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Naslov:Hereditary systemic autoinflammatory diseases and Schnitzler's syndrome
Avtorji:Kačar, Mark (Avtor)
Pathak, Shelly (Avtor)
Savic, Sinisa (Avtor)
Jezik:Angleški jezik
Tipologija:1.02 - Pregledni znanstveni članek
Organizacija:Logo UKPBAG - Univerzitetna klinika za pljučne bolezni in alergijo Golnik
Povzetek:The systemic autoinflammatory diseases are disorders of the innate immune system distinguished by severe inflammation resulting from dysregulation of the innate immune system. Hereditary fever syndromes, such as FMF, TNF receptor-associated periodic syndrome, cryopyrin-associated periodic syndromes and mevalonate kinase deficiency, were the first group of systemic autoinflammatory diseases for which a genetic basis was established, between 1999 and 2001. Currently according to the latest report of the international union of immunological societies, 37 separate monogenic disorders were classified as autoinflammatory. In addition to the abovementioned monogenic conditions, we describe Schnitzler's syndrome, a well-defined, acquired autoinflammatory condition without a clear genetic basis. For the purposes of this review, we discuss several conditions defined by the latest consensus process as systemic autoinflammatory diseases. We focus on those disorders where recent studies have contributed to further phenotypic characterization or had an impact on clinical management.
Ključne besede:pyrin, Schnitzler syndrome, haploinsufficiency, autoinflammatory diseases, pyrin-associated autoinflammatory diseases, NLRP3-related autoinflammatory diseases, undifferentiated systemic autoinflammatory disease, relopathies
Leto izida:2019
Založnik:Oxford University Press
Izvor:Velika Britanija
UDK:616-002
ISSN pri članku:1462-0332
COBISS_ID:58561539 Povezava se odpre v novem oknu
DOI:10.1093/rheumatology/kez448 Povezava se odpre v novem oknu
Opombe:Nasl. z nasl. zaslona; Opis vira z dne 7. 4. 2021;
Število ogledov:68
Število prenosov:45
Datoteke:.pdf PDF - Predstavitvena datoteka, prenos (455,31 KB)
URL URL - Izvorni URL, za dostop obiščite https://academic.oup.com/rheumatology/article-pdf/58/Supplement_6/vi31/31139742/kez448.pdf
 
Nadgradivo:Rheumatol.
Oxford University Press
 
Metapodatki:XML RDF-CHPDL DC-XML DC-RDF
Avtorske pravice:© The Author(s) 2019
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Licence

Licenca:CC BY-NC 4.0, Creative Commons Priznanje avtorstva-Nekomercialno 4.0 Mednarodna
Povezava:http://creativecommons.org/licenses/by-nc/4.0/deed.sl
Opis:Licenca Creative Commons, ki prepoveduje komercialno uporabo, vendar uporabniki ne rabijo upravljati materialnih avtorskih pravic na izpeljanih delih z enako licenco.
Začetek licenciranja:26.11.2019

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