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Hirschsprungs

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https://www.readbyqxmd.com/read/29137805/duhamel-operation-vs-transanal-endorectal-pull-through-procedure-for-hirschsprung-disease-a-systematic-review-and-meta-analysis
#1
Yong-Zhong Mao, Shao-Tao Tang, Shuai Li
OBJECTIVE: To compare treatment outcomes in children with Hirschsprung's disease who underwent treatment using the Duhamel or TERPT surgical procedures. METHODS: Medline, Cochrane, EMBASE, and Google Scholar databases were searched through December 26, 2016. Search strings included Hirschsprung's disease, fecal incontinence, transanal endorectal pull-through, and Duhamel operation. Randomized controlled studies (RCTs) and retrospective studies that compared the treatment of Hirschsprung's disease in with TERPT or Duhamel surgical procedures in neonates, infants, or children were included...
October 16, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29127965/minimally-invasive-neonatal-surgery-hirschsprung-disease
#2
REVIEW
Atsuyuki Yamataka, Go Miyano, Masahiro Takeda
Transanal pull-through (TAPT) is the procedure of choice for treating Hirschsprung disease and should be performed with laparoscopic assistance using the anorectal line (ARL) to ensure optimum postoperative bowel function (POBF). The dentate line (DL) has traditionally been used as the landmark for commencing dissection during TAPT, but we prefer the ARL because the DL is too subjective and can be associated with risk for injury to delicate sensory innervation required for normal defecation in the anal transition zone...
December 2017: Clinics in Perinatology
https://www.readbyqxmd.com/read/29110829/hirschsprung-disease-bowel-function-beyond-childhood
#3
Tomas Wester, Anna Löf Granström
Hirschsprung disease is a developmental defect of the enteric nervous system characterized by lack of enteric neurons in the distal hindgut. There are numerous reports on short-term outcomes indicating that impaired bowel function is common. Recently, several controlled studies show that bowel function outcomes are affected beyond childhood, in adolescents and adults, compared with healthy control subjects. Constipation and fecal incontinence are common. The impaired bowel function appears to have a negative impact on quality of life, although, a majority of patients have adapted to their symptoms...
October 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29108502/colonic-adventitial-fibromuscular-dysplasia-a-nonspecific-arteriopathy-associated-with-hirschsprung-disease-and-other-obstructive-disorders
#4
Ameet I Thaker, Raj P Kapur
Background Smooth muscle differentiation ("adventitial fibromuscular dysplasia," AFD) was purported as specific to arteries in the transition zone of Hirschsprung disease (HSCR) patients. We investigated AFD in an HSCR population and controls and consider the pathogenesis and significance of the vascular pathology. Design Vascular histology in sections from colonic HSCR resections (n = 55) was compared with age- and site-matched controls with (n = 19) and without (n = 28) non-HSCR obstructive conditions...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29098737/structural-and-functional-differences-in-phox2b-frameshift-mutations-underlie-isolated-or-syndromic-congenital-central-hypoventilation-syndrome
#5
Simona Di Lascio, Roberta Benfante, Eleonora Di Zanni, Silvia Cardani, Annalisa Adamo, Diego Fornasari, Isabella Ceccherini, Tiziana Bachetti
Heterozygous mutations in the PHOX2B gene are causative of Congenital Central Hypoventilation Syndrome (CCHS), a neurocristopathy characterised by defective autonomic control of breathing due to the impaired differentiation of neural crest cells (NCCs). Among PHOX2B mutations, polyalanine (polyAla) expansions are almost exclusively associated with isolated CCHS, whereas frameshift variants, although less frequent, are often more severe than polyAla expansions and identified in syndromic CCHS. This paper provides a complete review of all the frameshift mutations identified in cases of isolated and syndromic CCHS reported in the literature as well as those identified by us and not yet published...
November 2, 2017: Human Mutation
https://www.readbyqxmd.com/read/29093530/sporadic-hirschsprung-disease-mutational-spectrum-and-novel-candidate-genes-revealed-by-next-generation-sequencing
#6
Zhen Zhang, Qi Li, Mei Diao, Na Liu, Wei Cheng, Ping Xiao, Jizhen Zou, Lin Su, Kaihui Yu, Jian Wu, Long Li, Qian Jiang
Hirschsprung disease (HSCR) is a common cause of functional colonic obstruction in children. The currently available genetic testing is often inadequate as it mainly focuses on RET and several other genes, accounting for only 15-20% of cases. To identify novel, potentially pathogenic variants, we isolated a panel of genes from a whole-exome sequencing study and from the published mouse aganglionosis phenotypes, enteric nervous system development, and a literature review. The coding exons of 172 genes were analyzed in 83 sporadic patients using next-generation sequencing...
