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Hirschsprung

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https://www.readbyqxmd.com/read/29018955/the-effect-of-laminin-1-on-enteric-neural-crest-derived-cell-migration-in-the-hirschsprung-s-disease-mouse-model
#1
Nana Nakazawa-Tanaka, N Fujiwara, K Miyahara, S Nakada, E Arikawa-Hirasawa, C Akazawa, M Urao, A Yamataka
BACKGROUND/AIM: Laminin-1 regulates neurite outgrowth in various neuronal cells. We have previously demonstrated that laminin-1 promotes enteric neural crest-derived cell (ENCC) migration by using Sox10-VENUS transgenic mice, in which ENCCs are labeled with a green fluorescent protein, Venus. Mice lacking the endothelin-B receptor gene, Ednrb (-/-) mice, are widely used as a model for Hirschsprung's disease (HD). The aim of this study was to investigate the effects of laminin-1on ENCC migration in Sox10-VENUS(+)/Ednrb (-/-) mice, a newly created HD mice model...
October 10, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28985842/increased-population-of-immature-enteric-glial-cells-in-the-resected-proximal-ganglionic-bowel-of-hirschsprung-s-disease-patients
#2
Gakuto Tani, Christian Tomuschat, Anne Marie O'Donnell, David Coyle, Prem Puri
BACKGROUND: Enteric glial cells are essential for normal gastrointestinal function. Abnormalities in glial structure, development, or function lead to disturbances in gastrointestinal physiology. Fatty acid-binding protein 7 (FABP7) is a marker of immature enteric glial cells, whereas S100 is expressed only by mature glial cells. Patients with Hirschsprung's disease (HSCR) often suffer from dysmotility and enterocolitis despite proper surgery. We designed this study to determine the distribution and expression of glial cells in patients with HSCR compared to normal controls...
October 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28983778/probiotics-for-the-prevention-of-hirschsprung-associated-enterocolitis-a-systematic-review-and-meta-analysis
#3
H Nakamura, T Lim, P Puri
BACKGROUND/PURPOSE: Hirschsprung-associated enterocolitis (HAEC) is a life-threatening complication of Hirschsprung's disease. HAEC is reported to occur in 6-50% of patients preoperatively and in 2-35% postoperatively. The exact cause of HAEC is not fully understood, but disturbances of intestinal microbiota have recently been reported in patients with HAEC. In recent years, the administration of probiotics has been proposed to reduce the incidence of HAEC. We conducted a systematic review and meta-analysis to determine the effect of probiotics on postoperative HAEC...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28983691/hirschsprung-s-disease-in-the-laparoscopic-transanal-pull-through-era-implications-of-age-at-surgery-and-technical-aspects
#4
Go Miyano, Masahiro Takeda, Hiroyuki Koga, Manabu Okawada, Nana Nakazawa-Tanaka, Junya Ishii, Takashi Doi, Geoffrey J Lane, Tadaharu Okazaki, Masahiko Urao, Atsuyuki Yamataka
AIM: Detailed implications of age at laparoscopic transanal pull-through (LTAPT) on postoperative bowel function (POBF) in Hirschsprung's disease (HD) are somewhat obscure because of a spectrum of factors. METHODS: Age at surgery was used to categorize 106 consecutive postoperative HD cases treated by our modified LTAPT (JLTPAT) between 1997 and 2015; group A: < 3 months old (n = 31); group B: 3-11 months old (n = 44); group C: 1-3 years old (n = 19); and group D: ≥ 4 years old (n = 12)...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28983688/inflammatory-bowel-disease-in-patients-with-hirschsprung-s-disease-a-systematic-review-and-meta-analysis
#5
H Nakamura, T Lim, P Puri
AIM AND OBJECTIVES: Hirschsprung-associated enterocolitis (HAEC) continues to be an important cause of morbidity in patients with Hirschsprung's disease (HSCR). HAEC can occur at any time during the course of the disease. The reported incidence of HAEC before surgery ranges from 6 to 50%, and after surgery, it ranges from 2 to 35%. HAEC and inflammatory bowel disease (IBD) have similar clinical presentation including diarrhea, hematochezia, and abdominal pain. In recent years, isolated cases of IBD have been reported in patients who had surgical treatment for HSCR...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28983681/altered-expression-of-laminin-alpha1-in-aganglionic-colon-of-endothelin-receptor-b-null-mouse-model-of-hirschsprung-s-disease
#6
Naho Fujiwara, Nana Nakazawa-Tanaka, Katsumi Miyahara, Eri Arikawa-Hirasawa, Chihiro Akazawa, Atsuyuki Yamataka
PURPOSE: Laminin, an extracellular matrix molecule, is essential for normal development of the nervous system. The alpha1 subunit of laminin-1 (LAMA1) has been reported to promote neurites and outgrowth and is expressed only during embryogenesis. Previously, we developed a Sox10 transgenic version of the Endothelin receptor-B (Ednrb) mouse to visualize Enteric neural crest-derived cell (ENCC)s with a green fluorescent protein, Venus. We designed this study to investigate the expression of LAMA1 using Sox10-VENUS mice gut...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28975740/hirschsprung-disease
#7
EDITORIAL
Sebastian K King, Jonathan Karpelowsky
No abstract text is available yet for this article.
