The study was designed to explore a clinical manifestation-based quantitative scoring model to assist the differentiation between psychogenic pseudosyncope (PPS) and vasovagal syncope (VVS) in children. In this retrospective case-control study, the training set included 233 pediatric patients aged 5-17 years (183 children with VVS and 50 with PPS) and the validation set consisted of another 138 patients aged 5-15 years (100 children with VVS and 38 with PPS). In the training set study, the demographic characteristics and clinical presentation of patients were compared between PPS and VVS. The independent variables were analyzed by binary logistic regression, and the score for each variable was given according to the approximate values of odds ratio (OR) to develop a scoring model for distinguishing PPS and VVS. The cut-off scores and area under the curve (AUC) for differentiating PPS and VVS cases were calculated using receiver operating characteristic (ROC) curve. Then, the ability of the scoring model to differentiate PPS from VVS was validated by the true clinical diagnosis of PPS and VVS in the validation set. In the training set, there were 7 variables with significant differences between the PPS and VVS groups, including duration of loss of consciousness (DLOC) (p < 0.01), daily frequency of attacks (p < 0.01), BMI (p < 0.01), 24-h average HR (p < 0.01), upright posture (p < 0.01), family history of syncope (p < 0.05) and precursors (p < 0.01). The binary regression analysis showed that upright posture, DLOC, daily frequency of attacks, and BMI were independent variables to distinguish between PPS and VVS. Based on the OR values of each independent variable, a score of 5 as the cut-off point for differentiating PPS from VVS yielded the sensitivity and specificity of 92.0% and 90.7%, respectively, and the AUC value was 0.965 (95% confidence interval: 0.945-0.986, p < 0.01). The sensitivity, specificity, and accuracy of this scoring model in the external validation set to distinguish PPS from VVS were 73.7%, 93.0%, and 87.7%, respectively. Therefore, the clinical manifestation-based scoring model is a simple and efficient measure to distinguish between PPS and VVS.

目的 分析自主神经介导性晕厥(ANS)患儿中医辨证分型特点.方法 收集2018年7月—2021年2月就诊于复旦大学附属儿科医院儿童晕厥中心的ANS患儿85例,其中经直立倾斜试验(HUTT)诊断为血管迷走性晕厥(VVS)患者60例,体位性心动过速综合征(POTS)患者25例.分析总结患儿的中医辨证分型特点.结果 85例ANS患儿平时最常见的10项症状依次为:头晕、出冷汗、神疲乏力、常不开心情绪低落、胸闷气短、面色苍白、视物模糊、紧张焦虑、多梦、胆小.中医证型归结为肝郁气滞、气阴两虚、气血亏虚、痰热内蕴4种.单一证型31例(36.47％),其中气阴两虚证20例、肝郁气滞证9例、气血亏虚证2例;复合证型54例(63.53％),均以肝郁气滞证(54例)为主证型,兼气血亏虚(29例)、气阴两虚(14例)、痰热内蕴(11例)3个证型.在85例患者中,单一证型和复合证型中诊断为肝郁气滞证的比例最高(63例,74.12％);其次为气阴两虚证(34例,40.0％)、气血亏虚证(31例,36.47％),痰热内蕴证作为兼证11例(12.94％).60例VVS患者主要证型为肝郁气血亏虚证(20例,33.33％)、气阴两虚证(16例,26.67％).25例POTS患者主要证型为肝郁气血亏虚证(9例,36.0％)、肝郁气阴两虚证(6例,24.0％).结论 VVS及POTS患儿的常见症状依次为头晕、出冷汗、神疲乏力、常不开心情绪低落;肝郁气滞和气血阴阳失衡(气阴两虚证、气血亏虚证)是ANS患儿的主要中医辨证分型.

