Аутовоспалительное заболевание – синдром PFAPA: опыт одного Центра

Целью настоящей работы был анализ лабораторных и клинических данных 101 пациента (61 мальчик и 40 девочек) с диагнозом «синдром PFAPA/синдром Маршалла». Возраст манифестации синдрома PFAPA составил от 8 до 36 месяцев (медиана – 18 месяцев). Данное исследование одобрено независимым этическим комитетом и утверждено решением ученого совета НМИЦ ДГОИ им. Дмитрия Рогачева. В большинстве случаев клинические проявления включали рецидивирующие лихорадки, признаки острого тонзиллита, у 84% пациентов с налетом на миндалинах, шейным лимфаденитом. В меньшем количестве встречались такие клинические проявления, как диарея, рвота, миалгия, артралгия, сыпь. Все пациенты соответствовали критериям G.S. Marshall. Наш опыт ведения пациентов с синдромом PFAPA складывается из симптоматической терапии жаропонижающими препаратами, кратковременной терапии глюкокортикостероидами и тонзиллэктомии. В большинстве случаев к 7 годам наступает полное разрешение клинических проявлений синдрома PFAPA, независимо от варианта проводимой терапии.

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