目的:探讨华南地区汉族儿童CYP2C19基因多态性的分布,为个体化抗血小板治疗提供依据.方法:选择2015年5月至2016年5月在广州市妇女儿童医疗中心就诊的华南地区汉族儿童1311例,收集外周静脉血行CYP2C19基因rs4244285、rs4986893位点多态性检测,比较不同性别、不同地区CYP2C19代谢表型的分布特征.结果:在1311例儿童中,CYP2C19 rs4244285基因型分布为GG型46.61%、GA型43.25%、AA型10.14%,CYP2C19 rs4986893的基因型分布为GG型89.86%、GA型9.61%、AA型0.50%.CYP2C19基因型*1*1、*1*2、*1*3、*2*2、*2*3、*3*3的分布频率分别为39.36%、40.35%、6.86%、10.30%、2.59%、0.54%;CYP2C19等位基因*1、*2、*3的分布频率分别为62.97%、31.77%、5.26%.CYP2C19代谢表型分布频率为快代谢型39.30%、中间代谢型47.37%、慢代谢型13.33%,不同性别CYP2C19代谢表型分布一致(P>0.05).CYP2C19的等位基因和代谢表型分布与广州、北京、福建、湖北、重庆、云南地区比较,差异无统计学意义(均P＞0.05).结论:华南地区汉族儿童CYP2C19基因具有多态性,基因型以CYP2C19*1*2为主,等位基因以CYP2C19*1为主,代谢表型以中间代谢型为主.

为获得牛传染性鼻气管炎病毒(infectious bovine rhinotracheitis virus,IBRV)gI蛋白单克隆抗体,构建了gI截短基因重组原核表达质粒pET-32a-gI,并对其进行了诱导表达和纯化,通过SDS-PAGE和Western blot验证了gI蛋白在大肠杆菌内的表达情况.将纯化的gI蛋白免疫BALB/c小鼠,通过细胞融合、筛选及亚克隆,得到针对IBRV gI蛋白的单克隆细胞株gI14.利用体内诱生法制备抗IBRV gI蛋白单克隆抗体腹水,使用Western blot和间接免疫荧光(IFA)对单克隆抗体特异性进行检测和验证.Western blot结果显示:原核表达的gI融合蛋白相对分子质量为34 ku,gI蛋白单克隆抗体与gI表达蛋白反应性良好,与pET-32a(+)空载体蛋白无特异性结合;天然表达的gI蛋白相对分子质量为45 ku,gI蛋白单克隆抗体与细胞内感染的IBRV所表达的gI蛋白反应性良好.IFA结果显示,gI蛋白单克隆抗体及阳性对照组IBRV多抗均能与细胞内感染的IBRV发生特异性结合,使细胞出现特异性绿色荧光信号,而空白及阴性对照组均未见绿色荧光信号.结果表明,本研究基于高效表达的gI蛋白,成功制备了1株能稳定表达gI单克隆抗体的细胞株gI14,获得的gI蛋白单克隆抗体与原核表达的gI蛋白及IBRV全病毒均能特异性结合,这为进一步研制IBRV诊断试剂盒及探究IBRV gI蛋白抗原表位及蛋白功能奠定了基础.

