Boris Jeffrey R, Bernadzikowski Thomas
1Division of Cardiology,The Children's Hospital of Philadelphia and The Perelman School of Medicine,University of Pennsylvania,Philadelphia, Pennsylvania,USA.
2Division of Cardiology,The Children's Hospital of Philadelphia,Philadelphia, Pennsylvania,USA.
Cardiol Young. 2018 May;28(5):668-674. doi: 10.1017/S1047951117002888. Epub 2018 Jan 23.
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.
This is a retrospective review of clinical data obtained during initial outpatient evaluation.
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.
本研究旨在识别和评估一家三级医院专科门诊中患有体位性直立性心动过速综合征的儿科患者的人口统计学和临床特征。
这是一项对初次门诊评估期间获得的临床数据的回顾性研究。
共有708名患者符合评估标准。女性患者多于男性，比例为3.45:1。白种人占比过高，占患者总数的94.1%。诊断时的中位年龄为15.7岁。57.3%的患者有关节活动过度；22.4%患有活动过度型埃勒斯-当洛综合征；34.9%患有活动过度谱系障碍。活动过度患者症状出现的中位年龄为12.6岁，无活动过度患者为13.7岁（p=0.0001）。有活动过度患者症状持续的中位时间为3.3年，无活动过度患者为1.5年（p<0.00001）。可能的诱因包括23.6%的患者感染、11.4%的患者脑震荡以及2.8%的患者手术/外伤。5.2%的患者并发炎症性疾病。六种症状占患者最初主诉的80%。总体而言，66%的患者随后至少出现10种症状，50%的患者至少出现14种症状，30%的患者报告至少出现26种症状。症状主要涉及心血管、胃肠道和神经系统。在大型专科门诊中就诊的患有体位性直立性心动过速综合征的儿科患者以女性为主，大多为白种人，症状始于青春期早期，在诊断前症状持续超过两年。超过一半的患者有关节活动过度。超过三分之一的患者有可能的自身免疫或炎症诱因，包括感染、脑震荡或手术/外伤。患者在病程中经历的症状高度可变且源于多系统。
