{"entities":{"Q94225":{"pageid":84618,"ns":120,"title":"Item:Q94225","lastrevid":528152,"modified":"2026-08-16T09:21:28Z","type":"item","id":"Q94225","labels":{"mul":{"language":"mul","value":"ID_713433700"},"pt-br":{"language":"pt-br","value":"Dermopatia restritiva letal"},"en":{"language":"en","value":"Lethal restrictive dermopathy"}},"descriptions":{"pt-br":{"language":"pt-br","value":"S\u00edndrome de contratura cut\u00e2nea retesada letal \u00e9 uma s\u00edndrome heredit\u00e1ria caracterizada pela pele ser firmemente esticada sobre o rosto, causando nariz estreito e comprimido, boca pequena, mobilidade mandibular limitada, e entr\u00f3pio. Observa-se retardo de crescimento intrauterino grave e constante, bem como m\u00faltiplas contraturas articulares, e a pele r\u00edgida e tensa \u00e9 facilmente erodida com fissuras na axila e no pesco\u00e7o. As principais manifesta\u00e7\u00f5es s\u00e3o o resultado de movimentos intra-\u00fatero restritos secund\u00e1rios \u00e0 pele defeituosa."},"en":{"language":"en","value":"Lethal tight skin contracture syndrome is an inherited syndrome characterised by the skin being drawn tightly over the face causing a narrow, pinched nose, small mouth, limited jaw mobility, and entropion. Severe and constant intrauterine growth retardation is observed as well as multiple joint contractures, and rigid and tense skin is easily eroded with fissures in the axilla and neck. The major manifestations are the result of restricted in utero movements secondary to defective skin."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"876254394a2391056fbf29a9375d528fe0d54b44","datavalue":{"value":"https://id.who.int/icd/entity/713433700","type":"string"},"datatype":"url"},"type":"statement","id":"Q94225$B9B385D0-45D2-4B32-BE2D-47700F4019B6","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"51803f2a854615e34869a23c73632ff82beecbbc","datavalue":{"value":"CID11:ID_713433700","type":"string"},"datatype":"string"},"type":"statement","id":"Q94225$EF118298-B5A9-43ED-BB64-0DACA1777285","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"dea2406e61f4ca25df03e29b6d4c85ee61533869","datavalue":{"value":"dki-india-ID_713433700","type":"string"},"datatype":"string"},"type":"statement","id":"Q94225$E4EC7252-8797-4B56-B9C2-C09F8AEF90F1","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q94225$23D7E40B-A470-4FAA-900B-B251A8FA83BD","rank":"normal"}],"P78":[{"mainsnak":{"snaktype":"value","property":"P78","hash":"5f9b5be68b7400da549761cf15abfdbc4d1e075b","datavalue":{"value":"https://pauloleads.com.br/cases-publicos/","type":"string"},"datatype":"url"},"type":"statement","id":"Q94225$FBC18FCA-9D78-44D7-824A-67F9C19CAF22","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":"Q94225$0E3FEE2D-7805-4228-A2AD-B6A122E0CDF5","rank":"normal"}]},"sitelinks":{}}}}