抗磷脂抗体与儿童狼疮性肾炎

目的探讨儿童狼疮性肾炎(LN)抗磷脂抗体的阳性率及其与肾脏损害的临床表现/病理分型的关系。方法回顾性分析1997年—2009年确诊LN同时送检抗磷脂抗体的住院患儿的临床和病理资料。结果共50例患儿符合纳入标准,男11例、女39例,平均就诊年龄(11.8±2.1)岁。全部患儿均检测狼疮抗凝物(LA)和抗心磷脂抗体(aCL),31例检测抗β2糖蛋白I(β2-GPI)抗体,其中抗磷脂抗体阳性44例(88.0%),LA、aCL和抗β2-GPI抗体的阳性率分别为80.0%、52.0%和67.7%。抗磷脂抗体阳性与阴性的LN患儿相比,肾病水平蛋白尿、肉眼血尿、高血压和肾功能异常的差异无统计学意义(P均>0.05)。44例患儿进行了肾脏活检,抗磷脂抗体阳性和阴性LN患儿的病理分型差异无统计学意义(P=0.322),Ⅴ型LN仅见于抗磷脂抗体阳性的病例;肾小球微血栓的发生率为20.5%(9/44例),其中18.2%(8/44例)检测抗磷脂抗体阳性。抗磷脂抗体阳性患儿血小板减少的发生率为47.7%,阴性者33.3%(P=0.674)。3例患儿有肾外血栓形成,2例血栓形成在甲基泼尼松龙冲击、中心静脉置管之后,2例在抗磷脂抗体阳性8...     

脾脏、淋巴结及网状内皮系统疾病

052331川崎病患儿血清抗心磷脂抗体水平检测及其临床意义/刘桂英…//临床儿科杂志.-2005,23(3).-167～16920例川崎病(KD)患儿均未得到早期诊治,明确诊断时间平均为9.8d,并发冠状动脉扩张12例(60%),冠状动脉瘤5例(25%)。KD患儿抗心磷脂抗体(ACA)阳性率为55.0%,并发有冠脉损伤者ACA水平明显高于无冠脉损伤者。参12(何燕)052332川崎病86例临床分析/吴鸿基//重庆医学.-2005,34(3).-421～422对86例患儿资料、临床表现、变化特征及诊断情况和冶疗转归进行总结和分析。结果:首诊KD入院38例     

混合性结缔组织病并肺动脉高压1例并文献复习

目的探讨混合性结缔组织病(MCTD)并肺动脉高压(PAH)的诊断与治疗,以提高对此病的认识。方法对MCTD并PAH 1例患儿的临床表现、既往史、辅助检查、诊疗过程进行分析,并复习相关文献。结果该患儿以间断晕厥为主要表现,超声显示重度PAH,抗核糖核酸蛋白抗体(U1-RNP)阳性,予免疫抑制及降低PAH治疗,病情明显好转。结论儿童MCTD并PAH起病隐匿,危害严重,诊断困难,应早期诊断,及时治疗,可改善预后。     

过敏性紫癜的中枢神经系统损害及其机制探讨

目的 观察过敏性紫癜患儿中枢神经系统损害的发病情况并探讨其可能机制.方法 观察2006年1月-2009年4月收治的335例过敏性紫癜患儿中枢神经系统损害的发病情况,对有中枢神经系统损害的患儿进一步检测其血清和脑脊液中狼疮抗凝物(LAC)、抗心磷脂抗体(ACA)和抗B2糖蛋白-Ⅰ抗体水平.结果 335例过敏性紫癜患儿共有36例(10.75%)出现中枢神经系统损害,主要表现为情绪小稳、烦躁、精神行为改变、头痛、头晕、易激惹等非特异性改变,腩脊液、脑电图及头颅影像学检查无特异性变化;有中枢神经系统损害的过敏性紫癜患儿,脑脊液中LAC、ACA、抗β2糖蛋白-Ⅰ抗体阳性率明显升高(P＜0.05).结论 约有10%左右过敏性紫癜患儿有中枢神经系统损害,其发病机制可能与脑脊液中抗磷脂抗体阳性有关.     

