Spectrum and Management of Complement Immunodeficiencies (Excluding Hereditary Angioedema) Across Europe

Kód výsledku v 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>

Nalezeny alternativní kódy

RIV/00159816:_____/15:00061743 RIV/00179906:_____/15:10292423

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

Spectrum and Management of Complement Immunodeficiencies (Excluding Hereditary Angioedema) Across Europe

Popis výsledku v původním jazyce

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 &lt; 0.001).

Název v anglickém jazyce

Spectrum and Management of Complement Immunodeficiencies (Excluding Hereditary Angioedema) Across Europe

Popis výsledku anglicky

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 &lt; 0.001).

Druh

J_x - Nezařazeno - Článek v odborném periodiku (Jimp, Jsc a Jost)

CEP obor

EC - Imunologie

OECD FORD obor

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Projekt

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Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2015

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Journal of Clinical Immunology

ISSN

0271-9142

e-ISSN

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Svazek periodika

35

Číslo periodika v rámci svazku

2

Stát vydavatele periodika

US - Spojené státy americké

Počet stran výsledku

7

Strana od-do

199-205

Kód UT WoS článku

000350886300014

EID výsledku v databázi Scopus

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