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@article{1352651,
author = {Jansen, Anne and Deuren, Marcel van and Miller, Joanne and Litzman, Jiří and Gracia, Javier de and SáenzandCuesta, Matías and Szaflarska, Anna and Martelius, Timi and Takiguchi, Yuichi and Patel, Smita and Misbah, Siraj and Simon, Anna},
article_location = {San Diego},
article_number = {"neuvedeno"},
doi = {http://dx.doi.org/10.1016/j.clim.2016.07.025},
keywords = {Immunodeficiency; Thymoma; Infection; Good syndrome},
language = {eng},
issn = {1521-6616},
journal = {Clinical Immunology},
title = {Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective},
volume = {171},
year = {2016}
}
TY - JOUR
ID - 1352651
AU - Jansen, Anne - Deuren, Marcel van - Miller, Joanne - Litzman, Jiří - Gracia, Javier de - Sáenz-Cuesta, Matías - Szaflarska, Anna - Martelius, Timi - Takiguchi, Yuichi - Patel, Smita - Misbah, Siraj - Simon, Anna
PY - 2016
TI - Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective
JF - Clinical Immunology
VL - 171
IS - "neuvedeno"
SP - 12-17
EP - 12-17
PB - Academic Press Inc.
SN - 15216616
KW - Immunodeficiency
KW - Thymoma
KW - Infection
KW - Good syndrome
N2 - 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).
ER -
JANSEN, Anne, Marcel van DEUREN, Joanne MILLER, Jiří LITZMAN, Javier de GRACIA, Matías SÁENZ-CUESTA, Anna SZAFLARSKA, Timi MARTELIUS, Yuichi TAKIGUCHI, Smita PATEL, Siraj MISBAH a Anna SIMON. Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective. \textit{Clinical Immunology}. San Diego: Academic Press Inc., roč.~171, ''neuvedeno'', s.~12-17. ISSN~1521-6616. doi:10.1016/j.clim.2016.07.025. 2016.
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