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Saida H, Hayet Z, Jamila C, et al. Familial Near-Total Intestinal Aganglionosis. J Neonatal Surg. 2017;6(3):62doi: 10.21699/jns.v6i3.562.
Saida, H., Hayet, Z., Jamila, C., Sana, M., Samia, B., Amine, K., Badii, H., Imed, K., Lassad, S., Mongi, M., Mohsen, B., & Abdellatif, N. (2017). Familial Near-Total Intestinal Aganglionosis. Journal of neonatal surgery, 6(3), 62. https://doi.org/10.21699/jns.v6i3.562
Saida, Hidouri, et al. "Familial Near-Total Intestinal Aganglionosis." Journal of neonatal surgery vol. 6,3 (2017): 62. doi: https://doi.org/10.21699/jns.v6i3.562
Saida H, Hayet Z, Jamila C, Sana M, Samia B, Amine K, Badii H, Imed K, Lassad S, Mongi M, Mohsen B, Abdellatif N. Familial Near-Total Intestinal Aganglionosis. J Neonatal Surg. 2017 Aug 10;6(3):62. doi: 10.21699/jns.v6i3.562. eCollection 2017. PMID: 28920022; PMCID: PMC5593481.
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J Neonatal Surg. 2017 Aug 10;6(3):62. doi: 10.21699/jns.v6i3.562. eCollection 2017.

Familial Near-Total Intestinal Aganglionosis.

Journal of neonatal surgery

Hidouri Saida, Zitouni Hayet, Chahed Jamila, Mosbahi Sana, Belhassen Samia, Ksiaa Amine, Hmida Badii, Krichene Imed, Sahnoun Lassad, Mekki Mongi, Belguith Mohsen, Nouri Abdellatif

Affiliations

  1. Department of Pediatric Surgery, Medical School Hospital of Monastir, Tunisia.
  2. Research Laboratory LR12SP13. Faculty of Medicine of Monastir, Tunisia.
  3. Department of Radiology, Medical School Hospital of Monastir, Tunisia.

PMID: 28920022 PMCID: PMC5593481 DOI: 10.21699/jns.v6i3.562

Abstract

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.

Keywords: Familial; Genetic; Hirschsprung’s disease; Total intestinal aganglionosis

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