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https://www.readbyqxmd.com/read/28209955/-the-complications-of-intestinal-stoma-in-children
#1
S V Minaev, N I Bykov, A V Isaeva, A V Kachanov, E A Tovkan, N V Filip'yeva, I N Gerasimenko
AIM: To investigate the complications of intestinal stoma in children and to develop measures for decrease of their incidence. MATERIAL AND METHODS: The study included 152 children with congenital and acquired gastrointestinal pathology requiring surgical treatment with the imposition of intestinal stoma. Atresia of intestinal tube was observed in 28 (18.4%) children, meconium ileus - in 10 (6.6%) cases, Hirschsprung's disease - in 11 (7.2%)cases, anorectal malformations - in 39 (25...
2017: Khirurgiia
https://www.readbyqxmd.com/read/28209600/cell-therapy-for-gi-motility-disorders-comparison-of-cell-sources-and-proposed-steps-for-treating-hirschsprung-disease
#2
Lincon A Stamp
Cell therapeutic approaches to treat a range of congenital and degenerative neuropathies are under intense investigation. There have been recent significant advancements in the development of cell therapy to treat disorders of the enteric nervous system, enteric neuropathies. These advances include the efficient generation of enteric neural progenitors from pluripotent stem cells and the rescue of a Hirschsprung Disease model mouse following their transplantation into the bowel. Further, a recent study provides evidence of functional innervation of the bowel muscle by neurons derived from transplanted ENS-derived neural progenitors...
February 16, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28208866/fatal-toxic-megacolon-in-a-child-of-hirschsprung-disease
#3
Shiwani R Garg, Pragati A Sathe, Annapurna C Taware, Ketaki M Surve
Hirschsprung disease (HD) in late childhood is uncommon and often undiagnosed or misdiagnosed. However, in a patient with Hirschsprung disease, of greater significance is the occurrence of life threatening enterocolitis. In its more severe form, this is associated with gross dilatation of the colon and profound toxaemia, the combination being termed toxic megacolon. Because of its relative rarity, we report a case of 10-year-old child who had a history of chronic constipation for nine years. He later developed complications and presented to the emergency department with toxic megacolon, a rare occurrence due to neglected constipation...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28207737/the-enteric-nervous-system-promotes-intestinal-health-by-constraining-microbiota-composition
#4
Annah S Rolig, Erika K Mittge, Julia Ganz, Josh V Troll, Ellie Melancon, Travis J Wiles, Kristin Alligood, W Zac Stephens, Judith S Eisen, Karen Guillemin
Sustaining a balanced intestinal microbial community is critical for maintaining intestinal health and preventing chronic inflammation. The gut is a highly dynamic environment, subject to periodic waves of peristaltic activity. We hypothesized that this dynamic environment is a prerequisite for a balanced microbial community and that the enteric nervous system (ENS), a chief regulator of physiological processes within the gut, profoundly influences gut microbiota composition. We found that zebrafish lacking an ENS due to a mutation in the Hirschsprung disease gene, sox10, develop microbiota-dependent inflammation that is transmissible between hosts...
February 2017: PLoS Biology
https://www.readbyqxmd.com/read/28196663/nos-interacting-protein-nosip-is-increased-in-the-colon-of-patients-with-hirschsprungs-s-disease
#5
Christian Tomuschat, Anne-Marie O'Donnell, David Coyle, Nickolas Dreher, Danielle Kelly, Prem Puri
PURPOSE: Hirschsprung's associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung's disease (HSCR). Nitric oxide (NO) mediates intestinal homoeostasis and is inhibited by NOSIP, a modulator of NO production. We designed this study to investigate the expression of NOSIP in the colon of patients with HSCR. METHODS: We investigated NOSIP, endothelial NO synthase, and neuronal NO synthase expression in both the aganglionic and ganglionic regions of HSCR patients (n=10) versus normal control colon (n=10)...
