冠心病家族史儿童脂质三角的研究

目的：了解有冠心病(CHD)家族史儿童脂质三角［低密度脂蛋白-胆固醇(LDL-C),高密度脂蛋白-胆固醇(HDL-C)和甘油三脂(TG)］有无异常。方法：对83例有冠心病家族史的儿童检测血浆TG,LDL-C和HDL-C浓度,计算LDL-C/HDL-C,以无CHD家族史的健康儿童作为对照。结果：与对照组比较,有CHD家族史的儿童血TG,LDL-C浓度明显增高［(1.46±0.63) mmol/L vs (0.84±0.43) mmol/L,(2.09±1.13) mmol/L vs (0.96±0.87) mmol/L］,HDL-C水平降低［(1.48±0.48) mmol/L vs (1.72±0.53) mmol/L］,LDL-C/HDL-C升高(1.71±1.29 vs 0.96±0.68)(P1.7 mmol/L及LDL-C/HDL-C>2.5的发生率明显增高(20.5% vs 1.2%)(P均0.05)。有早发CHD家族史的儿童血TG,LDL-C水平［(1.86±0.63),(3.12±1.32) mmol/L］高于无早发CHD家族史儿童［(1.34±0.58),(1.79±0.87) mmol/L］及对照组［(0.84±0.43),(0.96±0.87) mmol/L］。有早发CHD家族史者LDL-C/HDL-C(2.85±1.21)高于无早发家族史组(1.37±1.11)和对照组(0.96±0.68)(P均<0.01),HDL-C水平［(1.11±0.26) mmol/L］低于无早发家族史者［(1.59±0.47) mmol/L］和对照组［(1.72±0.53) mmol/L］。脂质三角异常发生率(52.6%)高于无早发家族史组(10.9%)和对照组(1.2%)(P<0.01)。结论：有CHD家族史的儿童存在脂质三角异常,以有早发CHD家族史儿童明显。提示儿童期脂质三角异常与CHD家族史关系密切,有CHD家族史儿童成年后发生CHD的危险性显著增高。     

Factor Analysis of Markers of Inflammation and Oxidation and Echocardiographic Findings in Children with a Positive Family History of Premature Coronary Heart Disease

The objective of this study was to determine the predictive factor of a combination of traditional and new risk factors for coronary heart diseases (CHDs) as well as the echocardiographic findings in children of parents suffering premature myocardial infarction in comparison with controls. Overall, 239 adolescents aged 12 to 18 years including 112 children of parents with premature CHD (<55 years) and 127 age- and sex-matched controls without such a family history. In addition to measuring anthropometric indexes and blood pressure, serum fasting blood sugar, lipid profile, C-reactive protein, Lpa, and oxidized LDL as well as carotid-intima media thickness and left ventricular mass were determined. Factor analysis showed that clusters of inflammatory factors and markers of oxidation as well as carotid-intima media thickness and left ventricular mass were correlated with each other and were associated with a positive parental history of premature CHD in youths. These findings complement the functional and structural changes in arteries of adults with a familial predisposition to CHD and underscore the importance of using a high-risk approach for primordial/primary prevention of CHD from early life and of considering the screening of children and siblings in the management of patients with premature CHD.     

Plasma Lipid and Apolipoprotein Levels in Children Hereditarily Predisposed to Coronary Heart Disease

ABSTRACT. Plasma lipids and apoproteins A-I, A-II and B levels were measured in 125 children whose fathers had coronary atherosclerosis (paternal coronary heart disease group), in 172 children with blood pressure lability and in 154 children selected at random from a representative subsample (reference). The paternal coronary heart disease group had, compared with the reference group, lower levels of high density lipoprotein cholesterol and apoprotein A-I, higher levels of low density lipoprotein cholesterol, triglycerides and apoprotein B, and also a higher ratio of apoprotein B to apoprotein A-I. The paternal coronary heart disease group subjects with blood pressure lability had the most pronounced changes in the lipoprotein spectrum, which were similar to atherogenic changes in the lipoprotein profile, characteristic of patients with coronary atherosclerosis.     

