keyword
MENU ▼
Read by QxMD icon Read
search

Hirschsprung's disease

keyword
https://www.readbyqxmd.com/read/28087321/enteric-nervous-system-development-a-crest-cell-s-journey-from-neural-tube-to-colon
#1
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
https://www.readbyqxmd.com/read/28087137/nos-interacting-protein-nosip-is-increased-in-the-colon-of-patients-with-hirschsprung-s-disease
#2
Anne-Marie O'Donnell, David Coyle, Prem Puri
PURPOSE: Voltage-dependent K(+) channels (Kv channels) participate in electrical rhythmicity and smooth muscle responses and are regulated by excitatory and inhibitory neurotransmitters. Kv channels also participate in the interstitial cell of Cajal (ICC) and smooth muscle cell (SMC) responses to neural inputs. The Kv family consists of 12 subfamilies, Kv1-Kv12, with five members of the Kv7 family identified to date: Kv7.1-Kv7.5. A recent study identified the potassium channel Kv7.5 as having a role in the excitability of ICC-IM in the mouse colon...
January 3, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28060794/postnatal-human-enteric-neuronal-progenitors-can-migrate-differentiate-and-proliferate-in-embryonic-and-postnatal-aganglionic-gut-environments
#3
Lily S Cheng, Ryo Hotta, Hannah K Graham, Jaime Belkind-Gerson, Nandor Nagy, Allan M Goldstein
BACKGROUND: Enteric neural stem/progenitor cells (ENSCs) offer an innovative approach to treating Hirschsprung disease (HSCR) and other enteric neuropathies. However, postnatal-derived human ENSCs have not been thoroughly characterized and their behavior in the embryonic and postnatal intestinal environment is unknown. METHODS: ENSCs were isolated from the intestines of 25 patients undergoing bowel resection, including 7 children with HSCR. Neuronal differentiation and proliferation of ENSCs from submucosal and myenteric plexuses from patients with and without HSCR were characterized...
January 6, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28058486/current-status-of-hirschsprung-s-disease-based-on-a-nationwide-survey-of-japan
#4
Tomoaki Taguchi, Satoshi Obata, Satoshi Ieiri
PURPOSE: The diagnosis and surgical treatments of Hirschsprung's disease (HD) have undergone various changes in the last few decades because of establishment of laparoscopic procedures. A retrospective nationwide survey for 4 decades was performed to study the changing profile of HD in Japan. METHODS: The patient data were collected in 4 phases: Group 1, between 1978 and 1982; Group 2, between 1988 and 1992; Group 3, between 1998 and 2002; and Group 4, between 2008 and 2012...
January 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28050027/a-newly-recognized-association-of-hirschsprung-disease-with-cri-du-chat-syndrome
#5
Imdad Ullah, Lori Mahajan, David Magnuson
No abstract text is available yet for this article.
January 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/28040830/redo-pullthrough-for-hirschsprung-disease
#6
REVIEW
Matthew W Ralls, Arnold G Coran, Daniel H Teitelbaum
Pullthrough procedures for Hirschsprung diseases typically have favorable results. However, some children experience long-term postoperative complications comprising stooling disorders, such as intermittent enterocolitis, severe stool retention, intestinal obstruction, as well as incontinence. Reoperative Hirschsprung Disease surgery is complex. This begins with the workup after the initial presentation following primary pullthrough, continues with the definitive surgical correction with redo pullthrough, and ends with long-term follow-up of individuals...
December 31, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/28032565/surgical-site-infection-after-stoma-closure-in-children-outcomes-and-predictors
#7
Dani O Gonzalez, Erica Ambeba, Peter C Minneci, Katherine J Deans, Benedict C Nwomeh
BACKGROUND: Surgical site infection (SSI) is a burdensome complication following intestinal stoma closure, with reported rates ranging from 0% to 40%. We aimed to identify risk factors for SSI in children undergoing stoma closure. MATERIALS AND METHODS: Using 2012-2014 NSQIP Pediatric data, we identified patients aged 0-18 years undergoing stoma closure. Demographic, clinical, and 30-day outcome characteristics between children with and without SSI were compared...
November 4, 2016: Journal of Surgical Research
https://www.readbyqxmd.com/read/28006787/role-of-mir-215-in-hirschsprung-s-disease-pathogenesis-by-targeting-siglec-8
#8
Hao Lei, Hongxing Li, Hua Xie, Chunxia Du, Yankai Xia, Weibing Tang
BACKGROUND/AIMS: Hirschsprung's disease (HSCR), known as aganglionosis, is an infrequent congenital gut motility disorder characterized by absence of enteric neurons. In this study, we focus on the role of the intronic miR-215 and its host gene isoleucyl-tRNA synthetase 2 (IARS2) in the pathogenesis of HSCR. METHODS: Quantitative real time PCR and Western blot were used to detect the miRNA, mRNAs, and proteins levels. The dual-luciferase reporter gene assay confirmed the direct regulation of the specific mRNA and miRNAs in cell lines...