目的:探讨中国汉族人群中药物基因CYP4F2和CYP3A4基因多态性的分布情况.方法:收集2015年5月至2016年5月在广州市妇女儿童医疗中心就诊的汉族儿童1311例为研究对象,其中男855例、女456例,包括川崎病694例、非川崎病617例.收集外周静脉血进行CYP4F2 rs2108622、CYP3A4 rs2242480基因PCR扩增和序列分析.采用Hardy-Weinberg平衡进行总体样本的等位基因分布频率检测.分析性别与上述基因的基因型分布的关系;按疾病类型进行分组,分析疾病类型与上述基因的基因型和等位基因分布的关系.结果:纳入本研究的汉族儿童1311例,基因检测显示CYP4F2 rs2108622的CC、CT、TT基因型频率分别为59.57％(n=781)、35.39％(n=464)和5.04％(n=66),其中最小T等位基因频率为22.7％;CYP3A4 rs2242480的CC、CT、TT基因型频率分别为51.26％(n=672)、40.43％(n=530)和8.31％(n=109),其中最小T等位基因频率为28.5％..按性别分组,上述基因的基因型分布组间比较差异均无统计学意义(均P>0.05).按疾病类型分为川崎病组(n=694)和对照组(n=617),川崎病组与对照组CYP3A4 rs2242480基因型分布差异有统计学意义(P<0.05),而等位基因分布差异无统计学意义(P>0.05);川崎病组与对照组CYP4F2 rs2108622基因型及等位基因分布差异均无统计学意义(均P>0.05).结论:中国汉族儿童华法林药物基因CYP4F2 rs2108622以野生型为主,而CYP3A4 rs2242480以变异型为主,包括纯合变异和杂合变异.

Objective To explore the diagnostic value of transthoracic echocardiography (TTE) for anomalous origin of right pulmonary artery (AORPA).Methods Echocardiographic data of 26 patients with AORPA proved by surgical operation were analyzed retrospectively.Results TTE showed that the pulmonary trunk and the left pulmonary artery were displayed normally,and the right pulmonary artery originated from the ascending aorta.AORPA in all 26 patients were diagnosed,and the coincidence rate was 100％.Among 26 patients,9 associated with Berry's syndrome,1 combined with coarctation of aorta,22 combined with patent ductus arteriosus,23 combined with atrial septal defect or patent foramen ovale,25 were found with severe pulmonary artery hypertension,and the diagnostic accuracy rate of TTE was 100％ (26/26),100％ (26/26),96.15％ (25/26),92.31％ (24/26) and 100％ (26/26),respectively.Conclusion TTE can early and accurately diagnose AORPA,also has high accuracy in diagnosis of other concomitant malformations.Multi-section scan can reduce misdiagnosis.TTE can be taken as the preferred inspection method for diagnosing AORPA.

目的 评估VKORC1基因多态性对川崎病(KD)患儿华法林稳定剂量的影响.方法 对临床诊断KD巨大冠状动脉瘤(GCAA)、口服稳定剂量华法林≥2个月,同时期INR稳定在2.0～2.5≥2个月的患儿在华法林使用前或后行CYP2C9?2(rs1799853)、CYP2C9?3(rs1057910)和VKORC1(rs9923231)基因多态性检测,评估VKORC1基因多态性、调整华法林剂量时的年龄、体重、身高、体表面积与华法林稳定剂量的相关性.评估KD患儿华法林稳定剂量的主要影响因素,随访观察应用华法林后出现的不良反应.结果 42例进入本文分析,其中男35例,女7例,年龄为0.5～6.7岁,稳定华法林剂量为(1.47±0.45)mg·d-1,经体重矫正后稳定华法林剂量为(0.11±0.033)mg·kg-1·d-1.VKORC1 CT型6例、TT型36例,经体重矫正后华法林稳定剂量分别为(0.16±0.043)、(0.10±0.021)mg·kg-1·d-1,差异有统计学意义(P<0.05).检测基因之后用药比检测之前用药达稳态时间缩短(P<0.05).多元回归分析显示,经体重矫正华法林稳态剂量(mg·kg-1·d-1)=0.039+0.061×VKORC1 rs9923231基因型(1 if TT,2 if CT),R2为43.8％,华法林VKORC1 rs9923231基因型可解释6个月至7岁KD患儿华法林稳定剂量个体差异的43.8％.未经体重矫正华法林稳态剂量(mg·d-1)=-0.407+0.088×体重+0.580×VKORC1 rs9923231基因型(1if TT,2 if CT),体重和VKORC1 rs9923231基因型的R2分别为43.7％和19.5％,根据最佳回归模型得到的华法林稳定剂量预测公式可解释6个月至7岁川崎病儿童华法林稳定剂量个体差异的63.2％,其中VKORC1基因多态性、体重的贡献分别是19.5％和43.7％.因能解释更多华法林稳态剂量的个体差异,未经体重矫正华法林稳态剂量预测公式优于经体重矫正稳态剂量预测公式.结论 VKORC1 rs9923231基因型是影响6个月至7岁KD并发CAA患儿华法林稳定剂量的遗传因素之一,体重是华法林稳定剂量的主要影响因素.对于常规剂量INR易超标伴出血或不能达到目标INR的患儿,药物基因筛查有助于快速、有效指导用药,减少出血的并发症.

