title: Hirschsprung's associated enterocolitis.
abstract: OBJECTIVE
  Hirschsprung's disease (HSCR) is characterized by an absence of ganglion cells in the distal hindgut, extending from the rectum to a variable distance proximally, and results from a failure of cranial-caudal neural crest cell migration. Hirschsprung's-associated enterocolitis (HAEC) is a condition with classic manifestations that include abdominal distention, fever and foul-smelling stools, and is a significant and life-threatening complication of HSCR. The purpose of this review was to critically evaluate recent findings regarding the pathophysiology of HAEC.
  
  
  RESULTS
  Several recent studies have investigated the cause of HAEC in humans and mouse models. These studies suggest that alterations in the intestinal barrier, including goblet cell number and function, and Paneth cell function, impaired gastrointestinal mucosal immunity, including B-lymphocyte trafficking or function and secretory immunoglobulin A production, and dysbiosis of the intestinal microbiota may contribute to the development of HAEC.
  
  
  CONCLUSIONS
  Recent studies add to the body of literature, suggesting that the intestinal defects observed in HSCR are not restricted to the aganglionic segment but extend to the mucosal immune system within and beyond the gastrointestinal tract. Future studies further dissecting the mechanisms of HAEC and validating these findings in humans will allow for the development of directed therapeutic interventions.
authors.affiliation: aDivision of Pediatric Surgery, Department of Surgery bDepartment of Pediatrics cDepartment of Neuroscience, University of Wisconsin School of Medicine and Public Health dAmerican Family Children's Hospital, University of Wisconsin Hospital and Clinics Madison, Madison, Wisconsin, USA.
authors.forename: Ankush
authors.initials: A
authors.lastname: Gosain
authors.forename: Adam S
authors.initials: AS
authors.lastname: Brinkman
mesh_terms: D000818:Animals; D002648:Child; D004195:Disease Models, Animal; D004760:Enterocolitis / Q000209:etiology / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D006627:Hirschsprung Disease / Q000150:complications / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D007413:Intestinal Mucosa / Q000382:microbiology / Q000473:pathology / Q000503:physiopathology*; D007422:Intestines / Q000382:microbiology / Q000473:pathology / Q000503:physiopathology*; D051379:Mice; D018345:Mice, Knockout