2016: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28003043/genetic-impact-on-the-treatment-management-of-hirschsprung-disease
#9
REVIEW
Sam W Moore
BACKGROUND: The identification of Hirschsprung's disease (HD) as a genetic condition has been a major step forward in understanding the development of the enteric nervous system and conditions arising from ganglion cell maldevelopment. METHOD: A study of the role of genetics in HD was carried out based on previously published findings from more than 400 cases of HD. RESULTS: There are at least 7 pertinent clinical questions related to HD which were further investigated...
November 13, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27989362/complications-in-colorectal-surgery
#10
Jason S Frischer, Beth Rymeski
Colorectal pediatric surgery is a diverse field that encompasses many different procedures. The pullthrough for Hirschsprung disease, the posterior sagittal anorectoplasty for anorectal malformations including complex cloaca reconstructions and the ileal pouch anal anastomosis for ulcerative colitis and familial adenomatous polyposis present some of the most technically challenging procedures pediatric surgeons undertake. Many children prevail successfully following these surgical interventions, however, a small number of patients suffer from complications following these procedures...
December 2016: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/27988850/advances-in-understanding-functional-variations-in-the-hirschsprung-disease-spectrum-variant-hirschsprung-disease
#11
REVIEW
S W Moore
Hirschsprung disease (HSCR) is a fairly well understood congenital, genetically based functional obstruction due to the congenital absence of ganglion cells in the distal bowel. However, although over 90% of Hirschsprung cases conform to the normally accepted histological diagnostic criteria, it has become increasingly clear that in addition to HSCR, there is a group of functional disturbances relating to a number of other congenital neurodysplastic conditions causing some degree of gastrointestinal tract malfunction...
December 17, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27941923/variability-of-outcome-reporting-in-hirschsprung-s-disease-and-gastroschisis-a-systematic-review
#12
Benjamin Saul Raywood Allin, Amy Irvine, Nicholas Patni, Marian Knight
Heterogeneity in outcome reporting limits identification of gold-standard treatments for Hirschsprung's Disease(HD) and gastroschisis. This review aimed to identify which outcomes are currently investigated in HD and gastroschisis research so as to counter this heterogeneity through informing development of a core outcome set(COS). Two systematic reviews were conducted. Studies were eligible for inclusion if they compared surgical interventions for primary treatment of HD in review one, and gastroschisis in review two...
December 12, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27933652/nonobstructive-diffuse-dilated-bowel-loops-prenatal-diagnosis-fetal-characteristics-and-neonatal-outcomes
#13
Guy Katz, Ben Pode-Shakked, Michal Berkenstadt, Ron Bilik, Sylvie Polak Charcon, Iris Barshack, Reuven Achiron, Yinon Gilboa
OBJECTIVES: The purpose of this study was to describe the characteristics and outcomes of fetuses with a diagnosis of nonobstructive diffuse dilated bowel loops. METHODS: We conducted a retrospective study of all pregnancies with fetal diagnosis of nonobstructive diffuse dilated bowel loops over 14 years in a large tertiary referral center. Fetomaternal and neonatal characteristics and outcomes were assessed. RESULTS: Seven fetuses had sonograms showing diffuse dilated bowel loops; none of them had intestinal obstruction after labor...
January 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/27923407/nets-1hd-study-protocol-for-development-of-a-core-outcome-set-for-use-in-determining-the-overall-success-of-hirschsprung-s-disease-treatment
#14
Benjamin Allin, Timothy Bradnock, Simon Kenny, Gregor Walker, Marian Knight
BACKGROUND: Use of core outcome sets in research has been proposed as a method for countering the problems caused by heterogeneity of outcome measure reporting. Heterogeneity of outcome measure reporting occurs in Hirschsprung's disease (HD) research and is limiting the development of a robust evidence base to support clinical practice. METHODS: Candidate outcome measures have been identified through a systematic review. These outcome measures will form the starting point for a three-phase online Delphi process to be carried out in parallel by three panels of experts...
December 7, 2016: Trials
https://www.readbyqxmd.com/read/27916369/expression-of-connexin-26-and-connexin-43-is-reduced-in-hirschsprung-s-disease
#15
David Coyle, Brian Doyle, Justin M Murphy, Anne Marie O'Donnell, John Gillick, Prem Puri
BACKGROUND: Despite having an optimal pull-through operation, some children with Hirschsprung's disease (HSCR) continue to experience persistent bowel symptoms. Coordinated colonic electrical activity depends on intercellular communication between the enteric nerves, interstitial cells of Cajal (ICCs), smooth muscle cells, and fibroblast-like (platelet-derived growth factor receptor-alpha-positive) cells. Gap junctions are low-resistance channels composed of connexin (Cx) proteins which couple cells electrically and chemically...
