先天性心脏病患儿脑干听觉诱发电位的研究

目的：评价先天性心脏病（先心病）对患儿脑干发育的影响。方法：研究了先心病患儿脑干听觉诱发电位（ＢＡＥＰ）反应，并设正常儿为对照组。结果：年龄＜１２个月，青紫型先心病（ＣＣＨＤ）患儿Ⅰ波潜伏期（ＰＬ）正常，Ｉ－Ｖ波峰间潜伏期（ＩＰＬ）较正常儿显著延长，非青紫型先心病（ＮＣＣＨＤ）患儿Ｉ波ＰＬ、Ｉ－Ｖ波ＩＰＬ均显著延长；４～６岁者，ＮＣＣＨＤ和ＣＣＨＤ无反复缺氧发作者Ｉ波ＰＬ和Ｉ－Ｖ波ＩＰＬ与正常儿无差异，ＣＣＨＤ反复缺氧发作者Ｉ－Ｖ波ＩＰＬ较正常儿显著延长，异常率２３％。结论：先心病可延迟婴儿期脑干发育，缺氧发作可损害脑干功能，对缺氧发作者应尽早进行干预     

先天性心脏病患儿血清甲状旁腺素水平观察

目的探讨先天性心脏病(CHD)患儿甲状旁腺功能。方法用放射免疫法(IRMA)测定18例学龄前期CHD患儿血清甲状旁腺素(parathyrin,PTH)水平,并测定其血清Ca2 、Mg2 浓度;17例门诊体检的健康儿童作为对照。结果CHD患儿血清PTH水平(14.87±6.02ng/L)较对照组(19.68±5.76ng/L)明显降低,两者比较差异有显著性(P<0.05),CHD组中有8例(44.4%)患儿血清PTH<10ng/L,而对照组均高于10ng/L;CHD患儿血清Ca2 和Mg2 较对照组略低,但无统计学意义(P>0.05)。结论CHD患儿甲状旁腺素分泌不足,存在亚临床甲状旁腺功能低下。     

先天性心脏病外科修补术后患儿心理行为研究

目的 探讨先天性心脏病(CHD)患儿的心理行为状况,并对照研究外科修补手术与介入封堵治疗对CHD患儿心理行为的影响.方法 应用Aehenbach儿童行为量表分别对79例行外科修补手术和82例介入治疗的CHD患儿在术前和术后1年进行心理行为检测.选取80名与CHD患儿一般特征相匹配的健康儿童为对照组.结果 CHD儿童术前心理行为异常检出率及心理行为总粗分均显著高于对照组(P＜0.01),心理行为粗分均值亦高于对照组(P＜0.05).男孩主要表现为抑郁、交往不良、体诉、社会退缩、违纪及攻击方面,女孩主要表现为抑郁、社会退缩、体诉、违纪方面.术后1年测试,男、女二治疗组心理行为总粗分分别显著低于治疗前(P＜0.01),男、女二介入组总粗分与对照组比较差异无统计学意义;外科组男孩术后总粗分及攻击行为粗分分别高于介入组(P＜0.05),外科组女孩术后心理行为总粗分及抑郁、社会退缩和违纪行为粗分分别高于介入组(P＜0.05).心理行为因子异常检出率与病程呈正相关,病程越长检出率越高.结论 CHD患儿存在着明显的心理行为问题,宜早期治疗.与介入治疗相比,外科修补术后较长时间内患儿仍存有明显的心理行为问题,应引起更多重视.     

手术前后先天性心脏病患儿心率变异性的非线性动力学分析

目的 探讨心率变异（ＨＲＶ）性的非线性动力学分析在预测先天性心脏病术后风险中的应用。方法 应用非线性动力学方法和传统的频域方法分析两组先天性心脏病－「室间隔缺损（ＶＳＤ）和法洛四联症（ＴＯＦ）患儿术后心率变异的变化。结果 ⑴术前两组患儿ＨＲＶ频域及非线性动力指标均无明显差异（Ｐ〉０．０５）;⑵两组患儿术后第１天ＨＲＶ频域和非线性动力学指标较术前有明显改变（Ｐ〈０．０５,Ｐ〈０．０１）;⑶术后两组Ｈ     

先天性心脏病与免疫缺陷

先天性心脏病(先心病)患儿易患某些严重的感染,特别是呼吸道感染,感染性心内膜炎及脑脓肿。习惯上认为系血流动力学和机械性因素与之有关。然而我们最近证实,先心病儿有程度不等的免疫缺陷。25%的先心病儿免疫球蛋白水平低下,而某些园锥总干畸形(conotruncal malformation)的患儿缺乏淋巴细胞。从DiGeorge综合征中首先认识了     

