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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 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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??Abstract??Objective To raise awareness of pulmonary artery sling??PAS??and to improve its early diagnosis and reduce misdiagnosis. Methods By analyzing the information of two cases?? evaluate the importance of echocardiography??MRI and enhanced + 3D CT in early diagnosis. Results These two patients had major clinical manifestations of recurring bucking and vomiting??and laryngeal stridor.One case was confirmed by Enhanced + 3D CT??and the another case was confirmed by echocardiography and Enhanced + 3D CT. Conclusions The infants who had clinical manifestations such as repeatedly bucking and vomiting??laryngeal stridor??pneumonia or respiratory tract infection??and who were treated unsatisfactorily should consider PAS.Echocardiography??enhanced + 3D CT and MRI play an important role in the diagnosis of PAS.     

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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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??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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??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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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.     

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??Abstract??Objective??To determine the appropriate perioperative management of children with obstructive sleep apnea-hypopnea syndrome accompanied by pulmonary hypertension. Methods??Two children with obstructive sleep apnea-hypopnea syndrome accompanied by pulmonary hypertension were admitted to our department from April 2006 to May 2009?? their cases were analyzed and relevant literature was reviewed. Conventional adenotonsillectomy was performed for one child before controlling pulmonary hypertension. The other child received preoperative continuous positive airway pressure ventilation?? then coblation-assisted adenotonsillectomy was performed. Results??The child who had received preoperative continuous positive airway pressure ventilation underwent an uneventful operation?? no intra-or postoperative complications were reported. In contrast?? the child who did not receive preoperative pulmonary hypertension control experienced severe hypoxemia recurrently after surgery?? leading to difficulty of extubation. The child remained in the intensive care unit for two days before being transferred to the general ward. The two children were followed up for more than three months and both showed improved sleeping with less snoring?? open mouth breathing?? and dyspnea. Preoperative apnea-hypopnea indices were 90.0 and 29.2??and postoperative apnea-hypopnea indices were 3.5 and 4.6 for the children. Lowest nocturnal oxygen saturation also improved after surgery for both children ??0.43 and 0.63 vs 0.95 and 0.92??. Conclusion??Pulmonary hypertension is a rare but severe complication of obstructive sleep apnea-hypopnea syndrome in children. It can result in severe consequences or even death if not appropriately managed. Surgery is the first-line treatment for children with this syndrome who have enlarged tonsils and adenoids?? however?? the risk is considerable when accompanied by pulmonary hypertension?? and incidence of complications is high. Thus?? perioperative management with continuous positive airway pressure ventilation to control pulmonary hypertension is critical.     

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目的探讨组织多普勒(TDI)、二维斑点追踪技术(STI)和血清脑利钠肽(BNP)评估室间隔缺损(VSD)合并不同程度肺动脉高压(PH)患儿右心室功能的价值。方法 2008年6月至2010年6月在苏州大学附属儿童医院住院的VSD合并PH患儿68例,根据三尖瓣反流压差测得肺动脉压力程度分为轻度组(23例)、中度组(21例)、重度组(24例)。同时选择24名健康体检者作为正常对照组。采用GEVIVID7仪器测量右室长轴整体收缩期应变(GLS)和应变率(GLSR),三尖瓣环脉冲组织多普勒图测量三尖瓣环收缩期峰值速度(Sa)、等容收缩加速度(IVA)、Tei指数,用M型超声测量三尖瓣环收缩期位移(TAPSE)。采用ELIASA法测血清脑钠钛(BNP)浓度。结果 (1)IVA和TAPSE随着PAH升高而降低,而Tei指数随着PAH增加而增大,3个指标除正常对照组和轻度组间差异无统计学意义外(P>0.05),其他各组间差异均有统计学意义(P<0.05)。Sa在中、重度组间差异无统计学意义(P>0.05),在正常对照、轻度组间差异无统计学意义(P>0.05),其余组间有统计学意义(P<0.05)。(2)右室GLS、GLSR随着PAH升高而减小,GLS在4组间两两比较差异有统计学意义(P<0.05);GLSR中度和重度组与正常对照组和轻度组差异有统计学意义(P<0.05),中重度间及正常对照组和轻度间差异无统计学意义(P>0.05)。(3)血清BNP浓度在VSD组中随着PAH程度的加重而升高,在4组间两两比较差异有统计学意义(P<0.05)。(4)BNP与Tei指数及心功能分级相关性最好。结论 TDI和STI各个参数均能反映VSD合并PH患儿右心室功能,其中BNP和Tei指数与心功能相关性最好,而BNP和GLS可以反映轻度PAH时右室功能受损。     

