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BMC Pediatrics
Published in: BMC Pediatrics 1/2010

Open Access 01-12-2010 | Research article

Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome

Authors: Kelly L Brown, Per Wekell, Veronica Osla, Martina Sundqvist, Karin Sävman, Anders Fasth, Anna Karlsson, Stefan Berg

Published in: BMC Pediatrics | Issue 1/2010

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Abstract

Background

This study aimed to profile levels of blood cells and serum cytokines during afebrile and febrile phases of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome to advance pathophysiological understanding of this pediatric disease.

Methods

A cohort of patients with a median age of 4.9 years experiencing 'typical PFAPA' episodes participated in this study. Blood cells and serum cytokines were analyzed by CBC analysis and multiplex ELISA.

Results

Oscillations in the concentration of blood cells during the afebrile and febrile phases of typical PFAPA syndrome were observed; novel findings include increased monocytes and decreased eosinophils during a febrile episode and increased thrombocytes in the afebrile interval. Relatively modest levels of pro-inflammatory cytokines were present in sera. IFNγ-induced cytokine IP10/CXCL10 was increased after the onset of fever while T cell-associated cytokines IL7 and IL17 were suppressed during afebrile and febrile periods.

Conclusions

Identification of dysregulated blood cells and serum cytokines is an initial step towards the identification of biomarkers of PFAPA disease and/or players in disease pathogenesis. Future investigations are required to conclusively discern which mediators are associated specifically with PFAPA syndrome.
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Metadata
Title
Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome
Authors
Kelly L Brown
Per Wekell
Veronica Osla
Martina Sundqvist
Karin Sävman
Anders Fasth
Anna Karlsson
Stefan Berg
Publication date
01-12-2010
Publisher
BioMed Central
Published in
BMC Pediatrics / Issue 1/2010
Electronic ISSN: 1471-2431
DOI
https://doi.org/10.1186/1471-2431-10-65

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