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 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

  1. "Special basic science review: Pathogenesis of Hirschsprung's disease - ScienceDirect".
  2. 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.