目的 探讨锌水平在儿童和青少年自主神经介导性晕厥(NMS)中的临床价值.方法 选取行直立倾斜试验(HUTT)并检测微量元素和维生素(Vit)的NMS患儿102例(NMS组),根据HUTT结果分为HUTT阳性组(83例)和HUTT阴性组(19例).HUTT阳性组中血管迷走性晕厥(VVS)32例、体位性心动过速综合征(POTS)53例(有2例患儿同时合并VVS).以检测微量元素的健康体检儿童100名作为正常对照A组,检测Vit的健康体检儿童100名作为正常对照B组.检测所有研究对象全血锌、钙、铜、镁、铁水平和血清Vit A、Vit B1、Vit B2、Vit B6、Vit B9、Vit C、Vit D、Vit E水平.采用二元Logistic回归分析评估发生NMS的危险因素.采用受试者工作特征(ROC)曲线评估各项指标诊断NMS或鉴别诊断VVS与POTS的效能.结果 NMS组全血锌、钙、铁水平与正常对照A组比较,差异均有统计学意义(P<0.05);其他指标NMS组与2个正常对照组之间差异均无统计学意义(P>0.05).HUTT阳性组全血锌、钙、铁水平均低于正常对照A组(P<0.05).HUTT阴性组各项微量元素与HUTT阳性组和正常对照A组比较,差异均无统计学意义(P>0.05).VVS组全血锌水平低于正常对照A组(P<0.05).POTS组全血锌、钙、镁、铁水平均低于正常对照A组(P<0.05).VVS组全血镁水平高于POTS组(P<0.05).二元Logistic回归分析结果显示,锌水平下降是NMS发生的危险因素[比值比(odds ratio,OR)=0.726,95％可信区间(CI)为0.652～0.807],全血钙、铁水平降低与NMS发生无关(OR值分别为1.772、0.472,95％CI分别为0.639～4.909、0.090～2.476).ROC曲线分析结果显示,全血锌水平诊断NMS的曲线下面积(AUC)为0.800,最佳临界值为80.09μmol/L,敏感性为74.5％,特异性为80.0％.全血镁水平鉴别诊断VVS和POTS的AUC为0.631,最佳临界值为1.33 mmol/L,敏感性为67.9％,特异性为59.4％.结论 锌缺乏可能与儿童和青少年NMS的发生有关.镁对VVS与POTS的鉴别可能有一定的参考价值.

心因性假性晕厥(psychogenic pseudosyncope,PPS)貌似存在明显的短暂性意识丧失(TLOC),实际上为转换障碍所致,因存在显著的焦虑、抑郁等心理问题,患者生活质量受到严重影响.临床上,PPS与血管迷走性晕厥(vasovagalsyncope,VVS)的晕厥先兆及发病诱因有相似之处,有时两者难以区分.另一方面,临床医师对PPS的心理问题认识不充分,可能导致诊断率被低估.因此,重视PPS的病史特点,早期识别并治疗,对儿童心理健康尤为重要.

Objective: We aimed to establish useful models for the clinical differential diagnosis between vasovagal syncope (VVS) and psychogenic pseudosyncope (PPS). Methods: This bicentric study included 176 patients (150 VVS and 26 PPS cases) for model development. Based on the results of univariate and multivariate analyses, a logistic regression model and a scoring model were established and their abilities to differentiate VVS from PPS were tested. Another 78 patients (53 VVS and 25 PPS) were used for external validation. Results: In the logistic regression model, the outcome indicated that the QT-dispersion (QTd) (P < 0.001), syncope duration (P < 0.001), and upright posture (P < 0.001) acted as independent factors for the differentiation of VVS from PPS, which generated an area under the curve (AUC) of 0.892. A cutoff value of 0.234 yielded a sensitivity and specificity of 89.3 and 80.8%, respectively, for the differentiation between VVS and PPS in the logistic regression model. In the scoring model which consists of three variables, a cutoff score of three points yielded a sensitivity and specificity of 91.3 and 76.9%, respectively, with an AUC of 0.909. The external validation test indicated that the negative and positive predictive values of the scoring model were 78.8 and 91.7%, respectively, and the accuracy was 80.8%. Conclusion: The scoring model consisting of three variables is an easy-to-perform, inexpensive, and non-invasive measure for initial differential diagnosis between VVS and PPS.

川崎病(Kawasaki disease,KD)又称皮肤黏膜淋巴结综合征,系病因不明的急性自限性血管炎,临床以发热超过5天伴有皮疹、眼红、杨梅舌、肢端硬肿、颈部淋巴结肿大等为主要症状,5岁以下小儿多见.本病于1967年由日本的川崎富作先生首次在全球报道,川崎病因此得名.在发达国家,川崎病目前已成为儿童获得性心脏病的主要病因,并且可能是成年后发生缺血性心脏病的风险因素.

