INTRODUCTION Patients with postural orthostatic tachycardia syndrome (POTS) have been shown to exhibit comorbid joint hypermobility manifested as Ehlers-Danlos syndrome (EDS) or hypermobility spectrum disorder (HSD). The prevalence of EDS and HSD in POTS has been demonstrated in smaller studies combining adult and pediatric patients. We examined a large series of pediatric patients to determine their prevalence in children with POTS. MATERIALS AND METHODS Patients 18 years old, or less, at initial evaluation at our clinic were included. POTS was diagnosed based on at least six months of frequent debilitating symptoms of orthostatic intolerance, plus a consistent heart rate increase of at least 40 beats per minute without orthostatic hypotension on standing test. Patients with a Beighton score of at least 5/9 plus other systemic findings suggestive of EDS were further evaluated in Connective Tissue Disorders clinics. RESULTS There were 362 patients meeting inclusion criteria, of which 82 patients had EDS (22.7%) and 141 patients had HSD (39.0%). Patients with EDS had an earlier median age at symptom onset (12.1 vs. 13.5 years, p=0.004) and longer median symptom duration (2.5 vs. 1.5 years, p=0.0008) compared to patients without hypermobility. CONCLUSIONS Our evaluation of a large series of pediatric patients with POTS revealed that over one-fifth of patients had EDS and over one-third of patients had HSD. The awareness of the prevalence of comorbidities such as hypermobility disorders may help inform providers diagnosing and caring for these patients.

中文翻译：

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儿童体位性直立性心动过速综合征中关节过度活动综合征的患病率

引言 体位性直立性心动过速综合征 (POTS) 患者表现出共病关节过度活动，表现为 Ehlers-Danlos 综合征 (EDS) 或过度活动谱系障碍 (HSD)。POTS 中 EDS 和 HSD 的流行已在成人和儿童患者的小型研究中得到证实。我们检查了大量儿科患者以确定他们在 POTS 儿童中的患病率。材料和方法 包括在我们诊所进行初步评估时 18 岁或以下的患者。POTS 的诊断依据是至少 6 个月的直立性不耐受的频繁衰弱症状，加上持续的心率增加至少每分钟 40 次，在站立试验中没有直立性低血压。结缔组织疾病诊所对 Beighton 评分至少为 5/9 加上其他提示 EDS 的全身表现的患者进行了进一步评估。结果符合纳入标准的患者362例，其中EDS患者82例（22.7%），HSD患者141例（39.0%）。与没有过度活动的患者相比，EDS 患者出现症状的中位年龄更早（12.1 岁与 13.5 岁，p=0.004）和更长的中位症状持续时间（2.5 岁与 1.5 年，p=0.0008）。结论 我们对大量 POTS 儿科患者的评估显示，超过五分之一的患者患有 EDS，超过三分之一的患者患有 HSD。对过度活动障碍等合并症患病率的认识可能有助于为诊断和护理这些患者的提供者提供信息。