探索在患有PFAPA的儿童中预测colchicine反应的因素
Zeynep Özaslan1, Abdulvahap Şen2, Sıla Atamyıldız Uçar3
1Department of Pediatric Rheumatology, Faculty of Medicine, MD, Kocaeli University, Kocaeli, Turkey. zeynepgozaluludag@gmail.com.
European journal of pediatrics
|July 31, 2024
概括
在周期性发烧,口腔口炎,耳炎和宫腺炎综合征 (PFAPA) 中预测素反应至关重要. 喉炎,关节炎和频繁的发作预测不响应,而M694V变种预测响应.
科学领域:
- 儿科风湿病学 儿科风湿病学
- 自燃性炎症疾病 自燃性炎症疾病
- 遗传学 是一个遗传学.
背景情况:
- 周期性发烧,口腔口炎,耳炎和宫腺炎综合征 (PFAPA) 是儿童最常见的自身炎症性疾病.
- 胆固醇是PFAPA的常见治疗方法,而MEFV基因变异是已知的反应预测因子.
- 然而,其他影响胆固醇反应的临床和实验室参数需要进一步调查.
研究的目的:
- 确定临床和实验室参数,预测儿科PFAPA患者的胆固醇反应.
- 为了根据PFAPA患者对colchicine治疗的反应进行比较.
主要方法:
- 一项回顾性,多中心,横截面研究,涉及806名儿科患者,在9个类风湿中心诊断出PFAPA.
- 对患者进行了临床发现,攻击频率和MEFV基因变异 (包括M694V) 的分析.
- 进行了统计分析,以将临床/实验室参数与素反应相关联.
主要成果:
- 科尔奇辛治疗给了64.4%的患者,其中80.7%的患者表现出良好的反应.
- 喉炎,关节炎和发作频率增加与素不响应相关 (p<0.05).
- 携带M694V变种与素反应显著相关 (p=0.001).
结论:
- 喉炎,关节炎和更高的发作频率预测了PFAPA中对菌素的反应不佳.
- 在PFAPA中,M694V变异是成功治疗菌素的关键预测因素.
- 早期预测治疗反应可以优化PFAPA管理.
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