PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) is a childhood syndrome that affects both boys and girls. It causes repeated episodes of fever, mouth sores, sore throat, and swollen lymph nodes. PFAPA usually starts in early childhood between ages 2 and 5. In very rare cases, the syndrome may start in adulthood.

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever condition in children, with most cases appearing by the age of 5. Although PFAPA is generally a self-limited condition, it can have a major impact on a child's quality of life, as well as that of their family.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory disorder with an uncertain origin. PFAPA manifestations occur in the form of regular attacks accompanied by a rise in inflammatory markers.

Three chemokines [IP-10/CXCL10, monokine induced by IFN-γ (MIG/CXCL9), and macrophage inflammatory protein 1β (MIP-1β/CCL4)] that are known T-cell chemoattractants were significantly elevated during PFAPA flares compared with asymptomatic intervals (Fig. 3A), as were

2010年9月6日 · Proposed oscillations in the abundance of blood cell populations and inflammatory cytokines over time (x-axis: h, hours; d, days; wk, weeks) in a typical PFAPA cycle. Pro-inflammatory cytokines in the serum (TNFα, IL1β, IFNγ, IL6) appear early after the onset of fever and are maintained (IL6) or rapidly decline (TNFα, IL1β, IFNγ) as IFNγ ...

2015年9月17日 · Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome belongs to the spectrum of autoinflammatory diseases characterized by spontaneous episodes of inflammation which are not accompanied by the usual hallmarks of autoimmunity—high-titer autoantibodies or autoreactive T-cells .

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strategies and a lack of evidence-based treatment ...

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, adenitis) is a childhood syndrome that affects both boys and girls. It causes repeated episodes of fever, mouth sores, sore throat, and swollen lymph nodes. PFAPA usually starts in early childhood between ages 2 and 5. In very rare cases, the syndrome may start in adulthood.

2020年4月15日 · Results: The PFAPA working group developed CTPs resulting in 4 different treatment arms: 1. Antipyretic, 2. Abortive (corticosteroids), 3. Prophylaxis (colchicine or cimetidine) and 4. Surgical (tonsillectomy). Consensus was obtained among CARRA members for those defining patient characteristics who qualify for participation in the CTP PFAPA study.

2016年6月27日 · PFAPA syndrome is an immune mediated disease characterised by a cytokine dysfunction [3, 5]; moreover, the strong familial clustering suggest a potential genetic origin of the syndrome [6, 7]. The presence of variants in inflammasome related genes, mostly in NLRP3 and MEFV, suggest a possible role of these genes in PFAPA pathogenesis [7–9 ...