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231224s2013 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2013.05.007
|2 doi
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|a pubmed25n0761.xml
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|a (DE-627)NLM228414873
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|a (NLM)23773925
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|a (PII)S1521-6616(13)00127-7
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|a DE-627
|b ger
|c DE-627
|e rakwb
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|a eng
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|a Matharu, Kabir
|e verfasserin
|4 aut
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|a B-cell activating factor (BAFF) is elevated in chronic granulomatous disease
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|c 2013
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|a Text
|b txt
|2 rdacontent
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|a ƒaComputermedien
|b c
|2 rdamedia
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|a ƒa Online-Ressource
|b cr
|2 rdacarrier
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|a Date Completed 17.09.2013
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|a Date Revised 03.01.2025
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Published by Elsevier Inc.
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|a Chronic Granulomatous Disease (CGD) is an inherited defect in superoxide production leading to life-threatening infections, granulomas, and, possibly, abnormal immunoglobulin concentrations. We investigated whether factors controlling antibody production, such as B-cell activating factor (BAFF), were altered in CGD. CGD subjects had significantly increased mean (2.3-fold, p < 0.0001) plasma concentrations of BAFF compared to healthy donors. Patients on IFN-γ treatment had significantly higher BAFF concentrations compared with CGD patients not taking IFN-γ (1.6-fold, p < 0.005). Leukocytes from CGD subjects produced normal amounts of BAFF in response to IFN-γ or G-CSF in vitro. Expression of BAFF-R and TACI was significantly reduced on CGD B cells. Elevated BAFF in CGD correlated with CRP (R = 0.44), ESR (R = 0.49), and IgM (R = 0.47) and increased rapidly in healthy subjects following intravenous endotoxin administration. These findings suggest that elevated BAFF in CGD subjects and healthy donors is a consequence of acute and chronic inflammation
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|a Journal Article
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|a Research Support, N.I.H., Intramural
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|a Research Support, Non-U.S. Gov't
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|a B cell activating factor
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|a B cell receptor
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|a B-cell activating factor
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|a BAFF
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|a BCR
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|a CGD
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|a Chronic Granulomatous Disease
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|a Inflammation
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|a TACI
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|a transmembrane activator and CAML-interactor.
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|a B-Cell Activating Factor
|2 NLM
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|a Biomarkers
|2 NLM
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|a Endotoxins
|2 NLM
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|a Interleukins
|2 NLM
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|a Tumor Necrosis Factor Ligand Superfamily Member 13
|2 NLM
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|a Interleukin-21
|2 NLM
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|a MKM3CA6LT1
|2 NLM
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|a Zarember, Kol A
|e verfasserin
|4 aut
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|a Marciano, Beatriz E
|e verfasserin
|4 aut
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|a Kuhns, Douglas B
|e verfasserin
|4 aut
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|a Spalding, Christine
|e verfasserin
|4 aut
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|a Garofalo, Mary
|e verfasserin
|4 aut
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|a Dimaggio, Thomas
|e verfasserin
|4 aut
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|a Estwick, Tyra
|e verfasserin
|4 aut
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|a Huang, Chiung-Yu
|e verfasserin
|4 aut
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|a Fink, Danielle
|e verfasserin
|4 aut
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|a Priel, Debra L
|e verfasserin
|4 aut
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|a Fleisher, Thomas A
|e verfasserin
|4 aut
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|a Holland, Steven M
|e verfasserin
|4 aut
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|a Malech, Harry L
|e verfasserin
|4 aut
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|a Gallin, John I
|e verfasserin
|4 aut
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 148(2013), 2 vom: 17. Aug., Seite 258-64
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
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|g volume:148
|g year:2013
|g number:2
|g day:17
|g month:08
|g pages:258-64
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|u http://dx.doi.org/10.1016/j.clim.2013.05.007
|3 Volltext
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|a AR
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|d 148
|j 2013
|e 2
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|h 258-64
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