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Extremely rare cause of congenital diarrhea: Enteric anendocrinosis

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dc.creator Islek, Ali
dc.creator Sayar, Ersin
dc.creator YILMAZ, AYGEN
dc.creator Flanagan, Sarah E.
dc.creator ARTAN, REHA
dc.creator AKÇAM, Mustafa
dc.date 2013-09-30T21:00:00Z
dc.date.accessioned 2020-10-06T09:48:13Z
dc.date.available 2020-10-06T09:48:13Z
dc.identifier 3ebee3ed-b76d-4e59-a623-7e0115a9aab8
dc.identifier 10.1111/ped.12169
dc.identifier https://avesis.sdu.edu.tr/publication/details/3ebee3ed-b76d-4e59-a623-7e0115a9aab8/oai
dc.identifier.uri http://acikerisim.sdu.edu.tr/xmlui/handle/123456789/58136
dc.description Congenital diarrheal disorders consist of a variety of chronic enteropathies. There are approximately 30 different diseases that can be classified into four groups according to the mechanisms involved in pathogenesis: (i) absorption and transport of nutrients and electrolytes; (ii) enterocyte differentiation and polarization; (iii) enteroendocrine cell differentiation; and (iv) modulation of the intestinal immune response. Affected patients often present with life-threatening diarrhea, in the first few weeks of life. A new disorder, enteric anendocrinosis, which is characterized by severe malabsorptive diarrhea and a lack of intestinal enteroendocrine cells has recently been described in six patients with recessively inherited mutations in the Neurogenin-3 gene. In this report we describe a seventh case with a review of the literature.
dc.language eng
dc.rights info:eu-repo/semantics/closedAccess
dc.title Extremely rare cause of congenital diarrhea: Enteric anendocrinosis
dc.type info:eu-repo/semantics/article


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