Pediatric Infection and Vaccine

The Korean Society of Pediatric Infectious Diseases (대한소아감염학회)
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Clinical Manifestations of PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) Syndrome from a Single Center

단일기관에서 진단한 PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) 증후군의 임상양상

  • Shin, Minsoo (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Choi, Eun Hwa (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Han, Mi Seon (Department of Pediatrics, Seoul National University Children's Hospital)
  • 신민수 (서울대학교 어린이병원 소아청소년과) ;
  • 최은화 (서울대학교 어린이병원 소아청소년과) ;
  • 한미선 (서울대학교 어린이병원 소아청소년과)
  • Received : 2019.06.04
  • Accepted : 2019.09.17
  • Published : 2019.12.25
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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. 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.

목적: PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) 증후군은 소아 주기적 발열의 가장 흔한 원인이다. 본 연구에서는 단일기관에서 진단한 소아 PFAPA 증후군 환자들의 임상양상을 기술하고자 한다. 방법: 2011년 1월부터 2018년 12월까지 서울대학교 어린이병원에서 PFAPA 증후군으로 진단된 소아 13명이 연구에 포함되었다. 후향적으로 의무기록을 검토하여 환자들의 임상양상을 분석하였다. 결과: 13명의 소아 PFAPA 증후군 환자들 중 남자는 8명(61.5%)이었다. 환자들을 중앙값 3.3년(범위, 10개월-8.3년) 동안 추적 관찰하였다. 환자들은 중앙값 3세(범위, 1-6세)에 주기성 발열이 시작되었고 PFAPA 증후군으로 진단이 되기 전에 최소 5회의 주기성 발열을 경험하면서 경구 항생제로 치료받았다. 발열은 중앙값 3.9주의 간격으로 중앙값 4.2일 동안 지속되었다. 모든 환자들에서 발열 시 인두염이 동반되었고 12명(92.3%)에서 경부 림프절염이 있었다. 혈액검사를 시행한 12명 모두에서 호중구 감소는 관찰되지 않았다. C-반응 단백과 적혈구 침강속도는 각각 중앙값 4.5 mg/dL (범위, 0.4-13.2 mg/dL)와 29 mm/hr (범위, 16-49 mm/hr)로 상승되어 있었다. 인두 도찰 배양 검사에서 정상 상재균만 자랐으며 A군 사슬알균 신속항원 검사는 음성이었다. 9명(69.2%)의 환자가 중앙값 0.8 mg/kg 용량의 경구 프레드니솔론을 투약 받았고 6명(66.7%)의 환자들에서 증상이 수 시간 이내에 호전되었다. 3명(23.1%)의 환자들은 반복되는 발열로 편도 및 아데노이드 절제술을 시행 받았다. 결론: 반복적인 발열과 함께 아프타 구내염이나 인두염, 경부 림프절염이 동반되는 소아에서는 꼭 PFAPA 증후군을 의심하여야 한다. 경구 글루코코티코이드 투약으로 소아 PFAPA 증후군에서 증상을 호전시킬 수 있으며 불필요한 항생제 사용을 줄일 수 있다.

Keywords

References

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