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Hirschsprung's disease 

Hirschsprung's disease
Chapter:
Hirschsprung's disease
Source:
Paediatric Gastroenterology, Hepatology and Nutrition (Oxford Specialist Handbooks in Paediatrics) (1 ed.)
Author(s):

R. Mark Beattie

, Anil Dhawan

, and John W.L. Puntis

DOI:
10.1093/med/9780198569862.003.0039

Hirschprung's disease 280

Neuronal intestinal dysplasia 281

Intestinal pseudo-obstruction 281

Hirschsprung's disease is the absence of ganglion cells in the myenteric plexus of the most distal bowel. Presentation is with constipation. Incidence is 1 in 5000. Long-segment Hirschsprung's disease is familial, with equal sex incidence. The gene is on chromosome 10. It is associated with Down's syndrome and there is a high frequency of other congenital abnormalities....

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