Methods Children diagnosed with PFAPA were prospectively recruited from a large, tertiary hospital in Boston. Diagnosis was made by pediatric rheumatologists, otolaryngologists, or infectious diseases specialists with expertise in PFAPA. Clinical history was gathered at diagnosis, and laboratory testing was performed during flares. Years after diagnosis, we performed a retrospective chart revie...

OBJECTIVES To assess the levels of procalcitonin (PCT) and C-reactive protein (CRP) in children diagnosed with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) during their febrile attacks. METHODS 23 patients with diagnosis of PFAPA included into the study prospectively during a three years period. In these patients, CRP and PCT values were recorded during 78 f...

Adult-onset periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is rare. We report the case of a 23-year-old woman with PFAPA syndrome in Japan. Her symptoms lasted for approximately 5 days in each period regardless of nonsteroidal anti-inflammatory drug or antibiotic treatment. She was diagnosed with PFAPA syndrome based on her periodic self-limiting symptoms and no...

OBJECTIVE To assess the benefits of adenotonsillectomy in the treatment of pediatric patients with PFAPA (periodic fever, aphthous ulcers, pharyngitis, and adenitis) syndrome. DESIGN Prospective case series. SETTING Tertiary care pediatric hospital. PATIENTS Pediatric patients meeting criteria for PFAPA syndrome. INTERVENTION Tonsillectomy with or without adenoidectomy. MAIN OUTCOME M...

Periodic fevers (fevers that occur predictably at fixed intervals) are unusual in infants and children. The classic periodic fever syndrome is cyclic neutropenia (neutropenia followed by infections and fever that recur every 21 days). A new periodic fever syndrome PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) has been characterized over the past decade. PFAPA i...

PURPOSE This study aimed to evaluate (a) specific histologic findings in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome who had tonsillectomy and (b) to assess any improvement of symptoms after tonsillectomy with or without adenoidectomy. MATERIALS AND METHODS This project is a retrospective study performed at "Aghia Sophia" Children's H...

PFAPA syndrome is the most common autoinflammatory disorder in childhood with unknown etiology. The aim of our study was clinical evaluation of PFAPA patients from a single tertiary care center and to determine whether variations of AIM2, MEFV, NLRP3, and MVK genes are involved in PFAPA pathogenesis. Clinical and laboratory data of consecutive patients with PFAPA syndrome followed up at the Uni...

Results Statistical analysis with the use of C4.5 algorithm resulted in the following decision tree: viral infection if CRP≤19.1 mg/L; otherwise for cases with CRP>19.1 mg/L: PFAPA if PCT≤0.65 ng/mL, bacterial infection if PCT > 0.65 ng/mL. The rule was effective in 83.7% of the cases. Febrile episodes during PFAPA flares, bacterial and viral infections were classified with the sensitivity of 7...

INTRODUCTION The role of tonsillectomy in the periodic fever, aphthous stomatitis, pharyngitis, and adenitis, PFAPA syndrome, is controversial. Although some studies reported high success rates with tonsillectomy, further investigations are needed with larger numbers of patients. OBJECTIVE To seek the long-term outcomes of tonsillectomy in PFAPA syndrome. METHODS Case series; multi-center s...

OBJECTIVE The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a nonhereditary idiopathic febrile syndrome belonging to the group of autoinflammatory diseases. No longtime sequel was reported in this disease. Early diagnosis can lead physicians to treatment of this disorder with a short course steroid application and provide satisfaction of the patient'...