Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective.
Clin Immunol
; 171: 12-17, 2016 Oct.
Article
em En
| MEDLINE
| ID: mdl-27497628
ABSTRACT
Good syndrome (GS) or thymoma-associated immunodeficiency, is a rare condition that has only been studied in retrospective case series. General consensus was that GS has a worse prognosis than other humoral immunodeficiencies. In this study, physicians of GS patients completed two questionnaires with a two year interval with data on 47 patients, 499 patient years in total. Results on epidemiology, disease characteristics, and outcome are presented. Mean age at diagnosis was 60years and median follow-up from onset of symptoms was 9years. There was a high frequency of respiratory tract infections due to encapsulated bacteria. Median survival was 14years. Survival was reduced compared to age-matched population controls (5-year survival 82% versus 95%, p=0.008). In this cohort survival was not associated with gender (HR 0.9, 95% CI 0.3-3.0), autoimmune diseases (HR 2.9, 95% CI 0.8-10.1) or immunosuppressive use (HR 0.3, 95% CI 0.1-1.2).
Palavras-chave
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Timoma
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Neoplasias do Timo
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Síndromes de Imunodeficiência
Tipo de estudo:
Prognostic_studies
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Risk_factors_studies
Limite:
Aged
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Aged80
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Child
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
Clin Immunol
Assunto da revista:
ALERGIA E IMUNOLOGIA
Ano de publicação:
2016
Tipo de documento:
Article