儿童低凝血酶原血症-狼疮抗凝物综合征2例报告并文献复习

目的对儿童低凝血酶原血症-狼疮抗凝物综合征(hypoprothrombinemia-lupus anticoagulant syndrome,HLAS)的临床特点进行总结,以提高对该病的认识。方法回顾性分析收治的2例HLAS患儿及医学引文索引(Medline)检索到的27例HLAS患儿的临床资料,总结其临床特点。结果 HLAS女性多发;可继发于病毒感染(14/29,48.2%)或系统性红斑狼疮(14/29,48.2%);临床主要表现为不同程度的出血,以中重度出血多见(25/29,86.2%),出血严重程度与原发病及凝血酶原活性无明显相关性;29例HLAS患儿中未经特殊治疗缓解者12例(41.4%),经过替代和(或)免疫性治疗缓解者15例(51.7%),病情仍有波动2例(6.9%)。结论 HLAS极其罕见,常继发于病毒感染或系统性红斑狼疮,临床表现为出血及低凝血酶原血症,大部分患儿预后良好。掌握其临床特点及诊断流程有助于早期诊断,避免误诊。     

不同发作类型癫(癎)患儿血清抗心磷脂抗体变化及临床意义

目的 探讨不同发作类型癫(癎)患儿血清抗心磷脂抗体水平的变化及其临床意义.方法 采用ELISA法检测84例癫(癎)患儿治疗前后及38例正常儿童血清抗心磷脂抗体水平.结果 健康对照组、部分发作组、全身发作组及分类不明发作组血清抗心磷脂抗体阳性率分别为6.3%(2/32)、28.6%(8/28)、33.3%(12/36)、30.0%(6/20),各组比较差异有显著性(X2=9.98,P<0.05);部分发作组、全身发作组及分类不明发作组血清抗心磷脂抗体阳性率均明显高于健康对照组(P<0.01),癫(癎)不同发作类型组间比较差异无显著性(P>0.05).部分发作组、全身发作组及分类不明发作组癫(癎)患儿在治疗后血清抗心磷脂抗体阳性率明显降低,差异有显著性(X2=5.39,P<0.05;X2=14.40,P<0.01;X2=11.67,P<0.01).结论 癫(癎)患儿血清抗心磷脂抗体水平增加,提示癫(癎)发作中可能存在自身免疫异常;治疗后血清抗心磷脂抗体水平降低,为癫(癎)的有效防治提供重要依据.     

儿童肺动脉高压276例病因学分析

目的 探讨儿童肺动脉高压(PAH)的病因及相关因素,以提高儿童PAH的早期诊断和治疗.方法 回顾性分析1995年5月-2007年5月北京大学第一医院儿科住院患儿中包含PAH诊断并经超声心动图检查证实为肺动脉压力升高的病例,记录其年龄、性别、临床主要诊断、PAH相关症状及超声心动图估测的肺动脉压力,按病因进行分组统计,对不同病因组间经多普勒超声心动图三尖瓣返流速度估测的肺动脉收缩压(sPAP)进行比较.不属于威尼斯会议PAH临床诊断分类中第一类的PAH未统计在内.结果 共诊断PAH 276例.男168例,女108例;年龄1个月～17岁,中位年龄9个月.其中特发性PAH 9例(3.3%),余267例(96.7%)均为相关性PAH,其中以先天性心脏病(先心病)相关性PAH为主,共245例(88.7%),其中又以室间隔缺损等左向右分流先心病为主,共217例(88.6%),复杂先心病相关PAH 28例(11.4%).结缔组织病相关PAH 19例(6.9 %),PAH在SLE、幼年型类风湿关节炎及多发性大动脉炎中的发病率分别为10.3 %(13/126)、8.7%(4/46)、15.4%(2/13),余2例与门脉高压相关,1例与珠蛋白生成障碍性贫血相关.经三尖瓣返流速度估测的8例特发性PAH患儿 sPAP为(74.6±23.9) mmHg(1 mmHg=0.133 kPa),显著高于33例先心病相关PAH[(58.0±19.7) mmHg]及12例结缔组织病相关PAH[(49.6±18.9) mmHg](t=-2.052,-2.609 Pa<0.05).结论 儿童PAH主要是由多种病因引起的相关性PAH,常见病因为先心病和结缔组织病,特发性PAH患儿确诊时病情已较严重.     

