Prem Puri

Summary

Publications

  1. Nakamura H, O Donnell A, Puri P. Consanguinity and its relevance for the incidence of megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS): systematic review. Pediatr Surg Int. 2019;35:175-180 pubmed publisher
    ..Pre-marital and pre-conception counselling of consanguineous populations is recommended to prevent harmful consequences. ..
  2. O Donnell A, Nakamura H, Tomuschat C, Marayati N, Puri P. Altered expression of KCNG3 and KCNG4 in Hirschsprung's disease. Pediatr Surg Int. 2019;35:193-197 pubmed publisher
    ..KCNG3 and KCNG4 downregulation within ganglionic specimens highlights the physiologically abnormal nature of this segment in HSCR patients. ..
  3. O Donnell A, Nakamura H, Tomuschat C, Marayati N, Puri P. Abnormal Scn1b and Fxyd1 gene expression in the pulled-through ganglionic colon may influence functional outcome in patients with Hirschsprung's disease. Pediatr Surg Int. 2019;35:9-14 pubmed publisher
    ..These results add to mounting evidence suggesting that the pulled-through ganglionic segment of bowel in these patients is abnormal, despite the presence of ganglion cells. ..
  4. Nakamura H, O Donnell A, Marayati N, Tomuschat C, Coyle D, Puri P. Altered expression of inflammasomes in Hirschsprung's disease. Pediatr Surg Int. 2019;35:15-20 pubmed publisher
    ..Decreased expression of NLRP3, NLRP12, NLRC4, ASC and pro-IL-1β in the aganglionic and ganglionic bowel may increase susceptibility of HSCR patients to develop HAEC. ..
  5. Takahashi T, Friedmacher F, Zimmer J, Puri P. Expression of dispatched RND transporter family member 1 is decreased in the diaphragmatic and pulmonary mesenchyme of nitrofen-induced congenital diaphragmatic hernia. Pediatr Surg Int. 2019;35:35-40 pubmed publisher
    ..Decreased Disp-1 expression during diaphragmatic development and lung branching morphogenesis may interrupt mesenchymal cell proliferation, thus leading to diaphragmatic defects and PH in the nitrofen-induced CDH model. ..
  6. Tomuschat C, Virbel C, O Donnell A, Puri P. Reduced expression of the NLRP6 inflammasome in the colon of patients with Hirschsprung's disease. J Pediatr Surg. 2018;: pubmed publisher
    ..The observed decreased expression of NLRP6 may contribute to an altered colonic microbiome in patients with HSCR and increases the susceptibility to develop HAEC. ..
  7. Nakamura H, Tomuschat C, Coyle D, O Donnel A, Lim T, Puri P. Altered goblet cell function in Hirschsprung's disease. Pediatr Surg Int. 2018;34:121-128 pubmed publisher
    ..Altered goblet cell function may result in intestinal barrier dysfunction contributing to the development of HAEC. ..
  8. O Donnell A, Coyle D, Puri P. Altered expression of retinoblastoma 1 in Hirschsprung's disease. J Pediatr Surg. 2016;51:1830-1833 pubmed publisher
    ..As RB1 is known to colocalize with nitrergic neurons, the decreased expression of RB1 in the aganglionic bowel is most likely a secondary phenomenon because of the deficient nitrergic innervation in HD. ..
  9. Tomuschat C, O Donnell A, Coyle D, Puri P. Reduced expression of voltage-gated Kv11.1 (hERG) K(+) channels in aganglionic colon in Hirschsprung's disease. Pediatr Surg Int. 2016;32:9-16 pubmed publisher
    ..The decreased expression of hERG in the aganglionic colon may be responsible for the increased tone in the aganglionic narrow spastic segment of bowel. ..

More Information

Publications42

  1. Takahashi T, Friedmacher F, Zimmer J, Puri P. Increased c-kit and stem cell factor expression in the pulmonary vasculature of nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg. 2016;51:706-9 pubmed publisher
    ..Increased expression of c-Kit and SCF in the pulmonary vasculature of nitrofen-induced CDH lungs suggest that increased c-Kit signaling during lung vascular formation may contribute to vascular remodeling and thus to PPH. ..
