All Is Not Normal in “Normoganglionic” Bowel Regions of Hirschsprung Disease Model Mice
Heather M. Young
Abstract
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Heather M. Young
Abstract
Open-access reader
Hirschsprung disease is a congenital disorder in which neurons are missing from the distal bowel. As the enteric nervous system (ENS) is essential for propulsive gut motility, affected infants have intractable constipation. The current treatment for Hirschsprung disease involves surgical removal of the distal aganglionic region and anastomosis of the ganglionic bowel to the anus. Despite this, many patients with Hirschsprung disease suffer from enduring motility disturbances after removal of the aganglionic bowel, some of which do not appear to be related to the surgery.
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Hirschsprung disease is a congenital disorder in which neurons are missing from the distal bowel. As the enteric nervous system (ENS) is essential for propulsive gut motility, affected infants have intractable constipation. The current treatment for Hirschsprung disease involves surgical removal of the distal aganglionic region and anastomosis of the ganglionic bowel to the anus. Despite this, many patients with Hirschsprung disease suffer from enduring motility disturbances after removal of the aganglionic bowel, some of which do not appear to be related to the surgery.
Key concepts: Enteric nervous system, Hirschsprung's disease, Interstitial cell of Cajal, Gastroenterology, Motility, Disease, Megacolon, Internal medicine