نتایج جستجو برای: pfapa
تعداد نتایج: 261 فیلتر نتایج به سال:
BACKGROUND The pathogenesis of PFAPA (periodic fever, aphthous stomatitis, pharyngitis, adenitis) syndrome is unknown as yet. In order to understand whether genes implicated in other auto-inflammatory diseases might be involved in the pathogenesis of PFAPA, all variants in the genes causing familial Mediterranean fever (FMF), tumor necrosis factor (TNF) receptor-associated periodic syndrome (TR...
Objectives. To determine whether PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) patients have a positive family history (FH) for recurrent fever syndromes. Method. For all patients with PFAPA seen in two paediatric rheumatology centres (Romandy, Switzerland and Bordeaux, France), parents were interviewed to record the FH for periodic fever. As controls, we interv...
Methods PFAPA patients were prospectively recruited. All patients had genetic testing to exclude mutations in the known fever genes (MVK, MEFV, TNFRSF1A, NLRP3, and ELA2). These PFAPA patients have been classified as sporadic or familial cases based on family history. Familial cases included those with a family member having PFAPA or a family member having a feature of PFAPA (recurrent fever, o...
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...
AIMS We describe the presentations and clinical outcomes of pediatric patients diagnosed with PFAPA (Periodic Fever, Aphthous lesions, Pharyngitis, and cervical Adenitis). MATERIALS AND METHODS The medical records of children with recurrent fever and referred between 1998 and 2007 to a tertiary pediatric care hospital were reviewed. Children who met clinical criteria for PFAPA were then asked...
Methods Serum of three controls (CTRL) or PFAPA patients collected during (PFAPA-IN) and between (PFAPA-OUT) flares were incubated with monocytes isolated from healthy volunteers (n=3) and stimulated with ultra pure lipopolysaccharide (LPSup). IL-1b, TNF-a and IL-6 levels were measured by ELISA comparing serum stimulation alone and the impact of serum pre-incubation on LPSup induced cytokines.
BACKGROUND PFAPA syndrome is a chronic disease that is characterized by recurrent episodes of high fever, aphthous stomatitis, pharyngitis, and cervical adenitis. Knowledge regarding the etiology of PFAPA is limited. OBJECTIVES To provide up-to-date information considering etiology of PFAPA syndrome, by summarizing what has been explored and established in this area so far. MATERIALS AND ME...
Results During the asymptomatic phase the number of monocytes did not differ between the PFAPA and control tonsils. We observed a considerable recruitment of NK cells in tonsils of PFAPA patients with respect to CG. Surprisingly, we have detected a significant expansion of both CD56CD16 and CD56CD16NK cell subsets when compared to CG. Strict characterization of activating and inhibitory NK rece...
Background Tonsillectomy (TE) or adenotonsillectomy (ATE) may have a beneficial effect on the clinical course in children with the Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis (PFAPA) syndrome. However, an immunological reason for this effect remains unknown. This literature review summarizes the current knowledge of the effect of TE or ATE in the PFAPA syndrome. Met...
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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