结缔组织疾病患儿肺功能分析

目的 探讨结缔组织疾病(CTD)患儿肺功能受损情况,为CTD患儿肺部并发症的早期发现及预后评估提供参考.方法2006年10月-2007年7月住院或门诊随访的CTD患儿35例(CTD组),男16例,女19例,无咳嗽、气促等症状,胸片检查正常.应用Master-Screen Paed 肺功能仪测定其常规肺功能和脉冲振荡肺功能,并与健康对照组32例(男13例,女19例)比较.应用STATA 7.0软件对CTD组和健康对照组的肺功能数据进行统计学分析.结果CTD患儿肺功能异常率为57.14%,17例(48.57%)表现为轻度至中度的限制性通气障碍,9例(占25.71%)伴小呼吸道功能下降,2例(占5.71%)表现为限制性和阻塞性混合通气障碍.反映肺通气功能的参数(最大肺活量、用力肺活量、一秒钟用力呼气容积)CTD组均明显低于健康对照组[(77.66±20.22)%vs(94.39±11.27)%;(79.05±21.45)% vs(94.89±11.46)%;(84.91±23.29)%vs(103.62±12.96)%Pa<0.01].反映中、小呼吸道功能的参数(呼出50%肺活量时最大呼气流速、呼出75%肺活量时最大呼气流速)CTD组均明显低于健康对照组[(85.82±31.02)% vs(102.82±27.32)%;(74.52±32.59)% vs(98.75±40.59)%Pa<0.05].反映呼吸道阻力参数(总呼吸道阻力、中心呼吸道阻力、周边弹性阻力、共振频率)在CTD组和健康对照组间比较均无显著差异(Pa>0.05).结论CTD患儿肺功能改变主要为限制性通气障碍伴不同程度的小呼吸道功能下降,肺功能测定可早期反映CTD患儿病情发展的进程和程度,对CTD患儿肺部损伤的评估和及时治疗有较大帮助,应作为CTD患儿的常规检查项目.     

儿童颅内静脉窦血栓形成抗凝治疗的队列研究

目的 评估抗凝治疗是否可以改善患儿颅内静脉窦血栓形成(cerebral venous sinus thrombosis,CVST)的临床症状和长期生存质量.方法 资料选取2006年6月至2013年6月收治的符合标准的CVST患儿58例(1个月～16岁).其中,接受抗凝治疗(低分子肝素、华法林、尿激酶)38例,为抗凝治疗组(抗凝组);行非抗凝对症治疗20例,为非抗凝治疗组(非抗凝组).回顾性分析评价两组血栓复发率、抗凝相关出血率及长期预后情况.长期生存质量应用改良Rankin评分进行评估.结果 治疗后出现抗凝治疗相关出血2例(5.3％).复发5例(8.6％)中,抗凝组1例,非抗凝组4例,组间比较,差异有统计学意义(P=0.023).死亡5例(8.6％)中,抗凝组1例,非抗凝组4例,抗凝治疗同单纯对症治疗相比降低病死率25％～30％.长期随访45例(不包括死亡5例及失访8例)临床长期预后不良6例(13.3％),整体预后不良(包括死亡5例和长期预后不良6例)11例(22％).影响长期预后因素中足月儿预后较好(风险比0.12,P=0.045)、颅内病灶广泛预后较差(风险比15.16,P=0.042)和抗凝治疗为主要减少预后不良发生率因素(风险比0.007,P=0.024);起病初期颅高压发生的相关因素中,新生儿窒息史患儿颅高压发生率低(风险比0.35,P=0.025)、颅内病灶广泛发生率高(风险比8.73,P=0.048)和发病时间较短治疗及时的患儿发生颅高压率较低(风险比0.89,P =0.003).同时,抗凝治疗对于亚急性期颅高压控制有一定帮助(P=0.048).结论 对于儿童CVST(包括轻度颅内出血的CVST患儿)抗凝治疗是安全有效的治疗方法,可有效降低其复发率,延长生存时间和改善长期生存质量.     

