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目的探讨经皮球囊肺动脉瓣成形(PBPV)术治疗婴儿和新生儿肺动脉瓣狭窄(PS)及室间隔完整型肺动脉瓣闭锁(PA/IVS)的安全性及有效性。方法2006年1月至2009年4月,广东省心血管病研究所心儿科收治PS及PA/IVS婴儿和新生儿63例。其中危重新生儿20例(31.7%)。术前经超声心动图确诊为PS56例,PA/IVS7例。危重新生儿PS需先用直径2.5～4.0mm小球囊预扩张肺动脉瓣,PA/IVS患儿需先行射频瓣膜打孔术。PBPV术所选球囊直径为肺动脉瓣环径(PVAD)1.2～1.3倍(新生儿所选球囊直径为PVAD1.0～1.2倍)。结果全组63例(100%)PBPV术成功。球囊径/瓣环径:1.2±0.1。右室压力明显下降[术前(104.3±32.7)mmHg(1mmHg=0.133kPa),术后(52.0±10.5)mmHg,P<0.001],跨肺动脉瓣压力阶差明显下降[术前(99.2±23.5)mmHg,术后(27.7±12.4)mmHg,P<0.001]。平均手术时间(88.1±36.2)min,平均X线曝光时间(16.9±11.1)min。术中6例(9.5%)出现并发症,2例心包积液,2例低氧血...     

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目的了解经皮球囊肺动脉瓣成形术(PBPV)前后不同时间点血浆心肌钙蛋白-I以及肌酸磷酸激酶同工酶MB(CK-MB)水平的动态变化,以此来评估该治疗方法对患儿心肌细胞的影响。方法2004-01—2005-06在南京市儿童医院接受PBPV的肺动脉瓣狭窄患儿18例,通过酶联免疫吸附法以及免疫抑制法分别测定18例先天性肺动脉瓣狭窄患儿在行球囊扩张治疗不同时间点血浆心肌钙蛋白-I和CK-MB水平。结果术后心肌钙蛋白-I水平明显升高,术后即刻所测心肌钙蛋白-I达到所有时间点的峰值[为(1.34±0.32)μg/L],而1d后绝大多数患儿心肌钙蛋白-I仍维持在术后即刻水平,术后3d已显著下降[为(0.43±0.20)μg/L],与术前[(0.31±0.11)μg/L]相比差异已无显著性。CK-MB于术后即刻也明显升高[为(44.7±8.9)U/L],与术前[为(24.2±7.10)U/L]相比差异有显著性,而术后1d则明显下降,于术后3d降至术前水平[为(27.2±5.3)U/L]。结论经皮球囊肺动脉瓣成形术存在对心肌细胞的损伤作用,但这种损伤是暂时的、可逆的。     

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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.     

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目的评估缺氧新生儿肺动脉高压(PAH)的发生及其对左室形态及功能的影响,探讨缺氧性PAH新生儿血浆脑利钠肽(BNP)水平与左室舒张功能的关系。方法选择2003年11月至2008年11月解放军总医院和北京军区总医院附属八一儿童医院新生儿重症监护室(NICU)收治的缺氧新生儿52例,根据彩色多普勒超声检查是否存在PAH分为PAH组(34例)与对照组(18例),比较2组患儿彩色多普勒超声心动图资料及其与左室舒张功能、血浆BNP水平的关系。结果(1)与对照组相比,PAH组左室舒张末内径(LVDd)、右室内径(RV)、肺动脉内径(PA)明显增大(P<0.05);三尖瓣反流峰值(VTR)流速增快及肺动脉收缩压(PASP)升高(P<0.05),室间隔增厚不明显(P>0.05)。(2)PAH组二尖瓣口血流多普勒频谱A峰流速(AV)、舒张早期充盈时间速度积分(AVTI)及A峰流速与E峰流速比值(AV/EV)、左房充盈分数(AVTI/EVTI)均较对照组显著增加(P<0.01);PAH组的左室等容舒张时间(LIVRT)延长,但与对照组相比差异无统计学意义(P>0.05),EV、舒张末期充盈时间速度积分(EVTI)及E峰减速时间(...     

