scholarly journals Effectiveness of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome: a case series

2021 ◽  
Vol 61 (3) ◽  
pp. 171-4
Author(s):  
Kazuto Taniguchi ◽  
Sakumo Kii ◽  
Kazuyasu Uemichi
Keyword(s):  
Periodic Fever ◽  
Case Series ◽  
Oral Prednisolone ◽  
High Fever ◽  
Aphthous Stomatitis ◽  
Cervical Adenitis ◽  
Operative Complications ◽  
Pfapa Syndrome ◽  
Almost All ◽  
Post Operative Complications

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by periodic high fever (febrile attacks) lasting for 3–6 days and recurring regularly. In this syndrome, fever is associated with aphthous stomatitis, pharyngitis, and cervical adenitis.1–4 Almost all patients with PFAPA syndrome spontaneously resolve approximately 8 years after the onset of symptoms. However, the cause and etiology of PFAPA syndrome remain unclear.4 Oral prednisolone reduces fever during febrile attacks.5–7 Tonsillectomy has also been reported to induce remission of PFAPA syndrome.8–12 However, tonsillectomy is not a popular treatment for PFAPA syndrome because its effectiveness is still unclear. Furthermore, tonsillectomy is generally recommended for patients aged 3 years and older to avoid post-operative complications.13 We report here on four patients aged 3 years and older with PFAPA syndrome. Tonsillectomy completely eliminated febrile attacks and was safely performed without post-operative complications in all of our patients.  

scholarly journals THU0019 INTRON REGIONS OF PIK3AP1 (BCAP) AND SPON2 (SPONDIN-2) GENES ARE DIFFERENTIALLY METHYLATED IN PATIENTS WITH PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND ADENITIS (PFAPA) SYNDROME

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 223.3-223
Author(s):  
E. Lovšin ◽  
J. Kovac ◽  
T. Tesovnik ◽  
N. Toplak ◽  
D. Perko ◽  
...  
Keyword(s):  
Dna Methylation ◽  
Pattern Recognition ◽  
Periodic Fever ◽  
Aphthous Stomatitis ◽  
Extracellular Matrix Protein ◽  
Inflammasome Activation ◽  
Cervical Adenitis ◽  
Pfapa Syndrome ◽  
Pattern Recognition Molecule ◽  
Recognition Molecule

Background:Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is the most common autoinflammatory disease in children, often grouped together with hereditary periodic fever syndromes, although its cause and hereditary nature remain unexplained.Objectives:We investigated whether a differential DNA methylation was present in DNA from peripheral blood mononuclear cells (PBMC) in patients with PFAPA versus a group of healthy young individuals.Methods:A whole epigenome analysis (MeDIP and MBD) was performed using pooled DNA libraries enriched for methylated genomic regions. Of identified candidate genes, two with most significantly different methylation leves were further evaluated with methylation specific restriction enzymes coupled with qPCR (MSRE-qPCR).Results:The analysis showed thatPIK3AP1andSPON2intronic gene regions are differentially methylated in patients with PFAPA. MSRE-qPCR proved as a quick, reliable and cost-effective method to confirm results from MeDIP and MBD.Conclusion:Our findings indicate that B cell adapter protein (BCAP) as PI3K binding inhibitor of inflammation and spondin-2 (SPON2) as a pattern recognition molecule and integrin ligand could play a role in etiology of PFAPA. Their role and impact of changed DNA methylation in PFAPA etiology and autoinflammation need further investigation.References:[1]Wekell P. Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome – PFAPA syndrome. Press Medicale [Internet]. 2019;48(1):e77–87. Available from:https://doi.org/10.1016/j.lpm.2018.08.016[2]K. Theodoropoulou, F. Vanoni, and M. Hofer, “Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome: a Review of the Pathogenesis,”Curr. Rheumatol. Rep., vol. 18:18, 2016.[3]Carpentier SJ, Ni M, Duggan JM, James RG, Cookson BT, Hamerman JA. The signaling adaptor BCAP inhibits NLRP3 and NLRC4 inflammasome activation in macrophages through interactions with Flightless-1. Sci Signal. 2019;12(581).[4]He YW, Li H, Zhang J, Hsu CL, Lin E, Zhang N, et al. The extracellular matrix protein mindin is a pattern-recognition molecule for microbial pathogens. Nat Immunol. 2004;5(1):88–97.Disclosure of Interests:None declared

scholarly journals Management of Fever Attack in a Patient with PFAPA Syndrome

2021 ◽  
Vol 3 (4) ◽  
pp. 01-02
Author(s):  
Muhammet ENGİN
Keyword(s):  
Case Report ◽  
Infectious Diseases ◽  
Periodic Fever ◽  
Aphthous Stomatitis ◽  
Common Symptom ◽  
Periodic Fever Syndrome ◽  
Cervical Adenitis ◽  
Pfapa Syndrome ◽  
Fever Attack

Fever is a common symptom in childhood and primarily suggests infectious diseases. The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) is a periodic fever syndrome of unknown incidence and is not rare in childhood. In this case report, the management of a patient who was followed up for PFAPA syndrome during a fever attack is presented.

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