November 1, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29090971/downregulation-of-microrna-483-5p-promotes-cell-proliferation-and-invasion-by-targeting-gfra4-in-hirschsprung-s-disease
#7
Gang Wang, Feng Guo, Hefeng Wang, Wei Liu, Lijuan Zhang, Mingyu Cui, Xiangyu Wu
Recent studies have suggested the critical roles of miRNAs for disease progression. miRNA-483-5p (miR-483-5p) was previously found to have a relationship with tumor cell behavior, but its biological function in Hirschsprung's disease (HSCR) remains undefined. Thus, we explored the role of miR-483-5p in the pathogenesis of HSCR. Histological changes of colonic tissues were evaluated by hematoxylin and eosin (HE) staining. Quantitative real-time PCR and western blotting were used to determine relative expression levels of miRNA, mRNA, and proteins in 20 HSCR patients and 20 normal colon tissues...
November 2017: DNA and Cell Biology
https://www.readbyqxmd.com/read/29079318/transitions-in-care-from-pediatric-to-adult-general-surgery-evaluating-an-unmet-need-for-patients-with-anorectal-malformation-and-hirschsprung-disease
#8
Sarah B Cairo, Priscilla P L Chiu, Roshni Dasgupta, Karen A Diefenbach, Allan M Goldstein, Nicholas A Hamilton, Andrea Lo, Michael D Rollins, David H Rothstein
BACKGROUND: The provision of timely and comprehensive transition of care from pediatric to adult surgical providers for patients who have undergone childhood operations remains a challenge. Understanding the barriers to transition from a patient and family perspective may improve this process. METHODS: A cross-sectional survey was conducted of patients with a history of anorectal malformation (ARM) or Hirschsprung Disease (HD) and their families. The web-based survey was administered through two support groups dedicated to the needs of individuals born with these congenital abnormalities...
October 7, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29078343/dual-origin-of-enteric-neurons-in-vagal-schwann-cell-precursors-and-the-sympathetic-neural-crest
#9
Isabel Espinosa-Medina, Ben Jevans, Franck Boismoreau, Zoubida Chettouh, Hideki Enomoto, Thomas Müller, Carmen Birchmeier, Alan J Burns, Jean-François Brunet
Most of the enteric nervous system derives from the "vagal" neural crest, lying at the level of somites 1-7, which invades the digestive tract rostro-caudally from the foregut to the hindgut. Little is known about the initial phase of this colonization, which brings enteric precursors into the foregut. Here we show that the "vagal crest" subsumes two populations of enteric precursors with contrasted origins, initial modes of migration, and destinations. Crest cells adjacent to somites 1 and 2 produce Schwann cell precursors that colonize the vagus nerve, which in turn guides them into the esophagus and stomach...
October 24, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29075908/are-all-patients-with-short-segment-hirschsprung-s-disease-equal-a-retrospective-multicenter-study
#10
Patrick Ho Yu Chung, Kenneth Kak Yuen Wong, Paul Kwong Hang Tam, Michael Wai Yip Leung, Nicholas Sih Yin Chao, Kelvin Kam Wing Liu, Edwin Kin Wai Chan, Yuk Him Tam, Kim Hung Lee
BACKGROUND/PURPOSE: Short segment Hirschsprung's disease (HSCR) carries a better prognosis than long segment disease, but the definition of short is controversial. The objective of this study is to determine anatomically the extent of disease involvement that would be associated with a better functional outcome. METHODS: This is a retrospective multicenter (n = 3) study with patients (≥ 3 years) who had transanal pullthrough operation done for aganglionosis limited to the recto-sigmoid colon were reviewed...
October 26, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29059696/social-morbidity-in-relation-to-bowel-functional-outcomes-and-quality-of-life-in-anorectal-malformations-and-hirschsprung-s-disease
#11
Kristiina Kyrklund, Malla I Neuvonen, Mikko P Pakarinen, Risto J Rintala
No abstract text is available yet for this article.
October 23, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29050236/downregulation-of-lncrna-meg3-and-mir-770-5p-inhibit-cell-migration-and-proliferation-in-hirschsprung-s-disease
#12
Hongxing Li, Bo Li, Dongmei Zhu, Hua Xie, Chunxia Du, Yankai Xia, Weibing Tang
The long noncoding RNA (lncRNA) MEG3 is involved in various biological processes including cell migration and cell proliferation. In present study, it was found that MEG3 and the intronic miR-770-5p were decreased in samples from HSCR patients. Besides, knockdown of MEG3 and miR-770-5p suppressed cell migration and proliferation, while cell cycle and apoptosis were not affected in human 293T and SH-SY5Y cells. SRGAP1 mRNA and protein upregulation was inversely correlated with miR-770-5p expression in tissue samples and cell lines, which was confirmed to be a target gene of miR-770-5p by dual-luciferase reporter assay...
September 19, 2017: Oncotarget
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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