January 30, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28190556/cotransplantation-of-neuroepithelial-stem-cells-with-interstitial-cells-of-cajal-improves-neuronal-differentiation-in-a-rat-aganglionic-model
#6
Lijuan Zhang, Bin Zhao, Wei Liu, Rui Ma, Rongde Wu, Yingmao Gao
BACKGROUND: Recent advances have raised the possibility of treating Hirschsprung's disease (HSCR) with transplanted neural stem cells. Although these cells have been shown to migrate and differentiate after transplantation, the restoration of intestinal motility by grafted cells is also mediated via the interstitial cells of Cajal (ICCs). We therefore aimed to evaluate the effect of cografting with ICC on neural stem cells after transplanted into aganglionic bowel. METHODS: Neuroepithelial stem cells (NESCs) and ICCs were isolated from neural tube of embryonic rat (embryonic day 11...
February 4, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28190554/neuregulin-1-is-involved-in-enteric-nervous-system-development-in-zebrafish
#7
Jiarui Pu, Shaotao Tang, Qiangsong Tong, Guobin Wang, Haibo Jia, Qiong Jia, Kang Li, Dan Li, Dehua Yang, Jun Yang, Hang Li, Shuai Li, Hong Mei
BACKGROUND: Hirschsprung's disease (HD, also known as congenital colon aganglionosis) is a congenital disorder characterized by the absence of intramural ganglion cells in the distal gastrointestinal tract, which results in tonic contraction of the aganglionic gut segment and functional intestinal obstruction. Recent studies have indicated neuregulin 1 (NRG1) as a new candidate gene involved in the development of the enteric nervous system (ENS) in humans. METHODS: In our study, we investigated the role of NRG1 in zebrafish ENS development by assessing NRG1 expression patterns during ENS development...
January 23, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28185631/single-stage-transanal-endorectal-pull-through-procedure-for-correction-of-hirschsprung-disease-in-neonates-and-nonneonates-a-multicenter-study
#8
Changgui Lu, Guangjun Hou, Chunyi Liu, Qiming Geng, Xiaoqun Xu, Jie Zhang, Huan Chen, Weibing Tang
PURPOSE: The outcomes of single-stage transanal endorectal pull-through (SSTEPT) for Hirschsprung disease (HSCR) in young patients are favorable; however, reports have shown that diagnosis and surgery at young ages increase the risk for postoperative enterocolitis and slows postoperative recovery. The present study was primarily designed to evaluate the outcomes of SSTEPT in a multi-institutional cohort of neonates and nonneonates with HSCR. METHODS: Between August 2005 and May 2012, a total of 650 children with HSCR were divided into the following two groups: group A (neonatal group, operative age<28days [n=186]); and group B (nonneonatal group, operative age>28days [n=464])...
February 2, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28180937/guidelines-for-the-management-of-postoperative-obstructive-symptoms-in-children-with-hirschsprung-disease
#9
REVIEW
J C Langer, M D Rollins, M Levitt, A Gosain, L de la Torre, R P Kapur, R A Cowles, J Horton, D H Rothstein, A M Goldstein
Although most children with Hirschsprung disease ultimately do well, many experience a variety of ongoing problems after pull-through surgery. The most common include obstructive symptoms, soiling, enterocolitis and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative obstructive symptoms in children with Hirschsprung disease. The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review and expert consensus were then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with obstructive symptoms following pull-through for Hirschsprung disease...
February 8, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28169931/bowel-function-and-quality-of-life-after-transanal-endorectal-pull-through-for-hirschsprung-disease-controlled-outcomes-up-to-adulthood
#10
Malla I Neuvonen, Kristiina Kyrklund, Risto J Rintala, Mikko P Pakarinen
OBJECTIVE: The aim of this study was to define controlled outcomes up to adulthood for bowel function and quality of life (QoL) after transanal endorectal pull-through (TEPT) for Hirschsprung disease (HD). SUMMARY OF BACKGROUND DATA: Although TEPT is the surgical standard for HD, controlled long-term follow-up studies evaluating bowel function and QoL are lacking. METHODS: Patients aged ≥4 years operated for HD with TEPT between 1987 and 2011 answered detailed questionnaires on bowel function and QoL [Pediatric Quality of Life Inventory (PedsQL, age <18 yrs) or Gastrointestinal Quality of Life Index (GIQLI) and SF-36]...
