先天性心脏病患儿介入封堵术前后血浆血管紧张素Ⅱ和血清一氧化氮水平的变化

目的 观察先天性心脏病(CHD)介入封堵术前后血浆血管紧张素Ⅱ(AngⅡ)和血清一氧化氮(NO)水平的变化,探讨其在CHD并肺动脉高压(PH)中的作用及其意义.方法 选取90例CHD患儿.均经体检、辅助检查及心脏彩色B超确诊.其中单纯房间隔缺损40例.单纯室间隔缺损30例,单纯动脉导管未闭20例.依据心导管测压,将90例CHD患儿分为3组:中重度PH组(肺动脉收缩压6.66 kPa)30例和轻度PH组(3.99 kPa<肺动脉收缩压<6.66 kPa)30例、无PH组(肺动脉收缩压<3.99 kPa)30例.3个亚组的年龄及性别无统计学差异.分别采用放射免疫法和硝酸还原酶法测定各组术前、术后24 h、术后1、3、6及12个月血浆AngⅡ及血清NO水平.另选取年龄及性别与之相匹配的健康体检儿童30例作为健康对照组,用同样方法检测其血浆AngⅡ及血清NO水平.结果 介入封堵术前,CHD并PH组患儿血浆AngⅡ水平明显高于CHD无PH组(Pa<0.01);CHD并PH组患儿血清NO水平均明显低于CHD无PH组(Pa<0.01).CHD患儿介入封堵术24 h后,血浆AngⅡ逐渐下降,至12个月下降接近正常水平;CHD患儿介入封堵术24 h后,血清NO开始逐渐上升,至12个月上升接近正常水平.AngⅡ与肺动脉收缩压呈显著正相关(r=0.811 P<0.01);NO与肺动脉收缩压呈负相关(r=-0.421 P<0.05).结论 AngⅡ及NO可能参与CHD的发生、发展过程;血浆Ang Ⅱ及血清NO水平可作为判定其CHD患儿程度的参考指标之一.     

先天性心脏病伴肺动脉高压患儿血清ghrelin的表达及意义

目的探讨血清ghrelin在先天性心脏病(CHD)伴肺动脉高压(PAH)患儿中的表达水平及意义。方法选择2009年5月至2012年2月住院手术治疗的CHD患儿50例,依据肺动脉收缩压分为无PAH组(21例)、轻度PAH组(17例)和中重度PAH组(12例);另选健康儿童为对照组(15例)。测量各组儿童身高和体质量并计算体质指数(BMI),双抗体夹心ELISA法测定其血清ghrelin和内皮素-1(ET-1)水平。结果与对照组比较,各组CHD患儿的ghrelin和ET-1水平升高,差异均有统计学意义(P<0.01);与无PAH组比较,轻度和中重度PAH组患儿的ghrelin和ET-1水平升高,差异均有统计学意义(P<0.01);轻度和中重度PAH组患儿的血清ghrelin水平与血清ET-1水平和肺动脉收缩压均成正相关(r=0.577~0.840,P<0.05)。结论 Ghrelin在CHD伴PAH患儿中的表达水平增高,可能与PAH有密切关系。     

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。     

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。     

先天性心脏病合并肺动脉高压患儿手术前后血硫化氢及血红蛋白氧合酶-1变化的意义

目的探讨先天性心脏病(CHD)合并肺动脉高压(PH)患儿手术前后硫化氢(H2S)及血红蛋白氧合酶-1(HO-1)的变化及其临床意义。方法选取河北医科大学第四医院心脏外科收治的48例左向右分流的CHD患儿,均在复合麻醉中成功实施了根治手术。48例患儿行心脏多普勒术前测压,按照肺动脉收缩压高低(PASP)分为3组:无PH组(PASP<3.990kPa)15例,轻度PH组(PASP3.990～6.517kPa)15例,中重度PH组(PASP≥6.650kPa)18例。于术前、术后1h及术后24h采集各组患儿桡动脉血标本,用吸光度法测定血浆H2S水平,用双波长分光光度法测定血清HO-1活性。数据分析采用重复测量数据的方差分析、多因素方差分析、直线相关分析。结果各组患儿术后H2S水平呈上升趋势,术后24h与术前比较差异均有统计学意义(Pa<0.05)。术前、术后1h及术后24h,中重度PH组、轻度PH组血浆H2S水平均显著低于无PH组(Pa<0.05),中重度PH组血浆H2S水平均显著低于轻度PH组(Pa<0.05);术前、术后1h、术后24h,3组患儿血清HO-1活性比较差异无统计学意义;3组患儿血清HO-1活性组间...     

