Hirschsprung disease

L Montalva, LS Cheng, R Kapur, JC Langer… - Nature Reviews …, 2023 - nature.com
Hirschsprung disease (HSCR) is a rare congenital intestinal disease that occurs in 1 in
5,000 live births. HSCR is characterized by the absence of ganglion cells in the myenteric …

ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease

K Kyrklund, CEJ Sloots, I de Blaauw… - Orphanet journal of rare …, 2020 - Springer
Background Hirschsprung's disease (HSCR) is a serious congenital bowel disorder with a
prevalence of 1/5000. Currently, there is a lack of systematically developed guidelines to …

[HTML][HTML] Neurocristopathies: New insights 150 years after the neural crest discovery

GA Vega-Lopez, S Cerrizuela, C Tribulo… - Developmental biology, 2018 - Elsevier
The neural crest (NC) is a transient, multipotent and migratory cell population that generates
an astonishingly diverse array of cell types during vertebrate development. These cells …

Diagnosis of Hirschsprung disease

L Ambartsumyan, C Smith… - Pediatric and …, 2020 - journals.sagepub.com
Diagnosis or exclusion of Hirschsprung disease (HSCR) is a frequent exercise in any
pediatric hospital. Although HSCR may present at different ages and with varied clinical …

Hirschsprung's disease—recent understanding of embryonic aspects, etiopathogenesis and future treatment avenues

M Klein, I Varga - Medicina, 2020 - mdpi.com
Hirschsprung's disease is a neurocristopathy, caused by defective migration, proliferation,
differentiation and survival of neural crest cells, leading to gut aganglionosis. It usually …

On the prevalence of constipation and fecal incontinence, and their co-occurrence, in the Netherlands

RJ Meinds, MM van Meegdenburg, M Trzpis… - International journal of …, 2017 - Springer
Purpose Numerous studies have investigated the prevalence of constipation and fecal
incontinence (FI) in the general population and, even though these disorders are known to …

Enteric nervous system modulation of luminal pH modifies the microbial environment to promote intestinal health

MK Hamilton, ES Wall, CD Robinson… - PLoS …, 2022 - journals.plos.org
The enteric nervous system (ENS) controls many aspects of intestinal homeostasis,
including parameters that shape the habitat of microbial residents. Previously we showed …

Intestinal microbiota in Hirschsprung disease

MI Neuvonen, K Korpela, K Kyrklund… - Journal of pediatric …, 2018 - journals.lww.com
Objectives: The aim of the study was to characterize the microbiota profiles of patients with
Hirschsprung disease (HD) and to evaluate this in relation to postoperative bowel function …

Management and outcomes of gastrointestinal congenital anomalies in low, middle and high income countries: protocol for a multicentre, international, prospective …

NJ Wright - BMJ open, 2019 - bmjopen.bmj.com
Introduction Congenital anomalies are the fifth leading cause of death in children< 5 years of
age globally, contributing an estimated half a million deaths per year. Very limited literature …

Outcomes at five to eight years of age for children with Hirschsprung's disease

BSR Allin, C Opondo, TJ Bradnock, SE Kenny… - Archives of disease in …, 2021 - adc.bmj.com
Objective This study describes core outcomes of Hirschsprung's disease (HD) in a UK-wide
cohort of primary school-aged children. Design A prospective cohort study conducted from 1 …