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patho puzzler Hirschsprung disease: What the nurse needs to know By Linda Demarest, BSN, RN, CWOCN Hirschsprung disease is a congenital newborn are a swollen abdomen, vomiting, megacolon disorder with a reported prev- constipation, diarrhea, and/or gas. Symp- alence of 1:5,000 and a female-to-male ra- toms in infants include difficult bowel 1,2 tio of 1:4. It’s defined by the absence of movements, poor feeding, poor weight enteric neurons at the end of the bowel. gain, and progressive abdominal disten- Hirschsprung disease is caused by the sion. Hirschsprung disease causes 15% to failed migration of colonic ganglion cells 20% of intestinal obstructions that occur in during 4 to 12 weeks’ gestation, causing newborns. an absence of ganglion cells in all or part of the colon. This means that varying What’s the cause? lengths of the distal colon can’t relax, The specific causes of Hirschsprung disease causing functional colonic obstruction. are unknown. There’s strong evidence for a Hirschsprung disease most commonly in- genetic link, based on a significantly higher volves the rectosigmoid region of the co- incidence among siblings of individuals lon but can affect the entire colon and, with Hirschsprung disease. In some rarely, the small intestine. cases, it may
Nursing Made Incredibly Easy! – Wolters Kluwer Health
Published: Mar 1, 2023
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