Objective To evaluate and analyze echocardiography(ECG) in diagnosis of congenital vascular rings (CVR) to elevate accuracy of diagnosis.Methods 103 cases of children with CVR confirmed by surgery were analyzed retrospectively.We reviewed the ECG and CTA characteristics of this disease with the surgical results as the gold standard.Results Among the 103 CVR patients,61 cases were pulmonary artery sling (PAS),ECG sensitivity was 96.7 ％ (59/61),missed diagnosis rate was 3.3 ％ (2/61).21 cases were double aortic arch(DAA),ECG sensitivity was 57.1％ (12/21),missed diagnosis rate was 42.9 ％ (9/21).19 cases were right aortic arch with an aberrant left subclavian artery(RAA-ALSA),ECG sensitivity was 15.8％(3/19),missed diagnosis rate was 84.2％ (16/19).2 cases were left aortic arch with aberrant right subclavian artery(LAA-ARSA).All the CVR cases were diagnosed by CTA.77 CVR combined with intracardiac malformation and all diagnosed byECG and the missed diagnosis rate was 11.7 ％ for CTA.Conclusions Missed diagnosis are prone to take place in DAA and an aberrant subclavian artery than in PAS with ECG.CTA could show clearly the relationship of vascular rings and trachea.So integration of the two diagnosis methods could hand out more information to operation plan.

1病例报告 患儿,女,10岁,因"咳嗽4天,加重伴发热1天"于2016年3月4日就诊于东莞康华医院.入院4天前开始出现咳嗽、咳脓痰,伴喘息,2016年3月1日到东莞塘厦医院就诊,行血常规检查,结果提示白细胞13.16×10.9/L,C反应蛋白31.07mg/L,静脉输液1次(具体用药不详),咳嗽无缓解,1天前开始发热,咳嗽加重,2016年3月4日来本院就诊,门诊以"支气管肺炎"收入儿科病房.

Objective To summarize the clinical characteristics, follow-up and prognosis of Kawasaki disease (KD) in infants under 3 months. Method The clinical data of infants under 3 months diagnosed with KD during January 2009 to December 2013 were analyzed retrospectively. Results In a total of 1504 cases diagnosed with KD during during the study period, there were 40 (2.66%) infants under 3 months (30 males and 10 females). Except for fever, the frequencies of the other 5 main clinical manifestations were less than 50%. Laboratory tests suggested inflammatory reactions, thrombocytosis, anemia, and so on. Coronary artery disease was found in 32 cases (80%), including 24 cases of coronary artery dilatation, 8 cases of coronary artery aneurysm and 3 cases of coronary giant aneurysm. By the end of the follow-up period, there were no myocardial ischemia, myocardial infarction and death in all the patients; coronary artery diameter was normal in 37 cases (92.5%); 3 cases of giant coronary artery aneurysm still had coronary artery aneurysm and thrombosis. Conclusion The clinical manifestations of KD in little infants were atypical, the incidence of coronary artery disease is high, and color doppler echocardiography should be performed in time.

