{"entities":{"Q96350":{"pageid":86743,"ns":120,"title":"Item:Q96350","lastrevid":543436,"modified":"2026-08-16T12:19:01Z","type":"item","id":"Q96350","labels":{"mul":{"language":"mul","value":"ID_1183131753"},"pt-br":{"language":"pt-br","value":"Cardiomiopatia restritiva devida a desminopatia"},"en":{"language":"en","value":"Restrictive cardiomyopathy due to desminopathy"}},"descriptions":{"pt-br":{"language":"pt-br","value":"Cardiomiopatia restritiva devida \u00e0 desminopatia \u00e9 uma cardiomiopatia restritiva devida ao transtorno do filamento intermedi\u00e1rio associado a muta\u00e7\u00f5es nas prote\u00ednas prote\u00ednas que interagem estreitamente, desmina e alfaB-cristalina._x000D_ _x000D_ Informa\u00e7\u00f5es adicionais. O padr\u00e3o de heran\u00e7a pode ser autoss\u00f4mico dominante, autoss\u00f4mico recessivo ou espor\u00e1dico. Tipicamente, a doen\u00e7a se apresenta com fraqueza muscular de membros inferiores e superiores que se espalha lentamente para envolver os m\u00fasculos tronculares, flexores do pesco\u00e7o, faciais e bulbares, bem como cardiomiopatia e arritmias."},"en":{"language":"en","value":"Restrictive cardiomyopathy due to desminopathy is a restrictive cardiomyopathy due to the intermediate filament disorder associated with mutations in the closely interacting proteins, desmin and alphaB-crystallin. Additional information. The inheritance pattern may be autosomal dominant, autosomal recessive or sporadic. Typically, the illness presents with lower and later upper limb muscle weakness slowly spreading to involve truncal, neck-flexor, facial and bulbar muscles, as well as cardiomyopathy and arrhythmias."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"ffa9ce380b4a42993a3e602db5ccfdb1d9ef6125","datavalue":{"value":"https://id.who.int/icd/entity/1183131753","type":"string"},"datatype":"url"},"type":"statement","id":"Q96350$7A272815-FC2E-4C42-9DE3-942C31C59DDC","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"6b460e94e6431f633eb968e9bb5151aaa56847da","datavalue":{"value":"CID11:ID_1183131753","type":"string"},"datatype":"string"},"type":"statement","id":"Q96350$BA3D614B-8849-49F9-B6BC-56C78DC6F0F1","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"2a18011d508ae93f6b3933bc0c5c00c48b81fa28","datavalue":{"value":"dki-india-ID_1183131753","type":"string"},"datatype":"string"},"type":"statement","id":"Q96350$D4D32F73-C301-445D-9D6E-77F56976A266","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q96350$98927F6C-F141-4B37-99D7-FA06C6A14CBC","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":"Q96350$9751AF23-3B94-46CA-B3FC-830CA2415C32","rank":"normal"}],"P80":[{"mainsnak":{"snaktype":"value","property":"P80","hash":"378ac503ed43f21e1f461d2823a6ad59da580514","datavalue":{"value":{"time":"+2026-08-15T00:00:00Z","timezone":0,"before":0,"after":0,"precision":11,"calendarmodel":"http://www.wikidata.org/entity/Q1985727"},"type":"time"},"datatype":"time"},"type":"statement","id":"Q96350$D73CDC29-B7A4-46FA-AB0B-F17E838205CC","rank":"normal"}]},"sitelinks":{}}}}