What occurs in the gastrointestinal system of the child with Hirschsprung disease?
What causes Hirschsprung’s disease?Between the 4th and the 12th weeks of pregnancy, while the fetus is growing and developing, nerve cells form in the digestive tract, beginning in the mouth and finishing in the anus. For unknown reasons, the nerve cells do not grow past a certain point in the intestine in babies with Hirschsprung’s disease. Show
How often does Hirschsprung’s disease occur?Hirschsprung’s disease occurs in 1 out of every 5,000 live births. Who is at risk for Hirschsprung’s disease?Hirschsprung’s disease occurs five times more frequently in males than in females, and children with Down syndrome have a higher risk of having the disease. There is possibly a genetic, or inherited, cause for Hirschsprung’s disease. Why is Hirschsprung’s disease a concern?Because a segment of the intestine lacks normal nerve cells, digested food and stool cannot move forward through that portion of the digestive tract. The intestine becomes blocked with stool, and the baby will be constipated, or unable to have normal bowel movements. The obstruction (blockage) causes pressure on the inside of the intestine, causing part of the intestinal wall to wear thin. Eventually, a bacterial infection can develop in the digestive tract, causing serious problems. What are the symptoms of Hirschsprung’s disease?Most children with Hirschsprung’s disease show symptoms in the first few weeks of life. Children who only have a short segment of intestine that lacks normal nerve cells may not show symptoms for several months or years. The following are the most common symptoms of Hirschsprung’s disease. However, each person may experience symptoms differently. Symptoms may include:
Children who do not have early symptoms may also present the following:
Symptoms of Hirschsprung’s disease may resemble other conditions or medical problems. Please consult your child’s doctor for a diagnosis. How is Hirschsprung’s disease diagnosed?Our gastroenterologists perform a physical examination child, obtain a medical history and order a number of different tests in order to evaluate whether a child has Hirschsprung’s disease. These tests may include:
What is the treatment for Hirschsprung’s disease?Specific treatment for Hirschsprung’s disease will be determined by your child’s doctor based on the following:
Will my child have problems in the future?Problems in the future often depend on the amount of intestine that lacked nerve cells, and how much intestine was removed. Children who are able to have their colostomy closed may have temporary or intermittent problems, including the following:
Children who had a large section of intestine removed may have long-term problems. When a large portion of the intestine is removed, the digestive process can be affected. Nutrients and fluids are absorbed from food in the small intestine. Removing a large segment of the intestine can prevent a child from getting enough nutrients and fluids. Children can have problems with improper digestion, slow growth, and infection. Consult your child’s doctor to talk about your child’s outlook. Which of these manifestations would a child with Hirschsprung's disease is expected to have?Some infants with Hirschsprung disease have signs and symptoms of intestinal obstruction shortly after they are born. Common symptoms in older infants and children include chronic constipation and swelling of the abdomen.
What is the pathophysiology of Hirschsprung disease?Hirschsprung disease (HD) is a motor disorder of the gut, which is caused by the failure of neural crest cells (precursors of enteric ganglion cells) to migrate completely during intestinal development during fetal life. The resulting aganglionic segment of the colon fails to relax, causing a functional obstruction.
What cells are missing in Hirschsprung disease?The levels of Hirschsprung disease include: Ultrashort segment: Missing ganglion cells in the last one to two centimeters of the rectum. Short-segment: Missing ganglion cells in the rectum and sigmoid colon (the last segment of the colon). Long-segment: Also missing ganglion cells in the first third of the colon.
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