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231223s2011 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2011.01.003
|2 doi
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|a eng
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| 100 |
1 |
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|a Csuka, Dorottya
|e verfasserin
|4 aut
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|a Parameters of the classical complement pathway predict disease severity in hereditary angioedema
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|c 2011
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|a Text
|b txt
|2 rdacontent
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|a ƒaComputermedien
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|a ƒa Online-Ressource
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|a Date Completed 01.06.2011
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|a Date Revised 21.11.2013
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Copyright © 2011 Elsevier Inc. All rights reserved.
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|a Functional C1-inhibitor (C1-inh) and C4 are potential severity markers of hereditary angioedema due to deficiency of C1-inh (HAE-C1-inh), and the complexes generated through complement activation may be relevant. We studied the association between disease severity and complement parameters in 105 HAE-C1-inh patients. Disease severity was characterized by the number of angioedema attacks or alternatively, by the number of C1-inh concentrate ampoules (C1-inh-amp) used for the treatment of attacks. Median C1rC1sC1-inh level was higher (32.8 U/ml vs. 3.4 U/ml; p<0.0001) in patients, compared to controls. C1rC1sC1-inh and C1-inh strongly correlated with attack number and C1-inh-amp, both in the whole patient population and in the subgroup on danazol prophylaxis. Both C1rC1sC1-inh and C1-inh are suitable for predicting disease severity based on attack frequency and C1-inh-amp (OR=4.38[1.43-13.43], p=0.010 and 11.78[2.54-54.67], p=0.002, respectively). We presume that both C1rC1sC1-inh and C1-inh might prove sensitive predictors of the severity of HAE-C1-inh
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|a Journal Article
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|a Estrogen Antagonists
|2 NLM
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| 650 |
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|a Genetic Markers
|2 NLM
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| 650 |
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|a Danazol
|2 NLM
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| 650 |
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|a N29QWW3BUO
|2 NLM
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| 700 |
1 |
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|a Füst, George
|e verfasserin
|4 aut
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1 |
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|a Farkas, Henriette
|e verfasserin
|4 aut
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| 700 |
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|a Varga, Lilian
|e verfasserin
|4 aut
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| 773 |
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 139(2011), 1 vom: 04. Apr., Seite 85-93
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
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|g volume:139
|g year:2011
|g number:1
|g day:04
|g month:04
|g pages:85-93
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|u http://dx.doi.org/10.1016/j.clim.2011.01.003
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