{"entities":{"Q46717":{"pageid":37088,"ns":120,"title":"Item:Q46717","lastrevid":183663,"modified":"2026-08-13T15:30:22Z","type":"item","id":"Q46717","labels":{"mul":{"language":"mul","value":"LD28.01"},"pt-br":{"language":"pt-br","value":"S\u00edndrome de Marfan"},"en":{"language":"en","value":"Marfan syndrome"}},"descriptions":{"pt-br":{"language":"pt-br","value":"S\u00edndrome de Marfan \u00e9 uma doen\u00e7a sist\u00eamica do tecido conjuntivo caracterizada por uma combina\u00e7\u00e3o vari\u00e1vel de manifesta\u00e7\u00f5es cardiovasculares, musculoesquel\u00e9ticas, oft\u00e1lmicas e pulmonares. O envolvimento cardiovascular \u00e9 caracterizado por 1) dilata\u00e7\u00e3o progressiva da aorta acompanhada por um risco aumentado de dissec\u00e7\u00e3o a\u00f3rtica, que afeta o progn\u00f3stico e 2) insufici\u00eancia mitral. O envolvimento esquel\u00e9tico frequentemente \u00e9 o primeiro sinal da doen\u00e7a e pode incluir dolicostenomelia, tamanho grande, aracnodactilia, hipermobilidade articular, deforma\u00e7\u00f5es escoli\u00f3ticas, protrus\u00e3o do acet\u00e1bulo, deformidade tor\u00e1cica, dolicocefalia do eixo \u00e2ntero-posterior, micrognatismo ou hipoplasia malar. O envolvimento oft\u00e1lmico resulta em miopia axial, que pode levar a descolamento da retina e a deslocamento do cristalino."},"en":{"language":"en","value":"Marfan syndrome is a systemic disease of connective tissue characterised by a variable combination of cardiovascular, musculo-skeletal, ophthalmic and pulmonary manifestations. Cardiovascular involvement is characterised by 1) progressive dilation of the aorta accompanied by an increased risk of aortic dissection, which affects prognosis and 2) mitral insufficiency. Skeletal involvement is often the first sign of the disease and can include dolichostenomelia, large size, arachnodactyly, joint hypermobility, scoliotic deformations, acetabulum protrusion, thoracic deformity, dolichocephaly of the anteroposterior axis, micrognathism or malar hypoplasia. Ophthalmic involvement results in axile myopia, which can lead to retinal detachment and lens displacement."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"04d15996b91761821dc4a40b147ca0dd6848cd07","datavalue":{"value":"https://id.who.int/icd/entity/236564145","type":"string"},"datatype":"url"},"type":"statement","id":"Q46717$259041F9-BFCD-40FD-BB16-B6AFC9B615C7","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"a3591c21de7d6f2e6617bf382ca1611da54c6879","datavalue":{"value":"CID11:LD28.01","type":"string"},"datatype":"string"},"type":"statement","id":"Q46717$405D3C97-1FBF-43B0-AE7F-64B632A4A229","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"ff5ff166c7e3a0bd570a1692a29b726be4645493","datavalue":{"value":"dki-india-LD28.01","type":"string"},"datatype":"string"},"type":"statement","id":"Q46717$19A98353-8BEB-4D24-9398-44C5A239F294","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q46717$45216951-0009-4ABC-9ECF-19798D42D45A","rank":"normal"}],"P78":[{"mainsnak":{"snaktype":"value","property":"P78","hash":"5f9b5be68b7400da549761cf15abfdbc4d1e075b","datavalue":{"value":"https://pauloleads.com.br/cases-publicos/","type":"string"},"datatype":"url"},"type":"statement","id":"Q46717$B78C0FDF-BCFE-411A-8971-4CEB8B54B0C1","rank":"normal"}],"P80":[{"mainsnak":{"snaktype":"value","property":"P80","hash":"92b8222fcbb75718504f915073a3f43bd4003d18","datavalue":{"value":{"time":"+2026-08-13T00:00:00Z","timezone":0,"before":0,"after":0,"precision":11,"calendarmodel":"http://www.wikidata.org/entity/Q1985727"},"type":"time"},"datatype":"time"},"type":"statement","id":"Q46717$49A03358-0A5F-4D4F-B802-025EFFE2E267","rank":"normal"}],"P89":[{"mainsnak":{"snaktype":"value","property":"P89","hash":"da6de66391f74c9c5c562fc1767baaf46e3c1f9f","datavalue":{"value":{"entity-type":"item","numeric-id":874,"id":"Q874"},"type":"wikibase-entityid"},"datatype":"wikibase-item"},"type":"statement","id":"Q46717$14BC0887-5224-4C1C-82FB-74D2A9728E8E","rank":"normal"}]},"sitelinks":{}}}}