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??Abstract?? Objective To investigate the early clinical features of children's liver degeneration ??hepatolenticular degeneration??HLD?? and prenatal genetic types. Methods The misdiagnoses and prenatal genetic testing results of 5 cases of HLD in the First Hospital Affiliated to China Medical University from January 2002 to December 2013 were retrospectively analyzed??whose main clinical feature was liver enzymes elevated. Results Fivecaseshadelevatedliverenzymes. Among them??two cases were found in the nursery physical examination??and the others' initial symptom was upper respiratory tract infection ??1 case?? and abdominal discomfort ??2 cases??. The misdiagnosis time was two months to eighteen months. Moreover??a HLDchild’smother had aprenatalgenetictest??and the result showed that the second child was arecessivegenecarrier. However??the liverenzymes and ceruloplasmin??CP?? were normal when he was born at term. Conclusion Unexplained liver enzyme abnormalities should be suspected in children with HLD. We should detect serum CP??urinarycopperandcornealK-Fring timely for earlydiagnosis andtimelytreatmenttoimprovetheprognosis. The prenatal genetic test is an important factor to ensure the health of next generation.     

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目的通过分析不同的球瓣比和球囊长度对儿童经皮球囊肺动脉瓣成形术(PBPV)近期及中远期疗效的影响,探讨最适宜的球瓣比和球囊长度。方法1987～2005年山东省立医院儿科诊治119例肺动脉瓣狭窄患儿,使用不同球瓣比和长度的扩张球囊行PBPV术,扩张前后测量右室与肺动脉间的峰值压力阶差,并行左侧位右室造影,测量瓣环大小并观察有无右室流出道激惹。术前、术后定期行经胸超声心动图检查,估测最大跨肺动脉瓣压力阶差,并观察肺动脉瓣形态及其反流情况。结果超大球囊法行PBPV术后,患儿的近期及中远期跨肺动脉瓣压差持续下降,且压差下降率不随球瓣比的增加而增大;术后未发现有肺动脉瓣再狭窄者,所有患儿均有不同程度的肺动脉瓣反流,且反流的程度随时间的延长而加重,并与球瓣比成正相关。对于年龄较小(≤6岁)的儿童,球瓣比大且长度≥40mm的球囊较易引起右室流出道痉挛及三尖瓣反流。中远期三尖瓣反流的发生可能间接继发于肺动脉瓣反流所引起的右室容量负荷过重。结论PBPV治疗肺动脉瓣狭窄,最佳的球瓣比为1.0～1.2,疗效满意且并发症少;6岁以下儿童宜使用长度<40mm的球囊,可减少右室流出道痉挛及近期三尖瓣反流的发生。     

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??Abstract??Objective To study the methylphenidate hydrochloride controlled-release tablets??OROS-MPH??dose titration treatment for children with attention deficit hyperactivity disorder??ADHD??combining conduct disorder??CD??to achieve the best clinical efficacy. Methods According to the DSM-IV diagnostic criteria of attention deficit hyperactivity disorder??ADHD??combining conduct disorder??CD????120 cases of children were randomly divided into the original dose group and dose titration group.Two groups of children accepted the 24-week treatment of OROS-MPH??in which the original dose group were chosen to 18 mg/day?? dose titration group increased to 36 mg/day or 54 mg/day??respectively??in the fifth week and thirteenth week??and after the course of treatment??the following assessments were performed??SNAP-IV scale??Child Behavior Checklist??CBCL??and Treatment Emergent Symptom Scale??TESS??.Results ??1??The best response rate in the dose titration group was significantly higher than original dose group ??P??0.05??.??2??The SNAP-IV hyperactivity / impulsivity?? inattention and confrontation behavior items showed a significant difference between dose titration group and original dose group??P??0.05??.??3??In the CBCL behavior scales??dose titration group and the original dose group showed significant difference in the presence of adverse exchanges??forcedness??hyperactivity??aggression and discipline of the five dimensions??P??0.05??.??4??The incidence of side effects in both groups of children had no significant difference??P??0.05??.Conclusion With different doses of OROS-MPH for the treatment of children with ADHD and CD??effects differ more obviously-the higher dose??36 mg/day-54 mg/day??for the improvement of symptoms is better than lower dose??18 mg/day??.     

