PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, (PFAPA) SYNDROME AND NLRP3 GENE ASSOCIATION

Gülşah Ünsal

doi:10.7197/cmj.1310396

EN

PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, (PFAPA) SYNDROME AND NLRP3 GENE ASSOCIATION

Abstract

PFAPA syndrome, which also includes aphthous stomatitis, pharyngitis, and adenitis, is the most common type of recurrent fever in children. Usually happens before the age of five. This syndrome is characterized by attacks lasting 3-7 days, recurring every 2-8 weeks with high fever (39 C and above) accompanied by at least one of the signs of aphthous stomatitis, pharyngitis, and/or cervical adenitis. Between attacks, the child is completely healthy.PFAPA is a benign disease that regresses with age. Steroids are used in the treatment of attacks. Colchicine is often preferred in prophylactic treatment. However, if there is no response to medical treatment, surgery (tonsillectomy) can be performed. Genetic and environmental factors are considered in the etiology. Genetic susceptibility concentrated on the genes for Familiar Mediterranean Fever (FMF, MEFV), TNF-Receptor-Associated Periodic Syndrome (TRAPS, gene TNFRF1A), HyperIgDSyndrome (HIDS, gene MVK), and Cryopyrin-Associated Periodic Syndrome (CAPS, gene NLRP3). But its etiology is still unknown.

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Details

Primary Language

English

Subjects

Family Medicine

Journal Section

Review

Authors

Gülşah Ünsal ^*
0000-0003-4431-395X
Türkiye

Early Pub Date

June 30, 2023

Publication Date

June 30, 2023

Submission Date

June 6, 2023

Acceptance Date

June 25, 2023

Published in Issue

Year 2023 Volume: 45 Number: 2

DOI

https://doi.org/10.7197/cmj.1310396

IZ

https://izlik.org/JA47GL53PU

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RIS / Bibtex

AMA

1.Ünsal G. PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, (PFAPA) SYNDROME AND NLRP3 GENE ASSOCIATION. CMJ. 2023;45(2):1-6. doi:10.7197/cmj.1310396

PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, (PFAPA) SYNDROME AND NLRP3 GENE ASSOCIATION

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Keywords

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Supporting Institution

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References

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Subjects

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Acceptance Date

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