Hirschsprung

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Hirschsprung's Disease

Hirschsprung's disease (pronounced: hirsh-sproongz) is a congenital disorder that affects the colon or large intestine, causing problems with passing stool. The condition is present at birth and is often diagnosed in infancy, although in some cases it may not be detected until later in childhood.

Etymology

The disease is named after the Danish physician Harald Hirschsprung, who first described the condition in the late 19th century.

Symptoms

The most common symptom of Hirschsprung's disease is a newborn's failure to have a bowel movement within 48 hours after birth. Other symptoms can include a swollen belly, vomiting, constipation, and in older children, failure to thrive.

Causes

Hirschsprung's disease is caused by a lack of nerve cells in parts of the colon. These nerve cells control the muscles that move food and waste, or stool, through the colon. This lack of nerve cells is a result of a mutation in certain genes. The condition is often associated with other genetic conditions, such as Down syndrome.

Diagnosis

Diagnosis of Hirschsprung's disease involves a physical examination, a review of the patient's medical history, and certain tests. These tests may include a barium enema, anorectal manometry, and a rectal biopsy.

Treatment

Treatment for Hirschsprung's disease usually involves surgery to bypass or remove the diseased part of the colon. The two main types of surgery are the pull-through procedure and the ostomy surgery.

Prognosis

With early diagnosis and treatment, most children with Hirschsprung's disease lead a healthy life. However, complications can occur, including enterocolitis and slow transit constipation.

See also

External links

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