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??Objective??To explore the preventive effect of regular doses of captopril on the pulmonary arterial hypertension associated with ventricular septal defect??VSD?? and its mechanism. Methods??Forty cases of children with VSD in accordance with the inclusive criteria??who were hospitalized in Chengdu Women and Children’s Central Hospital from July 2013 to July 2015 ??were collected and randomly divided into blank control group??n??20??and captopril intervention group??n??20??. Plasma MMP-9 and TIMP-1 were examined by using ELISA??and PASP??the Qp/Qs and right ventricular Tei index were measured by cardiac color Doppler ultrasound at 0 week??1 week??4 weeks??8 weeks??and 12 weeks respectively??side effects in captopril intervention group were also observed. Results??PASP??the Qp/Qs??right ventricular Tei index??plasma MMP-9 and TIMP-1 level in blank control group were increased over time??the same indexes in captopril intervention group were decreased??the changing trend of two groups had statistically significant difference??all P??0.05??. The indexes were of no difference between the two groups at 0 week??all P??0.05????PASP and right ventricular Tei index in captopril intervention group were lower than blank control group at 1 week??4 weeks??8 weeks??and 12 weeks??all P??0.05????the Qp/Qs plasma??MMP - 9 and TIMP - 1 level in captopril intervention group were lower at 4 weeks??8 weeks??and 12 weeks??all P??0.05??. No side effects were found in captopril intervention group during follow-up. Conclusion??Oral regular doses of captopril in the VSD children undergoing elective surgery may reduce pulmonary vascular remodeling and prevent pulmonary hypertension in order to achieve the best age at surgery??one of the mechanisms might be improving indexes of PASP??right ventricular Tei index and the Qp/Qs by reducing the plasma MMP 9 and TIMP - 1 level.     

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目的 探讨雾化吸入伊洛前列素进行急性血管扩张试验的价值.方法 研究对象为2007年2月至2008年5月在广东省心血管病研究所住院的50例先天性心脏病合并肺动脉高压患儿,对所有患儿进行左右心导管检查,之后肺动脉内注射酚妥拉明或雾化吸入伊洛前列素进行急性血管扩张试验,试验后重复左右心导管检查.根据Fick公式计算血流动力学参数.综合判断肺动脉高压性质,将惠几分成两组:动力组和梗阻组.动力组患儿进行手术治疗,术后定期随访并修正术前诊断.结果 酚妥拉明会显著增高受试者的心率,而伊洛前列素对心率的影响较轻微;酚妥拉明和伊洛前列素都能够降低平均肺动脉压力和肺血管阻力,升高肺循环血流量;酚妥拉明同时会降低平均主动脉压力和体循环阻力,升高体循环血流量,而伊洛前列素对体循环没有明显的影响.在使用伊洛前列素的急性血管扩张试验中,平均肺动脉压力、肺血管阻力/体循环阻力和肺循环血流量/体循环血流量等参数的变化在动力组和梗阻组中差异无统计学意义(P值分别为0.016、0.024和0.030).而使用酚妥拉明的急性血管扩张试验中,平均肺动脉压力和肺血管阻力两项参数的变化在动力组和梗阻组中差异有统计学意义(P值分别为0.017和0.004).结论 在先天性心脏病合并肺动脉高压的患儿中,使用酚妥拉明或伊洛前列素进行急性血管扩张试验都能够有效区分动力性与梗阻性肺动脉高压.酚妥拉明用药前后,肺循环和体循环的压力、阻力和血流量都有明显变化.而伊洛前列素雾化吸入以影响肺循环为主,可以保持相对平稳的血流动力学,在安全性上优于酚妥拉明.     

内源性硫化氢对大鼠高肺血流性肺动脉高压的调节作用

目的应用内源性硫化氢(hydrogensulfide,H2S)生成酶胱硫醚-γ-裂解酶(cystathionineγ-lyase,CSE)的抑制剂炔丙基甘氨酸(propargylglycine,PPG),探讨H2S在大鼠高肺血流性肺动脉高压形成中的作用。方法雄性SD大鼠25只,体重120～140g,随机分为分流组(n=9)、分流 PPG组(n=8)和假手术组(n=8)。分流组及分流 PPG组大鼠经腹主动脉—下腔静脉穿刺术建立高肺血流动物模型,分流 PPG组大鼠腹腔内注射PPG37.5mg/(kg·d),分流组与假手术组大鼠每日给予等量生理盐水。分流术后4周,采用右心导管法测量各组大鼠肺动脉平均压(meanpulmonarypressure,MPAP),用敏感硫电极法测量大鼠血浆和肺组织H2S含量,分别计算大鼠心脏组织右心室/左心室 室间隔(rightventricle/leftventricle septum,RV/LV SP)和心脏重量/体重(heartweight/baseweight,HW/BW)的值。结果分流术后4周,分流组大鼠MPAP与假手术组比较差别无显著性;分流 PPG组大鼠MPAP较分流组及假手术组分别升高了17.70%和21.79%(P<0.05);分流组血浆H2S含量比假手术组明显升高(50.12±6.13vs45.16±5.56,P<0.05);分流 PPG组大鼠血浆H2S含量明显低于分流组和假手术组(38.56±4.98vs50.12±6.13、38.56±4.98vs45.16±5.56,P均<0.05);分流 PPG组大鼠RV/LV SP和HW/BW明显高于分流组和假手术组(0.33±0.03vs0.30±0.03、0.33±0.03vs0.29±0.02,P<0.05)。结论内源性H2S在高肺血流性肺动脉高压形成过程中发挥保护性调节作用。     

