Şahin Murat, Kır Mustafa, Makay Balahan, Keskinoğlu Pembe, Bora Elçin, Ünsal Erbil, Ünal Nurettin
Department of Pediatrics, Division of Cardiology, Dokuz Eylül University Faculty of Medicine, Izmir, Turkey.
Department of Pediatrics, Division of Rheumatology, Dokuz Eylül University Hospital, 35340, Balçova, Izmir, Turkey.
Clin Rheumatol. 2016 May;35(5):1237-44. doi: 10.1007/s10067-014-2714-z. Epub 2014 Jun 15.
Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory disease in the world. The long-term effects of subclinical inflammation in FMF are not well recognized. Some studies have suggested that FMF is associated with cardiac autonomic dysfunction in adult FMF patients. The objective of this study was to investigate the cardiac autonomic functions in pediatric FMF patients by using several autonomic tests. Thirty-five patients with FMF and 35 healthy controls were enrolled in this cross-sectional study. Demographic data, disease-specific data, and orthostatic symptoms were recorded. In all participants, 12-lead electrocardiography (ECG), 24 h ambulatory electrocardiographic monitoring, transthoracic echocardiography, treadmill exercise test, and head upright tilt-table (HUTT) test were performed. The heart rate recovery (HRR) indices of the two groups were similar. Also, chronotropic response was similar in both groups. The time-domain parameters of heart rate variability (HRV) were similar in both groups, except mean RR (p = 0.024). Frequencies of ventricular and supraventricular ectopic stimuli were similar in both groups. There were no statistically significant differences between the groups in average QT and average corrected QT interval length, average QT interval dispersion, and average QT corrected dispersion. There was no significant difference between the two groups regarding the ratio of clinical dysautonomic reactions on HUTT. However, we observed a significantly higher rate of dysautonomic reactions on HUTT in patients with exertional leg pain than that in patients without (p = 0.013). When the fractal dimension of time curves were compared, FMF patients exhibited significantly lower diastolic blood pressure parameters than controls in response to HUTT. Cardiovascular autonomic dysfunction in children with FMF is not prominent. Particularly, patients with exertional leg pain are more prone to have dysautonomic features. Further studies are needed to elucidate the exact mechanisms leading to impaired cardiac autonomic functions in FMF.
家族性地中海热（FMF）是世界上最常见的遗传性自身炎症性疾病。FMF中亚临床炎症的长期影响尚未得到充分认识。一些研究表明，FMF与成年FMF患者的心脏自主神经功能障碍有关。本研究的目的是通过使用多种自主神经测试来调查儿童FMF患者的心脏自主神经功能。35例FMF患者和35名健康对照者纳入了这项横断面研究。记录了人口统计学数据、疾病特异性数据和直立性症状。对所有参与者进行了12导联心电图（ECG）、24小时动态心电图监测、经胸超声心动图、平板运动试验和头高位倾斜试验（HUTT）。两组的心率恢复（HRR）指标相似。此外，两组的变时性反应相似。除平均RR外（p = 0.024），两组心率变异性（HRV）的时域参数相似。两组室性和室上性异位刺激的频率相似。两组在平均QT和平均校正QT间期长度、平均QT间期离散度和平均QT校正离散度方面无统计学显著差异。两组在HUTT上临床自主神经功能障碍反应的发生率无显著差异。然而，我们观察到，与无运动性腿痛的患者相比，有运动性腿痛的患者在HUTT上自主神经功能障碍反应的发生率显著更高（p = 0.013）。当比较时间曲线的分形维数时，FMF患者在HUTT时的舒张压参数显著低于对照组。FMF儿童的心血管自主神经功能障碍并不突出。特别是，有运动性腿痛的患者更容易出现自主神经功能障碍特征。需要进一步研究以阐明导致FMF患者心脏自主神经功能受损的确切机制。