October 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28965333/preliminary-identification-of-key-mirnas-signaling-pathways-and-genes-associated-with-hirschsprung-s-disease-by-analysis-of-tissue-microrna-expression-profiles
#8
Zhi-Gang Gao, Qing-Jiang Chen, Min Shao, Yun-Zhong Qian, Li-Feng Zhang, Yue-Bin Zhang, Qi-Xing Xiong
BACKGROUND: Hirschsprung's disease (HSCR) is a congenital gut motility disorder of infants, and if left untreated, it is fatal to the affected infants. This study aimed to identify key microRNAs (miRNAs), signaling pathways and genes involved in the pathogenesis of HSCR. METHODS: The miRNA microarray dataset GSE77296 was downloaded. Nine colon tissue samples were available: six from HSCR patients and three matched control samples. Differentially expressed miRNAs (DEMs) were identified after data preprocessing...
October 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28958095/duhamel-and-transanal-endorectal-pull-throughs-for-hirschsprung-disease-a-systematic-review-and-meta-analysis
#9
Shogo Seo, Hiromu Miyake, Alison Hock, Yuhki Koike, Chen Yong, Carol Lee, Bo Li, Agostino Pierro
No abstract text is available yet for this article.
September 28, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28956279/prevalence-risk-factors-and-prognosis-of-postoperative-complications-after-surgery-for-hirschsprung-disease
#10
Wen-Kai Huang, Xue-Li Li, Jin Zhang, Shu-Cheng Zhang
BACKGROUND: Although most of patients do well after surgery for Hirschsprung disease (HSCR), there are complications in some instances that impact social aspects and quality of life. The aim of this study was to explore the prevalence, risk factors, and prognosis of these complications, providing guidance for surgeons and healthcare personnel. METHODS: A cohort of patients (N = 229) was retrospectively reviewed in the aftermath of surgery for HSCR. All medical data and operative notes were assessed...
September 27, 2017: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
https://www.readbyqxmd.com/read/28941931/the-anal-canal-is-the-fine-line-between-fecal-incontinence-and-colitis-after-a-pull-through-for-hirschsprung-disease
#11
Luis De la Torre, Kimberly Cogley, Karla Santos, Otto Morales, Juan Calisto
BACKGROUND/PURPOSE: Fecal incontinence after a pull-through is associated with different factors, although damage to the anal canal seems to be the most important. The objective of this article is to identify the variables related to the presence of fecal control and colitis in a homogeneous group of children after pull-through. METHODS: A retrospective cross-sectional study was performed in patients with HD for evaluation of post-operative problems from May 2014 to November 2016...
September 4, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28940611/perianal-pseudoverrucous-papules-and-nodules-in-hirschsprung-s-disease-rapid-resolution-with-oral-loperamide
#12
Francis H X Yap, Graham Thom
We report the case of a 2-year-old boy with Hirschsprung's disease who developed perianal pseudoverrucous papules and nodules subsequent to persistent diarrhea and stool leakage after Giardia infection. Bleeding from the papules resulted in iron deficiency anaemia requiring blood transfusion and iron infusion. Topical therapies used over 6 months were of limited benefit and colostomy was considered, but the condition completely resolved after commencement of oral loperamide. This demonstrates that perianal pseudoverrucous papules and nodules can be severe but are entirely reversible upon removal of the source of skin irritation...
September 21, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28930629/large-scale-replication-study-identified-multiple-independent-snps-in-ret-synergistically-associated-with-hirschsprung-disease-in-southern-chinese-population
#13
Yan Zhang, Qiuming He, Ruizhong Zhang, Hong Zhang, Wei Zhong, Huimin Xia
Hischsprung disease (HSCR) is an intestinal disorder with strong genetic components. RET was considered as the strongest contributor. Multiple single nucleotide polymorphisms (SNP) were demonstrated as associated with HSCR in different populations. However, whether the associations of reported SNPs derived from one causal variants or congregations of multiple variants were still not clear. In this study, we successfully genotyped 16 SNPs in RET with a largest case-control study to date, totaling 1470 HSCR and 1473 control subjects in South Chinese population...
September 20, 2017: Aging
https://www.readbyqxmd.com/read/28927976/quality-of-life-outcomes-in-children-with-hirschsprung-disease
#14
Lucy Collins, Brennan Collis, Misel Trajanovska, Rija Khanal, John M Hutson, Warwick J Teague, Sebastian K King
BACKGROUND: Morbidity following repair of Hirschsprung disease (HD) is common. However, quality of life (QoL) results focused on HD children are contradictory. We aimed to measure QoL outcomes in HD children using validated questionnaires. METHODS: Patients with HD, managed at a large tertiary pediatric institution between 2004 and 2013, were identified. Parents completed validated questionnaires. Results were compared with published healthy population controls...
