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

      , MD 1 , , MD, Med , 1 , 2

      ACG Case Reports Journal

      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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          Most cited references 5

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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.
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            Enterocolitis in Hirschsprung's disease

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              Inflammatory bowel disease manifesting after surgical treatment for Hirschsprung disease.

              Eight children developed chronic inflammatory bowel disease (IBD) 4 to 21 years after surgery for Hirschsprung disease. Three had trisomy 21 and 6 experienced chronic or recurrent enterocolitis. Four had a family history of IBD. Clinical presentation included chronic diarrhea, hematochezia, abscess, and fistula formation. Three required surgery for fistula, stricture, and small bowel obstruction and the other 5 were managed medically. Recognition of this condition may be important in the long-term follow-up of children with Hirschsprung disease, and patients who have carried a diagnosis of chronic enterocolitis may warrant further investigation looking for evidence of IBD.
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                Author and article information

                Journal
                ACG Case Rep J
                ACG Case Rep J
                ACGCRJ
                ACGCRJ
                AC9
                ACG Case Reports Journal
                Wolters Kluwer (Maryland, MD )
                2326-3253
                March 2020
                20 March 2020
                : 7
                : 3
                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
                ACGCR-19-0654 00020
                10.14309/crj.0000000000000353
                7162129
                © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.

                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.

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                Categories
                Case Report
                Inflammatory Bowel Disease
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