对患有双致病原体外型10变体的儿科FMF患者的评估:专注于胸痛
Eray Tunce1, Sıla Atamyıldız Uçar1, Ekin İlayda Çağlar2
1Department of Pediatric Rheumatology, Ümraniye Training and Research Hospital, University of Health Sciences, İstanbul, Türkiye.
European journal of pediatrics
|February 11, 2026
概括
胸部疼痛是儿科家族地中海热 (FMF) 中常见的一种肺炎症状. 患有胸痛的患者经历了更频繁的发作和更高的胆固醇耐药性,特别是M694V同胞菌.
科学领域:
- 儿科风湿病学 儿科风湿病学
- 单源性自身炎症性疾病 单源性自身炎症性疾病
- 遗传学 遗传学是一种遗传学.
背景情况:
- 胸痛是已知的家族地中海热 (FMF) 的表现,这是一个常见的单一性自身炎症性疾病.
- 在FMF中M694V同卵性基因型与更严重的炎症表型有关.
研究的目的:
- 在一个大规模,基因均的儿科FMF队列中表征胸痛,具有双异构10个MEFV变体.
- 为了比较FMF患者的临床和遗传特征,胸痛和没有胸痛.
主要方法:
- 对918名儿科FMF患者进行横截面,单中心的研究,这些患者具有双样性致病性MEFV外型10个变异.
- 追溯收集人口统计,临床和遗传信息的数据.
- 胸痛和没有胸痛的患者之间的比较分析.
主要成果:
- 胸部疼痛发生在23.7%的患者中,仅限于肺炎.
- 患有胸痛的患者每年发作频率更高,腹部疼痛增加.
- 在患有胸痛的患者中,观察到较高的胆固醇抵抗率 (21.1%) 和M694V/M694V基因型 (p=0.017).
- 在5%的患有胸痛的患者中检测到腹腔溢出,与素耐药性相关.
结论:
- 胸部疼痛是这个儿科FMF队列中常见的,多发性表现.
- 胸部疼痛与增加的疾病活性和胆固醇素耐药性有关,特别是在M694V同胞菌中.
- 胸部疼痛的FMF患者的多流液表明,抗素耐药性的可能性更高.
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