{"entities":{"Q93776":{"pageid":84169,"ns":120,"title":"Item:Q93776","lastrevid":524838,"modified":"2026-08-16T08:52:43Z","type":"item","id":"Q93776","labels":{"mul":{"language":"mul","value":"ID_286285535"},"pt-br":{"language":"pt-br","value":"Anemia hemol\u00edtica devida a defici\u00eancia da glutationa redutase"},"en":{"language":"en","value":"Haemolytic anaemia due to glutathione reductase deficiency"}},"descriptions":{"pt-br":{"language":"pt-br","value":"A anemia hemol\u00edtica devido a defici\u00eancia de glutationa redutase (GSR) \u00e9 caracterizada por aus\u00eancia quase completa da atividade da GSR nos eritr\u00f3citos. Esta doen\u00e7a foi descrita em tr\u00eas crian\u00e7as (um homem e duas mulheres) nascidas de pais consangu\u00edneos. Ambos os pais tinham n\u00edvel intermedi\u00e1rio de atividade da GRS. Uma crian\u00e7a apresentou favismo e duas com catarata. A transmiss\u00e3o \u00e9 autoss\u00f4mica recessiva. Esta doen\u00e7a deve ser diferenciada da defici\u00eancia da glutationa redutase secund\u00e1ria a defici\u00eancia diet\u00e9tica de riboflavina e do fen\u00f3tipo farmacol\u00f3gico agudo induzido pela carmustina (BCNU). A atividade da GSR n\u00e3o \u00e9 restaurada pela riboflavina in vivo ou por FAD in vitro"},"en":{"language":"en","value":"Haemolytic anaemia due to glutathione reductase (GSR) deficiency is characterised by nearly complete absence of GSR activity in erythrocytes. It has been described in three children (one male and two females) born to consanguineous parents. Both parents had intermediate levels of GSR activity. One child presented with favism and two with cataracts. Transmission is autosomal recessive. This disease should be distinguished from glutathione reductase deficiency secondary to dietary riboflavin deficiency and from the acute pharmacologic phenocopy induced by carmustine (BCNU). GSR activity is not restored by riboflavin in vivo or by FAD in vitro."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"dc12ddea99c962d07472864d01f576a2da74905e","datavalue":{"value":"https://id.who.int/icd/entity/286285535","type":"string"},"datatype":"url"},"type":"statement","id":"Q93776$B941A01B-2590-4162-B8A0-F1DBC952ABD6","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"71d408e0ae5e1bf6a37fcbeb1e777ca31071fa89","datavalue":{"value":"CID11:ID_286285535","type":"string"},"datatype":"string"},"type":"statement","id":"Q93776$CBB33635-531B-44A8-9294-12F0D93DCC16","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"4ab51f218f8803831cbced6758de9ddf3cbe7c0b","datavalue":{"value":"dki-india-ID_286285535","type":"string"},"datatype":"string"},"type":"statement","id":"Q93776$0800CC7C-7EB4-42DB-86C0-E8B05320AFF7","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q93776$E4681068-1B42-4260-A38B-5F6682F20888","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":"Q93776$C2A812F7-19A3-4BFB-99D3-2507A8A20621","rank":"normal"}],"P80":[{"mainsnak":{"snaktype":"value","property":"P80","hash":"378ac503ed43f21e1f461d2823a6ad59da580514","datavalue":{"value":{"time":"+2026-08-15T00:00:00Z","timezone":0,"before":0,"after":0,"precision":11,"calendarmodel":"http://www.wikidata.org/entity/Q1985727"},"type":"time"},"datatype":"time"},"type":"statement","id":"Q93776$D887F44A-D55D-4050-87E8-813904EBFA0B","rank":"normal"}]},"sitelinks":{}}}}