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hirschsprung's enterocolitis

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
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
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
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
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
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
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
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
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
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
Christian Tomuschat, Anne Marie O'Donnell, David Coyle, Nickolas Dreher, Danielle Kelly, Prem Puri
BackgroundThe 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.MethodsWe investigated TREK-1 protein expression in ganglionic and aganglionic regions of HSCR patients (n=10) versus normal control colon (n=10)...
July 6, 2016: Pediatric Research
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
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
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
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
Ü Adıgüzel, K Ağengin, I Kırıştıoğlu, H Doğruyol
INTRODUCTION: Transanal endorectal pull-through (TEPT) is the latest advancement in the treatment of Hirschsprung's disease (HD). The aim of this study was to evaluate the safety and efficacy of TEPT as a definitive treatment for patients with HD. PATIENTS AND METHODS: A retrospective study of 50 patients who underwent TEPT at Uludağ University, Bursa, Turkey, between June 2001 and April 2012 was conducted. These patients were evaluated with regard to their age, sex, preoperative findings, and length of the aganglionic segment, intraoperative and postoperative complications, and results of the follow-up...
March 29, 2016: Irish Journal of Medical Science
Christian Tomuschat, Anne Marie O'Donnell, David Coyle, Nickolas Dreher, Danielle Kelly, 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 has been suggested to play a role in the causation of HAEC. In rodent experimental models of colitis, a decreased expression of K(ATP) channels (Subunits: Kir6.1/6.2 and SUR1/2) is reported. We designed this study to determine if K(ATP) channels exist within the human colon and to investigate the expression of different subunits in Hirschsprung's disease...
June 2016: Journal of Pediatric Surgery
Mariana Tresoldi das Neves Romaneli, Antonio Fernando Ribeiro, Joaquim Murray Bustorff-Silva, Rita Barbosa de Carvalho, Elizete Aparecida Lomazi
OBJECTIVE: To describe the case of an infant with Hirschsprung's disease presenting as total colonic aganglionosis, which, after surgical resection of the aganglionic segment persisted with irreversible functional intestinal obstruction; discuss the difficulties in managing this form of congenital aganglionosis and discuss a plausible pathogenetic mechanism for this case. CASE DESCRIPTION: The diagnosis of Hirschsprung's disease presenting as total colonic aganglionosis was established in a two-month-old infant, after an episode of enterocolitis, hypovolemic shock and severe malnutrition...
September 2016: Revista Paulista de Pediatria: Orgão Oficial da Sociedade de Pediatria de São Paulo
Ankush Gosain
Hirschsprung's disease (HSCR) is a common cause of neonatal bowel obstruction and the approach to diagnosis and surgical treatment is well defined and accepted. Hirschsprung's-associated enterocolitis (HAEC) remains a frequent cause of pre-operative and post-operative morbidity and mortality, with unchanged treatment guidelines over multiple decades. Recent advances in our understanding of the genetics underlying HSCR have allowed the development of animal models, some of which recapitulate the HAEC phenotype...
April 2016: Pediatric Surgery International
Sebastian O Ekenze, Obinna V Ajuzieogu, Benedict C Nwomeh
BACKGROUND: Disparity still exists in the outcome of neonatal surgery between high-income countries and low-income and middle-income countries. This study reviews publications on neonatal surgery in Africa over 20 years with a focus on challenges of management, trends in outcome, and potential interventions to improve outcome. METHODS: We did a literature review by searching PubMed and African Index Medicus for original articles published in any language between January 1995 and September 2014...
March 2016: Pediatric Surgery International
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