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International Journal of Molecular Medicine and Advance Sciences
2005, Volume 1, Issue 2 : 165-171 doi: ijmmas.2005.165.171
Research Article
Is Developmental Ganglion Cell Loss in Hirschsprung Disorders Inherently Linked to Myofiber Hypercontractility?
Published
March 30, 2005
Abstract

Systems of participation in the development of a state of essential aganglionosis of Hirschsprung’s type might primarily relate to a segmental complex of maldevelopmental processes ranging from bowel wall musculature to related vasculature to systems of asynchronized patterns of innervation and of disturbed trophic influence.The distal spastic segment of aganglionic bowel would be associated with the immediately proximal dilated bowel segment in terms particularly of failed continuity of the neural levels of innervating networks that necessarily impair even transformation of muscle wall tone and segmenting contractions to propulsive peristalsis of such bowel segments. It is in terms of such a primary segmentation of the disease process in Hirschsprung’s that one might consider different schemes of potential developmental progression resulting in aganglionosis of the bowel. Even with regard, however, to a failed innervation pathway of bowel segments in Hirschsprung’s, one would include various subsequent steps in the development of a segmental bowel spasticity that constitutes both dysfunctional and mechanistic bases for intestinal obstruction.Indeed, a central concept of selective vulnerability of intramural ganglion cells of bowel segments would account for a developmental series of multiple participants as a failed recruitment of and progressive loss of ganglion cell subpopulations in the bowel wall beyond simple contractility or spasticity effects. In terms of potential establishment of such segmental aganglionosis of the bowel wall, sources of failed viability of such ganglion cells might ultimately relate more to actual loss of actively interacting ganglion cells with bowel wall musculature and with vasculature smooth myofibers.

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