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??Objective To evaluate the effect of plugging tests in the diagnosis and interventional treatment of pediatric patients with patent duct arteriosus complicated with severe pulmonary hypertension ??PAH??. Methods??All the clinical data of 5 patients of PDA with severe PAH were retrospectively reviewed??including the heart catheterization data??the relevant parameters before and after acute pulmonary vasodilator tests and the changes of Pp??Ps??PVRI??and aortic blood oxygen saturation before and after the plugging tests. Results??Only 1 patient was with dynamic PAH according to heart catheterization test??however??for the other 4 patients it was difficult to judge the property of PAH. Acute pulmonary vasodilator tests were performed in 5 patients and 4 were diagnosed with dynamic PAH. Plugging tests were conducted in 5 patients and all of them were diagnosed with dynamic PAH. All the patients underwent interventional therapy well and the follow-up results were good. Conclusion??For PDA patients with severe PAH??plugging test diagnosis can be made on the properties of the PAH and if the determination is dynamic??the intervention treatment can be completed at the same time. This method can effectively avoid the limitations of cardiac catheterization and acute pulmonary vasodilator testing evaluation of PAH properties.     

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目的 探讨右室Tei指数(RV-Tei)在评价缺氧性肺动脉高压(HPH)新生儿右室功能中的作用.方法 对2006年6月至2008年1月在新疆医科大学第一附属医院新生儿病房住院的75例HPH新生儿(HPH组,其中轻度29例、中度21例、重度25例)及22名对照组新生儿,于生后3 d以超声心动图测定肺动脉收缩压(PASP)、右室射血分数(RVEF)、三尖瓣口舒张早期峰值(E峰)、舒张晚期峰值(A峰),计算E/A值,并从血流频谱测得右室RV-Tei.结果 (1)轻、中度HPH组RVEF、E/A值与对照组比较,差异无统计学意义.重度HPH组RVEF、E/A值较对照组减小(P<0.01).(2)HPH轻、中、重3组RV-Tei均较对照组升高(P<0.01).3组间RV-Tei差异亦有统计学意义(P<0.05).(3)HPH组RV-Tei与PASP呈正相关(r=0.75,P<0.05),与胎龄、体重、心率无相关.结论 肺动脉高压引起新生儿右室功能减低,RV-Tei是评价HPH新生儿右室功能早期变化的简便而敏感的多普勒超声新指标.     

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??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.     

Zeta链在肾脏相关疾病中表达的研究进展

Zeta(ζ)链在T细胞和自然杀伤细胞信号传递功能中起着重要作用,其异常表达导致免疫功能受损可能作为某些疾病发病机制中的重要一环,已发现在系统性红斑狼疮(SLE)、肾细胞癌及血液透析患者中存在.SLE患者中,T细胞受体(TCR)ζ链的缺失率高达78%,而且,TCRζ基因和SLE的遗传易患部位同位于染色体1q,提示TCRζ链表达异常可能与SLE发病机制有关;TCRζ链的下降涉及多种分子机制.肾细胞癌患者中ζ链的水平可能与肿瘤发展的进程相关,并可能影响其预后.血液透析患者体内ζ链的表达下降可能与其细胞免疫缺陷相关.最近已发现,ζ链的表达异常存在于特发性肾病综合征患者中,其作用如何值得进一步研究.