Hirschsprung's disease causes: Difference between revisions
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==Overview== | ==Overview== | ||
Hirschsprung's disease is caused by failure of | Hirschsprung's disease is caused by failure of the myentric and [[submucosal]] [[Nerve plexus|nerve plexuses]] to migrate craniocaudally into the distal parts of the [[bowel]]. | ||
==Causes== | ==Causes== | ||
*Normally, myenteric and submucosal nerve plexuses regulate motility by allowing relaxation. | *Normally, the myenteric and [[submucosal]] [[Nerve plexus|nerve plexuses]] regulate motility by allowing relaxation of [[bowel]] muscles. When the migration of the myenteric and [[submucosal]] [[Nerve plexus|nerve plexuses]] to the distal [[bowel]] fails, there may be overcontraction, preventing passage of stool.<ref name="urlSpecial basic science review: Pathogenesis of Hirschsprungs disease - ScienceDirect">{{cite web |url=http://www.sciencedirect.com/science/article/pii/S0022346800963875 |title=Special basic science review: Pathogenesis of Hirschsprung's disease - ScienceDirect |format= |work= |accessdate=}}</ref> | ||
* | *The absence of the myenteric and [[Submucosal plexus|submucosal nerve plexuses]] causes overcontraction of rectum [[Smooth muscle|smooth muscles]], leading to functional obstruction and [[chronic]] [[constipation]]. | ||
*Absence of these cells in the distal colon is believed to be due to a genetic mutation | *Absence of these cells in the distal [[colon]] is believed to be due to a [[genetic mutation]] which may lead to a failure of these cells to migrate craniocaudally. | ||
*Defective differentiation of neuroblasts into neural crest cells is another suggested hypothesis explaining the disease. | *Defective differentiation of [[neuroblasts]] into [[neural crest cells]] is another suggested hypothesis explaining the cause of the disease.<ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Mills GC, Alperin JB, Trimmer KB, Swett C, Smith RJ, Bryant RG, Brooks P, Lehmann FG, Havemann K, Sodomann CP, Malchow H, Wiesmann UN, DiDonato S, Herschkowitz NN |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |year=1975 |pmid=10 |doi= |url=}}</ref> | ||
==References== | ==References== |
Latest revision as of 14:30, 4 August 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Ahmed Younes M.B.B.CH [2]
Overview
Hirschsprung's disease is caused by failure of the myentric and submucosal nerve plexuses to migrate craniocaudally into the distal parts of the bowel.
Causes
- Normally, the myenteric and submucosal nerve plexuses regulate motility by allowing relaxation of bowel muscles. When the migration of the myenteric and submucosal nerve plexuses to the distal bowel fails, there may be overcontraction, preventing passage of stool.[1]
- The absence of the myenteric and submucosal nerve plexuses causes overcontraction of rectum smooth muscles, leading to functional obstruction and chronic constipation.
- Absence of these cells in the distal colon is believed to be due to a genetic mutation which may lead to a failure of these cells to migrate craniocaudally.
- Defective differentiation of neuroblasts into neural crest cells is another suggested hypothesis explaining the cause of the disease.[2]
References
- ↑ "Special basic science review: Pathogenesis of Hirschsprung's disease - ScienceDirect".
- ↑ Schmoldt A, Benthe HF, Haberland G, Mills GC, Alperin JB, Trimmer KB, Swett C, Smith RJ, Bryant RG, Brooks P, Lehmann FG, Havemann K, Sodomann CP, Malchow H, Wiesmann UN, DiDonato S, Herschkowitz NN (1975). "Digitoxin metabolism by rat liver microsomes". Biochem. Pharmacol. 24 (17): 1639–41. PMID 10.