Embryology and anatomy of Hirschsprung disease.

Autor: Hei Ha JL; Department of Surgery, School of Clinical Medicine, The University of Hong Kong, Hong Kong., Hang Lui VC; Department of Surgery, School of Clinical Medicine, The University of Hong Kong, Hong Kong; Dr. Li Dak-Sum Research Centre, The University of Hong Kong, Hong Kong., Hang Tam PK; Department of Surgery, School of Clinical Medicine, The University of Hong Kong, Hong Kong; Faculty of Medicine, Macau University of Science and Technology, Macau SAR, China. Electronic address: pkhtam@must.edu.mo.
Jazyk: angličtina
Zdroj: Seminars in pediatric surgery [Semin Pediatr Surg] 2022 Dec; Vol. 31 (6), pp. 151227. Date of Electronic Publication: 2022 Nov 16.
DOI: 10.1016/j.sempedsurg.2022.151227
Abstrakt: Bowel has its own elegant nervous system - the enteric nervous system (ENS) which is a complex network of neurons and glial clones. Derived from neural crest cells (NCCs), this little brain controls muscle contraction, motility, and bowel activities in response to stimuli. Failure of developing enteric ganglia at the distal bowel results in intestinal obstruction and Hirschsprung disease (HSCR). This Review summarises the important embryological development of the ENS including proliferation, migration, and differentiation of NCCs. We address the signalling pathways which determine NCC cell fate and discuss how they are altered in the context of HSCR. Finally, we outline the anatomical defects and the mechanisms underlying gut motility in HSCR.
Competing Interests: Declaration of Competing Interest Authors declare no conflict of interest
(Copyright © 2022. Published by Elsevier Inc.)
Databáze: MEDLINE
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