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231224s2014 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2014.04.005
|2 doi
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|a pubmed24n1446.xml
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|a (DE-627)NLM237464993
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|a (NLM)24743019
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|a DE-627
|b ger
|c DE-627
|e rakwb
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|a eng
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| 100 |
1 |
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|a Al-Zahrani, Daifulah
|e verfasserin
|4 aut
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| 245 |
1 |
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|a Successful interferon-alpha 2b therapy for unremitting warts in a patient with DOCK8 deficiency
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|c 2014
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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 12.09.2014
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|a Date Revised 20.06.2024
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Copyright © 2014. Published by Elsevier Inc.
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|a The autosomal recessive form of the Hyper IgE syndrome (AR-HIES) with dedicator of cytokinesis 8 (DOCK8) deficiency is associated with difficult to treat persistent viral skin infections, including papilloma virus infection. Type I interferons play an important role in the defense against viruses. We examined the effect of therapy with IFN-α 2b in an 11-year old boy with DOCK8 deficiency due to a homozygous splice donor site mutation in DOCK8 intron 40. His unremitting warts showed dramatic response to IFN-α 2b therapy. Immunological studies revealed decreased circulating plasmacytoid dendritic cells (pDCs) and profound deficiency of IFN-α production by his peripheral blood mononuclear cells in response to treatment with CpG oligonucleotides. These findings indicate that underlying pDC deficiency and impaired IFN-α production may predispose to chronic viral infections in DOCK8 deficiency. IFN-α 2b therapy maybe useful in controlling recalcitrant viral infections in these patients
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|a Case Reports
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|a Journal Article
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|a Research Support, N.I.H., Extramural
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| 650 |
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|a Research Support, Non-U.S. Gov't
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| 650 |
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|a DOCK8 deficiency
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| 650 |
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|a Hyper -immunoglobulin E syndrome
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| 650 |
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|a Interferon–α 2b
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| 650 |
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|a Papilloma Virus
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| 650 |
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4 |
|a Warts
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| 650 |
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|a DOCK8 protein, human
|2 NLM
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| 650 |
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|a Guanine Nucleotide Exchange Factors
|2 NLM
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| 650 |
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|a Immunologic Factors
|2 NLM
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| 650 |
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|a Interferon alpha-2
|2 NLM
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| 650 |
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|a Interferon-alpha
|2 NLM
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| 650 |
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|a Recombinant Proteins
|2 NLM
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| 700 |
1 |
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|a Raddadi, Ali
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Massaad, Michel
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Keles, Sevgi
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Jabara, Haifa H
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Chatila, Talal A
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Geha, Raif
|e verfasserin
|4 aut
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| 773 |
0 |
8 |
|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 153(2014), 1 vom: 05. Juli, Seite 104-108
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
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| 773 |
1 |
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|g volume:153
|g year:2014
|g number:1
|g day:05
|g month:07
|g pages:104-108
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|u http://dx.doi.org/10.1016/j.clim.2014.04.005
|3 Volltext
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