儿童自主神经介导性晕厥的治疗

自主神经介导性晕厥是儿童常见疾病,严重影响患儿的身心健康,给予及时有效的治疗尤为重要。儿童自主神经介导性晕厥的治疗在国内外学者的共同努力下取得了巨大的进展,特别是个体化治疗策略的提出,显著提高了治疗的疗效。今后仍需探索干预及预防新策略,不断优化个体化治疗的预测技术,为不断提高儿童身心健康水平付出不懈的努力。     

儿童青少年自主神经介导性晕厥的临床诊治

自主神经介导性晕厥是儿童晕厥中最常见的类型,是一种功能性心血管疾病,包括血管迷走性晕厥(vasovagal syncope,VVS)、体位性心动过速综合征(postural tachycardia syndrome,POTS)、直立性低血压(orthostatic hypotension,OH)、直立性高血压(orth...     

儿童自主神经介导性晕厥遗传学研究进展

儿童自主神经介导性晕厥（neurally mediated syncope,NMS）是最常见的晕厥类型,NMS的确切发病机制目前仍不详,根据其在家族中有簇集性发病的特点,提示有遗传学机制参与。近年来,NMS遗传学研究主要集中在与异常心血管反射相关的自主神经系统及其神经递质、血管活性分子等候选基因多态性、家族史研究和双胞胎研究方面等。     

自主神经介导性晕厥儿童的心率变异性研究（附29例分析）

探讨自主神经介导性晕厥（NMS）患儿心率变异性的变化。 方法　2007年4月至2008年3月在北京大学第一医院儿科就诊的NMS患儿29例,其中男11例,女18例;年龄8～17岁,平均（12.4±2.2）岁。所有患儿进行24h Holter监测。比较患儿心率变异性中的各项参数变化。采用SPSS10.0软件进行统计。结果　NMS组的窦性心搏间期标准差（SDNN）值与正常值相比,差异无统计学意义（P > 0.05）; 窦性心搏间期标准差的均值（SDNNi）与正常值相比增加了19.21%（P< 0.05）,rMSSD值与正常值相比增加了54.07%（P < 0.05）,相邻的NN间期之差大于50ms 的心搏数占总心搏数的百分率（pNN50）及三角指数值与正常值相比差异均无统计学意义（P > 0.05）。 结论　NMS患儿存在自主神经功能调节失衡。     

直立倾斜试验在神经介导性晕厥患儿中应用的几个问题

儿童自主神经介导性晕厥(neurally mediated syncope,NMS)是儿科门诊常见危急重症.直立倾斜试验(head-up tilt test,HUTT)在儿童NMS中具有较高的诊断价值,已广泛应用于临床,目前认为HUTT是一个相对安全的检查手段,其阳性率受多因素的影响.国内外关于HUTT阳性率影响因素、并发症、临床预测价值等问题的认识仍存在争议.该文对HUTT在儿童NMS应用中的这几个问题作一综述.     

儿童自主神经介导性晕厥与心因性假性晕厥的临床特征及鉴别

目的分析并比较儿童自主神经介导性晕厥(NMS)与心因性假性晕厥(PPS)的临床特征。方法回顾分析以晕厥为主诉,并排除心源性晕厥、境遇性晕厥、药源性晕厥及神经源性及代谢性疾病的短暂意识丧失患儿的临床特征,对比NMS与PPS临床特征的异同。结果入选106例晕厥患儿中,NMS85例(80.2%)、PPS13例(12.3%)、诊断不明晕厥8例(7.5%)。PPS多以近期精神刺激因素为诱因,晕厥次数频繁,直立不耐受评分高,先兆较少,发作持续时间长。NMS常以持久站立、运动、体位改变为主要诱因,先兆多以头晕、视物模糊、消化道症状为主,持续时间短5 min。结论 NMS是儿童晕厥最常见的原因,PPS是非晕厥型短暂意识丧失的重要原因,两者在临床特征上有相似点,应在诊断中予以鉴别。     

神经介导性晕厥儿童心室晚电位变化

神经介导性晕厥儿童存在自主神经功能紊乱,心室晚电位是预测恶性心律失常发生的指标,与自主神经功能密切相关,了解心室晚电位变化对临床预测神经介导性晕厥儿童的心血管事件具有一定价值。     

神经介导性晕厥患儿血压晨峰与血压昼夜节律的研究

目的:探讨神经介导性晕厥(NMS)患儿的血压晨峰(MBPS)及血压昼夜节律。方法:收集2018年7月至2019年6月因不明原因晕厥、先兆晕厥等症状初次就诊于中南大学湘雅二医院的135例患儿资料,其中男74例,女61例;年龄3～16岁[(10.12±2.53)岁];行直立倾斜试验(HUTT)的当日完成24 h动态血压监测...     

