| TURLEY, A.J., B. GATHMANN, C. BANGS, M. BRADBURY, S. SENEVIRATNE, L.I. GONZALEZ-GRANADO, S. HACKETT, N. KUTUKCULER, H. ALACHKAR, S. HAMBLETON, H. RITTERBUSCH, P. KRALICKOVA, L. MARODI, M.G. SEIDEL, G. DUECKERS, J. ROESLER, A. HUISSOON, H. BAXENDALE, Jiří LITZMAN a P.D. ARKWRIGHT. Spectrum and Management of Complement Immunodeficiencies (Excluding Hereditary Angioedema) Across Europe. Journal of Clinical Immunology. New York: Springer, 2015, roč. 35, č. 2, s. 199-205. ISSN 0271-9142. Dostupné z: https://dx.doi.org/10.1007/s10875-015-0137-5. |
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@article{1300080,
author = {Turley, A.J. and Gathmann, B. and Bangs, C. and Bradbury, M. and Seneviratne, S. and GonzalezandGranado, L.I. and Hackett, S. and Kutukculer, N. and Alachkar, H. and Hambleton, S. and Ritterbusch, H. and Kralickova, P. and Marodi, L. and Seidel, M.G. and Dueckers, G. and Roesler, J. and Huissoon, A. and Baxendale, H. and Litzman, Jiří and Arkwright, P.D.},
article_location = {New York},
article_number = {2},
doi = {http://dx.doi.org/10.1007/s10875-015-0137-5},
keywords = {Complement; immunodeficiency; meningococcemia; Streptococcus pneumoniae; atypical hemolytic uremic syndrome; glomerulopathy; vaccination; antibiotics},
language = {eng},
issn = {0271-9142},
journal = {Journal of Clinical Immunology},
title = {Spectrum and Management of Complement Immunodeficiencies (Excluding Hereditary Angioedema) Across Europe},
volume = {35},
year = {2015}
}
TY - JOUR
ID - 1300080
AU - Turley, A.J. - Gathmann, B. - Bangs, C. - Bradbury, M. - Seneviratne, S. - Gonzalez-Granado, L.I. - Hackett, S. - Kutukculer, N. - Alachkar, H. - Hambleton, S. - Ritterbusch, H. - Kralickova, P. - Marodi, L. - Seidel, M.G. - Dueckers, G. - Roesler, J. - Huissoon, A. - Baxendale, H. - Litzman, Jiří - Arkwright, P.D.
PY - 2015
TI - Spectrum and Management of Complement Immunodeficiencies (Excluding Hereditary Angioedema) Across Europe
JF - Journal of Clinical Immunology
VL - 35
IS - 2
SP - 199-205
EP - 199-205
PB - Springer
SN - 02719142
KW - Complement
KW - immunodeficiency
KW - meningococcemia
KW - Streptococcus pneumoniae
KW - atypical hemolytic uremic syndrome
KW - glomerulopathy
KW - vaccination
KW - antibiotics
N2 - 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). Physicians in the UK were more likely to prescribe antibiotic prophylaxis than colleagues on the Continent for patients with classical pathway defects. After diagnosis, 16 % of patients suffered serious bacterial infections. Age of the patient and use of prophylactic antibiotics were not associated with subsequent infection risk. Inflammatory/autoimmune diseases were not seen in patients with terminal pathway, but in one third of patients classical and alternative pathway defects. The clinical phenotypes of specific complement immunodeficiencies vary considerably both in terms of the predominant bacterial pathogen, and the risk and type of auto-inflammatory disease. Appreciation of these phenotypic differences should help both immunologists and other specialists in their diagnosis and management of these rare and complex patients.
ER -
TURLEY, A.J., B. GATHMANN, C. BANGS, M. BRADBURY, S. SENEVIRATNE, L.I. GONZALEZ-GRANADO, S. HACKETT, N. KUTUKCULER, H. ALACHKAR, S. HAMBLETON, H. RITTERBUSCH, P. KRALICKOVA, L. MARODI, M.G. SEIDEL, G. DUECKERS, J. ROESLER, A. HUISSOON, H. BAXENDALE, Jiří LITZMAN a P.D. ARKWRIGHT. Spectrum and Management of Complement Immunodeficiencies (Excluding Hereditary Angioedema) Across Europe. \textit{Journal of Clinical Immunology}. New York: Springer, 2015, roč.~35, č.~2, s.~199-205. ISSN~0271-9142. Dostupné z: https://dx.doi.org/10.1007/s10875-015-0137-5.
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