{"entities":{"Q100080":{"pageid":90473,"ns":120,"title":"Item:Q100080","lastrevid":570433,"modified":"2026-08-16T16:46:18Z","type":"item","id":"Q100080","labels":{"mul":{"language":"mul","value":"ID_1536004957"},"pt-br":{"language":"pt-br","value":"Displasia epitelial intestinal"},"en":{"language":"en","value":"Intestinal epithelial dysplasia"}},"descriptions":{"pt-br":{"language":"pt-br","value":"Displasia epitelial intestinal (DEI), tamb\u00e9m conhecida como enteropatia em tufos, \u00e9 uma enteropatia cong\u00eanita que se apresenta com diarreia intrat\u00e1vel grave de in\u00edcio precoce, \u00e0s vezes causando insufici\u00eancia intestinal irrevers\u00edvel. Acredita-se que a DEI esteja relacionada ao desenvolvimento e/ou diferencia\u00e7\u00e3o anormais de enter\u00f3citos, com anormalidades histol\u00f3gicas espec\u00edficas envolvendo o epit\u00e9lio com desorganiza\u00e7\u00e3o dos enter\u00f3citos de superf\u00edcie com apinhamento focal, lembrando tufos. V\u00e1rias caracter\u00edsticas espec\u00edficas associadas foram relatadas, incluindo deposi\u00e7\u00e3o anormal de laminina e proteoglicano de sulfato de heparan (PSH) na membrana basal, express\u00e3o aumentada de desmogle\u00edna, altera\u00e7\u00f5es ultraestruturais nos desmossomos e distribui\u00e7\u00e3o anormal de mol\u00e9culas de ades\u00e3o de integrina alfa2beta1."},"en":{"language":"en","value":"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."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"797dc7b70674c31ec9faf682010a6b3d3341435c","datavalue":{"value":"https://id.who.int/icd/entity/1536004957","type":"string"},"datatype":"url"},"type":"statement","id":"Q100080$AA7E64F2-4362-4BD1-8749-016A4FE335E4","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"82841cc84a6f4dcd214d77e764829327c7b09f51","datavalue":{"value":"CID11:ID_1536004957","type":"string"},"datatype":"string"},"type":"statement","id":"Q100080$50F66A09-DD91-4718-B8B0-33744CC8869C","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"fba41f7db5b5b22bba85e0e5540c190dcac6c614","datavalue":{"value":"dki-india-ID_1536004957","type":"string"},"datatype":"string"},"type":"statement","id":"Q100080$1E5254A9-21C3-44AE-B605-82FEF66C3FE7","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q100080$1F8E85D8-212A-4B35-A657-C2543BCFEF41","rank":"normal"}],"P78":[{"mainsnak":{"snaktype":"value","property":"P78","hash":"5f9b5be68b7400da549761cf15abfdbc4d1e075b","datavalue":{"value":"https://pauloleads.com.br/cases-publicos/","type":"string"},"datatype":"url"},"type":"statement","id":"Q100080$BA7E5239-E2B2-40BA-A3DE-9E2F81514A13","rank":"normal"}],"P80":[{"mainsnak":{"snaktype":"value","property":"P80","hash":"378ac503ed43f21e1f461d2823a6ad59da580514","datavalue":{"value":{"time":"+2026-08-15T00:00:00Z","timezone":0,"before":0,"after":0,"precision":11,"calendarmodel":"http://www.wikidata.org/entity/Q1985727"},"type":"time"},"datatype":"time"},"type":"statement","id":"Q100080$D9DC43D4-113E-4915-9691-1F80C3B0CFB7","rank":"normal"}]},"sitelinks":{}}}}