Tasher D, Somekh E, Dalal I
Department of Pediatrics, E Wolfson Medical Center, Holon, Israel.
Arch Dis Child. 2006 Dec;91(12):981-4. doi: 10.1136/adc.2005.084731. Epub 2006 Apr 4.
The recently described PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis) syndrome is characterised by periodic fever, aphthous stomatitis, pharyngitis and adenitis. However, there are currently relatively few data on the natural history of this syndrome.
To describe the presentation, clinical course, doctors' awareness, therapeutic response and long-term follow-up of children with PFAPA syndrome.
Children with PFAPA syndrome referred over a 5-year period (from January 1999 to January 2004) were enrolled in the study. Data were gathered from medical records, parents' interviews, physical examination and telephone calls.
54 patients with PFAPA syndrome were evaluated. Our patients had a higher rate of abdominal pain (65%) and a lower rate of aphthous stomatitis (39%) than those in previous reports. Four different patterns of disease evolution were identified, including the relatively common (n = 14, 26%) and newly described course of alternating remissions and relapses. The remissions lasted 8.5 months on average (range 4-36 months). Diagnosis was established by primary paediatricians in 30 of 54 (56%) patients. However, a substantial delay in diagnosis was apparent (mean 15 months). Episodes were curtailed by a much lower dose of prednisone or equivalent corticosteroid (mean 0.6 mg/kg/day, range 0.15-1.5 mg/kg/day) than reported previously. Tonsillectomy was successful in the prevention of recurrence of further episodes in all six patients who underwent the procedure.
We describe several new characteristics of PFAPA syndrome in children, contributing to our knowledge of this relatively unrecognised but troublesome syndrome. Early diagnosis and appropriate treatment can markedly improve the quality of life of both patients and families.
最近描述的PFAPA（周期性发热、阿弗他口炎、咽炎和腺炎）综合征的特征为周期性发热、阿弗他口炎、咽炎和腺炎。然而，目前关于该综合征自然病史的数据相对较少。
描述PFAPA综合征患儿的临床表现、临床病程、医生的认知、治疗反应及长期随访情况。
纳入5年期间（1999年1月至2004年1月）转诊的PFAPA综合征患儿。数据收集自病历、家长访谈、体格检查及电话随访。
对54例PFAPA综合征患者进行了评估。与既往报道相比，我们的患者腹痛发生率较高（65%），阿弗他口炎发生率较低（39%）。确定了四种不同的疾病演变模式，包括相对常见的（n = 14，26%）以及新描述的缓解与复发交替病程。缓解期平均持续8.5个月（范围4 - 36个月）。54例患者中有30例（56%）由初级儿科医生确诊。然而，诊断明显延迟（平均15个月）。与既往报道相比，使用低得多剂量的泼尼松或等效皮质类固醇（平均0.6 mg/kg/天，范围0.15 - 1.5 mg/kg/天）即可缩短发作期。扁桃体切除术对所有6例接受该手术的患者预防进一步发作均成功。
我们描述了儿童PFAPA综合征的几个新特征，增进了我们对这种相对未被认识但棘手综合征的了解。早期诊断和适当治疗可显著改善患者及其家庭的生活质量。
