{"entities":{"Q102551":{"pageid":92944,"ns":120,"title":"Item:Q102551","lastrevid":588364,"modified":"2026-08-16T19:37:38Z","type":"item","id":"Q102551","labels":{"mul":{"language":"mul","value":"ID_998522839"},"pt-br":{"language":"pt-br","value":"Miopatia relacionada \u00e0 desmina com inclus\u00f5es semelhantes ao corpo de Mallory"},"en":{"language":"en","value":"Desmin-related myopathy with Mallory body-like inclusions"}},"descriptions":{"pt-br":{"language":"pt-br","value":"Transtornos musculares familiares ou espor\u00e1dicos morfologicamente definidos por agregados intrassarcoplasm\u00e1ticos de desmina, o filamento intermedi\u00e1rio de c\u00e9lulas musculares, embora v\u00e1rias outras prote\u00ednas se acumulem nesses transtornos. A apresenta\u00e7\u00e3o semelhante ao corpo de Mallory caracteriza-se por hipotonia neonatal, fraqueza muscular axial e proximal, escoliose e n\u00edveis de creatinoquinase normais ou levemente elevados. \u00c9 definida pela presen\u00e7a, em aproximadamente 10% das fibras musculares, de placas hialinas desprovidas de qualquer atividade enzim\u00e1tica como NADH, SDH ou ATPase, que corresponderiam em n\u00edvel ultraestrutural a inclus\u00f5es intramiofibrilares peculiares."},"en":{"language":"en","value":"Familial or sporadic muscle disorders morphologically defined by intrasarcoplasmic aggregates of desmin, the intermediate filament of muscle cells, although several other proteins accumulate in these disorders. The Mallory body-like form presented with neonatal hypotonia, axial and proximal muscle weakness, scoliosis, and normal or mildly elevated creatine kinase levels. It is defined by the presence, in approximately 10% of muscle fibres, of hyaline plaques devoid of any enzyme activity such as NADH, SDH, or ATPase, that corresponded at the ultrastructural level to peculiar intramyofibrillar inclusions"}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"ab04fbc741ccc918fc074c2a014a940764b99419","datavalue":{"value":"https://id.who.int/icd/entity/998522839","type":"string"},"datatype":"url"},"type":"statement","id":"Q102551$F1AA580C-5E8B-4C2B-B6FB-0519D44E5E54","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"c499ba3c40ff38a3282c3e2149d2fa613c590b1b","datavalue":{"value":"CID11:ID_998522839","type":"string"},"datatype":"string"},"type":"statement","id":"Q102551$76639E8A-4F2E-483E-ACAC-EFB55C9011DB","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"d8738d13be7f404d4d6c9303e89eca2c0497e86d","datavalue":{"value":"dki-india-ID_998522839","type":"string"},"datatype":"string"},"type":"statement","id":"Q102551$A58EEE39-CA5A-468C-870F-ABCCA2606E7E","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q102551$D9AA4122-C88C-416F-8EFC-3AE966CA0460","rank":"normal"}],"P78":[{"mainsnak":{"snaktype":"value","property":"P78","hash":"5f9b5be68b7400da549761cf15abfdbc4d1e075b","datavalue":{"value":"https://pauloleads.com.br/cases-publicos/","type":"string"},"datatype":"url"},"type":"statement","id":"Q102551$E68E960C-B527-45A2-B9CD-133BB166EF7B","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":"Q102551$5D5CE613-2C22-4073-BFC5-140F2F121950","rank":"normal"}]},"sitelinks":{}}}}