自主神经介导性晕厥儿童消化道症状临床分析

目的认识自主神经介导性晕厥患儿中腹痛等消化道表现的临床意义。方法 2009年1月至2012年3月在北京大学第一医院儿科住院诊断为自主神经介导性晕厥及心因性发作的患儿共108例,对患儿发作前及直立倾斜试验(HUT)中诱发的腹痛、恶心、呕吐等消化道症状进行临床研究。结果 108例患儿中,男49例(45.4%),女59例(54.6%)。年龄5～17岁(中位年龄11岁)。共33例(35.5%)自主神经介导性晕厥患儿在发作前出现胃肠道不适,15例(16.1%)表现为腹痛,20例(21.5%)恶心,12例(12.9%)呕吐。27例(40.9%)在HUT时诱发出胃肠道表现,6例(9.1%)HUT时诱发出腹痛,24例(36.4%)恶心。共39例(59.1%)在发作前或HUT时出现消化道表现。HUT时出现的消化道表现在体位性心动过速综合征(POTS)患儿和血管迷走性晕厥(VVS)患儿分别达到40%及41.5%,心因性发作组为0;发作前及HUT时患儿消化道表现在POTS及VVS组分别达到60%及58.5%,明显高于心因性发作(10%)组(P<0.05)。自主神经介导性晕厥患儿发作前及HUT时出现消化道症状的危险度是心因性发作患儿的13倍(95%CI均>1)。结论消化道表现是自主神经介导性晕厥的常见症状之一,对于发作前及HUT时发生腹痛、恶心患儿,需注意自主神经介导性晕厥的可能。剧烈腹痛可作为自主神经介导性晕厥的伴随症状。     

血管迷走性晕厥患儿无症状期自主神经功能分析

目的分析无症状期心率减速力(DC)对血管迷走性晕厥(VVS)的诊断价值。方法选取2018年1月至2019年12月确诊为VVS的45例患儿作为VVS组,同期在门诊体检的45例健康儿童作为对照组,完善24小时动态心电图检查,比较VVS患儿和健康儿童的基础DC值、连续心率减速力(DRs)及心率变异性(HRV)各指标的变化情况,并按年龄段及性别进行分组比较;同时分析DC与HRV各指标间的相关性及DC对VVS诊断的预测价值。结果 VVS组的DC值、DR8及低频功率与高频功率之比(LF/HF)均明显高于对照组,差异均有统计学意义(P0.05)。在VVS组中,学龄期患儿的DC值、DR4及低频功率(LF)均明显低于青春期患儿,差异均有统计学意义(P0.05),而男女患儿的DC值、DRs、HRV各指标差异均无统计学意义(P0.05)。对照组年龄段及性别组间DC值、DRs、HRV各指标差异均无统计学意义(P0.05)。学龄期VVS组的DC值明显高于对照组,青春期VVS组的DC值、DR4、DR8及LF均明显高于对照组,差异有统计学意义(P0.05)。DC与HRV多项指标呈显著正相关(r=0.31～0.67,P均0.05),与LF/HF呈显著负相关(r=-0.36,P0.05)。行二分类logistic回归分析发现,青春期患儿仅DC与VVS相关(P0.05)。采用受试者工作特征曲线(ROC)分析发现,学龄期DC值以7.72 ms、青春期DC值以8.36 ms为界值时有较好的灵敏度及特异度。结论 VVS患儿无症状期自主神经功能存在异常,随年龄增长,自主神经功能异常更加显著。学龄期及青春期VVS患儿均有迷走神经张力升高,且青春期VVS患儿还有一定程度交感神经张力升高;DC与迷走神经张力间呈明显正相关,对VVS诊断有较好预测价值。     

先天性心脏病患儿血浆硫化氢变化的意义

目的研究先天性心脏病(CHD)患儿血浆硫化氢(H2S)含量与正常小儿有何差异,并探讨其病理生理学意义。方法先天性心脏病和对照组各9例,测定其心率、血压、肺动脉压及血浆H2S含量,分析CHD患儿与对照组血浆H2S含量的差异及其与肺动脉压力的关系。结果CHD患儿血浆H2S含量为(32.13±2.25)μmol/L,低于对照组的(43.69±2.05)μmol/L,差异有显著性(P<0.01)。血浆H2S含量与肺动脉压力呈明显负相关(r=-0.864P<0.05)。结论内源性H2S的下调可能在CHD继发肺动脉高压的发病过程中起重要作用。     

