Hirschsprung Disease

Summarized by Plex Health
Last Updated: 02 May 2022
medpix case - hirschsprung disease enterocolitis "medpix case - hirschsprung disease enterocolitis", by Original Source: Dawn Light, MD; Author: Tiffany Jane Owens (Childrens Hospital of Dayton, OH); Approved By: Dawn E Light (Childrens Hospital of Dayton, OH);. Baby with persistent gaseous abdomenal distension, ultimately found to have short-segment Hirshprungs Disease. Prone lateral images during the barium enema demostrates the transition zone approximately 4 cm above the anal verge....

Hirschsprung disease is a disease of the large intestinal tract or colon. People with this disease do not have the nerve cells in the intestinal tract needed to pass feceses from the body normally. Symptoms of Hirschsprung disease usually start in very children, however might occur later on. Without these nerves partially of the intestine, the material can not be pushed with, creating serious bowel irregularity or total blockage of the intestinal tract in people with Hirschsprung disease. According to the size of the intestinal tract section that do not have nerve cells, the disease can be split in short-segment disease and long-segment disease when the disease extends to the sigmoid colon. Due to the fact that the preliminary diagnosis of HSCR may be postponed until late childhood or adulthood, HSCR should be considered in any individual with long-lasting extreme irregular bowel movements. Enteric nerves cause the contraction that move stool through the intestinal tract. For unidentified factors, short-segment disease is 4 times more common in men than in women. Long-segment disease is found in roughly 20 percent of people with Hirschsprung disease and influences males and females similarly. Very hardly ever, nerve cells are missing from the entire large intestinal tract and sometimes part of the small intestinal tract or from all of the large and small intestinal tract. Hirschsprung disease can also occur without other problems, and these cases are described as separated or nonsyndromic. The absence of ganglion cells causes the muscular tissues in the bowels to lose their capability to move feces via the intestine. Meconium is the dark sticky substance that is normally present in the intestinal tract at birth and is passed as an infant's first defecation after birth. Infants with HSCR will very typically have abdominal swelling, abdominal pain, and throwing up. Hirschsprung-associated enterocolitis is the most regular difficulty of HSCR taking place in 30-40% of individuals with HSCR and can be mild to severe in nature. Some individuals with either untreated or serious Hirschsprung-associated enterocolitis may develop blood poisoning, which is a prevalent microbial infection of the bloodstream and is possibly life-threatening. It can not be exaggerated that a private with HSCR who develops these symptoms need to urgently look for clinical attention.

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