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 …

Digenic inheritance in medical genetics

AA Schäffer - Journal of medical genetics, 2013‏ - jmg.bmj.com
Digenic inheritance (DI) is the simplest form of inheritance for genetically complex diseases.
By contrast with the thousands of reports that mutations in single genes cause human …

Deriving human ENS lineages for cell therapy and drug discovery in Hirschsprung disease

F Fattahi, JA Steinbeck, S Kriks, J Tchieu, B Zimmer… - Nature, 2016‏ - nature.com
The enteric nervous system (ENS) is the largest component of the autonomic nervous
system, with neuron numbers surpassing those present in the spinal cord. The ENS has …

[HTML][HTML] Analysis of early human neural crest development

E Betters, Y Liu, A Kjaeldgaard, E Sundström… - Developmental …, 2010‏ - Elsevier
The outstanding migration and differentiation capacities of neural crest cells (NCCs) have
fascinated scientists since Wilhelm His described this cell population in 1868. Today, after …

The emerging genetic landscape of Hirschsprung disease and its potential clinical applications

A Karim, CSM Tang, PKH Tam - Frontiers in Pediatrics, 2021‏ - frontiersin.org
Hirschsprung disease (HSCR) is the leading cause of neonatal functional intestinal
obstruction. It is a rare congenital disease with an incidence of one in 3,500–5,000 live …

Hirschsprung's disease

SE Kenny, PKH Tam, M Garcia-Barcelo - Seminars in pediatric surgery, 2010‏ - Elsevier
Hirschsprung's disease (HSCR) is characterized by absence of the enteric nervous system
in a variable portion of the distal gut. Affected infants usually present in the days after birth …

[HTML][HTML] Contribution of rare and common variants determine complex diseases—Hirschsprung disease as a model

MM Alves, Y Sribudiani, RWW Brouwer, J Amiel… - Developmental …, 2013‏ - Elsevier
Finding genes for complex diseases has been the goal of many genetic studies. Most of
these studies have been successful by searching for genes and mutations in rare familial …

DIDA: A curated and annotated digenic diseases database

AM Gazzo, D Daneels, E Cilia, M Bonduelle… - Nucleic acids …, 2016‏ - academic.oup.com
DIDA (DIgenic diseases DAtabase) is a novel database that provides for the first time
detailed information on genes and associated genetic variants involved in digenic diseases …

Advances in paediatric gastroenterology

PKH Tam, PHY Chung, SD St Peter, CP Gayer… - The Lancet, 2017‏ - thelancet.com
Recent developments in paediatric gastrointestinal surgery have focused on minimally
invasive surgery, the accumulation of high-quality clinical evidence, and scientific research …

Hirschsprung disease—Bowel function beyond childhood

T Wester, AL Granström - Seminars in pediatric surgery, 2017‏ - Elsevier
Hirschsprung disease is a developmental defect of the enteric nervous system characterized
by lack of enteric neurons in the distal hindgut. There are numerous reports on short-term …