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Considering Hirschsprung-associated enterocolitis in infants dehydration: Misdiagnosis risk and management of an underestimated lethal disease.
Acta Paediatr
June 2024
Neonatal and Pediatric Intensive Care Unit, Emergency Department, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Hirschsprung Disease: A Literacy Analysis of Patient Information.
Cureus
October 2023
Primary Care/Internal Medicine, Kansas City University, Kansas City, USA.
Objective: Hirschsprung disease in newborns can be a potentially life-threatening condition, with risks for complications such as Hirschsprung-associated enterocolitis. Accessing health information in a readable format for complex diseases demonstrates an important outlet for families to address concerns. While it is important to seek out information from trusted providers, many individuals seek out ways to educate themselves further by using common search engines and turning to the internet.
View Article and Find Full Text PDFTransition Zone Pull-through in Patients with Hirschsprung Disease: Is Redo Surgery Beneficial for the Long-term Outcomes?
J Pediatr Surg
October 2023
Emma Children's Hospital Amsterdam, Amsterdam UMC Location University of Amsterdam, Department of Pediatric Surgery, Meibergdreef 9, Amsterdam, the Netherlands; Amsterdam Gastroenterology Endocrinology and Metabolism Research Institute, Amsterdam, the Netherlands; Amsterdam Reproduction and Development Research Institute, Amsterdam, the Netherlands.
Introduction: Transition zone pull-through (TZPT) is incomplete removal of the aganglionic bowel/transition zone (TZ) in patients with Hirschsprung disease (HD). Evidence on which treatment generates the best long-term outcomes is lacking. The aim of this study was to compare the long-term occurrence of Hirschsprung associated enterocolitis (HAEC), requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively to patients with TZPT treated with redo surgery to non-TZPT patients.
View Article and Find Full Text PDFHirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group.
J Pediatr Surg
May 2023
Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, ON, Canada; Department of Surgery, University of Toronto, Toronto, ON, Canada. Electronic address:
Background/purpose: A small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients.
View Article and Find Full Text PDFDifferent Fecal Microbiota in Hirschsprung's Patients With and Without Associated Enterocolitis.
Front Microbiol
June 2022
Institut NuMeCan, INRAE, INSERM, Univ Rennes, Rennes-Saint-Gilles, France.
Background And Objectives: Patients with Hirschsprung's disease are at risk of developing Hirschsprung-associated enterocolitis, especially in the first 2 years of life. The pathophysiology of this inflammatory disease remains unclear, and intestinal dysbiosis has been proposed in the last decade. The primary objective of this study was to evaluate in a large cohort if Hirschsprung-associated enterocolitis was associated with alterations of fecal bacterial composition compared with HD without enterocolitis in different age groups.
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