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hirschsprung's enterocolitis

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https://www.readbyqxmd.com/read/27912977/functional-outcomes-in-hirschsprung-disease-a-single-institution-s-12-year-experience
#1
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/27858582/long-term-outcome-of-laparoscopic-duhamel-procedure-for-extended-hirschsprung-s-disease
#2
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)...
November 18, 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
#3
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
#4
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...
October 27, 2016: 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
#5
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
#6
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
#7
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...
September 15, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27660002/increased-act1-il-17r-expression-in-hirschsprung-s-disease
#8
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)...
September 22, 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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/27570241/bowel-dysfunction-following-pullthrough-surgery-is-associated-with-an-overabundance-of-nitrergic-neurons-in-hirschsprung-disease
#12
Lily S Cheng, Dana M Schwartz, Ryo Hotta, Hannah K Graham, Allan M Goldstein
PURPOSE: Recent evidence suggests that patients with Hirschsprung disease (HD) have abnormal neurotransmitter expression in the ganglionated proximal colon. These alterations may cause persistent bowel dysfunction even after pullthrough surgery. We sought to quantify the proportion of nitrergic neurons in the ganglionic colon of HD patients and relate these findings to functional outcome. METHODS: The proximal resection margin from 17 patients with colonic HD who underwent a pullthrough procedure and colorectal tissue from 4 age-matched controls were immunohistochemically examined to quantify the proportion of nitrergic neurons...
November 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27502879/clinical-spectrum-of-neonates-presenting-with-pneumoperitoneum-a-retrospective-study
#13
Ahmed Hosni Morsi, Hossam Rashad Omar, Ahmed Osama, Ahmed Refaat Khodary
BACKGROUND: Neonatal pneumoperitoneum is attributed, in most of the reported cases, to necrotising enterocolitis (NEC). There are also other causes leading to free intraperitoneal air. The aim of this study is to describe the clinical spectrum, causes, management and outcome of neonates admitted with pneumoperitoneum in the paediatric surgery unit of a university hospital. SUBJECTS AND METHODS: This retrospective study included neonates having radiographic evidences of pneumoperitoneum from 2012 to the end of 2014...
July 2016: African Journal of Paediatric Surgery: AJPS
https://www.readbyqxmd.com/read/27471950/long-term-outcomes-of-children-with-transanal-endorectal-pull-through-and-a-review-of-the-literature
#14
Basak Erginel, Feryal Gun Soysal, Erbug Keskin, Aladdin Celik, Secil Yüksel, Tansu Salman
INTRODUCTION: The transanal endorectal pull-through (TERPT) procedure, the latest advancement in the surgical treatment of Hirschsprung's disease, has replaced most other surgical techniques in the last decade. PATIENTS AND METHODS: Between October 2002 and March 2014, a total of 22 patients diagnosed with Hirschsprung's disease underwent a one-stage TERPT operation. RESULTS: Resected segments included the rectosigmoid (seven patients), the descending colon (10 patients), and the transverse colon (five patients)...
July 29, 2016: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/27385111/long-term-results-of-transanal-pull-through-for-hirschsprung-s-disease-a-meta-analysis
#15
J Zimmer, C Tomuschat, P Puri
INTRODUCTION: Several operative techniques have been developed for the treatment of Hirschsprung's disease (HD) in the past decades. Since one-stage transanal pull-through (TAPT) was first performed in 1998, multiple studies have shown favourable short-and midterm results compared to other techniques with shorter operation length, shorter hospital stay and lower complication rates. The aim of this meta-analysis was to determine the longterm results following TAPT for HD. METHODS: A systematic literature search for relevant articles was performed in four databases using the following terms "Hirschsprung/Hirschsprung's disease", "aganglionosis", "transanal", "pullthrough/pull-through", "longterm/long-term" "results", "follow-up" and "outcome"...
