Hirschsprung's disease


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Noun1.Hirschsprung's disease - congenital condition in which the colon does not have the normal network of nerves; there is little urge to defecate so the feces accumulate and cause megacolon
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When this goes wrong, the bowels don't work, resulting in Hirschsprung's disease. Because neural crest cells travel throughout the body before they differentiate, they are one of the few normal cell populations that are most similar to metastatic tumour cells.
Hirschsprung's disease (HD) is a common paediatric surgical emergency encountered and it poses a great challenge to both the paediatricians as well as the paediatric surgical team.
(5) They observed that their "k values were close to the zero value expected by chance for the diagnoses normal and IND B" and concluded that "with current knowledge, rectal biopsy for diagnostic purposes should only be performed in constipated children for diagnosis of Hirschsprung's disease." Poor interobserver agreement is not surprising when one considers that only 1 among many sections from each ganglion is counted, boundaries of individual submucosal ganglia and individual ganglion cells are often ill-defined, even anuclear histochemically positive portions of ganglion cell cytoplasm are counted, and the total number of ganglia in a submucosal biopsy may be low, such that differences in interpretation of only 1 or 2 giant ganglia may determine whether the 20% threshold is met.
Hirschsprung's disease (HD) is generally believed that the intestinal nervous system develops disorders during the embryonic stage that lead to the development without any ganglia of the distal intestinal mucosa and the muscular layer.[1] A recent study has suggested that this may be associated with specific chromosomal abnormalities and syndromes.[2] In 1948, Swenson et al .
This syndrome may be accompanied by seizures, gastroesophageal reflux disease (GERD), Hirschsprung's disease, and dysrhythmia (2).
Amy Robinson - whose fouryear-old son Alfie suffers with Hirschsprung's disease, which affects the colon - said she hopes a better insight into his illness will help to treat it better.
These abnormalities may have caused volvulus, which is an infrequent complication of CIPO, even though eosinophilic plexitis, which occasionally occurs in Hirschsprung's disease, might have been a secondary phenomenon to the obstruction caused by volvulus (10, 11).
Less well-known associations include Hirschsprung's disease, tuberous sclerosis, familial adenomatous polyposis, and juvenile polyposis [4, 5].
Hirschsprung's disease (HD) is represented by a congenital segmental absence of the enteral nervous system in both the myenteric and submucosal plexus with variable proximal expression due to a failure of migration of neural crest cells during embryonic development [1].
Objective: To analyze and compare the efficacy of laparoscopic modified Soave operation and open radical resection in the treatment of Hirschsprung's disease.
Type III (Klein Waardenburg syndrome) has limb abnormalities while type IV is associated with Hirschsprung's disease (Shah Waardenburg syndrome).