The science of Hirschsprung disease: what we know and where we are headed

JL Mueller, AM Goldstein - Seminars in pediatric surgery, 2022 - Elsevier
The enteric nervous system (ENS) is a rich network of neurons and glial cells that comprise
the gastrointestinal tract's intrinsic nervous system and are responsible for controlling …

[HTML][HTML] Development of the intrinsic and extrinsic innervation of the gut

T Uesaka, HM Young, V Pachnis, H Enomoto - Developmental biology, 2016 - Elsevier
The gastrointestinal (GI) tract is innervated by intrinsic enteric neurons and by extrinsic
efferent and afferent nerves. The enteric (intrinsic) nervous system (ENS) in most regions of …

[HTML][HTML] Glial cell-derived neurotrophic factor induces enteric neurogenesis and improves colon structure and function in mouse models of Hirschsprung disease

R Soret, S Schneider, G Bernas, B Christophers… - Gastroenterology, 2020 - Elsevier
Background & Aims Hirschsprung disease (HSCR) is a life-threatening birth defect in which
the distal colon is devoid of enteric neural ganglia. HSCR is treated by surgical removal of …

[HTML][HTML] White paper on guidelines concerning enteric nervous system stem cell therapy for enteric neuropathies

AJ Burns, AM Goldstein, DF Newgreen, L Stamp… - Developmental …, 2016 - Elsevier
Over the last 20 years, there has been increasing focus on the development of novel stem
cell based therapies for the treatment of disorders and diseases affecting the enteric nervous …

Schwann cells in the aganglionic colon of Hirschsprung disease can generate neurons for regenerative therapy

W Pan, AA Rahman, R Stavely, S Bhave… - Stem cells …, 2022 - academic.oup.com
Cell therapy offers the potential to replace the missing enteric nervous system (ENS) in
patients with Hirschsprung disease (HSCR) and to restore gut function. The Schwann cell …

Neural crest cell implantation restores enteric nervous system function and alters the gastrointestinal transcriptome in human tissue-engineered small intestine

CR Schlieve, KL Fowler, M Thornton, S Huang, I Hajjali… - Stem Cell Reports, 2017 - cell.com
Acquired or congenital disruption in enteric nervous system (ENS) development or function
can lead to significant mechanical dysmotility. ENS restoration through cellular …

Regenerative medicine: postnatal approaches

PKH Tam, KKY Wong, A Atala, GG Giobbe… - The Lancet Child & …, 2022 - thelancet.com
Paper 2 of the paediatric regenerative medicine Series focuses on recent advances in
postnatal approaches. New gene, cell, and niche-based technologies and their …

Treatment and prevention of neurocristopathies

N Pilon - Trends in molecular medicine, 2021 - cell.com
Neurocristopathies form a heterogeneous group of rare diseases caused by abnormal
development of neural crest cells. Heterogeneity of neurocristopathies directly relates to the …

Opportunities for novel diagnostic and cell-based therapies for Hirschsprung disease

W Pan, AM Goldstein, R Hotta - Journal of pediatric surgery, 2022 - Elsevier
Despite significant progress in our understanding of the etiology and pathophysiology of
Hirschsprung disease (HSCR), early and accurate diagnosis and operative management …

Postnatal human enteric neuronal progenitors can migrate, differentiate, and proliferate in embryonic and postnatal aganglionic gut environments

LS Cheng, R Hotta, HK Graham, J Belkind-Gerson… - Pediatric …, 2017 - nature.com
Background: Enteric neural stem/progenitor cells (ENSCs) offer an innovative approach to
treating Hirschsprung disease (HSCR) and other enteric neuropathies. However, postnatal …