November 2016: Journal of Surgical Research
https://www.readbyqxmd.com/read/27916192/long-term-outcome-of-bowel-function-for-110-consecutive-cases-of-hirschsprung-s-disease-comparison-of-the-abdominal-approach-with-transanal-approach-more-than-30years-in-a-single-institution-is-the-transanal-approach-truly-beneficial-for-bowel-function
#16
Shun Onishi, Kazuhiko Nakame, Kouji Yamada, Waka Yamada, Takafumi Kawano, Motoi Mukai, Tatsuru Kaji, Satoshi Ieiri
BACKGROUND: We compared the long-term outcomes of the bowel function (BF) in Hirschsprung's disease (HD) patients who underwent transanal endorectal pull-through (TA) with those who underwent the Soave-Denda (SD) procedure. METHODS: Patient data were collected from 1984 to 2015 from 110 HD patients who underwent definitive diagnosis and operation. The follow-up data were analyzed retrospectively. BF was evaluated according to the evacuation score (ES) of the Japan Society of Anorectal Malformation Study Group at 3, 5, 7, 9, and 11years of age...
December 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27912977/functional-outcomes-in-hirschsprung-disease-a-single-institution-s-12-year-experience
#17
Hemanshoo S Thakkar, Christopher Bassett, Andy Hsu, Riccardo Manuele, Dorothy Kufeji, Catherine A Richards, Meena Agrawal, Alireza S Keshtgar
AIMS: Hirschsprung disease (HD) is a chronic condition associated with long-term morbidity. We assessed the short and long-term functional outcomes of operated patients in a single institution over a 12-year period. MATERIALS AND METHODS: We conducted a retrospective review of all children operated for HD between 2002 and 2014. Postoperative functional outcomes were assessed using the Rintala Bowel Function Score (BFS, 0-20, 20=best score). We assessed hospital admissions, complications including Hirschsprung associated enterocolitis (HAEC) and the need for further surgical procedures...
November 14, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27903978/circular-rna-znf609-functions-as-a-competitive-endogenous-rna-to-regulate-akt3-expression-by-sponging-mir-150-5p-in-hirschsprung-s-disease
#18
Lei Peng, Guanglin Chen, Zhongxian Zhu, Ziyang Shen, Chunxia Du, Rujin Zang, Yang Su, Hua Xie, Hongxing Li, Xiaoqun Xu, Yankai Xia, Weibing Tang
Research over the past decade suggested critical roles for circular RNAs in the natural growth and disease progression. However, it remains poorly defined whether the circular RNAs participate in Hirschsprung disease (HSCR). Here, we reported that the cir-ZNF609 was down-regulated in HSCR compared with normal bowel tissues. Furthermore, suppression of cir-ZNF609 inhibited the proliferation and migration of cells. We screened out several putative cir-ZNF609 ceRNAs of which the AKT3 transcript was selected. Finally, RNA immunoprecipitation and luciferase reporter assays demonstrated that cir-ZNF609 may act as a sponge for miR-150-5p to modulate the expression of AKT3...
November 26, 2016: Oncotarget
https://www.readbyqxmd.com/read/27902697/a-novel-zebrafish-ret-heterozygous-model-of-hirschsprung-disease-identifies-a-functional-role-for-mapk10-as-a-modifier-of-enteric-nervous-system-phenotype-severity
#19
Tiffany A Heanue, Werend Boesmans, Donald M Bell, Koichi Kawakami, Pieter Vanden Berghe, Vassilis Pachnis
Hirschsprung disease (HSCR) is characterized by absence of enteric neurons from the distal colon and severe intestinal dysmotility. To understand the pathophysiology and genetics of HSCR we developed a unique zebrafish model that allows combined genetic, developmental and in vivo physiological studies. We show that ret mutant zebrafish exhibit cellular, physiological and genetic features of HSCR, including absence of intestinal neurons, reduced peristalsis, and varying phenotype expressivity in the heterozygous state...
November 2016: PLoS Genetics
https://www.readbyqxmd.com/read/27898990/european-paediatric-surgeons-association-survey-on-the-management-of-hirschsprung-disease
#20
Augusto Zani, Simon Eaton, Francesco Morini, Prem Puri, Risto Rintala, Ernest van Heurn, Marija Lukac, Pietro Bagolan, Joachim F Kuebler, Florian Friedmacher, Rene Wijnen, Juan A Tovar, Michael E Hoellwarth, Agostino Pierro
: Aim This study aims to define patterns of Hirschsprung disease (HD) management. Methods An online questionnaire was sent to all European Paediatric Surgeons' Association (EUPSA) members. Results A total of 294 members (61 countries) answered (response rate: 61%). DIAGNOSIS: All respondents perform rectal biopsies (61% rectal suction [RSBs], 39% open full-thickness), 96% contrast enema, and 31% anorectal manometry. At RSB, 17% take the most distal biopsy 1 cm above the dentate line, 34% take 2 cm, 30% take 3 cm, and 19% take > 3 cm...
November 29, 2016: European Journal of Pediatric Surgery
keyword
keyword
63812
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"