肺动脉高压时肺小动脉弹性蛋白的表达变化

研究脉动脉压力对肺小动脉璧原弹性蛋白表达与颁的影响以帮助阐明先天性心脏病（先心病）肺动脉高压（肺高压）肺血管重建的发病机制。将１５例患儿分为对照组（组１）、先心病高肺血流组（组２）及先心病肺高压组（组３）。用免疫组化技术研究弹性蛋白在肺小动脉璧的含量与分布以了解弹性蛋白的合成情况;用原位杂交技术在转录水平研究肺小动脉间性蛋白ｍＲＮＡ的表达分布。结果：各组患儿间年龄比较无差异。组２、组３间肺动脉压力     

New therapeutic agents for pulmonary vascular disease

The improved understanding of the pathophysiology of pulmonary vascular diseases over the last decade has brought with it significant changes in disease management. Newer therapies have targeted the cardinal features of pulmonary hypertension, which include pulmonary vasoconstriction and vascular remodelling. In addition to prostacyclin, which had been the mainstay of therapy until recently, other available drugs include other prostacyclin analogues, endothelin receptor antagonists and phosphodiesterase inhibitors. This review will discuss some of the evidence for their use and will look towards the future in the search of treatments that may ultimately modify and even reverse the disease process.     

Echocardiographic findings of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum in utero

BACKGROUND: Neonates with right ventricular outflow obstruction and intact ventricular septum show serious hemodynamic problems, such as severe hypoxemia, congestive heart failure due to massive tricuspid regurgitation, respiratory distress related to huge pulmonary hypoplasia or ventricular dysfunction due to right ventricle-coronary communication. Recent advances in fetal diagnosis include many cases of in utero diagnosis of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum. METHOD: Among the fetuses examined from April 1994 to March 1998, five fetuses were found with pulmonary atresia (PA) or critical pulmonary stenosis (CPS). Fetal echocardiograms were reviewed to elucidate the accuracy of fetal information and the efficacy of fetal diagnosis in the perinatal management of patient with CPS or PA and intact ventricular septum. RESULTS: The five cases were divided into two groups: two with a very small right ventricle (group 1) and three with a tripartite right ventricle (group 2). Fetal cardiomegaly and right atrial dilatation were prominent in group 2, whereas cardiac sizes were normal in group 1. Serial fetal examination in one group 2 fetus revealed developing right ventricular hypertrophy in utero. All group 2 cases showed massive tricuspid regurgitation (TR). Estimated right ventricular pressures from TR always exceeded the systemic blood pressures of gestational age-matched neonates. Reversed flow through the ductus arteriosus was recorded in both groups and ductus-dependent pulmonary circulation after birth was anticipated. Patency of both tricuspid and pulmonary valves was difficult to recognize in utero, as was right ventricle-coronary artery communication. Four of the five cases were maternally transported and survived palliative and/or definitive intervention. One fetus with chromosomal abnormality was observed without intervention during infancy and received palliative surgery when she was two years old. CONCLUSION: Fetal hemodynamic information was useful for making decisions not only after birth, but also in utero, and may eventually result in improving the prognosis of babies with PA/CPS. Serial observation of the fetuses with PA/CPS may also suggest the possible pathogenesis of PA/CPS in utero.     

Congenital pulmonary lymphangiectasis

Background  Congenital pulmonary lymphangiectasis (CPL) characterized by dilatation of pulmonary lymphatic vessels occurs as a congenital anomaly. With poor prognosis, neonatal presentation of bilateral CPL is associated with the severe compromise of pulmonary gas exchange and high mortality. Methods  A male infant born at 39 weeks of gestation was found to have CPL. Cyanosis and cardiac arrest occurred a few minutes after birth, and the symptoms remained after artificial ventilation. The infant died of hypoxemic cardiac failure 45 minutes after birth. Autopsy showed neither pleural effusion nor valvular abnormalities. Results  Microscopically dilated vessels with lymphatics were seen in the lung of the infant. Atelectasis, CPL, inhalation of amniotic fluid, partial hydropic degeneration of hepatic cells, and scrotal edema were diagnosed. Conclusion  With regard to treatment and prognosis, CPL must be distinguished from interstitial emphysema and other diseases.     

