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Periodic Fever Syndromes


Authors: P. Król 1;  R. Katra 2;  P. Doležalová 1
Authors place of work: 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 the journal: Čes-slov Pediat 2009; 64 (10): 480-487.
Category: Review

Summary

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


Zdroje

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Štítky
Neonatology Paediatrics General practitioner for children and adolescents
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