一氧化氮治疗小儿急性呼吸衰竭和肺动脉高压

用我科自行研制的NO吸入装置对5例急性肺损伤伴呼吸衰竭(ALI ARF)和5例先天性心脏病伴肺动脉高压(CHD PH)患儿进行NO吸入治疗后,气体交换和血流动力学参数变化的观察,结果显示:ALI ARF组PaO_2/FiO_2上升48±17(54±15%)(T=4.52,P<0.01),氧合指数(OI)下降9±3(26±6%)(T=4.63,P<0.01),3例ARDS的 V_D/V_t下降5±1%(T=5.00,P<0.05);CHD PH组平均肺动脉压(mPAP)下降17±10%(T=5.345,P<0.01),肺血管阻力下降18±11%(T=5.432,P<0.01)。结论:NO吸入可改善ALI ARF患儿的动脉氧合,降低CHD PH患儿的肺动脉高压。     

左向右分流型先天性心脏病患儿血清肿瘤坏死因子-α的水平变化

目的 探讨不同肺动脉压水平下的左向右分流型先天性心脏病(CSID)患儿血清肿瘤坏死因子(TNF)-α水平的变化,进一步明确TNF-α与CHD伴肺动脉高压(PH)的关系.方法 利用酶联免疫吸附法测定48例CHD患儿(其中无PH、轻度PH、中度PH、重度PH各12例)在不同时间点静脉血清中TNF-α的水平变化.结果 在无PH组中,TNF-α在不同时间点的变化差异无显著性(P>0.05);中度PH组、轻度PH组术前TNF-α水平高于术后,在手术修补后,随着病情的好转,TNF-α水平逐渐下降,尤以术后第7天降低更为明显(P<0.05).在同一时间点但在不同肺高压水平下,重度PH组手术前TNF-α明显高于中度PH组、轻度PH组(P<0.01);在体外循环开始和修补术结束时,重度PH组TNF-α明显高于中度PH组、轻度PH组及无PH组(P<0.05);在手术后第1天,重度PH组TNF-α高于轻度PH组及无PH组(P<0.05);在手术后第4天,重度PH组TNF-α高于无PH组(P<0.01);在手术后第7天,重度PH组TNF-α高于中度及无PH组(P<0.05).在手术前后不同时间点,随着缺损的修补与病情好转,TNF-α在术后不同时间点均明显下降,尤以重度及中度PH组下降明显,但无PH组无显著变化.结论 TNF-α与CHD患儿PH的发生有关,通过检测TNF-α水平变化可估测CHD伴PH程度,判定手术指征及预后.     

先天性心脏病伴肺动脉高压患儿血清血管内皮生长因子和一氧化氮水平变化的研究

目的 探讨血管内皮生长因子(VEGF)与一氧化氮(NO)在先天性心脏病(CHD)伴肺动脉高压(PH)形成和发展中的作用及二者之间的关系.方法 采用酶联免疫吸附法和分光光度法分别检测25例CHD伴PH(PH组)及16例CHD不伴PH(对照组)患儿静脉血血清VEGF和NO的含量,并进行比较分析.结果 (1)PH组血清VEGF和NO含量明显高于对照组,差异有非常显著性(P＜0.01);(2)PH组血清VEGF和NO含量与平均肺动脉压力呈正相关(r=0.712,P＜0.01;r=0.602,P＜0.01);(3)PH组血清中VEGF与NO含量呈正相关(r=0.801,P＜0.01).结论 VEGF和NO在CHD伴PH的形成和发展中起保护作用;VEGF可能通过NO介导而发挥其血管重构作用.     

氨基末端脑钠素原在小儿先天性心脏病并肺动脉高压的诊断价值

目的探讨氨基末端脑钠素原(NT-proBNP)在小儿先天性心脏病(先天性心脏病)并肺动脉高压(肺高压)的诊断价值。方法2005年1月至2007年3月山东大学齐鲁儿童医院内科采用竞争性酶免疫法检测20例正常对照组小儿及65例先天性心脏病患儿血清NT-proBNP浓度。65例先天性心脏病患儿经用彩色多普勒超声心动图估测肺动脉压力,分为三组:无肺高压组20例、轻度肺高压组23例、中重度肺高压组22例。结果1、先天性心脏病无肺高压组较正常对照组NT-proBNP水平略有增高,但无明显统计学差异(P>0.05)。2、先天性心脏病并轻度肺高压组NT-proBNP水平明显高于先天性心脏病无肺高压组(P<0.001)。3、中重度肺高压组患儿NT-proBNP水平较轻度肺高压组明显增高(P<0.001)。4、以500 fmol/m l诊断轻度肺高压的敏感度为91%,特异度为90%,且经直线相关分析,显示NT-proBNP与肺高压高低呈正相关,r=0.80。结论血清NT-proBNP检测对先天性心脏病并肺高压具有敏感而特异的诊断价值,且可反映肺动脉压增高程度。     

