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2153 The plasma contact system and its role in common variable immunodeficiency (CVID): An explorative study

OBJECTIVES/SPECIFIC AIMS: Assess the presence of contact activation at baseline in sera from common variable immunodeficiency (CVID) patients with and without inflammatory complications compared with healthy controls. METHODS/STUDY POPULATION: CVID patients were recruited in the outpatient setting a...

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Autores principales: Smith, Tukisa, Ponda, Manish, Breslow, Jan, Cunningham-Rundles, Charlotte
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cambridge University Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6799162/
http://dx.doi.org/10.1017/cts.2018.136
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author Smith, Tukisa
Ponda, Manish
Breslow, Jan
Cunningham-Rundles, Charlotte
author_facet Smith, Tukisa
Ponda, Manish
Breslow, Jan
Cunningham-Rundles, Charlotte
author_sort Smith, Tukisa
collection PubMed
description OBJECTIVES/SPECIFIC AIMS: Assess the presence of contact activation at baseline in sera from common variable immunodeficiency (CVID) patients with and without inflammatory complications compared with healthy controls. METHODS/STUDY POPULATION: CVID patients were recruited in the outpatient setting and the measurement of cleaved plasma HK (cHK) levels was determined by Western blot analysis, under reducing conditions, with quantitation of total and cHK bands using an Odyssey imaging system (Licor). One-way ANOVA test for differences among the 3 studied groups will be applied. Biomarkers C3, C4, C1 inhibitor levels and hs-CRP were also measured. RESULTS/ANTICIPATED RESULTS: Participant enrollment continues and to date, 9 CVID patients were studied, 7 with and 2 without inflammatory complications. Repeated determinations of cleaved HK% (cHK%) revealed an average of 1.20% (range: 0.46%–2.66%) in CVID patients with inflammatory complications and those without complications averaged 1.07% (range: 0.79%–1.35%). Healthy controls had an average cHK of 1.15% (range: 0.60%–2.10%). DISCUSSION/SIGNIFICANCE OF IMPACT: Cleaved kininogen detected in the sera of CVID patients was found at similar levels compared with healthy controls (cHK<5%). Findings suggest that systemic activation of the contact system might be absent in CVID, however, future considerations include developing detection methods for local tissue activation.
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spelling pubmed-67991622019-10-28 2153 The plasma contact system and its role in common variable immunodeficiency (CVID): An explorative study Smith, Tukisa Ponda, Manish Breslow, Jan Cunningham-Rundles, Charlotte J Clin Transl Sci Basic/Translational Science/Team Science OBJECTIVES/SPECIFIC AIMS: Assess the presence of contact activation at baseline in sera from common variable immunodeficiency (CVID) patients with and without inflammatory complications compared with healthy controls. METHODS/STUDY POPULATION: CVID patients were recruited in the outpatient setting and the measurement of cleaved plasma HK (cHK) levels was determined by Western blot analysis, under reducing conditions, with quantitation of total and cHK bands using an Odyssey imaging system (Licor). One-way ANOVA test for differences among the 3 studied groups will be applied. Biomarkers C3, C4, C1 inhibitor levels and hs-CRP were also measured. RESULTS/ANTICIPATED RESULTS: Participant enrollment continues and to date, 9 CVID patients were studied, 7 with and 2 without inflammatory complications. Repeated determinations of cleaved HK% (cHK%) revealed an average of 1.20% (range: 0.46%–2.66%) in CVID patients with inflammatory complications and those without complications averaged 1.07% (range: 0.79%–1.35%). Healthy controls had an average cHK of 1.15% (range: 0.60%–2.10%). DISCUSSION/SIGNIFICANCE OF IMPACT: Cleaved kininogen detected in the sera of CVID patients was found at similar levels compared with healthy controls (cHK<5%). Findings suggest that systemic activation of the contact system might be absent in CVID, however, future considerations include developing detection methods for local tissue activation. Cambridge University Press 2018-11-21 /pmc/articles/PMC6799162/ http://dx.doi.org/10.1017/cts.2018.136 Text en © The Association for Clinical and Translational Science 2018 http://creativecommons.org/licenses/by/4.0/ This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Basic/Translational Science/Team Science
Smith, Tukisa
Ponda, Manish
Breslow, Jan
Cunningham-Rundles, Charlotte
2153 The plasma contact system and its role in common variable immunodeficiency (CVID): An explorative study
title 2153 The plasma contact system and its role in common variable immunodeficiency (CVID): An explorative study
title_full 2153 The plasma contact system and its role in common variable immunodeficiency (CVID): An explorative study
title_fullStr 2153 The plasma contact system and its role in common variable immunodeficiency (CVID): An explorative study
title_full_unstemmed 2153 The plasma contact system and its role in common variable immunodeficiency (CVID): An explorative study
title_short 2153 The plasma contact system and its role in common variable immunodeficiency (CVID): An explorative study
title_sort 2153 the plasma contact system and its role in common variable immunodeficiency (cvid): an explorative study
topic Basic/Translational Science/Team Science
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6799162/
http://dx.doi.org/10.1017/cts.2018.136
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