August 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27384506/altered-expression-of-a-two-pore-domain-k2p-mechano-gated-potassium-channel-trek-1-in-hirschsprung-s-disease
#16
Christian Tomuschat, Anne Marie O'Donnell, David Coyle, Nickolas Dreher, Danielle Kelly, Prem Puri
BACKGROUND: The pathophysiology of Hirschsprung's disease (HSCR) is not fully understood. A significant proportion of patients have persisting bowel symptoms such as constipation, soiling, and enterocolitis despite correctly performed operations. Animal data suggest that stretch-activated 2-pore domain K(+) channels play a critical role in the maintenance of intestinal barrier integrity. METHODS: We investigated TREK-1 protein expression in ganglionic and aganglionic regions of HSCR patients (n = 10) vs...
November 2016: Pediatric Research
https://www.readbyqxmd.com/read/27369964/laparoscopic-assisted-pull-through-operation-for-hirschsprung-s-disease-a-systematic-review-and-meta-analysis
#17
C Tomuschat, J Zimmer, P Puri
PURPOSE: In the last two decades, laparoscopic-assisted pull-through (LAPT) has gained much popularity in the treatment of Hirschsprung's disease. The aim of this meta-analysis was to determine the long-term outcome of patients treated laparoscopically. METHODS: A systematic literature-based search for relevant cohorts was performed using the terms "Hirschsprung's disease and Laparoscopy", "Laparoscopic-assisted pull-through outcome", "Laparoscopic-assisted Soave pull-through" "Laparoscopic-assisted Swenson pull-through" and Laparoscopic-assisted Duhamel pull-through...
August 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27270548/novel-insights-into-the-pathogenesis-of-hirschsprung-s-associated-enterocolitis
#18
REVIEW
Chun-Lei Jiao, Xu-Yong Chen, Jie-Xiong Feng
OBJECTIVE: To systematically summary the updated results about the pathogenesis of Hirschsprung's-associated enterocolitis (HAEC). Besides, we discussed the research key and direction based on these results. DATA SOURCES: Our data cited in this review were obtained mainly from PubMed from 1975 to 2015, with keywords "Hirschsprung enterocolitis", "Hirschsprung's enterocolitis", "Hirschsprung's-associated enterocolitis", "Hirschsprung-associated enterocolitis", "HAEC", and "EC"...
June 20, 2016: Chinese Medical Journal
https://www.readbyqxmd.com/read/27217698/altered-tryptophan-hydroxylase-2-expression-in-enteric-serotonergic-nerves-in-hirschsprung-s-associated-enterocolitis
#19
David Coyle, Justin M Murphy, Brian Doyle, Anne Marie O'Donnell, John Gillick, Prem Puri
AIM: To determine if expression of colonic tryptophan hydroxylase-2 (TPH2), a surrogate marker of neuronal 5-hydroxytryptamine, is altered in Hirschsprung's-associated enterocolitis. METHODS: Entire resected colonic specimens were collected at the time of pull-through operation in children with Hirschsprung's disease (HSCR, n = 12). Five of these patients had a history of pre-operative Hirschsprung's-associated enterocolitis (HAEC). Controls were collected at colostomy closure in children with anorectal malformation (n = 10)...
May 21, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27109981/ten-year-experience-with-bowel-transplantation-at-seoul-st-mary-s-hospital
#20
H K Chang, S Y Kim, J I Kim, S I Kim, J K Whang, J Y Choi, J M Park, E S Jung, S E Rha, D G Kim, I S Moon, M D Lee
A retrospective review of intestinal transplantation (ITx) at Seoul St. Mary's Hospital was made by collecting clinical data over the past 10 years. Fifteen consecutive cases from 2004 were analyzed. Five children and 10 adults (6 months to 69 years of age) were included. Primary diseases in adults included 4 mesenteric vessel thromboses, 2 strangulations, and 1 each of visceral myopathy, malignant gastrointestinal stromal tumor (GIST), mesenteric lymphangiectasis, and injury. Pediatric cases involved 2 Hirschsprung disease, 2 visceral myopathy, and 1 necrotizing enterocolitis...
March 2016: Transplantation Proceedings
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