结缔组织病合并全身炎症反应综合征患儿血清前降钙素测定及意义

目的 探讨结缔组织病(CTD)合并全身炎症反应综合征(SIRS)患儿血清前降钙素(PCT)检测的临床意义.方法 选取2005年9月至2007年6月我科收治的35例SIRS未合并感染的CTD患儿(CTD组)和30例伴SIRS的细菌感染患儿(细菌感染组)为研究对象,均在患儿入院后24 h内采集静脉血.采用双抗夹心免疫发光测量法测定血清PCT水平.结果 CTD组、细菌感染组PCT的平均水平分别为(0.3±0.2)μg/L、(1.6±0.7)μg/L,两组间差异有显著性(t=10.51,P＜0.001).35例CTD患儿中,3例PCT＞0.5μg/L(均为川崎病患儿),阳性率8.6%;30例细菌感染患儿中有22例PCT≥0.5μg/L,阳性率为73.3%,明显高于CTD组(X2=28.62,P＜0.001).结论 在伴发SIRS的CTD患儿中PCT水平一般不升高,但合并细菌感染时PCT水平可明显增高.因此,血清PCT值检测是鉴别感染性和非感染性SIRS的重要依据之一.     

Connective Tissue Disease Presenting With Signs and Symptoms of Pulmonary Hypertension in Children

Our case series describes three children who were initially diagnosed as having severe pulmonary arterial hypertension (PAH) and subsequently found to be positive for specific autoantibodies suggestive of an underlying autoimmune process. The signs and symptoms of PAH are subtle and may be part of the initial presentation of childhood connective tissue disease (CTD). Evaluation for connective tissue disease in the newly diagnosed pulmonary hypertension (PH) patient is important because early diagnosis of PH as well as CTD is crucial in the successful management of these complex patients. Ongoing monitoring for CTD in patients with severe PAH is warranted.     

6月龄以下小婴儿危重型肺动脉瓣狭窄经皮球囊肺动脉瓣扩张成形术治疗的随访报告

目的 探讨经皮球囊肺动脉瓣扩张成形术(percutaneous balloon pulmonary valvuloplasty,PBPV)治疗新生儿和6月龄以下小婴儿危重型肺动脉瓣狭窄(critical pulmonary stenosis,CPS)的疗效以及早中期随访.方法 采用PBPV治疗34例日龄波动于13～175 d的新生儿以及小婴儿CPS,并进行了6个月～4年的临床随访.记录患儿PBPV前后右室收缩压(right ventricular systolic pressure,RVSP),肺动脉瓣跨瓣压差(transvalvular peak to peak systolic gradient,△P)以及PBPV术后采用心脏彩色多普勒动态监测的肺动脉瓣跨瓣压差的动态变化.结果 34例中32例(94%)完成PBPV术,1例术后2 h内死亡,2例心包积血,3例术中小球囊预扩张后换用合适球囊完成PBPV术,3例右室流出道痉挛,1例术后30 min右下肢股动脉搏动减弱.28例PBPV成功,RVSP由(96±28)mm Hg(1 mm Hg=0.133 kPa)下降至(49±20)mm Hg(下降49%,P＜0.01),△P由(89±25)mm Hg下降到(25±12)mm Hg(下降72%,P＜0.01),右室/主动脉收缩比值1.2±0.5下降到0.7±0.3(P＜0.01).随访6个月～4年,3例失访,5例(其中3例新生儿)因残余压超过40 mm Hg行第二次PBPV术,23例彩色多普勒心脏超声监测肺动脉瓣跨瓣压有进一步下降到(20±13)mm Hg,无明显的肺动脉瓣反流,无需进一步处理.结论 对于6个月以下小婴儿CPS,PBPV仍有效,安全的治疗方法并具有相对好的短中期疗效.

