Hirschsprung's disease is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Hirschsprung's disease, or a subtype of Hirschsprung's disease, affects less than 200,000 people in the US population.
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In Hirschsprung’s disease, the aganglionic bowel segment contracts without the reciprocal relaxation needed to propel feces forward. In 90% of patients, this aganglionic segment is in the rectosigmoid area, but it occasionally extends to the entire colon and parts of the small intestine.
Hirschsprung’s disease is believed to be a familial, congenital defect. It’s up to 5 times more common in males than in females. The disease typically coexists with other congenital anomalies, particularly trisomy 21 and anomalies of the urinary tract such as megaloureter.
Source: Professional Guide to Diseases (Eighth Edition), 2005
Source: The 5-Minute Pediatric Consult, 2008
The term 'prevalence' of Hirschsprung's disease usually refers to the estimated population of people who are managing Hirschsprung's disease at any given time. The term 'incidence' of Hirschsprung's disease refers to the annual diagnosis rate, or the number of new cases of Hirschsprung's disease diagnosed each year. Hence, these two statistics types can differ: a short-lived disease like flu can have high annual incidence but low prevalence, but a life-long disease like diabetes has a low annual incidence but high prevalence. For more information see about prevalence and incidence statistics.
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