ArticleJournal of pediatric surgery2014
Intestinal dysbiosis and bacterial enteroinvasion in a murine model of Hirschsprung's disease.
Article in Journal of pediatric surgery, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 45 papers.
What it found
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Who cites it
45 citing papers in PubMed.
- Altered fecal short chain fatty acid composition in children with a history of Hirschsprung-associated enterocolitis.Journal of pediatric surgery · 2016Trial
- Longitudinal analysis of gut microbiota dysbiosis and bacterial signatures predictive of postoperative enterocolitis in children with Hirschsprung disease.Clinical and experimental pediatrics · 2026Article
- Review
- Preoperative gut microbial and metabolic signatures associated with postoperative Hirschsprung-associated enterocolitis in short-segment Hirschsprung disease.Frontiers in microbiology · 2026Article
- Mesenchymal Stem Cell Derived Exosomes Alleviates Hirschsprung-Associated Enterocolitis by Inhibiting AKT Phosphorylation in Macrophages Through miR-223.Stem cells international · 2025Article
- Molecular Mechanisms of Reelin in the Enteric Nervous System and the Microbiota-Gut-Brain Axis: Implications for Depression and Antidepressant Therapy.International journal of molecular sciences · 2024Review
- Hirschsprung-associated enterocolitis: a comprehensive review.World journal of pediatric surgery · 2024Review
- Paneth-like cells disruption and intestinal dysbiosis in the development of enterocolitis in an iatrogenic rectosigmoid hypoganglionosis rat model.Frontiers in surgery · 2024Article
- Hirschsprung disease.Nature reviews. Disease primers · 2023Review
- Comparation of robotic-assisted surgery and laparoscopic‑assisted surgery in children with Hirschsprung's disease: a single-centered retrospective study.BMC surgery · 2023Article
- Who's talking to whom: microbiome-enteric nervous system interactions in early life.American journal of physiology. Gastrointestinal and liver physiology · 2023Review
- Update on the Pathogenesis of the Hirschsprung-Associated Enterocolitis.International journal of molecular sciences · 2023Review
- Fecal microbiota transplantation enhances cell therapy in a rat model of hypoganglionosis by SCFA-induced MEK1/2 signaling pathway.The EMBO journal · 2023Article
- Effect of live combined Bifidobacterium and Lactobacillus tablets combined with Soave radical operation on postoperative intestinal flora and immune function in children with Hirschsprung disease.American journal of translational research · 2023Article
- A pilot study characterizing longitudinal changes in fecal microbiota of patients with Hirschsprung-associated enterocolitis.Pediatric surgery international · 2022Article
- Dental pulp stem cells as a therapy for congenital entero-neuropathy.Scientific reports · 2022Article
- Current understanding of Hirschsprung-associated enterocolitis: Pathogenesis, diagnosis and treatment.Seminars in pediatric surgery · 2022Review
- A Comprehensive Review on the Role of the Gut Microbiome in Human Neurological Disorders.Clinical microbiology reviews · 2022Review
- Enteric Nervous System: The Bridge Between the Gut Microbiota and Neurological Disorders.Frontiers in aging neuroscience · 2022Review
- Alterations of Gut Bacteria in Hirschsprung Disease and Hirschsprung-Associated Enterocolitis.Microorganisms · 2021Review
Corrections and comments
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Authors and funding
9 authors.
Funding
Abstract
background/purposeHirschsprung's disease (HSCR), characterized by the absence of ganglia in the distal colon, results in functional obstruction. Despite surgical resection of the aganglionic segment, around 40% of patients suffer recurrent life threatening Hirschsprung's-associated enterocolitis (HAEC). The aim of this study was to investigate whether gut microbiota and intestinal immunity changes contribute to the HAEC risk in an HSCR model.
methodsMice with neural crest conditional deletion of Endothelin receptor B (EdnrB) and their littermate controls were used (EdnrB-null and EdnrB-het). Bacterial DNA was prepared from cecal contents of P16-18 and P21-24 animals and pyrosequencing employed for microbiome analysis. Ileal tissue was isolated and secretory phospholipase A2 (sPLA2) expression and activity determined. Enteroinvasion of Escherichia coli into ileal explants was measured using an ex vivo organ culture system.
resultsEdnrB-het and EdnrB-nulls displayed similar flora, sPLA2 expression and activity at P16-18. However, by P21-24, EdnrB-hets demonstrated increased Lactobacillus and decreased Bacteroides and Clostridium, while EdnrB-nulls exhibited reciprocal changes. EdnrB-nulls also showed reduced sPLA2 expression and luminal activity at this stage. Functionally, EdnrB-nulls were more susceptible to enteroinvasion with E. coli ex vivo and released less sPLA2 than EdnrB-hets.
conclusionsInitially, EdnrB-het and EdnrB-nulls contain similar cecal flora but then undergo reciprocal changes. EdnrB-nulls display dysbiosis, demonstrate impaired mucosal defense, decreased luminal sPLA2 and increased enteroinvasion of E. coli just prior to robust colonic inflammation and death. These findings suggest a role for the intestinal microbiome in the development of HAEC.
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