Prognosis of Good syndrome : mortality and morbidity of thymoma associated immunodeficiency in perspective

Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

Bibliographische Detailangaben
Veröffentlicht in:Clinical immunology (Orlando, Fla.). - 1999. - 171(2016) vom: 01. Okt., Seite 12-17
1. Verfasser: Jansen, Anne (VerfasserIn)
Weitere Verfasser: van Deuren, Marcel, Miller, Joanne, Litzman, Jiri, de Gracia, Javier, Sáenz-Cuesta, Matías, Szaflarska, Anna, Martelius, Timi, Takiguchi, Yuichi, Patel, Smita, Misbah, Siraj, Simon, Anna, Good syndrome Study Group
Format: Online-Aufsatz
Sprache:English
Veröffentlicht: 2016
Zugriff auf das übergeordnete Werk:Clinical immunology (Orlando, Fla.)
Schlagworte:Journal Article Good syndrome Immunodeficiency Infection Thymoma
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520 |a 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) 
650 4 |a Journal Article 
650 4 |a Good syndrome 
650 4 |a Immunodeficiency 
650 4 |a Infection 
650 4 |a Thymoma 
700 1 |a van Deuren, Marcel  |e verfasserin  |4 aut 
700 1 |a Miller, Joanne  |e verfasserin  |4 aut 
700 1 |a Litzman, Jiri  |e verfasserin  |4 aut 
700 1 |a de Gracia, Javier  |e verfasserin  |4 aut 
700 1 |a Sáenz-Cuesta, Matías  |e verfasserin  |4 aut 
700 1 |a Szaflarska, Anna  |e verfasserin  |4 aut 
700 1 |a Martelius, Timi  |e verfasserin  |4 aut 
700 1 |a Takiguchi, Yuichi  |e verfasserin  |4 aut 
700 1 |a Patel, Smita  |e verfasserin  |4 aut 
700 1 |a Misbah, Siraj  |e verfasserin  |4 aut 
700 1 |a Simon, Anna  |e verfasserin  |4 aut 
700 0 |a Good syndrome Study Group  |e verfasserin  |4 aut 
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