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hirschsprung's enterocolitis

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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/28208866/fatal-toxic-megacolon-in-a-child-of-hirschsprung-disease
#2
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/28196663/nos-interacting-protein-nosip-is-increased-in-the-colon-of-patients-with-hirschsprungs-s-disease
#3
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/28185631/single-stage-transanal-endorectal-pull-through-procedure-for-correction-of-hirschsprung-disease-in-neonates-and-nonneonates-a-multicenter-study
#4
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
#5
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/28154902/guidelines-for-the-diagnosis-and-management-of-hirschsprung-associated-enterocolitis
#6
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/28058486/current-status-of-hirschsprung-s-disease-based-on-a-nationwide-survey-of-japan
#7
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/28040830/redo-pullthrough-for-hirschsprung-disease
#8
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/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...
February 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27912977/functional-outcomes-in-hirschsprung-disease-a-single-institution-s-12-year-experience
#10
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...
February 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27858582/long-term-outcome-of-laparoscopic-duhamel-procedure-for-extended-hirschsprung-s-disease
#11
Cécile O Muller, Guillaume Rossignol, Louise Montalva, Jerome Viala, Christine Martinez-Vinson, Alexis Mosca, Dominique Berrebi, Arnaud Bonnard
BACKGROUND: The aim of our study was to report our experience in extended Hirschsprung's disease (HD) in children operated on by laparoscopy. PATIENTS AND METHODS: Retrospective data collection from a single center from 1991 to 2013 concerned extended forms of HD operated on by laparoscopic Duhamel procedure and included extension of aganglionosis, comorbidities, short and late postoperative outcome, and results of endoscopy when performed. RESULTS: Thirty patients presented an extended form of aganglionosis: 5 involving the transverse colon, 10 the right colon, and 15 the ileum (median length = 15 cm, range 1-60)...
December 2016: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/27853811/altered-expression-of-il36%C3%AE-and-il36-receptor-il1rl2-in-the-colon-of-patients-with-hirschsprung-s-disease
#12
Christian Tomuschat, Anne Marie O'Donnell, David Coyle, Prem Puri
PURPOSE: Hirschsprung's disease associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung's disease (HSCR). Altered intestinal epithelial barrier function and abnormal microbiota are implicated in the pathogenesis of HAEC. IL-36γ, a member of the IL-1 superfamily, is involved in host defense and contributes to proinflammatory responses and development of inflammatory diseases. The IL36 receptor (IL1RL2) is an important mediator molecule in the inflammatory response...
November 16, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27836361/ultrasound-guided-intrasphincteric-botulinum-toxin-injection-relieves-obstructive-defecation-due-to-hirschsprung-s-disease-and-internal-anal-sphincter-achalasia
#13
COMPARATIVE STUDY
Joseph T Church, Samir K Gadepalli, Toghrul Talishinsky, Daniel H Teitelbaum, Marcus D Jarboe
PURPOSE: Chronic obstructive defecation can occur in patients with Hirschsprung Disease (HD) and internal anal sphincter (IAS) achalasia. Injection of Botulinum Toxin (BoTox) into the IAS can temporarily relieve obstructive defecation, but can be challenging when performed by tactile sense alone. We compared results of BoTox injections with and without ultrasound (US) guidance. METHODS: We retrospectively reviewed BoTox injections into the IAS for obstructive defecation over 5years...
January 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27813711/a-scoring-system-to-predict-the-severity-of-hirschsprung-disease-at-diagnosis-and-its-correlation-with-molecular-genetics
#14
Raquel Núñez-Ramos, Raquel M Fernández, Miguel González-Velasco, Jesús Ruiz-Contreras, Enrique Galán-Gómez, Ramón Núñez-Núñez, Salud Borrego
OBJECTIVES: Hirschsprung disease (HSCR) has a wide range of severity. There are non-severe forms treated conservatively until surgery and severe forms that require an early stoma and prolonged hospitalization. Our objective was to establish a clinical scoring system to predict the severity of HSCR and to evaluate the possible existence of a clinical-genetic correlation. METHODS: We carried out a retrospective observational study including all HSCR cases treated in our hospital...