September 2, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28924375/lncrna-afap1-as-functions-as-a-competing-endogenous-rna-to-regulate-rap1b-expression-by-sponging-mir-181a-in-the-hscr
#15
Guanglin Chen, Lei Peng, Zhongxian Zhu, Chunxia Du, Ziyang Shen, Rujin Zang, Yang Su, Yankai Xia, Weibing Tang
Background: Long noncoding RNAs (lncRNAs) have recently emerged as important regulators in a broad spectrum of cellular processes including development and disease. Despite the known engagement of the AFAP1-AS in several human diseases, its biological function in Hirschsprung disease (HSCR) remains elusive. Methods: We used qRT-PCR to detect the relative expression of AFAP1-AS in 64 HSCR bowel tissues and matched normal intestinal tissues. The effects of AFAP1-AS on cell proliferation, migration, cell cycle, apoptosis and cytoskeletal organization were evaluated using CCK-8, transwell assay, flow cytometer analysis and immunofluorescence, in 293T and SH-SY5Y cell lines, respectively...
2017: International Journal of Medical Sciences
https://www.readbyqxmd.com/read/28922260/increased-risk-of-inflammatory-bowel-disease-in-a-population-based-cohort-study-of-patients-with-hirschsprung-disease
#16
Anna Löf Granström, Leila Amin, Henrik Arnell, Tomas Wester
OBJECTIVES: Hirschsprung disease (HSCR) has previously been associated with inflammatory bowel disease (IBD). There are no data to show how common this association is. The aim of this study was to assess the risk of IBD in individuals with HSCR in a population-based cohort. METHODS: This was a nationwide, population-based cohort study. The study exposure was HSCR and the study outcome was IBD. The cohort included all individuals with HSCR registered in the Swedish National Patient Register between 1964-2013 and ten age- and sex-matched controls per patient, randomly selected from the Swedish Population Register...
September 16, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28920022/familial-near-total-intestinal-aganglionosis
#17
Hidouri Saida, Zitouni Hayet, Chahed Jamila, Mosbahi Sana, Belhassen Samia, Ksiaa Amine, Hmida Badii, Krichene Imed, Sahnoun Lassad, Mekki Mongi, Belguith Mohsen, Nouri Abdellatif
Near total aganglionosis represents the most extreme and rare form of Hirschsprung's disease. It can affect more than one member of family. We report three cases of near total intestinal aganglionosis in a family presenting with intestinal obstruction at birth. All of them were operated and a jejunostomy was performed. Outcome was dismal.
July 2017: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/28919319/a-population-based-description-of-familial-clustering-of-hirschsprung-s-disease
#18
Craig C Teerlink, Ryan Bernhisel, Lisa A Cannon-Albright, Michael D Rollins
BACKGROUND: Familial recurrence of Hirschsprung's disease (HSCR) is well documented, and risk estimates for relatives have been reported from various populations. We describe the familial clustering of HSCR cases using well-established unbiased familial aggregation techniques within the context of a population genealogy. METHODS: Patients included 264 HSCR cases identified using ICD-9 diagnosis coding from the two largest healthcare providers in Utah who also had linked genealogy data...
September 1, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28916047/anorectal-malformation-hirschsprung-s-disease-a-cross-sectional-comparison-of-quality-of-life-and-bowel-function-to-healthy-controls
#19
Shireen Anne Nah, Caroline C P Ong, Seyed Ehsan Saffari, Lin Yin Ong, Te-Lu Yap, Yee Low, Anette S Jacobsen
BACKGROUND: Patients with anorectal malformation (ARM) and Hirschsprung's disease (HD) face long-term disturbance in bowel function even after definitive surgery. This study evaluates the quality of life (QOL) of patients with ARM and HD, and compares them to healthy controls using self-report questionnaires. METHODOLOGY: A prospective study was performed recruiting patients with ARM or HD from September 2013 to December 2014 who had primary surgery done in our institution at least 2 years prior to participation...
August 25, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28910873/-differential-expression-and-clinical-significance-of-calretinin-in-total-colonic-aganglionosis
#20
L Q Ke, J Huang, L L Liu, M J Huang, Q Zhang, J C Wang, W Zhang
Objective: To evaluate the differential calretinin immunostaining in different segments of total colonic aganglionosis and its utility in the diagnosis. Methods: Nine specimens including ileum and colon segments were obtained from 9 patients with total colonic aganglionosis (TCA), from 2010 to 2016 year, in Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology. Another 9 ganglionic specimens including the same segments from patients with non-Hirschsprung disease (non-HD) patients were collected as control...
September 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
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