QT间期离散度对儿童及青少年体位性心动过速综合征预后估测价值

目的　探讨QT间期离散度（QTd）对儿童及青少年体位性心动过速综合征（POTS）的预后估测价值。方法　选取2012年7月至2020年5月因晕厥或晕厥先兆在中南大学湘雅二医院儿童晕厥专科门诊首次就诊或住院,明确诊断为POTS的儿童及青少年75例为POTS组,匹配同期在本院进行健康检查的儿童及青少年75例为对照组。测量两组基础状态下12导联心电图QT间期,计算QT间期离散度（QTd）。采用SPSS ?22.0统计软件进行数据处理。结果　（1）两组比较：POTS组较对照组最大QT间期（QTmax）、最小QT间期（QTmin）、QTd、校正最大QT间期（QTcmax）、校正最小QT间期（QTcmin）、校正QT间期离散度（QTcd）延长（P＜0.001或0.05）。（2）预后估测价值：POTS组干预后随访55（36,114） d,有反应组较无反应组QTd、QTcd延长（P＜0.001）。QTd的AUC最大（0.910）,其最佳截断值为30.00 ms时,预测对POTS干预有反应的灵敏度为82.90%,特异度为81.78%。结论　心电图QTd对儿童及青少年POTS的预后有较好的估测价值。     

血管迷走性晕厥儿童卧位、立位QT间期离散度及P波离散度的变化

目的探讨血管迷走性晕厥(VVS)儿童卧、立位QT间期离散度(QTd)及P波离散度(Pd)的变化。方法直立倾斜试验(HUTT)阳性的VVS患儿46例为研究组。年龄7~17岁,平均(11.91±2.09)岁。用SR-1000A心电综合自动分析仪描记卧位及立位12导联同步体表心电图(12ECG)。匹配健康儿童30例为对照组。选择窦性心律波形清晰的12ECG3个心动周期,测量心率(HR)、QTd与Pd,按Bazete公式校正心率后得出QTcmax、QTcmin、QTcd、Pcmax、Pcmin、Pcd。结果1.对照组卧位较立位最大QT间期(QTmax)、最小QT间期(QTmin)、最大P波时间(Pmax)均明显延长(Pa<0.01),最小P波时间(Pmin)缩短(P<0.05),Pd延长(P<0.05),QTd、QTcmax、QTcmin、QTcd、Pcmax、Pcmin、Pcd未见明显变化(Pa>0.05)。2.研究组卧位较立位QTmax、QTmin、QTd、QTcd、Pmax、Pd、Pcd明显延长(Pa<0.01),Pcmax延长(P<0.05),Pcmin明显缩短(P<0.01),QTcmax、QTcmin、Pmin未见明显变化(Pa>0.05)。3.研究组较对照组卧位QTmax、QTcmax、Pcd延长(Pa<0.05),QTd、QTcd、Pmax、Pcmax延长(Pa<0.01);立位Pcd延长(Pa<0.05),Pmax、Pcmax延长(Pa<0.01)。4.研究组较对照组卧位时HR稍降低(P>0.05),立位时HR明显增加(P<0·01),HR增加在研究组与对照组比较未见差异(分别增加15.29次/minvs12.57次/min,P>0.05)。5.VVS患儿在血管抑制型和混合型时卧、立位的QTd及Pd未见差异(Pa>0.05)。6.VVS患儿在卧位、立位时QTd及Pd不存在男女性别差异(Pa>0·05)。结论VVS患儿卧位时更易发生心律失常;当体位改变时QTd、Pd明显受自主神经功能影响,表明该法对心脏自主神经功能的评价具有指导价值。     

血管迷走性晕厥儿童的心理因素

目的探讨血管迷走性晕厥(VVS)儿童的心理因素。方法选取2007年6-11月在中南大学湘雅二医院儿童晕厥专科门诊就诊或住院的不明原因晕厥或先兆晕厥儿童84例(VVS组)。男47例,女37例;年龄7～16岁[(11.01±2.00)岁]。将VVS儿童分为直立倾斜试验(HUTT)阳性组(n=43)和HUTT阴性组(n=41)。受试儿童均完成儿童抑郁障碍自评量表与儿童焦虑性情绪障碍筛查表,采用SPSS11.0软件进行分析。结果1.儿童抑郁障碍自评量表得分,VVS组儿童睡得很香、吃东西香及爱与家人交谈等项目得分显著低于全国常模组(Pa<0.05,0.01),VVS组患儿总是想哭、肚子痛、感到孤单、感到悲哀、感到烦恼得分及抑郁总分高于全国常模组(Pa<0.05,0.01)。HUTT阳性组患儿盼望美好事物和容易高兴起来得分高于HUTT阴性组(Pa<0.01),HUTT阴性组患儿生活没有意思得分高于HUTT阳性组(P<0.05)。2.儿童焦虑性情绪障碍筛查表得分,躯体化/惊恐、广泛性焦虑、分离性焦虑、社交恐怖、学校恐怖、焦虑量表总分与简明焦虑量表等得分VVS组显著高于全国常模组(Pa<0.01)。HUTT阴性组与HUTT阳...     

