Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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PFAPA 증후군 1례

A case of PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome

  • 채주희 (가톨릭대학교 의과대학 소아과학교실) ;
  • 황아름 (가톨릭대학교 의과대학 소아과학교실) ;
  • 박소현 (가톨릭대학교 의과대학 소아과학교실) ;
  • 서병규 (가톨릭대학교 의과대학 소아과학교실)
  • Chae, Joo Hee (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Hwang, A Rum (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Park, So Hyun (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Suh, Byung Kyu (Department of Pediatrics, College of Medicine, The Catholic University of Korea)
  • 투고 : 2006.03.02
  • 심사 : 2006.05.11
  • 발행 : 2006.09.15
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초록

PFAPA 증후군은 소아 시기의 주기성 발열의 원인의 하나로, 급작스럽게 시작되는 발열과 더불어 아프타 구강 궤양, 인두염과 경부 림프절염이 동반되는 것을 특징으로 하는 질환이다. PFAPA 증후군의 원인은 아직 알려져 있지 않으며, 가족력이 없이 산발적으로 발생한다. 또한 검사실 소견에서도 특징적인 소견은 없다. 진단은 임상적인 소견을 바탕으로 이루어지며, 장기적인 합병증을 남기지는 않는다. 현재까지 알려진 치료로는 경구프레드니솔론이 증상을 조절하는 데 효과적이라고 알려져 있다. 저자들은 2년간의 임상 증상의 반복을 경험하였던 6세 여아에서 다른 질환을 배제한 후 PFAPA 증후군의 진단 요건을 만족하여 경구 프레드니솔론을 사용한 후 증세의 호전을 보인 PFAPA 증후군의 한 예를 보고하고자 한다.

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.

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참고문헌

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