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Journal articles on the topic 'Hirschsprung associated enterocolitis'

Author: Grafiati

Published: 5 June 2025

Last updated: 2 August 2025

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1

Li, Shuai, Yichun Zhang, Kang Li, et al. "Update on the Pathogenesis of the Hirschsprung-Associated Enterocolitis." International Journal of Molecular Sciences 24, no. 5 (2023): 4602. http://dx.doi.org/10.3390/ijms24054602.

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Despite the significant progress that has been made in terms of understanding the pathophysiology and risk factors of Hirschsprung-associated enterocolitis (HAEC), the morbidity rate has remained unsatisfactorily stable, and clinical management of the condition continues to be challenging. Therefore, in the present literature review, we summarized the up-to-date advances that have been made regarding basic research on the pathogenesis of HAEC. Original articles published between August 2013 and October 2022 were searched in a number of databases, including PubMed, Web of Science, and Scopus. T

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Tran Viet, Hoang, Tuan Huynh Minh, Nhan Vu Truong, et al. "Pre- and Postoperative Risk Factors for Hirschsprung-Associated Enterocolitis in Vietnamese Children." Gastrointestinal Disorders 7, no. 1 (2025): 17. https://doi.org/10.3390/gidisord7010017.

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Background/Objective: Hirschsprung-associated enterocolitis (HAEC) can occur before and after surgery, increasing the complication rates, hospital stay, and treatment costs. This study aims to determine the incidence of preoperative and postoperative Hirschsprung-associated enterocolitis HAEC and the related risk factors. Methods: This study is a prospective cohort study of Hirschsprung’s disease patients under 16 years of age at two Children’s Hospitals in Ho Chi Minh City, Vietnam from December 2022 to June 2024. The postoperative follow-up is 12 months. Results: We enrolled 84 pediatric pat

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Glushkova, Victoria A., Aleksey V. Podkamenev, and Tatyana V. Gabrusskaya. "Crohn’s disease in patients with Hirschsprung disease and Hirschsprung-associated enterocolitis: A review." Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care 14, no. 1 (2024): 97–106. http://dx.doi.org/10.17816/psaic1780.

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In the last decade, the incidence of inflammatory bowel disease in children has been increasing. Some studies have reported the development of Crohn’s disease in patients with Hirschsprung disease and Hirschsprung-associated enterocolitis. Suggestions have been made about the possible causes of the development of Crohn’s-like lesions after intestinal resection in early childhood. This study aimed to summarize information about Hirschsprung-associated Crohn’s disease and Crohn’s-like lesions after surgical treatment of Hirschsprung disease. The search was carried out in scientific databases of

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Morozov, D. A., E. S. Pimenova, and G. A. Korolev. "Hirschsprung-associated enterocolitis in children." Voprosy praktičeskoj pediatrii 14, no. 2 (2019): 35–42. http://dx.doi.org/10.20953/1817-7646-2019-2-35-42.

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Hagens, J., K. Reinshagen, and C. Tomuschat. "Prevalence of Hirschsprung-associated enterocolitis in patients with Hirschsprung disease." Pediatric Surgery International 38, no. 1 (2021): 3–24. http://dx.doi.org/10.1007/s00383-021-05020-y.

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Abstract Purpose Hirschsprung's associated enterocolitis (HAEC) is a complication of Hirschsprung's Disease (HD) with considerable morbidity and mortality. The variability in presentation leads to a wide variety of the reported prevalence pre-and postoperatively. This systematic review aimed to clarify the prevalence of HAEC in short—(S-HD), long (L-HD), TCA and the type of operation used. Methods A systematic literature-based search for relevant cohorts was performed using Pubmed/Medline, Cochrane Library from its inception to May 2021. Studies reporting on pre-and postoperative enterocolitis

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Ziogas, Ioannis A., Korah P. Kuruvilla, Ming Fu, and Ankush Gosain. "Hirschsprung-associated enterocolitis: a comprehensive review." World Journal of Pediatric Surgery 7, no. 3 (2024): e000878. http://dx.doi.org/10.1136/wjps-2024-000878.