Non-immune hydrops fetalis and bilateral pulmonary hypoplasia in a newborn infant with extralobar pulmonary sequestration

Extralobar pulmonary sequestration was found in a newborn premature infait that presented with non-immune hydrops fetalis, massive bilateral hydrothorax and polyhydramnios in utero. The baby died of severe respiratory insufficiency 15 h after birth. Postmortem examination revealed distended lymphatic vessels in the sequestered lung tissue probably due to impeded lymph drainage. We suggest that not extralobar pulmonary sequestration itself but a subsequent massive unilateral hydrothorax due to severe obstruction of lymph drainage was the cause of the non-immune hydrops fetalis, pulmonary hypoplasia and polyhydramnios. If these symptoms are diagnosed before delivery, a search for extralobar pulmonary sequestration is indicated.     

Common pulmonary vein atresia without anomalous pulmonary venous connection

Common pulmonary vein atresia without pulmonary venous connection is a rare form of congenital heart disease. No communication exists between the confluence of the pulmonary veins and the heart or a major systemic vessel. A case diagnosed antemortem is presented. Correct early diagnosis is imperative as surgery may be corrective.     

低分子肝素联合肺泡表面活性物质对幼年大鼠急性肺栓塞的治疗研究

目的探讨低分子肝素(LMH)联合肺泡表面活性物质(PS)对幼年大鼠急性肺栓塞的治疗作用。方法将雄性4周龄SD大鼠45只随机分为5组,对照组、肺栓塞组、LMH组、PS组、LMH-PS组,每组9只。除对照组外,其余各组均采用明胶海绵颗粒溶液经颈静脉注射制作急性肺栓塞模型;LMH组在术后给予LMH 0.01 ml/kg,2次/d皮下注射,PS组术后气管内灌注PS 120 mg/kg,1次/d。LMH-PS组则联合应用上述两种干预方式。对照组采用等量生理盐水颈静脉注射。各组均在术后1、4、7 d检测动脉血气、肺动脉压力(m PAP)及右心室压力(RVP)、肺组织内皮素-1(ET-1)和基质金属蛋白酶9(MMP-9)基因及蛋白表达水平和肺组织切片苏木精-伊红(HE)染色。结果各组大鼠血气分析Pa O2在1、4、7 d时的差异均具有统计学意义(P均0.05)。1 d和4 d时,LMH-PS组大鼠Pa O2分别为(85.4±6.8)和(87.8±4.7)mm Hg,显著高于栓塞组、LMH组和PS组,但低于对照组,差异均有统计学意义(P0.05)。LMH-PS组的m PAP在术后1 d时与对照组差异具有统计学意义(18.3±1.9 vs 16.3±1.5,P0.05),而在4 d和7 d时,两组差异已无统计学意义(P0.05),但LMH-PS组大鼠肺动脉压(m PAP)仍显著低于其余三组,差异有统计学意义(P0.05)。各组ET-1 m RNA及蛋白表达水平在4 d、7 d时差异均具有统计学意义(P0.05),LMH-PS组ET-1 m RNA及蛋白水平在术后4 d时迅速下降,低于其余3组,差异均有统计学意义(P均0.05),但与对照组的差异无统计学意义(P0.05)。各组MMP-1 m RNA及蛋白水平在4 d和7 d时的差异也均具有统计学意义(P0.05)。结论 LMH联合PS能够有效治疗幼年大鼠的急性肺栓塞。     

Intra-abdominal extralobar pulmonary sequestration

A left-upper-quadrant abdominal mass was identified in a routine ultrasound (US) examination in the 16th week of gestation. The sonographic features were those of a homogeneous, hyperechogenic lesion situated between the diaphragm and the left kidney. Other radiologic examinations (CT, MRI) confirmed the mass, but a presumptive diagnosis could not be made. The lesion was excised and histologic examination demonstrated an extralobar pulmonary sequestration. Although it is an uncommon type of congenital malformation, pulmonary sequestration should be included in the differential diagnosis when an echogenic intraabdominal mass is detected on antenatal US.     

儿童侵袭性肺部真菌感染

近年来,儿童侵袭性肺部真菌感染病例有上升趋势,早期诊断和及时治疗对于降低病死率非常重要.本文介绍了儿童侵袭性肺部真菌感染的发生因素、临床和影像学表现、微生物检查、诊断和治疗,以期提高对本病的诊治水平.