{"entities":{"Q43639":{"pageid":34007,"ns":120,"title":"Item:Q43639","lastrevid":156606,"modified":"2026-08-13T10:44:23Z","type":"item","id":"Q43639","labels":{"mul":{"language":"mul","value":"5C58.03"},"pt-br":{"language":"pt-br","value":"Colestase intra-hep\u00e1tica familiar progressiva"},"en":{"language":"en","value":"Progressive familial intrahepatic cholestasis"}},"descriptions":{"pt-br":{"language":"pt-br","value":"A colestase intra-hep\u00e1tica familiar progressiva (PFIC) se refere a um grupo heterog\u00eaneo de transtornos autoss\u00f4micos recessivos da inf\u00e2ncia que interferem na forma\u00e7\u00e3o da bile e se apresentam com colestase de origem hepatocelular. Tr\u00eas tipos de PFIC foram identificados e est\u00e3o relacionados a muta\u00e7\u00f5es nos genes do sistema de transporte hepatocelular envolvidos na forma\u00e7\u00e3o da bile. PFIC1 e PFIC2 geralmente aparecem nos primeiros meses de vida, enquanto o in\u00edcio de PFIC3 tamb\u00e9m pode ocorrer mais tarde na primeira inf\u00e2ncia, na inf\u00e2ncia tardia ou mesmo durante a idade adulta jovem."},"en":{"language":"en","value":"Progressive familial intrahepatic cholestasis (PFIC) refers to a heterogeneous group of autosomal recessive disorders of childhood that disrupt bile formation and present with cholestasis of hepatocellular origin. Three types of PFIC have been identified and are related to mutations in hepatocellular transport system genes involved in bile formation. PFIC1 and PFIC2 usually appear in the first months of life, whereas onset of PFIC3 may also occur later in infancy, in childhood or even during young adulthood."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"63ea94cdbe1d4b90338dd145d8e928c8c2999859","datavalue":{"value":"https://id.who.int/icd/entity/1457142642","type":"string"},"datatype":"url"},"type":"statement","id":"Q43639$1B1E0F87-F816-4952-975F-62EE5429777F","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"8d092cdf4eeb6eedaa1288cc506e8afb380d8437","datavalue":{"value":"CID11:5C58.03","type":"string"},"datatype":"string"},"type":"statement","id":"Q43639$7587017E-4119-413E-849D-747E0607661A","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"6b3d8e660412c6d7fb1e77909edb1bb26acbe7ef","datavalue":{"value":"dki-india-5C58.03","type":"string"},"datatype":"string"},"type":"statement","id":"Q43639$8B5DF201-1909-451F-8C40-D9F9B9FC105A","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q43639$5D9B9EA5-6A77-4631-9BFA-9F2AF501991C","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":"Q43639$6B81A259-9D74-4D0E-B566-7A8CB4DFAB2C","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":"Q43639$14A815ED-8702-4888-8E0E-48B9FA1B49C7","rank":"normal"}],"P89":[{"mainsnak":{"snaktype":"value","property":"P89","hash":"8e02d375a47b732c6c32f345612e2540a69d5d50","datavalue":{"value":{"entity-type":"item","numeric-id":19237,"id":"Q19237"},"type":"wikibase-entityid"},"datatype":"wikibase-item"},"type":"statement","id":"Q43639$550A75BD-0A9D-426E-95C2-0494D2E60DC0","rank":"normal"}]},"sitelinks":{}}}}