患有家族地中海热的儿童的胸痛
Emine Nur Sunar-Yayla1, Pelin Esmeray Şenol1, Deniz Gezgin Yıldırım1
1Division of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Gazi University, Ankara, Türkiye.
The Turkish journal of pediatrics
|January 11, 2024
概括
患有家族地中海热 (FMF) 患者的胸痛经历了较早的症状发作和疾病严重程度的增加. 在这些人中,M694V同卵性突变更为普遍,这表明了不同的临床和遗传特征.
科学领域:
- 遗传学 是一个遗传学.
- 免疫学 免疫学 免疫学
- 儿科 儿科 儿科
背景情况:
- 家庭地中海热 (FMF) 是一种自身炎症性疾病.
- 常见的症状包括发烧,腹痛,胸痛和关节炎.
- FMF是以一种自体相衰退模式遗传的.
研究的目的:
- 为了比较患有胸痛和没有胸痛的儿科FMF患者.
- 为了确定临床,实验室和遗传差异.
- 了解胸痛对FMF表现的影响.
主要方法:
- 对1134名FMF患者 (2006-2022) 的回顾性分析.
- 患者被分为有或没有复发性胸痛的组.
- 人口,临床,治疗和MEFV基因数据的比较.
主要成果:
- 14.3%的患者经历了复发的胸痛.
- 胸痛组的发病年龄较小,FMF家族病史较高,发作频率/持续时间增加.
- 在胸痛组中观察到较高的普拉斯病活性评分,黄素/抗IL-1治疗剂量和M694V同基突变.
结论:
- 在FMF中,反复出现的胸痛与早期发病和更严重的疾病严重程度有关.
- 患有胸痛的患者更有可能有FMF的家族病史.
- 同胞性M694V突变在患有胸痛的FMF患者中更为常见.
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