评估肥大细胞激活与儿童和青少年体位性正位性心动过速综合征之间的关系。

Northern clinics of Istanbul Pub Date : 2024-08-02 eCollection Date: 2024-01-01 DOI:10.14744/nci.2023.64920
Yunus Emre Bayrak, Ozlem Kayabey, Evic Zeynep Basar, Isil Eser Simsek, Metin Aydogan, Abdulkadir Babaoglu
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引用次数: 0

摘要

目的:体位性正位性心动过速综合征(POTS)是青少年常见的正位性不耐受综合征之一,会损害生活质量。POTS 是一种多系统疾病。POTS 的病因有多种机制,如自主神经失神经支配、低血容量、肾上腺素能亢进、低状态和过度警觉。最近,肥大细胞激活(MCA)也被提上了病因学的议事日程。关于肥大细胞激活与成年期 POTS 之间关系的文献研究很少。然而,有关儿童和青少年的数据却很有限。有鉴于此,我们旨在通过测量血清胰蛋白酶水平(MCA 的特异性标志物)来评估 POTS 与 MCA 之间的关系:这项前瞻性研究纳入了 2018 年 11 月至 2019 年 8 月期间因晕厥-阵发性晕厥入住科贾埃利大学医学院附属医院儿科心脏病学门诊的患者。接受TILT-table测试的患者被纳入研究。患有结构性心脏病或慢性心脏病的患者未纳入本研究。所有患者在接受TILT-table检测前均获得了血清胰蛋白酶水平,在检测终止后,对TILT-table检测呈阳性的POTS患者的血清胰蛋白酶水平进行了重新研究。确诊为 POTS 的患者被划分为第一组,其他患者被划分为第二组:在 58 名参与研究的患者中,有 28 名(平均年龄为 14.4±2.0 岁;38 名女孩,20 名男孩)被确诊为 POTS。POTS患者在测试过程中的平均心率增加率为38±6次/分(47.05%±15.65%)。组间基础血清胰蛋白酶水平无差异(分别为3.2±1.3 ng/ml和3.84±1.78 ng/ml;P=0.129),而出现MCA相关症状的患者血清胰蛋白酶水平(基线和TILT-table测试45-60分钟后)高于其他患者:在文献中,MCA 被认为是导致 POTS 的机制之一。结论:在文献中,MCA 被认为是导致 POTS 的机制之一,尽管其他机制,如神经病理性和低血容量性 POTS,可能在患者中也很活跃,但这些患者的 MCA 症状应受到常规质疑。
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Evaluation of the relationship between mast cell activation and postural orthostatic tachycardia syndrome in children and adolescents.

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.

Conclusion: 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.

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