Angiographical Studies on the Distribution of Coronary Arteries in Children | Part 2. Caliber and Distribution of Coronary Arteries in Congenital Heart Disease

In order to determine the normal size of various branches of the coronary arteries and to clarify the etiological factors that determine the dominant patterns of coronary arteries (dominance) and the relationship between the growth of the coronary arteries and the ventricles, the caliber and the distribution of major coronary arterial branches were measured by selective coronary arteriography in 41 cases with normal hearts and 49 cases with congential heart disease. In normal hearts, the incidences of right dominant(R), balanced(B) and left dominant(L) types were 78%, 15% adn 7%, respectively. In congenital heart disease, the incidences of R, B and L types in right ventricular loading hearts (RV group) were 94%, 0% and 6%, respectively and those in left ventricular loading hearts (LV group) were 58%, 27% and 15%, respectively. The ratios of left main trunk and proximal right corornary in size (LMT/RCA ratio) in RV group weremuch smaller and LMT/RCA ratios in RV group were much larger than those in normal hearts. these results indicated that in the different types of heart anomalies the loading of pressure and volume to the ventricles varies in grade, and subsequently induced the hypertrophy of the loaded parts. It was thought that the resultant hypertrophy also affected the growth and dominance of the coronary arteries.     

冠状动脉造影在川崎病冠状动脉损害检查中的作用

目的　通过对疑有冠状动脉损害川崎病 (KD) 1 2例患儿行经皮冠状动脉造影 ,旨在准确了解冠状动脉损害情况 ,以指导临床对KD患儿的后期治疗及管理。方法　在静脉麻醉下采用Sedinger法穿刺右股动脉 ,采用Judkin法将冠状动脉造影管送达冠状动脉口 ,推注非离子造影剂 ,同时行电影摄影记录。结果　接受冠状动脉造影检查 1 2例患儿中 9例冠状动脉异常 ,其中左冠状动脉和 (或 )其分支受累 5例 ,右冠状动受累、左右冠状动脉同时受累各 2例。与超声心动图结果不尽一致。结论　冠状动脉造影可明确显示冠状动脉损害位置、形状、数目及病变程度 ,是一种安全、有效的检查方法。     

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。     

高脂血症对儿童颈动脉弹性功能的影响

目的探讨儿童颈动脉弹性功能的影响因素及高脂血症对其影响。方法采用Aloka超声血管回声跟踪技术,测定57例正常儿童和23例高脂血症儿童的颈动脉弹性系数(Eρ)、硬度指数(β)及顺应性(AC),并对可能的影响因素进行相关分析。结果在正常健康儿童中,性别对于颈动脉的弹性功能没有影响,年龄、收缩压、脉压与颈动脉Eρ、β呈正相关,而与AC呈负相关。高脂血症组与正常组儿童比较,Eρ、β明显增高,AC值明显降低。结论高脂血症可以导致儿童颈动脉弹性功能的降低,年龄和血压也是儿童颈动脉弹性功能的影响因素。     

先天性心脏病患儿出生月份的分布规律

目的 探讨先天性心脏病(CHD)患儿出生月份的分布规律.方法 对2003年1月-2006年12月在本院接受检查或治疗的5 070例CHD患儿的出生月份分布情况进行统计分析,同时以本院产科2001-2006年出生的6 627例健康新生儿的出生月份分布情况作为背景对照,分析CHD患儿的出生月份分布规律.数据采用SPSS 12.0软件包进行χ2检验.结果 本组5 070例CHD患儿中,出生人数分别为1月份444例(8.8%),2月份432例(8.5%),3月份384例(7.6%),4月份339例(6.7%),5月份390例(7.7%),6月份393例(7.8%),7月份414例(8.2%),8月份489例(9.6%),9月份498例(9.8%),10月份492例(9.7%),11月份396例(7.8%),12月份399例(7.8%).将CHD患儿出生月份分布情况与6 627例新生儿的出生月份分布情况进行对比分析,CHD患儿在8～10月份出生者较高,共1 479例(29.1%),2～4月份出生者最低,为1 155例(22.8%),二者比较有统计学差异(P＜0.01).6 627例新生儿在全年各月份的出生人数基本在同一水平,CHD患儿与健康对照组出生月份比较有统计学差异(P＜0.01).结论 在8～10月出生者可能与发生CHD存在一定关联性.     

