Increased Intracellular Oxygen Radical Production in Neutrophils During Febrile Episodes of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome

2013 ◽  
Vol 65 (11) ◽  
pp. 2971-2983 ◽  
Author(s):  
Martina Sundqvist ◽  
Per Wekell ◽  
Veronica Osla ◽  
Johan Bylund ◽  
Karin Christenson ◽  
...  
Keyword(s):  
Periodic Fever ◽  
Oxygen Radical ◽  
Aphthous Stomatitis ◽  
Cervical Adenitis ◽  
Radical Production ◽  
Oxygen Radical Production ◽  
Febrile Episodes

scholarly journals A Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Responsive to Tonsillectomy in Japan

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Kohei Yamahara ◽  
Yuki Egawa ◽  
Kana Lee ◽  
Noriyuki Nakashima ◽  
Satoshi Ikegami
Keyword(s):  
Periodic Fever ◽  
Rare Condition ◽  
Japanese Patients ◽  
Aphthous Stomatitis ◽  
Adult Onset ◽  
Medication Therapy ◽  
Cervical Adenitis ◽  
Old Japanese ◽  
Pfapa Syndrome ◽  
Febrile Episodes

Adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a rare condition, having been reported in only three patients in Japan till date. While almost all pediatric PFAPA patients respond well to tonsillectomy, some European studies have reported that tonsillectomy may be ineffective for adult-onset PFAPA. All the Japanese patients with adult-onset PFAPA had been treated orally so far (cimetidine with or without prednisone), instead of tonsillectomy. We reported a case involving a 37-year-old Japanese man with PFAPA syndrome who presented with a history of febrile episodes associated with pharyngitis, cervical adenitis, and aphthous stomatitis for one year. The patient had been undergoing oral medication therapy without any significant improvement. Tonsillectomy was performed for the patient, and complete resolution of PFAPA was achieved. Our experience suggests that a tonsillectomy is a viable option for the treatment of adult-onset PFAPA.

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

Neutrophil oxygen radical production in pre-eclampsia with HELLP syndrome

2001 ◽  
Vol 99 (2) ◽  
pp. 213-218 ◽  
Author(s):  
Petra L.M Zusterzeel ◽  
Geert J.A Wanten ◽  
Wilbert H.M Peters ◽  
Hans M.W.M Merkus ◽  
Eric A.P Steegers
Keyword(s):  
Hellp Syndrome ◽  
Oxygen Radical ◽  
Radical Production ◽  
Oxygen Radical Production

Inhibitory effects of resveratrol analogs on unopsonized zymosan-induced oxygen radical production

1999 ◽  
Vol 57 (6) ◽  
pp. 705-712 ◽  
Author(s):  
Dae-Sig Jang ◽  
Bo-Seong Kang ◽  
Shi Yong Ryu ◽  
IL-Moo Chang ◽  
Kyung Rak Min ◽  
...  
Keyword(s):  
Oxygen Radical ◽  
Inhibitory Effects ◽  
Radical Production ◽  
Oxygen Radical Production ◽  
Resveratrol Analogs
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