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Hirschsprung-associated enterocolitis (HAEC) is a life-threatening complication of HSCR, which can occur either before or after surgical resection of the aganglionic bowel. Even though HAEC is a leading cause of death in HSCR patients, its etiology and pathophysiology remain poorly understood. Various factors have been associated with HAEC, including the mucus barrier, microbiota, immune function, obstruction of the colon, and genetic variations. In this review, we examine our current mouse model of HAEC and how it informs our understanding of the disease. We also describe current emerging research that highlights the potential future of HAEC treatment.<\/jats:p>","DOI":"10.1055\/s-0042-1745780","type":"journal-article","created":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T22:53:14Z","timestamp":1654123994000},"page":"383-390","source":"Crossref","is-referenced-by-count":8,"title":["Hirschsprung-Associated Enterocolitis: Transformative Research from Bench to Bedside"],"prefix":"10.1055","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7786-6238","authenticated-orcid":false,"given":"Zhen","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of General Surgery, Capital Institute of Pediatrics, Beijing, Beijing, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8298-8344","authenticated-orcid":false,"given":"Bo","family":"Li","sequence":"additional","affiliation":[{"name":"Translational Medicine Program, Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qian","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics, Capital Institute of Pediatrics, Beijing, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qi","family":"Li","sequence":"additional","affiliation":[{"name":"Department of General Surgery, Capital Institute of Pediatrics, Beijing, Beijing, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6742-6570","authenticated-orcid":false,"given":"Agostino","family":"Pierro","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Long","family":"Li","sequence":"additional","affiliation":[{"name":"Department of General Surgery, Capital Institute of Pediatrics, Beijing, Beijing, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2022,6,1]]},"reference":[{"issue":"04","key":"ref1","doi-asserted-by":"crossref","first-page":"120","DOI":"10.5409\/wjcp.v4.i4.120","article-title":"Hirschsprung's disease: historical notes and pathological diagnosis on the occasion of the 100(th) anniversary of Dr. Harald Hirschsprung's death","volume":"4","author":"C Sergi","year":"2015","journal-title":"World J Clin Pediatr"},{"issue":"01","key":"ref2","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1001\/archinte.1948.00220250085005","article-title":"Myenteric plexus in congenital megacolon; study of 11 cases","volume":"82","author":"F R Whitehouse","year":"1948","journal-title":"Arch Intern Med (Chic)"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1111\/j.1469-1809.1963.tb01983.x","article-title":"A family study of Hirschsprung's disease","volume":"26","author":"M Bodian","year":"1963","journal-title":"Ann Hum Genet"},{"issue":"03","key":"ref4","first-page":"133","article-title":"[Megacolon in adults. 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