儿童血管迷走性晕厥与焦虑的关系研究

目的 探讨儿童血管迷走性晕厥(VVS)与焦虑的关系.方法 2007年6月至2007年11月在中南大学湘雅二医院儿童晕厥专科门诊就诊或住院的VVS患儿84例,其中男47例,女37例;年龄7～16岁,平均(11.01±2.00)岁.将VVS患儿分为直立倾斜试验(HUTT)阴性组(41例)和HUTT阳性组(43例),再将HUTT阳性组依临床症状分为头晕组与晕厥组.所有受试儿童完成儿童焦虑性情绪障碍筛查表(SCARED),用统计软件SPSS11.0进行数据分析.结果 (1)HUTT阴性组与HUTT阳性组SCARED得分比较:躯体化或惊恐、广泛性焦虑与焦虑量表总分HUTT阴性组高于HUTT阳性组,分离性焦虑、社交恐怖与学校恐怖得分HUTT阴性组低于HUTT阳性组,但差异均无统计学意义(P>0.05).(2)HUTT阳性组、阴性组与全国城市常模组(常模组)SCARED得分比较:躯体化或惊恐、广泛性焦虑、分离性焦虑、社交恐怖、学校恐怖与焦虑量表总分均明显高于常模组(P<0.05).(3)HUTT阳性头晕组与晕厥组SCARED得分比较:躯体化或惊恐、广泛性焦虑、分离性焦虑、社交恐怖及焦虑量表总分头晕组高于晕厥组,但差异无统计学意义(P>0.05);学校恐怖得分头晕组明显高于晕厥组(P<0.05).结论 儿童VVS中焦虑发生率高.儿童VVS躯体化或惊恐、广泛性焦虑、分离性焦虑、社交恐怖与学校恐怖普遍存在,但这些焦虑症状与HUTT结果关联不明显.HUTT阳性患儿中,没有晕厥病史的VVS患儿焦虑评分更高.提示心理因素如焦虑在儿童VVS发生、发展、治疗及预后中可能起重要作用.     

心率变异性分析在先天性心脏病合并阵发性室上性心动过速患儿中的意义

目的探讨自主神经系统活动在先天性心脏病(CHD)患儿阵发性室上性心动过速(PSVT)发生中的作用。方法选择无CHD的PSVT患儿50例(PSVT组)、伴CHD的PSVT患儿30例(伴CHD组)及同期健康儿童50例(对照组),回顾性分析、比较三组患儿24 h心率变异性(HRV)长程时域指标的差异。结果各项HRV长程时域指标即NN间期标准差(SDNN)、NN间期均值标准差(SDANN)、NN间期标准差均值(SDNN Index)、NN间期差值个数百分比(p NN50)、相邻NN间期差值均方根值(RMSSD)在三组间的差异有统计学意义(F=80.32~263.18,P均0.01)。与对照组比较,PSVT组的SDNN、p NN50、RMSSD均下降,差异有统计学意义(P0.05);SDANN、SDNN Index虽有下降,但差异无统计学意义(P均0.05)。伴CHD组与PSVT组比较,SDNN Index、p NN50及RMSSD下降,差异有统计学意义(P均0.05);SDNN、SDANN下降差异有统计学意义(P均0.01)。SDNN、p NN 50、RMSSD、SDANN、SDNN Index在伴CHD组与对照组两组间比较差异有统计学意义(P均0.01)。结论 PSVT患儿存在自主神经系统的失衡,以迷走神经功能降低为主,而伴CHD的PSVT患儿同时存在交感神经张力增高。     

β_1肾上腺素受体基因多态性与小儿血管迷走性晕厥的相关性研究

目的探讨β1肾上腺素受体(ADRB1)基因多态性Arg389Gly与小儿血管迷走性晕厥(VVS)发病的相关性。方法晕厥组为不明原因晕厥(unexplained syncope,UPS)患儿54例,其中男18例,女36例,平均11.8岁;对照组为同期健康体检儿童54例,其中男20例,女34例,平均11.2岁。入选病例均行直立倾斜试验(head-up tilt test,HUTT),根据HUTT结果,分HUTT阳性组即VVS组和HUTT阴性组,各组病例均应用聚合酶链反应PCR和基因测序方法检测ADRB1基因Arg389Gly多态性。结果健康儿童的等位基因Arg389和Gly389的频率分别为73.15%和26.85%,HUTT阳性患儿等位基因Arg389和Gly389的频率分别为66.67%和33.33%,HUTT阴性患儿等位基因Arg389和Gly389的频率分别为85.42%和14.58%;HUTT阳性患儿的Gly389等位基因频率明显高于HUTT阴性组患儿及健康对照儿(P<0.05)。HUTT阳性组共30例(55.6%),其临床分型中心脏抑制型6例(20.0%),混合型9例(30.0%),血管抑制型15例(...     

