Hirschsprung disease is due to
The enteric nervous system is the pa of the autonomic nervous system that directly controls the gastrointestinal tract. Derived from a multipotent, migratory cell population called the neural crest, a complete enteric nervous system is necessary for proper gut function. Disorders that arise as a consequence of defective neural crest cell development are termed neurocristopathies. One such disorder is Hirschsprung disease, also known as congenital megacolon or intestinal aganglionosis. Hirschsprung disease occurs in 1/5000 live bihs, and typically presents with the inability to pass meconium, along with abdominal distension and discomfo that usually requires surgical resection of the aganglionic bowel. This disorder is characterized by a congenital absence of neurons in a poion of the intestinal tract, usually the distal colon, due to a disruption of normal neural crest cell migration, proliferation, differentiation, survival and/or apoptosis. ref : harrisons 21st ed
Failure of migration of Neural crest cells
due to loss of crypts
due to mucosa thickening
none
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