{"entities":{"Q96603":{"pageid":86996,"ns":120,"title":"Item:Q96603","lastrevid":545289,"modified":"2026-08-16T12:37:52Z","type":"item","id":"Q96603","labels":{"mul":{"language":"mul","value":"ID_1379284603"},"pt-br":{"language":"pt-br","value":"S\u00edndrome de Epstein"},"en":{"language":"en","value":"Epstein syndrome"}},"descriptions":{"pt-br":{"language":"pt-br","value":"S\u00edndrome de Epstein \u00e9 uma forma da \"s\u00edndrome MYH9\" (um grupo que inclui quatro formas cl\u00ednicas com manifesta\u00e7\u00f5es semelhantes resultantes de muta\u00e7\u00f5es no gene MYH9: anomalia de May-Hegglin, s\u00edndrome de Sebastian, s\u00edndrome de Fechtner e s\u00edndrome de Epstein), caracterizada geralmente por macrotrombocitopenia grave mas paradoxalmente resultando em poucos ou nenhum sintoma. A macrotrombocitopenia \u00e9 definida como a presen\u00e7a de plaquetas gigantes, com di\u00e2metro igual ou superior ao de uma hem\u00e1cia. A s\u00edndrome de Epstein assemelha-se clinicamente \u00e0 s\u00edndrome de Fechtner (envolvimento renal marcado por glomerulonefrite intersticial e surdez neurossensorial associada a trombopenia), mas pode ser distinguida pela aus\u00eancia de catarata."},"en":{"language":"en","value":"Epstein syndrome is one form of the ``MYH9 syndrome'' (a group that includes four clinical forms with similar manifestations resulting from mutations in the MYH9 gene: May-Hegglin anomaly, Sebastian syndrome, Fechtner syndrome, and Epstein syndrome), characterised by usually severe macrothrombocytopenia but paradoxically resulting in few if any symptoms. Macrothrombocytopenia is defined as the presence of giant platelets, with a diameter equal to or greater than that of a red blood cell. Epstein syndrome clinically resembles Fechtner syndrome (renal involvement marked by interstitial glomerulonephritis and sensorineural deafness associated with thrombopenia), but it can be distinguished by the absence of cataract."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"ec7e6dda2f78b39f8529a8b3074c8059c142a922","datavalue":{"value":"https://id.who.int/icd/entity/1379284603","type":"string"},"datatype":"url"},"type":"statement","id":"Q96603$B35E18AB-AC6A-4242-B6FF-2BB36596DE6F","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"20fc3bf6a42a0dedceaa027d1148312482bdd862","datavalue":{"value":"CID11:ID_1379284603","type":"string"},"datatype":"string"},"type":"statement","id":"Q96603$46F4A800-1043-4F64-87EA-78DDDCFF9398","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"5ba9b890ca8d0bc85569561c8644017b72524b79","datavalue":{"value":"dki-india-ID_1379284603","type":"string"},"datatype":"string"},"type":"statement","id":"Q96603$E8CFD792-F5E9-41BB-A959-E71E8B36E7E8","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q96603$C53DCD13-57CA-4228-BDE4-5AF1F67BFC5C","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":"Q96603$73D09C29-9C77-45C6-B2A3-B42B2742D4AB","rank":"normal"}],"P80":[{"mainsnak":{"snaktype":"value","property":"P80","hash":"378ac503ed43f21e1f461d2823a6ad59da580514","datavalue":{"value":{"time":"+2026-08-15T00:00:00Z","timezone":0,"before":0,"after":0,"precision":11,"calendarmodel":"http://www.wikidata.org/entity/Q1985727"},"type":"time"},"datatype":"time"},"type":"statement","id":"Q96603$EA82535E-2B10-4CFB-8C52-398E8D1552A4","rank":"normal"}]},"sitelinks":{}}}}