目的 分析杜氏肌营养不良(DMD)病儿的心电图特点,探索早期心电图异常改变及与其基因型关系,为临床诊治提供参考.方法 回顾性分析246例DMD病儿的270份心电图特点;选择抗肌萎缩蛋白(dystro-phin)基因含有大片段外显子缺失或重复病儿的158份心电图,分析心电图异常与其相应基因型关系.结果 心电图异常比例为22.96％(62/270),主要常见异常表现为左心室大/左心室高电压 、异常Q波 、V1导联深S波 、V1导联R/S>正常范围 、右心室大 、ST-T改变;其中,反映左心室心电生理改变表现的左心室大/左心室高电压 、V1导联S波加深及异常Q波所占总异常心电图的比例为66.13％(41/62).DMD病儿基因外显子缺失分布主要集中在外显子3-21与45-52区域,其中外显子1-2、44-54缺失组与其他位点缺失组病儿的心电图异常比例比较差异具有统计学意义(χ2=5.895,P<0.05).结论 DMD病儿心电图异常是较常见的现象,而且是以左心室大/左心室高电压 、V1导联S波加深 、异常Q波为最常见的表现.Dystrophin基因外显子1-2、44-54是DMD病儿心电图异常的高发区域.

目的 观察金百合剂治疗小儿急性病毒性咳嗽的临床疗效.方法 将符合观察标准的270例小儿急性病毒性咳嗽患儿随机分为金百组、清热止咳组、易坦静组,分别予以金百合剂、小儿清热止咳口服液、易坦静单药治疗,疗程5天.观察患儿症状分级量化指标并评价临床疗效,观察治疗前后中医证候积分的变化及临床试验过程中是否出现不良事件.结果 治疗前各组性别、年龄及病程均无统计学差异.金百组、清热止咳组、易坦静组临床总有效率分别为87.78％、72.22％和52.87％,金百组临床控制率明显优于清热止咳组和易坦静组(P＜0.01);各组中医证候积分均较治疗前降低(P＜0.01),其中金百组中医证候总积分减分差值显著高于清热止咳组(P＜0.05)和易坦静组(P＜0.01),咳嗽、咯痰积分减分差值大于清热止咳组和易坦静组(P＜0.01).各组均无用药不良事件.结论 金百合剂可显著改善急性上呼吸道感染引起的小儿咳嗽痰多等临床症状,临床控制率显著.

Syncope belongs to the transient loss of consciousness (TLOC), characterized by a rapid onset, short duration, and spontaneous complete recovery. It is common in children and adolescents, accounting for 1% to 2% of emergency department visits.Recurrent syncope can seriously affect children's physical and mental health, learning ability and quality of life and sometimes cardiac syncope even poses a risk of sudden death. The present guideline for the diagnosis and treatment of syncope in children and adolescents was developed for guiding a better clinical management of pediatric syncope. Based on the globally recent development and the evidence-based data in China, 2018 Chinese Pediatric Cardiology Society (CPCS) guideline for diagnosis and treatment of syncope in children and adolescents was jointly prepared by the Pediatric Cardiology Society, Chinese Pediatric Society, Chinese Medical Association (CMA)/Committee on Pediatric Syncope, Pediatricians Branch, Chinese Medical Doctor Association (CMDA)/Committee on Pediatric Cardiology, Chinese College of Cardiovascular Physicians, Chinese Medical Doctor Association (CMDA)/Pediatric Cardiology Society, Beijing Pediatric Society, Beijing Medical Association (BMA). The present guideline includes the underlying diseases of syncope in children and adolescents, the diagnostic procedures, methodology and clinical significance of standing test and head-up tilt test, the clinical diagnosis vasovagal syncope, postural orthostatic tachycardia syndrome, orthostatic hypotension and orthostatic hypertension, and the treatment of syncope as well as follow-up.

