The disease rarely presents as an acute intestinal obstruction. Our objective was to show the benefits of this antibody in diagnosis of hirschsprung s disease in biopsy specimens. Winner of the standing ovation award for best powerpoint templates from presentations magazine. This disease is characterized by a variable extent of contiguous aganglionosis extending from the anorectum proximally. Theyll give your presentations a professional, memorable appearance the kind of sophisticated look that todays audiences expect. Discussion megacolon is an abnormal dilation of the colon that can be caused by several factors. He thought that the colonic dilatation was the cause of the problem, although the actual pathology was in the distal contracted segment. The affected segment is of small caliber with proximal dilatation.
Dec 30, 2019 hirschsprung disease, a common cause of neonatal and infantile large gut obstruction, was first described in 1886 by harold hirschsprung as a cause of constipation in early infancy. Absence of enteric ganglia causes a loss of intrinsic excitatory and inhibitory innervation. Hirschsprung disease hscr, or congenital intestinal aganglionosis, is a birth defect characterized. Some infants with hirschsprungs disease hd do not pass meconiumthe dark poo normally passed in the first two days of life. Normalmente, las heces fecales son empujadas a traves del colon por. It usually presents in the newborn period as intestinal obstruction or in older children as constipation and abdominal distention. Classic hirschsprung disease invariably includes aganglionosis of the distal rectum and variable length of contiguous bowel. Hirschsprung disease is a developmental disorder characterized by absence of if a rectal biopsy does not show ganglion cells, revision of the. Plain abdominal radiograph showing a partz at the midsigmoid. Other symptoms include a distended swollen abdomen tummy and vomiting bile green fluid. It is caused by trypanosoma cruzi 1 that is transmitted by bloodsucking insects, blood transfusions or fetal transmission 1,2. In hirschsprung disease, there are no ganglion cells in the wall of the affected intestine. In typical cases the aganglionosis involves mainly the rectum or rectosigmoid colon and the lesion starts from the anal. New mutations associated with hirschsprung disease.
Plain abdominal radiograph showing a transition zone partz at the rectosigmoid. Short segment, long segment, total colonic and panintestinal forms as defined above. The age of patients ranges from 10 to 73 years old. Pdf hirschsprungs disease in adolescents and adults. Hirschsprung disease is a complex congenital condition of the intestine, which is recognized as being of genetic origin and results from a disturbance of the normal development of the enteric nervous system. Hirschsprungs disease was named after the name of dr. Proposed recommendations and guidelines for diagnosis of hirschsprung s disease in mucosal and submucosal. Total colonic aganglionosis associated with interstitial deletion of the long arm of chromosome 10. The main symptom of hirschsprungs disease is constipation, which cannot be treated using laxatives or softeners. Llegan al colon proximal a las 8 sg y al recto a las 12 sg. However, postoperative bowel functioning is not always satisfactory.
Gasera,b 4 a department of psychiatry, jena university hospital, jena, germany 5 b department of neurology, jena university hospital, jena, germany 6 7 article info. Partial least squares correlation of multivariate cognitive. Moreover, they must sign a model release that should be sent. Worlds best powerpoint templates crystalgraphics offers more powerpoint templates than anyone else in the world, with over 4 million to choose from. Hirschsprung s disease is a congenital disorder which is rare in adulthood. The immunohistochemistry from ganglia cell calretinin has been used to reduce the risk of misdiagnosis. However, postoperative hirschspruung functioning is not always satisfactory. Hirschsprung disease is a developmental disorder characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. Hirschsprungs disease has many modes of presentation varying from. Harold hirschsprung, who first gave the description of the disease. Hirschsprung disease aganglionosis, colonic colonic aganglionosis megacolon, congenital aganglionosis, rectosigmoid colon aganglionosis, total colonic congenital megacolon. Introduction hirschsprung s disease hd is a malformation of the large intestine characterized by the absence of ganglion cells in submucosal and myenteric plexus, which produces a functional obstruction and dilatation proximal to the affected segment 1. Introduction hirschsprung s disease hd is a malformation of the large intestine characterized by the absence of ganglion cells in submucosal and myenteric plexus, which produces a functional obstruction and dilatation proximal to. Hirschsprung s disease hscr is a common cause of intestinal obstruction that affects 1.
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