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北京大学学报（医学版） ›› 2022, Vol. 54 ›› Issue (5): 954-960. doi: 10.19723/j.issn.1671-167X.2022.05.024

• 论著 • 上一篇    下一篇

儿童直立不耐受和坐位不耐受的疾病谱及治疗方式十年回顾

崔雅茜1,杜军保1,2,张清友1,廖莹1,刘平1,王瑜丽1,齐建光1,闫辉1,徐文瑞1,刘雪芹1,孙燕1,孙楚凡1,张春雨1,陈永红1,金红芳1,*()   

  1. 1. 北京大学第一医院儿科, 北京 100034
    2. 教育部分子心血管学重点实验室, 北京 100191
  • 收稿日期:2022-06-03 出版日期:2022-10-18 发布日期:2022-10-14
  • 通讯作者: 金红芳 E-mail:jinhongfang51@126.com
  • 作者简介:金红芳，教授，研究员，博士生导师，北京大学第一医院儿科副主任，教育部长江学者青年学者，国家自然科学基金优秀青年基金获得者，国家高层次人才特殊支持计划(“万人计划”)青年拔尖人才，教育部新世纪优秀人才，北京市科技新星。担任Front Pediatr和Front Cardiovasc Med副主编，中国医师协会儿童重症医师分会心血管专业委员会主任委员，世界华人医师协会儿科医师分会心血管专业委员会主任委员，中华医学会儿科学分会心血管学组副组长，全国儿童心血管基础研究协作组组长。共发表SCI论文196篇，被引用4 229篇次，主持国家及部委级科研项目10余项，作为第一完成人获得教育部自然科学奖以及华夏医学科技奖，荣获茅以升科学技术奖——北京青年科技奖。
    在机体新的气体信号分子发现及儿科心血管疾病诊治领域取得突破性成就。首次发现并提出“内源性SO2是心血管调节的新的气体信号分子”，发现心血管系统存在内源性SO2的生成体系，揭示内源性SO2的心血管生理学意义以及病理生理学意义，阐明内源性SO2发挥生物学调节作用的分子机制，阐明气体信号分子H2S的表观调控机制，以及含硫气体信号分子间的相互作用及其生物学意义，进一步揭示了心血管疾病发病的新机制，推动形成了国际内源性SO2生物学研究的新领域。
    围绕影响儿童健康的重要疾病——儿童晕厥等功能性心血管病进行了系统、深入研究，在新疾病的发现、疾病的诊断技术与个体化治疗策略创立等方面取得了突出成绩。在国际上率先发现“儿童坐位不耐受”“坐位性心动过速综合征”“坐位性高血压”等导致儿童晕厥的系列新疾病，并通过全国多中心临床流行病学研究，建立了上述疾病的诊断标准，由此扩大了儿童晕厥及心血管病的基础疾病谱。在国内外首次建立了儿童晕厥个体化治疗决策体系，将儿童晕厥治疗有效率从65.6%提高至91.9%。应邀牵头主持撰写儿童青少年晕厥诊治国际指南，带动我国及国际116所医疗机构开展儿童晕厥及心血管疾病诊治工作，研究成果已推广至我国30个省(自治区、直辖市)以及英国、美国、日本
  • 基金资助:
    教育部长江学者奖励计划(青年学者)(Q2017004);北京市科技计划(Z171100001017253);中央高水平医院临床科研基金[北京大学第一医院国内多中心临床研究专项](2022CR59)

A 10-year retrospective analysis of spectrums and treatment options of orthostatic intolerance and sitting intolerance in children

Ya-xi CUI1,Jun-bao DU1,2,Qing-you ZHANG1,Ying LIAO1,Ping LIU1,Yu-li WANG1,Jian-guang QI1,Hui YAN1,Wen-rui XU1,Xue-qin LIU1,Yan SUN1,Chu-fan SUN1,Chun-yu ZHANG1,Yong-hong CHEN1,Hong-fang JIN1,*()   