Objective To illustrate the clinical features,efficiency of anti-arrhythmic medications and prognosis in pediatric tachycardia-induced cardiomyopathy( TIC). Methods Retrospective review of medical records was carried out in children with TIC,and defined as LVFS <30% associated with various tachyarrhythmia in Children's Hospital of Fudan University. Data with respect to the clinical findings,anti-arrhythmic therapy,electrocardiographic and echocardiographic evaluation and biomarkers were collected. Clinical follow-up was continued until both LVFS≥30% and Z-LVEDD>2. 0 were achieved. Multivariate linear and logistic regression analysis was performed to evaluate possible predictors of time to myocardial recovery. Results A total of 56 pediatric cases with TIC were included in our study from May 2003 to January 2016. Forty patients(71. 4%)were infants and 37 were boys(66. 1%). Atrial tachycardia and paroxysmal supraventricular tachycardia were the most common types of responsible tachyarrhythmia,with the percentage of 53. 6% and 37. 5%,respectively. Anti-arrhythmic medications as part of management were used in all 56 cases and complete,partial and none effective response to medications occurred in 29(51. 8%),23(41. 1%)and 4(7. 1%)cases,respectively. Amiodarone was the most commonly used medication,and its effectiveness was achieved in 92. 3%(36/39)children,including both used alone and in combination with other medications. Except for 2 cases of death,left ventricular fractional shortening(LVFS)was significantly increased from(23.7 ±3.7)% to(32.8 ±4.6)%(P<0.001),and Z-LVEDD was decreased from(2. 79 ± 3. 04)to(0. 37 ± 2. 0)( P<0. 001)in the remaining 54 cases. Median time for recovery of LVFS and Z-LVEDD was 10 days and 42 days,respectively. Plasma( N-terminal)pro B-type natriuretic peptide was reduced significantly from(15 711 ±11 453)pg·mL-1 to(1 287 ±1 510)pg·mL-1(P<0.001). Predictor of earlier left ventricular size normalization was smaller baseline Z-LVEDD(β =0. 528,95%CI:0. 127 -0. 506,P =0. 002). Conclusion Myocardial dysfunction is wholly or partially reversible after control of the responsible tachyarrhythmia in pediatric TIC. Early recognition and&nbsp;prompt treatment by clinicians are crucial for favorable recovery of left ventricular function.

Abstract Objective Most idiopathic right ventricular tachycardias originate from the outflow tract. We present a case series of idiopathic incessant ventricular tachycardia arising from unusual sites of the right ventricle in children, which were well resolved by catheter ablation. Methods A retrospective review was performed of all three patients who underwent ablation of idiopathic ventricular tachycardia below the level of the right ventricular outflow tract using three-dimensional mapping in our institute. Result All three patients presented with tachycardia-induced cardiomyopathy due to incessant ventricular tachycardia on first admission. The sites of successful ablation were at the proximal right bundle branch, distal right bundle branch, and apex of the right ventricle, respectively. No complications occurred, and there has been no recurrence of ventricular tachycardia after the final ablation at an average follow-up period of 9 months. All three patients have achieved normalisation of left ventricular size and systolic function. Conclusion Incessant idiopathic ventricular tachycardia originating from unusual sites of the right ventricle in children, resulting in significant symptoms and impaired ventricular function, can be successfully treated with catheter ablation.

儿童体位性心动过速综合征( postural tachycardia syndrome,POTS)是慢性直立不耐受疾病中最常见的类型之一。我国学者于2005年首先报道了儿童POTS[1]。临床上有别于血管迷走性晕厥,诊断儿童POTS需同时满足以下3项：①直立后出现头晕或眩晕、晕厥、胸闷、恶心、心悸、头痛、视物模糊、手抖、冷汗等直立不耐受表现。②直立试验和(或)直立倾斜试验(head-up tilt test,HUT)呈阳性反应。③除外其他可致自主神经系统症状的基础疾病[2]。其中HUT阳性标准为：患儿在直立10分钟内心率增加≥40次/分钟或心率最大值≥120次/分钟,同时伴有直立后的头晕或眩晕、胸闷、头痛、心悸、面色改变、视物模糊、倦怠,甚至晕厥等直立不耐受症状。近期有研究提出对于儿童HUT心率的观察时间可以缩短至5分钟,即：①HUT 5分钟内心率增加≥40次/分钟；或②HUT 5分钟内最大心率≥130次/分钟(年龄≤13岁)或≥120次/分钟(年龄≥14岁),满足其中之一时POTS的阳性诊断标准即成立[3]。

