{"entities":{"Q41174":{"pageid":31542,"ns":120,"title":"Item:Q41174","lastrevid":135823,"modified":"2026-08-13T07:14:05Z","type":"item","id":"Q41174","labels":{"mul":{"language":"mul","value":"8A44.4"},"pt-br":{"language":"pt-br","value":"Doen\u00e7a de Krabbe"},"en":{"language":"en","value":"Krabbe disease"}},"descriptions":{"pt-br":{"language":"pt-br","value":"Doen\u00e7a de Krabbe, tamb\u00e9m conhecida como leucodistrofia de c\u00e9lulas glob\u00f3ides, \u00e9 uma esfingolipidose que resulta da defici\u00eancia de galactosilceramida (ou galactocerebrosidase), uma enzima lisoss\u00f4mica que cataboliza o principal componente lip\u00eddico da mielina. Essa doen\u00e7a resulta na desmieliniza\u00e7\u00e3o do sistema nervoso central e perif\u00e9rico, que avan\u00e7a rapidamente a partir do primeiro ano de vida, apresenta ainda outras formas- juvenis, com in\u00edcio na adolesc\u00eancia ou na fase adulta j\u00e1 foram reportadas, com uma taxa de progress\u00e3o mais vari\u00e1vel."},"en":{"language":"en","value":"Krabbe disease, also called globoid cell leukodystrophy, is a sphingolipidosis resulting from galactosylceramidase (or galactocerebrosidase) deficiency, a lysosomal enzyme that catabolizes a major lipid component of myelin. The disease leads to demyelination of the central and peripheral nervous system which is rapidly progressive from the first year of life, but juvenile, adolescent or adult onset forms have also been reported, with a more variable rate of progression."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"55e044a8cff73d549ce02a58463e5f190b974b1b","datavalue":{"value":"https://id.who.int/icd/entity/796317173","type":"string"},"datatype":"url"},"type":"statement","id":"Q41174$FE73C070-B077-49C3-8C0A-55725967C9FF","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"2862e928cb52998576096ae8390594194d092d14","datavalue":{"value":"CID11:8A44.4","type":"string"},"datatype":"string"},"type":"statement","id":"Q41174$5ECB687F-A190-4FC3-8CB1-6CC0E816DCA1","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"53a675ccd0c81ce1a71a6fd6ae8431920f632c7e","datavalue":{"value":"dki-india-8A44.4","type":"string"},"datatype":"string"},"type":"statement","id":"Q41174$8D1F2439-7B00-4D92-A568-D9549697A0A4","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q41174$EF393CDC-54C4-47C5-B816-FAE103B0F2AC","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":"Q41174$AC8FB2D6-5C8A-47E5-87F3-9A84B54FE47D","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":"Q41174$1E3C50D9-42F5-4BDD-8C49-DF0095F3C36F","rank":"normal"}],"P89":[{"mainsnak":{"snaktype":"value","property":"P89","hash":"29ddc4967e9d84cc4591204580be9530d1c4649c","datavalue":{"value":{"entity-type":"item","numeric-id":41173,"id":"Q41173"},"type":"wikibase-entityid"},"datatype":"wikibase-item"},"type":"statement","id":"Q41174$43FB90C6-BD85-4940-9A66-86C670FF9214","rank":"normal"}]},"sitelinks":{}}}}