Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is an autoimmune disorder and the most common disorder characterized by periodic fever in children. A male patient has been followed ...Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is an autoimmune disorder and the most common disorder characterized by periodic fever in children. A male patient has been followed up from 11 months to 10 years of age because of repeated high fever. We prescribed antifebriles and antibiotics for each febrile episode without a beneficial effect to him each time. He required several days of rest at home to recover from each episode. During his most recent febrile episode at 10 years of age, we prescribed 5.0 g of shinbuto (TJ-30;Tsumura Co., Tokyo, Japan). His fever resolved the next day and he was able to attend school. His growth and development are normal. Shinbuto had a therapeutic effect in this patient with PFAPA syndrome and may be useful in this condition.展开更多
Periodic fever,aphthous stomatitis,pharyngitis,and adenitis(PFAPA)syndrome is the most common periodic fever condition in children,with most cases appearing by the age of 5.Although PFAPA is generally a self-limited c...Periodic fever,aphthous stomatitis,pharyngitis,and adenitis(PFAPA)syndrome is the most common periodic fever condition in children,with most cases appearing by the age of 5.Although PFAPA is generally a self-limited condition,it can have a major impact on a child’s quality of life,as well as that of their family.Recent research has continued to shed light on the genetic and immunologic factors that play a role in the pathogenesis of PFAPA.There also exists significant heterogeneity in treatment strategies,and progress has been made to develop evidence-based management strategies and establish a standard of care.This review will outline current knowledge regarding the pathogenesis of PFAPA,as well as treatment strategies and our clinical experience.展开更多
周期性发热-阿弗他口炎-咽炎-淋巴结炎(periodic fever,aphthous stomatitis,pharyngitis and adenitis,PFAPA)综合征通常被认为是一种具有复合病因和异质遗传的自身炎症性疾病,是儿童周期性发热最常见的原因。迄今为止,已经探究了许多...周期性发热-阿弗他口炎-咽炎-淋巴结炎(periodic fever,aphthous stomatitis,pharyngitis and adenitis,PFAPA)综合征通常被认为是一种具有复合病因和异质遗传的自身炎症性疾病,是儿童周期性发热最常见的原因。迄今为止,已经探究了许多可能的病因,包括感染因素、免疫机制和遗传易患性,但确切的病因仍不清楚。同时由于缺乏规范的诊疗标准,致使许多患儿接受了不必要的检查及不合理的治疗。该文旨在从临床特点、发病机制、诊断、鉴别诊断及治疗等方面详细分析PFAPA综合征,以期制定更具体的诊断标准及更规范的治疗方案,使PFAPA综合征患儿得到及时、准确的诊断并接受合理的治疗。展开更多
文摘Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is an autoimmune disorder and the most common disorder characterized by periodic fever in children. A male patient has been followed up from 11 months to 10 years of age because of repeated high fever. We prescribed antifebriles and antibiotics for each febrile episode without a beneficial effect to him each time. He required several days of rest at home to recover from each episode. During his most recent febrile episode at 10 years of age, we prescribed 5.0 g of shinbuto (TJ-30;Tsumura Co., Tokyo, Japan). His fever resolved the next day and he was able to attend school. His growth and development are normal. Shinbuto had a therapeutic effect in this patient with PFAPA syndrome and may be useful in this condition.
文摘Periodic fever,aphthous stomatitis,pharyngitis,and adenitis(PFAPA)syndrome is the most common periodic fever condition in children,with most cases appearing by the age of 5.Although PFAPA is generally a self-limited condition,it can have a major impact on a child’s quality of life,as well as that of their family.Recent research has continued to shed light on the genetic and immunologic factors that play a role in the pathogenesis of PFAPA.There also exists significant heterogeneity in treatment strategies,and progress has been made to develop evidence-based management strategies and establish a standard of care.This review will outline current knowledge regarding the pathogenesis of PFAPA,as well as treatment strategies and our clinical experience.
文摘周期性发热-阿弗他口炎-咽炎-淋巴结炎(periodic fever,aphthous stomatitis,pharyngitis and adenitis,PFAPA)综合征通常被认为是一种具有复合病因和异质遗传的自身炎症性疾病,是儿童周期性发热最常见的原因。迄今为止,已经探究了许多可能的病因,包括感染因素、免疫机制和遗传易患性,但确切的病因仍不清楚。同时由于缺乏规范的诊疗标准,致使许多患儿接受了不必要的检查及不合理的治疗。该文旨在从临床特点、发病机制、诊断、鉴别诊断及治疗等方面详细分析PFAPA综合征,以期制定更具体的诊断标准及更规范的治疗方案,使PFAPA综合征患儿得到及时、准确的诊断并接受合理的治疗。