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Hirschsprung-associated enterocolitis (HAEC) is an important cause of morbidity and the leading cause of mortality in patients with Hirschsprung disease. The pathophysiology of disease includes dysmotility of the enteric nervous system, dysbiosis of the microbiota, failure of the intestinal barrier, and impaired immunity. Common manifestations include fever, abdominal distension, lethargy, vomiting, and diarrhea. Given the non-specific signs and symptoms of HAEC, high clinical suspicion is warranted, especially in patients with risk factors. Diagnosis and management of HAEC depend on the sever

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Chantakhow, Sireekarn, Jiraporn Khorana, Kanokkan Tepmalai, Nonglak Boonchooduang, Nipon Chattipakorn, and Siriporn C. Chattipakorn. "Alterations of Gut Bacteria in Hirschsprung Disease and Hirschsprung-Associated Enterocolitis." Microorganisms 9, no. 11 (2021): 2241. http://dx.doi.org/10.3390/microorganisms9112241.

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Hirschsprung-associated enterocolitis (HAEC) is a common life-threatening complication of Hirschsprung disease (HSCR). It has been proposed that gut microbiota, which have an essential role in gut-homeostasis, are associated with HAEC. Recent studies demonstrated an increase in alpha diversity of fecal microbiota over time in HSCR mice and a decrease in diversity after surgery. In addition, clinical studies have reported a reduction in bacterial richness in HSCR children after surgery. Some studies revealed a difference in microbiota between the proximal ganglionic and distal aganglionic intes

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Frykman, Philip K., and Scott S. Short. "Hirschsprung-associated enterocolitis: prevention and therapy." Seminars in Pediatric Surgery 21, no. 4 (2012): 328–35. http://dx.doi.org/10.1053/j.sempedsurg.2012.07.007.

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Hadi, Kemal Fadhli, and Riana Pauline Tamba. "004. Confirming The Diagnosis of Hirschsprung Associated Enterocolitis Based on The Hirschsprung Associated Enterocolitis Guidelines: A Literature Review." JBN (Jurnal Bedah Nasional) 8, no. 2 (2024): 4. http://dx.doi.org/10.24843/jbn.2024.v08.is02.p004.

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Background: Hirschsprung's disease (HD) is one of the most common causes of intestinal obstruction in neonates. One of the most frequently encountered complications, and the leading cause of morbidity and mortality in HD patients, is Hirschsprung-associated enterocolitis (HAEC). In 2017, Gosain et al. developed a guideline to aid in the diagnosis of HAEC, but there are limited studies discussing this guideline and diagnosing HAEC remains unclear. The aim of this study is to establish the diagnosis of HAEC in patients using the HAEC guideline. Methods: The studies were selected from online data

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Mariana, Nita, Andi Asadul Isl, Mochammad Hatta, and Harsali Fransiscus. "IL23 mRNA Expression in Hirschsprung-Associated Enterocolitis." Journal of Medical Sciences 20, no. 2 (2020): 39–43. http://dx.doi.org/10.3923/jms.2020.39.43.

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Demehri, Farokh R., Ihab F. Halaweish, Arnold G. Coran, and Daniel H. Teitelbaum. "Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention." Pediatric Surgery International 29, no. 9 (2013): 873–81. http://dx.doi.org/10.1007/s00383-013-3353-1.

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12

Lampus, Harsani. "Overview of Hirschsprung Disease: A Narrative Literature Review." Sriwijaya Journal of Pediatrics 1, no. 1 (2023): 14–16. http://dx.doi.org/10.59345/sjped.v1i1.14.

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Hirschsprung disease is a congenital malformation resulting from the failure of neural crest cells to migrate into the gastrointestinal tract. The exact cause of this disorder is unknown, but it is suspected that there is an interaction of several factors, including gene mutations and epigenetic mechanisms. This literature review aimed to describe the pathophysiology, clinical symptoms, and treatment of Hirschprung disease. Mild to severe constipation is a common manifestation of Hirschsprung's disease with poor diet, poor weight gain, and progressive abdominal distention. However, diarrhea ma

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Song, Yue, Changqiang Yang, and Hua Wang. "Tuberculosis in an infant with Hirschsprung-associated enterocolitis: a case report." Journal of International Medical Research 49, no. 9 (2021): 030006052110434. http://dx.doi.org/10.1177/03000605211043412.

