目的:体位性心动过速综合征(POTS)是一种体位性不耐受综合征,常见于青少年,损害生活质量。POTS是一种多系统的疾病。在POTS病因学中已经定义了许多机制,比如自主神经支配,血容量不足,高肾上腺素能刺激,低条件,和高度警惕。最近,肥大细胞活化(MCA)在病因学中也已提上日程。关于成年期MCA与POTS关系的文献很少。然而,关于儿童和青少年的数据是有限的。根据这些信息,我们旨在通过测量血清类胰蛋白酶水平来评估POTS和MCA之间的关系,MCA的特异性标记。
方法:这项前瞻性研究纳入了2018年11月至2019年8月因晕厥-晕厥前晕厥而在Kocaeli大学医学院附属医院儿科心脏病门诊就诊的患者。接受TILT表测试的患者被纳入研究。本研究不包括结构性心脏病或慢性心脏病患者。在TILT-table测试之前,从所有患者获得血清类胰蛋白酶水平,在POTSTILT表测试阳性的患者终止测试后,重新研究了血清类胰蛋白酶水平。诊断为POTS的患者被归类为第1组,其他患者被归类为第2组。
结果:纳入研究的58例患者中有28例(平均:14.4±2.0岁;38名女孩,20名男孩)被诊断出患有POTS。其余30例患者被诊断为血管迷走性晕厥,并被纳入第2组。在POTS患者中,测试期间平均心率的增加为38±6次/分钟,为47.05%±15.65%。基础血清类胰蛋白酶水平组间无差异(3.2±1.3ng/ml和3.84±1.78ng/ml,分别为;p=0.129),而与其他患者相比,出现与MCA相关症状的患者的血清类胰蛋白酶水平(基线和TILT测试45~60分钟后)更高。
结论:在文献中,MCA被认为是导致POTS的机制之一。虽然其他机制,如神经性和低血容量的POTS,可能是活跃的病人,应常规询问这些患者的MCA症状.
OBJECTIVE: Postural orthostatic tachycardia syndrome (POTS) is one of the orthostatic intolerance syndromes that are common in young adolescents and impair quality of life. POTS is a multi-systemic disease. Many mechanisms have been defined in POTS etiology, such as autonomic denervation, hypovolemia, hyperadrenergic stimulation, low condition, and hypervigilance. Recently, mast cell activation (MCA) has also been on the agenda in etiology. There are few studies in the literature on the relationship between MCA and POTS in adulthood. However, data on children and adolescents is limited. In light of this information, we aimed to evaluate the relationship between POTS and MCA by measuring serum tryptase levels, a specific marker for MCA.
METHODS: This prospective study included patients who were admitted to Kocaeli University Faculty of Medicine Hospital Pediatric Cardiology outpatient clinic for syncope-presyncope between November 2018 and August 2019. Patients who underwent the TILT-table test were enrolled in the study. Patients with structural heart disease or chronic heart disease were not included in this study. Serum tryptase levels were obtained from all patients before the TILT-table test, and serum tryptase levels were re-studied after the test was terminated in patients with positive TILT-table tests for POTS. Patients diagnosed with POTS were classified as Group 1, and other patients were classified as Group 2.
RESULTS: Twenty-eight of the 58 patients included in the study (mean: 14.4±2.0 years; 38 girls, 20 boys) were diagnosed with POTS. The remaining 30 patients were diagnosed with vasovagal syncope and included in Group 2. The increase in mean heart rate during the test was 38±6 beats/min and 47.05%±15.65% in patients with POTS. Basal serum tryptase levels were not different between groups (3.2±1.3 ng/ml and 3.84±1.78 ng/ml, respectively; p=0.129), while serum tryptase levels (both baseline and after 45-60 min of the TILT-table test) were higher in patients presenting with symptoms related to MCA compared to others.
CONCLUSIONS: In the literature, MCA was considered to be one of the mechanisms leading to POTS. Although other mechanisms, such as neuropathic and hypovolemic POTS, may be active in the patients, the symptoms of MCA in these patients should be routinely questioned.