先天性心脏病患儿的免疫功能状态

目的：先天性心脏疾病(CHD)患儿易发生感染,有作者认为CHD可能是DiGeorge综合征(DGS)的一部分,CHD患儿对感染的易感性与其存在不同程度的免疫缺陷有关。本文探讨CHD患儿有无免疫功能缺陷,结合文献讨论CHD与DGS的关系。方法：通过胸部X片回顾性观察因患肺炎而住院的72例单纯性和34例复杂性CHD新生儿胸腺影的大小,50例同日龄肺炎新生儿作为对照。检测28例学龄前期CHD患儿外周血淋巴细胞亚群、淋巴细胞增殖功能及外周血单个核细胞(PBMC)白细胞介素-4(IL-4)和干扰素-γ(IFN-γ)mRNA表达情况及培养上清中IL-4和IFN-γ水平,血浆IgG、IgA、IgM及C3水平。20例同年龄健康儿童作为对照。结果：所有新生儿胸片均可见到胸腺影,单纯性和复杂性CHD新生儿胸腺影大小与肺炎新生儿比较差异均无显著性(P>0.05);学龄前期CHD患儿外周血CD3+,CD4+,CD8+,CD19+及CD16+CD56+T细胞和CD4+/CD8+与对照组差异无显著性(P>0.05);血浆免疫球蛋白及C3水平与对照组比,差异也无显著性(P>0.05);PBMC加植物血凝素(PHA)和脂多糖(LPS)刺激后的每分钟脉冲数、PBMC培养上清中IL-4,IFN-γ水平和mRNA表达与对照组比较差异无显著性(P均>0.05)。结论：并非所有CHD患儿均伴有胸腺发育不全或免疫功能缺陷,CHD患儿易感染不一定是先天性免疫功能低下的表现。     

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。     

婴幼儿先天性心脏病并心内膜弹力纤维增生症的临床特点

目的研究婴幼儿先天性心脏病(CHD)并心内膜弹力纤维增生症(EFE)的临床及实验室特点,诊断和治疗。方法对本院收治婴幼儿CHD并EFE患儿的临床及实验室检查资料进行回顾性分析、总结。结果3166例CHD中21例(0.66%)并EFE,其中动脉导管未闭(PDA)5例,房间隔缺损(ASD)6例,室间隔缺损(VSD)6例,PDA VSD 1例,ASD VSD 2例.法洛四联症1例。临床有不同程度心力衰竭,多伴肺炎,心脏增大较明显,起病绝大多数较急,病情多较重.预后较差。结论CHD是引起EFE的原因之一,但不除外其他原因尤其是病毒感染导致EFF;疾病预后可能与心脏扩大程度、心力衰竭程度、并发症严重程度及治疗及时与否有关。     

儿童先天性心脏病脑功能损害评估

相当一部分先天性心脏病患儿存在不同程度的脑功能损害,其病因包括先天性因素和后天获得性因素.早期准确地对先天性心脏病患儿的脑损伤进行评估相当重要.近年来在神经影像学、神经病理学及精神运动发育和智能评估等方面均取得了新进展,能更好地指导临床医师及时干预,使先天性心脏病患儿的脑功能损害程度减少到最小,提高先天性心脏病患儿的生存质量.     

