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

J 2016

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

JANSEN, Anne, Marcel van DEUREN, Joanne MILLER, Jiří LITZMAN, Javier de GRACIA et. al.

Základní údaje

Originální název

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

Autoři

JANSEN, Anne (528 Nizozemské království), Marcel van DEUREN (528 Nizozemské království), Joanne MILLER (826 Velká Británie a Severní Irsko), Jiří LITZMAN (203 Česká republika, garant, domácí), Javier de GRACIA (724 Španělsko), Matías SÁENZ-CUESTA (724 Španělsko), Anna SZAFLARSKA (616 Polsko), Timi MARTELIUS (246 Finsko), Yuichi TAKIGUCHI (392 Japonsko), Smita PATEL (826 Velká Británie a Severní Irsko), Siraj MISBAH (826 Velká Británie a Severní Irsko) a Anna SIMON (528 Nizozemské království)

Vydání

Clinical Immunology, San Diego, Academic Press Inc. 2016, 1521-6616

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30300 3.3 Health sciences

Stát vydavatele

Spojené státy

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 3.990

Kód RIV

RIV/00216224:14110/16:00090643

Organizační jednotka

Lékařská fakulta

DOI

http://dx.doi.org/10.1016/j.clim.2016.07.025

UT WoS

000386195100002

Klíčová slova anglicky

Immunodeficiency; Thymoma; Infection; Good syndrome

Štítky

EL OK

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 24. 11. 2016 12:59, Ing. Mgr. Věra Pospíšilíková

Anotace

V originále

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).

Citovat

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. Clinical Immunology. San Diego: Academic Press Inc., 2016, roč. 171, "neuvedeno", s. 12-17. ISSN 1521-6616. Dostupné z: https://dx.doi.org/10.1016/j.clim.2016.07.025.

@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., 2016, roč.~171, ''neuvedeno'', s.~12-17. ISSN~1521-6616. Dostupné z: https://dx.doi.org/10.1016/j.clim.2016.07.025.

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