The development of enteric nervous system and pathogenesis of Hirschsprung disease
Authors:
L. Zábojníková 1; V. Cingel 1; D. Murgaš 2; M. Fuňáková 1; T. Sláviková 3; I. Varga 3
Authors‘ workplace:
Klinika detskej chirurgie DFNsP a LFUK, Bratislava
prednosta doc. MUDr. J. Trnka, CSc.
1; Klinika detskej chirurgie, Univerzitná nemocnica Martin a JLF UK, Martin
prednosta doc. MUDr. M. Dragula, CSc., mim. prof.
2; Ústav histológie a embryológie LFUK, Bratislava
prednosta doc. MUDr. Š. Polák, CSc.
3
Published in:
Čes-slov Pediat 2012; 67 (6): 402-409.
Category:
Review
Overview
The most common congenital intestinal neuron dysplasia Hirschsprung´s disease is defined by a spatially patterned deficit of enteric neurons mostly confined to the distal parts of intestine. The enteric nervous system, the system of neurons and supporting cells located within the wall of the gut, is essential for normal gastrointestinal functions, e.g. regulation of motility, secretion and blood flow through the gut. Neurons and supporting cells are embryological derived from two regions of neural crest. Neural crest cells undergo extensive migration, proliferation, differentiation and survival during the development of gut to form a functional enteric nervous system.
The aim of this current review is to summarize recent advances in the understanding of the early deve-lopment of gut focused on development of enteric nervous system, and concentrates on mechanisms of development of Hirschsprung´s disease.
Key words:
enteric nervous system, Hirschsprung´s disease, neural crest cells, primitive gut, intestinal obstruction
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Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2012 Issue 6
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