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HIRCHSPRUNG'S DISEASE ((MANAGEMENT (Colostomy: The doctor leaves part…
HIRCHSPRUNG'S DISEASE
DEFINITION
Hirschsprung's (HIRSH-sproongz) disease is a condition that affects the large intestine (colon) and causes problems with passing stool.
PATHOPHYSIOLOGY
Proper neural crest cell migration, proliferation, differentiation, survival, and apoptosis all contribute to a functional ENS. Perturbation in any of these processes can lead to a Hirschsprung disease phenotype.
CAUSES
It's not clear what causes Hirschsprung's disease. It sometimes occurs in families and might, in some cases, be associated with a genetic mutation.
CLINICAL MANIFESTATIONS
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Vomiting, including vomiting a green or brown substance
Constipation or gas, which might make a newborn fussy
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MANAGEMENT
Colostomy: The doctor leaves part of the colon intact and connects it to the stoma. Stool leaves the body through the end of the large intestine.
Ileostomy. The doctor removes the entire colon and connects the small intestine to the stoma. Stool leaves the body through the stoma into a bag.
Pre-Operative Care
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If bottle fed, use compressible bottle, longer nipple, larger hole in nipple, any other special device for feeding the infant.
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Post-Operative Care
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Monitor weight, growth and developmental achievements.
DIET THERAPY
A high-fibre, low-sugar diet and regular eating throughout the day can help your child pass stool more easily.
Reducing lactose in your child's diet can ease bloating, gas and diarrhea.
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Keep a record of your child's symptoms as you try different foods so that you can learn what works best for your child.