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Small bowel (small intestine)
Other / benign tumors and tumor-like conditions
Tufting enteropathy
Reviewer: Hanni Gulwani, M.D. (see Reviewers
page)
Revised: 22 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.
General
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● Intractable diarrhea syndrome, sometimes familial, beginning in neonates
● Also called intestinal epithelial dysplasia (Orphanet J Rare Dis 2007;2:20)
● Due to Epithelial cell adhesion molecule gene (EpCAM) (Gastroenterology 2008;135:429, Gut Liver 2010;4:407)
Treatment
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● Total parental nutrition
Micro description
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● Variable villus abnormality, epithelial crowding, disorganization and focal tufting
● No epithelial lymphocytosis
Micro images
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Electron microscopy description
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● Basement membrane abnormalities; increase in desmosome length and number; decreased number of microvilli of surface enterocytes with structural disorganization
Electron microscopy images
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End of Small bowel (small intestine) > Benign tumors and tumor-like conditions > Tufting enteropathy
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