Abstract：

Objective To evaluate the effect and results of short and medium periods of follow-up of percutaneous balloon pulmonary valvuloplasty for critical pulmonary stenosis of neonates and infants under 6 months of age. Methods Between January 2002 and December 2008, 34 consecutive patients aged from 13to 175 days with critical pulmonary valvular stenosis underwent percutaneous balloon valvuloplasty. Patients records, catheterization data, angiograms and echocardiograms were reviewed. Patients were followed up for 6 months to 4 years ( mean 25.5 months ) by means of clinical examination and Doppler echocardiography.Results The pulmonary valvuloplasty was accomplished in 32 (94%) of 34 attempts. Immediately after dilation, right ventricular systolic pressure (RVSP) decreased from (96 ±28) mm Hg ( 1 mm Hg =0. 133kPa ) (49 ± 20 ) mm Hg ( P ＜ 0. 01 ), the transvalvular peak to peak systolic gradient (△P) decreased from (89±25) mm Hg to (25 ± 12) mm Hg (P ＜0.01 ), and the right ventricular/aortic systolic pressure ratio decreased from 1.2 ± 0. 5 to 0. 7 ± 0. 3 ( P ＜ 0. 01 ). One patient died because of cardiac tamponade following rupture of the pulmonary valve annulus, 2 patients developed pericardial effusion, 3 patients had infundibular spasm, 3 patients had a pre-dilation by small balloon and 1 patient had weakened femoral artery pollex. After a follow up period of 6 months to 4 years 3 of 31 patients lost to follow-up. Repeat valvuloplasty was performed in 5 patients (3 neonates), no patient required surgery, and the other 23 patients did not undergo further intervention, a mean peak systolic Doppler gradient of (20 ± 13) mm Hg was found and no significant pulmonary regurgitation was seen. Conclusions Percutaneous balloon pulmonary valvuloplasty was effective and safe for the treatment of critical pulmonary stenosis of neonates and infants under 6 months of age with good short and medium term results.     

儿童先天性心脏病血清Apelin水平与肺动脉压关系的初步探讨

目的 初步探讨先天性心脏病患儿血清Apelin 水平与肺动脉压的关系.方法 手术治疗的先心病患儿126 例,检测患儿术前及术后第7 天的血清Apelin 水平.建立体外循环前检测并计算肺动脉收缩压/体循环收缩压(Pp/Ps)的比值,依据Pp/Ps 分组:无肺动脉高组压(PAH)组、轻度PAH 组、中度PAH 组和重度PAH 组;术后第7 天超声心动图估测肺动脉平均压(PAMP).结果 无PAH,以及轻、中、重度PAH 各组术前及术后的血清Apelin 水平依次降低,差异有统计学意义(PPr=-0.51,-0.54,P结论 先心病患儿并发肺动脉高压及其发展与血清Apelin 水平降低有关系,血清Apelin 对诊断先心病患儿是否并发肺动脉高压及其程度的意义值得深入研究.     

多普勒超声心动图对先天性心脏病合并肺动脉高压儿童左室形态与功能的评价

目的：探讨多普勒超声心动图对先天性心脏病（CHD）儿童肺动脉高压（PAH）的诊断价值及PAH对左室重塑和舒张功能（LVDF）的影响。方法：对彩色多普勒超声心动图证实的45例继发肺动脉高压的先天性心脏病患儿的超声心动图和多普勒血流资料与22例无PAH的先天性心脏病患儿（对照组）对比分析。结果：PAH组及对照组的左室舒张末内径分别为18.24±1.71 mm vs 16.28±0.52 mm、右室内径 12.23±2.14 mm vs 8.14±0.73 mm、肺动脉内径 11.20±1.35 mm vs 7.92±0.21 mm。两组比较差异有显著性（P<0.05）；VTR 流速增快（2.56±0.46）m/s及PASP压力（40.23±4.56）mmHg升高 ，与对照组相比差异亦有显著性（P<0.05）；室间隔增厚与对照组相比差异不明显。对照组、PAH组的二尖瓣口血流多普勒频谱A峰流速分别为94.56±31.45 m/s vs 51.17±26.67 m/s、A峰流速速度时间积分为10.89±2.73 s vs 4.94±1.85 s及AV/EV为1.79±0.32 vs 0.59±0.19、AVTI/EVTI为1.61±0.49 vs 0.45±0.21 两组比较差异有显著性(P<0.01)。左室等容舒张时间分别为119. 86±54.62 s vs 52.31±28.06 s。PAH组明显延长(P<0.05)。E峰流速、E峰流速速度时间积分及E峰减速时间两组无明显改变(P>0.05)。而增高的肺动脉压与二尖瓣口血流频谱AV/EV比值呈正相关,相关系数 r=0.4 456， P<0.01。结论：多普勒超声心动图不仅是先天性心脏病儿童合并肺动脉高压最重要的诊断方法之一，还可提供肺动脉高压对左室重塑和舒张功能受损的指标，对临床判断病情轻重及预后有重要价值。［中国当代儿科杂志，2007，9（5）：422-424］     

Estimation of pulmonary artery pressure by contrast-enhanced Doppler signals and comparison with catheter-measured pressures.

