기립성 체위성 빈맥 증후군으로 진단된 소아청소년 환자의 임상적 특징 및 신경생리검사 소견

• 영문명: Clinical Characteristics and Neurophysiologic Findings of Postural Orthostatic Tachycardia Syndrome in Pediatric Patients
• 발행기관: 대한소아신경학회
• 저자명: 백종근(Jong Geun Baek) 김영수(Young Soo Kim) 박기종(Ki-Jong Park) 염정숙(Jung Sook Yeom) 박지숙(Ji Sook Park) 박은실(Eun Sil Park) 서지현(Ji Hyun Seo) 임재영(Jae Young Lim) 박찬후(Chan Hoo Park) 우향옥(Hyang Ok Woo) 윤희상(Hee Shang Youn)
• 간행물 정보: 『Annals of Child Neurology(구 대한소아신경학회지)』대한소아신경학회지 제18권 제2호, 275~283쪽, 전체 9쪽
• 주제분류: 의약학 > 소아과학
• 파일형태: PDF
• 발행일자: 2010.11.30

영문 초록

Purpose : No domestic report has been published on postural orthostatic tachycardia syndrome (POTS) in adolescents, although it has been increasingly studied in adolescents since first reported in 1999. This study analyzed the clinical characteristics and neurophysiologic findings of POTS in Korean pediatric patients. Methods : We reviewed the medical records of pediatric patients (<18 years) with POTS who visited Gyeongsang National University Hospital in Jinju between January 2008 and March 2010. All patients underwent tilt-table and autonomic function tests. Result : We identified 18 patients (72.2% male, mean age 13.6 ± 2.1 years). The mean orthostatic heart rate increment was 43.0 ± 9.1 beats/min. Most patients (83.3 %) had mild autonomic dysfunction, and three (16.6%) had moderate autonomic dys- function. Half of patients had sudomotor abnormalities in the lower limbs, implying peripheral postganglionic sympathetic denervation in the legs. Of these patients, 66.6 % also had adrenergic impairment. The degree of autonomic dysfunction correlated with the orthostatic heart rate increment (P =0.047). Patients needing pharmacologic treatment had a higher orthostatic heart rate increment (P =0.017), composite auto- nomic severity score (P =0.002), and sudomotor score (P =0.006), compared with non-pharmacologically treated patients. Conclusion : Autonomic dysfunction or autonomic neuropathy seems to be the main pathophysiology of POTS in pediatric patients, especially adolescents. Further, the degree of autonomic dysfunction influenced the prognosis of POTS. Additionally, unlike previous adult series, most of our patients were male. This male predominance in adolescents suggests that the growth spurt is an important factor triggering POTS at this age.

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키워드

Postural orthostatic tachycardia syndrome Orthostatic intolerance Adolescence