Hirschsprung's disease causes: Difference between revisions
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==Overview== | ==Overview== | ||
Hirschsprung's disease is caused by failure | Hirschsprung's disease is caused by failure in craniocaudal migration of myentric and [[submucosal]] [[Nerve plexus|nerve plexuses]] into distal parts of the [[bowel]]. | ||
==Causes== | ==Causes== | ||
*Normally, myenteric and [[submucosal]] [[Nerve plexus|nerve plexuses]] regulate motility by allowing relaxation. | *Normally, myenteric and [[submucosal]] [[Nerve plexus|nerve plexuses]] regulate motility by allowing relaxation. When their migration failed, there may be over contraction which prevents stool passage.<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> | ||
*Absence of myenteric and [[Submucosal plexus|submucosal plexuses]] causes over contraction of | *Absence of myenteric and [[Submucosal plexus|submucosal nerve plexuses]] causes over contraction 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 failure in migration of these cells craniocaudally. | ||
*Defective differentiation of [[neuroblasts]] into [[neural crest cells]] is another suggested hypothesis explaining 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> | *Defective differentiation of [[neuroblasts]] into [[neural crest cells]] is another suggested hypothesis explaining the disease cause.<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== | ||
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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 in craniocaudal migration of myentric and submucosal nerve plexuses into distal parts of the bowel.
Causes
- Normally, myenteric and submucosal nerve plexuses regulate motility by allowing relaxation. When their migration failed, there may be over contraction which prevents stool passage.[1]
- Absence of myenteric and submucosal nerve plexuses causes over contraction 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 failure in migration of these cells craniocaudally.
- Defective differentiation of neuroblasts into neural crest cells is another suggested hypothesis explaining the disease cause.[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.