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Hirschsprung-associated enterocolitis (HAEC) is a serious and life-threatening condition, and atypical tuberculosis (TB) associated with HAEC is even more serious. A male newborn aged 4 days was diagnosed with Hirschsprung disease and transanal Soave pull-through was performed at 4 months old. Six months later, he suffered from enterocolitis. Although he was treated with multiple broad-spectrum antibiotics for 2 weeks, he developed a fever without any other symptoms for TB infection. We found numerous, bilateral, uniformly distributed, small pulmonary nodules in the lower lobes in an abdominal

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Watanabe, Toshihiko, Masataka Takahashi, Kensuke Shoji, et al. "Human parechovirus-3 infection mimicking Hirschsprung-associated enterocolitis." Journal of Pediatric Surgery Case Reports 2, no. 2 (2014): 58–60. http://dx.doi.org/10.1016/j.epsc.2014.01.001.

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15

Lacher, Martin, Guido Fitze, Johanna Helmbrecht, et al. "Hirschsprung-associated enterocolitis develops independently of NOD2 variants." Journal of Pediatric Surgery 45, no. 9 (2010): 1826–31. http://dx.doi.org/10.1016/j.jpedsurg.2010.02.039.

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Abdul-Raheem, Jareatha, Lauren Gabreski, Meredith Schuldt, and Karla Adams. "Rare encounter of Hirschsprung’s disease and CARD11 mutation: A diagnostic challenge." Journal of Case Reports and Images in Pediatrics 6, no. 2 (2024): 11–16. https://doi.org/10.5348/100027z19yg2024cr.

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Introduction: Hirschsprung’s disease (HD), a congenital condition characterized by aganglionosis, is associated with enterocolitis, which requires repeated antibiotic exposure to drugs associated with drug-induced lupus (DIL). Drug-induced lupus mimics systemic lupus erythematosus (SLE) but typically resolves upon discontinuation of the triggering medication. CARD11 is essential for lymphocyte signaling and immune regulation, and its mutations are associated with immune disorders. Case Report: A five-year-old male with HD and Hirschsprung-associated enterocolitis (HAEC) developed symptoms of D

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Budianto, Iskandar Rahardjo, Agus Firmansyah, Yefta Moenadjat, Ahmad Aulia Jusuf, and Vivian Soetikno. "Paneth Cell Hyperplasia and Metaplasia in Hirschsprung-associated Enterocolitis in An Aganglionosis Rat Model." Indonesian Biomedical Journal 14, no. 4 (2022): 393–400. http://dx.doi.org/10.18585/inabj.v14i4.2007.

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BACKGROUND: Many hypotheses regarding the pathophysiology of enterocolitis in aganglionic megacolon or Hirschsprung disease (HSCR) has been proposed. Paneth cells are columnar intestinal epithelial cells that have an important role in maintaining of intestinal homeostasis as a bactericide. Since enterocolitis in HSCR may have association with Paneth cells metaplasia and hyperplasia, current study investigated Paneth cells metaplasia and hyperplasia in the sigmoid colon of HSCR rat model and its products, namely a-defensins and IL-1b, in the sigmoid colon tissues.METHODS: Aganglionosis-induced

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Larasati, Elika, I. Made Darmajaya, and Nyoman Golden. "Faktor-faktor pasca operasi satu tahap yang mempengaruhi kejadian hirschsprung associated enterocolitis pada pasien hirschsprung disease." Intisari Sains Medis 15, no. 1 (2024): 103–8. http://dx.doi.org/10.15562/ism.v15i1.1940.

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Background: Hirschsprung-Associated Enterocolitis (HAEC) is a life-threatening consequence of Hirschsprung Disease (HD) that was initially documented in the late 19th century. The occurrence of HAEC did not exhibit any changes from the preoperative phase to the postoperative phase and can manifest at any point from infancy to adulthood. To date, no research has been conducted on the factors influencing the occurrence of HAEC in Hirschsprung patients who undergo a one-stage procedure. Thus, this study aims to investigate the factors that influence the occurrence of HAEC in patients with Hirschs

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Sofyan, Ahmad, Wuri Iswarsigit, Joshua Alward Herdiman, and Pathur Rahman Nasution. "Short-Term Complications Between Duhamel and Soave Procedures in Hirschsprung Patients: A Systematic Review." Asian Journal of Healthy and Science 3, no. 8 (2024): 210–19. http://dx.doi.org/10.58631/ajhs.v3i8.124.

