Familial Near-Total Intestinal Aganglionosis

Near total aganglionosis represents the most extreme and rare form of Hirschsprung's disease. It can affect more than one member of family. We report three cases of near total intestinal aganglionosis in a family presenting with intestinal obstruction at birth. All of them were operated and a j...

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Bibliographic Details
Published in:	Journal of neonatal surgery Vol. 6; no. 3; p. 62
Main Authors:	Saida, Hidouri, Hayet, Zitouni, Jamila, Chahed, Sana, Mosbahi, Samia, Belhassen, Amine, Ksiaa, Badii, Hmida, Imed, Krichene, Lassad, Sahnoun, Mongi, Mekki, Mohsen, Belguith, Abdellatif, Nouri
Format:	Journal Article
Language:	English
Published:	Pakistan EL-MED-Pub 01-07-2017 EL-Med-Pub
Subjects:	Case Series Familial Genetic Hirschsprung’s disease Total intestinal aganglionosis Genetic Total intestinal aganglionosis Familial Hirschsprung’s disease
Online Access:	Get full text
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Summary:	Near total aganglionosis represents the most extreme and rare form of Hirschsprung's disease. It can affect more than one member of family. We report three cases of near total intestinal aganglionosis in a family presenting with intestinal obstruction at birth. All of them were operated and a jejunostomy was performed. Outcome was dismal.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	2226-0439 2226-0439
DOI:	10.21699/jns.v6i3.562