Neurally mediated syncope complicated with paroxysmal atrial fibrillation

Recently, it has come to be accepted that the result of the tilt test is specific to neurally mediated syncope (vasovagal syncope). Only rarely is a case of paroxysmal atrial fibrillation without any organic diseases in childhood reported. A case reported here of a 14-year-old boy with neurally mediated syncope; which was complicated by paroxysmal atrial fibrillation, and which was diagnosed by performing the tilt test. Atrial fibrillation can be induced by the extraordinary stimulation of the vagal nerve during syncope. In a child, neurally mediated syncope complicated with paroxysmal atrial fibrillation has not been previously reported.     

先天性心脏病并肺动脉高压患儿血浆一氧化氮和硫化氢的变化

目的研究先天性心脏病(CHD)并肺动脉高压(PH)患者血浆一氧化氮(NO)和硫化氢(H2S)的变化及其与PH相关性,探论PH的形成机制,为临床有效治疗PH提供新的思路和理论依据。方法对全部研究对象行彩色超声心动图检查,明确CHD类型并测定肺动脉收缩压。按彩色超声心动图结果分为4组。正常儿童25例,CHD 75例,其中无PH 25例,轻度PH 25例,中、重度肺PH 25例。取CHD组术前静脉血4 mL,迅速分离血浆,采用分光光度法测定血浆NO水平,采用敏感硫电极法测定H2S水平。结果CHD患儿血浆NO水平明显高于对照组,但血浆NO水平增加到一定程度后不再随肺动脉收缩压增加而增加,肺动脉收缩压与血浆NO水平无相关性。CHD并中重度PH组血浆H2S水平明显低于CHD并轻度CHD组,CHD并轻度PH组血浆H2S水平明显低于对照组,肺动脉收缩压与血浆H2S水平呈负相关。结论CHD致PH形成时NO水平升高,代偿性内源性NO上调可能对缓解PH起一定作用。CHD致PH形成时H2S明显降低,内源性H2S下调可能在PH形成中起重要的作用。     

Neurally Mediated Syncope in Children: Results of Tilt Testing, Treatment, and Long-Term Follow-Up

Despite a great deal of literature concerning children with neurally mediated syncope (NMS) there is no clear standard of diagnosis or consistent approach to therapy. This report reviews our experience with tilt testing all patients who presented with syncope during a 3-year period. All patients referred for evaluation of syncope who underwent tilt testing were retrospectively reviewed. Follow-up was obtained at return visits or by telephone interview. Sixty patients were identified. The average age was 13.5 ± 3.0 years. Twenty-six patients (43%) had positive tilt tests. Follow-up was available for 56 patients. Twelve of these patients had causes other than NMS found for their symptoms. The remaining 44 patients all had histories consistent with NMS. Nineteen of these patients had positive tilt tests. All 44 patients reported either a decrease or no recurrence of syncope, and 41 patients reported a decrease or no recurrence of presyncopal symptoms. Recurrence of syncope or symptoms was not related to the results of the tilt test. Twenty-five of the 44 patients used conservative measures (extra fluids or supplemental salt) and only 3 patients were taking medications. The use of conservative measures or medications was not related to the tilt test results. Tilt testing has a high false-negative and false-positive rate and should not be used as the standard for identifying patients with NMS. The long-term follow-up of patients with NMS demonstrates that regardless of the results of the tilt test, almost all have improved or resolved symptoms with simple interventions.     

新型气体信号分子硫化氢在高脂血症儿童中的变化

目的探讨血脂紊乱儿童的血浆硫化氢(H2S)水平及其影响因素。方法随机选取常规体检时发现血脂增高的儿童40例(男31例,女9例)为血脂紊乱组,血脂正常儿童60例(男38例,女22例)为对照组。通过对其身高、体质量的测量得出肥胖指数(BMI);并对其家族史进行调查。应用敏感硫电极法测定血浆H2S水平。结果血脂紊乱组儿童血浆内源性H2S水平较对照组儿童H2S水平明显降低(P<0.01);男童组与女童组血浆H2S水平无显著性差异(P>0.05);血脂紊乱组与对照组儿童的身高、体质量、BMI、腰围、上臂围及坐高比较,组间差异无统计学意义(P>0.05)。结论高脂血症儿童血浆H2S水平明显降低,H2S可能参与儿童高脂血症的发病过程。     

Spectral and Time-Domain Analyses of Heart-Rate Variability During Head-Upright Tilt-Table Testing in Children with Neurally Mediated Syncope