Objective To analyze the underlying disease spectrum of transient loss of consciousness (TLOC) in children through a multi-center and large sample chinical research.Methods The underlying disease spectrum of TLOC in 1203 children (559 male and 644 female children,aged 2-18 yrs,mean age 11.5 ±3.2 yrs) who came from Beijing,Hunan province,Hubei province and Shanghai of China was explored from August 1999 to April 2013.Results In 1 203 children with TLOC,1 125 (93.5％)were children with syncope,and 78 (6.5％) were non-syncope.In children with syncope,234 (19.5％)were of VVS-vasoinhibitory type (VVS-Ⅵ),346 (28.8％) POTS and 24 (2.0％)orthostatic hypertension (OHT).Conclusion Syncope was the most common disease of TLOC in children.And the most common hemodynamic pattern of syncope was VVS,followed by POTS.In three different hemodynamic pattems of VVS,the most common pattern was VVS vasoinhibitory one.In addition,OHT might also result in syncope.

以往冶们认为二氧化硫(sulfur dioxide,SO2)是一种大气污染物,也是当今许多工业生产过程中产生的废气之一.然而,近年来我国医学科学家研究发现,在心血管系统中存在SO2生成体系,并且内源性SO2具有重要的心血管生理学效应,同时内源性SO2在低氧性肺动脉高压、高血压、动脉粥样硬化、心肌损伤等心血管疾病发生中具有重要的病理生理学调节作用.因此,目前认为内源性SO2是继一氧化氮、一氧化碳和硫化氢之后的又一内源性心血管系统气体信号分子.

晕厥在小儿时期的发生率约为15％ ～25％,以15～19岁的儿童多见[1].急诊就诊率约3％.与成人不同的是,小儿晕厥病因中有60％ ～ 80％属血管反射性晕厥,且预后多数良好.而可能危及生命的心源性晕厥发生率仅占所有晕厥的2％ ～6.5％[1].香港中文大学附属医院儿科报道149例晕厥患儿心源性晕厥占2.1％;北京大学第一医院儿科报道154晕厥病例,心源性晕厥占6.5％,本院统计了414例晕厥患儿,发现心源性晕厥比例仅为1.2％.

AIM:This study was designed to investigate the value of history taking in identifying children with cardiac syncope, and to improve diagnostic efficiency and accuracy in children with cardiac syncope. METHODS AND RESULTS:We compared the characteristics of a group of children and adolescents with cardiac syncope at the Pediatric Syncope Unit of five hospitals in China with those with typical vasovagal syncope. We included a cohort of 275 patients in Pediatric Syncope Unit. A cardiac cause of syncope was established in 31 patients, autonomic-mediated reflex syncope in 214, non-syncopal attacks in 15, and in the remaining 15 the cause of syncope remained unexplained. Cardiac syncope was triggered by exercise, whereas vasovagal syncope by prolonged standing, warm-crowded place, and fear or pain emotion. Syncopal spells occurred at various positions in cardiac syncope. Children who had prodromal symptoms with cardiac syncope were significantly fewer than those with vasovagal syncope. Most children with cardiac syncope had history of abnormal electrocardiogram findings when compared with children suffering from vasovagal syncope. On multivariable analysis, history of abnormal electrocardiogram findings and exercise-triggered syncope were independent predictors of cardiac syncope. CONCLUSION:Children and adolescents with a history of abnormal electrocardiogram findings and exercise-related syncope spells were at high risk for cardiac syncope.

Objective To analyze the spectrum of underlying diseases in children with transient loss of consciousness (TLOC) through a multi-center and large sample clinical research.Methods Nine hundred and thirty-seven children with TLOC who came from Beijing,Hunan province,Hubei province and Shanghai of China from Aug 1999 to Apr 2011 were recruited in the present study,and then the spectrum of underlying diseases in children with TLOC was analyzed.Results In 937 children with TLOC,903 cases (96.4％ )were children with syncope,34 cases (3.6％) were non-syncope.And in 903 children with syncope,213 cases (23.6％) had vasovagal syncope (VVS) with vasoinhibitory response,46 cases (5.1％ ) had VVS with cardioinhibitory response,112 cases ( 12.4％ ) had VVS with mixed response,268 cases (29.7％ ) had postural tachycardia syndrome,22 cases (2.4％) had orthostatic hypotension,19 cases (2.1％ ) had situational syncope,21 cases (2.3％ ) had cardiogenic syncope,and 202 cases (22.4％ ) had unexplained syncope.Conclusion In children with TLOC,syncope was the most common underlying disease.And in children with syncope,the most common was VVS,followed by postural tachycardia syndrome.In three different hemodynamic patterns of VVS,the most common pattern was VVS vasoinhibitory pattern.

