Spectrum and Management of Complement Immunodeficiencies (Excluding Hereditary Angioedema) Across Europe
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216224%3A14110%2F15%3A00082978" target="_blank" >RIV/00216224:14110/15:00082978 - isvavai.cz</a>
Alternative codes found
RIV/00159816:_____/15:00061743 RIV/00179906:_____/15:10292423
Result on the web
<a href="http://dx.doi.org/10.1007/s10875-015-0137-5" target="_blank" >http://dx.doi.org/10.1007/s10875-015-0137-5</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1007/s10875-015-0137-5" target="_blank" >10.1007/s10875-015-0137-5</a>
Alternative languages
Result language
angličtina
Original language name
Spectrum and Management of Complement Immunodeficiencies (Excluding Hereditary Angioedema) Across Europe
Original language description
Complement immunodeficiencies (excluding hereditary angioedema and mannose binding lectin deficiency) are rare. Published literature consists largely of case reports and small series. We collated data from 18 cities across Europe to provide an overview of primarily homozygous, rather than partial genotypes and their impact and management. Patients were recruited through the ESID registry. Clinical and laboratory information was collected onto standardized forms and analyzed using SPSS software. Seventy-seven patients aged 1 to 68 years were identified. 44 % presented in their first decade of life. 29 % had C2 deficiency, defects in 11 other complement factors were found. 50 (65 %) had serious invasive infections. 61 % of Neisseria meningitidis infections occurred in patients with terminal pathway defects, while 74 % of Streptococcus pneumoniae infections occurred in patients with classical pathway defects (p < 0.001).
Czech name
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Czech description
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Classification
Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
EC - Immunology
OECD FORD branch
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Result continuities
Project
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2015
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Journal of Clinical Immunology
ISSN
0271-9142
e-ISSN
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Volume of the periodical
35
Issue of the periodical within the volume
2
Country of publishing house
US - UNITED STATES
Number of pages
7
Pages from-to
199-205
UT code for WoS article
000350886300014
EID of the result in the Scopus database
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