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Hirschsprung's disease, or congenital megacolon, is a birth defect where ganglions are absent in the gut wall, leading to constipation and intestinal obstruction. It affects 1 in 500 births worldwide and, if untreated or treated late, significantly impacts children's growth and quality of life. Understanding the short-term complications of the Soave and Duhamel methods in Hirschsprung disease patients. A systematic review was conducted based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. In our initial PubMed search and hand-searching, we identif

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Vilanova Sanchez, Alejandra, Paloma Triana Junco, S. Barrena, et al. "Reliability of the Hirschsprung-Associated Enterocolitis Score in Clinical Practice." European Journal of Pediatric Surgery 29, no. 01 (2019): 132–37. http://dx.doi.org/10.1055/s-0038-1677046.

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Introduction There is a lack of an agreed Hirschsprung-associated enterocolitis (HAEC) definition. In 2009, a HAEC score was proposed for the diagnosis of HAEC episodes. Our aim was to apply the HAEC score on HAEC episodes to determine its diagnostic efficiency and whether it correlated to its severity. Methods Retrospective study of patients with HAEC admitted between 2000 and 2016. Episodes of HAEC were identified and the HAEC score was calculated. A cut-off of ≥ 10 according to Pastor et al and ≥ 4 according to Frykman et al were used. A Pearson's correlation coefficient was performed for o

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Le-Nguyen, Annie, Franziska Righini-Grunder, Nelson Piché, Christophe Faure, and Ann Aspirot. "Factors influencing the incidence of Hirschsprung associated enterocolitis (HAEC)." Journal of Pediatric Surgery 54, no. 5 (2019): 959–63. http://dx.doi.org/10.1016/j.jpedsurg.2019.01.026.

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Mariana, Nita, Andi Asadul Islam, Muhammad Nasrum Massi, Warsinggih, Mochammad Hatta, and Ilhamjaya Patellongi. "ITGB2 (CD18) mRNA Expression in Hirschsprung-Associated Enterocolitis (HAEC)." Indian Journal of Public Health Research & Development 10, no. 8 (2019): 1015. http://dx.doi.org/10.5958/0976-5506.2019.02029.1.

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Li, B., Z. Zhang, D. Lee, C. Lee, Q. Jiang, and A. Pierro. "A35 INTESTINAL REGENERATION IS IMPAIRED IN HIRSCHSPRUNG-ASSOCIATED ENTEROCOLITIS." Journal of the Canadian Association of Gastroenterology 7, Supplement_1 (2024): 19–20. http://dx.doi.org/10.1093/jcag/gwad061.035.

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Wu, Lihua, Ya Gao, Ruijie Zhou, et al. "Predictive value of plasma zonulin for postoperative Hirschsprung-associated enterocolitis." World Journal of Pediatric Surgery 8, no. 3 (2025): e001057. https://doi.org/10.1136/wjps-2025-001057.

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Background Hirschsprung’s disease (HSCR) is a functional obstruction of the gastrointestinal tract characterized by abdominal distension, constipation, and vomiting. The protein zonulin serves as a biomarker for intestinal permeability. We sought to explore the changes in plasma zonulin levels in patients with HSCR and to assess its predictive role in the development of postoperative Hirschsprung-associated enterocolitis (HAEC). Methods There are 60 patients with HSCR were recruited for this study, categorized into short-segment disease (S-HSCR) (n=33), long-segment disease (L-HSCR) (n=15), an

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Falah, Erfano Muhammad. "PROFIL PENGGUNAAN ANTIBIOTIK PASIEN HAEC PASCA OPERASI DI RSUD DR. SOETOMO TAHUN 2015-2018." Jurnal Kedokteran Syiah Kuala 18, no. 2 (2018): 76–79. http://dx.doi.org/10.24815/jks.v18i2.17994.

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Abstract. HAEC (Hirschsprung-associated Enterocolitis) is one of serious and life-threatening complication of patient with HD (Hirschsprung’s Disease). The etiology of HAEC is still unknown until now, however, the existence of gram-negative bacterial especially C. Dificille has been linked to occurrence of patient with HAEC. HAEC frequently found at patient with HD post-operative surgery. The treatment for HD patient post-operative with HAEC are rectal irrigation, fluid therapy, and antibiotics. Because of the wide variety of sign and symptoms in HAEC post operative patient, under treatment ha

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Witarto, Andro Pramana, Alpha Fardah Athiyyah, I. G. B. Adria Hariastawa, and I. Gusti Made Reza Gunadi Ranuh. "RISK FACTORS INFLUENCING ENTEROCOLITIS DEVELOPMENT IN PEDIATRIC PATIENTS WITH HIRSCHSPRUNG’S DISEASE." Jurnal Berkala Epidemiologi 8, no. 3 (2020): 218. http://dx.doi.org/10.20473/jbe.v8i32020.218-227.

