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

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http://hdl.handle.net/10138/169329

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Jansen , A , van Deuren , M , Miller , J , Litzman , J , de Gracia , J , Saenz-Cuesta , M , Szaflarska , A , Martelius , T , Takiguchi , Y , Patel , S , Misbah , S , Simon , A & Good Syndrome Study Grp 2016 , ' Prognosis of Good syndrome : mortality and morbidity of thymoma associated immunodeficiency in perspective ' , Clinical Immunology , vol. 171 , pp. 12-17 . https://doi.org/10.1016/j.clim.2016.07.025

Title: Prognosis of Good syndrome : mortality and morbidity of thymoma associated immunodeficiency in perspective
Author: Jansen, Anne; van Deuren, Marcel; Miller, Joanne; Litzman, Jiri; de Gracia, Javier; Saenz-Cuesta, Matias; Szaflarska, Anna; Martelius, Timi; Takiguchi, Yuichi; Patel, Smita; Misbah, Siraj; Simon, Anna; Good Syndrome Study Grp
Contributor: University of Helsinki, Infektiosairauksien yksikkö
Date: 2016-10
Language: eng
Number of pages: 6
Belongs to series: Clinical Immunology
ISSN: 1521-6616
URI: http://hdl.handle.net/10138/169329
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 60 years and median follow-up from onset of symptoms was 9 years. There was a high frequency of respiratory tract infections due to encapsulated bacteria. Median survival was 14 years. 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). (C) 2016 The Authors. Published by Elsevier Inc.
Subject: Immunodeficiency
Thymoma
Infection
Good syndrome
ADULT-POPULATION
REFERENCE VALUES
TACI MUTATION
LICHEN-PLANUS
HYPOGAMMAGLOBULINEMIA
INFECTIONS
ABNORMALITIES
COMPLICATIONS
PHENOTYPES
DISORDERS
3121 General medicine, internal medicine and other clinical medicine
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