  1. 1. Department of Pediatrics, Peking University First Hospital, Beijing 100034, China
    2. Key Laboratory of Molecular Cardiovascular Sciences, Ministry of Education, Beijing 100191, China
  • Received:2022-06-03 Online:2022-10-18 Published:2022-10-14
  • Contact: Hong-fang JIN E-mail:jinhongfang51@126.com
  • Supported by:
    the Cheung Kong Scholars (Young Scholars) Award Program, Ministry of Education, China(Q2017004);Beijing Municipal Science and Technology Project(Z171100001017253);the National High Level Hospital Clinical Research Funding (Multi-center Clinical Research Project of Peking University First Hospital)(2022CR59)

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摘要：

目的: 分析儿童直立不耐受(orthostatic intolerance, OI)和坐位不耐受(sitting intolerance, SI)的基础疾病谱, 并了解其在临床上采用的经验性治疗方式。方法: 选择北京大学第一医院儿科2012年1月至2021年12月十年期间所有患儿的病例资料(包括病史、体格检查、实验室检查和影像学检查)进行回顾性分析, 将符合OI和SI诊断标准的患儿纳入研究, 分析患儿OI和SI的基础疾病谱, 并总结分析其治疗方式。结果: 共纳入OI和SI病例资料2 110例, 其中男943例(44.69%), 女1 167例(55.31%), 年龄4~18岁, 平均(11.34±2.84)岁。十年间总体OI和SI患儿例数呈逐渐增多趋势。OI疾病谱中占比最高的是体位性心动过速综合征(postural orthostatic tachycardia syndrome, POTS), 共826例(39.15%); 其次为血管迷走性晕厥(vasovagal syncope, VVS), 共634例(30.05%)。而SI疾病谱中占比最高的是坐位性心动过速综合征(sitting tachycardia syndrome, STS), 共8例(0.38%); 其次为坐位性高血压(sitting hypertension, SHT) 2例(0.09%); 此外, OI和SI合并疾病中占比最多的是POTS合并STS, 共36例(1.71%)。在OI和SI治疗方式中, 主要为自主神经功能锻炼757例(35.88%), 其次为口服补液盐(oral rehydration salts, ORS) 687例(32.56%), 美托洛尔307例(14.55%), 盐酸米多君142例(6.73%), ORS联合美托洛尔138例(6.54%)和ORS联合盐酸米多君79例(3.74%)。POTS合并VVS的患儿相对于POTS或VVS患儿, 接受药物治疗者更多(41.95% vs. 30.51% vs. 28.08%, χ2= 20.319, P < 0.01), 而POTS和VVS患儿的药物治疗占比没有统计学差异。结论: 儿童POTS及VVS是OI的主要基础疾病, SI是近年来新发现的疾病。OI和SI患儿的人数整体呈逐渐增加的趋势, 其主要治疗方式为自主神经功能锻炼和口服ORS。POTS合并VVS的患儿相对于单纯POTS或VVS患儿, 更倾向于接受药物治疗。

关键词: 儿童, 直立不耐受, 坐位不耐受, 疾病谱

Abstract:

Objective: To analyze the disease spectrums underlying orthostatic intolerance (OI) and sitting intolerance (SI) in Chinese children, and to understand the clinical empirical treatment options. Methods: The medical records including history, physical examination, laboratory examination, and imagological examination of children were retrospectively studied in Peking University First Hospital from 2012 to 2021. All the children who met the diagnostic criteria of OI and SI were enrolled in the study. The disease spectrums underlying OI and SI and treatment options during the last 10 years were analyzed. Results: A total of 2 110 cases of OI and SI patients were collected in the last 10 years, including 943 males (44.69%) and 1 167 females (55.31%) aged 4－18 years, with an average of (11.34±2.84) years. The overall case number was in an increasing trend over the year. In the OI spectrum, postural tachycardia syndrome (POTS) accounted for 826 cases (39.15%), followed by vasovagal syncope (VVS) (634 cases, 30.05%). The highest proportion of SI spectrum was sitting tachycardia (STS) (8 cases, 0.38%), followed by sitting hypertension (SHT) (2 cases, 0.09%). The most common comorbidity of OI and SI was POTS coexisting with STS (36 cases, 1.71%). The highest proportion of treatment options was autonomic nerve function exercise (757 cases, 35.88%), followed by oral rehydration salts (ORS) (687 cases, 32.56%), metoprolol (307 cases, 14.55%), midodrine (142 cases, 6.73%), ORS plus metoprolol (138 cases, 6.54%), and ORS plus midodrine (79 cases, 3.74%). The patients with POTS coexisting with VVS were more likely to receive pharmacological intervention than the patients with POTS and the patients with VVS (41.95% vs. 30.51% vs. 28.08%, χ2= 20.319, P < 0.01), but there was no significant difference in the proportion of treatment options between the patients with POTS and the patients with VVS. Conclusion: POTS and VVS in children are the main underlying diseases of OI, while SI is a new disease discovered recently. The number of children with OI and SI showed an increasing trend. The main treatment methods are autonomic nerve function exercise and ORS. Children with VVS coexisting with POTS were more likely to take pharmacological treatments than those with VVS or POTS only.