January 27, 2016: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/27765267/redo-pull-through-surgery-in-hirschsprung-s-disease-short-term-clinical-outcome
#15
Ajm Dingemans, Hjj van der Steeg, R Rassouli-Kirchmeier, M W Linssen, Ialm van Rooij, I de Blaauw
INTRODUCTION: Although surgery is effective in most patients with Hirschsprung's disease (HD), some have persistent obstructive symptoms. Additional medical treatment is generally sufficient, but a small fraction of these patients needs secondary surgery. Series on redo surgery are scarce. Aim of this study is to evaluate complications and clinical outcome of patients in need of redo surgery for HD. MATERIALS AND METHODS: Sixteen patients underwent redo endorectal pull-through surgery in our center between 2007 and 2015...
September 23, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27670962/enterocolitis-and-bowel-function-in-children-with-hirschsprung-disease-and-trisomy-21
#16
Natasha Kwendakwema, Ragheed Al-Dulaimi, Angela P Presson, Sarah Zobell, Austin M Stevens, Brian T Bucher, Douglas C Barnhart, Michael D Rollins
BACKGROUND/PURPOSE: The purpose of this study was to study the effect of trisomy 21 (T21) on enterocolitis rates and bowel function among children with Hirschsprung disease (HD). METHODS: A retrospective cohort study of patients with HD treated at our tertiary children's hospital (2000-2015) and a cohort of patients with HD treated in our pediatric colorectal center (CRC) (2011-2015) were performed. RESULTS: 26/207 (13%) patients with HD had T21...
December 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27660002/increased-act1-il-17r-expression-in-hirschsprung-s-disease
#17
Christian Tomuschat, Anne Marie O'Donnell, David Coyle, Prem Puri
PURPOSE: Hirschsprung's disease -associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung's disease (HSCR). Altered intestinal epithelial barrier function is implicated in the pathogenesis of HAEC. IL-17 is a proinflammatory cytokine that plays a crucial role in host defense against microbial organisms in the development of inflammatory diseases. Act1 is an essential adaptor molecule required for the IL-17-mediated inflammatory responses via interaction with IL-17 receptor (IL-17R)...
December 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27603009/characterization-of-intestinal-microbiomes-of-hirschsprung-s-disease-patients-with-or-without-enterocolitis-using-illumina-miseq-high-throughput-sequencing
#18
Yuqing Li, Valeriy Poroyko, Zhilong Yan, Liya Pan, Yi Feng, Peihua Zhao, Zhoulonglong Xie, Li Hong
Hirschsprung-associated enterocolitis (HAEC) is a life-threatening complication of Hirschsprung's disease (HD). Although the pathological mechanisms are still unclear, studies have shown that HAEC has a close relationship with the disturbance of intestinal microbiota. This study aimed to investigate the characteristics of the intestinal microbiome of HD patients with or without enterocolitis. During routine or emergency surgery, we collected 35 intestinal content samples from five patients with HAEC and eight HD patients, including three HD patients with a history of enterocolitis who were in a HAEC remission (HAEC-R) phase...
2016: PloS One
https://www.readbyqxmd.com/read/27586151/pathological-changes-of-interstitial-cells-of-cajal-and-ganglion-cells-in-the-segment-of-resected-bowel-in-hirschsprung-s-disease
#19
Xuyong Chen, Hongyi Zhang, Ning Li, Jiexiong Feng
OBJECTIVES: This study was conducted to investigate the pathological changes which occur in interstitial cells of Cajal (ICCs) and ganglion cells found in segments of resected bowel obtained from patients with Hirschsprung's disease (HD), as well as to explore the benefits of using a contrast enema (CE) with 24-h delayed X-ray films to predict the length of resected bowel. METHODS: We performed a retrospective analysis of 58 children with HD who had undergone the pull-through procedure...
November 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27581039/transition-zone-pull-through-in-hirschsprung-s-disease-a-tertiary-hospital-experience
#20
Dhruva N Ghosh, Yingrui Liu, Daniel T Cass, Soundappan S V Soundappan
BACKGROUND: Pull-through of ganglionic bowel is essential for successful treatment of Hirschsprung's disease. We studied the incidence of transition zone pull-through in our institution and compared its outcome with ganglionic bowel pull-through. METHODS: Children who underwent Soave's pull-through for Hirschsprung's disease from January 2005 to November 2012 were studied. Patients were divided into two groups: ganglionic bowel pull-throughs (Group 1) and transition zone pull-throughs (Group 2)...
August 31, 2016: ANZ Journal of Surgery
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