الفهرس 
Introduction & Aim of the WorkOnly 14 pages are availabe for public view
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Abstract
Introduction: Periodic Fever Syndromes are a heterogeneous group of disorders characterized by recurrent episodes of fever and localized inflammation, most commonly affecting the serosal membranes, joints, and skin. Objectives: to highlight the presentation, clinical course, physicians’ awareness, therapeutic response and long term follow up of PFS in infancy& childhood. Methods: the diagnosis of PFS based mainly on clinical suspicion but needs to be confirmed through detection of the corresponding mutations which carry correlation with the occurrence of amyloidosis. Results: the most distinctive PFS are (FMF) and (HIDS) which are autosomal recessive, whereas (TRAPS), (MWS), (FCAS) and (CINCA) syndrome are autosomal dominant. Another PFS (PFAPA) which is a common non genetic cause of recurrent fever in children. Amyloidosis is a long term consequence of their inflammatory episodes and complications related to renal failure and renal replacement therapy are the main cause of death. TNF α and interleukin (IL)-1 play important role in the pathogenesis of all the periodic fever syndromes and biologicals aimed against these cytokines are promising agents in the treatment PFS. Conclusions: PFS have extended our knowledge of inflammatory mechanisms and shed further light on the mechanisms of amyloidosis.