Objective To investigate the gene polymorphisms of warfarin-related CYP2C9 and VKORC1 in Han population in southern China. Methods Included in this study were a total of 1311 children of Han ethnicity from southern China who visited Guangzhou Women and Children's Medical Center between May 2015 and May 2016. Among them,there were 855 males and 456 females,comprising 694 cases of Kawasaki disease and 617 cases of non-Kawasaki disease. Peripheral venous blood was collected for PCR amplification and sequence analysis of CYP2C9*3 rs1057910,VKORC1 rs9923231,and VKORC1 rs9934438 genes. The relationship between gender and genotype distribution of these genes was analyzed. Stratified by disease category,the relationship between disease category and the genotypes and allelic distribution of these genes was also analyzed. Results Among the 1311 children of Han ethnicity from southern China,gene detection showed that the frequency of A allele in CYP2C9*3 rs1057910 was 0.9607, the proportion of genotype distribution was 92.37%for AA,7.40%for AC,and 0.23%for CC;the T allele frequency in the VKORC1 rs9923231 gene was 0.8951,the proportion of genotype distribution was 80.32%for TT,18.38%for CT,and 1.30%for CC;the frequency of the A allele in the VKORC1 rs9934438 gene was 0.8970,the proportion of genotype distribution was 79.94%for AA,18.00%for AG,and 1.30%for GG. There was no statistically significant difference in the genotypic distribution of these genes between both genders(all P>0.05). According to the diseases in these children categorized as Kawasaki disease group(n=694)and control group(n=617),there was no significant difference in genotype and allele distribution of these genes between two groups(all P>0.05). Conclusion Han children from southern China carry wild-type CYP2C9*3 rs1057910,and VKORC1 rs9923231 and rs9934438 mutant variants as the predominant warfarin-related genotypes.

Purpose Unilateral absence of pulmonary artery is a rare congenital pulmonary arterydysplasia whose untypical symptom often leads to missed diagnosis or misdiagnosis.Diagnostic and follow-up values of ultrasound cardiogram to pediatric congenital unilateral absence of pulmonary artery (UAPA) are explored in this paper.Materials and Methods Ultrasound cardiogram images of 35 UAPA patients confirmed by surgery or CT angiography in Guangzhou Women and Children's Medical Center from May 2009 to July 2016 were analyzed retrospectively,and images characteristics of the correctly diagnosed and missed diagnosis or misdiagnosed ultrasound cardiogram were analyzed.Results Among these 35 UAPA patients,21 cases were on the right side,14 cases on the left side,16 cases single UAPA and 19 cases combined with other intracardiac malformations.27 cases were initially diagnosed by ultrasound cardiogram,7 cases were missed diagnosed and 1 case misdiagnosed,with 77.1％ diagnosis accuracy.UAPA ultrasound cardiogram showed that normal bifurcation structure of pulmonary artery disappears.Main pulmonary artery continued to be one-side pulmonary artery,running to left or right and forming left branch or right branch of pulmonary artery.Pulmonary artery of the one-side absence of pulmonary segment supplied blood through aorta or other branches or vessels on the other side.After receiving unilateral pulmonary artery reconstruction surgery,ultrasound cardiogram of 8 patients of one month indicated that average flow rate of close-end anastomosis was (1.22± 0.17) m/s and far-end (2.17± 0.56) m/s.Conclusion Ultrasound cardiogram is efficient for UAPA preoperative diagnosis.Meanwhile,it is convenient,noninvasive and efficient in assessing anastomosis and pressure of pulmonary artery after surgery.