March 2017: Annals of Surgery
https://www.readbyqxmd.com/read/28154903/diagnostic-value-of-the-preoperatively-detected-radiological-transition-zone-in-hirschsprung-s-disease
#11
Xuyong Chen, Wu Xiaojuan, Hongyi Zhang, Chunlei Jiao, Kechi Yu, Tianqi Zhu, Jiexiong Feng
BACKGROUND AND OBJECTIVE: The cardinal diagnostic sign of congenital aganglionic megacolon, or Hirschsprung's disease (HD), is an aganglionic segment of the distal colon or rectum. To determine the surgical planning of a radiological transition zone (TZ) in HD, this study investigated the association between a radiological TZ and the bowel resection length. METHODS: A prospective observational study was conducted in children (n = 192) with suspected HD determined by radiological TZ on contrast barium enema, and who underwent pull-through operations...
February 2, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28154902/guidelines-for-the-diagnosis-and-management-of-hirschsprung-associated-enterocolitis
#12
REVIEW
Ankush Gosain, Philip K Frykman, Robert A Cowles, John Horton, Marc Levitt, David H Rothstein, Jacob C Langer, Allan M Goldstein
BACKGROUND: Patients with Hirschsprung disease are at risk for Hirschsprung-associated enterocolitis (HAEC), an inflammatory disorder of the bowel that represents the leading cause of serious morbidity and death in these patients. The diagnosis of HAEC is made based on clinical signs and symptoms which are often non-specific, making it difficult to establish a definitive diagnosis in many patients. The purpose of this guideline is to present a rational, expert-based approach to the diagnosis and management of HAEC...
February 2, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28139502/diagnostic-roles-of-calretinin-in-hirschsprung-disease-a-comparison-to-neuron-specific-enolase
#13
Zaidoon A Musa, Ban J Qasim, Haider F Ghazi, A Wahab A K Al Shaikhly
BACKGROUND/AIM: Diagnosis of Hirschsprung's disease (HD) can be hard and requires good experience, principally for pathologists who infrequently encounter the disease. However, diagnosis is not always possible with hematoxylin and eosin (H and E) because staining has limitations in the identification of immature ganglion cells in neonates and the submucosal area. AIM: To assess the diagnostic role of calretinin immunostaining in HD in comparison to neuron-specific enolase...
January 2017: Saudi Journal of Gastroenterology: Official Journal of the Saudi Gastroenterology Association
https://www.readbyqxmd.com/read/28131074/noncompaction-cardiomyopathy-in-hirschsprung-s-disease-a-case-report
#14
Silvia D Visonà, Gaetano Thiene, Savina Mannarino, Giulia Corana, Antonio Osculati, Annalisa Angelini, Stefania Rizzo
Noncompaction cardiomyopathy is a rare disorder, often associated with cardiac and noncardiac malformations. Hirschsprung's disease, a well-known aganglionosis, is associated with congenital heart diseases and has been reported to be due to impairment migration and differentiation of neural crest cells. Here, we present an 8-month-old male infant who died for cardiogenic shock after surgical resection of the involved bowel segment. The child was affected by both noncompaction cardiomyopathy and Hirschsprung's disease, two entities which can share a common neural crest-derived etiology...
January 2, 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/28129770/validation-of-the-french-versions-of-the-hirschsprung-s-disease-and-anorectal-malformations-quality-of-life-haql-questionnaires-for-adolescents-and-adults
#15
Corine Baayen, Fanny Feuillet, Pauline Clermidi, Célia Crétolle, Sabine Sarnacki, Guillaume Podevin, Jean-Benoit Hardouin
BACKGROUND: The Hirschsprung's disease Anorectal malformation QoL questionnaire (HAQL) is a disease-specific quality of life (QoL) questionnaire for patients with Hirschsprung's disease (HD) or anorectal malformations (ARM). It was originally proposed in Dutch and is currently being translated into other languages to obtain an internationally standardized instrument. In this work we validate a French adaptation of the HAQL for adolescents and adults. METHODS: The questionnaires were translated into French and sent to patients aged 12 years and older, followed for HD or ARM at three French university hospitals...