先天性心脏病的遗传率调查

许多作者认为先天性心脏病（先心病）是多基因遗传病，呈家族性发病，但我们在临床工作中发现患者的家族中发病率并不高。自1981年以来注意收集此类患儿进行遗传率的调查和研究，现报道如下．资科和方法1981年～1990年的10年间，共发现单纯先心病112例，其中住院和普通门诊患儿73例，儿保门诊和病残儿童鉴定门诊患儿39例，尚有1例属常染色体异常的多畸形综8征（21-三体）和2例弃婴未包括在内。均经全面体检及实验室检查（B超、心导管术、心脏X线、心音图等）确诊为先心病，而未发现有其它畸形同时存在。20例经外科手术矫治证实。对本组患儿…     

先天性心脏病患儿血浆内皮素及降低素基因相关肽的临床研究

目的 了解先天性心脏病患儿血浆降钙素基因相关肽（ＣＧＲＰ）及血浆内皮素（ＥＴ）的变化。探讨其在先天性心脏病继发的病理生理改变中的作用及其临床意义。方法 左向右流分型行鱿性心脏病患儿２９例,其中１１例成功地行修补术,正常对照组２５例。用放射免疫均相竞争法分别检测血浆ＣＧＲＰ及ＥＴ水平。结果 先天性心脏病患儿术前血浆ＣＧＲＰ较对照组低（Ｐ〈０．０１）,而ＥＴ较对照组高（Ｐ〈０．０１）;患儿术后血浆ＣＧ     

先天性心脏病儿童的心理行为研究

先天性心脏病是儿童期常见病之一,它不但影响了儿童的躯体健康,对儿童的心理行为也有着明显影响。本文综述了先天性心脏病儿童的智能发育、情绪状态、行为问题、自我意识及社会适应等诸方面的研究进展,分析了疾病类型、治疗方法及家庭环境等因素对患儿心理行为发育的影响,探讨了进行干预的方法和疗效。     

韧粘素在先天性心脏病合并肺高压患儿肺血管中的表达

为探讨韧粘素（ＴＮ）在先天性心脏病（先心病）合并肺高压发病机制中的作用及其其调节因素,采用免疫组化方法观察不同肺血清和／或不同肺动脉压力下,先心病患儿肺小动脉中ＴＮ的表达情况。图像分析结果表明,高肺血流伴肺高压时ＴＮ表达阳性面积比例明显高于高肺血流不伴肺高压和对照组（Ｐ均〈０．０１）,ＴＮ表达与肺动脉压力升高显著相关（ｒ＝０．７５,Ｐ〈０．０１）,与肺血流增多无关（ｒ＝－０．４８,Ｐ〉０．０５）。     

肺血流量对血浆内皮素—1水平影响的研究

为研究肺血流量对血浆ＥＴ－１水平的影响以帮助阐明肺高压的发病机制,本实验将先心患儿分为无高肺血流伴肺动脉压力正常的对照组及高肺血流组,高肺血流组再分为伴肺高压组及肺动脉压力正常组。     

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目的评价经食管超声心动图(TEE)对指导小儿先天性心脏病(先心病)外科手术和介入治疗的应用价值及其安全性。方法2000年11月至2005年1月,在上海市复旦大学附属儿科医院心血管中心接受外科开胸手术或经导管介入治疗的先心病患儿中有317例进行了TEE检查,年龄2个月至17岁6个月(平均4.7岁)。采用HP/SONOS2500超声诊断仪,频率5.5～7.5MHz双平面经食管探头,全麻状态下进行。结果与术前经胸超声心动图(TTE)比较,术前TEE检查对诊断作出修正或补充者51例(16.1%),其中因此而修正了手术治疗方案25例(7.9%)。术后TEE检查发现有并发症或残余问题57例(18.0%),其中8例(2.5%)因此立即再次手术。所有病例均未因TEE检查而引起并发症。结论TEE可安全地应用于小儿先心病围术期检查,对术前诊断做出修正或补充,术后可及时发现并发症或残余问题。     

孕妇感染弓形虫妊娠结局及对其子女生长发育的影响

优生、优育、优教,实现人口素质的提高是我国的基本国策。孕期感染弓形虫是引起胎儿发育障碍的重要因素,有关孕期感染弓形虫的危害,对出生儿童发育的影响,各地调查结果不尽一致。为了解孕期感染弓形虫后出生儿童体格发育、智力情况,笔者对河北省保定市1990年2月～1996年2月孕妇感染弓形虫后妊娠结局及分娩儿进行了追踪观察,现将结果报告如下。1材料和方法1.1对象选择保定市级以上医院1990年2月～1996年2月建保健册的孕妇,年龄21～34岁,平均26岁。孕妇分感染组322例(孕期弓形虫IgM或CA g阳性)和非感染组147例(同期弓形虫检查阴临床儿科杂志…     