Objective To summarize the clinical features,treatment and prognosis of infantile hepatic hemangioendothelioma(IHHE) combined with congestive heart failure(CHF) in children.Methods A retrospective analysis was made with the clinical data from 4 children with IHHE combined with CHF in Department of Pediatric Cardiology,Affiliated Guangzhou Women and Children's Medical Center of Guangzhou Medical University from May 2013 to December 2015.Results Among 4 infants,there were 3 male and 1 female,mean aged of 109 days (21-219 days),the average weight of 4 350 g (2 750-6 500 g),and 1 case was admitted because of abdominal swelling and respiratory distress,1 case of abdominal swelling,jaundice,shortness of breath admission,1 case of fever and shortness of breath admission,and 1 case of cyanosis and shortness of breath admission.Two patients were associated with hemangioma and Kasabach-Merritt syndrome.Three cases had multiple liver hemangioma scattered in the left lobe and right lobe of the liver,and 1 case had solitary liver hemangioma scattered in the right lobe.Tumor size diameters were ranged from 2-10 mm.Four children had been used Prednisone [1-2 mg/(kg · d)] and Digoxin,Dopamine,Furosemide,Spironolactone.Two cases responsed well to the therapy and were followed up for 2 years or 1 year and 6 months respectively,whose heart size,heart function and pulmonary hypertension became normal.One case with Kasabach-Merritt syndrome responsed poorly to the therapy.Two cases experienced transcatheter hepatic arteriovenous fistula embolization by using coil occlusion of hepatic artery-venous fistula.The postoperative hepatic tumor volume was significantly decreased,and the heart function was improved.One patient was followed up for 1 year and 6 months,and the heart size,pulmonary hypertension and heart function returned to normal.One case was still under follow-up.One case with Kasabach-Merritt syndrome died from heart failure and severe infection before surgery.The mortality rate was 25％ (1/4 cases).Conclusions IHHE is a rare cause of CHF in children.If heart failure is found unexplained in children,abdominal ultrasound or CT examination should be done immediatey in order to confirm the presence or absence of IHHE.The mortality rate in IHHE associated with CHF is very high.Early identification,early intervention,especially Prednisone combined with transcatheter hepatic arteriovenous fistula embolization can significantly improve heart function,reduce the mortality and improve the prognosis of IHHE associated with CHF in children.

OBJECTIVE To evaluate the prognosis and clinical features of patients with giant coronary artery aneurysm (GCAA) caused by Kawasaki disease (KD). METHOD KD complicated with GCAA was diagnosed in 55 patients between January 2003 and December 2012 in Guangzhou Women and Children's Medical Center.Of the 55 patients, 48 were studied(43 boys, 5 girls). According to the follow-up time, these patients were divided into four groups: ≤ 1 year follow-up group, > 1-3 years follow-up group, > 3-5 years follow-up group, and > 5-10 years follow-up group. These patients were investigated at the acute stage, and were followed up respectively at two weeks, one month, three months, six months, and one year after hospitalization and every 3 to 6 months after a year. All patients regularly accepted echocardiography and electrocardiographic examination, while some cases accepted CT coronary angiography (CTA) examination and coronary angiography (CAG) examination to confirm the condition of GCAA. RESULT (1) The age of 48 patients ranged from 2 months to 10 years. Twenty cases were less than 1 age (42%), 30 cases were under 3 years of age (62%) and follow-up time was (4.0 ± 3.1) years. Age at endpoint was 1.5 to 19 years, mean (6.8 ± 4.2) years respectively. (2) In ≤ 1 year follow-up group, the proportions of no significant changes, retraction, and stenosis were 48%, 48% and 4% respectively. In > 1-3 years follow-up group, the proportions of no significant changes, retraction, and stenosis were 39%, 39% and 22% respectively. In > 3-5 years follow-up group, the proportions of no significant changes, retraction, and stenosis were 30%, 35% and 35% respectively. In > 5-10 years follow-up group, the proportions of no significant changes, retraction, stenosis were 30%, 20% and 50% respectively. Compared with the ≤ 1 year group, a significant increase in the proportion of coronary artery stenosis occurred at the other three groups.Significant difference in the stenosis rate could be found between the ≤ 1 year group and the other three groups respectively (χ(2)=6.026, 11.121, 15.652; P=0.019, 0.002, 0.001). (3) The rate of retraction in bilateral GCAA group (20 cases) was lower than unilateral GCAA group (28 cases ); bilateral GCAA group had lower rate than the unilateral group(15% (3/20) vs. 36% (10/28)). There was no significant difference in coronary artery outcome between two groups (P > 0.05) . (4) There were six severe ischemic heart disease in 48 cases including 2 deaths. (5) CTA showed coronary artery wall thickening, mural thrombus and calcification, the CAG could display coronary artery occlusion and recanalization and collateral vessels formation. CONCLUSION KD complicated with GCAA may occur in infants under one year of age, especially infants under 6 months of age. A significant increase in the proportion of coronary artery stenosis occurred with the follow-up time extended. The proportion of bilateral GCAA patients who had ischemic heart disease and died was higher than the unilateral cases. The combined application of echocardiography, CTA and CAG may greatly help to discover coronary thrombosis, stenosis and occlusion.