Development of Personality and Intelligence in Children with Congenital Heart Disease

One hundred and twenty-eight children with congenital heart disease (CHD) and 89 healthy controls were compared according to their development of personality and intelligence. Presence of physical disability in daily life was the independent variable for dividing children with CHD into two groups: children with physical handicap due to CHD (n = 77) have lower IQ scores than healthy children. Unexpectedly, in the parents' opinion they show no difference in behaviour patterns. On introspective examination, these cardiac patients showed an increased feeling of inferiority and of basic anxiety and a more impetuous behaviour as their way of self-protection, but a reduced need for independence due to parental overprotection was not confirmed. None of these observations could be detected in children with CHD who are physically fit (n = 51).     

Posterior Descending Coronary Artery Flow Reserve Assessment by Doppler Echocardiography in Children with and without Congenital Heart Defect Comparison with Invasive Technique

To evaluate whether transthoracic Doppler echocardiography can reliably measure coronary flow velocity and coronary flow velocity reserve (CFVR) in the posterior descending coronary artery (PD) in children, we examined 32 patients who had congenital heart disease (ventricular septal defect in 10, tetralogy of Fallot in 6, tricuspid atresia in 3, double-outlet right ventricle in 2, patent ductus arteriosus in 2, and aortic valve stenosis in 2) and 7 patients who had a history of Kawasaki disease without stenosis or aneurysm formation of the coronary artery. Average peak flow velocity (APV) in the PD was measured by transthoracic Doppler echocardiography at the time of intracoronary Doppler study. CFVR was defined as the ratio of hyperemic to basal APV. Clear envelopes of basal and hyperemic APV in the PD were obtained in 23 of 32 patients by transthoracic Doppler echocardiography. APV obtained from transthoracic Doppler echocardiography correlated highly with that from the Doppler guidewire method (r = 0.91). The mean difference between transthoracic Doppler echocardiography and the Doppler guidewire method was 0.1 ± 2.9. There was an excellent correlation between transthoracic Doppler echocardiography and the Doppler guidewire method for the measurements of CFVR (r = 0.84). The mean difference between transthoracic Doppler echocardiography and Doppler guidewire was –0.016 ± 0.198. Noninvasive measurement of coronary flow velocity and CFVR in the PD using transthoracic Doppler echocardiography accurately reflects invasive measurement of coronary flow velocity and CFVR by the Doppler guidewire method in pediatric patients with various heart diseases.     

Beraprost Therapy in Children with Pulmonary Hypertension Secondary to Congenital Heart Disease

We present a report on children with severe pulmonary hypertension secondary to congenital heart disease who received 6 months of beraprost therapy. The children had an increase in intracardiac left-to-right shunt and a reduction of the pulmonary-to-systemic vascular resistance ratio, whereas the pulmonary artery pressure was not significantly changed.     

SCREENING SYSTEM: The Heart Disease Screening System for School Children in Japan and Its Results

The heart disease screening system for school children in Japan is useful for early detection of children with heart disease, and for their daily health care. In particular, the heart disease screening system using an electrocardiogram and/or phonocardiogram is an excellent one for finding atrial septal defect, pulmonic stenosis, idiopathic cardiomyopathy and arrythmia. Recently, computer-assisted electrocardiogram and phonocardiogram automatic analysis equipment for heart disease screening of school children has been developed, and a screening system using this equipment has been instituted.     

