De Cunto Carmen, Britos María, Eymann Alfredo, Deltetto Noelia, Liberatore Diana
Hospital Italiano de Buenos Aires, Argentina.
Arch Argent Pediatr. 2010 Oct;108(5):445-8. doi: 10.1590/S0325-00752010000500011.
PFAPA is a periodic fever syndrome characterized by: fever, aphthous stomatitis, pharyngitis and cervical adenitis. It is one of the autoinflammatory syndromes, but yet of unknown etiology. Our aim is to report our experience, describe clinical manifestations, laboratory findings, relapses occurrence and response to treatment. We present 12 PFAPA patients. Median age at onset: 1.15 years, mean duration of febrile episodes: 4 days, and relapses at regular intervals. All children received prednisone (1-2 mg/kg/dose) one to two doses, 9 patients responded immediately after the first dose and all experienced a lower periodicity of attacks. PFAPA is the most frequent periodic fever syndrome. To our knowledge, there are no other local series of PFAPA patients published. Recognizing this syndrome will prevent from ordering unnecessary studies and will favor family coping.
PFAPA是一种周期性发热综合征,其特征为:发热、阿弗他口炎、咽炎和颈部腺炎。它是自身炎症性综合征之一,但病因不明。我们的目的是报告我们的经验,描述临床表现、实验室检查结果、复发情况及对治疗的反应。我们呈现了12例PFAPA患者。发病时的中位年龄为1.15岁,发热发作的平均持续时间为4天,且有规律地复发。所有儿童均接受了泼尼松(1 - 2毫克/千克/剂量)1至2剂治疗,9例患者在第一剂后立即有反应,且所有患者发作的周期性均降低。PFAPA是最常见的周期性发热综合征。据我们所知,尚未发表其他关于PFAPA患者的本地系列研究。认识到这种综合征将避免进行不必要的检查,并有利于家庭应对。