Objective To discuss the value of color Doppler echocardiography in diagnosis of scimitar syndrome .Methods The echocardiographic results of 6 patients with a diagnosis of scimitar syndrome were reviewed retrospectively .Their sonographic and hemodynamic characteristics were also analyzed connected with the reports in the literature .Results Three cases had dextrocardia and the others had mesocardia .All cases got right ventricular dimension enlargement .Total or partial of right pulmonary venous connection to the inferior vena cava were 3 cases respectively .All cases had right pulmonary artery hypoplasia .All of 6 cases echocardiographic results were in accordance with the findings by CT angiography and 4 cases were confirmed by operation .Conclusions The sonographic features of scimitar syndrome were obvious ,and echocardiography was contribute to early diagnosis of scimitar syndrome .

Objective To explore the effects and follow-up findings of emergency and sub-emergency transcatheter closure of patent ductus arteriosus (PDA) in infants.Methods A retrospective study was performed to analyze the effects,complications, immediate and mid-term follow-up outcomes in 12 infants with PDA from Guangzhou Women and Children's Medical Center between March 2008 and April 2014 in order to evaluate the efficacy and safety of transcatheter closure of PDA in infants.All cases were diagnosed preoperatively as pneumonia, and 10 cases had congestive heart failure.Pulmonary symptoms showed no obvious change after regular internal medicine therapies.Results Twelve devices (100％) were successfully implanted.Echocardiogram was performed 3 days after the procedures and showed no residual shunt in any case.Left ventricular end-diastolic dimension [(31.3 ± 5.4) mm] ,left atrial diameter[(18.2 ± 3.4)mm], left ventricular ejection fraction (LVEF) [(56.8 ± 8.7) ％], pulmonary artery systolic pressure and mitral regurgitation were decreased or alleviated in contrast with those before the procedures(all P ＜ 0.05), but systolic aortic pressure was increased(P ＜0.01).Follow-up was conducted in 10 cases (83％) and there was no residual shunt in any case.LVEF was improved and MR was significantly alleviated 6 months after the procedure compared with those 3 days after the procedure (all P ＜ 0.05).Aortic regurgitation and tricuspid regurgitation 6 months after procedure unchanged (P ＞ 0.05).Left pulmonary artery blood flow velocity was increased in 3 cases.The maximal descending aorta blood flow velocity was 1.9 m/s and 2.4 m/s respectively in 2 cases, blood flow velocity of left pulmonary artery and descending aorta were blow 2.3 m/s, pressure gradient was less than 2.9 kPa in 23-28 months after the procedure and no patient received any other intervention.Conclusions Emergency and sub-emergency transcatheter closure of PDA in infants is safe and feasible, but long-term follow-up is needed in patients with increased left pulmonary artery blood flow velocity and/or descending aorta blood flow velocity.