Determination of pulmonary artery systolic pressure (PASP) is essential for the diagnosis, and the timing and type of management of patients with congenital heart disease (CHD). Usually cardiac catheterization, an expensive and invasive technique, is required for accurate measurement. A number of noninvasive methods for the assessment of PASP have been developed, one of which is estimation of PASP using contrast-enhanced tricuspid regurgitation Doppler signals (TRDS). In this study, right ventricular systolic pressures (RVSP) and PASP of 36 patients (19 girls, 17 boys; aged 5 months to 15 years) with CHD were estimated by TRDS before and after galactose solution (GS) and were compared with catheterization measurements. Significant TRDS (> 1 m sec.) were obtained in nine of 36 (25%), patients before GS and in 23 of 36 patients (64%) after GS. TRDS were increased significantly by contrast agent. Estimated RVSP and PASP were significantly different from the measured pressures before and after GS. There were significant correlations between the estimated RVSP and PASP and measured RVSP after GS. Estimated pressures were underestimated. We conclude that it is better to use the estimated PASP on patients with significant TRDS for the classification of PASP.     

Role of inhaled nitric oxide as a selective pulmonary vasodilator in pediatric cardiac surgical practice

Our aim was to assess the role of inhaled nitric oxide (NO) therapy in post operative cases of congenital heart defects who developed pulmonary arterial hypertensive (PAH) crisis and had no response with conventional management. From February '95 to January '97, inhaled NO therapy was used in 21 children. Age ranged from 2 months to 9 years (mean 5.6 years) and duration of therapy ranged from 1 to 13 days. Of 21 patients, 17 responded well with 5–20 ppm while 4 did not. The preoperative mean pulmonary systolic pressure was 88 mm Hg against mean systemic pressure of 96 mm Hg. Post operatively, their PA pressure reduced to 62 mm Hg, with systemic pressure of 98 mm Hg. After using inhaled NO, PA pressure dropped to 24 mm Hg (mean systolic) (p<0.007), after excluding the non responders. Of 4 non responders, two died due to irreversible pulmonary vascular disease and remaining two died due to residual defects. The study shows that inhaled NO is a selective pulmonary vasodilator, which is useful in postoperative PAH crisis and also reduces the transpulmonary gradient in single ventricle repair cases. It is safe and effective for prolonged use. It is very useful in Indian perspective, when more number of cases with congenital heart defects (CHD) alongwith severe PAH are encountered routinely.     

儿童特发性肺动脉高压的临床特点及预后的影响因素

目的：调查儿童特发性肺动脉高压( idiopathic pulmonary arterial hypertension,IPAH)的临床特点及预后的影响因素,为临床诊治及预后评估提供参考。方法回顾性分析2006年1月至2015年12月于我科诊断为IPAH患儿的临床资料,根据预后分为存活组和死亡组,对相关指标应用Logistic回归分析筛选出影响IPAH患儿预后的危险因素。结果共计26例患儿纳入研究,存活组17例,死亡组9例。儿童IPAH以学龄期儿童多见,平均年龄(6.6±3.6)岁,男女比例1.17∶1。所有患儿均有活动耐力下降和乏力症状,晕厥及呼吸困难、水肿的发生率分别为34.6％及23.1％,肺动脉瓣听诊区第2心音亢进为最常见体征(92.3％),50.0％患儿在诊断时心功能为Ⅲ~Ⅳ级。超声心动图检查示肺动脉收缩压(PASP)平均(79.0±20.3) mmHg(1 mmHg＝0.133 kPa),16例患儿右心室重度扩大。心电图以ST-T改变及右心室高电压最为常见,血浆脑钠肽(BNP)平均浓度为(870.4±720.9) pg/ml。单因素分析显示晕厥(OR＝26.25,95％CI 3.04~226.60,P＝0.003),心功能Ⅲ~Ⅳ级(OR＝19.199,95％CI 1.88~196.53,P＝0.0128),PASP≥70 mmHg(OR＝9.936,95％CI 1.81~∞,P＝0.005),BNP≥850 pg/ml(OR＝59.991,95％CI 4.69~767.62,P＝0.002)是预后不良的危险因素。结论儿童IPAH临床少见,预后不良。晕厥、心功能分级、PASP程度及BNP浓度是儿童IPAH预后的重要影响因素。 BNP作为IPAH的标志物在病情程度评估以及预后预测中具有一定的参考价值。     

