Demographics of a large paediatric Postural Orthostatic Tachycardia Syndrome Program
Demographics of a large paediatric Postural Orthostatic Tachycardia Syndrome Program
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DOI：
10.1017/s1047951117002888
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发表时间：
2018-05-01
影响因子：
1
通讯作者：
Bernadzikowski, Thomas
中科院分区：
文献类型：
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作者：
Boris, Jeffrey R.;Bernadzikowski, Thomas
目的：本研究的目的是确定和评估三级医院专科门诊中患有体位性心动过速综合征的儿童患者的人口统计学和临床特征。方法：这是一项对初始门诊评估期间获得的临床数据的回顾性分析。结果：共有708例患者符合评价标准。女性患者多于男性，为3.45：1。白人占患者的94.1%。诊断时的中位年龄为15.7岁。57.3%的患者发生关节过度活动; 22.4%的患者患有过度活动性Ehlers-Danlos综合征; 34.9%的患者患有过度活动性谱系障碍。出现症状的中位年龄为12.6岁的患者与13.7岁的那些没有过度活动（p = 0.0001）。症状的中位持续时间为3.3年，与1.5年没有过度活动（p < 0.00001）。假定的触发因素包括23.6%的患者感染，11.4%的患者脑震荡，2.8%的患者手术/创伤。在5.2%的患者中观察到并发炎性疾病。六种症状占患者初始投诉的80%。总体而言，66%的患者随后出现至少10种症状，50%的患者出现至少14种症状，30%的患者报告至少26种症状。症状主要是心血管、胃肠道和神经系统。在一家大型专科诊所中见到的体位性直立性心动过速综合征的儿科患者主要是女性，大多数是白人，在青春期早期出现症状，在诊断前症状持续两年以上。超过一半的患者有关节过度活动。超过三分之一的患者可能有自身免疫或炎症触发因素，包括感染、脑震荡或手术/创伤。患者在疾病过程中经历高度可变和多系统起源的症状。
Objective: The aim of this study was to identify and evaluate demographic and clinical features of paediatric patients with postural orthostatic tachycardia syndrome in a tertiary hospital speciality clinic. Method: This is a retrospective review of clinical data obtained during initial outpatient evaluation. Results: A total of 708 patients met the evaluation criteria. Female patients outnumbered males, 3.45:1. Caucasians were over-represented at 94.1% of patients. Median age at diagnosis was 15.7 years. Joint hypermobility occurred in 57.3% of patients; 22.4% had hypermobile Ehlers-Danlos syndrome; and 34.9% had hypermobility spectrum disorder. Median age of onset of symptoms was 12.6 years in patients with hypermobility versus 13.7 years in those without (p = 0.0001). Median duration of symptoms was 3.3 years with hypermobility versus 1.5 years without (p < 0.00001). Putative triggers included infection in 23.6% of patients, concussion in 11.4%, and surgery/trauma in 2.8%. Concurrent inflammatory disorders were noted in 5.2% of patients. Six symptoms comprised 80% of initial patient complaints. Overall, 66% of patients subsequently had at least 10 symptoms, 50% had at least 14 symptoms, and 30% reported at least 26 symptoms. Symptoms were largely cardiovascular, gastrointestinal, and neurological. Paediatric patients with postural orthostatic tachycardia syndrome seen in a large speciality clinic are predominantly female, are mostly Caucasian, have onset of symptoms in early adolescence, and have symptoms for over two years before diagnosis. Over half of patients have joint hypermobility. More than one-third of patients have a possible autoimmune or inflammatory trigger, including infection, concussion, or surgery/trauma. Patients experience symptoms that are highly variable and multi-system in origin over the course of illness.