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Background: Hirschsprung-associated enterocolitis (HAEC) is one of the worst and most common complications of Hirschsprung’s disease (HD). The mortality rate of HD patients with enterocolitis is still considered to be higher compared to that of those without enterocolitis. Purpose: This study aimed to identify and evaluate potential risk factors for HAEC development. Methods: A cross-sectional study was conducted using secondary data from the medical records of HD patients treated from January 2015 to September 2018 at Regional Public Hospital (RSUD) Dr. Soetomo, Surabaya. The inclusion criter

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Lewit, Ruth A., Laura V. Veras, Robert A. Cowles, et al. "Reducing Underdiagnosis of Hirschsprung-Associated Enterocolitis: A Novel Scoring System." Journal of Surgical Research 261 (May 2021): 253–60. http://dx.doi.org/10.1016/j.jss.2020.12.030.

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Parahita, Isidora Galuh, Akhmad Makhmudi, and Gunadi. "Comparison of Hirschsprung-associated enterocolitis following Soave and Duhamel procedures." Journal of Pediatric Surgery 53, no. 7 (2018): 1351–54. http://dx.doi.org/10.1016/j.jpedsurg.2017.07.010.

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Pruitt, Liese C. C., David E. Skarda, Michael D. Rollins, and Brian T. Bucher. "Hirschsprung-associated enterocolitis in children treated at US children's hospitals." Journal of Pediatric Surgery 55, no. 3 (2020): 535–40. http://dx.doi.org/10.1016/j.jpedsurg.2019.10.060.

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Gosain, Ankush, Philip K. Frykman, Robert A. Cowles, et al. "Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis." Pediatric Surgery International 33, no. 5 (2017): 517–21. http://dx.doi.org/10.1007/s00383-017-4065-8.

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Mariana, Nita, Mochammad Hatta, Emiliana Lia, et al. "Toll-like receptor-8 mRNA expression as a predictor of enterocolitis in hirschsprung disease: a cross-sectional study." International Journal of Surgery Open 62, no. 4 (2024): 401–5. http://dx.doi.org/10.1097/io9.0000000000000148.

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Introduction: Hirschsprung-associated enterocolitis (HAEC) is an inflammatory intestinal condition occurring in patients with Hirschsprung disease (HD). While the exact pathogenesis remains unclear, factors such as intestinal mucosal barrier injury, abnormal immune responses, and pathogenic infections contribute to HAEC. Toll-like receptors (TLRs) 7 and 8 are key in viral recognition and correlate with intestinal mucosal inflammation. In particular, TLR-8 contributes to antiviral responses, and chronic inflammation, and can be targeted with adjuvants. This study investigates the relationship b

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Culnan, Stephanie, Alyssa Carlson, and Meredith L. Farmer. "Bridging Knowledge and Practice: Insights Into Hirschsprung’s Disease and Home Management." Neonatal Network 44, no. 3 (2025): 167–76. https://doi.org/10.1891/nn-2024-0067.

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Hirschsprung’s disease (HD) is a congenital intestinal motility disorder characterized by the absence of ganglionic cells in the distal intestine, leading to functional bowel obstruction and affecting 1 in every 5,000 neonates. A serious complication of HD is Hirschsprung-associated enterocolitis (HAEC), a life-threatening systemic illness responsible for a significant proportion of HD-related mortality. Proper management of HD, especially preventing HAEC, includes the use of rectal irrigations, which caregivers must master before their neonate’s discharge. However, barriers such as short NICU

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Zhao, Lifu, Deepti Dhall, Zhi Cheng, et al. "Murine model of Hirschsprung-associated enterocolitis II: Surgical correction of aganglionosis does not eliminate enterocolitis." Journal of Pediatric Surgery 45, no. 1 (2010): 206–12. http://dx.doi.org/10.1016/j.jpedsurg.2009.10.035.

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Sellers, Margarita, Clara Udaondo, Bárbara Moreno, et al. "Hirschsprung-associated enterocolitis: Observational study in a paediatric emergency care unit." Anales de Pediatría (English Edition) 88, no. 6 (2018): 329–34. http://dx.doi.org/10.1016/j.anpede.2017.07.006.

