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      Very Early Onset of Inflammatory Bowel Disease in a Patient With Long-Segment Hirschsprung's Disease

      case-report
      Author(s): , MD 1 , , MD, Med 1 , 2 ,
      Publication date (Electronic):
      Journal: ACG Case Reports Journal
      Publisher: Wolters Kluwer

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          ABSTRACT

          Hirschsprung's disease (HSCR) is a congenital defect caused by impaired development of the enteric nervous system. Inflammatory bowel disease has an increased prevalence in patients with HSCR. We describe the clinical course of a patient with long-segment HSCR who, at the age of 12 months, developed diffuse intestinal inflammation most clinically consistent with very early onset inflammatory bowel disease. We further explore previous studies that implicate the underlying neuroenteric abnormalities in HSCR as possible explanations for this patient's intestinal immune and inflammatory dysregulation.

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          The pathogenesis of Hirschsprung's disease-associated enterocolitis.

          Joan K Austin (2012)
          Hirschsprung's disease-associated enterocolitis (HAEC) remains the most life-threatening complication in Hirschsprung disease (HD) patients. The pathogenesis of HAEC has not been determined and many hypotheses regarding the etiology of HAEC have been proposed. These include a possible causal relationship between the abnormal enteric nervous system development in HD and the development of enterocolitis. Based on the complex genetic causes of HD that have been discovered and the resultant heterogeneous group of patients that exists, the causes of HAEC are likely multiple. New insights regarding the relationship of the role of the enteric nervous system and its interaction between intestinal barrier function, innate host immunity, and commensal microflora have been discovered, which may shed light on this perplexing problem. This review presents current known risk factors of HAEC and the proposed theories and supporting evidence for the potential etiologies of HAEC. Copyright © 2012. Published by Elsevier Inc.
          Article 0 comments Cited 24 times – based on 0 reviews     Review now
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            Inflammatory bowel disease in patients with Hirschsprung's disease: a systematic review and meta-analysis.

            H Nakamura, T. Lim, P Puri (2018)
            Hirschsprung-associated enterocolitis (HAEC) continues to be an important cause of morbidity in patients with Hirschsprung's disease (HSCR). HAEC can occur at any time during the course of the disease. The reported incidence of HAEC before surgery ranges from 6 to 50%, and after surgery, it ranges from 2 to 35%. HAEC and inflammatory bowel disease (IBD) have similar clinical presentation including diarrhea, hematochezia, and abdominal pain. In recent years, isolated cases of IBD have been reported in patients who had surgical treatment for HSCR. The exact pathogenesis of HAEC or IBD is not known. However, both conditions are characterized by an abnormal intestinal mucosal barrier function, which may be a common pathway. The purpose of this meta-analysis was to determine the clinical presentation and outcome in patients with HSCR who developed IBD after pull-through operation.
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              Enterocolitis in Hirschsprung's disease

              P.M.R. Carneiro, R.J. Brereton, D.P Drake … (1992)
              Article 0 comments Cited 5 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): ACG Case Rep J
                Journal ID (iso-abbrev): ACG Case Rep J
                Journal ID (coden): ACGCRJ
                Journal ID (pmc): ACGCRJ
                Journal ID (publisher-id): AC9
                Title: ACG Case Reports Journal
                Publisher: Wolters Kluwer (Maryland, MD )
                ISSN (Electronic): 2326-3253
                Publication date Collection: March 2020
                Publication date (Electronic): 20 March 2020
                Volume: 7
                Issue: 3
                Electronic Location Identifier: e00353
                Affiliations
                [1 ]Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, TX
                [2 ]Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Texas Children's Hospital, Baylor College of Medicine, Houston, TX
                Author notes
                Correspondence: Kristin Whitfield Van Buren, MD, Med ( KLW@ 123456bcm.edu ).
                Article
                Publisher ID: ACGCR-19-0654 Accession ID: 00020
                DOI: 10.14309/crj.0000000000000353
                PMC ID: 7162129
                SO-VID: 1f53cae8-0926-4347-9f2c-c47d9caae7f0
                Copyright © © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

                History
                Date received : 03 September 2019
                Date accepted : 29 January 2020
                Categories
                Subject: Case Report
                Subject: Inflammatory Bowel Disease
                Custom metadata
                OPEN-ACCESS TRUE

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