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目的 探讨先天性心脏病(CHD)患儿肺动脉高压(PH)形成的影响因素.方法 研究对象均为2003-06-2005-02于北京大学深圳医院收集病例,以健康者30名为对照组(A组),以肺动脉压正常和并发PH的左向右分流的CHD患儿各30例为观察组(B组、C组).以高效液相色谱法、硝酸还原法及放射免疫法测定其血清精氨酸(L-Arg)、一氧化氮(NO)、血浆内皮素(ET-1)的浓度.结果 血清L-Arg浓度对照组(A组)为(72.00±18.01)nmol/mL,肺动脉压正常的患儿(B组)为(30.74±8.97)nmol/mL,伴PH的患儿(C组)为(23.51±12.37)nmol/mL.血清NO浓度A组为(76.10±17.10)nmol/mL,B组(90.55±26.57)nmol/mL,C组(60.05±17.60)nmol/mL.血浆ET-1浓度A组(50.82±7.58)pg/mL,B组(64.90±16.28)pg/mL,C组(69.64±10.66)pg/mL.结论 血清NO浓度和血浆ET-1浓度及其之间的平衡关系共同影响PH的形成及其程度.血浆ET-1浓度的升高是肺动脉压升高的直接因素,血清NO浓度的降低是间接因素,而血清NO浓度降低是由血清L-Arg浓度的降低引起.     

Comparison of patients with Kawasaki disease with retropharyngeal edema and patients with retropharyngeal abscess

Kawasaki disease with retropharyngeal edema (KD with RPE) is a rare complication, and it is diagnosed by neck CT. Most reported cases had a delayed diagnosis because those patients' conditions were misdiagnosed as retropharyngeal abscess (RPA). The purpose of this study was to differentiate KD with RPE from RPA. We performed a retrospective case–control study comparing children with KD with RPE to those with RPA hospitalized at the tertiary pediatric hospital in Tokyo between 2005 and 2011. The 39 patients revealing RPE on neck CT were divided into two groups: group A was classified as KD (n?=?21) and group B was classified as non-KD (n?=?18). Patients in group B were finally evaluated as having RPA clinically and were treated with antibiotic therapy. A significantly higher proportion of patients in group B complained of dysphagia (11 patients vs. 5 patients; p?=?0.0170) and neck pain (17 patients vs. 12 patients; p?=?0.0106). Neck CT revealed a ring enhancement (16 patients vs. no patients; p?<?0.0001) and mass effect in a greater proportion of patients in group B (11 patients vs. 1 patient; p?<?0.0003). Conclusion: Careful attention to manifestations and close analyses of CT imaging may allow clinicians to differentiate KD with RPE from RPA.     

Patients with moyamoya disease presenting with movement disorder

Moyamoya disease is a rare cerebrovascular disease characterized by idiopathic bilateral stenosis or occlusion of bilateral internal carotid arteries and the development of characteristic leptomeningeal collateral vessels at the base of the brain. Typical presentations include transient ischemic attacks or stroke, and hemorrhage. Presentation with movement disorders is extremely rare, especially in the pediatric population. The authors describe the cases of 4 children with moyamoya disease who presented with movement disorders. Among 446 patients (118 pediatric) with moyamoya disease surgically treated by the senior author, 4 pediatric patients had presented with movement disorders. The clinical records, imaging studies, surgical details, and postoperative clinical and imaging data were retrospectively reviewed. The initial presenting symptom was movement disorder in all 4 patients: chorea in 2, hemiballismus in 1, and involuntary limb shaking in 1. All the patients had watershed infarcts involving the frontal subcortical region on MR imaging. Additionally, 1 patient had a ganglionic infarct. Single-photon emission computed tomography studies showed frontoparietal cortical and subcortical hypoperfusion in all patients. Three patients had bilateral disease, whereas 1 had unilateral disease. All the patients underwent superficial temporal artery-middle cerebral artery bypass. Postoperatively, all 4 patients had complete improvement in their symptoms. The SPECT scans revealed normal perfusion in 3 patients and a small residual perfusion deficit in 1. Movement disorders are a rare presenting feature of moyamoya disease. Hypoperfusion of the frontal cortical and subcortical region was seen in all patients, and the symptomatology was attributed to ischemic dysfunction and imbalance in the cortical-subcortical-ganglionic-thalamic-cortical circuitry. Combined revascularization with superficial temporal artery-middle cerebral artery bypass and encephaloduroarteriosynangiosis leads to excellent results.     

Treatment with probucol of children with familial hypercholesterolaemia

Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2 +/- 1.45 mmol/l to 7.17 +/- 0.84 mmol/l (12.6%). This level was further reduced to 5.92 +/- 0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.