ObjectiveTo assess the values of 320-detector row dynamic volume CT angiography (CTA) and transthoracic echocardiography (TTE) in follow up of coronary artery aneurysm (CAA) caused by Kawasaki disease (KD).Methods320-de-tector row CTA and TTE were applied in long-term follow-up of 8 patients with CAA caused by KD.ResultsIn 8 patients, the mean age at onset was 41.63±22.70 months and the mean follow up time was 43.50±10.99 months. In acute phase, 3 cases of giant coronary artery aneurysms (GCAA) and 5 cases of mid-small CAA were diagnosed by TTE. A total of 16/32 arteries (50%) were involved. At the end of follow-up, 3 cases of GCAA and 2 cases of mid-small CAA were still diagnosed by TTE, and small CAAs were regressed in another 3 cases. A total of 6/32 arteries (18.75%) were involved. Simultaneously at the end of follow-up, a total of 7/32 arteries (21.9%) were involved by 320-detector row CTA. The distribution was consistent with that of TTE. Mean-while, there were one case of left circumlfex artery, one case of GCAA at distal of the right coronary artery, 2 cases of thrombus, 1 case of coronary stenosis and 2 cases of calciifcation.ConclusionsCAA caused by KD may be persistent for a long time. The thrombus, stenosis, and calciifcation of coronary can occurr at late phase in GCAA. TTE is sensitive and reliable to detect proxi-mal and middle segment of coronary lesions, but has limitations in detection of distal segment of coronary arteries. 320-detector row CTA has more comprehensively view of each coronary artery lesions and is especially sensitive and reliable to detect coro-nary thrombosis, calciifcation and narrowing in proximal and distal coronary arteries after acute phase.

1病例资料<br>　　男，8月龄，因肺炎入住广州市妇女儿童医疗中心。查体：未见特殊面容，体重6.3 kg（较同龄儿稍落后）；血压：右上臂84/32 mmHg，右下臂110/40 mmHg；安静状态不吸氧经皮氧饱和度0.88；颈静脉未见怒张，肝颈静脉回流征阴性，四肢末梢未见发绀，无杵状指趾。心界向左下扩大，心率145·min-1，律齐，胸骨左缘2~4肋间可闻及Ⅱ级连续性杂音，无周围血管征。

Objective To describe early clinical experience with the new amplatzer ductal occluder Ⅱ (ADO Ⅱ) for transcatheter patent ductus arteriosus(PDA) in children.Methods Twelve children were diagnosed as PDA from Jan.2013 to Apr.2014 in Guangzhou Children and Women's Hospital.All the children who were treated with the ADO Ⅱ had the indication of a successful interventional therapy successfully.The size of device was chosen according to aortographic and transthoracic echocardiography(TTE) results and pulmonary pressure.The device was delivered in a consequent or reverse way depending on the type of PDA,the minimal diameter of PDA and the size of duct ampulla.The device was delivered in a reverse way in ten patients,and two in a consequent way before detaching the device.Another aortogram was performed in order to check the position and form of the device,the velocities of blood flow in left pulmonary artery and the descending aorta though TTE and whether there was a residual shunt.All patients were examined by TTE in 24 hours after surgery and discharged without any complications 2 days later.The patients were programmed for the cardiologic consult including an TTE and electrocardiogram in 1,3,6 and 12 months after discharge.Results Twelve patients(7 male,5 female) with a median age of(1.59 ± 1.10) years(range 0.53-4.47 years),a median weight of (9.52 ± 3.41) kg(range 5.5-18.3 kg),a median pulmonary blood flow/systemic blood flow (Qp/ Qs) of 1.64 ± 0.45 (range 1.33-2.85),a median pulmonary artery systolic pressure (32.50 ± 10.05) mmHg (range 23-58 mmHg,1 mmHg =0.133 kPa),and the minimum (2.40 ±0.68) mm (1.6-3.8 mm),underwent transcatheter ductal closure with the ADO Ⅱ.Device sizes used were 3 mm ×4 mm(n =7),3 mm ×6 mm(n =3),6 mm ×6 mm (n =2),respectively and delivered with 4 or 5 F delivery catheters.The median fluoroscopy time was (6.39 ± 4.16) min(range 3.2-18.2 min).Complete ductal occlusion was achieved by the end of the procedure in 10 patients.The TTE showed good position of the occlusion and the velocities of blood flow in left pulmonary artery and the descending aorta were in a normal range.There was a trivial residual shunt after the surgery of 2 patients.No residual shunt was found after 24 hours in all 12 patients.In 1 case,the patient had a descending aortic obstruction with pressure gradient of 11 mmHg.Three months after surgery,the pressure descended to 10 mmHg by TTE.Complete ductal occlusion without aortic arch or left pulmonary artery stenosis had been identified in other 11 remaining patients on TTE follow-up of 6 months of 3 patients and 12 months of 6 patients.Conclusions The ADO Ⅱ achieves excellent ductal closure rates through low profile delivery systems in small infants and children with moderate and small PDA or morphologically varied PDAs.It is simple in use with few complications.Occlusion design allows closure with arterial or venous approach and delivery with 4 or 5 F delivery catheters.The children who used arterial approach,transthoracic echocardiography TTE is recommended to replace aortic angiography,so as to avoid puncturing the aorta and reduce vascular injury.