Long-term growth of children with congenital heart disease: a retrospective study

This retrospective study evaluates long-term growth of children with congenital heart disease (CHD) and looks for possible relationships between postsurgical catch-up growth and both severity of preoperative growth failure and operation age. Growth data of 123 children with isolated CHD were available. Mean z-scores and 95% confidence intervals for weight, height and weight-for-height were plotted for age-periods as well as for pre- and postoperative periods. Growth of children with a large VSD or a Tetralogy of Fallot was most abnormal and improved but did not normalize after operation. Catch-up growth for length was strongly correlated with severity of the preoperative growth failure ( r = 0.92, p < 0.05) but not with operation age ( r = 0.20, NS). We conclude that surgical correction results in catch-up growth for most individuals. Catchup growth is positively correlated with the severity of the initial growth disturbance and not with age at the moment of surgical correction.     

Auditory brainstem responses in children with congenital heart disease

BACKGROUND: Cyanotic congenital heart diseases usually lead to growth and developmental delay in children due to chronic hypoxemia and undernourishment that may affect the central nervous system. The auditory brainstem responses are determined to assess the maturation and function of the brainstem. Therefore, we used the auditory brainstem responses to investigate the effect of cyanotic congenital heart diseases on brainstem maturation. METHODS: The auditory brainstem responses were investigated in 45 children (23 cyanotic, 22 acyanotic) with congenital heart diseases and compared with the results of 30 healthy counterparts (all children were aged between 2 months and 15 years). RESULTS: The results of auditory brainstem responses were similar in acyanotic patients and in normal children. The cyanotic patients under 1 year of age had more prolonged I-V interpeak latencies than those of control and acyanotic patients (P < 0.05). There was no difference between all groups older than 1 year of age. In cyanotic children, I-V interpeak latencies showed significant negative correlation with arterial oxygen saturation and partial oxygen pressure (P < 0.05). CONCLUSIONS: Cyanotic congenital heart diseases may cause significant retardation on brainstem maturation due to chronic hypoxemia, especially in infants under 1 year of age, whereas acyanotic congenital heart diseases have no effect on auditory brainstem responses.     

IgG subclass deficiency in children with congenital heart disease

This study of 66 children with congenital heart disease found 26 (39%) with IgG subclass deficiency, the majority being of the IgG₄ isotype. Conventional immunoiogical assessment (IgG, IgA, IgM, T cell) revealed 21 (32%) with immunodeficiency, while inclusion of IgG subclass assessment revealed a total of 35 (53%) of the 66 children had immuno-deficiency. Children with conotruncal lesions appeared to be predisposed to immunodeficiency affecting T cells and IgG subclasses (especially IgG₄) while those with shunt and stenotic lesions had a broad spectrum of immunoglobulin deficiencies. There was significant correlation between immunodeficiency and proneness to infection in these children (p < 0.01). These results suggest that immunodeficiency is a frequent occurrence in children with congenital heart disease, and that IgG subclass measurements should be added to the diagnostic work-up.     

Fibrinogen half-life in children with cyanotic congenital heart disease

Fibrinogen half-life was determined in 3 healthy individuals and in 17 children with cyanotic congenital heart disease. Fibrinogen half-life was normal in the healthy individuals and shortened in 9 of the 17 patients. The simultaneously performed coagulation tests were often pathological but did not lead to a clear diagnosis of the haemostatic defect and did not correlate with the fibrinogen half-life. Significant correlations were found between fibrinogen half-life and red cell count (P<0.001), packed cell volume (P<0.001), mean corpuscular volume of the red cell (P<0.001), platelet count (P<0.01), aortic oxygen saturation (P<0.001), base excess (P<0.01), and maximal amplitude of the thrombelastography (P<0.001). The results indicate a definite relationship between the hypoxaemia and the frequently observed disturbance of haemostasis in cyanotic heart disease. The chronic hypoxaemia causes a partly compensated intravascular coagulation with increased fibrinogen metabolism.Supported by Land Nordrhein-Westfalen, Ministerium für Wissenschaft und Forschung     

先天性心脏病儿童神经发育异常的预测

相当一部分先天性心脏病患儿存在神经系统发育异常。多种生化及物理学检查指标可用于预测不良的神经发育预后,有助于早期干预和改善先天性心脏病所致的神经发育障碍。近年来,关于先天性心脏病神经发育异常的研究在实验室检测、神经物理学检查、围手术期处理等方面均取得了新进展。文章就预测先天性心脏病神经发育异常的相关因素进行综述。