January 28, 2017: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/28120235/identifying-key-genes-associated-with-hirschsprung-s-disease-based-on-bioinformatics-analysis-of-rna-sequencing-data
#16
Wei-Kang Pan, Ya-Fei Zhang, Hui Yu, Ya Gao, Bai-Jun Zheng, Peng Li, Chong Xie, Xin Ge
BACKGROUND: Hirschsprung's disease (HSCR) is a type of megacolon induced by deficiency or dysfunction of ganglion cells in the distal intestine and is associated with developmental disorders of the enteric nervous system. To explore the mechanisms of HSCR, we analyzed the RNA-sequencing data of the expansion and the narrow segments of colon tissues separated from children with HSCR. METHODS: RNA-sequencing of the expansion segments and the narrow segments of colon tissues isolated from children with HSCR was performed...
January 25, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28099057/a-provisional-experience-with-robot-assisted-soave-procedure-for-older-children-with-hirschsprung-disease-back-to-the-future
#17
Girolamo Mattioli, Luca Pio, Lorenzo Leonelli, Barbara Razore, Nicola Disma, Giovanni Montobbio, Vincenzo Jasonni, Paolo Petralia, Alessio Pini Prato
Hirschsprung disease is a congenital disease characterized by intestinal aganglionosis of various extents. Most patients are younger than 1 year of age. Though, a minority of cases can be older or even adult. Older the patient the more difficult and prolonged is the endorectal dissection required for the pull-through procedure. Longer surgery leads to longer anal dilatation and trauma with subsequent higher likelihood of continence impairment. The article aims at describing the first case series of robot-assisted Soave procedure, which was adopted as an alternative minimally invasive approach to older patients with Hirschsprung disease...
January 18, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/28094084/incontinence-and-psychological-symptoms-in-individuals-with-mowat-wilson-syndrome
#18
Justine Niemczyk, Stewart Einfeld, David Mowat, Monika Equit, Catharina Wagner, Leopold Curfs, Alexander von Gontard
BACKGROUND: Mowat-Wilson Syndrome (MWS) is caused by deletion/mutation of the ZEB2 gene on chromosome 2q22. MWS is characterized by a distinctive facial appearance, severe intellectual disability and other anomalies, e.g. seizures and/or Hirschsprung disease (HSCR). Most individuals have a sociable demeanor, but one third show psychological problems. AIMS: The aim was to investigate incontinence and psychological problems in MWS. METHODS AND PROCEDURES: 26 children (4-12 years), 13 teens (13-17 years) and 8 adults (>18years) were recruited through a MWS support group...
January 13, 2017: Research in Developmental Disabilities
https://www.readbyqxmd.com/read/28094015/a-nordic-multicenter-survey-of-long-term-bowel-function-after-transanal-endorectal-pull-through-in-200-patients-with-rectosigmoid-hirschsprung-disease
#19
Kristin Bjørnland, Mikko P Pakarinen, Pernilla Stenstrøm, Kjetil J Stensrud, Malla Neuvonen, Anna L Granström, Christina Graneli, Are H Pripp, Einar Arnbjörnsson, Ragnhild Emblem, Tomas Wester, Risto J Rintala
OBJECTIVE: Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome. METHODS: Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records...
January 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28087321/enteric-nervous-system-development-a-crest-cell-s-journey-from-neural-tube-to-colon
#20
REVIEW
Nandor Nagy, Allan M Goldstein
The enteric nervous system (ENS) is comprised of a network of neurons and glial cells that are responsible for coordinating many aspects of gastrointestinal (GI) function. These cells arise from the neural crest, migrate to the gut, and then continue their journey to colonize the entire length of the GI tract. Our understanding of the molecular and cellular events that regulate these processes has advanced significantly over the past several decades, in large part facilitated by the use of rodents, avians, and zebrafish as model systems to dissect the signals and pathways involved...
January 10, 2017: Seminars in Cell & Developmental Biology
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