Hirschsprung's disease causes
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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 migration of myentric and submucosal nerve plexuses craniocaudally into the distal part of the bowel.
Causes
- Normally, myenteric and submucosal nerve plexuses regulate motility by allowing relaxation. In the absence of their migration, there’s over contracting which prevents passage of stool.[1]
- Absence of myenteric and submucosal plexuses causes over contraction of the smooth muscles of the rectum causing functional obstruction and chronic constipation.
- Absence of these cells in the distal colon is believed to be due to a genetic mutation causing failure of migration of these cells craniocaudally.
- Defective differentiation of neuroblasts into neural crest cells is another suggested hypothesis explaining 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.