血管迷走性晕厥儿童心率变异性分析

目的探讨血管迷走性晕厥(VVS)儿童的心率变异性(HRV)。方法对27例不明原因晕厥患儿经直立倾斜试验(HUTT)检查阳性并诊断为VVS的HRV进行分析(研究组),并将29例健康儿童作为对照(对照组)。采用康泰TLC3000A12通道动态心电图分析系统描记未发生晕厥时24h心电图,分析时域指标和频域指标,应用SPSS11.0软件进行统计学处理。结果研究组低频功率(LF)与对照组比较明显降低(P<0.05);频域指标与时域指标在不同年龄段差别不明显(P>0.05);女性时域指标中SDANN及VLF、LF较男性明显降低(P<0.05);血管抑制型与混合型VVS的HRV指标差异不明显。结论VVS儿童基础自主神经功能发生改变;HRV主要受性别影响,而年龄影响不明显;HUTT不同反应类型VVS的HRV不存在差别。     

儿童血管迷走性晕厥QT离散度分析

目的 了解儿童血管迷走性晕厥QT离散度(QTd)的变化。方法 对24例血管迷走性晕厥患儿及18例健康儿童的QTd进行测量。结果 血管迷走性晕厥患儿QTd、QTcd、QTmax较对照组显著增大，差异具有显著性。结论 血管迷走性晕厥患儿QTd明显延长。QTd的分析对了解血管迷走性晕厥患儿的心电生理特点、辅助临床对晕厥患儿的鉴别诊断具有重要意义。     

儿童直立倾斜试验血流动力学变化与身高、体重及体质量指数的关系

目的 探讨儿童直立倾斜试验（HUTT）血流动力学变化与身高、体重及体质量指数（BMI）的关系.方法 收集2000年1月至2012年8月在中南大学湘雅二医院儿童晕厥专科 门诊就诊或住院的不明原因晕厥、头痛、头晕、胸闷、叹气等儿童1 906例,男964例,女942例,年龄2.00 ～ 17.92岁,平均10.84±2.97）岁.测量身高、体重,计算BMI.在取得受试者或监护人书面知情同意后,进行HUTT.根据HUTT结果及反应类犁分HUTT阴性组、体位性心动过速综合征（POTS）组、血管迷走性晕厥（VVS）血管抑制型组、VVS心脏抑制型组、VVS混合型组.结果 与HUTT阴性组相比,年龄在POTS组、VVS血管抑制型组、VVS混合型组较大（P＜0.05）,身高、体重、体表面积在POTS组、VVS血管抑制型组、VVS混合型组增加（P＜0.01）.与POTS组相比,体重、体表面积在VVS血管抑制型组、VVS混合型组降低（P＜0.05）,VVS心脏抑制型组与其他各组比较差异未见统计学意义（P＞0.05）.结论 儿童HUTT血流动力学变化与身高、体重及BMI存在一定关系.     

不同反应类型血管迷走性晕厥儿童的心率变异性

目的研究血管迷走性晕厥(VVS)儿童的心率变异性(HRV),探讨不同反应类型对VVS儿童HRV的影响。方法2003-01—2007-05在中南大学湘雅二医院晕厥专科诊治的不明原因晕厥(UPS)或接近晕厥儿童87例(晕厥组),其中男34例,女53例;年龄4～17(10.92±2.62)岁。匹配73例健康儿童为对照(对照组)。晕厥组与对照组儿童均行24h动态心电图(Holter)检查,将所记录数据输入TLC 3000A12通道动态心电图分析系统,程序自动分析结合人工干预生成HRV时域指标和频域指标。晕厥组儿童在Holter检查前后1d内行直立倾斜试验(HUTT)检查。用SPSS11.0软件进行数据统计分析。结果(1)晕厥组儿童HUTT阳性率62.1%(54/87);HUTT阳性儿童中男31.5%(17/54),女68.5%(37/54);血管抑制型38例(70.4%),混合型14例(25.9%),心脏抑制型2例(3.7%)。(2)与对照组比较,晕厥组儿童时域指标和频域指标均降低,其中SDNN、VLF(P<0.05)和TP、LF、HF(P<0.01)降低显著。(3)与对照组比较,HUTT阳性组儿童时域指标SDANN降低明显(P<0.05),其余时域指标和频域指标稍降低(P>0.05)。(4)血管抑制型与混合型儿童相比,各项时域指标均降低,以SDNN降低明显(P<0.05);频域指标TP、VLF、LF、HF稍降低(P>0.05)。结论VVS儿童基础自主神经功能异常,血管抑制型是最常见的反应类型,VVS儿童的HRV与反应类型有关。     

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

Resurgence of measles in Singapore: profile of hospital cases

OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (相似文献   

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孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.