Peripheral blood (PB) concentrations of neutrophil chemoattractant IL-8 and Th1-related cytokines are elevated during febrile episodes in children with periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome
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چکیده
Purpose PFAPA is an autoinflammatory syndrome of childhood characterized by recurrent attacks of fever, aphthous stomatitis, pharyngitis and adenitis. The underlying immune disorder is poorly understood, albeit activation of Th1 related cytokines has been observed. We aimed to compare concentration of proinflammatory cytokines and selected chemokines in PB during and between febrile episodes in children with PFAPA.
منابع مشابه
Elevated levels of CXCL10 in the Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome (PFAPA) during and between febrile episodes; an indication of a persistent activation of the innate immune system
BACKGROUND The Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome (PFAPA) is the most common periodic fever syndrome in childhood. Clinically, PFAPA may resemble autoinflammatory diseases, but the etiology is not fully understood. METHODS We measured inflammatory proteins in plasma and hematologic parameters in children with PFAPA during and between febrile episod...
متن کاملIn search of human proteins and infectious triggers involved in periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome
Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is a non-hereditary autoinflammatory disease, characterized by relatively regular recurrence of febrile episodes of 3-6 days duration, accompanied by aphthous stomatitis, pharyngitis/tonsillitis, and/or cervical adenititis. It is considered to be the most common periodic fever syndrome in Japan. Although no responsib...
متن کاملPFAPA syndrome: a review on treatment and outcome
The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) is the most common cause of periodic fever in childhood. The current pharmacological treatment includes corticosteroids, which usually are efficacious in the management of fever episodes, colchicine, for the prophylaxis of febrile episodes, and other medication for which efficacy has not been...
متن کاملProfile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome
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. ...
متن کاملPeriodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade.
The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common periodic fever disease in children. However, the pathogenesis is unknown. Using a systems biology approach we analyzed blood samples from PFAPA patients whose genetic testing excluded hereditary periodic fevers (HPFs), and from healthy children and pediatric HPF patients. Gene expr...
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