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Askarpour, S., H. Javaherizadeh, M. Peyvasteh, A. Mohammadi, and S. Soma Seyedsalehi. "Investigating the Diagnostic and Risk Factors for Enterocolitis in Children with Hirschsprung’s Disease." Acta Medica Bulgarica 52, no. 1 (2025): 48–52. https://doi.org/10.2478/amb-2025-0007.

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Abstract Aim This study aimed to identify the clinical risk factors associated with Hirschsprung-associated enterocolitis (HAEC) in children with Hirschsprung’s disease (HD). Method A total of 75 children diagnosed with HD were recruited in this observational prospective study. Then, the clinical and paraclinical symptoms of children with Hirschsprung’s disease (n = 57) were compared to those with HAEC (n = 18). P-value < 0.05 was considered statistically significant. Results No significant difference was found between the two groups in terms of age (P = 0.72), gender (P = 0.51), and family

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Govorukhina, Olga A. "Transanal Endorectal Pull-Through in Children with Hirschsprung Disease: 15 Years of Single-Center Experience." Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care 15, no. 2 (2025): 155–62. https://doi.org/10.17816/psaic1898.

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BACKGROUND: Hirschsprung disease is one of the most complex and severe congenital malformations. The choice of surgical treatment depends on the wide spectrum of disease forms and clinical manifestations. Currently, the transanal endorectal pull-through technique is considered the most promising surgical approach. The greatest challenge is the management of complications such as Hirschsprung-associated enterocolitis and coloanal anastomotic stricture. The implementation of new surgical techniques allows for a reduction in postoperative complications and improvement in treatment outcomes in pat

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Rentea, Rebecca M., Janelle R. Noel-MacDonnell, Brian T. Bucher, et al. "Impact of Botulinum Toxin on Hirschsprung-Associated Enterocolitis After Primary Pull-Through." Journal of Surgical Research 261 (May 2021): 95–104. http://dx.doi.org/10.1016/j.jss.2020.12.018.

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Svetanoff, Wendy Jo, Joseph Lopez, Pablo Aguayo, Richard J. Hendrickson, Tolulope A. Oyetunji, and Rebecca M. Rentea. "The impact of botulinum injection for hospitalized children with Hirschsprung-associated enterocolitis." Pediatric Surgery International 37, no. 10 (2021): 1467–72. http://dx.doi.org/10.1007/s00383-021-04966-3.

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Shi, Hao, Yong She, Wu Mao, et al. "16S rRNA Sequencing Reveals Alterations of Gut Bacteria in Hirschsprung-Associated Enterocolitis." Global Medical Genetics 11, no. 04 (2024): 263–69. http://dx.doi.org/10.1055/s-0044-1789237.

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AbstractHirschsprung-associated enterocolitis (HAEC) stands as most common and serious complication of Hirschsprung's disease. Variations in the microbiota composition may account for the differences observed between HAEC and healthy individuals, offering crucial insights into the disease's pathogenesis. Here, we performed a study to changes in the gut microbiome using 16sRNA amplicon sequencing in a cohort of HAEC patients (n = 16) and healthy controls (n = 14). Our result revealed a significant disparity in beta diversity between the two groups. Following correction for false discovery rate,

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Halleran, Devin R., Hira Ahmad, Elias Maloof, et al. "Does Hirschsprung-Associated Enterocolitis Differ in Children With and Without Down Syndrome?" Journal of Surgical Research 245 (January 2020): 564–68. http://dx.doi.org/10.1016/j.jss.2019.06.086.

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Rentea, Rebecca M., Janelle R. Noel-MacDonnell, Brian T. Bucher, et al. "Impact of Botulinum Toxin on Hirschsprung-associated Enterocolitis After Primary Pull-Through." Journal of Surgical Research 261 (May 2021): 95–104. http://dx.doi.org/10.1016/j.jss.2020.12.018.

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Lewit, Ruth, Laura V. Veras, Robert A. Cowles, et al. "Existing Scoring Systems for Hirschsprung-Associated Enterocolitis Result in Underdiagnosis and Overtreatment." Journal of the American College of Surgeons 231, no. 4 (2020): S196. http://dx.doi.org/10.1016/j.jamcollsurg.2020.07.559.

