Intestinal epithelial dysplasia (Q100080): Difference between revisions
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Displasia epitelial intestinal (DEI), também conhecida como enteropatia em tufos, é uma enteropatia congênita que se apresenta com diarreia intratável grave de início precoce, às vezes causando insuficiência intestinal irreversível. Acredita-se que a DEI esteja relacionada ao desenvolvimento e/ou diferenciação anormais de enterócitos, com anormalidades histológicas específicas envolvendo o epitélio com desorganização dos enterócitos de superfície com apinhamento focal, lembrando tufos. Várias características específicas associadas foram relatadas, incluindo deposição anormal de laminina e proteoglicano de sulfato de heparan (PSH) na membrana basal, expressão aumentada de desmogleína, alterações ultraestruturais nos desmossomos e distribuição anormal de moléculas de adesão de integrina alfa2beta1. | |||
| description / en | description / en | ||
Intestinal epithelial dysplasia, also known as tufting enteropathy, is a congenital enteropathy presenting with early-onset severe intractable diarrhoea sometimes causing irreversible intestinal failure. IED is thought to be related to abnormal enterocytes development and/or differentiation, with specific histological abnormalities involving the epithelium with disorganisation of surface enterocytes with focal crowding, resembling tufts. Several associated specific features were reported, including abnormal deposition of laminin and heparan sulphate proteoglycan (HSPG) in the basement membrane, increased expression of desmoglein and ultrastructural changes in the desmosomes, and abnormal distribution of alpha2beta1 integrin adhesion molecules. | |||
Revision as of 16:46, 16 August 2026
Intestinal epithelial dysplasia, also known as tufting enteropathy, is a congenital enteropathy presenting with early-onset severe intractable diarrhoea sometimes causing irreversible intestinal failure. IED is thought to be related to abnormal enterocytes development and/or differentiation, with specific histological abnormalities involving the epithelium with disorganisation of surface enterocytes with focal crowding, resembling tufts. Several associated specific features were reported, including abnormal deposition of laminin and heparan sulphate proteoglycan (HSPG) in the basement membrane, increased expression of desmoglein and ultrastructural changes in the desmosomes, and abnormal distribution of alpha2beta1 integrin adhesion molecules.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_1536004957 |
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| English | Intestinal epithelial dysplasia |
Intestinal epithelial dysplasia, also known as tufting enteropathy, is a congenital enteropathy presenting with early-onset severe intractable diarrhoea sometimes causing irreversible intestinal failure. IED is thought to be related to abnormal enterocytes development and/or differentiation, with specific histological abnormalities involving the epithelium with disorganisation of surface enterocytes with focal crowding, resembling tufts. Several associated specific features were reported, including abnormal deposition of laminin and heparan sulphate proteoglycan (HSPG) in the basement membrane, increased expression of desmoglein and ultrastructural changes in the desmosomes, and abnormal distribution of alpha2beta1 integrin adhesion molecules. |
