Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome
Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome
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DOI：
10.1186/1471-2431-10-65
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发表时间：
2010-09-06
期刊：
影响因子：
2.4
通讯作者：
Berg, Stefan
中科院分区：
文献类型：
--
作者：
Brown, Kelly L.;Wekell, Per;Berg, Stefan
背景资料：本研究的目的是配置文件的血细胞和血清细胞因子水平在无发热和发热阶段的周期性发热，口疮性口炎，咽炎和腺炎（PFAPA）综合征，以推进病理生理学的理解，这儿科diseases.Methods：一个队列的患者中位年龄为4.9岁，经历了“典型的PFAPA”事件参加了这项研究。血细胞和血清细胞因子进行了分析，CBC分析和多重ELISA.Results：在典型PFAPA综合征的发热和发热阶段的血细胞浓度的振荡进行了观察，新的发现包括增加单核细胞和减少嗜酸性粒细胞在发热发作和增加血小板在发热间隔。血清中存在相对适度水平的促炎细胞因子。IFN γ诱导的细胞因子IP 10/CXCL 10增加发热后发病，而T细胞相关的细胞因子IL 7和IL 17被抑制在无发热和发热periods.Conclusions：鉴定失调的血细胞和血清细胞因子是一个初步的步骤，对PFAPA疾病和/或球员的疾病发病机制的生物标志物的鉴定。未来的研究需要最终确定哪些介质与PFAPA综合征特异性相关。
Background: This study aimed to profile levels of blood cells and serum cytokines during afebrile and febrile phases of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome to advance pathophysiological understanding of this pediatric disease.Methods: A cohort of patients with a median age of 4.9 years experiencing 'typical PFAPA' episodes participated in this study. Blood cells and serum cytokines were analyzed by CBC analysis and multiplex ELISA.Results: Oscillations in the concentration of blood cells during the afebrile and febrile phases of typical PFAPA syndrome were observed; novel findings include increased monocytes and decreased eosinophils during a febrile episode and increased thrombocytes in the afebrile interval. Relatively modest levels of pro-inflammatory cytokines were present in sera. IFN gamma-induced cytokine IP10/CXCL10 was increased after the onset of fever while T cell-associated cytokines IL7 and IL17 were suppressed during afebrile and febrile periods.Conclusions: Identification of dysregulated blood cells and serum cytokines is an initial step towards the identification of biomarkers of PFAPA disease and/or players in disease pathogenesis. Future investigations are required to conclusively discern which mediators are associated specifically with PFAPA syndrome.