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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Detalles Bibliográficos
Autores principales: Saida, Hidouri, Hayet, Zitouni, Jamila, Chahed, Sana, Mosbahi, Samia, Belhassen, Amine, Ksiaa, Badii, Hmida, Imed, Krichene, Lassad, Sahnoun, Mongi, Mekki, Mohsen, Belguith, Abdellatif, Nouri
Formato: Online Artículo Texto
Lenguaje:English
Publicado: EL-MED-Pub 2017
Materias:
Case Series
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5593481/
https://www.ncbi.nlm.nih.gov/pubmed/28920022
http://dx.doi.org/10.21699/jns.v6i3.562
Descripción
Sumario: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.

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