Key words: Children, Orthostatic intolerance, Sitting intolerance, Disease spectrum

中图分类号: 

  • R725

图1

不同年份OI和SI患儿人数变化"

表1

直立不耐受和坐位不耐受患儿的疾病谱变化"

Disease 2012 to 2016 2017 to 2021
Total Male Female Total Male Female
POTS 478 (49.64) 217 (45.40) 261 (54.60) 348 (30.34) 153 (43.97) 195 (56.03)
VVS 300 (31.15) 130 (43.33) 170 (56.67) 334 (29.12) 147 (44.01) 187 (55.99)
POTS + VVS 48 (4.98) 26 (54.17) 22 (45.83) 281 (24.50) 104 (37.01) 177 (62.99)
POTS + OHT 57 (5.92) 34 (59.65) 23 (40.35) 29 (2.53) 16 (55.17) 13 (44.83)
OHT 65 (6.75) 31 (47.69) 34 (52.31) 11 (0.96) 6 (54.55) 5 (45.45)
OH 8 (0.83) 4 (50.00) 4 (50.00) 18 (1.57) 9 (50.00) 9 (50.00)
VVS + OHT 5 (0.52) 4 (80.00) 1 (20.00) 22 (1.92) 14 (63.64) 8 (36.36)
POTS + VVS + OHT 2 (0.21) 1 (50.00) 1 (50.00) 20 (1.74) 8 (40.00) 12 (60.00)
POTS + VVS + STS - - - 26 (2.26) 7 (26.92) 19 (73.08)
POTS + STS - - - 36 (3.14) 19 (52.78) 17 (47.22)
VVS + STS - - - 8 (0.70) 4 (50.00) 4 (50.00)
STS - - - 8 (0.70) 5 (62.50) 3 (37.35)
SHT - - - 2 (0.17) 2 (100.00) 0 (0.00)
OHT + SHT - - - 1 (0.09) 1 (100.00) 0 (0.00)
POTS + OHT + STS + SHT - - - 3 (0.26) 1 (33.33) 2 (66.67)
Total 963 447 (46.42) 516 (53.58) 1 147 496 (43.24) 651 (56.76)

表2

POTS、VVS以及POTS合并VVS三组患儿的治疗方式分析"

Treatment options POTS VVS POTS + VVS
Autonomic nervous function exercise 297 (35.96) 211 (33.28) 108 (32.83)
ORS 277 (33.53) 245 (38.64) 83 (25.23)
Metoprolol 140 (16.95) 60 (9.46) 42 (12.77)
Midodrine 43 (5.21) 55 (8.68) 34 (10.33)
ORS plus metoprolol 47 (5.69) 37 (5.84) 36 (10.94)
ORS plus midodrine 22 (2.66) 26 (4.10) 26 (7.90)
Total 826 634 329

表3

POTS、VVS以及POTS合并VVS三组患儿接受药物和非药物治疗构成分析"

Treatment options POTS VVS POTS + VVS
Non-pharmacological 574 (69.49) 456 (71.92) 191 (58.05)
Pharmacological 252 (30.51) 178 (28.08) 138 (41.95)
Total 826 634 329
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ISSN 1671-167X
CN 11-4691/R
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