先天性心脏病并心力衰竭患儿血浆氨基末端脑利钠肽前体水平变化的临床意义

目的 观察先天性心脏病(CHD)并心力衰竭(HF)患儿血浆氨基末端脑利钠肽前体(NT-proBNP)水平的变化,分析NT-proBNP与HF严重程度的关系及在心功能评估中的价值.方法 选择本院2008年6月-2011年6月住院CHD患儿46例,并根据纽约大学儿童HF指数(NYU PHFI)的评价标准将其分成3组:无HF组(15例)、轻度HF组(16例)、中重度HF组(15例).选取同期25例健康儿童作为健康对照组.分别留取各组儿童空腹静脉血2 mL,应用ELISA法测定其血浆NT-proBNP水平,同时测定其左心室射血分数(LVEF)、二尖瓣舒张早期流速峰值/二尖瓣舒张晚期流速峰值(E/A值).分析其血浆NT-proBNP水平与HF严重程度及LVEF、E/A值的相关性.结果 血浆NT-proBNP水平与HF严重程度呈明显正相关(r=0.82,P＜0.01),HF程度越重,血浆NT-proBNP水平升高越显著;中重度HF组NT-proBNP水平显著高于轻度HF组(P＜0.01),轻度HF组NT-proBNP水平显著高于无HF组(P＜0.01),无HF组NT-proBNP水平显著高于健康对照组(P＜0.05).血浆NT-proBNP水平与LVEF呈负相关(r=-1.20,P＜0.01).结论 血浆NT-proBNP水平随着HF的严重程度增加而升高,检测血浆NT-proBNP对评估CHD并HF患儿病情严重程度及判断预后具有重要临床意义.     

先天性心脏病患儿的血管紧张素转换酶基因多态性研究

目的对先天性心脏病(CHD)患儿血管紧张素转换酶(ACE)基因多态性的分析,探讨ACE基因插入/缺失(I/D)多态性与CHD的关系,寻找与CHD发病有关的易感基因。方法68例CHD患儿为观察组,64例非CHD患儿为对照组。应用饱和酚-氯仿法提取白细胞DNA,应用PCR技术对标本ACE基因进行扩增,扩增产物采用2%琼脂糖凝胶电泳,在紫外光下根据迁移率判定ACE不同基因型。结果观察组ACE基因为插入纯合型(II)12例,占17.65%;缺失纯合型(DD)32例,占47.06%;杂合子(ID)24例,占35.29%。对照组ACE基因为20例,占31.25%;DD型10例,占15.63%;ID 34例,占53.12%。结论CHD发病与ACE基因多态性有密切相关性,DD基因型发病相对危险度高,DD基因型可能为CHD易患基因型,D等位基因可能为CHD易感基因。     

血管内皮生长因子在发绀型先天性心脏病中的变化

目的观察血管内皮生长因子(VEGF)在发绀型先天性心脏病(CCHD)中变化及其与病情严重程度关系。方法采用ELISA方法检测和比较20例CCHD和18例健康儿童的血清VEGF,并探讨CCHD患儿血清VEGF与血红蛋白及动脉血氧饱和度间的关系。结果CCHD患儿组VEGF明显高于正常对照组,差异有显著性[(164.65±55.73)ng/Lvs(25.56±10.67)ng/L,t=10.41 P<0.01];VEGF水平与动脉血氧饱和度呈明显负相关(r=0.602 P<0.01);VEGF水平与血红蛋白浓度呈明显正相关(r=0.795 P<0.01)。结论CCHD患儿血清VEGF浓度升高,并与病情严重程度相关,提示VEGF在CCHD病理生理中可能起重要作用。     

川崎病患者易早发动脉粥样硬化

川崎病是一种急性自限性全身性血管炎,其心血管并发症,尤其是冠状动脉损害(冠状动脉扩张、冠状动脉瘤形成、冠状动脉狭窄甚至闭塞等)使其受到了临床医师,尤其是儿科临床医师的广泛关注.川崎病首次被认识至今已经40余年,随着那些最早的川崎病患者逐渐迈入中年,关于川崎病长期预后的研究引起了临床工作者的极大兴趣.虽然动脉粥样硬化性心血管疾病临床表现多出现在中老年期,但近年来其被认为是一种病变始于儿童时期,并在各种危险因素的作用下逐渐进展的疾病.越来越多的研究发现,川崎病尤其是伴有冠状动脉损害的川崎病可能是早发动脉粥样硬化的危险因素.