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- W3147293165 abstract "Background and Aims Researchers have not clearly described the clinical and pathogenetic features of hypoganglionosis and adult-onset Hirschsprung’s disease, which cause pseudo-obstruction or intractable constipation. We conducted this study to explore these features of hypoganglionosis and adult-onset Hirschsprung’s disease in Korean patients. Methods We enrolled 24 patients pathologically confirmed as having hypoganglionosis and 11 as having adultonset Hirschsprung’s disease. We recruited 26 subjects who had undergone operation for nonobstructive colon cancer and 45 healthy volunteers as controls. We described their clinical features, investigated ganglion cells and interstitial cells of Cajal (ICC), and analyzed RET, EDNRB, EDN3, and SOX10 genes. Results We classified hypoganglionosis patients into two groups: type I (focal type, n = 13), with focally narrowed transition zone (TZ); and type II (diffuse type, n = 11), without transition zone. Hypoganglionosis patients had significantly fewer ganglion cells than the controls, and those cells were scarcer in the transition zone than in the proximal dilated area (P 0.05). The ICC numbers in both diseases were significantly lower than in controls; however, they were similar between transition zone and the proximal dilated area in hypoganglionosis. In adult-onset Hirschsprung’s disease, two significant intronic RET polymorphic variants, IVS14-24G[A and IVS19?47T[C, were significantly associated with adult-onset Hirschsprung’s disease" @default.
- W3147293165 created "2021-04-13" @default.
- W3147293165 creator A5085295303 @default.
- W3147293165 date "2011-01-01" @default.
- W3147293165 modified "2023-09-24" @default.
- W3147293165 title "Novel Classification and Pathogenetic Analysis of Hypoganglionosis and Adult-Onset Hirschsprung's Disease" @default.
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