Hirschsprungs disease.


eMediNexus    29 January 2018

A new article published in Nature Reviews. Gastroenterology and Hepatology discussed that Hirschsprung’s disease is defined by the absence of enteric neurons at the end of the bowel. When the ENS is missing, neurally mediated propulsive motility patterns are absent, and the bowel remains contracted, causing functional obstruction. Symptoms of Hirschsprung’s disease include constipation, vomiting, abdominal distension, and growth failure. Untreated disease usually causes death in childhood because bloodstream bacterial infections manifest as bowel inflammation (enterocolitis) or bowel perforation. This article reported that current treatment of Hirschsprung’s disease comprises surgical resection of the bowel to remove or bypass regions where the ENS is missing, however, many children have problems after surgery. Despite the anatomy of Hirschsprung disease being simple, its diagnosis and management remain challenging. Although every affected child has missing neurons in the distal bowel at birth, the age of presentation and the type of symptoms vary profoundly among patient. Moreover, this article provided insights into the disease mechanisms that might lead to new prevention, diagnostic and treatment strategies.

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