• Pediatric Infection and Vaccine
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• v.14 no.2
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• pp.194-198
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• 2007

The PFAPA syndrome is characterized by periodic fever, malaise, aphthous stomatitis, pharyngitis and cervical lymphadenitis without long-term sequelae. It has been known as one of the cause of periodic fever in children. Because there is no specific laboratory findings, the diagnosis of PFAPA syndrome is not only made by exclusion of diseases which are related to the other causes of periodic fever, but also made by a careful history and characteristic clinical manifestations. The dramatic response to the single oral dose of corticosteroid is unique to this syndrome. The PFAPA syndrome tends to be resolved without any long-term adverse sequelae. We have reported a 3 year old boy who was diagnosed as having PFAPA syndrome by episodes of periodic fever for 3 months and by excluding other febrile diseases. He was treated with oral prednisolone and his symptoms had been improved dramatically.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.991-995
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• 2006

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is one of the causes of periodic fever in pediatrics with unknown etiology. It is characterized by abrupt onset of fever, malaise, aphthous stomatitis, pharyngitis and cervical adenitis without long-term sequelae. Laboratory findings of this sporadic and nonhereditary syndrome are so non-specific that the diagnosis is based on clinical findings. Oral prednisolone is quite effective in controlling the symptoms. We report a case of a 6-year-old girl who was diagnosed as having PFAPA syndrome after 2 years of episodes, by excluding other disease entities with similar clinical features. The patient was treated with oral prednisolone and her symptoms improved dramatically.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.1
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• pp.85-91
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• 2006

PFAPA syndrome is characterized by periodic fevers associated with aphthous stomatitis, pharyngitis, and cervical adenitis and is unusual in infants and children. We report on a case of PFAPA syndrome mimicking cyclic vomiting syndrome in a 42-month-old girl. She had experienced multiple episodes of cyclic vomiting with abdominal pain from age 20 to 30 months. When she was 30 months old, periodic fever with pharyngitis was combined with cyclic vomiting, and when 40 months old, aphthous stomatitis and cervical adenitis were added. These periodic symptoms and signs were not treated with prokinetics or antibiotics. Symptom duration of an episode was 3 days. After cimetidine therapy (150 mg three times daily for 6 months), her febrile and cyclic vomiting episodes ceased. At the time of writing she had not received therapy for 10 months and has remained well without periodic attack.

• Pediatric Infection and Vaccine
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• v.26 no.3
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• pp.179-187
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• 2019

Purpose: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a leading cause of periodic fever in children. This study describes the clinical characteristics of PFAPA syndrome in patients from a single center. Methods: Thirteen children diagnosed with PFAPA syndrome at Seoul National University Children's Hospital were included in this study. Retrospective medical chart reviews were performed. Results: Among the 13 patients, 8 (61.5%) were male. The median follow-up duration was 3.3 years (range, 10 months-8.3 years). The median age of periodic fever onset was 3 years (range, 1-6 years). All patients had at least 5 episodes of periodic fever and pharyngitis, managed with oral antibiotics, before diagnosis. The median occurrence of fever was every 3.9 weeks and lasted for 4.2 days. All patients had pharyngitis and 12 (92.3%) had cervical lymphadenitis. Blood tests were performed for 12 patients, and no patients had neutropenia. Both the C-reactive protein and erythrocyte sedimentation rate were elevated at medians of 4.5 mg/dL (range, 0.4-13.2 mg/dL) and 29 mm/hr (range, 16-49 mm/hr), respectively. Throat swab cultures and rapid streptococcal antigen tests were negative. Nine (69.2%) patients received oral prednisolone at a median dose of 0.8 mg/kg, and in 6 (66.7%) patients, fever resolved within a few hours. Three (23.1%) patients received tonsillectomy and adenoidectomy. Conclusions: PFAPA syndrome should be considered when a child presents with periodic fever along with aphthous stomatitis, pharyngitis, or cervical lymphadenitis. Glucocorticoid administration is effective for fever resolution and can reduce unnecessary use of antibiotics.