HCN3 and HCN4 protein expression in pull-through specimens from patients with Hirschsprung’s disease(HSCR,n=10)using the proximal-most ganglionic segment and distalmost aganglionic segment,as well as in healthy control specimens obtained at the time of sigmoid colostomy closure in children who had undergone anorectoplasty for imperforate anus(n=10).Fluorescent immunohistochemistry was performed to assess protein distribution,which was then visualized using confocal microscopy.RESULTS:No HCN1 channel expression was observed in any of the tissues studied.Both HCN2 and HCN4proteins were found to be equally expressed in the aganglionic and ganglionic bowel in HSCR and controls.HCN3 channel expression was found to be markedly decreased in the aganglionic colon vs ganglionic colon and controls.HCN2-4 channels were seen to be expressed within neurons of the myenteric and submucosal plexus of the ganglionic bowel and normal controls,and also co-localised to interstitial cells of Cajal in all tissues studied.CONCLUSION:We demonstrate HCN channel expression in human colon for the first time.Reduced HCN3expression in aganglionic bowel suggests its potential role in HSCR pathophysiology. | Anne Marie O’Donnell David Coyle Prem Puri | 2015 | World Journal of Gastroenterology2015，21，18： | 4 |
| 2 | Prenatal retinoic acid upregulates connexin 43 (Cx43) gene expression in pulmonary hypoplasia in the nitrofen-induced congenital diaphragmatic hernia rat model显示文摘 | Elke Maria Ruttenstock Takashi Doi Jens Dingemann Prem Puri | 2012 | Journal of Pediatric Surgery2012，，2： | 2 |
| 3 | Long-term outcome of patients with enterocolitis complicating Hirschsprung’s disease显示文摘 | Maria Menezes Prem Puri | 2006 | Pediatric Surgery International2006，，4： | 1 |
| 4 | Altered distribution of small-conductance calcium-activated potassium channel SK3 in Hirschsprung’s disease显示文摘 | David Coyle Anne Marie O’Donnell Prem Puri | 2015 | Journal of Pediatric Surgery2015，，10： | 1 |
| 5 | Antimalarial activity of 2,4,6-trisubstituted pyrimidines显示文摘 | Anu Agarwal Kumkum Srivastava S.K. Puri Prem M.S. Chauhan | 2005 | Bioorganic & Medicinal Chemistry Letters2005，，7： | 1 |
| 6 | Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of childhood显示文摘 | Udo Rolle Anna Piaseczna-Piotrowska Prem Puri | 2007 | Pediatric Surgery International2007，，12： | 1 |
| 7 | Synthesis of 4-pyrido-6-aryl-2-substituted amino pyrimidines as a new class of antimalarial agents显示文摘 | Anu Agarwal Kumkum Srivastava S.K. Puri Prem M.S. Chauhan | 2005 | Bioorganic & Medicinal Chemistry2005，，22： | 1 |
| 8 | Glial - de- rived growth factor signaling pathway in infantile hypertrophic pyloric stenosis显示文摘 | Nino Guarino Hideki Shima Takaharu Oue Prem Puri | 2000 | J Pediatr Surg2000，35，6： | 1 |
| 9 | Management of appendiceal mass in children显示文摘 | Prem Puri Barry O Dormell | 1989 | Pediatr Surg Int1989，4，： | 1 |
| 10 | Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of childhood显示文摘 | Udo Rolle Anna Piaseczna-Piotrowska Prem Puri | 2007 | Pediatric Surgery International2007，，12： | 1 |
| 11 | Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system: A report from the fourth International Symposium on Hirschsprung’s disease and related neurocristopathies显示文摘 | Giuseppe Martucciello Alessio Pini Prato Prem Puri Alexander M. Holschneider William Meier-Ruge Vincenzo Jasonni Juan A. Tovar Jay L. Grosfeld | 2005 | Journal of Pediatric Surgery2005，，： | 1 |
| 12 | Genome-wide High-Density SNP-Based Linkage Analysis of Infantile Hypertrophic Pyloric Stenosis Identifies Loci on Chromosomes 11q14-q22 and Xq23显示文摘 | Kate V. Everett Barry A. Chioza Christina Georgoula Ashley Reece Francesca Capon Keith A. Parker Cathy Cord-Udy Paul McKeigue Sally Mitton Agostino Pierro Prem Puri Hannah M. Mitchison Eddie M.K. Chung R. Mark Gardiner | 2008 | The American Journal of Human Genetics2008，，3： | 1 |
| 13 | International Survey on the Management of Esophageal Atresia显示文摘 | Augusto Zani Simon Eaton Michael Hoellwarth Prem Puri Juan Tovar Guenter Fasching Pietro Bagolan Marija Lukac Rene Wijnen Joachim Kuebler Giovanni Cecchetto Risto Rintala Agostino Pierro | 2013 | Eur J Pediatr Surg2013，，： | 1 |
| 14 | Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect显示文摘 | Jens Dingemann Prem Puri | 2010 | Pediatric Surgery International2010，，11： | 1 |
| 15 | Management of ap-pendiceal mass in children显示文摘 | Prem Puri Barry O'Donnell | 1989 | Pediatr Surg Int1989，4，： | 1 |
| 16 | Hepatic overexpression of MHC Class II antigens and macrophage-associated antigens (CD68) in patients with biliary atresia of poor prognosis显示文摘 | Hiroyuki Kobayashi Prem Puri D.Sean O’Briain Rajendra Surana Takeshi Miyano | 1997 | Journal of Pediatric Surgery1997，，4： | 1 |
| 17 | Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system显示文摘 | Giuseppe Martucciello Alessio pini Prato Prem Puri | 2005 | J Pediatr Surg2005，40，： | 1 |
| 18 | Intestinal Neuronal Dysplasia in Twins显示文摘 | Hiroyuki Kobayashi Anies Mahomed Prem Puri | 1996 | Journal of Pediatric Gastroenterology & Nutrition1996，，： | 1 |
| 19 | Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of childhood显示文摘 | Udo Rolle Anna Piaseczna-Piotrowska Prem Puri | 2007 | Pediatric Surgery International2007，，12： | 1 |
| 20 | Deficiency 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 | 2016 | World Journal of Gastroenterology2016，22，12： | 1 |
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