Periodic Fever Syndromes
Authors:
P. Król 1; R. Katra 2; P. Doležalová 1
Authors‘ workplace:
Klinika dětského a dorostového lékařství UK 1. LF a VFN, Praha
přednosta prof. MUDr. J. Zeman, DrSc.
1; Klinika ušní, nosní a krční UK 2. LF a FN Motol, Praha
přednosta doc. MUDr. Z. Kabelka
2
Published in:
Čes-slov Pediat 2009; 64 (10): 480-487.
Category:
Review
Overview
Periodic fever syndromes are clinical entities classified as autoinflammatory diseases. A relatively new group of periodic fever syndromes are characterised by failure of innate immunity mechanisms. Recurrent episodes of fever are accompanied by local inflammation.
They include two autosomal dominant disorders:
CAPS (Cryopyrin Associated Periodic Syndromes) and TRAPS (TNFR-Associated Periodic Syndrome) and autosomal recessive ones: FMF (Familiar Mediterranean Fever) and MAPS (Mevalonate kinase Associated Periodic fever Syndrome). PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis a Adenitis) syndrome is an idiopathic disease with unknown aetiology.
Key words:
periodic fever, innate immunity, inflammation
Sources
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Labels
Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2009 Issue 10
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