  2. Friedmacher F, Puri P. Current practice patterns of rectal suction biopsy in the diagnostic work-up of Hirschsprung's disease: results from an international survey. Pediatr Surg Int. 2016;32:717-22 pubmed publisher
    ..Therefore, a prospective multi-center study or larger global audit appears warranted to identify if the present survey reflects common surgical practice and to establish universal standards for RSB. ..
  3. Coyle D, O Donnell A, Gillick J, Puri P. Altered neurotransmitter expression profile in the ganglionic bowel in Hirschsprung's disease. J Pediatr Surg. 2016;51:762-9 pubmed publisher
    ..Imbalance of key excitatory and inhibitory neurotransmitters in the ganglionic bowel in HSCR may explain the basis of bowel dysmotility after an optimal pull-through operation in some patients. ..
  4. Takahashi T, Friedmacher F, Zimmer J, Puri P. Mesenchymal expression of the FRAS1/FREM2 gene unit is decreased in the developing fetal diaphragm of nitrofen-induced congenital diaphragmatic hernia. Pediatr Surg Int. 2016;32:135-40 pubmed publisher
  5. Takahashi H, Kutasy B, Friedmacher F, Takahashi T, Puri P. Expression of hepatic lipid droplets is decreased in the nitrofen model of congenital diaphragmatic hernia. Pediatr Surg Int. 2016;32:155-60 pubmed publisher
    ..Our findings provide strong evidence of hepatic hypertrophy and increased cell apoptosis in the liver of nitrofen-induced CDH. These morphological changes may affect liver lipid droplet expression function. ..
  6. Tomuschat C, O Donnell A, Coyle D, Dreher N, Kelly D, Puri P. Altered expression of ATP-sensitive K(+) channels in Hirschsprung's disease. J Pediatr Surg. 2016;51:948-52 pubmed publisher
    ..The decreased K(ATP) channel expression in HSCR specimens suggests that an altered K(ATP) expression may interfere with intestinal epithelium barrier function and predispose to HAEC. ..
  7. Friedmacher F, Fujiwara N, Hofmann A, Takahashi H, Alvarez L, Gosemann J, et al. Prenatal retinoic acid increases lipofibroblast expression in hypoplastic rat lungs with experimental congenital diaphragmatic hernia. J Pediatr Surg. 2014;49:876-81; discussion 881 pubmed publisher
    ..Increased LIF expression after prenatal treatment with ATRA in nitrofen-induced PH suggests that ATRA may have a therapeutic potential in attenuating CDH-associated PH by stimulating alveolar development. ..
  8. Takahashi T, Zimmer J, Friedmacher F, Puri P. Follistatin-like 1 expression is decreased in the alveolar epithelium of hypoplastic rat lungs with nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg. 2017;52:706-709 pubmed publisher
    ..2b (Centre for Evidence-Based Medicine, Oxford). ..
  9. Nakamura H, Zimmer J, Lim T, Puri P. Increased CaSR and TRPC6 pulmonary vascular expression in the nitrofen-induced model of congenital diaphragmatic hernia. Pediatr Surg Int. 2018;34:211-215 pubmed publisher
    ..Increased CaSR and TRPC6 expression in CDH lung suggests that CaSR interacting with TRPC6 may contribute to abnormal vascular remodeling resulting in pulmonary vasoconstriction and development of PPH. ..
  10. Zimmer J, Takahashi T, Hofmann A, Puri P. Downregulation of KCNQ5 expression in the rat pulmonary vasculature of nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg. 2017;52:702-705 pubmed publisher
    ..KCNQ5 channel activator drugs may be a potential therapeutic target for the treatment of PH in CDH. 2b (Centre for Evidence-Based Medicine, Oxford). ..
  11. Tomuschat C, O Donnell A, Coyle D, Dreher N, Kelly D, Puri P. NOS-interacting protein (NOSIP) is increased in the colon of patients with Hirschsprungs's disease. J Pediatr Surg. 2017;52:772-777 pubmed publisher
    ..The increased expression of NOSIP in the aganglionic and ganglionic bowel of HSCR may contribute to the development of enterocolitis by inhibiting local NO production in patients with Hirschsprung's disease. II. ..
