Navigation überspringen
Universitätsbibliothek Heidelberg
Status: Bibliographieeintrag

Verfügbarkeit
Standort: ---
Exemplare: ---
heiBIB
 Online-Ressource
Verfasst von:Brodszki, Nicholas [VerfasserIn]   i
 Frazer-Abel, Ashley [VerfasserIn]   i
 Grumach, Anete S. [VerfasserIn]   i
 Kirschfink, Michael [VerfasserIn]   i
 Litzman, Jiri [VerfasserIn]   i
 Perez, Elena [VerfasserIn]   i
 Seppänen, Mikko R. J. [VerfasserIn]   i
 Sullivan, Kathleen E. [VerfasserIn]   i
 Jolles, Stephen [VerfasserIn]   i
Titel:European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA) complement guideline
Titelzusatz:deficiencies, diagnosis, and management
Verf.angabe:Nicholas Brodszki, Ashley Frazer-Abel, Anete S. Grumach, Michael Kirschfink, Jiri Litzman, Elena Perez, Mikko R.J. Seppänen, Kathleen E. Sullivan, Stephen Jolles
E-Jahr:2020
Jahr:17 February 2020
Umfang:16 S.
Fussnoten:Gesehen am 23.06.2021
Titel Quelle:Enthalten in: Journal of clinical immunology
Ort Quelle:Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981
Jahr Quelle:2020
Band/Heft Quelle:40(2020), 4, Seite 576-591
ISSN Quelle:1573-2592
Abstract:This guideline aims to describe the complement system and the functions of the constituent pathways, with particular focus on primary immunodeficiencies (PIDs) and their diagnosis and management. The complement system is a crucial part of the innate immune system, with multiple membrane-bound and soluble components. There are three distinct enzymatic cascade pathways within the complement system, the classical, alternative and lectin pathways, which converge with the cleavage of central C3. Complement deficiencies account for ~5% of PIDs. The clinical consequences of inherited defects in the complement system are protean and include increased susceptibility to infection, autoimmune diseases (e.g., systemic lupus erythematosus), age-related macular degeneration, renal disorders (e.g., atypical hemolytic uremic syndrome) and angioedema. Modern complement analysis allows an in-depth insight into the functional and molecular basis of nearly all complement deficiencies. However, therapeutic options remain relatively limited for the majority of complement deficiencies with the exception of hereditary angioedema and inhibition of an overactivated complement system in regulation defects. Current management strategies for complement disorders associated with infection include education, family testing, vaccinations, antibiotics and emergency planning.
DOI:doi:10.1007/s10875-020-00754-1
URL:Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.

Volltext: https://doi.org/10.1007/s10875-020-00754-1
 DOI: https://doi.org/10.1007/s10875-020-00754-1
Datenträger:Online-Ressource
Sprache:eng
Sach-SW:alternative pathway
 classical pathway
 Complement
 complement deficiencies
 mannan-binding lectin
K10plus-PPN:1761101706
Verknüpfungen:→ Zeitschrift

Permanenter Link auf diesen Titel (bookmarkfähig):  https://katalog.ub.uni-heidelberg.de/titel/68751848   QR-Code
zum Seitenanfang