先天性心脏病肺动脉高压患儿左室舒张功能与血浆脑利钠水平的相关性研究

目的：探讨先天性心脏病 (CHD)合并肺动脉高压 (PAH) 患儿脑利钠肽（BNP）水平及与左室舒张功能的关系。方法：对95例CHD继发有PAH的患儿和42例无PHA的CHD患儿（对照组）的多普勒超声心动图资料与其血浆BNP水平进行对比分析。结果：与对照组相比, PAH组的左室舒张末内径（LVDd ）、右室舒张末内径（RVDd） 和肺动脉内径（PAd）明显增大(P<0.05),三尖瓣返流（VTR）速度增快及肺动脉收缩压（PASP）升高(P<0.05)。与对照组比较,PASP组患儿二尖瓣口多普勒血流频谱A峰流速（AV）、A峰流速积分（AVI）和E峰流速积分（EVI）及AV/EV和AVI/EVI比均逐渐明显增大(P<0.01);左室等容舒张时间明显延长(LIVRT)(P<0.05)。血浆BNP水平随着PASP增高而升高,与对照组相比差异有显著性（P<0.01）。PAH组先心病患儿其肺动脉压与二尖瓣口血流频谱AV/EV比值呈正相关（P<0.01）,二尖瓣口血流参数与血浆BNP水平亦呈正相关（P<0.01）。结论：CHD合并PAH患儿左室舒张功能与血浆BNP水平呈正相关;BNP在PAH引起左室舒张功能障碍的发生发展过程中发挥了重要作用。［中国当代儿科杂志,2010,12（1）：13-16］     

缺氧性肺动脉高压新生儿右心室功能的组织速度多普勒超声研究

目的 探讨组织速度多普勒成像(TVI)技术评价缺氧性肺动脉高压(HPH)新生儿右心室功能的价值.方法 35例HPH患儿及28例正常新生儿于生后3 d应用超声心动图测量肺动脉收缩压(PASP),用传统超声技术测定右室射血分数(RVEF)值、右心室舒张早期峰值(E峰)、舒张晚期峰值(A峰)、E/A值,同时以TVI技术测量三尖瓣环指标(收缩期波Sa、舒张早期波Ea、舒张晚期波Aa、Ea/Aa值).结果 HPH组三尖瓣环Sa、Ea、Ea/Aa及RVEF值较对照组减低(t=2.67～4.69,P均<0.01).HPH组的右心室E/A值较对照组减低,但差异无统计学意义.HPH组的三尖瓣环Sa与RVEF值呈正相关(r=0.451,P<0.05).结论 HPH新生儿的右心室舒张、收缩功能减低,TVI技术与传统超声技术结合能更敏感地发现右心室功能变化.     

�ζ�����ѹ����Ѫ��N����������ǰ��� ������Teiָ��������о�

??Abstract??Objective To investigate the correlation among plasma N-terminal pro-brain natriuretic peptide ??NT-proBNP?? and right ventricular Tei ??RVTei?? and right ventricular fractional area change ??RVFAC?? in children with pulmonary hypertension. Methods From January 2007 to November 2012??echocardiography and serological examination data of 38 patients from Samsung Seoul Hospital in Korea were retrospectively analyzed. Patients were divided into two groups ??16 cases with 30??PASP??70mmHg ??mild to moderate?? and 22 cases with PASP≥70mmHg ??severe??. Results ??1??RVFAC?? PASP and plasma concentration of NT-proBNP had a statistically significant difference??P??0.05?? between mild to moderate PAH group and severe PAH group . RVTei index had no difference between the two groups??P??0.05??. ??2??Correlation analysis??the plasma concentration of NT-proBNP had a positive correlation ??r = 0.544??P??0.05??with PASP??a negative correlation??r = -0.767??P??0.05??with RVFAC??RVFAC had a negative correlation??r = -0.711??-0.767??P??0.05?? with PASP and the plasma concentrations of NT-proBNP?? the RVTei had no correlation with the plasma concentration of NT-proBNP?? PASP or RVFAC??r = -0.041??-0.048??0.016??P??0.05??. Conclusion Whether there are correlations between the plasma concentration of NT-proBNP and RVFAC depends on the degree of PAH. This suggests that the plasma concentration of NT-proBNP and RVFAC can be used as evaluation of right ventricular function in children with pulmonary hypertension.