  12. Takahashi T, Friedmacher F, Zimmer J, Puri P. Gata-6 expression is decreased in diaphragmatic and pulmonary mesenchyme of fetal rats with nitrofen-induced congenital diaphragmatic hernia. Pediatr Surg Int. 2018;34:315-321 pubmed publisher
  13. Nakamura H, Lim T, Puri P. Probiotics for the prevention of Hirschsprung-associated enterocolitis: a systematic review and meta-analysis. Pediatr Surg Int. 2018;34:189-193 pubmed publisher
    ..With increasing knowledge of the role of microbiota in HAEC, we are likely to understand better the potential benefits of probiotics in this disease. ..
  14. Nakamura H, Lim T, Puri P. Inflammatory bowel disease in patients with Hirschsprung's disease: a systematic review and meta-analysis. Pediatr Surg Int. 2018;34:149-154 pubmed publisher
    ..Recognition of IBD may be important in the long-term follow-up of HSCR patients who have had postoperative HAEC. ..
  15. Takahashi T, Friedmacher F, Takahashi H, Hofmann A, Puri P. Kif7 expression is decreased in the diaphragmatic and pulmonary mesenchyme of nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg. 2015;50:904-7 pubmed publisher
  16. O Donnell A, Coyle D, Puri P. Decreased expression of Kv7 channels in Hirchsprung's disease. J Pediatr Surg. 2017;52:1177-1181 pubmed publisher
    ..A deficiency of Kv7.4 channels in the ganglionic and aganglionic bowel may place a role in colonic dysmotility in HSCR. ..
  17. Zimmer J, Takahashi T, Hofmann A, Puri P. Decreased Endoglin expression in the pulmonary vasculature of nitrofen-induced congenital diaphragmatic hernia rat model. Pediatr Surg Int. 2017;33:263-268 pubmed publisher
    ..Abnormal Eng/Tgf?r1/Tgf?r2 signalling may contribute to impaired vascular remodelling and development of PH in this CDH animal model. ..
  18. request reprint
    Zimmer J, Takahashi T, Hofmann A, Puri P. Downregulation of Forkhead box F1 gene expression in the pulmonary vasculature of nitrofen-induced congenital diaphragmatic hernia. Pediatr Surg Int. 2016;32:1121-1126 pubmed
    ..Altered FoxF1 gene expression during embryogenesis may participate in vascular maldevelopment resulting in PH in this animal model. ..
  19. request reprint
    Tomuschat C, O Donnell A, Coyle D, Puri P. Increased Act1/IL-17R expression in Hirschsprung's disease. Pediatr Surg Int. 2016;32:1201-1207 pubmed
    ..The increased expression of Act1 and Il-17 in the aganglionic and ganglionic bowel in HSCR may result in IL-17-mediated increased inflammatory response leading to the development of HAEC. ..
  20. Hofmann A, Takahashi T, Duess J, Gosemann J, Puri P. Increased expression of activated pSTAT3 and PIM-1 in the pulmonary vasculature of experimental congenital diaphragmatic hernia. J Pediatr Surg. 2015;50:908-11 pubmed publisher
    ..Markedly increased gene and protein expression of Pim-1 and activated pSTAT3 in the pulmonary vasculature of nitrofen-induced CDH lungs suggest that pSTAT3 and Pim-1 are important mediators of PH in nitrofen-induced CDH. ..
  21. Takahashi T, Friedmacher F, Zimmer J, Puri P. Decreased expression of monocarboxylate transporter 1 and 4 in the branching airway epithelium of nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg. 2016;51:896-9 pubmed publisher
    ..Decreased expression of MCT1/4 in distal airway epithelium may disrupt lung branching morphogenesis and thus contribute to the development of PH in the nitrofen-induced CDH model. ..
  22. Takahashi T, Friedmacher F, Zimmer J, Puri P. Expression of T-box transcription factors 2, 4 and 5 is decreased in the branching airway mesenchyme of nitrofen-induced hypoplastic lungs. Pediatr Surg Int. 2017;33:139-143 pubmed publisher
    ..Decreased expression of Tbx2, Tbx4 and Tbx5 in the pulmonary mesenchyme during fetal lung development may lead to a decrease or arrest of airway branching, thus contributing to PH in the nitrofen-induced CDH model. ..
