주기성 구토증으로 추적 관찰 중 진단된 PFAPA 증후군 1예

Abstract

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.

주기성 구토증 의심 하에 추적 관찰 중 주기적인 발열, 아프타성 구내염, 인두염 및 경부 림프절염 등의 특징적인 임상 증상을 보여 소아에서 주기적 발열을 일으키는 원인들을 증상과 검사실 소견을 통해 배제함으로써 PFAPA 증후군으로 진단된 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.