Titlebook: Hirschsprung‘s Disease and Allied Disorders; Prem Puri Book 2019Latest edition Springer Nature Switzerland AG 2019 Hirschsprung’s Disease

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,Genetics of Hirschsprung’s Disease,s attributed to a disorder of the enteric nervous system (ENS) whereby ganglion cells fail to innervate the lower gastrointestinal tract during embryonic development. HSCR is a complex disease that mainly results from the interaction of several genes and manifests with low, sex-dependent penetrance

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,Epidemiology and Clinical Characteristics of Hirschsprung’s Disease,ls 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, of normal signalling during development of enteric nerve cells, resulting in aganglionosis of the distal bowel. It is a frequent cause of neonatal intestinal obstruction and beyond..It occurs as an isolated phenotype in 70% but is not infrequently associated with congenital abnormalities (5–32%) an

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