  23. Tani G, Tomuschat C, O Donnell A, Coyle D, Puri P. Increased population of immature enteric glial cells in the resected proximal ganglionic bowel of Hirschsprung's disease patients. J Surg Res. 2017;218:150-155 pubmed publisher
  24. Tomuschat C, O Donnell A, Coyle D, Puri P. Reduction of hydrogen sulfide synthesis enzymes cystathionine-?-synthase and cystathionine-?-lyase in the colon of patients with Hirschsprungs disease. J Pediatr Surg. 2018;53:525-530 pubmed publisher
    ..The observed decreased expression of CBS and CSE may affect mucosal integrity and colonic contractility and thus render HSCR patients more susceptible to develop HAEC. ..
  25. Henderson D, Zimmer J, Nakamura H, Puri P. Hirschsprung's disease in twins: a systematic review and meta-analysis. Pediatr Surg Int. 2017;33:855-859 pubmed publisher
    ..Future studies investigating the disease-associated mutations in the already identified HSCR genes should provide insights into the genetic basis of HSCR in twins. ..
  26. Takahashi T, Friedmacher F, Zimmer J, Puri P. Down-regulation of N-deacetylase-N-sulfotransferase-1 signaling in the developing diaphragmatic vasculature of nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg. 2017;52:1035-1039 pubmed publisher
    ..Down-regulation of Ndst1 signaling in the developing diaphragm may impair endothelial cell migration and angiogenesis, thus leading to defective diaphragmatic vascular development and CDH. Ib. ..
  27. Tomuschat C, O Donnell A, Coyle D, Puri P. Altered expression of IL36? and IL36 receptor (IL1RL2) in the colon of patients with Hirschsprung's disease. Pediatr Surg Int. 2017;33:181-186 pubmed publisher
  28. Friedmacher F, Fujiwara N, Hofmann A, Takahashi H, Gosemann J, Puri P. Expression of Eya1 and Six1 is decreased in distal airways of rats with experimental pulmonary hypoplasia. J Pediatr Surg. 2014;49:301-4 pubmed publisher
  29. Takahashi T, Zimmer J, Friedmacher F, Puri P. Expression of Prx1 and Tcf4 is decreased in the diaphragmatic muscle connective tissue of nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg. 2016;51:1931-1935 pubmed publisher
    ..Decreased expression of Prx1 and Tcf4 in the fetal diaphragm may cause defects in the PPF-derived MCT, leading to development of CDH in the nitrofen model. Level 2c (Centre for Evidence-Based Medicine, Oxford). ..
  30. request reprint
    Takahashi T, Friedmacher F, Zimmer J, Puri P. Decreased Desmin expression in the developing diaphragm of the nitrofen-induced congenital diaphragmatic hernia rat model. Pediatr Surg Int. 2016;32:1127-1132 pubmed
  31. Takahashi T, Friedmacher F, Zimmer J, Puri P. Decreased expression of hepatocyte growth factor in the nitrofen model of congenital diaphragmatic hernia. Pediatr Surg Int. 2016;32:967-73 pubmed publisher
  32. Hofmann A, Friedmacher F, Takahashi T, Gosemann J, Puri P. Increased pulmonary vascular expression of receptor for advanced glycation end products (RAGE) in experimental congenital diaphragmatic hernia. J Pediatr Surg. 2015;50:746-9 pubmed publisher
  33. Hofmann A, Friedmacher F, Hunziker M, Takahashi H, Duess J, Gosemann J, et al. Upregulation of serotonin-receptor-2a and serotonin transporter expression in the pulmonary vasculature of nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg. 2014;49:871-4; discussion 874-5 pubmed publisher
    ..Increased gene and protein expression of 5HT2A and 5-HTT in the pulmonary vasculature of nitrofen-induced CDH lungs suggest that 5HT2A and 5-HTT are important mediators of PH in nitrofen-induced CDH. ..