Neurocardiac syncope (NS) is a common cause of syncope in children. The mechanism, though related to abnormalities in autonomic function, has not been fully elucidated, particularly in pediatric patients. This study assessed the heart-rate variability (HRV) response to head-upright tilt-table test (HUT) in children with NS and normal volunteers. Spectral and time-domain analysis of HRV was used to assess changes in autonomic function in 27 children (9 male, mean age 12.3 ± 1.6 years) with a history of at least one episode of syncope and positive passive HUT and 27 age-matched normal volunteers with negative passive HUT before and during postural tilt and to attempt to relate such changes to specific types of hemodynamic response to tilt. Frequency-domain measurements of the high-(HF) and low-(LF) frequency bands and the ratio LF/HF were derived from Holter recordings and computed by fast Fourier analysis for 5-min intervals. Time-domain measurements of the SDNN, SDNNI, SDANN, RMSSD, and triangular index were derived from 24-h Holter recordings. There were no significant differences between clinical characteristics, time-domain, and basal frequency domain parameters of the groups. Mean values of LF and LF/HF ratio was increased and HF was decreased significantly in response to tilt in both patient and control groups. Mean values of LF and LF/HF ratio were higher and HF was lower compared to controls immediately after tilt. LF and LF/HF ratio showed a statistically significant decrease and a significant increase in HF during syncope in patients. The three subgroups of patients had similar patterns of changes in autonomic activity. The results of this study show that although the basal autonomic function was similar to that of the control group, patients with NS have a different pattern of response to the HUT. In our study, patients with NS demonstrated an exaggerated response to the HUT. This exaggerated response may be the factor that activates the pathological reflexes of NS. The pathological mechanism leading to NS appears to be independent of the specific type of hemodynamic response to HUT.     

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??Neurally mediated syncope??NMS??is the most common type of syncope??whose exact pathogenesis is still unknown. The characteristics offamilyclustershow that NMS has a geneticmechanism. In recent years??geneticresearches of NMS are mainly concentrated on the candidate gene polymorphisms of autonomic nervous system??neurotransmitterand vasoactivemolecules??family history and twin study.     

急性白血病患儿柔红霉素化疗致心肌损害时血清硫化氢水平的变化

目的 探讨急性白血病(AL)患儿柔红霉素(DNR)化疗致心肌损害时血清硫化氢(H2S)水平变化.方法 2008年10月-2011年6月本院儿童血液科住院的AL患儿共159例,中位年龄7岁.均行禽DNR方案化疗,采用敏感硫电极法测定其化疗前后血清H2S水平变化,分析在不同DNR累积剂量时心肌细胞损害与H2S的关系.结果 随着DNR累积剂量的增加,心肌损害的发生率亦随之增高(Pa＜0.05),血清H2S水平亦随之增加.中剂量组患儿血清H2S水平较低剂量组升高,差异有统计学意义(P＜0.05);而高剂量组血清H2S水平较中剂量组进一步升高,差异有统计学意义(P＜0.01).心肌损害组血清H2S水平较无心肌损害组显著升高,差异有统计学意义(P＜0.01).对心肌损害组和无心肌损害组血清H2S水平进行ROC曲线分析,ROC曲线下面积为0.929 (95％ CI0.857 ～ 1.000).以假阳性率(1-特异性)2.8％为诊断点,血清H2S诊断心肌损害的诊断点为128.09 μmol·L-1,以此诊断的敏感性为83.3％,特异性为97.2％.对心肌损害患儿进行回顾性分析发现,DNR剂量达中剂量以上心肌细胞尚未受损时,其血清H2S水平已显著升高.以128.09 μmol·L-1为临界点,血清H2S水平升高的患儿其进一步化疗心肌损害的发生率亦显著升高.结论 血清H2S水平可反映心肌细胞的损害程度,是早期监测心肌细胞受损的有效指标.     

血管迷走性晕厥患儿40例

目的探讨不同类型血管迷走性晕厥(VVS)患儿的临床特征及实验室检查指标间的差异。方法经常规病史询问、体格检查、卧立位血压、辅助检查、直立倾斜试验(HUT)确诊的VVS患儿40例,比较不同类型患儿的临床特征及实验室指标间的差异。结果VVS患儿的血流动力学类型以血管抑制性为主。不同类型VVS患儿的临床特征,包括晕厥的诱因、先兆、发作频率、持续时间、基础心率、血压及血清电解质水平等均无显著差异。结论血管抑制型反应是血管迷走性晕厥患儿的主要血流动力学类型。