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Frykman, Philip K., Sungjin Kim, Tomas Wester, et al. "Critical evaluation of the Hirschsprung-associated enterocolitis (HAEC) score: A multicenter study of 116 children with Hirschsprung disease." Journal of Pediatric Surgery 53, no. 4 (2018): 708–17. http://dx.doi.org/10.1016/j.jpedsurg.2017.07.009.

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Patandianan, Yelisa Tanete, Farid Nurmantu, Nita Mariana, et al. "Relationship of nerve diameter using S-100 immunohistochemistry with Hirschsprung-associated enterocolitis degrees." Medicina Clínica Práctica 4 (April 2021): 100227. http://dx.doi.org/10.1016/j.mcpsp.2021.100227.

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Bachetti, Tiziana, Francesca Rosamilia, Martina Bartolucci, et al. "The OSMR Gene Is Involved in Hirschsprung Associated Enterocolitis Susceptibility through an Altered Downstream Signaling." International Journal of Molecular Sciences 22, no. 8 (2021): 3831. http://dx.doi.org/10.3390/ijms22083831.

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Hirschsprung (HSCR) Associated Enterocolitis (HAEC) is a common life-threatening complication in HSCR. HAEC is suggested to be due to a loss of gut homeostasis caused by impairment of immune system, barrier defense, and microbiome, likely related to genetic causes. No gene has been claimed to contribute to HAEC occurrence, yet. Genetic investigation of HAEC by Whole-Exome Sequencing (WES) on 24 HSCR patients affected (HAEC) or not affected (HSCR-only) by enterocolitis and replication of results on a larger panel of patients allowed the identification of the HAEC susceptibility variant p.H187Q

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Bokova, Elizaveta, Ninad Prasade, Sanjana Janumpally, et al. "State of the Art Bowel Management for Pediatric Colorectal Problems: Hirschsprung Disease." Children 10, no. 8 (2023): 1418. http://dx.doi.org/10.3390/children10081418.

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After an initial pull-though, patients with Hirschsprung disease (HD) can present with obstructive symptoms, Hirschsprung-associated enterocolitis (HAEC), failure to thrive, or fecal soiling. This current review focuses on algorithms for evaluation and treatment in children with HD as a part of a manuscript series on updates in bowel management. In constipated patients, anatomic causes of obstruction should be excluded. Once anatomy is confirmed to be normal, laxatives, fiber, osmotic laxatives, or mechanical management can be utilized. Botulinum toxin injections are performed in all patients

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Cheng, Siyang, Jun Wang, Weihua Pan, et al. "Pathologically assessed grade of Hirschsprung-associated enterocolitis in resected colon in children with Hirschsprung's disease predicts postoperative bowel function." Journal of Pediatric Surgery 52, no. 11 (2017): 1776–81. http://dx.doi.org/10.1016/j.jpedsurg.2017.03.056.

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Sytkov, Valentin V., T. E. Borovik, I. E. Smirnov, and I. V. Poddubnyy. "CHANGES IN MICROBIOTA AND PECULIARITIES OF THE USE OF PROBIOTICS FOR THE PREVENTION OF COMPLICATIONS OF THE SURGICAL TREATMENT OF HIRSCHSPRUNG DISEASE IN CHILDREN." Russian Pediatric Journal 20, no. 3 (2019): 172–79. http://dx.doi.org/10.18821/1560-9561-2017-20-3-172-179.

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In a review there are presented current data both on the formation of Hirschsprung disease (HD) in children and complications of its surgical treatment. The importance of interrelations of intestinal microbiota, agangliosis of the large intestine and the mechanisms of formation of HD-associated enterocolitis as a leading complication in the postoperative period are described. The value of the intestinal microbiota in its interaction with the immune system as a factor of pathogenesis and prevention of postoperative complications in HD patients is determined. Changes in the intestinal microbiota

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Frykman, Philip K., Zhi Cheng, Xiao Wang, and Deepti Dhall. "Enterocolitis causes profound lymphoid depletion in endothelin receptor B- and endothelin 3-null mouse models of Hirschsprung-associated enterocolitis." European Journal of Immunology 45, no. 3 (2015): 807–17. http://dx.doi.org/10.1002/eji.201444737.

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Cheng, Zhi, Xiao Wang, Deepti Dhall, et al. "Splenic lymphopenia in the endothelin receptor B-null mouse: implications for Hirschsprung associated enterocolitis." Pediatric Surgery International 27, no. 2 (2010): 145–50. http://dx.doi.org/10.1007/s00383-010-2787-y.

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