PFAPAPeriodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy
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From our institution, we identified 27 patients (12 female and 15 male) with clinical signs of PFS excluding those with classical PFAPA. Genetic screening showed 15 patients testing positive for a PFS gene and 12 patients testing negative for a PFS gene (figure 1).
Other oral diseases: Allergic contact stomatitis, Geographic Language, PFAPA syndrome.
Classical presentation of PFAPA is recurrent episodes of fever, which occurs at intervals of 3 to 5 weeks associated with one of the following cardinal signs: aphthous ulceration, stomatitis, tonsillitis, and cervical adenitis [3, 4].
Moradinejad, "Periodic fever, Aphthous stomatitis, Pharyngitis and cervical adenitis (PFAPA) syndrome in Iranian children: First report of iranian periodic fever and autoinflammatory registry (IPFAIR)," Iranian Journal of Pediatrics, vol.
After many unsuccessful treatments (like 2 weeks of antibiotics and steroids) and countless blood tests and diagnostic tests, we were referred to infectious disease specialist in the best children hospital in our region in NY where he was diagnosed with PFAPA syndrome.
We assessed CRP and PCT values in PFAPA patients during their febrile episodes and in two control groups consisting of children with various bacterial or viral infections.
Among stereotypical recurrent fevers, irregular episodes likely indicate a monogenic fever syndrome, while more predictable episodes could be cyclic neutropenia or PFAPA.
The syndrome is called periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis -- or PFAPA -- and is characterized by monthly flare-ups of fever, accompanied by sore throat, swollen glands and mouth lesions.
The most common treatments for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) include steroids, cimetidine, and tonsillectomy and adenoidectomy, said Dr.