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Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999

Common variable immunodeficiency (CVID) is the most clinically significant primary antibody immunodeficiency recognized in adulthood. Previously published data have shown an average diagnostic delay of 10 years for Polish adult patients with CVID. In the current study, we aimed to analyze the curren...

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Autores principales: Ziętkiewicz, Marcin, Więsik-Szewczyk, Ewa, Matyja-Bednarczyk, Aleksandra, Napiórkowska-Baran, Katarzyna, Zdrojewski, Zbigniew, Jahnz-Różyk, Karina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7326034/
https://www.ncbi.nlm.nih.gov/pubmed/32655544
http://dx.doi.org/10.3389/fimmu.2020.00982
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author Ziętkiewicz, Marcin
Więsik-Szewczyk, Ewa
Matyja-Bednarczyk, Aleksandra
Napiórkowska-Baran, Katarzyna
Zdrojewski, Zbigniew
Jahnz-Różyk, Karina
author_facet Ziętkiewicz, Marcin
Więsik-Szewczyk, Ewa
Matyja-Bednarczyk, Aleksandra
Napiórkowska-Baran, Katarzyna
Zdrojewski, Zbigniew
Jahnz-Różyk, Karina
author_sort Ziętkiewicz, Marcin
collection PubMed
description Common variable immunodeficiency (CVID) is the most clinically significant primary antibody immunodeficiency recognized in adulthood. Previously published data have shown an average diagnostic delay of 10 years for Polish adult patients with CVID. In the current study, we aimed to analyze the current diagnostic delay of adult patients with CVID in Poland. To this end, we identified patients from four immunological centers specialized in the care of adult patients with primary immunodeficiencies (PID). Demographic and clinical data of patients were collected using an internet database. We identified 103 adult patients (F:M 44.7%:55.3%) in Poland with CVID. The median age at onset of symptoms was 24 (0–66), 33 (4–70) at diagnosis, and 37 (18–73) years at the time of analysis. The median diagnostic delay for the entire study population was 6 (0–57) years. However, this delay was higher in patients with symptom onset before the year 2000 than after the year 1999 [15 (0–57) vs. 3 (0–19) years; p < 0.001]. Comparing patients (median ≤ 6 years, N = 53) with short diagnostic delay (SDD) and those (median > 6 years, N = 50) with long diagnostic delay (LDD), the LDD group had a statistically significant higher incidence of infections of the lower respiratory tract before diagnosis (90.0 vs. 71.70%). During the entire observation period, cytopenias (44.00 vs. 22.64%), granulomatous lesions (28.00 vs. 11.32%), and solid tumors (14.00 vs. 1.89%) were significantly more frequent in the LDD group. In conclusion, we found a significant reduction in the median diagnostic delay in Polish CVID patients with disease onset in the last two decades.
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spelling pubmed-73260342020-07-09 Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999 Ziętkiewicz, Marcin Więsik-Szewczyk, Ewa Matyja-Bednarczyk, Aleksandra Napiórkowska-Baran, Katarzyna Zdrojewski, Zbigniew Jahnz-Różyk, Karina Front Immunol Immunology Common variable immunodeficiency (CVID) is the most clinically significant primary antibody immunodeficiency recognized in adulthood. Previously published data have shown an average diagnostic delay of 10 years for Polish adult patients with CVID. In the current study, we aimed to analyze the current diagnostic delay of adult patients with CVID in Poland. To this end, we identified patients from four immunological centers specialized in the care of adult patients with primary immunodeficiencies (PID). Demographic and clinical data of patients were collected using an internet database. We identified 103 adult patients (F:M 44.7%:55.3%) in Poland with CVID. The median age at onset of symptoms was 24 (0–66), 33 (4–70) at diagnosis, and 37 (18–73) years at the time of analysis. The median diagnostic delay for the entire study population was 6 (0–57) years. However, this delay was higher in patients with symptom onset before the year 2000 than after the year 1999 [15 (0–57) vs. 3 (0–19) years; p < 0.001]. Comparing patients (median ≤ 6 years, N = 53) with short diagnostic delay (SDD) and those (median > 6 years, N = 50) with long diagnostic delay (LDD), the LDD group had a statistically significant higher incidence of infections of the lower respiratory tract before diagnosis (90.0 vs. 71.70%). During the entire observation period, cytopenias (44.00 vs. 22.64%), granulomatous lesions (28.00 vs. 11.32%), and solid tumors (14.00 vs. 1.89%) were significantly more frequent in the LDD group. In conclusion, we found a significant reduction in the median diagnostic delay in Polish CVID patients with disease onset in the last two decades. Frontiers Media S.A. 2020-05-27 /pmc/articles/PMC7326034/ /pubmed/32655544 http://dx.doi.org/10.3389/fimmu.2020.00982 Text en Copyright © 2020 Ziętkiewicz, Więsik-Szewczyk, Matyja-Bednarczyk, Napiórkowska-Baran, Zdrojewski and Jahnz-Różyk. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Immunology
Ziętkiewicz, Marcin
Więsik-Szewczyk, Ewa
Matyja-Bednarczyk, Aleksandra
Napiórkowska-Baran, Katarzyna
Zdrojewski, Zbigniew
Jahnz-Różyk, Karina
Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999
title Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999
title_full Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999
title_fullStr Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999
title_full_unstemmed Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999
title_short Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999
title_sort shorter diagnostic delay in polish adult patients with common variable immunodeficiency and symptom onset after 1999
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7326034/
https://www.ncbi.nlm.nih.gov/pubmed/32655544
http://dx.doi.org/10.3389/fimmu.2020.00982
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