Objective To analyze heart rate variability during asymptomatic periods in children with vasovagal syncope(VVS). Me-thods Forty-five children diagnosed as VVS in clinic were recruited as a study group,38 with positive and 7 with negative response to head-up tilt test(HUTT).Twenty healthy children were matched as healthy controls.Twenty-four hours holter monitoring was performed and time and frequency domain indices were studied. Results 1.Compared with the healthy controls,the values of time-domain SDNN(121.82±33.0 vs 152.95±31.66,P0.01) and SDANN(114.42±33.41 vs 134.7±27.43,P0.05) were lower,and the ratio of low frequency / high frequency(LF/ HF)(1.67±0.54 vs 1.25±0.2,P0.01) were higher in study group.2.There was no significant difference in the time-domain and frequency-domain indices(P0.05) in HUTT positive and negative children.3.Compared with cardioinhibitory response(CI) children,SDNN(100.61±23.02 vs 150.5±29.46,P0.01),SDANN(96.78±28.77 vs 141.25±29.06,P0.05),rMSSD(29.22±19.56 vs 53.75±23.08,P0.05) and PNN50(11.29±9.31 vs 23.96±12.54,P0.05) were significantly lower,LF(39.61±8.45 vs 19.68±4.17,P0.01) and LF/HF(2.12±0.55 vs 1.09±0.11,P0.01) were significantly higher in vasodepressor response(VD) children.And compared with mixed responses(MX)children,rMSSD(29.22±19.56 vs 52.57±18.54,P0.05) and PNN50(11.29±9.31 vs 22.96±11.13,P0.05) were lower significantly,LF(39.61±8.45 vs 29.72±9.87,P0.05) and LF/HF(2.12±0.55 vs 1.38±0.30,P0.01) were higher significantly in VD children,but there was no difference between CI and MX children(P0.05).4.Compared with the healthy controls,rMSSD and PNN50 obviously declined in VD group while LF significantly increased(P0.05). Conclusions There is a different alteration in autonomic activity in VVS children during asymptomatic periods.The changes in baseline of RMSSD,PNN50 and LF parameters may serve as a predictor to identify VD clinically.

BACKGROUND Syncope accounts for about 1-2% of emergency department visits, but the etiology in many patients with syncope is unclear. Recently, with the use of the head-up tilt test (HUT), the number of patients with unexplained syncope (UPS) has been decreasing; however, the spectrum of underlying diseases of syncope in children is unclear. This retrospective study aimed to analyze the spectrum of underlying diseases in children with syncope. MATERIAL/METHODS This multi-center clinical study consisted of 888 children (417 males, 471 females, aged 5-18 yrs, median age 12.0±3.0 yrs) with syncope who came from Beijing city, Hunan province, Hubei province and Shanghai from August 1999 to March 2009. The clinical and laboratory data of children were studied and the spectrum of underlying diseases in children with syncope was analyzed. RESULTS In 888 children with syncope, 175 (19.7%) had vasovagal syncope (VVS) with vasoinhibitory response, 35 (3.9%) had VVS with cardioinhibitory response, 73 (8.2%) had VVS with mixed response, 286 (32.2%) had postural orthostatic tachycardia syndrome (POTS), 19 (2.1%) had orthostatic hypotension, 7 (0.9%) had situational syncope, 13 (1.5%) had cardiogenic syncope, and 280 (31.5%) had unexplained syncope. CONCLUSIONS The data suggest that neurally-mediated syncope was the most common cause in children with syncope. POTS and VVS were the most common hemodynamic patterns of neurally-mediated syncope.

直立倾斜试验(head-up tilt test,HUTT)是通过改变被检查者的体位(从平卧位改变至一定角度的倾斜位),再现其晕厥发作过程的一种检查方法.20世纪80年代末Kenny等将其应用于临床诊断血管迷走性晕厥(vasovagal syncope,VVS).迄今已经历了20多年的发展历史.1997年,北京大学第一医院首先在国内开创了儿童HUTT.