Titlebook: Hirschsprung‘s Disease and Allied Disorders; Alexander Matthias Holschneider,Prem Puri Book 20083rd edition Springer-Verlag Berlin Heidelb

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,The Molecular Genetics of Hirschsprung’s Disease,ia in the submucosal and myenteric plexuses, along variable portions of the distal gut. Since it is caused by a premature arrest of the migration of neural crest cells along the hindgut, it is defined also as a neurocristopathy. The variable extent of aganglionosis correlates with severity of the di

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,Hirschsprung’s Disease: Clinical Features,s in the distal bowel beginning at the internal sphincter and extending proximally for varying distances. In the human fetus, neural crest-derived neuroblasts first appear in the developing esophagus at 5 weeks of gestation, and then migrate down to the anal canal in a craniocaudal direction during

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,Congenital Anomalies and Genetic Associations in Hirschsprung’s Disease,ssociated syndromes have been reported. These associations are of significance for at least two reasons. First, the majority may be attributed to abnormal genetic development signaling, yielding clues as to the genetic background of HSCR and its pathogenesis, and second, the influence of associated

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,Enterocolitis Complicating Hirschsprung’s Disease,e of blood-stained stools [1]. Enterocolitis is a significant complication of Hirschsprung’s disease (HD) both in the pre- and postoperative period [2]. Hirschsprung’s-associated enterocolitis (HAEC) can occur at any time from the neonatal period onwards into adulthood and can be independent of the

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,Diagnosis of Hirschsprung’s Disease and Allied Disorders,reening procedure, radiology still has an important role to play. The diagnosis of Hirschsprung’s disease may be suggested on plain films, which may also demonstrate the serious complication of enterocolitis. Barium enema is usually diagnostic and should show both the transition zone and the length

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,Histopathological Diagnosis and Differential Diagnosis of Hirschsprung’s Disease,anglion cells in the narrowed segment. The aganglionic segment starts at the anal ring and extends proximally for a variable length. A reliable diagnosis of HD on a hematoxylin-eosin (H&E) staining of a mucosal biopsy requires considerable experience. There is the risk of rendering a false-positive

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NADPH-Diaphorase Histochemistry, (HD) more difficult for the pathologist. Many histopathologists are reluctant to make a positive diagnosis of HD on the basis of suction rectal biopsy results, using conventional H&E stains. This reluctance stems from doubt as to the amount of submucosa that must be scanned before the absence of ga

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