{"entities":{"Q91609":{"pageid":82002,"ns":120,"title":"Item:Q91609","lastrevid":509632,"modified":"2026-08-15T18:38:27Z","type":"item","id":"Q91609","labels":{"mul":{"language":"mul","value":"ID_846453488"},"pt-br":{"language":"pt-br","value":"S\u00edndrome de Criger-Najjar tipo 2"},"en":{"language":"en","value":"Crigler-Najjar syndrome type 2"}},"descriptions":{"pt-br":{"language":"pt-br","value":"A s\u00edndrome de Crigler-Najjar (SCN) tipo 2 \u00e9 um dist\u00farbio heredit\u00e1rio do metabolismo da bilirrubina caracterizado por hiperbilirrubinemia n\u00e3o conjugada devido \u00e0 atividade reduzida e induz\u00edvel da bilirrubina hep\u00e1tica glucuronosiltransferase. Trata-se de uma forma mais branda que a s\u00edndrome tipo 1. As primeiras manifesta\u00e7\u00f5es cl\u00ednicas geralmente aparecem logo ap\u00f3s o nascimento. Pacientes com SCN tipo 2 s\u00e3o tem menor grau de ict\u00e9ricos em rela\u00e7\u00e3o aos do SCN tipo1, a bile pigmentada cont\u00e9m glicuron\u00eddeos de bilirrubina e geralmente n\u00e3o apresentam comprometimento neurol\u00f3gico ou intelectual. A encefalopatia bilirrub\u00ednica pode se desenvolver mais tarde na vida, quando os pacientes apresentam uma infec\u00e7\u00e3o superposta ou estresse."},"en":{"language":"en","value":"Type 2 Crigler-Najjar syndrome is a hereditary disorder of bilirubin metabolism characterised by unconjugated hyperbilirubinemia due to reduced and inducible activity of hepatic bilirubin glucuronosyltransferase (GT). CNS2 is a milder form of CNS than CNS1 (see these terms). First clinical manifestations usually appear soon after birth. CNS2 patients are less severely jaundiced than CNS1 patients, have pigmented bile that contains bilirubin glucuronides, and generally do not present neurologic or intellectual impairment. Bilirubin encephalopathy may develop in later life when patients experience a superimposed infection or stress."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"80ec0c8e36a40cbc0cb16a8e3ca425529616a382","datavalue":{"value":"https://id.who.int/icd/entity/846453488","type":"string"},"datatype":"url"},"type":"statement","id":"Q91609$D85F343E-FBC7-4B48-9C3A-4CF3DD961B87","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"519e9b6cf1dd96df232620d741c9755a4cfdd31e","datavalue":{"value":"CID11:ID_846453488","type":"string"},"datatype":"string"},"type":"statement","id":"Q91609$AFF5C4DA-1CF1-4004-800D-93B567B8FA1B","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"796dec3948038cfcceaf513c6ac220013b8ae38b","datavalue":{"value":"dki-india-ID_846453488","type":"string"},"datatype":"string"},"type":"statement","id":"Q91609$34043CDB-A625-4932-8F06-72F684F8AE01","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q91609$61D1CD06-8A52-4277-96E7-29476476C336","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":"Q91609$34A806CF-3E22-4D79-AD0A-20786E6C8942","rank":"normal"}],"P80":[{"mainsnak":{"snaktype":"value","property":"P80","hash":"92b8222fcbb75718504f915073a3f43bd4003d18","datavalue":{"value":{"time":"+2026-08-13T00:00:00Z","timezone":0,"before":0,"after":0,"precision":11,"calendarmodel":"http://www.wikidata.org/entity/Q1985727"},"type":"time"},"datatype":"time"},"type":"statement","id":"Q91609$B263B395-5EDE-4767-9318-1EC778EB7E54","rank":"normal"}]},"sitelinks":{}}}}