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Minsoo Shin
, Eun Hwa Choi, Mi Seon Han
, Eun Hwa Choi, Mi Seon Han
Abstract
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.
Figures and Tables
Table 1
Clinical manifestations of 13 patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome
Abbreviations: PD, prednisolone; T & A, tonsillectomy and adenoidectomy; CL, cervical lymphadenitis; P, pharyngitis; N, nausea; ITP, idiopathic thrombocytopenic purpura; AP, abdominal pain; FS, febrile seizure; R, rash; T, tongue change; GN, glomerulonephritis; H, headache.
*Father, mother, and brother had history of adenoid hypertrophy; †Rash on periorbital, cervical, and trunk area; ‡Patient 3 and 7 had strawberry and whitish tongue, respectively.
Table 2
Laboratory findings of 13 patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome at the time of diagnosis
Abbreviations: WBC, white blood cell count; Seg, segmented neutrophil; ANC, absolute neutrophil count; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; Ag, antigen; PCR, polymerase chain reaction; N.D., not done; AdV, adenovirus; RV, rhinovirus; EV, enterovirus.
*At the time of diagnosis, the patient did not have any respiratory symptoms.
Table 3
Clinical manifestations of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in children reported in Korea*
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