The main complications of Kawasaki diseases(KD) is relative to cardiovascular,especially coronary artery.Coronary artery lesions can be showed as arteritis,artery dilatation,aneurysm,arterial stenosis and so on.As a noninvasive,convenient,fast and well reproducible inspection method,echocardiography can offer dynamically observa tion of the evolution process of coronary artery lesions in each stage,accurately measure the coronary artery diameter,evaluate heart function in children,find other cardiac complications and which has become a standard diagnosis technics.With upgrades and new technology,the echocardiography plays a more and more important role in the diagnosis of acute period of KD and observation of curative effect,prognosis and long-term follow-up of recovery period of KD.

目的：探讨胸骨上窝特殊切面连续扫描探查在小儿双主动脉弓超声诊断中的应用价值，旨在提高超声对双主动脉弓诊断的准确率。方法针对在我科常规进行超声心动图检查的患儿，从剑下、心尖四腔、五腔，左室长轴、短轴、胸骨旁、胸骨上窝等部位顺序连续扫描进行检查，详细检查心房、心室、大动脉的位置，房室连接、心室大动脉连接关系。结果4000余例经过胸骨上窝长短轴及过渡切面连续扫描排除主动脉弓异常，2例经上述特殊切面扫描心脏彩超诊断为双主动脉弓所致血管环，1例行心脏CT进一步证实该诊断并在全麻下行“双主动脉弓矫治术”。另1例患儿无明显临床症状在随访中。结论超声心动图能较准确地诊断小儿双主动脉弓，关键在于胸骨上窝特殊切面的连续扫描及细致探查。

>患者,女,59岁。因"面色苍白、体倦乏力"于2013年9月30日就诊于天津医科大学总医院。体检重度贫血,中度脾大。骨髓活检病理检查示骨髓有核细胞增生较活跃（占0.75）,粒、红系细胞比例大致正常,粒系细胞以偏成熟阶段的细胞为主,细胞形态未见明显异常。红系以中晚幼阶段细胞为主,巨核细胞数量在正常范围,主要为分叶核巨核细胞。小淋巴细胞单一性小片状、灶性及散在性分布。免疫组化:CD20阳性,CD138偶见阳性,CD38少数阳性,CD43和CD3阴性。考虑为B细胞边缘区淋巴瘤累及骨髓。流式细胞术分析免疫表型:CD20 + 包率67%,CD22 + 细胞率58%,CD5 + 细胞率23%,CD19 + 细胞率67%,CD79a + 细胞率64%,K + CD19 + 细胞/CD19 + 细胞为99.31%,λ + CD19 + 细胞/CD19 + 细胞为2.07%,CD3、CD7、CD23、CD10均阴性。诊断:B-淋巴瘤