{"entities":{"Q97588":{"pageid":87981,"ns":120,"title":"Item:Q97588","lastrevid":552419,"modified":"2026-08-16T13:42:21Z","type":"item","id":"Q97588","labels":{"mul":{"language":"mul","value":"ID_1845332026"},"pt-br":{"language":"pt-br","value":"Imunodefici\u00eancia combinada grave T- B+ devida \u00e0 defici\u00eancia de JAK3"},"en":{"language":"en","value":"Severe combined immunodeficiency T- B+ due to JAK3 deficiency"}},"descriptions":{"pt-br":{"language":"pt-br","value":"Imunodefici\u00eancia combinada grave T- B+ devida \u00e0 defici\u00eancia de JAK3 \u00e9 uma forma autoss\u00f4mica recessiva de imunodefici\u00eancia combinada grave (IDCG). \u00c9 caracterizada por aus\u00eancia de c\u00e9lulas T e NK (Natural Killer) circulantes e n\u00edveis normais de linf\u00f3citos B. Pacientes com defici\u00eancia de JAK3 manifestam o quadro cl\u00e1ssico da IDCG nos poucos meses iniciais de vida, ou seja, diarreia cr\u00f4nica, falha no crescimento, infec\u00e7\u00e3o respirat\u00f3ria rescorrente e/ou infec\u00e7\u00e3o generalizada por pat\u00f3genos oportunistas, ou sinais de rea\u00e7\u00e3o enxerto-versus-hospedeiro (erup\u00e7\u00e3o cut\u00e2nea, altera\u00e7\u00e3o de fun\u00e7\u00e3o hep\u00e1tica, pancitopenia) por c\u00e9lulas T maternas adquiridas por via transplacent\u00e1ria."},"en":{"language":"en","value":"Severe combined immunodeficiency T- B+ due to JAK3 deficiency is an autosomal recessive form of severe combined immune deficiency (SCID). It is characterised by lack of circulating T and NK (Natural Killer) cells and normal number of B lymphocytes. JAK3-deficient patients present with the classical clinical features of SCID in the first few months of life, i.e. chronic diarrhoea, failure to thrive, recurrent respiratory infection and/or generalised infections from opportunistic pathogens, or signs of graft-versus-host reaction (skin rash, abnormalities of liver function, pancytopenia) from transplacental acquired maternal T cells."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"15164aaee08176c8cf11c744b3ade05ba4cc3f62","datavalue":{"value":"https://id.who.int/icd/entity/1845332026","type":"string"},"datatype":"url"},"type":"statement","id":"Q97588$45630250-FEEE-4AFC-B300-8B8E129EF3A0","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"d5659b7591d4354af7cbb696463f6bc8aa8b28c7","datavalue":{"value":"CID11:ID_1845332026","type":"string"},"datatype":"string"},"type":"statement","id":"Q97588$34877D44-0423-4716-92C3-FACD6A7B2442","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"28609c9566834f85890a4d9ed90f94a56a4bb1d4","datavalue":{"value":"dki-india-ID_1845332026","type":"string"},"datatype":"string"},"type":"statement","id":"Q97588$C3601D22-8787-4AD1-AFDC-C1DA424D90FC","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q97588$D279E401-08DF-4E7A-9486-CBA8A5ED8DAF","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":"Q97588$59FCC23D-0A17-4009-83FC-697898FB816F","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":"Q97588$18F31F4F-0DC9-4B7E-A0B5-1EA651F9936C","rank":"normal"}]},"sitelinks":{}}}}