KMID : 0816120030060020202
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Korean Journal of Pediatric Gastroenterolology and Nutrition 2003 Volume.6 No. 2 p.202 ~ p.207
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Intestinal Neuronal Dysplasia in Twins
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Lim In-Suk
Chung Ju-Young Chey Myoung-Jae Kim Sang-Woo Kim Hong-Ju Kim Jung-Yeon
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Abstract
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Intestinal neuronal dysplasia (IND) is a disorder of abnormal intestinal innervation resulting in dysfunctional colonic motility. IND shares clinical features with Hirschsprung¢¥s disease but differenfiated by histological findings such as hyperplasia of sutnnucosal and myenteric plexuses, giant (Tanglia, ectopic ganglion cell and increased acetylcholinesterase activity in lamina propria. Although IND may exist as an isolated condition, more commonly, it occurs in association with Hirschsprung¢¥s disease. We report a case of twins affected with IND. Both children manifested with delayed passage of meconium and severe abdominal distention after birth. Barium enema in both patients showed microcolon. They underwent emergency ileostomy under the impression of total a-anglionosis. But surgical biopsy specimens showed hyperganglionosis in submucosa with formation of oiant gan-lia. Both neonates suffers from several episodes of peudo-obstruction after the repair operation of colostomy.
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KEYWORD
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Intestinal neuronal dysplasia, Twins
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